bluuebunny/crossref_metadata_2025_split huggingface dataset:
Patient Perspectives on Outcome Domains of Medication Adherence Trials in Inflammatory Arthritis: An International OMERACT Focus Group Study
Authors: Bekker Charlotte L. , Bossina Sacha , de Vera Mary A. , Bartlett Susan J. , de Wit Maarten , March Lyn , Shea Beverley , Evans Vicki , Richards Dawn , Tong Allison , Tugwell Peter , Tymms Kathleen , Scholte-Voshaar Marieke , Kelly Ayano , van den Bemt Bart J.F. 
Published: May 2021

ObjectiveTo describe the perspectives of patients with inflammatory arthritis (IA) on outcome domains of trials evaluating medication adherence interventions.MethodsAdult patients (≥ 18 yrs) with IA taking disease-modifying antirheumatic drugs from centers across Australia, Canada, and the Netherlands participated in 6 focus groups to discuss outcome domains that they consider important when participating in medication adherence trials. We analyzed the transcripts using inductive thematic analysis.ResultsOf the 38 participants, 23 (61%) had rheumatoid arthritis and 21 (55%) were female. The mean age was 57.3 ± (SD 15.0) years. Improved outcome domains that patients wanted from participating in an adherence trial were categorized into 5 types: medication adherence, adherence-related factors (supporting adherence; e.g., medication knowledge), pathophysiology (e.g., physical functioning), life impact (e.g., ability to work), and economic impact (e.g., productivity loss). Three overarching themes reflecting why these outcome domains matter to patients were identified: how taking medications could improve patients’ emotional and physical fitness to maintain their social function; how improving knowledge and confidence in self-management increases patients’ trust and motivation to take medications as agreed with minimal risk of harms; and how respect and reassurance, reflecting health care that values patients’ opinions and is sensitive to patients’ individual goals, could improve medication-taking behavior.ConclusionPatients value various outcome domains related to their overall well-being, confidence in medication use, and patient–healthcare provider relationships to be evaluated in future adherence trials.
Systematic Analysis of the Literature in Search of Defining Systemic Sclerosis Subsets
Authors: Nevskaya Tatiana , Pope Janet E. , Turk Matthew A. , Shu Jenny , Marquardt April , van den Hoogen Frank , Khanna Dinesh , Fransen Jaap , Matucci-Cerinic Marco , Baron Murray , Denton Christopher P. , Johnson Sindhu R. 
Published: May 2021

ObjectiveSystemic sclerosis (SSc) is a multisystem disease with heterogeneity in presentation and prognosis.An international collaboration to develop new SSc subset criteria is underway. Our objectives were to identify systems of SSc subset classification and synthesize novel concepts to inform development of new criteria.MethodsMedline, Cochrane MEDLINE, the Cumulative Index to Nursing and Allied Health Literature, EMBASE, and Web of Science were searched from their inceptions to December 2019 for studies related to SSc subclassification, limited to humans and without language or sample size restrictions.ResultsOf 5686 citations, 102 studies reported original data on SSc subsets. Subset classification systems relied on extent of skin involvement and/or SSc-specific autoantibodies (n = 61), nailfold capillary patterns (n = 29), and molecular, genomic, and cellular patterns (n = 12). While some systems of subset classification confer prognostic value for clinical phenotype, severity, and mortality, only subsetting by gene expression signatures in tissue samples has been associated with response to therapy.ConclusionSubsetting on extent of skin involvement remains important. Novel disease attributes including SSc-specific autoantibodies, nailfold capillary patterns, and tissue gene expression signatures have been proposed as innovative means of SSc subsetting.
Majeed Syndrome: Five Cases With Novel Mutations From Unrelated Families in India With a Review of Literature
Authors: Chavan Pallavi Pimpale , Aksentijevich Ivona , Daftary Aditya , Panwala Hiren , Khemani Chetna , Khan Archana , Khubchandani Raju 
Published: May 2021

