bluuebunny/crossref_metadata_2025_split huggingface dataset:
Childhood-onset of primary Sjögren’s syndrome: phenotypic characterization at diagnosis of 158 children
Authors: Ramos-Casals Manuel , Acar-Denizli Nihan , Vissink Arjan , Brito-Zerón Pilar , Li Xiaomei , Carubbi Francesco , Priori Roberta , Toplak Nataša , Baldini Chiara , Faugier-Fuentes Enrique , Kruize Aike A , Mandl Thomas , Tomiita Minako , Gandolfo Saviana , Hashimoto Kunio , Hernandez-Molina Gabriela , Hofauer Benedikt , Mendieta-Zerón Samara , Rasmussen Astrid , Sandhya Pulukool , Sene Damien , Trevisani Virginia Fernandes Moça , Isenberg David , Sundberg Erik , Pasoto Sandra G , Sebastian Agata , Suzuki Yasunori , Retamozo Soledad , Xu Bei , Giacomelli Roberto , Gattamelata Angelica , Bizjak Masa , Bombardieri Stefano , Loor-Chavez Richard-Eduardo , Hinrichs Anneline , Olsson Peter , Bootsma Hendrika , Lieberman Scott M ,   and the Sjogren Big Data Consortium*, Kostov B , Horvath I -F , Szanto A , Seror R , Mariette X , Kvarnstrom M , Wahren-Herlenius M , Praprotnik S , Solans R , Nordmark G , Hammenfors D , Brun J G , Gheita T A , Atzeni F , Armagan B , Kilic L , Kalyoncu U , Nakamura T , Takagi Y , Consani S , Solorzano F Olivera 
Published: January 2021

Abstract

Objectives
To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS.

Methods
The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria.

Results
Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren’s syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease.

Conclusions
Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Serum uric acid and knee osteoarthritis in community residents without gout: a longitudinal study
Authors: Go Dong Jin , Kim Dong Hyun , Kim Jie Young , Guermazi Ali , Crema Michel Daoud , Hunter David J , Kim Hyun Ah 
Published: January 2021

Abstract

Objectives
Emerging evidence suggests a potential link between OA and gout; however, the association between serum uric acid (UA) itself and knee OA remains uncertain due to a lack of longitudinal studies. Here, we investigated the association between serum UA and knee OA according to cartilage status in elderly community residents without gout.

Methods
In this longitudinal study, participants without a history of gout were recruited from among the Korean cohort of the Hallym Aging Study (n = 296 for radiography study and n = 223 for MRI study). Weight-bearing knee radiographs and 1.5-T MRI scans, along with blood collection for analysis of serum UA, were performed at baseline and after 3 years. The severity and structural progression of knee OA were evaluated using the Kellgren–Lawrence grading system and the Whole-Organ MRI Score (WORMS) cartilage scoring method. Multivariable logistic regression analysis was conducted using generalized estimating equation (GEE) models.

Results
Serum UA levels were not associated with radiographic progression after adjusting for age, sex and BMI. There was no significant association between serum UA and tibiofemoral cartilage loss on MRI. However, baseline serum UA levels were negatively associated with patellofemoral cartilage loss over 3 years (adjusted odd ratio 0.70 per 1 mg/dl increase, 95% CI: 0.49, 0.98).

Conclusion
In this population-based cohort, serum UA was not a risk factor for knee OA progression. Further large-scale longitudinal studies in other populations are needed to validate the effects of UA on cartilage damage.
Focal vasculitic myositis as a primary manifestation of Behçet’s disease: a case series of 10 Korean patients in a locomotive medicine clinic
Authors: Kim Du Hwan , Kim Sun Woong , Yeo Seung Mi , Kang Min-Soo , Yoon Young Cheol , Sung Duk Hyun 
Published: January 2021

Abstract

Objectives
Muscle involvement in Behçet’s disease (BD) is rare, and several cases have been reported in the literature. Therefore, this study aimed to describe the clinical, laboratory and imaging findings in adult patients presenting with BD-associated myositis before the diagnosis of BD.

