bluuebunny/crossref_metadata_2025_split huggingface dataset:
P045 Tofacitinib Treatment in Refractory Juvenile Dermatomyositis: A Case Series
Authors: Bowler Hailey A , Patel Aarat M 
Published: November 2021

Abstract

Background
Juvenile Dermatomyositis (JDM) is a systemic, autoimmune disease affecting the skin, blood vessels, and proximal skeletal muscles of juvenile individuals. A subset of JDM patients present with subclinical or resolved muscle involvement but continue to have skin disease. In these cases, first and second line treatments including glucocorticoids are sometimes insufficient for controlling the disease, necessitating escalation of treatment. Several recent studies have investigated the response of Tofacitinib, an oral Janus Kinase inhibitor approved for the treatment of rheumatoid arthritis, in DM patients and patients with inflammatory skin diseases

Objectives
Due to the reported ability of JAK inhibitors to suppress type 1 interferon (IFN) signalling, which is suspected to be upregulated in JDM, we evaluated the efficacy of treatment with Tofacitinib in four JDM patients.

Methods
Three patients with juvenile dermatomyositis without evidence of current muscle involvement and one patient with evidence of muscle involvement began treatment with Tofacitinib 5 mg daily after they had failed or had adverse effects to first- and second-line immunosuppressive agents. Their medical records were reviewed with improvement measured using the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) activity score. Throughout their treatment they were additionally monitored for improvement in markers of inflammation and muscle enzymes including CK, LDH, and aldolase. Patients were monitored for adverse effects to Tofacitinib.

Results
Three of four patients within the case series showed significant improvement of their CDASI scores over the initial first three months and two out of four patients showed continued improvement over the first six months. Patient #2 required use of Intravenous Immunoglobulin (IVIG) therapy in response to a flare despite continued use of Tofacitinib within the first six months. This patient also continues to require concomitant use of Hydroxychloroquine and IVIG however remains in skin remission. This patient has developed calcinosis despite having no active rash or muscle disease. TOF dose was increased to 5 mg bid for this patient. Patient #4 initially presented with mild skin disease. Other outcomes included significant improvement in arthritis with the addition of methotrexate. This patient also recieved a topical skin steroid injection by dermatology.

Conclusion
Tofacitinib is believed to play a role in the inhibition of IFN signalling pathways that are overactive in dermatomyositis. Three of the four patients within this retrospective study showed significant improvement of cutaneous disease with Tofacitinib use. Further controlled studies will show the utility of JAK inhibition in JDM.
P047 Musculoskeletal symptoms as first clinical presentation of juvenile-onset systemic lupus erythematosus: Experience from a military internal medicine department
Authors: Hannech E , Boussetta N , Guediche N H , Arfaoui B , Ajili F , Abdelhafidh N , Sayhi S , Louzir B 
Published: November 2021

Abstract

Background
Juvenile-onset systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder occurring before the age of sixteen. The clinical manifestations are variable and the management of the disease at this age may be different. The aim of our study was to investigate first clinical presentation of juvenile-onset SLE.

Methods
We conducted a retrospective study in a military internal medicine department. We accessed medical records of 103 patients with SLE diagnosed according to the revised 1997 American College of Rheumatology (ACR) criteria or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. Only patients with an age of lupus-onset &lt; the age of sixteen were included. Clinical data were collected and analyzed. The statistical analysis was performed using SPSS 23.0 software.

Result
There were eight juvenile-onsets SLE: two males and six females. The median age at the diagnosis was 13.7 + 1.9 years-old (11–16). First clinical presentations are shown in table 1.
Arthritis and hematological involvement tended to be associated (p = 0.07). Hypocomplementemia and positive anti-DNA antibodies were detected in 50% of cases.
All patients received Hydroxychloroquine and most of them combined corticosteroids (7cases).

