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164例变应性支气管肺曲霉病患者的家族发病频率。

Frequency of familial occurrence in 164 patients with allergic bronchopulmonary aspergillosis.

作者信息

Shah Ashok, Kala Jaya, Sahay Sandeep, Panjabi Chandramani

机构信息

Department of Respiratory Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, Delhi, India.

出版信息

Ann Allergy Asthma Immunol. 2008 Oct;101(4):363-9. doi: 10.1016/S1081-1206(10)60311-0.

Abstract

BACKGROUND

Asthma is known to run in families. Allergic bronchopulmonary aspergillosis (ABPA) occurs predominantly in patients with asthma. However, there are only 6 reports of familial occurrence over a period of 35 years.

OBJECTIVE

To determine the frequency of familial occurrence in 164 patients with ABPA diagnosed over a period of 22 years in one unit.

METHODS

The 164 patients with ABPA were reviewed for the occurrence of familial ABPA. Symptomatic family members were evaluated for the presence of ABPA as well as allergic Aspergillus sinusitis (AAS). Allergic bronchopulmonary aspergillosis and AAS were diagnosed as per criteria established.

RESULTS

Of the 164 patients with ABPA, familial occurrence was detected in 4 pairs of first degree relatives, 2 of whom were parent-child while the other 2 were siblings. Familial ABPA was seen in 4.9% of the total patients. Of these 8 patients seven had symptoms of rhinitis while 4 had sinusitis confirmed on computed tomography of paranasal sinuses. Allergic Aspergillus sinusitis was detected in 3 of these 4 patients. The fourth patient with sinusitis did not consent to surgery required to confirm the diagnosis. Five of our 8 patients, prior to referral, had received antituberculous therapy. All patients responded favourably to oral prednisolone.

CONCLUSION

Familial occurrence was documented in 4.9% of the 164 patients with ABPA.

摘要

背景

已知哮喘具有家族聚集性。变应性支气管肺曲霉病(ABPA)主要发生于哮喘患者中。然而,在35年的时间里仅有6例家族发病的报道。

目的

确定在一个单位22年期间诊断的164例ABPA患者中家族发病的频率。

方法

对164例ABPA患者进行家族性ABPA发病情况的回顾。对有症状的家庭成员进行ABPA以及变应性曲霉性鼻窦炎(AAS)的评估。根据既定标准诊断变应性支气管肺曲霉病和AAS。

结果

在164例ABPA患者中,在4对一级亲属中检测到家族发病,其中2对是亲子关系,另外2对是兄弟姐妹关系。家族性ABPA在所有患者中占4.9%。在这8例患者中,7例有鼻炎症状,4例经鼻窦计算机断层扫描证实有鼻窦炎。在这4例患者中有3例检测到变应性曲霉性鼻窦炎。第四例鼻窦炎患者不同意进行确诊所需的手术。我们的8例患者中有5例在转诊前接受过抗结核治疗。所有患者对口服泼尼松龙反应良好。

结论

在164例ABPA患者中,4.9%有家族发病记录。

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