A5088248315- SARS-CoV-2 and COVID-19 Research
- Immunodeficiency and Autoimmune Disorders
- COVID-19 Clinical Research Studies
- Viral gastroenteritis research and epidemiology
- Parvovirus B19 Infection Studies
- Immune responses and vaccinations
- Respiratory viral infections research
- Blood disorders and treatments
- COVID-19 Impact on Reproduction
- Kawasaki Disease and Coronary Complications
- Autoimmune and Inflammatory Disorders Research
- Virus-based gene therapy research
- Congenital Diaphragmatic Hernia Studies
- Viral Infections and Immunology Research
- Ethics and Legal Issues in Pediatric Healthcare
- CAR-T cell therapy research
- Pneumocystis jirovecii pneumonia detection and treatment
- Viral-associated cancers and disorders
- Immune Cell Function and Interaction
National Institute of Allergy and Infectious Diseases
2020-2024
National Institutes of Health
2020-2024
Children's National
2020-2022
SARS-CoV-2 vaccination is recommended in patients with inborn errors of immunity (IEIs); however, little known about immunogenicity and safety these patients.
Immunocompromised patients, including those with inborn errors of immunity (IEI), may be at increased risk for severe or prolonged infections SARS-CoV-2 (Zhu et al. N Engl J Med. 382:727–33, 2020; Guan Minotti Infect. 81:e61–6, 2020). While antibody and T cell responses to structural proteins are well described in healthy convalescent donors, adaptive humoral cellular has not yet been characterized patients deficiency (Grifoni Cell. 181:1489–1501 e1415, Burbelo Long Nat 26:845–8, Braun...
Background Despite similar rates of infection, adults and children have markedly different morbidity mortality related to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Compared adults, infrequent manifestations infection but are uniquely at risk for the rare often Multisystem Inflammatory Syndrome in Children (MIS-C) following infection. We hypothesized that these differences presentation magnitude and/or antigen specificity SARS-CoV-2-specific T cell (CST) responses between...
Few studies have assessed hypomorphic variants in ZAP70, leading to profound, late-onset combined immunodeficiency. A previously healthy 21-year-old man presented with primary EBV B cell lymphoproliferative disease (LPD). He developed hemophagocytic lymphohistiocytosis treated rituximab, although LPD rapidly relapsed. subsequently underwent matched, unrelated donor hematopoietic transplantation complicated by fatal hepatic sinusoidal obstructive syndrome. The family history included profound...
Rationale Chronic norovirus infection (CNI) is a significant cause of morbidity and mortality in immunocompromised patients. We sought to determine clinical virologic features associated with CNI an emphasis on IEI. Methods Norovirus-positive stool samples from 88 patients at the NIH Clinical Center 2010 2023. Longitudinal data were collected. Results Forty-eight had CNI; 23 acute (ANI); 17 unclassifiable. Eighty-three percent underlying diagnosis IEI, including CID (N = 23), CVID 11), SCID...
Introduction Sarcoidosis is a granulomatous disease with an incidence of up to 18 in 100,000 some populations. Symptoms range from asymptomatic benign lesions organ failure, 90% patients presenting lung involvement. often diagnosis exclusion: inflammation, lymphadenopathy, and sterile granulomata without identifiable cause. Granuloma formation occurs primary immunodeficiencies, but the full landscape these deficiencies their association sarcoid not fully understood. Results We looked...
Somatic mosaicism is an important mechanism of immune dysregulation and explains a growing proportion the 70% patients with suspected IEI who lack molecular diagnosis. Identifying disease-causing somatic mutations challenging due to presence such variants in small subset cells, leading low variant allele fraction (VAF). We developed custom 71-gene capture-based panel for high-depth sequencing genes known or hypothesized be associated dominant dysregulation. Sequencing was performed samples...
Adoptive T cell immunotherapy has been used to restore immunity against multiple viral targets in immunocompromised patients after bone-marrow transplantation and proposed as a strategy for preventing coronavirus 2019 (COVID-19) this population. Ideally, expanded severe acute respiratory syndrome 2 (SARS-CoV-2)-virus-specific cells (CSTs) should demonstrate marked expansion, specificity, CD8+ skewing prior adoptive transfer. However, current methodologies using IL-4 + IL-7 result suboptimal...
Aggressive natural killer cell leukemia (ANKL) is a rare, aggressive hematologic malignancy which often presents as fulminant Epstein-Barr virus (EBV)- driven hemophagocytic lymphohistiocytosis (HLH). ANKL lacks distinct immunologic and morphologic signature, making early diagnosis particularly challenging. Here we present case of in patient presenting with EBV-HLH. After poor treatment response to the HLH-2004 protocol (etoposide dexamethasone), bone marrow biopsy demonstrated an atypical...
Abstract Background Chronic norovirus infection (CNI) causes significant morbidity in immunocompromised patients. No effective prevention or treatment currently exists. Methods Two patients with inborn errors of immunity, X-linked severe combined immunodeficiency (X-SCID) and DOCK8 deficiency, were followed longitudinally for clinical course, immune reconstitution, norovirus-specific T-cell (NST) response, B-cell antibody production. Samples obtained the peri-hematopoietic stem cell...
Noroviruses are an important viral cause of chronic diarrhea in immunocompromised individuals.
Background: Despite similar rates of infection, adults and children have markedly different morbidity mortality related to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Compared adults, infrequent manifestations infection but are uniquely at risk for the rare often Multisystem Inflammatory Syndrome in Children (MIS-C) following infection. The cause these differences presentation may relate host response between children. We thus set out measure SARS-CoV-2-specific T cell...