Medium and long-term follow-up of the Pul-Stent in treating postoperative branch pulmonary artery stenosis in children with congenital heart disease / 中华儿科杂志

Objective: To evaluate the effectiveness and safety of Pul-Stent as the treatment of postoperative branch pulmonary artery stenosis in children with congenital heart disease. Methods: This was a retrospective study. Thirty-three patients who underwent Pul-Stent implantation in Shanghai Children's Medical Center due to postoperative residual pulmonary artery stenosis from August 2014 to June 2015 were included. The immediate curative effect, follow-up and complications of Pul-Stent implantation were assessed. Comparisons between groups were performed with unpaired Student t test. Results: Pul-Stent implantation of 33 patients (19 males and 14 females) were performed successfully. Thirty-one patients underwent percutaneous stenting, and 2 patients underwent hybrid stenting. A total of 35 Pul-Stents were implanted (19 of model small, 15 of model medium and one of model large), 23 stents were planted in the proximal left pulmonary artery and 12 stents were in the proximal right pulmonary artery. The initial diameter of dilation balloon ranged from 6 to 16 mm, and the long sheath of percutaneous implantation ranged from 8 to 10 F in 29 patients (29/31, 94%). After stenting, the diameter of the narrowest segment of pulmonary artery increased from (4.0±1.7) mm to (9.1±2.1) mm in all patients (t=-21.60, P<0.001). The pressure gradient at the stenosis in 26 patients after biventricular correction decreased from (30.5±12.3) mmHg (1 mmHg=0.133 kPa) to (9.9±9.6) mmHg (t=12.92, P<0.001), and the right ventricular to aortic pressure ratio decreased from 0.57±0.14 to 0.44±0.12 (t=7.44, P<0.001). The pressure of the superior vena cava after stenting in 5 patients after cavopulmonary anastomosis decreased from (17.0±1.9) mmHg to (14.0±0.7) mmHg (t=2.86, P=0.046). Two patients died during reoperation for repairing other cardiac malformations. The remaining 31 patients were clinically stable during the follow-up period of (5.3±1.6) years, and one stent fracture was found on chest X-ray. Cardiac catheterization reexaminations in 16 patients showed that restenosis was found in one stent, while stent position and patency were satisfactory in the remaining stents. Nine children underwent post-dilation without stent fracture, displacement or aneurysm formation. Cardiac tomography showed no stent stenosis, fracture observed, or significant change in diameter of the stent in 8 patients. The inner diameter and pulmonary blood perfusion could not be accurately evaluated due to artifacts by cardiac magnetic resonance imaging in 4 patients. Conclusions: Pul-Stent has good compliance and adequate radial strength, and can dilate further over time to accommodate for somatic growth. It performs safely and effectively in treating post-operative branch pulmonary artery stenosis in children.

Transcatheter device closure for perimembranous ventricular septal defects with aortic valve prolapse:Effect and prognosis / 中国实用儿科杂志

OBJECTIVE: To evaluate the the effect and prognosis of transcatheter device closure for perimembranous ventricular septal defects(PmVSDs)with aortic valve prolapse(AVP). METHODS: The clinical data of 1108 patients who underwent percutaneous closure from January 2011 to January 2017 were collected to conduct this retrospective study,mainly including transthoracic echocardiography,digital subtraction angiography data,occluder type and sociodemographic information(age,weight and gender). T-test and Mann-Whitney U test were used to compare group measurement data. Wilcoxon signed rank test was used for the nonnormal distribution of paired design data. Fisher exact test was used for the four fold table. Monte Carlo approximation exact probability method was used for the R×C table.Multiple Logistic-regression analysis was used to estimate the risk factors of AR. RESULTS: Compared with 10(0.9%)of1053 patients without AVP,8(14.5%)of the 55 children with preoperative AVP presented aggravated postoperative aortic regulation(AR)and AR usually occurred in the long-term follow-up(>1 year). None of the AR progressed to moderate or severe. There were no significant changes in left ventricular ejection fraction,left ventricular shortening rate or AR before and after interventional operation in PmVSDs with AVP. CONCLUSION: Percutaneous closure for ventricular septal defect combined with AVP is an effective alternative to surgery when using appropriate occluders,meeting related standards and operated in an experienced cardiovascular center.

