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家族性和散发性病例中的克罗恩病严重程度

Crohn's disease severity in familial and sporadic cases.

作者信息

Carbonnel F, Macaigne G, Beaugerie L, Gendre J P, Cosnes J

机构信息

Service de Gastroentérologie et Nutrition, Hôpital Rothschild, Paris, France.

出版信息

Gut. 1999 Jan;44(1):91-5. doi: 10.1136/gut.44.1.91.

DOI:10.1136/gut.44.1.91
PMID:9862832
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1760050/
Abstract

BACKGROUND

Having a relative with inflammatory bowel disease increases the risk for Crohn's disease but may also increase its severity in affected patients.

AIMS

To evaluate the influence of a family history on Crohn's disease course and severity.

METHODS

1316 patients followed in the same unit were studied retrospectively. Age at onset, duration of illness, site, and extent of disease were determined in patients with and without a family history. Additionally, disease severity was estimated by the need for medical therapy (steroid and immunosuppressive requirement) and the frequency and extent of excisional surgery.

RESULTS

152 (12%) patients had a family history of inflammatory bowel disease. Duration of follow up was longer in patients with a family history and there were more operations for perforating complications in familial cases. However, the importance of medical therapy, and the incidence and extent of excisional surgery were similar in familial and and sporadic cases. Kaplan-Meier estimated time to prescription of immunosuppressive drugs and first intestinal resection were similar in familial and sporadic cases. When the 152 patients with familial Crohn's disease were paired for sex, location of disease at onset, date of birth, and date of diagnosis with 152 patients sporadic Crohn's disease, the disease severity remained similar in the two groups of paired patients.

CONCLUSION

Patients with Crohn's disease and a family history of inflammatory bowel disease do not have a more severe course.

摘要

背景

有炎症性肠病亲属会增加患克罗恩病的风险,但也可能增加患病患者的病情严重程度。

目的

评估家族史对克罗恩病病程和严重程度的影响。

方法

对同一科室随访的1316例患者进行回顾性研究。确定有无家族史患者的发病年龄、病程、病变部位和范围。此外,通过药物治疗需求(类固醇和免疫抑制剂需求)以及切除手术的频率和范围来评估疾病严重程度。

结果

152例(12%)患者有炎症性肠病家族史。有家族史患者的随访时间更长,家族性病例中因穿孔并发症进行的手术更多。然而,家族性和散发性病例在药物治疗的重要性、切除手术的发生率和范围方面相似。卡普兰-迈耶估计家族性和散发性病例中开始使用免疫抑制药物和首次肠道切除的时间相似。当152例家族性克罗恩病患者与152例散发性克罗恩病患者按性别、发病部位、出生日期和诊断日期进行配对时,两组配对患者的疾病严重程度仍然相似。

结论

有炎症性肠病家族史的克罗恩病患者病程并不更严重。

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本文引用的文献

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[Comparison of long-term course of perforating and non-perforating Crohn disease].[穿孔性与非穿孔性克罗恩病的长期病程比较]
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Anticipation in familial Crohn's disease.家族性克罗恩病中的遗传早现现象。
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Clinical characteristics of Crohn's disease in 72 families.72个家族中克罗恩病的临床特征
Gastroenterology. 1996 Sep;111(3):604-7. doi: 10.1053/gast.1996.v111.pm8780563.
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Familial aggregation in Crohn's disease: increased age-adjusted risk and concordance in clinical characteristics.克罗恩病的家族聚集性:年龄调整后的风险增加及临床特征的一致性
Gastroenterology. 1996 Sep;111(3):597-603. doi: 10.1053/gast.1996.v111.pm8780562.
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Gastroenterology. 1996 Sep;111(3):587-96. doi: 10.1053/gast.1996.v111.pm8780561.
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