Endocrine Abstracts

INTRODUCTION

Type IV hypertriglyceridemia (HTG IV) or endogenous HTG is a rare inheriteddisorder of the lipoprotein metabolism due to increased hepatic triglyceride synthesis(TG). Although HTGIV is not usually expressed until adulthood, it might be unmasked earlier. We report the case of an early and severe HTGIV complicated with chronic pancreatitis.

Case report

A 6-year-old childwith no particular history was hospitalized in the paediatric department of the UHC Hedi Chaker sfax, Tunisia for an acute pancreatitis (amylasemiawas 264 IU/l). The appearance of the fasting serum was lactescent. The triglyceride level was high at 5.4 g/l with VLDL at 74% without chylomicrons, cholesterol levelwas normal at 1.65 g/l. The diagnosis of primary HTG type IV was retained after exclusion of secondary causes. Dietary modifications were initiated and then at the age of ten a pharmalogical treatment (fibrates) was added. Despite treatment, TG levels were always high and the child has several episodes of acute pancreatitis. At the age of 18, he was hospitalized in the endocrinology department of the UHC hedi Chaker Sfax, Tunisia for paroxysmal abdominal pain and polyuropolydipsic syndrome. A weightloss of 5 kg in 1 year has been noted. The biological assessment showed a blood sugar level of 21 mmol/l with a glycated hemoglobin at 14.7%, without acute metabolic complication. The liver profile was normal but a high lipasemia level of 3 times the upper limit was noted, hence an abdominal CT scan was done revealing an appearance of acute cephalic pancreatitis with atrophy of the pancreatic tail. The diagnosis of diabetes secondary to chronic pancreatitis complicating HTG IV was most likely, but antibodies directed to the pancreas were requested as well.

Discussion–conclusion

Primary HTG can be discovered at an early age. His management is difficult : dietary restrictions remains the mainstory of therapy supplemented by TG levellowering medications. Close monitoring is alsomandatory due to the risk of complications.