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Background Health behaviours in adolescence have a high impact on both current and future health. The literature claims a focus on social determinants and shows that these behaviours are associated. The concept of 'health lifestyles' should be considered to account for co-occurring health behaviours and determinants interactions. The aim of this study is to increase our understanding of adolescents' health lifestyles and to (i) cluster adolescents according to a comprehensive number of health behaviours and (ii) describe these groups according to sociodemographic characteristics, perceptions of life contexts (family, school, peers or neighbourhood) and perceived physical and psychosocial health conditions. Methods In Italy, 906 15-year-old students participated in the cross-national Health Behaviour of School-aged Children study. Clusters were identified by applying the KAMILA clustering method and compared using analysis of variance and chi-squared tests. Results: Four clusters were identified: 'substance consumers', 'media lovers', 'active students' and 'passive students'. Each cluster exhibited different characteristics related to health behaviours and social determinants. Conclusions Interesting associations between health behaviours were identified, which showed the relevance of considering the adolescents' overall lifestyles. The description of each cluster permitted the identification of risks and protective factors, which may be important for designing effective health promotion activities. [ABSTRACT FROM AUTHOR]

Objectives: Physical activity (PA) is a key factor in cardiovascular disease prevention. Through the Health Action Process Approach (HAPA), the present study investigated the process of change in PA in coronary patients (CPs) and hypertensive patients (HPs). Design: Longitudinal survey study with two follow-up assessments at 6 and 12 months on 188 CPs and 169 HPs. Main outcome measures: Intensity and frequency of PA. Results: A multi-sample analysis indicated the equivalence of almost all the HAPA social cognitive patterns for both patient populations. A latent growth curve model showed strong interrelations among intercepts and slopes of PA, planning and maintenance self-efficacy, but change in planning was not associated with change in PA. Moreover, increase in PA was associated with the value of planning and maintenance self-efficacy reached at the last follow-up Conclusions: These findings shed light on mechanisms often neglected by the HAPA literature, suggesting reciprocal relationships between PA and its predictors that could define a plausible virtuous circle within the HAPA volitional phase. Moreover, the HAPA social cognitive patterns are essentially identical for patients who had a coronary event (i.e. CPs) and individuals who are at high risk for a coronary event (i.e. HPs). [ABSTRACT FROM AUTHOR]

Hypoxia-inducible factor prolyl hydroxylase inhibitors (HIF-PHIs) are new drugs developed for the treatment of anemia associated with chronic kidney disease (CKD). This class of drugs stimulates endogenous erythropoietin production and, at the same time, improves iron absorption and mobilization of iron stores (less evident with daprodustat, vadadustat and enarodustat). Several studies have been published in the last few years showing that these agents are not inferior to standard therapy in correcting anemia associated with CKD. The efficacy of HIF-PHIs is coupled with a safety profile comparable to that of standard erythropoiesis stimulating agent (ESA) treatment. However, studies with HIF-PHIs were not long enough to definitively exclude the impact of new drugs on adverse events, such as cancer, death and possibly cardiovascular events, that usually occur after a long follow-up period. Kidney Disease: Improving Global Outcomes (KDIGO) recently reported the conclusions of the Controversies Conference on HIF-PHIs held in 2021. The goal of the present position paper endorsed by the Italian Society of Nephrology is to better adapt the conclusions of the latest KDIGO Conference on HIF-PHIs to the Italian context by reviewing the efficacy and safety of HIF-PHIs as well as their use in subpopulations of interest as emerged from more recent publications not discussed during the KDIGO Conference., (© 2024. The Author(s).)

