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Sample records for bilateral renal hypoplasia

  1. Bilateral renal artery variation

    OpenAIRE

    Üçerler, Hülya; Üzüm, Yusuf; İkiz, Z. Aslı Aktan

    2014-01-01

    Each kidney is supplied by a single renal artery, although renal artery variations are common. Variations of the renal arteryhave become important with the increasing number of renal transplantations. Numerous studies describe variations in renalartery anatomy. Especially the left renal artery is among the most critical arterial variations, because it is the referred side forresecting the donor kidney. During routine dissection in a formalin fixed male cadaver, we have found a bilateral renal...

  2. 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.)

  3. 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.)

  4. Bilateral triple renal arteries

    International Nuclear Information System (INIS)

    Pestemalci, Turan; Yildiz, Yusuf Zeki; Yildirim, Mehmet; Mavi, Ayfer; Gumusburun, Erdem

    2009-01-01

    Knowledge of the variations of the renal artery has grown in importance with increasing numbers of renal transplants, vascular reconstructions and various surgical and radio logic techniques being performed in recent years. We report the presence of bilateral triple renal arteries, discovered on routine dissection of a male cadaver. On the right side, one additional renal artery originated from the abdominal aorta (distributed to superior pole of the kidney) and one other originated from the right common iliac artery (distributed to lower pole of the kidney). On the left side, both additional renal arteries originated from the abdominal aorta. Our observation has been compared with variations described in the literature and their clinical importance has been emphasized. (author)

  5. BILATERAL DUPLICATION OF RENAL ARTERIES

    OpenAIRE

    Prajkta A Thete; Mehera Bhoir; M.V.Ambiye

    2014-01-01

    Routine dissection of a male cadaver revealed the presence of bilateral double renal arteries. On the right side the accessory renal artery originated from the abdominal aorta just above the main renal artery. On the left side the accessory renal artery originated from the abdominal aorta about 1 cm above the main renal artery. Knowledge of the variations of renal vascular anatomy has importance in exploration and treatment of renal trauma, renal transplantation, renal artery embolization, su...

  6. Bilateral papillary renal cell carcinoma

    International Nuclear Information System (INIS)

    Gossios, K.; Vazakas, P.; Argyropoulou, M.; Stefanaki, S.; Stavropoulos, N.E.

    2001-01-01

    Papillary renal cell carcinoma is a subgroup of malignant renal epithelial neoplasms. We report the clinical and imaging findings of a case with multifocal and bilateral renal cell carcinoma which are nonspecific. (orig.)

  7. Bilateral renal calculi

    Science.gov (United States)

    Sreenevasan, G

    1974-01-01

    Bilateral renal calculi were present in 114 (10.7%) of 1,070 cases of proved urinary calculus admitted to the Urological Department of the General Hospital, Kuala Lumpur, during the period November 1968—May 1973. The management of bilateral renal calculi is discussed with reference to the first 100 cases in this series. The introduction of renography has greatly facilitated the decision as to which kidney should be operated on first. The management of patients with and without uraemia is discussed and the use of the modified V and V—Y incisions for the removal of staghorn calculi is described. Complications and results are briefly reviewed. ImagesFig. 1Fig. 4Fig. 6Fig. 7 PMID:4845653

  8. 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.

  9. 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

  10. 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.

  11. Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction

    International Nuclear Information System (INIS)

    Doody, O.; Adam, W. R.; Foley, P. T.; Lyon, S. M.

    2009-01-01

    Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

  12. 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.)

  13. 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.

  14. Cutaneous metastasis of bilateral renal cell carcinoma.

    Science.gov (United States)

    Abbasi, Fariba; Alizadeh, Mansur; Noroozinia, Farahnaz; Moradi, Amin

    2013-01-01

    Renal cell carcinoma (RCC) is a malignant lethal tumour with high potential of metastasis. However, metastasis from RCC to the skin is much less common. It is virtually a sign of poor prognosis. We represent a 42 years old man with bilateral RCC of clear cell type followed by metastasis to the scalp one month later. In this case the relatively young age of the patient, bilaterality of RCC and occurance of skin metastasis in the absence of recurrent kidney tumour are interesting.

  15. Bilateral peri-renal lymphangiomatosis

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    T P Rajeev

    2006-01-01

    Full Text Available A 30 yrs old female with non-specific abdominal pain is presented. Ultrasound, computerised tomography abdomen were performed. A finely septated fluid collection surrounding both kidneys were found. Ultrasound guided fluid aspiration; chemical and cytological evaluation of the fluid was done. Exploration was done on the right side with the idea of removing the peri- renal cystic lymphatic collection. The entire cystic collection was removed. Patient followed up for the last two years and there is no recurrence of the cystic collection and the kidney function is preserved.

  16. [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.

  17. Bilateral multicystic renal dysplasia with potter sequence: A case with penile agenesis

    International Nuclear Information System (INIS)

    Dursun, Ahmet; Ermis, Bahri; Bahadir, Burak; Seckiner, Ilker

    2006-01-01

    Hereditary renal adysplasia (HRA) is rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low set ears and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thin skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association and Coloboma, heart anomaly, choanal atresia, retardation, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. (author)

  18. 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.)

  19. 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.)

  20. Bilateral Psoas Haematomata Complicating Renal Transplantation

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    Jacob A. Akoh

    2014-01-01

    Full Text Available Background. The challenge in managing patients undergoing renal transplantation is how to achieve optimum levels of anticoagulation to avoid both clotting and postoperative bleeding. We report a rare case of severe postoperative retroperitoneal bleeding including psoas haematomata complicating renal transplantation. Case Report. SM, a 55-year-old female, had a past history of aortic valve replacement, cerebrovascular event, and thoracic aortic aneurysm and was on long-term warfarin that was switched to enoxaparin 60 mg daily a week prior to her living donor transplantation. Postoperatively, she was started on a heparin infusion, but this was complicated by a large retroperitoneal bleed requiring surgical evacuation on the first postoperative day. Four weeks later, she developed features compatible with acute femoral neuropathy and a CT scan revealed bilateral psoas haematomata. Following conservative management, she made steady progress and was discharged home via a community hospital 94 days after transplantation. At her last visit 18 months after transplantation, she had returned to full fitness with excellent transplant function. Conclusion. Patients in established renal failure who require significant anticoagulation are at increased risk of bleeding that may involve prolonged hospitalisation and more protracted recovery and patients should be carefully counselled about this.

  1. Bilateral impacted femoral neck fracture in a renal disease patient ...

    African Journals Online (AJOL)

    Spontaneous bilateral femoral neck facture in a renal disease patient is not common. We report a case of 47-year-old female patient with chronic renal failure and on regular hemodialysis for the past 5 years who sustained bilateral impacted femoral neck fracture without history of trauma and injury and refused any surgical ...

  2. Outcome and renal function following salvage surgery for bilateral ...

    African Journals Online (AJOL)

    Objective: The aim of this study was to determine the surgical outcomes and renal function following salvage surgery for bilateral Wilms tumor (BWT). Summary background data The challenge for the surgeon treating BWT lies in striking a fine balance between renal preservation and oncological clearance. Methods: This is ...

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

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    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.

  4. 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.

  5. Bilateral renal dysplasia, nephroblastomatosis, and bronchial stenosis. A new syndrome?

    Science.gov (United States)

    Rodriguez, Maria Matilde; Correa-Medina, Mayrin; Whittington, Elizabeth E

    2015-06-01

    Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB.

  6. Chronic renal failure in a patient with bilateral ureterocele

    OpenAIRE

    Dada, Samuel A.; Rafiu, Mojeed O.; Olanrewaju, Timothy O.

    2015-01-01

    Ureterocele is a congenital anomaly, in which there is mal-development of the caudal segments of the ureter. There is a female preponderance with most cases seen in Caucasians. Among the reported complications of this condition, chronic renal failure occurring in the setting of ureterocele has not been well documented. We report a case of a young girl with bilateral ureterocele presenting with chronic renal failure, whose management presented a diagnostic failure and inadequate treatment.

  7. Chronic renal failure in a patient with bilateral ureterocele

    Science.gov (United States)

    Dada, Samuel A.; Rafiu, Mojeed O.; Olanrewaju, Timothy O.

    2015-01-01

    Ureterocele is a congenital anomaly, in which there is mal-development of the caudal segments of the ureter. There is a female preponderance with most cases seen in Caucasians. Among the reported complications of this condition, chronic renal failure occurring in the setting of ureterocele has not been well documented. We report a case of a young girl with bilateral ureterocele presenting with chronic renal failure, whose management presented a diagnostic failure and inadequate treatment. PMID:26108593

  8. Urethral dysontogenic metaplasia in cat with bilateral renal dysplasia

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    Carolina da Fonseca Sapin

    2017-10-01

    Full Text Available This paper to describe a case of dysontogenic urethral metaplasia in a one month old mongrel feline who also had bilateral renal dysplasia. Dysontogenic metaplasia in cats are scarce and this change may be associated with renal dysplasia and/or lower urinary tract. The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters. The urethra was thickened, hard to cut, and histologically, was replacing the connective tissue, cartilage and endochondral ossification areas, which features dysontogenic metaplasia. Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described.

  9. Bilateral Renal Angiomyolipomas with Invasion of the Renal Vein: A Case Report

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    C. Blick

    2008-01-01

    Full Text Available An angiomyolipoma (AML is usually a benign, rare, and, more commonly, a unilateral renal tumour. Bilateral tumours are very rare, particularly in the absence of tuberous sclerosis complex. Only in a few isolated cases have features of malignancy been associated with an AML. We present a unique case of bilateral AMLs mimicking invasive tumours in the absence of any other features of tuberous sclerosis complex.

  10. Bilateral Vascular Variations at the Renal Hilum: A Case Report

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    Naveen Kumar

    2012-01-01

    Full Text Available Imaging technology with its advancement in the field of urology is the boon for the patients who require minimally invasive approaches for various kidney disorders. These approaches require a precise knowledge of the normal and variant anatomy of vascular structures at the hilum of the kidney in terms of their pattern of arrangement and division. The present paper describes a bilateral anomalous arrangement of the structures at the renal hilum as well as their peculiar branching pattern which is of clinical and surgical relevance. Multiple branching of the renal vessels was observed in both kidneys due to which the hila were congested. The right renal artery immediately after its origin divided into 2 branches. The upper branch represented an aberrant artery whereas the lower branch gave 5 divisions. The left renal artery also divided into 2 branches much before the hilum as anterior and posterior divisions. The anterior branch took an arched course and gave 6 branches. The posterior branch gave 3 terminal branches before entering the renal substance. In addition to anomalous hilar structures, normal architecture of both kidneys was altered and the hilum of the left kidney was found on its anterior surface.

  11. 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

  12. 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.

  13. 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 ...

  14. Evaluation of Significance of Diffusely Increased Bilateral Renal Uptake on Bone Scan

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Mi Sook; Yang, Woo Jin; Byun, Jae Young; Park, Jung Mi; Shinn, Kyung Sub; Bahk, Yong Whee [Catholic University College of Medicine, Seoul (Korea, Republic of)

    1990-03-15

    Unexpected renal abnormality can be detected on bone scan using {sup 99m}Tc-MDP. The purpose of the study is to evaluate the diagnostic significance of diffusely increased bilateral renal uptake on bone scan. 1,500 bone scan were reviewed and 43 scans which showed diffusely increased bilateral renal uptake were selected for analysis. Laboratory findings for renal and liver function tests including routine urinalysis were reviewed in 43 patients. 26 of 43 case showed abnormality in urinalysis and renal function study. 20 of 43 cases showed abnormal liver function study and 3 of these cases were diagnosed as hepatorenal syndrome later. 13 of those 20 cases had liver cirrhosis with or without hepatoma. 12 of 43 cases showed abnormality both in renal and liver function studies. 2 of 43 cases showed diffusely increased bilateral renal uptake after chemotherapy for cancer but not on previous scans before chemotherapy. 2 of 43 cases showed hypercalcaemia and 8 of 43 cases had multifocal bone uptake due to metastasis or benign bone lesion. But the latter showed no hypercalcaemia at all. There was no significant correlation between increased renal uptake and MDP uptake in soft tissue other than kidneys. This study raised the possibility that the impaired liver and/or renal function may result in diffuse increase of bilateral renal uptake of MDP of unknown mechanism. It seems to need further study on this correlation.

  15. Evaluation of Significance of Diffusely Increased Bilateral Renal Uptake on Bone Scan

    International Nuclear Information System (INIS)

    Sung, Mi Sook; Yang, Woo Jin; Byun, Jae Young; Park, Jung Mi; Shinn, Kyung Sub; Bahk, Yong Whee

    1990-01-01

    Unexpected renal abnormality can be detected on bone scan using 99m Tc-MDP. The purpose of the study is to evaluate the diagnostic significance of diffusely increased bilateral renal uptake on bone scan. 1,500 bone scan were reviewed and 43 scans which showed diffusely increased bilateral renal uptake were selected for analysis. Laboratory findings for renal and liver function tests including routine urinalysis were reviewed in 43 patients. 26 of 43 case showed abnormality in urinalysis and renal function study. 20 of 43 cases showed abnormal liver function study and 3 of these cases were diagnosed as hepatorenal syndrome later. 13 of those 20 cases had liver cirrhosis with or without hepatoma. 12 of 43 cases showed abnormality both in renal and liver function studies. 2 of 43 cases showed diffusely increased bilateral renal uptake after chemotherapy for cancer but not on previous scans before chemotherapy. 2 of 43 cases showed hypercalcaemia and 8 of 43 cases had multifocal bone uptake due to metastasis or benign bone lesion. But the latter showed no hypercalcaemia at all. There was no significant correlation between increased renal uptake and MDP uptake in soft tissue other than kidneys. This study raised the possibility that the impaired liver and/or renal function may result in diffuse increase of bilateral renal uptake of MDP of unknown mechanism. It seems to need further study on this correlation.

  16. Bilateral enlargement of the orbital muscles: first manifestation of renal adenocarcinoma

    International Nuclear Information System (INIS)

    Wolosker, Angela M. Borri; Bekhor, Daniel; Goes, Paulo; Attie, Greicie Cristina Gerra

    2000-01-01

    The authors present an unusual case of a patient with orbital metastases from renal carcinoma involving the extra ocular muscles bilaterally. The importance of computed tomography for the differential diagnosis with other orbital lesions is emphasized. (author)

  17. Laparoscopic bilateral nephroureterectomy and bladder cuff excision for native renal pelvic and ureteral transitional cell carcinoma after renal transplantation.

    Directory of Open Access Journals (Sweden)

    Chen C

    2003-01-01

    Full Text Available A 37-years-old female who was suffering from end-stage renal disease for about 6 years received allograft renal transplantation 4 years ago. She has been receiving 50mg of Cyclosporin A orally daily for immuno-suppression since then. Gross haematuria was noted and computerised tomography showed native left renal pelvic and ureteral multi-focal transitional cell carcinoma with severe hydronephrosis. Laparoscopic bilateral nephroureterectomy and bladder cuff excision were performed. In the past, history of previous operation was considered a relative contraindication for laparoscopic surgery. To our knowledge, we present the first case of laparoscopic treatment for native renal pelvic and ureteral transitional cell carcinoma after renal allograft transplantation without a hand-assisted device. This case shows the feasibility of laparoscopic bilateral nephroureterectomy in patients with transplanted kidneys.

  18. Salvage of bilateral renal artery occlusion after endovascular aneurysm repair with open splenorenal bypass

    Directory of Open Access Journals (Sweden)

    Samuel Jessula, MDCM

    2017-09-01

    Full Text Available We report renal salvage maneuvers after accidental bilateral renal artery coverage during endovascular aneurysm repair of an infrarenal abdominal aortic aneurysm. A 79-year-old man with an infrarenal abdominal aortic aneurysm was treated with endovascular aneurysm repair. Completion angiography demonstrated coverage of the renal arteries. Several revascularization techniques were attempted, including endograft repositioning and endovascular stenting through the femoral and brachial approach. The patient eventually underwent open splenorenal bypass with a Y Gore-Tex graft (W. L. Gore & Associates, Flagstaff, Ariz. After 3 months, computed tomography showed no evidence of endoleak and patent renal arteries. Renal function was well maintained, and the patient did not require dialysis.

  19. Bilateral renal masses in a 10-year-old girl with renal failure and urinary tract infection: the importance of functional imaging

    Energy Technology Data Exchange (ETDEWEB)

    Urbania, Thomas H. [University of California, San Francisco, Department of Radiology and Biomedical Imaging, 505 Parnassus Ave., Box 0628, San Francisco, CA (United States); Kammen, Bamidele F.; Nancarrow, Paul A. [Children' s Hospital and Research Center Oakland, Department of Diagnostic Imaging, Oakland, CA (United States); Morrell, Rose Ellen [Children' s Hospital and Research Center Oakland, Department of Nephrology, Oakland, CA (United States)

    2009-02-15

    Renal sonography is a routine step in the evaluation of new onset renal failure. When renal masses are discovered in this setting, functional imaging may be critical. We report a case of bilateral renal masses in a girl with urinary tract infection and renal insufficiency found to have vesicoureteral reflux. Renal scintigraphy revealed these masses to be the only remaining functional renal tissue, preventing potentially harmful resection. (orig.)

  20. Bilateral renal masses in a 10-year-old girl with renal failure and urinary tract infection: the importance of functional imaging

    International Nuclear Information System (INIS)

    Urbania, Thomas H.; Kammen, Bamidele F.; Nancarrow, Paul A.; Morrell, Rose Ellen

    2009-01-01

    Renal sonography is a routine step in the evaluation of new onset renal failure. When renal masses are discovered in this setting, functional imaging may be critical. We report a case of bilateral renal masses in a girl with urinary tract infection and renal insufficiency found to have vesicoureteral reflux. Renal scintigraphy revealed these masses to be the only remaining functional renal tissue, preventing potentially harmful resection. (orig.)

  1. 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.

  2. Bilateral Giant Renal Angiomyolipoma in a Patient with Tuberous Sclerosis Complex: A Case Report

    Directory of Open Access Journals (Sweden)

    Andika Afriansyah

    2018-04-01

    Full Text Available Tuberous sclerosis complex (TSC has several renal manifestations including angiomyolipomas (AML and renal epithelial neoplasms. A bilateral giant renal AML is extremely rare. We report a case of giant bilateral AML and discuss the diagnosis and treatment of it. The 22-year-old man was admitted due to bilateral flank pain, gross hematuria, and abdominal fullness. He had history of epilepsy, mental retardation, and delayed development during childhood. He had angiofibroma on his face since 10 years ago. Abdominal CT and MRI revealed large lobulated heterogeneous mass with fatty content. Based on those findings, we diagnosed the patient with bilateral giant renal AML. We gave conservative management for the patient and planned to total nephrectomy on the left kidney if the continued bleeding occurred. AML associated with TSC occur more frequently as multiple lesions and grows to larger size than idiopathic AML. Bilateral giant AML, which is very rare, could be treated with conservative management if no significant hemorrhage occurred.

  3. Cephalad-renal ectopia: Bilateral subdiaphragmatic kidneys in a patient of omphalocele with ventral hernia

    Directory of Open Access Journals (Sweden)

    Jitendra Parmar

    2016-04-01

    Full Text Available Renal ectopia is a rare congenital anomaly. Thoracic ectopic kidney was being considered as rarest, however no case of bilateral subdiaphragmatic kidneys in omphalocele patients presented with ventral hernia has been reported yet, as per our best of knowledge. This is a report of a 5- year-old male patient who presented with ventral hernia after omphalocele. A thorough examination, laboratory, and radiological investigations including ultrasonography, plain abdominal x-ray, intravenous urogram, and computerized tomography revealed bilateral subdiaphragmatic ectopic kidneys with azygos continuation of inferior vena cava, retro-aortic left renal vein and spina bifida

  4. The diagnosis of bilateral primary renal paragangliomas in a cat

    Directory of Open Access Journals (Sweden)

    Ryan B. Friedlein

    2017-01-01

    Full Text Available A 9-year-old sterilised female domestic short-hair cat was referred with a history of vomiting and anorexia of 3 months’ duration. Biochemistry, full-blood counts, thoracic radiographs, feline pancreatic-specific lipase, abdominal ultrasonography and feline immunodeficiency virus/feline leukaemia virus (FIV/FeLV SNAP tests had been performed. Mild hypochloraemia and moderate hypokalaemia were evident on initial presentation. Abdominal ultrasonography initially revealed unilateral renal nodules on the left side. These were subjected to fine-needle aspiration and cytological evaluation. A neuroendocrine tumour was suspected, and biopsies via midline coeliotomy were taken to confirm the diagnosis. Initial histopathology diagnosed primary renal carcinomas or neuroendocrine neoplasia; however, the definitive diagnosis became renal paragangliomas after immunohistochemistry and transmission electron microscopy were performed. The cat was regularly monitored with serum biochemistry parameters, blood pressure determinations, thoracic radiographs and subsequent abdominal ultrasonography. Biochemistry, radiography and blood pressures remained normal over a 24-week follow-up period, while subsequent ultrasonography revealed tumour progression in both number and size in both kidneys. Primary neuroendocrine tumours of the kidney are frequently incorrectly diagnosed as other renal tumours such as renal cell carcinoma, mesonephric tumours or undifferentiated carcinomas. This case report highlights the importance of additional testing, including immunohistochemistry and transmission electron microscopy, to obtain a definitive diagnosis of paragangliomas.

  5. Bilateral breast metastases of a renal carcinoma: a case report and review of the literature.

    Science.gov (United States)

    Ganapathi, S; Evans, G; Hargest, R

    2008-01-01

    Metastatic tumours account for mastectomy and excision of right breast lump was done. Histology of both lesions confirmed them as metastatic deposits. Bilateral breast metastasis from a renal cancer is very rare and this is the second reported case. This case illustrates the potential for rare sites of metastases and for the consideration of metastasis in the presence of previous renal cancer. Recognition as metastatic neoplasm is important to prevent unnecessary radical procedures.

  6. Bilateral Stress Fractures of the Femoral Neck from Renal Osteomalacia: A Case Report

    Directory of Open Access Journals (Sweden)

    S Sengupta

    2008-04-01

    Full Text Available A rare case of spontaneous bilateral stress fractures of femoral neck leading to coxa vara in a young female with history of chronic renal disease and secondary osteomalacia is described. Once the underlying disease was controlled, the fracture was treated by valgus osteotomy with good result.

  7. Simultaneous Bilateral Femur Neck Fracture in A Young Adult with Chronic Renal Failure- A Case Report and Review of Literature.

    Science.gov (United States)

    V, Sathyanarayana; Patel, Maulik Tulsibhai; S, Raghavan; D, Naresh

    2015-01-01

    Pathological bilateral femoral neck fracture due to renal osteodystrophy is rare. This is a report of a chronic renal failure patient who had sustained bilateral intra-capsular displaced fracture neck of femur following an episode of convulsion and the difficulties encountered in early diagnosis and treatment. The pathophysiology of renal osteodystrophy and the treatment of hip fractures in patients with renal failure are also discussed. A 23 years old male patient admitted with h/o dysuria, pyuria and loss of appetite since 3 months. He was a known case of chronic renal failure and reflux nephropathy. On investigating, patient's renal parameters were high and he was started with haemodialysis. The next day patient had c/o bilateral hip pain and inability to move bilateral lower limbs following an episode of seizure. Radiograph of pelvis showed vertical sub capital fractures of bilateral neck of femur. In this patient, considering his age, general condition & prognosis, an elective surgery in the form of bilateral uncemented modular bipolar hemiarthroplasty was done. Overall risk of hip fracture among patients with chronic renal failure is considerably higher than in the general population, independent of age and gender. Simultaneous spontaneous bilateral fractures of the femoral neck are rare and a delayed diagnosis is usual. The study of etiological factors of these fractures is essential to guide us in choosing the treatment of choice. Obviously patient's age, life expectancy as well as renal co morbidity has an influence over deciding treatment and outcome.

  8. Evidence-based, ethically justified counseling for fetal bilateral renal agenesis

    Science.gov (United States)

    Thomas, Alana N.; McCullough, Laurence B.; Chervenak, Frank A.; Placencia, Frank X.

    2017-01-01

    Background Not much data are available on the natural history of bilateral renal agenesis, as the medical community does not typically offer aggressive obstetric or neonatal care asbilateral renal agenesis has been accepted as a lethal condition. Aim To provide an evidence-based, ethically justified approach to counseling pregnant women about the obstetric management of bilateral renal agenesis. Study design A systematic literature search was performed using multiple databases. We deploy an ethical analysis of the results of the literature search on the basis of the professional responsibility model of obstetric ethics. Results Eighteen articles met the inclusion criteria for review. With the exception of a single case study using serial amnioinfusion, there has been no other case of survival following dialysis and transplantation documented. Liveborn babies die during the neonatal period. Counseling pregnant women about management of pregnancies complicated by bilateral renal agenesis should be guided by beneficence-based judgment informed by evidence about outcomes. Conclusions Based on the ethical analysis of the results from this review, without experimental obstetric intervention, neonatal mortality rates will continue to be 100%. Serial amnioinfusion therefore should not be offered as treatment, but only as approved innovation or research. PMID:28222038

  9. Association between renal cystic lesions and bilateral Wilms' tumours

    Energy Technology Data Exchange (ETDEWEB)

    Simanovsky, Natalia; Hiller, Nurith [Hadassah-Hebrew University Medical Center at Mt Scopus, Department of Medical Imaging, POB 12000, Jerusalem (Israel); Revel-Vilk, Shoshana; Weintraub, Michael [Hadassah-Hebrew University Medical Center, Department of Pediatric Hematology/ Oncology, Jerusalem (Israel)

    2016-06-15

    Evaluate a potential association between Wilms' tumour (WT) and renal cystic lesions. Digital records and imaging files of consecutive patients diagnosed with WT between 2004 and 2014 were retrospectively reviewed under an Institutional Review Board waiver of informed consent. The locations of renal cysts seen on US, CT, and/or MRI were recorded and compared with the locations of newly developed WT. A total of 48 patients (mean age 3 years 9 months) presented with newly diagnosed WT in the study period. Mean follow-up was 4.5 (range 1-10) years. WT was unilateral in 40 children, bilateral in 8. Renal cysts were identified in only one of the forty patients (2.5 %) with unilateral disease - in the contralateral kidney. In contrast, renal cysts were found in seven of eight patients with bilateral WT (87.5 %), in two of whom, new tumours developed in the same location where cysts had been seen on previous imaging studies. Renal cystic lesions in patients with Wilms' tumour should be regarded as potential tumour precursors, and followed with frequent imaging. (orig.)

  10. Bilateral renal cell carcinoma in a horseshoe kidney: preoperative assessment with MRI and digital subtraction angiography

    International Nuclear Information System (INIS)

    Schubert, R.A.; Soeldner, J.; Kaiser, W.A.; Steiner, T.; Schubert, J.

    1998-01-01

    Renal cell carcinoma in a horseshoe kidney is an unusual entity. To our knowledge, only 123 cases have been published to date. We report the first bilateral case of two clear-cell carcinomas in an asymmetrically fused kidney. Optimum preservation of renal function after radical tumor removal requires accurate preoperative imaging. Since the vascular supply in fusion anomalies is extremely variable, angiography is mandatory. Magnetic resonance imaging was most suitable to predict the tumor extent and localization, because it simultaneously gave the most comprehensive anatomical overview of the malformation. (orig.)

  11. [Bilateral nephrectomy in patients with end-stage renal failure and chronic active pyelonephritis].

    Science.gov (United States)

    Lysenko, M A; Vtorenko, V I; Trushkin, R N; Lubennikov, A E; Sysoev, A M; Sokolov, A A

    2016-02-01

    This study analyzed the results of bilateral nephrectomy in 14 patients with end-stage renal disease (ESRD) and chronic active pyelonephritis. Seven patients had urosepsis, and 10 patients had a purulent form of pyelonephritis, which was one-sided in 7 of them. In the early postoperative period, on average, after 9.3 days, 9 patients died. Statistically significant risk factors for death were: chronic hemodialysis, long-term antibiotic therapy, and existing sepsis. Intraoperative complications and postoperative morbidity were not significantly associated with death. The study results imply the need of differentiated approach to bilateral nephrectomy in patients with ESRD and risk factors for fatal outcome. It must be performed on the strong indications since the intervention does not lead to eradication of sepsis. It is advisable to perform "preventive, sanation" bilateral nephrectomy in the "cold period" in patients at risk for developing urosepsis.

  12. Bilateral multiple cystic kidney disease and renal cortical abscess in a Boerboel

    Directory of Open Access Journals (Sweden)

    A. M. Kitshoffa

    2011-04-01

    Full Text Available Cystic renal disease is rare in dogs and although infected renal cysts have been reported in humans, no report could be found in dogs. A 58 kg, 5-year-old, castrated, male Boerboel presented with weight loss, pyrexia, lethargy and vomiting, 20 months after an incident of haematuria was reported. The initial ultrasonographic diagnosis was bilateral multiple renal cysts of unknown aetiology. The cysts had significantly increased in size over the 20-month period and some contained echogenic specks which could be related to infection, normal cellular debris or haemorrhage. In both kidneys the renal contours were distorted (the left more than the right. The abnormal shape of the left kidney was largely due to multiple cysts and a large crescent-shaped septate mass on the cranial pole of the kidney. Aspirates of the septate mass were performed (left kidney and the cytology and culture were indicative of an abscess. It is suggested that the previous incident of haematuria provided a portal of entry for bacteria into the cysts resulting in renal cortical abscess formation.

  13. Bilateral Renal Lymphoma a Differential Diagnosis of Policyst Renal Disease. Case Report

    International Nuclear Information System (INIS)

    Poveda S, Cesar A; Rodriguez P, Jose L

    2010-01-01

    A case report of a patient with abdominal pain, diaphoresis and fever of three months duration imaging studies were interpreted as polycystic kidneys. The patient went to another institution, where a different approach led to a kidney biopsy that confirmed renal lymphoma. The case is interesting, by the way it is diagnosed and it is important to note the differential diagnosis.

  14. Fetal bilateral renal agenesis, phocomelia, and single umbilical artery associated with cocaine abuse in early pregnancy.

    Science.gov (United States)

    Kashiwagi, Maki; Chaoui, Rabih; Stallmach, Thomas; Hürlimann, Sandra; Lauper, Urs; Hebisch, Gundula

    2003-11-01

    Maternal cocaine abuse in pregnancy is associated with complications such as intrauterine growth retardation, abruptio placentae, and preterm delivery. We report what is, to our knowledge, the first published observation of fetal bilateral renal agenesis associated with a vascular disruption syndrome comprising upper limb reduction defect and a single umbilical artery following maternal cocaine abuse in early pregnancy. This constellation in a fetus aborted at 18 weeks extends the spectrum of complications possibly associated with cocaine abuse in pregnancy. Copyright 2003 Wiley-Liss, Inc.

  15. Bilateral renal agenesis, a severe anomaly in a premature infant with VACTERL association: A case report

    Directory of Open Access Journals (Sweden)

    Erol Basuguy

    2017-11-01

    Full Text Available We report on a preterm male (birth weight 1,100 g with bilateral renal agenesis, a lethal malformation. Additionally, the child suffered from an atrial septal defect, ventricular septal defect, right aortic arch anomaly, a high type of anal atresia, vertebral anomalies, limbs defects (VACTERL association. The infant during first day of life was treated with an emergency sigmoid ostomy and peritoneal dialysis because of increasing abdominal dilatation and high urea and creatinine levels in blood. Important congenital anomalies associated with VACTERL association and prematurity are very serious causes of mortality in the early period

  16. Giant Bilateral Renal Angiomyolipomas and Lymphangioleiomyomatosis Presenting after Two Successive Pregnancies Successfully Treated with Surgery and Rapamycin

    Directory of Open Access Journals (Sweden)

    Ramón Peces

    2011-01-01

    Full Text Available We report the case of a 25-year-old woman who presented with abdominal and flank pain with two successive pregnancies and was diagnosed of giant bilateral renal AMLs and pulmonary LAM associated with TSC in the post-partum of her second pregnancy. This case illustrates that in women with TSC rapid growth from renal AMLs and development of LAM may occur with successive pregnancies. It also stresses the potential for preservation of renal function despite successive bilateral renal surgery of giant AMLs. Moreover, the treatment with a low-dose rapamycin may be an option for LAM treatment. Finally, a low-dose rapamycin may be considered as an adjuvant treatment together to kidney-sparing conservative surgery for renal AMLs.

  17. Leiomyosarcoma of inferior vena cava involving bilateral renal veins: Surgical challenges and reconstruction with upfront saphenous vein interposition graft for left renal vein outflow

    Directory of Open Access Journals (Sweden)

    Rishi Nayyar

    2010-01-01

    Full Text Available Leiomyosarcoma of inferior vena cava (IVC involving bilateral renal veins presents a surgical challenge. Herein, we report the successful management of two such cases using restoration of left renal venous outflow by saphenous vein interposition graft as first step of surgery. Then radical resection of tumor and right kidney was done. IVC was lastly reconstructed using Gore-Tex graft. This report highlights the surgical challenges to ensure radical resection. Furthermore, the importance of restoring left renal outflow in presence of concomitant right nephrectomy is discussed. Both the patients were disease free at six months with no loss of left renal glomerular filtration rate.

  18. Optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    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.

  19. An exceedingly rare cause of secondary hypertension: bilateral renal artery dissection possibly secondary to extracorporeal shock-wave lithotripsy (ESWL).

    Science.gov (United States)

    Orhan, Ozbek; Kultigin, Turkmen; Osman, Koc; Yalcin, Solak; Melih, Anil; Niyazi, Gormus

    2011-01-01

    Extracorporeal shock-wave lithotripsy (ESWL) is an effective and relatively non-invasive treatment modality for ureteral or renal calculi. Although it has been accepted as a safe procedure, minor and major complications have been reported after ESWL. Spontaneous renal artery dissection (SRAD) is a rare and usually misdiagnosed condition because of non-specific presentation of the patients. Depending on the severity of the extent of the dissection non-operative or surgical treatment modalities could be performed. We represent a patient with complaints of bilateral flank pain, hematuria and hypertensive urgency who was diagnosed as having bilateral SRAD possibly secondary to ESWL and chronic hypertension.

  20. Genetics Home Reference: pontocerebellar hypoplasia

    Science.gov (United States)

    ... 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 ...

  1. Percutaneous nephrolithotomy of bilateral staghorn renal calculi in pediatric patients: 12 years experience in a tertiary care centre.

    Science.gov (United States)

    Purkait, Bimalesh; Kumar, Manoj; Sokhal, Ashok Kumar; Bansal, Ankur; Sankhwar, Satya Narayan; Gupta, Ashok Kumar

    2017-08-01

    To assess the outcomes of percutaneous nephrolithotomy (PCNL) in bilateral staghorn calculi in pediatric patients, we have performed a retrospective analysis. Staghorn calculus is defined as stone that fills a greater part of the pelvic-caliceal system. Still, in developing countries, patients may present with staghorn calculus. PCNL is the preferred treatment modality for staghorn calculus both in adult and children. Our study included fifty-one pediatric patients (calculus in children needs expertise. PCNL in B/L staghorn renal calculus in children is safe and effective. B/L staghorn renal calculi with compromised renal function have higher chance of complications including bleeding.

  2. Complicated Candida parapsilosis peritonitis on peritoneal dialysis in a neonate with renal failure because of bilateral adrenal abscesses

    Directory of Open Access Journals (Sweden)

    I. Cheng

    2011-10-01

    Full Text Available We present a full-term female infant with a difficult delivery course complicated with Escherichia coli sepsis and bilateral adrenal abscesses. She developed renal failure and received peritoneal dialysis. Peritonitis of Candida parapsilosis developed later. The infant was successfully treated with hemofiltration and a combination of antifungal agents.

  3. 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

  4. Glenoid hypoplasia: a report of 2 patients.

    Science.gov (United States)

    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.

  5. 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

  6. Radioisotopic investigation of renal arterial hypertension using 99m Tc-DTPA

    International Nuclear Information System (INIS)

    Champailler, A.; Defour-Decousus, M.; Houzard, C.; Healy, J.C.; Gonthier, R.; Juge, J.; Berthoux, F.C.

    1984-01-01

    To evaluate the clinical validity of renal investigation using 99m Tc-DTPA, 60 hypertensive patients divided into four groups according to diagnosis were studied: 1) renovascular hypertension (22 patients); 2) chronic pyelo-nephritis (11 patients); 3) renal hypoplasia (12 patients); 4) hypertension due to bilateral parenchymatous (15 patients). A good correlation was found between clearance of DTPA and creatinine clearance/1.73 m 2 body surface (n = 51, r = 0.68, p 2 BS [fr

  7. A case of treatment in a patient with synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands: Clinical observation

    Directory of Open Access Journals (Sweden)

    V. R. Latypov

    2014-11-01

    Full Text Available Synchronous bilateral renal cell carcinoma occurs in 1.4 % of cases. The probability of bilateral adrenal metastases from renal cell carcinoma is less than 0.5 %. The clinical observation presents a case of synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands. A 55‑year-old male patient was adm tted with the signs of hematuria and anemia to the Unit of Urology, Clinic of General Surgery, Siberian State Medical University. He was found to have synchronous bilateral renal cell carcinoma and simultaneous bilateral adrenal involvement. Sequential surgical treatment – radical nephrectomy (with adrenal gland removal on the right side and, after 3 months, adrenalectomy and kidney resection on the left side were performed. All the organs removed displayed tumors that proved to be renal cell carcinomas (a clear cell variant. There were lymph node metastases in the right-sided renal portal. Postoperatively, the investigators performed hormone replacement therapy for adrenal insufficiency, an immunotherapy cycle, three cycles of targeted therapy withsorafenib and sunitinib (at an interval of 0.5–2 years, and insulin therapy for new-onset diabetes mellitus. The duration of a follow-up was 6.2 years. When describing the case, the patient was alive and showed a generalized tumorous process with extensive tumor involvement of the solitary kidney. Sunitinib therapy was used.

  8. A case of treatment in a patient with synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands: Clinical observation

    Directory of Open Access Journals (Sweden)

    V. R. Latypov

    2014-01-01

    Full Text Available Synchronous bilateral renal cell carcinoma occurs in 1.4 % of cases. The probability of bilateral adrenal metastases from renal cell carcinoma is less than 0.5 %. The clinical observation presents a case of synchronous bilateral renal cell carcinoma and simultaneous metastatic involvement of both adrenal glands. A 55‑year-old male patient was adm tted with the signs of hematuria and anemia to the Unit of Urology, Clinic of General Surgery, Siberian State Medical University. He was found to have synchronous bilateral renal cell carcinoma and simultaneous bilateral adrenal involvement. Sequential surgical treatment – radical nephrectomy (with adrenal gland removal on the right side and, after 3 months, adrenalectomy and kidney resection on the left side were performed. All the organs removed displayed tumors that proved to be renal cell carcinomas (a clear cell variant. There were lymph node metastases in the right-sided renal portal. Postoperatively, the investigators performed hormone replacement therapy for adrenal insufficiency, an immunotherapy cycle, three cycles of targeted therapy withsorafenib and sunitinib (at an interval of 0.5–2 years, and insulin therapy for new-onset diabetes mellitus. The duration of a follow-up was 6.2 years. When describing the case, the patient was alive and showed a generalized tumorous process with extensive tumor involvement of the solitary kidney. Sunitinib therapy was used.

