title: Transient chylomicronemia preceding the onset of insulin-dependent diabetes in a young girl with no humoral markers of islet autoimmunity.
abstract: OBJECTIVE
  We investigated the possible causes of diabetes in a young child who presented with hyperglycemia associated with severe hypertriglyceridemia (>166 mmol/l), hypercholesterolemia (>38 mmol/l) and fasting chilomicrons.
  
  
  RESULTS
  The patient did not have any of the HLA and autoantibody markers typically associated with type 1 diabetes. A glucose clamp failed to demonstrate insulin resistance (peripheral glucose utilization rate (M)=4.3 mg/kg per min) and there was no family history of type 2 diabetes or maturity onset diabetes in youth. Both fasting and stimulated C-peptide levels, including those in response to i.v. glucagon, were below the limit of detection. This is consistent with loss of beta-cell function. The family history did not reveal the existence of relatives with lipid abnormalities, coronary heart disease, and pancreatitis. We did not find any abnormality of plasma apoCII, lipoproteinlipase and hepatic lipase activities. The patients had a epsilon3/epsilon3 apoE genotype and she rapidly cleared an oral fat load after normalization of plasma lipids.
  
  
  CONCLUSIONS
  The mild hyperglycemia seems an unlikely explanation for both the severe hypertriglyceridemia and chylomicronemia. A more plausible explanation is transient lipoproteinlipase deficiency. This rare condition, occasionally associated with a high-fat diet, could have caused the rapid and dramatic hypertriglyceridemia observed in this patient, which in turn might have led to the beta-cell destruction by direct lipid toxicity.
authors.affiliation: Department of Internal Medicine, Faculty of Medicine, Universita di Palermo, Italy.
authors.forename: Carlo M
authors.initials: CM
authors.lastname: Barbagallo
authors.forename: Maurizio R
authors.initials: MR
authors.lastname: Averna
authors.forename: Roberto
authors.initials: R
authors.lastname: Citarrella
authors.forename: Manfredi
authors.initials: M
authors.lastname: Rizzo
authors.forename: Marco
authors.initials: M
authors.lastname: Amato
authors.forename: Davide
authors.initials: D
authors.lastname: Noto
authors.forename: Alberto
authors.initials: A
authors.lastname: Pugliese
authors.forename: Angelo B
authors.initials: AB
authors.lastname: Cefalù
authors.forename: Aldo
authors.initials: A
authors.lastname: Galluzzo
authors.forename: Carla
authors.initials: C
authors.lastname: Giordano
mesh_terms: D001323:Autoantibodies / Q000097:blood; D015551:Autoimmunity; D002096:C-Peptide / Q000097:blood; D002648:Child; D002914:Chylomicrons / Q000097:blood*; D003922:Diabetes Mellitus, Type 1 / Q000097:blood* / Q000175:diagnosis / Q000209:etiology*; D005215:Fasting; D005260:Female; D005934:Glucagon; D015309:Glucose Clamp Technique; D006801:Humans; D006937:Hypercholesterolemia; D006943:Hyperglycemia; D015228:Hypertriglyceridemia; D007515:Islets of Langerhans / Q000276:immunology*; D008071:Lipoprotein Lipase / Q000172:deficiency
chemical_list: D001323:Autoantibodies; D002096:C-Peptide; D002914:Chylomicrons; D005934:Glucagon; D008071:Lipoprotein Lipase