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Patellar tendinopathy, or Jumper’s knee, is a painful knee condition caused by inflammation of the patella tendon. This condition is most frequently observed in subjects who play sports that require repetitive regular jumping. Jumper’s knee is frequently misdiagnosed as a minor injury and many athletes, like our patient, keep on training and competing and either tend to ignore the injury or attempt to treat it themselves. However, jumper’s knee is a serious condition that requires a correct and timely diagnosis, which often necessitates ultrasound investigation in order to start the most appropriate treatment.

The objective of this prospective study was to investigate the transition from primary (PRP) to secondary (SRP) Raynaud’s phenomenon (RP), in a large cohort of patients affected by isolated RP. A total of 2065 patients with RP were investigated by clinical interview, laboratory examinations, and nailfold videocapillaroscopy (NVC). Patients with negative NVC at first visit were yearly followed to monitor either the appearance of specific morphological alterations at NVC, or clinical manifestations of an underlying disease. Capillary abnormalities at NVC were scored, as well as the qualitative patterns of microangiopathy (Early, Active and Late). NVC was found negative at first visit in 1500 subjects; among them, 412 patients were evaluable and they were followed for a mean time of 5±4 years (range 2-13 years). Sixty-eight patients (16%) achieved a diagnosis of SRP during follow-up, showing normal or not specific capillary alterations at NVC 4% of patients (the diagnosis was undifferentiated connective tissue diseases), Early scleroderma-pattern 57%, Active scleroderma-pattern 7%, Late scleroderma-pattern 12%, and scleroderma-like pattern 18% of patients. The time of transition from normal/not specific capillary alterations to Early scleroderma-pattern was 4.4±3.8 years. Enlarged capillaries (diameter between 20 and 50 microns) and mild reduction of capillary density were found the more frequent markers at first NVC visit in patients who progressed to a scleroderma pattern (P=0.01). This study demonstrates in a large cohort, that almost 16% of patients initially diagnosed as affected by RP with negative NVC may transit to SRP during a mean follow-up of 4.4 years. PRP patients showing major notspecific alterations of nailfold capillaries at first NVC should be strictly monitored at least once a year since at higher risk of transition to SRP.

Not available.

Introduction: Italian Cystic Fibrosis Registry (ICFR) collects data of patients with cystic fibrosis (CF) through the collaboration with Italian CF referral and support Centres (Italian law 548/93). It aims at analysing medium and long-term clinical and epidemiological trends, identifying healthcare needs at regional and national levels, contributing to healthcare programmes, and resource allocation. Italian data are also compared at international level through the collaboration with the European CF Registry for sharing epidemiological data on general aspects like CF epidemiology and specific topics such as the use of CFTR modulators., Objectives: The purpose of this Report is to provide updated demographic and clinical data of the Italian FC population for the years 2021 and 2022, to contribute essential information for the implementation of projects aimed at improving the management of patients affected by this disease., Design: Analyses and results presented in this Report pertain to patients currently under care at Italian National Referral and Support Centres for Cystic Fibrosis and Paediatric Hospital 'Bambino Gesù' in the 2021-2022 period. Data were submitted by clinical Centres through a dedicated web-based software and underwent dual quality control (QC) measures: automated quantitative QC within the software and secondary QC at the European level before the integration into the European Cystic Fibrosis Registry. These measures ensure data completeness, accuracy, and longitudinal consistency with European core data., Setting and Participants: A total of 27 CF Centres, including referral and support centres, as well as 'Bambino Gesù' Children's Hospital CF centre, submitted their data to ICFR for the years 2021-2022. Althourgh CF Centres in Verona and Messina do not use the ICFR software, their data are centrally collected and subsequently forwarded to the European Registry. Data from service centres in Treviso and Rovereto are transmitted via the Verona CF Centre. Data from Sardinia Centre are currently unavailable., Results: The results section provides a comprehensive overview of various aspects of CF epidemiology and patient characteristics. 1.Demography: in 2021 and 2022, 5,977 and 6,077 CF patients were respectively included in the ICFR, with median ages of 23.3 and 23.7 years. The prevalence rates were 10.1 and 10.3 per 100,000 residents in Italy for the respective years, with males comprising 51.6% on average. The distribution by age showed a higher frequency among patients aged 7 to 35 years; adult patients constituted 63.5% on average in both years. 2. Diagnosis: most CF patients were diagnosed before the age of two (mean value 57.9%), with a significant percentage diagnosed in adult age (35.4% in 2021 and 25.6% in 2022). 3.New diagnoses: there were 113 new diagnoses in 2021 and 121 in 2022, with estimated incidences of 1 in 9,097 living births in 2021 and 1 in 6,232 in 2022. 4. Genetics: genetic analyses were conducted on 99.9% of patients, revealing CFTR gene mutations in over 98% of cases. The F508del mutation was the most common (44% of alleles in 2021), with 18% of patients having at least one "residual function" mutation. Gating mutations were present in 3.4% of Italian patients, while 20% had at least one-stop codon mutation. 5.Lung function: lung function, measured by percent predicted (pp)FEV1 (Forced Expiratory Volume in the first second) progressively declined before adulthood, with the majority of paediatric patients (92.8% in 2021 and 93.8% in 2022) maintaining a ppFEV1≥70%. 6.Nutrition: critical periods for nutrition were identified as the first 6 months of life and adolescence, with higher prevalence of malnourished male adolescents compared to females. Suboptimal BMI values were more common in adult females (28.7% in 2021 and 26.9% in 2022) compared to males (14.2% in 2021 and 12.6% in 2022). 7. Complications: CF-related liver disease without cirrhosis was prevalent in patients under 18 years (21.9% in 2021 and 21.2 in 2022), while CF-related diabetes was most frequent in adults (24.2%). 8.Transplantation: over the two-year period, 28 patients underwent double-lung transplantation, with median ages of 29.1 in 2021 and 35.3 in 2022, respectively. Median waiting times ranged from 9.4 to 11.6 months. 9.Microbiology: chronic Pseudomonas aeruginosa infection affected 37.2% of adult patients in 2021 and 36.0% in 2022, compared to 7.4% and 6.5% in paediatric patients. Staphylococcus aureus infection rates were 34.6% and 42.2% in 2021 among adults and 34.4% and 36.7% in 2022 among paediatric patients. 10. Mortality: a total of 34 patients died during the 2021-22 period (19 females, 15 males), with median ages at death of 43.7 years in 2021 and 46 years in 2022 (excluding transplanted patients)., Conclusions: The present Report is an update of the data published in the past years and summarizes the main epidemiological and clinical data regarding Italian CF subjects in the years 2021 and 2022. The number of patients registered in 2021 was 5,977, while in 2022 was 6,077. The population coverage estimates for 2022 to be around 97%. In 2020, 60.5% of patients were older than 18 years, in 2022 adult patients account for 63.5% of the Italian CF population. Over the years, therefore, an increase in the median age of Italian CF patients has been observed, reaching 23.7 years in 2022. The absolute number of new diagnoses per year remains substantially unchanged over the years (a total of 234 in the period under review). The median age at diagnosis in 2022 was 2.5 months, 62.6% of subjects are really diagnosed within the first year of life and almost 90% of them are diagnosed through neonatal screening. In 2022, almost all patients underwent genetic analysis (99.9%). Data collected confirm the great variability among Italian CF patients. As regards respiratory function, what is reported in previous reports is here confirmed, with an ever-increasing percentage of subjects under the age of 18 having normal respiratory function, moreover, less than 1% of paediatric patients has a severe lung function (ppFEV1<40). The marked improvement in this indicator in the adult population seems to be mainly due to the introduction from 2021 in Italy of therapy with highly effective CFTR modulators. At the same time, the close positive correlation between nutritional status and respiratory function is confirmed for the adult population. As regards chronic infection by Pseudomonas aeruginosa, in 2022, a reduction in the percentage of chronic infection is observed both among adults (36% vs 38.8% in 2020) and in paediatric patients (6.5% vs 7.6% in 2020). The most frequent complication in both paediatric and adult populations is liver disease (respectively, in 24.2% and 41.3% of subjects). In the two-year period, 34 patients died; their median age at death was between 43 and 46 years (transplant patients excluded); only two patients under the age of 18 died in the period 2021 and 2022, confirming once again that mortality in paediatric age is a rare event. The data presented in this Report shows how the register can be a national and international point of reference for CF patients and the scientific community, a tool for describing the Italian CF population over the years, and a starting point for planning epidemiological studies and clinical studies.

