Serum Vascular Adhesion Protein-1 and Endothelial Dysfunction in Hepatic Cirrhosis: Searching for New Prognostic Markers.

Endothelial dysfunction plays a key role in the development of liver cirrhosis. Among the biomarkers of endothelial dysfunction, the soluble form of Vascular Adhesion Protein-1 (sVAP-1) is an unconventional and less known adhesion molecule endowed also with amine oxidase activity. The aim of this study was to explore and correlate the behavior of sVAP-1 with that of the soluble vascular cell adhesion molecule-1 (sVCAM-1) and intercellular adhesion molecule-1 (sICAM-1) and with the severity of liver cirrhosis. A cross-sectional study was carried out by enrolling 28 controls, 59 cirrhotic patients without hepatocellular carcinoma, and 56 patients with hepatocellular carcinoma (HCC), mainly caused by alcohol abuse. The levels of adhesion molecules and of the pro-inflammatory cytokines (IL-6 and TNF-αα) were determined by immunoassay and the enzymatic activity of sVAP-1 by a fluorometric assay. In non-diabetic patients without HCC, a specific behavior of sVAP-1 was highlighted. Differently from sVCAM-1, sICAM-1, and cytokines, the sVAP-1 level was significantly increased only in the early stage of disease, and then, it decreased in the last stage (866 ± 390 ng/mL vs. 545 ± 316 ng/mL, in Child-Pugh class A vs. C, respectively, p < 0.05). Bivariate analysis correlates sVAP-1 to sVCAM-1, in the absence of HCC (Spearman's rho = 0.403, p < 0.01). Multiple linear regression analysis revealed that sVCAM-1 appears to be a predictor of sVAP-1 (ß coefficient = 0.374, p = 0.021). In conclusion, in non-diabetic and non-HCC cirrhotic patients, sVAP-1 may be a potential prognostic biomarker that, together with sVCAM-1 and pro-inflammatory cytokines, may provide information on the progression of sinusoidal liver endothelium damage.

Systemic sclerosis sine scleroderma in children is more aggressive than in adults.

OBJECTIVES: To compare the clinical and laboratory features of pediatric systemic sclerosis sine scleroderma (ssJSSc) with adult-onset ssSSc. METHODS: Demographic, clinical and laboratory data of ssJSSc, retrospectively retrieved from our hospital medical records, case reports from the literature and from the PRES JSSc registry, were compared with the Padua cohort of adult patients with ssSSc. Patients were defined as having ssSSc if they never had skin involvement but all the following features: (I) Raynaud's Phenomenon (RP) and/or digital vasculopathy, (II) positive antinuclear antibodies (ANA), (III) internal organs involvement typical of scleroderma, (IV) no other defined connective tissue diseases. RESULTS: Eighteen juvenile and 38 adult-onset ssSSc patients, mean disease duration 5.8 and 9.7 years, respectively, entered the study. The frequency of females affected was significantly lower in ssJSSc (38.9% vs 89.5%, p < 0.0001). When compared to adults, ssJSSc displayed less SSc-specific capillaroscopy abnormalities (68.8% vs 94.7%, p = 0.02) while significantly higher vascular (digital pitting scars, ulcers 35.3% vs 10.5%, p = 0.042), respiratory (50.0% vs 23.7%, p = 0.02) and cardiac involvement (50.0% vs 2.6%, p < 0.0001). The outcome was significantly worse in ssJSSc as six patients (33%) died (n = 3) or reached an end-stage organ failure (n = 3) in comparison to only two deaths (5.3%) in the adult cohort. Anti-centromere antibodies were significantly lower in children (20.0% vs 68.4%, p = 0.001) while no difference was noted for other SSc-specific autoantibodies. CONCLUSION: Compared to adults where ssSSc generally has an indolent course, children present with aggressive disease that heralds a worse prognosis characterized by high cardiorespiratory morbidity and mortality.Key Indexing Terms: scleroderma, juvenile systemic sclerosis, outcome, heart, pulmonary arterial.

Is artificial intelligence ready to replace specialist doctors entirely? ENT specialists vs ChatGPT: 1-0, ball at the center.

