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Hyperlipoproteinemia Type V

A severe type of hyperlipidemia, sometimes familial, that is characterized by the elevation of both plasma CHYLOMICRONS and TRIGLYCERIDES contained in VERY-LOW-DENSITY LIPOPROTEINS. Type V hyperlipoproteinemia is often associated with DIABETES MELLITUS and is not caused by reduced LIPOPROTEIN LIPASE activity as in HYPERLIPOPROTEINEMIA TYPE I .
Also Known As:
Hyperchylomicronemia Late Onset; Hyperchylomicronemia With Hyperprebetalipoproteinemia, Familial; Hyperchylomicronemia, Late-Onset; Hyperlipemia, Combined Fat And Carbohydrate-Induced; Hyperlipemia, Mixed; Hyperlipidemia, Type V; Hyperlipoproteinemia Type 5; Hyperlipoproteinemia, Type V; Hyperchylomicronemia Late Onsets; Hyperchylomicronemia, Late Onset; Hyperchylomicronemias, Late-Onset; Hyperlipemia, Combined Fat And Carbohydrate Induced; Hyperlipemias, Mixed; Hyperlipidemias, Type V; Hyperlipoproteinemia Type 5s; Hyperlipoproteinemias, Type V; Late-Onset Hyperchylomicronemia; Late-Onset Hyperchylomicronemias; Mixed Hyperlipemia; Mixed Hyperlipemias; Type V Hyperlipidemia; Type V Hyperlipidemias; Type V Hyperlipoproteinemia; Type V Hyperlipoproteinemias
Networked: 101 relevant articles (0 outcomes, 9 trials/studies)

Disease Context: Research Results

Related Diseases

1. Hyperlipidemias (Hyperlipidemia)
2. Xanthomatosis (Xanthoma)
3. Hyperlipoproteinemia Type II (Familial Hypercholesterolemia)
4. Hypertriglyceridemia
5. Pancreatitis

Experts

1. Yamashita, Shizuya: 2 articles (09/2013 - 01/2012)
2. Ishibashi, Shun: 2 articles (01/2012 - 11/2005)
3. Kobayashi, Junji: 2 articles (01/2012 - 06/2005)
4. Moulin, Philippe: 2 articles (02/2010 - 10/2005)
5. Sassolas, Agnès: 2 articles (02/2010 - 10/2005)
6. Abe, Taro: 1 article (04/2022)
7. Kokutou, Hiroyuki: 1 article (04/2022)
8. Morimoto, Kozo: 1 article (04/2022)
9. Ohta, Ken: 1 article (04/2022)
10. Shimoda, Masafumi: 1 article (04/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type V:
1. LipidsIBA
2. Triglycerides (Triacylglycerol)IBA
3. Lipoproteins (Lipoprotein)IBA
4. VLDL LipoproteinsIBA
5. Insulin (Novolin)FDA Link
6. Glucose (Dextrose)FDA LinkGeneric
7. Fish OilsIBA
8. ClofibrateFDA Link
9. Apolipoprotein A-VIBA
09/01/2016 - "We report a novel homozygous apolipoprotein A5 (APOA5) frameshift mutation (c.G425del-C, p.Arg143AlafsTer57) identified in a 12-year-old boy of Pakistani origin with severe hypertriglyceridemia (up to 35 mmol/L) and type V hyperlipoproteinemia. "
04/01/2006 - "The sequencing of the APOA5 gene in patients with primary hypertriglyceridemia, in whom mutations of the LPL and APOC2 genes had been excluded, led to the identification of four families with two different mutations in this gene predicted to result in truncated apolipoprotein A-V. The first mutation (Q148X) was found in a homozygous state in a child with severe type V hyperlipidemia, some clinical manifestations of chylomicronemia syndrome and a slight reduction in plasma postheparin lipoprotein lipase activity. "
10/01/2006 - "The relevance of apolipoprotein A-V (apoA-V) for human lipid homeostasis is underscored by genetic association studies and the identification of truncation-causing mutations in the APOA5 gene as a cause of type V hyperlipidemia, compatible with an LPL-activating role of apoA-V. An inverse correlation between plasma apoA-V and triglyceride (TG) levels has been surmised from animal data. "
04/01/2006 - "Mutations in the APOA5 gene, leading to truncated apolipoprotein A-V devoid of lipid-binding domains located in the carboxy-terminal end of the protein, if present in the homozygous state, are expected to cause severe type V hyperlipidemia in patients with no mutations in LPL or APOC2 genes. "
10. EnzymesIBA

Therapies and Procedures

1. Therapeutics
2. Renal Dialysis (Hemodialysis)
3. Fat-Restricted Diet (Diet, Fat Restricted)
4. Plasmapheresis
5. Laparotomy