5/11/2008

Hyperlipoproteinemia the clinical manifestations

Type I Hyperlipoproteinemia

Extremely rare, a genetic disease, congenital defects of lipoprotein lipase, triglycerides can not be derived from hydrolysis, resulting in massive accumulation of chylomicrons in blood. The disease often in adolescence, and within the 10-year-old was found, it was reported a week after birth was discovered. The main clinical features are:

, Skin changes, for the symptoms first appeared. In the elbow, back and arm that measles-yellow tumor. However, no eyelid and muscle-related macular tumor of yellow.
When serum triglyceride, 2000> Moore 22.6 cents / liter, the eyes may appear clothes hyperlipidemia retinopathy.
Hepatosplenomegaly, 2000, with the extent of its size and changes in blood triglyceride levels.
, Recurrent abdominal pain.

Its biological and chemical characteristics: Because chylomicrons increased, a plasma-like butter, at 4 ℃ refrigerator overnight, the upper deck was "cream"-like cover, lower clarify; significantly increased triglyceride and cholesterol is normal or only slightly higher Cholesterol / triglyceride ratio <0.2.

Ⅱ-Hyperlipoproteinemia

Also called familial hypercholesterolemia, is the dominant genetic diseases. Comparing this type Most of its main clinical features as:

, Of yellow, occurred in the Department of the eyelids, around the eyes showed a yellow spot, known as the yellow of the eyelids. May also occur in the muscles licensing, for example, in the elbow, with the muscle-like uplift plane was Qiu, known as the muscle-yellow tumor. In addition, visible to the festival-like yellow skin tumors, occurs in the skin vulnerable to oppression, such as knee, elbow and arm the side of God. Sometimes found in the fingers and palm crease Department.
, Early-onset atherosclerosis, about 60 percent of the cases in which a 40-year-old former angina, such as atherosclerosis performance.
Fat, corneal bow.

Ⅱ a type Ⅱ b and the clinical performance of similar, but its biochemical characteristics is different. Ⅱ a plasma-placed appearance fully clarified, increased cholesterol levels and triglyceride levels normal, cholesterol / triglyceride ratio> 1.5; Ⅱ b-placed after the appearance of the majority of plasma clarification, but there are also a few mild haze, cholesterol and glycerin 3 ester at the same time increased cholesterol / triglyceride ratio uncertain.

Ⅲ-Hyperlipoproteinemia

Less often for the family, is recessive genetic diseases. Often in patients 30 to 40-year-old when a flat of yellow (orange and yellow lipid calmly, and often occurred in the palm Division), to the section of the rash of yellow-and yellow skin tumors, and other early-onset coronary artery disease frequently Obesity and high blood uric acid, about 40% of the patients have abnormal glucose tolerance.

Biochemical characteristics: plasma unusual lipoprotein, the plasma is usually placed turbidity, and often a vague "cream" layer; cholesterol and triglyceride almost all increased cholesterol / triglyceride ratio ≥ 1.

Type IV Hyperlipoproteinemia

Clinical very Most of the 20-year-old Chang after the onset, for the family, a dominant genetic. Characterized by abnormally high levels of endogenous triglycerides, may be due to an increase of the liver, remove or weaken due to surrounding tissue.

Main clinical manifestations are: yellow skin tumors, skin nodules of yellow, yellow-like rash of yellow spots and eyelid tumor; retinal lipid hyperlipidemia; rapid progress of atherosclerosis; can be associated with pancreatitis, blood uric acid increased with the majority Abnormal glucose tolerance.

Biochemical characteristics: look at the plasma clarify or muddy, but no top-level chylomicrons; significantly increased triglyceride and cholesterol most normal or slightly higher cholesterol / triglyceride ratio uncertain.
V-Hyperlipoproteinemia
Of the type I and type IV of the mixture, which can have two types of features. The most commonly secondary to acute metabolic disorder, such as diabetes allocation of acidosis, pancreatitis and nephrotic syndrome, but also for the hereditary. Patients often after the age of onset was 20. Hepatosplenomegaly, abdominal pain associated with pancreatitis as the main clinical manifestations. Patients on diet and endogenous triglyceride intolerance, and often have abnormal glucose tolerance and hyperuricemia. At the upper plasma was "cream"-like, lower turbidity; significantly increased triglyceride and cholesterol increased, the cholesterol / triglyceride ratio> 1.15 and <0.6.

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