Type I hyperlipidemia

Treatment Low fat diet. No drug therapy is effective for Type I hyperlipidemia. 

A patient with hereditary type I hyperlipidemia presents with elevated levels of chylomicrons and VLDL triglycerides in the blood. The main function of the chylomicrons in circulation is to do which of the following ... 

Genetic deficiency of LPL (type I hyperlipidemia) is associated with significant increases in the circulating levels of chylomicrons and VLDLs, and the absence of LPL from postheparin plasma. VLDLs are increased to a smaller extent than are chylomicrons, probably because intact VLDLs, unlike intact chylomicrons, can be taken up — albeit inefficiently — by the liver. Apo C2 deficiency has a phenotype almost indistinguishable from that of type I hyperlipidemia, demonstrating that the major, or only, function of this apolipoprotein lies... 

Excess lipid in the blood can result from primary genetic deficiencies, most of which are rare, or as a secondary consequence of another disease. Two primary hyperlipidemias, type I hypertriglyceridemia and type IIA hypercholesterolemia, are summarized in Table 1-15-2. 

The answer is a. (Hardman, pp 875-898.) In type I hyperlipoproteinemia, drugs that reduce levels of lipoproteins are not useful, but reduction of dietary sources of fat may help. Cholesterol levels are usually normal, but triglycerides are elevated. Maintenance of ideal body weight is recommended in all types of hyperlipidemia. Clofibrate effectively reduces the levels of VLDLs that are characteristic of types 111, IV, and V hyperlipoproteinemia administration of cholestyramine resin and lovastatin in conjunction with a low-cholesterol diet is regarded as effective therapy for type 11a, or primary, hyperbetalipoproteinemia, except in the homozygous familial form. 

Metabolism of plasma lipoproteins and related genetic diseases. CM=chylomicron, TG=triacylglycerol, VLDL=very low density lipoprotein, LDL=low density lipoprotein, IDL=intermediate density lipoprotein, apo Cll= apolipoprotein Cl I found in chylomicrons and VLDL. The Roman numerals in the white circles refer to specific genetic types of hyperlipidemias summarized on the facing page. 

The European Health Food Manufacturers Federation restricts over-the-counter supplements to 500 mg per day (Shrimpton, 1997). Where niacin is being used to treat clinically significant hyperlipidemia, and in trials for the prevention of type I diabetes mellitus, a tentative upper limit has been set at 3 g per day (Knip et al., 2000). 

Type II hyperlipoproteinemia has been divided inlutyrc I la and Ilb. Type I la is characterized by elevated levels LDL (j3-lipoproteins) and nomral levels of triglyceiiilr This subtype disorder is very common and may be ciusx by disturbed catabolism of LDL. Type Ilb differs ffomiyp lla. in that this hyperlipidemia has elevated VLDL Mdietary restrictions on eholcsteml and. saturated fuLs Tlii type of hyperlipoproteinemia responds to some fomi i ... 

A control and two patients with hyperlipidemia are studied after an overnight fast. Their plasma lipoprotein electrophoresis patterns are shown below, the control being in the middle lane. One of the patients has a pattern typical of type I lipoprotein lipase deficiency, and the other of type Ila familial hypercholesterolemia. Which of the bands observed in the electrophoretic gel patterns represents a lipoprotein fraction that is abnormally abundant after fasting and that is most enriched in triacylglycerides ... 

Fibrates are approved to treat hypertriglyceridemia and familial combined hyperlipidemia (Fredrickson s type lla, lib, IV, and V) (Table 30.2) in patients who are at risk of pancreatitis and have not responded to dietary adjustments or in patients who are at risk of CHD and have not responded to weight loss, dietary adjustments, and other pharmacological treatment. They can be used either alone or in combination with niacin, bile acid sequestrants, or FlMGRIs. If used with bile acid sequestrants, fibrates must be taken either 1 hour before or 4 to 6 hours after the sequestrant. As discussed previously and reemphasized below, caution should be used it fibrates are combined with HMGRIs. Fibrates are not effective in the treatment of hypertriglyceridemia associated solely to elevated chylomicron levels (Fredrickson s type I). 

Column G5000PW+G5000PW. Sample whole serum of hyperlipidemia of type I - V. 

Type I IB. Hypercholesterolemia with hyperglyceridemia or combined hyperlipidemia (increased LDL and VLDL). 

Primary or genetic lipoprotein disorders are classified into six categories for the phenotypic description of dyslipidemia. The types and corresponding lipoprotein elevations include the following I (chylomicrons), Ha (LDL), lib (LDL + very low density lipoprotein, or VLDL), III (intermediate-density lipoprotein), IV (VLDL), and V (VLDL + chylomicrons). Secondary forms of hyperlipidemia also exist, and several drug classes may elevate lipid levels... 

The referral form also includes check boxes for the physician to mark the appropriate ICD-9-CM codes. For diabetes education, he includes the common ICD-9 codes 250.00 (i.e., uncomplicated type 2 diabetes, controlled), 250.02 (i.e., uncomplicated type 2 diabetes, uncontrolled), and an Other field with room for the physician to add a more specific code (Buck and Lockyear, 2007). For the hyperlipidemia component, he has also included the common codes used to describe these conditions. Within the referral form is the statement of medical necessity and contact information for the physician and patients to reach the pharmacy to make appointments. Although Dr. Brouchard likes the form that he has created, he plans on revising the form based on his experience and input from the physicians office after a few months of use. He wants to make sure that the referral form is as easy to use as possible and that it contains most of the information he needs to submit claims successfully. 

Mortality secondary to cardiovascular disease is 10 to 30 times greater in dialysis patients than in the general population. In addition to traditional cardiac risk factors such as diabetes, hypertension, hyperlipidemia, tobacco use, and physical inactivity, patients with kidney disease have other unique risk factors. Among these are hyper-homocysteinemia, elevated levels of C-reactive protein, increased oxidant stress, and hemodynamic overload. Complications previously discussed such as anemia and metabolic disorders of CKD are also contributory. In particular, arterial vascular disease (i.e., atherosclerosis) and cardiomyopathy are the primary types of cardiovascular disorders present in the CKD population. These disorders lead to development of ischemic heart disease and its manifestations including myocardial infarction. As a predominant comorbidity, cardiovascular disorders and their sequela are the leading cause of death in the ESKD population. ... 

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