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Chronic organ failure in adult sickle cell disease

机译:成人镰状细胞病的慢性器官衰竭

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摘要

Sickle cell disease is now a chronic adult illness characterized by progressive multiorgan failure, particularly involving the brain and kidney. The etiology is multifactorial; it includes hemolysis and nitric oxide deficiency. As patients age, most experience neurologic insult. Twenty-five percent of older adults have had a clinical stroke and at least half of the population have had a silent infarct, cortical atrophy, and neurocognitive impairment. Periodic screening with neuroimaging and neurocognitive testing is recommended. Identification and correction of modifiable risk factors such as nocturnal hypoxemia, obstructive sleep apnea, and physical exercise programs should be implemented. Patients with neurocognitive impairment require cognitive remediation and educational accommodations. Chronic renal disease occurs in 25% of older adults and results in 50% of their deaths. Renal failure often develops insidiously. It can be prevented or minimized by early screening and treatment of modifiable risk factors including hypertension and microalbuminuria. There is an increasing number of therapeutic options, including inhibitors of the renin angiotensin system, angiotensin-II receptor blockers, endothelin-1 receptor antagonist, and haptoglobin therapy. Patients with sickle cell disease have increased mortality rates from renal failure compared with nonsickle cell patients, in part from a lack of access to early multidisciplinary care, including timely initiation of dialysis and renal transplantation.
机译:镰状细胞病现在是一种慢性成人疾病,其特征是进行性多器官功能衰竭,尤其是涉及脑和肾脏。病因是多因素的。它包括溶血和一氧化氮缺乏症。随着患者年龄的增长,大多数患者会遭受神经系统损伤。 25%的成年人患有中风,至少一半的人口患有无声梗塞,皮质萎缩和神经认知障碍。建议定期进行神经影像学检查和神经认知测试。应当识别和纠正诸如夜间低氧血症,阻塞性睡眠呼吸暂停和体育锻炼等可改变的危险因素。神经认知障碍患者需要认知矫正和教育适应。慢性肾病发生在25%的老年人中,并导致50%的死亡。肾衰竭通常会隐匿地发展。可以通过早期筛查和治疗可改变的危险因素(包括高血压和微量白蛋白尿)来预防或减少这种疾病。越来越多的治疗选择,包括肾素血管紧张素系统抑制剂,血管紧张素II受体阻滞剂,内皮素1受体拮抗剂和触珠蛋白疗法。与非镰状细胞病患者相比,镰状细胞病患者的肾衰竭死亡率更高,部分原因是缺乏早期的多学科护理,包括及时开始透析和肾移植。

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