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Classifying cardiac Anomalies in Right and Left Isomerism:Concordant and Discordant Patterns

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congenital Heart Disease 2023年第1期18卷 97-111页

作者： Lilia Oreto Giuseppe Mandraffino Paolo Ciliberti Teresa P.Santangelo Placido Romeo Antonio Celona Placido Gitto Lorenzo Galletti Fiore S.Iorio Alfredo Di Pino Aurelio Secinaro Paolo Guccione Robert H.Anderson Salvatore Agati Mediterranean Pediatric Cardiology Center Bambino GesùChildren’s HospitalVia SirinaTaorminaItaly Internal Medicine unit Department of Clinical and Experimental MedicineUniversity of MessinaMessinaItaly Department of Pediatric Cardiology and cardiac surgery Bambino GesùChildren’s HospitalRomaItaly Advanced Cardiovascular Imaging unit Department of ImagingBambino GesùChildren’s HospitalRomaItaly Radiology Department S.Vincenzo HospitalTaorminaItaly Biosciences Institute Newcastle UniversityNewcastle upon TyneUK

Aims:Evidence is emerging that,in the setting of isomerism,the atrial and bronchial arrangement are not always concordant,nor are these patterns always harmonious with the arrangement of the abdominal *** aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism,seeking to determine whether it was feasible to assess complexity on this basis,in this regard taking note of the potential value of bronchial as opposed to appendage *** and Results:We studied 78 patients known to have isomerism of the bronchuses,43 with right and 35 with left *** anatomy could be determined in 49 cases(63%),all but one of these being concordant with bronchial *** assessing abdominal features,in only 59 cases(76%)was splenic morphology in keeping with the thoracic *** expected,right isomerism was associated with greater complexity of cardiac malformations,with an odds ratio of 6.53,with confidence intervals from 2.2–19.3(pcardiac *** discordance was found between the systems,however,the cardiac anomalies were less typical of the anticipated findings for right *** isomerism of the appendages.

关键词： Isomerism asplenia polysplenia congenital heart disease bronchial isomerism atrial appendages

Studying the surgical stress in open and laparoscopic treatment of inguinal hernia in children

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Pediatric Investigation 2023年第2期7卷 71-74页

作者： Charikleia D.Demiri Christos Kaselas Stavros Iliadis Maria Tsopozidi Ioannis Spyridakis Second Department of paediatric surgery School of MedicineAristotle University of ThessalonikiThessalonikiGreece Laboratory of Biological Chemistry Department School of MedicineAristotle University of ThessalonikiThessalonikiGreece

congenital INGUINAL HERNIA congenital inguinal hernia is defined as the prolapse of intra-abdominal viscera through the open elytroperitoneal fold due to its failure to *** incidence of inguinal hernia in the pediatric population ranges from 0.8%–5%,while it increases up to 30%in premature newborns.^(1,2)Its treatment is necessary due to the risk of strangulation of the intestine,testicle,or ovary,which is observed in 3%–16%of children presenting this clinical entity.^(1,3)

关键词： hernia intestine treatment

Left atrial dysfunction in elderly patients with patent foramen ovale and atrial septal aneurysm

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Journal of Geriatric Cardiology 2009年第4期6卷 195-198页

作者： Gianluca Rigatelli Fabio Dell＇Avvocata Federico Ronco Massimo Giordan Paolo Cardaioli Section of Adult congenital and Adult Heart Disease Cardiovascular Diagnosis and Endoluminal Interventions Rovigo GeneralHospital Rovigo 45100 Italy

Objective Recently it has been suggested that, in patients with large patent foramen ovale （PFO） and atrial septal aneurysms （ASA）, a certain amount of left atrial （LA） dysfunction may be active as an alternate mechanism promoting arterial embolism. Following this hypothesis, elderly patients, being more susceptible to atrial chambers stiffness, should present a more severe LA dysfunction profile. We sought to evaluate the grade of LA dysfunction in elderly patients submitted to transcatheter PFO closure. Methods We retrospectively enrolled 28 consecutive patients with previous stroke （mean age 674-12.5 years, 18 females） referred to our centre for catheter-based PFO closure after recurrent stroke. Baseline values of LA passive and active emptying, LA conduit function, LA ejection fraction, and spontaneous echocontrast （SEC） in the LA and LA appendage were compared with those of 50 atrial fibrillation patients, as well as a sex/age/cardiac risk matched population of 70 healthy controls. Results Pre-closure elderly subjects demonstrated significantly greater reservoir function as well as passive and active emptying, with reduced conduit function and LA ejection fraction, when compared to healthy and younger patients. After closure in elderly patients, LAparameters did not return completely to the levels of healthy patients, whereas LA dysfunction in younger subjects returned normal. Conclusions This study suggests that elderly patients have more severe LA dysfunction than younger patients, which affects the LA remodelling after closure.

