Quadricuspid aortic valve, parossistyc supraventricular tachycardia and double right kidney: an uncommon association

Fourteen years after surgery for replacement of the aortic valve, an interesting case previously unreported was brought to our attention. The female patient came to our OP Dept for a routine follow-up: she had been found at surgery to have a quadricuspid aortic valve. Operation dated October 1985. At a careful appraisal of the world-wide literature, we noticed that such an association of pathologies had never been reported before: quadricuspid aortic valve, paroxystical supraventricular tachycardia and right double kidney with double renal pelvis and double proximal ureter. Other anomalies associated with the quadricuspid valve, available in the literature are: patent duct, subvalvular fixed aortic stenosis, ventricular septal defect, hypoplastic anterior mitral leaflet and pulmonary stenosis. The pathologic findings at autopsy of this congenital malformation vary between 0.008% and 0.033%; attention must be turned to the fact that the incidence can be underestimated if not expressly searched for. The first quadricuspid aortic valve was described in 1862 by Balington in an autoptic report and sixty other cases have been reported since. In rare cases this pathology has been diagnosed at angiography. At follow up our patient remains in SR with rare transitory episodes of supraventricular tachycardia.     

Costs of high-dose salvage therapy and blood stem cell transplantation for resistant-relapsed malignant lymphomas in a southern Italian hospital

BACKGROUND AND OBJECTIVE: Analysis of costs of high technological procedures such as peripheral blood stem cell (PBSC) autotransplantation in lymphomas are generally finalized at disclosing whether the improvement of survival in a subset of patients is cost effective and whether the cost of the procedure could be reduced. With the aim of revealing a possibility of reducing costs with respect to conditions of safety, we present our experience with PBSC autotransplantation in a particularly poor prognosis subset of patients with lymphoma. DESIGN AND METHODS: The expenses are analyzed for groups of cost and main resources necessary at unitary cost are considered separately. Groups of cost include various phases of the PBSC autotransplantation such as preparative procedures, execution of myeloablative therapy, reinfusion of CD34 cells, supportive therapy after reinfusion until discharge of the patient, general support for the management of patient. All costs are calculated according to 1997 prices and salaries and reported in dollars. The analysis was conducted on 21 patients with lymphoma resistant to other therapies treated by myeloablative therapy and PBSC autotransplantation in an hematologic unit in an open ward; the assistance was provided by staff not exclusively dedicated to bone marrow transplant procedures, with some help from a family member. RESULTS: The PBSC procedure, including all phases, costs from $17,761.9 to $18,259.9 depending on the type of myeloablative therapy employed; the mean cost was $18,092.6. The preparative phase with mobilization of CD34 cells, cryopreservation and reinfusion costed $3,538.7 (19.6% of the total cost); a major cost of this phase was cryopreservation and CD34 manipulation ($857.1). The second phase with myeloablative therapy and reinfusion of CD34 cells had a mean cost of $2,785.9 (15.4% of the total cost); a major cost of this phase was the hospitalization ($1,119.8). The third phase of patient's support after treatment had a total cost of $7,649 (42.3% of the cost of the total procedure) with the major cost being due to hospitalization ($2,571) calculated on a mean of 15 days after the reinfusion of CD-34. The last group of costs, including management support, accounted for $4,119 (22.7%) with a major cost being amortization of the structure ($1,600). The general cost for nurse's assistance to the patient was $1,355.1 (7.5%). INTERPRETATION AND CONCLUSIONS: A procedure of PBSC autotransplantation in resistant lymphoma is affordable without the strict precautions generally given in intensive care units. This provides a substantial reduction of expenses because of the low number of specifically trained staff members and the generally low cost of the necessary supplies. Before, however, proposing PBSC autotransplantation in most patients with resistant lymphoma, an evaluation of whether costs could be further reduced and whether the procedure has a cost benefit impact is needed.     

