health education

What is A-alphalipoprotein Neuropathy?

A-alphalipoprotein Neuropathy is a medical condition that is also called as Tangier Disease. This is called Tangier Disease because the first case was first reported in the Tangier Island off the coast of Virginia. This is first seen in a five-year-old child that has noticeable orange tonsils and a very low level of high density lipoprotein.

This is an autosomal recessive familial disorder which means that this may be acquired from the parents. The parents should have the mutated gene in each cell but both of the parents don’t have any signs or symptoms. This is a problem about the cholesterol metabolism in the body which is seen in the low total of the high density lipoprotein-cholesterol or the one that is called as the good cholesterol. This is referred as the good cholesterol because it will help a person to lessen the risk of getting heart and blood vessel disease. The HDL-cholesterol is not well transported to the cells for people who suffer from this disease. This disease also has an increased triglyceride in the levels of the blood. Tangier disease occurs when the body don’t have gene to make a certain protein which is called Apolipoprotein A1. This protein is needed to transports the fats from the tissue for important usage.

This is considered as a rare disorder because there are a statistics that only 100 cases are reported worldwide. Some of the disease are not really or was not reported. As the name implies this disease has a disturbances in nerve function (neuropathy). Other symptoms include enlarged orange-colored tonsils, atherosclerosis, enlarged spleen (splenomegaly), enlarged liver (hepatomegaly), and clouding of the clear covering in the eye (corneal clouding). In 50% cases that have this disease there are also problems occurring in the sensorimotor or distal sensory called polyneuropathy.