Atypical diabetes in children: ketosis-prone type 2 diabetes

Abstract

Ketosis-prone type 2 diabetes mellitus also known as atypical or flatbush diabetes is being increasingly recognised worldwide. These patients are typically obese, middle-aged men with a strong family history of type 2 diabetes. The aetiology and pathophysiological mechanism is still unclear but some initial research suggests that patients with ketosis-prone type 2 diabetes have a unique predisposition to glucose desensitisation. These patients have negative autoantibodies typically associated with type 1 diabetes but have shown to have human leucocyte antigen (HLA) positivity. At initial presentation, there is an impairment of both insulin secretion and action. β Cell function and insulin sensitivity can be markedly improved by initiating aggressive diabetes management to allow for discontinuation of insulin therapy within a few months of treatment. These patients can be maintained on oral hypoglycaemic agents and insulin therapy can be safely discontinued after few months depending on their β cell function.

An Insight Into Flatbush Diabetes: A Rare Form of Diabetes

“…A diagnosis of KPD is based on several important hallmark characteristics: (1) an abrupt and unexplained elevation of blood glucose, (2) insulin resistance seen on initial presentation in addition to severe deficits in insulin secretion without the presence of islet autoantibodies, (3) an early recovery of insulin secretion following treatment in the acute phase of presentation, (4) later recovery of normal insulin sensitivity relative to the BMI of the patient [ 10 ]. Additionally, these patients must have an elevated ketone level that is not better explained by other precipitating factors for metabolic decompensation, such as medication or infection [ 6 ].…”

“…The patient in our case report has similar lab values and meets all the hallmark criteria, pointing to a diagnosis of KPD. Typically, patients with this subtype of diabetes can go into remission, defined as an HbA1C less than 6.3% without the use of pharmacologic agents, for up to two years at a time between episodes of unexplained and spontaneous ketosis and hyperglycemia [ 10 ].…”

“…The prevalence of KPD is highest in African-Americans and Hispanics and is estimated to be between 20-50%, with Asians and Caucasians reporting a prevalence of less than 10% [ 3 , 10 ]. Typically, these patients tend to be obese with a BMI on average above 28.5 [ 4 , 6 ], have a family history of T2DM [ 6 ], and are middle-aged males [ 10 ].…”

“…The prevalence of KPD is highest in African-Americans and Hispanics and is estimated to be between 20-50%, with Asians and Caucasians reporting a prevalence of less than 10% [ 3 , 10 ]. Typically, these patients tend to be obese with a BMI on average above 28.5 [ 4 , 6 ], have a family history of T2DM [ 6 ], and are middle-aged males [ 10 ]. While the predominance of males in KPD seems to be independent of obesity, there is no clear reason to explain the two-to-three-fold increased incidence in men [ 7 ].…”

The Influencing Factors Associated with Ketosis-Prone Type 2 Diabetes Mellitus: A Syndrome of Diabetes Mellitus

“…In any case, 2015 examination in China in the constrained discourse in the writing on the conceivable instruments hidden the overabundance vulnerability of Males to KPDM, recommended that the way of life and sexual orientation hormones in Males might be capable. Be that as it may, regardless of whether and to what degree those components add to the marvel of male prevalence in KPDM is as yet a debate [22]. In a 2004 Sub-Saharan African examination, it was discovered that family history is unequivocally connected with ketosis in T 2 DM [8].…”