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Fulminant type 1 diabetes mellitus (FT1DM) is a clinical condition that is characterized by remarkably rapid and complete pancreatic ß-cell destruction, rapid onset of hyperglycemic symptoms followed by ketoacidosis. In most cases this process takes a few days. Although rare, there have been clinical manifestations with a prolonged progress that lasts longer than one week.
This study focused on the case of a 35-monthold boy who was referred to our clinic with the diagnosis of diabetic ketoacidosis, and later had a modest elevation in hemoglobin A1c (HbA1c) levels (6.7 %) incompatible with his significantly elevated blood glucose levels. The autoantibodies against pancreatic ß-cells were negative. On the basis of these above mentioned findings, our patient was then diagnosed with fulminant type 1 diabetes mellitus.
If patients with diabetic ketoacidosis have no elevation in HbA1c levels, they should be assessed for possible clinical factors that can lead to lower detectable levels of HbA1c. Furthermore, FT1DM which is characterized by very rapid and potentially fatal progression should be considered as a differential diagnosis in these patients.
Key words: childhood, diabetic ketoacidosis, insulin dependent diabetes mellitus.
Fulminant type 1 diabetes mellitus (FT1DM) is a subtype of type 1 diabetes mellitus (T1DM) reported in 2000 by Imagawa et al1. FT1DM develops as a result of a very rapid and almost complete pancreatic ß-cell destruction. Symptoms related to hyperglycemia include polydipsia, polyuria and weight loss and tend to occur in less than a week. Symptoms tend to develop within a mean of 4.4 ±3.1 days.2 FT1DM has previously been shown to account for approximately 20% of Japanese patients diagnosed with T1DM.3 In South Korea, the prevalence of FT1DM among patients with a novel diagnosis of T1DM has been found to be 7.1%, while rates are much higher for patients with adult-onset diabetes (30.4%).4 The etiology of FT1DM has not been fully understood yet, but hereditary factors such as specific human leukocyte antigens (HLA) class, and environmental factors, such as viral infections, are thought to play crucial roles in the progress of pancreatic ß-cell dysfunction.2,5
FT1DM is characterized by onset of diabetic ketoacidosis within a short period mostly less than 7 days, normal or near-normal hemoglobin A1c (HbA1c) levels at onset of the disease, together with complete ß-cell destruction.2 The...