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by de la Morena, M. Teresa, MD and Leonard, David, PhD and Torgerson, Troy R., MD, PhD and Cabral-Marques, Otavio, PhD and Slatter, Mary, MD and Aghamohammadi, Asghar, MD and Chandra, Sharat, MD and Murguia-Favela, Luis, MD and Bonilla, Francisco A., MD, PhD and Kanariou, Maria, MD and Damrongwatanasuk, Rongras, MD and Kuo, Caroline Y., MD and Dvorak, Christopher C., MD and Meyts, Isabelle, MD and Chen, Karin, MD and Kobrynski, Lisa, MD, MPH and Kapoor, Neena, MD and Richter, Darko, MD and DiGiovanni, Daniela, MD and Dhalla, Fatima, MD and Farmaki, Evangelia, MD and Speckmann, Carsten, MD and Español, Teresa, MD and Shcherbina, Anna, MD and Hanson, Imelda Celine, MD and Litzman, Jiri, MD and Routes, John M., MD and Wong, Melanie, MD, PhD and Fuleihan, Ramsay, MD and Seneviratne, Suranjith L., MD and Small, Trudy N., MD and Janda, Ales, MD and Bezrodnik, Liliana, MD and Seger, Reinhard, MD and Raccio, Andrea Gomez, MD and Edgar, J. David M., MD and Chou, Janet, MD and Abbott, Jordan K., MD and van Montfrans, Joris, MD and González-Granado, Luis Ignacio, MD and Bunin, Nancy, MD and Kutukculer, Necil, MD and Gray, Paul, MD and Seminario, Gisela, MD and Pasic, Srdjan, MD and Aquino, Victor, MD and Wysocki, Christian, MD, PhD and Abolhassani, Hassan, MD and Dorsey, Morna, MD and Cunningham-Rundles, Charlotte, MD, PhD and Knutsen, Alan P., MD and Sleasman, John, MD and Costa Carvalho, Beatriz Tavares, MD and Condino-Neto, Antonio, MD and Grunebaum, Eyal, MD and Chapel, Helen, MD and Ochs, Hans D., MD, PhD and Filipovich, Alexandra, MD and Cowan, Mort, MD and Gennery, Andrew, MD and Cant, Andrew, MD and Notarangelo, Luigi D., MD and Roifman, Chaim M., MD
Journal of Allergy and Clinical Immunology, The, ISSN 0091-6749, 2016, Volume 139, Issue 4, pp. 1282 - 1292
Background X-linked hyper-IgM syndrome (XHIGM) is a primary immunodeficiency with high morbidity and mortality compared with those seen in healthy subjects.... 
Allergy and Immunology | hematopoietic cell transplantation | CD40 ligand | primary immunodeficiency | defects in class-switch recombination | long-term outcomes | X-linked hyper-IgM syndrome | Karnofsky/Lansky scores | CD40 LIGAND DEFICIENCY | BONE-MARROW-TRANSPLANTATION | CHRONIC GRANULOMATOUS-DISEASE | WISKOTT-ALDRICH-SYNDROME | CLINICAL-FEATURES | IMMUNOLOGY | CHILDREN | DEFECTIVE EXPRESSION | PRIMARY IMMUNODEFICIENCY DISEASES | ALLERGY | EUROPEAN EXPERIENCE | T-CELLS | Hyper-IgM Immunodeficiency Syndrome - therapy | Follow-Up Studies | Humans | Middle Aged | Kaplan-Meier Estimate | Proportional Hazards Models | Child, Preschool | Infant | Male | Time | Hematopoietic Stem Cell Transplantation - mortality | Young Adult | Hyper-IgM Immunodeficiency Syndrome - mortality | Adolescent | Adult | Female | Retrospective Studies | Child | Cohort Studies | Transplantation | Hematopoietic stem cells | Analysis | Therapy | Disease | Liver | Central nervous system | Nervous system | Antiinfectives and antibacterials | Blood | Risk factors | Antibody response | Neurodegeneration | Bone marrow | Genetics | Diagnosis | Conditioning | Neutropenia | Binding | Wiskott-Aldrich syndrome | Immunoglobulins | Liver diseases | Mortality | Agammaglobulinemia | BCG | Cholangitis | Hazards | Regression analysis | Survival | Bile duct | Studies | Stem cells | Accident