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1.
Chinese Journal of Applied Clinical Pediatrics ; (24): 1506-1509, 2021.
Article in Chinese | WPRIM | ID: wpr-908000

ABSTRACT

Fulminant myocarditis (FM) in children is an acute inflammatory myocardial disease, prominently manifesting as hemodynamic disorder.Because of acute onset and rapid progression, FM can cause myocardial cell edema, necrosis, cardiac shock and sudden death.Early symptoms of FM are usually atypical and laboratory tests are nonspecific, thus leading to misdiagnosis.If in time identification and diagnosis of FM is failed, its premature mortality rate can be significantly risen.Electrocardiogram, echocardiogram and cardiac magnetic resonance examinations are of great importance for early detection of FM and prognosis evaluation.Currently, mechanical circulation support is the effective therapeutic strategy for FM.A growing number of studies have focused on the clinical diagnosis and treatment of FM in children, especially the early diagnosis and active interventions, which is of great significance to improve the survival and quality of life in the future.In this paper, the advance on current diagnosis and treatment of FM in children were reviewed.

2.
Chinese Pediatric Emergency Medicine ; (12): 1103-1106, 2021.
Article in Chinese | WPRIM | ID: wpr-930792

ABSTRACT

Kawasaki disease(KD) is an acute systemic medium and small vasculitis which commonly occurred in children under 5 years old.Coronary artery lesion(CAL) is the most important complication of KD.So KD is the leading cause of acquired heart disease in children.Due to the lack of gold standard for early diagnosis, accurate diagnosis of KD and early prediction of critical KD are the major clinical challenges.Early accurate diagnosis of KD, prediction of sensibility for intravenous immunoglobulin and prediction of CAL are of great application value in clinical diagnosis, treatment and reduction of CAL.So, we reviewed the progress of diagnostic and prediction model of KD.

3.
Chinese Pediatric Emergency Medicine ; (12): 654-656, 2020.
Article in Chinese | WPRIM | ID: wpr-864977

ABSTRACT

Kawasaki disease(KD) with macrophage activation syndrome(MAS) is a rare and serious complication.The clinical symptoms of KD with MAS are critical, and if not early diagnosed, the prognosis is often very worse.At present, there is no unified guideline for diagnosis standard and treatment scheme of KD with MAS.So, we performed the review to realize the diagnostic criteria, epidemiology, treatment progress and prognosis of KD with MAS.It is helpful to provide evidence for clinical diagnosis and treatment of such severe KD.

4.
Journal of Clinical Pediatrics ; (12): 453-455, 2018.
Article in Chinese | WPRIM | ID: wpr-694703

ABSTRACT

Objective To explore the value of transesophageal atrial pacing (TEAP) and temporary cardiac pacing in the treatment of acute intractable tachyarrhythmia in children. Method The clinical data of children with acute intractable tachyarrhythmia treated with TEAP (48 cases) or temporary cardiac pacing (6 cases) from January 2014 to December 2017 were retrospectively analyzed. Results In the 48 cases aged 1 day to 13 years (28 males and 20 females) treated with TEAP, 25 cases had paroxysmal supraventricular tachycardia, in which there were 15 cases of rapid atrial flutter, 5 cases of atrial tachycardia and 3 cases of left ventricular idiopathic bundle branch reentrant tachycardia. The rates of successful cardioversion to sinus rhythm through TEAP were 84%, 80%, 20% and 100% respectively. In the 6 cases aged 2 to 10 years (1 male and 5 females) treated with temporary cardiac pacing, there were 5 cases of fulminant myocarditis and 1 case of noncompaction of the ventricular myocardium and their conditions were well controlled after treatment. Conclusion TEAP and temporary cardiac pacing have good curative effect in the treatment of acute intractable tachyarrhythmia in children. However, temporary electricity therapy can only temporarily rectify the hemodynamic abnormality threatening the life of children, and active comprehensive treatment for the primary diseases should be carried out simultaneously.

