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Sample records for left-lung hypoplasia left

  1. Unusual congenital pulmonary anomaly with presumed left lung hypoplasia in a young dog.

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    Lee, C M; Kim, J H; Kang, M H; Eom, K D; Park, H M

    2014-05-01

    A seven-month-old, entire, male miniature schnauzer dog was referred with acute vomiting, inappetence and depression primarily as a result of a gastric foreign body (pine cones). During investigations, thoracic radiographs revealed increased volume of the right lung lobes, deviated cardiomediastinal structures and elevation of the heart from the sternum. Thoracic computed tomography revealed left cranial lung lobe hypoplasia and extension of the right cranial lung parenchyma across the midline to the left hemithorax. Branches of the right pulmonary vessels and bronchi also crossed the midline and extended to the left caudal lung lobe. These findings suggested that the right and left lungs were fused. In humans this finding is consistent with horseshoe lung, which is an uncommon congenital malformation. To the authors' knowledge, this case represents the first report of such a pulmonary anomaly in a dog. © 2014 British Small Animal Veterinary Association.

  2. Isolated left-sided pulmonary artery agenesis with left lung hypoplasia: A report of two cases

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    V Govindaraj

    2017-01-01

    Full Text Available Unilateral absence of pulmonary artery or pulmonary artery agenesis (UAPA is a rare congenital malformation that can present as an isolated lesion or in association with other cardiac anomalies. Though congenital, presentation in adults are also reported. Most common presentation in adults is of exercise intolerance. The developing lung on the affected side is hypoplastic. Diagnosis of UAPA is established by imaging methods like CT and MRI . There is no specific treatment for this condition. Treatment depends on patients symptomatology, presence of pulmonary hypertension and collateral circulation. Presence of pulmonary hypertension carries a bad prognosis. We present two adult patients with isolated left sided unilateral pulmonary artery agenesis with ipsilateral lung hypoplasia. The diagnosis was confirmed by CT chest and perfusion scan.

  3. A case of hypoplasia of left lung with very rare associations with congenital absence of left pulmonary artery and right-sided aortic arch

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    Trilok Chand

    2017-01-01

    Full Text Available The absence of one of the pulmonary artery with associated hypoplasia of lung and great vessel abnormality is a rare finding. The incidence of this rare congenital abnormality is around 1 in 200,000 live birth. The absence of the left side pulmonary artery is again uncommon, and associated cardiac malformations are usually tetralogy of fallot or septal defects rather than an aortic arch defect. Our case is a unique case in It’s associated congenital anomalies. He was presented with recurrent pneumothorax and hemoptysis, and on thorough workup, he was diagnosed to have an absence of left pulmonary artery with hypoplasia of the left lung and associated right-sided aortic arch. The patient’s family has declined the surgical option, and he was managed conservatively and kept in close follow-up.

  4. Bronchial compression by an enlarged left atrium in infants; a cause of hypovascularity of the left lung

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    Corr, L.; Hallidie-Smith, K.A.; McCarthy, P.A.; Lavender, J.P.

    1988-09-01

    In three infants seen recently at our institution we noted signs of compression of the left main bronchus associated with enlarged left atria. None of our cases demonstrated the more usual signs of hyperinflation which are a hyperlucent lung field, depressed hemidiaphragm and mediastinal shift away from the affected side. In addition, hypoperfusion of the left lung was noted in each case. We believe that bronchial compression due to an enlarged left atrium, with consequent hypoxic vasoconstriction is a clinically significant entity, which is not well described and may be unappreciated in infants in whom the typical signs of hyperinflation are absent.

  5. Congenital bicuspid stenosis with left ventricular hypoplasia in a kitten.

    Science.gov (United States)

    van Nie, C J; van Messel, M A; Straatman, T J

    1980-01-15

    Congenital bicuspid stenosis with left ventricular hypoplasia was diagnosed in a kitten. Clinical weakness, dyspnoea and marked cardiomegaly (X rays) were related to postmortem findings. The cardiomegaly had resulted from an enlargement of the left auricular appendage. It is supposed the cardiomegaly developed after the closing of the foramen ovale.

  6. Lateral chest radiographic findings in lobar collapse of the left lung : the distance between both upper lobe bronchi

    International Nuclear Information System (INIS)

    Chin, G. H.; Sung, D. W.; Yoon, Y.; Kim, H. C.

    1996-01-01

    To evaluate the distance between both upper love bronchi on lateral radiographs and its change in left upper or lower lobe collapse. 144 true lateral radiographs were analyzed on which both upper lobe bronchi were clearly identified. They included 116 normal cases, 11 cases of left upper lobe collapse, 13 of left lower lobe collapse, and 4 cases of left lower lobe lobectomy. Line A was drawn parallel to the vertebral end plate through the upper margin of the lift upper lobe bronchus. Line B was drawn parallel to line A through the upper margin of the right upper love bronchus. The shortest distance between line A and line B was measured as the distance between both upper lobe bronchi. In normal cases, the mean value of the distance was 2.19 cm ± S.D. 0.37 cm on right and on right and 2.16 cm ± S.D. 0.40 cm on left lateral radiographs ; these results were not significantly different(P=0.79). In cases of collapse, the mean value of the distance was 0.43 cm ± S.D. 0.99 cm in upper lobe collapse and 3.56 cm ± S.D. 0.72 cm in lower lobe collapse, results which were significantly different from those of normal cases(p<0.01). In eight cases(73%) of left upper lobe collapse, the distance was less than 1 cm and in 10 cases(77%) of left lower lobe collapse, the distance was more than 3 cm. The distance between both upper lobe bronchi varies markedly in case of lobar collapse. A distance of less than 1 cm suggests collapse of the left upper lobe and a distance more than 3 cm suggests collapse of the left lower lobe

  7. Radiological sign of the hilus from the left lung large than the right one in valvular pulmonic stenosis. Presentation of 30 cases

    International Nuclear Information System (INIS)

    Medeiros Sobrinho, J.H. de; Luiz, C.; Perozo, D.; Cedeno, R.; Silva, M.V.D. da; Fontes, V.F.

    1982-01-01

    Thirty cases of valvular pulmonic stenosis are reported 19 with isolated valvular pulmonic stenosis, ten associated with atrial septal defect and one associated with ventricular septal defect in which the roentgenologic sign of the left hilus was larger than the right one in the frontal and oblique ROP and LOP positions. The presence of the roentgenologic sign was positive in 23 of the 30 cases (76 %). It was found in all three positions in 12 cases, only in the ROP and LOP in 11 cases, and absent in seven cases, almost all in children. As for what causes the dilatation in left branch of the pulmonary artery, we believe, based on the opinion of some authors and on cineangiocardiography that it results from the anatomical disposition or the pulmonary trunk and its branches so that blood flow through the stenotic valve is predominantly directed to the left branch. (author)

  8. EXTRAPULMONARY SEQUESTRATION WITH PULMONARY HYPOPLASIA AND MULTICYSTIC RENAL DYSPLASIA : A RARE CASE REPORT

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    Anita

    2015-05-01

    Full Text Available Pulmonary sequestration is a rare anomaly which consists of the presence of pulmonary tissue that is not attached to the rest of the lung and does not communicate with the trachea. [1] It could be intrapulmonary or extrapulmonary. We report a case of extrapulmonary sequestration with brief review of literature. A 22 years old primigravida underwent an ultrasonography at 24 weeks of gestation which revealed a single live fetus with bilateral pleural effusion, fetal hydrops and the fetal thorax showed mediastinal shift to the right. A hyperechoic mass was present in the left thoracic cavity with a systemic blood supply to it. Termination of pregnancy was advised as the findings were incompatible with life and the fetus autopsied. Significant gross findings were a hypoplastic left lung, a grey - white spongy mass adjacent to the left lung but no t attached to it and present outside the pleural cavity which derived its blood supply via a branch from the thoracic aorta and caused a shift in the mediastinal structures to the right. Both kidneys showed multiple cystic spaces. Microscopically the mass showed multiple cystically dilated alveolar spaces and ducts lined by cuboidal to tall columnar epithelium, the left lung showed features of pulmonary hypoplasia and the microscopic findings in both the kidneys were suggestive of multicystic renal dysplasi a. Hence, it was reported as a case of left sided extrapulmonary sequestration with hypoplastic left lung and bilateral renal cystic dysplasia.

  9. The Infant with Aortic Arch Hypoplasia and Small Left Heart Structures: Echocardiographic Indices of Mitral and Aortic Hypoplasia Predicting Successful Biventricular Repair.

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    Plymale, Jennifer M; Frommelt, Peter C; Nugent, Melodee; Simpson, Pippa; Tweddell, James S; Shillingford, Amanda J

    2017-08-01

    In infants with aortic arch hypoplasia and small left-sided cardiac structures, successful biventricular repair is dependent on the adequacy of the left-sided structures. Defining accurate thresholds of echocardiographic indices predictive of successful biventricular repair is paramount to achieving optimal outcomes. We sought to identify pre-operative echocardiographic indices of left heart size that predict intervention-free survival in infants with small left heart structures undergoing primary aortic arch repair to establish biventricular circulation (BVC). Infants ≤2 months undergoing aortic arch repair from 1999 to 2010 with aortic and/or mitral valve hypoplasia, (Z-score ≤-2) were included. Pre-operative and follow-up echocardiograms were reviewed. Primary outcome was successful biventricular circulation (BVC), defined as freedom from death, transplant, or single ventricular conversion at 1 year. Need for catheter based or surgical re-intervention (RI), valve annular growth, and significant late aortic or mitral valve obstruction were additional outcomes. Fifty one of 73 subjects (79%) had successful BVC and were free of RI at 1 year. Seven subjects failed BVC; four of those died. The overall 1 year survival for the cohort was 95%. Fifteen subjects underwent a RI but maintained BVC. In univariate analysis, larger transverse aorta (p = 0.006) and aortic valve (p = 0.02) predicted successful BVC without RI. In CART analysis, the combination of mitral valve (MV) to tricuspid valve (TV) ratio ≤0.66 with an aortic valve (AV) annulus Z-score ≤-3 had the greatest power to predict BVC failure (sensitivity 71%, specificity 94%). In those with successful BVC, the combination of both AV and MV Z-score ≤-2.5 increased the odds of RI (OR 3.8; CI 1.3-11.4). Follow-up of non-RI subjects revealed improvement in AV and MV Z-score (median AV annulus changed over time from -2.34 to 0.04 (p indices. In this complex population, 1 year survival is high, but

  10. Three dimensional endo-cardiovascular volume-rendered cine computed tomography of isolated left ventricular apical hypoplasia; A case report and literature review

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    Hong, Sun Hwa; Kim, Yang Min; Lee, Hyun Jong [Sejong General Hospital, Bucheon (Korea, Republic of)

    2016-02-15

    We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy.

  11. Cerebellar Hypoplasia

    Science.gov (United States)

    ... hypoplasia is a feature of a number of congenital (present at birth) malformation syndromes, such as Walker-Warburg syndrome (a form ... hypoplasia is a feature of a number of congenital (present at birth) malformation syndromes, such as Walker-Warburg syndrome (a form ...

  12. Genetics Home Reference: pontocerebellar hypoplasia

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    ... PCH1 also have very weak muscle tone (hypotonia), joint deformities called contractures, vision impairment, and breathing and ... InfoSearch: Pontocerebellar hypoplasia type 6 MalaCards: pontocerebellar hypoplasia Neuromuscular Disease Center, Washington University, St. Louis: Pontocerebellar Hypoplasia ...

  13. Focal dermal hypoplasia without focal dermal hypoplasia

    NARCIS (Netherlands)

    Contreras-Capetillo, Silvina N.; Lombardi, Maria Paola; Pinto-Escalante, Doris; Hennekam, Raoul C.

    2014-01-01

    Focal dermal hypoplasia (FDH; Goltz-Gorlin syndrome) is an X-linked dominant disorder affecting mainly tissues of ectodermal and mesodermal origin. The phenotype is characterized by hypoplastic linear skin lesions, eye malformations, hair and teeth anomalies, and multiple limbs malformations. The

  14. Abnormal platelet-derived growth factor signaling accounting for lung hypoplasia in experimental congenital diaphragmatic hernia.

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    Dingemann, Jens; Doi, Takashi; Ruttenstock, Elke; Puri, Prem

    2010-10-01

    The pathogenesis of pulmonary hypoplasia in congenital diaphragmatic hernia (CDH) is not fully understood. Platelet-derived growth factor A (PDGFA) and platelet-derived growth factor receptor α (PDGFRα) play a crucial role in lung development. It has been reported that PDGF induces H(2)O(2)-production and that oxidative stress may be an important mechanism for the impaired lung development in the nitrofen rat model. We hypothesized that pulmonary expression of PDGFA and PDGFRα is altered in the nitrofen induced CDH model. Pregnant rats received 100 mg nitrofen or vehicle on gestational day 9 (D9) and were sacrificed on D15, D18 or D21. RNA was extracted from fetal left lungs and mRNA levels of PDGFA and PDGFRα were determined using real-time polymerase chain reaction. Immunohistochemistry for protein expression of PDGFA and PDGFRα was performed. Pulmonary H(2)O(2) was measured colorimetrically. mRNA levels of PDGFRα at D15 (4.50 ± 0.87) and PDGFA at D18 (2.90 ± 1.38) were increased in the nitrofen group (P stress during lung development. Copyright © 2010 Elsevier Inc. All rights reserved.

  15. Vermian aplasia and hypoplasia

    International Nuclear Information System (INIS)

    Fitz, C.R.

    1990-01-01

    This paper reports vermian aplasia or hypoplasia unrelated to Dandy-Walker complex or Joubert syndrome. Vermian hypoplasia is most commonly associated with the malformations of Dandy-Walker cyst or variant. There have been few reports of familial vermian anomaly (Joubert syndrome) with episodes of polypnea and apnea. Five patients with vermian hypoplasia or absence were prospectively discovered among 1,130 children undergoing brain MR imaging. Ages ranged from 2 mo to 15 y at the time of discovery: The abnormalities were poorly defined in three patients who had previously undergone CT. There was no common link in these patients with a variable number of other systemic, brain, or facial abnormalities. No unifying symptoms were identified. Two patients had the MR imaging appearance of Joubert syndrome, but none had the cyclical respiratory symptoms usually associated with this syndrome

  16. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

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    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong [Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang (Korea, Republic of); Kim, Seon Jeong [Dept. of Radiology, Myongji Hospital, Goyang (Korea, Republic of)

    2017-05-15

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia.

  17. Isolated unilateral pulmonary artery hypoplasia with accompanying pulmonary parenchymal findings on CT: A case report

    International Nuclear Information System (INIS)

    Park, Surin; Cha, Yoon Ki; Kim, Jeung Sook; Kwon, Jae Hyun; Jeong, Yun Jeong; Kim, Seon Jeong

    2017-01-01

    Unilateral pulmonary artery hypoplasia or agenesis without congenital cardiovascular anomalies is rare in adults. We report a case of a 36-year-old man with isolated left unilateral pulmonary artery hypoplasia with recurrent hemoptysis. On computed tomography (CT), the left pulmonary artery showed hypoplasia with multiple collateral vessels seen in the mediastinum and the left hemithorax. Also, parenchymal bands and peripheral linear opacities were seen in the affected lung, which were probably due to chronic infarction induced by unilateral pulmonary artery hypoplasia. There are only a few reports focusing on the radiologic findings in the pulmonary parenchyma induced by unilateral pulmonary artery hypoplasia, such as parenchymal bands and peripheral linear opacities. Therefore we report this case, which focused on the CT findings in the pulmonary parenchyma due to isolated unilateral pulmonary artery hypoplasia

  18. Cerebral white matter hypoplasia

    International Nuclear Information System (INIS)

    Dietrich, R.B.; Shields, W.D.; Sankar, R.

    1990-01-01

    This paper demonstrates the MR imaging findings in children with cerebral white matter hypoplasia (CWMH). The MR studies of four children, aged 3-7 y (mean age, 2.3 y) with a diagnosis of CWMH were reviewed. In all cases multiplanar T1-weighted and T2-weighted spin-echo images were obtained. All children had similar histories of severe developmental delay and nonprogressive neurologic deficits despite normal gestational and birth histories. In two cases there was a history of maternal cocaine abuse. Autopsy correlation was available in one child. The MR images of all four children demonstrated diffuse lack of white matter and enlarged ventricles but normal-appearing gray matter. The corpus callosum, although completely formed, was severely thinned. There was no evidence of gliosis or porencephaly, and the distribution of myelin deposition was normal for age in all cases. Autopsy finding in one child correlated exactly with the MR finding

  19. Ischial hypoplasia, tibial hypoplasia and facial abnormalities: a new syndrome?

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    Nishimura, G. [Department of Radiology, Dokkyo University School of Medicine (Japan); Haga, Yoshihiko [Department of Orthopaedics, Shizuoka Children`s Hospital, Shizuoka (Japan); Aoki, Katsuhiko [Department of Radiology, Shizuoka Children`s Hospital, Shizuoka (Japan); Hasegawa, Tomoko [Division of Clinical Genetics, Shizuoka Children`s Hospital, Shizuoka (Japan)

    1998-12-01

    A child with facial abnormalities, short stature and a variety of skeletal alterations is reported. The facial abnormalities comprised low-set ears, short nose with a long philtrum, micrognathia and cleft palate. The skeletal alterations included ischial hypoplasia, malformations of the cervical spine, hypoplasia of the lesser trochanters, tibial hypoplasia with bowing of the lower legs, tibio-fibular diastasis with malformed distal tibial epiphyses, clubfeet and brachymesophalangy. The constellation of clinical and radiological findings in the present patient do not fit any known malformation syndrome. (orig.) With 4 figs., 8 refs.

  20. Ischial hypoplasia, tibial hypoplasia and facial abnormalities: a new syndrome?

    International Nuclear Information System (INIS)

    Nishimura, G.; Haga, Yoshihiko; Aoki, Katsuhiko; Hasegawa, Tomoko

    1998-01-01

    A child with facial abnormalities, short stature and a variety of skeletal alterations is reported. The facial abnormalities comprised low-set ears, short nose with a long philtrum, micrognathia and cleft palate. The skeletal alterations included ischial hypoplasia, malformations of the cervical spine, hypoplasia of the lesser trochanters, tibial hypoplasia with bowing of the lower legs, tibio-fibular diastasis with malformed distal tibial epiphyses, clubfeet and brachymesophalangy. The constellation of clinical and radiological findings in the present patient do not fit any known malformation syndrome. (orig.)

  1. Glenoid hypoplasia: a report of 2 patients.

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    Lynch, Christopher J; Taylor, John A M; Buchberger, Dale J

    2008-06-01

    This article discusses the imaging findings, clinical findings, and conservative chiropractic management of 2 patients with glenoid hypoplasia. Conventional radiographs of both patients revealed a hypoplastic glenoid bilaterally. Notch-like defects along with signs of degenerative disease were evident within the lower portion of the glenoid rims bilaterally in 1 patient and in the left glenoid rim of the other patient. Magnetic resonance imaging revealed a degenerative cyst or cortical defect in one patient along the anterior humeral head. The second patient showed a small slightly lobulated cystic region just posterior to the glenoid rim, consistent with the appearance of a synovial or ganglion cyst. Computed tomography with 3-dimensional reconstruction in 1 patient confirmed the presence of large posterior and superior osteophytes arising from the significantly hypoplastic glenoid. These images also revealed a slight posterior subluxation of the humeral head, widening of the anterior glenohumeral joint space, and retroversion of the glenoid. Treatment consisted of manual joint manipulation, soft tissue therapies, and therapeutic exercise for both patients. Both patients experienced improvements in symptoms, function, and physical examination findings. Glenoid hypoplasia is a developmental anomaly of the scapular neck which is predominantly bilateral and symmetric. Cross-sectional imaging studies should be considered in patients with symptoms that fail to improve over time. Conservative chiropractic care may be effective in managing symptoms in patients with glenoid hypoplasia.

  2. Optic nerve hypoplasia

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    Savleen Kaur

    2013-01-01

    Full Text Available Optic nerve hypoplasia (ONH is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65% than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED.

  3. Genetics Home Reference: focal dermal hypoplasia

    Science.gov (United States)

    ... in people with focal dermal hypoplasia is an omphalocele , which is an opening in the wall of ... Dermal Hypoplasia MedlinePlus Encyclopedia: Ectodermal dysplasia MedlinePlus Encyclopedia: Omphalocele General Information from MedlinePlus (5 links) Diagnostic Tests ...

  4. MR findings in pontocerebellar hypoplasia

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    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M. [Department of Radiology, Division of Paediatric Radiology, University Hospital of Freiburg, Freiburg (Germany); Darge, K. [Department of Paediatric Radiology, Children`s Hospital of the University of Heidelberg, Heidelberg (Germany); Baborie, A. [Department of Neuropathology, Neurozentrum, University of Freiburg, Freiburg (Germany); Korinthenberg, R. [Department of Neuropaediatrics, Children`s Hospital, University of Freiburg, Freiburg (Germany)

    1998-07-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to `float` in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.) With 3 figs., 2 tabs., 13 refs.

  5. MR findings in pontocerebellar hypoplasia

    International Nuclear Information System (INIS)

    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M.; Darge, K.; Baborie, A.; Korinthenberg, R.

    1998-01-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to 'float' in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.)

  6. Unusual right ventricle aneurysm and dysplastic pulmonary valve with mitral valve hypoplasia

    Directory of Open Access Journals (Sweden)

    Ozge Pamukcu

    2013-01-01

    Full Text Available We report a newborn with an unusual combination of aneurysmally dilated thin-walled right ventricle with hypertrophy of the apical muscles of the right ventricle. There was narrow pulmonary annulus, pulmonary regurgitation, and hypoplasia of the mitral valve and left ventricle. We propose that this heart represents a partial form of Uhl`s anomaly.

  7. Isolated Left Pulmonary Artery Agenesis: A Case Report

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    Tansel Ansal Balcı

    2012-08-01

    Full Text Available Unilateral pulmonary artery agenesis without any cardiovascular malformation is a rare anomaly. We present the imaging findings of a patient who was diagnosed as isolated left pulmonary artery agenesis. A 27-year-old female patient was admitted to our hospital due to dyspnea during exercise for five years. Chest X-ray revealed minimally small left pulmonary hilum and left lung. She was admitted to our clinic with the suspicion of pulmonary artery pathology. Absent perfusion of the left lung with normal ventilation was visualized on scintigraphy. MDCT angiography of pulmonary arteries showed absent left main pulmonary artery with systemic collaterals around left hemithorax. Pulmonary artery agenesis can be asymptomatic and isolated until adulthood. Both scintigraphy and CT angiography images of pulmonary artery agenesis of a patient are rare in the literature. Pulmonary ventilation- perfusion scintigraphy can be used not only for pulmonary embolism but also pathologies involving pulmonary artery and its branches. (MIRT 2012;21:80-83

  8. Heterochrony and Early Left-Right Asymmetry in the Development of the Cardiorespiratory System of Snakes

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    van Soldt, Benjamin J.; Metscher, Brian D.; Poelmann, Robert E.; Vervust, Bart; Vonk, Freek J.; Müller, Gerd B.; Richardson, Michael K.

    2015-01-01

    Snake lungs show a remarkable diversity of organ asymmetries. The right lung is always fully developed, while the left lung is either absent, vestigial, or well-developed (but smaller than the right). A ‘tracheal lung’ is present in some taxa. These asymmetries are reflected in the pulmonary arteries. Lung asymmetry is known to appear at early stages of development in Thamnophis radix and Natrix natrix. Unfortunately, there is no developmental data on snakes with a well-developed or absent left lung. We examine the adult and developmental morphology of the lung and pulmonary arteries in the snakes Python curtus breitensteini, Pantherophis guttata guttata, Elaphe obsoleta spiloides, Calloselasma rhodostoma and Causus rhombeatus using gross dissection, MicroCT scanning and 3D reconstruction. We find that the right and tracheal lung develop similarly in these species. By contrast, the left lung either: (1) fails to develop; (2) elongates more slowly and aborts early without (2a) or with (2b) subsequent development of faveoli; (3) or develops normally. A right pulmonary artery always develops, but the left develops only if the left lung develops. No pulmonary artery develops in relation to the tracheal lung. We conclude that heterochrony in lung bud development contributes to lung asymmetry in several snake taxa. Secondly, the development of the pulmonary arteries is asymmetric at early stages, possibly because the splanchnic plexus fails to develop when the left lung is reduced. Finally, some changes in the topography of the pulmonary arteries are consequent on ontogenetic displacement of the heart down the body. Our findings show that the left-right asymmetry in the cardiorespiratory system of snakes is expressed early in development and may become phenotypically expressed through heterochronic shifts in growth, and changes in axial relations of organs and vessels. We propose a step-wise model for reduction of the left lung during snake evolution. PMID:25555231

  9. Heterochrony and early left-right asymmetry in the development of the cardiorespiratory system of snakes.

    Science.gov (United States)

    van Soldt, Benjamin J; Metscher, Brian D; Poelmann, Robert E; Vervust, Bart; Vonk, Freek J; Müller, Gerd B; Richardson, Michael K

    2015-01-01

    Snake lungs show a remarkable diversity of organ asymmetries. The right lung is always fully developed, while the left lung is either absent, vestigial, or well-developed (but smaller than the right). A 'tracheal lung' is present in some taxa. These asymmetries are reflected in the pulmonary arteries. Lung asymmetry is known to appear at early stages of development in Thamnophis radix and Natrix natrix. Unfortunately, there is no developmental data on snakes with a well-developed or absent left lung. We examine the adult and developmental morphology of the lung and pulmonary arteries in the snakes Python curtus breitensteini, Pantherophis guttata guttata, Elaphe obsoleta spiloides, Calloselasma rhodostoma and Causus rhombeatus using gross dissection, MicroCT scanning and 3D reconstruction. We find that the right and tracheal lung develop similarly in these species. By contrast, the left lung either: (1) fails to develop; (2) elongates more slowly and aborts early without (2a) or with (2b) subsequent development of faveoli; (3) or develops normally. A right pulmonary artery always develops, but the left develops only if the left lung develops. No pulmonary artery develops in relation to the tracheal lung. We conclude that heterochrony in lung bud development contributes to lung asymmetry in several snake taxa. Secondly, the development of the pulmonary arteries is asymmetric at early stages, possibly because the splanchnic plexus fails to develop when the left lung is reduced. Finally, some changes in the topography of the pulmonary arteries are consequent on ontogenetic displacement of the heart down the body. Our findings show that the left-right asymmetry in the cardiorespiratory system of snakes is expressed early in development and may become phenotypically expressed through heterochronic shifts in growth, and changes in axial relations of organs and vessels. We propose a step-wise model for reduction of the left lung during snake evolution.

  10. Heterochrony and early left-right asymmetry in the development of the cardiorespiratory system of snakes.

    Directory of Open Access Journals (Sweden)

    Benjamin J van Soldt

    Full Text Available Snake lungs show a remarkable diversity of organ asymmetries. The right lung is always fully developed, while the left lung is either absent, vestigial, or well-developed (but smaller than the right. A 'tracheal lung' is present in some taxa. These asymmetries are reflected in the pulmonary arteries. Lung asymmetry is known to appear at early stages of development in Thamnophis radix and Natrix natrix. Unfortunately, there is no developmental data on snakes with a well-developed or absent left lung. We examine the adult and developmental morphology of the lung and pulmonary arteries in the snakes Python curtus breitensteini, Pantherophis guttata guttata, Elaphe obsoleta spiloides, Calloselasma rhodostoma and Causus rhombeatus using gross dissection, MicroCT scanning and 3D reconstruction. We find that the right and tracheal lung develop similarly in these species. By contrast, the left lung either: (1 fails to develop; (2 elongates more slowly and aborts early without (2a or with (2b subsequent development of faveoli; (3 or develops normally. A right pulmonary artery always develops, but the left develops only if the left lung develops. No pulmonary artery develops in relation to the tracheal lung. We conclude that heterochrony in lung bud development contributes to lung asymmetry in several snake taxa. Secondly, the development of the pulmonary arteries is asymmetric at early stages, possibly because the splanchnic plexus fails to develop when the left lung is reduced. Finally, some changes in the topography of the pulmonary arteries are consequent on ontogenetic displacement of the heart down the body. Our findings show that the left-right asymmetry in the cardiorespiratory system of snakes is expressed early in development and may become phenotypically expressed through heterochronic shifts in growth, and changes in axial relations of organs and vessels. We propose a step-wise model for reduction of the left lung during snake evolution.

  11. A Case of Fatal Pulmonary Hypoplasia with Congenital Diaphragmatic Hernia, Thoracic Myelomeningocele, and Thoracic Dysplasia.

    Science.gov (United States)

    Ito, Ai; Fujinaga, Hideshi; Matsui, Sachiko; Tago, Kumiko; Iwasaki, Yuka; Fujino, Shuhei; Nagasawa, Junko; Amari, Shoichiro; Kaneshige, Masao; Wada, Yuka; Takahashi, Shigehiro; Tsukamoto, Keiko; Miyazaki, Osamu; Yoshioka, Takako; Ishiguro, Akira; Ito, Yushi

    2017-10-01

    Background  Congenital diaphragmatic hernia (CDH) is fatal in severe cases of pulmonary hypoplasia. We experienced a fatal case of pulmonary hypoplasia due to CDH, thoracic myelomeningocele (MMC), and thoracic dysplasia. This constellation of anomalies has not been previously reported. Case Report  A male infant with a prenatal diagnosis of thoracic MMC with severe hydrocephalus and scoliosis was born at 36 weeks of gestation. CDH was found after birth and the patient died of respiratory failure due to pulmonary hypoplasia and persistent pulmonary hypertension of the newborn at 30 hours of age despite neonatal intensive care. An autopsy revealed a left CDH without herniation of the liver or stomach into the thoracic cavity, severe hydrocephalus, Chiari malformation type II, MMC with spina bifida from Th4 to Th12, hemivertebrae, fused ribs, deformities of the thoracic cage and legs, short trunk, and agenesis of the left kidney. Conclusion  We speculate that two factors may be associated with the severe pulmonary hypoplasia: decreased thoracic space due to the herniation of visceral organs caused by CDH and thoracic dysplasia due to skeletal deformity and severe scoliosis.

  12. MR assessment of fetal pulmonary hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kuwashima, Shigeko; Kohno, Atsushi; Saiki, Natoru; Iimura, Fumitoshi; Kohno, Tatsuo; Hashimoto, Teisuke; Fujioka, Mutsuhisa [Dokkyo Univ. School of Medicine, Mibu, Tochigi (Japan)

    2000-08-01

    The purpose of this study is to evaluate pulmonary hypoplasia of the fetus using MRI. The subjects consisted of 36 fetuses (18 to 40 weeks' gestation). All fetuses or mothers had major anomalies diagnosed on fetal ultrasonography. MR imaging was performed with a 1.5-T magnet and HASTE (half-Fourier acquisition single-shot turbo spin-echo) sequence. MR images were evaluated with special attention to the intensity of the lung. A diagnosis of pulmonary hypoplasia was based on the clinical, surgical, and autopsy findings. All fetuses with normal pulmonary development showed high intensity in the lung, while all fetuses with pulmonary hypoplasia showed a low intensity in the lung, obscured pulmonary vessels and a small thorax. There was a close correlation between the lung intensity and pulmonary growth. MR assessment of lung intensity may facilitate the diagnosis of pulmonary hypoplasia, particularly after 26 weeks' gestation. Some of the normally developing lung showed a low intensity from 20 to 24 weeks of gestational age. The change to normal lung intensity may occur during this period. (author)

  13. Genetics Home Reference: Leydig cell hypoplasia

    Science.gov (United States)

    ... normal male sexual development before birth and during puberty. In Leydig cell hypoplasia , affected individuals with a typical male chromosomal pattern (46,XY) may have a range of genital abnormalities. Affected males may have a small penis (micropenis), the opening of the urethra on the ...

  14. Focal dermal hypoplasia: A rare case report

    Directory of Open Access Journals (Sweden)

    Sahana M Srinivas

    2015-01-01

    Full Text Available Focal dermal hypoplasia (Goltz syndrome is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

  15. Tooth enamel hypoplasia in PHACE syndrome.

    Science.gov (United States)

    Chiu, Yvonne E; Siegel, Dawn H; Drolet, Beth A; Hodgson, Brian D

    2014-01-01

    Individuals with PHACE syndrome (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe syndrome) have reported dental abnormalities to their healthcare providers and in online forums, but dental involvement has not been comprehensively studied. A study was conducted at the third PHACE Family Conference, held in Milwaukee, Wisconsin, in July 2012. A pediatric dentist examined subjects at enrollment. Eighteen subjects were enrolled. The median age was 4.2 years (range 9 mos-9 yrs; 14 girls, 4 boys). Eleven of 18 patients had intraoral hemangiomas and five of these (50%) had hypomature enamel hypoplasia. None of the seven patients without intraoral hemangiomas had enamel hypoplasia. No other dental abnormalities were seen. Enamel hypoplasia may be a feature of PHACE syndrome when an intraoral hemangioma is present. Enamel hypoplasia increases the risk of caries, and clinicians should refer children with PHACE syndrome to a pediatric dentist by 1 year of age. © 2014 Wiley Periodicals, Inc.

  16. A case of luftsichel sign for left upper lobe collapse

    Directory of Open Access Journals (Sweden)

    Erden Erol Ünlüer

    2015-01-01

    Full Text Available The differential diagnosis of dyspnea in Emergency Department (ED patients is broad and atelectasis is one of the differentials among these. We present a 29-year-old women presented to our ED for evaluation of shortness of breath. On her chest examination, air entry and breath sounds were diminished on the left side but normal on the right. A posteroanterior chest radiograph showed radioluscent area in the upper zone of the left lung, around the aortic arch and also hyperdens area neighbouring this, like covered by a veil. Luftsichel sign together with this hiperdensity were consistent with the diagnose of left lung upper lobe collapse. The Luftsichel sign represents the hyperexpanded superior segment of the left lower lobe interposed between the atelectatic left upper lobe and aortic arch. Patient was discharged to home with chest physiotherapy and breathing exercises together with analgesic prescreption.

  17. Ectopic KIT copy number variation underlies impaired migration of primordial germ cells associated with gonadal hypoplasia in cattle (Bos taurus.

    Directory of Open Access Journals (Sweden)

    Heli Venhoranta

    Full Text Available Impaired migration of primordial germ cells during embryonic development causes hereditary gonadal hypoplasia in both sexes of Northern Finncattle and Swedish Mountain cattle. The affected gonads exhibit a lack of or, in rare cases, a reduced number of germ cells. Most affected animals present left-sided gonadal hypoplasia. However, right-sided and bilateral cases are also found. This type of gonadal hypoplasia prevails in animals with white coat colour. Previous studies indicated that gonadal hypoplasia is inherited in an autosomal recessive fashion with incomplete penetrance. In order to identify genetic regions underlying gonadal hypoplasia, a genome-wide association study (GWAS and a copy number variation (CNV analysis were performed with 94 animals, including 21 affected animals, using bovine 777,962 SNP arrays. The GWAS and CNV results revealed two significantly associated regions on bovine chromosomes (BTA 29 and 6, respectively (P=2.19 x 10(-13 and P=5.65 x 10(-6. Subsequent cytogenetic and PCR analyses demonstrated that homozygosity of a ~500 kb chromosomal segment translocated from BTA6 to BTA29 (Cs29 allele is the underlying genetic mechanism responsible for gonadal hypoplasia. The duplicated segment includes the KIT gene that is known to regulate the migration of germ cells and precursors of melanocytes. This duplication is also one of the two translocations associated with colour sidedness in various cattle breeds.

  18. Left Vocal Cord Paralysis Detected by PET/CT in a Case of Lung Cancer

    Directory of Open Access Journals (Sweden)

    Ali Ozan Oner

    2015-01-01

    Full Text Available We report a patient with lung cancer. The first PET/CT imaging revealed hypermetabolic mass in the left aortopulmonary region and hypermetabolic nodule in the anterior segment of the upper lobe of the left lung. After completing chemotherapy and radiotherapy against the primary mass in the left lung, the patient underwent a second PET/CT examination for evaluation of treatment response. This test demonstrated, compared with the first PET/CT, an increase in the size and metabolic activity of the primary mass in the left lung in addition to multiple, pathologic-sized, hypermetabolic metastatic lymph nodes as well as multiple metastatic sclerotic areas in bones. These findings were interpreted as progressive disease. In addition, an asymmetrical FDG uptake was noticed at the level of right vocal cord. During follow-up, a laryngoscopy was performed, which demonstrated left vocal cord paralysis with no apparent mass. Thus, we attributed the paralytic appearance of the left vocal cord to infiltration of the left recurrent laryngeal nerve by the primary mass located in the apical region of the left lung. In conclusion, the knowledge of this pitfall is important to avoid false-positive PET results.

  19. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

    Energy Technology Data Exchange (ETDEWEB)

    Katorza, Eldad; Nahama-Allouche, Catherine; Ducou le Pointe, Hubert; Garel, Catherine [Hopital d' Enfants Armand-Trousseau, Service de Radiologie, Paris (France); Castaigne, Vanina [Hopital Saint-Antoine, Service de Gynecologie-Obstetrique, Paris (France); Gonzales, Marie; Marlin, Sandrine [Hopital d' Enfants Armand-Trousseau, Service de Genetique et Embryologie medicales, Paris (France); Galliani, Eva [Hopital d' Enfants Armand-Trousseau, Service de Chirurgie maxillo-faciale, Paris (France); Jouannic, Jean-Marie; Rosenblatt, Jonathan [Hopital d' Enfants Armand-Trousseau, Service de Gynecologie-Obstetrique, Centre pluridisciplinaire de diagnostic prenatal, Paris (France)

    2011-05-15

    Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia. (orig.)

  20. Circulatory CNP Rescues Craniofacial Hypoplasia in Achondroplasia.

    Science.gov (United States)

    Yamanaka, S; Nakao, Kazumasa; Koyama, N; Isobe, Y; Ueda, Y; Kanai, Y; Kondo, E; Fujii, T; Miura, M; Yasoda, A; Nakao, Kazuwa; Bessho, K

    2017-12-01

    Achondroplasia is the most common genetic form of human dwarfism, characterized by midfacial hypoplasia resulting in occlusal abnormality and foramen magnum stenosis, leading to serious neurologic complications and hydrocephalus. Currently, surgery is the only way to manage jaw deformity, neurologic complications, and hydrocephalus in patients with achondroplasia. We previously showed that C-type natriuretic peptide (CNP) is a potent stimulator of endochondral bone growth of long bones and vertebrae and is also a potent stimulator in the craniofacial region, which is crucial for midfacial skeletogenesis. In this study, we analyzed craniofacial morphology in a mouse model of achondroplasia, in which fibroblast growth factor receptor 3 (FGFR3) is specifically activated in cartilage ( Fgfr3 ach mice), and investigated the mechanisms of jaw deformities caused by this mutation. Furthermore, we analyzed the effect of CNP on the maxillofacial area in these animals. Fgfr3 ach mice exhibited midfacial hypoplasia, especially in the sagittal direction, caused by impaired endochondral ossification in craniofacial cartilage and by premature closure of the spheno-occipital synchondrosis, an important growth center in craniomaxillofacial skeletogenesis. We crossed Fgfr3 ach mice with transgenic mice in which CNP is expressed in the liver under the control of the human serum amyloid-P component promoter, resulting in elevated levels of circulatory CNP ( Fgfr3 ach /SAP-Nppc-Tg mice). In the progeny, midfacial hypoplasia in the sagittal direction observed in Fgfr3 ach mice was improved significantly by restoring the thickness of synchondrosis and promoting proliferation of chondrocytes in the craniofacial cartilage. In addition, the foramen magnum stenosis observed in Fgfr3 ach mice was significantly ameliorated in Fgfr3 ach /SAP-Nppc-Tg mice due to enhanced endochondral bone growth of the anterior intraoccipital synchondrosis. These results clearly demonstrate the therapeutic

  1. Causes of congenital unilateral pulmonary hypoplasia

    International Nuclear Information System (INIS)

    Currarino, G.; Williams, B.; Children's Medical Center, Dallas, TX

    1985-01-01

    A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

  2. Congenital Unilateral Hypoplasia of Depressor Anguli Oris

    Directory of Open Access Journals (Sweden)

    Seckin O. Ulualp

    2012-01-01

    Full Text Available Objectives. Asymmetric facial appearance may originate from abnormalities of facial musculature or facial innervation. We describe clinical features of congenital hypoplasia of depressor anguli oris muscle in a child. Material and Methods. Chart of a 10-month-old female referred to a tertiary care pediatric hospital for assessment of facial paralysis was reviewed. Data included relevant history and physical examination, diagnostic work up, and management. Results. The child presented with asymmetric movement of lower lip since birth. Asymmetry of lower lip was more pronounced when she smiled and cried. Rest of the face movement was symmetric. On examination, the face appeared symmetric at rest. The child had inward deviation of right lower lip when she smiled. Facial nerve function, as determined by frowning/forehead, wrinkling, eye closure, nasolabial fold depth, and tearing, was symmetric. Magnetic resonance imaging of the temporal bones and internal auditory canals were within normal limits. Echocardiogram did not show cardiac abnormality. Auditory brainstem response showed no abnormality. Conclusions. Congenital hypoplasia of depressor anguli oris is a rare anomaly that causes asymmetric crying face. Pediatricians and otolaryngologists need to be cognizant of cardiac, head and neck, and central nervous system anomalies associated with congenital unilateral hypoplasia of depressor anguli oris.

  3. Method for imaging pulmonary arterial hypoplasia

    International Nuclear Information System (INIS)

    Triantafillou, M.

    2000-01-01

    Full text: Pulmonary hypoplasia represents an incomplete development of the lung, resulting in the reduction of distended lung volume. This is associated with small or absent number of airway divisions, alveoli, arteries and veins. Unilateral pulmonary Hypoplasia is often asymptomatic and may be demonstrated as a hypodense lung on a chest X-ray. Computer Tomography (CT) scanning would show anatomical detail and proximal vessels. Magnetic Resonance Imaging (MRI) will show no more detail than which the CT scan has already demonstrated. It is, also, difficult to visualise collateral vessels from systemic and/or bronchial vessels on both these modalities. Pulmonary Angiography would give the definitive answer, but it is time consuming and has significant risks associated with the procedure. There are high costs associated with these modalities. Nuclear Medicine Ventilation/Perfusion (V/Q) scan performed on these patients would demonstrate diminished ventilation due to reduced lung volume and absence of perfusion to the hypoplastic lung. To date, we have performed V/Q lung scan on two children in our department. Both cases demonstrate diminished ventilation with no perfusion to the hypoplastic lung. Though the gold standard is Pulmonary Angiography, V/Q scanning is cost effective, less time consuming and a non invasive procedure that can be performed as an outpatient. It is accurate as it demonstrates absent lung perfusion, confirming the patient has pulmonary arterial hypoplasia. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

  4. Pontoneocerebellar hypoplasia: definition of MR features

    International Nuclear Information System (INIS)

    Goasdoue, P.; Lalande, G.; Adamsbaum, C.; Rodriguez, D.; Moutard, M.-L.; Robain, O.

    2001-01-01

    We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission. (orig.)

  5. Ischemic stroke in patient with existing congenital hypoplasia of the middle cerebral artery

    International Nuclear Information System (INIS)

    Manchev, I.; Manolova, T.; Manchev, L.

    2015-01-01

    Presented is a clinical case of a woman 29 years old with ischemic stroke (IS), which has developed abruptly in existing congenital hypoplasia and occlusion of the middle cerebral artery. There are no other well or less well documented risk factors for cerebrovascular disease. In family history noted that the father of the patient died suddenly at the age of 45 years from stroke, also without evidence of vascular disease. On magnetic resonance imaging (MRI) of the brain is found high signal zone in the left nucleus lentiformis. We discussed the possibilities for implementing conventional angiography and eventually surgical procedures unfortunately rejected due to the high risk to the patient. Key words: Ischemic Stroke. Magnetic Resonance Imaging. Hypoplasia

  6. Unilateral hypoplasia with contralateral hypertrophy of anterior belly of digastric muscle: a case report.

    Science.gov (United States)

    Ochoa-Escudero, Martin; Juliano, Amy F

    2016-10-01

    Anomalies of the anterior belly of the digastric muscle (DM) are uncommon. We present a case of hypoplasia of the anterior belly of the left DM with hypertrophy of the anterior belly of the contralateral DM. The importance of recognizing this finding is to differentiate hypoplasia of the anterior belly of the DM from denervation atrophy, and not to confuse contralateral hypertrophy with a submental mass or lymphadenopathy. In denervation atrophy of the anterior belly of the DM, associated atrophy of the ipsilateral mylohyoid muscle is present. Hypertrophy of the anterior belly of the contralateral DM can be differentiated from a submental mass or lymphadenopathy by recognizing its isodensity on computed tomography and isointensity on magnetic resonance imaging to other muscles, without abnormal contrast enhancement.

  7. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism.

    Science.gov (United States)

    Masuda, Naonori; Hasegawa, Taiji; Yamashita, Mariko; Ogata, Nahoko

    2014-01-01

    Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism.

  8. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

    Directory of Open Access Journals (Sweden)

    Masuda N

    2014-09-01

    Full Text Available Naonori Masuda, Taiji Hasegawa, Mariko Yamashita, Nahoko Ogata Department of Ophthalmology, Nara Medical University, Nara, Japan Abstract: Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism. Keywords: albinism, foveal hemorrhage, foveal hypoplasia, simple hemorrhage

  9. Brain stem hypoplasia associated with Cri-du-Chat syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Jin Ho; Lee, Ha Young; Lim, Myung Kwan; Kim, Mi Young; Kang, Young Hye; Lee, Kyung Hee; Cho, Soon Gu [Dept. of Radiology, Inha University Hospital, Inha University School of Medicine, Incheon (Korea, Republic of)

    2013-12-15

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  10. The radiological features of Goltz syndrome: Focal dermal hypoplasia

    International Nuclear Information System (INIS)

    Boothyrod, A.E.; Hall, C.M.

    1988-01-01

    Two female infants with Goltz syndrome (focal dermal hypoplasia) were recently investigated for severe feeding problems and failure to thrive. Both demonstrated severe skeletal malformations and marked gastrooesophageal reflux with laxity of the hiatus. One child (case 1) exhibited nasal regurgitation during feeding. Interestingly, both children had undergone surgery; Case 1 or a right parasagittal abdominal hernia associated with focal dermal hypoplasia of the abdominal wall and Case 2 for an exomphalos also associated with dermal hypoplasia. This observation suggests more widespread mesodermal abnormality. (orig./GDG)

  11. Pontocerebellar hypoplasia associated with respiratory-chain defects

    NARCIS (Netherlands)

    de Koning, T. J.; de Vries, L. S.; Groenendaal, F.; Ruitenbeek, W.; Jansen, G. H.; Poll-The, B. T.; Barth, P. G.

    1999-01-01

    Pontocerebellar hypoplasias are congenital disorders of brain morphogenesis which include such diverse etiologies as carbohydrate-deficient glycoprotein syndrome type 1, cerebromuscular dystrophies (Walker-Warburg syndrome, Fukuyama syndrome, muscle-eye-brain disease) and at least two types of

  12. Genetics Home Reference: VLDLR-associated cerebellar hypoplasia

    Science.gov (United States)

    ... hypoplasia include moderate to profound intellectual disability, impaired speech (dysarthria) or a lack of speech, and eyes that do not look in the ... the United States. This condition has also been reported in families from Iran and Turkey. Related Information ...

  13. Enamel hypoplasia in the middle pleistocene hominids from Atapuerca (Spain).

    Science.gov (United States)

    Bermúdez de Castro, J M; Pérez, P J

    1995-03-01

    The prevalence and chronology of enamel hypoplasias were studied in a hominid dental sample from the Sima de los Huesos (SH) Middle Pleistocene site at the Sierra de Atapuerca (Burgos, northern Spain). A total of 89 permanent maxillary teeth, 143 permanent mandibular teeth, and one deciduous lower canine, belonging to a minimum of 29 individuals, were examined. Excluding the antimeres (16 maxillary and 37 mandibular cases) from the sample, the prevalence of hypoplasias in the permanent dentition is 12.8% (23/179), whereas the deciduous tooth also showed an enamel defect. No statistically significant differences were found between both arcades and between the anterior and postcanine teeth for the prevalence of hypoplasias. In both the maxilla and the mandible the highest frequency of enamel hypoplasias was recorded in the canines. Only one tooth (a permanent upper canine) showed two different enamel defects, and most of the hypoplasias were expressed as faint linear horizontal defects. Taking into account the limitations that the incompleteness of virtually all permanent dentitions imposes, we have estimated that the frequency by individual in the SH hominid sample was not greater than 40%. Most of the hypoplasias occurred between birth and 7 years (N = 18, X = 3.5, SD = 1.3). Both the prevalence and severity of the hypoplasias of the SH hominid sample are significantly less than those of a large Neandertal sample. Furthermore, prehistoric hunter-gatherers and historic agricultural and industrial populations exhibit a prevalence of hypoplasias generally higher than that of the SH hominids. Implications for the survival strategies and life quality of the SH hominids are also discussed.

  14. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    Energy Technology Data Exchange (ETDEWEB)

    Robida, A.; Fettich, D.

    1985-09-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease.

  15. Tetralogy of Fallot with origin of left pulmonary artery from the ascending aorta

    International Nuclear Information System (INIS)

    Robida, A.; Fettich, D.

    1985-01-01

    Anomalous origin of the left pulmonary artery in tetralogy of Fallot was diagnosed in a 4-year-old boy by cardiac catheterization. Corrective surgery was performed. The child died immediately following the surgical procedure. Postmortem examination revealed obstructive pulmonary vascular disease of the left lung and normal histology of right lung vessels. Early recognition and surgical correction of the anomaly is important with the view to preventing obstructive pulmonary vascular disease. (orig.)

  16. Blaschko Linear Enamel Defects - A Marker for Focal Dermal Hypoplasia: Case Report of Focal Dermal Hypoplasia

    Directory of Open Access Journals (Sweden)

    Stefan Gysin

    2015-05-01

    Full Text Available Focal dermal hypoplasia (FDH is a rare genetic skin disorder. The inheritance of FDH or Goltz-Gorlin syndrome is X-linked dominant and the disease is associated with a PORCN gene mutation. This gene plays a key role in the Wnt pathway, which has an impact on embryonic development. Every tissue derived from meso- and ectoderm can be affected. Patients suffer from cutaneous, ocular, osseous, oral and dental defects. The skin and dental alterations manifest along the Blaschko lines. We present a woman (born in 1962 suffering from FDH with congenital skin changes and Blaschko linear enamel defects. Typical symptoms (e.g. fat herniations, scoliosis, syndactyly, microphthalmia, caries and alopecia plus vertical grooving of all teeth gave a first indication. Molecular genetic testing confirmed the definitive diagnosis of FDH. We hypothesize that, in the context of typical skin changes, visible Blaschko lines on the teeth in the form of vertical grooves are almost pathognomonic for FDH.

  17. Biliary atresia and cerebellar hypoplasia in polysplenia syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Vanderdood, Kurt; Op de Beeck, Bart; Desprechins, Brigitte; Osteaux, Michel [Department of Radiology, Free University Brussels, AZ-VUB, Laarbeeklaan 101, 1090 Brussels (Belgium)

    2003-09-01

    We report a 3.5-month-old boy with polysplenia syndrome who demonstrated hemiazygos continuation of the inferior vena cava, extrahepatic biliary atresia, multiple splenunculi, bowel malrotation, and the rare finding of brainstem and cerebellar hypoplasia. A possible pathogenesis for cerebellar hypoplasia in this syndrome is suggested after review of the literature. The importance of seeking associated anomalies in biliary atresia, which may be possible indicators of polysplenia syndrome, is stressed since these patients need appropriate management when surgery is considered. (orig.)

  18. Trans-sinusal maxillary distraction for correction of midfacial hypoplasia: long-term clinical results.

    Science.gov (United States)

    Nadjmi, N; Schutyser, F; Van Erum, R

    2006-10-01

    Maxillary distraction osteogenesis is indicated in severe angle class III malocclusions, and severe maxillary hypoplasia among some cleft patients and other craniofacial deformities. Twenty patients, aged 8-48 years (mean 17.8+/-10.5 SD) with maxillary and midfacial hypoplasia were treated. The follow-up period was 13-65 months (mean 35+/-16.3 SD). A trans-sinusal maxillary distractor was placed intraorally at each side of the maxilla. The distraction vector was predicted using specialist software, and was transferred to the patients using stereolithographic models and individual templates. A (high) Le Fort I type osteotomy was performed. The amount of activation varied from 8 to 17.5 mm (mean 13.1+/-2.9 SD). Soft and hard tissue formation resulted in complete healing across the distraction gaps. The distractors are almost completely submerged, and can be left in place as long as necessary to avoid relapse. Wit's appraisal was used to measure the stability of the long-term distraction results. Results up to 5 years after distraction showed considerable maxillary advancement with long-term stability. Ongoing growth of the facial skeleton must be considered when distraction osteogenesis is chosen in growing patients.

  19. Liver segment IV hypoplasia as a risk factor for bile duct injury.

    Science.gov (United States)

    Mercado, Miguel Angel; Franssen, Bernardo; Arriola, Juan Carlos; Garcia-Badiola, Artemio; Arámburo, Rigoberto; Elnecavé, Alejandro; Cortés-González, Rubén

    2011-09-01

    Bile duct injury remains constant in the era of laparoscopic cholecystectomy and misidentification of structures remains one of the most common causes of such injuries. Abnormalities in liver segment IV, which is fully visible during laparoscopic cholecystectomy, may contribute to misidentification as proposed herein. We describe the case of a 36-year-old female who had a bile duct injury during a laparoscopic cholecystectomy where the surgeon noticed an unusually small distance between the gallbladder and the round ligament. We define hypoplasia of liver segment IV as well as describe the variation of the biliary anatomy in the case. We also intend to fit it in a broader spectrum of developmental anomalies that have both hyopoplasia of some portion of the liver and variations in gallbladder and bile duct anatomy that may contribute to bile duct injury. To our knowledge, hypoplasia of liver segment IV has not been suggested in the literature as a risk factor for bile duct injury except in the extreme case of a left-sided gallbladder. Surgeons should be vigilant during laparoscopic cholecystectomy when they become aware of an unusually small distance between the gallbladder bed and the round ligament prior to beginning their dissection, variations in the common bile duct and cystic duct should be expected.

  20. Rhabdomyolysis in pontocerebellar hypoplasia type 2 (PCH-2)

    NARCIS (Netherlands)

    Barth, Peter G.; Ryan, Monique M.; Webster, Richard I.; Aronica, Eleonora; Kan, Alex; Ramkema, Marja; Jardine, Philip; Poll-The, Bwee Tien

    2008-01-01

    Pontocerebellar hypoplasia type 2, an autosomal recessive neurodegeneration with prenatal onset, is characterised by progressive microcephaly and chorea/dystonia and has not previously been associated with muscular involvement. The gene associated with PCH-2 is unknown. An episode of rhabdomyolysis

  1. Amelogenesis Imperfect, Enamel Hypoplasia and Fluorosis Dental - Literature Review

    Directory of Open Access Journals (Sweden)

    Flávia Magnani Bevilacqua

    2015-12-01

    Full Text Available The developmental disorders of enamel are abnormalities of structure which can affect both dentitions. These abnormalities include amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. The amelogenesis imperfecta is a hereditary change and enamel hypoplasia is a quantitative defect of enamel that occurs as a result of systemic problems, local and also inherited factors, or even the combination of them. Dental fluorosis is a hypoplasia caused by the chronic ingestion of fluoride during odontogenesis. All these anomalies have similar clinical characteristics, and it is necessary to be careful in their assessment. It is extremely important to know these abnormalities to establish a differential diagnosis and, consequently, a treatment plan, which can be set for each situation. Therefore, the purpose of this study was to review the literature regarding these three anomalies: amelogenesis imperfecta, enamel hypoplasia and dental fluorosis. It was concluded that to establish the differential diagnosis of these abnormalities as well as a proper treatment plan, it is indispensable the professional knowledge associated with the clinical examination. The examination has to consist of medical history and physical examination, and in some cases, x-ray examination.

  2. Hypoplasia of the internal carotid artery with intercavernous anastomosis

    International Nuclear Information System (INIS)

    Chen, C.J.; Wang, L.J.; Wong, Y.C.; Chen, S.T.; Hsieh, F.Y.

    1998-01-01

    We report a symptomatic case of unilateral hypoplasia of the internal carotid artery with an intercavernous anastomosis, a very rare developmental anomaly. The symptoms were caused by occlusion of the proximal middle cerebral artery which possibly related to the haemodynamic stress caused by the anomalous intercavernous anastomosis. (orig.)

  3. [Left postpneumonectomy syndrome: early endoscopic treatment].

    Science.gov (United States)

    Rombolá, Carlos A; León Atance, Pablo; Honguero Martínez, Antonio Francisco; Rueda Martínez, Juan Luis; Núñez Ares, Ana; Vizcaya Sánchez, Manuel

    2009-12-01

    Postpneumonectomy syndrome is characterized by postoperative bronchial obstruction caused by mediastinal shift. The syndrome is well documented in the medical literature as a late complication of right pneumonectomy; however, it rarely occurs following resection of the left lung, and only 10 cases have been published. The pathophysiology, clinical manifestations, prognosis, and treatment are similar for both sides of the lung. We present the case of an adult patient who underwent left pneumonectomy and developed postpneumonectomy syndrome 15 months later. Stenosis of the intermediate bronchus occurred between the vertebral body and the right pulmonary artery. Endoscopic treatment with a self-expanding metal stent was successful, and complete remission was observed over the 6 months of follow-up.

  4. Bilateral bony fusion around the supraspinatus muscle inducing muscle hypoplasia and shoulder pain

    Energy Technology Data Exchange (ETDEWEB)

    Son, YeNa; Jin, Wook; Park, So Young [Kyung Hee University Hospital at Gangdong, Department of Radiology, 892, Dongnam-ro, Gangdong-gu, Seoul (Korea, Republic of); Ryu, Kyung Nam; Park, Ji Seon [Kyung Hee University Hospital, Department of Radiology, 23 Kyunghee-daero, Dongdaemun-gu, Seoul (Korea, Republic of)

    2017-03-15

    We describe the case of a 30-year-old man who developed chronic bilateral shoulder pain that relapsed and remitted over the course of 1 year. The patient was diagnosed with congenital shoulder fusion anomalies. The right shoulder showed anomalous accessory articulation between the distal third of the clavicle and the acromion along with normal articulation of the shoulder on CT. At the left shoulder, bony fusions were present between the distal portion of the clavicle, the acromion, and the coracoid process, and between the coracoid process, upper portion of the glenoid, and upper body of the scapula, which formed a bony canal and was responsible for hypoplasia of the supraspinatus muscle on CT and MRI. To our knowledge, this is the first description of such congenital shoulder anomalies with extreme bony fusion and is an illustrative example of how imaging may be used to differentiate fusion from other congenital abnormalities of the shoulder to aid diagnosis. (orig.)

  5. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

    Directory of Open Access Journals (Sweden)

    Shailesh Solanki

    2016-01-01

    Full Text Available A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

  6. Treatment of enamel hypoplasia in a patient with Usher syndrome.

    Science.gov (United States)

    de la Peña, Victor Alonso; Valea, Martín Caserío

    2011-08-01

    Usher syndrome (USH) is a group of autosomal recessive diseases characterized by the association of retinitis pigmentosa with sensorineural hearing loss. There are three types of USH. In addition, in people with USH and hypoplasia, the thickness of the enamel is reduced. The authors describe a case of a patient with USH type II associated with severe enamel hypoplasia and multiple unerupted teeth. The authors placed direct composite crowns and extracted severely affected and impacted molars. There is little information available on the oral pathologies of USH. Because the authors did not know how the patient's condition would progress and the patient still was growing, the authors treated the patient conservatively by placing direct composite crowns. The treatment has met both esthetic and functional expectations for 10 years. Copyright © 2011 American Dental Association. All rights reserved.

  7. Ischio-pubic-patellar hypoplasia: is it a new syndrome?

    International Nuclear Information System (INIS)

    Habboub, H.K.; Thneibat, W.A.

    1997-01-01

    Aplasia/hypoplasia of the patella has been described as an isolated finding or, more commonly, as a part of congenital syndromes. We describe here bilateral absence of the patella in an 11-year-old girl with absence of the ischial and inferior pubic rami bilaterally. Other associated skeletal and soft-tissue deformities are also reported. To our knowledge, the constellation of these findings has not been described previously and represents a unique syndrome. (orig.). With 1 fig

  8. Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

    OpenAIRE

    Juniarti, Devi Eka

    2010-01-01

    Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel h...

  9. SU-G-TeP1-11: Predictors of Cardiac and Lung Dose Sparing in DIBH for Left Breast Treatment

    Energy Technology Data Exchange (ETDEWEB)

    Cao, N; Kalet, A; Fang, L; Dempsey, C; Young, L; Kim, J; Mayr, N; Meyer, J [University of Washington Medical Center, Seattle, WA (United States); Lavilla, M; Richardson, H; McClure, R [Seattle Cancer Care Alliance, Seattle, WA (United States)

    2016-06-15

    Purpose: This retrospective study of left sided whole breast radiation therapy (RT) patients investigates possible predictive parameters correlating to cardiac and left lung dose sparing by deep inspiration breath-hold (DIBH) technique compared to free-breathing (FB). Methods: Thirty-one patients having both DIBH and FB CT scans were included in the study. All patients were planned with a standard step-and-shoot tangential technique using MV photons, with prescription of 50Gy or 50.4Gy. The displacement of the breath hold sternal mark during DIBH, the cardiac contact distances of the axial (CCDax) and parasagittal (CCDps) planes, and lateral-heart-to-chest (LHC) distance on FB CT scans were measured. Lung volumes, mean dose and dose-volume histograms (V5, V10 and V20) were analyzed and compared for heart and left lung for both FB and DIBH techniques. Correlation analysis was performed to identify the predictors for heart and left lung dose sparing. Two-tailed Student’s t-test and linear regression were used for data analysis with significance level of P≤0.05. Results: All dosimetric metrics for the heart and left lung were significantly reduced (P<0.01) with DIBH. Breath hold sternal mark displacement ranged from 0.4–1.8 cm and correlated with mean (P=0.05) and V5 (P=0.02) of heart dose reduction by DIBH. FB lung volume showed correlation with mean lung dose reduction by DIBH (P<0.01). The FB-CCDps and FB-LHC distance had strong positive and negative correlation with FB mean heart dose (P<0.01) and mean heart dose reduction by DIBH (P<0.01), respectively. FB-CCDax showed no correlation with dosimetric changes. Conclusion: DIBH technique has been shown to reduce dose to the heart and left lung. In this patient cohort, FB-CCDps, FB-LHC distance, and FB lung volume served as significant predictors for heart and left lung. These parameters can be further investigated to be used as a tool to better select patients who will benefit from DIBH.

  10. Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

    Directory of Open Access Journals (Sweden)

    Emma A Webb

    Full Text Available OBJECTIVES: To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. PATIENTS AND METHODS: Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex was performed. RESULTS: Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05 than controls (4 had scores in the clinically significant range. Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, p<0.02, r = -0.46, p<0.049 respectively. There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores. CONCLUSIONS: Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic

  11. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ashish Suryawanshi

    2011-01-01

    Full Text Available Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured specific rehabilitation exercises for the shoulder.

  12. Hypoplasia of the thumb. Clinical presentation and reconstruction

    International Nuclear Information System (INIS)

    Vergara A, Enrique M

    2008-01-01

    In the genesis of the partial or total absence of the thumb they are genetic, environmental factors or a combination of both. It is take part of a syndrome or to be isolated and frequently associated with problems of radial longitudinal deficiency of the forearm. Objective. The purpose of this study is shown the experience, the focus of the processing and the results obtained since the point esthetic and functional view. The most it accepted classification is the proposal by Blauth that helps to determine the forecast and the processing. Materials and methods. it is a work type series of cases in 22 children with hypoplasia of the thumb, with a minimum of 12 months, (average 28 months). In 15 cases there were association of radial dysplasia or another anomaly among them 4 patients with VATER, and the 7 remaining they corresponded to hypoplasia of the thumb as only entity. We carried out tendon transfer, with opening of the first comisure in 2 patients with hypoplasia type II. In 3 patients, with hypoplasia type III A, one carries out corner opening, transfer of the superficial flexor of the 4 finger to correct instability of the articulation MF and opposition of the thumb, and transfer for extension of the thumb. In 17 cases one carries out politicization of the index. Results. The outcome was evaluated in: non pinch, lateral pinch and fingertip pinch; the grade of opposition like good, minimal and non opposition, and the aesthetic result according to the satisfaction of the parents in bad, regular and good. The five children reconstructed with transfers of tendons and comisure opening had good result. In 17 children with politicization one patient had a necrosis, of the 16 remaining a good or acceptable result was obtained. Discussion. It is not easy to follow a good system of measure of the functional results. We find that a practical way to evaluate was the clip, opposition and aesthetics. Previous to the surgeries it is required to evaluate alterations

  13. Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

    Energy Technology Data Exchange (ETDEWEB)

    Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden [Istanbul Faculty of Medicine, Department of Radiology, Istanbul (Turkey); Ozmen, Meral [Istanbul Faculty of Medicine, Department of Paediatric Neurology, Istanbul (Turkey)

    2004-11-01

    Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

  14. Congenital bilateral perisylvian syndrome with pituitary hypoplasia and ectopic neurohypophysis

    International Nuclear Information System (INIS)

    Yekeler, Ensar; Genchellac, Hakan; Dursun, Memduh; Acunas, Gulden; Ozmen, Meral

    2004-01-01

    Congenital bilateral perisylvian syndrome (CBPS) is a congenital neurological syndrome characterized by pseudobulbar palsy, cognitive deficits and bilateral perisylvian abnormalities observed on imaging. The described abnormality in CBPS is polymicrogyria located in the frontal, parietal, and/or occipital lobes. A few syndromes or abnormalities associated with this syndrome have been documented. Pituitary abnormalities are rare disorders. Association of CBPS with pituitary abnormalities has not been reported previously. In this case, a combination of bilateral perisylvian polymicrogyria with pituitary hypoplasia and ectopic neurohypophysis, caused by a possible single common insult, is presented. (orig.)

