Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011849 (diabetes)
 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five infants, three dying neonatally and two later in the first year of life, had renal, hepatic, and pancreatic dysplasia, a combination of abnormalities first described by Ivemark et al [1959]. The renal malformation consisted of cystic dysplasia, with abnormally differentiated ducts, deficient nephron differentiation, and glomerular cysts. The hepatic abnormality consisted of enlarged portal areas containing numerous elongated biliary "profiles," with a tendency to perilobular fibrosis. Serial liver biopsies in one child with cholestasis from birth showed a progression from bile duct paucity at 1 1/2 wk to typical biliary "dysgenesis" at 7 mo. Four of the five children had intrahepatic ductal dilatation, diagnosed ante mortem in the two older children as Caroli disease. The pancreatic abnormality consisted of fibrosis and cysts, with a diminution of parenchymal tissue. The clinical and functional reflection of these abnormalities in the two children surviving the newborn period included renal insufficiency, chronic jaundice, and insulin-dependent diabetes mellitus. Similar renal, hepatic, and pancreatic abnormalities occur in other syndromes, including trisomy 9, Meckel syndrome, Jeune, Saldino-Noonan, and Elejalde types of chondrodysplasia, and glutaric aciduria II. After exclusion of identifiable syndromes, the remaining cases of renal-hepatic-pancreatic dysplasia do not necessarily constitute a homogeneous group.
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PMID:Renal-hepatic-pancreatic dysplasia: a syndrome reconsidered. 381 91

Nineteen children underwent subtotal pancreatectomy for hyperinsulinemic hypoglycemia. Of the four who were older than 10 years of age at onset of symptoms, three had islet adenomas, and one had endocrine cell dysplasia (ECD). The two patients with multiple adenomas had a family history of multiple endocrine neoplasia, type 1 (MEN 1). Of the 15 who were younger than 1 year of age at onset of symptoms, 12 had ECD alone, and three had ECD with adenomatosis. Four children required a second surgical procedure and near-total pancreatectomy because of persistent hypoglycemia. All 19 patients' conditions improved, with no postsurgical complications. After near-total pancreatectomy, all four patients were treated for fat malabsorption, but only two required insulin because of secondary diabetes mellitus. We concluded that subtotal pancreatectomy is a safe, effective adjunct to the treatment of children with hyperinsulinemic hypoglycemia.
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PMID:Surgical management of hyperinsulinemic hypoglycemia in children. 614 50

A series of 108 prospectively collected human pancreata were evaluated histologically for the presence of acinar cell and ductal lesions. Foci of dysplastic acinar cells were present in 44% of the series. Some of the focal acinar cell abnormalities were similar to atypical acinar cell nodules which have been described in carcinogen-treated experimental animals. The incidence of nodules was higher among patients with a history of heavy cigarette smoking than among nonsmokers, and among patients with a history of alcohol abuse than among abstainers. The presence of dysplastic acinar cell nodules in a pancreas seemed unrelated to the presence of cancer in other sites, or diabetes. Ductal epithelial abnormalities were more frequent than focal acinar cell dysplasia.
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PMID:Focal acinar cell dysplasia in human pancreas. 624 10

The syndrome of caudal dysplasia (CDS) and the wide spectrum of associated skeletal and other anomalies are reviewed, and a further case of this disorder is presented. The syndrome of CDS should be distinguished from the familial forms of sacral dysgenesis, three forms of which are tentatively identified. Two of these usually involve some degree of "hemi-sacrum." The third is usually manifested as partial sacral agenesis with absent distal segments. All these familial types are probably genetic dominants, and none is associated with maternal diabetes. Usually CDS is not familial, but it often is associated with a tendency toward diabetes in the mother. The suggestion is advanced here that CDS is the result of a combination of two principal factors represented by (a) a maternal diabetic tendency and (b) separate nondiabetogenic genes. Determination of the human leucocyte antigen (HLA) haplotypes involved in CDS is suggested to investigate the possibility of genetically distinctive factors in this condition.
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PMID:The syndrome of caudal dysplasia: a review, including etiologic considerations and evidence of heterogeneity. 639 99

We have reviewed ten children who underwent surgical therapy for persistent neonatal hypoglycemia over a 5-year period. All had inappropriately high insulin levels in the face of hypoglycemia, and all failed medical management with intravenous glucose, frequent feeds, diazoxide and glucagon. Two groups of five patients each were analysed retrospectively. Group 1 underwent 95% pancreatectomy, leaving a small amount of pancreatic tissue on the duodenum and common bile duct. The only major complication in this group was in one patient with common duct obstruction requiring choledochoduodenostomy. All these children are developing normally, without diabetes, steatorrhea, or recurrent hypoglycemia. Group 2 underwent 85% pancreatectomy, leaving the uncinate process in situ. Two of these children are well. Two required conversion to 95% resection because of recurrent hypoglycemia; one of these required a subsequent total pancreatectomy, at which time the pancreatic remnant had significantly regenerated. The other Group II patient was normoglycemic but died at age 3 from pneumonia. Pathology in nine cases showed islet cell dysplasia; 5 of these also had microadenomatosis. One case had a histologically normal pancreas. We conclude that 95% pancreatectomy is a safe operation with a lower failure rate than less radical resections, and should be used early in the management of this condition.
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PMID:Surgical management of persistent neonatal hypoglycemia due to islet cell dysplasia. 639 33

