Maes M, Sokal E, Otte J B
Department of Pediatrics, Cliniques Universitaires St Luc, Catholic University of Louvain, Brussels, Belgium.
Pediatr Transplant. 1997 Nov;1(2):171-5.
Growth failure is a common observation in children with end-stage liver disease (ESLD). The liver is an important endocrine organ producing potent anabolic growth factors such as IGF-I (insulin-like growth factor-I) and its major binding-proteins, called IGFBP-1, -2 and -3. Circulating IGF-I and IGFBP-3 levels are low in children with chronic liver failure despite increased GH secretion. This discrepancy suggests that GH resistance is present in chronic liver failure and is mainly due to the associated malnutrition. The advent of orthotopic liver transplantation (OLT) has dramatically improved the life expectancy of children with ESLD. Nevertheless, the growth of 15-20% of the children in recent studies remains poor after successful transplantation. Several factors such as age and height deficit at the time of OLT, etiology of the liver disease and graft function as well as the dose and mode of administration of glucocorticoids have been implicated in the lack of complete catch-up growth following surgery. Few studies have explored the possibility that anomalies in the GH-IGF-I cascade could explain growth retardation. However, it is unlikely that major anomalies of the GH-IGF-I axis contribute to impaired growth. Treatment with GH alone or in combination with IGF-I before or after OLT may improve the growth of children. Randomized multi-center studies are needed to address this issue.
生长发育迟缓是终末期肝病（ESLD）患儿的常见表现。肝脏是一个重要的内分泌器官，可产生如胰岛素样生长因子-I（IGF-I）等强效合成代谢生长因子及其主要结合蛋白，即IGFBP-1、-2和-3。尽管生长激素（GH）分泌增加，但慢性肝功能衰竭患儿的循环IGF-I和IGFBP-3水平较低。这种差异表明慢性肝功能衰竭存在GH抵抗，且主要归因于相关的营养不良。原位肝移植（OLT）的出现显著提高了ESLD患儿的预期寿命。然而，在最近的研究中，15%至20%的患儿在成功移植后生长情况仍然不佳。OLT时的年龄和身高缺陷、肝脏疾病的病因、移植物功能以及糖皮质激素的剂量和给药方式等多种因素与术后未能完全实现追赶生长有关。很少有研究探讨GH-IGF-I级联异常是否可解释生长发育迟缓。然而，GH-IGF-I轴的主要异常不太可能导致生长受损。在OLT前后单独使用GH或联合使用IGF-I进行治疗可能会改善患儿的生长情况。需要进行随机多中心研究来解决这一问题。