PURPOSE: The purpose of this study is to evaluate ChatGPT's responses to Ear, Nose and Throat (ENT) clinical cases and compare them with the responses of ENT specialists. METHODS: We have hypothesized 10 scenarios, based on ENT daily experience, with the same primary symptom. We have constructed 20 clinical cases, 2 for each scenario. We described them to 3 ENT specialists and ChatGPT. The difficulty of the clinical cases was assessed by the 5 ENT authors of this article. The responses of ChatGPT were evaluated by the 5 ENT authors of this article for correctness and consistency with the responses of the 3 ENT experts. To verify the stability of ChatGPT's responses, we conducted the searches, always from the same account, for 5 consecutive days. RESULTS: Among the 20 cases, 8 were rated as low complexity, 6 as moderate complexity and 6 as high complexity. The overall mean correctness and consistency score of ChatGPT responses was 3.80 (SD 1.02) and 2.89 (SD 1.24), respectively. We did not find a statistically significant difference in the average ChatGPT correctness and coherence score according to case complexity. The total intraclass correlation coefficient (ICC) for the stability of the correctness and consistency of ChatGPT was 0.763 (95% confidence interval [CI] 0.553-0.895) and 0.837 (95% CI 0.689-0.927), respectively. CONCLUSIONS: Our results revealed the potential usefulness of ChatGPT in ENT diagnosis. The instability in responses and the inability to recognise certain clinical elements are its main limitations.

Causes of death in women with breast cancer: a risks and rates study on a population-based cohort.

Introduction: The increasing survival of patients with breast cancer has prompted the assessment of mortality due to all causes of death in these patients. We estimated the absolute risks of death from different causes, useful for health-care planning and clinical prediction, as well as cause-specific hazards, useful for hypothesis generation on etiology and risk factors. Materials and methods: Using data from population-based cancer registries we performed a retrospective study on a cohort of women diagnosed with primary breast cancer. We carried out a competing-cause analysis computing cumulative incidence functions (CIFs) and cause-specific hazards (CSHs) in the whole cohort, separately by age, stage and registry area. Results: The study cohort comprised 12,742 women followed up for six years. Breast cancer showed the highest CIF, 13.71%, and cardiovascular disease was the second leading cause of death with a CIF of 3.60%. The contribution of breast cancer deaths to the CIF for all causes varied widely by age class: 89.25% in women diagnosed at age <50 years, 72.94% in women diagnosed at age 50-69 and 48.25% in women diagnosed at age ≥70. Greater CIF variations were observed according to stage: the contribution of causes other than breast cancer to CIF for all causes was 73.4% in women with stage I disease, 42.9% in stage II-III and only 13.2% in stage IV. CSH computation revealed temporal variations: in women diagnosed at age ≥70 the CSH for breast cancer was equaled by that for cardiovascular disease and "other diseases" in the sixth year following diagnosis, and an early peak for breast cancer was identified in the first year following diagnosis. Among women aged 50-69 we identified an early peak for breast cancer followed by a further peak near the second year of follow-up. Comparison by geographic area highlighted conspicuous variations: the highest CIF for cardiovascular disease was more than 70% higher than the lowest, while for breast cancer the highest CIF doubled the lowest. Conclusion: The integrated interpretation of absolute risks and hazards suggests the need for multidisciplinary surveillance and prevention using community-based, holistic and well-coordinated survivorship care models.

Prognostic role of euthyroid sick syndrome in MIS-C: results from a single-center observational study.

