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06 October 2006, Volume 21 Issue 10 Previous Issue    Next Issue
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Retreatment and risk factors of IVIG nonresponsiveness. Du Zhongdong,Zhang Yonglan,Zhao Di 2006, 21(10): 738-741 .  Abstract ( )   AbstractObjectiveTo evaluate the incidence and risk factors of children with refractory Kawasaki disease (KD). MethodsClinical data of all children with KD in 45 hospitals in Beijing were summarized from 2000 through 2004.Refractory KD was defined as those who remained febrile with a temperature of >38.5℃ 36 hours after initial intravenous immunoglobulin treatment (IVIG). ResultsA total of 1107 patients were included as study subjects.Of them,1092 patients had data on acute treatment,1052 (96.3%) received initial IVIG therapy,135 did not respond to the initial IVIG treatment with an incidence of 12.8%.Logistic regression revealed that ESR,GPT,WBC,serum albumin,time from onset to IVIG treatment and IVIG dosage were independent risk factors for refractory KD (P<0.05).Children with refractory KD were retreated with a second IVIG of 2g/kg in 8 patients with 5 responding (62.5%),1 g/kg in 114 with 35 responding (30.7%),and 400~600 mg/kg in 11 with 1 responding (9.1%).Steriod was used in 2 children with both responding (P=0.015). ConclusionThe incidence of refractory KD in Beijing was 12.8%.ESR,GPT, WBC,serum albumin,the time from onset to IVIG treatment and IVIG dosage are risk factors for refractory KD.Retreatment of children with refractory KD with a 2g/kg IVIG is probably more effective for fever defervescence than a dosage of 1 g/kg or 400~600 mg/kg.

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Risk factors of coronary lesions in children with Kawasaki disease. Zhang Yonglan,Du Zhongdong. 2006, 21(10): 742-744 .  Abstract ( )   AbstractObjectiveTo summarize the clinical pictures and laboratory study results of children with Kawasaki disease,and further analyze the risk factors of coronary complications in Kawasaki disease. MethodsAll charts of consecutive children with a diagnosis or suspicious diagnosis of Kawasaki disease from January 1,2000 to December 31,2004 were reviewed retrospectively.Children with clinical pictures satisfied to the 5th revision of diagnostic criteria of Kawasaki disease were included.Patients gender,age,duration of fever,use of intravenous gamma immunoglobublin (IVIG),corticosteriod use,laboratory study results,and echocardiographic study results were summarized.Chisquare test and logistic regression were performed to analyze the risk factors of coronary abnormalities. ResultsA total of 644 patients with Kawasaki disease were enrolled as the subjects of this study.Their age ranged from 1 month to 13.3 years of age,Laboratory study showed most of patients had elevation of ESR,Creactive protein,WBC count,platelet count,while some of them had hypoalbuminemia and anemia.Out of all patients,644 had coronary dilation with an incidence of 36.3%.Coronary aneurysm was identified in 16 patients with an incidence of 2.5%.Statistical analysis showed that gender,duration between onset and IVIG treatment,dosage of IVIG,duration of fever,ESR and CRP were related to coronary abnormalities(P<0.05).Multiple logistic regression identified that gender,duration of fever and dosage of IVIG were independent factors contributing to the development of coronary abnormalities. ConclusionBeing male,longer duration of fever,duration between onset and IVIG treatment and IVIG dosage,ESR and CRP are independent risk factors contributing to the development of coronary abnormalities.

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A metabolic imbalance of hydrogen sulfide and homocysteine in essential hypertensive children. Ding Yaguang,Du Junbao,Mi Jie 2006, 21(10): 745-747 .  Abstract ( )   AbstractObjectiveTo probe into the metabolic imbalance status of hydrogen sulfide (H2S) and homocysteine (Hcy) in children with essential hypertension,and its significance in the pathogenesis of essential hypertension. MethodsTwentyfive children with essential hypertension and 30 healthy children with normal blood pressure were included in the study.Medical history was investigated and physical examination was conducted for the subjects.Plasma H2S level was detected by modified method with sulfide electrode.Plasma Hcy content was examined by FPIA. ResultsPlasma Hcy levels were (12.68±9.69)μmol/L in hypertensive cases and (6.62±4.79)μmol/L in normal subjects (P<0.01).Plasma H2S levels were (51.93±6.01)μmol/L in hypertensive cases and (65.70±5.50)μmol/L in normal subjects (P<0.01).A negative correlation existed between plasma Hcy and H2S concentrations (r=-0.379,P<0.05).And a negative correlation existed between systolic pressure and plasma H2S /Hcy ratio (r=-0.687,P<0.05). ConclusionThere is a metabolic imbalance of hydrogen sulfide and homocysteine in essential hypertensive children.