  15. Abolished ventilation and perfusion of lung caused by blood clot in the left main bronchus

    DEFF Research Database (Denmark)

    Afzelius, P; Bergmann, A; Henriksen, J H

    2015-01-01

    /Q) scintigraphy with single-photon emission CT (SPECT)/CT. V/Q SPECT/CT demonstrated abolished ventilation due to obstruction of the left main bronchus and markedly reduced perfusion of the entire left lung, a condition that was completely reversed after removal of a blood clot. We present the first pictorially......It is generally assumed that the lungs possess arterial autoregulation associated with bronchial obstruction. A patient with pneumonia and congestive heart failure unexpectedly developed frequent haemoptysis. High-resolution CT and diagnostic CT were performed as well as ventilation/perfusion (V...

  16. Indirect veneer treatment of anterior maxillary teeth with enamel hypoplasia

    Directory of Open Access Journals (Sweden)

    Devi Eka Juniarti

    2010-09-01

    Full Text Available Background: Nowadays, aesthetic rehabilitation becomes a necessity. It is affected by patient’s background, especially career, social and economic status. The aesthetic abnormality of anterior teeth i.e discoloration, malposition and malformation can affect patient’s appearance, especially during smile. These dental abnormalities, as a result, can decrease patient’s performance. Dental malformation, for instance, can be caused by developmental tooth defect, such as enamel hypoplasia. Enamel hypoplasia is a developmental defect caused by the lack of matrix amount which leads to thin and porous enamel. Enamel hypoplasia can also be caused by matrix calcification disturbance starting from the formation and development of enamel matrix causing defect and permanent changes which can occur on one or more tooth. Purpose: The aim of the study is to improve dental discoloration and tooth surface texture on anterior maxillary teeth with enamel hypoplasia by using indirect veneer with porcelain material. Case: A 20 years-old woman with enamel hypoplasia came to the Dental Hospital, Faculty of Dentistry Airlangga University. The patient wanted to improve her anterior maxillary teeth. It is clinically known that there were some opaque white spots (chalky spotted and porous on anterior teeth’s surface. Case management: Indirect veneer with porcelain material had been chosen as a restoration treatment which has excellent aesthetics and strength, and did not cause gingival irritation. As a result, the treatment could improve the confidence of the patient, and could also make their function normal. Conclusion: Indirect veneer is an effective treatment, which can improve patient’s appearance and self confidence.Latar belakang: Saat ini perbaikan estetik menjadi suatu kebutuhan. Kebutuhan akan estetik dipengaruhi latar belakang penderita, terutama karir, status sosial dan ekonomi. Hal ini disebabkan, kelainan estetik seperti diskolorasi, malposisi

  17. Trans-sinusal maxillary distraction for correction of midfacial hypoplasia: long-term clinical results.

    NARCIS (Netherlands)

    Nadjmi, N.; Schutyser, F.A.C.; Erum, R. van

    2006-01-01

    Maxillary distraction osteogenesis is indicated in severe angle class III malocclusions, and severe maxillary hypoplasia among some cleft patients and other craniofacial deformities. Twenty patients, aged 8-48 years (mean 17.8+/-10.5 SD) with maxillary and midfacial hypoplasia were treated. The

  18. Maxillary Hypoplasia With Congenital Oligodontia Treated by Maxillary Distraction Osteogenesis.

    Science.gov (United States)

    Mishima, Sayaka; Yamaguchi, Takako; Watanabe, Takuma; Komatani, Toru; Nakao, Kazumasa; Takahashi, Katsu; Bessho, Kazuhisa

    2018-02-27

    It is known that congenitally missing teeth can often cause differences in craniofacial morphology; however, there are few reported cases of orthognathic surgical treatment for these patients. Herein, the authors report a rare case of maxillary hypoplasia with congenital oligodontia treated by maxillary distraction osteogenesis with internal device. A 17-year-old male presenting with multiple tooth agenesis and maxillary recession was referred to our hospital for orthognathic surgical treatment. Preoperative simulation surgery was performed using Full-Color 3-dimensional salt model. After surgery, improvement in maxillary recession and occlusal stability was observed. This report demonstrates the advantages of the method used herein, which includes reduction in operating time with increase in the safety of the procedure.

  19. Distraction osteogenesis of radiation-induced orbitozygomatic hypoplasia.

    Science.gov (United States)

    Grover, Ramon; Murray, Dylan; Fialkov, Jeffrey A

    2008-05-01

    In the last decade, the application of distraction osteogenesis to the craniofacial skeleton has grown to include not only deformities of the mandible, but of the midface, palate, dentoalveolar region, and calvarium. A major advantage of distraction osteogenesis lies in the simultaneous soft tissue histogenesis that accompanies the bony distraction process, allowing for potentially lower relapse rates and improved cosmesis. Although this may seem appropriately suited to irradiation-induced deformities of both hard and soft tissues, there is little in the literature as to the efficacy of this technique in patients who have received radiotherapy. To introduce an effective application of this technology, and highlight some advantages and disadvantages of its application in the irradiated craniofacial skeleton, we present a case of distraction osteogenesis of the orbitozygomatic complex in a patient with radiation induced orbitozygomatic hypoplasia.

  20. Bone marrow hypoplasia associated with fenbendazole administration in a dog.

    Science.gov (United States)

    Gary, Anthony T; Kerl, Marie E; Wiedmeyer, Charles E; Turnquist, Susan E; Cohn, Leah A

    2004-01-01

    A 1.5-year-old Doberman pinscher was presented with sudden-onset of fever and malaise. Twelve days prior to presentation, fenbendazole therapy was initiated for a suspected lungworm infection. Results of a complete blood count on presentation showed pancytopenia, while histopathological evaluation of a bone marrow core sample revealed bone marrow hypoplasia of undetermined etiology. Bactericidal antibiotics and fluid therapy, as well as discontinuation of fenbendazole administration, led to a complete resolution of clinical and hematological abnormalities within 15 days. An idiosyncratic reaction to fenbendazole was suspected based on the absence of infectious, neoplastic, autoimmune, and toxic etiologies, as well as resolution of clinical signs and pancytopenia upon drug withdrawal.

  1. Internal jugular vein thrombosis associated with venous hypoplasia and protein S deficiency revealed by ultrasonography.

    Science.gov (United States)

    Lim, Byung Gun; Kim, Young Min; Kim, Heezoo; Lim, Sang Ho; Lee, Mi Kyoung

    2011-12-01

    A 41-year-old woman, who had no thrombotic risk factors and past history except congenital scoliosis, underwent central venous catheterization (CVC) before correction of the scoliosis. When internal jugular vein (IJV) catheterization using the anatomical landmark technique failed, CVC under ultrasound guidance was tried. As a consequence, thrombosis and hypoplasia of the right IJV were incidentally detected by ultrasonography. Central venous catheters were then successfully placed in other veins under ultrasound guidance. Also, after examinations to rule out the possibility of pulmonary embolism and to clarify the causes of the IJV thrombosis, the patient was found to have protein S deficiency. CVC under ultrasound guidance should be recommended to prevent the failure of cannulation and complications such as thromboembolism in patients who could possibly have anomalies of vessels as a result of anatomical deformities caused by severe scoliosis, even if patients do not have thrombotic risk factors such as a history of central catheter insertion or intravenous drug abuse, cancer, advanced age, cerebral infarction, and left ventricular dysfunction. Also, if venous thrombosis is found in patients without predisposing risk factors, one should ascertain the cause of the hypercoagulable state, for example protein S deficiency, and perform appropriate treatment and prevention of venous thromboembolism.

  2. Pulmonary Hypoplasia Caused by Fetal Ascites in Congenital Cytomegalovirus Infection Despite Fetal Therapy

    Directory of Open Access Journals (Sweden)

    Kazumichi Fujioka

    2017-11-01

    Full Text Available We report two cases of pulmonary hypoplasia due to fetal ascites in symptomatic congenital cytomegalovirus (CMV infections despite fetal therapy. The patients died soon after birth. The pathogenesis of pulmonary hypoplasia in our cases might be thoracic compression due to massive fetal ascites as a result of liver insufficiency. Despite aggressive fetal treatment, including multiple immunoglobulin administration, which was supposed to diminish the pathogenic effects of CMV either by neutralization or immunomodulatory effects, the fetal ascites was uncontrollable. To prevent development of pulmonary hypoplasia in symptomatic congenital CMV infections, further fetal intervention to reduce ascites should be considered.

  3. Osteopathia striata: a characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

    Energy Technology Data Exchange (ETDEWEB)

    Barthels, W.; Boepple, D.; Petzel, H.

    1982-12-01

    Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality.

  4. Hypoplasia of bone marrow and cytopenia in non-hemic diseases

    International Nuclear Information System (INIS)

    Turbina, N.S.

    1987-01-01

    Common (stereotype) and different links in pathogenesis of hemodepressions pronounced in the form of cytopenia and/or hypoplasia of hemopoiesis are considered. Pathogenesis and signs of the diseases, as well as their diagnosis, are studied in detail

  5. Clinical Features and Management of Cartilage-Hair Hypoplasia: A Narrative Review

    Directory of Open Access Journals (Sweden)

    Kobra Shiasi Arani

    2015-01-01

    Full Text Available Context: Cartilage-hair hypoplasia is a rare hereditary cause of short stature. The aim of this study was to familiarize physicians with this rare but important disease. Evidence Acquisition: This article is a narrative review of the scientific literature to inform about clinical features and management of Cartilage-hair hypoplasia. A systematic search identified 127 papers include original and review articles and case reports. Results: Cartilage-Hair Hypoplasia characterized by short-limb dwarfism associated with metaphyseal chondrodysplasia. The inheritance is autosomal recessive. Other findings include hair hypoplasia, anemia, immunodeficiency, propensity to infections, gastrointestinal disorders (Hirschsprung disease, anal stenosis, esophageal atresia and malabsorption, defective spermatogenesis, increased risk of malignancies and higher rate of mortality. Immunodeficiency in cartilage-hair hypoplasia may be an isolated B-cell or isolated T-cell immunodeficiency or combined B and T-cell immunodeficiency; however, severe combined immunodeficiency is rare. There is no known treatment for hair hypoplasia. Growth hormone was used with conflicting results for short stature in children with Cartilage-hair hypoplasia. Skeletal problems must be managed with physiotherapy and appropriate orthopedic interventions. Hirschsprung disease, anal stenosis and esophageal atresia should be surgically corrected. Patients with severe hypoplastic anemia require repeated transfusions. Bone marrow transplantation may be required for patients with severe combined immunodeficiency or severe persistent hypoplastic anemia. Treatment with G-CSF is useful for neutropenia. Patients should be monitored closely for developing malignancy such as skin neoplasms, lymphomas and leukemias. Conclusions: Cartilage-hair hypoplasia is an important hereditary disease with different medical aspects. The high rate of consanguineous marriages in Iran necessitates considering CHH in any

  6. Cerebellar Hypoplasia and Dysmorphia in Neurofibromatosis Type 1.

    Science.gov (United States)

    Toelle, Sandra P; Poretti, Andrea; Weber, Peter; Seute, Tatjana; Bromberg, Jacoline E C; Scheer, Ianina; Boltshauser, Eugen

    2015-12-01

    Unidentified bright objects (UBO) and tumors are well-known cerebellar abnormalities in neurofibromatosis type 1 (NF1). Literature reports on malformative cerebellar anomalies in neurofibromatosis type 1 (NF1), however, are scant. We retrospectively studied the clinical and neuroimaging findings of 5 patients with NF1 (4 females, age 6 to 29 years at last follow-up) and cerebellar anomalies. Cerebellar symptoms on neurological examination were mild or even not evident whereas learning disabilities were more or less pronounced in four patients. Two patients had cerebellar hypoplasia (diffusely enlarged cerebellar interfoliar spaces) and three cerebellar dysmorphias involving mainly one cerebellar hemisphere. In NF1, malformative cerebellar anomalies are rare (estimated prevalence of about 1%), but most likely underestimated and easily overlooked, because physicians tend to focus on more prevalent, obvious, and well-known findings such as optic pathway gliomas, other tumors, and UBO. This kind of cerebellar anomaly in NF1 has most likely a malformative origin, but the exact pathogenesis is unknown. The individual clinical significance is difficult to determine. We suggest that cerebellar anomalies should be systematically evaluated in neuroimaging studies of NF1 patients.

  7. Regional disc change in segmental hypoplasia of the lumbosacral vertebral bodies: MR findings

    International Nuclear Information System (INIS)

    Kim, Sung Kyu; Lee Seung Ro; Moon, Won Jin; Park, Dong Woo; Hahm, Chang Kok

    2000-01-01

    To classify types of vertebral hypoplasia and to investigate the prevalence and patterns of associated disc degeneration. Defining vertebral hypoplasia as occurring when the AP diameter of a lower vertebral body is smaller than that of an upper ones, we retrospectively reviewed the MR images obtained in 34 cases of this condition involving young adults. Two major types and two subtypes, a total of four different entities were classified as follows; type I: hypoplasia involving a single vertebral body; type II: hypoplasia involving serial lower segmental vertebral bodies; subtype a: hypoplastic body located anteriorly along the anterior spinal line; subtype b: hypoplastic body located posteriorly along the posterior spinal line. We also investigated each type of vertebral hypoplasia and patterns of associated disc changes. Three different types were observed. In type IIa (n=3D29), posterior disc occurred in 8/29 cases, diffuse degeneration in 21/29 patients, and posterior disc herniation in all. All type Ia cases (3/3) showed diffuse disc degeneration at both upper and lower disc levels, with posterior disc herniation, while both type IIb cases (2/2) showed diffuse disc degeneration, with bidirectional disc herniation. By identifying the exact patterns of vertebral hypoplasia, we were able to predict which portion of the disc was likely to degenerate. (author)

  8. Evaluation of a new irradiation left breast method in the inhalation phase

    International Nuclear Information System (INIS)

    Silva, Laura E. da; Gullo, Rafael G.; Ferreira, Diogo A.V.; Silva, Leonardo P. da

    2016-01-01

    Radiation therapy is the primary therapeutic approach to breast cancer and involves significant exposure of heart and lungs, especially in cases of left breast. The implementation of a methodology to reduce the dose in these sites is important to ensure better quality of life to the patient. This work aims reduced heart and lung dose when performing the radiotherapy planning considering only the inhalation phase. Three patients with breast left present were scanning with CT during free breathing and respiratory monitoring and the planning has done on both images series. The results showed a reduction in mean heart dose of 53% on average. As for lung volumes, was obtained a mean reduction of 44.6% and 51% of the left lung which received 10 Gy and 20 Gy, respectively. This study showed that the use of the respiratory gating radiotherapy in the left breast in inhalation phase can reduce heart and lung doses. (author)

  9. Use of the Amplatzer ASD Occluder for Closing a Persistent Left Vertical Vein

    International Nuclear Information System (INIS)

    Zanchetta, Mario; Zennaro, Marco; Zecchel, Roberto; Mancuso, Daniela; Pedon, Luigi

    2009-01-01

    We report the case of a very large anomalous connection of the veins draining the upper lobe of the left lung to both the left-sided vertical vein and the left atrium, associated with mild rheumatic mitral valve stenosis, in which the atrial septum was intact and the remaining venous system, including the coronary sinus, was otherwise normal (a variant of Lutembacher's syndrome). In order to abolish the left-to-right shunting, a transcatheter approach to close this venous structure was successfully attempted using an Amplatzer ASD Occluder device. The technical aspects and the alternative options of performing a procedure with a device for a purpose outside the scope of its approved label are discussed.

  10. CT findings of a displaced left upper division bronchus in adults: Its importance for performing safe left pulmonary surgery

    Energy Technology Data Exchange (ETDEWEB)

    Oshiro, Yasuji, E-mail: oshiro4211@yahoo.co.jp [Department of Radiology, National Hospital Organization Okinawa Hospital, 20-14 Ganeko 3-chome, Ginowan city, Okinawa 901-2214 (Japan); Murayama, Sadayuki [Department of Radiology, University of the Ryukus School of Medicine, 207 Uehara, Nishihara-cho, Okinawa 903-0215 (Japan); Ohta, Morio [Department of Surgery, Nakagami Hospital, 6-25-5 Chibana, Okinawa-city, Okinawa 904-2195 (Japan); Teruya, Takao [Second Department of Surgery, University of the Ryukus School of Medicine, 207 Uehara, Nishihara-cho, Okinawa 903-0215 (Japan)

    2013-08-15

    Purpose: The aim of this study was to describe the CT findings of a displaced left upper division bronchus (DLUDB) in adults. Materials and methods: Ten patients with DLUDB were identified. The following CT features were assessed: origin of the DLUDB; distance between the origin of the DLUDB and the origin of the left upper lobe (LUL) bronchus; height of the origin of the DLUDB against the left pulmonary artery (LPA); difference of the main bronchial length; ventilated segment; course of the left pulmonary artery against the DLUDB; and presence of an accessory fissure or other anomalies. Results: DLUDB arose from the posterolateral or lateral aspect of the left main bronchus immediately proximal to the origin of the LUL bronchus. It tended to course along the posterior wall of the LPA and to ventilate the apicoposterior segment with or without the anterior segment. The LPA passed between the displaced bronchus and the lingular bronchus. The origin of the DLUDB was located lower than the inferior wall of the proximal LPA in 6 patients. The accessory fissure between the associated segment and remaining part of the LUL and right tracheal bronchus coexisted in 7 and 3 patients respectively. Conclusion: DLUDB has characteristic findings on CT. Radiologists should be aware of this entity and inform the surgeon as it can prevent serious complications in a patient who may undergo lobectomy of the left lung.

  11. CT findings of a displaced left upper division bronchus in adults: Its importance for performing safe left pulmonary surgery

    International Nuclear Information System (INIS)

    Oshiro, Yasuji; Murayama, Sadayuki; Ohta, Morio; Teruya, Takao

    2013-01-01

    Purpose: The aim of this study was to describe the CT findings of a displaced left upper division bronchus (DLUDB) in adults. Materials and methods: Ten patients with DLUDB were identified. The following CT features were assessed: origin of the DLUDB; distance between the origin of the DLUDB and the origin of the left upper lobe (LUL) bronchus; height of the origin of the DLUDB against the left pulmonary artery (LPA); difference of the main bronchial length; ventilated segment; course of the left pulmonary artery against the DLUDB; and presence of an accessory fissure or other anomalies. Results: DLUDB arose from the posterolateral or lateral aspect of the left main bronchus immediately proximal to the origin of the LUL bronchus. It tended to course along the posterior wall of the LPA and to ventilate the apicoposterior segment with or without the anterior segment. The LPA passed between the displaced bronchus and the lingular bronchus. The origin of the DLUDB was located lower than the inferior wall of the proximal LPA in 6 patients. The accessory fissure between the associated segment and remaining part of the LUL and right tracheal bronchus coexisted in 7 and 3 patients respectively. Conclusion: DLUDB has characteristic findings on CT. Radiologists should be aware of this entity and inform the surgeon as it can prevent serious complications in a patient who may undergo lobectomy of the left lung

  12. Two cases of pontocerebellar hypoplasia: ethical and prenatal diagnostic dilemma.

    Science.gov (United States)

    Ajibola, Ayodeji J; Netzloff, Michael; Samaraweera, Ranji; Omar, Said A

    2010-02-01

    We report the clinical characteristics and the outcome of two cases of pontocerebellar hypoplasia (PCH) in one family. The objective of this report is to describe the mode of presentation, discuss the clinical course, and address the dilemma of prenatal diagnosis and the prospects for genetic diagnosis for PCH. The first case is a 4-year-old boy in whom the diagnosis was made in the neonatal period. Despite extensive prenatal follow-up during the mother's subsequent pregnancy, prenatal diagnosis could not be made and a second affected child was born. Both siblings have severe developmental delay. The cases raise an important ethical dilemma about the most appropriate intervention if the mother of a child affected with PCH becomes pregnant. PCH is considered to have an autosomal-recessive mode of inheritance and a recurrence risk of 25% in each pregnancy. Until recently when genetic mutations in PCH types 2, 4, and 6 began to be identified, the lack of well-recognized genetic testing precluded experts from making clear recommendations. The best advice to these parents was difficult or elusive. With two children currently affected, should the parents terminate or continue with the latest pregnancy? Extensive monitoring with serial prenatal ultrasound failed in the previous pregnancy and resulted in the birth of the second affected child. It is evident that serial ultrasound scan may not be helpful in making the diagnosis prenatally. Therefore, other diagnostic modalities such as magnetic resonance imaging may be necessary and should be considered. With the identification of genetic basis or mutations in PCH types 2, 4, and 6 and possible development of commercial genetic testing for these types of PCH, reproductive decision or genetic testing during pregnancy should be recommended to affected families to enable informed choices. Thieme Medical Publishers.

  13. Internal Carotid Artery Hypoplasia: Role of Color-Coded Carotid Duplex Sonography.

    Science.gov (United States)

    Chen, Pei-Ya; Liu, Hung-Yu; Lim, Kun-Eng; Lin, Shinn-Kuang

    2015-10-01

    The purpose of this study was to determine the role of color-coded carotid duplex sonography for diagnosis of internal carotid artery hypoplasia. We retrospectively reviewed 25,000 color-coded carotid duplex sonograms in our neurosonographic database to establish more diagnostic criteria for internal carotid artery hypoplasia. A definitive diagnosis of internal carotid artery hypoplasia was made in 9 patients. Diagnostic findings on color-coded carotid duplex imaging include a long segmental small-caliber lumen (52% diameter) with markedly decreased flow (13% flow volume) in the affected internal carotid artery relative to the contralateral side but without intraluminal lesions. Indirect findings included markedly increased total flow volume (an increase of 133%) in both vertebral arteries, antegrade ipsilateral ophthalmic arterial flow, and a reduced vessel diameter with increased flow resistance in the ipsilateral common carotid artery. Ten patients with distal internal carotid artery dissection showed a similar color-coded duplex pattern, but the reductions in the internal and common carotid artery diameters and increase in collateral flow from the vertebral artery were less prominent than those in hypoplasia. The ipsilateral ophthalmic arterial flow was retrograde in 40% of patients with distal internal carotid artery dissection. In addition, thin-section axial and sagittal computed tomograms of the skull base could show the small diameter of the carotid canal in internal carotid artery hypoplasia and help distinguish hypoplasia from distal internal carotid artery dissection. Color-coded carotid duplex sonography provides important clues for establishing a diagnosis of internal carotid artery hypoplasia. A hypoplastic carotid canal can be shown by thin-section axial and sagittal skull base computed tomography to confirm the final diagnosis. © 2015 by the American Institute of Ultrasound in Medicine.

  14. Intertooth patterns of hypoplasia expression: implications for childhood health in the classic Maya collapse.

    Science.gov (United States)

    Wright, L E

    1997-02-01

    Enamel hypoplasias, which record interacting stresses of nutrition and illness during the period of tooth formation, are a key tool in the study of childhood health in prehistory. But interpretation of the age of peak morbidity is complicated by differences in susceptibility to stress both between tooth positions and within a single tooth. Here, hypoplasias are used to evaluate the prevailing ecological model for the collapse of Classic Period Lowland Maya civilization, circa AD 900. Hypoplasias were recorded in the full dentition of 160 adult skeletons from six archaeological sites in the Pasion River region of Guatemala. Instead of constructing a composite scale of stress experience, teeth are considered separately by position in the analysis. No statistical differences are found in the proportion of teeth affected by hypoplasia between "Early," Late Classic, and Terminal Classic Periods for anterior teeth considered to be most susceptible to stress, indicating stability in the overall stress loads affecting children of the three chronological periods. However, hypoplasia trends in posterior teeth may imply a change in the ontogenetic-timing of more severe stress episodes during the final occupation and perhaps herald a shift in child-care practices. These results provide little support for the ecological model of collapse but do call to attention the potential of posterior teeth to reveal subtle changes in childhood morbidity when consideredindividually.

  15. Management of a case of left tracheal sleeve pneumonectomy under cardiopulmonary bypass: Anesthesia perspectives

    Directory of Open Access Journals (Sweden)

    Aman Jyoti

    2014-01-01

    Full Text Available The lung tumors with carinal involvement are frequently managed with tracheal sleeve pneumonectomy and tracheobronchial anastomosis without use of cardiopulmonary bypass (CPB. Various modes of ventilation have been described during tracheal resection and anastomosis. Use of CPB during this period allows the procedure to be conducted in a more controlled way. We performed tracheal sleeve pneumonectomy for adenoid cystic carcinoma of left lung involving carina. The surgery was performed in two stages. In the first stage, left pneumonectomy was performed and in the second stage after 48 h, tracheobronchial resection and anastomosis was performed under CPB. Second stage was delayed to avoid excessive bleeding (due to heparinization from the extensive vascular raw area left after pneumonectomy. Meticulous peri-operative planning and optimal post-operative care helped in successful management of a complex case, which is associated with high morbidity and mortality.

  16. Enamel hypoplasia and its role in identification of individuals: A review of literature

    Science.gov (United States)

    Kanchan, Tanuj; Machado, Meghna; Rao, Ashwin; Krishan, Kewal; Garg, Arun K.

    2015-01-01

    Identification of individuals is the mainstay of any forensic investigation especially in cases of mass disasters when mutilated remains are brought for examination. Dental examination helps in establishing the identity of an individual and thus, has played a vital role in forensic investigation process since long. In this regard, description on the role of enamel hypoplasia is limited in the literature. The present article reviews the literature on the enamel hypoplasia and discusses its utility in forensic identification. Enamel hypoplasia is a surface defect of the tooth crown caused by disturbance of enamel matrix secretion. Enamel defects can be congenital or acquired. In cases of mass disasters, or when the body is completely charred, putrefied and mutilated beyond recognition, the unique dental features can help in identification of the victims. PMID:26097340

  17. Pregnancy Outcome in Cartilage-Hair Hypoplasia, a Rare Form of Dwarfism

    Directory of Open Access Journals (Sweden)

    Harshithaa Thavarajah

    2017-01-01

    Full Text Available Background. This case report discusses the pregnancy outcome of a patient with cartilage-hair hypoplasia, a rare form of dwarfism, and multiple previous orthopedic surgeries. Literature on pregnancy outcomes in patients with cartilage-hair hypoplasia is limited. Case. A 32-year-old patient with cartilage-hair hypoplasia presented at 12 weeks’ gestation to the high-risk obstetrics clinic for care. Preterm labor resulted in cesarean delivery at 34 weeks’ gestation with general anesthetic. Breastfeeding was stopped at 6 weeks due to neonatal complications. Conclusion. Pregnancy and delivery were uncomplicated. A multidisciplinary approach allowed for effective management during pregnancy and postnatal care. This is the first known documented case of prenatal care, delivery, and breastfeeding in a woman with this rare disorder.

  18. Analysis of the enamel hypoplasia using micro-CT scanner versus classical method.

    Science.gov (United States)

    Marchewka, Justyna; Skrzat, Janusz; Wróbel, Andrzej

    2014-01-01

    This article demonstrates the use of micro-CT scanning of the teeth surface for recognizing and evaluating severity of the enamel hypoplasia. To test capabilities of the microtomography versus classical method of evaluation hypoplastic defects of the enamel we selected two human teeth (C, M(2)) showing different types of enamel hypoplasia: linear, pits, and groove. Examined samples derive from archeological material dated on XVII-XVIII AD and excavated in Poland. In the current study we proved that micro-CT scanning is a powerful technique not only for imaging all kinds of the enamel hypoplasia but also allows to perform accurate measurements of the enamel defects. We figure out that contrary to the classical method of scoring enamel defects, the micro-computed tomography yields adequate data which serve for estimating the length of stress episode and length of interval between them.

  19. Goldenhar Syndrome with Dextrocardia and Right Pulmonary Hypoplasia: An Unusual Association

    Directory of Open Access Journals (Sweden)

    Nagendra Chaudhary

    2017-01-01

    Full Text Available Goldenhar syndrome (GS, a rare condition, occurring due to defect in development of first and second branchial arches, is characterized by a combination of various anomalies involving face, eyes, ears, vertebrae, heart, and lungs. The etiology of GS is not fully known, although various hypotheses have been proposed along with its genetic association and many other causes. Facial asymmetry and hypoplasia of the mandible are characteristic features of GS along with microtia and preauricular appendages and pits. Dextrocardia or pulmonary hypoplasia in GS has previously been reported separately. We report a 7-year-old female child of GS with combination of anomalies, dextrocardia, and pulmonary hypoplasia, which is a rare association.

  20. Use of repeat anterior maxillary distraction to correct residual midface hypoplasia in cleft patients.

    Science.gov (United States)

    Richardson, Sunil; Krishna, Shreya; Bansal, Avi

    2017-12-01

    The study was designed to evaluate the efficacy of performing a second, repeat anterior maxillary distraction (AMD) to treat residual cleft maxillary hypoplasia. Five patients between the ages of 12 to 15 years with a history of AMD and with residual cleft maxillary hypoplasia were included in the study. Inclusion was irrespective of gender, type of cleft lip and palate, and the amount of advancement needed. Repeat AMD was executed in these patients 4 to 5 years after the primary AMD procedure to correct the cleft maxillary hypoplasia that had developed since the initial procedure. Orthopantomogram (OPG) and lateral cephalograms were taken for evaluation preoperatively, immediately after distraction, after consolidation, and one year postoperatively. The data obtained was tabulated and a Mann Whitney U-test was used for statistical comparisons. At the time of presentation, a residual maxillary hypoplasia was observed with a well maintained distraction gap on the OPG which ruled out the occurrence of a relapse. Favorable movement of the segments without any resistance was seen in all patients. Mean maxillary advancement of 10.56 mm was achieved at repeat AMD. Statistically significant increases in midfacial length, SNA angle, and nasion perpendicular to point A distance was achieved ( P =0.012, P =0.011, and P =0.012, respectively). Good profile was achieved for all patients. Minimal transient complications, for example anterior open bite and bleeding episodes, were managed. Addressing the problem of cleft maxillary hypoplasia at an early age (12-15 years) is beneficial for the child. Residual hypoplasia may develop in some patients, which may require additional corrective procedures. The results of our study show that AMD can be repeated when residual deformity develops with the previous procedure having no negative impact on the results of the repeat procedure.

  1. X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

    Directory of Open Access Journals (Sweden)

    Metwalley Kotb

    2012-12-01

    Full Text Available Abstract Introduction X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1 gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1 gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature. Case presentation A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized. Conclusions We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due

  2. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

    1998-01-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  3. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J.

    1998-01-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases

  4. Left-Right Asymmetry in Spectral Characteristics of Lung Sounds Detected Using a Dual-Channel Auscultation System in Healthy Young Adults.

    Science.gov (United States)

    Tsai, Jang-Zern; Chang, Ming-Lang; Yang, Jiun-Yue; Kuo, Dar; Lin, Ching-Hsiung; Kuo, Cheng-Deng

    2017-06-07

    Though lung sounds auscultation is important for the diagnosis and monitoring of lung diseases, the spectral characteristics of lung sounds have not been fully understood. This study compared the spectral characteristics of lung sounds between the right and left lungs and between healthy male and female subjects using a dual-channel auscultation system. Forty-two subjects aged 18-22 years without smoking habits and any known pulmonary diseases participated in this study. The lung sounds were recorded from seven pairs of auscultation sites on the chest wall simultaneously. We found that in four out of seven auscultation pairs, the lung sounds from the left lung had a higher total power (P T ) than those from the right lung. The P T of male subjects was higher than that of female ones in most auscultation pairs. The ratio of inspiration power to expiration power (R I/E ) of lung sounds from the right lung was greater than that from the left lung at auscultation pairs on the anterior chest wall, while this phenomenon was reversed at auscultation pairs on the posterior chest wall in combined subjects, and similarly in both male and female subjects. Though the frequency corresponding to maximum power density of lung sounds (F MPD ) from the left and right lungs was not significantly different, the frequency that equally divided the power spectrum of lung sounds (F 50 ) from the left lung was significantly smaller than that from the right lung at auscultation site on the anterior and lateral chest walls, while it was significantly larger than that of from the right lung at auscultation site on the posterior chest walls. In conclusion, significant differences in the P T , F MPD , F 50 , and R I/E between the left and right lungs at some auscultation pairs were observed by using a dual-channel auscultation system in this study. Structural differences between the left and the right lungs, between the female and male subjects, and between anterior and posterior lungs might

  5. Critically Underdeveloped Left Heart Morphology Associated with Prematurity and Low Birth Weight: Conditional Staged Rehabilitation Towards Biventricular Repair and Time-Related Growth of Left Heart Structures.

    Science.gov (United States)

    Ahmad, Fareed; Mangano, Robert; Shore, Shirah; Polimenakos, Anastasios

    2017-10-01

    This is a case report of premature low birth weight infant with hypoplasia of left heart structures and a large malaligned VSD who underwent successful staged approach of biventricular repair. We obtained qualitative and quantitative echocardiographic, MRI, and conventional catheterization data to support stepwise strategy towards LV rehabilitation to sustain adequate cardiac output. A thorough and intense follow-up has shown significant growth of left heart structures and favorable clinical status following staged biventricular repair. Our data indicate usefulness of qualitative and quantitative advanced complimentary multi-imaging modalities in predicting the postnatal growth potential of critically underdeveloped left heart structures.

  6. Growth hormone treatment in cartilage-hair hypoplasia: effects on growth and the immune system.

    NARCIS (Netherlands)

    Bocca, G.; Weemaes, C.M.R.; Burgt, C.J.A.M. van der; Otten, B.J.

    2004-01-01

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disorder characterized by metaphyseal chondrodysplasia with severe growth retardation and impaired immunity. We studied the effects of growth hormone treatment on growth parameters and the immune system in four children with CHH. The

  7. WDR73 missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in a consanguineous family.

    Science.gov (United States)

    Jiang, Chen; Gai, Nan; Zou, Yongyi; Zheng, Yu; Ma, Ruiyu; Wei, Xianda; Liang, Desheng; Wu, Lingqian

    2017-01-01

    Galloway-Mowat syndrome (GMS) is a very rare autosomal-recessive disorder characterized by nephrotic syndrome associated with microcephaly, and various central nervous system abnormalities, mostly cerebral hypoplasia or cerebellar atrophy, intellectual disability and neural-migration defects. WDR73 is the only gene known to cause GMS, and has never been implicated in other disease. Here we present a Chinese consanguineous family with infantile onset intellectual disability and cerebellar hypoplasia but no microcephaly. Whole exome sequencing identified a WDR73 p.W371G missense mutation. The mutation is confirmed to be segregated in this family by Sanger sequencing according to a recessive inheritance pattern. It is predicted to be deleterious by multiple algorithms and affect highly conserved site. Structural modeling revealed conformational differences between the wild type protein and the p.W371G protein. Real-time PCR and Western blotting revealed altered mRNA and protein levels in mutated samples. Our study indicates the novel WDR73 p.W371G missense mutation causes infantile onset intellectual disability and cerebellar hypoplasia in recessive mode of inheritance. Our findings imply that microcephaly is a variable phenotype in WDR73-related disease, suggest WDR73 to be a candidate gene of severe intellectual disability and cerebellar hypoplasia, and expand the molecular spectrum of WDR73-related disease. Copyright © 2016 Elsevier B.V. All rights reserved.

  8. CARTILAGE HAIR HYPOPLASIA, METAPHYSEAL CHONDRODYSPLASIA TYPE MCKUSICK - DESCRIPTION OF 7 PATIENTS AND REVIEW OF THE LITERATURE

    NARCIS (Netherlands)

    VANDERBURGT, [No Value; HARALDSSON, A; OOSTERWIJK, JC; VANESSEN, AJ; WEEMAES, C; HAMEL, B

    1991-01-01

    We describe 7 cases of cartilage hair hypoplasia (CHH) with emphasis on the clinical and immunological aspects. The literature on CHH is reviewed and symptoms in 63 non-Amish cases are summarized. In this autosomal recessive disorder the immunodeficiency, hair abnormalities, and severity of skeletal

  9. Osteopathia striata: A characteristic X-ray finding in focal dermal hypoplasia (Goltz-Gorlin syndrome)

    International Nuclear Information System (INIS)

    Barthels, W.; Boepple, D.; Petzel, H.

    1982-01-01

    Two cases of the very rare Goltz-Gorlin syndrome are presented. The relationship of osteopathia striata and focal dermal hypoplasia is discussed, and it is concluded that the osteopathia striata represents the characteristic picture of this ectopic mesodermal abnormality. (orig.)

  10. Mutations in X-linked PORCN, a putative regulator of Wnt signaling, cause focal dermal hypoplasia

    Science.gov (United States)

    Focal dermal hypoplasia is an X-linked dominant disorder characterized by patchy hypoplastic skin and digital, ocular, and dental malformations. We used array comparative genomic hybridization to identify a 219-kb deletion in Xp11.23 in two affected females. We sequenced genes in this region and fou...

  11. Thanatophoric dysplasia type II with encephalocele and aortic hypoplasia diagnosed in an anatomical specimen

    NARCIS (Netherlands)

    Jap-A-Joe, Simone M. E. A. A.; Oostra, Roelof-Jan; Maas, Mario; Stoker, Jaap; van der Horst, Chantal M. A. M.

    2003-01-01

    A hitherto unknown combination of congenital anomalies was found in an anatomical specimen of a female neonate. External examination and additional CT and MRI studies showed thanatophoric dysplasia type II with cloverleaf skull and concomitant parietal meningoencephalocele and hypoplasia of the

  12. Parvovirus associated cerebellar hypoplasia and hydrocephalus in day-old broiler chickens

    Science.gov (United States)

    Cerebellar hypoplasia and hydrocephalus were detected in day-old broiler chickens. Brains of chickens evaluated at necropsy appeared to be abnormal; some were disfigured and cerebellae appeared to be smaller than normal. Histopathologic examination of brains revealed cerebellar folia that were sho...

  13. Recessive Mutations in SLC38A8 Cause Foveal Hypoplasia and Optic Nerve Misrouting without Albinism

    NARCIS (Netherlands)

    Poulter, James A.; Al-Araimi, Musallam; Conte, Ivan; van Genderen, Maria M.; Sheridan, Eamonn; Carr, Ian M.; Parry, David A.; Shires, Mike; Carrella, Sabrina; Bradbury, John; Khan, Kamron; Lakeman, Phillis; Sergouniotis, Panagiotis I.; Webster, Andrew R.; Moore, Anthony T.; Pal, Bishwanath; Mohamed, Moin D.; Venkataramana, Anandula; Ramprasad, Vedam; Shetty, Rohit; Saktivel, Murugan; Kumaramanickavel, Govindasamy; Tan, Alex; Mackey, David A.; Hewitt, Alex W.; Banfi, Sandro; Ali, Manir; Inglehearn, Chris F.; Toomes, Carmel

    2013-01-01

    Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism

  14. Bilateral familial vertical Duane Syndrome with synergistic convergence, aberrant trigeminal innervation, and facial hypoplasia

    Directory of Open Access Journals (Sweden)

    Malvika Gupta

    2014-01-01

    Full Text Available A 5-year-old girl presented with bilateral familial vertical  Duane retraction syndrome with alternating esotropia, elevation deficit, Marcus gunn phenomenon, and facial hypoplasia. Abnormal adducting downshoots on attempting abduction suggestive of a synergistic convergence were noted. Hypothesis suggests aberrant innervations or peripheral anatomic connections between inferior and medial recti.

  15. SU-E-T-532: Left-Sided Breast Cancer Irradiation Using Volumatric Modulated Arc Therapy: An Evaluation of Multiple Commercial Systems

    International Nuclear Information System (INIS)

    Liu, R; Liu, T; Qi, S

    2015-01-01

    Purposes: There has been growing interest in treating breast cancer using VMAT technique. Our goal is to compare the dosimetry and treatment delivery parameters for the left-sided breast cancer treatment using various VMAT platforms from commercially available planning systems. Methods: Five consecutive left-sided breast cancer patients initially treated with conventional 3D-conformal radiotherapy (3DCRT) were selected. Four VMAT plans using most popular treatment planning systems, including Eclipse (Version 11, Varian), Pinnacle (Version 9.8, Philips), Monaco (Version 2.03, Elekta) and helical Tomotherapy (V4.0, Accuray). The same structure set and same planning goals were used for all VMAT plans. The dosimetric parameters including target coverage and minimum/maximum/mean, dose-volume endpoints for the selected normal structures: the heart, ipsilateral-/contralateral lung and breast, were evaluated. Other dosimetric indices including heterogeneity index (HI) were evaluated. The treatment delivery parameters, such as monitor unit (MUs) and delivery time were also compared. Results: VMAT increases dose homogeneity to the treated volume and reduces the irradiated heart and left-lung volumes. Compared to the 3DCRT technique, all VMAT plans offer better heart and left-lung dose sparing; the mean heart doses were 4.5±1.6(Monaco), 1.2±0.4(Pinnacle), 1.3± (Eclipse) and 5.6±4.4(Tomo), the mean left-lung doses were 5.9±1.5(Monaco), 3.7±0.7(Pinnacle), 1.4± (Eclipse) and 5.2±1.6 (Tomo), while for the 3DCRT plan, the mean heart and left-Lung doses were 2.9±2.0, and 6.8±4.4 (Gy) respectively. The averaged contralateral-breast and lung mean doses were higher in VMAT plans than the 3DCRT plans but were not statistically significant. Among all the VMAT plans, the Pinnacle plans often yield the lowest right-lung/breast mean doses, and slightly better heterogeneity indices that are similar to Tomotherapy plans. Treatment delivery time of the VMAT plans (except helical

  16. Patient-Reported Esthetic and Functional Outcomes of Primary Total Laparoscopic Intestinal Vaginoplasty in Transgender Women With Penoscrotal Hypoplasia

    NARCIS (Netherlands)

    Bouman, M.B.; Sluis, W.B. van der; Woudenberg Hamstra, L.E. van; Buncamper, M.E.; Kreukels, B.P.; Meijerink, W.J.H.J.; Mullender, M.G.

    2016-01-01

    INTRODUCTION: Puberty-suppressing hormonal treatment may result in penoscrotal hypoplasia in transgender women, making standard penile inversion vaginoplasty not feasible. For these patients, intestinal vaginoplasty is a surgical alternative, but knowledge on patient-reported postoperative outcomes

  17. MRI and three dimensional ultrasonography in the assessment of pulmonary hypoplasia in fetuses with urinary tract anomalies

    Directory of Open Access Journals (Sweden)

    Mariam Raafat

    2016-12-01

    Conclusion: There is a good concordance between 3D-US and MRI in the evaluation of PH in fetuses with UTM. MRI could be reserved for borderline cases of pulmonary hypoplasia and the difficult diagnostic situations.

  18. Hypoplastic left heart syndrome and pulmonary veno-occlusive disease in an infant.

    Science.gov (United States)

    D'Souza, Marise; Vergales, Jeffrey; Jayakumar, K Anitha

    2013-01-01

    This report describes an infant with heterotaxy syndrome and severe hypoplasia of the left heart who presented with profound cyanosis at birth despite a large patent ductus arteriosus. Pulmonary venous return was difficult to demonstrate by echocardiography. Angiography showed total anomalous pulmonary venous return via a plexus that drained through the paravertebral veins and bilateral superior vena cavae. Autopsy confirmed these findings, and histopathology demonstrated severe occlusive changes within the pulmonary veins.

  19. Management of Cleft Maxillary Hypoplasia with Anterior Maxillary Distraction: Our Experience

    OpenAIRE

    Chacko, Tojan; Vinod, Sankar; Mani, Varghese; George, Arun; Sivaprasad, K. K.

    2013-01-01

    Maxillary hypoplasia is a common developmental problem in cleft lip and palate deformities. Since 1970s these deformities have traditionally been corrected by means of orthognathic surgery. Management of skeletal deformities in the maxillofacial region has been an important challenge for maxillofacial surgeons and orthodontists. Distraction osteogenesis is a surgical technique that uses body’s own repairing mechanisms for optimal reconstruction of the tissues. We present four cases of anterio...

  20. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

    International Nuclear Information System (INIS)

    Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

    2015-01-01

    Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

  1. Bilateral congenital absence of flexor pollicis longus with thumb hypoplasia and thenar atrophy

    International Nuclear Information System (INIS)

    Chaudhary, Vikas; Sehgal, Harsha; Bano, Shahina; Parmar, Pranjali R; Kumar, Sanjay

    2014-01-01

    Congenital absence of flexor pollicis longus with or without associated anomalies of thenar muscles and thumb is of rare occurrence. Inability to flex the interphalangeal joint of the thumb and absent dorsal wrinkles and flexion creases of the thumb are important clues to the diagnosis. Routine radiography and cross-sectional imaging help to confirm and document the condition. This article presents an extremely rare case of bilateral congenital absence of flexor pollicis longus tendon with thumb hypoplasia and thenar atrophy

  2. Correction of axial deformity during lengthening in fibular hypoplasia: Hexapodal versus monorail external fixation.

    Science.gov (United States)

    Chalopin, A; Geffroy, L; Pesenti, S; Hamel, A; Launay, F

    2017-09-01

    Childhood fibular hypoplasia is a rare pathology which may or may not involve limb-length discrepancy and axial deformity in one or more dimensions. The objective of the present study was to compare the quality of the axial correction achieved in lengthening procedures by hexapodal versus monorail external fixators. The hypothesis was that the hexapodal fixator provides more precise correction. A retrospective multicenter study included 52 children with fibular hypoplasia. Seventy-two tibias were analyzed, in 2 groups: 52 using a hexapodal fixator, and 20 using a monorail fixator. Mean age was 10.2 years. Mean lengthening was 5.7cm. Deformities were analyzed and measured in 3 dimensions and classified in 4 preoperative types and 4 post-lengthening types according to residual deformity. Complete correction was achieved in 26 tibias in the hexapodal group (50%) and 2 tibias in the monorail group (10%). Mean post-correction mechanical axis deviation was smaller in the hexapodal group: 12.83mm, versus 14.29mm in the monorail group. Mean post-correction mechanical lateral distal femoral angle was 87.5° in the hexapodal group, versus 84.3° in the monorail group (P=0.002), and mean mechanical medial proximal tibial angle 86.9° versus 89.5°, respectively (P=0.015). No previous studies focused on this congenital pathology in lengthening and axial correction programs for childhood lower-limb deformity. The present study found the hexapodal fixator to be more effective in conserving or restoring mechanical axes during progressive bone lengthening for fibular hypoplasia. The hexapodal fixator met the requirements of limb-length equalization in childhood congenital lower-limb hypoplasia, providing better axial correction than the monorail fixator. IV. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  3. Unilateral Pulmonary Artery Agenesis with Ipsilateral Pulmonary Hypoplasia as Incidental Finding in an Asthmatic Patient

    Directory of Open Access Journals (Sweden)

    Catalina Contreras-Arias

    2014-01-01

    Full Text Available Unilateral absence of a pulmonary artery is an uncommon congenital heart disease. It can be related to respiratory symptoms such as asthma, an unsual nding in some of these patients. This paper reports the case of a 4-year-old male with recurrent respiratory infections and asthma symptoms, in who further studies found agenesia of right pulmonary artery with pulmonary hypoplasia of the same side.

  4. Generalized seizures in the right hippocampus sclerosis combined with hypoplasia of the right vertebral artery

    International Nuclear Information System (INIS)

    Manchev, L.; Toneva, J.; Manolova, T.; Manchev, I.; Valcheva, V.

    2016-01-01

    We present a clinical case of generalized epileptic seizures, occurring suddenly. The common finding from MRI of the brain is sclerosis of the right hippocampus, while MR angiography shows hypoplasia of the right vertebral artery. There are EEG signs for single foci of abnormal activity more on the right side. An anticonvulsant and symptomatic treatment demonstrate a favorable result. Under discussion is the question of surgery treatment. Key words: Hippocampal Sclerosis. MRI. Epileptic Seizures

  5. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos

    Energy Technology Data Exchange (ETDEWEB)

    Zhang, Ting, E-mail: zting@webmail.hzau.edu.cn; Zhou, Xin-Ying, E-mail: 290356082@qq.com; Ma, Xu-Fa, E-mail: xufama@mail.hzau.edu.cn; Liu, Jing-Xia, E-mail: ichliu@mail.hzau.edu.cn

    2015-10-15

    Highlights: Using high-throughput in situ hybridization screening, we found that genes labeling the neural crest and its derivative pigment cells were sensitive to cadmium toxicity during zebrafish organogenesis, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in cadmium-exposed embryos. Based on neural crest markers, we identified the doses and times of cadmium exposure that cause damage to the zebrafish organogenesis, and we also found that compounds BIO or RA could neutralize the toxic effects of cadmium. - Abstract: Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd.

  6. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Osama M Sarhan

    2013-01-01

    Full Text Available Association between Prune belly syndrome (PBS and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

  7. Prune belly syndrome with urethral hypoplasia and vesico-cutaneous fistula: A case report and review of literature.

    Science.gov (United States)

    Sarhan, Osama M; Al-Ghanbar, Mustafa S; Nakshabandi, Ziad M

    2013-10-01

    Association between Prune belly syndrome (PBS) and urethral hypoplasia is an unusual condition. It is usually fatal unless there is a communication between the fetal bladder and the amniotic sac. We report a case of PBS with urethral hypoplasia and congenital vesico-cutaneous fistula in a male neonate. Patient underwent cutaneous vesicostomy and was discharged for close follow up of his renal function and for future reconstruction.

  8. Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

    Science.gov (United States)

    Fryns, J P; Moerman, P

    1993-01-01

    A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia). Images PMID:8487282

  9. Short limbed dwarfism, genital hypoplasia, sparse hair, and vertebral anomalies: a variant of Ellis-van Creveld syndrome?

    OpenAIRE

    Fryns, J P; Moerman, P

    1993-01-01

    A male newborn with acromesomelic short limbed dwarfism, genital hypoplasia, and vertebral anomalies is reported. As the child had an important number of clinical and radiological symptoms seen in patients with Ellis-van Creveld syndrome, we raise the question of whether he may represent a variant example of this syndrome despite the absence of cardinal symptoms such as postaxial polydactyly and ectodermal changes (nail hypoplasia).

  10. [Risk factors for teeth aplasia and hypoplasia in cleft lip and palate children].

    Science.gov (United States)

    Korolenkova, M V; Starikova, N V; Ageeva, L V

    2016-01-01

    The aim of the study was to assess the significance of environmental risk factors for teeth aplasia and hypoplasia in cleft lip and palate children. Two hundred and forty-seven cleft lip and palate (CLP) children were enrolled in the study including 105 (42.5%) with bilateral CLP and 57.5% with unilateral CLP. The mean age was 11.2±4.9 years. Teeth condition was assessed clinically and radiologically. The impact of risk factors for teeth anomalies was analyzed by retrospective data obtained from computer database (absence of preoperative orthopedic treatment, palatal defects after primary palatoplasty and type of primary procedures). Surgical trauma by early periosteoplasty (at the age of 3-4 months), excessive scarring and tissue traction due to absence of early orthopedic treatment and palatal defect were associated with significantly higher incidence of incisors hypoplasia (both developmental enamel defects and microdentia) and aplasia of central incisors not seen in the other study subgroups. Incisors aplasia and hypoplasia in CLP patients do not always have disembryogenic origin but may depend on external environmental factors, including surgical trauma.

  11. Urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome: long-term results.

    Science.gov (United States)

    Kajbafzadeh, Abdol-Mohammad; Rasouli, Mohammad Reza; Dianat, SeyedSaeid; Nezami, Behtash G; Mahboubi, Amir Hassan; Sina, Alireza

    2010-11-01

    The aim of the study was to evaluate the efficacy and safety of urethral hydrodistension for management of urethral hypoplasia in prune belly syndrome (PBS). During a 10-year period, 7 infants with PBS and urethral hypoplasia presented either with open urachus or surgically created urinary diversion referred to our hospital. Five milliliters of normal saline was pushed via a 22-gauge plastic angiocatheter into the urethra with simultaneous finger pressure on the perineum to occlude the proximal urethra that was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. Median age of the infants was 6 (1-8) months. All urethral hydrodistension were successful after 1 to 3 sessions. Follow-up imaging studies showed significant improvement in all patients except one. Natural and surgically created urinary diversions were closed in 6 infants. The hydrodistension create an equal and constant pressure into the urethral wall without any urethral damage. This technique can be considered along with the other available methods for management of urethral hypoplasia in selected cases of PBS. Copyright © 2010 Elsevier Inc. All rights reserved.

  12. Diffuse large B-cell lymphoma chemotherapy reveals a combined immunodeficiency syndrome in cartilage hair hypoplasia.

    Science.gov (United States)

    Nguyen, Alexandre; Martin Silva, Nicolas; de Boysson, Hubert; Damaj, Gandhi; Aouba, Achille

    2018-04-24

    Cartilage hair hypoplasia (CHH) is a rare autosomal recessive ribosomopathy characterised by skeletal and integumentary system manifestations. It may also present with varied forms and intensities of haematopoietic and/or immune disorders. We report a 27-year-old female who presented a picture of combined immunodeficiency after receiving an adriamycin-based chemotherapy regimen followed by autologous stem cell transplantation. Her medical history indicated neonatal dwarfism, recurrent ear, nose and throat and respiratory infections, and hypogammaglobulinaemia, which were suggestive of a primary minor B-cell immune deficiency. Taken together, the diagnosis of cartilage hair hypoplasia was suspected and confirmed by means of molecular biological analysis. Here, we discuss the causal relationship and molecular mechanisms existing between both primary immunodeficiency and lymphoma conditions and between chemotherapy cytotoxicity and aggravation of the immune system and associated hematopoietic dysfunction, considering the role of all these components in light of the initially undiagnosed cartilage hair hypoplasia. Finally, this case highlights the importance of screening for primary immunodeficiencies in the setting of a diagnosis of lymphoma and/or dwarfism; moreover, CHH must be distinguished from other causes of small size; its diagnosis and complete check-up must include the molecular characterisation, and its management must be global in collaboration with haematologists, immunologists and internists.

  13. [Pulmonary hypoplasia: An analysis of cases over a 20-year period].

    Science.gov (United States)

    Delgado-Peña, Yanny Paola; Torrent-Vernetta, Alba; Sacoto, Gabriela; de Mir-Messa, Inés; Rovira-Amigo, Sandra; Gartner, Silvia; Moreno-Galdó, Antonio; Molino-Gahete, José Andrés; Castillo-Salinas, Felíx

    2016-08-01

    Pulmonary hypoplasia is the most frequent congenital anomaly associated with perinatal mortality. A retrospective and descriptive review was conducted on cases of patients diagnosed with pulmonary hypoplasia between 1995 and 2014 in a tertiary university hospital. An analysis was made of the prenatal imaging, clinical manifestations, post-natal diagnostic tests, treatment and management, long-term follow up, and survival data. A total of 60 cases were identified, all of them with prenatal imaging. Sixteen patients required foetal surgery. Congenital diaphragmatic hernia was the most frequent diagnosis. Main clinical presentation was respiratory distress with severe hypoxemia and high requirements of mechanical ventilation. Mortality rate was 47% within first 60 days of life, and 75% for the first day of life. Pneumonia and recurrent bronchitis episodes were observed during follow-up. They had a lung function obstructive pattern, and their quality of life and exercise tolerance was good. High neonatal mortality and significant long-term morbidity associated with pulmonary hypoplasia requires an early diagnosis and a specialised multidisciplinary team management. Copyright © 2015 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  14. Maxillary hypoplasia in the cleft patient: contribution of orthodontic dental space closure to orthognathic surgery.

    Science.gov (United States)

    Lee, Justine C; Slack, Ginger C; Walker, Ryann; Graves, Lindsay; Yen, Sandra; Woo, Jessica; Ambaram, Rishal; Martz, Martin G; Kawamoto, Henry K; Bradley, James P

    2014-02-01

    Cleft lip and palate surgery in the developing child is known to be associated with maxillary hypoplasia. However, the effects of nonsurgical manipulations on maxillary growth have not been well investigated. The authors present the contribution of orthodontic dental space closure with canine substitution to maxillary hypoplasia and the need for orthognathic surgery. Cleft lip/palate and cleft palate patients older than 15 years of age were reviewed for dental anomalies, orthodontic canine substitution, and Le Fort I advancement. Skeletal relationships of the maxilla to the skull base (SNA), mandible (ANB), and facial height were determined on lateral cephalograms. Logistic regression analyses were performed to estimate odds ratios. Ninety-five patients were reviewed (mean age, 18.1 years). In 65 patients with congenitally missing teeth, 55 percent with patent dental spaces required Le Fort I advancement. In contrast, 89 percent who underwent canine substitution required Le Fort I advancement (p = 0.004). Canine substitution is associated with a statistically significant increase in maxillary retrognathia when compared with dental space preservation on lateral cephalograms (mean SNA, 75.2 and 79.0, respectively; p = 0.006). Adjusting for missing dentition, logistic regression analyses demonstrated that canine substitution is an independent predictor for orthognathic surgery (OR, 6.47) and maxillary retrusion defined by SNA orthodontic cleft closure using canine substitution with maxillary hypoplasia and subsequent Le Fort I advancement, and suggest systematic criteria for management of cleft-related dental agenesis. Therapeutic, III.

  15. Mechanisms of cadmium-caused eye hypoplasia and hypopigmentation in zebrafish embryos.

    Science.gov (United States)

    Zhang, Ting; Zhou, Xin-Ying; Ma, Xu-Fa; Liu, Jing-Xia

    2015-10-01

    Cadmium-caused head and eye hypoplasia and hypopigmentation has been recognized for a long time, but knowledge of the underlying mechanisms is limited. In this study, we found that high mortality occurred in exposed embryos after 24 hpf, when cadmium (Cd) dosage was above 17.8 μM. Using high-throughput in situ hybridization screening, we found that genes labelling the neural crest and its derivative pigment cells exhibited obviously reduced expression in Cd-exposed embryos from 24 hpf, 2 days earlier than head and eye hypoplasia and hypopigmentation occurred. Moreover, based on expression of crestin, a neural crest marker, we found that embryos before the gastrula stage were more sensitive to cadmium toxicity and that damage caused by Cd on embryogenesis was dosage dependent. In addition, by phenotype observation and detection of neural crest and pigment cell markers, we found that BIO and retinoic acid (RA) could neutralize the toxic effects of Cd on zebrafish embryogenesis. In this study, we first determined that Cd blocked the formation of the neural crest and inhibited specification of pigment cells, which might contribute to the molecular mechanisms underlying the phenotype defects of head and eye hypoplasia and hypopigmentation in Cd-exposed embryos. Moreover, we found that compounds BIO or RA could neutralize the toxic effects of Cd. Copyright © 2015 Elsevier B.V. All rights reserved.

  16. Evaluation of a new irradiation left breast method in the inhalation phase; Avaliacao de uma nova metodologia de irradiacao de mama esquerda na fase de inspiracao

    Energy Technology Data Exchange (ETDEWEB)

    Silva, Laura E. da; Gullo, Rafael G.; Ferreira, Diogo A.V.; Silva, Leonardo P. da, E-mail: aura.emilia.fm@gmail.com [Instituto Nacional de Cancer (INCA), Rio de Janeiro, RJ (Brazil)

    2016-07-01

    Radiation therapy is the primary therapeutic approach to breast cancer and involves significant exposure of heart and lungs, especially in cases of left breast. The implementation of a methodology to reduce the dose in these sites is important to ensure better quality of life to the patient. This work aims reduced heart and lung dose when performing the radiotherapy planning considering only the inhalation phase. Three patients with breast left present were scanning with CT during free breathing and respiratory monitoring and the planning has done on both images series. The results showed a reduction in mean heart dose of 53% on average. As for lung volumes, was obtained a mean reduction of 44.6% and 51% of the left lung which received 10 Gy and 20 Gy, respectively. This study showed that the use of the respiratory gating radiotherapy in the left breast in inhalation phase can reduce heart and lung doses. (author)

  17. The posterior communicating artery: morphometric study in 3D angio-computed tomography reconstruction. The proof of the mathematical definition of the hypoplasia.

    Science.gov (United States)

    Dzierżanowski, J; Szarmach, A; Słoniewski, P; Czapiewski, P; Piskunowicz, M; Bandurski, T; Szmuda, T

    2014-08-01

    The aim of this study was to investigate the morphometry of the posterior communicating artery (PCoA), on the basis of angio-computed tomography (CT), and to give proof of the mathematical definition of the term "hypopal sia of the PCoA". One hundred 3-dimensional (3D) angio-CT images, performed in adult patients with bilateral reconstruction of the PCoA (200 results) were used tocalculate the morphometry of the vessel. The average length of the vessel on the right side was 14.48 ± 3.47 mm, andon the left side 14.98 ± 4.77 mm (in women 14.75 mm, in men 14.70 mm). The mean of the diameter at the "proximal" point (the junction with P1) on the right side was 1.49 ± 0.51 mm, and on the left 1.46 ± 0.47 mm (in women 1.44 mm and in men 1.51 mm). The mean of the diameter in the "distal" part (the connection with ICA) on the right side was 1.4 ± 0.49 mm, and on the left 1.37 ± 0.41 mm (in women 1.38 mm, and in men 1.39 mm). No statistical correlation between the length and the diameter of the PCoA in relation to the sex and side was shown. On the basis of our measurements, we defined the hypoplasia of the artery as the estimated value less than the average diameter minus the standard deviation. The percentage distribution was as follows: the left artery 15.5%, the right artery 24%, women 11.5%, and the men 9%. Similarly to the above parameters, we have not found any statistical differences. The presence of the foetal origin was noted in 25% of the radiological examinations. The infundibular widening was visualised in 11.5% of cases of 3D reconstructions. The agenesis of PCoA was found in 9% (never bilaterally), and in 1 case the unilateral duplication of the artery was observed. No statistical differences between those parameters in relation to sex and the examined side were revealed. Morphological calculation of the PCoA on the basis of angio-CT from adult patients did not show any statistical differences depending on sex or the investigated side. The presented

  18. Enamel hypoplasia in the deciduous teeth of great apes: variation in prevalence and timing of defects.

    Science.gov (United States)

    Lukacs, J R

    2001-11-01

    The prevalence of enamel hypoplasia in the deciduous teeth of great apes has the potential to reveal episodes of physiological stress in early stages of ontogenetic development. However, little is known about enamel defects of deciduous teeth in great apes. Unresolved questions addressed in this study are: Do hypoplastic enamel defects occur with equal frequency in different groups of great apes? Are enamel hypoplasias more prevalent in the deciduous teeth of male or female apes? During what phase of dental development do enamel defects tend to form? And, what part of the dental crown is most commonly affected? To answer these questions, infant and juvenile skulls of two sympatric genera of great apes (Gorilla and Pan) were examined for dental enamel hypoplasias. Specimens from the Powell-Cotton Museum (Quex Park, UK; n = 107) are reported here, and compared with prior findings based on my examination of juvenile apes at the Cleveland Museum of Natural History (Hamman-Todd Collection; n = 100) and Smithsonian Institution (National Museum of Natural History; n = 36). All deciduous teeth were examined by the author with a x10 hand lens, in oblique incandescent light. Defects were classified using Fédération Dentaire International (FDI)/Defects of Dental Enamel (DDE) standards; defect size and location on the tooth crown were measured and marked on dental outline charts. Enamel defects of ape deciduous teeth are most common on the labial surface of canine teeth. While deciduous incisor and molar teeth consistently exhibit similar defects with prevalences of approximately 10%, canines average between 70-75%. Position of enamel defects on the canine crown was analyzed by dividing it into three zones (apical, middle, and cervical) and calculating defect prevalence by zone. Among gorillas, enamel hypoplasia prevalence increases progressively from the apical zone (low) to the middle zone to the cervical zone (highest), in both maxillary and mandibular canine teeth

  19. The use of internal maxillary distraction for maxillary hypoplasia: a preliminary report.

    Science.gov (United States)

    Van Sickels, Joseph E; Madsen, Mathew J; Cunningham, Larry L; Bird, Douglas

    2006-12-01

    Distraction osteogenesis is a useful alternative to advance the maxilla in complicated cases of maxillary hypoplasia. The purpose of this article is to review the workup, experience, and preliminary results with the use of internal distraction osteogenesis for maxillary hypoplasia at one teaching institution. Over a 5-year period, more than 300 patients with craniofacial and dentofacial defects have undergone oral and maxillofacial surgery at our center to correct their skeletal discrepancies. Of these, 10 have had maxillary distraction osteogenesis done with internal distractors. Follow-up of 6 months or more was available for 8 patients. Stereolithographic models were used to bend distractors prior to surgery in 6 patients. Latency prior to the start of distraction was 3 to 7 days and varied with the age of the patient. Distraction occurred at approximately 1 mm per day with an average distraction length of 8.5 mm (range, 6-10 mm). Excellent occlusal results were obtained in 5 patients. Major complications including nonunion and failure to achieve acceptable occlusal results were observed in 3 patients. Minor complications including pain and loosening of the distracter devices were observed in 2 patients, but did not appear to affect the esthetic and functional results. Distraction osteogenesis is a useful alternative to traditional orthognathic surgery to treat maxillary hypoplasia. Internal distractions are attractive to patients, but are more difficult to place and can cause discomfort to patients when trying to achieve an ideal primary vector of distraction. Stereolithographic models can help with placement of the device. Changes in design of distractors may help with patient discomfort.

