Haffner Dieter, Zivicnjak Miroslav
Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625, Hannover, Germany.
Pediatr Nephrol. 2017 Jun;32(6):949-964. doi: 10.1007/s00467-016-3432-3. Epub 2016 Jul 27.
Impairment of pubertal growth and sexual maturation resulting in reduced adult height is an significant complication in children suffering from chronic kidney disease (CKD). Delayed puberty and reduced pubertal growth are most pronounced in children with pre-existing severe stunting before puberty, requiring long-term dialysis treatment, and in transplanted children with poor graft function and high glucocorticoid exposure. In pre-dialysis patients, therapeutic measures to improve pubertal growth are limited and mainly based on the preservation of renal function and the use of growth hormone treatment. In patients with end-stage CKD, early kidney transplantation with steroid withdrawal within 6 months of renal transplantation allows for normal pubertal development in the majority of patients. This review focuses on the underlying pathophysiology and strategies for improving height and development in these patients.
青春期生长和性成熟受损导致成人身高降低是慢性肾脏病(CKD)患儿的一个重要并发症。青春期延迟和青春期生长减缓在青春期前已有严重发育迟缓、需要长期透析治疗的患儿,以及移植肾功能差且糖皮质激素暴露量高的移植患儿中最为明显。在透析前患者中,改善青春期生长的治疗措施有限,主要基于肾功能的维持和生长激素治疗的应用。在终末期CKD患者中,早期肾移植并在肾移植后6个月内停用类固醇可使大多数患者实现正常的青春期发育。本综述重点关注这些患者身高和发育改善的潜在病理生理学及策略。