Hypertrophic cardiomyopathy associated with mitochondrial disease in children / 中国实用儿科杂志

Mitochondrial disease is a heterogeneous group of hereditary diseases caused by the defects in the mitochondrial respiratory chain and abnormal cellular energy metabolism.Heart is one of the most common organs involved,and hypertrophic cardiomyopathy is the most common and important type of cardiac involvement in mitochondrial disease. Hypertrophic cardiomyopathy in the patients with mitochondrial disease with childhood onset is more common than those with adulthood onset. Mortality in children with cardiac involvement caused by mitochondrial disease is significantly higher than that in children without cardiac involvement,so the early diagnosis and treatment is very important. But the early diagnosis is still difficult due to the complexity of clinical manifestations of mitochondrial disease. There is no specific treatment for mitochondrial disease and its associated hypertrophic cardiomyopathy,so supportive therapy is still the main treatment.

Operation practice of residency program management in pediatric standardized residency training specialty base / 中国实用儿科杂志

Strengthening the organization and administration of standardized residency training is essential to ensuring the function and quality of residency training. On the level of secondary discipline of pediatrics,a residency program committee should be established including program director,site coordinators and resident representatives,and so on.The program committee is responsible for the resident admission,training,evaluation and program quality control.Through implementing program director responsibility system,regular meeting of residency program committee and regular seminars,the program management in pediatric standardized residency training specialty base is carried out,so as to ensure the smooth progress of residency training,effectively realizing the clinical teaching professionalization,and ensuring the quality of standardized residency training.

Gut microbiota analysis and its significance in vasovagal syncope in children / 中华医学杂志(英文版)

BACKGROUND@#Vasovagal syncope (VVS) is common in children and greatly affect both physical and mental health. But the mechanisms have not been completely explained. This study was designed to analyze the gut microbiota in children with VVS and explore its clinical significance.@*METHODS@#Fecal samples from 20 VVS children and 20 matched controls were collected, and the microbiota were analyzed by 16S rRNA gene sequencing. The diversity and microbiota compositions of the VVS cases and controls were compared with the independent sample t test or Mann-Whitney U test. The correlation between the predominant bacteria and clinical symptoms was analyzed using Pearson or Spearman correlation test.@*RESULTS@#No significant differences in diversity were evident between VVS and controls (P > 0.05). At the family level, the relative abundance of Ruminococcaceae was significantly higher in VVS children than in controls (median [Q1, Q3]: 22.10% [16.89%, 27.36%] vs. 13.92% [10.31%, 20.18%], Z = -2.40, P  4, P < 0.05). The relative abundance of Ruminococcaceae in VVS patients was positively correlated with the frequency of syncope (r = 0.616, P < 0.01). In terms of its correlation with hemodynamics, we showed that relative abundance of Ruminococcaceae was negatively correlated with the systolic and diastolic pressure reduction at the positive response in head-up tilt test (HUTT; r = -0.489 and -0.448, all P < 0.05), but was positively correlated with the mean pressure drop and decline rate (r = 0.489 and 0.467, all P < 0.05) as well as diastolic pressure drop and decline rate at the HUTT positive response (r = 0.579 and 0.589, all P < 0.01) in VVS patients.@*CONCLUSION@#Ruminococcaceae was the predominant gut bacteria and was associated with the clinical symptoms and hemodynamics of VVS, suggesting that gut microbiota might be involved in the development of VVS.

