Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In the present report we describe a patient with multiple myeloma and long-standing paraproteinemia who developed xanthoma in the absence of an elevation in plasma cholesterol or triglyceride concentrations. Studies demonstrated that our patient's monoclonal IgG antibody interacted with apoprotein B-100. The LDL-antibody complex isolated from our patient did not affect the degradation of LDL by human fibroblasts, indicating that while IgG derived from our patient interacted with LDL it did not alter the metabolism of this lipoprotein by the LDL receptor pathway. Since the LDL receptor pathway is the major route of LDL metabolism, this probably explains why our patient was not hyperlipidemic. In contrast to an absence of effect on the LDL receptor, our patient's LDL-antibody complex stimulated cholesterol esterification within macrophages indicating the uptake and degradation of the LDL-antibody complex. The LDL-antibody complex inhibited the degradation of acetyl LDL by macrophages (scavenger pathway), demonstrating that our patient's LDL-antibody complex was recognized as a modified LDL. Moreover, mixing Ig from our patient with normal LDL also resulted in the normal LDL increasing the esterification of cholesterol by macrophages. One can hypothesize that our patient's monoclonal IgG-LDL complex interacted with the macrophage scavenger receptor, thereby resulting in the occurrence of xanthoma in the absence of hyperlipidemia.
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PMID:Cutaneous xanthoma in association with paraproteinemia in the absence of hyperlipidemia. 292 22

Osseous xanthomatosis and a pathologic fracture of the femoral neck associated with hyperlipoproteinemia occurred in a 48-year-old woman. Widely distributed skeletal lesions suggested a primary neoplasm such as malignant lymphoma or multiple myeloma; however, needle aspiration cytology of the fracture site, cutaneous manifestations, and abnormally high concentrations of lipoproteins established a diagnosis of intraosseous xanthomatosis associated with hyperlipidemia. Histologically, the excised femoral head showed a dense aggregate of lipid-laden macrophages and depletion of normal bone trabeculae. The hyperlipidemia is classified as Type IIb hyperlipoproteinemia.
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PMID:Osseous xanthomatosis and a pathologic fracture in a patient with hyperlipidemia. A case report. 318 May 83

Sixteen monoclonal antibodies (MAbs) directed against toxic shock syndrome toxin-1 (TSST-1) were generated by immunization of mice with purified TSST-1 and subsequent fusion of spleen cells with myeloma cells. Antibody-producing clones, identified by an enzyme-linked immunosorbent assay, were maintained as ascites tumors, and MAbs were purified by protein A chromatography. High-titered clones were further characterized and tested for the ability to neutralize several biological activities of TSST-1. The MAbs, which are of several immunoglobulin subtypes, reacted specifically with purified TSST-1 and TSST-1 present in Staphylococcus aureus culture supernatants. Three MAbs neutralized TSST-1-induced mitogenesis in a dose-dependent manner. Three of eight MAbs tested were able to neutralize induction by TSST-1 of interleukin-1 production by human monocytes. One neutralizing MAb, 8-5-7, was tested for the ability to protect rabbits from a constant infusion of TSST-1. Rabbits given the MAb had an attenuated clinical illness and were protected from the hypocalcemia, lipemia, and hepatic and renal insufficiency seen in control rabbits. Six of seven control rabbits died, compared with only one of seven rabbits treated with MAb 8-5-7. These experiments suggest that MAb 8-5-7 is directed against an antigenic determinant critical to the toxicity of TSST-1 and that the MAbs should be useful as probes in structure-function analyses of the TSST-1 molecule.
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PMID:Neutralization of toxic shock syndrome toxin-1 by monoclonal antibodies in vitro and in vivo. 325 1

There were 72 patients (19 with hepatic failure, 10 with fulminant hepatitis, eight with paraquat poisoning, eight with rheumatoid arthritis, five with myasthenia gravis, four with hyperlipidemia, four with systemic arteriosclerosis including brain infarction, three with pemphigus vulgaris, two with multiple myeloma, two with systemic lupus erythematosus, two cases non-specific Ig-G antibody, two cases medication with an anticancer drug, one with multiple sclerosis, one with Crohn's disease with amyloid kidney and one with chronic myeloblastic leukemia) treated by plasma exchange in the Kidney Center, Tokai University School of Medicine from Jan. 1983 to Dec. 1986. We performed plasma exchange using fresh frozen plasma in 40 cases and Lactate-Ringer's solution containing albumin (4.0-5.0%) in 20 cases as the replacement fluid. In 17 cases, we performed double filtration plasma exchange with the recycle system and no replacement fluid. Although PE therapy did not constitute a basic therapy for hyperlipidemia, pemphigus vulgaris, rheumatoid arthritis, myasthenia gravis, and systemic lupus erythematosus, it was effective in relieving severe clinical symptoms. At the present time, conventional plasma exchange does not improve the survival rate of patients with hepatic failure and fulminant hepatitis. Developments of a new artificial liver support apparatus and identity of many toxic substances in hepatic failure are necessary. No hypotension, hypovolemic shock or other significant complications were experienced.
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PMID:Clinical reports on plasma exchange in the Kidney Center, Tokai University School of Medicine. 344 83

