Differences in individual and environmental factors between cutaneous melanoma and atypical Spitz tumour in children and adolescents.

It is not known if children and adolescents with atypical Spitz tumour and cutaneous melanoma differ in terms of etiological factors. The aim of this study was to explain differences in individual and environmental factors between cutaneous melanoma and atypical Spitz tumour. In the context of a study on melanocytic lesions, all subjects aged under 20 years with either cutaneous melanoma or atypical Spitz tumour were included (N = 105). Information on socio-demographic characteristics, individual and environmental factors were collected for both mother and child. The Fisher's exact test and the Mann-Whitney U test were used for categorical variables and continuous variables respectively. A multivariate logistic model was used to explain differences in outcome by differences in explanatory variables. In comparison to patients with cutaneous melanoma, patients with atypical Spitz tumour had less freckles (p = 0.020), lower number of common nevi (p = 0.002), and lower body mass index (p = 0.001) and experienced less sunburns episodes (p = 0.008). However, in the multivariate analysis, only a low number of common nevi remained statistically significant. Children and adolescents with cutaneous melanoma have a high number of nevi in comparison to the same-age group with atypical Spitz tumour.Conclusion: The results of this study suggest that the only difference in individual and environmental risk factors between cutaneous melanoma and atypical Spitz tumour in children and adolescents is the number of nevi. What is Known: •Atypical Spitz tumours and cutaneous melanoma in children and adolescents are clinically similar, but compared with melanoma, they have a good overall prognosis. •Risk factors for cutaneous melanoma in children and adolescents are similar to the ones found in adults in the literature What is New: •Differences in individual and environmental risk factors for atypical Spitz tumour in children and adolescents are described for the first time in this study. •Individual and environmental factors for atypical Spitz tumour in children and adolescents are comparable to cutaneous melanoma, except for the presence of low number of nevi.

Quality of life of hemodialysis patients in Central and Southern Italy: cross-sectional comparison between Hemodiafiltration with endogenous reinfusion (HFR) and Bicarbonate Hemodialysis.

INTRODUCTION: in hemodialysis (HD) patients, poor health-related quality of life (HR-QoL) is prevalent and associated with adverse outcomes. HR-QoL is strictly linked to nutritional status of HD patients. Hemodiafiltration with endogenous reinfusion (HFR) is an alternative dialysis technique that combines diffusion, convection and absorption. It reduces burden of inflammation and malnutrition and this effect may cause beneficial effect on HR-QoL. However no data on HR-QoL in HFR is currently available. METHODS: we designed a cross-sectional multicentre study in order to compare the HR-QoL in patients treated with HFR versus Bicarbonate HD (BHD). We enrolled adult patients HFR treated for at least 6 months, with life expectancy greater than six months and without overt cognitive deficit. The recruited patients in HFR were matched for age, gender, dialytic vintage and performance in activities of daily living (Barthel index) with BHD treated patients. SF-36 questionnaire for the assessment of HR-QoL was administered. RESULTS: one hundred fourteen patients (57 HFR vs 57 BHD) were enrolled (age 65.413.5 years; dialysis vintage 5.4 (3.3-10.3) years; 53% males) from 18 dialysis non-profit centres in central and southern Italy. As result of matching, no difference in age, gender, dialytic age and Barthel index was found between HFR and BHD patients. In HFR patients we observed better values of physical component score (PCS) of SF-36 than BHD patients (P=0.048), whereas no significant difference emerged in the mental component score (P=0.698). In particular HFR patients were associated with higher Physical Functioning (P=0.045) and Role Physical (P=0.027). CONCLUSIONS: HFR is associated with better physical component of HR-QoL than BHD, independently of age, gender, dialysis vintage and invalidity score. Whether these findings translate into a survival benefit must be investigated by longitudinal studies.

The effect of time to sentinel lymph node biopsy on cutaneous melanoma survival.

BACKGROUND: Whether timing of sentinel lymph node biopsy (SLNB) in cutaneous melanoma improves survival is not yet clear. The aim of this study was to investigate if the timing of SLNB influences long-term melanoma mortality. METHODS: A 10-year retrospective cohort study was conducted on 748 cutaneous melanoma patients who underwent excision of the SLN. Hazard ratios and 95% confidence intervals were estimated from Cox proportional hazards models. RESULTS: After adjusting for sex, age, Breslow thickness, mitotic rate, ulceration, and histologic type, patients who underwent early SLNB (≤30 days) and resulted positive on final pathology had a 3 times decreased risk of melanoma mortality (hazard ratio = .29; 95%confidence interval = .11 to .77) in comparison to patients who underwent delayed SLNB (≥31 days) and resulted positive on final pathology. CONCLUSIONS: Our findings suggest that early SLNB (≤30 days) improves melanoma survival.

Efforts in blood safety: Integrated approach for serological diagnosis of syphilis.

