家族中有几个兄弟姐妹同时患有囊性纤维化为预后不良的危险因素
家族中有几个兄弟姐妹同时患有囊性纤维化为预后不良的危险因素
Several siblings with Cystic Fibrosis as a risk factor for poor outcome
Moran Lavieee， Ofer Shemer， Ifat Sarouk， Bat el Bar aluma， Adi Dagan， Ori Efrati， Daphna Vilozni
Background
Occurrence of Cystic Fibrosis (CF) in more than one member in a family is not uncommon. The aim of our study was to assess the influence of multiple siblings with CF on disease expression and outcome.
Methods
Study group consisted of 2-siblings (2-sibs, n = 42) or 3/4 siblings (3/4-sibs, n = 22) with CF in one family. Each sibling was matched by age, mutation, and gender to a single CF patient.
Results
3/4-sibs subgroup compared to singles showed a lower mean FEV1 with a faster decline rate (58.4 ± 27.5 vs. 72.7 ± 25.4 and −5 ± 6.4 vs. −1.7 ± 2.8 %predicted decline/year respectively, p < .05), more airway colonization by Pseudomonas aeruginosa and Mycobacterium abscessus (15 (68%) vs. 8 (36%) and 7 (32%) vs. 4 (18%), respectively, p < .05) and more lung transplants (5 (23%) vs. 2 (9%), respectively, p < .02). Last mean FEV1 within 3/4-sibs was significantly lower for the youngest sib (p < .05).
Conclusions
Three or more CF patients in one family may be a risk factor for more severe disease and poor prognosis. In our view this reflects the burden of disease on the patients and families.
respiratory medicine
January 2015Volume 109, Issue 1, Pages 74–78
DOI: http://dx.doi.org/10.1016/j.rmed.2014.11.012 |
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