82 research outputs found
Sudden unexpected fatal encephalopathy in adults with OTC gene mutations-Clues for early diagnosis and timely treatment.
Acute myocarditis and left ventricular aneurysm as presentations of systemic lupus erythematosus.
A case of systemic lupus erythematosus (SLE) associated with fever, heart failure, and left ventricular (LV) aneurysm is reported. A diagnosis of SLE was suspected owing to the presence of active lymphocytic myocarditis and fibrinous endocarditis at LV endomyocardial biopsy and was confirmed by identification of 4 of the 11 criteria proposed by the American Rheumatism Association for the definition of SLE. A 2-month period of steroid therapy was followed by a remarkable recovery of LV function and progression of endomyocarditis to a healed phase at control LV biopsy. The LV aneurysm disappeared, likely because thrombosis occurred as a result of the hypercoagulable state accompanying the presence of anticardiolipin antibodies. This is the first reported case of LV aneurysm induced by SLE and is a rare clinicohistologic documentation of the effectiveness of steroid treatment on lupus endomyocarditis
[Amyloidosis in a mixed pattern. Sensitivity to colchicine and melphalan].
The Authors describe a case of systemic amyloidosis with hepatic renal, intestinal and presumably cardiac involvement in the course of multiple myeloma (light K chain type). Notwithstanding the presumptions of the pathogenesis of amyloidosis and the capacity of colchicine to inhibit amyloid synthesis in rats treated with casein, the form in question derived little benefit from the association of colchicine (0.5 mg t.i.d.) and Melphalan (cycles of 4 days every 6 weeks - 0.25 mg/Kg/die)
Giant cell myocarditis responding to immunosuppressive therapy
An unusual case of giant cell myocarditis presenting with cardiogenic shock that dramatically responded to conventional dose of steroids and azathioprine is reported. Cardiac recovery was rapid, complete (left ventricular ejection fraction rose to 55% from 10%), and was accompanied bq the disappearance of the inflammatory infiltrates including giant cells in the control endomyocardial biopsy. Maintenance of the recovery at 16 months of follow-up on a low dose of azathioprine suggests that giant cell myocarditis might be a heterogeneous disease having either a negative untreatable trend necessitating cardiac transplantation, or a curable substrate responding to immunosuppressive drugs
[Ectopic calcifications in adult hypophosphatasia].
The first case of hypophosphatasia in adults reported in Italy (20th in the world) is described with remarks on the epidemiology, pathogenesis and therapeutic possibilities. The formation of renal and cholecystic calculi after long-term, intensive vitamin D and Calcium therapy indicates the limited capacity of pyrophosphate (PPi), even in large doses, to prevent the precipitation in man of calcium and phosphorus and the subsequent formation of ectopic calcifications
Mycotic pollution prevention in the indoor environments // Il controllo del rischio da miceti negli ambienti confinati
The increasing time spent in indoor environment is related to an increase in respiratory and allergic diseases due, in part, to mycetes. Mycetes contamination (mainly by Cladosporium, Penicillium, Alternaria, Streptomyces) regards homes, offices, hospitals and other industrial buildings and is favoured using cellulose products in environments with relative humidity >70% and scarce ventilation. Literature suggests a standard of 500 cfu/m3 for homes, but guidelines for other indoor environments, hospitals included, are not available. The authors describe the commonest mycetes' reservoir and conclude offering some guidelines for the mycetes pollution prevention in the indoor environments
Modulating effect of omega-3 fatty acids on the proliferative pattern of human colorectal mucosa.
The use of biomarkers to assess cancer risk is based on the model of cancer as a multistep process; such markers are assumed to reflect an early stage in this process. A valid biomarker of risk must therefore show differential expression in normal and high-risk subjects, as well as quantitative correlation with the stage of carcinogenesis. It should also be easy to detect in small tissue specimens and responsive to modulation by chemopreventive agents. Cell proliferation is one of the most widely investigated markers of cancer risk. Case-control studies have shown that epithelial cell proliferation parameters, assessed in rectal mucosal biopsies by means of in vitro autoradiographic or immunohistochemical techniques, can discriminate between populations with normal and high risks for colon cancer. However, we recently reviewed rectal biopsies from 152 subjects (43 controls, 84 with adenomas, 25 resected for colon cancer) processed for in vitro 3H-thymidine autoradiography, and attempted to correlate various proliferative parameters with clinical and pathological variables by means of multiple regression analysis
REVERSIBLE DILATED CARDIOMYOPATHY DUE TO GROWTH-HORMONE DEFICIENCY
An unusual case of severe dilated cardiomyopathy is described in a woman with postpartum hypopituitary function, which was characterized by marked decrease of cardiac myofibrils (decreased ratio of myofibrillar volume:cell volume) and somatomedin C deficiency. The patient was treated with a daily intramuscular dose of 4 UI of recombinant human growth hormone for 3 months. Clinical and laboratory assessment and follow-up consisted of noninvasive (echocardiograms, Doppler study, and clinical laboratory tests) and invasive procedures (angiography and endomyocardial biopsy). Morphologic study included optical and electron microscopic examinations and morphometric analysis. The patient exhibited a relevant improvement of cardiac function after recombinant human growth hormone administration. The clinical improvement was confirmed by normalization of electrocardiographic voltages, increased myocardial mass, angiography, and optical and electron microscopic examination, which showed a dramatic recovery of myofibrillar content in myocardiocytes. Furthermore, withdrawal of growth hormone was followed by reduction of electrocardiographic voltages and myocardial mass as well as by impairment of left ventricular function. The authors suggest that there are forms of dilated cardiomyopathy that may benefit dramatically by growth hormone administration. These forms should be identified by growth hormone/somatomedin C axis deficiency and by decrease in myofibrillar content at endomyocardial biopsy
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