  9. 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.

  10. 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

  11. Erythropoietin deficiency in acute crescentic glomerulonephritis and in total bilateral renal cortical necrosis

    DEFF Research Database (Denmark)

    Thaysen, J H; Nielsen, O J; Brandi, L

    1991-01-01

    -life and plasma clearance of intravenously injected recombinant human erythropoietin (rhEPO) were determined. The results indicate that the lack of compensatory increase in serum EPO to the anaemic stimulus is not due to increased catabolism, but to decreased synthesis of the renal hormone. Two patients were...

  12. MR-Guided PTA in Experimental Bilateral Rabbit Renal Artery Stenosis and MR Angiography Follow-Up Versus Histomorphometry

    International Nuclear Information System (INIS)

    Le Blanche, Alain-Ferdinand; Rossert, Jerome; Wassef, Michel; Levy, Bernard; Bigot, Jean-Michel; Boudghene, Frank

    2000-01-01

    Purpose: To assess in vivo 1) MR-guided percutaneous transluminal renal angioplasty (PTRA) in experimental bilateral rabbit renal artery stenosis (RAS); 2) postprocedural follow-up by gadolinium-enhanced MR angiography versus histomorphometry.Methods: Fifteen male NZW rabbits of mean weight 4.0 kg (range 3.5-4.2 kg) underwent bilateral RAS induction by combined overdilation-deendothelialization with a gadolinium-filled balloon, passively MR-guided by the artifact of a 0.014-inch guidewire. After 4 weeks the rabbits were randomized into two groups: group A (n = 8) underwent right-sided PTRA for treatment of RAS, group B (n = 7) underwent left-sided PTRA. After another 4 weeks the rabbits were killed to assess by histomorphometry recurrent stenosis and contralateral induction injury stenosis lesions. Each step was preceded by gadolinium-enhanced three-dimensional MR angiography, and the cortex-to-aorta (C/A) signal intensity ratio was calculated.Results: RAS induction was successful in all cases. Fourteen arteries developed restenosis and 13 only initial stenosis. MR-guided PTRAs were feasible in 22 arteries (73%). For a successful catheterization of the ostium (20 arteries, 66% success rate), 10-25 steps were required. Five to eight steps were required for balloon localization and inflation for each PTRA. The restenosis effect was reflected by a 16% (12%-27%) decrease in C/A values on MR angiograms (p < 0.05).Conclusion: MR guidance and MR angiography represent a feasible, less invasive alternative for performing and assessing experimental PTRA in RAS

  13. 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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  15. 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.)

  16. Bilateral Proximal Femur Fractures in a Patient with Renal Tubular Acidosis: A Case Report

    Directory of Open Access Journals (Sweden)

    Charl SS

    2018-03-01

    Full Text Available The diagnosis of pathological fractures is on the rise. The morbidity involved does not only burden the patient and their families but it has a great toll on the healthcare system as well. Early identification of the patient at risk is an invaluable tool to cut cost and improve the patient’s quality of life. Multiple renal pathologies have been highlighted in relation to the risk of pathological fractures; however, complications in renal tubular acidosis have been rarely documented. Nevertheless, prompt action with adequate and relevant patient education ultimately can reduce the associated morbidity. We present a case of poor control of the disease and its debilitating pathological fracture complications.

  17. The diagnostic value of contrast-enhanced CT in Acute bilateral renal cortical necrosis: a case report

    International Nuclear Information System (INIS)

    Choi, Pil Youb; Lee, Su Han; Lee, Woo Dong

    1996-01-01

    Acute renal cortical necrosis in which there is destruction of the renal cortex and sparing of the renal medulla, is a relatively rare cause of acute renal failure. A definitive diagnosis of acute renal cortical necrosis is based on renal biopsy, but on CT(computed tomography) the rather specific contrast-enhanced appearance of acute renal cortical necrosis has been described. As renal biopsy is not available, contrast-enhanced CT is a useful, noninvasive investigate modality for the early diagnosis of acute renal cortical necrosis. We report the characteristic CT findings of acute renal cortical necrosis in a patient with acute renal failure following an operation for abdominal trauma

  18. Bilateral primary renal lymphoma in a pediatric patient: staging and response evaluation with ¹⁸F-FDG PET/CT.

    Science.gov (United States)

    Dhull, V S; Mukherjee, A; Karunanithi, S; Durgapal, P; Bal, C; Kumar, R

    2015-01-01

    Primary renal lymphoma (PRL) is a rare disease. We here present the case of an 8-year-old child who presented with bilateral renal masses. On biopsy, it was confirmed to be B-cell non-Hodgkin's lymphoma. (18)F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography-computed tomography (PET/CT) for staging demonstrated (18)F-FDG avid bilateral renal masses, with no other abnormal focus. Follow up (18)F-FDG PET/CT showed complete resolution of the disease after six cycles of chemotherapy. Here we have highlighted the potential role of (18)F-FDG PET/CT in staging and response evaluation of a patient with PRL and presented a brief review. Copyright © 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved.

  19. Cryptophthalmos and Bilateral Renal Agenesis with Cleft Lip and Palate: Fraser Syndrome: Case Report

    Directory of Open Access Journals (Sweden)

    Emre Pabuçcu

    2012-12-01

    Full Text Available Fraser syndrome is a rare autosomal recessive disorder consisting of multiple anomalies including variable expression of cryptophthalmos, syndactyly, abnormal genitalia, malformations of the nose, ear and larynx, renal agenesis, oro-facial clefts, skeletal defects, umbilical hernia and mental retardation. Antenatally detected multiple congenital fetal anomalies during 22nd week of gestation is reported in this paper. Fraser Syndrome was diagnosed according to major and minor criteria. Early antenatal detection is mandatory and clinician should be awere of the high recurrence rates of this syndrome among siblings threatening subsequent pregnancies and should inform affected families.

  20. Bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman: a case report.

    Science.gov (United States)

    Pallangyo, Pedro; Lyimo, Frederick; Nicholaus, Paulina; Masatu, Stephano; Janabi, Mohamed

    2016-12-03

    Renal vein anomalies are relatively infrequent and generally asymptomatic. Preoperative knowledge of such variants is, however, of paramount importance in several angiographic and surgical procedures including renal venography, renal vein sampling, spermatic embolization, and renal transplantation. Inadequate knowledge and failure to recognize such anatomic variations may lead to several operative hazards including hemorrhage, nephrectomy, and even death. We report a case of bilateral anomalous drainage of the posterior divisions of renal veins into the azygos venous system in a 20-year-old woman of African descent from Tanzania who presented to us with a 12-year history of recurrent anemia. She had anemia, a positive sickling test, and hemoglobin electrophoresis revealed a sickle cell trait (AS). She underwent computed tomography angiography of her chest and abdomen to rule out the presence of arteriovenous malformations. Aortography findings were normal but venography results revealed features of tortuously dilated azygos and hemiazygos veins each receiving blood from its respective posterior division of renal vein. Although venous anomalies are relatively infrequent and generally lack a clinical significance, a thorough understanding of embryologic development and its associated errors is of immense importance in equipping angiographers and surgeons to select appropriate interventional/operative techniques, anticipate risks, and prevent intervention-related complications.

  1. 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 ...

  2. Management for Patients with De Novo or Recurrent Tumors in the Residual Kidney after Surgery for Nonfamilial Bilateral Renal Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Noboru Hara

    2009-01-01

    Full Text Available The tumor de novo in the residual kidney after surgery for nonfamilial bilateral renal cell carcinoma (RCC is problematic. We reviewed 5 patients who experienced such a situation. Three patients had had metachronous bilateral RCC, treated with radical nephrectomy in one kidney and nephron-sparing surgery (NSS in the other. Two patients had had synchronous disease; one patient had received radical nephrectomy and NSS, and the other bilateral NSS. The 5 patients had another solid mass/de novo tumor in the residual kidney 16–88 (mean 46.8 months after surgery. For the tumor de novo in earlier years (1992–1999, one patient underwent surgery and hemodialysis, and the other selected a conservative observation. In recent years (2000–2007, one patient was conservatively observed; the remaining 2 received computerized-tomography-guided radiofrequency ablation, and the local tumors were well controlled postoperatively for 20 and 12 months with their renal function unimpaired. Ablative techniques can potentially strike a balance between oncological and nephrological outcomes in patients with sporadic multiple RCC, successful management of which was difficult previously.

  3. MR findings in pontocerebellar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    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.

  4. 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.)

  5. Bilateral peri-renal lymphangioma - a case report and review of literature; Linfangioma perirrenal bilateral - relato de um caso e revisao bibliografica

    Energy Technology Data Exchange (ETDEWEB)

    Teixeira, Sonia Marcelino; Borges, Aurea Valeria Rosa Mohana; Dinoa, Vanessa de Albuquerque [Hospital Universitario Antonio Pedro, Niteroi, RJ (Brazil). Dept. de Radiologia; Marchiori, Edson; Mello, Walter Assis de; Teixeira, Graca Helena Maia do Canto [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Inst. Biomedico. Dept. de Radiologia

    1996-11-01

    Lymphangioma are rare benign lesions and it is difficult to state whether they are true neoplasm, hamartomas or lymphangiectasias. They are most commonly seen in children and occur in regions of the neck and axilla. They increase in size both by proliferation and by the collection of fluid, thus forming cysts. The authors report a case of a 70-year-old man, who presented abdominal swelling, a palpable mass in both flanks and polycythemia. Abdominal sonography, kidney arteriograpy, CT scan and MRI have shown multiple cysts bilaterally surrounding the kidneys without either parenchymal or functional involvement. During surgery several intercommunicating cysts were found surrounding both kidneys. Biopsies were taken from the perirenal fat, the wall of the cysts and the kidney. The histologic diagnosis was cystic lymphangioma. (author) 11 refs., 4 figs.

  6. Long-Term Outcome after Rehabilitation of Bilateral Total Hip Arthroplasty in Renal Transplant Recipient – A Case Report

    Directory of Open Access Journals (Sweden)

    Erieta Nikolikj Dimitrova

    2016-02-01

    CONCLUSION: Rehabilitation is integral part of multidisciplinary treatment of renal transplant recipient after total hip arthroplasty. Regular exercise training of these patients is very important for improving of their long-term outcome.

  7. Meier–Gorlin syndrome: An additional Egyptian patient with gastroesophageal reflux, hydronephrosis, renal stones and hypoplastic labia majora and minora with clitromegaly

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2016-10-01

    Full Text Available We report a 4.5 year old female child with the classical triad of Meier–Gorlin syndrome (microtia, absent patella and short stature with normal mentality. She had small triangular face, long peaked nose, high nasal bridge, bilateral low set very small ears (microtia, retromicrognathia, high arched palate, maxillary hypoplasia, decayed teeth, and bilateral partial syndactyly between 2nd and 3rd toes. Our patient had a gastroesophageal reflux, renal stones, hydronephrosis and hypoplastic labia majora and minora with clitromegaly.

  8. 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.

  9. 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.

  10. Bilateral renal metastasis of 261-265huerthle cell thyroid cancer with discordant uptake between I-131 sodium iodide and F-18 FDG

    Energy Technology Data Exchange (ETDEWEB)

    Claimon, Apichaya; Suh, Min Seok; Cheon, Gi Jeong; Lee, Dong Soo; Chung, June Key [Dept. of Nuclear Medicine, Seoul National University Hospital, Seoul (Korea, Republic of); Kim, E. Edmund [Dept. of Radiological Sciences, University of California, Irvine (United States)

    2017-09-15

    Renal metastasis of thyroid cancer is extremely rare. We report the case of a 62-year-old woman with Hürthle cell thyroid cancer (HCTC) with lungs, bones, and bilateral kidneys metastases. The renal metastatic lesions were clearly demonstrated by {sup 131}I whole body scan (WBS) with SPECT/CT. However, they exhibited false-negative results in {sup 18}F-FDG PET/CT, kidney ultrasonography, and contrast-enhanced CT scan. The findings imply that tumors have low glucose metabolism and are able to accumulate radioiodine, which is not commonly found in the relatively aggressive nature of HCTC. The patient received two sessions of 200 mCi {sup 131}I therapy within 6 months duration. There was complete treatment response as evaluated by the second post-therapeutic {sup 131}I SPECT/CT and serum thyroglobulin. To our knowledge, renal metastasis from HCTC with positive {sup 131}I but negative {sup 18}F-FDG uptake has not been reported in the literature. This case suggests that {sup 131}I SPECT/CT is useful for lesion localization and prediction of {sup 131}I therapy response.

  11. Fatal bilateral dioctophymatosis.

    Science.gov (United States)

    Li, Gang; Liu, Caigang; Li, Fang; Zhou, Maoyi; Liu, Xiangyong; Niu, Yuanjie

    2010-12-01

    Dioctophyma renale is a parasite that frequently occurs in animals but rarely in humans. The present report describes the clinical observations of a D. renale infection in a 51-yr-old woman. Its clinical signs and diagnostic findings were unspecific until giant worms were observed in the urine and histological findings confirmed it was a D. renale infection. She refused treatment and died of bilateral renal function failure. This is the first confirmed report to follow the natural progression of D. renale infection in a human. Here, we discuss a conservative therapeutic approach and features associated with this parasitic infection.

  12. Bilateral duplication of the internal auditory canal

    International Nuclear Information System (INIS)

    Weon, Young Cheol; Kim, Jae Hyoung; Choi, Sung Kyu; Koo, Ja-Won

    2007-01-01

    Duplication of the internal auditory canal is an extremely rare temporal bone anomaly that is believed to result from aplasia or hypoplasia of the vestibulocochlear nerve. We report bilateral duplication of the internal auditory canal in a 28-month-old boy with developmental delay and sensorineural hearing loss. (orig.)

  13. 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.)

  14. Prune belly syndrome associated with bilateral multicystic dysplastic kidneys and urethral obstruction: A case report

    Directory of Open Access Journals (Sweden)

    Arzu Akdag

    2015-06-01

    Full Text Available Prune belly syndrome is a rare congenital disorder defined by a characteristic clinical triad: Abdominal muscle deficiency, severe urinary tract abnormalities, and bilateral cryptorchidism. We describe a preterm neonate of Prune Belly syndrome who had abdominal muscle deficiency, multicystic dysplastic kidney, urethral hypoplasia and pulmonary hypoplasia. We presented this rare case with the data gathered from the literatüre.

  15. sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

    Energy Technology Data Exchange (ETDEWEB)

    Hosokawa, S; Daijo, K; Okabe, T; Kawamura, J; Hara, A [Kyoto Univ. (Japan). Hospital

    1979-08-01

    Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1.

  16. sup(99m)Tc-DMSA renal scintigraphy in renal failure due to various renal diseases

    International Nuclear Information System (INIS)

    Hosokawa, Shin-ichi; Daijo, Kazuyuki; Okabe, Tatsushiro; Kawamura, Juichi; Hara, Akira

    1979-01-01

    Renal contours in renal failure were studied by means of sup(99m)Tc-dimercaptosuccinic acid (DMSA) renoscintigraphy. Renal cortical images were obtained even in renal failure cases. Causes of renal failure were chronic glomerulonephritis in 7, bilateral renal tuberculosis in 2, chronic pyelonephritis in 3, bilateral renal calculi in 3, diabetic nephropathy in 2, polycystic kidney disease in 2 and stomach cancer in 1. (author)

  17. Live Donor Renal Transplant With Simultaneous Bilateral Nephrectomy for Autosomal Dominant Polycystic Kidney Disease Is Feasible and Satisfactory at Long-term Follow-up.

    Science.gov (United States)

    Ahmad, Sarwat B; Inouye, Brian; Phelan, Michael S; Kramer, Andrew C; Sulek, Jay; Weir, Matthew R; Barth, Rolf N; LaMattina, John C; Schweitzer, Eugene J; Leeser, David B; Niederhaus, Silke V; Bartlett, Stephen T; Bromberg, Jonathan S

    2016-02-01

    Timing of bilateral nephrectomy (BN) is controversial in patients with refractory symptoms of autosomal dominant polycystic kidney disease (APKD) in need of a renal transplant. Adults who underwent live donor renal transplant (LRT) + simultaneous BN (SBN) from August 2003 to 2013 at a single transplant center (n = 66) were retrospectively compared to a matched group of APKD patients who underwent LRT alone (n = 52). All patients received general health and polycystic kidney symptom surveys. Simultaneous BN increased operative duration, estimated blood loss, transfusions, intravenous fluid, and hospital length of stay. Most common indications for BN were pain, loss of abdominal domain, and early satiety. There were more intraoperative complications for LRT + SBN (6 vs 0, P = 0.03; 2 vascular, 2 splenic, and 1 liver injury; 1 reexploration to adjust graft positioning). There were no differences in Clavien-Dindo grade I or II (39% vs 25%, P = 0.12) or grade III or IV (7.5% vs 5.7%, P = 1.0) complications during the hospital course. There were no surgery-related mortalities. There were no differences in readmission rates (68% vs 48%, P = 0.19) or readmissions requiring procedures (25% vs. 20%, P = 0.51) over 12 months. One hundred percent of LRT + SBN allografts functioned at longer than 1 year for those available for follow-up. Survey response rate was 40% for LRT-alone and 56% for LRT + SBN. One hundred percent of LRT + SBN survey responders were satisfied with their choice of having BN done simultaneously. Excellent outcomes for graft survival, satisfaction, and morbidity suggest that the combined operative approach be preferred for patients with symptomatic APKD to avoid multiple procedures, dialysis, and costs of staged operations.

  18. A Correlation between Renal Anomalies and Vesicoureteral Reflux

    International Nuclear Information System (INIS)

    Kim, Seung Soo; Kim, Young Tong; Kim, Il Young; Shin, Hyeong Cheol

    2011-01-01

    To investigate the frequency of vesicoureteral reflux (VUR) in children with renal anomalies a evaluate the correlation between renal anomalies and VUR. Eighty-one children (1 day-8 years) with renal anomalies underwent voiding cystourethrogram between 2006 and 2009 were reviewed. This study included ureteropelvic junction stenosis (n = 32), ureteropelvic duplication (n = 20), multicystic dysplastic kidney (n = 12), fusion anomaly (n = 11), renal agenesis (n = 3), unilateral renal hypoplasia (n = 2), and ectopic kidney (n = 1). The frequency, grade, and location of VUR were evaluated. The grade of VUR according to age and anomaly type was statistically analyzed, and the patients with VUR were followed. The VUR was present in 14 (17.3%); ipsilateral VUR was present in 8 (57.1%), bilateral VUR in 4 (28.6%), and contralateral VUR in 2 (14.2%). VUR was detected in 9 patients under the age of one. There was no statistical correlation between VUR grade and either age or anomaly type of the nine patients showed continuous VUR on up. The frequency of VUR in children with renal anomalies was 17.3%. VUR was most frequently detected in children under the age of one, and VUR grade was not related to age and anomaly type.

  19. A Correlation between Renal Anomalies and Vesicoureteral Reflux

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seung Soo; Kim, Young Tong; Kim, Il Young; Shin, Hyeong Cheol [Dept. of Radiology, Cheonan Hospital, Soonchunhyang University College of Medicine, Cheonan (Korea, Republic of)

    2011-12-15

    To investigate the frequency of vesicoureteral reflux (VUR) in children with renal anomalies a evaluate the correlation between renal anomalies and VUR. Eighty-one children (1 day-8 years) with renal anomalies underwent voiding cystourethrogram between 2006 and 2009 were reviewed. This study included ureteropelvic junction stenosis (n = 32), ureteropelvic duplication (n = 20), multicystic dysplastic kidney (n = 12), fusion anomaly (n = 11), renal agenesis (n = 3), unilateral renal hypoplasia (n = 2), and ectopic kidney (n = 1). The frequency, grade, and location of VUR were evaluated. The grade of VUR according to age and anomaly type was statistically analyzed, and the patients with VUR were followed. The VUR was present in 14 (17.3%); ipsilateral VUR was present in 8 (57.1%), bilateral VUR in 4 (28.6%), and contralateral VUR in 2 (14.2%). VUR was detected in 9 patients under the age of one. There was no statistical correlation between VUR grade and either age or anomaly type of the nine patients showed continuous VUR on up. The frequency of VUR in children with renal anomalies was 17.3%. VUR was most frequently detected in children under the age of one, and VUR grade was not related to age and anomaly type.

  20. 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.

  1. 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

  2. Múltiplos fibroadenomas bilaterais após transplante renal e imunossupressão com ciclosporina A Multiple bilateral fibroadenomas after kidney transplantation and immunossuppression with cyclosporine A

    Directory of Open Access Journals (Sweden)

    Afonso Celso Pinto Nazário

    2007-07-01

    Full Text Available O fibroadenoma é a neoplasia benigna mais freqüente da mama feminina e é considerado tumor misto, constituído por quantidades variáveis de tecido conjuntivo e epitelial. A ciclosporina parece ter implicações no desenvolvimento de fibroadenomas mamários em pacientes transplantadas renais em idade reprodutiva. Descrevemos o caso no qual a paciente, em uso terapêutico de ciclosporina A, após transplante renal, apresentou vários nódulos mamários bilaterais na evolução. O exame físico e os achados de imagem sugeriram fibroadenoma, diagnóstico que foi confirmado após biópsias.Fibroadenoma is the most frequent benign neoplasia in the female breast and it is considered a mixed tumor, constituted by variable amounts of connective and epithelial tissue. Cyclosporine A seems to be related with the development of mamary fibroadenomas in patients who underwent kidney transplantation in reproductive age. We reported the case in which the patient, in therapeutic use of cyclosporine A, after kidney transplantation, presented several bilateral lumps. The imaging and palpable findings suggested fibroadenoma, confirmed after biopsy.

  3. 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)

  4. 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 ...

  5. 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.

  6. 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.

  7. 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.

  8. Bilateral microperc in a severe kyphoscoliosis

    OpenAIRE

    Dağgülli, Mansur; Penbegül, Necmettin; Dede, Onur; Utanğaç, Mehmet Mazhar

    2016-01-01

    Percutaneous nephrolithotomy is the standard modality for large renal calculi in normal and abnormal renal anatomic situations. This case report describes a 57-year-old male patient who presented with bilateral kidney stones and severe kyphoscoliosis. He had successfully been treated with a bilateral microperc technique.

  9. BILATERAL SINGLE SESSION URETEROSCOPY FOR URETERAL ...

    African Journals Online (AJOL)

    Objectives: To determine the feasibility, safety and success rate of bilateral single session rigid retrograde ureteroscopy (URS) for bilateral ureteral calculi. Patients and Methods: Thirty-five patients underwent bilateral single session ureteroscopic calculus removal. Results: Out of 70 renal units in 35 patients treated, ...

  10. 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

  11. 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.)

  12. 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.

  13. 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

  14. 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

  15. [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.

  16. 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.)

  17. Renal abnormalities in congenital chloride diarrhea

    International Nuclear Information System (INIS)

    Al-Hamad, Nadia M.; Al-Eisa, Amal A.

    2004-01-01

    Congenital chloride diarrhea CLD is a rare autosomal recessive disorder caused by a defect in the chloride/ bicarbonate exchange in the ileum and colon. It is characterized by watery diarrhea, abdominal distension, hypochloremic hypokalemic metabolic alkalosis with high fecal content of chloride >90 mmol/l. We report 3 patients with CLD associated with various renal abnormalities including chronic renal failure secondary to renal hypoplasia, nephrocalcinosis and congenital nephrotic syndrome. (author)

  18. Irreversibel nyreinsufficiens under behandling med angiotensin I konverterende enzymhaemmer ved bilateral nyrearteriestenose

    DEFF Research Database (Denmark)

    Kamper, A L

    1989-01-01

    A case of irreversible renal failure during treatment with enalapril in bilateral renal artery stenosis is described. In the use of converting enzyme inhibitors, caution and monitoring of renal function during treatment is advised....

  19. 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.

  20. 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)

  1. 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

  2. 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 ...

  3. Severe rhabdomyolysis and acute renal failure in an adolescent with hypothyroidism.

    Science.gov (United States)

    Comak, Elif; Koyun, Mustafa; Kiliçarslan-Akkaya, Bahar; Bircan, Iffet; Akman, Sema

    2011-01-01

    Hypothyroidism has been reported rarely as the cause of rhabdomyolysis in adults and children. We present here a non-compliant adolescent with a diagnosis of hypothyroidism who developed rhabdomyolysis and acute renal failure with no additional predisposing factor. A 13-year-old girl with a previous history of hypothyroidism due to thyroid hypoplasia presented with generalized myalgia, malaise, vomiting, and oliguria lasting for three days. Neurological examination revealed bilateral marked weakness and tenderness of muscles of both lower and upper extremities. Urine had bloody appearance and urine analysis showed blood reaction with dipstick test, but there were no erythrocytes on microscopic examination. Serum creatine phosphokinase and myoglobin levels were elevated. Thyroid stimulating hormone (TSH) levels were high, and free thyroxine (T4) and triiodothyronine (T3) levels were low, compatible with uncontrolled hypothyroidism. Renal function tests showed acute renal failure. Other causes of rhabdomyolysis such as muscular trauma, drugs, toxins, infections, vigorous exercise, and electrolyte abnormalities were excluded. Hemodialysis was administered for 24 sessions. After L-thyroxine therapy, thyroid function tests normalized, muscle strength improved, serum muscle enzyme levels returned to normal levels, and renal function tests recovered. One must be aware that rhabdomyolysis may develop in a non-compliant patient with hypothyroidism.

  4. [Bilateral torticollis].

    Science.gov (United States)

    Kustos, T; Magdics, M

    1993-12-19

    Bilateral torticollis is a very rare form of a well known deformity i.e. muscular torticollis. This malformation might present a differential diagnostic problem both for orthopaedic surgeons and ophthalmologists, as well as, for neurologists. In agreement with the literature, the role of an injury during labour or defective embriogenesis is suggested to play a part.

  5. 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.

  6. Bilateral hydronephrosis

    Science.gov (United States)

    ... problem are often found before birth during a pregnancy ultrasound. A urinary tract infection in a newborn baby can signal a blockage ... for blockage. A higher than normal number of urinary tract infections is often the only symptom of the problem. Common symptoms in ... (fetal) ultrasound Renal scan Ultrasound of the abdomen ...

  7. Ascites, a New Cause for Bilateral Hydronephrosis: Case Report

    Science.gov (United States)

    Jain, Deepika; Dorairajan, Smrita; Misra, Madhukar

    2009-01-01

    Bilateral hydronephrosis secondary to urinary obstruction leads to a buildup of back pressure in the urinary tract and may lead to impairment of renal function. We present a case of a 57-year-old male with a history of alcoholic liver cirrhosis, who presented with tense ascites and acute renal failure. Bilateral hydronephrosis was seen on abdominal ultrasound. Multiple large-volume paracenteses resulted in resolution of hydronephrosis and prompt improvement in renal function. PMID:19802498

  8. Bilateral agreements

    International Nuclear Information System (INIS)

    Anon.

    2004-01-01

    The bilateral agreements concern Brazil with United States relative to the co operation in nuclear energy, Germany with Russian Federation relative to the elimination and disposal of nuclear weapons; The multilateral agreements concerns the signature of the Protocols to amend the Paris and Brussels Conventions, the multilateral nuclear environmental programme in the Russian Federation, the status of Conventions in the field of nuclear energy. (N.C.)

  9. 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.

  10. 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.

  11. Renal acidification defects in medullary sponge kidney

    DEFF Research Database (Denmark)

    Osther, P J; Hansen, A B; Røhl, H F

    1988-01-01

    Thirteen patients with medullary sponge kidney underwent a short ammonium chloride loading test to investigate their renal acidification capacity. All but 1 presented with a history of recurrent renal calculi and showed bilateral widespread renal medullary calcification on X-ray examination. Nine...... of renal calculi in medullary sponge kidney, have considerable therapeutic implications....

  12. 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.)

  13. Acute renal failure in children

    International Nuclear Information System (INIS)

    Vergesslich, K.A.; Balzar, E.; Weninger, M.; Ponhold, W.; Sommer, G.; Wittich, G.R.; Vienna Univ.

    1987-01-01

    Acute renal failure (ARF) may be due to obstructive uropathy or renal parenchymal disease. Twenty-five children with acute renal failure secondary to renal parenchymal disease underwent ultrasonographic examination of the kidneys. Changes of renal size and cortical echogenicity were correlated with renal function. All patients presented with bilaterally enlarged kidneys with the exception in renal function resulted in normalization of renal size. With regard to cortical echogenicity two groups were formed. Group A comprised 11 patients whose kidneys had the same echogenicity as the liver, while in group B the kidneys were more echogenic (14 patients). Cortical echogenicity was always increased. Determination of creatinine levels showed a statistically significant difference between group A (3.32 mg% ± 1.40 S.D.) and group B (5.95 mg% ± 1.96 S.D.), p < 0.001. Changes in renal function were paralleled by rapid changes in renal size and cortical echogenicity. (orig.)

  14. 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

  15. 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.

  16. 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.

  17. 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.)

  18. Bilateral agreements

    International Nuclear Information System (INIS)

    1998-01-01

    Ten bilateral agreements are presented. These are: 1) Co-operation agreement relating to the peaceful uses of nuclear energy between Argentina and EURATOM (1996); 2) Agreement on co-operation in the peaceful uses of nuclear energy between Argentina and Greece (1997); 3) Implementing arrangement for technical exchange and co-operation in the area of peaceful uses of nuclear energy between Argentina and the United States (1997); 4) Agreement concerning co-operation in nuclear science and technology between Australia and Indonesia (1997); 5) Implementation of the 1985 Agreement for co-operation concerning the peaceful uses of nuclear energy between the People's Republic of China and the United States (1998); 6) Protocol of co-operation between France and Lithuania (1997); 7) Agreement on co-operation in energy research, science and technology, and development between Germany and the United States (1998); 8) Agreement on early notification of a nuclear accident and exchange of information on nuclear facilities between Greece and Romania (1997); 9) Agreement on early notification of nuclear accidents and co-operation in the field of nuclear safety between Hungary and the Ukraine (1997); 10) Agreement in the field of radioactive waste management between Switzerland and the United States (1997). (K.A.)

  19. Fibromuscular dysplasia of renal arteries

    International Nuclear Information System (INIS)

    Akhtar, N.; Ahmed, T.M.

    2007-01-01

    This case reports a young child having uncontrolled hypertension, resulting from bilateral renal artery stenosis due to fibromuscular dysplasia presenting with abdominal pain, headache and visual disturbance. Diagnostic features and management is discussed. (author)

  20. Bilateral Supernumerary Kidney: A Very Rare Presentation

    International Nuclear Information System (INIS)

    Keskin, Suat; Batur, Abdussamet; Keskin, Zeynep; Koc, Abdulkadir; Firat Ozcan, Irfan

    2014-01-01

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys

  1. Bilateral Supernumerary Kidney: A Very Rare Presentation

    Science.gov (United States)

    Keskin, Suat; Batur, Abdussamet; Keskin, Zeynep; Koc, Abdulkadir; Firat Ozcan, Irfan

    2014-01-01

    To our knowledge, bilateral supernumerary kidney is a very rare renal abnormality and there are five cases presented in the literature. It is difficult to diagnose supernumerary kidney and clinicians have not detected most cases preoperatively. Laboratory and imaging studies were acquired and carefully examined. The normal laboratory tests were found. Emergency ultrasonography was performed and they revealed no signs of parenchymal abnormality in both kidneys. Serial imaging study including enhanced computed tomography (CT) was performed. An imaging study identified bilateral supernumerary kidney with expanded collecting systems. On each side, significant rotation anomaly was found. In addition, there were two different renal arteries originating from the aorta. This report presents radiological determinations of supernumerary kidney bilaterally in a young man. We think that CT commonly appears to be enough for the diagnosis of supernumerary kidneys. PMID:25780543

  2. Bilateral clear cell sarcoma of the kidney

    International Nuclear Information System (INIS)

    Zekri, W.; Yehia, D.; Alfaar, A.S.; Elshafie, M.M.; Younes, A.A.; Zaghloul, M.S.; El-Kinaai, N.; Taha, H.; Refaat, A.; Zekri, W.; Elshafie, M.M.; Zaghloul, M.S.; Taha, H.; Refaat, A.; Younes, A.A.; Alfaar, A.S.; Yehia, D.

    2015-01-01

    Clear cell sarcoma of the kidney (CCSK) accounts for 2-5% of all pediatric renal malignancies, and is known for its propensity to metastasize to bone and other sites. We are reporting two cases with bilateral CCSK that were diagnosed at our institution. One patient initially presented with bilateral renal masses, as well as pulmonary, hepatic and bone metastasis; while other present only with bilateral masses with no evident distant metastasis. Both patients received aggressive neo-adjuvant chemotherapy to decrease tumor size. One patient completed his designated treatment and initially showed complete remission (CR); eventually suffering from relapse. The other patient’s tumor progressed during the course of chemotherapy. Both cases manifested brain dissemination at the time of relapse or progression. This emphasizes the importance of staging stratification in CCSK. This also illustrates CCSK’s ability to metastasize to bone and other sites including the brain (a primary relapse site in our cases)

  3. 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.

  4. 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.

  5. 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...

  6. Bilateral nephroblastoma - case report

    International Nuclear Information System (INIS)

    Luczynska, E.; Aniol, J.

    2008-01-01

    Wilms tumor is the most common renal tumor in children Synchronous bilateral Wilms tumor (BWT) accounts for 5% of all patients registered to the National Wilms Tumor Study Group (NWSTG). A 28-year-old female patient was presented to Oncology Institute with right kidney tumor. Her left kidney was resected due to Wilms tumor in the sixth month of her life. Abdominal ultrasound was performed and demonstrated a massive right kidney tumor. Then the abdominal CT was undertaken with the use of 16-slice CT scanner, revealing rotation displacement of the right kidney, with signs of compensative overgrowth and displaced upwards. In the lower and peripheral part of the kidney a nodular mass 7 X 10 X 9 cm in size was visible. The tumor was well-demarcated, showing heterogeneous contrast enhancement, extending from the inferio-lateral renal pole.The tumor was adjacent to iliopsoas muscle and abdominal cavity walls; no infiltration of those structures was noted. Described lesion surrounded inferio-medial part of the kidney, approaching kidney pelvis without infiltrating them. Supero-anterior and medial part of the kidney showed normal structure with correct contrasted urine excretion. Lymph nodes enlargement within periaortal area was not detected. Surgical procedure was performed resulting in excision of the tumor with kidney preservation. Histopathology examination revealed nephroblastoma. The tumor was surrounded by a thin, fibrous capsule. Surrounding parenchyma and far tissue were not involved. The CT examination performed 4 months after nephron sparing surgery revealed: right kidney of 137 X 51 mm in size, normally located, with correct structure and function. Multislice abdominal CT have clearly visualized the tumor of the single kidney, showing precise localization of the mass with relation to the kidney pelvis and vessels, allowing for nephron sparing surgical excision. (authors)

  7. Bilateral vision loss in a captive cheetah (Acinonyx jubatus).

    Science.gov (United States)

    Walser-Reinhardt, Ladina; Wernick, Morena B; Hatt, Jean-Michel; Spiess, Bernhard M

    2010-09-01

    The following case report describes a 1-year-old female cheetah (Acinonyx jubatus) with bilateral blindness and unresponsive pupils. For comparison, a second healthy 2.5-year-old male cheetah without visual deficits was also examined. Clinical examination of both animals included biomicroscopy, indirect ophthalmoscopy, tonometry, and electroretinography. The young female cheetah showed no menace response, no direct or indirect pupillary light reflex, and no dazzle reflex in either eye. Fundus lesions, as detected by indirect ophthalmoscopy, are described for the female animal. In both eyes, the fundus color was green/turquoise/yellow with multiple hyperpigmented linear lesions in the tapetal area around the optic nerve. The optic nerve head was dark gray and about half the normal size suggesting bilateral optic nerve hypoplasia and retinal dysplasia or differentially optic nerve atrophy and chorioretinal scarring. The ERG had low amplitudes in the right eye but appeared normal in the left eye compared with the male cheetah. Blood levels did not suggest current taurine deficiency. This is addressed to some degree in the discussion. Bilateral optic nerve hypoplasia or optic nerve atrophy is a rare anomaly in cats and has not yet been described in a cheetah.

  8. 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.

  9. Bilateral s-shaped kidneys: A rare congenital malformation

    Directory of Open Access Journals (Sweden)

    Nikhil Ranjan

    2015-01-01

    Full Text Available A bilateral S-shaped kidney is a rare anomaly in which both the kidneys are in their normal position, in contrast to the commonly reported S-shaped fusion anomaly, in which the contralateral kidney crosses the midline to fuse with opposite kidney leaving the ipsilateral renal fossa empty. Here we present the diagnosis and management of a case of bilateral S-shaped renal anomaly with associated left pelviureteric junction obstruction and nonfunctioning kidney and right renal stones. Left kidney was managed by open nephrectomy and right kidney by PNL.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  11. 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

  12. Bilateral tumors of the upper urothelium

    Directory of Open Access Journals (Sweden)

    Đokić Milan

    2006-01-01

    Full Text Available Introduction: The incidence of tumors of the upper urothelium is high in our country, apart from their relation to specific regions (BEN and PBEN and their frequent bilateralism. Bilateral forms are present in significant percentage and are followed, in most cases, by renal failure, which speaks in favor of conservative surgery, if possible. Objective: The aim of the study was to present epidemiological, pathoanatomical and clinical characteristics of bilateral tumors of the upper urothelium and evaluate the Results of their treatment. Method: Our retrospective study analyzed 12 patients with bilateral tumors of the upper urothelium who were treated in the period from 1992 to 1996, according to their epidemiological, clinical, pathoanatomical and pathohistological characteristics, type of surgical treatment and relevant success. Results: In the observed period, bilateral tumors of the upper urothelium were found in 8.2% of our patients. In the group of 12 patients, 5 females and 7 males, 11 cases were from the region of Balkan Endemic Nephropathy (BEN. Renal failure was recorded in high percentage (66%. Radical surgical treatment - total nephroureterectomy was performed in 9 kidney units, and conservative operation in 15 units. Relapse significantly depended on tumor stage and grade, not on type of surgical treatment in the majority of cases. Five-year survival was 58.33%; major cause of death was associated with further evolution of tumor, recurrence and tumor dissemination, respectively, while renal failure complications were the cause of death in one case. Conclusion: The success of treatment mainly depends on tumor stage and grade and not on type of surgical Method in conservative treatment, but renal failure and its complications are an important risk factor in these patients.

