The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry.

Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.

Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.

Evaluation of Myocarditis in Patients With Still Disease: Clinical Findings From the Multicenter International AIDA Network Still Disease Registry.

Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.

Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.

Ototoxicity associated with hematopoietic stem cell transplantation; what are the risk factors?

Background: Hematopoietic stem cell transplantation (HSCT) is a critical treatment for various hematologic malignancies but can lead to complications, including ototoxicity.

Aim/objectives: This study aims to explore the relationship between patient-specific factors and ototoxicity in adult HSCT patients.

Material And Methods: We conducted a retrospective analysis of 129 adult patients who underwent HSCT between 2003 and 2020.

Clinical practice guideline for the prevention and management of peritoneal dialysis associated infections in children: 2024 update.

Infection-related complications remain the most significant cause for morbidity and technique failure in infants, children and adolescents who receive maintenance peritoneal dialysis (PD). The 2024 update of the Clinical Practice Guideline for the Prevention and Management of Peritoneal Dialysis Associated Infection in Children builds upon previous such guidelines published in 2000 and 2012 and provides comprehensive treatment guidance as recommended by an international group of pediatric PD experts based upon a review of published literature and pediatric PD registry data. The workgroup prioritized updating key clinical issues contained in the 2012 guidelines, in addition to addressing additional questions developed using the PICO format.

A Cockayne-Syndrome-Like Phenotype with a Homozygous Truncating Variant: Might This Be a New Cause?

Introduction: UV-sensitive syndrome and Cockayne syndrome (CS) are rare autosomal recessive and transcription-coupled nucleotide excision repair disorders with different clinical manifestations, although some types are allelic.

Case Presentation: We report on a patient who passed away at 15 years old with a progeroid-like appearance, cachexia, hearing loss, and dental anomalies, which led us to the diagnosis of Cockayne-like progeroid syndromes. Our clinical exome sequencing including all the known genes of progeroid syndromes revealed a homozygous stop-gain variant in the gene.

Changes in the cardiovascular risk profile in children approaching kidney replacement therapy.

Background: Despite significant cardiovascular (CV) morbidity in children on dialysis and after kidney transplantation, data on the evolution of CV damage in children with chronic kidney disease (CKD) approaching kidney replacement therapy (KRT) is unknown.

Methods: The burden, progression, and predictors of CV damage before KRT onset were explored in two prospective multicenter cohorts from Europe and Canada: Cardiovascular Comorbidity in Children with CKD (4C) and Haemodiafiltration, Heart and Height (3H) studies, conducted from 2009-19 and 2013-16, respectively. CV damage and risk factors were evaluated (i) cross sectionally at KRT-start (n = 248), and (ii) longitudinally over the 2-years preceding KRT start (n = 157; 331 patient-visits).

Prognostic Role of OX40, LAG-3, TIM-3 and PD-L1 Expression in Bone and Soft Tissue Sarcomas.

The current study aims to evaluate the OX40, TIM-3, LAG-3, and PD-L1 targeted pathways in the regulation of T-cell activity in sarcoma patients to determine their relationship with overall survival (OS). This study included one hundred and eleven patients with bone and soft tissue sarcoma diagnosed in two centers between 2010 and 2020. OX40, LAG-3, TIM-3 and PD-L1 expression levels were evaluated immunohistochemically from pathology preparations.

Prospective Study of Modifiable Risk Factors of Arterial Hypertension and Left Ventricular Hypertrophy in Pediatric Patients on Hemodialysis.

Introduction: Fluid and salt overload in patients on dialysis result in high blood pressure (BP), left ventricular hypertrophy (LVH) and hemodynamic instability, resulting in cardiovascular morbidity.

Methods: Analysis of 910 pediatric patients on maintenance hemodialysis/hemodiafiltration (HD/HDF), prospectively followed-up with 2758 observations recorded every 6-months in the International Pediatric Hemodialysis Network (IPHN).

Results: Uncontrolled hypertension was present in 55% of observations, with 27% of patients exhibiting persistently elevated predialysis BP.

Clinical characteristics and disease course before and after SARS-CoV-2 infection in a large cohort of systemic sclerosis patients.

