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PubMed1997

A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis.

BACKGROUND/PURPOSE: The authors evaluated prospectively the utility of ultrasonography, Tc-99m-DISIDA hepatobiliary scintigraphy, and liver needle biopsy in differentiating biliary atresia (BA) from intrahepatic cholestasis in 73 consecutive infants who had cholestasis. METHODS: Sixty three ultrasonographic examinations of 61 infants with 7.0-MHz transducer were carried out, focusing on the fibrous tissue at the porta hepatis. The authors defined the triangular cord (TC) as visualization of a triangular or tubular shaped echogenic density just cranial to the portal vein bifurcation on a transverse or longitudinal scan. RESULTS: Although 17 of 20 ultrasonographic examinations from infants who had BA denoted TC, 43 ultrasonographic examinations from infants with either neonatal hepatitis (NH) or other causes of cholestasis denoted no TC, showing a diagnostic accuracy of 95% with 85% sensitivity and 100% specificity. Investigation with Tc-99m-DISIDA hepatobiliary scintigraphy showed that 24 of 25 infants who had BA had no gut excretion, and 16 of 46 infants who had either NH or other causes of cholestasis had gut excretion, showing a diagnostic accuracy of 56% with 96% sensitivity and 35% specificity. Therefore, gut excretion of tracer excluded BA, but no gut excretion of tracer needed further investigations as liver needle biopsy. Forty-four liver needle biopsies were carried out in 19 infants who had BA and 24 infants who had either NH or other causes of cholestasis. Although 18 of 20 biopsy findings in infants who had BA were correctly interpreted as having BA, 23 of 24 biopsy results in infants who had either NH or other causes of cholestasis were correctly diagnosed, showing a diagnostic accuracy of 93% with 90% sensitivity and 96% specificity. CONCLUSIONS: Since the introduction of ultrasonographic TC sign in the diagnosis of BA by our institution, we have found that it seemed to be a simple, time-saving, highly reliable, and non-invasive tool in the diagnosis of BA from other causes of cholestasis. The authors propose a new diagnostic strategy in the evaluation of infantile cholestasis with emphasis on ultrasonographic TC sign as first priority of investigations. When the TC is visualized, prompt exploratory laparotomy is mandatory without further investigations. When the TC is not visualized, hepatobiliary scintigraphy is the next step. Excretion of tracer into the small bowel actually rules out BA. Liver needle biopsy is reserved only for the infants with no excretion of tracer. The authors believe that a correct decision regarding the need for surgery can be made in almost all cases with infantile cholestasis by this multidisciplinary approach.

Algorithms↗

PubMed2000

A missense mutation in FIC1 is associated with greenland familial cholestasis.

Greenland familial cholestasis is a severe form of intrahepatic cholestasis described among indigenous Inuit families in Greenland. Patients present with jaundice, pruritus, bleeding episodes, and steatorrhea, and die in childhood due to end-stage liver disease. We investigated the possibility that Greenland familial cholestasis is caused by a mutation in FIC1, the gene defective in patients with progressive familial intrahepatic cholestasis type 1 and many cases of benign recurrent intrahepatic cholestasis. Using single-strand conformation polymorphism analysis and sequencing of the FIC1 exons, a missense mutation, 1660 G-->A (D554N), was detected and was shown to segregate with the disease in Inuit patients from Greenland and Canada. Examination of liver specimens from 3 Inuit patients homozygous for this mutation revealed bland canalicular cholestasis and, on transmission electron microscopy, coarsely granular Byler bile, as previously described in patients with progressive familial intrahepatic cholestasis type 1. These data establish Greenland familial cholestasis as a form of progressive familial intrahepatic cholestasis type 1 and further underscore the importance of unimpeded FIC1 activity for normal bile formation.

Adenosine Triphosphatases↗

PubMed1973

Alteration of bile canalicular enzymes in cholestasis. A possible cause of bile secretory failure.