Clinical analysis and follow-up study of cardiavascular system involvement in 10 children with methylmalonic aciduria combined with hyperhomocysteinemia / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the clinical features and treatment outcomes of cardiovascular system involvement in children with methylmalonic aciduria combined with hyperhomocysteinemia (MMACHC).</p><p><b>METHODS</b>The clinical data of 10 children with methylmalonic aciduria combined with hyperhomocysteinemia and who had cardiovascular system involvement were retrospectively analyzed and the treatment outcomes were followed up.</p><p><b>RESULTS</b>In the 10 patients, there were 4 cases with initial presentations of cardiovascular system symptoms such as shortness of breath and dyspnea, 3 cases with urinary tract symptoms such as edema, hematuria and proteinuria, and 3 cases with nervous system symptoms such as developmental retardation and convulsions. The 10 patients had different types and severity of cardiovascular injuries. After 3 months to 8 years of follow-up, the congenital heart defects resolved naturally in 2 cases, and the patient with arrhythmia had no obvious changes. In 5 cases of hypertension, blood pressures recovered to normal in 3 cases, and 1 case was lost to follow-up. In 5 patients with pulmonary hypertension, 2 died, 2 recovered, and 1 case had mildly elevated pulmonary artery pressure. Seven patients underwent MMACHC gene testing, and 5 showed c.80A>G mutations.</p><p><b>CONCLUSIONS</b>Metabolic disease should be taken into account for the children with unexplained pulmonary hypertension and hypertension with the onset of the shortness of breath and dyspnea. The severity of cardiovascular system involvement might be one of the most important factors affecting the prognosis of children with MMACHC. Cardiavascular system involvement of the patients may be related to MMACHC c.80A>G mutations.</p>

A family with autosomal dominant Emery-Dreifuss muscular dystrophy and literature review / 中华实用儿科临床杂志

Objective To analyze the clinical characteristics,muscle pathological features and pathogenic gene mutation of a family with autosomal dominant Emery-Dreifuss muscular dystrophy (AD-EDMD).Methods Clinical data of the proband and her family members,a Chinese family of AD-EDMD,were collected.Skeletal muscle specimens were collected from the proband for pathological analysis.Genomic DNA from the proband and her parents was extracted using standard procedures from the peripheral blood leukocytes.PCR and DNA direct sequencing were employed to analyze all of the 12 exons of the LMNA gene to determine the gene mutation,and the case was summarized along with related literature review.Results The proband,female,4 years and 5 months old now,presented with muscle weakness during her early childhood,the proximally was more prominent,mild pectus excavatum.Her CK level was elevated,her electromyogram showed myogenic injuries,the muscle biopsy showed myopathy changes.Her father had the same symptom,with disease progressed,showed elbow contractures in early stage,stiff neck,tight achilles tendon,slowly progressive muscle weakness of the limbs,sinus bradycardia.A heterozygous missense mutation c.1580G ＞ C (p.Arg527Pro) was identified in exon 9 of the LMNA gene in the proband and her father,but not in her mother.This heterozygous missense mutation had been reported as a pathogenic gene mutation.Conclusions The patient who has elbow contractures in early stage,limited neck flexion,spine stiffness,muscle weakness with the proximal upper limbs and distal lower limbs,and arrhythmia,should have an analysis of the LMNA gene.It's important for the early diagnosis of EDMD,assessment of the prognosis,timely and effectively monitoring the changes of arrhythmia,then taking interventions to improve the quality of life and prolong life.So genetic analysis is most reliable method to diagnose EDMD.

Adrenomedullin alleviates collagen accumulation in pulmonary arteries of rats with hypoxic pulmonary hypertension / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To observe the effect of adrenomedullin (ADM) on the pulmonary vascular collagen metabolism in hypoxic rats in order to study the effect of ADM on chronic hypoxic pulmonary vascular structural remodeling and its possible mechanism.</p><p><b>METHODS</b>Nineteen male Wistar rats were randomly divided into three groups: normal control (n=6), hypoxia (n=7) and ADM-treated hypoxia (n=6). ADM was subcutaneously administered into rats of the ADM-treated hypoxia group by mini-osmotic pump (300 ng/h) for two weeks. After two weeks of hypoxic challenge, mean pulmonary arterial pressure (mPAP) was evaluated using a right cardiac catheterization procedure. The ratio of right ventricular mass to left ventricular plus septal mass[RV/ (LV+S)] was measured. The changes of pulmonary vascular microstructure were observed. Meanwhile, the expression levels of collagen I, collagen III and transforming growth factor (TGF)-β in pulmonary arteries were detected by immunohistochemical assay.</p><p><b>RESULTS</b>mPAP and RV/(LV+S) increased significantly in the hypoxia group compared with normal controls (P<0.01). The muscularization of small pulmonary vessels and the relative medial thickness of pulmonary arteries increased obviously in the hypoxia group compared with those in the normal control group (P<0.01). Meanwhile, the expression levels of collagen I, collagen III and TGF-β of pulmonary arteries in the hypoxia group increased markedly compared with those in the normal control group. However, mPAP and RV/(LV+S) were significantly reduced in the ADM-treated hypoxia group compared with those in the hypoxia group (P<0.01). ADM ameliorated pulmonary vascular structural remodeling of hypoxic rats, with a decrease in the expression of collagen I, collagen III and TGF-β of pulmonary arteries.</p><p><b>CONCLUSIONS</b>ADM might play a regulatory role in the development of hypoxic pulmonary hypertension and hypoxic pulmonary vascular remodeling, through inhibiting the expression of TGF-β and alleviating the collagen accumulation of pulmonary arteries.</p>

