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中年男性以灾难性抗磷脂抗体综合征为首发表现的原发性抗磷脂抗体综合征。

Catastrophic antiphospholipid antibody syndrome as a first manifestation of primary antiphospholipid antibody syndrome in a middle-aged man.

机构信息

Department of Nephrology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, 74200, Pakistan.

Department of Pathology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, 74200, Pakistan.

出版信息

CEN Case Rep. 2020 May;9(2):152-158. doi: 10.1007/s13730-020-00446-0. Epub 2020 Jan 27.

Abstract

Catastrophic antiphospholipid antibody syndrome (CAPS) is a severe form of antiphospholipid antibody syndrome (APS) that sometimes represents the first manifestation of the later syndrome. The clinical manifestations of CAPS are relatively non-specific. Hence, the diagnosis may be delayed, resulting in high mortality. We herein present a case of a 40-year-old male who presented with rapid-onset renal failure, gangrene of finger and toe tips and hematological abnormalities with no underlying secondary cause for this complication. The symptoms were precipitated by febrile illness of short duration. A provisional diagnosis of CAPS was made and treatment instituted. With timely diagnosis and intervention, both the life of the patient and kidney function were salvaged. A high index of suspicion for CAPS is important as early treatment can be lifesaving.

摘要

灾难性抗磷脂抗体综合征 (CAPS) 是一种严重的抗磷脂抗体综合征 (APS) 形式,有时代表后者综合征的首次表现。CAPS 的临床表现相对非特异性。因此,诊断可能会延迟,导致高死亡率。本文介绍了 1 例 40 岁男性患者,其表现为急性发作性肾衰竭、手指和脚趾末端坏疽和血液学异常,且无潜在的继发性并发症。这些症状由短暂发热性疾病诱发。CAPS 的初步诊断成立并开始治疗。由于及时诊断和干预,患者的生命和肾功能均得以挽救。对 CAPS 的高度怀疑很重要,因为早期治疗可以挽救生命。

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