{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T01:54:14Z","timestamp":1778810054516,"version":"3.51.4"},"reference-count":136,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2025,11,29]],"date-time":"2025-11-29T00:00:00Z","timestamp":1764374400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>Background: Inborn errors of immunity (IEIs) are a heterogeneous group of genetically determined disorders that lead to immune dysfunction, recurrent infections, and organ-specific complications. The lungs are among the most commonly affected organs, with both infectious and noninfectious manifestations that significantly contribute to morbidity and mortality. This study aimed to provide a comprehensive overview of pulmonary manifestations in IEI, with emphasis on pathophysiological mechanisms, diagnostic approaches, and therapeutic strategies. Methods: A narrative review and synthesis of current literature and clinical guidelines were conducted, focusing on pulmonary involvement in IEI as classified by the International Union of Immunological Societies (IUIS). The analysis included data on infectious and noninfectious complications, imaging findings, immunological assessments, and management strategies, supported by clinical evidence and expert consensus. Results: Pulmonary manifestations in IEI encompass a wide spectrum of conditions. Infectious complications include recurrent bacterial pneumonias, bronchitis, and opportunistic infections, frequently resulting in irreversible lung damage such as bronchiectasis. Noninfectious complications, including granulomatous\u2013lymphocytic interstitial lung disease (GLILD) and interstitial lung disease (ILD), are common in disorders such as common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA). Early diagnosis using high-resolution computed tomography (HRCT) and immunological testing, combined with the timely initiation of immunoglobulin replacement therapy and anti-biotic prophylaxis, significantly improves prognosis. Conclusions: Pulmonary complications are key clinical indicators of IEI and often precede systemic manifestations. Early, integrated, and interdisciplinary diagnostic and therapeutic management are crucial for preventing irreversible lung damage and improving patient outcomes. Regular monitoring and individualized therapy, including immunoglobulin replacement, targeted immunosuppression, and vaccination, are the cornerstones of effective long-term care in IEI.<\/jats:p>","DOI":"10.3390\/life15121838","type":"journal-article","created":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T08:49:22Z","timestamp":1764665362000},"page":"1838","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pulmonary Manifestations of Inborn Errors of Immunity: Diagnostic and Therapeutic Insights"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2202-3222","authenticated-orcid":false,"given":"Katarzyna","family":"Napiorkowska-Baran","sequence":"first","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"},{"name":"Department of Pulmonology, Allergology and Pulmonary Oncology, Poznan University of Medical Sciences, 61-701 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Szczepan","family":"Cofta","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"},{"name":"Department of Pulmonology, Allergology and Pulmonary Oncology, Poznan University of Medical Sciences, 61-701 Poznan, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8407-4642","authenticated-orcid":false,"given":"Pawe\u0142","family":"Treichel","sequence":"additional","affiliation":[{"name":"Doctoral School of Medical and Health Sciences, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University,\u00a087-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marta","family":"Tykwinska","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2651-8468","authenticated-orcid":false,"given":"Kinga","family":"Lis","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4038-5016","authenticated-orcid":false,"given":"Aleksandra","family":"Matyja-Bednarczyk","sequence":"additional","affiliation":[{"name":"Center for Transplantology and Interstitial Lung Diseases, Jagiellonian University Medical College, 31-008 Krakow, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-4374-5331","authenticated-orcid":false,"given":"Bart\u0142omiej","family":"Szymczak","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Jan Biziel University Hospital No. 2 in Bydgoszcz, 85-168 Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maciej","family":"Szota","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"},{"name":"Doctoral School of Medical and Health Sciences, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University,\u00a087-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jozef","family":"Slawatycki","sequence":"additional","affiliation":[{"name":"Doctoral School of Medical and Health Sciences, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University,\u00a087-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michal","family":"Kulakowski","sequence":"additional","affiliation":[{"name":"Clinical Department of Orthopedics and Traumatology, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University,\u00a087-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8328-1386","authenticated-orcid":false,"given":"Zbigniew","family":"Bartuzi","sequence":"additional","affiliation":[{"name":"Department