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妊娠合并血栓性血小板减少性紫癜伴肾病综合征:一例报告

Pregnancy-onset thrombotic thrombocytopenic purpura with nephrotic syndrome: a case report.

作者信息

Noda Ryunosuke, Kakinuma Yuki, Suzuki Kensuke, Ide Sanae, Bae Yuan, Miyauchi Akito, Ishibashi Yoshitaka

机构信息

Department of Nephrology, Japanese Red Cross Medical Center, 4-1-22 Hiro-o, Shibuya-Ku, Tokyo, 150-8935, Japan.

Department of Obstetrics, Japanese Red Cross Medical Center, 4-1-22 Hiro-o, Shibuya-Ku, Tokyo, 150-8935, Japan.

出版信息

CEN Case Rep. 2022 May;11(2):203-207. doi: 10.1007/s13730-021-00654-2. Epub 2021 Oct 8.

Abstract

Pregnancy-onset thrombotic thrombocytopenic purpura (TTP) was reported by many obstetricians and hematologists, but less by nephrologists, and the detailed clinical course of its renal complication is not known. Here, we report a case of a 33-year-old pregnant woman who suffered from pregnancy-onset TTP with nephrotic syndrome which was controlled by the termination of pregnancy. On admission, she had periorbital and lower leg edema at 32 weeks of gestation. Her serum albumin level was 2.8 g/dL and the urine protein/creatinine ratio was 4.1 g/g Cr. Besides those, she had thrombocytopenia, hemolytic anemia, and severe deficiency of A Disintegrin-like and Metalloproteinase with Thrombospondin type 1 motifs 13 (ADAMTS-13) activity. Thus, she was diagnosed with nephrotic syndrome due to pregnancy-onset TTP. A cesarean section was performed without complications for the patient and her baby. Then, all her symptoms improved shortly. She was suspected of congenital TTP because of no ADAMTS-13 inhibitor results and the persistent deficiency of ADAMTS-13 activity even after her condition improved. Pregnancy-onset TTP can cause nephrotic syndrome. Termination of pregnancy should be considered in cases with pregnancy-onset TTP to protect kidney function.

摘要

许多产科医生和血液科医生都报告过妊娠起病的血栓性血小板减少性紫癜(TTP),但肾病科医生的报告较少,其肾脏并发症的详细临床过程尚不清楚。在此,我们报告一例33岁的孕妇,她患有妊娠起病的TTP并伴有肾病综合征,通过终止妊娠病情得到控制。入院时,她在妊娠32周时出现眶周和小腿水肿。她的血清白蛋白水平为2.8g/dL,尿蛋白/肌酐比值为4.1g/g Cr。除此之外,她还患有血小板减少症、溶血性贫血以及具有血小板反应蛋白基序的解聚素样金属蛋白酶13(ADAMTS-13)活性严重缺乏。因此,她被诊断为妊娠起病的TTP所致的肾病综合征。为该患者及其婴儿进行了剖宫产,无并发症发生。随后,她的所有症状很快得到改善。由于没有ADAMTS-13抑制剂检测结果,且病情改善后ADAMTS-13活性仍持续缺乏,她被怀疑患有先天性TTP。妊娠起病的TTP可导致肾病综合征。对于妊娠起病的TTP患者,应考虑终止妊娠以保护肾功能。

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