I型神经纤维瘤病腹膜后纤维化引起的终末期肾脏疾病。

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL
Luis Guilherme Ramanzini, Luís Fernando Muniz Camargo, Thaís Lorrany Oliveira Caixeta, Rafael Cardoso Louzada, Julia Maria Frare
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引用次数: 0

摘要

由于输尿管受压,腹膜后纤维化(RF)通常导致肾脏损害,约8%的患者最终发展为终末期肾病(ESRD)。我们报告一个61岁女性1型神经纤维瘤病(NF1)并发ESRD的RF病例。她表现为肾后急性肾损伤,最初接受输尿管导管治疗。腹部核磁共振显示右输尿管壁增厚,经膀胱瓣及腰肌结行右输尿管再植术。右输尿管有广泛的纤维化和炎症。活检显示非特异性纤维化,与RF一致。虽然手术很成功,但她还是患上了ESRD。我们回顾非典型表现的射频和肾损伤的原因在NF1。RF应被认为是NF1患者慢性肾脏疾病的可能原因,可能是由于未知的潜在机制。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
End-stage renal disease due to retroperitoneal fibrosis in neurofibromatosis type I.

Retroperitoneal fibrosis (RF) commonly leads to renal impairment due to compression of ureters, and around 8% of patients eventually progress to end-stage renal disease (ESRD). We present a case of RF in a 61-year-old female patient with neurofibromatosis type 1 (NF1) who developed ESRD. She presented with a postrenal acute kidney injury, being initially treated with an ureteral catheter. A magnetic resonance imaging of the abdomen showed parietal thickening of the right ureter, and she underwent right ureter reimplantation through bladder flap and psoas hitch. There was an extensive area of fibrosis and inflammation over the right ureter. Biopsy disclosed nonspecific fibrosis, which was consistent with RF. Although the procedure was successful, she developed ESRD. We review atypical presentations of RF and causes of renal injury in NF1. RF should be considered a possible cause of chronic kidney disease in patients with NF1, perhaps due to an unknown underlying mechanism.

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Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
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