bluuebunny/crossref_metadata_2025_split huggingface dataset:
A rare disease with many faces: a multicentre registry of IgG4-related disease in children
Authors: Kaya Akca Ummusen , Kose Hulya , Kurt Tuba , Ulu Kadir , Guliyeva Vafa , Kılbas Gulsah , Arslanoglu Ceyda , Yildirim Deniz Gezgin , Demir Selcan , Sahin Sezgin , Kısaarslan Aysenur Pac , Kasap Demir Belde , Sonmez Hafize Emine , Koker Oya , Yardimci Gozde Kubra , Ekici Mustafa , Kilic Sara Sebnem , Celikel Acar Banu , Sozeri Betul , Aktay Ayaz Nuray , Yuksel Selcuk , Bakkaloglu Sevcan Azime , Kasapcopur Ozgur , Saglam Emine Arzu , Karadag Omer , Ozen Seza , Bilginer Yelda 
Published: September 2024

Abstract

Objectives
We aimed to report the characteristics of paediatric IgG4-related disease (IgG4-RD) through a multicentre registry, to assess disease clusters, and to evaluate the performances of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria in this cohort.

Methods
Data of IgG4-RD patients in 13 paediatric rheumatology centres were recorded to a web-based registration system. The diagnosis of IgG4-RD was made according to the 2011 comprehensive diagnostic criteria.

Results
Thirty-five children (19 females and 16 males) with IgG4-RD were enrolled. The median age at diagnosis was 13.3 (25p-75p; 9.9–15.2) years. The most common organ involvement was the eye (n = 21, 60%), followed by lymph nodes (n = 12, 34.3%), musculoskeletal system (n = 12, 34.3%), and neurological system (n = 9, 25.7%). We identified three clusters in our study cohort: those with eye involvement (n = 11, 31.4%), those with eye involvement and neurological findings (n = 15, 42.9%), and those with pancreato-hepatobiliary disease and lymph node involvement (n = 9, 25.7%). Serum IgG4 levels were high in 19 out of 28 patients (67.8%). All patients except one received corticosteroid treatment, and azathioprine was the most preferred drug as a steroid-sparing agent. The sensitivities of the 2019 ACR/EULAR classification criteria and the 2020 RCD criteria were 5.7% and 88.5%, respectively.

Conclusion
IgG4-RD has a wide variety of clinical manifestations; however, in children, the most common presentation was orbital involvement. The 2020 RCD criteria had a better performance whereas the 2019 ACR/EULAR classification criteria performed poorly in paediatric patients.
Partisanship and attribution of responsibility during the COVID-19 pandemic in Japan
Authors: Okamoto Kakeru , Zenkyo Masahiro 
Published: September 2024

Abstract
This study examines the attribution of responsibility for government failures during the coronavirus disease 2019 (COVID-19) pandemic in Japan—specifically, responsibility for the collapse of the healthcare system. The intent is to reveal which political actors Japanese voters hold accountable and how partisanship is associated with perceptions of responsibility. While there have been previous studies on retrospective voting in Japan, little empirical work has been done to shed light on the attribution of responsibility. Based on the results of an online survey conducted in four prefectures in August 2021, this study yields two main findings. First, Japanese voters tend to blame national politicians rather than local officials or government employees for the failures of the healthcare system, and are particularly likely to attribute responsibility to the prime minister as well as to politicians from the ruling and opposition parties. Second, both positive and negative partisanship are associated with attribution of responsibility, and negative partisanship is more strongly correlated with attribution of responsibility than positive partisanship. Thus, negative partisanship plays an important role in shaping voters’ perceptions of government failures in Japan.
Clinical profile and thiamine transporter gene (SLC19A2 and SLC19A3) variations in infants with thiamine-responsive pulmonary hypertension and acute respiratory infection
Authors: Shenoy Swathi , Deekshit Vijaya Kumar , Rao Swathi Sunil , Ashwini Prathibha Shankar , Shenoy Rathika Damodara 
Published: September 2024

