[MIM 125 850, 125 851, 600 496, 601 410, 606 176, 606 391, 606 392, 606 394, 609 812, 610 410, 610 508, 610 582, 612 225, 613 370, 613 375, ]

Acronym for Maturity Onset Diabetes of Youth. Sometimes called 'monogenic' diabetes to distinguish it from diabetes type 1 and 2 which are called 'polygenic'. Autosomal dominant transmission. Familial non-insulin-dependent (at least during the first years) diabetes that causes dysfunction of β cells . Slowly progressive. Treatment with diet and oral hypoglycemic agents for several years before insulin therapy becomes necessary. Eleven subtypes have been individualized according to the enzyme deficiency and the severity of the clinical picture.

The most common forms are:

-         MODY1: mutation of the HNF4a gene coding for nuclear 4-a factor in the hepatocytes (20q); treatment: sulfonylurea

-         MODY2: mutation of the gene coding for glucokinase (7 p): treatment: diet; neonatal diabetes when homozygous; 

-         MODY3: most frequent form; mutation of the gene coding for transcription 1 factor HNF1α in the liver (12q); treatment sulfonylurea; common complications: microangiopathy especially retinal; sometimes microalbuminuria; risk of hepatic adenoma

-         MODY4: mutation of the  IPF-1 or PDX-1 (insulin promoter factor) gene (13q); the homozygous form causes agenesis of the pancreas

-         MODY5: HNF1β gene mutation (hepatic factor 2 transcription (17q); severity of the diabetes is  variable and associated with renal  (cysts, hypoplasia) (see glomerulokystic kidney disease) and genital malformations as well as a disturbance of liver tests

Anesthetic implications: 

blood glucose monitoring; in case of treatment with GLP1 receptor agonists (semaglutide), there is a significant delay in gastric emptying, with an increased risk of aspiration of gastric contents (induction of general anesthesia, sedation): the child's diabetologist should be contacted to discuss stopping this treatment several days or weeks before the operation.

References : 

• www-diabetesgenes.org
• Quintos JB, Pingul MM, Boney CM. 
  Arteriovascular calcification leading to diagnosis of maturity-onset diabetes of the youth type 3. 
  J Pediatr 2013; 163:608. 
• Jones PM, Hobai IA, Murphy PM.
  Anesthesia and glucagon-like peptide-1 receptor agonists: proceed with caution !
  Can J Anesth 2023 ; doi.org/10.1007/s12630-023-02550-y
• Benhamou D, Catargi B, Cheisson G, Cosson E, Ichai C, Jacqueminet S, Ouattara A, Tauvenon I, Valensi P.
  Agonistes du récepteur GLP1(AR-GLP1) et dérivés, vidange gastrique et anesthésie.
  Anesth Réanim 2023 ; 9 : 510-1

Updated: December 2023