Goodpaste病的非典型形式。

Q4 Medicine Vnitrni lekarstvi Pub Date : 2023-01-01 DOI:10.36290/vnl.2023.062
Michaela Habánová, Petra Divácká, Jitka Řehořová, Iva Svobodová
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引用次数: 0

摘要

Goodpaste病和抗肾小球基底膜肾炎(抗GBM肾炎)是一种罕见的自身免疫性小血管血管炎,主要影响年轻男性。古德帕斯特病在肺肾综合征的鉴别诊断中起着重要作用。循环自身抗体的证据、肾脏活检的典型组织学表现、新月形肾小球肾炎的发现以及肾病综合征的临床表现在诊断中起着重要作用。我们的病例报告描述了一例年轻男性抗GBM肾炎,表现为快速进行性肾小球肾炎(RPGN),进展为透析依赖性肾衰竭。该病例的非典型症状是发展为肾病综合征伴体积依赖性高血压。该病例并发肝素诱导的血小板减少症。在联合免疫抑制治疗和血浆置换的过程中,患者的病情已经稳定。然而,肾功能没有恢复。由于免疫抑制治疗、血浆置换和血液透析,这种以前致命的疾病现在有很好的存活率。
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Atypical form of Goodpasture's disease.

Goodpasture's disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. Goodpasture's disease plays an important part in differential diagnosis of pulmonary - renal syndrome. The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure. The atypical sign of the case was development of nephrotic syndrome with volume-dependent hypertension. The case was complicated by heparin-induced thrombocytopenia. During combined immunosuppressive therapy with plasmapheresis, the condition of the patient has stabilized. However, renal function did not recover. This previously fatal disease has nowadays a very good survival rate because of immunosuppresion therapy, plasmapheresis and hemodialysis.

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来源期刊
Vnitrni lekarstvi
Vnitrni lekarstvi Medicine-Internal Medicine
CiteScore
0.50
自引率
0.00%
发文量
104
期刊介绍: Vnitřní lékařství je tiskovým orgánem České internistické společnosti České lékařské společnosti Jana Evangelisty Purkyně a Slovenskej internistickej spoločnosti Slovenskej lekárskej spoločnosti. Je vydáván nepřetržitě od roku 1955. Časopis vychází jako měsíčník, tedy 12krát do roka a podle potřeby jsou v běžném ročníku vydávána jeho suplementa, která jsou obsahově zaměřena k určitému tématu. Tematicky je časopis zaměřen široce na oblast interní medicíny se zvláštní pozorností ke kardiologii, diabetologii a poruchám metabolizmu.
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