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06 June 2007, Volume 22 Issue 06 Previous Issue    Next Issue
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Research on AGT(M235T) and ACE(I/D) gene polymorphisms in children with primary nephrotic syndrome. MENG Qun,SHEN Ying,JIANG Zai-fang. 2007, 22(06): 420-423 .  Abstract ( )   AbstractObjectiveTo investigate angiotensinogen(AGT) M235T and angiotensin converting enzyme (ACE) gene insertion/deletion (I/D) polymorphisms,and their effects on clinical features of northern Han nationality children with primary nephrotic syndrome (PNS). MethodsPolymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) techniques was used to identify the AGT (M235T) and ACE(I/D) gene polymorphisms in 148 northern Han nationality children with PNS and 50 children as control group. ResultsThe ACE DD genotype,D allele and the AGT MT genotype frequencies were significantly higher in PNS group than that in the control group(P=0.004,0.032,0.036).The ratio of the patients with D and T genotype simultaneously was also higher (P=0.004).D allele frequency was significantly higher in PNS patients with relapse than that of the patients without relapse (P=0.000).The ACE DD genotype frequency was higher in steroid-resistant PNS than the steroid responsive PNS(34.21% vs 20.91%).The ACE DD genotype frequency was higher in patients with both hypercholesterolemia and hypertriglyceridemia than the control group (P=0.006).The lengths of the inserted gene sequence of ACE gene was 289bp.There were several nucleotides polymorphisms of the inserted sequence.They were at position 103 and 151;one G was inserted separately. ConclusionNorthern Han nationality children with ACE(D allele) genotype,with AGT(MT) genotype,with both D allele of ACE gene and T allele of AGT gene simultaneously may correlate with a predisposing factor for PNS.Patients with DD genotype may have much higher risk of relapse,hypercholesterolemia and hypertriglyceridemia and steroid resistence.The length of the inserted gene of ACE is 289bp,and the necleotide sequence is somewhat different from that of foreign countries.

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The study of serum procalcitonin and interleukin-6 levels in children with Kawasaki disease. Wang Yun,Wang Li,Sun Liping 2007, 22(06): 424-427 .  Abstract ( )   AbstractObjectiveTo study the changes of serum PCT and IL-6 levels in Kawasaki disease (KD) patients, and evaluate the relationships of them with complications. MethodsTotally 88 KD patients and 33 healthy children were recruited in this study. KD patients were divided into different groups. Serum PCT and IL-6 concentrations of KD patients were compared with healthy children, and compared within different groups. Results(1) Serum PCT concentrations of acute patients were significantly higher than subacute patients and healthy children ,0.55(0.30～1.81)ng/ml vs. 0.49(0.20～0.50)ng/ml(P<0.05) and 0.55(0.30～1.81)ng/ml vs. 0.16(0.14～0.24)ng/ml(P<0.01). (2) Serum concentrations of PCT were significantly higher in acute patients with noncardiac complication as compared to those without noncardiac complication, 1.67(0.49～4.14) ng/ml vs. 0.47(0.27～0.74) ng/ml (P<0.01). Noncardiac complications occurred in 16.3%(8/41) acute patients if PCT levels were ≤0.5ng/mL, and the incidence increased to 41.0% (16/23) if PCT levels were > 0.5ng/mL, χ2 = 6. 68( P<0.05). (3) Serum IL-6 concentrations of acute patients were significantly higher than subacute patients and healthy children, 43.34（13.67～132.72）pg/ml vs. 8.62（7.56～56.54）pg/ml(P<0.01) , and 43.34（13.67～ 132.72）pg/ml vs. 8.80（6.00～13.05）pg/ml(P<0.01). (4) Significantly higher serum IL-6 concentrations were shown in acute patients with noncardiac complication than those without noncardiac complication, 110.97(21.00～148.79) pg/ml vs. 26.13(9.34 ～78.84) pg/ml, P<0.01. Conclusion(1) There is significant elevation of serum PCT and IL-6 concentrations in all KD patients,and the concentrations of them gradually decreased with the development of KD. High levels of PCT can last longer than those of IL-6. (2) Significantly high serum PCT and IL-6 concentrations were observed in KD patients with noncardiac complications,and it may be a useful index for evaluating KD patients.