Necrobiotic xanthogranuloma is a newly recognized cutaneous manifestation associated with paraproteinemia. Necrobiotic xanthogranuloma is distinctive clinically by yellowish-red nodular lesions, often ulcerated, with predilection for the periorbital area and ocular involvement. Histologically, it is characterized by the combination of inflammatory xanthogranulomas together with areas of necrobiosis. These skin lesions are associated with a dysglobulinemia and preceded the development of an IgG lambda myeloma in a previous patient. Hyperlipidemia may be found in some patients. A case of necrobiotic xanthogranuloma is reported, occurring in a 46-year-old man and associated with an IgG kappa myeloma.
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PMID:Necrobiotic xanthogranuloma with myeloma. A case report. 379 Nov 68

Clinicopathologic findings are reported of a woman with plane xanthomatosis, multiple myeloma (IgG kappa) and normolipemia. Plasma lipoproteins were bound to the monoclonal immunoglobulin. The complex was separated by ultracentrifugation, then the proteins were measured by radial immunodiffusion and laser immunonephelometry. Monoclonal IgG kappa interact with the low-density lipoproteins. The literature about the association between normolipemic or hyperlipemic xanthomatosis and myeloma was reviewed and the physiopathology of this association discussed. Several hypotheses are suggested but, at present, it is shown that lipoprotein-paraprotein complexing in some patients may be due to autoantibody activity of the myeloma protein against serum lipoprotein; immune complexes interfere with normal lipoprotein catabolism resulting in xanthomas and normolipemia or hyperlipemia.
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PMID:[Normolipemic plane xanthomas and IgGk myeloma with anti-lipoprotein activity. Apropos of a case. Review of the literature]. 392 16

Patients with myeloma disease showed in general very important abnormalities of circulating lipoproteins. Beside the presence of lipoprotein complexes with abnormal electrophoresis migration, we can observe either an hyperlipidemia associated with antilipoprotein auto-antibodies either, in contrary, an hypolipidemia. We studied 34 myeloma and one alpha heavy chain disease. In these subjects, all presenting clinical and biological myeloma tests but free of hepatic disease we evaluate after HDL separation their major lipid components: cholesterol and phospholipid. By electro immunodiffusion method we evaluate apolipoproteins A1 and B. We observe a decrease of Chol-HDL and PL-HDL in 51 p. cent of cases and a decrease of apo A1 in 80 p. cent of cases, this more marked in myeloma with gamma isotype. In contrary, apo B in all subjects studied remain normal. A correlation test with monoclonal immunoglobulin levels and its isotype is studied; the different parameters of monoclonal disease are discussed in relation with lipoprotein abnormalities.
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PMID:[Plasma lipoproteins and multiple myeloma. Variations of lipid constituents of HDL and apolipoproteins A1 and B]. 641 41

Human low density lipoproteins are metabolized by cultured human fibroblasts, through a specific metabolic pathway, which entails the regulation of intracellular cholesterol synthesis. When anti-lipoprotein IgA coming from patients with myeloma, mixed hyperlipidaemia and xanthomatosis were introduced into the system, we observed a decrease of the protein degradation of the LDL molecule, and a disappearance of the regulation of intracellular cholesterol synthesis. In the same system, an anti-lipoprotein IgA from a case of myeloma with mixed hyperlipidaemia, but without xanthomatosis, or control IgG and IgA were inactive and did not modify the LDL pathway.
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PMID:Interaction between fibroblasts, lipoproteins and three antilipoproteins IgA kappa. 677 Oct 77

Necrobiotic xanthogranuloma with paraproteinemia is characterized by multiple nodules or plaques that involve the periorbital area along with other parts of the body. A dysproteinemia due to an IgG paraprotein is associated with the condition; low serum complement, cryoglobulinemia, leukopenia, and hyperlipemia are also sometimes seen. Multiple myeloma is present in some cases. Two cases of necrobiotic xanthogranuloma with IgG monoclonal gammopathy were seen. Both initially had ocular symptoms and in the second case, the ocular manifestations have dominated the clinical picture. Histologically, these granulomas are characterized by collagen necrobiosis and by the presence of many foamy histiocytes and Touton giant cells. Because necrobiotic xanthogranuloma with monoclonal gammopathy frequently has prominent manifestations in the orbital region, may result in dysfunction of the eyelids or extraocular muscles, and is associated with potentially life-threatening systemic conditions, its recognition by the ophthalmologist is important.
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PMID:Necrobiotic xanthogranuloma of the eyelid. 684 54

We evaluated an accessory that enables the Du Pont aca discrete analyzer to measure Na+ and K+ by direct potentiometry (aca/ISE). Na+ and K+ gave linear responses in both the blood and urine modes, with no carryover. No interfering species were identified in the blood mode. Intra-assay and interassay precision for Na+ and K+ were more than adequate and analytical recoveries comparable to those with flame photometry were found for plasma. The Na+ and K+ values for plasma were comparable with those obtained by use of another direct potentiometric analyzer (the Orion SS-30), but slightly higher than flame-photometric values. Samples from patients with hyperlipemia and multiple myeloma gave clinically relevant values with the aca/ISE, but the flame photometric values were artefactually low. Whole blood could not be accurately analyzed. The aca/ISE is a precise, easy-to-use instrument, which gives values for plasma similar to those obtained with direct potentiometric analyzers. For analysis of urine, certain precautions are necessary.
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PMID:Evaluation of a direct potentiometric method for sodium and potassium used in the Du Pont aca. 685 Nov 1


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