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Sample records for pediatric moyamoya disease

  1. Applicability of PROSET-MRA for evaluating pediatric moyamoya disease

    International Nuclear Information System (INIS)

    Tsujimura, Asuka; Kojima, Hideyuki; Yabe, Hitoshi

    2011-01-01

    MR angiography (MRA) for pediatric moyamoya disease is important as a non-invasive examination to diagnose blood flow in the brain. Generally, the conventional 3D-time of flight (TOF) MRA is used for moyamoya disease. However, retrobulbar and subcutaneous fat of the head show high intensity signals. We found that using the conventional MRA to diagnose the details of brain blood flow is difficult and that it cannot differentiate moyamoya vessels and fat. It similarly obscures the ophthalmic artery and superficial temporal artery that overlap with fat in the direction of the maximum intensity projection (MIP). Therefore, we devised an MRA technique with fat suppression to diagnose blood flow in moyamoya disease patients: MR angiography with the principle of selective excitation technique (PROSET). The scan time does not need to be increased. We studied the TOF effect in constant and pulsatile flows and the water selective excitation method with the binominal pulse (PROSET) for the fat suppression effect for moyamoya disease. The results showed that PROSET-MRA achieved better image results than conventional MRA. The development of collaterals of the superficial temporal artery and occipital artery in pre- and post-operation moyamoya disease could be clearly visualized and evaluated. The PROSET-MRA method is useful for evaluating pre- and post-operation (encephalo-duro-arterio-synangiosis, encephalo-myo-synangiosis) blood flow reconstruction for patients who have moyamoya disease. (author)

  2. Treatment of pediatric moyamoya disease by simultaneous bilateral dual EDASs

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    Shimoji, Takeyoshi; Nagamine, Tomoaki; Yamashiro, Katumi

    2007-01-01

    Treatment of moyamoya disease should be considered, especially in children in whom the disease develops rapidly and causes mental retardation. To address these concerns, we have been treating these patients with simultaneous bilateral dual encephaloduro-arterio-synangiosis (EDAS). The patients were 12 children and one adult. Ten of the children developed symptoms under 6 years of age. Their initial symptoms were transient ischemic attack (TIA) in 7, convulsions in 2, hemiparesis secondary to infarction and hemorrhage in 2, TIA and convulsion in 1, and headache in 1. All patients had diagnosis made by the cerebral angiography. All of them underwent EDAS using anterior and posterior branches of superficial temporal artery simultaneously and bilaterally. In this procedure, it is important to dissect both branches more than 10 cm. The mean operation time was 8 hours 25 minutes. Postoperatively, two patients developed hemiparesis secondary to cerebral infarction; both, however, recovered with the aid of rehabilitation. TIAs decreased immediately after surgery and disappeared in a couple of years except in one case. Convulsions ceased immediately. One patient with pre-op TIA developed convulsions 2 years after surgery. Headaches decreased in frequency. One developed cerebral infarction after surgery and mental status deteriorated, but the others maintained stable mental condition post-operatively. Post-operative angiographies were performed 3 and 9 months after surgery. Most patients attained excellent revascularization in the frontal to parietal regions except for three cases. Two of them finally showed good anastomosis 2 and 8 years later. One remained poor because the patient still had early stage of moyamoya disease. It may be postulated that the use of simultaneous bilateral dual EDAS prevents the rapid progression of and the development of mental problems seen in child moyamoya disease. (author)

  3. Changes to the geometry and fluid mechanics of the carotid siphon in the pediatric Moyamoya disease.

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    Jamil, Muhammad; Tan, Germaine Xin Yi; Huq, Mehnaz; Kang, Heidi; Lee, Zhi Rui; Tang, Phua Hwee; Hu, Xi Hong; Yap, Choon Hwai

    2016-12-01

    The Moyamoya disease is a cerebrovascular disease that causes occlusion of the distal end of the internal carotid artery, leading to the formation of multiple tiny collateral arteries. To date, the pathogenesis of Moyamoya is unknown. Improved understanding of the changes to vascular geometry and fluid mechanics of the carotid siphon during disease may improve understanding of the pathogenesis, prognosis techniques and disease management. A retrospective analysis of Magnetic Resonance Angiography (MRA) images was performed for Moyamoya pediatric patients (MMD) (n = 23) and control (Ctrl) pediatric patients (n = 20). The Ctrl group was composed of patients who complained of headache and had normal MRA. We performed segmentation of MRA images to quantify geometric parameters of the artery. Computational fluid dynamics (CFD) was performed to quantify the hemodynamic parameters. MMD internal carotid and carotid siphons were smaller in cross-sectional areas, and shorter in curved vascular length. Vascular curvature remained constant over age and vascular size and did not change between Ctrl and MMD, but MMD carotid siphon had lower tortuosity in the posterior bend, and higher torsion in the anterior bend. Wall shear stress and secondary flows were significantly lower in MMD, but the ratio of secondary flow kinetic energy to primary flow kinetic energy were similar between MMD and Ctrl. There were alterations to both the geometry and the flow mechanics of the carotid siphons of Moyamoya patients but it is unclear whether hemodynamics is the cause or the effect of morphological changes observed.

  4. Revascularization surgery for pediatric moyamoya disease. Significance of peri-operative management to avoid surgical complication

    International Nuclear Information System (INIS)

    Fujimura, Miki; Tominaga, Teiji

    2011-01-01

    Moyamoya disease is a chronic occlusive cerebrovascular disease with unknown etiology, which is one of the most common causes of child-onset stroke in Japan. Surgical revascularizations, both direct and indirect procedures, prevent cerebral ischemic attack by improving cerebral blood flow, while neurological deterioration during the acute stage after revascularization is not rare. The objective of this study was to clarify the concept of revascularization surgery for pediatric moyamoya disease while considering the risk of surgical complications in the acute stage. The present study includes 19 consecutive patients with moyamoya disease aged from 2 to 14 years old (mean 8.5), who underwent superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis with indirect pial synangiosis for 32 affected hemispheres. Single-photon emission computed tomography (SPECT) was performed 1 and 7 days after surgery in all cases to evaluate hemodynamic alteration after surgery. Long-term outcome was evaluated by the neurological status 3 months after surgery, and the underlying pathology of surgical complications in the acute stage was diagnosed based on SPECT and magnetic resonance findings. In 28 of 32 hemispheres (87.5%), patients showed a complete disappearance of ischemic attack, 4 of 32 hemispheres (12.5%) showed a reduction of ischemic attack, while none showed deterioration of their symptoms (0%). Transient focal neurologic deterioration due to cerebral hyperperfusion was evident in 2 patients (6.3%), and was resolved by blood pressure lowering. One patient developed pseudo-laminar necrosis probably due to a thrombosis one week after surgery (3.1%), which did not affect his long-term outcome. STA-MCA anastomosis with pial synangiosis is a safe and effective treatment for pediatric moyamoya disease. Routine cerebral blood flow measurement in the acute stage is essential to avoid surgical complications including both cerebral ischemia and hyperperfusion. (author)

  5. Moyamoya disease: Diagnostic imaging

    International Nuclear Information System (INIS)

    Tarasów, Eugeniusz; Kułakowska, Alina; Łukasiewicz, Adam; Kapica-Topczewska, Katarzyna; Korneluk-Sadzyńska, Alicja; Brzozowska, Joanna; Drozdowski, Wiesław

    2011-01-01

    Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. The incidence of moyamoya disease is high in Asian countries; in Europe and North America, the prevalence of the disease is considerably lower. Clinically, the disease may be of ischaemic, haemorrhagic and epileptic type. Cognitive dysfunction and behavioral disturbance are atypical symptoms of moyamoya disease. Characteristic angiographic features of the disease include stenosis or occlusion of the arteries of the circle of Willis, as well as the development of collateral vasculature. Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease. The most common surgical treatment combines the direct arterial anastomosis between the superficial temporal artery and middle cerebral, and the indirect synangiosis involving placement of vascularised tissue in the brain cortex, in order to promote neoangiogenesis. Due to progressive changes, correct and early diagnosis is of basic significance in selecting patients for surgery, which is the only effective treatment of the disease. An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures. Despite the rare occurrence of moyamoya disease in European population, it should be considered as one of causes of ischaemic or haemorrhagic strokes, especially in young patients

  6. Moyamoya Disease Mimicking Encephalitis

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    Maryam Khalesi

    2014-09-01

    Full Text Available Moyamoya disease is a rare vaso-occlusive illness with an unknown etiology characterized by stenosis of the internal carotid arteries with spontaneous development of a collateral vascular network. A 15-month-old girl was referred to the emergency ward of Imam Reza Hospital due to decreased level of consciousness, focal seizures and fever during the previous 24 hours with an impression of encephalitis. Physical examination revealed left side hemiparesis; however brain CT-Scan did not show any significant lesions. Initial therapy with vancomycin, ceftriaxone and acyclovir was administered. CSF analysis did not show any abnormality and the blood as well as CSF cultures results were negative. Brain MRI showed hyperintensity at right frontal and parietal regions, suggesting vascular lesion. Magnetic resonance angiography (MRA showed bilaterally multiple torsions in vessels at the basal ganglia consistent with moyamoya vessels. In all children exhibiting encephalitis, vascular events such as moyamoya disease should be considered. Brain MRI is a critical tool for this purpose. Common causes of encephalitis such as herpes simplex should also be ruled out.

  7. Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease - A case report and brief review of literature

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    Soumya Patra

    2012-01-01

    Full Text Available Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.

  8. Genetics Home Reference: moyamoya disease

    Science.gov (United States)

    ... as neurofibromatosis type 1 , sickle cell disease , or Graves disease . These individuals are said to have moyamoya syndrome. ... altered gene in each cell is sufficient to cause the disorder. However, some people who have a ...

  9. Assessment of the hemodynamic changes after EDAS combined with bifrontal EGS in pediatric patients with moyamoya disease

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    Song, Yoo Sung; Kim, Yu Kyeong; Lee, Jae Sung; Kim, Seung Ki; Lee, Dong Soo; Chung, June Key; Lee, Myung Chul [Seoul National Univ. College of Medicine, Seoul (Korea, Republic of)

    2007-07-01

    To assess the effect of encephaloduroarteriosynangiosis (EDAS) with or without bifrontal encephalogaleosynangiosis (EGS) in children with moyamoya disease, we evaluated cerebral hemodynamic changes using brain Tc-99m HMPAO SPECT. Total 34 pediatric patients (M: F=12:22, mean age;93 yrs) enrolled. Bypass surgery for both hemispheres (EDAS with EGS on one side, and EDAS on the other side) in 25 patients, unilateral EDAS with EGS in 7, and unilateral EDAS only in 2 were underwent. Perfusion SPECT before surgery, and 4 to 18 months after final surgery were done. The vascular territories for ICA, MCA and the brain regions for the frontal, parietal, temporal, and the occipital cortices were determined using standard ROls based on K-SPAM. Additionally, medial frontal cortex was selected to assess the effect of EGS. Basal/acetazolamide challenged cerebral blood flow (CBF), and cerebral vascular index (CVRI) were determined using normalized regional brain uptake to the cerebellum. 24 patients became symptom free, and 6 were improved but having some residual symptoms at the last follow up period. The other 3 were worsened after operation. Overall basal/acetazolamide stress CBF and CVRI for each brain region after surgery were increased, however, only the changes of CVRI were significant. Meanwhile, the improvement of CBF or CVRI in the brain regions ipsilateral to the hemisphere having EDAS with EGS was not significantly different when compared with those for the brain regions with EDAS only. Also, the hemodynamic improvement for the mesial frontal cortex in patients after EDAS with EGS was not significant, and showed no difference with those in patient with EDAS only. Quantitative perfusion SPECT demonstrated the hemodynamic improvement after EDAS with or without EGS in pediatric moyamoya disease. Cerebrovascular reserve showed meaningful improvement after surgery, implicating the effect of vascular anastomosis in ischemic areas.

  10. Assessment of the hemodynamic changes after EDAS combined with bifrontal EGS in pediatric patients with moyamoya disease

    International Nuclear Information System (INIS)

    Song, Yoo Sung; Kim, Yu Kyeong; Lee, Jae Sung; Kim, Seung Ki; Lee, Dong Soo; Chung, June Key; Lee, Myung Chul

    2007-01-01

    To assess the effect of encephaloduroarteriosynangiosis (EDAS) with or without bifrontal encephalogaleosynangiosis (EGS) in children with moyamoya disease, we evaluated cerebral hemodynamic changes using brain Tc-99m HMPAO SPECT. Total 34 pediatric patients (M: F=12:22, mean age;93 yrs) enrolled. Bypass surgery for both hemispheres (EDAS with EGS on one side, and EDAS on the other side) in 25 patients, unilateral EDAS with EGS in 7, and unilateral EDAS only in 2 were underwent. Perfusion SPECT before surgery, and 4 to 18 months after final surgery were done. The vascular territories for ICA, MCA and the brain regions for the frontal, parietal, temporal, and the occipital cortices were determined using standard ROls based on K-SPAM. Additionally, medial frontal cortex was selected to assess the effect of EGS. Basal/acetazolamide challenged cerebral blood flow (CBF), and cerebral vascular index (CVRI) were determined using normalized regional brain uptake to the cerebellum. 24 patients became symptom free, and 6 were improved but having some residual symptoms at the last follow up period. The other 3 were worsened after operation. Overall basal/acetazolamide stress CBF and CVRI for each brain region after surgery were increased, however, only the changes of CVRI were significant. Meanwhile, the improvement of CBF or CVRI in the brain regions ipsilateral to the hemisphere having EDAS with EGS was not significantly different when compared with those for the brain regions with EDAS only. Also, the hemodynamic improvement for the mesial frontal cortex in patients after EDAS with EGS was not significant, and showed no difference with those in patient with EDAS only. Quantitative perfusion SPECT demonstrated the hemodynamic improvement after EDAS with or without EGS in pediatric moyamoya disease. Cerebrovascular reserve showed meaningful improvement after surgery, implicating the effect of vascular anastomosis in ischemic areas

  11. Childhood moyamoya disease: hemodynamic MRI

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    Tzika, A.A. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Robertson, R.L. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Barnes, P.D. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Vajapeyam, S. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Burrows, P.E. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Treves, S.T. [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States); Scott, R.M. l [Department of Radiology, Children`s Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115 (United States)

    1997-09-01

    Background. Childhood moyamoya disease is a rare progressive cerebrovascular disease. Objective. To evaluate cerebral hemodynamics using dynamic Gd-DTPA-enhanced imaging in children with moyamoya disease. Materials and methods. Eight children (2-11 years of age) with the clinical and angiographic findings typical of moyamoya disease, before and/or after surgical intervention (pial synangiosis), underwent conventional MR imaging (MRI) and hemodynamic MR imaging (HMRI). HMRI used a spoiled gradient-echo with low flip angle (10 deg) and long TE (TR/TE = 24/15 ms) to minimize T 1 effects and emphasize T 2{sup *} weighting. Raw and calculated hemodynamic images were reviewed. Three-dimensional time-of-flight MR angiography (MRA) and perfusion brain single photon emission computed tomography (SPECT) were also performed. Results. Abnormal hemodynamic maps resulting from vascular stenosis or occlusion and basal collaterals were observed in six patient studies. HMRI depicted perfusion dynamics of affected cerebrovascular territories, detected cortical perfusion deficits, and complemented conventional MRI and MRA. HMRI findings were consistent with those of catheter angiography and perfusion SPECT. Conclusion. Our preliminary experience suggests that HMRI may be of value in the preoperative and postoperative evaluation of surgical interventions in moyamoya disease. (orig.). With 4 figs., 3 tabs.

  12. Childhood moyamoya disease: hemodynamic MRI

    International Nuclear Information System (INIS)

    Tzika, A.A.; Robertson, R.L.; Barnes, P.D.; Vajapeyam, S.; Burrows, P.E.; Treves, S.T.; Scott, R.M. I

    1997-01-01

    Background. Childhood moyamoya disease is a rare progressive cerebrovascular disease. Objective. To evaluate cerebral hemodynamics using dynamic Gd-DTPA-enhanced imaging in children with moyamoya disease. Materials and methods. Eight children (2-11 years of age) with the clinical and angiographic findings typical of moyamoya disease, before and/or after surgical intervention (pial synangiosis), underwent conventional MR imaging (MRI) and hemodynamic MR imaging (HMRI). HMRI used a spoiled gradient-echo with low flip angle (10 deg) and long TE (TR/TE = 24/15 ms) to minimize T 1 effects and emphasize T 2 * weighting. Raw and calculated hemodynamic images were reviewed. Three-dimensional time-of-flight MR angiography (MRA) and perfusion brain single photon emission computed tomography (SPECT) were also performed. Results. Abnormal hemodynamic maps resulting from vascular stenosis or occlusion and basal collaterals were observed in six patient studies. HMRI depicted perfusion dynamics of affected cerebrovascular territories, detected cortical perfusion deficits, and complemented conventional MRI and MRA. HMRI findings were consistent with those of catheter angiography and perfusion SPECT. Conclusion. Our preliminary experience suggests that HMRI may be of value in the preoperative and postoperative evaluation of surgical interventions in moyamoya disease. (orig.). With 4 figs., 3 tabs

  13. Coexisting diseases of moyamoya vasculopathy.

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    Wei, Yi-Chia; Liu, Chi-Hung; Chang, Ting-Yu; Chin, Shy-Chyi; Chang, Chien-Hung; Huang, Kuo-Lun; Chang, Yeu-Jhy; Peng, Tsung-I; Lee, Tsong-Hai

    2014-07-01

    Several coexisting diseases have been reported in patients with moyamoya vasculopathy (MMV), but studies of quasi-moyamoya disease (quasi-MMD) are rare. This study aims to investigate the frequency of known coexisting diseases in patients with quasi-MMD and to compare quasi-MMD with moyamoya disease (MMD). Between 2000 and 2011, we retrospectively screened patients with International Classification of Diseases, Ninth Revision, code of 4375 (MMD) in the Health Information System of our hospital. The vascular images of each patient were confirmed by 2 neurologists and 1 neuroradiologist based on the diagnostic criteria of Japan Ministry of Health and Welfare. We excluded the patients with missing images and erroneous diagnosis. Demographics, coexisting diseases, laboratory data, treatment, and recurrent strokes were recorded. The eligible patients were divided into quasi-MMD and MMD groups according to the presence or absence of coexisting diseases. MMV was found in 90 patients including 37 (41.1%) quasi-MMD and 53 (58.9%) MMD. Atherosclerosis (32.4%) and thyroid disease (29.7%) were the leading coexisting diseases in quasi-MMD. Patients with MMD became symptomatic in a bimodal age distribution, whereas patients with quasi-MMD became symptomatic in a single-peak distribution. The prognosis of recurrent strokes was similar between quasi-MMD and MMD based on Kaplan-Meier analysis. A bimodal distribution of onset age was noted in MMD, whereas a single-peak distribution was found in quasi-MMD. Coexisting diseases were usually underevaluated but were more common than expected in patients with MMV. Atherosclerosis and thyroid diseases were the leading coexisting diseases in different preferential age. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  14. Moyamoya disease and syndromes: from genetics to clinical management

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    Guey S

    2015-02-01

    Full Text Available Stéphanie Guey,1,3 Elisabeth Tournier-Lasserve,1,2 Dominique Hervé,1,3 Manoelle Kossorotoff4 1Inserm UMR-S1161, Université Paris 7 Denis Diderot, Sorbonne Paris Cité, Paris, France; 2AP-HP, Groupe hospitalier Lariboisière-Saint-Louis, Service de génétique neurovasculaire, Paris, France; 3Service de Neurologie, Centre de Référence des maladies Vasculaires Rares du Cerveau et de l'Œil (CERVCO, Groupe Hospitalier Saint-Louis Lariboisière-Fernand Widal, Assistance Publique-Hôpitaux de Paris, Paris, France; 4Pediatric Neurology Department, French Center for Pediatric Stroke, University Hospital Necker-Enfants Malades, AP-HP Assistance publique-Hôpitaux de Paris, Paris, France Abstract: Moyamoya angiopathy is characterized by a progressive stenosis of the terminal portion of the internal carotid arteries and the development of a network of abnormal collateral vessels. This chronic cerebral angiopathy is observed in children and adults. It mainly leads to brain ischemic events in children, and to ischemic and hemorrhagic events in adults. This is a rare condition, with a marked prevalence gradient between Asian countries and Western countries. Two main nosological entities are identified. On the one hand, moyamoya disease corresponds to isolated moyamoya angiopathy, defined as being “idiopathic” according to the Guidelines of the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis. This entity is probably multifactorial and polygenic in most patients. On the other hand, moyamoya syndrome is a moyamoya angiopathy associated with an underlying condition and forms a very heterogeneous group with various clinical presentations, various modes of inheritance, and a variable penetrance of the cerebrovascular phenotype. Diagnostic and evaluation techniques rely on magnetic resonance imaging (MRI, magnetic resonance angiography (MRA conventional angiography, and cerebral hemodynamics measurements

  15. The role of VEGF and KDR polymorphisms in moyamoya disease and collateral revascularization.

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    Young Seok Park

    Full Text Available We conducted a case-control study to investigate whether vascular endothelial growth factor (VEGF -2578, -1154, -634, and 936 and kinase insert domain containing receptor (KDR -604, 1192, and 1719 polymorphisms are associated with moyamoya disease. Korean patients with moyamoya disease (n = 107, mean age, 20.9±15.9 years; 66.4% female and 243 healthy control subjects (mean age, 23.0±16.1 years; 56.8% female were included. The subjects were divided into pediatric and adult groups. Among the 64 surgical patients, we evaluated collateral vessel formation after 2 years and divided patients into good (collateral grade A or poor (collateral grade B and C groups. The frequencies and distributions of four VEGF (-2578, -1154, -634, and 936 and KDR (-604, 1192, and 1719 polymorphisms were assessed from patients with moyamoya disease and compared to the control group. No differences were observed in VEGF -2578, -1154, -634, and 936 or KDR -604, 1192, and 1719 polymorphisms between the control group and moyamoya disease group. However, we found the -634CC genotype occurred less frequently in the pediatric moyamoya group (p = 0.040 whereas the KDR -604C/1192A/1719T haplotype increased the risk of pediatric moyamoya (p = 0.024. Patients with the CC genotype of VEGF -634 had better collateral vessel formation after surgery. Our results suggest that the VEGF -634G allele is associated with pediatric moyamoya disease and poor collateral vessel formation.

  16. Angiographic profile in childhood moyamoya disease

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    Jayakumar, P.N.; Arya, B.Y.T.; Vasudev, M.K.

    1991-01-01

    The cerebral angiograms of 8 patients with childhood moyamoya disease showed that the common findings were stenosis/occlusion of the supraclinoid internal carotid artery and the proximal segments of the anterior and middle cerebral arteries and basal moyamoya. The volume of basal moyamoya and its collateral supply depended upon the stage of the disease. Leptomeningeal collaterals were frequent in the later stages. Stenotic lesions in the posterior circulation were seen in a majority (75%) of patients. A feature unique to the study was evidence of intracranial small-vessel disease and stenotic cervical internal carotid artery in half of the cases. The disease in the ethnic caucasian Indians seems largely similar to the classical disease frequently reported in the Japanese literature. (orig.)

  17. Evaluation of regional cerebral circulation and metabolism in moyamoya disease using positron emission computed tomography

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    Kuwabara, Yasuo

    1986-01-01

    Regional cerebral blood flow, oxygen extraction fraction, metabolic rate of oxygen, blood volume and transit time were evaluated in 11 patients with moyamoya disease and 3 with suspected moyamoya disease using positron emission computed tomography. Eight of them were examined before and after EC-IC bypass surgery. Moyamoya patients were classified into four groups, namely, pediatric bilateral chronic type (over 5 years from onset), pediatric bilateral early type (within 5 years from onset), pediatric unilateral early type and adult type, according to age, duration of disease from onset and angiographic findings. These four groups showed different patterns on PET images; diffusely decreased CBF and CMRO2 in pediatric bilateral chronic type, decreased CBF and increased OEF in the frontal or temporoparietal region in pediatric bilateral early type, diffusely decreased CBF and increased OEF in the unilateral cerebral hemisphere in pediatric unilateral cerebral hemisphere in pediatric unilateral early type, and decreased CBF and CMRO2 in adult type. An increase of rCBV was demonstrated in frontal regions or basal ganglia in all groups, more prominently in pediatric patients. This was thought to be a common finding in moyamoya disease, corresponding to moyamoya vessels. Staging of moyamoya disease by PET was presented and compared to the angiographic staging. They were significantly correlated, and the stage 3 on PET image with decreased CMRO2 corresponded to the stage 3 or 4 on angiography, the most active stage of moyamoya disease. PET revealed increased CBF in the cortical area around EC-IC bypass but no remarkable changes in mean values of rCBF, OEF, CMRO2 and CBV in cerebral hemisphere. Some patients showed decreased rCBV in the basal ganglia. (J.P.N.)

  18. T2 shortening in childhood moyamoya disease

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    Takanashi, J.; Sugita, K.; Tanabe, Y.; Ito, C.; Date, H.; Niimi, H.

    1996-01-01

    We examined T2 shortening in six children with infarcts due to moyamoya disease to clarify whether there are characteristic patterns of T2 shortening in the deep grey and white matter. Profound T2 shortening in the deep grey and white matter was observed in the acute stage of infarct in two cases, which changed to high intensity in the chronic stage; in this stage no T2 shortening was demonstrated in any case. Neither haemorrhagic infarction nor calcification was seen on CT or MRI. There could be longitudinally different T2 shortening patterns between infarcts due to moyamoya disease and other disorders. (orig.). With 2 figs., 1 tab

  19. Assessment of Cerebral Hemodynamic Changes in Pediatric Patients with Moyamoya Disease Using Probabilistic Maps on Analysis of Basal/Acetazolamide Stress Brain Perfusion SPECT

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    Lee, Ho Young; Lee, Jae Sung; Kim, Seung Ki; Wang, Kyu Chang; Cho, Byung Kyu; Chung, June Key; Lee, Myung Chul; Lee, Dong Soo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2008-06-15

    To evaluate the hemodynamic changes and the predictive factors of the clinical outcome in pediatric patients with moyamoya disease, we analyzed pre/post basal/acetazolamide stress brain perfusion SPECT with automated volume of interest (VOIs) method. Total fifty six (M:F=33:24, age 6.7{+-}3.2 years) pediatric patients with moyamoya disease, who underwent basal/acetazolamide stress brain perfusion SPECT within 6 before and after revascularization surgery (encephalo-duro-arterio-synangiosis (EDAS) with frontal encephalo-galeo-synangiosis (EGS) and EDAS only followed on contralateral hemisphere), and followed-up more than 6 months after post-operative SPECT, were included. A mean follow-up period after post-operative SPECT was 33{+-}21 months. Each patient's SPECT image was spatially normalized to Korean template with the SPM2. For the regional count normalization, the count of pons was used as a reference region. The basal/acetazolamide-stressed cerebral blood flow (CBF), the cerebral vascular reserve index (CVRI), and the extent of area with significantly decreased basal/acetazolamide- stressed rCBF than age-matched normal control were evaluated on both medial frontal, frontal, parietal, occipital lobes, and whole brain in each patient's images. The post-operative clinical outcome was assigned as good, poor according to the presence of transient ischemic attacks and/or fixed neurological deficits by pediatric neurosurgeon. In a paired t-test, basal/acetazolamide-stressed rCBF and the CVRI were significantly improved after revascularization (p<0.05). The significant difference in the pre-operative basal/acetazolamide-stressed rCBF and the CVRI between the hemispheres where EDAS with frontal EGS was performed and their contralateral counterparts where EDAS only was done disappeared after operation (p<0.05). In an independent student t-test, the pre-operative basal rCBF in the medial frontal gyrus, the post-operative CVRI in the frontal lobe and the parietal

  20. Moyamoya disease associated with antiphospholipid syndrome

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    Mahmut Abuhandan

    2011-12-01

    Full Text Available Moyamoya (MMD is a disease that often involves the vascular structures of anterior cerebral circulation, particularly the proximal segments of anterior and middle cerebral arteries. The etiology of the disease is unknown. MMD often presents with cerebral ischemia and rarely with cerebral hemorrhage. The pathology is termed Moyamoya syndrome (MMS when the pathological cerebral angiography findings are accompanied by meningitis, neurofibromatosis, neoplasm, Down syndrome or polycystic kidney disease. Autoimmune diseases including Graves’ disease, Behcet’s disease and antiphospholipid syndrome might also lead to the development of MMS. In this manuscript, we presented an interesting case of MMD associated with antiphospholipid syndrome, which is quite a rare cause of acute cerebral infarction in childhood

  1. Dental Management of a Pediatric Patient with Moyamoya Syndrome: A Rare Clinical Entity.

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    Ko, Brittany L; Unkel, John H

    2018-01-01

    Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive constriction of the internal carotid artery and its branches. The disease has a particularly aggressive course in very young patients, and early surgical intervention is often necessary to prevent permanent neurological damage. MMD patients have an increased risk of stroke development, which may be provoked by pain or anxiety. Currently, no reports of pediatric patients with MMD exist in the dental literature. The purpose of this paper was to discuss the dental management of a two-year-old with moyamoya disease who presented with early childhood caries and dental fear, offering recommendations for dental providers with emphasis on stroke prevention, collaboration with the medical team, anesthesia considerations for patients with increased stroke risk, and the challenges to maintain the oral health of a patient undergoing complex medical treatment.

  2. Psychomotor delay, a possible rare presentation of moyamoya disease

    International Nuclear Information System (INIS)

    Ashrafi, M. R.; Alizadeh, H.; Yazdani, Sh.; Mohseni, M.; Mohamadi, M.

    2011-01-01

    Moyamoya disease is a rare, chronic cerebrovascular occlusive disease of unknown etiology. It is characterized by progressive stenosis of the arteries of the circle of Willis leading to ischemic strokes in young people and cerebral hemorrhage, which is more frequent in adults. Secondarily, an abnormal network of fine collateral vessels arises at the base of the brain. The term moyamoya refers to the angiographic appearance of the cerebral vasculature. We present such a disease in an 18-month-old Iranian girl with global developmental delay, which is a very rare presentation of moyamoya disease. She was diagnosed by magnetic resonance imaging and magnetic resonance angiography.

  3. Medical image of the week: moyamoya disease

    Directory of Open Access Journals (Sweden)

    Pak S

    2017-11-01

    Full Text Available No abstract available. Article truncated at 150 words. A 52-year-old, right-handed, Caucasian woman with a history of hypertension and morbid obesity presented with acute onset of word-finding difficulty and slurred speech. Her medical and family history was negative for cerebral vascular event, coronary artery disease or smoking. Computed tomography of the patient's brain showed narrow caliber middle cerebral artery vasculature bilaterally. This abnormal finding prompted further investigation with cerebral angiogram. The angiogram showed bilateral high-grade stenosis of the anterior and middle cerebral arteries, worse on the left (Figure 1. Magnetic resonance imaging revealed multiple left sided punctate infarcts in the frontal and parietal lobes (Figure 2. Diagnosis of ischemic stroke secondary to moyamoya disease was established. This patient was not a candidate for fibrinolytic therapy since it had been more than 4 hours from initial presentation. She was treated with aspirin, clopidogrel, and atorvastatin for secondary prevention of ischemic stroke. Two months after her discharge date, the patient …

  4. Presentation of moyamoya disease with occipital hemorrhage: a case report

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    Serkan Demir

    2012-12-01

    Full Text Available Moyamoya disease is a cerebrovascular disease which is characterized with stenosis and occlusions at the distal part of internal carotid artery and at the proximal part of anterior and middle cerebral arteries. It rarely causes temporary or recurrent hemiparesis due to intracranial hemorrhage while symptoms like headache, convulsion, nystagmus, aphasia and ataxia may also occur. In this paper, we present a case of Moyamoya disease which was diagnosed with a 23 year old female patient who was admitted to our emergency department with headache, nausea and vomiting complaints and whose radiological findings showed occipital lobe hemorrhage.

  5. Adult Moyamoya disease angiographic images evolutive characters and treatment methods

    International Nuclear Information System (INIS)

    Qian Jiangnan; Ling Feng

    2000-01-01

    Objective: To discuss the angiographic images with evolutional characters and the treatment methods of the Moyamoya disease. Methods: The clinical manifestations, the radiographic changes and the comparative analysis between medicine treatment and surgery treatment, together with the laboratory tests findings were analyzed in one cases adult Moyamoya disease during six years. Conclusions: The angiographic characteristics of MMD show the supplied artery trunk stenosis, and followed by occlusion, with later appearance of vascular smoking sign. Medical treatment proved to be of null. Direct or indirect intra or extra cranial vascular anastomosis are effective for treatment

  6. Moyamoya Disease Clinical Course and Severity in Childhood

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    Ayse Kacar Bayram

    2016-01-01

    Full Text Available Aim: Moyamoya disease (MMD is a rare, progressive and oclusive cerebrovascular disorder, predominantly affecting the terminal segment of the internal carotid arteries (ICA and its main branches. The purpose of this study is to evaluate the clinical course and severity of MMD in pediatric patients. Material and Method: We examined 5 consecutive pediatric patients with MMD, focusing on clinical and radiological features, the therapy and outcome over the 58-month follow-up period. Results: The study population consisted of 3 boys and 2 girls. The mean age at diagnosis of patients was 7.2 ± 3.4 years (age range: 3-10 years. The mean duration of follow-up was 30.4 ± 17.4 months (follow-up interval: 12-58 months. Neurological findings at presentation included: motor deficit in 4 patients (80.0%, epileptic seizures in 2 patients (40.0%, movement disorders in 3 patients (60.0%, and headache in 1 patients (20.0%. There was areas of infarction on brain MRI in all patients. Angiographic findings included: internal carotid artery stenosis in all patients, anterior cerebral artery stenosis in 3 patients, middle cerebral artery stenosis in 3 patients, posterior cerebral artery stenosis in 2 patients, and vertebral artery stenosis in 1 patient. Enoxaparine therapy was started to all patients. Subdural hematoma developed in 1 patient during follow-up. Cerebral infarctions recurred despite medical treatment in 4 patients. Discussion: Although this disease is rare, it is an important cause of pediatric stroke. MMD shows different clinical course and disease severity in childhood. Early diagnosis and appropriate treatment are crucial.

  7. Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome

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    Shahvaiz Magsi

    2016-01-01

    Full Text Available Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS, in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA. Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease.

  8. Steal phenomenon through the anterior communicating artery in Moyamoya disease

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    Lim, Soo Mee [Ewha Womans University, Department of Radiology, Mok-dong Hospital, College of Medicine, Seoul (Korea); Chae, Eun Jin; Kim, Min Yeong; Kim, Sang Joon; Choi, Choong Gon; Pyun, Hae Wook; Suh, Dae Chul [University of Ulsan College of Medicine, Asan Medical Center, Department of Radiology and Research Institute of Radiology, Seoul (Korea); Kim, Jae Kyun [Seoul Veterans Hospital, Department of Radiology, Seoul (Korea); Ahn, Jae Sung; Ra, Young-Shin [University of Ulsan, Asan Medical Center, Department of Neurosurgery, College of Medicine, Seoul (Korea); Kim, Jong-Uk; Hahm, Kyung Don [University of Ulsan, Asan Medical Center, Department of Anesthesiology, College of Medicine, Seoul (Korea)

    2007-01-15

    Branch occlusion of the anterior cerebral artery (ACA) is regarded as a part of Moyamoya disease. The purpose of this study is to define the ACA steal phenomenon (SP) in Moyamoya disease and to evaluate temporal changes according to the disease progression. From 139 Moyamoya patients we defined ACASP as narrowing of the ipsilateral A1-2 junction while preserving the anterior communicating artery and supplying the contralateral ACA cortical branches with the development of leptomeningeal collaterals by the ipsilateral middle cerebral artery into the hypoperfused ipsilateral ACA territory. Direction of the steal related to the stage in both hemispheres by Suzuki classification was statistically analyzed using the binomial test based on binomial distribution. Follow-ups of ACASP were evaluated in five patients. We identified ACASP in 13 (9%) patients (male:female=7:6, mean age 18 years, range: 2-58 years) of the 139 study patients. The presenting pattern was ischemic in 12 and hemorrhagic in one. The direction of SP occurred from the hemisphere in the lower to the higher stage of Suzuki classification (two-tail P value=0.0002). After revascularization surgery, ACASP disappeared or diminished. ACASP may occur in bilaterally different stages of Moyamoya disease as a transient self-adaptive process. It regresses after revascularization surgery. (orig.)

  9. Two adolescent patients with coexistent Graves' disease and Moyamoya disease in Korea.

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    Cheon, Chong Kun; Kim, Su Yung; Yoo, Jae-Ho

    2014-06-01

    Moyamoya disease is a cerebrovascular condition that results in the narrowing of the vessels of the circle of Willis and collateral vessel formation at the base of the brain. Although relationships between Graves' disease and cerebrovascular accidents in Moyamoya disease are obscure, the coexistence of the two diseases is noteworthy. Moyamoya disease has been rarely reported in adolescent patients with thyrotoxicosis. Recently, we encountered two adolescent Korean patients with Moyamoya disease associated with Graves' disease who presented with episodic right-sided hemiparesis and syncope. These two girls who had Graves' disease had no history of other diseases or head trauma. A thyroid function test revealed a euthyroid state and a high thyroid-stimulating hormone (TSH) receptor antibody titer at that time. The patients were diagnosed with Moyamoya disease based on brain magnetic resonance angiography and cerebral four-vessel angiography. The patients underwent cranial revascularization by encephalo-duroarterio-synangiosis as soon as a diagnosis was made, which resulted in successful symptom resolution. They fared well and had no additional neurological symptoms as of their last follow-up visits. Here, we report these two cases of confirmed Moyamoya disease complicated by Graves' disease with a review of the literature, and discuss the possible association between the two diseases. To our knowledge, this is the first report in South Korea on Moyamoya disease associated with Graves' disease in adolescents with a euthyroid.

  10. Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels

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    Sawada, Takeshi; Yamamoto, Akira; Okada, Tomohisa; Kanagaki, Mitsunori; Kasahara, Seiko; Togashi, Kaori [Kyoto University, Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto (Japan); Miki, Yukio [Osaka City University, Department of Radiology, Graduate School of Medicine, Osaka (Japan); Kikuta, Ken-ichiro [Fukui University, Division of Neurosurgery, Department of Sensory and Locomotor Medicine, Faculty of Medical Sciences, Fukui (Japan); Miyamoto, Susumu; Takahashi, Jun C. [Kyoto University, Department of Neurosurgery, Graduate School of Medicine, Kyoto (Japan); Fukuyama, Hidenao [Kyoto University, Human Brain Research Center, Graduate School of Medicine, Kyoto (Japan)

    2012-10-15

    The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. Participants comprised 20 consecutive patients with MMD (4 males, 16 females) diagnosed clinically using conventional angiography and 20 controls (13 male and 7 female arteriosclerosis patients). In these participants, 3-T MRI/MRA was evaluated by the existing MR criteria, which use MMVs in the basal ganglia, and the proposed MR criteria, which use cisternal MMVs, and then these two criteria were statistically compared by McNemar's test. Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD. (orig.)

  11. Diagnosis of moyamoya disease using 3-T MRI and MRA: value of cisternal moyamoya vessels

    International Nuclear Information System (INIS)

    Sawada, Takeshi; Yamamoto, Akira; Okada, Tomohisa; Kanagaki, Mitsunori; Kasahara, Seiko; Togashi, Kaori; Miki, Yukio; Kikuta, Ken-ichiro; Miyamoto, Susumu; Takahashi, Jun C.; Fukuyama, Hidenao

    2012-01-01

    The purpose of this study was to propose new magnetic resonance (MR) criteria of diagnosing moyamoya disease (MMD) from cisternal moyamoya vessels (MMVs) on 3-T magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and compare the diagnostic accuracy of the existing MR criteria and the proposed MR criteria. Participants comprised 20 consecutive patients with MMD (4 males, 16 females) diagnosed clinically using conventional angiography and 20 controls (13 male and 7 female arteriosclerosis patients). In these participants, 3-T MRI/MRA was evaluated by the existing MR criteria, which use MMVs in the basal ganglia, and the proposed MR criteria, which use cisternal MMVs, and then these two criteria were statistically compared by McNemar's test. Diagnostic accuracy was 62.5% with the existing MR criteria and 97.5% with the proposed MR criteria. The proposed MR criteria was more sensitive (1.00) than the existing MR criteria (0.45), but less specific (0.95) than the existing MR criteria (1.00). The proposed MR criteria using cisternal MMVs showed significantly higher diagnostic accuracy than the existing MR criteria. We believe that our proposed MR criteria will be beneficial for diagnosing MMD. (orig.)

  12. Clinical significance of posterior cerebral artery stenosis/occlusion in moyamoya disease

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    Kuroda, Satoshi; Ishikawa, Tatsuya; Iwasaki, Yoshinobu [Hokkaido Univ., Sapporo (Japan). Graduate School of Medicine; Houkin, Kiyohiro [Sapporo Medical Univ. (Japan)

    2002-12-01

    The present study was aimed at clarifying the clinical significance of posterior cerebral artery (PCA) stenosis/occlusion in pediatric and adult moyamoya disease. This study included a total of 132 patients (52 children and 80 adults) who were diagnosed as by cerebral angiography having moyamoya disease. CT or MRI was performed to examine the location of cerebral infarction in all subjects. Cerebral blood flow and vasoreactivity to acetazolamide were measured in 80 patients before surgery, using single photon emission computed tomography (SPECT). Three-dimensional MR angiography (3D-MRA) was repeated in 32 pediatric patients after surgery in order to clarify the natural course of the PCA stenosis/occlusion. Of 264 sides in 132 patients, PCA stenosis/occlusion was observed in 50 sides of 40 patients (30.3%). Its incidence was significantly higher in ischemic-type patients than in hemorrhagic-type and asymptomatic patients, and was higher in patients in the advanced stage of the disease. The hemisphere ipsilateral to PCA stenosis/occlusion had higher incidence of ischemic symptoms, cerebral infarction, and impaired cerebral hemodynamics. Transient ischemic attack (TIA) (hemianopsia) or cerebral infarction in the occipital lobe was noted in 4 (10%) of 40 patients during follow-up periods after bypass surgery for anterior circulation. Of 32 pediatric patients, none showed progression of PCA stenosis on 3D-MRA during follow-up periods. The present study showed that the involvement of PCA could increase the risk of TIA and/or cerebral infarction in both anterior and posterior circulation areas, suggesting that the PCA plays an important collateral role in moyamoya disease. (author)

  13. Endovascular treatment of basilar tip aneurysms associated with moyamoya disease

    International Nuclear Information System (INIS)

    Arita, K.; Kurisu, K.; Ohba, S.; Shibukawa, M.; Kiura, H.; Sakamoto, S.; Uozumi, T.; Nakahara, T.

    2003-01-01

    We report the efficacy and safety of endovascular treatment of basilar tip aneurysms (BTA) in five patients with moyamoya disease. The patients underwent intra-aneurysmal embolisation with detachable platinum coils. Three BTA presented with subarachnoid haemorrhage (SAH); the other two were asymptomatic. In four cases, one embolisation procedure produced >95% angiographic obliteration of the aneurysm. In the other patient, 80-90% obliteration was achieved initially, but due to growth of the residual aneurysm, the procedure was repeated 7 months later. Two patients experienced transient oculomotor paresis as a procedure-related complication. Mean follow-up was 43.6±34.0 months (range 8-92 months). One patient died of putaminal haemorrhage unrelated to the aneurysm 15 months after embolisation. The other four had no subsequent SAH and survived without sequelae. Endovascular embolisation using detachable platinum coils proved to be a safe and efficient treatment modality for BTA associated with moyamoya disease. (orig.)

  14. Risk factors of moyamoya disease in Canada and the USA.

    Science.gov (United States)

    Peerless, S J

    1997-10-01

    Over the past 28 years, 39 patients with Moyamoya disease or syndrome defined as spontaneous occlusion of the circle of Willis with extensive basal collateral vessels have been treated by the author in Canada and the USA. All patients presented with clinical or radiologic evidence of hemorrhage (23) or ischemia and infarction (16). A total of 12 patients had associated cerebral aneurysms and seven of these patients with aneurysms presented with subarachnoid hemorrhage. The patients ages ranged from 5 to 47 years. Of these 58% were female. The patients racial origin included North American Indian, Innuit, East Indian/Pakistani, Japanese, Chinese, Filipino, Korean, Malayasian, Hispanic, African American and Caucasian. Familial clustering was seen in North American Indian, Innuit and Caucasian patients. Associated disorders (tuberculosis, pharyngitis, thalassemia, fibromuscular hyperplasia, polycystic kidney, sickle cell trait and hypertension) were common in these patients, as was the use of tobacco, alcohol and in the adult females, oral contraceptives. It may be concluded from this series that the etiology of Moyamoya disease or syndrome is probably multifactorial, but that some racial and familial groups are more susceptible. Furthermore, in that the clinical and angiographic features are identical, the separation between Moyamoya disease and syndrome may not be helpful in understanding the etiology and pathophysiology of this disorder.

  15. Computed tomographic evaluation in 8 patient of cerebrovascular moyamoya disease

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    Ko, Young Tae; Lee, Jung Suk; Suh, Soo Jhi; Kim, Soon Yong [School of Medicine, Kyung-Hee University Hospital, Seoul (Korea, Republic of)

    1980-12-15

    CT findings in eight cerebrovascular moyamoya disease were compared with carotid angiographic findings and the results were as follows: 1. The patient's age was ranged from 2 to 49 years. Of eight patients, five were less than 17 years old and the rests were two 23 years and one 49 years of age. 2. Except one 6 years old boy, all of them were female patients. 3. In precontrast CT scan of 8 cases, 6 cases of cortical atrophy, 6 cases of ventricular dilatation and 5 cases of low density area were observed. In postcontrast study the children group shows abnormal contrast enhancement in 3 out of 5 patients but all adult reveal no contrast enhancement. 4. The isodense area in CT were thought to represent good collateral manifested by angiographic moyamoya vascular network and leptomeningeal anastomosis while the low density area in CT appears to poor colleaterals manifested by transdural anastomosis. 5. To evaluate the possibility of this disease, carotid angiography should be performed to the hemiplgic child who shows multiple low density area and abnormal enhancement in CT scan. 6. CT is not only reliable for evaluation of ventricular hemorrhage but also brain damage in patients with moyamoya disease.

  16. Moyamoya disease and its effect on cerebral perfusion

    International Nuclear Information System (INIS)

    Turner, James K.

    2009-01-01

    Full text: Objective: Moyamoya disease is a rare progressive occlusive disorder which affects the vasculature of the brain. It results in stroke like systems and can cause death due to haemorrhage. This poster follows the clinical case of a 12 year old South Korean Female diagnosed with Moyamoya disease in January 2004 and the role nuclear medicine played in her management. Methods: The clinical history of the patient was examined closely, with all previous medical imaging reports and nursing entries collated. An extensive literature review of Moyamoya disease was then performed using a range of resources. Results: The patient's clinical work-up involved a range of medical imaging procedures the results of which did not provide a clear pathway for clinical management. A Nuclear Medicine Cerebral Perfusion Diamox Challenge study was subsequently performed providing a critical result. The results of this study were indicative of no change in vascular reserve both pre and post Diamox infusion. Ultimately the patient's physicians then delayed the need for anastmosis surgery, with alternate methods of treatment required. Conclusion: Overall this case highlights the need for functional imaging and the importance of drug therapies including Diamox in nuclear medicine procedures. This study ensured the patients quality of life was maintained and eliminated any risk involved in performing further surgery.

  17. Autocrine release of angiopoietin-2 mediates cerebrovascular disintegration in Moyamoya disease.

    Science.gov (United States)

    Blecharz, Kinga G; Frey, Dietmar; Schenkel, Tobias; Prinz, Vincent; Bedini, Gloria; Krug, Susanne M; Czabanka, Marcus; Wagner, Josephin; Fromm, Michael; Bersano, Anna; Vajkoczy, Peter

    2017-04-01

    Moyamoya disease is a rare steno-occlusive cerebrovascular disorder often resulting in hemorrhagic and ischemic strokes. Although sharing the same ischemic stimulus with atherosclerotic cerebrovascular disease, Moyamoya disease is characterized by a highly instable cerebrovascular system which is prone to rupture due to pathological neovascularization. To understand the molecular mechanisms underlying this instability, angiopoietin-2 gene expression was analyzed in middle cerebral artery lesions obtained from Moyamoya disease and atherosclerotic cerebrovascular disease patients. Angiopoietin-2 was significantly up-regulated in Moyamoya vessels, while serum concentrations of soluble angiopoietins were not changed. For further evaluations, cerebral endothelial cells incubated with serum from these patients in vitro were applied. In contrast to atherosclerotic cerebrovascular disease serum, Moyamoya disease serum induced an angiopoietin-2 overexpression and secretion, accompanied by loss of endothelial integrity. These effects were absent or inverse in endothelial cells of non-brain origin suggesting brain endothelium specificity. The destabilizing effects on brain endothelial cells to Moyamoya disease serum were partially suppressed by the inhibition of angiopoietin-2. Our findings define brain endothelial cells as the potential source of vessel-destabilizing factors inducing the high plasticity state and disintegration in Moyamoya disease in an autocrine manner. We also provide new insights into Moyamoya disease pathophysiology that may be helpful for preventive treatment strategies in future.

  18. Presumed Perinatal Stroke in a Child with Down Syndrome and Moyamoya Disease

    Science.gov (United States)

    Pysden, Karen; Fallon, Penny; Moorthy, Bhagavatheswaran; Ganesan, Vijeya

    2010-01-01

    Moyamoya disease describes a cerebral arteriopathy characterized by stenosis or occlusion of the terminal internal carotid and/or the proximal middle cerebral arteries. We report a female child with trisomy 21 and bilateral moyamoya disease who presented, unusually, with a presumed perinatal cerebral infarct. The clinical, radiological, and…

  19. Moyamoya disease in a child with previous acute necrotizing encephalopathy

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    Kim, Taik-Kun; Cha, Sang Hoon; Chung, Kyoo Byung; Kim, Jung Hyuck; Kim, Baek Hyun; Chung, Hwan Hoon [Department of Diagnostic Radiology, Korea University College of Medicine, Ansan Hospital, 516 Kojan-Dong, Ansan City, Kyungki-Do 425-020 (Korea); Eun, Baik-Lin [Department of Pediatrics, Korea University College of Medicine, Seoul (Korea)

    2003-09-01

    A previously healthy 24-day-old boy presented with a 2-day history of fever and had a convulsion on the day of admission. MRI showed abnormal signal in the thalami, caudate nuclei and central white matter. Acute necrotising encephalopathy was diagnosed, other causes having been excluded after biochemical and haematological analysis of blood, urine and CSF. He recovered, but with spastic quadriparesis. At the age of 28 months, he suffered sudden deterioration of consciousness and motor weakness of his right limbs. MRI was consistent with an acute cerebrovascular accident. Angiography showed bilateral middle cerebral artery stenosis or frank occlusion with numerous lenticulostriate collateral vessels consistent with moyamoya disease. (orig.)

  20. Varied presentations of moyamoya disease in a tertiary care hospital of north-east India

    Directory of Open Access Journals (Sweden)

    Papori Borah

    2014-01-01

    Full Text Available Introduction: Moyamoya disease is a chronic progressive cerebrovascular disorder, characterized by stenosis or occlusion of bilateral internal carotid arteries (ICAs, anterior cerebral arteries (ACAs and middle cerebral arteries (MCAs, accompanied by a collateral network of vessels formed at the base of the brain. Ischemia and intracranial hemorrhage are the common typical manifestations. However moyamoya disease has been associated with atypical presentations like headache, seizures and involuntary movements. Although frequently reported from Asian countries like Japan, China and Korea, only few studies reported on clinical manifestations of moyamoya disease from India. Objectives: To study the varied presentations of moyamoya disease in a tertiary care hospital of north-east India. Material and Methods: Relevant investigations were done to rule out other causes of moyamoya syndrome. Results: We report 6 cases of moyamoya disease with varied presentations from a tertiary care referral government hospital. Case 1, 2 and 6 presented with alternating hemiparesis. Case 3 had amaurosis fugax. Case 4 had history suggestive of ischemic stroke and presented with hemichorea. Case 4 had focal seizure as the only manifestation. Cases 4 and 5 notably had stenosis of posterior cerebral artery (PCA in addition to stenosis of bilateral ICAs, ACAs and MCAs. Conclusion: Owing to its low incidence in India, moyamoya disease is easily overlooked as a possible diagnosis. However, because of its progressive nature, it is imperative to diagnose this disease early and offer surgical treatment to the patients.

  1. Elevation of Proenkephalin 143-183 in Cerebrospinal Fluid in Moyamoya Disease.

    Science.gov (United States)

    Yokoyama, Kinya; Maruwaka, Mikio; Yoshikawa, Kazuhiro; Araki, Yoshio; Okamoto, Sho; Sumitomo, Masaki; Kawamura, Akino; Sakamoto, Yusuke; Shimizu, Kenzo; Izumi, Takashi; Wakabayashi, Toshihiko

    2018-01-01

    In moyamoya disease (MMD), the causes of differences in clinical features between children and adults and of the dramatic temporal changes in moyamoya vessels are poorly understood. We previously discovered elevated levels of m/z 4588 and m/z 4473 peptides in cerebrospinal fluid (CSF) in patients with MMD. This study examined the amino acid sequences of these peptides and quantified in specimens. The m/z 4588 and m/z 4473 peptides in CSF from patients with MMD were purified and concentrated by high-performance liquid chromatography and ultrafiltration. Liquid chromatography coupled with tandem mass spectrometry analysis was performed to identify the amino acid sequences of these peptides. We quantified these peptides in samples using sandwich enzyme-linked immunosorbent assay, and concentrations in CSF were compared between MMD (n = 40, 19 male; median age, 37 years) and non-MMD intracranial disease (n = 40, 19 male; median age, 39 years) as controls. These peptides were identified as proenkephalin 143-183 (PENK 143-183). The concentration of PENK 143-183 was significantly greater in patients with MMD (median, 8,270 pmol/L) than control patients (median, 3,760 pmol/L; P < 0.001) and decreased in an age-dependent manner in MMD (r = -0.57; P < 0.001). The area under the receiver operating characteristic curve in children (age <18 years) was 0.885 (95% confidence interval 0.741-1). The correlation between proenkephalin concentration and temporal changes in moyamoya vessels was suggested. Proenkephalin 143-183 in CSF may offer a helpful diagnostic biomarker in pediatric MMD. The effect of enkephalin peptides through opioid growth factor receptor or delta opioid receptor might be associated with the pathophysiology of MMD. Copyright © 2017 Elsevier Inc. All rights reserved.

  2. Indirect revascularization surgery for moyamoya disease in children and its special considerations

    Directory of Open Access Journals (Sweden)

    Kyu-Chang Wang

    2012-11-01

    Full Text Available Moyamoya disease (MMD is the most common pediatric cerebrovascular disease in Far Eastern countries. In children, MMD frequently manifests as ischemic symptomatology. Cerebral perfusion gradually decreases as the disease progresses, which often leads to cerebral infarction. The benefits of revascularization surgery, whether direct or indirect, have been well established in MMD patients with ischemic symptoms. In adults, the increase in cerebral blood flow achieved with indirect revascularization is often unsatisfactory, and direct revascularization is usually feasible. In children, however, direct revascularization is frequently technically not feasible, whereas the response to indirect revascularization is excellent, although 1 or 2 weeks are required for stabilization of symptoms. The authors describe surgical procedures and perioperative care in indirect revascularization for MMD. In addition, special considerations with regard to very young patients, patients with recent cerebral infarction, and patients with hyperthyroidism are discussed.

  3. [Moyamoya disease as a rare cause of ischaemic stroke--case report].

    Science.gov (United States)

    Kułakowska, Alina; Kapica-Topczewska, Katarzyna; Borowik, Helena; Drozdowski, Wiesław

    2009-10-01

    Moyamoya disease is a rare, progressive disease of the vessels diagnosed according to characteristic abnormalities of brain arteries in the angiography. The incidence of moyamoya disease in Europe is lower than in Asia and its clinical course in European population is probably different from Asiatic (older age of onset and rare incidence of hemorrhagic strokes). Two young patients were diagnosed as moyamoya disease on the basis of clinical symptoms (ischaemic stroke) and results of brain vessels' angiography, which documented an occlusion of both internal carotid arteries above branching-off the ocular arteries in the first patient and stenosis of distal internal carotid arteries and proximal medial and anterior cerebral arteries in the second one. Both patients are under control of the Neurological Outpatient Department and their neurological state is stable. Despite that moyamoya disease is a rare cause of ischaemic stroke, it should be always considered as one of etiologic factors, especially in young patients.

  4. Revascularization experience and results in ischaemic cerebrovascular disease: Moyamoya disease and carotid occlusion.

    Science.gov (United States)

    Arikan, Fuat; Rubiera, Marta; Serena, Joaquín; Rodríguez-Hernández, Ana; Gándara, Darío; Lorenzo-Bosquet, Carles; Tomasello, Alejandro; Chocrón, Ivette; Quintana-Corvalan, Maximiliano; Sahuquillo, Juan

    2018-03-14

    Cerebral revascularization techniques are an indispensable tool in the current armamentarium of vascular neurosurgeons. We present revascularization surgery experience and results in both moyamoya disease and occlusive cerebral ischaemia. Patients with ischaemic occlusive disease and moyamoya disease who underwent microsurgical revascularization between October 2014 and September 2017 were analysed. In the study period, 23 patients with occlusive ischaemic disease underwent microsurgical revascularization. Three patients presented with serious postoperative complications (2 intraparenchymal haemorrhages in the immediate postoperative period and one thrombosis of the femoral artery). All patients, except one, achieved normalization of the cerebral hemodynamic reserve (CHR) in the SPECT study. Twenty patients had a good neurological result, with no ischaemic recurrence of the revascularized territory. Among patients with moyamoya, 20 had moyamoya disease and 5 had moyamoya syndrome with unilateral involvement. Five patients were treated at paediatric age. Haemorrhagic onset occurred in 2 patients. The CHR study showed hemodynamic compromise in all patients. Cerebral SPECT at one year showed resolution of the hemodynamic failure in all patients. There have been 4 postoperative complications (acute subdural hematoma, two subdural collections and one dehiscence of the surgical wound). No patient presented with neurological worsening at 6 and 12months of follow-up. Cerebral revascularization through end-to-side anastomosis between the superficial temporal artery and a cortical branch of the middle cerebral artery is an indisputable technique in the treatment of moyamoya disease and possibly in a subgroup of patients with symptomatic occlusive ischaemic cerebrovascular disease. Copyright © 2018 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.

  5. Moyamoya disease associated with asymptomatic mosaic Turner syndrome: a rare cause of hemorrhagic stroke.

    Science.gov (United States)

    Manjila, Sunil; Miller, Benjamin R; Rao-Frisch, Anitha; Otvos, Balint; Mitchell, Anna; Bambakidis, Nicholas C; De Georgia, Michael A

    2014-01-01

    Moyamoya disease is a rare cerebrovascular anomaly involving the intracranial carotid arteries that can present clinically with either ischemic or hemorrhagic disease. Moyamoya syndrome, indistinguishable from moyamoya disease at presentation, is associated with multiple clinical conditions including neurofibromatosis type 1, autoimmune disease, prior radiation therapy, Down syndrome, and Turner syndrome. We present the first reported case of an adult patient with previously unrecognized mosaic Turner syndrome with acute subarachnoid and intracerebral hemorrhage as the initial manifestation of moyamoya syndrome. A 52-year-old woman was admitted with a subarachnoid hemorrhage with associated flame-shaped intracerebral hemorrhage in the left frontal lobe. Physical examination revealed short stature, pectus excavatum, small fingers, micrognathia, and mild facial dysmorphism. Cerebral angiography showed features consistent with bilateral moyamoya disease, aberrant intrathoracic vessels, and an unruptured 4-mm right superior hypophyseal aneurysm. Genetic analysis confirmed a diagnosis of mosaic Turner syndrome. Our case report is the first documented presentation of adult moyamoya syndrome with subarachnoid and intracerebral hemorrhage as the initial presentation of mosaic Turner syndrome. It illustrates the utility of genetic evaluation in patients with cerebrovascular disease and dysmorphism. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  6. Cerebral hemodynamics in patients with moyamoya disease, (2)

    International Nuclear Information System (INIS)

    Takeuchi, Shigekazu

    1983-01-01

    Regional cerebral blood flow (rCBF) was measured by the 133 Xe inhalation method in 19 patients with moyamoya disease aged 5 to 46 and compared with that in 17 healthy volunteers aged 7 to 67. In healthy volunteers, mean hemispheric flow values (mCBF) in the steady state decreased and cerebrovascular resistance (CVR) increased with advancing age. Most young patients showed low values of mCBF in both hemispheres in comparison with healthy volunteers. About half of the young patients showed higher values of CVR than young healthy volunteers. The distribution of rCBF showed a hyperfrontal pattern in healthy volunteers. However, in the patients, regional distribution of hemispheric flow showed a different pattern with low flow in the upper frontal region and mean flow in the posterotemporal and occipital regions. rCBF measurements were carried out during hyperventilation in five healthy volunteers and in one patient, and during 5% CO 2 inhalation in one healthy volunteer and two patients. CO 2 reactivity was uniformly present in the hemispheres of healthy volunteers. rCBF in both hemispheres was reduced by hyperventilation, more markedly in the patient than in healthy volunteers. On the other hand, in two patients, the flow was increased in the temporo-occipital regions and was decreased in the frontal region by 5% CO 2 inhalation, and mCBF was slightly increased. Postoperative rCBF measurements in 21 sides of 12 young patients indicated a gradual increase of mCBF in 14 sides of nine patients from 3 months after surgery. These results indicate that rCBF measurements by the 133 Xe inhalation method are useful in determining cerebral hemodynamics in patients with moyamoya disease, especially in children. (J.P.N.)

  7. Statistical image analysis of cerebral blood flow in moyamoya disease

    International Nuclear Information System (INIS)

    Yamada, Masaru; Yuzawa, Izumi; Suzuki, Sachio; Kurata, Akira; Fujii, Kiyotaka; Asano, Yuji

    2007-01-01

    The Summary of this study was to investigate pathophysiology of moyamoya disease, we analyzed brain single photon emission tomography (SPECT) images of patients with this disease by using interface software for a 3-dimensional (3D) data extraction format. Presenting symptoms were transient ischemic attack (TIA) in 21 patients and hemorrhage in 6 patients. All the patients underwent brain SPECT scan of 123 I-iofetamine (IMP) at rest and after acetazolamide challenge (17 mg/kg iv, 2-day method). Cerebral blood flow (CBF) was quantitatively measured using arterial blood sampling and an autoradiography model. The group of the patients who presented with TIAs showed decreased CBF in the frontal lobe at rest compared to that of patients with hemorrhage, but Z-score ((mean-patient data)/ standard deviation (SD)) did not reach statistical significance. Significant CBF decrease after acetazolamide challenge was observed in a wider cerebral cortical area in the TIA group than in the hemorrhagic group. The brain region of hemodynamic ischemia (stage II) correlated well with the responsible cortical area for clinical symptoms of TIA. A hemodynamic ischemia stage image clearly represented recovery of reserve capacity after bypass surgery. Statistical evaluation of SPECT may be useful to understand and clarify the pathophysiology of this disease. (author)

  8. A study on measurement of the regional cerebral blood flow using autoradiographic method in moyamoya disease

    International Nuclear Information System (INIS)

    Sasaki, Tomohiro; Kiya, Katsuzo; Yuki, Kiyoshi; Kawamoto, Hitoshi; Mizoue, Tatsuya; Kiura, Yoshihiro; Uozumi, Tohru; Ikawa, Fusao

    1997-01-01

    Development of Autoradiographic method (ARG) has provided measurement of cerebral blood flow in moyamoya disease. We evaluate a cerebral vasodilatory capacity (CVC) for moyamoya disease using ARG method. We used 5 patients with moyamoya disease as a candidate for measurement of the cerebral blood flow (CBF) who admitted to Hiroshima Prefectural Hospital during the past one year. There were 3 patients in an adult age and 2 patients in a young age. We tried to measure the regional CBF (rCBF) using ARG method which was a easy way to estimate the rCBF on SPECT. The CVC was calculated from the difference of the rCBF between resting SPECT and Diamox-loading SPECT. Results were as follows; Reactivity of cerebral vessels to CO 2 loading and CVC weakened in moyamoya disease. The rCBF and CVC in the territories of anterior and middle cerebral arteries reduced in comparison with those in the area supplied by the posterior cerebral artery. The CVC at the treated side with surgical reconstruction recovered somewhat in an adult type. From these results, measurement of CBF using ARG method seems to be useful for evaluation of the CVC in moyamoya disease. (author)

  9. Cerebral hemodynamics in adult ischemic-type patients with moyamoya disease compared with those of atherothrombotic middle cerebral artery occlusion

    International Nuclear Information System (INIS)

    Idei, Masaru; Yamane, Kanji; Nishida, Masahiro; Manabe, Kazufumi; Yokota, Akira

    2005-01-01

    We measured regional cerebral blood flow (rCBF) in adult ischemic-type patients with moyamoya disease and in patients with atherothrombotic middle cerebral artery occlusion (MCAO) to investigate cerebral hemodynamics in adult ischemic-type of moyamoya disease. In this study we measured rCBF and regional cerebro-vascular response (rCVR) using acetazolamide by Xe-non-enhanced CT. Our subjects consisted of 15 adult ischemic-type patients with moyamoya disease and 27 atherothrombotic stroke patients with proximal occlusion of the middle cerebral artery. The region of inter est was conducted in the anterior cerebral artery, middle cerebral artery and posterior cerebral artery territories as well as basal ganglia regions. rGBF was preserved in all regions of patients with moyamoya disease. However, rCVR severely decreased in the anterior circulation territory in patients with moyamoya disease compared with those of MCAO. These results suggest that rCBF in the anterior circulation territory of adult ischemic-type patients with moyamoya disease is preserved by vasodilation of the cerebral arteries, while cerebral hemodynamic reserve capacity is severely reduced. The results indicated that basal moyamoya vessels are dilated. These findings may be one of the reasons why stroke occurs more frequently in adult than child patients with moyamoya disease. (author)

  10. Continent Idiopathic Vesicovaginal Fistula Coexisting with Moyamoya Disease

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    İbrahim Güven Kartal

    2017-09-01

    Full Text Available Vesicovaginal fistula (VVF is the abnormal anatomic communication between the female bladder and vagina. Usually presents itself as continuous urinary incontinence. There are many etiologic risk factors such as obstetric trauma, pelvic surgery, infections, congenital abnormalities, foreign materials, malignancy and pelvic radiation (1. Due to high education and sociocultural levels, the incidence seems to be low in the developed countries. On the other hand, in the developing countries, there is an obviously high incidence and prevalence. Considering the low level of medical informative feed-back mechanisms and poor obstetrics health care services, it seems very hard to keep VVF patient data that can be used for epidemiological research studies. In those areas, due to high maternal mortality and obstetric complications, the incidence of VVF is significantly increased (2. In this paper, we report a female patient with known moyamoya disease and a stable right ovarian cyst who was regularly followed up by the departments of neurology, gynecology and presented with radiological and cystoscopy findings of incidentally diagnosed asymptomatic continent VVF.

  11. Moyamoya disease and sagittal sinus thrombosis in a child with Down's syndrome

    International Nuclear Information System (INIS)

    Del-Rio Camacho, G.; Leal Orozco, A.; Camino Lopez, M.; Ruiz-Moreno, M.; Perez-Higueras, A.; Al-Assir, I.

    2001-01-01

    A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy. (orig.)

  12. Moyamoya disease and artery tortuosity as rare phenotypes in a patient with an elastin mutation.

    Science.gov (United States)

    Ishiwata, Tsukasa; Tanabe, Nobuhiro; Shigeta, Ayako; Yokota, Hajime; Tsushima, Kenji; Terada, Jiro; Sakao, Seiichiro; Morisaki, Hiroko; Morisaki, Takayuki; Tatsumi, Koichiro

    2016-07-01

    Sporadic and familial elastin mutations can occur in large vessel stenosis such as supravalvular aortic stenosis and narrowing of the descending aorta. However, there are very few reports regarding the arteriopathy of cerebral, pulmonary or abdominal arteries in elastin mutations. We herein report the case of a Japanese female patient presenting with multiple arteriopathy including moyamoya disease, a tortuosity of abdominal arteries and pulmonary hypertension due to peripheral pulmonary artery stenosis. This case suggests the possible progression of cerebral arteriopathy including moyamoya disease in patients with elastin mutations. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  13. Moyamoya disease in two patients with Noonan-like syndrome with loose anagen hair.

    Science.gov (United States)

    Lo, Fu-Sung; Wang, Chao-Jan; Wong, Mun-Ching; Lee, Ni-Chung

    2015-06-01

    Moyamoya disease is a unique chronic cerebrovascular condition caused by progressive stenosis of the arteries around the circle of Willis with prominent arterial collateral circulation. Noonan-like syndrome with loose anagen hair (NSLH) is characterized by short stature, characteristic facial phenotype, darkly pigmented and hairless skin, mild psychomotor delay with attention deficit disorder, and easily pluckable, sparse, thin, slow growing hair. Mutations in SHOC2 have been reported to underlie NSLH. In this paper, we describe two individuals with NSLH who also have moyamoya disease and in whom heterozygous germline mutation in SHOC2 was found. © 2015 Wiley Periodicals, Inc.

  14. Thyroid Autoantibodies and the Clinical Presentation of Moyamoya Disease: A Prospective Study.

    Science.gov (United States)

    Lanterna, Luigi A; Galliani, Silvia; Zangari, Rosalia; Conti, Luciano; Brembilla, Carlo; Gritti, Paolo; Colleoni, Maria Luisa; Bernucci, Claudio

    2018-05-01

    Moyamoya is a rare cerebrovascular disease characterized by the progressive occlusion of the intracranial carotid artery. Thyroid autoantibodies have been found to be associated with the disease, but their clinical significance has never been studied. The objective of this study was to investigate the relationship between thyroid autoantibodies and the clinical presentation of moyamoya. This is a prospective study including 37 patients with moyamoya disease (MMD) or unilateral moyamoya (uMM). Thyroid function and thyroid autoantibodies (e.g., antithyroperoxidase and antithyroglobulin) were investigated. We studied the effect of gender, age, type of moyamoya (uMM versus MMD), and thyroid autoantibodies on the clinical presentation, dichotomized into aggressive (hemorrhage, major stroke, or frequent transient ischemic attack [TIA]) and nonaggressive presentation (headache, rare TIAs, and incidental diagnosis) according to the criteria of the Research Committee on Spontaneous Occlusion of the Circle of Willis. Of the 37 patients included in the study, the autoantibodies were elevated in 9 (24.3%). An aggressive presentation occurred in 21 patients (hemorrhage in 11, major stroke in 9, frequent TIAs in 1). The autoantibodies were elevated in 8 of the 21 patients (38.09%) with an aggressive presentation and in 1 of those presenting with minor symptoms (6.2%). The presence of elevated autoantibodies was the only variable associated with an aggressive presentation in the multivariate logistic analysis (P = .048). When the serum concentration of the thyroid autoantibodies is increased, the patients have a higher risk of an aggressive presentation. Our results support the hypothesis that activation of immune-mediated processes affects the moyamoya physiopathology. Copyright © 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  15. Enfermedad de moyamoya, macrocefalia y déficit intelectual en un adolescente Moyamoya disease, macrocephaly and intellectual impairment in an adolescent

    Directory of Open Access Journals (Sweden)

    José Vargas Díaz

    2013-03-01

    Full Text Available El patrón moyamoya se caracteriza por un estrechamiento crónico progresivo de la porción terminal de la arteria carótida interna o sus principales ramas terminales principalmente. Los niños con moyamoya típicamente se presentan con ictus arteriales isquémicos, o con accidentes isquémicos transitorios. Se presenta el caso clínico de un adolescente, con déficit intelectual y macrocránea, con evidencia en los estudios de resonancia magnética nuclear cerebral de infartos arteriales isquémicos a repetición, e imágenes sugestivas de moyamoya, y se comprueba el patrón moyamoya por angiografía de sustracción digital. Comprobado el diagnóstico de enfermedad de moyamoya, el objetivo con este enfermo fue tratar de mejorar la irrigación cerebral, planteando la necesidad de someter al paciente a cirugía de revascularización, y, mientras esto no fuera posible, usar aspirina como antiagregante plaquetario, en un intento de impedir o disminuir el riesgo de nuevos infartos cerebrales o de accidentes transitorios isquémicos a repetición.The moyamoya pattern is characterized by chronic progressive narrowing of the terminal portion of the internal carotid artery or of its main terminal branches. Children suffering moyamoya disease regularly present with ischemic arterial ictus or transient ischemic strokes. Here is the case of an adolescent with intellectual impairment and macrocrania. The magnetic resonance imaging study of his brain showed repeated ischemic arterial infarctions and images suggestive of moyamoya disease, the pattern of which was later confirmed by digital subtraction angiography. The first objective was to intend to improve his cerebral irrigation by performing a revascularization surgery, but as long as this action was not accomplished, then taking aspirin as anti-platelet aggregation drug could prevent or reduce the risk of new cerebral infarctions or of repeated transient ischemic strokes.

  16. Direct versus indirect revascularization procedures for moyamoya disease: a comparative effectiveness study.

    Science.gov (United States)

    Macyszyn, Luke; Attiah, Mark; Ma, Tracy S; Ali, Zarina; Faught, Ryan; Hossain, Alisha; Man, Karen; Patel, Hiren; Sobota, Rosanna; Zager, Eric L; Stein, Sherman C

    2017-05-01

    OBJECTIVE Moyamoya disease (MMD) is a chronic cerebrovascular disease that can lead to devastating neurological outcomes. Surgical intervention is the definitive treatment, with direct, indirect, and combined revascularization procedures currently employed by surgeons. The optimal surgical approach, however, remains unclear. In this decision analysis, the authors compared the effectiveness of revascularization procedures in both adult and pediatric patients with MMD. METHODS A comprehensive literature search was performed for studies of MMD. Using complication and success rates from the literature, the authors constructed a decision analysis model for treatment using a direct and indirect revascularization technique. Utility values for the various outcomes and complications were extracted from the literature examining preferences in similar clinical conditions. Sensitivity analysis was performed. RESULTS A structured literature search yielded 33 studies involving 4197 cases. Cases were divided into adult and pediatric populations. These were further subdivided into 3 different treatment groups: indirect, direct, and combined revascularization procedures. In the pediatric population at 5- and 10-year follow-up, there was no significant difference between indirect and combination procedures, but both were superior to direct revascularization. In adults at 4-year follow-up, indirect was superior to direct revascularization. CONCLUSIONS In the absence of factors that dictate a specific approach, the present decision analysis suggests that direct revascularization procedures are inferior in terms of quality-adjusted life years in both adults at 4 years and children at 5 and 10 years postoperatively, respectively. These findings were statistically significant (p indirect and combination procedures may offer optimal results at long-term follow-up.