of Allergology, Clinical Immunology and Internal Diseases, Collegium Medicum Bydgoszcz, Nicolaus Copernicus University, 87-100 Torun, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,11,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1016\/j.jaci.2022.08.024","article-title":"The Lung in Inborn Errors of Immunity: From Clinical Disease Patterns to Molecular Pathogenesis","volume":"150","author":"Gutierrez","year":"2022","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s12016-019-08756-7","article-title":"The Epidemiology and Clinical Manifestations of Autoimmunity in Selective IgA Deficiency","volume":"58","author":"Odineal","year":"2020","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"e20250003","DOI":"10.70962\/jhi.20250003","article-title":"Human Inborn Errors of Immunity: 2024 Update on the Classification from the International Union of Immunological Societies Expert Committee","volume":"1","author":"Poli","year":"2025","journal-title":"J. Hum. Immun."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/s12016-021-08916-8","article-title":"Future of Therapy for Inborn Errors of Immunity","volume":"63","author":"Perez","year":"2022","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1186\/s12931-018-0923-8","article-title":"Primary Immunodeficiency Diseases in Lung Disease: Warning Signs, Diagnosis and Management","volume":"19","year":"2018","journal-title":"Respir. Res."},{"key":"ref_6","unstructured":"(2025, September 23). Human Inborn Errors of Immunity: An Expanding Universe | Science Immunology. Available online: https:\/\/www.science.org\/doi\/abs\/10.1126\/sciimmunol.abb1662."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"e20250002","DOI":"10.70962\/jhi.20250002","article-title":"The 2024 Update of IUIS Phenotypic Classification of Human Inborn Errors of Immunity","volume":"1","author":"Bousfiha","year":"2025","journal-title":"J. Hum. Immun."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1602","DOI":"10.1016\/j.jaip.2023.04.018","article-title":"Inborn Errors of Immunity and Autoimmune Disease","volume":"11","author":"Gray","year":"2023","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Esmaeilzadeh, H., Esmaeilbeig, M., Askarisarvestani, A., Alyasin, S., and Nabavizadeh, S.H. (2025). The Prevalence of Allergic Manifestations in Inborn Errors of Immunity: A Retrospective Cohort Study. BMC Immunol., 26.","DOI":"10.1186\/s12865-025-00758-9"},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Tiri, A., Masetti, R., Conti, F., Tignanelli, A., Turrini, E., Bertolini, P., Esposito, S., and Pession, A. (2021). Inborn Errors of Immunity and Cancer. Biology, 10.","DOI":"10.3390\/biology10040313"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e833","DOI":"10.1002\/iid3.833","article-title":"Organ-Based Clues for Diagnosis of Inborn Errors of Immunity: A Practical Guide for Clinicians","volume":"11","author":"Mohammadi","year":"2023","journal-title":"Immun. Inflamm. Dis."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Napi\u00f3rkowska-Baran, K. (2025). Evaluation of the Asthmatic Patient for Primary and Secondary Immunodeficiencies and Importance of Vaccinations in Asthma. Asthma\u2014Diagnosis, Management and Comorbidities, ResearchGate.","DOI":"10.5772\/intechopen.1011041"},{"key":"ref_13","unstructured":"(2025, September 23). Leading the Mission to Cure Primary Immunodeficiency (PI). Available online: https:\/\/info4pi.org\/."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"S39","DOI":"10.1016\/j.jped.2020.10.014","article-title":"Combined Immunodeficiencies","volume":"97","author":"Aranda","year":"2021","journal-title":"J. Pediatr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"5510","DOI":"10.4049\/jimmunol.1102243","article-title":"Cutting Edge: Tissue-Retentive Lung Memory CD4 T Cells Mediate Optimal Protection to Respiratory Virus Infection","volume":"187","author":"Teijaro","year":"2011","journal-title":"J. Immunol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1038\/mi.2013.67","article-title":"Lung Niches for the Generation and Maintenance of Tissue-Resident Memory T Cells","volume":"7","author":"Turner","year":"2014","journal-title":"Mucosal Immunol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1111\/imr.12963","article-title":"Classical CD4 T Cells as the Cornerstone of Antimycobacterial Immunity","volume":"301","author":"Morgan","year":"2021","journal-title":"Immunol. Rev."},{"key":"ref_18","first-page":"e47759","article-title":"Severe Combined Immunodeficiency (SCID) and Its New Treatment Modalities","volume":"15","author":"Wadbudhe","year":"2023","journal-title":"Cureus"},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"George, K., and Govindaraj, G. (2023). Infections in Inborn Errors of Immunity with Combined Immune Deficiency: A Review. Pathogens, 12.","DOI":"10.3390\/pathogens12020272"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Funato, N. (2022). Craniofacial Phenotypes and Genetics of DiGeorge Syndrome. J. Dev. Biol., 10.","DOI":"10.3390\/jdb10020018"},{"key":"ref_21","first-page":"52","article-title":"Predominantly Antibody Deficiencies","volume":"1","author":"Azizi","year":"2018","journal-title":"Immunol. Genet. J."