Background: Euthyroid sick syndrome (ESS) is characterized by low serum levels of free triiodothyronine (fT3) with normal or low levels of thyroid stimulating hormone (TSH) and free thyroxine (fT4) and is reported in different acute clinical situations, such as sepsis, diabetic ketoacidosis and after cardiac surgery. Our aim was to evaluate the predicting role of ESS for disease severity in patients with Multisystem Inflammatory Syndrome in children (MIS-C). Methods: A single-centre observational study on consecutive patients with MIS-C. Before treatment clinical, and laboratory data were collected and, in a subset of patients, thyroid function tests were repeated 4 weeks later. Variables distribution was analyzed by Mann-Whitney U-test and correlations between different parameters were calculated by Spearman's Rho coefficient. Results: Forty-two patients were included and 36 (85.7%) presented ESS. fT3 values were significantly lower in patients requiring intensive care, a strong direct correlation was shown between fT3 and Hb, platelet count and ejection fraction values. A significant inverse correlation was retrieved between fT3 levels and C-reactive protein, brain natriuretic peptide, IL-2 soluble receptor and S-100 protein. Subjects with severe myocardial depression (EF < 45%) had lower fT3 values than subjects with higher EF. The thyroid function tests spontaneously normalized in all subjects who repeated measurement 4 weeks after admission. Conclusion: ESS is a frequent and transient condition in acute phase of MIS-C. A severe reduction of fT3 must be considered as important prognostic factor for severe disease course, with subsequent relevant clinical impact in the management of these patients.

Monoarticular juvenile idiopathic arthritis as a distinct clinical entity A proof-of-concept study.

BACKGROUND: Currently, monoarticular Juvenile Idiopathic Arthritis (monoJIA) is included in the ILAR classification as oligoarticular subtype although various aspects, from clinical practice, suggest it as a separate entity. OBJECTIVES: To describe the clinical characteristics of persistent monoJIA. METHODS: Patients with oligoJIA and with at least two years follow-up entered the study. Those with monoarticular onset and persistent monoarticular course were compared with those with oligoJIA. Variables considered were: sex, age at onset, presence of benign joint hypermobility (BJH), ANA, uveitis, therapy and outcome. Patients who had not undergone clinical follow-up for more than 12 months were contacted by structured telephone interview. RESULTS: Of 347 patients with oligoJIA, 196 with monoarticular onset entered the study and 118 (60.2%), identified as persistent monoJIA, were compared with 229 oligoJIA. The mean follow-up was 11.4 years. The switch from monoarticular onset to oligoarticular course of 78 patients (38.8%) occurred by the first three years from onset. In comparison with oligoJIA, the most significant features of monoJIA were later age at onset (6.1 vs. 4.7 years), lower female prevalence (70.3 vs. 83.4%), higher frequency of BJH (61.9 vs. 46.3%), lower frequency of uveitis (14.4 vs. 34.1%) and ANA+ (68.6 vs. 89.5%) and better long-term outcome. CONCLUSIONS: MonoJIA, defined as persistent arthritis of unknown origin of a single joint for at least three years, seems to be a separate clinical entity from oligoJIA. This evidence may be taken into consideration for its possible inclusion into the new classification criteria for JIA and open new therapeutic perspectives.

Strategic use of levofolinic acid for methotrexate-induced side effects in juvenile idiopathic arthritis: a prospective observational study.

OBJECTIVE: To evaluate the efficacy of levofolinic acid (LVF) administered 48 h before methotrexate (MTX) in reducing gastrointestinal side effects without interference with drug efficacy. METHODS: A prospective observational study was performed including patients with Juvenile Idiopathic Arthritis (JIA) reporting significant gastrointestinal discomfort after MTX despite taking a dose of LVF 48 h after MTX. Patients with anticipatory symptoms were excluded. A LVF supplemental dose was added 48 h before MTX and patients were followed every 3-4 months. At each visit data on gastrointestinal symptoms, disease activity (JADAS, ESR, CRP values) and treatment changes were collected. Friedman test for repeated measures analyzed differences between these variables over time. RESULTS: Twenty-one patients were recruited and followed for at least 12 months. All patients received MTX subcutaneously (mean 9.54 mg/m2) and LVF 48 h before and after MTX (mean 6.5 mg/dose), 7 received a biological agent too. Complete remission of gastrointestinal side effects was reported in 61.9% of study patients at first visit (T1) and increased over time (85.7%, 95.2%, 85.7% and 100% at T2, T3, T4, T5, respectively). MTX efficacy was maintained as showed by significant reduction of JADAS and CRP (p = 0.006 and 0.008) from T1 to T4 and it was withdrawn for remission in 7/21. CONCLUSIONS: LVF given 48 h before MTX significantly reduced gastrointestinal side effects and did not reduce drug's efficacy. Our results suggest that this strategy may improve compliance and quality of life in patients with JIA and other rheumatic diseases treated with MTX.

