Objective To study the clinical characteristics and curing methods of non-compaction of ventricular myocardium(NVM) in children with a view to improve its diagnosis and treatment.Methods From Jan.2009 to Dec.2012,the clinical data,treatment and follow-up of 22 patients with NVM in Beijing Children's Hospital Affiliated to Capital Medical University were analyzed.Results The 22 patients included 13 boys and 9 girls aged from 4 months to 13 years and 3 months with an average age of 3 years and 7 months.Five of them had a family history of cardiac diease.Twenty patients suffered from ventricular dysfunction,with 2 cases at level Ⅰ,5 cases at level Ⅱ,7 cases at level Ⅲ and 6 cases at level Ⅳ.Asymptomatic heart murmur was heard in 6 patients and creatine kinase car-diac isoenzymes raise was found in 6 patients.X-ray indicated that heart shadow enlargement and electrocardiographic abnormalities in all 22 patients.Echocardiography features of all cases indicated left ventricular enlargement,and 20 cases of the children had ventricular systolic dysfunction.Left ventricle was detected in 21 patients,while double ventricle dysfunction detected in 1 patient.Thrombus was found in none of the patients.Magnetic resonance imaging was performed in 8 cases of the patients and CT was performed in 14 cases,all of them showed abnormalities.Anti-heart failure medications were administered to 20 patients and aspirin to all of the children.The patients were followed up for 0.5 to 3.0years.Two patients died from heart failure during the follow-up.In 2 cases whose cardiac ejection fraction was more than 60％ had little change of the internal diameter of left ventricle and cardiac function.In 3 cases,the left ventricular diameter narrowed by 3 to 5 millimeter and ejection fraction increased by 5％ to 10％ over the previous.In 5 cases,the left ventricular diameter expanded by 5 to 7 millimeter and ejection fraction decreased by 3％ to 5％.In 10 cases,the left ventricular diameter and heart function had no significant change.Conclusions The main clinical manifestions of NVM in children are cardiac dysfunction,and the prognosis is usually poor.Echocardiography is the principal means of diagnosis.Magnetic resonance imaging can contribute to the diagnosis.

Objective To investigate the clinical characteristics,diagnosis,treatment and prognosis of hypertrophic cardiomyopathy(HCM) in children.Methods Twenty-five cases of children confirmed with HCM in Beijing Children's Hospital from October 2000 to September 2013 were analyzed retrospectively through their clinical manifestations,laboratory tests,treatment and prognosis.Results HCM was found to be more common in older children.The average age of the patients in the studied cases was 8 years and 9 months and the male to female ratio was 3.1:1.Their clinical manifestations were as the follows:5 cases with shortness of breath and fatigue,4 cases with chest tightness and long breath,3 cases with precordial discomfort,5 cases with cough and wheezing and found to have cardiac abnormalities,8 cases found with asymptomatic heart murmurs with no other symptom,2 cases with syncope episodes,6 cases with a clear HCM family history,3 cases with a family history of infant deaths (specific condition not clear).ECG:18 cases showing left ventricular and/or right ventricular hypertrophy and ST-T changes,3 cases showing abnormal Q waves,2 cases showing ST-T changes,2 cases with Ⅰ ° atrio-ventricular block,1 case with left bundle branch block,and 1 case with left anterior division block.Cardiac enzymes were elevated in 5 cases.Chest X-rays showed enlarged heart shadow in 17 cases.Echocardiography revealed non-obstructive hypertrophic cardiomyopathy in 23 cases and obstructive hypertrophic cardiomyopathy in 2 cases.Left ventricular systolic function was decreased in 2 cases,and diastolic dysfunction in 3 cases.Twenty-one patients took oral metoprolol,1 patient took verapamil,1 patient took propranolol and 2 patients took no medication.The follow-up period of 2 months to 13 years witnessed 2 cases of death and 23 cases of survival.Conclusion HCM in children lacks specific clinical manifestations and the conditions of the patient are usually severe with progressive development.HCM is one of the main reasons of sudden death in adolescent,with poor prognosis.