Minimally invasive thoracotomy for myocardial revascularisation. A study of 32 cases

BACKGROUND: This study aimed to evaluate the advantages offered by a myocardial revascularisation technique proposed by Kolessov in 1967: minimally invasive thoracotomy for myocardial revascularisation. The aim was to assess its short and medium-term benefits. During the course of the 1980s, the problems linked to extracorporeal circulation (ECC) and the contraindications for traditional myocardial revascularisation led to a renewed popularity of "beating heart" revascularisation techniques. METHODS: The largest and most extensive series of patients in the literature undergoing myocardial revascularisation during left mini-thoracotomy was reported by Calafiore et al. In this study we report the series treated by our centre which, albeit involving only 32 cases, obtained good results in the short and medium term. This surgical procedure can only be applied to patients presenting monovasal obstructive coronary disease affecting the anterior interventricular artery (IVA) which must not present small calibre, calcified walls or a lateralised or intramyocardial anatomic position. RESULTS: Invasive and non-invasive instrumental tests carried out to control the distal anastomoses of the left internal mammary artery (IMA) did not reveal angulations and/or stenosing tractions before the anastomosis of IMA to a significant extent also for treatment, when isolating IMA, able to obtain the longest possible length and the best mobility. No infection of surgical wounds was reported postoperatively, as sometimes occurs in median longitudinal sternotomy. CONCLUSIONS: The 2-year follow-up showed the resolution of angina in 100% of the patients studied, as well as a satisfactory and rapid renewal of social relations.     

Alemtuzumab (Campath-1H) and CHOP chemotherapy as first-line treatment of peripheral T-cell lymphoma: results of a GITIL (Gruppo Italiano Terapie Innovative nei Linfomi) prospective multicenter trial

To evaluate in a prospective multicenter trial the feasibility and clinical efficacy of the combination of alemtuzumab (Campath-1H) with the cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) regimen (CHOP-C) as the primary treatment for patients with peripheral T-cell lymphoma (PTCL), between January 2003 and December 2005, 24 consecutive patients with PTCL entered the study and received 8 CHOP courses. Alemtuzumab was added at 30 mg subcutaneously at day -1 initially to the first 4 courses (4 patients), and then to all 8 courses (20 patients). Complete remission (CR) was achieved in 17 (71%) patients, 1 had partial remission, and 6 had stable/progressive disease. At a median follow-up of 16 months (range, 5-42 months), 14 patients were alive, 9 had died from progressive disease, and 1 had died from pneumonia at day +198 while in CR. So far, 13 are disease-free, with an overall median duration of response of 11 months. The most frequent side effects were grade 4 neutropenia and cytomegalovirus (CMV) reactivation. Major infections were Jacob-Creutzfeldt (J-C) virus reactivation, pulmonary invasive aspergillosis, Staphylococcus sepsis, and pneumonia. This study shows that CHOP-C: (1) is a feasible chemoimmunotherapy regimen; (2) is effective in PTCL with a high rate of CR achievement; and (3) is associated with mostly manageable infectious complications. This clinical trial was registered with the Osservatorio Nazionale sulla Sperimentazione cinica as ID no. 141202.     

Psychological Predictors of Outcome in Vertical Banded Gastroplasty: a 6 Months Prospective Pilot Study

BACKGROUND: At present, bariatric surgery is the most effective treatment for morbid obesity. Several factors appear to influence the patient's ability to adjust to the postoperative condition, but reliable predictors are lacking. The aim of this study was to assess whether psychological presurgical variables can predict outcome of vertical banded gastroplasty (VBG) in the short term. METHODS: 38 severely obese patients (6 men and 32 women) underwent laparoscopic VBG. All were assessed prospectively at TO (before surgery) and at T6 (6 months after surgery) with a semi-structured interview and a battery of psychological tests: State Trait Anger Expression Inventory (STAXI), Eating Disorder Inventory (EDI-2), Symptom Checklist 90 (SCL-90), Beck Depression Inventory (BDI), Binge Eating Scale (BES), Body Shape Questionnaire (BSQ), and (only at TO) the Temperament and Character Inventory (TCI). RESULTS: The comparison between TO and T6 found a significant weight loss and an improvement in several dimensions of EDI-2, BDI, and BSQ, together with an increase in the frequency of vomiting. Self-directedness (TCI) and Body Dissatisfaction (EDI-2) appear to be predictors of short-term outcome regarding weight loss. Self-transcendence (TCI) is associated with emerging side-effects. CONCLUSION: Although larger and longer studies are necessary to confirm these data, Self directedness and Self trascendence emerge as predictors of 6 months clinical and psychological outcome of VBG.     