Bile secretory failure (cholestasis) may result from several possible mechanisms involved in bile secretion. We have examined the possibility that abnormalities in enzyme content, composition, and turnover of liver plasma membrane constituents are altered in cholestasis. Severe and mild cholestasis were produced by 5 days of bile duct ligation and ethinyl estradiol administration, respectively. Bile duct ligation but not ethinyl estradiol treatments was associated with elevations of the serum bilirubin level and 5'-nucleotidase activity. However, basal bile flow and bilirubin transport maximum (T(m)) were significantly reduced after ethinyl estradiol treatment. Liver plasma membrane fractions rich in canalicular membranes were prepared from groups of rats in each of three categories; normal, after bile duct ligation, or ethinyl estradiol administration, and their respective controls. Electron microscopy and enzyme marker studies demonstrated plasma membrane fractions free of significant contamination. Plasma membrane fractions prepared from mild as well as severe cholestasis had increased alkaline phosphatase activity, and reduced 5'-nucleotidase and Mg(2+)-ATPase activities. Co(2+)-CMPase activity was unchanged. Kinetic analysis of 5'-nucleotidase and Mg(2+)-ATPase activities in plasma membrane fractions demonstrated reduced V(maz) (but unaltered K(m)). Reducted V(maz) was unrelated to addition in vitro of di-or trihydroxy bile salts or ethinyl estradiol and, therefore, suggests that reduced activities in cholestasis are due to decreased enzyme content. Cholestasis was not associated with changes in the synthesis or degradation rate of pulse-labeled plasma membrane proteins or alterations in the major protein bands separated on sodium dodecyl sulfate (SDS)-polyacrylamide gel electrophoresis. Plasma membrane cholesterol, phospholipid, and neutral sugar content was unaltered, but sialic acid content was significantly increased in both forms of cholestasis. Alterations in specific canalicular enzymes in two forms of cholestasis suggest that these changes may be involved in the pathogenesis of bile secretory failure, or may result from cholestasis.

Adenosine Triphosphatases↗

PubMed1974

Effect of maternal intrahepatic cholestasis on fetal steroid metabolism.

Estriol, estriol sulfate, progesterone, and 17 neutral steroid sulfates, including estriol precursors and progesterone metabolites, were determined in 27 cord plasma samples collected after pregnancies complicated by intrahepatic cholestasis of the mother. The levels of these steroids were compared with those in the cord plasma of 42 healthy controls. In the cord plasma, the steroid profile after pregnancies complicated by maternal intrahepatic cholestasis differed greatly from that seen after uncomplicated pregnancy. Two main differences were found. In the disulfate fraction, the concentrations of two pregnanediol isomers, 5alpha-pregnane-3alpha,20alpha-diol and 5beta-pregnane-3alpha,20alpha-diol, were high after cholestasis. Other investigators have shown that, as a result of cholestasis, these pregnanediol sulfates circulate in greatly elevated amounts in the maternal plasma. Our results indicate that in cholestasis these steroids cross the placenta into the fetal compartment, where they circulate in elevated amounts as disulfates. Secondly, the concentrations of several steroid sulfates known to be synthesized by the fetus were significantly lower in the cholestasis group than in the healthy controls. This was especially true of 16alpha-hydroxydehydroepiandrosterone sulfate and 16alpha-hydroxypregnenolone sulfate. These results suggest that, in pregnancies complicated by maternal intrahepatic cholestasis, impairment of fetal steroid synthesis, and especially of 16alpha-hydroxylation, occurs in the fetal compartment.Thus, the changes in maternal steroid metabolism caused by cholestasis are reflected in the steroid profile of the fetoplacental circulation. Furthermore, maternal intrahepatic cholestasis may result in the production of some substance which crosses the placenta and affects fetal steroid metabolism.

17-alpha-Hydroxypregnenolone↗

PubMedDate not supplied

[Parenteral nutrition related cholestasis: infection as the main risk factor].

