A5057494233- Immunodeficiency and Autoimmune Disorders
- Inflammasome and immune disorders
- Blood disorders and treatments
- Viral Infections and Vectors
- Hematopoietic Stem Cell Transplantation
- Autoimmune and Inflammatory Disorders Research
- Eosinophilic Disorders and Syndromes
- Blood groups and transfusion
Dmitry Rogachev National Research Center of Pediatric Hematology, Oncology and Immunology
2021-2024
Ministry of Health of the Russian Federation
2022
Subcutaneous immunoglobulins (SCIgs) have been widely used in patients with inborn errors of immunity (IEIs) and shown to be effective well tolerated. Here, we assessed efficacy safety replacement therapy SCIgs a large heterogeneous cohort IEIs. The study was approved by the Independent Ethics Committee Scientific Council Dmitry Rogachev National Medical Research Center Pediatric Hematology, Oncology Immunology Ministry Healthcare Russia. All and/or their legal representatives signed...
The aim of this study was to analyze the clinical, laboratory and molecular genetic data 20 patients (9 boys, 11 girls) diagnosed with cryopyrin-associated periodic syndrome (CAPS) an assessment efficacy safety therapy in 6 inhibitor interleukin-1 receptor – anakinra. approved by Independent Ethics Committee Scientific Council D. Rogachev NMRCPHOI. patients' parents gave their consent use child's data, including photographs, for research purposes publications. age CAPS manifestation ranged...
The aim of this study was to analyze the clinical, laboratory and molecular genetic data 26 patients (15 boys, 11 girls) diagnosed with mevalonate kinase deficiency syndrome (MKD). Subjects methods . age MKD manifestation ranged from 0 30.0 months (M – 1.5 months). Clinical manifestations their severity were extremely diverse: symptoms resembling Marshall’s severe systemic respiratory failure, hepatosplenomegaly pancytopenia. Results/Conclusion All had homozygous/compound-heterozygous...
The article describes a clinical case of patient with Wiskott–Aldrich syndrome, in whom long- term persistence mixed chimerism was determined after hematopoietic stem cell transplantation (HSCT) from haploidentical donor. Based on the analysis patient's picture HSCT, it shown that presence of> 50% donor cells myeloid lineage is necessary for correction thrombocytopenia. In addition, B-lymphocytes possibly contributed to development autoimmune complications patient, as well persistent...