  20. Posterior communicating artery hypoplasia as a risk factor for acute ischemic stroke in the absence of carotid artery occlusion.

    Science.gov (United States)

    Chuang, Yu-Ming; Liu, Chih-Yang; Pan, Po-Jung; Lin, Ching-Po

    2008-12-01

    Posterior communicating artery (PCoA) hypoplasia is a fetal variant of the Circle of Willis. According to angiograms and autopsy reports, this congenital variation is found in 6-21% of the general population. PCoA hypoplasia only becomes a risk factor for ischemic stroke in the presence of ipsilateral internal carotid artery (ICA) occlusion. The aim of our study was to determine the role of PCoA hypoplasia in acute ischemic stroke in the absence of ICA occlusion. We examined 310 acute ischemic stroke patients (mean age+/-standard deviation; 68.9+/-15.6 years). Cerebral magnetic resonance angiography was performed within 72 hours of ischemic stroke onset. For comparison, a risk factor-matched control group was recruited. Conditional logistic regression was used to calculate odds ratios (OR) and 95% confidence intervals (CI) to estimate the independent effect of potential risk factors. The overall incidence of PCoA hypoplasia in our experimental group was 19.35% (n=60), which was significantly higher than in the control group (8.20%, n=22, p=0.036, OR, 3.21; 95% CI, 1.43-9.62). The most common ischemic event was ipsilateral thalamic lacunar infarctions with or without occipital lobe involvement. Based on our results, PCoA hypoplasia appears to be a contributor to the risk of ischemic stroke, even in the absence of ICA occlusion. This risk is especially pronounced for strokes involving arteries that penetrate the thalamus.

  1. Prenatal and neonatal variables associated with enamel hypoplasia in deciduous teeth in low birth weight preterm infants

    Directory of Open Access Journals (Sweden)

    Kátia Maria Dmytraczenko Franco

    2007-12-01

    Full Text Available This study investigated possible prenatal and neonatal variables that may influence the prevalence of tooth enamel hypoplasia in preterm and low birth weight children (LBW and a matched control group of term children with normal birth weight (NBW. The study sample consisted of 61 children born preterm and with LBW examined at 18-34 months of age. The control group was formed by 61 infants born full term and with NBW examined at 31-35 months of age. All children were born at the Center of Integrated Attention of Women's Health (CAISM-UNICAMP. FDI criteria were followed for dental examination. Medical data was collected retrospectively from hospital records. Among preterms, 57.4% had some type of developmental defects of enamel (DDE, 52.5 % had opacities and 21.3 % presented hypoplasia. Among full-term children, 24.6% presented DDE, 24.6% had opacities and 3.3% had hypoplasia. LBW preterm infants presented a higher prevalence of hypoplasia than NBW controls. The deciduous teeth most affected by hypoplasia were maxillary incisors. There was no significant association with prenatal variables; among neonatal variables there was a significant association with respiratory distress syndrome and neurological examination at discharge with an altered result.

  2. Prediction of the articular eminence shape in a patient with unilateral hypoplasia of the right mandibular ramus before and after distraction osteogenesis-A simulation study.

    Science.gov (United States)

    de Zee, Mark; Cattaneo, Paolo M; Svensson, Peter; Pedersen, Thomas K; Melsen, Birte; Rasmussen, John; Dalstra, Michel

    2009-05-29

    The aim of this work was to predict the shape of the articular eminence in a patient with unilateral hypoplasia of the right mandibular ramus before and after distraction osteogenesis (DO). Using a patient-specific musculoskeletal model of the mandible the hypothesis that the observed differences in this patient in the left and right articular eminence inclinations were consistent with minimisation of joint loads was tested. Moreover, a prediction was made of the final shape of the articular eminence after DO when the expected remodelling has reached a steady state. The individual muscle forces and the average TMJ loading were computed for each combination of articular eminence angles both before and after DO. This exhaustive parameter study provides a full overview of average TMJ loading depending on the angles of the articular eminences. Before DO the parameter study resulted in different articular eminence inclinations between left and right sides consistent with patient data obtained from CT scans, indicating that in this patient the articular eminence shapes result from minimisation of joint loads. The simulation model predicts development of almost equal articular eminence shapes after DO. The same tendency was observed in cone beam CT scans (NewTom) of the patient taken 6.5 years after surgery.

  3. Management of Cleft Maxillary Hypoplasia with Anterior Maxillary Distraction: Our Experience.

    Science.gov (United States)

    Chacko, Tojan; Vinod, Sankar; Mani, Varghese; George, Arun; Sivaprasad, K K

    2014-12-01

    Maxillary hypoplasia is a common developmental problem in cleft lip and palate deformities. Since 1970s these deformities have traditionally been corrected by means of orthognathic surgery. Management of skeletal deformities in the maxillofacial region has been an important challenge for maxillofacial surgeons and orthodontists. Distraction osteogenesis is a surgical technique that uses body's own repairing mechanisms for optimal reconstruction of the tissues. We present four cases of anterior maxillary distraction osteogenesis with tooth borne distraction device-Hyrax, which were analyzed retrospectively for the efficacy of the tooth borne device-Hyrax and skeletal stability of distracted anterior maxillary segment.

  4. Congenital hypoplasia of the medical hallucial sesamoid with avascular necrosis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Song, Yoonah; Lee, Seunghun; Joo, Kyung Bin; Choi, Chan Bum [Hanyang University College of Medicine, Seoul Hospital, Seoul (Korea, Republic of); Ryu, Jeong Ah [Dept. of Radiology, Hanyang University College of Medicine, Guri Hospital, Guri (Korea, Republic of); Bae, Ji Yoon [Dept. of Pathology, National Police Hospital, Seoul (Korea, Republic of)

    2013-10-15

    Avascular necrosis of the hallucial sesamoids is an uncommon cause of metatarsalgia, and the congenital absence of the medial sesamoid is also a rarely reported condition in the podiatric literature. It must be distinguished from other painful conditions of the sesamoid due to the opposite direction of treatment. To our knowledge, there is no reported case of congenital hypoplasia of the medial sesamoid with osteonecrosis. We report a case of nontraumatic metatarsal pains with progressive sclerosis and fragmentation of the medial sesamoid on serial radiographs, magnetic resonance imaging, and ultrasonography with an incidental finding for the absence of contralateral medial sesamoid in a 33-year-old female.

  5. Schizencephaly with optic nerve hypoplasia simulating septo-optic dysplasia and other syndromes

    International Nuclear Information System (INIS)

    Osborn, R.E.; Byrd, S.E.

    1988-01-01

    Three patients with fused-lip schizencephaly with optic nerve hypoplasia were evaluated radiographically. The computed tomographic and magnetic resonance findings demonstrated unilateral or bilateral gray matter lined clefts in the cerebral hemisphere which allowed differentiation from the clinical diagnosis of septo-optic dysplasia in each case. Schizencephaly has also been mistakenly labelled as part of a syndrome known as the syndrome of absence of the septum pellucidum with porencephalies and as heterotopias with absence of the septum pellucidum. 15 refs.; 3 figs

  6. Clinical and molecular diagnosis of a cartilage-hair hypoplasia with IGF-1 deficiency.

    Science.gov (United States)

    Castilla-Cortázar, Inma; Rodríguez De Ita, Julieta; Martín-Estal, Irene; Castorena, Fabiola; Aguirre, Gabriel A; García de la Garza, Rocío; Elizondo, Martha I

    2017-02-01

    Cartilage-hair hypoplasia syndrome (CHH) is a rare autosomal recessive condition characterized by metaphyseal chondrodysplasia and characteristic hair, together with a myriad of other symptoms, being most common immunodeficiency and gastrointestinal complications. A 15-year-old Mexican male initially diagnosed with Hirschsprung disease and posterior immunodeficiency, presents to our department for genetic and complementary evaluation for suspected CHH. Physical, biochemical, and genetic studies confirmed CHH together with IGF-1 deficiency. For this reason, we propose IGF-1 replacement therapy for its well-known actions on hematopoiesis, immune function and maturation, and metabolism. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  7. Congenital hypoplasia of the medical hallucial sesamoid with avascular necrosis: A case report

    International Nuclear Information System (INIS)

    Song, Yoonah; Lee, Seunghun; Joo, Kyung Bin; Choi, Chan Bum; Ryu, Jeong Ah; Bae, Ji Yoon

    2013-01-01

    Avascular necrosis of the hallucial sesamoids is an uncommon cause of metatarsalgia, and the congenital absence of the medial sesamoid is also a rarely reported condition in the podiatric literature. It must be distinguished from other painful conditions of the sesamoid due to the opposite direction of treatment. To our knowledge, there is no reported case of congenital hypoplasia of the medial sesamoid with osteonecrosis. We report a case of nontraumatic metatarsal pains with progressive sclerosis and fragmentation of the medial sesamoid on serial radiographs, magnetic resonance imaging, and ultrasonography with an incidental finding for the absence of contralateral medial sesamoid in a 33-year-old female.

  8. Severe hypoplasia of the omasal laminae in a Japanese Black steer with chronic bloat--a case report.

    Science.gov (United States)

    Takagi, Mitsuhiro; Mukai, Shuhei; Fushimi, Yasuo; Matsushita, Kouhei; Miyoshi, Nobuaki; Yasuda, Nobuhiro; Kitajima, Hideo; Takamure, Senro; Matsushita, Toshihiko; Kitamura, Nobuo; Deguchi, Eisaburo

    2007-12-01

    An 11-month-old Japanese Black steer with chronic bloat underwent clinical and histological analyses. During the observation period, it showed normal appetite and fecal volume but persistent chronic bloat symptoms. Compared to controls, the steer's feces contained undigested large straws. Necropsy revealed normal rumen, reticulum, and abomasum but a small omasum. The rumen, reticulum, and abomasum mucosa was normal, with well-developed ruminal papillae. However, severe hypoplasia of the omasal laminae was observed along with hypoplasia reticular groove and ruminoreticular fold. The contents of the reticulum, omasum, and abomasums comprised undigested large sized hay particles. The omasum papillae showed no pathological abnormalities. This is a rare case of a steer with chronic bloat probably caused by severe hypoplasia of the omasal laminae.

  9. Treatment of Severe Maxillary Hypoplasia With Combined Orthodontics and Distraction Osteogenesis.

    Science.gov (United States)

    Lucchese, Alessandra; Albertini, Paolo; Asperio, Paolo; Manuelli, Maurizio; Gastaldi, Giorgio

    2018-01-05

    Distraction osteogenesis (DO) is a technique that allows the generation of new bone in a gap between 2 vascularized bone surfaces in response to the application of graduated tensile stress across the bone gap.Distraction osteogenesis has become a routine treatment of choice to correct skeletal deformities and severe bone defects in the craniofacial complex over the past decade. Distraction osteogenesis has been successfully chosen in lengthening the maxilla and the mandible; in the maxilla and recently in the mandible, the jawbones have been distracted and widened transversely to relieve severe anterior dental crowding and transverse discrepancies between the dental arches.Distraction osteogenesis for maxillary advancement started in 1993 and is now widely used, especially in patients with skeletal Class III malocclusion caused by maxillary hypoplasia.The aim of this study was to present the efficiency of combined orthodontic and DO in the severe maxillary hypoplasia.A 35-year-old Italian man presented to our clinical practice with the chief complaint of esthetic and functionally problems because of skeletal Class III malocclusion with anterior crossbite.Considering that the severity of the skeletal discrepancy is remarkable but compensated by the DO potential, the combined orthodontic and DO treatment was considered adequate, like less invasive and equally effective.It was obtained a good alignment with the upper and lower arch dental alveolar maxillary advancement that allowed to correct the sagittal relationships.The patient was satisfied for the treatment results and had considerable improvement in his self-esteem.

  10. Hereditary renal adysplasia, pulmonary hypoplasia and Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a case report

    Science.gov (United States)

    2010-01-01

    Background Hereditary renal adysplasia is an autosomal dominant trait with incomplete penetrance and variable expression that is usually associated with malformative combinations (including Müllerian anomalies) affecting different mesodermal organs such as the heart, lung, and urogenital system. Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. The i.v. pyelography showed right renal agenesis with a normal left kidney and ureter. Ultrasound and Magnetic Resonance Imaging also showed right renal agenesis with multicystic embryonary remnants in the right hemipelvis probably corresponding to a dysgenetic kidney. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. She was scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft. Laparoscopy confirmed the absence of the uterus. On both sides, an elongated, solid, rudimentary uterine horn could be observed. Both ovaries were also elongated, located high in both abdominal flanks and somewhat dysgenetics. A conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH detecting 11 copy number variants described as normal variants in the databases. The 17q12 and 22q11.21 microdeletions described in other MRKH patients were not present in this case. Four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. The geneticists have studied her family history and the pedigree of the family is shown. Conclusions We suggest that primary damage to the mesoderm (paraaxil, intermediate, and lateral) caused by mutations in a yet unidentified gene is responsible for: 1) skeletal dysplasia, 2) inappropriate interactions between the bronchial mesoderm and endodermal

  11. Discrepancy in compliance between the clinical and genetic diagnosis of choroidal hypoplasia in Danish Rough Collies and Shetland Sheepdogs

    DEFF Research Database (Denmark)

    Fredholm, Merete; Larsen, R. C.; Jönsson, M.

    2016-01-01

    Collie eye anomaly (CEA) is a congenital, inherited ocular disorder which is widespread in herding breeds. Clinically, the two major lesions associated with CEA are choroidal hypoplasia (CH) and coloboma, and both lesions are diagnosed based on ophthalmological examination. A 7.8-kb intronic dele...

  12. Perceptual Speech Assessment After Anterior Maxillary Distraction in Patients With Cleft Maxillary Hypoplasia.

    Science.gov (United States)

    Richardson, Sunil; Seelan, Nikkie S; Selvaraj, Dhivakar; Khandeparker, Rakshit V; Gnanamony, Sangeetha

    2016-06-01

    To assess speech outcomes after anterior maxillary distraction (AMD) in patients with cleft-related maxillary hypoplasia. Fifty-eight patients at least 10 years old with cleft-related maxillary hypoplasia were included in this study irrespective of gender, type of cleft lip and palate, and amount of required advancement. AMD was carried out in all patients using a tooth-borne palatal distractor by a single oral and maxillofacial surgeon. Perceptual speech assessment was performed by 2 speech language pathologists preoperatively, before placement of the distractor device, and 6 months postoperatively using the scoring system of Perkins et al (Plast Reconstr Surg 116:72, 2005); the system evaluates velopharyngeal insufficiency (VPI), resonance, nasal air emission, articulation errors, and intelligibility. The data obtained were tabulated and subjected to statistical analysis using Wilcoxon signed rank test. A P value less than .05 was considered significant. Eight patients were lost to follow-up. At 6-month follow-up, improvements of 62% (n = 31), 64% (n = 32), 50% (n = 25), 68% (n = 34), and 70% (n = 35) in VPI, resonance, nasal air emission, articulation, and intelligibility, respectively, were observed, with worsening of all parameters in 1 patient (2%). The results for all tested parameters were highly significant (P ≤ .001). AMD offers a substantial improvement in speech for all 5 parameters of perceptual speech assessment. Copyright © 2016 The American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.

  13. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    International Nuclear Information System (INIS)

    Bruckheimer, Elchanan; Dagan, Tamir; Atar, Eli; Schwartz, Michael; Kachko, Ludmila; Superina, Riccardo; Amir, Gabriel; Shapiro, Rivka; Birk, Einat

    2013-01-01

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ∼50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5–13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2–8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia

  14. X-linked adrenal hypoplasia congenita: a case report and ethical dilemma.

    Science.gov (United States)

    Ismail, Heba M; Rincon, Marielisa

    2014-07-01

    Our objective is to present the first case report of X-linked adrenal hypoplasia congenita in a child conceived by a donated egg and which also presented atypically, with initial mineralocorticoid deficiency. Case report with literature review. A late preterm fraternal twin male, conceived by in vitro fertilization of donated eggs, presented shortly after birth with feeding intolerance, hyponatremia, and hyperkalemia. Testing revealed a low aldosterone level, high plasma renin activity, normal cortisol level, and normal 17-hydroxyprogesterone level. He was diagnosed with 18-hydroxylase deficiency based on low 18-hydroxycorticosterone levels and was treated with mineralocorticoid successfully for 17 months. At age 18 months, he presented with dehydration secondary to herpetic gingivostomatitis and was found to be hypoglycemic, hyponatremic, hyperkalemic, and acidotic, with a low serum cortisol level. An adrenocorticotropic hormone (ACTH) stimulation test revealed low levels of all adrenal cortex products, with an elevated ACTH level. He was started on glucocorticoids. Genetic testing confirmed X-linked adrenal hypoplasia congenita (AHC). His asymptomatic fraternal twin underwent genetic testing and the results were negative. The fertility center records indicated that the mother had donated eggs to other families, but none of the children were known to have this disorder. The egg donor was informed but did not pursue genetic testing. We report a case of X-linked AHC presenting in the context of extraordinary ethical considerations. Our case raises a question unique to the era of assisted reproduction: should routine genetic screening of gamete donors be done for rare but potentially life-threatening conditions?

  15. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    Energy Technology Data Exchange (ETDEWEB)

    Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net; Dagan, Tamir [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Atar, Eli; Schwartz, Michael [Schneider Children' s Medical Center Israel, Section of Radiology (Israel); Kachko, Ludmila [Schneider Children' s Medical Center Israel, Section of Anesthesiology (Israel); Superina, Riccardo; Amir, Gabriel [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Shapiro, Rivka [Schneider Children' s Medical Center Israel, Section of Gastroenterology (Israel); Birk, Einat [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel)

    2013-12-15

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

  16. Reliability of magnetic resonance imaging for the detection of hypopituitarism in children with optic nerve hypoplasia.

    Science.gov (United States)

    Ramakrishnaiah, Raghu H; Shelton, Julie B; Glasier, Charles M; Phillips, Paul H

    2014-01-01

    It is essential to identify hypopituitarism in children with optic nerve hypoplasia (ONH) because they are at risk for developmental delay, seizures, or death. The purpose of this study is to determine the reliability of neurohypophyseal abnormalities on magnetic resonance imaging (MRI) for the detection of hypopituitarism in children with ONH. Cross-sectional study. One hundred one children with clinical ONH who underwent MRI of the brain and orbits and a detailed pediatric endocrinologic evaluation. Magnetic resonance imaging studies were performed on 1.5-Tesla scanners. The imaging protocol included sagittal T1-weighted images, axial fast fluid-attenuated inversion-recovery/T2-weighted images, and diffusion-weighted images of the brain. Orbital imaging included fat-saturated axial and coronal images and high-resolution axial T2-weighted images. The MRI studies were reviewed by 2 pediatric neuroradiologists for optic nerve hypoplasia, absent or ectopic posterior pituitary, absent pituitary infundibulum, absent septum pellucidum, migration anomalies, and hemispheric injury. Medical records were reviewed for clinical examination findings and endocrinologic status. All patients underwent a clinical evaluation by a pediatric endocrinologist and a standardized panel of serologic testing that included serum insulin-like growth factor-1, insulin-like growth factor binding protein-3, prolactin, cortisol, adrenocorticotropic hormone, thyroid-stimulating hormone, and free thyroxine levels. Radiologists were masked to patients' endocrinologic status and funduscopic findings. Sensitivity and specificity of MRI findings for the detection of hypopituitarism. Neurohypophyseal abnormalities, including absent pituitary infundibulum, ectopic posterior pituitary bright spot, and absent posterior pituitary bright spot, occurred in 33 children. Magnetic resonance imaging disclosed neurohypophyseal abnormalities in 27 of the 28 children with hypopituitarism (sensitivity, 96%). A

  17. Le Fort I distraction using internal devices for maxillary hypoplasia in patients with cleft lip, palate, and alveolus: complications and their prevention and management.

    Science.gov (United States)

    Mitsukawa, Nobuyuki; Satoh, Kaneshige; Morishita, Tadashi

    2010-09-01

    In recent years, advancement has been made in distraction osteogenesis in the facial area. It has been applied actively to treat maxillary hypoplasia in patients with cleft lip, palate, and alveolus. Le Fort I distraction using internal devices does not lead to disability in daily living, including during the retention period, and has low surgical invasiveness, facile management, and superior aesthetics. Thus, there have been occasional reports on this procedure in recent years. Because an internal device is unidirectional, the greatest disadvantage is the inability to adjust the direction of distraction after its placement. However, past reports have emphasized its advantages, and its complications have rarely been discussed. We performed Le Fort I distraction using internal devices on 15 young patients. Our results showed notable complications in a few cases. When serial cephalometric analysis was performed after maxillary distraction, it demonstrated that the maxilla assumes various three-dimensional distraction morphologies. In particular, cases with insufficient bone grafting of alveolar clefts developed collapse mainly in the alveolar cleft region during postoperative distraction. Mobility and deviation of the maxillary bone fragment occurred. We implemented preventative measures against complications such as using modifications to place the devices parallel on the left and right sides and using a bite splint for distraction. These measures produced improved outcomes, and we describe here the details.

  18. Treatment of severe maxillary cleft hypoplasia in a case with missing premaxilla with anterior maxillary distraction using tooth-borne hyrax appliance

    Directory of Open Access Journals (Sweden)

    Akshai Shetty

    2015-01-01

    Full Text Available Cleft orthodontics generally poses a challenge and a missing premaxilla adds to the difficulty in managing them. The lack of bone support and anterior teeth in a case with missing premaxilla accounts not only for difficulty in rehabilitation but also in increasing the maxillary hypoplasia. This article presents a case report where planned orthodontic and surgical management using distraction has helped treat a severe maxillary hypoplasia in a patient with missing premaxilla. The treatment plan and method can be used to treat severe maxillary hypoplasia and yield reasonably acceptable results for such patients.

  19. Tooth-Borne Anterior Maxillary Distraction for Cleft Maxillary Hypoplasia: Our Experience With 147 Patients.

    Science.gov (United States)

    Richardson, Sunil; Selvaraj, Dhivakar; Khandeparker, Rakshit V; Seelan, Nikkie S; Richardson, Shweta

    2016-12-01

    To evaluate the results of anterior maxillary distraction for its efficacy and long-term stability in the management of cleft maxillary hypoplasia in a large series of patients with a long-term follow-up extending to 4 years. One hundred sixty-four patients at least 10 years old with cleft maxillary hypoplasia who presented to the authors' unit from January 2009 through October 2014 were evaluated retrospectively, irrespective of gender, type of cleft lip and palate, and amount of advancement needed. Anterior maxillary distraction using a tooth-borne distractor appliance was carried out in all patients and all patients were followed up to 4 years (range, 1 to 4 yr) to evaluate the stability of the procedure and to document any relapse using digitalized lateral cephalograms taken before distraction, immediately after distraction (T2), and at the last follow-up visit (T3; range, 1 to 4 yr). Seventeen patients were subsequently lost to follow-up; therefore, a complete set of records was available for 147 patients. In a subset of 50 patients, perceptual speech assessment was carried out preoperatively and 6 months postoperatively by 2 speech pathologists using the Perkins scoring system that allowed the evaluation of 5 parameters (velopharyngeal insufficiency, resonance, nasal air emission, articulation, and intelligibility). None of these patients underwent speech therapy during the course of evaluation. The development of complications intra- or postoperatively was noted. The data were tabulated and analyzed. An advancement ranging from 4.0 to 13.1 mm (mean, 9.42 mm) was achieved in all patients. One hundred forty patients (95.23%) showed stable results on lateral cephalograms and when T2 values were compared with T3 values. Seven patients (4.76%) exhibited skeletal relapse in various linear and angular measurements assessed on lateral cephalograms. At 6-month follow-up, improvements of 62% (n = 31), 64% (n = 32), 50% (n = 25), 68% (n = 34), and 70% (n

  20. Prevalence of dental anomalies and enamel hypoplasia in primary dentition among preschool children of West Godavari District, Andhra Pradesh -A cross - sectional study

    Directory of Open Access Journals (Sweden)

    Suzan Sahana

    2013-01-01

    Full Text Available Background: It is axiomatic that Pediatric dental anomalies and enamel hypoplasia (E.H are routinely encountered in primary dentition and early detection and prudent management of the condition facilitates normal occlusal development. Objectives: To determine the prevalence of various dental anomalies and enamel hypoplasia in preschool children between two to six years of age. Materials & Method: A total of 1898 children, between two to six years were randomly selected and screened for dental anomalies and enamel hypoplasia The chi square test was used to analyze the data statistically. Results: The overall prevalence rate of dental anomalies and enamel hypoplasia in this study was 0.63% and 8.95% respectively. Double teeth were the most frequently reported dental anomaly while supernumerary teeth were least reported. None of them reported with hypodontia.

  1. CT volumetry of lumbar vertebral bodies in patients with hypoplasia L5 and bilateral spondylolysis and in normal controls

    International Nuclear Information System (INIS)

    Wilms, Guido E.; Demaerel, Philippe; Keyzer, Frederik de; Willems, Endry

    2012-01-01

    To examine the feasibility and results of calculating the volume of lumbar vertebral bodies in normal patients and patients with suspected hypoplasia of L5. Lumbar multi-detector CT was performed in 38 patients with bilateral spondylolysis and hypoplasia of L5 and in 38 normal patients. Lumbar vertebral body volume of L3, L4 and L5 was measured by CT volumetry with a semi-automated program, created with MeVisLab. In the control group, the average vertebral body volume (in cubic centimeters) of L3 was 35.93 (±7.33), 36.34 (±7.13) for L4 and 34.63 (±6.88) for L5. In patients with suspected hypoplasia L5 the average body volume (in cubic centimeters) of L3 was 36.85 (±7.37), 36.90 (±6.99) for L4 and 33.14 (±6.57) for L5. The difference in mean vertebral body volume for L3, L4 and L5 between both groups was statistically not significant. However, there was a statistically significant difference of the ratio L5/L4 (P < 0.001) between both groups: the mean ratio L5/L4 in the control group was 95.3 ± 3.9%, the ratio for the hypoplastic L5 group was 89.9 ± 6.3%. There was no significant difference in the vertebral body volume for L3, L4 and L5 between both groups due to inter-patient variability. However, the relation between the body volume of L5 and L4 is significantly different between both groups. The volume of the vertebral body of L5 proved to be on average 10.2% smaller than the volume of L4 in the group with hypoplasia L5 versus 4.7% in the control group. (orig.)

  2. A detailed study of enamel hypoplasia in a post-medieval adolescent of known age and sex.

    Science.gov (United States)

    King, T; Hillson, S; Humphrey, L T

    2002-01-01

    Developmental disturbances that affect the secretion of enamel matrix can cause defective enamel structure. Linear hypoplasia is one type of enamel defect and manifests itself as a furrow that runs around the circumference of the tooth. Such defects range in size from the microscopic to those that are several millimetres wide. Enamel defects have been widely used by anthropologists for the investigation of growth disruptions in past populations, as they provide a permanent record of disturbances during much of a child's developmental period. This is a detailed case study of enamel growth disruptions in a 15-year-old female from the 18th and 19th century crypt of Christ Church, Spitalfields. The method used relates linear enamel hypoplasia to the incremental structures in the enamel surface, the perikymata, in order to investigate the timing of growth disturbances. Linear enamel hypoplasia was defined here as a greater than expected spacing between neighbouring pairs of perikymata. In addition, this study used recently published histological data on the precise timing of tooth development to establish chronologies for growth disruptions. Defects were matched in at least two teeth with overlapping developmental schedules to ensure that systemic disturbances, as opposed to localised traumas, were identified. Thirteen enamel defects were matched between five different teeth from the same individual from Spitalfields. Most linear enamel hypoplasias were evident on the anterior dentition. Using an 8-day average perikymata periodicity, the age at first defect in this individual was calculated as 1.5 years and the last growth disruption occurred when she was 4.6 years of age. The distribution of the defects was examined to identify any seasonal pattern in the occurrence of the growth disturbances.

  3. CT volumetry of lumbar vertebral bodies in patients with hypoplasia L5 and bilateral spondylolysis and in normal controls

    Energy Technology Data Exchange (ETDEWEB)

    Wilms, Guido E.; Demaerel, Philippe; Keyzer, Frederik de [UZ Leuven, Campus Gasthuisberg, Department of Radiology, Leuven (Belgium); Willems, Endry [ZOL, Department of Radiology, Genk (Belgium)

    2012-08-15

    To examine the feasibility and results of calculating the volume of lumbar vertebral bodies in normal patients and patients with suspected hypoplasia of L5. Lumbar multi-detector CT was performed in 38 patients with bilateral spondylolysis and hypoplasia of L5 and in 38 normal patients. Lumbar vertebral body volume of L3, L4 and L5 was measured by CT volumetry with a semi-automated program, created with MeVisLab. In the control group, the average vertebral body volume (in cubic centimeters) of L3 was 35.93 ({+-}7.33), 36.34 ({+-}7.13) for L4 and 34.63 ({+-}6.88) for L5. In patients with suspected hypoplasia L5 the average body volume (in cubic centimeters) of L3 was 36.85 ({+-}7.37), 36.90 ({+-}6.99) for L4 and 33.14 ({+-}6.57) for L5. The difference in mean vertebral body volume for L3, L4 and L5 between both groups was statistically not significant. However, there was a statistically significant difference of the ratio L5/L4 (P < 0.001) between both groups: the mean ratio L5/L4 in the control group was 95.3 {+-} 3.9%, the ratio for the hypoplastic L5 group was 89.9 {+-} 6.3%. There was no significant difference in the vertebral body volume for L3, L4 and L5 between both groups due to inter-patient variability. However, the relation between the body volume of L5 and L4 is significantly different between both groups. The volume of the vertebral body of L5 proved to be on average 10.2% smaller than the volume of L4 in the group with hypoplasia L5 versus 4.7% in the control group. (orig.)

  4. Anterior maxillary segmental distraction in the treatment of severe maxillary hypoplasia secondary to cleft lip and palate.

    Science.gov (United States)

    Li, Hongliang; Dai, Jiewen; Si, Jiawen; Zhang, Jianfei; Wang, Minjiao; Shen, Steve Guofang; Yu, Hongbo

    2015-01-01

    Anterior maxillary segmental distraction (AMSD) is an effective surgical procedure in the treatment of maxillary hypoplasia secondary to cleft lip and palate. Its unique advantage of preserving velopharyngeal function makes this procedure widely applied. In this study, the application of AMSD was described and its long-term stability was explored. Eight patients with severe maxillary hypoplasia secondary to CLP were included in this study. They were treated with AMSD using rigid external distraction (RED) device. Cephalometric analysis was performed twice at three time points for evaluation: before surgery (T1), after distraction (T2), and 2 years after treatment (T3). One-way analysis of variance was used to assess the differences statistically. All the distractions completed smoothly, and maxilla was distracted efficiently. The value of SNA, NA-FH, Ptm-A, U1-PP, overjet and PP (ANS-PNS) increased significantly after the AMSD procedure (P 0.05). Changes of palatopharyngeal depth and soft palatal length were insignificant. AMSD with RED device provided an effective way to correct maxillary hypoplasia secondary to CLP, extended the palatal and arch length, avoided damage on velopharyngeal closure function and reduced the relapse rate. It is a promising and valuable technique in this potentially complicated procedure.

  5. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    Energy Technology Data Exchange (ETDEWEB)

    Drozd, V.; Danilova, L.; Lushchyk, M.; Leonova, T.; Platonova, T. [International Fund Arnica, Minsk (Belarus); Grigorovich, A.; Sivuda, V. [Brest Regional Endocrinological Dispensary, Brest (Belarus); Branovan, I. [Chernobyl Project, New-York (United States); Biko, I.; Reiners, C. [Clinic and Policlinic of Nuclear Medicine, University of Wurzburg, Wursburg (Germany)

    2012-07-01

    For the 24 years passed after the Chernobyl catastrophe a significant experience in estimation of medical consequences of thyroid irradiation among Belarus patients had been accumulated. The aim of our screening of ultrasonic examination was the detection of the thyroid hypoplasia prevalence in the regions affected with radionuclide fallout. Since 2004 to 2007 thyroid ultrasound with volume estimation was performed in 3311 Belarus subjects, living on the areas of Brest region with the different contamination rate density. Examined subjects were divided in 3 groups: 1) irradiated at the age of 1 to 3 years old at the moment of Chernobyl catastrophe, 2) irradiated in utero, and 3) born after the catastrophe. It was revealed that thyroid hypoplasia was detected in 3% of group 1 (out of 1876 persons), in 5, 8% of group 2 (out of 503 persons, P<0.05) and in 1, 7% of the third group (out of 932 persons). The separation of the irradiated in utero subjects (group 2) to subgroups in dependence of the gestation period, showed the highest prevalence of thyroid hypoplasia among the irradiated in the first trimester of gestation: 7, 7% (P<0.05), in the second trimester: 5, 3%, in the third trimester: 4, 7%

  6. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    International Nuclear Information System (INIS)

    Drozd, V.; Danilova, L.; Lushchyk, M.; Leonova, T.; Platonova, T.; Grigorovich, A.; Sivuda, V.; Branovan, I.; Biko, I.; Reiners, C.

    2012-01-01

    For the 24 years passed after the Chernobyl catastrophe a significant experience in estimation of medical consequences of thyroid irradiation among Belarus patients had been accumulated. The aim of our screening of ultrasonic examination was the detection of the thyroid hypoplasia prevalence in the regions affected with radionuclide fallout. Since 2004 to 2007 thyroid ultrasound with volume estimation was performed in 3311 Belarus subjects, living on the areas of Brest region with the different contamination rate density. Examined subjects were divided in 3 groups: 1) irradiated at the age of 1 to 3 years old at the moment of Chernobyl catastrophe, 2) irradiated in utero, and 3) born after the catastrophe. It was revealed that thyroid hypoplasia was detected in 3% of group 1 (out of 1876 persons), in 5, 8% of group 2 (out of 503 persons, P<0.05) and in 1, 7% of the third group (out of 932 persons). The separation of the irradiated in utero subjects (group 2) to subgroups in dependence of the gestation period, showed the highest prevalence of thyroid hypoplasia among the irradiated in the first trimester of gestation: 7, 7% (P<0.05), in the second trimester: 5, 3%, in the third trimester: 4, 7%

  7. [Bilateral hypoplasia of the internal carotid arteries associated with aneurysm of the right posterior communicating artery. Apropos of a case].

    Science.gov (United States)

    el Khamlichi, A; Amrani, F; el Azzusi, M; el Oufir, M; Khamlichi, A M

    1989-01-01

    The authors report a case of bilateral hypoplasia of the internal carotid arteries associated with aneurysm of the right posterior communicating artery in a 17 year old female patient. This anomaly was discovered following a meningeal haemorrhage, which recurred 18 months later, causing the patient's death. Surgical operation was refused by the patient and her family. Bilateral hypoplasia of the internal carotid arteries is a rare congenital malformation (16 cases have been reported in the literature, our case constitutes the 17th). It is distinguished from aplasia by the presence of a patent but very reduced vascular lumen, while aplasia is associated with vestiges of non-patent vessels. The mechanism of development of such a malformation is unclear: some authors have suggested secondary regression of the internal carotid artery following a phase of normal development, while others consider it to represent arrest of the development of the internal carotid artery, at a given moment in time. The frequency of associated aneurysm would be due to the haemodynamic disruption induced by the malformation, especially as parietal defects are more frequent in a malformed vasculature. Bilateral hypoplasia of the internal carotid arteries may be compatible with normal life for an indefinite period of time due to the development of a large number of collateral vessels. However, the new vasculature is threatened by rupture with meningeal haemorrhage and by acute ischaemia, which would probably involve another aetiological factor.

  8. New mutations of DAX-1 genes in two Japanese patients with X-linked congenital adrenal hypoplasia and hypogonadotropic hypogonadism

    Energy Technology Data Exchange (ETDEWEB)

    Yanase, Toshihiko; Takayanagi, Ryoichi; Oba, Koichi [Kyushu Univ., Fukuoka (Japan)] [and others

    1996-02-01

    Congenital adrenal hypoplasia, an X-linked disorder, is characterized by primary adrenal insufficiency and frequent association with hypogonadotropic hypogonadism. The X-chromosome gene DAX-1 has been most recently identified and shown to be responsible for this disorder. We analyzed the DAX-1 genes of two unrelated Japanese patients with congenital adrenal hypoplasia and hypogonadotropic hypogonadism by using PCR amplification of genomic DNA and its complete exonic sequencing. In a family containing several affected individuals, the proband male patient had a stop codon (TGA) in place of tryptophan (TGG) at amino acid position 171. As expected, his mother was a heterozygous carrier for the mutation, whereas his father and unaffected brother did not carry this mutation. In another male patient with noncontributory family history, sequencing revealed a 1-bp (T) deletion at amino acid position 280, leading to a frame shift and, subsequently a premature stop codon at amino acid position 371. The presence of this mutation in the patients` genome was further confirmed by digestion of genomic PCR product with MspI created by this mutation. Family studies using MspI digestion of genomic PCR products revealed that neither parent of this individual carried the mutation. These results clearly indicate that congenital adrenal hypoplasia and hypogonadotropic hypogonadism result from not only inherited but also de novo mutation in the DAX-1 gene. 31 refs., 4 figs., 2 tabs.

  9. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  10. Limb/pelvis hypoplasia/aplasia with skull defect (Schinzel phocomelia): distinctive features and prenatal detection.

    Science.gov (United States)

    Olney, R S; Hoyme, H E; Roche, F; Ferguson, K; Hintz, S; Madan, A

    2001-11-01

    Schinzel phocomelia syndrome is characterized by limb/pelvis hypoplasia/aplasia: specifically, intercalary limb deficiencies and absent or hypoplastic pelvic bones. The phenotype is similar to that described in a related multiple malformation syndrome known as Al-Awadi/Raas-Rothschild syndrome. The additional important feature of large parietooccipital skull defects without meningocele, encephalocele, or other brain malformation has thus far been reported only in children with Schinzel phocomelia syndrome. We recently evaluated a boy affected with Schinzel phocomelia born to nonconsanguineous healthy parents of Mexican origin. A third-trimester fetal ultrasound scan showed severe limb deficiencies and an absent pelvis. The infant died shortly after birth. Dysmorphology examination, radiographs, and autopsy revealed quadrilateral intercalary limb deficiencies with preaxial toe polydactyly; an absent pelvis and a 7 x 3-cm skull defect; and extraskeletal anomalies including microtia, telecanthus, micropenis with cryptorchidism, renal cysts, stenosis of the colon, and a cleft alveolar ridge. A normal 46,XY karyotype was demonstrated, and autosomal recessive inheritance was presumed on the basis of previously reported families. This case report emphasizes the importance of recognizing severe pelvic and skull deficiencies (either post- or prenatally) in differentiating infants with Schinzel phocomelia from other multiple malformation syndromes that feature intercalary limb defects, including thalidomide embryopathy and Roberts-SC phocomelia. Copyright 2001 Wiley-Liss, Inc.

  11. Mandibular hypoplasia, deafness, progeroid features and lipodystrophy (MDPL) syndrome in the context of inherited lipodystrophies.

    Science.gov (United States)

    Reinier, Frederic; Zoledziewska, Magdalena; Hanna, David; Smith, Josh D; Valentini, Maria; Zara, Ilenia; Berutti, Riccardo; Sanna, Serena; Oppo, Manuela; Cusano, Roberto; Satta, Rosanna; Montesu, Maria Antonietta; Jones, Chris; Cerimele, Decio; Nickerson, Deborah A; Angius, Andrea; Cucca, Francesco; Cottoni, Francesca; Crisponi, Laura

    2015-11-01

    Lipodystrophies are a large heterogeneous group of genetic or acquired disorders characterized by generalized or partial fat loss, usually associated with metabolic complications such as diabetes mellitus, hypertriglyceridemia and hepatic steatosis. Many efforts have been made in the last years in identifying the genetic etiologies of several lipodystrophy forms, although some remain to be elucidated. We report here the clinical description of a woman with a rare severe lipodystrophic and progeroid syndrome associated with hypertriglyceridemia and diabetes whose genetic bases have been clarified through whole-exome sequencing (WES) analysis. This article reports the 5th MDPL (Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome) patient with the same de novo p.S605del mutation in POLD1. We provided further genetic evidence that this is a disease-causing mutation along with a plausible molecular mechanism responsible for this recurring event. Moreover we overviewed the current classification of the inherited forms of lipodystrophy, along with their underlying molecular basis. Progress in the identification of lipodystrophy genes will help in better understanding the role of the pathways involved in the complex physiology of fat. This will lead to new targets towards develop innovative therapeutic strategies for treating the disorder and its metabolic complications, as well as more common forms of adipose tissue redistribution as observed in the metabolic syndrome and type 2 diabetes. Copyright © 2015 Elsevier Inc. All rights reserved.

  12. Recessive mutations in SLC38A8 cause foveal hypoplasia and optic nerve misrouting without albinism.

    Science.gov (United States)

    Poulter, James A; Al-Araimi, Musallam; Conte, Ivan; van Genderen, Maria M; Sheridan, Eamonn; Carr, Ian M; Parry, David A; Shires, Mike; Carrella, Sabrina; Bradbury, John; Khan, Kamron; Lakeman, Phillis; Sergouniotis, Panagiotis I; Webster, Andrew R; Moore, Anthony T; Pal, Bishwanath; Mohamed, Moin D; Venkataramana, Anandula; Ramprasad, Vedam; Shetty, Rohit; Saktivel, Murugan; Kumaramanickavel, Govindasamy; Tan, Alex; Mackey, David A; Hewitt, Alex W; Banfi, Sandro; Ali, Manir; Inglehearn, Chris F; Toomes, Carmel

    2013-12-05

    Foveal hypoplasia and optic nerve misrouting are developmental defects of the visual pathway and only co-occur in connection with albinism; to date, they have only been associated with defects in the melanin-biosynthesis pathway. Here, we report that these defects can occur independently of albinism in people with recessive mutations in the putative glutamine transporter gene SLC38A8. Nine different mutations were identified in seven Asian and European families. Using morpholino-mediated ablation of Slc38a8 in medaka fish, we confirmed that pigmentation is unaffected by loss of SLC38A8. Furthermore, by undertaking an association study with SNPs at the SLC38A8 locus, we showed that common variants within this gene modestly affect foveal thickness in the general population. This study reveals a melanin-independent component underpinning the development of the visual pathway that requires a functional role for SLC38A8. Copyright © 2013 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  13. Children with optic nerve hypoplasia face a high risk of neurodevelopmental disorders.

    Science.gov (United States)

    Dahl, Sara; Wickström, Ronny; Ek, Ulla; Teär Fahnehjelm, Kristina

    2018-03-01

    Optic nerve hypoplasia (ONH) is a congenital ocular malformation that has been associated with neurodevelopmental disorders, but the prevalence in unilateral disease and less severe visual impairment is unknown. We studied intellectual disability and autism spectrum disorders (ASDs) in patients with ONH. This was a population-based cross-sectional cohort study of 65 patients (33 female) with ONH below 20 years of age, living in Stockholm in December 2009, with data analysed in January 2016. Of these 35 were bilateral and 30 were unilateral. Neurodevelopmental disorders were diagnosed or confirmed by neurological assessments, the Five to Fifteen parent questionnaire and reviewing previous neuropsychological investigations or conducting neuropsychological tests. Bilateral ONH patients had lower mean full scale intelligence quotient scores than unilateral patients (84.4 and 99.4, respectively, p = 0.049). We assessed intellectual disability in 55 eligible patients, and it was more common in patients with bilateral ONH (18 of 32, 56%) than unilateral ONH (two of 23, 9%, p neurodevelopmental disorders, especially intellectual disability. The risk was lower in unilateral ONH, but the levels of neurodevelopmental disorders warrant screening of both groups. ©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  14. Growth, nutritional, and gastrointestinal aspects of focal dermal hypoplasia (Goltz-Gorlin syndrome).

    Science.gov (United States)

    Motil, Kathleen J; Fete, Mary; Fete, Timothy J

    2016-03-01

    Focal dermal hypoplasia (FDH) is a rare genetic disorder caused by mutations in the PORCN gene located on the X-chromosome. In the present study, we characterized the pattern of growth, body composition, and the nutritional and gastrointestinal aspects of children and adults (n = 19) affected with this disorder using clinical anthropometry and a survey questionnaire. The mean birth length (P < 0.06) and weight (P < 0.001) z-scores of the participants were lower than the reference population. The mean head circumference (P < 0.001), height (length) (P < 0.001), weight (P < 0.01), and BMI (P < 0.05) for age z-scores of the participants were lower than the reference population. The height-for-age and weight-for-age z-scores of the participants did not differ significantly between birth and current measurements. Three-fourths of the group reported having one or more nutritional or gastrointestinal problems including short stature (65%), underweight (77%), oral motor dysfunction (41%), gastroesophageal reflux (24%), gastroparesis (35%), and constipation (35%). These observations provide novel clinical information about growth, body composition, and nutritional and gastrointestinal aspects of children and adults with FDH and underscore the importance of careful observation and early clinical intervention in the care of individuals affected with this disorder. © 2016 Wiley Periodicals, Inc.

  15. Enamel hypoplasias and physiological stress in the Sima de los Huesos Middle Pleistocene hominins.

    Science.gov (United States)

    Cunha, E; Rozzi, F Ramirez; Bermúdez de Castro, J M; Martinón-Torres, M; Wasterlain, S N; Sarmiento, S

    2004-11-01

    This study presents an analysis of linear enamel hypoplasias (LEH) and plane-form defects (PFD) in the hominine dental sample from the Sima de los Huesos (SH) Middle Pleistocene site in Atapuerca (Spain). The SH sample comprises 475 teeth, 467 permanent and 8 deciduous, belonging to a minimum of 28 individuals. The method for recording PFD and LEH is discussed, as well as the definition of LEH. The prevalence of LEH and PFD in SH permanent dentition (unilateral total count) is 4.6% (13/280). Only one deciduous tooth (lower dc) showed an enamel disruption. Prevalence by individual ranges from 18.7-30%. The most likely explanation for the relatively low LEH and PFD prevalence in the SH sample suggests that the SH population exhibited a low level of developmental stress. The age at occurrence of LEH and PFD was determined by counting the number of perikymata between each lesion and the cervix of the tooth. Assuming a periodicity of nine days for the incremental lines, the majority of LEH in the SH sample occurred during the third year of life and may be related to the metabolic stress associated with weaning.

  16. Vertebral Artery Hypoplasia and Posterior Circulation Infarction in Patients with Isolated Vertigo with Stroke Risk Factors.

    Science.gov (United States)

    Zhang, Dao Pei; Lu, Gui Feng; Zhang, Jie Wen; Zhang, Shu Ling; Ma, Qian Kun; Yin, Suo

    2017-02-01

    We aimed in this study to investigate the prevalence of vertebral artery hypoplasia (VAH) in a population with isolated vertigo in association with stroke risk factors, to determine whether VAH is an independent risk factor for posterior circulation infarction (PCI). We sequentially enrolled 245 patients with isolated vertigo with at least 1 vascular risk factor, who were divided into PCI and non-PCI groups, according to present signs of acute infarction on diffusion-weighted magnetic resonance imaging. All patients underwent magnetic resonance angiography and cervical contrast-enhanced magnetic resonance angiography to screen for VAH. Univariate and multivariate logistic regression analyses were performed to identify the significant risk factors for PCI. VAH was found in 64 of 245 patients (26%). VAH (odds ratio [OR] = 2.70, 95%confidence interval [CI] 1.17-6.23, P = .020), median stenosis of the posterior circulation (OR = 7.09, 95%CI = 2.54-19.79, P vertigo with PCI complicated by VAH was mainly small-artery occlusion. Our findings suggest that VAH is an independent risk factor for PCI in patients with isolated vertigo with confirmed risk from stroke. Copyright © 2017 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  17. Constitutive Activation of Smoothened in the Renal Collecting Ducts Leads to Renal Hypoplasia, Hydronephrosis, and Hydroureter.

    Science.gov (United States)

    Gupta, Deepak Prasad; Hwang, Jae-Won; Cho, Eui-Sic; Kim, Won; Song, Chang Ho; Chai, Ok Hee

    2017-01-01

    Sonic Hedgehog (Shh) signaling plays a major role in and is essential for regulation, patterning, and proliferation during renal development. Smoothened (Smo) plays a pivot role in transducing the Shh-glioma-associated oncogene Kruppel family member. However, the cellular and molecular mechanism underlying the role of sustained Smo activation in postnatal kidney development is still not clearly understood. Using a conditional knockin mouse model that expresses a constitutively activated form of Smo (SmoM2) upon Homeobox-B7-mediated recombination (Hoxb7-Cre), the effects of Shh signaling were determined in postnatal kidney development. SmoM2;Hoxb7-Cre mutant mice showed growth retardation with a reduction of body weight. Constitutive activation of Smo in the renal collecting ducts caused renal hypoplasia, hydronephrosis, and hydroureter. The parenchymal area and glomerular numbers were reduced, but the glomerular density was increased in SmoM2;Hoxb7-Cre mutant mice. The expression of Patched 1, the receptor of Shh and a downstream target gene of the Shh signaling pathway, was highly restricted and it was upregulated in the inner medullary collecting ducts of the kidney. The proliferative cells in the mesenchyme and collecting ducts were decreased in SmoM2;Hoxb7-Cre mutant mice. This study showed for the first time that sustained Smo inhibits postnatal kidney development by suppressing the proliferation of the mesenchyme and medullary collecting ducts in mice. © 2017 S. Karger AG, Basel.

  18. Spondylolysis and isthmic spondylolisthesis: impact of vertebral hypoplasia on the use of the Meyerding classification.

    Science.gov (United States)

    Niggemann, P; Kuchta, J; Grosskurth, D; Beyer, H K; Hoeffer, J; Delank, K S

    2012-04-01

    Spondylolysis and isthmic spondylolisthesis are common multifactorial disorders. The extent of slipping of the spondylolytic vertebra is considered a major predicator for prognosis and further follow-up. Vertebral hypoplasia is a common finding associated with spondylolysis. The purpose of this study is to evaluate the incidence of hypoplastic vertebral bodies in patients with spondylolysis and in the general population and to analyse the impact of the findings on the measurement and grading of spondylolisthesis. 140 patients with 141 levels of spondylolysis identified by MRI were included in this study. The slippage of the spondylolytic vertebral body and the size in the midline sagittal image were measured and correlated. In addition, a randomised control group was evaluated to test the hypothesis that shortened, hypoplastic vertebral bodies can also be found in the general population. Shortened, hypoplastic vertebrae were found in 50 patients with spondylolysis and none was found in the control group. These shortened vertebrae mimicked spondylolisthesis and in 19 patients the slippage equalled the shortening, thus mimicking spondylolisthesis, although only spondylolysis was present. Sagittal shortening of the spondylolytic vertebra is common and may mimic spondylolisthesis. In order to define and measure spondylolisthesis the shortening of the spondylolytic vertebra has to be taken into account.

  19. Yeast artificial chromosome cloning in the glycerol kinase and adrenal hypoplasia congenita region of Xp21

    Energy Technology Data Exchange (ETDEWEB)

    Worley, K.C.; Ellison, K.A.; Zhang, Y.H.; Wang, D.F.; Mason, J.; Roth, E.J.; Adams, V.; Fogt, D.D.; Zhu, X.M.; Towbin, J.A. [Baylor College of Medicine, Houston, TX (United States)] [and others

    1993-05-01

    The adrenal hypoplasia congenita (AHC) and glycerol kinase (GK) loci are telomeric to the Duchenne muscular dystrophy locus in Xp21. The authors developed a pair of yeast artificial chromosome (YAC) contigs spanning at least 1.2 Mb and encompassing the region from the telomeric end of the Duchenne muscular dystrophy (DMD) locus to beyond YHX39 (DXS727), including the genes for AHC and GK. The centromeric contig consists of 13 YACs reaching more than 600 kb from DMD through GK. The telomeric contig group consists of 8 YACs containing more than 600 kb including the markers YHX39 (DXS727) and QST-59 (DXS319). Patient deletion breakpoints in the region of the two YAC contigs define at least eight intervals, and seven deletion breakpoints are contained within these contigs. In addition to the probes developed from YAC ends, they have mapped eight Alu-PCR probes amplified from a radiation-reduced somatic cell hybrid, two anonymous DNA probes, and one Alu-PCR product amplified from a cosmid end, for a total of 26 new markers within this region of 2 Mb or less. One YAC in the centromeric contig contains an insert encompassing the minimum interval for GK deficiency defined by patient deletion breakpoints, and this clone includes all or part of the GK gene. 33 refs., 3 figs., 5 tabs.

  20. Clinical and immunologic outcome of patients with cartilage hair hypoplasia after hematopoietic stem cell transplantation.

    Science.gov (United States)

    Bordon, Victoria; Gennery, Andrew R; Slatter, Mary A; Vandecruys, Els; Laureys, Genevieve; Veys, Paul; Qasim, Waseem; Waseem, Qasim; Friedrich, Wilhelm; Wulfraat, Nico M; Scherer, Franziska; Cant, Andrew J; Fischer, Alain; Cavazzana-Calvo, Marina; Cavazanna-Calvo, Marina; Bredius, Robbert G M; Notarangelo, Luigi D; Mazzolari, Evelina; Neven, Benedicte; Güngör, Tayfun; Tayfun, Güngör

    2010-07-08

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disease caused by mutations in the RMRP gene. Beside dwarfism, CHH has a wide spectrum of clinical manifestations including variable grades of combined immunodeficiency, autoimmune complications, and malignancies. Previous reports in single CHH patients with significant immunodeficiencies have demonstrated that allogeneic hematopoietic stem cell transplantation (HSCT) is an effective treatment for the severe immunodeficiency, while growth failure remains unaffected. Because long-term experience in larger cohorts of CHH patients after HSCT is currently unreported, we performed a European collaborative survey reporting on 16 patients with CHH and immunodeficiency who underwent HSCT. Immune dysregulation, lymphoid malignancy, and autoimmunity were important features in this cohort. Thirteen patients were transplanted in early childhood ( approximately 2.5 years). The other 3 patients were transplanted at adolescent age. Of 16 patients, 10 (62.5%) were long-term survivors, with a median follow-up of 7 years. T-lymphocyte numbers and function have normalized, and autoimmunity has resolved in all survivors. HSCT should be considered in CHH patients with severe immunodeficiency/autoimmunity, before the development of severe infections, major organ damage, or malignancy might jeopardize the outcome of HSCT and the quality of life in these patients.

  1. Serum prolactin concentrations in relation to hypopituitarism and obesity in children with optic nerve hypoplasia.

    Science.gov (United States)

    Vedin, Amy M; Garcia-Filion, Pamela; Fink, Cassandra; Borchert, Mark; Geffner, Mitchell E

    2012-01-01

    The majority of children with optic nerve hypoplasia (ONH) develop hypopituitarism and many also become obese. These associated conditions are a major cause of morbidity and are possibly due to hypothalamic dysfunction. Because mild hyperprolactinemia often occurs in subjects with disorders of the hypothalamus, we examined whether hyperprolactinemia was present in children with ONH during the first 3 years of life and whether it was a marker for hypopituitarism and/or obesity. Data were retrospectively analyzed from a registry study of children with ONH. The initial serum prolactin was obtained prior to age 36 months (n = 125) and compared with pituitary function and body mass index at age 5. 72% of subjects had an elevated initial serum prolactin and 60% had hypopituitarism. An elevated initial prolactin was associated with hypopituitarism (OR 2.58; 95% CI 1.16, 5.73), specifically with growth hormone deficiency (OR 2.77; 95% CI 1.21, 6.34). 31% of subjects had a body mass index ≥ 85th percentile, but this did not correlate with initial hyperprolactinemia. Early hyperprolactinemia correlates with the presence of hypopituitarism in children with ONH, but it is not a reliable prognosticator of hypopituitarism. Additionally, hyperprolactinemia does not predict future weight excess. Copyright © 2012 S. Karger AG, Basel.

  2. Compliance between clinical and genetic diagnosis of choroidal hypoplasia in 103 Norwegian Border Collie puppies.

    Science.gov (United States)

    Grosås, Siv; Lingaas, Frode; Prestrud, Kristin Wear; Ropstad, Ernst-Otto

    2017-11-07

    To describe the frequency of the nonhomologous end-joining factor 1 (NHEJ1) mutation and the compliance between clinical and genetic diagnosis of choroidal hypoplasia (CH) in a group of Norwegian Border Collies. Border collie puppies in the age from 5 to 8 weeks. Puppies included in the study had a complete ophthalmological examination. All findings were recorded, and an ECVO scheme form was issued for each puppy. DNA samples were achieved from buccal swabs. Genetic typing was performed for the 7.8-kb deletion in the gene encoding NHEJ1. Dogs with none, one, or two copies of the mutated allele were classified as free, carriers, and affected, respectively. 103 Border Collie puppies from 16 litters, 52 females and 51 males, were included in the study. Ages ranged from 5.1 to 8.9 weeks. One puppy had clinical findings consistent with CH and optic nerve coloboma compatible with the diagnosis Collie Eye Anomaly (CEA). Findings on ophthalmological examination of the remaining puppies were within normal limits. On genetic testing, 85 puppies were clear of the mutation in the NHEJ1 gene, 17 puppies were carriers, and one puppy was genetically affected. A good compliance between the clinical diagnosis and the genetic test results was found in all of the puppies examined. The allele frequency of the mutation was 6.3%. © 2017 American College of Veterinary Ophthalmologists.

  3. Helical Tomotherapy Planning for Left-Sided Breast Cancer Patients With Positive Lymph Nodes: Comparison to Conventional Multiport Breast Technique

    International Nuclear Information System (INIS)

    Goddu, S. Murty; Chaudhari, Summer; Mamalui-Hunter, Maria; Pechenaya, Olga L.; Pratt, David; Mutic, Sasa; Zoberi, Imran; Jeswani, Sam; Powell, Simon N.; Low, Daniel A.

    2009-01-01

    Purpose: To evaluate the feasibility of using helical tomotherapy for locally advanced left-sided breast cancer. Methods and Materials: Treatment plans were generated for 10 left-sided breast cancer patients with positive lymph nodes comparing a multiport breast (three-dimensional) technique with the tomotherapy treatment planning system. The planning target volumes, including the chest wall/breast, supraclavicular, axillary, and internal mammary lymph nodes, were contoured. The treatment plans were generated on the tomotherapy treatment planning system to deliver 50.4 Gy to the planning target volume. To spare the contralateral tissues, directional blocking was applied to the right breast and right lung. The optimization goals were to protect the lungs, heart, and right breast. Results: The tomotherapy plans increased the minimal dose to the planning target volume (minimal dose received by 99% of target volume = 46.2 ± 1.3 Gy vs. 27.9 ± 17.1 Gy) while improving the dose homogeneity (dose difference between the minimal dose received by 5% and 95% of the planning target volume = 7.5 ± 1.8 Gy vs. 37.5 ± 26.9 Gy). The mean percentage of the left lung volume receiving ≥20 Gy in the tomotherapy plans decreased from 32.6% ± 4.1% to 17.6% ± 3.5%, while restricting the right-lung mean dose to <5 Gy. However, the mean percentage of volume receiving ≥5 Gy for the total lung increased from 25.2% ± 4.2% for the three-dimensional technique to 46.9% ± 8.4% for the tomotherapy plan. The mean volume receiving ≥35 Gy for the heart decreased from 5.6% ± 4.8% to 2.2% ± 1.5% in the tomotherapy plans. However, the mean heart dose for tomotherapy delivery increased from 7.5 ± 3.4 Gy to 12.2 ± 1.8 Gy. Conclusion: The tomotherapy plans provided better dose conformity and homogeneity than did the three-dimensional plans for treatment of left-sided breast tumors with regional lymph node involvement, while allowing greater sparing of the heart and left lung from doses

  4. Frequency and developmental timing of linear enamel hypoplasia defects in Early Archaic Texan hunter-gatherers

    Directory of Open Access Journals (Sweden)

    J. Colette Berbesque

    2018-02-01

    Full Text Available Digital photographs taken under controlled conditions were used to examine the incidence of linear enamel hypoplasia defects (LEHs in burials from the Buckeye Knoll archaeological site (41VT98 Victoria county, Texas, which spans the Early to Late Archaic Period (ca. 2,500–6,500 BP uncorrected radiocarbon. The majority (68 of 74 burials date to the Texas Early Archaic, including one extremely early burial dated to 8,500 BP. The photogrammetric data collection method also results in an archive for Buckeye Knoll, a significant rare Archaic period collection that has been repatriated and reinterred. We analyzed the incidence and developmental timing of LEHs in permanent canines. Fifty-nine percent of permanent canines (n = 54 had at least one defect. There were no significant differences in LEH frequency between the maxillary and mandibular canines (U = 640.5, n1 = 37, n2 = 43, p = .110. The sample studied (n = 92 permanent canines had an overall mean of 0.93 LEH defect per tooth, with a median of one defect, and a mode of zero defects. Average age at first insult was 3.92 (median = 4.00, range = 2.5–5.4 and the mean age of all insults per individual was 4.18 years old (range = 2.5–5.67. Age at first insult is consistent with onset of weaning stress—the weaning age range for hunter-gatherer societies is 1–4.5. Having an earlier age of first insult was associated with having more LEHs (n = 54, rho = −0.381, p = 0.005.

  5. Cardiac dose-sparing effects of deep-inspiration breath-hold in left breast irradiation : Is IMRT more beneficial than VMAT?

    Science.gov (United States)

    Sakka, Mazen; Kunzelmann, Leonie; Metzger, Martin; Grabenbauer, Gerhard G

    2017-10-01

    .01) techniques as compared with FB radiation. There were no significant changes in left lung dose by IMRT; however, with VMAT planning, mean dose to the left lung was reduced by -4% (p = 0.0004). In addition, DIBH significantly increased the mean dose to the contralateral breast with IMRT (+14%, p = 0.002) and significantly reduced the dose to the contralateral breast with VMAT planning (-9%, p = 0.003) compared with the FB position. Additionally, in comparison with VMAT, the IMRT technique reduced mean heart dose both in the FB and the DIBH-position by -30% (p = 0.0004) and -26% (p = 0.002), respectively. Furthermore, IMRT increased the mean dose to the left lung in both the FB and the DIBH position (+5%, p = 0.003, p = 0.006), respectively. There were no significant changes in dose to the right lung and contralateral breast either in the FB or DIBH position between IMRT and VMAT techniques. Left-sided breast irradiation is best performed in the DIBH position, since a considerable dose sparing to the heart and LADCA can be achieved by using either IMRT or VMAT techniques. A significant additional decrease in heart and LADCA dose by IMRT in both FB and DIBH irradiation was seen compared with VMAT.

  6. Left heart ventricular angiography

    Science.gov (United States)

    ... blood vessels. These x-ray pictures create a "movie" of the left ventricle as it contracts rhythmically. ... 22578925 www.ncbi.nlm.nih.gov/pubmed/22578925 . Review Date 9/26/2016 Updated by: Michael A. ...

  7. Left heart catheterization

    Science.gov (United States)

    Catheterization - left heart ... to help guide the catheters up into your heart and arteries. Dye (sometimes called "contrast") will be ... in the blood vessels that lead to your heart. The catheter is then moved through the aortic ...

  8. Enamel hypoplasia in deciduous teeth of great apes: do differences in defect prevalence imply differential levels of physiological stress?

    Science.gov (United States)

    Lukacs, J R

    1999-11-01

    This paper presents new data on enamel hypoplasia in the deciduous canine teeth of great apes. The enamel defect under consideration is known as localized hypoplasia of primary canines (LHPC), and is characterized by an area of thin or missing enamel on the labial surface of deciduous canine teeth (Skinner [1986a] Am. J. Phys. Anthropol. 69:59-69). Goals of this study are: 1) to determine if significant differences in the frequency of LHPC occur among three genera of great apes, and 2) to evaluate variation in LHPC prevalence among great apes as evidence of differential physiological stress. Infant and juvenile apes with deciduous teeth were examined at the Cleveland Museum of Natural History (n = 100) and at the Smithsonian Institution, National Museum of Natural History (n = 36). Deciduous teeth were observed under oblique incandescent light, with the naked eye and with a 10x hand lens. Enamel hypoplasia was scored using Federation Dentaire International (FDI)-Defects of Dental Enamel (DDE) standards. Hypoplasias were recorded by drawing defect location and size on a dental chart, and by measuring defect size and location with Helios needlepoint dial calipers. The prevalence of LHPC is reported by genus and sex, using two approaches: 1) the frequency of affected individuals-those having one or more deciduous canine teeth scored positive for LHPC; and 2) the number of canine teeth scored positive for LHPC as a percentage of all canine teeth observed. Variation in defect size and location will be described elsewhere. Localized hypoplasia of primary canine teeth was found in 62.5% of 128 individual apes, and in 45.5% of 398 great ape deciduous canines. As in humans, LHPC is the most common form of enamel hypoplasia in deciduous teeth of great apes, while LEH is rare or absent. The distribution and pattern of expression of LHPC in great apes is similar to that described in humans: side differences are not significant, but mandibular canines exhibit the defect two to

  9. Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

    Science.gov (United States)

    Webb, Emma A; O'Reilly, Michelle A; Clayden, Jonathan D; Seunarine, Kiran K; Dale, Naomi; Salt, Alison; Clark, Chris A; Dattani, Mehul T

    2013-01-01

    To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years) underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years) underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex) was performed. Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (pchildren with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, pchildren with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic radiations of children with mild to moderate or no visual impairment raises questions as to the pathogenesis of these changes which will need to be addressed by future studies.

