Pasqualini Titania, Ferraris Jorge
Sección de Endocrinología, Crecimiento y Desarrollo, Sección de Trasplante Renal Pediátrico, Departamento de Pediatría, Hospital Italiano de Buenos Aires, Argentina.
Medicina (B Aires). 2003;63(6):731-6.
Many children with chronic renal insufficiency (CRI) show growth retardation. Our objective is to describe the natural history of growth in patients with CRI, its pathogenesis and its optimization. Final height remains below percentile 3 in 77% of male and 71% of female patients. The etiology of growth retardation in these children is multifactorial age at onset, primary renal disease, fluid and electrolyte abnormalities especially acidosis, renal osteodystrophy, inadequate caloric intake and perturbations of growth factors are all implicated. Post Tx, immunosuppressive corticoid therapy and reduced glomerular filtration rate have a significantly negative effect on final height. Growth retardation in both CRI and renal Tx patients is not the result of abnormal growth hormone secretion or decreased levels of IGF-I, but rather of elevated levels of IGF-I binding proteins inhibiting the bioavailability of the IGFs. Optimization of growth includes reduced corticoid dose, alternate-day instead of daily prednisone therapy, or substituting deflazacort for methylprednisone. Several studies have shown that growth hormone (GH) therapy at a dose of 30 UI/m2/week results in growth improvement and this led the Food and Drug Administration to approve the use of GH prior to Tx. The response to GH is better during conservative therapy, less in allograft recipients and substantially less while undergoing dialysis. In conclusion, in those children with short stature, GH treatment should begin at an early age and during conservative therapy, trying to shorten dialysis in order to attain better height at the time of renal transplantation.
许多慢性肾功能不全(CRI)患儿存在生长发育迟缓。我们的目的是描述CRI患者生长发育的自然史、其发病机制及其优化方法。77%的男性患者和71%的女性患者最终身高仍低于第3百分位数。这些儿童生长发育迟缓的病因是多因素的,发病年龄、原发性肾脏疾病、液体和电解质异常尤其是酸中毒、肾性骨营养不良、热量摄入不足以及生长因子紊乱均有牵连。肾移植后,免疫抑制性皮质类固醇治疗和肾小球滤过率降低对最终身高有显著负面影响。CRI患者和肾移植患者的生长发育迟缓并非生长激素分泌异常或胰岛素样生长因子-I(IGF-I)水平降低所致,而是IGF-I结合蛋白水平升高抑制了IGF的生物利用度。生长的优化包括降低皮质类固醇剂量、泼尼松改为隔日治疗或用去氟可特替代甲泼尼龙。多项研究表明,每周30 UI/m²的生长激素(GH)治疗可改善生长,这使得美国食品药品监督管理局批准在肾移植前使用GH。保守治疗期间对GH的反应较好,同种异体移植受者反应较差,透析期间反应则明显更差。总之,对于那些身材矮小的儿童,GH治疗应在早期和保守治疗期间开始,尽量缩短透析时间,以便在肾移植时获得更好的身高。