Involvement of the nitric oxide/protein kinase G pathway in polychlorinated biphenyl-induced cell death in SH-SY 5Y neuroblastoma cells

Polychlorinated biphenyls (PCB) are persistent environmental contaminants whose chronic exposure can affect nervous system development and function. The cellular and molecular mechanisms underlying neuronal damage are not yet clear. In the present study, we investigated whether nitric oxide (NO) could be involved in aroclor 1254 (A1254; a PCB mixture)-induced cytotoxicity in SH-SY5Y human neuroblastoma cells. Prolonged exposure (24 hr) to A1254 (10-100 microg/ml) caused a dose-dependent reduction of cell viability that was attenuated in the presence of a calcium entry blocker, gadolinum (Gd(3+)) at 10 microM, a concentration able to block voltage-sensitive calcium channels. In addition, A1254 caused an increase of cytosolic calcium that was dependent on extracellular calcium, as measured by fura-2 videomicroscopy. A1254-induced calcium rise may stimulate NO production through an activation of neuronal NOS (nNOS). Indeed, the concomitant addition of the selective nNOS inhibitor N(omega)-propyl-L-arginine (NPLA) and A1254 prevented cell injury, suggesting that NO production plays a major role in A1254-evoked cell injury. Furthermore, the exposure (14 hr) to A1254 (30 microg/ml) produced an up-regulation of the expression of beta isoform of nNOS. This up-regulation was calcium dependent and was accompanied by an enhancement of NO production as demonstrated by an increase of nitrite formation. Moreover, A1254-induced cell injury was prevented when KT 5823, a selective cGMP/PKG inhibitor, was added concomitantly to 30 microg/ml A1254. These results suggest that PCB-induced cell death in neuroblastoma cells is mediated by an activation of the cGMP/PKG pathway triggered by NO production.     

Cellular properties of human erythrocytes preserved in saline-adenine-glucose-mannitol in the presence of L-carnitine

L-Carnitine (LC) in the preservation medium during storage of red blood cells (RBC) can improve the mean 24-hr percent recovery in vivo and increase RBC life-span after reinfusion. The purpose of the study was to investigate the differences in the biochemical properties of RBCs stored in the presence or absence of LC, and the cell-age related responses to storage conditions and to LC. RBC concentrates in saline-adenine-glucose-mannitol (SAG-M) were stored in the presence or absence of 5 mM LC at 4 degrees C for up to 8 weeks. RBC subpopulations of different densities were prepared by centrifugation on Stractan density gradient. Cells were sampled at 0, 3, 6, and 8 weeks, and hematological and cellular properties analyzed (MCV, MCHC, 4.1a/4.1b ratio as a cell age parameter, intracellular Na(+) and K(+)). After 6 weeks, MCV of RBC stored in the presence of LC was lower than that of controls (6 weeks MCV: controls 95.4 +/- 1.8 fl; LC 91.5 +/- 2.0 fl; n = 6; P < 0.005). This was due to swelling of control cells, and affected mainly older RBCs. LC appeared to reduce or retard cell swelling. Among the osmotically active substances whose changes during storage could contribute to cell swelling, only intracellular Na(+) and K(+) differed between stored control RBCs and LC-treated cells. LC reduces the swelling of older cells during storage at 4 degrees C in SAG-M, possibly by acting on the permeability of cell membrane to monovalent cations.     

Long-term follow-up study on the role of serum CA-125 as a prognostic factor in 221 newly diagnosed patients with Hodgkin's lymphoma

