1/71. Excessive nodular hyperplasia of brunner glands associated with gastric hypersecretion and lipomatous atrophy of the pancreas.The case of a 34-year-old woman complaining of diarrhoea and abdominal pain is presented. Contrast radiography and endoscopy showed multiple polypoid tumours in the second part of the duodenum. Moreover, a severe fatty infiltration of the pancreas was shown by magnetic resonance and computed tomography scans. Due to pain, pancreatoduodenectomy (Whipple operation) was performed, and subsequent histopathologic examinations showed excessive Brunner gland hyperplasia of the duodenum and severe lipomatous atrophy of the pancreas. The occurrence of these two rare conditions in one patient has not been described previously, and it is conceivable that the lipomatous atrophy and exocrine insufficiency of the pancreas may have caused a compensatory stimulation of the submucosal structures of the duodenum.- - - - - - - - - - ranking = 1keywords = gland, insufficiency (Clic here for more details about this article) |
2/71. The development of hypertrophic pyloric stenosis in a patient with prostaglandin-induced foveolar hyperplasia.BACKGROUND: Hypertrophic pyloric stenosis (HPS) has been described in association with several obstructive antropyloric lesions including idiopathic foveolar hyperplasia (gastric mucosal hypertrophy), feeding tubes, eosinophilic gastroenteritis, and hypertrophic antral polyps. Non obstructive antral webs have also been described with HPS. PATIENT AND methods: We present a case of gastric-outlet obstruction in association with HPS, namely, prostaglandin-induced foveolar hyperplasia. This entity has been previously described, but rarely in association with HPS. We report a female infant requiring prostaglandin therapy for pulmonary atresia who developed dose-related prostaglandin-induced foveolar hyperplasia and symptoms of progressive non-bilious vomiting. RESULTS: Initially, ultrasonography demonstrated evidence of antral mucosal hypertrophy as the cause for gastric-outlet obstruction. The patient subsequently developed progressive thickening of the antropyloric muscle, resulting in sonographic appearances of hypertrophic pyloric stenosis. Pyloromyotomy was eventually required for treatment of HPS. CONCLUSION: A common denominator of most of the above-described entities is thickening and/or hypertrophy of the antral mucosa. We suggest that the antropyloric musculature may hypertrophy in an effort to overcome the gastric-outlet obstruction caused by the adjacent thickened antral mucosa. In other words, these entities may represent examples of "secondary" hypertrophic pyloric stenosis.- - - - - - - - - - ranking = 1.2268021305952keywords = gland (Clic here for more details about this article) |
3/71. Proportion of cells with paternal 11p15 uniparental disomy correlates with organ enlargement in Wiedemann-beckwith syndrome."Genetic mosaicism" describes the presence of two or more populations of cells within a single individual that differ in their genomic constitution. Although the occurrence of asymmetric overgrowth in Wiedemann-Beckwith syndrome (WBS) suggests that mosaicism has some role in the WBS phenotype, no direct evidence for this has been published. WBS is a congenital overgrowth syndrome with variable phenotype linked to the imprinted gene cluster on chromosome region 11p15. We have performed a molecular survey of multiple organs and tissues in a case of WBS with a high degree of mosaic paternal 11p15 uniparental disomy (UPD). The organs most severely affected were those with the highest percentage of cells with UPD. In particular there was a striking difference in the degree of mosaicism for 11p15 UPD between the extremely enlarged left adrenal and non-enlarged right adrenal gland. This result indicates that the proportion of paternal 11p15 UPD cells correlates with the tissue phenotype of WBS. Our results suggest that high proportions of abnormal cells result from a combination of stochastic events and cell selection. mosaicism may explain the variable phenotypes including hemihyperplasia and predisposition to childhood cancers in WBS patients.- - - - - - - - - - ranking = 4.4043553948742keywords = adrenal, adrenal gland, gland (Clic here for more details about this article) |
4/71. A child with adrenocortical adenoma accompanied by congenital hemihypertrophy: report of a case.We report herein the findings of a 7-year-old male child with a ruptured adrenocortical adenoma and congenital hemihypertrophy which was incidentally detected after suffering a trauma. A review of 21 pediatric cases of adrenocortical neoplasms in the literature was made. The patient showed precocious puberty such as pubis and advanced bone age, but an endocrinological examination revealed no definite abnormalities. The right adrenal tumor with hematoma was resected after these evaluations. adrenocortical adenoma is considered to occur more frequently in female children. However, the incidence of adrenocortical tumors accompanied by congenital hemihypertrophy does not differ between males and females. The outcomes were relatively good, although the observation periods were short in some patients. A large number of patients presented with a tumor and hemihypertrophy on the same side. This finding is of interest when considering the possible association between hemihypertrophy of the organs and tumor proliferation. However, their association in terms of development was unclear. It is necessary for patients with hemihypertrophy to have regular examinations for the possible development of malignant tumors, especially in the kidney, adrenal gland, and liver.- - - - - - - - - - ranking = 4.4043553948742keywords = adrenal, adrenal gland, gland (Clic here for more details about this article) |
5/71. Hydrops of placental stem villi complicated with fetal congenital adrenal hyperplasia.The authors present a case report of hydrops of placental stem villi. Numerous small aechoic spaces were demonstrated by prenatal ultrasonography. The patient spontaneously delivered a female newborn at 26 weeks' gestation. The infant showed hypertrophied clitoris and urogenital sius, and had a normal 46, XX karyotype. Endocrinological examination revealed that 3beta-hydroxysteroid dehydrogenase deficiency caused the anomaly. To our knowledge, this is the first report that congenital genital malformation complicated the placental mesenchymal dysplasia.- - - - - - - - - - ranking = 7.4040092735782keywords = adrenal (Clic here for more details about this article) |