Total parenteral nutrition-associated cholestasis (TPN-AC) is a clinical problem of special interest in neonates and in other age groups. This retrospective cohort study attempted to determine the incidence and the appearing time of cholestasis in Intensive Care Unit (ICU) patients beyond neonatal period, submitted to total parenteral nutrition (TPN) for more than five consecutive days, focusing on the relation of TPN-AC and severe infection. Data on one hundred and thirteen patients, aged from one month to 14 years, collected over a period of ten years, were analyzed. All patients, followed up to the TPN stop or their death, were classified in two groups: with and without cholestasis. They were evaluated in relation to cholestasis potential risk factors, such as severe infection. Cholestasis was identified by a conjugated bilirubin (DB) and total bilirubin rate equal or superior to 40% and/or a DB equal or superior to 0.5 mg/dl during the TPN laboratory monitoring. Logistic regression analysis was used to control and to adjust for the cholestasis confusion factors. The incidence of cholestasis was 18%, but it was very important in patients younger than 6 months (26% vs 12%) (p=0.06), in patients that use TPN for more than 2 weeks (25% vs 8%) (p=0.01), as well as in patients with severe infection (24% vs 3%) (p=0.02). On the average cholestasis appeared 17.6 days after TPN inception, with a range of 5 to 35 days. Control of the simultaneous effect of age, TPN duration, and severe infection has shown that in patients with less than 6 months the relative risk was 2.6 (NS); in patients with TPN longer than 2 weeks it was 3.1 (p=0.02); and in patients with severe infection it was 7.4 (p=0.006). The results suggest that TPN-AC in children beyond neonatal period is a relatively frequent event (18%), and severe infection is the most important risk factor for cholestasis.

English Abstract↗

PubMedDate not supplied

Drug-induced cholestasis.

Intrahepatic cholestasis, defined as arrested bile flow, mimics extrahepatic obstruction in its biochemical, clinical and morphological features. It may be due to hepatocyte lesions of which there are three types, termed canalicular, hepatocanalicular and hepatocellular, respectively; or it may be due to ductal lesions at the level of the cholangiole or portal or septal ducts. Defective bile flow due to hepatic lesions reflects abnormal modification of the ductular bile. Defective formation of canalicular bile may involve bile acid-dependent or independent flow. It appears to result most importantly from defective secretion of bile acid-dependent flow secondary to defective uptake from sinusoidal blood, defective transcellular transport and defective secretion; or from regurgitation of secreted bile via leaky tight junctions. An independent defect in bile acid-independent flow is less clear. Defective flow of bile along the canaliculus may reflect increased viscosity and impaired canalicular contractility secondary to injury of the pericanalicular microfibrillar network. Impaired flow beyond the canaliculus may result from ductal injury. Sites of lesions that contribute to cholestasis include the sinusoidal and canalicular plasma membrane, the pericanalicular network and the tight junction and, less certainly, microtubules and microfilaments and Golgi apparatus. A number of drugs that lead to cholestasis have been found to lead to injury at one or more of these sites. Other agents (alpha-naphthylisothiocyanate, methylenedianiline, contaminated rapeseed oil, paraquat) lead to ductal injury resulting in cholestasis. Reports of inspissated casts in ductules (benoxaprofen jaundice) and injury to the major excretory tree (5-fluorouridine after hepatic artery infusion) have led to other forms of ductal cholestasis. Most instances of drug-induced cholestasis present as acute, transient illness, although important chronic forms also occur. The clinical features include the reflection of the cholestasis (pruritus, jaundice), systemic manifestations and extrahepatic organ involvement. While nearly all classes of medicinal agents include some that can lead to cholestasis, there are differences among the various categories. Phenothiazines and related antipsychotic and 'tranquillizer' drugs characteristically lead to cholestatic hepatic injury. The tricyclic antidepressants may lead to cholestatic or hepatocellular injury.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗

PubMed2000

Cholestasis with altered structure and function of hepatocyte tight junction and decreased expression of canalicular multispecific organic anion transporter in a rat model of colitis.

