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ABSTRACT: To assess the value of magnetic resonance imaging T2* tests in the detection of myocardial and liver iron overload in patients with β-thalassemia major (β-TM).
From 2010 to 2011, 28 β-TM patients over 10 years old under blood transfusion therapy and chelation care with serum ferritin (SF)>1000 µg/L underwent myocardial and liver MRI T2* tests on a voluntary basis. The results were analyzed in relation with age, SF, and left ventricular ejection fraction (LVEF).
Fourteen out of the 28 cases (50%) were found to have myocardial iron overload, including 7 severe cases, 2 moderate cases, and 5 mild cases. All the 28 cases had liver iron overload, including 2 mild cases, 7 moderate cases, and 19 severe cases. Two out of the 28 cases had lowered LVEF (7.14%), and one of them had severe myocardial iron overload. There was a negative correlation between myocardial MRI T2* and SF (r=-0.479, P=0.01). Myocardial MRI T2* was positively correlated with liver MRI T2* (r=0.378, P=0.047). Age was not significantly correlated with SF, LVEF, or liver MRI T2*.
Magnetic resonance imaging (T2*) detection is an effective and non-invasive means for detecting myocardial and liver iron overload in patients with β-thalassemia major receiving blood transfusion. T2* combined with SF is the main diagnostic indicator to assess iron overload in the vital organs.
Nan fang yi ke da xue xue bao = Journal of Southern Medical University 02/2013; 33(2):249-52.
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ABSTRACT: To analyze the risk factors of hemorrhagic cystitis after allogeneic hematopoietic stem cell transplantation for beta-thalassemia in children.
The clinical records of 30 children with beta-thalassemia undergoing allogeneic hematopoietic stem cell transplantation between December, 2008 and November, 2009 were analyzed.
Hemorrhagic cystitis occurred in 8 of the 33 patients with an incidence of 24.24%, including 1 with grade I, 6 with grade II and 1 with grade III hemorrhagic cystitis. The median time of hemorrhagic cystitis onset was 22.9 days (range 6-35 days) and the median duration was 11.9 days(range 3-27 days). Univariate analysis indicated that the different types of transplantation and acute graft-versus-host disease affect the occurrence of hemorrhagic cystitis. The children with Allo-PBSCT had higher incidence than those receiving Allo-PBSCT+Allo-UBT and Allo-BMT (P<0.05). The children at an age >or=6 years had obviously higher incidence of hemorrhagic cystitis than those at younger ages.
Age is the major factor that affects the occurrence of hemorrhagic cystitis in children undergoing allogeneic hematopoietic stem cell transplantation for beta-thalassemia.
Nan fang yi ke da xue xue bao = Journal of Southern Medical University 04/2010; 30(4):838-41.
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Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 01/2010; 12(1):73.
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ABSTRACT: The aim of the study is to review the surgical experiences with pectus excavatum (PE) chest deformities at the Department of Pediatric Surgery, West China Hospital of Sichuan University (Sichuan, PR China), during a 30-year period.
Records of 398 PE patients (396 congenital, 2 acquired) who underwent surgical repair between 1975 and 2005 were reviewed. Modified sternal elevation was applied in all patients. Repair was performed with subperiosteal resection of the abnormal cartilages, transverse wedge osteotomy of the anterior sternum, and internal support with a metal strut for 1 year. Five technical details were strictly followed for each case. Three hundred twelve patients (78.39%) were followed up from 1 to 16 years.
There were no deaths. Normal contour of the costal cage was constructed postoperatively in 98.74% (393/398) of the patients. Exercise tolerance was improved, and cardiac function recovered to the healthy level of same age. But pulmonary function recovered slowly after surgery.
The 5 technical details are key principles for sternal elevation. Normal appearance of chest wall can be recovered; normal cardiopulmonary function can be restored by the modified sternal elevation with excellent long-term physiologic, cosmetic results and low rate of complications.
Journal of Pediatric Surgery 07/2008; 43(7):1270-4. · 1.45 Impact Factor
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ABSTRACT: To explore the effect of mouse bone marrow mesenchymal stem cells (MSCs) on the expression of chemokine receptors in T lymphocytes in vitro.