Chronic kidney disease (CKD) represents a major public health issue worldwide and entails a high burden of cardiovascular events and mortality. Dyslipidaemia is common in patients with CKD and it is characterized by a highly atherogenic profile with relatively low levels of HDL-cholesterol and high levels of triglyceride and oxidized LDL-cholesterol. Overall, current literature indicates that lowering LDL-cholesterol is beneficial for preventing major atherosclerotic events in patients with CKD and in kidney transplant recipients while the evidence is less clear in patients on dialysis. Lipid lowering treatment is recommended in all patients with stage 3 CKD or worse, independently of baseline LDL-cholesterol levels. Statin and ezetimibe are the cornerstones in the management of dyslipidaemia in patients with CKD, however alternative and emerging lipid-lowering therapies may acquire a central role in near future. This position paper endorsed by the Italian Society of Nephrology aims at providing useful information on the topic of dyslipidaemia in CKD and at assisting decision making in the management of these patients.

In this study, we aimed at disclosing the main features of paroxysmal nocturnal hemoglobinuria (PNH) clones, their association with presentation syndromes, and their changes during follow-up. A large-scale, cooperative collection (583 clones from 529 patients) of flow cytometric and clinical data was entered into a national repository. Reason for testing guidelines were provided to the 41 participating laboratories, which followed the 2010 technical recommendations for PNH testing by Borowitz. Subsequently, the 30 second-level laboratories adopted the 2012 guidelines for high-resolution PNH testing, both upon order by the local clinicians and as an independent laboratory initiative in selected cases. Type3 and Type2 PNH clones (total and partial absence of glycosyl-phosphatidyl-inositol-anchor, respectively) were simultaneously present in 54 patients. In these patients, Type3 component was sevenfold larger than Type2 (p < 0.001). Frequency distribution analysis of solitary Type3 clone size (N = 442) evidenced two discrete patterns: small (20% of peripheral neutrophils) and large (> 70%) clones. The first pattern was significantly associated with bone marrow failure and myelodysplastic syndromes, the second one with hemolysis, hemoglobinuria, and thrombosis. Pediatric patients (N = 34) showed significant preponderance of small clones and bone marrow failure. The majority of PNH clones involved neutrophils, monocytes, and erythrocytes. Nevertheless, we found clones made exclusively by white cells (N = 13) or erythrocytes (N = 3). Rare cases showed clonal white cells restricted only to monocytes (6 cases) or neutrophils (3 cases). Retesting over 1-year follow-up in 151 cases showed a marked clone size increase in 4 cases and a decrease in 13, demonstrating that early breaking-down of PNH clones is not a rare event (8.6% of cases). This collaborative nationwide study demonstrates a clear-cut difference in size between Type2 and Type3 clones, emphasizes the existence of just two classes of PNH presentations based on Type3 clone size, depicts an asymmetric cellular composition of PNH clones, and documents the possible occurrence of changes in clone size during the follow-up.

The skeletal muscle ryanodine receptor (RyR1), i.e., the Ca 2+ channel of the sarco/endoplasmic reticulum (S/ER), and the voltage-dependent calcium channel Cav1.1 are the principal channels involved in excitation-contraction coupling in skeletal muscle. RYR1 gene variants are linked to distinct skeletal muscle disorders, including malignant hyperthermia susceptibility and central core disease (CCD), mainly with autosomal dominant inheritance, and autosomal recessive myopathies with a broad phenotypic and histopathological spectrum. The age at onset of RYR1 -related myopathies varies from infancy to adulthood. We report the identification of four RYR1 variants in two Italian families: one with myopathy and variants c.4003C>T (p.R1335C) and c.7035C>A (p.S2345R), and another with CCD and variants c.9293G>T (p.S3098I) and c.14771&#95;14772insTAGACAGGGTGTTGCTCTGTTGCCCTTCTT (p.F4924&#95;V4925insRQGVALLPFF). We demonstrate that, in patient-specific lymphoblastoid cells, the c.4003C>T (p.R1335C) variant is not expressed and the in-frame 30-nucleotide insertion variant is expressed at a low level. Moreover, Ca 2+ release in response to the RyR1 agonist 4-chloro-m-cresol and to thapsigargin showed that the c.7035C>A (p.S2345R) variant causes depletion of S/ER Ca 2+ stores and that the compound heterozygosity for variant c.9293G>T (p.S3098I) and the 30-nucleotide insertion increases RyR1-dependent Ca 2+ release without affecting ER Ca 2+ stores. In conclusion, we detected and functionally characterized disease-causing variants of the RyR1 channel in patient-specific lymphoblastoid cells. This paper is dedicated to the memory and contribution of Luigi Del Vecchio.