Recent efforts in transfusion medicine are focused on improving blood safety as well as establishing effective and efficient diagnostic algorithms for donor screening. To date, syphilis is a transfusion-transmitted infection re-emerged in many countries as a public health threat especially among populations at specific risk. This task requires new diagnostic tools and hemovigilance programs. The current diagnostic methodologies are debated, since presenting limitations and unresolved issues with special regard to the clinical interpretation of serological patterns, especially in asymptomatic patients and in blood donors. Furthermore, the switch from the traditional to alternative diagnostic algorithms underlines the lack of a gold standard, which has not been supported by shared guidelines. Besides, a lot of ongoing clinical trials on the performance of diagnostic assays, on the serological response associated with different pharmacological treatments, as well as on the prevention programs are currently under investigation. Here, we review the recent literature about the diagnosis of syphilis especially for low-risk populations proposing the adoption of an algorithm for blood donor screening that should satisfy the need of increasing safety for transfusion-transmitted infections in the modern blood transfusion centers.

Impact of epigenetic mechanisms on therapeutic approaches of hemoglobinopathies.

Hemoglobinopathies are inherited disorders characterized by anomalies of structure, function or production of globin chains. From conception to adulthood, the different expressions over time of the various globin chains depend on the activation/deactivation of different globin genes through methylation and chromatin remodeling processes. The most significant clinical disorders are ß-thalassemia and sickle cell disease. The clinical management of these disorders engages regular blood transfusions. Another therapy is represented by allogeneic hematopoietic cells transplantation. There are several studies based on the innovative therapeutic strategies that involve some epigenetic mechanisms focused on the reactivation of γ-globin gene expression. The induction of fetal hemoglobin expression in adulthood is an effective therapy for these disorders. Particularly interesting are the recent data on miRNAs showing the interaction of these molecules with different transcription factors such as MYB, KLF, BCL11A and SOX6. The aim of this review was to report an update on the dynamic epigenetic modifications as targets for therapy in hemoglobinopathies.

Current clinical applications of extracorporeal photochemotherapy.

From the 1980s, extracorporeal photochemotherapy (ECP) has been shown to be effective in a variety of pathological conditions such as erythrodermic cutaneous T-cell lymphoma, autoimmune diseases, solid organ allograft rejection and graft versus host disease. To date, ECP represents a non-aggressive immune modulatory therapy with a low spectrum of toxicity. ECP reduces the alloreactivity promoting the immune tolerance to self. At the same time, it allows the maintenance of immune response integrity of both naive and memory T-cells. However, the molecular mechanisms of action by which ECP exerts its therapeutic activity are still under investigation. Here, we review molecular mechanisms and clinical applications involved in ECP. The outcome of ECP is difficult due to the lack of reliable predictor factors for the selection of patients and their adequate follow-up. Since the study of such predictors is important, we also describe some biological markers that enable us to investigate the clinical management of the patients considered for the use of ECP.

Screening tests for hepatitis B virus, hepatitis C virus, and human immunodeficiency virus in blood donors: evaluation of two chemiluminescent immunoassay systems.

Automated chemiluminescent immunoassays (CLIAs) are useful for the detection of hepatitis B virus (HBV), hepatitis C virus (HCV), and human immunodeficiency virus 1/2 antigen/antibodies (HIV 1/2 Ag/Ab) in blood donor screening. Eight hundred and forty serum samples were tested for hepatitis B surface antigen (HBsAg), HCV antibodies (anti-HCV), and HIV1/2 Ag/Ab in parallel using 2 different CLIAs (Abbott Architect i2000SR and Roche Cobas e411). The concordance between the 2 systems was high (Cohen's kappa 0.97 for HBsAg, 0.77 for anti-HCV, 0.92 for HIV1/2 Ag/Ab) and the specificity and the positive predictive value were comparable. Among the 12 discrepant results, 11 were false-positive and 1 (reactive by Architect) was true-positive for anti-HCV. Positivity for HBV DNA, HCV RNA, and HIV RNA was recorded in 90.9%, 38.9%, and 100% of true-positive samples, respectively. This study represents the first stringent comparison between Architect i2000SR and Cobas e411 in blood donors. We observed a good correlation and high agreement among HBV, HCV, and HIV with the 2 automated systems.

Effect of hemodiafiltration with endogenous reinfusion on overt idiopathic chronic inflammation in maintenance hemodialysis patients: a multicenter longitudinal study.

