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| http://dx.doi.org/10.1590/abd1806-4841.20176216 | DOI Listing |
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Optimizing Drug Selection in Children with Multiple Sclerosis: What Do We Know and What Remains Unanswered?
Paediatr Drugs
December 2024
Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Pediatric-onset multiple sclerosis (POMS) refers to multiple sclerosis with onset before 18 years of age. It is characterized by a more inflammatory course, more frequent clinical relapses, and a greater number of magnetic resonance imaging (MRI) lesions compared with adult-onset MS (AOMS), leading to significant impacts on both disability progression and cognitive outcomes in affected individuals. Managing POMS presents distinct challenges due to the unique needs of pediatric patients and the limited number of disease-modifying therapies (DMTs) approved for pediatric use.
View Article and Find Full Text PDFType I interferon and mitochondrial dysfunction are associated with dysregulated cytotoxic CD8+ T cell responses in juvenile systemic lupus erythematosus.
Clin Exp Immunol
December 2024
Centre for Adolescent Rheumatology Versus Arthritis at UCL, UCLH, GOSH, London, UK.
Juvenile systemic lupus erythematosus (JSLE) is an autoimmune condition which causes significant morbidity in children and young adults and is more severe in its presentation than adult-onset SLE. While many aspects of immune dysfunction have been studied extensively in adult-onset SLE, there is limited and contradictory evidence of how cytotoxic CD8+ T cells contribute to disease pathogenesis and studies exploring cytotoxicity in JSLE are virtually non-existent. Here, we report that CD8+ T cell cytotoxic capacity is reduced in JSLE versus healthy controls, irrespective of treatment or disease activity.
View Article and Find Full Text PDFBreaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
Proc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFComparison of Pediatric- and Adult-Onset Mycosis Fungoides Patients in Terms of Clinical Features and Prognosis in a Large Series.
J Cutan Med Surg
December 2024
Department of Dermatology and Venereology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Background: Studies comparing the clinical and prognostic differences between pediatric- and adult-onset mycosis fungoides (MF) are limited.
Objectives: To determine the impact of childhood-onset MF on clinical features and disease course in a large series.
Methods: Consecutive MF patients seen in a single centre between 2007 and 2021 were categorized into 3 groups: (i) MF patients diagnosed in the pediatric ages (≤18 years) (pediatric group), (ii) MF patients with disease onset in the pediatric period and diagnosis in adulthood (lately diagnosed pediatric-onset group), and (iii) MF patients with disease onset in the adulthood period (>18 years) (adult-onset group).
Adult-onset dysphagia.
Aust J Gen Pract
December 2024
FACRRM, MBBS, JCCA, Rural Generalist Specialist with an Ear Nose and Throat Special Interest, Torres and Cape York Hospital Health Service, Cairns, Qld.
Background: Dysphagia, characterised by a difficulty in swallowing, stems from various causes and is frequently encountered in general practice. The rise in dysphagia in Australia's ageing population necessitates proper management to prevent complications. Recognising and managing dysphagia improves outcomes and quality of life, and reduces secondary complications.
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