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/j.autrev.2016.07.011","article-title":"Autoimmunity and Infection in Common Variable Immunodeficiency (CVID)","volume":"15","author":"Patuzzo","year":"2016","journal-title":"Autoimmun. Rev."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"103442","DOI":"10.1016\/j.autrev.2023.103442","article-title":"Autoimmune Lymphoproliferative Syndrome: A Disorder of Immune Dysregulation","volume":"22","author":"Paskiewicz","year":"2023","journal-title":"Autoimmun. Rev."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1111\/nyas.13011","article-title":"From IPEX Syndrome to FOXP3 Mutation: A Lesson on Immune Dysregulation","volume":"1417","author":"Bacchetta","year":"2018","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"299","DOI":"10.2500\/aap.2024.45.240049","article-title":"Immunodeficiency: Quantitative and Qualitative Phagocytic Cell Defects","volume":"45","author":"Mickey","year":"2024","journal-title":"Allergy Asthma Proc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1111\/pai.12654","article-title":"Modern Management of Phagocyte Defects","volume":"28","author":"Lanini","year":"2017","journal-title":"Pediatr. Allergy Immunol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1111\/nyas.12763","article-title":"Toll-like Receptor Signaling in Primary Immune Deficiencies","volume":"1356","author":"Maglione","year":"2015","journal-title":"Ann. N. Y. Acad. Sci."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"e20220202","DOI":"10.1084\/jem.20220202","article-title":"Respiratory Viral Infections in Otherwise Healthy Humans with Inherited IRF7 Deficiency","volume":"219","author":"Campbell","year":"2022","journal-title":"J. Exp. Med."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"307ra154","DOI":"10.1126\/scitranslmed.aac4227","article-title":"Human IFNAR2 Deficiency: Lessons for Antiviral Immunity","volume":"7","author":"Duncan","year":"2015","journal-title":"Sci. Transl. Med."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1111\/odi.12448","article-title":"Genetically Defined Autoinflammatory Diseases","volume":"22","year":"2016","journal-title":"Oral Dis."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"102979","DOI":"10.1016\/j.jaut.2022.102979","article-title":"The Complement System and Human Autoimmune Diseases","volume":"137","author":"Coss","year":"2023","journal-title":"J. Autoimmun."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"49","DOI":"10.5045\/br.2022.2022012","article-title":"Overview of Inherited Bone Marrow Failure Syndromes","volume":"57","author":"Park","year":"2022","journal-title":"Blood Res."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Aghamohammadi, A., Abolhassani, H., Rezaei, N., and Yazdani, R. (2021). Chapter 10\u2014Phenocopies of Inborn Errors of Immunity. Inborn Errors of Immunity, Academic Press.","DOI":"10.1016\/B978-0-12-821028-4.00001-4"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1002\/ppul.24599","article-title":"Bronchiectasis in Common Variable Immunodeficiency: A Systematic Review and Meta-Analysis","volume":"55","author":"Ramzi","year":"2020","journal-title":"Pediatr. Pulmonol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.4187\/respcare.04479","article-title":"Sinopulmonary Complications in Subjects With Primary Immunodeficiency","volume":"61","author":"Owayed","year":"2016","journal-title":"Respir. Care"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"465","DOI":"10.2500\/aap.2019.40.4273","article-title":"Approach to Primary Immunodeficiency","volume":"40","author":"Devonshire","year":"2019","journal-title":"Allergy Asthma Proc."},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Naporowski, P., Witkowska, D., Lewandowicz-Uszy\u0144ska, A., and Gamian, A. (2018). Bacterial, viral and fungal infections in primary immunodeficiency patients. Post\u0119py Higieny i Medycyny Do\u015bwiadczalnej, 72. 557\u2013572.","DOI":"10.5604\/01.3001.0012.1734"},{"key":"ref_38","first-page":"387","article-title":"Microbial Isolates and Antimicrobial Resistance Patterns in Adults with Inborn Errors of Immunity: A Retrospective Longitudinal Analysis of Sputum Cultures","volume":"186","author":"Karabiber","year":"2024","journal-title":"Int. Arch. Allergy Immunol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"689","DOI":"10.18295\/2075-0528.2896","article-title":"Causes of Mortality in Patients with Inborn Errors of Immunity: An 18-Year Retrospective Cohort Study","volume":"25","author":"Jabri","year":"2025","journal-title":"Sultan Qaboos Univ. Med. J."},{"key":"ref_40","doi-asserted-by":"crossref","unstructured":"Costagliola, G., and Consolini, R. (2023). Primary Immunodeficiencies: Pathogenetic Advances, Diagnostic and Management Challenges. J. Clin. Med., 12.","DOI":"10.3390\/jcm12144651"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1007\/s10875-019-00642-3","article-title":"Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity","volume":"39","author":"Bustamante","year":"2019","journal-title":"J. Clin. Immunol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"4142","DOI":"10.1007\/s00330-023-10334-7","article-title":"Primary Immunodeficiency Diseases of Adults: A Review of Pulmonary Complication Imaging Findings","volume":"34","author":"Grenier","year":"2024","journal-title":"Eur. Radiol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"72","DOI":"10.56164\/PediatrRespirJ.2024.44","article-title":"How Primary Immune Deficiencies Impact the Lung in Childrens","volume":"2","author":"Plebani","year":"2024","journal-title":"Pediatr. Respir. J."