5.
Journal of Clinical Pediatrics ; (12): 367-371, 2018.
Article in Chinese | WPRIM | ID: wpr-694685

ABSTRACT

Objective To investigate the acute phase expression of serum cytokines in children with Kawasaki disease (KD) and its association with coronary artery lesions (CALs). Methods Expression of 13 cytokines in serum of 104 KD patients including 11 cases with CALs and 74 febrile control cases admitted to the hospital from October 2016 to March 2017 were retrospectively analyzed. Results Interleukin (IL)-8, IL-1, IL-2, IL-6, IL-10, IL-17A, IL-18, tumor necrotic factor-α and SCD25 (IL-2R) were declined significantly after intravenous immunoglobulin administration (IVIG) in KD patients (all P<0.05). Compared with the fever control group, Pre-IVIG, an IL-17A level ≥ 0.155 pg/mL had a sensitivity of 50% and 93.2% specificity for predicting KD; IL-18 level ≤ 15.43 pg/mL had a sensitivity of 71.2%, and the specificity was 54.1%; SCD25 (IL-2R) ≥ 29475.29 pg/mL had a 65.4% sensitivity and 81.1% specificity for predicting KD. Before IVIG treatment, the level of IL-10 was significantly lower in KD patients with CALs than in those without CALs. Conclusions Determination of various cytokines profile may be helpful for predicting the disease prognosis and targeting treatment strategies in patients with KD.

6.
Journal of Clinical Pediatrics ; (12): 364-366, 2018.
Article in Chinese | WPRIM | ID: wpr-694684

ABSTRACT

Objective To explore clinical characteristics of J wave syndromes. Methods Clinical data was retrospectively analyzed in a child with fainting and showing big J wave in ECG. Meanwhile, related literatures were reviewed. Results A 10-year-old boy was admitted to hospital with fainting. ECG showed J point up, reflecting Osbron wave. There was no abnormality in EEG and cranial CT. Ultrasound cardiograph showed approximately normal heart structure and left ventricular systolic function. Fainting was frequently occurred during follow-up and accompanied by malignant arrhythmia. Conclusions Patients with J wave syndromes has high risks of malignant arrhythmia and sudden cardiac death.

7.
International Journal of Pediatrics ; (6): 532-536,542, 2018.
Article in Chinese | WPRIM | ID: wpr-692541

ABSTRACT

Objective To analyze the possible risk factors of IVIG non-response of Kawasaki disease (KD),Shanghai Children's Hospital and Shanghai Junze Software develop an research platform,which is based on E-Science model.Through the mathematical model by integrating the risk factors to explore the method of effective prediction for IVIG non-response,and to provide the clinical basis for timely and effective treatment and prognosis of the disease.Methods The data of KD children who were hospitalized in Shanghai Children's Hospital from January 2013 to November 2016 were included.The indexes included gender,age,time of IVIG treatment,and laboratory examinations.The multivariate logistic regression was used to analyze the influencing factors of IVIG non-response.The indexes in the model were deduced according to the independent variables of the logistic regression equation.The ROC curve and the area under the curve were calculated for the new prediction model.The sensitivity and specificity of the new prediction model were calculated according to the cutoff value.Finally,the new model was compared with the Kobayashi and Egami scoring model.Results The levels of CRP,NLR,LDH,ALB and FDP in children with KD were influencing factors for IVIG nonresponse (P < 0.05).According to the logistic regression equation,the sensitivity and specificity of the model used to predict IVIG non-response were 69.7% and 80.4%,respectively,and the area under the ROC curve was 0.825 (95% CI:0.769-0.882).Kobayashi and Egami scoring models were tested,the sensitivity and specificity of the new scoring system were better than previous ones.Condusion The scoring model established in this study has a good effect in predicting IVIG non-response in KD patients and could be used in clinical practice,and it is worthy to be validated and adjusted by large-scale data.

