Surgical Care Of Children Research Articles

Interfacility Transfer of Children With Time-Sensitive Surgical Conditions, 2002-2017

Surgical care for children in the United States has become increasingly regionalized among fewer centers over time. The degree to which regionalization may be associated with access to urgent surgical care for time-sensitive conditions is not clear. To investigate whether transfers and travel distance have increased for 4 surgical conditions, and whether changes in transfers and travel distance have been more pronounced for rural vs urban children. This retrospective cross-sectional study analyzed data from 9 State Inpatient Databases from 2002 to 2017. Participants included children aged younger than 18 years undergoing urgent or emergent procedures for malrotation with volvulus, esophageal foreign body, and ovarian and testicular torsion. Residential and hospital zip codes were categorized as rural or urban. Hospitals were categorized as pediatric hospitals, adult hospitals with pediatric services, and adult hospitals without pediatric services. Primary outcomes were transfer for care and travel distance between patients' home residences and the hospitals where care was provided. Transfer and travel distance were analyzed using multivariable regression models. Among the 5865 children younger than 18 years undergoing procedures for malrotation with volvulus, esophageal foreign body, ovarian torsion, or testicular torsion, 461 (7.9%) resided in a rural area; 1097 (20.5%) were Hispanic, 1334 (24.9%) were non-Hispanic Black, and 2255 (42.0%) were non-Hispanic White; 2763 (47.1%) were covered by private insurance and 2535 (43.2%) were covered by Medicaid; and the median (IQR) age was 9 (2-14) years. Most care was provided at adult hospitals (73.4% with and 16.9% without pediatric services); the number of hospitals providing this care decreased from 493 to 292 hospitals (2002 vs 2017). Transfer was associated with rural residence (adjusted odds ratio [aRR], 2.3 [95% CI, 1.8-3.0]; P < .001) and increased over time (2017 vs 2002: aOR, 2.8 [95% CI, 2.0-3.8]; P < .001). Similarly, travel distance was associated with rural residence (adjusted risk ratio [aRR], 4.4 [95% CI, 3.9-4.8]; P < .001) and increased over time (2017 vs 2002: aRR, 1.3 [95% CI, 1.2-1.4]; P < .001). Rural children were more frequently transferred (2017 vs 2002) for esophageal foreign body (48.0% [12 of 25] vs 7.3% [4 of 55]; P < .001), ovarian torsion (26.7% [4 of 15] vs 0% [0 of 18]; P = .01), and testicular torsion (18.2% [2 of 11] vs 0% [0 of 16]; P = .04). Travel distance for rural children increased the most for torsions, from a median (IQR) of 19.1 (2.3-35.4) to 43.0 (21.6-98.8) miles (P = .03) for ovarian torsion and from 7.3 (0.4-23.7) to 44.5 (33.1-48.8) miles (P < .001) for testicular torsion. In this cross-sectional study of children with time-sensitive surgical conditions, the number of hospitals providing urgent surgical care to children decreased over time. Transfers of care, especially among rural children, and travel distance, especially for those with ovarian and testicular torsion, increased over time.

Performance of OpenAI’s GPT-4 in a mock MRCS Part A Examination

Introduction: OpenAI’s latest iteration of a Large Language Model (LLM); GPT-4 (Generative Pre-trained Transformer 4) has demonstrated its proficiency against various professional examination standards like the USMLE (United States Medical Licensing Examination), FRCS (Fellowship of the Royal Colleges of Surgeons) and the United States Bar. However, GPT-4’s capability with the MRCS (Membership of the Royal College of Surgeons) Part A has not yet been investigated. Methodology: A representative MRCS Part A examination that was prepared and provided by “TeachMeSurgery” based on the MRCS Intercollegiate Curriculum was used to assess GPT-4's performance. Each question was processed via the web-based interface of ChatGPT (Chat Generative Pre-trained Transformer) Plus. Results: GPT-4 scored 87.2% on Applied Basic Sciences (157/180) and 86.7% on Principles of Surgery in General (104/120 questions), achieving an overall score of 261/300 (87%), which is above the typical passing threshold. GPT-4 scored 100% in four out of the eleven predefined curriculum areas, which included: Pharmacology, Microbiology, Data Interpretation and Audit, and The Surgical Care of Children. GPT-4’s weakest performance was in the Medico-Legal Aspects of Surgical Practice, in which it scored 33.3%. Conclusion: GPT-4 successfully passed the mock MRCS Part A without any specialised preparatory training, however further research could look at integrating the GPT-4 model to enhance a trainee surgeon’s learning and use it as an effective tool to deliver high quality &amp; efficient patient care.

