Cholestatic Syndromes in Infancy: Diagnostic Value of Serum Bile Acid Pattern and Cholestyramine Administration

Javitt, Norman B.; Morrissey, Kevin P.; Siegel, Elliot R.; Goldberg, Henry P.; Gartner, Lawrence M.; Hollander, Melvin; Kok, Engeline

doi:10.1203/00006450-197303000-00003

Cited by 47 publications

(5 citation statements)

References 14 publications

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“…(Porter et al, 1972;Christen, Bau, and Halsband, 1975). Unfortunately, indirect methods of diagnosing EHBA, such as the ratio of serum bile salts (Javitt et al, 1973), red cell haemolysis test (Lubin et al, 1971), and serum ao-fetoprotein concentration (Zeltzef et al, 1974), which have been claimed to be or discriminatory value, have not been confirmed in practice (Manthrope and Mowat 1975;Mowat, 1973;Johnston et al, 1976). The safety and value of percutaneous liver biopsy in distinguishing patients with EHBA from those with hepatitis is well illustrated in this study.…”

Section: Discussionmentioning

confidence: 99%

Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants.

Mowat¹,

Psacharopoulos²

1976

Archives of Disease in Childhood

123

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R. (1976). Archives of Disease in Childhood, 51, 763. Extrahepatic biliary atresia versus neonatal hepatitis: review of 137 prospectively investigated infants. In a prospective regional survey of neonatal hepatitis syndrome 32 infants had extrahepatic biliary atresia (EHBA) and 103 had hepatitis. No cause for the lesion was found in infants with extrahepatic biliary atresia, but in 32 with hepatitis a specific cause was identified, 24 having genetic deficiency of the serum protein oc1-antitrypsin. No differences were observed in parental age, mother's health in pregnancy, month of birth, birth order, or sex of the infants. Familial idiopathic hepatitis occurred in 3 of 67 sibs of patients with idiopathic hepatitis, but the 33 sibs of EHBA patients had no liver disease. Of the infants with hepatitis, 36 were of low birthweight, <2-5 kg, and 23 were born prematurely. Infants with biliary atresia were all of normal birthweight and only one was born prematurely.Consideration of clinical and biochemical abnormalities in the first 2 months of life showed no differences between the two groups except that infants with EHBA were more commonly jaundiced from birth (80%) and had more frequently acholic stools (83%). The frequency of these features in patients with hepatitis being 68% and 52%. Standard tests of liver function were not discriminatory. Percutaneous liver biopsies were diagnostic in 75 % of those with EHBA and in 92% of those with hepatitis. The 131I Rose Bengal faecal excretion was less than 10°/o in 26 of 28 infants with EHBA and in only 5 of 18 with hepatitis. These latter two investigations together allowed a correct preoperative diagnosis of EHBA in all instances. Bile drainage was achieved surgically in only 3 cases. A major reason for these poor results may have been the late referral of cases for diagnosis and laparotomy, which should be performed as soon as the diagnosis is suspected and always by 70 days of age.

show abstract

Section: Discussionmentioning

confidence: 99%

Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants.

Mowat¹,

Psacharopoulos²

1976

Archives of Disease in Childhood

123

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“…Cholestyramine binds bile acids in the intestinal lumen, increases fecal excretion of these substances, and interrupts the enterohepatic bile-acid cycle. This condition probably decreases the negative feedback to the liver and enhances the conversion of cholesterol to bile acids (6,(10)(11)(12)(13)(14)(15)(16). Significant reductions in bile acids and LDL-C are achievable during treatment with cholestyramine in about 50% of eligible children.…”

Section: Discussionmentioning

confidence: 99%

“…The aim of this study was to report the experience of a trial in a series of children with AS and hypercholesterolemia managed with variable doses of cholestyramine, a nonabsorbable anion exchange resin used in the treatment of hypercholesterolemia in children (7,(10)(11)(12)(13)(14)(15)(16).…”

mentioning

confidence: 99%

Lack of Cholesterol‐Lowering Effect of Graded Doses of Cholestyramine in Children With Alagille Syndrome: A Pilot Study

Larrosa‐Haro,

Sáenz‐Rivera,

González‐Ortiz

et al. 2003

J. pediatr. gastroenterol. nutr.

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BackgroundThere is controversy about the potential risk of sustained high concentrations of cholesterol and triglyceride in patients with cholestatic chronic liver disease. However, it is currently accepted that cholesterol‐lowering therapy may reduce morbidity and mortality rates in hypercholesterolemic patients without preexisting coronary heart disease, as well as in those with coronary heart disease. The objective of this study was to evaluate the effect of cholestyramine on the serum lipid profile of a group of children with Alagille syndrome and hypercholesterolemia.MethodsFive children with Alagille syndrome and basal serum cholesterol concentrations greater than 230 mg/dL were included. Total serum cholesterol, triglyceride, low‐density, and high‐density lipoprotein cholesterol concentrations were measured on days 0, 10, 20, and 30 after the administration of oral cholestyramine 100, 250, and 500 mg(kg·d), respectively. Lipid fractions were reported as mean ± 1 SD. Statistical analysis was performed with Friedman analysis of variance.ResultsThe basal values and those of the three 10‐day subsequent 100‐, 250‐, and 500‐mg(kg·d) cholestyramine periods were as follows: total cholesterol: 327.6 ± 77.1, 305.4 ± 52.1, 290.6 ± 24.1, and 320.6 ± 32.3, respectively (P = 0.668); triglyceride: 136.4 ± 14.6, 144.8 ± 41.3, 161 ± 30.9, and 165.4 ± 40.7, respectively (P = 0.356); low‐density lipoprotein cholesterol: 245.4 ± 57.8, 239.8 ± 48.6, 242.2 ± 68.6, and 246.4 ± 49.5, respectively (P = 0.782); and high‐density lipoprotein cholesterol: 44.4 ± 11.2, 41.8 ± 12.8, 44.6.2 ± 13.2, and 47 ± 8.5, respectively (P = 0.431).ConclusionUnder the conditions of the current study, no significant effect of variable doses of cholestyramine could be demonstrated on the serum lipid profile of a series of children with Alagille syndrome. While the controversy on the potential atherogenic risk of low‐density lipoprotein hypercholesterolemia in patients with chronic liver disease persists, new, prospective pharmacologic or nutritional trials are required.

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“…Unfortunately, there is no evidence that this ratio, or any other bile acid measurement, can separate patients with intrahepatic cholestasis from those with extrahepatic cholestasis in adult practice. Javitt et al (1973) considered that the ratio was useful in distinguishing biliary atresia (low values) from intrahepatic cholestatic syndromes (high values) in infants. This observation, however, has been disputed (Mowat, 1976).…”

Section: Serum Bile Acids and Nature Of Hepatic Diseasementioning

confidence: 99%

Serum Bile Acids in Hepatobiliary Disease

González²,

R³

et al. 1982

The Lancet

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Cholestatic Syndromes in Infancy: Diagnostic Value of Serum Bile Acid Pattern and Cholestyramine Administration

Cited by 47 publications

References 14 publications

Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants.

Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants.

Lack of Cholesterol‐Lowering Effect of Graded Doses of Cholestyramine in Children With Alagille Syndrome: A Pilot Study

Serum Bile Acids in Hepatobiliary Disease

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