Introduction: Italian cystic fibrosis registry (ICFR) collects data from cystic fibrosis (CF) patients through the collaboration with Italian CF referral and support Centres (Italian law 548/93). ICFR contributes: • to the analysis of medium and long term clinical and epidemiological trends of the disease; • to the identification of the main health care needs at regional and national level to contribute to the Health Care programmes and to the distribution of resources; • to the comparison of the Italian data with international ones. This latter is based on the collaboration with the European CF registry and, due the COVID-19 pandemic emergency, with important global projects., Objectives: The purpose of this Report is to update the demographic and clinical data of the Italian FC population in the years 2019 and 2020, contributing to the information necessary to implement projects to improve the management of patients affected by this disease., Design: Analyses and results described in the present Report are referred to patients currently followed at the Italian National Referral and Support Centres for Cystic Fibrosis in the 2019-2020 period. Data were sent by clinical Centres through a dedicated web-based software. Data undergo a double quality control (QC): the first is automatically performed by the software (quantitative QC), the second is performed at a European level (before the inclusion of the Italian data within the European Cystic Fibrosis Registry). These QCs assure the completeness and the accuracy of data as well as their longitudinal consistency with the European core data., Setting and Participants: A total of 29 CF Centres (referral and support centres and 'Bambino Gesù' Children's Hospital CF centre) sent to ICFR their data referred referred to years 2019-2020. CF Centres of Verona, Messina, and Palermo (this latter only for 2019) do not use the ICFR software; however, their data are firstly collected in a centralized manner, then sent to the European Registry. Data from support centres of Treviso and Rovereto are sent through the Verona CF Center. Finally, data from Sardinia Centre are still missing., Results: The present Report has been organized into 10 sections. 1. Demography: in 2019, 5,585 CF patients were registered in the ICFR and 5,801 in 2020; median age was 21.6 years in 2019 and 22.4 years in 2020. Prevalence was 9.36/100,000 and 9.79/100,000 residents in Italy in 2019 and in 2020, respectively. Male percentage was 51.5% in 2019 and 2020 and CF distribution by age range showed higher frequency in patients aged 7 to 35 years. Adult patients (aged more than 18 years) were 59.5% on average in both years. 2. Diagnoses: most of the CF patients were diagnosed before two years of age (median value 68.5%); a significant percentage of patients (12.9% in 2019 and 13.4% in 2020) was diagnosed in adult age. 3. New diagnoses: new diagnoses were 136 in 2019 and 96 in 2020. Estimated incidence was 1/5.568 living births in 2019 and 1/7.369 in 2020. 4. Genetics: 99.9% of patients underwent genetic analyses and in 98.2% of these patients a mutation in Cystic Fibrosis Transmembrane Regulator (CFTR) gene was identified. The F508del mutation was the most frequent (identified in 44.7% allele; 2019 data). Furthermore, on average 17.3% of patients had at least one ‘residual function’ mutation. At least one gating mutation is present in 3.3% of Italian patients. Finally, 20.5% of patients had at least one stop codon mutation (class 1). 5. Lung function: percent predicted FEV1 (Forced Expiratory Volume in the first second) progressively declined before adult age, in accordance with the natural history of the disease. The majority of paediatric patients (6-17 years of age), i.e., 86.7% in 2019 and 90.5% in 2020, had percent predicted FEV1 >=70%; whereas paediatric patients with a FEV1% >=40% are less than 2% in the study period. 6. Nutrition: the two most critical periods are the first 6 months of life and adolescence. Prevalence of malnourished adolescent males (12-17 years of age) is higher than the prevalence observed in females. Increasing percentages of female patients with a suboptimal BMI value (33.5% and 31.4%, respectively, in 2019 and 2020) are observed in adult age. 7. Complications: in 2019, CF-related liver disease without cirrhosis was the main complication both in patients aged less than 18 years (20.3% on average) and in adults (37.5%). CF-related diabetes was also frequent in CF adults (23.4%). 8. Transplantation: in 2019-2020, 64 patients received a double-lung transplantation. Median and range of age were 33 years (12.29-57.46) in 2017 and 32.9 (16.5-53.6) years in 2020. Median waiting times for lung transplantation in the two-year period ranged from 6 to 8 months. 9. Microbiology: percentage of adult patients with chronic Pseudomonas aeruginosa infection was 41.6% in 2019 and 38.8% in 2020 vs 14.3% in 2019 and 7.6% in 2020 in paediatric age. Staphylococcus aureus infection is present in 31.1% and 35.9% of adult patients in 2019 and in 33.5% and 34.7% of paediatric patients in 2020. 10. Mortality: a total of 51 patients died in the 2019-2020 period (28 females and 23 males); median age at death was 35.7 years in 2019 and 39 years in 2020 (transplanted patients are not included)., Conclusions: The present report shows that the Italian CF population is growing (4,159 in 2010 vs 5,801 in 2020). Median age of patients increased in the 2010-2020 period (17 years in 2010 vs 22.4 years in 2020). Prevalence of adult patients is increasing (in 2020, 60.5% of patients is more than 18 years old). About 68.5% of new patients is diagnosed within the second year of life and median age at death (transplanted patients not included) increased in 2020 up to 39 years (in 2018 this value was 35.8). Some statistical differences between 2019 and 2020 are mainly due to the absence of about 200 patients not included in 2019 data by a participating centre for a technical problem.

