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[伴有葡萄膜炎、感染、心内膜受累及腹部淋巴瘤的惠普尔病隐匿病程——病例报告及文献复习]

[Masked course of Whipple disease with uveitis, infection, endocardial involvement and abdominal lymphomas--case report and review of the literature].

作者信息

Hollerbach S, Holstege A, Muscholl M, Mohr V, Rüschoff J, Geissler A, Schölmerich J

机构信息

Kliniken und Polikliniken für Innere Medizin I, Universität Regensburg.

出版信息

Z Gastroenterol. 1995 Jul;33(6):362-7.

PMID:7545342
Abstract

Whipple's disease is a systemic disease which may virtually affect any organ system, but in many cases it involves the small intestine causing gastrointestinal symptoms. The differential diagnosis is difficult since symptoms may be nonspecific. We report the case of a 44-year old white male patient with a history of migrating arthralgia and chronic fatigue. The patient newly developed an uveitis and underwent a vitrectomy; the further clinical work-up including gastroscopy with intestinal biopsy revealed no sufficient diagnosis. Subsequently, the patient's condition deteriorated with marked weight loss, fever and progressive weakness. An anaerobic sepsis with a corynebacterium was confirmed and with i.v.-antibiotics the patients's condition improved markedly. The further examinations disclosed enlarged mesenteric lymph nodes and the involvement of other organs (endocard, liver). CT-guided biopsy only showed fatty degeneration, but operative adenectomy confirmed Whipple's disease. The patient remained without relapse on long-term antibiotic treatment with doxycycline until today. Obviously, in our case the intestinal biopsies failed to detect Whipple's disease after the successful initiation of antibiotic treatment. In the absence of gastrointestinal findings and with concomitant secondary diseases the definitive diagnosis can be difficult. In addition, the previous uveitis and the endocardial involvement are most interesting.

摘要

惠普尔病是一种全身性疾病,实际上可累及任何器官系统,但在许多情况下它会累及小肠,引起胃肠道症状。由于症状可能不具有特异性,鉴别诊断较为困难。我们报告一例44岁白人男性患者,有游走性关节痛和慢性疲劳病史。该患者新出现了葡萄膜炎并接受了玻璃体切除术;包括胃镜检查及肠道活检在内的进一步临床检查未得出明确诊断。随后,患者病情恶化,体重显著减轻、发热且进行性虚弱。确诊为棒状杆菌引起的厌氧性败血症,静脉使用抗生素后患者病情明显改善。进一步检查发现肠系膜淋巴结肿大及其他器官(心内膜、肝脏)受累。CT引导下活检仅显示脂肪变性,但手术切除淋巴结确诊为惠普尔病。该患者长期使用强力霉素进行抗生素治疗,至今未复发。显然,在我们这个病例中,抗生素治疗成功开始后,肠道活检未能检测出惠普尔病。在没有胃肠道表现且伴有继发性疾病的情况下,明确诊断可能很困难。此外,先前的葡萄膜炎和心内膜受累情况非常值得关注。

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