Cholestasis is frequently associated with inflammatory bowel disease. Because some cholestasis is resulted from altered hepatocyte tight junctions (TJs) or the canalicular multispecific organic anion transporter, we have investigated the following topics in a rat model of inflammatory bowel disease: (1) alterations in hepatocyte TJs and in the canalicular multispecific organic anion transporter, (2) etiologic factors for cholestasis, and (3) effects of antibiotics on cholestasis. Rats with trinitrobenzene sulfonic acid-induced colitis were studied 24 hours after treatment. Hepatocyte TJs and the canalicular multispecific organic anion transporter were evaluated by immunostaining for TJ-associated proteins, 7H6 and ZO-1, and multidrug resistance protein 2 (mrp2). To investigate etiologic factors causing cholestasis, portal endotoxin and proinflammatory cytokines were examined. The effects of polymyxin B, penicillin G, or metronidazole on immunostaining for 7H6, ZO-1, mrp2, and cholestasis were investigated. (1) Immunostaining for 7H6 and ZO-1 colocalized outlining the bile canaliculi and immunostaining for mrp2 localized on the canalicular membrane in controls. Treatment with trinitrobenzene sulfonic acid induced significant cholestasis and caused translocation of immunostaining for 7H6, but not that for ZO-1, to the cytoplasm and diminished immunostaining for mrp2 on the canaliculus membrane. (2) The levels of portal endotoxin, but not proinflammatory cytokines, was increased. (3) Polymyxin B, but not the other antibiotics, prevented alterations in immunostaining for both 7H6 and mrp2, and cholestasis. We described that both hepatocyte TJs and the canalicular multispecific organic anion transporter were altered and that gut-derived endotoxin levels in the portal blood were increased in this rat colitis model.

Animals↗

PubMed1995

Early cholestasis in premature infants receiving total parenteral nutrition: a possible consequence of shock and hypoxia.

We report 18 premature infants (gestational age: 31.1 weeks +/- 2.6 [mean +/- SD] (range: 28-36]) with necrotizing enterocolitis (NEC) who developed total parenteral nutrition (TPN) associated cholestasis. Liver function tests were performed at the start of TPN (D1) and repeated once a week. Considering the date of cholestasis onset (direct bilirubin > 30 mumol/l and/or serum bile salts > 10 mumol/l), the patients can be divided in two groups. The first group consisted of 9 patients who had cholestasis at D1. In these patients shock and/or hypoxia occurred prior to D1 and were the only risk factors of cholestasis identified before D1. The second group consisted of 9 patients who developed cholestasis after D1 and in whom the cause of cholestasis was multifactorial (sepsis, lack of enteral feeding, shock and/or hypoxia). These results suggest that shock and/or hypoxia can be responsible for early cholestasis in premature infants. We conclude that shock and hypoxia should be considered when discussing TPN-associated cholestasis.

Cholestasis↗

PubMed2000

Differential expression of canalicular membrane Ca2+/Mg(2+)-ecto-ATPase in estrogen-induced and obstructive cholestasis in the rat.

