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At least 19 recordsLinked to original sources
PubMed2002

Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum.

Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive liver disease, characterised by intermittent attacks of cholestasis, which can start at any age and last for several weeks to months. Characteristically serum GGT activity is low and normal liver structure is preserved. Progressive familial intrahepatic cholestasis (PFIC) is another liver disease, characterised by severe cholestasis, starting almost invariably before 6 months of age. All patients progress to cirrhosis, liver failure and death, unless a liver transplantation is performed. We now identified four patients who presented in childhood with recurrent attacks of cholestasis, while in the course of the disease the cholestasis gradually became permanent. Although liver biopsies performed in the early stages of the disease showed normal liver architecture, late stage biopsies revealed evident fibrosis with porto-portal septa formation. In conclusion, the disease of these patients started with the clinical and histopathological characteristics of BRIC but progressed to PFIC.

Adolescent↗
PubMed1981

Cholestasis and hepatic drug metabolism. Comparison of metabolic clearance rate of antipyrine in patients with intrahepatic or extrahepatic cholestasis.

The antipyrine metabolic clearance rate (MCR) was studied in two groups of patients with similar degrees of cholestasis and hepatic damage, but differing mechanisms of cholestasis. The plasma disappearance rate of antipyrine in 18 patients with extrahepatic cholestasis and 11 patients with intrahepatic cholestasis was compared with that of two groups of control subjects without liver disease who were matched for age. Whereas no significant difference was observed for the antipyrine MCR between patients with extrahepatic cholestasis and their controls [30.7 +/- 11.2 (SD) as against 31.6 +/- 10.0 ml/min], the antipyrine MCR was significantly lower (P less than 0.001) in the patients with intrahepatic cholestasis than in their controls (16.2 +/- 4.5 vs 37.4 +/- 17.3 ml/min). These results suggest that cholestasis per se does not change the rate of metabolism of drugs by the liver. The decrease of antipyrine MCR in patients with intrahepatic cholestasis could be due to a reduced functional parenchymal mass related to some degree of hepatic necrosis.

Adult↗
PubMed1985

Cholestasis in alcoholic chronic pancreatitis. Diagnostic value of the transaminase ratio for differentiation between extra- and intra-hepatic cholestasis.

In our long-term study of alcoholic chronic pancreatitis (median follow-up time, 10.4 years) 84 of 173 patients (48.6%) developed transient or persistent cholestasis with or without hyperbilirubinemia. We studied the discriminative value of the ASAT/ALAT ratio to differentiate intrahepatic (IHC) and extrahepatic cholestasis (EHC; due to stenosis of the distal common bile duct) in 75 of these patients. In 45 patients with persistent or recurrent cholestasis (group A) the cause of cholestasis was documented by accurate morphological methods. Thirty of the other 39 patients with transient cholestasis (group B) were classified on the basis of the overall clinical evaluation. Of 37 patients with IHC 36 had an ASAT/ALAT ratio of 1.5 or higher (one patient had normal values for both transaminases). Out of 38 patients classified as EHC 29 had an ASAT/ALAT ratio of 1.4 or lower (9 with normal transaminases being excluded). One patient with cholangitis secondary to EHC had a transient elevation of the ASAT/ALAT ratio to above 2.5. Thus our data suggest that in alcoholic chronic pancreatitis associated with cholestasis an ASAT/ALAT ratio of 1.4 or lower (or normal transaminases) usually indicates EHC. Diagnostic study and surgical decompression is mandatory in these cases if EHC persists.

Alanine Transaminase↗
PubMedDate not supplied

[Ursodeoxycholic acid treatment shortens the course of cholestasis in two patients with benign recurrent intrahepatic cholestasis].