Objective To study the clinical characteristics of children with chaotic atrial tachycardia (CAT),and to improve its clinical diagnosis and treatment.Methods Clinical data,follow-up,treatment and prognosis concerning 33 patients with CAT from Sep.2001 to Oct.2013 in Beijing Children's Hospital were analyzed.Results The 33 patients included 23 boys and 10 girls with ages ranging from 3 days to 13 months,5 months on the average.CAT was conformed by electrocardiogram in all patients:multifocal atrial tachycardia defined by 3 distinct P'-waveforms,irregular P'R,RR and P'-P' intervals,and isoelectric baseline between P'-waves.Combined with symptoms of atrial flutter,atrial fibrillation,atrial premature beats,atrial tachycardia,and interior conduction,differences were detected by 24-hour holter electrocardiography.Echocardiographic features indicated mild heart enlargement in 19 cases,medium enlargement in 4 cases,severe enlargement in 3 cases,atrial septal defect in 5 cases,ventricular septal defect in 3 cases,atrial septal defect coupled with patent ductus arteriosus in 1 case,congenital total anomalous pulmonary venous cormection in 1 case,and patent foramen ovale closure in 12 cases.Twenty-three patients had heart failure.Digoxine,amiodarone and Betaloc were administered to the patients for maintaining their heart functions.No antiarrhythmic drug therapy was used in 2 patients.Twenty-six of the patients (78.8％) had normal electrocardiographic sinus rhythm within 3 to 18 months after their discharge from hospital.Three of the patients (9.1％) still had CAT symptoms in their eletroccardiogram,but their heart rates were kept under control during the 12-month follow-up clinical visits.Of 26 children with cardiac enlargement,echocardiograms in 21 cases (80.8％) returned to normal within 1-3 months after their electrocardiograms returned to normal,and for the other 5 cases (19.2％),the recovery took 6-12 months.One patient died and 3 patients did not participate in the follow-up visits.Conclusions CAT is often found in newborns and infants.Its diagnosis relies on electrocardiography and 24-hour holter electrocardiography.Treatment with Digoxine,and/or β-receptor block,and amiodarone according to with the heart functions of patients can restore sinus rhythm,and achieve good prognostic results.

Objective To study the clinical characteristics and therapy of children with idiopathic ventricular tachycardia(IVT).Method Clinical features,laboratory examination,treatment and outcomes of cases involving 36 children with IVT were retrospectively and statistically analysed.Results IVT often happened in teenagers.The average age of selected patients was 7 years and 8 months,and the ratio of male and female was 1.11.Electrokardiogram(EKG)results showed 23 cases(63.8%)were VT that arised from the left ventricular tachycardia(ILVT)with right bundle branch block,and 13 cases(36%)were VT that arise from the right ventricular outflow tract(RVOT VT)with left bundle branch block.Among the 23 children with tachycardia,15 children had palpitation,2 children had syncope.Of the 13 children with right ventricular tachycardia,3 children had increased heartbeat,and 1 child suffered from weakness,others had no significant clinical symptoms.Cases with ILVT had more serious clinical symptoms than cases with right ventricular tachycardia.It was of marked significance to compare their differences(P

Objective To explore the clinical characters,therapy and prognoses of hyperthyroid cardiac disease in children.Method Fourteen hospitalized cases with hyperthyroid cardiac disease(5-14 years old) collected from January 2001 to January 2005 were analyzed(retrospecti)vely.Results Fourteen children with hyperthyroid cardiac disease had different degree of enlarged of heart,arrhythmia and heart failure.All of the cases were received treatment of hyperthyroidism and anti-arrhythmia.After admission for 2 weeks,the symptoms of 13 cases relived or disappeared,and after 4-6 weeks the enlarged heart,arrhythmia and heart failure disappeared.One case died after recurrent heart failure.Conclusions Hyperthyroid cardiac disease in children can be curative and the changes of cardiac blood-vessel can be recuperative.It is critical for doctors to know the manifestations of hyperthyroidism and take the cardiac examination and the function of the thyroidism and take proper treatment as soon as possible.

Objective To investigate the value of 99Tcm-MDP imaging for the differential diagnosis between infection and aseptic loosening after total hip arthroplasty.Methods During February 2008 to August 2011,74 patients (32 males,42 females,average age (64.3±11.2) years) with hip pain after arthroplasty underwent 3-phase (blood flow,blood pool and bone phases) 99Tcm-MDP imaging.All patients had measurements of serum C reactive protein (CRP) and erythrocyte sedimentation rate (ESR).Joint infection was defined as either increased peri-prosthetic soft tissue activity during perfusion and blood pool phases or increased peri-prosthetic bone activity during uptake phase,or positive in all 3 phases.Aseptic loosening was defined as having negative 99Tcm-MDP in all 3 phases.Clinical diagnosis was chosen as the gold standard.The blood flow-pool imaging was compared with the serum examinations.x2 test was used for statistical analysis.Results There were 74 symptomatic joints in 74 patients,including 24 joint infections and 50 aseptic loosening.For the detection of peri-prosthetic infection,combined perfusion-blood-pool phase was more accurate than bone uptake phase (90.5％ (67/74) vs 55.4％ (41/74) ;x2=23.159,P＜0.001),with the sensitivity of 91.7％ (22/24)vs 70.8％ (17/24) and specificity of 90.0％ (45/50) vs 48.0％ (24/50),respectively.The blood flow-pool imaging was also more accurate than CRP (73.0％ (54/74) ;x2 =7.656,P＜0.05) and ESR (71.6％ (53/74) ; x2 =8.633,P＜0.05),respectively.Conclusion 99Tcm-MDP perfusion/blood flow-pool imaging is an accurate modality for differentiating peri-prosthetic infection from aseptic loosening in patients with hip pain after arthroplasty.