8.
Chinese Journal of Pediatrics ; (12): 529-533, 2017.
Article in Chinese | WPRIM | ID: wpr-808949

ABSTRACT

Objective@#To screen and identify the mutations in Kawasaki disease by targeted enrichment of genomic region sequencing technique and investigate susceptibility genes associated with coronary artery lesion.@*Method@#This was a case-control study.A total of 114 patients diagnosed as Kawasaki disease treated in Shanghai Children′s Hospital between December 2015 and November 2016 were studied and another 45 healthy children who were physically examined in outpatient department were enrolled as control group. Patients were divided into two groups based on the results of echocardiogram. Peripheral venous blood was obtained from patients and controls. Genomic DNA was extracted. SeqCap EZ Choice libraries were prepared by targeted enrichment of genomic region technology. Then the libraries were sequenced to identify susceptibility genes associated with coronary artery lesion in patients diagnosed as Kawasaki disease.Susceptible genes were identified by Burden test, Pearson chi-square test or Fisher′s exact probability test.@*Result@#There was statistically significant difference in TNFRSF11B(rs2073618)G>C(p.N3K)mutation and GG/GC/CC genotype between Kawasaki disease group and control group(χ2=15.52, P=0.00). There was statistically significant difference in TNFRSF13B(rs34562254)C>T(p.P251L)mutation(χ2=10.40, P=0.01)and LEFTY1(rs360057)T>G(p.D322A)mutation(χ2=8.505, P=0.01)between patients with coronary artery lesions and those without.@*Conclusion@#Targeted enrichment of genomic region sequencing technology can be used to do primary screening for the susceptible genes associated with coronary artery lesions in Chinese Kawasaki patients and may provide theoretical basis for larger sample investigation of risk prediction score standard in Kawasaki disease.

9.
Chinese Journal of Applied Clinical Pediatrics ; (24): 63-66, 2017.
Article in Chinese | WPRIM | ID: wpr-505119

ABSTRACT

Objective To investigate the effects of T-type calcium channel inhibitors (ProTx-1,micromolar Ni2+ and Mibefradil) on Monocrotaline (MCT)-induced pulmonary arterial hypertension (PAH) in rats.Methods Forty male Sprague-Dawley rats were randomly divided into 5 groups:normal control group,MCT group,ProTx-1 group,micromolar Ni2+ group and Mibefradil group (8 cases in each group).The right ventricular systolic pressure (RVSP),the right ventricular hypertrophy index (RVHI),and the index of pulmonary vascular remodeling(MA%) were measured on day 28 after MCT-treatment.Western blot was used to detect the expression of proliferating cell nuclear antigen(PCNA) and Cleaved Caspase-3 in pulmonary artery.Results (1)RVSP and RVHI in MCT group were significandy higher than those in the other 4 groups (F =21.55,P < 0.01;F =15.63,P < 0.01).The two indexes in 3 intervention groups were higher than those in normal control group (all P < 0.05),nevertheless,significantly lower than those in MCT group,and 3 intervention groups showed no significant differences (all P > 0.05).(2) MA% in normal control group [(23.43 ± 1.95) %] was lower than that in MCT group [(80.42 ± 4.30) %],ProTx-1 group [(60.35 ± 3.83)%],micromolar Ni2+ group[(62.44 ± 3.81)%] and Mibefradil group[(58.66 ± 4.23)%] (F =216.2,P < 0.01);3 intervention groups showed no significant differences (all P > 0.05),however,they were all significantly lower than that in MCT group.(3) The expression of PCNA in MCT group was higher than that in normal control group,meanwhile,3 intervention groups were significantly lower than that in MCT group.The expression of Cleaved Caspase-3 in MCT group was higher than that in normal control group,nevertheless,3 intervention groups showed no significant changes compared with MCT group,respectively.Conclusions T-type calcium channel inhibitors could ameliorate the progression of MCT-PAH in rats,mainly through suppressing the proliferation of pulmonary artery smooth muscle cells.

