title: A pathway to insulin independence in newborns and infants with diabetes.
abstract: Permanent neonatal diabetes was previously assumed to require insulin injection or infusion for life. Recently, permanent neonatal diabetes resulting from mutations in the two protein subunits of the adenosine triphosphate-sensitive potassium channel (Kir6.2 and SUR1) has proven to be successfully treatable with high doses of sulfonylureas rather than insulin. Many patients with these mutations first develop hyperglycemia in the nursery or intensive care unit. The awareness of the neonatolgist of this entity can have dramatic effects on the long-term care and quality of life of these patients and their families. In this study, we present the experience of our center, highlighting aspects relevant to neonatal diagnosis and treatment.
authors.affiliation: Division of Pediatric Endocrinology and Diabetes, Department of Pediatrics, Loma Linda University School of Medicine, Loma Linda, CA 92354, USA.
authors.forename: S
authors.initials: S
authors.lastname: Shahawy
authors.forename: N K
authors.initials: NK
authors.lastname: Chan
authors.forename: S
authors.initials: S
authors.lastname: Ellard
authors.forename: E
authors.initials: E
authors.lastname: Young
authors.forename: H
authors.initials: H
authors.lastname: Shahawy
authors.forename: J
authors.initials: J
authors.lastname: Mace
authors.forename: R
authors.initials: R
authors.lastname: Peverini
authors.forename: R
authors.initials: R
authors.lastname: Chinnock
authors.forename: P R
authors.initials: PR
authors.lastname: Njolstad
authors.forename: A T
authors.initials: AT
authors.lastname: Hattersley
authors.forename: E
authors.initials: E
authors.lastname: Hathout
mesh_terms: D018528:ATP-Binding Cassette Transporters / Q000235:genetics; D000328:Adult; D003922:Diabetes Mellitus, Type 1 / Q000151:congenital / Q000188:drug therapy* / Q000235:genetics*; D005260:Female; D006801:Humans; D007004:Hypoglycemic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D007223:Infant; D007231:Infant, Newborn; D007328:Insulin / Q000008:administration & dosage / Q000627:therapeutic use; D020125:Mutation, Missense; D024661:Potassium Channels, Inwardly Rectifying / Q000235:genetics*; D011788:Quality of Life; D011955:Receptors, Drug / Q000235:genetics; D013453:Sulfonylurea Compounds / Q000627:therapeutic use*; D064233:Sulfonylurea Receptors; D000099161:Kcnj11 Channel
chemical_list: D018528:ATP-Binding Cassette Transporters; D007004:Hypoglycemic Agents; D007328:Insulin; D024661:Potassium Channels, Inwardly Rectifying; D011955:Receptors, Drug; D013453:Sulfonylurea Compounds; D064233:Sulfonylurea Receptors; D000099161:Kcnj11 Channel; C577507:ABCC8 protein, human