Scaparrotta Alessandra, Di Pillo Sabrina, Attanasi Marina, Consilvio Nicola Pietro, Cingolani Anna, Rapino Daniele, Mohn Angelika, Chiarelli Francesco
Department of Pediatrics, University of Chieti, Italy.
J Pediatr Endocrinol Metab. 2012;25(5-6):393-405. doi: 10.1515/jpem-2012-0012.
Poor linear growth and inadequate weight gain are very common problems in cystic fibrosis (CF) children. The most important factors involved in growth failure are undernutrition or malnutrition, chronic inflammation, lung disease, and corticosteroid treatment. Nutritional support and pharmacological therapy with recombinant human growth hormone are essential for a good management of children with CF, although these children are shorter and lighter than healthy children, and despite the catch-up growth observed after diagnosis, deficit in length/height and weight continues to be seen until adulthood. Early diagnosis is essential to ensure better nutritional status and growth, potentially associated with better respiratory function and prognosis. The aims of this review are try to explain etiology and pathogenetic mechanisms of growth failure in CF children and clarify their role in the disease morbidity and in clinical outcome, especially in relation to progressive decline of pulmonary function.
生长发育迟缓及体重增加不足是囊性纤维化（CF）患儿非常常见的问题。导致生长发育不良的最重要因素包括营养不足或营养不良、慢性炎症、肺部疾病以及皮质类固醇治疗。尽管CF患儿比健康儿童身材更矮小、体重更轻，且在诊断后观察到有追赶生长现象，但直至成年，其身高和体重仍持续低于正常水平，因此营养支持及重组人生长激素药物治疗对于CF患儿的良好管理至关重要。早期诊断对于确保更好的营养状况及生长发育至关重要，这可能与更好的呼吸功能及预后相关。本综述的目的是试图解释CF患儿生长发育不良的病因及发病机制，并阐明其在疾病发病及临床结局中的作用，尤其是与肺功能进行性下降的关系。