This study was designed to validate the correlation between integrated pharmacokinetic and therapeutic effects of alkaloids using bile processed Rhizoma Coptidis (BRC). Rats were divided into three groups: normal, disease model, model+BRC. Rats were induced to have an excessive heat syndrome. Rectal temperatures were collected at 0, 3, 6 and 9 h after single oral administration of the drugs. The plasma concentrations of three alkaloids were quantified at different times by UPLC-MS/MS after the administration of BRC. An approach of self-defined weighting coefficiency was created to the holistic pharmacokinetic profiles of alkaloids in BRC. The classified and integrated synthetic concentrations were obtained, and then the pharmacokinetic parameters of alkaloids were calculated from non-compartmental model analysis. The potential relationship between the integrated mean concentration of alkaloids and the antifebrile efficacy was investigated. The holistic t(max) of alkaloids was 1.11 h, the antifebrile effect of BRC at 3 h was improved over the model group. Double peaking appeared in the integrated blood concentration-time curve, the second t(max) of alkaloids was 4.82 h. The antifebrile effects of BRC at 3-6 h were significant, and the antifebrile effects at 6-9 h was decreased significantly. Dynamic variation of alkaloids of BRC in the body exhibited the similarity to the pattern of its antifebrile effect.
Administration, Oral ; Alkaloids ; pharmacokinetics ; Animals ; Bile ; Coptis ; chemistry ; Drugs, Chinese Herbal ; chemistry ; pharmacokinetics ; Rats ; Rats, Sprague-Dawley ; Rhizome ; chemistry ; Tandem Mass Spectrometry

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Endometrial Neoplasms ; metabolism ; pathology ; surgery ; Endometrial Stromal Tumors ; metabolism ; pathology ; surgery ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVE To study the diagnosis and treatment of parotid acinic cell carcinoma. METHODS The clinical data of 19 cases with parotid acinic cell carcinoma treated from 1985 to 2007 were retrospectively reviewed.Four patients underwent superficial parotidectomy,5 patients underwent total parotidectomy,6 patients underwent superficial parotidectomy plus partial deep parotidectomy,6 patients underwent total parotidectomy and neck dissection.Eight patients received postoperative radiotherapy.One patient only received radical radiotherapy after biopsy.All patients were followed up for 1 to 20 years.RESULTS Nobody died after operation.Five cases recurred after operation, and the recurrence rate was 26.3%(5/19) CONCLUSION The main treatment for parotid acinic cell carcinoma is complete resection and postoperative radiotherapy.

This study was designed to validate the correlation between integrated pharmacokinetic and therapeutic effects of alkaloids using bile processed Rhizoma Coptidis (BRC). Rats were divided into three groups: normal, disease model, model+BRC. Rats were induced to have an excessive heat syndrome. Rectal temperatures were collected at 0, 3, 6 and 9 h after single oral administration of the drugs. The plasma concentrations of three alkaloids were quantified at different times by UPLC-MS/MS after the administration of BRC. An approach of self-defined weighting coefficiency was created to the holistic pharmacokinetic profiles of alkaloids in BRC. The classified and integrated synthetic concentrations were obtained, and then the pharmacokinetic parameters of alkaloids were calculated from non-compartmental model analysis. The potential relationship between the integrated mean concentration of alkaloids and the antifebrile efficacy was investigated. The holistic t(max) of alkaloids was 1.11 h, the antifebrile effect of BRC at 3 h was improved over the model group. Double peaking appeared in the integrated blood concentration-time curve, the second t(max) of alkaloids was 4.82 h. The antifebrile effects of BRC at 3-6 h were significant, and the antifebrile effects at 6-9 h was decreased significantly. Dynamic variation of alkaloids of BRC in the body exhibited the similarity to the pattern of its antifebrile effect.