Cases reported "Hyperlipidemias"

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1/16. Avascular necrosis of multiple carpal bones. A case report.

    A case of a 66-year-old female patient with hyperlipaemia, corticosteroid osteoporosis and chronic obstructive lung disease with avascular necrosis of the proximal row of the carpus and hamate is described. No other sites of avascular bone necrosis were found. A proximal row carpectomy was performed with an excellent outcome.
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2/16. Thalamic and hypothalamic tumors of childhood: endocrine late effects.

    Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.
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3/16. Sea-blue histiocytes in mycosis fungoides.

    A patient with tumor-stage mycosis fungoides and lymph node involvement had sea-blue (ceroid-containing) histiocytes in her bone marrow. leukopenia, eosinophilia, and a mild type II beta-hyperlipoproteinemia were also features. Sea-blue histiocytes were demonstrated in the skin after the mycosis fungoides infiltrate had cleared with treatment. There is a possible interrelationship between the sea-blue histiocytes, the abnormal mycosis cells in the skin, and lipid metabolism.
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4/16. Histopathology and regression of retinal hard exudates in diabetic retinopathy after reduction of elevated serum lipid levels.

    PURPOSE: To describe a regression of retinal hard exudates in 2 patients with diabetic maculopathy, and to report immunohistologic findings reflecting lipid deposition in the retina. DESIGN: Two interventional case reports. methods: Two patients with exudative diabetic maculopathy were treated to normalize serum lipids. Histologic examination and immunohistochemistry of each patient's eyes were performed to assess the localization of apolipoprotein B and cholesteryl ester, both of which are principal components of low-density lipoprotein. RESULTS: Both patients showed a dramatic regression of retinal hard exudates after correction of dyslipidemia. Histopathology revealed diffuse lipids and cholesteryl ester in the retina. Apolipoprotein B and macrophages were colocalized in the perivascular space. CONCLUSIONS: The regression of hard exudates was most likely due to the aggressive lipid lowering in both patients. The novel histopathologic findings of hard exudate and diabetic maculopathy are similar to the pathologic changes observed in larger atherosclerotic lesions, except that they occur in the intraretinal perivascular space.
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ranking = 188.88068583714
keywords = macrophage
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5/16. Clinical, pathologic, and biochemical features of a cholesterol lipidosis accompanied by hyperlipidemia and xanthomas.

    We describe the unique clinical and histopathologic features of a child with biochemical and immunocytochemical features of Niemann-Pick disease type C (NPC). Clinically, she was found to have multiple xanthomas of the upper aerodigestive tract with dysphagia and expressive language delay, splenomegaly, bony infarcts, and type IIb hyperlipidemia. neurologic examination was otherwise normal. microscopy revealed foam cells in her bone marrow, liver, tongue, tonsils, glottis, and in normal-appearing peritonsillar mucosa. Lipid analysis of a liver biopsy specimen showed a small increase in phospholipids, a twofold increase in sphingomyelin, a fivefold increase in cholesterol, and a marked (25-fold) increase in bis(monoacylglycerol) phosphate. Lysosomal acid hydrolase activities in cultured skin fibroblasts were nondiagnostic. Biochemical and immunocytochemical studies of cultured fibroblasts demonstrated lysosomal accumulation of unesterified LDL-derived cholesterol as well as delayed induction of homeostatic responses to endogenous cholesterol consistent with a diagnosis of NPC. Based upon these observations, we speculate that this patient could have a new phenotypic expression of NPC or represents a new cholesterol lipidosis biochemically resembling NPC. The chance occurrence of two separate lipid disorders seems less likely.
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6/16. Apolipoprotein E deficiency with a depressed mRNA of normal size.

