Immune Reconstitution Inflammatory Syndrome After Hematopoietic Stem Cell Transplantation in a FOXN1-deficient Patient

Name: Work Video:
Duration: 3 min 30 s
Osman Corbali; Betül Gemici Karaaslan; Sezin Aydemir; Pınar Önal; Yasemin Kendir Demirkol; Serdar Nepesov; Ayça Kıykım; Haluk Çokuğraş
{'id': 'https://openalex.org/W4372258403', 'doi': 'https://doi.org/10.1097/mph.0000000000002677', 'title': 'Immune Reconstitution Inflammatory Syndrome After Hematopoietic Stem Cell Transplantation in a FOXN1-deficient Patient', 'display_name': 'Immune Reconstitution Inflammatory Syndrome After Hematopoietic Stem Cell Transplantation in a FOXN1-deficient Patient', 'publication_year': 2023, 'publication_date': '2023-05-01', 'ids': {'openalex': 'https://openalex.org/W4372258403', 'doi': 'https://doi.org/10.1097/mph.0000000000002677', 'pmid': 'https://pubmed.ncbi.nlm.nih.gov/37146104'}, 'language': 'en', 'primary_location': {'is_oa': False, 'landing_page_url': 'https://doi.org/10.1097/mph.0000000000002677', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S15952725', 'display_name': 'Journal of Pediatric Hematology/Oncology', 'issn_l': '1077-4114', 'issn': ['1077-4114', '1536-3678'], 'is_oa': False, 'is_in_doaj': False, 'is_core': True, 'host_organization': 'https://openalex.org/P4310315671', 'host_organization_name': 'Lippincott Williams & Wilkins', 'host_organization_lineage': ['https://openalex.org/P4310315671', 'https://openalex.org/P4310318547'], 'host_organization_lineage_names': ['Lippincott Williams & Wilkins', 'Wolters Kluwer'], 'type': 'journal'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}, 'type': 'article', 'type_crossref': 'journal-article', 'indexed_in': ['crossref', 'pubmed'], 'open_access': {'is_oa': False, 'oa_status': 'closed', 'oa_url': None, 'any_repository_has_fulltext': False}, 'authorships': [{'author_position': 'first', 'author': {'id': 'https://openalex.org/A5019422051', 'display_name': 'Osman Corbali', 'orcid': 'https://orcid.org/0000-0002-3320-1998'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': True, 'raw_author_name': 'Osman Corbali', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine', 'institution_ids': ['https://openalex.org/I4210112471']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5060099013', 'display_name': 'Betül Gemici Karaaslan', 'orcid': 'https://orcid.org/0000-0002-0303-7146'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Hatice Betul Gemici Karaaslan', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy', 'institution_ids': ['https://openalex.org/I4210112471']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5028857015', 'display_name': 'Sezin Aydemir', 'orcid': 'https://orcid.org/0000-0002-5876-2601'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Sezin Aydemir', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy', 'institution_ids': ['https://openalex.org/I4210112471']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5069274160', 'display_name': 'Pınar Önal', 'orcid': 'https://orcid.org/0000-0002-7143-3132'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Pinar Onal', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Infectious Diseases'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Infectious Diseases', 'institution_ids': ['https://openalex.org/I4210112471']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5013774390', 'display_name': 'Yasemin Kendir Demirkol', 'orcid': 'https://orcid.org/0000-0001-8016-5224'}, 'institutions': [{'id': 'https://openalex.org/I170153556', 'display_name': 'University of Health Science', 'ror': 'https://ror.org/04jpmg381', 'country_code': 'KH', 'type': 'education', 'lineage': ['https://openalex.org/I170153556']}, {'id': 'https://openalex.org/I4392021132', 'display_name': 'University of Health Sciences', 'ror': 'https://ror.org/00etaks59', 'country_code': None, 'type': 'education', 'lineage': ['https://openalex.org/I4392021132']}], 'countries': ['KH'], 'is_corresponding': False, 'raw_author_name': 'Yasemin Kendir Demirkol', 'raw_affiliation_strings': ['University of Health Sciences, Umraniye Training and Research Hospital'], 'affiliations': [{'raw_affiliation_string': 'University of Health Sciences, Umraniye