Clinical characteristics and follow-up study of tachycardia-induced cardiomyopathy in 12 children / 中华儿科杂志

<p><b>OBJECTIVE</b>Tachycardia induced cardiomyopathy (TIC), secondary to various tachyarrhythmias, is a reversible condition which can lead to cardiac enlargement and heart failure. The impairment of both structure and function of heart can be reverted completely or partially if tachyarrhythmias are ceased without delay. This study aimed to explore the clinical characteristics, therapeutic regimen and outcome of TIC in children.</p><p><b>METHODS</b>Clinical data of 12 children with TIC, who came from Peking University First Hospital from Feb. 2003 to Jun. 2009, were retrospectively analyzed and followed up. The echocardiogram data on admission were compared with those from 12 homochronous cases with idiopathic dilated cardiomyopathy matched with 12 TIC cases in age and gender.</p><p><b>RESULTS</b>Atrial tachycardia is the commonest arrhythmia in 12 TIC cases (75%). Four cases underwent catheterization for radiofrequency ablation and all succeeded. The cardiac rhythm of 6 out of 8 cases treated with drugs became sinus rhythm after 3 days to 2 weeks antiarrhythmic drugs treatment. The remaining 2 cases still retained atrial rhythm, but the ventricular heart rates declined to normal. The left ventricular end-diastolic dimensions of the 12 cases were decreased compared with those of pretherapy [(37.5 ± 5.3) mm vs. (43.0 ± 5.7) mm, P < 0.01], and the left ventricular ejection fractions were increased [(60.5% ± 5.6%) vs. (33.7% ± 10.3%), P < 0.01], after (3.4 ± 2.3) months. In our (4.3 ± 2.4) year-follow-up, all cases were fine, except in one case the tachyarrhythmia relapsed because of discontinuation of the drug treatment by her parents. The left ventricular end-diastolic dimensions in 12 TIC cases were smaller than those of the 12 age- and gender-matched idiopathic dilated cardiomyopathy [(43.0 ± 5.7) mm vs. (54.8 ± 7.5) mm, t = 7.9, P < 0.01], and the ejection fractions were higher [(33.7% ± 10.3%) vs. (21.8% ± 7.5%), t = 3.7, P < 0.01].</p><p><b>CONCLUSION</b>The diagnosis of TIC should be considered for the children with tachycardia, cardiac enlargement and cardiac insufficiency. The degree of cardiac enlargement and cardiac insufficiency might be of value for the differential diagnosis between TIC and idiopathic dilated cardiomyopathy. The rhythm control and ventricular rates control could all result in a favorite therapeutic efficacy.</p>

Clinical characteristics of cardiac syncope in children / 中华儿科杂志

<p><b>OBJECTIVES</b>To explore the clinical characteristics of cardiac syncope (CS) in children, and understand their significance in predicting the cardiac syncope.</p><p><b>METHODS</b>Twenty-three patients were referred to our department for evaluation of syncope. The diagnosis of the above cases was cardiac syncope. Each patient was interviewed using a standard questionnaire. The clinical histories and standard baseline electrocardiogram were analyzed to identify the variables contributing to the diagnosis of CS in children.</p><p><b>RESULTS</b>A cardiac cause was identified in 23 syncopal patients presenting to the Department of Pediatrics, Peking University First Hospital: sick sinus syndrome in 7, congenital long QT syndrome in 4, third degree atrioventricular block in 2, supraventricular tachycardia in 2, ventricular tachycardia in 1, atrial fibrillation in 1, pacemaker dysfunction in 1, idiopathic pulmonary hypertension in 3, hypertrophic cardiomyopathy in 1, and dilated cardiomyopathy in 1. The average age of CS patients was 9 years. In totally 23 patients, exertion related syncope spells were found in 14 cases (60.9%), syncope spells at various position 7/23 (30.4%), absence of prodromes in 12/23 (52.2%), syncope spells with incontinence in 4/23 (17.4%), history of heart disease in 4/23 (17.4%). Abnormal standard baseline electrocardiogram was found in 21 cases (91.7%).</p><p><b>CONCLUSIONS</b>The children with cardiac syncope have overt clinical features, especially abnormal findings in electrocardiogram and exertion related syncope spells are the most common clinical features.</p>