Objectives: to describe the course of COVID-19 epidemic in the hospitals of the ASST of Mantua (Lombrady Region, Northern Italy) from February 2020 to April 2021., Design: observational study., Setting and Participants: data from hospital discharging chart of all patients admitted to the hospitals of ASST were collected from 26.02.2020 to 30.04.2021 with COVID-19 diagnosis. Data from Emergency Rooms for patients evaluated but not admitted to departments were also collected., Main Outcome Measures: the data from hospital discharging were crossed for diagnosis with data from laboratory. The department were classified into 'low intensity' and 'middle/high intensity'. The comparison was according to the different periods of epidemic., Results: patients admitted to the hospitals were 2,738: 510 died (17.3%) and 1,736 patients were evaluated in the Emergency Rooms but not admitted to departments. Among these patients, 166 died (9.6%). The prevailing age class were >=65 years, with a trend to reduction in the third wave. The proportion of admission in middle/high intensity departments was significantly higher in the second wave than in the first. N. 510 deaths by 2,738 (17.3%) were observed, with significant reduction in the second and third waves in the low intensity departments (from 21.9% to 14.3% and 12.7%) (p<0.001), while mortality was substantially unchanged in the middle/high intensity departments (28.0%, 29.6%, and 28.3%). The mortality for patients with >=65 years was 26.7%. Females showed lower mortality (OR 0.690; CI95% 0.560-0.840) and lower incidence of admissions in middle/high intensity departments (OR 0.556; CI95% 0.459-0.673) in the three waves. Finally, including also the patients not admitted, the general mortality was 15.1%., Conclusions: a worse outcome by mortality and severity of disease was observed for male gender compared to female and for older age classes. Moreover, a significant improvement of outcomes in the second and third waves, compared to the first, was pointed out.

