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Safety and Efficacy of Long-term Use of Infliximab in Severe Juvenile Dermatomyositis - 12 Years of Follow-up.
Acta Dermatovenerol Croat
November 2024
Prof. Marija Jelušić, MD, PhD, Department of Paediatrics, University of Zagreb, School of Medicine, Division of Clinical Immunology, Rheumatology and Allergology, Centre of Reference for Paediatric and Adolescent Rheumatology of Ministry of Health of the Republic Croatia, University Hospital Centre Zagreb, Kispaticeva 12, 10 000 Zagreb, Croatia;
Juvenile dermatomyositis with emphasized vasculopathy is rare, but the most severe form of the disease, with a poor prognosis with relapsing and chronic course or, in some cases, lethal outcome. We present a case of a 19-year-old Caucasian female, who developed severe acute juvenile dermatomyositis with emphasized multisystem vasculopathy, including retinal vasculopathy and maculopathy (cotton-wool spots, retinal hemorrhages, macular edema) at the age of 8. Due to no response to standard treatment protocols and rapid worsening of clinical symptoms and laboratory findings, a TNF inhibitor (infliximab) was introduced after the third week of treatment resulting in complete normalisation of muscle enzyme levels and complete resolution of eye changes within the next 2 weeks with a gradual general recovery.
View Article and Find Full Text PDFRare DDX3X Gene Mutation in a Male Newborn With Super-refractory Status Epilepticus Responding to Lacosamide Drug Therapy.
Cureus
December 2024
Pediatric Neurology, Bahrain Defence Force Hospital, Riffa, BHR.
Super-refractory status epilepticus (SRSE) is defined as status epilepticus that persists or recurs after treatment with anesthetic agents for more than 24 hours, including cases with recurrent seizures on reduction or withdrawal of anesthetic drugs. Super-refractory status epilepticus presents a significant challenge for neurologists, particularly when standard treatments fail to achieve seizure control. Lacosamide, which has a unique mechanism involving modulating voltage-gated sodium channels by enhancing their slow inactivation, has emerged as a potential option for managing SRSE.
View Article and Find Full Text PDFIntroducing the Next Era in Assessment.
Perspect Med Educ
January 2025
A Professor at the Medical College of Wisconsin, Wisconsin, USA.
In this introduction, the guest editors of the "Next Era in Assessment" special collection frame the invited papers by envisioning a next era in assessment of medical education, based on ideas developed during a summit that convened professional and educational leaders and scholars. The authors posit that the next era of assessment will focus unambiguously on serving patients and the health of society, reflect its sociocultural context, and support learners' longitudinal growth and development. As such, assessment will be characterized as transformational, development-oriented and socially accountable.
View Article and Find Full Text PDFA simple phylogenetic approach to analyze hypermutated HIV proviruses reveals insights into their dynamics and persistence during antiretroviral therapy.
Virus Evol
November 2024
Faculty of Health Sciences, Simon Fraser University, 8888 University Drive, Burnaby, BC V5A 1S6, Canada.
Hypermutated proviruses, which arise in a single Human Immunodeficiency Virus (HIV) replication cycle when host antiviral APOBEC3 proteins introduce extensive guanine to adenine mutations throughout the viral genome, persist in all people living with HIV receiving antiretroviral therapy (ART). However, hypermutated sequences are routinely excluded from phylogenetic trees because their extensive mutations complicate phylogenetic inference, and as a result, we know relatively little about their within-host evolutionary origins and dynamics. Using >1400 longitudinal single-genome-amplified HIV sequences isolated from six women over a median of 18 years of follow-up-including plasma HIV RNA sequences collected over a median of 9 years between seroconversion and ART initiation, and >500 proviruses isolated over a median of 9 years on ART-we evaluated three approaches for masking hypermutation in nucleotide alignments.
View Article and Find Full Text PDFArguments against a "general and permanent" ban on pediatric intersex surgery: A response to Clune-Taylor.
Bioethics
January 2025
Institute of Social Sciences, Centre for Sociological and Anthropological Research, Belgrade, Serbia.
The paper offers a critical response to the proposed "dis/analogy" between the restriction of Jehovah's Witness parental right to refuse life-saving blood transfusions for their minor children and a "general" and "permanent" ban on "unnecessary" pediatric intersex surgery. The main argument of the analogy is "securing the patient's future autonomy." Feinberg's theory of rights is used to demonstrate that the proposed analogy is untenable.
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