Methods
We retrospectively screened patients who visited a locomotive medicine clinic presenting with myalgia, local swelling, or tenderness of extremities without an established diagnosis of BD. We enrolled patients whose pain in the extremities was proven to be suggestive of focal vasculitic myositis and who were eventually diagnosed as having BD at the initial visit or during follow-up. We thoroughly reviewed the clinical, histological and imaging findings and treatment outcomes in patients who presented with focal vasculitic myositis as the primary manifestation of BD.

Results
Ten adult patients with focal vasculitic myositis as the primary manifestation of BD were enrolled. The lower and upper extremities were affected in eight and two patients, respectively. The affected lower extremities were the calf (n = 6) and thigh muscles (n = 2). The common findings of MRI included high signal intensity of the affected muscles and intermuscular fascia on fat-suppressed images, suggestive of myofascitis and oedematous changes in the subcutaneous layer. The results of skin or muscle biopsy were suggestive of vasculitis. All the patients were pain-free at the short-term follow-up (1–3 weeks) after oral steroid therapy.

Conclusion
Focal vasculitic myositis can be a primary manifestation of BD warranting medical attention. BD-associated myositis responds well to oral steroid therapy.
Hyperactivity and Seizure Induced by Tricresyl Phosphate Are Isomer Specific and Not Linked to Phenyl Valerate-Neuropathy Target Esterase Activity Inhibition in Zebrafish
Authors: Knoll-Gellida Anja , Dubrana Leslie E , Bourcier Laure M , Mercé Théo , Gruel Gaëlle , Soares Magalie , Babin Patrick J 
Published: January 2021

Abstract
Environmental exposure to tricresyl phosphate (TCP) may lead to severe neurotoxic effects, including organophosphate (OP)-induced delayed neuropathy. TCP has three symmetric isomers, distinguished by the methyl group position on the aromatic ring system. One of these isomers, tri-ortho-cresyl phosphate (ToCP), has been reported for years as a neuropathic OP, targeting neuropathic target esterase (NTE/PNPLA6), but its mode of toxic action had not been fully elucidated. Zebrafish eleuthero-embryo and larva were used to characterize the differential action of the TCP isomers. The symmetric isomers inhibited phenyl valerate (PV)-NTE enzymatic activity in vivo with different IC50, while no effect was observed on acetylcholinesterase activity. Moreover, the locomotor behavior was also affected by tri-para-cresyl phosphate and tri-meta-cresyl phosphate, only ToCP exposure led to locomotor hyperactivity lasting several hours, associated with defects in the postural control system and an impaired phototactic response, as revealed by the visual motor response test. The electric field pulse motor response test demonstrated that a seizure-like, multiple C-bend-spaghetti phenotype may be significantly induced by ToCP only, independently of any inhibition of PV-NTE activity. Eleuthero-embryos exposed to picrotoxin, a known gamma-aminobutyric acid type-A receptor inhibitor, exhibited similar adverse outcomes to ToCP exposure. Thus, our results demonstrated that the TCP mode of toxic action was isomer specific and not initially related to modulation of PV-NTE activity. Furthermore, it was suggested that the molecular events involved were linked to an impairment of the balance between excitation and inhibition in neuronal circuits.
Prolonged coma in scrub typhus: consider non-convulsive status epilepticus
Authors: Kalita Jayantee , Jadhav Mahesh S , Singh Varun K , Misra Usha K 
Published: January 2021

Abstract

Background
Scrub typhus is prevalent in the Tsutsugamushi belt and may manifest with meningoencephalitis and seizures. We report a patient with scrub typhus who had non-convulsive status epilepticus (NCSE).

Methods
A 50-y-old female with fever and altered sensorium for 5 d was diagnosed as scrub typhus based on serum IgM ELISA. She was on mechanical ventilation and received doxycycline and ceftriaxone, but did not improve until the third day of admission.

