Primary hypertriglyceridemia in children with familial chylomicronemia syndrome

  • Dewi Jumantan Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito General Hospital, Yogyakarta, Central Java
  • Titis Widowati Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito General Hospital, Yogyakarta, Central Java
  • Irma Sri Hidayati Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito General Hospital, Yogyakarta, Central Java
  • Endy P. Prawirohartono Department of Child Health, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada/Dr. Sardjito General Hospital, Yogyakarta, Central Java
Keywords: chylomicronemia; hypertriglyceridemia; medium-chain triglyceride; low-fat complementary food

Abstract

Hypertriglyceridemia can be caused by primary (genetic) or secondary etiology. One of the primary causes is hyperlipoproteinemia type I or known as familial chylomicronemia syndrome. Familial chylomicronemia syndrome is a rare autosomal recessive disease that occurs in 1-2 per 1,000,000 people, with specific characteristic signs, namely severe increment of fasting plasma triglyceride up to 100 times the normal value (about 1500-15,000 mg/dl) caused by lipoprotein lipase (LPL) mutation.

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Published
2024-12-02
How to Cite
1.
Jumantan D, Widowati T, Hidayati I, Prawirohartono EP. Primary hypertriglyceridemia in children with familial chylomicronemia syndrome. PI [Internet]. 2Dec.2024 [cited 21Dec.2024];64(6). Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/3063
Issue
Vol 64 No 6 (2024): November 2024
Section
Pediatric Nutrition & Metabolic Disease

Copyright (c) 2024 Dewi Jumantan, Titis Widowati, Irma Sri Hidayati, Endy P. Prawirohartono

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Received 2022-06-20
Accepted 2024-12-02
Published 2024-12-02