INTRODUCTION: Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal-crecessive or sporadic disorder, characterized by repeated episodes of unexplained cholestasis followed by prolonged asymptomatic periods. CASE REPORT: We present two male patients aged 12 and 15 years old who initially presented cholestasis and who had no family history of liver or biliary disease. Typically, alkaline phosphatase and bilirubin levels were elevated, with a slight increase in transaminases and gamma-glutamyltransferase. In both patients biliary tract disease was ruled out by endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography while no significant abnormalities of the liver parenchyma were found on liver biopsy. Early treatment with ursodeoxycholic acid (UDCA) reduced the duration of the cholestasis episode compared with that of other episodes in which the patient had received other treatment or the treatment was late (1 or 2 months vs 4 months in patient 1 and 1-3 months vs 5 months in patient 2, respectively). DISCUSSION: Consensus is lacking on the treatment of BRIC although several treatment have been used. UDCA could be effective in increasing bile acid secretion and in reducing serum bilirubin levels. In the two patients described, the initial dosage was 15 mg/kg/day, which was subsequently maintained at 6 mg/kg/day, which was subsequently maintained 6 mg/kg/day until biochemical parameters returned to normal. Nevertheless, larger studies are required to confirm that UDA shortens episodes of cholestasis in BRIC.

Adolescent↗
PubMed1990

A multivariate analysis of factors predictive of parenteral nutrition-related cholestasis (TPN cholestasis) in VLBW infants.

To identify the factors predictive of development of TPN-related cholestasis, we conducted a historical cohort analysis of 62 VLBW infants who received TPN of which 17 developed cholestasis (27.4%). In the cholestatic group, the mean duration of TPN administration was significantly longer (25.7 days vs 8 days, p less than 0.001), the maximum daily amino-acids and lipids in the TPN infusate was significantly higher (amino-acids 2.25 vs 1.25 gm/kg/day, p less than 0.001; lipids 2.0 gm/kg/day vs 1.25 gm/kg/day, p less than 0.01), the duration of fasting was significantly longer (20.7 days vs 6.3 days, p less than 0.001) and the incidence of necrotizing enterocolitis was significantly higher (58.8% vs 15.5%, p 0.02). Using a linear discriminant analysis model with development of cholestasis as the dependent variable, the duration of TPN (p 0.0000) and the maximum daily amino-acid in infusate (p 0.0000) were found to be independent variables predictive of development of cholestasis. This model may serve to identify VLBW infants who will develop cholestasis during TPN.

Amino Acids↗
PubMed1986

Extrahepatic cholestasis in the dog and the differentiation of extrahepatic and intrahepatic cholestasis.

The clinical and pathological features of extrahepatic cholestasis in 19 dogs are presented. The condition was most often caused by partial or complete occlusion of the choledochal duct due to neoplastic diseases (12 cases), inflammatory processes (3 cases), or eventration and incarceration of the liver (1 case). In only three cases was the condition due to intraluminal obstruction of the choledochal duct. The anamnestic, laboratory and pathological parameters of these 19 dogs were compared with the findings in 21 dogs with intrahepatic cholestasis. Statistical analysis of these parameters indicated that the presence of acholic faeces, the degree of icterus and the level of plasma gamma GT where the only parameters of value in the clinical differentiation of extrahepatic from intrahepatic cholestasis. Histological examination of liver biopsies is a simple aid to a confident in vivo diagnosis of extrahepatic cholestasis.

Animals↗
PubMed2002

Extracorporal albumin dialysis (MARS) improves cholestasis and normalizes low apo A-I levels in a patient with benign recurrent intrahepatic cholestasis (BRIC).