Results
An EEG revealed &gt;2.5 Hz generalised epileptiform discharges, which were suppressed by intravenous lorazepam suggesting NCSE. Following valproate and levetiracetam, she became conscious and had a full recovery.

Conclusion
A scrub typhus patient recovers even after prolonged NCSE.
Mechanotransduction in Wound Healing: From the Cellular and Molecular Level to the Clinic
Authors: Fu Siqi , Panayi Adriana , Fan Jincai , Mayer Horacio F. , Daya Mahendra , Khouri Roger K. , Gurtner Geoffrey C. , Ogawa Rei , Orgill Dennis P. 
Published: January 2021

ABSTRACT
Skin provides a critical protective barrier for humans that is often lost following burns, trauma, or resection. Traditionally, skin loss is treated with transfer of tissue from other areas of the body such as a skin graft or flap. Mechanical forces can provide powerful alternatives and adjuncts for skin replacement and scar modulation. This article first provides an overview of the various mechanical forces that affect fibroblasts, keratinocytes, endothelial cells, and adipocytes at the cellular and molecular level. This is followed by a review of the mechanical devices currently in clinical use that can substantially augment the restoration of skin integrity and reduce scarring. Methods described include tissue expanders, external volume expansion, negative-pressure wound therapy, and skin taping.

GENERAL PURPOSE
To review the various mechanical forces that affect fibroblasts, keratinocytes, endothelial cells, and adipocytes at the cellular and molecular level as well as scar-reducing mechanical devices currently in clinical use.

TARGET AUDIENCE
This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care.

LEARNING OBJECTIVES/OUTCOMES
After participating in this educational activity, the participant will:
1. Compare and contrast the responses of various types of cells to mechanical forces.
2. Identify the mechanical devices and techniques that can help restore skin integrity.
Mode of blood pressure monitoring and morbidity after noncardiac surgery
Authors: Abbott Tom E.F. , Howell Simon , Pearse Rupert M. , Ackland Gareth L. 
Published: January 2021

BACKGROUND
Control of blood pressure remains a key goal of peri-operative care, because hypotension is associated with adverse outcomes after surgery.

OBJECTIVES
We explored whether increased vigilance afforded by intra-arterial blood pressure monitoring may be associated with less morbidity after surgery.

DESIGN
A prospective observational cohort study.

SETTING
Four UK secondary care hospitals.

PATIENTS
A total of 4342 patients ≥45 years who underwent noncardiac surgery.

METHODS
We compared outcome of patients who received peri-operative intra-arterial blood pressure monitoring with those whose blood pressure was measured noninvasively.

OUTCOMES
The primary outcome was peri-operative myocardial injury (high-sensitivity troponin-T ≥ 15 ng l−1 within 72 h after surgery), compared between patients who received intra-arterial versus noninvasive blood pressure monitoring. Secondary outcomes were morbidity within 72 h of surgery (postoperative morbidity survey), and vasopressor and fluid therapy. Multivariable logistic regression analysis explored associations between morbidity and age, sex, location of postoperative care, mode of blood pressure/haemodynamic monitoring and Revised Cardiac Risk Index.

RESULTS
Intra-arterial monitoring was used in 1137/4342 (26.2%) patients. Myocardial injury occurred in 440/1137 (38.7%) patients with intra-arterial monitoring compared with 824/3205 (25.7%) with noninvasive monitoring [OR 1.82 (95% CI 1.58 to 2.11), P < 0.001]. Intra-arterial monitoring remained associated with myocardial injury when adjusted for potentially confounding variables [adjusted OR 1.56 (1.29 to 1.89), P < 0.001). The results were similar for planned ICU versus ward postoperative care.

