title: Hemoglobin H disease in Guangxi province, Southern China: clinical review of 357 patients.
abstract: The clinical characteristics of 357 patients with hemoglobin H (HbH) disease from the Guangxi province of Southern China were studied. One hundred and ninety-one (53.3%) patients were diagnosed with HbH-Constant Spring, 19 were diagnosed with HbH Westmead. Ten patients were shown to have coinherited HbH-Constant Spring/QS with a β-thalassemia mutation. Coinheritance of the β-thalassemia gene does not alleviate anemia (8.2 ± 2.3 vs. 7.6 ± 1.7 g/dl, p = 0.276), or influence age at diagnosis (20.2 ± 19.6 vs. 12.9 ± 11.0 years, p = 0.276). Ferritin levels were significantly higher in the group of patients with the nondeletional form of the disease (475 ± 719 vs. 249 ± 264 ng/ml, p = 0.005).
authors.affiliation: Department of Hematology, 303rd Hospital of PLA, Nanning, Guangxi, China.
authors.forename: Xiao-Llin
authors.initials: XL
authors.lastname: Yin
authors.forename: Xin-Hua
authors.initials: XH
authors.lastname: Zhang
authors.forename: Tian-Hong
authors.initials: TH
authors.lastname: Zhou
authors.forename: Tian-Lang
authors.initials: TL
authors.lastname: Zhang
authors.forename: Rui-Gui
authors.initials: RG
authors.lastname: Luo
authors.forename: Li
authors.initials: L
authors.lastname: Wang
authors.forename: Ya-Li
authors.initials: YL
authors.lastname: Zhou
authors.forename: Yong-Sheng
authors.initials: YS
authors.lastname: Chen
authors.forename: Xiang-Jin
authors.initials: XJ
authors.lastname: Kong
authors.forename: Bo
authors.initials: B
authors.lastname: Liang
authors.forename: Yuan-Yuan
authors.initials: YY
authors.lastname: He
authors.forename: Lin
authors.initials: L
authors.lastname: Peng
authors.forename: Li-Biao
authors.initials: LB
authors.lastname: Lu
authors.forename: Su-Ping
authors.initials: SP
authors.lastname: Fang
authors.forename: Zhi-Kui
authors.initials: ZK
authors.lastname: Wu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D044466:Asian People / Q000235:genetics / Q000706:statistics & numerical data; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D005260:Female; D005293:Ferritins / Q000097:blood; D020022:Genetic Predisposition to Disease / Q000208:ethnology; D005838:Genotype; D006447:Hemoglobin H / Q000235:genetics*; D006456:Hemoglobinuria / Q000208:ethnology* / Q000235:genetics* / Q000378:metabolism; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D055815:Young Adult; D017085:alpha-Thalassemia / Q000208:ethnology* / Q000235:genetics* / Q000378:metabolism; D017086:beta-Thalassemia / Q000208:ethnology / Q000235:genetics / Q000378:metabolism
chemical_list: D005293:Ferritins; D006447:Hemoglobin H