Chronic inflammation is widely diffuse in maintenance hemodialysis (MHD) patients and is associated with poor survival. Hemodiafiltration with endogenous reinfusion (HFR) is a dialysis technique, highly biocompatible, able to adsorb proinflammatory cytokines and to decrease amino acids and antioxidants loss. These features could be helpful in MHD patients affected by idiopathic chronic inflammation, but this issue remains to be elucidated. We performed a multicenter longitudinal study to assess the effect of the switching from bicarbonate HD to HFR in patients with serum C-reactive Protein (CRP) > 5 mg/L coupled with albumin <4.0 g/dL in the last 6 months. We enrolled 24/176 (14%) patients, of which 20 patients were assessed at 4 months and 18 completed the study. We excluded 11 patients with evident causes of inflammation. At baseline, serum levels of CRP (18.7[7.0-39.4] mg/L) and albumin (3.5[3.3-3.7] g/dL) were significantly correlated (r = -0.49; P = 0.028). The effect on CRP and albumin was almost evident in the first 4 months and remained stable until to eighth month. A strict correlation (R = -0.49; 0.040) between percentage change of CRP (-35%) and albumin (+14%) after 8 months of HFR. These effects were associated with the reduction of IL-6, IL-1ß, and TNF-α and the increment of pre-albumin and leptin, whereas the serum levels of Branched Chain Amino Acid (BCAA) remained unchanged. In MHD patients affected by idiopathic chronic inflammation the switching from BHD to HFR is associated with improvement of inflammation. Whether these favorable effects may modify the outcomes of these high-risk patients needs to be confirmed by studies ad hoc.

Incidence and clinical predictors of Kaposi's sarcoma among 1721 Italian solid organ transplant recipients: a multicenter study.

Kaposi's sarcoma (KS) can be a complication of solid organ transplantation, but with an important incidence rate variability in different geographical areas. Here we analyzed the incidence rate, timing and clinical correlates of KS, in a cohort of Italian solid organ transplant recipients from four distinct transplantation centers. A total of 1721 renal, heart and liver transplant recipients were recruited between 1997 and 2004. KS was diagnosed in 40 patients, after a median follow up of 1 year (range 0.8-5.1). Visceral involvement was detected in 7/40 patients. Incidence rate of KS in the whole population was 2.3 cases per 1000 individuals per year. The standardized incidence rate (SIR) for KS in renal transplant recipients was 149.9 (95% CI 103.0-212.0), with the excess risk greater among women (SIR 316.0) than among men (SIR 133.6). In a Cox proportional hazard regression model, age at transplantation equal or older than 30 years and only combined immunosuppressive therapy with mycophenolate mofetil + cyclosporine + prednisolone were independently associated with KS. Italian organ transplant recipients have an increased risk (about 100 times greater) for KS compared to the general population, especially during the first two years after transplantation. Age older than 30 years at transplantation and a more aggressive immunosuppressive regimen were both independent risk factors for the disease.

Factors increasing the risk for stone formation in adult patients with cystic fibrosis.

BACKGROUND: Patients with cystic fibrosis (CF) are at high risk of nephrolithiasis (NL), but controversy still exists in terms of causes, including low urine output, hypercalciuria, hyperoxaluria, hyperuricosuria and hypocitraturia. Moreover, heterozygotes (H-CF), which may exhibit altered renal concentrating and diluting ability, have never studied so far. We, therefore, evaluated the metabolic and physicochemical data of adult CF and H-CF patients, comparing them to controls (C). METHODS: Twenty-nine CF patients (16 females, aged 28.4 +/- 7.1 years), 20 H-CF (12 females, aged 58.6 +/- 6.3 years) and 30 C (19 females, aged 39.1 +/- 11.5 years) underwent kidney ultrasound and metabolic evaluation to assess stone risk profile. RESULTS: There was a 21% prevalence of NL in CF vs 15% in H-CF. The CF group had elevated uric acid, but no other serological differences compared with the H-CF and C group. Conversely, the citrate and oxalate content in the urine differed significantly, being lower and higher, respectively. These changes held after correction for urine creatinine. Consequently, urine specimens were more supersaturated with calcium oxalate, despite exhibiting no differences for other relevant constituents. Uric acid increased only after normalization for the body weight and urine creatinine. Lower urine volume and more acidic pH produced mild supersaturation with uric acid in samples from CF, while urine from both H-CF and C remained undersaturated. H-CF had only minor increases in both urine oxalate and calcium oxalate supersaturation. CONCLUSIONS: This study confirms a high prevalence of kidney stones among CF patients associated with supersaturated urine. Their longer survival justifies diets and/or medications aimed at reducing the risk of forming stones.

A post-partum hemolytic-uremic-like-syndrome in a patient with pre-eclampsia: description of a clinical case.

HEMOLYTIC UREMIC SYNDROME POST-PARTUM: We describe a case of a 37-year-old woman admitted for severe renal failure to our hospital immediately after the delivery by caesarean section of twins. She had anuria, anemia, and moderate thrombocytopenia. A diagnosis of hemolytic-uremic syndrome was made. Plasma exchange was started, substitution was performed with fresh frozen plasma and eight consecutive plasmapheresis sessions were given. She received hydrocortisone and ACE inhibitors. After about fifteen days from the beginning of the illness, signs of active haemolysis disappeared and renal function was partially recovered. A genetic study demonstrated the absence of HF1 and MCP mutations but a polymorphic variant of the HF1 gene (C-257T promoter region). This polymorphism is strongly associated with non-diarrhoea-HUS (D-HUS). Post-partum HUS is quite a rare syndrome and has a poor outcome; however prompt diagnosis and efficacious therapy could save lives without clinical consequences. The excellent outcome of this patient seems to corroborate this concept.