{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T10:10:36Z","timestamp":1779358236885,"version":"3.51.4"},"reference-count":5,"publisher":"American Diabetes Association","issue":"9","license":[{"start":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T00:00:00Z","timestamp":1630454400000},"content-version":"vor","delay-in-days":2941,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0\/"}],"content-domain":{"domain":["diabetesjournals.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2013,9,1]]},"abstract":"<jats:sec>\n                  <jats:title>OBJECTIVE<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>HISTORY AND EXAMINATION<\/jats:title>\n                  <jats:p>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.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>INVESTIGATION<\/jats:title>\n                  <jats:p>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 \u221233 mmol\/L (patient 1) and 34 mmol\/L [613 mg\/dL], 7.03, and \u221214 mmol\/L (patient 2).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>CONCLUSIONS<\/jats:title>\n                  <jats:p>This anecdotal observation supports the notion that a history of DKA does not exclude MODY.<\/jats:p>\n               <\/jats:sec>","DOI":"10.2337\/dc13-0058","type":"journal-article","created":{"date-parts":[[2013,4,23]],"date-time":"2013-04-23T08:26:31Z","timestamp":1366705591000},"page":"2573-2574","update-policy":"https:\/\/doi.org\/10.2337\/ada-journal-policies","source":"Crossref","is-referenced-by-count":35,"title":["Two Cases of Diabetic Ketoacidosis in HNF1A-MODY Linked to Severe Dehydration"],"prefix":"10.2337","volume":"36","author":[{"given":"Stepanka","family":"Pruhova","sequence":"first","affiliation":[{"name":"Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Dusatkova","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Neumann","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Faculty of Medicine Hradec Kralove, Charles University in Prague and University Hospital, Hradec Kralove, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Hollay","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ondrej","family":"Cinek","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jan","family":"Lebl","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zdenek","family":"Sumnik","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1167","published-online":{"date-parts":[[2013,8,13]]},"reference":[{"key":"2022031301555155000_B1","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1111\/j.1399-5448.2009.00571.x","article-title":"The diagnosis and management of monogenic diabetes in children and adolescents","volume":"10","author":"Hattersley","year":"2009","journal-title":"Pediatr Diabetes"},{"key":"2022031301555155000_B2","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1007\/s00125-001-0770-9","article-title":"The genetic abnormality in the beta cell determines the response to an oral glucose load","volume":"45","author":"Stride","year":"2002","journal-title":"Diabetologia"},{"key":"2022031301555155000_B3","doi-asserted-by":"crossref","first-page":"131","DOI":"10.2337\/diacare.24.1.131","article-title":"Management of hyperglycemic crises in patients with diabetes","volume":"24","author":"Kitabchi","year":"2001","journal-title":"Diabetes Care"},{"key":"2022031301555155000_B4","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1038\/ncpendmet0778","article-title":"Clinical implications of a molecular genetic classification of monogenic beta-cell diabetes","volume":"4","author":"Murphy","year":"2008","journal-title":"Nat Clin Pract Endocrinol Metab"},{"key":"2022031301555155000_B5","doi-asserted-by":"crossref","first-page":"2504","DOI":"10.1007\/s00125-010-1799-4","article-title":"Maturity-onset diabetes of the young (MODY): how many cases are we missing?","volume":"53","author":"Shields","year":"2010","journal-title":"Diabetologia"}],"container-title":["Diabetes Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/diabetesjournals.org\/care\/article-pdf\/36\/9\/2573\/619355\/2573.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/diabetesjournals.org\/care\/article-pdf\/36\/9\/2573\/619355\/2573.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,13]],"date-time":"2022-03-13T06:00:40Z","timestamp":1647151240000},"score":1,"resource":{"primary":{"URL":"https:\/\/diabetesjournals.org\/care\/article\/36\/9\/2573\/37823\/Two-Cases-of-Diabetic-Ketoacidosis-in-HNF1A-MODY"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,8,13]]},"references-count":5,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2013,8,13]]},"published-print":{"date-parts":[[2013,9,1]]}},"URL":"https:\/\/doi.org\/10.2337\/dc13-0058","relation":{},"ISSN":["0149-5992","1935-5548"],"issn-type":[{"value":"0149-5992","type":"print"},{"value":"1935-5548","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8,13]]}}}