Tangier disease (redirected from Analphalipoproteinemia)

Tan·gier disease (tn-jîr)

n.

An inheritable disorder of lipid metabolism characterized by almost complete absence from plasma of high-density lipoproteins, by storage of cholesterol esters in foam cells, and by enlargement of the liver, spleen, and lymph nodes. Also called familial high-density lipoprotein disease.

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Tangier disease Analphalipoproteinemia A rare AR condition caused by a deficiency in α-lipoprotein Clinical Deposits of cholesteryl esters in tonsils, other lymphoid tissues, lymphadenopathy, hepatosplenomegaly, peripheral neuropathy, intermittent diarrhea, corneal opacification Lab Absent HDL, ↓ cholesterol < 120 mg/dl, ↓ phospholipids, ↓ apoA-I and apoA-II, ↑ TGs Prognosis Usually benign, rarely CAD. See Triglyceride.