Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient with the painless onset of a cholecystocolonic fistula associated with virtually complete common bile duct obstruction due to stones provided a unique opportunity to assess the consequences of prolonged bile acid depletion on the digestion and absorption of nutrients. Over 2 years, the patient insidiously developed steatorrhea, osteomalacia with an atraumatic pelvic fracture, and congestive heart failure complicated by polymorphic ventricular tachycardia (torsade de pointes) all of which could be attributed to malabsorption of fat and fat-soluble vitamins.
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PMID:Cholecystocolonic fistula: malabsorptive consequences of lost bile acids. 232 84

Neonatal cholestatic disturbances are frequent in premature neonates. An important role in the group of causes which may induce cholestasis is that of hypoplasia of the intrahepatic biliary pathways. Ten cases are described, of prematurely born infants with hypoplasia of the intrahepatic biliary pathways. The children had 980-1,900 g at birth. The diagnosis was made on the basis of the clinical picture, of conjugated hyperbilirubinemia, and of the necropsy findings in 7 of the cases. The incidence and the lethality were high in very small premature infants, in whom compensation responses of the hepatic tissue are rather a particular case. In all the cases the authors noted the presence of malabsorption syndromes, of atrophic enteritis, and a defect in the growth rate.
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PMID:[Hypoplasia of the intrahepatic biliary tract in premature infants]. 251 16

A 41-yr-old woman with primary biliary cirrhosis developed weakness and wasting in proximal muscles, areflexia, and decreased proprioceptive and vibratory sensation. Investigations revealed law serum levels of vitamin E and electromyographic and muscle biopsy changes consistent with a neuropathy. Sural nerve histology demonstrated axonal dystrophy with patchy demyelination. These features closely resemble a neurologic syndrome associated with chronic cholestatic liver disease and vitamin E deficiency in children. Adults with chronic cholestasis may also be susceptible to neurologic damage from prolonged malabsorption of vitamin E.
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PMID:Neurologic syndrome associated with low levels of vitamin E in primary biliary cirrhosis. 301 81

The role of the liver as a contributory factor in the vitamin D deficiency of cholestatic liver disease has been studied in vivo in dogs with chronic bile duct ligation, whereas controls underwent diversion of the bile flow through the urinary bladder via a choledococystostomy anastomosis. The hepatic extraction of vitamin D3 was evaluated by the multiple indicator dilution technique, and the formation of 25-hydroxyvitamin D3 was assessed by directly sampling the hepatic effluent for up to 150 min after vitamin D3 administration. The serum and hemodynamic data indicate that dogs with chronic bile duct ligation had severe cholestasis and hepatocellular injury; histologically, macronodular cirrhosis was present. Dogs with choledococystostomy anastomosis had normal livers and normal liver function. The data indicate that the absence of normal bile flow into the intestinal lumen led to a progressive depletion of vitamin D reserve in both animals with choledococystostomy anastomosis and those with chronic bile duct ligation. However, neither the hepatic fractional extraction of vitamin D3, its hepatic clearance nor its transformation into 25-hydroxyvitamin D3 was significantly changed by chronic bile duct ligation. The results of the present studies indicate that the hepatic handling of vitamin D3 including its C-25 hydroxylation, is well preserved in the presence of severe cholestasis. They also suggest that the state of vitamin D depletion which often accompanies chronic cholestatic liver disease can largely be accounted for by factors such as secondary malabsorption of the vitamin due to the absence of adequate amounts of bile salts in the intestinal lumen, or by other factors which seem independent of the hepatic metabolism of vitamin D.
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PMID:Severe cholestasis leads to vitamin D depletion without perturbing its C-25 hydroxylation in the dog. 319 71

Despite the recent advent of total parenteral nutrition (TPN), the long-term survival of the patients with short gut syndrome is not satisfactory with complications of catheter-related sepsis and cholestasis. The causes of short gut syndrome in pediatric surgery are multiple intestinal atresias, necrotizing enterocolitis (NEC), and midgut volvulus. For the multiple atresias, multiple anastomosis without resection has been successfully undertaken to avoid developing short gut syndrome for the last 17 years. For the massive involvement of the intestines due to NEC or volvulus, high jejunostomy and peritoneal drainage with TPN support have been tried for the last two patients and successfully weaned from the TPN within two months after closure of jejunostomy. However, in case 2 malabsorption has been persistent presumably due to a severe degree of mucosal damage occurring in the remaining intestines. Whether this eventually gets back to the normal or not is unknown. For the patients with short gut syndrome, small bowel reversal procedure was successfully done and now doing well 10 years after surgery. This paper reports details of these 3 cases.
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PMID:[Surgical managements of massive involvement of small bowel and of short gut syndrome]. 322 90

Four cases of extrahepatic biliary atresia are reported. Each of these infants presented with haemorrhagic phenomena rather than with prolonged jaundice. The increased bleeding tendency was due to a vitamin K deficiency, probably caused by cholestasis-induced malabsorption. Therefore extrahepatic biliary atresia should be considered in each infant with a bleeding diathesis.
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PMID:Bleeding as the first symptom of extrahepatic biliary atresia. 349 31