A Comprehensive Examination of Vegan Lifestyle in Italy.

The popularity of veganism and plant-based diets is rapidly increasing worldwide, including in Italy, where more individuals and families are adopting this lifestyle. However, medical and health professionals often lack the necessary knowledge and are skeptical about this diet despite the scientific evidence. It is important for them to provide support and expertise to those following this diet. The survey evaluated various aspects of the lifestyle of Italian vegans living in Italy and abroad, including food frequency, vitamin and mineral supplementation, relationship with medical and health professionals, and perceived difficulties in daily life. The emphasis was on potentially critical aspects for those following this dietary choice. A cross-sectional survey was conducted in Italy between March and April 2022. A questionnaire was distributed through social media platforms such as Instagram, Facebook, and Telegram, and 2180 Italian adults who follow a vegan diet completed it. The survey found that most of the vegan population surveyed were female, showed a greater sensitivity to ethical issues, were aware of the need for vitamin B12 supplementation, and followed healthy-eating guidelines. It is evident that despite the increasing popularity of plant-based diets, many medical and health professionals remain cautious and hesitant to recommend them.

Evaluating Measurement Properties of the Adapted Interprofessional Collaboration Scale through Rasch Analysis.

This study aimed to evaluate the validity and reliability of the German and Italian versions of the Interprofessional Collaboration Scale (IPC scale) by applying a Rasch analysis. Data were gathered from 1182 nurses participating in a cross-sectional study in northern Italy. The scale demonstrated good reliability (Cronbach's alpha = 0.92). Item polarity of all 13 items was positive, indicating good construct validity. However, revising one item would further improve the validity of the scale. Item stability was confirmed for work experience, workplace, age range, the language version, and gender. The analysis, applying a non-classical test theory, confirmed that the IPC scale is a valid and reliable instrument to measure interprofessional collaboration between nurses and physicians.

Adolescent gambling behavior: a gender oriented prevention strategy is required?

BACKGROUND: Studies published on gender-related differences in the gambling behavior of adolescents have focused mainly on psychological and social factors. The aim of this study was to develop separate risk factor models for male and female adolescents, considering the environmental, psychological, behavioral and socio-economic factors related to their gambling. METHODS: A survey was conducted through a questionnaire developed on a dedicated web site in 2014 on a representative sample of the Italian 15-19-years-old population, including 34,922 students attending 438 secondary schools. The SOGS-RA questionnaire was used to measure gambling behavior. To verify the risk factors associated with gambling a logistic regression stratified by gender was performed. RESULTS: In our representative sample of Italian adolescents nationwide, the prevalence of each level of gambling was higher in males than in females. The logistic regression stratified by gender found that for both genders, gambling was positively associated with internet surfing, playing sports, getting into a fight, having unprotected sex, pulling stunts, drinking alcohol at least once in the previous month, having not a satisfactory relationship with teachers, receiving pocket money from parents, spending each week much money and having someone in the family (father, sister/brother, other relatives) who gambles. On the other hand, having poor or average school marks, going to ED in the previous year, smoking at least once in the previous month, having dissatisfied with relationships with father and having a lower family income than their friends was only associated with gambling in boys. Having an accident or injury in the previous year and having a mother who gambled was associated only in girls with higher odd of at risk or problem gambling behavior. A low psychological distress is protective only in girls for risk of gambling. CONCLUSIONS: Understanding the gender-related differences, and how they emerge in younger people at the start of their gambling careers, can suggest how best to educate individuals, families and the community on the topic of gambling. Programs to prevent substance use and abuse should be multifaceted, and include efforts to prevent gambling with a gender perspective approach.

Methotrexate Monotherapy in Juvenile Idiopathic Arthritis Associated Uveitis: Myth or Reality?