关键词： patent foramen ovale stroke embolism cardiac anatomy echocardiography

Timing of pubertal development in boys born with cryptorchidism and hypospadias:a nation wide cohort study

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Asian Journal of Andrology 2019年第6期21卷 551-556页

作者： Linn Hakonsen Arendt Andreas Ernst Lea Lykke Braskhoj Lauridsen Nis Brix Jorn Olsen Cecilia Host Ramlau-Hansen Department of Public Health Section for EpidemiologyAarhus UniversityAarhus 8000Denmark Perinatal Epidemiology Research unit Department of Paediatric and Adolesce nt Medici neAarhus Un iversity HospitalAarhus 8000Den mark Department of Clin ical Epidemiology Aarhus University HospitalAarhus 8000Denmark.

Pubertal development may be altered in boys with cryptorchidism and hypospadias,but existing kno wledge is inc on siste ***,we investigated the association between cryptorchidism and hypospadias and pubertal development in a large cohort *** in the Puberty Cohort,a cohort nested within the Danish National Birth Cohort,were in eluded in this *** on cryptorchidism and hypospadias was retrieved from the Danish National Patient *** 11 years until 18 years or full pubertal developme nt,info rmatio n on physical markers of pubertal developme nt was provided biannu ally,in eluding Tanner stages,axillary hair,acne,voice break,and first *** multivariate regression models for interval censored data,the mean(95%confidence intervals[Cis])d iff ere nces in months in obtaining the pubertal markers between boys with and without the anomalies were *** 7698 boys,196(2.5%)had cryptorchidism and 60(0.8%)had *** with hypospadias experienced first ejaculation and voice break 7.7(95%Cl:2.5-13.0)months and 4.5(95%Cl:0.3-8.7)months later than boys without hypospadias?The age at attaining the Tanner stages for gonadal and pubic hair growth was also higher,though not statistically *** development seemed unaffected in boys with mild as well as severe cryptorchidism?In conclusion,hypospadias may be associated with delayed pubertal development,but pubertal development seems unaffected by *** relation between hypospadias and later pubertal development may be due to the underlying shared in utero risk or genetic factors.

关键词： congenital abnormalities cryptorchidism hypospadias prenatal exposure delayed effects puberty Tanner stages

Late Presentation of a congenital Intrinsic Duodenal Obstruction in a Patient with Anorectal Malformation

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Surgical Science 2013年第7期4卷 329-331页

作者： Parkash Mandhan Kirtikumar J. Rathod Dilip Sankhla Department of Radiology Sultan Qaboos University Hospital Muscat Oman Division of paediatric surgery Department of Surgery Sultan Qaboos University Hospital Muscat Oman

Anorectal Malformations are known to be associated with various other congenital anomalies including duodenal atresia. An association of congenital intrinsic duodenal obstruction causing partial duodenal obstruction in a patient with anorectal malformation is not described in literature. We describe a case of delayed presentation of congenital intrinsic duodenal obstruction in a child with high-type anorectal malformations causing diagnostic dilemma.

关键词： Anorectal Malformations Duodenal Obstruction VACTERL Association Delayed Presentation Infant

Recent progress of pediatric cardiac surgery in China

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Chinese Medical Journal 2006年第23期119卷 2005-2012页

作者： WU Kai-hong LU Xiao-dong LIU Ying-long Pediatric cardiac Center Department of Surgery CardiovascularInstitute and Fuwai Hospital Chinese Academy of MedicalSciences and Peking Union Medical College Beijing 100037China

Pediatric cardiac surgery in China has madegreat progress since the first successful open heart operation for correction of ventricular septal defect （VSD） with an artificial heart-lung machine in 1958.1 In recent years, the state has paid much attention to both the basic research and the primary, secondary and tertiary prevention of congenital heart disease. The surgical treatment for congenital heart diseases in infancy was listed as one of the Family Planning Technology Specific Projects among the Key Tasks in the Tenth Five-Year Plan. Due to economic and social conditions in China primary or secondary prevention and treatment of congenital heart disease are still in the early phase of development. Therefore, tertiary prevention -- surgical treatment--is still of crucial importance. Here we review the current status of pediatric cardiac surgery in China and highlight the latest advances in this exciting field.

