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[成人斯蒂尔病:急性发热性肝细胞溶解的一种未被认识的病因。对12例患者的研究]

[Adult Still's disease: an unrecognized cause of acute febrile hepatic cytolysis. Study of twelve patients].

作者信息

Andrès E, Ruellan A, Pflumio F, Perrin A E, Goichot B, Grunenberger F, Imler M, Schlienger J L

机构信息

Service de Médecine Interne et Nutrition, Hôpitaux Universitaires, Strasbourg, France.

出版信息

Gastroenterol Clin Biol. 2001 Apr;25(4):353-5.

Abstract

OBJECTIVE

Certain liver test abnormalities have been described in adult Still's disease. The objective of the present study was to analyze their type and frequency.

PATIENTS

In a 10 year retrospective study, patients were included if they fulfilled Kahn's and/or Yamaguchi's diagnostic criteria (median follow-up: 6.5 years).

RESULTS

Twelve patients were selected. The median age was 25 years old and the sex ratio H/F was 2.7. Fever was present in 100% of patients and hepatomegaly in 41%. Liver test abnormalities were identified in 92% of patients: moderate cytolysis (level of transaminases between 2 and 5 N) (83%), severe cytolysis (level of transaminases > 5 N) (17%), cholestasis (elevated levels of GGT and/or alkaline phosphatase) (75%), and an increase in the LDH level (41%). All these liver abnormalities resolved spontaneously or during treatment (83%), within a median of 18 days.

CONCLUSION

Our study confirms the high frequency of liver test abnormalities (> 2/3 of the patients) in adult Still's disease. These abnormalities are generally moderate and asymptomatic (3/4 of the cases), but severe cytolysis may exist. This emphasizes the need to consider a diagnosis of adult Still's disease in the presence of fever and elevated transaminase activity.

摘要

目的

成人斯蒂尔病中已描述了某些肝脏检查异常情况。本研究的目的是分析其类型和发生率。

患者

在一项为期10年的回顾性研究中,符合卡恩和/或山口诊断标准的患者被纳入研究(中位随访时间:6.5年)。

结果

选择了12例患者。中位年龄为25岁,男女比例为2.7。100%的患者有发热,41%的患者有肝肿大。92%的患者存在肝脏检查异常:中度细胞溶解(转氨酶水平在2至5倍正常上限之间)(83%)、重度细胞溶解(转氨酶水平>5倍正常上限)(17%)、胆汁淤积(γ-谷氨酰转移酶和/或碱性磷酸酶水平升高)(75%)以及乳酸脱氢酶水平升高(41%)。所有这些肝脏异常情况在中位18天内自发缓解或在治疗期间缓解(83%)。

结论

我们的研究证实了成人斯蒂尔病中肝脏检查异常的高发生率(超过2/3的患者)。这些异常情况通常为中度且无症状(3/4的病例),但可能存在重度细胞溶解。这强调了在出现发热和转氨酶活性升高时考虑成人斯蒂尔病诊断的必要性。

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