OBJECTIVE: To evaluate the long-term efficacy of methotrexate (MTX) monotherapy in patients with juvenile idiopathic arthritis-associated uveitis (JIA-U). METHODS: We analyzed a cohort of patients with JIA-U treated with MTX monotherapy, divided into two groups depending on whether MTX was started before (on-MTX group) or after uveitis diagnosis (MTX-naïve group). The primary endpoint was the time between uveitis inactivity and first relapse. RESULTS: 84 patients entered the study. The median duration of remission on MTX monotherapy resulted 8.2 months. The on-MTX group showed a significant longer time interval between arthritis and uveitis onset and higher need for biologic agents (bDMARD). During follow-up, 40 patients (47.6%) needed bDMARD due to poor control of uveitis. Clinical remission off medication was achieved in 11.9% of patients, all belonging to the MTX-naïve group. CONCLUSIONS: MTX monotherapy, although effective in early stages of JIA-U, showed poor disease control in the long term.

Impact of the COVID-19 pandemic on head and neck cancer diagnosis: data from a single referral center, South Tyrol, northern Italy.

OBJECTIVE: The aim of the study was to evaluate the impact of the COVID-19 pandemic on new diagnoses of head and neck cancer (HNC) in South Tyrol, northern Italy in terms of the number of new diagnoses and worsening disease stage due to diagnostic delay. METHODS: Patients were divided into two groups: the control group with a first diagnosis of HNC in 10 months before the national lockdown (March 9th, 2020) and the study group with a first diagnosis of HNC in 10 months after lockdown. RESULTS: A total of 124 patients were included in the study. Before the spread of COVID-19, 79 new diagnoses of HNCs were registered, while in the period after the lockdown, 45 new cancers cases were diagnosed and the difference was statistically significant (p = 0.01278). Early clinical T-stage results showed 52 cases in the control group and 21 in the study group, again with a significant difference (p = 0.03711). Advanced T-stage results showed 27 cases in the control group and 24 in the study group. CONCLUSIONS: This study highlights the impact of the COVID-19 pandemic on HNCs, showing a statistically significant difference in the number of diagnoses before and after the lockdown which was related to the spread of the SARS-CoV-2 virus, and with a relevant decrease in early cT-staged HNCs.

Osteoperiostitis in children: proposal for a diagnostic algorithm.

Juvenile osteoperiostites (JOP) are a group of inflammatory bone diseases whose differential diagnosis is often difficult. The main conditions are acute osteomyelitis (AOM), chronic non-bacterial osteomyelitis (CNO) and the Goldbloom syndrome (GS). The study was aimed to develop an algorithm to enable an early diagnosis of JOP. Clinical records of patients with AOM, CNO and GS, followed at our Center over the past 10 years, were reviewed. Twelve additional patients with GS were selected from PubMed/MEDLINE literature search. Data collected included demographics, clinical manifestations, laboratory and instrumental investigations at disease onset. The association between categorical variables was investigated, and the segmentation of patients with different diagnoses was analyzed through a classification tree model (CTREE package) in order to build up a diagnostic algorithm. Ninety-two patients (33 CNO, 44 AOM, 15 GS) entered the study. Among 30 variables considered at onset, nine (age at onset, fever, weight loss, symmetry, focality, functional limitation, anemia, elevated ESR, CRP) resulted statistically significant in differentiating the three clinical entities from each other and were chosen to build up a decisional tree. Three variables, symmetry of bone involvement, presence of fever and age at disease onset, resulted significant to discriminate each of the three diseases from the others. The performance of the diagnostic algorithm was validated by comparing the diagnoses provided by the model with the real diagnoses and showed 85.9% accuracy.Conclusion: We propose a diagnostic algorithm, based on simple clinical data, which can help guide a prompt and appropriate diagnosis of JOP. What is Known: • Juvenile osteoperiostitis (JOP) are a group of inflammatory bone diseases followed by various pediatric specialists. • The distinction between these conditions is not easy as clinical and laboratory features often overlap. What is New: • We propose a diagnostic algorithm, based on clinical data of real patients, with high degree accuracy. • This instrument can help guide the prompt and appropriate diagnosis of JOP.