The familial cholestatic diseases Benign Recurrent Intrahepatic Cholestasis (BRIC) and Progessive Familial Intrahepatic Cholestasis type 1 (PFIC1) are characterized by intermittent or permanently elevated plasma bile salt levels, therapy-resistant extreme pruritus and peculiar biochemical abnormalities including low apolipoprotein apo A-I. Previously, symptomatic improvement has been demonstrated in BRIC patients after extracorporal albumin dialysis (MARS). We hypothesized that MARS improves cholestasis, induces changes in the bile salt profile and normalizes apo A-I serum levels in BRIC. A 17-year-old-female patient with BRIC experienced an episode of cholestasis lasting for more than 6 months with extreme pruritus and diarrhoea not responding to standard therapy. During a period of five days the patient was treated 3 x 8 h with MARS. The procedures were well tolerated and resulted in reduction of plasma bile salts by 58%. The plasma bile salt profile changed into a more hydrophilic composition after MARS. Diarrhoea discontinued and the pruritus improved significantly from 9 to 4 on a subjective scale. These effects lasted 4 months until a relapse occurred. Low plasma apo A-I levels (0.52 g/l) normalized after MARS (0.98 g/l). The procedures were well tolerated. Fatigue was noted as the only transient side-effect. In conclusion, MARS may induce a long-term symptomatic improvement and decrease of cholestatic markers in BRIC. Further studies evaluating efficacy and mechanism of MARS in patients with BRIC are needed.

Adolescent↗
PubMed1979

Studies on drug-induced allergic intrahepatic cholestasis--hepatic cholestasis induced in dogs by lymphocyte culture supernatant.

When peripheral lymphocytes from patients with drug-induced allergic intrahepatic cholestasis were stimulated with a specific drug in vitro in the presence of a liver microsome fraction or soluble liver specific antigen fraction, lympholine production was seen in many cases. By the injection of culture supernatant of stimulated lymphocytes into the mesentery vein of dogs, cholestasis was induced in the liver, chiefly in the central zones of lobules. However, no cholestasis could be observed in dogs administered the supernatants of lymphocyte cultures prepared from normal individuals in the presence of drugs. Moreover, only slight swelling of the hepatocytes was observed in the liver when normal lymphocytes were stimulated with PHA-P and culture supernatant was injected into the mesentery vein of dogs. These results suggest that sensitized lymphocytes may produce a factor (or factors) by stimulation with a specific drug-carrier and this factor (or factors) causes cholestasis in the liver.

Adult↗
PubMed1976

Familial benign recurrent intrahepatic cholestasis. Interrelation with intrahepatic cholestasis of pregnancy and from oral contraceptives?

A family study was prompted by the presence of benign recurrent intrahepatic cholestasis in three members and probably in a fourth. Pruritus and/or jaundice occurred during pregnancy in nine members. Two additional members suffered from pruritus while using oral contraceptives. The literature on benign recurrent intrahepatic cholestasis since 1959 describes at least 57 cases. A familial form of this condition proved to be fairly common. There seems to be a relation to the intrahepatic cholestasis sometimes seen during pregnancy and during oral contraceptive use. The data of the family study are suggestive for an interrelation of the three types of intrahepatic cholestasis, although a common denominator remains obscure.

Adult↗
PubMed1975

[Ultrastructural-morphometric analysis of liver biopsies in patients with intrahepatic cholestasis. I. Correlations between morphometry of bile canaliculi and so-called "marker enzymes of cholestasis" (author's transl)].

Reports on correlations between the activity of so-called "marker enzymes of cholestasis" in serum and the ultrastructural changes of the liver are rare. Therefore studies of ultrastructural changes were carried out in 40 patients with intrahepatic cholestasis. In the patients' serum activity of alkaline phosphatase, bile duct alkaline phosphatase, leucine-aminopeptidase (LAP), and 5'-nucleotidase (5'-Nu) as well as the concentration of bilirubin were determined. The results showed a significant correlation between the morphometry of the bile canaliculi and the serum activity of LAP and 5'-Nu. In patients with elevated LAP, an enlargement of the bile canaliculi could be proved. An increased serum activity of 5'-Nu correlated with a higher incidence of bile canaliculi in the ultrastructural picture. The results suggest an investigation of the ultrastructure of bile canaliculi and the determination of marker enzymes of cholestasis in the serum may both contribute to the assessment of cholestatic liver disease.

Alkaline Phosphatase↗
PubMed1991

[Cholestasis and prostatic carcinoma. Description of a case and review of paraneoplastic cholestasis].

