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Publications (20)5.46 Total impact

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    ABSTRACT: To testify whether absolute neutrophil count (ANC) response to preimmunosuppressive-therapy (pre-IST) granulocyte-stimulating factor (G-CSF) treatment could predict early response to IST in severe aplastic anemia (SAA). Clinical data and hematologic response of 125 SAA patients treated with antithymocyte globulin (r-ATG) combined with cyclosporine were retrospectively analyzed. Correlation of ANC response to pre-IST G-CSF treatment and early response to IST were statistically analyzed, and receiver operating characteristic (ROC) curve was used to estimate the value of increased ANC (∆ANC) in predicting early IST response. The hematologic response (HR) rate to IST in ANC reponded patients was significantly higher than non-responded group (3-month HR 49.0% vs 28.9%, P=0.023; 6-month HR 61.2% vs 40.8%, P=0.026). With ∆ANC≥0.5×10⁹/L as cutoff level, the best point to predict early IST response was 10 days after G-CSF (d 10). Response of ANC to pre-IST G-CSF treatment at d 10 was among the independent factors of predicting 3-month (P=0.004), but not for 6-month response to IST. The overall 5-year survival rate was 92.8% and 69.5% in ANC responded and non-responed groups, respectively (P=0.025). Responding to pre-IST G-CSF treatment reflected the residual bone marrow hematopoiesis, and could act as a convenient and practical predictor to early IST response as well as long-term survival in SAA.
    11/2014; 35(11):974-9.
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    ABSTRACT: OBJECTIVE:To identify the characteristics of chronic natural killer cell lymphocytosis (CNKL).
    07/2014; 35(7):609-613.
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    ABSTRACT: To report the clinical data of a case of iron-refractory iron deficiency anemia (IRIDA), so as to improve the understanding of IRIDA.
    06/2014; 35(6):486-90.
  • Li Zhang, Kang Zhou, Fengkui Zhang
    05/2014; 35(5):479.
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    ABSTRACT: To evaluate the value of serum soluble transferrin receptor (sTfR) concentration in predicting early response to immunosuppressive therapy (IST) in severe aplastic anemia (SAA). Clinical data and hematologic responses of 140 SAA patients treated with rabbit antithymocyte globulin (rATG) combination with cyclosporine in our hospital were retrospectively analyzed. Correlation of pre-IST baseline of sTfR and IST responses was statistically analyzed and receiver operating characteristic (ROC) curve was used to estimate the sensitivity and specificity of sTfR in prediction of early responses. Serum concentration of sTfR in very SAA (VSAA)patients were significantly lower than SAA and transfusion dependent non-SAA cases (P=0.001). The responders, especially at 3 months, had significantly higher pre- IST baseline of sTfR[median, 0.89 (range, 0.21-2.42) mg/L]than that [median, 0.58 (range, 0.13-1.88) mg/L]of non-responders (P=0.005). The cutoff level of 0.91 mg/L and 0.88 mg/L for predicting responses at 3 and 6 months were established based on the ROC curve, with the degree of accuracy of 65.0% and 60.7% respectively. Multivariate analysis showed that pre-IST baseline of sTfR was the independent factor of predicting response at 3 months (P=0.007) and at 6 months (P=0.021). As a indicator of bone marrow failure severity, sTfR could predict early response to IST therapy in aplastic anemia.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 08/2013; 34(8):709-13.
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    ABSTRACT: To investigate the clinical features and therapeutic method for severe aplastic anemia (SAA) associated with β-thalassemia, and to improve the recognition of the disease. One patient hospitalized for pancytopenia was reported and the related literatures were reviewed. A 14-years old girl who presented with anemia from her childhood was hospitalized for acute onset of pancytopenia. Routine blood test showed that WBC count was 1.28×10⁹/L, hemoglobin 65 g/L, platelet count 18×10⁹/L, reticulocyte count 2×10⁹/L, neutrophil count 0.03×10⁹/L and mean corpuscular volume 59.6 fl, respectively. Both bone marrow aspiration and biopsy showed hypoplasia. Her red blood cells presented as microcytic hypochromic and target erythrocytes were common on peripheral blood smear. DNA analysis of the patient and her mother showed exon 17 heterozygous β-thalassemia (c.52 A>T). A diagnosis of SAA associated with β-thalassemia was clarified and high-dose cyclophosphamide (HD-CTX, 1.2 g/d×4 d) plus cyclosporine were offeved, which eventually led to a complete hematologic remission 12 months later. This was the first report of SAA associated with β-thalassemia, and the regimen of HD-CTX led to a complete hematologic remission.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 06/2013; 34(6):532-535.