  17. Edaravone Reduces Hyperperfusion-Related Neurological Deficits in Adult Moyamoya Disease: Historical Control Study.

    Science.gov (United States)

    Uchino, Haruto; Nakayama, Naoki; Kazumata, Ken; Kuroda, Satoshi; Houkin, Kiyohiro

    2016-07-01

    Postoperative hyperperfusion-related transient neurological deficits (TNDs) are frequently observed in adult patients with moyamoya disease who undergo direct bypass procedures. The present study evaluated the effect of the free radical scavenger edaravone on postoperative hyperperfusion in adult moyamoya disease. This study included 92 hemispheres in 72 adult patients who underwent direct bypass for moyamoya disease. Serial measurements of cerebral blood flow were conducted immediately after surgery and on postoperative days 2 and 7. In 40 hemispheres for 36 patients, edaravone (60 mg/d) was administered from the day of surgery to postsurgical day 7. The incidence of postoperative hyperperfusion and associated TNDs were compared with a control group that included 52 hemispheres in 36 patients. Radiological hyperperfusion was observed in 28 of 40 (70.0%) and 39 of 52 (75.0%) hemispheres in the edaravone and control groups, respectively (P=0.30). Hyperperfusion-related TND incidences were significantly lower in the edaravone group compared with the control group (12.5% versus 32.7%; P=0.024). Multivariate analysis demonstrated that edaravone administration (P=0.009) and left-sided surgery (P=0.037) were significantly correlated with hyperperfusion-related TNDs (odds ratios, 0.3 and 4.2, respectively). Perioperative administration of edaravone reduced the incidence of hyperperfusion-related TNDs after direct bypass procedures in adult patients with moyamoya disease. © 2016 American Heart Association, Inc.

  18. Anestesia em paciente portadora de doença de moyamoya: relato de caso Anestesia en paciente portadora de enfermedad de moyamoya: relato de caso Anesthesia in patient with moyamoya disease: case report

    Directory of Open Access Journals (Sweden)

    Adriano Bechara de Souza Hobaika

    2005-06-01

    , la paciente fue mantenida en normocapnia y normotermia. La extubación fue realizada y la paciente transferida a la sala de recuperación pos-anestésica sin complicaciones. CONCLUSIONES: Este artículo presenta los cuidados anestésicos dados a una paciente portadora de la enfermedad de moyamoya.BACKGROUND AND OBJECTIVES: Moyamoya disease is an uncommon progressive cerebral vasculopathy, more frequently diagnosed among Asian individuals, but which has also been described in Brazil. Moyamoya patients may be submitted to different surgical procedures throughout their lives. Anesthesiologists must understand the pathophysiology of the disease and institute adequate perioperative measures to improve patients' prognosis. CASE REPORT: Female patient, 22 yr-old, chronic renal failure, with moyamoya disease, scheduled for surgical arterial-venous fistula installation. Anesthesia was induced with fentanyl, propofol and atracurium and maintained with sevoflurane. Patient was maintained in normocapnia and normothermia throughout the procedure. Patient was extubated and transferred to the post-anesthetic care unit without complications. CONCLUSIONS: This article describes the anaesthetic care of a moyamoya disease patient.

  19. Focal time-to-peak changes on perfusion MRI in children with Moyamoya disease: correlation with conventional angiography

    International Nuclear Information System (INIS)

    Choi, Hyun Seok; Kim, Dong-Seok; Shim, Kyu-Won; Kim, Jinna; Kim, Eun Soo; Lee, Seung-Koo

    2011-01-01

    Background: Moyamoya disease is a chronic progressive steno-occlusion of the distal internal carotid arteries with unknown etiology. As the classical presentation of childhood Moyamoya disease is ischemic stroke, cerebral hemodynamic evaluation is important for patient selection for surgery to prevent recurrent ischemic attacks. Perfusion MR imaging has been applied to evaluate cerebral hemodynamics. Purpose: To correlate the 'basal time-to-peak preservation sign', 'auto-synangiosis sign', and 'posterior involvement sign' on time-to-peak map of perfusion MRI with catheter angiography. Material and Methods: Thirty-four children (6.91 ± 3.08 years) with Moyamoya disease who underwent both perfusion-weighted MRI and catheter angiography were enrolled in this study. Given catheter angiography as a reference standard, basal time-to-peak preservation sign, auto-synangiosis sign, and posterior involvement sign were evaluated on time-to-peak maps. Results: The basal time-to-peak preservation sign was accurate for the diagnosis of childhood Moyamoya disease; both sensitivity and specificity were 100%. The auto-synangiosis sign showed lower sensitivity (65%), however, with an acceptable specificity (98%). The posterior involvement sign showed lower sensitivity (61%) but had an acceptable specificity (96%). Conclusion: The basal time-to-peak preservation sign may be a universal finding in childhood Moyamoya disease. The auto-synangiosis and posterior involvement sign may be useful in determining transdural collateral status and posterior circulation involvement in childhood Moyamoya disease

  20. Leptomeningeal high signal intensity (ivy sign) on fluid-attenuated inversion-recovery (FLAIR) MR images in moyamoya disease

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    Fujiwara, Hirokazu [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan)]. E-mail: hirokazu_fujiwara@ybb.ne.jp; Momoshima, Suketaka [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan); Kuribayashi, Sachio [Department of Diagnostic Radiology, School of Medicine, Keio University, Tokyo 1608582 (Japan)

    2005-08-01

    Purpose: There are a few reports on leptomeningeal high signal intensity (LMHI: ivy sign) on fluid-attenuated inversion-recovery (FLAIR) images in moyamoya disease, but the feature of this finding has not been completely understood. The purpose of this study was to characterize LMHI on FLAIR images in moyamoya disease and to assess usefulness of this finding in the diagnosis of moyamoya disease in conventional MR imaging. Material and methods: MR imaging of 28 patients with moyamoya disease was retrospectively reviewed. The grade of LMHI on FLAIR images was classified as 'absent,' 'minimal,' 'moderate' and 'marked.' Fifty-four hemispheres of 28 patients (2 patients had unilateral disease) were assessed for the frequency of visualization and distribution of LMHI. The correlations between LMHI on FLAIR images, moyamoya vessels on T1- and T2-weighted images and MR angiography findings were also analyzed. Results: Moderate and marked LMHI was seen in 31 out of 54 hemispheres (57%). LMHI was seen more prominently in the frontal and parietal lobes than in the temporal and occipital lobes. Although there was a tendency for LMHI on FLAIR images to be prominent in groups with moderate and marked moyamoya vessels on T1- and T2-weighted images, there was no significant correlation. More prominent LMHI was observed in the hemispheres in which cortical branches of the middle cerebral arteries were poorly visualized on MR angiography. Conclusion: Leptomeningeal high signal intensity (ivy sign) on FLAIR images is predominantly seen in the frontal and parietal lobes. Because this sign can be seen in patients with unremarkable moyamoya vessels, LMHI is a useful sign in conventional MR imaging for the diagnosis of moyamoya disease.

  1. Multiple intracranial aneurysms and moyamoya disease associated with microcephalic osteodysplastic primordial dwarfism type II: surgical considerations.

    Science.gov (United States)

    Waldron, James S; Hetts, Steven W; Armstrong-Wells, Jennifer; Dowd, Christopher F; Fullerton, Heather J; Gupta, Nalin; Lawton, Michael T

    2009-11-01

    Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is a rare genetic syndrome characterized by extremely small stature and microcephaly, and is associated in 25% of patients with intracranial aneurysms and moyamoya disease. Although aneurysmal subarachnoid hemorrhage and stroke are leading causes of morbidity and death in these patients, MOPD II is rarely examined in the neurosurgical literature. The authors report their experience with 3 patients who presented with MOPD II, which includes a patient with 8 aneurysms (the most aneurysms reported in the literature), and the first report of a patient with both moyamoya disease and multiple aneurysms. The poor natural history of these lesions indicates aggressive microsurgical and/or endovascular therapy. Microsurgery, whether for aneurysm clip placement or extracranial-intracranial bypass, is challenging due to tight surgical corridors and diminutive arteries in these patients, but is technically feasible and strongly indicated when multiple aneurysms must be treated or cerebral revascularization is needed.

  2. High rebleeding risk associated with choroidal collateral vessels in hemorrhagic moyamoya disease: analysis of a nonsurgical cohort in the Japan Adult Moyamoya Trial.

    Science.gov (United States)

    Funaki, Takeshi; Takahashi, Jun C; Houkin, Kiyohiro; Kuroda, Satoshi; Takeuchi, Shigekazu; Fujimura, Miki; Tomata, Yasutake; Miyamoto, Susumu

    2018-03-02

    OBJECTIVE Choroidal collateral vessels typical of moyamoya disease have received attention as a potential bleeding source. The authors' previous angiographic cross-sectional analysis suggested a possible association between choroidal collaterals and posterior hemorrhage, indicating a high risk for rebleeding. The present longitudinal analysis is intended to determine whether choroidal collaterals are a predictor of rebleeding in hemorrhagic moyamoya disease. METHODS The Japan Adult Moyamoya Trial group designed an ancillary cohort study using 5-year follow-up data on 37 patients included in the nonsurgical arm of the original randomized controlled trial and compared the rebleeding rate of those with and those without choroidal collaterals, represented by the connection between the anterior or posterior choroidal arteries and the medullary arteries. An expert panel determined whether a choroidal collateral was present in each patient through the measurement of baseline angiography studies. The rebleeding rate comparison was adjusted for age, diagnosis of hypertension, and involvement of the posterior cerebral artery. RESULTS Choroidal collaterals were present in 21 patients (56.8%). The rebleeding rate was 13.1% per year in the collateral-positive group as compared with 1.3% in the negative group (p = 0.008, log-rank test). The adjusted hazard ratio for rebleeding in the collateral-positive group relative to the negative group remained statistically significant (HR 11.10, 95% CI 1.37-89.91). Radiographic assessment of the collateral-positive group revealed good correspondence between the distribution of collaterals and rebleeding sites. CONCLUSIONS Results of this study suggest that choroidal collaterals are a bleeding source with a high risk for hemorrhagic recurrence and a predictor of rebleeding in hemorrhagic moyamoya disease.

  3. Surgical anatomy and preservation of the middle meningeal artery during bypass surgery for moyamoya disease.

    Science.gov (United States)

    Hori, Satoshi; Kashiwazaki, Daina; Akioka, Naoki; Hayashi, Tomohide; Hori, Emiko; Umemura, Kimiko; Horie, Yukio; Kuroda, Satoshi

    2015-01-01

    The middle meningeal artery (MMA) is known to function as one of the important collateral routes in moyamoya disease. However, the anterior branch frequently courses within the lesser wing of the sphenoid bone and can easily be damaged during craniotomy for bypass surgery. This prospective study aimed to study the surgical anatomy of the MMA and to establish the technique to preserve it during bypass surgery for moyamoya disease. Twenty-two patients with moyamoya disease underwent STA-MCA anastomosis combined with indirect bypass on 27 sides. The anatomical relationship between the anterior branch of the MMA and lesser wing was classified into three types: the bridge, monorail, and tunnel types. During surgery, the lesser wing was carefully resected with a rongeur or high-speed diamond drill to preserve the anterior branch of the MMA. The anterior branch of the MMA was classified into the bridge type in 5 sides (18.5 %), monorail type in 10 sides (37.0 %), and tunnel type in 12 sides (44.5 %). Patient age was closely related to the anatomical findings (χ (2) test, p = 0.0168). Careful resection of the lesser wing with a rongeur could preserve bridge- and monorail-type MMAs (100 and 71.4 %, respectively). However, drilling out of the lesser wing under a surgical microscope was essential to preserve the tunnel-type MMA. Intraoperative indocyanine green videoangiography was useful to confirm patency during surgery. It is essential to understand the surgical anatomy of the MMA around the pterion in order to preserve its anterior branch during bypass surgery for moyamoya disease.

  4. Localized 1H-MR spectroscopy in moyamoya disease before and after revascularization surgery

    International Nuclear Information System (INIS)

    Lim, Soo Mee; Choi, Hye Young; Suh, Jung Soo; Lee, Jung Hee; Lim, Keun Ho; Suh, Dae Chul; Lee, Ho Kyu; Lim, Tae Hwan; Ra, Young Shin

    2003-01-01

    To evaluate, using localized proton magnetic resonance spectroscopy (1H-MRS), the cerebral metabolic change apparent after revascularization surgery in patients with moyamoya disease. Sixteen children with moyamoya disease and eight age-matched normal controls underwent MR imaging, MR angiography, conventional angiography, and 99m Tc- ECD SPECT. Frontal white matter and the basal ganglia of both hemispheres were subjected to localized 1 H-MRS, and after revascularization surgery, four patients underwent follow-up 1 H-MRS. Decreased NAA/Cr ratios (1.35±0.14 in patients vs. 1.55±0.24 in controls) and Cho/Cr ratios (0.96±0.13 in patients vs. 1.10±0.11 in controls) were observed in frontal white matter. After revascularization surgery, NAA/Cr and Cho/Cr ratios in this region increased. In the basal ganglia, there is no abnormal metabolic ratios. Localized 1H-MRS revealed abnormal metabolic change in both hemispheres of children with moyamoya disease. Because of its non-invasive nature, 1 H-MRS is potentially useful for the preoperative evaluation of metabolic abnormalities and their postoperative monitoring

  5. Localized 1H-MR spectroscopy in moyamoya disease before and after revascularization surgery

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    Lim, Soo Mee; Choi, Hye Young; Suh, Jung Soo [Ewha Womans University Hospital, Seoul (Korea, Republic of); Lee, Jung Hee; Lim, Keun Ho; Suh, Dae Chul; Lee, Ho Kyu; Lim, Tae Hwan; Ra, Young Shin [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    2003-06-01

    To evaluate, using localized proton magnetic resonance spectroscopy (1H-MRS), the cerebral metabolic change apparent after revascularization surgery in patients with moyamoya disease. Sixteen children with moyamoya disease and eight age-matched normal controls underwent MR imaging, MR angiography, conventional angiography, and {sup 99m}Tc- ECD SPECT. Frontal white matter and the basal ganglia of both hemispheres were subjected to localized {sup 1}H-MRS, and after revascularization surgery, four patients underwent follow-up {sup 1}H-MRS. Decreased NAA/Cr ratios (1.35{+-}0.14 in patients vs. 1.55{+-}0.24 in controls) and Cho/Cr ratios (0.96{+-}0.13 in patients vs. 1.10{+-}0.11 in controls) were observed in frontal white matter. After revascularization surgery, NAA/Cr and Cho/Cr ratios in this region increased. In the basal ganglia, there is no abnormal metabolic ratios. Localized 1H-MRS revealed abnormal metabolic change in both hemispheres of children with moyamoya disease. Because of its non-invasive nature, {sup 1}H-MRS is potentially useful for the preoperative evaluation of metabolic abnormalities and their postoperative monitoring.

  6. Collateral vessels in moyamoya disease : comparison of MR and MRA with conventional angiography

    International Nuclear Information System (INIS)

    Shim, Joo Eun; Yoon, Dae Young; Yi, Jeong Geun; Kim, Ho Chul; Choi, Chul Sun; Bae, Sang Hoon

    1998-01-01

    To determine the value of magnetic resonance imaging (MR) and magnetic resonance angiography (MRA) in assessing collateral vessels of moyamoya disease. Twenty-four patients with moyamoya disease who underwent MR, 3D TOF MRA, and conventional angiography participated in this study. Two radiologists working independently and with no knowledge of the angiographic findings, interpreted the MR and MRA images. To determine the presence of parenchymal and leptomeningeal collaterals (48 hemispheres) and transdural collaterals (38 hemispheres in 19 patients were depicted by angiography of the external carotid), the findings were compared with those of angiography. Parenchymal, leptomeningeal, and transdural collaterals were depicted by conventional angiography in 34 (71%), 32 (67%), and 11 (29%) hemispheres respectively. The sensitivity and specificity of MR/MRA for collateral vessels were 79.1/88.1 % for parenchymal collaterals, 72.1/88.1 % for leptomeningeal collaterals, and 0.1/18.1 % for transdural collaterals, respectively. Respective sensitivity and specificity of MR/MRA were 88.94/94.1% for leptomeningeal collaterals, and 18.93/55.1 % for transdural collaterals, when the prominent posterior cerebral and external carotid artery were regarded as secondary signs of leptomeningeal and transdural collateral vessels. In moyamoya disease, MR and MRA are useful imaging modalities for the assessment of collateral vessels. The prominent posterior cerebral artery and external carotid artery can be useful secondary signs of leptomeningeal and transdural collateral vessels. (author). 18 refs., 2 figs

  7. Bleeding points in cerebral hemorrhage caused by Moyamoya disease in adults

    International Nuclear Information System (INIS)

    Sasaki, Tatsuya; Sakurai, Yoshiharu; Shimizu, Yukihiko; Ogawa, Akira; Komatsu, Shinro.

    1983-01-01

    Even before the introduction of CT we reported that the intracranial hemorrhage in Moyamoya disease was not subarachnoid hemorrhage but intraventricular hemorrhage and that the bleeding point was the paraventricular subependymal region of lateral ventricles; these findings were based on our experience with three Moyamoya cases in which ventricular hemorrhage occurred and pseudoaneurysms were revealed in the territory of the posterior choroidal artery. Twelve cases with intracranial hemorrhage caused by Moyamoya disease have now been studied by CT in order to determine (1) whether the hemorrhage is subarachnoid or intraventricular, and (2) where the bleeding point is. In the results for the eight cases for which the CT scan was performed within one day after the onset, intraventricular hemorrhage was shown in all cases. The bleeding point was examined in twelve cases; in four cases it was recognized by initial CT only, but if five cases in which ventricular hemorrhage only appeared in the initial CT,follow-up plain and contrast-enhanced CT were necessary. In a total of nine cases, then, bleeding points were recognized. In one case putaminal hemorrhage penetrated into the lateral ventricle, while in eight cases the intracerebral hematoma was located in the paraventricular region of the lateral ventricle, such as at the head of the caudate nucleus or the thalamus. In some cases, small subependymal hematoma projected into the lateral ventricle. In cases with symptoms of intracranial hemorrhage at the onset, the bleeding points were at the paraventricular parenchyma of the lateral ventricle in almost all cases. (author)

  8. Cases similar to the cerebrovascular 'Moyamoya disease'. Investigation by angiography and computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Tomura, Noriaki; Inugami, Atsushi; Higano, Shuichi; Fujita, Hideaki; Abe, Toushi; Shishido, Fumio; Uemura, Kazuo

    1988-10-01

    Findings of cerebral computed tomographies were investigated in 13 patients who did not satisfy the criteria of cerebrovascular Moyamoya disease, but showed the Moyamoya vessels (so-called pseudo-Moyamoya disease). Their ages ranged from 15 to 70 years old. Single patients were diagnosed as having pituitary adenoma after radiation therapy, head trauma, aplastic anemia, and the Rainoud phenomenon, and five as having hypertension. Angiography revealed occlusion and stenosis in the unilateral carotid forks in 7 and 5 patients, respectively. Other appearances included transdural anastomosis via the superficial temporal arteries, the anterior ethmoid arteries, and the collateral pathway from the perforating branches to the cortical branches. Low density areas were seen on CT in 10 patients, seven of whom had multiple areas. Low density areas were seen in the cerebral basal ganglia, watershed zone, and the outer side of the lateral cerebral body, in that order. Four patients had intracranial hemorrhage, subarachnoid hemorrhage, and hemorrhage infarction. Pseudo-Moyamoya disease was sometimes of the incomplete form of the cerebrovascular Moyamoya disease, and attributable to arterial stenosis or constricture due to radiation therapy. (Namekawa, K.).

  9. Hemodynamic evaluation of vascular reconstructive surgery for childhood moyamoya disease using single photon emission computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Takikawa, Shugo; Kamiyama, Hiroyasu; Abe, Hiroshi [Hokkaido Univ., Sapporo (Japan). School of Medicine; Mitsumori, Kenji; Tsuru, Mitsuo

    1990-06-01

    To evaluate the efficacy of vascular reconstructive surgery for childhood moyamoya disease, the cerebral blood flow (CBF) in 31 hemispheres of 16 patients was examined by single photon emission computed tomography (SPECT) using the {sup 133}Xe inhalation method. Results were divided into two groups; 17 hemispheres with superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis (A(+) group) and 14 hemispheres without anastomosis (A(-) group). The mean hemispheric CBF (mCBF) and regional CBF (rCBF) in the frontal, temporal, occipital, and basal ganglia regions were calculated. Pre- and postoperative SPECT on the 10 hemispheres of the A(+) group showed an increase in mCBF in 6 hemispheres, the disappearance of the low perfusion area (LPA) in all 5 hemispheres where LPA was present before surgery, and an improvement in rCBF distribution (an increase in rCBF in the frontal and temporal lobes and a decrease in the basal ganglia). This suggests that vascular reconstruction is greatly effective in treating this disease. A comparison between the A(+) group and the A(-) group by postoperative SPECT, as well as the clinical outcomes and the postoperative findings of electroencephalography and angiography, revealed that the A(+) group was superior to the A(-) group in the frequency of LPA (12% and 43%, respectively) and rCBF in the frontal region where STA-MCA anastomosis was usually performed. These results indicate that STA-MCA anastomosis with indirect synangiosis is the most effective treatment of childhood moyamoya disease. (author).

  10. Germline mutation of CBL is associated with moyamoya disease in a child with juvenile myelomonocytic leukemia and Noonan syndrome-like disorder.

    Science.gov (United States)

    Hyakuna, Nobuyuki; Muramatsu, Hideki; Higa, Takeshi; Chinen, Yasutsugu; Wang, Xinan; Kojima, Seiji

    2015-03-01

    Germline mutations in CBL have been identified in patients with Noonan syndrome-like phenotypes, while juvenile myelomonocytic leukemia (JMML) harbors duplication of a germline CBL, resulting in acquired isodisomy. The association between moyamoya disease and Noonan syndrome carrying a PTPN11 mutation has recently been reported. We present a patient with JMML who developed moyamoya disease and neovascular glaucoma. Our patient exhibited a Noonan syndrome-like phenotype. Genetic analysis revealed acquired isodisomy and a germline heterozygous mutation in CBL. This is a rare case of CBL mutation associated with moyamoya disease. Prolonged RAS pathway signaling may cause disruption of cerebrovascular development. © 2014 Wiley Periodicals, Inc.

  11. Moyamoya disease: impact on the performance of oral and written language.

    Science.gov (United States)

    Lamônica, Dionísia Aparecida Cusin; Ribeiro, Camila da Costa; Ferraz, Plínio Marcos Duarte Pinto; Tabaquim, Maria de Lourdes Merighi

    Moyamoya disease is an unusual form of occlusive, cerebrovascular disorder that affects the arteries of the central nervous system, causing acquired language alterations and learning difficulties. The study aim was to describe the oral/written language and cognitive skills in a seven-year-and-seven-month-old girl diagnosed with Moyamoya disease. The assessment consisted of interviews with her parents and application of the following instruments: Observation of Communicative Behavior, Peabody Picture Vocabulary Test, Academic Performance Test, Profile of Phonological Awareness, Raven's Progressive Matrices Test, Special Scale, Wechsler Intelligence Scale for Children, Bender Visual Motor Gestalt Test, and Wisconsin Card Sorting Test. Two episodes of stroke in the left and right temporal-parietal and left frontal areas occurred until the age of six years and five months. Revascularization surgery and medication treatment were conducted. The audiologic and ophthalmologic assessments indicated normality. At the time of the study, the girl was attending the second grade of elementary school. She presented changes in oral and written language (syllabic-alphabetic), non-naming of all graphemes, low arithmetic and writing means, reading skill below first grade level and psycholinguistic delay, and pre-school level phonological processing skills. The psychological evaluation indicated satisfactory intellectual level; however, it also showed cognitive performance impairment in verbal and execution tasks and limitations on graphic-perceptual-motor skills and sequential logic organization. The stroke episodes influenced the performance of learning processes, affecting the analysis, integration, and interpretation of relevant visual and auditory information.

  12. Medical management of moyamoya disease and recurrent stroke in an infant with Majewski osteodysplastic primordial dwarfism type II (MOPD II).

    Science.gov (United States)

    Kılıç, Esra; Utine, Eda; Unal, Sule; Haliloğlu, Göknur; Oğuz, Kader Karli; Cetin, Mualla; Boduroğlu, Koray; Alanay, Yasemin

    2012-10-01

    We report an infant diagnosed with Majewski osteodysplastic primordial dwarfism type II at age 8 months, who experienced cerebrovascular morbidities related to this entity. Molecular analysis identified c.2609+1 G>A, intron 14, homozygous splice site mutation in the pericentrin gene. At age 18 months, she developed recurrent strokes and hemiparesis. Brain magnetic resonance imaging and magnetic resonance angiography showed abnormal gyral pattern, cortical acute infarcts, bilateral stenosis of the internal carotid arteries and reduced flow on the cerebral arteries, consistent with moyamoya disease. In Majewski osteodysplastic primordial dwarfism type II, life expectancy is reduced because of high risk of stroke secondary to cerebral vascular anomalies (aneurysms, moyamoya disease). Periodic screening for vascular events is recommended in individuals with Majewski osteodysplastic primordial dwarfism type II every 12-18 months following diagnosis. Our patient was medically managed with low molecular weight heparin followed with aspirin prophylaxis, in addition to carbamazepine and physical rehabilitation. We report an infant with moyamoya disease and recurrent stroke presenting 10 months after diagnosis (at age 18 months), and discuss the outcome of nonsurgical medical management. The presented case is the second youngest case developing stroke and moyamoya disease.

  13. Moyamoya Disease – a Vasculopahty and an Uncommon Cause of Recurrent Cerebrovascular Accidents

    Directory of Open Access Journals (Sweden)

    Yasmin S Hamirani

    2008-09-01

    Full Text Available

    Moyamoya disease is a very rare chronic cerebrovascular disease of unknown etiology characterized by recurrent ischemic or hemorrhagic strokes. Initially diagnosed in Japan and named after finding puff of smoke like collateral blood vessels around the occluded blood vessels of circle of Willis. With increase awareness this disease is now diagnosed more often. Medical and surgical treatment have been used to treat the disease, with surgical treatment been mostly experimental. Special attention should be given to the surgical treatment which has shown to have an edge over the medical treatment in some clinical trials especially in young patients with recurrent strokes to prevent progressive cognitive decline and to improve their quality of life. In our patient, who is a young man, the diagnosis was picked up late and when surgical evaluation was performed, it was considered to be fruitless with findings of nonviable brain tissue on MRI imaging.

  14. Ischemic Stroke in Young Adults with Moyamoya Disease: Prognostic Factors for Stroke Recurrence and Functional Outcome after Revascularization.

    Science.gov (United States)

    Zhao, Meng; Deng, Xiaofeng; Gao, Faliang; Zhang, Dong; Wang, Shuo; Zhang, Yan; Wang, Rong; Zhao, Jizong

    2017-07-01

    Stroke in young adults is uncommon and rarely described. Moyamoya disease is one of the leading causes of stroke in young adults. We aimed to study the prognostic factors for stroke recurrences and functional outcomes in young stroke patients with moyamoya disease after revascularization. We reviewed 696 consecutive patients with moyamoya disease admitted to our hospital from 2009-2015 and identified patients aged 18-45 years with first-ever stroke. Follow-up was conducted via face-to-face or structured telephone interviews. Outcome measures were recurrent stroke events and unfavorable functional outcomes (modified Rankin Scale >2). We included 121 young patients with moyamoya disease suffering from stroke (initial presentation age, 35.4 ± 7.5 years). All patients underwent revascularization after the acute phase of initial stroke events as the secondary prevention for recurrences. During follow-up (median, 40 months), 9 patients (7.4%) experienced recurrent strokes and 8 of them (6.6%) suffered unfavorable functional outcomes. In the multivariate analysis, diabetes was an independent predictor for stroke recurrences (hazard ratio 6.76; 95% confidence interval 1.30-35.11; P = 0.02) and was significantly associated with unfavorable functional outcomes (odds ratio 7.87; 95% confidence interval 1.42-38.74; P = 0.01). We identified diabetes as an independent risk factor for recurrent strokes and unfavorable functional outcomes after revascularization in young stroke patients with moyamoya disease. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. How to minimize ischemic complication related to swollen temporalis muscle following indirect revascularization surgery in moyamoya disease: a technical report.

    Science.gov (United States)

    Joo, Sung Pil; Kim, Tae Sun; Moon, Hyung Sik

    2014-05-01

    There are several reports in the literature of postoperative ischemic events due to swelling of the temporalis muscle after indirect revascularization surgery. Here, we report our surgical technique for preventing ischemic events during the acute postoperative recovery period in moyamoya patients. We used various types of titanium mesh to cover the bony defect area in 8 patients (10 operations) with moyamoya disease. The mesh was cut and manipulated according to the shape of the bony defect. Surgical results were favorable, with no newly developed ischemic event or infarction in the acute recovery period. The mesh formed an outer table of skull, so there was no compressive effect on the temporalis muscle and no cosmetic defects. The titanium mesh appears to be effective and useful for prevention of ischemic insult in the treatment of moyamoya disease. The choice of this procedure depends on both the operative findings of temporalis muscle thickness and the status of ischemic vulnerability of moyamoya brain. Georg Thieme Verlag KG Stuttgart · New York.

  16. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study)

    OpenAIRE

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu

    2016-01-01

    Objectives To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Design Retrospective, multicentre, non-randomised, observational study. Setting Nationwide registry data in Japan. Participants A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. Main outcome measure We performed propensity score-matc...

  17. Pediatric Celiac Disease

    Science.gov (United States)

    ... a protein found in wheat, rye, and barley. Pediatric Celiac Disease If your child has celiac disease, ... physician. Established by the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) Celiac Disease Eosinophilic ...

  18. Effect of Aspirin in Postoperative Management of Adult Ischemic Moyamoya Disease.

    Science.gov (United States)

    Zhao, Yahui; Zhang, Qian; Zhang, Dong; Zhao, Yuanli

    2017-09-01

    Aspirin has been implicated in the postoperative management of moyamoya disease (MMD) in order to avoid bypass failure and decrease the incidence of subsequent stroke. However, its effect has not been completely determined yet. In this study, we retrospectively reviewed data of 184 adult patients (197 hemispheres) presented with ischemic-onset MMD who had undergone direct or combined revascularization in our hospital, to clarify the effect of postoperative aspirin therapy in the management of moyamoya disease. Fifty-nine hemispheres that had been administered with aspirin (100 mg/day) after bypass surgery were defined as the "aspirin group," whereas 138 that hadn't been given aspirin postoperatively were defined as the "control group". Among 197 hemispheres, the mortality rate was 0. The incidence of postoperative newly developed infarction, transient ischemic attack, and hemorrhage were not significantly different between the aspirin and control groups. The patency rate of bypass graft was not significantly different between the groups, either. Notably, more patients experienced major stroke in the control group (9/138) than the aspirin group (1/59), but no statistical difference was found (P > 0.05). In the aspirin group, more patients had improved outcome than the control group (P = 0.04). Our findings showed that aspirin might not decrease the incidence of postoperative ischemic stroke or increase patency rate of bypass graft, but it does not increase the risk of hemorrhages, either. Also, postoperative aspirin therapy might improve outcome. More studies are needed to provide evidence for postoperative antiplatelet therapy in MMD management. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Voxel Based Analysis of Surgical Revascularization for Moyamoya Disease: Pre- and Postoperative SPECT Studies.

    Directory of Open Access Journals (Sweden)

    Yasutaka Fushimi

    Full Text Available Moyamoya disease (MMD is a chronic, progressive, cerebrovascular occlusive disease that causes abnormal enlargement of collateral pathways (moyamoya vessels in the region of the basal ganglia and thalamus. Cerebral revascularization procedures remain the preferred treatment for patients with MMD, improving the compromised cerebral blood flow (CBF. However, voxel based analysis (VBA of revascularization surgery for MMD based on data from pre- and postoperative data has not been established. The latest algorithm called as Diffeomorphic Anatomical Registration Through Exponentiated Lie Algebra (DARTEL has been introduced for VBA as the function of statistical parametric mapping (SPM8, and improved registration has been achieved by SPM8 with DARTEL. In this study, VBA was conducted to evaluate pre- and postoperative single photon emission computed tomography (SPECT images for MMD by SPM8 with DARTEL algorithm, and the results were compared with those from SPM8 without DARTEL (a conventional method. Thirty-two patients with MMD who underwent superficial temporal artery-middle cerebral artery (STA-MCA bypass surgery as the first surgery were included and all patients underwent pre- and postoperative 3D T1-weighted imaging and SPECT. Pre- and postoperative SPECT images were registered to 3D T1-weighted images, then VBA was conducted. Postoperative SPECT showed more statistically increased CBF areas in the bypassed side cerebral hemisphere by using SPM8 with DARTEL (58,989 voxels; P<0.001, and increased ratio of CBF after operation was less than 15%. Meanwhile, postoperative SPECT showed less CBF increased areas by SPM8 without DARTEL. In conclusion, VBA was conducted for patients with MMD, and SPM8 with DARTEL revealed that postoperative SPECT showed statistically significant CBF increases over a relatively large area and with at most 15% increase ratio.

  20. Longitudinal anterior-to-posterior shift of collateral channels in patients with moyamoya disease: an implication for its hemorrhagic onset.

    Science.gov (United States)

    Yamamoto, Shusuke; Hori, Satoshi; Kashiwazaki, Daina; Akioka, Naoki; Kuwayama, Naoya; Kuroda, Satoshi

    2018-03-23

    OBJECTIVE This study aimed to assess longitudinal changes in the collateral channels originating from the lenticulostriate artery (LSA), posterior communicating artery (PCoA), and anterior and posterior choroidal arteries (AChA and PChA, respectively) during disease progression and/or aging. The impact of collateral channels on onset type was also examined. METHODS This study included 71 involved hemispheres in 41 patients with moyamoya disease. The disease was categorized into 6 stages according to Suzuki's angiographic staging system. The degree of development of each moyamoya vessel was categorized into 3 grades. RESULTS The LSA started to dilate in stage 2, showed the most prominent development in stage 3, and decreased in more advanced stages (p PCoA started to dilate in stage 3 and showed the most prominent development in stage 4 (p = 0.03). The PChA started to dilate in stage 3 and showed the most prominent development in stages 4 to 5 (p PCoA (p = 0.02, R = 0.28) and PChA (p PCoA, AChA, and PChA more distinctly developed in hemispheres with intracerebral or intraventricular hemorrhage than in hemispheres with ischemic stroke or transient ischemic attack (p < 0.001, p = 0.03, and p = 0.03, respectively). CONCLUSIONS This study suggests that the collateral channels through moyamoya vessels longitudinally shift from the anterior to posterior component during disease progression and aging, which may be closely related to the onset of hemorrhagic stroke in adult moyamoya disease.

  1. Clinical Significance of the Champagne Bottle Neck Sign in the Extracranial Carotid Arteries of Patients with Moyamoya Disease.

    Science.gov (United States)

    Yasuda, C; Arakawa, S; Shimogawa, T; Kanazawa, Y; Sayama, T; Haga, S; Morioka, T

    2016-05-26

    The champagne bottle neck sign represents a rapid reduction in the extracranial ICA diameters and is a characteristic feature of Moyamoya disease. However, the clinical significance of the champagne bottle neck sign is unclear. We investigated the relationship between the champagne bottle neck sign and the clinical and hemodynamic stages of Moyamoya disease. We analyzed 14 patients with Moyamoya disease before revascularization (5 men, 9 women; age, 43.2 ± 19.3 years). The ratio of the extracranial ICA and common carotid artery diameters was determined using carotid ultrasonography or cerebral angiography; a ratio of champagne bottle neck sign-positive. The clinical disease stage was determined using the Suzuki angiographic grading system. CBF and cerebral vasoreactivity also were measured. The ICA/common carotid artery ratio (expressed as median [interquartile range]) decreased as the clinical stage advanced (stages I-II, 0.71 [0.60-0.77]; stages III-IV, 0.49 [0.45-0.57]; stages V-VI, 0.38 [0.34-0.47]; P champagne bottle neck sign-positive arteries were classified as Suzuki stage ≥III, 73% were symptomatic, and 89% exhibited reduced cerebral vasoreactivity. In contrast, all champagne bottle neck sign-negative arteries were Suzuki stage ≤III, 67% were asymptomatic, and all showed preserved cerebral vasoreactivity. The champagne bottle neck sign was related to advanced clinical stage, clinical symptoms, and impaired cerebral vasoreactivity. Thus, detection of the champagne bottle neck sign might be useful in determining the clinical and hemodynamic stages of Moyamoya disease. © 2016 American Society of Neuroradiology.

  2. Moyamoya disease-associated protein mysterin/RNF213 is a novel AAA+ ATPase, which dynamically changes its oligomeric state

    Science.gov (United States)

    Morito, Daisuke; Nishikawa, Kouki; Hoseki, Jun; Kitamura, Akira; Kotani, Yuri; Kiso, Kazumi; Kinjo, Masataka; Fujiyoshi, Yoshinori; Nagata, Kazuhiro

    2014-03-01

    Moyamoya disease is an idiopathic human cerebrovascular disorder that is characterized by progressive stenosis and abnormal collateral vessels. We recently identified mysterin/RNF213 as its first susceptibility gene, which encodes a 591-kDa protein containing enzymatically active P-loop ATPase and ubiquitin ligase domains and is involved in proper vascular development in zebrafish. Here we demonstrate that mysterin further contains two tandem AAA+ ATPase modules and forms huge ring-shaped oligomeric complex. AAA+ ATPases are known to generally mediate various biophysical and mechanical processes with the characteristic ring-shaped structure. Fluorescence correlation spectroscopy and biochemical evaluation suggested that mysterin dynamically changes its oligomeric forms through ATP/ADP binding and hydrolysis cycles. Thus, the moyamoya disease-associated gene product is a unique protein that functions as ubiquitin ligase and AAA+ ATPase, which possibly contributes to vascular development through mechanical processes in the cell.

  3. Moyamoya disease-associated protein mysterin/RNF213 is a novel AAA+ ATPase, which dynamically changes its oligomeric state

    Science.gov (United States)

    Morito, Daisuke; Nishikawa, Kouki; Hoseki, Jun; Kitamura, Akira; Kotani, Yuri; Kiso, Kazumi; Kinjo, Masataka; Fujiyoshi, Yoshinori; Nagata, Kazuhiro

    2014-01-01

    Moyamoya disease is an idiopathic human cerebrovascular disorder that is characterized by progressive stenosis and abnormal collateral vessels. We recently identified mysterin/RNF213 as its first susceptibility gene, which encodes a 591-kDa protein containing enzymatically active P-loop ATPase and ubiquitin ligase domains and is involved in proper vascular development in zebrafish. Here we demonstrate that mysterin further contains two tandem AAA+ ATPase modules and forms huge ring-shaped oligomeric complex. AAA+ ATPases are known to generally mediate various biophysical and mechanical processes with the characteristic ring-shaped structure. Fluorescence correlation spectroscopy and biochemical evaluation suggested that mysterin dynamically changes its oligomeric forms through ATP/ADP binding and hydrolysis cycles. Thus, the moyamoya disease-associated gene product is a unique protein that functions as ubiquitin ligase and AAA+ ATPase, which possibly contributes to vascular development through mechanical processes in the cell. PMID:24658080

  4. Surgical excision of heterotopic ossification of hip in a rare case of Moyamoya disease with extra articular ankylosis

    Directory of Open Access Journals (Sweden)

    Dhanasekararaja Palanisami

    2012-01-01

    Full Text Available We report a case of isolated ossification of iliopsoas with ankylosis of the left hip in a 27-year-old female. The patient was diagnosed to have Moyamoya disease, a rare chronic occlusive disorder of cerebrovascular circulation following an acute onset of hemiplegia. The patient presented 9 months later to us with ankylosis of left hip which was successfully treated by surgical excision of the heterotopic bone and there was no recurrence at the end of 5 years. A review of literature failed to reveal a similar case with isolated and complete ossification of iliopsoas muscle associated with Moyamoya disease which required surgical intervention. Surgical excision resulted in dramatic improvement in the quality of life. Surgical excision of neurogenic type of heterotopic ossification is a very successful procedure and timely intervention after maturity of mass is very important to prevent the onset of secondary complications and to avoid recurrence.

  5. Moyamoya Disease

    Science.gov (United States)

    ... Stroke Association 9707 East Easter Lane Suite B Centennial CO Centennial, CO 80112-3747 info@stroke.org http://www. ... Stroke Association 9707 East Easter Lane Suite B Centennial CO Centennial, CO 80112-3747 info@stroke.org ...

  6. Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease: Correlation with Clinical Severity and Old Brain Lesions

    OpenAIRE

    Seo, Kwon-Duk; Suh, Sang Hyun; Kim, Yong Bae; Kim, Ji Hwa; Ahn, Sung Jun; Kim, Dong-Seok; Lee, Kyung-Yul

    2015-01-01

    Purpose Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. Materials and Methods FLAIR images of 83 patients were reviewed. Each cerebr...

  7. Ivy signs on FLAIR images before and after STA-MCA anastomosis in patients with Moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Ideguchi, Reiko; Enokizono, Mikako; Uetani, Masataka (Dept. of Radiological Sciences, Nagasaki Univ. Graduate School of Biomedical Sciences, Nagasaki (Japan)), e-mail: qqtt37gd9@forest.ocn.ne.jp; Morikawa, Minoru (Dept. of Radiology, Nagasaki Univ. School of Medicine, Nagasaki (Japan)); Ogawa, Yoji (Dept. of Radiology, Hanwa Daini Senboku Hospital, Osaka (Japan)); Nagata, Izumi (Dept. of Neurosurgery, Nagasaki Univ. School of Medicine, Nagasaki (Japan))

    2011-04-15

    Background: Leptomeningeal high signal intensity (ivy sign) on fluid-attenuated inversion-recovery (FLAIR) MR imaging is one of the features of Moyamoya disease. However, the correlation between ivy sign and cerebral perfusion status has not been fully evaluated. Purpose: To characterize ivy sign on FLAIR images in Moyamoya disease and compare this finding with hemodynamic alterations on perfusion single-photon emission CT (SPECT) obtained before and after bypass surgery. Material and Methods: Sixteen patients with angiographically confirmed Moyamoya disease who underwent superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis were included in the study. The presence of ivy sign on FLAIR images was classified as 'negative', 'minimal' and 'positive'. We evaluated the relationship between ivy sign and findings of SPECT, including cerebral vascular reserve (CVR) before and after surgery. Results: Minimal or positive ivy sign was seen in 13 (81%) of 16 patients, and 21 (66%) of 32 hemispheres. CVR in the areas with positive or minimal ivy sign was lower than that in the areas with negative ivy sign. After STA-MCA anastomosis, ivy sign disappeared or decreased in all 21 hemispheres demonstrating ivy sign. SPECT demonstrated apparent hemodynamic improvement in areas demonstrating disappearance or decrease of ivy sign. Conclusion: Ivy sign on FLAIR image is seen in areas with decreased cerebral perfusion. The sign is useful for non-invasive assessment of cerebral hemodynamic status before and after surgery

  8. Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging finings

    Energy Technology Data Exchange (ETDEWEB)

    Noguchi, Tomoyuki, E-mail: tnogucci@radiol.med.kyushu-u.ac.jp [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Kawashima, Masatou [Department of Neurosurgery, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Nishihara, Masashi; Hirai, Tetsuyoshi [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Matsushima, Toshio [Department of Neurosurgery, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan); Irie, Hiroyuki [Department of Radiology, Faculty of Medicine, Saga University, 5-1-1, Nabeshima, Saga 849-8501 (Japan)

    2013-12-01

    Purpose: Our purpose was to identify the causal factors for the perfusion distribution obtained with ASL-MRI by comparing ASL-MRI with clinical information and other MRI findings in moyamoya disease. Methods: Seventy-one patients with moyamoya disease underwent ASL-MRI and other MRI, including fluid-attenuated inversion recovery imaging (FLAIR) and three-dimensional time-of-flight magnetic resonance angiography (MRA) on 3.0-Tesla MRI system. Cerebral blood flow (CBF) values (ASL values) for the cerebral hemispheres (142 sides) were measured on CBF maps generated by ASL-MRI. Relationships between the ASL values and the following 9 factors were assessed: sex, family history, revascularization surgery, age at MR exam, age at onset, the steno-occlusive severity on MRA (MRA score), degree of basal collaterals, degree of leptomeningeal high signal intensity seen on FLAIR, and size of ischemic or hemorrhagic cerebrovascular accident lesion (CVA score). Results: Patients with a family history had significantly higher ASL values than those without such a history. There were significant negative correlations between ASL values and age at MR exam, MRA score, and CVA score. Conclusions: ASL-MRI may have cause-and-effect or mutual associations with family history, current patient age, size of CVA lesion, and intracranial arterial steno-occlusive severity in Moyamoya disease.

  9. Arterial spin-labeling MR imaging in moyamoya disease compared with clinical assessments and other MR imaging finings

    International Nuclear Information System (INIS)

    Noguchi, Tomoyuki; Kawashima, Masatou; Nishihara, Masashi; Hirai, Tetsuyoshi; Matsushima, Toshio; Irie, Hiroyuki

    2013-01-01

    Purpose: Our purpose was to identify the causal factors for the perfusion distribution obtained with ASL-MRI by comparing ASL-MRI with clinical information and other MRI findings in moyamoya disease. Methods: Seventy-one patients with moyamoya disease underwent ASL-MRI and other MRI, including fluid-attenuated inversion recovery imaging (FLAIR) and three-dimensional time-of-flight magnetic resonance angiography (MRA) on 3.0-Tesla MRI system. Cerebral blood flow (CBF) values (ASL values) for the cerebral hemispheres (142 sides) were measured on CBF maps generated by ASL-MRI. Relationships between the ASL values and the following 9 factors were assessed: sex, family history, revascularization surgery, age at MR exam, age at onset, the steno-occlusive severity on MRA (MRA score), degree of basal collaterals, degree of leptomeningeal high signal intensity seen on FLAIR, and size of ischemic or hemorrhagic cerebrovascular accident lesion (CVA score). Results: Patients with a family history had significantly higher ASL values than those without such a history. There were significant negative correlations between ASL values and age at MR exam, MRA score, and CVA score. Conclusions: ASL-MRI may have cause-and-effect or mutual associations with family history, current patient age, size of CVA lesion, and intracranial arterial steno-occlusive severity in Moyamoya disease

  10. Posterior cerebral artery involvement in moyamoya disease: initial infarction and angle between PCA and basilar artery.

    Science.gov (United States)

    Lee, Ji Yeoun; Kim, Seung-Ki; Cheon, Jung-Eun; Choi, Jung Won; Phi, Ji Hoon; Kim, In-One; Cho, Byung-Kyu; Wang, Kyu-Chang

    2013-12-01

    Moyamoya disease (MMD) is a chronic cerebrovascular occlusive disease, and progressive involvement of the posterior cerebral artery (PCA) has been reported. However, majority of MMD articles are presenting classic anterior circulation related issues. This study investigates the preoperative factors related to the long-term outcome of posterior circulation in MMD. Retrospective review of 88 MMD patients (166 PCAs in either hemisphere) without symptomatic disease involvement of PCA at initial diagnosis was done. Data at initial diagnosis regarding age, presence of infarction, status of the PCA, type of posterior communicating artery, and the angle between PCA and basilar artery were reviewed. Progressive stenosis of PCA was evaluated by symptom or radiological imaging during follow up. During an average follow up of 8.3 years, 29 out of 166 (18 %) evaluated PCAs showed progressive disease involvement. The average time of progression from the initial operation was 4.9 years, with the latest onset at 10.8 years. The patients who showed progressive stenosis of the PCA tended to be younger, present with infarction, have smaller angle between PCA and basilar artery, and have asymptomatic stenosis of the PCA at initial presentation. However, multivariate analysis confirmed only the presence of initial infarction and a smaller angle between PCA and basilar artery to be significantly associated with progressive stenosis of PCA. Involvement of PCA in MMD may occur in a delayed fashion, years after the completion of revascularization of anterior circulation. Persistent long-term follow-up regarding the posterior circulation is recommended.

  11. 99mTc-ECD brain SPECT in patients with Moyamoya disease: a reflection of cerebral perfusion status at tissue level in the disease process

    International Nuclear Information System (INIS)

    Kashyap, Raghava; Mittal, Bhagwant Rai; Sunil, Hejjaji Venkataramarao; Bhattacharya, Anish; Singh, Baljinder; Mukherjee, Kanchan Kumar; Gupta, Sunil Kumar

    2011-01-01

    Moyamoya disease is a rare, progressive cerebrovascular disorder caused by intracranial stenosis of the circle of Willis, resulting in successive ischemic events. Computed tomography (CT) and magnetic resonance imaging (MRI) play a major role in diagnosis. The aim of the study was to describe the spectrum of findings on brain SPECT in patients with Moyamoya disease and to compare the findings with other investigations. 99m Tc-ECD SPECT scans of seventeen patients (7 children and 10 adults) were analysed to study the brain perfusion. Features of Moyamoya disease were detected on DSA in 11 patients, CTA in one, MR angiography in one patient. Brain perfusion SPECT analysis showed unilateral perfusion defects in 11 patients, normal perfusion in 2 and bilateral defects in 4 patients. No perfusion defects despite bilateral vascular changes were noted in one patient. Cerebral infarcts were detected on MRI unilaterally in three subjects while multiple infarcts were identified in one. 99m Tc-ECD Brain SPECT showed perfusion defects that were more extensive compared to those detected on MRI. Post acetazolamide studies for assessment of cerebrovascular reserve were done in three patients. Two of them showed good cerebrovascular reserve (>1). Follow-up studies post-surgical procedures (Myo-dura synangiosis) done in two patients showed partial resolution of perfusion defects in the involved areas. Brain perfusion scintigraphy is an important adjunct in evaluation of patients with Moyamoya disease yielding information about the direct end results of the pathology in the vessels and also prognostic information. (author)

  12. "Ocular moyamoya" syndrome in a patient with features of microcephalic osteodysplastic primordial dwarfism type II.

    Science.gov (United States)

    Bang, Genie M; Kirmani, Salman; Patton, Alice; Pulido, Jose S; Brodsky, Michael C

    2013-02-01

    Primordial dwarfism refers to severely impaired growth beginning early in fetal life. There are many genetic causes of primordial dwarfism, including disorders classified as microcephalic osteodysplastic primordial dwarfism. Microcephalic osteodysplastic primordial dwarfism type II is an autosomal-recessive disease characterized by small stature, bone and dental anomalies, and characteristic facies. Affected patients have a high risk of stroke secondary to progressive cerebral vascular anomalies, which often are classified as moyamoya disease. We present the case of a boy with features suggestive of MOPD II with unilateral moyamoya cerebrovascular changes and correlative moyamoya collaterals involving the iris of the ipsilateral eye. Copyright © 2013 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

  13. Perfusion characteristics of Moyamoya disease: an anatomically and clinically oriented analysis and comparison.

    Science.gov (United States)

    Schubert, Gerrit Alexander; Czabanka, Marcus; Seiz, Marcel; Horn, Peter; Vajkoczy, Peter; Thomé, Claudius

    2014-01-01

    Moyamoya disease (MMD) is characterized by unique angiographic features of collateralization. However, a detailed quantification as well as comparative analysis with cerebrovascular atherosclerotic disease (CAD) and healthy controls have not been performed to date. We reviewed 67 patients with MMD undergoing Xenon-enhanced computed tomography, as well as 108 patients with CAD and 5 controls. In addition to cortical, central, and infratentorial regions of interest, particular emphasis was put on regions that are typically involved in MMD (pericallosal territory, basal ganglia). Cerebral blood flow (CBF), cerebrovascular reserve capacity (CVRC), and hemodynamic stress distribution were calculated. MMD is characterized by a significant, ubiquitous decrease in CVRC and a cortical but not pericallosal decrease in CBF when compared with controls. Baseline perfusion is maintained within the basal ganglia, and hemodynamic stress distribution confirmed a relative preservation of central regions of interest in MMD, indicative for its characteristic proximal collateralization pattern. In MMD and CAD, cortical and central CBF decreased significantly with age, whereas CVRC and hemodynamic stress distribution are relatively unaffected by age. No difference in CVRC of comparable regions of interest was seen between MMD and CAD, but stress distribution was significantly higher in MMD, illustrating the functionality of the characteristic rete mirabilis. Our data provide quantitative support for a territory-specific perfusion pattern that is unique for MMD, including central preservation of CBF compared with controls and patients with CAD. This correlates well with its characteristic feature of proximal collateralization. CVRC and hemodynamic stress distribution seem to be more robust parameters than CBF alone for assessment of disease severity.

  14. Identification of RNF213 as a susceptibility gene for moyamoya disease and its possible role in vascular development.

    Directory of Open Access Journals (Sweden)

    Wanyang Liu

    Full Text Available Moyamoya disease is an idiopathic vascular disorder of intracranial arteries. Its susceptibility locus has been mapped to 17q25.3 in Japanese families, but the susceptibility gene is unknown.Genome-wide linkage analysis in eight three-generation families with moyamoya disease revealed linkage to 17q25.3 (P<10(-4. Fine mapping demonstrated a 1.5-Mb disease locus bounded by D17S1806 and rs2280147. We conducted exome analysis of the eight index cases in these families, with results filtered through Ng criteria. There was a variant of p.N321S in PCMTD1 and p.R4810K in RNF213 in the 1.5-Mb locus of the eight index cases. The p.N321S variant in PCMTD1 could not be confirmed by the Sanger method. Sequencing RNF213 in 42 index cases confirmed p.R4810K and revealed it to be the only unregistered variant. Genotyping 39 SNPs around RNF213 revealed a founder haplotype transmitted in 42 families. Sequencing the 260-kb region covering the founder haplotype in one index case did not show any coding variants except p.R4810K. A case-control study demonstrated strong association of p.R4810K with moyamoya disease in East Asian populations (251 cases and 707 controls with an odds ratio of 111.8 (P = 10(-119. Sequencing of RNF213 in East Asian cases revealed additional novel variants: p.D4863N, p.E4950D, p.A5021V, p.D5160E, and p.E5176G. Among Caucasian cases, variants p.N3962D, p.D4013N, p.R4062Q and p.P4608S were identified. RNF213 encodes a 591-kDa cytosolic protein that possesses two functional domains: a Walker motif and a RING finger domain. These exhibit ATPase and ubiquitin ligase activities. Although the mutant alleles (p.R4810K or p.D4013N in the RING domain did not affect transcription levels or ubiquitination activity, knockdown of RNF213 in zebrafish caused irregular wall formation in trunk arteries and abnormal sprouting vessels.We provide evidence suggesting, for the first time, the involvement of RNF213 in genetic susceptibility to moyamoya

  15. Arterial spin labelling MRI for assessment of cerebral perfusion in children with moyamoya disease: comparison with dynamic susceptibility contrast MRI

    Energy Technology Data Exchange (ETDEWEB)

    Goetti, Robert [University Children' s Hospital Zurich, Department of Diagnostic Imaging, Zurich (Switzerland); University Hospital Zurich, Department of Diagnostic and Interventional Radiology, Zurich (Switzerland); O' Gorman, Ruth [University Children' s Hospital Zurich, Center for MR Research, Zurich (Switzerland); Khan, Nadia [University Children' s Hospital Zurich, Moyamoya Center, Division of Neurosurgery, Department of Surgery, Zurich (Switzerland); Kellenberger, Christian J.; Scheer, Ianina [University Children' s Hospital Zurich, Department of Diagnostic Imaging, Zurich (Switzerland)

    2013-05-15

    This study seeks to evaluate the diagnostic accuracy of cerebral perfusion imaging with arterial spin labelling (ASL) MR imaging in children with moyamoya disease compared to dynamic susceptibility contrast (DSC) imaging. Ten children (7 females; age, 9.2 {+-} 5.4 years) with moyamoya disease underwent cerebral perfusion imaging with ASL and DSC on a 3-T MRI scanner in the same session. Cerebral perfusion images were acquired with ASL (pulsed continuous 3D ASL sequence, 32 axial slices, TR = 5.5 s, TE = 25 ms, FOV = 24 cm, matrix = 128 x 128) and DSC (gradient echo EPI sequence, 35 volumes of 28 axial slices, TR = 2,000 ms, TE = 36 ms, FOV = 24 cm, matrix = 96 x 96, 0.2 ml/kg Gd-DOTA). Cerebral blood flow maps were generated. ASL and DSC images were qualitatively assessed regarding perfusion of left and right ACA, MCA, and PCA territories by two independent readers using a 3-point-Likert scale and quantitative relative cerebral blood flow (rCBF) was calculated. Correlation between ASL and DSC for qualitative and quantitative assessment and the accuracy of ASL for the detection of reduced perfusion per territory with DSC serving as the standard of reference were calculated. With a good interreader agreement ({kappa} = 0.62) qualitative perfusion assessment with ASL and DSC showed a strong and significant correlation ({rho} = 0.77; p < 0.001), as did quantitative rCBF (r = 0.79; p < 0.001). ASL showed a sensitivity, specificity and accuracy of 94 %, 93 %, and 93 % for the detection of reduced perfusion per territory. In children with moyamoya disease, unenhanced ASL enables the detection of reduced perfusion per vascular territory with a good accuracy compared to contrast-enhanced DSC. (orig.)

  16. Evolution into moyamoya disease in an infant with internal carotid artery aneurysms

    Directory of Open Access Journals (Sweden)

    Ryosuke Tanaka

    2017-03-01

    Conclusions: This is the first report of MMD in which ICA aneurysms and occlusions developed bilaterally in early infancy without moyamoya collateral vessels. Our case indicates that angiogenesis at the base of the brain may occur following extracellular matrix remodeling at the terminal portion of the ICAs.

  17. Outcome in adult patients with hemorrhagic moyamoya disease after combined extracranial-intracranial bypass.

    Science.gov (United States)

    Jiang, Hanqiang; Ni, Wei; Xu, Bin; Lei, Yu; Tian, Yanlong; Xu, Feng; Gu, Yuxiang; Mao, Ying

    2014-11-01

    The outcome of patients with hemorrhagic moyamoya disease (MMD) after cerebral revascularization is uncertain. The purpose of this study was to delineate the efficacy of this surgical method in the treatment of hemorrhagic MMD. Between January 2007 and August 2011, a consecutive cohort of 113 patients with hemorrhagic MMD was enrolled into this prospective single-center cohort study. The surgical method was combined direct and indirect bypass. The cumulative probability of the primary end point (all stroke and deaths from surgery through 30 days after surgery and ipsilateral recurrent hemorrhage afterward) was analyzed. The angiographic outcome was measured by the following parameters: bypass patency, reduction of basal MMD vessels, improved degree of dilation, and branch extension of the anterior choroidal and posterior communicating arteries (AChA-PCoA). Of the 113 enrolled cases, CT scans revealed pure intraventricular hemorrhage (IVH) in 63 cases (55.7%), pure intracranial hemorrhage (ICH) in 14 cases (12.4%), and ICH with IVH in 36 cases (31.9%). In 74 of 113 hemorrhagic hemispheres (65.5%), the AChA-PCoA was extremely dilated with extensive branches beyond the choroidal fissure. A total of 114 surgeries were performed. No patient suffered ischemic or hemorrhagic stroke through 30 days after surgery. Ipsilateral rebleeding occurred in 5 patients, 4 of whom died of the rebleeding event. The cumulative probability of the primary end point was 0% at 1 year and 1.9% at 2 years. The annual rebleeding rate was 1.87%/person/year. The improvement in AChA-PCoA extension was observed in 75 of 107 operated hemispheres (70.1%), which was higher than that in 7 of 105 unoperated hemispheres (35.2%). Revascularization may provide a benefit over conservative therapy for hemorrhagic MMD patients. The improvement of dilation and branch extension of AChA-PCoA might be correlated with the low rebleeding rate.

  18. Comparison of 7T and 3T MRI in patients with moyamoya disease.

    Science.gov (United States)

    Oh, Byeong Ho; Moon, Hyeong Cheol; Baek, Hyeon Man; Lee, Youn Joo; Kim, Sang Woo; Jeon, Young Jai; Lee, Gun Seok; Kim, Hong Rae; Choi, Jai Ho; Min, Kyung Soo; Lee, Mou Seop; Kim, Young Gyu; Kim, Dong Ho; Kim, Won Seop; Park, Young Seok

    2017-04-01

    Magnetic resonance imaging and magnetic resonance angiography (MRI/MRA) are widely used for evaluating the moyamoya disease (MMD). This study compared the diagnostic accuracy of 7Tesla (T) and 3T MRI/MRA in MMD. In this case control study, 12 patients [median age: 34years; range (10-66years)] with MMD and 12 healthy controls [median age: 25years; range (22-59years)] underwent both 7T and 3T MRI/MRA. To evaluate the accuracy of MRI/MRA in MMD, five criteria were compared between imaging systems of 7T and 3T: Suzuki grading system, internal carotid artery (ICA) diameter, ivy sign, flow void of the basal ganglia on T2-weighted images, and high signal intensity areas of the basal ganglia on time-of-flight (TOF) source images. No difference was observed between 7T and 3T MRI/MRA in Suzuki stage, ICA diameter, and ivy sign score; while, 7T MRI/MRA showed a higher detection rate in the flow void on T2-weighted images and TOF source images (p<0.001). Receiver operating characteristic curves of both T2 and TOF criteria showed that 7T MRI/MRA had higher sensitivity and specificity than 3T MRI/MRA. Our findings indicate that 7T MRI/MRA is superior to 3T MRI/MRA for the diagnosis of MMD in point of detecting the flow void in basal ganglia by T2-weighted and TOF images. Copyright © 2016. Published by Elsevier Inc.

  19. Neurologic Deterioration in Patients with Moyamoya Disease during Pregnancy, Delivery, and Puerperium.

    Science.gov (United States)

    Park, Wonhyoung; Ahn, Jae Sung; Chung, Jaewoo; Chung, Yeongu; Lee, Seungjoo; Park, Jung Cheol; Kwun, Byung Duk

    2018-03-01

    We reviewed our clinical experience of patients with moyamoya disease (MMD) who gave birth and assessed characteristics of those experiencing neurologic deterioration. The patients were classified into patients diagnosed with MMD during pregnancy and puerperium (group 1) and those diagnosed before pregnancy (group 2). We retrospectively reviewed patient characteristics, MMD treatment, neurologic symptoms before and during pregnancy and/after puerperium, obstetrical history, and delivery type in groups 1 and 2. Group 1 included 2 patients with deterioration of pre-existing transient ischemic attacks (TIAs) and acute cerebral infarction and 1 patient with seizures and newly developed TIAs during pregnancy and/or puerperium. Group 2 included 20 patients with 23 pregnancies. In group 2, 4 patients had deterioration of TIAs during pregnancy and puerperium. There were significant differences between the cases without neurologic deterioration and with deterioration in group 2 (TIAs ≥10 before pregnancy, 0% vs. 75%, P = 0.002; severely reduced regional cerebrovascular reserve on single-photon emission computed tomography, 10.5% vs. 100%, P = 0.002; and surgical revascularization before pregnancy, 75% vs. 15.8%, P = 0.04). In groups 1 and 2, 6 of the 7 cases in which TIAs occurred or worsened during pregnancy or puerperium recovered to prepregnancy TIA levels after puerperium. Patients with severely reduced regional cerebrovascular reserve on single-photon emission computed tomography and frequent TIAs before pregnancy may experience neurologic deterioration during pregnancy, delivery, and puerperium. Surgical revascularization before pregnancy may decrease neurologic deterioration during these periods. Copyright © 2017 Elsevier Inc. All rights reserved.

  20. The relationship between cerebral infarction on MR and angiographic findings in moyamoya disease: significance of the posterior circulation

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    Lee, Eun Ja; Song, Soon Young [College of Medicine, Kwangdong Univ., Koyang (Korea, Republic of); Yu, Won Jong; Jung, So Lyung; Chung, Bong Gak; Kag, Si Won [College of Medicine, The Catholic Univ. of Korea, Seoul (Korea, Republic of); Kim, Man Deuk [College of Medicine, Pochon CHA Univ., Pochon (Korea, Republic of)

    2002-06-01

    To investigate the relationship between changes in the posterior and anterior circulation, as seen at angiography, and the frequency and extent of cerebral infarction revealed by MR imaging in moyamoya disease. This study involved 34 patients (22 females and 12 males, aged 2-52 years) in whom cerebral angiography revealed the presence of moyamoya disease (bilateral; unilateral= 24:10; total hemispheres=58) and who also underwent brain MR imaging. To evaluate the angiographic findings, we applied each angiographic staging system to the anterior and posterior circulation. Leptomeningeal collateral circulation from the cortical branches of the posterior cerebral artery (PCA) was also assigned one of four grades. At MR imaging, areas of cerebral cortical or subcortical infarction in the hemisphere were divided into six zones. White matter and basal ganglionic infarction, ventricular dilatation, cortical atrophy, and hemorrhagic lesions were also evaluated. To demonstrate the statistical significance of the relationship between the angiographic and the MR findings, both the Mantel-Haenszel chi-square test for trend and the chi-square test were used. The degree of steno-occlusive PCA change correlated significantly with the internal carotid artery (ICA) stage (p<0.0001). As PCA stages advanced, the degree of leptomeningeal collaterals from the PCA decreased significantly (P<0.0001), but ICA stages were not significant (p>0.05). The prevalence of infarction showed significant correlation with the degree of steno-occlusive change in both the ICA and PCA. The degree of cerebral ischemia in moyamoya patients increased proportionally with the severity of PCA stenosis rather than with that of steno-occlusive lesins of the anterior circulation. Infarctions tended to be distributed in the anterior part of the hemisphere at PCA state I or II, while in more advanced PCA lesions, they were also found posteriorly, especially in the territories of the posterior middle cerebral artery

  1. An evaluation of the cerebral hemodynamics in moyamoya disease with acetazolamide (Diamox) 99mTc-HMPAO SPECT

    International Nuclear Information System (INIS)

    Qin-Yi, Gao; Kuwabara, Yasuo; Ichiya, Yuichi

    1994-01-01

    We studied the cerebral hemodynamics using Diamox 99m Tc-HMPAO SPECT in 9 patients with moyamoya disease, consisting of 4 children and 5 adults. Diamox 99m Tc-HMPO SPECT studies were performed by the double injection method. Hypoperfusion areas were scored by a visual inspection as follows: ++, definite positive; +, probable positive; -, negative. Diamox test was interpreted as positive when the score increased over one degree. In the resting state, 6 out of 9 patients showed definite or probable positive hypoperfusion, while all of 9 patients showed a similar finding after Diamox injection. The Diamox test was positive in 6 out of 9 patients. It was positive in all 3 patients who showed a negative hypoperfusion in the resting state. The regional cerebrovascular response to Diamox was most severely impaired in the frontal region. However, it was relatively well preserved in the cerebellum and thalamus according to the semiquantiative analysis. Thus, the Diamox HOPAO SPECT was considered to be useful in evaluating the hemodynamics in patients with moyamoya disease. (author)

  2. Prevention of the Rerupture of Collateral Artery Aneurysms on the Ventricular Wall by Early Surgical Revascularization in Moyamoya Disease: Report of Two Cases and Review of the Literature.

    Science.gov (United States)

    Kanamori, Fumiaki; Takasu, Syuntaro; Ota, Shinji; Seki, Yukio

    2018-01-01

    Collateral artery aneurysms are a source of intracranial hemorrhage in moyamoya disease. Several reports have shown that surgical revascularization leads to the obliteration of collateral artery aneurysms. However, its effect on the prevention of rebleeding has not been established, and the optimal timing of the operation remains unclear. The purpose of the present study is to evaluate the effects of surgical revascularization and to investigate the optimal operation timing in patients with moyamoya disease who have ruptured collateral artery aneurysms on the ventricular wall. Two patients with moyamoya disease who presented with intraventricular hemorrhage caused by rupture of collateral artery aneurysms on the wall of the lateral ventricle are presented here. In both cases, the aneurysms reruptured approximately 1 month after the initial hemorrhage. Both patients successfully underwent superficial temporal artery-middle cerebral artery anastomosis combined with indirect bypass in the subacute stage. The aneurysms decreased with the development of collateral circulation through the direct bypasses, and rebleeding did not occur after the surgery. Because ruptured collateral artery aneurysms on the wall of the lateral ventricle in moyamoya disease are prone to rerupture within 1 month, surgical revascularization may be recommended as soon as the patients are stable and able to withstand the operation. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study).

    Science.gov (United States)

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu; Nakamura, Fumiaki; Kamitani, Satoru; Nishimura, Ataru; Kurogi, Ryota; Sayama, Tetsuro; Iihara, Koji

    2016-03-15

    To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Retrospective, multicentre, non-randomised, observational study. Nationwide registry data in Japan. A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. We performed propensity score-matched analysis to examine the association between prehospital antiplatelet use and no significant disability on hospital admission, as defined by a modified Rankin Scale score of 0 or 1. Propensity-matched patients who received prehospital antiplatelet drugs were associated with a good outcome on hospital admission (OR adjusted for all covariates, 3.82; 95% CI 1.22 to 11.99) compared with those who did not receive antiplatelet drugs prior to hospital admission. Prehospital antiplatelet use was significantly associated with good functional status on hospital admission among patients with non-haemorrhagic moyamoya disease in Japan. Our results suggest that prehospital antiplatelet use should be considered when evaluating outcomes of patients with non-haemorrhagic moyamoya disease. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  4. Prehospital antiplatelet use and functional status on admission of patients with non-haemorrhagic moyamoya disease: a nationwide retrospective cohort study (J-ASPECT study)

    Science.gov (United States)

    Onozuka, Daisuke; Hagihara, Akihito; Nishimura, Kunihiro; Kada, Akiko; Nakagawara, Jyoji; Ogasawara, Kuniaki; Ono, Junichi; Shiokawa, Yoshiaki; Aruga, Toru; Miyachi, Shigeru; Nagata, Izumi; Toyoda, Kazunori; Matsuda, Shinya; Suzuki, Akifumi; Kataoka, Hiroharu; Nakamura, Fumiaki; Kamitani, Satoru; Nishimura, Ataru; Kurogi, Ryota; Sayama, Tetsuro; Iihara, Koji

    2016-01-01

    Objectives To elucidate the association between antiplatelet use in patients with non-haemorrhagic moyamoya disease before hospital admission and good functional status on admission in Japan. Design Retrospective, multicentre, non-randomised, observational study. Setting Nationwide registry data in Japan. Participants A total of 1925 patients with non-haemorrhagic moyamoya disease admitted between 1 April 2012 and 31 March 2014 in Japan. Main outcome measure We performed propensity score-matched analysis to examine the association between prehospital antiplatelet use and no significant disability on hospital admission, as defined by a modified Rankin Scale score of 0 or 1. Results Propensity-matched patients who received prehospital antiplatelet drugs were associated with a good outcome on hospital admission (OR adjusted for all covariates, 3.82; 95% CI 1.22 to 11.99) compared with those who did not receive antiplatelet drugs prior to hospital admission. Conclusions Prehospital antiplatelet use was significantly associated with good functional status on hospital admission among patients with non-haemorrhagic moyamoya disease in Japan. Our results suggest that prehospital antiplatelet use should be considered when evaluating outcomes of patients with non-haemorrhagic moyamoya disease. PMID:27008684

  5. Significant association of RNF213 p.R4810K, a moyamoya susceptibility variant, with coronary artery disease.

    Science.gov (United States)

    Morimoto, Takaaki; Mineharu, Yohei; Ono, Koh; Nakatochi, Masahiro; Ichihara, Sahoko; Kabata, Risako; Takagi, Yasushi; Cao, Yang; Zhao, Lanying; Kobayashi, Hatasu; Harada, Kouji H; Takenaka, Katsunobu; Funaki, Takeshi; Yokota, Mitsuhiro; Matsubara, Tatsuaki; Yamamoto, Ken; Izawa, Hideo; Kimura, Takeshi; Miyamoto, Susumu; Koizumi, Akio

    2017-01-01

    The genetic architecture of coronary artery disease has not been fully elucidated, especially in Asian countries. Moyamoya disease is a progressive cerebrovascular disease that is reported to be complicated by coronary artery disease. Because most Japanese patients with moyamoya disease carry the p.R4810K variant of the ring finger 213 gene (RNF213), this may also be a risk factor for coronary artery disease; however, this possibility has never been tested. We genotyped the RNF213 p.R4810K variant in 956 coronary artery disease patients and 716 controls and tested the association between p.R4810K and coronary artery disease. We also validated the association in an independent population of 311 coronary artery disease patients and 494 controls. In the replication study, the p.R4810K genotypes were imputed from genome-wide genotyping data based on the 1000 Genomes Project. We used multivariate logistic regression analyses to adjust for well-known risk factors such as dyslipidemia and smoking habits. In the primary study population, the frequency of the minor variant allele was significantly higher in patients with coronary artery disease than in controls (2.04% vs. 0.98%), with an odds ratio of 2.11 (p = 0.017). Under a dominant model, after adjustment for risk factors, the association remained significant, with an odds ratio of 2.90 (95% confidence interval: 1.37-6.61; p = 0.005). In the replication study, the association was significant after adjustment for age and sex (odds ratio = 4.99; 95% confidence interval: 1.16-21.53; p = 0.031), although it did not reach statistical significance when further adjusted for risk factors (odds ratio = 3.82; 95% confidence interval: 0.87-16.77; p = 0.076). The RNF213 p.R4810K variant appears to be significantly associated with coronary artery disease in the Japanese population.

  6. Biomarker research for moyamoya disease in cerebrospinal fluid using surface-enhanced laser desorption/ionization time-of-flight mass spectrometry.

    Science.gov (United States)

    Maruwaka, Mikio; Yoshikawa, Kazuhiro; Okamoto, Sho; Araki, Yoshio; Sumitomo, Masaki; Kawamura, Akino; Yokoyama, Kinya; Wakabayashi, Toshihiko

    2015-01-01

    Moyamoya disease (MMD) is a rare cerebrovascular disease characterized by steno-occlusive change in bilateral internal carotid arteries with unknown etiology. To discover biomarker candidates in cerebrospinal fluid from MMD patients, proteome analysis was performed by the surface-enhanced laser desorption/ionization time-of-flight mass spectrometry. Three peptides, 4473Da, 4475Da, and 6253Da, were significantly elevated in MMD group. A positive correlation between 4473Da peptide and postoperative angiogenesis was determined. Twenty MMD patients were enrolled in this pilot study, including 11 pediatric cases less than 18 years of age (mean age, 8.67 years) and 9 adult MMD patients (mean age, 38.1 years). This study also includes 17 control cases with the mean age of 27.9 years old. In conclusion, 4473Da peptide is supposed to be a reliable biomarker of MMD. 4473Da peptide showed higher intensity peaks especially in younger MMD patients, and it was proved to be highly related to postoperative angiogenesis. Further study is needed to show how 4473Da peptide is involved with the etiology and the onset of MMD. Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  7. De novo appearance of primitive neuroectodermal tumor in a patient with systemic lupus erythematosus and moyamoya disease.

    Science.gov (United States)

    Park, D J; Kim, T J; Lee, H J; Lee, K E; Lee, S J; Seo, S R; Yoon, W; Moon, K S; Lee, K W; Lee, S S; Park, Y W

    2010-07-01

    Primitive neuroectodermal tumor is a rare brain tumor composed of undifferentiated or poorly differentiated neuroepithelial cells with a high malignant potential that usually occurs in children, and which is only occasionally encountered in adults. A 19-year-old female with systemic lupus erythematosus presented with right hemiparesis and a headache of 10 days duration. Brain magnetic resonance imaging showed a large solid mass with necrotic portions in the left frontoparietal lobe. Primitive neuroectodermal tumor was confirmed by a neuronavigator-guided brain biopsy. This is the first case report of primitive neuroectodermal tumor associated with systemic lupus erythematosus and moyamoya disease. This case demonstrates that brain tumors, such as primitive neuroectodermal tumor, should be included in the differential diagnosis of neurological manifestations in children and adolescent patients with systemic lupus erythematosus.

