bluuebunny/crossref_metadata_2025_split huggingface dataset:
75 Juvenile systemic sclerodermas: a case report
Authors: Remilaoui A , Hafirassou H , Ferhat A , Dahou-Makhloufi C 
Published: October 2022

Abstract

Background
Systemic scleroderma (SSc) is a rare generalized connective tissue disorder of poorly understood aetiology resulting in systemic fibrosis. It affects mainly women, usually in adulthood, but can occur at any age, although it is rare in children. Its prognosis is conditioned by cardiopulmonary involvement, which is the primary cause of mortality. Some innovative therapies have improved the management of this orphan disease.
We report an observation of a 14-year-old child with systemic scleroderma.

Observation
Child B.Z, 14 years old, without any particular history, consulted for a Raynaud's phenomenon that had appeared 4 months earlier. Clinical examination reveals localized skin sclerosis on the face and extremities of the upper limbs (Rodnan score = 11), associated with inflammatory-type arthralgias. Biologically: SV = 38 mmH1, CRP = 87 mg/l, FAN at 1/320 and positive Anti-Scl70. Periungual capillaroscopy showed megacapillaries with altered capillary architecture. The frontal chest X-ray did not reveal any interstitial syndrome, the cardiac ultrasound was without abnormalities (no PAH). The diagnosis of SSc was retained, the patient was put on calcium channel blockers (Diltiazem at a dose of 120 mg/d) associated with dietary rules and NSAIDs on demand with good improvement.

Conclusion
Very few cases of juvenile SSc have been reported in the literature. Its diagnosis should be based on the same ACR-EULAR 2013 criteria as those for adults. Its prognosis is conditioned by visceral damage which must be diagnosed and treated early.
87 Intracerebral inflammatory pseudotumor in Behçet’s disease: a pediatric case report
Authors: Hadef Djohra , Slimani Samy , Nezzar Mouna , Tahrour Issam 
Published: October 2022

Abstract

Introduction
Behçet's disease is a chronic, relapsing, multisystem vasculitis. The diagnosis is essentially clinical, due to the absence of specific biological criteria, which remains very difficult to establish in pediatric age because of often insidious or atypical disease onset. The association of Pseudo Inflammatory Non-Specific Tumors (PTINS) and Behcet's disease (BD) is exceptionally reported. We report the case of a 12-year-old boy with Behçet's disease who presented with an inflammatory pseudotumor during the course of his disease.

Methods and results
A 12-year-old boy, from Batna (Algeria), with no relevant past medical history, admitted in 2010 for the management of a venous thrombosis of the right lower limb without any obvious cause and which responded well to anticoagulant treatment.
A year later, the child was readmitted for polymorphic clinical signs made up of joint damage (inflammatory arthralgia of the large joints), digestive (glairo-bloody diarrhea) and ocular (decreased visual acuity) and skin and mucous membranes (oral-genital apthosis and erythema nodosum of the 4 limbs).
Lab work-up showed significant inflammatory syndrome with erythrocyte sedimentation rate (ESR) of 100 mm at the first h, C-reactive protein (CRP) up to 400 mg/l and thrombocytosis (700 000–900 000 elements/mm2). HLA B51 was negative.
The patient was treated with Colchicine, Steroids, and Aspirin with partial response. Subsequently, a treatment with azathioprine was started, with clinical improvement (despite occasional digestive crises) but with persistent biologic inflammation.
In 2012 the patient presented with a unilateral decrease in visual acuity. A CT scan was performed showing an intracranial tumor compressing the right optic nerve with all the criteria of a malignant tumor (rupture of the cortex, increase in volume on 2 successive CT scans). A transsphenoidal biopsy of the mass revealed a non-specific inflammation. Biological treatment with anti-TNF alpha (Infliximab) was associated with a spectacular tumor regression, allowing retrospectively the diagnosis of an inflammatory granulomatous lesion related to Behçet's disease rather than a malignant tumor. The outcomes were favorable with clinical and biological improvement for the first time since the onset of the disease.