Background: recently, healthcare network models have been proposed to improve general awareness of rare diseases for patients and specific knowledge about diagnosis, treatment, and management for healthcare services. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare haematological disease that still has no framing in an official network., Objectives: to describe the use of network models in diagnosis, treatment, and management of PNH patients both in Italy and abroad and its impact on patients and healthcare service. DISEGN: literature search was performed using the keywords "Hemoglobinuria", "Network", "PHN", and "Screening" in both MedLine and EMBASE. Search was restricted to the articles published in the last 5 years and written in English, French or Italian language., Results: from the total 251 articles of the initial search, only 21 were finally included in our review. None of the included study explicitly described a network model. In general, we were able to identify two different kind of networks implicitly described in the studies: laboratory networks for diagnostic harmonization or screening of the population at risk of PNH (10/21 studies) and PNH registry as network of clinical information to be use for better understanding of the natural history of the disease and to assess therapeutic effectiveness (11/21 studies)., Conclusions: few network approaches in PNH diagnosis, treatment, and management are described in literature. Despite the scarce application of the networks, our review highlights the positive impact that networks have in both patients and healthcare services.

Background and Aims: The prognostic role of LDL in non-dialysis chronic kidney disease (CKD) is still undefined. We addressed this question in a multicenter prospective study including patients referred to nephrologist for management., Methods and Results: 1306 patients with CKD stage III-V were studied at basal visit in 79 Italian nephrology clinics in 2004-2006, and then followed for survival analyses. Study endpoints were incident cardiovascular -CV events (fatal and major non-fatal) and renal events (start of renal replacement therapy or eGFR halving). Mean age was 67.6 ± 11.8 years, male 65%, diabetes 25%, CV disease 27%, and eGFR 35.8 ± 12.5 mL/min/1.73 m(2). LDL was 119 ± 40 mg/dL, with high levels in 50.1% and 82.8% defined on the basis of the individual CV risk profile estimated according to ATPIII 2001 and ESC 2012 guidelines (LDL 100 to 160, and >70 or >100 mg/dL, respectively). Over a median follow up of 2.87 years, 178 CV and 181 renal events occurred. At multivariable Cox analyses, CV risk linearly increased with higher LDL (hazard ratio-HR per 40 mg/dL higher LDL: 1.20, 95% confidence intervals-CI 1.03-1.39); risk doubled when considering high LDL defined according to ESC 2012 (HR 2.37, 95%CI 1.39-4.03) while this association was not significant when considering the higher threshold levels of ATPIII 2001 (HR 1.10, 95%CI 0.82-1.49). No association emerged between LDL and renal risk., Conclusion: In non-dialysis CKD patients, CV risk increases linearly with higher LDL and is more than doubled when considering the lower threshold values currently indicated for defining optimal LDL level., (Copyright © 2015 Elsevier B.V. All rights reserved.)

The authors deal with the theme of the genesis and the increase of the obstetrical collection of pelvic abnormalities, i.e. the "dry" specimens stored at the Ospedale Maggiore (Major Hospital) of Milan (Italy), and of their scientific and educational values. Recently, following the restoration of the crypt of the Annunciation Church of the Ospedale Maggiore, a large space was converted, which could accommodate the pelvises collection permanently. A first step tofinally be able to introduce these extraordinary specimens not only to experts, but also to a wider audience.

