Familial Benign Recurrent Intrahepatic Cholestasis

Pagter, A.G.F De; Henegouwen, G. P. van Berge; Huinink, J.A. ten Bokkel; Brandt, K H

doi:10.1016/s0016-5085(76)80187-4

Cited by 104 publications

(6 citation statements)

References 27 publications

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“…Thus, it is tempting to speculate that the ABCB4 (MDR3) mutations associated with ICP cases may be responsible for the afore-mentioned high prevalence of cholelithiasis in ICP patients [40,41]. In mothers of patients with the benign recurrent intrahepatic cholestasis (BRIC), a higher incidence of ICP has been observed [43,44]. BRIC is a rare autosomal-recessive or sporadic disorder.…”

Section: Pathophysiologymentioning

confidence: 99%

Diagnosis and Therapy of Intrahepatic Cholestasis of Pregnancy

et al. 2004

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Intrahepatic cholestasis of pregnancy (ICP) is characterized by the occurrence of pruritus mostly in the third trimenon. Diagnosis is based on the presence of pruritus and elevated levels of serum bile acids in the absence of pruritic skin diseases. There is strong evidence of a genetic predisposition for ICP. Numerous studies have investigated the association of known cholestasis genes such as ABCB4 (also designated MDR3), ABCB11 ( BSEP) and ATP8B1 ( FIC1) with ICP. The results of these studies implicate a heterogeneous etiology of this syndrome. ICP increases the risk of preterm delivery and fetal loss. Furthermore, intense pruritus may necessitate premature induction of labor with its known higher frequency of complications for mother and child. Therefore, ICP pregnancies should be managed as high-risk pregnancies. Pharmaceuticals to alleviate pruritus or improve cholestasis like antihistamines, phenobarbital, anion exchange resins, dexamethasone or S-adenosylmethionine are not widely accepted because of questionable efficacy or side effects. Recent randomized studies have shown beneficial effects of ursodeoxycholic acid (UDCA) on laboratory data and pruritus in patients with ICP. Improved knowledge about the diagnostic classification of different types and pathophysiological mechanisms of ICP may allow for a more targeted treatment of this disease in future.

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Section: Pathophysiologymentioning

confidence: 99%

Diagnosis and Therapy of Intrahepatic Cholestasis of Pregnancy

et al. 2004

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“…Tygstrup & Jensen7 proposed the following criteria for the diagnosis of BRIC: (1) several episodes of pronounced jaundice with severe pruritus and biochemical evidence of cholestasis; (2) bile plugs on liver biopsy; (3) normal intraand extrahepatic bile ducts on direct cholangiography; (4) absence of a factor known to produce intrahepatic cholestasis; (5) symptom-free intervals of several months or years.…”

Section: Discussionmentioning

confidence: 99%

“…However, development towards biliary cirrhosis has been documented.2'4 In addition, most of the literature concerning BRIC is in the form of case reports, with few papers reporting long term follow-up. 5 We wish to report the course ofour patient with no evidence of chronic liver disease after over 25 years of follow-up.…”

Section: Introductionmentioning

confidence: 99%

Benign recurrent intrahepatic cholestasis--25 years of follow-up

Putterman

Keidar

Brook

1987

Postgraduate Medical Journal

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Summary Only 70 cases of recurrent intrahepatic cholestasis have been reported in the literature since the original description of this entity in 1959. The benign nature of the disease has been questioned, some authors suggesting progression to biliary cirrhosis. We report our follow-up of one such patient for over 25 years with no adverse physical consequences or histological deterioration. Sequential liver biopsies were obtained during this period. A conservative approach to diagnosis and treatment is therefore indicated.

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“…Values for individual BA s were expressed as relative proportions of the total , as well as in milligram s per kilo gram per day. Fecal fat was measured by the Van de Kamer method (15). Statistical significance between groups was calculated using Student' s t test.…”

Section: Discussionmentioning

confidence: 99%

Bile Acid Malabsorption in Cystic Fibrosis With and Without Pancreatic Insufficiency

Colombo,

Roda,

Roda

et al. 1984

J. pediatr. gastroenterol. nutr.

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Serum conjugated cholic acid (CCA) and conjugated chenodeoxycholic acid (CCDCA) fasting levels were measured in 30 children with cystic fibrosis (CF) without liver involvement, and mean levels were not significantly different from control values. In seven children (four with partially corrected pancreatic insufficiency and three without pancreatic insufficiency) serum levels of both primary bile acids (BAs) were also measured after the ingestion of a standard liquid meal; the values were then compared with those for total and fractional fecal BA excretion. The CCA mean peak increase was significantly reduced in patients with pancreatic insufficiency (p < 0.01), as well as in those without pancreatic insufficiency (p < 0.05), as compared to controls. The CCDCA mean peak increase was reduced only in patients with pancreatic insufficiency (p < 0.01). Fecal results confirmed serum data, showing a significantly increased excretion of cholic and deoxycholic acids in patients without pancreatic insufficiency as compared to controls (p < 0.02), despite a similar total BA excretion. In patients with pancreatic insufficiency, total fecal BA levels were markedly increased compared to control values (p < 0.001); the fecal percentage of chenodeoxycholic and lithocholic acids was greater than that recorded in patients without pancreatic insufficiency (p < 0.05), in agreement with the different behaviour of serum CCDCA postprandial curves for the two groups of patients. The results are consistent with selective malabsorption of cholic acid in CF, independent of the presence of pancreatic insufficiency; they confirm the usefulness of serum BA postprandial determinations in measuring BA malabsorption.

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Familial Benign Recurrent Intrahepatic Cholestasis

Cited by 104 publications

References 27 publications

Diagnosis and Therapy of Intrahepatic Cholestasis of Pregnancy

Diagnosis and Therapy of Intrahepatic Cholestasis of Pregnancy

Benign recurrent intrahepatic cholestasis--25 years of follow-up

Bile Acid Malabsorption in Cystic Fibrosis With and Without Pancreatic Insufficiency

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