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    ABSTRACT: To investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism. Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed. Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangment in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine. Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 06/2013; 34(6):536-539.
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    ABSTRACT: T-cell large granular lymphocyte leukemia (T-LGLL) is a rare chronic lymphoproliferative disorder. Available reported data on the treatment regimens of this disease are variable and limited due to low number of patients. We analyzed the efficiency of cyclosporine A (CsA) in the treatment of 28 patients with T-LGLL. The overall response rate (ORR) was 82.1% with hematologic complete remission (HCR) rate of 57.1%. The median time to response (TTR) was 1.8 months and treatment duration with CsA-based regimens was 34.5 months. CsA shows low and manageable toxicity during treatment. Twenty-one patients survived with a median follow-up time of 42.0 months. Our results indicate that CsA is efficacious and safe in the treatment of T-LGLL.
    Leukemia research 02/2013; · 2.36 Impact Factor
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    ABSTRACT: A modified high-dose cyclophosphamide (HD-CTX) plus cyclosporine (CsA) regimen was adopted for severe/very severe aplastic anemia (SAA/VSAA) patients, and the effectiveness was compared with that of antithymocyte globulin (ATG) plus CsA regimen. A total of 121 patients enrolled in this study received either CTX plus CsA (CTX group, 48 cases) or ATG plus CSA (ATG group, 73 cases). The early death rate was 4.2% in CTX group and 8.2% in ATG group, showing no significant difference (p=0.312). The total response rate in CTX group and in ATG group was 54.2% and 57.5% at 3 months, 64.6% and 72.6% at 6 months, and 72.9% and 78.1% at 12 months, respectively (p>0.05). The overall 5-year survival rate was 81.2% and 80.7%, and the event free survival rate was 68.2% and 67.3% in CTX and ATG groups, respectively (p>0.05). The total medical cost of CTX group was 54.8% less than that of ATG regimen (p=0.000). In summary, treatment of SAA/VSAA with CTX plus CsA has comparable effectiveness to conventional regimen and less cost.
    Experimental hematology 01/2013; · 3.11 Impact Factor
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    ABSTRACT: To investigate the clinical and laboratory features of congenital dyserythropoietic anemia type II (CDA-II) in order to improve the recognition of the disease. A case of CDA-II was reported and the related literatures were reviewed. The 32-years old female presented with moderate anemia, jaundice and hepatosplenomegaly from her childhood and was misdiagnosed as hereditary spherocytosis for a long time. There were no increased reticulocytes in the peripheral blood and her bone marrow showed erythroid hyperplasia with 43% of binucleated erythroblasts. Electron microscopy examination revealed stretches of double membrane lining the inner surface of the erythroblast cell membrane. CDA-II is a rare congenital anemia characterized by ineffective erythropoiesis with unique laboratory features, and is relatively easy to be misdiagnosed. It is necessary to improve the awareness of CDA-II, and to set-up its responsible gene analysis, i.e., CDAN2 gene and SEC23B gene detection.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 04/2012; 33(4):270-3.
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    ABSTRACT: To evaluate the effects of cyclosporine A (CsA) whole-blood concentration on the early response to immunosuppressive therapy (IST) in severe and very severe aplastic anemia (SAA/VSAA). Ninety SAA/VSAA patients treated with rabbit antithymocyte globulin (ATG) plus CsA as first line therapy in our hospital were retrospectively analysed. CsA levels between the response group and non-response group, and response rates of patients with variant CsA levels were compared respectively. (1) There was no significant difference in the beginning unmodified CsA blood concentration between IST responded and non-responded SAA/VSAA patients. The beginning unmodified C(0) 133.91 ug/L in IST 2-month responders was higher than that of 49.9 ug/L in non-responded SAA patients (P = 0.009); (2) The mean CsA C(0) and C(2) levels during the third month following IST were significantly different in responders and non-responders(197.52 µg/L vs 161.49 µg/L, P = 0.024, and 738.76 µg/L vs 615.46 µg/L, P = 0.009), and no significant difference in other periods of IST (P > 0.05); (3) The response rate (87.5%) was significantly higher in patients with CsA C(0) ≥ 200µg/L the third month following IST than those of 55.6% in patients with CsA C(0) 150 - 200 µg/L (P = 0.023) and 59.3% in patients with CsA C(0) < 150 µg/L (P = 0.046), respectively. The response rate was significantly higher of C(2) ≥ 700 µg/L group than that of C(2) < 700 µg/L group (80.5%vs 55.3%, P = 0.012). The CsA concentration related to the early IST response. The third month CsA concentrations was the most important for the response and maintaining CsA levels with C(0) ≥ 200 µg/L and C(2) ≥ 700 µg/L may improve the response to IST in SAA/VSAA.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 11/2011; 32(11):766-71.
