Primary hypertriglyceridemia in children and adolescents

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“…Regarding pharmacological management, there are no FDA-approved TG level-lowering medications for use under 18 years of age, and there are no established indications for their use in children [7,19]. Nevertheless, extrapolation of adult guidelines may be judiciously applied in this scenario [7,8,13].…”

“…A TG level greater than or equal to 100 mg/dL for children of ages 0 to 9 years and a level greater than or equal to 130 mg/dL for children of ages 10 to 19 years are considered above the 95 th percentile [3][4][5][6]. Hypertriglyceridemia can be classified as mild to borderline high (150-199 mg/dL), moderate to high (200-499 mg/dL), very high (500-999 mg/dL), severe (1000-1999 mg/dL) and very severe (> 2000 mg/ dL) [5,7,8].…”

“…Secondary hypertriglyceridemia is frequently caused by interplay of combinations of hormonal and environmental conditions, as well as genetic causes. Obesity, metabolic syndrome, insulin resistance and uncontrolled diabetes play a central role in secondary hypertriglyceridemia, but other causes have been described as shown in table 2 [7,[11][12][13].…”

Lipoprotein lipase deficiency presenting with neonatal perianal abscesses

“…Hypertriglyceridaemia presenting in the neonatal period is most commonly due to mutations in the LPL gene, which results in elevated levels of chylomicrons, while a number of other genes may be involved in genetic hypertriglyceridaemia presenting in adulthood, including APOE 6. While hypertriglyceridaemia presenting in the neonatal period is most commonly primary (genetic), the differential diagnosis of hypertriglyceridaemia in children and adolescents includes secondary causes such as hypothyroidism, obesity and the metabolic syndrome, cholestasis, nephrotic syndrome, glycogen storage disease type 1, poorly controlled diabetes and medications such as oral retinoids, the contraceptive pill and atypical antipsychotics 6 7…”

“…While hypertriglyceridaemia presenting in the neonatal period is most commonly primary (genetic), the differential diagnosis of hypertriglyceridaemia in children and adolescents includes secondary causes such as hypothyroidism, obesity and the metabolic syndrome, cholestasis, nephrotic syndrome, glycogen storage disease type 1, poorly controlled diabetes and medications such as oral retinoids, the contraceptive pill and atypical antipsychotics 6 7…”