  13. Isolated bilateral congenital lacrimal gland agenesis – Report of two cases

    Directory of Open Access Journals (Sweden)

    Manar Alwohaib

    2017-10-01

    Full Text Available Congenital lacrimal gland agenesis, also called congenital alacrima, is a rare cause of dry eye and is characterized by aplasia or hypoplasia of lacrimal glands. We present two 5-year old children with congenital lacrimal gland agenesis. The two cases had the final diagnosis of isolated bilateral congenital lacrimal gland agenesis and we document the clinical aspects, treatment and present a literature review related to this rare condition. Keywords: Alacrima, Lacrimal gland, Lacrimal gland agenesis, Punctal plugs

  14. 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.

  15. Study of acute renal insufficiency and chronic renal insufficiency using radioisotopes

    International Nuclear Information System (INIS)

    Raynaud, C.

    1976-01-01

    Radioisotopic renal function tests are of assistance to diagnose and follow-up the course of renal insufficiency. The radioisotopic renogram is useful in assessing the response to therapy of child obstructive uropathies and evaluating renal transplant function. The renal scan is helpful, in an emergency service, to differenciate chronic renal insufficiency from acute renal insufficiency. Hg renal uptake test provides informations on physiopathological problems. Among them, the following problems are emphasized: evolution of a nonfunctioning kidney, control of the success of a reparative surgery and of bilateral obstructive uropathies with unilateral symptoms [fr

  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. BILATERAL HYDRONEPHROSIS IN A SUGAR GLIDER (PETAURUS BREVICEPS).

    Science.gov (United States)

    Cusack, Lara; Schnellbacher, Rodney; Howerth, Elizabeth W; Jiménez, David A; Mayer, Joerg; Divers, Stephen

    2016-09-01

    An adult, intact male sugar glider ( Petaurus breviceps ) presented for acute caudal abdominal swelling. Treatment by the referring veterinarian included aspiration of urine from the swelling. On physical examination, mild depression, pale mucus membranes, and caudal abdominal swelling were noted. Focused ultrasonographic assessment revealed a fluid-filled caudal abdominal structure and subjective bladder wall thickening. The following day, the sugar glider was severely depressed. Hematology results included hypoglycemia, hyperkalemia, hyponatremia, and azotemia. Ultrasonography revealed bilateral hydronephrosis and hydroureter. Despite supportive care, the animal died. Postmortem examination confirmed bilateral ureteral dilation, renal petechial hemorrhage, and dilation of the right renal pelvis. Submucosal edema, hemorrhage, and lymphoplasmacytic infiltration of the urinary bladder, ureters, and renal pelvises were noted. Hyperplasia of the urinary bladder and ureteral epithelium, coupled with inflammation, may have caused functional obstruction leading to bilateral hydronephrosis and hydroureter. This is the first reported case of hydronephrosis in a marsupial.

  19. 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.

  20. 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.

  1. 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.

  2. Bilateral type emphysematous pyelonephritis: imaging evaluation

    International Nuclear Information System (INIS)

    Salvador Cueto-Alvarez, L.; Piriz-Campos, E.; Ruiz-Garcia, T.; Roldan-Lora, F.

    2000-01-01

    Emphysematous pyelonephritis (EPN) is a serious infectious disease that occurs more frequently in diabetic patients. Radiological studies are indispensable for distinguishing between the two types since each is associated with a markedly different prognosis. We present a case of bilateral EPN diagnosed as type I on the basis of computed tomography findings, differentiating it from type II EPN, taking into account the prognostic value of this distinction. Type I EPN is characterized by the destruction of the renal parenchyma, a mottled or linear gas pattern and the absence of renal and perirenal fluid collection. Type II EPN is characterized by a loculated or bubbly gas pattern, the existence of fluid collection and less marked renal destruction. Type I is more aggressive and is associated with high rate of mortality. (Author) 7 refs

  3. Does the presence of accessory renal arteries affect the efficacy of renal denervation?

    Science.gov (United States)

    Id, Dani; Kaltenbach, Benjamin; Bertog, Stefan C; Hornung, Marius; Hofmann, Ilona; Vaskelyte, Laura; Sievert, Horst

    2013-10-01

    This study sought to assess the efficacy of catheter-based renal sympathetic denervation in patients with accessory renal arteries and to compare the blood pressure (BP)-lowering effect with that observed in patients with bilateral single renal arteries after renal denervation. Catheter-based renal sympathetic denervation causes significant BP reductions in patients with resistant hypertension. Seventy-four patients were included in this study. Patients were assigned to 2 main groups: a bilateral single renal arteries group I (n = 54) and an accessory renal arteries group II (n = 20). Group II consisted of 9 patients whose accessory renal arteries were all denervated (group IIa), and 11 patients whose accessory renal arteries were not, or only incompletely, denervated (group IIb). The primary endpoint was the change in office systolic BP after 6 months. The procedure was successful in all patients. Group I: mean BP at baseline was 166.2/89.4 ± 20.5/14.6 mm Hg and decreased by -16.6 (p renal denervation in patients with accessory renal arteries is less pronounced than in patients with bilateral single renal arteries. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  4. Renal manifestations in children with Alagille syndrome.

    Science.gov (United States)

    Di Pinto, Diana; Adragna, Marta

    2018-04-01

    Alagille syndrome (AS) is a cholestatic disease secondary to scarcity of interlobular bile ducts. It is associated with extrahepatic manifestations, and renal involvement is frequent. To describe the prevalence, type and outcome of renal pathology in children with AS. The presence and outcome of renal pathology was retrospectively studied in 21 children who met AS criteria. Renal pathology was observed in 18 patients (85.7%): (1) ultrasound variations in 7 patients (6 cases of bilateral renal dysplasia and 1 case of renal agenesis); (2) distal renal tubular acidosis in 2 patients; (3) a drop in glomerular filtration and/or proteinuria in 16 patients. The frequency of a drop in glomerular filtration was similar between patients with and without pathological kidney ultrasound findings. Our study confirms a high prevalence of renal involvement, which enhances the importance of diagnosis and renal function follow-up in children with AS. Sociedad Argentina de Pediatría.

  5. Prenatal natural history of isolated fetal mild bilateral pyelectasis

    Directory of Open Access Journals (Sweden)

    Gustavo de Paula Pereira

    Full Text Available OBJECTIVE: To analyze the prenatal outcomes in a cohort of fetuses with mild bilateral pyelectasis and determine whether performing serial ultrasounds is a good follow-up strategy. METHODS: A prospective longitudinal study was conducted on 62 fetuses with mild bilateral pyelectasis. Fetal mild bilateral pyelectasis was considered when the renal pelvis measured (in millimeters ≥5.0 to 10.0, ≥7.0 to 10.0, and ≥10.0 to 15 at ≤23 weeks 6 days, 24 to 31 weeks 6 days, and ≥32 weeks, respectively, with no uretero-calyceal dilatation. Ultrasounds were performed every 3 weeks to assess whether the mild bilateral pyelectasis regressed, remained unchanged (Group 1 or progressed (Group 2. RESULTS: Group 1 consisted of 53 fetuses (85.4%, and progression was observed in 9 cases (Group 2, 14.6%. The initial renal pelvis diameter was significantly larger in fetuses with progression (p=0.028. Statistically significant differences in the renal pelvis diameter were also found at weeks 31 and 35 for both kidneys (p<0.05. The cases requiring intrauterine procedures or early delivery were not observed. CONCLUSION: Fetal mild bilateral pyelectasis with no calyceal dilatation is a benign condition that can be managed in the postnatal period. The initial renal pelvis diameter and the diameter in week 31 or 35 were valuable parameters for identifying cases that would eventually need specific postnatal procedures.

  6. Renal pelvis urothelial carcinoma of the upper moiety in complete right renal duplex: a case report.

    Science.gov (United States)

    Zhang, Yiran; Yu, Quanfeng; Zhang, Zhihong; Liu, Ranlu; Xu, Yong

    2015-01-01

    Urothelial carcinoma (UC) originated from renal pelvis is the common tumor of the urinary system, however, neoplasia of the renal pelvis in duplex kidneys is extremely rare, especially in the complete renal and ureteral duplex cases. We present the first case of renal pelvis UC of the upper moiety in a complete right renal duplex. This male patient has bilateral complete renal and ureteral duplex. To the best of our knowledge, this is the first reported case of renal pelvis UC in a complete renal duplex system. After this experience we feel that the diagnosis of renal pelvis UC in duplex kidneys is not so easy, and once the diagnosis is determined, the whole renal duplex units and bladder cuff or ectopic orifice should be excised radically.

  7. Bilateral herpes zoster

    Directory of Open Access Journals (Sweden)

    Singh K

    1993-01-01

    Full Text Available A case of bilateral herpes zoster of lumbosacral region is reported in association with diabetes mellitus in a 55 years old female. The case is of interest due to bilateral distribution which is rare and sacral region involvement which is quite uncommon.

  8. Bilateral herpes zoster

    OpenAIRE

    Singh K; Bajaj A; Dwivedi N; Merchery A

    1993-01-01

    A case of bilateral herpes zoster of lumbosacral region is reported in association with diabetes mellitus in a 55 years old female. The case is of interest due to bilateral distribution which is rare and sacral region involvement which is quite uncommon.

  9. 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.

  10. Unilateral occlusion of duplicated uterus with ipsilateral renal anomaly in young girls: a study with MRI

    International Nuclear Information System (INIS)

    Li, Y.W.; Shieh, C.P.; Chen, W.J.

    1995-01-01

    Twenty-four young girls (mean age 13.0 years) with unilateral occlusion of a duplicated uterus and ipsilateral renal agenesis, dysplasia or hypoplasia were studied with magnetic resonance imaging (MRI) following ultrasound examination. Hydrocolpos (n=4), hydrometrocolpos (n=2), hematocolpos (n=11), hematometrocolpos (n=5), hematocolpometra, hematosalpinx (n=3) and hematometra, hematosalpinx (n=1) were noted (two of these patients had presented with hydrocolpos and hematocolpos before and after the menarche). Twenty-two of these girls presented with ipsilateral renal agenesis (right 11, left 11) with ectopic ureters to Gartner's dust cysts (GDC) in two, in one renal hypoplasia and in one renal dysplasia with ectopic ureters to GDC. MRI offered specific images of the genital tract, showing the exact type of muellerian duct anomaly and providing high diagnostic accuracy. Such preoperative identification of a uterine anomaly, complemented with appropriate surgical intervention, can assist young girls in achieving normal fertility in the future. (orig.)

  11. [Bilateral cochlear implants].

    Science.gov (United States)

    Müller, J

    2017-07-01

    Cochlear implants (CI) are standard for the hearing rehabilitation of severe to profound deafness. Nowadays, if bilaterally indicated, bilateral implantation is usually recommended (in accordance with German guidelines). Bilateral implantation enables better speech discrimination in quiet and in noise, and restores directional and spatial hearing. Children with bilateral CI are able to undergo hearing-based hearing and speech development. Within the scope of their individual possibilities, bilaterally implanted children develop faster than children with unilateral CI and attain, e.g., a larger vocabulary within a certain time interval. Only bilateral implantation allows "binaural hearing," with all the benefits that people with normal hearing profit from, namely: better speech discrimination in quiet and in noise, as well as directional and spatial hearing. Naturally, the developments take time. Binaural CI users benefit from the same effects as normal hearing persons: head shadow effect, squelch effect, and summation and redundancy effects. Sequential CI fitting is not necessarily disadvantageous-both simultaneously and sequentially fitted patients benefit in a similar way. For children, earliest possible fitting and shortest possible interval between the two surgeries seems to positively influence the outcome if bilateral CI are indicated.

  12. 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.

  13. 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

  14. CT findings of renal abscess

    International Nuclear Information System (INIS)

    Lee, Myung Jun; Kim, Mi Young; Woo, Jung Ju; Kim, Ho Kyun; Kim, Won Hong; Jeon, Jeong Dong; Jeon, Woo Ki; Han, Chang Yul

    1996-01-01

    The purpose of this study is to determine characteristic CT findings in renal abscess. Twenty cases of renal abscess were retrospectively analyzed for CT findings relating to the shape and extent of the abscess, change of nephrogram, peripheral rim enhancement, wedge-shaped enhancement on delayed scans, enlargement of the kidney involved and associated findings. Seven patients had a renal abscess at the right kidney, nine at the lift kidney and two bilaterally. The abscesses were round in 18 cases and finger-like in two. Rim enhancement around renal abscess was seen in four cases (20%). Changes in the nephrogram around the abscess were seen in 12 cases (60%). In all six patients who had undergone delayed postcontrast scans, wedge-shaped enhancement was shown around the abscess (100%). In the observation of the extent of renal abscesses, 14 cases were within the kidney, six cases extended the beyond renal capsule, and two were loculated in the renal fascia itself. Renal enlargement was seen in nine cases (45%). These results suggest that CT findings such as delayed wedge-shaped enhancement, change of nephrogram, peripheral rim enhancement, renal enlargement, and associated findings are valuable for diagnosis, and that CT also gives information concerning the extent, evolution and complication of a renal abscess

  15. Pregnancy following bilateral salpingectomy

    DEFF Research Database (Denmark)

    Oturai, Annette Bang

    2008-01-01

    This report presents a rare case of spontaneous pregnancy following bilateral salpingectomy. A woman with a history of bilateral salpingectomy was admitted to hospital because of abdominal pain and positive urine HCG. Surprisingly, ultrasound confirmed a live intrauterine fetus. The pregnancy...... was unwanted, and the woman decided to terminate the pregnancy. She was offered diagnostic examination to localise a potential fistula, but she declined. In a MEDLINE search of English literature this is only the second case of spontaneous pregnancy following bilateral salpingectomy Udgivelsesdato: 2008/4/21...

  16. Bilateral ureteropelvic junction obstruction presenting with hypertension and cerebral vascular accident.

    Science.gov (United States)

    Tourchi, Ali; Kajbafzadeh, Abdolmohammad; Nejat, Farideh; Golmohammadi, Abolfazl; Alizadeh, Farshid; Mahboobi, Amir Hassan

    2010-11-01

    Cerebrovascular accident and renal hypertension secondary to ureteropelvic junction obstruction (UPJO) are extremely rare. A 6-year-old girl presented with intracranial hemorrhage because of hypertension secondary to the bilateral UPJO. This was successfully treated with craniotomy and subsequent percutaneous nephrostomy placement and bilateral pyeloplasty. Brain computerized tomography revealed right-sided intracranial hemorrhage, and renal ultrasonography confirmed bilateral severe hydronephrosis. Craniotomy with evacuation of intracerebral hematoma and bilateral nephrostomy under ultrasound guidance were performed. One week later, she underwent bilateral pyeloplasty in 2 stages. The patient has been well with normalized renal function and is completely cured of her hypertension in long-term follow-up. Copyright © 2010 Elsevier Inc. All rights reserved.

  17. 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

  18. A case report of intracranial vertebral-basilar artery hypoplasia ...

    African Journals Online (AJOL)

    It accompanied with headache, bilateral tinnitus and blurred vision. Time-of-flight magnetic resonance angiogram with T2 true fast imaging in steady state precession confirmed the VBAH, rather than a stenosis. Symptoms subsided after daily oral aspirin and life-style change has been recommended for three months.

  19. 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.

  20. 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)

  1. THE PURE RED BLOOD CELL APLASIA IN RENAL TRANSPLANT RECIPIENT

    OpenAIRE

    B. T. Dzumabaeva; L. S. Birjukova; L. B. Kaplanskaya; D. P. Maksimov

    2011-01-01

    The pure red blood cell aplasia of renal transplant recipients caused by parvovirus B19 (PB19) is characterized by persistent anemia which resistant to erythropoietin therapy, lack of reticulocytes, bone marrow hypoplasia, and clinically accompanied by severe recurrent bacterial, fungal and viral infection. In case of reactivation PB19 it is necessarv, first of all, eliminate the causes activation of this virus and to cancel or reduce the dose of drugs which depressed the normal hematopoiesis...

  2. Bilateral spontaneous rupture of flexor digitorum profundus tendons.

    LENUS (Irish Health Repository)

    O'Sullivan, S T

    2012-02-03

    Spontaneous tendon rupture is an unusual condition usually associated with underlying disease processes such as rheumatoid arthritis, chronic renal failure or bony abnormalities of the hand. We report a case of spontaneous, non-concurrent bilateral rupture of flexor profundus tendons in an otherwise healthy individual. Treatment was successful and consisted of a two-stage reconstruction of the ruptured tendon.

  3. Nephron-sparing surgery for bilateral Wilms' tumours: A single ...

    African Journals Online (AJOL)

    All three with unfavourable histology are alive. Four of the five metachronous presentations are alive, as are eight of 12 patients with synchronous bilateral tumours who presented since 2000. Conclusions: Appropriate chemotherapy and nephron-sparing surgery can achieve good results with preservation of adequate renal ...

  4. Bilateral type emphysematous pyelonephritis: imaging evaluation; Pielonefritis enfisematosa bilateral tipo I. Evaluacion por la imagen

    Energy Technology Data Exchange (ETDEWEB)

    Salvador Cueto-Alvarez, L.; Piriz-Campos, E.; Ruiz-Garcia, T.; Roldan-Lora, F. [Hospital Universitario Virgen Macarena. Sevilla (Spain)

    2000-07-01

    Emphysematous pyelonephritis (EPN) is a serious infectious disease that occurs more frequently in diabetic patients. Radiological studies are indispensable for distinguishing between the two types since each is associated with a markedly different prognosis. We present a case of bilateral EPN diagnosed as type I on the basis of computed tomography findings, differentiating it from type II EPN, taking into account the prognostic value of this distinction. Type I EPN is characterized by the destruction of the renal parenchyma, a mottled or linear gas pattern and the absence of renal and perirenal fluid collection. Type II EPN is characterized by a loculated or bubbly gas pattern, the existence of fluid collection and less marked renal destruction. Type I is more aggressive and is associated with high rate of mortality. (Author) 7 refs.

  5. Bilateral simultaneous quadriceps tendon rupture in a patient with secondary hyperparathyroidism: a case report

    International Nuclear Information System (INIS)

    Lee, Yeon Soo; Son, Sang Beom; Han, Chang Whan; Kang, Si Won

    2001-01-01

    Simultaneous bilateral rupture of the quadriceps tendon without a significant history of trauma may occur in association with chronic metabolic disorders such as chronic renal failure and secondary hyperparathyroidism, though has rarely been reported. We describe a case of spontaneous bilateral quadriceps tendon rupture in a 36-year-old female patient with secondary hyperparathyroidism

  6. Retrospective study of renal images on whole bone scanning

    International Nuclear Information System (INIS)

    Yanagisawa, Munetoshi; Machida, Toyohei; Miki, Makoto; Ohishi, Yukihiko; Ueda, Masataka

    1978-01-01

    One hundred and twenty-seven cases were surveyed by sup(99m)Tc-pyrophosphate at Jikei hospital. Renal images on whole-bone scanning were observed in all cases; 75% of all renal images were normal and 25% were abnormal. Thirteen percent of these abnormal images were symmetric and 87% were asymmetric. Four of the symmetric renal images were bilaterally bad. Three of the four bilaterally bad renal images involved prostate carcinomas with general metastases and the last involved serious bilateral hydronephrosis. The reason for the high percentage of asymmetric renal images was that the materials involved many urogenital cases. Asymmetric renal images other than the urogenital cases, were recognised in 8% of all cases. This percentage is consistent with Hattner's report. Unilateral abnormal renal images involved 8 hydronephrosis cases, 2 unilateral nonfunctioning kidneys and one malrotation kidney. Among the hydronephrosis cases, serious cases gave low uptake and mild cases gave high uptake. The reason for this phenomenon was, presumably, that there were differences in renal uptake, renal excretion and renal pelvic accumulation. In nine cases, one kidney was not visualized on whole-bone scanning, 8 of them involved nephrectomy and the remainining one unilateral nonfunctioning kidney. Six cases presented locally abnormal renal images on whole-bone scanning, three of them suffered renal cell carcinomas and the rest renal solitary cyst. Eighty-eight percent of the abnormal renal images agreed with IVP findings. The renal images of whole-bone scanning faithfully reflected the original renal lesion. Two cases of renal carcinoma and renal solitary cyst recognized on whole-bone scanning are presented, to indicate the usefulness of renal images on whole-bone scanning. (auth.)

  7. 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.

  8. 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.

  9. A case report of renal cell carcinoma in a dog

    Directory of Open Access Journals (Sweden)

    A.-S. Paşca

    2013-10-01

    Full Text Available Mix renal carcinoma was noticed during the necropsic examination of a 14 year old mix breed female. Tumours were bilateral and metastasis was noticed in the spleen and myocard. Histological examination evidenced morphological aspects characteristic to the mixt renal carcinoma. Histological aspects described in this individual characterize renal cell carcinoma, also known as renal adenocarcinoma, hypernephroma or, in older literature, Grawitz tumour.

  10. Staged bilateral carotid endarterectomy

    DEFF Research Database (Denmark)

    Schroeder, T; Sillesen, H; Engell, Hans Christian

    1986-01-01

    In a series of 56 staged bilateral carotid endarterectomies, new neurologic symptoms developed in 5% and 20% following the first and second procedure, respectively. All complications were transient or minor. The incidence of postendarterectomy hypertension was significantly higher following...... the second procedure, when operations were staged less than 3 weeks apart. A correlation between these hypertensive episodes and the occurrence of new neurologic symptoms could not be shown. However, as this correlation has been proved in several other reports, bilateral carotid endarterectomy is advised...... to be staged at least 3 weeks apart. In addition, a conservative attitude towards contralateral asymptomatic lesions is proposed....

  11. Association of Nasal Nostril Stenosis with Bilateral Choanal Atresia: A Case Report

    Directory of Open Access Journals (Sweden)

    Shahin Abdollahifakhim

    2014-01-01

    Full Text Available Introduction: Neonatal nasal airway obstruction induces various degrees of respiratory distress. The management of this disease, including surgical repair, will depend on the severity and location of the obstruction. We describe here a case of congenital nasal nostril stenosis that required surgical repair for stenting of both nares after coanal atresia repair.   Case Report: A 2 days old female newborn referred to neonatal department of Tabriz Children’s Hospital affiliated to the University of Medical Sciences of Tabriz, Iran on the 3rd of December, 2011 immediately after birth with respiratory distress due to bilateral coanal atresia and nasal hypoplasia with very small nostrils. CT scan showed normal brain and bilateral choanal atresia with normal size Pyriform apertures.   Conclusion: Nasal obstruction can lead to airway compromise and respiratory distress. Congenital bony nasal deformities are being recognized as an important cause of newborn airway obstruction. Nasal hypoplasia is seen in many craniofacial syndromes. Although our patient had hypoplastic nostrils with respiratory distress due to bilateral coanal atresia, correction of hypoplastic nostrils was necessary for completing the operation of choanal atresia.

  12. 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.

  13. Renal rescue of dopamine D2 receptor function reverses renal injury and high blood pressure

    Science.gov (United States)

    Konkalmatt, Prasad R.; Asico, Laureano D.; Zhang, Yanrong; Yang, Yu; Drachenberg, Cinthia; Zheng, Xiaoxu; Han, Fei; Jose, Pedro A.; Armando, Ines

    2016-01-01

    Dopamine D2 receptor (DRD2) deficiency increases renal inflammation and blood pressure in mice. We show here that long-term renal-selective silencing of Drd2 using siRNA increases renal expression of proinflammatory and profibrotic factors and blood pressure in mice. To determine the effects of renal-selective rescue of Drd2 expression in mice, the renal expression of DRD2 was first silenced using siRNA and 14 days later rescued by retrograde renal infusion of adeno-associated virus (AAV) vector with DRD2. Renal Drd2 siRNA treatment decreased the renal expression of DRD2 protein by 55%, and DRD2 AAV treatment increased the renal expression of DRD2 protein by 7.5- to 10-fold. Renal-selective DRD2 rescue reduced the expression of proinflammatory factors and kidney injury, preserved renal function, and normalized systolic and diastolic blood pressure. These results demonstrate that the deleterious effects of renal-selective Drd2 silencing on renal function and blood pressure were rescued by renal-selective overexpression of DRD2. Moreover, the deleterious effects of 45-minute bilateral ischemia/reperfusion on renal function and blood pressure in mice were ameliorated by a renal-selective increase in DRD2 expression by the retrograde ureteral infusion of DRD2 AAV immediately after the induction of ischemia/reperfusion injury. Thus, 14 days after ischemia/reperfusion injury, the renal expression of profibrotic factors, serum creatinine, and blood pressure were lower in mice infused with DRD2 AAV than in those infused with control AAV. These results indicate an important role of renal DRD2 in limiting renal injury and preserving normal renal function and blood pressure. PMID:27358912

  14. Renal artery anatomy affects the blood pressure response to renal denervation in patients with resistant hypertension.

    Science.gov (United States)

    Hering, Dagmara; Marusic, Petra; Walton, Antony S; Duval, Jacqueline; Lee, Rebecca; Sata, Yusuke; Krum, Henry; Lambert, Elisabeth; Peter, Karlheinz; Head, Geoff; Lambert, Gavin; Esler, Murray D; Schlaich, Markus P

    2016-01-01

    Renal denervation (RDN) has been shown to reduce blood pressure (BP), muscle sympathetic nerve activity (MSNA) and target organ damage in patients with resistant hypertension (RH) and bilateral single renal arteries. The safety and efficacy of RDN in patients with multiple renal arteries remains unclear. We measured office and 24-hour BP at baseline, 3 and 6 months following RDN in 91 patients with RH, including 65 patients with single renal arteries bilaterally (group 1), 16 patients with dual renal arteries on either one or both sides (group 2) and 10 patients with other anatomical constellations or structural abnormalities (group 3). Thirty nine out of 91 patients completed MSNA at baseline and follow-up. RDN significantly reduced office and daytime SBP in group 1 at both 3 and 6 months follow-up (Pkidney function in any group. While RDN can be performed safely irrespective of the underlying renal anatomy, the presence of single renal arteries with or without structural abnormalities is associated with a more pronounced BP and MSNA lowering effect than the presence of dual renal arteries in patients with RH. However, when patients with dual renal arteries received renal nerve ablation in all arteries there was trend towards a greater BP reduction. Insufficient renal sympathetic nerve ablation may account for these differences. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  15. Bilateral primary lymphoma of kidney. Report of a case

    International Nuclear Information System (INIS)

    León Acosta, Pedro; Perdomo Hernando, Yaslin; Ceballos Nápoles, Yanis Janel; Pila Pérez, Rafael; Pila Peláez, Rafael

    2016-01-01

    Background: We present a case of primary renal lymphoma no bilateral Hodgkin of both kidneys which is uncommon Objective: To present the clinical case of a patient that dies four days of their entrance in which was discovered a bilateral renal mass with the anatomopathological diagnostic of primary renal lymphoma for necropsy stud . Case presentation: It is a 40 year-old patient, with family antecedents of neoplasias and personal of gastritis of seven months of evolution. The patient was entered for fever of 39 ℃ an important abdominal pain, in which was important the anemic and constitutional syndrome with loss of 11 kg in the last months; and diminished diuresis. A tumor was verified between 12 and 15 cm in both flanks of hard and not painful irregular surface. The patient presented unfavorable evolution for what was moved to room of cares intermissions. In the analytic study they were verified as exams of importance the Hb 89 g/L, the speed of globular sedimentation of 95 mm/first hour, the LDH 1495 UI/L, the creatinine of 537 mmol /L and several pathological glycemias one of them of 20 mmol/L. The x- ray results as much the ultrasonography as the contrasted on-line axial tomography guided the possibility to be a renal tumor. The medullogram and the laparoscopic were not useful for the diagnosis. The patient died and she was carried out histopathological necropsy study, which informed a lymphoma not bilateral Hodgkin of kidney of cells B, without an affectation to another organ Conclusions: They are uncommon the cases of primary renal lymphoma, since the renal affectation for a process lymphproliferative is in general secondary to a systemic illness. This case completes the fundamental approaches of Malbrain to consider it as a lymphoma non Hodgkin. The diagnosis is carried out by means of renal biopsy, during the surgery or necropsy for anatomopathological study. (author)

  16. 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.

  17. Special distraction osteogenesis before bone grafting for alveolar cleft defects to correct maxillary deformities in patients with bilateral cleft lips and palates: distraction osteogenesis performed separately for each bone segment.

    Science.gov (United States)

    Mitsukawa, Nobuyuki; Saiga, Atsuomi; Morishita, Tadashi; Satoh, Kaneshige

    2014-07-01

    Patients with bilateral cleft lips and palates have premaxillary protrusion and characteristic jaw deformities involving three-dimensional malposition of the premaxilla and bilateral maxillary bone segments. This study examined patients with bilateral cleft lips and palates who had deviation and hypoplasia of the premaxillas and bilateral maxillary segments. Before bone grafting, the patients were treated with special distraction performed separately for each bone segment using a halo-type external device. This report describes this novel treatment method which produced good results. The subjects were five patients with severe jaw deformities due to bilateral cleft lip and palate. They were treated with maxillary Le Fort I osteotomy and subsequent distraction performed separately for each bone segment using a halo device. In three of five patients, premaxillary osteotomy was not performed, and osteotomy and distraction were performed only for the right and left lateral segments with severe hypoplasia. All patients achieved distraction close to the desired amount. The widths of the alveolar clefts were narrowed, and satisfactory occlusion and maxillary arch form were achieved. After the surgery, three of five patients underwent bone grafting for bilateral alveolar cleft defects and the bone graft survival was satisfactory. This method had many benefits, including narrowing of alveolar clefts, improvement of maxillary hypoplasia, and achievement of a good maxillary arch form. In addition, subsequent bone grafting for alveolar cleft defects was beneficial, dental prostheses were unnecessary, and frequency of surgery and surgical invasiveness were reduced. This method is a good surgical procedure that should be considered for patients with bilateral cleft lips and palates who have premaxillary protrusion and hypoplasia of the right and left lateral segments. Copyright © 2013 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights

  18. Bilateral meandering pulmonary veins

    Energy Technology Data Exchange (ETDEWEB)

    Thupili, Chakradhar R.; Udayasankar, Unni [Pediatric Imaging, Imaging Institute Cleveland Clinic, Cleveland, OH (United States); Renapurkar, Rahul [Imaging Institute Cleveland Clinic, Thoracic Imaging, L10, Cleveland, OH (United States)

    2015-06-15

    Meandering pulmonary veins is a rare clinical entity that can be mistaken for more complex congenital syndromes such as hypogenetic lung syndrome. We report imaging findings in a rare incidentally detected case of bilateral meandering pulmonary veins. We briefly discuss the role of imaging in diagnosing this condition, with particular emphasis on contrast-enhanced CT. (orig.)

  19. Bilateral Periventricular Nodular Heterotopia

    OpenAIRE

    J Gordon Millichap

    1998-01-01

    Two unrelated boys, ages 8 and 5 years, with a syndrome of bilateral periventricular nodular heterotopia (BPNH), regional cortical dysplasia, mild mental retardation, and frontonasal malformation (FNM) are reported from the Instituto di Neuropsychiatria Infantile, University of Pisa, Italy, and the Institute of Human Genetics, University of Minnesota Medical School, Minneapolis, MN.

  20. Homogeneous bilateral block shifts

    Indian Academy of Sciences (India)

    Douglas class were classified in [3]; they are unilateral block shifts of arbitrary block size (i.e. dim H(n) can be anything). However, no examples of irreducible homogeneous bilateral block shifts of block size larger than 1 were known until now.

  1. Synchronous, bilateral tonsillar carcinomas

    DEFF Research Database (Denmark)

    Nami Saber, Camelia; Grønhøj, Christian; Jensen, David Hebbelstrup

    2017-01-01

    INTRODUCTION: The incidence of oropharyngeal squamous cell carcinoma (OPSCC) is increasing, but data on the incidence of synchronous, bilateral tonsillar squamous cell carcinomas (BiTSCCs) is sparse. In this study, we report the incidence and tumour characteristics of BiTSCCs in a population-base...

  2. Bilateral breast carcinoma

    International Nuclear Information System (INIS)

    Kim, Eung Jo; Oh, Ki Keun

    1990-01-01

    We evaluate 311 breast cancer patients admitted to Yong Dong Severance Hospital Between October 1st 1985 and July 31th 1989, and were able to obtain the following conclusions. 1) There were 14(4.5%) bilateral breast cancers among the 311 confirmed breast cancers. 2) Among the bilateral breast cancers, 5(31%) were synchronous and 9(69%) metachronous. 3) Average interval between the first and the second breast cancer in metachronous cancers was 3.8 year(1-15 years). 4) Bilateral breast cancer was most prevalent in the fifth decade (6/14) with the mean age of 47 years. 5) Film mammogram and sonomammogram showed findings of typical breast malignancies. There was no additional specific findings for each cancer in bilateral breast cancers which was different from unilateral cancers. Therefor, in the patients with unilateral breast cancer, possibility of the second lesion in the contralateral side must be considered and a close follow up observation should be done for at least 3 years

  3. Pediatric renal leukemia: spectrum of CT imaging findings

    International Nuclear Information System (INIS)

    Hilmes, Melissa A.; Dillman, Jonathan R.; Mody, Rajen J.; Strouse, Peter J.

    2008-01-01

    The kidneys are a site of extramedullary leukemic disease that can be readily detected by CT. To demonstrate the spectrum of CT findings in children with renal leukemic involvement. Twelve children were identified retrospectively as having renal leukemic involvement by contrast-enhanced CT of the abdomen. Contrast-enhanced CT images through the kidneys of each patient were reviewed by two pediatric radiologists. Pertinent imaging findings and renal lengths were documented. The electronic medical record was accessed to obtain relevant clinical and pathologic information. Five patients with renal leukemic involvement presented with multiple bilateral low-attenuation masses, while three patients demonstrated large areas of wedge-shaped and geographic low attenuation. Four other patients presented with unique imaging findings, including a solitary unilateral low-attenuation mass, solitary bilateral low-attenuation masses, multiple bilateral low-attenuation masses including unilateral large conglomerate masses, and bilateral areas of ill-defined parenchymal low attenuation. Two patients showed unilateral nephromegaly, while eight other patients showed bilateral nephromegaly. Two patients had normal size kidneys. Two patients had elevated serum creatinine concentrations at the time of imaging. Renal leukemic involvement in children can present with a variety of CT imaging findings. Focal renal abnormalities as well as nephromegaly are frequently observed. Most commonly, renal leukemic involvement does not appear to impair renal function. (orig.)

  4. Pediatric renal leukemia: spectrum of CT imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Hilmes, Melissa A. [University of Michigan Health System, C.S. Mott Children' s Hospital, Section of Pediatric Radiology, Ann Arbor, MI (United States); Vanderbilt University Children' s Hospital, Section of Pediatric Radiology, Nashville, TN (United States); Dillman, Jonathan R. [University of Michigan Health System, C.S. Mott Children' s Hospital, Section of Pediatric Radiology, Ann Arbor, MI (United States); University of Michigan Health System, Department of Radiology, Ann Arbor, MI (United States); Mody, Rajen J. [University of Michigan Health System, C.S. Mott Children' s Hospital, Division of Pediatric Hematology-Oncology and Bone Marrow Transplantation, Ann Arbor, MI (United States); Strouse, Peter J. [University of Michigan Health System, C.S. Mott Children' s Hospital, Section of Pediatric Radiology, Ann Arbor, MI (United States)

    2008-04-15

    The kidneys are a site of extramedullary leukemic disease that can be readily detected by CT. To demonstrate the spectrum of CT findings in children with renal leukemic involvement. Twelve children were identified retrospectively as having renal leukemic involvement by contrast-enhanced CT of the abdomen. Contrast-enhanced CT images through the kidneys of each patient were reviewed by two pediatric radiologists. Pertinent imaging findings and renal lengths were documented. The electronic medical record was accessed to obtain relevant clinical and pathologic information. Five patients with renal leukemic involvement presented with multiple bilateral low-attenuation masses, while three patients demonstrated large areas of wedge-shaped and geographic low attenuation. Four other patients presented with unique imaging findings, including a solitary unilateral low-attenuation mass, solitary bilateral low-attenuation masses, multiple bilateral low-attenuation masses including unilateral large conglomerate masses, and bilateral areas of ill-defined parenchymal low attenuation. Two patients showed unilateral nephromegaly, while eight other patients showed bilateral nephromegaly. Two patients had normal size kidneys. Two patients had elevated serum creatinine concentrations at the time of imaging. Renal leukemic involvement in children can present with a variety of CT imaging findings. Focal renal abnormalities as well as nephromegaly are frequently observed. Most commonly, renal leukemic involvement does not appear to impair renal function. (orig.)

  5. 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

  6. 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.

  7. 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.

  8. 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.

  9. Renal Osteodystrophy

    Directory of Open Access Journals (Sweden)

    Aynur Metin Terzibaşoğlu

    2004-12-01

    Full Text Available Chronic renal insufficiency is a functional definition which is characterized by irreversible and progressive decreasing in renal functions. This impairment is in collaboration with glomeruler filtration rate and serum creatinine levels. Besides this, different grades of bone metabolism disorders develop in chronic renal insufficiency. Pathologic changes in bone tissue due to loss of renal paranchyme is interrelated with calcium, phosphorus vitamine-D and parathyroid hormone. Clinically we can see high turnover bone disease, low turnover bone disease, osteomalacia, osteosclerosis and osteoporosis in renal osteodystropy. In this article we aimed to review pathology of bone metabolism disorders due to chronic renal insufficiency, clinic aspects and treatment approaches briefly.

  10. 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.

  11. 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

  12. Dynamic renal scintigraphy in aortic disorders

    International Nuclear Information System (INIS)

    Terae, Satoshi; Itoh, Kazuo; Tsukamoto, Eriko; Nakada, Kunihiro; Fujimori, Kenji; Hashimoto, Masato; Tanabe, Tatsuzo; Furudate, Masayori; Irie, Goro

    1986-01-01

    Dynamic renal scintigraphy has been reviewed for evaluation of renal arterial involvement in aortic disorders such as arteriosclerosis obliterans, abdominal aortic aneurysm and dissecting aneurysm. As a diagnostic finding and parameters, we used blood perfusion images of both kidneys and relative split renal function index obtained with analysis of the time-activity curves which were generated using a renal region of interest. In the diagnosis of unilateral renal arterial involvement, sensitivity and specificity of blood perfusion images were 100 % (9/9) and 77 % (10/13) and those of relative split renal function index were 78 % (7/9) and 92 % (12/13), respectively. Dynamic renal scintigraphy was useful for evaluating unilateral renal arterial involvement in aortic diseases. However, scintigraphic diagnosis of bilateral renal arterial involvement were difficult. And in a severe case, we could not differentiate renal parenchymal damage due to renovascular involvement from senile renal dysfunction or hypertensive renal disease which is often a cause of aortic disorders. (author)

  13. Successful catheter directed thrombolysis of IVC and renal vein occlusive thrombus.

    LENUS (Irish Health Repository)

    McCarthy, E

    2011-11-01

    Thrombus formation is a recognised complication of IVC filter placement, however IVC and bilateral renal vein occlusion secondary to thrombus is much less common. We present a case of infrahepatic caval and bilateral renal vein occlusion secondary to thrombosis of a suprarenal IVC filter. With progressive clinical deterioration and failure of conservative medical management the patient underwent successful mechanical disruption and catheter directed thrombolysis.

