Survival benefit and immune-related toxicities after hematopoietic stem cell transplantation in Krabbe disease: a systematic review and meta-analysis

BackgroundKrabbe disease is a rare, rapidly progressive leukodystrophy with high early mortality. Hematopoietic stem cell transplantation (HSCT) is the main disease-modifying intervention used in clinical practice and may act through donor-derived myel…

Distinct phenotypic clusters associated with progressive pulmonary fibrosis in anti-synthetase syndrome-associated interstitial lung disease

BackgroundThe clinical manifestations of anti-synthetase syndrome-associated interstitial lung disease (ASyS-ILD) are highly heterogeneous, and the mechanisms underlying its development and progression remain poorly understood. This study aimed to iden…

Do not mistake symptomatic improvement for disease modification: therapeutic endpoints in secondary lower-extremity lymphedema require inflammation–fibrosis anchoring

Secondary lower-extremity lymphedema has long been regarded primarily as a disorder of impaired lymphatic fluid return. However, growing evidence indicates that its core pathology is a lymph stasis-driven immune–stromal remodeling process characterized…

IL-17 ligand-targeting inhibitors in psoriatic arthritis: a focused systematic review and network meta-analysis of efficacy, safety, and certainty of evidence

BackgroundIL-17 pathway inhibitors are established treatments for active psoriatic arthritis (PsA), but comparative evidence within this therapeutic class remains limited. We compared the efficacy, safety, treatment rankings, and certainty of evidence …

Mitochondrial DNA in systemic lupus erythematosus: pathogenic mechanisms, clinical biomarkers, and precision therapeutic strategies

Mitochondrial DNA (mtDNA) is increasingly recognized as an active driver of immune dysregulation in systemic lupus erythematosus (SLE), yet most existing reviews treat it as a single damage signal rather than a multifunctional pathological mediator. Th…

Expanding the clinical spectrum of CD3γ deficiency: comprehensive characterization of adult-onset disease and integrated reevaluation of all reported patients

ObjectiveCD3γ deficiency is an ultrarare autosomal recessive inborn error of immunity characterized by immune dysregulation and variable immunodeficiency. To date, only 16 predominantly pediatric cases have been reported. Here, we describe two addition…

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