The Experts below are selected from a list of 9 Experts worldwide ranked by ideXlab platform
Julie C. Fanburg-smith - One of the best experts on this subject based on the ideXlab platform.
-
Plexiform xanthomatous tumor: a report of 20 cases in 12 patients.
The American journal of surgical pathology, 2002Co-Authors: Michal Michal, Julie C. Fanburg-smithAbstract:We present 12 patients with 20 plexiform xanthomatous tumors (PXTs). All patients were male. Patient ages ranged from 20 to 59 years (mean 45 years). Clinical information was available for 11 (92%) patients. Only one patient with markedly elevated cholesterol levels had a family history of hypercholesterolemia; none of the others had a family or personal history of diabetes mellitus, hypercholesterolemia, or hyperlipoproteinemia. Three patients had markedly elevated serum triglyceride levels. The tumors were solitary in seven patients and multiple in five patients: three patients had two tumors, one presented had three, and one had four. PXTs were located on the knee (n = 8), elbow (n = 5), foot or hand (n = 3), and one each on the Achilles tendon, buttock, toe, and back. PXT was white to yellow in color and ranged in size from 0.7 to 5 cm (mean 2.7 cm). The tumors were located in the dermis and subcutis, had a distinctive plexiform arrangement, and were composed of various admixtures of uniform epithelioid and xanthomatous cells. All tumors in patients with solitary or multiple lesions had a plexiform architecture. Most of the nodules of the plexiform pattern of PXTs measured 0.5-2 mm. Rarely cholesterol clefts, necrosis, sparse inflammation, and multinucleated Touton giant cells were present. In two patients with multiple tumors, the PXT completely lacked the xanthoma cells and thus resembled an epithelioid lesion. Immunohistochemically, all lesions were KP1 (CD68) and vimentin positive and lysozyme, S-100 protein, HMB-45, epithelial membrane antigen, cytokeratins, factor VIIIrag, CD34, muscle-specific actin, alpha-smooth muscle actin, desmin (D33), desmin (Der-11), chromogranin, synaptophysin, neurofilament protein, and glial fibrillary acidic protein negative. Two patients with multiple lesions noted recurrences over 10 years. With the exception of one patient who died of an unknown cause, all 10 patients with follow-up were alive, some with residual disease, over a mean of 9 years (range 1-25 years). Some PXTs may represent a morphologic variant of tuberous or tendinous xanthoma, yet its exclusive occurrence in men, absence of personal/Familial Hyperlipemia/hypercholesterolemia in some patients, and relative paucity of inflammation and cholesterol clefts may make this a distinctive entity.
Charles F. Wilkinson - One of the best experts on this subject based on the ideXlab platform.
-
SPACED FAT FEEDING: A REGIME OF MANAGE- MENT FOR Familial Hyperlipemia *
2016Co-Authors: Charles F. WilkinsonAbstract:IT seems desirable to present this paper at this time in order to describe a regime of treatment for Familial Hyperlipemia. We feel that if we waited until our current investigation into the genetic and metabolic aspects of Familial Hyperlipemia was ready for publication, a very satisfactory and practical method of treating these patients would be withheld from the pro-fession longer than would be desirable. This regime has been presented orally a number of times from 1948 until the present. The use of it, however, is not widespread, and there does seem to be a need for such an effective and simple method of control. Familial Hyperlipemia was first described in 1932 by Buerger and Griitz1 and appeared to be a rare disease, since it was reported only when the complete syndrome was present. Only 40 cases have been reported to date in the literature.2 It is our present belief that this condition as reported represents the homozygous abnormal. We feel that the heterozygous ab-normal is far more common than is generally believed, and frequently over
Michal Michal - One of the best experts on this subject based on the ideXlab platform.
-
Plexiform xanthomatous tumor: a report of 20 cases in 12 patients.
