Alkhunaizi Ahmed M, Al-Khatti Adil A, Al-Mueilo Samir H, Amir Abdulrazack, Yousif Bedri
Specialty Medicine Department, Nephrology Section, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia.
Cancer Institute, Cancer Care Unit, Johns Hopkins Aramco Healthcare, Dhahran, Saudi Arabia.
Saudi J Kidney Dis Transpl. 2017 Jul-Aug;28(4):751-757.
Sickle cell nephropathy is a severe complication of sickle cell disease (SCD) that has a wide range of manifestations, from asymptomatic microalbuminuria to end-stage renal disease (ESRD). The data on patients with SCD who develop ESRD are scarce. The aim of this study was to explore the course of patients with SCD who developed ESRD and received renal replacement therapy (RRT). The course of patients with SCD who developed ESRD and started dialysis at two centers in the Eastern Province of Saudi Arabia was retrospectively analyzed. Parameters included age at initiation of dialysis, survival until death or kidney transplantation, hospitalization due to pain crisis, disease-related parameters, and requirement for blood transfusion. Sixteen patients with SCD developed ESRD and started RRT with either hemodialysis or peritoneal dialysis. The mean age at initiation of dialysis was 46.6 years. The majority of patients (10 out of 16) were resistant to erythropoiesis-stimulating agents (ESA) and required blood transfusion repeatedly. Pain crises were infrequently encountered. Median survival was 54 months. Four patients received kidney transplantation with good outcome. In conclusion, most patients with SCD who developed ESRD were resistant to ESA and required repeated blood transfusion. The rate of hospitalization due to pain crisis was relatively low. Survival on dialysis was comparable to that of patients with no SCD, and the post-transplant course was relatively benign.
镰状细胞肾病是镰状细胞病（SCD）的一种严重并发症，有多种表现形式，从无症状微量白蛋白尿到终末期肾病（ESRD）。关于发展为ESRD的SCD患者的数据很少。本研究的目的是探讨发展为ESRD并接受肾脏替代治疗（RRT）的SCD患者的病程。对沙特阿拉伯东部省份两个中心发展为ESRD并开始透析的SCD患者的病程进行了回顾性分析。参数包括开始透析时的年龄、直至死亡或肾移植的生存期、因疼痛危象住院情况、疾病相关参数以及输血需求。16例SCD患者发展为ESRD并开始接受血液透析或腹膜透析的RRT。开始透析时的平均年龄为46.6岁。大多数患者（16例中的10例）对促红细胞生成素（ESA）耐药，需要反复输血。疼痛危象很少见。中位生存期为54个月。4例患者接受了肾移植，效果良好。总之，大多数发展为ESRD的SCD患者对ESA耐药，需要反复输血。因疼痛危象住院的发生率相对较低。透析患者的生存率与无SCD患者相当，移植后的病程相对良好。
