Aim: The purpose of this study was to investigate the consequences of pregnancy on women with CF and to clarify the impact of the disease on newborn health.
Methods: It was a retrospective study; a postal questionnaire was sent to 12 woman having a registered pregnancy in National Cystic Fibrosis Registry between 1984 and 2004 (2 to 23 years after delivery).
Results: There was a 100% response rate. 16 pregnancies have been reported (4 patients had two pregnancies); there were 16 live births of which 11 (69%) were premature, 10 (62%) of newborns had low weight. There was one neonatal death. The mean prepregnancy FEV1 (% predicted) and BMI (kg/m2) were 56,6 +/- 14,8% (range 35-95) and 19,9 +/- 1,5 (range 18-23) respectively. The mean FEV1 during pregnancy was 47,7 +/- 15,1% (range 23-87), mean maternal weight gain during pregnancy was 5,5kg. There was a significant decline in FEV1 during pregnancy (16.6 +/- 6,4%, p<0.05) as compared to prepregnancy values. Two women had diabetes mellitus and two developed gestational diabetes. Mean postpregnancy (12 months after delivery) FEV1 was 54.1 +/- 18.8% (range 22-103) and mean BMI was 18.7 +/- 1.8 kg/m2 (range 15-22). There was a significant postpregnancy decline in FEV1 (6.2 +/- 10.6%, p=0.027) and in BMI (1 +/- 1.14 kg/m2, p<0.01) as compared to prepregnancy values. Microbiological status remains unchanged. 11 patients are alive; one patient died 11 years after delivery. The mean follow up time was 8.5 +/- 3.6 years (range 2-23).
Conclusions: Pregnancy was well tolerated in our population of CF women, although affected the rate of yearly decline in FEV1 and BMI. Increased risk of premature and low newborn weight were observed. All pregnancies in CF women should be planned and monitored by CF team.
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Turk Arch Pediatr
January 2025
Department of Pediatric Pulmonology, Marmara University School of Medicine, İstanbul, Türkiye.
Objective: Nebulizer contamination has potential harmful effects on the respiratory system. The aim was to investigate the contamination profile of the nebulizers in cystic fibrosis patients and evaluate the relationship between hygiene practices and microbial contamination. Materials and Methods: Microbiological swab samples were taken from 3 different locations of the nebulizers of 102 patients.
View Article and Find Full Text PDFJ Cyst Fibros
January 2025
Department NEUROFARBA, University of Florence; Paediatric and Liver Unit, Meyer Children's Hospital IRCCS, Florence, Italy.
Background: Elexacaftor-tezacaftor-ivacaftor (ETI) has significantly improved the clinical course of people with cystic fibrosis (pwCF) and eligible CFTR variants. In this study, we prospectively evaluated liver elastography, liver fibrosis indices and liver tests in children with CF aged 6-12 years started on ETI therapy.
Methods: Body mass index, sweat test, percent predicted forced expiratory volume in one second, serum markers of liver injury or portal hypertension, liver fibrosis indices, controlled attenuation parameter and liver stiffness were assessed before starting ETI and three and twelve months post-ETI, according to new international guidelines.
J Cyst Fibros
January 2025
The Lundquist Institute, Harbor-UCLA Medical Center, Torrance 90502 CA, USA. Electronic address:
Background: Cystic Fibrosis-related Bone Disease is an emerging challenge faced by 50 % of adult people with cystic fibrosis (CF). The multifactorial causes of this comorbidity remain elusive. However, congenital bone defects have been observed in animal models with CFTR mutations, suggesting its importance.
View Article and Find Full Text PDFIntroduction: The Lung Clearance Index (LCI) is an established research test, but its role in clinical decision-making is not well defined. This study estimated the proportion of treatment decisions that are changed or supported by the added information provided by LCI.
Methods: A mixed methods prospective observational study was conducted in North America.
Dig Liver Dis
January 2025
Unit of Clinical and Molecular Epidemiology, IRCCS San Raffaele Pisana, 00166 Rome, Italy; Department of Human Sciences and Quality of Life Promotion, San Raffaele University, 00166 Rome, Italy.
Background: In pediatric patients, celiac disease (CD) may influence the health-related quality of life (HRQoL).
Aims: The study aimed to assess HRQoL and further characterise the clinical factors associated with reduced HRQoL, in a large multicenter pediatric cohort with CD.
Methods: The disease-specific questionnaire CD Dutch Questionnaire (CDDUX) and the generic questionnaire Paediatric Quality of Life Inventory (PedsQL) were used to assess the HRQoL.
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