Abstract
Maternal thiamine deficiency is prevalent in low- and middle-income countries. Thiamine-responsive pulmonary hypertension (TRPHTN) in exclusively breastfed infants is reported in India. Thiamine transporter gene (ThTR) variations have not been studied. This study compared the presentation of exclusively breastfed infants with respiratory distress diagnosed as TRPHTN or acute respiratory infection (ARI). We investigated pathogenic variations in the SLC19A2 and SLC19A3 ThTr genes in a representative sample. Observational study. Tertiary care pediatric unit of a teaching hospital in southern India. Data collection was prospective. We included exclusively breastfed infants between 1 and 6 months of age with respiratory distress. Infants with PHTN in echocardiography and lactic acidosis (LA) received thiamine. TRPHTN was diagnosed based on response within 72 h. Infants with fever, chest findings, and positive microbiology were managed as ARI. The ThTr genes were sequenced and analyzed. Chi-square and stratified analysis were done to determine TRPHTN risk. Forty infants with TRPHTN and 42 with ARI were included. The median pulmonary arterial pressure in the TRPHTN group was 51.5 mmHg. Mild PHTN was seen in 65%, moderate in 22.5%, and severe in 12.5%. Cardiac failure (P &lt; .001), stridor and aphonia (P &lt; .001), encephalopathy (P = .024), LA (P &lt; .001), and PHTN (P &lt;.001) facilitated the diagnosis. The adjusted risk was 17.3 (95% confidence interval 7.8–38.3; P &lt;.001). The ThTR sequencing showed wild-type genotypes. TRPHTN has a distinct, identifiable presentation. Lactate and pulmonary pressure estimations are useful investigations in thiamine deficiency endemic areas. We could not demonstrate a genetic variation that determines susceptibility.
Emerging severe acute respiratory syndrome coronavirus 2 variants and their impact on immune evasion and vaccine-induced immunity
Authors: Pandey Ramendra Pati , Kumar Sachin , Rao D N , Gupta Dablu Lal 
Published: September 2024

Abstract
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) variants harboring mutations in the structural protein, especially in the receptor binding domain (RBD) of spike protein, have raised concern about potential immune escape. The spike protein of SARS-CoV-2 plays a vital role in infection and is an important target for neutralizing antibodies. The mutations that occur in the structural proteins, especially in the spike protein, lead to changes in the virus attributes of transmissibility, an increase in disease severity, a notable reduction in neutralizing antibodies generated and thus a decreased response to vaccines and therapy. The observed multiple mutations in the RBD of the spike protein showed immune escape because it increases the affinity of spike protein binding with the ACE-2 receptor of host cells and increases resistance to neutralizing antibodies. Cytotoxic T-cell responses are crucial in controlling SARS-CoV-2 infections from the infected tissues and clearing them from circulation. Cytotoxic T cells efficiently recognized the infected cells and killed them by releasing soluble mediator's perforin and granzymes. However, the overwhelming response of T cells and, subsequently, the overproduction of inflammatory mediators during severe infections with SARS-CoV-2 may lead to poor outcomes. This review article summarizes the impact of mutations in the spike protein of SARS-CoV-2, especially mutations of RBD, on immunogenicity, immune escape and vaccine-induced immunity, which could contribute to future studies focusing on vaccine design and immunotherapy.
Successfully treated refractory Sjögren’s syndrome myelopathy with tocilizumab; case report
Authors: Shahada Zienab , Kudsi Maysoun 
Published: September 2024

Introduction and importance:
myelitis in patients with Sjögren’s syndrome is rare and its incidence does not exceed 5%. It is usually treated with corticosteroids, immunosuppressants, and different treatment lines that have not yet been agreed upon.

Case presentation:
A 59-year-old man with a 10-year history of primary Sjögren’s syndrome (PSs) based on the presence of dry mouth and eyes, Schirmer’s test positivity, and anti-SS-A positivity. Suffering from about 3 months of history of weakness in the upper and lower extremities, about 3 months ago, Magnetic resonance imaging (MRI) consisting of cervical myelitis was performed. Because there was no other cause due to his previous Sjögren’s condition, Sjögren’s myelitis was considered a complication of PSs. He was placed on a treatment plan that included cyclophosphamide and corticosteroids, with good improvement clinically and radiologically on the MRI. Accordingly, he began gradually reducing the cortisone, but unfortunately, the patient relapsed and the symptoms worsened significantly, both clinically and on the MRI, before the patient entered the hospital. An increase in serum amyloid A protein (SAA) level was also found. Here, the treatment was moved to biological treatment. The patient was given subcutaneous tocilizumab (TCZ), 162 mg every 2 weeks, and the gradual improvement was good. No relapses occurred, and the level of SAA remained low until 8 months from the start of treatment. Corticosteroids were started to be reduced, and no relapses occurred in clinically and radiological symptoms.