Conclusion
Our study showed that musculoskeletal manifestations especially arthritis were frequent at the diagnosis of juvenile-onset SLE. According to the aggressive pharmacological treatment, a multidisciplinary approach should be required
P063 Consensus-based recommendations for treat to target management of immunoglobulin A vasculitis
Authors: Abu-Zaid M H , Tabra S A , Salah S , Lotfy H , Abdulhady H , Salah H , El Gaafary M , Farag Y , Eissa M , Maher S Esam , Radwan A , El-Shanawany Amira T , Medhat B M , El Mikkawy D , Mosa D M , El Deriny G , Mortada M , Osman N S , Fouad N A , Elkaraly N E , Mohamed S S , Hassan W A , Amer Y , Nasef S I , El Miedany Y 
Published: November 2021

Abstract

Background
IgAvasculitis (IgAV) is the commonest cause of vasculitis in childhood.
It is characterized by small vessel vasculitis of the skin, gastrointestinal tract, kidneys, joints, and, rarely, the lungs and the central nervous system. There is paucity of international guidelines for management of IgA V. the Objective is to develop guidelines specific for Egyptian children with IgA vasculitis.

Method
This study was carried out to achieve an Egyptian expert consensus on a treat-to-target management strategy for IgA vasculitis using Delphi technique. The preliminary scientific committee identified a total of 16 key clinical questions according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) approach. Delphi process was implemented (2-rounds) to reach a consensus.

Results
An online questionnaire were sent to expert panel (n = 26) who participated in the two rounds. At the end of round 2, a total of 20 recommendation items, categorized into 2 sections were obtained. Agreement with the recommendations (rank 7–9) ranged from 91.7–100%. Consensus was reached (i.e. ≥75% of respondents strongly agreed or agreed) on the wording of all the 20 clinical standards identified by the scientific committee. Algorithms for the management have been suggested.

Conclusion
This was an expert, consensus recommendations for the diagnosis and treatment of IgA V and IgA V nephritis, based on best available evidence and expert opinion.
O006. Clinical features of paediatric HIV arthropathy
Authors: Harrison Michael J , Brice Nicola , Slamang Waheba , Webb Kate , Scott Christiaan 
Published: November 2021

Abstract

Background
Advanced HIV infection is associated with an inflammatory arthritis, however few reports have described this disorder in children. This study aimed to describe the clinical features of HIV arthropathy in a case series of children in South Africa and compare these with features of JIA.

Methods
Retrospective data were collected from HIV-infected children with HIV arthropathy enrolled in a Paediatric Rheumatology clinic in Cape Town, South Africa. Data from a recently described, published cohort of children with JIA enrolled in the same clinic were included for comparison. Ethical approval was granted by the Human Research Ethics Committee of the University of Cape Town, with a waiver for consent.

Results
Eleven cases of HIV arthropathy were identified. Cases predominantly affected boys (8/11), and the median age of onset was 10.3 years (IQR 6.9–11.6). Most cases presented in the setting of advanced immunosuppression, with a median absolute CD4+ count of 389 cells/uL (IQR 322–449) and median CD4+ proportion of 19.5% (IQR 14.8–25.0) at presentation. The clinical presentation was variable, with both oligoarthritis (6/11) and polyarthritis (5/11) being prevalent. All cases exhibited large joint involvement, which was usually asymmetrical. In addition, four children had asymmetrical small joint involvement. Associated features included enthesitis (4/11) and dactylitis (1/11). The most consistent laboratory feature was elevated acute phase reactants, and typical ultrasonographic findings were joint effusions and synovial hypertrophy. JIA and HIV arthropathy presented at a similar age, with median age at HIV arthritis onset of 10.3 years (IQR 6.9–11.6) versus 9.25 years (IQR 4.5–12.3) at arthritis onset in the JIA subgroup. HIV arthropathy cases were predominantly male (M/F ratio 3.0), whereas JIA cases had an equal sex distribution (M/F ratio 0.9). Oligo-articular disease was more frequently described in children with HIV arthropathy (55%), compared to those with JIA (38%).

Conclusion
In this series, most cases of HIV arthropathy exhibited asymmetrical large joint oligoarthritis or polyarthritis, and presented in older boys with advanced immunosuppression. HIV arthropathy appears to present at a similar age to JIA, with a comparable pattern of joint involvement to oligo-articular and poly-articular JIA subtypes.
A Machine Learning Approach for Predicting Real-time Risk of Intraoperative Hypotension in Traumatic Brain Injury
Authors: Feld Shara I. , Hippe Daniel S. , Miljacic Ljubomir , Polissar Nayak L. , Newman Shu-Fang , Nair Bala G. , Vavilala Monica S. 
Published: November 2021

Background:
Traumatic brain injury (TBI) is a major cause of death and disability. Episodes of hypotension are associated with worse TBI outcomes. Our aim was to model the real-time risk of intraoperative hypotension in TBI patients, compare machine learning and traditional modeling techniques, and identify key contributory features from the patient monitor and medical record for the prediction of intraoperative hypotension.