Fatty acid oxidation disorders and cardiomyopathy / 中国实用儿科杂志

myocardium. Fatty acid oxidation disorders(FAODs)can give rise to insufficiency of mitochondrial energy production and accumulation of metabolic intermediates in cardiomyocytes,such as lipid and long-chain acyl carnitine,leading to myocardial lesions.FAODs include carnitine-dependent fatty acid transport disorders and mitochondrial fatty acid beta-oxidation disorders,with any enzyme or transporter defect in fatty acid oxidation process resulting in this disease. Although FAODs are one of the rare causes of pediatric cardiomyopathy,some patients with FAODs are expected to have cardiomyopathy alleviated and quality of life improved by early correction of fatty acid metabolic disorders.

Improvement of Suoquan Capsules on bladder function of diabetic mice / 中成药

AIM To investigate the improving effect of Suoquan Capsules (Linderae Radix,Alpiniae oxyphyllae Fructus and Dioscoreae Rhizoma) on mice with diabetic cystopathy and its mechanism of action.METHODS Sixty mice were randomly assigned into normal group (n =8) and model group (n =52);the diabetic models of the latter were induced by high-fat feeding combined with streptozotocin (STZ) injection,then modeled mice (n =32) were randomly divided into model,Mecobalamin Tablets,low-and high-dose Suoquan Capsules groups.The influences of Suoquan Capsules on fasting blood glucose (FBG),glycated serum protein (GSP) level and general conditions were observed.The bladder leak point pressure (BLPP) was determined.Histopathological staining was performed on urinary bladder.And the expressions of substance P and NK1 receptor were detected by double immunofluorescent staining.RESULTS There were no significant differences in FBG,GSP,body weight,food intake and water consumption among various groups.The high-dose Suoquan Capsules significantly decreased urine volume of mice.Compared with the model group,the treatment with Suoquan Capsules markedly increased BLPP,the expressions of substance P and NK1 receptor were significantly increased,and the histopathology of bladder in mice was obviously improved.CONCLUSION Suoquan Capsules improves the diabetic cystopathy in mice,and its mechanism maybe related to the up-regulation of substance P and NK1 receptor expressions in bladder tissue.

Mechanism of anti-Helicobacter pylori urease activity of patchouli alcohol / 中国中药杂志

To investigate the effect of patchouli alcohol on inhibiting Helicobater pylori urease activity, and its effect on expression levels of related genes, and lay the foundation for further research on the effect of patchouli alcohol on H. pylori colonization and infection. H. pyloriwas cultured and identified by gram staining, rapid urease test (RUT) and PCR method. Then agar dilution method was used to detect the bacterial survival after 1 h intervention by different concentrations of patchouli alcoholin the acidic (pH 5.3) and neutral (pH 7.0) conditions; berthelot method was used to detect urease activity and RT-qPCR method was used to detect the expression changes of ureA, ureB, ureE, ureH, ureI, and nixA related urease genes. The results showed that the survival rate of H. pyloriwas not significantly changed but the urease activity was obviously decreased after intervention by different concentrations of patchouli alcohol; meanwhile, the expression levels of ureA, ureB, ureE, ureH, ureI, and nixA were decreased to different degrees. Therefore, patchouli alcohol could inhibit H. pylori urease activity in both acidic and neutral conditions, and the mechanism may be related to down-regulation of urease gene expression.

