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)

Seven women, mean age 47.7 years, with primary biliary cirrhosis (6 patients in the II-III stage and I patient in IV stage of the disease) were treated in the course of 16 months with ursodeoxycholic acid (Ursofalk) 500 mg daily. At the end of the 3-d month of treatment the itching had passed in 3 of the patients and in the remaining 4 patients it had substantially decreased. In all patients the subjective complaints, dyspeptic syndrome, appetite and sleep improved. The serum concentrations of bilirubin, copper and cholesterol started to decrease and the serum activity of the enzymes alkaline phosphatase, ALAT and ASAT also decreased. In one patient the treatment was discontinued in the 6-th month because of allergic reaction. After 16 month treatment in the 6 patients who completed the treatment the itching passed and the working capacity improved. The serum concentrations of bilirubin, cholesterol, copper and IgG significantly fell (p less than 0.01), the serum activity of alkaline phosphatase, gamma glutamyl transpeptidase, ALAT and ASAT fell near the upper normal range. The hepatomegaly, splenomegaly, McLagan's flocculation test, serum concentration of IgM and the titer of the specific antimitochondrial antibodies (M2) did not change in spite of the treatment. The results show the ursodeoxycholic acid as a perspective therapeutic means for primary biliary cirrhosis which lowers or overcomes the syndrome of intrahepatic cholestasis and limits the activity of the cirrhotic process in the liver. Ursodeoxycholic acid is well tolerated.
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PMID:[The treatment of primary biliary liver cirrhosis with ursodeoxycholic acid (preliminary report)]. 177 66

To investigate the effect of extrahepatic cholestasis on integrity of the inner mitochondrial membrane, a study was conducted on two groups of rats: sham-operated control animals (N = 10) and rats subjected to extrahepatic cholestasis (EHC, N = 10) by double ligation of the hepatic duct. The animals were observed for 7 days and then sacrificed. The EHC group presented significantly higher serum levels of alanine aminotransferase, total bilirubins and alkaline phosphatase than the controls (P less than 0.01). Basal mitochondrial respiration (state IV), analyzed separately using either alpha-ketoglutarate or alpha-ketoglutarate + pyruvate as substrates, was similar in the two groups (P greater than 0.01). ADP-activated respiration, state III, diminished significantly in the EHC group. The results show that the decrease in mitochondrial function that has been reported by several investigators to occur in EHC is due to mitochondrial alterations not related to the ability of these organelles to maintain the proton gradient, since the inner mitochondrial membrane continued to be energized throughout the observation period.
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PMID:Integrity of liver inner mitochondrial membrane in rats with extrahepatic cholestasis. 182 30

Sonography revealed multiple echo-poor lesions in the liver of a 51-year-old man with nonspecific symptoms (fatigue, drop in performance, pressure sensation in the upper abdomen), increased blood sedimentation rate (68/110 mm) and evidence of cholestasis (gamma-GT 126 U/l, alkaline phosphatase 444 U/l, leucine-aminopeptidase 64 U/l). Under the diagnosis of liver metastases the primary tumour was looked for. These investigations and a fine-needle biopsy having proved unsuccessful, laparoscopy was performed. The biopsies so obtained showed whitish yellow, tight elastic structures indicating gummas of the liver in tertiary syphilis. Treponema-specific IgM antibodies in serum characterized active syphilis requiring treatment. Administration of antibiotics (penicillin 1 mega U daily i.m.; because of allergy replaced after four days by erythromycin 2 g daily for six weeks) resulted in complete normalization of all biochemical findings and, some time later, regression of the gummas. The patient has now been symptom-free for three years. This case illustrates the need even to-day of including syphilis in the differential diagnosis of unclear space-occupying lesions of the liver.
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PMID:[Tertiary syphilis with liver gummata]. 182 7

The medical records and liver biopsies of nine sickle cell patients with chronically elevated liver function tests were retrospectively reviewed to determine the etiology of chronic liver disease. There were eight women and one man with a mean age of 30 yr. All patients had hemoglobin SS. Eight patients were referred for elevated aminotransferases and one for an elevated alkaline phosphatase. Hemosiderosis was present in all of the biopsies. Two patients had cirrhosis. Chronic hepatitis was noted in two patients, and five patients had cholestasis. Two patients had serologic markers demonstrating HBV exposure but no patients were HBsAg positive. Erythrophagocytosis, sinusoidal dilatation, and Kupffer cell hyperplasia were present in all of the liver biopsies. Transfusion-related causes were the most common significant pathologic findings in our patients, and appeared to be the etiologies of chronic liver disease in sickle cell patients.
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PMID:Transfusion-related chronic liver disease in sickle cell anemia. 188 2

