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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Iron-chelating treatment is indicated in all children on prolonged transfusion therapy (i.e., chiefly patients with thalassemia and Blackfan-Diamond anemia). The purpose of iron-chelating treatment is to prevent the development of manifestations of iron overload including cardiac hemosiderosis and insulin-dependent diabetes mellitus (which are two potentially fatal complications), hepatic cirrhosis, hypoparathyroidism, hypothyroidism, and delayed puberty. Deferoxamine is the only effective iron-chelating agent and should be given in a daily dose of 40 mg/kg at initiation of the transfusion program. Administration is by subcutaneous infusions from 8 to 10 hours per day. The goal of iron-chelating treatment is to maintain serum ferritin levels between 500 and 1,000 ng/ml. This long-term treatment is a significant burden for patients and it can be hoped that non-toxic iron-chelating agents, active by mouth, will become available.
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PMID:[Iron chelation in children]. 268 51

Abnormal liver tests, right upper quadrant pain and hepatomegaly occurring in an obese or in a diabetic patient may point to the presence of fat or of glycogen accumulation in the liver parenchymal cells. Marked hepatomegaly due to cytoplasmic glycogen deposition is mainly found in poorly controlled insulin-dependent diabetic patients. If accompanied by cushingoid features, growth retardation and by delayed puberty, a diagnosis of Mauriac syndrome can be made. Hyperglycaemia, insulin administration and increased concentrations of the counterregulatory hormone cortisol may all play a role in the glycogen deposition by their concerted actions on the glycogen phosphorylase and synthase enzymes, promoting the accumulation of glycogen. Hypercortisolism may be responsible for growth retardation and delayed puberty in Mauriac patients. Regression of hepatomegaly and of the associated clinical characteristics may be obtained by a better metabolic control due to the administration of long-acting insulin and the change from single to twice daily injections. Fatty liver is rare in insulin-dependent diabetic patients and is indicative of a poor diabetic control. This process is quickly reversible by adequate insulin treatment. Steatosis is frequently found in maturity-onset diabetics and in obese patients. The pathogenetic mechanisms leading to the accumulation of triglycerides and of fatty acids in the hepatocytes can easily be understood from the normal cycling of fatty acids between the adiopose tissue and the liver. Histologic features of nonalcoholic steatohepatitis can also be found in obese and in diabetic patients. Steatohepatitis may rarely evolve into cirrhosis. In general, there is no correlation between the degree of the biochemical alterations and the severity of the histological findings.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver disturbances in obesity and diabetes mellitus. 858 Oct 74

The etiology of hyperandrogenic amenorrhea in a 16 year-old girl after a successful Kasai operation for biliary atresia was unclear. Delayed puberty and menarche were observed. There was no luteinizing hormone-follicular stimulating hormone surge. A provocative luteinizing hormone-releasing hormone test showed a normal response. Peripheral aromatization of androgens appeared to function normally. However, plasma levels of sex hormone-binding globulin and total testosterone were high and the free testosterone level was normal. The anovulatory menstrual cycle continued after menarche at 18 years of age. A combination of estrogen and progesterone therapy was effective. The etiology might be associated with delayed metabolic clearance of testosterone accompanied by the compensatory cirrhosis and portosystemic shunt.
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PMID:Biliary atresia with hyperandrogenic amenorrhea: case report. 1066 56

The etiology of hyperandrogenic amenorrhea in a patient with biliary atresia successfully treated by a Kasai operation was unclear. Delayed puberty and menarche were evident at 16 years of age. Investigations showed no luteinizing hormone (LH)-follicle-stimulating hormone surge. A LH-releasing hormone provocative test showed a normal response. Peripheral aromatization of androgens appeared to function normally. Free testosterone (T) was normal, however, plasma levels of sex-hormone-binding globulin and total T were high. After menarche at 18 years of age, anovulatory menstrual cycles continued. A combination of estrogen and progesterone therapy was effective. A possible explanation may be that metabolic clearance of T is reduced in the presence of liver cirrhosis and a portosystemic shunt.
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PMID:Biliary atresia with hyperandrogenic amenorrhea. 1131 91

Present in 85-90% of patients, pancreatic exocrine insufficiency contributes to growth retardation and delayed puberty in the child, and low weight in the adult. The incidence of hepatic disease varies according to whether one considers the discovery of histological abnormalities at autopsy of patients who have died from other complications (20-70%) or the presence of a focal or multilobular biliary cirrhosis complicated or not by portal hypertension (2-10%). Gastro-oesophageal reflux can contribute to the degradation of the nutritional state and exacerbate the respiratory symptomatology. All deviations of anthropometric parameters (weight/height ratio, body mass index) from standard references are a warning sign and justify nutritional assistance, of which one distinguishes 3 successive stages by chronological order: fractionated oral supplementation, nasogastric enteral nutrition and parenteral nutrition.
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PMID:[Digestive diseases and nutrition in cystic fibrosis]. 1266 46