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    ABSTRACT: To analyze the efficacy and side-effects of combination of rabbit antithymocyte globulin (ATG) and cyclosporine A (CsA) as the first-line immunosuppressive therapy (IST) for adult severe aplastic anemia (SAA) patients. Adult SAA or very severe aplastic anemia (VSAA) patients treated with rabbit ATG + CsA as first line therapy in our hospital from 2003 to 2008 were retrospectively analysed and the therapeutic response relevant factors were analysed. Seventy-nine patients were enrolled. Of all these patients, 6 died within 3 months after IST. The overall response rate was 82.2% and the median time to transfusion independent was 60 days. The therapeutic response rate in 32 SAA patients (100%) was significantly higher than that in 41 VSAA cases (68.3%) (P = 0.001). Patients with neutrophil response to G-CSF treatment had a higher IST response rate than those without response to G-CSF (100% vs 67.5%, P = 0.001). Sixty-one patients (77.2%) occurred serum sickness reaction. Three patients relapsed and two developed clonal hematological abnormalities after IST. The 3-year overall survival for all the patients was 88.9%. Rabbit ATG in combination with CsA as first-line IST for adult SAA can lead to excellent treatment outcomes with minor adverse effects.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 01/2011; 32(1):38-42.
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    ABSTRACT: To evaluate bone marrow hematopoietic cells genetic instability (BMHCGI) in patients with aplastic anemia (AA) and to explore its influence on immunosupressive therapy for AA and significance on late clonal hematologic disorders. Genetic instability of bone marrow mononuclear cells (BMMNC) was measured by Comet assay. The relationship between bone marrow failure parameters and genetic instability results was evaluated. The reciprocity of genetic instability and treatment responses to immunosuppressive therapy (IST) was investigated. Comet assay parameters \[tail moment (TM), olive TM (OTM), comet %\] of AA patients were significantly higher than that of control group (P < 0.05). There was no statistic correlation of comet parameters of severe AA (SAA) BM hematopoietic cells with age, gender and peripheral blood cell count (P > 0.05). For the treatment response rate at six months after IST there was no statistical difference between comet cells of < 21.64% and of >/= 21.64%, and so did between OTM < 1.58 and >/= 1.58 in SAA patients. IST had no effect on SAA BMHCGI, whereas, the Comet%, TM and OTM in SAA PR patients and Comet% in CR patients were significantly decreased than those before treatment. Comet parameters of two SAA patients were significantly increased before the development of clonal cytogenetic abnormalities. Increased BMHCGI may be one of the elements in the pathogenetic mechanisms in AA. The genetic instability is irrelevant to the SAA patients overall response rate of IST at six months, but IST can alleviate the genetic instabilities in responded SAA patients.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 04/2010; 31(4):219-22.
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    ABSTRACT: To analyse the efficacy and side-effects of rabbit antithymocyte globulin (ATG) and cyclosporin A (CsA) as the first-line therapy for childhood severe aplastic anemia (SAA). Seventy-one childhood SAA patients treated with rabbit ATG + CsA as first line therapy were retrospectively analysed. Seventy-one SAA patients, including 38 SAA and 33 very severe aplastic anemia (VSAA), were enrolled. The median age was 12 years. Of these patients, 3 died within 3 months after the immunosuppressive therapy (IST). The overall response rate was 67.6% (46/68) and the median time to transfusion independent was 53 days. Thirty-three patients (48.5%) obtained remission in 3 months after the IST and 45 (67.2%) in 6 months. The response rates were 57.7% (15/26), 56.5% (13/23) and 94.7% (18/19) for patients less than 10 years old, 10 - 15 year-old and 15 - 18 year-old, respectively. Sixty patients suffered from serum sickness on the IST. Three patients relapsed and another 3 unrespond patients received retreatment of IST, and one patient progressed to myelodysplastic syndromes (MDS). Rabbit ATG in combination with CsA as first line therapy for childhood SAA/VSAA can lead to overall response rate of 67.6% with minor adverse effects.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 11/2009; 30(11):749-53.
