Essential Xanthomatosis

Abstract

Rowland1 defined essential xanthomatosis as "a heredofamilial, constitutional disorder of metabolism in which a deposition of fat-like mixtures, particularly cholesterol and its ester, takes place in the tissues and blood. . . . The pathologic change appears in the form of granuloma-like accumulations arising in various parts of the body."Since an accumulation of excessive amounts of cholesterol is a dominant feature of this disease, restriction of cholesterol in the diet is a rational therapeutic procedure, and it has been tried in a few instances, with considerable success. Herrmann and Nathan 2 observed definite clinical improvement in two patients after treatment with a low cholesterol diet (Pflanzenkost), the description of which was not given. In one of them the cholesterol content of the blood serum decreased from 580 to 300 mg. per hundred cubic centimeters during four months on the diet; in the other the amount of serum cholesterol remained within normal limits throughout the period of study. Ga\l=a'\l3 reported on similar treatment in a case of severe xanthomatosis, in which there were rather dramatic results. With a low cholesterol diet the amount of serum cholesterol fell from 660 to 170 mg. per hundred cubic centimeters in nineteen days. Simultaneously there was considerable clinical improve¬ ment. Schoenheimer 4 described extensive metabolic studies in a case This investigation was assisted by a grant from the Josiah Macy Jr. Foundation.

Rapid, Direct Analysis of Cholesterol by Charge Labeling in Reactive Desorption Electrospray Ionization

“…The concentration of total cholesterol, consisting of free cholesterol and cholesterol esters, ranges from 1300 to 2500 μg/mL in healthy adult human serum, and the concentration of free cholesterol is in the range of ∼200 to 550 μg/mL 28-30. Reactive DESI-MS allows free cholesterol to be detected selectively in serum because cholesterol esters do not react with BA.…”

Rapid, Direct Analysis of Cholesterol by Charge Labeling in Reactive Desorption Electrospray Ionization

“…The concentration of total cholesterol, consisting of free cholesterol and cholesterol esters, ranges from 1300 to 2500 μg/mL in healthy adult human serum, and the concentration of free cholesterol is in the range of ∼200 to 550 μg/mL 28-30. Reactive DESI-MS allows free cholesterol to be detected selectively in serum because cholesterol esters do not react with BA.…”

Neonatal and Maternal Cholesterol Levels in Blood at Birth

“…Hodges et al (1943) showed that adult levels were reached by the age of six months. Sperry (1936) found a mean total cholesterol of 61 mg. per 100 ml. in cord blood, rising to 133 mg., in the first 25 days (using the Schoenheimer-Sperry method).…”

Plasma Lipids and Atherosclerosis

“…In health the plasma cholesterol level is not constant but may fluctuate, for reasons largely unexplained, by as much as 20% (Levere et al, 1958). In newborn infants the level is low but rises rapidly within the first ten days and thereafter gradually until puberty (Sperry, 1936). In men plasma cholesterol continues to rise slightly until about the middle thirties after which it remains fairly constant (Adlersberg et al, 1956).…”

Rapid, Direct Analysis of Cholesterol by Charge Labeling in Reactive Desorption Electrospray Ionization

“…The concentration of total cholesterol, consisting of free cholesterol and cholesterol esters, ranges from 1300 to 2500 μg/mL in healthy adult human serum, and the concentration of free cholesterol is in the range of ∼200 to 550 μg/mL 28-30. Reactive DESI-MS allows free cholesterol to be detected selectively in serum because cholesterol esters do not react with BA.…”

Neonatal and Maternal Cholesterol Levels in Blood at Birth

“…Hodges et al (1943) showed that adult levels were reached by the age of six months. Sperry (1936) found a mean total cholesterol of 61 mg. per 100 ml. in cord blood, rising to 133 mg., in the first 25 days (using the Schoenheimer-Sperry method).…”

Plasma Lipids and Atherosclerosis

“…In health the plasma cholesterol level is not constant but may fluctuate, for reasons largely unexplained, by as much as 20% (Levere et al, 1958). In newborn infants the level is low but rises rapidly within the first ten days and thereafter gradually until puberty (Sperry, 1936). In men plasma cholesterol continues to rise slightly until about the middle thirties after which it remains fairly constant (Adlersberg et al, 1956).…”

Rapid, Direct Analysis of Cholesterol by Charge Labeling in Reactive Desorption Electrospray Ionization

“…The concentration of total cholesterol, consisting of free cholesterol and cholesterol esters, ranges from 1300 to 2500 μg/mL in healthy adult human serum, and the concentration of free cholesterol is in the range of ∼200 to 550 μg/mL 28-30. Reactive DESI-MS allows free cholesterol to be detected selectively in serum because cholesterol esters do not react with BA.…”

Neonatal and Maternal Cholesterol Levels in Blood at Birth

“…Hodges et al (1943) showed that adult levels were reached by the age of six months. Sperry (1936) found a mean total cholesterol of 61 mg. per 100 ml. in cord blood, rising to 133 mg., in the first 25 days (using the Schoenheimer-Sperry method).…”

Plasma Lipids and Atherosclerosis

“…In health the plasma cholesterol level is not constant but may fluctuate, for reasons largely unexplained, by as much as 20% (Levere et al, 1958). In newborn infants the level is low but rises rapidly within the first ten days and thereafter gradually until puberty (Sperry, 1936). In men plasma cholesterol continues to rise slightly until about the middle thirties after which it remains fairly constant (Adlersberg et al, 1956).…”