Introduction: On the 15th of November 2020, the National Centre for Rare Diseases of the Italian National Health Institute, clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, Children's Hospital "Bambino Gesù", Italian Cystic Fibrosis Society, Italian League for Cystic Fibrosis renewed the agreement about CF data flow for a 3-year period. The possibility to access data by third parties is among the most important innovation introduced within the agreement., Objectives: Aim of the present Report is to improve the know-how of cystic fibrosis (CF) through a better characterization of Italian patients. Furthermore, the present Report aims at improving the care of CF patient. In particular, this Report should contribute to the following objectives: • to analyse the medium- and long-term clinical and epidemiological trends of the disease; • to identify the main healthcare needs at regional and national level, in order to contribute to the healthcare programmes and to the distribution of resources; • to compare Italian data with international ones., Design: Analyses and results described in the present Report are referred to patients currently followed at the Italian National Referral and Support Centres for Cystic Fibrosis in the 2017-2018 period. Data were sent by clinical Centres through a new-committed software. Data underwent a double quality control (QC): the first is automatically performed by the software (quantitative QC), the second is performed at a European level (before the inclusion of the Italian data within the European Cystic Fibrosis Registry). These QCs assure the completeness and the accuracy of data as well as their consistency with the European core data., Setting and Participants: The present Report has been organized into 10 sections. 1. Demography: in the ICFR, 5,565 CF patients were registered in 2017 and 5,501 in 2018; median age was 21.4 years in 2017 and 21.2 years in 2018. Prevalence was 9.20/100,000 residents in Italy in 2017 and in 2018. Male percentage was 51.65% in 2017 and 2018, CF distribution by age range showed higher frequency in patients aged 7 to 35 years. Adult patients (aged more than 18 years) were 56.4% on average in 2017 and 2018. 2. Diagnoses: most of the CF patients were diagnosed before two years of age (median value 66.4%); a significant percentage of patients (21.6% in 2017 and 18.3% in 2018) was diagnosed in adult age. 3. New diagnoses: new diagnoses were 162 in 2017 and 142 in 2018. Estimated incidence was 1/5.214 living births in 2017 and 1/5.442 in 2018. 4. Genetics: 99.8% of patients underwent genetic analyses and in 97.1% of these patients a mutation in Cystic Fibrosis Transmembrane Regulator (CFTR) gene was identified. The F508del mutation was the most frequent (44.6% in 2018). Furthermore, 16.3% of patients in 2017 and 16.9% of patients in 2018 had at least one 'residual function' mutation. At least one gating mutation is present in 3.3% of Italian patients. Finally, 20.5% of patients had at least one stop codon mutation (class 1). 5. Lung function: percent predicted FEV1 (Forced Expiratory Volume in the first second) progressively declined before adult age, in accordance with the natural history of the disease. The majority of paediatric patients (6-17 years of age), i.e., 86.70% in 2017 and 90.50% in 2018, had percent predicted FEV1 ≥70%; whereas paediatric patients with a FEV1% ≤40% are less than 2% in the 2017-2018 period. 6. Nutrition: the two most critical periods are the first 6 months of life and adolescence. Prevalence of malnourished adolescent males (12-17 years of age) is higher than the prevalence observed in females. Increasing percentages of adult female patients with a suboptimal BMI value (39.1% and 36.1%, respectively, in 2017 and 2018) are observed. 7. in 2018, CF-related liver disease without cirrhosis was the main complication both in patients aged less than 18 years (17.0% on average) and in adults (31.5%). CF-related diabetes was also frequent in CF adults (23.4%). 8. Transplantation: in 2017-2018, 83 patients received a double-lung transplantation. Median and range of age were 29.3 years (11.8-60.2) in 2017 and 29.1 (7.8-45.6) years in 2018. Median waiting times for lung transplantation in the two considered years were 8.6 and 7.7, respectively. 9. Microbiology: percentage of adult patients with chronic Pseudomonas aeruginosa infection was 51.3% in 2017 and 46.3% in 2018 vs 15.6% in 2017 and 10.2% in 2018 in paediatric age. Staphylococcus aureus infection is present in 53.4% and 53.5% of adult patients in 2017 and in 41.6% and 37.5% of paediatric patients in 2018. 10. Mortality: a total of 89 patients died in the 2017-2018 period (49 females); median age at death was 33.9 years in 2017 and 35.8 years in 2018 (transplanted patients are not included)., Conclusions: The present report shows that the Italian CF population is growing (4,159 in 2010 vs 5,501 in 2018; +1,342). Quality of data collected has been improved by the drastic reduction of missing data, thanks to the new software for data collection. Median age of patients increased in the 2010-2018 period (17 years in 2010 vs 21.2 years in 2018). Paediatric death is a very rare event. A very low percentage of paediatric population was characterized by severe lung disease (FEV1% <40). Prevalence of adult patients is increasing (56.4% in 2018). Age at diagnosis is decreasing (4.2 months in 2017 vs 3.8 months in 2018). Median age at death (transplanted patients not included) was 33.9 in 2017 and 35.8 in 2018. RIFC is completely compliant with the GDPR (UE 2016/679 regulation) and its role in national and international CF communities is confirmed., Competing Interests: None

