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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Obstructive jaundice, pruritus, and malabsorption developed in twin brothers in infancy. Early liver biopsy specimens showed intracellular and canalicular cholestasis with normal bile ducts. By the age of 3 years, both had cirrhosis and portal hypertension. Each died during the teen years from hepatocellular carcinoma. These brothers represent the tenth reported family with familial cholestatic cirrhosis, and they are the first patients with this syndrome in whom hepatoma developed.
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PMID:Hepatoma in familial cholestatic cirrhosis of childhood: its occurrence in twin brothers. 21 1

Chronic pancreatitis has to be treated in the first line by medical means. Dietary procedures, absolute abstinence from alcohol, compensation of malabsorption and treatment of diabetes will do in most cases. Surgery is indicated in cases resistent to this form of therapy of when complications arise (cysts, persistent jaundice, portal hypertension). The patient has to be given high caloric parenteral nutrition preoperatively. Pancreato-jejunostomy with an Y shaped loop and surgical removal of various amounts of pancreatic tissue are the only efficient surgical procedures. In cases of dilatation of the pancreatic duct drainage has to be performed. Results of surgery are satisfactory in 2/3 of cases.
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PMID:[Medical and surgical management of chronic pancreatitis (author's transl)]. 100 87

It is reported on the estimation of the vitamin A absorption test in 17 patients with chronic liver diseases, 26 patients with diseases of the extrahepatic biliary system, and in 12 patients with diseases of the stomach. In the latter there are no changes in the vitamin A test. On the contrary patients with diseases of the liver and biliary system show differences in the serum vitamin A levels. A separate consideration of active liver cirrhosis and active cholangitis indicates individualities in the constellation of the 3 and 6 hour values after vitamin A ingestion: in liver cirrhosis the first value is normal while the 6 hour value seems significant lowered so that the maximum vitamin A concentration is found near 3 hours. In active cholangitis both values are reduced, the first one more than the second one. The reason is to be seen in a decrease of the conjugated bile acids, whereas in the group of liver diseases an interpretation is somewhat problematic. It is to discuss whether these results depend on the development of portal hypertension, while the intestinal absorption seems normal and liver damage only of secondary importance. The fact that this test is influenced by diseases of the liver and biliary system shortens its significance as a screening test for malabsorption or maldigestion though a further differentiation between sprue and pancreatic insufficiency seems possible.
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PMID:[Vitamin A absorption test. II. Diseases of the liver, the extrahepatic biliary system and the stomach (author's transl)]. 118 79

Cirrhotic patients often present muscle and adipose tissue depletion as well as reduced visceral protein concentration. Impaired absorption of nutrients may contribute to this altered nutritional status. To verify this hypothesis fecal fat excretion, intestinal mucosal function evaluated by means of the combined sugar oral load test, and intestinal clearance of alpha-1-Antirypsin were studied in 25 cirrhotic patients with clinical and biochemical signs of liver insufficiency and with portal hypertension. About 50% of the patients showed clinical evidence of malnutrition. Three of the 12 well-nourished, and 8 of the 13 malnourished patients presented significant steatorrhoea. Cirrhotics showed no impairment in mediated malabsorption and in passive permeability, as plasma D-xylose/3-O-methyl-glucose concentration and urinary lactulose/L-rhamnose excretion ratios were within the normal range. An increased value of alpha-1-Antitrypsin clearance was found only in two patients. These findings indicate that fat malabsorption is frequent in cirrhotic patients, particularly when malnourished, and does not depend on the presence of mucosal intestinal damage.
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PMID:Malabsorption and nutritional abnormalities in patients with liver cirrhosis. 213 41

Jaundice in the pediatric patient requires prompt and directed evaluation. This dictum is highlighted in infants with biliary atresia, in whom the progressive sclerosing process results in complete obliteration of patent but microscopic hilar biliary structures by 4 months of age. Kasai's operation, if done before that time, will re-establish bile drainage in 90% of infants. One fourth to one third of patients achieve long-term jaundice-free survival. Complications of cholangitis, portal hypertension, and fat malabsorption are experienced by many patients. In children with early or late operative failure, liver replacement now offers legitimate hope for extended survival. Choledochal cyst is a conglomerate of pancreaticobiliary anomalies consisting of a choledochal cyst, a common-channel-type pancreaticobiliary junction, intrahepatic cystic disease, and partial obstruction of the distal common bile duct. Many patients have one or more of these malformations. It is now widely accepted that the preferred treatment of choledochal cyst is total excision of the diseased biliary duct with reconstruction by Roux-en-Y choledochojejunostomy. "Internal" excision avoids injury to other structures in the hepatoduodenal ligament, particularly if pericystic inflammation is present. Congenital perforation of the common bile duct responds in most cases to simple peritoneal drainage of the perforation. Retention of the tube cholecystostomy is useful for subsequent cholangiographic follow-up. Tube cholecystostomy may also be useful for irrigation of the biliary tract in infants with inspissated bile syndrome.
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PMID:Congenital biliary tract disease. 224 22