An analysis on the prognostic factors of community -acquired pneumonia among children / 预防医学

Objective To explore the prognostic factors of community -acquired pneumonia (CAP)among children. Methods A total of 783 children with CAP admitted to hospital from 2009 to 2012 were selected.Related information including baseline data was extracted from the hospital health records and analyzed by univariate and multiple logistic regression analysis.Results The logistic regression analysis showed that obnubilation (OR =5.866,95%CI =2.020 -17.033),dysphoria (OR =1.420,95%CI =1.038 -1.942),multi -infection(OR =1.376,95%CI =0.993 -1.907) and polypnea(OR =1.026,95%CI =1.004 -1.049)were the independent risk factors that influenced the prognosis of patients with community -acquired pneumonia.Conclusion More attentions should be paid to CAP children with the symptoms of obnubilation,dysphoria,multi -infection and polypnea.

A study on genetic characteristics of Enterovirus type 71 of hand-foot-mouth disease in Shaoxing city / 预防医学

Objective To undersyand the genetic characteristics of Enterovirus Type 71 (EV 71 )isolated from clinical specimens of children with hand-foot-mouth disease (HFMD)in Shaoxing city from 2012 to 2013.Methods RNA was extracted from specimens collected from stool,throat swab and vesicle from children with HFMD,and EV 71 were identified by reverse transcriptase polymerase chain reaction (RT-PCR).Ten positive samples were isolated and were amplified by segmented PCR,then,they were spliced into a full-length after sequenced according to the reference strains. Homology analysis on region VP1 and phylogenetic tree were carried out based on nucleotide sequence and amino acid sequence.Results The results indicated that the ten isolated strains were all EV 71 positive.Sequence alignment of VP1 showed that the nucleotide and amino acid homogeneity of these 10 strains were related to C4a genotypes,with 95.06%-97.87% and 93.60%-98.99% respectively.Phylogenetic tree based on VP1 indicated that these 10 strains all belonged to C4a genotype.Conclusion The identified EV 71 strains isolated from HFMD children in Shaoxing city belong to genotype C4a.

Transcatheter closure of patent ductus arteriosus with the new Amplatzer Duct OccluderII / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the indications, methodology and results of the transcatheter closure of patent ductus arteriosus (PDA) with the new Amplatzer Duct Occluder II (ADO-II).</p><p><b>METHOD</b>Totally 51 patients underwent transcatheter closure of PDA with the new ADO-II. The devices were delivered by 4F or 5F sheath through arterial or venous side respectively. The descending aorta angiography and transthoracic echocardiography was performed to evaluate the device position, residual shunt and complications caused by the device during and after implantation.</p><p><b>RESULT</b>Forty-nine patients had successful transcatheter closure of the PDA without significant residual shunts and artery obstruction during the short-term follow-up. One patient received the ADO-II dislodgment and first generation ADO re-implantation for the obvious descending aortal obstruction caused by ADO-II. Another patient had the ADO-II dislodgment and left pulmonary artery shaping surgery, because the ADO-II implantation led to obstruction of the left pulmonary artery. Both the obstructions in these two patients were ameliorated afterwards.</p><p><b>CONCLUSION</b>The transcatheter closure using the ADO-II is safe and effective for the non-window type PDA with a small size.</p>

Efficacy of the transcatheter closure of perimembranous and muscular ventricular septal defects with the Amplatzer duct occluder II / 中华心血管病杂志