  8. Regional cerebral blood flow of Moyamoya disease in the chronic state as studied using a three-dimensional method. With special reference to CO/sub 2/ response and autoregulation

    Energy Technology Data Exchange (ETDEWEB)

    Ejiri, Takao; Endo, Hideo; Kubo, Naohiko; Kanaya, Haruyuki

    1986-02-01

    Regional cerebral blood flow (rCBF) was measured in six adult and five juvenile cases with moyamoya disease, using single-photon-emission CT and the Xe-133 inhalation method. In the resting state, the rCBF value in the basal moyamoya region was within normal limits. The characteristic of rCBF was a decrease in the cerebral cortex, especially in the temporal region of the juvenile cases. CO/sub 2/ response by 5 % CO/sub 2/ inhalation was disturbed not only in the cerebral cortex, but also in the basal moyamoya region; also the degree of such impairment showed site-to-site differences. On the other hand, rCBF changes under hyperventilation showed a good response in the cerebral cortex, but was mostly absent in the basal moyamoya region. Autoregulation under induced hypertension seemed to be preserved both in the cerebral cortex and in the basal moyamoya region. Under induced hypotension, however, an impairment of autoregulation was shown both in the cerebral cortex and in the basal moyamoya region. The rCBF value in the cerebellum was slightly higher than that in the cerebrum. The vascular response of the cerebellum was not preserved under hypercapnia and hypotension; however, it showed almost normal behavior under hypocapnia and hypertension. In summary, it might be concluded that the impairment of vascular response in the basal moyamoya region during various functional tests was not due to the secondary effects following the decrease in the rCBF. It may also be speculated that these impairments of vascular response may be directly attributed to various pathological alterations in the arterial vascular wall, i.e., the maximal dilatation and rigidity of the vessels in the moyamoya region, as evidenced in the previous reports.

  9. Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso Enfermedad de Moyamoya y anestesia con sevoflurano fuera del centro quirúrgico: relato de caso Moyamoya disease and sevoflurane anesthesia outside the surgery center: case report

    Directory of Open Access Journals (Sweden)

    Sheila Braga Machado

    2002-06-01

    causa de accidente vascular cerebral en individuos jóvenes. El objetivo de este relato es presentar el caso de un paciente con enfermedad de Moyamoya que fue sometido a anestesia general con sevoflurano para procedimiento diagnóstico fuera del centro quirúrgico. RELATO DEL CASO: Niño con 13 años, estado físico ASA IV, portador de enfermedad de Moyamoya con secuela neurológica después de tres accidentes vasculares cerebrales, insuficiencia renal crónica e hipertensión arterial sistémica, sometido a endoscopia digestiva alta. En decúbito dorsal y después monitorización, se realizó inducción inalatoria por la cánula de traqueostomía con sevoflurano (aumento gradual de la concentración inspiratoria hasta 6% y mezcla de oxígeno/óxido nitroso a 50%. Un catéter venoso fue introducido para infusión de solución glucosada a 5%. Fue realizada ventilación controlada manual, siendo la manutención de la anestesia hecha con sevoflurano a 4% y mezcla de oxígeno/óxido nitroso a 50%. Al final del procedimiento los agentes anestésicos fueron descontinuados simultáneamente y fue administrado oxígeno a 100%. La anestesia fue satisfactoria, con buena estabilidad hemodinámica, sin ocurrencia de complicaciones durante el procedimiento y con un precoz despertar. CONCLUSIONES: El sevoflurano puede ofrecer nuevas perspectivas para la anestesia inhalatoria en pacientes con enfermedad neurológica que realizan procedimiento ambulatorial, ya que permite buena estabilidad hemodinamica y despertar precoz, preservando la fisiología cerebral.BACKGROUND AND OBJECTIVES: Moyamoya disease is a progressive cerebrovascular disorder implying anesthetic challenges due to patients’ poor brain perfusion, in addition to being a major cause for stroke in young people. This report aimed at describing a case of Moyamoya’s disease in a patient submitted to general anesthesia with sevoflurane for a diagnostic procedure outside the surgery center. CASE REPORT: Male child, 13 years

  10. What Is a Pediatric Infectious Diseases Specialist?

    Science.gov (United States)

    ... Text Size Email Print Share What is a Pediatric Infectious Diseases Specialist? Page Content Article Body If ... the teen years. What Kind of Training Do Pediatric Infectious Diseases Specialists Have? Pediatric infectious diseases specialists ...

  11. The Role of RNF213 4810G>A and 4950G>A Variants in Patients with Moyamoya Disease in Korea

    Directory of Open Access Journals (Sweden)

    Young Seok Park

    2017-11-01

    Full Text Available Although a founder variant of RNF213 4810G>A is a major genetic risk factor for moyamoya disease (MMD in East Asians, the frequency and disease susceptibility of RNF213 variants remain largely unknown. This study investigated the mutation analysis of RNF213 (4448, 4810, 4863, and 4950 between Korean MMD and healthy controls. We performed a polymerase chain reaction-restriction fragment length polymorphism analysis. To identify the association between RNF213 gene polymorphisms and MMD disease, we performed statistical analyses such as multivariable logistic regression and Fisher’s exact test. Genetic data from 117 MMD patients were analyzed and compared with 253 healthy controls. We assessed and compared single nucleotide polymorphisms of RNF213 (4448, 4810, 4863, and 4950 between MMD and control groups. We performed genome-wide association studies to investigate the genetic pathophysiology of MMD. Among the RNF213 variants (4448G>A, 4810G>A, 4863G>A, and 4950G>A, RNF213 4810G>A and 4950G>A variants were more frequent in MMD patients. In a subgroup analysis, the RNF213 4810G>A was more frequent in moyamoya disease, and the comparison with GG+AA genotype was also significantly different in moyamoya patients. These results confirm that RNF213 4810G>A and RNF213 4950G>A were more frequent in MMD patients. We have confirmed that RNF213 4810G>A and 4950G>A are strongly associated with Korean MMD in children and adults as well as for the ischemic and hemorrhagic types.

  12. Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease: Correlation with Clinical Severity and Old Brain Lesions.

    Science.gov (United States)

    Seo, Kwon-Duk; Suh, Sang Hyun; Kim, Yong Bae; Kim, Ji Hwa; Ahn, Sung Jun; Kim, Dong-Seok; Lee, Kyung-Yul

    2015-09-01

    Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance of ivy signs and whether they correlate with old brain lesions and the severity of clinical symptoms in patients with MMD. FLAIR images of 83 patients were reviewed. Each cerebral hemisphere was divided into 4 regions and each region was scored based on the prominence of the ivy sign. Total ivy score (TIS) was defined as the sum of the scores from the eight regions and dominant hemispheric ivy sign (DHI) was determined by comparing the ivy scores from each hemisphere. According to the degree of ischemic symptoms, patients were classified into four subgroups: 1) nonspecific symptoms without motor weakness, 2) single transient ischemic attack (TIA), 3) recurrent TIA, or 4) complete stroke. TIS was significantly different as follows: 4.86±2.55 in patients with nonspecific symptoms, 5.89±3.10 in patients with single TIA, 9.60±3.98 in patients with recurrent TIA and 8.37±3.39 in patients with complete stroke (p=0.003). TIS associated with old lesions was significantly higher than those not associated with old lesions (9.35±4.22 vs. 7.49±3.37, p=0.032). We found a significant correlation between DHI and motor symptoms (p=0.001). Because TIS has a strong tendency with severity of ischemic motor symptom and the presence of old lesions, the ivy sign may be useful in predicting severity of disease progression.

  13. Role of CT perfusion imaging in evaluating the effects of multiple burr hole surgery on adult ischemic Moyamoya disease

    Energy Technology Data Exchange (ETDEWEB)

    Dai, Dong Wei; Zhao, Wen Yuan; Yang, Zhi Gang; Li, Qiang; Liu, Jian Min [Second Military Medical University, Department of Neurosurgery, Changhai Hospital, Shanghai (China); Zhang, Yong Wei [Second Military Medical University, Department of Neurology, Changhai Hospital, Shanghai (China); Xu, Bing; Ma, Xiao Long; Tian, Bing [Second Military Medical University, Department of Radiology, Changhai Hospital, Shanghai (China)

    2013-12-15

    To evaluate the effects of the multiple burr hole (MBH) revascularization on ischemic type adult Moyamoya disease (MMD) by computed tomography perfusion (CTP). Eighty-six ischemic MMD patients received CTP 1 week before and 3 weeks after MBH operation. Fifty-seven patients received it again at 6 month and underwent digital subtraction angiography (DSA) and mRS follow-up. Cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), time to peak (TTP), and relative values of ischemic symptomatic hemispheres were measured. Differences in pre- and post-surgery perfusion CT values were assessed. There were significant differences of CBF, TTP, and relative time to peak (rTTP) in ischemic hemisphere between 1 week before and 3 weeks after surgery, and no significant difference in relative cerebral blood flow (rCBF), CBV, relative cerebral blood volume (rCBV), MTT, relative mean transit time (rMTT). According to whether there was symptom improvement or not on 3 weeks after MBH, the rTTP value was not statistically significant in the patients whose symptoms were not improved at all on 3 weeks after operation. Six-month follow-up showed that CBF, rCBF, and rCBV values were significantly higher than those before operation. Postoperative MTT, TTP, rMTT, and rTTP values were significantly lower than those before operation. CTP is a sensitive method to obtain functional imaging of cerebral microcirculation, which can be a noninvasive assessment of the abnormalities of intracranial arteries and cerebral perfusion changes in MMD before and after surgery. CBF and TTP map, especially the relative values of TTP, seems to have the capability of being quite sensitive to the presence of altered brain perfusion at early time after indirect revascularization. (orig.)

  14. Association between Champagne Bottle Neck Sign of Internal Carotid Artery and Ipsilateral Hemorrhagic Stroke in Patients with Moyamoya Disease.

    Science.gov (United States)

    Wang, Jian; Chen, Gong; Yang, Yongbo; Zhang, Bing; Jia, Zhongzhi; Gu, Peiyuan; Wei, Dong; Ji, Jing; Hu, Weixing; Zhao, Xihai

    2018-06-15

    To assess the association between champagne bottle neck sign (CBNS) in carotid artery and intracranial hemorrhage in patients with moyamoya disease (MMD). From January 2016 to December 2017, a total of 76 consecutive patients with MMD without definite risk factors associated intracranial hemorrhage who underwent preoperative angiography were included in this retrospective study. CBNS was defined as luminal diameter of internal carotid artery (ICA)/common carotid artery (CCA) ≤ 0.5 on angiographic imaging. The right and left cerebral hemisphere in each patient was separately identified as hemorrhagic and none-hemorrhagic. The association between CBNS and intracranial hemorrhage was analyzed. Of 76 MMD patients, intracranial hemorrhage was found in 44 (28.9%) hemispheres of 152 and 6.8% (3/44) had multiple events. Compared carotid arteries without intracranial hemorrhage in the ipsilateral hemispheres, those with intracranial hemorrhage in the ipsilateral hemispheres had significantly smaller luminal diameter ratio of ICA/CCA (0.49 ± 0.11 vs. 0.55 ± 0.12, p < 0.01) and higher prevalence of CBNS (63.7% vs. 41.7%, p = 0.01). For hemispheres with intracranial hemorrhage, those with ipsilateral carotid artery CBNS had significantly higher prevalence of hemorrhage at posterior territories than those without (57.1% vs. 23.1%, p=0.05). Logistic regression revealed that CBNS was significantly associated with ipsilateral intracranial hemorrhage before (OR, 2.45; 95% CI, 1.19-5.05; p=0.02) and after (OR, 3.43; 95% CI, 1.50-7.87; p<0.01) adjusted for female, lenticulostriate anastomosis, and choroidal anastomosis. CBNS is significantly associated with intracranial hemorrhage at ipsilateral hemisphere in MMD patients, particularly for intracranial hemorrhage at posterior territories. Copyright © 2018. Published by Elsevier Inc.

  15. Chronologic Evaluation of Cerebral Hemodynamics by Dynamic Susceptibility Contrast Magnetic Resonance Imaging After Indirect Bypass Surgery for Moyamoya Disease.

    Science.gov (United States)

    Ishii, Yosuke; Tanaka, Yoji; Momose, Toshiya; Yamashina, Motoshige; Sato, Akihito; Wakabayashi, Shinichi; Maehara, Taketoshi; Nariai, Tadashi

    2017-12-01

    Although indirect bypass surgery is an effective treatment option for patients with ischemic-onset moyamoya disease (MMD), the time point after surgery at which the patient's hemodynamic status starts to improve and the time point at which the improvement reaches a maximum have not been known. The objective of the present study is to evaluate the hemodynamic status time course after indirect bypass surgery for MMD, using dynamic susceptibility contrast-magnetic resonance imaging (DSC-MRI). We retrospectively analyzed the cases of 25 patients with MMD (37 sides; mean age, 14.7 years; range, 3-36 years) who underwent indirect bypass surgery and repeated DSC-MRI measurement within 6 months after the operation. The difference in the mean transit time (MTT) between the target regions and the control region (cerebellum) was termed the MTT delay, and we measured the MTT delay's chronologic changes after surgery. The postoperative MTT delay was 1.81 ± 1.16 seconds within 1 week after surgery, 1.57 ± 1.01 at weeks 1-2, 1.55 ± 0.68 at weeks 2-4, 1.32 ± 0.68 at months 1-2, 0.95 ± 0.32 at months 2-3, and 0.77 ± 0.33 at months 3-6. Compared with the preoperative value (2.11 ± 0.98 seconds), the MTT delay decreased significantly from 2 to 4 weeks after surgery (P surgery began soon after surgery and gradually reached a maximum at 3 months after surgery. DSC-MRI detected small changes in hemodynamic improvement, which are suspected to be caused by the initiation of angiogenesis and arteriogenesis in the early postoperative period. Copyright © 2017. Published by Elsevier Inc.

  16. Mechanism of the re-buildup phenomenon in moyamoya disease; Analysis of local cerebral hemodynamics with intra-arterial digital subtraction angiography

    Energy Technology Data Exchange (ETDEWEB)

    Touho, Hajime; Karasawa, Jun; Shishido, Hisashi; Morisako, Toshitaka; Yamada, Keisuke; Nagai, Shigeki; Shibamoto, Kenji [Osaka Neurological Institute, Osaka (Japan)

    1990-10-01

    The authors investigated the mechanism of the re-buildup phenomenon on electroencephalogram in 14 patients of moyamoya disease with superficial temporal artery-middle cerebral artery anastomosis. Visualization of the lateral view of the common carotid angiography was performed with intra-arterial digital subtraction angiography (IA-DSA), using a 4/sec x 3 sec + 2/sec x 5 sec + 1/sec x 5 sec film sequence. The catheter tip was inserted into C5/6 level and 250 mgl/ml of iopamidol was used as the contrast agent; 6 ml in total was injected over 1.5 seconds. Circulation times of the common carotid artery (C{sub 3} portion)-ascending parietal vein ({delta}TTP{sub s}) and common carotid artery-internal cerebral vein ({delta}TTP{sub D}) were measured before hyperventilation (HV), immediately after HV, and 3 minutes after HV during pre- and postoperative periods. {delta}TTP{sub D} in the preoperative period was prolonged by HV and was normalized at 3 minutes after HV but {delta}TTP{sub S} were prolonged immediately after and 3 minutes after HV. In the postoperative period, however, these values did not change significantly immediately after and 3 minutes after HV. These findings indicate that delayed cerebral blood flow response to HV is a pathogenetic factor of the re-buildup phenomenon in moyamoya disease. (author).

  17. The moyamoya disease susceptibility variant RNF213 R4810K (rs112735431) induces genomic instability by mitotic abnormality

    Energy Technology Data Exchange (ETDEWEB)

    Hitomi, Toshiaki [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Habu, Toshiyuki [Radiation Biology Center, Kyoto University, Kyoto (Japan); Kobayashi, Hatasu; Okuda, Hiroko; Harada, Kouji H. [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Osafune, Kenji [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Taura, Daisuke; Sone, Masakatsu [Department of Medicine and Clinical Science, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Asaka, Isao; Ameku, Tomonaga; Watanabe, Akira; Kasahara, Tomoko; Sudo, Tomomi; Shiota, Fumihiko [Center for iPS Cell Research and Application (CiRA), Kyoto University, Kyoto (Japan); Hashikata, Hirokuni; Takagi, Yasushi [Department of Neurosurgery, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Morito, Daisuke [Faculty of Life Sciences, Kyoto Sangyo University, Kyoto (Japan); Miyamoto, Susumu [Department of Neurosurgery, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Nakao, Kazuwa [Department of Medicine and Clinical Science, Graduate School of Medicine, Kyoto University, Kyoto (Japan); Koizumi, Akio, E-mail: koizumi.akio.5v@kyoto-u.ac.jp [Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University, Kyoto (Japan)

    2013-10-04

    Highlights: •Overexpression of RNF213 R4810K inhibited cell proliferation. •Overexpression of RNF213 R4810K had the time of mitosis 4-fold and mitotic failure. •R4810K formed a complex with MAD2 more readily than wild-type. •iPSECs from the MMD patients had elevated mitotic failure compared from the control. •RNF213 R4810K induced mitotic abnormality and increased risk of aneuploidy. -- Abstract: Moyamoya disease (MMD) is a cerebrovascular disease characterized by occlusive lesions in the Circle of Willis. The RNF213 R4810K polymorphism increases susceptibility to MMD. In the present study, we characterized phenotypes caused by overexpression of RNF213 wild type and R4810K variant in the cell cycle to investigate the mechanism of proliferation inhibition. Overexpression of RNF213 R4810K in HeLa cells inhibited cell proliferation and extended the time of mitosis 4-fold. Ablation of spindle checkpoint by depletion of mitotic arrest deficiency 2 (MAD2) did not shorten the time of mitosis. Mitotic morphology in HeLa cells revealed that MAD2 colocalized with RNF213 R4810K. Immunoprecipitation revealed an RNF213/MAD2 complex: R4810K formed a complex with MAD2 more readily than RNF213 wild-type. Desynchronized localization of MAD2 was observed more frequently during mitosis in fibroblasts from patients (n = 3, 61.0 ± 8.2%) compared with wild-type subjects (n = 6, 13.1 ± 7.7%; p < 0.01). Aneuploidy was observed more frequently in fibroblasts (p < 0.01) and induced pluripotent stem cells (iPSCs) (p < 0.03) from patients than from wild-type subjects. Vascular endothelial cells differentiated from iPSCs (iPSECs) of patients and an unaffected carrier had a longer time from prometaphase to metaphase than those from controls (p < 0.05). iPSECs from the patients and unaffected carrier had significantly increased mitotic failure rates compared with controls (p < 0.05). Thus, RNF213 R4810K induced mitotic abnormalities and increased risk of genomic instability.

  18. The moyamoya disease susceptibility variant RNF213 R4810K (rs112735431) induces genomic instability by mitotic abnormality

    International Nuclear Information System (INIS)

    Hitomi, Toshiaki; Habu, Toshiyuki; Kobayashi, Hatasu; Okuda, Hiroko; Harada, Kouji H.; Osafune, Kenji; Taura, Daisuke; Sone, Masakatsu; Asaka, Isao; Ameku, Tomonaga; Watanabe, Akira; Kasahara, Tomoko; Sudo, Tomomi; Shiota, Fumihiko; Hashikata, Hirokuni; Takagi, Yasushi; Morito, Daisuke; Miyamoto, Susumu; Nakao, Kazuwa; Koizumi, Akio

    2013-01-01

    Highlights: •Overexpression of RNF213 R4810K inhibited cell proliferation. •Overexpression of RNF213 R4810K had the time of mitosis 4-fold and mitotic failure. •R4810K formed a complex with MAD2 more readily than wild-type. •iPSECs from the MMD patients had elevated mitotic failure compared from the control. •RNF213 R4810K induced mitotic abnormality and increased risk of aneuploidy. -- Abstract: Moyamoya disease (MMD) is a cerebrovascular disease characterized by occlusive lesions in the Circle of Willis. The RNF213 R4810K polymorphism increases susceptibility to MMD. In the present study, we characterized phenotypes caused by overexpression of RNF213 wild type and R4810K variant in the cell cycle to investigate the mechanism of proliferation inhibition. Overexpression of RNF213 R4810K in HeLa cells inhibited cell proliferation and extended the time of mitosis 4-fold. Ablation of spindle checkpoint by depletion of mitotic arrest deficiency 2 (MAD2) did not shorten the time of mitosis. Mitotic morphology in HeLa cells revealed that MAD2 colocalized with RNF213 R4810K. Immunoprecipitation revealed an RNF213/MAD2 complex: R4810K formed a complex with MAD2 more readily than RNF213 wild-type. Desynchronized localization of MAD2 was observed more frequently during mitosis in fibroblasts from patients (n = 3, 61.0 ± 8.2%) compared with wild-type subjects (n = 6, 13.1 ± 7.7%; p < 0.01). Aneuploidy was observed more frequently in fibroblasts (p < 0.01) and induced pluripotent stem cells (iPSCs) (p < 0.03) from patients than from wild-type subjects. Vascular endothelial cells differentiated from iPSCs (iPSECs) of patients and an unaffected carrier had a longer time from prometaphase to metaphase than those from controls (p < 0.05). iPSECs from the patients and unaffected carrier had significantly increased mitotic failure rates compared with controls (p < 0.05). Thus, RNF213 R4810K induced mitotic abnormalities and increased risk of genomic instability

  19. Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso Enfermedad de Moyamoya y anestesia con sevoflurano fuera del centro quirúrgico: relato de caso Moyamoya disease and sevoflurane anesthesia outside the surgery center: case report

    OpenAIRE

    Sheila Braga Machado; Florentino Fernandes Mendes; Adriana de Campos Angelini

    2002-01-01

    JUSTIFICATIVA E OBJETIVOS: A doença de Moyamoya é uma desordem cerebrovascular progressiva que representa um desafio anestésico em virtude da precária circulação cerebral destes pacientes, constituindo-se numa importante causa de acidente vascular cerebral em indivíduos jovens. O objetivo deste relato é apresentar o caso de um paciente com doença de Moyamoya que foi submetido à anestesia geral com sevoflurano para procedimento diagnóstico fora do centro cirúrgico. RELATO DO CASO: Criança com ...

  20. Pediatric Inflammatory Bowel Diseases

    DEFF Research Database (Denmark)

    Lauritzen, Didde; Andreassen, Bente Utoft; Heegaard, Niels Henrik H

    2018-01-01

    Background: Kidney disease has been reported in adults with inflammatory bowel disease (IBD) and is regarded an extraintestinal manifestation or more rarely a side effect of the medical treatment. Methods: In this cross-sectional study we describe the extent of kidney pathology in a cohort of 56...... children with IBD. Blood and urine samples were analyzed for markers of kidney disease and ultrasonography was performed to evaluate pole-to-pole kidney length. Results: We found that 25% of the patients had either previously reported kidney disease or ultrasonographic signs of chronic kidney disease...... are at risk of chronic kidney disease, and the risk seems to be increased with the severity of the disease....

  1. Pediatric GERD (Gastro-Esophageal Reflux Disease)

    Science.gov (United States)

    ... Marketplace Find an ENT Doctor Near You Pediatric GERD (Gastro-Esophageal Reflux Disease) Pediatric GERD (Gastro-Esophageal ... What symptoms are displayed by a child with GERD? While GER and EER in children often cause ...

  2. The usefulness of the ivy sign on fluid-attenuated intensity recovery images in improved brain hemodynamic changes after superficial temporal artery-middle cerebral artery anastomosis in adult patients with moyamoya disease.

    Science.gov (United States)

    Lee, Jung Keun; Yoon, Byul Hee; Chung, Seung Young; Park, Moon Sun; Kim, Seong Min; Lee, Do Sung

    2013-10-01

    MR perfusion and single photon emission computerized tomography (SPECT) are well known imaging studies to evaluate hemodynamic change between prior to and following superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis in moyamoya disease. But their side effects and invasiveness make discomfort to patients. We evaluated the ivy sign on MR fluid attenuated inversion recovery (FLAIR) images in adult patients with moyamoya disease and compared it with result of SPECT and MR perfusion images. We enrolled twelve patients (thirteen cases) who were diagnosed with moyamoya disease and underwent STA-MCA anastomosis at our medical institution during a period ranging from September of 2010 to December of 2012. The presence of the ivy sign on MR FLAIR images was classified as Negative (0), Minimal (1), and Positive (2). Regions were classified into four territories: the anterior cerebral artery (ACA), the anterior MCA, the posterior MCA and the posterior cerebral artery. Ivy signs on preoperative and postoperative MR FLAIR were improved (8 and 4 in the ACA regions, 13 and 4 in the anterior MCA regions and 19 and 9 in the posterior MCA regions). Like this result, the cerebrovascular reserve (CVR) on SPECT was significantly increased in the sum of CVR in same regions after STA-MCA anastomosis. After STA-MCA anastomosis, ivy signs were decreased in the cerebral hemisphere. As compared with conventional diagnostic modalities such as SPECT and MR perfusion images, the ivy sign on MR FLAIR is considered as a useful indicator in detecting brain hemodynamic changes between preoperatively and postoperatively in adult moyamoya patients.

  3. Down syndrome and moyamoya: clinical presentation and surgical management.

    Science.gov (United States)

    See, Alfred P; Ropper, Alexander E; Underberg, Daniel L; Robertson, Richard L; Scott, R Michael; Smith, Edward R

    2015-07-01

    OBJECT Moyamoya can cause cerebral ischemia and stroke in Down syndrome (DS) patients. In this study, the authors defined a surgically treated population of patients with DS and moyamoya and compared their clinical presentation, response to surgical treatment, and long-term prognosis with those of the general population of patients with moyamoya but without DS. METHODS This study was a retrospective review of a consecutive operative series of moyamoya patients with DS treated at Boston Children's Hospital from 1985 through 2012. RESULTS Thirty-two patients, average age 9.7 years (range 1.8-29.3 years), underwent surgery for moyamoya in association with DS. The majority presented with ischemic symptoms (87% stroke, 42% transient ischemic attacks). Twenty-four patients (75%) had congenital heart disease. Nineteen patients (59%) had bilateral moyamoya on presentation, and 13 presented with unilateral disease, of which 2 progressed to surgery on the opposite side at a later date. Patients were followed for a median of 7.5 years (1-20.2 years) after surgery, with no patients lost to follow-up. Follow-up arteriography demonstrated Matsushima Grade A collaterals in 29 of 39 (74%) hemispheres, Grade B in 5 (13%), and Grade C in 5 (13%). Complications included postoperative strokes in 2 patients, which occurred within 48 hours of surgery in both; one of these patients had arm weakness and the other confusion (both had recovered completely at follow-up). Seizures occurred in 5 patients perioperatively, including one who had a new seizure disorder related to hypocalcemia. CONCLUSIONS Moyamoya disease is a cause of stroke in patients with DS. Both the incidence of preoperative stroke (87% vs 67%) and the average age at diagnosis for children under age 21 (8.4 vs 6.5 years) were greater in patients with DS and moyamoya than in the general moyamoya surgical population, suggesting a possible delay in reaching a correct diagnosis of the cause of cerebral ischemia in the DS patient

  4. Disease phenotype at diagnosis in pediatric Crohn's disease

    DEFF Research Database (Denmark)

    de Bie, Charlotte I; Paerregaard, Anders; Kolacek, Sanja

    2013-01-01

    It has been speculated that pediatric Crohn's disease (CD) is a distinct disease entity, with probably different disease subtypes. We therefore aimed to accurately phenotype newly diagnosed pediatric CD by using the pediatric modification of the Montreal classification, the Paris classification....

  5. Atypical disease phenotypes in pediatric ulcerative colitis

    DEFF Research Database (Denmark)

    Levine, Arie; de Bie, Charlotte I; Turner, Dan

    2013-01-01

    Definitive diagnosis of pediatric ulcerative colitis (UC) may be particularly challenging since isolated colitis with overlapping features is common in pediatric Crohn's disease (CD), while atypical phenotypes of UC are not uncommon. The Paris classification allows more accurate phenotyping...... of atypical inflammatory bowel disease (IBD) patients. Our aim was to identify the prevalence of atypical disease patterns in new-onset pediatric UC using the Paris classification....

  6. Clostridium difficile and pediatric inflammatory bowel disease

    DEFF Research Database (Denmark)

    Martinelli, Massimo; Strisciuglio, Caterina; Veres, Gabor

    2014-01-01

    BACKGROUND: Clostridium difficile infection is associated with pediatric inflammatory bowel disease (IBD) in several ways. We sought to investigate C. difficile infection in pediatric patients with IBD in comparison with a group of children with celiac disease and to evaluate IBD disease course o...

  7. "STA-MCA bypass with encephalo-duro-myo-synangiosis combined with bifrontal encephalo-duro-periosteal-synangiosis" as a one-staged revascularization strategy for pediatric moyamoya vasculopathy

    NARCIS (Netherlands)

    Esposito, G.; Kronenburg, A.; Fierstra, J.; Braun, K.P.; Klijn, C.J.M.; Zwan, A. van der; Regli, L.

    2015-01-01

    PURPOSE: Moyamoya vasculopathy progressively compromises cerebral blood flow resulting in chronic hypoperfusion. The middle cerebral artery (MCA) territory and the bifrontal areas are the regions most frequently affected. Although most techniques aim to only revascularize the MCA territory,

  8. [Periodontal disease in pediatric rheumatic diseases].

    Science.gov (United States)

    Fabri, Gisele M C; Savioli, Cynthia; Siqueira, José T; Campos, Lucia M; Bonfá, Eloisa; Silva, Clovis A

    2014-01-01

    Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding. In JIA patients, gingivitis and periodontitis were related to mechanical factors, chronic arthritis with functional disability, dysregulation of the immunoinflammatory response, diet and drugs, mainly corticosteroids and cyclosporine. In C-SLE, gingivitis was associated with longer disease period, high doses of corticosteroids, B-cell hyperactivation and immunoglobulin G elevation. There are scarce data on periodontal diseases in JDM population, and a unique gingival pattern, characterized by gingival erythema, capillary dilation and bush-loop formation, was observed in active patients. In conclusion, gingivitis was the most common periodontal disease in PRD. The observed association with disease activity reinforces the need for future studies to determine if resolution of this complication will influence disease course or severity. Copyright © 2014 Elsevier Editora Ltda. All rights reserved.

  9. Management of moyamoya syndrome in patients with Noonan syndrome.

    Science.gov (United States)

    Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

    2016-06-01

    A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

  10. Antibiotics, pediatric dysbiosis, and disease.

    Science.gov (United States)

    Vangay, Pajau; Ward, Tonya; Gerber, Jeffrey S; Knights, Dan

    2015-05-13

    Antibiotics are by far the most common medications prescribed for children. Recent epidemiological data suggests an association between early antibiotic use and disease phenotypes in adulthood. Antibiotic use during infancy induces imbalances in gut microbiota, called dysbiosis. The gut microbiome's responses to antibiotics and its potential link to disease development are especially complex to study in the changing infant gut. Here, we synthesize current knowledge linking antibiotics, dysbiosis, and disease and propose a framework for studying antibiotic-related dysbiosis in children. We recommend future studies into the microbiome-mediated effects of antibiotics focused on four types of dysbiosis: loss of keystone taxa, loss of diversity, shifts in metabolic capacity, and blooms of pathogens. Establishment of a large and diverse baseline cohort to define healthy infant microbiome development is essential to advancing diagnosis, interpretation, and eventual treatment of pediatric dysbiosis. This approach will also help provide evidence-based recommendations for antibiotic usage in infancy. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Brain Stem Infarction Due to Basilar Artery Dissection in a Patient with Moyamoya Disease Four Years after Successful Bilateral Revascularization Surgeries.

    Science.gov (United States)

    Abe, Takatsugu; Fujimura, Miki; Mugikura, Shunji; Endo, Hidenori; Tominaga, Teiji

    2016-06-01

    Moyamoya disease (MMD) is a rare cerebrovascular disease with an unknown etiology and is characterized by intrinsic fragility in the intracranial vascular walls such as the affected internal elastic lamina and thinning medial layer. The association of MMD with intracranial arterial dissection is extremely rare, whereas that with basilar artery dissection (BAD) has not been reported previously. A 46-year-old woman developed brain stem infarction due to BAD 4 years after successful bilateral superficial temporal artery-middle cerebral artery anastomosis with indirect pial synangiosis for ischemic-onset MMD. She presented with sudden occipitalgia and subsequently developed transient dysarthria and mild hemiparesis. Although a transient ischemic attack was initially suspected, her condition deteriorated in a manner that was consistent with left hemiplegia with severe dysarthria. Magnetic resonance (MR) imaging revealed brain stem infarction, and MR angiography delineated a double-lumen sign in the basilar artery, indicating BAD. She was treated conservatively and brain stem infarction did not expand. One year after the onset of brain stem infarction, her activity of daily living is still dependent (modified Rankin Scale of 4), and there were no morphological changes associated with BAD or recurrent cerebrovascular events during the follow-up period. The association of MMD with BAD is extremely rare. While considering the common underlying pathology such as an affected internal elastic lamina and fragile medial layer, the occurrence of BAD in a patient with MMD in a stable hemodynamic state is apparently unique. Copyright © 2016 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  12. Cerebrovascular disease in pediatric patients

    Directory of Open Access Journals (Sweden)

    Rotta Newra Tellechea

    2002-01-01

    Full Text Available Although rare in childhood, stroke may have a serious impact when it happens in this stage of life. Also, it may be the first sign of a systemic disease. We report 12 cases of patients with stroke treated in the Neuropediatrics Unit of Hospital de Clínicas de Porto Alegre (HCPA from March 1997 to March 2000. All patients, from term infants to 12-year-old children hospitalized in the Pediatrics Unit of HCPA, had clinical suspicion of stroke, which was later confirmed by radiological studies. Patient follow up ranged from 1 to 6 years (mean = 3.4 years. Presenting symptoms were hemiparesis in 9 patients, seizures in 7, deviation of labial commissure in 3, and loss of consciousness in 1. The increase in the number of cases of childhood stroke identified and later confirmed by noninvasive methods had helped in the determination of different ethiologies of stroke: the most frequent being hematologic, cardiac and genetic diseases. However, our study included 6 newborns with stroke whose ethiology was not identified. Seven children with seizures received phenobarbital. Six term infants had neonatal seizures secondary to stroke and restricted to the first 72 hours of life.

  13. Moyamoya vasculopathy - Patient demographics and characteristics in the Finnish population.

    Science.gov (United States)

    Saarela, Marika; Mustanoja, Satu; Pekkola, Johanna; Tyni, Tiina; Hernesniemi, Juha; Kivipelto, Leena; Tatlisumak, Turgut

    2017-01-01

    Background and purpose Moyamoya vasculopathy, a rare steno-occlusive progressive cerebrovascular disorder, has not been thoroughly studied in Caucasian populations. We established a registry of Finnish patients treated at the Helsinki University Hospital, to collect and report demographic and clinical data. Methods We collected data both retrospectively and prospectively from all the patients with a moyamoya vasculopathy referred to our hospital between January 1987 and December 2014. All patients underwent a neurological outpatient clinic visit. Results We diagnosed 61 patients (50 females, 10 children) with moyamoya vasculopathy. The mean age at the disease-onset was 31.5 ± 17.9 years. The two most common presenting symptoms were ischemic stroke (n = 31) and hemorrhage (n = 8). Forty-four percent underwent revascularization surgery, and 70% were prescribed antithrombotic treatment. Conclusions The results support in part the Western phenotype of the disease considering the later presentation and larger female predominance compared to the Asian moyamoya vasculopathy reports. However, the proportion of ischemic strokes and hemorrhagic strokes is closer to Japanese population than German population. The absence of familial cases points to a different genetic profile in the Finnish patients.

  14. Comparison of 7.0- and 3.0-T MRI and MRA in ischemic-type moyamoya disease: preliminary experience.

    Science.gov (United States)

    Deng, Xiaofeng; Zhang, Zihao; Zhang, Yan; Zhang, Dong; Wang, Rong; Ye, Xun; Xu, Long; Wang, Bo; Wang, Kai; Zhao, Jizong

    2016-06-01

    OBJECT The authors compared the image quality and diagnostic sensitivity and specificity of 7.0-T and 3.0-T MRI and time-of-flight (TOF) MR angiography (MRA) in patients with moyamoya disease (MMD). METHODS MR images of 15 patients with ischemic-type MMD (8 males, 7 females; age 13-48 years) and 13 healthy controls (7 males, 6 females; age 19-28 years) who underwent both 7.0-T and 3.0-T MRI and MRA were studied retrospectively. The main intracranial arteries were assessed by using the modified Houkin's grading system (MRA score). Moyamoya vessels (MMVs) were evaluated by 2 grading systems: the MMV quality score and the MMV area score. Two diagnostic criteria for MMD were used: the T2 criteria, which used flow voids in the basal ganglion on T2-weighted images, and the TOF criteria, which used the high-intensity areas in the basal ganglion on source images from TOF MRA. All data were evaluated by 2 independent readers who were blinded to the strength field and presence or absence of MMD. Using conventional angiography as the gold standard, the sensitivity and specificity of 7.0-T and 3.0-T MRI/MRA in the diagnosis of MMD were calculated. The differences between 7.0-T and 3.0-T MRI and MRA were statistically compared. RESULTS No significant differences were observed between 7.0-T and 3.0-T MRA in MRA score (p = 0.317) or MRA grade (p = 0.317). There was a strong correlation between the Suzuki's stage and MRA grade in both 3.0-T (rs = 0.930; p 3.0-T MRA, suggested by both the MMV quality score (p = 0.001) and the MMV area score (p = 0.001). The correlation between the Suzuki's stage and the MMV area score was moderate in 3.0-T MRA (rs = 0.738; p = 0.002) and strong in 7.0-T MRA (rs = 0.908; p 3.0-T MRI/MRA (sensitivity 0.692; specificity 0.933) in diagnosing MMD; based on the TOF criteria, 7.0-T MRI/MRA was more sensitive (1.000 vs 0.733, respectively) and more specific (1.000 vs 0.923, respectively) than 3.0-T MRI/MRA. CONCLUSIONS Compared with 3.0-T MRI/MRA, 7.0-T

  15. A Case of Moyamoya-like Vessels with History of Brain Radiation

    Directory of Open Access Journals (Sweden)

    M Zare

    2004-08-01

    Full Text Available A 13 years old boy underwenta surgical operation of craniopharyngioma which followed by postoperative radiation therapy. Eighteen monthes later he was admitted to the neurological ward due to sudden sensory aphasia. Brain CT showed infarction of left hemisphere. Since no other predisposing factor was present, the cause of stroke in this patient can be related to Moyamoya - like disease after radiation. Keywords: Moyamoya, Stroke, Aphasia, Craniopharyngioma, Radiation Therapy

  16. Pediatric Cushing′s disease: Management Issues

    Directory of Open Access Journals (Sweden)

    Martin O Savage

    2012-01-01

    Full Text Available Cushing′s disease (CD, caused by an ACTH-secreting pituitary corticotroph adenoma, is the commonest cause of Cushing syndrome in children over 5 years of age. It is rare in the pediatric age range and presents difficult diagnostic and therapeutic challenges. Key presenting features include weight gain, growth failure and change in facial appearance. Most pediatric endocrinologists have limited experience managing children or adolescents with CD and thus benefit from close consultation with adult colleagues. We describe a diagnostic protocol which broadly follows the model for adult patients. Treatment strategies are examined and appraised. The management of pediatric CD patients after cure is also discussed.

  17. Moyamoya syndrome as a risk factor for stroke in Saudi children: Novel and usual associations

    International Nuclear Information System (INIS)

    Salih, Mustafa A.; Al-Jarallah, Ahmed A.; Kentab, Amal Y.; Murshid, Waleed R.; Elgamal, Essam A.; Al-Salman, Mussaad M.; Abdel-Gader, Abdel-Galil M.; Alorainy, Ibrahim A.; Hassan, Hamdy H.; Othman, Saleh A.; El-Desouki, Mahmoud I.; Maldergem, L. V.

    2006-01-01

    To report on moyamoya syndrome (MMS) as a risk factor for stroke in a prospective and retrospective cohort of Saudi children. The usual and novel associations of MMS in this cohort will also be described. Children with stroke were evaluated at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 (retrospective study) and February201 to March 2003 (retrospective study). Investigations for suspected cases included hemostatic assays, biochemical, and serological tests. Neuroimaging included CT, MRI, magnetic resonance angiography (MRA), single photon computerized tomography (SPECT) brain scan and conventional cerebral angiography. Moyamoya syndrome was the underlying risk factor for stroke in 6 (5.8%) of the 104 children (aged one month to 12 years). They were 4 females and 2 males. Their first cerebral ischemic event occurred at a mean age of 45 months (median = 44 months, range 17-66 months). In all 6 cases, MMS was associated with an underlying hematologic abnormality or other diseases. Protein C deficiency was identified in one girl and protein S deficiency in another. Two patients had retrospectively, sickle cell disease (SCD) and sickle cell-b-thalassemia (Sb-thalssemia), which had been associated in the latter with membranous ventricular septal defect. Adams-Oliver syndrome (AOS, OMIM 100300) was associated with MMS in an 18-month-old girl. A 4-year-old boy had wrinkly skin syndrome (WWS, OMIM 278250) phenotype. The association of MMS and protein C deficiency was first reported in this cohort of patients, whereas the association of the syndrome with WWS and AOS has not, hitherto, been described. The 3 patients who had MMS associated with protein C deficiency, SCD, and AOS underwent successful revascularization surgery in the form of encephaloduroarteriosynangiosis. Moyamoya syndrome constitutes an important risk factor of

  18. Recommended Curriculum for Training in Pediatric Transplant Infectious Diseases.

    Science.gov (United States)

    Danziger-Isakov, Lara; Allen, Upton; Englund, Janet; Herold, Betsy; Hoffman, Jill; Green, Michael; Gantt, Soren; Kumar, Deepali; Michaels, Marian G

    2015-03-01

    A working group representing the American Society of Transplantation, Pediatric Infectious Diseases Society, and International Pediatric Transplant Association has developed a collaborative effort to identify and develop core knowledge in pediatric transplant infectious diseases. Guidance for patient care environments for training and core competencies is included to help facilitate training directed at improving the experience for pediatric infectious diseases trainees and practitioners in the area of pediatric transplant infectious diseases. © The Author 2013. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  19. Magnetic resonance enterography in pediatric celiac disease.

    Science.gov (United States)

    Koc, Gonca; Doganay, Selim; Sevinc, Eylem; Deniz, Kemal; Chavhan, Govind; Gorkem, Sureyya B; Karacabey, Neslihan; Dogan, Mehmet S; Coskun, Abdulhakim; Aslan, Duran

    To assess if magnetic resonance enterography is capable of showing evidence/extent of disease in pediatric patients with biopsy-proven celiac disease by comparing with a control group, and to correlate the magnetic resonance enterography findings with anti-endomysial antibody level, which is an indicator of gluten-free dietary compliance. Thirty-one pediatric patients (mean age 11.7±3.1 years) with biopsy-proven celiac disease and 40 pediatric patients as a control group were recruited in the study. The magnetic resonance enterography images of both patients with celiac disease and those of the control group were evaluated by two pediatric radiologists in a blinded manner for the mucosal pattern, presence of wall thickening, luminal distention of the small bowel, and extra-intestinal findings. Patient charts were reviewed to note clinical features and laboratory findings. The histopathologic review of the duodenal biopsies was re-conducted. The mean duration of the disease was 5.6±1.8 years (range: 3-7.2 years). In 24 (77%) of the patients, anti-endomysial antibody levels were elevated (mean 119.2±66.6RU/mL). Magnetic resonance enterography revealed normal fold pattern in all the patients. Ten (32%) patients had enlarged mesenteric lymph nodes. Although a majority of the patients had elevated anti-endomysial antibody levels indicating poor dietary compliance, magnetic resonance enterography did not show any mucosal abnormality associated with the inability of magnetic resonance enterography to detect mild/early changes of celiac disease in children. Therefore, it may not be useful for the follow-up of pediatric celiac disease. Copyright © 2017 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  20. Electroconvulsive therapy in a patient with moyamoya syndrome.

    Science.gov (United States)

    Ghignone, Erica; Rosenthal, Lisa; Lloyd, Robert Brett; Mouli, Samdeep; Dinwiddie, Stephen

    2015-03-01

    We report on a 30-year-old woman diagnosed with moyamoya syndrome resulting from sickle cell disease who developed catatonia and was successfully treated with electroconvulsive therapy (ECT). Neuroimaging revealed severe tandem narrowing of the left internal carotid artery with diminished cerebral blood flow, moderate narrowing of the right supraclinoid aspect of the right internal carotid artery, and associated numerous lenticulostriate collaterals bilaterally, consistent with moyamoya. The patient presented with mutism; posturing; immobility; stupor; withdrawal; refusal to eat, drink, or speak; and staring, supporting a diagnosis of catatonia. It initially responded to a lorazepam challenge; however, a complicated hospital course and deterioration of the patient's condition, including septic shock, delirium, and continued catatonic symptoms, led to the pursuit of ECT to treat her symptoms. We discuss the risks involved with the administration of ECT in a patient with fragile cerebral vasculature and the successful treatment of catatonia in this patient without resultant stroke or cerebral hemorrhage.

  1. Pharmacological treatments and infectious diseases in pediatric inflammatory bowel disease.

    Science.gov (United States)

    Dipasquale, Valeria; Romano, Claudio

    2018-03-01

    The incidence of pediatric inflammatory bowel disease (IBD) is rising, as is the employment of immunosuppressive and biological drugs. Most patients with IBD receive immunosuppressive therapies during the course of the disease. These molecules are a double-edged sword; while they can help control disease activity, they also increase the risk of infections. Therefore, it is important that pediatricians involved in primary care, pediatric gastroenterologists, and infectious disease physicians have a thorough knowledge of the infections that can affect patients with IBD. Areas covered: A broad review of the major infectious diseases that have been reported in children and adolescents with IBD was performed, and information regarding surveillance, diagnosis and management were updated. The possible correlations with IBD pharmacological tools are discussed. Expert commentary: Opportunistic infections are possible in pediatric IBD, and immunosuppressive and immunomodulator therapy seems to play a causative role. Heightened awareness and vigilant surveillance leading to prompt diagnosis and treatment are important for optimal management.

  2. Pediatric Non-Alcoholic Fatty Liver Disease

    Directory of Open Access Journals (Sweden)

    Haley Bush

    2017-06-01

    Full Text Available Abstract: With the increase in the prevalence of obesity, non-alcoholic fatty liver disease (NAFLD has become among the leading causes of chronic liver disease in the pediatric age group. Once believed to be a “two-hit process”, it is now clear that the actual pathophysiology of NAFLD is complex and involves multiple pathways. Moreover, NAFLD is not always benign, and patients with non-alcoholic steatohepatitis (NASH are at increased risk of developing advanced stages of liver disease. It has also been shown that NAFLD is not only a liver disease, but is also associated with multiple extrahepatic manifestations, including cardiovascular diseases, type 2 diabetes, and low bone mineral density. Although the data is scarce in the pediatric population, some studies have suggested that long-term mortality and the requirement of liver transplantation will continue to increase in patients with NAFLD. More studies are needed to better understand the natural history of NAFLD, especially in the pediatric age group.

  3. Radioiodine treatment for pediatric hyperthyroid Grave's disease.

    Science.gov (United States)

    Chao, Ma; Jiawei, Xie; Guoming, Wang; Jianbin, Liu; Wanxia, Liu; Driedger, Al; Shuyao, Zuo; Qin, Zhang

    2009-10-01

    Grave's disease (GD) is an autoimmune disease in which excessive amounts of thyroid hormones circulate in the blood. Treatment for pediatric GD includes (1) antithyroid drugs (ATD), (2) radioiodine, and (3) thyroidectomy. Yet, the optimal therapy remains controversial. We collected studies from all electronically available sources as well as from conferences held in China. All studies using radioiodine and/or ATD and/or thyroidectomy were included. Information was found on 1,874 pediatric GD patients treated with radioiodine, 1,279 patients treated with ATD and 1,362 patients treated surgically. The cure rate for radioiodine was 49.8%; the incidence of hypothyroidism, 37.8%; of relapse, 6.3%; of adverse effects, 1.55%; and of drop outs, 0.6%. These data show that radioiodine treatment is safe and effective in pediatric GD with significant lower incidence of relapse and adverse effects but significantly higher incidence of hypothyroidism as compared with both ATD and thyroidectomy. For the time being, radioiodine treatment for pediatric GD remains an excellent first-line therapy and a good second-line therapy for patients with ATD failure, severe complications, or poor compliance.

  4. Pediatric Hodgkin's disease - up, up, and beyond

    International Nuclear Information System (INIS)

    Donaldson, Sarah S.

    2002-01-01

    Juan A. del Regato, 1909-1999, was a superb clinician-educator who recognized the radiocurability of Hodgkin's disease but questioned treatment without late effects, particularly in children. The remarkable progress in pediatric Hodgkin's disease today is a tribute to this influential pioneer, who served as a role model to many. Combined modality therapy using low-dose, involved-field radiation and multiagent chemotherapy today results in a 5-year relative survival rate of 94% among American children with Hodgkin's disease. However, several areas hold promise for future advances, including a new pathology classification and biology studies that distinguish classic Hodgkin's disease from other lymphomas; new noninvasive staging techniques, including 18F-fluorodeoxyglucose-positron emission tomography; the definition of risk groups to segregate low-, intermediate-, and high-risk groups on the basis of a prognostic index, facilitating risk-adapted therapy; and myeloablative therapy followed by hematopoietic stem cell transplantation. Currently used for children with relapse, it is associated with a 5-year survival of 65% and should be considered as the initial therapy for high-risk groups. Idiopathic diffuse pulmonary toxicity after autologous transplantation is high among children with an atopic history; thus, atopy should be considered when selecting children appropriate for transplantation. Finally, novel therapies, such as the anti-CD20 antibody, rituximab, may be useful for children with CD20+, lymphocyte-predominant Hodgkin's disease. The universal goal of cure without late effects is realistic for almost all children with Hodgkin's disease today

  5. Pattern of pediatric heart diseases in Pakistan

    International Nuclear Information System (INIS)

    Sadiq, M.; Roshan, B.; Khan, A.; Latif, F.; Bashir, I.; Sheikh, S.A.

    2002-01-01

    Objective: To assess the pattern, age distribution and relative incidence of heart diseases in pediatric patients aged 16 years and less. Design: A five-year analysis of all children undergoing echocardiography for possible heart disease in a single center. Setting: Tertiary referral center for pediatric and adult cardiac services in the central and southern Punjab, Pakistan. Patients and Methods: Data of all new children undergoing detailed echocardiography was reviewed for type of lesion age at presentation and gender. Results: over a period of five years, (may 1996 to April 2001), 7400 patients underwent echocardiography. Of these, 6620 had cardiac lesions while 780 patients were normal and excluded from the study. Of 6620 patients, 4184 (63.2%) had congenital heart defects (CHD) while 2335 (35.3%) acquired heart disease (AHD) and 101 (1.5%) were placed in miscellaneous group. Of CHD, ventricular septal defect was the most common lesion (32% of all patients with CHD), followed by atrial septal defects (13.2%) and persistent arterial dust (12.8%). Majority was males (65%) and the mean age of presentation was 5.8 years for acyanotic and 4.8 years for cyanotic heart defects. Tetralogy of fallout was the most common cyanotic lesion (16.06%) with mean age of presentation being 4.2 years. The relative incidence of patients with critical health lesions was much less and only 586 patients (14%) were under the age of one year at presentation. Children presenting less than one month of age were only 3% (127 patients). Amongst AHD, 71.5% (1670) had rheumatic heart disease (RHD) while 24.5% (572) had mycocardial disease,clinically diagnosed as myocarditis or dilated cardiomyopathy. The mean age of presentation for myocarditis was 2.3 year and majority was clustered in the months of March, April, September and October. Amongst RHD, mitral regurgitation was the commonest lesion: 681 patients(40.8%), followed by mixed lesion of mitral and aortic regurgitation in 382 patients

  6. Magnetic resonance imaging in pediatric neurological disease

    International Nuclear Information System (INIS)

    Tsukiyama, Takashi; Nishimoto, Hiroshi; Fujioka, Mutsuhisa; Aihara, Toshinori; Tanaka, Osamu.

    1986-01-01

    In this paper, we summarize our initial experience with Magnetic Resonance Imaging(MRI) in the evaluation of pediatric neurological disease. 17 children between the ages of 2 month and 8.5 year have been examined with MRI. All subjects tolerated the MRI procedure well, although sedation was necessary for young children. Result as follows : (1) MRI does not utilize ionising radiation to produce an image. (2) MRI images more clearly demonstrate cerebral gray and white matter than X-ray CT. (3) Compared with X-ray CT, MRI proved to be advantageous in detection and characterization of the pathology, especially when the abnormality was located along the posterior fossa and spinal canal. It is suggested that these nature of MRI makes it the ideal diagnostic method for children. (author)

  7. Pediatric invasive pneumococcal disease in Senegal.

    Science.gov (United States)

    Ba, I D; Ba, A; Faye, P M; Thiongane, A; Attiyé Kane, M; Sonko, A; Diop, A; Deme Ly, I; Diouf, F N; Ndiaye, O; Leye, M M M; Cissé, M F; Ba, M

    2015-01-01

    We aimed to describe the clinical, epidemiological, and outcome characteristics of IPD case patients hospitalized at the Albert-Royer National Children's Hospital (French acronym CHNEAR) to evaluate the disease burden of IPDs in a pediatric hospital of Dakar (Senegal). All children aged 0-15 years hospitalized at the CHNEAR between January 1st, 2008 and December 31st, 2013 for a documented IPD were included in the study. Medical history, risk factors, clinical, bacteriological, and outcome data was collected. Data was then analyzed using the SPSS software, version 16 (Pearson's Chi(2) test: a P-valueSenegal. Infants<2 years of age are particularly affected. The very high case fatality (17%) was significantly associated with meningeal infection sites hence the need for better access to pneumococcal vaccines. Copyright © 2015. Published by Elsevier SAS.

  8. Diagnostic imaging in pediatric renal inflammatory disease

    International Nuclear Information System (INIS)

    Sty, J.R.; Wells, R.G.; Schroeder, B.A.; Starshak, R.J.

    1986-01-01

    Some form of imaging procedure should be used to document the presence of infection of the upper urinary tract in troublesome cases in children. During the past several years, sonography, nuclear radiology, and computed tomography (CT) have had a significant influence on renal imaging. The purpose of this article is to reevaluate the noninvasive imaging procedures that can be used to diagnose pediatric renal inflammatory disease and to assess the relative value of each modality in the various types of renal infection. The authors will not discuss the radiologic evaluation of the child who has had a previous renal infection, in whom cortical scarring or reflux nephropathy is a possibility; these are different clinical problems and require different diagnostic evaluation

  9. Malignancy and mortality in pediatric patients with inflammatory bowel disease

    DEFF Research Database (Denmark)

    de Ridder, Lissy; Turner, Dan; Wilson, David C

    2014-01-01

    working group of ESPGHAN conducted a multinational-based survey of cancer and mortality in pediatric IBD. METHODS: A survey among pediatric gastroenterologists of 20 European countries and Israel on cancer and/or mortality in the pediatric patient population with IBD was undertaken. One representative...... were diagnosed with IBD (ulcerative colitis, n = 21) at a median age of 10.0 years (inter quartile range, 3.0-14.0). Causes of mortality were infectious (n = 14), cancer (n = 5), uncontrolled disease activity of IBD (n = 4), procedure-related (n = 3), other non-IBD related diseases (n = 3), and unknown...

  10. Leukotrienes and leukotriene modifiers in pediatric allergic diseases

    African Journals Online (AJOL)

    Ehab

    ... 12(1):3-12. 3. Leukotrienes and leukotriene modifiers in pediatric allergic diseases ... childhood allergic diseases. This review ... produce cysteinyl-LTs and LTB4 spontaneously in vitro.8. Although nonleukocyte cells generally do not have sufficient 5-LO and FLAP to synthesize .... uninterrupted sleep in these patients.55.

  11. Magnetic resonance enterography in pediatric celiac disease

    Directory of Open Access Journals (Sweden)

    Gonca Koc

    2017-07-01

    Conclusion: Although a majority of the patients had elevated EMA levels indicating poor dietary compliance, MRE did not show any mucosal abnormality associated with the inability of MRE to detect mild/early changes of CD in children. Therefore, it may not be useful for the follow‐up of pediatric CD.

  12. A Case of Isolated Middle Cerebral Artery Stenosis with Hemichorea and Moyamoya Pattern Collateralization

    Directory of Open Access Journals (Sweden)

    Seok Jong Chung

    2013-05-01

    Full Text Available Isolated middle cerebral artery (MCA stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.

  13. Surgical care of the pediatric Crohn's disease patient.

    Science.gov (United States)

    Stewart, Dylan

    2017-12-01

    Despite the significant advances in the medical management of inflammatory bowel disease over the last decade, surgery continues to play a major role in the management of pediatric Crohn's disease (CD). While adult and pediatric Crohn's disease may share many clinical characteristics, pediatric Crohn's patients often have a more aggressive phenotype, and the operative care given by the pediatric surgeon to the newly diagnosed Crohn's patient is very different in nature to the surgical needs of adult patients after decades of disease progression. Children also have the unique surgical indication of growth failure to consider in the overall clinical decision making. While surgery is never curative in CD, it has the ability to transform the disease process in children, and appropriately timed operations may have tremendous impact on a child's physical and mental maturation. This monograph aims to address the surgical care of Crohn's disease in general, with a specific emphasis on the surgical treatment of small intestinal and ileocecal involvement. Copyright © 2017 Elsevier Inc. All rights reserved.

  14. Thyroid disease in the pediatric patient: emphasizing imaging with sonography

    International Nuclear Information System (INIS)

    Babcock, Diane S.

    2006-01-01

    Thyroid disease does occur in the pediatric patient, and imaging plays an important role in its evaluation. A review is presented of normal development of the thyroid gland, the technique and indications for thyroid sonography, and key imaging features of congenital thyroid disorders (ectopic or absent thyroid, infantile goiter, thyroglossal duct remnants), benign thyroid masses (follicular adenoma, degenerative nodules, colloid and thyroid cysts), malignant masses (follicular, papillary and medullary carcinoma) and diffuse thyroid disease (acute bacterial thyroiditis, Hashimoto's thyroiditis, Grave's disease). (orig.)

  15. Pediatric surgical diseases. A radiological surgical case study approach

    International Nuclear Information System (INIS)

    Esposito, Ciro; Esposito, Giovanni

    2009-01-01

    Radiologic evaluation of an infant or child suspected of having a surgical disease can be a complex problem. With this volume, the editors have created a book focused on pediatric imaging written by pediatricians, pediatric surgeons and pediatric radiologists. This book is a collection of over 200 case reports. The concept is a case study approach: The reader is given radiologic images (plain radiography, computed tomography, magnetic resonance imaging, ultrasonography, etc.) and the clinical history of the patient. On the basis of this information, the reader is asked to identify a diagnostic and therapeutic strategy. Each case is complemented by information on the disease affecting the patient and the management of the case shown, including therapy and follow-up. This educational text is targeted at all medical professionals faced with a variety of diagnostic and therapeutic problems affecting infants and children. (orig.)

  16. Pediatric surgical diseases. A radiological surgical case study approach

    Energy Technology Data Exchange (ETDEWEB)

    Esposito, Ciro [Federico II Univ. of Naples (Italy). Chair of Pediatric Surgery; Esposito, Giovanni (eds.) [Federico II Univ. of Naples (Italy). School of Medicine

    2009-07-01

    Radiologic evaluation of an infant or child suspected of having a surgical disease can be a complex problem. With this volume, the editors have created a book focused on pediatric imaging written by pediatricians, pediatric surgeons and pediatric radiologists. This book is a collection of over 200 case reports. The concept is a case study approach: The reader is given radiologic images (plain radiography, computed tomography, magnetic resonance imaging, ultrasonography, etc.) and the clinical history of the patient. On the basis of this information, the reader is asked to identify a diagnostic and therapeutic strategy. Each case is complemented by information on the disease affecting the patient and the management of the case shown, including therapy and follow-up. This educational text is targeted at all medical professionals faced with a variety of diagnostic and therapeutic problems affecting infants and children. (orig.)

  17. Pediatric gastroesophageal reflux disease: Current diagnosis and management

    NARCIS (Netherlands)

    van der Pol, R.J.

    2014-01-01

    Pediatric gastroesophageal reflux disease (GERD) is a disorder difficult to diagnose and to treat. Due to the current definition of GERD, i.e. gastroesophageal reflux (GER) causing bothersome symptoms and/or complications, diagnosis is subject to broad interpretation. This thesis consists of studies

  18. Profile of the Pediatric Infectious Disease Workforce in 2015.

    Science.gov (United States)

    Yeh, Sylvia H; Vijayan, Vini; Hahn, Andrea; Ruch-Ross, Holly; Kirkwood, Suzanne; Phillips, Terri Christene; Harrison, Christopher J

    2017-12-22

    Almost 20 years have elapsed since the last workforce survey of pediatric infectious disease (PID) subspecialists was conducted in 1997-1998. The American Academy of Pediatrics Section on Infectious Diseases in collaboration with the Pediatric Infectious Diseases Society sought to assess the status of the current PID workforce. A Web-based survey conducted in 2015 collected data on demographics, practice patterns, and job satisfaction among the PID workforce, and identified factors related to job placement among recent fellowship graduates. Of 946 respondents (48% response rate), 50% were female. The average age was 51 years (range, 29-88 years); 63% were employed by an academic center/hospital, and 85% provided direct patient care; and 18% were not current PID practitioners. Of the 138 (21%) respondents who had completed a PID fellowship within the previous 5 years, 83% applied for maintain the pipeline and improve satisfaction among its physicians. © The Author 2017. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  19. [Common pediatric infectious diseases following natural disasters].

    Science.gov (United States)

    Yao, Kai-Hu

    2013-06-01

    Natural disasters may lead to the outbreaks of infectious diseases because they increase the risk factors for infectious diseases. This paper reviews the risk factors for infectious diseases after natural disasters, especially earthquake, and the infectious diseases following disasters reported in recent years. The infectious diseases after earthquake include diarrhea, cholera, viral hepatitis, upper respiratory tract infection, tuberculosis, measles, leptospirosis, dengue fever, tetanus, and gas gangrene, as well as some rare infections. Children are vulnerable to infectious diseases, so pediatricians should pay more attention to the research on relationship between infectious diseases and natural disasters.

  20. Serial 99mTc-HMPAO Brain SPECT for Assessing Perfusion Improvement after DEAS in Moyamoya Patients

    International Nuclear Information System (INIS)

    Lee, Kyung Han; Lee, Sang Hyung; Yeo, Jeong Seok; Kwark, Chul Eun; Chung, June Key; Lee, Myoung Chul; Cho, Byoung Kyu; Koh, Chang Soon

    1994-01-01

    Encephalo-duro-arterio-synangiosis (EDAS) is a relatively new surgical procedure for treatment of childhood moyamoya disease. We assessed regional cerebral perfusion in moyamoya patients before (1.3 mo) and after (6.8 mo) EDAS with 99m Tc-HMPAO brain SPECT. A total of 21 EDAS operations in 17 moyamoya patients was included. Preoperative CT or MRI showed cerebral infarction in 14 patients and carotid angiography showed Suzuki grade 1 to V stenosis in 6%, 9%, 62%, 12% and 12% of the hemispheres respectively. Preoperative SPECT showed regional hypoperfusion in all patients, bilateral frontal and temporal loves being the most frequently involved site. 4 X 4 pixel sized ROIs were applied on the frontotemmporal cortex in 3 slice averaged transverse tomographic images. An index of regional perfusion was measured as; PI (%)=average FT activity/average cerebellar activity X 100 Pre-EDAS ipsilateral PI ranged from 23.7 to 98.4% (mean:74.3 ± 17%) and increased significantly after operation (81.4 ± 17%, p 90, 0.5) (p<0.001). The amount of perfusion improvement (PI) showed significant correlation with CI (r-0.42, p=0.04). PI did not, however, correlate with the amount of neovascularization assessed angiographically in 8 patients. Serial HMPAO SPECT is an useful noninvasive study for assessing perfusion improvement after EDAS in childhood moyamoya patients.

  1. Applications of cardiac MRI in pediatric heart diseases

    International Nuclear Information System (INIS)

    Tao Xiaojuan; Zeng Jinjin; Sun Jihang; Cheng Hua; Yin Guangheng

    2009-01-01

    Objective: To evaluate the value of magnetic resonance imaging in pediatric heart diseases. Methods: Ninety-seven cases received cardiac MR scanning in this present study. The age range was 2 day to 13 years including 62 boys and 35 girls, the median age was 6 years. They were performed on h 5 T scanner with cardiac phased-array coil and VCG. Results: Eighty-five of the 97 cases were positive. Those positive findings included cardiomyopathy in 41 cases, congenital heart disease in 20 cases, constrictive pericarditis in 4 cases, pericardiac effusions with or without other cardiovascular diseases in 17 cases, cardiac tumor in 2 cases,thrombus in 3 cases and in 5 other cases. Conclusion: Cardiac MRI is an excellent imaging modality for the anatomical and functional abnormalities of pediatric heart diseases. (authors)

  2. Pediatric Blood Pressure and Adult Preclinical Markers of Cardiovascular Disease

    Science.gov (United States)

    Magnussen, Costan G.; Smith, Kylie J.

    2016-01-01

    A high blood pressure level in adults is considered the single most important modifiable risk factor for global disease burden, especially those of cardiovascular (CV) origin such as stroke and ischemic heart disease. Because blood pressure levels have been shown to persist from childhood to adulthood, elevations in pediatric levels have been hypothesized to lead to increased CV burden in adulthood and, as such, might provide a window in the life course where primordial and primary prevention could be focused. In the absence of substantive data directly linking childhood blood pressure levels to overt adult CV disease, this review outlines the available literature that examines the association between pediatric blood pressure and adult preclinical markers of CV disease. PMID:27168729

  3. Cytomegalovirus infection in pediatric rheumatic diseases: a review

    Directory of Open Access Journals (Sweden)

    Wolf Dana G

    2010-05-01

    Full Text Available Abstract Human cytomegalovirus (HCMV is familiar to pediatric rheumatologists mainly as a cause of opportunistic disease in pharmacologically immune suppressed patients. However, HCMV also has a variety of immuno-modulatory effects, through which it may influence the course of rheumatic conditions. In this article we discuss the interplay between HCMV and the immune system, and review the clinical manifestations, diagnosis, and treatment of HCMV infection in children with rheumatic disease.

  4. New serological markers in pediatric patients with inflammatory bowel disease

    Science.gov (United States)

    Kovács, Márta; Müller, Katalin Eszter; Papp, Mária; Lakatos, Péter László; Csöndes, Mihály; Veres, Gábor

    2014-01-01

    The spectrum of serological markers associated with inflammatory bowel disease (IBD) is rapidly growing. Due to frequently delayed or missed diagnoses, the application of non-invasive diagnostic tests for IBD, as well as differentiation between ulcerative colitis (UC) and Crohn’s disease (CD), would be useful in the pediatric population. In addition, the combination of pancreatic autoantibodies and antibodies against Saccharomyces cerevisiae antibodies/perinuclear cytoplasmic antibody (pANCA) improved the sensitivity of serological markers in pediatric patients with CD and UC. Some studies suggested that age-associated differences in the patterns of antibodies may be present, particularly in the youngest children. In CD, most patients develop stricturing or perforating complications, and a significant number of patients undergo surgery during the disease course. Based on recent knowledge, serum antibodies are qualitatively and quantitatively associated with complicated CD behavior and CD-related surgery. Pediatric UC is characterized by extensive colitis and a high rate of colectomy. In patients with UC, high levels of anti-CBir1 and pANCA are associated with the development of pouchitis after ileal pouch-anal anastomosis. Thus, serologic markers for IBD can be applied to stratify IBD patients into more homogeneous subgroups with respect to disease progression. In conclusion, identification of patients at an increased risk of rapid disease progression is of great interest, as the application of early and more aggressive pharmaceutical intervention could have the potential to alter the natural history of IBD, and reduce complications and hospitalizations. PMID:24803798

  5. Current pharmacotherapy for treating pediatric nonalcoholic fatty liver disease.

    Science.gov (United States)

    Della Corte, Claudia; Liccardo, Daniela; Ferrari, Federica; Alisi, Anna; Nobili, Valerio

    2014-12-01

    In the past decade, nonalcoholic fatty liver disease (NAFLD) had rapidly become one of the most common liver diseases. If efficient therapeutic strategies will not reduce the prevalence of NAFLD in children soon, serious deleterious effects on the quality of life of these patients in adulthood are expected. Lifestyle modification is the current first-line therapy for pediatric NAFLD, even though it is difficult to obtain and to maintain. Therefore, lifestyle changes are usually ineffective and long-lasting improvement of the NAFLD-associated liver damage is rarely observed. As guidelines for the management of NAFLD in children are still lacking, the identification of effective treatments represents a challenge for pediatric hepatologists in the near future. Here, we review the existing therapeutic approaches for treating NAFLD in children and overview all ongoing clinical trials for new promising drugs in pediatric setting. Considering the multifactorial pathogenesis and the wide spectrum of histological and clinical features of NAFLD, we believe that a drug mix, containing agents that are effective against the principal pathogenetic factors, associated with lifestyle modification, could represent the winning choice of treatment for pediatric NAFLD.

  6. Radiological imaging in pediatric rheumatic diseases

    International Nuclear Information System (INIS)

    Matuszewska, Genowefa; Zaniewicz-Kaniewska, Katarzyna; Włodkowska-Korytkowska, Monika; Smorawińska, Patrycja; Saied, Fadhil; Kunisz, Wojciech; Sudoł-Szopińska, Iwona

    2014-01-01

    Radiological imaging plays a fundamental role in the diagnosis and monitoring of rheumatic diseases. The basic method of imaging is a classic X-ray picture, which for many years has been used as a single method for the recognition and evaluation of the effects of disease management. In today’s modern day treatment of rheumatic diseases, ultrasonography and magnetic resonance are more commonly performed for early detection of inflammatory changes in the region of soft tissue, subchondral bone and bone marrow. In spite of their usefulness and fundamental role in the diagnosis, X-ray still remains an essential tool in the diagnosis of rheumatoid arthritis in children and is complementary to today’s methods of imaging diagnostics. In clinical practice, X-ray imaging is still an important examination performed not only to recognize the disorders, but also to provide a differential diagnosis. It helps estimate disease progression and is used to monitor the effects of treatment and the development of possible complications. Differential diagnosis of rheumatic diseases is performed on the basis of localization and type of radiographic changes. The surrounding periarticular soft tissues, bone structures, joint space, with special attention to articular bone surfaces and epiphyses, are analyzed. The aim of this work is to describe characteristic inflammatory changes present on X-ray imaging typical for the most commonly diagnosed rheumatic diseases in children, such as juvenile idiopathic arthritis, systemic lupus erythematosus, systemic scleroderma, mixed connective tissue disease, juvenile dermatomyositis, juvenile spondyloarthropathy and systemic vascular disease

  7. Managing the pediatric patient with celiac disease: a multidisciplinary approach

    Directory of Open Access Journals (Sweden)

    Isaac DM

    2016-10-01

    Full Text Available Daniela Migliarese Isaac,1 Jessica Wu,2 Diana R Mager,3,4 Justine M Turner1 1Department of Pediatric Gastroenterology and Nutrition, Faculty of Medicine and Dentistry, University of Alberta; 2Alberta Health Services–Child Health Nutrition Services, Stollery Children’s Hospital; 3Department of Agriculture, Food and Nutritional Science; 4Department of Pediatrics, University of Alberta, Edmonton, AB, Canada Abstract: Celiac disease (CD is an autoimmune reaction to gluten, leading to intestinal inflammation, villous atrophy, and malabsorption. It is the most common autoimmune gastrointestinal disorder, with an increasing prevalence. A life-long gluten-free diet (GFD is an effective treatment to alleviate symptoms, normalize autoantibodies, and heal the intestinal mucosa in patients with CD. Poorly controlled CD poses a significant concern for ongoing malabsorption, growth restriction, and the long-term concern of intestinal lymphoma. Achieving GFD compliance and long-term disease control poses a challenge, with adolescents at particular risk for high rates of noncompliance. Attention has turned toward innovative management strategies to improve adherence and achieve better disease control. One such strategy is the development of multidisciplinary clinic approach, and CD is a complex life-long disease state that would benefit from a multifaceted team approach as recognized by multiple national and international bodies, including the National Institutes of Health. Utilizing the combined efforts of the pediatric gastroenterologist, registered dietitian, registered nurse, and primary care provider (general practitioner or general pediatrician in a CD multidisciplinary clinic model will be of benefit for patients and families in optimizing diagnosis, provision of GFD teaching, and long-term adherence to a GFD. This paper discusses the benefits and proposed structure for multidisciplinary care in improving management of CD. Keywords: celiac disease

  8. Thyroid disease in the pediatric patient: emphasizing imaging with sonography

    Energy Technology Data Exchange (ETDEWEB)

    Babcock, Diane S. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States)

    2006-04-15

    Thyroid disease does occur in the pediatric patient, and imaging plays an important role in its evaluation. A review is presented of normal development of the thyroid gland, the technique and indications for thyroid sonography, and key imaging features of congenital thyroid disorders (ectopic or absent thyroid, infantile goiter, thyroglossal duct remnants), benign thyroid masses (follicular adenoma, degenerative nodules, colloid and thyroid cysts), malignant masses (follicular, papillary and medullary carcinoma) and diffuse thyroid disease (acute bacterial thyroiditis, Hashimoto's thyroiditis, Grave's disease). (orig.)

  9. Survey of Pediatric Infectious Diseases Society Members About Congenital Chagas Disease.

    Science.gov (United States)

    Edwards, Morven S; Abanyie, Francisca A; Montgomery, Susan P

    2018-01-01

    Participants in a survey about congenital Chagas disease, distributed electronically to Pediatric Infectious Diseases Society members, perceived having limited knowledge about congenital Trypanosoma cruzi infection. Most rarely or never consider the diagnosis in infants born to parents from Latin America. Improved awareness of congenital Chagas disease and assessment of at-risk infants is needed.

  10. Computational modeling and engineering in pediatric and congenital heart disease.

    Science.gov (United States)

    Marsden, Alison L; Feinstein, Jeffrey A

    2015-10-01

    Recent methodological advances in computational simulations are enabling increasingly realistic simulations of hemodynamics and physiology, driving increased clinical utility. We review recent developments in the use of computational simulations in pediatric and congenital heart disease, describe the clinical impact in modeling in single-ventricle patients, and provide an overview of emerging areas. Multiscale modeling combining patient-specific hemodynamics with reduced order (i.e., mathematically and computationally simplified) circulatory models has become the de-facto standard for modeling local hemodynamics and 'global' circulatory physiology. We review recent advances that have enabled faster solutions, discuss new methods (e.g., fluid structure interaction and uncertainty quantification), which lend realism both computationally and clinically to results, highlight novel computationally derived surgical methods for single-ventricle patients, and discuss areas in which modeling has begun to exert its influence including Kawasaki disease, fetal circulation, tetralogy of Fallot (and pulmonary tree), and circulatory support. Computational modeling is emerging as a crucial tool for clinical decision-making and evaluation of novel surgical methods and interventions in pediatric cardiology and beyond. Continued development of modeling methods, with an eye towards clinical needs, will enable clinical adoption in a wide range of pediatric and congenital heart diseases.

  11. Neurocognitive Aspects of Pediatric Sickle Cell Disease.

    Science.gov (United States)

    Brown, Ronald T.; And Others

    1993-01-01

    This literature review on neurocognitive functioning and learning of children with sickle cell disease found diffuse neurocognitive deficits, with much variability across subjects. Studies of psychosocial development of these children indicate that behavioral problems, low self-esteem, and body image disturbances are frequently characteristic.…

  12. Is Autosomal Dominant Polycystic Kidney Disease Becoming a Pediatric Disorder?

    Directory of Open Access Journals (Sweden)

    Stéphanie De Rechter

    2017-12-01

    Full Text Available Autosomal dominant polycystic kidney disease (ADPKD affects 1 in 400 to 1,000 live births, making it the most common monogenic cause of renal failure. Although no definite cure is available yet, it is important to affect disease progression by influencing modifiable factors such as hypertension and proteinuria. Besides this symptomatic management, the only drug currently recommended in Europe for selected adult patients with rapid disease progression, is the vasopressin receptor antagonist tolvaptan. However, the question remains whether these preventive interventions should be initiated before extensive renal damage has occurred. As renal cyst formation and expansion begins early in life, frequently in utero, ADPKD should no longer be considered an adult-onset disease. Moreover, the presence of hypertension and proteinuria in affected children has been reported to correlate well with disease severity. Until now, it is controversial whether children at-risk for ADPKD should be tested for the presence of the disease, and if so, how this should be done. Herein, we review the spectrum of pediatric ADPKD and discuss the pro and contra of testing at-risk children and the challenges and unmet needs in pediatric ADPKD care.

