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

OBJECTIVES: To compare the clinical and laboratory features of paediatric SSc 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 Pediatric Rheumatology European Society 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) RP and/or digital vasculopathy, (ii) positive ANA, (iii) internal organs involvement typical of scleroderma and (iv) no other defined CTD. 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 with adults, ssJSSc displayed fewer SSc-specific capillaroscopy abnormalities (68.8% vs 94.7%, P = 0.02) while having 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 (50.0% vs 2.6%, P < 0.0001) involvement. 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 with only two deaths (5.3%) in the adult cohort. ACA 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 with 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.

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.

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.

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.

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.

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.

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.

Perceptions of collaborative relationships between seven different health care professions in Northern Italy.

This article presents quantitative findings from a mixed method study that aimed to explore the status quo of interprofessional collaboration (IPC) in a Health Trust, located in a trilingual region in Northern Italy. The survey targeted seven health professions (physicians, nurses, dieticians, occupational therapists, physiotherapists, speech therapists, and psychologists). The survey was distributed online to more than 5,000 health professionals and completed by 2,238. This paper presents results on the frequency of collaboration as well as data from a multiple-group measurement scale for assessing IPC. Descriptive statistics were calculated for continuous variables while categorical data were analysed as counts and percentages. Pearson's Chi-square test and Fisher's exact test were calculated while Mann Whitney and Kruskal Wallis tests were applied to analyse statistical differences in IPC between groups according to sociodemographic variables. In general, our survey data showed that participants, perceived IPC in a positive way, even if the analysis indicated heterogeneity in the level of collaboration expressed. We also found that not all professions had an opportunity to collaborate with others. In addition, we found evidence to suggest that the way health care is organised impacted on perceptions of collaboration between the health professionals in this study. This study provides an initial insight into the perceived levels of IPC within a North Italian context. As such, it offers an account of the strengths and weaknesses of IPC from seven different professional groups based in this region of Europe.

[Types and frequencies of complications associated with midline catheters and PICCs in a South Tyrolean district hospital: a retrospective cohort study]. / Arten und Häufigkeiten von Komplikationen bei Midline-Kathetern und PICCs in einem Südtiroler Bezirkskrankenhaus ­ eine retrospektive Kohortenstudie.

Types and frequencies of complications associated with midline catheters and PICCs in a South Tyrolean district hospital: a retrospective cohort study Abstract. BACKGROUND: Specialized registered nurses play a key role in the insertion and management of peripherally inserted central catheters (PICCs) and midline catheters in Anglo-Saxon countries. From the German-speaking area no data on the use of PICCs and midline catheters are available. AIM: The aim of this study was to describe the types and frequencies of complications of PICCs and midline catheters which were inserted by specialized registered nurses in a South Tyrolean district hospital. METHODS: We performed a retrospective cohort study of PICCs and midline catheters inserted between 2013 and 2015 in one surgical unit in the district hospital Bruneck (South Tyrol / Italy). RESULTS: Data from 900 catheters (421 midline catheters and 479 PICCs) inserted in 686 adult patients (404 women, 282 men) were analysed. The cumulative incidence was 29.2 % complications for midline catheters (incidence rate: 13 complications / 1000 catheter days) and 16.0 % for PICCs (incidence rate: 3 complications / 1000 catheter days). The most frequent complication was the removal of the catheter by the patients (PICCs: 6.7 %, midline catheters: 15.7 %). Other less frequent complications were mechanical complications, occlusions, infections and thromboses. CONCLUSIONS: This study in one district hospital revealed similar types and frequencies of complications as previous international studies. Specialized and clinically competent nurses in German-speaking countries could develop advanced roles in the insertion and management of PICCs and midline catheters.

A multiple-group measurement scale for interprofessional collaboration: Adaptation and validation into Italian and German languages.

This article presents a study that aimed to validate a translation of a multiple-group measurement scale for interprofessional collaboration (IPC). We used survey data gathered over a three month period as part of a mixed methods study that explored the nature of IPC in Northern Italy. Following a translation from English into Italian and German the survey was distributed online to over 5,000 health professionals (dieticians, nurses, occupational therapists, physicians, physiotherapists, speech therapists and psychologists) based in one regional health trust. In total, 2,238 different health professions completed the survey. Based on the original scale, three principal components were extracted and confirmed as relevant factors for IPC (communication, accommodation and isolation). A confirmatory analysis (3-factor model) was applied to the data of physicians and nurses by language group. In conclusion, the validation of the German and Italian IPC scale has provided an instrument of acceptable reliability and validity for the assessment of IPC involving physicians and nurses.