Introduction: On the 27th of October 2017 the National Center for Rare Diseases of the Italian National Health Institute (NHI), clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, Paediatric Hospital "Bambino Gesù", Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis renewed the agreement about FC data flow for a 3 years period. The possibility to access data by third parties is among the most important new introduced within the agreement., Objectives: Aim of the present report is to improve the know-how on cystic fibrosis (CF) through a better characterization of Italian patients. Furthermore, the present Report aims at improving the care of CF patient. In particular, the Report should contribute to the following objectives: * to analize medium- and long-term clinical and epidemiological trends of the disesase; * to identify the main health care needs at regional and national level in order to contribute to the healthcare programmes and to the distribution of resources; * to compare Italian data with international ones., Design: Analyses and results described in the present Report are referred to patients in charge to the Italian National Referral and Support Centers for Cystic Fibrosis in the period 2015-2016. Data were sent by Centres by means of a specific software (Camilla, Ibis Informatica). Data underwent to a double quality control (QC): the first by NHI and the second at a European level (before the inclusion of the italian data within the European Cystic Fibrosis Registry). These QCs assure the completeness and the accuracy of data as well as their consistency with European core data. Finally, in 2017, an additional CQ was performed to further reduce the number of missing data and consequently improve the precision and the consistency in the nomenclature adopted for genetic mutations., Setting and Participants: A total of 29 different CF Centres (referral, support, and Paediatric Hospital "Bambino Gesù") sent their data referred to 2015-2016 years to ICFR . Data regarding Sardinia (Southern Italy) are missing and those from Treviso (Veneto Region, Northern Italy) and Rovereto (Trentino-Alto Adige Region, Northern Italy) are sent through Verona CF Centre., Results: The present Report has been organized into 10 sections. 1. Demography: estimated CF patients is 5,204 in 2015 and 5,362 in 2016; median age is 20.6 and 21.0, respectively. Prevalence is 8.6/100,000 residents in Italy in 2015 and 8.8 in 2016. Male percentage is 51.6% on average for 2015 and 2016; CF distribution showed higher frequency in patients aged from 7 to 35 years. The mean of patients aged more than 18 years is 56.5% on average in 2015 and 2016. 2. Diagnoses: most of the CF patients were diagnosed before 2 years of age (median value: 68%); a significant percentage of patients (median value: 13%) was diagnosed in adult age. 3. New diagnoses: new diagnoses were 169 in 2015 and 153 in 2016. Estimated incidence in 2015 was 1/4,176 living births in 2015 and 1/5,510 in 2016. 4. Genetics: 99.5% of patients underwent genetic analyses and in 96% of patients a mutation in Cystic Fibrosis Transmembrane Regulator (CFTR) gene was identified. [delta]508F was the most frequent mutation (44,7% in 2016). Furthermore, 16.0% and 3.4% of patients was characterized by the presence of at least one "residual function" mutation and gating, respectively. Finally, 21% of patients was a stop codons (class 1 mutation) carrier. 5. Lung function: FEV1 (forced expiratory volume in the first second) scores progressively decreased before adult age, in accordance with the natural history of the disease. FEV1% values in patients between 6 and 17 years of age is ≥70%; patients with a FEV1% value of 40% are less than 2% in the period 2015-2016. 6. Nutrition: most critical periods are during the first 6 months of life and during adolescence. Prevalence of malnourished male aged 12-17 years is constant in 2015-2016 and is always more than the prevalence observed in female. An increasing percentage of female patient with a suboptimal BMI value (35.5%) is observed among patients aged more than 18 years 7., Complications: it was estimated that, in 2016, hepatopathies without cirrhosis (17.7%) is the principal complications in patients aged less than 18 years; in patients aged more than 18 years the principal complication was due to hepatopathies without cirrhosis (29.5%) and diabetes (23.3%). 8. Transplantation: in 2015-2016, 74 patients were bipulmunary transplanted; age was comprised between 8 and 52 years, median age at transplantation was 29,6 years. Median waiting times for transplantation is estimated in 17 months (24 months in 2015 and 14 months in 2016). 9. Microbiology: analyses were referred to test performed in 2016. Percentage of adult patients with chronic Pseudomonas aeruginosa infection is 52.1% compared to 15.2% of paediatric patients; Staphylococcus aureus infection is present in 53.2% of adult patients and 52.8% of paediatric ones; Burkholderia Cepacia complex is present almost exclusively in adult patients (4.3%); Nontuberculous mycobacteria is present in 1.2% and 0.4% of adult and paediatric patients, respectively; Stenotrophomonas maltophilia infection is present in the 6.1% of adult patients and 4.9 of paediatric patients. 10. Mortality: 102 patients (49 males and 53 females; median age 36.9 years in 2015 and 36.5 in 2016) died in 2015-2016 (transplanted patients are not included)., Conclusions: The present Report shows that Italian CF population is growing (median age) and paediatric mortality is decreasing. A very low percentage of paediatric population is characterized by complication of pulmonary function; adult patients are characterized by an increase of age at death (more than 36 years of age in 2016).

Summary: Introduction. Burnout syndrome (BOS) can be defined as a chronic work strain characterized by three dimensions: emotional exhaustion, depersonalization (or cynicism), reduced professional effectiveness. BOS typically strikes the helping professions like the teachers. Objective. The aims of this study are: 1) clarify the concept of BOS, as currently configured in the international scientific literature; 2) analyze the psychosocial risk factors in teachers; 3) develop a methodology for the assessment and prevention of the risk of BOS in the teachers. Methods. The literature review followed some of the PRISMA guidelines criteria. Results and discussion. The results of the study highlight BOS as a risk factor specific and distinct from labor-related stress. A strategy called VA.RI.B.O is proposed (Burn-Out Recognition) for teachers., Competing Interests: The authors of this article have no conflict of interests to disclose., (Copyright© by Aracne Editrice, Roma, Italy.)

Objectives: Aim of our study was to adapt the Claustrophobia Questionnaire (CLQ) to the Italian context., Methods: In our study, a sample of 50 claustrophobic patients was compared to 50 healthy people (control group). All of them answered the Claustrophobia Questionnaire and the Stait-Trait Anxiety Inventory Form Y1 and Y2, as well as demographic questions., Results: As it was theoretically expected, our results confirmed the two-factor structure and showed that the Italian version of the CLQ has good psychometric properties. Indeed, it was observed that claustrophobic patients scored higher in claustrophobia than those from the control group., Conclusions: In conclusion, the Italian version of the CLQ is a reliable and valid instrument to assess claustrophobic fear., Competing Interests: The authors of this article have no conflict of interests to disclose., (Copyright© by Aracne Editrice, Roma, Italy.)

