Cardiac involvement in active uncomplicated acromegaly

Increased cardiovascular mortality is present in acromegaly. A late frequent complication of this disease is congestive heart failure. At present it is unclear whether the acromegalic heart disease is principally due to the growth hormone excess or to diabete mellitus, hypertension, and coronary artery disease often present in acromegalic patients. The aim of this study was an attempt to clarify this issue. Thus, we studied by echocardiography and radionuclide ventriculography at rest and during exercise 11 patients with active uncomplicated acromegaly in comparison with 12 normal subjects, comparable for age and sex distribution. The results have shown comparable heart rate and blood pressure in patients and control subjects. Moreover, increased left ventricular mass (117±19 vs 79±18 g/m²; p<0.001) with normal internal dimensions; normal ejection fraction at rest, but impaired during exercise (59±8 vs 74±7 %, p<0.001); impairment of diastolic function as documented by reduced peak filling rate (2.6±0.6 vs 3.17±0.3 EDV/s, p<0.01) and prolonged time to peak filling rate (209±78 vs 134±53 ms, p<0.01); and reduction of left ventricular end-systolic stress (49±7 vs 62±8×10 dynes/cm², p<0.001) were demonstrated in acromegalic patients. These results confirm those of the previous studies and better clarify the pathophysiological mechanisms at the base of acromegalic heart disease.Presented at the 36th Annual World Congress, International College of Angiology, New York, New York, July 1994     

Unrelated donor marrow transplantation for chronic myelogenous leukaemia

Between January 1989 and July 1995 the search for an unrelated donor (UD) was started for 379 consecutive Italian patients with Philadelphia positive (Ph⁺) chronic myelogenous leukaemia (CML). 89 (23%) were transplanted. The overall probability of transplant before and after December 1991 was 16% and 49%, respectively (P = 0.0001), and average interval between search activation and graft was 23 months and 13 months, respectively (P = 0.0001). Disease-free survival (DFS) following 60 consecutive transplants performed before February 1996 was 41.5% at 48 months and was 64% for patients grafted after January 1993. In univariate analysis, five variables had a favourable effect on DFS: year of bone marrow transplantation (BMT) after 1993 (P = 0.0002), HLA-DRB1 donor/recipient (D/R) match (P = 0.0006), total body irradiation (TBI) containing regimen (P = 0.0006), graft-versus-host disease (GvHD) prophylaxis including ‘early’ cyclosporin before the transplant, and a marrow cell dose > 3 × 10⁸/kg of recipient body weight (P = 0.04). Multivariate analysis confirmed that HLA identity (P = 0.006), TBI-containing regimen (P = 0.0001) and ‘early cyclosporin’ (P = 0.04) were associated with higher DFS. Transplant-related mortality (TRM) was 67% in patients grafted before January 1993 and 30% in patients grafted subsequently (P = 0.002). Multivariate analysis confirmed DRB1 identity (P = 0.03) and TBI-containing regimen (P = 0.0005) to be independent factors predictive of low TRM. This suggests that the outcome of patients transplanted from an HLA DRB1 matched donor, after a TBI-containing preparative regimen, is similar to results recently reported in patients transplanted from geno-identical siblings. These results indicate that the search should be initiated at diagnosis for patients < 45 years of age and UD BMT should be considered early in the disease course for those with an available DRB1-matched unrelated donor.     

A randomized trial of circumferential pulmonary vein ablation versus antiarrhythmic drug therapy in paroxysmal atrial fibrillation: the APAF Study.

