title: Two cases of diabetic ketoacidosis in HNF1A-MODY linked to severe dehydration: is it time to change the diagnostic criteria for MODY?
abstract: OBJECTIVE
  Hepatocyte nuclear factor-1A maturity-onset diabetes of the young (HNF1A-MODY) is a monogenic form of diabetes caused by heterozygous mutations in HNF1A. Currently, a history of diabetic ketoacidosis (DKA) is an exclusion criterion for genetic testing for MODY.
  
  
  METHODS
  In this article, we describe two unrelated patients aged 17 and 24 years with severe DKA developed several years after the diagnosis of HNF1A-MODY.
  
  
  METHODS
  Both patients were treated with insulin, but their metabolic control was poor (HbA1c 15%, 140 mmol/mol and 13%, 119 mmol/mol, respectively) due to noncompliance and missed insulin injections. In both patients, DKA followed a course of recurrent vomiting with dehydration and prerenal acute kidney injury. Their glycemia, blood pH, and base excess at admission were 97 mmol/L [1,748 mg/dL], 6.80, and -33 mmol/L (patient 1) and 34 mmol/L [613 mg/dL], 7.03, and -14 mmol/L (patient 2).
  
  
  CONCLUSIONS
  This anecdotal observation supports the notion that a history of DKA does not exclude MODY.
authors.affiliation: Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic. stepanka.pruhova@fnmotol.cz
authors.forename: Stepanka
authors.initials: S
authors.lastname: Pruhova
authors.forename: Petra
authors.initials: P
authors.lastname: Dusatkova
authors.forename: David
authors.initials: D
authors.lastname: Neumann
authors.forename: Erik
authors.initials: E
authors.lastname: Hollay
authors.forename: Ondrej
authors.initials: O
authors.lastname: Cinek
authors.forename: Jan
authors.initials: J
authors.lastname: Lebl
authors.forename: Zdenek
authors.initials: Z
authors.lastname: Sumnik
mesh_terms: D000293:Adolescent; D000328:Adult; D003681:Dehydration / Q000150:complications*; D003924:Diabetes Mellitus, Type 2 / Q000150:complications / Q000378:metabolism; D016883:Diabetic Ketoacidosis / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism*; D051538:Hepatocyte Nuclear Factor 1-alpha / Q000378:metabolism*; D006801:Humans; D008297:Male; D055815:Young Adult
chemical_list: D051538:Hepatocyte Nuclear Factor 1-alpha