Kanai Hiroaki, Sawanobori Emi, Kobayashi Anna, Goto Miwa
Department of Pediatrics, Suwa Central Hospital, Chino, JPN.
Department of Pediatrics, Faculty of Medicine, University of Yamanashi, Chuo, JPN.
Cureus. 2024 Jul 5;16(7):e63923. doi: 10.7759/cureus.63923. eCollection 2024 Jul.
Progressive kidney dysfunction is often observed in children with bilateral hypoplastic kidneys. While glomerulopathy can exacerbate hypoplastic kidney progression, only IgA nephropathy and post-streptococcal acute glomerulonephritis have been noted in such cases. Herein, we present a case of a four-year-old female patient with bilateral hypoplastic kidney, kidney dysfunction, and significant proteinuria (urinary protein/creatinine ratio > 1 g/gCr), prompting referral owing to persistent hematuria since two years of age. Enalapril was initiated; however, urinary findings exhibited no improvement despite stable symptoms and kidney function. Subsequently, a kidney biopsy was performed at six years of age, and C1q nephropathy was diagnosed. Given the presence of only mild mesangial proliferation, steroids were not administered; enalapril treatment was continued. By seven years of age, the patient's hematuria had resolved, and proteinuria levels had decreased. On the latest follow-up at 12 years of age, kidney function was preserved with only mild proteinuria. This case report highlights the favorable prognosis of asymptomatic C1q nephropathy characterized by mild mesangial proliferation, even in patients with hypoplastic kidneys, renal dysfunction, and significant proteinuria. It emphasizes the significance of timely pathological evaluation for guiding appropriate interventions in such patients.
双侧肾发育不全的儿童常出现进行性肾功能不全。虽然肾小球病可加重发育不全肾脏的进展，但在此类病例中仅发现IgA肾病和链球菌感染后急性肾小球肾炎。在此，我们报告一例4岁女性患者，患有双侧肾发育不全、肾功能不全及显著蛋白尿（尿蛋白/肌酐比值>1 g/gCr），因自两岁起持续血尿而转诊。开始使用依那普利治疗；然而，尽管症状和肾功能稳定，但尿液检查结果并无改善。随后，患者6岁时进行了肾活检，诊断为C1q肾病。鉴于仅存在轻度系膜增生，未给予类固醇治疗；继续依那普利治疗。到7岁时，患者血尿消失，蛋白尿水平降低。在12岁的最新随访中，肾功能得以保留，仅存在轻度蛋白尿。本病例报告强调了无症状C1q肾病以轻度系膜增生为特征的良好预后，即使是在患有肾发育不全、肾功能不全及显著蛋白尿的患者中。它强调了及时进行病理评估以指导对此类患者进行适当干预的重要性。
