BACKGROUND Sickle cell disease (SCD) is a highly prevalent genetic disease worldwide. In the natural evolution of SCD, glomerular lesions can develop, presenting histopathological patterns of segmental or focal membranoproliferative glomerulosclerosis, with or without thrombotic microangiopathy. We report two cases of acute post-infectious glomerulonephritis (APIGN), with atypical presentations, in patients with SCD. CASE PRESENTATION Case 1: An 18-year-old female with SCD presented with a 21-day history of progressive oedema, accompanied by dyspnoea, productive cough, fever, and chest pain. Blood tests showed the following: haemoglobin 6.1 g/dl; leucocytes 18,820 cells/mm3; and creatinine 0.49 mg/dl. A urine sample evidenced leucocyturia and haematuria. The 24-h proteinuria was 8.99 g, serum albumin level was 1.2 g/dl, low serum C3 levels and high levels of anti-streptolysin O. Renal biopsy was consistent with APIGN. The patient was treated with diuretic and anti-proteinuric agents, subsequently evolving to reversal of the renal alterations. Case 2: A 12-year-old male with SCD presented with a 20-day history of a non-productive cough and progressive oedema, together with hypertension. The serum creatinine concentration was 0.48 mg/dl. A urine sample evidenced leukocyturia and haematuria. The 24-h proteinuria was 12.5 g, and the serum albumin level was 2.6 g/dl. The levels of C3 and C4 were normal. Renal biopsy revealed APIGN. The patient was treated with diuretic and anti-proteinuric agents, subsequently evolving reversal of the renal alterations. CONCLUSIONS The presentation of the two cases reported here are not typical of SCD-related kidney injury. Analysis of the renal biopsy specimens elucidated the diagnosis, affecting the prognosis, because that of APIGN is highly favourable, unlike that of nephrotic syndrome associated with SCD glomerulopathy.

中文翻译：

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镰状细胞病患者急性感染后肾小球肾炎的非典型表现：两例报告。

背景技术镰状细胞病（SCD）是全世界高度流行的遗传病。在SCD的自然进化过程中，肾小球病变会发展，表现为节段性或局灶性膜增生性肾小球硬化的组织病理学模式，伴或不伴有血栓性微血管病。我们报告SCD患者中的两个急性感染后肾小球肾炎（APIGN），具有非典型表现。病例介绍病例1：一名18岁的SCD女性患者，出现21天的进行性水肿病史，并伴有呼吸困难，咳嗽，发烧和胸痛。验血结果如下：血红蛋白6.1 g / dl；白细胞18,820细胞/ mm3; 和肌酸酐0.49 mg / dl。尿液样本显示有白细胞增多症和血尿。24小时蛋白尿为8.99 g，血清白蛋白水平为1.2 g / dl，低血清C3水平和高水平抗链球菌溶血素O。肾活检与APIGN一致。该患者接受了利尿剂和抗蛋白尿药物的治疗，随后发展为肾功能改变的逆转。病例2：一名12岁的SCD男性患者出现20天无生产性咳嗽和进行性水肿以及高血压的病史。血清肌酐浓度为0.48mg / dl。尿液样本显示有白细胞增多症和血尿。24小时蛋白尿为12.5 g，血清白蛋白水平为2.6 g / dl。C3和C4水平正常。肾活检显示APIGN。该患者接受了利尿剂和抗蛋白尿药物的治疗，随后逐渐发展为肾脏改变。结论本文报道的两例病例并非典型的SCD相关性肾损伤。