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1097\/RTI.0000000000000350","article-title":"Pulmonary Manifestations of Common Variable Immunodeficiency","volume":"33","author":"Bang","year":"2018","journal-title":"J. Thorac. Imaging"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"572","DOI":"10.1007\/s10875-022-01206-8","article-title":"Predictive Factors for and Complications of Bronchiectasis in Common Variable Immunodeficiency Disorders","volume":"42","author":"Sperlich","year":"2022","journal-title":"J. Clin. Immunol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.pcl.2020.09.004","article-title":"Pulmonary Manifestations of Immunodeficiency and Immunosuppressive Diseases Other than Human Immunodeficiency Virus","volume":"68","author":"Pacheco","year":"2021","journal-title":"Pediatr. Clin. N. Am."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1613","DOI":"10.1183\/09031936.00118414","article-title":"Pulmonary Manifestations in Adult Patients with Chronic Granulomatous Disease","volume":"45","author":"Salvator","year":"2015","journal-title":"Eur. Respir. J."},{"key":"ref_48","doi-asserted-by":"crossref","unstructured":"Baumann, U., Routes, J.M., Soler-Palac\u00edn, P., and Jolles, S. (2018). The Lung in Primary Immunodeficiencies: New Concepts in Infection and Inflammation. Front Immunol., 9.","DOI":"10.3389\/fimmu.2018.01837"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"213","DOI":"10.18176\/jiaci.0166","article-title":"Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders","volume":"27","author":"Yazdani","year":"2017","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_50","doi-asserted-by":"crossref","unstructured":"Blom, M., Bredius, R.G.M., and van der Burg, M. (2021). Future Perspectives of Newborn Screening for Inborn Errors of Immunity. Int. J. Neonatal Screen., 7.","DOI":"10.3390\/ijns7040074"},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Lehrnbecher, T., Russo, A., and Rohde, G. (2025). A Survey of Diagnosis and Therapy of Inborn Errors of Immunity among Practice-Based Physicians and Clinic-Based Pneumologists and Hemato-Oncologists. Front. Immunol., 16.","DOI":"10.3389\/fimmu.2025.1597635"},{"key":"ref_52","doi-asserted-by":"crossref","unstructured":"Mauracher, A.A., and Henrickson, S.E. (2022). Leveraging Systems Immunology to Optimize Diagnosis and Treatment of Inborn Errors of Immunity. Front. Syst. Biol., 2.","DOI":"10.3389\/fsysb.2022.910243"},{"key":"ref_53","unstructured":"(2025, October 10). Approach to Diagnosing Inborn Errors of Immunity\u2014Rheumatic Disease Clinics. Available online: https:\/\/www.rheumatic.theclinics.com\/article\/S0889-857X(23)00061-3\/abstract."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.jped.2020.11.007","article-title":"Inborn Errors of Immunity: How to Diagnose Them?","volume":"97","author":"Grumach","year":"2021","journal-title":"J. Pediatr."},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Patel, T., Ilangovan, G., and Balaganesan, H. (2020). Comparative Study of X-ray and High Resolution Computed Tomography in the Diagnosis of Interstitial Lung Disease. Int. J. Contemp. Med. Surg. Radiol., 5.","DOI":"10.21276\/ijcmsr.2020.5.1.58"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"e0887","DOI":"10.1002\/rcr2.887","article-title":"Chronic Pulmonary Aspergillosis in a Patient with Hyper-IgE Syndrome","volume":"10","author":"Kasuga","year":"2022","journal-title":"Respirol. Case Rep."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1700629","DOI":"10.1183\/13993003.00629-2017","article-title":"European Respiratory Society Guidelines for the Management of Adult Bronchiectasis","volume":"50","author":"Polverino","year":"2017","journal-title":"Eur. Respir. J."},{"key":"ref_58","doi-asserted-by":"crossref","unstructured":"Sharma, R., Lewis, S., and Wlodarski, M.W. (2020). DNA Repair Syndromes and Cancer: Insights Into Genetics and Phenotype Patterns. Front. Pediatr., 8.","DOI":"10.3389\/fped.2020.570084"},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Jo\u0144czyk-Potoczna, K., Potoczny, J., and Szczawi\u0144ska-Pop\u0142onyk, A. (2022). Imaging in Children with Ataxia-Telangiectasia\u2014The Radiologist\u2019s Approach. Front. Pediatr., 10.","DOI":"10.3389\/fped.2022.988645"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1178","DOI":"10.1016\/j.jaci.2015.04.027","article-title":"Radiation-Sensitive Severe Combined Immunodeficiency: The Arguments for and against Conditioning before Hematopoietic Cell Transplantation\u2014What to Do?","volume":"136","author":"Cowan","year":"2015","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Wall, L.A., Wisner, E.L., Gipson, K.S., and Sorensen, R.U. (2020). Bronchiectasis in Primary Antibody Deficiencies: A Multidisciplinary Approach. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.00522"},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Bintalib, H.M., van de Ven, A., Jacob, J., Davidsen, J.R., Fevang, B., Hanitsch, L.G., Malphettes, M., van Montfrans, J., Maglione, P.J., and Milito, C. (2023). Diagnostic Testing for Interstitial Lung Disease in Common Variable Immunodeficiency: A Systematic Review. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1190235"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/j.anai.2020.01.019","article-title":"Comprehensive Assessment of Respiratory Complications in Patients with Common Variable Immunodeficiency","volume":"124","author":"Moazzami","year":"2020","journal-title":"Ann. Allergy Asthma Immunol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1016\/j.intimp.2004.02.011","article-title":"Immunoglobulin Therapy to Control Lung Damage in Patients with Common Variable Immunodeficiency","volume":"4","author":"Vendrell","year":"2004","journal-title":"Int. Immunopharmacol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"100018","DOI":"10.1016\/j.waojou.2019.100018","article-title":"X-Linked Agammaglobulinemia (XLA): Phenotype, Diagnosis, and Therapeutic Challenges around the World","volume":"12","author":"Abramova","year":"2019","journal-title":"World Allergy Organ. J."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Perazzio, S.F., Palmeira, P., Moraes-Vasconcelos, D., Rangel-Santos, A., de Oliveira, J.B., Andrade, L.E.C., and Carneiro-Sampaio, M. (2021). A Critical Review on the Standardization and Quality Assessment of Nonfunctional Laboratory Tests Frequently Used to Identify Inborn Errors of Immunity. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.721289"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"338","DOI":"10.1007\/s12098-016-2038-x","article-title":"Common Variable Immunodeficiency","volume":"83","author":"Saikia","year":"2016","journal-title":"Indian J. Pediatr."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1023","DOI":"10.1016\/j.jaip.2016.09.013","article-title":"Clinical Phenotypes of Hyper-IgM Syndromes","volume":"4","year":"2016","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1136\/archdischild-2015-309060","article-title":"How to Use Immunoglobulin Levels in Investigating Immune Deficiencies","volume":"101","author":"Ladomenou","year":"2016","journal-title":"Arch. Dis. Child.\u2014Educ. Pract. Ed."},{"key":"ref_70","doi-asserted-by":"crossref","unstructured":"Khan, S.R., van der Burgh, A.C., Peeters, R.P., van Hagen, P.M., Dalm, V.A.S.H., and Chaker, L. (2021). Determinants of Serum Immunoglobulin Levels: A Systematic Review and Meta-Analysis. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.664526"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"45","DOI":"10.4103\/JME.JME_103_23","article-title":"Pattern of Serum Protein Electrophoresis: Advances in Laboratory Medicine","volume":"6","author":"Solanki","year":"2025","journal-title":"J. Med. Evid."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1111\/cei.12369","article-title":"Calculated Globulin (CG) as a Screening Test for Antibody Deficiency","volume":"177","author":"Jolles","year":"2014","journal-title":"Clin. Exp. Immunol."},{"key":"ref_73","first-page":"407","article-title":"Flow Cytometry in the Diagnosis and Follow Up of Human Primary Immunodeficiencies","volume":"30","author":"Salzer","year":"2019","journal-title":"EJIFCC"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1080\/1744666X.2021.1954908","article-title":"Lymphocytes Subsets in Correlation with Clinical Profile in CVID Patients without Monogenic Defects","volume":"17","author":"Mirshafiey","year":"2021","journal-title":"Expert Rev. Clin. Immunol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.aller.2012.07.016","article-title":"Lymphocytes and B-Cell Abnormalities in Patients with Common Variable Immunodeficiency (CVID)","volume":"42","year":"2014","journal-title":"Allergol. Immunopathol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"032066","DOI":"10.1088\/1742-6596\/1569\/3\/032066","article-title":"The Correlation between CD4 Count Cell and Opportunistic Infection among HIV\/AIDS Patients","volume":"1569","author":"Jayani","year":"2020","journal-title":"J. Phys. Conf. Ser."},{"key":"ref_77","doi-asserted-by":"crossref","unstructured":"Hansen, A.T., S\u00f6derstr\u00f6m, A., J\u00f8rgensen, C.S., Larsen, C.S., Petersen, M.S., and Bernth Jensen, J.M. (2021). Diagnostic Vaccination in Clinical Practice. Front. Immunol., 12.","DOI":"10.3389\/fimmu.2021.717873"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"113350","DOI":"10.1016\/j.jim.2022.113350","article-title":"Application of Vaccine Response in the Evaluation of Patients with Suspected B-Cell Immunodeficiency: Assessment of Responses and Challenges with Interpretation","volume":"510","author":"Peterson","year":"2022","journal-title":"J. Immunol. Methods"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"113819","DOI":"10.1016\/j.jim.2025.113819","article-title":"Clinical Utility of the Lymphocyte Proliferation Assay, an in Vitro Functional Readout of the Adaptive Immune Response","volume":"537","author":"Peterson","year":"2025","journal-title":"J. Immunol. Methods"},{"key":"ref_80","doi-asserted-by":"crossref","unstructured":"Rezahosseini, O., M\u00f8ller, D.L., Knudsen, A.D., S\u00f8rensen, S.S., Perch, M., Gustafsson, F., Rasmussen, A., Ostrowski, S.R., and Nielsen, S.D. (2020). Use of T Cell Mediated Immune Functional Assays for Adjustment of Immunosuppressive or Anti-Infective Agents in Solid Organ Transplant Recipients: A Systematic Review. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.567715"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1661","DOI":"10.4161\/hv.28350","article-title":"Polysaccharide-Specific B Cell Responses to Vaccination in Humans","volume":"10","author":"Mitchell","year":"2014","journal-title":"Hum. Vaccines Immunother."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"S1","DOI":"10.1016\/j.jaci.2012.07.002","article-title":"Use and Interpretation of Diagnostic Vaccination in Primary Immunodeficiency: A Working Group Report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology","volume":"130","author":"Orange","year":"2012","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1007\/s12519-015-0056-9","article-title":"Immune Neutropenias of Infancy and Childhood","volume":"12","author":"Farruggia","year":"2016","journal-title":"World J. Pediatr."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.earlhumdev.2010.11.003","article-title":"Transplacental Transport of IgG Antibodies to Preterm Infants: A Review of the Literature","volume":"87","author":"Westerbeek","year":"2011","journal-title":"Early Hum. Dev."},{"key":"ref_85","first-page":"57","article-title":"Immunolog kliniczny potrzebny od zaraz?","volume":"14","author":"Siebert","year":"2020","journal-title":"Forum Med. Rodz."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"101901","DOI":"10.1016\/j.smim.2024.101901","article-title":"NGS Data Analysis for Molecular Diagnosis of Inborn Errors of Immunity","volume":"74\u201375","author":"Yang","year":"2024","journal-title":"Semin. Immunol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1186\/s12943-018-0779-z","article-title":"Role of Bruton\u2019s Tyrosine Kinase in B Cells and Malignancies","volume":"17","author":"Dammeijer","year":"2018","journal-title":"Mol. Cancer"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"736","DOI":"10.1016\/j.jaci.2021.04.039","article-title":"Therapeutic Options for CTLA-4 Insufficiency","volume":"149","author":"Egg","year":"2022","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1111\/cei.13600","article-title":"Comprehensive Comparison between 222 CTLA-4 Haploinsufficiency and 212 LRBA Deficiency Patients: A Systematic Review","volume":"205","author":"Jamee","year":"2021","journal-title":"Clin. Exp. Immunol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1016\/j.hoc.2018.04.004","article-title":"Germline GATA2 Mutation and Bone Marrow Failure","volume":"32","author":"McReynolds","year":"2018","journal-title":"Hematol. Oncol. Clin. N. Am."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"292","DOI":"10.4168\/aair.2020.12.2.292","article-title":"Flow Cytometry for the Diagnosis of Primary Immunodeficiency Diseases: A Single Center Experience","volume":"12","author":"Kwon","year":"2020","journal-title":"Allergy Asthma Immunol. Res."},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Rawat, A., Arora, K., Shandilya, J., Vignesh, P., Suri, D., Kaur, G., Rikhi, R., Joshi, V., Das, J., and Mathew, B. (2019). Flow Cytometry for Diagnosis of Primary Immune Deficiencies-A Tertiary Center Experience From North India. Front. Immunol., 10.","DOI":"10.3389\/fimmu.2019.02111"},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.humimm.2021.01.014","article-title":"The Immune Potential of Decidua-Resident CD16+CD56+ NK Cells in Human Pregnancy","volume":"82","author":"Zhang","year":"2021","journal-title":"Hum. Immunol."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"367","DOI":"10.5114\/ceji.2013.37748","article-title":"Clinical and Immunological Analysis of Patients with X-Linked Agammaglobulinemia: Single Center Experience","volume":"3","author":"Pac","year":"2013","journal-title":"Central Eur. J. Immunol."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"299","DOI":"10.18176\/jiaci.0147","article-title":"Clinical, Laboratory, and Molecular Findings for 63 Patients With Severe Combined Immunodeficiency: A Decade\u2019s Experience","volume":"27","author":"Fazlollahi","year":"2017","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_96","doi-asserted-by":"crossref","unstructured":"Rawat, A., Jindal, A.K., Suri, D., Vignesh, P., Gupta, A., Saikia, B., Minz, R.W., Banday, A.Z., Tyagi, R., and Arora, K. (2021). Clinical and Genetic Profile of X-Linked Agammaglobulinemia: A Multicenter Experience From India. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.612323"},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1680","DOI":"10.1056\/NEJMoa2202348","article-title":"Reappraisal of Idiopathic CD4 Lymphocytopenia at 30 Years","volume":"388","author":"Lisco","year":"2023","journal-title":"N. Engl. J. Med."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1016\/j.jcrc.2016.06.026","article-title":"Comparison of 1,3-\u03b2-d-Glucan with Galactomannan in Serum and Bronchoalveolar Fluid for the Detection of Aspergillus Species in Immunosuppressed Mechanical Ventilated Critically Ill Patients","volume":"36","author":"Lahmer","year":"2016","journal-title":"J. Crit. Care"},{"key":"ref_99","first-page":"103","article-title":"Intravenous Immunoglobulin Replacement Treatment Reduces in Vivo Elastase Secretion in Patients with Common Variable Immune Disorders","volume":"17","author":"Prezzo","year":"2019","journal-title":"Blood Transfus."