Two cases of diabetic ketoacidosis in HNF1A-MODY linked to severe dehydration: is it time to change the diagnostic criteria for MODY?
Language English Country United States Media print-electronic
Document type Case Reports, Journal Article, Research Support, Non-U.S. Gov't
PubMed
23610083
PubMed Central
PMC3747930
DOI
10.2337/dc13-0058
PII: dc13-0058
Knihovny.cz E-resources
- MeSH
- Dehydration complications MeSH
- Diabetes Mellitus, Type 2 complications metabolism MeSH
- Diabetic Ketoacidosis diagnosis etiology metabolism MeSH
- Adult MeSH
- Hepatocyte Nuclear Factor 1-alpha metabolism MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
- Research Support, Non-U.S. Gov't MeSH
- Names of Substances
- Hepatocyte Nuclear Factor 1-alpha MeSH
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. HISTORY AND EXAMINATION: 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. INVESTIGATION: 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.
See more in PubMed
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How can maturity-onset diabetes of the young be identified among more common diabetes subtypes?
Hidden MODY-Looking for a Needle in a Haystack
