Tangier disease

Orpha code: 31150OMIM code: 205400

Definition

A rare, genetic neurometabolic disease characterized biochemically by an almost complete absence of plasma high-density lipoproteins (HDL), and clinically by liver, spleen, lymph node and tonsil enlargement along with multifocal peripheral neuropathy, corneal, skin and nail and, occasionally, cardiovascular disease.

Disease data
Classification

Disease

Synonyms
ATP-binding cassette transporter A1 deficiency
Analfalipoproteinemia
Defekt kasety wiążącej trójfosforan adenozyny transportera A1
Niedobór kasety wiążącej ATP transportera A1
Analphalipoproteinemia
ORPHA code
31150
OMIM code
205400
ICD10 code
E78.6
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl