Clinical Features and Genotypes of Patients with Hemoglobin H Disease in Taiwan.
In: Laboratory Medicine, Jg. 50 (2019-04-01), Heft 2, S. 168-173
Online
academicJournal
Zugriff:
Background The genetic background of patients with hemoglobin (Hb) H disease in Taiwan has been investigated; however, the clinical features and treatment outcomes were not reported. Objective To analyze the clinical features and genotypes of patients with HbH who reside in Taiwan. Methods We conducted a retrospective analysis of the clinical and molecular characteristics of 38 patients with HbH disease who were undergoing treatment at Kaohsiung Medical University Hospital, Taiwan. Results Initial Hb levels were lower and the numbers of patients requiring iron-chelation therapy were higher in the nondeletional HbH group than in the deletional HbH group (P <.05). Compared with the healthy population, the patients with HbH disease exhibited short body length, low body weight, and low body mass index (BMI). Conclusions Patients with nondeletional HbH disease had lower Hb levels and a higher requirement for splenectomy and iron-chelation therapy than did those with deletional HbH disease. Also, growth status was compromised in patients with HbH disease. [ABSTRACT FROM AUTHOR]
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Clinical Features and Genotypes of Patients with Hemoglobin H Disease in Taiwan.
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Autor/in / Beteiligte Person: | Lin, Pei-Chin ; Chang, Tai-Tsung ; Liao, Yu-Mei ; Tsai, Shih-Pien ; Chen, Yen-Chu ; Hsu, Wan-Yi ; Su, Hsiu-Lan ; Zeng, Yu-Sheng ; Tseng, Yu-Hsin ; Chiou, Shyh-Shin |
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Zeitschrift: | Laboratory Medicine, Jg. 50 (2019-04-01), Heft 2, S. 168-173 |
Veröffentlichung: | 2019 |
Medientyp: | academicJournal |
ISSN: | 0007-5027 (print) |
DOI: | 10.1093/labmed/lmy043 |
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