BACKGROUND The aim of this study was to investigate the histopathological findings in kidney biopsies in children with atypical hemolytic uremic syndrome (aHUS) and to determine whether specific pathological findings in aHUS have a prognostic value. METHODS Renal biopsy specimens of 29 patients who were recorded in the national Turkish aHUS registry database were available for review. Histopathological findings were compared with the clinical and laboratory features at the presentation and the final outcome. RESULTS The mean age at presentation and follow-up period was 4.9 ± 3.9 and 3.9 ± 3.0 years, respectively. The median time interval from the first symptom to biopsy was 10 days. Vascular thrombosis and interstitial fibrosis were significantly related to chronic kidney disease (CKD) requiring dialysis or kidney transplantation during follow-up (5.6-fold, for both). Glomerular necrosis, cortical necrosis, and glomerular sclerosis were markedly associated with CKD without dialysis (6.2-fold, 13.3-fold, and 8.8-fold, respectively). However, presence of endothelial swelling, subendothelial widening, and fragmented erythrocytes was found to be correlated with a favorable final outcome. CONCLUSIONS Presence of vascular thrombosis, cortical necrosis, and glomerular sclerosis in histopathological evaluation correlated with developing CKD. Chronic changes in the interstitial compartment were also related to poor prognosis, a finding that has been shown for the first time in pediatric aHUS cases.

中文翻译：

---

非典型溶血性尿毒症综合征儿童的肾活检预后结果。

背景本研究的目的是调查非典型溶血性尿毒症综合征 (aHUS) 儿童肾脏活检的组织病理学发现，并确定 aHUS 中的特定病理学发现是否具有预后价值。方法 土耳其国家 aHUS 登记数据库中记录的 29 名患者的肾活检标本可供审查。将组织病理学发现与就诊时的临床和实验室特征以及最终结果进行比较。结果 就诊和随访期间的平均年龄分别为 4.9 ± 3.9 和 3.9 ± 3.0 岁。从第一个症状到活检的中位时间间隔为 10 天。血管血栓形成和间质纤维化与随访期间需要透析或肾移植的慢性肾病 (CKD) 显着相关（两者均为 5.6 倍）。肾小球坏死、皮质坏死和肾小球硬化与未经透析的 CKD 显着相关（分别为 6.2 倍、13.3 倍和 8.8 倍）。然而，发现内皮肿胀、内皮下增宽和红细胞碎片的存在与有利的最终结果相关。结论 组织病理学评估中血管血栓形成、皮质坏死和肾小球硬化的存在与发展为 CKD 相关。间质室的慢性变化也与预后不良有关，这一发现首次在儿科 aHUS 病例中得到证实。肾小球坏死、皮质坏死和肾小球硬化与未经透析的 CKD 显着相关（分别为 6.2 倍、13.3 倍和 8.8 倍）。然而，发现内皮肿胀、内皮下增宽和红细胞碎片的存在与有利的最终结果相关。结论 组织病理学评估中血管血栓形成、皮质坏死和肾小球硬化的存在与发展为 CKD 相关。间质室的慢性变化也与预后不良有关，这一发现首次在儿科 aHUS 病例中得到证实。肾小球坏死、皮质坏死和肾小球硬化与未经透析的 CKD 显着相关（分别为 6.2 倍、13.3 倍和 8.8 倍）。然而，发现内皮肿胀、内皮下增宽和红细胞碎片的存在与有利的最终结果相关。结论 组织病理学评估中血管血栓形成、皮质坏死和肾小球硬化的存在与发展为 CKD 相关。间质室的慢性变化也与预后不良有关，这一发现首次在儿科 aHUS 病例中得到证实。并且发现破碎的红细胞与有利的最终结果相关。结论 组织病理学评估中血管血栓形成、皮质坏死和肾小球硬化的存在与发展为 CKD 相关。间质室的慢性变化也与预后不良有关，这一发现首次在儿科 aHUS 病例中得到证实。并且发现破碎的红细胞与有利的最终结果相关。结论 组织病理学评估中血管血栓形成、皮质坏死和肾小球硬化的存在与发展为 CKD 相关。间质室的慢性变化也与预后不良有关，这一发现首次在儿科 aHUS 病例中得到证实。