Extracellular adenosine triphosphate (ATP) may regulate hepatocyte and cholangiocyte functions, and under some conditions it may have deleterious effects on bile secretion and cause cholestasis. The canalicular membrane enzyme Ca2+/Mg2+-ecto-ATPase (ecto-ATPase) hydrolyzes ATP/adenosine diphosphate (ATP/ADP) and regulates hepatic extracellular ATP concentration. Changes in liver ecto-ATPase in estrogen-induced cholestasis were examined in male rats receiving 17alpha-ethinylestradiol (E groups) for 1, 3, or 5 days (5 mg/kg/day, sc) and compared with changes in rats subjected to obstructive cholestasis (O groups) for 1, 3, or 8 days. Activity of ecto-ATPase, protein mass in canalicular membranes and bile (estimated by Western blotting), steady state mRNA levels (by Northern blotting), and cellular and acinar distributions of the enzyme (histochemistry and immunocytochemistry) were assessed in these groups. Activity of ecto-ATPase, protein mass in isolated canalicular membranes, and enzyme mRNA levels were significantly increased in E group rats as compared with controls. In contrast, these parameters were markedly decreased in O group rats, and the enzyme protein was undetectable in bile. The ecto-ATPase histochemical reaction was markedly increased in the canalicular membrane of E group rats, extending from acinar zone 2 to zone 1, whereas it decreased in the O group. The ecto-ATPase immunocytochemical reaction was present in the canalicular membrane and pericanalicular vesicles in control and E group hepatocytes, but it decreased in obstructive cholestasis and was localized only to the canalicular membrane. Thus, significant changes in liver ecto-ATPase were apparent in 17alpha-ethinylestradiol-induced cholestasis that were opposite to those observed in obstructive cholestasis. Assuming that the alterations observed in obstructive cholestasis are the result of the cholestatic phenomenon, we conclude that changes in ecto-ATPase in 17alpha-ethinylestradiol-treated rats might be either primary events or part of an adaptive response in 17alpha-ethinylestradiol-induced cholestasis.

Adenosine Triphosphatases↗

PubMed2005

Cholestasis in acute stroke: an investigation on its prevalence and etiology.

OBJECTIVE: Liver cholestatic enzymes and/or bilirubin occasionally occur in acute stroke and are usually, but not always, ascribed to comorbid conditions. We investigated the frequency and possible etiology of this phenomenon. MATERIAL AND METHODS: Prospective evaluation of post-admission biochemical cholestasis of all patients hospitalized with acute stroke was conducted during a 21-month period. RESULTS: Of 169 consecutive patients, 18 (10.7%) developed cholestasis. In 7 of the patients (4.1%; 4 M, 3 F, median age 70 years, range 57-82 years) no apparent cause of cholestasis could be found, and they were further evaluated (Group A). These patients were compared with 21 randomly selected stroke patients without cholestasis, matched for age and gender, and who acted as controls (Group B). Group A patients were in a deeper coma than the controls (Glasgow Coma Scale 3.4 +/- 0.8 versus 1.9 +/- 0.7, p < 0.001), associated with severe autonomic and hypothalamic involvement, while no such manifestations were present in Group B (p < 0.001). Cholestasis started on the 3rd-6th day and lasted up to the 11th-25th day, with maximum median levels of gamma-GT and serum alkaline phosphatase (SAP) of up to 4.38 (range 2.33-8.25) and 1.49 (range 0.63-2.56) times the upper limit of normal, respectively. Serum alanine aminotransferase (ALAT), total and direct bilirubin increased in Group A but not in Group B. The common bile duct was significantly wider in Group A than in Group B (7.7 +/- 0.5 versus 4.7 +/- 0.6 mm, p < 0.001) and within Group A during and after cholestasis (7.7 +/- 0.5 versus 4.7 +/- 0.5 mm, p < 0.001). CONCLUSIONS: Transient cholestasis may occur in 4.1% of patients following acute stroke. Cholestasis is associated with deeper coma, autonomic and hypothalamic involvement and common bile duct dilatation without obstruction, possibly related to inordinate hypertonia of the sphincter of Oddi.

Age Distribution↗

PubMed1989

Benign recurrent intrahepatic cholestasis. A report of 26 cases.