ObjectiveMajeed syndrome (MJS) is an autosomal recessive, systemic autoinflammatory disease (SAID) caused by biallelic loss-of-function variants in theLPIN2gene. It is characterized by early-onset chronic recurrent multifocal osteomyelitis (CRMO), dyserythropoietic anemia, and neutrophilic dermatosis. We analyzed a cohort of uncharacterized Indian patients for pathogenic variants inLPIN2and other genes associated with SAIDs.MethodsWe performed whole-exome sequencing (WES) for 1 patient and next-generation sequencing (NGS) targeted gene panel for SAIDs in 3 patients. One patient was a referral from neurology after clinical exome sequencing identified a novel variant inLPIN2. We reviewed the literature for all published studies of mutation-positive MJS patients and have summarized their clinical features and disease-causing variants.ResultsWe describe the largest series of patients with MJS outside of the Middle East. All 5 patients are homozygous for novel, possibly pathogenic variants in theLPIN2gene. Two of these variants are missense substitutions, and 3 are predicted to alter transcript splicing and create a truncated protein. In addition to the classical features of CRMO and anemia, patients exhibited previously unreported features, including abdominal pain, recurrent diarrhea/ear discharge, and erythema nodosum.ConclusionPatients with MJS may present initially to different specialists, and thus it is important to create awareness in the medical community. In India, consanguinity is a common sociocultural factor in many ethnic communities and an abbreviated NGS gene panel for autoinflammatory diseases should include MJS. The unavailability of interleukin 1 inhibitors in some countries poses a treatment challenge.
Supporting Equity in Rheumatoid Arthritis Outcomes in Canada: Population-specific Factors in Patient-centered Care
Authors: Pianarosa Emilie , Hazlewood Glen S. , Thomas Megan , Hsiao Ralph , Barnabe Cheryl 
Published: May 2021

ObjectiveHealth equity considerations have not been incorporated into prior Canadian Rheumatology Association guidelines. Our objective was to identify the challenges and possible solutions to mitigate threats to health equity in rheumatoid arthritis (RA) care in Canada.MethodsA consultation process informed selection of priority populations, determined to be rural and remote, Indigenous, elderly with frailty, first-generation immigrant and refugee, low income and vulnerably housed, and diverse gender and sex populations. Semistructured interviews were completed with patients with lived experience, healthcare providers, and equity-oriented researchers. These interviews probed on population factors, initial and ongoing healthcare access issues, and therapeutic considerations influencing RA care. Known or proposed solutions to mitigate inequities during implementation of service models for the population group were requested. The research team used a phenomenological thematic analysis model and mapped the data into a logic model. Solutions applicable to several population groups were proposed.ResultsThirty-five interviews were completed to identify realities for each population in accessing RA care. Five themes emerged as primary solutions to population-based inequities, including actively improving the patient–practitioner relationship, increasing accessibility and coordination of care through alternative models of care, upholding autonomy in treatment selection while actively addressing logistical barriers and individualized therapy needs, collaborating with health supports valued by the patient, and being advocates for policy change and health system restructuring to ensure appropriate resource redistribution.ConclusionThe challenges for populations facing inequities in rheumatology care and promising solutions should inform guideline development and implementation, policy change, and health system restructuring.
Reducing Medication Errors on a Busy Tertiary Neonatal Intensive Care Unit Using a Quality Improvement Approach
Authors: Ibrahim Chokkiyil Ponnambath Hafis , Ofoegbu Bibian , Yahya Lamia , Catroon Khlood , Al Masri Duaa Adel , Saliba Adeline , Ghassa Lina 
Published: May 2021

Objectives:
Due to a high rate of reported medication errors on our neonatal intensive care unit (NICU), a quality improvement project was undertaken to tackle the problem in 2015. The steps included the development of a customized formulary, which was aligned to the electronic prescribing system and the smart pump libraries. The incidence of medication errors after serial introduction of the interventions was studied.

Methods:
Data on medication errors were extracted from the online incident reporting system. Additional data were derived from the pharmacy database. Trend on errors for the whole year was analyzed. Results: The medication error rate fell from 25.7/1000 to 6.7/1000 patient-days with the implementation of the project which was sustained even after the project implementation was complete. There was a statistically significant reduction in the rate of medication errors over the course of the year.