From intestinal failure to transplantation: Review on the current need for transplant indications under multidisciplinary transplant programs worldwide.

Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. The review is based on the authors' experience supported by an in-depth review of the published literature. The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.

Children's Anaesthesia and perioperative care challenges, and innovations

The 2015 Sustainable Development Goals emphasise good health to all with reduced inequalities, and surgical and anaesthesia care is essential to achieve these. https://sdgs.un.org/goals. However, it has been estimated that 1.7 billion children do not have access to safe anaesthesia and surgery when needed and this disproportionately affects children in low- and middle-income countries (1). It is alarming that 1 in 10 individuals in LMICs do not have access to safe surgical care. Both safe surgery and anaesthesia are essential for ensuring that individuals receive proper medical attention. Economically viable public health initiatives that can avert many disability-adjusted years are needed. (2–4)Morbidity and mortality from surgical disease and anaesthesia care remain high in low-income countries, unlike in high-income countries. The incidence of severe anaesthesia-related critical events and perioperative cardiac arrest is between three and ten times more in LMICs than in HICs (5–7) A baseline POMR that is 100 times higher in LMICs compared to HICs is reported. (8) This perioperative morbidity and mortality gap is more evident in neonates and younger age groups, especially in children with congenital abnormalities. The challenges facing providers of anaesthesia and perioperative care are multifactorial and include but are not limited to the inadequate workforce, inadequate and inappropriate infrastructure, lack of adequate and appropriately sized equipment, including monitors, and safe monitoring capacity, supply chain challenges for medicines and reusable consumables, unreliable supply of oxygen and blood products, lack of data and research for policy formulation, inadequate resource allocation from governments and lack of safety culture among other things. In paediatrics, this is further multiplied by the variability in the sizes of the patients, from neonates to older children (9).1.Improved perioperative care must include anaesthesia and nursing to improve perioperative outcomes for children.2.Perioperative care for children in LMICs is predominantly by non-physician anaesthesia providers or non-specialty-trained anaesthesiologists.3.There is a need to train physician anaesthesia leaders to direct and oversee the care of children undergoing anaesthesia4.Infrastructure and equipment for the safe provision of paediatric anaesthesia/perioperative care are usually wanting/often times inadequate and inappropriate.5.The GICS OReCS document provides a valuable guide for the bare minimum requirements for the provision of safe paediatric anaesthetic and surgical care services, starting at the district hospital.6.Equipment donations should be conscientious, in consultation with local leads. Considerations will include biomedical support for maintenance, availability of spare parts, and electrical compatibility, among others.7.Context-specific innovations have been shown to work in LMICs and include paediatric anaesthesia courses for all providers and simulation-based training using low technology.8.Any worthwhile programs aimed at improving anaesthetic and surgical care for children need to ultimately fit into national healthcare systems.

EPIDEMIOLOGY OF PEDIATRIC SURGICAL CONDITIONS OBSERVED IN A RURAL TERTIARY CARE HOSPITAL

Background: Little is known about the surgical conditions affecting the Pediatric population in developing countries. Data on the spectrum of surgical diseases and the burden of Pediatric surgical diseases on health systems are lacking. In this article we describe the epidemiology of Pediatric surgical diseases observed in our rural tertiary care hospital. Methods: We retrospectively reviewed the records of all children (0–14 years) admitted to the Pediatric ICU from January 2020 to October 2022. We also reviewed the records of all the patients admitted to the Neonatal ICU and among them we selected the ones in which a surgical diagnosis was present. Four hundred fty three children were admitted to Pediatric ICU and four hundred and sixty six babies were admitted to neonatal ICU during the study period. Results: 453 children were admitted to pediatric ICU of which 183 were surgical cases and 466 babies were admitted to NICU of which 2 were surgical cases. Males were 122 (66%) and females were 63 (34%). Largest number 114 (62%) of patients were in the age group 0-5 years followed by 6-10 years age group 53 (28%).The highest number of admissions were for the OMFS; cleft lip 44(24%), cleft palate 25(14%), followed by genitourinary system which included hydrocele 11(6%) and inguinal hernia 11(6%), undescended testis 6(3%), hypospadias 5(2.7%) followed by Neurosurgery cases which included hydrocephalus 11(6%), and subdural hematoma 2(1%). GI conditions were acute appendicitis with appendicular perforation 10(5.4%), cholelithiasis 3(1.6%) and small bowel obstruction 2(1%) Conclusion: Surgical conditions are important part of pediatric and neonatal care.Management of congenital surgical condition is important to decrease infant and under ve mortality and other comorbidities as well. In developing countries, surgical care for children should be considered an important part of child health programs