The American journal of surgical pathology, 2002Co-Authors: Michal Michal, Julie C. Fanburg-smithAbstract:We present 12 patients with 20 plexiform xanthomatous tumors (PXTs). All patients were male. Patient ages ranged from 20 to 59 years (mean 45 years). Clinical information was available for 11 (92%) patients. Only one patient with markedly elevated cholesterol levels had a family history of hypercholesterolemia; none of the others had a family or personal history of diabetes mellitus, hypercholesterolemia, or hyperlipoproteinemia. Three patients had markedly elevated serum triglyceride levels. The tumors were solitary in seven patients and multiple in five patients: three patients had two tumors, one presented had three, and one had four. PXTs were located on the knee (n = 8), elbow (n = 5), foot or hand (n = 3), and one each on the Achilles tendon, buttock, toe, and back. PXT was white to yellow in color and ranged in size from 0.7 to 5 cm (mean 2.7 cm). The tumors were located in the dermis and subcutis, had a distinctive plexiform arrangement, and were composed of various admixtures of uniform epithelioid and xanthomatous cells. All tumors in patients with solitary or multiple lesions had a plexiform architecture. Most of the nodules of the plexiform pattern of PXTs measured 0.5-2 mm. Rarely cholesterol clefts, necrosis, sparse inflammation, and multinucleated Touton giant cells were present. In two patients with multiple tumors, the PXT completely lacked the xanthoma cells and thus resembled an epithelioid lesion. Immunohistochemically, all lesions were KP1 (CD68) and vimentin positive and lysozyme, S-100 protein, HMB-45, epithelial membrane antigen, cytokeratins, factor VIIIrag, CD34, muscle-specific actin, alpha-smooth muscle actin, desmin (D33), desmin (Der-11), chromogranin, synaptophysin, neurofilament protein, and glial fibrillary acidic protein negative. Two patients with multiple lesions noted recurrences over 10 years. With the exception of one patient who died of an unknown cause, all 10 patients with follow-up were alive, some with residual disease, over a mean of 9 years (range 1-25 years). Some PXTs may represent a morphologic variant of tuberous or tendinous xanthoma, yet its exclusive occurrence in men, absence of personal/Familial Hyperlipemia/hypercholesterolemia in some patients, and relative paucity of inflammation and cholesterol clefts may make this a distinctive entity.
Pérez Jiménez F - One of the best experts on this subject based on the ideXlab platform.
-
Possible relationship between overweightness and prevalence of Hyperlipemia in the children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia
Medicina clinica, 1995Co-Authors: S. Jansen, José Luis Zambrana, López Miranda J, Angeles Blanco, Julia Blanco, Espino A, Pérez Jiménez FAbstract:BACKGROUND Heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia are associated to a greater risk of coronary disease. Combined Familial Hyperlipemia has classically been indicated to manifest after the second decade in life. The aim of this study was to establish whether a systematic search would demonstrate the existence of combined Familial Hyperlipemia earlier and analyze whether the antropometric parameters related with the overweightedness accompany the appearance of the lipid disorders of this disease found at an early age. PATIENTS AND METHODS Different lipid parameters were studied in 89 subjects under the age of 18 who were children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia. Likewise the weight, height and waist/hip quotient were evaluated. Hyperlipemia was considered as the presence of cholesterol/LDL and/or triglicerides greater than the 95 percentile for age and sex. RESULTS Hyperlipemia was observed in 51% and 40% of the children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia, respectively. The body mass index and the waist/hip quotient of the latter children significantly correlated with the cholesterol-HDL values and the LDL/HDL quotient. CONCLUSIONS The patients with known combined Familial Hyperlipemia have a high percentage of children with Hyperlipemia during infancy. These data suggest a possible association between obesity in the appearance of Hyperlipemia in the children of patients with combined Familial Hyperlipemia at this age.
S. Jansen - One of the best experts on this subject based on the ideXlab platform.
-
Possible relationship between overweightness and prevalence of Hyperlipemia in the children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia
Medicina clinica, 1995Co-Authors: S. Jansen, José Luis Zambrana, López Miranda J, Angeles Blanco, Julia Blanco, Espino A, Pérez Jiménez FAbstract:BACKGROUND Heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia are associated to a greater risk of coronary disease. Combined Familial Hyperlipemia has classically been indicated to manifest after the second decade in life. The aim of this study was to establish whether a systematic search would demonstrate the existence of combined Familial Hyperlipemia earlier and analyze whether the antropometric parameters related with the overweightedness accompany the appearance of the lipid disorders of this disease found at an early age. PATIENTS AND METHODS Different lipid parameters were studied in 89 subjects under the age of 18 who were children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia. Likewise the weight, height and waist/hip quotient were evaluated. Hyperlipemia was considered as the presence of cholesterol/LDL and/or triglicerides greater than the 95 percentile for age and sex. RESULTS Hyperlipemia was observed in 51% and 40% of the children of patients with heterozygote Familial hypercholesterolemia and combined Familial Hyperlipemia, respectively. The body mass index and the waist/hip quotient of the latter children significantly correlated with the cholesterol-HDL values and the LDL/HDL quotient. CONCLUSIONS The patients with known combined Familial Hyperlipemia have a high percentage of children with Hyperlipemia during infancy. These data suggest a possible association between obesity in the appearance of Hyperlipemia in the children of patients with combined Familial Hyperlipemia at this age.