Conclusion
Behcet is rare in the pediatric age group and it is difficult to diagnose. Nonspecific inflammatory pseudotumors can be associated with Behçet's disease and constitute a real diagnostic and therapeutic challenge.

Disclosure of Interest
None declared
88 Tubulointerstitial nephropathy and uveitis (TINU syndrome): a paediatric case report
Authors: Mouffouk Hassan , Hadef Djohra , Hezzil Widad , Bahamma Nacera , Guedjiba Asma , Rougui Mohamed Fateh 
Published: October 2022

Abstract

Introduction
TINU (Tubulo-interstitial Nephritis and Uveitis) or oculo-renal syndrome was first described by Dobrin et al. in 1975. &gt;100 cases (mostly paediatric) have been reported since then. It should be considered as diagnosis of elimination, as some iatrogenic, infectious, or systemic diseases may cause similar oculo-renal manifestations. There is no clear therapeutic consensus. However, the rapid initiation of high-dose corticosteroid therapy was associated with favorable outcome in the majority of published cases, as well as in our patient.

Case report
We report the case of an acute tubulointerstitial nephritis associated with uveitis in 11-year-old girl who presented with abdominal pain, vomiting and asthenia of one month. Laboratory tests revealed acute renal failure with proteinuria and aseptic leucocyturia. An inflammatory syndrome was found with erythrocyte sedimentation rate of 92 mm and inflammatory anaemia. Hypoproteinemia and polyclonal hypergamma-globulinaemia was found. No aetiology was found. The renal biopsy revealed a tubulo-nephritis with an interstitial inflammatory infiltrate of lymphoid cells and normal glomeruli. The patient was treated with high-dose of steroids (Methylprednisolone pulse followed by oral Prednisone 1 mg/kg/d with gradual weaning). We noted a progressive improvement of the renal function and the appearance of ocular pain and a decrease in visual acuity. The ophthalmological examination revealed bilateral uveitis. The diagnosis of TINU syndrome was confirmed and the patient received steroids with progressive resolution of the uveitis.

Conclusion
In the presence of any uveitis of unknown cause associated with disturbed renal function the diagnosis of TINU syndrome should be considered. This syndrome requires more precise diagnostic criteria and standardized management.

Disclosure of Interest
None declared
‘I turned in my man card’: a qualitative study of the experiences, coping styles and support needs of men with systemic sclerosis
Authors: Flurey Caroline A , Pauling John D , Saketkoo Lesley Ann , Denton Christopher P , Galdas Paul , Khanna Dinesh , Williams Adrian , Hughes Michael 
Published: October 2022

Abstract

Objectives
Men with SSc have a more severe clinical phenotype and reduced survival compared with women. No previous psychosocial studies have focused solely on men with SSc. This study aimed to explore experiences, coping strategies and support preferences of men with SSc.

Material and methods
An international qualitative research study comprising seven focus groups (three USA, four UK) of 25 men with SSc. Transcripts were analysed using reflexive thematic analysis.

Results
Three overarching themes and one underpinning theme were identified. In ‘impact of SSc on masculinity’, the men described an ‘impact on roles and activities’, reported ‘sex, intimacy, and erectile dysfunction’ as a salient issue that may be overlooked by clinicians, and experienced challenges to ‘masculine self-image’. ‘Dealing with SSc’ meant ‘always being prepared’, ‘becoming an expert’ and ‘balancing priorities’ in responsibilities, activities and symptom management. In ‘support for living with SSc’ men were selective in ‘(Not) talking about SSc’, would ‘(reluctantly) accept help’ and described ‘preferences for support’. Underpinning these experiences was ‘facing an uncertain future’ with some participants preferring not to focus on an unpredictable future, and others worrying about disease progression.