10.
Journal of Clinical Pediatrics ; (12): 488-490, 2017.
Article in Chinese | WPRIM | ID: wpr-613683

ABSTRACT

Objective To explore the clinical characteristics and treatment of paroxysmal supraventricular tachycardia (PSVT) in children. Methods The clinical data of 67 children with PSVT were analyzed retrospectively, and the therapeutic effects of different treatments were compared. Results The clinical manifestations of infants were paleness, shortness of breath, irritability and sweating, and children showed chest tightness, palpitations, abdominal discomfort and fatigue. The curative effect of electric cardioversion, transesophageal atrial pacing, physical therapy, and drug therapy was statistically different (P<0.05), The different cardioversion rates of them were observed for the treatment of paroxysmal supraventricular tachycardia. The cardioversion rate of transesophageal atrial pacing, was the highest, and the rate of physical therapy was the lowest. There was no significant difference in the cardioversion rate between propafenone, digoxin and amiodarone. Conclusion The clinical manifestations of PSVT in infants are atypical and easily to be ignored. There are many methods for treatment of PSVT. The vagus nerve can be stimulated first, and, if no response, either drugs or electric cardioversion and transesophageal atrial pacing can be used. The cardioversion rate of transesophageal atrial pacing is higher. The drug effectiveness for the treatment of PSVT depends on many factors, and our choice of medication varies from person to person.

11.
Journal of Clinical Pediatrics ; (12): 494-498, 2016.
Article in Chinese | WPRIM | ID: wpr-496440

ABSTRACT

Objectives To explore the clinical features and pathogenesis of Kawasaki disease (KD) combined with sterile pyuria. Methods A total of 420 patients diagnosed of KD were recruited and divided into pyuria group ( 95 patients) and control group ( 325 patients) according to urine routine examination on admission. The clinical data between the two groups were compared. Results There was no difference in gender, age, and the incidence of atypical KD (P all?>?0 . 05 ). The levels of C-reactive protein, D-dimer concentrations, fibrinogen degradation products, alanine aminotransferase, aspartate aminotransferase, and urine retinol binding protein were higher in pyuria group than those in control group (all P>?0 . 05 ). No difference was found in the duration of fever before admission between two groups (P>?0 . 05 ). However, pyuria group had longer duration of fever after treatment with immunoglobulin (P?0.05). Conclusion The morbidity of sterile pyuria in KD patients was 22 . 6%. KD patients with sterile pyuria have more intense inlfammatory response, markedly high coagulation condition, and mild or subclinical renal damage.

12.
Journal of Clinical Pediatrics ; (12): 465-469, 2016.
Article in Chinese | WPRIM | ID: wpr-492843

ABSTRACT

Objectives To explore the effect of mibefradil, a kind of novel calcium channel antagonists, on proliferation of human pulmonary artery smooth muscle cells (HPASMCs) induced by platelet-derived growth factor (PDGF). MethodsHPASMCs were culturedin vitro, and randomly divided into control group, PDGF group, Mib group, and PDGF+Mib group. The PDGF group was stimulated by 25 ng/ml of PDGF. Mib group was intervented by 10 μmol/L of Mib. PDGF+Mib group was treated by PDGF and Mib. The reproduction rate in 48 hours and 72 hours were detected by MMT. Cell cycle was detected by lfow cytometry. The expression of proliferating cell nuclear antigen (PCNA) was observed by immunolfuorescence staining (IFS).ResultsThere were statistical differences among four groups in both 48 hours and 72 hours (P all??0.05). There were statistically differences of G0/G1 phase and S phase among four groups (P?

