The prevalence and etiology of extreme hypertriglyceridemia in children: Data from a tertiary children's hospital.

BACKGROUND

Extreme hypertriglyceridemia (eHTG; serum triglycerides ≥ 2000 mg/dL) poses a significant risk for acute pancreatitis. There is paucity of data regarding the prevalence and etiology of eHTG in children.

OBJECTIVE

To determine the prevalence, clinical features and etiologies of patients with eHTG at a tertiary children's hospital in the United States and in the United States National Health and Nutrition Examination Survey (NHANES).

METHODS

A retrospective analysis was conducted of the electronic medical records of the Children's Medical Center, Dallas, from 2000-2015, and the NHANES data from 2005-2014 for eHTG.

RESULTS

Of 30,623 children, 36 (∼ 1 in 1000) had eHTG and one-third of them developed acute pancreatitis. They tended to be female (61%), Hispanic (39%), and nonobese (median body mass index z-score 1.60 and 1.25 in males and females, respectively). Most patients had secondary causes such as uncontrolled diabetes mellitus (30%), L-asparaginase and high-dose corticosteroid therapy for acute lymphoblastic leukemia (28%), and sirolimus/tacrolimus therapy after solid organ transplantation (14%). Five patients (14%) had type 1 hyperlipoproteinemia (T1HLP; familial chylomicronemia syndrome). The NHANES data revealed that none of the 2362 children had eHTG, and the prevalence in adults was 0.02%.

CONCLUSIONS

Extreme HTG is rare in children and majority of the children had secondary causes. Patients with diabetes mellitus or receiving drugs, such as, L-asparaginase, corticosteroids, and sirolimus, should be closely monitored for eHTG. Prevalence of T1HLP is approximately 1 in 6000 at a tertiary care center with an estimated population prevalence of 1 in 3,00,000. Early neonatal screening and intervention for T1HLP can prevent life-threatening morbidities such as acute pancreatitis.