Discussion:
We mentioned in this manuscript the case of a patient with PSs who had a complication of a neurological injury, namely cervical myelitis. He improved significantly on treatment with subcutaneous TCZ every 2 weeks, with complete remission of clinical and radiological symptoms and a decrease in the serum level of SAA, even after reducing the dose of cortisone without the risk of serious adverse events such as infections.

Conclusion:
In this case, subcutaneous TCZ has proven effective in treating myelitis when high protein amyloid A (SAA) levels are present in the blood. This may be the first case describing the effectiveness of TCZ in treating this condition.
Changes in Skin Paddle Morphology after Autologous Breast Reconstruction
Authors: Iizuka Chika , Matsumine Hajime , Sakurai Hiroyuki 
Published: September 2024

Background:
Immediate autologous breast reconstruction (IABR) can provide favorable aesthetic outcomes after skin-sparing mastectomy. However, it is known that the morphology of the reconstructed breast changes over time. Therefore, it is necessary to be able to predict the likely amount of change preoperatively to reconstruct a symmetrical breast. In this study, we retrospectively examined the change in position and morphology of the skin paddle of the reconstructed breast over time.

Methods:
Thirty-five patients who underwent IABR after skin-sparing mastectomy for unilateral breast cancer were included. Three-dimensional images were obtained at 1 month and 12 months postoperatively to compare changes in the position and size of the skin paddle over time.

Results:
Significant increases were observed in the distance between the center of the skin paddle and the midpoint of the clavicle, the distance between the center of the skin paddle and the sternal notch, and projection. No significant change was observed in the distance between the inframammary fold and the center of the skin paddle. There was a significant increase in the area and short axis of the skin paddle.

Conclusions:
Our main findings were that the skin paddle shifts toward the outer caudal side after IABR and tends to become larger. When planning delayed nipple reconstruction with a local flap designed on a skin paddle, the paddle should be positioned slightly more mediocranially than the healthy nipple and should be narrower.
Algorithm-based Management of Infantile Hemangiomas: Reducing Sequelae and Surgical Interventions
Authors: Nishimoto Akana , Ogawa Rei , Akaishi Satoshi 
Published: September 2024

Background:
In Japan, oral propranolol (PPL) and pulsed dye laser are available for infantile hemangioma (IH) treatment without patient cost-sharing. However, no standardized algorithm exists to guide treatment selection that balances efficacy, potential side effects, and aesthetic risks. This study presents a comprehensive approach utilizing a treatment algorithm and aesthetic risk scoring system.

Methods:
This retrospective study analyzed outcomes from 156 patients with IHs. Oral PPL was used in IH patients with functional issues, whereas the rest underwent an aesthetic risk assessment that categorized them into low-, moderate-, or high-risk groups to guide treatment choices. Final treatment decisions depended on parental preference. The outcomes of algorithm-compliant and noncompliant patients were compared statistically.

Results:
The risk score's interrater reliability was 0.973 (95% confidence interval 0.933–0.992), with a mean intrarater reliability of 0.968 ± 0.027 and a mean evaluation time of 14.1 ± 5.0 seconds per case. Among the 156 patients, 88% pursued the algorithm-recommended treatment, whereas 12% opted for different approaches. Algorithm-compliant patients experienced significantly fewer sequelae than did noncompliant patients (5% versus 33%, P < 0.001). Compared with noncompliant patients, algorithm-compliant patients tended to require shorter treatment durations (17.9 versus 25.4 mo, P = 0.08) and fewer laser sessions (5.8 versus 7.2, P = 0.30), with a younger age at resolution (21.3 versus 29.0 mo, P = 0.08).

Conclusions:
Aesthetic concerns can be crucial for patients with IHs. This study introduces a comprehensive IH management algorithm to reduce the sequelae requiring surgical interventions and improve patients’ quality of life.
The Effects of Abnormal Exposure on Individual Dose Monitoring with TLD Dosimeters
Authors: Yi Yanling , Stabin Michael G. 
Published: September 2024