Training and Research Hospital', 'institution_ids': ['https://openalex.org/I170153556', 'https://openalex.org/I4392021132']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5031249944', 'display_name': 'Serdar Nepesov', 'orcid': 'https://orcid.org/0000-0002-4551-5433'}, 'institutions': [{'id': 'https://openalex.org/I3125470973', 'display_name': 'Istanbul Medipol University', 'ror': 'https://ror.org/037jwzz50', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I3125470973']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Serdar Nepesov', 'raw_affiliation_strings': ['Istanbul Medipol University, Pediatric Immunology and Allergy, Istanbul, Turkey'], 'affiliations': [{'raw_affiliation_string': 'Istanbul Medipol University, Pediatric Immunology and Allergy, Istanbul, Turkey', 'institution_ids': ['https://openalex.org/I3125470973']}]}, {'author_position': 'middle', 'author': {'id': 'https://openalex.org/A5030953316', 'display_name': 'Ayça Kıykım', 'orcid': 'https://orcid.org/0000-0001-5821-3963'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Ayca Kiykim', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy', 'institution_ids': ['https://openalex.org/I4210112471']}]}, {'author_position': 'last', 'author': {'id': 'https://openalex.org/A5010274078', 'display_name': 'Haluk Çokuğraş', 'orcid': 'https://orcid.org/0000-0002-0086-3936'}, 'institutions': [{'id': 'https://openalex.org/I4210112471', 'display_name': 'Istanbul University-Cerrahpaşa', 'ror': 'https://ror.org/01dzn5f42', 'country_code': 'TR', 'type': 'education', 'lineage': ['https://openalex.org/I4210112471']}], 'countries': ['TR'], 'is_corresponding': False, 'raw_author_name': 'Haluk Cokugras', 'raw_affiliation_strings': ['Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy', 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Infectious Diseases'], 'affiliations': [{'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Immunology and Allergy', 'institution_ids': ['https://openalex.org/I4210112471']}, {'raw_affiliation_string': 'Istanbul University-Cerrahpasa, Cerrahpasa School of Medicine, Pediatric Infectious Diseases', 'institution_ids': ['https://openalex.org/I4210112471']}]}], 'institution_assertions': [], 'countries_distinct_count': 2, 'institutions_distinct_count': 4, 'corresponding_author_ids': ['https://openalex.org/A5019422051'], 'corresponding_institution_ids': ['https://openalex.org/I4210112471'], 'apc_list': None, 'apc_paid': None, 'fwci': 0.637, 'has_fulltext': False, 'cited_by_count': 2, 'citation_normalized_percentile': {'value': 0.999913, 'is_in_top_1_percent': True, 'is_in_top_10_percent': True}, 'cited_by_percentile_year': {'min': 79, 'max': 84}, 'biblio': {'volume': '45', 'issue': '5', 'first_page': '275', 'last_page': '277'}, 'is_retracted': False, 'is_paratext': False, 'primary_topic': {'id': 'https://openalex.org/T11725', 'display_name': 'Immunodeficiency and Autoimmune Disorders', 'score': 0.9878, 'subfield': {'id': 'https://openalex.org/subfields/2403', 'display_name': 'Immunology'}, 'field': {'id': 'https://openalex.org/fields/24', 'display_name': 'Immunology and Microbiology'}, 'domain': {'id': 'https://openalex.org/domains/1', 'display_name': 'Life Sciences'}}, 'topics': [{'id': 'https://openalex.org/T11725', 'display_name': 'Immunodeficiency and Autoimmune Disorders', 'score': 0.9878, 'subfield': {'id': 'https://openalex.org/subfields/2403', 'display_name': 'Immunology'}, 'field': {'id': 'https://openalex.org/fields/24', 'display_name': 'Immunology and Microbiology'}, 'domain': {'id': 'https://openalex.org/domains/1', 'display_name': 'Life Sciences'}}, {'id': 'https://openalex.org/T12226', 'display_name': 'Neuroblastoma Research and Treatments', 'score': 0.9845, 'subfield': {'id': 'https://openalex.org/subfields/2728', 'display_name': 'Neurology'}, 'field': {'id': 'https://openalex.org/fields/27', 'display_name': 'Medicine'}, 'domain': {'id': 'https://openalex.org/domains/4', 'display_name': 'Health Sciences'}}, {'id': 'https://openalex.org/T11591', 'display_name': 'NF-κB Signaling Pathways', 'score': 0.9815, 'subfield': {'id': 'https://openalex.org/subfields/1306', 'display_name': 'Cancer Research'}, 'field': {'id': 