Methods:
The data included neurosurgical procedures in 1005 TBI patients at an academic level 1 trauma center. The clinical event was intraoperative hypotension, defined as mean arterial pressure <65 mm Hg for 5 or more consecutive minutes. Two types of models were developed: one based on preoperative patient-level predictors and one based on intraoperative predictors measured per minute. For each of these models, we took 2 approaches to predict the occurrence of a hypotensive event: a logistic regression model and a gradient boosting tree model.

Results:
The area under the receiver operating characteristic curve for the intraoperative logistic regression model was 0.80 (95% confidence interval [CI]: 0.78-0.83), and for the gradient boosting model was 0.83 (95% CI: 0.81-0.85). The area under the precision-recall curve for the intraoperative logistic regression model was 0.16 (95% CI: 0.12-0.20), and for the gradient boosting model was 0.19 (95% CI: 0.14-0.24). Model performance based on preoperative predictors was poor. Features derived from the recent trend of mean arterial pressure emerged as dominantly predictive in both intraoperative models.

Conclusions:
This study developed a model for real-time prediction of intraoperative hypotension in TBI patients, which can use computationally efficient machine learning techniques and a streamlined feature-set derived from patient monitor data.
UVEAL EFFUSION ASSOCIATED WITH LOCALIZED SCLERODERMA BECAUSE OF SCLERAL FIBROSIS
Authors: Li Siyan , Liu Wenbo , Zhao Mingwei , Li Yuhui , Li Xiaoxin , Shi Xuan 
Published: November 2021

Background/Purpose:
To report a case of uveal effusion associated with localized scleroderma because of scleral collagen fibrosis. Partial-thickness sclerectomy treatment was successful in acquiring the resolution of the uveal effusion.

Methods:
Case report.

Results:
A 44-year-old Chinese woman with known localized scleroderma visited the retinal clinic complaining of insidious onset blurring of vision in both eyes for 8 months. The best-corrected visual acuity was 20/200. Ophthalmoscopy revealed apparent inferior bullous serous retinal detachments in the right eye. Optical coherence tomography showed subretinal fluid and folds of the retinal pigment epithelium layer in both eyes. B-scan ultrasonographic image of the right eye confirmed a 360-degree serous retinal detachment in the right eye accompanied with increased thickness of the ocular wall. Ultrasound biomicroscopy of the anterior segment detected a shallow ciliary body detachment in the right eye. Fluorescein angiography and indocyanine green angiography demonstrated the leopard-spot pattern in all phases. Partial-thickness sclerectomy treatment was successful in acquiring the resolution of the uveal effusion. Histopathologic examinations of the sclera flaps revealed scleral collagen fibrosis.

Conclusion:
This clinicopathologic report first describes a patient with localized scleroderma and scleral collagen fibrosis, resulting in uveal effusion that responded to partial-thickness sclerectomy.
Observation of Conjunctiva-Associated Lymphoid Tissue With In Vivo Confocal Microscopy in Healthy Patients and Patients With Meibomian Gland Dysfunction
Authors: Liu Yuting , Wang Yingbin , Jin Xin , Zhang Nan , Shi Yan , Zhu Rui , Wang Jingrao , Dong Yueyan , Zhang Hong 
Published: November 2021

Purpose:
The purpose of this study was to assess the distribution and morphological variation of conjunctiva-associated lymphoid tissue (CALT) in healthy human subjects and patients with meibomian gland dysfunction (MGD) using laserscanningin vivo confocal microscopy.

Methods:
A total of 34 healthy subjects and 32 patients with MGD were enrolled. All subjects underwent a conventional examination consisting of slitlamp biomicroscopy, tear film break-up time, and the Schirmer test. In vivo microscopy was applied to analyze the morphological changes in the diffuse lymphoid layer and lymphoid follicles in CALT. Conjunctival impression cytology (CIC) of samples of patients' palpebral conjunctiva and immunofluorescence staining of CD4 and CD8 antibodies were also performed to indicate the immune response status of CALT.