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Clinical analysis of 39 children with constrictive pericarditis. Zhang Jing,Tian Jie,Qian Yongru. 2006, 21(10): 748-750 .  Abstract ( )   AbstractObjectiveTo study the clinical characteristics of children with constrictive pericarditis(CP). MethodsThe diagnosis and treatment effect of 39 children with CP were reviewed retrospectively. ResultsThe clinical manifestations were diverse.The main symptoms included fatigue(92.31%),edema(89.74%) and dyspnea(82.05%).The main signs included hepatic enlargement(89.74%),neck vein distention(79.49%),and low or distant heart sounds(79.49%).The positive rate of Xray was 61.54% and echocardiography was 76.32%.Causes of CP were paragonimus for 7,tuberculosis for 5,bacterium for 3 and idiopathic for 24. ConclusionThe diagnosis of constrictive pericarditis remains a challenge because of its physical findings and hemodynamics mimic restrictive cardiomyopathy.Echocardiography is considered as the most valuable testing for the diagnosis of constrictive pericarditis up to date.It is important to take the pericardial resection as early as possible after CP diagnosis.

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Clinical analysis of 21 cases of interrupted aortic arch. Liu Fang,Huang Guoying,Sheng Feng 2006, 21(10): 751-752 .  Abstract ( )   AbstractObjectiveInterruption of the aortic arch (IAA) is defined as absence of luminal continuity between the ascending and the descending aorta.It is a rare but severe malformation,usually with a deteriorative congestive heart failure and high mortality.We analysed the clinical informations of IAA in order to make improvement in the diagnosis and management. MethodsThe records of 21 IAA patients,aged 12 days to 11 years (mean age 1.5 months) were reviewed. ResultsThere were ten male and 11 female,accounting for 0.1% of the patients with congenital heart disease of the same period.The anatomic subtype included type A 18 (85.7%),type B 3 (14.3%),and no type C in the records.All cases were associated with large PDA and VSD,some also with ASD (II),bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was made in 10 of the patients and suspected in 8 by echocardiography.However,echocardiography failed to make diagnosis in 3 (14.3%).Surgery repair was performed in 9 of the patients.Two older patients were successfully operated in two stages.Seven patients underwent total correction,of which 4 were successful,3 died of ventricular fibrillation,shock due to allergy to protamine sulpate and severe pneumonia during perioperative stage. ConclusionEchocardiography is the primary important method to diagnose IAA,but angiography is necessary for determining diagnosis in some cases.Mortality is relatively higher for totally correction,and the complications during perioperative stage are the important causes of death.

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Analysis of influencing factors for capillary leak syndrome in children after cardiopulmonary bypass. Liu Chengjun,Liu Lu,Xu Feng 2006, 21(10): 753-755 .  Abstract ( )   AbstractObjectiveTo determine influencing factors of capillary leak syndrome after cardiopulmonary bypass in children with congenital heart disease and offer reference for the prevention and control of CLS. MethodsClinical data were collected from children who underwent cardiopulmonary bypass in our hospita1 between January 2004 and November 2005.Several statistically significant influencing factors were screened out with univarite analysis，independent influencing factors of CLS were determined with multivarite stepwise logistic regression analysis. ResultsThe morbidity and mortality rate of CLS was 16.78% and 19.23% respectively.Logistic analysis showed the influencing factors of CLS were duration of cardiopulmonary bypass,type of congenital heart disease,lowest temperature during CBP and age. ConclusionThe influencing factors of CLS in children after cardiopulmonary bypass are duration of cardiopulmonary bypass,type of congenital heart disease,lowest temperature during CBP and age.