13.
Chinese Journal of Pediatrics ; (12): 34-39, 2015.
Article in Chinese | WPRIM | ID: wpr-293878

ABSTRACT

<p><b>OBJECTIVE</b>To explore the value of electronic data capture (EDC) system in large-sample size studies on Kawasaki disease (KD).</p><p><b>METHOD</b>The clinical data of 602 KD cases from 2007 to 2012 admitted to Shanghai Children's Hospital with EDC system connected with hospital information system (HIS) were retrospectively analyzed. Age, gender, acute symptoms, laboratory results, echocardiography, therapy were collected. The differences in parameters were compared between KD with and without coronary artery lesion (CAL). Furthermore, the difference between intravenous immunoglobulin (IVIG) resistant group and sensitive group were compared. Multi-factor logistic regression analyses were used to analyze the risk factors. The sensitivity and specificity of IVIG resistance parameters were detected with receiver operating characteristic curve (ROC) analysis.</p><p><b>RESULT</b>The male to female ratio of KD cases was 1.85: 1. The median age of KD cases was 2.0 years (one month to 11.7 years old); 20.1% cases (121/602) exhibited CAL. Compared with KD without CAL (n = 481), the incidence of bright red cracked lips (71.1% vs. 88.6%, P = 0.001), peeling of the skin of the toes (28.1% vs. 41.6%, P = 0.021) and perianal skin peeling (29.8% vs. 38.9%, P = 0.031) were statistically lower in KD with CAL (n = 121). The incidence of CAL in KD IVIG resistant group was significantly higher than KD IVIG sensitive group (34.6% (9/26) vs.21.3% (112/525), P = 0.05 ). Male ratio (80.8% vs. 63.4%, P = 0.05), time of IVIG ( (6 ± 2) vs. (8 ± 5) d, P = 0.009), erythrocyte sedimentation rate(ESR) ( (81 ± 2) vs. (66 ± 30) mm/1 h, P = 0.014), C-reactive protein ((107 ± 51) vs. (87 ± 52) mg/L, P = 0.017), blood platelet ( (599 ± 178) vs. (489 ± 182) ×10(9)/L, P = 0.003), hemoglobin ( (96 ± 13) vs. (102 ± 19) g/L, P = 0.032) and albumin ((34 ± 6) vs. (37 ± 6) g/L, P = 0.020) were significantly different between IVIG resistant group and sensitive group. Logistic regression analysis showed that alanine aminotransferase (ALT) ≥ 80 U/L was the independent risk factor of IVIG resistance (P = 0.012). C-reactive protein = 104 mg/L (sensitivity 61.5%, specificity 62.7%), ESR = 106 mm/1 h (sensitivity 26.9%, specificity 93.6%) and blood platelet = 187×10(9)/L (sensitivity 76.9%, specificity 53.1%) of KD in acute phase were predictive for IVIG resistance with receiver operate characteristic curve analysis.</p><p><b>CONCLUSION</b>EDC system can acquire KD clinical data quickly and accurately. It is helpful for multicenter retrospective analysis of KD.</p>


Subject(s)
Child , Child, Preschool , Female , Humans , Male , Blood Platelets , Blood Sedimentation , C-Reactive Protein , China , Coronary Artery Disease , Echocardiography , Electronic Health Records , Hemoglobins , Immunoglobulins, Intravenous , Mucocutaneous Lymph Node Syndrome , Retrospective Studies , Risk Factors
14.
Chinese Journal of Applied Clinical Pediatrics ; (24): 30-33, 2015.
Article in Chinese | WPRIM | ID: wpr-466778

ABSTRACT

Objective To improve the knowledge of paediatric pulmonary arterial hypertension(PAH) and to elevate the level of early diagnosis and treatment.Methods The clinical data of 45 PAH patients admitted in Shanghai Children's Hospital from Jan.2006 to Dec.2012 were reviewed,including clinical manifestation,laboratory examination,diagnosis,treatment and prognosis.Results Of the 45 PAH patients,21 cases (46.7%) were male and 24 cases (53.3 %) were female,with an average age of 2.5 years old.Among them idiopathic PAH was in 24 cases (53.3 %) and secondary PAH was in 21 cases [including interstitial lung disease in 4 cases,upper airway obstruction in 3 cases,systemic lupus erythematosus in 3 cases,hepatic disease in 3 cases,including hepatic hemangioma 1 case,liver cirrhosis portal hypertension 1 case and autoimmune hepatic sarcoidosis 1 case,tachycardia induced cardiomyopathy in 2 cases,extensive pulmonary small artery stenosis in 2 cases,human immunodeficiency virus (HIV) infection in 1 case,hypothyroidism in 1 case,and familial PAH in 2 cases].Main clinical manifestations were anhelation after exercise (71.1%),fatigue (68.9%),cough (48.9%),chest tightness (26.7%),chest pain (33.3%),syncope (8.9%),et al.The most common physical signs were splitting of the second sound in pulmonary valve area (93.3%),followed by tricuspid murmur (77.8%),cyanosis (53.3%),hepatomegaly (42.2%),hydropericardium (28.9%),and oedema (11.1%),et al.Twenty-seven cases did cardiac catheterization,compared with idiopathic PAH and secondary PAH,pulmonary artery systolic pressure,mean pulmonary artery pressure,pulmonary capillary wedge pressure,pulmonary arteriolar resistance index had significant difference(P < 0.05).Thirty-one cases' condition improved after treatment,11 cases without any improvement and 3 cases died during follow-up visit.Conclusions PAH is a rare disease with no specific symptom and can be easily misdiagnosed in children.Ultrasonic cardiogram and cardiac catheterization are helpful in diagnosis.Actively looking for the cause can improve the prognosis.