Conclusions:
Medication errors are common in neonatal care. A quality improvement approach with enhancements of existing systems significantly reduced the reported medication errors on the local NICU.
Experiences and Outcomes of Frenotomy in Children with Ankyloglossia in Multiple Tertiary Centers in Saudi Arabia
Authors: Bawazir Osama A. , Bawazir Abdullah O. , Bawazir Razan O. , Bawazir Faisal Ahmed , Halabi Nawaf F. 
Published: May 2021

Aims:
The aim of the study was to examine the experiences and outcomes of frenotomy treatment in neonates and children with ankyloglossia and to explore any subsequent complications.

Patients and Methods:
This is a retrospective, cross-sectional study of all pediatric cases of ankyloglossia that initially underwent frenotomy over a period of 5 years, in 2015–2020, across a number of departments in three tertiary centers in Jeddah, Saudi Arabia.

Results:
The most common indications were feeding difficulty in infants under 4 months and speech difficulty in children over 4 months. Following frenotomy, improvements were observed in symptomatic (96%) and asymptomatic (≈70%) children. Overall complications in the frenotomy procedure, including any minor bleeding, were found to be minimal.

Conclusion:
Ankyloglossia is a common disorder, but its effects on feeding and speaking are difficult to determine objectively, owing to the complexity of individual cases. Most of the babies in the present study improved following frenotomy, which is a simple, time-efficient, low-cost, and safe procedure.
Family-Centered Lactation Counseling and Breastfeeding in Preterm Infants upon Neonatal Intensive Care Discharge
Authors: Ongun Hakan , Demir Meltem 
Published: May 2021

Background:
There is great variability in breastfeeding implications upon neonatal intensive care unit discharge for preterm infants. Aims and Objectives: To examine the breastfeeding rates and the impact of lactation-counseling on the nutrition following hospital discharge in preterm infants.

Materials and Methods:
A three-page survey was applied to the families of infants of gestational age ≤34 weeks who were hospitalized between 2016-2018. Exclusion criteria were family reluctance to consent, foster-care placement, acquiring enteral feeding by orogastric tube/gastrostomy. The group categorization was based on lactation-counselling that involved both parents and elderly relatives who would assist the mother at neonatal care. Statistics were performed using SPSS-22 for covariates of neonatal intensive care interventions and post-discharge nutrition.

Results:
Exclusive breastfeeding was 49.2% at hospital-discharge and declined to 31.3% at six months. Early introduction of complementary foods was 51.1%. Total duration of breastfeeding was 7.38±3.98 months. Lactation-counseling prolonged breastfeeding duration to 8.47±3.87 months. The program presented the highest odds of extending breastfeeding interventions beyond six months (OR: 2.183, 95% CI: 1.354–3.520). It favored the outcomes by reducing the introduction of formulas and complementary foods before six months (P = 0.044, P = 0.018). The physical contribution of the father towards nutrition was the most significant benefit claimed by the participants. (71.6 versus 51.8%).

Conclusion:
Family-centered peer lactation-counseling by the medical staff and increasing awareness for infant nutrition are promising local strategies in reaching the goals of national nutrition policies guided by the international recommendations in preterm infants.
Outcome of Nonurinary Surgical Malformations Predicted by Fetal Abdominal Signs on Prenatal Ultrasound
Authors: Gupta Amit , Ezung Leonard J. , Kumar Manisha , Kumar Vipan , Choudhury Subhasis Roy , Yadav Partap S. 
Published: May 2021

Aim:
The aim of our study was to study the outcome of “nonurinary” surgical malformations predicted by fetal abdominal signs on prenatal ultrasound (US).

Methods:
This prospective observational study was done over a 3-year period.