prevention | Ligands | Mutation | Protocol (computers) | Bile | Immunoglobulin M | Pneumonia | Transplants & implants | Physicians | Immunoglobulin G | Medical services | Prophylaxis | Infections | Malignancy | Defects | Respiratory tract | Allografts | Bacteria | Children | Age | Medical personnel | Bacterial infections | Complications | Blood cells | Hypersensitivity | Switching | Morbidity | Computer programs | Lymphocytes B | Aplasia | Collaboration | Medical prognosis | Health hazards | Cancer | Lansky scores | Karnofsky
Journal Article
Journal of Allergy and Clinical Immunology, The, ISSN 0091-6749, 2016, Volume 139, Issue 3, pp. 900 - 912.e7
Journal Article
Journal of Allergy and Clinical Immunology, The, ISSN 0091-6749, 2011, Volume 129, Issue 3, pp. 778 - 786
Background Patients with X-linked hyper-IgM syndrome (X-HIGM) due to CD40 ligand (CD40L) mutations are susceptible to fungal pathogens; however, the underlying... 
Allergy and Immunology | primary immunodeficiency | dendritic cells | CD40 ligand deficiency | X-linked hyper-IgM syndrome | fungal infections | IMMUNE-RESPONSE | STIMULATION | CD40 LIGAND | PRIMARY IMMUNODEFICIENCIES | IMMUNOLOGY | HUMAN MONOCYTES | DEFICIENCY | FUNGAL-INFECTIONS | DEFECTIVE EXPRESSION | ALLERGY | TOLL-LIKE RECEPTORS | T-CELLS | Candidiasis - genetics | Paracoccidioides - immunology | B7-2 Antigen - genetics | B7-1 Antigen - genetics | Cell Proliferation | Coculture Techniques | Dendritic Cells - immunology | Humans | Child, Preschool | Dendritic Cells - pathology | Hyper-IgM Immunodeficiency Syndrome, Type 1 - complications | Male | CD40 Ligand - immunology | Paracoccidioidomycosis - genetics | Cell Differentiation - genetics | Hyper-IgM Immunodeficiency Syndrome, Type 1 - genetics | Lymphocyte Activation - genetics | CD40 Ligand - metabolism | Th17 Cells - metabolism | Paracoccidioidomycosis - immunology | Dendritic Cells - drug effects | Female | Histocompatibility Antigens Class II - metabolism | Candidiasis - immunology | Dendritic Cells - virology | Child | Dendritic Cells - metabolism | Th17 Cells - pathology | Candida albicans - pathogenicity | Cytokines - metabolism | Gene Expression Regulation - genetics | Cells, Cultured | B7-1 Antigen - metabolism | Candida albicans - immunology | Mutation - genetics | Candidiasis - complications | Gene Expression Regulation - drug effects | B7-2 Antigen - metabolism | CD40 Ligand - genetics | Cell Differentiation - drug effects | Lymphocyte Activation - drug effects | Paracoccidioidomycosis - complications | Adolescent | Th17 Cells - immunology | Paracoccidioides - pathogenicity | Histocompatibility Antigens Class II - genetics | Hyper-IgM Immunodeficiency Syndrome, Type 1 - immunology | Dendritic cells | Medical colleges | Cell culture | Cell growth | Bacterial infections | Lymphocytes | T cell receptors | Fungal infections | Immune system | Cell proliferation | CD40 antigen | CD86 antigen | Helper cells | X chromosome | Lymphocytes T | Transforming growth factor- beta | CD40L protein | Fungi | Interleukin 4 | Interleukin 5 | Job's syndrome | Pathogens | CD80 antigen | Interleukin 12 | Interleukin 17 | Infection | Monocytes | Major histocompatibility complex | Interleukin 10 | gamma -Interferon | Histocompatibility antigen HLA | Mutation | Differentiation
Journal Article
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