  13. Use of probiotics in pediatric infectious diseases.

    Science.gov (United States)

    Caffarelli, Carlo; Cardinale, Fabio; Povesi-Dascola, Carlotta; Dodi, Icilio; Mastrorilli, Violetta; Ricci, Giampaolo

    2015-01-01

    We summarize current evidence and recommendations for the use of probiotics in childhood infectious diseases. Probiotics may be of benefit in treating acute infectious diarrhea and reducing antibiotic-associated diarrhea. Potential benefits of probiotic on prevention of traveler's diarrhea,Clostridium difficile-associated diarrhea, side effects of triple therapy in Helicobacter pylori eradication, necrotizing enterocolitis, acute diarrhea, acute respiratory infections and recurrent urinary tract infections remain unclear. More studies are needed to investigate optimal strain, dosage, bioavailability of drops and tablets, duration of treatment and safety. Probiotics and recombinant probiotic strain represent a promising source of molecules for the development of novel anti-infectious therapy.

  14. Canine osteosarcoma: a naturally occurring disease to inform pediatric oncology.

    Science.gov (United States)

    Fenger, Joelle M; London, Cheryl A; Kisseberth, William C

    2014-01-01

    Osteosarcoma (OSA) is the most common form of malignant bone cancer in children and dogs, although the disease occurs in dogs approximately 10 times more frequently than in people. Multidrug chemotherapy and aggressive surgical techniques have improved survival; however, new therapies for OSA are critical, as little improvement in survival times has been achieved in either dogs or people over the past 15 years, even with significant efforts directed at the incorporation of novel therapeutic approaches. Both clinical and molecular evidence suggests that human and canine OSA share many key features, including tumor location, presence of microscopic metastatic disease at diagnosis, development of chemotherapy-resistant metastases, and altered expression/activation of several proteins (e.g. Met, ezrin, phosphatase and tensin homolog, signal transducer and activator of transcription 3), and p53 mutations, among others. Additionally, canine and pediatric OSA exhibit overlapping transcriptional profiles and shared DNA copy number aberrations, supporting the notion that these diseases are similar at the molecular level. This review will discuss the similarities between pediatric and canine OSA with regard to histology, biologic behavior, and molecular genetic alterations that indicate canine OSA is a relevant, spontaneous, large animal model of the pediatric disease and outline how the study of naturally occurring OSA in dogs will offer additional insights into the biology and future treatment of this disease in both children and dogs. © The Author 2014. Published by Oxford University Press on behalf of the Institute for Laboratory Animal Research. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  15. Missing relationship of moyamoya and persistent primitive artery in Europeans. Another distinctive feature or artifact?

    Science.gov (United States)

    Wenz, Holger; Wenz, Ralf; Förster, Alex; Fontana, Johann; Kerl, Hans Ulrich; Groden, Christoph; Scharf, Johann

    2015-11-01

    Previous studies found higher incidence of persistent primitive arteries in Asian moyamoya (MM) patients than in the general population, which was thought to be a characteristic trait of the MM entity in general. We analyzed incidence of persistent primitive arteries and demographics of patients with European MM treated in one single center. First, we compared our large dataset to existing literature and second, we raised the question whether European MM demonstrates similar high prevalence of persistent primitive arteries as it was previously presented within Asian MM. All European MM on whom revascularization surgery was performed from 1999 to 2013 were included. Demographics and associated diseases were obtained by retrospective chart review. Two independent readers evaluated 122 MM angiograms to determine the occurrence of persistent primitive arteries as well as the Suzuki score. We identified 112 cases with MM disease, 10 with MM syndrome. Mean age at time of diagnosis was 38.2 (range 6-64 years); a peak incidence in early childhood was not observed. Ninety (73.8%) were women, associated systemic diseases were found in four patients. Seven cases (5.7%) presented with unilaterally affected vessels. The majority of patients (71; 58.2%) were graded Suzuki Score 3. One 14-year-old boy with moyamoya presented with a primitive trigeminal artery (0.89%). We did not find a bimodal age distribution, but only a second peak during adulthood. Unlike previous studies on Asian moyamoya patients, our collective does not exhibit a higher prevalence of persistent primitive arteries than the normal population.

  16. Gastroesophageal reflux disease in pediatric practice: current topical issues

    Directory of Open Access Journals (Sweden)

    O.Yu. Bielousova

    2017-03-01

    Full Text Available In recent years, much attention has been paid to the upper digestive tract diseases in children, particularly gastroesophageal reflux disease, as a cause that has an impact on the quality of life, even in children of school age, and thereafter in young adults. Consequently, there are searches for optimization of early detection, new me-thods of non-invasive diagnosis, screening of this pathology in children’s population in order to determine persons with risk factors and to control disease development and complicated course, as well as searches for the formation of preventive activities algorithm. Scientists came to a consensus that all examinations, which are used in pediatric practice, must be maximally available, simple and non-invasive to the extent of child’s condition. The question about advisability of performing esophagogastroduodenoscopy for all patients with complaints of heartburn and with other symptoms of gastroesophageal reflux disease, the question relative to performing ultrasonography of the esophagus in children as an additional method of examination, usage of questionnaire in pediatric practice, formation of disease course prediction algorithm, and identification of preventive measures specific to every patient remain open. In order to explain their application, the developmental mechanisms of this pathology must be well-understood, and individual risk factors that may influence disease severity and disease course prediction, which occur in children in different periods of life, must be taken into account. Therefore, the goal of this research is to provide an overview of modern literature with reference to topical issues of clinical evidence, risk factors, diagnosis, prediction of gastroesophageal reflux disease course in children of different ages (regarding main causative and pathogenic factors, clinical evidence (esophageal and extra-esophageal, diagnostic methods and modern approaches to gastroesophageal reflux disease

  17. Vedolizumab Therapy in Severe Pediatric Inflammatory Bowel Disease.

    Science.gov (United States)

    Conrad, Máire A; Stein, Ronen E; Maxwell, Elizabeth C; Albenberg, Lindsey; Baldassano, Robert N; Dawany, Noor; Grossman, Andrew B; Mamula, Petar; Piccoli, David A; Kelsen, Judith R

    2016-10-01

    Vedolizumab is effective for inducing and maintaining remission in adults with inflammatory bowel disease (IBD); however, there is limited pediatric data. This study aimed to describe the adverse events and clinical response to vedolizumab in refractory pediatric IBD. Disease activity indices, clinical response, concomitant medication use, and adverse events were measured over 22 weeks in an observational prospective cohort study of children with refractory IBD who had failed anti-tumor necrosis factor therapy and subsequently initiated vedolizumab therapy. Twenty-one subjects, 16 with Crohn disease, received vedolizumab. Clinical response was observed in 6/19 (31.6%) of the evaluable subjects at week 6 and in 11/19 (57.9%) by week 22. Before induction, 15/21 (71.4%) participants were treated with systemic corticosteroids, as compared with 7/21 (33.3%) subjects at 22 weeks. Steroid-free remission was seen in 1/20 (5.0%) subjects at 6 weeks, 3/20 (15.0%) at 14 weeks, and 4/20 (20.0%) at 22 weeks. There was statistically significant improvement in serum albumin and hematocrit; however, C-reactive protein increased by week 22 (P < 0.05). There were no infusion reactions. Vedolizumab was discontinued in 2 patients because of severe colitis, requiring surgical intervention. There is limited experience with vedolizumab therapy in pediatric IBD. There seems to be a marked number of subjects with clinical response in the first 6 weeks that increases further by week 22 despite the severity of disease in this cohort. Adverse events may not be directly related to vedolizumab. This study is limited by small sample size, and larger prospective studies are warranted.

  18. Antibiotic prophylaxis for children with sickle cell disease: a survey of pediatric dentistry residency program directors and pediatric hematologists.

    Science.gov (United States)

    Tate, Anupama Rao; Norris, Chelita Kaye; Minniti, Caterina P

    2006-01-01

    The purposes of this study were to: (1) investigate the current clinical practice regarding the use of antibiotic prophylaxis by pediatric dentistry residency program directors and pediatric hematologists for children with sickle cell disease (SCD) requiring dental treatment; and (2) evaluate the perceived relative risk of bacteremia following specific dental procedures, as defined by pediatric dentistry residency program directors and pediatric hematologists. A written survey depicting various clinical scenarios of SCD children requiring common dental procedures was mailed to directors of pediatric dental advanced education programs and distributed to pediatric hematologists attending the 2003 Annual Sickle Cell Disease Association of America conference in Washington, DC. Surveys were returned by 60% (N=34/57) of the pediatric dentistry residency program directors. The surveys were obtained from 51% of pediatric hematologists at the meeting (N=72/140). At least 50% of all respondents recommended prophylaxis for the following clinical situations: dental extractions, treatment under general anesthesia, and status post splenectomy. The perceived risk of infectious complication was highest for extractions, followed by restorative treatment and tooth polishing. Dental residency program directors were more likely (71%, N=24/34) to recommend additional antibiotic therapy for patients taking penicillin prophylaxis if they required an invasive oral surgical procedure. Conversely, only 38% (N=25/66) of pediatric hematologists recommended additional antibiotic therapy (P=.001). Eighty-six percent of dental residency program directors (N=25/29) chose amoxicillin for prophylaxis whereas only 62% of pediatric hematologists (N=36/58) recommended amoxicillin. (Pchildren undergoing dental treatments. Further research and risk/benefit assessment is needed to create a unified approach.

  19. Everything is connected: social determinants of pediatric health and disease.

    Science.gov (United States)

    Tarazi, Carine; Skeer, Margie; Fiscella, Kevin; Dean, Stephanie; Dammann, Olaf

    2016-01-01

    Carine Tarazi, MA, is an Assistant Editor for Pediatric Research in Boston, Massachusetts, USA. Margie Skeer, ScD, MPH, MSW, served as a Guest Editor for this special issue. Dr. Skeer is Assistant Professor of Public Health and Community Medicine at Tufts University. Her research focuses on adolescent substance misuse and sexual risk prevention, both from epidemiologic and intervention-development perspectives. Kevin Fiscella, MD, MPH, served as a Guest Editor for this special issue. Dr. Fiscella is Tenured Professor of Family Medicine, Public Health Sciences and Community Health at the University of Rochester Medical Center. Dr. Fiscella's research focuses on health and health care disparities, particularly practical strategies to improve health equity. Stephanie Dean, MBA, is Managing Editor of Pediatric Research and is based out of editorial office in The Woodlands, Texas. Olaf Dammann, MD, served as a Guest Editor for this special issue. Dr. Dammann is a Professor of Public Health and Community Medicine, Pediatrics, and Ophthalmology at Tufts University School of Medicine in Boston, Massachusetts, USA, as well as Professor of Perinatal Neuroepidemiology at Hannover Medical School, Hannover, Germany. His research interests include the elucidation of risk factors for brain damage and retinopathy in preterm newborns, the theory of risk and causation in biomedical and public health research, and the development of computational chronic disease models.

  20. Our experience with percutaneous nephrolithotomy in pediatric renal stone disease.

    Science.gov (United States)

    Oral, İlknur; Nalbant, İsmail; Öztürk, Ufuk; Can Şener, Nevzat; Yeşil, Süleyman; Göksel Göktuğ, H N; Abdurrahim İmamoğlu, M

    2013-03-01

    In this paper, we present our experience with percutaneous nephrolithotomy (PNL) in a pediatric patient group. From June 2007 to September 2010, we performed PNL on 57 pediatric patients. children with a mean age of 7.56 (1-15) years. Study population consisted of 30 male, and 27 female children with a mean age of 7.56 (1-5) years. Mean stone burden was calculated to be 312.2 (95-1550) mm(2). Percutaneous access was performed under fluoroscopy. Tract dilatation was accomplished with 20 F Amplatz dilators. Pneumatic lithotripsy was used to fragment the renal calculi. Mean operating time was 34 (3-80) minutes. With a single session of PNL, complete stone-free rates were achieved in 55 (96.4%) patients. Residual fragments were remained in 2 (3.5%) patients. Two patients had a febrile episode without signs and symptoms of bacteremia. Subcostal access was used in all of the patients, and none of the patients had any complications. Based on our experience, we conclude that PNL is a safe and effective method in the management of pediatric stone disease.

  1. Recent advances in delivery mechanisms for aerosol therapy during pediatric respiratory diseases.

    Science.gov (United States)

    Wu, Yue'E; Zhang, Chonglin; Zhen, Qing

    2018-04-01

    The treatment of pediatric surgery diseases via utilization of aerosol delivery mechanisms is in progress for the betterment of pediatric care. Over the years, aerosol therapy has come to play an integral role in the treatment of pediatric respiratory diseases. Inhaled aerosol agents such as bronchodilators, corticosteroids, antibiotics, and mucolytics are commonly delivered to spontaneously breathing pediatric patients with a tracheostomy. Administering therapeutic inhaled aerosols to pediatric patients is challenging. The pediatric population ranges in age, which means patients with different airway sizes, breathing patterns, and cooperation levels. These patient-related factors impact the deposition of aerosol drugs in the lungs. The present review article will discuss the recent advancements in the delivery mechanisms for aerosol therapy in pediatric patients with respiratory diseases.

  2. Chronic subdural hematoma associated with moyamoya phenomenon after radiotherapy for medulloblastoma; A case report

    Energy Technology Data Exchange (ETDEWEB)

    Fuse, Takahisa; Takagi, Takuji; Fukushima, Tsuneyuki; Mizuno, Shiroh; Hashimoto, Nobukazu; Suzuki, Osamu (Nagoya City Higashi General Hospital (Japan))

    1994-04-01

    A 9-year-old boy had been diagnosed at the age of 9 months as having a cerebellar medulloblastoma and had received 40 Gy of radiation therapy to the brain after removal of the tumor. Cerebral angiography at the time of initial diagnosis did not show any evidence of occlusive disease involving the internal carotid circulation. At the age of 6 years, the patient developed generalized seizures. On examination, he was drowsy and had right hemiparesis. CT scan demonstrated a low-density area in the left frontal lobe. Cerebral angiography showed a marked narrowing of the bilateral internal carotid arteries with moyamoya vessels. The patient was treated medically with aspirin (100 mg/day) and anticonvulsants. His neurological deficits improved gradually. At the age of 8 years, there was no recurrence of the tumor although a slight left subdural hematoma was seen on CT scan. On August 10, 1993, at the age of 9 years, he was admitted for treatment of a developing subdural hematoma. MRI showed a chronic subdural hematoma with thick outer and inner membranes. Cerebral angiography showed occlusion of the left internal carotid artery which fed the right frontal lobe through moyamoya vessels, marked narrowing of the right internal carotid artery distal to the ophthalmic artery, moyamoya vessels at the base, and cortical revascularization througth the ophthalmic, posterior cerebral and middle meningeal arteries. Trepanation and aspiration of the hematoma were performed. The outer membrane of the hematoma was about 2 mm thick and the hematoma cavity was filled with a partially organized hematoma. In this case, we speculate that development of the chronic subdural hematoma involved the following factors: (1) transdural external-internal carotid anastomosis after radiation-induced cerebrovasculopathy; (2) repeated mild head trauma due to gait disturbance after removal of the cerebellar tumor; and (3) administration of acetylsalicylic acid. (author).

  3. TNF promoter polymorphisms and modulation of growth retardation and disease severity in pediatric Crohn's disease.

    Science.gov (United States)

    Levine, Arie; Shamir, Raanan; Wine, Eytan; Weiss, Batya; Karban, Amir; Shaoul, Ron R; Reif, Shimon S; Yakir, Benjamin; Friedlander, Marcello; Kaniel, Yael; Leshinsky-Silver, Esther

    2005-07-01

    Delayed growth is common in pediatric Crohn's disease (CD). Multiple factors have been shown to affect growth in this situation, the most prominent being the presence and severity of inflammation and inadequate nutritional intake. Inflammation, anorexia, and weight loss are all manifestations of circulating TNF-alpha, which is elevated in CD. The ability to secrete TNF-alpha may be affected by polymorphisms in the TNF-alpha promoter. The aim of our study was to determine whether growth retardation and disease severity in pediatric onset CD are affected by TNF promoter genotype. Genotyping for TNF-alpha and NOD2/CARD15 single nucleotide polymorphisms was performed in 87 patients with detailed growth records. Parameters including disease location and disease severity were recorded, and the effect of these polymorphisms on Z-scores for height and weight at disease onset and during follow-up were analyzed. Lower age of onset was linked to more height retardation, while the presence of colonic disease and the absence of ileal disease were more likely to predict the absence of growth retardation. The presence of two polymorphisms thought to decrease circulating TNF-alpha was associated with higher mean Z-scores for height and a trend toward less growth retardation. Two other polymorphisms were modestly associated with disease severity. Polymorphisms in the TNF-alpha promoter may independently modulate growth and disease severity in pediatric onset CD. The effect of these polymorphisms does not appear to be mediated via weight loss, and is relatively modest.

  4. ANTISECRETORY TREATMENT FOR PEDIATRIC GASTROESOPHAGEAL REFLUX DISEASE - A SYSTEMATIC REVIEW.

    Science.gov (United States)

    Mattos, Ângelo Zambam de; Marchese, Gabriela Meirelles; Fonseca, Bárbara Brum; Kupski, Carlos; Machado, Marta Brenner

    2017-12-01

    Proton pump inhibitors and histamine H2 receptor antagonists are two of the most commonly prescribed drug classes for pediatric gastroesophageal reflux disease, but their efficacy is controversial. Many patients are treated with these drugs for atypical manifestations attributed to gastroesophageal reflux, even that causal relation is not proven. To evaluate the use of proton pump inhibitors and histamine H2 receptor antagonists in pediatric gastroesophageal reflux disease through a systematic review. A systematic review was performed, using MEDLINE, EMBASE and Cochrane Central Register of Controlled Trials databases. The search was limited to studies published in English, Portuguese or Spanish. There was no limitation regarding date of publication. Studies were considered eligible if they were randomized-controlled trials, evaluating proton pump inhibitors and/or histamine H2 receptor antagonists for the treatment of pediatric gastroesophageal reflux disease. Studies published only as abstracts, studies evaluating only non-clinical outcomes and studies exclusively comparing different doses of the same drug were excluded. Data extraction was performed by independent investigators. The study protocol was registered at PROSPERO platform (CRD42016040156). After analyzing 735 retrieved references, 23 studies (1598 randomized patients) were included in the systematic review. Eight studies demonstrated that both proton pump inhibitors and histamine H2 receptor antagonists were effective against typical manifestations of gastroesophageal reflux disease, and that there was no evidence of benefit in combining the latter to the former or in routinely prescribing long-term maintenance treatments. Three studies evaluated the effect of treatments on children with asthma, and neither proton pump inhibitors nor histamine H2 receptor antagonists proved to be significantly better than placebo. One study compared different combinations of omeprazole, bethanechol and placebo for the

  5. A Pediatric Case of Cowden Syndrome with Graves’ Disease

    Directory of Open Access Journals (Sweden)

    Cláudia Patraquim

    2017-01-01

    Full Text Available Cowden syndrome (CS is a rare dominantly inherited multisystem disorder, characterized by an extraordinary malignant potential. In 80% of cases, the human tumor suppressor gene phosphatase and tensin homolog (PTEN is mutated. We present a case of a 17-year-old boy with genetically confirmed CS and Graves’ disease (GD. At the age of 15, he presented with intention tremor, palpitations, and marked anxiety. On examination, he had macrocephaly, coarse facies, slight prognathism, facial trichilemmomas, abdominal keratoses, leg hemangioma, and a diffusely enlarged thyroid gland. He started antithyroid drug (ATD therapy with methimazole and, after a 2-year treatment period without achieving a remission status, a total thyroidectomy was performed. Diagnosis and management of CS should be multidisciplinary. Thyroid disease is frequent, but its management has yet to be fully defined. The authors present a case report of a pediatric patient with CS and GD and discuss treatment options.

  6. Clinical Features and Outcome of Ebola Virus Disease in Pediatric Patients

    DEFF Research Database (Denmark)

    Damkjær, Mads; Rudolf, Frauke; Mishra, Sharmistha

    2016-01-01

    Clinical and outcome data on pediatric Ebola virus disease are limited. We report a case-series of 33 pediatric patients with Ebola virus disease in a single Ebola Treatment Center in 2014-2015. The case-fatality rate was 42%, with the majority of deaths occurring within 10 days of admission....

  7. Low Hepatic Tissue Copper in Pediatric Nonalcoholic Fatty Liver Disease.

    Science.gov (United States)

    Mendoza, Michael; Caltharp, Shelley; Song, Ming; Collin, Lindsay; Konomi, Juna V; McClain, Craig J; Vos, Miriam B

    2017-07-01

    Animal models and studies in adults have demonstrated that copper restriction increases severity of liver injury in nonalcoholic fatty liver disease (NAFLD). This has not been studied in children. We aimed to determine if lower tissue copper is associated with increased NAFLD severity in children. This was a retrospective study of pediatric patients who had a liver biopsy including a hepatic copper quantitation. The primary outcome compared hepatic copper concentration in NAFLD versus non-NAFLD. Secondary outcomes compared hepatic copper levels against steatosis, fibrosis, lobular inflammation, balloon degeneration, and NAFLD activity score (NAS). The study analysis included 150 pediatric subjects (102 with NAFLD and 48 non-NAFLD). After adjusting for age, body mass index z score, gamma glutamyl transferase, alanine aminotransferase, and total bilirubin, NAFLD subjects had lower levels of hepatic copper than non-NAFLD (P = 0.005). In addition, tissue copper concentration decreased as steatosis severity increased (P steatosis alone. Further studies are needed to explore the relationship between copper levels and NAFLD progression.

  8. Pediatrics

    Science.gov (United States)

    Spackman, T. J.

    1978-01-01

    The utilization of the Lixiscope in pediatrics was investigated. The types of images that can presently be obtained are discussed along with the problems encountered. Speculative applications for the Lixiscope are also presented.

  9. Pediatrics

    NARCIS (Netherlands)

    Rasheed, Shabana; Teo, Harvey James Eu Leong; Littooij, Annemieke Simone

    2015-01-01

    Imaging of pediatric patients involves many diverse modalities, including radiography, ultrasound imaging, computed tomography, magnetic resonance imaging, and scintigraphic and angiographic studies. It is therefore important to be aware of potential pitfalls that may be related to these modalities

  10. Association Between Newborn Metabolic Profiles and Pediatric Kidney Disease

    Directory of Open Access Journals (Sweden)

    Manish M. Sood

    2018-05-01

    Full Text Available Introduction: Metabolomics offers considerable promise in early disease detection. We set out to test the hypothesis that routine newborn metabolic profiles at birth, obtained through screening for inborn errors of metabolism, would be associated with kidney disease and add incremental information to known clinical risk factors. Methods: We conducted a population-level cohort study in Ontario, Canada, using metabolic profiles from 1,288,905 newborns from 2006 to 2015. The primary outcome was chronic kidney disease (CKD or dialysis. Individual metabolites and their ratio combinations were examined by logistic regression after adjustment for established risk factors for kidney disease and incremental risk prediction measured. Results: CKD occurred in 2086 (0.16%, median time 612 days and dialysis in 641 (0.05%, median time 99 days infants and children. Individual metabolites consisted of amino acids, acylcarnitines, markers of fatty acid oxidation, and others. Base models incorporating clinical risk factors only provided c-statistics of 0.61 for CKD and 0.70 for dialysis. The addition of identified metabolites to risk prediciton models resulted in significant incremental improvement in the performance of both models (CKD model: c-statistic 0.66 NRI 0.36 IDI 0.04, dialysis model: c-statistic 0.77 NRI 0.57 IDI 0.09. This was consistent after internal validation using bootstrapping and a sensitivity analysis excluding outcomes within the first 30 days. Conclusion: Routinely collected screening metabolites at birth are associated with CKD and the need for dialytic therapies in infants and children, and add incremental information to traditional clinical risk factors. Keywords: chronic kidney disease, dialysis, end-stage kidney disease, metabolomics, newborn screening, pediatric, renal failure

  11. Anti-thyroid drugs in pediatric Graves′ disease

    Directory of Open Access Journals (Sweden)

    Mathew John

    2015-01-01

    Full Text Available Graves′ disease is the most common cause of hyperthyroidism in children. Most children and adolescents are treated with anti-thyroid drugs as the initial modality. Studies have used Methimazole, Carbimazole and Propylthiouracil (PTU either as titration regimes or as block and replacement regimes. The various studies of anti-thyroid drug (ATD treatment of Graves′ disease in pediatric patients differ in terms of the regimes, remission rate, duration of therapy for adequate remission, follow up and adverse effects of ATD. Various studies show that lower thyroid hormone levels, prolonged duration of treatment, lower levels of TSH receptor antibodies, smaller goiter and increased age of child predicted higher chance of remission after ATD. A variable number of patients experience minor and major adverse effects limiting initial and long term treatment with ATD. The adverse effects of various ATD seem to more in children compared to that of adults. In view of liver injury including hepatocellular failure need of liver transplantation associated with PTU, the use has been restricted in children. The rate of persistent remission with ATD following discontinuation is about 30%. Radioactive iodine therapy is gaining more acceptance in older children with Graves′s disease in view of the limitations of ATD. For individual patients, risk-benefit ratio of ATD should be weighed against benefits of radioactive iodine therapy and patient preferences.

  12. Síndrome de Down e Moyamoya: estudo através de metanálise Moyamoya and Down syndrome: study conducted by meta-analysis

    Directory of Open Access Journals (Sweden)

    Paulo Alves Junqueira

    2002-06-01

    Full Text Available Apresentamos o estudo clínico-epidemiológico de dois pacientes e metanálise (período 1977-2000 sobre a comorbidade entre síndrome de Down (SD e síndrome de "moyamoya" (SMM. Entre os 42 pacientes catalogados no presente estudo, a metanálise permitiu verificar: maior número de publicações de pesquisadores do Japão e Estados Unidos, seguidos pelo Brasil e Itália; predomínio do acidente vascular cerebral (AVC em lactentes e pré-escolares; sintomatologia inaugural, hemiparesia (78,6%, distúrbio da fala (26,2%; predomínio de infarto isquêmico (76,2%; episódios isquêmicos recorrentes (62%; comprometimento bilateral (83,3%. Esta análise permitiu concluir que, na investigação clínico-neurológica de pacientes com SD e episódios de hemiparesia aguda, a SMM deve ser incluída como diagnóstico mais provável .We present a clinic-epidemiological study of two patients and meta-analysis (period 1977-2000 of the co-morbidity of the Down syndrome (DS and moyamoya syndrome (MMS. Among the 42 patients listed in this survey, meta-analysis permitted to find the highest number of publications by researchers from Japan and United States, followed by Brazil and Italy; prevalence of cerebrovascular disease in suckling and pre school children; first symptomatology was hemiparesis (78.6%, speech disorders (26.2%; ischemic infarction (76.2%; recurring ischemic episodes (62%; bilateral impairment (83.3%. This analysis led to the conclusion that in the clinic-neurological investigation of DS patients with acute hemiparesis episodes, MMS should be included as the most probable diagnosis.

  13. Disease progression of acute pancreatitis in pediatric patients.

    Science.gov (United States)

    Hao, Fabao; Guo, Hongjie; Luo, Qianfu; Guo, Chunbao

    2016-05-15

    Approximately 10% of patients with acute pancreatitis (AP) progress to chronic pancreatitis. Little is known about the factors that affect recurrence of pancreatitis after an initial episode. We retrospectively investigated patients with AP, focusing on their outcomes and the predictors for disease progression. Between July 2003 and June 2015, we retrospectively enrolled first-time AP patients with medical records on disease etiology, severity (according to the Atlanta classifications), and recurrence of AP. Independent predictors of recurrent AP (RAP) and chronic pancreatitis were identified using the logistic regression model. Of the total 159 patients, 45 (28.3%) developed RAP, including two episodes of RAP in 19 patients, and 9 (5.7%) developed chronic pancreatitis. The median duration from the time of AP to the onset of RAP was 5.6 ± 2.3 months. RAP patients were identified as more common among patients with idiopathic first-time AP. The presence of severe ascites, pancreatic necrosis, and systemic complications was independent predictors of RAP in pediatric patients. Experiencing over two RAP episodes was the predictor for developing chronic pancreatitis. No influence of age or number of AP episodes was found on the occurrence of abdominal pain, pain severity, and the prevalence of any pain. Severity of first-time AP and idiopathic first-time AP are related to RAP. Recurrence increases risk for progression to chronic pancreatitis. The risk of recurrence increased with increasing numbers of AP episodes. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. REVIEW OF CELIAC DISEASE PRESENTATION IN A PEDIATRIC TERTIARY CENTRE.

    Science.gov (United States)

    Oliveira, Gracinda Nogueira; Mohan, Rajiv; Fagbemi, Andrew

    2018-01-01

    Celiac disease is an immune-mediated disorder with a multiform presentation and therefore a challenging diagnosis. Our purpose is to identify the epidemiological, clinical, laboratory and histologic characteristics of children with celiac disease at diagnosis and on follow-up. Children with previously established or newly diagnosed celiac disease, admitted in a tertiary centre in a two-year period (2014-2016) were recruited. Data was collected retrospectively from electronic medical records and clinical notes, and subsequently analysed with SPSS version 20.0. A total of 159 patients, out of 312, were included. Age ranged from 1 to 17 years (mean ± SD: 8.5±4.5 years, 69% girls). Disease presentation was classical in 60%, non-classical in 25%, subclinical in 10% and 5% classified as potential celiac disease. Non-classical and subclinical profiles had a higher mean age at presentation but not statistically significant (P-value 0.24). The most frequent gastrointestinal features at presentation were abdominal pain (58%), diarrhea (43%) and bloating (27%). A positive family history for celiac disease was present in 24% (n=35). We found anaemia in 23%, low ferritin in 63% and a moderate to severe deficiency of 25-hydroxyvitamin D in 62%. celiac disease -specific serologic testing and esophagogastroduodenoscopy were performed in 99%. Histology revealed modified Marsh 2 or 3 enteropathy in 94%, the remaining had normal histology but positive human leukocyte antigen typing. Clinical improvement at 12 months of gluten-free diet was complete in 51% and partial in 49%. IgA tTG normalized after 12-30 months of gluten-free diet in 45%. On growth assessment at diagnosis and after 12-28 months of gluten-free diet, 100% had height increase (mean ±SD: 7.11±4.43 cm) and 96% weight gain (mean ±SD: 5.60±4.91 kg). Our findings outline the diverse clinical presentations of pediatric celiac disease that should be considered irrespective of age. Increased clinician's awareness will

  15. The efficacy of the direct clinical intervention for infectious diseases by a pediatric infectious disease specialist in the pediatric ward of a tertiary medical facility without a pediatric antimicrobial stewardship program.

    Science.gov (United States)

    Hoshina, T; Yamamoto, N; Ogawa, M; Nakamoto, T; Kusuhara, K

    2017-08-01

    Antimicrobial stewardship programs (ASPs) have been introduced in most hospital complexes; however, they are not always useful for pediatric patients. The aim of this study is to investigate the efficacy of direct clinical intervention for infectious diseases by a pediatric infectious disease specialist in a tertiary medical facility without pediatric ASP. This retrospective study included 1,821 patients who were hospitalized in the pediatric ward of a large metropolitan hospital from 2010 to 2015. The clinical course, the use of intravenous antimicrobial agents and the results of a microbiological analysis were compared between the period after the beginning of direct intervention by the specialist (post-intervention period) and the previous period (pre-intervention period). In the post-intervention period, the proportion of the patients who received intravenous antimicrobial agents, the number of antimicrobial agents used for each episode, and the proportion of episodes in which an antimicrobial agent was re-administrated were significantly lower (P = 0.006, P = 0.004, P = 0.036, respectively), and the duration of antimicrobial treatment was significantly shorter (P infectious diseases specialist is useful for the treatment of infectious diseases in the pediatric ward of a tertiary medical facility without a pediatric ASP. The creation of a pediatric ASP is recommended in hospital complexes.

  16. Transition of adolescents with inflammatory bowel disease from pediatric to adult care: a survey of adult gastroenterologists

    NARCIS (Netherlands)

    Hait, Elizabeth J.; Barendse, Renée M.; Arnold, Janis H.; Valim, Clarissa; Sands, Bruce E.; Korzenik, Joshua R.; Fishman, Laurie N.

    2009-01-01

    Transition of patients with inflammatory bowel disease (IBD) from pediatric to adult providers requires preparation. Gastroenterologists for adult patients ("adult gastroenterologists") may have expectations of patients that are different from those of pediatric patients. We sought to explore the

  17. Comparison of outcomes parameters for induction of remission in new onset pediatric Crohn's disease

    DEFF Research Database (Denmark)

    Levine, Arie; Turner, Dan; Pfeffer Gik, Tamar

    2014-01-01

    BACKGROUND: Robust evaluation of induction therapies using both clinical and inflammatory outcomes in pediatric Crohn's disease (CD) are sparse. We attempted to evaluate clinical, inflammatory, and composite outcomes of induction of remission therapies (normal C reactive protein [CRP] remission) ...

  18. Patients’ Diets and Preferences in a Pediatric Population with Inflammatory Bowel Disease

    Directory of Open Access Journals (Sweden)

    Timothy J Green

    1998-01-01

    Full Text Available PURPOSE: To determine the dietary practices of the pediatric inflammatory bowel disease population at the Children's Hospital of the Hamilton Health Sciences Corporation and the reported effectiveness of those diets.

  19. Patients’ Diets and Preferences in a Pediatric Population with Inflammatory Bowel Disease

    OpenAIRE

    Green, Timothy J; Issenman, Robert M; Jacobson, Kevan

    1998-01-01

    PURPOSE: To determine the dietary practices of the pediatric inflammatory bowel disease population at the Children's Hospital of the Hamilton Health Sciences Corporation and the reported effectiveness of those diets.PATIENTS AND METHODS: A questionnaire mailed to 153 pediatric patients was returned by 125 patients (76 Crohn's disease [CD] and 49 ulcerative colitis [UC] patients) - an 82% response rate.RESULTS: The median age of respondents was 13 years, and 62% were male. Ninety per cent and ...

  20. Pediatric cardiology. Clinical and practical experiences with heart diseases of children, juveniles and young adults

    International Nuclear Information System (INIS)

    Haas, Nikolaus A.

    2011-01-01

    The book on pediatric cardiology covers the following chapters: (I) Fundamentals and diagnostics: pediatric cardiologic anamnesis, electrocardiograms, thorax X-radiography, MRT and CT of the heart, nuclear medical diagnostics, exercise tests, heart catheter examination, electrophysiological tests. (II) Leading symptoms: Cyanosis, cardiac murmur, thorax pain, palpitation, syncopes. (III) Disease pictures: congenital heart defects, acquired heart defects, cardiomyopathies, heart rhythm disturbances, heart insufficiency, arterial hypertension, pulmonary hypertension, other heart involving syndromes. (IV) Therapy: Catheter interventional therapy, post-surgical pediatric cardiac therapy, surgery involving the life-support machine, mechanical cardiovascular support systems, initial treatment of newborns with critical heart defects, heart transplantation, vaccination of children with heart diseases, medicinal therapy.

  1. Three-dimensional printing and pediatric liver disease.

    Science.gov (United States)

    Alkhouri, Naim; Zein, Nizar N

    2016-10-01

    Enthusiastic physicians and medical researchers are investigating the role of three-dimensional printing in medicine. The purpose of the current review is to provide a concise summary of the role of three-dimensional printing technology as it relates to the field of pediatric hepatology and liver transplantation. Our group and others have recently demonstrated the feasibility of printing three-dimensional livers with identical anatomical and geometrical landmarks to the native liver to facilitate presurgical planning of complex liver surgeries. Medical educators are exploring the use of three-dimensional printed organs in anatomy classes and surgical residencies. Moreover, mini-livers are being developed by regenerative medicine scientist as a way to test new drugs and, eventually, whole livers will be grown in the laboratory to replace organs with end-stage disease solving the organ shortage problem. From presurgical planning to medical education to ultimately the bioprinting of whole organs for transplantation, three-dimensional printing will change medicine as we know in the next few years.

  2. Device therapy in pediatric and congenital heart disease patients

    Directory of Open Access Journals (Sweden)

    Naokata Sumitomo, MD, PhD

    2014-12-01

    Conclusions: Despite the limited experience, limitations of device implantations owing to the size of the devices, and necessity for nonendocardial electrode placement, device implantations are required in more pediatric and CHD patients than expected.

  3. Call-Center Based Disease Management of Pediatric Asthmatics

    National Research Council Canada - National Science Library

    Quinn, James M

    2005-01-01

    .... This intervention will be compared to a control population of pediatric asthma patients receiving printed education materials and usual care at three DoD military treatment facilities in a similar geographic region...

  4. Virtual Pediatric Hospital

    Science.gov (United States)

    ... Thoracopaedia - An Imaging Encyclopedia of Pediatric Thoracic Disease Virtual Pediatric Hospital is the Apprentice's Assistant™ Last revised ... pediatric resources: GeneralPediatrics.com | PediatricEducation.org | SearchingPediatrics.com Virtual Pediatric Hospital is curated by Donna M. D' ...

  5. The impact of pediatric skin disease on self-esteem

    OpenAIRE

    K.L. Vivar, MD; L. Kruse, MD

    2018-01-01

    Background: Pediatric skin disorders can affect children’s self-esteem, relationships with caregivers and peers, and performance in school and activities. Objective: This review describes common pediatric congenital and acquired dermatologic disorders and the impact that these disorders can have on children’s self-esteem. Methods: A review of current, English-language literature was conducted with use of the PubMed database. Search terms included atopic dermatitis, acne, infantile heman...

  6. Risk Factors Associated with Childhood Strabismus: The Multi-Ethnic Pediatric Eye Disease and Baltimore Pediatric Eye Disease Studies

    Science.gov (United States)

    Cotter, Susan; Varma, Rohit; Tarczy-Hornoch, Kristina; McKean-Cowdin, Roberta; Lin, Jesse; Wen, Ge; Wei, Jolyn; Borchert, Mark; Azen, Stan; Torres, Mina; Tielsch, James M.; Friedman, David S.; Repka, Michael X.; Ibironke, Joanne Katz Josephine; Giordano, Lydia

    2011-01-01

    Objective To investigate risk factors associated with esotropia or exotropia in infants and young children. Design Population-based cross-sectional prevalence study. Participants Population-based samples of 9970 children ages 6 to 72 months from California and Maryland. Methods Participants were preschool African-American, Hispanic, and non-Hispanic white children participating in the Multiethnic Pediatric Eye Disease Study and the Baltimore Eye Disease Study. Data were obtained by parental interview and ocular examination. Odd ratios and 95% confidence intervals were calculated to evaluate the association of demographic, behavioral, and clinical risk factors with esotropia and exotropia. Main Outcome Measures Odds ratios (ORs) for various risk factors associated with esotropia or exotropia diagnosis based on cover testing. Results In multivariate logistic regression analysis, esotropia was independently associated with prematurity, maternal smoking during pregnancy, older preschool age (48–72 months), anisometropia, and hyperopia. There was a severity-dependent association of hyperopia with the prevalence of esotropia, with ORs increasing from 6.4 for 2.00 Diopters (D) to strabismus, female sex, astigmatism (OR 2.5 for 1.50 to <2.50 D, and 5.9 for ≥ 2.5 D of astigmatism), and aniso-astigmatism in the J0 component (OR ≥ 2 for J0 aniso-astigmatism ≥ 0.25 D). Conclusions Prematurity and maternal smoking during pregnancy are associated with a higher risk of having esotropia and exotropia. Refractive error is associated in a severity-dependent manner to the prevalence of esotropia and exotropia. Because refractive error is correctable, these risk associations should be considered when developing guidelines for the screening and management of refractive error in infants and young children children. PMID:21856012

  7. Assessment of Type I Interferon Signaling in Pediatric Inflammatory Disease.

    Science.gov (United States)

    Rice, Gillian I; Melki, Isabelle; Frémond, Marie-Louise; Briggs, Tracy A; Rodero, Mathieu P; Kitabayashi, Naoki; Oojageer, Anthony; Bader-Meunier, Brigitte; Belot, Alexandre; Bodemer, Christine; Quartier, Pierre; Crow, Yanick J

    2017-02-01

    Increased type I interferon is considered relevant to the pathology of a number of monogenic and complex disorders spanning pediatric rheumatology, neurology, and dermatology. However, no test exists in routine clinical practice to identify enhanced interferon signaling, thus limiting the ability to diagnose and monitor treatment of these diseases. Here, we set out to investigate the use of an assay measuring the expression of a panel of interferon-stimulated genes (ISGs) in children affected by a range of inflammatory diseases. A cohort study was conducted between 2011 and 2016 at the University of Manchester, UK, and the Institut Imagine, Paris, France. RNA PAXgene blood samples and clinical data were collected from controls and symptomatic patients with a genetically confirmed or clinically well-defined inflammatory phenotype. The expression of six ISGs was measured by quantitative polymerase chain reaction, and the median fold change was used to calculate an interferon score (IS) for each subject compared to a previously derived panel of 29 controls (where +2 SD of the control data, an IS of >2.466, is considered as abnormal). Results were correlated with genetic and clinical data. Nine hundred ninety-two samples were analyzed from 630 individuals comprising symptomatic patients across 24 inflammatory genotypes/phenotypes, unaffected heterozygous carriers, and controls. A consistent upregulation of ISG expression was seen in 13 monogenic conditions (455 samples, 265 patients; median IS 10.73, interquartile range (IQR) 5.90-18.41), juvenile systemic lupus erythematosus (78 samples, 55 patients; median IS 10.60, IQR 3.99-17.27), and juvenile dermatomyositis (101 samples, 59 patients; median IS 9.02, IQR 2.51-21.73) compared to controls (78 samples, 65 subjects; median IS 0.688, IQR 0.427-1.196), heterozygous mutation carriers (89 samples, 76 subjects; median IS 0.862, IQR 0.493-1.942), and individuals with non-molecularly defined autoinflammation (89 samples, 69

  8. Phase I trial of sargramostim in pediatric Crohn's disease.

    Science.gov (United States)

    Kelsen, Judith R; Rosh, Joel; Heyman, Mel; Winter, Harland S; Ferry, George; Cohen, Stanley; Mamula, Petar; Baldassano, Robert N

    2010-07-01

    Improving granulocyte function may represent an effective therapy for Crohn's disease (CD). We performed a Phase I-2 trial of sargramostim (SRG) in children with CD. This was multicenter, open-label study in 6-16-year-old patients with moderate to severely active CD. Patients received either 4 or 6 microg/kg SRG subcutaneously daily for 8 weeks, with and without concomitant corticosteroids (CS). The primary endpoint was identification of a safe and tolerable dose in children. The secondary endpoint was establishment of the pharmacokinetics (PK). Efficacy, a tertiary endpoint, was measured by the Pediatric CD Activity Index (PCDAI). Response was defined as a decrease from baseline of > or =12.5 points and remission as absolute PCDAI of < or =10. In all, 22 patients were enrolled: 12 and 10 received 4 and 6 mg/kg, respectively; 19 completed the course. Both doses were found to be safe and well tolerated. Mild injection-site reactions occurred in 90% of patients. Three patients required dose reductions due to elevated absolute neutrophil counts. Following 4 microg/kg the mean area under the curve (AUC) was 2.64 and 2.80 ngh/mL for the 6-11- and 12-16-year-old groups, respectively. The mean half-life (t(1/2)) was 1.22 and 1.59 hours, respectively. Following 6 microg/kg, the mean AUC was 5.01 ngh/mL for the 12-16-year-old group, a 1.8-fold increase. A total of 16/18 patients (88%) achieved remission or response. Sargramostim at both 4 and 6 mg/kg was well tolerated. PK analysis suggested dose proportionality unaffected by CS exposure. Remission and response data are encouraging, but further trials are needed to assess efficacy.

  9. Profile of infliximab in the treatment of pediatric Crohn’s disease

    Directory of Open Access Journals (Sweden)

    Kierus J

    2015-06-01

    Full Text Available Jaroslaw Kierkus,1 Edyta Szymanska,2 Grzegorz Oracz,1 Anna Wiernicka,1 Maciej Dadalski1 1Department of Gastroenterology, Hepatology, Feeding Disorders and Pediatrics, 2Department of Pediatrics, Nutrition and Metabolic Disorders, The Children’s Memorial Health Institute, Warsaw, Poland Abstract: In recent years, a novel biologic therapy with monoclonal antibodies against tumor necrosis factor-alpha has revolutionized the treatment of Crohn’s disease. Infliximab, the first biologic agent, has been demonstrated to considerably improve both clinical and endoscopic outcomes. In view of the growing popularity of infliximab in the management of Crohn’s disease, we review the profile of the agent in the treatment of this disease in a pediatric setting. Keywords: infliximab, Crohn’s disease, children, biologic therapy, anti-TNF-agents

  10. Conn syndrome and Crohn disease in a pediatric case: an interesting parallel.

    Science.gov (United States)

    Rodriguez, Manuel A; Alkhoury, Fuad; Malvezzi, Leopoldo; Diaz, Alejandro

    2013-01-01

    Conn syndrome is characterized by surreptitious secretion of aldosterone in which patients are found to have hypertension, hypokalemia, and metabolic alkalosis. Although rare, the most common presentation in the pediatric population is bilateral hyperplasia of the adrenal glands as opposed to an adenoma. Crohn disease is part of the spectrum of inflammatory bowel disease, which manifests in children as flare-ups of bloody diarrhea and abdominal pain. The association of concurrent Conn syndrome and Crohn disease has been previously presented in two cases in adults. This is the first pediatric case to be reported in the literature.

  11. Low revision rate after total hip arthroplasty in patients with pediatric hip diseases

    DEFF Research Database (Denmark)

    Engesæter, Lars B; Engesæter, Ingvild Ø; Fenstad, Anne Marie

    2012-01-01

    Background The results of primary total hip arthroplasties (THAs) after pediatric hip diseases such as developmental dysplasia of the hip (DDH), slipped capital femoral epiphysis (SCFE), or Perthes' disease have been reported to be inferior to the results after primary osteoarthritis of the hip (OA.......9%) were operated due to pediatric hip diseases (3.1% for Denmark, 8.8% for Norway, and 1.9% for Sweden) and 288,435 THAs (77.8%) were operated due to OA. Unadjusted 10-year Kaplan-Meier survival of THAs after pediatric hip diseases (94.7% survival) was inferior to that after OA (96.6% survival......). Consequently, an increased risk of revision for hips with a previous pediatric hip disease was seen (risk ratio (RR) 1.4, 95% CI: 1.3-1.5). However, after adjustment for differences in sex and age of the patients, and in fixation of the prostheses, no difference in survival was found (93.6% after pediatric hip...

  12. Primary headaches in pediatric patients with chronic rheumatic disease.

    Science.gov (United States)

    Uluduz, Derya; Tavsanli, Mustafa Emir; Uygunoğlu, Uğur; Saip, Sabahattin; Kasapcopur, Ozgur; Ozge, Aynur; Temel, Gulhan Orekici

    2014-11-01

    To assess the presence, prevalence and clinical characteristics of primary headaches in pediatric patients with chronic rheumatic diseases such as juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF), and to analyze the common pathophysiological mechanisms. In this noncontrolled, cross-sectional study, a semi-structured 53 item headache questionnaire was administered to subjects with FMF and JIA, and interviewed a total sample size of 601 patients younger than16years of age. The questionnaires were then analyzed according to the International Headache Society's diagnostic criteria. Children with FMF (n=378) and JIA (n=223) were studied. Each group was then divided into two subgroups according to whether the subjects reported headache or not. 29.5% of subjects with FMF reported having migraine, 37.6% probable migraine and 32.9% tension type headache (TTH). In JIA group 28.2% were diagnosed with migraine; 41.2% with probable migraine and 30.6% with TTH. No significant difference was found between all subjects with (n=258) and without (n=343) headache for variables such as living in a crowded family (p=0.95), being the first child in the family (p=0.63), academic achievement of the child (p=0.63), high education level (higher than high school) of the mother (p=0.52) and father (p=0.46). The presence of systemic disease was reported not to be effecting the daily life at the time of evaluation by 90.2% of the children with headache and 91.0% of the children without headache (p=0.94). 81.4% of the children reported their headaches were not aggravating with the exacerbation periods of their systemic disease. Family history of hypertension was reported higher by the subjects with headache (13.5% with headache and 4.0% without headache p=0.001). Diabetes mellitus was also reported higher (5.8% with headache; 0.5% without headache; p=0.006). Family history of headache was reported in 28.2% of the patients with headache whereas it was 17.4% of the

  13. PedAM: a database for Pediatric Disease Annotation and Medicine.

    Science.gov (United States)

    Jia, Jinmeng; An, Zhongxin; Ming, Yue; Guo, Yongli; Li, Wei; Li, Xin; Liang, Yunxiang; Guo, Dongming; Tai, Jun; Chen, Geng; Jin, Yaqiong; Liu, Zhimei; Ni, Xin; Shi, Tieliu

    2018-01-04

    There is a significant number of children around the world suffering from the consequence of the misdiagnosis and ineffective treatment for various diseases. To facilitate the precision medicine in pediatrics, a database namely the Pediatric Disease Annotations & Medicines (PedAM) has been built to standardize and classify pediatric diseases. The PedAM integrates both biomedical resources and clinical data from Electronic Medical Records to support the development of computational tools, by which enables robust data analysis and integration. It also uses disease-manifestation (D-M) integrated from existing biomedical ontologies as prior knowledge to automatically recognize text-mined, D-M-specific syntactic patterns from 774 514 full-text articles and 8 848 796 abstracts in MEDLINE. Additionally, disease connections based on phenotypes or genes can be visualized on the web page of PedAM. Currently, the PedAM contains standardized 8528 pediatric disease terms (4542 unique disease concepts and 3986 synonyms) with eight annotation fields for each disease, including definition synonyms, gene, symptom, cross-reference (Xref), human phenotypes and its corresponding phenotypes in the mouse. The database PedAM is freely accessible at http://www.unimd.org/pedam/. © The Author(s) 2017. Published by Oxford University Press on behalf of Nucleic Acids Research.

  14. Biomarker Correlates of Survival in Pediatric Patients with Ebola Virus Disease

    Centers for Disease Control (CDC) Podcasts

    2014-08-19

    Dr. Mike Miller reads an abridged version of the article, Biomarker Correlates of Survival in Pediatric Patients with Ebola Virus Disease.  Created: 8/19/2014 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 8/19/2014.

  15. The impact of pediatric skin disease on self-esteem

    Directory of Open Access Journals (Sweden)

    K.L. Vivar, MD

    2018-03-01

    Conclusion: Pediatric dermatologic disorders impact self-esteem throughout childhood. In addition to the surgical and medical management of these disorders, clinicians can also take an active role in the assessment and improvement of the psychosocial impact of these skin disorders.

  16. Role of endoscopic ultrasound in pediatric gastrointestinal diseases

    Directory of Open Access Journals (Sweden)

    Rajiv Ranjan Singh

    2016-01-01

    Full Text Available Endoscopic ultrasound (EUS is a well-established diagnostic and therapeutic modality for adults. It is extremely helpful for a broad range of diagnostic indications including upper gastrointestinal (GI malignancies, submucosal lesions, pancreatic lesions (masses, cystic lesions, chronic pancreatitis, etiological workup of recurrent acute pancreatitis, common bile duct evaluation (calculi versus tumor, gallbladder lesions/microliths, and rectal malignancy; well-established therapeutic roles include fine-needle aspiration of lesions/lymph nodes, pancreatic fluid collection drainage, pancreatic duct drainage, biliary drainage, gallbladder drainage, pelvic abscess drainage, celiac plexus block, or neurolysis. Some recent studies have reported the use of EUS in the pediatric population. EUS is safe and easy to perform in the pediatric population also. However, there is paucity of data on use of EUS in pediatric population. In contrast with its regular therapeutic use in adults, EUS is not commonly performed in children for therapeutic reasons and most of the data are available on diagnostic use only. All of studies have shown that EUS is safe and a useful modality with a positive impact on management in majority of study population. EUS is very useful in pediatric population for the evaluation of upper GI tract submucosal lesions or rectal masses, pancreaticobiliary disorders, characterization of esophageal strictures, and for evaluation of enteric duplication cysts. The advent of miniprobe that can be passed through conventional endoscopes has increased the applicability of EUS in infants and children. Although there are limited data regarding use of EUS in pediatric population, it appears to be a very promising diagnostic and therapeutic tool.

  17. Beta-thalassemia intermedia associated with moyamoya syndrome.

    Science.gov (United States)

    Göksel, Basak Karakurum; Ozdogu, Hakan; Yildirim, Tulin; Oğuzkurt, Levent; Asma, Suheyl

    2010-07-01

    Moyamoya syndrome (MMS) is a progressive disorder. We report a 19-year-old boy with beta-thalassemia who presented with a left hemiparesis. Brain MRI showed old middle cerebral artery and left frontal subcortical white matter infarcts. Brain magnetic resonance angiography and digital subtraction angiography revealed occlusion of the bilateral internal carotid arteries with a rich network of basal collateral vessels. To our knowledge this is the third report of beta-thalassemia intermedia and MMS, and the first report of a patient in Turkey. It emphasizes the potential for cerebral infarct due to anemia, protein S and thrombocytosis.

  18. Evolution of disease phenotype in adult and pediatric onset Crohn’s disease in a population-based cohort

    Science.gov (United States)

    Lovasz, Barbara Dorottya; Lakatos, Laszlo; Horvath, Agnes; Szita, Istvan; Pandur, Tunde; Mandel, Michael; Vegh, Zsuzsanna; Golovics, Petra Anna; Mester, Gabor; Balogh, Mihaly; Molnar, Csaba; Komaromi, Erzsebet; Kiss, Lajos Sandor; Lakatos, Peter Laszlo

    2013-01-01

    AIM: To investigate the evolution of disease phenotype in adult and pediatric onset Crohn’s disease (CD) populations, diagnosed between 1977 and 2008. METHODS: Data of 506 incident CD patients were analyzed (age at diagnosis: 28.5 years, interquartile range: 22-38 years). Both in- and outpatient records were collected prospectively with a complete clinical follow-up and comprehensively reviewed in the population-based Veszprem province database, which included incident patients diagnosed between January 1, 1977 and December 31, 2008 in adult and pediatric onset CD populations. Disease phenotype according to the Montreal classification and long-term disease course was analysed according to the age at onset in time-dependent univariate and multivariate analysis. RESULTS: Among this population-based cohort, seventy-four (12.8%) pediatric-onset CD patients were identified (diagnosed ≤ 17 years of age). There was no significant difference in the distribution of disease behavior between pediatric (B1: 62%, B2: 15%, B3: 23%) and adult-onset CD patients (B1: 56%, B2: 21%, B3: 23%) at diagnosis, or during follow-up. Overall, the probability of developing complicated disease behaviour was 49.7% and 61.3% in the pediatric and 55.1% and 62.4% in the adult onset patients after 5- and 10-years of follow-up. Similarly, time to change in disease behaviour from non stricturing, non penetrating (B1) to complicated, stricturing or penetrating (B2/B3) disease was not significantly different between pediatric and adult onset CD in a Kaplan-Meier analysis. Calendar year of diagnosis (P = 0.04), ileal location (P < 0.001), perianal disease (P < 0.001), smoking (P = 0.038) and need for steroids (P < 0.001) were associated with presence of, or progression to, complicated disease behavior at diagnosis and during follow-up. A change in disease location was observed in 8.9% of patients and it was associated with smoking status (P = 0.01), but not with age at diagnosis. CONCLUSION: Long

  19. Managing acute complications of sickle cell disease in pediatric patients [digest].

    Science.gov (United States)

    Subramaniam, Sathyaseelan; Chao, Jennifer H; Chaudhari, Pradip

    2016-11-22

    Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management. Patients with sickle cell trait generally have a benign course, but are also subject to serious complications. This issue provides a current review of evidence-based management of the most common acute complications of sickle cell disease seen in pediatric patients in the emergency department. [Points & Pearls is a digest of Pediatric Emergency Medicine Practice].

  20. A Disease-Associated Microbial and Metabolomics State in Relatives of Pediatric Inflammatory Bowel Disease Patients.

    Science.gov (United States)

    Jacobs, Jonathan P; Goudarzi, Maryam; Singh, Namita; Tong, Maomeng; McHardy, Ian H; Ruegger, Paul; Asadourian, Miro; Moon, Bo-Hyun; Ayson, Allyson; Borneman, James; McGovern, Dermot P B; Fornace, Albert J; Braun, Jonathan; Dubinsky, Marla

    2016-11-01

    Microbes may increase susceptibility to inflammatory bowel disease (IBD) by producing bioactive metabolites that affect immune activity and epithelial function. We undertook a family based study to identify microbial and metabolic features of IBD that may represent a predisease risk state when found in healthy first-degree relatives. Twenty-one families with pediatric IBD were recruited, comprising 26 Crohn's disease patients in clinical remission, 10 ulcerative colitis patients in clinical remission, and 54 healthy siblings/parents. Fecal samples were collected for 16S ribosomal RNA gene sequencing, untargeted liquid chromatography-mass spectrometry metabolomics, and calprotectin measurement. Individuals were grouped into microbial and metabolomics states using Dirichlet multinomial models. Multivariate models were used to identify microbes and metabolites associated with these states. Individuals were classified into 2 microbial community types. One was associated with IBD but irrespective of disease status, had lower microbial diversity, and characteristic shifts in microbial composition including increased Enterobacteriaceae, consistent with dysbiosis. This microbial community type was associated similarly with IBD and reduced microbial diversity in an independent pediatric cohort. Individuals also clustered bioinformatically into 2 subsets with shared fecal metabolomics signatures. One metabotype was associated with IBD and was characterized by increased bile acids, taurine, and tryptophan. The IBD-associated microbial and metabolomics states were highly correlated, suggesting that they represented an integrated ecosystem. Healthy relatives with the IBD-associated microbial community type had an increased incidence of elevated fecal calprotectin. Healthy first-degree relatives can have dysbiosis associated with an altered intestinal metabolome that may signify a predisease microbial susceptibility state or subclinical inflammation. Longitudinal prospective

  1. Regional cerebral blood flow in various pediatric neurological patients using 123I-IMP SPECT

    International Nuclear Information System (INIS)

    Konishi, Tohru; Naganuma, Yoshihiro; Hongou, Kazuhisa; Murakami, Miyako; Yamatani, Miwa; Okada, Toshio

    1988-01-01

    The recent development of a new radiopharmaceutical 123 I-isopropyl-iodoamphetamine (IMP), which is taken up by the brain from the blood flow, has offered a possibility of constructing scintigraphy maps of regional cerebral blood flow (rCBF) using single photon emission CT. We used this mehtod in various pediatric neurological diseases. Six patients with cerebro-vascular disorders (moya-moya disease 2, infarction 3 and HHE syndrome 1), 6 patients with infectious diseases of CNS (acute encephalitis 4, septic meningitis 1 and SSPE 1) and a miscellaneous group of six patients were studied. The rCBF abnormalities in cerebro-vascular diseases were more extensive and frequent than x-ray CT abnormalities. Repeated studies of IMP-SPECT revealed usefulness for the understanding of changeable hemodynamic pathophysiology and for the judgment of theraptic effectiveness and prognosis. The rCBF decrease in infectious diseases tended to be more diffuse and slight than that in cerebro-vascular diseases. In almost all patients, the area of rCBF decrease coincided with the area of EEG slowing evaluated by EEG topographic analysis. Brain imaging using 123 I-IMP SPECT may reveal functional abnormalities as well as organic lesions. 123 I-IMP SPECT has introduced a new era for the useful application of nuclear medicine to the investigation of pediatric neurological diseases. (author)

  2. Regional cerebral blood flow in various pediatric neurological patients using /sup 123/I-IMP SPECT

    Energy Technology Data Exchange (ETDEWEB)

    Konishi, Tohru; Naganuma, Yoshihiro; Hongou, Kazuhisa; Murakami, Miyako; Yamatani, Miwa; Okada, Toshio

    1988-03-01

    The recent development of a new radiopharmaceutical /sup 123/I-isopropyl-iodoamphetamine (IMP), which is taken up by the brain from the blood flow, has offered a possibility of constructing scintigraphy maps of regional cerebral blood flow (rCBF) using single photon emission CT. We used this mehtod in various pediatric neurological diseases. Six patients with cerebro-vascular disorders (moya-moya disease 2, infarction 3 and HHE syndrome 1), 6 patients with infectious diseases of CNS (acute encephalitis 4, septic meningitis 1 and SSPE 1) and a miscellaneous group of six patients were studied. The rCBF abnormalities in cerebro-vascular diseases were more extensive and frequent than x-ray CT abnormalities. Repeated studies of IMP-SPECT revealed usefulness for the understanding of changeable hemodynamic pathophysiology and for the judgment of theraptic effectiveness and prognosis. The rCBF decrease in infectious diseases tended to be more diffuse and slight than that in cerebro-vascular diseases. In almost all patients, the area of rCBF decrease coincided with the area of EEG slowing evaluated by EEG topographic analysis. Brain imaging using /sup 123/I-IMP SPECT may reveal functional abnormalities as well as organic lesions. /sup 123/I-IMP SPECT has introduced a new era for the useful application of nuclear medicine to the investigation of pediatric neurological diseases.

  3. Comparison of MR enterography and histopathology in the evaluation of pediatric Crohn disease

    Energy Technology Data Exchange (ETDEWEB)

    Dillman, Jonathan R.; Ladino-Torres, Maria F.; Strouse, Peter J. [University of Michigan Health System, Department of Radiology, C.S. Mott Children' s Hospital, Section of Pediatric Radiology, Ann Arbor, MI (United States); Adler, Jeremy; DeMatos-Malliard, Vera [University of Michigan Health System, Department of Pediatrics and Communicable Diseases, C. S. Mott Children' s Hospital, Division of Pediatric Gastroenterology, Ann Arbor, MI (United States); McHugh, Jonathan B. [University of Michigan Health System, Department of Pathology, Ann Arbor, MI (United States); Khalatbari, S. [University of Michigan Institute for Clinical Health Research (MICHR), Ann Arbor, MI (United States)

    2011-12-15

    While MR enterography (MRE) is commonly used to identify segments of bowel affected by Crohn disease in children, there is little data to support this practice. To assess the ability of MRE to identify segments of small bowel and colon affected by pediatric Crohn disease using histopathology as the reference standard. Pediatric MRE examinations performed between April 2009 and December 2010 were identified. Subjects were excluded if they did not have Crohn disease, if they had Crohn disease but no correlative histopathology within 2 months of imaging, or if they had undergone prior bowel resection. Pertinent MRE and histopathology reports were reviewed, and normal and abnormal bowel segments were documented. Thirty-two pediatric MRE examinations were identified with correlative histopathology. MRE had an overall sensitivity of 94% for detecting the presence of Crohn disease, in general. At the bowel segmental level, MRE had a sensitivity of 66%, specificity of 90%, positive predictive value of 85% and negative predictive value of 76%. The terminal ileum was abnormal by MRE in 11 of 15 (73%) subjects lacking a diagnostic biopsy of this bowel segment. MRE successfully identifies small bowel and colon segments affected by pediatric Crohn disease. (orig.)

  4. Parenting stress in pediatric IBD: relations with child psychopathology, family functioning, and disease severity.

    Science.gov (United States)

    Gray, Wendy N; Graef, Danielle M; Schuman, Shana S; Janicke, David M; Hommel, Kevin A

    2013-05-01

    Parenting stress in pediatric inflammatory bowel disease (IBD) has been under-examined. Data validating use of the Pediatric Inventory for Parents (PIP), a measure of parenting stress associated with caring for a chronically ill child, in chronic diseases with intermittent, unpredictable disease courses, such as IBD, are needed. This study presents validity data in support of the PIP in pediatric IBD and examines relations between parenting stress and important psychosocial and medical outcomes. Adolescents (N = 130) with IBD and their caregivers across 3 sites completed measures of parenting stress, family functioning, and emotional/behavioral functioning. Disease severity was also assessed for each participant. The PIP demonstrates excellent internal consistency. Parenting stress was significantly higher among those with unhealthy general family functioning and those with children with borderline or clinically elevated internalizing symptoms. Caregiving stress was greater among parents of youth with more active Crohn's disease. Results supported the reliability and validity of the PIP for assessing caregiving stress in pediatric IBD. Routine assessment of parenting stress is recommended, particularly among parents reporting unhealthy family functioning and parents of youth with borderline or clinically elevated internalizing symptoms and more active disease.

  5. The impact of pediatric skin disease on self-esteem.

    Science.gov (United States)

    Vivar, K L; Kruse, L

    2018-03-01

    Pediatric skin disorders can affect children's self-esteem, relationships with caregivers and peers, and performance in school and activities. This review describes common pediatric congenital and acquired dermatologic disorders and the impact that these disorders can have on children's self-esteem. A review of current, English-language literature was conducted with use of the PubMed database. Search terms included atopic dermatitis, acne, infantile hemangiomas, port wine stains, congenital melanocytic nevi, hidradenitis suppurativa, and self-esteem. During infancy and toddlerhood, skin disorders such as infantile hemangiomas primarily affect the attachment between child and caregiver. School-aged children with port wine stains and atopic dermatitis report increased bullying, teasing, and social isolation. Acne and hidradenitis typically affect older children and teens and these conditions are associated with increased risks of depression and suicidal ideation. Effective management of these conditions has been shown to increase patients' self-esteem. Pediatric dermatologic disorders impact self-esteem throughout childhood. In addition to the surgical and medical management of these disorders, clinicians can also take an active role in the assessment and improvement of the psychosocial impact of these skin disorders.

  6. Fructose Induced Endotoxemia in Pediatric Nonalcoholic Fatty Liver Disease

    Directory of Open Access Journals (Sweden)

    Ran Jin

    2014-01-01

    Full Text Available In preclinical studies of fructose-induced NAFLD, endotoxin appears to play an important role. We retrospectively examined samples from three pediatric cohorts (1 to investigate whether endotoxemia is associated with the presence of hepatic steatosis; (2 to evaluate postprandial endotoxin levels in response to fructose beverage in an acute 24-hour feeding challenge, and (3 to determine the change of fasting endotoxin amounts in a 4-week randomized controlled trial comparing fructose to glucose beverages in NAFLD. We found that adolescents with hepatic steatosis had elevated endotoxin levels compared to obese controls and that the endotoxin level correlated with insulin resistance and several inflammatory cytokines. In a 24-hour feeding study, endotoxin levels in NAFLD adolescents increased after fructose beverages (consumed with meals as compared to healthy children. Similarly, endotoxin was significantly increased after adolescents consumed fructose beverages for 2 weeks and remained high although not significantly at 4 weeks. In conclusion, these data provide support for the concept of low level endotoxemia contributing to pediatric NAFLD and the possible role of fructose in this process. Further studies are needed to determine if manipulation of the microbiome or other methods of endotoxin reduction would be useful as a therapy for pediatric NAFLD.

  7. Nutrition in Pediatric Inflammatory Bowel Disease: A Position Paper on Behalf of the Porto Inflammatory Bowel Disease Group of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition.

    Science.gov (United States)

    Miele, Erasmo; Shamir, Raanan; Aloi, Marina; Assa, Amit; Braegger, Christian; Bronsky, Jiri; de Ridder, Lissy; Escher, Johanna C; Hojsak, Iva; Kolaček, Sanja; Koletzko, Sibylle; Levine, Arie; Lionetti, Paolo; Martinelli, Massimo; Ruemmele, Frank; Russell, Richard K; Boneh, Rotem Sigall; van Limbergen, Johan; Veereman, Gigi; Staiano, Annamaria

    2018-04-01

    A growing body of evidence supports the need for detailed attention to nutrition and diet in children with inflammatory bowel disease (IBD). We aimed to define the steps in instituting dietary or nutritional management in light of the current evidence and to offer a useful and practical guide to physicians and dieticians involved in the care of pediatric IBD patients. A group of 20 experts in pediatric IBD participated in an iterative consensus process including 2 face-to-face meetings, following an open call to Nutrition Committee of the European Society of Pediatric Gastroenterology, Hepatology and Nutrition Porto, IBD Interest, and Nutrition Committee. A list of 41 predefined questions was addressed by working subgroups based on a systematic review of the literature. A total of 53 formal recommendations and 47 practice points were endorsed with a consensus rate of at least 80% on the following topics: nutritional assessment; macronutrients needs; trace elements, minerals, and vitamins; nutrition as a primary therapy of pediatric IBD; probiotics and prebiotics; specific dietary restrictions; and dietary compounds and the risk of IBD. This position paper represents a useful guide to help the clinicians in the management of nutrition issues in children with IBD.

  8. Identifying Local Hotspots of Pediatric Chronic Diseases Using Emergency Department Surveillance

    Science.gov (United States)

    Lee, David C.; Yi, Stella S.; Fong, Hiu-Fai; Athens, Jessica K.; Ravenell, Joseph E.; Sevick, Mary Ann; Wall, Stephen P.; Elbel, Brian

    2016-01-01

    Objective To use novel geographic methods and large-scale claims data to identify the local distribution of pediatric chronic diseases in New York City. Methods Using a 2009 all-payer emergency claims database, we identified the proportion of unique children aged 0 to 17 with diagnosis codes for specific medical and psychiatric conditions. As a proof of concept, we compared these prevalence estimates to traditional health surveys and registry data using the most geographically granular data available. In addition, we used home addresses to map local variation in pediatric disease burden. Results We identified 549,547 New York City children who visited an emergency department at least once in 2009. Though our sample included more publicly insured and uninsured children, we found moderate to strong correlations of prevalence estimates when compared to health surveys and registry data at pre-specified geographic levels. Strongest correlations were found for asthma and mental health conditions by county among younger children (0.88, p=0.05 and 0.99, pdisease prevalence with higher geographic resolution. More studies are needed to investigate limitations of these methods and assess reliability of local disease estimates. What’s New This study demonstrated how emergency department surveillance may improve estimates of pediatric disease prevalence with higher geographic resolution. We identified 29% of New York City children with a single year of data and identified local hotspots of pediatric chronic diseases. PMID:28385326

  9. Methotrexate for the Treatment of Pediatric Crohn's Disease: A Systematic Review and Meta-analysis.

    Science.gov (United States)

    Colman, Ruben J; Lawton, Rachel C; Dubinsky, Marla C; Rubin, David T

    2018-04-23

    Methotrexate (MTX) is an immunomodulator used for the treatment of pediatric inflammatory bowel disease (IBD). There are currently no RCTs that assess the treatment efficacy of methotrexate within the pediatric IBD patient population. This systematic review and meta-analysis assesses the efficacy of MTX therapy among the existing pediatric literature. A systematic literature search was performed using MEDLINE and the Cochrane library from inception until March 2016. Synonyms for 'pediatric', 'methotrexate' and 'IBD' were utilized as both free text and MESH search terms. The studies included contained clinical remission (CR) rates for MTX treatment of pediatric IBD patients 18 yrs old, as mono- or combination therapy. Case studies with <10 patients were excluded. Quality assessment was performed with the Newcastle-Ottawa Scale. Meta-analysis calculated pooled CR rates. A random-effects meta-analysis with forest plots was performed using R. Fourteen (11 monotherapy, 1 combination therapy, 2 both; n = 886 patients) observational studies were eligible out of 202 studies. No interventional studies were identified. The pooled achieved CR rate for pediatric CD patients on monotherapy within 3-6 months was 57.7% (95% CI 48.2-66.6%), (P =0.22; I2 = 29.8%). The CR was 37.1% (95% CI 29.5-45.5%), (P = 0.20; I2 = 37.4%) for maintenance therapy at 12 months. Sub-analysis could not identify CR differences between MTX administration types, thiopurine exposure. This meta-analysis demonstrated that, over 50% of pediatric Crohn's disease patients induced with methotrexate achieved clinical remission, while 12-month remission rate was only 37%. Prospective controlled interventional trials should assess treatment efficacy among patient subgroups. 10.1093/ibd/izy078_video1izy078.video15774883936001.

  10. Comparison of the Phenotype and Approach to Pediatric Versus Adult Patients with Nonalcoholic Fatty Liver Disease

    Science.gov (United States)

    Nobili, V; Alisi, A; Newton, Kimberly P.; Schwimmer, Jeffrey B.

    2016-01-01

    Nonalcoholic fatty liver disease (NAFLD) is one of the main chronic non-communicable diseases in westernized societies; its worldwide prevalence has doubled during the last 20 years. NAFLD has serious health implications not only for adults, but also for children. However, pediatric NAFLD is not only an important global problem in itself, but it is likely to be associated with increases in comorbidities such as metabolic syndrome and cardiovascular diseases. There are several differences between NAFLD in children and adults and it is not clear whether the disease observed in children is the initial phase of a process that progresses with age. The increasing prevalence of pediatric NAFLD has serious implications for the future adult population requiring appropriate action. Studies of NAFLD progression, pathogenesis, and management should evaluate disease phenotypes in children and follow these over patient lifetimes. We review the similarities and differences of NAFLD between children and adults. PMID:27003600

  11. Genetics of Pediatric-Onset Motor Neuron and Neuromuscular Diseases

    Science.gov (United States)

    2015-08-24

    Spinal Muscular Atrophy; Charcot-Marie-Tooth Disease; Muscular Dystrophy; Spinal Muscular Atrophy With Respiratory Distress 1; Amyotrophic Lateral Sclerosis; Motor Neuron Disease; Neuromuscular Disease; Peroneal Muscular Atrophy; Fragile X Syndrome

  12. Economic Burden of Pediatric Asthma: Annual Cost of Disease in Iran.

    Science.gov (United States)

    Sharifi, Laleh; Dashti, Raheleh; Pourpak, Zahra; Fazlollahi, Mohammad Reza; Movahedi, Masoud; Chavoshzadeh, Zahra; Soheili, Habib; Bokaie, Saied; Kazemnejad, Anoushiravan; Moin, Mostafa

    2018-02-01

    Asthma is the first cause of children hospitalization and need for emergency and impose high economic burden on the families and governments. We aimed to investigate the economic burden of pediatric asthma and its contribution to family health budget in Iran. Overall, 283 pediatric asthmatic patients, who referred to two tertiary pediatric referral centers in Tehran capital of Iran, included from 2010-2012. Direct and indirect asthma-related costs were recorded during one-year period. Data were statistically analyzed for finding association between the costs and factors that affect this cost (demographic variables, tobacco smoke exposure, control status of asthma and asthma concomitant diseases). Ninety-two (32.5%) females and 191(67.5%) males with the age range of 1-16 yr old were included. We found the annual total pediatrics asthma related costs were 367.97±23.06 USD. The highest cost belonged to the medications (69%) and the lowest one to the emergency (2%). We noticed a significant increasing in boys' total costs ( P =0.011), and 7-11 yr old age group ( P =0.018). In addition, we found significant association between total asthma costs and asthma control status ( P =0.011). The presence of an asthmatic child can consume nearly half of the health budget of a family. Our results emphasis on improving asthma management programs, which leads to successful control status of the disease and reduction in economic burden of pediatric asthma.

  13. Patient health communication mediating effects between gastrointestinal symptoms and gastrointestinal worry in pediatric inflammatory bowel disease

    Science.gov (United States)

    To investigate the effects of patient health communication regarding their inflammatory bowel disease (IBD) to their health care providers and significant others in their daily life as a mediator in the relationship between gastrointestinal symptoms and gastrointestinal worry in pediatric patients. ...