  14. Impact of evolving strategy on clinical outcomes and central pulmonary artery growth in patients with bilateral superior vena cava undergoing a bilateral bidirectional cavopulmonary shunt.

    Science.gov (United States)

    Honjo, Osami; Tran, Kim-Chi D; Hua, Zhongdong; Sapra, Priya; Alghamdi, Abdullah A; Russell, Jennifer L; Caldarone, Christopher A; Van Arsdell, Glen S

    2010-09-01

    We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes. Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990-1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999-2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined. The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality. 2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  15. 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.)

  16. 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

  17. Increased release of norepinephrine and dopamine from canine kidney during bilateral carotid occlusion

    International Nuclear Information System (INIS)

    Bradley, T.; Hjemdahl, P.; DiBona, G.F.

    1987-01-01

    The renal overflow of norepinephrine (NE) and dopamine (DA) to plasma from the innervated kidney was studied at rest and during sympathetic nervous system activation by bilateral carotid artery occlusion (BCO) in vagotomized dogs under barbiturate or barbiturate/nitrous oxide anesthesia. BCO elevated arterial pressure and the arterial plasma concentration of NE, DA, and epinephrine (Epi). Renal vascular resistance (renal arterial pressure kept constant) increased by 15 +/- 7% and the net renal venous outflows (renal veno-arterial concentration difference x renal plasma flow) of NE and DA were enhanced. To obtain more correct estimates of the renal contribution to the renal venous catecholamine outflow, they corrected for the renal extraction of arterial catecholamines, assessed as the extractions of [ 3 H]NE, [ 3 H]DA, or endogenous Epi. The [ 3 H]NE corrected renal NE overflow to plasma increased from 144 +/- 40 to 243 +/- 64 pmol-min -1 during BCO, which, when compared with a previous study of the [ 3 H]NE corrected renal NE overflow to plasma evoked by electrical renal nerve stimulation, corresponds to a 40% increase in nerve impulse frequency from ∼ 0.6 Hz. If the renal catecholamine extraction was not taken into account the effect of BCO was underestimated. The renal DA overflow to plasma was about one-fifth of the NE overflow both at rest and during BCO, indicating that there was no preferential activation of noradrenergic or putative dopaminergic nerves by BCO

  18. Imaging of renal metastases

    International Nuclear Information System (INIS)

    Bruneton, J.N.; Normand, F.; Balu-Maestro, C.; Rogopoulos, A.; Drouillard, J.; Laurent, F.

    1988-01-01

    Metastases are the most frequent malignant tumors of the kidney, but these lesions are of late onset in neoplastic disease. The 19 cases reported here were all investigated with various imaging techniques (CT 12 cases, ultrasonography 12 cases, urography 8 cases, angiography 2 cases, MRI 1 case). The most common primary malignancies were lung cancer, melanoma and cancer of the controlateral kidney. In this series, 8 of the lesions were solitary, and 9 were unilateral. Tumor vascularity was evaluated in 15 cases: 14 of these lesions were hypovascular. The differential diagnosis includes small cysts, lymphoma, bilateral renal cancer, multiple small abscesses and multiple small infarcts [fr

  19. Renal venogram

    Science.gov (United States)

    ... be black. Other structures will be shades of gray. Veins are not normally seen in an x- ... Venogram - kidney; Renal vein thrombosis - venogram Images Kidney anatomy Kidney - blood and urine flow Renal veins References ...

  20. Bilateral chronic subdural hematoma

    DEFF Research Database (Denmark)

    Andersen-Ranberg, Nina Christine; Rom Poulsen, Frantz; Bergholt, Bo

    2017-01-01

    OBJECTIVE Bilateral chronic subdural hematoma (bCSDH) is a common neurosurgical condition frequently associated with the need for retreatment. The reason for the high rate of retreatment has not been thoroughly investigated. Thus, the authors focused on determining which independent predictors...... are associated with the retreatment of bCSDH with a focus on surgical laterality. METHODS In a national database of CSDHs (Danish Chronic Subdural Hematoma Study) the authors retrospectively identified all bCSDHs treated in the 4 Danish neurosurgical departments over the 3-year period from 2010 to 2012...... that a separated hematoma density and the absence of postoperative drainage were independent predictors of retreatment. CONCLUSIONS In bCSDHs bilateral surgical intervention significantly lowers the risk of retreatment compared with unilateral intervention and should be considered when choosing a surgical...

  1. Bilateral Wilms' tumor

    International Nuclear Information System (INIS)

    Malcolm, A.W.; Jaffe, N.; Folkman, M.J.; Cassady, J.R.

    1980-01-01

    Twenty children with bilateral Wilms' tumor were presented to the Children's Hospital Medical Center and Children's Cancer Research Foundation, Sidney Farber Cancer Institute, and Joint Center for Radiation Therapy (CHMC-CCRF, SFCI, JCRT) from January 1, 1956 to December 31, 1976. Of these 20, 16 had simultaneous and 4 had metachronous disease on presentation. All patients were treated with surgery, radiation and chemotherapy. Of the 16 patients with simultaneous disease, 10 (63%) are alive and free of disease 12+ to 175+ months post diagnosis and treatment, with median follow-up of 121 months. There were no long-term survivors in the metachronous group; all were dead of disease within 21 months from initial presentation of original tumor. With these data we relate prognosis to extent of disease and discuss a general approach to the management of bilateral Wilms' tumor

  2. Multilateral and bilateral agreements

    International Nuclear Information System (INIS)

    Koponen, H.

    1993-01-01

    Finland has made both multilateral and bilateral agreements on the exchange of information related to radiation safety. The first arrangements for international agreements and exchange of information were made after the Chernobyl accident. In 1987, Finland joined the convention on early notification of a nuclear power accident coordinated by International Atomic Energy Agency. The convention is applied to accidents that cause of may cause emissions of radioactive substances that might affect the radiation safety of another country. Besides the convention on early notification, some other individual agreements have also been made. These include the International Nuclear Event Scale (INES) system and power companies own information exchange systems. Finland has conducted bilateral agreements with the Nordic countries and the Soviet Union on the notification of accidents and exchange of nuclear power plant information. Today, Russia answers for the Soviet Union's contractual obligations. (orig.)

  3. Acute renal failure in rats

    International Nuclear Information System (INIS)

    Cederholm, C.; Almen, T.; Bergquist, D.; Golman, K.; Takolander, R.; Malmoe Allmaenna Sjukhus

    1989-01-01

    It was demonstrated in rats that renal injury which follows transient renal hypoxia is potentiated by the contrast media metrizoate, ioxaglate, iopamidol and iohexol. Intravenous injection of 1 g I/kg of all four media alone to 82 rats caused no significant increase in serum urea 1, 3 and 7 days later. The percentage increase of serum urea is given in median values and interquartile range (in parentheses). Bilateral renal arterial occlusion alone for 40 minutes in 42 rats increased serum urea one day later by 40% (20-130). Intravenous injection of the media followed in one hour by bilateral renal arterial occlusion for 40 minutes in 104 rats caused serum urea to increase one day later by 130% (70-350) after metrizoate, by 220% (50-380) after ioxaglate, by 290 % (60-420) after iopamidol and by 160% (50-330) after iohexol. There were no significant differences between the potentiating effects of the various media on ischemic renal failure. (orig.)

  4. File: International bilateral relations

    International Nuclear Information System (INIS)

    Feltin, Ch.; Rabouhams, J.; Bravo, X.; Rousseau, M.; Le Breton, S.; Saint Raymond, Ph.; Brigaud, O.; Pertuis, V.; McNair, J.; Sayers, M.R.; Bye, R.; Scherrer, J.

    1998-01-01

    Since its creation in 1973, the Authority of Safety was assigned missions in the international field with following objectives: to develop information exchanges with its foreign counterpart, to make know and to explain the French approach and practice; to give to concerned countries the useful information on french nuclear facilities situated near the border; This file shows with some examples, how bilateral relations allow to fill up these objectives and how the French Authority got the foreign experience. (N.C.)

  5. Idiopathic bilateral male breast abscess.

    Science.gov (United States)

    Sinha, Rajan Kumar; Sinha, Mithilesh Kumar; Gaurav, Kumar; Kumar, Amar

    2014-03-10

    A 38-year-old man presented with bilateral breast swelling, along with pain and redness for 7 days. Bilateral axillary nodes were also palpable; which were multiple and discrete. A provisional diagnosis of bilateral breast abscess was made with suspicion of underlying malignancy. Incision and drainage through subareolar incision was performed and the adjoining tissue was excised and sent for histopathological examination.

  6. Late unıon of the renal veıns wıth the ınterposed left renal artery

    OpenAIRE

    Çavdar, Safiye; Şehirli, Ümit; Malcic-Gürbüz, Jasna; Gümüşçü, Burak; Akalın, Aytül

    2003-01-01

    The complicated embryological development of the renal vein shows extensive variability in its anatomy. In this study, the rare bilateral late union of the renal veins with the interposed renal artery was observed during dissection for educational purposes. Its significance in different clinical conditions has been discussed.

  7. 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

  8. 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.

  9. 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.

  10. 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

  11. 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.)

  12. 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...

  13. 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

  14. 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.

  15. 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...

  16. 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

  17. Renal perfusion scintiscan

    Science.gov (United States)

    ... Radionuclide renal perfusion scan; Perfusion scintiscan - renal; Scintiscan - renal perfusion Images Kidney anatomy Kidney - blood and urine flow Intravenous pyelogram References Rottenberg G, Andi AC. Renal ...

  18. A novel heterozygous SOX2 mutation causing congenital bilateral anophthalmia, hypogonadotropic hypogonadism and growth hormone deficiency.

    Science.gov (United States)

    Macchiaroli, Annamaria; Kelberman, Daniel; Auriemma, Renata Simona; Drury, Suzanne; Islam, Lily; Giangiobbe, Sara; Ironi, Gabriele; Lench, Nicholas; Sowden, Jane C; Colao, Annamaria; Pivonello, Rosario; Cavallo, Luciano; Gasperi, Maurizio; Faienza, Maria Felicia

    2014-01-25

    Heterozygous de novo mutations in SOX2 have been reported in approximately 10-20% of patients with unilateral or bilateral anophthalmia or microphthalmia. An additional phenotype of hypopituitarism, with anterior pituitary hypoplasia and hypogonadotropic hypogonadism, has been reported in patients carrying SOX2 alterations. We report a novel heterozygous mutation in the SOX2 gene in a male affected with congenital bilateral anophthalmia, hypogonadotrophic hypogonadism and growth hormone deficiency. The mutation we describe is a cytosine deletion in position 905 (c905delC) which causes frameshift and an aberrant C-terminal domain. Our report highlights the fact that subjects affected with eye anomalies and harboring SOX2 mutations are at high risk for gonadotropin deficiency, which has important implications for their clinical management. Copyright © 2013 Elsevier B.V. All rights reserved.

  19. Recurrent Bilateral Focal Myositis.

    Science.gov (United States)

    Nagafuchi, Hiroko; Nakano, Hiromasa; Ooka, Seido; Takakuwa, Yukiko; Yamada, Hidehiro; Tadokoro, Mamoru; Shimojo, Sadatomo; Ozaki, Shoichi

    This report describes a rare case of recurrent bilateral focal myositis and its successful treatment via methotrexate. A 38-year-old man presented myalgia of the right gastrocnemius in May 2005. Magnetic resonance imaging showed very high signal intensity in the right gastrocnemius on short-tau inversion recovery images. A muscle biopsy revealed inflammatory CD4+ cell-dominant myogenic change. Focal myositis was diagnosed. The first steroid treatment was effective. Tapering of prednisolone, however, repeatedly induced myositis relapse, which progressed to multiple muscle lesions of both lower limbs. Initiation of methotrexate finally allowed successful tapering of prednisolone, with no relapse in the past 4 years.

  20. A Case of Polyarteritis Nodosa with Bilateral Ureteral Obstruction

    Science.gov (United States)

    Yoo, Bin; Kim, Hong Kyu; Choi, Seung Won; Moon, Hee Bom

    1996-01-01

    We report a case of bilateral ureteral obstruction most likely caused by polyarteritis nodosa. The diagnosis was based upon muscle biopsy which showed typical necrotizing vasculitis in medium size artery in conjunction with microaneurysms in renal angiography. Ureteral obstruction is a rare manifestation of polyarteritis nodosa. This condition is thought to result from vasculitis of periureteral vessels. The patient was managed with prednisolone alone, which resulted in complete resolution of ureteral obstruction on both sides. PMID:8854655

  1. Bilateral acoustic neuromas.

    Science.gov (United States)

    Anand, V T; Byrnes, D P; Walby, A P; Kerr, A G

    1993-10-01

    This article reviews 12 patients with bilateral acoustic neuromas. The sex incidence was equal and the mean age at diagnosis was 26.2 years. The family history was positive in nine of the patients. Five patients have had incomplete surgical removal of acoustic neuromas on both sides. Two of them are completely deaf and the other three have severe sensorineural hearing loss in one ear and no hearing in the other ear. In five patients the tumour on one side has been operated on and the other side is being observed with at least short-term preservation of good hearing. The remaining two patients died of intra-cranial complications, one of them post-operatively. Four patients developed facial palsy immediately following surgery and one developed facial weakness 6 months after surgery. Guidelines are discussed for the care of these patients including the timing of surgery and alternative treatment options (observation, radio-surgery and chemotherapy). This is essentially a group of young individuals who have had multiple operations for bilateral acoustic tumours and associated manifestations and for whom the disease and the sequelae of treatment can be tragic.

  2. RENAL CRYOABLATION

    Directory of Open Access Journals (Sweden)

    A. V. Govorov

    2012-01-01

    Full Text Available Renal cryoablation is an alternative minimally-invasive method of treatment for localized renal cell carcinoma. The main advantages of this methodology include visualization of the tumor and the forming of "ice ball" in real time, fewer complications compared with other methods of treatment of renal cell carcinoma, as well as the possibility of conducting cryotherapy in patients with concomitant pathology. Compared with other ablative technologies cryoablation has a low rate of repeat sessions and good intermediate oncological results. The studies of long-term oncological and functional results of renal cryoablation are presently under way.

  3. [Obstructive renal insufficiency caused by amoxicillin crystalluria].

    Science.gov (United States)

    Boursas, M; Benhassine, L; Kempf, J; Petit, B; Vuillemin, F

    1997-01-01

    A 76-year-old woman was admitted to the ICU for a meningitis with rhombencephalitis due to Listeria monocytogenes. The treatment included amoxicillin (250 mg.kg-1.day-1) and gentamicin (3 mg.kg-1.day-1 over 6 days). Neurological outcome was favourable. However at the 14th day, an acute renal failure occurred, following macroscopic haematuria and milkiness urine. CT scan and sonography confirmed the diagnosis of obstructive renal failure with bilateral ureteral obstruction. Crystalluria caused by amoxicillin was suspected. Endoscopic ureteral insertion of double-J catheters permitted the recovery of a normal renal function.

  4. Neglected simultaneous bilateral femoral neck fractures secondary to narcotic drug abuse treated by bilateral one-staged hemiarthroplasty: a case report

    Directory of Open Access Journals (Sweden)

    Vahedi Ehsan

    2010-06-01

    Full Text Available Abstract Simultaneous bilateral femoral neck fractures are extremely rare and associated with various conditions. Up to now Most cases had correlations with major trauma, repetitive minor trauma, seizure, parathyroid or renal dysfunction, anti-epileptic medications, seizure, etc. A 28-year-old addict man referred to us with a 10-year history of narcotic drug abuse and history of 8 months bilateral groin pain. He admitted with displaced bilateral femoral neck fracture. Because of long duration of this condition and osteonecrosis revealed on bone scan, one-staged bilateral hip hemiarthroplasty was done. A good function was noted after surgery to 4-month follow up. Up to now, have not be founded in the literature that a case of bilateral femoral neck fracture associated with narcotic drug abuse. Because of negative effects of opium or smoking on bone tissues, a simple bone pain should aware us about the risk of stress or fatigue fracture.

  5. Radiologic observation of renal tuberculosis

    International Nuclear Information System (INIS)

    Kim, S. W.; Ra, Y. W.; Kim, Y. J.

    1981-01-01

    Radiographic findings of thirty eight cases of renal tuberculosis treated at this hospital during last 4 years were analysed with following results. The cases examined were 24 male and 14 female patients. Age distribution was broad and evenly distributed ranging from 2nd decades to 5th decades. Main symptoms complained were urinary frequency, hematuria, dysuria and flank pain. Findings of physical examination revealed tenderness of costovertebral angle, palpable mass on flank area and epididymal indutration. The simple chest films showed pulmonary tuberculosis in 22 cases including 6 cases of active military type. Thirty one cases showed increased ESR, 8 cases showed AFB positive in urine and 12 cases showed bilateral renal tuberculosis. Through urographic findings nonvisualization, cyceopelviectasis, motheaten appearance of minor calyx, contracted bladder, delayed visualization, ureteral stricture and beading were observed in order of frequency. Five cases with miliary tuberculosis showed advanced renal lesion on urogram

  6. The Effects of Renal Denervation on Renal Hemodynamics and Renal Vasculature in a Porcine Model.

    Directory of Open Access Journals (Sweden)

    Willemien L Verloop

    Full Text Available Recently, the efficacy of renal denervation (RDN has been debated. It is discussed whether RDN is able to adequately target the renal nerves.We aimed to investigate how effective RDN was by means of functional hemodynamic measurements and nerve damage on histology.We performed hemodynamic measurements in both renal arteries of healthy pigs using a Doppler flow and pressure wire. Subsequently unilateral denervation was performed, followed by repeated bilateral hemodynamic measurements. Pigs were terminated directly after RDN or were followed for 3 weeks or 3 months after the procedure. After termination, both treated and control arteries were prepared for histology to evaluate vascular damage and nerve damage. Directly after RDN, resting renal blood flow tended to increase by 29±67% (P = 0.01. In contrast, renal resistance reserve increased from 1.74 (1.28 to 1.88 (1.17 (P = 0.02 during follow-up. Vascular histopathology showed that most nerves around the treated arteries were located outside the lesion areas (8±7 out of 55±25 (14% nerves per pig were observed within a lesion area. Subsequently, a correlation was noted between a more impaired adventitia and a reduction in renal resistance reserve (β: -0.33; P = 0.05 at three weeks of follow-up.Only a small minority of renal nerves was targeted after RDN. Furthermore, more severe adventitial damage was related to a reduction in renal resistance in the treated arteries at follow-up. These hemodynamic and histological observations may indicate that RDN did not sufficiently target the renal nerves. Potentially, this may explain the significant spread in the response after RDN.

  7. The Effects of Renal Denervation on Renal Hemodynamics and Renal Vasculature in a Porcine Model

    Science.gov (United States)

    Verloop, Willemien L.; Hubens, Lisette E. G.; Spiering, Wilko; Doevendans, Pieter A.; Goldschmeding, Roel; Bleys, Ronald L. A. W.; Voskuil, Michiel

    2015-01-01

    Rationale Recently, the efficacy of renal denervation (RDN) has been debated. It is discussed whether RDN is able to adequately target the renal nerves. Objective We aimed to investigate how effective RDN was by means of functional hemodynamic measurements and nerve damage on histology. Methods and Results We performed hemodynamic measurements in both renal arteries of healthy pigs using a Doppler flow and pressure wire. Subsequently unilateral denervation was performed, followed by repeated bilateral hemodynamic measurements. Pigs were terminated directly after RDN or were followed for 3 weeks or 3 months after the procedure. After termination, both treated and control arteries were prepared for histology to evaluate vascular damage and nerve damage. Directly after RDN, resting renal blood flow tended to increase by 29±67% (P = 0.01). In contrast, renal resistance reserve increased from 1.74 (1.28) to 1.88 (1.17) (P = 0.02) during follow-up. Vascular histopathology showed that most nerves around the treated arteries were located outside the lesion areas (8±7 out of 55±25 (14%) nerves per pig were observed within a lesion area). Subsequently, a correlation was noted between a more impaired adventitia and a reduction in renal resistance reserve (β: -0.33; P = 0.05) at three weeks of follow-up. Conclusion Only a small minority of renal nerves was targeted after RDN. Furthermore, more severe adventitial damage was related to a reduction in renal resistance in the treated arteries at follow-up. These hemodynamic and histological observations may indicate that RDN did not sufficiently target the renal nerves. Potentially, this may explain the significant spread in the response after RDN. PMID:26587981

  8. Coexisting first and bilateral second branchial fistulas in a child with nonfamilial branchio-otic syndrome.

    Science.gov (United States)

    Lapeña, Jose F; Jimena, Genilou Liv M

    2013-07-01

    We describe what we believe is only the third reported case of coexisting first and bilateral second branchial fistulas associated with nonfamilial branchio-otic syndrome. The patient was a 6-year-old girl who presented with bilaterally draining anterior neck puncta, a preauricular sinus, and moderately severe bilateral hearing loss. She had no family history of branchial anomalies. Compared with branchial cysts and sinuses, branchial fistulas are rare. Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies, as only 10 cases have been previously reported in the English-language literature. Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 2 had nonfamilial branchio-otic syndrome, and 1 had nonfamilial branchio-oto-renal syndrome.

  9. Ultrasonography of hydronephrosis and renal masses

    International Nuclear Information System (INIS)

    Lee, Kyung Weon; Kim, Chong Gun; Kim, Yeon Jin; Rhee, Byung Chull

    1984-01-01

    We have analyzed ultrasonographic findings of 55 cases of hydronephrosis and 34 cases of renal masses. The results are as follows: 1. 55 cases of hydronephrosis revealed renal enlargement in 55 cases, separation of central echo complex in 27 cases, multiple anechoic areas radiating from the center in 25 cases and dilated renal pelvis in 24 cases. 2. Among the masses in 34 cases, simple renal cyst were 15 cases, polycystic kidney in 8 cases, hypernephroma in 8 cases, Wilm's tumor in 2 cases and agiomyolipoma in 1 case. 3. Simple renal cyst revealed single in 14 cases (93%) and well defined anechoic mass with posterior enhancement in all cases. 4. Polycystic kidney revealed bilateral irregular shaped renal enlargement and multiple anechoic cysts throughout the kidney. 2 cases (25%) involved liver. 5. 6 cases (75%) of hypernephroma revealed ill defined moderately echogenic mass without posterior enhancement. 6. All cases of Wilm's tumor revealed well defined large mixed echogenic mass in right kidney. 7. Angiomyolipoma revealed bilateral dense echogenic mass with large hemorrhage cyst in right kidney. 8. The ultrasonography is useful noninvasive diagnostic modality of evaluation of renal masses and hydronephrosis.

  10. Bilateral tubercular mastitis

    Directory of Open Access Journals (Sweden)

    Kant Surya

    2007-01-01

    Full Text Available Breast tuberculosis is a rare form of tuberculosis. Moreover the disease is often overlooked and misdiagnosed as carcinoma or pyogenic abscess. Reports on breast tuberculosis from India have been few; reported incidence of breast tuberculosis amongst the total number of mammary conditions varies between 0.64 and 3.59 per cent. Bilateral involvement is still more uncommon (3%. Most accepted view for spread of infection is centripetal lymphatic spread as axillary node involvement was shown to occur in 50 to 75 per cent of cases of tubercular mastitis. Here we re-port a case of a young female who presented with draining sinuses in the breast and no axillary lymphadenopathy. Fine needle aspiration cytology (FNAC of breast lump showed evidence of granulomatous mastitis. She was given therapeutic trial of four drug antitubercular treatment. Both the lump disappeared and sinus healed after six months of antitubercular treatment. Thus a retrospective diagnosis of tu-bercular mastitis was made.

  11. Idiopathic Bilateral Bloody Tearing

    Directory of Open Access Journals (Sweden)

    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  12. Bilateral Antepartum Mastitis

    Directory of Open Access Journals (Sweden)

    Peyman Alibeigi

    2010-12-01

    Full Text Available Antepartum mastitis is a rare condition, whereas postpartum orlactation mastitis is a common problem. This report introducesa case of complicated bilateral antepartum mastitis, which wastreated successfully by drain insertion and antibiotic therapy.The patient was a 23-year-old woman in the 23rd week of herfirst pregnancy. Her chief complaint was progressive swelling,redness and radicular pain in both breasts, which had beenstarted gradually from the 18th week of pregnancy. The patientwas admitted to hospital, and received oral and intravenous antibioticsempirically, which was not effective. The patient wastreated by drainage and oral antibiotic therapy. Based on theapproaches employed and the outcomes achieved it is suggestedthat early surgical insertion in the presence of fluid collection inantepartum mastitis will shorten hospitalization and course ofintravenous antibiotic therapy.Iran J Med Sci 2010; 35(4: 327-330.

  13. Extragenitourinary retroperitoneal primary hydatid cyst: a rare cause of bilateral lower ureteric obstruction and unilateral limb edema

    Directory of Open Access Journals (Sweden)

    Amit Goel

    2013-01-01

    Full Text Available Hydatid cyst is an endemic disease in our country. Most commonly, it occurs in the liver and lungs. Bilateral hydroureteronephrosis is one of the rare presentations of hydatid disease. Herein, we are reporting an unusual case of hydatid disease where the primary mode of presentation was external iliac vein compression with chronic renal failure because of bilateral ureteric involvement. The patient was treated with bilateral double-J stenting to improve the renal function and operated later for removal of hydatid cyst under albendazole drug treatment.

  14. Bilateral nephrocalcinosis and amelogenesis imperfecta: A case report

    Directory of Open Access Journals (Sweden)

    Alok Patel

    2015-01-01

    Full Text Available Amelogenesis imperfecta (AI is a group of hereditary disorders that affect the quality and/or quantity of dental enamel. This paper describes the clinicopathological features of a patient who was born of nonconsanguineous parents and who presented with oral alterations, including yellow and misshapen teeth, intrapulpal calcifications, delayed tooth eruption, and gum enlargement. Scanning electron microscopy of the teeth revealed hypoplastic enamel, and a renal ultrasound detected bilateral nephrocalcinosis, leading to a diagnosis of AI and nephrocalcinosis syndrome. Since nephrocalcinosis is often asymptomatic and can be associated with impaired renal function, dentists who see children with a generalized and thin hypoplastic AI should consider a renal ultrasound scan and referral to a Nephrologist. Children with nephrocalcinosis should also be considered for a dental check.

  15. Renal cancer.

    NARCIS (Netherlands)

    Corgna, E.; Betti, M.; Gatta, G.; Roila, F.; Mulder, P.H.M. de

    2007-01-01

    In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers). Tobacco, obesity and a diet poor in vegetables are all

  16. Renal cancer

    NARCIS (Netherlands)

    Corgna, Enrichetta; Betti, Maura; Gatta, Gemma; Roila, Fausto; De Mulder, Pieter H. M.

    2007-01-01

    In Europe, renal cancer (that is neoplasia of the kidney, renal pelvis or ureter (ICD-9 189 and ICD-10 C64-C66)) ranks as the seventh most common malignancy in men amongst whom there are 29,600 new cases each year (3.5% of all cancers). Tobacco, obesity and a diet poor in vegetables are all

  17. Severe bilateral adrenal hemorrhages in a newborn complicated by persistent adrenal insufficiency

    OpenAIRE

    Zessis, Nicholas R; Nicholas, Jennifer L; Stone, Stephen I

    2018-01-01

    Summary Bilateral adrenal hemorrhages rarely occur during the neonatal period and are often associated with traumatic vaginal deliveries. However, the adrenal gland has highly regenerative capabilities and adrenal insufficiency typically resolves over time. We evaluated a newborn female after experiencing fetal macrosomia and a traumatic vaginal delivery. She developed acidosis and acute renal injury. Large adrenal hemorrhages were noted bilaterally on ultrasound, and she was diagnosed with a...

  18. 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

  19. 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.

  20. 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

  1. 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.

  2. 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...

  3. 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.

  4. 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.

  5. 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.

  6. 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

  7. 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.

  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. 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

  10. 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).

  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. 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.

  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. A boy with bilateral SUNA

    DEFF Research Database (Denmark)

    Vuković Cvetković, Vlasta; Jensen, Rigmor Højland

    2017-01-01

    secondary causes should be searched for vigorously if there are bilateral symptoms. Despite a number of therapeutic trials, effective management for the majority of SUNA patients is not available at present. Management of SUNA is often difficult. Case We report the case of a young boy with bilateral SUNA...

  19. Silicosis with bilateral spontaneous pneumothorax

    Directory of Open Access Journals (Sweden)

    Fotedar Sanjay

    2010-01-01

    Full Text Available Presentation with simultaneous bilateral pneumothorax is uncommon and usually in the context of secondary spontaneous pneumothorax.The association of pneumothorax and silicosis is infrequent and most cases are unilateral. Bilateral pneumothorax in silicosis is very rare with just a few reports in medical literature.

  20. Bilateral persistent hyperplastic primary vitreous

    Directory of Open Access Journals (Sweden)

    Jain Tarun

    2009-01-01

    Full Text Available A case of bilateral persistent hyperplastic primary vitreous (PHPV in a 3-month-old male infant, who had bilateral leukokoria, is presented. The child was referred for imaging with a clinical suspicion of retinoblastoma. Gray-scale ultrasound evaluation revealed an echogenic band in the posterior segment of both globes, extending from the posterior surface of the lens capsule to the optic disc. Doppler examination revealed the presence of arterial flow in the band in both globes. Associated echogenic hemorrhage was also seen, which was confirmed by computed tomography. Most cases of PHPV are sporadic and unilateral, and bilateral PHPV is rare. The imaging features in this case suggest the diagnosis of bilateral PHPV and differentiate it from retinoblastoma. This entity, although infrequent, should be considered in the differential diagnosis while evaluating bilateral leukokoria.

  1. Bilateral disease and new trends in Wilms tumour

    Energy Technology Data Exchange (ETDEWEB)

    Owens, Catherine M.; Olsen, Oeystein E. [Great Ormond Street Hospital for Children NHS Trust, Department of Radiology, London (United Kingdom); Brisse, Herve J. [Institut Curie, Service de Radiodiagnostic, Paris (France); Begent, Joanna [University College Hospital, Paediatric Oncology, London (United Kingdom); Smets, Anne M. [Academic Medical Center Amsterdam, Department of Radiology, Amsterdam (Netherlands)

    2008-01-15

    Wilms tumour is a great therapeutic success story within paediatric oncology; its prognosis is excellent. Although mainly sporadic, occurring in otherwise well children, it occurs in a small number of genetically predisposed children. Thus regular surveillance imaging is performed in predisposed children in parts of the USA and Europe. The risks and benefits of surveillance are unclear, as the existing ad-hoc surveillance protocols are lacking in consistency of practice and equity of provision. We present guidelines for Wilms tumour surveillance based on a review of current practice and available evidence, outlined by a multidisciplinary working group in the UK. Wilms tumours are bilateral in 4-13% of affected children. Bilateral synchronous nephroblastomas are observed in 5% of affected children and are usually associated with the presence of nephrogenic rests, congenital malformations and predisposing syndromes. The major challenge in bilateral disease is to achieve a cure and at the same time to preserve sufficient functional renal tissue for normal growth and development. The association among Wilms tumour, nephrogenic rests and nephroblastomatosis makes detection and characterization of renal lesions with imaging extremely important. We discuss the relative strengths and weaknesses of the different modalities used for diagnosis and follow-up in bilateral renal disease. We also discuss newly emerging diagnostic imaging tests such as {sup 18}F-fluorodeoxyglucose positron emission tomography (FDG-PET). This technique, when fused with CT (PET-CT), allows accelerated metabolic activity to be accurately anatomically localised and so is potentially useful for staging, assessment of treatment response, and for surgical and radiotherapy planning. In addition, quantitative MRI techniques have been proved to be valuable in intracranial tumours, but no such role has been validated in abdominal disease. Diffusion-weighted imaging with calculation of ADC maps is feasible in

  2. Bilateral disease and new trends in Wilms tumour

    International Nuclear Information System (INIS)

    Owens, Catherine M.; Olsen, Oeystein E.; Brisse, Herve J.; Begent, Joanna; Smets, Anne M.

    2008-01-01

    Wilms tumour is a great therapeutic success story within paediatric oncology; its prognosis is excellent. Although mainly sporadic, occurring in otherwise well children, it occurs in a small number of genetically predisposed children. Thus regular surveillance imaging is performed in predisposed children in parts of the USA and Europe. The risks and benefits of surveillance are unclear, as the existing ad-hoc surveillance protocols are lacking in consistency of practice and equity of provision. We present guidelines for Wilms tumour surveillance based on a review of current practice and available evidence, outlined by a multidisciplinary working group in the UK. Wilms tumours are bilateral in 4-13% of affected children. Bilateral synchronous nephroblastomas are observed in 5% of affected children and are usually associated with the presence of nephrogenic rests, congenital malformations and predisposing syndromes. The major challenge in bilateral disease is to achieve a cure and at the same time to preserve sufficient functional renal tissue for normal growth and development. The association among Wilms tumour, nephrogenic rests and nephroblastomatosis makes detection and characterization of renal lesions with imaging extremely important. We discuss the relative strengths and weaknesses of the different modalities used for diagnosis and follow-up in bilateral renal disease. We also discuss newly emerging diagnostic imaging tests such as 18 F-fluorodeoxyglucose positron emission tomography (FDG-PET). This technique, when fused with CT (PET-CT), allows accelerated metabolic activity to be accurately anatomically localised and so is potentially useful for staging, assessment of treatment response, and for surgical and radiotherapy planning. In addition, quantitative MRI techniques have been proved to be valuable in intracranial tumours, but no such role has been validated in abdominal disease. Diffusion-weighted imaging with calculation of ADC maps is feasible in

  3. Recurrent bilateral renal calculi in a tetraplegic patient

    DEFF Research Database (Denmark)

    Vaidyanathan, S; Soni, B M; Biering-Sorensen, F

    1998-01-01

    An 18-year-old male developed C-5 complete tetraplegia following a motor-cycle accident in May 1975. The neuropathic bladder was managed by an indwelling urethral catheter. He developed recurrent episodes of urinary infection with Proteus species. In September 1975, an X-ray of the abdomen revealed...... small calculi in both the kidneys. In July 1976, he underwent transurethral resection of the bladder neck and division of the external urethral sphincter; subsequently, he was put on a penile sheath drainage. He continued to suffer from repeated episodes of urinary tract infection with Proteus...

  4. Outcome and renal function following salvage surgery for bilateral ...

    African Journals Online (AJOL)

    Chemotherapy followed by nephron-sparing surgery. (NSS) has become the ... 10 showed a mean. 24% decrease in tumor size following chemotherapy. .... month postoperatively (cases 1 and 3). The type of operation did not seem to correlate with the need for antihypertensives in our study as compared with the study by ...

  5. Renal carcinoma: bilateral breast metastases in a child

    International Nuclear Information System (INIS)

    Pursner, M.; Petchprapa, C.; Haller, J.O.; Orentlicher, R.J.

    1997-01-01

    We report the successful insertion and subsequent retrieval of a Gunther-Tulip vena cava filter in a patient with an anomalous left-sided inferior vena cava, who developed a right ilio-femoral venous thrombosis prior to planned surgical resection of a right femoral osteosarcoma. The indication was for short-term prophylaxis against pulmonary embolism during manipulative leg surgery. (orig.)

  6. THE PURE RED BLOOD CELL APLASIA IN RENAL TRANSPLANT RECIPIENT

    Directory of Open Access Journals (Sweden)

    B. T. Dzumabaeva

    2011-01-01

    Full Text Available The pure red blood cell aplasia of renal transplant recipients caused by parvovirus B19 (PB19 is characterized by persistent anemia which resistant to erythropoietin therapy, lack of reticulocytes, bone marrow hypoplasia, and clinically accompanied by severe recurrent bacterial, fungal and viral infection. In case of reactivation PB19 it is necessarv, first of all, eliminate the causes activation of this virus and to cancel or reduce the dose of drugs which depressed the normal hematopoiesis germs, thus to reduce the pancytopenia associating complications in this population. 

  7. 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

  8. Successful angioplasty of tripolar renal arteries in a single setting: a case report.

    Science.gov (United States)

    Sharma, Gyarsi Lal; Morice, Marie-Claude; Catineau, Patrick

    2002-08-01

    Renal artery stenosis (RAS) is one of the important causes of correctable hypertension. There are various modes of therapy for RAS, including percutaneous transluminal renal angioplasty (PTRA) and surgery. PTRA has emerged as the treatment of choice in cases of renal artery stenosis. PTRA combined with stenting is associated with good immediate and long-term results. This case report describes successful angioplasty of bilateral multiple renal arteries in a single setting with good immediate and follow-up results.

  9. Renal scan

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/003790.htm Renal scan To use the sharing features on this ... anaphylaxis . Alternative Names Renogram; Kidney scan Images Kidney anatomy Kidney - blood and urine flow References Chernecky CC, ...

  10. Renal Hemangiopericytoma

    Directory of Open Access Journals (Sweden)

    İbrahim Halil Bozkurt

    2015-03-01

    Full Text Available Hemangiopericytoma is an uncommon perivascular tumor originating from pericytes in the pelvis, head and tneck, and the meninges; extremely rarely in the urinary system. We report a case of incidentally detected renal mass in which radiologic evaluation was suggestive of renal cell carcinoma. First, we performed partial nephrectomy, and then, radical nephrectomy because of positive surgical margins and the pathological examination of the surgical specimen that revealed a hemangiopericytoma. No additional treatment was administered.

  11. Bilateral Olecranon Tophaceous Gout Bursitis

    Directory of Open Access Journals (Sweden)

    Güzelali Özdemir

    2017-01-01

    Full Text Available In this case, we present a patient with the diagnosis of bilateral olecranon tophaceous gout. After the surgical treatment, there was no limitation of range of motion or wound problem at 6th month control.

  12. Bilateral, independent juvenile nasopharyngeal angiofibroma

    DEFF Research Database (Denmark)

    Mørkenborg, Marie-Louise; Frendø, M; Stavngaard, T

    2015-01-01

    BACKGROUND: Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating...... from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning...... embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. CONCLUSION: This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral...

  13. Bilateral internal laryngoceles mimicking asthma

    Directory of Open Access Journals (Sweden)

    Elif A Aksoy

    2013-01-01

    Full Text Available Laryngocele is an air-filled, abnormal dilation of the laryngeal saccule that extends upward within the false vocal fold, in communication with the laryngeal lumen. A case of 43-year-old male with bilateral internal laryngoceles, who has been treated as asthma for 4 years, is presented. The patient had dyspnea, cough, and excessive phlegm for a month and a late onset stridor. Flexible nasopharyngolaryngoscopy showed bilateral cystic enlargements of the false vocal folds and true vocal folds could not be visualized. Laryngeal CT without contrast enhancement showed bilateral internal laryngoceles. Submucosal total excision of bilateral cystic masses including parts of false vocal folds was performed. The symptoms resolved immediately after surgery. Although the incidence of internal laryngocele is rare, it should be remembered in the differential diagnosis of upper airway problems and diagnostic flexible nasopharnygolaryngoscopy is routinely indicated for airway evaluation in at-risk patients.

