Growth and bone health in chronic liver disease and following liver transplantation in children.

Improved survival of orthotopic liver transplantation (OLT) has shifted the focus of patient care to quality of life, including prevention and treatment of pre- and post-transplant complications. End-stage liver failure affects bone length and strength, causing growth failure and hepatic osteodystrophy. Growth failure affects 60% of children assessed for OLT. Optimization of nutrition may prevent further stunting of growth before OLT but is rarely successful. Catch-up growth is observed following steroid withdrawal usually from 18 months post OLT. Whether growth hormone treatment would benefit the 20% of children who fail to regain normal height needs to be tested in randomized controlled trials. Hepatic osteodystrophy in children comprises vitamin D deficiency rickets, low bone mass and fractures caused by malnutrition and malabsorption. Vitamin D deficiency should be treated aggressively with cholecalciferol (D2) or ergocalciferol (D3). The active vitamin D metabolites alphacalcidol or calcitriol are used to increase calcium absorption from the gut but do nothing to replace vitamin D stores. Children before and after OLT have an increased prevalence of fractures of 10-13% and 12-38%, respectively. Most fractures are vertebral, and are related to low spine BMD. They often occur asymptomatically but may also cause chronic pain and later scoliosis. The main risk groups are infants with cholestatic liver disease, and adolescents with later OLT and greater BMI. Fracture prediction in these children is limited. OLT also bears the risk of avascular bone necrosis (4%), and development of scoliosis (13-38%). This paper reviews the literature and presents preventative and therapeutic strategies to improve bone length and strength.