原发性纤毛运动障碍与体液免疫缺陷——是否存在关联缺失？
Primary ciliary dyskinesia and humoral immunodeficiency--is there a missing link?
作者信息
Boon Mieke, De Boeck Kris, Jorissen Mark, Meyts Isabelle
机构信息
Department of Pediatrics, Pediatric Pulmonology, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.
Department of Pediatrics, Pediatric Pulmonology, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.
出版信息
Respir Med. 2014 Jun;108(6):931-4. doi: 10.1016/j.rmed.2014.03.009. Epub 2014 Apr 2.
BACKGROUND
Primary ciliary dyskinesia (PCD) and humoral mmunodeficiency (HID) are both rare disorders which cause recurrent upper and lower respiratory tract infections.
OBJECTIVE
To examine the concurrence of PCD and HID in a patient cohort with known PCD.
METHODS
Retrospective review of the patient files.
RESULTS
We describe 11 patients of a cohort of 168 patients with PCD (6.5%) with a combination of PCD and some form of HID. The patients all presented with typical clinical symptoms for PCD, however the role of the concomitant immunological abnormalities is not clear.
CONCLUSION
PCD and HID coincided in 6.5% of the patients. We suggest that a common pathophysiological pathway results in both disorders.
背景
原发性纤毛运动障碍（PCD）和体液免疫缺陷（HID）均为罕见疾病，可导致反复的上、下呼吸道感染。
目的
在已知患有PCD的患者队列中检测PCD和HID的并发情况。
方法
对患者病历进行回顾性分析。
结果
我们描述了168例PCD患者队列中的11例患者（6.5%），他们同时患有PCD和某种形式的HID。这些患者均表现出PCD的典型临床症状，然而，伴随的免疫异常的作用尚不清楚。
结论
6.5%的患者同时患有PCD和HID。我们认为，共同的病理生理途径导致了这两种疾病。
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