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1111\/cei.13068","article-title":"Bronchiectasis and Deteriorating Lung Function in Agammaglobulinaemia despite Immunoglobulin Replacement Therapy","volume":"191","author":"Stubbs","year":"2018","journal-title":"Clin. Exp. Immunol."},{"key":"ref_101","unstructured":"(2025, October 10). Bronchiectasis and Intravenous Immunoglobulin Therapy. Available online: https:\/\/www.aaaai.org\/allergist-resources\/ask-the-expert\/answers\/2023\/bronchiectasis."},{"key":"ref_102","first-page":"514","article-title":"Efficacy of Intravenous Immunoglobulin Treatment in Children with Common Variable Immunodeficiency","volume":"21","author":"Baris","year":"2011","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1016\/j.chest.2019.05.009","article-title":"Common Variable Immune Deficiency and Associated Complications","volume":"156","author":"Gupta","year":"2019","journal-title":"Chest"},{"key":"ref_104","unstructured":"Arumugham, V.B., and Rayi, A. (2025). Intravenous Immunoglobulin (IVIG). StatPearls, StatPearls Publishing."},{"key":"ref_105","doi-asserted-by":"crossref","unstructured":"Barahona Afonso, A.F., and Jo\u00e3o, C.M.P. (2016). The Production Processes and Biological Effects of Intravenous Immunoglobulin. Biomolecules, 6.","DOI":"10.3390\/biom6010015"},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"125","DOI":"10.7861\/clinmedicine.15-2-125","article-title":"False Interpretation of Diagnostic Serology Tests for Patients Treated with Pooled Human Immunoglobulin G Infusions: A Trap for the Unwary","volume":"15","author":"Bright","year":"2015","journal-title":"Clin. Med."},{"key":"ref_107","doi-asserted-by":"crossref","unstructured":"Suresh, J., and Kyle, B.D. (2021). Clinical False Positives Resulting from Recent Intravenous Immunoglobulin Therapy: Case Report. BMC Infect. Dis., 21.","DOI":"10.1186\/s12879-021-05986-z"},{"key":"ref_108","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1016\/j.aller.2012.09.006","article-title":"Guidelines for the Use of Human Immunoglobulin Therapy in Patients with Primary Immunodeficiencies in Latin America","volume":"42","author":"Grumach","year":"2014","journal-title":"Allergol. Immunopathol."},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"382","DOI":"10.1007\/s10875-020-00841-3","article-title":"Immunoglobulin Replacement Therapy Versus Antibiotic Prophylaxis as Treatment for Incomplete Primary Antibody Deficiency","volume":"41","author":"Smits","year":"2021","journal-title":"J. Clin. Immunol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"100048","DOI":"10.1016\/j.clinpr.2020.100048","article-title":"Antibiotic Prophylaxis for the Prevention of Respiratory Tract Infections in Antibody Deficient Patients: A Retrospective Cohort Study","volume":"7\u20138","author":"Grammatikos","year":"2020","journal-title":"Clin. Infect. Pract."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"dlad135","DOI":"10.1093\/jacamr\/dlad135","article-title":"Antibiotic Resistance, Bacterial Transmission and Improved Prediction of Bacterial Infection in Patients with Antibody Deficiency","volume":"5","author":"Rofael","year":"2023","journal-title":"JAC-Antimicrob. Resist."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1097\/ACI.0b013e328332be33","article-title":"Antimicrobial Prophylaxis for Primary Immunodeficiencies","volume":"9","author":"Freeman","year":"2009","journal-title":"Curr. Opin. Allergy Clin. Immunol."},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"ofaa112","DOI":"10.1093\/ofid\/ofaa112","article-title":"Low-Dose TMP-SMX in the Treatment of Pneumocystis jirovecii Pneumonia: A Systematic Review and Meta-Analysis","volume":"7","author":"Smyth","year":"2020","journal-title":"Open Forum Infect. Dis."},{"key":"ref_114","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1080\/1040841X.2021.1884641","article-title":"Candida Albicans Targets That Potentially Synergize with Fluconazole","volume":"47","author":"Lu","year":"2021","journal-title":"Crit. Rev. Microbiol."},{"key":"ref_115","doi-asserted-by":"crossref","unstructured":"Olum, R., Baluku, J.B., Kazibwe, A., Russell, L., and Bongomin, F. (2020). Tolerability of Oral Itraconazole and Voriconazole for the Treatment of Chronic Pulmonary Aspergillosis: A Systematic Review and Meta-Analysis. PLoS ONE, 15.","DOI":"10.1371\/journal.pone.0240374"},{"key":"ref_116","unstructured":"Taylor, M., and Gerriets, V. (2025). Acyclovir. StatPearls, StatPearls Publishing."},{"key":"ref_117","unstructured":"(2025, November 11). Ganciclovir and Valganciclovir: An Overview\u2014UpToDate. Available online: https:\/\/www.uptodate.com\/contents\/ganciclovir-and-valganciclovir-an-overview."},{"key":"ref_118","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/s12948-019-0113-3","article-title":"Immunosuppressive Therapy with Rituximab in Common Variable Immunodeficiency","volume":"17","author":"Pecoraro","year":"2019","journal-title":"Clin. Mol. Allergy"},{"key":"ref_119","doi-asserted-by":"crossref","first-page":"2091","DOI":"10.1007\/s10875-023-01587-4","article-title":"Rituximab Monotherapy Is Effective as First-Line Treatment for Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in CVID Patients","volume":"43","author":"Tessarin","year":"2023","journal-title":"J. Clin. Immunol."