Department of Pediatrics, Division Nephrology and Hypertension, The University of Tennessee and Le Bonheur Children's Hospital, 49 N. Dunlap, Memphis, TN, 38105, USA.
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics, Division of Hematology/Oncology, Emory University School of Medicine, Atlanta, GA, USA.
Pediatr Nephrol. 2019 Aug;34(8):1435-1445. doi: 10.1007/s00467-019-04237-3. Epub 2019 Apr 3.
Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome.
This is a multicenter retrospective analysis of renal biopsy findings and clinical outcomes in children and adolescents with SCD. We included children and adolescents (age ≤ 20 years) with SCD who had a kidney biopsy performed at a pediatric nephrology unit. The clinical indication for biopsy, biopsy findings, subsequent treatments, and outcomes were analyzed.
Thirty-six SCD patients (ages 4-19 years) were identified from 14 centers with a median follow-up of 2.6 years (0.4-10.4 years). The indications for biopsy were proteinuria (92%) and elevated creatinine (30%). All biopsies had abnormal findings, including mesangial hypercellularity (75%), focal segmental glomerulosclerosis (30%), membranoproliferative glomerulonephritis (16%), and thrombotic microangiopathy (2%). There was increased use of hydroxyurea, angiotensin-converting-enzyme inhibitors, and angiotensin receptor blockers following renal biopsy. At last follow-up, 3 patients were deceased, 2 developed insulin-dependent diabetes mellitus, 6 initiated chronic hemodialysis, 1 received a bone marrow transplant, and 1 received a kidney transplant.
Renal biopsies, while not commonly performed in children with SCD, were universally abnormal. Outcomes were poor in this cohort of patients despite a variety of post-biopsy interventions. Effective early intervention to prevent chronic kidney disease (CKD) is needed to reduce morbidity and mortality in children with SCD.
肾脏损伤是镰状细胞病（SCD）的一种进行性并发症。SCD 患儿常伴有微量白蛋白尿，而少数患儿则有更严重的肾脏表现，需要进行肾活检。目前，关于 SCD 患儿的肾活检结果以及随后的治疗和结局，相关信息有限。
这是一项对患有 SCD 的儿童和青少年的肾活检结果和临床结局进行的多中心回顾性分析。我们纳入了在儿科肾脏病学单位进行肾活检的患有 SCD 的儿童和青少年（年龄≤20 岁）。分析了活检的临床指征、活检结果、后续治疗和结局。
从 14 个中心确定了 36 例 SCD 患者（年龄 4-19 岁），中位随访时间为 2.6 年（0.4-10.4 年）。活检的指征是蛋白尿（92%）和肌酐升高（30%）。所有活检均有异常发现，包括系膜细胞增生（75%）、局灶节段性肾小球硬化（30%）、膜增殖性肾小球肾炎（16%）和血栓性微血管病（2%）。肾活检后羟脲、血管紧张素转换酶抑制剂和血管紧张素受体阻滞剂的应用增加。最后一次随访时，3 例患者死亡，2 例发生胰岛素依赖型糖尿病，6 例开始慢性血液透析，1 例接受骨髓移植，1 例接受肾移植。
尽管在 SCD 患儿中，肾活检并不常见，但在本研究中所有患儿的活检结果均为异常。尽管进行了多种活检后干预，但该患者队列的结局仍较差。需要采取有效的早期干预措施来预防慢性肾脏病（CKD），以降低 SCD 患儿的发病率和死亡率。
