BackgroundAtypical presentations of diabetes mellitus (DM) have been reported in
non-European ethnic populations under various names. It is unclear whether
those names are used for the same or different clinical phenotypes. Unclear
terminology may lead to inappropriate treatment and an underestimation of
the burden caused by atypical diabetes phenotypes overlapping with classic
types of diabetes. This review aimed to describe the terms used for atypical
forms of diabetes and to investigate whether the terms are used for similar
or different phenotypes.MethodsPubMed and Scopus were searched for relevant publications in French or
English available before 15 September 2015 using the terms: ”Atypical
diabetes”, “Malnutrition Related Diabetes Mellitus
(MRDM)”, “Fibro-calculus pancreatic diabetes (FCPD)”,
Protein deficient Pancreatic Diabetes (PDPD)”, “African
diabetes”, “Ketosis prone-type 2 diabetes”,
“tropical diabetes”, “Flatbush diabetes”,
“J-type diabetes”. Titles, abstracts screening and quality
assessment were performed by two independent authors. Observational studies
addressing atypical diabetes in humans aged 14 years and above were
included. One author extracted data from selected articles.Results22 articles among 350 identified articles were retained for data extraction.
Two atypical diabetes phenotypes were identified, each of them with a
variety of names but similar definitions. One phenotype occurred in very
thin people less than 30 years of age, typically from poor socio-economic
backgrounds and requires insulin for life. It differs from type 1 diabetes
in the tolerance of high blood glucose without ketosis in the absence of
exogenous insulin. The second phenotype resembles type1 diabetes as it
presents with ketosis at onset but responds well, as type2 diabetes, to oral
hypoglycemic drugs after initial stabilization with insulin. It occurs in
individuals who are usually over 30 years of age, with normal or overweight
and absence of auto antibodies mainly found in type 1 diabetes.ConclusionThe scarce existing literature used various terms for similar diabetes
phenotypes. Agreement on nomenclature for the various forms of diabetes
using the above reported characteristics are needed in populations where
atypical forms of diabetes exist as well as better characterization of
phenotypes and genotypes to inform evidence based treatment.