Bone Disease in Chronic Childhood Cholestasis II. Better Absorption of 25-OH Vitamin D than Vitamin D in Extrahepatic Biliary Atresia

Heubi, James E.; Hollis, Bruce W.; Tsang, Reginald C.

doi:10.1203/00006450-199001000-00006

Cited by 41 publications

(21 citation statements)

References 33 publications

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“…If a higher cutoff were to be used, as has been suggested by others, 14 then a higher frequency of vitamin D deficiency would have been identified (Figs 1 and 2). Cross-sectional studies of cholestatic children have revealed that .50% will have biochemical evidence of insufficiency of vitamins A, D, and E. 4,15,[20][21][22][23] In the current study, the effectiveness of a standardized regimen of vitamin supplementation in achieving normal serum levels of FSV was assessed in infants with BA after HPE. Supplementation with the FSV/TPGS preparation plus additional vitamin K failed to prevent FSV insufficiency in up to 58% of infants.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

Shneider

Magee²,

Bezerra

et al. 2012

Pediatrics

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WHAT'S KNOWN ON THIS SUBJECT: Cholestasis predisposes to the development of fat-soluble vitamin (FSV) deficiency. D-a tocopheryl polyethylene glycol-1000 succinate and coadministered FSVs are absorbed in spite of cholestasis. WHAT THIS STUDY ADDS:Infants with biliary atresia with total bilirubin .2 mg/dL are at risk for fat-soluble vitamin (FSV) deficiency. A multivitamin preparation containing d-a tocopheryl polyethylene glycol-1000 succinate alone is not effective in treating biochemical FSV insufficiency in cholestatic infants. abstract OBJECTIVE: Cholestasis 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. METHODS:Infants received FSV/TPGS coadministered with additional vitamin K as routine clinical care in a randomized double-blinded, placebocontrolled 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.RESULTS: Ninety-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.CONCLUSIONS: Biochemical 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. Pediatrics 2012;130:e607-e614 AUTHORS:

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Section: Discussionmentioning

confidence: 99%

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

Shneider

Magee²,

Bezerra

et al. 2012

Pediatrics

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“…1,2 Severe vitamin D deficiency may result in hypophosphatemia in addition to hypocalcemia; however, renal reabsorption of phosphorus is maximized. Elevated fibroblast growth factor 23 (FGF23), a phosphaturic factor, is the cause for hereditary hypophosphatemic rickets (HHPR) and, less commonly, tumor-induced osteomalacia.…”

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confidence: 99%

Two Case Reports of FGF23-Induced Hypophosphatemia in Childhood Biliary Atresia

Wasserman¹,

Ikomi²,

Hafberg³

et al. 2016

Pediatrics

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Cholestatic liver disease has long been associated with childhood rickets, secondary to impaired absorption of fat-soluble vitamin D. Elevated serum levels of fibroblast growth factor 23 (FGF23), secondary to genetic defects or tumor-induced osteomalacia, causes hypophosphatemic rickets in childhood. We present 2 infants with end-stage liver disease due to biliary atresia (BA) who developed hypophosphatemia with renal phosphate wasting. Serum FGF23 levels were elevated more than 8 times the upper limit of normal, and the older infant showed radiographic evidence of rickets. Both infants required large supplements of phosphate in addition to calcitriol. Following liver transplantation, FGF23 normalized in both patients and phosphate and calcitriol supplementation were discontinued. Immunohistochemistry revealed ectopic overexpression of FGF23 by hepatocytes in the BA liver. These observations highlight a unique cause of hypophosphatemic rickets in childhood and suggest the need for further investigation into the relationship between BA and other cholestatic disorders, and bone metabolism.