Benign recurrent intrahepatic cholestasis is characterized by attacks of cholestasis. The purpose of our study of 26 patients was to emphasize some features uncommonly or never reported in this disease: (a) in each patient, the attacks of cholestasis were stereotypic; (b) attacks of cholestasis were not associated with pruritus in 15% of our patients; (c) the occurrence of attacks of cholestasis during pregnancy or oral contraceptive use might be a fortuitous coincidence; (d) gallstones were found in several patients with benign recurrent intrahepatic cholestasis and might be present earlier than in the general population; (e) in some of our patients, during attacks of cholestasis, serum transaminases were very high, exceeding 15 times the upper limit of normal; (f) mild portal inflammatory infiltration was found in one third of our patients; (g) no treatment shortened the duration of cholestasis, and in a few patients, plasmapheresis seemed to diminish jaundice and improve biochemical disorders.

Adult↗

PubMed1999

The effects of acetylsalicylic acid, interferon-alpha, and vitamin E on prevention of parenteral nutrition-associated cholestasis: an experimental study.

BACKGROUND: Cholestasis is one of the major complications of parenteral nutrition. The purpose of this experimental study was to detect the effects of acetylsalicylic acid (ASA), vitamin E (Vit E), and interferon-alpha (IFN-alpha) on prevention of parenteral nutrition-associated cholestasis. METHODS: Ten experimental groups, each consisting of 10 4-week-old Wistar albino rats, were formed: control 10- and 20-day groups (C10 and C20), parenteral nutrition-only 10- and 20-day groups (T10 and T20), ASA-supplemented parenteral nutrition 10- and 20-day groups (TA10 and TA20), Vit E-supplemented parenteral nutrition 10- and 20-day groups (TE10 and TE20), and IFN-alpha-supplemented 10- and 20-day groups (TF10 and TF20). Acetylsalicylic acid, Vit E, and IFN-alpha were administered in the parenteral nutrition solution through an intraperitoneal route. At the end of the study, serum total bile acids, serum aspartate and alanine aminotransferases, and alkaline phosphatase were measured biochemically. In addition, the histopathologic findings of cholestasis were evaluated by using a morphologic portal inflammation index. RESULTS: Although the difference in the serum levels of transferases and alkaline phosphatase was not significant among all groups (p > 0.05), it was significant in total bile acid levels (p < 0.05). There was also a significant correlation between the histopathologic changes of the liver and serum total bile acid concentrations (p < 0.05). Portal inflammation in varying degrees was seen in all experimental groups, but not in the control groups. Serum total bile acid concentrations in parenteral nutrition groups receiving ASA were significantly lower than those in the parenteral nutrition-only group (p < 0.01). Although Vit E-supplemented parenteral nutrition was effective in preventing the development of cholestasis in the 10-day group (p < 0.05), it was not effective in the 20-day group when compared with incidence of cholestasis in the parenteral nutrition-only group (p > 0.05). Conversely, IFN-alpha-supplemented parenteral nutrition had no effect on cholestasis in the 10-day group (p > 0.05) but lowered cholestasis in the 20-day group when compared with incidence the parenteral nutrition-only group (p < 0.05). CONCLUSION: Our results indicate that acetylsalicylic acid may be beneficial in preventing, and (alpha-interferon in treating, parenteral nutrition-associated cholestasis.

Alanine Transaminase↗

PubMed2006

Plasma lipid profiles of women with intrahepatic cholestasis of pregnancy.

OBJECTIVE: Intrahepatic cholestasis of pregnancy is associated with dyslipidemia, but the gestational lipid profile in relation to clinical diagnosis of the disease is unknown. The aim of this study was to undertake a detailed analysis of plasma lipids in women presenting with intrahepatic cholestasis of pregnancy and pruritus gravidarum. METHODS: Plasma lipid concentrations were assessed in nonfasting blood samples from 63 women with intrahepatic cholestasis of pregnancy (n = 54, recruited at the time of diagnosis, and n = 9, who later developed the disease), 43 women with pruritus gravidarum, and 26 healthy pregnant controls during pregnancy and at 4-6 weeks postpartum. RESULTS: Intrahepatic cholestasis of pregnancy was associated with an abnormal lipid profile. Low-density lipoprotein (LDL) cholesterol, apolipoprotein B-100, and total cholesterol concentrations were significantly raised during pregnancy in women with intrahepatic cholestasis of pregnancy compared with pruritus gravidarum and controls, and LDL-cholesterol was raised before clinical diagnosis. High-density lipoprotein cholesterol was lower in women with intrahepatic cholestasis of pregnancy compared with the pruritus gravidarum group. Ursodeoxycholic acid did not alter plasma lipid concentrations. CONCLUSION: Intrahepatic cholestasis is associated with dyslipidemia, which may contribute to the pathogenesis of the disease. The elevation of LDL cholesterol and reduction of high-density lipoprotein cholesterol before clinical diagnosis may prove to be a useful biomarker for the early identification of intrahepatic cholestasis of pregnancy and differentiation from pruritus gravidarum. LEVEL OF EVIDENCE: II-2.