The development of biliary strictures simulating sclerosing cholangitis is now well-known. We report a patient undergoing intraarterial chemotherapy for metastatic colon cancer of the liver in whom segmental intrahepatic biliary strictures occurred remote from known metastases. The patient remains alive 3-4 years after documented liver metastases with severe progressive portal hypertension, hypersplenism, and fat malabsorption. A table of cases so far reported is given.
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PMID:Sclerosing cholangitis from intraarterial floxuridine. 294 3

An infant boy with severe portal hypertension developed failure to thrive and malabsorption. Clinical findings and ultrasonography suggested the presence of an arteriovenous shunt, and hepatic angiography confirmed the diagnosis and outlined the anatomy of the vascular malformation, which was an intrahepatic arterioportal fistula. Ligature of the hepatic artery gave excellent results as demonstrated by disappearance of portal hypertension and malabsorption and subsequent growth catch-up.
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PMID:Portal hypertension secondary to congenital arterioportal fistula. 332 40

Zinc absorption was examined in 25 nonalcoholic cirrhotic patients using the oral zinc tolerance test and comparing results to a healthy control group. With 22.5 mg elementary zinc, the increase in plasma zinc was significantly lower in the cirrhotic patients than in the control group with P less than 0.01 in the first and second hours and P less than 0.05 in the fourth hour. The zinc malabsorption may result from an abnormal small intestinal mucosa. Indeed small intestinal biopsies in all patients showed partial shortening and prominent distension of villi and intense stromal edema with inflammatory cell infiltration of the lamina propria. However, it is not clear whether these intestinal changes are due to zinc deficiency or to portal hypertension. Thus zinc malabsorption appears to contribute to zinc deficiency in nonalcoholic cirrhotics and seems to result, in part, from pathological changes in the mucosa.
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PMID:Zinc plasma levels after oral zinc tolerance test in nonalcoholic cirrhosis. 340 96

Three hundred and sixteen patients with cystic fibrosis were seen at the Brompton Hospital during 1965-83; 178 (56.3%) of them were male and 136 female, and their ages ranged from 12 to 51 years. Most patients presented in infancy with respiratory symptoms and malabsorption, but 19 (6%) were diagnosed in adult life, three in their 30s. Pulmonary disease was almost universal (99.7%), being responsible for 97% of all deaths and three quarters of hospital admissions. All patients had developed a productive cough by the age of 21 and over half before the age of 5. Many complained of wheezing, but reversible airflow obstruction was present in only 40% of those tested. Minor haemoptysis was very common (62%), but major episodes less so (10%). Pneumothorax was seen in 61 cases (19%), and was often recurrent. Some irreversible airflow obstruction was present in all patients with pulmonary disease. Two patients have been followed for over 20 years without showing appreciable decline in lung function. Thirty five patients (11%) had no symptoms of malabsorption. Acute meconium ileus equivalent was seen in 16% and a chronic partial obstruction with episodic symptoms in a further 19%. Diabetes mellitus developed in 36 patients, 13 of whom were insulin dependent. Hepatomegaly was common (29%), often occurring without abnormal results in biochemical tests of liver function; only 1% of patients developed portal hypertension with varices and ascites. Skin reactions to at least one common allergen, including Aspergillus fumigatus, were positive in 70%, but very few patients suffered from hay fever or eczema. One hundred and twenty one patients have died, 97% from infection or other pulmonary complications, and 195 were alive in December 1983 (mean age 23 years). Seventy eight per cent of patients were in full time education or full or part time employment, or were housewives, and only 41 were unemployed for reasons for health. Many patients are married and 10 women have borne children. Most patients were admitted to hospital only three or four times during the period of follow up and 50 individuals (16%) have never been in hospital at all. The improvement in prognosis and quality of life for adults with cystic fibrosis should encourage a positive attitude in those who care for them.
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PMID:Cystic fibrosis in adolescents and adults. 343 96

Idiopathic neonatal hepatitis is one of the more important causes of neonatal cholestasis. It is regarded one of the clinical presentations of 'idiopathic obstructive cholangiopathy', just like extrahepatic biliary atresia. Is it not possible to discriminate between intrahepatic and extrahepatic causes of neonatal cholestasis, or between idiopathic neonatal hepatitis and metabolic, infectious, or toxic causes, by using clinical or laboratory parameters. Liver histology is slightly more helpful: giant cell formation, focal liver necrosis, and lymphocytic and neutrophilic infiltration may be found in idiopathic neonatal hepatitis. In infectious hepatitis liver pathology mostly is only a lesser part of the symptomatology. Sporadic idiopathic neonatal hepatitis has a better prognosis than familial; about 75% of children with sporadic hepatitis experience complete recovery as compared to less than 25% of children with familial hepatitis. Therapy is confined to the prevention and treatment of complications such as itching, portal hypertension and variceal bleeding, and (fat) malabsorption.
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PMID:[Idiopathic neonatal hepatitis]. 812 25


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