  14. Simultaneous bilateral patellar tendon rupture ?

    OpenAIRE

    Moura, Diogo Lino; Marques, Jos? Pedro; Lucas, Francisco Manuel; Fonseca, Fernando Pereira

    2016-01-01

    Bilateral patellar tendon rupture is a rare entity, often associated with systemic diseases and patellar tendinopathy. The authors report a rare case of a 34-year-old man with simultaneous bilateral rupture of the patellar tendon caused by minor trauma. The patient is a retired basketball player with no past complaints of chronic knee pain and a history of steroid use. Surgical management consisted in primary end-to-end tendon repair protected temporarily with cerclage wiring, followed by a s...

  15. Bilateral sarkoidose i glandula parotis

    DEFF Research Database (Denmark)

    Hahn, Pernille; Krogdahl, Annelise; Godballe, Christian

    2012-01-01

    We describe an unusual case of sarcoidosis in which the patient presented with a bilateral swelling of the parotid salivary glands and no other manifestation of the disease. Sarcoidosis is a multisystem granulomatous disorder of unknown cause in which there may be multiple exocrine involvement......, including the salivary glands. This case emphasises the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling....

  16. 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.

  17. 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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

  3. A huge bladder calculus causing acute renal failure.

    Science.gov (United States)

    Komeya, Mitsuru; Sahoda, Tamami; Sugiura, Shinpei; Sawada, Takuto; Kitami, Kazuo

    2013-02-01

    A 81-year-old male was referred to our emergency outpatient unit due to acute renal failure. The level of serum creatinine was 276 μmol/l. A CT scan showed bilateral hydronephroureter, large bladder stone (7 cm × 6 cm × 6 cm) and bladder wall thickness. He was diagnosed as post renal failure due to bilateral hydronephroureter. Large bladder stone is thought to be the cause of bilateral hydronephroureter and renal failure. To improve renal failure, we performed open cystolithotomy and urethral catheterization. Three days after the surgery, the level of serum creatinine decreased to 224 μmol/l. He was discharged from our hospital with uneventful course. Bladder calculus is thought to be a rare cause of renal failure. We summarize the characteristics of bladder calculus causing renal failure. We should keep that long-term pyuria and urinary symptom, and repeated urinary tract infection can cause huge bladder calculus and renal failure in mind.

  4. Flash pulmonary edema in patients with renal artery stenosis--the Pickering Syndrome

    DEFF Research Database (Denmark)

    Pelta, Anna; Andersen, Ulrik B; Just, Sven

    2010-01-01

    We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases.......We report the prevalence of flash pulmonary edema in patients consecutively referred for balloon angioplasty of uni- or bilateral renal artery stenosis (PTRA), and describe the characteristics of this special fraction of the patients. We further report two unusual cases....

  5. 38 CFR 4.26 - Bilateral factor.

    Science.gov (United States)

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Bilateral factor. 4.26... DISABILITIES General Policy in Rating § 4.26 Bilateral factor. When a partial disability results from disease... disability. The bilateral factor will be applied to such bilateral disabilities before other combinations are...

  6. Pneumomediastinum and bilateral pneumothorax following near drowning in shallow water

    Directory of Open Access Journals (Sweden)

    Santhiya Govindaraj

    2011-10-01

    Full Text Available We report pneumomediastinum, bilateral pneumothorax and acute respiratory distress syndrome in a victim of near drowning who was intoxicated and did not have thoracic or neck trauma. Chest radiograph revealed the above findings, later confirmed by computed tomography. He was in shock and also had gastrointestinal (GI bleeding and renal dysfunction. With adequate resuscitative measures including fluids, blood transfusions, intercostal tube drainage and mechanical ventilation he made a complete recovery. Good prognostic indicators in near drowning patients include higher Glasgow Coma Scale, short submersion time and quick resuscitative measures even in the presence of serious cardiorespiratory or hemodynamic compromise.

  7. Pneumomediastinum and bilateral pneumothorax following near drowning in shallow water

    Directory of Open Access Journals (Sweden)

    Stalin Viswanathan

    2011-09-01

    Full Text Available We report pneumomediastinum, bilateral pneumothorax and acute respiratory distress syndrome in a victim of near drowning who was intoxicated and did not have thoracic or neck trauma. Chest radiograph revealed the above findings, later confirmed by computed tomography. He was in shock and also had gastrointestinal (GI bleeding and renal dysfunction. With adequate resuscitative measures including fluids, blood transfusions, intercostal tube drainage and mechanical ventilation he made a complete recovery. Good prognostic indicators in near drowning patients include higher Glasgow Coma Scale, short submersion time and quick resuscitative measures even in the presence of serious cardiorespiratory or hemodynamic compromise.

  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. Defining and predicting 'intrauterine fetal renal failure' in congenital lower urinary tract obstruction.

    Science.gov (United States)

    Ruano, Rodrigo; Safdar, Adnan; Au, Jason; Koh, Chester J; Gargollo, Patricio; Shamshirsaz, Alireza A; Espinoza, Jimmy; Cass, Darrell L; Olutoye, Oluyinka O; Olutoye, Olutoyin A; Welty, Stephen; Roth, David R; Belfort, Michael A; Braun, Michael C

    2016-04-01

    The aim of this study was to identify predictors of 'intrauterine fetal renal failure' in fetuses with severe congenital lower urinary tract obstruction (LUTO). We undertook a retrospective study of 31 consecutive fetuses with a diagnosis of LUTO in a tertiary Fetal Center between April 2013 and April 2015. Predictors of 'intrauterine fetal renal failure' were evaluated in those infants with severe LUTO who had either a primary composite outcome measure of neonatal death in the first 24 h of life due to severe pulmonary hypoplasia or a need for renal replacement therapy within 7 days of life. The following variables were analyzed: fetal bladder re-expansion 48 h after vesicocentesis, fetal renal ultrasound characteristics, fetal urinary indices, and amniotic fluid volume. Of the 31 fetuses included in the study, eight met the criteria for 'intrauterine fetal renal failure'. All of the latter had composite poor postnatal outcomes based on death within 24 h of life (n = 6) or need for dialysis within 1 week of life (n = 2). The percentage of fetal bladder refilling after vesicocentesis at time of initial evaluation was the only predictor of 'intrauterine fetal renal failure' (cut-off <27 %, area under the time-concentration curve 0.86, 95 % confidence interval 0.68-0.99; p = 0.009). We propose the concept of 'intrauterine fetal renal failure' in fetuses with the most severe forms of LUTO. Fetal bladder refilling can be used to reliably predict 'intrauterine fetal renal failure', which is associated with severe pulmonary hypoplasia or the need for dialysis within a few days of life.

  10. Bilateral Endoscopic Medial Maxillectomy for Bilateral Inverted Papilloma

    Science.gov (United States)

    Kodama, Satoru; Kawano, Toshiaki; Suzuki, Masashi

    2012-01-01

    Inverted papilloma (IP) is a benign tumor of the nasal cavity and paranasal sinuses that is unilateral in most cases. Bilateral IP, involving both sides of the nasal cavity and sinuses, is extremely rare. This paper describes a large IP that filled in both sides of the nasal cavity and sinuses, mimicking association with malignancy. The tumor was successfully treated by bilateral endoscopic medial maxillectomy (EMM). The patient is without evidence of the disease 24 months after surgery. If preoperative diagnosis does not confirm the association with malignancy in IP, endoscopic sinus surgery (ESS) should be selected, and ESS, including EMM, is a good first choice of the treatment for IP. PMID:22953103

  11. Bilateral perinephric pseudocysts and polycystic kidneys in a ferret

    International Nuclear Information System (INIS)

    Puerto, D.A.; Walker, L.M.; Saunders, H.M.

    1998-01-01

    A 3-year-old castrated male domestic ferret was evaluated for abdominal distention. Survey lateral and dorsoventral abdominal radiographs were made. There were two soft tissue radiopacities consistent with grossly enlarged kidneys displacing small bowel and colon cranially, ventrally and caudally. Abdominal ultrasound was performed and revealed bilateral perinephric pseudocysts and polycystic kidneys. The perinephric pseudocysts were found to be dilated renal capsules on exploratory surgery and were drained. On follow up examinations, the pseudocysts were drained by ultrasound-guided paracentesis. The perinephric cyst fluid was distinguished from urine by measuring creatinine concentration and plans were made to resect the renal capsules due to rapid re-accumulation of pseudocyst fluid. The ferret's condition deteriorated and euthanasia was performed. Post-mortem examination was declined by the owner. Perinephric pseudocysts are rare and this is the first published report in a ferret. Ultrasound examination is the most rapid, accurate and non-invasive method for diagnosis of perinephric pseudocysts

  12. Bilateral spontaneous hemotympanum: Case report

    Directory of Open Access Journals (Sweden)

    Economou Nicolas C

    2006-10-01

    Full Text Available Abstract Background The most common causes of hemotympanum are therapeutic nasal packing, epistaxis, blood disorders and blunt trauma to the head. Hemotympanum is characterized as idiopathic, when it is detected in the presence of chronic otitis media. A rare case of spontaneous bilateral hemotympanum in a patient treated with anticoagulants is presented herein. Case presentation A 72-year-old male presented with acute deterioration of hearing. In the patient's medical history aortic valve replacement 1 year before presentation was reported. Since then he had been administered regularly coumarinic anticoagulants, with INR levels maintained between 3.4 and 4.0. Otoscopy revealed the presence of bilateral hemotympanum. The audiogram showed symmetrical moderately severe mixed hearing loss bilaterally, with the conductive component predominating. Tympanograms were flat bilaterally with absent acoustic reflexes. A computerized tomography scan showed the presence of fluid in the mastoid and middle ear bilaterally. Treatment was conservative and consisted of a 10-day course of antibiotics, anticongestants and temporary interruption of the anticoagulant therapy. After 3 weeks, normal tympanic membranes were found and hearing had returned to previous levels. Conclusion Anticoagulant intake should be included in the differential diagnosis of hemotympanum, because its detection and appropriate treatment may lead to resolution of the disorder.

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

    Science.gov (United States)

    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

  14. 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.

  15. 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.

  16. 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.

  17. Exercise Induced Rhabdomyolysis with Compartment Syndrome and Renal Failure

    Directory of Open Access Journals (Sweden)

    Mary Colleen Bhalla

    2014-01-01

    Full Text Available Exertional rhabdomyolysis is sequela that is occasionally seen after strenuous exercise. The progression to compartment syndrome or renal failure is a rare complication that requires prompt recognition and treatment to prevent morbidity (Giannoglou et al. 2007. We present a case of a 22-year-old college football player who presented to the emergency department (ED after a typical leg workout as part of his weight conditioning. He was found to have rhabdomyolysis with evidence of renal insufficiency. His condition progressed to bilateral compartment syndrome and renal failure requiring dialysis. After bilateral fasciotomies were performed he had resolution of his compartment syndrome. He continued to be dialysis dependent and had no return of his renal function at discharge 12 days after admission.

  18. Acute renal failure after ingestion of guaifenesin and dextromethorphan.

    Science.gov (United States)

    Small, Evan; Sandefur, Benjamin J

    2014-07-01

    Guaifenesin is a common nonprescription medication that has been implicated in drug-induced nephrolithiasis. Dextromethorphan, a nonprescription antitussive found in some guaifenesin-containing preparations, is increasingly recognized as a substance of abuse by many youth and young adults. Renally excreted medications known to have poor solubility in urine have the potential to precipitate when ingested in large quantity, leading to acute obstruction of the ureters and renal failure. We describe the case of a 22-year-old male who developed severe bilateral flank pain, hematuria, and oliguria after an isolated recreational ingestion of guaifenesin and dextromethorphan. The patient was found to have bilateral ureteral obstruction and acute renal failure, suspected to be secondary to precipitation of medication metabolites in the urine. This case highlights the potential for acute renal failure secondary to guaifenesin and dextromethorphan abuse. Copyright © 2014 Elsevier Inc. All rights reserved.

  19. Achondroplasia Associated with Bilateral Keratoconus

    Directory of Open Access Journals (Sweden)

    Ammar M. Al Mahmood

    2012-01-01

    Full Text Available We report a rare case of bilateral keratoconus in association with achondroplasia. A 26-year-old male, with a known case of achondroplasia, complained of bilateral gradual deterioration in vision for the past few years. Slit lamp biomicroscopy showed bilateral central corneal protrusion and stromal thinning at the apex consistent with keratoconus. a trial of hard contact lens fitting failed to improve VA in the left eye (LE. Right eye (RE improved to 20/25. The patient underwent penetrating keratoplasty (PKP in his LE. Twenty-seven months postoperatively, uncorrected visual acuity (UCVA was 20/30. Ophthalmologists should be aware that patients with achondroplasia who complain of poor vision should be suspected of having keratoconus once other more common conditions are ruled out.

  20. Prognostic factors in neonatal acute renal failure

    International Nuclear Information System (INIS)

    Chevalier, R.L.; Campbell, F.; Brenbridge, A.N.

    1984-01-01

    Sixteen infants, 2 to 35 days of age, had acute renal failure, a diagnosis based on serum creatinine concentrations greater than 1.5 mg/dL for at least 24 hours. Eight infants were oliguric (urine flow less than 1.0 mL/kg/h) whereas the remainder were nonoliguric. To determine clinical parameters useful in prognosis, urine flow rate, duration of anuria, peak serum creatinine, urea (BUN) concentration, and nuclide uptake by scintigraphy were correlated with recovery. Nine infants had acute renal failure secondary to perinatal asphyxia, three had acute renal failure as a result of congenital cardiovascular disease, and four had major renal anomalies. Four oliguric patients died: three of renal failure and one of heart failure. All nonoliguric infants survived with mean follow-up serum creatinine concentration of 0.8 +/- 0.5 (SD) mg/dL whereas that of oliguric survivors was 0.6 +/- 0.3 mg/dL. Peak serum creatinine concentration did not differ between those patients who were dying and those recovering. All infants who were dying remained anuric at least four days and revealed no renal uptake of nuclide. Eleven survivors were anuric three days or less, and renal perfusion was detectable by scintigraphy in each case. However, the remaining survivor (with bilateral renal vein thrombosis) recovered after 15 days of anuria despite nonvisualization of kidneys by scintigraphy. In neonates with ischemic acute renal failure, lack of oliguria and the presence of identifiable renal uptake of nuclide suggest a favorable prognosis

  1. Prognostic factors in neonatal acute renal failure

    Energy Technology Data Exchange (ETDEWEB)

    Chevalier, R.L.; Campbell, F.; Brenbridge, A.N.

    1984-08-01

    Sixteen infants, 2 to 35 days of age, had acute renal failure, a diagnosis based on serum creatinine concentrations greater than 1.5 mg/dL for at least 24 hours. Eight infants were oliguric (urine flow less than 1.0 mL/kg/h) whereas the remainder were nonoliguric. To determine clinical parameters useful in prognosis, urine flow rate, duration of anuria, peak serum creatinine, urea (BUN) concentration, and nuclide uptake by scintigraphy were correlated with recovery. Nine infants had acute renal failure secondary to perinatal asphyxia, three had acute renal failure as a result of congenital cardiovascular disease, and four had major renal anomalies. Four oliguric patients died: three of renal failure and one of heart failure. All nonoliguric infants survived with mean follow-up serum creatinine concentration of 0.8 +/- 0.5 (SD) mg/dL whereas that of oliguric survivors was 0.6 +/- 0.3 mg/dL. Peak serum creatinine concentration did not differ between those patients who were dying and those recovering. All infants who were dying remained anuric at least four days and revealed no renal uptake of nuclide. Eleven survivors were anuric three days or less, and renal perfusion was detectable by scintigraphy in each case. However, the remaining survivor (with bilateral renal vein thrombosis) recovered after 15 days of anuria despite nonvisualization of kidneys by scintigraphy. In neonates with ischemic acute renal failure, lack of oliguria and the presence of identifiable renal uptake of nuclide suggest a favorable prognosis.

  2. Fetal polycystic renal disease: prenatal sonographic findings with pathologic correlation

    International Nuclear Information System (INIS)

    Jun, Soon Ae; Park, Yong Hyun; Cha, Sun Hee; Kay, Jung Woong; Cho, Joo Yeon; Cha, Kwang Yul; Cha, Kyung Sub; Chi, Je G.

    1990-01-01

    Polycystic renal disease are congenital disorders, most of which are fatal in the postnatal period. A series of ten cases of polycystic renal disease diagnosed prenatally by ultrasonography is presented. Diagnostic criteria of ultrasonography for cystic renal disease are; 1. enlarge kidney (4 cases) 2. echogenic density of kidney (3 cases) 3. 0.4 - 0.9cm sized multiple cysts within the renal cortex (3 cases) 4. decreased amount of amniotic fluid (4 cases) 5. hydronephrosis (4 cases) 6. distended bladder (2 cases) 7. absence of bladder (2 cases) Eight of ten cases were confirmed by autopsy. Seven cases had other associated congenital anomalies, i.e. pulmonary hypoplasia (5), hepatic fibrosis (3), congenital heart disease (3), tracheoesophageal fistula with imperforate anus (1), caudal regression syndrome (1), Meckel-Gruber syndrome (1) and ambiguous genitalia (2). Additional cytogenetic study of the fetus and the careful family history taking followed by prenatal diagnosis of cystic renal disease. Precise prenatal diagnosis may allow patients the option of elective abortion or may prevent unnecessary obstetric intervention

  3. Simultaneous bilateral patellar tendon rupture.

    Science.gov (United States)

    Moura, Diogo Lino; Marques, José Pedro; Lucas, Francisco Manuel; Fonseca, Fernando Pereira

    2017-01-01

    Bilateral patellar tendon rupture is a rare entity, often associated with systemic diseases and patellar tendinopathy. The authors report a rare case of a 34-year-old man with simultaneous bilateral rupture of the patellar tendon caused by minor trauma. The patient is a retired basketball player with no past complaints of chronic knee pain and a history of steroid use. Surgical management consisted in primary end-to-end tendon repair protected temporarily with cerclage wiring, followed by a short immobilization period and intensive rehabilitation program. Five months after surgery, the patient was able to fully participate in sport activities.

  4. Simultaneous bilateral patellar tendon rupture

    Directory of Open Access Journals (Sweden)

    Diogo Lino Moura

    Full Text Available ABSTRACT Bilateral patellar tendon rupture is a rare entity, often associated with systemic diseases and patellar tendinopathy. The authors report a rare case of a 34-year-old man with simultaneous bilateral rupture of the patellar tendon caused by minor trauma. The patient is a retired basketball player with no past complaints of chronic knee pain and a history of steroid use. Surgical management consisted in primary end-to-end tendon repair protected temporarily with cerclage wiring, followed by a short immobilization period and intensive rehabilitation program. Five months after surgery, the patient was able to fully participate in sport activities.

  5. Idiopathic renal hematuria in a dog; the usefulness of a method of partial occlusion of the renal artery.

    Science.gov (United States)

    Mishina, M; Watanabe, T; Yugeta, N; Maeda, H; Fujii, K; Wakao, Y; Takahashi, M; Yamamura, H

    1997-04-01

    Exploratory laparotomy was performed on a dog suspected of having idiopathic renal hematuria. Two catheters were inserted into the bilateral ureters, and hematuria from the left kidney was confirmed. The blood flow was occluded in the ventral and dorsal rami of the left renal artery in order to localize the site of hemorrhage. As hematuria disappeared when the dorsal ramus was occluded, the site of renal hematuria was localized to the area dominated by the dorsal ramus of the renal artery. As a result of ligating the dorsal ramus of the left renal artery in this dog, renal hematuria subsided, and the dog has shown a favorable course, to date, one year after surgery.

  6. Computed tomography of renal cell carcinoma in patients with terminal renal impairment

    International Nuclear Information System (INIS)

    Ferda, Jiri; Hora, Milan; Hes, Ondrej; Reischig, Tomas; Kreuzberg, Boris; Mirka, Hynek; Ferdova, Eva; Ohlidalova, Kristyna; Baxa, Jan; Urge, Tomas

    2007-01-01

    Purpose: An increased incidence of renal tumors has been observed in patients with end-stage-renal-disease (ESRD). The very strong association with acquired renal cystic disease (ACRD) and increased incidence of the renal tumors (conventional renal cell carcinoma (CRCC), papillary renal cell carcinoma (PRCC) or papillary renal cell adenoma (PRCA)) was reported. This study discusses the role of computed tomography (CT) in detecting renal tumors in patients with renal impairment: pre-dialysis, those receiving dialysis or with renal allograft transplants. Materials and methods: Ten patients (nine male, one female) with renal cell tumors were enrolled into a retrospective study; two were new dialysis patients, three on long-term dialysis, and five were renal transplant recipients with history of dialysis. All patients underwent helical CT, a total of 11 procedures were performed. Sixteen-row detector system was used five times, and a 64-row detector system for the six examinations. All patients underwent nephrectomy of kidney with suspected tumor, 15 nephrectomies were performed, and 1 kidney was assessed during autopsy. CT findings were compared with macroscopic and microscopic assessments of the kidney specimen in 16 cases. Results: Very advanced renal parenchyma atrophy with small cysts corresponding to ESRD was found in nine patients, chronic pyelonephritis in remained one. A spontaneously ruptured tumor was detected incidentally in one case, patient died 2 years later. In the present study, 6.25% (1/16) were multiple PRCA, 12.5% (2/16) were solitary PRCC, 12.5% tumors (2/16) were solitary conventional renal cell carcinomas (CRCC's), 12.5% tumors (2/16) were multiple conventional renal cell carcinomas (CRCC's), 25% (4/16) were CRCC's combined with multiple papillary renal cell carcinomas with adenomas (PRCC's and PRCA's), and 25% (4/16) of the tumors were multiple PRCC's combined with PRCA's without coexisting CRCC's. Bilateral renal tumors were found in our study

  7. Unusual renal angiomyolipoma in tuberous sclerosis

    International Nuclear Information System (INIS)

    Schwartz, A.M.

    1980-01-01

    A patient with tuberous sclerosis and a normal intravenous urogram 5 years previously presented with a large and palpable upper pole renal mass. Since patients with tuberous sclerosis have small bilateral hamartomas, a Wilms' tumor was suspected. In retrospect, inhomogeneous nephrograms should have alerted the radiologist to the multiplicity of other small lesions. Also, a partially lucent rim should have substantiated that the lesion was not a Wilms' tumor. (orig.) [de

  8. Bilateral tension pneumothorax after acupuncture.

    Science.gov (United States)

    Mohammad, Nurashikin

    2018-04-19

    Acupuncture is an ancient complementary medicine which is currently used worldwide. Many serious adverse events have been reported which include a spectrum of mild-to-fatal complications. However, the level of awareness with regard to complications is still low both to physicians and patients. We report a 63-year-old who presented with acute shortness of breath 2 hours after having had acupuncture. On examination, there was absent breath sound heard on the left lung and slightly reduced breath sound on the right lung. She had type 1 respiratory failure. Urgent chest radiograph confirmed bilateral pneumothorax which was more severe on the left with tension pneumothorax and mediastinal shift. Chest tubes were inserted bilaterally after failed needle aspiration attempts. Subsequently, the pneumothoraces resolved, and she was discharged well. The bilateral pneumothoraces caused by acupuncture were curable but could have been potentially fatal if diagnosis was delayed. This case report adds to the limited current literature on the complications of acupuncture leading to bilateral pneumothoraces. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  9. Bilateral breast in brothers - abreast

    Directory of Open Access Journals (Sweden)

    Altamash Mohammed Yusuf Shaikh

    2013-01-01

    Full Text Available Gynecomastia is a common occurrence in pubertal age group, and is physiological in up to 65 percent of cases. When occurs in the family it should be investigated in order not to miss on a treatable etiology. Two brothers within the same family, presenting with bilateral gynecomastia of different causes and requiring different treatment are presented.

  10. Branchio-oto-renal syndrome caused by partial EYA1 deletion due to LINE-1 insertion

    DEFF Research Database (Denmark)

    Morisada, Naoya; Rendtorff, Nanna Dahl; Nozu, Kandai

    2010-01-01

    A 7-year-old Japanese girl with conductive deafness and preauricular fistulae developed proteinuria. She had renal insufficiency, and ultrasound revealed bilateral small kidneys. These findings indicated that she had branchio-oto-renal (BOR) syndrome. In the present patient, we identified, by usi...

  11. Renal lymphangiectasia: incidental finding at multislice computed tomography and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Vasconcelos, Rodrigo Abdalla de; Pereira, Emanuelle Santiago [Hospital de Base do Distrito Federal (HBDF), Brasilia, DF (Brazil); Bauab Junior, Tufik [Instituto de Radiodiagnostico Rio Preto - Ultra-X and Hospital de Base de Faculdade de Medicina de Sao Jose do Rio Preto (Famerp), Sao Jose do Rio Preto, SP (Brazil); Valente, Rodolfo Silva [Hospital Universitario de Brasilia (HUB), Brasilia, DF (Brazil)

    2012-05-15

    Renal lymphangiectasia is a rare condition characterized by parapyelic and perirenal fluid collections, which may progress from asymptomatic condition to chronic renal failure. The present report describes a case of incidental computed tomography finding of bilateral lymphangiectasia in an asymptomatic patient, as well as the main imaging findings with a comprehensive literature review. (author)

  12. Coarctation of the aorta and renal artery stenosis in tuberous sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Flynn, P M; Robinson, M B; Stapleton, F B; Roy, S III; Koh, G; Tonkin, I L.D.

    1984-07-01

    Among neurocutaneous disorders, coarctation of the abdominal aorta and renal artery stenosis have traditionally been associated with neurofibromatosis. We report a 5-year-old girl who was discovered to have bilateral renal artery stenosis, coarctation of the abdominal aorta, renal cysts and typical skin lesions of tuberous clerosis during the evaluation of asymptomatic hypertension. Renal vascular hypertension has not been reported previously in tuberous sclerosis. We conclude that the tuberous sclerosis complex should be expanded to include vascular malformations and the hypertension should not be assumed to be secondary to renal hamartomata or cysts in patients with tuberous sclerosis.

  13. Coarctation of the aorta and renal artery stenosis in tuberous sclerosis

    International Nuclear Information System (INIS)

    Flynn, P.M.; Robinson, M.B.; Stapleton, F.B.; Roy, S. III; Koh, G.; Tonkin, I.L.D.; Tennessee Univ., Memphis; Tennessee Univ., Memphis; LeBonheur Children's Medical Center, Memphis, TN

    1984-01-01

    Among neurocutaneous disorders, coarctation of the abdominal aorta and renal artery stenosis have traditionally been associated with neurofibromatosis. We report a 5-year-old girl who was discovered to have bilateral renal artery stenosis, coarctation of the abdominal aorta, renal cysts and typical skin lesions of tuberous clerosis during the evaluation of asymptomatic hypertension. Renal vascular hypertension has not been reported previously in tuberous sclerosis. We conclude that the tuberous sclerosis complex should be expanded to include vascular malformations and the hypertension should not be assumed to be secondary to renal hamartomata or cysts in patients with tuberous sclerosis. (orig.)

  14. Renal candidiasis

    International Nuclear Information System (INIS)

    Khanna, S.; Malik, N.; Khandelwal, N.

    1990-01-01

    Most fungal infections of the urinary tract are caused by Candida albicans, a yeast-like saprophytic fungus which may become apathogen under various conditions which lower the host resistance. The use of computed tomography in the diagnosis of renal fungus balls is the subject of this communication with emphasis on the radiologists role in the recognition of this entity. (H.W.). 6 refs.; 2 figs

  15. Renal hemangioma

    Directory of Open Access Journals (Sweden)

    Theodorico F. da Costa Neto

    2004-06-01

    Full Text Available INTRODUCTION: Renal hemangioma is a relatively rare benign tumor, seldom diagnosed as a cause of hematuria. CASE REPORT: A female 40-year old patient presented with continuous gross hematuria, anemia and episodic right lumbar pain, with onset about 3 months previously. The patient underwent multiple blood transfusions during her hospital stay and extensive imaging propedeutics was performed. Semi-rigid ureterorenoscopy evidenced a bleeding focus in the upper calix of the right kidney, with endoscopic treatment being unfeasible. The patient underwent right upper pole nephrectomy and presented a favorable outcome. Histopathological analysis of the surgical specimen showed that it was a renal hemangioma. COMMENTS: Imaging methods usually employed for diagnostic investigation of hematuria do not have good sensitivity for renal hemangioma. However, they are important to exclude the most frequent differential diagnoses. The ureterorenoscopy is the diagnostic method of choice and endoscopic treatment can be feasible when the lesion is accessible and electrocautery or laser are available. We emphasize the open surgical treatment as a therapeutic option upon failure of less invasive methods.

  16. Postradiation leiomyosarcoma of the orbit complicating bilateral retinoblastoma

    International Nuclear Information System (INIS)

    Font, R.L.; Jurco, S.; Brechner, R.J.

    1983-01-01

    A 31-year-old woman had bilateral retinoblastoma diagnosed in early childhood. The right eye was enucleated at the age of 1 year, and the left eye was treated with radiation therapy (a total dose of 16,000 rad). Twenty-three years later, in 1975, a subcutaneous mass was noted in the left periorbital region. A biopsy specimen of the mass was taken and a diagnosis of pleomorphic postradiation sarcoma was made. Electron microscopic studies of the periorbital mass confirmed the diagnosis of leiomyosarcoma. After additional radiation therapy, the residual mass was surgically excised. Five years later, a right renal mass, which histologically proved to be a renal cell carcinoma, was discovered. She was treated with nephrectomy, radiation, and chemotherapy. A recent follow-up examination disclosed that the patient is alive and apparently without any evidence of metastatic disease, 30 years after the diagnosis of bilateral retinoblastoma was made. The literature is reviewed regarding postradiation sarcomas and the occurrence of second malignant neoplasms in patients with retinoblastoma

  17. Postradiation leiomyosarcoma of the orbit complicating bilateral retinoblastoma.

    Science.gov (United States)

    Font, R L; Jurco, S; Brechner, R J

    1983-10-01

    A 31-year-old woman had bilateral retinoblastoma diagnosed in early childhood. The right eye was enucleated at the age of 1 year, and the left eye was treated with radiation therapy (a total dose of 16,000 rad). Twenty-three years later, in 1975, a subcutaneous mass was noted in the left periorbital region. A biopsy specimen of the mass was taken and a diagnosis of pleomorphic postradiation sarcoma was made. Electron microscopic studies of the periorbital mass confirmed the diagnosis of leiomyosarcoma. After additional radiation therapy, the residual mass was surgically excised. Five years later, a right renal mass, which histologically proved to be a renal cell carcinoma, was discovered. She was treated with nephrectomy, radiation, and chemotherapy. A recent follow-up examination disclosed that the patient is alive and apparently without any evidence of metastatic disease, 30 years after the diagnosis of bilateral retinoblastoma was made. The literature is reviewed regarding postradiation sarcomas and the occurrence of second malignant neoplasms in patients with retinoblastoma.

  18. 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...

  19. Obesity and renal hemodynamics

    NARCIS (Netherlands)

    Bosma, R. J.; Krikken, J. A.; van der Heide, J. J. Homan; de Jong, P. E.; Navis, G. J.

    2006-01-01

    Obesity is a risk factor for renal damage in native kidney disease and in renal transplant recipients. Obesity is associated with several renal risk factors such as hypertension and diabetes that may convey renal risk, but obesity is also associated with an unfavorable renal hemodynamic profile

  20. Radiopharmaceuticals for renal studies

    International Nuclear Information System (INIS)

    Verdera, Silvia

    1994-01-01

    Between the diagnostic techniques using radiopharmaceuticals in nuclear medicine it find renal studies.A brief description about renal glomerular filtration(GFR) and reliability renal plasma flux (ERPF),renal blood flux measurement agents (RBF),renal scintillation agents and radiation dose estimates by organ physiology was given in this study.tabs

  1. Bilateral Endoscopic Medial Maxillectomy for Bilateral Inverted Papilloma

    Directory of Open Access Journals (Sweden)

    Satoru Kodama

    2012-01-01

    Full Text Available Inverted papilloma (IP is a benign tumor of the nasal cavity and paranasal sinuses that is unilateral in most cases. Bilateral IP, involving both sides of the nasal cavity and sinuses, is extremely rare. This paper describes a large IP that filled in both sides of the nasal cavity and sinuses, mimicking association with malignancy. The tumor was successfully treated by bilateral endoscopic medial maxillectomy (EMM. The patient is without evidence of the disease 24 months after surgery. If preoperative diagnosis does not confirm the association with malignancy in IP, endoscopic sinus surgery (ESS should be selected, and ESS, including EMM, is a good first choice of the treatment for IP.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  4. Device-based approaches for renal nerve ablation for hypertension and beyond

    OpenAIRE

    Alicia Ann Thorp; Markus Peter Schlaich; Markus Peter Schlaich

    2015-01-01

    Animal and human studies have demonstrated that chronic activation of renal sympathetic nerves is critical in the pathogenesis and perpetuation of treatment-resistant hypertension. Bilateral renal denervation has emerged as a safe and effective, non-pharmacological treatment for resistant hypertension that involves the selective ablation of efferent and afferent renal nerves to lower blood pressure. However, the most recent and largest randomized controlled trial failed to confirm the primacy...

  5. Effects of renal denervation on tubular sodium handling in rats with CBL-induced liver cirrhosis

    DEFF Research Database (Denmark)

    Jonassen, T.E.; Brond, L.; Torp, M.

    2003-01-01

    This study was designed to examine the effect of bilateral renal denervation (DNX) on thick ascending limb of Henle's loop (TAL) function in rats with liver cirrhosis induced by common bile duct ligation (CBL). The CBL rats had, as previously shown, sodium retention associated with hypertrophy...... renal sympathetic nerve activity known to be present in CBL rats plays a significant role in the formation of sodium retention by stimulating sodium reabsorption in the TAL via increased renal abundance of NKCC2....

  6. 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.

  7. 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

  8. 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?

  9. 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.

  10. 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

  11. Bilateral Malrotation and a Congenital Pelvic Kidney with Varied Vasculature and Altered Hilar Anatomy

    Directory of Open Access Journals (Sweden)

    J. Singh

    2015-01-01

    Full Text Available Variations of structure and position of the kidney along with variations of renal vessels are most frequently reported. Rotational variations form a rare entity that are not cited in most embryology textbooks. During an educational cadaveric dissection of a 42-year-old male, a complex picture of bilateral anatomical variants was encountered. Malrotation of both kidneys and a left lobulated ectopic kidney along with open hilum was observed. The left kidney showed a pelvic position in front of sacral promontory with three renal arteries retaining its embryological aortoiliac branches and two renal veins draining into right common iliac vein. These variations have an embryological base. Pelvic kidney with rotational variation though comparatively rare assumes great importance in view of present-day surgical procedures like laparoscopic radical nephrectomy, percutaneous nephrectomy, and renal transplantation.

  12. Bilateral pallidotomy for generalized dystonia Palidotomia bilateral para distonias generalizadas

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    Hélio A. G. Teive

    2001-06-01

    Full Text Available OBJECTIVE: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM. Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.As distonias generalizadas são freqüentemente um desafio terapêutico, com pobres respostas aos tratamentos farmacológicos. As cirurgias estereotáxicas, como a palidotomia, têm sido utilizadas com êxito no tratamento da doença de Parkinson e estudos

  13. Immediate Sequential Bilateral Cataract Surgery

    DEFF Research Database (Denmark)

    Kessel, Line; Andresen, Jens; Erngaard, Ditte

    2015-01-01

    The aim of the present systematic review was to examine the benefits and harms associated with immediate sequential bilateral cataract surgery (ISBCS) with specific emphasis on the rate of complications, postoperative anisometropia, and subjective visual function in order to formulate evidence......-based national Danish guidelines for cataract surgery. A systematic literature review in PubMed, Embase, and Cochrane central databases identified three randomized controlled trials that compared outcome in patients randomized to ISBCS or bilateral cataract surgery on two different dates. Meta-analyses were...... performed using the Cochrane Review Manager software. The quality of the evidence was assessed using the GRADE method (Grading of Recommendation, Assessment, Development, and Evaluation). We did not find any difference in the risk of complications or visual outcome in patients randomized to ISBCS or surgery...

  14. China's Bilateral Currency Swap Lines

    OpenAIRE

    Zhitao, Lin; Wenjie, Zhan; Cheung, Yin-Wong

    2016-01-01

    We study the determinants of China’s bilateral local currency swap lines that were established since the recent global finance crisis. It is found that economic factors, political considerations, and institutional characteristics including trade intensity, economic size, strategic partnership, free trade agreement, corruption, and stability affect the decision of signing a swap line agreement. Once a swap line agreement decision is made, the size of the swap line is then mainly affected by tr...

  15. Renal tuberculosis

    Directory of Open Access Journals (Sweden)

    Džamić Zoran

    2016-01-01

    Full Text Available Tuberculosis is still a significant health problem in the world, mostly in developing countries. The special significance lies in immunocompromised patients, particularly those suffering from the HIV. Urogenital tuberculosis is one of the most common forms of extrapulmonary tuberculosis, while the most commonly involved organ is the kidney. Renal tuberculosis occurs by hematogenous dissemination of mycobacterium tuberculosis from a primary tuberculosis foci in the body. Tuberculosis is characterized by the formation of pathognomonic lesions in the tissues - granulomata. These granulomata may heal spontaneously or remain stable for years. In certain circumstances in the body associated with immunosuppression, the disease may be activated. Central caseous necrosis occurs within tuberculoma, leading to formation of cavities that destroy renal parenchyma. The process may gain access to the collecting system, forming the caverns. In this way, infection can be spread distally to renal pelvis, ureter and bladder. Scaring of tissue by tuberculosis process may lead to development of strictures of the urinary tract. The clinical manifestations are presented by nonspecific symptoms and signs, so tuberculosis can often be overlooked. Sterile pyuria is characteristic for urinary tuberculosis. Dysuric complaints, flank pain or hematuria may be presented in patients. Constitutional symptoms of fever, weight loss and night sweats are presented in some severe cases. Diagnosis is made by isolation of mycobacterium tuberculosis in urine samples, by cultures carried out on standard solid media optimized for mycobacterial growth. Different imaging studies are used in diagnostics - IVU, CT and NMR are the most important. Medical therapy is the main modality of tuberculosis treatment. The first line anti-tuberculosis drugs include isoniazid, rifampicin, pyrazinamide and ethambutol. Surgical treatment is required in some cases, to remove severely damaged kidney, if

  16. Simultaneous and staged bilateral total hip arthroplasty

    DEFF Research Database (Denmark)

    Lindberg-Larsen, Martin; Joergensen, Christoffer Calov; Husted, Henrik

    2013-01-01

    Bilateral total hip arthroplasty (BTHA) and bilateral simultaneous total hip arthroplasty (BSTHA) are done increasingly. Previous studies evaluating outcomes after bilateral procedures have found different results. The aim of this study was to investigate length of hospital stay (LOS), 30 days...