Clinical characteristics and therapy of severe Mycoplasma pneumonia in children / 中国当代儿科杂志

<p><b>OBJECTIVE</b>To study the clinical characteristics, therapeutic regimen and outcome of severe Mycoplasma pneumonia (MP) in children.</p><p><b>METHODS</b>Clinical data of 79 children with MP, including 69 mild and 10 severe cases, were retrospectively analyzed. The 10 children with severe MP were followed-up.</p><p><b>RESULTS</b>In severe MP cases, the fever duration prior to hospitalization and the total fever duration were more prolonged, peripheral blood leucocytes counts, C-reactive protein and erythrocyte sedimentation rate increased, and serum IgM and IgE levels increased as compared to mild MP cases. Of the 10 cases of severe MP, 4 manifested as pulmonary consolidation, 4 as pulmonary consolidation complicated by moderate to large pleural effusion and 2 as progressively worsening pulmonary radiographic findings. Nine severe MP cases were administered with glucocorticoid as well as antibiotics, and the therapeutic effect was satisfactory. In the convalescence stage, bronchofiberoscope lavages were used in 5 severe cases because of persistent pulmonary consolidation.</p><p><b>CONCLUSIONS</b>Severe MP was characterized by rapid progression, pulmonary consolidation, moderate to severe pleural effusion, obviously increased inflammatory indexes, and poor therapeutic reaction to simple macrolide antibiotics. Besides antibiotics, glucocorticoid should be used for severe MP cases as soon as possible. For severe cases with persistent pulmonary consolidation, bronchofiberoscope lavages are recommended.</p>

Imbalance of endogenous homocysteine and hydrogen sulfide metabolic pathway in essential hypertensive children / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Hypertension is a common disease of the cardiovascular system. So far, the pathogenesis of primary hypertension remains unclear. The elaboration of its pathogenesis is an important topic in the field which calls for urgent resolution. The aim of this study was to probe into the metabolic imbalance of homocysteine (Hcy) and hydrogen sulfide (H(2)S) in children with essential hypertension, and its significance in the pathogenesis of essential hypertension.</p><p><b>METHODS</b>Twenty-five children with essential hypertension and 30 healthy children with normal blood pressure were enrolled in the study. The medical history was investigated and a physical examination was conducted on the subjects. Plasma Hcy content was examined by fluorescence polarization immunoassay (FPIA). The plasma H(2)S level was detected by a modified method with a sulfide electrode. Data were presented as mean +/- standard deviation. The t test was applied to the mean values of both groups. Pearson linear correlation analysis was applied to the plasma Hcy and H(2)S as well as to the systolic pressure against the plasma H(2)S/Hcy ratio.</p><p><b>RESULTS</b>Plasma Hcy, an intermittent metabolite of the endogenous methionine pathway, was markedly increased but plasma H(2)S, a final product of this pathway was significantly decreased in hypertensive cases when compared with normal subjects ((Hcy: (12.68 +/- 9.69) micromol/L vs (6.62 +/- 4.79) micromol/L (t = 2.996, P < 0.01); H(2)S: (51.93 +/- 6.01) micromol/L vs (65.70 +/- 5.50) micromol/L) (t = -8.670, P < 0.01)). The ratio of plasma H(2)S/Hcy in children with hypertension was 5.83 +/- 2.91, while that of the control group was 11.60 +/- 3.30, and the difference is significant with a t = -6.610 and P < 0.01. A negative correlation existed between plasma Hcy and H(2)S concentrations, r = -0.379, P < 0.05. And a negative correlation was found between systolic blood pressure and the plasma H(2)S/Hcy ratio, r = -0.687, P < 0.05.</p><p><b>CONCLUSION</b>There was a metabolic imbalance of homocysteine and hydrogen sulfide in essential hypertensive children.</p>