Conclusion
These novel data suggest SSc impacts male patients’ masculine identity and roles, and although they will accept practical help, they may mask the full emotional impact. Sex and intimacy are important overlooked issues with erectile dysfunction often not discussed at diagnosis. Further research should develop a self-management intervention for men with rheumatic diseases with a combination of disease-specific and common core components.
A scoping review of implementation of health-focused interventions in vulnerable populations
Authors: Daniels Sarah I , Cheng Hannah , Gray Caroline , Kim Bo , Stave Christopher Donald , Midboe Amanda M 
Published: October 2022

Abstract
Vulnerable populations face significant challenges in getting the healthcare they need. A growing body of implementation science literature has examined factors, including facilitators and barriers, relevant to accessing healthcare in these populations. The purpose of this scoping review was to identify themes relevant for improving implementation of healthcare practices and programs for vulnerable populations. This scoping review relied on the methodological framework set forth by Arksey and O’Malley, and the Consolidated Framework for Implementation Research (CFIR) to evaluate and structure our findings. A framework analytic approach was used to code studies. Of the five CFIR Domains, the Inner Setting and Outer Setting were the most frequently examined in the 81 studies included. Themes that were pertinent to each domain are as follows—Inner Setting: organizational culture, leadership engagement, and integration of the intervention; Outer Setting: networks, external policies, and patients’ needs and resources; Characteristics of the Individual: knowledge and beliefs about the intervention, self-efficacy, as well as stigma (i.e., other attributes); Intervention Characteristics: complexities with staffing, cost, and adaptations; and Process: staff and patient engagement, planning, and ongoing reflection and evaluation. Key themes, including barriers and facilitators, are highlighted here as relevant to implementation of practices for vulnerable populations. These findings can inform tailoring of implementation strategies and health policies for vulnerable populations, thereby supporting more equitable healthcare.
Multi-stakeholder perspectives on the implementation of a clinic-based food referral program for patients with chronic conditions: a qualitative examination
Authors: DePuccio Matthew J , Garner Jennifer A , Hefner Jennifer L , Coovert Nicolette , Clark Aaron , Walker Daniel M 
Published: October 2022

Abstract
Clinic-based food referral programs (FRPs) may help alleviate food insecurity and improve access to nutritious foods by systematically identifying and referring food-insecure primary care patients to community-based food resources. The purpose of this study was to examine
the barriers to and facilitators of implementation of an FRP offered to primary care patients who screen positive for food insecurity and have a qualifying chronic condition. we used a multi-stakeholder approach to conduct semi-structured interviews with healthcare providers and administrators from an academic medical center (AMC) (n = 20), representatives of a regional foodbank and its affiliated pantries (n = 11), and patients referred to the FRP (n = 20), during the initial phase of FRP implementation from April to September 2020. Interviews were audio-recorded, transcribed verbatim, and coded using a deductive dominant approach that allowed for the identification of emergent themes. Seven major themes emerged across the two domains of analysis: barriers to and facilitators of FRP implementation. Key barriers were (a) provider time constraints and competing demands; (b) inadequate physician feedback regarding patient use of the program; (c) patient transportation barriers; and (d) stigma associated with food pantry use. Key facilitators of implementation included (a) program champions; (b) screening and referral coordination; and (c) addressing food pantry-related stigma. This study identifies factors that deter and facilitate the implementation of an AMC-based FRP. Our findings highlight opportunities for healthcare and community-based organizations to refine and optimize FRP models toward the ultimate aim of advancing health equity for food-insecure patients.
Provider discussion of genetic counseling among high-risk Spanish-preferring Latina breast cancer survivors
Authors: Conley Claire C , Rivera Rivera Jessica N , Castro-Figueroa Eida M , Moreno Laura , Dutil Julie , García Jennifer D , Ricker Charité , Quinn Gwendolyn P , Soliman Hatem , Vadaparampil Susan T 
Published: October 2022

