Reichel Syndrome in Children: A Case Report.

Reichel syndrome or primary synovial chondromatosis (PSC) is an uncommon benign metaplastic condition that usually affects large joints. Though shoulder involvement was scarce, there are only a few cases in the pediatric population. A 14- year-old boy was admitted to the Pediatric Orthopedics department with right shoulder pain for 14 months.

Development of the paediatric society of the African league against rheumatism (PAFLAR) JIA registry and clinical profile of JIA in Africa from the PAFLAR JIA registry.

Background: The spectrum of Juvenile Idiopathic Arthritis (JIA) in Africa is still largely unknown. We thus set out to illustrate how we set up the PAFLAR JIA registry and describe the clinical profile of Juvenile Idiopathic Arthritis across various regions in Africa.

Methods: We carried out a retrospective observational cohort study where collaborators were trained on use of the existing PAFLAR REDCAP database to enter data for the JIA patients currently under their care capturing their epidemiological data, clinical features, laboratory investigations, diagnosis and therapy at initial diagnosis.

An Atypical Cause of a Child Limp: A Gorham-Stout Disease with a Vanishing Hip.

Gorham-Stout disease (GSD) is a rare bone disease characterized by an abnormal proliferation of endothelial-lined vessels and destruction of the affected bone. As it affects commonly children and young adults, it is associated with significant morbidity and mortality. To date, there is no established treatment strategy for GSD.

Navigating the Factors Affecting Functional Impairment in Spondyloarthritis.

Objectives: This study aimed to assess the predictive factors of functional impairment in spondyloarthritis (SpA) patients assessed with bath ankylosing spondylitis functional index (BASFI) and Lequesne Index (LI).

Methods: This retrospective study was conducted at the Rheumatology Department of Mohamed Kassab Institute of Orthopedics, Manubah, Tunisia, and collected data from 2008 to 2019 over a period of 4 months (August to November 2019). Socio-demographic and disease-related data of SpA patients were collected.

Pediatric and adult osteoporosis: a contrasting mirror.

Pediatric osteoporosis (PO) is a condition that is currently gaining recognition. Due to the lack of official definitions over the past few decades, the exact incidence of PO is unknown. The research does not provide a specific prevalence of PO in different world regions.

[Clinical examination of the child's foot].

CLINICAL EXAMINATION OF THE CHILD'S FOOT. The clinical examination of the child's foot is part of the musculoskeletal examination and requires a good knowledge of morphological and physiological features of foot during growth. This article describes the stages of the clinical assessment of the child's foot from walking age onwards, as well as the main disorders to be investigated.

Anterior Chest Wall Non-traumatic Arthropathies: A Crucial but Often Overlooked Site.

Objective: The purpose of this study was to describe the distribution of Anterior Chest Wall (ACW) arthropathies in a tertiary care center and identify clinical, biological and imaging findings to differentiate osteoarthritis (OA) from non-osteoarthritis (N-OA) etiologies.

Methods: Search from medical records from January 2009 to April 2022, including patients with manubriosternal and/or sternoclavicular and/or sternocostal joint changes confirmed by ultrasonography, computed tomography or magnetic resonance imaging. The final study group was divided into OA and N-OA subgroups.

The childhood arthritis radiographic score of the hip: the proposal cut-off value using cluster analysis.

Background: Juvenile idiopathic arthritis (JIA) is a chronic rheumatic disease that affects children. It is crucial to detect and treat hip involvement in JIA early to prevent functional impairment and reduced quality of life. The Childhood Arthritis Radiographic Score of the Hip (CARSH) is a validated radiographic scoring system used to assess hip involvement in JIA.

Probability of the 10-year Risk of Hip and Major Osteoporotic Fracture in Non-radiographic Axial Spondyloarthritis.

Background: Fracture risk in non-radiographic spondyloarthritis is underestimated. A reliable tool such as the Fracture Risk Assessment tool (FRAX) may assess this risk probability. This study aimed to assess the fracture risk by the FRAX score in patients with nr-axSpA and to determine factors associated with high fracture risk.

Denosumab use in osteogenesis imperfecta: an update on therapeutic approaches.

Osteogenesis imperfecta (OI) is an inherited skeletal disorder that leads to bone fragility and multiple fractures. Given advances in the genetic understanding of existing phenotypes and newly discovered mutations, therapeutic management of OI has become challenging. Denosumab, a monoclonal antibody that inhibits the interaction between the receptor activator of nuclear factor kappa B ligand (RANKL) and its receptor RANK, has been approved to treat postmenopausal osteoporosis and emerged as an important therapy for malignancies and other skeletal disorders, including pediatric skeletal conditions such as OI.

Predictors factors of atlanto-axial subluxation in Tunisian patients with rheumatoid arthritis.

Background: The aim of this study was to describe clinical and imaging features of atlantoaxial subluxation (AAS) and the associated risk factors in patients with rheumatoid arthritis (RA).

Methods: We conducted a retrospective and comparative study including 51 RA patients with AAS and 51 RA patients without AAS. Atlantoaxial subluxation was defined by the presence of an anterior C1C2 diastasis on the cervical spine radiograph in hyperflexion and/or an anterior, posterior, lateral or rotatory C1C2 dislocation on MRI with/without inflammatory signal.

Osteonecrosis of the Tarsal Navicular: Not Always Spontaneous!

Background: Mueller-Weiss disease, a rare and complex foot condition, is defined as spontaneous and progressive navicular fragmentation leading to midfoot pain and deformity. However, its exact etiopathogenesis remains unclear. We report a case series of tarsal navicular osteonecrosis to describe the clinical and imaging characteristics and etiologic profile of the disease.