Background: In patients chronically treated with hemodialysis, the prevalence of heart failure is high with a consequently poor prognosis. The role played by blood pressure (BP) on cardiovascular (CV) mortality of these patients has not been clearly defined., Methods: In this follow-up study, we investigated the relationship of pre- and postdialysis measurements of BP with CV and all-cause mortality in a cohort of 557 dialysis patients with a left ventricular (LV) ejection fraction <50%., Results: During the follow-up (mean = 21.6 ± 8.8 months), 179 deaths were recorded. Ninety-eight patients died from CV causes. By the Cox multivariable analysis, we constructed a predictive model of CV mortality including age, duration on dialysis, diabetes, serum albumin, diffusive dialysis technique, predialysis mean arterial pressure (MAP) (hazard ratio (HR) = 0.978; 95% confidence interval (CI) = 0.956-0.999), and postdialysis MAP (HR = 1.035; 95% CI = 1.010-1.061). The relationship with mortality was inverse for predialysis MAP and direct for postdialysis MAP. In a subsequent analysis, we found that pre- and postdialysis systolic BP, but not diastolic BP, were predictive of CV mortality. Predialysis MAP was in a direct relationship with body mass index. Postdialysis MAP had an inverse relationship with weight loss during dialysis session., Conclusions: CV mortality in dialysis patients with LV dysfunction is associated with both pre- and postdialysis BP interacting in a complex relationship. Nutritional state and fluid balance and removal are possible clues to this relationship.

Background: Hypertension is very common among patients receiving hemodialysis; however, little is known about its prevalence and control following the publication of the Kidney Disease Outcomes Quality Initiative (KDOQI) recommendations., Methods: This was a multicenter, observational, prospective, cross-sectional study aimed at evaluating the prevalence of hypertension and its awareness in a large sample of hemodialysis patients in Italy, and assessing possible relationships between high blood pressure (BP) values and traditional and nontraditional cardiovascular risk factors. Predialysis hypertension was defined as systolic BP (SBP) / diastolic BP (DBP) =140/90 mm Hg, and postdialysis hypertension as SBP/DBP =130/80 mm Hg or the use of antihypertensive medications., Results: We collected data for 4,022 patients (men/women 2,478/1,544, mean age 67.14 ± 14.08 years) from 77 dialysis centers. Of these, 2,832 patients (70.3%) were defined as having predialysis hypertension. At logistic regression analysis, diabetes, months on dialysis, serum albumin levels and treatment with erythropoiesis-stimulating agent (ESA) were independent factors predicting predialysis hypertension. Antihypertensive agents were used in 57.7% of the patients, leading to adequate BP control in only 40% of them. Factors independently predicting inadequate BP control were diabetes, ESA therapy, high serum cholesterol and higher Kt/V values., Conclusions: Hypertension is highly prevalent in this Italian hemodialysis population; achievement of adequate BP control is inadequate. It is unclear whether this may reflect suboptimal diagnosis or treatment of hypertension or, more likely, the allowance of higher predialysis BP values to try to avoid abrupt BP falls during the dialytic session.

Studies on the relationship between blood pressure (BP) and mortality among hemodialysis patients have yielded conflicting results. Reports have come mostly from North America and have dealt with dialysis patients as a homogenous population and differed in methods and time of BP measurement and the optimal BP target. In a prospective nationwide study in 3674 unselected Caucasian patients with end-stage renal disease undergoing chronic hemodialysis from 73 dialysis units, the authors sought to examine the relationship between the different measurements of BP and mortality according to antihypertensive treatment. The mean age of patients was 67.2±14.1 years and the prevalence of diabetes was 19.5%. During follow-up (26.5±10.5 months), 977 deaths were recorded. In the whole cohort, BP was not associated with mortality. After grouping the patients according to antihypertensive treatment, the analysis showed that only in patients who did not take antihypertensive medications (1613) was there an inverse relationship between postdialysis systolic BP and mortality. These patients differed from the others in BP, dialysis vintage, prevalence of diabetes, and type of dialysis technique. This study suggests that with respect to the relationship of BP with mortality, dialysis patients are not a homogenous population. Differences in demographic characteristics and in dialysis technique may therefore explain the reported variability of previous results., (© 2013 Wiley Periodicals, Inc.)