Adult↗

PubMed1970

A clinical and histochemical study of cholestasis.

A histochemical study of conjugated and total bilirubin was made on liver biopsies of 150 patients. Three types of cholestasis were defined: type I, characterized by the presence of intracellular pigment granules in the hepatocytes; type II, showing intracellular granules and extracellular thrombi (this group may be subdivided according to the eventual presence of coarse pigment deposits in the parenchymal cells); and type III, showing intracellular granules, extracellular thrombi, and also bile pigment in the Kupffer cells. The fine liver cell granules correspond mainly to conjugated bilirubin, whereas the coarse deposits usually contain unconjugated pigment. The extracellular thrombi mostly contain conjugated bilirubin; the Kupffer cell pigment is predominantly of the unconjugated type. In cholestasis types II and III a diffuse directly reacting pigment is also observed. The finding of unconjugated pigment in different locations and the eventual deconjugation by beta-glucuronidase is discussed. A correlation was found between the different types of cholestasis, the level of bilirubinaemia, and its dynamic evolution. This suggests that the proposed types of cholestasis may represent successive stages of increasing cholestasis. The type of cholestasis and the nature of the pigment are largely independent of the aetiology. It is shown that a large percentage of minor degrees of cholestasis is missed when only conventional histological methods are used. The mechanisms by which morphological cholestasis is brought about are discussed in the light of the present findings.

Bilirubin↗

PubMed1995

Fetal mortality associated with cholestasis of pregnancy and the potential benefit of therapy with ursodeoxycholic acid.

Cholestasis of pregnancy is associated with increased fetal morbidity and mortality and should be treated actively. The significance attached to pruritus in pregnancy is often minimal, but it is a cardinal symptom of cholestasis of pregnancy, which may have no other clinical features. Eight women with previous cholestasis of pregnancy were referred to The Liver Unit within a 12 month period for advice concerning future pregnancies. Thirteen pregnancies had been affected by cholestasis of pregnancy and 12 had been treated expectantly with resultant perinatal morbidity or mortality in 11 (one normal delivery), including; eight stillbirths, two premature deliveries with fetal distress (one died in perinatal period), and an emergency caesarean section for fetal distress. The other pregnancy was treated actively and delivery was uncomplicated. Subsequently, three of these cases with recurrent cholestasis of pregnancy were referred while pregnant. In each, cholestasis developed with severe pruritus, gross increase of serum bile acids, and deranged liver tests. Each was treated with the choleretic agent ursodeoxycholic acid, with rapid clinical improvement and resolution of deranged biochemistry. In conclusion, cholestasis of pregnancy continues to be treated expectantly despite its association with increased morbidity and mortality and evidence suggesting improved prognosis with active treatment and the potential of reducing the associated perinatal mortality. In an uncontrolled series of three patients with cholestasis of pregnancy, ursodeoxycholic acid seemed to provide safe and effective therapy.

Adult↗

PubMed2003

Transient cholestasis in newborn infants with perinatal asphyxia.

