Rare manifestations of sarcoidosis in a young boy.

Sarcoidosis is a chronic multisystem granulomatous disease of unknown etiology. It is rare in young children. A 9-year-old boy presented with failure to thrive, skin rashes, persistent fever, and respiratory symptoms since 5 years of age.

Gender differences in juvenile systemic sclerosis patients: Results from the international juvenile scleroderma inception cohort.

Objective: To compare organ involvement and disease severity between male and female patients with juvenile onset systemic sclerosis.

Methods: Demographics, organ involvement, laboratory evaluation, patient-reported outcomes and physician assessment variables were compared between male and female juvenile onset systemic sclerosis patients enrolled in the prospective international juvenile systemic sclerosis cohort at their baseline visit and after 12 months.

Results: One hundred and seventy-five juvenile onset systemic sclerosis patients were evaluated, 142 females and 33 males.

Identification of musculoskeletal problems using pGALS examination in school going boys in Chennai, India.

Background And Aim: pGALS (pediatric Gait Arms Legs Spine) is a useful screening tool in identifying musculoskeletal problems in children. Although musculoskeletal problems are common in children, only a small fraction of them have an underlying serious pathology. There is limited data on this subject from north India but none from south India.

Analysis of Clinicopathological Characteristics and Its Correlation With the Prognosis of Pediatric Lupus Nephritis: A Tertiary Care Center Experience.

Aim To study the various pathological patterns of pediatric lupus nephritis (LN) by renal biopsies and to correlate the histopathological data with the clinical and biochemical outcomes. Methods This is a retrospective study in children between 1 month and 18 years of age with renal biopsy-proven lupus nephritis, conducted between January 2015 and December 2019. Various pathological and clinical parameters were compared between the groups with lupus nephritis activity and those without activity.

Juvenile dermatomyositis: a case of delayed recognition with unusual complication of nephrocalcinosis.

Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children and is characterised by the presence of proximal muscle weakness, heliotrope dermatitis, Gottron's papules and occasionally auto antibodies. The disease primarily affects skin and muscles, but can also affect other organs. Renal manifestations though common in autoimmune conditions like lupus are rare in JDM.

Differences Sustained Between Diffuse and Limited Forms of Juvenile Systemic Sclerosis in an Expanded International Cohort.

Objective: To evaluate the baseline clinical characteristics of juvenile systemic sclerosis (SSc) patients in the international juvenile SSc inception cohort, and to compare these characteristics between the classically defined juvenile diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc) subtypes and among those with overlap features.

Methods: A cross-sectional study was performed using baseline visit data. Information on demographic characteristics, organ system evaluation, treatment, and patient- and physician-reported outcomes was extracted and summary statistics applied.

Disseminated tuberculosis with macrophage activation syndrome in a child with lupus nephritis.

In children with systemic lupus erythematosus on immunosuppressive therapy, infection is a known complication. We present a case of a 12-year-old girl who was previously diagnosed with lupus nephritis but had stopped taking allopathic medications and had been on herbal medicines for a year. She was referred to us with persistent fever and disease activity in spite of restarting immunosuppressive treatment.

Underdetection of Interstitial Lung Disease in Juvenile Systemic Sclerosis.

Objective: Utilizing data obtained from a prospective, international, juvenile systemic sclerosis (SSc) cohort, the present study was undertaken to determine if pulmonary screening with forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLco) is sufficient to assess the presence of interstitial lung disease (ILD) in comparison to high-resolution computed tomography (HRCT) in juvenile SSc.

Methods: The juvenile SSc cohort database was queried for patients enrolled from January 2008 to January 2020 with recorded pulmonary function tests (PFTs) parameters and HRCT to determine the discriminatory properties of PFT parameters, FVC, and DLco in detecting ILD.

Results: Eighty-six juvenile SSc patients had both computed tomography imaging and FVC values for direct comparison.

Achromobacter xylosoxidans Sepsis Unveiling X-linked Agammaglobulinemia Masquerading as Systemic-onset Juvenile Idiopathic Arthritis.

Background: X-linked agammaglobulinemia, a primary immunodeficiency, can present with musculoskeletal manifestations.

Case Characteristics: A 4-year-old boy, diagnosed as systemic juvenile idiopathic arthritis at the age of 3 years and treated with biological agents, presented with fever, dyspnea and chest pain. Blood culture and pericardial fluid culture revealed Achromobacter xylosoxidans.

Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? Clinical characteristics of a juvenile systemic sclerosis cohort.

Introduction: Juvenile systemic sclerosis is an orphan disease. Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes.

Therapeutic plasma exchange for pediatric nonrenal disease indications and outcomes: A single-center experience.

Introduction: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients.

Subjects And Methods: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken.

Familial Blau syndrome:First molecularly confirmed report from India.

Blau syndrome (BS) is a rare autoinflammatory disorder characterized by the clinical triad of arthritis, uveitis, and dermatitis due to heterozygous gain-of-function mutations in the NOD2 gene. BS can mimic juvenile idiopathic arthritis (JIA)-associated uveitis, rheumatoid arthritis, and ocular tuberculosis. We report a family comprising a mother and her two children, all presenting with uveitis and arthritis.