TY - JOUR
T1 - Cerebrotendinous Xanthomatosis
AU - Grundy, Scott M
PY - 1984/12/27
Y1 - 1984/12/27
N2 - Xanthomatosis typically occurs with hyperlipidemia, and tendon xanthomas are seen most commonly in familial hypercholesterolemia, a disorder associated with an increase in the plasma level of low-density lipoproteins. However, xanthomas are occasionally found in the absence of hyperlipidemia. For many years such lesions were unexplained, but recent investigations have shown that most cases of normolipidemic xanthomatosis are the result of various defects in the metabolism of sterols or lipoproteins. Although tendon and cutaneous xanthomas are not life-threatening, the defect causing them may predispose patients to atherosclerotic coronary heart disease. For this reason, normolipidemic patients with xanthomas should not be considered.
AB - Xanthomatosis typically occurs with hyperlipidemia, and tendon xanthomas are seen most commonly in familial hypercholesterolemia, a disorder associated with an increase in the plasma level of low-density lipoproteins. However, xanthomas are occasionally found in the absence of hyperlipidemia. For many years such lesions were unexplained, but recent investigations have shown that most cases of normolipidemic xanthomatosis are the result of various defects in the metabolism of sterols or lipoproteins. Although tendon and cutaneous xanthomas are not life-threatening, the defect causing them may predispose patients to atherosclerotic coronary heart disease. For this reason, normolipidemic patients with xanthomas should not be considered.
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U2 - 10.1056/NEJM198412273112609
DO - 10.1056/NEJM198412273112609
M3 - Editorial
C2 - 6504107
AN - SCOPUS:0021720599
SN - 0028-4793
VL - 311
SP - 1694
EP - 1695
JO - New England Journal of Medicine
JF - New England Journal of Medicine
IS - 26
ER -