[Analysis of amyloidosis in myeloma. Clinical, biological and prognostic study].
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Biomedical subjects
Publications and source records attributed to F Arnalich.
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A 35-year-old man with definite Behçet's disease developed acute mitral and aortic regurgitation. Valvular disease, we believed, was another manifestation of this disease. Cardiac involvement in Behçet's disease and the role of prednisone therapy are discussed.
One hundred pregnant women, not anemic and not receiving iron or vitamin supplements, were chosen at random among the three trimesters of pregnancy to determine the incidence of unexpected iron, plasma folate and/or cobalamin deficiency, and the significance of fluctuating levels of their respective binding proteins. Pregnant females had a nonsignificant fall in serum iron and a fourfold decline in serum ferritin in the 3rd trimester compared with 1st trimester values. There was a steady decrease in plasma cobalamin and cobalophilin levels in every trimester but no difference in transcobalamin II values. Unsaturated cobalamin (UBBC) and unsaturated transcobalamin binding capacity (UTCBC) were lower in the 1st trimester fully recovering afterwards. Plasma folate levels were not lower, although there was a steady reduction in total and unsaturated folate binding capacity throughout pregnancy.
Thirty-two patients with myelomatosis (3 with a solitary plasmocytoma and 29 with multiple myeloma) and neurological complications, from a group of 110 unselected patients with myelomatous disease were recorded. Spinal cord compression was the most frequent complication seen in 12 cases. In 9 subjects, it was the presenting symptom, and in 11 cases was caused by extradural plasmocytoma of the thoracic spine. Early decompressive laminectomy was curative in 3 cases, but in the rest, delay in diagnosis resulted in only partial recovery even with radiotherapy. Of the 6 patients with nerve root involvement, radiotherapy led to full recovery in 2 of 3 cases, and laminectomy resulted in complete improvement in 2 other cases. Peripheral polyneuropathy was seen in 8 cases, 2 related to systemic amyloidosis and 6 unassociated with amyloidosis. Both forms of neuropathy shared a slow progression, independent of the course of the myeloma. Acute encephalopathy, found in 6 cases, was due to hypercalcemia and/or serum hyperviscosity, and led to a significant shortening of mean survival. One patient had third cranial nerve palsy due to the overlying lesion of the skull base, and another suffered acute bacterial meningitis.
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We performed non-invasive assessment of cardiac size and function by clinical criteria, standard electrocardiography, chest X-ray, systolic time intervals and echo-cardiography in 27 patients with Paget's disease of bone and in 20 control subjects. The patients were divided into two groups on the basis of the degree of skeletal involvement (less than 15% in Group I and greater than 15% in Group II). No differences in heart size parameters of left ventricular performance were noted between Group I and controls. Cardiomegaly, increased left ventricular diastolic dimension and increased left ventricular mass indicative of ventricular hypertrophy were found in Group II compared to control subjects. In addition, patients with more extensive skeletal involvement had signs of depressed myocardial contractility, increased left ventricular volumes in diastole and systole and enlarged stroke volume, with no differences in echographic cardiac output compared to Group I and controls. The findings show an above normal incidence of cardiac enlargement and disturbed left ventricular performance in patients with Paget's disease and osseous lesions in greater than 15% of skeleton. The clinical implications of the altered cardiac function in patients with Paget's disease are briefly discussed.
The hospital records of one hundred and thirty-three patients with fever of unknown origin (FUO) were studied. An etiologic diagnosis had been made in 105 patients: 41 patients had an infection, 24 had a neoplasm, 17 had a connective tissue disease, and 23 had various diseases grouped under "miscellaneous." FUO was self-limiting in 25 of the remaining patients. Invasive procedures (arteriography, biopsy, laparoscopy, paparotomy) were necessary to establish a diagnosis in 67 patients, non-invasive tests (sero-immunologic, bacteriologic, conventional radiologic tests, clinical course and treatment response) were sufficient in 27 patients. In 11 patients, the cause of FUO was determined in necropsy. Thirty patients died of FUO: 6 patients with a neoplasm, 4 with a connective tissue disease, and 7 with diseases termed miscellaneous.
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