Aljouda, L.; Nagy, L.; Schulze, A. Long-Term Treatment of Lipoprotein Lipase Deficiency with Medium-Chain Triglyceride-Enriched Diet: A Case Series. Nutrients2023, 15, 3535.
Aljouda, L.; Nagy, L.; Schulze, A. Long-Term Treatment of Lipoprotein Lipase Deficiency with Medium-Chain Triglyceride-Enriched Diet: A Case Series. Nutrients 2023, 15, 3535.
Aljouda, L.; Nagy, L.; Schulze, A. Long-Term Treatment of Lipoprotein Lipase Deficiency with Medium-Chain Triglyceride-Enriched Diet: A Case Series. Nutrients2023, 15, 3535.
Aljouda, L.; Nagy, L.; Schulze, A. Long-Term Treatment of Lipoprotein Lipase Deficiency with Medium-Chain Triglyceride-Enriched Diet: A Case Series. Nutrients 2023, 15, 3535.
Abstract
Background: Lipoprotein lipase (LPL) deficiency is a genetic condition. Affected individuals typically develop symptoms before age 10 with severe hypertriglyceridemia. No pharmacological treatment has lowered triglyceride levels in LPL deficiency patients successfully. The purpose of this study is to report on clinical outcomes of patients with LPL deficiency who responded to dietary therapy with successful reduction of their triglyceride (TG) levels with a long-chain triglyceride (LCT)-restricted, medium-chain triglyceride (MCT)-supplemented diet.
Methods: A single-center retrospective case review study of LPL deficiency patients who met the inclusion criteria and were evaluated at the Hospital of Sick Children between January 2000 to December 2022. Data collected include demographic and clinical data, biochemical profiles at diagnosis and after dietary treatment initiation, and molecular results.
Results: The study included seven patients with a suspected diagnosis of LPL deficiency (hypertriglyceridemia > 20 mmol/L). Six patients had a confirmed molecular diagnosis of LPL deficiency, and one had a Glycogen storage disease 1a (GSD1a) diagnosis. After presenting with hypertriglyceridemia, an LCT-restricted and MCT-supplemented diet was initiated, resulting in immediate and sustained TG reduction in all LPL deficiency patients that adhered to the diet. Additionally, they all maintained excellent growth and development. LPL deficiency-related complications were not observed under the MCT-enriched diet, except for increased pancreatic enzymes in one patient after dietary error. As expected, the diet failed to treat hypertriglyceridemia in the GSD1 patient.
Conclusions: The dietary restriction of LCT in combination with MCT supplementation was feasible and effective in the long-term management of patients with LPL deficiency. In our experience, this treatment eliminated LPL-related complications and led to good clinical outcomes.
Keywords
lipoprotein lipase deficiency, MCT diet, inborn errors of metabolism
Subject
Medicine and Pharmacology, Endocrinology and Metabolism
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
