Bone disease in infants and children with hepatobiliary disease

Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

“…The most frequent symptom is prolonged jaundice. Several reports have shown that osteodystrophy was associated with severe chronic liver disease despite the administration of vitamin and mineral supplements [1] . Argao et al [7] suggested that the bone mineral content of patients with hepatic osteodystrophy did not improve despite successful normalization of the serum 25-OH vitamin D concentration by enhancing vitamin D absorption from the gastrointestinal tract.…”

“…Clinical findings in children with biliary atresia (BA) characteristically include jaundice and acholic stools at 1 or 2 mo after birth [1] . Osteodystrophy is a well-recognized complication of chronic liver disease.…”

“…Osteodystrophy is a well-recognized complication of chronic liver disease. BA is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy [1] . Vitamin D is hydroxylated at the carbon 25 position to form 25-hydroxy-vitamin D (25-OH-D) [2] .…”

“…This occurs primarily in the liver [2] . Bile is important for the intestinal absorption of calcium and magnesium because it is necessary for the absorption of vitamin D [1] . In chronic liver disease, particularly where there is chronic cholestasis, generalized skeletal demineralization or rachitic change is seen [3] .…”

“…Vitamin D and hence calcium absorption are thus diminished. 25-OH-D is thought to be converted to more active forms, 125-or 2125-dehydro-OH-D. Rickets and osteoporosis were reported to be found in 23 of 39 patients (59%) with surgically unrepaired BA [1] . We herein report two infants: one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy, and the other at 4 wk after Kasai hepatic portoenterostomy.…”