'https://openalex.org/fields/13', 'display_name': 'Biochemistry, Genetics and Molecular Biology'}, 'domain': {'id': 'https://openalex.org/domains/1', 'display_name': 'Life Sciences'}}], 'keywords': [{'id': 'https://openalex.org/keywords/immune-dysregulation', 'display_name': 'Immune Dysregulation', 'score': 0.5697381}, {'id': 'https://openalex.org/keywords/primary-immunodeficiency', 'display_name': 'Primary Immunodeficiency', 'score': 0.5576303}], 'concepts': [{'id': 'https://openalex.org/C2777408962', 'wikidata': 'https://www.wikidata.org/wiki/Q1128050', 'display_name': 'Hematopoietic stem cell transplantation', 'level': 3, 'score': 0.77274305}, {'id': 'https://openalex.org/C2777607303', 'wikidata': 'https://www.wikidata.org/wiki/Q641307', 'display_name': 'Immunodeficiency', 'level': 3, 'score': 0.7157929}, {'id': 'https://openalex.org/C2779468541', 'wikidata': 'https://www.wikidata.org/wiki/Q1334408', 'display_name': 'Severe combined immunodeficiency', 'level': 3, 'score': 0.7059644}, {'id': 'https://openalex.org/C203014093', 'wikidata': 'https://www.wikidata.org/wiki/Q101929', 'display_name': 'Immunology', 'level': 1, 'score': 0.6059496}, {'id': 'https://openalex.org/C8891405', 'wikidata': 'https://www.wikidata.org/wiki/Q1059', 'display_name': 'Immune system', 'level': 2, 'score': 0.5972113}, {'id': 'https://openalex.org/C2780194698', 'wikidata': 'https://www.wikidata.org/wiki/Q6005429', 'display_name': 'Immune dysregulation', 'level': 3, 'score': 0.5697381}, {'id': 'https://openalex.org/C2779019163', 'wikidata': 'https://www.wikidata.org/wiki/Q3043160', 'display_name': 'Primary immunodeficiency', 'level': 3, 'score': 0.5576303}, {'id': 'https://openalex.org/C71924100', 'wikidata': 'https://www.wikidata.org/wiki/Q11190', 'display_name': 'Medicine', 'level': 0, 'score': 0.5416783}, {'id': 'https://openalex.org/C2911091166', 'wikidata': 'https://www.wikidata.org/wiki/Q106419912', 'display_name': 'Transplantation', 'level': 2, 'score': 0.5199467}, {'id': 'https://openalex.org/C28328180', 'wikidata': 'https://www.wikidata.org/wiki/Q48196', 'display_name': 'Stem cell', 'level': 2, 'score': 0.49549758}, {'id': 'https://openalex.org/C109159458', 'wikidata': 'https://www.wikidata.org/wiki/Q919283', 'display_name': 'Haematopoiesis', 'level': 3, 'score': 0.4343664}, {'id': 'https://openalex.org/C86803240', 'wikidata': 'https://www.wikidata.org/wiki/Q420', 'display_name': 'Biology', 'level': 0, 'score': 0.16672787}, {'id': 'https://openalex.org/C126322002', 'wikidata': 'https://www.wikidata.org/wiki/Q11180', 'display_name': 'Internal medicine', 'level': 1, 'score': 0.14593932}, {'id': 'https://openalex.org/C104317684', 'wikidata': 'https://www.wikidata.org/wiki/Q7187', 'display_name': 'Gene', 'level': 2, 'score': 0.09334585}, {'id': 'https://openalex.org/C54355233', 'wikidata': 'https://www.wikidata.org/wiki/Q7162', 'display_name': 'Genetics', 'level': 1, 'score': 0.062227696}], 'mesh': [{'descriptor_ui': 'D018380', 'descriptor_name': 'Hematopoietic Stem Cell Transplantation', 'qualifier_ui': 'Q000009', 'qualifier_name': 'adverse effects', 'is_major_topic': False}, {'descriptor_ui': 'D006801', 'descriptor_name': 'Humans', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D054019', 'descriptor_name': 'Immune Reconstitution Inflammatory Syndrome', 'qualifier_ui': 'Q000209', 'qualifier_name': 'etiology', 'is_major_topic': False}, {'descriptor_ui': 'D007223', 'descriptor_name': 'Infant', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D008297', 'descriptor_name': 'Male', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D010641', 'descriptor_name': 'Phenotype', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}, {'descriptor_ui': 'D016511', 'descriptor_name': 'Severe Combined Immunodeficiency', 'qualifier_ui': 'Q000235', 'qualifier_name': 'genetics', 'is_major_topic': False}, {'descriptor_ui': 'D013950', 'descriptor_name': 'Thymus Gland', 'qualifier_ui': '', 'qualifier_name': None, 'is_major_topic': False}], 'locations_count': 2, 'locations': [{'is_oa': False, 'landing_page_url': 'https://doi.org/10.1097/mph.0000000000002677', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S15952725', 'display_name': 'Journal of Pediatric