1/390. Activation of monocytes in vivo causes intracellular accumulation of lipoprotein-derived lipids and marked hypocholesterolemia--a possible pathogenesis of necrobiotic xanthogranuloma.necrobiotic xanthogranuloma (NXG) is a rare histiocytic disease with generalized xanthomatosis. However, most cases with NXG are normolipidemic or hypolipidemic. The mechanism for the formation of xanthoma in NXG has not yet been clarified. We observed a case of NXG with severe hypocholesterolemia (total cholesterol: 1.69 mmol/l) and analyzed the function of monocytes in this case. Histological examinations by light microscopy revealed a large amount of lipid deposition in the patient's freshly isolated monocytes. The patient's monocytes showed a 3-fold increase in cholesteryl ester content and a 3-fold enhancement of acetyl low density lipoprotein (LDL) uptake compared with the control monocytes. However, no significant difference was noted in the expression of CD36 protein and the mRNA levels of scavenger receptor-class A (SR-A) between the monocytes of the patient and the control. The phagocytotic ability of the patient's monocytes was enhanced 1.5-fold compared with that of the control monocytes. These findings suggest that the activated monocytes may have degraded the modified LDL via a pathway other than CD36 or SR-A, and accumulated a great amount of lipids in vivo. In conclusion, the present study has demonstrated a possible pathogenesis of NXG that the activation of monocytes in vivo may contribute to the intracellular accumulation of lipoprotein-derived lipids leading to non-inherited xanthomatosis and the marked hypocholesterolemia.- - - - - - - - - - ranking = 1keywords = iv (Clic here for more details about this article) |
2/390. Verruciform xanthoma and concomitant lichen planus of the oral mucosa. A report of three cases.Verruciform xanthomas are benign muco-cutaneous lesions of unknown aetiology. They have a papillated surface and histologically they are characterised by the presence of foam cells in connective tissue papillae between elongated parakeratinised epithelial rete ridges. Three cases are reported in which oral mucosal verruciform xanthoma and oral mucosal lichen planus occurred concomitantly.- - - - - - - - - - ranking = 0.058823529411765keywords = iv (Clic here for more details about this article) |
3/390. Ultrasonic demonstration of xanthogranulomatous pyelonephritis.An ultrasound examination performed on a young woman with a long history of urinary tract infection demonstrated multiple subcapsular sonolucencies suggestive of multiple abscesses. The combination of the ultrasonic appearance, the clinical history, and a large nonfunctioning kidney with calculi on intravenous pyelography allowed us to suggest the diagnosis of xanthogranulomatous pyelonephritis. Ultrasound plays an even greater role in patients who are allergic to iodinated contrast material in whom intravenous pyelograms and arteriograms may be contraindicated.- - - - - - - - - - ranking = 0.058823529411765keywords = iv (Clic here for more details about this article) |
4/390. Xanthogranulomatous oophoritis--a case report.This is a case of a 25 year old unmarried women who presented with intermittent fever and lower abdominal pain. laparotomy revealed a large cystic left sided tuboovarian mass adherent to surrounding structures and containing foul smelling fluid. microscopy showed extensive replacement of the ovary by a chronic inflammatory exudate composed predominantly of foamy macrophages.- - - - - - - - - - ranking = 0.058823529411765keywords = iv (Clic here for more details about this article) |
5/390. Determination of extent and activity with radionuclide imaging in erdheim-chester disease.erdheim-chester disease usually involves the diaphyseal and metaphyseal regions of tubular bones and various visceral organs. A 56-year-old woman presented with the histologically confirmed diagnosis of erdheim-chester disease. A Tc-99m MDP bone scan revealed the entire extent of the skeletal disease and showed unusual involvement of the epiphyses and axial skeleton. In addition to MRI, a Ga-67 citrate scan including SPECT showed extensive soft-tissue infiltration of different organs. Both Tc-99m MDP and Ga-67 scintigraphy are useful tools in determining the distribution of this rare disease.- - - - - - - - - - ranking = 0.29411764705882keywords = iv (Clic here for more details about this article) |
6/390. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 0.11764705882353keywords = iv (Clic here for more details about this article) |