Results:
In the MGD group, the density of diffuse lymphocytes (P < 0.001), follicles (P < 0.001), and perifollicular lymphocytes was higher (P < 0.001) and the central reflection of the follicles was stronger (P < 0.001) than in the control group, while there was no difference in the follicle area (P = 0.758). Besides, diffuse lymphocyte density was correlated with telangiectasia, and follicular center reflection intensity was correlated with plugging. CIC immunofluorescence staining showed a higher percentage of CD4+ (P < 0.001) and CD8+ (P < 0.001) cells in the MGD group than in the control group.

Conclusions:
Using laser scanning in vivo confocal microscopy and CIC immunofluorescence staining, we observed the activation of CALT in patients with MGD, and some CALT-related parameters correlated with the lid margin findings of patients with MGD.
Marking DMEK Grafts Using Bandage Contact Lens Interface Technique: Doubling the Utilization During the Acute Shortage of Donor Corneas
Authors: Basak Samar K. , Basak Soham 
Published: November 2021

Purpose:
The purpose of this study was to describe a novel bandage contact lens (BCL) interface technique for marking the Descemet membrane endothelial keratoplasty (DMEK) graft so that a single donor cornea can be used effectively for 2 recipients during acute shortage.

Methods:
This was a retrospective comparative case series. In group A, 37 eyes underwent DMEK using a graft marked by the ‟BCL interface technique” that was compared with 49 conventional DMEK grafts marked through the stromal window (group B). In group A, a resized BCL with a central 3-mm hole with the concavity up was placed between the stroma and peeled-off DM. This BCL with DM was flipped for S-stamping on the DM side. Final trephination was performed on a second Teflon block. The remaining anterior lamellar tissues of group A were used on the same day for other keratoplasty procedures. Endothelial cell density (ECD) and endothelial cell loss between the 2 groups were compared after 3 and 6 months.

Results:
The ECD at 3 months in group A (n = 35) versus group B (n = 45) was 2228 ± 270/mm2 versus 2302 ± 254/mm2 (P = 0.48), and the ECD at 6 months (n = 23 and 22) was 2058 ± 324/mm2 versus 2118 ± 260/mm2 (P = 0.72). The corresponding endothelial cell loss was 23.3% ± 6.8% versus 20.3% ± 6.1% (P = 0.18) at 3 months and 29.1% ± 8.4% versus 26.7% ± 8.0% (P = 0.34) at 6 months. Among anterior tissues of group A, 17 (45.9%) were used for deep anterior lamellar keratoplasty, 18 (48.6%) were used for larger therapeutic and tectonic grafts, and 2 were used as keratoprosthesis carriers. Donor detachment rate (8.6% vs. 8.9%) was similar in both groups without primary graft failures.

Conclusions:
The BCL interface technique is a simple and safe technique for stamping DMEK grafts. Anterior corneal tissues can be used for additional keratoplasties during donor shortage.
Facilitators and Barriers to Glaucoma Medication Adherence
Authors: Gatwood Justin , Brooks Callee , Meacham Ryan , Abou-Rahma Joseph , Cernasev Alina , Brown Eric , Kuchtey Rachel W. 
Published: November 2021

Précis:
Patient education and dosing self-efficacy are important factors related to ongoing adherence to glaucoma topical treatment, and patients view their disease management as a shared effort with their provider.

Purpose:
Glaucoma affects nearly 3 million Americans, and medication adherence has been reported to be as low as 20% in this patient population; however, key limitations to our understanding of this behavior in adults with glaucoma exist.

Patients and Methods:
This research used an electronic survey including validated concepts related to topical medication use and an in-person interview to investigate the influencers of and solutions for challenges to medication adherence in adults with glaucoma. Patient eligibility was determined upon arrival to a regularly-scheduled visit to the Vanderbilt Eye Institute, during which they were asked for consent to complete the survey. Responses were captured by tablet and assessed using descriptive and inferential statistics. The primary focus was instrument correlations with the Adherence to Refills and Medications Scale score and were run between Adherence to Refills and Medications Scale, and the totaled score for each individual questionnaire as well as individual items. Recorded interviews were thematically assessed by multiple study team members.

