In children with end-stage liver disease, little is known regarding the long-term effects of liver transplantation on bone. In this 2-year prospective study, we evaluated the effects of liver transplantation on bone mineral density (BMD) and on other parameters of bone metabolism in 30 consecutive children with biliary atresia who underwent liver transplantation after failed Kasai operation. Tacroli B one metabolism in the perioperative period of liver transplantation changes dynamically with the function of the liver and the systemic status of the recipient. 1 Objective information regarding the impact of liver transplantation on this aspect is lacking, especially for transplants in children. Biliary atresia (BA) is one of the most typical cholestatic diseases that cause end-stage liver disease in children. In the course of BA, multifactorial pathologic phenomena disturb normal bone metabolism to various degrees, causing atraumatic fractures and severe growth retardation. 2 Bone mineral density (BMD) is a reliable objective parameter of bone metabolism. The aims of this study were, first, to assess, with BMD, the growth retardation and defective skeletal mineralization before liver transplantation of pediatric patients with end-stage liver disease caused by BA, and, second, to evaluate the effects of liver transplantation for these patients and the mechanism of improvement of the bone metabolism after liver transplantation.
Patients and MethodsThirty-six consecutive patients with BA who underwent liver transplantation in our institution were included in this study. Informed consent was obtained from the families of all the patients. To adequately evaluate the effect of the liver transplantation, 6 patients (16%) who developed life-threatening posttransplant complications, (peritonitis [4 cases], sepsis [1] or chronic rejection [1]) were excluded from this study. Four children with peritonitis had surgical repair and did not receive standard immunosuppressants because of their severe septic condition. They were not given the steroids, and two of them died. The other 2 patients could not continue to follow up because of poor general condition. The patients with sepsis and chronic rejection died 1 month and 3 months after transplantation, respectively. The remaining 30 patients (15 boys and 15 girls), age ranging from 7 months to 181 months (median, 34 months), were enrolled. All patients had previously undergone a Kasai operation at around 2 months of age. The indications for liver transplantation in these patients were gastrointestinal bleeding (9 cases), recurrent cholangitis (7), growth retardation (5), intractable ascites (3), worsening of jaundice (3), hepatopulmonary syndrome (2), and hypersplenism (1). All patients underwent living-related liver transplantation as described elsewhere. 3 The postoperative immunosuppressant regimen was the combination of tacrolimus and steroids. The patients were weaned off steroids with stable liver function at around 6 months after the transplantation. 4 All children had r...