Background/aim: The objective of this study is to evaluate the clinical presentations and adverse outcomes of Coronavirus Disease 2019 (COVID-19) in patients with systemic sclerosis (SSc) and assess the impact of SSc features on the clinical course of COVID-19.

Materials And Methods: In this multicenter, retrospective study, SSc patients with COVID-19 were included. Clinical features of SSc, along with detailed COVID-19 data, were extracted from medical records and patient interviews.

Impact of HLA-B51 on Uveitis and Retinal Vasculitis: Data from the AIDA International Network Registries on Ocular Inflammatory Disorders.

Purpose: The clinical relevance of human leukocyte antigen (HLA) subtypes such as HLA-B51 on Behçet's disease (BD)-related uveitis and non-infectious uveitis (NIU) unrelated to BD remains largely unknown.

Methods: Data were prospectively collected from the International AIDA Network Registry for BD and for NIU. We assessed differences between groups (NIU unrelated to BD and positive for HLA-B51, BD-related uveitis positive for HLA-B51 and BD-related uveitis negative for HLA-B51) in terms of long-term ocular complications, visual acuity (VA) measured by best corrected visual acuity (BCVA), anatomical pattern, occurrence of retinal vasculitis (RV) and macular edema over time.

Totally Implantable Venous Access Device Care Practices and Experiences of Pediatric and Adult Oncology Nurses.

This study aimed to determine the practices of nurses working in pediatric and adult oncology clinics regarding totally implantable venous access device (TIVAD) care. The descriptive study was conducted with 227 oncology nurse members of the Oncology Nursing Association. The data were collected online with a survey form, which included questions about the participants' sociodemographic characteristics, professional experience, and TIVAD implementation practices.

Neonatal somatic oxygenation and perfusion assessment using near-infrared spectroscopy : Part of the series on near-infrared spectroscopy by the European Society of Paediatric Research Special Interest Group "Near-Infrared Spectroscopy".

In this narrative review, we summarize the current knowledge and applications of somatic near-infrared spectroscopy (NIRS), with a focus on intestinal, renal, limb, and multi-site applications in neonates. Assessing somatic oxygenation at various body locations in neonates may aid in the understanding of underlying pathophysiology of organ injury. Considering cerebral autoregulation may be active to protect the brain during systemic circulatory failure, peripheral somatic oxygenation may potentially provide an early indication of neonatal cardiovascular failure and ultimate hypoxemic injury to vital organs including the brain.

Adnexal masses and pregnancy: a single-center experience of 9 years.

Purpose: This study aims to analyze the experience of a tertiary health center about the management of adnexal masses that have been diagnosed during pregnancy or detected accidentally during cesarean delivery.

Methods: This is a retrospective review of 160 women who underwent concurrent surgery for adnexal mass during cesarean section, 24 women who delivered vaginally and subsequently had surgery due to the prenatal diagnosis of adnexal mass and 10 women who underwent surgery for adnexal mass during pregnancy. Corresponding to the delivery and surgery times, 200 women who had no diagnosis of pregnancy-associated adnexal mass served as controls.

Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network.

Introduction: This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD).

Methods: This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled.

Pericardiectomy after constrictive pericarditis associated with second dose of BNT162b2 vaccine: A case report.

Constrictive pericarditis is quite rare complication of messenger ribonucleic acid-based severe acute respiratory syndrome-Coronavirus 2 (SARS-CoV-2) vaccine. It is a severe clinical picture with clinical symptoms of right ventricular failure. Initial physical examination, laboratory work-up, and chest X-ray may yield non-specific findings.

Improving resolution of panoramic radiographs: super-resolution concept.

Objectives: Dental imaging plays a key role in the diagnosis and treatment of dental conditions, yet limitations regarding the quality and resolution of dental radiographs sometimes hinder precise analysis. Super-resolution with deep learning refers to a set of techniques used to enhance the resolution of images beyond their original size or quality using deep neural networks instead of traditional image interpolation methods which often result in blurred or pixelated images when attempting to increase resolution. Leveraging advancements in technology, this study aims to enhance the resolution of dental panoramic radiographs, thereby enabling more accurate diagnoses and treatment planning.