关键词： cardiac surgery cardiopulmonary bypass congenital heart defect pediatrics

Expression of ghrelin and insulin-like growth factor-1 in immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow

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Chinese Medical Journal 2011年第15期124卷 2354-2360页

作者： WANG Dong LIU Ying-long LU Xiao-dong ZHU Yao-bin LING Feng LIU Ai-jun LI Gang XU Yu-lin Department of Pediatric cardiac surgery Center Fuwai Hospital Chinese Academy of Medical Sciences and Peking Union Medical College Beijing 100037 China

Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 （IGF-1） using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow. Methods Twelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction. Results Four weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension （（68.73±15.09） mmHg）, arterial oxygen saturation （（82.35±8.63）%）, and higher arterial carbon dioxide tension （（51.83±6.12） mmHg）, hematocrit （（42.67±3.83）%） and hemoglobin concentration （（138.17±16.73） g/L） than the control piglets （（194.08±98.79） mmHg, （96.43±7.91）%, （36.9±4.73） mmHg, （31.17±3.71）%, （109.83±13.75） g/L）（all P 〈0.05）. Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control （P=0.004）, and the plasma IGF-1 level was significantly lower than control （P=0.030）. Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group （P=0.000）, and the expression of IGF-1 mRNAs was elevated （P=0.001）. Conclusions Chronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma iGF-1 level and myocardial ghrelin mRNA expression

关键词： congenital heart defects cyanosis piglet ghrelin insulin-like growth factor-1

congenital Diaphragmatic Hernia Revealed by an Inaugural Diabetic Ketoacidosis in a 9-Year-Old Child

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Open Journal of Pediatrics 2019年第4期9卷 296-301页

作者： C. Mboutol-Mandavo E. Moyen P. Mawandza G. F. Otiobanda A. Oko I. P. L. Ondima M. Elombila J.-C. Miéret Department of paediatric surgery University Hospital of Brazzaville Brazzaville Republic of the Congo Department of paediatric Reanimation University Hospital of Brazzaville Brazzaville Republic of the Congo Department of Anaesthesiology and Reanimation University Hospital of Brazzaville Brazzaville Republic of the Congo

Introduction: congenital diaphragmatic hernia is often detected during the prenatal or neonatal period by severe respiratory symptoms. Late-presenting congenital diaphragmatic hernia is uncommon entity resulting in frequent misdiagnosis and inappropriate treatment. Case Report: We report the case of a left congenital diaphragmatic hernia revealed by an inaugural diabetic ketoacidosis in a 9-year-old girl. She has presented progressive weight loss without loss of appetite associated with polyuro-polydipsia, then epigastric pain with vomiting. Blood glucose was 3.2 g/L, ketonuria and 2+ glycosuria. Despite a well-conducted treatment, there was persistence of dyspnea. Chest X-ray and chest CT-scan confirmed the presence of a left diaphragmatic hernia. Evolution was marked by the death of the child on day 2 post-operative from a multivisceral failure. Conclusion: Clinical and radiological signs of congenital diaphragmatic hernia after the neonatal period may be difficult to interpret and may result in delayed diagnosis, erroneous treatment and potentially fatal outcome.

关键词： Inaugural Diabetic Ketoacidosis Late-Presenting congenital Diaphrag-matic Hernia Child

Assessment of cardiac function in infants with transposition of the great arteries after surgery: comparison of two methods

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World Journal of Pediatrics 2018年第4期14卷 373-377页

作者： Fan Yang Roberta Iacobelli Jian-Ming Wang Fiore Salvatore Iorio cardiac surgery Department Hebei Province Children's HospitalNo.133Jianhua South StreetShijiazhuang 050031China Pediatric Cardiology Center of Ospedale Pediatrico Bambino Gesù Piazza S.Onofrio 400165 RomeItaly

Background Assessment of cardiac function is crucial in pediatric patients undergoing cardiovascular surgery, monitoring cardiac output and changing hemodynamic conditions during surgery accordingly is important to improve post-surgical outcome. We aimed to measure cardiac index (CI) and maximal rate of the increase of left ventricular pressure dp/dt(max) with the pressure recording analytic method (PRAM, MostCare?) and compared it with transthoracic echocardiographic cardiac index estimation in infants with transposition of the great arteries (TGA) undergoing surgical correction. Methods We enrolled 74 infants with TGA consecutively into this study. CI and dp/dt(max) were measured with PRAM and echocardiography at 0, 4, 8, 12, 24 and 48 h postoperatively. Blood brain natriuretic peptide (BNP) and blood lactate (Lac) were measured at baseline and after operation. Results The median age at surgery was 13 days (range 1–25 days) with an average weight of 3.24 kg (range 2.31–4.17 kg). CI esti-mated by PRAM was 1.11 ± 0.12 L/min/m2 (range 0.69–1.36) and by Doppler echocardiography was 1.13 ± 0.13 L/min/m2 (range 0.76–1.40). dp/dt(max) estimated by PRAM was 1.31 ± 0.03 mmHg/s (range 1.23–1.43) and by Doppler echocardiography was 1.31 ± 0.04 L/min/m2 (range 1.25–1.47). CI (r = 0.817,Pcardiac index and dp/dt(max) value compared with echocardiographic measurements. PRAM through mostcare? is a reliable continuous monitoring method for peri-operative management in children with congenital heart disease.

关键词： cardiac index Doppler echocardiography dp/dt(max) Pressure recording analytic method Transposition of the great arteries