  17. Renal artery origins and variations: angiographic evaluation of 855 consecutive patients.

    Science.gov (United States)

    Ozkan, Uğur; Oğuzkurt, Levent; Tercan, Fahri; Kizilkiliç, Osman; Koç, Zafer; Koca, Nihal

    2006-12-01

    To determine angiographically the origins and variations of renal arteries. The study included 855 consecutive patients (163 females, 692 males; mean age, 61 years) living in the Cukurova region of Turkey, who underwent either aortofemoropopliteal (AFP) angiography for the investigation of peripheral arterial disease, or renal angiography for renovascular hypertension, and were prospectively evaluated. Renal arteries were visualized by non-selective catheterization during AFP angiography and by selective or non-selective catheterization during renal angiography. Locations of renal artery origins and renal artery variations, including the presence of extra renal arteries and division patterns were analyzed on angiograms. The origin of main renal arteries off the aorta was between the upper margin of L1 and lower margin of L2 vertebra in 98% of the patients, and in 74%, this was the origin of extra renal arteries. The most common location for renal artery origin was the L1-L2 intervertebral disc level. A single renal artery was present in both kidneys in 76% of patients. Renal artery variations included multiple arteries in 24%, bilateral multiple arteries in 5%, and early division in 8% of the cases. Additional renal arteries on the right side were found in 16% and on the left side in 13% of cases. Of all the extra renal arteries, the percentage of accessory and aberrant renal arteries were 49% and 51%, respectively. Renal arteries originated between the first and the second lumbar vertebral levels in most patients. Extra renal arteries were quite frequent. These results should be kept in mind when a non-invasive diagnostic search is performed for renal artery stenosis, or when renal surgery related to renal arteries is performed.

  18. Renal denervation

    DEFF Research Database (Denmark)

    Olsen, Lene Kjær; Kamper, Anne-Lise; Svendsen, Jesper Hastrup

    2015-01-01

    PURPOSE OF REVIEW: Renal denervation (RDN) has, within recent years, been suggested as a novel treatment option for patients with resistant hypertension. This review summarizes the current knowledge on this procedure as well as limitations and questions that remain to be answered. RECENT FINDINGS...... selection, anatomical and physiological effects of RDN as well as possible beneficial effects on other diseases with increased sympathetic activity. The long awaited Symplicity HTN-3 (2014) results illustrated that the RDN group and the sham-group had similar reductions in BP. SUMMARY: Initial studies...

  19. Renal papillary necrosis

    Science.gov (United States)

    ... asking your provider. Alternative Names Necrosis - renal papillae; Renal medullary necrosis Images Kidney anatomy Kidney - blood and urine flow References Bushinsky DA, Monk RD. Nephrolithiasis and nephrocalcinosis. ...

  20. 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.

  1. Renal calculus

    CERN Document Server

    Pyrah, Leslie N

    1979-01-01

    Stone in the urinary tract has fascinated the medical profession from the earliest times and has played an important part in the development of surgery. The earliest major planned operations were for the removal of vesical calculus; renal and ureteric calculi provided the first stimulus for the radiological investigation of the viscera, and the biochemical investigation of the causes of calculus formation has been the training ground for surgeons interested in metabolic disorders. It is therefore no surprise that stone has been the subject of a number of monographs by eminent urologists, but the rapid development of knowledge has made it possible for each one of these authors to produce something new. There is still a technical challenge to the surgeon in the removal of renal calculi, and on this topic we are always glad to have the advice of a master craftsman; but inevitably much of the interest centres on the elucidation of the causes of stone formation and its prevention. Professor Pyrah has had a long an...

  2. Bilateral cochlear implantation in a patient with bilateral temporal bone fractures.

    Science.gov (United States)

    Chung, Jae Ho; Shin, Myung Chul; Min, Hyun Jung; Park, Chul Won; Lee, Seung Hwan

    2011-01-01

    With the emphasis on bilateral hearing nowadays, bilateral cochlear implantation has been tried out for bilateral aural rehabilitation. Bilateral sensorineural hearing loss caused by head trauma can get help from cochlear implantation. We present the case of a 44-year-old man with bilateral otic capsule violating temporal bone fractures due to head trauma. The patient demonstrated much improved audiometric and psychoacoustic performance after bilateral cochlear implantation. We believe bilateral cochlear implantation in such patient can be a very effective tool for rehabilitation. Copyright © 2011 Elsevier Inc. All rights reserved.

  3. Does Renal Artery Supply Indicate Treatment Success of Renal Denervation?

    International Nuclear Information System (INIS)

    Schmid, Axel; Ditting, Tilmann; Sobotka, Paul A.; Veelken, Roland; Schmieder, Roland E.; Uder, Michael; Ott, Christian

    2013-01-01

    PurposeRenal denervation (RDN) emerged as an innovative interventional antihypertensive therapy. With the exception of pretreatment blood pressure (BP) level, no other clear predictor for treatment efficacy is yet known. We analyzed whether the presence of multiple renal arteries has an impact on BP reduction after RDN.MethodsFifty-three patients with treatment-resistant hypertension (office BP ≥ 140/90 mmHg and 24-h ambulatory BP monitoring (≥130/80 mmHg) underwent bilateral catheter-based RDN. Patients were stratified into one-vessel (OV) (both sides) and at least multivessel (MV) supply at one side. Both groups were treated on one vessel at each side; in case of multiple arteries, only the dominant artery was treated on each side.ResultsBaseline clinical characteristics (including BP, age, and estimated glomerular filtration rate) did not differ between patients with OV (n = 32) and MV (n = 21). Office BP was significantly reduced in both groups at 3 months (systolic: OV −15 ± 23 vs. MV −16 ± 20 mmHg; diastolic: OV −10 ± 12 vs. MV −8 ± 11 mmHg, both p = NS) as well as 6 months (systolic: OV −18 ± 18 vs. MV −17 ± 22 mmHg; diastolic: OV −10 ± 10 vs. −10 ± 12 mmHg, both p = NS) after RDN. There was no difference in responder rate (rate of patients with office systolic BP reduction of at least 10 mmHg after 6 months) between the groups.ConclusionIn patients with multiple renal arteries, RDN of one renal artery—namely, the dominant one—is sufficient to induce BP reduction in treatment-resistant hypertension

  4. Does Renal Artery Supply Indicate Treatment Success of Renal Denervation?

    Energy Technology Data Exchange (ETDEWEB)

    Schmid, Axel, E-mail: axel.schmid@uk-erlangen.de [University of Erlangen-Nuremberg, Department of Radiology (Germany); Ditting, Tilmann, E-mail: tilmann.ditting@uk-erlangen.de [University of Erlangen-Nuremberg, Department of Nephrology and Hypertension (Germany); Sobotka, Paul A., E-mail: sobotka@alumni.stanford.edu [Ohio State University (United States); Veelken, Roland, E-mail: roland.veelken@uk-erlangen.de; Schmieder, Roland E., E-mail: roland.schmieder@uk-erlangen.de [University of Erlangen-Nuremberg, Department of Nephrology and Hypertension (Germany); Uder, Michael, E-mail: michael.uder@uk-erlangen.de [University of Erlangen-Nuremberg, Department of Radiology (Germany); Ott, Christian, E-mail: christian.ott@uk-erlangen.de [University of Erlangen-Nuremberg, Department of Nephrology and Hypertension (Germany)

    2013-08-01

    PurposeRenal denervation (RDN) emerged as an innovative interventional antihypertensive therapy. With the exception of pretreatment blood pressure (BP) level, no other clear predictor for treatment efficacy is yet known. We analyzed whether the presence of multiple renal arteries has an impact on BP reduction after RDN.MethodsFifty-three patients with treatment-resistant hypertension (office BP {>=} 140/90 mmHg and 24-h ambulatory BP monitoring ({>=}130/80 mmHg) underwent bilateral catheter-based RDN. Patients were stratified into one-vessel (OV) (both sides) and at least multivessel (MV) supply at one side. Both groups were treated on one vessel at each side; in case of multiple arteries, only the dominant artery was treated on each side.ResultsBaseline clinical characteristics (including BP, age, and estimated glomerular filtration rate) did not differ between patients with OV (n = 32) and MV (n = 21). Office BP was significantly reduced in both groups at 3 months (systolic: OV -15 {+-} 23 vs. MV -16 {+-} 20 mmHg; diastolic: OV -10 {+-} 12 vs. MV -8 {+-} 11 mmHg, both p = NS) as well as 6 months (systolic: OV -18 {+-} 18 vs. MV -17 {+-} 22 mmHg; diastolic: OV -10 {+-} 10 vs. -10 {+-} 12 mmHg, both p = NS) after RDN. There was no difference in responder rate (rate of patients with office systolic BP reduction of at least 10 mmHg after 6 months) between the groups.ConclusionIn patients with multiple renal arteries, RDN of one renal artery-namely, the dominant one-is sufficient to induce BP reduction in treatment-resistant hypertension.

  5. Bilateral olecranon bursitis – A rare clinical presentation of gout

    Directory of Open Access Journals (Sweden)

    Betul Sargın

    2018-04-01

    Full Text Available Background: Gout is the most common form of crystal arthropathy. Monoarthritis affecting the first metatarsophalangeal joint is the common initial presentation. Bilateral olecranon bursitis is a rare presentation of gout. Aim of the work: To describe the clinical features of bilateral olecranon bursitis as an initial presentation of gout. Case report: A 62-year old male patient presented to the rheumatology clinic , Adnan Menderes University with sudden bilateral elbow pain and swelling for three months . Swellings gradually increased to the size of a golf ball with minimal restriction in the elbow extension (170°. He didn’t have arthritis in the elbows. The patient had medical history of heart failure and chronic obstructive pulmonary disease and medications received included acetylsalicylic acid and diuretics. Blood tests revealed elevated serum uric acid (12.6 mg/dL, with normal renal function tests, erythrocyte sedimentation rate (ESR 43 mm/h and C-reactive protein (CRP 8.8 mg/L. Rheumatoid factor and hepatitis markers were negative. Diagnostic bursal aspiration excluded septic bursitis and under polarized microscopy monosodium urate (MSU crystals were identified with typical negative birefringence. A diagnosis of gout was established. ESR and CRP were normalized after diclofenac potassium (100 mg and colchicine (1.5 mg. Allopurinol 300 mg was added when his joint pain was relieved. Conclusion: This is the first gout case initially presenting with bilateral olecranon bursitis. Bursal fluid analysis is important in such atypical presentation to look for MSU crystals and establish a diagnosis. Keywords: Olecranon bursitis, Gout, Crystal arthropathy, Monosodium urate

  6. Prenatal Diagnosis of Bilateral Ectrodactyly and Radial Agenesis Associated with Trisomy 10 Mosaicism

    Directory of Open Access Journals (Sweden)

    Jonathan Lévy

    2013-01-01

    Full Text Available Ectrodactyly or split hand and foot malformations (SHFMs are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks’ gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy.

  7. Síndrome branquio-oto-renal: A propósito de una familia Branchio-oto-renal síndrome: Apropos of a family

    Directory of Open Access Journals (Sweden)

    Gretsy Arcas Ermeso

    2005-06-01

    Full Text Available El síndrome branquio-oto-renal es un trastorno que se transmite con un patrón autosómico dominante caracterizado por lesiones producidas por un trastorno embriológico branquial (fístulas o quistes branquiales, alteraciones del oído (hoyuelos o fositas preauriculares, apéndices, hipoplasias de pabellones auriculares y sordera, así como diversos tipos de displasia renal que pueden ir desde duplicaciones, desplazamientos e hipoplasias, hasta la agenesia renal y otras malformaciones mayores. Se presenta una familia integrada por un padre y dos hijos de ambos sexos y de diferentes matrimonios, en la cual se constató la presencia de este síndrome poco frecuenteThe branchio-oto-renal syndrome is a disorder that is transmitted with a dominant autosomic pattern characterized y lesions produced by a branchial embriological disorder (fistulas or branchial cysts, alterations of the ear (preauricular small pits or fossae, appendices, hypoplasias of auricular pavilions and deafness, as well as diverse types of renal dysplasia that may go from duplications and displacements to renal agenesia and other major malformations. A family composed of a father and two children of both sexes from different marriages, in which the presence of this uncommon syndrome was confirmed, is presented

  8. An unusual case of Y-shaped right renal vein.

    Science.gov (United States)

    Lavy, M; Martin, L; Eouzan, D; Turco, C; Heyd, B; Mantion, G; Parratte, B; Tatu, L

    2015-01-01

    Vascular renal anomalies are frequent, multiple and well described and result from errors in vessel embryogenesis between the 6th and 10th week of gestation. Historically, variations are described in anatomic dissection and currently mostly in image interpretation. We report an anatomic variation concerning the right renal vein which, to our knowledge, has never been described in the literature either by dissection or by radiological examination. This variation was discovered during the routine dissection of an embalmed male body. It consists of a Y-shaped right renal vein and is associated with multiple retroperitoneal variations: a bilateral accessory renal artery, a trident ending of the right renal artery and a left testicular vein variation. Venous and arterial renal anatomy and its variations are fundamentally important in renal surgery, especially concerning living donor renal grafts. These variations may be diagnosed thanks to injected tomodensitometry which has a good sensitivity and specificity for anomalies. Preoperative diagnosis of an anatomic vascular renal variation may reduce morbidity during surgery, which is why precise examination of injected tomography should be mandatory.

  9. 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

  10. Congenital Nephrogenic Diabetes Insipidus Presented With Bilateral Hydronephrosis and Urinary Infection: A Case Report.

    Science.gov (United States)

    Zheng, Kewen; Xie, Yi; Li, Hanzhong

    2016-05-01

    Nephrogenic diabetes insipidus (NDI) is a condition resulting from the kidney's impaired response to circulating antidiuretic hormone (ADH), leading to polydipsia and polyuria. Urinary tract dilatation caused by NDI is a rare situation. Here, we report a case of congenital NDI presented with bilateral hydronephrosis.A 15-year-old boy complaining a history of intermittent fever was admitted to Peking Union Medical College Hospital. He voided 10 to 15 L of urine daily. Radiographic examination revealed severe dilatation of bilateral renal pelvis, ureter, and bladder. Urinalysis shows hyposthenuria.He was diagnosed NDI since born. Transient insertion of a urethral catheter helped to relieve fever. Medical therapy of hydrochlorothiazide and amiloride was prescribed and effective.Dilatation of urinary tract caused by diabetes insipidus is rare, but may be present in severe condition. Therefore, it is crucial for clinicians to perform early treatment to avoid impairment of renal function.

  11. Hydraulic bilateral construction robot; Yuatsushiki bilateral kensetsu robot

    Energy Technology Data Exchange (ETDEWEB)

    Maehata, K.; Mori, N. [Kayaba Industry Co. Ltd., Tokyo (Japan)

    1999-05-15

    Concerning a hydraulic bilateral construction robot, its system constitution, structures and functions of important components, and the results of some tests are explained, and the researches conducted at Gifu University are described. The construction robot in this report is a servo controlled system of a version developed from the mini-shovel now available in the market. It is equipped, in addition to an electrohydraulic servo control system, with various sensors for detecting the robot attitude, vibration, and load state, and with a camera for visualizing the surrounding landscape. It is also provided with a bilateral joy stick which is a remote control actuator capable of working sensation feedback and with a rocking unit that creates robot movements of rolling, pitching, and heaving. The construction robot discussed here, with output increased and response faster thanks to the employment of a hydraulic driving system for the aim of building a robot system superior in performance to the conventional model designed primarily for heavy duty, proves after tests to be a highly sophisticated remotely controlled robot control system. (NEDO)

  12. Bilateral cleft lip nasal deformity

    Directory of Open Access Journals (Sweden)

    Singh Arun

    2009-01-01

    Full Text Available Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity. Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention. There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth. This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it′s management both at the time of cleft lip repair

  13. TRANSPLANTE RENAL

    Directory of Open Access Journals (Sweden)

    Soraia Geraldo Rozza Lopes

    2014-01-01

    Full Text Available El objetivo del estudio fue comprender el significado de espera del trasplante renal para las mujeres en hemodiálisis. Se trata de un estudio cualitativo-interpretativo, realizado con 12 mujeres en hemodiálisis en Florianópolis. Los datos fueron recolectados a través de entrevistas en profundidad en el domicilio. Fue utilizado el software Etnografh 6.0 para la pre-codificación y posterior al análisis interpretativo emergieron dos categorías: “las sombras del momento actual”, que mostró que las dificultades iniciales de la enfermedad están presentes, pero las mujeres pueden hacer frente mejor a la enfermedad y el tratamiento. La segunda categoría, “la luz del trasplante renal”, muestra la esperanza impulsada por la entrada en la lista de espera para un trasplante.

  14. Neural regulation of the kidney function in rats with cisplatin induced renal failure

    Science.gov (United States)

    Goulding, Niamh E.; Johns, Edward J.

    2015-01-01

    Aim: Chronic kidney disease (CKD) is often associated with a disturbed cardiovascular homeostasis. This investigation explored the role of the renal innervation in mediating deranged baroreflex control of renal sympathetic nerve activity (RSNA) and renal excretory function in cisplatin-induced renal failure. Methods: Rats were either intact or bilaterally renally denervated 4 days prior to receiving cisplatin (5 mg/kg i.p.) and entered a chronic metabolic study for 8 days. At day 8, other groups of rats were prepared for acute measurement of RSNA or renal function with either intact or denervated kidneys. Results: Following the cisplatin challenge, creatinine clearance was 50% lower while fractional sodium excretion and renal cortical and medullary TGF-β1 concentrations were 3–4 fold higher in both intact and renally denervated rats compared to control rats. In cisplatin-treated rats, the maximal gain of the high-pressure baroreflex curve was only 20% that of control rats, but following renal denervation not different from that of renally denervated control rats. Volume expansion reduced RSNA by 50% in control and in cisplatin-treated rats but only following bilateral renal denervation. The volume expansion mediated natriuresis/diuresis was absent in the cisplatin-treated rats but was normalized following renal denervation. Conclusions: Cisplatin-induced renal injury impaired renal function and caused a sympatho-excitation with blunting of high and low pressure baroreflex regulation of RSNA, which was dependent on the renal innervation. It is suggested that in man with CKD there is a dysregulation of the neural control of the kidney mediated by its sensory innervation. PMID:26175693

  15. Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

    OpenAIRE

    Yun Luo; Ying Zhang; Shi-feng Lou

    2013-01-01

    We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the...

  16. Hydronephrosis Resulting from Bilateral Ureteral Stenosis: A Late Complication of Polyoma BK Virus Cystitis?

    Science.gov (United States)

    Basara, N; Rasche, F-M; Schwalenberg, T; Wickenhauser, C; Maier, M; Ivovic, J; Niederwieser, D; Lindner, T H

    2010-01-01

    We report here a case of acute lymphoblastic leukemia in remission presenting a late-onset bilateral hydronephrosis probably due to polyoma BK virus-induced proliferation of bladder endothelium on both ostii. The diagnosis was made virologically by BK virus Polymerase Chain Reaction (PCR) detection in the absence of any other bladder disease. Awareness of this late complication is necessary not only in patients after renal transplantation but also in patients after hematopoietic stem cell transplantation from matched unrelated donor.

  17. Hydronephrosis Resulting from Bilateral Ureteral Stenosis: A Late Complication of Polyoma BK Virus Cystitis?

    Science.gov (United States)

    Basara, N.; Rasche, F.-M.; Schwalenberg, T.; Wickenhauser, C.; Maier, M.; Ivovic, J.; Niederwieser, D.; Lindner, T. H.

    2010-01-01

    We report here a case of acute lymphoblastic leukemia in remission presenting a late-onset bilateral hydronephrosis probably due to polyoma BK virus-induced proliferation of bladder endothelium on both ostii. The diagnosis was made virologically by BK virus Polymerase Chain Reaction (PCR) detection in the absence of any other bladder disease. Awareness of this late complication is necessary not only in patients after renal transplantation but also in patients after hematopoietic stem cell transplantation from matched unrelated donor. PMID:20936157

  18. Hydronephrosis Resulting from Bilateral Ureteral Stenosis: A Late Complication of Polyoma BK Virus Cystitis?

    OpenAIRE

    Basara, N.; Rasche, F.-M.; Schwalenberg, T.; Wickenhauser, C.; Maier, M.; Ivovic, J.; Niederwieser, D.; Lindner, T. H.

    2010-01-01

    We report here a case of acute lymphoblastic leukemia in remission presenting a late-onset bilateral hydronephrosis probably due to polyoma BK virus-induced proliferation of bladder endothelium on both ostii. The diagnosis was made virologically by BK virus Polymerase Chain Reaction (PCR) detection in the absence of any other bladder disease. Awareness of this late complication is necessary not only in patients after renal transplantation but also in patients after hematopoietic stem cell tra...

  19. Bilateral Malrotation and a Congenital Pelvic Kidney with Varied Vasculature and Altered Hilar Anatomy

    OpenAIRE

    Singh, J.; Singh, N.; Kapoor, K.; Sharma, M.

    2015-01-01

    Variations of structure and position of the kidney along with variations of renal vessels are most frequently reported. Rotational variations form a rare entity that are not cited in most embryology textbooks. During an educational cadaveric dissection of a 42-year-old male, a complex picture of bilateral anatomical variants was encountered. Malrotation of both kidneys and a left lobulated ectopic kidney along with open hilum was observed. The left kidney showed a pelvic position in front of...

  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. [Orthotopic renal transplant: our experience].

    Science.gov (United States)

    De Gracia, R; Jiménez, C; Gil, F; Escuin, F; Tabernero, A; Sanz, A; Hidalgo, L

    2007-01-01

    Orthotopic renal transplant (ORT) is useful in cases of severe atherosclerosis, heterotopic bilateral transplant, unsuitable pelvic vessels and in aortic thrombosis, but it is not available in all the institutions and it is only realized of exceptional form. To review the indication, surgical technique and outcome of the ORT at our hospital. The studied included five cases between January 1990 and December 2005. We analyzed several variables: demographic characteristics, characteristics of the donor, ischemia times, evolution of renal function and morbi-mortality associated. Left ORT was performed in three men and two women. Mean patient age was 52+/-5 years, all the patients received kidneys from cadaveric donors. Mean creatinine and urea one month postoperative were 2.2+/-0.72 mg/dl and 103+/-17.2 mg/dl and at 6 months postoperative were 1.8+/-0.59 mg/dl and 78+/-14 mg/dl respectively. Immediately all patients received prophylaxis with low molecular weight heparin but it was indicated antiaggregation to two patients when they left the hospital, anticoagulation to two patients and to one of them was decided to anticoagulation nor antiagregation for history of bled digestive. A patient died for bleeding episode at level of the renal graft six months after the transplant, she was in treatment with dicumarinics, they were indicated by venous deep thrombosis in right leg. The survival a year is 80 % of the graft and the patient. Only two patients returned to hospital later, one of them for presenting an episode of diverticulitis and the other one for renal obstructive failure that needed laying of catheter pig-tail. Four patients presented stenosis of renal native vassels detected in control magnetic nuclear resonance, not symptomatic. There are two patients who take more than three years transplanted with renal stable function (creatinina 1.3 mg/dl and 1.4 mg/dl respectively). ORT is an excellent option in patients with co-morbidity increased for atherosclerosis and

  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. Device-based approaches for renal nerve ablation for hypertension and beyond.

    Science.gov (United States)

    Thorp, Alicia A; Schlaich, Markus P

    2015-01-01

    Animal and human studies have demonstrated that chronic activation of renal sympathetic nerves is critical in the pathogenesis and perpetuation of treatment-resistant hypertension. Bilateral renal denervation has emerged as a safe and effective, non-pharmacological treatment for resistant hypertension that involves the selective ablation of efferent and afferent renal nerves to lower blood pressure. However, the most recent and largest randomized controlled trial failed to confirm the primacy of renal denervation over a sham procedure, prompting widespread re-evaluation of the therapy's efficacy. Disrupting renal afferent sympathetic signaling to the hypothalamus with renal denervation lowers central sympathetic tone, which has the potential to confer additional clinical benefits beyond blood pressure control. Specifically, there has been substantial interest in the use of renal denervation as either a primary or adjunct therapy in pathological conditions characterized by central sympathetic overactivity such as renal disease, heart failure and metabolic-associated disorders. Recent findings from pre-clinical and proof-of-concept studies appear promising with renal denervation shown to confer cardiovascular and metabolic benefits, largely independent of changes in blood pressure. This review explores the pathological rationale for targeting sympathetic renal nerves for blood pressure control. Latest developments in renal nerve ablation modalities designed to improve procedural success are discussed along with prospective findings on the efficacy of renal denervation to lower blood pressure in treatment-resistant hypertensive patients. Preliminary evidence in support of renal denervation as a possible therapeutic option in disease states characterized by central sympathetic overactivity is also presented.

  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. 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.

  8. Severe bilateral adrenal hemorrhages in  a  newborn complicated by persistent adrenal insufficiency

    Directory of Open Access Journals (Sweden)

    Nicholas R Zessis

    2018-02-01

    Full Text Available Bilateral adrenal hemorrhages rarely occur during the neonatal period and are often associated with traumatic vaginal deliveries. However, the adrenal gland has highly regenerative capabilities and adrenal insufficiency typically resolves over time. We evaluated a newborn female after experiencing fetal macrosomia and a traumatic vaginal delivery. She developed acidosis and acute renal injury. Large adrenal hemorrhages were noted bilaterally on ultrasound, and she was diagnosed with adrenal insufficiency based on characteristic electrolyte changes and a low cortisol (4.2 μg/dL. On follow-up testing, this patient was unable to be weaned off of hydrocortisone or fludrocortisone despite resolution of hemorrhages on ultrasound. Providers should consider bilateral adrenal hemorrhage when evaluating critically ill neonates after a traumatic delivery. In extreme cases, this may be a persistent process.

  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. 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.

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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.

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

    Science.gov (United States)

    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.

  18. 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.

  19. 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.

  20. 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.

  1. 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.

  2. Bilateral Testicular Infarction from IgA Vasculitis of the Spermatic Cords

    Directory of Open Access Journals (Sweden)

    Mazen Toushan

    2017-01-01

    Full Text Available A 51-year-old man with type 2 diabetes mellitus and chronic obstructive pulmonary disease presented to the emergency room with increasing bilateral leg pain, rash, and scrotal swelling with pain. Skin biopsy from his thigh revealed IgA-associated vasculitis. Due to hematuria, a renal biopsy was performed and showed an IgA glomerulonephritis with focal fibrinoid necrosis and neutrophil accumulation. Bilateral orchiectomies were performed in two separate procedures ten and thirteen days after the renal biopsy, as a result of uncontrolled abscess formation in testicles. Microscopically, both testicles revealed large abscess formation destroying almost the entire testicular parenchyma without tumor cells. Spermatic cord margins were further scrutinized microscopically to show bilateral vasculitis in many small size vessels, confirmed by positive endothelial staining for IgA. Some of the affected arteries revealed central organizing thrombi with recanalization features, highly suggestive of vasculitis-associated thrombi formation, resulting in testicular ischemic infarction and abscess formation. We conclude that this adult patient developed a severe form of Henoch-Schönlein purpura, with vasculitis affecting multiple organs, including the most serious and unusual complication of bilateral testicular infarction.

  3. Malignant renal tumors in pediatrics

    International Nuclear Information System (INIS)

    Pena, C.; Torterolo, J.; Irigoyen, B.; Bel, M.; Elias, E.

    2004-01-01

    Introduction: Professionals who work in pediatric oncology, we see childhood cancer as a common disease, but in fact constitutes about 2% of all cancers diagnosed worldwide. Wilms tumor accounts for 6% of all childhood tumors and presentation bilateral accounts for 4-6% of all Wilms tumors diagnosed. Theoretical Framework: In the period between the year 1994-2003 period were attended in the Pediatric Hematology-Oncology Center, a total of 29 cases of malignant renal tumors, corresponding to 86% (25 cases) to Wilms tumor or nephroblastoma tumor. The Wilms is of embryonic origin, capable of metastatic spread, (85% lungs 15% liver). Very sensitive to chemotherapy and radiotherapy, which confers high cure rates (85%); having a multidisciplinary treatment model, combining surgery, chemotherapy, and radiotherapy. The role of nursing in comprehensive cancer care child is essential in the prevention and early detection of side effects or complications. Case report: S.D. currently 10 years old. In 10/1994, at 8 months of age, was diagnosed with bilateral Wilms tumor. On admission her weight was 8200gr with abdominal circumference 50cm. Conducted pre-operative MDT and 02/1995 nephrectomy of the left kidney and right kidney lumpectomy (tumor nodule 420gr. and a 250gr.). MDT begins in 03/1995 01/1996 ending. 09/2003 with abdominal pain and vomiting, and kidney failure. 10/2003 lumpectomy biopsy (sclerotic nodule associated with maturation nephroblastoma). Currently severe renal insufficiency plan enters dialysis. Nursing process: Objectives: 1) To prepare the child and family to the side effects and possible complications of chemotherapy and / or radiotherapy 2) Prevent and minimize related complications tumor and / or treatment. Care Plan comprises four stages: A) rating and customer income. B) Implement care chemotherapy C) post-operative Care D) Implement radiation care

  4. Prevalence of dental anomalies in children with cleft lip and unilateral and bilateral cleft lip and palate.

    Science.gov (United States)

    Rullo, R; Festa, V M; Rullo, R; Addabbo, F; Chiodini, P; Vitale, M; Perillo, L

    2015-09-01

    To examine the prevalence of different types of dental anomalies in children with nonsyndromic cleft lip, unilateral cleft lip-palate, and bilateral cleft lip-palate. A sample of 90 patients (aged 4-20 years) affected by isolated cleft lip, unilateral and bilateral cleft lip and palate was examined. Cleft patients were classified into one of three groups according to cleft type: (1) Unilateral Cleft Lip-Palate, (2) Bilateral Cleft Lip-Palate, and (3) Cleft Lip. Intraoral exams, panoramic radiographs and dental casts, were used to analyse the prevalence of the various dental anomalies included in this study. There were no statistically significant differences between patients with cleft lip, unilateral cleft lip and palate and bilateral cleft lip and palate. The congenital absence of the cleft-side lateral incisor was observed in 40% of the sample, and a total of 30% patients showed supernumerary teeth at the incisors region. Second premolar agenesis was found in 4.4% of patients, whereas in 18.9% of the sample there was an ectopic dental eruption. Lateral or central incisors rotation was noted in 31.1% of the sample, while shape anomaly, lateral incisor microdontia, and enamel hypoplasia were detected respectively in 25.6%, 5.6% and 18.9% of cleft patients. High prevalence of different dental anomalies in children with cleft lip and unilateral and bilateral cleft lip and palate has been confirmed. This study, in particular, shows the presence of ectopic and rotated teeth in the cleft area.

  5. Prenatal diagnosis of bilateral anophthalmia by 3D "reverse face" view ultrasound and magnetic resonance imaging.

    Science.gov (United States)

    Araujo Júnior, Edward; Kawanami, Tatiana Emy; Nardozza, Luciano Marcondes Machado; Milani, Hérbene José Figuinha; Oliveira, Patrícia Soares; Moron, Antonio Fernandes

    2012-12-01

    Primary anophthalmia is a rare congenital malformation that affects 0.6/10,000 liveborn infants. It is usually associated with central nervous system malformations, aneuploidies, cytomegalovirus infection and mental retardation and it can also be part of genetic conditions such as Fraser, Goltz, Goldenhar, Waardenburg and Lenz syndromes. Neonatal prognosis depends on whether anophthalmia is an isolated malformation, or it is associated with other defects or part of a syndrome. A healthy 43-year-old woman, G4 P3 with three previous healthy children, was referred to our clinic for a routine obstetric ultrasound at 28 weeks' gestation. The fetal eye globes and lenses could not be seen on two-dimensional (2D) ultrasound, which led to the diagnosis of bilateral congenital anophthalmia. No other fetal malformations were detected. At 30 weeks' gestation, a three-dimensional (3D) ultrasound was performed using the rendering mode and "reverse face" view. Using this technique, the absence of both eye globes could be clearly seen through a "slit". 3D-ultrasound allowed the parents to better understand their child's problem and possible postnatal implications. Fetal magnetic resonance imaging (MRI) was also performed, to study the fetal cortex in more detail. This exam revealed right cerebral hemisphere sulci and gyri hypoplasia. At 41 1/7 weeks, she went into spontaneous labor and delivered vaginally a 3525 g male infant with Apgar scores of 9 and 10. Postnatal exams confirmed bilateral congenital anophthalmia. This is the first case report in the literature of prenatal diagnosis of bilateral anophthalmia using 3D "reverse face" view ultrasound and MRI. Copyright © 2012. Published by Elsevier B.V.

  6. Bilateral absence of musculocutaneous nerve

    Directory of Open Access Journals (Sweden)

    Mathada V Ravishankar

    2012-01-01

    Full Text Available Brachial plexus is an important group of spinal nerve plexus that supplies the muscles of the upper limb via the ventral rami of the Cervical 5 - Thoracic 1 fibers of the spinal nerves. It is not uncommon to notice the variations during cadaveric dissections in many regions of the body, at different levels, such as, roots, trunks, division, cords, communications, and branches as reported in the literature. Although the nerve supply of the body musculature takes place in the fetal life itself, its course, branching pattern, innervations, and communication can show variable patterns as the fetal development progresses. One such anomaly was noticed during our routine cadaveric dissection in the Department of Anatomy, Jawaharlal Nehru Medical College, Belgaum, showing bilateral absence of the musculocutaneous nerve, which obviously drew the attention of the students of medicine, physiotherapy, and learning clinicians as well.

  7. Bilateral supernumerary primary maxillary canines

    Directory of Open Access Journals (Sweden)

    Santanu Mukhopadhyay

    2018-01-01

    Full Text Available Supernumerary teeth are more common in the permanent than in primary dentition. In the primary dentition, the anomaly is most frequently observed in the maxillary lateral incisor region, followed by the maxillary midline where they are termed as mesiodens. Supernumerary teeth in the primary canine region are rare. This paper describes a rare case of nonsyndromic supernumerary primary maxillary canine distributed bilaterally in a 4-year-old boy. Both the supernumeraries resembled size and shape of normal primary canine. The right supplemental canine is high labially placed, whereas the left one is seen normally aligned in the dental arch distal to lateral incisor. One of the most significant sequelae of primary supernumerary teeth is their duplication in the permanent series. Radiographic examination of supernumerary primary canine did not indicate any such anomaly in the permanent dentition. The patient was kept under observation.

  8. Renal nerves dynamically regulate renal blood flow in conscious, healthy rabbits.

    Science.gov (United States)

    Schiller, Alicia M; Pellegrino, Peter R; Zucker, Irving H

    2016-01-15

    Despite significant clinical interest in renal denervation as a therapy, the role of the renal nerves in the physiological regulation of renal blood flow (RBF) remains debated. We hypothesized that the renal nerves physiologically regulate beat-to-beat RBF variability (RBFV). This was tested in chronically instrumented, healthy rabbits that underwent either bilateral surgical renal denervation (DDNx) or a sham denervation procedure (INV). Artifact-free segments of RBF and arterial pressure (AP) from calmly resting, conscious rabbits were used to extract RBFV and AP variability for time-domain, frequency-domain, and nonlinear analysis. Whereas steady-state measures of RBF, AP, and heart rate did not statistically differ between groups, DDNx rabbits had greater RBFV than INV rabbits. AP-RBF transfer function analysis showed greater admittance gain in DDNx rabbits than in INV rabbits, particularly in the low-frequency (LF) range where systemic sympathetic vasomotion gives rise to AP oscillations. In the LF range, INV rabbits exhibited a negative AP-RBF phase shift and low coherence, consistent with the presence of an active control system. Neither of these features were present in the LF range of DDNx rabbits, which showed no phase shift and high coherence, consistent with a passive, Ohm's law pressure-flow relationship. Renal denervation did not significantly affect nonlinear RBFV measures of chaos, self-affinity, or complexity, nor did it significantly affect glomerular filtration rate or extracellular fluid volume. Cumulatively, these data suggest that the renal nerves mediate LF renal sympathetic vasomotion, which buffers RBF from LF AP oscillations in conscious, healthy rabbits. Copyright © 2016 the American Physiological Society.

  9. Cigarette smoking and cardio-renal events in patients with atherosclerotic renal artery stenosis.

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    Christopher A Drummond

    Full Text Available Cigarette smoking causes cardiovascular disease and is associated with poor kidney function in individuals with diabetes mellitus and primary kidney diseases. However, the association of smoking on patients with atherosclerotic renal artery stenosis has not been studied. The current study utilized data from the Cardiovascular Outcomes in Renal Atherosclerotic Lesions (CORAL, NCT00081731 clinical trial to evaluate the effects of smoking on the risk of cardio-renal events and kidney function in this population. Baseline data showed that smokers (n = 277 out of 931 were significantly younger at enrollment than non-smokers (63.3±9.1 years vs 72.4±7.8 years; p<0.001. In addition, patients who smoke were also more likely to have bilateral renal artery stenoses and peripheral vascular disease (PVD. Longitudinal analysis showed that smokers experienced composite endpoint events (defined as first occurrence of: stroke; cardiovascular or renal death; myocardial infarction; hospitalization for congestive heart failure; permanent renal replacement; and progressive renal insufficiency defined as 30% reduction of GFR from baseline sustained for ≥ 60 days at a substantially younger age compared to non-smokers (67.1±9.0 versus 76.1±7.9, p<0.001. Using linear regression and generalized linear modeling analysis controlled by age, sex, and ethnicity, smokers had significantly higher cystatin C levels (1.3±0.7 vs 1.2±0.9, p<0.01 whereas creatinine and estimated glomerular filtration rate (eGFR were not different from non-smokers. From these data we conclude that smoking has a significant association with deleterious cardio-renal outcomes in patients with renovascular hypertension.

  10. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    Directory of Open Access Journals (Sweden)

    Gultekin Gulbahar

    2015-01-01

    Full Text Available First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  11. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    OpenAIRE

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare en...

  12. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures.

    Science.gov (United States)

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  13. Endovascular treatment of renal artery stenoses

    International Nuclear Information System (INIS)

    Lashari, M.N.

