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J Cyst Fibros. 2019 Oct 31. pii: S1569-1993(19)30916-6. doi: 10.1016/j.jcf.2019.10.009. [Epub ahead of print]

Cystic fibrosis related diabetes in Europe: Prevalence, risk factors and outcome; Olesen et al.

Author information

1
Dept of Pediatrics and Adolescent Medicine, Aarhus University Hospital, Denmark. Electronic address: hvo@dadlnet.dk.
2
Department of Medical Microbiology, Motol University Hospital and 2nd Faculty of Medicine, Charles University, Prague, Czech Republic. Electronic address: pavel.drevinek@lfmotol.cuni.cz.
3
Dutch Cystic Fibrosis Foundation (NCFS), Baarn, The Netherlands. Electronic address: V.Gulmans@ncfs.nl.
4
Paediatric Pulmonology and CF Unit, Hippokration Hospital of Thessaloniki, Aristotle University of Thessaloniki, Thessaloniki, Greece.
5
Paediatric Pulmonology, University Children`s Hospital Zurich, Zurich, Switzerland. Electronic address: andreas.jung@kispi.uzh.ch.
6
Pulmonary Institute, Schneider Children's Medical Center of Israel, Sackler Faculty of Medicine, Tel Aviv University, Israel.
7
Department of Pulmonology, Mother and Child Healthcare Institute of Serbia, Belgrade, Serbia.
8
Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. Electronic address: Muriel.thomas@sciensano.be.
9
Belgian Cystic Fibrosis Registry, Sciensano, Belgium. Electronic address: anna.zolin@unimi.it.

Abstract

BACKGROUND:

Cystic fibrosis related diabetes (CFRD) has implications for morbidity and mortality with several risk factors identified. We studied the epidemiology of CFRD in the large dataset of the European Cystic Fibrosis Society Patient registry.

METHODS:

Data on CF patients were investigated for the prevalence of CFRD as well as for any association with suggested risk factors and effects.

RESULTS:

CFRD increased by approximately ten percentage points every decade from ten years of age. Prevalence was higher in females in the younger age groups. CFRD was associated with severe CF genotypes (OR = 3.11, 95%CI: 2.77-3.48), pancreatic insufficiency (OR = 1.46, 95%CI: 1.39-1.53) and female gender (OR = 1.28, 95%CI: 1.21-1.34). Patients with CFRD had higher odds of being chronically infected with Pseudomonas aeruginosa, Burkholderia cepacia complex and Stenotrophomonas maltophilia than patients without CFRD, higher odds of having FEV1% of predicted <40% (OR = 1.82, 95%CI: 1.70-1.94) and higher odds of having BMI SDS ≤-2 than patients without CFRD (OR = 1.24, 95%CI: 1.15-1.34).

CONCLUSIONS:

Severe genotype, pancreatic insufficiency and female gender remain considerable intrinsic risk factors for early acquisition of CFRD. CFRD is associated with infections, lower lung function and poor nutritional status. Early diagnosis and aggressive treatment of CFRD are more important than ever with increasing life span.

KEYWORDS:

Body mass index; Cystic fibrosis; Diabetes; Gender; Lung function

PMID:
31680042
DOI:
10.1016/j.jcf.2019.10.009

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