Hyper-lipoproteinemia



Hyper-lipoproteinemia





About one in five persons with elevated plasma lipid and lipoprotein levels has hyperlipoproteinemia, an inherited disorder marked by increased plasma concentrations of one or more lipoproteins. Hyperlipoproteinemia may also occur secondary to other conditions, such as diabetes, pancreatitis, hypothyroidism, and renal disease.

This disorder affects lipid transport in serum and produces varied clinical changes, from relatively mild symptoms that can be corrected by dietary
management to potentially fatal pancreatitis.

Hyperlipoproteinemia occurs as five distinct metabolic disorders. Types I and III are transmitted as autosomal recessive traits; types II, IV, and V are transmitted as autosomal dominant traits.


Causes

Each type of hyperlipoproteinemia has distinct causes and incidence. (See Types of hyperlipoproteinemia, page 414.)


Signs and symptoms

Clinical features of hyperlipoproteinemia vary according to the type of disorder:



  • Type I: recurrent attacks of severe abdominal pain similar to pancreatitis, usually preceded by fat intake; abdominal spasm, rigidity, or rebound tenderness; hepatosplenomegaly with liver or spleen tenderness; papular or eruptive xanthomas (pinkish yellow cutaneous deposits of fat) over pressure points and extensor surfaces; lipemia retinalis (reddish white retinal vessels); malaise; anorexia; and fever


  • Type II: tendinous xanthomas (firm masses) on the Achilles tendons and tendons of the hands and feet, tuberous xanthomas, xanthelasma, juvenile corneal arcus (opaque ring surrounding the corneal periphery), accelerated atherosclerosis and premature coronary artery disease (CAD), and recurrent polyarthritis and tenosynovitis


  • Type III: peripheral vascular disease manifested by claudication or tuberoeruptive xanthomas (soft, inflamed, pedunculated lesions) over the elbows and knees; palmar xanthomas on the hands, particularly fingertips; premature atherosclerosis


  • Type IV: predisposition to atherosclerosis and early CAD, exacerbated by excessive calorie intake, obesity, diabetes, and hypertension


  • Type V: abdominal pain (most common), pancreatitis, peripheral neuropathy, eruptive xanthomas on extensor surfaces of the arms and legs, lipemia retinalis, and hepatosplenomegaly.


Diagnosis

Diagnostic findings vary among the five types of hyperlipoproteinemia.

Jun 16, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Hyper-lipoproteinemia

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