Introduction: The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis., Objectives: The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients. The members of the Scientific and Technical Committee have to write a report on data collected by ICFR, in order to contribute to achieve the aims of ICFR itself, i.e., to improve the care of CF patients. In particular, the Report should contribute to the following objectives: - to analyze the medium and long-term clinical and epidemiological trends of the disease; - to identify the main healthcare needs at regional and national level in order to contribute to the healthcare programmes and to the distribution of resources; - to compare Italian data with the international ones., Design: Analyses and results described in the present Report are referred to patients in charge to the Italian National Referral and Support Centres for Cystic Fibrosis in the period 2011-2014. Data were sent by Centres by means of a specific software (Camilla, Ibis Informatica) and has undergone a double quality control (QC): the first by NIH and the second at a European level (before the inclusion of the Italian data within the European Cystic Fibrosis Registry). These QCs assure the completeness and accuracy of data as well as their consistency with European core data., Setting and Participants: A total of 29 different CF centres (referral, support, and Paediatric Hospital "Bambino Gesù") sent their data to ICFR; data referred to the period 2011-2014. Data regarding Sardinia Region (Southern Italy) are missing; data from Molise (Southern Italy) CF centre refer only to 2014., Results: The present Report has been organized into 10 sections. 1. Demography - number of Italian patients with cystic fibrosis (CF) in 2014 was 4,981 and their median age was 20.4 years; estimated 2014 CF prevalence was 8.2/100,000 residents in Italy; on average, 52.1% of the patients were male and CF distribution showed higher frequency in patients aged from 7 to 35 years. On average, 53.7% of CF patients are aged more than 18 years. 2. Diagnoses - most of the CF patients were diagnosed before two years of age (around 66%); a significant proportion of patients (on average, 12%) was diagnosed in adult age. 3. New diagnoses - new diagnoses were 187 in 2011, 200 in 2012, 160 in 2013, and 135 in 2014. Estimated incidence was 1/4,052 live births in 2011; 1/4,313 in 2012; 1/5,189 in 2013 and 1/8,243 in 2014. 4. Genetics - 99.5% of patients was studied at the molecular level, with identification of 90.1% of Cystic Fibrosis Transmembrane Regulator CFTR mutations; [delta]508F was the most frequent mutation (44.8% in 2014). 5. Lung function - FEV₁ (Forced Expiratory Volume in the first second) scores progressively decreased shortly before the start of adult age, in accordance with the natural history of the disease. Most of the patients between 6 and 17 years of age reported a FEV₁ % ≥ 70% of the predicted value, while the proportion of patients with severe lung disease (FEV₁ % <40% of the predicted value) is <2% over the period 2011-2014. 6. Nutrition - most critical periods come out during the first 6 months of life and during adolescence. Prevalence of malnourished male aged 12-17 years decreases over the period 2011-2014; an increasing percentage of patient (both male and female) with a suboptimal body mass index value is observed among patients aged more than 18 years 7. Complications - the presence of missing data represents an obstacle in the correct evaluation of prevalence value of complications related to Italian patients within ICFR. Nevertheless, it was estimated that, in 2014, the principal complication in patients aged <18 years was hepatopathies (15%), while in patients aged more than 18 years the principal complications were due to hepatopathies (25%) and diabetes (22%). 8. Transplantation - during the period 2011-2014, 135 patients ageed between 7 and 53 years received a double lung transplant; median age at transplantation was 32.5 years. Median duration of waiting list for transplantation is estimated in 11 months. 9. Microbiology - analyses were referred to test performed in 2014. Prevalence of adult patients with Pseudomonas aeruginosa chronic infection is 49.4% compared to 14.5% of paediatric patients; Staphylococcus aureus chronic infection is present in 48% of adult patients and 45.6% of paediatric patients; Burkholderia Cepacia complex is present almost exclusively in adult patients (4.9%); Nontuberculous mycobacteria is present in 0.9% and 0.3% of adult and paediatric patients, respectively; Stenotrophomonas maltophilia infection is present in 4.6% of patients (both adults and paediatric). 10. Mortality - RIFC data show that 176 patients (median age 32 years; 81 males and 95 females) died in the period 2011-2014., Conclusions: The present Report shows that CF population is growing (median age), so paediatric mortality is decreasing. A very low percentage of paediatric population is characterized by complication of pulmonary functions; adult patients are characterized by an increase of age at death (more than 30 years of age). ICFR Report may represent an important tool to analyze clinical and epidemiological trends of the disease as well as to identify the main healthcare needs at regional and national level to contribute to the healthcare programmes and to the distribution of the resources.

The incidence and prevalence of end-stage renal disease increased steadily for 35 years in the population of Italy's Mantua province until the end of 2007, when they started to decrease. We describe the results of providing information and raising awareness among residents of the province's capital, Mantua, and of direct teaching and short training courses in hospital wards for general practitioners over a period of 3 years. During this period there was also more consultation activity for all kidney outpatients, from the first to the last stages of chronic kidney disease. Clinical data collected by the local NHS section (ASL) showed an increase in age and comorbidities (mainly type 2 diabetes) in kidney patients. As a result of the preventive measures, there has been an effective reduction of the yearly incidence and prevalence for all kinds of treatment, with the exception of kidney transplants, which have increased progressively during the last years.