6/71. Disseminated tuberculosis causing bilateral adrenal enlargement and Addison's disease.The clinical features and computed tomography imaging of a patient with acute adrenal failure following disseminated tuberculosis is described.- - - - - - - - - - ranking = 9.2550115919727keywords = adrenal (Clic here for more details about this article) |
7/71. adult-onset nesidioblastosis causing hypoglycemia: an important clinical entity and continuing treatment dilemma.HYPOTHESIS: nesidioblastosis is an important cause of adult hyperinsulinemic hypoglycemia, and control of this disorder can often be obtained with a 70% distal pancreatectomy. DESIGN: The records of all adult patients operated on for hypoglycemia between 1974 and 1999 were reviewed retrospectively. patients with the pathologic diagnosis of nesidioblastosis were contacted for follow-up (1.5-21 years) and are presented. patients' results were compared with those of 36 other individuals with this disorder who were previously reported in the literature. SETTING: The University of chicago Medical Center (chicago, Ill), a tertiary care facility. patients: A consecutive sample of all patients operated on for hypoglycemia. INTERVENTIONS: Seventy percent distal pancreatectomy for all patients with nesidioblastosis, and maintenance therapy with verapamil hydrochloride for 2 patients. MAIN OUTCOME MEASURES: achievement of normoglycemia with and without medication, development of insulin-dependent diabetes mellitus, pancreatic exocrine insufficiency, and need for reoperation. RESULTS: Of 32 adult patients who underwent surgical exploration for hyperinsulinemic hypoglycemia at our institution, 27 (84%) were found to have 1 or more insulinomas, and 5 (16%) were diagnosed with nesidioblastosis. Each patient with nesidioblastosis underwent a 70% distal pancreatectomy. Follow-up duration for the 5 patients ranged from 1.5 to 21 years, with 3 patients (60%) asymptomatic and taking no medications, and 2 patients (40%) experiencing some recurrences of hypoglycemia. The 2 patients with recurrences are now successfully treated with a calcium channel blocker, an approach, to our knowledge, never before reported for adult-onset nesidioblastosis. CONCLUSIONS: nesidioblastosis is an uncommon but clinically important cause of hypoglycemia in the adult population, and must always be considered in a patient with a presumptive preoperative diagnosis of insulinoma. This study indicates that a 70% distal pancreatectomy is often successful in controlling hypoglycemia, and rarely results in diabetes mellitus. However, the optimal treatment of this disorder remains to be determined.- - - - - - - - - - ranking = 0.12371276386056keywords = insufficiency (Clic here for more details about this article) |
8/71. Adenomatoid hyperplasia of the palate mimicking clinically as a salivary gland tumor.This report describes an illustrative case of adenomatoid hyperplasia (AH) of the minor salivary glands on the palate of a 31-year-old man. The clinical features of the present lesion corresponded with those of pleomorphic adenoma, but histologically large lobules of normal-appearing mucous acini were found. The cell proliferative activity demonstrated in histological sections, by an immunohistochemical staining of proliferating cell nuclear antigen and Ki-67, showed no statistically significant differences among AH and a matched control group of normal palatal salivary glands. This case suggests that AH apparently exhibits an idiopathic, focal hypertrophic lesion of intraoral mucous glands with limited growth potential.- - - - - - - - - - ranking = 1.2268021305952keywords = gland (Clic here for more details about this article) |
9/71. Hypertrophic non-union of the first rib causing thoracic outlet syndrome: a case report.We experienced a rare case of thoracic outlet syndrome caused by hypertrophic nonunion of the first rib. A diagnosis was made mainly upon provocative tests and imaging studies. Pain and tingling could be reproduced and the radial pulse obliterated by the hyperabduction test. Abundant callus formation on the posterior aspect of the first rib with fracture line was visible on plain radiograph. Two-dimensional computed tomography showed right thoracic outlet narrowing mainly caused by the mass-effect of the callus. Dynamic arteriographic studies revealed an external compression of the right subclavian artery and duplex ultrasonography demonstrated a reduction in right subclavian artery blood flow when the shoulder is in 90 degrees of abduction. Surgery was performed after the conservative management for three months which failed to relieve the patient of his complaints. Resection of the first rib via transaxillary approach was undergone uneventfully in combination with the myotomy of the scalenus anticus muscle. At postoperative one year follow up, the patient was free of symptoms, and had a full range of motion of the right shoulder with no evidence of arterial insufficiency.- - - - - - - - - - ranking = 0.12371276386056keywords = insufficiency (Clic here for more details about this article) |
10/71. Severe progressive form of congenital muscular dystrophy with calf pseudohypertrophy, macroglossia and respiratory insufficiency.A novel form of congenital muscular dystrophy in four unrelated patients is proposed. Congenital hypotonia, markedly increased CK, calf pseudohypertrophy and proximal weakness were common early findings. Two cases were severely affected since infancy and never walked. The phenotypical homogeneity was not very evident until advanced stages of the disease. All the patients showed catastrophic progression of the weakness, severe restrictive respiratory insufficiency, macroglossia, peculiar extreme amyotrophy of hands and feet, and a round and 'puffy' face. All patients became tetraplegic and required mechanical ventilation. Two cases had signs of mild cardiac involvement. The only non-tracheotomised patient died of respiratory complications. No mental retardation or specific brain abnormalities were observed. All patients showed secondary deficit of laminin 2 and up-regulation of laminin 5 in muscle. Expression of -dystroglycan was severely reduced in two available muscle samples. The known loci for congenital muscular dystrophies were excluded in the only consanguineous case by linkage analysis. Clinical, immunohistochemical and genetic findings strongly suggest a distinct entity.- - - - - - - - - - ranking = 0.61856381930279keywords = insufficiency (Clic here for more details about this article) |
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