Clinical features and outcome of children with hereditary spherocytosis
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Dosyalar
Tarih
2021
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Lippincott Williams and Wilkins
Erişim Hakkı
info:eu-repo/semantics/closedAccess
Özet
Objective: 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.
Açıklama
Anahtar Kelimeler
Children, Hemolytic Anemia, Hereditary Spherocytosis, Splenectomy
Kaynak
Journal of Pediatric Hematology/Oncology
WoS Q Değeri
Q4
Scopus Q Değeri
Q3
Cilt
Sayı
Künye
Kiliç, 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.