In a pilot study 5 females with primary biliary cirrhosis (PBC), histological stages I-III, were treated with methotrexate (7.5-15.0 mg by mouth weekly) for 15 months. Pruritus and fatigue decreased in 3 patients and cholestyramine could be reduced or discontinued. The concentration of alkaline phosphatase decreased significantly until the 6th month of treatment (P less than 0.002), but only after the methotrexate dosage had been increased to 15 mg weekly. However, the improvement in cholestasis parameters persisted until the end of the period of observation in only 3 patients in stages I and II. In only one case, initially in stage III with increased serum bilirubin concentration of 3.5 mg/dl, was there a change in the histological stage, to stage IV, after treatment. These preliminary results indicate that methotrexate can influence the symptoms and cholestasis enzymes in the early stages of PBC. Controlled studies should therefore only be conducted on anicteric patients in an early stage of the disease.
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PMID:[Methotrexate in the therapy of primary biliary cirrhosis]. 188 74

In samples collected from 170 dogs suspected of having hepatobiliary disease, preprandial serum bile acids (PRSBA) and postprandial serum bile acids (POSBA) concentrations were measured, using a spectrophotometric enzymatic method. Dogs were assigned to 8 disease groups and 1 control group on the basis of hepatic histopathologic findings. Pre- and postprandial SBA concentrations and results of routine biochemical analyses (including total bilirubin, albumin, and BUN concentrations, and serum alkaline phosphatase (ALP), alanine transaminase (ALT), and aspartate transaminase (AST) activities) were expressed, using 4 indices: sensitivity, specificity, positive predictive value, and negative predictive value. Single tests and combinations of tests in series were evaluated. For diagnosis of hepatobiliary disease, the specificity of PRSBA was 100% at values greater than 20 mumol/L and of POSBA was 100% at values greater than 25 mumol/L. Test combinations with the best sensitivity for diagnosing the following diseases were: PRSBA-POSBA for cirrhosis, portosystemic vascular anomaly, and glucocorticoid hepatopathy; PRSBA-POSBA or PRSBA-ALP for cholestasis; PRSBA-POSBA or ALT-AST for chronic hepatitis; PRSBA-ALT for hepatic necrosis and passive congestion; and PRSBA-ALP for neoplasia. Test combinations with the overall highest sensitivity and positive predictive value for the fewest number of tests were PRSBA-POSBA, and either PRSBA or POSBA combined with an enzyme activity (ALT, AST, or ALP). The overall test efficacy for PRSBA vs POSBA was nearly identical: for PRSBA, it was 82.4%, and for POSBA, it was 82.3%. On the basis of the results of this study, PRSBA greater than 20 mumol/L or POSBA greater than 25 mumol/L (measured by use of an enzymatic procedure) indicates histopathologic abnormalities of the hepatobiliary system or portosystemic vascular anastomosis. Seemingly, determination of SBA concentrations can be used to indicate the propriety for hepatic biopsy. Pre- and postprandial serum bile acids concentrations should be evaluated in conjunction with routinely used hepatobiliary screening tests for best diagnostic advantage.
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PMID:Evaluation of twelve-hour preprandial and two-hour postprandial serum bile acids concentrations for diagnosis of hepatobiliary disease in dogs. 189 31

A 58-year-old man was admitted to hospital because of exertional dyspnoea and a cardiac murmur not previously heard. For one year he was known to have left-sided fibrinous pleuritis. For 5 years he had been taking methysergide for cluster headaches. From 1950-1980 he had worked with asbestos-containing insulating material. Erythrocyte sedimentation rate was greatly increased to 117/136 mm and there was an hypochromic anaemia (Hb 10.4 g/dl). The cholestasis enzymes were elevated (gamma-GT 88 U/l; alkaline phosphatase 511 U/l). Computed tomography of the thorax demonstrated left-sided pleural thickening of up to 3 cm. Endocarditis was excluded (sterile blood culture; normal echocardiogram). There was no evidence of an infectious, immunological or malignant cause for the pleural fibrosis. One year after pleurectomy and having discontinued methysergide all biochemical tests were normal.
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PMID:[Pleural fibrosis as a side effect of years-long methysergide therapy]. 189 55