15.
International Journal of Pediatrics ; (6): 565-567, 2014.
Article in Chinese | WPRIM | ID: wpr-454632

ABSTRACT

Kawasaki disease has been the major etiology of aquired heart problems in children. Mecha-nisms of Kawasaki disease are still unclear in the past years. Some Kawasaki disease patients were not responsive to intravenous immunoglobulin therapy. And corticosteroid therapy remains controversial in the treatment of Ka-wasaki disease. A few patients with Kawasaki disease presented severe systemic symptoms,and even died for it. It is difficult to evaluate the severity of coronary artery lesions and prognosis of Kawasaki disease. In this article, we reviewed some new concepts on diagnosis and treatments on Kawasaki disease.

16.
Chinese Journal of Perinatal Medicine ; (12): 727-731, 2012.
Article in Chinese | WPRIM | ID: wpr-430448

ABSTRACT

Objective To evaluate the clinical value of emergency bedside-echocardiography in neonatal intensive care unit.Methods Six hundred and sixty-eight infants with cardiac pathological murmurs,cyanosis and shortness of breath were detected by emergency bedside echocardiography (Sonosite Micromax 1 portable ultrasound or Philips iE33 ultrasonic systems) during January 2007 to July 2011.The accuracy of emergency bedside-echocardiography in the diagnosis of neonatal heart diseases was evaluated according to the results of surgical exploration.Results Among 668 enrolled neonates with the mean age of (7.2±1.3) d,there were 347 males and 321 females,and 309 term infants [mean gestational age (39.1±0.6) weeks (37.0~42.1 weeks)] and 359 premature infants [mean gestational age (33.7±0.91) weeks (28.9~36.9 weeks)].Totally,507 cases were found cardiac abnormality by emergency bedside-echocardiography,including 268 cases of patent ductus arteriosus (232 premature and 36 term infants),115 congenital heart disease,99 persistent fetal circulation and 25 arrythmia.Surgeries were performed on 54 infants and invasive therapy was performed on one infant,and none of them received CT,magnetic resonance imaging or invasive examinations before operation; among which,51 surgeries successed and 4 infants died.Other 452 infants were treated with medications,392 infants recovered and discharged,26 infants did not recover,14 cases died and 20 cases left the hospital before recovery.The accuracy rate of Micromax 1 portable ultrasound in diagnosing congenital heart diseases was 94.5% (52/55),while 96.4 % (53/55) for philips iE33 ultrasonic systems.Conclusions Emergency bedside-echocardiography could provide instant and valuable information of cardiovascular system,which would be helpful in making quick clinical decisions.