Factors associated with poor prognosis of hip arthritis in juvenile idiopathic arthritis: Data from the JIR cohort.

Objectives: Hip involvement remains a predictor of severe juvenile idiopathic arthritis (JIA) course and carries a high risk of disability. This study aims to determine the factors of poor prognosis of hip involvement in patients with JIA and to assess the treatment response.

Methods: This is a multicenter observational cohort study.

Vertebral sarcoidosis: diagnosis to management.

Sarcoidosis is a systemic inflammatory granulomatous disease that can develop in almost any organ system. Rheumatologists may encounter sarcoidosis in different situations varying from arthralgia to bone involvement. While the peripheral skeleton was a frequent location, data regarding axial involvement is scarce.

Non-pharmacological therapies in Fibromyalgia: New horizons for physicians, new hopes for patients.

Introduction: Fibromyalgia (FM) is a chronic musculoskeletal condition characterised by reduced quality of life and severe limitations in daily living activities. Considering the wide spectrum of symptoms and the ineffectiveness of a single pharmacological approach, the latest clinical guidelines recommend non-pharmacological therapies as both an alternative and a better-tolerated approach. Several studies have been conducted to determine the effectiveness of non-pharmacological therapies in the management of FM.

The concept of telemedicine in pediatric rheumatology in Tunisia: Parents' perceptions.

Introduction: With the COVID-19 pandemic, health care systems are facing challenges in delivering proper patient care. Children and adolescents with juvenile idiopathic arthritis require specialized and comprehensive attention. In this context, telemedicine is an alternative that has the potential to improve access to healthcare in addition to cost savings.

Unexpected kinetically controlled organoselenium-based isomaleimide: X-ray structure, hirshfeld surface analysis, 3D energy framework approach, and density functional theory calculation.

Reduction of 4,4'-diselanediyldianiline () followed by the reaction with bromo-4-(bromomethyl)benzene afforded the corresponding 4-((4-bromobenzyl)selanyl)aniline () in 85% yield. -Maleanilic acid was obtained in 94% yield via the reaction of selenoamine with toxilic anhydride. Subsequent dehydration of -maleanilic acid using acetic anhydride furnished the unexpected isomaleimide 5-((4-((4-bromophenyl)selanyl)phenyl)imino)furan-2(5H)-one () instead of the maleimide .

New Organic-Inorganic Salt Based on Fluconazole Drug: TD-DFT Benchmark and Computational Insights into Halogen Substitution.

In this study, we report the synthesis of a new organic-inorganic molecular salt of the clinically used antifungal drug fluconazole, (H2Fluconazole).SnCl.2HO.

Factors associated with the inflammatory process in pain in ankylosing spondylitis.

Introduction: sleep disorders, closely related to any chronic pain process, are frequent among patients with rheumatic diseases, mainly ankylosing spondylitis (AS). Our study aimed to determine the association between sleep disturbances and the inflammatory process in pain in AS patients compared with lower back pain (LBP) patients. We have additionally examined factors associated with sleep disorders among AS patients.

Impact of Lean and Fat Mass on Disease Activity and Function in Spondyloarthritis.

Background And Aims: Changes in body mass composition, such as reduced lean and fat mass, can occur in a patient with rheumatic diseases. However, data about body composition in spondyloarthritis (SpA) showed conflicting results. The aim of the present study was to assess by DXA the distribution of lean and fat mass in SpA patients compared to healthy controls and to investigate the association between body mass composition and disease characteristics.

A Competition between Hydrogen, Stacking, and Halogen Bonding in -(4-((3-Methyl-1,4-dioxo-1,4-dihydronaphthalen-2-yl)selanyl)phenyl)acetamide: Structure, Hirshfeld Surface Analysis, 3D Energy Framework Approach, and DFT Calculation.

-(4-((3-Methyl-1,4-dioxo-1,4-dihydronaphthalen-2-yl)selanyl)phenyl)acetamide (5), CHNOSe, was prepared in two steps from 4,4'-diselanediyldianiline (3) via reduction and subsequent nucleophilic reaction with 2-methyl-3-bromo-1,4-naphthalenedione, followed by acetylation with acetic anhydride. The cytotoxicity was estimated against 158N and 158JP oligodendrocytes and the redox profile was also evaluated using different in vitro assays. The technique of single-crystal X-ray diffraction is used to confirm the structure of compound 5.

Which Metrological Index, the BASMI or EDASMI, is Best Correlated with Disease-Related Parameters in Spondylarthritis Patients?

Objective: This study aimed at comparing the Edmonton Ankylosing Spondylitis Metrology Index (EDASMI) and Bath Ankylosing Spondylitis Metrology Index (BASMI) to determine which of the two is best correlated with disease-related parameters in axial spondyloarthritis (axSpA) patients.

Methods: A cross-sectional study was carried out involving 86 patients with radiographic axSpA. Sociodemographic data, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Ankylosing Spondylitis Disease Activity Score (ASDAS), the Bath Ankylosing Spondylitis Functional Index (BASFI), and the Ankylosing Spondylitis Quality of Life (ASQoL) questionnaire were applied.

Case report of Osteopoikilosis in patient with psoriatic arthritis.

Osteopoikilosis (OPK) is one of the rare genetic musculoskeletal, non-inflammatory disorders that we should increase awareness toward. We report a case of a patient diagnosed with psoriatic arthritis with incidental imaging findings of lesions suggestive of osteopoikilosis.