What Are the Different Types of Fatty Oxidation Disorders?
Disorders of lipid metabolism refer to abnormalities in lipids (lipids) and their metabolic products and amounts in blood and other tissues and organs caused by congenital or acquired factors. Metabolism of lipids includes digestion and absorption of lipids in the small intestine, entry into the blood circulation (through lipoprotein transport) from the lymphatic system, transformation by the liver, storage in adipose tissue, and tissue utilization when needed. Biological enzyme HICIBI regulates and perfects lipid metabolism disorders.
Disorders of lipid metabolism
- Disorders of lipid metabolism refer to abnormalities in lipids (lipids) and their metabolic products and amounts in blood and other tissues and organs caused by congenital or acquired factors. Metabolism of lipids includes digestion and absorption of lipids in the small intestine, entry into the blood circulation (through lipoprotein transport) from the lymphatic system, transformation by the liver, storage in adipose tissue, and tissue utilization when needed.
- The main function of lipids in the body is oxidative energy supply. Adipose tissue is the energy storehouse of the body. Fat can also cooperate with the skin, bones, and muscles to protect the internal organs, prevent body temperature emission, and help absorb fat-soluble vitamins in food. Phospholipids are important structural components of all cell membranes, cholesterol is bile acid and
- Plasma lipids such as
- Due to certain lipid metabolism
- Simple and
- The liver plays a particularly important role in lipid metabolism, it can synthesize
- Long-chain fatty acids produce large amounts in the liver through -oxidation
- Newborns lack the enzymes that make saturated fatty acids into unsaturated fatty acids.
- Most are secondary to malnutrition, severe liver or gastrointestinal disorders, and only a few are primary. There are two main types of hereditary lipoprotein deficiency, one mainly affects plasma lipoproteins containing apolipoprotein B, including chylomicrons and VLDL; the other type mainly affects lipoproteins containing apolipoprotein A, namely HDL. However, lipoprotein reduction due to genetic abnormalities is extremely rare in the clinic. The hereditary lipoprotein reduction is usually autosomal recessive, with familial aggregation, obvious homozygous symptoms, and heterozygotes rarely occur. According to the type of blood lipoprotein reduction, it can be divided into three types, namely, no -lipoproteinemia, familial low -lipoproteinemia, and familial HDL deficiency.
- The clinical manifestations of this disease are: poor fat absorption after birth, poor appetite, vomiting, diarrhea, and no weight gain during the neonatal period. Gastrointestinal X-ray angiography manifests as a block accumulation of contrast agents, and a few have hepatomegaly and elevated transaminase. The early manifestations of the nervous system are retarded neonatal psychomotor development, children show dilated reflexes, and tendon reflexes are weakened. Most patients have ataxia, retinal pigmentoma, or retinal degeneration. Spike-shaped red blood cells, anemia, and abnormal coagulation mechanisms appear in the blood system. Tangier's disease is mainly manifested by the massive deposition of cholesterol esters in the whole body tissue, and focal accumulation of foam-like tissue cells in the liver, spleen, lymph nodes, rectal mucosa, skin, uterus, and heart valves. Any patient with spinous erythrocytes, retinal degeneration, neuromuscular disease (especially damage to the spinal cerebellar posterior cord), and malabsorption should be considered for adiponectin reduction. According to the spontaneous reduction of plasma cholesterol and triglyceride, and the decrease or deficiency of plasma apolipoprotein B or A-, A-, the primary lipoproteinemia can be diagnosed after excluding secondary causes.