{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T16:11:07Z","timestamp":1790611867394,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2017,6,18]],"date-time":"2017-06-18T00:00:00Z","timestamp":1497744000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["098395"],"award-info":[{"award-number":["098395"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["110082\/Z\/15\/Z"],"award-info":[{"award-number":["110082\/Z\/15\/Z"]}],"id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100004440","name":"Wellcome Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004440","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000288","name":"Royal Society","doi-asserted-by":"publisher","award":["105636\/Z\/14\/Z"],"award-info":[{"award-number":["105636\/Z\/14\/Z"]}],"id":[{"id":"10.13039\/501100000288","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000272","name":"National Institute for Health Research","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000272","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Diabet. Med."],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Children with neonatal diabetes often present with diabetic ketoacidosis and hence are at risk of cerebral oedema and subsequent long\u2010term neurological deficits. These complications are difficult to identify because neurological features can also occur as a result of the specific genetic aetiology causing neonatal diabetes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case reports<\/jats:title><jats:p>We report two cases of neonatal diabetes where ketoacidosis\u2010related cerebral oedema was the major cause of their permanent neurological disability. Case 1 (male, 18\u00a0years, compound heterozygous <jats:italic><jats:styled-content style=\"fixed-case\">ABCC<\/jats:styled-content>8<\/jats:italic> mutation) and case 2 (female, 29\u00a0years, heterozygous <jats:italic><jats:styled-content style=\"fixed-case\">KCNJ<\/jats:styled-content>11<\/jats:italic> mutation) presented with severe diabetic ketoacidosis at 6 and 16\u00a0weeks of age. Both had reduced consciousness, seizures and required intensive care for cerebral oedema. They subsequently developed spastic tetraplegia. Neurological examination in adulthood confirmed spastic tetraplegia and severe disability. Case 1 is wheelchair\u2010bound and needs assistance for transfers, washing and dressing, whereas case 2 requires institutional care for all activities of daily living. Both cases have first\u2010degree relatives with the same mutation with diabetes, who did not have ketoacidosis at diagnosis and do not have neurological disability.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Ketoacidosis\u2010related cerebral oedema at diagnosis in neonatal diabetes can cause long\u2010term severe neurological disability. This will give additional neurological features to those directly caused by the genetic aetiology of the neonatal diabetes. Our cases highlight the need for increased awareness of neonatal diabetes and earlier and better initial treatment of the severe hyperglycaemia and ketoacidosis often seen at diagnosis of these children.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dme.13328","type":"journal-article","created":{"date-parts":[[2017,2,7]],"date-time":"2017-02-07T23:16:36Z","timestamp":1486509396000},"page":"1000-1004","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Hyperglycaemia\u2010related complications at the time of diagnosis can cause permanent neurological disability in children with neonatal diabetes"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6020-510X","authenticated-orcid":false,"given":"J. O.","family":"Day","sequence":"first","affiliation":[{"name":"Royal Devon and Exeter NHS Foundation Trust Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. E.","family":"Flanagan","sequence":"additional","affiliation":[{"name":"Institute of Biomedical and Clinical Science University of Exeter Medical School Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. H.","family":"Shepherd","sequence":"additional","affiliation":[{"name":"Royal Devon and Exeter NHS Foundation Trust Exeter UK"},{"name":"National Institute for Health Research (NIHR) Exeter Clinical Research Facility Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. W.","family":"Patrick","sequence":"additional","affiliation":[{"name":"Edinburgh Centre for Endocrinology and Diabetes NHS Lothian Edinburgh UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N.","family":"Abid","sequence":"additional","affiliation":[{"name":"Royal Belfast Hospital for Sick Children Belfast UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Torrens","sequence":"additional","affiliation":[{"name":"Neuroport Ltd Didcot UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. J.","family":"Zeman","sequence":"additional","affiliation":[{"name":"Royal Devon and Exeter NHS Foundation Trust Exeter UK"},{"name":"Cognitive and Behavioural Neurology University of Exeter Medical School Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K. A.","family":"Patel","sequence":"additional","affiliation":[{"name":"Royal Devon and Exeter NHS Foundation Trust Exeter UK"},{"name":"Institute of Biomedical and Clinical Science University of Exeter Medical School Exeter UK"},{"name":"National Institute for Health Research (NIHR) Exeter Clinical Research Facility Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. T.","family":"Hattersley","sequence":"additional","affiliation":[{"name":"Royal Devon and Exeter NHS Foundation Trust Exeter UK"},{"name":"Institute of Biomedical and Clinical Science University of Exeter Medical School Exeter UK"},{"name":"National Institute for Health Research (NIHR) Exeter Clinical Research Facility Exeter UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,6,18]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.81.4.318"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.12.041"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.85.1.16"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200101253440404"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.d4092"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep10358"},{"key":"e_1_2_10_8_1","first-page":"669","article-title":"Ketoacidosis at onset of type 1 diabetes mellitus in pediatric age in Spain and review of the literature","volume":"9","author":"Oyarzabal Irigoyen M","year":"2012","journal-title":"Pediatr Endocrinol Rev"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.2337\/diabetes.54.9.2503"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/sj.ejhg.5201629"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(15)60098-8"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.2471\/BLT.09.067231"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.2337\/dc15-0837"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.2337\/diacare.13.1.22"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1067\/mpd.2002.128888"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.2337\/diacare.27.7.1541"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2012-006769"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1464-5491.2006.01822.x"},{"key":"e_1_2_10_19_1","first-page":"1885","article-title":"Central brain herniation secondary to juvenile diabetic ketoacidosis","volume":"20","author":"Shrier DA","year":"1999","journal-title":"Am J Neuroradiol"}],"container-title":["Diabetic Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fdme.13328","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/dme.13328","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,2]],"date-time":"2023-10-02T13:22:40Z","timestamp":1696252960000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/dme.13328"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,6,18]]},"references-count":18,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2017,7]]}},"alternative-id":["10.1111\/dme.13328"],"URL":"https:\/\/doi.org\/10.1111\/dme.13328","archive":["Portico"],"relation":{},"ISSN":["0742-3071","1464-5491"],"issn-type":[{"value":"0742-3071","type":"print"},{"value":"1464-5491","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,6,18]]},"assertion":[{"value":"2017-02-03","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2017-06-18","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}