Alterations of proadrenomedullin N-terminal 20-peptide in rats with pulmonary hypertension induced by high pulmonary blood flow / 中国当代儿科杂志

<p><b>OBJECTIVE</b>The mechanism of high pulmonary blood flow-induced pulmonary hypertension remains unclear. The aim of this study was to explore the effect of proadrenomedullin N-terminal 20-peptide (PAMP) on pulmonary hypertension, through examining the alterations of pulmonary PAMP expression and plasma PAMP concentration in rats with pulmonary hypertension induced by high pulmonary blood flow.</p><p><b>METHODS</b>Sixteen male Sprague-Dawley rats were randomly divided into control (n=8) and shunt groups (n=8). Aortocaval shunting was produced in the shunt group. After 11 weeks of shunting, systolic pulmonary artery pressure (sPAP), diastolic pulmonary artery pressure (dPAP) and mean pulmonary artery pressure (mPAP) were evaluated by using a right cardiac catheterization procedure. The ultrastructural changes in intra-acinar pulmonary arteries were observed. The concentration of plasma PAMP was measured by radioimmunoassay. The expression of PAMP in pulmonary arteries was detected by immunohistochemical assay.</p><p><b>RESULTS</b>sPAP, dPAP and mPAP were significantly increased in shunt rats compared with controls (P < 0.01). Ultrastructural changes, such as hyperplasia and swelling of endothelial cells, irregularity of internal elastic laminar, and hypertrophy and increased number of synthetic phenotype of smooth muscle cells, were found in intra-acinar pulmonary muscularized arteries in the shunt group. Plasma PAMP concentration (616 +/- 195 pg /mL vs 427 +/- 90 pg /mL) and PAMP expression in endothelial cells (0.62 +/- 0.09 vs 0.38 +/- 0.12) and in smooth muscle cells (0.24 +/- 0.07 vs 0.14 +/- 0.05) of pulmonary arteries increased significantly in the shut group compared with controls.</p><p><b>CONCLUSIONS</b>The up-regulation of pulmonary and plasm PAMP expression might be involved in the development of high pulmonary blood flow-induced pulmonary hypertension.</p>

Regulation of endogenous cystathionine-gamma-lyase gene expression in high pulmonary flow by nitric oxide precursor / 中国应用生理学杂志

<p><b>AIM</b>Pulmonary hypertension is a common complication of congenital heart disease with a left-to right shunt. The mechanism of pulmonary hypertension induced by high pulmonary blood flow is still not fully understood. Recent studies showed that hydrogen sulfide (H2S) could relax vascular smooth muscle cells. But the change of the system of H2S in pulmonary hypertension induced by high pulmonary blood flow was not reported. We studied the influence on expression of CSE mRNA and production of hydrogen sulfide in rat lung tissues by L-Arginine, in order to demonstrate a regulating role of nitric oxide (NO) in the regulation of cystathionine-gamma-lyase/hydrogen sulfide system (CSE/H2S).</p><p><b>METHODS</b>Thirty male SD rats were randomly divided into shunting group, shunting with L-Arginine group, and control group. Abdominal aorta and inferior vena cava shunting was produced in rats of the later group. Pulmonary artery mean pressure (mPAP) and the hypertrophy of right ventricle of each rat were analyzed. The expression of lung tissue CSE mRNA was measured using quantitative reverse transcription-polymerase chain reaction and in situ hybridization. The activity of CSE in lung tissue was measured according to chemical analysis.</p><p><b>RESULTS</b>mPAP was significantly increased in shunted rats compared with normal control (P < 0.01), the expression of lung tissue CSE mRNA and the activity of CSE in lung tissue were decreased in shunt group (P < 0.01). However, L-arginine significantly attenuated pulmonary artery pressure, but augmented the expression of lung tissue CSE mRNA as well as the activity of CSE in lung tissue.</p><p><b>CONCLUSION</b>L-Arginine reverses the down-regulation of CSE/H2S system in high pulmonary blood flow-induced pulmonary hypertension.</p>

