Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0008370 (cholestasis)
 9,378 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Report of a 10-year-old boy with congenital hypoplasia of the intrahepatic bile ducts, the socalled MacMahon-Thannhauser-Syndrome. The patient had been suffering from a varying degree of jaundice since his 2nd day of life and from pruritus since his 21st month of life. Furthermore, he had hepatomegaly, a systolic cardiac murmur, hypogenitalism, retarded growth, and finally hypertension. Transitory xanthomas existed between 1 3/4 and 2 3/4 years of age. Signs of persistent intrahepatic cholestasis was manifested by increased levels of bilirubin and bile acids in serum as well as raised activities of leucine aminopeptidase, gamma-glutamyl transpeptidase and alkaline phosphatase. Pathological values of serum glutamic dehydrogenase pointed to a persistent destruction of liver cells. Without treatment, the activities of vitamin K dependent clotting factors were decreased. Cholesterol, phosphatides and triglycerides in serum were increased and lipoprotein-X was detectable. Aortography revealed stenosis of both renal arteries. An exploratory laparotomy and 5 liver biopsies led to the diagnosis of hypoplasia of the intrahepatic bile ducts. Therapeutic trials with steroids and the anion exchange resin "cholestyramine" were ineffective. Phenobarbital relieved the pruritus. Parenteral administration of fat soluble vitamins restored the activity of vitamin K dependent clotting factors to normal. The high blood pressure fell significantly due to treatment with adelphan. The etiology of hypoplasia of the intrahepatic bile ducts is unknown. It may be a malformation or an obliteration secondary to inflammation. In our patient, narrowing of the renal arteries, increase of plasma-renin activity and hypertension were probably secondary to hyperlipidemia. It has been suggested that hyperlipemia secondary to cholestasis may be due to a disturbance of lipoprotein metabolism. A review of reports on 118 patients suffering from intrahepatic bile ducts hypoplasia is included.
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PMID:[Hypertension and bilateral stenosis of the renal artery associated with congenital hypoplasia of the intrahepatic bile ducts (author's transl)]. 124 84

Aluminum remains a significant contaminant of total parenteral nutrition (TPN) solutions and may be elevated in bone, urine, and plasma of infants receiving TPN. Aluminum accumulation in tissues of uremic patients and adult TPN patients has been associated with low-turnover bone disease. Furthermore, aluminum has also been linked with encephalopathy and anemia in uremic patients and with hepatic cholestasis in experimental animals. Because of the toxic effects of aluminum, the Food and Drug Administration (FDA) recently published a notice of intent to set an upper limit of 25 micrograms/L for aluminum in large-volume parenterals and to require manufacturers of small-volume parenterals, such as calcium and phosphate salts, to measure aluminum content and note this content on the package label. The ASCN/A.S.P.E.N. Working Group on Standards for Aluminum Content of Parenteral Nutrition Solutions supports these intentions and further urges the FDA to require that cumulative aluminum intake in terms of safe, unsafe, and toxic quantities of aluminum per kilogram be made known to physicians and pharmacists preparing the TPN solutions, to ensure that manufacturers use appropriate control procedures in aluminum measurements, and to employ a standard unit of aluminum measurement.
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PMID:Parenteral drug products containing aluminum as an ingredient or a contaminant: response to Food and Drug Administration notice of intent and request for information. ASCN/A.S.P.E.N. Working Group on Standards for Aluminum Content of Parenteral Nutrition Solutions. 173 29

Parenteral S-adenosylmethionine proved to be effective in reversing intrahepatic cholestasis in pregnant women. Based on these findings, a prospective multicenter, double-blind, placebo-controlled trial was planned to assess whether oral S-adenosylmethionine is effective in cholestatic patients with chronic liver disease. Accordingly, 220 inpatients (26% chronic active hepatitis, 68% cirrhosis, 6% primary biliary cirrhosis) with stable (1 month or more) at least twofold increases in serum total and conjugated bilirubin and alkaline phosphatase volunteered for the trial. Serum markers of cholestasis significantly (P less than 0.01) decreased after oral S-adenosylmethionine administration (1600 mg/day), and their values were significantly (P less than 0.01) lower than the corresponding values in the placebo group. S-adenosylmethionine significantly (P less than 0.01) improved subjective symptoms such as pruritus, fatigue, and feeling of being unwell, whereas placebo was ineffective. Two patients in the S-adenosylmethionine group and 9 controls (P less than 0.05) withdrew from the trial for reduced compliance because of inefficacy of treatment. Oral S-adenosylmethionine was tolerated to the same extent as placebo. In conclusion, short-term administration of oral S-adenosylmethionine is more effective than placebo in improving clinical and laboratory measures of intrahepatic cholestasis and offers a new therapeutic modality for the symptomatic management of this syndrome.
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PMID:Oral S-adenosylmethionine in the symptomatic treatment of intrahepatic cholestasis. A double-blind, placebo-controlled study. 218 71