A prospective study was undertaken to evaluate fat malabsorption during intrahepatic cholestasis of pregnancy (ICP), a disease characterized by a mild cholestasis of short duration appearing in otherwise healthy young women. An abnormal fecal fat excretion (mean 15.8 g/24 h, range 6-31 g/24 h) was demonstrated in 10 of 12 patients with the icteric form of ICP and in 2 of 11 patients with pruritus gravidarum. The increased fecal fat excretion was generally asymptomatic, could be detected as early as 3 wk after the clinical onset of ICP, remained stable during the affected pregnancies, and returned to normal from 3 to 9 wk after delivery. Steatorrhea correlated with the severity of ICP, estimated by serum levels of bilirubin, total bile salts, and glutamic pyruvic transaminase. A significant fall in the maternal weight/height index was detected after the onset of ICP, being more intense in patients with steatorrhea than in those without it (to 92.6% +/- 3.0% of initial values versus 96.7% +/- 2.8%, respectively; p less than 0.05). A high risk of premature deliveries and fetal distress was demonstrated in these patients, also correlating with the severity of ICP. No direct relationship could be established between steatorrhea or maternal nutritional impairment and fetal prognosis.
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PMID:Steatorrhea in patients with intrahepatic cholestasis of pregnancy. 360 67

The serum levels of apolipoprotein A-IV (apo A-IV) were measured by rocket immunoelectrophoresis in disease-free humans, at fasting and after oral and intravenous fat administration. The studies were extended to patients with chronic pancreatitis, malabsorption syndrome, to postoperative patients on total parenteral nutrition and to patients with liver diseases, cholestasis, diabetes mellitus and chronic renal failure. Oral fat ingestion resulted in an increase of apo A-IV levels which remained elevated even when the postprandial hypertriglyceridemia had disappeared. A transient increase in apo A-IV levels was observed after intravenous fat infusion but the level declined simultaneously with decreases in triglyceride levels. Levels of serum apo A-IV were decreased under conditions where decreased fat intake or malabsorption of nutrients might have been present, such as in patients with chronic pancreatitis, malabsorption syndrome, acute hepatitis in the early stage, obstructive jaundice and in postoperative patients on total parenteral nutrition. On the other hand, the apo A-IV levels were high in patients with chronic renal failure and in those with diabetes mellitus and proteinuria.
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PMID:Alterations in plasma levels of apolipoprotein A-IV in various clinical entities. 378 Nov 71

Although secondary vitamin E deficiency causes a reversible neurologic disorder in children with chronic cholestasis, the effect of this deficiency state on other organ systems is unknown. We studied the effects of vitamin E therapy on selected gastrointestinal and hepatic functions in five children with chronic cholestasis and well-documented biochemical and neurologic evidence of vitamin E deficiency. After 2 to 3 years of oral or parenteral vitamin E therapy, there was no improvement in fecal fat losses, severity of vitamin E malabsorption (as measured by an oral vitamin E tolerance test) or total serum fatty acid concentrations. Serial analyses of liver function blood tests demonstrated a marked decline in fasting serum cholylglycine concentrations during 18 to 31 months of vitamin E therapy, while other liver function tests showed no consistent changes. We conclude that vitamin E deficiency does not appear to alter intestinal absorption of fat or vitamin E; however, vitamin E deficiency may further impair already compromised hepatic function during pathologic conditions such as cholestasis.
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PMID:Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function. 379 2

A male born to first cousins presented at 12 months with hypocalcemic convulsions, rickets, epistaxis due to vitamin K deficiency, and extremely low serum levels of beta-carotene and vitamin A. Liver function was altered moderately (glutamic-oxaloacetic transaminase, 55 U/L; glutamic-pyruvic transaminase, 37 U/L; lactate dehydrogenase, 255 U/L; alkaline phosphatase, 437 U/L). To correct the deficiencies, 8,000 IU vitamin D/day, 10,000 IU vitamin A/day, and intramuscular administration of vitamin K1 were required. At 9 years, he presented signs of neuromuscular affection, and the serum vitamin E level (measured for the first time) was extremely low. Classic lipid malabsorption syndromes (abetalipoproteinemia, chronic cholestasis, mucoviscidosis, coeliac disease, Whipple's disease) were excluded by appropriate examinations. Composition of duodenal bile acids was characterized by undetectable levels of cholic acid metabolites, and only chenodeoxycholic acid metabolites were present. Serum total bile acid concentration was normal, with an atypical low cholic acid/chenodeoxycholic acid ratio and abnormal presence of 3 beta-OH-delta 5-cholenic acid and 6-OH-bile acids. Urinary bile acid composition was also characterized by elevated 6-OH-bile acids. Known enzymopathies of the bile acid synthetic pathway were excluded (cerebrotendinous xanthomatosis, cerebro-hepato-renal syndrome of Zellweger, coprostanic acidemia). Bile acid pool sizes were determined by using stable isotopes: cholic acid pool size [2.90 (N, 32 +/- 16) microM/kg] and chenodeoxycholic acid pool size [10.8 (N, 32.6 +/- 9.9) microM/kg] were extremely low; fractional turnover rates of both bile acids were in a normal range.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Malabsorption of liposoluble vitamins in a child with bile acid deficiency. 379 31


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