Case Report of Brucellar Meningitis in Children and Literature Review / 实用儿科临床杂志

Objective To explore the clinical characteristics and therapy of brucellar meningitis in children.Method One case of brucellar meningitis was reported and relevant literatures were reviewed.A 10 years old boy was admitted to hospital with a history of half a month fever and cough and 3 days headache.His family raised sheep,but he denied contacting closely with sheep and eating raw goat's milk and mutton.Results The course of this reported child was subacute.Clinical manifestations included fever,headache and meningeal irritation sign positive.The cerebrospinal fluid(CSF)findings were similar to those of tuberculous meningitis,where WBC count was elevated with predominant lymphocytes,glucose and chloride decreased and protein increased.The serum bacterium burgeri antibody was positive,agglutination titer was 1160.After brucellar meningitis was diagnosed,three antibiotics including rifampin,minocycline and ceftriaxone sodium and other symptomatic treatment were used.The CSF findings were normal 2 weeks later.This child taked orally minocycline,rifampin and trimethoprim-sulfamethoxazole for 8 weeks post discharge,the recovery was good.The domestic and foreign documents showed that the incidence of the nervous system complications of brucellosis in children was lower than that in adult.The main clinical manifestations were acute meningitis /meningocephalitis.Brucellar meningitis could be misdiagnosed as tuberculous meningitis,because the course of disease,clinical manifestations and CSF findings were similar.Therapeutic alliance including 3 kinds of antibiotics,which could easily pass through blood-brain barrier,was used to treat brucellar meningitis.The total course of treatment shouldn't be shorter than 8 weeks.Conclusions Brucellar meningitis should be screened through epidemiological investigation,serum agglutination test and cerebrospinal fluid culture,in case the reason of meningitis of meningoencephalitis is unknown,especially its clinical manifestation resembles to tuberculous meningitis.

Effect of Adrenomedullin Subcutaneously Administered by Mini-Osmotic Pump on Hypoxic Pulmonary Hypertension in Rats / 实用儿科临床杂志

Objective To study the modulation effect of adrenomedullin (ADM) on hypoxia pulmonary hypertension in rats.Methods Twenty-four male Wistar rats were randomly divided into control group ( n =8), hypoxic group ( n =8), hypoxic with ADM group ( n =8). ADM was subcutaneously administered into rats of hypoxic with ADM group by mini-osmotic pump (300 ng/h). After two weeks hypoxic challenge, systolic pulmonary arterial pressure (sPAP) and mean pulmonary arterial pressure (mPAP) were evaluated by a right cardiac catheterization procedure. Mean systemic artery pressure (mSAP) was measured. The ratio of right ventricular mass to left ventricular plus septal mass [RV/(LV+S)] was detected.Results sPAP, mPAP and RV/(LV+S) significantly increased in hypoxic rats compared with controls ( P

Changes Hydrogen Sulfide in Plasma of Children with Congenital Heart Disease / 实用儿科临床杂志

Objective To investigate the changes of hydrogen sulfide (H_2S) level in plasma in order to explore the role of H_2S in the development of pulmonary hypertension (PH) secondary to congenital heart disease (CHD).Methods There were 9 CHD patients and 9 normal children in this study. The plasma concentration of H_2S and pulmonary artery pressure (PAP) of each child were measured. Meanwhile, the relationship between H_2S level and PAP was analyzed.Results The plasma level of H_2S in the group of CHD significantly decreased compared with control group (32.13?2.25) ?mol/L vs [(43.69?2.05)?mol/L, P

Endogenous nitric oxide pathway in high pulmonary blood flow-induced pulmonary vascular structural remodeling / 中华儿科杂志

