Characteristics and diagnosis of fulminant type 1 diabetes mellitus

Metabolic Department of Endocrinology, Xiangya No.2 Hospital, Central South UniversityFulminant type 1 diabetes type 1 diabetes mellitus (FT


Metabolic Department of Endocrinology, Xiangya No.2 Hospital, Central South University

Fulminant type 1 diabetes type 1 diabetes mellitus (FT1D) is proposed by Japanese scholars in 2000 (T1D) subtypes. The disease of islet beta cells in a week or so to complete destruction, hyperglycemia and ketoacidosis occurs rapidly, sudden onset, severe illness, prognosis, metabolic disease is often involved in clinical science medicine and pediatrics, obstetrics and Gynecology, Department of Dermatology, Department of emergency and severe. The clinical features are not familiar to domestic doctors. In view of this, it is necessary to introduce the clinical features, diagnosis and differential diagnosis of FT1D.

Prevalence of FT1D

It is reported that the vast majority of FT1D are found in Asian countries, with few reports in caucasians. Many survey center of Japan, from 1991 to 2000 were 19.4% with ketosis or ketoacidosis in patients with T1D for FT1D; Ehime study showed that FT1D accounted for 8.9% of all T1D, accounting for 0.2% consecutive 4980 cases of diabetic patients. A retrospective study of the Seoul hospital in South Korea showed that FT1D accounted for 7.1% of newly diagnosed T1D patients, accounting for more than 30.4% of patients aged over 18 years. I study from 2001-2008 all hospital identified 11 cases of FT1D patients with diabetes, accounting for all 1.24 consecutive inpatients with diabetes%, accounting for 1.5% of the T1D patients, accounting for 10.3% of the patients with new onset T1D. FT1D recently reported there were few Caucasians, suggesting that this type of diabetes also exists in the Caucasian population, but no relevant epidemiological data. Integrated East Asian population data, the incidence of FT1D intensity distribution, and no significant differences in the prevalence rate, the rate increased with age in male and female age at onset is often smaller than male, and most associated with pregnancy. From the age of onset of age accounted for the vast majority of adults over the age of 20, which is different from the classic T1D.

Clinical features of FT1D

The etiology and pathogenesis of FT1D is not very clear, and it may be related to human leukocyte antigen (HLA) gene, viral infection and autoimmunity. Because of the low incidence of FT1D, the case is difficult to obtain, so far its clinical research is still very little. At present, the only comprehensive and the largest number of cases of FT1D is a multi center study in japan.

At home and abroad and the research, the main clinical features of FT1D patients are summarized as follows: 1 appear polyuria, polydipsia, weight loss and other symptoms of hyperglycemia to ketoacidosis in a very short time, usually within 1 weeks, the average was 4.4 days, significantly shorter than the classical T1D patients 36.4 days; II. At the onset of severe hyperglycemia, near normal glycated hemoglobin (HbA1c); more than 90% of the patients with pancreatic enzyme increased, 70% of the patients before the onset of fever, sore throat, headache, joint pain and other "flu like" or abdominal pain, diarrhea and other symptoms of abdominal discomfort, about 50% of the patients with the onset of a disturbance of consciousness. Some even had severe rhabdomyolysis, leukemoid reaction, T wave of ECG changes, atrial fibrillation and multiple organ failure; the onset of a severe ketoacidosis and electrolyte disorder, with more than 90% of the patients with ketoacidosis, often accompanied by obvious electrolyte disorder, characterized by high potassium, low sodium, hypochloraemia, at the same time can increase the blood and urine amylase. The FT1D patients at the onset of blood electrolyte disorder was significantly higher than that of classical and idiopathic T1D is more serious; the onset of islet function was almost completely lost and irreversible, the classic T1D onset are a small amount of beta cell residues, often through several years to complete loss of islet function. The FT1D patients in the onset of islet function has almost completely lost. Seoul Hospital of Korea, 7 cases of FT1D patients followed up and found no cases of honeymoon". A 7 year follow-up of a few cases in Japan showed that insulin secretion in patients with FT1D did not improve and recover after onset. In our previous study, we did not find the improvement and recovery of islet function in 1 patients during the follow-up period of 3 years for FT1D patients.

Diagnosis of FT1D

According to the Japan Diabetes Society in 2004 proposed standards, must meet the following 3 points: 1. FT1D can only be diagnosed hyperglycemic symptoms within 1 weeks appear ketosis or ketoacidosis; the first diagnosed blood glucose > 16mmol/L and HbA1c < 8.5%; the onset of fasting C peptide (pmol/L < 100 0.1nmoL/L or 0.3 g/L) and 2 hours after meal C peptide pmol/L (0.17nmol/L < 170 or 0.5 g/L).

The diagnostic supplement: support FT1D in the diagnosis of other clinical manifestations include: before the onset often prodromal symptoms such as fever, upper respiratory tract infection or gastrointestinal symptoms; islet autoantibodies such as glutamic acid decarboxylase antibody (GADA), protein tyrosine phosphatase antibody (IA-2A) and zinc transporter 8 antibody (ZnT8A) can be negative most of the patients appeared; trypsin, muscle enzymes, elevated transaminases; the disease can occur during pregnancy or after birth. For the diabetic ketosis or ketoacidosis manifestations of patients should be routinely considered or whether screening with FT1D diagnostic criteria. To meet the diagnostic criteria of second and third, but the course of more than 1 weeks, should be highly suspected as FT1D, and improve the diagnosis of islet autoantibodies, trypsin, muscle enzymes, transaminase and other related inspection. The study found that patients with FT1D serum glycated albumin (GA) and the ratio of HbA1c increased significantly, which may contribute to the FT1D and other types of diabetes identification.

Differential diagnosis of FT1D

Mainly with the classic T1D identification, the classic T1D younger age at onset, symptoms of hyperglycemia and ketoacidosis often more than 1 weeks in January, the incidence of higher level of HbA1c, GADA, IA-2A or ZnT8A positive. The FT1D increased gastrointestinal symptoms and urine amylase, especially cholelithiasis, ketoacidosis should identify with severe pancreatitis caused by secondary diabetes mellitus. The latter abdominal symptoms, signs are more obvious, blood amylase increased more than 3 times the upper limit of the normal, pancreatic enhancement of CT can help identify. When FT1D combined with abnormal myocardial enzymes and electrocardiogram changes, it needs to be differentiated from acute myocardial infarction. The latter often has the risk factors of coronary heart disease, and has the characteristics of severe chest pain, myocardial enzymes and ECG can appear a series of characteristic dynamic changes. The changes of ECG in FT1D were mostly transient and disappeared with the correction of metabolic disorder.

(source: Editorial Department of International Diabetes)

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