Hereditary Spherocytosis: Evaluation of 68 Children

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dc.contributor.authorKonca, Capan
dc.contributor.authorSoker, Murat
dc.contributor.authorTas, Mehmet Ali
dc.contributor.authorYildirim, Ruken
dc.date.accessioned2024-04-24T16:02:48Z
dc.date.available2024-04-24T16:02:48Z
dc.date.issued2015
dc.departmentDicle Üniversitesien_US
dc.description.abstractTo determine the clinical and hematologic features of 68 children with hereditary spherocytosis (HS). In this retrospective study, we analyzed recorded information of 68 HS patients diagnosed between March 1997 and March 2007, including clinical manifestations at admission, gender, median age at diagnosis, family history, hematologic and biochemical data, patient management, complications, median age of splenectomy, and median follow-up time. Sixty-eight patients with HS (36 male and female) were investigated. The median age at diagnosis was 5.6 years (range 3 months to 18 years). Twenty-seven (39.7 %) had parents with consanguineous marriages, and 20 (29.4 %) had parents with first-degree consanguinity. Predominant clinical manifestations at admission were anemia in 59 patients (86.76 %), splenomegaly in 49 (72.05 %), and jaundice in 33 (48.52 %). Patients were classified as mild, moderate, or severe in 29.4, 61.7, and 8.8 % of patients, respectively. Five patients (7.3 %) underwent splenectomy. Major complications of HS were hemolytic, aplastic, and megaloblastic crises and cholelithiasis in 7 (10.2 %), 1 (1.4 %), 7 (10.2 %), and 6 (8.8 %) of patients, respectively. There were no deaths during follow-up. HS should be considered in evaluating possible diagnoses in patients with hemolytic anemia. In this study, the clinical course of patients with HS was relatively benign, with low proportions of patients having splenectomized and aplastic crises.en_US
dc.identifier.doi10.1007/s12288-014-0379-z
dc.identifier.endpage132en_US
dc.identifier.issn0971-4502
dc.identifier.issn0974-0449
dc.identifier.issue1en_US
dc.identifier.pmid25548458
dc.identifier.startpage127en_US
dc.identifier.urihttps://doi.org/10.1007/s12288-014-0379-z
dc.identifier.urihttps://hdl.handle.net/11468/14917
dc.identifier.volume31en_US
dc.identifier.wosWOS:000346904000022
dc.identifier.wosqualityQ4
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakPubMed
dc.language.isoenen_US
dc.publisherSpringer Indiaen_US
dc.relation.ispartofIndian Journal of Hematology and Blood Transfusion
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subject[No Keyword]en_US
dc.titleHereditary Spherocytosis: Evaluation of 68 Childrenen_US
dc.titleHereditary Spherocytosis: Evaluation of 68 Children
dc.typeArticleen_US

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