Pediatric Ophthalmology Scope of Practice

ABSTRACT Purpose To determine practice patterns of pediatric ophthalmologists with respect to types of medical conditions cared for and age of patients treated due to limited data regarding pediatric ophthalmologists’ scope of practice. Methods A survey was sent to 1408 international and United States members of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) membership through the group’s internet listserv. Responses were collated and analyzed. Results Ninety members (6.4%) responded. 89% of respondents confine their practices to pediatric ophthalmology and adult strabismus. The percentages of respondents who provide primary surgical and medical treatment of the following conditions include 68% for ptosis and anterior orbital lesions, 49% for cataracts, 38% for uveitis, 25% for retinopathy of prematurity, 19% for glaucoma, and 7% for retinoblastoma. For conditions other than strabismus, 59% restrict their practice to patients less than 21 years of age. Conclusion Pediatric ophthalmologists provide primary medical and surgical care for children with a wide variety of ocular conditions, including complex disorders. Awareness of this variety of practice might prove beneficial in encouraging residents to consider careers in pediatric ophthalmology. Consequently, fellowship education in pediatric ophthalmology should include exposure to these areas.

Анатомія, фізіологія, клінічні прояви та лікування перекруту яєчка в дітей (огляд літератури)

У статті наведено сучасні дані вітчизняної та зарубіжної літератури про основні проблеми хірургічної корекції перекруту яєчка в дітей. Розглянуто ефективність різних методів оперативного лікування й можливість їх практичного застосування. Подані нові концепції надання хірургічної допомоги дітям різного віку на етапах лікування.

Barriers and potential solutions for improved surgical care for children with hernia in Eastern Uganda

Five billion people lack timely, affordable access to surgery. A large proportion of these are children. Qualitative research investigating the barriers to surgical care for children and ways of overcoming them is lacking. This study focused on children with hernia, a very common paediatric surgical condition for which surgery is the only effective treatment. The main aim of this qualitative study was to explore barriers to surgical care for children and identify potential solutions. Data were collected from parents of children with hernia and from health care providers at Soroti Regional Referral Hospital in eastern Uganda. Parents’ experiences, motives and barriers when accessing care were explored. The health care providers’ knowledge, perceptions and practices relating to children with hernia were investigated. The data were analysed using thematic content analysis. Traditional beliefs and gender inequality were considered major issues. Possible solutions included partnering with the local community in efforts to increase knowledge and acceptability in the community in general and by parents in particular. A formation of a surgical team dedicated to the management of children with surgical conditions was suggested as way to improve quality and increase volume of surgery for children.

Complication profile of augmentation cystoplasty in contemporary paediatric urology: a 20-year review.

The aim of this study was to describe the complication profile of augmentation cystoplasty in contemporary paediatric urology as well as its effect on bladder metrics. Consecutive operative cases were retrospectively reviewed at a single institution over 20 years (1999-2019). Short- and long-term outcomes and complications following augmentation cystoplasty were defined. Of the 71 operative cases; the most common underlying diagnoses were neurogenic bladder (34%), exstrophy-epispadias complex (30%) and posterior urethral valves (23%). The most common tissue-type utilized was ileal (58%) and ureteric (30%). Peri-operative urine leak affected nine (13%) children but reservoir perforations were less common (4%). Mean end-of-study detrusor pressure improved significantly following bladder augmentation (38-17 cmH2 O, P< 0.001). Bladder capacity improved significantly (67-89%, P= 0.041). The median follow-up was 4.5 years (interquartile range: 1.9-10 years). Bladder urolithiasis affected 13 (18%) patients, and symptomatic urinary tract infections 36 (51%) patients. Formation of a continent catheterisable channel contributed a number of complications relating predominantly to stenosis (50%). Repeat augmentation cystoplasty was necessary in three (4%) cases. Augmentation cystoplasty is a surgical intervention that improves bladder metrics. Given the potential complications, careful patient selection and appropriate pre-operative counselling are essential. Furthermore, pro-active post-operative management and transitional care are vital in the surgical care of children following augmentation cystoplasty.