    2015-01-01

    To evaluate the procedure success and effect on hypertension after stenting of incidentally diagnosed atherosclerotic renal artery stenoses. Study Design: An experimental study. Place and Duration of Study: A multicentric study was conducted at the Plastic Surgery and General Hospital, National Medical Center and Ziauddin University Hospital, Karachi, Pakistan from January 2009 to March 2013. Methodology: Hypertension (systolic blood pressure > 160 and diastolic > 90 mmHg with two or more than two medications) with coronary artery disease were initially evaluated for coronary angiography, Renal artery angiography was also endovascular performed and stent was deployed for atherosclerotic renal artery stenosis when found. Blood pressure readings, reduction in need of antihypertensive medication and serum creatinine levels were taken as outcome measures. Patients having renal artery stenoses secondary to connective tissue disorders and fibromuscular dysplasia were excluded. Results: There were 25 patients, 14 (56%) male and 11 (44%) female, with mean age of 49 +- 6 years. Diabetes mellitus, dyslipidemia and smoking were seen in 11 (44%), 10 (40%) and 4 (16%) patients respectively. Renal insufficiency (serum creatinine > 1.5 mg/dl) was seen in one (04%) patient. Bilateral, and isolated right and left renal artery stenoses was seen in 5 (20%), 9 (36%) and 11(44%) patients respectively. Mean percentage of renal artery stenoses was 89%, ranged from 70% to 99% while ostial lesion was found in 20 (80%) patients. A significant decrease in systolic (168.20 +- 9.987 vs. 140.60 +- 5.649 mmHg, p < 0.001) and diastolic blood pressure (88.60 +- 5.50 vs. 77.20 +- 5.017 mmHg, p < 0.001) and reduction of medication (2.72 +- 0.458 vs. 1.5 +- 0.510, p < 0.01) were noted without a change in renal function (p= 0.061) after renal artery stenting. Conclusion: Endovascular stenting of renal artery stenoses in patients with poorly controlled hypertension is a safe and effective treatment

  14. Renal dysplasia and MRI: a clinician's perspective

    Energy Technology Data Exchange (ETDEWEB)

    Greenbaum, Larry A. [Emory University, Division of Pediatric Nephrology, Children' s Healthcare of Atlanta, Atlanta, GA (United States)

    2008-01-15

    Renal dysplasia is a common abnormality in children. The role of MRI in evaluating children with renal dysplasia is evolving. More information is clearly necessary before MRI replaces conventional imaging modalities. In order to appropriately use MRI, the radiologist must have an understanding of the clinical questions that are important in the management of children with renal dysplasia. This review provides background information on renal dysplasia for the pediatric radiologist. The focus is on unilateral disease, especially multicystic dysplastic kidneys, and bilateral dysplasia, which is the most common cause of kidney failure in children. The emphasis is on the important clinical issues, and the potential of MRI as a methodology for providing clinically useful information not otherwise available from other imaging modalities. (orig.)

  15. 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.

  16. Radionuclide evaluation of renal transplants

    International Nuclear Information System (INIS)

    Yang Hong; Zhao Deshan

    2000-01-01

    Radionuclide renal imaging and plasma clearance methods can quickly quantitate renal blood flow and function in renal transplants. They can diagnose acute tubular necrosis and rejection, renal scar, surgical complications such as urine leaks, obstruction and renal artery stenosis after renal transplants. At the same time they can assess the therapy effect of renal transplant complications and can also predict renal transplant survival from early post-operative function studies

  17. Bilateral Ischemic Optic Neuropathy Developed under Interferon Therapy

    Directory of Open Access Journals (Sweden)

    Fatih Selcukbiricik

    2012-01-01

    Full Text Available Introduction. Interferon is a glycoprotein produced by assigned cells of immune system. It has been used in many different diseases. Although flu-like syndrome, myalgia, rash, hypotension, thrombocytopenia and peripheral neuropathy due to interferon use are encountered frequently, ocular side effects are rare, generally mild and transient. Case Report. 47-year-old female patient, presented with a mass lesion in right renal pelvis. Right radical nephrectomy was applied and the histopathological examination was consistent with papillary renal cell carcinoma. Interferon alpha treatment was started subcutaneously at the dose of 5 MIU/3 times in a week. Four weeks after the interferon therapy, suddenly bilateral visual loss developed. We discussed the diagnosis, followup, and treatment of the patient who developed irreversible ischemic optic neuropathy and had no previous known primary systemic disease to cause this condition. Conclusion. We suggest that patients should be screened for risk factors causing optic ischemic neuropathy, before interferon therapy. Although there was no adequate information in the literature for the followup, patients should be monitorized before, 1 month after, and 2 months after the treatment. And if there is no complication, we suggest that they should be followed up at 3-month intervals.

  18. Bilateral Simultaneous Macular Infarction with Spontaneous Visual ...

    African Journals Online (AJOL)

    To report the rare and dramatic event of bilateral macular infarction in a sickle cell hemoglobinopathy (SS genotype) patient, resulting in bilateral severe reduction in visual acuity. Without any intervention, the patient's vision gradually improved over the follow‑up period. Central visual field defects however persisted.

  19. Infectious mononucleosis presenting as bilateral acute dacryocystitis.

    Science.gov (United States)

    Atkinson, P L; Ansons, A M; Patterson, A

    1990-01-01

    A case of infectious mononucleosis presenting as bilateral acute dacryocystitis in a 7-year-old girl is reported. Acute dacryocystitis is uncommon in this age group, and an underlying systemic illness should be suspected particularly when it is bilateral. Images PMID:2275940

  20. Bilateral elastofibroma dorsi: A case report

    OpenAIRE

    Molini, L.; Ciortan, E.; Bianchi, S.

    2010-01-01

    Elastofibroma dorsi is a rare pseudotumoral lesion located in the periscapular region. Ultrasound can be used to evaluate its dimensions, margins, and internal structure. In the presence of bilateralism, diagnosis can be made on the basis of clinical and sonographic findings. The authors present a case of bilateral elastofibroma dorsi in which the clinical and ultrasound diagnosis was confirmed by magnetic resonance imaging.

  1. Bilateral locked facets in the thoracic spine

    NARCIS (Netherlands)

    M.H.A. Willems; Braakman, R. (Reinder); B. van Linge (Bert)

    1984-01-01

    textabstractTwo cases of traumatic bilateral locked facets in the thoracic spine are reported. Both patients had only minor neurological signs. They both made a full neurological recovery after surgical reduction of the locked facets. Bilateral locked facets are very uncommon in the thoracic spine.

  2. Bilateral spontaneous adrenal haemorrhage complicating acute pancreatitis

    International Nuclear Information System (INIS)

    Pianta, M.; Varma, D. K.

    2007-01-01

    Bilateral adrenal haemorrhage is an event that mandates prompt diagnosis and treatment to prevent primary adrenocortical insufficiency and potential death. Presentation can be non-specific and incidentally diagnosed with imaging alone, primarily CT. We present a case of acute pancreatitis with spontaneous bilateral adrenal haemorrhage and briefly discuss imaging and treatment implications

  3. A case of bilateral trench foot.

    Science.gov (United States)

    Parsons, S L; Leach, I H; Charnley, R M

    1993-12-01

    A case of severe bilateral trench foot is presented in a patient who lived rough for 3 weeks without removing his boots. Non-operative management yielded no clinical improvement and bilateral below-knee amputation was necessary. Histology revealed subcutaneous and muscle necrosis with secondary arterial thrombosis.

  4. Pielonefritis enfisematosa aguda bilateral: Un desafío terapéutico Acute bilateral emphysematous pyelonephritis: A therapeutic challenge

    Directory of Open Access Journals (Sweden)

    Marcelo J. Melero

    2007-06-01

    Full Text Available La pielonefritis enfisematosa es una forma poco común de infección renal, caracterizada por la presencia de bacterias coliformes productoras de gas que afecta preferentemente a los pacientes diabéticos. Comunicamos el caso de una mujer diabética de 57 años de edad que ingresó en el hospital por un shock séptico, signos de pielonefritis enfisematosa aguda bilateral y cetoacidosis diabética. En los cultivos de las muestras de orina y sangre desarrolló Escherichia coli. La paciente fue tratada exitosamente con antibióticos de amplio espectro por un tiempo prolongado, control diabético y medidas de sostén solamente. No fue necesario el drenaje con catéteres o la nefrectomía para superar esta situación potencialmente letal.Emphysematous pyelonephritis is a rare form of kidney infection characterized by the presence of gas-forming coliform bacteria which affects more frequently diabetic subjects. We report the case of a 57-years-old diabetic woman, who was admitted in septic shock, signs of acute bilateral emphysematous pyelonephritis, and diabetic ketoacidosis. Both blood and urine cultures yielded Escherichia coli. The patient was successfully treated using long-term broad-spectrum antibiotics, diabetic control and supportive measures alone. Catheter drainage and nephrectomy were not necessary to overcome this life threatening situation.

  5. An interesting case of renal amyloidosis

    Directory of Open Access Journals (Sweden)

    A Hajra

    2016-01-01

    Full Text Available In amyloidosis, there is an extracellular deposition of beta-sheet fibrils. Over 25 proteins have been demonstrated to form amyloid. One of them is Ig amyloid light (AL chains. We are presenting a 40-year-old female who presented with progressive kyphoscoliosis for last 2 years following a minor trauma and bilateral pedal edema for last 3 months. On further investigation, we found that she had a biclonal variety of MM with amyloidosis of kidney leading to massive proteinuria. Very few case reports are available where patient with biclonal variety of MM develop renal amyloidosis.

  6. POSSIBILITIES OF ORGAN-PRESERVING TREATMENT OF PATIENTS WITH MULTIPLE RENAL TUMORS

    Directory of Open Access Journals (Sweden)

    B. Ya. Alekseev

    2017-01-01

    Full Text Available Renal cell carcinoma (RCC occupies one of the leading places in the world for morbidity among malignant neoplasms of the genitourinary system. The frequency of occurrence of bilateral RCC according to different authors is 2–6% of the total population of patients with RCC. Currently, the only effective method of treatment of bilateral RCC is surgical treatment. Patients with bilateral RCC are at high risk of dev eloping of local recurrence or progression of the disease after organ-preserving surgeries, which is why the surgeon is faced with a choice between a high risk of developing renal failure or relapse and/or progression of the disease, depending on the extent of the surgical intervention. According to the literature, in patients with bilateral RCC there was an increase in the incidence of papillary variant of RCC up to 19% and the presence of multifocal lesion. Surgical treatment of bilateral RCC is the only effective method to achieve satisfactory oncological results at a low incidence of complications. The m ost justified option for the treatment of bilateral RCC is the implementation of bilateral organ-preserving treatment, which allows achieving the optimal functional results. This article presents a clinical case of successful surgical treatment of a patient with bilateral RCC with multiple tumors.

  7. Distal renal tubular acidosis

    Science.gov (United States)

    ... this disorder. Alternative Names Renal tubular acidosis - distal; Renal tubular acidosis type I; Type I RTA; RTA - distal; Classical RTA Images Kidney anatomy Kidney - blood and urine flow References Bose A, Monk RD, Bushinsky DA. Kidney ...

  8. Renal origin of rat urinary epidermal growth factor

    DEFF Research Database (Denmark)

    Nexø, Ebba; Poulsen, Steen Seier

    1984-01-01

    The origin of rat urinary epidermal growth factor (EGF) has been investigated. Unilateral nephrectomy decreased the concentration, total output of EGF and EGF/creatinine ratio by approximately 50%, while the output of creatinine was unchanged. Removal of the submandibular glands and duodenal...... Brunner's glands, organs known to produce EGF, had no influence on the output of EGF in urine. Renal clearance of EGF exceeded that of creatinine, and after bilateral nephrectomy or bilateral ligation of the ureters, the concentration of creatinine in serum increased, while the concentration of EGF...

  9. Bilateral Internal Mammary Artery Utilization in Diabetics: Friend or Foe?

    Science.gov (United States)

    Crawford, Todd C; Zhou, Xun; Fraser, Charles D; Magruder, J Trent; Suarez-Pierre, Alejandro; Alejo, Diane; Bobbitt, Jennifer; Fonner, Clifford E; Wehberg, Kurt; Taylor, Brad; Kwon, Christopher; Fiocco, Michael; Conte, John V; Salenger, Rawn; Whitman, Glenn J

    2018-05-11

    Bilateral internal mammary artery (BIMA) grafting in diabetic patients undergoing coronary artery bypass grafting (CABG) remains controversial. Our study compared morbidity and mortality between 1)diabetic and non-diabetic BIMA patients and 2)diabetic BIMA versus diabetic patients who underwent left internal mammary artery (LIMA) grafting only. Patients who underwent isolated CABG from July 2011 - June 2016 at any of the 10 centers in Maryland were propensity scored across 16 variables. Diabetic BIMA were matched 1:1 by nearest neighbor matching to non-diabetic BIMA patients, and were separately matched 1:1 to diabetic LIMA patients. We calculated observed to expected (O/E) ratios for composite morbidity/mortality, operative mortality, unplanned reoperation, stroke, renal failure, prolonged ventilation, and deep sternal wound infection) and compared ratios among matched populations. Over the study period, 812 CABG patients received BIMA grafts, including 302 patients (37%) with diabetes. We matched 259 diabetic and non-diabetic BIMA patients. O/E ratios were higher in matched diabetic (vs non-diabetic) BIMA patients when comparing composite morbidity/mortality, reoperation, stroke, renal failure, and prolonged ventilation (all O/E>1.0), however, O/E for operative mortality was higher in non-diabetic BIMA patients. We additionally matched 292 diabetic BIMA to diabetic LIMA patients. Diabetic BIMA patients had a higher O/E for composite morbidity/mortality, operative mortality, stroke, renal failure, and prolonged ventilation. In this statewide analysis, diabetics who received BIMA grafts (compared to diabetics with LIMA grafts or non-diabetics with BIMA grafts) had higher O/E ratios for composite morbidity/mortality as a result of higher O/E ratios for major complications. Copyright © 2018. Published by Elsevier Inc.

  10. Cardio-renal syndrome

    OpenAIRE

    Gnanaraj, Joseph; Radhakrishnan, Jai

    2016-01-01

    Cardio-renal syndrome is a commonly encountered problem in clinical practice. Its pathogenesis is not fully understood. The purpose of this article is to highlight the interaction between the cardiovascular system and the renal system and how their interaction results in the complex syndrome of cardio-renal dysfunction. Additionally, we outline the available therapeutic strategies to manage this complex syndrome.

  11. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

    Heerwagen, S T; Jensen, C; Bagi, P

    2007-01-01

    Renal inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor of controversial etiology with a potential for local recurrence after incomplete surgical resection. The radiological findings in renal IMT are not well described. We report two cases in adults with a renal mass treated...

  12. 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

  13. Bilateral bone conduction devices: improved hearing ability in children with bilateral conductive hearing loss.

    Science.gov (United States)

    Dun, Catharina A J; Agterberg, Martijn J H; Cremers, Cor W R J; Hol, Myrthe K S; Snik, Ad F M

    2013-01-01

    The aim of the study was to investigate whether children with bilateral conductive hearing loss benefit from their second device (i.e., the bilateral bone conduction device [BCD]). Speech recognition in noise was assessed in 10 children fitted with bilateral BCDs during childhood. Speech recognition was measured in 2 conditions with both BCDs active. Spatial resolution was tested with the Minimum Audible Angle test in the bilateral and monaural listening conditions. Children demonstrated an improvement in speech recognition when speech was presented from the front and noise was presented from the right-hand side as compared with both speech and noise being presented from the front. The minimum audible angle decreased from 57° in the best monaural condition to 13° in the bilateral condition. The audiological outcomes demonstrate the advantage of bilateral BCD fitting in children with bilateral conductive hearing loss.

  14. Renal Metastasis from Primary Cervical Cancer: A Case Report

    International Nuclear Information System (INIS)

    Jeon, Seong Woo; Kim, See Hyung; Kwon, Sun Young

    2013-01-01

    Metastasis of malignant tumors to the kidney is clinically rare and often discovered by autopsy. Primary lymphoma and lung cancer are known that can metastasize to the kidney. Other malignant tumor metastasis to the kidney is very unusual. Primary cervical cancer metastasis to adjacent pelvic organs and lymph nodes are well known followed by abdominal solid organs such as the liver and adrenal glands. However, reported primary cervical cancer metastasis to the kidney is extremely rare and mostly appeared as bilateral multiple renal masses. We report here on a rare case of unilateral single renal metastasis from primary cervical cancer after concur- rent chemoradiotherapy.

  15. 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.

  16. 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.

  17. 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.

  18. 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

  19. Exercise renogram. A new approach documents renal involvement in systemic hypertension

    International Nuclear Information System (INIS)

    Clorius, J.H.; Schmidlin, P.

    1983-01-01

    Hippurate functional scintiscans were obtained in 51 hypertensive patients and in 15 controls. The authors investigated the influence that posture and exercise have on hippurate kinetics in patients with hypertension. A posture- or exercise-induced disturbance of renal hippurate transport was sought. All persons were examined in prone and standing positions, as well as during exercise. When prone and upright renograms were compared, 24% of the hypertensives demonstrated bilateral orthostatic renal dysfunction. Exercise caused the hippurate transport disturbance to increase. Fifty-seven percent of all hypertensives developed evidence of marked, bilateral, renal dysfunction during ergometric stress, so that exercise renography was shown to be a more sensitive test of the presence of transient tubular dysfunction in hypertension than the standing renogram. In normotensive controls the hippurate functional scintigram failed to be influenced by posture and exercise. The results suggest presence in hypertension of transient, posture- and exercise-mediated alterations of renal cortical blood flow

  20. The exercise renogram. A new approach documents renal involvement in systemic hypertension

    International Nuclear Information System (INIS)

    Clorius, J.H.; Schmidlin, P.

    1983-01-01

    Hippurate functional scintiscans were obtained in 51 hypertensive patients and in 15 controls. We investigated the influence that posture and exercise have on hippurate kinetics in patients with hypertension. A posture- or exercise-induced disturbance of renal hippurate transport was sought. All persons were examined in prone and standing positions, as well as during exercise. When prone and upright renograms were compared, 24% of the hypertensives demonstrated bilateral orthostatic renal dysfunction. Exercise caused the hippurate transport disturbance to increase. Fifty-seven percent of all hypertensives developed evidence of marked, bilateral, renal dysfunction during ergometric stress, so that exercise renography was shown to be a more sensitive test of the presence of transient tubular dysfunction in hypertension than the standing renogram. In normotensive controls the hippurate functional scintigram failed to be influenced by posture and exercise. The results suggest presence in hypertension of transient, posture- and exercise-mediated alterations of renal cortical blood flow

  1. Diffuse Lymphomatous Infiltration of Kidney Presenting as Renal Tubular Acidosis and Hypokalemic Paralysis: Case Report

    Science.gov (United States)

    Jhamb, Rajat; Gupta, Naresh; Garg, Sandeep; Kumar, Sachin; Gulati, Sameer; Mishra, Deepak; Beniwal, Pankaj

    2007-01-01

    We report the case of a 22-year-old woman who presented with acute onset flaccid quadriparesis. Physical examination showed mild pallor with cervical and axillary lymphadenopathy, hepatomegaly, and bilateral smooth enlarged kidneys. Neurological examination revealed lower motor neuron muscle weakness in all the four limbs with hyporeflexia and normal sensory examination. Laboratory investigations showed anemia, severe hypokalemia, and metabolic acidosis. Urinalysis showed a specific gravity of 1.010, pH of 7.0, with a positive urine anion gap. Ultrasound revealed hepatosplenomegaly with bilateral enlarged smooth kidneys. Renal biopsy was consistent with the diagnosis of non-Hodgkin lymphoma (B cell type). Metabolic acidosis, alkaline urine, and severe hypokalemia due to excessive urinary loss in our patient were suggestive of distal renal tubular acidosis. Renal involvement in lymphoma is usually subclinical and clinically overt renal disease is rare. Diffuse lymphomatous infiltration of the kidneys may cause tubular dysfunction and present with hypokalemic paralysis. PMID:18074421

  2. Dynamic renal scintigraphy of dissecting aneurysm of abdominal aorta

    International Nuclear Information System (INIS)

    Tsukamoto, Eriko; Itoh, Kazuo; Furudate, Masayori

    1990-01-01

    Dynamic renal scintigraphy on 15 patients with dissecting aneurysm of abdominal aorta extending below the renal arteries were retrospectively studied. The results were reviewed and classified into 3 types according to perfusion images and parenchymal tissue uptake: Type A--symmetric perfusion and parenchymal tissue uptake; Type B--asymmetrical perfusion and parenchymal tissue uptake; Type C--delayed perfusion of one kidney and symmetric parenchymal tissue uptake. The number of patients who showed Type A, Type B, and Type C were 3 cases, 6 cases, and 6 cases, respectively. In all Type A and 3 of Type B, perfusion of bilateral renal arteries was mainly from true lumen. In the remaining 3 cases of Type B, 2 had one renal artery obstructed with thrombus; 1 had a deformity of one kidney (the blood supply was mainly from a false lumen), suggestive of renal infarction. On the other hand, in all 6 Type C cases, the renal artery where perfusion was delayed was apparently supplied from false lumen. It is suggested in this retrospective study that the findings in Type C, where delayed perfusion of one kidney and symmetric parenchymal tissue uptake were found, were due to the presence of delayed flow through the false lumen; and therefore, specific to cases where the main blood supply of one renal artery from false lumen. (author)

  3. Acute Bilateral Superior Branch Vestibular Neuropathy

    Directory of Open Access Journals (Sweden)

    Dario A. Yacovino

    2018-05-01

    Full Text Available The rapid onset of a bilateral vestibular hypofunction (BVH is often attributed to vestibular ototoxicity. However, without any prior exposure to ototoxins, the idiopathic form of BVH is most common. Although sequential bilateral vestibular neuritis (VN is described as a cause of BVH, clinical evidence for simultaneous and acute onset bilateral VN is unknown. We describe a patient with an acute onset of severe gait ataxia and oscillopsia with features compatible with acute BVH putatively due to a bilateral VN, which we serially evaluated with clinical and laboratory vestibular function testing over the course of 1 year. Initially, bilateral superior and horizontal semicircular canals and bilateral utricles were impaired, consistent with damage to both superior branches of each vestibular nerve. Hearing was spared. Only modest results were obtained following 6 months of vestibular rehabilitation. At a 1-year follow-up, only the utricular function of one side recovered. This case is the first evidence supporting an acute presentation of bilateral VN as a cause for BVH, which would not have been observed without critical assessment of each of the 10 vestibular end organs.

  4. Bilateral optic neuropathy in acute cryptococcal meningitis

    Institute of Scientific and Technical Information of China (English)

    Qi Zhe Ngoo; Li Min Evelyn Tai; Wan Hazabbah Wan Hitam; John Tharakan

    2016-01-01

    We reported a case of cryptococcal meningitis presenting with bilateral optic neuropathy in an immunocompetent patient. A 64-year-old Malay gentleman with no medical comorbidities presented with acute bilateral blurring of vision for a week, which was associated with generalised throbbing headache and low grade fever. He also had som-nolence and altered consciousness. Visual acuity in both eyes was no perception of light with poor pupillary reflexes. Extraocular muscle movements were normal. Anterior segments were unremarkable bilaterally. Fundoscopy revealed bilateral optic disc swelling. CT scan of the brain showed multifocal infarct, but no meningeal enhancement or mass. Cerebrospinal fluid opening pressure was normal, while its culture grew Cryptococcus neoformans. A diagnosis of cryptococcal meningitis with bilateral optic neuropathy was made. Patient was treated with a six-week course of intravenous flu-conazole and started concomitantly on a fortnight's course of intravenous amphotericin B. After that, his general condition improved, but there was still no improvement in his visual acuity. On reviewing at two months post-initiation of treatment, fundi showed bilateral optic atrophy. Bilateral optic neuropathy secondary to cryptococcal meningitis was rare. The prognosis was guarded due to the sequelae of optic atrophy. Anti-fungal medication alone may not be sufficient to manage this condition. However, evidence for other treatment modalities is still lacking and further clinical studies are required.

  5. Bilateral, independent juvenile nasopharyngeal angiofibroma: case report.

    Science.gov (United States)

    Mørkenborg, M-L; Frendø, M; Stavngaard, T; Von Buchwald, C

    2015-10-01

    Juvenile nasopharyngeal angiofibroma is a benign, vascular tumour that primarily occurs in adolescent males. Despite its benign nature, aggressive growth patterns can cause potential life-threatening complications. Juvenile nasopharyngeal angiofibroma is normally unilateral, originating from the sphenopalatine artery, but bilateral symptoms can occur if a large tumour extends to the contralateral side of the nasopharynx. This paper presents the first reported case of true bilateral extensive juvenile nasopharyngeal angiofibroma involving clinically challenging pre-surgical planning and surgical strategy. A 21-year-old male presented with increasing bilateral nasal obstruction and discharge. Examination revealed tumours bilaterally and imaging demonstrated non-contiguous tumours. Pre-operative angiography showed strictly ipsilateral vascular supplies requiring bilateral embolisation. Radical removal performed as one-step, computer-assisted functional endoscopic sinus surgery was performed. The follow-up period was uncomplicated. This case illustrates the importance of suspecting bilateral juvenile nasopharyngeal angiofibroma in patients presenting with bilateral symptoms. Our management, including successful pre-operative planning, enabled one-step total removal of both tumours and rapid patient recovery.

  6. Traumatic renal infarction

    International Nuclear Information System (INIS)

    Yashiro, Naobumi; Ohtomo, Kuni; Kokubo, Takashi; Itai, Yuji; Iio, Masahiro

    1986-01-01

    Four cases of traumatic renal artery occlusion were described and illustrated. In two cases, direct blows to the abdomen compressed the renal artery against the vertebral column. Clinically, they were severely injured with macroscopic hematuria. Aortograms showed abrupt truncation of renal arteries. In the other two, rapid deceleration caused sudden displacement of the kidney producing an intimal tear with resultant thrombosis. Although they showed little injury without macrohematuria, aortograms revealed tapered occlusion of renal arteries. One of them developed hypertension. ''Rim sign'' of post-contrast CT and hypertension resulted from traumatic renal artery occlusion were reviewed. (author)

  7. Giant Urinary Bladder and Bilateral Giant Hydronephrosis due to Bladder Neck Obstruction: One Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Mohammed Fadl Tazi

    2012-01-01

    Full Text Available Bilateral hydronephrosis secondary to urinary obstruction leads to a buildup of back pressure in the urinary tract and may lead to impairment of renal function. Cases of giant hydronephrosis are rare and usually contain no more than 1-2 litres of fluid in the collecting system. Here, we report a rarely seen case with giant urinary bladder and bilateral giant hydronephrosis due to bladder neck obstruction which contains 4000 mL fluid in the collecting system of the kidney mimicking an ascites in an adult male.

  8. Value of renal cortical scintigraphy in children with acute pyelonephritis

    International Nuclear Information System (INIS)

    Paul, A.K.; Miah, M.S.R.; Rahman, H.A.; Hasan, M.H.

    2004-01-01

    Purpose: Acute pyelonephritis is a major cause of morbidity in children with urinary tract infection and can result in irreversible renal scarring leading to hypertension and end-stage renal disease. Tc-99m-dimercaptosuccinic acid (DMSA) scintigraphy is the imaging modality of choice for the detection of acute pyelonephhfis and renal scarfing. This study evaluated the importance of renal cortical scintigraphy to identify children at risk from renal damage due to acute pyelonephritis. Methods: Forty-nine children (ages 9 months to 11 years) with urinary tract infection having positive urine culture were studied. A DMSA scan was performed within 72 hours of receiving antibiotic during acute infection. Single or multiple areas of varying degrees of diminished cortical uptake or diffusely decreased uptake in an enlarged kidney was considered for the diagnosis of acute pyelonephrifis. Follow-up scintigraphy was done at 6 months of initial scan in children with acute pyelonephritis documented by DMSA scan. Renal scarring was considered if the affected kidney shows cortical thinning or focal cortical defect with loss of volume or become small kidney. Children with known renal tract abnormalities were excluded from the study. RESULTS: Twenty-seven children (55%) wine considered acute pyelonephritis by DMSA scintigraphy and the abnormality was bilateral in 17(63%) cases and unilateral in 10(37%) cases. Among these 44 abnormal kidneys, scintigraphy showed solitary defect in 29 kidneys, multiple defects in 6 kidneys and diffuse decreased uptake in 9 kidneys. Of them, twenty children were available for follow-up evaluation and scintigraphy demonstrated complete recovery in 21 of 34 (62%) kidneys and renal scarfing in 13 of 34 (38%) kidneys. Renal scarring was found in 5 of 7 kidneys (71%) with diffuse decreased uptake, 2 of 5 kidneys (40%) with multiple cortical defect and 6 of 22 (27%) with single focal detect. Conclusion: The scintigraphic pattern of acute pyelonephritis

  9. A pediatric renal lymphoma case presenting with central nervous system findings.

    Science.gov (United States)

    Baran, Ahmet; Küpeli, Serhan; Doğru, Omer

    2013-06-01

    In pediatric patients renal lymphoma frequently presents in the form of multiple, bilateral mass lesions, infrequently as a single or retroperitoneal mass, and rarely as diffuse infiltrative lesions. In patients with apparent central nervous system involvement close attention to other physical and laboratory findings are essential for preventing a delay in the final diagnosis. Herein we present a pediatric patient with renal lymphoma that presented with central nervous system findings that caused a delay in diagnosis. None declared.

  10. Renal Artery Variations, Hilar Arrangement and Its Distances to Ventral Branches of Abdominal Aorta: A Morphometric Study

    Directory of Open Access Journals (Sweden)

    D'Souza A

    2015-10-01

    Full Text Available Purpose: To measure the distance of origin of renal artery in relation to the ventral branches of abdominal aorta and also to study the variations in the number and the hilar branching pattern of renal arteries. Materials and methods: The present study was carried out using ten embalmed adult cadavers. The distances were measured bilaterally from the origin of renal artery to the origin of superior and inferior mesenteric artery and the bifurcation of abdominal aorta. Results: Out of ten cadavers studied, bilateral accessory renal artery was observed in two cases. The hilar branching pattern varied from a single artery to maximum of six branches. The mean and standard deviations of the measured parameters were calculated. Conclusion: Knowledge of variations of renal artery is important for surgeons in performing many procedures and may help to avoid clinical complications in the abdominal region.

  11. Problems in severe bilateral urinary tract anomalies

    DEFF Research Database (Denmark)

    Thorup, Jørgen Mogens

    1989-01-01

    function. There were two deaths. Five children have severe and two a moderate reduction of renal function; twenty good renal function. There were ten nephrectomies and four heminephrectomies. Seven patients had a temporary and eight a permanent urinary diversion. However, four of the latter were later...

  12. A longitudinal study of the bilateral benefit in children with bilateral cochlear implants.

    Science.gov (United States)

    Asp, Filip; Mäki-Torkko, Elina; Karltorp, Eva; Harder, Henrik; Hergils, Leif; Eskilsson, Gunnar; Stenfelt, Stefan

    2015-02-01

    To study the development of the bilateral benefit in children using bilateral cochlear implants by measurements of speech recognition and sound localization. Bilateral and unilateral speech recognition in quiet, in multi-source noise, and horizontal sound localization was measured at three occasions during a two-year period, without controlling for age or implant experience. Longitudinal and cross-sectional analyses were performed. Results were compared to cross-sectional data from children with normal hearing. Seventy-eight children aged 5.1-11.9 years, with a mean bilateral cochlear implant experience of 3.3 years and a mean age of 7.8 years, at inclusion in the study. Thirty children with normal hearing aged 4.8-9.0 years provided normative data. For children with cochlear implants, bilateral and unilateral speech recognition in quiet was comparable whereas a bilateral benefit for speech recognition in noise and sound localization was found at all three test occasions. Absolute performance was lower than in children with normal hearing. Early bilateral implantation facilitated sound localization. A bilateral benefit for speech recognition in noise and sound localization continues to exist over time for children with bilateral cochlear implants, but no relative improvement is found after three years of bilateral cochlear implant experience.

  13. 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.

  14. 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.

  15. A case of bilateral tubal pregnancy

    Directory of Open Access Journals (Sweden)

    Ayano Funamizu

    2017-11-01

    Full Text Available Bilateral tubal pregnancy is very rare and occurs in only 1 out of every 200,000 spontaneous pregnancies. In this case, a 29-year-old woman with a history of primary infertility underwent treatment with human menopausal gonadotropin (hMG-human chorionic gonadotropin (hCG, and became pregnant. A gestational sac (GS was not detected in the uterus and transvaginal ultrasonography (USG revealed GS with fetal heartbeat in the left adnexa at 7 weeks and 6 days of gestation. The patient underwent laparoscopic surgery and ultimately, bilateral tubal pregnancy was diagnosed. Consequently, bilateral fallopian tube resection was performed. Afterwards, she conceived by assisted reproductive technology (ART and delivered vaginally. This case suggests that even if a GS is found in one fallopian tube by USG, it is important to evaluate the other fallopian tube carefully. Keywords: bilateral tubal pregnancy, ectopic pregnancy, human menopausal gonadotropin, laparoscopy

  16. Bilateral nasolabial cysts associated with recurrent dacryocystitis.

    Science.gov (United States)

    Kyrmizakis, Dionysios E; Lachanas, Vassilios A; Benakis, Antonios A; Velegrakis, George A; Aslanides, Ioannis M

    2005-05-01

    Nasolabial cysts are rare, nonodontogenic, soft-tissue, developmental cysts occurring inferior to the nasal alar region. They are thought to arise from remnants of the nasolacrimal ducts and they are frequently asymptomatic. We report a rare case of bilateral nasolabial cysts accompanied by bilateral chronic dacryocystitis. A 48-year-old woman suffering from bilateral chronic dacryocystitis was referred to our department for endonasal dacryocystorhinostomy. She had undergone external dacryocystorhinostomy on the left side a few years earlier. Physical examination and computed tomography scan revealed nasolabial cysts bilaterally inferior to the nasal alar region. The cysts were removed via a sublabial approach and endoscopic dacryocystorhinostomy was performed on the right side. Ten months after surgery, the patient was asymptomatic. There may be a correlation, due to embryological reasons, between the presence of nasolabial cysts and the presence of chronic dacryocystitis. Both can be corrected surgically, under the same anaesthesia, without visible scar formation.

  17. THE EUROPEAN UNION’S BILATERAL APPROACH

    Directory of Open Access Journals (Sweden)

    Ludmila BORTA

    2014-12-01

    Full Text Available The EU is a world economic power and a major trading partner for most countries. All the time, this region has been interested and has acted towards a free and fair trade. The decrease and even the elimination of tariff and non-tariff barriers in the world trade are among the main objectives of the EU strategy for international trade. At the moment, the elusive outcome of the WTO Doha Round has led to the proliferation of bilateral trade agreements worldwide. Although the EU remains committed to further development of the multilateral trading system, however, the EU still has appealed also to the development of bilateral trade relations. The aim of this paper is to illustrate the current bilateral dimension of the common commercial policy of the EU. In conclusion, to describe this bilateral approach of the EU we are using one word, namely “diversity”.

  18. FLOWING BILATERAL FILTER: DEFINITION AND IMPLEMENTATIONS

    Directory of Open Access Journals (Sweden)

    Maxime Moreaud

    2015-06-01

    Full Text Available The bilateral filter plays a key role in image processing applications due to its intuitive parameterization and its high quality filter result, smoothing homogeneous regions while preserving the edges of the objects. Considering the image as a topological relief, seeing pixel intensities as peaks and valleys, we introduce a way to control the tonal weighting coefficients, the flowing bilateral filter, reducing "halo" artifacts typically produced by the regular bilateral filter around a large peak surrounded by two valleys of lower values. In this paper we propose to investigate exact and approximated versions of CPU and parallel GPU (Graphical Processing Unit based implementations of the regular and flowing bilateral filter using the NVidia CUDA API. Fast implementations of these filters are important for the processing of large 3D volumes up to several GB acquired by x-ray or electron tomography.

  19. EXERTIONAL RHABDOMYOLYSIS OF THE BILATERAL ADDUCTOR MAGNUS

    Directory of Open Access Journals (Sweden)

    Tolga Saka

    2007-12-01

    Full Text Available We present a case study of a person (63 year-old man, who has been using statins for 18 years, with rhabdomyolysis of the bilateral adductor muscles associated with strenuous and prolonged eccentric exercises (hiking in a hot environment. Clinical examination showed predominantly on the right side muscle swelling and palpational pain of the bilateral adductor muscle groups and bilateral tibial edema. His serum creatine kinase (CK level was 12218 IU/L. T2-weighted magnetic resonance (MR images showed a high signal intensity in the bilateral adductor muscles of the hip. The patient did not develop complications and returned to his previous performance level in 30 days following adequate hydration and resting of the affected muscles. Strenuous eccentric exercise should be avoided during the course of statin use and clinicians should be aware of present observations when considering the significance of acute CK elevations in patients on statin treatment

  20. Bilateral Tubal Pregnancy without Known Risk Factor

    Directory of Open Access Journals (Sweden)

    Hyacinthe Zamané

    2017-01-01

    Full Text Available Spontaneous bilateral ectopic gestation is very rare. The authors report a case diagnosed and taken care of at Yalgado Ouedraogo Teaching Hospital, Ouagadougou. It was a 30-year-old patient with no known pathological history. She had presented at the obstetric emergencies with a state of hypovolemic shock by haemoperitoneum with digestive disorders, pelvic pain, vaginal bleeding, and a mention of delayed menstruation. The ultrasound coupled with the urinary immunological pregnancy test confirmed the diagnosis of ruptured ectopic pregnancy and a bilateral form was suspected. A laparotomy in emergency confirmed the diagnosis of bilateral ectopic gestation with a right ampullary unruptured pregnancy and a left isthmic ruptured gestation. A bilateral salpingectomy was performed and counseling was made for the use of medical help of procreation in case of future need of pregnancy.

  1. Identification of a duplication of Xq28 associated with bilateral periventricular nodular heterotopia.

    Science.gov (United States)

    Fink, J M; Dobyns, W B; Guerrini, R; Hirsch, B A

    1997-01-01

    Bilateral periventricular nodular heterotopia (BPNH) is a malformation of neuronal migration and is characterized by nodules of heterotopic gray matter lining the lateral ventricles of the brain. The majority of BPNH patients are female and have epilepsy as a sole clinical manifestation of their disease. Familial BPNH has been mapped to Xq28 by linkage analysis. A multiple congenital anomaly-mental retardation syndrome (BPNH/MR) was recently delineated in three unrelated boys with BPNH, cerebellar hypoplasia, severe mental retardation, epilepsy, and syndactyly. High-resolution chromosome analysis revealed a subtle abnormality of Xq28 in one of the boys with BPNH/MR syndrome. FISH with cosmids and YACs from Xq28 further characterized this abnormality as a 2.25-3.25-Mb inverted duplication. No abnormality of Xq28 was detected by G-banding or FISH in the other two boys. These data support the linkage assignment of BPNH to band Xq28 and narrow the critical region to the distal 2.25-3.25 Mb of Xq28. Images Figure 1 Figure 3 Figure 4 Figure 5 PMID:9311743

  2. 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.

  3. Bilateral calcaneal epiphysiolysis in a dog.

    Science.gov (United States)

    Font, J; Pèlach, M; Font, C; Cairo, J

    2013-01-01

    A case of bilateral calcaneal epiphysiolysis in a six-month-old female Dobermann Pinscher is described in this report. The absence of a traumatic event and the clinical, radiographic and histopathological abnormalities led us to the diagnosis of simultaneous bilateral epiphysiolysis of the calcaneus. A tension band and a type II transarticular external fixator were placed. The clinical signs were resolved only temporarily because of the gravity of the bone changes.