Results:
Out of 66 cases, 15 different malformations were detected. The accuracy of prenatal US was 83.3%. There were four still births, two medical termination of pregnancy, and sixty live births (91%). Postnatal surgery was necessary in 35 neonates (62.5%) with postoperative survival of 71.4%. Sixteen neonates (26.7%), 7 with normal postnatal US and 9 asymptomatic lesions, were managed conservatively. Overall 1-year survival rate of fetuses was 62.1%. The most common malformations confirmed postnatally were duodenal atresia (n = 14, 23.3%), followed by gastroschisis (n = 9; 15%), esophageal atresia (n = 8; 13.3%), meconium peritonitis (n = 4; 6.7%), and ovarian cyst (n = 4; 6.7%). Prematurity (n = 34; 56.7%) and low birth weight (n = 44; 66.7%) had no significant effect on survival due to in utero transfer to tertiary care. Salient factors adversely affecting the survival were: (1) type of anomaly-omphalocele major and fetal ascites (FA) had no survivors, (2) postoperative complications, and (3) associated cardiac anomalies. It was also notable that, among fetal signs of meconium peritonitis, isolated intra-abdominal calcification had a good prognosis with 75% survival, whereas FA had no survivors.

Conclusions:
Results of this study will be helpful in realistic prognostication and postnatal management of these anomalies in the appropriate hospital setting.
Paracetamol for Closure of Patent Ductus Arteriosus in Preterm Babies Born Before 32-Week Gestational Age
Authors: Mahmoud Nagwa Sabry , Asklany Hany 
Published: May 2021

Background:
It remains unclear which treatment approach is most advantageous for closure of patent ductus arteriosus (PDA) in preterm infants. Despite the presence of previous trials and meta-analysis, a correct and univocal strategy for closure of PDA is still under debate. Till now, standard medical treatment for PDA closure has been indomethacin/ibuprofen, percutaneous transcatheter closure, or surgical ligation. Up to date, new strategies have been reported with paracetamol. The aim of this study was to present our experience with intravenous (IV) and oral paracetamol for closing PDA in preterm neonates born before 32-week gestational age (GA).

Patients and Methods:
We conducted a prospective study in a neonatal intensive care unit (NICU) from a tertiary hospital in Minia University; Egypt. Eighty-seven preterm neonates born before 32-week GA with hemodynamically significant PDA (hsPDA) were enrolled. They received 15 mg/kg/6 h for 5 days IV or oral paracetamol for ductal closure. Our NICU guidelines before the current study state that only hsPDA is treated with oral ibuprofen based on the decision of the neonatologist after discussion with pediatric cardiologist. The study aims to vary the practice to use IV or oral paracetamol instead of ibuprofen to avoid side effects of nonsteroidal drugs in preterm babies.

Results:
During the study period, 140 babies <32 weeks admitted to the NICU assessed for eligibility to the study, 87 of them received IV or oral paracetamol for the treatment of hsPDA for a total of 5 days. Forty-five babies received IV paracetamol, while 42 received oral form with closure rate of 82.22% and 80.95%, respectively. Successful closure on paracetamol was achieved in 71 of 87 babies (81.6%).

Conclusion:
This study concluded that paracetamol is not only an alternative treatment in closing PDA but also may be the treatment of choice in future.
Efficacy of Expressed Breast Milk Alone or in Combination with Paracetamol in Reducing Pain during ROP Screening
Authors: Naik Anjali , D'Lima Annely , Sreekumar Kavita , Silveira Maria Piedade 
Published: May 2021

Introduction:
Retinopathy of prematurity (ROP) has been widely acknowledged to be the primary cause of preventable childhood blindness in developing countries. However, the procedure for screening is extremely painful. In this study, we attempted to relieve the pain experienced by these babies using breast milk alone or in combination with oral paracetamol.

Materials and Methods:
A total of 120 preterm neonates were randomized into three groups: (Group A – control group = 40, Group B – breast milk group = 40, and Group C – oral paracetamol + breast milk = 40). Group B received 2 ml expressed breast milk (EBM) through a sterile syringe orally 2 min prior to procedure, Group C received syrup paracetamol, 15 mg/kg 30 min prior to procedure and EBM as in Group B. Pain experienced was measured by the premature infant pain profile (PIPP) score 20 s prior, during and 2 min after procedure. All procedures were video recorded. The video recorder and analyzer were both blinded to the intervention.