Long-Term Outcomes and the Post-Intensive Care Syndrome in Critically Ill Children: A North American Perspective

Advances in medical and surgical care for children in the pediatric intensive care unit (PICU) have led to vast reductions in mortality, but survivors often leave with newly acquired or worsened morbidity. Emerging evidence reveals that survivors of pediatric critical illness may experience a constellation of physical, emotional, cognitive, and social impairments, collectively known as the “post-intensive care syndrome in pediatrics” (PICs-P). The spectrum of PICs-P manifestations within each domain are heterogeneous. This is attributed to the wide age and developmental diversity of children admitted to PICUs and the high prevalence of chronic complex conditions. PICs-P recovery follows variable trajectories based on numerous patient, family, and environmental factors. Those who improve tend to do so within less than a year of discharge. A small proportion, however, may actually worsen over time. There are many gaps in our current understanding of PICs-P. A unified approach to screening, preventing, and treating PICs-P-related morbidity has been hindered by disparate research methodology. Initiatives are underway to harmonize clinical and research priorities, validate new and existing epidemiologic and patient-specific tools for the prediction or monitoring of outcomes, and define research priorities for investigators interested in long-term outcomes.

What is a Pediatric Surgeon?

Patient Education| 2021 What is a Pediatric Surgeon? Connected Content Translation: ¿Qué es un Cirujano Pediátrico?: (What is a Pediatric Surgeon?) Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Share Icon Share Facebook Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation What is a Pediatric Surgeon?. Pediatric Patient Education 2021; 10.1542/peo_document173 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All AAP SitesAll PublicationsPediatric Patient Education Search Advanced Search Subjects: Pediatric Specialist Handouts Topics: academic medical centers, american board of surgery, bronchoscopy, colonoscopy, congenital abnormality, endoscopic surgery, endoscopy, general surgery, gunshot wounds, hospitals, community If your child has an illness, injury, or disease that requires surgery, a Pediatric Surgeon has the experience and qualifications to treat your child. Surgical problems seen by pediatric surgeons are often quite different from those commonly seen by adult or general surgeons. Special training in pediatric surgery is important. Pediatric surgeons are medical doctors who have had At least 4 years of medical schoolFive additional years of general surgeryTwo additional years of residency training in pediatric surgeryCertification by the American Board of Surgery Pediatric surgeons treat children from the newborn stage through late adolescence. They choose to make pediatric care the core of their medical practice, and the unique nature of medical and surgical care of children is learned from advanced training and experience in practice. Pediatric surgeons diagnose, treat, and manage children's surgical needs including: Surgical repair... Copyright © 20002000American Academy of Pediatrics You do not currently have access to this content.

Assessing the previously repaired patient with an anorectal malformation who is not doing well

In this review, the care of children with a previously repaired anorectal malformation is explored. We know that the surgical care of children with anorectal malformations is complex; however, despite an increased understanding of the congenital anomaly and significant technical advances in the operative repair, many of these children continue to have poor functional outcomes. In this article we focus on the common surgical complications, discuss typical presentations, consider appropriate investigations, and review the risks and benefits of revisional surgery in those patients that are ‘not doing well’ following their primary reconstruction.

Epidemiology of Pediatric Surgery in the United States.

The epidemiology of pediatric surgery in the United States and whether disparities in access to surgical care exist on a national level remain inadequately described. We determined rates of surgical intervention and associations with sociodemographic factors among children 0-17years of age in the United States. Analysis of the 2005-2018 National Health Interview Survey samples included 155,064 children. Parents reported on whether their child had a surgery or surgical procedure either as an inpatient or outpatient over the past 12months. Multivariate logistic regression models, adjusted for age, sex, race and ethnicity, income, language, parent education, region, having a usual source of care, and comorbid conditions, examined odds ratios for sociodemographic factors associated with surgery, analyzing the most recent data (2016-2018; 25544 children). In the most recent data, 4.7% of children had surgical intervention each year, with an average of 3.9 million surgeries performed annually. Rates of surgery were stable between 2005 and 2018. Minority children had lower adjusted odds (aOR) of surgical intervention as compared to white, non-Hispanic children (aOR=0.6, 95%CI=0.5-0.8 for black children, and aOR=0.7, 95%CI=0.5-0.9 for Hispanic children). Other sociodemographic factors associated with a lower adjusted odd of surgical intervention included uninsured status (aOR=0.5; 95%CI=0.3-0.9), and primary language other than English (aOR=0.5; 95%CI 0.3-0.9). Income was not associated with surgical intervention. On average, 3.9 million surgeries are performed on children 0-17years of age in the United States each year. Significant disparities exist in surgical care for children, with black and Hispanic children having lower rates of surgery over and above contribution of other disparity domains. These findings in a nationally representative sample highlight the need for national policies to eliminate disparity of care received by minority children.