Methotrexate in Linear Scleroderma: Long-Term Efficacy in Fifty Children From a Single Pediatric Rheumatology Center.

OBJECTIVE: To study disease course and long-term outcome in children with linear scleroderma (SSc) treated with methotrexate (MTX) since diagnosis. METHODS: The present study was retrospective and cross-sectional and included consecutive children with linear SSc who were treated with MTX for >1 year and were followed up for at least 2 years. Disease course was analyzed by the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan-Meier analysis, comparing patients with linear SSc and those with other juvenile localized scleroderma (JLS) disease subtypes. Disease activity and damage were assessed by the Localized Scleroderma Cutaneous Assessment Tool and thermography. RESULTS: Fifty patients with a mean follow-up duration of 7.8 years and a mean MTX treatment duration of 3.1 years were included. Sixteen percent of patients did not respond to the first course of MTX, and 16% had at least 1 flare. Complete remission was observed in 18.2% of patients who were followed up for 2-5 years, in 80.0% of patients followed up for 10 years, and in 87.5% of patients followed up for >10 years. No significant difference in relapse-free survival between patients with linear SSc and in 17 patients with other JLS disease subtypes was observed. Tissue damage was mild in 42% of patients, moderate in 32%, and severe in 26%. The correlations between severity of tissue damage and linear SSc subtype, disease duration, relapses, and remission were not significant. The relationships between treatment duration and disease relapses (P < 0.05) and severity of tissue damage (P < 0.005) were significant. CONCLUSION: Most patients with linear SSc who are treated with MTX achieve complete and long-lasting remission. Overall aesthetic and functional sequelae are moderate, most likely because tissue damage is established early and treatment likely stabilizes the damage. Early diagnosis and MTX treatment, as well as long-term monitoring, are crucial to improve outcome and promptly identify flares.

Application of an adapted relationship scale for assessing the occurrence of six different relationships as perceived by seven health care professions in Northern Italy.

Interprofessional working relationships can influence the quality of collaborative practices, with consequences for patient safety outcomes. This article reports findings of an adapted relationship scale comprising six different relationship types, ranging from hostile to collegial, between seven health-care professions: physicians, nurses, dieticians, occupational therapists, physiotherapists, speech therapists, and psychologists. Survey data were gathered within amixed-method study aiming to explore the current status quo of interprofessional collaboration in aHealth Trust, located in Northern Italy. An online questionnaire was completed by 2,238 health professionals achieving aresponse rate of 44%. The working relationship element was answered by 1,897 respondents. The results of the survey are used as abasis for recommending strategies for advancing interprofessional collaboration in the Trust. Descriptive statistics were used to examine relationship-types, with frequency of occurrence considered. The non-parametric Mann Whitney and Kruskal Wallis tests were used to explore relationship differences among groups defined by sociodemographic variables. Participants reported overall positive relationships with other health professions. We noted variability in the occurrence of different relationship-types amongst the health professions. In particular, the six professions viewed their relationships with doctors more negatively than physicians who reported amore positive perception of their relationships with the six professions.

Non-human papilloma virus associated adenocarcinomas of the cervix uteri. Cytologic features and diagnostic agreement using whole slide digital cytology imaging.

BACKGROUND: Non-Human Papilloma Virus associated adenocarcinomas (NHPVAs) are uncommon tumors of the cervix uteri which often show a deceptive morphology. Therefore, their diagnostic assessment may be challenging. Slide digital cytology imaging may be an useful tool to improve cytological diagnostic accuracy. However, this novel technology has not been applied to NHPVAs associated cytologies yet. METHODS: The study included 31 whole slide digital cytology cases from 10 women with a proven histological diagnosis of NHPVA. As a control group, three further digital slides, from two women with a histological diagnosis of squamous intraepithelial lesion (SIL), were included. The digitally scanned cytological slides were revised to assess the concordance rate among three observers and to find out the most relevant NHPVA cytological criteria. RESULTS: Overall diagnostic agreement between observers was 67.60% (K = 0.50; P < 0.0001). At the consensus diagnosis 34 cases were re-classified as at least suspicious for glandular lesion (n = 24), SIL (n = 2) and negative (n = 8). The most relevant cytologic features for atypical glandular cells or adenocarcinoma at consensus were evident nucleoli, nuclear overlapping and atypical enlarged nuclei. CONCLUSIONS: The diagnosis of NHPVA in digital cytology is feasible using criteria which are also used in conventional microscopy. Our study shows a moderate agreement for the cytological diagnosis of NHPVAs using whole slide digital cytology approach. These results are discussed taking into account the most relevant differential diagnostic issues.