Abstract
Objectives: To analyze the effects of normal x-ray inspection, machine washing, and machine drying on thermoluminescent dosimeter (TLD) measurements during external individual monitoring and to provide suggestions for determining individual monitoring measurements under the mentioned abnormal situations. In this study, we focused on three abnormal situations: x-ray inspection, machine washing, and machine drying, which are common in external individual dose monitoring. We measured and compared the doses from TLD with and without 11, 23, 35, and 50 security checks. We used different radiation sources to expose the TLDs before or after machine washing with or without hot drying. The three radiation sources are natural background radiation, 137Cs γ rays, and 320 kVp x-rays. We measured 20 TLDs for each situation. The average doses for the TLDs with 11, 23, 35, 50 security checks are 27.7 μGy, 59.7 μGy, 84.1 μGy, and 121.0 μGy, respectively. We measured an average dose of 2.5 μGy per exposure. The doses showed no significant difference between different times of washing with different radiation sources, natural background radiation, 137Cs, or x-ray exposures. There was also no significant difference between the dose coming from the controlled group, drying at 60 °C and 90 °C for 1 h after exposure to 137Cs γ rays and 320 kVp x-rays. The common machine drying under the temperature of 90 °C did not affect TLD measured doses.
The Prognosis in Children With Pneumonia of Respiratory Syncytial Virus Co-detection With Airway Dominant Flora
Authors: Li Lu , Xu Ximing , Liu Enmei , Deng Yu 
Published: September 2024

Background:
Airway bacterial microbiota influences the prognosis in children with respiratory syncytial virus infection. The study aimed to investigate the effect of the airway-dominant bacterial microbiota on disease severity in children with pneumonia of respiratory syncytial virus infection.

Methods:
A retrospective study was conducted in the Children’s Hospital of Chongqing Medical University, which involved a cohort of patients with respiratory syncytial virus (RSV)-infected pneumonia from January 2012 to December 2021. Patients were assigned to a normal flora group or to a dominant flora group (with the top 5 individual bacteria) based on the nasopharyngeal aspirates culture and matched using propensity-score matching. Univariate analysis and multivariate analysis were performed to estimate the risk factors of poor prognosis in dominant flora.

Results:
Five thousand five hundred and twelve patients in the normal flora and 4556 in the dominant flora were included (Escherichia coli 514, Streptococcus pneumoniae 1516, Staphylococcus aureus 506, Moraxella catarrhalis 509 and Haemophilus influenzae 1516, respectively). The dominant flora had more patients developing severe pneumonia, needing mechanical ventilation/tracheal intubation (up to 15.8% in the S. aureus) and admission to the intensive care unit (up to 4.5% in the E. coli) than in the normal flora (28.5% vs. 25.9%; P = 0.001; 9.8% vs. 5.4%; P < 0.001; 2.0% vs. 1.2%; P <0.001). And the hospitalization was longer in the dominant flora than in the normal flora [8 (6–9) vs. 8 (7–9) days; P < 0.001], the E. coli and S. aureus had the longest hospitalization [8 (7–10) days]. Several factors were associated with critical illness in Dominant flora according to multivariate analysis (P < 0.001), including age (OR: 0.965; CI: 0.954–0.976; P < 0.001), anhelation (OR: 0.530; CI: 0.446–0.631; P < 0.001), disorders of consciousness (OR: 0.055; CI: 0.016–0.185; P < 0.001) as well as assisted respiration (OR: 0.115; CI: 0.097–0.138; P < 0.001), C-reactive protein >10 mg/L (OR: 0.686; CI: 0.560–0.839; P < 0.001), SpO2 <90% (OR: 0.366; CI: 0.214–0.628; P < 0.001), pulmonary consolidation (OR: 0.511; CI: 0.364–0.717; P < 0.001) and pulmonary atelectasis (OR: 0.362; CI: 0.236–0.555; P < 0.001).

Conclusions:
The airway-dominant bacterial microbiota influenced disease severity and comorbidities in children with RSV-infected pneumonia. Clinicians should pay attention to the nasopharyngeal aspirate culture, especially after detecting S. aureus and E. coli in RSV-infected children with pneumonia, closely observe the disease progression and take timely measures to avoid adverse outcomes.
The impact of the COVID-19 pandemic on dyslipidemia in peritoneal dialysis patients
Authors: Chen Fan-Yu , Li Shu-En , Tsai Jen-En , Lu Po-Yen , Li Yu-Hsuan , Tan Ann Charis , Li Szu-Yuan , Chuang Yi-Fang , Chen Chyong-Mei , Lin Chih-Ching 
Published: September 2024

Background:
The coronavirus disease 2019 (COVID-19) pandemic has had a profound impacted on various aspects of society, including the healthcare system and patient care. In this context, this study aimed to evaluate the impact of COVID-19 control strategies on the lipid profile and blood sugar levels of peritoneal dialysis (PD) patients in Taiwan, a crucial focus for understanding the pandemic’s influence on individuals with chronic kidney disease (CKD).