Abstract
Among high-risk breast cancer (BC) survivors, genetic counseling (GC) and genetic testing (GT) may inform cascade testing and risk management. Compared to non-Hispanic White BC survivors, Spanish-preferring Latina BC survivors are less likely to report discussing GC with a healthcare provider. However, few studies have examined Latinas’ experiences with GC/GT, particularly outside of the mainland USA. This study aimed to compare frequency of provider discussion of GC between Spanish-preferring Latina BC survivors living in Florida (FL) and Puerto Rico (PR). We conducted secondary data analysis of baseline assessments from a randomized pilot of an educational intervention for Spanish-preferring Latina BC survivors. Participants (N = 52) were GC/GT-naive, but met clinical criteria for GC/GT referral. Participants self-reported sociodemographic, clinical, and cultural variables, including previous provider discussion of GC. Descriptive statistics characterized frequency of GC discussion. Logistic regression examined the relationships between sociodemographic, clinical, and cultural characteristics and GC discussion. Only 31% of participants reported previous GC discussion. More participants from PR reported having GC discussions (43% vs. 21% in the mainland USA). In multivariable analyses, greater likelihood of GC discussion was associated with PR (vs. mainland USA) residence (odds ratio [OR] = 6.00, p = .03), older age at baseline (OR = 1.19, p = .04), and younger age at BC diagnosis (OR = 0.80, p = .03). Few high-risk Spanish-preferring Latina BC survivors in the mainland USA and PR had discussed GC with their providers. These results highlight a gap in the implementation of evidence-based genetics guidelines. Provider-directed interventions may be needed to increase uptake of GC/GT among Latina BC survivors.
Emerging research areas and contributions of NIH in violence research across the lifespan and throughout different settings
Authors: Burgdorf Caitlin E , Maholmes Valerie , O’Connor Stephen , Blachman-Demner Dara R 
Published: October 2022

Abstract
This commentary provides background for NIH’s interest in research designed to better understand the causes and consequences of violence and the development, evaluation, and implementation of preventive and treatment interventions to address the resulting trauma, injuries, and mortality from violence. The manuscript describes the context that contributed to a range of initiatives from the NIH focused on violence research, with a particular emphasis on firearms violence prevention research, and opportunities and gaps for future research.
It is time to Go All In: a call to action
Authors: Baskin Monica L 
Published: October 2022

Lay Summary
Even though there have been numerous advances in medicine and technology in the past few decades, the length and quality of life vary significantly based on a person’s race, ethnicity, gender identity, sexual orientation, disability status, geographic residence, and/or socioeconomic status. In fact, membership in one or more marginalized sociodemographic groups is particularly associated with worse healthcare access and health outcomes. Behavioral medicine is uniquely equipped to advance health equity through inclusive and innovative research and practice and exerted influence on policies and practices that contribute to health disparities.
Transitioning to Telehealth: The Multifaceted Impact of a Midcareer Transition in Practice
Authors: Stoynova Valeria , Eva Kevin W. 
Published: October 2022

Purpose
The medical education continuum is interrupted by several transition periods that can adversely affect performance. Most of what has been learned about such periods focuses upon movement from one stage of training to another and movement from training to practice. Established physicians, however, experience transitions throughout their careers at idiosyncratic times and with little assistance. Better understanding how physicians experience transition, where they struggle and how they adapt, would enable better support to be provided. We investigated the COVID-19-forced transition in clinical practice to virtual care, particularly its effect on physician roles and the ways that established physicians faced challenges they encountered when transitioning to virtual care.

Method
Ten semistructured interviews were conducted between November 2020 and February 2021 with physicians across different specialties and practice contexts who transitioned their practice to virtual care during the COVID-19 pandemic. Interview data were analyzed iteratively using “generic qualitative methodology” with constant comparison to identify themes in relation to observations.

Results
The transition to telehealth had implications that extended beyond the patient encounter, appearing to affect all aspects of the physician’s practice. To reflect that, CanMEDS was chosen as a useful organizing framework. The effects, captured in the theme “changes to the physician’s roles,” were nuanced, illustrated a consistent need to adapt to context, and could be framed positively or negatively or both. Additionally identified themes were labeled “physicians’ mental health” and “strategies to mitigate challenges.” These themes highlighted that, despite the effort involved and novelty of the situation, all participants found remarkably similar ways of grappling with the challenges faced.

