PT  - JOURNAL ARTICLE
AU  - Alhakim, Fatima Riyadh
AU  - AlKhayyat, Haya
TI  - Two siblings with a rare type of maturity-onset diabetes of the young (MODY)
AID  - 10.1136/bcr-2022-249362
DP  - 2023 Feb 01
TA  - BMJ Case Reports
PG  - e249362
VI  - 16
IP  - 2
4099  - http://casereports.bmj.com/content/16/2/e249362.short
4100  - http://casereports.bmj.com/content/16/2/e249362.full
SO  - BMJ Case Reports2023 Feb 01; 16
AB  - Maturity-onset diabetes of the young (MODY) is a type of diabetes that does not entirely fit the usually encountered type 1 or type 2 diabetes. It is a monogenic, familial and non-autoimmune diabetes among children and young adults resulting from autosomal dominant gene mutations.MODY diagnosis is confirmed by molecular genetic testing, which is costly. Therefore, it is rarely done.Nearly 1%–6% of diabetes has a monogenic cause but this is frequently misclassified as type 1 diabetes mellitus due to the lack of genetic testing. Therefore, a correct diagnosis of MODY is crucial for determining the plan of management. Furthermore, having a patient with MODY in a family indicates screening other family members. In this case report, we highlight that paediatric diabetes cases are not always type 1 or type 2 diabetes mellitus since two siblings incidentally presented with hyperglycaemia and a confirmed diagnosis of MODY type 10 was ultimately made. Moreover, their mother who was previously labelled as a case of type 1 diabetes mellitus was diagnosed later as MODY after genetic testing.