Combined Pancreatic Islet-Lung-Liver Transplantation in a Pediatric Patient with Cystic Fibrosis-Related Diabetes.

Détails

ID Serval
serval:BIB_4D9568F3A3DE
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Combined Pancreatic Islet-Lung-Liver Transplantation in a Pediatric Patient with Cystic Fibrosis-Related Diabetes.
Périodique
Hormone research in paediatrics
Auteur⸱e⸱s
Klee P., Dirlewanger M., Lavallard V., McLin V.A., Mornand A., Pernin N., Petit L.M., Soccal P.M., Wildhaber B.E., Zumsteg U., Blouin J.L., Berney T., Schwitzgebel V.M.
ISSN
1663-2826 (Electronic)
ISSN-L
1663-2818
Statut éditorial
Publié
Date de publication
2018
Peer-reviewed
Oui
Volume
90
Numéro
4
Pages
270-274
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Résumé
Cystic fibrosis-related diabetes (CFRD) is the most frequent extrapulmonary complication of cystic fibrosis (CF).
We report the first combined pancreatic islet-lung-liver transplantation in a 14-year-old adolescent. CFTR was analyzed by Sanger sequencing. Further genes were analyzed by high-throughput sequencing.
The patient was diagnosed with CF at the age of 14 months. Nine years later, after diagnosis of CFRD, the patient's BMI and lung function began to decline. Bilateral lung transplantation with simultaneous liver transplantation was performed at the age of 14.5 years. The first islet transplantation (IT) was carried out 10 days later. Six months later, C-peptide secretion after arginine stimulation showed peak values of 371 pmol/L (vs. 569 pmol/L before IT) and insulin doses had slightly increased (1.40 vs. 1.11 units/kg/day before IT). A second IT was performed at the age of 15 years, a third at 16 years. Two years after the first IT, arginine-stimulated C-peptide secretion increased to 2,956 pmol/L and insulin doses could be reduced to 0.82 units/kg/day. HbA1c decreased from 7.3% (57.4 mmol/mol) to 5.9% (41.0 mmol/mol).
IT following lung and liver transplantation, with injection of islets into a transplanted organ, is feasible. It improves C-peptide secretion, decreases insulin needs, and lowers HbA1c.
Mots-clé
Cystic fibrosis, Cystic fibrosis-related diabetes, Islets of Langerhans transplantation, Liver transplantation, Lung transplantation
Pubmed
Création de la notice
08/10/2018 9:09
Dernière modification de la notice
20/08/2019 15:02
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