Mycophenolate mofetil for methotrexate-resistant juvenile localized scleroderma.

OBJECTIVES: To investigate safety and efficacy of MMF in patients with severe or MTX-refractory juvenile localized scleroderma. METHODS: Consecutive juvenile localized scleroderma patients undergoing systemic treatment were included in a retrospective longitudinal study. Patients treated with MMF because they were refractory or intolerant to MTX (MMF-group) were compared with responders to MTX (MTX-group). Disease activity was assessed by Localized Scleroderma Cutaneous Assessment Tool and thermography. Disease course was established on the number of relapses and treatment changes. Relapse-free survival was examined by Kaplan-Meier analysis. RESULTS: MMF and MTX groups included 22 and 47 patients, respectively. No significant difference in demographics, follow-up duration and treatment before diagnosis was observed between groups. The most represented clinical subtypes in the MMF-group were pansclerotic morphea and mixed subtype (P = 0.008 and P = 0.029, respectively), and linear scleroderma of the face in the MTX-group (P = 0.048). MMF was started because of MTX resistance (18 patients), relapse during MTX tapering/withdrawal (3 patients) and anaphylaxis to MTX (1 patient). After mean 9.4 years of follow-up, 90.9% of patients on MMF and 100% of those on MTX had inactive disease. No significant difference in relapse-free survival between the groups was found (P = 0.066, log-rank test), although MMF likely induced more persistent remission. MMF was well tolerated and combination of MMF and MTX did not increase its efficacy. CONCLUSION: The present study adds strong evidence on the efficacy and tolerance of MMF in severe and/or MTX-refractory juvenile localized scleroderma. Further controlled studies are needed to prove its efficacy as first line treatment.

Do Health Professionals Sufficiently Address Patients' Disposition Toward Changing Their Nutritional and Physical Activity Habits? Findings from a Pilot Study among People with Type 2 Diabetes in Northern Italy.

The aim of our study was to evaluate the disposition of individuals with type 2 diabetes mellitus (DM2) toward changing their nutritional and physical activity habits and associated factors-particularly their perceptions about interacting and communicating with four health professions. Working with a local patients' association, we invited 364 individuals with DM2, all at least 18 years old, to complete a paper-based survey with questions addressing their experiences of interacting and communicating with general practitioners, nurses, dieticians and diabetologists and about their readiness to change targeted habits, their health literacy and their clinical status. Of the 109 questionnaires collected, 100 were eligible for descriptive and inferential statistical analysis. Regarding nutritional habits, the highest percentage of participants were at the maintenance stage (26%), whereas regarding physical activity habits the highest percentage of participants were at the preparation stage (31%). Significant differences between the habits emerged for four of the five stages and for two psychological processes. The precontemplation stage was most associated with communication-related variables, whereas the maintenance stage was associated with higher health literacy for both habits, and waist-to-height ratio was associated with several stages of change and psychological processes for physical activity habits. Considering aggregated stages (i.e., active or passive stage), significant differences were observed for all psychological processes except readiness to change nutritional habits. Logistic regression analysis revealed associations of the active stage with higher self-efficacy and lower discrepancy processes for both habits. Nutritional habits were associated with normal HbA1c values and physical activity habits with high cholesterol. Understanding the combination of the stages of change and how they relate to psychological processes can afford meaningful insights into the potential internal and external communication skills of health professions and should be examined as possible elements for a patient evaluation model.