  10. Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2015-07-01

    Full Text Available We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.

  11. The first Japanese patient with mandibular hypoplasia, deafness, progeroid features and lipodystrophy diagnosed via POLD1 mutation detection.

    Science.gov (United States)

    Okada, Asami; Kohmoto, Tomohiro; Naruto, Takuya; Yokota, Ichiro; Kotani, Yumiko; Shimada, Aki; Miyamoto, Yoko; Takahashi, Rizu; Goji, Aya; Masuda, Kiyoshi; Kagami, Shoji; Imoto, Issei

    2017-01-01

    Mandibular hypoplasia, deafness, progeroid features and lipodystrophy (MDPL) syndrome is a rare autosomal dominant disorder caused by heterozygous POLD1 mutations. To date, 13 patients affected by POLD1 mutation-caused MDPL have been described. We report a clinically undiagnosed 11-year-old male who noted joint contractures at 6 years of age. Targeted exome sequencing identified a known POLD1 mutation [NM_002691.3:c.1812_1814del, p.(Ser605del)] that diagnosed him as the first Japanese/East Asian MDPL case.

  12. A Giant Gastroschisis Associated with Pulmonary Hypoplasia and Spinal Anomaly: A Case Report and a Literature Review

    Directory of Open Access Journals (Sweden)

    Surasak Puvabanditsin

    2018-01-01

    Full Text Available Gastroschisis most often occurs as an isolated anomaly and extragastrointestinal associations are rare. Most commonly, the anomalies associated with gastroschisis are cardiac and central nervous system abnormalities. Respiratory insufficiency has sometimes been reported in association with giant abdominal wall defects. Poor outcomes and prolonged ventilator support have been reported in giant gastroschisis and omphalocele, especially if associated with herniation of the majority of the liver. We report a case of a large gastroschisis that was associated with a kyphoscoliosis and pulmonary hypoplasia.

  13. Congenital dislocation of the deep digital flexor tendon associated with hypoplasia of the sustentaculum tali in a Thoroughbred colt

    International Nuclear Information System (INIS)

    Lepage, O.M.; Leveille, R.; Breton, L.; Marcoux, M.

    1995-01-01

    An 11-month-old Thoroughbred colt was presented with a hard swelling at the proximal third of the right 4th metatarsal bone. A medial dislocation of the deep digital flexor tendon (flexor digitorum profundus) was also observed on the same leg. On the plantaroproximal-plantarodistal projection of the calcaneus, there was flattening and shortening of the sustentaculum tali. The smooth bony proliferation at the proximal third of the right 4th metatarus was compatible with a chronic splint bone fracture. This report describes a medial deep digital flexor dislocation associated with hypoplasia of the sustentaculum tali

  14. [Successful One-lung Ventilation with a Right-sided Double-lumen Tube in a Patient with a Right Upper Tracheal Bronchus, who Underwent Left Pneumonectomy for Left Hilar Lung Cancer].

    Science.gov (United States)

    Kawagoe, Izumi; Kohchiyama, Tsukasa; Hayashida, Masakazu; Satoh, Daizoh; Suzuki, Kenji; Inada, Eiichi

    2016-06-01

    A 60-year-old male patient with left hilar lung cancer was scheduled to undergo left pneumonectomy or left sleeve lower lobectomy. Preoperative computer tomographic and bronchoscopic examinations revealed that the bronchus (B1) to the right apical segment (S1) was a tracheal bronchus (TB) originating from the trachea approximately 10 mm above the carina. Because the left main bronchus was to be dissected, a right-sided double-lumen tube (DLT) was selected to completely protect the right lung from spillage of secretions or cancer cells from the left lung. The right-sided DLT was placed so as to fit its lateral opening of the bronchial lumen to normal upper branches (B2, B3), while sacrificing ventilation of S1 with an abnormal branch (B1). However, one-lung ventilation (OLV) of the right lung could not be achieved, since a gas leakage from the opened tracheal lumen occurred, most probably due to intra-lobar micro-airway communications between S1 and S2/S3. The DLT was withdrawn until the blue bronchial cuff occluded the orifice of the TB (B1). Although the upper half of the blue bronchial cuff appeared above the tracheal carina, OLV through the two bronchial lumen openings could be achieved due to a specific, slanted doughnut shape of the blue bronchial cuff and the location of the abnormal branch (B1) approximate to the carina. Left pneumonectomy using successful OLV was completed safely without hypoxemia or hypercapnea. Our experience indicates that management of OLV for patients with a thoracheal bronchus needs special considerations of the exact location of the TB and intra-lobar micro-airway communications, in addition to types of scheduled surgical procedures.

  15. VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

    Directory of Open Access Journals (Sweden)

    L. Sbragia

    2014-02-01

    Full Text Available Changes in vascular endothelial growth factor (VEGF in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1 and VEGFR2 (Flk-1, in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each of four different gestational days (GD 18.5, 19.5, 20.5, 21.5: external control (EC, exposed to olive oil (OO, exposed to 100 mg nitrofen, by gavage, without CDH (N-, and exposed to nitrofen with CDH (CDH on GD 9.5 (term=22 days. The morphological variables studied were: body weight (BW, total lung weight (TLW, left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216. All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05 and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression.

  16. VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

    Energy Technology Data Exchange (ETDEWEB)

    Sbragia, L. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Nassr, A.C.C. [Departamento de Hidrobiologia do Centro de Ciências Biológicas e da Saúde, Universidade Federal de São Carlos, São Carlos, SP, Brasil, Departamento de Hidrobiologia do Centro de Ciências Biológicas e da Saúde, Universidade Federal de São Carlos, São Carlos, SP (Brazil); Gonçalves, F.L.L. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Schmidt, A.F. [Pediatrics House Office, Cincinnati Children' s Hospital Medical Center, Cincinnati, OH, USA, Pediatrics House Office, Cincinnati Children' s Hospital Medical Center, Cincinnati, OH (United States); Zuliani, C.C. [Departamento de Clínica Médica, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, SP, Brasil, Departamento de Clínica Médica, Faculdade de Ciências Médicas, Universidade Estadual de Campinas, Campinas, SP (Brazil); Garcia, P.V. [Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP, Brasil, Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP (Brazil); Gallindo, R.M. [Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil, Divisão de Cirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP (Brazil); Pereira, L.A.V. [Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP, Brasil, Departamento de Histologia e Embriologia, Instituto de Biologia, Universidade Estadual de Campinas, UNICAMP, Campinas, SP (Brazil)

    2014-02-17

    Changes in vascular endothelial growth factor (VEGF) in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH) and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1) and VEGFR2 (Flk-1), in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each) of four different gestational days (GD) 18.5, 19.5, 20.5, 21.5: external control (EC), exposed to olive oil (OO), exposed to 100 mg nitrofen, by gavage, without CDH (N-), and exposed to nitrofen with CDH (CDH) on GD 9.5 (term=22 days). The morphological variables studied were: body weight (BW), total lung weight (TLW), left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216). All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05) and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression.

  17. VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

    International Nuclear Information System (INIS)

    Sbragia, L.; Nassr, A.C.C.; Gonçalves, F.L.L.; Schmidt, A.F.; Zuliani, C.C.; Garcia, P.V.; Gallindo, R.M.; Pereira, L.A.V.

    2014-01-01

    Changes in vascular endothelial growth factor (VEGF) in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH) and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1) and VEGFR2 (Flk-1), in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each) of four different gestational days (GD) 18.5, 19.5, 20.5, 21.5: external control (EC), exposed to olive oil (OO), exposed to 100 mg nitrofen, by gavage, without CDH (N-), and exposed to nitrofen with CDH (CDH) on GD 9.5 (term=22 days). The morphological variables studied were: body weight (BW), total lung weight (TLW), left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216). All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05) and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression

  18. Anterior maxillary segmental distraction for correction of maxillary hypoplasia and dental crowding in cleft palate patients: a preliminary report.

    Science.gov (United States)

    Wang, X-X; Wang, X; Li, Z-L; Yi, B; Liang, C; Jia, Y-L; Zou, B-S

    2009-12-01

    To evaluate the feasibility of anterior maxillary segmental distraction (AMSD) to correct maxillary hypoplasia and severe dental crowding in cleft lip and palate (CLP) patients, 7 patients (average age 16.4 years) with maxillary hypoplasia, shortened maxillary dental arch length and severe anterior dental crowding secondary to CLP were selected for this study. After anterior maxillary segmental osteotomy, 3 patients were treated using bilateral internal distraction devices, and 4 patients were treated using rigid external distraction devices. Photographs and radiographs were taken to review the improvement in facial profile and occlusion after distraction. An average 10.25 mm anterior maxillary advancement was obtained in all patients after 10-23 days of distraction and 9-16 weeks of consolidation. The sella-nasion-point A (SNA) angle increased from 69.5 degrees to 79.6 degrees. Midface convexity was greatly improved and velopharyngeal competence was preserved. The maxillary dental arch length was greatly increased by 10.1 mm (P<0.01). Dental crowding and malocclusion were corrected by orthodontic treatment. These results show that AMSD can effectively correct the hypoplastic maxilla and severe dental crowding associated with CLP by increasing the midface convexity and dental arch length while preserving velopharyngeal function, and dental crowding can be corrected without requiring tooth extraction.

  19. Distraction osteogenesis versus orthognathic surgery for the treatment of maxillary hypoplasia in cleft lip and palate patients: a systematic review.

    Science.gov (United States)

    Austin, S L; Mattick, C R; Waterhouse, P J

    2015-05-01

    To compare the effectiveness of distraction osteogenesis to orthognathic surgery for the treatment of maxillary hypoplasia in individuals with cleft lip and palate. A systematic review of prospective randomized, quasi-randomized or controlled clinical trials. MEDLINE, EMBASE, Scopus, Web of Science, CINAHL, CENTRAL, trial registers and grey literature were searched. Hand searching of five relevant journals was completed. Two reviewers independently completed inclusion assessment. Data extraction and risk of bias assessment were completed by a single reviewer and checked by a second reviewer. Five publications all reporting different outcomes of a single randomized controlled trial are included within the review. The quality of the evidence was low with a high risk of bias. Both surgical interventions produce significant soft tissue improvement. Horizontal relapse of the maxilla was statistically significantly greater following orthognathic surgery. There was no statistically significant difference in speech and velo-pharyngeal function between the interventions. Maxillary distraction initially lowered social self-esteem, but this improved with time resulting in higher satisfaction with life in the long term. The low quality of evidence included within the review means there is insufficient evidence to conclude whether there is a difference in effectiveness between maxillary distraction and osteotomy for the treatment of cleft-related maxillary hypoplasia. There is a need for further high-quality randomized controlled trials to allow conclusive recommendations to be made. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Evaluation of posterior lateral femoral condylar hypoplasia using axial MRI images in patients with complete discoid meniscus.

    Science.gov (United States)

    Xu, Zhihong; Chen, Dongyang; Shi, Dongquan; Dai, Jin; Yao, Yao; Jiang, Qing

    2016-03-01

    Hypoplasia of the lateral femoral condyle has been reported in discoid lateral meniscus patients, but associated imaging findings in the axial plane have not been characterized. In this study, we aimed to identify differences in the lateral femoral condyle between patients with discoid lateral meniscus and those with normal menisci using axial MRI images. Twenty-three patients (24 knees) with complete discoid lateral meniscus, 43 (45 knees) with incomplete discoid lateral meniscus, and 50 with normal menisci (50 knees) were enrolled and distributed into three groups. Two new angles, posterior lateral condylar angle (PLCA) and posterior medial condylar angle (PMCA), were measured on axial MRI images; the posterior condylar angle (PCA) was also measured. Differences between the three groups in the PLCA, PMCA, PCA, and PLCA/PMCA were analysed. The predictive value of PLCA and PLCA/PMCA for complete discoid lateral meniscus was assessed. In the complete discoid lateral meniscus group, PLCA and PLCA/PMCA were significantly smaller compared with the normal meniscus group and the incomplete discoid lateral meniscus group (P meniscus group compared with the incomplete discoid lateral meniscus group (P meniscus group (P meniscus. Hypoplasia of the posterior lateral femoral condyle is typically seen in patients with complete discoid lateral meniscus. PLCA and PLCA/PMCA can be measured from axial MRI images and used as excellent predictive parameters for complete discoid lateral meniscus. Diagnostic study, Level III.

  1. Application of mitomycin C after endoscopic lysis of congenital laryngeal web combined with epiglottic hypoplasia in a middle-aged man.

    Science.gov (United States)

    Roh, Jong-Lyel

    2006-04-01

    Laryngeal webs and epiglottic hypoplasias are uncommon congenital anomalies. Anterior glottic web combined with epiglottic hypoplasia was found in a middle-aged man presenting with hoarseness and dyspnea on exertion. This can be considered as a unique isolated defect of the larynx during early fetal development. The laryngeal web can be successfully treated in a single stage with endoscopic lysis and topical application of mitomycin C for prevention of anterior glottic restenosis. This case and prior reports suggest that the novel approach may be effective in the treatment of laryngeal webs.

  2. Triplets with growth failure, microcephaly, mental retardation, nail hypoplasia and corpus callosum agenesis: is it a variant of Coffin-Siris or a new syndrome?

    Science.gov (United States)

    Kirel, B; Kural, N; Yakut, A; Adapinar, B

    2000-01-01

    We report eight-year-old triplet girls whose clinical features included microcephaly, severe mental retardation, hypoplasia of distal phalanges of both fifth and second fingers and nail hypoplasia on second fingers, dysmorphic facial features, and partial corpus callosum agenesis. During infancy, a Pavlik harness was used for congenital hip dislocation, and they had difficulty in feeding. One had been operated for patent ductus arteriosus. To our knowledge, this rare combination has not been previously reported in triplets whose clinical features closely resemble those of Coffin-Siris syndrome. The other diagnostic possibilities are also reviewed.

  3. No Community Left Behind

    Science.gov (United States)

    Schlechty, Phillip C.

    2008-01-01

    The debate over the reauthorization of No Child Left Behind (NCLB) generally overlooks--or looks past--what may be the most fundamental flaw in that legislation. As the law is now written, decisions regarding what the young should know and be able to do are removed from the hands of parents and local community leaders and turned over to officials…

  4. The Children Left Behind

    Science.gov (United States)

    Gillard, Sarah A.; Gillard, Sharlett

    2012-01-01

    This article explores some of the deficits in our educational system in regard to non-hearing students. It has become agonizingly clear that non-hearing students are being left out of the gallant sweep to enrich our children's educations. The big five areas of literacy, at best, present unique challenges for non-hearing students and, in some…

  5. Left atrial appendage occlusion

    Directory of Open Access Journals (Sweden)

    Ahmad Mirdamadi

    2013-01-01

    Full Text Available Left atrial appendage (LAA occlusion is a treatment strategy to prevent blood clot formation in atrial appendage. Although, LAA occlusion usually was done by catheter-based techniques, especially percutaneous trans-luminal mitral commissurotomy (PTMC, it can be done during closed and open mitral valve commissurotomy (CMVC, OMVC and mitral valve replacement (MVR too. Nowadays, PTMC is performed as an optimal management of severe mitral stenosis (MS and many patients currently are treated by PTMC instead of previous surgical methods. One of the most important contraindications of PTMC is presence of clot in LAA. So, each patient who suffers of severe MS is evaluated by Trans-Esophageal Echocardiogram to rule out thrombus in LAA before PTMC. At open heart surgery, replacement of the mitral valve was performed for 49-year-old woman. Also, left atrial appendage occlusion was done during surgery. Immediately after surgery, echocardiography demonstrates an echo imitated the presence of a thrombus in left atrial appendage area, although there was not any evidence of thrombus in pre-pump TEE. We can conclude from this case report that when we suspect of thrombus of left atrial, we should obtain exact history of previous surgery of mitral valve to avoid misdiagnosis clotted LAA, instead of obliterated LAA. Consequently, it can prevent additional evaluations and treatments such as oral anticoagulation and exclusion or postponing surgeries including PTMC.

  6. Hypoplastic left heart syndrome

    Directory of Open Access Journals (Sweden)

    Thiagarajan Ravi

    2007-05-01

    Full Text Available Abstract Hypoplastic left heart syndrome(HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. HLHS has been reported to occur in approximately 0.016 to 0.036% of all live births. Newborn infants with the condition generally are born at full term and initially appear healthy. As the arterial duct closes, the systemic perfusion becomes decreased, resulting in hypoxemia, acidosis, and shock. Usually, no heart murmur, or a non-specific heart murmur, may be detected. The second heart sound is loud and single because of aortic atresia. Often the liver is enlarged secondary to congestive heart failure. The embryologic cause of the disease, as in the case of most congenital cardiac defects, is not fully known. The most useful diagnostic modality is the echocardiogram. The syndrome can be diagnosed by fetal echocardiography between 18 and 22 weeks of gestation. Differential diagnosis includes other left-sided obstructive lesions where the systemic circulation is dependent on ductal flow (critical aortic stenosis, coarctation of the aorta, interrupted aortic arch. Children with the syndrome require surgery as neonates, as they have duct-dependent systemic circulation. Currently, there are two major modalities, primary cardiac transplantation or a series of staged functionally univentricular palliations. The treatment chosen is dependent on the preference of the institution, its experience, and also preference. Although survival following initial surgical intervention has improved significantly over the last 20 years, significant mortality and morbidity are present for both surgical strategies. As a result pediatric cardiologists continue to be challenged by discussions with families regarding initial decision

  7. Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

    Energy Technology Data Exchange (ETDEWEB)

    Abolmaali, Nasreddin; Koch, Arne [Dresden University of Technology, OncoRay - Molecular and Biological Imaging, Medical Faculty Carl Gustav Carus, Dresden (Germany); Goetzelt, Knut; Vogelberg, Christian [University Clinics Carl Gustav Carus, Dresden University of Technology, Clinic and Policlinic for Pediatrics - Pediatric Pulmonology, Dresden (Germany); Hahn, Gabriele [University Clinics Carl Gustav Carus, Dresden University of Technology, Institute and Policlinic for Radiology - Pediatric Radiology, Dresden (Germany); Fitze, Guido [University Clinics Carl Gustav Carus, Dresden University of Technology, Clinic and Policlinic for Pediatric Surgery, Dresden (Germany)

    2010-07-15

    To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment. (orig.)

  8. Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results.

    Science.gov (United States)

    Abolmaali, Nasreddin; Koch, Arne; Götzelt, Knut; Hahn, Gabriele; Fitze, Guido; Vogelberg, Christian

    2010-07-01

    To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment.

  9. Lung volumes, ventricular function and pulmonary arterial flow in children operated on for left-sided congenital diaphragmatic hernia: long-term results

    International Nuclear Information System (INIS)

    Abolmaali, Nasreddin; Koch, Arne; Goetzelt, Knut; Vogelberg, Christian; Hahn, Gabriele; Fitze, Guido

    2010-01-01

    To compare MRI-based functional pulmonary and cardiac measurements in the long-term follow-up of children operated on for left-sided congenital diaphragmatic hernia (CDH) with age- and body size-matched healthy controls. Twelve children who received immediate postnatal surgery for closure of isolated left-sided CDH were included and received basic medical examinations, pulmonary function testing and echocardiography. MRI included measurement of lung volume, ventricular function assessment and velocity-encoded imaging of the pulmonary arteries and was compared with the data for 12 healthy children matched for age and body size. While patients' clinical test results were not suspicious, comparison between the MRI data for patients and those for healthy controls revealed significant differences. In patients, the volumes of the left lungs were increased and the tidal volume was larger on the right side. While the stroke volumes of both ventricles were reduced, heart rate and ejection fraction were increased. Flow, acceleration time and cross-sectional area of the left pulmonary artery were reduced. Functional MRI detected pulmonary and cardiac findings in the late follow-up of CDH children which may be missed by standard clinical methods and might be relevant for decisions regarding late outcome and treatment. (orig.)

  10. Left Ventricular Assist Devices

    Directory of Open Access Journals (Sweden)

    Khuansiri Narajeenron

    2017-04-01

    Full Text Available Audience: The audience for this classic team-based learning (cTBL session is emergency medicine residents, faculty, and students; although this topic is applicable to internal medicine and family medicine residents. Introduction: A left ventricular assist device (LVAD is a mechanical circulatory support device that can be placed in critically-ill patients who have poor left ventricular function. After LVAD implantation, patients have improved quality of life.1 The number of LVAD patients worldwide continues to rise. Left-ventricular assist device patients may present to the emergency department (ED with severe, life-threatening conditions. It is essential that emergency physicians have a good understanding of LVADs and their complications. Objectives: Upon completion of this cTBL module, the learner will be able to: 1 Properly assess LVAD patients’ circulatory status; 2 appropriately resuscitate LVAD patients; 3 identify common LVAD complications; 4 evaluate and appropriately manage patients with LVAD malfunctions. Method: The method for this didactic session is cTBL.

  11. Cerebellar and brainstem hypoplasia in a child with a partial monosomy for the short arm of chromosome 5 and partial trisomy for the short arm of chromosome 10

    NARCIS (Netherlands)

    Arts, W F M; Hofstee, Y; Drejer, G F; Beverstock, G C; Oosterwijk, J C

    A child with hypoplasia of the cerebellum and brainstem in association with an unbalanced translocation, resulting in a partial deletion of the short arm of chromosome 5 and a partial trisomy of the short arm of chromosome 10, is described. A balanced translocation was present in his mother and

  12. A novel single nucleotide splice site mutation in FHL1 confirms an Emery-Dreifuss plus phenotype with pulmonary artery hypoplasia and facial dysmorphology

    NARCIS (Netherlands)

    Pen, A.E.; Nyegaard, M.; Fang, M.; Jiang, H.; Christensen, R.; Molgaard, H.; Andersen, H.; Ulhoi, B.P.; Ostergaard, J.R.; Vaeth, S.; Sommerlund, M.; Brouwer, A.P.M. de; Zhang, X.; Jensen, U.B.

    2015-01-01

    We describe a Danish family with an, until recently, unknown X-linked disease with muscular dystrophy (MD), facial dysmorphology and pulmonary artery hypoplasia. One patient died suddenly before age 20 and another was resuscitated from cardiac arrest at the age of 28. Linkage analysis pointed to a

  13. Inherited occipital hypoplasia/syringomyelia in the cavalier King Charles spaniel: experiences in setting up a worldwide DNA collection.

    Science.gov (United States)

    Rusbridge, Clare; Knowler, Penny; Rouleau, Guy A; Minassian, Berge A; Rothuizen, Jan

    2005-01-01

    Inherited diseases commonly emerge within pedigree dog populations, often due to use of repeatedly bred carrier sire(s) within a small gene pool. Accurate family records are usually available making linkage analysis possible. However, there are many factors that are intrinsically difficult about collecting DNA and collating pedigree information from a large canine population. The keys to a successful DNA collection program include (1) the need to establish and maintain support from the pedigree breed clubs and pet owners; (2) committed individual(s) who can devote the considerable amount of time and energy to coordinating sample collection and communicating with breeders and clubs; and (3) providing means by which genotypic and phenotypic information can be easily collected and stored. In this article we described the clinical characteristics of inherited occipital hypoplasia/syringomyelia (Chiari type I malformation) in the cavalier King Charles spaniel and our experiences in establishing a pedigree and DNA database to study the disease.

  14. Left regular bands of groups of left quotients

    International Nuclear Information System (INIS)

    El-Qallali, A.

    1988-10-01

    A semigroup S which has a left regular band of groups as a semigroup of left quotients is shown to be the semigroup which is a left regular band of right reversible cancellative semigroups. An alternative characterization is provided by using spinned products. These results are applied to the case where S is a superabundant whose set of idempotents forms a left normal band. (author). 13 refs

  15. Why Dora Left

    DEFF Research Database (Denmark)

    Gammelgård, Judy

    2017-01-01

    The question of why Dora left her treatment before it was brought to a satisfactory end and the equally important question of why Freud chose to publish this problematic and fragmentary story have both been dealt with at great length by Freud’s successors. Dora has been read by analysts, literary...... problem toward femininity, both Dora’s and his own. In Dora, it is argued, Freud took a new stance toward the object of his investigation, speaking from the position of the master. Freud presents himself as the one who knows, in great contrast to the position he takes when unraveling the dream. Here he...

  16. Neutrosophic Left Almost Semigroup

    Directory of Open Access Journals (Sweden)

    Mumtaz Ali

    2014-06-01

    Full Text Available In this paper we extend the theory of neutrosophy to study left almost semigroup shortly LAsemigroup. We generalize the concepts of LA-semigroup to form that for neutrosophic LA-semigroup. We also extend the ideal theory of LA-semigroup to neutrosophy and discuss different kinds of neutrosophic ideals. We also find some new type of neutrosophic ideal which is related to the strong or pure part of neutrosophy. We have given many examples to illustrate the theory of neutrosophic LA-semigroup and display many properties of neutrosophic LA-semigroup in this paper.

  17. Non-compact left ventricle/hypertrabeculated left ventricle

    International Nuclear Information System (INIS)

    Restrepo, Gustavo; Castano, Rafael; Marmol, Alejandro

    2005-01-01

    Non-compact left ventricle/hypertrabeculated left ventricle is a myocardiopatie produced by an arrest of the normal left ventricular compaction process during the early embryogenesis. It is associated to cardiac anomalies (congenital cardiopaties) as well as to extracardial conditions (neurological, facial, hematologic, cutaneous, skeletal and endocrinological anomalies). This entity is frequently unnoticed, being diagnosed only in centers with great experience in the diagnosis and treatment of myocardiopathies. Many cases of non-compact left ventricle have been initially misdiagnosed as hypertrophic myocardiopatie, endocardial fibroelastosis, dilated cardiomyopatie, restrictive cardiomyopathy and endocardial fibrosis. It is reported the case of a 74 years old man with a history of chronic arterial hypertension and diabetes mellitus, prechordial chest pain and mild dyspnoea. An echocardiogram showed signs of non-compact left ventricle with prominent trabeculations and deep inter-trabecular recesses involving left ventricular apical segment and extending to the lateral and inferior walls. Literature on this topic is reviewed

  18. [Left-handedness and health].

    Science.gov (United States)

    Milenković, Sanja; Belojević, Goran; Kocijancić, Radojka

    2010-01-01

    Hand dominance is defined as a proneness to use one hand rather than another in performing the majority of activities and this is the most obvious example of cerebral lateralization and an exclusive human characteristic. Left-handed people comprise 6-14% of the total population, while in Serbia, this percentage is 5-10%, moving from undeveloped to developed environments, where a socio-cultural pressure is less present. There is no agreement between investigators who in fact may be considered a left-handed person, about the percentage of left-handers in the population and about the etiology of left-handedness. In the scientific literature left-handedness has been related to health disorders (spine deformities, immunological disorders, migraine, neurosis, depressive psychosis, schizophrenia, insomnia, homosexuality, diabetes mellitus, arterial hypertension, sleep apnea, enuresis nocturna and Down Syndrome), developmental disorders (autism, dislexia and sttutering) and traumatism. The most reliable scientific evidences have been published about the relationship between left-handedness and spinal deformities in school children in puberty and with traumatism in general population. The controversy of other results in up-to-now investigations of health aspects of left-handedness may partly be explained by a scientific disagreement whether writing with the left hand is a sufficient criterium for left-handedness, or is it necessary to investigate other parameters for laterality assessment. Explanation of health aspects of left-handedness is dominantly based on Geschwind-Galaburda model about "anomalous" cerebral domination, as a consequence of hormonal disbalance.

  19. Nasomaxillary hypoplasia with a congenitally missing tooth treated with LeFort II osteotomy, autotransplantation, and nickel-titanium alloy wire.

    Science.gov (United States)

    Ishida, Takayoshi; Ikemoto, Shigehiro; Ono, Takashi

    2015-09-01

    In some skeletal Class III adult patients with nasomaxillary hypoplasia, the LeFort I osteotomy provides insufficient correction. This case report describes a 20-year-old woman with a combination of nasomaxillary hypoplasia and a protrusive mandible with a congenitally missing mandibular second premolar. We performed a LeFort II osteotomy for maxillary advancement. Autotransplantation of a tooth was also performed; the donor tooth was used to replace the missing permanent tooth. To increase the chance of success, we applied light continuous force with an improved superelastic nickel-titanium alloy wire technique before extraction and after transplantation. The patient's profile and malocclusion were corrected, and the autotransplanted tooth functioned well. The postero-occlusal relationships were improved, and ideal overbite and overjet relationships were achieved. The methods used in this case represent a remarkable treatment. Copyright © 2015 American Association of Orthodontists. Published by Elsevier Inc. All rights reserved.

  20. Chronic Bilateral Slipped Capital Femoral Epiphysis as an Unusual Presentation of Congenital Panhypopituitarism due to Pituitary Hypoplasia in a 17-Year-Old Female

    Directory of Open Access Journals (Sweden)

    Sasigarn A. Bowden

    2009-01-01

    Full Text Available We report an interesting case of a 17-year-old normal-statured female who was diagnosed with congenital panhypopituitarism due to pituitary hypoplasia at the presentation of bilateral slipped capital femoral epiphysis. We emphasized the importance of endocrinologic evaluation in patients with atypical slipped capital femoral epiphysis to prevent potential complication of adrenal crisis during surgery. This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital hypopituitarism in this patient.

  1. Chronic Bilateral Slipped Capital Femoral Epiphysis as an Unusual Presentation of Congenital Panhypopituitarism due to Pituitary Hypoplasia in a 17-Year-Old Female

    Directory of Open Access Journals (Sweden)

    Klingele KevinE

    2009-11-01

    Full Text Available We report an interesting case of a 17-year-old normal-statured female who was diagnosed with congenital panhypopituitarism due to pituitary hypoplasia at the presentation of bilateral slipped capital femoral epiphysis. We emphasized the importance of endocrinologic evaluation in patients with atypical slipped capital femoral epiphysis to prevent potential complication of adrenal crisis during surgery. This case also demonstrates growth without growth hormone which resulted in a delay in diagnosis of congenital hypopituitarism in this patient.

  2. Improved imaging of cochlear nerve hypoplasia using a 3-Tesla variable flip-angle turbo spin-echo sequence and a 7-cm surface coil.

    Science.gov (United States)

    Giesemann, Anja M; Raab, Peter; Lyutenski, Stefan; Dettmer, Sabine; Bültmann, Eva; Frömke, Cornelia; Lenarz, Thomas; Lanfermann, Heinrich; Goetz, Friedrich

    2014-03-01

    Magnetic resonance imaging of the temporal bone has an important role in decision making with regard to cochlea implantation, especially in children with cochlear nerve deficiency. The purpose of this study was to evaluate the usefulness of the combination of an advanced high-resolution T2-weighted sequence with a surface coil in a 3-Tesla magnetic resonance imaging scanner in cases of suspected cochlear nerve aplasia. Prospective study. Seven patients with cochlear nerve hypoplasia or aplasia were prospectively examined using a high-resolution three-dimensional variable flip-angle turbo spin-echo sequence using a surface coil, and the images were compared with the same sequence in standard resolution using a standard head coil. Three neuroradiologists evaluated the magnetic resonance images independently, rating the visibility of the nerves in diagnosing hypoplasia or aplasia. Eight ears in seven patients with hypoplasia or aplasia of the cochlear nerve were examined. The average age was 2.7 years (range, 9 months-5 years). Seven ears had accompanying malformations. The inter-rater reliability in diagnosing hypoplasia or aplasia was greater using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence (fixed-marginal kappa: 0.64) than with the same sequence in lower resolution (fixed-marginal kappa: 0.06). Examining cases of suspected cochlear nerve aplasia using the high-resolution three-dimensional variable flip-angle turbo spin-echo sequence in combination with a surface coil shows significant improvement over standard methods. © 2013 The American Laryngological, Rhinological and Otological Society, Inc.

  3. Hypoplasia of the basi-occipital bone and persistance of the spheno-occipital synchondrosis in a patient with transitory supplementary fissure of the basi-occipital

    International Nuclear Information System (INIS)

    Wackenheim, A.

    1985-01-01

    The author had the opportunity to observe the progressive development of a special form of basilar impression characterized by transitory supplementary fissure of the basi-occipital bone, persistance of the spheno-occipital synchondrosis and hypoplasia of the basi-occipital. He proposes to dissociate the general concept of basilar impression and to consider anatomo-clinical entities such as the example described in this paper. (orig.)

  4. Dental enamel Hypoplasia. Investigations on the Bones Exhumed from the Medieval Necropole of Lozova (Republic of Moldova, XIVth–XVth Centuries

    Directory of Open Access Journals (Sweden)

    Robert Daniel Simalcsik

    2014-10-01

    Full Text Available Dental hypoplasia is a developmental anomaly based on perturbations of amelogenesis. Hypoplasia defects are part of the unspecific quantitative indicators for the state of health and / or nutritional state during the formation of the dental buds. It is a response of the human organism to physiological stress. The incidence of this dysplasia in a past population can indicate its biological frailty in its attempt to adapt to the environmental changes. The osteological material was excavated in the interval 2010 – 2011 by archaeologists from the Archaeology Centre in Chisinau, from the Medieval cemetery of Lozova (Straseni County, Republic of Moldova, dated for the XIVth and XVth centuries. Fifty one skeletons from 50 inhumation graves have been excavated and analyzed so far. Only 40 individuals had most of their teeth present. The enamel hypoplasia is of linear transversal type, located on the labial surface of the dental crowns, in the median third. The canine is the most affected tooth, followed by the incisors. The incidence of dental enamel hypoplasia at population level (based on the data collected and on the number of graves excavates so far, which does not illustrate the entire population of the cemetery is 7.5%. The incidence of dental caries is 23.53%, of cribra orbitalia – 11.75%, and of cribra cranii externa – 1.96%. The results obtained for a relatively small rural community illustrate a good adaptation to the stressing environmental factors. The possible malnutrition and illness episodes suffered during early childhood were recovered along the growth and development processes.

  5. Radionuclide detection and differential diagnosis of left-to-right cardiac shunts by analysis of time-activity curves

    International Nuclear Information System (INIS)

    Kim, Ok-Hwa

    1986-01-01

    The noninvasive nature of the radionuclide angiocardiography provided a useful approach for the evaluation of left-to-right cardiac shunts (LRCS). While the qualitative information can be obtained by inspection of serial radionuclide angiocardiograms, the quantitative information of radionuclide angiocardiography can be obtained by the analysis of time-activity curves using advanced computer system. The count ratios method and pulmonary-to-systemic flow ratio (QP/QS) by gamma variate fit method were used to evaluate the accuracy of detection and localization of LRCS. One hundred and ten time-activity curves were analyzed. There were 46 LRCS (atrial septal defects 11, ventricular septal defects 22, patent ductus arteriosus 13) and 64 normal subjects. By computer analysis of time-activity histograms of the right atrium, ventricle and the lungs separately, the count ratios modified by adding the mean cardiac transit time were calculated in each anatomic site. In normal subjects the mean count ratios in the right atrium, ventricle and lungs were 0.24 on average. In atrial septal defects, the count ratios were high in the right atrium, ventricle and lungs, whereas in ventricular septal defects the count ratios were higher only in the right ventricle and lungs. Patent ductus arteriosus showed normal count ratios in the heart but high count ratios were obtained in the lungs. Thus, this count ratios method could be separated normal from those with intracardiac or extracardiac shunts, and moreover, with this method the localization of the shunts level was possible in LRCS. Another method that could differentiate the intracardiac shunts from extracardiac shunts was measuring QP/QS in the left and right lungs. In patent ductus arteriosus, the left lung QP/QS was hight than those of the right lung, whereas in atrial septal defects and ventricular septal defects QP/QS ratios were equal in both lungs. (J.P.N.)

  6. Skeletal manifestations of stress in child victims of the Great Irish Famine (1845-1852): prevalence of enamel hypoplasia, Harris lines, and growth retardation.

    Science.gov (United States)

    Geber, Jonny

    2014-09-01

    The Great Irish Famine of 1845-1852 is among the worst food crises in human history. While numerous aspects of this period have been studied by generations of scholars, relatively little attention has so far been given to the physiological impact it is likely to have had on the people who suffered and succumbed to it. This study examines the prevalence of enamel hypoplasia, Harris lines, and growth retardation in the nonadult proportion of a skeletal population comprising victims of the Famine who died in the workhouse in the city of Kilkenny between 1847 and 1851. The frequency of enamel hypoplasia in these children does not appear to have increased as a consequence of famine, although this fact is likely to be a reflection of the osteological paradox. Harris lines and growth retardation; however, were very prevalent, and the manifestation and age-specific distribution of these may be indicators of the Famine experience. While there was no clear correlation in the occurrence of the assessed markers, the presence of cribra orbitalia displayed a significant relationship to enamel hypoplasia in 1- to 5-year-old children. While starvation, metabolic disorders and infectious diseases are likely to have greatly contributed to the manifestation of the markers, the psychosocial stress relating to institutionalization in the workhouse should not be underestimated as a substantial causative factor for skeletal stress in this population. Copyright © 2014 Wiley Periodicals, Inc.

  7. [Exploration for micro-osteotomy assisted orthodontic treatment of skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region].

    Science.gov (United States)

    Wang, Bo; Shen, Guo-fang; Fang, Bing; Sun, Liang-yan; Wu, Yong; Jiang, Ling-yong; Zhu, Min

    2012-10-01

    To investigate the changes of periodontal conditions after micro-osteotomy assisted lower incisor decompensation for skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region. The sample consisted of 22 cases diagnosed as skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region, selected from consecutive patients of Department of Oral & Cranio-maxillofacial Science of Shanghai Ninth People's Hospital during 2009-2012. The samples were divided into 2 groups; G1 comprised 10 patients who accepted micro-osteotomy assisted lower incisor decompensation; G2 comprised 12 patients who chose traditional pre-surgical decomposition. The changes of periodontal conditions of both groups were evaluated with the help of cone-beam CT(CBCT). Data was processed using SAS8.02 software package. For subjects in G1, during the micro-osteotomy assisted pre-surgical orthodontics, no significant difference was found in the amount of root resorption of lower incisors.But labial and lingual vertical alveolar bone loss were 2.60 mm and 2.22 mm; alveolar bone thickness increased by 3.05 mm on the labial side and decreased by 0.88 mm on the lingual side (Ppre-surgical orthodontics was much safer than traditional orthodontics for skeletal Class III malocclusions with alveolar hypoplasia in the lower anterior region.

  8. Maxillary distraction osteogenesis in cleft lip and palate cases with midface hypoplasia using rigid external distractor: an alternative technique.

    Science.gov (United States)

    Dua, Gaurav; Navin Kumar, Andrews; Roy, Indranil Deb; Roy, Supriyo Kumar

    2014-05-01

    Patients with operated cleft lip and palate present with a problem of midface hypoplasia, and such patients have been traditionally treated with orthognathic surgery. Such a procedure has its own limitations of relapse and hence a newer modality of distraction osteogenesis with histiogenesis can be chosen to overcome such limitations for midfacial advancement. The purpose of this study is to evaluate an alternative technique and its postoperative stability in maxillary distraction osteogenesis in patients of cleft lip and cleft palate using a rigid external device (RED). Nine patients with midface bone stock deficiency were selected for maxillary advancement. At the first surgery under general anesthesia, after Le Fort I osteotomy, RED system was used with the alternative technique. After distraction, evaluation was done for ease of the procedure, stability, and complications. Lateral cephalograms were evaluated at 3 stages: T1, pre-distraction; T2, post-distraction; and T3, 1 year post-distraction. A mean 13.4-mm midface advancement was shown with bone formation at the pterygomaxillary region without losing the vector and having a standby mode in case the wire broke during distraction The results were stable even at 1 year of follow-up. Maxillary position improved in relation to the cranial base. This study showed that the RED was versatile in midface advancement.

  9. Progressive behavioral changes during the maturation of rats with early radiation-induced hypoplasia of fascia dentata granule cells

    International Nuclear Information System (INIS)

    Mickley, G.A.; Ferguson, J.L.; Mulvihill, M.A.; Nemeth, T.J.

    1989-01-01

    Localized exposure of the neonatal rat brain to X-rays produces neuronal hypoplasia specific to the granule cell layer of the hippocampal dentate gyrus. This brain damage causes locomotor hyperactivity, slowed acquisition of passive avoidance tasks and long bouts of spontaneous turning (without reversals) in a bowl apparatus. Here we report how these behavioral deficits change as a function of subject aging and behavioral test replications. Portions of the neonatal rat cerebral hemispheres were X-irradiated in order to selectively damage the granule cells of the dentate gyrus. The brains of experimental animals received a fractionated dose of X rays (13 Gy total) over postnatal days 1 to 16 and control animals were sham-irradiated. Rats between the ages of 71-462 days were tested 3 separate times on each of the following 3 behavioral tests: (1) spontaneous locomotion, (2) passive avoidance acquisition, and (3) spontaneous circling in a large plastic hemisphere. Rats with radiation-induced damage to the fascia dentata exhibited long bouts of slow turns without reversals. Once they began, irradiated subjects perseverated in turning to an extent significantly greater than sham-irradiated control subjects. This irradiation effect was significant during all test series. Moreover, in time, spontaneous perseverative turning was significantly potentiated in rats with hippocampal damage but increased only slightly in controls. Early radiation exposure produced locomotor hyperactivity in young rats. While activity levels of controls remained fairly stable throughout the course of the experiment, the hyperactivity of the irradiated animals decreased significantly as they matured

  10. Lung hypoplasia and its associated major congenital abnormalities in perinatal death: an autopsy study of 850 cases.

    Science.gov (United States)

    Aghabiklooei, A; Goodarzi, P; Kariminejad, Mohammad H

    2009-11-01

    To determine the relative frequency of causes of lung hypoplasia (LH) and its associated congenital malformations among perinatal deaths. 850 medical reports of perinatal autopsies, in a 25-year period, assessed for LH as a cause of death. LH found in 96 (11.3%) cases, 89 (92.7%) were associated with major congenital malformation (secondary type) and primary type was seen in 7 cases (7.3%). Fourteen cases were associated with multiple congenital anomalies. 32 cases (33.3%) with Genito-urinary anomalies were the most common associated major malformations, followed by 19 cases (19.8%) of diaphragmatic impairment, 15 cases (15.6%) of musculoskeletal abnormalities and 11 cases (11.4%) of kidney agenesis. The most common musculoskletal abnormality was thanatophoric dwarfism in 10 cases (10.4%). Meckle-Gruber syndrome with 7 affected fetuses (7.3%) was the most common malformation syndrome associated with LH. More than ninety percent of LH was secondary to pathology outside the respiratory tract. Renal agenesis is the most common association observed in LH, followed by diaphragmatic hernia and thanatophoric dysplasia.

  11. Optical coherence tomography detection of characteristic retinal nerve fiber layer thinning in nasal hypoplasia of the optic disc.

    Science.gov (United States)

    Haruta, M; Kodama, R; Yamakawa, R

    2017-12-01

    PurposeTo determine the clinical usefulness of optical coherence tomography (OCT) for detecting thinning of the retinal nerve fiber layer (RNFL) in eyes with nasal hypoplasia of the optic discs (NHOD).Patients and methodsThe medical records of five patients (eight eyes) with NHOD were reviewed. The ratio of the disc-macula distance to the disc diameter (DM/DD) and the disc ovality ratio of the minimal to maximal DD were assessed using fundus photographs. The RNFL thicknesses of the temporal, superior, nasal, and inferior quadrants were evaluated using OCT quadrant maps.ResultsAll eight eyes had temporal visual field defects that respected the vertical meridians that needed to be differentiated from those related to chiasmal compression. The mean DM/DD ratio was 3.1 and the mean disc ovality ratio was 0.81. The mean RNFL thicknesses of the temporal, superior, nasal, and inferior quadrants were 90.3, 103.1, 34.8, and 112.8 microns, respectively.ConclusionSmall optic discs and tilted discs might be associated with NHOD. Measurement of the RNFL thickness around the optic disc using OCT scans clearly visualized the characteristic RNFL thinning of the nasal quadrants corresponding to the temporal sector visual field defects in eyes with NHOD. OCT confirmed the presence of NHOD and might differentiate eyes with NHOD from those with chiasmal compression.

  12. Left-handedness and health

    Directory of Open Access Journals (Sweden)

    Milenković Sanja

    2010-01-01

    Full Text Available Hand dominance is defined as a proneness to use one hand rather than another in performing the majority of activities and this is the most obvious example of cerebral lateralization and an exclusive human characteristic. Left-handed people comprise 6-14% of the total population, while in Serbia, this percentage is 5-10%, moving from undeveloped to developed environments, where a socio-cultural pressure is less present. There is no agreement between investigators who in fact may be considered a left-handed person, about the percentage of left-handers in the population and about the etiology of left-handedness. In the scientific literature left-handedness has been related to health disorders (spine deformities, immunological disorders, migraine, neurosis, depressive psychosis, schizophrenia, insomnia, homosexuality, diabetes mellitus, arterial hypertension, sleep apnea, enuresis nocturna and Down Syndrome, developmental disorders (autism, dislexia and sttutering and traumatism. The most reliable scientific evidences have been published about the relationship between left-handedness and spinal deformities in school children in puberty and with traumatism in general population. The controversy of other results in up-to-now investigations of health aspects of left-handedness may partly be explained by a scientific disagreement whether writing with the left hand is a sufficient criterium for left-handedness, or is it necessary to investigate other parameters for laterality assessment. Explanation of health aspects of left-handedness is dominantly based on Geschwind-Galaburda model about 'anomalous' cerebral domination, as a consequence of hormonal disbalance. .

  13. Low estriol levels in the maternal marker screen as a predictor of X-linked adrenal hypoplasia congenita: Case report

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    Durković Jasmina

    2014-01-01

    Full Text Available Introduction. X-linked adrenal hypoplasia congenita (AHC is a rare cause of adrenocortical insufficiency. Early postnatal diagnosis may prevent severe hypoglycemia, Addisonian crises and death. Low maternal estriol (E3 levels in the second trimester of pregnancy could indicate the possibility that the fetus suffers from a disorder that causes adrenal insufficiency. Suspicion is based on the fact that E3 originates from dehydroepiandrosterone (DHEA synthesized in the fetal adrenals. In case of adrenal insufficiency, the impaired production of fetal DHEA leads to a subsequent reduction of E3 concentrations in maternal serum. There are only a few reports of AHC suspected prenatally due to low maternal E3 levels. Case Outline. We describe two brothers with adrenal insufficiency due to AHC. The older brother was admitted to the hospital at the age of 33 days due to failure to thrive, vomiting, and dehydration. Genetic analysis revealed a hemizygous mutation in DAX-1 gene, thus confirming the diagnosis of ACH. The same mutation was detected in his mother. In the second pregnancy, E3 concentrations were determined from maternal serum. Estriol levels during the second trimester were extremely low suggesting the diagnosis of AHC. The diagnosis was confirmed during the neonatal period by genetic testing, and replacement therapy was started at the age of 10 days. This boy never experienced an adverse episode such as hypoglycemia or adrenal crises. Conclusion. Since determination of E3 is a simple, sensitive, noninvasive and cheap method, its use as an obligatory prenatal screening test should be accepted as a standard practice in Serbia.

  14. A deletion in the VLDLR gene in Eurasier dogs with cerebellar hypoplasia resembling a Dandy-Walker-like malformation (DWLM.

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    Martina Gerber

    Full Text Available Dandy-Walker-like malformation (DWLM is the result of aberrant brain development and mainly characterized by cerebellar hypoplasia. DWLM affected dogs display a non-progressive cerebellar ataxia. Several DWLM cases were recently observed in the Eurasier dog breed, which strongly suggested a monogenic autosomal recessive inheritance in this breed. We performed a genome-wide association study (GWAS with 9 cases and 11 controls and found the best association of DWLM with markers on chromosome 1. Subsequent homozygosity mapping confirmed that all 9 cases were homozygous for a shared haplotype in this region, which delineated a critical interval of 3.35 Mb. We sequenced the genome of an affected Eurasier and compared it with the Boxer reference genome and 47 control genomes of dogs from other breeds. This analysis revealed 4 private non-synonymous variants in the critical interval of the affected Eurasier. We genotyped these variants in additional dogs and found perfect association for only one of these variants, a single base deletion in the VLDLR gene encoding the very low density lipoprotein receptor. This variant, VLDLR:c.1713delC is predicted to cause a frameshift and premature stop codon (p.W572Gfs*10. Variants in the VLDLR gene have been shown to cause congenital cerebellar ataxia and mental retardation in human patients and Vldlr knockout mice also display an ataxia phenotype. Our combined genetic data together with the functional knowledge on the VLDLR gene from other species thus strongly suggest that VLDLR:c.1713delC is indeed causing DWLM in Eurasier dogs.

  15. Prenatal ethanol exposure in mice phenocopies Cdon mutation by impeding Shh function in the etiology of optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Benjamin M. Kahn

    2017-01-01

    Full Text Available Septo-optic dysplasia (SOD is a congenital disorder characterized by optic nerve, pituitary and midline brain malformations. The clinical presentation of SOD is highly variable with a poorly understood etiology. The majority of SOD cases are sporadic, but in rare instances inherited mutations have been identified in a small number of transcription factors, some of which regulate the expression of Sonic hedgehog (Shh during mouse forebrain development. SOD is also associated with young maternal age, suggesting that environmental factors, including alcohol consumption at early stages of pregnancy, might increase the risk of developing this condition. Here, we address the hypothesis that SOD is a multifactorial disorder stemming from interactions between mutations in Shh pathway genes and prenatal ethanol exposure. Mouse embryos with mutations in the Shh co-receptor, Cdon, were treated in utero with ethanol or saline at embryonic day 8 (E8.0 and evaluated for optic nerve hypoplasia (ONH, a prominent feature of SOD. We show that both Cdon−/− mutation and prenatal ethanol exposure independently cause ONH through a similar pathogenic mechanism that involves selective inhibition of Shh signaling in retinal progenitor cells, resulting in their premature cell-cycle arrest, precocious differentiation and failure to properly extend axons to the optic nerve. The ONH phenotype was not exacerbated in Cdon−/− embryos treated with ethanol, suggesting that an intact Shh signaling pathway is required for ethanol to exert its teratogenic effects. These results support a model whereby mutations in Cdon and prenatal ethanol exposure increase SOD risk through spatiotemporal perturbations in Shh signaling activity.

  16. Optical Coherence Tomography in Optic Nerve Hypoplasia: Correlation With Optic Disc Diameter, Nerve Fiber Layer Thickness, and Visual Function.

    Science.gov (United States)

    Kelly, John P; Baran, Francine; Phillips, James O; Weiss, Avery H

    2017-12-15

    The correlation between optic disc diameters (DDs) with average retinal nerve fiber layer thickness (RNFLT) and visual function in children with optic nerve hypoplasia (ONH) having nystagmus is unknown. Data were obtained from a retrospective review of 28 children (mean age: 9.4 years; ±5.1). Optic DD was defined as the maximal horizontal opening of Bruch membrane with spectral optical coherence tomography combined with a confocal laser ophthalmoscope. Average RNFLT was obtained from circumpapillary b-scans. RNFLT was also remeasured at eccentricities that were proportionate with DD to rule out potential sampling artifacts. Visual function was assessed by visual acuity at last follow-up and by visual evoked potentials (VEP) in 11 patients. The eye with the larger DD, which had better visual acuity, was analyzed to exclude potential effects of amblyopia. DD was correlated with average RNFLT (r = 0.61), visual acuity (r = 0.32), and VEPs (r = 0.66). The relationship between RNFLT and DD was as follows: average RNFLT (μm) = 0.074 * DD (μm) - 18.8. RNFLT also correlated with the ratio of horizontal optic DD to macula-disc-margin distance (DD:DM; r = 0.59). RNFLT measured at eccentricities proportionate with DD showed progressive decrease in thickness only for DDs <1,100 μm. All patients with DD <1,000 μm had subnormal visual acuity, whereas those with DD <1,200 μm had subnormal VEPs. DD correlates with average RNFLT and with visual function in children with ONH. Using OCT imaging, DD can be obtained in children with nystagmus and provides objective information.

  17. Radionuclide Angiocardiographic Evaluation of Left-to-Right Cardiac Shunts: Analysis of Time-Active Curves

    International Nuclear Information System (INIS)

    Kim, Ok Hwa; Bahk, Yong Whee; Kim, Chi Kyung

    1987-01-01

    The noninvasive nature of the radionuclide angiocardiography provided a useful approach for the evaluation of left-to-right cardiac shunts (LRCS). While the qualitative information can be obtained by inspection of serial radionuclide angiocardiograms, the quantitative information of radionuclide angiocardiography can be obtained by the analysis of time-activity curves using advanced computer system. The count ratios method and pulmonary-to-systemic flow ratio (QP/QS) by gamma variate fit method were used to evaluate the accuracy of detection and localization of LRCS. One hundred and ten time-activity curves were analyzed. There were 46 LRCS (atrial septal defects 11, ventricular septal defects 22, patent ductus arteriosus 13) and 64 normal subjects. By computer analysis of time-activity curves of the right atriurn, ventricle and the lungs separately, the count ratios modified by adding the mean cardiac transit time were calculated in each anatomic site. In normal subjects the mean count ratios in the right atrium, ventricle and lungs were 0.24 on average. In atrial septal defects, the count ratios were high in the right atrium, ventricle and lungs, whereas in ventricular septal defects the count ratios were higher only in the right ventricle and lungs. Patent ductus arteriosus showed normal count ratios in the heart but high count ratios were obtained in the lungs. Thus, this count ratios method could be separated normal from those with intracardiac or extracardiac shunts, and moreover, with this method the localization of the shunt level was possible in LRCS. Another method that could differentiate the intracardiac shunts from extracardiac shunts was measuring QP/QS in the left and right lungs. In patent ductus arteriosus, the left lung QP/QS was higher than those of the right lung, whereas in atrial septal defects and ventricular septal defects QP/ QS ratios were equal in both lungs. From this study, it was found that by measuring QP/QS separately in the lungs

  18. Validity of PRV margins around lung and heart during left breast irradiation

    International Nuclear Information System (INIS)

    Stefanovski, Zoran

    2010-01-01

    Planning organ at risk volumes (PRV) has a minor use in radiotherapy treatment planning. During left breast irradiation two critical volumes are of special importance the lung and the heart. The aim of this study was to evaluate the changes in volume doses after adding appropriate margins around these organs at risk and compare them with the effect that the systematic positioning error has on the volume doses. Methods: Treatment plans for 44 patients with left breast cancer were analyzed. Two changes for each plan were made, and dose-volume histogram values for hearts and lungs volumes were recorded. In the first case margins of 5 mm to hearts and lungs were added. Volumes that were enclosed by 30% isodose for hearts and volumes that were enclosed by 20% isodose of lungs were recorded. In the second case plans were made with a systematic error of 5 mm employed, depicting a translation of isocenter posterior and to the right. In this second case, monitor units were taken from the original plan. The critical volumes for hearts and lungs were recorded as in the first case. Results: Our policy for breast cancer irradiation demands that the lung volume receiving 20 Gy should be kept under 25% of the whole left-lung volume, and no more than 10% of the heart volume should receive more than 30 Gy. The first case simulation showed that 23% of the patients have a heart overdose while 11% of them have a lung overdose according to the criteria above. Simulation of the second kind showed that the systematic error in isocenter positioning of 5 mm gives bigger a volume of the heart (in average 0.69% of heart volume) to be enclosed in critical isodose than in PRV case. For the lung the situation was opposite; namely in PRV case the lung volume that is encompassed with critical isodose is greater (in average 1.47% of lung volume) than in a case of displaced isocenter. Conclusions: Adding PRV margins around the heart and the lung does not give straightforward and unambiguous result

  19. Left bundle-branch block

    DEFF Research Database (Denmark)

    Risum, Niels; Strauss, David; Sogaard, Peter

    2013-01-01

    The relationship between myocardial electrical activation by electrocardiogram (ECG) and mechanical contraction by echocardiography in left bundle-branch block (LBBB) has never been clearly demonstrated. New strict criteria for LBBB based on a fundamental understanding of physiology have recently...

  20. Producing The New Regressive Left

    DEFF Research Database (Denmark)

    Crone, Christine

    members, this thesis investigates a growing political trend and ideological discourse in the Arab world that I have called The New Regressive Left. On the premise that a media outlet can function as a forum for ideology production, the thesis argues that an analysis of this material can help to trace...... the contexture of The New Regressive Left. If the first part of the thesis lays out the theoretical approach and draws the contextual framework, through an exploration of the surrounding Arab media-and ideoscapes, the second part is an analytical investigation of the discourse that permeates the programmes aired...... becomes clear from the analytical chapters is the emergence of the new cross-ideological alliance of The New Regressive Left. This emerging coalition between Shia Muslims, religious minorities, parts of the Arab Left, secular cultural producers, and the remnants of the political,strategic resistance...

  1. Left main percutaneous coronary intervention.

    Science.gov (United States)

    Teirstein, Paul S; Price, Matthew J

    2012-10-23

    The introduction of drug-eluting stents and advances in catheter techniques have led to increasing acceptance of percutaneous coronary intervention (PCI) as a viable alternative to coronary artery bypass graft (CABG) for unprotected left main disease. Current guidelines state that it is reasonable to consider unprotected left main PCI in patients with low to intermediate anatomic complexity who are at increased surgical risk. Data from randomized trials involving patients who are candidates for either treatment strategy provide novel insight into the relative safety and efficacy of PCI for this lesion subset. Herein, we review the current data comparing PCI with CABG for left main disease, summarize recent guideline recommendations, and provide an update on technical considerations that may optimize clinical outcomes in left main PCI. Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  2. Left ventricular apical ballooning syndrome

    International Nuclear Information System (INIS)

    Rahman, N.; Tai, J.; Soofi, A.

    2007-01-01

    The transient left ventricular apical ballooning syndrome, also known as Takotsubo cardiomyopathy, is characterized by transient left ventricular dysfunction in the absence of obstructive epicardial coronary disease. Although the syndrome has been reported in Japan since 1990, it is rare in other regions. Rapid recognition of the syndrome can modify the diagnostic and therapeutic attitude i.e. avoiding thrombolysis and performing catheterization in the acute phase. (author)

  3. Apraxia in left-handers.

    Science.gov (United States)

    Goldenberg, Georg

    2013-08-01

    In typical right-handed patients both apraxia and aphasia are caused by damage to the left hemisphere, which also controls the dominant right hand. In left-handed subjects the lateralities of language and of control of the dominant hand can dissociate. This permits disentangling the association of apraxia with aphasia from that with handedness. Pantomime of tool use, actual tool use and imitation of meaningless hand and finger postures were examined in 50 consecutive left-handed subjects with unilateral hemisphere lesions. There were three aphasic patients with pervasive apraxia caused by left-sided lesions. As the dominant hand is controlled by the right hemisphere, they constitute dissociations of apraxia from handedness. Conversely there were also three patients with pervasive apraxia caused by right brain lesions without aphasia. They constitute dissociations of apraxia from aphasia. Across the whole group of patients dissociations from handedness and from aphasia were observed for all manifestations of apraxia, but their frequency depended on the type of apraxia. Defective pantomime and defective tool use occurred rarely without aphasia, whereas defective imitation of hand, but not finger, postures was more frequent after right than left brain damage. The higher incidence of defective imitation of hand postures in right brain damage was mainly due to patients who had also hemi-neglect. This interaction alerts to the possibility that the association of right hemisphere damage with apraxia has to do with spatial aptitudes of the right hemisphere rather than with its control of the dominant left hand. Comparison with data from right-handed patients showed no differences between the severity of apraxia for imitation of hand or finger postures, but impairment on pantomime of tool use was milder in apraxic left-handers than in apraxic right-handers. This alleviation of the severity of apraxia corresponded with a similar alleviation of the severity of aphasia as

  4. Right colon cancer: Left behind.

    Science.gov (United States)

    Gervaz, P; Usel, M; Rapiti, E; Chappuis, P; Neyroud-Kaspar, I; Bouchardy, C

    2016-09-01

    Prognosis of colon cancer (CC) has steadily improved during the past three decades. This trend, however, may vary according to proximal (right) or distal (left) tumor location. We studied if improvement in survival was greater for left than for right CC. We included all CC recorded at the Geneva population-based registry between 1980 and 2006. We compared patients, tumor and treatment characteristics between left and right CC by logistic regression and compared CC specific survival by Cox models taking into account putative confounders. We also compared changes in survival between CC location in early and late years of observation. Among the 3396 CC patients, 1334 (39%) had right-sided and 2062 (61%) left-sided tumors. In the early 1980s, 5-year specific survival was identical for right and left CCs (49% vs. 48%). During the study period, a dramatic improvement in survival was observed for patients with left-sided cancers (Hazard ratio [HR]: 0.42, 95% confidence interval [CI]: 0.29-0.62, p colon cancer patients, those with right-sided lesions have by far the worse prognosis. Change of strategic management in this subgroup is warranted. Copyright © 2016 Elsevier Ltd. All rights reserved.

  5. Aphasia following left thalamic hemorrhage

    International Nuclear Information System (INIS)

    Makishita, Hideo; Miyasaka, Motomaro; Tanizaki, Yoshio; Yanagisawa, Nobuo; Sugishita, Morihiro.

    1984-01-01

    We reported 7 patients with left thalamic hemorrhage in the chronic stage (from 1.5 months to 4.5 months), and described language disorders examined by Western Aphasia Battery (WAB) and measured cerebral blood flow by single photon emission CT. Examination of language by WAB revealed 4 aphasics out of 7 cases, and 3 patients had no language deficit. The patient with Wernicke's aphasia showed low density area only in the left posterior thalamus in X-ray CT, and revealed severe low blood flow area extending to left temporal lobe in emission CT. In the case with transcortical sensory aphasia, although X-ray CT showed no obvious low density area, emission CT revealed moderate low flow area in watershed area that involved the territory between posterior cerebral and middle cerebral arteries in the left temporooccipital region in addition to low blood flow at the left thalamus. In one of the two patients classified as anomic aphasia, whose score of repetition (8.4) was higher than that of comprehension (7.4), emission CT showed slight low flow area at the temporo-occipital region similarly as the case with transcortical sensory aphasia. In another case with anomic aphasia, scored 9 on both fluensy and comprehension subtests and 10 on repetition, there was wide low density area all over the left thalamus and midline shift to the right in X-ray CT, and emission CT showed severe low blood flow in the same region spreading widely toward the cerebral surface. On the other hand, in all of the 3 patients without aphasia, emission CT showed low flow region restricted to the left thalamus. (J.P.N.)

  6. Patient-Reported Esthetic and Functional Outcomes of Primary Total Laparoscopic Intestinal Vaginoplasty in Transgender Women With Penoscrotal Hypoplasia.

    Science.gov (United States)

    Bouman, Mark-Bram; van der Sluis, Wouter B; van Woudenberg Hamstra, Leonora E; Buncamper, Marlon E; Kreukels, Baudewijntje P C; Meijerink, Wilhelmus J H J; Mullender, Margriet G

    2016-09-01

    Puberty-suppressing hormonal treatment may result in penoscrotal hypoplasia in transgender women, making standard penile inversion vaginoplasty not feasible. For these patients, intestinal vaginoplasty is a surgical alternative, but knowledge on patient-reported postoperative outcomes and quality of life is lacking. To assess patient-reported functional and esthetic outcomes, quality of life, satisfaction, and sexual well-being after primary total laparoscopic intestinal vaginoplasty in transgender women. A survey study was performed on transgender women who underwent primary total laparoscopic intestinal vaginoplasty with at least 1 year of clinical follow-up. Thirty-one transgender women completed the questionnaires (median age at time of surgery = 19.1 years, range = 18.3-45.0) after a median clinical follow-up of 2.2 years (range = 0.8-7.5). Consenting women were asked to complete a combined questionnaire of the Subjective Happiness Scale, the Satisfaction With Life Scale, Cantril's Ladder of Life Scale, the Female Sexual Function Index, the Female Genital Self-Imaging Scale, the Amsterdam Hyperactive Pelvic Floor Scale-Women, and a questionnaire addressing postoperative satisfaction. Patient-reported functional and esthetic outcomes and postoperative quality of life. Patients graded their life satisfaction a median of 8.0 (range = 4.0-10.0) on Cantril's Ladder of Life Scale. Patients scored a mean total score of 27.7 ± 5.8 on the Satisfaction With Life Scale, which indicated high satisfaction with life, and a mean total score of 5.6 ± 1.4 on the Subjective Happiness Scale. Functionality was graded a median score of 8.0 of 10 (range = 1.0-10.0) and esthetics a score of 8.0 out of 10 (range = 3.0-10.0). The mean Female Sexual Function Index total score of sexually active transgender women was 26.0 ± 6.8. This group of relatively young transgender women reported satisfactory functional and esthetic results of the neovagina and a good quality of life

  7. Comparison of Heidelberg Retina Tomograph with disc-macula distance to disc diameter ratio in diagnosing optic nerve hypoplasia.