CONCLUSIONS
Intra-arterial monitoring is associated with greater risk of morbidity after noncardiac surgery, after controlling for surgical and patient factors. These data provide useful insights into the design of a definitive monitoring trial.
Altered nociception in Alzheimer disease is associated with striatal-enriched protein tyrosine phosphatase signaling
Authors: Lee Zhung-Fu , Huang Tzu-Hsuan , Chen Shih-Pin , Cheng Irene Han-Juo 
Published: January 2021

Abstract
Alzheimer disease (AD) is the most common form of dementia, accounting for approximately 60% of cases. In addition to memory loss, changes in pain sensitivity are found in a substantial proportion of patients with AD. However, the mechanism of nociception deficits in AD is still unclear. Here, we hypothesize that the nociception abnormality in AD is due to the aberrant activation of striatal-enriched protein tyrosine phosphatase (STEP) signaling, which modulates proteins related to nociception transduction. Our results indicated that the transgenic mice carrying human amyloid precursor protein (APP) gene had lower sensitivity to mechanical and thermal stimulation than the wild-type group at the ages of 6, 9, and 12 months. These APP mice exhibited elevated STEP activity and decreased phosphorylation of proteins involved in nociception transduction in hippocampi. The pharmacological inhibition of STEP activity using TC-2153 further reversed nociception and cognitive deficits in the APP mice. Moreover, the phosphorylation of nociception-related proteins in the APP mice was also rescued after STEP inhibitor treatment, indicating the key role of STEP in nociception alteration. In summary, this study identifies a mechanism for the reduced nociceptive sensitivity in an AD mouse model that could serve as a therapeutic target to improve the quality of life for patients with AD.
For Infants With Fractures
Authors: Bataenjer Reto , Grotzer Michael , Seiler Michelle 
Published: January 2021

Objective
The objective of this study was to compare the frequency at which abuse is detected in institutions with mandatory skeletal surveys for infants with fractures to that in institutions with discretionary referral to child protection teams (CPTs).

Methods
A retrospective chart review of all infants with fractures diagnosed at an emergency department from 2014 to 2018 was conducted to analyze factors leading to a discretionary referral to CPTs and to identify the frequency of nonaccidental trauma.

Results
Seventy-two infants with a median age of 6 months were included in this study. The most frequent fracture site was the skull (73.6%), followed by fractures of the femur (12.5%) and the upper arm and forearm (each 4.2%). Discretionary referral to a CPT occurred in only 25% of cases, and abuse was detected in 2.8%.

Conclusions
The abuse detection rate in institutions with discretionary CPT referral is lower than that in institutions with mandatory skeletal surveys. Therefore, we recommend that in institutions with no mandatory skeletal surveys for infants with fractures, every infant with a fracture must be discussed with a CPT.
New Device Makes Tracheostomy Dressing Changes Easier and More Comfortable
Authors: Morris Linda L. , Kuncis Mary , Perleberg Debra , Kuczek Anna , Kashul Kristen , Zabel Douglas , Heinemann Allen W. , Semik Patrick 
Published: January 2021

Abstract

Purpose
This study compared the use of the tracheostomy dressing applicator (TDA) to the standard procedure for tracheostomy dressing changes.

Design
A prospective quasi-experimental study was performed.

Methods
Nineteen patients and 117 nurses answered a survey after changing the tracheostomy dressing with the TDA and using standard procedure.

Findings
Nurses rated the TDA easier to use in patients with average-sized necks and more favorably on observed discomfort in patients with average-sized and larger necks. Patients rated less discomfort with the TDA compared with the standard method of dressing change.

Conclusion
The TDA is an effective device to facilitate tracheostomy dressing changes, possibly improving compliance and decreasing skin complications.

Clinical Relevance
The TDA is a useful device that can improve efficiency of dressing changes with the potential to save time and improve outcomes.
Grief and Loss Among Veterans With Spinal Cord Injury: A Qualitative Study
Authors: Burkhart Lisa , Kale Ibuola O. , LaVela Sherri L. 
Published: January 2021

Abstract

Purpose
The objective of this study was to understand veteran perspectives of grief/loss associated with their spinal cord injury (SCI).