  4. Recurrent Bilateral Breast Abscesses after Sternotomy

    Directory of Open Access Journals (Sweden)

    Hamza Cinar

    2012-01-01

    Full Text Available Median sternotomy is the most commonly used incision in cardiothoracic procedures. Development of breast abscess after sternotomy is a very rare situation. We present a case of sternal wound infection with recurrent bilateral breast abscess after sternotomy. Our case is the first and only case in the literature due to the presence of sternal wound infection with recurrent bilateral breast abscess after sternotomy.

  5. Bilateral anophthalmia with septo-optic dysplasia

    OpenAIRE

    Jana, Manisha; Sharma, Sanjay

    2010-01-01

    Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral ano...

  6. Bilateral anophthalmia with septo-optic dysplasia

    Directory of Open Access Journals (Sweden)

    Manisha Jana

    2010-01-01

    Full Text Available Bilateral anophthalmia is a rare entity and association with septo-optic dysplasia is an even rare condition. The condition is characterized by absent eyeballs in the presence of eyelids, conjunctiva or lacrimal apparatus. Though anophthalmia can be diagnosed clinically, imaging plays a crucial role in delineating the associated anomalies. In addition, often clinical anophthalmia may prove to be severe microphthalmia on imaging. We describe the imaging findings in an infant with bilateral anophthalmia and septo-optic dysplasia.

  7. Bilateral cerebellopontine arachnoid cyst: A rare entity.

    Science.gov (United States)

    Sharma, Anand; Sharma, Achal; Mittal, Radhey S; Gandhi, Ashok

    2015-01-01

    Bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs) are very rare: only one case is reported in literature. Pathogenesis of those cysts is unknown; they are thought to be congenital. The presenting symptoms of CPA AC are frequently nonspecific or otological. The management of ACs of the CPA is controversial. We are reporting two cases of bilateral CPA AC with their pathophysiology and review of literature.

  8. BILATERAL CHOROIDAL EXCAVATION IN JUVENILE LOCALIZED SCLERODERMA.

    Science.gov (United States)

    Franklin, Mackenzie L; Day, Shelley

    2018-01-01

    To describe a case of bilateral choroidal excavation in a patient with juvenile localized scleroderma. Case report. An asymptomatic 12-year-old boy with localized scleroderma presented for examination and was found to have bilateral areas of choroidal excavation temporal to the fovea. Previous reports of ocular complications of localized scleroderma have primarily described adnexal and anterior segment changes. This is the second report of choroidal changes in a patient with localized scleroderma, and the first in a pediatric patient.

  9. Bilateral acute retinal necrosis associated with bilateral uveal effusion in an immunocompetent patient: A challenging association

    Directory of Open Access Journals (Sweden)

    S Bala Murugan

    2018-01-01

    Full Text Available Bilateral uveal effusion syndrome associated with bilateral acute retinal necrosis is a diagnostic and therapeutic challenge. A 52 year old man presented with bilateral angle closure with choroidal detachment. With restricted fundus view, parenteral steroid was started. During close follow up bilateral discrete areas of peripheral retinitis were noted. Parenteral steroids were promptly stopped and parenteral antivirals with oral steroids were continued. It showed healing response with nil recurrences till last follow up. Aggressive treatment of bilateral uveal effusion with parenteral steroids can cause progression of bilateral acute retinal necrosis leading to phthisis bulbi. However early diagnosis, prompt intervention and close follow up are the key elements to therapeutic success even during diagnostic surprises and avoid costly mistakes.

  10. A SECOND CASE OF BILATERAL RHEGMATOGENOUS RETINAL DETACHMENTS REPAIRED WITH SIMULTANEOUS BILATERAL PNEUMATIC RETINOPEXY.

    Science.gov (United States)

    Rubin, Uriel; De Jager, Cornelis; Zakour, Moayed; Gonder, J Thomas

    2017-01-01

    To present a case of a patient with simultaneous bilateral retinal detachments treated successfully with bilateral pneumatic retinopexy. Case report. This is a case of an otherwise healthy 49-year-old woman with no remarkable ocular history that presented with simultaneous phakic superior bilateral rhegmatogenous retinal detachments. Treatment on the day of presentation included laser retinopexy of the inferior lattice degeneration in the left eye and bilateral intravitreal injection of 0.4 cc of 100% C3F8 gas preceded by topical anesthesia. After 48 hours, both retinas were completely reattached, and bilateral laser retinopexy was performed to the superior tears. After a review of the literature, the authors could find only two reported cases of simultaneous bilateral retinal detachments treated successfully with pneumatic retinopexy. This is not only a cost-effective procedure but also allows treatment when there is no immediate operating room availability or a when a quick referral for surgery is not possible.

  11. [Case of distal renal tubular acidosis complicated with renal diabetes insipidus, showing aggravation of symptoms with occurrence of diabetes mellitus].

    Science.gov (United States)

    Liu, Hexing; Tomoda, Fumihiro; Koike, Tsutomu; Ohara, Maiko; Nakagawa, Taizo; Kagitani, Satoshi; Inoue, Hiroshi

    2011-01-01

    We report herein a 27-year-old male case of inherited distal renal tubular acidosis complicated with renal diabetes insipidus, the symptoms of which were aggravated by the occurrence of diabetes mellitus. At 2 months after birth, he was diagnosed as having inherited distal renal tubular acidosis and thereafter supplementation of both potassium and alkali was started to treat his hypokalemia and metabolic acidosis. At the age of 4 years, calcification of the bilateral renal medulla was detected by computed tomography. Subsequently his urinary volume gradually increased and polyuria of approximately 4 L/day persisted. At the age of 27 years, he became fond of sugar-sweetened drinks and also often forgot to take the medicine. He was admitted to our hospital due to polyuria of more than 10 L day, muscle weakness and gait disturbance. Laboratory tests disclosed worsening of both hypokalemia and metabolic acidosis in addition to severe hyperglycemia. It seemed likely that occurrence of diabetes mellitus and cessation of medications can induce osmotic diuresis and aggravate hypokalemia and metabolic acidosis. Consequently, severe dehydration, hypokalemia-induced damage of his urinary concentration ability and enhancement of the renin angiotensin system occurred and thereby possibly worsened his hypokalemia and metabolic acidosis. As normalization of hyperglycemia and metabolic acidosis might have exacerbated hypokalemia further, dehydration and hypokalemia were treated first. Following intensive treatment, these abnormalities were improved, but polyuria persisted. Elevated plasma antidiuretic hormone (12.0 pg/mL) and deficit of renal responses to antidiuretic hormone suggested that the polyuria was attributable to the preexisting renal diabetes insipidus possibly caused by bilateral renal medulla calcification. Thiazide diuretic or nonsteroidal anti-inflammatory drugs were not effective for the treatment of diabetes insipidus in the present case.

  12. Rapid estimation of split renal function in kidney donors using software developed for computed tomographic renal volumetry

    International Nuclear Information System (INIS)

    Kato, Fumi; Kamishima, Tamotsu; Morita, Ken; Muto, Natalia S.; Okamoto, Syozou; Omatsu, Tokuhiko; Oyama, Noriko; Terae, Satoshi; Kanegae, Kakuko; Nonomura, Katsuya; Shirato, Hiroki

    2011-01-01

    Purpose: To evaluate the speed and precision of split renal volume (SRV) measurement, which is the ratio of unilateral renal volume to bilateral renal volume, using a newly developed software for computed tomographic (CT) volumetry and to investigate the usefulness of SRV for the estimation of split renal function (SRF) in kidney donors. Method: Both dynamic CT and renal scintigraphy in 28 adult potential living renal donors were the subjects of this study. We calculated SRV using the newly developed volumetric software built into a PACS viewer (n-SRV), and compared it with SRV calculated using a conventional workstation, ZIOSOFT (z-SRV). The correlation with split renal function (SRF) using 99m Tc-DMSA scintigraphy was also investigated. Results: The time required for volumetry of bilateral kidneys with the newly developed software (16.7 ± 3.9 s) was significantly shorter than that of the workstation (102.6 ± 38.9 s, p < 0.0001). The results of n-SRV (49.7 ± 4.0%) were highly consistent with those of z-SRV (49.9 ± 3.6%), with a mean discrepancy of 0.12 ± 0.84%. The SRF also agreed well with the n-SRV, with a mean discrepancy of 0.25 ± 1.65%. The dominant side determined by SRF and n-SRV showed agreement in 26 of 28 cases (92.9%). Conclusion: The newly developed software for CT volumetry was more rapid than the conventional workstation volumetry and just as accurate, and was suggested to be useful for the estimation of SRF and thus the dominant side in kidney donors.

  13. Rapid estimation of split renal function in kidney donors using software developed for computed tomographic renal volumetry

    Energy Technology Data Exchange (ETDEWEB)

    Kato, Fumi, E-mail: fumikato@med.hokudai.ac.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Kamishima, Tamotsu, E-mail: ktamotamo2@yahoo.co.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Morita, Ken, E-mail: kenordic@carrot.ocn.ne.jp [Department of Urology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, 060-8638 (Japan); Muto, Natalia S., E-mail: nataliamuto@gmail.com [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Okamoto, Syozou, E-mail: shozo@med.hokudai.ac.jp [Department of Nuclear Medicine, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, 060-8638 (Japan); Omatsu, Tokuhiko, E-mail: omatoku@nirs.go.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Oyama, Noriko, E-mail: ZAT04404@nifty.ne.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Terae, Satoshi, E-mail: saterae@med.hokudai.ac.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan); Kanegae, Kakuko, E-mail: IZW00143@nifty.ne.jp [Department of Nuclear Medicine, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, 060-8638 (Japan); Nonomura, Katsuya, E-mail: k-nonno@med.hokudai.ac.jp [Department of Urology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, 060-8638 (Japan); Shirato, Hiroki, E-mail: shirato@med.hokudai.ac.jp [Department of Radiology, Hokkaido University Graduate School of Medicine, N15, W7, Kita-ku, Sapporo, Hokkaido 060-8638 (Japan)

    2011-07-15

    Purpose: To evaluate the speed and precision of split renal volume (SRV) measurement, which is the ratio of unilateral renal volume to bilateral renal volume, using a newly developed software for computed tomographic (CT) volumetry and to investigate the usefulness of SRV for the estimation of split renal function (SRF) in kidney donors. Method: Both dynamic CT and renal scintigraphy in 28 adult potential living renal donors were the subjects of this study. We calculated SRV using the newly developed volumetric software built into a PACS viewer (n-SRV), and compared it with SRV calculated using a conventional workstation, ZIOSOFT (z-SRV). The correlation with split renal function (SRF) using {sup 99m}Tc-DMSA scintigraphy was also investigated. Results: The time required for volumetry of bilateral kidneys with the newly developed software (16.7 {+-} 3.9 s) was significantly shorter than that of the workstation (102.6 {+-} 38.9 s, p < 0.0001). The results of n-SRV (49.7 {+-} 4.0%) were highly consistent with those of z-SRV (49.9 {+-} 3.6%), with a mean discrepancy of 0.12 {+-} 0.84%. The SRF also agreed well with the n-SRV, with a mean discrepancy of 0.25 {+-} 1.65%. The dominant side determined by SRF and n-SRV showed agreement in 26 of 28 cases (92.9%). Conclusion: The newly developed software for CT volumetry was more rapid than the conventional workstation volumetry and just as accurate, and was suggested to be useful for the estimation of SRF and thus the dominant side in kidney donors.

  14. 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.

  15. 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.

  16. Bilateral Keratectasia 34 Years after Corneal Transplant

    Directory of Open Access Journals (Sweden)

    Xavier Valldeperas

    2010-07-01

    Full Text Available We report the clinical findings of a patient with severe bilateral keratectasia 34 years after a penetrating keratoplasty (PK in both eyes. An otherwise healthy 67-year-old man complained of deterioration of the eyesight in both eyes over the last 6 months. The patient was diagnosed with bilateral keratoconus at the age of 32 years, and he underwent a bilateral PK. At presentation, visual acuity was 20/200 in the right eye and light perception in the left eye. A Pentacam pachymetric map revealed a central pachymetry of 720 µm in the right eye and of 710 µm in the left eye, as well as an average paracentral pachymetry of 436 and 270 µm in the 9-mm zone in the right and the left eye, respectively. Corneal topography revealed bilateral irregular and asymmetric bowing with generalized steepening and high corneal power. We describe a case of bilateral keratectasia 34 years after PK in a patient who was originally diagnosed with bilateral keratoconus.

  17. Bilateral tension pneumothorax related to acupuncture.

    Science.gov (United States)

    Tagami, Rumi; Moriya, Takashi; Kinoshita, Kosaku; Tanjoh, Katsuhisa

    2013-06-01

    We report on a patient with a rare case of bilateral tension pneumothorax that occurred after acupuncture. A 69-year-old large-bodied man, who otherwise had no risk factors for spontaneous pneumothorax, presented with chest pressure, cold sweats and shortness of breath. Immediately after bilateral pneumothorax had been identified on a chest radiograph in the emergency room, his blood pressure and percutaneous oxygen saturation suddenly decreased to 78 mm Hg and 86%, respectively. We confirmed deterioration in his cardiopulmonary status and diagnosed bilateral tension pneumothorax. We punctured his chest bilaterally and inserted chest tubes for drainage. His vital signs promptly recovered. After the bilateral puncture and drainage, we learnt that he had been treated with acupuncture on his upper back. We finally diagnosed a bilateral tension pneumothorax based on the symptoms that appeared 8 h after the acupuncture. Because the patient had no risk factors for spontaneous pneumothorax, no alternative diagnosis was proposed. We recommend that patients receiving acupuncture around the chest wall must be adequately informed of the possibility of complications and expected symptoms, as a definitive diagnosis can be difficult without complete information.

  18. MODERN VIEWS ON BILATERAL BREAST CANCER

    Directory of Open Access Journals (Sweden)

    Ye. A. Fesik

    2014-01-01

    Full Text Available Presented modern literature data on the features of the pathogenesis, course, clinical and morphological expression and tumor characteristics, parameters and nodal metastasis of hematogenous bilateral breast cancer. Highlight the results of domestic and foreign studies in recent years to determine the prognostic factors and recurrence of synchronous and metachronous bilateral breast cancer. It was revealed that the frequency of bilateral breast tumor lesions varies widely, ranging from 0.1 to 20%, with metachronous tumors recorded significantly higher (69.6% than the synchronous (22.7%. The probability of occurrence of metachronous breast cancer is higher in women with a family history, as well as if they have a gene mutation BRCA-1. Found that the most common histological type of breast tumor with bilateral lesions is invasive ductal. However, the incidence of invasive lobular cancer and non-invasive lobular cancer is slightly higher among synchronous bilateral cancer compared with unilateral disease. Studies have shown that in a double-sided synchronous breast cancer tumor, as a rule, has a lower degree of differentiation, and the higher the expression level of estrogen receptors and progesterone receptors. Relevance of the issue because the identification of patterns in the study of lymphatic and hematogenous features bilateral metastasis of mammary tumors provides a basis for speculation about the differences in the progression of neoplastic disease in these groups and is a cause for further detailed research in this area to identify and evaluate the prognosis and also the choice of tactics of such patients.

  19. Posterior urethral valves: Risk factors for progression to renal failure.

    Science.gov (United States)

    Bilgutay, Aylin N; Roth, David R; Gonzales, Edmond T; Janzen, Nicolette; Zhang, Wei; Koh, Chester J; Gargollo, Patricio; Seth, Abhishek

    2016-06-01

    Posterior urethral valves (PUVs) are the most common etiology for congenital urethral obstruction and congenital bilateral renal obstruction. PUVs produce a spectrum of urologic and renal sequelae. Our aims were to assess outcomes of PUV patients, to determine whether vesicoureteral reflux (VUR) is a risk factor for progression to renal failure, and to identify other risk factors for poor outcomes. We conducted a retrospective analysis of PUV patients from 2006 to 2014. Data collected included demographics, initial renal ultrasound (RUS) findings, creatinine at presentation and nadir, pre- and postoperative VUR status, presence or absence of recurrent urinary tract infections (UTIs), and surgical intervention(s). Univariate and multivariate analyses were used to determine risk factors for renal failure. Of 104 patients, 42.3% (44/104) were diagnosed prenatally, 31.8% (14/44) of whom underwent prenatal intervention. Postnatally, 90.4% (94/104) initially underwent transurethral resection of PUVs (TUR-PUVs). Vesicostomy was the next most common index surgery (4.8%). Forty-two percent (44/104) required >1 surgery. The predominant second surgery was repeat TUR-PUV in 16 patients. At last follow-up (mean 28.8 months after initial surgery), 20.2% had chronic kidney disease (CKD) of at least stage IIIA, and 8.6% had progressed to end-stage renal disease (ESRD). Antenatal diagnosis, prematurity, abnormal renal cortex, and loss of corticomedullary differentiation (CMD) on initial RUS were associated with CKD and ESRD on univariate analysis, as were elevated creatinine on presentation and at nadir. Presence of pre- or postoperative VUR and recurrent UTIs were associated with the need for multiple surgeries, but not with poor renal outcomes. On multivariate analysis, nadir creatinine was the only independent predictor of final renal function. Our finding that creatinine is the only independent risk factor for poor renal outcomes in PUV patients is consistent with the

  20. Disruption of cyclooxygenase-2 prevents downregulation of cortical AQP2 and AQP3 in response to bilateral ureteral obstruction in the mouse

    DEFF Research Database (Denmark)

    Nilsson, Line; Madsen, Kirsten Morill; Topcu, Sukru Oguzkan

    2012-01-01

    Bilateral ureteral obstruction (BUO) in rats is associated with increased cyclooxygenase type 2 (COX-2) expression, and selective COX-2 inhibition prevents downregulation of aquaporins (AQPs) in response to BUO. It was hypothesized that a murine model would display similar changes in renal COX-2 ...

  1. Long-term followup of bilateral high (Sober) urinary diversion in patients with posterior urethral valves and its effect on bladder function

    NARCIS (Netherlands)

    Ghanem, MA; Nijman, RJM

    Purpose: Although valve ablation is the treatment of choice in patients with posterior urethral valves (PLTV), temporary high (ureterostomy) diversion remains controversial. In this study we evaluated the effect of bilateral Sober high urinary diversion on renal and bladder function. Materials and

  2. Cyclosporine-A therapy-induced multiple bilateral breast and accessory axillary breast fibroadenomas: a case report

    Directory of Open Access Journals (Sweden)

    Darwish Ahmed

    2010-08-01

    Full Text Available Abstract Introduction Breast adenoma is common. However, in the setting of post-transplantation immune suppression it may be expressed differently. Case presentation A 35-year-old Sudanese woman, with a history of renal transplantation two and half years prior to presentation, was on a single immune suppression therapy in the form of cyclosporine-A since the transplantation. During a regular follow-up visit, she was noticed to have gingival hypertrophy and bilateral breast and axillary swellings. She underwent successful surgical resection of the bilateral fibroadenomas. Conclusions Cyclosporine-A therapy post renal transplantation is associated with an increased incidence of benign breast changes as fibroadenoma. Regular follow-up and appropriate selection of immunosuppressant therapy are essential in the post transplantation management of these patients.

  3. Late renal function following whole abdominal irradiation

    International Nuclear Information System (INIS)

    Irwin, C.; Fyles, A.; Wong, S.C.; Cheung, C.M.; Zhu, Y.

    1996-01-01

    Sixty patients treated with whole abdominal radiotherapy who had remained disease-free since completion of treatment participated in a study to assess the late clinical and biochemical effects of bilateral renal irradiation. Minimum follow-up was 5 years with a maximum of 20 years and a median of 9 years. Fifty-two patients in the study group were treated for primary ovarian cancer. Seven had non-Hodgkins lymphoma arising in the gastrointestinal tract and one patient had a carcinoid tumour arising in small bowel. None of the patients received chemotherapy. Abdominal radiation was given using an open beam technique to a mean dose of 22.92 Gy (range 6.68-27.54 Gy) in 1.02 to 1.25 Gy fractions treated once daily. Posterior kidney shields were used in order to limit the renal dose to <20 Gy. Mean radiation dose to both kidneys (retrospectively calculated) was 19.28 Gy (range 6.68-22.99 Gy). Patients ranged in age from 32-81 years with a median of 61 years. No patient had clinical evidence of renal impairment. Nine patients were hypertensive prior to radiotherapy and a further five patients became hypertensive after treatment. Serum creatinine values ranged from 44-123 μmol/l, with a mean of 87 μmol/l. Creatinine clearance ranged from 0.61-2.38 ml/s (mean 1.28 ml/s). Tubular function tests revealed one borderline high 24-h protein excretion and normal 24-h phosphorous and uric acid. Using a multiple linear regression analysis with creatinine clearance as the endpoint, age was the only significant variable (P < 0.00001) and renal dose and interval from treatment were not independently significant. There was no evidence of late renal toxicity more than 5 years after whole abdominal radiotherapy delivered with this technique and dose/fractionation schedule, and using the clinical and biochemical endpoints assessed in this study

  4. Vesicoureteral refluxed volume and renal function

    International Nuclear Information System (INIS)

    Markovic, V.; Capkun, V.; Eterovic, D.; Stanicic, A.; Saraga, M.

    1994-01-01

    The therapeutical approach to vesicoureteral reflux (VUR) depends on assessment of the renal involvement. The effective renal plasma flow (ERPF) and parenchymal mean transit time of radiotracer (pMTT) of the affected kidney are sensitive functional parameters. We investigated the association of these functional indices with the volume of refluxed urine. In 64 children (mean age 6.4 yrs) the presence of VUR was confirmed with direct radionuclide cystography in 80 ureters (48 unilateral and 32 bilateral) and the maximal volume of refluxed urine (MVRU) was determined for each uretero-renal unit. All patients also underwent dynamic renal scintigraphy with I-131-hippuran, providing the values of pMTT and relative renal hippuran clearances of the respective kidneys by deconvolution analysis. In 37 of the affected kidneys ERPF was also determined by combining the latter results with total ERPF, determined by plasma clearance of hippuran. Using the borderline value of MVRU of 4 ml, the group with higher MVRU exhibited significantly lower ERPF of the affected kidney (194±93 vs. 270±77 ml/min/1.73 m2, p=.002) and significantly higher proportion of pMTT's over 3.5 min (31/33 vs. 17/47, p=.003). The negative linear correlation between MVRU and ERPF was found (r=-.45, p=.006). We conclude that quantitative radionuclide cystography, aside from diagnosis and follow-up of VUR, may also provide insight in the function of the affected kidney and thus contribute in designing the therapeutical approach. (author)

  5. Renal artery stent angioplasty for renovascular hypertension

    International Nuclear Information System (INIS)

    Li Gang; Li Haiqing; Wang Lin

    2005-01-01

    Objective: To evaluate the therapeutic results of expandable stent for treatment of atherosclerotic renovascular obstructive disease. Methods: 15 patients (10 men and 5 women, 41-75 years old; mean age, 52 years) with renal arterial hypertension underwent renal stent angioplasty including renal arterial stenosis 89%(n=13) and fully obstruction without function in 2, of which 2 patients had bilateral involvement. The stenotic range of all arterial segments showed 60% to 90% width of the normal arterial diameter. 16 stents were implanted under the guidance of fluoroscopy. The most of stents implanted were Palmaz (n=12, 75%) with regular clinical and angiographic follow up. Results: Technical success (residual stenosis <30%) was achieved in all patients without serious complication. During the follow-up (6-15 months; mean, 8 ± 4 months), hypertension was improved in 9 patients and cured in 4 patients with a total benefit of 86% and no efficacy in 2(13%). The average systolic blood pressure decreased from 27.12 ± 3.09 kPa to 18.62 ± 3.12 kPa and the average diastolic blood pressure decreased from 17.73 ± 1.92 kPa to 11.12 ± 2.43 kPa after stent treatment (P<0.05). Serum creatinine remained stable in 60% (n=9) patients with improvement in 33% (n=5) and worsened in 6% (n=1) patients. Follow-up angiography was performed in all patients with 1 case of a restenosis. 6 months after expanding through stent by using balloon, the two follow up angiographies showed a stable restenosis about 20%. Conclusions: Percutaneous transluminal stent placement is highly beneficial for patients who had renal arterial obstructive disease. The success of stent angioplasty of complete obstructive renal arteries reveals wide prospects for interventional method. (authors)

  6. Imaging of renal osteodystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Jevtic, V. E-mail: vladimir.jevtic@mf.uni-lj.si

    2003-05-01

    Chronic renal insufficiency, hemodialysis, peritoneal dialysis, renal transplantation and administration of different medications provoke complex biochemical disturbances of the calcium-phosphate metabolism with wide spectrum of bone and soft tissue abnormalities termed renal osteodystrophy. Clinically most important manifestation of renal bone disease includes secondary hyperparathyroidism, osteomalacia/rickets, osteoporosis, adynamic bone disease and soft tissue calcification. As a complication of long-term hemodialysis and renal transplantation amyloid deposition, destructive spondyloarthropathy, osteonecrosis, and musculoskeletal infections may occur. Due to more sophisticated diagnostic methods and more efficient treatment classical radiographic features of secondary hyperparathyroidism and osteomalacia/rickets are now less frequently seen. Radiological investigations play an important role in early diagnosis and follow-up of the renal bone disease. Although numerous new imaging modalities have been introduced in clinical practice (scintigraphy, CT, MRI, quantitative imaging), plain film radiography, especially fine quality hand radiograph, still represents most widely used examination.

  7. Imaging of renal osteodystrophy

    International Nuclear Information System (INIS)

    Jevtic, V.

    2003-01-01

    Chronic renal insufficiency, hemodialysis, peritoneal dialysis, renal transplantation and administration of different medications provoke complex biochemical disturbances of the calcium-phosphate metabolism with wide spectrum of bone and soft tissue abnormalities termed renal osteodystrophy. Clinically most important manifestation of renal bone disease includes secondary hyperparathyroidism, osteomalacia/rickets, osteoporosis, adynamic bone disease and soft tissue calcification. As a complication of long-term hemodialysis and renal transplantation amyloid deposition, destructive spondyloarthropathy, osteonecrosis, and musculoskeletal infections may occur. Due to more sophisticated diagnostic methods and more efficient treatment classical radiographic features of secondary hyperparathyroidism and osteomalacia/rickets are now less frequently seen. Radiological investigations play an important role in early diagnosis and follow-up of the renal bone disease. Although numerous new imaging modalities have been introduced in clinical practice (scintigraphy, CT, MRI, quantitative imaging), plain film radiography, especially fine quality hand radiograph, still represents most widely used examination

  8. Novel resveratrol analogues attenuate renal ischemic injury in rats

    Science.gov (United States)

    Khader, Adam; Yang, Weng-Lang; Kuncewitch, Michael; Prince, Jose M.; Marambaud, Philippe; Nicastro, Jeffrey; Coppa, Gene F.; Wang, Ping

    2014-01-01

    Background Renal ischemia-reperfusion (I/R) is a severe clinical complication with no specific treatment. Resveratrol has been shown as a promising experimental agent in renal I/R due to its effect on cellular energy metabolism, oxidative stress, and inflammation. Recently, we identified two biologically active resveratrol analogues (RSVAs), RSVA405 and RSVA314. We hypothesized that both RSAVs would attenuate I/R-induced renal injury. Methods Adult male rats were subjected to renal I/R through bilateral renal pedicle clamping for 60 min, followed by reperfusion. RSVA405 (3 mg/kg BW), RSVA314 (3 mg/kg BW), or vehicle (10% DMSO and 33% Solutol in PBS) was administered by intraperitoneal injection 1 h prior to ischemia. Blood and renal tissues were collected 24 h after I/R for evaluation. Results Administration of RSVA405 and RSVA314 significantly reduced the serum levels of renal dysfunction and injury markers, including creatinine, blood urea nitrogen, aspartate aminotransferase, and lactate dehydrogenase, compared to vehicle. The protective effect of RSVA405 and RSVA314 was also reflected on histologic evaluation. Both RSVAs reduced the number of apoptotic cells by more than 60% as determined by TUNEL assay, compared to vehicle. The renal ATP levels of the vehicle group was decreased to 52.4% of control, while those of the RSVA405 and RSVA314 groups were restored to 72.3% and 79.6% of control, respectively. Both RSVAs significantly reduced the protein expression of inducible nitric oxide synthase and nitrotyrosine, and the mRNA levels of TNF-α, IL-6 and IL-1β. Conclusions RSVA405 and RSVA314 attenuate I/R-induced renal injury through the modulation of energy metabolism, oxidative stress, and inflammation. PMID:25214260

  9. Bilateral Pseudarthrosis of the Femoral Neck in a 25-Year-Old Male with Hereditary Hypophosphatemic Rickets

    Directory of Open Access Journals (Sweden)

    Joris Anthonissen

    2014-01-01

    Full Text Available Hereditary hypophosphatemic rickets (HHR is a rare disorder of renal phosphate wasting and the most common form of heritable rickets. Here, we report a case of an active 25-year-old male with HHR showing atraumatic bilateral femoral neck pseudarthrosis after 4 years of consecutive knee pain. A conservative therapy was administered, taking into account both the risks of surgical treatment and the little impairment even in the sport activities which the patient experienced.

  10. Bilateral Femoral Neck Fatigue Fracture due to Osteomalacia Secondary to Celiac Disease: Report of Three Cases.

    Science.gov (United States)

    Selek, Ozgur; Memisoglu, Kaya; Selek, Alev

    2015-08-01

    Bilateral non traumatic femoral neck fatigue fracture is a rare condition usually occurring secondary to medical conditions such as pregnancy, pelvic irradiation, corticosteroid exposure, chronic renal failure and osteomalacia. In this report, we present three young female patients with bilateral femoral neck fracture secondary to osteomalacia. The underlying cause of osteomalacia was Celiac disease in all patients. The patients were treated with closed reduction and internal fixation with cannulated lag screws. They were free of pain and full weight bearing was achieved at three months. There were no complications, avascular necrosis and nonunion during the follow up period. In patients with bone pain, non traumatic fractures and muscle weakness, osteomalacia should be kept in mind and proper diagnostic work-up should be performed to identify the underlying cause of osteomalacia such as celiac disease.

  11. Hypertension after bilateral kidney irradiation in young and adult rats

    International Nuclear Information System (INIS)

    Jongejan, H.T.; van der Kogel, A.J.; Provoost, A.P.; Molenaar, J.C.

    1987-01-01

    The mechanism of a rise in blood pressure after kidney irradiation is unclear but most likely of renal origin. We have investigated the role of the renin-angiotensin system and dietary salt restriction in the development of systolic hypertension after bilateral kidney irradiation in young and adult rats. Three to 12 months after a single X-ray dose of 7.5 or 12.5 Gy to both kidneys of young and adult rats, the systolic blood pressure (SBP) and plasma renin concentration (PRC) were measured regularly. A single X-ray dose of 12.5 Gy caused a moderate rise in SBP and a slight reduction in PRC in both young and adult rats. A dose of 7.5 Gy did not significantly alter the SBP or PRC during the follow-up period of 1 year. In a second experiment, the kidneys of young rats received an X-ray dose of 20 Gy. Subsequently, rats were kept on a standard diet (110 mmol sodium/kg) or a sodium-poor diet (10 mmol sodium/kg). On both diets, SBP started to rise rapidly 3 months after kidney irradiation. Sodium balance studies carried out at that time revealed an increased sodium retention in the irradiated rats compared to controls on the same diet. In rats on a low sodium intake, there was neither a delay nor an alleviation in the development of hypertension. Compared to controls, the PRC tended to be lower in irradiated rats up to 4 months after irradiation. Subsequently, malignant hypertension developed in all 20 Gy rats, resulting in pressure natriuresis, stimulating the renin-angiotensin system. Our findings indicated that hypertension after bilateral kidney irradiation was not primarily the result of an activation of the renin-angiotensin system. Although there were some indications that sodium retention played a role, dietary sodium restriction did not influence the development of hypertension

  12. Renal artery stenosis

    International Nuclear Information System (INIS)

    Desberg, A.; Paushter, D.M.; Lammert, G.K.; Hale, J.; Troy, R.; Novic, A.; Nally, J. Jr.

    1989-01-01

    Renal artery disease is a potentially correctable cause of hypertension. Previous studies have suggested the utility of duplex sonography in accurately detecting and grading the severity of renal artery stenosis. The purpose of this paper is to evaluate color flow Doppler for this use. Forty-three kidneys were examined by color-flow Doppler and conventional duplex sampling in patients with suspected renovascular hypertension or those undergoing aortography for unrelated reasons. Doppler tracings were obtained from the renal arteries and aorta with calculation of the renal aortic ratio (RAR) and resistive index (RI). Results of Doppler sampling with color flow guidance were compared with aortograms in a blinded fashion

  13. Incidental renal neoplasms

    DEFF Research Database (Denmark)

    Rabjerg, Maj; Mikkelsen, Minne Nedergaard; Walter, Steen

    2014-01-01

    On the basis of associations between tumor size, pathological stage, histological subtype and tumor grade in incidentally detected renal cell carcinoma vs symptomatic renal cell carcinoma, we discussed the need for a screening program of renal cell carcinoma in Denmark. We analyzed a consecutive...... series of 204 patients with renal tumors in 2011 and 2012. The tumors were classified according to detection mode: symptomatic and incidental and compared to pathological parameters. Eighty-nine patients (44%) were symptomatic, 113 (55%) were incidental. Information was not available in two patients...

  14. Penile gangrene in diabetes mellitus with renal failure: A poor prognostic sign of systemic vascular calciphylaxis

    Directory of Open Access Journals (Sweden)

    Mayank Mohan Agarwal

    2007-01-01

    Full Text Available Penile gangrene associated with chronic renal failure is very uncommon. A 52-year-old man with diabetes mellitus, diffuse atherosclerosis, ischemic cardiomyopathy and end-stage renal disease presented with blackening of distal penis for 10 days. His general condition was poor and gangrene of prepuce and glans was noted. Doppler and magnetic-resonance angiography revealed bilateral internal iliac artery obstruction. He underwent trocar suprapubic cystostomy and was planned for partial penectomy. But he died of severe diabetic complications in the interim period. Penile gangrene is a manifestation of widespread vascular calcifications associated with end-stage renal disease and is a marker of poor prognosis.

  15. Percutaneous transluminal angioplasty in renal artery stenosis by takayasu's arteritis

    International Nuclear Information System (INIS)

    Zeon, Seok Kil; Kim, Ok Bae; Kim, Hyun Chul

    1984-01-01

    Authors want to report that PTA was an out-standing therapeutic method in treatment of renovascular hypertension by renal artery stenosis due to Takayasu's arteritis in an 18-years-old Korean female. Abdominal aortogram and bilateral renal arteriogram showed mild smooth narrowing of the mid abdominal aorta of 4cm length from origin of renal arteries, complete occlusion of left renal artery from it's origin with a few small collateral arteries and severe stenosis of proximal right renal artery. PTA of right renal artery was performed with Gruntzig Balloon dilatation catheter, and the patient's blood pressure was down to 120/80 from 220/130, stable for 8 months follow-up examination. Takayasu's arteritis is one of the most important cause of renovascular hypertension in Orientals. Multiple treatment modalities, such as steroid administration or variable surgical intervention, were attempted and limited response was presented. But PTA is a simple, cost-effective, repeated, and relatively complication-free method in treatment of renovascular hypertension due to Takayasu's arteritis, with an outstanding result, as authors case

  16. Renal cell carcinoma in patient with crossed fused renal ectopia

    Directory of Open Access Journals (Sweden)

    Ozgur Cakmak

    2016-01-01

    Full Text Available Primary renal cell carcinomas have rarely been reported in patients with crossed fused renal ectopia. We presented a patient with right to left crossed fused kidney harbouring renal tumor. The most frequent tumor encountered in crossed fused renal ectopia is renal cell carcinoma. In this case, partial nephrectomy was performed which pave way to preservation of the uninvolved both renal units. Due to unpredictable anatomy, careful preoperative planning and meticulous delineation of renal vasculature is essential for preservation of the uninvolved renal units.

  17. Bilateral blindness secondary to optic nerve ischemia from severe amlodipine overdose: a case report.

    Science.gov (United States)

    Kao, Raymond; Landry, Yves; Chick, Genevieve; Leung, Andrew

    2017-08-03

    Calcium channel blockers are commonly prescribed medications; calcium channel blocker overdose is becoming increasingly prevalent. The typical presentation of a calcium channel blocker overdose is hypotension and decreased level of consciousness. We describe a case of a calcium channel blocker overdose that led to bilateral cortical blindness, a presentation that has not previously been reported. A 49-year-old white woman with known bilateral early optic atrophy presented to our hospital with hypotension and obtundation following a known ingestion of 150 mg of amlodipine. She was transferred to our intensive care unit where she was intubated, mechanically ventilated, and required maximal vasopressor support (norepinephrine 40 mcg/minute, epinephrine 40 mcg/minute, and vasopressin 2.4 units/hour) along with intravenously administered crystalloid boluses. Despite these measures, she continued to deteriorate with persistent hypotension and tachycardia, as well as anuria. Intralipid emulsion therapy was subsequently administered to which no initial response was observed. A chest X-ray revealed diffuse pulmonary edema; intravenous diuresis as well as continuous renal replacement therapy was initiated. Following the initiation of continuous renal replacement therapy, her oxygen requirements as well as urine output began to improve, and 3 days later she was liberated from mechanical ventilation. Following extubation, she complained of new onset visual impairment, specifically seeing only red-green colors, but no objects. An ophthalmologic examination revealed that this was due to bilateral optic atrophy from prolonged hypotension during the first 24 hours after the overdose. Persistent hypotension in the setting of a calcium channel blocker overdose can lead to worsening optic atrophy resulting in bilateral cortical blindness.

  18. Stages of Renal Cell Cancer

    Science.gov (United States)

    ... Tumors Treatment Genetics of Kidney Cancer Research Renal Cell Cancer Treatment (PDQ®)–Patient Version General Information About Renal Cell Cancer Go to Health Professional Version Key Points Renal ...

  19. Concomitant gentamicin-induced nephrotoxicity and bilateral ...

    African Journals Online (AJOL)

    2015-11-20

    Nov 20, 2015 ... author is credited and the new creations are licensed under the identical terms. For reprints .... of aminoglycosides varies from center to center. The ... underlying age‑related reduced renal reserve and impaired hearing ability.

  20. Tumoral calcinosis in a dog with chronic renal failure

    International Nuclear Information System (INIS)

    Spotswood, T.C.

    2003-01-01

    A 2-year-old male German shepherd dog in poor bodily condition was evaluated for thoracic limb lameness due to a large, firm mass medial to the left cranial scapula. Radiography revealed several large cauliflower-like mineralized masses in the craniomedial left scapula musculature, pectoral region and bilaterally in the biceps tendon sheaths. Urinalysis, haematology and serum biochemistry showed that the dog was severely anaemic, hyperphosphataemic and in chronic renal failure. The dog was euthanased and a full post mortem performed. A diagnosis of chronic renal failure with secondary hyperparathyroidism was confirmed. The mineralised masses were grossly and histopathologically consistent with a diagnosis of tumoral calcinosis. Tumoral calcinosis associated with chronic renal failure that does not involve the foot pads is rarely seen