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Updated: Apr 19, 2026

Cystic Fibrosis Aggregate Biofilm Model to Study Infection-relevant Gene Expression
Published on: April 18, 2025
Several siblings with Cystic Fibrosis as a risk factor for poor outcome
Moran Lavie1, Ofer Shemer1, Ifat Sarouk1
1Pediatric Pulmonary Unit and the National Center for Cystic Fibrosis, The Edmond and Lily Safra Children Hospital, Sheba Medical Center, Tel Hashomer, Israel; Sackler Medical School, Tel-Aviv University, Israel.
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.
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