Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy
Sudden unexpected nocturnal death among patients with diabetes occurs approximately ten times more commonly than in the general population. Malignant ventricular arrhythmia due to Brugada syndrome has been postulated as a cause, since a glucose-insulin bolus can unmask the Brugada electrocardiograph...
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2014/647252 |
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| author | Jonathan R. Skinner Renate Marquis-Nicholson Alix Luangpraseuth Rick Cutfield Jackie Crawford Donald R. Love |
| author_facet | Jonathan R. Skinner Renate Marquis-Nicholson Alix Luangpraseuth Rick Cutfield Jackie Crawford Donald R. Love |
| author_sort | Jonathan R. Skinner |
| collection | DOAJ |
| description | Sudden unexpected nocturnal death among patients with diabetes occurs approximately ten times more commonly than in the general population. Malignant ventricular arrhythmia due to Brugada syndrome has been postulated as a cause, since a glucose-insulin bolus can unmask the Brugada electrocardiographic signature in genetically predisposed individuals. In this report we present a 16-year-old male with insulin-dependent diabetes who died suddenly at night. His diabetes had been well controlled, without significant hypoglycaemia. At autopsy, he had a full stomach and a glucose level of 7 mmol/L in vitreous humor, excluding hypoglycaemia. Genetic analysis of autopsy DNA revealed a missense mutation, c.370A>G (p.Ile124Val), in the GPD1L gene. A parent carried the same mutation and has QT prolongation. Mutations in this gene have been linked to Brugada syndrome and sudden infant death. The patient may have died from a ventricular arrhythmia, secondary to occult Brugada syndrome, triggered by a full stomach and insulin. The data suggest that molecular autopsies are warranted to investigate other cases of the diabetic dead-in-bed syndrome. |
| format | Article |
| id | doaj-art-4324aef21fd040a78a183f72e6a4f4a2 |
| institution | OA Journals |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-4324aef21fd040a78a183f72e6a4f4a22025-08-20T02:37:57ZengWileyCase Reports in Medicine1687-96271687-96352014-01-01201410.1155/2014/647252647252Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion ChannelopathyJonathan R. Skinner0Renate Marquis-Nicholson1Alix Luangpraseuth2Rick Cutfield3Jackie Crawford4Donald R. Love5Cardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New ZealandDiagnostic Genetics, LabPlus, Auckland City Hospital, Auckland 1148, New ZealandSchool of Biological Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New ZealandDiabetes Service, North Shore Hospital, Takapuna, Auckland 0622, New ZealandCardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New ZealandCardiac Inherited Disease Group, Auckland City Hospital, Auckland 1148, New ZealandSudden unexpected nocturnal death among patients with diabetes occurs approximately ten times more commonly than in the general population. Malignant ventricular arrhythmia due to Brugada syndrome has been postulated as a cause, since a glucose-insulin bolus can unmask the Brugada electrocardiographic signature in genetically predisposed individuals. In this report we present a 16-year-old male with insulin-dependent diabetes who died suddenly at night. His diabetes had been well controlled, without significant hypoglycaemia. At autopsy, he had a full stomach and a glucose level of 7 mmol/L in vitreous humor, excluding hypoglycaemia. Genetic analysis of autopsy DNA revealed a missense mutation, c.370A>G (p.Ile124Val), in the GPD1L gene. A parent carried the same mutation and has QT prolongation. Mutations in this gene have been linked to Brugada syndrome and sudden infant death. The patient may have died from a ventricular arrhythmia, secondary to occult Brugada syndrome, triggered by a full stomach and insulin. The data suggest that molecular autopsies are warranted to investigate other cases of the diabetic dead-in-bed syndrome.http://dx.doi.org/10.1155/2014/647252 |
| spellingShingle | Jonathan R. Skinner Renate Marquis-Nicholson Alix Luangpraseuth Rick Cutfield Jackie Crawford Donald R. Love Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy Case Reports in Medicine |
| title | Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy |
| title_full | Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy |
| title_fullStr | Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy |
| title_full_unstemmed | Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy |
| title_short | Diabetic Dead-in-Bed Syndrome: A Possible Link to a Cardiac Ion Channelopathy |
| title_sort | diabetic dead in bed syndrome a possible link to a cardiac ion channelopathy |
| url | http://dx.doi.org/10.1155/2014/647252 |
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