Several siblings with Cystic Fibrosis as a risk factor for poor outcome

Moran Lavie, Ofer Shemer, Ifat Sarouk, Bat El Bar Aluma, Adi Dagan, Ori Efrati, Daphna Vilozni

Research output: Contribution to journalArticlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)74-78
Number of pages5
JournalRespiratory Medicine
Volume109
Issue number1
DOIs
StatePublished - 1 Jan 2015

Keywords

  • Cystic fibrosis
  • Outcome
  • Siblings

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