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Klin Padiatr 2020; 232(04): 210-216
DOI: 10.1055/a-1117-3771
Original Article

Glucose Tolerance in Patients with Cystic Fibrosis – Results from the German Cystic Fibrosis Registry

Glukosetoleranz bei Patienten mit zystischer Fibrose – Daten aus dem Deutschen Mukoviszidose Register
Nicole Prinz 
1   Institute of Epidemiology and Medical Biometry (ZIBMT), Ulm University, Ulm
2   German Center for Diabetes Research (DZD), Munich-Neuherberg
,
Julia Wosniok
3   Interdisciplinary Centre for Clinical Trials (IZKS), University Medical Centre Mainz, Mainz
,
Doris Staab
4   Paed. Pneumology, Univ. Childrenʼs Hospital Charité, Berlin
,
Manfred Ballmann
5   Pediatric Clinic, University Medicine Rostock, Rostock
,
Christian Dopfer
6   Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover
,
Nicole Regenfuß
3   Interdisciplinary Centre for Clinical Trials (IZKS), University Medical Centre Mainz, Mainz
,
Josef Rosenecker
7   Department of Pediatrics, Dr. von Hauner Childrenʼs Hospital, Ludwig Maximilian University, Munich
,
Dirk Schramm
8   Department of General Pediatrics, Neonatology, and Pediatric Cardiology, University Childrenʼs Hospital, Medical Faculty, Heinrich Heine University, Düsseldorf
,
Reinhard Holl W.
1   Institute of Epidemiology and Medical Biometry (ZIBMT), Ulm University, Ulm
2   German Center for Diabetes Research (DZD), Munich-Neuherberg
,
Lutz Nährlich
9   Department of Pediatrics, Justus-Liebig-University giessen, Giessen
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Abstract

Background Oral glucose tolerance (OGT) deteriorates progressively in cystic fibrosis (CF). Clinical registries provide a unique basis to study real-world data.

Patients & methods OGT tests (OGTTs) documented in the German CF-registry in 2016 were classified according WHO, modified by ADA: normal glucose tolerance (NGT), indeterminate glycaemia (INDET), impaired fasting glucose (IFG), impaired glucose tolerance (IGT), IFG+IGT, diabetes mellitus (DM). To study the association with lung function, multivariable regression adjusted for age, sex, and CFTR mutation was performed.

Results Overall, OGTT screening was done in 35% of CF patients ≧10 years. Of the 996 patients (46.4% females; median age (IQR): 19 (14–27) years) with evaluable OGTTs, 56.2% had either NGT or INDET, whereas 34% had a pre-diabetic OGTT (IFG; IGT; IFG+IGT) and 9.8% a diabetic OGTT. 7 patients had glucose tolerance abnormalities <10 years. DM was more common in females or patients with F508del homozygote mutation, whereas IFG was more frequent in males (all p<0.05). Nearly 75% of patients after transplantation and about half with enteral/parental nutrition and/or steroid use had either a pre-diabetic or diabetic glucose tolerance. In the adjusted model, age (p<0.001) and OGTT category (p=0.013) had both a significant impact on %FEV1.

Conclusion Our data of the German CF-registry highlights incidence of glucose tolerance abnormalities in second decade of life in CF patients. However, it also underlines the need for improvement of the documentation and/or performance of OGTT screening in real-world CF care.

Zusammenfassung

Hintergrund Bei Mukoviszidose (zystischer Fibrose: CF) verschlechtert sich die orale Glukosetoleranz (OGT) im Krankheitsverlauf.

Patienten & Methoden OGT Tests (OGTTs), die 2016 im Deutschen CF-Register dokumentiert waren, wurden gemäß WHO (modifiziert nach ADA) kategorisiert: Normale Glukosetoleranz (NGT), intermittierende Glykämie (INDET), eingeschränkte Nüchternglukosetoleranz (IFG), gestörte Glukosetoleranz (IGT), IFG+IGT, Diabetes Mellitus (DM). Um den Zusammenhang mit der Lungenfunktion zu analysieren, wurde eine multivariable Regressionsanalyse adjustiert für Alter, Geschlecht und CFTR Mutation durchgeführt.

Ergebnisse Insgesamt wurden 35% der CF-Patienten ≥10 Jahre mittels OGTT gescreent. Von den 996 Patienten (46,4% weiblich, medianes Alter (IQR): 19 (14–27) Jahre) mit auswertbaren OGTTs hatten 56,2% entweder NGT oder INDET, wohingegen bei 34% ein prädiabetischer (IFG; IGT; IFG+IGT) und bei 9,8% ein diabetischer OGTT beobachtet wurde. Bei 7 Patienten zeigten sich vor dem 10. LJ Abnormalitäten im Glukosestoffwechsel. DM war häufiger bei Frauen und Patienten mit homozygoter F508del Mutation, wobei IFG öfters bei Männern vorlag (alle p<0,05). Ca. 75% der Patienten mit Transplantation und etwa die Hälfte der Patienten mit künstlicher Ernährung und/oder Steroidgabe hatten eine prädiabetische oder diabetische Glukosetoleranz. Das Alter (p<0,001) und die OGTT Kategorie (p=0,013) zeigten im adjustierten Modell eine signifikante Assoziation mit %FEV1.

Schlussfolgerung Unsere Daten unterstreichen das Auftreten von Abnormalitäten im Glukosestoffwechsel bei CF im 2. Lebensjahrzehnt. Jedoch weißt es auf die Notwendigkeit eines regelmäßigen Diabetesscreenings und/oder Dokumentation von OGTTs bei CF hin.

Keywords

Cystic fibrosis-related diabetes - oral glucose tolerance - lung function - clinical registry

Schlüsselwörter

Mukoviszidose bedingter Diabetes - oraler Glukosetoleranztest - Lungenfunktion - klinisches Register


Publikationsverlauf

Artikel online veröffentlicht:
16. März 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 

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