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1.
Article in Chinese | WPRIM | ID: wpr-733156

ABSTRACT

Objective To investigate the characteristics of different electrophysiology of Ca2+-activated Cl-channels (ClCa) in pulmonary arterial smooth muscle cells (PASMCs) between pulmonary arterial hypertension (PAH) rats induced by left-to-right shunt and normotensive rats,and to study its possible role in the progress of PAH induced by high pulmanry blood flow.Methods Forty SD rats were randomly divided into 3 groups:control group (n =10),sham group (n =10),PAH model group (n =20).After molding,the rats were raised in the same condition for 11 weeks.Right ventricular systolic pressure (RVSP) of each rat was measured by right cardiac catheterization procedure.Right ventricular hypertrophy index (RVHI) was calculated.Single PASMC was obtained by acute enzyme separation method and the conventional whole-cell patch clamp technique was used to record the resting membrane potential (Em),ClCa and current density.The Ⅰ-Ⅴ curve between each group were compared.Results Compared with control group and sham group,the Ⅰ-Ⅴ curve about Itail of PAH model group was significantly shift downward; the difference between control group and sham group was not significant.There were positive correlations between Em and RVSP,RVHI (all P < 0.01),and negative correlations between Itail and RVSP,RVHI and Em(all P < 0.01).Conclusions During the formation process of left-to-right shunt induced PAH,with the step up of pulmonary arterial pressure,the Em of PASMCs stepping up.The absolute value of current density of inward ClCa currents was increased,and its Ⅰ-Ⅴ curve was shift downward.These suggest that the change of ClCa currents may play a role in the PAH induced by left-to-right shunting.

2.
Article in Chinese | WPRIM | ID: wpr-733089

ABSTRACT

Objective To conduct a retrospective study on 119 cases and followed-up in order to improve the therapeutic efficacy of congenital heart disease(CHD) with pulmonary arterial hypertension(PAH).Methods Totally 119 cases of CHD with PAH were collected from Jan.2005 to Dec.2010.The information included the clinical data and relevant auxiliary examinations.The ages varied from 1 month to 15 years old,and the average age was (42.4 ± 2.8) months.They were treated with drugs,interventional therapy or surgical treatment.Regular follow-up monitored the symptom,sign and the auxiliary examination including chest X ray and echocardiography.Results There were 66 children who had been treated with cardiac catheterization and 1 case had hemolysis 1 day after the catheterization,but finally all of them were cured.There were 53 cases who received surgical operation and 49 cases were improved.After the operation,1 case became complicated with infection of incision wound,8 cases had upper respiratory infection,8 cases had arrhythmia,and 1 case complicated with pneumonia.Four cases died because of low cardiac output syndrome and pulmonary hypertension articulo.After the treatment,the pulmonary artery pressure came to the scope within from 8 to 90 mm Hg[(20.23 ± 7.13) mm Hg],which showed a significant statistic difference compared to before (t =3.812,P < 0.01),and there were 79 cases (66.4%) whose pulmonary pressure decreased > 40 mm Hg.There were 17 cases of severe PAH,who received surgical operation after the drug treatment.There were 14 cases(82%) whose pulmonary artery pressure decreased > 20 mm Hg.There were 2 cases had low cardiac output syndrome,and 1 case had PAH articulo.After the therapy,the pulmonary arterial pressure,cardiac thoracic ratio,chambers size,the systolic function of left heart were all improved compared with before.Conclusions It is important to diagnose as early as possible the CHD children with PAH,and to choose proper time for interventional or surgical therapy,especially for the moderate and severe cases,so that the cure rate as well as survival quality can be improved.