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The pathological character of renal biopsy and clinical study of infants. Dang Xiqiang,Yi Zhuwen,He Xiaojie 2006, 21(10): 756-758 .  Abstract ( )   AbstractObjectiveTo understand the relationship between pathological features and clinical manifestations in children under 3 years suffering from renal glomerular disease in Hunan Province. MethodsRenal biopsy by rapid percutaneous punctuation was performed on 73 children under the age of 6 years who were all diagnosed clinically as kidney diseases of 10 different kinds totally.The specimens were divided into 3 parts for microscope,electronmicroscope and immunofluorescence examination separately and processed by HE,PAS,PASM and Masson staining.Immunofluorescence was used to detect the deposition of IgG,IgM,IgA,C3,C4,C1q and fibrin in renal tissue.Additional examinations were done to detect HBsAg and HBcAg deposition in some cases with positive serum HBsAg. ResultsAll the renal biopsy performances were successful.The most common pathological type (51/73,69.86%) was mesangial proliferation glomerulonephritis (MsPGN).Diseases with similar clinical features might present different pathological types,and the similar pathological type might be seen in diseases with different clinical manifestations. ConclusionRenal biopsy is of great value for the diagnosis,treatments and estimation of prognosis for kidney disease in infants.Electron microscope examination plays an important role in pathological diagnosis,too.

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Inhibition of the gene expression of respirovirus in steroid responsive simple nephrotic syndrome by antisense oligonucleotides. Liu Xiaoming,Wang Zheng,Zhu Xiaoshi. 2006, 21(10): 759-761 .  Abstract ( )   AbstractObjectiveTo study the inhibition of antisense oligonucleotides (ASONs) on the gene expression of respiroviruses in peripheral blood mononuclear cells(PBMCs)of children with steroid responsive simple nephrotic syndrome (SRSNS),aiming to provide more proof for the infection of respiroviruses in SRSNS. MethodsAfter transfecting ASONs to PBMCs of children with SRSNS,the gene expression of respiroviruses was detected by RTPCR. ResultsAfter ASONs transfection,the gene expression of respiroviruses was inhibited significantly in SRSNS(active stage). ConclusionThe study demonstrates that respiroviruses infection actually exists in SRSNS(active stage),and the gene expression of the respiroviruses is inhibited by ASONs,thus suggesting that respiroviruses infection may be one of the triggers of SRSNS.

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Levels and clinical significance of CD62P and CD44 in blood of children with primary nephritic syndrome. Zhang Qiaoling1，Jiang Xiaoyun2，Lai Feng 2006, 21(10): 762-764 .  Abstract ( )   AbstractObjectiveTo probe the levels and clinical significance of CD62P and CD44 in children with primary nephritic syndrome (PNS). MethodsTotally 54 children were divided into four groups,steroidsensitive NS at active stage(19 patients),and at remission stage(13 patients),steroidresistance at active stage (12 patients) and at remission stage(10 patients).The levels of plasma CD62P and serum CD44 were assayed by radioimmunoassay (RIA) and enzyme linked immunoabsorbant assay (ELISA) in each group and in 20 normal age and sexmatched controls,and its correlation with 24hour proteinuria,serum albumin,cholesterol,urea nitrogen and creatinine were also analyzed. Results(1) In the steroidsensitive patients,the levels of CD62P and CD44 in patients at active stage were much higher than those of in patients at remission stage (P<0.01).(2) In the steroidresistant patients,the levels of CD44 in patients at active stage were higher than that in patients at remission stage (P<0.05),but there were no statistical significant differences between the level of serum CD62P in patients at active stage and that in patients at remission stage (P>0.05).(3) The levels of CD62P and CD44 in PNS children at active stage were positively correlated with that of 24hour proteinuria and serum cholesterol,and negatively correlated with serum albumin. ConclusionThe levels of serum CD62P and CD44 are related to the development and progression of children with PNS,and could be used to estimate the state and the remedial effect of children with PNS.

