Publications by authors named "K Masjosthusmann"
A neonatal female patient exhibited a congenital intricate vascular malformation affecting the liver, encompassing anomalies in the arterial, venous, and portal venous systems and notably including an aneurysm within the portal vein. The management strategy involved a staged endovascular approach, initially using retrograde embolization via the venous outflow tract. Subsequently, transarterial embolization was performed to address complications associated with pulmonary and portal hypertension.
View Article and Find Full Text PDFAntimicrobial peptides (AMPs) are crucial components of the innate immune system in various organisms, including humans. Beyond their direct antimicrobial effects, AMPs play essential roles in various physiological processes. They induce angiogenesis, promote wound healing, modulate immune responses, and serve as chemoattractants for immune cells.
View Article and Find Full Text PDFMultisystem inflammatory syndrome in children (MIS-C) shares several clinical and immunological features with Kawasaki Disease (KD) and pediatric hyperinflammation, but the immuno-phenotypic overlap among these clinical mimics is still incompletely understood. Here we analyzed serum samples from treatment-naïve patients with MIS-C (n = 31) and KD (n = 11), pediatric hyperinflammation (n = 13) and healthy controls (HC, n = 10) by proximity extension assay (PEA) to profile 184 blood biomarkers. Collectively, immunophenotypic overlap between MIS-C and hyperinflammation exceeds overlap with KD.
View Article and Find Full Text PDFArticle Synopsis
- The study investigates autoimmune lymphoproliferative syndrome (ALPS) and similar severe conditions characterized by lymphoproliferation and autoimmune cytopenias, aiming to better classify these disorders based on genetic and clinical features.
- Conducted in Germany with 431 children referred for ALPS evaluation, the study categorized patients based on specific criteria related to lymphoproliferation and associated immune deficiencies, with a median diagnostic age of about 9.8 years.
- Findings revealed that 55% of enrolled children were diagnosed with ALPS, and genetic assessments helped differentiate ALPS from autoimmune lymphoproliferative syndrome-like diseases, enhancing understanding and classification of these immune disorders.
Objective: Rheumatic diseases are characterized by different patterns of immune overactivation. This study investigated the correlation of whole blood type 1 interferon (IFN) stimulated gene (ISG), IL18, and CXCL9 expression with clinical disease activity in pediatric rheumatic diseases and assessed the required number of ISGs to be included in a composite type 1 IFN score.
Methods: Whole blood-derived RNA and clinical data were collected from 171 mostly pediatric patients with connective tissue diseases (CTDs), systemic autoinflammatory diseases (SAIDs), monogenic interferonopathies (IFNPs) and other inflammatory diseases, and from 38 controls.