LAUSR

Fulminant type 1 diabetes mellitus (FT1DM) is a subtype of type 1 diabetes mellitus (T1DM). FT1DM is characterized by three major criteria: (i) diabetic ketosis or ketoacidosis within 1 week of the onset of hyperglycemic symptoms; (ii) glucose level ≥288 mg/dL (16.0 mmol/L) and a glycated hemoglobin (HbA1c) level <8.5% (based on the Japan Diabetes Society criteria) at diagnosis; and, (iii) urinary C-peptide level <10 μg/day or serum C-peptide level <0.3 ng/mL. The reference criteria were negative islet cell-related autoantibodies and elevated pancreatic enzymes in 98% of patients. FT1DM is rare in children, and it was reported that FT1DM accounted for 1% of child-onset T1DM; therefore, it is unclear whether pediatric FT1DM has the same characteristics as in adults. We, herein, describe the first case of FT1DM in a 10year-old girl with severe motor and intellectual disability (SMID) presenting with hypovolemic shock. A 10-year-old girl with SMID, due to severe neonatal asphyxia at birth, had no spontaneous breathing with tracheostomy, mechanical ventilator treatment with oxygen saturation, and heart rate monitoring. Enteral nutrition was provided through gastrostomy. Blood glucose level was 122 mg/dL at the time of regular visits 1 month pre-admission. Polyuria was noticed 2 days before admission by her parents. She had no prodrome or preceding signs of infections. She was brought to the emergency department of our hospital at night due to an increased heart rate. Her vital signs were as follows: blood pressure 76/51 mmHg, heart rate 105 bpm. Blood examination revealed the following results: white blood cell count, 8,600/μL; hemoglobin, 16.7 g/ dL; platelet, 189 000/μL; urine nitrogen, 31 mg/dL; creatinine, 0.19 mg/dL; Na, 155 mmol/L; K, 4.1 mmol/L; C-reactive protein, 12.8 mg/dL; amylase, 260 U/L; lipase, >3,000 U/L; elastase-1, 7,281 ng/dL; glucose, 1,083 mg/dL; HbA1c, 7.1%; glycoalbumin, 29.4 %; C-peptide immunoreactivity (CPR), 0.59 ng/ml; b-hydroxybutyrate, 2,760 lmol/L; pH, 7.128; pCO2, 30.9 mmHg; HCO3-, 9.8 mmol/L; anion-Gap, 22.5 mmol/L; lactate, 5.2 mmol/L; plasma osmolality, 382 mOsm/L; and islet related antibody, negative. Urine examination showed specific gravity >1.050; glucose, 2,000 mg/ dL; and CPR, 4.2 μg/day. Her class II human leukocyte antigen haplotype was DRB1*09: 01-DQB1*03: 03, which is reported as a susceptible haplotype in FT1DM. Enhanced CT showed no finding of acute pancreatitis despite the marked elevation of pancreatic enzymes. Cardiac ultrasound showed a collapse of the heart and inferior vena cava, indicating hypovolemic shock. Blood pressure could not be measured after hospitalization and the patient was judged to be in hypovolemic shock; therefore, saline was administered through intravenous infusion. Moreover, intravenous insulin was administered continuously after the diagnosis of FT1DM. Owing to suspicion of pancreatitis, protease inhibitor (nafamostat mesylate), and antibiotics (meropenem) were also administered. Hypovolemic shock rapidly improved after saline infusion. Blood glucose level and plasma osmolality also improved after saline infusion and continuous intravenous insulin therapy (Fig. 1), which was switched to subcutaneous injection on day 9. Discharge was allowed after parents were taught how to administer insulin through subcutaneous injection. This is the first report of pediatric FT1DM in a child with SMID. Symptoms of FT1DM are at first similar to those of flu, followed by polydipsia and polyuria due to hyperglycemia, which progresses rapidly to nausea, vomiting, and impaired consciousness caused by ketoacidosis. This patient was unable to express dry mouth and nausea. DM patients usually try to compensate for polyuria with polydipsia; however, here, the same amount of enteral nutrition was provided through gastrostomy by her parents, following a hypovolemic shock. Her condition of consciousness was unclear; therefore, her parents were only aware of the symptoms of polyuria and elevated heart rate before the development of hypovolemic shock. This patient was considered to have a high risk of sudden death due to unclear symptoms. The most prominent risk factor for sudden death in FT1DM adults is hyperglycemia, with blood glucose levels >1,000 mg/ dL. At diagnosis, the blood glucose level in our patient was greater than the cut-off, with high plasma osmolality, which is considered to be complicated with hyperosmolar hyperglycemic syndrome (HHS). Mixed HHS and diabetic Correspondence: Yuji Fujita, MD, Department of Pediatrics, Dokkyo Medical University, 880 Kitakobayashi, Mibu, Shimotsuga, Tochigi, 321-0293, Japan. Email: [email protected] Received 13 April 2020; revised 11 June 2020; accepted 17 June 2020. doi: 10.1111/ped.14357