Background: The available self-report questionnaire for the quality of life in patients with primary biliary cirrhosis (PBC-40) is currently validated only in the British population but it lacks an evaluation of its dimensionality., Aims: To validate the Italian and Japanese versions of PBC-40 and to assess the dimensionality of the original structure of PBC-40 by a confirmatory factor analysis. PBC-40 was translated to Italian and Japanese using the forward-backward method and then reviewed in focus groups in the framework of a large multicentric study., Methods: A sample of 290 patients with PBC (125 Italian and 165 Japanese) was administered two questionnaires previously validated for PBC-specific (PBC-40) and general quality of life (SF-36)., Results: The confirmatory model failed to fit adequately the original hypothesized structure. A principal component analysis led to a seven-factor structure, with exclusion of 13 items characterized by lower load; PBC-27 questionnaire was the final instrument. The validity of the PBC-27 was supported by its strong correlation with the SF-36 scores., Conclusion: We here propose an alternative structure of the quality of life questionnaire for PBC, namely PBC-27, which appears to be effective in detecting the impact of PBC on quality of life in Italian and Japanese patients., (Copyright 2010 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

The mortality rate in patients with end-stage renal disease (ESRD) is extremely high, mainly because of the high prevalence of cardiovascular disease. In addition to traditional cardiovascular risk factors, other factors peculiar to chronic kidney disease play a role. Anemia and calcium-phosphate disorders are of particular interest, not only because they have been related to an increased risk of death but, more importantly, because they can be reversed by treatment, thereby providing the opportunity to prevent or delay the onset of cardiovascular disease. Despite a clear association between higher hemoglobin levels and better survival, data from interventional trials do not seem to show a significant positive effect of hemoglobin normalization with erythropoiesis-stimulating agents on survival and left ventricular mass in ESRD patients. Nevertheless, partial correction of anemia is still an important goal to be reached, as is also suggested by international guidelines. Disorders of calcium-phosphate metabolism have also been clearly related to increased mortality. Unlike anemia, which can be easily corrected by treatment in most cases, mineral metabolism is much less effectively treated. New agents, such as phosphate binders not containing calcium and aluminum, vitamin D analogs with lower calcemic activity, and calcimimetics, are becoming increasingly available in everyday clinical practice and are likely to allow a higher percentage of patients to achieve the recommended targets for calcium-phosphate and parathyroid hormone. Given that these molecules have only been introduced recently, clear data from interventional studies showing improved survival after adequate correction of mineral metabolism parameters are still lacking.

Background: Data collected from registries provide a useful source of information for clinical practice. Therefore, several regional and national registries of end-stage renal disease (ESRD) patients have been established. The Lombardy Registry of Dialysis and Transplantation (RLDT) was established in 1982, with participation of all 44 dialysis units that were present at that time within the region., Methods: Demographic and clinical data on ESRD patients are collected yearly. We present here the results of some of the analyses that have been performed on RLDT data since it was started., Results: Briefly, data on epidemiology of ESRD, cardiovascular disease, patterns of care and patients' outcomes have been considered. Comparisons with international registries have also been performed., Conclusions: This analysis shows how data collected from a homogeneous patient population receiving similar patterns of care provide precise information on that population. A clear example is provided by the similar results obtained in the comparison of high-flux vs low-flux membranes in a randomized control trial, the HEMO study, and in an analysis of RLDT data. Therefore, analysis of data collected by registries represents an important tool to improve clinical practice and possibly patients' outcomes.

