title: Pathogenesis of rickets in chronic hepatobiliary disease in children.
abstract: To investigate whether hepatobiliary rickets is caused by defective intestinal absorption of vitamin D or by impaired hepatic hydroxylation of the vitamin, we studied three children who developed severe rickets, hypocalcemia, and hypophosphatemia, two despite having received 400 to 800 IU vitamin D per day by mouth, and one despite prolonged treatment with 10,000 IU daily. On oral vitamin D therapy, plasma vitamin D and 25-hydroxyvitamin D levels were low. When two children were treated with weekly intravenous doses of 3,000 IU vitamin D to approximate the recommended prophylactic allowance, their plasma calcium and phosphate values improved promptly, the radiographic lesions healed, and the plasma concentrations of vitamin D and 25-hydroxyvitamin D became normal. Our studies indicate that the primary cause of hepatobiliary rickets is intestinal malabsorption of vitamin D, not impairment of the hepatic metabolism of the vitamin.
mesh_terms: D001660:Biliary Tract Diseases / Q000150:complications* / Q000503:physiopathology; D002908:Chronic Disease; D005260:Female; D006801:Humans; D007223:Infant; D007408:Intestinal Absorption; D008107:Liver Diseases / Q000150:complications* / Q000503:physiopathology; D012279:Rickets / Q000209:etiology*; D014807:Vitamin D / Q000378:metabolism
chemical_list: D014807:Vitamin D