<p><b>OBJECTIVES</b>To evaluate the feasibility and efficacy of transcatheter closure of perimembranous ventricular septal defects (pmVSD) with aneurysmatic formation and muscular ventricular septal defects (mVSD) with Amplatzer duct occluder II.</p><p><b>METHODS</b>This retrospective analysis included 48 cases received transcatheter closure of pmVSD aneurysmatic formation or mVSD from February 2011 to March 2012 in our hospital (42 pmVSD with aneurysmatic formation and 6 mVSD). Median age was 5.2 years (range: 1.8 - 15 years), and median weight was 20.2 kg (range: 12 - 44 kg). Amplatzer duct occluder II was selected depending on the condition of ventricular septal defect. The device was implanted by antegrade or retrograde approach. Complications such as residual shunt, valvular regurgitation and arrhythmia were evaluated by echocardiography or angiography. Median follow-up was 9.5 months (range: 1 - 13 months).</p><p><b>RESULTS</b>The mean ratio of pulmonary (Qp) to systemic (Qs) blood flow was 1.35 ± 0.15 before transcatheter closure. The diameter of exit hole of ventricular septal defects was (2.46 ± 0.53) mm measured by transthoracic echocardiography, and (2.35 ± 0.40) mm by angiography. Successful implantation of the device was achieved in 46 patients (96%) and unsuccessful in two cases due to acute aortic insufficiency. Forty-two (92%) patients were closed successfully, and trivial residual leak was evidenced in four patients and remained unchanged during follow-up. One patient with mVSD still had trivial residual shunt at 6 months post procedure. New trivial tricuspid insufficiency was observed in 1 patient (2.1%) during follow-up. Two patients developed procedural related left anterior fascicular block and remained unchanged during follow-up.</p><p><b>CONCLUSIONS</b>pmVSD with aneurysm and mVSD could be successfully treated with Amplatzer duct occluder II. However, the long waist and large disc of the device could interfere with tricuspid valve function and cause tricuspid insufficiency.</p>

Clinical presentation and therapeutic outcomes of carnitine deficiency-induced cardiomyopathy / 中华儿科杂志

<p><b>OBJECTIVE</b>Carnitine deficiency has been associated with progressive cardiomyopathy due to compromised energy metabolism. The objective of this study was to investigate clinical features of carnitine deficiency-induced cardiomyopathy and the therapeutic efficacy of L-carnitine administration.</p><p><b>METHOD</b>Between January 2010 and December 2011, filter-paper blood spots were collected from 75 children with cardiomyopathy. Free carnitine and acylcarnitine profiles were measured for each individual by tandem mass spectrometry (MS/MS). For those in whom carnitine deficiency was demonstrated, treatment was begun with L-carnitine at a dose of 150 - 250 mg/(kg·d). Clinical evaluation, including physical examination, electrocardiography, chest x-ray, echocardiography and tandem mass spectrometry, was performed before therapy and during follow-up.</p><p><b>RESULT</b>Of 75 cardiomyopathy patients, the diagnosis of carnitine deficiency was confirmed in 6 patients, which included 1 boy and 5 girls. Their age ranged from 0.75 to 6 years. Free carnitine content was (1.55 ± 0.61) µmol/L (reference range 10 - 60 µmol/L). Left ventricular end-diastolic diameter (LVDd) was (5.04 ± 0.66) cm and left ventricular ejection fraction (LVEF) was (38.5 ± 10.5)%. After 10 - 30 d therapy of L-carnitine, free carnitine content rose to (30.59 ± 15.02) µmol/L (t = 4.79, P < 0.01). LVDd decreased to (4.42 ± 0.67) cm (t = 4.28, P < 0.01) and LVEF increased to (49.1 ± 7.6)% (t = 6.59, P < 0.01). All patients received follow-up evaluations beyond 6 months of treatment. Clinical improvement was dramatic. LVEF returned to normal completely in all the 6 patients. LVDd decreased further in all the 6 patients and returned to normal levels in 3 patients. No clinical signs or symptoms were present in any of the 6 patients. The only complications of therapy had been intermittent diarrhea in 1 patient.</p><p><b>CONCLUSION</b>Tandem mass spectrometry is helpful to diagnose carnitine deficiency and should be performed in all children with cardiomyopathy. L-carnitine has a good therapeutic effect on carnitine deficiency-induced cardiomyopathy.</p>