A relevant part of deadly work accidents and of severe work accidents reported in national specific statistics coming from INAIL is until today caused by workers' falls during their at height work activities. Medical evaluation of eventual workers' hyper susceptibility to fall caused by balance alterations, if included in medical surveillance, is frequently carried out by medical doctors using different subjective tests included in different clinical protocols. Use of a computerized stabylometric footboard on the contrary allows to obtain objective and reproducible data concerning vestibular function which, associated with traditional clinical medical evaluation, seem to better detect eventual presence of balance disturbances, especially those non clinically perceivable by workers and therefore more dangerous. We report results evidenced in a research carried out in a group of 185 workers engaged in building firms and having work tasks including phases of at height work.

The aim of this study was to report the frequency of kidney diseases related to gender, age, clinical presentation and renal function at the time of kidney biopsy in the population of Mantua province (400,000 residents). We collected the results of 132 real-time ultrasound-guided fine-needle (18 G) kidney biopsies by optical and immunofluorescence microscopy. The clinical presentation at the time of biopsy was nephrotic syndrome in 57%, nephritic syndrome in 22%, and urinary abnormalities in 21% of cases. Serum creatinine was >-1.5 mg/dL in 48% of patients. Membranous nephropathy was the most frequent histological finding (21.4%), followed by IgA nephropathy (14.5%), focal glomerulosclerosis (11.5%), diabetic nephropathy (8.4%), and chronic interstitial nephritis (6.9%). Primary glomerulonephritis including membranous glomerulonephritis and IgA nephropathy showed a male predominance. The pathological correlations in native biopsy-proven renal disease provided useful information for clinical practice. The histological findings in our patient series are similar to those recorded in Italian and European registries. A less invasive policy in the case of isolated urinary abnormalities and a normal eGFR resulted in a lower incidence of IgA nephropathy in our series than was recorded in the national Italian registry.

Hypertensive crises (i.e., blood pressure > 180/120 mmHg) are common issues in the emergency department. This term is used to indicate both emergencies and urgencies, characterized by presence and absence of target organ damage, respectively. A wide range of therapeutic options are available. The aim of this article is to review current knowledge and treatment trends in the literature to provide clinicians with diagnostic and therapeutic tools.

The cardiovascular disease is largely increased in chronic renal failure and the patients have a 10-20 times higher mortality respect normal population. Besides habitual risk-factors they add the mineral metabolism alterations, iperomocisteine and chronical vessel flogosis. In these patients the vascular disease is often lately diagnosed, but early diagnosis would be extremely important to establish appropriate pharmacologic or surgical treatment (PTA or by pass). The basic diagnostic methods are still digital angiography, angio-NMR or angio-CT. In our experience appears that dialysed patients present high total mortality and re-vascolarization (particularly for peripheral occlusive disease) gives less guarantee of success. During last years endovascular surgery procedures extremely improved short-term prognosis for these patients. When there is no space for the re-vascolarization and the situation is strongly compromised by the presence of extended gangrene or infected lesion, amputation is still indicated and can be considered the only possible solution.

Aim of the study was to investigate polypharmacy and psychotropic drugs as risk factors for falls in a Long-term Care Setting for elderly patients. In a cross-sectional study we investigated the characteristics of 414 subjects: 207 fallen patients 44 (21.3%) male e 163 (78.7%) female mean age 84.16 +/- 8.3 y.o. First generation neuroleptics (OR 1.739 CI 95% 1.047-2.889), benzodiazepines (OR 2.357 CI 95% 1.5-3.702) and mood stabilizers (OR 1.889 CI 95% 1.091-3.270) were associated to falls when adjusted for age, sex and comorbidities. Polypharmacotherapy (> or = 4 drugs) was a risk factor for falls when associated to a risk medication in the daily regimen only (OR 2.157 CI 95% 1.447-3.217). What raised has to be taken into account in the prescriptive activities in a Long term Care Setting for elderly patients.

According to/After the conferences taken place on 30th October and on 18th December 2008, the company doctor functions have been enlarged: at present they include the duty of effectuating toxicological tests in order to find psychotropic and drug substances in some specific working classes, mainly in workers employed in public transports. Company doctors have the duty to report any possible positive matching in these workers'tests. From there, regulations in force are analyzed focusing to critical situations described in the complex and well articulated frame of reference.

Background: Sentinel node biopsy is a minimally invasive technique alternative to routine axillary dissection in breast cancer staging. This technique selects women with positive nodes who may benefit from axillary dissection, avoiding unnecessary operations in negative node biopsies., Aims: In this article we report a 5 year multi disciplinary experience in sentinel node biopsy involving the General Surgery Unit of Imola Hospital in collaboration with Radiologist, Pathologists and Specialists in Nuclear Medicine., Methods: From 2000 to 2004 the Authors treated 209 women performing 214 sentinel node biopsies (in 5 cases the tumor was bilateral). Sentinel node identification was undertaken by lymphoscintigraphy; in 15 cases we associated intradermal injection of blue dye. Lymph nodes were examined by at least 60 hematoxylin and eosin stained sections and when nodes found negative were further studied with immunohistochemical stains for cytokeratins., Results: Sentinel node identification rate was 99.1%. In 62 patients sentinel node was metastatic and in 17 such nodes micrometastases were detected. In 6 cases with single metastatic cells, axillary dissection was not perfomed, in accordance to current opinions. In 50 of 62 women with metastatic axillary nodes (80.6%) the sentinel node was the only metastatic one. Number of axillary dissections decreased of more than 70% in four years, from theoretical 214 to 62., Conclusion: Sentinel node biopsy is currently a validated technique and many breast cancer patients are spared a regional lymph node dissection without compromising local control and the accuracy of staging.

