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Clinical Characteristics and Sequelae of Severe Hypertriglyceridemia in Pediatrics
Insights
Severe hypertriglyceridemia in children is often secondary to other conditions like diabetes or cancer, and frequently persists. Management requires addressing underlying causes for better triglyceride level control.
Area of Science:
- Pediatric Endocrinology
- Metabolic Disorders
- Cardiovascular Health
Background:
- Severe hypertriglyceridemia (HTG), defined as plasma triglycerides (TGs) >1,000 mg/dL, is rare in children but poses significant health risks.
- This condition is understudied, necessitating a comprehensive evaluation of its causes, clinical features, and outcomes in pediatric populations.
Purpose of the Study:
- To investigate the various etiologies contributing to severe pediatric HTG.
- To characterize the clinical presentation and identify associated complications of severe HTG in children.
- To assess the long-term sequelae and management challenges of severe HTG in this age group.
Main Methods:
- A retrospective review of electronic medical records was conducted for pediatric patients diagnosed with severe HTG.
- The study encompassed a 17-year period at a tertiary referral children's hospital.
- Data analysis focused on identifying underlying causes, TG levels, time to normalization, and comorbidities.
Main Results:
- A total of 124 pediatric patients with severe HTG were identified.
- Common etiologies included hemato-oncologic (48), diabetes/insulin resistance (46), renal (12), and total parenteral nutrition (TPN)-related (6) causes.
- Pancreatitis occurred in 11 patients, and 54% experienced persistent HTG, particularly those with diabetes, who required longer for TG normalization.
Conclusions:
- Severe pediatric HTG is predominantly caused by secondary factors, with diabetes and insulin resistance being significant contributors.
- Patients with diabetes often face a prolonged course of dyslipidemia.
- Persistent dyslipidemia suggests an underlying genetic predisposition exacerbated by secondary metabolic insults.
Objective:
Severe hypertriglyceridemia (HTG) (i.e., plasma triglycerides [TGs] >1,000 mg/dL) in children is a rare but pernicious and understudied condition. Our objective was to evaluate the etiology, characteristics, and sequelae of severe pediatric HTG.
Methods:
This was a retrospective electronic medical record review of pediatric patients with severe HTG at a tertiary referral Children's hospital over a 17-year period.
Results:
There were a total of 124 patients with severe HTG. The etiology varied: hemato-oncologic (n = 48), diabetes and insulin resistance-related (n = 46), total parenteral nutrition (TPN)-related (n = 6), renal (n = 12), and miscellaneous (n = 12). There was considerable variability in the number of days for the plasma TGs to decrease to <1,000 mg/dL (147.7 ± 567.3 days) and to further decrease to <500 mg/dL (136.84 ± 230.9 days). Patients with diabetes required the longest time to improve their plasma TGs (165.8 ± 305.7 days) compared to other groups. There were 11 cases of pancreatitis, comorbid with diabetes (n = 5), hemato-oncologic conditions (n = 3), and TPN (n = 3). Sixty-seven patients (54%) had persistent HTG.
Conclusion:
Severe HTG in pediatrics is commonly due to secondary causes. Patients with diabetes tend to have a longer course of dyslipidemia. A substantial number of patients had persistent dyslipidemia, indicating underlying genetic susceptibility to HTG that is phenotypically expressed consequent to a secondary metabolic insult.
Abbreviations:
DKA = diabetic ketoacidosis; EMR = electronic medical record; GSD = glycogen storage disorder; HbA1c = hemoglobin A1c; HIV = human immunodeficiency virus; HTG = hypertriglyceridemia; ICD-9 = International Classification of Diseases-Ninth Revision; IV = intravenous; LCHAD = long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency; LPL = lipoprotein lipase; NPO = nothing by mouth; PCOS = polycystic ovary syndrome; T1DM = type 1 diabetes mellitus; T2DM = type 2 diabetes mellitus; TG = triglyceride; TPN = total parenteral nutrition; VLDL = very-low-density lipoprotein.
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