ESPE Abstracts (2014) 82 P-D-3-3-756

Haemolysis and Acute Pancreatitis During Diabetic Ketoacidosis Treatment in a 14-Year-Old Boy with Unknown Glucose-6-Phosphate Dehydrogenase Deficiency

Federica Ortolania, Albina Tummolob, Cataldo Torellia, Maristella Masciopintoa, Stefania Fedelea, Maria Paola Lanzillottod, Francesco Nicastroa, Francesco Papadiaa, Marcella Vendemialec & Elvira Piccinnoa


aMetabolic Diseases, Clinical Genetics and Diabetology, Pediatric Hospital Giovanni XXIII, Bari, Italy; bUOC Hereditary Metabolic Diseases, Azienda Ospedaliera Policlinico di Padova, Padova, Italy; cClinical Psychology, Pediatric Hospital ‘Giovanni XXIII’, Bari, Italy; dUOC Pediatric Surgery, Pediatric Hospital Giovanni XXIII, Bari, Italy


Background: G6PD deficiency is conventionally affiliated with drug induced oxidative stress, but an association with diabetes mellitus is seldom reported. Hypertriglyceridemia from insulin deficiency can be the cause of severe pancreatitis complicating DKA in children.

Case report: A 14-year-old Bulgarian boy, no significant past medical history, hospitalized in Pediatric Surgery Department for abdominal pain, hematemesis insorted during a cruise trip. Referred recent use of aspirin, clarithromycin, vitamin C. Started i.v. physiologic saline solution and omeprazole. Naso-gastric tube drained hematic material. AP 146/83 mmHg, HR 142 b.p.m., and BR 42/min. Venous blood gas analysis: pH 6.867, Hb 15 g/dl, K 4 mmol/l, glucose 462 mg/dl, lactate 2.9 mmol/l, and BE – 30.1 mmol/l. Capillary ketonemia 4.2 mmol/l, HbA1c 10.4%. I.v. sodium bicarbonate (50 mEq HCO3), transferred to diabetology department (glycemia 486 mg/dl, and ketonemia 2.4 mmol/l), continued on i.v. fluids. Regular insulin was started (0.04 U/kg per h). Suspecting cerebral edema (lethargy, neurological state deterioration) mannitol i.v. infusion (18 g/100 cc in 20 min). Brain CT scan confirmed edema. Low grade fever (37.7 °C) PCR 170.5 mg/l: started i.v. meropenem. On day 2 venous blood pH 7.3, alpha amylase 1077 U/l (<90), and lipase 882 U/l (7–60). Ultrasound: mild heterogeneously enlarged and hypoechoic pancreas. Started gabexate mesilate and parenteral feeding. Serum triglyceride 168 mg/dl (70–150), normal total cholesterol. On day 4 jaundice and pallor. Hematological assessment: RBC 1 500 000/mm3, reticulocytes 8.7%, Hb 4.8 g/dl, Hct 14.4%, total bilirubin 5.45 mg/dl, LDH 866 U/l. G-6-PD was measured and found to be deficient (1 IU/109). Transfused with red blood cells. Antibiotic therapy integrated with clarithromycin after detection of IgM positivity for micoplasma pneumoniae. On day 7 suspended parenteral nutrition, transitioned to s.c. insulin. Continued gabexate mesilate for other 5 days. Dismissed after 18 days of hospitalization in good general condition. Now in follow up for type 1 (positive insulin autoantibodies) diabetes mellitus.