Vascular access (VA) for dialysis is defined as the 'Achilles heel', but also the 'Cinderella' of dialysis, indicating the poor consideration of the problem whether in the surgical environment, or in incomprehensible way in that nephrologic. It can only aspire to the definition 'Fundamental detail'. However, presupposed effective dialysis is a blood flow rate of 300-350 mL/min. Good VA must be easy to prepare, long lasting, free from complications, and aesthetically acceptable and economical. The arteriovenous fistula (AVF) of Cimino and Brescia, from 1966, represents the gold standard and the model of comparison for other systems, more technologically advanced. It must be programmed with an adapted margin (1-2 months) to allow maturation and access certainty for the first puncture, and never carried out sooner than 14 days from the operation. It is known from hemodynamic studies that the good functional flow of the new fistula can already regain 400-500 ml/min in the first week, with cardiological implications like the increase in cardiac throw, in ejection fraction and in the cardiac index. Health workers, patients and dialysis staff must follow a continuous educational program to protect the VA and avoid 'routine and absent-minded management', a basis for its premature failure. The nephrologist must take the responsibility upon himself not to carry out 'medical malpractice'. In the Dialysis Center of Mantova, the VA 'road map' previews all patients (young and old, affections from mono or pluropathology), first the fistula to the wrist, then the cephalic proximal. It follows the basilic vein transposition, the vascular graft to the arm or to the groin, as an alternative to peritoneal dialysis. The permanent central venous catheter (CVCp) is the last choice in patients with reduced life expectancy, heart failure, neoplastic patients with vascular patrimony destroyed by chemotherapy and ischemic lesions produced by the fistula. There were 180 afferent prevailing patients at the Mantova Dialysis Center . The natural fistula rate was 91%, grafts 7% and CVCps 2%. Between 2000 and 31 March 2004 we prepared 367 VAs. Average patient age was 65 yrs, range 20-90 yrs; 59% male and 41% female. Eighty-eight percent of operations were performed by the nephrologist (distal fistula, rescue and cephalic proximal) and 12% by the vascular surgeon (basilic vein transposition, graft in PTFE stretch to the arm and to the groin and permanent catheter in the jugular vein). The fistula with native veins was the better solution, the graft must be prepared after the exhaustion of natural possibilities, and the CVCp, for serious complications (inadequate flow and infections), must be the last alternative.

Many abdominal wall reconstruction techniques have generally failed to pay attention to a number of anatomical considerations concerning the continuity of the thoraco-lumboabdominal fascia that envelops the dorsal and ventral muscles. We have introduced a new surgical technique (round mesh) developed to improve the abdominal wall weakness or pathology (hernia, laparocele) with the aim of restoring the muscular synergy between the anterior and posterior trunk compartments, thus improving sacroiliac stability, posture, and standing effort endurance. One hundred patients of both sexes were enrolled in this investigation. All were affected by abdominal wall impairment, frank hernia or laparocele, and had been complaining of lumbar and sciatic pain for long periods without any definite intervertebral disk pathology. They underwent pre- and postoperative subjective and objective evaluation and insertion of a prefascial polypropylene mesh with a posterior martingale that passes across the spine and paravertebral muscles, ending in two wider rectangles that are criss-crossed ventrally and finally sutured to the iliopubic brim. All the patients improved either subjectively or objectively with the round mesh procedure. This new technique is particularly useful in cases of reduction or impairment of the recti abdominis, transverse and oblique muscles, because simple suture and plication of these muscles is no guarantee of long-term functional restoration.

Background: One of the biggest debates in modern cardiology regards the relative merit of primary percutaneous transluminal coronary angioplasty (PTCA) versus thrombolysis for the treatment of acute myocardial infarction with persistent ST-segment elevation. After the excellent results with primary PTCA in trials and meta-analyses, the next question is whether such results might be duplicated in "real world" conditions., Methods: Between January 1995 and April 2003, 1000 consecutive patients with acute myocardial infarction, out of 2272 (44%) with ST-segment elevation admitted to the coronary care unit at the Cardiology Department of the S. Anna Hospital, were treated with PTCA. Our Institution is a medium-high volume center, without on-site surgery. Usual clinical and interventional practice, adjunctive antithrombotic therapy and results are described in this paper., Results: Primary PTCA has been performed in 825 patients (75%) out of 1095 undergoing emergency angiography, "facilitated" in 140 (13%), rescue in 35 (3.2%). Eighty patients of the "facilitated" PTCA group had been pre-treated with tissue-type plasminogen activator 50 mg i.v. bolus, 50 with abciximab and 10 with reduced doses of fibrinolytic and abciximab. One hundred and seventy patients (16%) had been transferred to our Institution from community hospitals. Nine patients out of 1000 undergoing PTCA (0.9%) have been transferred immediately after the procedure (bail-out, failure) to perform urgent coronary artery bypass grafting. PTCA has been completed by stenting in 919 patients (92%). The median door-to-balloon time was 58 min (25th-75th percentile 49-71). The in-hospital total mortality rate was 4.9% (49 deaths): 5.3% (44 deaths) in the primary PTCA group, 2.1% (3 deaths) in the "facilitated" PTCA group (p = 0.042), and 5.7% (2 deaths) in the rescue PT-CA group. Early reinfarction rate was 1.5% (15 cases). The median time to hospital discharge was 10 days (25th-75th percentile 7-14)., Conclusions: Since 9 years, our practice in the treatment of acute myocardial infarction with persistent ST-segment elevation is going on extending the use of primary PTCA, integrating pharmacological and mechanical options in selected cases.