Transcriptome characterization of intestinal microbial flora in different pregnant women using Illumina sequencing / 中国病理生理杂志

[ ABSTRACT] AIM:To investigate the characteristics of the intestinal microbial flora in the pregnant women with congenital heart disease fetus ( PW group) and normal pregnant women ( NW group) .METHODS: Stool samples were collected from 15 NW and 17 PW cases.The bacterial genomic DNA was extracted.The 16S rDNA was amplified by PCR, and the second generation of Illumina sequencing was conducted.RESULTS: We obtained 2 696 276 ( NW group) and 2 445 530 ( PW group) optimized sequences.The coverage was greater than 97%.We obtained 77 243 operational taxono-mic units ( OTUs) in NW group and 75 600 OTUs in PW group after a 97%similarity merge.In NW group, the Chao 1 in-dex and the Shannon index were greater than those in PW group.The diversity analysis of microbial population indicated that they were mainly composed of Firmicutes, Proteobacteria and Actinobacteria.In family, the Bifidobacteriaceae and Cori-obacteriaceae were significantly different through analysis of variance.CONCLUSION: The Bifidobacteriaceae and Cori-obacteriaceae may play an important role in the occurrence of congenital heart disease.

A comparative study of right ventricular outflow tract pacing and right ventricular apical pacing in children / 实用医学杂志

Objective To compare the impacts of right ventricular outflow tract pacing and right ventricular apical pacing on short-term cardiac function, QRS duration and pacing parameters in children undergoing VVI pacemaker therapy. Methods The clinical data of 38 children undergoing VVI pacemaker treatment in our department from July 2006 to November 2013 were retrospectively reviewed and analyzed to make grouping: 22 with right ventricular outflow tract pacing based on the operational records were assigned in one group and 16 with right ventricular apical pacing as the other group. The two groups were compared in terms of cardiac function indexes , QRS complex width and intra- and post- operative parameters of the implanted pacemakers. Results There were no significant differences between the two groups in pre-and post-operative cardiac function indexes , intra- and post-operative difference in pacing thresholds, pacing wire impedance and R-wave amplitude. The pre-and post-operative QRS duration [(134.95 ± 12.86)ms vs.(147.44 ± 22.35)ms, t=1.35, P=0.01] was statistically significant between the groups. Conclusion The right ventricular outflow tract pacing for children patients is safe and feasible. Although the two pacing approaches achieved lengthened duration of QRS, the right ventricular outflow tract pacing is more effective in lengthening the duration of QRS.

Experimental study of bioabsorbable iron-based pulmonary artery stent / 中华实用儿科临床杂志

Objective To monitor the degradation and bio-safety features of the bioabsorbable iron-based stent planted in the pig pulmonary artery.Methods A total of 19 miniature pigs were randomly assigned into 3 groups.Three cases were assigned as the control group; ten cases were assigned as stent implantation group A with one stent implanted in the pig pulmonary artery; the other six cases were assigned as stent implantation group B with two stents implanted in the pig pulmonary artery.Follow-up studies for 24 months were conducted in all cases.Repeated measures ANOVA were used to collect and analyze statistical processing data at multiple time points of the follow-up.Results After surgery,pulmonary artery blood flow velocity and right ventricular pressure were measured by echocardiography and cardiac catheterization.There was no significant difference in pulmonary artery blood flow velocity (Fgroup×time =1.06,P =0.40) and right ventricular pressure (Fgroup ×time =0.58,P =0.86) among each group,and no vascular renarrow circumstance was indicated.Pulmonary artery diameter expansion rate between stent group and control group was statistically different (Fgroup =3.57,P =0.05 ; Ftime =12.89,P ＜ 0.001 ; Fgroup ×time =2.99,P =0.03),suggesting that the stent could maintain a good expansion of the role of vascular in the follow-up period even though in degradation.Difference in the level of serum iron concentrations among the groups was not statistically significant (Fgroup ×time =0.94,P =0.52),suggesting that there was no evidence of iron overload.All animals survived to the follow-up endpoint,and no serious side effects caused by stent implantation were found.Conclusions Bioabsorbable iron-based stent planted in the pig pulmonary artery may experience a certain degree of degradation,and it is safe and stabile in animals.