Female CBA mice made diabetic with a single intravenous dose of streptozotocin (STZ) were either grafted with cultured fetal mouse pancreatic islets onto the splenic capsule, treated with insulin, or left untreated. An age- and sex-matched group of nondiabetic mice served as normal controls. All islets-grafted and most insulin-treated mice survived and had normal fasting blood glucose levels. By contrast, of the untreated diabetic mice, one died and the survivors showed poor weight gain. Light-microscopic examination of the islet isografts showed a progressive increase in graft size and beta-cell granulation over the 9-month study period. Quantitative electron-microscopic examination of the kidney showed that, whereas the islet-grafted and nondiabetic control mice had similar glomerular capillary basement membrane (GCBM) thickness, the untreated diabetic and insulin-treated mice had markedly thickened GCBM. All STZ-treated mice develop diffuse hepatic dysplasia and, at later time points, some showed biliary hyperplasia, intrahepatic cysts, and occasionally nodular dysplasia. With increasing time after STZ, most mice developed renal adenomas. One untreated diabetic mouse also developed a solitary functional pancreatic beta-cell adenoma. STZ effects were not affected by treatment of diabetes.
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PMID:Long-term isografts of cultured fetal mouse pancreatic islets. The oncogenic effects of streptozotocin and the prevention of diabetic renal complications. 645 32

From March 1960 through January 1968, 71 patients underwent operations for renovascular hypertension at our center. There were three operative deaths in 94 procedures. Primary nephrectomy was performed in 26 patients. Attempted revascularization of 62 kidneys was successful in 46 (74%). In 13 (87%) of the 15 cases considered operative failures, the patients underwent either secondary nephrectomy (11) or repeat revascularization (two). Based on the results of the final operation, initial blood pressure response (1 to 6 months postoperatively) in the surviving patients indicated 44% cured (30 patients), 40% improved (27), and 16% unchanged (11). The sequential clinical, functional, and anatomic follow-up evaluations to time of death or to date are available in 66 of the 68 patients (97%) who survived operation and form the basis of this report. Fifteen- to 20-year arteriographic follow-up in 16 patients revealed one late neointimal anastomotic stenosis and an additional three aortic suture line false aneurysms in Dacron aortorenal grafts. During this 15- to 23-year follow-up, 71% of atherosclerotic (AS) patients and 23% of fibromuscular dysplasia (FMD) patients died. Cardiovascular (CV) morbid events occurred in 77% of AS patients and in 19% of FMD patients. The cumulative incidence of death and CV morbid events during follow-up is examined by Kaplan-Meier life tables and Cox's proportional hazards regression analysis in these respective groups to identify preoperative markers predictive of longer event-free survival in relation to blood pressure benefit by operation (for example, focal vs. diffuse AS, presence of cerebrovascular disease, ischemic heart disease, left ventricular hypertrophy seen by electrocardiography, azotemia, smoking, diabetes, and hyperlipidemia).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Operative management of renovascular hypertension. Results after a follow-up of fifteen to twenty-three years. 648 67

Papers from the American Journal of Ophthalmology, Archives of Ophthalmology, and British Journal of Ophthalmology from July 1977 to June 1978 were selected on the basis of potential value to clinical optometrists. The 54 papers pertaining to various aspects of retinal pathology are presented under these headings--damage from fiber optics light, preretinal membranes, acute posterior multifocal placoid pigment epitheliopathy, toxoplasmosis, histoplasmosis, diabetes, retinal pigment epithelium, retinitis pigmentosa, retinoblastoma, retrolental fibroplasia and retinal dysplasia, other retinopathies, and retinal tears and detachments.
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PMID:A selected review of retinal research and study. 677 Jun 91

We found at postmortem examination the association of bilateral renal agenesis and of apparently complete Di George syndrome in an infant whose mother was diabetic. Vertebral abnormalities and hallux duplication were present as well. There is a correlation between maternal diabetes and the bilateral renal agenesis-caudal dysplasia complex on one hand, and maternal diabetes and cardiac malformations on the other hand. Moreover, it has been suggested that the absence of thymus and parathyroids in Di George syndrome is causally linked to the cardiac malformation. Therefore we suggest that the association in our case is not coincidental; both sets of malformations may be due to the maternal diabetes.
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PMID:Association of bilateral renal agenesis and Di George syndrome in an infant of a diabetic mother. 689 97

The association of the Albright syndrome (polyostotic fibrous dysplasia of bone, hyperpigmented skin macules, and endocrine disorders) with acromegaly has been infrequently substantiated. The case of an 18-year-old girl with the classic Albright syndrome and acromegaly is described. The patient had a history of coarsening of acral and facial features, an insulin-resistant form of diabetes mellitus and elevated fasting growth hormone values. Neuro-endocrine studies demonstrated failure of growth hormone to suppress to less than 5 ng/ml during an oral glucose tolerance test, and the abnormal release of growth hormone upon injection of thyrotropin-releasing hormone. Although L-dopa failed to decrease growth hormone levels, bromocriptine produced a modest decline in growth hormone within two hours of ingestion. The patient had also experienced secondary amenorrhea with sub-normal follicle-stimulating-hormone (FSH) and luteinizing hormone (LH) levels, both of which demonstrated a prolonged sluggish response to an injection of gonadotropin-releasing hormone (GnRH); this response suggested hypogonadotropic hypogonadism, possibly on the basis of a tumor involving both pituitary and hypothalamus. Sellar polytomography demonstrated an enlarged sella with dorsal erosion and an asymmetric floor. Computerized tomography of the brain visualized a suprasellar mass extending into the hypothalamus. These findings suggest a hypersecretion of hypothalamic releasing factors, pituitary hormones, or both as an etiology for the endocrinopathy in this patient, and lend support to the theory that the endocrinopathies associated with the Albright syndrome result from over-production of hypothalamic-releasing hormones or autonomous secretion of pituitary hormones from an adenoma.
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PMID:The Albright syndrome associated with acromegaly: report of a case and review of the literature. 701 31


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