  14. Health-related quality of life in pediatric patients with functional and organic gastrointestinal diseases

    Science.gov (United States)

    The objective of our study was to compare health-related quality of life (HRQOL) in pediatric patients with functional gastrointestinal disorders (FGIDs) and organic gastrointestinal (GI) diseases with an age-, sex-, and race/ethnicity-matched healthy sample across GI diagnostic groups and with one ...

  15. Limitations of fecal calprotectin at diagnosis in untreated pediatric Crohn's disease

    DEFF Research Database (Denmark)

    Shaoul, Ron; Sladek, Marlgozata; Turner, Dan

    2012-01-01

    Fecal Calprotectin (FC) is a validated screening test for intestinal inflammation in Crohn's disease (CD). The objective of the study was to prospectively evaluate the limitations of FC for identifying CD in newly diagnosed untreated pediatric patients and to assess the association of FC levels w...

  16. 'Shovel-Ready' applications of stem cell advances for pediatric heart disease.

    Science.gov (United States)

    Files, Matthew D; Boucek, Robert J

    2012-10-01

    The past decade has seen remarkable advances in the field of stem cell biology. Many new technologies and applications are passing the translational phase and likely will soon be relevant for the clinical pediatric cardiologist. This review will focus on two advances in basic science that are now translating into clinical trials. The first advance is the recognition, characterization, and recent therapeutic application of resident cardiac progenitor cells (CPCs). Early results of adult trials and scattered case reports in pediatric patients support expanding CPC-based trials for end-stage heart failure in pediatric patients. The relative abundance of CPCs in the neonate and young child offers greater potential benefits in heart failure treatment than has been realized to date. The second advance is the technology of induced pluripotent stem cells (iPSCs), which reprograms differentiated somatic cells to an undifferentiated embryonic-like state. When iPSCs are differentiated into cardiomyocytes, they model a patient's specific disease, test pharmaceuticals, and potentially provide an autologous source for cell-based therapy. The therapeutic recruitment and/or replacement of CPCs has potential for enhancing cardiac repair and regeneration in children with heart failure. Use of iPSCs to model heart disease holds great potential to gain new insights into diagnosis, pathophysiology, and disease-specific management for genetic-based cardiovascular diseases that are prevalent in pediatric patients.

  17. Diamox-enhanced brain SPECT in cerebrovascular diseases

    International Nuclear Information System (INIS)

    Choi, Yun Young

    2007-01-01

    Acute event in cerebrovascular disease is the second most common cause of death in Korea following cancer, and it can also cause serious neurologic deficits. Understanding of perfusion status is important for clinical applications in management of patients with cerebrovascular diseases, and then the attacks of ischemic neurologic symptoms and the risk of acute events can be reduced. Therefore, the normal vascular anatomy of brain, various clinical applications of acetazolamide-enhanced brain perfusion SPECT, including meaning and role of assessment of vascular reserve in carotid stenosis before procedure, in pediatric Moyamoya disease before and after operation, in prediction of development of hyperperfusion syndrome before procedure, and in prediction of vasospasm and of prognosis in subarachnoid hemorrhage were reviewed in this paper

  18. From the American Academy of Pediatrics: Policy statement--Tobacco use: a pediatric disease.

    Science.gov (United States)

    2009-11-01

    Tobacco use and secondhand tobacco-smoke (SHS) exposure are major national and international health concerns. Pediatricians and other clinicians who care for children are uniquely positioned to assist patients and families with tobacco-use prevention and treatment. Understanding the nature and extent of tobacco use and SHS exposure is an essential first step toward the goal of eliminating tobacco use and its consequences in the pediatric population. The next steps include counseling patients and family members to avoid SHS exposures or cease tobacco use; advocacy for policies that protect children from SHS exposure; and elimination of tobacco use in the media, public places, and homes. Three overarching principles of this policy can be identified: (1) there is no safe way to use tobacco; (2) there is no safe level or duration of exposure to SHS; and (3) the financial and political power of individuals, organizations, and government should be used to support tobacco control. Pediatricians are advised not to smoke or use tobacco; to make their homes, cars, and workplaces tobacco free; to consider tobacco control when making personal and professional decisions; to support and advocate for comprehensive tobacco control; and to advise parents and patients not to start using tobacco or to quit if they are already using tobacco. Prohibiting both tobacco advertising and the use of tobacco products in the media is recommended. Recommendations for eliminating SHS exposure and reducing tobacco use include attaining universal (1) smoke-free home, car, school, work, and play environments, both inside and outside, (2) treatment of tobacco use and dependence through employer, insurance, state, and federal supports, (3) implementation and enforcement of evidence-based tobacco-control measures in local, state, national, and international jurisdictions, and (4) financial and systems support for training in and research of effective ways to prevent and treat tobacco use and SHS

  19. MR enterography of ileocolovesicular fistula in pediatric Crohn disease

    Energy Technology Data Exchange (ETDEWEB)

    Sakala, Michelle D. [Wayne State University School of Medicine, Detroit, MI (United States); Dillman, Jonathan R.; Ladino-Torres, Maria F. [University of Michigan Health System, Department of Radiology, C.S. Mott Children' s Hospital, Section of Pediatric Radiology, Ann Arbor, MI (United States); McHugh, Jonathan B. [University of Michigan Health System, Department of Pathology, Ann Arbor, MI (United States); Adler, Jeremy [University of Michigan Health System, Department of Pediatrics and Communicable Diseases, C. S. Mott Children' s Hospital, Division of Pediatric Gastroenterology, Ann Arbor, MI (United States)

    2011-05-15

    Crohn disease, a form of chronic inflammatory bowel disease is characterized by discontinuous inflammatory lesions of the gastrointestinal tract, has a variety of behavioral patterns, including penetrating or fistulous disease. While magnetic resonance enterography (MRE) excellently depicts inflamed bowel segments, it can also be used to assess for a variety of Crohn-disease-related extraintestinal complications, including fistulae. We present the MRE findings of a complex ileocolovesicular fistula in a 14-year-old boy with Crohn disease, where the fistulous tract to the urinary bladder was best delineated on precontrast T1-W imaging because of the presence of fecal material. (orig.)

  20. Genital Involvement In Pre-Pubertal Pediatric Population: A Rare Aspect of Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Qurratul Ann Warsi

    2016-09-01

    Full Text Available Crohn’s disease is an inflammatory bowel disease (IBD, characterized by chronic intestinal inflammation that causes the loss of immune tolerance leading to bizarre inflammatory signals and disruption of mucosal barriers. Environmental triggers and interaction of genetic determinants also play an indispensible role. In this case report, we present a pre-pubertal girl with intermittent and refractory genital swelling. We emphasize that Crohn’s disease must be considered in the differential diagnosis of recurrent, non-tender, erythematous and edematous lesions of the genital area. We conclude with future directions for diagnosing and managing vulvar Crohn’s disease in pediatric population.

  1. Clinical application of exhaled nitric oxide measurement in pediatric lung diseases

    Directory of Open Access Journals (Sweden)

    Manna Angelo

    2012-12-01

    Full Text Available Summary Fractional exhaled nitric oxide (FeNO is a non invasive method for assessing the inflammatory status of children with airway disease. Different ways to measure FeNO levels are currently available. The possibility of measuring FeNO levels in an office setting even in young children, and the commercial availability of portable devices, support the routine use of FeNO determination in the daily pediatric practice. Although many confounding factors may affect its measurement, FeNO is now widely used in the management of children with asthma, and seems to provide significantly higher diagnostic accuracy than lung function or bronchial challenge tests. The role of FeNO in airway infection (e.g. viral bronchiolitis and common acquired pneumonia, in bronchiectasis, or in cases with diffuse lung disease is less clear. This review focuses on the most recent advances and the current clinical applications of FeNO measurement in pediatric lung disease.

  2. Integrative Therapies and Pediatric Inflammatory Bowel Disease: The Current Evidence.

    Science.gov (United States)

    Misra, Sanghamitra M

    2014-08-25

    Inflammatory bowel disease (IBD) primarily describes two distinct chronic conditions with unknown etiology, ulcerative colitis (UC) and Crohn's disease (CD). UC is limited to the colon, while CD may involve any portion of the gastrointestinal tract from mouth to anus. These diseases exhibit a pattern of relapse and remission, and the disease processes are often painful and debilitating. Due to the chronic nature of IBD and the negative side effects of many of the conventional therapies, many patients and their families turn to complementary and alternative medicine (CAM) for symptom relief. This article focuses on the current available evidence behind CAM/integrative therapies for IBD.

  3. Decreased ADAMTS 13 Activity is Associated With Disease Severity and Outcome in Pediatric Severe Sepsis

    Science.gov (United States)

    Lin, Jainn-Jim; Chan, Oi-Wa; Hsiao, Hsiang-Ju; Wang, Yu; Hsia, Shao-Hsuan; Chiu, Cheng-Hsun

    2016-01-01

    Abstract Decreased ADAMTS 13 activity has been reported in severe sepsis and in sepsis-induced disseminated intravascular coagulation. This study aimed to investigate the role of ADAMTS 13 in different pediatric sepsis syndromes and evaluate its relationship with disease severity and outcome. We prospectively collected cases of sepsis treated in a pediatric intensive care unit, between July 2012 and June 2014 in Chang Gung Children's Hospital in Taoyuan, Taiwan. Clinical characteristics and ADAMTS-13 activity were analyzed. All sepsis syndromes had decreased ADAMTS 13 activity on days 1 and 3 of admission compared to healthy controls. Patients with septic shock had significantly decreased ADAMTS 13 activity on days 1 and 3 compared to those with sepsis and severe sepsis. There was a significant negative correlation between ADAMTS 13 activity on day 1 and day 1 PRISM-II, PELOD, P-MOD, and DIC scores. Patients with mortality had significantly decreased ADAMTS 13 activity on day 1 than survivors, but not on day 3. Different pediatric sepsis syndromes have varying degrees of decreased ADAMTS 13 activity. ADAMTS 13 activity is strongly negatively correlated with disease severity of pediatric sepsis syndrome, whereas decreased ADAMTS 13 activity on day 1 is associated with increased risk of mortality. PMID:27100422

  4. Protecting Bone Health in Pediatric Rheumatic Diseases: Pharmacological Considerations.

    Science.gov (United States)

    Zhang, Yujuan; Milojevic, Diana

    2017-06-01

    Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health. Most commonly used assessment tools are laboratory testing including serum 25-OH-vitamin D measurement and bone mineral density measurement by a variety of methods, dual-energy X-ray absorptiometry as the most widely used. Early disease control, use of steroid-sparing medications such as disease-modifying anti-rheumatic drugs and biologics, supplemental vitamin D and calcium, and promotion of weight-bearing physical activities can help optimize bone health. Additional treatment options for osteoporosis such as bisphosphonates are still controversial in children with chronic rheumatic diseases, especially those with decreased bone density without fragility fractures. This article reviews common risk factors leading to compromised bone health in children with chronic rheumatic diseases and discusses the general approach to prevention and treatment of bone fragility.

  5. Pediatric Dupuytren's disease: case report and review of the literature

    African Journals Online (AJOL)

    children, delayed diagnosis of DD can result in debilitating disease and ... 1). The boy was subsequently taken to the operating room, where a Bruner incision was made on the palmar aspect of ... history, diabetes mellitus, and sex. However ...

  6. Comparison between a pediatric health promotion center and a pediatric obesity clinic in detecting metabolic syndrome and non-alcoholic fatty liver disease in children.

    Science.gov (United States)

    Yang, Hye Ran; Yi, Dae Yong; Choi, Hyoung Soo

    2014-12-01

    This study was done to evaluate the efficacy of health check-ups in children in detecting metabolic syndrome and non-alcoholic fatty liver disease (NAFLD) by comparing the pediatric health promotion center with the pediatric obesity clinic. Children who visited a pediatric health promotion center (n=218) or a pediatric obesity clinic (n=178) were included. Anthropometric data, blood pressure, laboratory tests, and abdominal ultrasonography were evaluated. Two different criteria were applied to diagnose metabolic syndrome. The prevalence of metabolic syndrome in the 2 units was 3.2%-3.7% in a pediatric health promotion center and 23%-33.2% in a pediatric obesity clinic. Significant differences were observed in the prevalence of each component of metabolic syndrome between the 2 units including abdominal adiposity, blood pressure, serum triglycerides, and fasting blood glucose (Pobesity clinic targeting obese children than that among patients visiting the health promotion center offering routine check-ups. An obesity-oriented approach is required to prevent obesity-related health problems in children.

  7. Digital subtraction angiography in pediatric cerebrovascular occlusive disease

    International Nuclear Information System (INIS)

    Faerber, E.N.; Griska, L.A.B.; Swartz, J.D.; Capitanio, M.A.; Popky, G.L.

    1984-01-01

    While conventional angiography has been used to demonstrate cerebrovascular occlusive disease in the past, digital subtraction angiography (DSA) is capable of showing progressive vascular involvement with ease, simplicity, and extremely low morbidity, making it particularly well suited for children and outpatients either alone or coordinated with computed tomography. The authors discuss the usefulness and advantages of DSA as demonstrated in 7 infants and children with hemiplegia, 4 of whom had sickle-cell disease

  8. Pediatric Gastrointestinal Diseases in Nigeria: Histopathologic Analysis of 74 Cases

    Directory of Open Access Journals (Sweden)

    Abudu

    2013-10-01

    Full Text Available BACKGROUND: Children are vulnerable to a vast number of diseases including gastrointestinal disorders, which may be associated with life threatening complications that sometimes result in mortality especially if left untreated. OBJECTIVE: To establish the age and sex distribution of children in the study population as well as the histopathological characteristics of gastrointestinal diseases that occurred in those children who were aged 14years and below in Sagamu, Southwestern Nigeria. MATERIALS AND METHODS: Demographic data such as age, sex, and clinical summary of children in the study population were extracted from the medical records of Olabisi Onabanjo University Teaching Hospital, Sagamu, Ogun State from January 2003 to December 2009. Based on this information, a review of paraffin embedded blocks and slides as well as histopathological reports of gastrointestinal diseases that occurred in those children aged 14years and below was undertaken at the Morbid Anatomy Department of the hospital. RESULTS: Seventy–four cases of gastrointestinal diseases were seen in children aged 14years and below. The majority (39.2% of gastrointestinal diseases were accounted for by appendiceal lesions. Hirschsprung’s disease, intussusceptions, enterocolitis and jejunal atresia accounted for 29.7%, 10.8%, 6.8% and 4.1% of cases respectively. Adenocarcinoma of the intestine was the predominant gastrointestinal tumour, occurring in 5 out of 7 children. Two cases of non-Hodgkin lymphoma were also seen. The ages of the children ranged from 2 to 14 years, with a mean age of 8.6years and a peak age incidence of gastrointestinal disease in the 10-14year age group. Male children were more commonly affected with the exception of appendiceal lesions, which occurred more in females (M:F ratio= 1.6:1.0. Acute suppurative appendicitis was the most prevalent lesion of the appendix, occurring in 13 out of 29 appendiceal lesions. Moderately differentiated to poorly

  9. Enfermedad de moyamoya, macrocefalia y déficit intelectual en un adolescente

    Directory of Open Access Journals (Sweden)

    José Vargas Díaz

    2013-03-01

    Full Text Available El patrón moyamoya se caracteriza por un estrechamiento crónico progresivo de la porción terminal de la arteria carótida interna o sus principales ramas terminales principalmente. Los niños con moyamoya típicamente se presentan con ictus arteriales isquémicos, o con accidentes isquémicos transitorios. Se presenta el caso clínico de un adolescente, con déficit intelectual y macrocránea, con evidencia en los estudios de resonancia magnética nuclear cerebral de infartos arteriales isquémicos a repetición, e imágenes sugestivas de moyamoya, y se comprueba el patrón moyamoya por angiografía de sustracción digital. Comprobado el diagnóstico de enfermedad de moyamoya, el objetivo con este enfermo fue tratar de mejorar la irrigación cerebral, planteando la necesidad de someter al paciente a cirugía de revascularización, y, mientras esto no fuera posible, usar aspirina como antiagregante plaquetario, en un intento de impedir o disminuir el riesgo de nuevos infartos cerebrales o de accidentes transitorios isquémicos a repetición.

  10. Leptomeningeal contrast enhancement in moyamoya: its potential role in postoperative assessment of circulation through the bypass

    International Nuclear Information System (INIS)

    Komiyama, M.; Nakajima, H.; Nishikawa, M.; Yasui, T.; Kitano, S.; Sakamoto, H.

    2001-01-01

    Leptomeningeal contrast enhancement (LMCE) is one of the MRI features of moyamoya. Its clinical significance, however, is not elucidated. Our purpose was to characterise LMCE on MRI and to evaluate its role in the assessment of circulation through a surgically established bypass in moyamoya. We studied 16 patients with idiopathic moyamoya (seven males, nine females, includingt four children, aged 7 to 54 years, mean 24 years) who underwent T1-weighted MRI before and after intravenous contrast medium. The presence of LMCE, its intensity and anatomical distribution, catheter angiographic findings, and relation of LMCE to the bypass surgery were assessed. More LMCE was seen in the cerebrum in most patients with moyamoya than in normal controls. LMCE in the brain stem and cerebellum was minimal, similar to that seen in the controls. LMCE was less prominent following surgery than before operation or in patients who did not undergo surgery. In three patients examined both before and after operation LMCE became less prominent following bypass surgery. As LMCE becomes less prominent after ''effective'' bypass surgery, this may be used for evaluation of effectiveness of surgery in moyamoya. (orig.)

  11. Pediatric Crohn's disease: epidemiology and emerging treatment options

    Directory of Open Access Journals (Sweden)

    Kansal S

    2014-07-01

    Full Text Available Shivani Kansal,1–3 Anthony G Catto-Smith1,2 1Department of Paediatrics, University of Melbourne, 2Department of Gastroenterology, The Royal Children's Hospital Melbourne, 3Murdoch Children's Research Institute, Melbourne, VIC, Australia Abstract: There has been a dramatic increase in the incidence of Crohn's disease over the last two to three decades worldwide, which has affected both the developed world and increasingly also the developing world. Crohn's disease is a disease of youth and can have a profound effect on the growing child, both in terms of growth and skeletal health as well psychosocial maturation. Environmental risk factors appear to be crucially important, but it is not clear at present whether improved hygiene, especially in childhood, influences immunological conditioning, or whether there is a direct impact on the gut from a disturbed gut microbiota. Genetic variation appears to relate to how the host interacts with its microbiota, determining susceptibility rather than causation. The outcome is a sustained immune response, clinically presenting as a relapsing/remitting disease process. There is no current cure for Crohn's disease; treatments are designed to reduce symptoms and control inflammation, initially by inducing a remission, then trying to maintain it. Historical therapies have included 5-aminosalicylic acid-based drugs, corticosteroids, and immunomodulators. Two approaches which are gaining increasing interest are the use of exclusive enteral nutrition and biologicals. Enteral nutrition is a remarkably effective approach, though there is a limited understanding of its mechanism and difficulties in acceptance among the medical community. Biologicals are a class of drugs which specifically target molecules and pathways central to the inflammatory process; they are also very effective, but patients can develop a secondary loss of response as a result of antibodies to the biological agent. Infection and the development

  12. Use of complementary and alternative medicine by pediatric patients with functional and organic gastrointestinal diseases: results from a multicenter survey

    NARCIS (Netherlands)

    Vlieger, Arine M.; Blink, Marjolein; Tromp, Ellen; Benninga, Marc A.

    2008-01-01

    OBJECTIVES: Many pediatric patients use complementary and alternative medicine, especially when facing a chronic illness for which treatment options are limited. So far, research on the use of complementary and alternative medicine in patients with functional gastrointestinal disease has been

  13. Pediatric differentiated thyroid carcinoma in stage I: risk factor analysis for disease free survival

    International Nuclear Information System (INIS)

    Wada, Nobuyuki; Rino, Yasushi; Masuda, Munetaka; Ito, Koichi; Sugino, Kiminori; Mimura, Takashi; Nagahama, Mitsuji; Kitagawa, Wataru; Shibuya, Hiroshi; Ohkuwa, Keiko; Nakayama, Hirotaka; Hirakawa, Shohei

    2009-01-01

    To examine the outcomes and risk factors in pediatric differentiated thyroid carcinoma (DTC) patients who were defined as TNM stage I because some patients develop disease recurrence but treatment strategy for such stage I pediatric patients is still controversial. We reviewed 57 consecutive TNM stage I patients (15 years or less) with DTC (46 papillary and 11 follicular) who underwent initial treatment at Ito Hospital between 1962 and 2004 (7 males and 50 females; mean age: 13.1 years; mean follow-up: 17.4 years). Clinicopathological results were evaluated in all patients. Multivariate analysis was performed to reveal the risk factors for disease-free survival (DFS) in these 57 patients. Extrathyroid extension and clinical lymphadenopathy at diagnosis were found in 7 and 12 patients, respectively. Subtotal/total thyroidectomy was performed in 23 patients, modified neck dissection in 38, and radioactive iodine therapy in 10. Pathological node metastasis was confirmed in 37 patients (64.9%). Fifteen patients (26.3%) exhibited local recurrence and 3 of them also developed metachronous lung metastasis. Ten of these 15 achieved disease-free after further treatments and no patients died of disease. In multivariate analysis, male gender (p = 0.017), advanced tumor (T3, 4a) stage (p = 0.029), and clinical lymphadenopathy (p = 0.006) were risk factors for DFS in stage I pediatric patients. Male gender, tumor stage, and lymphadenopathy are risk factors for DFS in stage I pediatric DTC patients. Aggressive treatment (total thyroidectomy, node dissection, and RI therapy) is considered appropriate for patients with risk factors, whereas conservative or stepwise approach may be acceptable for other patients

  14. Cannabis and pediatric inflammatory bowel disease: change blossoms a mile high

    OpenAIRE

    Hoffenberg, Edward J.; Newman, Heike; Collins, Colm; Leinwand, Kristina; Tarbell, Sally

    2017-01-01

    The trend towards decriminalization of cannabis (marijuana) continues sweeping across the United States. Colorado has been a leader of legalization of medical and recreational cannabis use. The growing public interest in the medicinal properties of cannabis and its use by patients with a variety of illnesses including inflammatory bowel disease (IBD) makes it important for pediatric gastroenterologists to understand this movement and its potential impact on patients. This article describes th...

  15. Spotlight on taliglucerase alfa in the treatment of pediatric patients with type 1 Gaucher disease

    Directory of Open Access Journals (Sweden)

    Gupta P

    2017-06-01

    Full Text Available Punita Gupta,1 Gregory M Pastores2 1Division of Genetics, Department of Pediatrics, St. Joseph’s Children’s Hospital, Paterson, New Jersey, USA; 2National Center for Inherited Metabolic Disorders, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland Abstract: Gaucher disease (GD is a heritable storage disorder caused by functional defects of the lysosomal acid β-glucosidase and the accumulation of glucosylceramide within macrophages, resulting in multiple organ dysfunction. There are three commercially available enzyme replacement therapy (ERT products for the treatment of GD type 1 (GD1: imiglucerase, velaglucerase alfa, and taliglucerase alfa. Imiglucerase and velaglucerase alfa are produced in different mammalian cell systems; imiglucerase requires postproduction deglycosylation to expose terminal α-mannose residues, which are required for mannose receptor-mediated uptake by target macrophages. These steps are critical to the success of ERT for the treatment of visceral and hematologic manifestations of GD. Taliglucerase alfa is the first US Food and Drug Administration-approved plant-cell-expressed recombinant human protein, using carrot root cell cultures. Furthermore, it does not require postproduction glycosidic modifications. It is indicated for treatment of adults with GD1 in the US, Israel, Australia, Canada, Chile, Brazil, and other countries, and it is additionally approved for the treatment of pediatric patients in the US, Australia, and Canada and for the treatment of hematologic manifestations in pediatric patients with Type 3 GD in Canada and other countries. Our review focuses on the role of taliglucerase alfa in the pediatric population. A literature search through PubMed (from 1995 up till November 2016 of English language articles was performed with the following terms: Gaucher disease, lysosomal storage disease, taliglucerase. Secondary and tertiary references were obtained by reviewing

  16. Toward Enteral Nutrition in the Treatment of Pediatric Crohn Disease in Canada: A Workshop to Identify Barriers and Enablers

    Directory of Open Access Journals (Sweden)

    Johan Van Limbergen

    2015-01-01

    Full Text Available The treatment armamentarium in pediatric Crohn disease (CD is very similar to adult-onset CD with the notable exception of the use of exclusive enteral nutrition (EEN [the administration of a liquid formula diet while excluding normal diet], which is used more frequently by pediatric gastroenterologists to induce remission. In pediatric CD, EEN is now recommended by the pediatric committee of the European Crohn’s and Colitis Organisation and the European Society for Paediatric Gastroenterology Hepatology and Nutrition as a first-choice agent to induce remission, with remission rates in pediatric studies consistently >75%. To chart and address enablers and barriers of use of EEN in Canada, a workshop was held in September 2014 in Toronto (Ontario, inviting pediatric gastroenterologists, nurses and dietitians from most Canadian pediatric IBD centres as well as international faculty from the United States and Europe with particular research and clinical expertise in the dietary management of pediatric CD. Workshop participants ranked the exclusivity of enteral nutrition; the health care resources; and cost implications as the top three barriers to its use. Conversely, key enablers mentioned included: standardization and sharing of protocols for use of enteral nutrition; ensuring sufficient dietetic resources; and reducing the cost of EEN to the family (including advocacy for reimbursement by provincial ministries of health and private insurance companies. Herein, the authors report on the discussions during this workshop and list strategies to enhance the use of EEN as a treatment option in the treatment of pediatric CD in Canada.

  17. [Transition from pediatric to adult health care services for adolescents with chronic diseases: Recommendations from the Adolescent Branch from Sociedad Chilena de Pediatría].

    Science.gov (United States)

    Zubarew, Tamara; Correa, Loreto; Bedregal, Paula; Besoain, Carolina; Reinoso, Alejandro; Velarde, Macarena; Valenzuela, María Teresa; Inostroza, Carolina

    2017-01-01

    The Adolescent Branch from Sociedad Chilena de Pediatría supports the implementation of planned programs for transition from child to adult health centers, oriented to adolescents with chronic diseases, in order to ensure an appropriate follow-up and a high-quality health care. Recommendations for care are set out in the FONIS and VRI PUC project carried out by the Division of Pediatrics of the Universidad Católica de Chile: “Transition process from pediatric to adult services: perspectives of adolescents with chronic diseases, caregivers and health professionals”, whose goal was to describe the experience, barriers, critical points, and facilitators in the transition process. Critical points detected in this study were: existence of a strong bond between adolescents, caregivers and the pediatric team, resistance to transition, difficulty developing autonomy and self-management among adolescents; invisibility of the process of adolescence; and lack of communication between pediatric and adult team during the transfer. According to these needs, barriers and critical points, and based on published international experiences, recommendations are made for implementation of gradual and planned transition processes, with emphasis on the design and implementation of transition policies, establishment of multidisciplinary teams and transition planning. We discuss aspects related to coordination of teams, transfer timing, self-care and autonomy, transition records, adolescent and family participation, need for emotional support, ethical aspects involved, importance of confidentiality, need for professional training, and the need for evaluation and further research on the subject.

  18. Second Harmonic Generation Reveals Subtle Fibrosis Differences in Adult and Pediatric Nonalcoholic Fatty Liver Disease.

    Science.gov (United States)

    Liu, Feng; Zhao, Jing-Min; Rao, Hui-Ying; Yu, Wei-Miao; Zhang, Wei; Theise, Neil D; Wee, Aileen; Wei, Lai

    2017-11-20

    Investigate subtle fibrosis similarities and differences in adult and pediatric nonalcoholic fatty liver disease (NAFLD) using second harmonic generation (SHG). SHG/two-photon excitation fluorescence imaging quantified 100 collagen parameters and determined qFibrosis values by using the nonalcoholic steatohepatitis (NASH) Clinical Research Network (CRN) scoring system in 62 adult and 36 pediatric NAFLD liver specimens. Six distinct parameters identified differences among the NASH CRN stages with high accuracy (area under the curve, 0835-0.982 vs 0.885-0.981, adult and pediatric). All portal region parameters showed similar changes across early stages 0, 1C, and 2, in both groups. Parameter values decreased in adults with progression from stage 1A/B to 2 in the central vein region. In children, aggregated collagen parameters decreased, but nearly all distributed collagen parameters increased from stage 1A/B to 2. SHG analysis accurately reproduces NASH CRN staging in NAFLD, as well as reveals differences and similarities between adult and pediatric collagen deposition not captured by currently available quantitative methods. © American Society for Clinical Pathology, 2017. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  19. Pediatric Inflammatory Bowel Disease with Cytoplasmic Staining of Antineutrophil Cytoplasmic Antibodies

    Directory of Open Access Journals (Sweden)

    Omar I. Saadah

    2013-01-01

    Full Text Available Background. It is unusual for the antineutrophil cytoplasmic antibody with cytoplasmic pattern (cANCA to present in patients with inflammatory bowel disease (IBD without vasculitis. The purpose of this study was to describe the occurrence and characteristics of pediatrics IBD with cANCA. Methods. A retrospective review of pediatric IBD associated with cANCA serology in patients from King Abdulaziz University Hospital, Saudi Arabia, between September 2002 and February 2012. Results. Out of 131 patients with IBD screened for cANCAs, cANCA was positive in 7 (5.3% patients of whom 4 had ulcerative colitis and 3 had Crohn's disease. The median age was 8.8 years (2–14.8 years. Six (86% were males. Of the 7 patients, 5 (71% were Saudi Arabians and 2 were of Indian ethnicity. The most common symptoms were diarrhea, abdominal pain, weight loss, and rectal bleeding. None had family history or clinical features suggestive of vasculitis involving renal and respiratory systems. No difference in the disease location or severity was observed between cANCA positive and cANCA negative patients apart from male preponderance in cANCA positive patients. Conclusion. The occurrence of cANCA in pediatric IBD is rare. Apart from male preponderance, there were no peculiar characteristics for the cANCA positive patients.

  20. Enteral Nutrition in the Management of Pediatric and Adult Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Tawnya Hansen

    2018-04-01

    Full Text Available Genetic and environmental factors are thought to profoundly influence the pathophysiology of Crohn’s disease (CD. Changes in dietary and hygiene patterns affect the interactions between the immune system and environment. The gut microbiome is responsible for mediating host immune response with significant dysbiosis observed in individuals with CD. Diet therapy using exclusive enteral nutrition (EEN has been studied as primary therapy for the management of CD. EEN may cultivate the presence of beneficial microbiota, improve bile acid metabolism, and decrease the number of dietary microparticles possibly influencing disease and immune activity. In this review, we will address the current evidence on EEN in the management of adult and pediatric CD. In adults, EEN appears to be moderately beneficial for the induction of remission of CD; however, its use is understudied and underutilized. Stronger evidence is in place to support the use of EEN in pediatric CD with the added benefit of nutrition support and steroid-sparing therapy during the growth phase. Overall, EEN is an established therapy in inducing CD remission in the pediatric population while its role as primary therapy of adult Crohn’s disease remains to be defined.

  1. Duodenal microbiota composition and mucosal homeostasis in pediatric celiac disease

    NARCIS (Netherlands)

    Cheng, J.C.; Kalliomäki, M.; Heilig, G.H.J.; Palva, A.; Lähteenoja, H.; Vos, de W.M.; Salojärvi, J.; Satokari, R.

    2013-01-01

    BACKGROUND: Celiac disease (CD) is an autoimmune disorder of the small intestine which is triggered by dietary gluten in genetically predisposed (HLA-DQ2/DQ8 positive) individuals. Only a fraction of HLA-DQ2/DQ8 positive individuals develop CD indicating that other factors have a role in the

  2. Imaging of disease progression in a case of idiopathic moyamoya ...

    African Journals Online (AJOL)

    Comparison is also made with conventional angiography at the last visit. Characteristic imaging appearances of asymmetric narrowing of the internal carotid arteries (especially the supraclinoid portion) with numerous collateral vessels around the brainstem, especially within the ambient and quadrigeminal plate cisterns, ...

  3. Anesthesia and Databases: Pediatric Cardiac Disease as a Role Model.

    Science.gov (United States)

    Vener, David F; Pasquali, Sara K; Mossad, Emad B

    2017-02-01

    Large data sets have now become ubiquitous in clinical medicine; they are particularly useful in high-acuity, low-volume conditions such as congenital heart disease where data must be collected from many centers. These data fall into 2 categories: administrative data arising from hospital admissions and charges and clinical data relating to specific diseases or procedures. In congenital cardiac diseases, there are now over a dozen of these data sets or registries focusing on various elements of patient care. Using probabilistic statistic matching, it is possible to marry administrative and clinical data post hoc using common elements to determine valuable information about care patterns, outcomes, and costs. These data sets can also be used in a collaborative fashion between institutions to drive quality improvement (QI). Because these data may include protected health information (PHI), care must be taken to adhere to federal guidelines on their use. A fundamental principle of large data management is the use of a common language and definition (nomenclature) to be effective. In addition, research derived from these information sources must be appropriately balanced to ensure that risk adjustments for preoperative and surgical factors are taken into consideration during the analysis. Care of patients with cardiac disease both in the United States and abroad consistently shows wide variability in mortality, morbidity, and costs, and there has been a tremendous amount of discussion about the benefits of regionalization of care based on center volume and outcome measurements. In the absence of regionalization, collaborative learning techniques have consistently been shown to minimize this variability and improve care at all centers, but before changes can be made it is necessary to accurately measure accurately current patient outcomes. Outcomes measurement generally falls under hospital-based QI initiatives, but more detailed analysis and research require

  4. North American Pediatric Gastroenterology Fellowship Needs Assessment in Inflammatory Bowel Disease: Trainee and Program Director Perspectives.

    Science.gov (United States)

    Dotson, Jennifer L; Falaiye, Tolulope; Bricker, Josh B; Strople, Jennifer; Rosh, Joel

    2016-07-01

    Pediatric inflammatory bowel disease (IBD) care is complex and rapidly evolving. The Crohn's and Colitis Foundation of America and North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition cosponsored a needs assessment survey of pediatric gastroenterology trainees and program directors (PDs) to inform on educational programming. A Web-based, self-completed survey was provided to North American trainees and PDs during the 2013-2014 academic year. Standard descriptive statistics summarized demographics and responses. One hundred sixty-six of 326 (51%) trainees (62% female) and 37 of 74 (50%) PDs responded. Median trainees per program = 5 and median total faculty = 10 (3 IBD experts); 15% of programs did not have a self-identified "IBD expert" faculty member. Sixty-nine percent of trainees were confident/somewhat confident in their IBD inpatient training, whereas 54% were confident/somewhat confident in their outpatient training. Trainees identified activities that would most improve their education, including didactics (55%), interaction with national experts (50%), trainee-centered IBD Web resources (42%), and increased patient exposure (42%). Trainees were most confident in managing inpatient active Crohn's disease/ulcerative colitis, phenotype classification, managing biological therapies, and using clinical disease activity indices. They were least confident in managing J-pouch complications, performing pouchoscopy, managing extraintestinal manifestations, and ostomy-related complications. Eighty-five percent would like an IBD-focused training elective. Most directors (86%) would allow trainees to do electives at other institutions. This IBD needs assessment survey of pediatric gastroenterology trainees and PDs demonstrated a strong resource commitment to IBD training and clinical care. Areas for educational enrichment emerged, including pouch and ostomy complications.

  5. Somatic USP8 Gene Mutations Are a Common Cause of Pediatric Cushing Disease.

    Science.gov (United States)

    Faucz, Fabio R; Tirosh, Amit; Tatsi, Christina; Berthon, Annabel; Hernández-Ramírez, Laura C; Settas, Nikolaos; Angelousi, Anna; Correa, Ricardo; Papadakis, Georgios Z; Chittiboina, Prashant; Quezado, Martha; Pankratz, Nathan; Lane, John; Dimopoulos, Aggeliki; Mills, James L; Lodish, Maya; Stratakis, Constantine A

    2017-08-01

    Somatic mutations in the ubiquitin-specific protease 8 (USP8) gene have been recently identified as the most common genetic alteration in patients with Cushing disease (CD). However, the frequency of these mutations in the pediatric population has not been extensively assessed. We investigated the status of the USP8 gene at the somatic level in a cohort of pediatric patients with corticotroph adenomas. The USP8 gene was fully sequenced in both germline and tumor DNA samples from 42 pediatric patients with CD. Clinical, biochemical, and imaging data were compared between patients with and without somatic USP8 mutations. Five different USP8 mutations (three missense, one frameshift, and one in-frame deletion) were identified in 13 patients (31%), all of them located in exon 14 at the previously described mutational hotspot, affecting the 14-3-3 binding motif of the protein. Patients with somatic mutations were older at disease presentation [mean 5.1 ± 2.1 standard deviation (SD) vs 13.1 ± 3.6 years, P = 0.03]. Levels of urinary free cortisol, midnight serum cortisol, and adrenocorticotropic hormone, as well as tumor size and frequency of invasion of the cavernous sinus, were not significantly different between the two groups. However, patients harboring somatic USP8 mutations had a higher likelihood of recurrence compared with patients without mutations (46.2% vs 10.3%, P = 0.009). Somatic USP8 gene mutations are a common cause of pediatric CD. Patients harboring a somatic mutation had a higher likelihood of tumor recurrence, highlighting the potential importance of this molecular defect for the disease prognosis and the development of targeted therapeutic options. Copyright © 2017 Endocrine Society

  6. [Presence of Helicobacter pylori in gastric biopsies and feces of pediatric patients with celiac disease].

    Science.gov (United States)

    Medina, M G; Medina, M L; Martín, G T; Dikstein, B C; Picón, S O; Gorodner, J O; Merino, L A

    2009-01-01

    Helicobacter pylori (H. pylori) is the main etiologic agent of chronic gastritis and it is an important cause of gastric damage. The celiac disease can affect the morphology and the function of the gastrointestinal tract from the stomach to the colon and it is frequently associated with chronic gastritis. to assess the presence of H. pylori in gastric biopsies and in feces of pediatric patients with celiac disease and to relate it with the symptoms. Pediatric patients with celiac disease attending the Gastroenterology Service at the "Avelino Castelán" Hospital in Resistencia (Argentina) were included in the study. Gastric biopsies samples were obtained by endoscopy for histological studies, the symptoms and socio-epidemiological characteristics were recorded and the polimerase chain reaction(PCR) was applied in feces in order to detect the presence of H. pylori. Thirty one patients with celiac disease were studied (16 female and 15 male; age range:1-14 years; median 6.7 years); 14 (45.2%) were positive for H. pylori in gastric biopsy and among them, only 2 (14.2%) were positive for H. pylori in stool samples. There were not significant differences between symptoms between H. pylori positive and negative patients. 45.2% of the patients with celiac disease were infected by H. pylori. There was no correlation between the frequencies of bacterial detection in feces and in gastric biopsies. The clinical manifestations of celiac disease did not increase in children infected with H. pylori.

  7. Diverting Ileostomy for the Treatment of Severe, Refractory, Pediatric Inflammatory Bowel Disease.

    Science.gov (United States)

    Maxwell, Elizabeth C; Dawany, Noor; Baldassano, Robert N; Mamula, Petar; Mattei, Peter; Albenberg, Lindsey; Kelsen, Judith R

    2017-09-01

    Diverting ileostomy is used as a temporizing therapy in patients with perianal Crohn disease; however, little data exist regarding its use for colonic disease. The primary aim of the present study was to determine the role of diversion in severe refractory colonic inflammatory bowel disease (IBD) in a pediatric population. Retrospective study of patients who underwent diverting ileostomy at The Children's Hospital of Philadelphia from 2000 to 2014 for the management of severe, refractory colonic IBD. Clinical variables were compared in the 1 year before ileostomy and 1 year after diversion. Surgical and disease outcomes including changes in diagnosis were reviewed through 2015. Twenty-four patients underwent diverting ileostomy for refractory colonic disease. Initial diagnoses were Crohn disease in 10 (42%), ulcerative colitis in 1 (4%), and IBD-unclassified in 13 patients (54%). Comparing data before and after surgery, there were statistically significant improvements in height and weight velocities, height velocity z score, blood transfusion requirement, hemoglobin, and hospitalization rates. Chronic steroid use decreased from 71% to 22%. At the conclusion of the study, 10 patients had undergone subsequent colectomy, 7 had successful bowel reanastomosis, and 7 remain diverted. Seven patients (29%) had a change in diagnosis. There were 13 surgical complications in 7 subjects, including prolapse reduction, stoma revision, and resection of ischemic bowel. In pediatric patients with refractory colonic IBD, diverting ileostomy can be a successful intervention to induce clinical stability. Importantly, diversion is a steroid-sparing therapy and allows additional time to clarify the diagnosis.

  8. Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

    Directory of Open Access Journals (Sweden)

    Cron Randy Q

    2011-01-01

    Full Text Available Abstract Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology.

  9. Bone marrow in pediatric patients with Hodgkin's disease.

    Science.gov (United States)

    Khan, Fauzia Shafi; Hasan, Rabiya Fayyaz

    2012-01-01

    Hodgkin's disease is a malignant process of lymphoreticular system that constitutes 6% of childhood cancers Accurate staging of lymphoma is the basis for rational therapeutic planning and assessment of the presence or absence of marrow involvement is a basic part of the staging evaluation. The objective of this study was to determine the incidence of marrow infiltration in paediatric patients with Hodgkin's disease and to ascertain its morphological spectrum in the marrow. The study included 85 paediatric patients with diagnosed Hodgkin's disease seen at The Children's Hospital/Institute of Child Health, Lahore, from January 2010 to December 2011, referred to haematology department for bone marrow biopsies. Ages ranged between two years to fourteen years with an average age of seven years, the male female ratio being 13:1. Mixed cellularity was the commonest histological type present in 66 (78%) cases. The presenting feature common in all cases was superficial lymphadenopathy followed by hepatomegaly in 17 (20%) cases and splenomegaly in 16 (19%). All the marrow aspirates were negative for infiltration. Trephine biopsies revealed marrow infiltration in 9 (10.5%). Five (56%) cases had bilateral while 4 (44%) had unilateral involvement. Pattern of infiltration was diffuse in 8 (89%) and focal in one (11%) trephines. Increased marrow fibrosis was present in eight (89%) cases. Diagnostic Reed Sternberg cells were identified in only one case and the mononuclear variants were present in six cases and atypical cells were present in two cases in these immunohistochemistry for CD15 and CD30 was performed which was positive. Granulomas in one and lymphoid aggregates were present in two trephine biopsies otherwise negative for Hodgkin's infiltration. Bone marrow infiltration was present in 10.5% cases, immunohistochemistry was used to confirm infiltration in two cases, the pattern of infiltration being diffuse in majority (89%).

  10. Reimbursement for pediatric diabetes intensive case management: a model for chronic diseases?

    Science.gov (United States)

    Beck, Joni K; Logan, Kathy J; Hamm, Robert M; Sproat, Scott M; Musser, Kathleen M; Everhart, Patricia D; McDermott, Harrold M; Copeland, Kenneth C

    2004-01-01

    . Differences between participating and nonparticipating groups included age (participants were of younger age), double-parent households (participants were more likely to be from double parent households), and number of medical visits kept (participants kept more follow-up visits). No differences in duration of diabetes, months followed in the program, sex, or ethnicity were observed. Contracting with third-party payers for pediatric diabetes intensive case management services reduces costs by reducing emergency department and inpatient hospital utilizations, likely a result of intensive education and immediate access to the diabetes health care team for crisis management. Such strategies may prove to be cost saving not only for diabetes management but also for managing other costly and personnel-intensive chronic diseases.

  11. Feeding and Swallowing Disorders in Pediatric Neuromuscular Diseases: An Overview.

    Science.gov (United States)

    van den Engel-Hoek, Lenie; de Groot, Imelda J M; de Swart, Bert J M; Erasmus, Corrie E

    2015-11-20

    Feeding and swallowing problems in infants and children have a great impact on health and wellbeing. The aim of this study was to provide an overview of recognized feeding and swallowing problems in different groups of children with neuromuscular diseases, based on relevant literature and expert opinion, and to propose recommendations for the assessment and treatment of these problems. Almost all pediatric neuromuscular diseases are accompanied by feeding and swallowing problems during the different phases of deglutition, problems that give rise to a wide variety of signs and symptoms, which emphasizes the importance of a comprehensive feeding and swallowing assessment by a speech and language therapist.

  12. Still's Disease in a Pediatric Patient after Liver Transplantation

    Directory of Open Access Journals (Sweden)

    Juan-Carlos Meza

    2013-01-01

    Full Text Available Still's disease (SD is a multisystemic inflammatory disease characterized by persistent arthritis and in many cases with fever of unknown origin. Diagnosis of SD is challenging because of nonspecific characteristics and especially in the case of a patient with solid organ transplantation and immunosuppressive therapy where multiple causes of fever are possible. There is no diagnostic test for SD, even though some useful diagnostic criteria or laboratory findings, such as serum ferritin levels, have been proposed, and useful imaging studies for the diagnosis or followup of SD have not been developed. We report the case of a 9-year-old child who presented with high grade fever associated with joint pain after a history of liver transplantation and immunosuppressive therapy. Laboratory tests showed increased acute phase reactants, elevated ferritin, and leukocytosis. An 18 F-fluorodeoxyglucose positron emission tomography (18F-FDG PET was performed identifying abnormal hypermetabolic areas localized in spleen, transplanted liver, and bone marrow secondary to inflammatory process. All infectious, autoimmune, and malignant causes were ruled out. A diagnosis of SD was performed and a steroid-based regimen was initiated with adequate response and no evidence of recurrence. To our knowledge this is the first case of SD following a solid organ transplant.

  13. Therapeutic options in pediatric non alcoholic fatty liver disease: current status and future directions

    Directory of Open Access Journals (Sweden)

    Vajro Pietro

    2012-10-01

    Full Text Available Abstract The epidemics of overweight and obesity has resulted in a significant increase of non alcoholic fatty liver disease (NAFLD, a potentially progressive condition. Currently, obesity related hepatopathy represents therefore the main cause of pediatric chronic liver disease. The first choice treatment at all ages is weight loss and/or lifestyle changes, however compliance is very poor and a pharmacological approach has become necessary. In the present article we present a systematic literature review focusing on established pediatric NALFD drugs (ursodeoxycholic acid, insulin sensitizers, and antioxidants and on innovative therapeutic options as well. Regarding the former ones, a pediatric pilot study highlighted that ursodeoxycholic acid is not efficient on transaminases levels and bright liver. Similarly, a recent large scale, multicenter randomized clinical trial (TONIC study showed that also insulin sensitizers and antioxidant vitamin E have scarce effects on serum transaminase levels. Among a large series of novel therapeutic approaches acting on recently proposed different pathomechanisms, probiotics seem hitherto the most interesting and reasonable option for their safety and tolerability. Toll-like receptors modifiers, Pentoxifylline, and Farnesoid X receptors agonists have been still poorly investigated, and will need further studies before becoming possible promising innovative therapeutic strategies.

  14. Atypical presentation of multicentric Castleman disease in a pediatric patient: pleural and pericardial effusion.

    Science.gov (United States)

    Akman, Alkim Oden; Basaran, Ozge; Ozyoruk, Derya; Han, Unsal; Sayli, Tulin; Cakar, Nilgun

    2016-06-01

    Castleman disease (CD) is a rare poorly understood lymphoproliferative disorder. Pediatric onset CD has been reported before. However, most of them have benign unicentric pattern. Multicentric CD (MCD) is quite rare in children. Herein, we report a 13-year-old adolescent boy with MCD of the hyaline vascular variant presenting with pleural and pericardial effusion, which is an uncommon presentation. MCD should be considered in the differential diagnosis of pleural and/or pericardial effusion with unexplained lymph nodes in children. What is Known •Pediatric Castleman disease (CD) most commonly occurs in the unicentric form, which typically is asymptomatic and cured by lymph node excision. •The diagnosis of MCD can be difficult owing to the heterogeneity of presentation and potential for nonspecific multisystem involvement. What is New •A 13-year-old adolescent boy was diagnosed with MCD of the hyaline vascular variant presenting with pleural and pericardial effusion, which is an uncommon presentation. •In a pediatric patient with fever, pleural-pericardial effusion and multiple lymph nodes, MCD should be considered in differantial diagnosis.

  15. Pediatric Non-alcoholic Fatty Liver Disease: Current Thinking.

    Science.gov (United States)

    Nobili, Valerio; Socha, Piotr

    2017-10-31

    Non-alcoholic fatty liver disease (NAFLD), an increasingly prevalent paediatric disorder is diagnosed and managed by both paediatric gastroenterologists / hepatologists but also frequently by the general paediatrician. This paper updates recent advances in diagnostic and therapeutic approach which may be applied to everyday practice. Diagnosis of NAFLD takes into account the risk factor profile and is a diagnosis of exclusion. Techniques such as transient elastography and specific biomarkers aimed at improving diagnosis and monitoring of NAFLD need further validation in the paediatric population. Defining the risk to develop cirrhosis seems to be of primary importance already in childhood and a combination of genetic, clinical and environmental factors can help in monitoring and making decisions on therapy. Weight reduction therapy should be the aim of treatment approach but the compliance is poor and pharmacological treatment would be helpful- DHA, some probiotics, vitamin E are to be considered but evidence is not sufficient to recommend widespread use.

  16. Lead Toxicity in the Pediatric Patient with Sickle Cell Disease: Unique Risks and Management.

    Science.gov (United States)

    Jung, Josephine Misun; Peddinti, Radhika

    2018-01-01

    Lead toxicity is the result of lead ingestion, one of the most common ingestions in the pediatric population. Nationwide and statewide efforts to recognize and curtail this epidemic have led to declining rates of toxicity. In patients with sickle cell disease (SCD), lead toxicity can be an elusive diagnosis due to overlapping symptom profiles, and inconsistent follow-up with a primary care physician can make the diagnosis even more difficult. In this article, two illustrative cases of lead toxicity in patients with SCD are described. The discussion reviews the current risk factors, screening, and inpatient management of lead toxicity, as well as describing the unique and sometimes confounding presentations of lead toxicity versus sickle cell crisis. [Pediatr Ann. 2018;47(1):e36-e40.]. Copyright 2018, SLACK Incorporated.

  17. Animal models of pediatric chronic kidney disease. Is adenine intake an appropriate model?

    Directory of Open Access Journals (Sweden)

    Débora Claramunt

    2015-11-01

    Full Text Available Pediatric chronic kidney disease (CKD has peculiar features. In particular, growth impairment is a major clinical manifestation of CKD that debuts in pediatric age because it presents in a large proportion of infants and children with CKD and has a profound impact on the self-esteem and social integration of the stunted patients. Several factors associated with CKD may lead to growth retardation by interfering with the normal physiology of growth plate, the organ where longitudinal growth rate takes place. The study of growth plate is hardly possible in humans and justifies the use of animal models. Young rats made uremic by 5/6 nephrectomy have been widely used as a model to investigate growth retardation in CKD. This article examines the characteristics of this model and analyzes the utilization of CKD induced by high adenine diet as an alternative research protocol.

  18. Unanticipated hospital admission in pediatric patients with congenital heart disease undergoing ambulatory noncardiac surgical procedures.

    Science.gov (United States)

    Yuki, Koichi; Koutsogiannaki, Sophia; Lee, Sandra; DiNardo, James A

    2018-05-18

    An increasing number of surgical and nonsurgical procedures are being performed on an ambulatory basis in children. Analysis of a large group of pediatric patients with congenital heart disease undergoing ambulatory procedures has not been undertaken. The objective of this study was to characterize the profile of children with congenital heart disease who underwent noncardiac procedures on an ambulatory basis at our institution, to determine the incidence of adverse cardiovascular and respiratory adverse events, and to determine the risk factors for unscheduled hospital admission. This is a retrospective study of children with congenital heart disease who underwent noncardiac procedures on an ambulatory basis in a single center. Using the electronic preoperative anesthesia evaluation form, we identified 3010 patients with congenital heart disease who underwent noncardiac procedures of which 1028 (34.1%) were scheduled to occur on an ambulatory basis. Demographic, echocardiographic and functional status data, cardiovascular and respiratory adverse events, and reasons for postprocedure admission were recorded. Univariable analysis was conducted. The unplanned hospital admission was 2.7% and univariable analysis demonstrated that performance of an echocardiogram within 6 mo of the procedure and procedures performed in radiology were associated with postoperative admission. Cardiovascular adverse event incidence was 3.9%. Respiratory adverse event incidence was 1.8%. Ambulatory, noncomplex procedures can be performed in pediatric patients with congenital heart disease and good functional status with a relatively low unanticipated hospital admission rate. © 2018 John Wiley & Sons Ltd.

  19. Sleep disorders in pediatric chronic kidney disease patients.

    Science.gov (United States)

    Stabouli, Stella; Papadimitriou, Eleni; Printza, Nikoleta; Dotis, John; Papachristou, Fotios

    2016-08-01

    The prevalence of sleep disorders during childhood has been estimated to range from 25 to 43 %. The aim of this review is to determine the prevalence of sleep disorders and possible associations with chronic kidney disease (CKD)-related factors and health-related quality of life (HRQOL) in children with CKD. An electronic systematic literature search for sleep disorders in children with CKD in Pubmed, Embase and the Cochrane Library Databases identified seven relevant articles for review, all of which reported an increased prevalence of sleep disorders in children with CKD. Five studies included children with CKD undergoing dialysis, and two studies included only non-dialysis patients. In all studies the presence of sleep disturbances was assessed by questionnaires; only one study compared the results of a validated questionnaire with laboratory-based polysomnography. The prevalence of any sleep disorder ranged from 77 to 85 % in dialysis patients, to 32-50 % in transplanted patients and 40-50 % in non-dialysis patients. The most commonly studied disorder was restless legs syndrome, which presented at a prevalence of 10-35 %. Three studies showed significant associations between presence of sleep disorders and HRQOL. We found consistent evidence of an increased prevalence of sleep disturbances in children with CKD, and these seemed to play a critical role in HRQOL.

  20. [Computer-assisted diet therapy in pediatric kidney diseases].

    Science.gov (United States)

    Dartois, A M; Ducamp, S; Decaux, F; Broyer, M

    1989-01-01

    The authors present a computer program written in UCSD Pascal) which monitors the dietary management of children with kidney diseases. Diet is established according to height, weight, chronological and statural age and recommended dietary allowances (USRDA). The composition of the prescribed diet and of food intake is given as amounts of animal and vegetable protein, fat, carbohydrates, energy, water, Na, K, Ca, P and renal solute load, per day and per kilo BW as compared to RDA. The amount of food is presented in tabular form, per day, per meal, per feed, or per tube-feeding with the schedule. It is possible to calculate the nutrients of food recipes. The food table includes 500 items, that can be modified as required. The drug table contains 100 items. The program calculates also average food consumption for dietary surveys. Diets, recipes and food tables may be viewed and modified before print out at each step of the calculation. The diet data bank stores 100 diets per floppy disk.

  1. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E. [Department of Radiology, Children`s Hospital Medical Center, Boston, MA (United States); Eldredge, E.A. [Department of Anesthesia, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States); Scott, R.M. [Department of Neurosurgery, Children`s Hospital Medical Center and Harvard Medical School, Boston, MA (United States)

    1998-11-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher`s exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.) With 8 tabs., 37 refs.

  2. Neurologic complications of cerebral angiography in childhood moyamoya syndrome

    International Nuclear Information System (INIS)

    Robertson, R.L.; Chavali, R.V.; Robson, C.D.; Barnes, P.D.; Burrows, P.E.; Eldredge, E.A.; Scott, R.M.

    1998-01-01

    Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders. (orig.)

  3. Association between MDR1 gene polymorphisms and the risk of Crohn's disease in a cohort of Algerian pediatric patients.

    Science.gov (United States)

    Bouzidi, Amira; Mesbah-Amroun, Hamida; Boukercha, Aziza; Benhassine, Fadila; Belboueb, Réda; Berkouk, Karima; Messadi, Wassila; Touil-Boukoffa, Chafia

    2016-12-01

    The multi-drug resistance gene (MDR1) has raised increasing interest as a susceptibility gene for Crohn's disease (CD). The role of MDR1 single-nucleotide polymorphisms (SNPs) in the predisposition and behavior of CD in the pediatric population is still elusive. Here, we investigated whether SNPs in MDR1 are associated with CD in Algerian pediatric patients. A case-control study was conducted enrolling 47 pediatric CD patients and 100 controls. All subjects were genotyped for the most common MDR1 SNPs (C3434T, C1236T, and G2677A/T) using PCR-RFLP method. We also explored the association between polymorphisms and clinical sub-phenotypes. We have detected no significant association of C3435T SNP and pediatric CD. However, we observed a significantly higher frequency of the risk alleles, 1236T and 2677T/A among the CD patients compared to controls. Moreover, the risk allele 1236T was associated to a higher risk for resective surgery. Our data suggest that the C1236T and G2677A/T SNPs in the MDR1 gene are associated with CD and the C1236T risk allele with a more severe course of disease in Algerian pediatric patients. Further analysis using larger patients group and functional studies would be interesting to elucidate the role of MDR1 gene in pediatric CD.Pediatric Research (2016); doi:10.1038/pr.2016.163.

  4. Effect of communication style and physician-family relationships on satisfaction with pediatric chronic disease care.

    Science.gov (United States)

    Swedlund, Matthew P; Schumacher, Jayna B; Young, Henry N; Cox, Elizabeth D

    2012-01-01

    Over 8% of children have a chronic disease and many are unable to adhere to treatment. Satisfaction with chronic disease care can impact adherence. We examine how visit satisfaction is associated with physician communication style and ongoing physician-family relationships. We collected surveys and visit videos for 75 children ages 9-16 years visiting for asthma, diabetes, or sickle cell disease management. Raters assessed physician communication style (friendliness, interest, responsiveness, and dominance) from visit videos. Quality of the ongoing relationship was measured with four survey items (parent-physician relationship, child-physician relationship, comfort asking questions, and trust in the physician), while a single item assessed satisfaction. Correlations and chi square were used to assess association of satisfaction with communication style or quality of the ongoing relationship. Satisfaction was positively associated with physician to parent (p relationships (p communication style and the quality of the ongoing relationship contribute to pediatric chronic disease visit satisfaction.

  5. Vaccine-preventable diseases in pediatric patients: a review of measles, mumps, rubella, and varicella [digest].

    Science.gov (United States)

    Levine, Deborah A; Pade, Kathryn H

    2016-12-22

    Vaccine-preventable diseases such as measles, mumps, rubella, and varicella continue to plague children and adults worldwide. Although public health programs have helped decrease the prevalence and sequelae of these diseases, outbreaks still occur. To limit the spread of these diseases, emergency clinicians must be able to readily identify the characteristic presentations of the rashes associated with measles, rubella, and varicella, as well as the common presenting features associated with mumps. Diagnostic laboratory studies are not usually necessary, as a complete history and physical examination usually lead to an accurate diagnosis. Treatment for these vaccine-preventable diseases usually consists of supportive care, but, in some cases, severe complications and death may occur. This issue provides a review of the clinical features, differential diagnoses, potential complications, and treatment options for measles, mumps, rubella, and varicella. [Points & Pearls is a digest of Pediatric Emergency Medicine Practice].

  6. Differentiation of mucosal disease from partial development of the paranasal sinuses in pediatric patients

    International Nuclear Information System (INIS)

    Duerinckx, A.J.; Whyte, A.M.; Lufkin, R.B.; Hall, T.R.; Kangarloo, H.

    1988-01-01

    On magnetic resonance (MR) images of pediatric patients, sinus mucosal disease may have an appearance similar to that of the normal partially developed sinus, leading to an increase in the number of patients labeled as having incidental sinusitis. The paranasal sinuses were retrospectively evaluated in 27 infants and children aged 0-11 years undergoing brain MR imaging for indications both unrelated and related to sinus disease. The authors developed criteria for grading paranasal sinus development and mucosal disease. Incidental mucosal disease is not uncommon, occurring in 28% of patients aged 0-7 years. In children under 3 years of age, inflammatory mucosal thickening and marrow surrounding the partially developed sinus have a high signal on many MR sequences and may be confused. Recognition of the low-intensity peripheral cortical margin of the sinus and awareness of the stages of normal sinus development allow differentiation

  7. When and why is surgical revascularization indicated for the treatment of moyamoya syndrome in patients with RASopathies? A systematic review of the literature and a single institute experience.

    Science.gov (United States)

    Scala, Marcello; Fiaschi, Pietro; Capra, Valeria; Garrè, Maria Luisa; Tortora, Domenico; Ravegnani, Marcello; Pavanello, Marco

    2018-07-01

    Moyamoya disease (MMD) is a cerebrovascular disorder characterized by the progressive occlusion of the supraclinoid internal carotid artery (ICA), resulting in the formation of an abnormal cerebral vascular network. When MMD occurs in association with an underlying medical condition, including some distinctive genetic disorders, it is named moyamoya syndrome (MMS). The discrimination between MMD and MMS has been validated by recent genetic researches and international reviews. Similarly to patients suffering from MMD, patients with MMS generally become symptomatic because of ischemic complications, which lead to hemiparesis, transient ischemic events, seizures, and sensory symptoms. RASopathies are a group of neurodevelopmental disorders that can be associated with MMS. We retrospectively reviewed 18 RASopathy patients with MMS treated at our institution from 2000 to 2015 (16 neurofibromatosis type 1, 1 Costello syndrome, and 1 Schimmelpenning syndrome). Here, we report clinical data, performed surgical procedures, and clinic-radiological outcome of these patients. Most of them received both indirect revascularization and medical therapy. At the moment, there are no univocal recommendations on which of these two treatment strategies is the treatment of choice in patients with RASopathies and MMS. We suggest that patients with a good overall prognosis (primarily depending on the distinctive underlying genetic disorder) and initial cerebrovascular disease could benefit from a prophylactic surgical revascularization, in order to prevent the cognitive impairment due to the progression of the vasculopathy.

  8. Patients' diets and preferences in a pediatric population with inflammatory bowel disease.

    Science.gov (United States)

    Green, T J; Issenman, R M; Jacobson, K

    1998-01-01

    To determine the dietary practices of the pediatric inflammatory bowel disease population at the Children's Hospital of the Hamilton Health Sciences Corporation and the reported effectiveness of those diets. A questionnaire mailed to 153 pediatric patients was returned by 125 patients (76 Crohn's disease [CD] and 49 ulcerative colitis [UC] patients)--an 82% response rate. The median age of respondents was 13 years, and 62% were male. Ninety per cent and 71% of CD and UC patients, respectively, had changed their diets since diagnosis. Caloric supplements (eg, BOOST [Mead Johnson Nutritionals]), sole source nutrition, low fibre and lactose-free diets were used by more than 15% of CD patients, whereas lactose-free, nonspicy, low acid, additive-free, caloric supplement and low fibre diets were used by more than 15% of UC patients. A diet supplement was more commonly used in CD patients (P juice. A benefit was reported for 103 of 141 reported diets, with the most commonly alleviated symptoms being abdominal pain, diarrhea and flatulence. Many children with inflammatory bowel disease have altered their diets to manage their disease and have attributed symptomatic relief to these diets.

  9. Mothers and Fathers Experience Stress of Congenital Heart Disease Differently: Recommendations for Pediatric Critical Care.

    Science.gov (United States)

    Sood, Erica; Karpyn, Allison; Demianczyk, Abigail C; Ryan, Jennie; Delaplane, Emily A; Neely, Trent; Frazier, Aisha H; Kazak, Anne E

    2018-03-10

    To inform pediatric critical care practice by examining how mothers and fathers experience the stress of caring for a young child with congenital heart disease and use hospital and community supports. Qualitative study of mothers and fathers of young children with congenital heart disease. Tertiary care pediatric hospital in the Mid-Atlantic region of the United States. Thirty-four parents (20 mothers, 14 fathers) from diverse backgrounds whose child previously underwent cardiac surgery during infancy. Subjects participated in semi-structured, individual interviews about their experiences and psychosocial needs at the time of congenital heart disease diagnosis, surgical admission, and discharge to home after surgery. Qualitative interview data were coded, and consistent themes related to emotional states, stressors, and supports were identified. Fathers experience and respond to the stressors and demands of congenital heart disease in unique ways. Fathers often described stress from not being able to protect their child from congenital heart disease and the associated surgeries/pain and from difficulties balancing employment with support for their partner and care of their congenital heart disease child in the hospital. Fathers were more likely than mothers to discuss support from the work environment (coworkers/managers, flexible scheduling, helpful distraction) and were less likely to describe the use of hospital-based resources or congenital heart disease peer-to-peer supports. This study highlights the importance of understanding the paternal experience and tailoring interventions to the unique needs of both mothers and fathers. Opportunities for critical care practice change to promote the mental health of mothers and fathers following a diagnosis of congenital heart disease are discussed.

  10. The potential of methylxanthine-based therapies in pediatric respiratory tract diseases.

    Science.gov (United States)

    Oñatibia-Astibia, Ainhoa; Martínez-Pinilla, Eva; Franco, Rafael

    2016-03-01

    Caffeine, theophylline and theobromine are the most known methylxanthines as they are present in coffee, tea and/or chocolate. In the last decades, a huge experimental effort has been devoted to get insight into the variety of actions that these compounds exert in humans. From such knowledge it is known that methylxanthines have a great potential in prevention, therapy and/or management of a variety of diseases. The benefits of methylxanthine-based therapies in the apnea of prematurity and their translational potential in pediatric affections of the respiratory tract are here presented. Copyright © 2016 Elsevier Ltd. All rights reserved.

  11. Colonic inflammation in pediatric inflammatory bowel disease: detection with magnetic resonance enterography

    Energy Technology Data Exchange (ETDEWEB)

    Campari, Alessandro [E. Bassini Hospital - ASST Nord Milano, Radiology Department, Milan (Italy); V. Buzzi Children' s Hospital - ASST Fatebenefratelli-Sacco, Pediatric Radiology Department, Milan (Italy); Napolitano, Marcello [V. Buzzi Children' s Hospital - ASST Fatebenefratelli-Sacco, Pediatric Radiology Department, Milan (Italy); Zuin, Giovanna [V. Buzzi Children' s Hospital - ASST Fatebenefratelli-Sacco, Pediatric Department, Milan (Italy); Maestri, Luciano [V. Buzzi Children' s Hospital - ASST Fatebenefratelli-Sacco, Pediatric Surgery Department, Milan (Italy); Di Leo, Giovanni [IRCCS Policlinico San Donato, Radiology Unit, Milan (Italy); Sardanelli, Francesco [IRCCS Policlinico San Donato, Radiology Unit, Milan (Italy); Universita degli Studi di Milano, Department of Biomedical Sciences for Health, Milan (Italy)

    2017-06-15

    Colonic involvement in pediatric inflammatory bowel disease is common. Magnetic resonance (MR) enterography is considered the best imaging modality for pediatric inflammatory bowel disease evaluation. It is unclear whether the lack of a dedicated large bowel preparation prevents a reliable colonic assessment. To determine the diagnostic performance of standard MR enterography in detecting and grading colonic inflammatory activity. We retrospectively evaluated children who underwent both MR enterography and ileocolonoscopy with biopsies <4 weeks apart. Two radiologists independently reviewed MR examinations and quantified inflammation in each of the five colonic segments using a standardized MR score system. Findings were compared with histological examination of the corresponding segment. Mann-Whitney, Kruskal-Wallis, Jonckheere-Terpstra and Bland-Altman statistics were used. One hundred seventy-five segments from 37 examinations were included. MR enterography diagnostic performance for inflammation was as follows: sensitivity 94% (95% confidence interval [CI]: 90-97%), specificity: 64% (95% CI: 57-71%). A significant positive correlation was found between MR score and inflammatory activity histologically graded (P<0.001, Jonckheere-Terpstra test). The interobserver agreement was good (mean difference between MR enterography scores was -0.03; limits of agreement -2.8 to 2.7). Standard MR enterography is sensitive for the detection of actively inflamed colonic segments. MR enterography might provide useful information for guiding biopsies and its role as an alternative to ileocolonoscopy in monitoring colonic disease activity in children should be further investigated. (orig.)

  12. Sono-Guided Percutaneous Automated Gun Biopsy in Pediatric Renal Disease

    International Nuclear Information System (INIS)

    Kim, Jong Chul

    1996-01-01

    To evaluate whether sono-guided percutaneous automated gun biopsy is also useful in pediatricpatients with renal diseases. In the prone position of twenty pediatric patients with renal parenchymal diseases, percutaneous biopsy was done through lateral aspect of the lower pole of left kidney with automated biopsy gun under the guidance of ultrasonography. The biopsy needle was either of 18 or 20 gauge. The obtained core of renal tissue was examined with light, immunofluorescent or electron microscope by the renal pathologist. In 18 among 20 patients, adequate renal tissue core sufficient to be pathologically diagnosed was obtained. The histologic findings were as follows : IG A nephropathy (n = 2), lupus nephritis (n =2), minimal change glomerulonephritis (n = 5), membranoproliferative glomerulonephritis (n = 3), mesangialproliferative glomeru-lonephritis (n = 1), diffuse proliferative glomerulonephritis (n = 3), focalglomerulo-sclerosis (n = 1), membranous glomerulopathy (n = 1). No significant complications occurred during or after the biopsy. Sono-guided percutaneous renal biopsy using automated biopsy gun is also useful todiagnose renal parenchymal diseases without significant complications in pediatric patients

  13. Retinal phenotype-genotype correlation of pediatric patients expressing mutations in the Norrie disease gene.

    Science.gov (United States)

    Wu, Wei-Chi; Drenser, Kimberly; Trese, Michael; Capone, Antonio; Dailey, Wendy

    2007-02-01

    To correlate the ophthalmic findings of patients with pediatric vitreoretinopathies with mutations occurring in the Norrie disease gene (NDP). One hundred nine subjects with diverse pediatric vitreoretinopathies and 54 control subjects were enrolled in the study. Diagnoses were based on retinal findings at each patient's first examination. Samples of DNA from each patient underwent polymerase chain reaction amplification and direct sequencing of the NDP gene. Eleven male patients expressing mutations in the NDP gene were identified in the test group, whereas the controls demonstrated wild-type NDP. All patients diagnosed as having Norrie disease had mutations in the NDP gene. Four of the patients with Norrie disease had mutations involving a cysteine residue in the cysteine-knot motif. Four patients diagnosed as having familial exudative vitreoretinopathy were found to have noncysteine mutations. One patient with retinopathy of prematurity had a 14-base deletion in the 5' untranslated region (exon 1), and 1 patient with bilateral persistent fetal vasculature syndrome expressed a noncysteine mutation in the second exon. Mutations disrupting the cysteine-knot motif corresponded to severe retinal dysgenesis, whereas patients with noncysteine mutations had varying degrees of avascular peripheral retina, extraretinal vasculature, and subretinal exudate. Patients exhibiting severe retinal dysgenesis should be suspected of carrying a mutation that disrupts the cysteine-knot motif in the NDP gene.

  14. Colonic inflammation in pediatric inflammatory bowel disease: detection with magnetic resonance enterography

    International Nuclear Information System (INIS)

    Campari, Alessandro; Napolitano, Marcello; Zuin, Giovanna; Maestri, Luciano; Di Leo, Giovanni; Sardanelli, Francesco

    2017-01-01

    Colonic involvement in pediatric inflammatory bowel disease is common. Magnetic resonance (MR) enterography is considered the best imaging modality for pediatric inflammatory bowel disease evaluation. It is unclear whether the lack of a dedicated large bowel preparation prevents a reliable colonic assessment. To determine the diagnostic performance of standard MR enterography in detecting and grading colonic inflammatory activity. We retrospectively evaluated children who underwent both MR enterography and ileocolonoscopy with biopsies <4 weeks apart. Two radiologists independently reviewed MR examinations and quantified inflammation in each of the five colonic segments using a standardized MR score system. Findings were compared with histological examination of the corresponding segment. Mann-Whitney, Kruskal-Wallis, Jonckheere-Terpstra and Bland-Altman statistics were used. One hundred seventy-five segments from 37 examinations were included. MR enterography diagnostic performance for inflammation was as follows: sensitivity 94% (95% confidence interval [CI]: 90-97%), specificity: 64% (95% CI: 57-71%). A significant positive correlation was found between MR score and inflammatory activity histologically graded (P<0.001, Jonckheere-Terpstra test). The interobserver agreement was good (mean difference between MR enterography scores was -0.03; limits of agreement -2.8 to 2.7). Standard MR enterography is sensitive for the detection of actively inflamed colonic segments. MR enterography might provide useful information for guiding biopsies and its role as an alternative to ileocolonoscopy in monitoring colonic disease activity in children should be further investigated. (orig.)

  15. Bartonelosis (Carrion's Disease) in the pediatric population of Peru: an overview and update.

    Science.gov (United States)

    Huarcaya, Erick; Maguiña, Ciro; Torres, Rita; Rupay, Joan; Fuentes, Luis

    2004-10-01

    Bartonellosis, or Carrion's Disease, is an endemic and reemerging disease in Peru and Ecuador. Carrion's Disease constitutes a health problem in Peru because its epidemiology has been changing, and it is affecting new areas between the highland and the jungle. During the latest outbreaks, and previously in endemic areas, the pediatric population has been the most commonly affected. In the pediatric population, the acute phase symptoms are fever, anorexia, malaise, nausea and/or vomiting. The main signs are pallor, hepatomegaly, lymphadenopathies, cardiac murmur, and jaundice. Arthralgias and weight loss have also commonly been described. The morbidity and mortality of the acute phase is variable, and it is due mainly to superimposed infections or associated respiratory, cardiovascular, neurological or gastrointestinal complications. The eruptive phase, also known as Peruvian Wart, is characterized by eruptive nodes (which commonly bleed) and arthralgias. The mortality of the eruptive phase is currently extremely low. The diagnosis is still based on blood culture and direct observation of the bacilli in a blood smear. In the chronic phase, the diagnosis is based on biopsy or serologic assays. There are nationally standardized treatments for the acute phase, which consist of ciprofloxacin, and alternatively chloramphenicol plus penicillin G. However, most of the treatments are based on evidence from reported cases. During the eruptive phase the recommended treatment is rifampin, and alternatively, azithromycin or erythromycin.

  16. Bartonelosis (Carrion's Disease in the pediatric population of Peru: an overview and update

    Directory of Open Access Journals (Sweden)

    Erick Huarcaya

    Full Text Available Bartonellosis, or Carrion's Disease, is an endemic and reemerging disease in Peru and Ecuador. Carrion's Disease constitutes a health problem in Peru because its epidemiology has been changing, and it is affecting new areas between the highland and the jungle. During the latest outbreaks, and previously in endemic areas, the pediatric population has been the most commonly affected. In the pediatric population, the acute phase symptoms are fever, anorexia, malaise, nausea and/or vomiting. The main signs are pallor, hepatomegaly, lymphadenopathies, cardiac murmur, and jaundice. Arthralgias and weight loss have also commonly been described. The morbidity and mortality of the acute phase is variable, and it is due mainly to superimposed infections or associated respiratory, cardiovascular, neurological or gastrointestinal complications. The eruptive phase, also known as Peruvian Wart, is characterized by eruptive nodes (which commonly bleed and arthralgias. The mortality of the eruptive phase is currently extremely low. The diagnosis is still based on blood culture and direct observation of the bacilli in a blood smear. In the chronic phase, the diagnosis is based on biopsy or serologic assays. There are nationally standardized treatments for the acute phase, which consist of ciprofloxacin, and alternatively chloramphenicol plus penicillin G. However, most of the treatments are based on evidence from reported cases. During the eruptive phase the recommended treatment is rifampin, and alternatively, azithromycin or erythromycin.

