A rare case of Fulminant type 1B Diabetes — ASN Events
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A rare case of Fulminant type 1B Diabetes (#321)

Mohammad Mir 1 , Louise Ciin
  1. Royal Darwin hospital, Tiwi, NT, Australia

Background: Fulminant type 1B Diabetes was first proposed in 2000. This subtype occurs in 7-20% of acute onset type 1 Diabetes in Southeast Asians1,2. Three criteria are early ketosis with short duration of preceding symptoms, high glucose with low HbA1c at presentation, low or unrecordable C-peptide1. This has negative anti-islet autoantibodies in 95%, elevated pancreatic enzymes in 98%, preceding GI or flu like symptoms in 70%, and is associated with Class 2 HLA, most probably DRB1 and DQB1 haplotypes1.

Clinical case: A 48 years old Chinese gentleman presented with newly diagnosed Diabetes and DKA following a two-day history of polyuria, polydipsia, abdominal pain, vomiting, diarrhoea and left sided chest pain. There was no preceding illness, weight loss or family history of diabetes.

Clinically, he was conscious, hypotensive, tachycardiac and dehydrated. BMI was 21. There was a strong smell of ketones but other examinations were unremarkable.

Initial Glucose 53mmol/l, Ketones 5.2, pH 6.96, HCO3 5.7, potassium 7.6 mmol/L, urea 18.5mmol/L, creatinine 264 mmol/L, lactate 8.2. He was resuscitated with intravenous fluid, insulin infusion and electrolytes correction with dramatic improvement.

His lipase was 1500U/L (23-300), peak troponin I rise 0.362 mcg/L (0.00-0.08) and CK 642 U/L (0-220).

HbA1c 7.1%. Islet cell and anti-GAD antibodies were negative, C-peptide were undetectable. Virology screen was normal.

ECGs showed concave ST elevation in all leads consistent with pericarditis. Echocardiogram showed thickened pericardium. CT pancreas was normal.

He was subsequently commenced on basal bolus insulin. His biochemistry returned to normal on the third day.

Discussion: Previously fit Chinese man presented with abrupt onset, severe metabolic derangement and pancreatic involvement, suggestive of fulminant type 1B Diabetes without insulinitis3. Hyperlipasemia correlates with degree of metabolic derangement but not with diabetic control or triglyceride level. Myopericarditis could well be due to dehydration, acidosis or uraemia. HLA typing would be helpful1.

  1. 1. Fulminant type 1 diabetes – an important subtype in East Asia. A Imagawa. Diabetes metab Res Rev 2011; 27:959-964.
  2. 2. A novel subtype of type 1 diabetes mellitus characterized by a rapid onset and an absence of diabetes related antibodies. Osaka IDDM Study Group. Imagawa A, Hahafusa T, NEJM ISSN: 0028-4793, 2000 Feb 3; Vol. 342 (5), pp. 301-7; PMID: 10655528;
  3. 3. Fulminant onset type 1 diabetes with positivity for anti-GAD antibody and elevated pancreatic exocrine enzyme concentrations. T. Kahara Internal Medicine 42: 517-720, 2003.
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