Tissue engineering: from the bedside to the bench and back to the bedside.

The field of Tissue Engineering and Regenerative Medicine has evolved rapidly over the past thirty years. This review will summarize its history, current status and direction through the lens of clinical need, its progress through science in the laboratory and application back into patients. We can take pride in the fact that much effort and progress began with the surgical problems of children and that many surgeons in the pediatric surgical specialties have become pioneers and investigators in this new field of science, engineering, and medicine. Although the field has yet to fulfill its great promise, there have been several examples where a therapy has progressed from the first idea to human application within a short span of time and, in many cases, it has been applied in the surgical care of children.

Barriers to Surgical Care Among Children in Somaliland: An Application of the Three Delays Framework.

There are complex barriers that increase delays to surgical care in low- and middle-income countries, particularly among the vulnerable population of children. Understanding these barriers to surgical care can result in targeted and strategic intervention efforts to improve care for children. The three-delay model is a widely used framework in global health for evaluating barriers associated with seeking (D1), reaching (D2), and receiving health care (D3). The goal of our study is to evaluate reasons for delays in the surgical care for children in Somaliland using the three-delay framework. Data were collected in a cross-sectional study in Somaliland from 1503 children through a household survey. Among children with a surgical need, we quantified the number of children seeking, reaching, and receiving care along the surgical care continuum, according to the three-delay framework. We evaluated predictors of the three delays through a multivariate logistic regression model, including the child's age, gender, village type, household income level, region, and household size. Of the 196 children identified with a surgical condition, 50 (27.3%) children had a delay in seeking care (D1), 28 (20.6%) children had a delay in reaching care (D2), and 84 (71.2%) children had a delay in receiving care (D3), including 10 children who also experienced D1 and D2. The main reasons cited for D1 included seeking a traditional healthcare provider, while lack of money and availability of care were main reasons cited for D2. Significant predictors for delays included household size for D1 and D3 and condition type and region for D2. Children in Somaliland experience several barriers to surgical care along the entire continuum of care, allowing for policy guidance tailored to specific local challenges and resources. Since delays in surgical care for children can substantially impact the effectiveness of surgical interventions, viewing delays in surgical care under the lens of the three-delay framework can inform strategic interventions along the pediatric surgical care continuum, thereby reducing delays and improving the quality of surgical care for children.

Prevalence of Pediatric Surgical Conditions Across Somaliland

Although surgical conditions are increasingly recognized as causing a significant health care burden among adults in low- and middle-income countries (LMICs), the burden of surgical conditions among children in LMICs remains poorly defined. To estimate the prevalence of pediatric surgical conditions across Somaliland using a nationwide community-based household survey. This cross-sectional study was conducted through a national community-based sampling survey from August through December 2017 in Somaliland. Participants were 1503 children surveyed using the Surgeons OverSeas Assessment of Surgical Need (SOSAS). The SOSAS survey contains 2 components, including a section on household demographics, deaths, and financial information and sections querying children's history of surgical conditions. In this cross-sectional study that included 1503 children (55.6% male; mean [SE] age, 6.4 [0.1] years), 221 surgical conditions were identified among 196 children, yielding a mean (SE) prevalence of pediatric surgical conditions of 12.2% (1.5%). Only 53 of these 221 surgical conditions (23.7%) had been surgically corrected at the time of the survey. The most common conditions encountered were congenital anomalies (33.8%) and wound-related injuries (24.6%). Nationally, an estimated 256 745 children have surgical conditions, with an estimated 88 345 to 199 639 children having unmet surgical needs. Using national sampling, this study found that children have a high burden of surgical conditions in Somaliland. These data highlight the need for a scale-up of pediatric surgical infrastructure and resources to provide the needed surgical care for children in LMICs.