Intravenous ferric carboxymaltose is effective and safe in patients with inflammatory rheumatic diseases.

BACKGROUND: The aim of this study was to evaluate the efficacy and safety of ferric carboxymaltose in rheumatic patients with iron deficiency anaemia. MATERIALS AND METHODS: The study retrospectively evaluated a cohort of 34 patients with iron deficiency anaemia affected by inflammatory rheumatic diseases that are refractory or intolerant to oral iron therapy. They were treated with ferric carboxymaltose for a total of 56 cycles of treatment. The primary end point was to evaluate the increase of haemoglobin after ferric carboxymaltose treatment. The secondary end point was safety, including the occurrence of disease flare. RESULTS: Median age of the cohort was 60 years (range 31-91 years), with a male/female ratio of 4/30. Nine (26.5%) were affected by rheumatoid arthritis, 10 (29.4%) by spondyloarthritis, and 15 (44.1%) by other autoimmune connective tissue diseases. Median time from diagnosis was 7 years (IQR 2-12). At time of treatment (T0), median haemoglobin was 9.3 g/dL (IQR 8.2-10.3), transferrin saturation 6.2% (IQR 3.8-9.8), and ferritin 8.5 ng/mL (IQR 6.0-12.8). Median ferric carboxymaltose dose was 1,000 mg. At 6 weeks from T0, median haemoglobin was 12.3 g/dL (IQR 11.6-13.3), with a mean increase of 3.0 g/dL (p<0.01). Twelve (35.3%) patients needed re-treatment with ferric carboxymaltose for recurrence of iron deficiency anaemia. Four (4.3%) patients developed mild grade side effects. One suspected flare reaction has been observed. DISCUSSION: In patients affected by inflammatory rheumatic diseases, ferric carboxymaltose is safe and effective in correcting iron deficiency anaemia.

Infrared thermography in children: a reliable tool for differential diagnosis of peripheral microvascular dysfunction and Raynaud's phenomenon?

BACKGROUND: Infrared Thermography (IRT) has been used for over 30 years in the assessment of Raynaud Phenomenon (RP) and other peripheral microvascular dysfunctions in adults but, to date, very little experience is available on its use in children for this purpose. The first aim of the study was to assess reproducibility of thermographic examination after cold exposure by comparing inter-observer agreement in thermal imaging interpretation. The secondary aim was to evaluate whether IRT is reliable to diagnose and differentiate peripheral circulation disturbances in children. METHODS: Children with clinical diagnosis of primary Raynaud's phenomenon (PRP), secondary RP (SRP), acrocyanosis (AC) and age-matched controls underwent sequential measurements of skin temperature at distal interphalangeal (DIP) and metacarpophalangeal (MCP) joints with IRT at baseline and for 10 min after cold challenge test. Intraclass correlation coefficient (ICC) was calculated for inter-rater reliability in IRT interpretation, then temperature variations at MCP and DIP joints and the distal-dorsal difference (DDD) were analysed. RESULTS: Fourteen PRP, 16 SRP, 14 AC and 15 controls entered the study. ICC showed excellent agreement (> 0.93) for DIPs and MCPs in 192 measures for each subject. Patients with PRP, SRP and acrocyanosis showed significantly slower recovery at MCPs (p < 0.05) and at DIPs (p < 0.001) than controls. At baseline, higher temperature at DIPs and lower at MCPs was observed in PRP compared with SRP with significantly lower DDD (p < 0.001). Differently from AC, both PRP and SRP showed gain of temperature at DIPs and less at MCPs after cold challenge. PRP but not SRP patients returned to DIPs basal temperature by the end of re-warming time. Analysis of DDD confirmed that controls and PRP, SRP and AC patients significantly differed in fingers recovery pattern (p < 0.05). CONCLUSION: IRT appears reliable and reproducible in identifying children with peripheral microvascular disturbances. Our results show that IRT examination pointed out that PRP, SRP and AC patients present significant differences in basal extremities temperature and in re-warming pattern after cold challenge therefore IRT can be suggested as an objective tool for diagnosis and monitoring of disease.