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

doi:10.1016/j.rmed.2014.11.012

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

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

5 Scopus citations

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 language	English
Pages (from-to)	74-78
Number of pages	5
Journal	Respiratory Medicine
Volume	109
Issue number	1
DOIs	https://doi.org/10.1016/j.rmed.2014.11.012
State	Published - 1 Jan 2015

Keywords

Cystic fibrosis
Outcome
Siblings

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1016/j.rmed.2014.11.012

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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.",

keywords = "Cystic fibrosis, Outcome, Siblings",

author = "Moran Lavie and Ofer Shemer and Ifat Sarouk and {El Bar Aluma}, Bat and Adi Dagan and Ori Efrati and Daphna Vilozni",

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AU - Lavie, Moran

AU - Shemer, Ofer

AU - Sarouk, Ifat

AU - El Bar Aluma, Bat

AU - Dagan, Adi

AU - Efrati, Ori

AU - Vilozni, Daphna

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N2 - 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.

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

Abstract

Keywords

UN SDGs

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