Bone fractures in children with chronic cholestasis.

The clinical features, biochemical data, roentgenographical bone changes and liver histological studies of four children with fractures related to chronic cholestasis are described. Two of them had Alagille syndrome and the other two, biliary atresia. Only one patient presented with forearm fracture associated with typical radiographic evidence of rickets. The others revealed generalized osteopenia together with fractures in arms, forearms, and wrists respectively. Of these, one with rickets had hypocalcemia and hypophosphatemia; one had decreased parathyroid hormone with mild hypocalcemia and hyperphosphatemia; one had hyperphosphatemia solely. Eucalcemia and euphosphatemia were noted in the remaining patient. Their bone lesions deteriorated in spite of treatment, except for the one with clinical rickets. All the patients succumbed eventually. It was concluded that the real pathogenesis of bone lesions in end-stage liver disease of chronic cholestatic children remains unclear. However, because rickets may intervene, continuing efforts to supply vitamin D and calcium since diagnosis of chronic cholestasis on a long-term basis, to prevent fracture, is still mandatory.