Transient chylomicronemia preceding the onset of insulin-dependent diabetes in a young girl with no humoral markers of islet autoimmunity

Carlo M. Barbagallo, Maurizio R. Averna, Roberto Citarrella, Manfredi Rizzo, Marco Amato, Davide Noto, Alberto Pugliese, Angelo B. Cefalù, Aldo Galluzzo, Carla Giordano

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


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 β-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 aε 3/ε3 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 β-cell destruction by direct lipid toxicity.

Original languageEnglish (US)
Pages (from-to)831-836
Number of pages6
JournalEuropean Journal of Endocrinology
Issue number6
StatePublished - Jun 2004

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology


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