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    ABSTRACT: To analyze the clinical and laboratory features of patients with congenital dyserythropoietic anemia type I (CDA-I), and improve the clinical diagnostic accuracy. The clinical and hematological features of 5 patients diagnosed as CDA-I in our hospital between July 2002 and July 2007 were analyzed retrospectively, and the related literatures was reviewed. Five CDA-I patients, 1 male and 4 females, all had a long history of varied degree of chronic anemia. One patient had congenital malformations, 3 jaundice and 4 hepatosplenomegaly. Bone marrow specimens invariably showed hypercellularity due to erythroid hyperplasia with megaloblastic changes, irregularly shaped nuclear, and chromatin bridges in 0.2% to 0.6% of all erythroblasts. All the 5 patients' bone marrow erythroblasts showed spongy heterochromatin appearances (swiss-cheese) with electron microscopy examination. There was no morphologic abnormality in the granulocytes and megakaryocytes. Serum ferritin levels were increased in 3/4 patients. One patient had been misdiagnosed as hereditary spherocytosis and performed splenectomy in the local hospital with no improvement in Hb level. CDA-I is a rare congenital anemia characterized by ineffective erythropoiesis, jaundice, hepatosplenomegaly and iron overload, and may be misdiagnosed. Keeping these manifestations in mind should avoid misdiagnosis.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 06/2009; 30(6):377-80.
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    ABSTRACT: To analyze the characteristics of T-cell large granular lymphocyte leukemia (T-LGLL). Retrospectively analyze the clinical and laboratory data of 27 patients with T-LGLL diagnosed between 1999 and 2007 in our hospital. The median age at diagnosis was 48 years. All patients were symptomatic, mainly complaining of fatigue. Of the 27 patients, 14 (51.9%) had splenomegaly, and 4(14.8%) hepatomegaly. Rheumatoid arthritis was not present in any patients. The most frequent hematological abnormality was anemia (24 patients, 88.9%) with a median Hb level of 57.5 g/L. Pure red cell aplasia was found in 18 patients (66.67%). The median WBC count was 4.24 x 10(9)/L and 19 cases were neutropenia (ANC < 1.5 x 10(9)/L). The median LGL count in peripheral blood was 1.45 x 10(9)/L and most of them (77.8%) were less than 2.0 x 10(9)/L. Twenty-two patients (81.5%) showed the CD3+ CD8+ CD57+ CD56(-) LGL phenotype. With immunosuppressive therapy, 91.3% of patients responded and complete hematological remission rate was 65.2%. T-LGLL mainly presented with anemia and complete hematological remission rate was 65.2%. Pure red cell aplasia was commonly associated with the disease. The patients had a good response to immunosuppressive therapy.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 03/2009; 30(3):179-82.
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    ABSTRACT: To investigate the impact of immunosuppressive therapy (IST) on genetic instabilities of bone marrow hematopoietic cells (BMHCs) in patients with aplastic anemia (AA). Comet assay as used to detect genetic instabilities of hematopoietic cells from patients, and the percent of DNA in comet tail (TDNA), tail length (TL), tail moment (TM), olive tail moment (OTM) and the rate of comet cells were measured. BMHCs from AA patients were examined with comet assay before and after IST, and the results were compared with those from controls. Comet parameters from 91 AA patients including TDNA, TL, TM, OTM comet cell percentage were (5.0 +/- 4.0)%, 11.3 +/- 7.2, 1.7 +/- 2.0, 1.5 +/- 1.4, (16.8 +/- 13.7)%, respectively, which were significantly higher than those from control group (P < 0.05). There were statistical differences between the comet parameters of severe AA (SAA)/non-SAA (NSAA) and those of control group (P < 0.05), but no difference in the comet parameters between SAA and NSAA patients (P > 0.05). The TDNA, TL, TM, OTM and comet cells percentage were (4.4 +/- 3.6)%, 10.4 +/- 7.5, 1.4 +/- 1.6, 1.3 +/- 1.4 and (20.2 +/- 21.2)%, respectively at 3 months after IST in 53 SAA patients and were (3.7 +/- 3.3)%, 10.0 +/- 7.2, 1.2 +/- 1.8, 1.1 +/- 1.3 and (18.5 +/- 19.0)% respectively at 6 months after IST in 30 SAA patients, being no statistical difference from those of 58 SAA patients before IST (P values were all > 0.05). BMHCs of AA had inherent genetic instabilities which were not increased by recent IST. It indicated that there was no correlation between IST and the development of clonal hematologic disorders in AA.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 12/2008; 29(11):728-32.