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“…Studies using serial serum measurements of vitamin D after an oral load, as performed in the present study, have shown that normal adults and children have a predictable rise in serum vitamin D, whereas adults and children with malabsorption syndromes, including cholestatic diseases, have reduced postdose serum rises (6,10,14). In patients with chronic cholestatic liver disease, intraluminal bile acid deficiency results in malabsorption of fat and fatsoluble substances, including vitamin D. Malabsorption is the primary cause of reduced serum 25(OH)D in adults with primary biliary cirrhosis (30)(31)(32) and in children with chronic cholestasis (6).…”

Section: Discussionmentioning

confidence: 99%

“…Several reports have claimed success in preventing or treating bone disease in cholestatic patients, in whom oral vitamin D was ineffective, with the use of oral 25(0H)-vitamin D (8,9,35). We have previously recommended the use of 25(OH)D to prevent bone disease in patients with "failed" portoenterostomy while awaiting orthotopic liver transplantation (10). These patients face the additional risk of decreased formation of skin cholecalciferol because of lack of sun exposure as a result of recurrent hospital admissions for biliary tract infections, sepsis, and/or nutritional complications, all occurring at a crucial time.…”

Section: Discussionmentioning

confidence: 99%

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d-α-Tocopheryl Polyethylene Glycol-1000 Succinate Enhances the Absorption of Vitamin D in Chronic Cholestatic Liver Disease of Infancy and Childhood

et al. 1992

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ABSTRACT. Rickets and osteopenia, common problems in chronic childhood cholestasis, have been attributed to vitamin D malabsorption leading to reduced serum levels of 25(0H)-vitamin D. d-a-Tocopheryl polyethylene glycol-1000 succinate (TPGS), a water-soluble form of vitamin E, forms micelles at low concentration. We evaluated the potential role of TPGS in enhancing vitamin D absorption in eight children (aged 5 mo to 19 y) with severe chronic cholestasis (three extrahepatic biliary atresia, three nonsyndromic intrahepatic cholestasis, and two Alagille syndrome). To evaluate vitamin D absorption, the subjects received vitamin D3 1000 IU/kg (maximum dose of 50 000 IU); they then received the same dose of vitamin Dg mixed with TPGS (25 IU/kg). Serial serum vitamin D3 levels and areas under the curve were measured. All patients had enhanced absorption of vitamin D when it was administered in a mixture with TPGS. Mean area under the curve for serum vitamin D3 was 403.0 -C 83.1 nmol x h/L (155.6 f 32.1 ng x h/mL), with a mean rise above baseline of 13.5 f 1.8 nmol/L (5.2 f 0.7 ng/mL) with vitamin D/TPGS compared with no rise when vitamin D was given alone (both p < 0.001). Seven patients have been followed for at least 3 mo while receiving the vitamin D/TPGS combination. Those with initially low serum 25(OH)-vitamin D levels (C37.5 nmol/L or 15 ng/mL) had normalization (range 37.5-146 nmol/L) within 1 mo, whereas those with initially normal levels remained normal. While the patients were receiving vitamin D/TPGS, serum vitamin E to total lipid ratio either normalized or remained normal. In conclusion, 1 ) TPGS enhances vitamin D absorption in infants and children with severe chronic cholestasis and 2) the enhanced absorption of both vitamins is sufficient to normalize or maintain adequate levels of serum 25(OH)-vitamin D and vitamin E to total lipid ratio. (Pediatr Res 31: 146-150,1992) Abbreviations 25(OH)D, 25-hydroxy-vitamin D TPGS, d-a-tocopheryl polyethylene glycol-1000 succinate

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Bone Disease in Chronic Childhood Cholestasis II. Better Absorption of 25-OH Vitamin D than Vitamin D in Extrahepatic Biliary Atresia

Cited by 41 publications

References 33 publications

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

Efficacy of Fat-Soluble Vitamin Supplementation in Infants With Biliary Atresia

Two Case Reports of FGF23-Induced Hypophosphatemia in Childhood Biliary Atresia

d-α-Tocopheryl Polyethylene Glycol-1000 Succinate Enhances the Absorption of Vitamin D in Chronic Cholestatic Liver Disease of Infancy and Childhood

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