Efficacy of percutaneous balloon aortic valvuloplasty for severe aortic valve stenosis in four infants under three months of age / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the outcome of percutaneous balloon aortic valvuloplasty (PBAV) for severe aortic valve stenosis in infants younger than 3 months of age.</p><p><b>METHODS</b>Four infants under the age of 3 months (ranged from 34 to 87 days) underwent PBAV for severe aortic stenosis between June 2010 and March 2011 were included in this study. The weight of infants ranged from 2.8 to 4.8 kg. The peak systolic valve gradient, left ventricular ejection fraction (LVEF) and aortic regurgitation were measured in all patients just before and immediately after balloon dilation respectively. Patients were followed-up up to 1 month after PBAV.</p><p><b>RESULTS</b>The aortic annulus diameter ranged from 7.0 to 8.8 mm. The ratio of balloon to aortic annulus diameter ranged from 0.86 to 1.00. PBAV was successful in all cases. The peak systolic valve gradient measured by Doppler echocardiography was (60.6 ± 15.2) mm Hg (1 mm Hg = 0.133 kPa) and LVEF was (47.6 ± 7.5)% before PBAV. Immediately after PBAV, the peak systolic valve gradient decreased to (29.5 ± 8.0) mm Hg (P < 0.01) and LVEF increased to (52.2 ± 18.9)% (P > 0.05). Two patients experienced significant bradycardia during PBAV and restored normal cardiac rhythm after cardiopulmonary resuscitation. At 1 month after PBAV, the peak systolic valve gradient measured by Doppler echocardiography was (36.5 ± 11.0) mm Hg (P < 0.05 vs. pre-PBAV) and LVEF was (81.0 ± 1.1)% (P < 0.01 vs. pre-PBAV). Only trivial to mild aortic regurgitation was detected post PBAV in the 4 patients.</p><p><b>CONCLUSION</b>PBAV is a feasible palliative procedure for infants with isolated aortic valve stenosis without annular or ventricular hypoplasia.</p>

Adenosine for pulmonary vasodilator testing in children with idiopathic pulmonary arterial hypertension / 中华儿科杂志

<p><b>OBJECTIVE</b>The assessment of pulmonary vascular reactivity plays an important role in the management of idiopathic pulmonary arterial hypertension (IPAH). The aim of this study was to explore the indications and methodology of pulmonary vasodilator testing in children with IPAH.</p><p><b>METHODS</b>From October 2009 to June 2011, a cohort of pediatric patients with IPAH in WHO functional classes II to III were enrolled in the study. Right heart catheterization was performed in all patients. After baseline hemodynamics were obtained, adenosine infusions were started at a dose of 50 µg/(kg·min), increased by 25 µg/(kg·min) at 2 min intervals to a maximum of 250 µg/(kg·min) or until a positive acute response.</p><p><b>RESULTS</b>A total of 15 patients with IPAH were enrolled in the study. The mean age of the patients was 6.3 yrs. Mean pulmonary artery pressure (mPAP) was (67.1 ± 15.9) mm Hg. Pulmonary capillary wedge pressure (PCWP) was (9.7 ± 2.9) mm Hg. Pulmonary vascular resistance index (PVRI) was (17.9 ± 7.5) Wood U·m(2). Three patients were responders, defined as a fall in mPAP of at least 10 mm Hg to a pressure level of 40 mm Hg or lower. Twelve patients were nonresponders according to the same criteria. Five out of the 15 patients experienced adverse effects, including chest discomfort (n = 1), systemic hypotension (n = 3) and bradycardia (n = 1). All side effects abated within 30-60 s of the discontinuation of the adenosine infusion.</p><p><b>CONCLUSION</b>Adenosine is an effective vasodilator in children with IPAH and can be used for safe and rapid assessment of vasodilator reserve in these patients.</p>