3.
Chinese Journal of Pediatrics ; (12): 901-904, 2011.
Article in Chinese | WPRIM | ID: wpr-356346

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the electrophysiological changes of voltage-gate potassium channel (Kv) of pulmonary arterial smooth muscle cells of pulmonary arterial hypertension in rats induced by left to right shunt, and to analyze the role of Kv during the progress of pulmonary arterial hypertension.</p><p><b>METHODS</b>Forty male SD rats were randomly divided into three groups, group A (control, n = 10), group B (sham operated only group, n = 10), and group C (PAH model group, n = 20). Mean pulmonary artery pressure (mPAP) and right ventricular hypertrophy index (RVHI) of each rat were measured, single pulmonary artery smooth muscle cell (PASMC) was obtained by acute enzyme separation method (collagenase I plus papain) and the conventional whole-cell patch clamp technique was used to record resting membrane potential (Em), potassium ion current of voltage-gated potassium channel, the I-V curve between each 2 groups was compared, and correlation of each parameter was analyzed.</p><p><b>RESULT</b>(1) The mPAP and RVHI of group C were significantly higher than those of group A and group B (P < 0.01, respectively). (2) The Em of group C [(-33.00 ± 4.09) mV] was significantly higher than that of group A [(-48.10 ± 4.54) mV] and group B [(-51.11 ± 3.66) mV], P < 0.01. (3) The peak current at +50 mV of voltage-gated potassium channel: in group C [(64.80 ± 8.40) pA/pF], it was significantly lower than that of group A [(120.85 ± 11.66) pA/pF] and group B [(118.03 ± 10.18) pA/pF] (P < 0.01, respectively). None of the parameters showed any significant difference between group A and group B (P > 0.05 for all comparisons). (4) Compared with group A and group B, the I-V curve of group C significantly downward shifted (P < 0.01, respectively). The difference in I-V curve between group A and group B was not significant, P > 0.05. (5) The correlation of resting membrane potential and mPAP and RVHI had significantly positive correlation (P < 0.001, respectively); but the correlation of membrane current, membrane current density and mPAP, RVHI and resting membrane potential had significantly negative correlation (P < 0.001, respectively).</p><p><b>CONCLUSION</b>During the formation process of left-to-right shunt induced pulmonary arterial hypertension, function of Kv channel was inhibited, suggesting that Kv channel may be the mechanism of pulmonary arterial hypertension induced by left-to-right shunting.</p>


Subject(s)
Animals , Hypertension, Pulmonary , Metabolism , Male , Muscle, Smooth, Vascular , Metabolism , Patch-Clamp Techniques , Potassium , Metabolism , Potassium Channels, Voltage-Gated , Metabolism , Pulmonary Artery , Metabolism , Rats , Rats, Sprague-Dawley
4.
Chinese Journal of Cardiology ; (12): 409-412, 2009.
Article in Chinese | WPRIM | ID: wpr-294726

ABSTRACT

<p><b>OBJECTIVE</b>To elucidate the association between GATA-4 gene mutations and congenital cardiac septal defects in Han Chinese patients.</p><p><b>METHODS</b>Fifty Han Chinese patients with congenital cardiac septal defects and 100 normal subjects with the same ethnical background were studied. Total six exons and the intron-exon boundaries of GATA-4 were amplified by the polymerase chain reaction. The polymerase chain reaction products were purified and directly sequenced with automatic sequencer.</p><p><b>RESULTS</b>Two novel heterozygous mutations were discovered in the GATA-4 gene of patients with congenital cardiac septal defects, His28Tyr in exon 2 and His436Tyr in exon 7 respectively, which were absent in the control population and not reported in the SNP database (http://www.ncbi.nlm.nih.gov/SNP).</p><p><b>CONCLUSION</b>Our finding suggests that the mutations in the transcription factor GATA-4 may be related to congenital cardiac septal defects in Han Chinese patients.</p>


Subject(s)
Adolescent , Adult , Asians , Ethnology , Child , Child, Preschool , Exons , Female , GATA4 Transcription Factor , Genetics , Genotype , Heart Septal Defects , Ethnology , Genetics , Heterozygote , Humans , Infant , Male , Mutation , Young Adult
5.
Article in Chinese | WPRIM | ID: wpr-287408

ABSTRACT

<p><b>OBJECTIVE</b>To perform the functional analysis of a novel H436Y mutation of GATA-4 gene identified in Han Chinese patients with congenital cardiac septal defects.</p><p><b>METHODS</b>Using bioinformatics to predict if the H436Y mutation in the GATA-4 gene affects its protein function. H436Y mutation in the GATA-4 gene was generated by Quick Change Lightning site-directed mutagenesis kit and verified by DNA sequencing. GATA-4-wt or GATA-4-mut DNA was cotransfected into Hela cells with DNA for the luciferase reporter gene atrial natriuretic factor (ANF), and luciferase activity was measured by an LKB luminometer 48 h after transient transfection.</p><p><b>RESULTS</b>Alignment of the GATA-4 amino acid sequence indicated that the histidine residue at position 436 was conserved, and H436Y mutation in the GATA-4 gene is expected to affect its protein function. The H436Y mutation significantly reduced the transcriptional activation of downstream reporter ANF when compared to wild-type GATA-4 (P<0.01).</p><p><b>CONCLUSION</b>The mutation c.1306C-->T of the GATA-4 gene impaired the activation of the downstream target, suggesting that the H436Y mutation in the C-terminal region of the GATA-4 gene might prevent its biological function.</p>


Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , GATA4 Transcription Factor , Genetics , Heart Septal Defects , Genetics , Humans , Infant , Male , Molecular Sequence Data , Mutagenesis, Site-Directed , Sequence Alignment , Sequence Analysis, DNA , Young Adult
6.
Article in Chinese | WPRIM | ID: wpr-640175

ABSTRACT

950 mL/L for 2 hours.Gross anatomical changes and histological changes(HE staining)of lungs were observed,VEGF expression was detected by immunohistochemical method.Results Two rats in hypothermia-hypoxia group and 4 rats in rewarming-reoxygenating group died while none in control group.Lungs of hypothermia-hypoxia group and rewarming-reoxygenating group represented edema and punctiform,local and diffuse pulmonary hemorrahge.Histopathological changes included pulmonary edema,alveolar septum broken,pulmonary alveoli fusion and pulmonary hemorrahge.More severe pathological change could be found in rewarming-reoxygenating group.Optical density value of VEGF expression in 3 groups were 0.29?0.06,0.36?0.05,0.22?0.05,respectively,there were significant diffe-rences of VEGF expression between 3 groups(F=15.64 P

7.
Article in Chinese | WPRIM | ID: wpr-640018

ABSTRACT

Pulmonary artery hypertension (PAH) is a combination of factors caused abnormal pulmonary hemodynamics.Pulmonary vascular resistance (PVR) progressive as clinical features of PAH.In recent years,studies had shown that pulmonary artery smooth muscle cell membrane potassium channels at the time of PAH and pulmonary vasoconstriction (HPV) and vascular remodeling were closely related to potassium channel in the article on the role of PAH in progress of the study review.

8.
Article in Chinese | WPRIM | ID: wpr-638359

ABSTRACT

Objective To investigate the changes of plasma vasoactive intestinal peptide (VIP) and glucagon (Glu) in congestive heart failure and its clinical significance. Methods The levels of plasma VIP and Glu were measured with radioimmunoassay in 40 patients with congestive heart failure, including 15 patients with cardiac function in degree Ⅰ, 25 cases with cardiac function in degree ⅡⅣ, and 23 normal children who were served as controls. Results The levels of plasma VIP and Glu increased than that in normal control group[(43.22?5 48) ng/L vs(32.24?4.46) ng/L, P

9.
Article in Chinese | WPRIM | ID: wpr-640209

ABSTRACT

Objective To explore the features of clinical manifestations and electrophysiology of spinal muscular atrophy(SMA) in children.Methods The clinical features and laboratory data were analyzed in 32 children with SMA,electromyography tests were carried out in 28 patients and the muscle biopsy were performed in 24 cases.Results The 32 cases were subdivided into 3 clinical groups,15 cases were SMAⅠ,12 cases were SMAⅡ,5 cases were SMAⅢ.They were all characterized by progressive muscle weakness associated with hyptonia and atrophy.The clinical distinction between SMAⅠto SMAⅢ reflected different age of onset and disease severity.All cases of SMAⅠhad symptoms of respiratory disability,only 1 case of SMAⅡ had paradoxical breathing and none of SMAⅢ had similarly symptoms.Electromyographic studies showed a pattern of denervation with no sensory involvement.The rate of spontaneous potential was 87%,with gentle strain,the duration of motor unit was extended(30%-150%) and the amplitude of it was increased(90%-450%),the motor nerve conduction velocity was reduced slightly in 28% patients.The muscle biopsy provided evidence of skeletal muscle denervation with groups of atrophy.Conclusions The clinical features,the changes of electromyography and the muscle biopsy are valuable for diagnosis of SMA.Respiratory management will prolong survival and improve the quality of life for these patients.

10.
Article in Chinese | WPRIM | ID: wpr-639144

ABSTRACT

95 mL/L O2) for 2 hours.The gross anatomical and histological changes(HE staining)in lungs were observed,VEGF mRNA expressions were studied by reverse transcription polymerase chain reaction(RT-PCR).Results Lungs of experimental groups represented edema,inaddition,punctiform,local and diffuse pulmonary hemorrhage were observed in groups of HH,HHR and HHRO2.Histopathological changes included pulmonary alveoli and interstitial edema,spacer breaking,pulmonary alveolidilating,fusion and hemorrhage,in which the most severe cases involved in group HHRO2.VEGF 188 mRNA expression increased significantly in group H and HH(P

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