title: Several siblings with Cystic Fibrosis as a risk factor for poor outcome.
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.
authors.affiliation: Pediatric 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. Electronic address: moran.lavie@sheba.health.gov.il.
authors.forename: Moran
authors.initials: M
authors.lastname: Lavie
authors.affiliation: Pediatric 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.
authors.forename: Ofer
authors.initials: O
authors.lastname: Shemer
authors.affiliation: Pediatric 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.
authors.forename: Ifat
authors.initials: I
authors.lastname: Sarouk
authors.affiliation: Pediatric 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.
authors.forename: Bat el
authors.initials: Be
authors.lastname: Bar Aluma
authors.affiliation: Pediatric 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.
authors.forename: Adi
authors.initials: A
authors.lastname: Dagan
authors.affiliation: Pediatric 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.
authors.forename: Ori
authors.initials: O
authors.lastname: Efrati
authors.affiliation: Pediatric 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.
authors.forename: Daphna
authors.initials: D
authors.lastname: Vilozni
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy*; D018450:Disease Progression; D005192:Family Health; D005260:Female; D005541:Forced Expiratory Volume; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007557:Israel; D008137:Longitudinal Studies; D016040:Lung Transplantation / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D035781:Siblings; D055815:Young Adult
keywords: Cystic fibrosis; Outcome; Siblings