},{"key":"ref_120","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1016\/j.jaci.2020.07.021","article-title":"Rituximab and Antimetabolite Treatment of Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency","volume":"147","author":"Verbsky","year":"2021","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_121","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/s13223-022-00661-7","article-title":"Monitoring Patients with Uncomplicated Common Variable Immunodeficiency: A Systematic Review","volume":"18","author":"Lee","year":"2022","journal-title":"Allergy Asthma Clin. Immunol."},{"key":"ref_122","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1111\/cei.13012","article-title":"Screening Protocols to Monitor Respiratory Status in Primary Immunodeficiency Disease: Findings from a European Survey and Subclinical Infection Working Group","volume":"190","author":"Jolles","year":"2017","journal-title":"Clin. Exp. Immunol."},{"key":"ref_123","doi-asserted-by":"crossref","first-page":"2208","DOI":"10.1007\/s10875-023-01593-6","article-title":"Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry","volume":"43","author":"Nino","year":"2023","journal-title":"J. Clin. Immunol."},{"key":"ref_124","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1016\/j.jaip.2016.09.012","article-title":"Vaccination in Primary Immunodeficiency Disorders","volume":"4","author":"Sobh","year":"2016","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_125","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1016\/j.jaci.2017.12.980","article-title":"Update: Vaccines in Primary Immunodeficiency","volume":"141","author":"Bonilla","year":"2018","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_126","first-page":"202401813","article-title":"Vaccination against Respiratory Tract Pathogens in Primary Immune Deficiency Patients Receiving Immunoglobulin Replacement Therapy","volume":"72","author":"Durmaz","year":"2024","journal-title":"Tuberk Toraks"},{"key":"ref_127","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1186\/s13223-024-00938-z","article-title":"Inborn Errors of Immunity (Primary Immunodeficiencies)","volume":"20","author":"Kim","year":"2025","journal-title":"Allergy Asthma Clin. Immunol."},{"key":"ref_128","first-page":"171","article-title":"Vaccination in Patients with Primary Immunodeficiency Disorders","volume":"3","author":"Khalili","year":"2020","journal-title":"Immunol. Genet. J."},{"key":"ref_129","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/s13223-018-0290-5","article-title":"Primary Immunodeficiency","volume":"14","author":"McCusker","year":"2018","journal-title":"Allergy Asthma Clin. Immunol."},{"key":"ref_130","doi-asserted-by":"crossref","unstructured":"Meyts, I., Bousfiha, A., Duff, C., Singh, S., Lau, Y.L., Condino-Neto, A., Bezrodnik, L., Ali, A., Adeli, M., and Drabwell, J. (2020). Primary Immunodeficiencies: A Decade of Progress and a Promising Future. Front. Immunol., 11.","DOI":"10.3389\/fimmu.2020.625753"},{"key":"ref_131","doi-asserted-by":"crossref","first-page":"154","DOI":"10.15586\/aei.v51i1.627","article-title":"Outcomes and Impact of Multidisciplinary Team Care on Immunologic and Hemato-Oncologic Pediatric Patients","volume":"51","author":"Estirado","year":"2023","journal-title":"Allergol. Immunopathol."},{"key":"ref_132","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1007\/s10875-022-01345-y","article-title":"Current Transition Practice for Primary Immunodeficiencies and Autoinflammatory Diseases in Europe: A RITA-ERN Survey","volume":"43","author":"Israni","year":"2023","journal-title":"J. Clin. Immunol."},{"key":"ref_133","doi-asserted-by":"crossref","unstructured":"Mej\u00eda Gonz\u00e1lez, M.A., Quijada Morales, P., Escobar, M.\u00c1., Ju\u00e1rez Guerrero, A., and Seoane-Reula, M.E. (2023). Navigating the Transition of Care in Patients with Inborn Errors of Immunity: A Single-Center\u2019s Descriptive Experience. Front. Immunol., 14.","DOI":"10.3389\/fimmu.2023.1263349"},{"key":"ref_134","doi-asserted-by":"crossref","first-page":"1929","DOI":"10.1016\/j.jaip.2019.02.013","article-title":"Health-Related Quality of Life in Children and Adults with Primary Immunodeficiencies: A Systematic Review and Meta-Analysis","volume":"7","author":"Peshko","year":"2019","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_135","doi-asserted-by":"crossref","unstructured":"Campos, J.S., and Henrickson, S.E. (2022). Defining and Targeting Patterns of T Cell Dysfunction in Inborn Errors of Immunity. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.932715"},{"key":"ref_136","doi-asserted-by":"crossref","unstructured":"Ottaviano, G., Chiesa, R., Feuchtinger, T., Vickers, M.A., Dickinson, A., Gennery, A.R., Veys, P., and Todryk, S. (2019). Adoptive T Cell Therapy Strategies for Viral Infections in Patients Receiving Haematopoietic Stem Cell Transplantation. Cells, 8.","DOI":"10.3390\/cells8010047"}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1838\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,12,2]],"date-time":"2025-12-02T09:10:28Z","timestamp":1764666628000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/15\/12\/1838"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,29]]},"references-count":136,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["life15121838"],"URL":"https:\/\/doi.org\/10.3390\/life15121838","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,29]]}}}