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)

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

The pathologic changes associated with human cryptosporidiosis have not been well characterized. In this report, 15 cases of cryptosporidiosis in acquired immunodeficiency syndrome patients are described. Organisms were found in autopsy tissue specimens in 13 cases, and only in antemortem stool samples in two. Gastrointestinal/hepatobiliary distribution of organisms was as follows: small intestine (13 cases), extrahepatic bile ducts (eight), intrahepatic bile ducts (seven), large intestine (six), pancreas (five), stomach (three), and esophagus (one). At all sites, infection was usually associated with nonspecific reactive epithelial changes, architectural abnormalities such as villous flattening in the small intestine, and interstitial edema with mixed inflammatory cell infiltrates. Presence of organisms and associated mucosal injury were patchy and of variable severity in the intestine. In the biliary tract, injury was commonly diffuse and severe. Pancreatic duct injury was generally mild and often limited to hyperplastic squamous metaplasia. In late-stage acquired immunodeficiency syndrome patients with cryptosporidiosis, widespread infection in the gastrointestinal and biliary systems by this coccidian was more common and severe than previously suggested. Although the mechanisms have yet to be determined, infection usually is accompanied by pathologic changes that may be causally related to pathophysiologic abnormalities, such as diarrhea and malabsorption, and may account for other clinical manifestations of pancreatitis, cholangitis, and obstructive cholestasis.
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PMID:Cryptosporidiosis in the acquired immunodeficiency syndrome: a study of 15 autopsy cases. 174 28

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

Distal common bile duct stenosis was observed in 16 (9%) of 170 alcoholic patients admitted to a Veterans Administration Medical Center in the last five years. The following clinical and biochemical features were significantly more common (P less than 0.05) among the 16 patients with common bile duct stenosis than in 154 without: jaundice, cholangitis, hyperbilirubinemia, alkaline phosphatasemia, pancreatic calcification, and malabsorption. Surgical decompression of biliary tree was necessitated in 13 of 16 cases due to obstructive jaundice in seven, cholangitis in four, portal fibrosis in one, and persistent abdominal pain in one. The mean (+/- SE) time interval between initial serum alkaline phosphatase elevation and surgical intervention was 308 +/- 108 days. Liver histology in eight cases was remarkable for portal fibrosis in seven and biliary cirrhosis in one. These data suggest that distal common bile duct stenosis is a progressive lesion which is quite prevalent in patients with advanced pancreatic disease of alcoholic etiology.
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PMID:Prevalence and natural history of distal common bile duct stenosis in alcoholic pancreatitis. 647 79

A number of hepatobiliary tract and pancreatic disorders have been documented in patients with celiac disease. Some disorders have shared immunological or genetic factors, including chronic hepatitis, primary biliary cirrhosis and sclerosing cholangitis. Other hepatic or pancreatic pathological changes in celiac disease have been documented with severe malnutrition and malabsorption, including hepatic steatosis and pancreatic insufficiency, sometimes with pancreatic calcification. Finally, celiac disease may be associated with other very rare hepatic complications, such as hepatic T cell lymphoma.
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PMID:Hepatobiliary tract and pancreatic disorders in celiac disease. 911 4

The excision of the choledochal cyst with bile drainage through intestinal conduit is a standard operative procedure in the surgical management of choledochal cysts. During the last eight years five patients have been treated with this operation at the University Children's Hospital in Zagreb. All the patients were girls aged from two months to twelve years. The classical triad of pain, jaundice and abdominal mass was observed in only one patient, an eight-year-old girl. The only symptom in infancy was jaundice. Diagnosis was made by abdominal ultrasound, bibliography, CT scan with hepatotropic contrast and in older children by ERCP. Four cysts were type Todani I, and one cyst was Todani type II. The complete excision of the choledochal cyst with the Roux-Y jejunal conduit without antireflux valve was performed. There was neither operative morbidity nor mortality. Three months postoperatively the control ultrasonography and liver laboratory tests were without abnormalities. The routine control which followed did not show episodes of cholangitis, lithiasis, lipid malabsorption, blood clotting abnormalities or growth failure. The complete excision of the cyst with Roux-Y hepaticoenterostomy is an operative treatment with good results in infancy and childhood.
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PMID:[Choledochal cysts: surgical treatment]. 1093 33

We report a 60-year-old immunocompetent patient with chronic biliary isosporiasis who failed to respond to orally administered cotrimoxazole prophylaxis and orally administered treatment with nitazoxanide, a 5-nitrothiazole benzamide compound. Severe malabsorption was regarded as responsible for the subtherapeutic levels of nitazoxanide in plasma and bile, resulting in treatment failure. Intravenously administered cotrimoxazole stopped the shedding of Isospora belli oocysts in bile within 5 days, excluding initially suspected resistance to cotrimoxazole. Patients with malabsorption and cholangitis due to Coccidia such as Isospora belli and Cryptosporidium spp. or due to protozoa that cause microsporidiasis seem to be predisposed to fail to respond to otherwise effective treatment.
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PMID:Case report: Nitazoxanide treatment failure in chronic isosporiasis. 1150 98

Improved neonatal medical care and renal replacement technology have improved the long-term survival of patients with ARPKD. Ten-yr survival of those surviving the first year of life is reported to be 82% and is continuing to improve further. However, despite increases in overall survival and improved treatment of systemic hypertension and other complications of their renal disease, nearly 50% of survivors will develop ESRD within the first decade of life. In addition to renal pathology, patients with ARPKD develop ductal plate malformations with cystic dilation of intra- and extrahepatic bile ducts resulting in CHF and Caroli syndrome. Many patients with CHF will develop portal hypertension with resulting esophageal varices, splenomegaly, hypersplenism, protein losing enteropathy, and gastrointestinal bleeding. Management of portal hypertension may require EBL of esophageal varices or porto-systemic shunting. Complications of hepatic involvement can include ascending cholangitis, cholestasis with malabsorption of fat-soluble vitamins, and rarely benign or malignant liver tumors. Patients with ARPKD who eventually reach ESRD, and ultimately require kidney transplantation, present a unique set of complications related to their underlying hepato-biliary disease. In this review, we focus on new approaches to these challenging patients, including the indications for liver transplantation in ARPKD patients with severe chronic kidney disease awaiting kidney transplant. While survival in patients with ARPKD and isolated kidney transplant is comparable to that of age-matched pediatric patients who have received kidney transplants due to other primary renal diseases, 64-80% of the mortality occurring in ARPKD kidney transplant patients is attributed to cholangitis/sepsis, which is related to their hepato-biliary disease. Recent data demonstrate that surgical mortality among pediatric liver transplant recipients is decreased to <10% at one yr. The immunosuppressive regimen used for kidney transplant recipients is adequate for most liver transplant recipients. We therefore suggest that in a select group of ARPKD patients with recurrent cholangitis or complications of portal hypertension, combined liver-kidney transplant is a viable option. Although further study is necessary to confirm our approach, we believe that combined liver-kidney transplantation can potentially decrease overall mortality and morbidity in carefully selected ARPKD patients with ESRD and clinically significant CHF.
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PMID:New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney-liver complications. 2359 29

For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant 'autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.
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PMID:Association of Extrahepatic Manifestations with Autoimmune Hepatitis. 2664 98


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