The prevalances and patient characteristics of primary immunodeficiency diseases in Turkey-two centers study

dc.contributor.authorKaraca, Neslihan Edeer
dc.contributor.authorAksu, Güzide
dc.contributor.authorKütükçüler, Necil
dc.contributor.buuauthorKılıç, Sara Şebnem
dc.contributor.buuauthorÖzel, Mustafa
dc.contributor.buuauthorHafızoğlu, Demet
dc.contributor.departmentUludağ Üniversitesi/Tıp Fakültesi/Pediatrik İmmünoloji Anabilim Dalı.tr_TR
dc.contributor.orcid0000-0001-8571-2581tr_TR
dc.contributor.researcheridAAH-1658-2021tr_TR
dc.contributor.scopusid34975059200tr_TR
dc.contributor.scopusid55356742500tr_TR
dc.contributor.scopusid36711582000tr_TR
dc.date.accessioned2022-10-05T12:36:23Z
dc.date.available2022-10-05T12:36:23Z
dc.date.issued2013-01
dc.description.abstractPurpose Primary immunodeficiency diseases (PIDs) are inherited disorders of the immune system resulting in increased susceptibility to unusual infections and predisposition to autoimmunity and malignancies. The European Society for Immunodeficiencies (ESID) has developed an internet-based database for clinical and research data on patients with PID. This study aimed to provide a minimum estimate of the prevalence of each disorder and to determine the clinical characteristics and outcomes of patients with PID in Turkey. Methods Clinical features of 1435 patients with primary immunodeficiency disorders are registered in ESID Online Patient Registry by the Pediatric Immunology Departments of the Medical Faculties of Uludag University and Ege University Between 2004 and 2010. These two centers are the major contributors reporting PID patients to ESID database from Turkey. Results Predominantly antibody immunodeficiency (73.9 %) was the most common category followed by autoinflammatory disorders (13.3 %), other well defined immunodeficiencies (5.5 %), congenital defects of phagocyte number, function or both (3.5 %), combined T and B cell immunodeficiencies (2 %), defects in innate immunity (1 %), and diseases of immune dysregulation (0.7 %) and complement deficiencies (0.4 %). Patients between 0 and 18 years of age constitued 94 % of total and the mean age was 9.2 +/- 6 years. The consanguinity rate within the registered patients was 14.3 % (188 of 1130 patients). The prevalance of all PID cases ascertained from the registry was 30.5/100.000. The major cause of the mortality was severe infection which was seen in forty-two of seventy five deceased patients. The highest mortality was observed in patients with severe combined immunodeficiencies and ataxia-telangiectasia. Conclusion Promoting the awareness of PID among the medical professionals and the general public is required if premature death and serious morbidity occurs due to late diagnosis of the wider spectrum of PID are to be avoided.en_US
dc.identifier.citationKılıç, S. Ş. vd.(2013). "The prevalances and patient characteristics of primary immunodeficiency diseases in Turkey-two centers study". Journal of Clinical Immunology, 33(1), 74-83.en_US
dc.identifier.endpage83tr_TR
dc.identifier.issn0271-9142
dc.identifier.issue1tr_TR
dc.identifier.pubmed22983506tr_TR
dc.identifier.scopus2-s2.0-84876430340tr_TR
dc.identifier.startpage74tr_TR
dc.identifier.urihttps://doi.org/10.1007/s10875-012-9763-3
dc.identifier.urihttps://pubmed.ncbi.nlm.nih.gov/22983506/
dc.identifier.urihttp://hdl.handle.net/11452/28973
dc.identifier.volume33tr_TR
dc.identifier.wos000323117100010tr_TR
dc.indexed.pubmedPubMeden_US
dc.indexed.scopusScopusen_US
dc.indexed.wosSCIEen_US