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The outcome of syngeneic peripheral stem cell transplantation in four pediatric patients with leukemia and aplastic anemia. Zhou Xuan,Wu Minyuan,Zhu Guanghua. 2006, 21(10): 765-767 .  Abstract ( )   AbstractObjectiveTo investigate the outcome,engraftment and prognosis of syngeneic peripheral stem cell transplantation in pediatric leukemia and aplastic anemia. MethodsFour cases who suffered from chronic myeloid leukemia (CP),acute myeloid leukemia (M2),severe aplastic anemia,and acute lymphoblastic leukemia(Ph+) were analyzed,respectively.Three leukemia patients achieved the first complete remission.after the regular chemotherapy.Conditioning regimens were all different.All donors who received rhGCSF mobilization were identicaltwins. ResultsThe average amount of MNC is 10.08×108/kg.The engraftment of WBC and PLT were +10.7d and +12.3d,respectively.No severe toxicity and infection were observed.Case 4 relapsed 5 months post transplantation,and died due to no further therapy.Other cases were well.Follow up time was more than 16 months. ConclusionSyngeneic peripheral stem cell transplantation is the effective method to treat some kinds of blood disorders,including leukemia.However,the future effect needs to be evaluated.

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Application of bronchodilation test by tidal breathing in 0~6year old children with airway hyperresponsive diseases. Wang Junping,Zhang Hao,Wang Libo 2006, 21(10): 768-771 .  Abstract ( )   AbstractObjectiveTo evaluate the bronchodilation test in tidal breathing of young children with airway hyperresponsive disease,and to establish the diagnostic standard of tidal breathing in young children's bronchodilation test. MethodsSixtyone children who saw doctor (because of coughing) at the Children s Hospital of Fudan University,aged 4~72 months.They were classified into two groups：one group was coughing with wheeze(n=36),one group was just coughing(n=25).Each group received salbutamol and ipratropium bromide.Tidal breathing flowvolume loop and parameters were obtained before and after aerosol inhalation to evaluate the effects of drugs.We evaluated the change of two groups of tidal breathing flowvolume loops and parameters. Results(1) After inhaling the drugs,there were significant recovery (increase) in tPTEF/tE and VPEF/VE in wheezying bronchitis group.(2)There were significant differences in the change of tPTEF/tE and VPEF/VE between two groups.(3)If we used tPTEF/tE rises≥15% or VPEF/VE rises≥15% as the diagnostic standard,the best combination of sensitivity and specificity was achieved.The sensitivity was 72.2%;the specificity was 80%.The positive rate of wheezy group was 72.2%. ConclusionWe suggest that tPTEF/tE rise≥15% or VPEF/VE rise≥15% to be the diagnostic standard in younger children’s bronchodilation test by tidal breathing.

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Screening study on the melanocortin 4 receptor gene are in Chinese obese Han Children. Wang Chunlin,Shen Zheng,Wang Haijun 2006, 21(10): 772-774 .  Abstract ( )   AbstractObjectiveTo search for mutations in the MC4R in obese and normal weight Chinese children. MethodsScreen the coding region of the MC4R gene by PCR and resequencing.The clinical and biochemical data of 200 obese children were analysed. ResultsIn the obese children we detected two heterozygotes for novel nonsynonymous mutations (c.496G>A resulting in Val166Ile;c.929G>A resulting in Arg310Lys) and a heterozygote for a novel nonsense mutation (c.831T>A resulting in a premature stop codon Cys277Stop) in the MC4R gene in three of the obese individuals.In both,the obese individuals and the normal weight controls heterozygotes for a novel nonsynonymous mutation (c.68T>G resulting in Leu23Arg,0.5% and 1%,respectively) and the Val103Ile (c.307G>A,3%and 2%,respectively).We analysed ALT、AST、TG、CHO and WBISI in mutation and nonmutation obese group and found there were no difference.Heterozygotes for the Val103Ile polymorphism were detected in similar frequencies in obese and normal weight controls.The prevalence for heterozygous MC4R mutations was 1.5% in the obese. ConclusionTwo novel heterozygous nonsynonymous mutations (Val166Ile;Arg310Lys) and a novel heterozygous nonsense mutation (Cys277Stop) are detected in Chinese obese individuals.Leu23Arg variant might be a polymorphism in the Chinese population.