產(chǎn)后溶血性尿毒癥綜合征診治進展
發(fā)布時間:2018-03-06 23:22
本文選題:產(chǎn)后溶血性尿毒癥綜合征 切入點:血栓性微血管疾病 出處:《實用婦產(chǎn)科雜志》2017年08期 論文類型:期刊論文
【摘要】:產(chǎn)后溶血性尿毒癥綜合征(PHUS)屬于血栓性微血管疾病,是非典型溶血性尿毒癥綜合征中的一種,主要臨床表現(xiàn)為產(chǎn)后10周內(nèi)發(fā)生不可逆急性腎功能衰竭伴血小板減少、微血管病性貧血,臨床罕見,預后較差,少數(shù)病例發(fā)生于妊娠晚期。目前研究認為其發(fā)病的病理生理機制為原發(fā)性或繼發(fā)性補體系統(tǒng)調(diào)節(jié)異常。早期診斷和及時治療可以明顯改善患者預后,但其診斷與鑒別診斷較為困難,目前仍以臨床診斷為主,并應盡快啟動診斷性治療,特異性基因和分子檢測有助于明確診斷。血液透析、血漿置換仍然是PHUS首選的治療方法,而針對病因的特異性補體抑制劑可用于該病的長期治療。
[Abstract]:Postpartum hemolytic uremia syndrome (PHUSS) belongs to thrombotic microvascular disease and is one of atypical hemolytic uremic syndrome. The main clinical manifestation is irreversible acute renal failure with thrombocytopenia within 10 weeks of postpartum. Microvascular anemia, rare clinical, poor prognosis, A few cases occur in late pregnancy. The pathophysiological mechanism of the disease is primary or secondary complement system regulation abnormality. Early diagnosis and timely treatment can significantly improve the prognosis of patients. But its diagnosis and differential diagnosis are more difficult. At present, it is still mainly clinical diagnosis, and should start diagnostic therapy as soon as possible. Specific gene and molecular detection is helpful to definite diagnosis. Hemodialysis and plasma exchange are still the preferred treatment methods for PHUS. The specific complement inhibitors can be used in the long-term treatment of the disease.
【作者單位】: 四川大學華西第二醫(yī)院出生缺陷與相關婦兒疾病教育部重點實驗室;四川省醫(yī)學科學院四川省人民醫(yī)院;
【分類號】:R714.46
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本文編號:1576954
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