Tangier Disease

Summarized by Plex Health
Last Updated: 02 May 2022
effects of miglustat treatment in a patient affected by an atypical form of tangier disease. "effects of miglustat treatment in a patient affected by an atypical form of tangier disease.", by Sechi A, Dardis A, Zampieri S, Rabacchi C, Zanoni P, Calandra S, De Maglio G, Pizzolitto S, Maruotti V, Di Muzio A, Platt F, Bembi B. Fig3: Improvement of dorsal skin lesions after miglustat treatment. Images showing the improvement on miglustat treatment of the dorsal skin lesions resembling prurigo nodularis of the patient with Tangier Disease described in this study. Pictures...

Tangier disease is an acquired disorder defined by considerably reduced degrees of high-density lipoprotein in the blood. HDL transportations cholesterol and certain fats called phospholipids from the body's cells to the liver, where they are eliminated from the blood. HDL is typically described as "good cholesterol" since high levels of this substance reduce the possibilities of developing heart and capillary disease. They have a moderately increased risk of cardio disease due to the fact that people with Tangier disease have very low degrees of HDL. Additional signs and symptoms of Tangier disease consist of a slightly raised quantity of fat in the blood; disturbances in nerve function; and enlarged, orange-colored tonsils. Tangier disease is triggered by mutations in the ABCA1 genetics. A build-up of cholesterol can be poisonous to cells, leading to damaged cell function or cell death. HDL-cholesterol is frequently described as the good cholesterol as it can assist in the elimination of cholesterol out of the walls of arteries, especially the coronary arteries. Tangier disease may additionally be connected with an increased risk of heart disease, modest altitude in triglycerides, nerve disruptions, and hardly ever an opaqueness in the treatment of the eye. Symptoms of Tangier disease are variable and depend on which organs are entailed and the severity of those manifestations. Tangier disease is usually characterized by bigger orange- or yellow-colored tonsils. Cardiovascular disease has been reported in adults with Tangier disease.

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