Design/Methods
Qualitative descriptive design using semistructured interviews with veterans with SCI (n = 15) was performed using interview questions informed by the literature. Analysis was conducted using a deductive/inductive approach.

Results
The 15 participants’ mean age was 64 years; six had paraplegia, eight had tetraplegia, one had unknown level of injury, six were ambulatory, and nine were not ambulatory. The grief/loss experience was individual and constant, consisting of seven phases: experiencing shock, releasing emotion, withdrawing, focusing on losses, problem solving, realizing strength and motivation, and accepting a new life. Veteran coping strategies used in each phase of the grief experience were also identified, as well as triggers that increased or decreased the grief/loss experience.

Conclusions/Clinical Relevance
A better understanding of veterans living with SCI grief experience, coping strategies, and triggering events can help healthcare providers support veterans with SCI.
Designing and Evaluating a Goal-Setting Tip Sheet for Stroke Family Caregiver Health
Authors: Bakas Tamilyn , Jones Holly , Israel Jahmeel , Kum Cleopatra , Miller Elaine L. 
Published: January 2021

Abstract

Purpose
The aim of the study was to design and evaluate a goal-setting tip sheet to improve stroke family caregiver health as part of the Telephone Assessment and Skill-Building Kit program.

Design
A multimethod design using open-ended questions and rating forms was used in this study.

Methods
In two studies, the goal-setting tip sheet was evaluated by caregivers (N = 12; N = 6), researchers (N = 10; N = 3), clinicians (N = 8; N = 4), and technology experts (N = 4; N = 4). Rigorous qualitative content analysis procedures were used with descriptive statistics for ratings.

Findings
Qualitative strengths and areas for improvement refined the goal-setting tip sheet. From 1 = strongly disagree to 5 = strongly agree, ratings averaged from 3.25 to 5.00 for strategies, accuracy, feasibility, and acceptability.

Conclusions
The goal-setting tip sheet was found to be accurate, feasible, and acceptable. Future research is needed to determine the impact on caregiver health.

Clinical Relevance
Stroke caregivers often neglect their own health; goal setting has the potential to improve caregiver health.
Unilateral Cleft Lip Repair Using the Chang Gung Technique
Authors: Tangco Ivy , Bhandari Kishor , Yao Chuan-Fong , Liu Amber Wei Chen , Chen Philip Kuo-Ting , Lu Ting-Chen 
Published: January 2021

Objective
This retrospective study analyzed the outcomes of patients undergoing complete or incomplete unilateral cleft lip repair using the Chang Gung technique. The goal was to compare the symmetry and change of the technique through the measurement of anthropometric points on digital photographs.

Methods
From 2010 to 2016, a total of 274 complete and incomplete cleft lip patients without other craniofacial deformities were included in the study. All included patients had a minimum 1-year follow-up with frontal view photographs taken. The vermilion area, lip width, vermilion height, lateral lip length, lip height, and Cupid's bow width of both cleft and noncleft sides were measured for all patients. The Cleft Lip Component Symmetry Index was used to determine the symmetry of the cleft and noncleft sides in both incomplete and complete cleft groups.

Results
A total of 152 complete and 122 incomplete cleft lip patients were included in the study. The mean Cleft Lip Component Symmetry Index values showed that the vermilion area, lip height, and Cupid's bow width were symmetric on both cleft and noncleft sides. Lip width and lateral lip length were noted to be shorter, whereas the vermilion height was thicker on the cleft side than on the noncleft side. Significant differences between the complete and incomplete cleft group measurements were found for lateral lip length and lip height, and complete cleft measurements were shorter than those for incomplete clefts. There were no significant differences in vermilion area, lip width, vermilion height, and Cupid's bow width.

Conclusions
The outcome analysis showed that vermilion height reduction and modifications could be made for both complete and incomplete groups. The complete cleft lip has been found to have a significantly shorter lateral lip length and lip height, reflecting a more severe anatomical soft tissue deficiency in this group. A long-term outcome anthropometric point study may further verify the results of this surgical technique.