<p><b>OBJECTIVE</b>Pulmonary vascular structural remodeling induced by high pulmonary blood flow is an important pathologic basis of pulmonary hypertension with congenital heart disease of left-to-right shunt. However, the mechanism is still not clear. The present study aimed to examine the alteration of endogenous nitric oxide (NO) pathway in high pulmonary blood flow-induced pulmonary vascular structural remodeling, so as to explore the role of NO pathway in pulmonary hypertension induced by high pulmonary blood flow.</p><p><b>METHODS</b>Sixteen male SD rats were randomly divided into control group (n = 8) and shunting group (n = 8). Aortocaval shunting was produced for 11 weeks in shunt rats. Pulmonary artery mean pressure (mPAP) of each rat was evaluated using right cardiac catheterization. The ratio of right ventricular mass to left ventricular plus septal mass [RV/(LV + S)] was detected. Pulmonary vascular micro-and ultra-structure was examined by using a light microscope and a transmitted electronic microscope. Meanwhile, the concentration of plasma NO was measured by spectrophotometry. The expressions of endothelial NO synthase (eNOS) mRNA and protein by pulmonary arteries were detected by in situ hybridization and immunohistochemistry, respectively.</p><p><b>RESULTS</b>After 11-week aortocaval shunting, mPAP was significantly increased [(22.5 +/- 2.6) mmHg vs. (15.8 +/- 2.8) mmHg, 1 mmHg = 0.133 kPa, t = 4.97, P < 0.01], and RV/(LV + S) was also markedly increased (0.267 +/- 0.022 vs. 0.221 +/- 0.016, t = 4.85, P < 0.01). The percentage of muscularized arteries was obviously increased in shunt rats compared with controls [(23.2 +/- 2.4)% vs. (13.5 +/- 2.1)%, t = 7.82, P < 0.01], and relative medial thickness of pulmonary arteries was obviously increased in shunt rats [median pulmonary artery: (7.76 +/- 0.56)% vs. (4.82 +/- 1.03)%, t = 6.23, P < 0.01; small pulmonary artery: (11.94 +/- 0.66)% vs. (6.91 +/- 0.53)%, t = 14.96, P < 0.01]. Ultrastructural changes, such as hyperplasia and degeneration of endothelial cells, irregularity of internal elastic laminar and hypertrophy and the increased number of synthetic phenotype of smooth muscle cells, were found in intrapulmonary arteries of shunt rats. Meanwhile, plasma NO concentration was increased [(30.2 +/- 7.9) micromol/L vs (19.7 +/- 5.7) micromol/L, t = 3.05, P < 0.01) and eNOS mRNA and protein expressions by pulmonary arteries were significantly augmented in rats of shunting group.</p><p><b>CONCLUSION</b>The upregulation of eNOS/NO might be an adaptive response of pulmonary circulation to an increased blood flow in the development of pulmonary hypertension and pulmonary vascular structural remodeling.</p>

Impact of high pulmonary blood flow on pulmonary vascular structure and human urotensin II in intrapulmonary arteries of rats / 中国应用生理学杂志

<p><b>AIM</b>To study human urotensin II (hUII) expression in intrapulmonary arteries of rats with pulmonary hypertension induced by high pulmonary blood flow and explore the role of hU II in the development of pulmonary hypertension induced by left to right shunt.</p><p><b>METHODS</b>Aortocaval shunting was produced for 11 weeks in rats. Pulmonary artery mean pressure (PAMP) of each rat was evaluated using right cardiac catheterization. The pulmonary vascular structural changes, including the percentage of muscularized arteries of small pulmonary vessels and relative medial thickness of intra-acinar pulmonary arteries were examined. Meanwhile, the expression of hU II by pulmonary arteries was detected by immunohistochemistry.</p><p><b>RESULTS</b>After 11-week aortocaval shunting, PAMP was significantly increased. The percentage of muscularized arteries of small pulmonary vessels and relative medial thickness of pulmonary arteries were obviously increased in shunting rats compared with controls (P < 0.01, respectively). Meanwhile, hU II expression by pulmonary artery endothelial cells and smooth muscle cells was significantly augmented in rats of shunt group, which was positively correlated with PAMP and the structural changes in pulmonary arteries.</p><p><b>CONCLUSION</b>The up-regulation of hU II in pulmonary arteries might be involved in the development of pulmonary vascular structural remodeling and pulmonary hypertension induced by high pulmonary blood flow.</p>

Effect of Hyperlipidemia on Carotid Artery Wall Elasticity in Children / 实用儿科临床杂志

