title: Relapsing 6q24-related transient neonatal diabetes mellitus successfully treated with a dipeptidyl peptidase-4 inhibitor: a case report.
abstract: The most common form of transient neonatal diabetes mellitus (TNDM) is 6q24-related TNDM. Patients are treated with insulin during the neonatal period until spontaneous remission. However, diabetes often recurs in adolescence, and there is no standard therapy for patients with a relapse. A paternal duplication at the 6q24 critical region spanning the pleiomorphic adenoma gene-like 1 PLAGL1 gene was found in a Japanese patient with TNDM relapse. The patient was treated with a dipeptidyl peptidase-4 (DPP4) inhibitor, alogliptin, at a dose of 25 mg per day. Immediately after treatment initiation, his hemoglobin A1c (HbA1c) levels dropped from 7.0-7.5% (52-58 mmol/mol) to 6.0-6.5% (41-47 mmol/mol) and remained stable for over a year. We reported the successful treatment of relapsed 6q24-related TNDM with a DPP4 inhibitor. Although insulin has been the conventional treatment for such patients, treatments targeting the GLP1 pathway can be a useful alternative because these patients retain the β cell mass and responsiveness through G protein-coupled pathways.
authors.affiliation: Department of Pediatric Endocrinology and Metabolism, Children's Medical Center, Osaka City General Hospital, Osaka, Japan; Clinical Research Center, Osaka City General Hospital, Osaka, Japan.
authors.forename: Tohru
authors.initials: T
authors.lastname: Yorifuji
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Hashimoto
authors.forename: Rie
authors.initials: R
authors.lastname: Kawakita
authors.forename: Yuki
authors.initials: Y
authors.lastname: Hosokawa
authors.forename: Rika
authors.initials: R
authors.lastname: Fujimaru
authors.forename: Kazue
authors.initials: K
authors.lastname: Hatake
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Tamagawa
authors.forename: Hisakazu
authors.initials: H
authors.lastname: Nakajima
authors.forename: Masayo
authors.initials: M
authors.lastname: Fujii
mesh_terms: D003920:Diabetes Mellitus / Q000188:drug therapy; D054873:Dipeptidyl-Peptidase IV Inhibitors / Q000627:therapeutic use*; D006801:Humans; D007004:Hypoglycemic Agents / Q000627:therapeutic use*; D008297:Male; D010880:Piperidines / Q000627:therapeutic use; D012008:Recurrence; D016896:Treatment Outcome; D014498:Uracil / Q000031:analogs & derivatives / Q000627:therapeutic use; D055815:Young Adult; D000096803:Imprinting Disorders
chemical_list: D054873:Dipeptidyl-Peptidase IV Inhibitors; D007004:Hypoglycemic Agents; D010880:Piperidines; D014498:Uracil; C520853:alogliptin
keywords: dipeptidyl peptidase 4 inhibitors; transient neonatal diabetes