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我如何治疗镰状细胞病中的肾脏并发症。

How I treat renal complications in sickle cell disease.

机构信息

Department of Renal Medicine, King's College London, London, United Kingdom; Department of Renal Medicine, King's College Hospital NHS Foundation Trust, London, United Kingdom;

Division of Cancer Studies, Molecular Haematology, King's College London, London, United Kingdom; and Department of Haematological Medicine, King's College Hospital NHS Foundation Trust, London, United Kingdom.

出版信息

Blood. 2014 Jun 12;123(24):3720-6. doi: 10.1182/blood-2014-02-557439. Epub 2014 Apr 24.

Abstract

Renal disease is one of the most frequent and severe complications experienced by patients with sickle cell disease; its prevalence is likely to increase as the patient population ages. We recommend regular monitoring for early signs of renal involvement and a low threshold for the use of hydroxyurea as preventative measures for end-stage renal disease. Once renal complications are detected, a careful assessment of the patient is required to rule out other causes of renal disease. Proteinuria and hypertension should be managed aggressively and the patient referred to a specialist nephrology center when progressive decline in renal function is noted. For the few patients who develop advanced chronic kidney disease, timely planning for dialysis and transplantation can significantly improve outcome, and we recommend an exchange blood transfusion policy for all patients on the transplant waiting list and for those with a functioning graft. Alongside the invasive treatment regimes, it is important to remember that renal failure in conjunction with sickle cell disease does carry a significant burden of morbidity and that focusing on symptom control has to be central to good patient care.

摘要

肾脏疾病是镰状细胞病患者最常见和最严重的并发症之一;随着患者年龄的增长,其患病率可能会增加。我们建议定期监测肾脏受累的早期迹象,并将羟基脲作为预防终末期肾病的措施,及早使用。一旦发现肾脏并发症,需要仔细评估患者以排除其他肾脏疾病的原因。应积极治疗蛋白尿和高血压,并在肾功能进行性下降时将患者转介至专科肾脏病中心。对于少数出现晚期慢性肾脏病的患者,及时进行透析和移植规划可以显著改善预后,我们建议对所有移植等待名单上的患者和有功能移植物的患者实施输血交换政策。除了侵入性治疗方案外,还必须记住,镰状细胞病合并肾衰竭会带来重大的发病负担,因此关注症状控制必须是良好患者护理的核心。

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