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The clinical analysis of Kawasaki disease in little infants under 4 months. LI Tie-geng,SHI Xiang-li,QIN Yu-chun. 2007, 22(06): 428-430 .  Abstract ( )   AbstractObjectiveFor early diagnosis of Kawasaki disease and to decrease the misdiagnosis rate,this article analyze clinical features of Kawasaki disease in the infants. MethodsThe clinical data of 30 infants aged 1 to 4 months suffering from Kawasaki disease were retrospectively analyzed. ResultsIn this study,the number of boys was more than that of girls.No differences in seasons.The incidence of the typical Kawasaki disease was higher,but the atypical Kawasaki diseases still existed in about 20% patients.The average final diagnosis time of the atypical Kawasaki disease was longer than that of the typical one and the misdiagnosis rate was higher. ConclusionThe abnormality of the coronary artery is the only gold index for final diagnosis of the atypical Kawasaki disease.Meanwhile,when the following things happen you ought to think of the disease in the baby who is under 4 months old:fever persisting at least 5 days,ardent fever again during the convalescent period of infection disease,and persistent fever after premonition.

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Assessment of left ventricular function by mitral annular velocity in heart failure children. LIU Xue-qin,SONG Xiu-ting,WANG Yu-li 2007, 22(06): 431-433 .  Abstract ( )   AbstractObjectiveTo investigate the change of mitral velocity by tissue Doppler imaging(TDI) and to evaluate the value of TDI method in the assessment of left ventricular function in heart failure children. MethodsThe study consisted of two groups: heart failure (HF) group consisted of 21 children aged 8 months to 17 years with heart failure (HF group) and left ventricular enlargement and a left ventricular ejection fraction(LVEF)<50%,heart function was graded by NYHA;another 21 age-and sex-matched normal children were as the control group.Conventional echocardiography was done to obtain left ventricular ejection fraction(LVEF) and transmitral inflow E and A wave velocities and E/A ratio.Pulsed wave TDI velocities were obtained in the lateral and septal mitral annulus(MA-L,MA-S) separately and Sa,Ea and Aa velocities were masured;Ea/Aa and E/Ea were calculated. ResultsSa,Ea and Ea/Aa at both site of the mitral annulus were significantly lower(P all <0.01),and E/Ea significantly higher (P all <0.01) compared with that of the control group respectively.Sa of both sites and Ea of MA-L correlated positively with LVEF(r=0.60,0.44,0.52,P all <0.05);Sa and Ea of both sites correlated negatively with NYHA grading(r=-0.64,-0.55,-0.68,- 0.54,P all <0.05);E/Ea correlated negatively with LVEF(r=-0.59,-0.47,P all <0.05) and positively with NYHA grading (r=0.77,0.75,P all <0.001).Sa also correlated closely with Ea and E/Ea at each site. ConclusionChildren with heart failure have both left ventricular systolic and diastolic disfunction,and they have a close correlation.Mitral annulus velocity detected by TDI is sensitive and reliable to evaluate left ventricular function in heart failure children.

