Fulminant type 1 diabetes has been classified from a traditional type 1 diabetes and characterized by 1) abrupt onset, 2) diabetic ketoacidosis (DKA) at diagnosis, 3) lack of islet-related autoantibodies, 4) elevated pancreatic enzymes, and 5) difficulty in diagnosis during routine screenings (14). Flare-ups of some pancreatic exocrine enzymes have been reported for a patient with fulminant type 1 diabetes before DKA onset (3). However, those enzymes are not measured in routine blood analyses.

This report describes a case of fulminant type 1 diabetes with a flare-up of serum amylase before DKA onset, which is easily detectable on routine emergent blood examinations. We discuss the importance of hyperamylasemia at initial diagnosis of the disease.

A 40-year-old pregnant female patient presented with malaise, sore throat, and cough with neither abdominal nor back pain at 36 weeks of gestation. She was in no acute distress. Her white blood cell count was 9.5 × 109/l, C-reactive protein 18 mg/l, plasma glucose 60 mg/dl, and serum amylase 683 IU/l. Ultrasonography yielded no medically or obstetrically abnormal findings. Lack of signs of acute pancreatitis suggested acute tonsillitis or a common cold as a preliminary diagnosis.

Five days after the initial visit, she was admitted to our hospital in a coma. Her blood pressure was 129/86 mmHg; her pulse was 70 bpm. Arterial blood gas analysis showed pH 7.055 and base excess = −17.6 mEq/l. Her marked elevation of ketone bodies reflected severe ketoacidosis. Plasma glucose was 936 mg/dl, HbA1c 5.4%, serum C-peptide 0.15 ng/ml, and serum amylase 307 IU/l. Ultrasonography revealed an intrauterine fetal death and no abnormalities in her pancreas. After treatment for ketoacidosis improved her consciousness level, an emergent cesarean section was conducted. Serological testing for autoantibodies was later reported as negative for islet cell antibodies, glutamic acid decarboxylase autoantibodies, and insulinoma-associated protein 2 antibodies. Final diagnosis was made as fulminant type 1 diabetes.

A diagnosis of fulminant type 1 diabetes is not made without an initial occurrence of DKA (14). However, timely measurement of serum amylase in the appropriate high-risk group for fulminant type 1 diabetes might enable early diagnosis. Sekine et al. (3) recently reported the flare-up of trypsin, elastase I, and lipase before DKA occurrence in a patient who had never been diagnosed as having type 1 diabetes. In our case, the serum amylase concentration flared-up before DKA occurrence. It decreased gradually from 683 to 307 IU/l at the onset, which showed the same pattern of response as that reported by Sekine et al. (3), who provided no amylase levels. In patients with fulminant type 1 diabetes, hyperamylasemia at or after DKA onset is of diagnostic value (14). However, hyperamylasemia without the features of fulminant type 1 diabetes (absence of autoantibodies and normal HbA1c) cannot be used to diagnose this disease because elevated amylase levels (although mostly of salivary gland origin) frequently accompany autoimmune DKA. On the other hand, we have also provided the time course of serum amylase before DKA onset, which might be of predictive value.

The cause of fulminant type 1 diabetes involves viral infection and pregnancy because flu-like symptoms are frequent (1, 2), and some viral DNA have been detected in patients (24). Apparently, the disease’s incidence increases in the latter term of pregnancy (2, 4). In conclusion, pregnant women presenting with flu-like symptoms should have serum amylase levels measured. Subsequently, the time course of serum amylase concentration should be followed-up carefully to prevent this concealed life-threatening disease.

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Diabetes Care
Sekine N, Motokura T, Oki T, Umeda Y, Sasaki N, Hayashi M, Sato H, Fujita T, Kaneko T, Asano Y, Kikuchi K: Rapid loss of insulin secretion in a patient with fulminant type 1 diabetes mellitus and carbamazepine hypersensitivity syndrome.
Shimizu I, Makino H, Osawa H, Kounoue E, Imagawa A, Hanafusa T, Kawasaki E, Fujii Y: Association of fulminant type 1 diabetes with pregnancy.
Diabetes Res Clin Pract