A novel mutation in the BMPR2 gene in familial pulmonary arterial hypertension / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Familial pulmonary arterial hypertension (FPAH) is an autosomal dominant disorder characterized by plexiform lesions of endothelial cells in pulmonary arterioles which leads to elevated pulmonary arterial pressure, right-sided heart failure and death. Heterozygous mutations in the bone morphogenetic protein type II receptor gene (BMPR2) have been found to underlie a majority of FPAH cases. More than 140 distinct mutations have been identified in FPAH cases and in idiopathic pulmonary arterial hypertension (IPAH) cases, but only one mutation has been reported in Chinese patients.</p><p><b>METHODS</b>A three-generation pedigree of FPAH and another 10 patients with IPAH were collected. In the family, two of the 9 surviving and one deceased family member were diagnosed as FPAH. The entire protein-coding region and intron/exon boundaries of the BMPR2 gene were amplified by PCR using DNA samples from affected individuals. Direct sequencing of PCR products was performed on both the sense and antisense strands. To confirm the segregation of the mutation within the family and exclude the presence of the mutation in normal subjects, the relevant exon was amplified by PCR, followed by mutation-specific RPLP analysis.</p><p><b>RESULTS</b>In the Chinese pedigree with FPAH an A-to-T transition at position 1157 in exon 9 of the BMPR2 gene was identified which resulted in a Glu386Val mutation. We confirmed the segregation of the mutation within the family and excluded the presence of the mutation in a panel of 200 chromosomes from normal subjects. No mutation was detected in BMPR2 in the other 10 patients with IPAH.</p><p><b>CONCLUSIONS</b>This amino acid substitution occurs at a glutamic acid that is highly conserved in all type II TGF-beta receptors. The nearly invariant Glu forms an ion pair with an invariant Arg at position 491 thereby helping to stabilize the large lobe. Substitution of Arg at position 491 is the most frequently observed missense mutation in FPAH, but until now no mutations at position 386 have been found in FPAH. The predicted functional impact of the Glu386Val mutation and its absence in healthy controls support the mutation as the cause of FPAH.</p>

Effect of elastase inhibitor on pulmonary hypertension induced by monocrotaline / 中华儿科杂志

<p><b>OBJECTIVE</b>Pulmonary hypertension is a proliferative vascular disease characterized by pulmonary vascular structural remodeling. Until now, the pathogenesis of pulmonary hypertension is still not fully understood. Although considerable progress has been made, there is, to date, no cure for advanced pulmonary vascular disease. Recently, a number of studies suggest that endogenous vascular elastase (EVE) play a role in the vascular changes associated with pulmonary hypertension. The purpose of the study was to determine whether an elastase inhibitor might reverse advanced pulmonary vascular disease produced in rats by injection of monocrotaline.</p><p><b>METHODS</b>One hundred and twenty male Sprague-Dawley rats were used in this study. The rats were divided into three groups: control, model and ZD-0892 groups. In the model and ZD-0892 groups, the rats were subjected to a single subcutaneous injection of monocrotaline (60 mg/kg) in the hind flank, while the rats in control group received an equivalent volume of 0.9% saline. From day 21, the rats in the ZD-0892 and model groups received twice-daily gavage tube feedings of either ZD-0892 at a dose of 240 mg/kg per day or its administration vehicle, while the rats in control group were subjected to an equivalent volume of 0.9% saline. On days 21, 28 and 35 post-injection, the elastolytic activity was measured with a fluorescence microplate reader and pulmonary artery pressure was detected via catheterization. Meanwhile, the lungs were evaluated morphologically, using the barium-gelatin perfusion technique.</p><p><b>RESULTS</b>The injection of monocrotaline led to severe pulmonary hypertension in rats 21 days later and pulmonary artery elastolytic activity increased remarkably. A 1-week treatment with ZD-0892 resulted in declines in elastase activity. This was associated with significant declines in pulmonary artery pressure, decreases in muscularization of peripheral arteries and reductions in medial hypertrophy. After 2 weeks, elastase activity returned to normal level. Pulmonary artery pressure and structure were normalized.</p><p><b>CONCLUSION</b>Increased elastase activity is important in the development of vascular changes and progressive pulmonary hypertension. ZD-0892 can suppress the elastase activity and completely reverse the fatal pulmonary hypertension induced by monocrotaline in rats.</p>