Conclusions
While the basic roles of the physician do not appear to have changed through the transition to telehealth, our findings indicated that these roles were redefined in fundamental ways in response to changing societal needs.
Program Evaluation in Health Professions Education: An Innovative Approach Guided by Principles
Authors: Balmer Dorene F. , Anderson Hannah , West Daniel C. 
Published: October 2022

Problem
Program evaluation approaches that center the achievement of specific, measurable, achievable, realistic, and time-bound goals are common in health professions education (HPE) but can be challenging to articulate when evaluating emergent programs. Principles-focused evaluation is an alternative approach to program evaluation that centers on adherence to guiding principles, not achievement of goals. The authors describe their innovative application of principles-focused evaluation to an emergent HPE program.

Approach
The authors applied principles-focused evaluation to the Children’s Hospital of Philadelphia Medical Education Collaboratory, a works-in-progress program for HPE scholarship. In September 2019, the authors drafted 3 guiding principles. In May 2021, they used feedback from Collaboratory attendees to revise the guiding principles: Advance Excellence, Build Bridges, and Cultivate Learning.

Outcomes
In July 2021, the authors queried participants about the extent to which their experience with the Collaboratory adhered to the revised guiding principles. Twenty of the 38 Collaboratory participants (53%) responded to the survey. Regarding the guiding principle Advance Excellence, 9 respondents (45%) reported that the Collaboratory facilitated engagement in scholarly conversation only by a small extent, and 8 (40%) reported it facilitated professional growth only by a small extent. Although some respondents expressed positive regard for the high degree of rigor promoted by the Collaboratory, others felt discouraged because this degree of rigor seemed unachievable. Regarding the guiding principle Build Bridges, 19 (95%) reported the Collaboratory welcomed perspectives within the group. Regarding the guiding principle Cultivate Learning, 19 (95%) indicated the Collaboratory welcomed perspectives within the group and across disciplines, and garnered collaboration.

Next Steps
Next steps include improving adherence to the principle of Advancing Excellence, fostering a shared mental model of the Collaboratory’s guiding principles, and applying a principles-focused approach to the evaluation of multi-site HPE programs.
Local Recurrence Rates of Extramammary Paget Disease Are Lower After Mohs Micrographic Surgery Compared With Wide Local Excision: A Systematic Review and Meta-Analysis
Authors: Kim Grace Y. , Campbell Elliott H. , Brewer Jerry D. 
Published: October 2022

BACKGROUND
Extramammary Paget disease (EMPD) is a rare, slow growing neoplasm that presents most commonly in the anogenital region of older adults.

OBJECTIVE
To analyze the difference in local recurrence rates of EMPD in patients treated with wide local excision (WLE) versus Mohs micrographic surgery (MMS).

MATERIALS AND METHODS
A systematic review of the literature and meta-analysis were performed. Inclusion criteria were adults greater than 18 years of age with a diagnosis of EMPD who have undergone surgical intervention and had follow-up data. Studies were independently reviewed by 2 coinvestigators with discrepancies resolved by the principal investigator.

RESULTS
Twenty-seven studies met the inclusion criteria. Patients had a 2.67 times higher chance of local recurrence after WLE than MMS (95% confidence interval [CI]:1.47, 4.85; p = .001). Meta-analysis of single-arm studies revealed a 7.3% local recurrence rate after MMS (95% CI: 0.039, 0.107; p < .001) versus a 26.3% recurrence rate after WLE (95% CI: 0.149, 0.376; p < .001). After excluding recurrent tumors, the odds ratio for recurrence in WLE versus MMS was 2.3 (95% CI: 0.285, 18.43, p = .435).

CONCLUSION
There is a clinically and statistically increased risk of local recurrence of EMPD after WLE compared with MMS.