Methods:
A retrospective cohort study was conducted, analyzing data from the medical records of 170 PD patients who visited the nephrology division of Taipei Veterans General Hospital in 2021. The generalized estimating equations method was used to analyze the longitudinal data and assess the changes in biomarker levels over different periods. Covariates were taken into consideration in the statistical models.

Results:
The study enrolled 70 (41%) males and 100 (59%) females, with an average age of 56 years old. Over 12 months in 2021, from the first period (January to April: pre-COVID-19) to the second period (May to August: COVID-19 surge), there was a notable decline in both high-density lipoprotein (HDL) and low-density lipoprotein (LDL) levels, and a significant surge in triglyceride (TG) levels. However, total cholesterol (TC) and hemoglobin (HbA1c) levels remained stable. Furthermore, the TG to HDL, TG to LDL, TC to HDL, and TC to LDL ratios were analyzed, revealing a pronounced increase during the second period.

Conclusion:
Our findings underscore the significant impact of COVID-19 pandemic-related disruptions in the healthcare system and the subsequent management strategies on dyslipidemia in PD patients while not affecting dysglycemia. These results provide valuable insights for healthcare professionals to enhance their strategies and interventions for CKD patients undergoing PD during a pandemic.
Neuromonitoring in the ICU: noninvasive and invasive modalities for critically ill children and neonates
Authors: Agarwal Neha , Benedetti Giulia M. 
Published: September 2024

Purpose of review
Critically ill children are at risk of neurologic dysfunction and acquiring primary and secondary brain injury. Close monitoring of cerebral function is crucial to prevent, detect, and treat these complications.

Recent findings
A variety of neuromonitoring modalities are currently used in pediatric and neonatal ICUs. These include noninvasive modalities, such as electroencephalography, transcranial Doppler, and near-infrared spectroscopy, as well as invasive methods including intracranial pressure monitoring, brain tissue oxygen measurement, and cerebral microdialysis. Each modality offers unique insights into neurologic function, cerebral circulation, or metabolism to support individualized neurologic care based on a patient's own physiology. Utilization of these modalities in ICUs results in reduced neurologic injury and mortality and improved neurodevelopmental outcomes.

Summary
Monitoring of neurologic function can significantly improve care of critically ill children. Additional research is needed to establish normative values in pediatric patients and to standardize the use of these modalities.
18F-FDG, 68Ga-PSMA, and 64Cu-DOTATATE PET/CT—One Patient, Three Cancers
Authors: Rahman Ryan , Palot Manzil Fathima Fijula , Eichhorn Joshua 
Published: September 2024

Abstract
A 67-year-old man with a history of multiple myeloma, prostate cancer, and rectal carcinoid tumor underwent 18F-FDG PET/CT for surveillance, which revealed a non–FDG-avid lesion in the liver. To rule out metastasis from prostate cancer and carcinoid tumor, 68Ga-PSMA and 64Cu-DOTATATE PET/CT scans were ordered for respective malignancies. The 64Cu-DOTATATE scan showed radiotracer uptake in the hepatic lesion compatible with metastasis of the carcinoid tumor. Additionally, both 68Ga-PSMA and 64Cu-DOTATATE PET/CT scans revealed nodal metastasis within the pelvis. This case highlights the importance of appropriate radiotracer selection in conjunction with patient history in the evaluation of patients with multiple malignancies.
Microbe Profile: Pseudonocardia: antibiotics for every niche
Authors: Whatmough Bonnie , Holmes Neil A. , Wilkinson Barrie , Hutchings Matthew I. , Parra Jonathan , Duncan Katherine R. 
Published: September 2024

Graphical abstract
Scanning electron microscopy (SEM) images of Pseudonocardia sp. (a). Ae707-Ps1 and (b). Ae707-Ps2. These are the two lineages of Pseudonocardia associated with Acromyrmex echinatior leafcutter ants collected in Gamboa, Panama. (c). Four specialised metabolites are shown that are produced by Pseudonocardia strains isolated from diverse ecosystems: they are 9-methoxyrebeccamycin, selvamicin, pseudonochelin and attinimicin. (d). This tree shows a subset of phylogenetic diversity showing strain origin (outer ring colour), genome size (Mb, red box) and number of biosynthetic gene clusters (BGCs, purple box). The specialised metabolites (c) are coloured to match the producing strains, the boxed numbers show the genome size (red) and number of BGCs (purple) (d).