Division of Pediatric Hematology Oncology, University of Rochester, Rochester, NY.
Division of Hematology Oncology Akron Children's Hospital.
J Pediatr Hematol Oncol. 2023 Aug 1;45(6):309-314. doi: 10.1097/MPH.0000000000002642. Epub 2023 Feb 20.
Sickle cell nephropathy is a progressive morbidity, beginning in childhood, which is incompletely understood partially due to insensitive measures. We performed a prospective pilot study of pediatric and young adult patients with sickle cell anemia (SCA) to assess urinary biomarkers during acute pain crises. Four biomarkers were analyzed with elevations potentially suggesting acute kidney injury: (1) neutrophil gelatinase-associated lipocalin (NGAL), (2) kidney injury molecule-1, (3) albumin, and (4) nephrin. Fourteen unique patients were admitted for severe pain crises and were found to be representative of a larger SCA population. Urine samples were collected at the time of admission, during admission, and at follow-up after discharge. Exploratory analyses compared cohort values to the best available population values; individuals were also compared against themselves at various time points. Albumin was found to be moderately elevated for an individual during admission compared with follow-up ( P = 0.006, Hedge g : 0.67). Albumin was not found to be elevated compared with population values. Neutrophil gelatinase-associated lipocalin, kidney injury molecule-1, and nephrin were not found to be significantly elevated compared with population values or comparing admission to follow-up. Though albumin was found to be minimally elevated, further research should focus on alternative markers in efforts to further understand kidney disease in patients with SCA.
镰状细胞肾病是一种进行性疾病,始于儿童期,部分原因是由于检测手段不敏感,其发病机制尚未完全阐明。我们对患有镰状细胞贫血症 (SCA) 的儿科和年轻成年患者进行了一项前瞻性试点研究,以评估急性疼痛危象期间的尿生物标志物。分析了 4 种具有潜在提示急性肾损伤的升高标志物:(1) 中性粒细胞明胶酶相关脂质运载蛋白 (NGAL),(2) 肾损伤分子-1,(3) 白蛋白,和 (4) 足细胞裂孔隔膜蛋白。14 名独特的患者因严重疼痛危象入院,他们具有代表性的是更大的 SCA 人群。入院时、住院期间和出院后随访时采集尿液样本。探索性分析将队列值与最佳可用的人群值进行比较;还将个体在不同时间点与自身进行比较。与随访相比,个体在住院期间白蛋白中度升高 (P=0.006,Hedge g: 0.67)。与人群值相比,白蛋白未升高。与人群值或比较入院与随访相比,NGAL、肾损伤分子-1 和足细胞裂孔隔膜蛋白均未显著升高。虽然白蛋白略有升高,但应进一步研究替代标志物,以进一步了解 SCA 患者的肾脏疾病。