Early treatment of neonatal diabetes with oral glibenclamide in an extremely preterm infant

Author:

Galderisi Alfonso12ORCID,Kermorvant‐Duchemin Elsa34,Daruich Alejandra45,Bonnard Adeline Alice67,Lapillonne Alexandre8,Aubelle Marie‐Stéphanie9,Perrella Bruna9,Vial Yoann67,Cave Héléne67,Berdugo Marianne4,Jarreau Pierre‐Henri9,Polak Michel1101112,Beltrand Jacques11112

Affiliation:

1. Hôpital Universitaire Necker‐Enfants Malades, Service d'endocrinologie Gynécologie et Diabétologie Pédiatrique Hôpital Necker‐Enfants Malades Paris France

2. Department of Woman and Child's Health University of Padova Padova Italy

3. Department of Neonatal Medicine Hôpital Universitaire ‐ Enfants Malades, Université Paris Cité Paris France

4. Inserm, Centre de Recherche des Cordeliers, Sorbonne University Paris Cité University, Physiopathology of Ocular Diseases: Therapeutic Innovations Paris France

5. Ophthalmology Department Necker‐Enfants Malades University Hospital, Assistance Publique Hôpitaux de Paris, Paris Cité University Paris France

6. Département de Génétique Hôpital Universitaire Robert Debré Paris France

7. INSERM UMR_S1131 ‐ Institut de Recherche Saint‐Louis Paris France

8. Hôpital Universitaire Necker‐Enfants Malades, Service de Pédiatrie et Réanimation Néonatales Université Paris Cité Paris France

9. Neonatal Intensive Care Unit of Port‐Royal APHP. Centre ‐ Université Paris Cité, APHP Paris France

10. Institut IMAGINE, INSERM U1163 Paris France

11. Institut Cochin INSERM U1016 Paris France

12. Centre des maladies endocriniennes rares de la croissance et du développement Hôpital universitaire Necker‐Enfants malades Paris France

Abstract

AbstractEarly treatment of neonatal diabetes with sulfonylureas has been proven to produce marked improvements of neurodevelopment, beside the demonstrated efficacy on glycemic control. Several barriers still prevent an early treatment in preterm babies including the limited availability of suitable galenic form of glibenclamide. We adopted oral glibenclamide suspension (Amglidia) for the early treatment of neonatal diabetes due to an homozygous variant of KCNJ11 gene c.10C>T [p.Arg4Cys] in an extremely preterm infant born at 26 + 2 weeks' of gestational age. After ~6 weeks of insulin treatment with a low glucose intake (4.5 g/kg/day), the infant was switched to Amglidia 6 mg/ml diluted in maternal milk, via nasogastric tube (0.2 mg/kg/day) progressively reduced to 0.01 mg/kg/day (after ~3 months). While on glibenclamide, the patient exhibited a mean daily growth of 11 g/kg/day. The treatment was suspended at month 6 of birth (weight 4.9 kg [5th–10th centile], M3 of c.a.) for normalization of glucose profile. During the treatment, the patient exhibited a stable glucose profile within the range of 4–8 mmol/L in the absence of hypo or hyperglycemic episodes with 2–3 blood glucose tests per day. The patient was diagnosed with retinopathy of prematurity Stade II in Zone II without plus disease at 32 weeks, with progressive regression and complete retinal vascularization at 6 months of birth. Amglidia could be regarded as the specific treatment for neonatal diabetes even in preterm babies due to its beneficial effect on the metabolic and neurodevelopmental side.

Publisher

Wiley

Subject

Biochemistry, Genetics and Molecular Biology (miscellaneous),Endocrinology, Diabetes and Metabolism,Internal Medicine

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