  17. Thyroid storm following radioactive iodine (RAI) therapy for pediatric graves disease.

    Science.gov (United States)

    Rohrs, Henry J; Silverstein, Janet H; Weinstein, David A; Amdur, Robert J; Haller, Michael J

    2014-01-01

    Female, 11 FINAL DIAGNOSIS: Thyroid storm Symptoms: Diarrhea • tachycardia • tachypnea • tremor • wheezing - Clinical Procedure: - Specialty: - Rare disease. A growing number of pediatric endocrinologists treat Graves disease with radioactive iodine (RAI) therapy due to the typically definitive nature of I-131 therapy. Given the published benefits and perceived low risks of RAI when compared to surgery or long-term anti-thyroid medication, the trend towards therapy with RAI is likely to continue. Nevertheless, RAI is not without significant risk. An 11-year-old girl with newly diagnosed Graves disease received RAI for definitive treatment of her hyperthyroidism. Within 24 hours of receiving I-131, she developed increasing sleepiness and eventually became unresponsive. Upon arrival at the emergency department she had a tonic-clonic seizure and was diagnosed with thyroid storm. Despite best efforts to manage her hyperthyroidism, she suffered a stroke of the left cerebral hemisphere that left her with persistent neurological deficits. Although thyroid storm after thyroid ablation is rare, the significant morbidity and potential mortality of pediatric thyroid storm warrant further studies to determine if children with markedly elevated thyroid hormone concentrations at diagnosis should receive prolonged pretreatment with anti-thyroid drugs. While such an approach may reduce the efficacy of I-131 ablation, it can also reduce and hopefully eliminate the risk of post-ablative thyroid storm.

  18. Unilateral and Bilateral Breast Cancer in Women Surviving Pediatric Hodgkin's Disease

    International Nuclear Information System (INIS)

    Basu, Swati K.; Schwartz, Cindy; Fisher, Susan G.; Hudson, Melissa M.; Tarbell, Nancy; Muhs, Ann; Marcus, Karen J.; Mendenhall, Nancy; Mauch, Peter; Kun, Larry E.; Constine, Louis S.

    2008-01-01

    Purpose: To define demographic and therapeutic associations with the risk of breast cancer in children treated for Hodgkin's disease (HD), particularly the frequency and interval to the development of contralateral breast cancer. Methods and Materials: All 398 female patients ( 12 years) were significant predictors of secondary breast cancer. Conclusions: Women surviving pediatric HD were found to have a 37-fold increase in the risk of breast cancer and a high likelihood of rapidly developing bilateral disease. Early-stage HD and age greater than 12 years at diagnosis of HD were independent risk factors. Higher radiation doses may augment risk, and pelvic radiation may be protective. Breast cancer screening methodology and frequency, plus the role of prophylaxis in patients with unilateral disease, require definition

  19. Impact of vessel wall lesions and vascular stenoses on cerebrovascular reactivity in patients with intracranial stenotic disease

    NARCIS (Netherlands)

    Cogswell, Petrice M; Davis, Taylor L; Strother, Megan K; Faraco, Carlos C; Scott, Allison O; Jordan, Lori C; Fusco, Matthew R; Frederick, Blaise deB; Hendrikse, Jeroen; Donahue, Manus J

    2017-01-01

    PURPOSE: To compare cerebrovascular reactivity (CVR) and CVR lagtimes in flow territories perfused by vessels with vs. without proximal arterial wall disease and/or stenosis, separately in patients with atherosclerotic and nonatherosclerotic (moyamoya) intracranial stenosis. MATERIALS AND METHODS:

  20. Utility of computed axial tomography angiography in anatomic evaluation of pediatric patients with congenital heart diseases

    International Nuclear Information System (INIS)

    Mosquera, Walter A; Reyes P, Rafael; Aguilera N, Favio M; Breton, Cesar A; Buitrago, Danuby A; Suarez J Ramiro; Castillo, Victor

    2007-01-01

    Although echocardiogram and cardiac catheterization are used as first option tools for congenital heart diseases diagnosis, computed tomography angiography is a minimally invasive exam that through two to three dimensional images in real time gives an adequate approach to patients having this type of pathologies that require a rapid and precise evaluation of its extra cardiac anatomy. Objective: describe the institutional experience from August 2005 to August 2006 in the use of angiography by tomography as a complementary diagnostic method in the evaluation of pediatric patients with congenital heart diseases. Method: serial descriptive study. 58 pediatric patients with clinical and echocardiographic diagnosis of congenital heart diseases were evaluated through the General Electric Multislice Light peed/16 scanner. Results: 58 patients with history of congenital heart disease were evaluated through CT angiography. Mean age was 2.4 ± 4.03 years. Twenty (33.8%) had diagnosis of pulmonary atresia, four (6.7%) had tricuspid atresia, eight (13.5%,) had double-outlet right ventricle, seven (11.8%) had tetralogy of Fallot, nine (15.2%) had alterations of the aortic arch, seven (11.8%) had coarctation of the aorta two (3.3%) had interrupted aortic arch, six (10.3%) had persistent ductus arteriosus, four (6.7%) had anomalous venous drainage and three (5.1 %) had transposition of the great arteries. High quality images that allowed assessing the precise vascular anatomy were obtained. Conclusions: computed tomography angiography turned out to be a useful tool in the diagnostic approach of congenital heart diseases, because it allowed a tridimensional anatomic reconstruction. New studies that may permit the assessment of sensitivity, specificity and concordance level of this technique with other invasive diagnostic methods available for the diagnosis of this type of diseases, are required

  1. Validation of Antibody-Based Strategies for Diagnosis of Pediatric Celiac Disease Without Biopsy.

    Science.gov (United States)

    Wolf, Johannes; Petroff, David; Richter, Thomas; Auth, Marcus K H; Uhlig, Holm H; Laass, Martin W; Lauenstein, Peter; Krahl, Andreas; Händel, Norman; de Laffolie, Jan; Hauer, Almuthe C; Kehler, Thomas; Flemming, Gunter; Schmidt, Frank; Rodrigues, Astor; Hasenclever, Dirk; Mothes, Thomas

    2017-08-01

    A diagnosis of celiac disease is made based on clinical, genetic, serologic, and duodenal morphology features. Recent pediatric guidelines, based largely on retrospective data, propose omitting biopsy analysis for patients with concentrations of IgA against tissue transglutaminase (IgA-TTG) >10-fold the upper limit of normal (ULN) and if further criteria are met. A retrospective study concluded that measurements of IgA-TTG and total IgA, or IgA-TTG and IgG against deamidated gliadin (IgG-DGL) could identify patients with and without celiac disease. Patients were assigned to categories of no celiac disease, celiac disease, or biopsy required, based entirely on antibody assays. We aimed to validate the positive and negative predictive values (PPV and NPV) of these diagnostic procedures. We performed a prospective study of 898 children undergoing duodenal biopsy analysis to confirm or rule out celiac disease at 13 centers in Europe. We compared findings from serologic analysis with findings from biopsy analyses, follow-up data, and diagnoses made by the pediatric gastroenterologists (celiac disease, no celiac disease, or no final diagnosis). Assays to measure IgA-TTG, IgG-DGL, and endomysium antibodies were performed by blinded researchers, and tissue sections were analyzed by local and blinded reference pathologists. We validated 2 procedures for diagnosis: total-IgA and IgA-TTG (the TTG-IgA procedure), as well as IgG-DGL with IgA-TTG (TTG-DGL procedure). Patients were assigned to categories of no celiac disease if all assays found antibody concentrations celiac disease if at least 1 assay measured antibody concentrations >10-fold the ULN. All other cases were considered to require biopsy analysis. ULN values were calculated using the cutoff levels suggested by the test kit manufacturers. HLA typing was performed for 449 participants. We used models that considered how specificity values change with prevalence to extrapolate the PPV and NPV to populations with lower

  2. Transition of pediatric to adult care in inflammatory bowel disease: Is it as easy as 1, 2, 3?

    Science.gov (United States)

    Afzali, Anita; Wahbeh, Ghassan

    2017-05-28

    Inflammatory bowel disease (IBD) is a heterogeneous group of chronic diseases with a rising prevalence in the pediatric population, and up to 25% of IBD patients are diagnosed before 18 years of age. Adolescents with IBD tend to have more severe and extensive disease and eventually require graduation from pediatric care toadult services. The transition of patients from pediatric to adult gastroenterologists requires careful preparation and coordination, with involvement of all key players to ensure proper collaboration of care and avoid interruption in care. This can be challenging and associated with gaps in delivery of care. The pediatric and adult health paradigms have inherent differences between health care models, as well as health care priorities in IBD. The readiness of the young adult also influences this transition of care, with often times other overlaps in life events, such as school, financial independence and moving away from home. These patients are therefore at higher risk for poorer clinical disease outcomes. The aim of this paper is to review concepts pertinent to transition of care of young adults with IBD to adult care, and provides resources appropriate for an IBD pediatric to adult transition of care model.

  3. Outcomes analysis of radioactive iodine and total thyroidectomy for pediatric Graves' disease.

    Science.gov (United States)

    Cohen, Reuven Zev; Felner, Eric I; Heiss, Kurt F; Wyly, J Bradley; Muir, Andrew B

    2016-03-01

    The majority of pediatric patients with Graves' disease will ultimately require definitive therapy in the form of radioactive iodine (RAI) ablation or thyroidectomy. There are few studies that directly compare the efficacy and complication rates between RAI and thyroidectomy. We compared the relapse rate as well as the acute and long-term complications of RAI and total thyroidectomy among children and adolescents with Graves' disease treated at our center. Medical records from 81 children and adolescents with a diagnosis of Graves' disease who received definitive therapy over a 12-year period were reviewed. Fifty one patients received RAI and 30 patients underwent thyroidectomy. The relapse rate was not significantly different between RAI and thyroidectomy (12.1% vs. 0.0%, p=0.28). There were no acute or long-term complications in the RAI group, but there were eight cases of hypoparathyroidism (two transient and six permanent) in the thyroidectomy group. None of the patients developed a recurrent laryngeal nerve injury. RAI is a safe and effective option for treatment of children and adolescents with Graves' disease. In light of the rate of permanent hypoparathyroidism seen at our center with thyroidectomy and previously published long-term safety of RAI, we recommend RAI as the first line treatment for children and adolescents with Graves' disease. For those centers performing thyroidectomies, we recommend that each center select 1-2 high-volume pediatric surgeons to perform all thyroid procedures, allowing individuals to increases case volume and potentially decrease long-term complications of thyroidectomy.

  4. Cognitive remediation in pediatric chronic kidney disease and end-stage kidney disease: rationale, candidate interventions, and applicability.

    Science.gov (United States)

    Javalkar, Karina; Ferris, Maria E; Cuttance, Jessica; Hooper, Stephen R

    2017-11-01

    The purpose of this paper is to address the potential use of cognitive remediation interventions for children and adolescents with chronic kidney disease (CKD) and end-stage kidney disease (ESKD). The prevalence and risk for neurocognitive dysfunction in children with this condition remains high, but, to date, interventions targeting these challenges have not been attempted either individually or as part of a larger treatment program. This is the next logical step in addressing the neurocognitive dysfunction that can be present in pediatric CKD/ESKD, with the field needing to determine the efficacy of cognitive remediation approaches for this population. To our knowledge, this paper is the first to raise this possibility by identifying candidate treatments addressing the neurocognitive challenges observed in children and adolescents with CKD/ESKD. Initially, we present the rationale for the importance of addressing the cognitive difficulties in this population, including an overview of the literature documenting the neurocognitive deficits associated with pediatric-onset CKD/ESKD. This is followed by a review of five candidate cognitive remediation programs that may be applicable to patients with this condition, and associated factors that could affect such treatment. The paper concludes with suggestions for both clinical and research initiatives that could be implemented to examine cognitive remediation as potential components of a larger treatment program for children and adolescents with CKD/ESKD.

  5. Plasma levels of acylated and total ghrelin in pediatric patients with chronic kidney disease.

    Science.gov (United States)

    Naufel, Maria Fernanda Soares; Bordon, Milena; de Aquino, Talita Marques; Ribeiro, Eliane Beraldi; de Abreu Carvalhaes, João Tomás

    2010-12-01

    This cross-sectional study set out to compare total and acyl ghrelin levels in children with mild chronic kidney disease (CKD) undergoing conservative treatment (n = 19) with children with end-stage renal disease (ESRD) undergoing hemodialysis (n = 24), and with healthy controls (n = 20). The relationship between ghrelin levels and parameters of renal function, nutritional status, and selective hormones were investigated. ESRD patients had higher total ghrelin levels than those with mild CKD or control individuals. However, acyl ghrelin did not differ between groups, indicating that the excess circulating ghrelin was desacylated. Since desacyl ghrelin has been shown to inhibit appetite, increased levels might contribute to protein-energy wasting in pediatric renal patients. When all 43 renal patients were combined, multiple regression analysis found age and glomerular filtration rate (GFR) to be significant negative predictors of total ghrelin. Acyl ghrelin was influenced negatively by age and positively by energy intake. Acyl to total ghrelin ratio related positively to GFR and energy intake. The results indicate that total but not acyl ghrelin is influenced by low GFR in children with CKD and suggests that ghrelin activation may be impaired in these patients. Since energy intake is a positive predictor of acyl ghrelin, the physiological control of ghrelin secretion appears to be altered in pediatric renal patients.

  6. Alterations of the Subgingival Microbiota in Pediatric Crohn's Disease Studied Longitudinally in Discovery and Validation Cohorts.

    Science.gov (United States)

    Kelsen, Judith; Bittinger, Kyle; Pauly-Hubbard, Helen; Posivak, Leah; Grunberg, Stephanie; Baldassano, Robert; Lewis, James D; Wu, Gary D; Bushman, Frederic D

    2015-12-01

    Oral manifestations are common in Crohn's disease (CD). Here we characterized the subgingival microbiota in pediatric patients with CD initiating therapy and after 8 weeks to identify microbial community features associated with CD and therapy. Pediatric patients with CD were recruited from The Children's Hospital of Pennsylvania. Healthy control subjects were recruited from primary care or orthopedics clinic. Subgingival plaque samples were collected at initiation of therapy and after 8 weeks. Treatment exposures included 5-ASAs, immunomodulators, steroids, and infliximab. The microbiota was characterized by 16S rRNA gene sequencing. The study was repeated in separate discovery (35 CD, 43 healthy) and validation cohorts (43 CD, 31 healthy). Most subjects in both cohorts demonstrated clinical response after 8 weeks of therapy (discovery cohort 88%, validation cohort 79%). At week 0, both antibiotic exposure and disease state were associated with differences in bacterial community composition. Seventeen genera were identified in the discovery cohort as candidate biomarkers, of which 11 were confirmed in the validation cohort. Capnocytophaga, Rothia, and TM7 were more abundant in CD relative to healthy controls. Other bacteria were reduced in abundance with antibiotic exposure among CD subjects. CD-associated genera were not enriched compared with healthy controls after 8 weeks of therapy. Subgingival microbial community structure differed with CD and antibiotic use. Results in the discovery cohort were replicated in a separate validation cohort. Several potentially pathogenic bacterial lineages were associated with CD but were not diminished in abundance by antibiotic treatment, suggesting targets for additional surveillance.

  7. Discordant Impact of HLA on Viral Replicative Capacity and Disease Progression in Pediatric and Adult HIV Infection.

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    Emily Adland

    2015-06-01

    Full Text Available HLA class I polymorphism has a major influence on adult HIV disease progression. An important mechanism mediating this effect is the impact on viral replicative capacity (VRC of the escape mutations selected in response to HLA-restricted CD8+ T-cell responses. Factors that contribute to slow progression in pediatric HIV infection are less well understood. We here investigate the relationship between VRC and disease progression in pediatric infection, and the effect of HLA on VRC and on disease outcome in adult and pediatric infection. Studying a South African cohort of >350 ART-naïve, HIV-infected children and their mothers, we first observed that pediatric disease progression is significantly correlated with VRC. As expected, VRCs in mother-child pairs were strongly correlated (p = 0.004. The impact of the protective HLA alleles, HLA-B*57, HLA-B*58:01 and HLA-B*81:01, resulted in significantly lower VRCs in adults (p<0.0001, but not in children. Similarly, in adults, but not in children, VRCs were significantly higher in subjects expressing the disease-susceptible alleles HLA-B*18:01/45:01/58:02 (p = 0.007. Irrespective of the subject, VRCs were strongly correlated with the number of Gag CD8+ T-cell escape mutants driven by HLA-B*57/58:01/81:01 present in each virus (p = 0.0002. In contrast to the impact of VRC common to progression in adults and children, the HLA effects on disease outcome, that are substantial in adults, are small and statistically insignificant in infected children. These data further highlight the important role that VRC plays both in adult and pediatric progression, and demonstrate that HLA-independent factors, yet to be fully defined, are predominantly responsible for pediatric non-progression.

  8. Celiac Disease and Pediatric Type 1 Diabetes: Diagnostic and Treatment Dilemmas

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    Daneman Denis

    2010-05-01

    Full Text Available Abstract Despite the advent of sensitive and specific serologic testing, routine screening for celiac disease (CD in diabetic populations may not be universal practice, and many clinicians struggle to find the optimal approach to managing CD in pediatric Type 1 diabetes (T1D patients. While some clinicians advocate screening for CD in all patients with T1D, others are unsure whether this is warranted. The diagnosis of patients who present with symptomatic CD, including malabsorption and obvious pathology upon biopsy, remains straightforward, with improvements noted on a gluten-free diet. Many patients identified by screening, however, tend to be asymptomatic. Evidence is inconclusive as to whether the benefits of screening and potentially treating asymptomatic individuals outweigh the harms of managing a population already burdened with a serious illness. This review focuses on current knowledge of CD in children and youth with T1D, highlighting important elements of the disease's pathophysiology, epidemiology, clinical presentation, and diagnostic challenges.

  9. A Disease-Associated Microbial and Metabolomics State in Relatives of Pediatric Inflammatory Bowel Disease PatientsSummary

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    Jonathan P. Jacobs

    2016-11-01

    Full Text Available Background & Aims: Microbes may increase susceptibility to inflammatory bowel disease (IBD by producing bioactive metabolites that affect immune activity and epithelial function. We undertook a family based study to identify microbial and metabolic features of IBD that may represent a predisease risk state when found in healthy first-degree relatives. Methods: Twenty-one families with pediatric IBD were recruited, comprising 26 Crohn’s disease patients in clinical remission, 10 ulcerative colitis patients in clinical remission, and 54 healthy siblings/parents. Fecal samples were collected for 16S ribosomal RNA gene sequencing, untargeted liquid chromatography–mass spectrometry metabolomics, and calprotectin measurement. Individuals were grouped into microbial and metabolomics states using Dirichlet multinomial models. Multivariate models were used to identify microbes and metabolites associated with these states. Results: Individuals were classified into 2 microbial community types. One was associated with IBD but irrespective of disease status, had lower microbial diversity, and characteristic shifts in microbial composition including increased Enterobacteriaceae, consistent with dysbiosis. This microbial community type was associated similarly with IBD and reduced microbial diversity in an independent pediatric cohort. Individuals also clustered bioinformatically into 2 subsets with shared fecal metabolomics signatures. One metabotype was associated with IBD and was characterized by increased bile acids, taurine, and tryptophan. The IBD-associated microbial and metabolomics states were highly correlated, suggesting that they represented an integrated ecosystem. Healthy relatives with the IBD-associated microbial community type had an increased incidence of elevated fecal calprotectin. Conclusions: Healthy first-degree relatives can have dysbiosis associated with an altered intestinal metabolome that may signify a predisease microbial

  10. Demographic, Clinical and Genetic Characteristics of Child Gaucher Disease Patients in Russia: Pediatric Register Data

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    G. B. Movsisyan

    2016-01-01

    Full Text Available Background: Registers are an effective tool for tracing the dynamics of patients with rare pathologies.Objective: Our aim was to examine the demographic, clinical and genetic features of child Gaucher disease patients in Russia.Methods: We held a retrospective survey of the pediatric register data with regard to children suffering from Gaucher disease. The period of data accounting was from 2006 to 2016.Results: 115 children with Gaucher disease aged from 3 months to 17 years (the median age of diagnosis is 5 years were registered; 62 them (53.9% are girls. The prevalence of the disease was 0.32 cases for 100,000 children. 95 (82.6% children had 1st type of Gaucher disease, 6 (5.2% — 2nd, and 1 (12.2% — 3rd. Maximum morbidity was in Central (27; 23.5% and Volga (27; 23.5% Federal Districts; minimal — in the Far East (3; 2.6%. By the time of diagnosis all the patients were suffering from splengomegaly. The genotype and phenotype correlations in 90 children with Gaucher disease were as follows: in case of 1st type (n = 77, in 21 (27.3% cases, the p.N370S/р.L444P genotype was set, in 12 (15.6% — the р.N370S/other mutation; in case of 2nd and 3rd types, in 13 children with neuropathic forms, in 9 (62.9% cases — the p.L444P/p.L444P, in 3 (231% — the p.L444P/p. D409H. The rest of genotypes were presented by other mutations, 13 of which were revealed for the first time. The p.W223R (p.W184R mutation is specific for Russian patients. Enzyme replacement therapy was carried out for 109 patients (94.8%: in 105 (96.3% children (1st and 3rd types of Gaucher disease with imiglucerase, in 4 (3.7% children with 1st type — with velaglucerase alfa. Pathogenetic treatment stops the main symptoms in most patients.Conclusion: The pediatric Gaucher disease register allows to systemize the data concerning the disease course in children and optimizing the approaches to its monitoring in Russia.

  11. Prevalence of Lactose Malabsorption and Lactose Intolerance in Pediatric Patients with Selected Gastrointestinal Diseases.

    Science.gov (United States)

    Pawłowska, Katarzyna; Umławska, Wioleta; Iwańczak, Barbara

    2015-01-01

    Lactase is an enzyme involved in the hydrolysis of lactose. Deficiency of the enzyme (hypolactasia) may be determined genetically or arise secondarily to disease of small intestine. Under this condition, lactose enters the colon where it is fermented by intestinal microflora and turns to gases and short-chain fatty acids, causing gastrointestinal symptoms known as lactose intolerance (LI). To investigate the incidence of lactose malabsorption (LM), LI and the coexistence of these two conditions in children with upper gastrointestinal tract diseases (UGTD), malabsorption syndrome, inflammatory bowel disease (IBD) and functional gastrointestinal disorders (FGID). Hydrogen breath test (HBT) was conducted in 387 pediatric patients in years 2010-2013. Two hundred thirty two children with gastrointestinal tract diseases were selected and assigned to groups - UGTD, malabsorption syndrome, IBD or FGID. For each group the frequency of LM, frequency and severity of LI and the frequency of their co-occurrence were calculated. Lactose malabsorption was observed in 37.08% of patients with gastrointestinal diseases. Positive HBT result was the most common in children with malabsorption syndrome (52.50%) and less common in UGTD (30.85%), especially in ulcer disease (23.53%). Symptoms after lactose ingestion affected 36.64% of the subjects, and were more specific to lactose malabsorbers than to lactose absorbers (72.10% vs. 15.75%). The higher frequency of LI was noted in children with FGID, especially in irritable bowel syndrome (IBS) (65.22%). The lowest incidence of symptoms was obtained in children with UGTD, especially in those with ulcer disease (27.44%). The incidence of LM with LI was noted in 27.16% of all patients and was the highest in IBS (47.83%) and the lowest in ulcer disease (15.78%). Lactose malabsorption is a common problem in children with gastrointestinal diseases, especially in children with bowel diseases. Lactose intolerance is related to LM, but does not

  12. Pediatric MS

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    ... Pediatric MS Share this page Facebook Twitter Email Pediatric MS Pediatric MS Pediatric MS Support Pediatric Providers ... system through the Pediatric MS Support Group . Treating pediatric MS In 2018 the U.S. Food and Drug ...

  13. Short-term effects of ambient air pollution on pediatric outpatient visits for respiratory diseases in Yichang city, China

    International Nuclear Information System (INIS)

    Liu, Yuewei; Xie, Shuguang; Yu, Qing; Huo, Xixiang; Ming, Xiaoyan; Wang, Jing; Zhou, Yun; Peng, Zhe; Zhang, Hai; Cui, Xiuqing; Xiang, Hua; Huang, Xiji; Zhou, Ting; Chen, Weihong; Shi, Tingming

    2017-01-01

    Previous studies have suggested that short-term exposure to ambient air pollution was associated with pediatric hospital admissions and emergency room visits for certain respiratory diseases; however, there is limited evidence on the association between short-term air pollution exposure and pediatric outpatient visits. Our aim was to quantitatively assess the short-term effects of ambient air pollution on pediatric outpatient visits for respiratory diseases. We conducted a time-series study in Yichang city, China between Jan 1, 2014 and Dec 31, 2015. Daily counts of pediatric respiratory outpatient visits were collected from 3 large hospitals, and then linked with air pollution data from 5 air quality monitoring stations by date. We used generalized additive Poisson models to conduct linear and nonlinear exposure-response analyses between air pollutant exposures and pediatric respiratory outpatient visits, adjusting for seasonality, day of week, public holiday, temperature, and relative humidity. Each interquartile range (IQR) increase in PM 2.5 (lag 0), PM 10 (lag 0), NO 2 (lag 0), CO (lag 0), and O 3 (lag 4) concentrations was significantly associated with a 1.91% (95% CI: 0.60%, 3.23%), 2.46% (1.09%, 3.85%), 1.88% (0.49%, 3.29%), 2.00% (0.43%, 3.59%), and 1.91% (0.45%, 3.39%) increase of pediatric respiratory outpatient visits, respectively. Similarly, the nonlinear exposure-response analyses showed monotonic increases of pediatric respiratory outpatient visits by increasing air pollutant exposures, though the associations for NO 2 and CO attenuated at higher concentrations. These associations were unlikely modified by season. We did not observe significant association for SO 2 exposure. Our results suggest that short-term exposures to PM 2.5 , PM 10 , NO 2 , CO, and O 3 may account for increased risk of pediatric outpatient visits for respiratory diseases, and emphasize the needs for reduction of air pollutant exposures for children. - Highlights: • PM 2

  14. Symptoms and Mucosal Changes Stable During Rapid Increase of Pediatric Celiac Disease in Norway.

    Science.gov (United States)

    Beitnes, Ann-Christin R; Vikskjold, Florin B; Jóhannesdóttir, Gróa B; Perminow, Gøri; Olbjørn, Christine; Andersen, Solveig N; Bentsen, Beint S; Rugtveit, Jarle; Størdal, Ketil

    2017-04-01

    We aimed to study whether the incidence of pediatric celiac disease (CD) in South-Eastern Norway changed from 2000 to 2010. We also examined whether there was a change in symptoms and histopathological morphology in the duodenal biopsies during the same period. In 3 hospitals in South-Eastern Norway, records from pediatric patients (0-14.9 years) diagnosed with CD during two 3-year periods (2000-2002 and 2008-2010) were reviewed. Only cases with a duodenal biopsy diagnosis of CD classified as Marsh grade 2 and 3a-c were included. Frequencies of symptoms, anthropometric data, and laboratory results were compared, in addition to re-examinations of histological sections from one of the hospitals. A total of 400 cases were diagnosed with a female to male ratio of 1.5:1. The incidence rate for 2000 to 2002 was 15.9 cases per 100,000 person-years (95% confidence interval 12.8-19.4), compared with 45.5 cases per 100,000 person-years during 2008 to 2010 (95% confidence interval 40.5-50.9), P symptoms and the distribution of histopathological changes were similar in the 2 periods, whereas weight z scores and hemoglobin levels were significantly lower in the first period. We found a 3-fold increase in the incidence rate for CD in the Norwegian pediatric population during the decade 2000 to 2010. Slightly higher weight and hemoglobin levels at diagnosis in the latter period may be due to improved CD awareness. Unaltered relative frequencies of symptoms and histopathological changes in the gut, however, suggest a true increase of CD in Norwegian children.

  15. Prescribing for Children With Rheumatic Disease: Perceived Treatment Approaches Between Pediatric and Adult Rheumatologists.

    Science.gov (United States)

    van Mater, Heather; Balevic, Stephen J; Freed, Gary L; Clark, Sarah J

    2018-02-01

    To compare practice patterns and prescribing differences for juvenile idiopathic arthritis (JIA) between adult rheumatologists (ARs) and pediatric rheumatologists (PRs), the perceived educational needs, and factors that enhance or impede co-management. Two parallel, cross-sectional surveys focusing on JIA were administered in 2009 to a random sample of 193 PRs and 500 ARs using the American College of Rheumatology membership file. Bivariate analysis was conducted for common items. The response rate was 62.1% for ARs (n = 306) and 72.3% for PRs (n = 138). Only 23% of responding ARs (n = 69) reported caring for children with JIA. Of these, 94% strongly agreed/agreed feeling comfortable diagnosing JIA; however, only 76% felt comfortable treating JIA. Clinical vignettes highlighted several prescribing differences. Forty-eight percent of ARs and 31% of PRs felt medications to treat JIA did not have clear dosing guidelines. Though PRs initiated disease-modifying antirheumatic drugs and biologic agents earlier, treatments were similar after 3 months. To enhance co-management, 74% of pediatric respondents endorsed shared medical records. Nearly one-quarter of surveyed ARs care for children with JIA, with most limiting their practice to older children. There was more discomfort in treating JIA than diagnosing it, and there were significant prescribing differences. Both provider types identified the need for better dosing and treatment resources. Updated management guidelines along with exposure to pediatric rheumatology in fellowship could reduce treatment differences and enhance the care of children with JIA. Shared medical records and improvement in reimbursement may optimize co-management. © 2017, American College of Rheumatology.

  16. X-ray diagnosis and treatment for severe respiratory complications during cardiac catheterizations on pediatric congenital heart disease

    International Nuclear Information System (INIS)

    Wang Cheng; Zhao Shihua; Jiang Shiliang; Huang Lianjun; Xu Zhongying; Ling Jian; Zheng Hong; Xie Ruolan; Lu Minjie

    2005-01-01

    Objective: To study the radiological features of severe respiratory complications during cardiac catheterizations on pediatric congenital heart disease so as to make early diagnosis and treatment. We also intend to find the mechanism of these complications. Methods: A total of 9 pediatric cases with severe respiratory complications during cardiac catheterizations were included in the study. The clinical manifestations, radiological features, and corresponding treatments were reviewed. Results: Most of the cases had severe hypoxia, with 6 cases presenting with bradycardia. Opacification of two lung fields was found in 7 cases, pulmonary edema in 1 case, and atelectasis of the upper right lung in 1 case. With intubation, oxygen inhalation and administration of certain drugs, all cases were saved except 1 case with pulmonary edema. Conclusion: Severe respiratory complications during cardiac catheterizations on pediatric congenital heart disease are emergent and critical, and they often presented with various manifestations. Early diagnosis and correct treatment are the key to successful salvage. (authors)

  17. Association of Food Allergies, Cow’s Milk Allergy, and Asthma With Pediatric Inflammatory Bowel Disease

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    Gholam-Hossein Fallahi

    2018-05-01

    Full Text Available There are controversies on the association of childhood allergic diseases with inflammatory bowel diseases (IBD. The aim of this study was to examine the association between food allergy, cow’s milk allergy (CMA, and asthma with pediatric IBD in Iranian population. This case-control study was conducted on 200 individuals less than 18-year-old (100 with IBD and 100 as control group. Medical records, clinical presentation, and laboratory and para-clinical findings related to food allergy, CMA, and asthma were reviewed for all participants in both groups and were recorded. Among 100 children with IBD, 40 had Crohn's disease, and 60 had ulcerative colitis. The frequency of food allergy, cow's milk allergy, and asthma in children with IBD was significantly higher than the control group (P<0.001. Asthma in children with Crohn's disease was significantly more prevalent than children with ulcerative colitis (P=0.008. Food allergy (OR: 22.1, 95% CI: 5.1-95.05, P<0.001, CMA (OR: 15, 95% CI: 3-67, P<0.001, and asthma (OR: 10, 95% CI: 3-37.05, P<0.001 were significantly associated with increased risk of IBD in children. Food allergy, CMA in infancy and asthma are more prevalent in children with different subtypes of IBD. The diagnosis of these risk factors is associated with increased risk of Crohn's disease and ulcerative colitis.

  18. Inflammation, Antibiotics, and Diet as Environmental Stressors of the Gut Microbiome in Pediatric Crohn's Disease.

    Science.gov (United States)

    Lewis, James D; Chen, Eric Z; Baldassano, Robert N; Otley, Anthony R; Griffiths, Anne M; Lee, Dale; Bittinger, Kyle; Bailey, Aubrey; Friedman, Elliot S; Hoffmann, Christian; Albenberg, Lindsey; Sinha, Rohini; Compher, Charlene; Gilroy, Erin; Nessel, Lisa; Grant, Amy; Chehoud, Christel; Li, Hongzhe; Wu, Gary D; Bushman, Frederic D

    2015-10-14

    Abnormal composition of intestinal bacteria--"dysbiosis"-is characteristic of Crohn's disease. Disease treatments include dietary changes and immunosuppressive anti-TNFα antibodies as well as ancillary antibiotic therapy, but their effects on microbiota composition are undetermined. Using shotgun metagenomic sequencing, we analyzed fecal samples from a prospective cohort of pediatric Crohn's disease patients starting therapy with enteral nutrition or anti-TNFα antibodies and reveal the full complement and dynamics of bacteria, fungi, archaea, and viruses during treatment. Bacterial community membership was associated independently with intestinal inflammation, antibiotic use, and therapy. Antibiotic exposure was associated with increased dysbiosis, whereas dysbiosis decreased with reduced intestinal inflammation. Fungal proportions increased with disease and antibiotic use. Dietary therapy had independent and rapid effects on microbiota composition distinct from other stressor-induced changes and effectively reduced inflammation. These findings reveal that dysbiosis results from independent effects of inflammation, diet, and antibiotics and shed light on Crohn disease treatments. Copyright © 2015 Elsevier Inc. All rights reserved.

  19. Proteomics: a tool to develop novel diagnostic methods and unravel molecular mechanisms of pediatric diseases.

    Science.gov (United States)

    Torres-Arroyo, Angélica; Ruiz-Lara, Arturo; Castillo-Villanueva, Adriana; Méndez-Cruz, Sara Teresa; Espinosa-Padilla, Sara Elvia; Espinosa-Rosales, Francisco Javier; Zarate-Mondragón, Flora; Cervantes-Bustamante, Roberto; Bosch-Canto, Vanessa; Vizzuett-López, Iris; Ordaz-Fávila, Juan Carlos; Oria-Hernández, Jesús; Reyes-Vivas, Horacio

    Proteomics is the study of the expression of changes and post-translational modifications (PTM) of proteins along a metabolic condition either normal or pathological. In the field of health, proteomics allows obtaining valuable data for treatment, diagnosis or pathophysiological mechanisms of different illnesses. To illustrate the aforementioned, we describe two projects currently being performed at the Instituto Nacional de Pediatría: The immuno-proteomic study of cow milk allergy and the Proteomic study of childhood cataract. Cow's milk proteins (CMP) are the first antigens to which infants are exposed and generate allergy in some of them. In Mexico, the incidence of CMP allergy has been estimated at 5-7%. Clinical manifestations include both gastrointestinal and extra-gastrointestinal symptoms, making its diagnosis extremely difficult. An inappropriate diagnosis affects the development and growth of children. The goals of the study are to identify the main immune-reactive CMP in Mexican pediatric population and to design more accurate diagnostic tools for this disease. Childhood cataract is a major ocular disease representing one of the main causes of blindness in infants; in developing countries, this disease promotes up to 27% of cases related to visual loss. From this group, it has been estimated that close to 60% of children do not survive beyond two years after vision lost. PTM have been pointed out as the main cause of protein precipitation at the crystalline and, consequently, clouding of this tissue. The study of childhood cataract represents an outstanding opportunity to identify the PTM associated to the cataract-genesis process. Copyright © 2017 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  20. Diagnosis features of pediatric Gaucher disease patients in the era of enzymatic therapy, a national-base study from the Spanish Registry of Gaucher Disease.

    Science.gov (United States)

    Andrade-Campos, Marcio; Alfonso, Pilar; Irun, Pilar; Armstrong, Judith; Calvo, Carmen; Dalmau, Jaime; Domingo, Maria-Rosario; Barbera, Jose-Luis; Cano, Horacio; Fernandez-Galán, Maria-Angeles; Franco, Rafael; Gracia, Inmaculada; Gracia-Antequera, Miguel; Ibañez, Angela; Lendinez, Francisco; Madruga, Marcos; Martin-Hernández, Elena; O'Callaghan, Maria Del Mar; Del Soto, Alberto Pérez; Del Prado, Yolanda Ruiz; Sancho-Val, Ignacio; Sanjurjo, Pablo; Pocovi, Miguel; Giraldo, Pilar

    2017-05-03

    The enzymatic replacement therapy (ERT) availability for Gaucher disease (GD) has changed the landscape of the disease, several countries have screening programs. These actions have promoted the early diagnosis and avoided many complications in pediatric patients. In Spain ERT has been available since 1993 and 386 patients have been included in the Spanish Registry of Gaucher Disease (SpRGD). The aim of this study is to analyze the impact of ERT on the characteristics at time of diagnosis and initial complications in pediatric Gaucher disease patients. To analyze the impact of ERT on the characteristics at time of diagnosis and initial complications in pediatric Gaucher disease patients. A review of data in SpRGD from patients' diagnosed before 18 years old was performed. The cohort was split according the year of diagnosis (≤1994, cohort A; ≥1995, cohort B). A total of 98 pediatric patients were included, GD1: 80, GD3: 18; mean age: 7.2 (0.17-16.5) years, 58 (59.2%) males and 40 (40.8%) females. Forty-five were diagnosed ≤ 1994 and 53 ≥ 1995. Genotype: N370S/N370S: 2 (2.0%), N370S/L444P: 27 (27.5%), N370S/other: 47 (48%), L444P/L444P: 7 (7.1%), L444P/D409H: 2 (2.0%), L444P/other: 3 (6.2%), other/other: 10 (10.2%). The mean age at diagnosis was earlier in patients diagnosed after 1995 (p Gaucher disease in the era of ERT availability has permitted to reduce the incidence of severe and irreversible initial complication in pediatric patients, and this has permitted better development of these patients. This is the largest pediatric cohort from a national registry.

  1. Prevalence of Attention Deficit Hyperactivity Disorder in Pediatrics Patients Newly Diagnosed with Gastroesophageal Reflux Disease

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    Mohammad Effatpanah

    2017-09-01

    Full Text Available Background: Gastroesophageal reflux disease (GERD is associated with a number of comorbidities in pediatrics. However, its association with attention deficit hyperactivity disorder (ADHD has not been reported. The aim of the present study was to investigate the prevalence of ADHD in pediatric patients newly diagnosed with GERD. Materials and Methods: Sixty newly-diagnosed treatment naive GERD patients and sixty healthy controls aging between 5 to 12 years referring to the Children and Adolescent’s medical center, Tehran, Iran were recruited in a case-control study during the year 2015. Then patients were evaluated for ADHD by a psychiatrist according to the DSM-IV criteria. The revised Conners' Parent Rating Scale (CPRS-R was used for assessment of the symptoms of ADHD. To screen for psychiatry disorders other than ADHD, the Kiddie-Sads-Present and Lifetime Version (K-SADS-PL questionnaire was used. Logistic regression analysis was used for modeling the association between GERD and ADHD in the study sample. Results: The mean age of GERD patients was 5.77±2.27 and for non-GERD controls was 6.03±2.52 (P= 0.543. Thirty-three out of 60 (55% GERD patients and 37 out of 60(61.66% non-GERD controls were male (P: 0.579. Prevalence of ADHD was 33.60 (55% in GERD patients and 10.60 (16.66% in non-GERD (P

  2. Fecal Microbiota in Pediatric Inflammatory Bowel Disease and Its Relation to Inflammation.

    Science.gov (United States)

    Kolho, Kaija-Leena; Korpela, Katri; Jaakkola, Tytti; Pichai, Madharasi V A; Zoetendal, Erwin G; Salonen, Anne; de Vos, Willem M

    2015-06-01

    Inflammatory bowel disease (IBD) is considered to result from interplay between host and intestinal microbiota. While IBD in adults has shown to be associated with marked changes in the intestinal microbiota, there are only a few studies in children, and particularly studies focusing on therapeutic responses are lacking. Hence, this prospective study addressed the intestinal microbiota in pediatric IBD especially related to the level of inflammation. In total, 68 pediatric patients with IBD and 26 controls provided stool and blood samples in a tertiary care hospital and 32 received anti-tumor necrosis factor-α (anti-TNF-α). Blood inflammatory markers and fecal calprotectin levels were determined. The intestinal microbiota was characterized by phylogenetic microarray and qPCR analysis. The microbiota varied along a gradient of increasing intestinal inflammation (indicated by calprotectin levels), which was associated with reduced microbial richness, abundance of butyrate producers, and relative abundance of Gram-positive bacteria (especially Clostridium clusters IV and XIVa). A significant association between microbiota composition and inflammation was indicated by a set of bacterial groups predicting the calprotectin levels (area under curve (AUC) of 0.85). During the induction of anti-TNF-α, the microbial diversity and similarity to the microbiota of controls increased in the responder group by week 6, but not in the non-responders (PEubacterium rectale and Bifidobacterium spp. predicted the response to anti-TNF-α medication. Intestinal microbiota represents a potential biomarker for correlating the level of inflammation and therapeutic responses to be further validated.

  3. A rare cause of facial nerve palsy in children : Hyperostosis corticalis generalisata (Van Buchem disease). Three new pediatric cases and a literature review

    NARCIS (Netherlands)

    van Egmond, M. E.; Dikkers, F. G.; Boot, A. M.; van Lierop, A. H. J. M.; Papapoulos, S. E.; Brouwer, O. F.

    2012-01-01

    Differential diagnosis of facial nerve palsy in children is extensive. We report on three pediatric cases presenting with facial nerve palsy caused by hyperostosis corticalis generalisata (Van Buchem disease). This autosomal recessive disease is characterized by progressive bone overgrowth, with

  4. 78 FR 58316 - Complex Issues in Developing Medical Devices for Pediatric Patients Affected by Rare Diseases...

    Science.gov (United States)

    2013-09-23

    ... Office of Orphan Products Development (OOPD) and is being held in conjunction with the Center for Drug... process; Pediatric Specialty-Specific Practice Areas; Clinical Trials and Registries; and Pediatric Needs... pediatric populations? B. HUD/HDE 1. Is there any confusion about the designation process for HUDs or the...

  5. Optimization of folic acid, vitamin B-12, and vitamin B-6 supplements in pediatric patients with sickle cell disease

    NARCIS (Netherlands)

    van der Dijs, Fey P L; Fokkema, M Rebecca; Dijck-Brouwer, D A Janneke; Niessink, Bram; van der Wal, Thaliet I C; Schnog, John-John B; Duits, Ashley J; Muskiet, Fred D; Muskiet, Frits A J

    Using homocysteine as a functional marker, we determined optimal folic acid, vitamin B-12, and vitamin B-6 dosages in 21 pediatric sickle cell disease (SCD) patients (11 HbSS, 10 HbSC; 7-16 years). Daily supplements of folic acid (400, 700, or 1,000 mug), vitamin B-12 (1, 3, or 5 U.S. 1989 RDA), and

  6. Dosimetric studies in monocorte TC teams in adult and pediatric and its application in children with chronic neurological diseases

    International Nuclear Information System (INIS)

    Casal, M. D.; Jimenez, M.; Urena, A.; Sanchez, G.; Herrador, M.

    2010-01-01

    The aim of this paper is to measure and analyze some of the parameters directly related to the doses administrated both for adult and pediatric patients in clinical practice at the Hospital Virgen del Rocio, in Seville. Among the latter group of patients doses in also estimated in children suffering from chronic neurological diseases. (Author) 14 refs.

  7. Young adult patients with a history of pediatric disease: impact on course of life and transition into adulthood

    NARCIS (Netherlands)

    Stam, Heleen; Hartman, Esther E.; Deurloo, Jacqueline A.; Groothoff, Jaap; Grootenhuis, Martha A.

    2006-01-01

    PURPOSE: To assess the course of life of young adults who grew up with a chronic or life-threatening disease, and to compare their course of life with that of peers from the general population. Optimal transition from pediatric to adult health care requires knowledge of the psychosocial history of

  8. Lyme disease and pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS: an overview

    Directory of Open Access Journals (Sweden)

    Rhee H

    2012-02-01

    Full Text Available Hanna Rhee1, Daniel J Cameron21Medicine, San Diego, CA, 2Northern Westchester Hospital, Mount Kisco, NY, USAAbstract: Lyme disease (LD is a complex, multisystemic illness. As the most common vector-borne disease in the United States, LD is caused by bacterial spirochete Borrelia burgdorferi sensu stricto, with potential coinfections from agents of anaplasmosis, babesiosis, and ehrlichiosis. Persistent symptoms and clinical signs reflect multiorgan involvement with episodes of active disease and periods of remission, not sparing the coveted central nervous system. The capability of microorganisms to cause and exacerbate various neuropsychiatric pathology is also seen in pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS, a recently described disorder attributed to bacterium Streptococcus pyogenes of group A beta-hemolytic streptococcus in which neurologic tics and obsessive-compulsive disorders are sequelae of the infection. In the current overview, LD and PANDAS are juxtaposed through a review of their respective infectious etiologies, clinical presentations, mechanisms of disease development, courses of illness, and treatment options. Future directions related to immunoneuropsychiatry are also discussed.Keywords: neuroborreliosis, infection, obsessive-compulsive disorder, tic disorder, Borrelia burgdorferi, strep throat

  9. Pain Management for Sickle Cell Disease in the Pediatric Emergency Department: Medications and Hospitalization Trends.

    Science.gov (United States)

    Cacciotti, Chantel; Vaiselbuh, Sarah; Romanos-Sirakis, Eleny

    2017-10-01

    The majority of emergency department (ED) visits and hospitalizations for patients with sickle cell disease (SCD) are pain related. Adequate and timely pain management may improve quality of life and prevent worsening morbidities. We conducted a retrospective chart review of pediatric patients with SCD seen in the ED, selected by sickle cell-related ICD-9 codes. A total of 176 encounters were reviewed from 47 patients to record ED pain management and hospitalization trends. Mean time to pain medication administration was 63 minutes. Patients received combination (nonsteroidal anti-inflammatory drug [NSAID] + narcotic) pain medications for initial treatment at a minority of ED encounters (19%). A higher percentage of patients who received narcotics alone as initial treatment were hospitalized as compared with those who received combination treatment initially ( P= 0.0085). Improved patient education regarding home pain management as well as standardized ED guidelines for assessment and treatment of sickle cell pain may result in superior and more consistent patient care.

  10. Preliminary Study of Neurodevelopmental Outcomes and Parenting Stress in Pediatric Mitochondrial Disease.

    Science.gov (United States)

    Eom, Soyong; Lee, Young-Mock

    2017-06-01

    Little is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, information that is crucial for improving the quality of life (QOL) for both patients and parents. We aimed to delineate neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the preliminary investigation of adequate intervention methods, better prognoses, and improved QOL for both patients and parents. Seventy children diagnosed with mitochondrial diseases were neuropsychologically evaluated. Neurocognitive (development, intelligence) and psychological (behavior, daily living function, maternal depression, parenting stress) functions were analyzed. Clinical variables, including the first symptom, epileptic classification, organ involvement, lactic acidosis, brain magnetic resonance imaging findings, muscle pathology, biochemical enzyme assay results, and syndromic diagnosis of mitochondrial diseases, were also reviewed. Prediagnostic assessments indicated that cognitive and psychomotor developments were significantly delayed. Group mean full scale intelligence quotient (IQ) scores indicated mild levels of intellectual disability, borderline levels of verbal IQ impairment, and mild levels of intellectual disability on performance IQ. Many children exhibited clinically significant levels of behavioral problems, whereas mothers of children with mitochondrial diseases exhibited significant increases in parenting stress relative to mothers of healthy children. Furthermore, 65% of mothers exhibited significant levels of depression. Early onset of the first symptoms, diffuse brain atrophy, and drug-resistant epilepsy negatively influenced neurodevelopmental and adaptive functions. Better understanding of the functional levels and profiles of neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the prediagnostic period is essential for adequate support and QOL of children with

  11. Central Nervous System Disease, Education, and Race Impact Radiation Refusal in Pediatric Cancer Patients.

    Science.gov (United States)

    Patel, Chirayu G; Stavas, Mark; Perkins, Stephanie; Shinohara, Eric T

    2017-07-01

    To investigate the determinants of radiation therapy refusal in pediatric cancer, we used the Surveillance, Epidemiology, and End Results registry to identify 24,421 patients who met the eligibility criteria, diagnosed between 1974 and 2012. Patients had any stage of cancer, were aged 0 to 19, and received radiation therapy or refused radiation therapy when it was recommended. One hundred twenty-eight patients (0.52%) refused radiation therapy when it was recommended. Thirty-two percent of patients who refused radiation therapy ultimately died from their cancer, at a median of 7 months after diagnosis (95% confidence interval, 3-11 mo), as compared with 29.0% of patients who did not refuse radiation therapy died from their cancer, at a median of 17 months after diagnosis (95% confidence interval, 17-18 mo). On multivariable analysis, central nervous system (CNS) site, education, and race were associated with radiation refusal. The odds ratio for radiation refusal for patients with CNS disease was 1.62 (P=0.009) as compared with patients without CNS disease. For patients living in a county with ≥10% residents having less than ninth grade education, the odds ratio for radiation refusal was 1.71 (P=0.008) as compared with patients living in a county with education. Asian, Pacific Islander, Alaska Native, and American Indian races had an odds ratio of 2.12 (P=0.002) for radiation refusal as compared with black or white race. Although the radiation refusal rate in the pediatric cancer population is low, we show that CNS site, education level, and race are associated with a significant difference in radiation refusal.

  12. Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law.

    Science.gov (United States)

    Rassi, Marcio S; Hulou, M Maher; Almefty, Kaith; Bi, Wenya Linda; Pravdenkova, Svetlana; Dunn, Ian F; Smith, Timothy R; Al-Mefty, Ossama

    2018-05-01

    Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins' law for embryonal tumors. Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

  13. A Canadian Study toward Changing Local Practice in the Diagnosis of Pediatric Celiac Disease

    Directory of Open Access Journals (Sweden)

    Seema Rajani

    2016-01-01

    Full Text Available Background. The European Society for Pediatric Gastroenterology, Hepatology and Nutrition endorses serological diagnosis (SD for pediatric celiac disease (CD. The objective of this study was to pilot SD and to prospectively evaluate gastrointestinal permeability and mucosal inflammation at diagnosis and after one year on the gluten-free diet (GFD. We hypothesized that SD would be associated with similar short term outcomes as ED. Method. Children, 3–17 years of age, referred for possible CD were eligible for SD given aTTG level ≥200 U/mL, confirmed by repeat aTTG and HLA haplotypes. Gastrointestinal permeability, assessed using sugar probes, and inflammation, assessed using fecal calprotectin (FC, at baseline and after one year on a GFD were compared to patients who had ED. Results. Enrolled SD (n=40 and ED (n=48 patients had similar demographics. ED and SD groups were not different in baseline lactulose: mannitol ratio (L : M (0.049 versus 0.034; p=0.07, fractional excretion of sucrose (%FES; 0.086 versus 0.092; p=0.44, or fecal calprotectin (FC; 89.6 versus 51.4; p=0.05. At follow-up, urine permeability improved and was similar between groups, L : M (0.022 versus 0.025; p=0.55 and %FES (0.040 versus 0.047; p=0.87 (p>0.05. FC improved but remained higher in the SD group (37.1 versus 15.9; p=0.04. Conclusion. Patients on the GFD showed improved intestinal permeability and mucosal inflammation regardless of diagnostic strategy. This prospective study supports that children diagnosed by SD have resolving mucosal disease early after commencing a GFD.

  14. Operative neurosurgery. Personal view and historical backgrounds. (9) Moyamoya angiopathy (MMA). Past history and status presens

    International Nuclear Information System (INIS)

    Yonekawa, Yasuhiro

    2012-01-01

    The second international meeting on the Moyamoya angiopathy (MMA) was held in mid. July 2011 at the children's hospital Zurich by Frau PD Dr.Khan and Prof.Meuli. On this occasion I was asked to give a survey of this disease, so the following points were presented and discussed: Who was the person who discovered this disease. How has the Research Committee of the Ministry of Health and Welfare, Japan (RCMHWJ) contributed to clarify the epidemiology, etiology, pathophysiology and treatments. What is the current situation in foreign countries especially Euroamerican to date on these topics. How the treatment technique developed and who did the initial revascularization procedures for the first. Update of the disease. Established view; discovery of the disease by Tekeuchi and Shimizu in 1955 could have been called somewhat into question as they described neither abnormal vasculature nor transdural anastomosis. Kudo who described ''spontaneous occlusion of the circle of Willis'' more precisely, but seemed to have thought that the occlusion site of the internal carotid artery (ICA) is around the origin of the ophthalmic artery. Suzuki and Takaku who coined the name Moyamoya disease (MMD) in 1969 and described 6 stages of progression on the basis of observation on 20 cases. The RCMHWJ founded in 1977 has contributed to clarifying the epidemiology, pathophysiology, treatment and etiology by interdisciplinary cooperative study having some epoch making events especially; by setting the guide lines -diagnostic criteria of the disease at the end of 1970, applying MRI and MR angiography (MRA) at the beginning of 1990 for the diagnosis instead of angiography used until then. By finding and focusing, therefore, on the cases of asymptomatic or oligosymptomatic presentation around the middle of 2000, which have almost doubled or tripled in incidence and/or prevalence and also changed the age distribution with the higher peak for adult cases. Achievements of research for the

  15. Evaluating the association of allergies with multiple sclerosis susceptibility risk and disease activity in a pediatric population.

    Science.gov (United States)

    Bourne, Theresa; Waltz, Michael; Casper, T C; Kavak, K; Aaen, G; Belman, A; Benson, L; Candee, M; Chitnis, T; Graves, J; Greenberg, B; Gorman, M; Harris, Y; Krupp, L; Lotze, T; Mar, S; Ness, J; Olsen, C; Roalstad, S; Rodriguez, M; Rose, J; Rubin, J; Schreiner, T; Tillema, J M; Kahn, I; Waldman, A; Barcellos, L; Waubant, E; Weinstock-Guttman, B

    2017-04-15

    Multiple sclerosis (MS) and allergies are both considered to be related to imbalanced Th1 and Th2 immune responses. Previous studies evaluating the relationship between MS and allergies provide conflicting results. To assess allergies and asthma as risk factors for MS and as predictors of MS relapses in a pediatric cohort. The environment and genetic risk factors for pediatric MS study is a national case-control project with 16 participating US sites. An environmental questionnaire is used that includes history of allergies in the first five years of life. Case-control data are entered in the pediatric MS Network database and cases at 12 of the 16 sites enter relapse data prospectively. Annualized relapse rate was calculated for patients with follow-up and adjusted for age at disease onset, gender, race, ethnicity, and use of disease-modifying therapy (DMT). We included 271 cases (mean age at disease onset of 15.7years and 62% female) and 418 controls. Relapse data were available for 193 cases. There was no difference in prevalence of allergies or asthma between cases and controls. Patients with food allergies had fewer relapses compared to patients without food allergies (0.14 vs 0.48, p=0.01). While allergies and asthma are not associated with pediatric MS, cases with food allergies have fewer relapses compared to those without food allergies. Copyright © 2017 Elsevier B.V. All rights reserved.

  16. Immunotherapeutics in Pediatric Autoimmune Central Nervous System Disease: Agents and Mechanisms.

    Science.gov (United States)

    Nosadini, Margherita; Sartori, Stefano; Sharma, Suvasini; Dale, Russell C

    2017-08-01

    Beyond the major advances produced by careful clinical-radiological phenotyping and biomarker development in autoimmune central nervous system disorders, a comprehensive knowledge of the range of available immune therapies and a deeper understanding of their action should benefit therapeutic decision-making. This review discusses the agents used in neuroimmunology and their mechanisms of action. First-line treatments typically include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while for severe disease second-line "induction" agents such as rituximab or cyclophosphamide are used. Steroid-sparing agents such as mycophenolate, azathioprine, or methotrexate are often used in potentially relapsing or corticosteroid-dependent diseases. Lessons from adult neuroimmunology and rheumatology could be translated into pediatric autoimmune central nervous system disease in the future, including the potential utility of monoclonal antibodies targeting lymphocytes, adhesion molecules for lymphocytic migration, cytokines or their receptors, or complement. Finally, many agents used in other fields have multiple mechanisms of action, including immunomodulation, with potential usefulness in neuroimmunology, such as antibiotics, psychotropic drugs, probiotics, gut health, and ketogenic diet. All currently accepted and future potential agents have adverse effects, which can be severe; therefore, a "risk-versus-benefit" determination should guide therapeutic decision-making. Copyright © 2017 Elsevier Inc. All rights reserved.

  17. Meta-analysis of shared genetic architecture across ten pediatric autoimmune diseases

    Science.gov (United States)

    Li, Yun R; Li, Jin; Zhao, Sihai D; Bradfield, Jonathan P; Mentch, Frank D; Maggadottir, S Melkorka; Hou, Cuiping; Abrams, Debra J; Chang, Diana; Gao, Feng; Guo, Yiran; Wei, Zhi; Connolly, John J; Cardinale, Christopher J; Bakay, Marina; Glessner, Joseph T; Li, Dong; Kao, Charlly; Thomas, Kelly A; Qiu, Haijun; Chiavacci, Rosetta M; Kim, Cecilia E; Wang, Fengxiang; Snyder, James; Richie, Marylyn D; Flatø, Berit; Førre, Øystein; Denson, Lee A; Thompson, Susan D; Becker, Mara L; Guthery, Stephen L; Latiano, Anna; Perez, Elena; Resnick, Elena; Russell, Richard K; Wilson, David C; Silverberg, Mark S; Annese, Vito; Lie, Benedicte A; Punaro, Marilynn; Dubinsky, Marla C; Monos, Dimitri S; Strisciuglio, Caterina; Staiano, Annamaria; Miele, Erasmo; Kugathasan, Subra; Ellis, Justine A; Munro, Jane E; Sullivan, Kathleen E; Wise, Carol A; Chapel, Helen; Cunningham-Rundles, Charlotte; Grant, Struan F A; Orange, Jordan S; Sleiman, Patrick M A; Behrens, Edward M; Griffiths, Anne M; Satsangi, Jack; Finkel, Terri H; Keinan, Alon; Prak, Eline T Luning; Polychronakos, Constantin; Baldassano, Robert N; Li, Hongzhe; Keating, Brendan J; Hakonarson, Hakon

    2016-01-01

    Genome-wide association studies (GWASs) have identified hundreds of susceptibility genes, including shared associations across clinically distinct autoimmune diseases. We performed an inverse χ2 meta-analysis across ten pediatric-age-of-onset autoimmune diseases (pAIDs) in a case-control study including more than 6,035 cases and 10,718 shared population-based controls. We identified 27 genome-wide significant loci associated with one or more pAIDs, mapping to in silico–replicated autoimmune-associated genes (including IL2RA) and new candidate loci with established immunoregulatory functions such as ADGRL2, TENM3, ANKRD30A, ADCY7 and CD40LG. The pAID-associated single-nucleotide polymorphisms (SNPs) were functionally enriched for deoxyribonuclease (DNase)-hypersensitivity sites, expression quantitative trait loci (eQTLs), microRNA (miRNA)-binding sites and coding variants. We also identified biologically correlated, pAID-associated candidate gene sets on the basis of immune cell expression profiling and found evidence of genetic sharing. Network and protein-interaction analyses demonstrated converging roles for the signaling pathways of type 1, 2 and 17 helper T cells (TH1, TH2 and TH17), JAK-STAT, interferon and interleukin in multiple autoimmune diseases. PMID:26301688

  18. Reversible Restricted Diffusion in the Corpus Callosum in Various Pediatric Diseases

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Won Kyung; Hong, Hyun Sook; Lee, A Leum; Cha, Jang Gyu; Lee, Hae Kyung [Dept. of Radiology, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon (Korea, Republic of); Bae, Won Kyung [Dept. of Radiology, Soonchunhyang University Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan (Korea, Republic of)

    2012-04-15

    To evaluate the reversible restricted diffusion in the corpus callosum in pediatric patients with clinical findings, and to discuss the possible pathogenesis of these lesions. Between 2007 and 2011, seven children with reversible signal abnormalities in the corpus callosum were identified and retrospectively reviewed. Diseases and conditions associated with lesions included: trauma (n = 3), neonatal seizure (n = 1), clinically suspected mild encephalopathy (n = 1), multiple sclerosis (n = 1), and seizure with subdural hygroma (n = 1). The callosal lesions were located in the splenium and the genu (n = 2), the splenium and the body (n = 1), and the splenium only (n 4). The shape of the lesions was round-to-ovoid (n = 4) or linear (n = 3). Follow-up MRI scans showed completely resolved (n = 6) or persistent (n = 1) signal abnormalities on diffusion-weighted imaging as well as apparent diffusion coefficient mapping. Clinical outcomes were good in six of the patents but poor in the seventh. Reversible restricted diffusion in the corpus callosum can develop in various diseases. Knowledge of the MRI findings and associated diseases might be helpful in predicting patients' conditions and clinical outcomes.

  19. Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

    Science.gov (United States)

    Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

    2015-03-01

    Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

  20. Spectrum of heart diseases in children: an echocardiographic study of 1,666 subjects in a pediatric hospital, Yaounde, Cameroon.

    Science.gov (United States)

    Chelo, David; Nguefack, Félicitée; Menanga, Alain P; Ngo Um, Suzanne; Gody, Jean C; Tatah, Sandra A; Koki Ndombo, Paul O

    2016-02-01

    Children's health programs in Sub-Saharan Africa have always been oriented primarily to infectious diseases and malnutrition. We are witnessing in the early 21(st) century an epidemiological transition marked by the decline of old diseases and the identification of new diseases including heart disease. Therefore, it is necessary to describe the spectrum of these diseases in order to better prepare health workers to these new challenges. We conducted a cross-sectional study focused on heart disease diagnosed by echocardiography in children seen from January 2006 to December 2014 in a pediatric hospital of Yaounde. We collected socio-demographic data and the types of heart disease from registers, patients files as well as the electronic database of echocardiographic records. A total of 2,235 patients underwent echocardiographic examination during the study period including 1,666 subjects with heart disease. Congenital cardiopathies were found in 1,230 (73.8%) patients and acquired abnormalities in 429 (25.8%). Seven children (0.4%) had a combination of both types. Congenital heart defects (CHD) were dominated by ventricular septal defect (VSD). Acquired heart disease was mostly rheumatic valvulopathies. Dyspnea on exertion was the most frequent presenting complaint (87.6%). Discovery of a heart murmur was the principal clinical finding on physical examination (81.4%). The median age was 9 months for congenital heart disease and 132 months for acquired heart disease. As infectious diseases recede and the diagnostic facilities are improving, pediatric heart diseases occupy a more important position in the spectrum of pediatric diseases in our context. However, the ability to evoke the diagnosis remains unsatisfactory by the majority of health personnel and therefore needs to be improved. Apart from congenital heart diseases, the impact of acquired heart diseases, rheumatic valvulopathy being the highest ranking, is remarkable in pediatrics. Awareness of health

  1. Short-term effects of ambient air pollution on pediatric outpatient visits for respiratory diseases in Yichang city, China.

    Science.gov (United States)

    Liu, Yuewei; Xie, Shuguang; Yu, Qing; Huo, Xixiang; Ming, Xiaoyan; Wang, Jing; Zhou, Yun; Peng, Zhe; Zhang, Hai; Cui, Xiuqing; Xiang, Hua; Huang, Xiji; Zhou, Ting; Chen, Weihong; Shi, Tingming

    2017-08-01

    Previous studies have suggested that short-term exposure to ambient air pollution was associated with pediatric hospital admissions and emergency room visits for certain respiratory diseases; however, there is limited evidence on the association between short-term air pollution exposure and pediatric outpatient visits. Our aim was to quantitatively assess the short-term effects of ambient air pollution on pediatric outpatient visits for respiratory diseases. We conducted a time-series study in Yichang city, China between Jan 1, 2014 and Dec 31, 2015. Daily counts of pediatric respiratory outpatient visits were collected from 3 large hospitals, and then linked with air pollution data from 5 air quality monitoring stations by date. We used generalized additive Poisson models to conduct linear and nonlinear exposure-response analyses between air pollutant exposures and pediatric respiratory outpatient visits, adjusting for seasonality, day of week, public holiday, temperature, and relative humidity. Each interquartile range (IQR) increase in PM 2.5 (lag 0), PM 10 (lag 0), NO 2 (lag 0), CO (lag 0), and O 3 (lag 4) concentrations was significantly associated with a 1.91% (95% CI: 0.60%, 3.23%), 2.46% (1.09%, 3.85%), 1.88% (0.49%, 3.29%), 2.00% (0.43%, 3.59%), and 1.91% (0.45%, 3.39%) increase of pediatric respiratory outpatient visits, respectively. Similarly, the nonlinear exposure-response analyses showed monotonic increases of pediatric respiratory outpatient visits by increasing air pollutant exposures, though the associations for NO 2 and CO attenuated at higher concentrations. These associations were unlikely modified by season. We did not observe significant association for SO 2 exposure. Our results suggest that short-term exposures to PM 2.5 , PM 10 , NO 2 , CO, and O 3 may account for increased risk of pediatric outpatient visits for respiratory diseases, and emphasize the needs for reduction of air pollutant exposures for children. Copyright © 2017

  2. Lyme disease in children in southeastern Connecticut. Pediatric Lyme Disease Study Group.

    Science.gov (United States)

    Gerber, M A; Shapiro, E D; Burke, G S; Parcells, V J; Bell, G L

    1996-10-24

    Although the incidence of Lyme disease is highest in children, there are few prospective data on the clinical manifestations and outcomes in children. We conducted a prospective, longitudinal, community-based cohort study of children with newly diagnosed Lyme disease in an area of Connecticut in which the disease is highly endemic. We obtained clinical and demographic information and performed serial antibody tests and follow-up evaluations. Over a period of 20 months, 201 consecutive patients were enrolled; their median age was 7 years (range, 1 to 21). The initial clinical manifestations of Lyme disease were a single erythema migrans lesion in 66 percent, multiple erythema migrans lesions in 23 percent, arthritis in 6 percent, facial-nerve palsy in 3 percent, aseptic meningitis in 2 percent, and carditis in 0.5 percent. At presentation, 37 percent of the patients with a single erythema migrans lesion and 89 percent of those with multiple erythema migrans lesions had antibodies against Borrelia burgdorferi. All but 3 of the 201 patients were treated for two to four weeks with conventional antimicrobial therapy, which was administered orally in 96 percent. All had prompt clinical responses. After four weeks, 94 percent were completely asymptomatic (including the two patients whose parents had refused to allow antimicrobial treatment). At follow-up a mean of 25.4 months later, none of the patients had evidence of either chronic or recurrent Lyme disease. Six patients subsequently had a new episode of erythema migrans. About 90 percent of children with Lyme disease present with erythema migrans, which is an early stage of the disease. The prognosis is excellent for those with early Lyme disease who are treated promptly with conventional courses of antimicrobial agents.

  3. Intestinal lesions in pediatric Crohn disease: comparative detectability among pulse sequences at MR enterography

    International Nuclear Information System (INIS)

    Sohn, Beomseok; Kim, Myung-Joon; Lee, Mi-Jung; Koh, Hong; Han, Kyung Hwa

    2014-01-01

    Variable sequences can be used in MR enterography, and no consensus exists for the best protocol in children with Crohn disease. To compare the lesion detectability of various MR enterography sequences and to correlate the findings of these sequences with the Pediatric Crohn's Disease Activity Index (PCDAI) in children with Crohn disease. Children with clinically or pathologically confirmed Crohn disease underwent MR enterography, including a single-shot fast spin-echo (SSFSE) sequence, motility imaging (coronal 2-D balanced fast field echo), diffusion-weighted imaging (DWI), and dynamic contrast enhancement imaging (including arterial, portal and delayed phases). The lesion detectability of each sequence was graded 0-2 for each involved bowel segment. The lesion detectability and PCDAI result on different sequences were compared using the weighted least squares method and Student's t-test, respectively. Fifteen children (11 boys, 4 girls, mean age 13.7 ± 1.4 years) with a total of 41 lesions were included in this study. All lesions detected in more than two sequences were visible on the single-shot fast spin-echo (SSFSE) sequence. The relative lesion detection rate was 78.1% on motility imaging, 90.2% on DWI, and 92.7% on arterial, 95.1% on portal and 95.1% on delayed phase imaging. Compared to the SSFSE sequence, motility imaging (P < 0.001) and DWI (P = 0.039) demonstrated lower detectability. The mean PCDAI result in the detected lesions was statistically higher only on dynamic enhancement imaging (P < 0.001). All MR enterography sequences were found to have relatively high lesion detectability in children with Crohn disease, while motility imaging showed the lowest lesion detectability. Lesions detected on dynamic enhancement imaging showed a higher PCDAI result, which suggests that this sequence is specific for active inflammation. (orig.)

  4. Exhaled volatile substances mirror clinical conditions in pediatric chronic kidney disease.

    Directory of Open Access Journals (Sweden)

    Juliane Obermeier

    Full Text Available Monitoring metabolic adaptation to chronic kidney disease (CKD early in the time course of the disease is challenging. As a non-invasive technique, analysis of exhaled breath profiles is especially attractive in children. Up to now, no reports on breath profiles in this patient cohort are available. 116 pediatric subjects suffering from mild-to-moderate CKD (n = 48 or having a functional renal transplant KTx (n = 8 and healthy controls (n = 60 matched for age and sex were investigated. Non-invasive quantitative analysis of exhaled breath profiles by means of a highly sensitive online mass spectrometric technique (PTR-ToF was used. CKD stage, the underlying renal disease (HUS; glomerular diseases; abnormalities of kidney and urinary tract or polycystic kidney disease and the presence of a functional renal transplant were considered as classifiers. Exhaled volatile organic compound (VOC patterns differed between CKD/ KTx patients and healthy children. Amounts of ammonia, ethanol, isoprene, pentanal and heptanal were higher in patients compared to healthy controls (556, 146, 70.5, 9.3, and 5.4 ppbV vs. 284, 82.4, 49.6, 5.30, and 2.78 ppbV. Methylamine concentrations were lower in the patient group (6.5 vs 10.1 ppbV. These concentration differences were most pronounced in HUS and kidney transplanted patients. When patients were grouped with respect to degree of renal failure these differences could still be detected. Ammonia accumulated already in CKD stage 1, whereas alterations of isoprene (linked to cholesterol metabolism, pentanal and heptanal (linked to oxidative stress concentrations were detectable in the breath of patients with CKD stage 2 to 4. Only weak associations between serum creatinine and exhaled VOCs were noted. Non-invasive breath testing may help to understand basic mechanisms and metabolic adaptation accompanying progression of CKD. Our results support the current notion that metabolic adaptation occurs early during the time

  5. Disease severity, not operative approach, drives organ space infection after pediatric appendectomy.

    Science.gov (United States)

    Kelly, Kristin N; Fleming, Fergal J; Aquina, Christopher T; Probst, Christian P; Noyes, Katia; Pegoli, Walter; Monson, John R T

    2014-09-01

    This study examines patient and operative factors associated with organ space infection (OSI) in children after appendectomy, specifically focusing on the role of operative approach. Although controversy exists regarding the risk of increased postoperative intra-abdominal infections after laparoscopic appendectomy, this approach has been largely adopted in the treatment of pediatric acute appendicitis. Children aged 2 to 18 years undergoing open or laparoscopic appendectomy for acute appendicitis were selected from the 2012 American College of Surgeons Pediatric National Surgical Quality Improvement Program database. Univariate analysis compared patient and operative characteristics with 30-day OSI and incisional complication rates. Factors with a P value of less than 0.1 and clinical importance were included in the multivariable logistic regression models. A P value less than 0.05 was considered significant. For 5097 children undergoing appendectomy, 4514 surgical procedures (88.6%) were performed laparoscopically. OSI occurred in 155 children (3%), with half of these infections developing postdischarge. Significant predictors for OSI included complicated appendicitis, preoperative sepsis, wound class III/IV, and longer operative time. Although 5.2% of patients undergoing open surgery developed OSI (odds ratio = 1.82; 95% confidence interval, 1.21-2.76; P = 0.004), operative approach was not associated with increased relative odds of OSI (odds ratio = 0.99; confidence interval, 0.64-1.55; P = 0.970) after adjustment for other risk factors. Overall, the model had excellent predictive ability (c-statistic = 0.837). This model suggests that disease severity, not operative approach, as previously suggested, drives OSI development in children. Although 88% of appendectomies in this population were performed laparoscopically, these findings support utilization of the surgeon's preferred surgical technique and may help guide postoperative counsel in high-risk children.

  6. Cardiac Catheterization and Intervention in Pediatric Cardiac Disease: A Narrative Review of Current Indications, Techniques, and Complications

    Directory of Open Access Journals (Sweden)

    Bahram Pishgoo

    2017-05-01

    Full Text Available Context In the past 20 to 30 years, the area of pediatric interventional cardiology has had noteworthy development. Technological revolutions have significantly progressed management of cardiovascular disease in both children and adults with congenital heart disease (CHD. This article reviews the current indications, techniques and complications of interventional therapy for CHD. Evidence Acquisition Training and publications in this field are rare. Overall, 64 article from January 1953 to February 2014 were studied. A total of 26 articles were involved in pediatric evaluation. Results There have been several catheter-based interventions for congenital heart disease. Percutaneous intervention in pediatric cardiac disease has been established in the past 2 to 3 decades. There are currently devices accepted for percutaneous closure of ASDs, patent ductus arteriosus (PDAs, and muscular ventricular septal defects (VSDs. The period of percutaneous valve implantation is just beginning, and the next few years may bring about advances in miniaturized valve distribution methods to allow insertion in smaller children. Conclusions Completely prepared catheterization laboratory, surgical holdup, and extracorporeal membrane oxygenation support capabilities must be accessible at any center to achieve interventional cardiac catheterization. Additional understanding of normal history of interventions more than 2 decade post process, novel strategies and methods will certainly lead to an increase in the methods for managing of congenital heart disease.

  7. Hydroxyurea in Pediatric Patients With Sickle Cell Disease: What Nurses Need to Know.

    Science.gov (United States)

    Rees, Allison L

    2016-09-01

    Sickle cell disease (SCD) is an inherited disorder in which sickled red blood cells occlude the small vessels of the body, reducing oxygen delivery to tissues and ultimately negatively affecting many of the body's major organs. Hydroxyurea has proven beneficial in the treatment of SCD and prevention of disease-related complications. The 2014 guidelines put forth by the National Heart, Lung, and Blood Institute recommend hydroxyurea treatment in infants 9 months and older, children, and adolescents with SCD-SS or SCD-Sβ(0) thalassemia regardless of clinical severity. This is a change from the 2002 guidelines in which hydroxyurea was recommended for adolescents and children with SCD-SS or SCD-Sβ(0) thalassemia with frequent episodes of pain, a history of acute chest syndrome, severe and symptomatic anemia or other severe vaso-occlusive events. Nurses play a critical role in working with patients and families to provide education, guidance, and support to improve compliance to mitigate the long-term effects of SCD. © 2015 by Association of Pediatric Hematology/Oncology Nurses.