Chronic kidney disease (CKD) affects a significant percentage of the Italian population, particularly among the elderly. It is estimated that more than 300 patients per million population (pmp) are diagnosed as having CKD each year, and about 0.8% of Italians are thought to have serum creatinine levels >=1.5 mg/dL. The number of patients being admitted to renal replacement therapies (RRT) has been growing up rapidly in the last decades, leading to 134 patients pmp starting RRT throughout 2000 and to 804 patients pmp on chronic RRT in the same year. As such therapies are very expensive, CKD must be therefore considered as a striking problem also by a socio-economical point of view. As a consequence, any medical intervention being able to halt or at least to slow down the progression of CKD and/or to prevent the development of related complications or comorbidities is of paramount importance. Several therapeutical interventions, including hypertension and proteinuria control, protein restriction, anemia, calcium-phosphate disorders and dyslipidemia correction and smoking cessation, showed to be actually effective in at least partially achieving these objectives. Other emerging therapeutical approaches, although well promising, need further evidence to be definitively included in the management of CKD patients. Particular efforts should be made in order to refer these patients to the nephrologist as early as possible, as it has been widely demonstrated that an early and regular nephrological care leads to decreased morbidity and mortality and also to decreased social costs.

Objective: This study presents the results of a 5-year surveillance program involving the prospective follow-up of healthcare workers (HCWs) in the Veneto region of Italy exposed to blood-borne viruses., Design: All HCWs who reported an occupational exposure to blood-borne infection joined the surveillance program. Both HCWs and patients were tested for viral markers (hepatitis B surface antigen [HBsAg], antibody to hepatitis B surface antigen [anti-HBs], antibody to hepatitis B core antigen [anti-HBc], antibody to hepatitis C virus [anti-HCV], HCV RNA, and antibody to human immunodeficiency virus [HIV]) and had these markers plus transaminases assayed at 3, 6, and 12 months and then yearly thereafter. Moreover, a program of hepatitis B virus (HBV) prophylaxis was offered to those whose anti-HBs levels were less than 10 IU/mL., Participants: Two hundred forty-five HCWs (156 women and 89 men) with a mean age of 37 (+/- 10) years who reported occupational exposure during the 5-year period., Results: At the time of exposure, 1 HCW was positive for HBsAg (0.4%) and 2 were positive for HCV RNA (0.8%). Among the patients involved, 28 (11.4%) were positive for HBsAg, 68 (27.8%) were positive for HCV RNA, 6 (2.4%) were positive for HIV, and 147 (60.0%) were negative for all viral markers (4 patients were positive for both HCV and HIV). During the follow-up period after exposure (mean, 2.7 [+/- 1.6] years), there was no increase in transaminases or seroconversions to any of the viral markers., Conclusion: Our accurate postexposure follow-up revealed a lack of transmission of HBV, HCV, and HIV.

Fifteen cases of ulnar nerve neuropathy at cubital tunnel level were observed in women aged between 16 and 37 years employed in shoe factories. The women operated column sewing machines resting their elbows on the work table. Past evidence showed that the interval between the beginning of work and onset of neuropathy was generally more than one year but less than that for similar diseases observed in other jobs where arthritis of the elbow played a more important role and the average age was higher. It can therefore be supposed that arthritis in these shoes workers was of less significance, whereas local trauma, which was not prevented by the use of small soft cushions under the elbows (4 out of the 15 subjects had been using them for some time), seemed of greater importance. A period of less than one year (in one case only a few days) was deemed due to the presence of a pre-existing neuropathy of the elbow which had so far been asymptomatic. Cases treated within six months of onset showed marked improvement. However, rapidly worsening symptoms, independent of the degree of damage reached, often led to early treatment with favourable outcome in a short time, while slow and insidious onset brought a longer prognosis. The primary prevention suggested is to instruct workers as to the correct work posture, i.e., avoiding resting the elbows on the work table. As a secondary preventive measure, bearing in mind the need of early diagnosis and treatment, medical check-ups every six months are proposed.

A group of 20 patients affected with thyroid carcinoma, all from Southern Italy, was typed for HLA-A, -B, -C and -DR antigens. Sixteen of them (80%) typed for DR1, while in the control group (120 unrelated healthy individuals from the same region) only 22 (18.3%) shared the same antigen (chi 2 = 32.96). The data indicate a highly significant association between the HLA-DR1 gene and the thyroid carcinoma.