Table 4.

Treatment of Manifestations in Individuals with Tangier Disease

Manifestation/ConcernTreatmentConsiderations/Other
Enlarged hyperplastic
palatine tonsils
TonsillectomyConsider if tonsils cause airway obstruction or mass symptoms.
Hepatosplenomegaly Standard treatmentIncl standard precautions such as avoidance of high-impact sports or activities that could → splenic rupture. Abdominal masses may complicate splenectomy.
Peripheral
neuropathy
Clinically proven effective treatments are not yet available.
Transient bracing (e.g., w/wrist splint or ankle-foot orthosis) may be useful.Those w/residual foot drop may need to use an ankle-foot orthosis permanently.
Exercise as appropriate to person's capacityTo maintain balance & strength
Corneal opacification Corneal transplantationConsider if this interferes w/daily living.
Coronary artery
disease
Standard treatment, incl use of dietary & pharmacologic therapiesSee Prevention of Primary Manifestations.
Thrombocytopenia /
Hemolytic anemia
Standard treatment, if severe

From: Tangier Disease

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