    Science.gov (United States)

    Pang, Yi; Frantz, Kelly A

    2016-05-01

    To evaluate whether Heidelberg Retinal Tomograph (HRT) is a valid test for diagnosing congenital optic nerve hypoplasia (CONH) compared to the ratio of the distance between the centre of the optic disc and the centre of the macula and the mean optic disc diameter (DM:DD ratio). Furthermore, to determine the optimal cut-off value of HRT disc area to differentiate a hypoplastic disc from a normal optic disc. A total of 33 subjects with CONH (4-67 years old) and 160 normal subjects (5-65 years old) were recruited and underwent comprehensive eye examinations, fundus photography and HRT. Receiver operating characteristic curves for DM:DD ratio and HRT disc area were constructed based on data from the 46 CONH eyes and 160 control eyes. Mean (±S.D.) HRT disc area was 1.94 (±0.54) mm(2) for the control eyes and 0.84 (±0.35) mm(2) for the CONH eyes (p < 0.0001). The area under the curve (AUC) for DM:DD ratio was 0.83 (95% confidence interval: 0.76-0.90). The AUC for HRT disc area was 0.96 (95% confidence interval: 0.94-0.99). A statistically significant difference was found between AUC for HRT disc area and that for DM:DD ratio (p = 0.0004). The optimal cut-off value for HRT disc area was 1.42 mm(2) with 95% sensitivity and 85% specificity. The optimal cut-off value for DM:DD ratio was 3.20 with 78% sensitivity and 78% specificity. Both HRT and the DM:DD ratio are valid tests to aid diagnosis of CONH. HRT is superior to DM:DD ratio in diagnosing CONH with higher sensitivity and specificity. We suggest the optimal cut-off value for HRT disc area as 1.42 mm(2) in order to discriminate a hypoplastic disc from a normal optic disc. © 2016 The Authors Ophthalmic & Physiological Optics © 2016 The College of Optometrists.

  8. Pulmonary hypoplasia on preterm infant associated with diffuse chorioamniotic hemosiderosis caused by intrauterine hemorrhage due to massive subchorial hematoma: report of a neonatal autopsy case.

    Science.gov (United States)

    Yamada, Sohsuke; Marutani, Takamitsu; Hisaoka, Masanori; Tasaki, Takashi; Nabeshima, Atsunori; Shiraishi, Mika; Sasaguri, Yasuyuki

    2012-08-01

    A male infant born prematurely at 31 weeks of gestation weighed 789 g and had mildly brown-colored oral/tracheal aspirates at delivery. The amniotic fluid was also discolored, and its index was below 5. The patient died of hypoxemic respiratory and cardiac failure 2 hours after birth. The maternal profiles showed placenta previa and intrauterine growth restriction (IUGR) at 22 weeks of gestation, and revealed recurrent episodes of antenatal and substantial vaginal bleeding and oligohydramnios, indicating chronic abruption-oligohydramnios sequence. The thickened placenta, weighing 275 g, grossly displayed unevenness and diffuse opacity with green to brown discoloration in the chorioamniotic surface, and revealed chronic massive subchorial hematomas (Breus' mole) with old peripheral blood clot, circumvallation, and infarction. Microscopically, diffuse Berlin-blue staining-positive hemosiderin deposits were readily encountered in the chorioamniotic layers of the chorionic plate, consistent with diffuse chorioamniotic hemosiderosis (DCH) due to Breus' mole, accompanied by diffuse amniotic necrosis. At autopsy, an external examination showed several surface anomalies and marked pulmonary hypoplasia, 0.006 (less 0.012) of lung:body weight ratio. Since Breus' mole has a close relationship with intrauterine hemorrhage, resulting in DCH, IUGR, and/or pulmonary hypoplasia of the newborn, the present features might be typical. © 2012 The Authors. Pathology International © 2012 Japanese Society of Pathology and Blackwell Publishing Asia Pty Ltd.

  9. Original Research. The Evaluation of Caries Severity Index and Dental Hypoplasia in Children with Acute Lymphoblastic Leukemia. Results from a Romanian Medical Center

    Directory of Open Access Journals (Sweden)

    Bica Cristina

    2017-03-01

    Full Text Available Acute lymphoblastic leukemia (ALL is a type of cancer that most frequently affects children, and its treatment involves intensive chemotherapy, which might interfere with the normal development of dental tissues. The aim of our study was to measure the incidence of dental caries and enamel hypoplasia in children diagnosed with ALL treated according to the Berlin-Frankfurt-Munster-95 (ALL-BFM-95 protocol during the complete remission phase. Two groups of children between 8-12 years of age were investigated: Group 1 consisted of 36 children with ALL, and Group 2 of 58 control age-matched children. The decay-missing-filling index for the deciduous teeth (DMFT and the presence of hypoplasia in the first permanent molars (MH or in both incisors and molars (MIH were recorded. The results were statistically analyzed and showed that there were no differences between the groups regarding the DMFT values (p >0.05, but there was a statistically significant difference in the incidence of MH and MIH between groups (p <0.05. According to our results, chemotherapy was not responsible for the decay process, as there were no differences in DMFT indices between the groups, but the high incidence of MH and MIH in the ALL group indicates the need of a good dental care for these children in order to prevent future dental complications.

  10. Systolic left ventricular function according to left ventricular concentricity and dilatation in hypertensive patients

    DEFF Research Database (Denmark)

    Bang, Casper; Gerdts, Eva; Aurigemma, Gerard P

    2013-01-01

    Left ventricular hypertrophy [LVH, high left ventricular mass (LVM)] is traditionally classified as concentric or eccentric based on left ventricular relative wall thickness. We evaluated left ventricular systolic function in a new four-group LVH classification based on left ventricular dilatation...... [high left ventricular end-diastolic volume (EDV) index and concentricity (LVM/EDV)] in hypertensive patients....

  11. Comparison of conventional inserts and an add-on electron MLC for chest wall irradiation of left-sided breast cancer

    International Nuclear Information System (INIS)

    Vatanen, Tero; Lahtinen, Tapani; Traneus, Erik

    2009-01-01

    Background. Collimation of irregularly shaped clinical electron beams is currently based on electron inserts made of low melting point alloys. The present investigation compares a conventional electron applicator with insert and add-on eMLC-based dose distributions in the postoperative chest wall irradiation of left-sided breast cancer. Material and methods. Voxel Monte Carlo++ (VMC++) calculated dose distributions related to electron fields were compared with 10 left-sided breast cancer patients after radical mastectomy. The prescription dose was 50 Gy at a build-up maximum. The same dose was prescribed for the ipsilateral axillary, parasternal and supraclavicular lymph nodes that were treated with photons and calculated with a pencil beam algorithm. The insert beams were shaped with 1.5 cm thick Wood's metal electron inserts in an electron applicator of a Varian 2100 C/D linac. Doses for the eMLC-shaped beams were calculated for an eMLC prototype with 2 cm thick and 5 mm wide steel leaves. The same collimator-to-surface distance (CSD) of 5.8 cm was used for both collimators. Results. The mean PTV dose was slightly higher for the eMLC plans (50.7 vs 49.5 Gy, p<0.001, respectively). The maximum doses assessed by D5% for the eMLC and insert were 60.9 and 59.1 Gy (p<0.001). The difference was due to the slightly higher doses near the field edges for the eMLC. The left lung V20 volumes were 34.5% and 34.0% (p<0.001). There was only a marginal difference in heart doses. Discussion: Despite a slight increase of maximum dose in PTV the add-on electron MLC for chest wall irradiation results in practically no differences in dose distributions compared with the present insert-based collimation

  12. ''Natural'' left-right symmetry

    International Nuclear Information System (INIS)

    Mohapatra, R.N.; Pati, J.C.

    1975-01-01

    It is remarked that left-right symmetry of the starting gauge interactions is retained as a ''natural'' symmetry if it is broken in no way except possibly by mass terms in the Lagrangian. The implications of this result for the unification of coupling constants and for parity nonconservation at low and high energies are stressed

  13. Interpreting sex differences in enamel hypoplasia in human and non-human primates: Developmental, environmental, and cultural considerations.

    Science.gov (United States)

    Guatelli-Steinberg, D; Lukacs, J R

    1999-01-01

    The purpose of this review is to provide a synoptic, critical evaluation of the evidence of, and potential etiological factors contributing to, sex differences in the expression of enamel hypoplasia (EH). Specifically, this review considers theoretical expectations and empirical evidence bearing on two central issues. The first of these is the impact of a theorized inherent male vulnerability to physiological stress on sex differences in EH. The second issue is the potential contribution to sex differences in EH of intrinsic differences in male and female enamel composition and development. To address this first issue, EH frequencies by sex are examined in samples subject to a high degree of physiological stress. Based on the concept of inherent male vulnerability (or female buffering), males in stressful environments would be expected to exhibit higher EH frequencies than females. This expectation is evaluated in light of cultural practices of sex-biased investment that mediate the relationship between environmental stress and EH expression. Defects forming prenatally afford an opportunity to study this relationship without the confounding effects of sex-biased postnatal investment. Data bearing on this issue derive from previously conducted studies of EH in permanent and deciduous teeth in both modern and archaeological samples as well as from new data on Indian schoolchildren. To address the second issue, fundamental male-female enamel differences are evaluated for their potential impact on EH expression. A large sex difference in the duration of canine crown formation in non-human primates suggests that male canines may have greater opportunity to record stress events than those of females. This expectation is examined in great apes, whose canines often record multiple episodes of stress and are sexually dimorphic in crown formation times. With respect to the first issue, in most studies, sex differences in EH prevalence are statistically nonsignificant

  14. Left ventricular filling under elevated left atrial pressure

    Science.gov (United States)

    Gaddam, Manikantam; Samaee, Milad; Santhanakrishnan, Arvind

    2017-11-01

    Left atrial pressure (LAP) is elevated in diastolic dysfunction, where left ventricular (LV) filling is impaired due to increase in ventricular stiffness. The impact of increasing LAP and LV stiffness on intraventricular filling hemodynamics remains unclear. We conducted particle image velocimetry and hemodynamics measurements in a left heart simulator (LHS) under increasing LAP and LV stiffness at a heart rate of 70 bpm. The LHS consisted of a flexible-walled LV physical model fitted within a fluid-filled chamber. LV wall motion was generated by a piston pump that imparted pressure fluctuations in the chamber. Resistance and compliance elements in the flow loop were adjusted to obtain bulk physiological hemodynamics in the least stiff LV model. Two LV models of increasing stiffness were subsequently tested under unchanged loop settings. LAP was varied between 5-20 mm Hg for each LV model, by adjusting fluid level in a reservoir upstream of the LV. For constant LV stiffness, increasing LAP lowered cardiac output (CO), while ejection fraction (EF) and E/A ratio were increased. For constant LAP, increasing LV stiffness lowered CO and EF, and increased E/A ratio. The implications of these altered hemodynamics on intraventricular filling vortex characteristics will be presented.

  15. Left Activism, Succour and Selfhood

    DEFF Research Database (Denmark)

    Hughes, Celia Penelope

    2014-01-01

    At the height of mass activity on the Left, the ascendancy of the women's liberation movement (WLM), and the beginnings of real social and personal change for men and women, the 1970s are increasingly seen as the decade when sixties permissiveness began to be truly felt in Britain. This article...... draws upon a personal archive of correspondence from this turbulent decade, between two revolutionary women, Di Parkin and Annie Howells. It argues that the women's letters form an important contribution to new understandings about the construction of the post-war gendered self. The letters represent...... an interchange of motherhood, domesticity, far-left politics, and close female friendship. The article will show how the women's epistolary friendship offers intimate insight into female self-fashioning at a breakthrough social and political moment in 1970s Britain. As they reflected on some of the key political...

  16. Time left in the mouse.

    Science.gov (United States)

    Cordes, Sara; King, Adam Philip; Gallistel, C R

    2007-02-22

    Evidence suggests that the online combination of non-verbal magnitudes (durations, numerosities) is central to learning in both human and non-human animals [Gallistel, C.R., 1990. The Organization of Learning. MIT Press, Cambridge, MA]. The molecular basis of these computations, however, is an open question at this point. The current study provides the first direct test of temporal subtraction in a species in which the genetic code is available. In two experiments, mice were run in an adaptation of Gibbon and Church's [Gibbon, J., Church, R.M., 1981. Time left: linear versus logarithmic subjective time. J. Exp. Anal. Behav. 7, 87-107] time left paradigm in order to characterize typical responding in this task. Both experiments suggest that mice engaged in online subtraction of temporal values, although the generalization of a learned response rule to novel stimulus values resulted in slightly less systematic responding. Potential explanations for this pattern of results are discussed.

  17. Spectrum of pontocerebellar hypoplasia in 13 girls and boys with CASK mutations: confirmation of a recognizable phenotype and first description of a male mosaic patient

    Directory of Open Access Journals (Sweden)

    Burglen Lydie

    2012-03-01

    Full Text Available Abstract Background Pontocerebellar hypoplasia (PCH is a heterogeneous group of diseases characterized by lack of development and/or early neurodegeneration of cerebellum and brainstem. According to clinical features, seven subtypes of PCH have been described, PCH type 2 related to TSEN54 mutations being the most frequent. PCH is most often autosomal recessive though de novo anomalies in the X-linked gene CASK have recently been identified in patients, mostly females, presenting with intellectual disability, microcephaly and PCH (MICPCH. Methods Fourteen patients (12 females and two males; aged 16 months-14 years presenting with PCH at neuroimaging and with clinical characteristics unsuggestive of PCH1 or PCH2 were included. The CASK gene screening was performed using Array-CGH and sequencing. Clinical and neuroradiological features were collected. Results We observed a high frequency of patients with a CASK mutation (13/14. Ten patients (8 girls and 2 boys had intragenic mutations and three female patients had a Xp11.4 submicroscopic deletion including the CASK gene. All were de novo mutations. Phenotype was variable in severity but highly similar among the 11 girls and was characterized by psychomotor retardation, severe intellectual disability, progressive microcephaly, dystonia, mild dysmorphism, and scoliosis. Other signs were frequently associated, such as growth retardation, ophthalmologic anomalies (glaucoma, megalocornea and optic atrophy, deafness and epilepsy. As expected in an X-linked disease manifesting mainly in females, the boy hemizygous for a splice mutation had a very severe phenotype with nearly no development and refractory epilepsy. We described a mild phenotype in a boy with a mosaic truncating mutation. We found some degree of correlation between severity of the vermis hypoplasia and clinical phenotype. Conclusion This study describes a new series of PCH female patients with CASK inactivating mutations and confirms that

  18. Does the serum uric acid level have any relation to arterial stiffness or blood pressure in adults with congenital renal agenesis and/or hypoplasia?

    Science.gov (United States)

    Yazici, Raziye; Guney, İbrahim; Altintepe, Lutfullah; Yazici, Mehmet

    2017-01-01

    The relationship between serum uric acid and arterial stiffness or blood pressure is not clear. The serum uric acid level and its association with cardiovascular risk is not well known in patients with reduced renal mass. We aimed to investigate the relation between serum uric acid levels and arterial stiffness and also blood pressure in patients with congenital renal agenesis and/or hypoplasia. In this single center, cross-sectional study, a total of 55 patients (39 (% 70.9) with unilateral small kidney and 16 (%29.1) with renal agenesis) were included. The median age was 35 (21-50) years. The study population was divided into tertiles of serum uric acid (according to 2.40-3.96, 3.97-5.10, and 5.11-9.80 mg/dl cut-off values of serum uric acid levels). Official and 24-h ambulatory non-invasive blood pressures of all patients were measured. The arterial stiffness was assessed by pulse wave velocity (PWV). PWV values were increased from first to third tertile (5.5 ± 0.6, 5.7 ± 0.8, 6.1 ± 0.7, respectively), but this gradual increase between tertiles did not reach significance. Linear regression analyses showed a positive correlation between serum uric acid levels and PWV (β = 0.40, p = 0.010), but no correlation was found between uric acid and daytime systolic blood pressure (β = 0.24, p = 0.345). In congenital renal agenesis/hypoplasia, the serum uric acid level was positively correlated with arterial stiffness, but there was no correlation with blood pressure.

  19. Rebuilding the US Health Left

    Directory of Open Access Journals (Sweden)

    Victor W. Sidel, MD

    2010-02-01

    Full Text Available With this issue Social Medicine begins a series of invited papers on the topic: “Rebuilding the US Health Left.” In this editorial we will outline our vision for this series. We undertake this project aware that our good friend and mentor, Dr. Walter Lear, one of the leading health activists of the 20th century, lies critically ill. Walter was the creator and custodian of the US Health Left Archives, a collection that is now with the University of Pennsylvania library. The collection reminds us of the important role left health care workers played in US history throughout the 20th century. They advocated for a national health program (Committee on the Costs of Medical Care, Physicians Forum, Medical Care Section/APHA, HealthPAC, Physicians for a National Health Program, National Physicians Alliance, provided international solidarity (American Soviet Medical Society, international brigades during the Spanish Civil War, Central American Solidarity Movement, Committee to Help Chilean Health Workers, Doctors for Global Health, traced the connections between disease and social class (Sigerist Circle, Spirit of 1848, APHA, fought for workers’ health (Councils for Occupational Safety and Health; Occupational Health and Safety Section, APHA participated in anti-war movements (Medical Committee for Human Rights, Physicians for Social Responsibility, International Physicians for the Prevention of Nuclear War, created new models of health care delivery (Health Cooperatives, Prepaid Health Maintenance Organizations, Community Health Centers, National Health Service Corps, Free Clinics, were central to the struggle for women’s rights (Planned Parenthood, Physicians for Reproductive Choice and Health, supported the civil rights movement both in medicine and in the broader society (National Medical Association, Medical Committee for Human Rights, played key roles in the movement for gay rights (ACT-UP, Gay & Lesbian Medical Association, Lesbian, Gay

  20. Bilateral Persistent Sciatic Arteries Complicated with Acute Left Lower Limb Ischemia

    Directory of Open Access Journals (Sweden)

    Hsuan-Yin Wu

    2007-12-01

    Full Text Available Persistent sciatic artery (PSA is a rare congenital malformation. In the early embryonic stage, the sciatic artery is the major blood supply for the lower limb bulb and is later replaced by the iliofemoral artery as the limb develops. Its failure to regress, sometimes associated with femoral arterial hypoplasia, and therefore becoming the dominant inflow to the lower extremity is called PSA. This anomaly is often associated with a higher rate of aneurysm formation or thromboembolic complications causing lower extremity ischemia. Here, we describe a 79-year-old male patient who presented with acute left lower extremity ischemia. He was treated initially with conventional embolectomy through inguinal and popliteal incisions. The bilateral PSA with thrombosed aneurysms was not identified at first on computed tomographic angiography. It was later diagnosed intraoperatively due to the discontinuity of the superficial femoral artery and popliteal artery found with embolectomy catheter, and was managed successfully with ePTFE graft bypass. Careful interpretation of the imaging study may be helpful in preoperative diagnosis.

  1. Flavoured Dark Matter moving left

    Science.gov (United States)

    Blanke, Monika; Das, Satrajit; Kast, Simon

    2018-02-01

    We investigate the phenomenology of a simplified model of flavoured Dark Matter (DM), with a dark fermionic flavour triplet coupling to the left-handed SU(2) L quark doublets via a scalar mediator. The DM-quark coupling matrix is assumed to constitute the only new source of flavour and CP violation, following the hypothesis of Dark Minimal Flavour Violation. We analyse the constraints from LHC searches, from meson mixing data in the K, D, and B d,s meson systems, from thermal DM freeze-out, and from direct detection experiments. Our combined analysis shows that while the experimental constraints are similar to the DMFV models with DM coupling to right-handed quarks, the multitude of couplings between DM and the SM quark sector resulting from the SU(2) L structure implies a richer phenomenology and significantly alters the resulting impact on the viable parameter space.

  2. Syndrome of hypoplasic left ventricle, Description of a clinical case with survival after carrying out of Norwood technique

    International Nuclear Information System (INIS)

    Velasco B, Ana M; Lince V, Rafael; Zapata S, Jorge A and others

    2003-01-01

    A clinical case of a 72 hours newborn patient admitted to the pediatric intensive care unit for reduced cardiac output syndrome showing anuria, metabolic acidosis and respiratory distress, is reported. Mechanical ventilation, inotropic support and continuous prostaglandin e1 infusion were initiated. The echocardiogram showed hypoplasia of the left ventricle. The Norwood procedure stage i under extracorporeal circulation with deep hypothermia and circulatory arrest, was performed at the 6th day of life. During the early postoperative period, the patient developed hypoxaemia and pulmonary hypertension, which improved with controlled hyperventilation and vasodilatation therapy. Other complications were clinical sepsis and abstinence syndrome, which resolved with medical management. The patient was extubated on the sixth postoperative day. Subsequent echocardiograms showed tricuspid valve regurgitation, right ventricular dysfunction with dilation and an organized thrombus in the left atrium. These improved with diuretics, dopamine, milrinone and heparin. The patient was discharged after 32 days of the surgery without signs of congestive heart failure. This case is important, given the high mortality rate of this pathology. The Norwood procedure proved to be a palliative strategy versus the ortotopic cardiac transplant in newborns, because of the difficulty in finding donators at this age and the immunosuppressive therapy complications. This represents a great advance in the Colombian pediatric cardiology because until recent years these patients did not have any other surgical alternative

  3. An elusive persistent left superior vena cava draining into left atrium

    NARCIS (Netherlands)

    A. Soward; F.J. ten Cate (Folkert); P.M. Fioretti (Paolo); P.W.J.C. Serruys (Patrick); J.R.T.C. Roelandt (Jos)

    1986-01-01

    textabstractA case report of a persistent left superior vena cava draining into left atrium with a fibromuscular left ventricular outflow tract obstruction and a small atrial septal defect. The anomalous vessel escaped detection during two right and left heart catheterizations from the right arm and

  4. HIPOPLASIA CEREBELAR E PORENCEFALIA EM BOVINOS CHAROLÊS NO SUL DO RIO GRANDE DO SUL CEREBELLAR HYPOPLASIA AND PORENCEPHALY IN CHAROLAIS CATLLE IN SOUTHERN BRAZIL

    Directory of Open Access Journals (Sweden)

    Ana Lucia Schild

    2001-02-01

    Full Text Available Descrevem-se dois casos de hipoplasia cerebelar congênita observados em terneiros da raça Charolês, pertencentes a um estabelecimento localizado no município de Capão do Leão. Um dos animais apresentava sinais clínicos cerebelares ao nascimento, caracterizados por severa incoordenação motora, hipermetria e tremores de intenção, principalmente da cabeça e do pescoço. O outro animal não conseguia manter-se em estação e apresentava opistótono acentuado. As lesões macroscópicas caracterizaram-se por hipoplasia cerebelar, observando-se apenas remanescentes das regiões do flóculo e do paraflóculo cerebelares e uma cavidade preenchida por líquido e recoberta pelas leptomeninges, que estavam aderidas aos resquícios das folhas cerebelares. Ao corte do encéfalo, havia hidrocefalia discreta e a presença de cavidades císticas (porencefalia. São discutidas as possíveis etiologias, sugerindo-se a possibilidade de que a enfermidade tenha sido causada pelo vírus da diarréia viral bovina-doença das mucosas, apesar dos resultados negativos da imuno-histoquímica realizada em fragmentos do cerebelo dos animais doentes para detecção desse vírus.Two cases of cerebellar hypoplasia in Charolais calves from a herd of a farm located in the municipality of Capão do Leão, Southern Brazil are described. One calf with cerebellar clinical signs at birth, showed incoordination, hypermetria and intention tremors, mainly in the neck and head. The other calf was recumbent with opisthotonos. The gross lesions were characterized by cerebellar hypoplasia, with only remnants of cerebellar floculus and parafloculus and an expanded fluid-filled space covered by leptomeninges. Mild hydrocephalus and porencephaly were observed in the cerebrum. It was discussed the possible etiologies and suggested the possibility of the disease has been caused by bovine viral diarrhea-mucosal disease (BVD-MD virus despite of negative results of imuno

  5. Exome sequencing reveals a de novo POLD1 mutation causing phenotypic variability in mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome (MDPL).

    Science.gov (United States)

    Elouej, Sahar; Beleza-Meireles, Ana; Caswell, Richard; Colclough, Kevin; Ellard, Sian; Desvignes, Jean Pierre; Béroud, Christophe; Lévy, Nicolas; Mohammed, Shehla; De Sandre-Giovannoli, Annachiara

    2017-06-01

    Mandibular hypoplasia, deafness, progeroid features, and lipodystrophy syndrome (MDPL) is an autosomal dominant systemic disorder characterized by prominent loss of subcutaneous fat, a characteristic facial appearance and metabolic abnormalities. This syndrome is caused by heterozygous de novo mutations in the POLD1 gene. To date, 19 patients with MDPL have been reported in the literature and among them 14 patients have been characterized at the molecular level. Twelve unrelated patients carried a recurrent in-frame deletion of a single codon (p.Ser605del) and two other patients carried a novel heterozygous mutation in exon 13 (p.Arg507Cys). Additionally and interestingly, germline mutations of the same gene have been involved in familial polyposis and colorectal cancer (CRC) predisposition. We describe a male and a female patient with MDPL respectively affected with mild and severe phenotypes. Both of them showed mandibular hypoplasia, a beaked nose with bird-like facies, prominent eyes, a small mouth, growth retardation, muscle and skin atrophy, but the female patient showed such a severe and early phenotype that a first working diagnosis of Hutchinson-Gilford Progeria was made. The exploration was performed by direct sequencing of POLD1 gene exon 15 in the male patient with a classical MDPL phenotype and by whole exome sequencing in the female patient and her unaffected parents. Exome sequencing identified in the latter patient a de novo heterozygous undescribed mutation in the POLD1 gene (NM_002691.3: c.3209T>A), predicted to cause the missense change p.Ile1070Asn in the ZnF2 (Zinc Finger 2) domain of the protein. This mutation was not reported in the 1000 Genome Project, dbSNP and Exome sequencing databases. Furthermore, the Isoleucine1070 residue of POLD1 is highly conserved among various species, suggesting that this substitution may cause a major impairment of POLD1 activity. For the second patient, affected with a typical MDPL phenotype, direct sequencing

  6. A Newborn with Icthyosis, Corpus Callosum Hypoplasia, Microcephaly, Atrichia and Intra Uterine Growth Retardation (IUGR: AVariant of Icthyosis Follicularis Atrichia Photophobia (IFAP or Brain Anomalies, Retardation, Ectodermal Dysplasia, Skeletal Deformities, Hirschsprung Disease, Ear/Eye Anomalies, Cleft Palate, Cryptorchidism (BRESHECK?

    Directory of Open Access Journals (Sweden)

    Gurudutt S. Joshi

    2017-10-01

    Full Text Available A full term newborn small for gestational age Intra Uterine Growth Retardation (IUGR admitted with congenital dysmorphic features with icthyosis, atrichia, microcephaly and eye abnormalities, when explored further for other congenital malformations, revealed Corpus callosum hypoplasia and closely related features with two rare syndromes Icthyosis Follicularis Atrichia Photophobia (IFAP or Brain Anomalies Retardation, Ectodermal Dysplasia, Skeletal Deformities, Hirschsprung Disease, Hemivertebrae, Ear/Eye Anomalies, and Kidney Dysplasia (BRESHECK.

  7. Association of A Dilated Coronary Sinus in the Fetus with Actual and Apparent Coarctation of the Aorta and Diminutive Left Heart Structures.

    Science.gov (United States)

    Ramaswamy, Prema; Rafii, Daniela; Osmolovsky, Marina; Agarwal, Arpit; Amirtharaj, Cynthia

    2016-12-01

    Evidence suggests an association between left heart obstructive lesions and dilated coronary sinus (DCS), but this has not been studied in fetuses. A retrospective review of fetal echocardiograms (FE) over an 8-year period was conducted, and patients with DCS were identified and confirmed postnatally. There were 5840 FE performed on 4920 women during this period. Of 49 patients with DCS, 22 had normal intracardiac anatomy and 27 patients had congenital heart disease (CHD) yielding an incidence of 4.6 % in the presence of CHD (27/584). Of 27 patients with DCS and CHD, approximately a third had either hypoplastic left ventricles and/or coarctations (10/27, 37 %). The incidence of left heart obstructive lesions was much higher in the presence of a DCS (37 % vs 45/557, 8 %, p < 0.0001). The odds ratio of left heart hypoplasia in fetuses with CHD and a DCS was 6.6 (95 % CI 2.8-15.3). Comparison of patients with postnatally confirmed coarctation with those with normal intracardiac anatomy with DCS, revealed that in the former, the right ventricle (p = 0.005), pulmonic valve annulus (p = 0.0001) and the tricuspid inflow were larger (p = 0.001) compared to corresponding left-sided structures. The size of the DCS was not significantly different between the two groups, but in the former, the DCS was more closely related to the posterior leaflet of the mitral valve and caused a significant diminution of the mitral inflow. Our study suggests a strong association, possibly causal, between left heart obstructive lesions and DCS in utero.

  8. Pulmonary hypoplasia-diaphragmatic hernia-anophthalmia-cardiac defect (PDAC) syndrome due to STRA6 mutations--what are the minimal criteria?

    Science.gov (United States)

    Segel, Reeval; Levy-Lahad, Ephrat; Pasutto, Francesca; Picard, Elie; Rauch, Anita; Alterescu, Gheona; Schimmel, Michael S

    2009-11-01

    Microphthalmic syndrome 9 (OMIM601186) is a genetically and phenotypically variable condition, comprising anophthalmia, pulmonary hypoplasia, diaphragmatic hernia, and cardiac malformations (PDAC syndrome). Reported cases have all been associated with fetal/neonatal death or developmental delay. Recessive stimulated by retinoic acid gene 6 homolog (STRA6) mutations have recently been identified as the cause of cases of PDAC in which distinct, "bushy" eyebrows have been observed. We describe a patient with clinical anophthalmia, bushy eyebrows, patent ductus arteriosus, and normal development at age 30 months, who is a compound heterozygote for two novel STRA6 missense mutations. This patient's phenotype is consistent with the multisystemic malformations of PDAC syndrome, but is somewhat milder. This is the first living patient with compound heterozygous STRA6 mutations, which may explain her milder phenotype. We conclude that STRA6 analysis should be considered in all patients with clinical anophthalmia. Genetic counseling should be cautious with respect to long-term developmental outcomes. Copyright 2009 Wiley-Liss, Inc.

  9. ALDH1A3 loss of function causes bilateral anophthalmia/microphthalmia and hypoplasia of the optic nerve and optic chiasm.

    Science.gov (United States)

    Yahyavi, Mani; Abouzeid, Hana; Gawdat, Ghada; de Preux, Anne-Sophie; Xiao, Tong; Bardakjian, Tanya; Schneider, Adele; Choi, Alex; Jorgenson, Eric; Baier, Herwig; El Sada, Mohamad; Schorderet, Daniel F; Slavotinek, Anne M

    2013-08-15

    The major active retinoid, all-trans retinoic acid, has long been recognized as critical for the development of several organs, including the eye. Mutations in STRA6, the gene encoding the cellular receptor for vitamin A, in patients with Matthew-Wood syndrome and anophthalmia/microphthalmia (A/M), have previously demonstrated the importance of retinol metabolism in human eye disease. We used homozygosity mapping combined with next-generation sequencing to interrogate patients with anophthalmia and microphthalmia for new causative genes. We used whole-exome and whole-genome sequencing to study a family with two affected brothers with bilateral A/M and a simplex case with bilateral anophthalmia and hypoplasia of the optic nerve and optic chiasm. Analysis of novel sequence variants revealed homozygosity for two nonsense mutations in ALDH1A3, c.568A>G, predicting p.Lys190*, in the familial cases, and c.1165A>T, predicting p.Lys389*, in the simplex case. Both mutations predict nonsense-mediated decay and complete loss of function. We performed antisense morpholino (MO) studies in Danio rerio to characterize the developmental effects of loss of Aldh1a3 function. MO-injected larvae showed a significant reduction in eye size, and aberrant axonal projections to the tectum were noted. We conclude that ALDH1A3 loss of function causes anophthalmia and aberrant eye development in humans and in animal model systems.

  10. Optic nerve size evaluated by magnetic resonance imaging in children with optic nerve hypoplasia, multiple pituitary hormone deficiency, isolated growth hormone deficiency, and idiopathic short stature.

    Science.gov (United States)

    Birkebaek, Niels Holtum; Patel, Leena; Wright, Neville Bryce; Grigg, John Russell; Sinha, Smeeta; Hall, Catherine Margaret; Price, David Anthony; Lloyd, Ian Christopher; Clayton, Peter Ellis

    2004-10-01

    To objectively define criteria for intracranial optic nerve (ON) size in ON hypoplasia (ONH) on magnetic resonance imaging (MRI) scans. Intracranial ON sizes from MRI were compared between 46 children with ONH diagnosed by ophthalmoscopy (group 1, isolated ONH, 8 children; and group 2, ONH associated with abnormalities of the hypothalamic-pituitary axis and septum pellucidum, 38 children) and children with multiple pituitary hormone deficiency (group 3, multiple pituitary hormone deficiency, 14 children), isolated growth hormone deficiency (group 4, isolated growth hormone deficiency, 15 children), and idiopathic short stature (group 5, idiopathic short stature, 10 children). Intracranial ON size was determined by the cross-sectional area, calculated as [pi x (1/2) height x (1/2) width]. Groups 1 and 2 had lower intracranial ON size than did groups 3, 4, and 5 (P imaging of the ONs with cross-sectional area short child more than 12 months of age, with or without hypothalamic-pituitary axis abnormalities, confirms the clinical diagnosis of ONH.

  11. Neuroimaging findings in Joubert syndrome with C5orf42 gene mutations: A milder form of molar tooth sign and vermian hypoplasia.

    Science.gov (United States)

    Enokizono, Mikako; Aida, Noriko; Niwa, Tetsu; Osaka, Hitoshi; Naruto, Takuya; Kurosawa, Kenji; Ohba, Chihiro; Suzuki, Toshifumi; Saitsu, Hirotomo; Goto, Tomohide; Matsumoto, Naomichi

    2017-05-15

    Little is known regarding neuroimaging-genotype correlations in Joubert syndrome (JBTS). To elucidate one of these correlations, we investigated the neuroimaging findings of JBTS patients with C5orf42 mutations. Neuroimaging findings in five JBTS patients with C5orf42 mutations were retrospectively assessed with regard to the infratentorial and supratentorial structures on T1-magnetization prepared rapid gradient echo (MPRAGE), T2-weighted images, and color-coded fractional anisotropy (FA) maps; the findings were compared to those in four JBTS patients with mutations in other genes (including three with AHI1 and one with TMEM67 mutations). In C5orf42-mutant patients, the infratentorial magnetic resonance (MR) images showed normal or minimally thickened and minimally elongated superior cerebellar peduncles (SCP), normal or minimally deepened interpeduncular fossa (IF), and mild vermian hypoplasia (VH). However, in other patients, all had severe abnormalities in the SCP and IF, and moderate to marked VH. Supratentorial abnormalities were found in one individual in other JBTS. In JBTS with all mutations, color-coded FA maps showed the absence of decussation of the SCP (DSCP). The morphological neuroimaging findings in C5orf42-mutant JBTS were distinctly mild and made diagnosis difficult. However, the absence of DSCP on color-coded FA maps may facilitate the diagnosis of JBTS. Copyright © 2017 Elsevier B.V. All rights reserved.

  12. Are all pulmonary hypoplasias the same? A comparison of pulmonary outcomes in neonates with congenital diaphragmatic hernia, omphalocele and congenital lung malformation.

    Science.gov (United States)

    Akinkuotu, Adesola C; Sheikh, Fariha; Cass, Darrell L; Zamora, Irving J; Lee, Timothy C; Cassady, Christopher I; Mehollin-Ray, Amy R; Williams, Jennifer L; Ruano, Rodrigo; Welty, Stephen E; Olutoye, Oluyinka O

    2015-01-01

    Patients with congenital diaphragmatic hernias (CDH), omphaloceles, and congenital lung malformations (CLM) may have pulmonary hypoplasia and experience respiratory insufficiency. We hypothesize that given equivalent lung volumes, the degree of respiratory insufficiency will be comparable regardless of the etiology. Records of all fetuses with CDH, omphalocele, and CLM between January 2000 and June 2013 were reviewed. MRI-based observed-to-expected total fetal lung volumes (O/E-TFLV) were calculated. An analysis of outcomes in patients with O/E-TFLV between 40% and 60%, the most inclusive range, was performed. 285 patients were evaluated (161, CDH; 24, omphalocele; 100, CLM). Fetuses with CDH had the smallest mean O/E-TFLV. CDH patients were intubated for longer and had a higher incidence of pulmonary hypertension. Fifty-six patients with the three diagnoses had an O/E-TFLV of 40%-60%. The need for ECMO, supplemental oxygen at 30days of life, and 6-month mortality were similar among groups. CDH patients had a significantly longer duration of intubation and higher incidence of pulmonary hypertension than the other two diagnoses. Given equivalent lung volumes (40%-60% of expected), CDH patients require more pulmonary support initially than omphalocele and CLM patients. In addition to lung volumes, disease-specific factors, such as pulmonary hypertension in CDH, also contribute to pulmonary morbidity and overall outcome. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. A Family with Mental Retardation, Epilepsy and Cerebellar Hypoplasia Showing Linkage to Chromosome 20p11.21-q11.23

    Directory of Open Access Journals (Sweden)

    Fatih Bayrakli

    2014-01-01

    Full Text Available Background: Cerebellar hypoplasia (CH is a rare malformation caused by various etiologies, usually manifesting clinically as nonprogressive cerebellar ataxia with or without mental retardation. The molecular pathogenesis of the autosomal recessive cerebellar ataxias has a wide range of mechanisms. Differential diagnosis and categorization of the recessive cerebellar ataxias, however, need more specific, biochemical and genetic investigation. Methods: This study applied whole-genome linkage analysis to study a family with nonprogressive cerebellar ataxia and additional mental retardation, epilepsy, and facial dysmorphic features. Genotyping and linkage analysis was done using the GeneChip Mapping 250K NspI Array (Affymetrix Inc., Santa Clara, Calif., USA for genome-wide linkage analysis of the genotyping data from the affected children and their parents. Results: Allegro software version 1.2 was used for multipoint linkage analysis. We assumed an autosomal recessive inheritance pattern and assigned a penetrance of 0.999. Single-nucleotide polymorphism allele frequencies were estimated from the Affymetrix data of the Caucasian family studied. Using these parameters, a theoretical maximum logarithm of the odds score of 2.69 was identified at chromosome 20p11.21-q11.23. Conclusions: This chromosomal locus is unprecedented in autosomal recessive and nonprogressive ataxia disorder. Further investigation might reveal a new causative gene generating the CH phenotype.

  14. Comprehensive investigation of CASK mutations and other genetic etiologies in 41 patients with intellectual disability and microcephaly with pontine and cerebellar hypoplasia (MICPCH.

    Directory of Open Access Journals (Sweden)

    Shin Hayashi

    Full Text Available The CASK gene (Xp11.4 is highly expressed in the mammalian nervous system and plays several roles in neural development and synaptic function. Loss-of-function mutations of CASK are associated with intellectual disability and microcephaly with pontine and cerebellar hypoplasia (MICPCH, especially in females. Here, we present a comprehensive investigation of 41 MICPCH patients, analyzed by mutational search of CASK and screening of candidate genes using an SNP array, targeted resequencing and whole-exome sequencing (WES. In total, we identified causative or candidate genomic aberrations in 37 of the 41 cases (90.2%. CASK aberrations including a rare mosaic mutation in a male patient, were found in 32 cases, and a mutation in ITPR1, another known gene in which mutations are causative for MICPCH, was found in one case. We also found aberrations involving genes other than CASK, such as HDAC2, MARCKS, and possibly HS3ST5, which may be associated with MICPCH. Moreover, the targeted resequencing screening detected heterozygous variants in RELN in two cases, of uncertain pathogenicity, and WES analysis suggested that concurrent mutations of both DYNC1H1 and DCTN1 in one case could lead to MICPCH. Our results not only identified the etiology of MICPCH in nearly all the investigated patients but also suggest that MICPCH is a genetically heterogeneous condition, in which CASK inactivating mutations appear to account for the majority of cases.

  15. Cardiac dose-sparing effects of deep-inspiration breath-hold in left breast irradiation. Is IMRT more beneficial than VMAT

    Energy Technology Data Exchange (ETDEWEB)

    Sakka, Mazen; Grabenbauer, Gerhard G. [Coburg Cancer Center, Department of Radiation Oncology, Coburg (Germany); Friedrich-Alexander University of Erlangen-Nuernberg, Faculty of Medicine, Erlangen (Germany); Kunzelmann, Leonie; Metzger, Martin [Coburg Cancer Center, Department of Radiation Oncology, Coburg (Germany)

    2017-10-15

    .01) techniques as compared with FB radiation. There were no significant changes in left lung dose by IMRT; however, with VMAT planning, mean dose to the left lung was reduced by -4% (p = 0.0004). In addition, DIBH significantly increased the mean dose to the contralateral breast with IMRT (+14%, p = 0.002) and significantly reduced the dose to the contralateral breast with VMAT planning (-9%, p = 0.003) compared with the FB position. Additionally, in comparison with VMAT, the IMRT technique reduced mean heart dose both in the FB and the DIBH-position by -30% (p = 0.0004) and -26% (p = 0.002), respectively. Furthermore, IMRT increased the mean dose to the left lung in both the FB and the DIBH position (+5%, p = 0.003, p = 0.006), respectively. There were no significant changes in dose to the right lung and contralateral breast either in the FB or DIBH position between IMRT and VMAT techniques. Left-sided breast irradiation is best performed in the DIBH position, since a considerable dose sparing to the heart and LADCA can be achieved by using either IMRT or VMAT techniques. A significant additional decrease in heart and LADCA dose by IMRT in both FB and DIBH irradiation was seen compared with VMAT. (orig.) [German] Auf der Basis von Metaanalysen, die eine signifikante Verbesserung der Lokalrezidivraten, aber auch des Ueberlebens zeigten, gilt die adjuvante Radiotherapie heute als Standardbehandlung nach brusterhaltender Therapie des Mammakarzinoms. Beim linksseitigen Mammakarzinom wurde allerdings eine erhoehte Rate fataler kardiovaskulaerer Ereignisse infolge einer Exposition des Herzens mit ionisierender Strahlung ermittelt. Es besteht die Hypothese, dass diese kardiale Toxizitaet minimiert werden kann, und zwar durch die Bestrahlung in tiefer Inspiration (DIBH) und die Anwendung aktueller Techniken, insbesondere der intensitaetsmodulierten Radiotherapie (IMRT) und der volumetrischen Rotationsbestrahlung (VMAT). Primaere Zielsetzung dieser Studie war, beim linksseitigen

  16. Left Paraduodenal Hernia: An Autopsy Case

    DEFF Research Database (Denmark)

    Omland, Silje Haukali; Hougen, Hans Petter

    2011-01-01

    We present a case of a left paraduodenal hernia diagnosed at autopsy. A left paraduodenal hernia is an internal hernia of congenital origin due to the abnormal rotation of the midgut during embryonic development. Internal hernias are a rare cause of intestinal obstruction, with the paraduodenal...

  17. Basal cell carcinoma on the left cheek

    International Nuclear Information System (INIS)

    Jancar, B.

    2007-01-01

    A 91-year-old female patient was treated with irradiation for histologically confirmed basal cell carcinoma on the left cheek. The tumour, measuring 3 x 3 cm, with the depth of 2 cm, was extending up to the lower lid of the left eye. (author)

  18. Pure Left Neglect for Arabic Numerals

    Science.gov (United States)

    Priftis, Konstantinos; Albanese, Silvia; Meneghello, Francesca; Pitteri, Marco

    2013-01-01

    Arabic numerals are diffused and language-free representations of number magnitude. To be effectively processed, the digits composing Arabic numerals must be spatially arranged along a left-to-right axis. We studied one patient (AK) to show that left neglect, after right hemisphere damage, can selectively impair the computation of the spatial…

  19. Congenital Absence of Left Circumflex Coronary Artery

    Directory of Open Access Journals (Sweden)

    Zahra Ansari

    2009-09-01

    Full Text Available Congenital absence of left circumflex artery is a rare congenitalanomaly of the coronary arteries. The prevalence of theanomaly in different studies ranges from 0.6% to 1.3%. Ofthese, 80% are benign and asymptomatic and 20% are clinicallyimportant. We report a 56-year-old man presented withacute resting chest pain who was diagnosed as having acuteanterolateral infarction accompanied by electrocardiographicchanges and elevated cardiac enzymes. Coronary angiographyin different views was conducted, however, no left circumflexartery was found. The territory supplied by the artery had beenperfused by the super dominant right coronary artery. Therewas no left circumflex coronary artery with anomalous origin.Sever stenosis of left anterior ascending artery superimposedto the absent left circumflex artery was presented as acute anterolateralinfarction. Although absence of the artery is mostlyconsidered as a benign condition, atherosclerotic lesions maybe more important in such cases because of diminished compensatingmechanisms.

  20. VARIATION IN THE OPENINGS (OSTIA OF LEFT PULMONARY VEINS INTO THE LEFT ATRIUM: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Sesi

    2015-03-01

    Full Text Available During early embryonic development, absorption of pulmonary venous network by the left primitive atrial chamber results in opening of four pulmonary veins which drain independently into its chamber. The extent of absorption and hence, the number of pulmon ary veins which open into the left atrium, may vary. Here we report a variation in the opening of the Left upper (superior pulmonary vein into the Left atrium. A total of six openings observed

  1. Repair of aorto-left ventricular tunnel arising from the left sinus of valsalva.

    Science.gov (United States)

    Nezafati, Mohammad Hassan; Maleki, Mahmood Hosseinzadeh; Javan, Hadi; Zirak, Nahid

    2010-05-01

    Aortico-left ventricular tunnel (ALVT) is a rare congenital cardiac defect that bypasses the aortic valve via a para-valvular connection from the left ventricle to the aorta. In most cases, the tunnel arises from the right aortic sinus. In this case report, we are presenting a case of ALVT, of which the aortic orifice arose from the left aortic sinus, requiring special attention to avoid the left coronary artery injury at the time of surgical repair.

  2. Left Atrial Decompression by Percutaneous Left Atrial Venting Cannula Insertion during Venoarterial Extracorporeal Membrane Oxygenation Support

    Directory of Open Access Journals (Sweden)

    Ha Eun Kim

    2016-06-01

    Full Text Available Patients with venoarterial extracorporeal membrane oxygenation (ECMO frequently suffer from pulmonary edema due to left ventricular dysfunction that accompanies left heart dilatation, which is caused by left atrial hypertension. The problem can be resolved by left atrium (LA decompression. We performed a successful percutaneous LA decompression with an atrial septostomy and placement of an LA venting cannula in a 38-month-old child treated with venoarterial ECMO for acute myocarditis.

  3. TMS over the Left Angular Gyrus Impairs the Ability to Discriminate Left from Right

    Science.gov (United States)

    Hirnstein, Marco; Bayer, Ulrike; Ellison, Amanda; Hausmann, Markus

    2011-01-01

    The underlying cognitive and neural mechanisms of the ability to discriminate left from right are hardly explored. Clinical studies from patients with impairments of left-right discrimination (LRD) and neuroimaging data suggest that the left angular gyrus is particularly involved in LRD. Moreover, it is argued that the often reported sex…

  4. Social aspects of left-handedness

    Directory of Open Access Journals (Sweden)

    Belojević Goran

    2010-01-01

    Full Text Available Throughout human history left-handedness has been considered as sinful. It has been associated with the devil, weakness, female gender, unhealthiness, evil, something that has to be turned to a “good” - right side by force. Left-handedness is being more and more acceptable at rational level, but in everyday life it is still considered to be unusual if someone writes with the left hand. Lessening of the number of lefthanders is associated with ageing. There are about 13% lefthanders among people in twenties and less than 1% lefthanders among those in eighties. This finding may be explaned with more pronounced socio-cultural pressure on left-handed people in the past, compared to nowadays. On the other hand, this may also support the hypothesis about a reduced life span of lefthanded people. With cross-exercising of left-handedness, certain typical characteristics and behavioral patterns appear in these people. This was a sort of provoked behavior and an attack on the integrity of an emotional attitude toward oneself. Stuttering may also appear as a consequence of unsuccessful cross-exercising of left-handedness. The hypothesis about left-handedness as an advantage is supported with the reports about relatively more lefthanders in some specific groups such as: mathematicians, sculptors, architects, painters, musicians, actors, tennis players, as well as famous army commanders and rulers.

  5. The spinal muscular atrophy with pontocerebellar hypoplasia gene VRK1 regulates neuronal migration through an amyloid-β precursor protein-dependent mechanism.

    Science.gov (United States)

    Vinograd-Byk, Hadar; Sapir, Tamar; Cantarero, Lara; Lazo, Pedro A; Zeligson, Sharon; Lev, Dorit; Lerman-Sagie, Tally; Renbaum, Paul; Reiner, Orly; Levy-Lahad, Ephrat

    2015-01-21

    Spinal muscular atrophy with pontocerebellar hypoplasia (SMA-PCH) is an infantile SMA variant with additional manifestations, particularly severe microcephaly. We previously identified a nonsense mutation in Vaccinia-related kinase 1 (VRK1), R358X, as a cause of SMA-PCH. VRK1-R358X is a rare founder mutation in Ashkenazi Jews, and additional mutations in patients of different origins have recently been identified. VRK1 is a nuclear serine/threonine protein kinase known to play multiple roles in cellular proliferation, cell cycle regulation, and carcinogenesis. However, VRK1 was not known to have neuronal functions before its identification as a gene mutated in SMA-PCH. Here we show that VRK1-R358X homozygosity results in lack of VRK1 protein, and demonstrate a role for VRK1 in neuronal migration and neuronal stem cell proliferation. Using shRNA in utero electroporation in mice, we show that Vrk1 knockdown significantly impairs cortical neuronal migration, and affects the cell cycle of neuronal progenitors. Expression of wild-type human VRK1 rescues both proliferation and migration phenotypes. However, kinase-dead human VRK1 rescues only the migration impairment, suggesting the role of VRK1 in neuronal migration is partly noncatalytic. Furthermore, we found that VRK1 deficiency in human and mouse leads to downregulation of amyloid-β precursor protein (APP), a known neuronal migration gene. APP overexpression rescues the phenotype caused by Vrk1 knockdown, suggesting that VRK1 affects neuronal migration through an APP-dependent mechanism. Copyright © 2015 the authors 0270-6474/15/350936-08$15.00/0.

  6. Correlation of performance with endoscopic and radiographic assessment of epiglottic hypoplasia in racehorses with epiglottic entrapment corrected by use of contact neodymium:yttrium aluminum garnet laser

    International Nuclear Information System (INIS)

    Tulleners, E.P.

    1991-01-01

    Epiglottic entrapment in 35 Thoroughbred and 44 Standardbred horses was corrected transendoscopically by use of a neodymium:yttrium aluminum garnet laser. Before surgery, the entrapped epiglottis was classified as hypoplastic or normal in each horse on the basis of endoscopic appearance alone. Using a digitizer, thyroepiglottic length was determined from lateral-view laryngeal radiographs. For 78 racehorses, earnings (less than $5,000 or greater than $5,000) were compared before and after surgery. Earnings category and racing performance after surgery were tested for association with endoscopically determined epiglottic hypoplasia and radiographically determined thyroepiglottic length. Endoscopy and radiography were useful methods of evaluating the epiglottis in horses with epiglottic entrapment. Mean (+/- SD) thyroepiglottic length for both breeds of horses with epiglottic entrapment was significantly (P = 0.0001) smaller (Thoroughbreds, 7.28 +/- 0.67 cm; Standardbreds, 7.21 +/- 0.62 cm), compared with thyroepiglottic length measured from control groups composed of clinically normal Thoroughbred (8.56 +/- 0.29 cm) and Standardbred (8.74 +/- 0.38 cm) racehorses. Both breeds of horses with epiglottic entrapment that had endoscopically apparent hypoplastic epiglottis had significantly (P less than 0.0001) smaller thyroepiglottic length (Thoroughbreds, 6.64 +/- 0.60 cm; Standardbred, 6.93 +/- 0.72 cm) than did horses with epiglottic entrapment that had endoscopically normal epiglottis (Throughbreds, 7.57 +/- 0.47 cm, Standardbreds, 7.36 +/- 0.50 cm). Significant difference was not detected in endoscopic appearance of the epiglottis among age, gender, or breed distributions

  7. Dosimetric reproduction of a left-breast 3DCRT field-in-field radiation therapy planning in an anthropomorphic and anthropometric phantom

    Energy Technology Data Exchange (ETDEWEB)

    Nogueira, Luciana B., E-mail: lucibn19@yahoo.com.br, E-mail: jonymarques@uol.com.br [Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG (Brazil). Departamento de Anatomia e Imagem; Barsanelli, Cristiane; Geraldo, Jony M., E-mail: cbarsanelli@yahoo.com.br [Hospital Luxemburgo, Instituto Mário Penna, Belo Horizonte, MG (Brazil); Aquino, Jean Carlos; Campos, Tarcísio P. Ribeiro, E-mail: jeancarlosaquino@outlook.com, E-mail: tprcampos@yahoo.com.br [Universidade Federal de Minas Gerais (UGMG), Belo Horizonte, MG (Brazil). Departamento de Engenharia Nuclear

    2017-07-01

    The proposal of this study was to reproduce the dosimetry established in a treatment planning system (TPS) following a 3D conformational radiation therapy (3DCRT) protocol of two parallel-opposite fields applied to the left-breast in a thorax phantom, with the use of the field-in-field technique. Computed tomography (CT) images of the anthropomorphic and anthropometric phantom of the thorax with mobile breasts were generated. This phantom was developed by the NRI / UFMG research group. The generated images were transferred to the planning system XiO version-5 for the elaboration of the breast tele therapeutic planning with 2 Gy per fraction, in 25 fractions, with prescribed dose of 50 Gy. A set of ten EBT2 radiochromic films were irradiated at different doses. The values of RGB (Red, Green, Blue) of the radiochromic films were obtained by scanning and data transformed in optical density (OD), whose values were used to construct the calibration curve. EBT2 radiochromic films were positioned outside and inside of the thorax phantom: internally in the right and left lungs, on the face of the heart, in the glandular breast tissue-equivalent (TE) and in the left breast skin. After phantom radiation at the linear accelerator 6 MV Elekta Precise reproducing the 3DCRT, the radiochromic films were digitized after 24 h of exposure. The measurements of the intensities of the films in RGB were measured in the software ImageJ, transformed in optical density and converted in bidimensional dose distributions, applying the calibration curve. The experimental dosimetric data were analyzed and compared with values generated in the TPS. In addition, graphics and dose-volume histograms (DVH) were developed. The dose measurements in the glandular-TE in breast did not present statistically significant differences in relation to values at equivalent positions generated in the TPS. The organs at risk received doses below the reference values, according to TPS. It was verified the

  8. Dosimetric reproduction of a left-breast 3DCRT field-in-field radiation therapy planning in an anthropomorphic and anthropometric phantom

    International Nuclear Information System (INIS)

    Nogueira, Luciana B.; Aquino, Jean Carlos; Campos, Tarcísio P. Ribeiro

    2017-01-01

    The proposal of this study was to reproduce the dosimetry established in a treatment planning system (TPS) following a 3D conformational radiation therapy (3DCRT) protocol of two parallel-opposite fields applied to the left-breast in a thorax phantom, with the use of the field-in-field technique. Computed tomography (CT) images of the anthropomorphic and anthropometric phantom of the thorax with mobile breasts were generated. This phantom was developed by the NRI / UFMG research group. The generated images were transferred to the planning system XiO version-5 for the elaboration of the breast tele therapeutic planning with 2 Gy per fraction, in 25 fractions, with prescribed dose of 50 Gy. A set of ten EBT2 radiochromic films were irradiated at different doses. The values of RGB (Red, Green, Blue) of the radiochromic films were obtained by scanning and data transformed in optical density (OD), whose values were used to construct the calibration curve. EBT2 radiochromic films were positioned outside and inside of the thorax phantom: internally in the right and left lungs, on the face of the heart, in the glandular breast tissue-equivalent (TE) and in the left breast skin. After phantom radiation at the linear accelerator 6 MV Elekta Precise reproducing the 3DCRT, the radiochromic films were digitized after 24 h of exposure. The measurements of the intensities of the films in RGB were measured in the software ImageJ, transformed in optical density and converted in bidimensional dose distributions, applying the calibration curve. The experimental dosimetric data were analyzed and compared with values generated in the TPS. In addition, graphics and dose-volume histograms (DVH) were developed. The dose measurements in the glandular-TE in breast did not present statistically significant differences in relation to values at equivalent positions generated in the TPS. The organs at risk received doses below the reference values, according to TPS. It was verified the

  9. Left atrial systolic force in hypertensive patients with left ventricular hypertrophy: the LIFE study

    DEFF Research Database (Denmark)

    Chinali, M.; Simone, G. de; Wachtell, K.

    2008-01-01

    In hypertensive patients without prevalent cardiovascular disease, enhanced left atrial systolic force is associated with left ventricular hypertrophy and increased preload. It also predicts cardiovascular events in a population with high prevalence of obesity. Relations between left atrial...... systolic force and left ventricular geometry and function have not been investigated in high-risk hypertrophic hypertensive patients. Participants in the Losartan Intervention For Endpoint reduction in hypertension echocardiography substudy without prevalent cardiovascular disease or atrial fibrillation (n...... = 567) underwent standard Doppler echocardiography. Left atrial systolic force was obtained from the mitral orifice area and Doppler mitral peak A velocity. Patients were divided into groups with normal or increased left atrial systolic force (>14.33 kdyn). Left atrial systolic force was high in 297...

  10. Anatomic relationship between left coronary artery and left atrium in patients undergoing atrial fibrillation ablation.

    Science.gov (United States)

    Anselmino, Matteo; Torri, Federica; Ferraris, Federico; Calò, Leonardo; Castagno, Davide; Gili, Sebastiano; Rovera, Chiara; Giustetto, Carla; Gaita, Fiorenzo

    2017-07-01

    Atrial fibrillation transcatheter ablation (TCA) is, within available atrial fibrillation rhythm control strategies, one of the most effective. To potentially improve ablation outcome in case of recurrent atrial fibrillation after a first procedure or in presence of structural myocardial disease, isolation of the pulmonary veins may be associated with extensive lesions within the left atrium. To avoid rare, but potentially life-threatening, complications, thorough knowledge and assessment of left atrium anatomy and its relation to structures in close proximity are, therefore, mandatory. Aim of the present study is to describe, by cardiac computed tomography, the anatomic relationship between aortic root, left coronary artery and left atrium in patients undergoing atrial fibrillation TCA. The cardiac computed tomography scan of 21 patients affected by atrial fibrillation was elaborated to segment left atrium, aortic root and left coronary artery from the surrounding structures and the following distances measured: left atrium and aortic root; left atrium roof and aortic root; left main coronary artery and left atrium; circumflex artery and left atrium appendage; and circumflex artery and mitral valve annulus. Above all, the median distance between left atrium and aortic root (1.9, 1.5-2.1 mm), and between circumflex artery and left atrium appendage ostium (3.0, 2.1-3.4 mm) were minimal (≤3 mm). None of measured distances significantly varied between patients presenting paroxysmal versus persistent atrial fibrillation. The anatomic relationship between left atrium and coronary arteries is extremely relevant when performing atrial fibrillation TCA by extensive lesions. Therefore, at least in the latter case, preablation imaging should be recommended to avoid rare, but potentially life-threatening, complications with the aim of an as well tolerated as possible procedure.

  11. Introduction to left-right symmetric models

    International Nuclear Information System (INIS)

    Grimus, W.

    1993-01-01

    We motivate left-right symmetric models by the possibility of spontaneous parity breaking. Then we describe the multiplets and the Lagrangian of such models. Finally we discuss lower bounds on the right-handed scale. (author)

  12. Left ventricular hypertrophy: virtuous intentions, malign consequences.

    Science.gov (United States)

    Pokharel, Saraswati; Sharma, Umesh C; Pinto, Yigal M

    2003-06-01

    Left ventricular hypertrophy (LVH) is currently the focus of intense cardiovascular research, with the resultant rapid evolution of novel concepts relating to its exceedingly complex pathophysiology. In addition to the alterations in signal transduction and disturbances in Ca(2+) homeostasis, there are structural changes in myofilaments, disorganization of the cytoskeletal framework and increased collagen synthesis. LVH is associated with progressive left ventricular remodeling that culminates to heart failure. The modern treatment of left ventricular hypertrophy is now largely based on the hypothesis that neuroendocrine activation is important in the progression of the disease and inhibition of neurohormones is likely to have long-term benefit with regard to morbidity and mortality. Drugs specifically designed to unload the left ventricle, such as diuretics and vasodilators, appears to be less effective in reducing LV mass and improving prognosis. Thus, the evolution of treatment for LVH itself has provided much enlightenment for our understanding of the fundamental biology of the disorder.

  13. Quantum levitation by left-handed metamaterials

    Energy Technology Data Exchange (ETDEWEB)

    Leonhardt, Ulf; Philbin, Thomas G [School of Physics and Astronomy, University of St Andrews, North Haugh, St Andrews KY16 9SS (United Kingdom)

    2007-08-15

    Left-handed metamaterials make perfect lenses that image classical electromagnetic fields with significantly higher resolution than the diffraction limit. Here, we consider the quantum physics of such devices. We show that the Casimir force of two conducting plates may turn from attraction to repulsion if a perfect lens is sandwiched between them. For optical left-handed metamaterials, this repulsive force of the quantum vacuum may levitate ultra-thin mirrors.

  14. Quantum levitation by left-handed metamaterials

    International Nuclear Information System (INIS)

    Leonhardt, Ulf; Philbin, Thomas G

    2007-01-01

    Left-handed metamaterials make perfect lenses that image classical electromagnetic fields with significantly higher resolution than the diffraction limit. Here, we consider the quantum physics of such devices. We show that the Casimir force of two conducting plates may turn from attraction to repulsion if a perfect lens is sandwiched between them. For optical left-handed metamaterials, this repulsive force of the quantum vacuum may levitate ultra-thin mirrors

  15. Assessment of left ventricular global function

    International Nuclear Information System (INIS)

    Breuel, H.-P.; Baehre, M.

    1985-01-01

    Radionuclide ventriculography (RNV), i.e. noninvasive evaluation of left venticular performance following the application of radionuclides has had a major impact on many aspects of cardiology and has proven its clinical value and reliability in the last few years. This article deals mainly with the changes in global left ventricular function. The clinical applications of RNV for coronary artery diseases and valvular heart diseases are described. (Auth.)

  16. Aetiological factors in left-handedness

    Directory of Open Access Journals (Sweden)

    Milenković Sanja M.

    2005-01-01

    Full Text Available Lateralisation associates the extremities and senses of one side of the body, which are connected by afferent and efferent pathways, with the primary motor and sensory areas of the hemisphere on the opposite side. Dominant laterality denotes the appearance of a dominant extremity or sense in the performance of complex psychomotor activities. Laterality is manifested both as right-handedness or left-handedness, which are functionally equivalent and symmetrical in the performance of activities. Right-handedness is significantly more common than left-handedness. Genetic theory is most widely accepted in explaining the onset of lateralisation. According to this theory, the models of brain organisation asymmetry (anatomical, functional, and biochemical are strongly, genetically determined. However, the inability to clearly demonstrate the association between genetic factors and left-handedness has led researchers to investigate the effects of the environment on left-handedness. Of particular interest are the intrauterine environment and the factors influencing foetal development, of which hormones and ultrasound exposure are the most significant. It has been estimated that an extra five cases of nonright-handed lateralisation can be expected in every 100 males who were exposed to ultrasound in utero compared to those who were not. Socio-cultural pressure on left-handed individuals was much more severe in the past, which is confirmed by scientific findings that left-handedness is present in 13% of individuals in their twenties, while in less than 1% of individuals in their eighties.

  17. New strict left bundle branch block criteria reflect left ventricular activation differences

    DEFF Research Database (Denmark)

    Emerek, Kasper Janus Grønn; Risum, Niels; Hjortshøj, Søren Pihlkjær

    2015-01-01

    AIMS: Pacing lead electrical delays and strict left bundle branch block (LBBB) criteria were assessed against cardiac resynchronization therapy (CRT) outcome. METHODS: Forty-nine patients with LBBB and QRS duration >130 milliseconds underwent CRT-implantation. Sensed right ventricular to left ven....... CONCLUSION: Interventricular electrical delay predicts left ventricular remodeling after CRT and new, strict ECG criteria of LBBB are superior in predicting remodeling.......AIMS: Pacing lead electrical delays and strict left bundle branch block (LBBB) criteria were assessed against cardiac resynchronization therapy (CRT) outcome. METHODS: Forty-nine patients with LBBB and QRS duration >130 milliseconds underwent CRT-implantation. Sensed right ventricular to left...... ventricular electrical delay (RV-LV-IED) was measured. Response to CRT was defined as ≥15% decrease in left ventricular end-systolic volume. RESULTS: Eighteen of 20 (90%) patients with non-ischemic dilated cardiomyopathy (DCM) and 18 of 29 (62%) with ischemic heart disease (IHD) responded to CRT, p

  18. Focal dermal hypoplasia (Goltz-Gorlin syndrome): a new case with a novel variant in the PORCN gene (c.1250T>C:p.F417S) and unusual spinal anomaly.

    Science.gov (United States)

    Garavelli, Livia; Simonte, Graziella; Rosato, Simonetta; Wischmeijer, Anita; Albertini, Enrico; Guareschi, Elisa; Longo, Caterina; Albertini, Giuseppe; Gelmini, Chiara; Greco, Chiara; Errico, Stefania; Savino, Gustavo; Pavanello, Marco; Happle, Rudolf; Unger, Sheila; Superti-Furga, Andrea; Grzeschik, Karl-Heinz

    2013-07-01

    Focal dermal hypoplasia (FDH; Goltz-Gorlin syndrome; OMIM 305600) is a disorder that features involvement of the skin, skeletal system, and eyes. It is caused by loss-of-function mutations in the PORCN gene. We report a young girl with FDH, microphthalmos associated with colobomatous orbital cyst, dural ectasia and cystic malformation of the spinal cord, and a de novo variant in PORCN. This association has not been previously reported, and based on these observations the phenotypic spectrum of FDH might be broader than previously appreciated. It would be prudent to alter the suggested surveillance for this rare disorder. Copyright © 2013 Wiley Periodicals, Inc.

  19. Monophasic Synovial Sarcoma Presenting as Mitral Valve Obstruction

    OpenAIRE

    Chokesuwattanaskul, Warangkana; Terrell, Jason; Jenkins, Leigh Ann

    2010-01-01

    We report the case of a 26-year-old man who experienced progressive left-sided chest pain and 2 episodes of near-syncope. Studies revealed a 15-cm mass in the upper left lung, a 10-cm mass in the medial base of the left lung, and a 5-cm left atrial mass that involved the left lung, infiltrated the left pulmonary vein, and prolapsed into the mitral valve, causing intermittent obstruction. The patient underwent surgical excision of the left atrial tumor. Pathologic evaluation confirmed the diag...

  20. Mapping of the left-sided phrenic nerve course in patients undergoing left atrial catheter ablations.

    Science.gov (United States)

    Huemer, Martin; Wutzler, Alexander; Parwani, Abdul S; Attanasio, Philipp; Haverkamp, Wilhelm; Boldt, Leif-Hendrik

    2014-09-01

    Catheter ablation of atrial fibrillation has been associated with left-sided phrenic nerve palsy. Knowledge of the individual left phrenic nerve course therefore is essential to prevent nerve injury. The aim of this study was to test the feasibility of an intraprocedural pace mapping and reconstruction of the left phrenic nerve course and to characterize which anatomical areas are affected. In patients undergoing left atrial catheter ablation, a three-dimensional map of the left atrial anatomical structures was created. The left-sided phrenic nerve course was determined by high-output pace mapping and reconstructed in the map. In this study, 40 patients with atrial fibrillation or atrial tachycardias were included. Left phrenic nerve capture was observed in 23 (57.5%) patients. Phrenic nerve was captured in 22 (55%) patients inside the left atrial appendage, in 22 (55%) in distal parts, in 21 (53%) in medial parts, and in two (5%) in ostial parts of the appendage. In three (7.5%) patients, capture was found in the distal coronary sinus and in one (2.5%) patient in the left atrium near the left atrial appendage ostium. Ablation target was changed due to direct spatial relationship to the phrenic nerve in three (7.5%) patients. No phrenic nerve palsy was observed. Left-sided phrenic nerve capture was found inside and around the left atrial appendage in the majority of patients and additionally in the distal coronary sinus. Phrenic nerve mapping and reconstruction can easily be performed and should be considered prior catheter ablations in potential affected areas. ©2014 Wiley Periodicals, Inc.

  1. Screening of TYR, OCA2, GPR143, and MC1R in patients with congenital nystagmus, macular hypoplasia, and fundus hypopigmentation indicating albinism

    Science.gov (United States)

    Preising, Markus N.; Gonser, Miriam; Lorenz, Birgit

    2011-01-01

    Background A broad spectrum of pigmentation of the skin and hair is found among patients diagnosed with ocular albinism (OA) and oculocutaneous albinism (OCA). Even though complexion is variable, three ocular features, i.e., hypopigmentation of the fundus, hypoplasia of the macula, and nystagmus, are classical pathological findings in these patients. We screened 172 index patients with a clinical diagnosis of OA or OCA based on the classical findings, to evaluate the frequency of sequence variants in tyrosinase (TYR), P-gene, P-protein (OCA2), and the G-protein-coupled receptor 143 gene, OA1 (GPR143). In addition, we investigated the association of sequence variants in the melanocortin receptor 1 gene (MC1R) and OCA2. Methods Pigmentation of the hair, skin, iris, and fundus were included in the evaluation of OCA and OA. Male OA patients showing X-linked inheritance were screened for GPR143. Females showing OA without family history were regarded as representing autosomal recessive OA (OA3). Direct sequencing was applied to PCR products showing aberrant single-strand conformation polymorphism–banding patterns. Results Fifty-seven male index patients were screened for OA. We identified 16 potentially pathogenic sequence variations in GPR143 (10 novel) in 22 males. In TYR, we identified 23 (7 novel), and in OCA2 28 (11 novel) possibly pathogenic variants. Variants on both alleles were identified in TYR or OCA2 in 29/79 OCA patients and 14/71 OA patients. Sequence changes in TYR were identified almost exclusively in OCA patients, while sequence changes in OCA2 occurred in OCA and OA patients. MC1R sequencing was performed in 47 patients carrying mutations in OCA2 and revealed MC1R mutations in 42 of them. Conclusions TYR gene mutations have a more severe effect on pigmentation than mutations in OCA2 and the GPR143 gene. Nevertheless, mutations in these genes affect the development of visual function either directly or by interaction with other genes like MC1R, which

  2. Both rare and de novo copy number variants are prevalent in agenesis of the corpus callosum but not in cerebellar hypoplasia or polymicrogyria.

    Directory of Open Access Journals (Sweden)

    Samin A Sajan

    Full Text Available Agenesis of the corpus callosum (ACC, cerebellar hypoplasia (CBLH, and polymicrogyria (PMG are severe congenital brain malformations with largely undiscovered causes. We conducted a large-scale chromosomal copy number variation (CNV discovery effort in 255 ACC, 220 CBLH, and 147 PMG patients, and 2,349 controls. Compared to controls, significantly more ACC, but unexpectedly not CBLH or PMG patients, had rare genic CNVs over one megabase (p = 1.48×10⁻³; odds ratio [OR] = 3.19; 95% confidence interval [CI] = 1.89-5.39. Rare genic CNVs were those that impacted at least one gene in less than 1% of the combined population of patients and controls. Compared to controls, significantly more ACC but not CBLH or PMG patients had rare CNVs impacting over 20 genes (p = 0.01; OR = 2.95; 95% CI = 1.69-5.18. Independent qPCR confirmation showed that 9.4% of ACC patients had de novo CNVs. These, in comparison to inherited CNVs, preferentially overlapped de novo CNVs previously observed in patients with autism spectrum disorders (p = 3.06×10⁻⁴; OR = 7.55; 95% CI = 2.40-23.72. Interestingly, numerous reports have shown a reduced corpus callosum area in autistic patients, and diminished social and executive function in many ACC patients. We also confirmed and refined previously known CNVs, including significantly narrowing the 8p23.1-p11.1 duplication present in 2% of our current ACC cohort. We found six novel CNVs, each in a single patient, that are likely deleterious: deletions of 1p31.3-p31.1, 1q31.2-q31.3, 5q23.1, and 15q11.2-q13.1; and duplications of 2q11.2-q13 and 11p14.3-p14.2. One ACC patient with microcephaly had a paternally inherited deletion of 16p13.11 that included NDE1. Exome sequencing identified a recessive maternally inherited nonsense mutation in the non-deleted allele of NDE1, revealing the complexity of ACC genetics. This is the first systematic study of CNVs in congenital brain malformations, and

  3. Hypothesis for the causes and periodicity of repetitive linear enamel hypoplasia in large, wild African (Pan troglodytes and Gorilla gorilla) and Asian (Pongo pygmaeus) apes.

    Science.gov (United States)

    Skinner, Mark F; Hopwood, David

    2004-03-01

    Repetitive linear enamel hypoplasia (rLEH) is often observed in recent large-bodied apes from Africa and Asia as well as Mid- to Late Miocene sites from Spain to China. The ubiquity and periodicity of rLEH are not understood. Its potential as an ontogenetic marker of developmental stress in threatened species (as well as their ancient relatives) makes rLEH an important if enigmatic problem. We report research designed to show the periodicity of rLEH among West African Pan troglodytes (12 male, 32 female), Gorilla gorilla (10 male, 10 female), and Bornean and Sumatran Pongo pygmaeus (11 male, 9 female, 9 unknown) from collections in Europe. Two methods were employed. In the common chimpanzees and gorillas, the space between adjacent, macroscopically visible LEH grooves on teeth with two or more episodes was expressed as an absolute measure and as a ratio of complete unworn crown height. In the orangutans, the number of perikymata between episode onsets, as well as duration of rLEH, was determined from scanning electron micrographs of casts of incisors and canines. We conclude that stress in the form of LEH commences as early as 2.5 years of age in all taxa and lasts for several years, and even longer in orangutans; the stress is not chronic but episodic; the stressor has a strong tendency to occur in pulses of two occurrences each; and large apes from both land masses exhibit rLEH with an average periodicity of 6 months (or multiples thereof; Sumatran orangutans seem to show only annual stress), but this needs further research. This is supported by evidence of spacing between rLEH as well as perikymata counts. Duration of stress in orangutans averages about 6 weeks. Finally, the semiannual stressor transcends geographic and temporal boundaries, and is attributed to regular moisture cycles associated with the intertropical convergence zone modified by the monsoon. While seasonal cycles can influence both disease and nutritional stress, it is likely the combination of

  4. What is Beyond Right/Left?

    DEFF Research Database (Denmark)

    Dyrberg, Torben Bech

    2009-01-01

    The article looks at New Labour's move beyond right/left in the mid/late 1990s, which is an occasion to spell out the nature of right/left and what it means for democracy. In contrast to both defenders and critics of this move I argue in the first part that right/left is not an empty label bound up...... with the cleavage-lines of industrial society, but that it is an orientational metaphor which is articulated with others-in/out, up/down and front/back-and that we are in the process of moving beyond the industrial society right/left prototype. The second part looks at where New Labour is heading when moving beyond...... right/left. I argue that the new hegemonic orientation is that of front/back, which designs political renewal as a response to the social changes cutting across the outdated lines of contestation of partisan politics. The democratic problem of this move lies in squeezing politics between technocratic...

  5. Left Right Patterning, Evolution and Cardiac Development

    Science.gov (United States)

    Dykes, Iain M.

    2018-01-01

    Many aspects of heart development are determined by the left right axis and as a result several congenital diseases have their origins in aberrant left-right patterning. Establishment of this axis occurs early in embryogenesis before formation of the linear heart tube yet impacts upon much later morphogenetic events. In this review I discuss the differing mechanisms by which left-right polarity is achieved in the mouse and chick embryos and comment on the evolution of this system. I then discus three major classes of cardiovascular defect associated with aberrant left-right patterning seen in mouse mutants and human disease. I describe phenotypes associated with the determination of atrial identity and venous connections, looping morphogenesis of the heart tube and finally the asymmetric remodelling of the embryonic branchial arch arterial system to form the leftward looped arch of aorta and associated great arteries. Where appropriate, I consider left right patterning defects from an evolutionary perspective, demonstrating how developmental processes have been modified in species over time and illustrating how comparative embryology can aide in our understanding of congenital heart disease. PMID:29755990

  6. Grammatical distinctions in the left frontal cortex.

    Science.gov (United States)

    Shapiro, K A; Pascual-Leone, A; Mottaghy, F M; Gangitano, M; Caramazza, A

    2001-08-15

    Selective deficits in producing verbs relative to nouns in speech are well documented in neuropsychology and have been associated with left hemisphere frontal cortical lesions resulting from stroke and other neurological disorders. The basis for these impairments is unresolved: Do they arise because of differences in the way grammatical categories of words are organized in the brain, or because of differences in the neural representation of actions and objects? We used repetitive transcranial magnetic stimulation (rTMS) to suppress the excitability of a portion of left prefrontal cortex and to assess its role in producing nouns and verbs. In one experiment subjects generated real words; in a second, they produced pseudowords as nouns or verbs. In both experiments, response latencies increased for verbs but were unaffected for nouns following rTMS. These results demonstrate that grammatical categories have a neuroanatomical basis and that the left prefrontal cortex is selectively engaged in processing verbs as grammatical objects.

  7. Left-Wing Extremism: The Current Threat

    Energy Technology Data Exchange (ETDEWEB)

    Karl A. Seger

    2001-04-30

    Left-wing extremism is ''alive and well'' both in the US and internationally. Although the current domestic terrorist threat within the U. S. is focused on right-wing extremists, left-wing extremists are also active and have several objectives. Leftist extremists also pose an espionage threat to U.S. interests. While the threat to the U.S. government from leftist extremists has decreased in the past decade, it has not disappeared. There are individuals and organizations within the U.S. who maintain the same ideology that resulted in the growth of left-wing terrorism in this country in the 1970s and 1980s. Some of the leaders from that era are still communicating from Cuba with their followers in the U.S., and new leaders and groups are emerging.

  8. Clustered survival data with left-truncation

    DEFF Research Database (Denmark)

    Eriksson, Frank; Martinussen, Torben; Scheike, Thomas H.

    2015-01-01

    Left-truncation occurs frequently in survival studies, and it is well known how to deal with this for univariate survival times. However, there are few results on how to estimate dependence parameters and regression effects in semiparametric models for clustered survival data with delayed entry....... Surprisingly, existing methods only deal with special cases. In this paper, we clarify different kinds of left-truncation and suggest estimators for semiparametric survival models under specific truncation schemes. The large-sample properties of the estimators are established. Small-sample properties...

  9. Left ventricular function in right ventricular overload

    International Nuclear Information System (INIS)

    Iwanaga, Shiro; Handa, Shunnosuke; Abe, Sumihisa; Onishi, Shohei; Nakamura, Yoshiro; Kunieda, Etsuo; Ogawa, Koichi; Kubo, Atsushi

    1989-01-01

    This study clarified regional and global functions of the distorted left ventricle due to right ventricular overload by gated radionuclide ventriculography (RNV). Cardiac catheterization and RNV were performed in 13 cases of atrial septal defect (ASD), 13 of pure mitral stenosis (MS), 10 of primary pulmonary hypertension (PPH), and 10 of normal subjects (NL). Right ventricular systolic pressure (RVSP) was 32.9±13.9, 45.0±12.2, 88.3±17.1, and 21.2±4.5 mmHg, respectively. The end-systolic LAO view of the left ventricle was halved into septal and free-wall sides. The end-diastolic halves were determined in the same plane. Ejection fractions of the global left ventricle (LVEF), global right ventricle (RVEF), the septal half of the left ventricle (SEPEF), and the free-wall half of the left ventricle (FWEF) were obtained. LVEF was 56.8±9.8% in NL, 52.8±10.5% in ASD, and 49.5±12.9% in PPH. In MS, LVEF (47.0±13.0%) was smaller than those in the other groups. RVEF was 37.0±5.2% in NL, 43.7±15.5% in ASD, and 32.8±11.5% in MS. In PPH, RVEF (25.0±10.6%) was smaller than those in the other groups. SEPEF was smaller in ASD (42.5±13.2%), MS (40.4±13.1%), PPH (40.5±12.5%) than in NL (53.5±8.5%). Systolic function of the septal half of the left ventricle was disturbed by right ventricular overload. RVEF (r=-0.35, p<0.05) and SEPEF (r=-0.51, p<0.01) had negative correlations with RVSP. As RVSP rose, systolic function of the septal half of the left ventricle was more severely disturbed. FWEF was the same among the four groups; NL (57.0±12.6%), ASD (48.6±15.2%), MS (50.5±12.0%), and PPH (51.1±12.3%). There was a good correlation between SEPEF and LVEF in NL (r=0.81), although in PPH this correlation was poor (r=0.64). These data showed that the distorted left ventricular due to right ventricular overload maintains its global function with preserved function of the free-wall side. (J.P.N.)

  10. Left ventricular performance during psychological stress

    International Nuclear Information System (INIS)

    Young, D.Z.; Massachusetts General Hospital, Boston; Dimsdale, J.E.; Moore, R.H.; Barlai-Kovach, M.; Newell, J.B.; McKusick, K.A.; Boucher, C.A.; Fifer, M.A.; Strauss, H.W.

    1989-01-01

    Left ventricular ejection fraction, systolic blood pressure and plasma norepinephrine were measured in six normotensive and six mildly hypertensive subjects during rest and psychological stress. Compared with rest, 8 of the 12 subjects developed significant changes in ejection fraction (increase in 6, decrease in 2); 10 of 12 subjects developed significant elevations of plasma norepinephrine; and all developed significant increases in systolic blood pressure. When the stress effects were examined for the total group, as opposed to within subjects, there were significant increases in plasma norepinephrine and systolic blood pressure but, interestingly, mean ejection fraction and stroke volume remained unchanged, implying stress led to increased left ventricular contractility. (orig.)

  11. Aortic atresia with normal sized left ventricle

    Directory of Open Access Journals (Sweden)

    Priya Jagia

    2016-01-01

    Full Text Available Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best of our knowledge, use of multi-detector computed tomography in aortic atresia with well developed left ventricle has not been reported in literature till date.

  12. Left brain, right brain: facts and fantasies.

    Science.gov (United States)

    Corballis, Michael C

    2014-01-01

    Handedness and brain asymmetry are widely regarded as unique to humans, and associated with complementary functions such as a left-brain specialization for language and logic and a right-brain specialization for creativity and intuition. In fact, asymmetries are widespread among animals, and support the gradual evolution of asymmetrical functions such as language and tool use. Handedness and brain asymmetry are inborn and under partial genetic control, although the gene or genes responsible are not well established. Cognitive and emotional difficulties are sometimes associated with departures from the "norm" of right-handedness and left-brain language dominance, more often with the absence of these asymmetries than their reversal.

  13. Echocardiographic study of left atrial myxoma

    Directory of Open Access Journals (Sweden)

    Dalal J

    1979-01-01

    Full Text Available Four cases of left atrial myxoma were diagnosed pre-operatively by echocardiography. All cases showed characteristic echocardio-graphic features of variegated shadows behind the mitral valve in diastole and within the left atrium in systole. In two cases the my-xomas were surgically removed and confirmed on histology. In one case the post-operative echocardiogram showed complete dis-appearance of the abnormal shadows. Echocardiography is the most reliable method today for the diagnosis of a myxoma.

  14. Left ventricular assist device implantation via left thoracotomy: alternative to repeat sternotomy.

    Science.gov (United States)

    Pierson, Richard N; Howser, Renee; Donaldson, Terri; Merrill, Walter H; Dignan, Rebecca J; Drinkwater, Davis C; Christian, Karla G; Butler, Javed; Chomsky, Don; Wilson, John R; Clark, Rick; Davis, Stacy F

    2002-03-01

    Repeat sternotomy for left ventricular assist device insertion may result in injury to the right heart or patent coronary grafts, complicating intraoperative and postoperative management. In 4 critically ill patients, left thoracotomy was used as an alternative to repeat sternotomy. Anastomosis of the outflow conduit to the descending thoracic aorta provided satisfactory hemodynamic support.

  15. Left-forbidding cooperating distributed grammar systems

    Czech Academy of Sciences Publication Activity Database

    Goldefus, F.; Masopust, Tomáš; Meduna, A.

    2010-01-01

    Roč. 411, 40-42 (2010), s. 3661-3667 ISSN 0304-3975 Institutional research plan: CEZ:AV0Z10190503 Keywords : cooperating distributed grammar system * cooperating derivation mode * left-forbidding grammar * generative power * descriptional complexity Subject RIV: BA - General Mathematics Impact factor: 0.838, year: 2010 http://www.sciencedirect.com/science/article/pii/S0304397510003440

  16. Magnetization of left-handed metamaterials

    International Nuclear Information System (INIS)

    Kourakis, I; Shukla, P K

    2006-01-01

    We propose a possible mechanism for the generation of magnetic fields in negative refraction index composite metamaterials. Considering the propagation of a high-frequency modulated amplitude electric field in a left-handed material (LHM), we show that the ponderomotive interaction between the field and low-frequency potential distributions leads to spontaneous generation of magnetic fields, whose form and properties are discussed

  17. Beware the left-sided gallbladder

    African Journals Online (AJOL)

    An abdominal ultrasound scan showed multiple stones in the gallbladder with no evidence of acute cholecystitis and a common bile duct (CBD) diameter of 4 mm. Owing to the atypical nature of her pain, a computed tomography (CT) scan was requested which showed a left-sided gallbladder containing gallstones (Fig. 1).

  18. Laughter-induced left bundle branch block.

    Science.gov (United States)

    Chow, Grant V; Desai, Dipan; Spragg, David D; Zakaria, Sammy

    2012-10-01

    We present the case of a patient with ischemic heart disease and intermittent left bundle branch block, reproducibly induced by laughter. Following treatment of ischemia with successful deployment of a drug-eluting stent, no further episodes of inducible LBBB were seen. Transient ischemia, exacerbated by elevated intrathoracic pressure during laughter, may have contributed to onset of this phenomenon. © 2012 Wiley Periodicals, Inc.

  19. Left-right subtraction of brain CT

    International Nuclear Information System (INIS)

    Ishiguchi, Tsuneo; Sakuma, Sadayuki

    1986-01-01

    A new image-processing method to obtain a left-right subtraction image of CT was designed for the automated detection of abnormalities in brain CT. An original CT image was divided in two by a centerline. Then the right half of the image was subtracted from the left half by calculating the absorption value of the pixels on the symmetrical positions against the centerline. The mean and the standard deviation of the absorption value of the pixels in the subtraction image were used as parameters for analysis, and the detectability of abnormal CT findings was evaluated in 100 cases - 50 cases each with normal and abnormal CT. The presence of abnormalities could be diagnosed with a sensitivity of 86 %, a specificity of 90 %, and an overall accuracy of 88 % when the borderline of these parameters between normal and abnormal CT was set at the mean + 2SD in the normal group. As a further analysis, the CT image was subdivided into several areas from a functional or anatomical viewpoint, such as cerebral vascular territories, and the left-right subtraction image of each area was obtained. The possibilities of diagnosing the location of an abnormality and of detecting smaller lesions with this method were shown. Left-right subtraction was considered to be a useful method for the detection of asymmetric abnormalities in the automated diagnosis of brain CT. (author)

  20. Left ventricular hypertrophy : virtuous intentions, malign consequences

    NARCIS (Netherlands)

    Pokharel, S; Sharma, UC; Pinto, YM

    Left ventricular hypertrophy (LVH) is currently the focus of intense cardiovascular research, with the resultant rapid evolution of novel concepts relating to its exceedingly complex pathophysiology. In addition to the alterations in signal transduction and disturbances in Ca2+ homeostasis, there

  1. Left ventricular hypertrophy: virtuous intentions, malign consequences

    NARCIS (Netherlands)

    Pokharel, Saraswati; Sharma, Umesh C.; Pinto, Yigal M.

    2003-01-01

    Left ventricular hypertrophy (LVH) is currently the focus of intense cardiovascular research, with the resultant rapid evolution of novel concepts relating to its exceedingly complex pathophysiology. In addition to the alterations in signal transduction and disturbances in Ca(2+) homeostasis, there

  2. Left-sided portal hypertension revisited

    Directory of Open Access Journals (Sweden)

    Antonio Manenti

    2016-12-01

    Conclusions: In every case of left-sided portal hypertension, upper digestive endoscopy and close follow-up are recommended. Besides, computed tomography can demonstrate particular conditions directly favoring gastroesophageal varices, and aid in selection of the appropriate therapeutic decisions. [Arch Clin Exp Surg 2016; 5(4.000: 211-215

  3. Scimitar syndrome of atypical, rare drainage of venous vessel to the superior vena cava. A case report

    International Nuclear Information System (INIS)

    Sybilski, Adam J.; Michalczuk, Małgorzata; Chudoba, Anna; Tolak-Omernik, Katarzyna; Bulski, Tomasz; Walecki, Jerzy

    2013-01-01

    Scimitar syndrome is a rare and complex congenital anomaly characterized by partial or complete anomalous pulmonary venous return from the right or left lung into the inferior vena cava, through drainage into the hepatic vein, right atrium or left atrium. The syndrome is commonly associated with hypoplasia of the right lung and right pulmonary artery. We present an 11-year-old female with atypical and rare type of scimitar syndrome. The girl has had cough for 2 months before admission, without fever or abnormalities on medical examination. X-ray films revealed inflammatory and atelectatic changes with mediastinal shift to the right. CT and CT angiography – hypoplasia of the right lung with no visible interlobar fissures. No areas of consolidation in the pulmonary parenchyma. Mediastinum shifted to the right. Single wide venous vessels draining the upper part of the right lung entering the superior vena cava. In our patient, clinical symptoms are mild, but a thorough physical examination could have helped diagnose the syndrome earlier

  4. Left ventricular function in chronic aortic regurgitation

    International Nuclear Information System (INIS)

    Iskandrian, A.S.; Hakki, A.H.; Manno, B.; Amenta, A.; Kane, S.A.

    1983-01-01

    Left ventricular performance was determined in 42 patients with moderate or severe aortic regurgitation during upright exercise by measuring left ventricular ejection fraction and volume with radionuclide ventriculography. Classification of the patients according to exercise tolerance showed that patients with normal exercise tolerance (greater than or equal to 7.0 minutes) had a significantly higher ejection fraction at rest (probability [p] . 0.02) and during exercise (p . 0.0002), higher cardiac index at exercise (p . 0.0008) and lower exercise end-systolic volume (p . 0.01) than did patients with limited exercise tolerance. Similar significant differences were noted in younger patients compared with older patients in ejection fraction at rest and exercise (both p . 0.001) and cardiac index at rest (p . 0.03) and exercise (p . 0.0005). The end-diastolic volume decreased during exercise in 60% of the patients. The patients with a decrease in volume were significantly younger and had better exercise tolerance and a larger end-diastolic volume at rest than did patients who showed an increase in volume. The mean corrected left ventricular end-diastolic radius/wall thickness ratio was significantly greater in patients with abnormal than in those with normal exercise reserve (mean +/- standard deviation 476 +/- 146 versus 377 +/- 92 mm Hg, p less than 0.05). Thus, in patients with chronic aortic regurgitation: 1) left ventricular systolic function during exercise was related to age, exercise tolerance and corrected left ventricular end-diastolic radius/wall thickness ratio, and 2) the end-diastolic volume decreased during exercise, especially in younger patients and patients with normal exercise tolerance or a large volume at rest

  5. Left bronchial artery arising from a replaced left hepatic artery in a patient with massive hemoptysis

    Energy Technology Data Exchange (ETDEWEB)

    Khil, Eun Kyung; Lee, Jae Myung [Dept. of Radiology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon (Korea, Republic of)

    2015-09-15

    A 70-year-old man with a 3-year history of bronchiectasis presented with massive hemoptysis that had lasted for 3 days. In our attempt to perform bronchial artery embolization, upper abdominal angiography was required to locate the left bronchial artery, which in this case was of anomalous origin, arising from a replaced left hepatic artery, which arose from the left gastric artery-a very unusual anatomical variant. We performed embolization with polyvinyl alcohol particles, and the patient's symptoms resolved completely, with no additional complications after conservative treatment.

  6. Evaluation of left ventricular function by cardiac CT

    International Nuclear Information System (INIS)

    Naito, Hiroaki; Kozuka, Takahiro

    1982-01-01

    Left ventricular function was evaluated by CT, which was compared with the data of left ventriculography for various cardiac diseases. The end diastolic volume of the left ventricle can be readily computed from CT, with a satisfactory correlation with that of left ventriculography (r = 0.95). The left ventricular ejection fraction, calculated from the areal ratio of the left ventricular lumen in end-diastolic imaging to that in end-sytolic imaging, also roughly reflects left ventricular contractile function, but shows correlation with left ventriculography by only r = 0.79. Although the cardiac output is not sensitive for functional evaluation, it can be directly calculated by means of dynamic scanning and shows a satisfactory correlation with the ear piece pigment dilution (r = 0.85). Evaluation of left ventricular function by CT shows a high precision in comparison with left ventriculography, but still lacks temporal resolving power. (Chiba, N.)

  7. Dyscalculia, Dysgraphia, and Left-Right Confusion from a Left Posterior Peri-Insular Infarct

    Directory of Open Access Journals (Sweden)

    S. Bhattacharyya

    2014-01-01

    Full Text Available The Gerstmann syndrome of dyscalculia, dysgraphia, left-right confusion, and finger agnosia is generally attributed to lesions near the angular gyrus of the dominant hemisphere. A 68-year-old right-handed woman presented with sudden difficulty completing a Sudoku grid and was found to have dyscalculia, dysgraphia, and left-right confusion. Magnetic resonance imaging (MRI showed a focus of abnormal reduced diffusivity in the left posterior insula and temporoparietal operculum consistent with acute infarct. Gerstmann syndrome from an insular or peri-insular lesion has not been described in the literature previously. Pathological and functional imaging studies show connections between left posterior insular region and inferior parietal lobe. We postulate that the insula and operculum lesion disrupted key functional networks resulting in a pseudoparietal presentation.

  8. Dyscalculia, dysgraphia, and left-right confusion from a left posterior peri-insular infarct.

    Science.gov (United States)

    Bhattacharyya, S; Cai, X; Klein, J P

    2014-01-01

    The Gerstmann syndrome of dyscalculia, dysgraphia, left-right confusion, and finger agnosia is generally attributed to lesions near the angular gyrus of the dominant hemisphere. A 68-year-old right-handed woman presented with sudden difficulty completing a Sudoku grid and was found to have dyscalculia, dysgraphia, and left-right confusion. Magnetic resonance imaging (MRI) showed a focus of abnormal reduced diffusivity in the left posterior insula and temporoparietal operculum consistent with acute infarct. Gerstmann syndrome from an insular or peri-insular lesion has not been described in the literature previously. Pathological and functional imaging studies show connections between left posterior insular region and inferior parietal lobe. We postulate that the insula and operculum lesion disrupted key functional networks resulting in a pseudoparietal presentation.

  9. Minimal Left-Right Symmetric Dark Matter.

    Science.gov (United States)

    Heeck, Julian; Patra, Sudhanwa

    2015-09-18

    We show that left-right symmetric models can easily accommodate stable TeV-scale dark matter particles without the need for an ad hoc stabilizing symmetry. The stability of a newly introduced multiplet either arises accidentally as in the minimal dark matter framework or comes courtesy of the remaining unbroken Z_{2} subgroup of B-L. Only one new parameter is introduced: the mass of the new multiplet. As minimal examples, we study left-right fermion triplets and quintuplets and show that they can form viable two-component dark matter. This approach is, in particular, valid for SU(2)×SU(2)×U(1) models that explain the recent diboson excess at ATLAS in terms of a new charged gauge boson of mass 2 TeV.

  10. A constrained supersymmetric left-right model

    Energy Technology Data Exchange (ETDEWEB)

    Hirsch, Martin [AHEP Group, Instituto de Física Corpuscular - C.S.I.C./Universitat de València, Edificio de Institutos de Paterna, Apartado 22085, E-46071 València (Spain); Krauss, Manuel E. [Bethe Center for Theoretical Physics & Physikalisches Institut der Universität Bonn, Nussallee 12, 53115 Bonn (Germany); Institut für Theoretische Physik und Astronomie, Universität Würzburg,Emil-Hilb-Weg 22, 97074 Wuerzburg (Germany); Opferkuch, Toby [Bethe Center for Theoretical Physics & Physikalisches Institut der Universität Bonn, Nussallee 12, 53115 Bonn (Germany); Porod, Werner [Institut für Theoretische Physik und Astronomie, Universität Würzburg,Emil-Hilb-Weg 22, 97074 Wuerzburg (Germany); Staub, Florian [Theory Division, CERN,1211 Geneva 23 (Switzerland)

    2016-03-02

    We present a supersymmetric left-right model which predicts gauge coupling unification close to the string scale and extra vector bosons at the TeV scale. The subtleties in constructing a model which is in agreement with the measured quark masses and mixing for such a low left-right breaking scale are discussed. It is shown that in the constrained version of this model radiative breaking of the gauge symmetries is possible and a SM-like Higgs is obtained. Additional CP-even scalars of a similar mass or even much lighter are possible. The expected mass hierarchies for the supersymmetric states differ clearly from those of the constrained MSSM. In particular, the lightest down-type squark, which is a mixture of the sbottom and extra vector-like states, is always lighter than the stop. We also comment on the model’s capability to explain current anomalies observed at the LHC.

  11. Left brain, right brain: facts and fantasies.

    Directory of Open Access Journals (Sweden)

    Michael C Corballis

    2014-01-01

    Full Text Available Handedness and brain asymmetry are widely regarded as unique to humans, and associated with complementary functions such as a left-brain specialization for language and logic and a right-brain specialization for creativity and intuition. In fact, asymmetries are widespread among animals, and support the gradual evolution of asymmetrical functions such as language and tool use. Handedness and brain asymmetry are inborn and under partial genetic control, although the gene or genes responsible are not well established. Cognitive and emotional difficulties are sometimes associated with departures from the "norm" of right-handedness and left-brain language dominance, more often with the absence of these asymmetries than their reversal.

  12. A disappearing left-sided neck mass.

    Science.gov (United States)

    Lutwak, Nancy; Dill, Curt

    2012-07-04

    The patient was a 48-year-old man who presented to the emergency department with complaints of a left-sided painful neck mass, which changed in size relative to ingestion of meals. He denied voice change, fever, chills, weight loss, dysphagia and hoarseness. Physical examination was unremarkable. CT scan demonstrated a 3.9 mm calculus of the submandibular gland duct. Therapeutic sialendocopy was successfully performed.

  13. The New Arab Left and 1967

    DEFF Research Database (Denmark)

    Haugbølle, Sune

    2017-01-01

    In Arab political culture, the Naksa of 1967 had a number of watershed effects. Scholars have paid a lot of attention to the decline of secular Arab nationalism and the concurrent rise of Islamism. Much less research has been done on the way 1967 spurred radical left organizations, also known as ...... moment that followed. This moment has had a lasting impact on Arab political culture and is being re-interpreted in interesting ways today by Arab revolutionaries post-2011....

  14. Aortic atresia with normal sized left ventricle

    OpenAIRE

    Priya Jagia; Arun Sharma; Saurabh K Gupta; Munish Guleria

    2016-01-01

    Aortic atresia with an associated ventricular septal defect and adequate sized left ventricle is extremely rare. We present two cases in which an alternate diagnosis was suggested on echocardiography because the hypoplastic aortic trunk was missed due to its small caliber. The final diagnosis was, however, clinched on dual source computed tomography, which not only showed the thin aortic trunk but also clearly depicted the coronary artery origins from the hypoplastic aortic root. To the best ...

  15. Total agenesis of the left pericardium

    Directory of Open Access Journals (Sweden)

    J. Glauco Lobo Fº

    1999-09-01

    Full Text Available This is the report of a 46-year-old patient with the preoperative diagnosis of an atrial septal defect (ASD of the ostium secudum type. After sternectomy, partial agenesis of the left pericardium was diagnosed. It is our opinion that, if the radiographic picture is suggestive of this entity, a clinical search for cardiopulmonary anomalies should be performed, because the majority of these associated anomalies can and should be surgically corrected.

  16. Clustering Of Left Ventricular Wall Motion Patterns

    Science.gov (United States)

    Bjelogrlic, Z.; Jakopin, J.; Gyergyek, L.

    1982-11-01

    A method for detection of wall regions with similar motion was presented. A model based on local direction information was used to measure the left ventricular wall motion from cineangiographic sequence. Three time functions were used to define segmental motion patterns: distance of a ventricular contour segment from the mean contour, the velocity of a segment and its acceleration. Motion patterns were clustered by the UPGMA algorithm and by an algorithm based on K-nearest neighboor classification rule.

  17. Implantation of a HeartMate II left ventricular assist device via left thoracotomy.

    Science.gov (United States)

    Cho, Yang Hyun; Deo, Salil V; Schirger, John A; Pereira, Naveen L; Stulak, John M; Park, Soon J

    2012-11-01

    Left thoracotomy was used as an approach for the implantation of pulsatile ventricular assist devices. Avoiding the standard approach of median sternotomy is attractive in patients undergoing complicated redo cardiac surgery, especially with prior mediastinal radiation. We report a case of the use of left thoracotomy for the implantation of the HeartMate II axial-flow pump. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  18. Left hemisphere regions are critical for language in the face of early left focal brain injury

    OpenAIRE

    Raja Beharelle, Anjali; Dick, Anthony Steven; Josse, Goulven; Solodkin, Ana; Huttenlocher, Peter R.; Levine, Susan C.; Small, Steven L.

    2010-01-01

    A predominant theory regarding early stroke and its effect on language development, is that early left hemisphere lesions trigger compensatory processes that allow the right hemisphere to assume dominant language functions, and this is thought to underlie the near normal language development observed after early stroke. To test this theory, we used functional magnetic resonance imaging to examine brain activity during category fluency in participants who had sustained pre- or perinatal left h...

  19. Pulmonary hypertension due to left heart disease.

    Science.gov (United States)

    Berthelot, Emmanuelle; Bailly, Minh Tam; Hatimi, Safwane El; Robard, Ingrid; Rezgui, Hatem; Bouchachi, Amir; Montani, David; Sitbon, Olivier; Chemla, Denis; Assayag, Patrick

    Pulmonary hypertension due to left heart disease, also known as group 2 pulmonary hypertension according to the European Society of Cardiology/European Respiratory Society classification, is the most common cause of pulmonary hypertension. In patients with left heart disease, the development of pulmonary hypertension favours right heart dysfunction, which has a major impact on disease severity and outcome. Over the past few years, this condition has been considered more frequently. However, epidemiological studies of group 2 pulmonary hypertension are less exhaustive than studies of other causes of pulmonary hypertension. In group 2 patients, pulmonary hypertension may be caused by an isolated increase in left-sided filling pressures or by a combination of this condition with increased pulmonary vascular resistance, with an abnormally high pressure gradient between arteries and pulmonary veins. A better understanding of the conditions underlying pulmonary hypertension is of key importance to establish a comprehensive diagnosis, leading to an adapted treatment to reduce heart failure morbidity and mortality. In this review, epidemiology, mechanisms and diagnostic approaches are reviewed; then, treatment options and future approaches are considered. Copyright © 2017. Published by Elsevier Masson SAS.

  20. Left-Deviating Prism Adaptation in Left Neglect Patient: Reflexions on a Negative Result

    Directory of Open Access Journals (Sweden)

    Jacques Luauté

    2012-01-01

    Full Text Available Adaptation to right-deviating prisms is a promising intervention for the rehabilitation of patients with left spatial neglect. In order to test the lateral specificity of prism adaptation on left neglect, the present study evaluated the effect of left-deviating prism on straight-ahead pointing movements and on several classical neuropsychological tests in a group of five right brain-damaged patients with left spatial neglect. A group of healthy subjects was also included for comparison purposes. After a single session of exposing simple manual pointing to left-deviating prisms, contrary to healthy controls, none of the patients showed a reliable change of the straight-ahead pointing movement in the dark. No significant modification of attentional paper-and-pencil tasks was either observed immediately or 2 hours after prism adaptation. These results suggest that the therapeutic effect of prism adaptation on left spatial neglect relies on a specific lateralized mechanism. Evidence for a directional effect for prism adaptation both in terms of the side of the visuomanual adaptation and therefore possibly in terms of the side of brain affected by the stimulation is discussed.

  1. Clinical psychomotor skills among left and right handed medical students: are the left-handed medical students left out?

    Science.gov (United States)

    Alnassar, Sami; Alrashoudi, Aljoharah Nasser; Alaqeel, Mody; Alotaibi, Hala; Alkahel, Alanoud; Hajjar, Waseem; Al-Shaikh, Ghadeer; Alsaif, Abdulaziz; Haque, Shafiul; Meo, Sultan Ayoub

    2016-03-22

    There is a growing perception that the left handed (LH) medical students are facing difficulties while performing the clinical tasks that involve psychomotor skill, although the evidence is very limited and diverse. The present study aimed to evaluate the clinical psychomotor skills among Right-handed (RH) and left-handed (LH) medical students. For this study, 54 (27 left handed and 27 right handed) first year medical students were selected. They were trained for different clinical psychomotor skills including suturing, laparoscopy, intravenous cannulation and urinary catheterization under the supervision of certified instructors. All students were evaluated for psychomotor skills by different instructors. The comparative performance of the students was measured by using a global rating scale, each selected criteria was allotted 5-points score with the total score of 25. There were no significant differences in the performance of psychomotor skills among LH and RH medical students. The global rating score obtained by medical students in suturing techniques was: LH 15.89 ± 2.88, RH 16.15 ± 2.75 (p = 0.737), cannulation techniques LH 20.44 ± 2.81, RH 20.70 ± 2.56 (p = 0.725), urinary catheterization LH 4.33 ± 0.96 RH 4.11 ± 1.05 (p = 0.421). For laparoscopic skills total peg transfer time was shorter among LH medical students compared to RH medical students (LH 129.85 ± 80.87 s vs RH 135.52 ± 104.81 s) (p = 0.825). However, both RH and LH students completed their procedure within the stipulated time. Among LH and RH medical students no significant difference was observed in performing the common surgical psychomotor skills. Surgical skills for LH or RH might not be a result of innate dexterity but rather the academic environment in which they are trained and assessed. Early laterality-related mentoring in medical schools as well as during the clinical residency might reduce the inconveniences faced by the left

  2. The New Left in the European Democracies: The Case of the German Radical Left

    Directory of Open Access Journals (Sweden)

    Marco Damiani

    2015-03-01

    Full Text Available The new form of the social and political conflict cannot be explained by the traditional categories of right and left, but it articulates to them on two plans, that of the establishment, intended like plan of the structured political conflict from the traditional actors, and that of the anti-establishment, in which new representations of politics emerge. The New Left is characterized by type of intermittent participation and new perspectives on mobilization inside the parties and the social movements. This type of parties differs moreover from the traditional ones left of the socialist and social democratic left in not arranging of organizations collaterals placed under the direction of the leadership of the same party. The mobilization that spontaneously assumes not conventional forms of active participation of the citizens, or is primed by the action of an associative network of which the same parties take part, than however does not monopo-lize the collective action. In this regard, the attention will be dedicated to the study of Die Linke: an anti-establishment party of the non-socialist German left-wing, heir to the communist tradition. The choice was affected to the German model because: 1 Germany is a country with a strong social democratic tradition, but 25 years after the fall of the Berlin Wall the German political system identifies a new antagonist political party; 2 Die Linke represents an interesting case in the political landscape of the European radical left because is a one-party that gives up at the federation of parties to try to unify the political parties of German radical left-wing.

  3. Dosimetric comparison for volumetric modulated arc therapy and intensity-modulated radiotherapy on the left-sided chest wall and internal mammary nodes irradiation in treating post-mastectomy breast cancer

    International Nuclear Information System (INIS)

    Zhang, Qian; Yu, Xiao Li; Hu, Wei Gang; Chen, Jia Yi; Wang, Jia Zhou; Ye, Jin Song; Guo, Xiao Mao

    2015-01-01

    The aim of the study was to evaluate the dosimetric benefit of applying volumetric modulated arc therapy (VMAT) on the post-mastectomy left-sided breast cancer patients, with the involvement of internal mammary nodes (IMN). The prescription dose was 50 Gy delivered in 25 fractions, and the clinical target volume included the left chest wall (CW) and IMN. VMAT plans were created and compared with intensity-modulated radiotherapy (IMRT) plans on Pinnacle treatment planning system. Comparative endpoints were dose homogeneity within planning target volume (PTV), target dose coverage, doses to the critical structures including heart, lungs and the contralateral breast, number of monitor units and treatment delivery time. VMAT and IMRT plans showed similar PTV dose homogeneity, but, VMAT provided a better dose coverage for IMN than IMRT (p = 0.017). The mean dose (Gy), V 30 (%) and V 10 (%) for the heart were 13.5 ± 5.0 Gy, 9.9% ± 5.9% and 50.2% ± 29.0% by VMAT, and 14.0 ± 5.4 Gy, 10.6% ± 5.8% and 55.7% ± 29.6% by IMRT, respectively. The left lung mean dose (Gy), V 20 (%), V 10 (%) and the right lung V 5 (%) were significantly reduced from 14.1 ± 2.3 Gy, 24.2% ± 5.9%, 42.4% ± 11.9% and 41.2% ± 12.3% with IMRT to 12.8 ± 1.9 Gy, 21.0% ± 3.8%, 37.1% ± 8.4% and 32.1% ± 18.2% with VMAT, respectively. The mean dose to the contralateral breast was 1.7 ± 1.2 Gy with VMAT and 2.3 ± 1.6 Gy with IMRT. Finally, VMAT reduced the number of monitor units by 24% and the treatment time by 53%, as compared to IMRT. Compared to 5-be am step-and-shot IMRT, VMAT achieves similar or superior target coverage and a better normal tissue sparing, with fewer monitor units and shorter delivery time

  4. Left ventricular heart failure and pulmonary hypertension†

    Science.gov (United States)

    Rosenkranz, Stephan; Gibbs, J. Simon R.; Wachter, Rolf; De Marco, Teresa; Vonk-Noordegraaf, Anton; Vachiéry, Jean-Luc

    2016-01-01

    Abstract In patients with left ventricular heart failure (HF), the development of pulmonary hypertension (PH) and right ventricular (RV) dysfunction are frequent and have important impact on disease progression, morbidity, and mortality, and therefore warrant clinical attention. Pulmonary hypertension related to left heart disease (LHD) by far represents the most common form of PH, accounting for 65–80% of cases. The proper distinction between pulmonary arterial hypertension and PH-LHD may be challenging, yet it has direct therapeutic consequences. Despite recent advances in the pathophysiological understanding and clinical assessment, and adjustments in the haemodynamic definitions and classification of PH-LHD, the haemodynamic interrelations in combined post- and pre-capillary PH are complex, definitions and prognostic significance of haemodynamic variables characterizing the degree of pre-capillary PH in LHD remain suboptimal, and there are currently no evidence-based recommendations for the management of PH-LHD. Here, we highlight the prevalence and significance of PH and RV dysfunction in patients with both HF with reduced ejection fraction (HFrEF) and HF with preserved ejection fraction (HFpEF), and provide insights into the complex pathophysiology of cardiopulmonary interaction in LHD, which may lead to the evolution from a ‘left ventricular phenotype’ to a ‘right ventricular phenotype’ across the natural history of HF. Furthermore, we propose to better define the individual phenotype of PH by integrating the clinical context, non-invasive assessment, and invasive haemodynamic variables in a structured diagnostic work-up. Finally, we challenge current definitions and diagnostic short falls, and discuss gaps in evidence, therapeutic options and the necessity for future developments in this context. PMID:26508169

  5. Preoperative left ventricular ejection fraction and left atrium reverse remodeling after mitral regurgitation surgery.

    Science.gov (United States)

    Machado, Lucia R; Meneghelo, Zilda M; Le Bihan, David C S; Barretto, Rodrigo B M; Carvalho, Antonio C; Moises, Valdir A

    2014-11-06

    Left atrium enlargement has been associated with cardiac events in patients with mitral regurgitation (MR). Left atrium reverse remodeling (LARR) occur after surgical correction of MR, but the preoperative predictors of this phenomenon are not well known. It is therefore important to identify preoperative predictors for postoperative LARR. We enrolled 62 patients with chronic severe MR (prolapse or flail leaflet) who underwent successful mitral valve surgery (repair or replacement); all with pre- and postoperative echocardiography. LARR was defined as a reduction in left atrium volume index (LAVI) of ≥ 25%. Stepwise multiple regression analysis was used to identify independent predictors of LARR. LARR occurred in 46 patients (74.2%), with the mean LAVI decreasing from 85.5 mL/m2 to 49.7 mL/m2 (p <0.001). These patients had a smaller preoperative left ventricular systolic volume (p =0.022) and a higher left ventricular ejection fraction (LVEF) (p =0.034). LVEF was identified as the only preoperative variable significantly associated with LARR (odds ratio, 1.086; 95% confidence interval, 1.002-1.178). A LVEF cutoff value of 63.5% identified patients with LARR of ≥ 25% with a sensitivity of 71.7% and a specificity of 56.3%. LARR occurs frequently after mitral valve surgery and is associated with preoperative LVEF higher than 63.5%.

  6. Photonic-resonant left-handed medium

    International Nuclear Information System (INIS)

    Shen Jianqi

    2006-01-01

    A new scheme to realize simultaneously negative permittivity and permeability in a coherent atomic vapor medium (photonic-resonant material) via a coherent driving mechanism is suggested. It is verified that the atomic system coherently driven by a strong optical field will give rise to a negative refractive index in certain probe frequency ranges. One of the most remarkable features of the present scheme is such that a slab fabricated by the left-handed vapor medium is an ideal candidate for designing perfect lenses since the photonic-resonant atomic vapor cannot only exhibit an isotropic negative refractive index, but also provide a good impedance match at the air-medium interfaces

  7. Mycobacterium chimaera left ventricular assist device infections.

    Science.gov (United States)

    Balsam, Leora B; Louie, Eddie; Hill, Fred; Levine, Jamie; Phillips, Michael S

    2017-06-01

    A global outbreak of invasive Mycobacterium chimaera infections after cardiac surgery has recently been linked to bioaerosols from contaminated heater-cooler units. The majority of cases have occurred after valvular surgery or aortic graft surgery and nearly half have resulted in death. To date, infections in patients with left ventricular assist devices (LVADs) have not been characterized in the literature. We report two cases of device-associated M. chimaera infection in patients with continuous-flow LVADs and describe challenges related to diagnosis and management in this population. © 2017 Wiley Periodicals, Inc.

  8. Heart Failure with Transient Left Bundle Branch Block in the Setting of Left Coronary Fistula

    Directory of Open Access Journals (Sweden)

    Stephen P. Juraschek

    2011-01-01

    Full Text Available Coronary arterial fistulas are rare communications between vessels or chambers of the heart. Although cardiac symptoms associated with fistulas are well described, fistulas are seldom considered in the differential diagnosis of acute myocardial ischemia. We describe the case of a 64-year-old man who presented with left shoulder pain, signs of heart failure, and a new left bundle branch block (LBBB. Cardiac catheterization revealed a small left anterior descending (LAD-to-pulmonary artery (PA fistula. Diuresis led to subjective improvement of the patient's symptoms and within several days the LBBB resolved. We hypothesize that the coronary fistula in this patient contributed to transient ischemia of the LAD territory through a coronary steal mechanism. We elected to observe rather than repair the fistula, as his symptoms and ECG changes resolved with treatment of his heart failure.

  9. Coronary artery to left ventricle fistula

    Directory of Open Access Journals (Sweden)

    Kumar Vivek

    2005-11-01

    Full Text Available Abstract Background Coronary cameral fistulas are an uncommon entity, the etiology of which may be congenital or traumatic. They involve abnormal termination of a coronary artery, usually the right coronary, into a cardiac chamber, usually the right ventricle. Case Presentation We describe a case of female patient with severe aortic stenosis and interventricular septal hypertrophy that underwent bioprosthetic aortic valve replacement with concomitant septal myectomy. On subsequent follow-up an abnormal flow traversing the septum into the left ventricle was identified and Doppler interrogation demonstrated a continuous flow, with a predominantly diastolic component, consistent with coronary arterial flow. Conclusion The literature on coronary cameral fistulas is reviewed and the etiology of the diagnostic findings discussed. In our patient, a coronary artery to left ventricle fistula was the most likely explanation secondary to trauma to the septal perforator artery during myectomy. Since the patient was asymptomatic at the time of diagnosis no intervention was recommended and has done well on follow-up.

  10. Nonlinear left-handed transmission line metamaterials

    International Nuclear Information System (INIS)

    Kozyrev, A B; Weide, D W van der

    2008-01-01

    Metamaterials, exhibiting simultaneously negative permittivity ε and permeability μ, more commonly referred to as left-handed metamaterials (LHMs) and also known as negative-index materials, have received substantial attention in the scientific and engineering communities [1]. Most studies of LHMs (and electromagnetic metamaterials in general) have been in the linear regime of wave propagation and have already inspired new types of microwave circuits and devices. The results of these studies have already been the subject of numerous reviews and books. This review covers a less explored but rapidly developing area of investigation involving media that combine nonlinearity (dependence of the permittivity and permeability on the magnitude of the propagating field) with the anomalous dispersion exhibited by LHM. The nonlinear phenomena in such media will be considered on the example of a model system: the nonlinear left-handed transmission line. These nonlinear phenomena include parametric generation and amplification, harmonic and subharmonic generation as well as modulational instabilities and envelope solitons. (topical review)

  11. Rare occurrence of the left maxillary horizontal third molar impaction ...

    African Journals Online (AJOL)

    Rare occurrence of the left maxillary horizontal third molar impaction, the right maxillary third molar vertical impaction and the left mandibular third molar vertical impaction with inferior alveolar nerve proximity in a 30 year old female: a case report.

  12. challenges left-handed students face in kenyan girls' secondary

    African Journals Online (AJOL)

    IICBA01

    Previous research on left-handedness has mainly taken place in developed countries. This ... participants were five left-handed science students and their respective subject teachers from a ... Qualitative data was collected through classroom.

  13. Behavioral evidence for left-hemisphere specialization of motor planning

    NARCIS (Netherlands)

    Janssen, L.; Meulenbroek, R.G.; Steenbergen, B.

    2011-01-01

    Recent studies suggest that the left hemisphere is dominant for the planning of motor actions. This left-hemisphere specialization hypothesis was proposed in various lines of research, including patient studies, motor imagery studies, and studies involving neurophysiological techniques. However,

  14. Incidental retroaortic left innominate vein in adult patient

    Directory of Open Access Journals (Sweden)

    Alexandre Semionov, MD, PhD

    2017-09-01

    Full Text Available Retro-aortic left innominate vein is a rare vascular abnormality, usually associated with congenital heart disease. Here we report a case of isolated retro-aortic left innominate vein in an adult female.

  15. Anesthetic implications of total anomalous systemic venous connection to left atrium with left isomerism

    Directory of Open Access Journals (Sweden)

    Parimala Prasanna Simha

    2012-01-01

    Full Text Available Total anomalous systemic venous connection (TASVC to the left atrium (LA is a rare congenital anomaly. An 11-year-old girl presented with complaints of palpitations and cyanosis. TASVC with left isomerism and noncompaction of LV was diagnosed after contrast echocardiogram and computed tomography angiogram. The knowledge of anatomy and pathophysiology is essential for the successful management of these cases. Anesthetic concerns in this case were polycythemia, paradoxical embolism and rhythm abnormalities. The patient was successfully operated by rerouting the systemic venous connection to the right atrium.

  16. Quantitative pulmonary perfusion imaging at 3.0 T of 2-year-old children after congenital diaphragmatic hernia repair: initial results

    International Nuclear Information System (INIS)

    Zoellner, F.G.; Schad, L.R.; Zahn, K.; Schaible, T.; Schoenberg, S.O.; Neff, K.W.

    2012-01-01

    To investigate whether dynamic contrast-enhanced MR imaging of the lung following congenital diaphragmatic hernia repair is feasible at 3.0 T in 2-year-old children and whether associated lung hypoplasia (reflected in reduced pulmonary microcirculation) can be demonstrated in MRI. Twelve children with a mean age 2.0 ± 0.2 years after hernia repair underwent DCE-MRI at 3.0 T using a time-resolved angiography with stochastic trajectories sequence. Quantification of lung perfusion was performed using a pixel-by-pixel deconvolution approach. Six regions of interest were placed (upper, middle and lower parts of right and left lung) to assess differences in pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) while avoiding the inclusion of larger pulmonary arteries and veins. The difference in PBF and PBV between ipsilateral and contralateral lung was significant (P < 0.5). No significant differences could be detected for the MTT (P = 0.5). DCE-MRI in 2-year-old patients is feasible at 3.0 T. Reduced perfusion in the ipsilateral lung is reflected by significantly lower PBF values compared with the contralateral lung. DCE-MRI of the lung in congenital diaphragmatic hernia can help to characterise lung hypoplasia initially and in the long-term follow-up of children after diaphragmatic repair. (orig.)

  17. A Right Brain/Left Brain Model of Acting.

    Science.gov (United States)

    Bowlen, Clark

    Using current right brain/left brain research, this paper develops a model that explains acting's underlying quality--the actor is both himself and the character. Part 1 presents (1) the background of the right brain/left brain theory, (2) studies showing that propositional communication is a left hemisphere function while affective communication…

  18. Left Ventricular Geometry In Nigerians With Type II Diabetes Mellitus ...

    African Journals Online (AJOL)

    Background: Left ventricular hypertrophy is independently associated with increased incidence of cardiovascular disease, cardiovascular and all cause mortality. In a relatively healthy hypertensive adult population, type II diabetes is associated with higher left ventricular mass, concentric left ventricular geometry and lower ...

  19. Accessory left gastric artery: angiographic anatomy

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Kang Soo; Lim, Hyung Guhn; Kim, Hong Soo; Jeon, Doo Sung [Presbyterian Medical Center, Chunju (Korea, Republic of); Chung, Jin Wook; Park, Jae Hyung [College of Medicine and the Institute of Radiation Medicine, Seoul National University, Seoul (Korea, Republic of); Song, Soon Young [Myongji Hospital, College of Medicine, Kwandong University, Seoul (Korea, Republic of)

    2000-09-01

    To evaluate the angiographic anatomy of the accessory left gastric artery (accLGA). We evaluated the angiographic findings of the accLGA in 50 patients (Angiostar; Siemens, Erlangen, Germany). Performing celiac and selective angiography in 50 and 34 patients, respectively. By means of celiac angiography, (1) site of origin, (2) anatomical course, (3) diameter, (4) degree of tortuosity, and (5) distal tapering were evaluated, while selective angiography was used to determine (1) arterial branching, (2) area of blood supply, and (3) patterns of gastric wall stain. Celiac angiography showed that the accLGA arose from the left hepatic artery (LHA) in 45 cases (90%) and from the proper hepatic artery in five (10%). If the accLGA arose from the LHA, its origin entirely depended on the branching pattern of the latter. It always arose from the lateral branch of the LHA furthest to the left and uppermost, and proximal to its umbilical point. The most common anatomical course of the accLGA, seen in 27 cases (54%), was between the S2 and S3 segmental branch. The diameter and degree of tortuosity of the accLGA were similar to those of adjacent intrahepatic branches in 21 (42%) and 33 cases (66%), respectively. The degree of tapering was less than that of adjacent intrahepatic vessel in 28 (56%). Selective angiography demonstrated esophageal branching of the acc LGA in 27 cases (79%), inferior phrenic arterial branching in three (9%), a mediastinal branch in one (3%), and hypervascularity of the lung in one (3%). In 15 cases (44%), bifurcation of the accLGA was recognized. The vascular territory of the accLGA was the gastric fundus together with the distal esophagus in 21 cases (62%), mainly the gastric fundus in six (18%), and mainly the distal esophagus in four (12%). The pattern of gastric mucosal stain was curvilinear wall in 31 cases (91%) and nodular in three (9%). A knowledge of the angiographic anatomy of the accLGA facilitates accurate recognition of this artery on

  20. Related anomalies of origin of left vertebral and left inferior thyroid arteries

    International Nuclear Information System (INIS)

    Sartor, K.; Freckmann, N.; Boeker, D.K.; Allgemeines Krankenhaus Altona, Hamburg

    1981-01-01

    The known rare occurrence of related anomalies of origin of vertebral and inferior thyroid arteries appears not to be documented in the angiographic literature. Three cases with manifestation on the left side are presented. Embryology is discussed briefly. Knowledge of such anomalies is of importance to surgeons as well as angiographers. (orig.) [de

  1. Visceral subpleural hematoma of the left diaphragmatic surface following left upper division segmentectomy

    Directory of Open Access Journals (Sweden)

    Yasushi Mizukami

    2017-10-01

    Full Text Available Abstract Background Pulmonary visceral subpleural hematoma is rare. We report visceral subpleural hematoma of the left diaphragmatic surface following left upper division segmentectomy. This very rare case was difficult to distinguish from thoracic abscess. Case presentation A 68-year-old man with hypertension had undergone video-assisted thoracoscopic left upper division segmentectomy for suspected lung carcinoma. Deep vein thrombosis of the lower leg was identified and edoxaban, a so-called novel oral anticoagulant, was started on postoperative day 7. The chest drainage tube was removed on postoperative day 12 because of persistent air leakage, but fever appeared the same day. Computed tomography revealed a cavity with mixed air and fluid, so antibiotics were started on suspicion of abscess. Computed tomography-guided drainage was attempted, but proved unsuccessful. Fever continued and surgical investigation was therefore performed. Visceral subpleural hematoma was identified under the diaphragmatic surface of the left basal lung. We excised the pleura, then performed drainage and applied running sutures. The parenchyma and visceral pleura were covered with polyglycolic acid sheet and fibrin glue. Edoxaban was restarted on postoperative day 12 of video-assisted thoracoscopic surgery and no recurrence of hematoma has been revealed. Conclusions Visceral subpleural hematoma after thoracic surgery is extremely rare. Furthermore, correct diagnosis was difficult and surgery offered a good diagnostic and therapeutic procedure.

  2. Count-based left ventricular volume determination utilizing a left posterior oblique view for attenuation correction

    International Nuclear Information System (INIS)

    Rabinovitch, M.A.; Kalff, V.; Koral, K.

    1984-01-01

    This study aimed to determine the inherent error of the left ventricular volume measurement from the gated equilibrium blood pool scintigram utilizing the count-based technique. The study population consisted of 26 patients who had undergone biplane contrast ventriculography. The patients were imaged with a parallel-hole collimator in the left anterior oblique position showing the septum to best advantage. A reference blood sample was counted and radionuclide volumes calculated without correction for attenuation. Attenuation corrected volumes were derived with the factor 1/e/sup -/+d/, where d = distance from skin marker to center of the left ventricle in the orthogonal left posterior oblique view and μ = linear attenuation coefficient. A series of μ values from 0.08 to 0.15 cm -1 was evaluated. The tightest 95% confidence limits achieved for an end-diastolic 150-ml ventricle were +/- 44ml, and for an end-systolic 75-ml ventricle +/- 32 ml. In view of the magnitude of inherent error, the count-based volume measurement may be more suitable for group analyses and in cases in which an individual patient serves as his own control

  3. Left Hemisphere Regions Are Critical for Language in the Face of Early Left Focal Brain Injury

    Science.gov (United States)

    Beharelle, Anjali Raja; Dick, Anthony Steven; Josse, Goulven; Solodkin, Ana; Huttenlocher, Peter R.; Levine, Susan C.; Small, Steven L.

    2010-01-01

    A predominant theory regarding early stroke and its effect on language development, is that early left hemisphere lesions trigger compensatory processes that allow the right hemisphere to assume dominant language functions, and this is thought to underlie the near normal language development observed after early stroke. To test this theory, we…

  4. Effects of sedation on echocardiographic variables of left atrial and left ventricular function in healthy cats.

    Science.gov (United States)

    Ward, Jessica L; Schober, Karsten E; Fuentes, Virginia Luis; Bonagura, John D

    2012-10-01

    Although sedation is frequently used to facilitate patient compliance in feline echocardiography, the effects of sedative drugs on echocardiographic variables have been poorly documented. This study investigated the effects of two sedation protocols on echocardiographic indices in healthy cats, with special emphasis on the assessment of left atrial size and function, as well as left ventricular diastolic performance. Seven cats underwent echocardiography (transthoracic two-dimensional, spectral Doppler, color flow Doppler and tissue Doppler imaging) before and after sedation with both acepromazine (0.1 mg/kg IM) and butorphanol (0.25 mg/kg IM), or acepromazine (0.1 mg/kg IM), butorphanol (0.25 mg/kg IM) and ketamine (1.5 mg/kg IV). Heart rate increased significantly following acepromazine/butorphanol/ketamine (mean±SD of increase, 40±26 beats/min) and non-invasive systolic blood pressure decreased significantly following acepromazine/butorphanol (mean±SD of decrease, 12±19 mmHg). The majority of echocardiographic variables were not significantly different after sedation compared with baseline values. Both sedation protocols resulted in mildly decreased left ventricular end-diastolic dimension and mildly increased left ventricular end-diastolic wall thickness. This study therefore failed to demonstrate clinically meaningful effects of these sedation protocols on echocardiographic measurements, suggesting that sedation with acepromazine, butorphanol and/or ketamine can be used to facilitate echocardiography in healthy cats.

  5. LEFT RENAL TUMOR: A RARE CADAVERIC FINDINGS

    Directory of Open Access Journals (Sweden)

    Siva Prasad

    2015-08-01

    Full Text Available A benign tumour is a non - cancerous growth that does not spread (metastasize to other parts of the body and is not usually life - threatening. Many researchers believe that most of the following types of kidney tumours are benign. However, others feel that some of these tumours have the potential to develop into renal cell carcinoma. There are no diagnostic tests that can confirm if a benign tumour has the potential to become cancerous. Benign tumours are sometimes found along with renal cell carcinoma when a removed kidney is examined by a pathologist. For these reasons, benign kidney tumours are treated like malignant tumours. In this case we have observed a male cadaver with a large tumour in the left kidney during our routine dissections of undergraduates .

  6. Rectal volvulus following laparoscopic left hemicolectomy

    Science.gov (United States)

    Sutton, Paul Anthony; Lee, Han Sian; Din, Islah; Vimalachandran, Dale

    2013-01-01

    A 60-year-old lady with a history of Dukes B2 (T3N0M0) colorectal cancer presented some 2 years following a laparoscopic left hemicolectomy with a 4-day history of absolute constipation. A plain radiograph demonstrated large bowel obstruction, and subsequent CT of the abdomen showed the level of the obstruction to be at the rectum. Initially the aetiology was believed to be recurrence at the site of the anastomosis; however, subsequent review of the imaging and indeed endoscopic examination of the rectum showed it to be volvulus. This was initially treated with endoscopic decompression and later by the insertion of a flatus tube to good effect. The patient was discharged 3 days later with no recurrence of her symptoms at 2 months. PMID:23608861

  7. Polycythemia vera presenting with left hemichoreiform movements

    International Nuclear Information System (INIS)

    Mori, Tamiharu; Shimomura, Chikako; Ishibashi, Hiroshi; Tsujihata, Mitsuhiro; Nagataki, Shigenobu.