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    ABSTRACT: To analyze the characteristics of acquired pure red cell aplasia (PRCA) secondary to T cell large granular lymphocyte leukemia (T-LGLL). Fourteen patients with T-LGLL associated with PRCA between 2000 and 2006 in our hospital were retrospectively analyzed. The median age at diagnosis was 61 years with equal gender distribution. The PRCA had indolent process, mainly presenting with anemia. Of the 14 patients, 9 had mild to moderate splenomegaly, one hepatomegaly and one lymphadenopathy. The median Hb level was 61.5 g/L and the median WBC count 4.3 x 10(9)/L. The median percentage and count of LGL in peripheral blood were 0.36 and 1.9 x 10(9)/L respectively. The median percentage of LGL in BM was 0.165 (0.085 - 0.410). Some patients had serologic abnormalities. All the 12 cases with available bone marrow cell cytogenetics showed normal karyotypes. With cyclosporine A or glucocorticoid immunosuppressive therapy, the overall response was 91%. T-LGLL was one of the major causes of acquired PRCA. This type of PRCA has the similar clinical and laboratory feature to that of other type of PRCA and has a good response to immunosuppressive therapy.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 06/2008; 29(5):312-5.
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    ABSTRACT: To reveal the clinical features of patients with acute arrest of hemopoiesis (AAH), and explore the dissimilarity between AAH, severe aplastic anemia (SAA) and very severe aplastic anemia (vSAA). The clinical and laboratory features of 23 AAH patients diagnosed and treated in our hospital from May 1993 to May 2006 were analysed retrospectively and compared to the 111 cases of SAA and vSAA patients diagnosed at anaemia therapeutic centre of the hospital from Jul 2002 to May 2006. Twenty-three patients accorded with the criteria for AAH, and 16 of them with the criteria for severe acute arrest of hemopoiesis (SAAH). They could spontaneously reconstitute their bone marrow hematopoiesis at a median of 17 days (range, 8-50), and had remarkable older age (median age 35.5 vs 21), positive history of other disease and taking medication. Fever as one of presenting symptom were noticed in 10 of 16 STAA patients. The laboratory results were similar with SAA or vSAA, had more frequent decreased serum albumin level and total iron binding capacity and higher CFU-GM. Patients with AAH often had similar clinical symptoms with SAA or vSAA. Although they were diagnosed retrospectively, identification of the pathogenesis and laboratory examination may help for the early diagnosis.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 12/2007; 28(11):750-3.
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    ABSTRACT: To study the bone marrow microvessel density (MVD) and vascular endothelial growth factor (VEGF) expression and their clinical significance in patients with aplastic anemia (AA). Bone marrow biopsies in 51 newly diagnosed patients with AA were evaluated the MVD and VEGF expression by immunostaining with anti-factor VIII related antigen and VEGF monoclonal antibodies at regular time points after immunosuppressive therapy (IT). The mean bone marrow MVD in AA group was 5.5 +/- 3.5, being significantly lower than that in normal control group (8.7 +/- 3.4, P < 0.05). MVDs of SAA and NSAA patients were 7.4 +/- 2.9 and 4.3 +/- 3.4, respectively, being significantly different (P < 0.01). The VEGF expression in AA group was significantly lower than that in control group [(6.7 +/- 8.4)% vs (14.7 +/- 6.1)%, P < 0.01], but there was no difference between SAA and NSAA. Bone marrow MVD and VEGF were significantly increased after IT in 22 responded AA patients. Bone marrow MVD and VEGF expression are low in AA patients which may be one of pathophysiologic mechanisms of bone marrow failure in AA. Proangiogenic and ameliorating microcirculation agents together with IT might accelerate the recovery of hematopoiesis in AA patients.
    Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 09/2007; 28(8):528-31.