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[惠普尔病——一项具有挑战性的诊断]

[Whipple's disease--a challenging diagnosis].

作者信息

Carneiro Ana C, Lima Palmira, Barbosa Isabel P, Chaves F Carneiro

机构信息

Serviço de Medicina A, Hospital S. João, Porto.

出版信息

Acta Med Port. 2004 Nov-Dec;17(6):481-6. Epub 2005 Jan 18.

Abstract

Whipple's Disease is a rare multisystemic disease caused by Gram-positive bacillus, Tropheryma whippelii, family of Actinobacterias and group of Actinomycetes. Because the disease is rare and has different forms of presentation the diagnosis comes frequently late. The authors present the case of a white man, 50 years old, admitted with a clinical picture characterized by weight loss, abdominal pain, ascites, diarrhea. He suffered of arthralgias for four years. The diagnosis was made by biopsy of mesenteric adenopathies and liver, during the laparotomy. The biopsy specimens showed numerous aggregates of foamy macrophages containing granular periodic acid-shiff (PAS)--positive material, diastase resistant. Afterwards, the bacillus was identified by electronic microscopy. The patient was treated with trimethoprim-sulfamethoxazole. Symptoms disappeared and biological values returned to normal. The authors present the case and discuss auxiliary exams, differential diagnosis, follow-up, treatment and review the main characteristics of the disease.

摘要

惠普尔病是一种由革兰氏阳性杆菌——放线菌科放线菌属的惠普尔嗜组织细胞菌引起的罕见多系统疾病。由于该疾病罕见且有不同的表现形式,诊断往往较晚。作者报告了一例50岁白人男性病例,其临床表现为体重减轻、腹痛、腹水、腹泻。他有关节痛症状已持续四年。诊断通过剖腹手术时对肠系膜淋巴结和肝脏进行活检做出。活检标本显示大量泡沫状巨噬细胞聚集,含有颗粒状过碘酸希夫(PAS)阳性物质,耐淀粉酶消化。之后,通过电子显微镜鉴定出该杆菌。患者接受了甲氧苄啶 - 磺胺甲恶唑治疗。症状消失,各项生物学指标恢复正常。作者展示了该病例,并讨论了辅助检查、鉴别诊断、随访、治疗方法,同时回顾了该疾病的主要特征。

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