OBJECTIVES: We compared ablation strategy with antiarrhythmic drug therapy (ADT) in patients with paroxysmal atrial fibrillation (PAF). BACKGROUND: Atrial fibrillation (AF) ablation strategy is superior to ADT in patients with an initial history of PAF, but its role in patients with a long history of AF as compared with ADT remains a challenge. METHODS: One hundred ninety-eight patients (age, 56 +/- 10 years) with PAF of 6 +/- 5 years' duration (mean AF episodes 3.4/month) who had failed ADT were randomized to AF ablation by circumferential pulmonary vein ablation (CPVA) or to the maximum tolerable doses of another ADT, which included flecainide, sotalol, and amiodarone. Crossover to CPVA was allowed after 3 months of ADT. RESULTS: By Kaplan-Meier analysis, 86% of patients in the CPVA group and 22% of those in the ADT group who did not require a second ADT were free from recurrent atrial tachyarrhythmias (AT) (p < 0.001); a repeat ablation was performed in 9% of patients in the CPVA group for recurrent AF (6%) or atrial tachycardia (3%). At 1 year, 93% and 35% of the CPVA and ADT groups, respectively, were AT-free. Ejection fraction, hypertension, and age independently predicted AF recurrences in the ADT group. Circumferential pulmonary vein ablation was associated with fewer cardiovascular hospitalizations (p < 0.01). One transient ischemic attack and 1 pericardial effusion occurred in the CPVA group; side effects of ADT were observed in 23 patients. CONCLUSIONS: Circumferential pulmonary vein ablation is more successful than ADT for prevention of PAF with few complications. Atrial fibrillation ablation warrants consideration in selected patients in whom ADT had already failed and maintenance of sinus rhythm is desired. (A Controlled Randomized Trial of CPVA Versus Antiarrhythmic Drug Therapy in for Paroxysmal AF: APAF/01; http://clinicaltrials.gov/ct/show; NCT00340314).     

Robotic magnetic navigation for atrial fibrillation ablation.

OBJECTIVES: We assessed feasibility of magnetic catheter guidance in patients with atrial fibrillation (AF) undergoing circumferential pulmonary vein ablation (CPVA). BACKGROUND: No data are available on feasibility of remote navigation for AF ablation. METHODS: Forty patients underwent CPVA for symptomatic AF using the NIOBE II remote magnetic system (Stereotaxis Inc., St. Louis, Missouri). Ablation was performed with a 4-mm tip, magnetic catheter (65 degrees C, maximum 50 W, 15 s). The catheter tip was guided by a uniform magnetic field (0.08-T), and a motor drive (Cardiodrive unit, Stereotaxis Inc.). Left atrium maps were created using an integrated CARTO RMT system (Stereotaxis Inc.). End point of ablation was voltage abatement >90% of bipolar electrogram amplitude. RESULTS: Remote ablation was successful in 38 of 40 patients without complications. The median mapping and ablation time was 152.5 min (range, 90 to 380 min) but was much longer in the first 12 patients (192.5 min vs. 148 min; p = 0.012). Median ablation time was 49.5 min (range, 17 to 154 min), but it was much shorter in the last 28 patients than in the first 12 patients (49 min vs. 70 min; p = 0.021). Patients receiving remote ablation had longer procedure times than control patients (p < 0.001) with similar mapping time but shorter ablation time on right-sided pulmonary veins. Many more mapping points regardless of their location were collected remotely (p < 0.001). CONCLUSIONS: Remote magnetic navigation for AF ablation is safe and feasible with a short learning curve. Although all procedures were performed by a highly experienced operator, remote AF ablation can be performed even by less experienced operators.     

Reconstitution of normal neutrophil function in chronic granulomatous disease by bone marrow transplantation

Allogeneic bone marrow transplantation was carried out on an 11-year-old boy with chronic granulomatous disease and severe chronic pulmonary insufficiency of restrictive type. After preparative regimen with busulfan (13 mg/kg) and cyclophosphamide (200 mg/kg), the patient received marrow cells from his HLA-identical and MLC-nonreactive sister. Durable sustained engraftment of donor hematopoietic and lymphoid populations occurred, as documented by analysis of genetic markers and complete reversal of the neutrophil function defect. No episode of infection occurred in the post-transplant course and, currently, 40 months after transplantation the patient is in excellent health and growing normally and showing an increasing improvement of his respiratory capacity. The successful outcome in this patient demonstrates that marrow transplantation is at present the only curative approach for this congenital disorder of neutrophil function.     

Active vaccination campaign to increase seasonal influenza vaccination coverage: a monocenter experience in a cohort of Italian patients with systemic autoimmune diseases

Clinical Rheumatology - Seasonal influenza is a frequent cause of hospitalization and mortality among patients with systemic autoimmune diseases. Despite this evidence, vaccination coverage is...     

Dermoscopy features of infantile perianal pyramidal protrusion

Infantile perianal pyramidal protrusion (IPPP) is a benign condition generally noted in childhood but may persist for several years. Dermoscopy may help to distinguish it from other conditions, particularly genital warts. We report six cases of IPPP and describe the dermoscopic features that will distinguish these lesions from verrucae.