UNLABELLED: In asphyxiated newborn infants, cholestasis often leads to extensive investigations and a cause can rarely be found. OBJECTIVE: To assess the frequency of transient neonatal cholestasis in an unselected group of asphyxiated newborn infants in a mother-child centre. METHOD: Charts of 181 asphyxiated newborn infants born with appropriate birth weight for gestational age (AGA) or small weight for gestational age (SGA) at Sainte-Justine Hospital, Montreal, Quebec between 1989 and 1993 were reviewed. RESULTS: Transient neonatal cholestasis was found in 8.5% of asphyxiated AGA and 33% of SGA newborn infants, compared with 3.94% cholestasis of any etiology in nonasphyxiated SGA infants. Asphyxiated neonates born before the age of 35 weeks had an increased risk for transient neonatal cholestasis (odds ratio 2.84, CI 1.0-8.1) CONCLUSION: Transient neonatal cholestasis is associated with several contributing factors related to the severity of the neonatal distress. Asphyxia is frequently accompanied by cholestasis in this group of newborns and without symptoms other than uncomplicated cholestasis. Investigations should be focussed on conditions requiring immediate therapy.

Apgar Score↗

PubMed2006

Idiopathic cholestasis as a paraneoplastic phenomenon in Hodgkin's lymphoma.

Paraneoplastic cholestasis is an uncommon presenting symptom in Hodgkin's lymphoma (HL). Two distinct entities causing this clinical picture have been described: idiopathic cholestasis and vanishing bile duct syndrome (VBDS). We report a patient with idiopathic cholestasis and early-stage HL with favorable risk factors whose liver biopsy results were consistent with intrahepatic cholestasis and no ductopenia. Other causes for cholestatic jaundice were ruled out. He was treated with subtotal lymphoid irradiation and subsequently experienced a steady improvement in his liver function tests to near normal over 21 months. This case illustrates a rare paraneoplastic phenomenon, and a review of the available literature is included. We also discuss the differences between HL-related idiopathic cholestasis and VBDS. The distinction between these 2 diseases has prognostic implications: idiopathic cholestasis is usually reversible in early-stage HL after treatment, whereas patients who develop VBDS commonly die from their disease. There is no established approach to the management of paraneoplastic cholestasis. We postulate that single-modality radiation therapy alone should be considered a valid treatment alternative in early-stage HL with idiopathic cholestasis.

Adult↗

PubMedDate not supplied

Infantile cholestasis--advances in its understanding: new concepts.

Cholestasis is decrease or absence of bile flow into the duodenum. It can be either or in combination pathology of hepatocytes, intrahepatic bile ducts or extrahepatic bile ducts. Hepatocyte with their bile secretory apparatus and tight junction between hepatocytes are of specific importance in this. Bile is formed by several different energy-dependent transport processes. Secretion of bile is a complex metabolic process, which depends upon multiple structural and functional components in the hepatocytes and bile duct cells. The regulation of bile flow is regulated by many hormones. Bile is secreted in bile ducts having pressure of 15-25 cm of water. Rise in pressure in these bile ducts of more than 35 cm of water result suppression of bile flow and jaundice. A rise of conjugated serum bilirubin above the value of 400-500 mumol/L finds an alternate excretory pathway like urine. Various conditions are responsible for infantile cholestasis and can have different outcome of chronic cholestasis. These can be extrahepatic or intrahepatic and acute or chronic. Pathological consequences of infantile cholestasis are mainly because of malabsorption of fat and fat-soluble vitamins and hepatocellular dysfunctions. A battery of tests are required to diagnose the early infantile cholestasis. In the management of cholestasis diet rich in MCT is needed. Further, a high caloric intake up to 200 kcal/day to get adequate weight gain is desirable. Phototherapy, phenobarbitone and rifampicin is helpful in the pruritus of cholestasis by enhancing the excretion of bile. Ursodeoxycholic acid is specifically helpful in the cholestasis. A number of anti-inflammatory and immunosuppressive agents and a new compound, FK 506 has specific role in the management.

Bile↗