We report a patient with cholestasis associated with prostatic carcinoma. When metastasic hepatic occupation, bile tract obstruction and other causes were ruled out, cholestasis was attributed to carcinoma itself as a paraneoplastic syndrome. This possibility has been previously reported as exceptional in prostatic carcinoma and in other tumors. We review cholestasis associated with tumors and we discuss its possible etiological and pathogenetical mechanisms.

Adenocarcinoma↗
PubMed1981

Studies on intrahepatic cholestasis in drug-induced allergic hepatitis: intrahepatic cholestasis induced in the rat by the culture supernatant of activated lymphocytes.

A marked reduction in bile flow and bile acid excretion was whenever peripheral lymphocytes from patients with drug-induced allergic intrahepatic cholestasis were stimulated with a specific drug in vitro in the presence of a soluble liver-specific antigen fraction, and their culture supernatant injected into the mesenteric vein of rats. A gel filtration study of the active fraction of the supernatant that caused a reduction in bile flow, suggested that the molecular size of this active principle is similar to that of the macrophage migration inhibitory factor (MIF). Histologically, dilated bile canaliculi with decreased microvilli were observed via electron microscopy in rat liver after injection of culture supernatant. No such changes were observed in rats after injection of the supernatant of a lymphocyte culture similarly prepared from normal individuals. These results strongly suggested that sensitized lymphocytes obtained from patients with drug-induced intrahepatic cholestasis produce a factor (of factors) causing cholestasis when stimulated with a specific drug in the presence of liver-specific antigen fractions.

Animals↗
PubMed1978

A father and son with cholestasis and peripheral pulmonic stenosis: a distinct form of intrahepatic cholestasis.

We report a father and son with the syndrome of cholestasis and peripheral pulmonic stenosis. These cases demonstrate the clinical and biochemical features noted in previous reports of this entity and allows us to differentiate clearly this syndrome, with its benign course, from other more progressive forms of intrahepatic cholestasis. The vertical transmission supports a genetic etiology for this disease. Although serum bile acid levels are elevated in these patients, the individual bile acids do not display a distinctive pattern and no abnormal bile acids are identified.

Adult↗
PubMed1995

Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region.

A locus for progressive familial intrahepatic cholestasis (PFIC), also known as Byler disease, has been mapped to a 19 cM region of chromosome 18 by a search for shared segments, using patients from the Amish kindred in which the disorder was originally described. A similar liver disease, benign recurrent intrahepatic cholestasis (BRIC), recently has been mapped to the same region, suggesting that these two diseases are caused by mutations in the same gene. Although PFIC and BRIC are clinically distinct diseases, episodic attacks of jaundice and pruritus, with elevated concentrations of bile acid in serum, are seen in both disorders. In PFIC patients, these attacks result in progressive liver damage and death. The clinical and biochemical features of PFIC and BRIC are suggestive of a defect in primary bile acid secretion. The biology of bile secretion is of great interest because of its vital importance in digestion of dietary fats as well as in secretion of xenobiotics and metabolic waste products. Cloning of the gene (or genes) responsible for PFIC and BRIC will likely provide important insights into this pathway.

Alleles↗
PubMed1978

Study of the activity of antithrombin-III in latent cholestasis. (A clinico-pharmacological study of the relationship between antithrombin-III activity and steroid cholestasis).

The relationship between steroid cholestasis and antithrombin-III activity were examined in users of oral contraceptives (Infecundin or Bisecurin) and in patients receiving anabolic hormone therapy (Nerobol). The control group for the oral contraceptive users consisted of patients with spontaneous anovulation. The untreated control group consisted of healthy women in the reproductive age. The increase in antithrombin-III activity was found to be directly related to the decline of anion excretion. Latent cholestasis in itself is not associated with an increased antithrombin-III activity, nor is the activity of antithrombin-III affected by long-continued use of those anabolic steroids which produce no decrease in anion excretion.

Adult↗