17.
Chinese Pediatric Emergency Medicine ; (12): 59-61, 2012.
Article in Chinese | WPRIM | ID: wpr-424425

ABSTRACT

ObjectiveTo explore the relation of heart rate variability (HRV) in neonatal asphyxia with myocardial injury.MethodsContinuous electrocardiographic monitoring by 24-hour Holter recordings was performed in 53 neonates with asphyxia and 40 healthy newborn.The difference of HRV with sinus rhythm was analyzed.Time-domain indexs included standard deviation of R-R intervals (SDNN) ; standard deviation of all mean 5-minute R-R intervals (SDANN) ;standard deviation of all R-R intervals for all 5-minute segments of 24 hours (SDNNindex ) ;root mean squared successive difference (rMSSD) ;percent of NN50 in the total number R-R intervals ( PNN50 ).Results( 1 ) Maximum heart rate,minimum heart rate and average heart rate of 24-hour Holter in healthy newborn were faster than those in newborn with neonatal asphyxia ( P < 0.05 ).And the heart rate was faster in newborn with mild neonatal asphyxia than that in newborn with serious neonatal asphyxia ( P < 0.05 ).(2) SDNN,SDANN of HRV index analysis showed significantly difference between healthy newborn and asphyxia newhom ( P < 0.05 ).There were no difference of SDNN,SDANN,SDNNindex,rMSSD and PNN50 between mild and serious neonatal asphyxia (P > 0.05 ).No significant differences of SDNNindex,rMSSD and PNN50 were found among three groups.ConclusionMyocardial injury caused by neonatal asphyxia can lead to damage of cardiac autonomic nevous and affect heart rate changes.The degree of myocardial injury is related to the degree of neonatal asphyxia.

18.
International Journal of Pediatrics ; (6): 59-61, 2010.
Article in Chinese | WPRIM | ID: wpr-390559

ABSTRACT

Marfan's syndrome(MFS) is a relatively common autosomal dominant hereditary disorder with prominent manifestations in the skeletal, ocular, and cardiovascular systems. MFS has a high penetrance. Recent research on the pathogenesis and diagnosis and therapy of MFS has made a lot of progress. So we reviewed the advance on the relation of molecular genetics and phenotype of MFS.We discussed the molecular hasis, gene mutation and location, diagnosis, clinical features and therapy. Now the detection on the molecular level has been a very important method in diagnose MFS very early or before bom.

19.
Journal of Clinical Pediatrics ; (12): 901-905, 2009.
Article in Chinese | WPRIM | ID: wpr-434221

ABSTRACT

Objective To evaluate the effect of different kind of intravenous immunoglobulin (IVIG) therapy in treating Kawasaki disease (KD) and preventing cardiac consequences (coronary artery lesion, CAL). Methods A questionnaire form and guideline for KD diagnosis were sent to 50 hospitals providing pediatric medical care in Shanghai. The data from a total of 1 682 KD patients were collected. It included 1 064 males and 618 females from 1998 through 2008 in Shanghai. The average age of the KD patients was (2.57±2.33) years old (0.1-18.8 years).The patients had been divided into 6 groups for different IVIG therapy, which included 1 g/kg once, 2 g/kg once, 0.4-0.5 g/kg five times, 1 g/kg twice, 2 g/kg twice and others. SAS 6.12 software was used for statistical analysis. Results In all KD patients, the patients treated with IV1G in 5th-10th day of illness has the least cardiac complication and CAL incidence and the group with IVIG therapy of 1 g/kg twice also has the least cardiac complication and CAL incidence. Conclusions The best doses of IVIG in treating KD is 1 g/kg twice and the IVIG therapy should be used in 5th-10th day of KD illness.

20.
Journal of Practical Radiology ; (12)2001.
Article in Chinese | WPRIM | ID: wpr-537280

ABSTRACT

Objective To study the value of clinical application for X-ray diagnosis in myodystrophy followed by osteoarticular change such as scapula,etc. Methods Different osteoarticular position,size,structure were observed on X-ray films.X-ray features of dysostosis,malformation and dislocation of joint caused by myodsytrophy were analysed in three cases.Results X-ray findings of myodsytrophy in 3 cases included:revolving and displacement of scapula,scapula getting shorterand smaller,dislocation of acromioc cleido joint and shoulder joint and bending and malformation of shoulder joint.Conclusion It can be certain that myodsytrophy directly causes the maltormation of osteoartthritis to make a diagnosis for one who suffers from a myodystrophy with obvious osteodysplasty by X-ray and find out the harm of this kind of disease.Finally we can predict how it goes in anaphase.

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