  8. High frequency analysis of cough sounds in pediatric patients with respiratory diseases.

    Science.gov (United States)

    Kosasih, K; Abeyratne, U R; Swarnkar, V

    2012-01-01

    Cough is a common symptom in a range of respiratory diseases and is considered a natural defense mechanism of the body. Despite its critical importance in the diagnosis of illness, there are no golden methods to objectively assess cough. In a typical consultation session, a physician may briefly listen to the cough sounds using a stethoscope placed against the chest. The physician may also listen to spontaneous cough sounds via naked ears, as they naturally propagate through air. Cough sounds carry vital information on the state of the respiratory system but the field of cough analysis in clinical medicine is in its infancy. All existing cough analysis approaches are severely handicapped by the limitations of the human hearing range and simplified analysis techniques. In this paper, we address these problems, and explore the use of frequencies covering a range well beyond the human perception (up to 90 kHz) and use wavelet analysis to extract diagnostically important information from coughs. Our data set comes from a pediatric respiratory ward in Indonesia, from subjects diagnosed with asthma, pneumonia and rhinopharyngitis. We analyzed over 90 cough samples from 4 patients and explored if high frequencies carried useful information in separating these disease groups. Multiple regression analysis resulted in coefficients of determination (R(2)) of 77-82% at high frequencies (15 kHz-90 kHz) indicating that they carry useful information. When the high frequencies were combined with frequencies below 15kHz, the R(2) performance increased to 85-90%.

  9. Screening for residual disease in pediatric burkitt lymphoma using consensus primer pools.

    Science.gov (United States)

    Agsalda, Melissa; Kusao, Ian; Troelstrup, David; Shiramizu, Bruce

    2009-01-01

    Assessing molecular persistent or minimal residual disease (PD/MRD) in childhood Burkitt lymphoma (BL) is challenging because access to original tumor is usually needed to design patient-specific primers (PSPs). Because BL is characterized by rearranged immunoglobulin heavy chain (IgV(H)) genes, IgV(H) primer pools from IgV(H1)-IgV(H7) regions were tested to detect PD/MRD, thus eliminating the need for original tumor. The focus of the current study was to assess the feasibility of using IgV(H) primer pools to detect disease in clinical specimens. Fourteen children diagnosed with B-NHL had follow-up repository specimens available to assess PD/MRD. Of the 14 patients, 12 were PD/MRD negative after 2 months of therapy and remained in remission at the end of therapy; 2/14 patients were PD/MRD positive at 2-3 months and later relapsed. PSP-based assays from these 14 patients showed 100% concordance with the current assay. This feasibility study warrants further investigation to assess PD/MRD using IgV(H) primer pools, which could have clinical significance as a real-time assessment tool to monitor pediatric BL and possibly other B-cell non-Hodgkin lymphoma therapy.

  10. Patterns of reflux in gastroesophageal reflux disease in pediatric population of New South Wales.

    Science.gov (United States)

    Narayanan, Sarath Kumar; Cohen, Ralph Clinton; Karpelowsky, Jonathan Saul

    2017-02-01

    This study is to determine the association of ambulatory pH monitoring (24hr pH) with symptoms of gastroesophageal reflux and its other investigations. The clinical and epidemiological profiles of subjects referred for reflux disorders are also studied. Symptoms or group of symptoms, profiles and prior investigations of 1259 consecutive pediatric subjects (with 1332 24hr pH studies performed) referred for evaluation of reflux disorders between 1988 and 2012 were retrospectively studied. Chi-square or fisher exact test was used for hypothesis testing, student t-test for the comparison of means and the Wilcoxon rank-sum test for comparing medians of continuous variables. Gastroesophageal reflux disease (GERD), defined as reflux causing major symptoms and complications, was diagnosed in 57.5% subjects of the total sample. Forty-three percent were girls and 56.7% were boys. The most common age group was between 4 months and 2 years (51.2%). Vomiting (64.4%) and irritability (74%) were the most common symptoms with the neurological conditions (23.2%) being the most frequent underlying condition. The parameters used in 24hr pH were significantly higher in those diagnosed with GERD (P reflux disorders. 24hr pH is reliable and should be considered routine in reflux disorders, as it identifies patients with pathologic reflux and avoids a needless surgery. © 2016 International Society for Diseases of the Esophagus.

  11. Incidence Trends and Geographical Variability of Pediatric Inflammatory Bowel Disease in Slovenia: A Nationwide Study.

    Science.gov (United States)

    Urlep, Darja; Blagus, Rok; Orel, Rok

    2015-01-01

    The aims of the study were to determine the incidence rate of pediatric inflammatory bowel disease (PIBD) and its trends for the period of 2002-2010 and to assess the geographical distribution of PIBD in Slovenia. Medical records of patients (0-18 years) with newly diagnosed IBD during the study period were retrospectively reviewed. The mean incidence rate for IBD in 2002-2010 was 7.6 per 100,000 children and adolescents per year, 4.5 for Crohn's disease (CD), 2.9 for ulcerative colitis (UC), and 0.2 for IBD-unclassified, respectively. The incidence rate increased from 5.8 per 100,000 per year in 2002-2004 to 8.6 in 2005-2007 and remained stable afterwards. Statistically significant difference in the incidence rate between the Northeastern and Southwestern parts of the country was observed (p = 0.025). This nationwide study demonstrates that Slovenia is among the European countries with the highest PIBD incidence. During the study period a substantial rise of PIBD incidence was observed during the first half of the study and it seems to have stabilized in the second half. The significant difference in PIBD incidence between Northeastern and Southwestern parts of the country merits further exploration of the possible environmental factors.

  12. Screening for Residual Disease in Pediatric Burkitt Lymphoma Using Consensus Primer Pools

    Directory of Open Access Journals (Sweden)

    Melissa Agsalda

    2009-01-01

    Full Text Available Assessing molecular persistent or minimal residual disease (PD/MRD in childhood Burkitt lymphoma (BL is challenging because access to original tumor is usually needed to design patient-specific primers (PSPs. Because BL is characterized by rearranged immunoglobulin heavy chain (IgVH genes, IgVH primer pools from IgVH1–IgVH7 regions were tested to detect PD/MRD, thus eliminating the need for original tumor. The focus of the current study was to assess the feasibility of using IgVH primer pools to detect disease in clinical specimens. Fourteen children diagnosed with B-NHL had follow-up repository specimens available to assess PD/MRD. Of the 14 patients, 12 were PD/MRD negative after 2 months of therapy and remained in remission at the end of therapy; 2/14 patients were PD/MRD positive at 2-3 months and later relapsed. PSP-based assays from these 14 patients showed 100% concordance with the current assay. This feasibility study warrants further investigation to assess PD/MRD using IgVH primer pools, which could have clinical significance as a real-time assessment tool to monitor pediatric BL and possibly other B-cell non-Hodgkin lymphoma therapy.

  13. Pediatric Crohn disease patients exhibit specific ileal transcriptome and microbiome signature.

    Science.gov (United States)

    Haberman, Yael; Tickle, Timothy L; Dexheimer, Phillip J; Kim, Mi-Ok; Tang, Dora; Karns, Rebekah; Baldassano, Robert N; Noe, Joshua D; Rosh, Joel; Markowitz, James; Heyman, Melvin B; Griffiths, Anne M; Crandall, Wallace V; Mack, David R; Baker, Susan S; Huttenhower, Curtis; Keljo, David J; Hyams, Jeffrey S; Kugathasan, Subra; Walters, Thomas D; Aronow, Bruce; Xavier, Ramnik J; Gevers, Dirk; Denson, Lee A

    2014-08-01

    Interactions between the host and gut microbial community likely contribute to Crohn disease (CD) pathogenesis; however, direct evidence for these interactions at the onset of disease is lacking. Here, we characterized the global pattern of ileal gene expression and the ileal microbial community in 359 treatment-naive pediatric patients with CD, patients with ulcerative colitis (UC), and control individuals. We identified core gene expression profiles and microbial communities in the affected CD ilea that are preserved in the unaffected ilea of patients with colon-only CD but not present in those with UC or control individuals; therefore, this signature is specific to CD and independent of clinical inflammation. An abnormal increase of antimicrobial dual oxidase (DUOX2) expression was detected in association with an expansion of Proteobacteria in both UC and CD, while expression of lipoprotein APOA1 gene was downregulated and associated with CD-specific alterations in Firmicutes. The increased DUOX2 and decreased APOA1 gene expression signature favored oxidative stress and Th1 polarization and was maximally altered in patients with more severe mucosal injury. A regression model that included APOA1 gene expression and microbial abundance more accurately predicted month 6 steroid-free remission than a model using clinical factors alone. These CD-specific host and microbe profiles identify the ileum as the primary inductive site for all forms of CD and may direct prognostic and therapeutic approaches.

  14. [Management of urinary tract infections in children. Recommendations of the Pediatric Infectious Diseases Group of the French Pediatrics Society and the French-Language Infectious Diseases Society].

    Science.gov (United States)

    Cohen, R; Raymond, J; Faye, A; Gillet, Y; Grimprel, E

    2015-06-01

    Urine dipsticks have to be used more frequently for the screening of urinary tract infections (UTI) in febrile infants and children (grade A). Confirmation of the UTI by urine culture should prefer other methods of sampling than the urine bag: sampling jet, urethral catheterization, or pubic puncture (grade A). The percentage of Escherichia coli producing extended-spectrum beta-lactamases (ESBL) in children accounts for less than 10 % in France and does not justify revising the 2007 recommendations (grade B). An increase in the use of carbapenems in first-line treatment is a major environmental hazard and exposes the patient to the risk of untreatable infections. For febrile UTI, the expert group recommended: (1) recover the results of susceptibility testing as soon as possible to quickly adapt treatment for possible resistant strains; (2) favor initial treatment with aminoglycosides (particularly amikacin) which remain active in the majority of ESBL strains for patients seen in the pediatric emergency department and/or hospital; (3) ceftriaxone (IV or IM) remains an appropriate treatment for patients seen in the emergency department or outpatient clinic because the percentage of ESBL-producing enterobacteria strains remains low; (4) use oral cefixime (grade B) in nonsevere cases and low-risk patients defined as age>3 months, general condition preserved, disease duration of feverurinary tract infection, uropathy, or prior antibiotic therapy in the last 3 months; (5) oral relay for parenteral treatment is guided by in vitro susceptibility testing, in an attempt to reduce the use of oral cephalosporins to limit the selection of resistant bacterial strains. The total duration of treatment recommended is usually 10 days. Except for special circumstances, there is no need to prescribe retrograde cystography or antibiotic prophylaxis after a first febrile urinary tract infection. For cystitis, the panel recommends systematic urinalysis and initial prescription before the

  15. Pediatric Interstitial Lung Disease Masquerading as Difficult Asthma: Management Dilemmas for Rare Lung Disease in Children

    Directory of Open Access Journals (Sweden)

    EY Chan

    2005-01-01

    Full Text Available Idiopathic nontransplant-related childhood bronchiolitis obliterans is an uncommon disease. Most patients present with chronic recurrent dyspnea, cough and wheezing, which are also features of asthma, by far a much more common condition. The present case study reports on a six-year-old girl who presented to a tertiary care centre with recurrent episodes of respiratory distress on a background of baseline tachypnea, chronic hypoxemia and exertional dyspnea. Her past medical history revealed significant lung disease in infancy, including respiratory syncytial virus bronchiolitis and repaired gastroesophageal reflux. She was treated for 'asthma exacerbations' throughout her early childhood years. Bronchiolitis obliterans was subsequently diagnosed with an open lung biopsy. She did not have sustained improvement with systemic corticosteroids, hydroxychloroquine or clarithromycin. Cardiac catheterization confirmed the presence of secondary pulmonary hypertension. Treatment options remain a dilemma for this patient because there is no known effective treatment for this condition, and the natural history is not well understood. The present case demonstrates the need for careful workup in 'atypical asthma', and the urgent need for further research into the rare lung diseases of childhood.

  16. Clinical features and prevalence of gastroesophageal reflux disease in infants attending a pediatric gastroenterology reference service.

    Science.gov (United States)

    Koda, Yu Kar Ling; Ozaki, Marcos J; Murasca, Kelly; Vidolin, Eliana

    2010-01-01

    In infants, it is not always easy to distinguish between pathological and physiological gastroesophageal reflux based only on clinical criteria. In Brazil, studies about gastroesophageal reflux disease in infants are few and are even rare those that used prolonged esophageal pH monitoring for its evaluation. To describe the clinical features of gastroesophageal reflux disease and to determine its prevalence in infants with gastroesophageal reflux attending a tertiary Pediatric Gastroenterology Service and submitted to esophageal pH monitoring for investigation. Descriptive study in 307 infants in whom esophageal pH monitoring (Mark III Digitrapper, Synectics Medical AB, Sweden) was performed during the period December, 1998-December, 2008. The clinical features studied were age group (1-12 months and 13-24 months), and clinical manifestations that motivated the indication of pH monitoring. One hundred twenty-four (40.4%) were female and 183 (59.6%) male with mean age 12.2 +/- 6.2 months (1-23 months). The prevalence of gastroesophageal reflux disease was 18.2% (56/307). One hundred forty-eight (48.2%) were 1-12 months old and 159 (51.8%), 13-24 months. No significant difference was found between the prevalence of these two age groups (P = 0.3006). Gastroesophageal reflux disease was more frequent in those with digestive manifestations (24.2%), crisis of cyanosis/apnea (23.8%) and mixed manifestations (21.5%). Respiratory manifestations were the most frequent indication (39.1%) of pH monitoring. However, the prevalence of gastroesophageal reflux disease was lower (12.5%) in this group compared with in those with digestive manifestations (P = 0.0574), crisis of cyanosis/apnea (P = 0.0882) and mixed manifestations (P = 0.1377). All infants that presented clinical manifestations as crisis of cyanosis/apnea and abnormal pH-metry were Service, the prevalence of gastroesophageal reflux disease associated with acid reflux in infants revealed elevated. Infants with crisis

  17. Heat shock protein-derived T-cell epitopes contribute to autoimmune inflammation in pediatric Crohn's disease.

    Directory of Open Access Journals (Sweden)

    Gisella L Puga Yung

    Full Text Available Pediatric Crohn's disease is a chronic auto inflammatory bowel disorder affecting children under the age of 17 years. A putative etiopathogenesis of Crohn's disease (CD is associated with disregulation of immune response to antigens commonly present in the gut microenvironment. Heat shock proteins (HSP have been identified as ubiquitous antigens with the ability to modulate inflammatory responses associated with several autoimmune diseases. The present study tested the contribution of immune responses to HSP in the amplification of autoimmune inflammation in chronically inflamed mucosa of pediatric CD patients. Colonic biopsies obtained from normal and CD mucosa were stimulated with pairs of Pan HLA-DR binder HSP60-derived peptides (human/bacterial homologues. The modulation of RNA and protein levels of induced proinflammatory cytokines were measured. We identified two epitopes capable of sustaining proinflammatory responses, specifically TNF< and IFN induction, in the inflamed intestinal mucosa in CD patients. The responses correlated positively with clinical and histological measurements of disease activity, thus suggesting a contribution of immune responses to HSP in pediatric CD site-specific mucosal inflammation.

  18. Recurrence rate of clostridium difficile infection in hospitalized pediatric patients with inflammatory bowel disease.

    Science.gov (United States)

    Kelsen, Judith R; Kim, Jason; Latta, Dan; Smathers, Sarah; McGowan, Karin L; Zaoutis, Theodore; Mamula, Petar; Baldassano, Robert N

    2011-01-01

    The incidence and associated morbidity of Clostridium difficile (CD) infection has been increasing at an alarming rate in North America. Clostridium difficile-associated diarrhea (CDAD) is the leading cause of nosocomial diarrhea in the USA. Patients with CDAD have longer average hospital admissions and additional hospital costs. Evidence has demonstrated that patients with inflammatory bowel disease (IBD) have a higher incidence of CD in comparison to the general population. The aim of this study was to compare the rate of recurrence of CD in hospitalized pediatric patients with IBD compared to hospitalized controls. The secondary aim was to evaluate whether infection with CD resulted in a more severe disease course of IBD. This was a nested case control retrospective study of hospitalized pediatric patients. Diagnosis of CD was confirmed with stool Toxin A and B analysis. The following data were obtained from the medical records: demographic information, classification of IBD including location of disease, IBD therapy, and prior surgeries. In addition, prior hospital admissions within 1 year and antibiotic exposure were recorded. The same information was recorded following CD infection. Cases were patients with IBD and CD; two control populations were also studied: patients with CD but without IBD, and patients with IBD but without CD. For aim 1, a total of 111 eligible patients with IBD and CD infection and 77 eligible control patients with CD infection were included. The rate of recurrence of CD in the IBD population was 34% compared to 7.5% in the control population (P < 0.0001). In evaluating the effect of CD infection on IBD disease severity, we compared the 111 IBD patients with CD to a second control population of 127 IBD patients without CD. 57% of IBD-CD patients were readmitted with an exacerbation of disease within 6 months of infection with CD and 67% required escalation of therapy following CD infection, compared to 30% of IBD patients without CD (P

  19. Original Research: Use of hydroxyurea and phlebotomy in pediatric patients with hemoglobin SC disease.

    Science.gov (United States)

    Summarell, Carly C Ginter; Sheehan, Vivien A

    2016-04-01

    Hydroxyurea is an excellent therapeutic agent for the pharmacological induction of HbF in patients with sickle cell disease (SCD). However, all completed clinical trials of hydroxyurea have excluded patients with hemoglobin SC (HbSC) disease. HbSC differs significantly in pathophysiology from HbSS, as HbC does not sickle, but instead causes cellular dehydration which potentiates sickling of HbS. Many severely affected HbSC patients have been placed on hydroxyurea on a case by case basis, but there are no large scale prospective data on safety or efficacy of hydroxyurea in this subset of patients with SCD. Here, we report a case series of 14 pediatric patients with HbSC treated to maximum tolerated dose (MTD) with hydroxyurea. Those who failed to show clinical improvement after at least six months at MTD were offered phlebotomy in addition to hydroxyurea. Five out of 11 patients with HbSC who achieved MTD failed to demonstrate clinical improvement on hydroxyurea. Of the four placed on dual hydroxyurea and phlebotomy therapy, all showed at least partial clinical improvement. Percent dense red blood cells (%DRBC) were measured via an ADVIA hematology analyzer. A marked rise in percent dense cells preceded clinical complications in three patients. Dual therapy with hydroxyurea and phlebotomy may be an effective approach to patients with HbSC that do not experience improvement with hydroxyurea alone. Monitoring of %DRBC may predict adverse events and aid in assessing hydroxyurea compliance. Large scale clinical trials are needed to evaluate the safety and efficacy of hydroxyurea and hydroxyurea with phlebotomy in patients with HbSC disease. © 2016 by the Society for Experimental Biology and Medicine.

  20. Neuropsychological assessment, neuroimaging, and neuropsychiatric evaluation in pediatric and adult patients with sickle cell disease (SCD

    Directory of Open Access Journals (Sweden)

    Christopher L Edwards

    2007-01-01

    Full Text Available Christopher L Edwards1, Renee Dunn Raynor1, Miriam Feliu1, Camela McDougald1, Stephanie Johnson2, Donald Schmechel3, Mary Wood1, Gary G Bennett4, Patrick Saurona5, Melanie Bonner1, Chante’ Wellington1, Laura M DeCastro6, Elaine Whitworth6, Mary Abrams6, Patrick Logue1, Lekisha Edwards1, Salutario Martinez7, Keith E Whitfield81Department of Psychiatry and Behavioral Sciences, Duke University Medical Center, Durham, NC, USA; 2American Psychological Association, Science Directorate, Washington, DC, USA; 3Department of Medicine, Division of Neurology, Duke University Medical Center, Durham, NC, USA; 4Department of Society, Human Development, and Health, Harvard School of Public Health, Boston, MA, USA; 5Taub Institute For Research on Alzheimer’s Disease and The Aging Brain, Columbia University, New York, NY, USA; 6Department of Medicine, Division of Hematology, Duke University Medical Center, Durham, NC, USA; 7Department of Radiology, Duke University Medical Center, Durham, NC, USA; 8Duke University, Durham, NC, USAAbstract: Traditionally, neuropsychological deficits due to Sickle Cell Disease (SCD have been understudied in adults. We have begun to suspect, however, that symptomatic and asymptomatic Cerebrovascular Events (CVE may account for an alarming number of deficits in this population. In the current brief review, we critically evaluated the pediatric and adult literatures on the neurocognitive effects of SCD. We highlighted the studies that have been published on this topic and posit that early detection of CVE via neurocognitive testing, neuropsychiatric evaluations, and neuroimaging may significantly reduce adult cognitive and functional morbidities.Keywords: cerebral vascular event, neuropsychological assessment, sickle cell disease, neuroimaging

  1. Radiodiagnosis in pediatrics today

    International Nuclear Information System (INIS)

    Baklanova, V.F.

    1982-01-01

    The fields of radiodiagnosis application in pediatrics are considered. The improvement of roentgenologic methods and application of various contrast proparations enable to study and precisely differentiate congenital and acquired diseases. The scope of roentgenology application in pediatrics extends due to differentiation of pediatric specialities. New methods of investigation with decreasing radiation exposure to minimal are realized [ru

  2. Doença de Moyamoya e anestesia com sevoflurano fora do centro cirúrgico: relato de caso

    OpenAIRE

    Machado, Sheila Braga; Mendes, Florentino Fernandes; Angelini, Adriana de Campos

    2002-01-01

    JUSTIFICATIVA E OBJETIVOS: A doença de Moyamoya é uma desordem cerebrovascular progressiva que representa um desafio anestésico em virtude da precária circulação cerebral destes pacientes, constituindo-se numa importante causa de acidente vascular cerebral em indivíduos jovens. O objetivo deste relato é apresentar o caso de um paciente com doença de Moyamoya que foi submetido à anestesia geral com sevoflurano para procedimento diagnóstico fora do centro cirúrgico. RELATO DO CASO: Criança com ...

  3. Association of Parental Overweight and Cardiometabolic Diseases and Pediatric Adiposity and Lifestyle Factors with Cardiovascular Risk Factor Clustering in Adolescents

    Directory of Open Access Journals (Sweden)

    Chun-Ying Lee

    2016-09-01

    Full Text Available Cardiometabolic risk factors or their precursors are observed in childhood and may continue into adulthood. We investigated the effects of parental overweight and cardiometabolic diseases and pediatric lifestyle factors on the clustering of cardiovascular risk factors among adolescents, and examined the mediating and modifying effects of pediatric adiposity on these associations. Representative adolescents (n = 2727; age, 12–16 years were randomly recruited through multistage stratified sampling from 36 schools in Southern Taiwan. Adolescent and parent surveys were conducted in schools and participant homes, respectively. Their demographic factors, diet patterns, and physical, anthropometric, and clinical parameters were collected and analyzed. Adolescents with 1–2 and ≥3 risk components for pediatric metabolic syndrome (MetS were defined as potential MetS (pot-MetS and MetS, respectively. Adolescents whose parents were overweight/obese, or with diabetes and hypertension had a higher prevalence ratio of pot-MetS and MetS (1.5–1.6 and 1.9–4.2-fold, respectively. Low physical activity (<952.4 MET·min/week, long screen time (≥3 h/day and high sugar-sweetened beverage intake (>500 mL/day were associated with a 3.3- (95% confidence intervals (CI = 1.5–7.3, 2.2- (95% CI = 1.1–4.4, and 26.9-fold (95% CI = 3.2–229.0 odds ratio (OR of MetS, respectively. Pediatric body mass index (BMI accounted for 18.8%–95.6% and 16.9%–60.3% increased prevalence ratios of these parental and pediatric risk factors for MetS. The OR of pot-MetS + MetS for sugar-sweetened beverage consumption was multiplicatively enhanced among adolescents with overweight/obesity (combined OR, 8.6-fold (95% CI = 4.3–17.3; p for multiplicative interaction, 0.009. The results suggest that parental overweight and cardiometabolic diseases and pediatric sedentary and high sugar-intake lifestyles correlate with the development of adolescent MetS, and an elevated child BMI

  4. Pediatric cardiology. Clinical and practical experiences with heart diseases of children, juveniles and young adults; Kinderkardiologie. Klinik und Praxis der Herzerkrankungen bei Kindern, Jugendlichen und jungen Erwachsenen

    Energy Technology Data Exchange (ETDEWEB)

    Haas, Nikolaus A. [Herz- und Diabeteszentrum NRW, Bad Oeynhausen (Germany). Klinik fuer angeborene Herzfehler; Kleideiter, Ulrich [Klinik fuer Kinder- und Jugendmedizin, Coesfeld (Germany)

    2011-07-01

    The book on pediatric cardiology covers the following chapters: (I) Fundamentals and diagnostics: pediatric cardiologic anamnesis, electrocardiograms, thorax X-radiography, MRT and CT of the heart, nuclear medical diagnostics, exercise tests, heart catheter examination, electrophysiological tests. (II) Leading symptoms: Cyanosis, cardiac murmur, thorax pain, palpitation, syncopes. (III) Disease pictures: congenital heart defects, acquired heart defects, cardiomyopathies, heart rhythm disturbances, heart insufficiency, arterial hypertension, pulmonary hypertension, other heart involving syndromes. (IV) Therapy: Catheter interventional therapy, post-surgical pediatric cardiac therapy, surgery involving the life-support machine, mechanical cardiovascular support systems, initial treatment of newborns with critical heart defects, heart transplantation, vaccination of children with heart diseases, medicinal therapy.

  5. Cannabis and Pediatric Inflammatory Bowel Disease: Change Blossoms a Mile High.

    Science.gov (United States)

    Hoffenberg, Edward J; Newman, Heike; Collins, Colm; Tarbell, Sally; Leinwand, Kristina

    2017-02-01

    The trend toward decriminalization of cannabis (marijuana) continues sweeping across the United States. Colorado has been a leader of legalization of medical and recreational cannabis use. The growing public interest in the medicinal properties of cannabis and its use by patients with a variety of illnesses including inflammatory bowel disease (IBD) makes it important for pediatric gastroenterologists to understand this movement and its potential effect on patients. This article describes the path to legalization and "medicalization" of cannabis in Colorado and the public perception of safety despite the known adverse health effects of use. We delineate the mammalian endocannabinoid system and our experience of caring for children and adolescents with IBD in an environment of increasing awareness and acceptance of its use. We then summarize the rationale for considering that cannabis may have beneficial and harmful effects for patients with IBD. Finally, we highlight the challenges federal laws impose on conducting research on cannabis in IBD. The intent of this article is to inform health care providers about the issues around cannabis use and research in adolescents and young adults with IBD.

  6. T1-weighted dual-echo MRI for fat quantification in pediatric nonalcoholic fatty liver disease.

    Science.gov (United States)

    Pacifico, Lucia; Martino, Michele Di; Catalano, Carlo; Panebianco, Valeria; Bezzi, Mario; Anania, Caterina; Chiesa, Claudio

    2011-07-07

    To determine in obese children with nonalcoholic fatty liver disease (NAFLD) the accuracy of magnetic resonance imaging (MRI) in assessing liver fat concentration. A case-control study was performed. Cases were 25 obese children with biopsy-proven NAFLD. Controls were 25 obese children matched for age and gender, without NAFLD at ultrasonography and with normal levels of aminotransferases and insulin. Hepatic fat fraction (HFF) by MRI was obtained using a modification of the Dixon method. HFF ranged from 2% to 44% [mean, 19.0% (95% CI, 15.1-27.4)] in children with NAFLD, while in the controls this value ranged from 0.08% to 4.69% [2.0% (1.3-2.5), P steatosis (r = 0.883, P steatosis, the mean HFF was 8.7% (95% CI, 6.0-11.6) for mild, 21.6% (15.3-27.0) for moderate, and 39.7% (34.4-45.0) for severe fatty liver infiltration. With a cutoff of 4.85%, HFF had a sensitivity of 95.8% for the diagnosis of histological steatosis ≥ 5%. All control children had HFF lower than 4.85%; thus, the specificity was 100%. After 12 mo, children with weight loss displayed a significant decrease in HFF. MRI is an accurate methodology for liver fat quantification in pediatric NAFLD.

  7. Cannabis and pediatric inflammatory bowel disease: change blossoms a mile high

    Science.gov (United States)

    Hoffenberg, Edward J.; Newman, Heike; Collins, Colm; Leinwand, Kristina; Tarbell, Sally

    2016-01-01

    The trend towards decriminalization of cannabis (marijuana) continues sweeping across the United States. Colorado has been a leader of legalization of medical and recreational cannabis use. The growing public interest in the medicinal properties of cannabis and its use by patients with a variety of illnesses including inflammatory bowel disease (IBD) makes it important for pediatric gastroenterologists to understand this movement and its potential impact on patients. This article describes the path to legalization and “medicalization” of cannabis in Colorado as well as the public perception of safety despite the known adverse health effects of use. We delineate the mammalian endocannabinoid system and our experience of caring for children and adolescents with IBD in an environment of increasing awareness and acceptance of its use. We then summarize the rationale for considering that cannabis may have beneficial as well as harmful effects for IBD patients. Finally, we highlight the challenges federal laws impose on conducting research on cannabis in IBD. The intent of this article is to inform health care providers about the issues around cannabis use and research in adolescents and young adults with IBD. PMID:27579692

  8. Psychosocial and pharmacological management of pain in pediatric sickle cell disease.

    Science.gov (United States)

    Hildenbrand, Aimee K; Nicholls, Elizabeth G; Daly, Brian P; Marsac, Meghan L; Tarazi, Reem; Deepti, Raybagkar

    2014-03-01

    For children with sickle cell disease (SCD), pain is associated with significant current and future morbidity and mortality. Unfortunately, few evidence-based guidelines exist for the management of pain episodes in children with SCD. To inform empirically based treatment strategies for pain management in pediatric SCD, this review integrates and evaluates the extant literature on psychosocial and pharmacological approaches to the management of pain. Findings reveal a paucity of rigorous investigations of psychosocial and pharmacological pain management interventions in children with SCD. Psychosocial interventions included were primarily cognitive-behavioral in nature, whereas pharmacological approaches targeted non-opioid analgesics (ie, nonsteroidal anti-inflammatory drugs and corticosteroids) and opioid medications (ie, morphine and oxycodone). However, to date there is not a "gold standard" for pain management among children with SCD. Because psychosocial and physiological processes each play a role in the etiology and experience of pain, effective pain management requires multidimensional, comprehensive treatment approaches. Considering the significant impact of pain on functional outcomes and quality of life among children with SCD, additional clinical trials are warranted to ensure that interventions are safe and efficacious.

  9. Pediatric to Adult Care Transition: Perspectives of Young Adults With Sickle Cell Disease.

    Science.gov (United States)

    Porter, Jerlym S; Wesley, Kimberly M; Zhao, Mimi S; Rupff, Rebecca J; Hankins, Jane S

    2017-10-01

    The aim of this study was to explore perspectives of transition and transition readiness of young adult patients (YAs) with sickle cell disease (SCD) who have transitioned to adult health care. In all, 19 YAs with SCD (ages 18-30 years) participated in one of three focus groups and completed a brief questionnaire about transition topics. Transcripts were coded and emergent themes were examined using the social-ecological model of adolescent and young adult readiness for transition (SMART). Themes were consistent with most SMART components. Adult provider relationships and negative medical experiences emerged as salient factors. YAs ranked choosing an adult provider, seeking emergency care, understanding medications/medication adherence, knowing SCD complications, and being aware of the impact of health behaviors as the most important topics to include in transition programming. The unique perspectives of YAs can inform the development and evaluation of SCD transition programming by incorporating the identified themes. © The Author 2017. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  10. Pediatric vasculitis.

    Science.gov (United States)

    Barut, Kenan; Sahin, Sezgin; Kasapcopur, Ozgur

    2016-01-01

    The aim of this review is to define childhood vasculitis and to highlight new causative factors and treatment modalities under the guidance of recently published studies. Childhood vasculitis is difficult to diagnose because of the wide variation in the symptoms and signs. New nomenclature and classification criteria were proposed for the diagnosis of pediatric vasculitis. Recently, progress has been made toward understanding the genetic susceptibility to pediatric vasculitis as it was in other diseases. Various radiological techniques provide great opportunities in establishing the diagnosis of pediatric vasculitis. Mild central nervous system disease can accompany Henoch-Schonlein purpura and can go unnoticed. Antineutrophilic cytoplasmic antibody-associated vasculitis is rare in children. Increased severity of the disease, subglottic stenosis, and renal disease are described more frequently among children. Biological therapies are used with success in children as in adults. Future studies, whose aims are to evaluate treatment responses, prognosis and to design guidelines for activity, and damage index of vasculitis for children are required. Henoch-Schonlein purpura and Kawasaki disease are the most frequent vasculitides of children. Experience from adult studies for treatment and prognosis are usually used because of low incidence of other vasculitides in children. Multicenter studies of pediatric vasculitis should be conducted to detail treatment responses and prognosis in children.

  11. Fifty-Five Years of Pediatric Endocrinology and 50 Years of the Department of Pediatric Endocrinology, Diabetes and Metabolic Diseases in Slovenia

    Science.gov (United States)

    BATTELINO, Tadej

    2015-01-01

    Paediatric endocrinology started its independent development early in the general development of this specialty, with a strong focus on research and clinical excellence. Slovenian paediatric endocrinology was an integral part of the European paediatric endocrinology from its beginnings and a founding member of the first ‘International Study Group for Diabetes in Children and Adolescents’. After the pioneering work of Prof. Lev Matajc, Prof. Ciril Kržišnik firmly integrated the Department of Pediatric Endocrinology, Diabetes and Metabolic Diseases at the University Children’s Hospital in Ljubljana in the international scientific community. In the last decade, the department participates in cutting-edge research and provides clinical services at highest international standards. PMID:27646909

  12. Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden.

    Science.gov (United States)

    Schwarzenberg, Sarah Jane; Bellin, Melena; Husain, Sohail Z; Ahuja, Monika; Barth, Bradley; Davis, Heather; Durie, Peter R; Fishman, Douglas S; Freedman, Steven D; Gariepy, Cheryl E; Giefer, Matthew J; Gonska, Tanja; Heyman, Melvin B; Himes, Ryan; Kumar, Soma; Morinville, Veronique D; Lowe, Mark E; Nuehring, Neil E; Ooi, Chee Y; Pohl, John F; Troendle, David; Werlin, Steven L; Wilschanski, Michael; Yen, Elizabeth; Uc, Aliye

    2015-04-01

    To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. We performed a cross-sectional study of data from the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test. Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were white; median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; one or more pancreatic surgeries were performed in 30 (39%). Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Alterations in diversity of the oral microbiome in pediatric inflammatory bowel disease.

    Science.gov (United States)

    Docktor, Michael J; Paster, Bruce J; Abramowicz, Shelly; Ingram, Jay; Wang, Yaoyu E; Correll, Mick; Jiang, Hongyu; Cotton, Sean L; Kokaras, Alexis S; Bousvaros, Athos

    2012-05-01

    Oral pathology is a commonly reported extraintestinal manifestation of Crohn's disease (CD). The host-microbe interaction has been implicated in the pathogenesis of inflammatory bowel disease (IBD) in genetically susceptible hosts, yet limited information exists about oral microbes in IBD. We hypothesize that the microbiology of the oral cavity may differ in patients with IBD. Our laboratory has developed a 16S rRNA-based technique known as the Human Oral Microbe Identification Microarray (HOMIM) to study the oral microbiome of children and young adults with IBD. Tongue and buccal mucosal brushings from healthy controls, CD, and ulcerative colitis (UC) patients were analyzed using HOMIM. Shannon Diversity Index (SDI) and Principal Component Analysis (PCA) were employed to compare population and phylum-level changes among our study groups. In all, 114 unique subjects from the Children's Hospital Boston were enrolled. Tongue samples from patients with CD showed a significant decrease in overall microbial diversity as compared with the same location in healthy controls (P = 0.015) with significant changes seen in Fusobacteria (P < 0.0002) and Firmicutes (P = 0.022). Tongue samples from patients with UC did not show a significant change in overall microbial diversity as compared with healthy controls (P = 0.418). As detected by HOMIM, we found a significant decrease in overall diversity in the oral microbiome of pediatric CD. Considering the proposed microbe-host interaction in IBD, the ease of visualization and direct oral mucosal sampling of the oral cavity, further study of the oral microbiome in IBD is of potential diagnostic and prognostic value. Copyright © 2011 Crohn's & Colitis Foundation of America, Inc.

  14. Executive Functioning and School Performance Among Pediatric Survivors of Complex Congenital Heart Disease

    Science.gov (United States)

    Gerstle, Melissa; Beebe, Dean W.; Drotar, Dennis; Cassedy, Amy; Marino, Bradley S.

    2016-01-01

    Objective To investigate the presence and severity of real-world impairments in executive functioning– responsible for children’s regulatory skills (metacognition, behavioral regulation) – and its potential impact on school performance among pediatric survivors of complex congenital heart disease (CHD). Study design Survivors of complex CHD aged 8–16 years (n=143)and their parents/guardians from a regional CHD survivor registry participated (81% participation rate). Parents completed proxy measures of executive functioning, school competency, and school-related quality of life (QOL). Patients also completed a measure of school QOL and underwent IQ testing. Patients were categorized into two groups based on heart lesion complexity: two-ventricle or single-ventricle. Results Survivors of complex CHD performed significantly worse than norms for executive functioning, IQ, school competency, and school QOL. Metacognition was more severely affected than behavioral regulation, and metacognitive deficits were more often present in older children. Even after taking into account demographic factors, disease severity, and IQ, metacognition uniquely and strongly predicted poorer school performance. In exploratory analyses, patients with single-ventricle lesions were rated as having lower school competency and school QOL, and patients with two-ventricle lesions were rated as having poorer behavioral regulation. Conclusions Survivors of complex CHD experience greater executive functioning difficulties than healthy peers, with metacognition particularly impacted and particularly relevant for day-to-day school performance. Especially in older children, clinicians should watch for metacognitive deficits, such as problems with organization, planning, self-monitoring, and follow-through on tasks. PMID:26875011

  15. [Clinical application evaluation of Guidelines for the Diagnosis and Treatment of Common Diseases of Pediatrics in Traditional Chinese Medicine].

    Science.gov (United States)

    Liu, Meng-Yu; Yang, Wei; Wang, Li-Ying; Zhao, Xue-Yao; Wang, Yue-Xi; Liu, Yu-Qi; Han, Xue-Jie; Lv, Ai-Ping

    2017-09-01

    Clinical application evaluation research of Guidelines for the Diagnosis and Treatment of Common Diseases of Pediatrics in Traditional Chinese Medicine intends to evaluate the quality level and clinical application of the guideline. A questionnaire and prospective case survey methods were used to evaluate the applicability evaluation based on the clinician questionnaire and the application evaluation based on clinical case observation. The applicability evaluation, familiarity and utilization rate of doctors' guidelines were 85.06%, 62.76%; Sort by technical grade, intermediate grade doctors have a higher familiarity rate and utilization rate, while the junior grade doctor's is lower; Guide quality level of applicability evaluation, other items' rational percentage are better than 96% except the items of health preserving and prevention and other treatment is relatively low; Items' applicable percentage of applicability evaluation are more than 91% except the item of guide simplicity. Comprehensive applicability evaluation, The percentage of the guideline applicable to clinical practice accounted for 94.94%. The consistency rate of syndrome differentiation and clinical application is more than 96% in addition to prescription medication, other treatments and health preserving and prevention of the guidelines apply consistency of application evaluation. The percentage of good treatment effect accounted for 92.96% of application effect evaluation. The safety percentage is 99.89% and economy is 97.45%. The research shows that of Guidelines for the Diagnosis and Treatment of Common Diseases of Pediatrics in Traditional Chinese Medicine quality level is good and is basically applicable to pediatric clinical practice which can be used as a standardized recommendation of pediatric common diseases' treatment specification. A small part of the guidelines are not applicable and need to be further consummated. Health preserving and prevention and other treatment of the

  16. Pediatric Neglected Tropical Diseases in a Major Metropolitan Children's Hospital in the United States, 2004-2013.

    Science.gov (United States)

    Sweet, Leigh R; Palazzi, Debra L

    2016-12-01

    We conducted a retrospective study of neglected tropical diseases (NTDs) diagnosed at Texas Children's Hospital between 2004 and 2013. Forty-three patients with an NTD were identified; 47% had never traveled outside of the United States. The results of this study highlight the importance of physician awareness of NTDs in children in the United States. © The Author 2015. Published by Oxford University Press on behalf of the Pediatric Infectious Diseases Society. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  17. Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages

    DEFF Research Database (Denmark)

    Moorthy, Lakshmi Nandini; Roy, Elizabeth; Kurra, Vamsi

    2014-01-01

    BACKGROUND: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several......, Hungarian, Japanese, Romanian, Serbian and Xhosa. CONCLUSION: SMILY©-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY©-Illness with its available translations may be used...... features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases. FINDINGS: We adapted Simple...

  18. Research protocol for an epidemiological study on estimating disease burden of pediatric HIV in Belgaum district, India

    Directory of Open Access Journals (Sweden)

    Anju Sinha

    2016-05-01

    Full Text Available Abstract Background Pediatric HIV is poised to become a major public health problem in India with the rising trend of HIV infection in pregnant women (Department of AIDS Control, Ministry of Health and Family Welfare, http://www.naco.gov.in. There is lack of information on the epidemiology of pediatric HIV infection in India. Existing surveillance systems tend to underestimate the Pediatric burden. The overall aim of the present study is to estimate the disease burden of pediatric HIV among children in Belgaum district in the state of Karnataka in Southern India. An innovative multipronged epidemiological approach to comb the district is proposed. Methods The primary objectives of the study would be attained under three strategies. A prospective cohort design for objective (i to determine the incidence rate of HIV by early case detection in infants and toddlers (0–18 months born to HIV infected pregnant women; and cross sectional design for objectives (ii to determine the prevalence of HIV infection in children (0–14 years of HIV infected parents and (iii to determine the prevalence of HIV in sick children (0–14 years presenting with suspected signs and symptoms using age specific criteria for screening. Burden of pediatric HIV will be calculated as a product of cases detected in each strategy multiplied by a net inflation factor for each strategy. Study participants (i (ii (iii: HIV infected pregnant women and their live born children (ii Any HIV-infected man/woman, of age 18–49 years, having a biological child of age 0–14 years (iii Sick children of age 0–14 years presenting with suspected signs and symptoms and satisfying age-specific criteria for screening. Setting and conduct: Belgaum district which is a Category ‘A’ district (with more than 1 % antenatal prevalence in the district over the last 3 years before the study. Age-appropriate testing is used to detect HIV infection. Discussion There is a need to strengthen

  19. Research protocol for an epidemiological study on estimating disease burden of pediatric HIV in Belgaum district, India.

    Science.gov (United States)

    Sinha, Anju; Nath, Anita; Sethumadhavan, Rajeev; Isac, Shajy; Washington, Reynold

    2016-05-26

    Pediatric HIV is poised to become a major public health problem in India with the rising trend of HIV infection in pregnant women (Department of AIDS Control, Ministry of Health and Family Welfare, http://www.naco.gov.in). There is lack of information on the epidemiology of pediatric HIV infection in India. Existing surveillance systems tend to underestimate the Pediatric burden. The overall aim of the present study is to estimate the disease burden of pediatric HIV among children in Belgaum district in the state of Karnataka in Southern India. An innovative multipronged epidemiological approach to comb the district is proposed. The primary objectives of the study would be attained under three strategies. A prospective cohort design for objective (i) to determine the incidence rate of HIV by early case detection in infants and toddlers (0-18 months) born to HIV infected pregnant women; and cross sectional design for objectives (ii) to determine the prevalence of HIV infection in children (0-14 years) of HIV infected parents and (iii) to determine the prevalence of HIV in sick children (0-14 years) presenting with suspected signs and symptoms using age specific criteria for screening. Burden of pediatric HIV will be calculated as a product of cases detected in each strategy multiplied by a net inflation factor for each strategy. Study participants (i) (ii) (iii): HIV infected pregnant women and their live born children (ii) Any HIV-infected man/woman, of age 18-49 years, having a biological child of age 0-14 years (iii) Sick children of age 0-14 years presenting with suspected signs and symptoms and satisfying age-specific criteria for screening. Setting and conduct: Belgaum district which is a Category 'A' district (with more than 1 % antenatal prevalence in the district over the last 3 years before the study). Age-appropriate testing is used to detect HIV infection. There is a need to strengthen existing pediatric HIV estimation methods in India and other

  20. Clinical Signs, Causes, and Risk Factors of Pediatric Chronic Kidney Diseases: a Hospital-based Case-control Study

    Directory of Open Access Journals (Sweden)

    Parsa Yousefichaijan

    2016-06-01

    Full Text Available Background This retrospective study aimed to determine the epidemiologic characteristics and risk factors of chronic kidney diseases (CKD in patients < 18 years old at a single referral center. Materials and Methods In a hospital-based case control study, 66 CKD patients less than 18 years old were compared to 81 control patients (also under 18 without CKD. A patient was defined as a CKD case with renal injury and/or had a glomerular filtration rate (GFR of Results Fever, chills, and urinary tract infections were the most common clinical signs in the referred patients. Urinary tract infection (39.5% and growth failure (12.9% were the most important causes in referred pediatric CKD. After controlling the effect of confounding variables, household income, using packed water for drinking, percentile of body mass index (BMI, and gestational age were the significant predictors of pediatric CKD (P

  1. Pediatric neurobehavioral diseases in Nevada counties with respect to perchlorate in drinking water: an ecological inquiry.

    Science.gov (United States)

    Chang, Soju; Crothers, Carol; Lai, Shenghan; Lamm, Steven

    2003-10-01

    contamination in the drinking water. Furthermore, no difference in overall fourth-grade school performance was observed. No evidence was found that children from the area with perchlorate in the drinking water (up to 24 microg/liter) had either an increase in pediatric neurobehavioral disease (ADHD and autism) or a decrease in fourth-grade academic performance. The limitations of this ecological study relate to diagnostic criteria and ascertainment of geographic and demographic differences and to data on individual residence and water consumption during pregnancy. Copyright 2003 Wiley-Liss, Inc.

  2. Pediatric neurocritical care.

    Science.gov (United States)

    Murphy, Sarah

    2012-01-01

    Pediatric neurocritical care is an emerging multidisciplinary field of medicine and a new frontier in pediatric critical care and pediatric neurology. Central to pediatric neurocritical care is the goal of improving outcomes in critically ill pediatric patients with neurological illness or injury and limiting secondary brain injury through optimal critical care delivery and the support of brain function. There is a pressing need for evidence based guidelines in pediatric neurocritical care, notably in pediatric traumatic brain injury and pediatric stroke. These diseases have distinct clinical and pathophysiological features that distinguish them from their adult counterparts and prevent the direct translation of the adult experience to pediatric patients. Increased attention is also being paid to the broader application of neuromonitoring and neuroprotective strategies in the pediatric intensive care unit, in both primary neurological and primary non-neurological disease states. Although much can be learned from the adult experience, there are important differences in the critically ill pediatric population and in the circumstances that surround the emergence of neurocritical care in pediatrics.

  3. Trends in pediatric echocardiography and the yield for congenital heart disease in a major cardiac referral hospital in Cameroon.

    Science.gov (United States)

    Nkoke, Clovis; Balti, Eric; Menanga, Alain; Dzudie, Anastase; Lekoubou, Alain; Kingue, Samuel; Kengne, Andre Pascal

    2017-01-01

    Congenital heart disease (CHD) is a common condition in children in Sub-Saharan Africa (SSA), where it is associated with poor outcomes. Diagnosis of CHD in SSA depends essentially on echocardiography, which is available only in few urban referral centers. Our aim was to assess time changes in the pattern of referral for pediatric echocardiography and the subsequent diagnosis of structural CHD in a major SSA city. All pediatric echocardiography performed between 2004 and 2013 at the echocardiography laboratory of the Yaounde General Hospital were reviewed. The primary indication of the study and the presence of structural CHD were recorded. Between 2004 and 2013, 9,390 echocardiograms were performed and 834 (8.9%) children aged 1 day to 15 years underwent echocardiography at the center, and 227 (27.2%) cases of definite structural CHD were diagnosed, with 123 (54.2%) in boys. The most frequent indications for echocardiography were heart murmurs (40%) and the suspicion of CHD (37.4%). The commonest CHD was ventricular septal defect (VSD) (30%) with tetralogy of Fallot being the most frequent cyanotic heart lesion (5.3%). The proportion of pediatric echocardiography decreased from 13.3% in 2004-2005 to 6.1% in 2012-2013 (P=0.001) but not in a linear fashion (P=0.072 for linear trend).The diagnosis of structural CHD increased from 25.1% in 2004-2005 to 27.1% in 2012-2013. This increase however was non-significant (P=0.523) and did not follow a linear trend (P=0.230). The pattern of referral for pediatric echocardiography at this center has changed over time, but diagnosis of structural CHD has remained the same. Improving access to this diagnostic procedure and subsequent treatment of diagnosed CHD will help improving the outcome of the disease in this setting.

  4. Role of docosahexaenoic acid treatment in improving liver histology in pediatric nonalcoholic fatty liver disease.

    Science.gov (United States)

    Nobili, Valerio; Carpino, Guido; Alisi, Anna; De Vito, Rita; Franchitto, Antonio; Alpini, Gianfranco; Onori, Paolo; Gaudio, Eugenio

    2014-01-01

    Nonalcoholic fatty liver disease (NAFLD) is one of the most important causes of liver-related morbidity and mortality in children. Recently, we have reported the effects of docosahexaenoic acid (DHA), the major dietary long-chain polyunsaturated fatty acids, in children with NAFLD. DHA exerts a potent anti-inflammatory activity through the G protein-coupled receptor (GPR)120. Our aim was to investigate in pediatric NAFLD the mechanisms underlying the effects of DHA administration on histo-pathological aspects, GPR120 expression, hepatic progenitor cell activation and macrophage pool. 20 children with untreated NAFLD were included. Children were treated with DHA for 18 months. Liver biopsies before and after the treatment were analyzed. Hepatic progenitor cell activation, macrophage pool and GPR120 expression were evaluated and correlated with clinical and histo-pathological parameters. GPR120 was expressed by hepatocytes, liver macrophages, and hepatic progenitor cells. After DHA treatment, the following modifications were present: i) the improvement of histo-pathological parameters such as NAFLD activity score, ballooning, and steatosis; ii) the reduction of hepatic progenitor cell activation in correlation with histo-pathological parameters; iii) the reduction of the number of inflammatory macrophages; iv) the increase of GPR120 expression in hepatocytes; v) the reduction of serine-311-phosphorylated nuclear factor kappa B (NF-κB) nuclear translocation in hepatocytes and macrophages in correlation with serum inflammatory cytokines. DHA could modulate hepatic progenitor cell activation, hepatocyte survival and macrophage polarization through the interaction with GPR120 and NF-κB repression. In this scenario, the modulation of GPR120 exploits a novel crucial role in the regulation of the cell-to-cell cross-talk that drives inflammatory response, hepatic progenitor cell activation and hepatocyte survival.

  5. Role of docosahexaenoic acid treatment in improving liver histology in pediatric nonalcoholic fatty liver disease.

    Directory of Open Access Journals (Sweden)

    Valerio Nobili

    Full Text Available Nonalcoholic fatty liver disease (NAFLD is one of the most important causes of liver-related morbidity and mortality in children. Recently, we have reported the effects of docosahexaenoic acid (DHA, the major dietary long-chain polyunsaturated fatty acids, in children with NAFLD. DHA exerts a potent anti-inflammatory activity through the G protein-coupled receptor (GPR120. Our aim was to investigate in pediatric NAFLD the mechanisms underlying the effects of DHA administration on histo-pathological aspects, GPR120 expression, hepatic progenitor cell activation and macrophage pool.20 children with untreated NAFLD were included. Children were treated with DHA for 18 months. Liver biopsies before and after the treatment were analyzed. Hepatic progenitor cell activation, macrophage pool and GPR120 expression were evaluated and correlated with clinical and histo-pathological parameters.GPR120 was expressed by hepatocytes, liver macrophages, and hepatic progenitor cells. After DHA treatment, the following modifications were present: i the improvement of histo-pathological parameters such as NAFLD activity score, ballooning, and steatosis; ii the reduction of hepatic progenitor cell activation in correlation with histo-pathological parameters; iii the reduction of the number of inflammatory macrophages; iv the increase of GPR120 expression in hepatocytes; v the reduction of serine-311-phosphorylated nuclear factor kappa B (NF-κB nuclear translocation in hepatocytes and macrophages in correlation with serum inflammatory cytokines.DHA could modulate hepatic progenitor cell activation, hepatocyte survival and macrophage polarization through the interaction with GPR120 and NF-κB repression. In this scenario, the modulation of GPR120 exploits a novel crucial role in the regulation of the cell-to-cell cross-talk that drives inflammatory response, hepatic progenitor cell activation and hepatocyte survival.

  6. Whole body BMC in pediatric Crohn disease: independent effects of altered growth, maturation, and body composition.

    Science.gov (United States)

    Burnham, Jon M; Shults, Justine; Semeao, Edisio; Foster, Bethany; Zemel, Babette S; Stallings, Virginia A; Leonard, Mary B

    2004-12-01

    Whole body BMC was assessed in 104 children and young adults with CD and 233 healthy controls. CD was associated with significant deficits in BMC and lean mass, relative to height. Adjustment for lean mass eliminated the bone deficit in CD. Steroid exposure was associated with short stature but not bone deficits relative to height. Children with Crohn disease (CD) have multiple risk factors for impaired bone accrual. The confounding effects of poor growth and delayed maturation limit the interpretation of prior studies of bone health in CD. The objective of this study was to assess BMC relative to growth, body composition, and maturation in CD compared with controls. Whole body BMC and lean mass were assessed by DXA in 104 CD subjects and 233 healthy controls, 4-26 years of age. Multivariable linear regression models were developed to sequentially adjust for differences in skeletal size, pubertal maturation, and muscle mass. BMC-for-height z scores were derived to determine CD-specific covariates associated with bone deficits. Subjects with CD had significantly lower height z score, body mass index z score, and lean mass relative to height compared with controls (all p BMC in CD relative to controls was significantly reduced in males (0.86; 95% CI, 0.83, 0.94) and females (0.91; 95% CI, 0.85, 0.98) with CD. Adjustment for pubertal maturation did not alter the estimate; however, addition of lean mass to the model eliminated the bone deficit. Steroid exposure was associated with short stature but not bone deficits. This study shows the importance of considering differences in body size and composition when interpreting DXA data in children with chronic inflammatory conditions and shows an association between deficits in muscle mass and bone in pediatric CD.

  7. Oral triiodothyronine normalizes triiodothyronine levels after surgery for pediatric congenital heart disease*.

    Science.gov (United States)

    Marwali, Eva M; Boom, Cindy E; Sakidjan, Indriwanto; Santoso, Anwar; Fakhri, Dicky; Kartini, Ay; Kekalih, Aria; Schwartz, Steven M; Haas, Nikolaus A

    2013-09-01

    This study was conducted to determine if oral triiodothyronine supplementation could prevent the decrease of serum triiodothyronine levels that commonly occurs after cardiopulmonary bypass for pediatric congenital heart surgery. Secondary objectives included identifying any significant adverse effects of oral triiodothyronine supplementation, including any effects on the thyroid/pituitary axis. Randomized, placebo-controlled, doubleblind clinical trial Operating room and ICU. Infants and children younger than 2 years of age undergoing congenital heart surgery using cardiopulmonary bypass (n = 43). Subjects were assigned to placebo (n = 15, group A) or one of two treatment groups: a low-dose group (group B, n = 14, 0.5 mcg/kg triiodothyronine orally every 24 hr for 3 d) or a high-dose group (group C, n = 14, 0.5 mcg/kg triiodothyronine orally every 12 hr for 3 d). Thyroid hormone, including total and free triiodothyronine levels at predetermined time points, potential side effects indicating hyperthyroidism, indicators of the thyroid-pituitary axis, and clinical endpoints. Oral triiodothyronine supplementation twice-daily maintained serum triiodothyronine levels within normal limits in group C, whereas serum levels progressively declined in groups A and B. A statistically significant difference in triiodothyronine levels between the treatment groups occurred between 18 and 36 hours post cross-clamp release, with the largest difference in serum levels between group C and group A noted at 36 hours post cross-clamp release (total triiodothyronine, 0.71 ± 0.15 [0.34-1.08] ng/mL [p triiodothyronine, 2.56 ± 0.49 [1.33-3.79] pg/mL [p triiodothyronine supplementation at a dose of 0.5 mcg/kg every 12 hours for 3 days can maintain total and free triiodothyronine levels within normal limits after open-heart surgery using cardiopulmonary bypass for congenital heart disease.

  8. Impacts of endoscopic gastroesophageal flap valve grading on pediatric gastroesophageal reflux disease.

    Directory of Open Access Journals (Sweden)

    Kai-Chi Chang

    Full Text Available Gastroesophageal flap valve (GEFV endoscopic grading is reported to be associated with gastroesophageal reflux disease (GERD in adults; however its role in pediatric groups remains unknown. This study aimed to investigate the significance of GEFV grading and the associations to multichannel intraluminal impedance and pH monitoring (MII-pH in children with GERD.A total of 48 children with GERD symptoms who received esophagogastroduodenoscopy and MII-pH monitoring were enrolled. The degree of GEFV was graded from I to IV according to the Hill classification, and classified into two groups: normal GEFV (Hill grades I and II, and abnormal GEFV (Hill grades III and VI. Endoscopic findings and MII-pH monitoring were analyzed among the groups.Thirty-six patients had normal GEFV while 12 had abnormal GEFV. The presence of erosive esophagitis was significantly more common in the patients with abnormal GEFV (p = 0.037, OR 9.84, 95% CI 1.15-84.42. Pathological acidic gastroesophageal reflux (GER determined by MII-pH was more prevalent in the patients with loosened GEFV geometry (p = 0.01, OR 7.0, 95% CI 1.67-27.38. There were significant positive correlations between GEFV Hill grading I to IV and the severity of erosive esophagitis (r = 0.49, p<0.001, percentage of supine acid reflux (r = 0.37, p = 0.009, percentage of total acid reflux (r = 0.3284, p = 0.023, and DeMeester score (r = 0.36, p = 0.01 detected by pH monitoring. In the impedance study, GEFV Hill grading also positively correlated to median number of acid reflux events (r = 0.3015, p = 0.037.GEFV dysfunction highly associated with acid GER and severe erosive esophagitis. An abnormal GEFV is a sign of acid GER in children.

  9. Early-onset stroke with moyamoya-like syndrome and extraneurological signs: a first reported paediatric series

    International Nuclear Information System (INIS)

    Law-ye, Bruno; Saliou, Guillaume; Toulgoat, Frederique; Tardieu, Marc; Deiva, Kumaran; Adamsbaum, Catherine; Husson, Beatrice

    2016-01-01

    Moyamoya syndrome is characterised by an occlusion of the carotid terminations with the development of collateral vessels. Our objective is to describe a series of infants presenting early-onset moyamoya-like syndrome, which may constitute a distinct entity. From a cohort of children with rare cerebral vascular pathologies, we studied eight infants (28 days-1 year) with early-onset moyamoya-like syndrome demonstrated by angiography. We retrospectively analysed the patterns on MRI and MRA, as well as all other available data. Median age at diagnosis was 7 months (IQR: 6-8) with arterial ischaemic stroke in the middle cerebral artery territory. All of the children experienced severe stroke recurrence within a median time of 11 months (IQR: 10-12), and all showed extraneurological symptoms. The anterior cerebral circulation was involved in all cases and the posterior circulation was involved in six. Two children died and all of the other children suffered permanent neurological deficits. The presence of extraneurological signs in cases of early-onset moyamoya syndrome is suggestive of a newly described systemic vasculopathy with predominantly cerebrovascular expression. Given its rapid progression marked by severe recurrent strokes and poor clinical outcome, early diagnosis could help in the decision to institute aggressive therapy. (orig.)

  10. Pediatric stroke

    International Nuclear Information System (INIS)

    Hoermann, M.

    2008-01-01

    Stroke in childhood has gained increasingly more attention and is accepted as an important disease in childhood. The reasons for this severe event and the consequences for the rest of the life are totally different than for adults. This is also true for the diagnosis and therapy. This paper gives a comprehensive overview on the characteristics of pediatric stroke to assist radiologists in making a rapid and safe diagnosis in order to identify the underlying disease. (orig.) [de

  11. Pediatric emergency medicine: Optimizing risk assessment and safety netting in children with infectious diseases: Spoedeisende Kindergeneeskunde: Optimaliseren van risico inschatting en het vangnet rondom kinderen met koorts

    OpenAIRE

    Geurts, Dorien

    2018-01-01

    markdownabstractIntroduction In the introduction, the importance of good quality pediatric emergency care is explained. Acute illnesses in children differ among countries and settings. Regarding our population of the ED at the Erasmus MC in Rotterdam, The Netherlands, 1) we still observe mortality, although rare, 2) 45% of children with medical (non-trauma) complaints suffer from infectious diseases and 3) the number of revisits is high. As the main focus in research in pediatric emergency me...

  12. High-frequency oscillatory ventilation in pediatric acute hypoxemic respiratory failure: disease-specific morbidity survival analysis.

    Science.gov (United States)

    Babbitt, Christopher J; Cooper, Michael C; Nussbaum, Eliezer; Liao, Eileen; Levine, Glenn K; Randhawa, Inderpal S

    2012-12-01

    Multiple ventilatory strategies for acute hypoxemic respiratory failure (AHRF) in children have been advocated, including high-frequency oscillatory ventilation (HFOV). Despite the frequent deployment of HFOV, randomized controlled trials remain elusive and currently there are no pediatric trials looking at its use. Our longitudinal study analyzed the predictive clinical outcome of HFOV in pediatric AHRF given disease-specific morbidity. A retrospective 8-year review on pediatric intensive care unit admissions with AHRF ventilated by HFOV was performed. Primary outcomes included survival, morbidity, length of stay (LOS), and factors associated with survival or mortality. A total of 102 patients underwent HFOV with a 66 % overall survival rate. Survivors had a greater LOS than nonsurvivors (p = 0.001). Mortality odds ratio (OR) for patients without bronchiolitis was 8.19 (CI = 1.02, 65.43), and without pneumonia it was 3.07 (CI = 1.12, 8.39). A lower oxygenation index (OI) after HFOV commencement and at subsequent time points analyzed predicted survival. After 24 h, mortality was associated with an OI > 35 [OR = 31.11 (CI = 3.25, 297.98)]. Sepsis-related mortality was associated with a higher baseline FiO(2) (0.88 vs. 0.65), higher OI (42 vs. 22), and augmented metabolic acidosis (pH of 7.25 vs. 7.32) evaluated 4 h on HFOV (p < 0.05). High-frequency oscillatory ventilation may be safely utilized. It has a 66 % overall survival rate in pediatric AHRF of various etiologies. Patients with morbidity limited to the respiratory system and optimized oxygenation indices are most likely to survive on HFOV.

  13. DNA Methylation Adds Prognostic Value to Minimal Residual Disease Status in Pediatric T-Cell Acute Lymphoblastic Leukemia.

    Science.gov (United States)

    Borssén, Magnus; Haider, Zahra; Landfors, Mattias; Norén-Nyström, Ulrika; Schmiegelow, Kjeld; Åsberg, Ann E; Kanerva, Jukka; Madsen, Hans O; Marquart, Hanne; Heyman, Mats; Hultdin, Magnus; Roos, Göran; Forestier, Erik; Degerman, Sofie

    2016-07-01

    Despite increased knowledge about genetic aberrations in pediatric T-cell acute lymphoblastic leukemia (T-ALL), no clinically feasible treatment-stratifying marker exists at diagnosis. Instead patients are enrolled in intensive induction therapies with substantial side effects. In modern protocols, therapy response is monitored by minimal residual disease (MRD) analysis and used for postinduction risk group stratification. DNA methylation profiling is a candidate for subtype discrimination at diagnosis and we investigated its role as a prognostic marker in pediatric T-ALL. Sixty-five diagnostic T-ALL samples from Nordic pediatric patients treated according to the Nordic Society of Pediatric Hematology and Oncology ALL 2008 (NOPHO ALL 2008) protocol were analyzed by HumMeth450K genome wide DNA methylation arrays. Methylation status was analyzed in relation to clinical data and early T-cell precursor (ETP) phenotype. Two distinct CpG island methylator phenotype (CIMP) groups were identified. Patients with a CIMP-negative profile had an inferior response to treatment compared to CIMP-positive patients (3-year cumulative incidence of relapse (CIR3y ) rate: 29% vs. 6%, P = 0.01). Most importantly, CIMP classification at diagnosis allowed subgrouping of high-risk T-ALL patients (MRD ≥0.1% at day 29) into two groups with significant differences in outcome (CIR3y rates: CIMP negative 50% vs. CIMP positive 12%; P = 0.02). These groups did not differ regarding ETP phenotype, but the CIMP-negative group was younger (P = 0.02) and had higher white blood cell count at diagnosis (P = 0.004) compared with the CIMP-positive group. CIMP classification at diagnosis in combination with MRD during induction therapy is a strong candidate for further risk classification and could confer important information in treatment decision making. © 2016 Wiley Periodicals, Inc.

  14. Pediatric Specialists

    Science.gov (United States)

    ... Healthy Children > Family Life > Medical Home > Pediatric Specialists Pediatric Specialists Article Body ​Your pediatrician may refer your child to a pediatric specialist for further evaluation and treatment. Pediatric specialists ...

  15. Dynamic observation of transforming growth factor-alpha content in plasma of pediatric patients with peptic ulcer disease

    International Nuclear Information System (INIS)

    Zhou Mingxiong; Zhang Xinlu

    2001-01-01

    Objective: To elicit the relationship between transforming growth factor alpha (TGF-α) and the pathogenesis as well as healing process of peptic ulcer disease (PUD) in pediatric patients. Methods: The levels of TGF-α in plasma were measured by radioimmunoassay in 57 Children with PUD. Results: TGF-α levels of plasma at active stage of peptic ulcer were significantly lower than those at healing stage as well as in controls (P 0.05). Conclusion: There is an abnormal secretion of TGF-α in PUD patients. Changes of TGF-α release might play a role in the pathogenesis of PUD

  16. Role of flexible bronchoscopy and bronchoalveolar lavage in the diagnosis of pediatric acquired immunodeficiency syndrome-related pulmonary disease.

    Science.gov (United States)

    Birriel, J A; Adams, J A; Saldana, M A; Mavunda, K; Goldfinger, S; Vernon, D; Holzman, B; McKey, R M

    1991-06-01

    Flexible fiberoptic bronchoscopy with bronchoalveolar lavage was performed in 16 pediatric patients with the acquired immunodeficiency syndrome (AIDS) and deterioration in pulmonary function suggestive of opportunistic infection. In 62% of the patients Pneumocystis carinii was identified. Culture results showed a pure growth of Pseudomonas aeruginosa for one patient in addition to the Pneumocystis carinii. Bronchoscopy with lavage was well tolerated, with few complications even among patients with significant tachypnea and hypoxia. Because of its relative safety and effectiveness, this procedure should be considered the first invasive measurement used for evaluation of parenchymal lung disease in this population of patients.

  17. Progress in pediatrics in 2011. Choices in endocrinology, gastroenterology, hemato-oncology, infectious diseases, otolaryngology, pharmacotherapy and respiratory tract illnesses

    Directory of Open Access Journals (Sweden)

    Caffarelli Carlo

    2012-06-01

    Full Text Available Abstract Main progresses in endocrinology, gastroenterology, hemato-oncology, infectious diseases, otolaryngology, pharmacotherapy, and respiratory tract illnesses selected from articles published in The Italian Journal of Pediatrics in 2011 were reviewed. Risk factors for gastroenteritis and appendicitis in developing countries may be useful in improving our understanding of these diseases. Childhood hearing impairment is a world-wide problem which continues to have an high prevalence in newborns. Among the mechanisms of diseases, obese children often have asthma and high hepcidin levels that may reduce serum iron concentrations. In cystic fibrosis, 18q distal deletion has been described as a novel mutation. Hypothyroidism in children with central nervous system infections may increase mortality rates. Infrared tympanic thermometer (IRTT in oral mode for the measurement of body temperature may be useful in fever screening in a busy setup. In newborns, the transmission of CMV infection through breast milk may be prevented through freezing or pasteurization. Recent advances in treatment of constipation, urinary tract infections, leukemia, pain in children with cancer, neonates with sepsis or difficult weaning from mechanical ventilation will likely contribute towards optimizing management of these common disorders. The work of the Family Pediatricians Medicines for Children Research Network aims to develop competence, infrastructure, networking and education for pediatric clinical trials.

  18. Conformal proton radiation therapy for pediatric low-grade astrocytomas

    International Nuclear Information System (INIS)

    Hug, E.B.; Loma Linda Univ. Medical Center, Loma Linda, CA; Darthmouth-Hitchcock Medical Center, Lebanon, New Hampshire; Muenter, M.W.; Archambeau, J.O.; DeVries, A.; Loredo, L.N.; Grove, R.I.; Slater, J.D.; Liwnicz, B.

    2002-01-01

    Background: To evaluate the safety and efficacy of proton radiation therapy (PRT) for intracranial low-grade astrocytomas, the authors analyzed the first 27 pediatric patients treated at Loma Linda University Medical Center (LLUMC). Patients and Method: Between September 1991 and August 1997, 27 patients (13 female, 14 male) underwent fractionated proton radiation therapy for progressive or recurrent low-grade astrocytoma. Age at time of treatment ranged from 2 to 18 years (mean: 8.7 years). Tumors were located centrally (diencephatic) in 15 patients, in the cerebral and cerebellar hemispheres in seven patients, and in the brainstem in five patients. 25/27 patients (92%) were treated for progressive, unresectable, or residual disease following subtotal resection. Tissue diagnosis was available in 23/27 patients (85%). Four patients with optic pathway tumors were treated without histologic confirmation. Target doses between 50.4 and 63.0 CGE (cobalt gray equivalent, mean: 55.2 CGE) were prescribed at 1.8 CGE per fraction, five treatments per week. Results: At a mean follow-up period of 3.3 years (0.6-6.8 years), 6/27 patients experienced local failure (all located within the irradiated field), and 4/27 patients had died. By anatomic site these data translated into rates of local control and survival of 87% (13/15 patients) and 93% (14/15 patients) for central tumors, 71% (5/7 patients) and 86% (6/7 patients) for hemispheric tumors, and 60% (3/5 patients) and 60% (3/5 patients) for tumors located in the brainstem. Proton radiation therapy was generally well tolerated. All children with local control maintained their performance status. One child with associated neurofibromatosis, Type 1, developed Moyamoya disease. All six patients with optic pathway tumors and useful vision maintained or improved their visual status. Conclusions: This report on pediatric low-grade astrocytomas confirms proton radiation therapy as a safe and efficacious 3-D conformal treatment

  19. Consensus guidelines of ECCO/ESPGHAN on the medical management of pediatric Crohn's disease

    DEFF Research Database (Denmark)

    Ruemmele, F M; Veres, G; Kolho, K L

    2014-01-01

    immunomodulator based maintenance therapy. The experts discuss several factors potentially predictive for poor disease outcome (such as severe perianal fistulizing disease, severe stricturing/penetrating disease, severe growth retardation, panenteric disease, persistent severe disease despite adequate induction...

  20. Factors associated with preparedness of the US healthcare system to respond to a pediatric surge during an infectious disease pandemic: Is our nation prepared?

    Science.gov (United States)

    Anthony, Christy; Thomas, Tito Joe; Berg, Bridget M; Burke, Rita V; Upperman, Jeffrey S

    2017-01-01

    Recent incidents have demonstrated that the US health system is unprepared for infectious pandemics resulting in a pediatric surge. Development of efficient plans and a structured and coordinated regional response to pediatric pandemic surge remains an opportunity. To address this gap, we conducted a literature review to assess current efforts, propose a response plan structure, and recommend policy actions. A literature review, utilizing MEDLINE and PubMed, through March 2017 identified articles regarding infectious disease pandemics affecting the US pediatric population. After review of current literature, a proposed response plan structure for a pediatric pandemic surge was designed. Inclusion and exclusion criteria reduced an initial screening of 1,787 articles to 162 articles. Articles ranged in their discussion of pediatric pandemic surge. Review of the articles led to the proposal of organizing the results according to 4 S's; (1) Structure, (2) Staff, (3) Stuff (Resources), and (4) Space. The review has supported the concern that the US health system is unprepared for a pediatric surge induced by infectious disease pandemics. Common themes suggest that response plans should reflect the 4Ss and national guidelines must be translated into regional response systems that account for local nuances.

  1. Noninvasive detection of coronary abnormalities in pediatric patients with Kawassaki disease using multi-slice spiral CT

    International Nuclear Information System (INIS)

    Hou Yang; Guo Wenli; Yue Yong; Chen Liying; Guo Qiyong; Yu Xianyi; Wang Hong

    2006-01-01

    Objective: To evaluate the feasibility and value of detecting coronary artery lesions in Kawasaki disease using multi-slice computed tomography (MSCT). Methods: Thirty-four pediatric patients underwent 16-slice or 64-slice CT coronary, angiography. 18 patients were also examined with 2 dimension echocardiography (2DE). In all cases, visibility of coronary artery segment was recorded. The diameter of the LCA, RCA were measured in MSCTA and compared with 2DE. Correlation coefficient of dimension and coincidence rate of two methods were calculated. Results: Coronary artery lesions were found in 14 patients (22 branches) of the 34 cases with KD on MSCT. Six cases were dialated, 3 cases were dialated with aneurysms, 2 cases had aneurysms without dialation. Coronary artery stenosis in 1 eases, calcification in 2 cases. Three cases had multiple aneurysms with the presence of alternate stenosis that made the artery a bead-like appearance. CC of LM and RCA were 0.85, 0.91, respectively (P>0.05). Three coronary artery aneurysm in the distal RCA was missed by 2DE. MSCT could not detect slight or moderate mitral regurgitation in 2 patients and artery wall thickening in 5 patients. Conclusion: MSCT would be an effective complementary or alternative method for CDEC to evaluate coronary artery lesions non-invasively in pediatric patients with Kawasaki disease. (authors)

  2. Use of complementary and alternative medicine by pediatric patients with functional and organic gastrointestinal diseases: results from a multicenter survey.

    Science.gov (United States)

    Vlieger, Arine M; Blink, Marjolein; Tromp, Ellen; Benninga, Marc A

    2008-08-01

    Many pediatric patients use complementary and alternative medicine, especially when facing a chronic illness for which treatment options are limited. So far, research on the use of complementary and alternative medicine in patients with functional gastrointestinal disease has been scarce. This study was designed to assess complementary and alternative medicine use in children with different gastrointestinal diseases, including functional disorders, to determine which factors predicted complementary and alternative medicine use and to assess the willingness of parents to participate in future studies on complementary and alternative medicine efficacy and safety. The prevalence of complementary and alternative medicine use was assessed by using a questionnaire for 749 children visiting pediatric gastroenterology clinics of 9 hospitals in the Netherlands. The questionnaire consisted of 35 questions on the child's gastrointestinal disease, medication use, health status, past and future complementary and alternative medicine use, reasons for its use, and the necessity of complementary and alternative medicine research. In this study population, the frequency of complementary and alternative medicine use was 37.6%. A total of 60.3% of this group had used complementary and alternative medicine specifically for their gastrointestinal disease. This specific complementary and alternative medicine use was higher in patients with functional disorders than organic disorders (25.3% vs 17.2%). Adverse effects of allopathic medication, school absenteeism, age effect of conventional treatment were predictors of specific complementary and alternative medicine use. Almost all (93%) of the parents considered it important that pediatricians initiate complementary and alternative medicine research, and 51% of parents were willing to participate in future complementary and alternative medicine trials. Almost 40% of parents of pediatric gastroenterology patients are turning to

  3. Comparison of the New Adult Ventilator-Associated Event Criteria to the Centers for Disease Control and Prevention Pediatric Ventilator-Associated Pneumonia Definition (PNU2) in a Population of Pediatric Traumatic Brain Injury Patients.

