Abstract
Background:
Clinical teaching for many years has been to assume that diabetic ketoacidosis is pathognomic of type 1 diabetes. However, this dogma is now increasingly questioned as increasing numbers of people are admitted with diabetic ketoacidosis without the typical features of autoimmune type 1 diabetes. Such individuals are increasingly diagnosed with ketosis-prone diabetes, a heterogenous syndrome where individuals present with diabetic ketoacidosis or unprovoked ketosis but are usually autoantibody negative and have no residual β-cell function. Classification of ketosis-prone diabetes using the Aβ system can aid in diagnosis and treatment decisions.
Case report:
An Afro-Caribbean male presented with hyperglycaemic ketosis. Initially diagnosed with type 1 diabetes and treated with a basal-bolus regimen, he was able to stop insulin completely within one month of presentation following rapid improvements in glycaemic control and repeated hypoglycaemia. Clinical investigation revealed he was negative for β-cell autoantibodies and had a stimulated C-peptide of >0.5 pmol/ml, suggesting preserved β-cell function. A diagnosis of ketosis-prone diabetes (A-β+) was made.
Discussion:
A high index of suspicion for ketosis-prone diabetes should arise when someone presents with diabetic ketoacidosis, who is obese, middle-aged, male and with a strong family history of type 2 diabetes and from a Black or Hispanic population. There is currently limited evidence on the pathophysiology of this transient β-cell defect, with suggestions that it may be related to glucotoxicity.
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