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Investigation of IVIG resistant Kawasaki disease. WANG Min,ZHAO Xiao-dong,TANG Xue-mei 2007, 22(06): 434-436 .  Abstract ( )   AbstractObjectiveTo investigate the prevalence,outcome,risk factors and treatment of IVIG resistant Kawasaki disease. MethodsMedical charts of 233 cases with a discharge diagnosis of Kawasaki disease from January 2005 to December 2005 were reviewed retrospectively in Children's Hospital of Chongqing Medical University.A follow-up survey was also given. ResultsThirty-one (13.3 percent) of the 233 patients failed to respond to initial treatment with IVIG.The male to female ratio of IVIG resistant Kawasaki disease was 4.17,significantly higher than IVIG responsive Kawasaki disease (1.43) (P<0.05).The age distributions of the 2 groups were not different.The patients who did not respond to IVIG had higher and more severe coronary artery lesions (CAL) compared with IVIG responder (6 of 31 (19.4 percent) versus 5 of 202(2.4 percent)).The following data were obtained and compared between resistance and responder: white blood cell,neutrophil,hemoglobulin,C-reactive protein,erythrocyte sedimentation rate,albumin,and lactate dehydrogenase(LDH).Male patients with decreased albumin may be related with resistance,but they could not be good predictors of resistance.Risk factors of coronary artery leisions with IVIG resistant Kawasaki disease were not found.Additional infusions of IVIG had effects on most IVIG resistant Kawasaki disease (14/31);steroid treatment was given to 8 patients whom additional infusion of IVIG had no effects on. ConclusionIVIG resistant Kawasaki diseases are not rare,and they have higher and more severe coronary artery lesions.The risk factors of IVIG resistance and coronary artery leisions with IVIG resistant Kawasaki disease are not sure.Additional infusion of IVIG and steroid treatment with antiplatelet and anticoagulant medications have good effects on IVIG resistant Kawasaki disease.

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The discussion of carotid artery ultrasound in diagnosing early atherosclerosis in obese adolescents. MAN Yong-xian,ZHENG Lin-lin,GUO Jun-yan 2007, 22(06): 437-438 .  Abstract ( )   AbstractObjectiveTo discuss carotid artery ultrasound method to diagnose early atherosclerosis of obese adolescents. MethodsChoose 47 moderate and severe obese adolescents and 36 natural adolescents randomly in Dandong city in July 2006,and they were checked in carotid artery with ultrasound. ResultsIn obese adolescents,the internal membranes were rough and unslick,with bad continuity and locational incrassation.The thickness of internal membrane(IMT) in common carotid artery was higher in obese adolescents than in natural ones(P<0.05),and the early atherosclerosis change of common carotid artery had positive correlation with obese degree (P<0.05). ConclusionObese adolescents may subsequently have atherosclerosis,and carotid artery ultrasound can be a preferred method to diagnose atherosclerosis.

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Determination and significance of serum thyroid hormones in pediatric patients with congestive heart failure. YANG Feng-mei,LI Ya-rui,QU Qi-ming 2007, 22(06): 439-441 .  Abstract ( )   AbstractObjectiveTo explore the changing rule of serum thyroid hormones(TH)in pediatric patients with congestive heart failure (CHF) through measuring the serum TH level in patients with CHF. Methods (1)Serum TH of patients with CHF were measured with radioimmunoassy and were compared with that of none CHF (N-CHF) group as well as normal control group.(2)The correlation between serum HT level and cardiac function was analysed.(3)Serum TH in patients with CHF was dynamically observed. Results(1)Triiodothyronine (T3) 、 free triiodothyronine (FT3) and free tetraiodothyronine (FT4) were significantly lower in patients with CHF in comparison with N-CHF group and normal control group (P<0.01),while reversed triiodothyronine(rT3) was significantly higher (P<0.01).(2)Along with the aggrevation of heart failure,serum level of T3 、 FT3、tetraiodothyronine (T4) and FT4 progressively decreased,while rT3 progressivly increased.(3)After 3 weeks treatment,serum TH level recovered in patients with remission of heart failure(P<0.01),while that in patients with stubborn heart failure did not recover. Conclusion (1) Serum thyroid hormones' changes exist in pediatric patients with CHF and the extent of its change is correlated with cardiac function.(2)As the heart failure is corrected the abnormal serum TH level restores.A persistent abnormal serum TH is often found in patients with stubborn heart failure and implicates an unfavorable therapeutic effect.