Parenteral nutrition associated cholestasis is a condition that challenges even the most astute clinician. The risk:benefit ratio of parenteral nutrition must be individualized for each neonate. The dilemma is based on weighing the risk of progressive cholestasis and its complications against the risk of starvation, malnutrition, and their consequences. Avoiding excessive nutrient infusion and providing even minimal enteral calories may prevent or mitigate cholestasis. Routine monitoring of hepatic function in all neonates receiving parenteral nutrition allows early detection and intervention. Affected infants must be evaluated for treatable causes of neonatal cholestasis. Parenteral nutrition related cholestasis remains a diagnosis of exclusion. Further research is needed to unravel the cause and to define the long-term consequences of parenteral nutrition associated cholestasis.
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PMID:Parenteral nutrition and hepatobiliary dysfunction. 308 61

Hepatobiliary scans using Tc-IDA are reliable in making the diagnosis of acute cholecystitis. Commonly, opioid drugs are administered in patients with acute cholecystitis to relieve pain. Opioid drugs cause biliary sphincter spasm. Whether these drugs adversely affect hepatobiliary scans is unknown. We studied 13 healthy volunteer subjects, performing three hepatobiliary scans in each one. Scans were performed without opioid drugs and 30 minutes after intramuscularly administered meperidine, morphine, hydroxyzine, hydroxyzine plus meperidine, butorphanol, and nalbuphine. Opioid drugs markedly delayed clearance of Tc-IDA from the common bile duct, simulating common bile duct obstruction. Hydroxyzine alone caused an insignificant delay. We have concluded that opioid drugs cause bile duct obstruction in healthy persons. If opioid drugs are administered before a diagnostic hepatobiliary scan, delayed clearance of Tc-IDA from the common bile duct might lead to an erroneous diagnosis and indicate a potentially unnecessary common bile duct exploration. Opioid drugs should not be administered for several hours before a diagnostic hepatobiliary scan.
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PMID:Opioid drugs cause bile duct obstruction during hepatobiliary scans. 653 76

Three Japanese patients with familial progressive intrahepatic cholestasis developed complications involving neurologic abnormalities characterized by ataxia and pigmentary retinopathy. Serum vitamin E concentrations were extremely low in all patients, suggesting a long-term vitamin E deficiency. High dose oral supplementation of alpha-tocopherol produced normal serum vitamin E levels in two patients. Parenteral administration of vitamin E resulted in no clinical improvement in one patient who first received the treatment at 14 years of age. In the other two patients, the progression of neurological abnormalities was slowed by vitamin E supplementation. Cholestyramine treatment resulted in an apparent decrease in serum vitamin E levels despite oral alpha-tocopherol supplementation.
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PMID:Familial intrahepatic cholestasis associated with progressive neuromuscular disease and vitamin E deficiency. 673 83

Daily renewed composition of parenteral nutrition for premature and full-term newborn infants in intensive care is time consuming and prone to inherent calculation errors. We developed a knowledge based system, VIE-PNN (Vienna Expert System for Calculating Parenteral Nutrition of Neonates) for calculating the proposed composition of parenteral nutrition on the basis of the calculating algorithm used at our neonatal intensive care unit. The system needs manual input on postnatal age, body weight, serum electrolytes (or normal values if not available), amount and content of additional oral feeds, venous access (peripheral or central), total amount of fluid intake, and complications such as sepsis (reduced lipid supply) or cholestasis (reduced amino acid supply). The parenteral nutrition proposal may interactively be modified by the attending physician. There are possibilities for error detection to reduce the probability of typing or calculation errors. The system was developed to run on IBM compatible PCs and has been tested clinically. We describe the problem domain, system structure, clinical evaluation of VIE-PNN and the calculation of a standard parenteral nutrition solution from the data stored in the system's database.
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PMID:[VIE-PNN: an expert system for calculating parenteral nutrition of intensive care premature and newborn infants]. 770 27