    We studied a 39-year-old man who had palmar xanthomas complicated with marked hyperlipidemia. His serum cholesterol and triglyceride were 2000 and 6300 mg/dl, respectively. serum apolipoprotein E (apo E) was undetectable in the patient by the methods of single radial immunodiffusion, sodium dodecyl sulfate-polyacrylamide gel electrophoresis, and radioimmunoassay. serum apo E concentrations of his father and sister were low. This evidence is consistent with a diagnosis of familial apo E deficiency. We studied the synthesis of apo E in cultures of peripheral blood monocyte macrophages (M-M cultures) obtained from the patient, and detected no secretion of apo E in the culture medium and no newly synthesized apo E in the cell lysate. There were only trace amounts of apo E mRNA of the M-M cultures and the size of the mRNA appeared the same as normal apo E mRNA, indicating a different mutation of the gene from that of the case reported by Zannis et al. (J. Biol. Chem., 260 (1985) 12891).
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ranking = 188.88068583714
keywords = macrophage
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7/16. plasma exchange for the management of cyclosporin A-induced hypertriglyceridemia.

    The authors report a case of hypertriglyceridemia complicating the course of a patient receiving cyclosporin A after bone marrow transplantation. When the patient was seen at the hemapheresis unit the clinical picture was characterized by headache, increasing visual and neurological disturbances. Plasma triglyceride level was 3215 mg/dl. Two plasma exchange sessions reduced triglycerides to 486 mg/dl and halted the disease progression. This may represent the first plasma exchange treatment of cyclosporin A-induced hypertriglyceridemia.
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8/16. Plane xanthoma and multiple myeloma with lipoprotein--paraprotein complexing.

    Clinicopathologic findings are reported of a woman with generalized plane xanthoma, multiple myeloma (IgG type K), and hyperlipemia with very high levels of serum cholesterol and triglyceride. Complexing of the serum lipoproteins and immunoglobulins had cryoglobulin properties and was separable by ultracentrifugation. Immunofluorescent studies of skin and bone marrow demonstrated deposits of IgG with low density lipoprotein apoprotein and IgG with beta-lipoprotein, respectively. Although immunosuppressive therapy resulted in return of serum IgG, lipid, and lipoprotein levels to normal, the patient died from the myeloma. serum lipoprotein-paraprotein complexes have been demonstrated in at least 20 other cases of cutaneous xanthomatosis and myeloma. This interaction may result in an autoimmune hyperlipemia.
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keywords = bone
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9/16. Pseudo-endophthalmitis caused by intravitreal lipid transudation in association with proliferative diabetic retinopathy and hyperlipidemia.

    A 20-year-old woman with a 13-year history of insulin-dependent diabetes mellitus presented with a history of malaise, fever, a non-healing ulcer of the great toe, and an insect bite incurred during recent travel to a foreign country. Milky white infiltrates accompanied by inflammatory cells located within close proximity to neovascular fronds in both vitreous cavities led to the suspicion of bilateral metastatic endophthalmitis. Studies conducted during hospitalization failed to show evidence of systemic blood born infection. A diagnostic vitrectomy in one eye failed to grow organisms, though lipid laden macrophages were identified by electron microscopy. The clinical appearance improved in both eyes during her hospitalization coincident with improved diabetic control. It was later concluded that the milky white infiltrates were associated with hyperlipidemia as a consequence of poorly controlled diabetes and a familial tendency toward hyperlipidemia.
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ranking = 188.88068583714
keywords = macrophage
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10/16. A case of congenital nephrotic syndrome associated with partial deficiency of lecithin cholesterol acyltransferase (LCAT) and hypothyroidism.

    The case of a 3 year-old boy with congenital nephrotic syndrome is reported, in whom decreased LCAT activity and hypothyroidism were also present. Renal biopsy confirmed a diffuse proliferative glomerulonephritis with a large number of foam cells in the capillary lumen of the glomerulus and the interstitium, which stained positively with acid phosphatase indicating the presence of macrophage with phagocyted lipid vacuole. The histological picture was similar to that of familial LCAT deficiency, but the reported case is one of secondary LCAT deficiency as a result of urinary loss of the enzyme. Replacement therapy with thyroid hormones resulted in improvement in growth and development.
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ranking = 188.88068583714
keywords = macrophage
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