7/390. Characterization of a novel cellular defect in patients with phenotypic homozygous familial hypercholesterolemia.Familial hypercholesterolemia (FH) is characterized by a raised concentration of LDL in plasma that results in a significantly increased risk of premature atherosclerosis. In FH, impaired removal of LDL from the circulation results from inherited mutations in the LDL receptor gene or, more rarely, in the gene for apo B, the ligand for the LDL receptor. We have identified two unrelated clinically homozygous FH patients whose cells exhibit no measurable degradation of LDL in culture. Extensive analysis of dna and mRNA revealed no defect in the LDL receptor, and alleles of the LDL receptor or apo B genes do not cosegregate with hypercholesterolemia in these families. FACS((R)) analysis of binding and uptake of fluorescent LDL or anti-LDL receptor antibodies showed that LDL receptors are on the cell surface and bind LDL normally, but fail to be internalized, suggesting that some component of endocytosis through clathrin-coated pits is defective. Internalization of the transferrin receptor occurs normally, suggesting that the defective gene product may interact specifically with the LDL receptor internalization signal. Identification of the defective gene will aid genetic diagnosis of other hypercholesterolemic patients and elucidate the mechanism by which LDL receptors are internalized.- - - - - - - - - - ranking = 0.23529411764706keywords = iv (Clic here for more details about this article) |
8/390. genetic variation of lysosomal acid lipase.Lysosomal acid lipase (LAL) activity was measured using a new fluorometric assay in cultured skin fibroblasts from eight control subjects, two obligate heterozygotes for Wolman's disease (WD), one patient with WD, and one patient with cholesteryl ester storage disease (CESD). The LAL activities (mean /-SD) were 25.8 /-8.2, 13.2 /-0.1,1.1, and 1.4 nmol 4-methylumbelliferyl oleate (4-MUO) hydrolyzed/min/mg protein, respectively. These results compare favorably with those obtained using standard radioassays. The LAL activities of two cultures of amniotic fluid cells were 12.1 and 10.5. The LAL activity (mean /-SD) of peripheral leukocytes obtained from 34 laboratory volunteers (19 females, 15 males) was 4.0 /-1.8. Partially purified lymphocytes contained about 25 times as much LAL activity as did granulocytes. Cellogel electrophoresis, followed by staining with 4-MUO, showed at least two bands of LAL (A and B) from normal fibroblasts, amniotic fluid cells, and lymphocytes. Band A was absent from WD and CESD fibroblasts and was reduced in fibroblasts of the WD heterozygotes.- - - - - - - - - - ranking = 0.35294117647059keywords = iv (Clic here for more details about this article) |
9/390. poems syndrome with xanthomatous cells. Polyneuropathy Organomegaly Endocrinopathy M-protein skin changes.A diffuse xanthomatous infiltration was detected on a biopsy of the hyperpigmented patches in a 40-year-old man with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, skin changes) syndrome. Multiple biopsies of the hyperpigmented patches showed diffuse or perivascular foamy histiocytes in the upper and mid dermis, a sparse infiltrate of lymphocytes, fibrosis, hyperpigmentation of the basal layer, and capillary proliferation. The foamy cells were positive for periodic acid-Schiff (PAS) with diastase, oil red-O, and CD68. To our knowledge, this is the first case with foamy histiocytes without xanthomatous-appearing skin lesions in poems syndrome. It would be worth searching for xanthomatous histiocytes in the hyperpigmented skin of patients with poems syndrome.- - - - - - - - - - ranking = 0.11764705882353keywords = iv (Clic here for more details about this article) |
10/390. Xanthogranulomatous tubo-ovarian abscess resulting from chronic diverticulitis.We report a case of xanthogranulomatous tubo-ovarian abscess which was preoperatively suspected to be an adnexal neoplasm. With foreign body material found in the abscess wall and vegetable fiber in the tubal lumen, a previously treated chronic diverticulitis was the presumed cause. culture studies showed polymicrobial isolates which included escherichia coli, an enteric pathogen. After surgery, administration of antibiotics, and revision of delayed subcutaneous wound healing, the patient is reportedly well.- - - - - - - - - - ranking = 0.35294117647059keywords = iv (Clic here for more details about this article) |
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