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The value of TT for diagnosing vasovagal syncope in children and the clinical efficacy of drinking-water. HUANG Yu-juan,HUANG Min,CHEN Xiu-yu 2007, 22(06): 442-444 .  Abstract ( )   AbstractObjectiveTo study the value of Tilt-table test (TT) for diagnosing vasovagal syncope and the clinical efficacy of drinking- water. MethodsIn prospective study,74 patients with unexplained syncope and 54 healthy children received the TT to 80° for 30 minutes.Positive-to-negative test was performed in the positive group,who eventually were treated with drinking-water,and were followed up. ResultsThe classic TT was positive in 55.4% of patients with unexplained syncope,but none in control subjects.Three patterns of positive response to TT were observed :vasodepressor pattern in 21/41,cardioinhibitory pattern in7/41,and mixed pattern in 13/41.Among the positive group,28/41turned negative after drinking water.Follow-up was available to 38 patients,the total effective rate being 86.8%. ConclusionTT has higher sensibility and specificity to the diagnosis of vasovagal syncope.Positive-to-negative test provides rational and experimental evidence.Drinking-water is a simple and effective measure for the prevention of vasovagal syncope.

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Intense immunoablative therapy for childhood refractory systemic lupus erythematosus:observation of 5 cases. LUO Xue-qun,MO Ying,KE Zhi-yong 2007, 22(06): 445-447 .  Abstract ( )   AbstractObjectiveIn this trial we investigated the safety and efficacy of nonmyeloablative but intense immunoablative chemotherapy without SCT in the treatment of childhood refractory SLE. MethodsFive refractory SLE patients were female aged 10~16 at the study，4 with WHO class Ⅳ nephritis，2 with immune hemolytic anemia and 1 with immune thrombocytopenia.Previous therapy including monthly I.V.cyclophasphamide (CTX) and high -dose methylprednisolone failed for the 5 cases.Among these cases,4 received regimen A and 1 received regimen B.The disease activity was measured by SLEDAI before and after the treatment. ResultsThere was no treatment-related death.The median time to achieve a neutrophile count of 0.5×109/L was 10 days after the last dose of chemotherapy in regimen A,and the time was 16 days in regimen B.The average SLEDAI decreased from 9.2 to 4.4,and the proteinuria decreased from (++++) to (0~+) in 3 weeks after the treatment.Two cases with complication of hemolytic anemia and 1 with thrombocytopenia before the treatment recovered to normal or nearly normal hemogram respectively.Among these 5 cases,1 patient had a short time improvement during the therapy,but died 50 days later because of refractory thrombosis of renal vein; 4 other patients achieved complete or partial remission within 5~16 months follow-up. ConclusionDose-controlled high-dose chemotherapy leads to hemotopoietic reconstitution without SCT and has significantly clinical benefit in children with refractory SLE by immunoablation.Therefore this approach deserves further study.

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Effect of all-trans retinoic acid combined with arsenic trioxide in chemotherapy on childhood acute promyelocytic leukemia. LI Yang,LAI Dong-bo,FANG Jian-pei 2007, 22(06): 448-451 .  Abstract ( )   AbstractObjective To evaluate effect of all-trans retinoic acid combined with arsenic trioxide in chemotherapy on childhood acute promyelocytic leukemia (APL). MethodsRetrospective analyses was done on the data of 14 cases of childhood APL who received combined treatment of all-trans retinoic acid with arsenic trioxide in the chemotherapy between 1999 and 2005:ten boys and four girls aged from 2.1 to 12 years old,mean age 6.7 years old.All the children had anaemia,skin ecchymosis or gum bleeding on onset.The percentage of blast plus juvenile cell in diagnostic bone marrow ranged from 59%to 97.5%, mean 80.71%±10.96%.There are nine cases of M3a and five cases of M3b according to FAB classification.PML-RAR alpha fusion gene at diagnosis was performed in eight patients,positive in six cases(75%).The effects of different therapeutic regimens or these regimens on various FAB grouping and relapse rate were analyzed. ResultsTwelve of the 14 patients (85.71%) achieved CR1 after the induction therapy.The median of CR1 was five weeks (ranged from two to nine weeks).The consolidation therapy consisted of arsenic trioxide,DA,HA,EA and DEA regimens or combined with other chemotherapies;two M3b patients relapsed.There were no differences for relapse rate among various strategies of consolidation (P=0.195);maintenance therapy consisting of arsenic trioxide may decrease the relapse rate.There was no significant difference for relapse rate with or without VP/6MP and MTX in the maintenance therapy (P=0.66). ConclusionCombined all-trans retinoic acid with arsenic trioxide in the chemotherapy is efficient treatment on childhood APL and can decraese relapse.