    Science.gov (United States)

    Cirulis, Meghan M; Hamele, Mitchell T; Stockmann, Chris R; Bennett, Tellen D; Bratton, Susan L

    2016-02-01

    The new Centers for Disease Control and Prevention paradigm for ventilator-associated events is intended to simplify surveillance of infectious and noninfectious complications of mechanical ventilation in adults. We assessed the ventilator-associated events algorithm in pediatric patients. A retrospective observational cohort study. This single-center study took place in a PICU at an urban academic medical facility. Pediatric (ages 0-18 yr old) trauma patients with moderate-to-severe traumatic brain injury ventilated for greater than or equal to 2 days. We assessed for pediatric ventilator-associated pneumonia (as defined by current Centers for Disease Control and Prevention PNU2 guidelines), adult ventilator-associated events, and an experimental ventilator-associated events definition modified for pediatric patients. We compared ventilator-associated events to ventilator-associated pneumonia to calculate the test characteristics. Thirty-nine of 119 patients (33%) developed ventilator-associated pneumonia. Sensitivity of the adult ventilator-associated condition definition was 23% (95% CI, 11-39%), which increased to 56% (95% CI, 40-72%) using the modified pediatric ventilator-associated pneumonia criterion. Specificity reached 100% for both original and modified pediatric probable ventilator-associated pneumonia using ventilator-associated events criteria. Children who developed ventilator-associated pneumonia or ventilator-associated condition had similar baseline characteristics: age, mechanism of injury, injury severity scores, and use of an intracranial pressure monitor. Diagnosis of ventilator-associated pneumonia and ventilator-associated condition portended similarly unfavorable outcomes: longer median duration of ventilation, ICU and hospital length of stay, and more discharges to rehabilitation, home health, or nursing care compared with patients with no pulmonary complication. Both current and modified ventilator-associated events criteria have poor

  4. Social pediatrics: weaving horizontal and vertical threads through pediatric residency.

    Science.gov (United States)

    van den Heuvel, Meta; Martimianakis, Maria Athina Tina; Levy, Rebecca; Atkinson, Adelle; Ford-Jones, Elizabeth; Shouldice, Michelle

    2017-01-13

    Social pediatrics teaches pediatric residents how to understand disease within their patients' social, environmental and political contexts. It's an essential component of pediatric residency training; however there is very little literature that addresses how such a broad-ranging topic can be taught effectively. The aim of this study was to determine and characterize social pediatric education in our pediatric residency training in order to identify strengths and gaps. A social pediatrics curriculum map was developed, attending to 3 different dimensions: (1) the intended curriculum as prescribed by the Objectives of Training for Pediatrics of the Royal College of Physicians and Surgeons of Canada (RCPSC), (2) the formal curriculum defined by rotation-specific learning objectives, and (3) the informal/hidden curriculum as reflected in resident and teacher experiences and perceptions. Forty-one social pediatric learning objectives were extracted from the RCPSC Objectives of Training for Pediatrics, most were listed in the Medical Expert (51%) and Health Advocate competencies (24%). Almost all RCPSC social pediatric learning objectives were identified in more than one rotation and/or seminar. Adolescent Medicine (29.2%), Pediatric Ambulatory Medicine (26.2%) and Developmental Pediatrics (25%) listed the highest proportion of social pediatric learning objectives. Four (10%) RCPSC social pediatric objectives were not explicitly named within learning objectives of the formal curriculum. The informal curriculum revealed that both teachers and residents viewed social pediatrics as integral to all clinical encounters. Perceived barriers to teaching and learning of social pediatrics included time constraints, particularly in a tertiary care environment, and the value of social pediatrics relative to medical expert knowledge. Despite the lack of an explicit thematic presentation of social pediatric learning objectives by the Royal College and residency training program

  5. High-field MR imaging in pediatric congenital heart disease: Initial results

    International Nuclear Information System (INIS)

    Nguyen, Kim-Lien; Khan, Sarah N.; Moriarty, John M.; Mohajer, Kiyarash; Renella, Pierangelo; Boechat, M.I.; Finn, J.P.; Satou, Gary; Ayad, Ihab; Patel, Swati

    2015-01-01

    Comprehensive assessment of pediatric congenital heart disease (CHD) at any field strength mandates evaluation of both vascular and dynamic cardiac anatomy for which diagnostic quality contrast-enhanced magnetic resonance angiography (CEMRA) and cardiac cine are crucial. To determine whether high-resolution (HR) CEMRA and steady-state free precession (SSFP) cine can be performed reliably at 3.0 T in children with CHD and to compare the image quality to similar techniques performed at 1.5 T. Twenty-eight patients with a median age of 5 months and average weight 9.0 ± 7.8 kg with suspected or known CHD were evaluated at 3.0 T. SSFP cine (n = 86 series) and HR-CEMRA (n = 414 named vascular segments) were performed and images were scored for image quality and artifacts. The findings were compared to those of 28 patients with CHD of similar weight who were evaluated at 1.5 T. Overall image quality on HR-CEMRA was rated as excellent or good in 96% (397/414) of vascular segments at 3.0 T (k = 0.49) and in 94% (349/371) of vascular segments at 1.5 T (k = 0.36). Overall image quality of SSFP was rated excellent or good in 91% (78/86) of cine series at 3.0 T (k = 0.55) and in 81% (87/108) at 1.5 T (k = 0.47). Off-resonance artifact was common at both field strengths, varied over the cardiac cycle and was more prevalent at 3.0 T. At 3.0 T, off-resonance dark band artifact on SSFP cine was absent in 3% (3/86), mild in 69% (59/86), moderate in 27% (23/86) and severe in 1% (1/86) of images; at 1.5 T, dark band artifact was absent in 16% (17/108), mild in 69% (75/108), moderate in 12% (13/108) and severe in 3% (3/108) of cine images. The signal-to-noise ratio and contrast-to-noise ratio of both SSFP cine and HR-CEMRA images were significantly higher at 3.0 T than at 1.5 T (P < 0.001). Signal-to-noise ratio and contrast-to-noise ratio of high-resolution contrast-enhanced magnetic resonance angiography and SSFP cine were higher at 3.0 T than at 1.5 T. Artifacts on SSFP cine were

  6. High-field MR imaging in pediatric congenital heart disease: Initial results

    Energy Technology Data Exchange (ETDEWEB)

    Nguyen, Kim-Lien [David Geffen School of Medicine at UCLA, Division of Cardiology, VA Greater Los Angeles Healthcare System, Los Angeles, CA (United States); Khan, Sarah N.; Moriarty, John M.; Mohajer, Kiyarash; Renella, Pierangelo; Boechat, M.I.; Finn, J.P. [University of California at Los Angeles, Department of Radiological Sciences, Los Angeles, CA (United States); Satou, Gary [David Geffen School of Medicine at UCLA, Division of Pediatric Cardiology, Los Angeles, CA (United States); Ayad, Ihab; Patel, Swati [David Geffen School of Medicine at UCLA, Department of Anesthesia, Los Angeles, CA (United States)

    2014-08-03

    Comprehensive assessment of pediatric congenital heart disease (CHD) at any field strength mandates evaluation of both vascular and dynamic cardiac anatomy for which diagnostic quality contrast-enhanced magnetic resonance angiography (CEMRA) and cardiac cine are crucial. To determine whether high-resolution (HR) CEMRA and steady-state free precession (SSFP) cine can be performed reliably at 3.0 T in children with CHD and to compare the image quality to similar techniques performed at 1.5 T. Twenty-eight patients with a median age of 5 months and average weight 9.0 ± 7.8 kg with suspected or known CHD were evaluated at 3.0 T. SSFP cine (n = 86 series) and HR-CEMRA (n = 414 named vascular segments) were performed and images were scored for image quality and artifacts. The findings were compared to those of 28 patients with CHD of similar weight who were evaluated at 1.5 T. Overall image quality on HR-CEMRA was rated as excellent or good in 96% (397/414) of vascular segments at 3.0 T (k = 0.49) and in 94% (349/371) of vascular segments at 1.5 T (k = 0.36). Overall image quality of SSFP was rated excellent or good in 91% (78/86) of cine series at 3.0 T (k = 0.55) and in 81% (87/108) at 1.5 T (k = 0.47). Off-resonance artifact was common at both field strengths, varied over the cardiac cycle and was more prevalent at 3.0 T. At 3.0 T, off-resonance dark band artifact on SSFP cine was absent in 3% (3/86), mild in 69% (59/86), moderate in 27% (23/86) and severe in 1% (1/86) of images; at 1.5 T, dark band artifact was absent in 16% (17/108), mild in 69% (75/108), moderate in 12% (13/108) and severe in 3% (3/108) of cine images. The signal-to-noise ratio and contrast-to-noise ratio of both SSFP cine and HR-CEMRA images were significantly higher at 3.0 T than at 1.5 T (P < 0.001). Signal-to-noise ratio and contrast-to-noise ratio of high-resolution contrast-enhanced magnetic resonance angiography and SSFP cine were higher at 3.0 T than at 1.5 T. Artifacts on SSFP cine were

  7. Out-of-pocket Cost Burden in Pediatric Inflammatory Bowel Disease: A Cross-sectional Cohort Analysis.

    Science.gov (United States)

    Sin, Aaron T; Damman, Jennifer L; Ziring, David A; Gleghorn, Elizabeth E; Garcia-Careaga, Manuel G; Gugig, Roberto R; Hunter, Anna K; Burgis, Jennifer C; Bass, Dorsey M; Park, K T

    2015-06-01

    Pediatric inflammatory bowel disease (IBD), consisting of Crohn's disease (CD) and ulcerative colitis (UC), can result in significant morbidity requiring frequent health care utilization. Although it is known that the overall financial impact of pediatric IBD is significant, the direct out-of-pocket (OOP) cost burden on the parents of children with IBD has not been explored. We hypothesized that affected children with a more relapsing disease course and families in lower income strata, ineligible for need-based assistance programs, disparately absorb ongoing financial stress. We completed a cross-sectional analysis among parents of children with IBD residing in California using an online HIPAA-secure Qualtrics survey. Multicenter recruitment occurred between December 4, 2013 and September 18, 2014 at the point-of-care from site investigators, informational flyers distributed at regional CCFA conferences, and social media campaigns equally targeting Northern, Central, and Southern California. IBD-, patient-, and family-specific information were collected from the parents of pediatric patients with IBD patients younger than 18 years of age at time of study, carry a confirmed diagnosis of CD or UC, reside in and receive pediatric gastroenterology care in California, and do not have other chronic diseases requiring ongoing medical care. We collected 150 unique surveys from parents of children with IBD (67 CD; 83 UC). The median patient age was 14 years for both CD and UC, with an overall 3.7 years (SD 2.8 yr) difference between survey completion and time of IBD diagnosis. Annually, 63.6%, 28.6%, and 5.3% of families had an OOP cost burden >$500, >$1000, and >5000, respectively. Approximately one-third (36.0%) of patients had emergency department (ED) visits over the past year, with 59.2% of these patients spending >$500 on emergency department copays, including 11.1% who spent >$5000. Although 43.3% contributed $2000 in the past year. Families with household income

  8. Quantitative MRI for hepatic fat fraction and T2* measurement in pediatric patients with non-alcoholic fatty liver disease.

    Science.gov (United States)

    Deng, Jie; Fishbein, Mark H; Rigsby, Cynthia K; Zhang, Gang; Schoeneman, Samantha E; Donaldson, James S

    2014-11-01

    Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. The gold standard for diagnosis is liver biopsy. MRI is a non-invasive imaging method to provide quantitative measurement of hepatic fat content. The methodology is particularly appealing for the pediatric population because of its rapidity and radiation-free imaging techniques. To develop a multi-point Dixon MRI method with multi-interference models (multi-fat-peak modeling and bi-exponential T2* correction) for accurate hepatic fat fraction (FF) and T2* measurements in pediatric patients with NAFLD. A phantom study was first performed to validate the accuracy of the MRI fat fraction measurement by comparing it with the chemical fat composition of the ex-vivo pork liver-fat homogenate. The most accurate model determined from the phantom study was used for fat fraction and T2* measurements in 52 children and young adults referred from the pediatric hepatology clinic with suspected or identified NAFLD. Separate T2* values of water (T2*W) and fat (T2*F) components derived from the bi-exponential fitting were evaluated and plotted as a function of fat fraction. In ten patients undergoing liver biopsy, we compared histological analysis of liver fat fraction with MRI fat fraction. In the phantom study the 6-point Dixon with 5-fat-peak, bi-exponential T2* modeling demonstrated the best precision and accuracy in fat fraction measurements compared with other methods. This model was further calibrated with chemical fat fraction and applied in patients, where similar patterns were observed as in the phantom study that conventional 2-point and 3-point Dixon methods underestimated fat fraction compared to the calibrated 6-point 5-fat-peak bi-exponential model (P fat fraction, T2*W (27.9 ± 3.5 ms) decreased, whereas T2*F (20.3 ± 5.5 ms) increased; and T2*W and T2*F became increasingly more similar when fat fraction was higher than 15-20%. Histological fat

  9. Quantitative MRI for hepatic fat fraction and T2* measurement in pediatric patients with non-alcoholic fatty liver disease

    Energy Technology Data Exchange (ETDEWEB)

    Deng, Jie; Rigsby, Cynthia K.; Donaldson, James S. [Ann and Robert H. Lurie Children' s Hospital of Chicago, Department of Medical Imaging, Chicago, IL (United States); Northwestern University, Department of Radiology, Feinberg School of Medicine, Chicago, IL (United States); Fishbein, Mark H. [Ann and Robert H. Lurie Children' s Hospital of Chicago, Division of Gastroenterology, Hepatology, and Nutrition, Chicago, IL (United States); Zhang, Gang [Ann and Robert H. Lurie Children' s Hospital of Chicago, Biostatistics Research Core, Chicago, IL (United States); Schoeneman, Samantha E. [Ann and Robert H. Lurie Children' s Hospital of Chicago, Department of Medical Imaging, Chicago, IL (United States)

    2014-11-15

    Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. The gold standard for diagnosis is liver biopsy. MRI is a non-invasive imaging method to provide quantitative measurement of hepatic fat content. The methodology is particularly appealing for the pediatric population because of its rapidity and radiation-free imaging techniques. To develop a multi-point Dixon MRI method with multi-interference models (multi-fat-peak modeling and bi-exponential T2* correction) for accurate hepatic fat fraction (FF) and T2* measurements in pediatric patients with NAFLD. A phantom study was first performed to validate the accuracy of the MRI fat fraction measurement by comparing it with the chemical fat composition of the ex-vivo pork liver-fat homogenate. The most accurate model determined from the phantom study was used for fat fraction and T2* measurements in 52 children and young adults referred from the pediatric hepatology clinic with suspected or identified NAFLD. Separate T2* values of water (T2*{sub W}) and fat (T2*{sub F}) components derived from the bi-exponential fitting were evaluated and plotted as a function of fat fraction. In ten patients undergoing liver biopsy, we compared histological analysis of liver fat fraction with MRI fat fraction. In the phantom study the 6-point Dixon with 5-fat-peak, bi-exponential T2* modeling demonstrated the best precision and accuracy in fat fraction measurements compared with other methods. This model was further calibrated with chemical fat fraction and applied in patients, where similar patterns were observed as in the phantom study that conventional 2-point and 3-point Dixon methods underestimated fat fraction compared to the calibrated 6-point 5-fat-peak bi-exponential model (P < 0.0001). With increasing fat fraction, T2*{sub W} (27.9 ± 3.5 ms) decreased, whereas T2*{sub F} (20.3 ± 5.5 ms) increased; and T2*{sub W} and T2*{sub F} became increasingly more similar when fat

  10. A patient with Moyamoya-like vessels after radiation therapy for a tumor in the basal ganglia

    International Nuclear Information System (INIS)

    Ishiyama, Koichi; Tomura, Noriaki; Kato, Koki; Takahashi, Satoshi; Watarai, Jiro; Sasajima, Toshio; Mizoi, Kazuo

    2001-01-01

    A patient with Moyamoya-like vessels after radiation therapy for treatment of a tumor in the basal ganglia is reported. He was diagnosed as Down syndrome at birth. He had a tumor in the left basal ganglionic region at 12 years of the age. The tumor increased in size at age 14. He underwent cerebral angiography, which did not show a stenosis nor occlusion of the internal carotid artery, anterior cerebral artery, nor the middle cerebral artery. He received radiation therapy with a total dose of 56 Gy. He presented a dressing apraxia at age 19. MRI showed cerebral infarction in the left temporo-occipital region. Right internal carotid angiography revealed a severe stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the right side. Moyamoya-like vessels were seen in the basal ganglionic region. Left internal carotid angiography also showed a stenosis of the internal carotid artery and anterior cerebral artery as well as a severe stenosis of the middle cerebral artery on the left side. Moyamoya-like vessels were seen in the basal ganglionic region. Leptomeningeal anastomose and transdural anastomose were bilaterally seen. These arterial occlusion and stenotic phenomenon corresponded to a previous radiation field. These Moyamoya-like vessels with arterial stenosis and occlusion were thought to be due to radiation-induced vasculopathy, because a previous cerebral angiography showed a normal caliber of cerebral arteries. This patient showed that patients with radiation therapy in their early childhood should be carefully observed considering the possibility of the phenomenon. (author)

  11. Evaluation of antibody response to polysaccharide vaccine and switched memory B cells in pediatric patients with inflammatory bowel disease.

    Science.gov (United States)

    Fallahi, Gholamhossein; Aghamohammadi, Asghar; Khodadad, Ahmad; Hashemi, Mojtaba; Mohammadinejad, Payam; Asgarian-Omran, Hossein; Najafi, Mehri; Farhmand, Fatemeh; Motamed, Farzaneh; Soleimani, Khadije; Soheili, Habib; Parvaneh, Nima; Darabi, Behzad; Nasiri Kalmarzi, Rasoul; Pourhamdi, Shabnam; Abolhassani, Hassan; Mirminachi, Babak; Rezaei, Nima

    2014-01-01

    Inflammatory bowel disease (IBD) is a chronic disease of the gastrointestinal tract, whose etiologies are still unknown. This study was performed to evaluate the humoral immune response in terms of B cell functions in selected IBD patients. Eighteen pediatric patients with IBD, including 12 cases of ulcerative colitis (UC) and six with Crohn disease (CD), were enrolled in this study. The pneumococcal vaccine was injected in all patients, and the IgG antibody level to the polysaccharide antigen was measured before and 4 weeks after injection. The B cell switch-recombination process was evaluated. Five patients with IBD (three CD and two UC) had defects in B cell switching, which was significantly higher than in controls (p=0.05). Ten patients had a specific antibody deficiency and exhibited a higher frequency of bacterial infection than the healthy group. The mean increased level of IgG after vaccination was lower in IBD patients (82.9±32.5 µg/mL vs 219.8±59.0 µg/mL; p=0.001). Among the patients who had an insufficient response, no significant difference in the number of switched memory B-cell was observed. A defect in B lymphocyte switching was observed in pediatric IBD patients, and especially in those patients with CD. Owing to an increased risk of bacterial infections in those patients with antibody production defects, pneumococcal vaccination could be recommended. However, not all patients can benefit from the vaccination, and several may require other prophylactic methods.

  12. Implantable cardioverter defibrillator therapy in pediatric and congenital heart disease patients: a single tertiary center experience in Korea

    Directory of Open Access Journals (Sweden)

    Bo Kyung Jin

    2013-03-01

    Full Text Available Purpose: The use of implantable cardioverter defibrillators (ICDs to prevent sudden cardiac death is increasing in children and adolescents. This study investigated the use of ICDs in children with congenital heart disease. Methods: This retrospective study was conducted on the clinical characteristics and effectiveness of ICD implantation at the department of pediatrics of a single tertiary center between 2007 and 2011. Results: Fifteen patients underwent ICD implantation. Their mean age at the time of implantation was 14.5±5.4 years (range, 2 to 22 years. The follow-up duration was 28.9±20.4 months. The cause of ICD implantation was cardiac arrest in 7, sustained ventricular tachycardia in 6, and syncope in 2 patients. The underlying disorders were as follows: ionic channelopathy in 6 patients (long QT type 3 in 4, catecholaminergic polymorphic ventricular tachycardia [CPVT] in 1, and J wave syndrome in 1, cardiomyopathy in 5 patients, and postoperative congenital heart disease in 4 patients. ICD coils were implanted in the pericardial space in 2 children (ages 2 and 6 years. Five patients received appropriate ICD shock therapy, and 2 patients received inappropriate shocks due to supraventricular tachycardia.During follow-up, 2 patients required lead dysfunction-related revision. One patient with CPVT suffered from an ICD storm that was resolved using sympathetic denervation surgery. Conclusion: The overall ICD outcome was acceptable in most pediatric patients. Early diagnosis and timely ICD implantation are recommended for preventing sudden death in high-risk children and patients with congenital heart disease.

  13. Integrating Interactive Web-Based Technology to Assess Adherence and Clinical Outcomes in Pediatric Sickle Cell Disease

    Directory of Open Access Journals (Sweden)

    Lori E. Crosby

    2012-01-01

    Full Text Available Research indicates that the quality of the adherence assessment is one of the best predictors for improving clinical outcomes. Newer technologies represent an opportunity for developing high quality standardized assessments to assess clinical outcomes such as patient experience of care but have not been tested systematically in pediatric sickle cell disease (SCD. The goal of the current study was to pilot an interactive web-based tool, the Take-Charge Program, to assess adherence to clinic visits and hydroxyurea (HU, barriers to adherence, solutions to overcome these barriers, and clinical outcomes in 43 patients with SCD age 6–21 years. Results indicate that the web-based tool was successfully integrated into the clinical setting while maintaining high patient satisfaction (>90%. The tool provided data consistent with the medical record, staff report, and/or clinical lab data. Participants reported that forgetting and transportation were major barriers for adherence to both clinic attendance and HU. A greater number of self-reported barriers (P<.01 and older age (P<.05 were associated with poorer clinic attendance and HU adherence. In summary, the tool represents an innovative approach to integrate newer technology to assess adherence and clinical outcomes for pediatric patients with SCD.

  14. [Formula: see text]Executive functioning and health-related quality of life in pediatric sickle cell disease.

    Science.gov (United States)

    Allen, Taryn M; Anderson, Lindsay M; Rothman, Jennifer A; Bonner, Melanie J

    2017-11-01

    Research consistently indicates that children with sickle cell disease (SCD) face multiple risk factors for neurocognitive impairment. Despite this, no empirical research to date has examined the impact of neurocognitive functioning on quality of life for this pediatric group. Thus, the current study aims to examine the relationship between executive functioning and quality of life in a sample of children with SCD and further explore psychosocial and family/caregiver resources as moderators of this relationship. A total of 45 children with SCD aged 8 to 16 years and their caregivers completed measures of quality of life, behavioral ratings of executive functioning, and psychosocial functioning. Hierarchical linear regression models were utilized to determine the impact of executive functioning on quality of life and further test the interaction effects of proposed moderating variables. Controlling for age, pain, and socioeconomic status (SES), executive functioning was found to significantly predict child- and parent-reported quality of life among youth with SCD. Psychosocial resources of the primary caregiver or family was not found to moderate the relationship between executive functioning and quality of life. These results provide the first empirical evidence that lower executive skills negatively predict quality of life for children with SCD, supporting clinical and research efforts which aim to establish efficacious interventions that target cognitive decrements within this pediatric population.

  15. Barriers and facilitators to successful transition from pediatric to adult inflammatory bowel disease care from the perspectives of providers.

    Science.gov (United States)

    Paine, Christine W; Stollon, Natalie B; Lucas, Matthew S; Brumley, Lauren D; Poole, Erika S; Peyton, Tamara; Grant, Anne W; Jan, Sophia; Trachtenberg, Symme; Zander, Miriam; Mamula, Petar; Bonafide, Christopher P; Schwartz, Lisa A

    2014-11-01

    For adolescents and young adults (AYA) with inflammatory bowel disease (IBD), the transition from pediatric to adult care is often challenging and associated with gaps in care. Our study objectives were to (1) identify outcomes for evaluating transition success and (2) elicit the major barriers and facilitators of successful transition. We interviewed pediatric and adult IBD providers from across the United States with experience caring for AYAs with IBD until thematic saturation was reached after 12 interviews. We elicited the participants' backgrounds, examples of successful and unsuccessful transition of AYAs for whom they cared, and recommendations for improving transition using the Social-Ecological Model of Adolescent and Young Adult Readiness to Transition framework. We coded interview transcripts using the constant comparative method and identified major themes. Participants reported evaluating transition success and failure using health care utilization outcomes (e.g., maintaining continuity with adult providers), health outcomes (e.g., stable symptoms), and quality of life outcomes (e.g., attending school). The patients' level of developmental maturity (i.e., ownership of care) was the most prominent determinant of transition outcomes. The style of parental involvement (i.e., helicopter parent versus optimally involved parent) and the degree of support by providers (e.g., care coordination) also influenced outcomes. IBD transition success is influenced by a complex interplay of patient developmental maturity, parenting style, and provider support. Multidisciplinary IBD care teams should aim to optimize these factors for each patient to increase the likelihood of a smooth transfer to adult care.

  16. Advances in pediatric dentistry.

    Science.gov (United States)

    Yoon, Richard K; Best, Jed M

    2011-07-01

    This article addresses advances in 4 key areas related to pediatric dentistry: (1) caries detection tools, (2) early interventions to arrest disease progression, (3) caries-risk assessment tools, and (4) trends in pediatric procedures and dental materials. Copyright © 2011 Elsevier Inc. All rights reserved.

  17. Neuroinflammation in Ischemic Pediatric Stroke.

    Science.gov (United States)

    Steinlin, Maja

    2017-08-01

    Over the last decades, the importance of inflammatory processes in pediatric stroke have become increasingly evident. Ischemia launches a cascade of events: activation and inhibition of inflammation by a large network of cytokines, adhesion and small molecules, protease, and chemokines. There are major differences in the neonatal brain compared to adult brain, but developmental trajectories of the process during childhood are not yet well known. In neonatal stroke ischemia is the leading pathophysiology, but infectious and inflammatory processes have a significant input into the course and degree of tissue damage. In childhood, beside inflammation lanced by ischemia itself, the event of ischemia might be provoked by an underlying inflammatory pathophysiology: transient focal arteriopathy, dissection, sickle cell anemia, Moyamoya and more generalized in meningitides, generalized vasculitis or genetic arteriopathies (as in ADA2). Focal inflammatory reactions tend to be located in the distal part of the carotid artery or the proximal medial arteries, but generalized processes rather tend to affect the small arteries. Copyright © 2017. Published by Elsevier Inc.

  18. Health-related quality of life in patients with pediatric onset of end-stage renal disease: state of the art and recommendations for clinical practice

    NARCIS (Netherlands)

    Tjaden, Lidwien A.; Grootenhuis, Martha A.; Noordzij, Marlies; Groothoff, Jaap W.

    2016-01-01

    Health-related quality of life (HRQoL) is increasingly recognized as a key outcome in both clinical and research settings in the pediatric population with end-stage renal disease (ESRD). This review aims to: (1) summarize the current knowledge on HRQoL and socioprofessional outcomes and (2) provide

  19. Paternal involvement in pediatric Type 1 diabetes: fathers' and mothers' psychological functioning and disease management.

    Science.gov (United States)

    Hansen, Jennifer A; Weissbrod, Carol; Schwartz, David D; Taylor, W Patrick

    2012-03-01

    Psychological functioning in fathers of children with Type 1 diabetes has received relatively little attention compared to mothers. This study examined fathers' perceived involvement in their children's diabetes care as it related to mothers' and fathers' pediatric parenting stress, depression, anxiety, marital satisfaction, and sleep, and to their children's diabetes regimen adherence and glycemic control. Eighty-two mothers and 43 fathers completed questionnaires. Multivariate linear regressions were conducted separately for mothers and fathers to determine the relationships between the perceived amount and the perceived helpfulness of father involvement in child diabetes care on parental psychosocial functioning and child diabetes control. Maternal perceptions of father helpfulness and amount of involvement in illness care were related to improved marital satisfaction and fewer depressive symptoms in mothers. In fathers, perception of their own amount of involvement was related to increased pediatric parenting stress and anxiety. Better child regimen adherence was associated with maternal perceptions of father helpfulness but not the amount of their involvement, while paternal perceptions of their own helpfulness were related to poorer glycemic control. These findings suggest that fathers and mothers may react differently to their roles in childhood illness and that perceptions of their involvement may be differently associated with children's glycemic control and regimen adherence.

  20. Progress in pediatrics in 2013: choices in allergology, endocrinology, gastroenterology, hypertension, infectious diseases, neonatology, neurology, nutrition and respiratory tract illnesses.

    Science.gov (United States)

    Caffarelli, Carlo; Santamaria, Francesca; Vottero, Alessandra; Dascola, Carlotta Povesi; Mirra, Virginia; Sperli, Francesco; Bernasconi, Sergio

    2014-07-12

    This review will provide new information related to pathophysiology and management of specific diseases that have been addressed by selected articles published in the Italian Journal of Pediatrics in 2013, focusing on allergology, endocrinology, gastroenterology, hypertension, infectious diseases, neonatology, neurology, nutrition and respiratory tract illnesses in children. Recommendations for interpretation of skin prick test to foods in atopic eczema, management of allergic conjunctivitis, hypertension and breastfeeding in women treated with antiepileptic drugs and healthy breakfast have been reported. Epidemiological studies have given emphasis to high incidence of autoimmune disorders in patients with Turner syndrome, increasing prevalence of celiac disease, frequency of hypertension in adolescents, incidence and risk factor for retinopathy of prematurity. Advances in prevention include elucidation of the role of probiotics in reducing occurrence of allergies and feeding intolerance, and events of foetal life that influence later onset of diseases. Mechanistic studies suggested a role for vitamin D deficiency in asthma and type 1 diabetes and for reactivation of Varicella-Zoster virus in aseptic meningitis. Regarding diagnosis, a new mean for the diagnosis of hyperbilirubinaemia in newborns, a score for recognition of impaired nutritional status and growth and criteria for early Dyke-Davidoff-Masson Syndrome have been suggested. New therapeutic approaches consist of use of etanercept for reducing insulin dose in type 1 diabetes, probiotics in atopic eczema, and melatonin in viral infections.

  1. A pediatric case with peripheral facial nerve palsy caused by a granulomatous lesion associated with cat scratch disease.

    Science.gov (United States)

    Nakamura, Chizuko; Inaba, Yuji; Tsukahara, Keiko; Mochizuki, Mie; Sawanobori, Emi; Nakazawa, Yozo; Aoyama, Kouki

    2018-02-01

    Cat scratch disease is a common infectious disorder caused by Bartonella henselae that is transmitted primarily by kittens. It typically exhibits a benign and self-limiting course of subacute regional lymphadenopathy and fever lasting two to eight weeks. The most severe complication of cat scratch disease is involvement of the nervous system, such as encephalitis, meningitis, and polyneuritis. Peripheral facial nerve palsy associated with Bartonella infection is rare; few reported pediatric and adult cases exist and the precise pathogenesis is unknown. A previously healthy 7-year-old boy presented with fever, cervical lymphadenopathy, and peripheral facial nerve palsy associated with serologically confirmed cat scratch disease. The stapedius muscle reflex was absent on the left side and brain magnetic resonance imaging revealed a mass lesion at the left internal auditory meatus. The patient's symptoms and imaging findings were gradually resolved after the antibiotics and corticosteroids treatment. The suspected granulomatous lesion was considered to have resulted from the host's immune reaction to Bartonella infection and impaired the facial nerve. This is the first case report providing direct evidence of peripheral facial nerve palsy caused by a suspected granulomatous lesion associated with cat scratch disease and its treatment course. Copyright © 2017. Published by Elsevier B.V.

  2. Pediatric Electrocardiographic Imaging (ECGI) Applications

    Science.gov (United States)

    Silva, Jennifer N. A.

    2014-01-01

    Summary Noninvasive electrocardiographic imaging (ECGI) has been used in pediatric and congenital heart patients to better understand their electrophysiologic substrates. In this article we focus on the 4 subjects related to pediatric ECGI: 1) ECGI in patients with congenital heart disease and Wolff-Parkinson-White syndrome, 2) ECGI in patients with hypertrophic cardiomyopathy and pre-excitation, 3) ECGI in pediatric patients with Wolff-Parkinson-White syndrome, and 4) ECGI for pediatric cardiac resynchronization therapy. PMID:25722754

  3. Pediatric Sinusitis

    Science.gov (United States)

    ... ENTCareers Marketplace Find an ENT Doctor Near You Pediatric Sinusitis Pediatric Sinusitis Patient Health Information News media interested in ... sinuses are present at birth. Unlike in adults, pediatric sinusitis is difficult to diagnose because symptoms of ...

  4. Pediatric Asthma

    Science.gov (United States)

    ... Science Education & Training Home Conditions Asthma (Pediatric) Asthma (Pediatric) Make an Appointment Refer a Patient Ask a ... meet the rising demand for asthma care. Our pediatric asthma team brings together physicians, nurses, dietitians, physical ...

  5. DNA Methylation Adds Prognostic Value to Minimal Residual Disease Status in Pediatric T-Cell Acute Lymphoblastic Leukemia

    DEFF Research Database (Denmark)

    Borssén, Magnus; Haider, Zahra; Landfors, Mattias

    2016-01-01

    . In modern protocols, therapy response is monitored by minimal residual disease (MRD) analysis and used for postinduction risk group stratification. DNA methylation profiling is a candidate for subtype discrimination at diagnosis and we investigated its role as a prognostic marker in pediatric T......: 29% vs. 6%, P = 0.01). Most importantly, CIMP classification at diagnosis allowed subgrouping of high-risk T-ALL patients (MRD ≥0.1% at day 29) into two groups with significant differences in outcome (CIR3y rates: CIMP negative 50% vs. CIMP positive 12%; P = 0.02). These groups did not differ...... regarding ETP phenotype, but the CIMP-negative group was younger (P = 0.02) and had higher white blood cell count at diagnosis (P = 0.004) compared with the CIMP-positive group. CONCLUSIONS: CIMP classification at diagnosis in combination with MRD during induction therapy is a strong candidate for further...

  6. Perinatal vitamin D levels are not associated with later risk of developing pediatric-onset inflammatory bowel disease

    DEFF Research Database (Denmark)

    Thorsen, Steffen U; Jakobsen, Christian; Cohen, Arieh

    2016-01-01

    )D at birth were associated with increased risk of developing pediatric-onset IBD. Material and methods A case-cohort study composed of cases diagnosed with Crohn’s disease, ulcerative colitis or indeterminate/unclassified colitis and healthy controls. Cases and controls were matched on date of birth and were...... and two-way ANOVA were used to test for season and birth year 25(OH)D variations. A total of 384 matched pairs were included in the statistical analyses. Results No significant association were found between levels of 25(OH)D and IBD risk in the adjusted model (OR [95% CI] (per 25 nmol/L increase), 1.......12 [0.88; 1.42], p = 0.35). 25(OH)D levels were found to fluctuate significantly with season (p controls. Conclusion Our study do not suggest that a window of vulnerability...

  7. Factors associated with childhood ocular morbidity and blindness in three ecological regions of Nepal: Nepal pediatric ocular disease study.

    Science.gov (United States)

    Adhikari, Srijana; Shrestha, Mohan Krishna; Adhikari, Kamala; Maharjan, Nhukesh; Shrestha, Ujjowala Devi

    2014-10-23

    Nepal Pediatric Ocular Diseases Study is a three year longitudinal population based study. Here we present the baseline survey report which aims to investigate various risk factors associated with childhood ocular morbidity and blindness in three ecological regions of Nepal. This baseline survey is a population based cross sectional study. The investigation was conducted in a district from each of the following regions: Terai, Hill and Mountain. The Village Development Committees (VDCs) from each district were selected by random sampling. Three Community health workers were given training on vision screening and identification of abnormal ocular signs in children. They conducted a house to house survey in their respected districts examining the children and gathering a standardized set of data variables. Children with abnormal vision or ocular signs were then further examined by pediatric ophthalmologists. A total of 10950 children aged 0-10 years (5403 from Terai, 3204 from the hills, 2343 from the mountains) were enrolled in the study. However 681 (6.2%) were non responders. The male to female ratio was 1.03. The overall prevalence of ocular morbidity was 3.7% (95% CI of 3.4%-4%) and blindness was 0.07% (95% CI of 0.02%-0.12%). Ocular morbidity was more prevalent in the mountain region whereas blindness was more prevalent in the Terai region.Children from the Terai region were more likely to suffer from congenital ocular anomalies compared to the other regions. Children whose mother smoked, drank alcohol, or was illiterate were significantly afflicted with ocular diseases (p Blindness was more prevalent in children who suffered from a systemic illness. Females and under-nourished children were more likely to have ocular morbidity and blindness. It was found that childhood blindness was more prevalent in the Terai region, the undernourished, females and in those with co-morbid systemic illnesses. This study strongly suggests that prevention of childhood

  8. Nosocomial transmission of Ebola virus disease on pediatric and maternity wards: Bombali and Tonkolili, Sierra Leone, 2014.

    Science.gov (United States)

    Dunn, Angela C; Walker, Tiffany A; Redd, John; Sugerman, David; McFadden, Jevon; Singh, Tushar; Jasperse, Joseph; Kamara, Brima Osaio; Sesay, Tom; McAuley, James; Kilmarx, Peter H

    2016-03-01

    In the largest Ebola virus disease (EVD) outbreak in history, nosocomial transmission of EVD increased spread of the disease. We report on 2 instances in Sierra Leone where patients unknowingly infected with EVD were admitted to a general hospital ward (1 pediatric ward and 1 maternity ward), exposing health care workers, caregivers, and other patients to EVD. Both patients died on the general wards, and were later confirmed as being infected with EVD. We initiated contact tracing and assessed risk factors for secondary infections to guide containment recommendations. We reviewed medical records to establish the index patients' symptom onset. Health care workers, patients, and caregivers were interviewed to determine exposures and personal protective equipment (PPE) use. Contacts were monitored daily for EVD symptoms. Those who experienced EVD symptoms were isolated and tested. Eighty-two contacts were identified: 64 health care workers, 7 caregivers, 4 patients, 4 newborns, and 3 children of patients. Seven contacts became symptomatic and tested positive for EVD: 2 health care workers (1 nurse and 1 hospital cleaner), 2 caregivers, 2 newborns, and 1 patient. The infected nurse placed an intravenous catheter in the pediatric index patient with only short gloves PPE and the hospital cleaner cleaned the operating room of the maternity ward index patient wearing short gloves PPE. The maternity ward index patient's caregiver and newborn were exposed to her body fluids. The infected patient and her newborn shared the ward and latrine with the maternity ward index patient. Hospital staff members did not use adequate PPE. Caregivers were not offered PPE. Delayed recognition of EVD and inadequate PPE likely led to exposures and secondary infections. Earlier recognition of EVD and adequate PPE might have reduced direct contact with body fluids. Limiting nonhealth-care worker contact, improving access to PPE, and enhancing screening methods for pregnant women, children

  9. Prevalence and Phylogenetic Analysis of Human Bocaviruses 1-4 in Pediatric Patients with Various Infectious Diseases.

    Directory of Open Access Journals (Sweden)

    Min Zhao

    Full Text Available Viral infections caused by human bocaviruses 1-4 (HBoV1-4 are more complicated than previously believed. A retrospective, large-scale study was undertaken to explore the prevalence of HBoV1-4 in pediatric patients with various infectious diseases and delineate their phylogenetic characteristics.Clinical samples from four specimen types, including 4,941 respiratory, 2,239 cerebrospinal fluid (CSF, 2,619 serum, and 1,121 fecal specimens, collected from pediatric patients with various infectious diseases were screened for HBoV1-4. A 690-nt fragment in each specimen was then amplified and sequenced for phylogenetic analysis. Clinical characteristics of HBoV-positive patients with different specimen types available were evaluated.Approximately 1.2% of patients were confirmed as HBoV-positive, with the highest positive rate in patients with gastrointestinal infection (2.2%, followed by respiratory (1.65%, central nervous system (0.8%, and hematological infections (0.2%. A single genetic lineage of HBoV1 circulated among children over the 8-year period, while a new cluster of HBoV2, via intra-genotype recombination between HBoV2A and HBoV2B, was prevalent. Some patients had HBoV1-positive respiratory and serum specimens or fecal specimens. Several cases became HBoV1-positive following the appearance of respiratory infection, while several cases were positive for HBoV2 only in CSF and serum specimens, rather than respiratory specimens.A single genetic lineage of HBoV1 is speculated as a viral pathogen of respiratory infection and causes both comorbid infection and acute gastroenteritis. Additionally, a new cluster of HBoV2 is prevalent in China, which may infect the host through sites other than the respiratory tract.

  10. High-Dose Methylprednisolone for Veno-Occlusive Disease of the Liver in Pediatric Hematopoietic Stem Cell Transplantation Recipients

    Science.gov (United States)

    Myers, Kasiani C.; Lawrence, Julia; Marsh, Rebecca A.; Davies, Stella M.; Jodele, Sonata

    2017-01-01

    Veno-occlusive disease (VOD) of the liver is a well-recognized serious complication of hematopoietic stem cell transplantation (HSCT), with few successful treatment modalities available for severe disease. Some reports have demonstrated success in adults with the use of high-dose steroid therapy, but experience in the pediatric population is lacking. We retrospectively reviewed HSCT patients treated at our institution since 2003 and identified 15 (2.4%) who developed VOD. Of these, nine (60%) were treated with intravenous high-dose methylprednisolone (500 mg/m2 per dose every 12 hours for six doses). Steroid therapy was initiated at or before first ultrasound evidence of reversal of portal venous flow and before meeting criteria for initiation of defibrotide therapy. Four patients were also treated with defibrotide starting 2 to 5 days after initiation of steroids. Eight of nine patients (88%) with VOD were diagnosed with multiorgan failure. Response to high-dose steroid therapy as defined by decrease in bilirubin by 50% in 10 days from therapy initiation was noted in six of nine patients (67%), occurring within 3 to 6 days of steroid therapy. Two patients died from multiorgan failure due to VOD. Seven survivors of VOD recovered at the median 6 days (range, 5 to 38) from VOD diagnosis. Overall, VOD survival as a group was 78%; however, survival among responders was 100%. No serious toxicities related to high-dose steroid therapy were observed. We conclude that high-dose steroid therapy if initiated early may reverse VOD of the liver in pediatric HSCT patients, abrogating the need for defibrotide therapy with its associated toxicities and regulatory difficulties. PMID:23211838

  11. Approaching a diagnostic point-of-care test for pediatric tuberculosis through evaluation of immune biomarkers across the clinical disease spectrum

    DEFF Research Database (Denmark)

    Jenum, Synne; Dhanasekaran, S; Lodha, Rakesh

    2016-01-01

    The World Health Organization (WHO) calls for an accurate, rapid, and simple point-of-care (POC) test for the diagnosis of pediatric tuberculosis (TB) in order to make progress "Towards Zero Deaths". Whereas the sensitivity of a POC test based on detection of Mycobacterium tuberculosis (MTB...... (CD14, FCGR1A, FPR1, MMP9, RAB24, SEC14L1, and TIMP2) or "downstream" towards a decreased likelihood of TB disease (BLR1, CD3E, CD8A, IL7R, and TGFBR2), suggesting a correlation with MTB-related pathology and high relevance to a future POC test for pediatric TB. A biomarker signature consisting of BPI...

  12. Novel use of an ultrafiltration device as an alternative method for fluid removal in critically ill pediatric patients with cardiac disease: a case series

    Directory of Open Access Journals (Sweden)

    Sujata Chakravarti

    2016-06-01

    Full Text Available Fluid overload (FO is a common complication for pediatric patients in the intensive care unit. When conventional therapy fails, hemodialysis or peritoneal dialysis is classically used for fluid removal. Unfortunately, these therapies are often associated with cardiovascular or respiratory instability. Ultrafiltration, using devices such as the AquadexTM system (Baxter Healthcare, Deerfield, IL, USA, is an effective tool for fluid removal in adult patients with congestive heart failure. As compared to hemodialysis, ultrafiltration can be performed using smaller catheters, and the extracorporeal volume and minimal blood flow rates are lower. In addition, there is no associated abdominal distension as is seen in peritoneal dialysis. Consequently, ultrafiltration may be better tolerated in critically ill pediatric patients. We present three cases of challenging pediatric patients with FO in the setting of congenital heart disease in whom ultrafiltration using the AquadexTM system was successfully utilized for fluid removal while cardiorespiratory stability was maintained.

  13. Causes of Pediatric Cardiomyopathy

    Science.gov (United States)

    ... inherited metabolic or congenital muscle disorder such as Noonan syndrome, Pompe disease, fatty acid oxidation defect or Barth ... where a specific chromosome is deleted or duplicated. Noonan syndrome is the most common form associated with pediatric ...

  14. Development of quality indicators for transition from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers.

    Science.gov (United States)

    Sobota, Amy E; Shah, Nishita; Mack, Jennifer W

    2017-06-01

    Transition from pediatric to adult care is a vulnerable time for young adults with sickle cell disease (SCD); however, improvements in transition are limited by a lack of quality indicators. The purpose of this study was to establish quality indicators for transition in SCD and to determine the optimal timing between the final pediatric visit and the first adult provider visit. We conducted a modified Delphi survey to reach a consensus on which quality indicators are most important for a successful transition. Our expert panel consisted of members of the Sickle Cell Adult Provider Network. In the first round, the participants ranked a list of quality indicators by importance. In the second round, the participants chose their "top 5" quality indicators in terms of importance and also ranked them on feasibility. The response rates for the two rounds were 68 and 96%, respectively. Nine quality indicators were chosen as "top 5" by a majority of respondents, including communication between pediatric and adult providers, timing of first adult visit, patient self-efficacy, quality of life, and trust with their adult provider. Based on the comments from round 1, respondents were also asked for the optimal timing between leaving pediatric care and entering adult care. Most recommended a first adult visit within 2 months of the final pediatric visit. By using these quality indicators chosen by the majority of respondents, we can better develop and evaluate transition programs for young adults with SCD and improve health outcomes for these vulnerable patients. © 2016 Wiley Periodicals, Inc.

  15. [Spanish Society for Pediatric Infectious Diseases guidelines on tuberculosis in pregnant women and neonates (i): Epidemiology and diagnosis. Congenital tuberculosis].

    Science.gov (United States)

    Baquero-Artigao, F; Mellado Peña, M J; Del Rosal Rabes, T; Noguera Julián, A; Goncé Mellgren, A; de la Calle Fernández-Miranda, M; Navarro Gómez, M L

    2015-10-01

    Tuberculosis (TB) screening in pregnancy using tuberculin skin test (TST) is recommended in case of symptoms of TB disease, close contact with a patient with infectious TB, or high risk of developing active disease. The new interferon gamma release assay (IGRA) tests are recommended in BCG-vaccinated pregnant women with positive TST and no known risk factors for TB, and in those immunocompromised, with clinical suspicion of TB but negative TST. TB diagnosis is difficult due to the non-specific symptoms, the increased frequency of extrapulmonary disease, the delay in radiological examinations, and the high rate of tuberculin anergy. Neonatal TB can be acquired in utero (congenital TB), or through airborne transmission after delivery (postnatal TB). Congenital TB is extremely rare and does not cause fetal malformations. It may be evident at birth, although it usually presents after the second week of life. In newborns with no family history of TB, the disease should be considered in cases of miliary pneumonia, hepatosplenomegaly with focal lesions, or lymphocytic meningitis with hypoglycorrhachia, especially in those born to immigrants from high TB-burden countries. TST is usually negative, and IGRAs have lower sensitivity than in older children. However, the yield of acid-fast smear and culture is higher, mostly in congenital TB. Molecular diagnosis techniques enable early diagnosis and detection of drug resistance mutations. There is a substantial risk of disseminated disease and death. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  16. Pediatric radiology

    International Nuclear Information System (INIS)

    Silverman, F.N.

    1982-01-01

    A literature review with 186 references of diagnostic pediatric radiology, a speciality restricted to an age group rather than to an organ system or technique of examination, is presented. In the present chapter topics follow the basic organ system divisions with discussions of special techniques within these divisions. The diagnosis of congenital malformations, infectious diseases and neoplasms are a few of the topics discussed for the head and neck region, the vertebrae, the cardiovascular system, the respiratory system, the gastrointestinal tract, the urinary tract, and the skeleton

  17. Pediatric neuroimaging

    International Nuclear Information System (INIS)

    Tidwell, A.S.; Solano, M.; Schelling, S.H.

    1994-01-01

    In this article, some of the common and not-so-common neuropediatric disorders were discussed. As in the full-grown animal, abnormalities of the CNS in the pediatric animal patient may be classified according to the type of insult present (eg, malformation, injury, neoplasia, inflammation, or degeneration). To recognize the imaging manifestations of such disorders, an appreciation of normal anatomy, the pathological response of nervous system tissue to insult, and the principles of image interpretation is required. These fundamentals may then be applied to any CNS disease, regardless of frequency and to any animal patient, regardless of age

  18. Barriers and facilitators to successful transition from pediatric to adult inflammatory bowel disease care from the perspectives of providers

    Science.gov (United States)

    Paine, Christine Weirich; Stollon, Natalie B.; Lucas, Matthew S.; Brumley, Lauren D.; Poole, Erika S.; Peyton, Tamara; Grant, Anne W.; Jan, Sophia; Trachtenberg, Symme; Zander, Miriam; Mamula, Petar; Bonafide, Christopher P.; Schwartz, Lisa A.

    2014-01-01

    Background For adolescents and young adults (AYA) with inflammatory bowel disease (IBD), the transition from pediatric to adult care is often challenging and associated with gaps in care. Our study objectives were to (1) identify outcomes for evaluating transition success and (2) elicit the major barriers and facilitators of successful transition. Methods We interviewed pediatric and adult IBD providers from across the United States with experience caring for AYAs with IBD until thematic saturation was reached after 12 interviews. We elicited the participants' backgrounds, examples of successful and unsuccessful transition of AYAs for whom they cared, and recommendations for improving transition using the Social-ecological Model of Adolescent and Young Adult Readiness to Transition framework. We coded interview transcripts using the constant comparative method and identified major themes. Results Participants reported evaluating transition success and failure using healthcare utilization outcomes (e.g. maintaining continuity with adult providers), health outcomes (e.g. stable symptoms), and quality of life outcomes (e.g. attending school). The patients' level of developmental maturity (i.e. ownership of care) was the most prominent determinant of transition outcomes. The style of parental involvement (i.e. helicopter parent vs. optimally-involved parent) also influenced outcomes as well as the degree of support by providers (e.g. care coordination). Conclusion IBD transition success is influenced by a complex interplay of patient developmental maturity, parenting style, and provider support. Multidisciplinary IBD care teams should aim to optimize these factors for each patient to increase the likelihood of a smooth transfer to adult care. PMID:25137417

  19. Attention and memory impairments in pediatric patients with cystic fibrosis and inflammatory bowel disease in comparison to healthy controls.

    Science.gov (United States)

    Piasecki, Bartosz; Stanisławska-Kubiak, Maia; Strzelecki, Wojciech; Mojs, Ewa

    2017-10-01

    The main aim of the study was to analyze and compare attention and memory performance in pediatric patients with cystic fibrosis (CF), inflammatory bowel disease (IBD) and in healthy controls. 28 patients with CF, 30 patients with IBD and 30 healthy subjects took part in the study (all in age range of 7-17). All subjects were in intellectual norm. To analyze the functioning of attention, the d2 Test of Attention by Brickenkamp (d2 test) was applied. Memory performance was assessed using the Benton Visual Retention Test (BVRT) and the Trial of 10 words. The CF and IBD groups committed significantly more errors in the d2 test than the healthy controls. The CF group also had significantly higher fluctuation rates and received significantly lower scores in overall concentration performance than the control group. Patients with CF made more mistakes and had fewer correct memory projections in BVRT than the healthy controls. Patients with IBD committed significantly more errors in BVRT than the control group. Patients with CF and IBD also got significantly lower scores in the Trial of 10 words than the control group. Pediatric patients with CF and IBD performed more poorly than the healthy controls on attention and memory tests. More distinct cognitive impairments were observed in the CF group. Further research is needed to find the underlying mechanisms and clinical and/or functional significance of observed cognitive deficits. © American Federation for Medical Research (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  20. Parents' knowledge and attitude regarding their child's cancer and effectiveness of initial disease counseling in pediatric oncology patients

    Directory of Open Access Journals (Sweden)

    Manjusha Nair

    2017-01-01

    Full Text Available Objective: To examine parent's knowledge, attitude and psychosocial response regarding their child's cancer and treatment after initial disease counseling by doctor. Materials and Methods: Structured questionnaire based study of 43 mothers of newly diagnosed pediatric cancer patients undergoing treatment in pediatric oncology division. Mothers received initial counseling regarding their child's cancer and treatment from the doctor. Questionnaire was administered 2-6 months after initial counseling and mothers self-reported their responses. Results: 83% mothers had school level education only and 84% belonged to lower and middle socio-economic status. More than 80% mothers knew the name of their child's cancer, type of treatment received by child and approximate duration of treatment. 93% knew regarding painful procedures and 84% mothers reported knowledge about chemotherapy side effects. Hope of cure and satisfaction with treatment were reported by 90% mothers. 81% mothers reported high levels of anxiety and 66% worried regarding painful procedures. As high as 60% of parents were afraid to send their child outside to play and 40% were afraid to send their child to school. 40% mothers wanted more information regarding child's higher education, married life & fertility. On statistical analysis, mother's age, educational status or family background did not influence their knowledge and attitude. Conclusion: Relevant information about child's cancer and treatment can be imparted effectively even to mothers with school level education. This knowledge helps to instill hopeful attitude, confidence and satisfaction in parents. Anxiety and fear related to cancer persists in mothers even after the initial stress period is over. Pain related to injections and procedures is a major concern in parents. Involvement of counselor in the treating team is desirable to overcome these problems.

  1. Association of secondhand smoke exposure with pediatric invasive bacterial disease and bacterial carriage: a systematic review and meta-analysis.

    Directory of Open Access Journals (Sweden)

    Chien-Chang Lee

    2010-12-01

    Full Text Available A number of epidemiologic studies have observed an association between secondhand smoke (SHS exposure and pediatric invasive bacterial disease (IBD but the evidence has not been systematically reviewed. We carried out a systematic review and meta-analysis of SHS exposure and two outcomes, IBD and pharyngeal carriage of bacteria, for Neisseria meningitidis (N. meningitidis, Haemophilus influenzae type B (Hib, and Streptococcus pneumoniae (S. pneumoniae.Two independent reviewers searched Medline, EMBASE, and selected other databases, and screened articles for inclusion and exclusion criteria. We identified 30 case-control studies on SHS and IBD, and 12 cross-sectional studies on SHS and bacterial carriage. Weighted summary odd ratios (ORs were calculated for each outcome and for studies with specific design and quality characteristics. Tests for heterogeneity and publication bias were performed. Compared with those unexposed to SHS, summary OR for SHS exposure was 2.02 (95% confidence interval [CI] 1.52-2.69 for invasive meningococcal disease, 1.21 (95% CI 0.69-2.14 for invasive pneumococcal disease, and 1.22 (95% CI 0.93-1.62 for invasive Hib disease. For pharyngeal carriage, summary OR was 1.68 (95% CI, 1.19-2.36 for N. meningitidis, 1.66 (95% CI 1.33-2.07 for S. pneumoniae, and 0.96 (95% CI 0.48-1.95 for Hib. The association between SHS exposure and invasive meningococcal and Hib diseases was consistent regardless of outcome definitions, age groups, study designs, and publication year. The effect estimates were larger in studies among children younger than 6 years of age for all three IBDs, and in studies with the more rigorous laboratory-confirmed diagnosis for invasive meningococcal disease (summary OR 3.24; 95% CI 1.72-6.13.When considered together with evidence from direct smoking and biological mechanisms, our systematic review and meta-analysis indicates that SHS exposure may be associated with invasive meningococcal disease. The

  2. Longitudinal patterns of predominant asthma disease activity in pediatric patients enrolled in an asthma-specific disease management program.

    Science.gov (United States)

    Scott, Lyne; Nichols, Breck; Choi Kwong, Kenny Yat; Morphew, Tricia; Jones, Craig A

    2008-08-01

    To determine if patterns of predominant asthma disease activity are more closely related than baseline asthma severity to measures of morbidity (acute asthma attack, emergency room visit/hospitalization, missed school days, and/or steroid burst). Retrospective analysis was performed for inner-city Los Angeles asthmatic children (3 to 18 years of age) during their first year of enrollment in an asthma-specific disease management program. All measures of morbidity were more closely related to patterns of predominant disease activity than baseline severity. We conclude that patterns of predominant disease activity are a more significant predictor of asthma morbidity than is baseline severity.

  3. Biphosphonate-induced radiographic changes in two pediatric patients with rheumatic diseases

    International Nuclear Information System (INIS)

    Fernandes, Joao L.; Rocha, Arthemizio L.; Viana, Sergio L.; Ribeiro, Maria C.; Castro, Luis C.

    2004-01-01

    Biphosphonates are now being used experimentally in children to increase bone mass, but their long-term effects remain an issue of concern. We report two cases of biphosphonate-induced radiographic changes in children with rheumatic diseases. Our experience supports the view that clinical improvement and radiographic findings after biphosphonate therapy are related to increased bone mineral density, without effects on the inflammatory process itself. Biphosphonates seem to act in rheumatic diseases by reducing bone turnover instead of improving disease activity. (orig.)

  4. Remission of aplastic anemia induced by treatment for Graves disease in a pediatric patient.

    Science.gov (United States)

    Das, Prabodh Kumar; Wherrett, Diane; Dror, Yigal

    2007-08-01

    Aplastic anemia (AA) is mediated by T-cell autoimmunity in the majority of cases; it is rare and mostly idiopathic in children. We describe a child, who developed AA following Graves' disease which could not be attributed to antithyroid drugs. We hypothesized that both diseases were caused by similar autoimmune process. We monitored the blood counts and did not administer any conventional treatment for AA assuming that the existing anti- hematopoietic stem cell humoral and cellular immunity might subside with induction of remission of Grave's disease. The child went into complete remission with the treatment of the Graves' disease.

  5. The Breathmobile Program: structure, implementation, and evolution of a large-scale, urban, pediatric asthma disease management program.

    Science.gov (United States)

    Jones, Craig A; Clement, Loran T; Hanley-Lopez, Jean; Morphew, Tricia; Kwong, Kenny Yat Choi; Lifson, Francene; Opas, Lawrence; Guterman, Jeffrey J

    2005-08-01

    Despite more than a decade of education and research-oriented intervention programs, inner city children with asthma continue to engage in episodic "rescue" patterns of healthcare and experience a disproportionate level of morbidity. The aim of this study was to establish and evaluate a sustainable community-wide pediatric asthma disease management program designed to shift inner city children in Los Angeles from acute episodic care to regular preventive care in accordance with national standards. In 1995 the Southern California Chapter of the Asthma and Allergy Foundation of America (AAFA), the Los Angeles County Department of Health Services (LAC DHS), and the Los Angeles Unified School District (LAUSD) established an agreement to initiate and sustain the Breathmobile Program. This program includes automated case identification, mobile school-based clinics, and highly structured clinical encounters supported by an advanced information technology solution. Interdisciplinary teams of asthma care specialists provide regular and ongoing care to children at school and county clinic sites over a wide geographic area of urban Los Angeles. Each team operates in a specially equipped mobile clinic (Breathmobile), efficiently moving a structured healthcare process to school and county clinic sites with large numbers of children. Demographic, clinical, and participation data is tracked carefully in an electronic medical record system. Program operations, clinical oversight, and patient tracking are centralized at a care coordination center. Clinical operations and methods have been replicated in fixed specialty clinic sites at the Los Angeles County + University of Southern California Medical Center. Clinical and process measures are regularly evaluated to assure quality, plan iterative improvement, and support evidence-based care. Four Breathmobiles deliver ongoing care at more than 90 school sites. The program has engaged over five thousand patients and their families in a

  6. Correlation between antibodies and histology in celiac disease: Incidence of celiac disease is higher than expected in the pediatric population

    Czech Academy of Sciences Publication Activity Database

    Makovický, P.; Rimárová, K.; Boor, A.; Makovický, P.; Vodička, Pavel; Samasca, G.; Kruzliak, P.

    2013-01-01

    Roč. 8, č. 4 (2013), s. 1079-1083 ISSN 1791-2997 R&D Projects: GA MZd(CZ) NT13424 Institutional support: RVO:68378041 Keywords : celiac disease * autoimmune disease * enterobiopsy Subject RIV: EB - Genetics ; Molecular Biology Impact factor: 1.484, year: 2013

  7. Behavioral and Pharmacological Adherence in Pediatric Sickle Cell Disease: Parent-Child Agreement and Family Factors Associated With Adherence.

    Science.gov (United States)

    Klitzman, Page H; Carmody, Julia K; Belkin, Mary H; Janicke, David M

    2018-01-01

    This study aimed to evaluate agreement between children and parents on a measure of behavioral and pharmacological adherence in children with sickle cell disease (SCD), and the associations among family factors (i.e., problem-solving skills, routines, communication) and adherence behaviors. In all, 85 children (aged 8-18 years) with SCD and their parents completed questionnaires assessing individual and family factors. Overall parent-child agreement on an adherence measure was poor, particularly for boys and older children. Greater use of child routines was associated with better overall child-reported adherence. Open family communication was associated with higher overall parent-reported adherence. While further research is needed before definitive conclusions can be drawn, results suggest the need to assess child adherence behaviors via both child and parent reports. Findings also suggest that more daily family routines and open family communication may be protective factors for better disease management. © The Author 2017. Published by Oxford University Press on behalf of the Society of Pediatric Psychology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com

  8. Sustained pediatric antimicrobial stewardship program with consultation to infectious diseases reduced carbapenem resistance and infection-related mortality.

    Science.gov (United States)

    Horikoshi, Yuho; Suwa, Junichi; Higuchi, Hiroshi; Kaneko, Tetsuji; Furuichi, Mihoko; Aizawa, Yuta; Fukuoka, Kahoru; Okazaki, Kaoru; Ito, Kenta; Shoji, Takayo

    2017-11-01

    The impact of pediatric antimicrobial stewardship programs (ASP) on antimicrobial resistance (AMR) remains largely unknown. This study aimed to evaluate the AMR for carbapenem of Gram-negative bacilli (GNB) and carbapenem use with infectious diseases consultation after the implementation of an ASP. This quasi-experimental study was conducted at Tokyo Metropolitan Children's Medical Center in Japan. The pre- and post-intervention periods were April 2010 to September 2011 and October 2011 to March 2017, respectively. The pre-intervention phase consisted of consultations with the infectious diseases service alone. The ASP was implemented during the post-intervention phase. The carbapenem resistance rates of GNB were calculated. The correlation between carbapenem resistance rates and carbapenem day of therapy (DOT) was examined. The outcome metrics were compared by average length of hospitalization, all-cause mortality, and infection-related mortality. A positive correlation was observed between the carbapenem resistance rate in Pseudomonas aeruginosa and DOT (0.76, p=0.04). The carbapenem resistance rate in P. aeruginosa (pcarbapenem use and resistance in P. aeruginosa, leading to favorable outcomes in terms of length of hospitalization and infection-related mortality. Copyright © 2017 The Author(s). Published by Elsevier Ltd.. All rights reserved.

  9. Application of convolutional artificial neural networks to echocardiograms for differentiating congenital heart diseases in a pediatric population

    Science.gov (United States)

    Perrin, Douglas P.; Bueno, Alejandra; Rodriguez, Andrea; Marx, Gerald R.; del Nido, Pedro J.

    2017-03-01

    In this paper we describe a pilot study, where machine learning methods are used to differentiate between congenital heart diseases. Our approach was to apply convolutional neural networks (CNNs) to echocardiographic images from five different pediatric populations: normal, coarctation of the aorta (CoA), hypoplastic left heart syndrome (HLHS), transposition of the great arteries (TGA), and single ventricle (SV). We used a single network topology that was trained in a pairwise fashion in order to evaluate the potential to differentiate between patient populations. In total we used 59,151 echo frames drawn from 1,666 clinical sequences. Approximately 80% of the data was used for training, and the remainder for validation. Data was split at sequence boundaries to avoid having related images in the training and validation sets. While training was done with echo images/frames, evaluation was performed for both single frame discrimination as well as sequence discrimination (by majority voting). In total 10 networks were generated and evaluated. Unlike other domains where this network topology has been used, in ultrasound there is low visual variation between classes. This work shows the potential for CNNs to be applied to this low-variation domain of medical imaging for disease discrimination.

  10. Oxidative Stress during Ovarian Torsion in Pediatric and Adolescent Patients: Changing The Perspective of The Disease

    Directory of Open Access Journals (Sweden)

    Antonio Simone Laganà

    2016-12-01

    Full Text Available Among the different causes of gynecological acute pelvic pain, ovarian torsion represents a surgical emergency. It is a rare case in the pediatric/adolescent aged group that must be included in the differential diagnosis of any girl with abdominal pain or pelvic/abdominal mass. Current recommendations suggest that laparoscopic detorsion should be performed in order to preserve the integrity of the ovaries and fertility, although oophoropexy may be considered in case of severe necrosis. Nevertheless, maintaining the circulation of the ovary after detorsion deteriorates the tissue injury and leads to a pathologic process called ischaemia/reperfusion (I/R injury, which is characterized by oxidative stress. During the detorsion process, an excess amount of molecular oxygen is supplied to the tissues, and reactive species of oxygen (ROS such as superoxide radical (O2 -, hydrogen peroxide (H2O2, hydroxyl radical (OH•, as well as reactive nitrogen species (RNS are produced in excess. ROS, RNS and their toxic products cause DNA damage and lipid peroxidation in the cellular and mitochondrial membranes, leading to cell death. In spite of attention on this topic, currently there is no shared and clear evidence about the use of anti-inflammatory and antioxidant agents to prevent I/R damage after laparoscopic ovarian detorsion. Considering this element, future research should aim to develop shared protocols for the clinical use (route of application, dosage and time of application of antioxidants after laparoscopic management of this condition.

  11. Serum IP-10 is useful for identifying renal and overall disease activity in pediatric systemic lupus erythematosus.

    Science.gov (United States)

    Zhang, Chen-Xing; Cai, Li; Shao, Kang; Wu, Jing; Zhou, Wei; Cao, Lan-Fang; Chen, Tong-Xin

    2018-05-01

    Traditional serological biomarkers often fail to assess systemic lupus erythematosus (SLE) disease activity and discriminate lupus nephritis (LN). The aim of this study was to identify novel markers for evaluating renal and overall disease activity in Chinese patients with pediatric systemic lupus erythematosus (pSLE). The study included 46 patients with pSLE (35 girls, 11 boys; average age 13.3 ± 2.6 years) and 31 matched healthy controls (22 girls, 9 boys; average age 12.3 ± 2.4 years). The SLE Disease Activity Index (SLEDAI) and renal SLEDAI were used to assess disease activity. Nine different soluble mediators in plasma, including tumor necrosis factor alpha (TNF-α), platelet-derived growth factor-BB (PDGF-BB), interferon (IFN) gamma inducible protein 10 (IP-10), interleukin (IL)-1β, IFN-γ, IL-17A, IL-2, Fas and Fas ligand, were measured by Luminex assay and compared between patients with active and inactive pSLE as well as between patients with pSLE with active and inactive renal disease. Receiver operating characteristic curve analysis was used to measure the discrimination accuracy. Of the 46 patients with pSLE, 30 (65.2%) had LN. These patients had significantly elevated levels of serum TNF-α, PDGF-BB, IP-10 and Fas. The serum levels of IP-10 were also significantly higher in patients with active pSLE. We found that IP-10 was also more sensitive and specific than conventional laboratory parameters, including anti-double-stranded DNA and complement components C3 and C4, for distinguishing active lupus from quiescent lupus. The serum level of IP-10 was also significantly increased in children with pSLE with active renal disease relative to those with inactive renal disease. There was also a positive correlation between serum IP-10 levels and renal SLEDAI scores as well as with 24 h urine protein. Serum IP-10 is useful for identifying renal and overall disease activity in children with pSLE.

  12. Preface [to: Practical Pediatric Dermatology: Controversies in Diagnosis and Treatment

    NARCIS (Netherlands)

    A.P. Oranje (Arnold); N. Al-Mutairi (Nawaf); T. Shwayder (Tor)

    2016-01-01

    markdownabstractPediatric dermatology is a young field that combines dermatologic and pediatric skills and expertises. Knowledge of dermatology and pediatrics is necessary for optimal care of children with skin diseases. A multidisciplinary approach in which there is cooperation between

  13. F-calprotectin and Blood Markers Correlate to Quality of Life in Pediatric Inflammatory Bowel Disease

    DEFF Research Database (Denmark)

    Carlsen, Katrine; Jakobsen, Christian; Kallemose, Thomas

    2017-01-01

    OBJECTIVES: Our aim was to investigate predictors of health-related quality of life (HRQoL) with respect to changes in disease parameters over time in children with inflammatory bowel disease. METHODS: This was a prospective longitudinal study examining the association between HRQoL (IMPACT III...

  14. Risk factors and treatment of pediatric chronic diseases : Type 1 diabetes, asthma and allergy

    NARCIS (Netherlands)

    Ahmadizar, F.

    2016-01-01

    Chronic diseases such as type 1 diabetes (T1DM) and asthma are leading causes of morbidity and mortality worldwide. The increase in the number of children with chronic diseases is a major concern. Early detection through improved screening and diagnostic tests, better diagnosis based on updated

  15. Contrast-enhanced magnetic resonance venography in pediatric patients with chronic kidney disease: initial experience with ferumoxytol

    International Nuclear Information System (INIS)

    Luhar, Aarti; Khan, Sarah; Ghahremani, Shahnaz; Griggs, Rachel; Hall, Theodore R.; Finn, J.P.; Zaritsky, Joshua; Salusky, Isidro

    2016-01-01

    Ferumoxytol is an ultra-small superparamagnetic iron oxide (USPIO) particle that is FDA-approved for parenteral treatment of iron deficiency anemia in adults with chronic kidney disease. Because of the association between gadolinium-based contrast agents and nephrogenic systemic fibrosis in patients with severe chronic kidney disease, we sought to evaluate the diagnostic role of ferumoxytol-enhanced MR venography in children with chronic kidney disease. Twenty children underwent 22 high-resolution ferumoxytol-enhanced MR venography examinations at 3.0 T. High-resolution 3-D contrast-enhanced imaging was performed at a minimum of 3 time points following injection of ferumoxytol at a total dose of 4 mg/kg. Two blinded pediatric radiologists independently scored six named veins on ferumoxytol-enhanced MR venography examinations according to a three-point subjective score, where a score ≥2 was considered diagnostic. Additionally, all relevant venous structures in the included field of view were analyzed for occlusive or non-occlusive thrombosis, compression and presence of collaterals. All patients underwent ferumoxytol-enhanced MR venography successfully and without adverse event. The overall scores of the reviewing radiologists for all venous structures were 2.7-2.9. In all cases, the reviewers were confident basing their diagnoses on the ferumoxytol-enhanced MR venography findings. In 12 of 22 examinations, findings on follow-up imaging or invasive procedures were available to correlate with the findings on ferumoxytol-enhanced MR venography. There was complete concordance between the findings from follow-up imaging and invasive procedures with findings from ferumoxytol-enhanced MR venography. Ferumoxytol holds promise as a powerful alternative to gadolinium-based contrast agents for reliable, high-resolution MR venography in children with chronic kidney disease. (orig.)

  16. [Spanish Society for Pediatric Infectious Diseases guidelines on tuberculosis in pregnant women and neonates (ii): Prophylaxis and treatment].

    Science.gov (United States)

    Baquero-Artigao, F; Mellado Peña, M J; del Rosal Rabes, T; Noguera Julián, A; Goncé Mellgren, A; de la Calle Fernández-Miranda, M; Navarro Gómez, M L

    2015-10-01

    In pregnant women who have been exposed to tuberculosis (TB), primary isoniazid prophylaxis is only recommended in cases of immunosuppression, chronic medical conditions or obstetric risk factors, and close and sustained contact with a patient with infectious TB. Isoniazid prophylaxis for latent tuberculosis infection (LTBI) is recommended in women who have close contact with an infectious TB patient or have risk factors for progression to active disease. Otherwise, it should be delayed until at least three weeks after delivery. Treatment of TB disease during pregnancy is the same as for the general adult population. Infants born to mothers with disseminated or extrapulmonary TB in pregnancy, with active TB at delivery, or with postnatal exposure to TB, should undergo a complete diagnostic evaluation. Primary isoniazid prophylaxis for at least 12 weeks is recommended for those with negative diagnostic tests and no evidence of disease. Repeated negative diagnostic tests are mandatory before interrupting prophylaxis. Isoniazid for 9 months is recommended in LTBI. Treatment of neonatal TB disease is similar to that of older children, but should be maintained for at least 9 months. Respiratory isolation is recommended in congenital TB, and in postnatal TB with positive gastric or bronchial aspirate acid-fast smears. Separation of mother and infant is only necessary when the mother has received treatment for less than 2 weeks, is sputum smear-positive, or has drug-resistant TB. Breastfeeding is not contraindicated, and in case of mother-infant separation expressed breast milk feeding is recommended. Copyright © 2014 Asociación Española de Pediatría. Published by Elsevier España, S.L.U. All rights reserved.

  17. Risk of Pediatric Celiac Disease According to HLA Haplotype and Country

    Science.gov (United States)

    Liu, Edwin; Lee, Hye-Seung; Aronsson, Carin A.; Hagopian, William A.; Koletzko, Sibylle; Rewers, Marian J.; Eisenbarth, George S.; Bingley, Polly J.; Bonifacio, Ezio; Simell, Ville; Agardh, Daniel

    2014-01-01

    BACKGROUND The presence of HLA haplotype DR3–DQ2 or DR4–DQ8 is associated with an increased risk of celiac disease. In addition, nearly all children with celiac disease have serum antibodies against tissue transglutaminase (tTG). METHODS We studied 6403 children with HLA haplotype DR3–DQ2 or DR4–DQ8 prospectively from birth in the United States, Finland, Germany, and Sweden. The primary end point was the development of celiac disease autoimmunity, which was defined as the presence of tTG antibodies on two consecutive tests at least 3 months apart. The secondary end point was the development of celiac disease, which was defined for the purpose of this study as either a diagnosis on biopsy or persistently high levels of tTG antibodies. RESULTS The median follow-up was 60 months (interquartile range, 46 to 77). Celiac disease autoimmunity developed in 786 children (12%). Of the 350 children who underwent biopsy, 291 had confirmed celiac disease; an additional 21 children who did not undergo biopsy had persistently high levels of tTG antibodies. The risks of celiac disease autoimmunity and celiac disease by the age of 5 years were 11% and 3%, respectively, among children with a single DR3–DQ2 haplotype, and 26% and 11%, respectively, among those with two copies (DR3–DQ2 homozygosity). In the adjusted model, the hazard ratios for celiac disease autoimmunity were 2.09 (95% confidence interval [CI], 1.70 to 2.56) among heterozygotes and 5.70 (95% CI, 4.66 to 6.97) among homozygotes, as compared with children who had the lowest-risk genotypes (DR4–DQ8 heterozygotes or homozygotes). Residence in Sweden was also independently associated with an increased risk of celiac disease autoimmunity (hazard ratio, 1.90; 95% CI, 1.61 to 2.25). CONCLUSIONS Children with the HLA haplotype DR3–DQ2, especially homozygotes, were found to be at high risk for celiac disease autoimmunity and celiac disease early in childhood. The higher risk in Sweden than in other countries

  18. Early and late endocrine effects in pediatric central nervous system diseases.

    Science.gov (United States)

    Aslan, Ivy R; Cheung, Clement C

    2014-01-01

    Endocrinopathies are frequently linked to central nervous system disease, both as early effects prior to the disease diagnosis and/or late effects after the disease has been treated. In particular, tumors and infiltrative diseases of the brain and pituitary, such as craniopharyngioma, optic pathway and hypothalamic gliomas, intracranial germ cell tumor, and Langerhans cell histiocytosis, can present with abnormal endocrine manifestations that precede the development of neurological symptoms. Early endocrine effects include diabetes i