Protein energy malnutrition is a common complication in cholestatic children in a hepatic transplant program, and may be detrimental to the postoperative outcome. Improvement of the nutritional status may be of obvious importance to improve the prognosis. This study compared oral nutrition with oral nutrition supplemented with nocturnal enteral feeding in children with prolonged cholestasis. In six children with prolonged cholestasis (conjugated bilirubin over 25 mg/L and/or GGT over 110 IU/L in infants aged less than 3 months or over 50 IU/L in older infants and/or alkaline phosphatase over 500 IU/L, for more than 3 months), we compared a 4 to 6 month period with oral nutrition and similar periods with 10 to 12 h nocturnal enteral feeding given at home as an energetic supplement. Energy intake during the second period was 180-200% of recommended dietary allowances. No ascites was found in the six patients during the study period. The Z scores of body weight, weight expressed as percent of ideal body weight (IBW), weight/height2, and arm circumference/head circumference were calculated at the beginning and at the end of each period. With only oral nutrition, a diminution in percentage of ideal W/H and a diminution in Z score for the body weight were observed in five of six patients. At the end of the second period, the average of all of the nutritional indexes was increased and the Z score for the body weight was also increased in four of six patients. Significant statistical differences (p less than 0.05) were found in W as percentage of IBW and the Z score for log W/H2.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Improvement of nutritional status in cholestatic children with supplemental nocturnal enteral nutrition. 190 35

Hepatic allograft rejection is presently classified into acute and chronic rejection based on histological features, timing and reversibility. However, because features of both types of rejection can occur at any time, and in many combinations, the terms "acute" and "chronic" seem inappropriate in some instances. Thus the term "cellular rejection" better defines the histological features of portal hepatitis, nonsuppurative destructive cholangitis and endotheliitis, which are independent of time and response to therapy. Similarly, because progressive bile duct destruction leading to a decrease in the number of interlobular and septal bile ducts is the major histological feature of "chronic rejection," the term "ductopenic rejection," defined as the loss of bile ducts in 50% or more of portal tracts independent of time and reversibility, seems more appropriate. The pathogenesis of cell-mediated rejection has not been completely explained; however, direct immunocytic attack on small bile ducts and small arteries appear to be the major feature. The process may lead to bile duct loss ("ductopenia"). The pathogenetic role of foam-cell arteritis resulting in ischemic bile duct injury and the role of humoral mechanisms in causing ductopenic rejection awaits further clarification. In the past, irreversible ductopenic rejection occurred in approximately 10% of all patients who underwent their first liver transplantation; this figure, however, appears to be decreasing. The clinical features of irreversible rejection include persistent and progressive cholestasis; rising serum levels of bilirubin, alkaline phosphatase and gamma-glutamyltransferase; and a decrease in hepatic synthetic function. Ductopenic rejection can occur early (2 to 5 wk after liver transplantation) but most often develops between 6 wk and 6 mo after transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Current concepts in cell-mediated hepatic allograft rejection leading to ductopenia and liver failure. 191 76

Over the period of two weeks a 19-year-old man developed gradually increasing painless jaundice with dark urine and light-coloured soft stools (6-7 times daily), as well as loss of appetite, nausea and nagging itch. Biochemical tests indicated marked cholestasis (alkaline phosphatase 800 U/l, gamma-GT 206 U/l). Abdominal ultrasound examination revealed high-grade stenosis of the distal choledochal duct caused by an enlargement of the head of the pancreas and computed tomography confirmed a tumour in this location. Endoscopic retrograde cholangiopancreatography demonstrated filiform stenosis of the major pancreatic duct and prepapillary stenosis of the choledochal duct. Several needle biopsies failed to establish a definitive diagnosis. A Whipple operation was performed: the stomach was preserved but about 40% of pancreatic tissue resected. Histologically there was chronic suppurative pancreatitis of the head of the pancreas. The patient was symptom-free 6 months after the operation. The case illustrates that it is not always possible in a painless pancreatic tumour to distinguish between pancreatitis and malignant tumour.
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PMID:[Chronic purulent, draining, indolent pancreatic head pancreatitis with extrahepatic cholestasis]. 193 34


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