Liver chemistry was studied in fifteen patients with vitamin K deficiency in infancy (VKDI). All except 2 were exclusively breast fed and 4 of the 15 infants had received intramuscular vitamin K prophylaxis. A high incidence of hepatic dysfunction was found during long term follow-up in patients with VKDI. Abnormal aminotransferase was noted either at the time of onset (n = 6) or during the ensuing few weeks (n = 6). Cholestasis was documented in six cases at onset and another two in a later period. Most cases had increased serum alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), or bile acid levels regardless of hepatic enzymes and bilirubin levels. The abnormal enzymes returned to normal after 5 weeks to 23 months. This study demonstrates a close relationship between hepatic dysfunction and VKDI. Liver function impairment other than cholestasis may play some role in the pathogenesis of VKDI, but the cause of hepatic dysfunction can not be defined. Follow-up of liver chemistry is recommended in patients with VKDI. Parenteral vitamin K prophylaxis at birth may not give sustained protection against VKDI, especially in those with underlying liver disease.
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PMID:Liver function in patients with vitamin K deficiency in infancy. 783 84

Cholestasis is one of the most frequent complications of Total Parenteral Nutrition (TPN). Among the components of TPN, amino acids (AA) and dextrose are considered responsible in terms of quantity. The aim of this study was to determine whether IV lipids have a protective effect on the liver lesions induced by TPN, as well as on the bile flow in rats. Two groups of 6 animals, 6 receiving TPN containing AA and dextrose (Group A-D) and 6 receiving AA, dextrose and lipids (Group A-D-L) were studied. Both groups received the same amount of AA (3.4 g/day) and the same amount of energy (50 kcal/day). The TPN lasted 3 days, after which blood samples were obtained for liver function tests and the bile flow was determined gravimetrically, subsequently the liver was removed for histological analysis. A significant weight loss (p < 0.01) was observed in both groups (Group A-D: -13 +/- 4 g; Group A-D-L: -16 +/- 2g). The bile flow was significantly lowered in A-D-L at 10.14 +/- 1.27 microliters/minute when compared to Group A-D (15.61 +/- 1.31 microliters/minute). The Aspartate Amino Transferase (AST) plasma level was elevated at 188 +/- 20 UI in Group A-D and at 185 +/- 33 UI in Group A-D-L. Gamma-Glutamyl-Transferase (gamma-GT) plasma level was in the normal range in both groups. In conclusion, this study indicates that TPN-associated cholestasis is significantly increased by lipid addition. However, the hepatocellular necrosis seems unchanged.
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PMID:The addition of lipids increases the total parenteral nutrition-associated cholestasis in the rat. 810 83

Parenteral infusion of amino acid solutions is known to produce cholestasis in experimental animal models and in the isolated perfused rat liver. To characterize the dose responsiveness and reversibility of amino acid-induced cholestasis, isolated rat livers were perfused with solutions containing 1.5, 3.0, or 6.0 g of amino acids for 1 hour and allowed to recover for 30 minutes. Perfusion of livers resulted in a rapid, dose-related decrease in bile flow (p < .0001 at doses of 3.0 and 6.0 g). When the amino acid solution was discontinued, bile flow recovered to near control rates. Infusion of taurocholate reduced the magnitude of the decrease in bile flow associated with amino acid infusion but did not prevent it. Infusion of amino acid solutions was associated with the following changes in bile: (1) dose-related increases in total free amino acid concentrations; (2) increased osmolarity; (3) increased glucose concentrations; (4) increased potassium concentrations; (5) decreased chloride concentrations; (6) increased oxygen uptake in livers not perfused with added taurocholate; and (7) increased total bile acid concentrations in livers perfused with added taurocholate. Additional investigations are needed to determine whether these associations are attributable to individual amino acids or the total metabolic load of the amino acids.
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PMID:Amino acid infusions induce reversible, dose-related decreases in bile flow in the isolated rat liver. 845 21


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