Results:
PIPP scores before the procedure (PIPP 1) in Groups A, B, and C were 4.09 ± 2.44, 3.25 ± 1.71, and 3.45 ± 2.20. Postprocedure PIPP score (PIPP 2) increased to 15.74 ± 2.42 in Group A, 15.44 ± 2.05 in Group B, and 15.83 ± 1.36 in Group C. There was no significant statistical difference in pain scores in the intervention groups (Groups B and C) compared to the control group (Group A),P= 0.724. PIPP scores recorded postprocedure in Groups A, B, and C were, 7.72 ± 3.43, 6.87 ± 3.46, and 7.85 ± 3.37 indicating residual pain.

Conclusion:
The procedure of ROP screening causes significant pain, with persistence of residual pain in premature neonates. However, there was no statistical difference in the pain scores noted in the intervention Groups B and C in comparison to the control Group A
An Observational Prospective Study to Compare Transcutaneous Bilirubin with Serum Bilirubin in Preterm Newborn Requiring Phototherapy
Authors: Sardar Syamal , Sarkar Nirmalya , Ghosh Moumita , Pal Somnath 
Published: May 2021

Background:
Transcutaneous bilirubin is used as a screening tool before starting phototherapy in preterm newborn. However, there is conflicting evidence regarding its utility after starting phototherapy. There is also doubt regarding the best site of measurement.

Objective:
The objective was to analyze the association between transcutaneous bilirubin and serum bilirubin in Indian preterm newborns at different sites before, during, and after phototherapy.

Methods:
This prospective observational study was conducted in a tertiary care neonatal unit of eastern India from May 2019 to April 2020.

Participants:
The study participants were 90 preterm newborns admitted for phototherapy within the first 7 days of life.

Intervention:
Transcutaneous bilirubin was assessed by Draeger JM-105 at the covered areas of the sternum and forehead and serum bilirubin was assessed by the Diazo method within 30 min of each other. Bilirubin assessment was done before starting phototherapy and then repeated every 12 h till 24 h postphototherapy.

Outcome:
To determine the correlation and agreement between serum bilirubin and transcutaneous bilirubin in preterm newborns before, during and after phototherapy.

Results:
Median gestational age and mean birth weight of the enrolled infants were 33.5 weeks and 1920 g, respectively. Transcutaneous bilirubin from sternum demonstrated good correlation with serum bilirubin before (r = 0.856), during (r = 0.723 at 24 h), and after phototherapy (r = 0.801 at 24 h postphototherapy). Measurements from the forehead showed better correlation with serum bilirubin compared to the sternum (r = 0.865 before, 0.732 during, 0.814 after phototherapy). At both sites, correlation worsened with phototherapy but improved gradually in the postphototherapy period. Bland–Altman analysis revealed wide upper limit of agreement between transcutaneous bilirubin and serum bilirubin at both sites and all points of time, suggesting a transcutaneous device significantly overestimated serum bilirubin.

Conclusion:
There was good correlation but poor agreement between transcutaneous bilirubin and serum bilirubin in Indian preterm newborns irrespective of timing and site of measurement.
Comparison between Suction Rectal Biopsy and Full-Thickness Rectal Biopsy in the Diagnosis of Hirschsprung's Disease
Authors: Redkar Rajeev , Chigicherla Swathi , Tewari Shruti , Sharma Rahul Deo 
Published: May 2021

Aim:
The aim of this study is to compare suction rectal biopsy (SRB) with full-thickness rectal biopsy (FTRB) in suspected cases of Hirschsprung's disease (HD).

Materials and Methods:
Between 2014 and 2018, we enrolled 41 consecutive children with suspected HD. We analyzed demographics, sex, age, clinical symptoms, radiological images, and biopsy reports. All the children had undergone X-ray of the abdomen and pelvis and contrast enema. All of them have undergone both SRB and FTRB, and their results were compared.

Results:
Out of 41 children, 26 were male and 15 were female. The children were aged from 5 days to 12 years. All of them presented with delayed passage of meconium, abdominal distension, and severe constipation. They all were on oral laxatives. The sensitivity and specificity of SRB are 80.95% and 90.00% when compared to FTRB which has 100% and 100%, respectively.

Conclusion:
FTRB is the gold standard test for diagnosing HD. SRB may be a good screening test in suspected HD cases. SRB is not as equal and effective as FTRB for diagnosing HD.