110032 100052 530021 530021 α- β- 3 α- β- 20 80 [1] 2009-11-04 2009-12-09 14 2009 973 2010CB530406 90409003 β- 30171199 β- mrna Tel 010-88001149 E-mail gamwuzhikui@sina.com 65
2010 Vol.12 No.1 [2 4] 2. β- Hemoglobin Hb 1. [2] WHO 1 30 β- 20 90 α- 40% [2~4] [3~4] 5- [5] 3 2000 [2 8] 1/4 α- Bart s β- β- β- WHO EB118/5 2006 5 4 66
1. β- 6 β- 3 [8~11] 1991 β- [5~7] 6 β- 3 5 1 β- 2004 10 ~2008 3 α- β- β- 1991 β- β- [5~6] β- 1988 CRF [5~7] β- α-hbh 2. 1. 1 2 Hb <120g L -1 4 1, 3 [15] 3. 1 3 2. 2 2 β- α-hbh 67
2010 Vol.12 No.1 177 303 β- 96 α-hbh 81 107 70 2~47 13.9±8.0 120 50 6-1 Hb 34~120 g L -1 79.6±18.6 g L -1 [14~15] RBC 1.7 10 12 ~6.3 10 12 L -1 4.1±1.0 10 12 L -1 Ret 0.5~23.0% 5.8± 3.9 % 96 β- 3 Hb 73.97±19.977 g L -1 84.75±21.07 g L -1 RBC 3.69 ±1.03 10 12 L -1 4.05±0.91 10 12 L -1 Ret 5.27± 4.07 % 7.47±5.84 % HbF 38.72±28.22 % 45.47±29.08 %, 1~3 P<0.01 81 α-hbh 3 α-hbh Hb RBC Ret 1~3 P< 0.01 Hb 86.16±14.45 g L -1 92.92±12.77 g L -1 RBC 4.48±0.71 10 12 L -1 4.71±0.64 10 12 L -1 Ret 6.36±3.53 % 7.53±3.88 % α-hbh β- α-hbh 300 1000 Hb β- 5g L -1 96 β- 78 81.3% 17 17.7% 81 α-hbh 62 76.5% 18 22.2% α- β- α- β- 79.1% β- α-hbh 68
Hematolgy 1996 33 1 70~73. 7. 1993 9 147~148. 8 β-.. 2006 3 293~302. mrna 9 Wu Zhikui Fang Suping Zhang Xinhua molecular priciple in treating β-thalassemia. CJIM 2003 9 4 [15~21] 2006 26 4 352~354. 11. β-. 2008 49 2 170~172. 12. 2007 48 8 726~729. 13 Fang Suping Wu Zhikui 2007 30 11 2084~2087. 15 1.. 7 591~594. 1999 40 10 626~628. 2. 2003 152 218. 3 Schrier SL Rachmilewitz E Mohandas N.Celluar and membrane properties of alpha and beta thalassemic erythrocytes are different:implication for differences in clinical monifestations. Blood. 1989 74 6. mrna. 2006 35 9 36~37. 2194~2202. 4 Scott MD Rouger -Fessard P Ba MS et al.alpha -and beta - haemoglobin chain induced changes in normal erythrocyte deformability:comparison to beta thalassaemia intermedia and HbH disease. Br J Haematol 1992 80 4 519~526. α-. 2007 32 7 609~612. 5.. 1998 48~59. 21 Zhang Chong Wu ZhiKui. Macular Pharmacological Basis of the YiSui 6 Cao A Calanello R Rosatelli MC et al. Clinical Experience of Man- ShenXu Granule in β-thalassemia therapy. Journal of Ethnopharmacolo- agement of β Thalassemia The Sardinian Experience. Seminars in. β- 6. - 2005 et al. Clinical effecancy and mechanism of nourishing shen and supplementing marrow 248~253. 10. β- 156.. Zhang Xinhua. Clinical observation on YiSuiShengXueGranule on Treating 156 Patients with β-thalassemia Major and the Molecular Mechanism Study. Biol. Pharm. Bull 14. β-. 2007,14 3 9~11... 2009 50 1 73~75. 16.. 2005 25 17. β- 18.. 2006 12 8 589~591. 19. β- α- AHSP GATA-1. 2006 4 3 247~250. 20. gy. 2008 120 2008 437~441. Intensive Study of Traditional Chinese Medicine Theory "Kidney Engenders Marrow" by Treating Beta-thalassemia Patients with Kidney Tonifying Formula Wu Zhikui 1 Fang Suping 1 Zhang Xinhua 2 Liu Yongmei 1 Wang Lei 1 Yi Jie 3 Wang Wenjuan 4 Li Min 1 Chai Limin 1 Wang Rongxin 2 Zhang Chong 1 Chen Yuying 1 Lv Xinxia 1 1. Guang'anmen Hospital, China Academy of Chinese Medicine Science, Beijing 100053, China ; 2. 303 Hospital of People's Liberation Army, Nanning 530021, China; 69
2010 Vol.12 No.1 3. Liaoning Unuversity of Tradtional Chinese Medicine, Shenyang 110032, China; 4. Capital Medical University, Beijing 100052, China Abstracts: Beta-thalassemia is an inherited disorder of hemoglobin synthesis with a high morbidity, but no effective measures are available for its treatment. This work aimed to carry out an intensive study concerning the Traditional Chinese Medicine Theory "Kidney Engenders Marrow" by treating beta -thalassemia patients with the kidney tonifying formula. Randomized, single-blind, placebo parallel-controlled and self-cross-referred trials were adopted. The beta-thalassemia patients (both α and β types ) from Guangxi Provice, an area with a high morbidity in China, were treated with YiSuiShengXueGranule (YSSXG) for 3 months. The results indicated that both two types (α and β) of patients suffering the same TCM syndrome of essence and blood deficiency and the kidney-yin and liver-yin got positive effects after the treatment with YSSXG. The significant improvement of the TCM syndrome kept in accordance with the enhancement of blood indexes. This study evidenced the objectivity of the nuclear TCM mechanism for treating beta-thalassemia, and partly revealed its effective specialty and main points. Keywords: Beta-thalassemia; Clinical; Nuclear mechanism; Kidney produce marrow; Marrow produce blood 70