The present study has explored the possible value of sCA-125 as a prognostic factor in Hodgkin's lymphoma (HL). From August 1992 to June 2005 sCA-125 was measured at presentation and at the end of the treatments in 221 newly diagnosed adult patients with HL. In this study 90/221 (41%) patients showed a value greater than the standard upper limit of 35 U/ml, and 79/90 (88%) with an abnormal sCA-125 were at an advanced stage of the disease. Patients with elevated sCA-125 showed a significant reduction in complete remission (CR) rate (76%vs. 98%; p < 0.0001). Failure of normalization of sCA-125 during the treatment revealed that CR had not been reached. Furthermore, no traces of the glycoprotein sCA-125 were found in a series of paraffin-embedded samples coming from 15 patients of this study. In addition, soluble CA-125 was not detected in supernatants coming from four different Hodgkin-derived cell lines. The long-term follow-up revealed that the group of patients with sCA-125 lower than 35 U/ml, at diagnosis, had an estimated 92% event free survival (EFS) rate and a 94% overall survival (OS) rate, while the group of patients with sCA-125 greater than 35 U/ml had only a 60% EFS rate (log-rank 33.43, p < 0.0001) and a 70% OS rate (log-rank 23.52, p < 0.0001). Extranodal disease, severe lymphocytopenia and age proved to be the only standard factors that could represent a poor chance to survive. At multivariate analysis, high sCA-125, E sites >1 and age were the only independent factors producing poor outcomes in terms of CR, EFS and OS. Therefore, we believe that sCA-125 is a simple, reliable and reproducible tool, which may improve existing prognostic systems.     

Citalopram versus fluoxetine for the treatment of patients with bulimia nervosa: A single-blind randomized controlled trial

The most studied and most frequently used pharmacologic treatments in bulimia nervosa are the selective serotonin reuptake inhibitors (SSRIs), in particular, fluoxetine. Less is known about the efficacy of the other SSRIs. To compare fluoxetine with citalopram in the treatment of bulimic patients, 37 bulimic patients were randomized to receive fluoxetine (n=18) or citalopram (n=19); these patients were assessed with regard to clinical (ie, body mass index, pathologic behaviors), psychopathologic (Eating Disorder Inventory-2, Body Shape Questionnaire, Binge-Eating Scale, Beck Depression Inventory), personality (Temperament and Character Inventory), and clinical global impression measures. These measures were compared between the 2 treatment groups at baseline and at the end of treatment. Dropout rates were similar in the 2 groups. Both groups showed significant improvement in eating psychopathology, angry feelings, and clinical global impression. Patients in the fluoxetine group displayed a greater reduction in introjected anger, whereas those in the citalopram group displayed a greater reduction in depressive feelings. Both treatments showed some effect on outcome measures, but efficacy profiles did not overlap. Citalopram may be useful in depressed patients with bulimia, whereas fluoxetine is more specific for those with introjected anger and bulimia.     

Brain white-matter volume loss and glucose hypometabolism precede the clinical symptoms of Huntington's disease.

We studied the anatomic and functional changes in various brain areas during the course of Huntington's disease (HD) in a large cohort of mutation-positive individuals (n = 71) encompassing the complete range of disability (presymptomatic through stage V), and in healthy controls, for the purpose of defining both degenerative and dysfunctional brain changes in the same subjects. METHODS: We used an MRI and unsupervised multiparametric segmentation procedure based on a relaxometric approach to measure in vivo brain volumes in 71 subjects with presymptomatic to advanced HD. The same population was evaluated by 18F-FDG PET to assess variations in brain glucose metabolism. To predict age at onset in unaffected mutation carriers, we considered the estimated number of years from each subject's age to manifested HD symptoms, for a given expanded triplet number. RESULTS: Age-adjusted analyses confirmed that the 71 subjects as a group, as well as the subgroup of 24 unaffected presymptomatic subjects at risk for HD, had significantly smaller gray-matter and white-matter volumes and larger cerebrospinal fluid volumes than did controls (P < 0.0001). In the 24 presymptomatic subjects, we observed a significant inverse linear correlation between white-matter volume reduction and the estimated time to symptom onset (r2= 0.39; P = 0.0011). Both clinically unaffected subjects at risk for HD and symptomatic patients had significantly decreased glucose uptake in the cortex (frontal and temporal lobes) and striatum (caudate and putamen). HD subjects who were followed up longitudinally showed progressive white-matter reduction in the preclinical subjects (n = 10) and decreased glucose uptake in the cortex and striatum in affected (n = 21) and preclinical (n = 10) subjects. CONCLUSION: White-matter volume loss may precede gray-matter atrophy and may be associated with neuronal dysfunction in early disease.