Objective To investigate the factors influencing the wall elasticity of carotid artery and the effect of hyperlipidemia on carotid artery elasticity in children.Methods Fifty-seven healthy children and 23 children with hyperlipidemia matched for age and sex were involved in the study.The pressure-strain elastic modulus(E?),stiffness parameter(?) and arterial compliance(AC) of carotid artery were measured by Aloka echo-tracking system.Correlations between age,blood pressure and E?,?,AC were analyzed in healthy children,respectively.Results In healthy children,age,systolic blood pressure and pulse pressure were positively correlated with E? and ?,and negatively correlated with AC.E? and ? significantly increased in children with hyperlipidemia compared to those in healthy children,and AC obviously decreased.Conclusions Hyperlipidemia might decrease the wall elasticity of carotid artery in children.Age and blood pressure are also factors affecting carotid artery elasticity in children.J Appl Clin Pediatr,2006,21(3):142-143

Clinical Analysis of 10 Children with Takayasu′s Arteritis / 实用儿科临床杂志

Objective To investigate the clinical features,treatment response and prognosis in children with Takayasu′s arteritis(TA) in order to improve the understanding of TA.Methods A retrospective study of 10 children with TA was performed.All of them were admitted and diagnosed in Peking University First Hospital from Jan.1998 to Oct.2008.The clinical features,laboratory tests,imaging modalities,treatment response and prognosis were all collected and evaluated.Results There were 3 boys and 7 girls in the 10 patients with TA,and the ratio of male to female was 12.3.The onset was from 4 months to 9 years old,with average age at 5.5 years old.The average duration of diagnosis was 7.6 months.The incidences of hypertension,vascular bruits,albuminuria,convulsion were present in 100%,100%,70% and 40%,respectively.The clinical types included typeⅡ(60%),type Ⅲ(10%) and type Ⅳ(30%).The acute phase inflammatory indices of activity such as erythrocyte sedimentation rate(ESR),C-reactive protein(CRP) and white blood cell(WBC) were not evidently increased.Tuberculosis infection was found in 6 out of 10 patients and anti-tuberculosis treatment was performed.Six patients were treated with steroids and 3 cases of them were also given immunosuppressives cyclophosphamide or methotrexate.Three of the 10 patients received anti-hypertensive and vasodilator.Two patients received percutaneous translurminal angioplasty and 1 patient received nephrectomy.One patient died of renal failure,heart failure and shock.Conclusions The patients with TA had high prevalence of tuberculosis infection,diagnosis as often late because of lack of specific clinical features at the acute inflammatory period.When organic ischaemia occurred,treatment response was usually unsatisfactory.Patients with multi-systemic and multi-viscera lesions should have comprehensive examination,especially for those with hypertension,pulseless and vascular bruits,in order to rule out TA.Early ultrasonography,computed tomography and magnetic resonnance image methods are valued in eariler diagnosis and they are the key factors to improve prognosis.

Clinical Analysis of Cardiac Involvement in Children with Mitochondriopathies / 实用儿科临床杂志

Objective To explore the clinical characteristics of cardiac involvement in children with mitochondriopathies.Methods The clinical data of 23 children with mitochondriopathies were reviewed.The changes of electrocardiography,echocardiography and heart enzymes were analyzed.Results In 15 cases of mitochondrial encephalomyopathy,lactic acidosis,and stroke-like episode(MELAS syndrome),electrocardiography was performed on 9 cases,6 of them showed abnormal electrocardiographic findings,including right bundle branch block,ST-T change,Wolff-Parkinson-White syndrome,et al.On echocardiographic examination in 9 MELAS syndrome ca-ses,only 1 case showed hypertrophy cardiomyopathy.Six cases had increased plasma creatine kinaseMB(CK-MB) mass and only one of 12 MELAS syndrome cases had increased cardiac troponin I(cTnI) level.In 8 cases of subacute necrotizing encephalomyopathy(Leigh syndrome),electrocardiography was performed on 5 cases,4 of them showed abnormal electrocardiographic findings,including sinus tachycardia,ST-T change and low voltage.Two cases showed normal electrocardiography.Three out of 6 cases with Leigh syndrome showed increased plasma CK-MB mass.The molecular genetic examinations were performed in 13 cases of MELAS syndrome and 6 cases of Leigh syndrome.The mitochondrial DNA nt 3243 A→G mutation was found in white blood cells of 9 MELAS syndrome cases,the mutation rate being 37%-60%.The mitochondrial DNA nt 8993 T→C mutation was found in white blood cells of 2 Leigh syndrome cases.Conclusion In children with mitochondriopathies,myocardiac involvement is comparatively common,and even cardiomyopathy can occur.