Hematology/Oncology', 'issn_l': '1077-4114', 'issn': ['1077-4114', '1536-3678'], 'is_oa': False, 'is_in_doaj': False, 'is_core': True, 'host_organization': 'https://openalex.org/P4310315671', 'host_organization_name': 'Lippincott Williams & Wilkins', 'host_organization_lineage': ['https://openalex.org/P4310315671', 'https://openalex.org/P4310318547'], 'host_organization_lineage_names': ['Lippincott Williams & Wilkins', 'Wolters Kluwer'], 'type': 'journal'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}, {'is_oa': False, 'landing_page_url': 'https://pubmed.ncbi.nlm.nih.gov/37146104', 'pdf_url': None, 'source': {'id': 'https://openalex.org/S4306525036', 'display_name': 'PubMed', 'issn_l': None, 'issn': None, 'is_oa': False, 'is_in_doaj': False, 'is_core': False, 'host_organization': 'https://openalex.org/I1299303238', 'host_organization_name': 'National Institutes of Health', 'host_organization_lineage': ['https://openalex.org/I1299303238'], 'host_organization_lineage_names': ['National Institutes of Health'], 'type': 'repository'}, 'license': None, 'license_id': None, 'version': None, 'is_accepted': False, 'is_published': False}], 'best_oa_location': None, 'sustainable_development_goals': [], 'grants': [], 'datasets': [], 'versions': [], 'referenced_works_count': 13, 'referenced_works': ['https://openalex.org/W1544884551', 'https://openalex.org/W1998522750', 'https://openalex.org/W2000180220', 'https://openalex.org/W2035918292', 'https://openalex.org/W2055859215', 'https://openalex.org/W2095711814', 'https://openalex.org/W2103130332', 'https://openalex.org/W2164313931', 'https://openalex.org/W2755773502', 'https://openalex.org/W2929206389', 'https://openalex.org/W2949075486', 'https://openalex.org/W3137342725', 'https://openalex.org/W3161056637'], 'related_works': ['https://openalex.org/W4301420608', 'https://openalex.org/W4233365492', 'https://openalex.org/W3180336193', 'https://openalex.org/W3099479999', 'https://openalex.org/W2918309724', 'https://openalex.org/W2766474652', 'https://openalex.org/W2581030728', 'https://openalex.org/W2408872566', 'https://openalex.org/W2003568505', 'https://openalex.org/W127566301'], 'abstract_inverted_index': {'The': [0], 'FOXN1': [1, 39, 64, 119], 'gene': [2], 'mutation': [3, 65], 'is': [4, 27, 34, 45], 'a': [5, 58, 62, 113], 'unique': [6], 'disorder': [7], 'that': [8], 'causes': [9], 'the': [10, 35, 42, 54, 102], 'nude': [11], 'severe': [12, 19], 'combined': [13, 20], 'immunodeficiency': [14], 'phenotype.': [15], 'In': [16, 49], 'patients': [17, 117], 'with': [18, 61, 67, 118], 'immunodeficiency,': [21], 'hematopoietic': [22], 'stem': [23], 'cell': [24], 'transplantation': [25, 33], '(HSCT)': [26], 'life-saving': [28], 'if': [29], 'performed': [30], 'earlier.': [31], 'Thymic': [32], 'curative': [36], 'treatment': [37, 114], 'for': [38], 'deficiency': [40], 'because': [41], 'main': [43], 'pathology': [44], 'thymic': [46], 'stromal': [47], 'changes.': [48], 'this': [50], 'report,': [51], 'we': [52, 96], 'describe': [53], 'clinical': [55], 'features': [56], 'of': [57, 104], 'Turkish': [59], 'patient': [60], 'homozygous': [63], 'treated': [66], 'HSCT': [68, 105], 'from': [69], 'his': [70], 'human': [71], 'leukocyte': [72], 'antigen-matched': [73], 'sibling.': [74], 'On': [75], 'follow-up,': [76], 'he': [77], 'showed': [78], 'Bacille': [79], 'Calmette': [80], 'Guerin': [81], 'adenitis': [82], 'and': [83, 106], 'was': [84], 'evaluated': [85], 'as': [86, 112], 'having': [87], 'immune': [88, 108], 'reconstitution': [89, 109], 'inflammatory': [90, 110], 'syndrome.': [91], 'By': [92], 'presenting': [93], 'our': [94], 'patient,': [95], 'aimed': [97], 'to': [98, 101], 'draw': [99], 'attention': [100], 'development': [103], 'subsequent': [107], 'syndrome': [111], 'option': [115], 'in': [116], 'deficiency.': [120]}, 'cited_by_api_url': 'https://api.openalex.org/works?filter=cites:W4372258403', 'counts_by_year': [{'year': 2024, 'cited_by_count': 1}, {'year': 2023, 'cited_by_count': 1}], 'updated_date': '2024-12-24T21:12:36.929081', 'created_date': '2023-05-07'}
Publication Information

Basic Information

Access and Citation

AI Researcher Chatbot

Primary Location

Authors

Topics

Keywords

Related Works
