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ObjectiveCholestasis predisposes to fat-soluble vitamin (FSV) deficiencies. A liquid multiple FSV preparation made with tocopheryl polyethylene glycol-1000 succinate (TPGS) is frequently used in infants with biliary atresia (BA) because of ease of administration and presumed efficacy. In this prospective multicenter study, we assessed the prevalence of FSV deficiency in infants with BA who received this FSV/TPGS preparation.MethodsInfants received FSV/TPGS coadministered with additional vitamin K as routine clinical care in a randomized double-blinded, placebo-controlled trial of corticosteroid therapy after hepatoportoenterostomy (HPE) for BA (identifier NCT 00294684). Levels of FSV, retinol binding protein, total serum lipids, and total bilirubin (TB) were measured 1, 3, and 6 months after HPE.ResultsNinety-two infants with BA were enrolled in this study. Biochemical evidence of FSV insufficiency was common at all time points for vitamin A (29%-36% of patients), vitamin D (21%-37%), vitamin K (10%-22%), and vitamin E (16%-18%). Vitamin levels were inversely correlated with serum TB levels. Biochemical FSV insufficiency was much more common (15%-100% for the different vitamins) in infants whose TB was ≥2 mg/dL. At 3 and 6 months post HPE, only 3 of 24 and 0 of 23 infants, respectively, with TB >2 mg/dL were sufficient in all FSV.ConclusionsBiochemical FSV insufficiency is commonly observed in infants with BA and persistent cholestasis despite administration of a TPGS containing liquid multiple FSV preparation. Individual vitamin supplementation and careful monitoring are warranted in infants with BA, especially those with TB >2 mg/dL.

Bone fractures in children without a history of injury are highly suspicious for child abuse. Biliary atresia is a disorder associated with metabolic bone disease, and there are numerous reports of osteopenia, rickets, and/or fractures in this population. We report 3 cases of children with biliary atresia who had bony fractures as well as osteopenia whose caretakers were investigated for child abuse. Pediatricians should be aware of an increased incidence of fractures and overall prevalence of bone disease in this population., ABBREVIATIONS. ED, emergency department; CPS, Child Protective Services. Bone fractures in young children without a history of injury are considered highly suspicious for nonaccidental trauma. In children There are childhood [...]

Abstract. Twenty-three children with heterozygous familial hypercholesterolemia were studied to determine whether long-term administration of colestipol hydrochloride in conjunction with a low-cholesterol diet would be effective in reducing plasma total and low density lipoprotein cholesterol and would affect the plasma concentrations of vitamins A, D, E, K or folic acid. Effect of the drug on calcium metabolism was studied by sequential determination of serum total and ionized calcium, phosphorus, alkaline phosphatase, and parathyroid hormone. Mean decrease in plasma cholesterol of children previously on ad libitum diets was 6% after three months of diet. After two months of colestipol therapy and diet in the 11 children with good drug adherence, total cholesterol decreased 19 +/- 3% (SEM) from drug baseline; after two years of therapy in five of these children, mean decrease from drug baseline was 13 +/- 5%. Diet did not significantly decrease serum concentration of any of the vitamins but during 24 months of colestipol therapy serum vitamin A and E concentrations did decrease in the five patients with good drug adherence (vitamin A, 68 +/- 11 vs 35 +/- 4 micro g/100 ml, P < .005; vitamin E, 14 +/- 1 vs 11 +/- 1 micro g/100 ml, P < 0.05). However, all vitamin concentrations remained within normal limits; calcium metabolism was not affected. Annual monitoring of serum concentrations of fat-soluble vitamins and folate in children receiving colestipol for more than two years is recommended but may prove unnecessary. Pediatrics 65:243-250, 1980; fat-soluble vitamins, hypercholesterolemia, colestipol. [ABSTRACT FROM AUTHOR]