PDF(Pubmed)
Abstract：
Cystic fibrosis (CF) is a multisystem disorder and represents the most common inherited condition leading to death in Western countries. Previous reports of chronic kidney disease (CKD) in CF focus on cases post lung, or other solid organ, transplantation but CKD in CF patients pre transplantation is increasingly recognized as a challenging complication of CF. CKD can evolve as a sequel to acute kidney injury for example after prolonged treatment with aminoglycoside antibiotics during episodes of infection. Nephrolithiasis, diabetic nephropathy and a variety of glomerular lesions, such as amyloidosis and Immunoglobulin A nephropathy are also seen. Muscle depletion is common in CF, hence creatinine-based estimates of kidney function may underestimate the degree of renal impairment and lead to delayed diagnosis and management. Improved treatment options for CF patients have resulted in a sustained increase in life expectancy with increasing numbers of CF patients with CKD approaching end-stage renal failure prior to consideration of lung transplantation. We believe that kidney or combined kidney-pancreas transplantation are under-utilized in this population. We provide a brief primer on the landscape of CF and CKD and discuss transplant options. Suitable patients with CF and advanced CKD should be formally assessed for kidney or kidney-pancreas transplantation.
摘要：
囊性纤维化（CF）是一种多系统疾病，是西方国家最常见的导致死亡的遗传性疾病。CF中慢性肾病（CKD）的先前报道集中在肺后病例，或其他实体器官，但CF患者移植前的CKD越来越被认为是CF的挑战性并发症。CKD可以发展为急性肾损伤的后遗症,例如在感染发作期间用氨基糖苷类抗生素长期治疗后。肾结石,糖尿病肾病和各种肾小球病变，如淀粉样变性和免疫球蛋白A肾病也可见。肌肉耗竭在CF中是常见的，因此,基于肌酐的肾功能评估可能会低估肾功能损害的程度,并导致延迟诊断和治疗.CF患者的改进的治疗选择导致预期寿命的持续增加，在考虑肺移植之前，患有CKD的CF患者接近终末期肾衰竭。我们认为，在该人群中，肾脏或肾胰腺联合移植的利用不足。我们简要介绍了CF和CKD的情况，并讨论了移植选择。应正式评估CF和晚期CKD患者的肾脏或肾胰腺移植。
