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[Hyperapo-beta-emia in children. Study of 17 families]
A Sarriá Chueca1, L Moreno Aznar, M Mur Llorente
1Departamento de Pediatría, Hospital Clínico Universitario Lozano Blesa, Zaragoza.
Insights
Children with high apolipoprotein B (apo-B) levels often have familial combined hyperlipidemia (FCH) or familial hypercholesterolemia. Early dietary intervention may prevent future high triglyceride levels in these children.
Area of Science:
- Cardiovascular Science
- Pediatric Endocrinology
Context:
- Apolipoprotein B (apo-B) is a known atherosclerotic risk factor in both adults and children.
- Familial Combined Hyperlipidemia (FCH) is associated with a phenotype of increased small, dense, apo-B-enriched low-density lipoproteins, termed Hyperapobetalipoproteinemia (Hyperapo-beta).
Purpose:
- To investigate the type of dyslipoproteinemia in children exhibiting elevated plasma apo-B levels.
- To analyze the familial patterns of dyslipoproteinemia, including FCH, Familial Hypercholesterolemia, and Polygenic Hypercholesterolemia, in relation to elevated apo-B.
Summary:
- This study focused on 19 children with elevated plasma apo-B levels, divided into two groups based on standard deviation thresholds (mean + 2 SD and mean + 3 SD).
- The majority of these children (18 out of 19) were diagnosed with type IIa hyperlipoproteinemia, with one child having type IIb.
- Within the families studied, 4 families in each group had FCH, while others had Familial Hypercholesterolemia or Polygenic Hypercholesterolemia. Families with FCH also exhibited hyperapo-beta.
Impact:
- Identifies specific hyperlipoproteinemia types in children with elevated apo-B, aiding in early diagnosis and risk stratification.
- Highlights the potential for developing high triglyceride levels in children with FCH and type IIa hyperlipoproteinemia, suggesting a need for proactive management.
- Emphasizes the role of diet in preventing future dyslipidemia complications in affected children.
Abstract:
Apolipoprotein (apo)-beta is an atherosclerotic risk factor in adults and children. In families with Familial Combined Hyperlipidemia (FCH) it has been described as a lipoprotein phenotype called Hyperapobetalipoproteinemia (Hyperapo-beta) and characterized by increased numbers of small, dense and apo-beta enriched low density lipoproteins. In our Lipids Clinic, we have studied 267 children, but for the purpose of this paper we have only taken into account the 19 of these children who showed increased plasma total apo-beta levels (Hyperapo-beta-emina). To investigate the type of dyslipoproteinemia of these children, we divided them into two groups: 1) Group 1: 10 children with apo-beta levels greater than mean + 2 standard deviations (SD); 2) Group 2: 9 children with apo-beta levels greater than mean + 3 SD. We have also studied the fathers, mothers, brothers and sisters of all the children. Only one child (Group 1) had type IIb hyperlipoproteinemia. The other children had type IIa hyperlipoproteinemia. In each group, 4 families had FCH and the others had either Familial Hypercholesterolemia or Polygenic Hypercholesterolemia. Families with FCH could also have hyperapo-beta. It is possible that in the future some children of FCH families and those with type IIa hyperlipoproteinemia will have increased plasma triglyceride levels. This could be prevented by a proper diet.