Among twelve patients with biliary atresia (BA) who have undergone a hepatic porto-jejunostomy (or porto-gastrostomy) since 1970, pathological fracture involving long bones (Clavicle, Radius, Ulna and Femur) was observed in six at the age from 2 years to 4 years and 7 months. Therefore, we measured serum level of 25-hydroxyvitamin D (25-OH-D), calcitonin, parathyroid hormone (PTH) and radiological changes in bone in eleven patients. Since bile juice is necessary for vitamin D absorption, we classified the postoperative BA patients into three groups; 1) internal jejunostomy, 2) oral-sided and 3) anal-sided external jejunostomy. The group of anal-sided external jejunostomy showed a large amount of bile loss (10.8±3.8ml/kg/day) from jejunostomy, and significantly low serum 25-OH-D level less than 5.0ng/ml. On the other hand, a small amount of bile loss (4.1±2.7ml/kg/day) and relative decrease in serum 25-OH-D level (17.8±13.0ng/ml) were recognized in the group of oral-sided external jejunostomy. However, serum levels of calcitonin and PTH were almost within normal range with wide deviation, and no significant relation to the operative methods was recognized. Since radiological examination of long bones revealed no rachitic changes except relatively thin cortex and decrease in bone density, pathogenesis of postoperative fracture in BA patients might be due to not only vitamin D defficiency, but also growth disturbance of osteoid tissues of the other causes.