Results:
Survey results of adults with glaucoma suggested that self-efficacy, forgetfulness, fear of side effects, and dosing ability were all related to self-reported medication adherence. Despite most having glaucoma for several years, discrepancies in disease knowledge were observed. Patient interviews uncovered 3 overall themes related to glaucoma treatment: (1) glaucoma management as a shared responsibility; (2) the importance of patient education; and (3) specific adherence facilitators and barriers.

Conclusion:
Glaucoma medication adherence interventions may benefit from focusing on developing patient medication-taking self-efficacy, disease-related education, and engagement with their provider.
Pelvic Abscess by Fusarium fujikuroi in an Immunocompetent Woman
Authors: Aggarwal Abhimanyu , DelPilar-Morales Esteban 
Published: November 2021

Abstract
Invasive Fusarium spp. infections are commonly seen as opportunistic infections, predominantly in immunocompromised patients. However, on very rare occasions, it has been reported to cause invasive diseases in immunocompetent patients, mostly after a breach in the normal physical barrier. We present the case of a woman who after delivery by cesarean section, developed pelvic abscess that was communicating with the endometrial cavity, culture noted positive for Escherichia coli and Fusarium fujikuroi. To our knowledge, this is also the first ever reported case of pelvic abscess by Fusarium spp. Follow-up imaging showed complete resolution of the abscess after treatment. Here, we present a review of all invasive Fusarium spp. infections in immunocompetent patients, except skin and soft tissue infections, reported in the literature to date.
Prevalence of Adverse Health Behaviors and Conditions Among Maritime Workers, BRFSS 2014 to 2018, 38 States
Authors: Evoy Richard , Case Samantha 
Published: November 2021

Objective:
To analyze health behaviors and conditions among maritime workers using Behavioral Risk Factor Surveillance System (BRSS).

Methods:
BRFSS data from 2014 to 2018 were used to calculate weighted prevalence estimates and adjusted prevalence ratios (aPRs) for 10 health behaviors and conditions. Logistic regression was used to compare aPRs between maritime workers and all other US workers.

Results:
Compared with other workers, maritime workers had higher weighted prevalence estimates for six of 10 health behaviors and conditions: binge drinking, smoking, obese/overweight, diabetes, cancer, and chronic obstructive pulmonary disease. Maritime workers had significantly higher aPRs for binge drinking (aPR = 1.28) and smoking cigarettes (aPR = 1.39) compared with all other US workers.

Conclusions:
This study uses BRFSS data to estimate the prevalence of adverse health conditions across maritime industries. This study can serve as the foundation for additional follow-on research.
Sonographic Screening of Distance Runners for the Development of Future Achilles and Patellar Tendon Pain
Authors: Cushman Daniel M. , Petrin Ziva , Cummings Keith , Eby Sarah F. , English Joy , Teramoto Masaru 
Published: November 2021

Objective:
The 2 primary aims of this study were to identify ultrasonographic tendon abnormalities in asymptomatic runners and to examine the likelihood of developing pain in runners with ultrasound abnormalities compared with those without abnormalities.

Design:
Longitudinal, prospective cohort study.

Setting:
2019 Salt Lake City Marathon.

Participants:
Recreational half-marathon and full-marathon runners.

Assessment of Risk Factors:
The Achilles and patellar tendons of asymptomatic runners were examined with ultrasound imaging before a running event. Runners were monitored for self-reported outcomes of pain in the examined tendons at 1, 3, 6, and 12 months after the event.

Main Outcome Measures:
Development of pain based on the presence of asymptomatic tendon abnormalities.

Results:
One hundred thirty-eight runners (36.2 ± 12.0 years, 49.3% men, and 31.2% full-marathon runners) were included. Ultrasound abnormalities of the Achilles and patellar tendons were identified in 24.6% and 39.1% of the runners before the race, respectively. Ultrasound abnormalities were significantly associated with approximately a 3-fold increase [hazard ratio (HR) = 2.55, P = 0.004] in the hazard of developing pain in the Achilles tendon and patellar tendon (HR = 1.67, P = 0.042) over the year after the race. Positive and negative predictive values of developing pain over the year were 34.1% and 87.2%, respectively, for abnormal findings in the Achilles tendon, and 22.9% and 85.0%, respectively, for the patellar tendon.

Conclusions:
The presence of ultrasonographic abnormalities is associated with increased development of pain in the Achilles and patellar tendons within 1 year of a marathon or half marathon.