The clinical experience of childhood cardiomyopathy caused by inborn errors of metabolism in 11 cases / 临床儿科杂志

Objectives To summarize the diagnosis and treatment of cardiomyopathy caused by inborn errors of metabo-lism (IEM). Methods The retrospective study included 11 cases diagnosed as metabolic cardiomyopathy through tandem mass spectrometry, activity of serum enzyme, detection of urine mucopolysaccharide and gene analysis from 2012 to 2013. Six cases were diagnosed as primary carnitine deficiency (PCD). Four cases were diagnosed as glycogen storage disease (GSD) and only 1 case was diagnosed as mucopolysaccharidosis. Six PCD cases received carnitine supplementation and anti-heart failure thera-py and received follow-up for 2-10 months. Other 5 cases received supportive treatment and follow-up. Results Patients with PCD recovered soon after treatment but other 5 cases have died within 5 months. Conclusion IEM is an important cause of chil-dren cardiomyopathy which varied in clinical manifestation, diagnosis, treatment and prognosis of different kinds of metabolic cardiomyopathy. Early diagnosis and treatment could be lifesaving for cardiomyopathy caused by IEM.

Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the mutation and background of SLC22A5 in 6 patients with primary carnitine deficiency (PCD) who only presented as cardiomyopathy.</p><p><b>METHOD</b>Genomic DNA were abstracted from the blood of the patients and their parents. Using high-throughput sequencing to determine the mutation site.Using Sanger method to confirm the mutated alleles in PCD patients and detect the corresponding sequences in their patients. Using SIFT and PolyPhen to predict the function of protein for detected missense mutations.</p><p><b>RESULT</b>Three different mutations were identified, including 2 nonsense mutations (R254X and R289X), 1 missense mutation (C113Y), R254X was the most frequently seen mutation. Four patients had compound heterozygous mutations and 2 patients had homozygous mutations. Their parents were found to have heterozygous mutations in corresponding alleles.</p><p><b>CONCLUSION</b>R254X, R289X and C113Y might be associated with primary carnitine deficiency.</p>

Efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty (RVP-PBAV) for congenital aortic stenosis (AS).</p><p><b>METHOD</b>A total of sixteen children with AS accepted the treatment with RRVP-PBAV. The patients were at ages 6 months to 15 years, their median age was 5.4 years. Their body weight was between 8.5 and 59.0 kg, average (22.3 ± 16.5) kg. The gradient pressure across the aortic valve was measured for all the patients and aortic regurgitation was observed. The follow-up time ranged from 1 month to 5.5 years.</p><p><b>RESULT</b>All patients underwent RVP-PBAV successfully. The ratios of balloon/valve were 0.86 to 1.12. The gradient pressure varied from preoperative Δp = (96 ± 32) mmHg (1 mmHg = 0.133 kPa) to the immediate postoperative ΔP = (41 ± 26) mmHg, (P < 0.05). One case had postoperative restenosis, and 3 cases were complicated with bicuspid aortic valve deformity.</p><p><b>CONCLUSION</b>The treatment with RVP-PBAV for congenital aortic stenosis is safe and reliable. Rapid ventricular pacing is a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and may decrease the incidence of aortic insufficiency.</p>

A new homemade secundum atrial septal defect closure device / 中国介入心脏病学杂志

Objective To evaluate the feasibility, safety and efficacy of a new homemade device for percutaneous closure of secundum atrial septal defects (ASDs) Methods Percutaneous closure of surgically created fossa ovalis ASD was attempted in 8 piglets The device was braided from Nitinol wires in the shape of two flat discs with a short connecting waist with a diameter corresponding to that of the defect to be closed Polyethylene filling was implanted to enhance thrombogenicity Pulmonary arteriography with levo phase was obtained before closure and immediately after closure Transthoracic echocardiography with color Doppler was used in all animals during closure and in follow up examinations The animals were killed at various intervals up to 6 months after occlusion for microscopic examination Results Successful placement of the device was achieved in all the 8 animals All ASDs were completely occluded in 1 month, observed by transthoracic echocardiography Postmortem gross and microscopic examination of the 8 specimens 1 to 6 months after placement showed complete or near complete fibrous neoendocardium coverage of both the right and left atrial discs Conclusion These observations show the feasibility of occlusion of secundum ASDs by the homemade device with the advantages of easy placement and repositionability in piglets