Mouse bone marrow MSCs were separated with Percoll, cultured and expanded in low glucose DMEM. C57BL/6 mouse spleenocytes were cultured in the 24-hole flasks by the density of 1 x10(6)/hole. Phytohemagglutinin (PHA) was then added to the holes and cultured for 72 hrs. This study consisted of three groups. Groups A and B were co-cultured by adding MSCs as the ratio of 0.1 and 0.01 to spleenocytes respectively. The control group was cultured without MSCs. Three days later the suspended spleenocytes were harvested for detecting the expression of three chemokine receptors CXCR3, CCR5 and CCR7 in T lymphocytes by the flow cytometry.
The expression of CD3(+)CCR5(+) and CD3(+)CCR7(+) were statistically different among the three groups. Group A had the strongest expression, followed by group B and the control group. The expression of CD3(+)CXCR3(+) in group A was statistically higher than that in group B and the control group.
MSCs could up-regulate the expression of chemokine receptors CXCR3, CCR5 and CCR7 in T lymphocytes stimulated by PHA.
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics 01/2008; 9(6):571-3.
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ABSTRACT: With more precise diagnostic criteria and risk classifications, more effective therapy administered in clinical trials, and better supportive care, the outcome of children with acute lymphoblastic leukemia (ALL) has been improved dramatically. Today, approximately 80% of children treated for this disease in developed countries enjoy long-term event free survival (EFS) and in most instances, would be cured. In this study, treatment outcome of 82 childhood ALL patients in the hospital were analyzed, and ways for how to improve the EFS rate in childhood ALL were explored.
Eighty-two patients with ALL were enrolled into the Nanfang ALL 99 protocol which derived from German BFM ALL 95 and Hong Kong-Singapore acute lymphoblastic leukemia 97 (HK-SG ALL 97). Dexamethasone instead of hydrocortisone was used for triple intrathecal therapy. Standard at risk patients who had been irregularly treated in other hospitals for short periods of time were classified as at intermediate risk. When ANC was > or = 1.0 x 10(9)/L and platelet > or = 100 x 10(9)/L, chemotherapy was started. Life table method was used to estimate survival rate and statistical analysis was done by using software SPSS for Windows.
From March 1999 to September 2003, 82 childhood ALL patients were treated with the Nanfang ALL 99 protocol and 78 (95.1%) patients attained complete remission (CR) in a median time of 33 days. Out of 82 patients, 13 patients dropped out of the the Nanfang ALL 99 protocol because of financial difficulty or other reasons. Sixty nine patients were consecutively treated with the Nanfang ALL 99 protocol. The overall EFS rate at 2 years, 3 years and 5 years were 91.3%, 85.9% and 75.2%, respectively, with a median observation duration of 34 months. Three patients died of complications (4.3%). The disease relapsed in 6 patients and they died finally.
The outcome of patients treated with the Nanfang ALL 99 protocol was favorable, and the mortality rate of this chemotherapeutic protocol was low. This protocol was well tolerated by Chinese patients and therefore the protocol is worthy of application in China.
Zhonghua er ke za zhi. Chinese journal of pediatrics 01/2006; 43(12):890-3.
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ABSTRACT: To investigate the effect of hematopoietic stem cell transplantation (HSCT) for beta-thalassemia major.
Fifteen beta-thalassemia major patients with a median age of 3. 5 years (range 1 - 10 years) received allogeneic HSCT. According to the Pesaro's classification for thalassemia, 12 patients were grade I - II, and 3 grade III. The bone marrow transplantation (BMT) plus peripheral blood stem cell (PBSC) transplant mobilized by granulocyte colony-stimulating factor (G-CSF) was used when donor is low body-weight.
Of the fifteen patients, nine were disease-free survival (DFS) at a median follow-up of 2.5 years (range 6 - 54 months). Of eight grade I - II patients received HLA identical sibling BMT, seven were DFS, and of two received HLA mismatched marrow from their mother, one DFS, another not engrafted. Two patients received unrelated cord blood HSCT were both not engrafted. Two patients received PBSC transplantation alone were not engrafted, but one of them soon received BMT from the same donor and was DFS. The incidences of grade I - II and grade III acute graft-versus-host disease (aGVHD) were 20% (3/15) and 6.7% (1/15), respectively. Interstitial pneumonia occurred in 4/15 (26.7%) patients. There were no long-term complications in the survivors.
Grade I - II beta-thalassemia major patients received HLA identical sibling BMT had higher DFS. It was propitious for engraftment to use BMT plus PBSC, but with a higher incidence of acute and chronic GVHD.
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi 05/2003; 24(4):174-6.
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Zhonghua er ke za zhi. Chinese journal of pediatrics 03/2003; 41(2):150-1.
