Relapsed 6q24-related transient neonatal diabetes mellitus successfully treated with sulfonylurea

[...]we report a Chinese male patient with a history of 6q24-related TNDM (hypomethylation of the maternal allele) who relapsed at 14 years old and was successfully treated with a low dose of glimepiride. Whole exome sequencing revealed no mutations. [...]the patient was treated with Lantus (8 U/day...

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Bibliographic Details
Published in:Chinese medical journal 2019-04, Vol.132 (7), p.846-848
Main Authors: Fu, Jun-Ling, Wang, Tong, Xiao, Xin-Hua
Format: Article
Language:English
Subjects:
Birth weight
Chromosomes
Correspondence
Diabetes
Endocrinology
Fasting
Genomes
Glucose
Hypoglycemia
Insulin
Kinases
Laboratories
Medical schools
Patients
Semiconductors
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Summary:[...]we report a Chinese male patient with a history of 6q24-related TNDM (hypomethylation of the maternal allele) who relapsed at 14 years old and was successfully treated with a low dose of glimepiride. Whole exome sequencing revealed no mutations. [...]the patient was treated with Lantus (8 U/day) together with lifestyle modifications. [2] Given that patients with 6q24-related TNDM have relatively healthy beta cell function at remission, sulfonylurea treatment has a biological rationale in patients with 6q24-related TNDM, as its potential mechanisms are: (1) patients with 6q24-related TNDM presumably have intact sulfonylurea receptors at the pancreatic ß-cell and (2) the beta cells of patients with 6q24-related TNDM have decreased sensitivity to glucose, so sulfonylurea helps to improve the release of insulin.
ISSN:0366-6999
2542-5641
DOI:10.1097/CM9.0000000000000147