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Influence of human cytomegalovirus infection on the expression of HOXB6 gene in human cord blood erythroid progenitor cell. RAN Ling,LIU Wen-jun,CHEN Ai 2007, 22(06): 452-454 .  Abstract ( )   AbstractObjectiveTo investigate the expression of HOXB6 gene in human cord blood colony forming unit erythroid (CFU-E),and the influence of human cytomegalovirus (HCMV)infection on the expression of the gene in order to explore the possible mechanism of HCMV-induced malformations. MethodsThe expression of HOXB6 gene in erythroid progenitor cells infected with HCMV and/or treated with ATRA were determined by fluorogenic quantitative reserve transcription polymerase chain reaction(FQ-RT-PCR)method. ResultsErythroid progenitor continually expressed HOXB6 gene.The expression of HOXB6 gene was reduced after HCMV infection.Treated with all-trans retinic acid (ATRA),the expression of HOXB6 gene in erythroid progenitor cells was increased.The expression of HOXB6 gene infected with HCMV and treates with ATRA was reduced in given time. ConclusionHCMV infection significantly down-regulated the expression of HOXB6 gene.The result suggests that the abnormal expression of HOXB6 gene induced by HCMV may play an important role in HCMV-induced abnormal hematogenic damage.

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Curative effect analysis of high dose cytosine arabinoside in the chemotherapy for childhood acute lymphoblastic leukemia. ZUO Ying-xi,LIU Gui-lan,LU Ai-dong 2007, 22(06): 455-458 .  Abstract ( )   AbstractObjectiveTo evaluate the curative effect of a short-peroid protocol on acute lymphoblastic leukemia,which involved high dose cytosine arabinoside (Ara-C) regimen. MethodsThe research included 84 diagnostic patients in our department from January 1992 to July 2001:52 boys and 32 girls.Data collection lasted until July 2006. ResultsAbout 79.59% of the standard-risk patients achieved event-free survival,while only 25.81% of the high-risk patients achieved event-free survival.The median event-free survival of patients was 9 years and 11 months (ranging from 5 years to 14 years and 6 months).Central nervous system leukemia happened in 6 patients.No death was related to high dose Ara-C chemotherapy. Conclusion1.The short period protocol,involving high dose cytosine arabinoside regimen,increases the complete remission rate and event-free survival rate,and has few adverse effects.2.Cranial irradiation isn't performed in this protocol,and no more patients suffer from central nervous system relapse.3.The sensitivity of cytosine arabinoside is eminently different from person to person.

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Expression of Daxx in pediatric acute leukemia and its relationship with expression of NF-κB. LIU Jing,ZHANG Liu-qing,LIN Han-hua 2007, 22(06): 459-461 .  Abstract ( )   AbstractObjectiveTo investigate the expression of Daxx in pediatric acute leukemia(AL) and its relationship with expression of NF-κB and clinical significance. MethodsThe expression of Daxx and NF-κB proteins were detected by immunohistochemical assay in 50 AL children.The control included 20 children with normal bone marrow. ResultsDaxx protein expression rate was 38.0% and NF-κB protein expression rate was 56.0% in 50 AL children.Both of them were significantly higher than that in control group(P<0.05).The expression of Daxx was positively correlated with NF-κB (P<0.05).The expression rates of Daxx and NF-κB were 55.6% and 77.8% respectively in HR ALL,0.0% and 16.7% respectively in SR ALL,and the differences were statistically significant (P<0.05). ConclusionThe over-expression of Daxx and NF-κB may promote tumor genesis and progression in AL.Daxx and NF-κB are also related to some clinical features,treatment outcome and prognostic factors of AL.