    1985-01-01

    A 65-year-old man developed abruptly choreiform movements involving the left face, arm and leg one day prior to admission. Physical examination revealed red face and palms, hyperemic conjunctivae and atrial fibrillations. Blood pressure was 168/90. Spleen was not palpable. Hemichoreiform movements of the left face and limbs were observed. There was no other neurological abnormalities. Laboratory studies showed RBC 880x10 4 , Hb 22.4g/dl, Hct 63%, WBC 8,100, platelets 22.9x10 4 , ESR 0mm/hr, RBC oxygen saturation 97%, serum iron 67 μg/dl, LDH 593 units, uric acid 14mg/dl, and erythropoietine (HI method) 19mIU/ml (normal 28-88). Bone marrow showed myeloid nucleated cell count 38.6x10 4 . ECG showed atrial fibrillations. Chest X-ray and scintigrams of liver and spleen were normal. CSF was normal. Brain CT scan on admission disclosed a low density area in right caudate nucleus. The choreiform movements were rapidly mitigated by venesection and by oral administration of haloperidol(3mg daily). There weeks after discontinuing haloperidol, the hemichorea returned. The routine hematology showed RBC 870x10 4 , Hb 19.8g/dl, Hct 62%, WBC 10,200, and plateret 37.4x10 4 . Another venesection reduced the chorea. Pipobroman was administered to control the polycythemia vera. He has been free of choreic movements thereafter. Choreiform movement is rarely observed in polycythemia vera. The pathogenesis is still unknown. The venous congestion, however, may play a role in this case because the choreic movements disappeared by venesection. (author)

  8. The effect of percutaneous transcatheter occlusion of left atrial appendage on left atrium and adjacent anatomic structure in canine

    International Nuclear Information System (INIS)

    Yang Zhihong; Wu Hong; Qin Yongwen; Hu Jianqiang; Ding Zhongru; Liu Zongjun; Liu Biao; Zheng Xing

    2009-01-01

    Objective: To observe the effect of percutaneous transcatheter occlusion of left atrial appendage (LAA) with a new self-manufactured LAA occluder on left atrium and adjacent anatomic structure in canine. Methods: A new self-manufactured LAA occluder was implanted into the LAA through a transseptal catheter in 20 dogs. Before and after the procedure, the experimental dogs were anaesthetized and examined by transthoracic echocardiography (TTE) to measure the diameter and the volume of the left atrium, the left superior pulmonary vein flow velocity and the left atrioventricular valve flow velocity separately. The contrast radiography of the LAA and the left coronary arteriography were performed. Results: The new LAA occluder was implanted successfully in 14 dogs. No obvious changes in the diameter and the volume of the left atrium, in left superior pulmonary vein flow velocity and in left atrioventricular valve flow velocity were found. On arteriography, left circumflex artery was normally displayed after the procedure. No migration of the occluder was seen on TTE and angiography after procedure. Conclusion: Percutaneous transcatheter occlusion of left atrial appendage with a new self-manufactured LAA occluder has no obvious effect on left atrium and adjacent anatomic structure in experimental canine, which indicates that the new-type device is a safe and feasible occluder for LAA. (authors)

  9. What's Left of the Left-Right Dimension? Why the Economic Policy Positions of Europeans Do Not Fit the Left-Right Dimension.

    Science.gov (United States)

    Otjes, Simon

    2018-01-01

    In political science the economic left-right dimension plays a central role. A growing body of evidence shows that the economic policy preferences of a large segment of citizens do not scale sufficiently. Using Mokken scale analysis, this study determines the causes of this phenomenon. Differences in the extent to which the economic policy preferences of citizens fit the left-right dimension can be explained in terms of the interaction between individual level and political system-level variables: citizens who spend more attention to politicians with views that conform to the left-right dimension, have views that conform to the left-right dimension. There is also a role for the legacy of communist dictatorship: citizens who were socialised in democratic countries have views that fit the left-right dimension better than those socialised during communism.

  10. UFO in the Left Atrium: How to Capture Metal Debris Floating in the Left Atrium.

    Science.gov (United States)

    Fassini, Gaetano; Moltrasio, Massimo; Conti, Sergio; Biagioli, Viviana; Tondo, Claudio

    2016-06-01

    Electrophysiology procedures involving left atrium navigation are becoming more frequent, mostly due to the increase of atrial fibrillation ablation. Mapping catheters of different shapes and size as well as dedicated sheaths are mandatory tools for the accomplishment of procedural end point. Therefore, technical issues are expected, usually unrelated to significant risk. However, any accidental intra-atrial device loss of integrity implies a risk of cerebrovascular embolization. The lack of clear evidence on how to manage these events and the need for a quick solution complicate the scenario. We report an empirical solution in the case of debris floating in the left atrium. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  11. Phase image characterization of ventricular contraction in left anterior hemiblock

    International Nuclear Information System (INIS)

    Ono, Akifumi; Mizuno, Haruyoshi; Tahara, Yorio; Ishikawa, Kyozo

    1991-01-01

    We investigated whether or not left anterior hemiblock is present in patients with left axis deviation using first-harmonic Fourier analysis of gated blood-pool images. Gated blood-pool images were taken in 50 patients without contraction abnormality. They included 14 normal subjects, 8 patients with right bundle branch block (RBBB), 20 with left axis deviation (LAD) and 8 with both RBBB and LAD (RBBB+LAD). ECG gated blood-pool scans were acquired in the anterior and 'best septal' left anterior oblique projections. First, the phase images were displayed cinematically as a continuous-loop movie. Next, for quantitative analysis of the phase image, the whole left ventricular and left ventricular high lateral regions of interest were drawn. The 'regional phase shift' (RPS) was then defined as {RPS=A-a} where 'A' is the mean value of the whole left ventricular phase angles and 'a' is that of phase angles in the high lateral region. The left ventricular phase changes and the RPSs in the RBBB and LAD groups were similar to those in the normal group. In the RBBB+LAD group, the latest phase changes occurred in the high anterolateral region. The RPSs of this group were significantly lower than those in the other 3 groups (p<0.01). These data suggest that left anterior hemiblock might coexist with RBBB in patients with RBBB+LAD, whereas left anterior hemiblock might not exist in the majority of patients with LAD alone. (author)

  12. [Surgical angioplasty of the left main coronary artery].

    Science.gov (United States)

    Vranes, Mile; Velinović, Milos; Kocica, Mladen; Mikić, Aleksandar; Velimirović, Dusan; Djukić, Petar

    2010-01-01

    The conventional treatment for isolated stenosis of the left main coronary artery is bypass surgery (myocardial revascularization). However, the process of atherosclerosis is not arrested by myocardial revascularization and it will lead to the occlusion of the left main coronary artery. Revascularization will establish retrograde perfusion for 50-70% of the myocardium of the left ventricle. Direct surgical angioplasty of the left main coronary artery enables normal physiological perfusion of the whole myocardium and better myocardial function. The aim of our study is to point out a new surgical approach of treating left main coronary artery stenosis. Between October 2002 and October 2003, direct surgical angioplasty of the main left coronary artery was performed on three patients with isolated stenosis of the left main coronary artery using the anterior approach and the pericardium as a patch. The procedure was performed under total endotracheal anaesthesia and standard cardiopulmonary circulation, moderate hypothermia, anterograde St. Tomas cardioplegia and local cooling. Patients were followed clinically, echocardiographically and by load-tests. All three patients were without complications. In postoperative follow-up (54-68 months) neither angina pectoris nor electrocardiographically registered ischaemic changes were found. Load-tests performed every six months on all three patients were negative. Surgical angioplasty of isolated stenosis of the left main coronary artery is a preferred method for treating this type of coronary disease. Contraindications for this type of treatment are stenosis of the left main coronary artery with bifurcation and advanced calcification of the left main coronary artery.

  13. Left hand polydactyly: a case report.

    Science.gov (United States)

    Mumoli, Nicola; Gandini, Daniele; Wamala, Edris Kalanzi; Cei, Marco

    2008-11-24

    Polydactyly is a congenital anomaly with a wide range of manifestations that occurs in many forms, ranging from varying degrees of mere splitting to completely duplicated thumb. When duplication occurs alone, it is usually unilateral and sporadic. In this case report we describe an otherwise healthy 19-year-old woman of Tibetan heritage with isolated left hand preaxial polydactyly. She experienced working related difficulties in her daily yak's milking. She subsequently underwent surgical correction, and the over number thumb was removed with associated meticulous skeletal and soft tissue reconstruction. Polydactyly is the most common congenital digital anomaly of the hand and foot. It can occur in isolation or as part of a syndrome. Surgery is necessary to create a single, functioning thumb and is indicated to improve cosmesis. Skin, nail, bone, ligament, and musculoskeletal elements must be combined to reconstruct an optimal digit. In this case (Tibetan society is almost exclusively a sheep-breeding one) surgery was necessary to leave a single, functioning thumb for her work as yak milkmaid.

  14. Assessment of left atrial volume and function

    DEFF Research Database (Denmark)

    Kühl, J Tobias; Lønborg, Jacob; Fuchs, Andreas

    2012-01-01

    dynamic LA volume changes. Conversely, cardiac magnetic resonance imaging (CMR) and multi-slice computed tomography (MSCT) appears more appropriate for such measures. We sought to determine the relationship between LA size assessed with TTE and LA size and function assessed with CMR and MSCT. Fifty......-four patients were examined 3 months post myocardial infarction with echocardiography, CMR and MSCT. Left atrial volumes and LA reservoir function were assessed by TTE. LA time-volume curves were determined and LA reservoir function (cyclic change and fractional change), passive emptying function (reservoir...... between CMR and MSCT, with a small to moderate bias in LA(max) (4.9 ± 10.4 ml), CC (3.1 ± 9.1 ml) and reservoir volume (3.4 ± 9.1 ml). TTE underestimates LA(max) with up to 32% compared with CMR and MSCT (P ...

  15. Left-right symmetric superstring supergravitation

    International Nuclear Information System (INIS)

    Burova, M.V.; Ter-Martirosyan, K.E.

    1988-01-01

    A left-right (L-R) symmetric model of four-dimensional supergravitation with a SO(10) gauge group obtained as the low-energy limit is superstring theory is considered. The spectrum of the gauge fields and their interactions are in agreement with the Weinberg-Salam theory. In addition, the model includes heavy W R ± and Z μ ' bosons. Beside the N g =3 generations of the 16-plets the SO(10) model includes the fragments of such generations which play the role of Higgs particles and also scalar chiral filds, the number of which exceeds by one the number of generations. As a result the neutrinos of each generation obtain a stable small Majorana mass. It is shown that the scalar field potential leads to spontaneous violation of the SU(2) R group and L-R symmetry and at low energies the standard Weinberg-Salam theory appears. However, reasonable values of X bosons masses M x and sun 2 Θ W (Θ W is the Weinberg angle) can be obtained in the model only in the case of high mass scale M R ∼10 10 -10 12 GeV of the right group SU(2) R violation

  16. Endocarditis in left ventricular assist device

    Science.gov (United States)

    Thyagarajan, Braghadheeswar; Kumar, Monisha Priyadarshini; Sikachi, Rutuja R; Agrawal, Abhinav

    2016-01-01

    Summary Heart failure is one of the leading causes of death in developed nations. End stage heart failure often requires cardiac transplantation for survival. The left ventricular assist device (LVAD) has been one of the biggest evolvements in heart failure management often serving as bridge to transplant or destination therapy in advanced heart failure. Like any other medical device, LVAD is associated with complications with infections being reported in many patients. Endocarditis developing secondary to the placement of LVAD is not a frequent, serious and difficult to treat condition with high morbidity and mortality. Currently, there are few retrospective studies and case reports reporting the same. In our review, we found the most common cause of endocarditis in LVAD was due to bacteria. Both bacterial and fungal endocarditis were associated with high morbidity and mortality. In this review we will be discussing the risk factors, organisms involved, diagnostic tests, management strategies, complications, and outcomes in patients who developed endocarditis secondary to LVAD placement. PMID:27672540

  17. Left ventricular noncompaction: Clinical-echocardiographic study

    Directory of Open Access Journals (Sweden)

    Nikolić Aleksandra

    2012-01-01

    Full Text Available Background/Aim. Left ventricular noncompaction (LVNC is a disorder in endomyocardial morphogenesis, seen either isolated (in the absence of other cardiac anomalies or in association with congenital heart disease and some neuromuscular diseases. Intrauterine arrest of the compaction of myocardial fibers is postulated to be the reason of LVNC. Recognition of this condition is extremely important due to its high mortality and morbidity that lead to progressive heart failure, ventricular arrhythmias and thromboembolic events. The aim of this study was to determine the prevalence and clinical presentation of LVNC among consecutive outpatients according to clinical and echocardiographyic findings. Methode. A total of 3,854 consecutive patients examined at the Institute for Cardiovascular Diseases within a period January 2006 - January 2007 were included in the study. All the patients underwent echocardiographic examination using the same equipment (Vivid 7, GE Medical System. Echocardiographic parameters and clinical presentation in patients with echocardiographic criteria for LVNC were analyzed. Results. Analyzing 3,854 consecutive outpatients, using two-dimensional Color Doppler echocardiography from January 2006 to January 2007, 12 patients met the criteria for LVNC. Seven of them were male. The mean age at diagnosis was 45 ± 15 years. Analyzing clinical manifestation of LVNC it was found that seven patients had signs of heart failure, six had arrhythmias with no embolic events. Conclusion. Our results suggest that the real prevalence of LVNC may be higher than expected. New studies have to be done to solve this problem.

  18. Evaluation of left cardiac function by exercise in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Konishi, Tokuji; Horayama, Norihisa; Hamada, Masayuki; Nakano, Takeshi; Takezawa, Hideo

    1981-01-01

    Left ventricular systolic and diastolic features at rest and exercise in hypertrophic cardiomyopathy were evaluated by Fourier analysis of blood pool scintigraphy (intracorporeal labelling with sup(99m)Tc-RBC). In the normal group (17 subjects), the left ventricular ejection fraction showed a linear increase, but no abnormality of regional ventricular wall motion, by multistage exercises. The hypertrophic cardiomyopathy group showed higher left ventricular ejection fractions at rest than those of the normal group, and in the HCM group (non-obstructive, from morphological features; 7 cases) the left ventricular ejection fraction did not increase any more when it reached a certain plateau in accordance with increased stress. In the HOCM (obstructive; 5 cases), the left ventricular ejection fraction showed a decreasing tendency as the stress was increased and also showed contractile abnormalities from the left ventricular center to the apex. Fourier analysis was effective for the evaluation of these changes. (Chiba, N.)

  19. Effect of left ventricular diastolic dysfunction on left atrial appendage function and thrombotic potential in nonvalvular atrial fibrillation.

    Science.gov (United States)

    Demirçelik, Muhammed Bora; Çetin, Mustafa; Çiçekcioğlu, Hülya; Uçar, Özgül; Duran, Mustafa

    2014-05-01

    We aimed to investigate effects of left ventricular diastolic dysfunction on left atrial appendage functions, spontaneous echo contrast and thrombus formation in patients with nonvalvular atrial fibrillation. In 58 patients with chronic nonvalvular atrial fibrilation and preserved left ventricular systolic function, left atrial appendage functions, left atrial spontaneous echo contrast grading and left ventricular diastolic functions were evaluated using transthoracic and transoesophageal echocardiogram. Patients divided in two groups: Group D (n=30): Patients with diastolic dysfunction, Group N (n=28): Patients without diastolic dysfunction. Categorical variables in two groups were evaluated with Pearson's chi-square or Fisher's exact test. The significance of the lineer correlation between the degree of spontaneous echo contrast (SEC) and clinical measurements was evaluated with Spearman's correlation analysis. Peak pulmonary vein D velocity of the Group D was significantly higher than the Group N (p=0.006). However, left atrial appendage emptying velocity, left atrial appendage lateral wall velocity, peak pulmonary vein S, pulmonary vein S/D ratio were found to be significantly lower in Group D (p=0.028, patrial appendage emptying, filling, pulmonary vein S/D levels and lateral wall velocities respectively (r=-0.438, r=-0.328, r=-0.233, r=-0.447). Left atrial appendage emptying, filling, pulmonary vein S/D levels and lateral wall velocities were significantly lower in SEC 2-3-4 than SEC 1 (p=0.003, p=0.029, patrial fibrillation and preserved left ventricular ejection fraction, left atrial appendage functions are decreased in patients with left ventricular diastolic dysfunction. Left ventricular diastolic dysfunction may constitute a potential risk for formation of thrombus and stroke.

  20. Left Sided Amyand's Hernia, A Rare Occurance: A Case Report.

    Science.gov (United States)

    Ravishankaran, Praveen; Mohan, G; Srinivasan, A; Ravindran, G; Ramalingam, A

    2013-06-01

    This is a case report about a 35 year old man admitted with complains of obstructed left sided inguinal hernia. On exploration of the left inguinal canal to our surprise a normal appendix was found in addition to a gangrenous omentum. Resection of the gangrenous omentum was done. Appendectomy was done. This case is reported for its rare occurance as only three such cases of left sided amyand's hernia has been reported so far in literature[4-6].

  1. On left (θ,ϕ-derivations in BCI-algebras

    Directory of Open Access Journals (Sweden)

    G. Muhiuddin

    2014-07-01

    Full Text Available The notion of (regular left (θ,ϕ-derivations of a BCI-algebra is introduced, some useful examples are discussed, and related properties are investigated. Conditions for a left (θ,ϕ-derivation to be regular are provided. The concepts of a d(θ,ϕ-invariant left (θ,ϕ-derivation and θ-ideal are introduced, and their relations are discussed. Furthermore, some more interesting results are established.

  2. Total anomalous systemic venous drainage in left heterotaxy syndrome.

    Science.gov (United States)

    Khandenahally, Ravindranath S; Deora, Surender; Math, Ravi S

    2013-04-01

    Total anomalous systemic venous drainage is an extremely rare congenital heart defect. In this study we describe an 11-year-old girl who presented with a history of fatigue and central cyanosis that she had had since early childhood with unremarkable precordial examination results. Investigations revealed left heterotaxy with all systemic venous drainage to the left-sided atrium with non-compaction of the left ventricle.

  3. Accessory hepatic lobe simulating a left hemidiaphragmatic tumor

    International Nuclear Information System (INIS)

    Kuroiwa, Toshiro; Hirata, Hitoshi; Iwashita, Akinori; Yasumori, Kotaro; Mogami, Hiroshi; Teraoka, Hiroaki

    1984-01-01

    A 72-year-old woman with a 20-year history of neuralgia was confirmed at surgery to have a tumor in the left hemidiaphragmatic region which was connected with the left lobe of the liver. Reassessment of radiological diagnosis after surgery revealed that hepatobiliary scintigraphy and computed tomography using left anterior oblique scanning are useful in differentiating the accessory hepatic lobe of the liver from a tumor and in confirming the diagnosis, respectively. (Namekawa, K.)

  4. Fallopian Tube Herniation through Left Sided Abdominal Drain Site.

    Science.gov (United States)

    Hussain, Khalid; Masood, Jovaria

    2016-06-01

    Intra-abdominal drains have been used since long to prevent intra-abdominal collection, and detect any anastomotic leaks. We report a case of left sided fallopian tube herniation from a left lower abdominal drain site in a 27-year female who underwent caesarian section for breach presentation. Several complications related to drain usage has been described but left sided fallopian tube prolapse through drain site has not been reported in literature.

  5. The Prognostic Value of Left Atrial Peak Reservoir Strain in Acute Myocardial Infarction Is Dependent on Left Ventricular Longitudinal Function and Left Atrial Size

    DEFF Research Database (Denmark)

    Ersbøll, Mads; Andersen, Mads J; Valeur, Nana

    2013-01-01

    of PALS and left ventricular longitudinal strain (global longitudinal strain) in large-scale populations in regard to prognosis. METHODS AND RESULTS: We prospectively included 843 patients (mean age 62.1+/-11.8; 74% male) with acute myocardial infarction and measured global longitudinal strain, left......BACKGROUND: Peak atrial longitudinal strain (PALS) during the reservoir phase has been proposed as a measure of left atrium function in a range of cardiac conditions, with the potential for added pathophysiological insight and prognostic value. However, no studies have assessed the interrelation...

  6. Detection of left ventricular thrombi by computerised tomography

    International Nuclear Information System (INIS)

    Nair, C.K.; Sketch, M.H.; Mahoney, P.D.; Lynch, J.D.; Mooss, A.N.; Kenney, N.P.

    1981-01-01

    Sixteen patients suspected of having left ventricular mural thrombi were studied. All had suffered transmural myocardial infarction. Fifteen patients had a ventricular aneurysm. One had had systemic emboli. The mean length of time between the myocardial infarction and the study was 14.8 months, with a range of one month to 79 months. All patients underwent computerised tomography of the heart, M-mode echocardiography (M-mode), and two-dimensional echocardiography (2-D). Eight patients underwent left ventricular cineangiography. Five patients had surgical confirmation. Computerised tomography, two-dimensional, and M-mode echocardiography predicted left ventricular mural thrombi in 10, eight, and one of the 16 patients, respectively. Left ventricular cineangiography predicted left ventricular mural thrombi in four out of eight patients. Computerised tomography and left ventricular cineangiography correctly predicted the presence or absence of left ventricular thrombi in all five patients who underwent operation. In the same group, however, two-dimensional and M-mode echocardiography failed to predict the presence of thrombi in one and three patients, respectively. Among the 11 patients without surgical confirmation, one, in whom no left ventricular thrombi were shown by M-mode and two-dimensional echocardiography, was found to have thrombi on computerised tomography. In another, two-dimensional echocardiography was positive but this finding was not confirmed either by computerised tomography or by left ventricular angiography. (author)

  7. Current status of ultrafast CT to evaluate the left ventricle

    International Nuclear Information System (INIS)

    MacMillan, R.M.; Rees, M.R.; Maranhao, V.

    1987-01-01

    Contrast-enhanced ultrafast CT with the cine mode can enable reliable assessment of left ventricular mass and function. Two oblique planar views, the long axis and short axis, are required. Right and left ventricular end-diastolic volumes, end-systolic volumes, stroke volume and ejection fraction, left ventricular segmental wall motion, and left ventricular mass can be measured. This exhibit emphasizes the methods and pitfalls in acquiring this information. Results from the authors' studies thus far to validate these measurements against reference standards are reviewed

  8. Extreme premature with persistent left superior vena cava.

    Science.gov (United States)

    Aboitiz-Rivera, Carlos Manuel; Blachman-Braun, Ruben; Parra-Pérez, Mariana Yazmin

    2017-10-01

    Persistent left superior vena cava (PLSVC) is a congenital anomaly, that results when there is an absence of the normal regression of the left common precardinal vein during embryogenesis. Usually, this anomaly remains asymptomatic, however, when the PLSVC drains into the left atrium this could lead to a right-to-left shunt. Additionally, this can result in inadvertent delivery of air or thrombus into the systemic circulation with potential neurologic, cardiac and renal complications. In this article, we present a case of an extreme premature Mexican newborn in which the diagnosis was made after placement of a percutaneous central venues catheter.

  9. Bilateral Persistent Sciatic Arteries Complicated with Acute Left Lower Limb Ischemia

    OpenAIRE

    Hsuan-Yin Wu; Yu-Jen Yang; Chao-Han Lai; Jun-Neng Roan; Chwan-Yau Luo; Chung-Dann Kan

    2007-01-01

    Persistent sciatic artery (PSA) is a rare congenital malformation. In the early embryonic stage, the sciatic artery is the major blood supply for the lower limb bulb and is later replaced by the iliofemoral artery as the limb develops. Its failure to regress, sometimes associated with femoral arterial hypoplasia, and therefore becoming the dominant inflow to the lower extremity is called PSA. This anomaly is often associated with a higher rate of aneurysm formation or thromboembolic complicat...

  10. Left atrial strain: a new parameter for assessment of left ventricular filling pressure.

    Science.gov (United States)

    Cameli, Matteo; Mandoli, Giulia Elena; Loiacono, Ferdinando; Dini, Frank Lloyd; Henein, Michael; Mondillo, Sergio

    2016-01-01

    In order to obtain accurate diagnosis, treatment and prognostication in many cardiac conditions, there is a need for assessment of left ventricular (LV) filling pressure. While systole depends on ejection function of LV, diastole and its disturbances influence filling function and pressures. The commonest condition that represents the latter is heart failure with preserved ejection fraction in which LV ejection is maintained, but diastole is disturbed and hence filling pressures are raised. Significant diastolic dysfunction results in raised LV end-diastolic pressure, mean left atrial (LA) pressure and pulmonary capillary wedge pressure, all referred to as LV filling pressures. Left and right heart catheterization has traditionally been used as the gold standard investigation for assessing these pressures. More recently, Doppler echocardiography has taken over such application because of its noninvasive nature and for being patient friendly. A number of indices are used to achieve accurate assessment of filling pressures including: LV pulsed-wave filling velocities (E/A ratio, E wave deceleration time), pulmonary venous flow (S wave and D wave), tissue Doppler imaging (E' wave and E/E' ratio) and LA volume index. LA longitudinal strain derived from speckle tracking echocardiography (STE) is also sensitive in estimating intracavitary pressures. It is angle-independent, thus overcomes Doppler limitations and provides highly reproducible measures of LA deformation. This review examines the application of various Doppler echocardiographic techniques in assessing LV filling pressures, in particular the emerging role of STE in assessing LA pressures in various conditions, e.g., HF, arterial hypertension and atrial fibrillation.

  11. Left-handedness and language lateralization in children.

    Science.gov (United States)

    Szaflarski, Jerzy P; Rajagopal, Akila; Altaye, Mekibib; Byars, Anna W; Jacola, Lisa; Schmithorst, Vincent J; Schapiro, Mark B; Plante, Elena; Holland, Scott K

    2012-01-18

    This fMRI study investigated the development of language lateralization in left- and righthanded children between 5 and 18 years of age. Twenty-seven left-handed children (17 boys, 10 girls) and 54 age- and gender-matched right-handed children were included. We used functional MRI at 3T and a verb generation task to measure hemispheric language dominance based on either frontal or temporo-parietal regions of interest (ROIs) defined for the entire group and applied on an individual basis. Based on the frontal ROI, in the left-handed group, 23 participants (85%) demonstrated left-hemispheric language lateralization, 3 (11%) demonstrated symmetric activation, and 1 (4%) demonstrated right-hemispheric lateralization. In contrast, 50 (93%) of the right-handed children showed left-hemispheric lateralization and 3 (6%) demonstrated a symmetric activation pattern, while one (2%) demonstrated a right-hemispheric lateralization. The corresponding values for the temporo-parietal ROI for the left-handed children were 18 (67%) left-dominant, 6 (22%) symmetric, 3 (11%) right-dominant and for the right-handed children 49 (91%), 4 (7%), 1 (2%), respectively. Left-hemispheric language lateralization increased with age in both groups but somewhat different lateralization trajectories were observed in girls when compared to boys. The incidence of atypical language lateralization in left-handed children in this study was similar to that reported in adults. We also found similar rates of increase in left-hemispheric language lateralization with age between groups (i.e., independent of handedness) indicating the presence of similar mechanisms for language lateralization in left- and right-handed children. Copyright © 2011 Elsevier B.V. All rights reserved.

  12. Left ventricular function in hypertrophic cardiomyopathy

    International Nuclear Information System (INIS)

    Takahashi, Hiromi; Yamaguchi, Ryutaro; Ifuku, Masayasu

    1985-01-01

    The present study was to investigate of left ventricular (LV) function during exercise in 26 patients with hypertrophic cardiomyopathy(HCM) usingTc-99m equilibrium angiocardiography, and to elucidate the mechanism of impaired functional reserve during exercise. In patients with HCM, LV ejection fraction decreased from 65 ± 8 (mean ± SD) % at rest to 59 ± 18 % at peak exercise, in contrast to an increase among controls (from 56 ± 9 % to 64 ± 9 %). As compared with resting values, cardiac output increased to 168 ± 24 % at peak exercise in HCM, but the increase was significantly less than that in controls (215 ± 47 %). Stroke volume decreased gradually to 83 ± 16 % during exercise in HCM, while it increased to 114 ± 10 % at an exercise level of half intensity, and it decreased slightly to 106 ± 16 % at peak exercise. LV end-systolic volume decreased among controls to 78 ± 27 % at peak exercise, but remained unchanged in HCM (118 ± 58 %). An increase in peak ejection rate at peak exercise was less in HCM than in controls (143 ± 26 % vs 170 ± 42 %). No significant differences were observed between the two groups concerning changes in indices of LV diastolic function including LV end-diastolic volume, peak filling rate or 1/3 filling rate during exercise. In the analysis of LV function curves, pulmonary arterial diastolic pressure increased to a greater extent in HCM than in controls (19 ± 6 mmHg vs 11 ± 6 mmHg); whereas, an increase in the stroke work index was less in HCM (80 ± 26 g.m/m 2 /beat vs 121 ± 21 g.m/m 2 /beat) at peak exercise. Thus, the LV function curve shifted downward and to the right in patients with HCM. The above findings indicate that LV functional reserve during exercise is impaired, especially as to systolic function in patients with HCM, while deterioration of diastolic function may be partly compromised by elevated filling pressure. (J.P.N.)

  13. Linear regression models for quantitative assessment of left ...

    African Journals Online (AJOL)

    Changes in left ventricular structures and function have been reported in cardiomyopathies. No prediction models have been established in this environment. This study established regression models for prediction of left ventricular structures in normal subjects. A sample of normal subjects was drawn from a large urban ...

  14. Impact of obstructive sleep apnea and snoring on left ventricular ...

    African Journals Online (AJOL)

    Background: Systemic hypertension (HTN) and obstructive sleep apnea (OSA) are individually associated with left ventricular structural and functional adaptations. However, little is known about the impact of OSA on the left ventricle in Africans with HTN. Aim: The aim of this study is to determine the association between ...

  15. Gender specific pattern of left ventricular cardiac adaptation to ...

    African Journals Online (AJOL)

    Background: Cardiac adaptation to hypertension and obesity may be related to many factors such as race, gender and haemodynamic status. Some gender specific associations with left ventricular structure and function have been described among Caucasians. Objectives: To describe the sex specific pattern of left ...

  16. Orthodontic intervention of an impacted upper left central incisor due ...

    African Journals Online (AJOL)

    A fixed orthodontic appliance was used to facilitate traction and correction of malalignement of the impacted upper left central incisor.Treatment outcome: Successful removal of the odontoma, full exposure of the crown of upper left central incisor and good alignment on the arch were achieved. Crowding on the upper and ...

  17. Diagnostic electrocardiographic dyad criteria of emphysema in left ventricular hypertrophy.

    Science.gov (United States)

    Lanjewar, Swapnil S; Chhabra, Lovely; Chaubey, Vinod K; Joshi, Saurabh; Kulkarni, Ganesh; Kothagundla, Chandrasekhar; Kaul, Sudesh; Spodick, David H

    2013-01-01

    The electrocardiographic diagnostic dyad of emphysema, namely a combination of the frontal vertical P-vector and a narrow QRS duration, can serve as a quasidiagnostic marker for emphysema, with specificity close to 100%. We postulated that the presence of left ventricular hypertrophy in emphysema may affect the sensitivity of this electrocardiographic criterion given that left ventricular hypertrophy generates prominent left ventricular forces and may increase the QRS duration. We reviewed the electrocardiograms and echocardiograms for 73 patients with emphysema. The patients were divided into two groups based on the presence or absence of echocardiographic evidence of left ventricular hypertrophy. The P-vector, QRS duration, and forced expiratory volume in one second (FEV1) were computed and compared between the two subgroups. There was no statistically significant difference in qualitative lung function (FEV1) between the subgroups. There was no statistically significant difference in mean P-vector between the subgroups. The mean QRS duration was significantly longer in patients with left ventricular hypertrophy as compared with those without left ventricular hypertrophy. The presence of left ventricular hypertrophy may not affect the sensitivity of the P-vector verticalization when used as a lone criterion for diagnosing emphysema. However, the presence of left ventricular hypertrophy may significantly reduce the sensitivity of the electrocardiographic diagnostic dyad in emphysema, as it causes a widening of the QRS duration.

  18. Left ventricular systolic function in sickle cell anaemia: an ...

    African Journals Online (AJOL)

    42) and 24.17 ± 4.39 ... Aortic root diameter (Ao), left atrial dimension (LAD), left ventricu- ... Key: *Statistically significant, a= Chi-square, SCA= sickle cell anaemia, SD= standard deviation. ..... of fluctuations in response to changes in sympathetic.

  19. Hemangioma of the left coronary artery: Diagnosis and treatment

    International Nuclear Information System (INIS)

    Just, A.; Wiesmann, W.; Peters, P.E.; Haesfeld, M.; Sciuk, J.

    1992-01-01

    The authors describe the case of a histologically proven hemangioma in the left coronary artery of the left ventricle of a 24-year-old woman. The results of the different medical imaging methods applied are described and the treatment of cardiac angiomas is discussed. (orig.) [de

  20. Radionuclide evaluation of left ventricular function with nonimaging probes.

    Science.gov (United States)

    Wexler, J P; Blaufox, M D

    1979-10-01

    Portable nonimaging probes have been developed that can evaluate left ventricular function using radionuclide techniques. Two modes of data acquisition are possible with these probe systems, first-pass and gated. Precordial radiocardiograms obtained after a bolus injection can be used to determine cardiac output, pulmonary transit time, pulmonary blood volume, left ventricle ejection fraction, and left-to-right shunts. Gated techniques can be used to determine left ventricular ejection fraction and sytolic time intervals. Probe-determined indices of left ventricular function agree excellently with comparable measurements determined by conventional camera-computer methods as well as by invasive techniques. These have begun to be used in a preliminary manner in a variety of clinical problems associated with left ventricular dysfunction. This review discusses the types of probe systems available, the methods used in positioning them, and details the specifics of their data acquisition and processing capacity. The major criticisms of probe methods are that they are nonimaging and that they measure global rather than regional left ventricular function. In spite of these criticisms, probe systems, because of their portability, high sensitivity, and relatively low cost are useful supplements to conventional camera-computer systems for the measurement of parameters of left ventricular performance using radionuclide techniques.

  1. Left subclavian artery revascularization as part of thoracic stent grafting

    NARCIS (Netherlands)

    Saouti, N.; Hindori, V.; Morshuis, W.J.; Heijmen, R.H.

    2015-01-01

    OBJECTIVES: Intentional covering of the left subclavian artery (LSA) as part of thoracic endovascular aortic repair (TEVAR) can cause (posterior) strokes or left arm malperfusion. LSA revascularization can be done as prophylaxis against, or as treatment of, these complications. We report our

  2. Reversible left ventricular dysfunction - important clinical problem of contemporary cardiology

    International Nuclear Information System (INIS)

    Witkowski, A.

    1994-01-01

    An important clinical issue there is determination whether left ventricular damages are reversible or not single photon emission computed tomography and positron computed tomography techniques are shown to provide valuable data in this problem. Article describes basic syndromes connected with left ventricular dysfunction, namely: hibernating myocardium, stunned myocardium and ischemic myocardium preconditioning. (author). 18 refs

  3. Evaluation of left ventricular function using digital subtraction angiography

    International Nuclear Information System (INIS)

    Kozuka, Takahiro; Ohta, Mitsushige

    1985-01-01

    To evaluate function of the left ventricle and myocardial perfusion images, digital subtraction angiography (DSA) was performed in 45 patients with ischemic heart disease. Validity of the technique was compared with data obtained from cine left ventriculogram in all patients and 201 T1 myocardial images in 20 patients. End-diastolic volume (EDV), end-systolic volume (ESV) and ejection fraction (EF) calculated from DSA were correlated closely with those from cine left ventriculogram (r = 0.92, r = 0.94 and r = 0.86, respectively). Regional contractility at the antero-lateral wall of the left ventricle, assessed by DSA, was also correlated well with cine left ventriculogram (r = 0.75). Evaluation of the inferior wall motion showed less correlation in both procedures (r = 0.68). Phase and amplitude analysis with the same technique with radionuclide cardiac angiography was successfully applied in left ventriculogram obtained by DSA. The procedure seems to be helpful for objective evaluation of the left ventricular wall motion. Myocardial perfusion image, obtained with modified Radtke's technique, showed good coincidence with 201 T1 images. Thus, DSA is applicable for evaluation of function of the left ventricle and myocardial perfusion in patients with ischemic heart disease. (author)

  4. The organisational and ideological development of Green Left

    NARCIS (Netherlands)

    Lucardie, Anthonie; Voerman, Gerrit; Botella, Joan; Ramiro, Luis

    2003-01-01

    In 1991 the Communist Party of the Netherlands (CPN) dissolved itself into the new organisation GreenLeft, founded in 1990. Thus, GreenLeft may be considered the major ‘post-communist party’ in the Netherlands. Yet it is not the only one. A minority of former CPN-members established in 1992 the New

  5. Urban School Principals and the "No Child Left Behind" Act

    Science.gov (United States)

    Gardiner, Mary E.; Canfield-Davis, Kathy; Anderson, Keith LeMar

    2009-01-01

    This exploratory study investigated how six practicing school principals responded to the requirements of the No Child Left Behind law (United States Congress Public Law 107-110, 2002, January, No Child Left Behind Act, http://www.ed.gov/policy/elsec/leg/esea02/107-110.pdf ) in light of the multicultural leadership demands presented by an urban…

  6. On Functional Inequalities Originating from Module Jordan Left Derivations

    OpenAIRE

    Kang Sheon-Young; Chang Ick-Soon; Kim Hark-Mahn

    2008-01-01

    Abstract We first examine the generalized Hyers-Ulam stability of functional inequality associated with module Jordan left derivation (resp., module Jordan derivation). Secondly, we study the functional inequality with linear Jordan left derivation (resp., linear Jordan derivation) mapping into the Jacobson radical.

  7. On Functional Inequalities Originating from Module Jordan Left Derivations

    Directory of Open Access Journals (Sweden)

    Kang Sheon-Young

    2008-01-01

    Full Text Available Abstract We first examine the generalized Hyers-Ulam stability of functional inequality associated with module Jordan left derivation (resp., module Jordan derivation. Secondly, we study the functional inequality with linear Jordan left derivation (resp., linear Jordan derivation mapping into the Jacobson radical.

  8. Transient left atrial dysfunction is a feature of Takotsubo syndrome

    DEFF Research Database (Denmark)

    Stiermaier, Thomas; Graf, Tobias; Möller, Christian

    2017-01-01

    BACKGROUND: Takotsubo syndrome (TTS) is characterized by a transient left and/or right ventricular dysfunction as a consequence of a distinctive pattern of regional wall motion abnormalities. However, a systematic evaluation of the left atrial (LA) function in patients with TTS is lacking. The ai...

  9. Right-Brained Kids in Left-Brained Schools

    Science.gov (United States)

    Hunter, Madeline

    1976-01-01

    Students who learn well through left hemisphere brain input (oral and written) have minimal practice in using the right hemisphere, while those who are more proficient in right hemisphere (visual) input processing are handicapped by having to use primarily their left brains. (MB)

  10. Evaluation of left ventricular volumes measured by magnetic resonance imaging

    DEFF Research Database (Denmark)

    Møgelvang, J; Thomsen, C; Mehlsen, J

    1986-01-01

    Left ventricular end-diastolic and end-systolic volumes were determined in 17 patients with different levels of left ventricular function by magnetic resonance imaging (MRI). A 1.5 Tesla Magnet was used obtaining ECG triggered single and multiple slices. Calculated cardiac outputs were compared...

  11. Benign Multicystic Mesothelioma in the Left Round Ligament: Case Report

    International Nuclear Information System (INIS)

    Bae, So Young; Yi, Boem Ha; Lee, Hae Kyung; Park, Seong Jin; Cho, Gyu Seok; Kwak, Jeong Ja

    2010-01-01

    Benign multicystic mesothelioma is a rare mesothelial lesion that forms multicystic masses in the upper abdomen, pelvis, and retroperitoneum. Most cases have a benign course. We present the ultrasound and MR findings of benign multicystic mesothelioma in the left round ligament, which caused a left inguinal hernia in a 46-year-old woman

  12. Benign Multicystic Mesothelioma in the Left Round Ligament: Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Bae, So Young; Yi, Boem Ha; Lee, Hae Kyung; Park, Seong Jin; Cho, Gyu Seok; Kwak, Jeong Ja [Soonchunhyang University Bucheon Hospital, Bucheon (Korea, Republic of)

    2010-02-15

    Benign multicystic mesothelioma is a rare mesothelial lesion that forms multicystic masses in the upper abdomen, pelvis, and retroperitoneum. Most cases have a benign course. We present the ultrasound and MR findings of benign multicystic mesothelioma in the left round ligament, which caused a left inguinal hernia in a 46-year-old woman.

  13. Effect of Left Ventricular Outflow Tract Obstruction on Left Atrial Mechanics in Hypertrophic Cardiomyopathy

    Directory of Open Access Journals (Sweden)

    Lynne K. Williams

    2015-01-01

    Full Text Available Left atrial (LA volumes are known to be increased in hypertrophic cardiomyopathy (HCM and are a predictor of adverse outcome. In addition, LA function is impaired and is presumed to be due to left ventricular (LV diastolic dysfunction as a result of hypertrophy and myocardial fibrosis. In the current study, we assess the incremental effect of outflow tract obstruction (and concomitant mitral regurgitation on LA function as assessed by LA strain. Patients with HCM (50 obstructive, 50 nonobstructive were compared to 50 normal controls. A subset of obstructive patients who had undergone septal myectomy was also studied. Utilising feature-tracking software applied to cardiovascular magnetic resonance images, LA volumes and functional parameters were calculated. LA volumes were significantly elevated and LA ejection fraction and strain were significantly reduced in patients with HCM compared with controls and were significantly more affected in patients with obstruction. LA volumes and function were significantly improved after septal myectomy. LVOT obstruction and mitral regurgitation appear to further impair LA mechanics. Septal myectomy results in a significant reduction in LA volumes, paralleled by an improvement in function.

  14. Topography of the accessory left gastric artery (ALGA) analyzed by CT angiography from the left hepatic artery

    International Nuclear Information System (INIS)

    Shioyama, Yasukazu; Takasaka, Isao; Onaya, Hiroaki

    2003-01-01

    To avoid gastric complications when we perform transcatheter treatment via left hepatic artery, we analyzed the topography of ALGA (accessory left gastric artery) by left hepatic arteriography and CT angiography from left hepatic artery. Six hundred seventy eight cases of CT angiography were performed between 1995 and 2000. Among them, selective left hepatic arteriography was done in 85 cases. We analyzed the frequency and the course of ALGA on the hepatic angiogram and CT angiogram. ALGA were identified in eighteen (21.2 %) of the 85 cases. We classified them into eleven cases of the proximal type and six cases of the distal type. When ALGA bifurcated from the left hepatic artery very close to the bifurcation of A2 (dorsolateral branch) and A3 (ventrolateral branch), we classified them as the distal type on hepatic angiogram. On the other hand, when ALGA bifurcated from the left hepatic artery apart from the bifurcation of A2 and A3 they were classified as the proximal type. In one rare case ALGA originated from the dorsolateral branch of the left hepatic artery. ALGA were classified as the distal and proximal types. Distal type of ALGA often overlapped dorsolateral branch of the left hepatic artery, and it was sometimes difficult to notice the existence of them. We should check the existence of ALGA on the arterial phase of dynamic CT before we plan to make a transcatheter treatment from the left hepatic artery. Then we can avoid gastric complications caused by a transcatheter treatment from the left hepatic artery. (author)

  15. Left ventricular lipoma with pseudoaneurysm-like appearance.

    Science.gov (United States)

    Kawarai, Shun-ichi; Yaginuma, Gen-ya; Abe, Kazuo; Hamasaki, Azumi; Ishikawa, Kazunori; Tanaka, Daizo

    2010-06-01

    We describe an extremely rare cardiac lipoma, with electrocardiographic changes in ST segments and T waves, suggesting ischemic heart disease. The abnormal electrocardiogram was identified during a routine physical examination of an asymptomatic 57-year-old man. Coronary angiography showed no stenotic or occlusive lesions in the coronary arteries except that the left anterior descending artery followed a winding course in the apical region. Left ventriculography revealed a pseudoaneurysm-like mass around the apical portion. Computed tomography and magnetic resonance imaging revealed a cardiac lipoma that communicated with the left ventricle and resembled a pseudoaneurysm. After complete resection of the lipoma and left ventricle patch plasty, the postoperative course was uneventful. Histologically, the tumor was an intramyocardiac lipoma with no evidence of malignancy. To our knowledge, we are the first to describe an intramyocardiac lipoma resembling a left ventricle pseudoaneurysm.

  16. Annular subvalvular left ventricular aneurysm in Bahia, Brazil.

    Science.gov (United States)

    Guimarães, A C; Filho, A S; Esteves, J P; Abreu, W N; Vinhaes, L A; de Almeida Souza, J A; Machado, A

    1976-10-01

    Two cases of left ventricular aneurysm, a 16-year-old black boy and a 23-year-old white girl, from Bahia, Brazil, are presented. In both patients there was enlargement of the cardiac silhouette and a prominent bulge of the left inferior border. On the right oblique view a ring of calcium at the ventricular opening of the aneurysms was visualized. A left ventriculogram showed a huge aneurysm in the first case and a bulge on the lateral wall of the left ventricle in the other. Cardiac catheterization showed a rise in left and right ventricular end-diastolic pressures and in the mean pulmonary artery pressure. In the first case the contour of the right ventricular pressure curve showed a restrictive pattern. The similarities of these aneurysms with the annular submitral type described in young black Africans are stressed.

  17. Left neglect dyslexia: Perseveration and reading error types.

    Science.gov (United States)

    Ronchi, Roberta; Algeri, Lorella; Chiapella, Laura; Gallucci, Marcello; Spada, Maria Simonetta; Vallar, Giuseppe

    2016-08-01

    Right-brain-damaged patients may show a reading disorder termed neglect dyslexia. Patients with left neglect dyslexia omit letters on the left-hand-side (the beginning, when reading left-to-right) part of the letter string, substitute them with other letters, and add letters to the left of the string. The aim of this study was to investigate the pattern of association, if any, between error types in patients with left neglect dyslexia and recurrent perseveration (a productive visuo-motor deficit characterized by addition of marks) in target cancellation. Specifically, we aimed at assessing whether different productive symptoms (relative to the reading and the visuo-motor domains) could be associated in patients with left spatial neglect. Fifty-four right-brain-damaged patients took part in the study: 50 out of the 54 patients showed left spatial neglect, with 27 of them also exhibiting left neglect dyslexia. Neglect dyslexic patients who showed perseveration produced mainly substitution neglect errors in reading. Conversely, omissions were the prevailing reading error pattern in neglect dyslexic patients without perseveration. Addition reading errors were much infrequent. Different functional pathological mechanisms may underlie omission and substitution reading errors committed by right-brain-damaged patients with left neglect dyslexia. One such mechanism, involving the defective stopping of inappropriate responses, may contribute to both recurrent perseveration in target cancellation, and substitution errors in reading. Productive pathological phenomena, together with deficits of spatial attention to events taking place on the left-hand-side of space, shape the manifestations of neglect dyslexia, and, more generally, of spatial neglect. Copyright © 2016 Elsevier Ltd. All rights reserved.

  18. Left ventricular mass in borderline hypertension assessed by echo cardiography

    International Nuclear Information System (INIS)

    Mezzasalma, L.; Ghione, S.; Palonebo, C.

    1989-01-01

    The relationship between clinical measurement of blood pressure (BP) and left ventricular hypertrophy in arterial hypertension appears to be weak in most studies. On the contrary, stronger correlations with target organ damage in general, and left ventricular hypertrophy in particular, have been reported for blood pressure measurements obtained by ambulatory monitoring; this finding may indicate a possible role for blood pressure response to naturally occurring stresses in determining left ventricular hypertrophy. Aim of this study was to investigate, in 18 patients with borderline arterial hypertension, the relationships between echocardiographically assessed left ventricular mass and, respectively, casual BP and BP responses to some standardized stress tests. Only three patients had a diastolic wall thickness of the interventricular septum and of the posterior wall ≥1.2 cm and none had a pathologically increased left ventricular mass index. The following statistically significant correlations were found: casual diastolic BP vs. left ventricular mass index (r=0.53, p<0.02), systolic BP response to bicycle exercise test vs. left ventricular mass index (r=0.55, p<0.05). Multiple regression analysis showed that almost fifty percent of the variability of left ventricular mass index could be predicted by these two BP measurements. These findings suggest that besides the chronically increased afterload, also the transient hypertensive responses to naturally occuring physical stresses may have a role in determining the extent of cardiac structural changes in borderline hypertensive patients. In addition, they indicate a direct relation between left ventricular mass and blood pressure levels also in borderline hypertension, as previously shown for established hypertension, despite the fact that left ventricular hypertrophy represents only an occasional finding in early stages of hypertension

  19. Increased prevalences of left-handedness and left-eye sighting dominance in individuals with Williams-Beuren syndrome

    NARCIS (Netherlands)

    J.W. van Strien (Jan)

    2005-01-01

    textabstractHandedness and eye sighting dominance were assessed in a sample of 50 individuals (25 male, 25 female; aged 5 – 38 years) with Williams-Beuren syndrome (WBS). The prevalences of left-handedness and left-eyedness were compared to the normative prevalences in the general population. We

  20. Left-right asymmetry in neutrino-produced hadron jets

    International Nuclear Information System (INIS)

    Ballagh, H.C.; Bingham, H.H.; Lawry, T.J.; Lys, J.; Lynch, G.R.; Sokoloff, M.D.; Stevenson, M.L.; Huson, F.R.; Schmidt, E.; Smart, W.; Treadwell, E.; Cence, R.J.; Harris, F.A.; Jones, M.D.; Koide, A.; Peters, M.W.; Peterson, V.Z.; Lubatti, H.J.; Moriyasu, K.; Wolin, E.

    1989-01-01

    In an experiment (E546) to study interactions of left-angle E right-angle=100 GeV and left-angle Q 2 right-angle=17 GeV 2 neutrinos in the Fermilab 15-foot bubble chamber, we have looked for a left-right asymmetry in the azimuthal angle φ of individual hadrons about the direction of the lepton momentum transfer (q vector). Significant asymmetry is found for forward positive hadrons; for x F >0.10, we find left-angle cosφ right-angle=-0.029±0.008, where x F is the Feynman x variable. Negative hadrons with x F >0.10 show no asymmetry, left-angle cosφ right-angle=0.004±0.011. A model which includes parton intrinsic transverse momentum k t reproduces the asymmetry of combined positive and negative hadrons with x F >0.10, left-angle cosφ right-angle=-0.018±0.0065, if left-angle k t 2 right-angle=0.065±0.024 GeV 2 /c 2 . But the model predicts almost equal asymmetries for positive and negative hadrons. The model also agrees poorly with the observed dependence on the kinematic variables x and Q 2 if the k t distribution is assumed to be independent of kinematic variables

  1. Anosognosia for hemiparesis after left-sided stroke.

    Science.gov (United States)

    Baier, Bernhard; Vucurevic, Goran; Müller-Forell, Wibke; Glassl, Oliver; Geber, Christian; Dieterich, Marianne; Karnath, Hans-Otto

    2014-12-01

    In patients with left-sided lesions, anosognosia for hemiparesis (AHP) seems to be a rare phenomenon. It has been discussed whether this rareness might be due to an inevitable bias due to language dysfunction and whether the left hemisphere's role for our self-awareness of motor actions thus is underestimated. By applying functional magnetic resonance imaging (fMRI) we examined whether patients with AHP following a left hemisphere stroke show a regular, left-sided or a reversed, right-sided lateralization of language functions. Only the former observation would argue for an original role of the left hemisphere in self-awareness about limb function. In a consecutive series of 44 acute left-sided stroke patients, only one patient (=2%) was identified showing AHP. In this case, we could verify by using fMRI that lateralization of AHP and spatial neglect on the one hand and of language functions on the other hand were reversed. The present single case observation thus argues against an original role of the left hemisphere in self-awareness about limb function. We discuss the data in the context of previous observations in the literature. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. Neglect severity after left and right brain damage.

    Science.gov (United States)

    Suchan, Julia; Rorden, Chris; Karnath, Hans-Otto

    2012-05-01

    While unilateral spatial neglect after left brain damage is undoubtedly less common than spatial neglect after a right hemisphere lesion, it is also assumed to be less severe. Here we directly test this latter hypothesis using a continuous measure of neglect severity: the so-called Center of Cancellation (CoC). Rorden and Karnath (2010) recently validated this index for right brain damaged neglect patients. A first aim of the present study was to evaluate this new measure for spatial neglect after left brain damage. In a group of 48 left-sided stroke patients with and without neglect, a score greater than -0.086 on the Bells Test and greater than -0.024 on the Letter Cancellation Task turned out to indicate neglect behavior for acute left brain damaged patients. A second aim was to directly compare the severity of spatial neglect after left versus right brain injury by using the new CoC measure. While neglect is less frequent following left than right hemisphere injury, we found that when this symptom occurs it is of similar severity in acute left brain injury as in patients after acute right brain injury. Copyright © 2012 Elsevier Ltd. All rights reserved.

  3. Left main coronary artery disease: pathophysiology, diagnosis, and treatment.

    Science.gov (United States)

    Collet, Carlos; Capodanno, Davide; Onuma, Yoshinobu; Banning, Adrian; Stone, Gregg W; Taggart, David P; Sabik, Joseph; Serruys, Patrick W

    2018-06-01

    The advent of coronary angiography in the 1960s allowed for the risk stratification of patients with stable angina. Patients with unprotected left main coronary artery disease have an increased risk of death related to the large amount of myocardium supplied by this vessel. Although coronary angiography remains the preferred imaging modality for the evaluation of left main coronary artery stenosis, this technique has important limitations. Angiograms of the left main coronary artery segment can be difficult to interpret, and almost one-third of patients can be misclassified when fractional flow reserve is used as the reference. In patients with clinically significant unprotected left main coronary artery disease, surgical revascularization was shown to improve survival compared with medical therapy and has been regarded as the treatment of choice for unprotected left main coronary artery disease. Two large-scale clinical trials published in 2016 support the usefulness of catheter-based revascularization in selected patients with unprotected left main coronary artery disease. In this Review, we describe the pathophysiology of unprotected left main coronary artery disease, discuss diagnostic approaches in light of new noninvasive and invasive imaging techniques, and detail risk stratification models to aid the Heart Team in the decision-making process for determining the best revascularization strategy for these patients.

  4. Left ventricular systolic and diastolic function in hyperthyroidism

    International Nuclear Information System (INIS)

    Friedman, M.J.; Okada, R.D.; Ewy, G.A.; Hellman, D.J.

    1982-01-01

    In order to assess the effect of hyperthyroidism on systolic and diastolic function of the left ventricle, M-mode echocardiograms and systolic time intervals were obtained in 13 patients while they were clinically hyperthyroid and again when they were euthyroid following radioactive iodine therapy. Echocardiographic tracings of the septum and left ventricular posterior wall were digitized and analyzed to provide the maximum velocity of shortening and maximum velocity of lengthening. These velocities were normalized for left ventricular diastolic dimension. The left ventricular minor axis fractional shortening and the normalized maximum velocity of shortening were both increased during the hyperthyroid state. The normalized maximum velocity of lengthening, a measure of diastolic left ventricular function, was also increased during the hyperthyroid state when compared to the euthyroid state. The preejection period index and the preejection period/left ventricular ejection time ratio were lower when the patients were hyperthyroid than when they were euthyroid. These data confirm the increased inotropic state and demonstrated increased diastolic relaxation velocities of the hyperthyroid left ventricle

  5. Fatal stroke after completion pneumonectomy for torsion of left upper lobe following left lower lobectomy

    Directory of Open Access Journals (Sweden)

    Apostolakis Efstratios

    2006-09-01

    Full Text Available Abstract Background The lobar torsion after lung surgery is a rare complication with an incidence of 0.09 to 0.4 %. It may occur after twisting of the bronchovascular pedicle of the remaining lobe after lobectomy, usually on the right side. The 180-degree rotation of the pedicle produces an acute obstruction of the lobar bronchus (atelectasis and of the lobar vessels as well. Without prompt treatment it progresses to lobar ischemia, pulmonary infarction and finally fatal gangrene. Case Presentation A 62 years old female patient was admitted for surgical treatment of lung cancer. She underwent elective left lower lobectomy for squamous cell carcinoma (pT2 N0. The operation was unremarkable, and the patient was extubated in the operating room. After eight hours the patient established decrease of pO2 and chest x-ray showed atelectasis of the lower lobe. To establish diagnosis, bronchoscopy was performed, demonstrating obstructed left lobar bronchus. The patient was re-intubated, and admitted to the operating room where reopening of the thoracotomy was performed. Lobar torsion was diagnosed, with the diaphragmatic surface of the upper lobe facing in an anterosuperior orientation. A completion pneumonectomy was performed. At the end of the procedure the patient developed a right pupil dilatation, presumably due to a cerebral embolism. A subsequent brain angio-CT scan established the diagnosis. She died at the intensive care unit 26 days later. Conclusion The thoracic surgeon should suspect this rare early postoperative complication after any thoracic operation in every patient with atelectasis of the neighboring lobe. High index of suspicion and prompt diagnosis may prevent catastrophic consequences, such as, infarction or gangrene of the pulmonary lobe. During thoracic operations, especially whenever the lung or lobe hilum is full mobilized, fixation of the remaining lobe may prevent this life threatening complication.

  6. Diagnostic electrocardiographic dyad criteria of emphysema in left ventricular hypertrophy

    Directory of Open Access Journals (Sweden)

    Lanjewar SS

    2013-11-01

    Full Text Available Swapnil S Lanjewar,1 Lovely Chhabra,1 Vinod K Chaubey,1 Saurabh Joshi,1 Ganesh Kulkarni,1 Chandrasekhar Kothagundla,1 Sudesh Kaul,1 David H Spodick21Department of Internal Medicine, 2Department of Cardiovascular Medicine, Saint Vincent Hospital, University of Massachusetts Medical School, Worcester, MA, USABackground: The electrocardiographic diagnostic dyad of emphysema, namely a combination of the frontal vertical P-vector and a narrow QRS duration, can serve as a quasidiagnostic marker for emphysema, with specificity close to 100%. We postulated that the presence of left ventricular hypertrophy in emphysema may affect the sensitivity of this electrocardiographic criterion given that left ventricular hypertrophy generates prominent left ventricular forces and may increase the QRS duration.Methods: We reviewed the electrocardiograms and echocardiograms for 73 patients with emphysema. The patients were divided into two groups based on the presence or absence of echocardiographic evidence of left ventricular hypertrophy. The P-vector, QRS duration, and forced expiratory volume in one second (FEV1 were computed and compared between the two subgroups.Results: There was no statistically significant difference in qualitative lung function (FEV1 between the subgroups. There was no statistically significant difference in mean P-vector between the subgroups. The mean QRS duration was significantly longer in patients with left ventricular hypertrophy as compared with those without left ventricular hypertrophy.Conclusion: The presence of left ventricular hypertrophy may not affect the sensitivity of the P-vector verticalization when used as a lone criterion for diagnosing emphysema. However, the presence of left ventricular hypertrophy may significantly reduce the sensitivity of the electrocardiographic diagnostic dyad in emphysema, as it causes a widening of the QRS duration.Keywords: emphysema, electrocardiogram, left ventricular hypertrophy, chronic

  7. Left-sided gall bladder: Report of two cases

    Directory of Open Access Journals (Sweden)

    Chrungoo R

    2007-01-01

    Full Text Available Left-sided gall bladder without situs inversus viscerum is a rare albeit recognized clinical entity. We report our experience of two cases of left-sided gall bladder in two women aged 36 and 48 who underwent laparoscopic cholecystectomy for chronic calculous cholecystitis. Left-sided gall bladder may provide an unusual surprise to the surgeons during laparoscopy as routine pre-operative studies may not always detect the anomaly. Awareness of the unpredictable confluence of the cystic duct into the common bile duct (CBD and selective use of intraoperative cholangiography aid in the safe laparoscopic management of this unusual entity.

  8. Primary left ventricular hydatid cyst in a child: case report

    International Nuclear Information System (INIS)

    Turkvatan, A.; Yelgec, N.S.; Calikoglu, U.; Olcer, T.

    2000-01-01

    The most common cause of echinococcosis in humans is Echinococcus granulosus. Although hydatid cyst is most frequently localized in liver (more than 65% of cases) and lung (25%) by means of portal and systemic circulation, it may involve other tissues and organs. Cardiac hydatid cysts account for only 0.5%-2% of all hydatid cysts, even in endemic areas. Of all cardiac hydatid cysts, the left ventricle accounts for 60%, right ventricle 10%, pericardium 7%, pulmonary artery 6%, left atrial appendage 6%, and interventricular septum 4%. We report the case of a myocardial hydatid cyst of the left ventricle in a 9-year-old boy. (author)

  9. Primary left ventricular hydatid cyst in a child: case report

    Energy Technology Data Exchange (ETDEWEB)

    Turkvatan, A. [Turkiye Yuksek Ihtisas Hospital, Dept. of Radiology, Ankara (Turkey); Yelgec, N.S. [Turkiye Yuksek Ihtisas Hospital, Dept. of Cardiology, Ankara (Turkey); Calikoglu, U.; Olcer, T. [Turkiye Yuksek Ihtisas Hospital, Dept. of Radiology, Ankara (Turkey)

    2000-12-01

    The most common cause of echinococcosis in humans is Echinococcus granulosus. Although hydatid cyst is most frequently localized in liver (more than 65% of cases) and lung (25%) by means of portal and systemic circulation, it may involve other tissues and organs. Cardiac hydatid cysts account for only 0.5%-2% of all hydatid cysts, even in endemic areas. Of all cardiac hydatid cysts, the left ventricle accounts for 60%, right ventricle 10%, pericardium 7%, pulmonary artery 6%, left atrial appendage 6%, and interventricular septum 4%. We report the case of a myocardial hydatid cyst of the left ventricle in a 9-year-old boy. (author)

  10. Heterotopic pancreatic tissue presenting as a solid and cystic lung lesion : A very unusual bronchopulmonary foregut malformation

    NARCIS (Netherlands)

    De Kruger, RR; Albers, MJIJ; Mooi, WJ; Bogers, J.J.C.

    2004-01-01

    We describe the history and lung pathology of a premature female infant, who presented with respiratory distress immediately after birth. A thoracic computerized tomography scan showed abnormalities suggestive of congenital cystic adenomatoid malformation of the left lung. In addition,

  11. Echocardiographic left ventricular masses in distance runners and weight lifters

    Science.gov (United States)

    Longhurst, J. C.; Gonyea, W. J.; Mitchell, J. H.; Kelly, A. R.

    1980-01-01

    The relationships of different forms of exercise training to left ventricular mass and body mass are investigated by echocardiographic studies of weight lifters, long-distance runners, and comparatively sized untrained control subjects. Left ventricular mass determinations by the Penn convention reveal increased absolute left ventricular masses in long-distance runners and competitive weight lifters with respect to controls matched for age, body weight, and body surface area, and a significant correlation between ventricular mass and lean body mass. When normalized to lean body mass, the ventricular masses of distance runners are found to be significantly higher than those of the other groups, suggesting that dynamic training elevates left ventricular mass compared to static training and no training, while static training increases ventricular mass only to the extent that lean body mass is increased.

  12. ["Habitual" left branch block alternating with 2 "disguised" bracnch block].

    Science.gov (United States)

    Lévy, S; Jullien, G; Mathieu, P; Mostefa, S; Gérard, R

    1976-10-01

    Two cases of alternating left bundle branch block and "masquerading block" (with left bundle branch morphology in the stnadard leads and right bundle branch block morphology in the precordial leads) were studied by serial tracings and his bundle electrocardiography. In case 1 "the masquerading" block was associated with a first degree AV block related to a prolongation of HV interval. This case is to our knowledge the first cas of alternating bundle branch block in which his bundle activity was recorded in man. In case 2, the patient had atrial fibrilation and His bundle recordings were performed while differents degrees of left bundle branch block were present: The mechanism of the alternation and the concept of "masquerading" block are discussed. It is suggested that this type of block represents a right bundle branch block associated with severe lesions of the "left system".

  13. Delusional misidentifications and duplications: right brain lesions, left brain delusions.

    Science.gov (United States)

    Devinsky, Orrin

    2009-01-06

    When the delusional misidentification syndromes reduplicative paramnesia and Capgras syndromes result from neurologic disease, lesions are usually bifrontal and/or right hemispheric. The related disorders of confabulation and anosognosis share overlapping mechanisms and anatomic pathology. A dual mechanism is postulated for the delusional misidentification syndromes: negative effects from right hemisphere and frontal lobe dysfunction as well as positive effects from release (i.e., overactivity) of preserved left hemisphere areas. Negative effects of right hemisphere injury impair self-monitoring, ego boundaries, and attaching emotional valence and familiarity to stimuli. The unchecked left hemisphere unleashes a creative narrator from the monitoring of self, memory, and reality by the frontal and right hemisphere areas, leading to excessive and false explanations. Further, the left hemisphere's cognitive style of categorization, often into dual categories, leads it to invent a duplicate or impostor to resolve conflicting information. Delusions result from right hemisphere lesions. But it is the left hemisphere that is deluded.

