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We have analysed a series of 96 patients with multiple myeloma (MM) to the purpose to verify the prognostic meaning of the beta2-microglobulin (B2M) and the validity of the staging systems proposed by Bataille and Jacobson who use the B2M respectively associated to the serum C-reactive protein (CRP) and to the serum albumin. In our series of patients the serum level of B2M at the diagnosis was directly correlated with the myelomatous cellular mass. We have found besides an inverse correlation between B2M and survival. Also the serum level of CRP was inversely correlated with the survival, while a direct correlation was found between the serum albumin and the survival: ipoalbuminemia was confirmed as a predictive index of short survival. Staging our patients with MM according to the proposal of Bataille, we identified also in our series three prognostic groups with different median survival: respectively 72, 40 and 18 months. Applying finally the staging proposal of Jacobson to our series, four stages were individualized, with median survival respectively of 70, 52, 26 and 10 months. We confirm the prognostic value of the B2M in MM.

A series of 24 consecutive patients affected by myelofibrosis with myeloid metaplasia was reviewed. The clinical-pathological features at onset were similar to those reported in literature; in particular in all the patients we observed splenomegaly and the typical leuko-erythroblastic picture in peripheral blood. The median survival of our series was of 57 months; the deaths were caused by severe anaemia and/or infection and/or haemorrhage; the blastic terminal transformations were rare. According to other authors, unfavourable prognostic factors in our patients were: male sex, advanced age, hepatomegaly, the presence of systemic signs, anaemia, leukocytosis, leukopenia, high number of circulating erythroblasts, thrombocytopenia, osteomyelosclerosis. We have confirmed the clinical value of three staging prognostic systems: the system proposed by Njoku based on haemoglobin level and reticulocytes number, the system proposed by Visani based on haemoglobin and circulating myeloid precursors number, the system proposed by Dupriez based on haemoglobin and white blood cells number.

The aim of the present research was to verify the prognostic value of some histologic bone marrow parameters in chronic myeloproliferative disorders. Diagnostic bone marrow biopsies were revised in 38 patients with chronic myeloid leukaemia, 30 with polycythemia vera, 14 with essential thrombocythemia and 16 with idiopathic myelofibrosis-myeloid metaplasia. An unfavourable clinical evolution was associated to "granulocytic" histotype in chronic myeloid leukaemia, to "erythrocytic/granulocytic and/or megacaryocytic" histotype in polycythemia vera, to "cluster" distribution of megacaryocytes in essential thrombocythemia, to classical myelofibrosis without osteomyelosclerosis in myelofibrosis-myeloid metaplasia. Bone marrow biopsy in chronic myeloproliferative disorders provides independent diagnostic and prognostic data; we support its routine execution in this group of hematologic malignancies.

The aim of this study was to present the experience of 2 Institutions (Department of Cardiology in Bergamo and Department of Pediatric Cardiology, Apuano Pediatric Hospital in Massa) in performing the balloon dilatation of the pulmonary valve. From January 1985 to August 1993 the balloon pulmonary valvuloplasty was performed in 220 patients, age ranging from 1 day to 60 years (mean age 5 years), 55 (25%) in the first year of life, 17 (8%) in the first week of life. The systolic gradient across the pulmonary valve fell from 78 +/- 31 mmHg (range 40-160) to 20 +/- 16 mmHg (range 0-70) immediately after the procedure (p < 0.001). In-hospital mortality happened in 3 cases (1.3%); 2 of them were neonates with critical pulmonary stenosis. In 7 cases (3%) the balloon valvuloplasty was repeated afterwards, because of inadequate relief of the gradient after the first dilatation. During the mean follow-up period of 4 years the other 210 patients (95%) did not need any further treatment and remain in stable clinical conditions. The balloon valvuloplasty gives excellent early and intermediate term results and should be considered the first choice procedure in the treatment of pulmonary stenosis at all ages.

Between 1972 and 1977, a permanent pacemaker has been implanted in 9 children for complete heart block, after repair of a congenital heart disease. Children's age ranged between 2 1/2 years and 13 years (mean age 8 years). The block had been caused by the operation in 8 children and was pre-existent in the last one. A permanent pacemaker was not implanted in another four children with postoperative complete heart block. In seven cases the pacemaker was implanted in the subclavear zone and connected to a transvenous electrode; in the remaining two cases the pacemaker was positioned subcutaneously in the abdomen and connected to an epicardial electrode. VVI-type pacemaker have always been used. Three children died after implantation because of chronic cardiac failure complicated, in one case, by surrhenalic insufficiency. In two cases the pacemaker was replaced because of battery exhaustion, as suggested by routine controls; in five cases, wire breakage occurred and it was replaced together with the pacemaker. In another case transvenous electrode displacement occurred; skin infection at pacemaker site occurred only once. No skin breakdown at pacemaker site has ever occurred, or any problem due to excessive pacemaker dimensions. Reappearance of normal synus rhythm was noted in one patient. No complication has been reported so far for the cases with postoperative complete heart block not treated with cardiac pacing.