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一名13个月大婴儿的淋巴瘤样肉芽肿病。

Lymphomatoid granulomatosis in a 13-month-old infant.

作者信息

Lehman T J, Church J A, Isaacs H

机构信息

Division of Pediatric Rheumatology, Hospital for Special Surgery, New York, NY 10021.

出版信息

J Rheumatol. 1989 Feb;16(2):235-8.

PMID:2545878
Abstract

Lymphomatoid granulomatosis is an infrequent, progressive, and frequently fatal vasculitis which typically occurs in middle aged males. We describe a 13-month-old infant with lymphomatoid granulomatosis who presented with chronic otitis media and "failure to thrive." Although it is well recognized that rheumatic conditions may result in failure to thrive, pediatricians often fail to consider this possibility and the proper diagnosis and treatment are correspondingly delayed. Lymphomatoid granulomatosis occurs with increased frequency in immunocompromised patients. In our case both hypogammaglobulinemia and persistent evidence of Epstein-Barr virus infection suggest that the infant was immunocompromised. Whether these factors predisposed this infant to lymphomatoid granulomatosis is uncertain. No patient with the onset of lymphomatoid granulomatosis prior to 7 years of age has been reported.

摘要

淋巴瘤样肉芽肿病是一种罕见的、进行性的且通常致命的血管炎,多见于中年男性。我们描述了一名13个月大患有淋巴瘤样肉芽肿病的婴儿,其表现为慢性中耳炎和“发育不良”。尽管人们普遍认识到风湿性疾病可能导致发育不良,但儿科医生常常未能考虑到这种可能性,相应地,正确的诊断和治疗也被延误。淋巴瘤样肉芽肿病在免疫功能低下的患者中发病率更高。在我们的病例中,低丙种球蛋白血症和爱泼斯坦-巴尔病毒感染的持续证据均表明该婴儿免疫功能低下。这些因素是否使该婴儿易患淋巴瘤样肉芽肿病尚不确定。此前尚无7岁前发病的淋巴瘤样肉芽肿病患者的报道。

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