  14. Unusual metastasis of left colon cancer: considerations on two cases.

    Science.gov (United States)

    Gubitosi, Adelmo; Moccia, Giancarlo; Malinconico, Francesca Antonella; Gilio, Francesco; Iside, Giovanni; Califano, Umberto G A; Foroni, Fabrizio; Ruggiero, Roberto; Docimo, Giovanni; Parmeggiani, Domenico; Agresti, Massimo

    2009-04-01

    Usually, left colon cancer metastasis concerns liver, abdominal lymph nodes and lungs. Other localizations are quite rare occurrences. In spite of this, some uncommon metastasis sites are reported in literature, such as: peritoneum, ovaries, uterus, kidney testis, bones, thyroid, oral cavity and central nervous system. We report two cases of unusual localizations of left colon cancer metastasis localization, one into the retroperitoneal space and the other at the left axillary lynphnodes and between liver and pancreas. In the first reported case the diffusion pathway may have been the lymphatic mesocolic vessels, partially left in place from the previous surgery. In the second case the alleged metastatic lane may have been through the periumbilical lymph nodes to the parasternal lymph nodes and then to the internal mammary ones, finally reaching the axillary limph nodes.

  15. Sonographic determination of spleen to left kidney ratio among Igbo ...

    African Journals Online (AJOL)

    The weight and height of the subjects were obtained with the participants wearing light ... Results: Measurement of spleen and left kidney lengths were reliable within and between .... obtained in the coronal plane passing through the renal.

  16. Right and left ventricular performance by nuclear stethoscope

    International Nuclear Information System (INIS)

    Nishimura, T.; Uehara, T.; Hayashida, K.; Kozuka, T.

    1983-01-01

    Right and left ventricular performance by Nuclear Stethoscope using Kr-81m and Tc-99m-RBC were performed. And physiological and pharmacological interventions were also investigated for the evaluation of coronary artery diseases (CAD)

  17. Ostial left main coronary stenosis in a frequent flyer.

    LENUS (Irish Health Repository)

    O'Sullivan, John F

    2009-05-15

    A 52 year old gentleman presented with chest pain, after a long distance flight from India; he had made long haul flights every 2 weeks over the last 5 years as part of his job. His ECG revealed T wave inversion in leads V1-3. Cardiac biomarkers including troponin were negative; we proceeded to exercise stress testing (EST). This revealed 2 mm ST depression at 2 min of the standard Bruce protocol, associated with chest pain. He was taken immediately to the coronary catheterization laboratory; engagement of the left main caused pressure damping with 6 French, then 5 French diagnostic Judkins left 4 catheters. An ostial left main stenosis was seen; the right and left coronary trees otherwise had no significant stenoses. He had normal LV function. He underwent inpatient CABG 7 days later.

  18. Left-Right Asymmetric Morphogenesis in the Xenopus Digestive System

    Science.gov (United States)

    Muller, Jennifer K.; Prather, D.R.; Nascone-Yoder, N. M.

    2003-01-01

    The morphogenetic mechanisms by which developing organs become left-right asymmetric entities are unknown. To investigate this issue, we compared the roles of the left and right sides of the Xenopus embryo during the development of anatomic asymmetries in the digestive system. Although both sides contribute equivalently to each of the individual digestive organs, during the initial looping of the primitive gut tube, the left side assumes concave topologies where the right side becomes convex. Of interest, the concave surfaces of the gut tube correlate with expression of the LR gene, Pitx2, and ectopic Pitx2 mRNA induces ectopic concavities in a localized manner. A morphometric comparison of the prospective concave and convex surfaces of the gut tube reveals striking disparities in their rate of elongation but no significant differences in cell proliferation. These results provide insight into the nature of symmetry-breaking morphogenetic events during left-right asymmetric organ development. ?? 2003 Wiley-Liss, Inc.

  19. Schwannoma of the left brachial plexus mimicking a ...

    African Journals Online (AJOL)

    Schwannoma of the left brachial plexus mimicking a cervicomediastinal ... Her voice was hoarse but there was no eye signs suggestive of thyrotoxicosis. ... A presumptive diagnosis of thyroid carcinoma with retrosternal extension was made.

  20. Left coronary arterial blood flow: Noninvasive detection by Doppler US

    International Nuclear Information System (INIS)

    Gramiak, R.; Holen, J.; Moss, A.J.; Gutierrez, O.H.; Picone, A.L.; Roe, S.A.

    1986-01-01

    Continuous wave (CW) and pulsed Doppler ultrasound studies with spectral analysis were used to detect the left coronary arterial blood flow in patients who were undergoing routine echocardiography. The pulmonary artery is a stable ultrasonic landmark from which detection of the blood flow can be effected. The left coronary artery can be distinguished by its blood flow toward the cardiac apex and by specific, functional flow features. Flow patterns vary among the left main, circumflex, and anterior descending arteries; patterns also vary with respiration cycles. In the present study, coronary arterial blood flow was detected in 58 of 70 patients (83%). Findings were validated by selectively injecting an agitated saline contrast medium into the left coronary artery and, in another study, by comparing human Doppler phasic flow waveforms with electromagnetic flowmeter recordings obtained in dogs

  1. The Colombian Left: A Paradoxical Past; A Promising Future?

    Directory of Open Access Journals (Sweden)

    Charles Bergquist

    2017-07-01

    Full Text Available The essay explores the paradoxical history of the left in Colombia: how and why one of the weakest lefts in Latin America brought about the strongest and most lasting Marxist insurrection in the hemisphere in the decades following the Cuban Revolution. The article argues that the terms of this paradox are related, that the historic weakness of the left partly explains the force and longevity of revolutionary guerrillas, and that said paradox helps clarify not only the failure of several attempts to achieve a negotiated settlement of the armed conflict, but also the negative vote in the October 2016 plebiscite. Finally, it envisions a more promising future for the country’s left, provided that a lasting peace is consolidated.

  2. Times of Crisis : Success or Decline of the Left

    Directory of Open Access Journals (Sweden)

    Valeria Tarditi

    2015-03-01

    Full Text Available The crisis that especially since the 2008 has shown the deep weakness of the capitalistic economic model induces to reflect on the role of the radical left parties and on their capacity to propose alternative policies, interpreting the citizens’ demands. Starting from these premises this article analyses three parties of the radical left party family- United Left, Left Freedom and Ecology and Syriza- that act respectively in Spain, Italy and Greece, three of the European democracies most affected by the social consequences of the crisis. The article proposes a diachronic comparison of the three parties’ policy pro-posals, organizational model and strategies in order to explain their greater or lesser ability to attract con-sensus in the last years.

  3. Phonological decisions require both the left and right supramarginal gyri

    DEFF Research Database (Denmark)

    Hartwigsen, Gesa; Baumgaertner, Annette; Price, Cathy J

    2010-01-01

    Recent functional imaging studies demonstrated that both the left and right supramarginal gyri (SMG) are activated when healthy right-handed subjects make phonological word decisions. However, lesion studies typically report difficulties with phonological processing after left rather than right...... the right or left SMG. Taken together, these findings provide converging evidence that the right SMG contributes to accurate and efficient phonological decisions in the healthy brain, with no evidence that the left and right SMG can compensate for one another during TMS. Our findings motivate detailed...... hemisphere damage. Here, we used a unique dual-site transcranial magnetic stimulation (TMS) approach to test whether the SMG in the right hemisphere contributes to modality-independent (i.e., auditory and visual) phonological decisions. To test task-specificity, we compared the effect of real or sham TMS...

  4. Contemporary Assessment of Left Ventricular Diastolic Function in Older Adults

    DEFF Research Database (Denmark)

    Shah, Amil M.; Claggett, Brian; Kitzman, Dalane

    2017-01-01

    Background: Although age-Associated changes in left ventricular diastolic function are well recognized, limited data exist characterizing measures of diastolic function in older adults, including both reference ranges reflecting the older adult population and prognostically relevant values...

  5. Left-sided native valve Staphylococcus aureus endocarditis

    NARCIS (Netherlands)

    Slabbekoorn, M.; Horlings, H. M.; van der Meer, J. T. M.; Windhausen, A.; Van der Sloot, J. A. P.; Lagrand, W. K.

    2010-01-01

    Despite improved diagnostic tools and expanded treatment options, left-sided native valve endocarditis caused by Staphylococcus aureus infection remains a serious and destructive disease. The high morbidity and mortality, however, can be reduced by early recognition, correct diagnosis, and

  6. Left ventricular structure and function in black normotensive type 2 ...

    African Journals Online (AJOL)

    Keywords: Black normotensive patients, left ventricular function, type 2 DM. Résumé ... sickle cell disease and structural heart disease were excluded ... Pulmonary venous flow (PVF) velocity ... had abnormal ECG pattern compared with 30%.

  7. Fetal Diagnosis of Abnormal Origin of the Left Pulmonary Artery.

    Science.gov (United States)

    Tretter, Justin T; Tretter, Eric M; Rafii, Daniela Y; Anderson, Robert H; Bhatla, Puneet

    2016-08-01

    Isolated anomalies of the branch pulmonary arteries are rare, more often occurring in the setting of complex congenital heart disease. These isolated anomalies are often not identified in the prenatal period. We describe two cases of isolated anomalies of the left pulmonary artery which were identified on fetal echocardiography and confirmed postnatally, an anomalous left pulmonary artery arising from the base of the left-sided brachiocephalic artery in the setting of a right-sided aortic arch, and a left pulmonary artery sling. These two cases support our current understanding of normal and abnormal development of the extrapericardial arterial vessels and highlight the importance of meticulous attention when sweeping from the three-vessel tracheal view. © 2016, Wiley Periodicals, Inc.

  8. Quantitation of global and regional left ventricular function by MRI

    NARCIS (Netherlands)

    van der Geest, RJ; Reiber, JHC; Reiber, JHC; VanDerWall, EE

    1998-01-01

    Magnetic resonance imaging (MRI) provides several imaging strategies for assessing left ventricular function. As a three-dimensional imaging technique, all measurements can be performed without relying on geometrical assumptions. Global and regional function parameters can be derived from

  9. Left-handed materials in metallic magnetic granular composites

    International Nuclear Information System (INIS)

    Chui, S.T.; Lin, Z.F.; Hu, L.-B.

    2003-01-01

    There is recently interests in the 'left-handed' materials. In these materials the direction of the wave vector of electromagnetic radiation is opposite to the direction of the energy flow. We present simple arguments that suggests that magnetic composites can also be left-handed materials. However, the physics involved seems to be different from the original argument. In our argument, the imaginary part of the dielectric constant is much larger than the real part, opposite to the original argument

  10. Political Left and Right: Our Hands-On Logic

    OpenAIRE

    Frits Bienfait; Walter E. A. van Beek

    2014-01-01

    The origins and immediate vitality of the left/right divide which emerged in French revolutionary politics from 1789 can only be understood against the background of a much older classification dynamic based on the primacy of the right hand, first described by Robert Hertz in 1909. This dynamic infused political thinking first in Versailles and since 1815 in democracies throughout the world. In the process, the classical left/right polarity acquired a new dimension: the complementary notions ...

  11. Jordan and left derivations on locally C*-algebras

    International Nuclear Information System (INIS)

    Shahzad, Naseer

    2002-07-01

    We show that left derivations as well as Jordan derivations on locally G*-algebras are always continuous. We also obtain some noncommutative extensions of the classical Singer-Wermer theorem for locally C*-algebras: (1) Every left derivation D on a locally (7*-algebra A is identically zero. (2) Every Jordan derivation D on a locally C*-algebra A which satisfies [D(x), x]D(x]=0 for all x in A, is identically zero. (author)

  12. Left Atrium Papillary Fibroelastomas: A Cause of Cerebral Emboli

    Directory of Open Access Journals (Sweden)

    A. G. Ciss

    2012-01-01

    Full Text Available Papillary fibroelastomas are cardiac benign tumours. Among the benign cardiac tumor, papillary fibroelastomas are reported second after myxomas. Most often diagnosed incidentally, papillary fibroelastomas may embolize to cerebral circulation. Valvular locations are predominant; location in left atrium is rare. In this paper, we present a case of papillary fibroelastoma located in left atrium with symptoms of cerebral embolization. Transoesophageal echocardiography diagnosed a mobile mass. The patient was treated with surgical resection without further embolic complication.

  13. Tensor product of left n-invertible operators

    Czech Academy of Sciences Publication Activity Database

    Duggal, B.P.; Müller, Vladimír

    2013-01-01

    Roč. 215, č. 2 (2013), s. 113-125 ISSN 0039-3223 R&D Projects: GA ČR GA201/09/0473 Institutional support: RVO:67985840 Keywords : Banach space * essentially left n- invertible operator * left n-invertible operator Subject RIV: BA - General Mathematics Impact factor: 0.630, year: 2013 http://journals.impan.pl/cgi-bin/doi?sm215-2-2

  14. Developmental dyslexia: dysfunction of a left hemisphere reading network

    Directory of Open Access Journals (Sweden)

    Fabio eRichlan

    2012-05-01

    Full Text Available This mini-review summarizes and integrates findings from recent meta-analyses and original neuroimaging studies on functional brain abnormalities in dyslexic readers. Surprisingly, there is little empirical support for the standard neuroanatomical model of developmental dyslexia, which localizes the primary phonological decoding deficit in left temporo-parietal regions. Rather, recent evidence points to a dysfunction of a left hemisphere reading network, which includes occipito-temporal, inferior frontal, and inferior parietal regions.

  15. Right Site, Wrong Route - Cannulating the Left Internal Jugular Vein.

    Science.gov (United States)

    Paik, Peter; Arukala, Sanjay K; Sule, Anupam A

    2018-01-09

    Central venous catheters are placed in approximately five million patients annually in the US. The preferred site of insertion is one with fewer risks and easier access. Although the right internal jugular vein is preferred, on occasion, the left internal jugular may have to be accessed. A patient was admitted for septic shock, cerebrovascular accident, and non-ST-segment elevation myocardial infarction. A central venous line was needed for antibiotic and vasopressor administration. Due to trauma from a fall to the right side and previously failed catheterization attempts at the left subclavian and femoral veins, the left internal jugular vein was accessed. On chest radiography for confirmation, the left internal jugular central venous catheter was seen projecting down the left paraspinal region. It did not take the expected course across the midline toward the right and into the superior vena cava (SVC). A review of a computed tomography (CT) scan of the chest with contrast done on a prior admission revealed a duplicated SVC on the left side that had not been reported in the original CT scan interpretation. A left-sided SVC is present in approximately 0.3% to 0.5% of the population, with 90% of these draining into the coronary sinus. During placements of central venous lines and pacemakers, irritation of the coronary sinus may result in hypotension, arrhythmia, myocardial ischemia, or cardiac arrest. A widened mediastinum can be an indication of a duplicated SVC. When attempting a left internal jugular vein central venous catheter placement, it is important to be aware of venous anomalies in order to prevent complications.

  16. Variation of left heart function and thyroid hormone in cirrhosis

    International Nuclear Information System (INIS)

    Liu Junchi; Du Sujun; Mao Shaorong; Lu Jun; Tang Xiaoling

    1996-01-01

    The observation of 52 cases of cirrhosis left heart function indices traced with 113 In m in quiet and motion, and the combining determination of thyroid hormone serum levels with RIA are described. The cirrhosis to the function of liver damage increased, the indices of left heart function has great change. Moreover, the serum levels of T 3 , FT 3 in thyroid hormone are decreased obviously and the serum level of rT 3 is also increased significantly

  17. Weak interaction models with spontaneously broken left-right symmetry

    International Nuclear Information System (INIS)

    Mohapatra, R.H.

    1978-01-01

    The present status of weak interaction models with spontaneously broken left-right symmetry is reviewed. The theoretical basis for asymptotic parity conservation, manifest left-right symmetry in charged current weak interactions, natural parity conservation in neutral currents and CP-violation in the context of SU(2)/sub L/ circled x SU (2)/sub R/ circled x U(1) models are outlined in detail. Various directions for further research in the theoretical and experimental side are indicated

  18. Automatic extraction of left ventricle in SPECT myocardial perfusion imaging

    International Nuclear Information System (INIS)

    Liu Li; Zhao Shujun; Yao Zhiming; Wang Daoyu

    1999-01-01

    An automatic method of extracting left ventricle from SPECT myocardial perfusion data was introduced. This method was based on the least square analysis of the positions of all short-axis slices pixels from the half sphere-cylinder myocardial model, and used a iterative reconstruction technique to automatically cut off the non-left ventricular tissue from the perfusion images. Thereby, this technique provided the bases for further quantitative analysis

  19. Foucault's Flirt? Neoliberalism, the Left and the Welfare State

    DEFF Research Database (Denmark)

    Hansen, Magnus Paulsen

    2015-01-01

    An essay on the review of works interpreting the lectures of Michel Foucault on neoliberalism is presented. It offers a history of neoliberalism as a way to reinvent the left and examines the way Foucault betrayed the left while resisting to be connected with a normative political programme...... or principle for a neoliberal stance. The author relates the risks in neoliberal thought for losing what is specific in definitive statements and the loss of neoliberal thought coherence within a reality....

  20. Giant left atrium encountered during right-sided thoracentesis

    Directory of Open Access Journals (Sweden)

    Rashmi Advani

    Full Text Available Giant left atrium is an uncommon pathology to encounter during bedside chest ultrasound, but is an important structure to be aware of when considering thoracentesis. This cardiac structure could easily be mistaken for loculated pleural fluid. This case also supports growing evidence that expert users can safely perform thoracentesis without completely reversing therapeutic anticoagulation. Keywords: Giant left atrium, Thoracentesis, Rheumatic heart disease, Ultrasound

  1. Impact of Atrial Fibrillation Ablation on Left Ventricular Filling Pressure and Left Atrial Remodeling

    Directory of Open Access Journals (Sweden)

    Simone Nascimento dos Santos

    2014-12-01

    Full Text Available Background: Left ventricular (LV diastolic dysfunction is associated with new-onset atrial fibrillation (AF, and the estimation of elevated LV filling pressures by E/e' ratio is related to worse outcomes in patients with AF. However, it is unknown if restoring sinus rhythm reverses this process. Objective: To evaluate the impact of AF ablation on estimated LV filling pressure. Methods: A total of 141 patients underwent radiofrequency (RF ablation to treat drug-refractory AF. Transthoracic echocardiography was performed 30 days before and 12 months after ablation. LV functional parameters, left atrial volume index (LAVind, and transmitral pulsed and mitral annulus tissue Doppler (e' and E/e' were assessed. Paroxysmal AF was present in 18 patients, persistent AF was present in 102 patients, and long-standing persistent AF in 21 patients. Follow-up included electrocardiographic examination and 24-h Holter monitoring at 3, 6, and 12 months after ablation. Results: One hundred seventeen patients (82.9% were free of AF during the follow-up (average, 18 ± 5 months. LAVind reduced in the successful group (30.2 mL/m2 ± 10.6 mL/m2 to 22.6 mL/m2 ± 1.1 mL/m2, p < 0.001 compared to the non-successful group (37.7 mL/m2 ± 14.3 mL/m2 to 37.5 mL/m2 ± 14.5 mL/m2, p = ns. Improvement of LV filling pressure assessed by a reduction in the E/e' ratio was observed only after successful ablation (11.5 ± 4.5 vs. 7.1 ± 3.7, p < 0.001 but not in patients with recurrent AF (12.7 ± 4.4 vs. 12 ± 3.3, p = ns. The success rate was lower in the long-standing persistent AF patient group (57% vs. 87%, p = 0.001. Conclusion: Successful AF ablation is associated with LA reverse remodeling and an improvement in LV filling pressure.

  2. Impact of Atrial Fibrillation Ablation on Left Ventricular Filling Pressure and Left Atrial Remodeling

    Energy Technology Data Exchange (ETDEWEB)

    Santos, Simone Nascimento dos, E-mail: simonens@cardiol.br [Instituto Brasília de Arritmia- Universidade de Brasília, DF (Brazil); Faculdade de Medicina (UnB), Brasília, DF (Brazil); Henz, Benhur Davi; Zanatta, André Rodrigues; Barreto, José Roberto; Loureiro, Kelly Bianca; Novakoski, Clarissa; Santos, Marcus Vinícius Nascimento dos; Giuseppin, Fabio F.; Oliveira, Edna Maria; Leite, Luiz Roberto [Instituto Brasília de Arritmia- Universidade de Brasília, DF (Brazil)

    2014-12-15

    Left ventricular (LV) diastolic dysfunction is associated with new-onset atrial fibrillation (AF), and the estimation of elevated LV filling pressures by E/e' ratio is related to worse outcomes in patients with AF. However, it is unknown if restoring sinus rhythm reverses this process. To evaluate the impact of AF ablation on estimated LV filling pressure. A total of 141 patients underwent radiofrequency (RF) ablation to treat drug-refractory AF. Transthoracic echocardiography was performed 30 days before and 12 months after ablation. LV functional parameters, left atrial volume index (LAVind), and transmitral pulsed and mitral annulus tissue Doppler (e' and E/e') were assessed. Paroxysmal AF was present in 18 patients, persistent AF was present in 102 patients, and long-standing persistent AF in 21 patients. Follow-up included electrocardiographic examination and 24-h Holter monitoring at 3, 6, and 12 months after ablation. One hundred seventeen patients (82.9%) were free of AF during the follow-up (average, 18 ± 5 months). LAVind reduced in the successful group (30.2 mL/m{sup 2} ± 10.6 mL/m{sup 2} to 22.6 mL/m{sup 2} ± 1.1 mL/m{sup 2}, p < 0.001) compared to the non-successful group (37.7 mL/m{sup 2} ± 14.3 mL/m{sup 2} to 37.5 mL/m{sup 2} ± 14.5 mL/m{sup 2}, p = ns). Improvement of LV filling pressure assessed by a reduction in the E/e' ratio was observed only after successful ablation (11.5 ± 4.5 vs. 7.1 ± 3.7, p < 0.001) but not in patients with recurrent AF (12.7 ± 4.4 vs. 12 ± 3.3, p = ns). The success rate was lower in the long-standing persistent AF patient group (57% vs. 87%, p = 0.001). Successful AF ablation is associated with LA reverse remodeling and an improvement in LV filling pressure.

  3. Impact of Atrial Fibrillation Ablation on Left Ventricular Filling Pressure and Left Atrial Remodeling

    International Nuclear Information System (INIS)

    Santos, Simone Nascimento dos; Henz, Benhur Davi; Zanatta, André Rodrigues; Barreto, José Roberto; Loureiro, Kelly Bianca; Novakoski, Clarissa; Santos, Marcus Vinícius Nascimento dos; Giuseppin, Fabio F.; Oliveira, Edna Maria; Leite, Luiz Roberto

    2014-01-01

    Left ventricular (LV) diastolic dysfunction is associated with new-onset atrial fibrillation (AF), and the estimation of elevated LV filling pressures by E/e' ratio is related to worse outcomes in patients with AF. However, it is unknown if restoring sinus rhythm reverses this process. To evaluate the impact of AF ablation on estimated LV filling pressure. A total of 141 patients underwent radiofrequency (RF) ablation to treat drug-refractory AF. Transthoracic echocardiography was performed 30 days before and 12 months after ablation. LV functional parameters, left atrial volume index (LAVind), and transmitral pulsed and mitral annulus tissue Doppler (e' and E/e') were assessed. Paroxysmal AF was present in 18 patients, persistent AF was present in 102 patients, and long-standing persistent AF in 21 patients. Follow-up included electrocardiographic examination and 24-h Holter monitoring at 3, 6, and 12 months after ablation. One hundred seventeen patients (82.9%) were free of AF during the follow-up (average, 18 ± 5 months). LAVind reduced in the successful group (30.2 mL/m 2 ± 10.6 mL/m 2 to 22.6 mL/m 2 ± 1.1 mL/m 2 , p < 0.001) compared to the non-successful group (37.7 mL/m 2 ± 14.3 mL/m 2 to 37.5 mL/m 2 ± 14.5 mL/m 2 , p = ns). Improvement of LV filling pressure assessed by a reduction in the E/e' ratio was observed only after successful ablation (11.5 ± 4.5 vs. 7.1 ± 3.7, p < 0.001) but not in patients with recurrent AF (12.7 ± 4.4 vs. 12 ± 3.3, p = ns). The success rate was lower in the long-standing persistent AF patient group (57% vs. 87%, p = 0.001). Successful AF ablation is associated with LA reverse remodeling and an improvement in LV filling pressure

  4. Skin Sodium Concentration Correlates with Left Ventricular Hypertrophy in CKD.

    Science.gov (United States)

    Schneider, Markus P; Raff, Ulrike; Kopp, Christoph; Scheppach, Johannes B; Toncar, Sebastian; Wanner, Christoph; Schlieper, Georg; Saritas, Turgay; Floege, Jürgen; Schmid, Matthias; Birukov, Anna; Dahlmann, Anke; Linz, Peter; Janka, Rolf; Uder, Michael; Schmieder, Roland E; Titze, Jens M; Eckardt, Kai-Uwe

    2017-06-01

    The pathogenesis of left ventricular hypertrophy in patients with CKD is incompletely understood. Sodium intake, which is usually assessed by measuring urinary sodium excretion, has been inconsistently linked with left ventricular hypertrophy. However, tissues such as skin and muscle may store sodium. Using 23 sodium-magnetic resonance imaging, a technique recently developed for the assessment of tissue sodium content in humans, we determined skin sodium content at the level of the calf in 99 patients with mild to moderate CKD (42 women; median [range] age, 65 [23-78] years). We also assessed total body overhydration (bioimpedance spectroscopy), 24-hour BP, and left ventricular mass (cardiac magnetic resonance imaging). Skin sodium content, but not total body overhydration, correlated with systolic BP ( r =0.33, P =0.002). Moreover, skin sodium content correlated more strongly than total body overhydration did with left ventricular mass ( r =0.56, P skin sodium content is a strong explanatory variable for left ventricular mass, unaffected by BP and total body overhydration. In conclusion, we found skin sodium content to be closely linked to left ventricular mass in patients with CKD. Interventions that reduce skin sodium content might improve cardiovascular outcomes in these patients. Copyright © 2017 by the American Society of Nephrology.

  5. Enhancing No Child Left Behind-School mental health connections.

    Science.gov (United States)

    Daly, Brian P; Burke, Robert; Hare, Isadora; Mills, Carrie; Owens, Celeste; Moore, Elizabeth; Weist, Mark D

    2006-11-01

    The No Child Left Behind Act of 2001 was signed into law by President George W. Bush in January 2002 and is regarded as the most significant federal education policy initiative in a generation. The primary focus of the No Child Left Behind Act is on promoting educational success for all children; however, the legislation also contains opportunities to advance school-based mental health. Unfortunately, the complexities of the provisions of the No Child Left Behind Act have made it difficult for educators, stakeholders, and mental health professionals to understand the legal and practical interface between No Child Left Behind and the school mental health movement. Therefore, the goals of this article are to (1) raise awareness about the challenges educators and school mental health professionals face as a result of the implementation of No Child Left Behind and (2) provide ideas and recommendations to advance the interface between No Child Left Behind and school mental health, which will support key provisions of the act and the growth of the field.

  6. Temporal order processing of syllables in the left parietal lobe.

    Science.gov (United States)

    Moser, Dana; Baker, Julie M; Sanchez, Carmen E; Rorden, Chris; Fridriksson, Julius

    2009-10-07

    Speech processing requires the temporal parsing of syllable order. Individuals suffering from posterior left hemisphere brain injury often exhibit temporal processing deficits as well as language deficits. Although the right posterior inferior parietal lobe has been implicated in temporal order judgments (TOJs) of visual information, there is limited evidence to support the role of the left inferior parietal lobe (IPL) in processing syllable order. The purpose of this study was to examine whether the left inferior parietal lobe is recruited during temporal order judgments of speech stimuli. Functional magnetic resonance imaging data were collected on 14 normal participants while they completed the following forced-choice tasks: (1) syllable order of multisyllabic pseudowords, (2) syllable identification of single syllables, and (3) gender identification of both multisyllabic and monosyllabic speech stimuli. Results revealed increased neural recruitment in the left inferior parietal lobe when participants made judgments about syllable order compared with both syllable identification and gender identification. These findings suggest that the left inferior parietal lobe plays an important role in processing syllable order and support the hypothesized role of this region as an interface between auditory speech and the articulatory code. Furthermore, a breakdown in this interface may explain some components of the speech deficits observed after posterior damage to the left hemisphere.

  7. Preterm Delivery in the Setting of Left Calyceal Rupture

    Directory of Open Access Journals (Sweden)

    Brent Hanson

    2015-01-01

    Full Text Available Spontaneous rupture of the renal collecting system is a rare but serious complication of pregnancy. We report a case of nontraumatic left renal calyceal rupture in a pregnancy which ultimately progressed to preterm delivery. A 29-year-old primigravida with a remote history of urolithiasis presented with left flank pain, suprapubic pain, and signs of preterm labor at 33 weeks of gestation. The patient was believed to have urolithiasis, although initial renal ultrasound failed to demonstrate definitive calculi. After a temporary improvement in flank pain with medication, the patient experienced acute worsening of her left flank pain. Urology was consulted and further imaging was obtained. Magnetic resonance imaging (MRI was consistent with bilateral hydronephrosis and rupture of the left renal calyx. Given the patient’s worsening pain in the setting of left calyceal rupture, the urology team planned for placement of a left ureteral stent. However, before the patient could receive her stent, she progressed to active labor and delivered a viable female infant vaginally. Following delivery, the patient’s flank pain resolved rapidly and spontaneously, so no surgical intervention was performed. A summary of the literature and the details of this specific clinical situation are provided.

  8. The new left and democracy in Latin America

    Directory of Open Access Journals (Sweden)

    Francisco Panizza

    2009-05-01

    Full Text Available Without embarking on a theoretical debate over the meaning of the term, it is obvious that the left is on the rise in Latin America. However, we should not ignore the fact that the coming to power of left-wing and centre-left parties and movements presents a certain amount of paradoxes and questions. One of the most important of the many paradoxes is the fact that, in spite of the leftwards electoral shift in the region, there is no evidence that the electorate of Latin America has moved to the left in a significant manner. As for the questions, these concern the connection between left-wing governments and democracy, and especially the future challenges that these governments must face to preserve and develop democracy. In an attempt to unravel the paradox and answer these questions, this article examines the roots, contexts and political challenges of left-wing governments in Latin America. To this end, the author analyses a number of issues, such as the tensions between different logics of political representation and their implications for democracy, in addition to discussing the conditions under which the tensions between the different logics of political representation can contribute (or not to the developing of democracy in the region.

  9. Phonological decisions require both the left and right supramarginal gyri.

    Science.gov (United States)

    Hartwigsen, Gesa; Baumgaertner, Annette; Price, Cathy J; Koehnke, Maria; Ulmer, Stephan; Siebner, Hartwig R

    2010-09-21

    Recent functional imaging studies demonstrated that both the left and right supramarginal gyri (SMG) are activated when healthy right-handed subjects make phonological word decisions. However, lesion studies typically report difficulties with phonological processing after left rather than right hemisphere damage. Here, we used a unique dual-site transcranial magnetic stimulation (TMS) approach to test whether the SMG in the right hemisphere contributes to modality-independent (i.e., auditory and visual) phonological decisions. To test task-specificity, we compared the effect of real or sham TMS during phonological, semantic, and perceptual decisions. To test laterality and anatomical specificity, we compared the effect of TMS over the left, right, or bilateral SMG and angular gyri. The accuracy and reaction times of phonological decisions were selectively disrupted relative to semantic and perceptual decisions when real TMS was applied over the left, right, or bilateral SMG. These effects were not observed for TMS over the angular gyri. A follow-up experiment indicated that the threshold-intensity for inducing a disruptive effect on phonological decisions was identical for unilateral TMS over the right or left SMG. Taken together, these findings provide converging evidence that the right SMG contributes to accurate and efficient phonological decisions in the healthy brain, with no evidence that the left and right SMG can compensate for one another during TMS. Our findings motivate detailed studies of phonological processing in patients with acute or long-term damage of the right SMG.

  10. Radical Left in Albania and Kosovo: Differences and Similarities

    Directory of Open Access Journals (Sweden)

    Këlliçi Klejd

    2017-06-01

    Full Text Available The main research question for this paper is: Are there radical left wing movements in Albania and Kosovo and what are their main traits? Through answering this question, we will explore the development (or lack thereof of radical left wing movements. With radical left we intend movements that reject the underlying socio-economic structure of contemporary capitalism and its values and practices without opposing democracy. Through a thorough desk research and several interviews with experts and activists both in Albania and Kosovo, we look at the two case Political Opportunity Structures to study the emergence, the strategies, success or failures of the radical left. In Albania the radical left has been in decline since 1992, with only a recent revival from 2009 on. In Kosovo, the Lëvizja Vetëvendojse! (Self-Determination Movement, a civil movement transformed in a party, has succeeded in imposing its left agenda. Both cases employ a new rhetoric unrelated to the past communist regimes thus changing the perspective of political debate in both countries.

  11. Left versus right deceased donor renal allograft outcome.

    LENUS (Irish Health Repository)

    Phelan, Paul J

    2009-12-01

    It has been suggested that the left kidney is easier to transplant than the right kidney because of the longer length of the left renal vein, facilitating the formation of the venous anastomosis. There are conflicting reports of differing renal allograft outcomes based on the side of donor kidney transplanted (left or right).We sought to determine the effect of side of donor kidney on early and late allograft outcome in our renal transplant population. We performed a retrospective analysis of transplanted left-right deceased donor kidney pairs in Ireland between January 1, 1998 and December 31, 2008. We used a time to death-censored graft failure approach for long-term allograft survival and also examined serum creatinine at different time points post-transplantation. All outcomes were included from day of transplant onwards. A total of 646 transplants were performed from 323 donors. The incidence of delayed graft function was 16.1% in both groups and there was no significant difference in acute rejection episodes or serum creatinine from 1 month to 8 years post-transplantation.There were 47 death-censored allograft failures in the left-sided group compared to 57 in the right-sided group (P = 0.24). These observations show no difference in renal transplant outcome between the recipients of left- and right-sided deceased donor kidneys.

  12. MYOCARDIAL DEFORMATION AND COMPLETE LEFT BUNDLE BRANCH BLOCK

    Directory of Open Access Journals (Sweden)

    E. N. Pavlyukova

    2015-12-01

    Full Text Available Tissue Doppler imaging is evolving as a useful echocardiographic tool for quantitative assessment of left ventricular systolic and diastolic function. Over the last 10 years, myocardial deformation imaging has become possible initially with tissue Doppler , and more recently with myocardial speckle-tracking using 2D echocardiography. Unlike simple tissue velocity measurements, deformation measurements are specific for the region of interest. Strain rate or strain measurements have been used as sensitive indicators for subclinical diseases, and it is the most widely used tool to assess mechanical dyssynchrony. Left bundle branch block is a frequent, etiologically heterogeneous, clinically hostile and diagnostically challenging entity. About 2% of patients underwent cardiac stress testing show stable or intermittent left bundle branch block. Presence of left bundle branch block is associated with a lower and slower diastolic coronary flow velocity especially during hyperemia. Stress echocardiography is the best option for the diagnosis of ischemic heart disease, albeit specificity and sensitivity reduce in patients with left bundle branch block in the territory of left anterior descending artery in presence of initial septum dyskinesia.

  13. Relationship of left ventricular systolic function to persistence or development of electrocardiographic left ventricular hypertrophy in hypertensive patients

    DEFF Research Database (Denmark)

    Okin, Peter M; Wachtell, Kristian; Gerdts, Eva

    2014-01-01

    left ventricular systolic function in patients with new or persistent ECG LVH. METHODS: Baseline and year-3 ECG LVH and left ventricular midwall shortening (MWS) were examined in 725 hypertensive patients in the Losartan Intervention For Endpoint reduction in hypertension (LIFE) echocardiographic...... 1.03-3.50, P = 0.040) at year 3. CONCLUSION: Persistence or development of new ECG LVH during antihypertensive therapy is associated with an increased risk of left ventricular systolic dysfunction after 3 years' follow-up. These findings provide insight into a possible mechanism by which changes......BACKGROUND: Persistence or development of ECG left ventricular hypertrophy (LVH) by Cornell product criteria is associated with an increased risk of developing heart failure compared with regression or continued absence of LVH. We postulated that this association might be in part mediated via worse...

  14. The left atrium, atrial fibrillation, and the risk of stroke in hypertensive patients with left ventricular hypertrophy

    DEFF Research Database (Denmark)

    Wachtell, K.; Devereux, R.B.; Lyle, P.A.

    2008-01-01

    was superior to atenolol-based treatment for reducing new-onset AF and complications, especially stroke, associated with new-onset or pre-existing AF. Potential mechanisms of AF prevention by angiotensin receptor blockade supported by LIFE results include greater reduction in left atrial size and LV......The Losartan Intervention For Endpoint reduction in hypertension (LIFE) study provided extensive data on predisposing factors, consequences, and prevention of atrial fibrillation (AF) in patients with hypertension and left ventricular (LV) hypertrophy. Randomized losartan-based treatment...... hypertrophy. Differential effects of antihypertensive treatment on the left atrium and left ventricle may help prevent AF and reduce risk of stroke associated with hypertensive heart disease Udgivelsesdato: 2008/12...

  15. Is there any relationship between right and left hand dominance and right and left nasal airflow dominance?

    Science.gov (United States)

    Price, A; Eccles, R

    2017-10-01

    Left- or right-handedness is a common human trait, and it has been previously reported that human nasal airflow dominance correlates with hand dominance. Any relationship between hand dominance and nasal airflow dominance would be unusual. This study aimed to measure nasal airflow and look for any relationship to handedness. The modified Glatzel mirror was used to record the dominant nasal passage at 15-minute intervals over a 6-hour period in 29 healthy participants consisting of 15 left-handers and 14 right-handers. In left-handers, the percentage of time that the left nasal passage was dominant ranged from 0 to 100 per cent. In right-handers, the percentage of time that the right nasal passage was dominant ranged from 4.2 to 95.8 per cent. No correlation between nasal airflow dominance and hand dominance was identified. The results do not support the hypothesis that nasal airflow and handedness are related.

  16. Longitudinal strain predicts left ventricular mass regression after aortic valve replacement for severe aortic stenosis and preserved left ventricular function.

    Science.gov (United States)

    Gelsomino, Sandro; Lucà, Fabiana; Parise, Orlando; Lorusso, Roberto; Rao, Carmelo Massimiliano; Vizzardi, Enrico; Gensini, Gian Franco; Maessen, Jos G

    2013-11-01

    We explored the influence of global longitudinal strain (GLS) measured with two-dimensional speckle-tracking echocardiography on left ventricular mass regression (LVMR) in patients with pure aortic stenosis (AS) and normal left ventricular function undergoing aortic valve replacement (AVR). The study population included 83 patients with severe AS (aortic valve area regression (all P regression in patients with pure AS undergoing AVR. Our findings must be confirmed by further larger studies.

  17. Ultrasound-guided catheterization of the left subclavian vein without recognition of persistent left superior vena cava

    OpenAIRE

    Park, Sun Young; Yoo, Jae Hwa; Kim, Mun Gyu; Kim, Sang Ho; Park, Byoung-Won; Oh, Hong Chul; Kim, Hojoon

    2017-01-01

    Abstract Rationale: A persistent left superior vena cava (PLSVC) is rare, but the most common thoracic venous anomaly. We report a case of PLSVC unrecognized during left subclavian vein catheterization using real-time ultrasound-guided supraclavicular approach. Patient concerns: A 79-year-old man with history of hypertension presented with traumatic subdural hemorrhage, subarachnoid hemorrhage, and epidural hemorrhage. Before the operation, a central venous catheter (CVC) was placed into the ...

  18. Left Ulnar Artery Pseudoaneurysm and Left Hand Swelling Simulated by Elephantiasis in a Patient with Neurofibromatosis Type 1

    Directory of Open Access Journals (Sweden)

    Ta-Pin Lee

    2017-06-01

    Full Text Available Elephantiasis is a condition featured by gross enlargement of body parts to massive proportions. Neurofibromatosis type 1 (NF1 is a multisystem genetic disorder. Vascular anomaly is one among the complications of NF1. We report a case of NF1 who had a left hand vascular pseudoaneurysm with left hand swelling mimicking elephantiasis. The characteristics of sonography make it an excellent imaging modality to investigate this sort of superficial vascular lesion.

  19. Modulation of left primary motor cortex excitability after bimanual training and intermittent theta burst stimulation to left dorsal premotor cortex.

    Science.gov (United States)

    Neva, Jason L; Vesia, Michael; Singh, Amaya M; Staines, W Richard

    2014-03-15

    Bimanual visuomotor movement training (BMT) enhances the excitability of human preparatory premotor and primary motor (M1) cortices compared to unimanual movement. This occurs when BMT involves mirror symmetrical movements of both upper-limbs (in-phase) but not with non-symmetrical movements (anti-phase). The neural mechanisms mediating the effect of BMT is unclear, but may involve interhemispheric connections between homologous M1 representations as well as the dorsal premotor cortices (PMd). The purpose of this study is to assess how intermittent theta burst stimulation (iTBS) of the left PMd affects left M1 excitability, and the possible combined effects of iTBS to left PMd applied before a single session of BMT. Left M1 excitability was quantified using transcranial magnetic stimulation (TMS) in terms of both the amplitudes and spatial extent of motor evoked potentials (MEPs) for the extensor carpi radialis (ECR) before and multiple time points following (1) BMT, (2) iTBS to left PMd or (3) iTBS to left PMd and BMT. Although there was not a greater increase in either specific measure of M1 excitability due to the combination of the interventions, iTBS applied before BMT showed that both the spatial extent and global MEP amplitude for the ECR became larger in parallel, whereas the spatial extent was enhanced with BMT alone and global MEP amplitude was enhanced with iTBS to left PMd alone. These results suggest that the modulation of rapid functional M1 excitability associated with BMT and iTBS of the left PMd could operate under related early markers of neuro-plastic mechanisms, which may be expressed in concurrent and distinct patterns of M1 excitability. Critically, this work may guide rehabilitation training and stimulation techniques that modulate cortical excitability after brain injury. Copyright © 2013 Elsevier B.V. All rights reserved.

  20. Monophasic Synovial Sarcoma Presenting as Mitral Valve Obstruction

    Science.gov (United States)

    Chokesuwattanaskul, Warangkana; Terrell, Jason; Jenkins, Leigh Ann

    2010-01-01

    We report the case of a 26-year-old man who experienced progressive left-sided chest pain and 2 episodes of near-syncope. Studies revealed a 15-cm mass in the upper left lung, a 10-cm mass in the medial base of the left lung, and a 5-cm left atrial mass that involved the left lung, infiltrated the left pulmonary vein, and prolapsed into the mitral valve, causing intermittent obstruction. The patient underwent surgical excision of the left atrial tumor. Pathologic evaluation confirmed the diagnosis of monophasic synovial sarcoma. To our knowledge, this is only the 3rd report of left atrial invasion and resultant mitral valve obstruction from a synovial sarcoma that infiltrated the pulmonary vein. We believe that this is the 1st documented case of a metastatic left atrial synovial sarcoma in monophasic form. PMID:20844626

  1. Evaluation of left ventricular function by digital subtraction angiography

    International Nuclear Information System (INIS)

    Kuribayashi, Sachio; Ootaki, Makoto; Matsuyama, Seiya; Kanemoto, Nariaki; Furuya, Hideo

    1985-01-01

    Effects of contrast medium doses on left ventriculographic images using intravenous digital subtraction angiography (IVDSA-LVG) were assessed. The validity of IVDSA-LVG in evaluating ejection fraction (FF) and left ventricular regional wall motion was determined by comparison with conventional left ventriculography using direct injection (direct LVG). The advantages of left ventriculography using intraarterial subtraction angiography (IADSA-LVG) performed by injecting small doses of contrast media directly into the left ventricle were stressed. 1. To assess the effects of doses of contrast media on IVDSA-LVG, 10, 20, and 30 ml Urografin-76 were injected into the superior vena cava in 16 patients, and the resulting images were compared in each patient. With only 10 ml contrast medium, left ventricular opacification was fairly good, and regional wall motion was evaluated in many cases, but 30 ml were needed to calculate ventricular volume and EF. 2. To determine the validity of IVDSA-LVG in evaluating EF and regional wall motion, we compared IVDSA-LVG using 30 ml of contrast medium with direct LVG in 18 patients. There was a good correlation between the two methods in determining EF (r = 0.877), and 90 % of the interpretations of regional wall motion were in agreement by the two methods. IVDSA-LVG was useful and accurate in evaluating EF and regional wall motion of the left ventricle. 3. IADSA-LVG was performed for five patients, and good quality images were obtained in many cases, even with relatively small doses (10 ml) of contrast media. These results suggested that this method may be used in cases with impaired LV function, to avoid hemodynamic derangement induced by conventional direct LVG using large doses of contrast medium. (author)

  2. Improving left spatial neglect through music scale playing.

    Science.gov (United States)

    Bernardi, Nicolò Francesco; Cioffi, Maria Cristina; Ronchi, Roberta; Maravita, Angelo; Bricolo, Emanuela; Zigiotto, Luca; Perucca, Laura; Vallar, Giuseppe

    2017-03-01

    The study assessed whether the auditory reference provided by a music scale could improve spatial exploration of a standard musical instrument keyboard in right-brain-damaged patients with left spatial neglect. As performing music scales involves the production of predictable successive pitches, the expectation of the subsequent note may facilitate patients to explore a larger extension of space in the left affected side, during the production of music scales from right to left. Eleven right-brain-damaged stroke patients with left spatial neglect, 12 patients without neglect, and 12 age-matched healthy participants played descending scales on a music keyboard. In a counterbalanced design, the participants' exploratory performance was assessed while producing scales in three feedback conditions: With congruent sound, no-sound, or random sound feedback provided by the keyboard. The number of keys played and the timing of key press were recorded. Spatial exploration by patients with left neglect was superior with congruent sound feedback, compared to both Silence and Random sound conditions. Both the congruent and incongruent sound conditions were associated with a greater deceleration in all groups. The frame provided by the music scale improves exploration of the left side of space, contralateral to the right hemisphere, damaged in patients with left neglect. Performing a scale with congruent sounds may trigger at some extent preserved auditory and spatial multisensory representations of successive sounds, thus influencing the time course of space scanning, and ultimately resulting in a more extensive spatial exploration. These findings offer new perspectives also for the rehabilitation of the disorder. © 2015 The British Psychological Society.

  3. Balloon-occluded retrograde transvenous obliteration of gastric varix draining via the left inferior phrenic vein into the left hepatic vein

    International Nuclear Information System (INIS)

    Ibukuro, Kenji; Mori, Koichi; Tsukiyama, Toshitaka; Inoue, Yoshihiro; Iwamoto, Yukako; Tagawa, Kazumi

    1999-01-01

    We encountered a patient with gastric varix draining not via the usual left suprarenal vein but via the left inferior phrenic vein joining the left hepatic vein. Transfemoral balloon-occluded retrograde transvenous obliteration (BRTO) of the varix was performed under balloon occlusion of the left inferior phrenic vein via the left hepatic vein and retrograde injection of the sclerosing agent (5% of ethanolamine oleate) into the gastric varix. Disappearance of the gastric varix was confirmed on endoscopic examination 2 months later.

  4. Comparison of human expert and computer-automated systems using magnitude-squared coherence (MSC) and bootstrap distribution statistics for the interpretation of pattern electroretinograms (PERGs) in infants with optic nerve hypoplasia (ONH).

    Science.gov (United States)

    Fisher, Anthony C; McCulloch, Daphne L; Borchert, Mark S; Garcia-Filion, Pamela; Fink, Cassandra; Eleuteri, Antonio; Simpson, David M

    2015-08-01

    Pattern electroretinograms (PERGs) have inherently low signal-to-noise ratios and can be difficult to detect when degraded by pathology or noise. We compare an objective system for automated PERG analysis with expert human interpretation in children with optic nerve hypoplasia (ONH) with PERGs ranging from clear to undetectable. PERGs were recorded uniocularly with chloral hydrate sedation in children with ONH (aged 3.5-35 months). Stimuli were reversing checks of four sizes focused using an optical system incorporating the cycloplegic refraction. Forty PERG records were analysed; 20 selected at random and 20 from eyes with good vision (fellow eyes or eyes with mild ONH) from over 300 records. Two experts identified P50 and N95 of the PERGs after manually deleting trials with movement artefact, slow-wave EEG (4-8 Hz) or other noise from raw data for 150 check reversals. The automated system first identified present/not-present responses using a magnitude-squared coherence criterion and then, for responses confirmed as present, estimated the P50 and N95 cardinal positions as the turning points in local third-order polynomials fitted in the -3 dB bandwidth [0.25 … 45] Hz. Confidence limits were estimated from bootstrap re-sampling with replacement. The automated system uses an interactive Internet-available webpage tool (see http://clinengnhs.liv.ac.uk/esp_perg_1.htm). The automated system detected 28 PERG signals above the noise level (p ≤ 0.05 for H0). Good subjective quality ratings were indicative of significant PERGs; however, poor subjective quality did not necessarily predict non-significant signals. P50 and N95 implicit times showed good agreement between the two experts and between experts and the automated system. For the N95 amplitude measured to P50, the experts differed by an average of 13% consistent with differing interpretations of peaks within noise, while the automated amplitude measure was highly correlated with the expert measures but was

  5. Evaluation of left ventricular function using digital subtraction ventriculography

    International Nuclear Information System (INIS)

    Yiannikas, J.; Detrano, R.

    1986-01-01

    Digital subtraction ventriculography following injections of contrast via peripheral veins provides excellent images to assess left ventricular function. The images are essentially identical to those following DCV, but allow more uniform mixing of contrast in the left ventricular chamber. Furthermore, few, if any, cardiac arrhythmias occur, hence obviating difficulties that arise from DCV. The spatial resolution of the method is such that regional wall motion assessment of ventricular function is more accurate than that of other noninvasive imaging methods. The use of video-densitometry allows accurate assessment of left ventricular function even when the left ventricular cavity is nonsymmetrically deformed and aneurysmal. In the setting of the cardiac catheterization laboratory, digital ventriculography may provide a safer means of assessing left ventricular function when critical coronary or myocardial disease is present and allows multiple assessments of ventricular function during the same study. Although excellent correlations with standard ventriculography have been noted by all workers, significant discrepancies still exist in individual patients, particularly in the calculations of end diastolic volumes. In the authors experience and in those of most workers, the largest discrepancies existed in patients in whom suboptimal studies are included for analysis. The most frequent reason for the occasional suboptimal study as with all digital subtraction work is the misregistration that results from motion

  6. CT assessment of tracheobronchial anomaly in left pulmonary artery sling

    Energy Technology Data Exchange (ETDEWEB)

    Zhong, Yu-Min M.; Zhu, Ming; Sun, Ai-Min M.; Wang, Qian [Shanghai Jiao Tong University School of Medicine, Department of Radiology, Shanghai Children' s Medical Center, Shanghai (China); Jaffe, Richard B. [Primary Children' s Medical Center, Department of Medical Imaging, Salt Lake City, UT (United States); Gao, Wei [Shanghai Jiao Tong University School of Medicine, Department of Cardiology, Shanghai Children' s Medical Center, Shanghai (China)

    2010-11-15

    The left pulmonary artery sling (LPAS) is a rare vascular anomaly where the left pulmonary artery arises from the right pulmonary artery, passes over the right bronchus, and goes posteriorly between the trachea and esophagus. The LPAS is frequently associated with cardiac and non-cardiac defects including tracheobronchial abnormalities. To evaluate the utility of multislice CT (MSCT) and helical CT (HCT) in diagnosing and defining the tracheobronchial anomaly and anatomic relationships between the trachea and aberrant left pulmonary artery. MSCT or HCT was performed in 27 children to determine the tracheobronchial anatomy and identify tracheobronchial stenosis. Eighteen children underwent surgery. According to the Wells [6] classification of LPAS, which includes two main types and two subtypes, there were eight cases of type 1A, five cases of type 1B, six cases of type 2A and eight cases of type 2B in this group. Twenty-four of the 27 children had substantial tracheobronchial stenosis. Four died before surgery; the 18 had reanastomosis of the left pulmonary artery. Five children also had tracheoplasty; three died after surgery. CT, especially MSCT, is an ideal modality for simultaneously identifying aberrant left pulmonary artery and any associated tracheobronchial anomaly. The Wells classification is useful for operative planning. (orig.)

  7. Etiological aspect of left-handedness in adolescents

    Directory of Open Access Journals (Sweden)

    Dragović Milan

    2013-01-01

    Full Text Available Introduction. Lateralization of brain functions such as language and manual dominance (hand preferences and fine motor control are most likely under genetic control. However, this does not preclude the effect of various environmental factors on functional brain lateralization. A strong association of non-right-handedness (left- and mixed-handedness with various neurodevelopmental conditions (e.g. schizophrenia, autism, Rett syndrome implies that in some cases, non-right-handedness may be acquired rather than inherited (i.e., pathologically determined. Objective. The aim of the study was: (a re-investigation of several known risk factors for left-handedness (age of mother and/or father, twin pregnancies, and birth order, and (b examination of hitherto uninvestigated factors (type of birth, Apgar score, maternal smoking during pregnancy. Methods. Putative, causative environmental agents for this shift in manual distributions are explored in a sample of 1031 high school students (404 males and 627 females from Belgrade. Both pre-existing (age of parents, twin pregnancy, and birth order and new (Apgar score, maternal smoking, type of birth putative agents are examined. Results. We found that maternal smoking and low Apgar score (2-6 can significantly increase risk for left-handedness (p=0.046 and p=0.042, respectively. The remaining factors showed no significant association with left-handedness in adolescents. Conclusion. Our study clearly demonstrates that left-handedness may be related to maternal smoking during pregnancy and a low Apgar score on birth.

  8. Etiological aspect of left-handedness in adolescents.

    Science.gov (United States)

    Dragović, Milan; Milenković, Sanja; Kocijancić, Dusica; Zlatko, Sram

    2013-01-01

    Lateralization of brain functions such as language and manual dominance (hand preferences and fine motor control) are most likely under genetic control. However, this does not preclude the effect of various environmental factors on functional brain lateralization. A strong association of non-right-handedness (left- and mixed-handedness) with various neurodevelopmental conditions (e.g. schizophrenia, autism, Rett syndrome) implies that in some cases, non-right-handedness may be acquired rather than inherited (i.e., pathologically determined). The aim of the study was: (a) re-investigation of several known risk factors for left-handedness (age of mother and/or father, twin pregnancies, and birth order), and (b) examination of hitherto uninvestigated factors (type of birth, Apgar score, maternal smoking during pregnancy). Putative, causative environmental agents for this shift in manual distributions are explored in a sample of 1031 high school students (404 males and 627 females) from Belgrade. Both pre-existing (age of parents, twin pregnancy, and birth order) and new (Apgar score, maternal smoking, type of birth) putative agents are examined. We found that maternal smoking and low Apgar score (2-6) can significantly increase risk for left-handedness (p=0.046 and p=0.042, respectively).The remaining factors showed no significant association with left-handedness in adolescents. Our study clearly demonstrates that left-handedness may be related to maternal smoking during pregnancy and a low Apgar score on birth.

  9. Prevalence of left-sided melanomas in an Irish population.

    LENUS (Irish Health Repository)

    de Blacam, C

    2012-02-01

    BACKGROUND: A predominance of melanomas on the left side of the body has recently been described. No associations between tumour laterality and gender, age or anatomical site have been identified. AIM: The aim of this study was to investigate the prevalence of left-sided melanomas in an Irish population and to examine potential associations with various patient and tumour characteristics. METHODS: A retrospective chart review of patients with cutaneous melanoma who were treated over a 10-year period was carried out. Lateral distribution of melanoma on either side of the body was compared using chi(2) analysis and evaluated by gender, age group, anatomic location, histologic subtype and Breslow depth. RESULTS: More melanomas occurred on the left side (57%, P = 0.015), and this finding was particularly significant in females. For both genders combined, there were no statistically significant differences in laterality by age group, anatomic location, type of melanoma and Breslow depth. There were significantly more superficial spreading melanomas on the left side in both men and women. CONCLUSIONS: This study demonstrates a predominance of left-sided melanomas in Irish patients. While a number of demographic and molecular associations have been proposed, further research is required to fully explain this phenomenon.

  10. Prevalence of left-sided melanomas in an Irish population.

    LENUS (Irish Health Repository)

    de Blacam, C

    2011-04-17

    BACKGROUND: A predominance of melanomas on the left side of the body has recently been described. No associations between tumour laterality and gender, age or anatomical site have been identified. AIM: The aim of this study was to investigate the prevalence of left-sided melanomas in an Irish population and to examine potential associations with various patient and tumour characteristics. METHODS: A retrospective chart review of patients with cutaneous melanoma who were treated over a 10-year period was carried out. Lateral distribution of melanoma on either side of the body was compared using χ(2) analysis and evaluated by gender, age group, anatomic location, histologic subtype and Breslow depth. RESULTS: More melanomas occurred on the left side (57%, P = 0.015), and this finding was particularly significant in females. For both genders combined, there were no statistically significant differences in laterality by age group, anatomic location, type of melanoma and Breslow depth. There were significantly more superficial spreading melanomas on the left side in both men and women. CONCLUSIONS: This study demonstrates a predominance of left-sided melanomas in Irish patients. While a number of demographic and molecular associations have been proposed, further research is required to fully explain this phenomenon.

  11. Enhanced activation of the left hemisphere promotes normative decision making.

    Science.gov (United States)

    Corser, Ryan; Jasper, John D

    2014-01-01

    Previous studies have reported that enhanced activation of the left cerebral hemisphere reduces risky-choice, attribute, and goal-framing effects relative to enhanced activation of the right cerebral hemisphere. The present study sought to extend these findings and show that enhanced activation of the left hemisphere also reduces violations of other normative principles, besides the invariance principle. Participants completed ratio bias (Experiment 1, N = 296) and base rate neglect problems (Experiment 2, N = 145) under normal (control) viewing or with the right or left hemisphere primarily activated by imposing a unidirectional gaze. In Experiment 1 we found that enhanced left hemispheric activation reduced the ratio bias relative to normal viewing and a group experiencing enhanced right hemispheric activation. In Experiment 2 enhanced left hemispheric activation resulted in using base rates more than normal viewing, but not significantly more than enhanced right hemispheric activation. Results suggest that hemispheric asymmetries can affect higher-order cognitive processes, such as decision-making biases. Possible theoretical accounts are discussed as well as implications for dual-process theories.

  12. Correlation of vocals and lyrics with left temporal musicogenic epilepsy.

    Science.gov (United States)

    Tseng, Wei-En J; Lim, Siew-Na; Chen, Lu-An; Jou, Shuo-Bin; Hsieh, Hsiang-Yao; Cheng, Mei-Yun; Chang, Chun-Wei; Li, Han-Tao; Chiang, Hsing-I; Wu, Tony

    2018-03-15

    Whether the cognitive processing of music and speech relies on shared or distinct neuronal mechanisms remains unclear. Music and language processing in the brain are right and left temporal functions, respectively. We studied patients with musicogenic epilepsy (ME) that was specifically triggered by popular songs to analyze brain hyperexcitability triggered by specific stimuli. The study included two men and one woman (all right-handed, aged 35-55 years). The patients had sound-triggered left temporal ME in response to popular songs with vocals, but not to instrumental, classical, or nonvocal piano solo versions of the same song. Sentimental lyrics, high-pitched singing, specificity/familiarity, and singing in the native language were the most significant triggering factors. We found that recognition of the human voice and analysis of lyrics are important causal factors in left temporal ME and provide observational evidence that sounds with speech structure are predominantly processed in the left temporal lobe. A literature review indicated that language-associated stimuli triggered ME in the left temporal epileptogenic zone at a nearly twofold higher rate compared with the right temporal region. Further research on ME may enhance understanding of the cognitive neuroscience of music. © 2018 New York Academy of Sciences.

  13. Genetics Home Reference: cartilage-hair hypoplasia

    Science.gov (United States)

    ... complex called mitochondrial RNA-processing endoribonuclease, or RNase MRP. The RNase MRP enzyme is thought to be involved in several ... energy-producing centers of cells ( mitochondria ). The RNase MRP enzyme probably also processes ribosomal RNA , which is ...

  14. Stereological Evidences of Epithelial Hypoplasia of Seminiferous ...

    African Journals Online (AJOL)

    BACKGROUND: Anabolic-androgenic steroid compounds are one of the most widely abused drugs by athletes and muscle builders with the goal of improving performance/ability, appearance, or muscle mass. In addition, these steroids are widely used in the treatment of male infertility and subfertility. However, increasing ...

  15. Caffeine improves left hemisphere processing of positive words.

    Science.gov (United States)

    Kuchinke, Lars; Lux, Vanessa

    2012-01-01

    A positivity advantage is known in emotional word recognition in that positive words are consistently processed faster and with fewer errors compared to emotionally neutral words. A similar advantage is not evident for negative words. Results of divided visual field studies, where stimuli are presented in either the left or right visual field and are initially processed by the contra-lateral brain hemisphere, point to a specificity of the language-dominant left hemisphere. The present study examined this effect by showing that the intake of caffeine further enhanced the recognition performance of positive, but not negative or neutral stimuli compared to a placebo control group. Because this effect was only present in the right visual field/left hemisphere condition, and based on the close link between caffeine intake and dopaminergic transmission, this result points to a dopaminergic explanation of the positivity advantage in emotional word recognition.

  16. Novel nonsurgical left ventricular assist device and system.

    Science.gov (United States)

    Misiri, Juna; DeSimone, Christopher V; Park, Soon J; Kushwaha, Sudhir S; Friedman, Paul A; Bruce, Charles J; Asirvatham, Samuel J

    2013-01-01

    Treatment options for advanced stages of congestive heart failure remain limited. Left ventricular assist devices (LVADs) have emerged as a means to support failing circulation. However, these devices are not without significant risk such as major open chest surgery. We utilized a novel approach for device placement at the aorto-left atria continuity as a site to create a conduit capable of accommodating a percutaneous LVAD system. We designed and developed an expandable nitinol based device for placement at this site to create a shunt between the LA and aorta. Our experiments support this anatomic location as an accessible and feasible site for accommodation of an entirely percutaneous LVAD. The novelty of this approach would bypass the left ventricle, and thereby minimize complications and morbidities associated with current LVAD placement. Copyright © 2013 Elsevier Inc. All rights reserved.

  17. Anomalous Origin of Left Coronary Artery from Pulmonary Artery (ALCAPA)

    International Nuclear Information System (INIS)

    Younus, Z.; Ahmed, I.; Iftikhar, R.

    2013-01-01

    Anomalous origin of the left coronary artery from the pulmonary artery also recognized as Bland White Garland syndrome is a very rare congenital condition. A two-months old baby boy presented with dyspnoea for two weeks and a pansystolic murmur on auscultation. The base line investigations showed cardiomegaly and bilateral basal haze on X-ray chest. ECG showed ST elevation in leads l and AVl and echocardiography showed situs solitus, levocardia, hypokinetic intraventricular septum, ejection fraction of 30%, mitral regurgitation of grade-I and an anomalous origin of the left coronary artery from pulmonary artery was diagnosed. Patient was in left heart failure. It was rectified surgically by creating a transpulmonary tunnel (Takeuchi repair). Postoperative course was uneventful and he was finally discharged in stable condition. (author)

  18. Early changes in left atrial volume after acute myocardial infarction

    DEFF Research Database (Denmark)

    Bakkestrom, R.; Andersen, Mads J; Ersboll, M.

    2016-01-01

    . The objective was to assess changes in LA volume early after MI in patients with diastolic dysfunction and the relation to invasive hemodynamics and natriuretic peptides. Methods: 62 patients with left ventricle ejection fraction (LVEF) >= 45%, diastolic E/e' > 8 and LA volume index >34 ml/m(2) within 48 h......Background: Dilatation of left atrium (LA) reflects chronic LA pressure or volume overload that possesses considerable prognostic information. Little is known regarding the interaction between LA remodeling after acute myocardial infarction (MI) and left atrial pressure at rest and during exercise...... of MI were enrolled. After 1 and 4 months blood sampling, echocardiography and right heart catheterization were performed during exercise test. Results: LA remodeling was considered in patients with a change from mild (35-41 ml/m(2)), to severe (>48ml/m(2)) dilatation after 4 months (Found in 22...

  19. Evolving Concepts of Pulmonary Hypertension Secondary to Left Heart Disease.

    Science.gov (United States)

    Ramu, Bhavadharini; Thenappan, Thenappan

    2016-04-01

    Pulmonary hypertension associated with left heart disease is the most common form of pulmonary hypertension. Although its pathophysiology remains incompletely understood, it is now well recognized that the presence of pulmonary hypertension is associated with a worse prognosis. Right ventricular failure has independent and additive prognostic value over pulmonary hypertension for adverse outcomes in left heart disease. Recently, several new terminologies have been introduced to better define and characterize the nature and severity of pulmonary hypertension. Several new treatment options including the use of pulmonary arterial hypertension specific therapies are being considered, but there is lack of evidence. Here, we review the recent advances in this field and summarize the diagnostic and therapeutic modalities of use in the management of pulmonary hypertension associated with left heart disease.

  20. Left-Handed W bosons at the LHC

    International Nuclear Information System (INIS)

    Dixon, Lance

    2011-01-01

    The production of W bosons in association with jets is an important background to new physics at the LHC. Events in which the W carries large transverse momentum and decays leptonically lead to large missing energy and are of particular importance. We show that the left-handed nature of the W coupling, combined with valence quark domination at a pp machine, leads to a large left-handed polarization for both W + and W - bosons at large transverse momenta. The polarization fractions are very stable with respect to QCD corrections. The leptonic decay of the W +- bosons translates the common left-handed polarization into a strong asymmetry in transverse momentum distributions between positrons and electrons, and between neutrinos and anti-neutrinos (missing transverse energy). Such asymmetries may provide an effective experimental handle on separating W +jets from top quark production, which exhibits very little asymmetry due to C invariance, and from various types of new physics.