Clinical features and outcome of children with hereditary spherocytosis

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dc.authoridGül Nihal Özdemir / 0000-0002-3204-4353en_US
dc.authorscopusidGül Nihal Özdemir / 34067792800
dc.authorwosidGül Nihal Özdemir / AAO-9962-2020
dc.contributor.authorKılıç, Mehmet A
dc.contributor.authorÖzdemir, Gül Nihal
dc.contributor.authorTahtakesen, Tuba Nur
dc.contributor.authorGökçe, Müge G.
dc.contributor.authorUysalol, Ezgi Paslı
dc.contributor.authorBayram, Cengiz
dc.contributor.authorAyçiçek, Ali
dc.contributor.authorAydo?an, Gönül
dc.date.accessioned2021-06-16T06:11:43Z
dc.date.available2021-06-16T06:11:43Z
dc.date.issued2021en_US
dc.departmentİstinye Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümüen_US
dc.description.abstractObjective: The aim of this study was to evaluate the demographics, clinical, and laboratory findings and treatment responses of patients with hereditary spherocytosis (HS). Materials and Methods: Data of children with HS were examined. Diagnosis was based on clinical history, physical examination, family history, presence of spherocytes on peripheral blood smear, and osmotic fragility test. Results: A total of 101 patients were included. The median (range) age at diagnosis was 38.0 (1 to 188) months. Mild, moderate, and severe forms of HS were present in 29 (28.7%), 15 (14.9%), and 57 (56.4%) patients, respectively. Family history was available in 73 patients and 56 of these (76.7%) had a positive family history for HS. Forty-five (44.5%) patients needed regular transfusions and all of these had severe disease. Although most patients did not require transfusion postsplenectomy, 2 of 45 (4.4%) patients continued to require transfusion. Transfusion dependence was significantly (P<0.001) higher in patients with severe spherocytosis. Conclusions: In HS, splenomegaly, pallor, and jaundice are the most common clinical features. Splenectomy dramatically reduces hemolysis in most cases and virtually abolishes further requirement for transfusion.en_US
dc.identifier.citationKiliç, M. A., Özdemir, G. N., Tahtakesen, T. N., Gökçe, M., Uysalol, E. P., Bayram, C., ... & Aydoğan, G. (2021). Clinical Features and Outcome of Children With Hereditary Spherocytosis. Journal of Pediatric Hematology/oncology.en_US
dc.identifier.doi10.1097/MPH.0000000000002211en_US
dc.identifier.issn1077-4114en_US
dc.identifier.pmid34054043en_US
dc.identifier.scopus2-s2.0-85107450404en_US
dc.identifier.scopusqualityQ3en_US
dc.identifier.urihttps://doi.org/10.1097/MPH.0000000000002211
dc.identifier.urihttps://hdl.handle.net/20.500.12713/1800
dc.identifier.wosWOS:000760391300012en_US
dc.identifier.wosqualityQ4en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.institutionauthorÖzdemir, Gül Nihal
dc.language.isoenen_US
dc.publisherLippincott Williams and Wilkinsen_US
dc.relation.ispartofJournal of Pediatric Hematology/Oncologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.subjectChildrenen_US
dc.subjectHemolytic Anemiaen_US
dc.subjectHereditary Spherocytosisen_US
dc.subjectSplenectomyen_US
dc.titleClinical features and outcome of children with hereditary spherocytosisen_US
dc.typeArticleen_US

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