Global paediatric surgery: meeting an unmet need\u2014the response of the British Association of Paediatric Surgeons

AimOutline the response from an organisation regarding the unmet needs in global children’s surgeryMethodThe burden of global surgical disease, whilst daunting, is becoming increasingly better defined as agencies, surgical colleges and professional specialty associations all attempt to increase capacity in terms of manpower, support education and find sustainable solutions to the deficit of health in treating women and children. However, definition of the problem does not in itself create change and similarly, humanitarian activities including volunteering by established surgical practitioners and other non-governmental organisations (NGOs) make only marginal improvement in the standards of care on offer at a global level.ResultsThe International Affairs Committee, British Association of Paediatric Surgeons (BAPS) has had its target firmly set on investing in potential leaders within paediatric surgery in low- and middle-income countries (LMICs), and sharing elements of the educational programme made available for training within the UK and Ireland with the aim of contributing to the solutions of inequity in the surgical standards available to the world’s children.ConclusionThis article outlines some of the practical steps that have been deployed by BAPS by way of sharing the responsibility for problem-solving at a global level. It also highlights the need for clarity in advocacy and the route through which effective communication can translate into wider and more effective delivery of surgical care for children.

The Pediatric Surgical Patient

The surgical care of pediatric patients requires familiarization with the physiologic principles that govern homeostasis in neonates, infants, children, and adolescents. These principles must be taken into consideration in the perioperative management and trauma care of pediatric patients. In addition to surgical problems common to all age groups, there are specific pathologic processes requiring surgery (or treatment by a surgeon) that are seen only in pediatric patients. Here we present an overview of the physiologic considerations important for the surgical care of children and a review of the most commonly encountered pediatric surgical conditions, with an emphasis on the acute management and stabilization of the patient. References to current guidelines are being provided when available. This review contains 2 figures, 5 tables, and 62 references. Key words: abdominal wall defects, diaphragmatic hernia, gastroschisis, nonaccidental trauma, nutrition, pediatric surgery, pediatric trauma, perioperative care

First liver transplantation for biliary atresia in children: The hidden effects of non-centralization.

The aim of our study was to determine the impact of initial orientation for medical and surgical care of children with BA on procedures and outcomes of the first LT. We retrospectively analyzed charts of children with BA who underwent first LT between 2006 and 2015. Patients were divided into two groups for comparison: a single-center management group (from diagnosis to transplantation) and a secondarily referred group (children referred after failure of KP). We focused analysis on disease severity at transplantation, blood transfusion, and overall survival. One hundred and eighty-five children were included. The median delay between pretransplant check-up and transplantation was shorter in patients secondarily referred. A severe undernutrition was observed in 23.7% of children secondarily referred compared to 11.1% in children with a single-center management (P=.024). At transplantation, INR and factor V level were higher in single-center group patients (respectively, 67% vs 55%, P<.001 and 61% vs 49%, P=.002). The total of red blood cell and fresh frozen plasma administrated during procedure was two times higher in patients secondarily referred. Finally, patients with a single-center management had a higher overall 12months of survival rate (92.1% vs 83.1%, P=.033). In a country without low-density population issues, the authors advocate an early referring to transplant center to further improving LT outcomes in children with BA.

心脏瓣膜病遗传学

心脏瓣膜病伴有显著的发病率和死亡率，并且常是由于先天性畸形引起心脏瓣膜结构和功能异常，导致瓣口狭窄或关闭不全所致的。然而，成年人心脏瓣膜病患病率日益增加，不仅是因为老龄化日益严重，而且还因为先天性心脏瓣膜缺陷儿童的医疗和外科护理的改善。人类基因组计划的成功和遗传技术的重大进展，加上我们对心脏瓣膜发育的了解越来越多，导致人们发现许多心脏瓣膜疾病的遗传因素。这些发现使用各种方法，包括家族性瓣膜病的检查和全基因组关联研究，以调查散发病例。本综述将讨论这些发现及其在瓣膜性心脏病治疗中的意义。 Valvular heart disease is associated with significant morbidity and mortality, and is often caused by congenital malformations resulting in abnormal cardiac valve structure and function, resulting in stenosis or insufficiency of the valve. However, the prevalence of valvular heart disease in adults is increasing, not only because of the growing aging, but also because of the improvement of medical and surgical care for children with congenital heart valve defects. The success of genomics project and the great progress of genetic technology, and our increasing understanding of cardiac valve development have led to the discovery of many genetic factors of heart valve disease. These findings use a variety of methods, including familial valvular disease examination and genome wide association studies to investigate sporadic cases. This review will discuss these findings and their significance in the treatment of valvular heart disease.