dc.language.isoenen_US
dc.publisherSpringer/Plenum Publishersen_US
dc.relation.collaborationYurt içitr_TR
dc.relation.journalJournal of Clinical Immunologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergitr_TR
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectImmunologyen_US
dc.subjectRegistryen_US
dc.subjectESIDen_US
dc.subjectOnline databaseen_US
dc.subjectPrimary immunodeficiencyen_US
dc.subjectTurkeyen_US
dc.subjectNational registryen_US
dc.subject1st reporten_US
dc.subjectDisordersen_US
dc.subjectChildrenen_US
dc.subjectAustraliaen_US
dc.subjectDatabaseen_US
dc.subjectUpdateen_US
dc.subjectIranen_US
dc.subject.emtreeImmunoglobulinen_US
dc.subject.emtreeAdolescenten_US
dc.subject.emtreeAdulten_US
dc.subject.emtreeAgeden_US
dc.subject.emtreeAlbers Schoenberg diseaseen_US
dc.subject.emtreeAntibiotic prophylaxisen_US
dc.subject.emtreeArticleen_US
dc.subject.emtreeAtaxia telangiectasiaen_US
dc.subject.emtreeAutoinflammatory diseaseen_US
dc.subject.emtreeChilden_US
dc.subject.emtreeChronic granulomatous diseaseen_US
dc.subject.emtreeClinical featureen_US
dc.subject.emtreeCommon variable immunodeficiencyen_US
dc.subject.emtreeComplement deficiencyen_US
dc.subject.emtreeCongenital disorderen_US
dc.subject.emtreeConsanguinityen_US
dc.subject.emtreeDisease courseen_US
dc.subject.emtreeEarly diagnosisen_US
dc.subject.emtreeFemaleen_US
dc.subject.emtreeHumanen_US
dc.subject.emtreeHumoral immune deficiencyen_US
dc.subject.emtreeHyper IgM syndromeen_US
dc.subject.emtreeImmune deficiencyen_US
dc.subject.emtreeImmune dysregulationen_US
dc.subject.emtreeImmunoglobulin A deficiencyen_US
dc.subject.emtreeInfanten_US
dc.subject.emtreeInnate immunityen_US
dc.subject.emtreeMajor clinical studyen_US
dc.subject.emtreeMaleen_US
dc.subject.emtreeMiddle ageden_US
dc.subject.emtreeMorbidityen_US
dc.subject.emtreeMortalityen_US
dc.subject.emtreeMucocutaneous candidiasisen_US
dc.subject.emtreeMulticenter studyen_US
dc.subject.emtreeNewbornen_US
dc.subject.emtreeOutcome assessmenten_US
dc.subject.emtreePhagocyteen_US
dc.subject.emtreePreschool childen_US
dc.subject.emtreePrevalenceen_US
dc.subject.emtreePriority journalen_US
dc.subject.emtreeReference databaseen_US
dc.subject.emtreeSchool childen_US
dc.subject.emtreeSevere combined immunodeficiencyen_US
dc.subject.emtreeTransient hypogammaglobulinemia of infancyen_US
dc.subject.emtreeTurkey (republic)en_US
dc.subject.emtreeVery elderlyen_US
dc.subject.emtreeWiskott Aldrich syndromeen_US
dc.subject.emtreeYoung adulten_US
dc.subject.emtreeCommon variable immunodeficiencyen_US
dc.subject.emtreeFactual databaseen_US
dc.subject.emtreeImmunologyen_US
dc.subject.emtreeRegisteren_US
dc.subject.emtreeTurkey (republic)en_US
dc.subject.emtreeUniversity hospitalen_US
dc.subject.meshAdolescenten_US
dc.subject.meshAdulten_US
dc.subject.meshChilden_US
dc.subject.meshChild, preschoolen_US
dc.subject.meshCommon variable immunodeficiencyen_US
dc.subject.meshDatabases, factualen_US
dc.subject.meshFemaleen_US
dc.subject.meshHospitals, universityen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshMaleen_US
dc.subject.meshPrevalenceen_US
dc.subject.meshRegistriesen_US
dc.subject.meshTurkeyen_US
dc.subject.meshYoung adulten_US
dc.subject.scopusCommon Variable Immunodeficiency; Immunoglobulin Deficiency; Immunosuppressionen_US
dc.subject.wosImmunologyen_US
dc.titleThe prevalances and patient characteristics of primary immunodeficiency diseases in Turkey-two centers studyen_US
dc.typeArticle
dc.wos.quartileQ3en_US

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