Abstract:
:Biliary atresia (BA) remains an enigmatic disease with a degree of etiologic heterogeneity. A number of variants can be defined clinically, and these include the syndromic group (typically BA splenic malformation), cystic BA, and cytomegalovirus (CMV) IgM +ve associated BA. The remainder, and still the largest group, may be termed isolated BA(.) There is a wide variation in incidence across the globe from 1 in 5000 in Taiwan to 1 in 20,000 live births in Northern Europe, although the reasons for such a disparity remain obscure. Management remains primarily surgical with an attempt to restore bile flow by resection of extrahepatic biliary remnants and a reconstruction portoenterostomy (the Kasai procedure), reserving liver transplantation for those where this fails or complications of chronic liver disease supervene. Clearance of jaundice to normal values has been achieved in 40%-55% of cases in large series from around the world, with an expectation of 5-year native liver survival of similar proportions.
journal_name
Semin Pediatr Surgjournal_title
Seminars in pediatric surgeryauthors
Davenport Mdoi
10.1053/j.sempedsurg.2012.05.010subject
Has Abstractpub_date
2012-08-01 00:00:00pages
175-84issue
3eissn
1055-8586issn
1532-9453pii
S1055-8586(12)00035-2journal_volume
21pub_type
杂志文章,评审abstract::Congenital tracheobronchial stenosis is a rare disease characterized by complete tracheal rings that can affect variable lengths of the tracheobronchial tree. It causes high levels of morbidity and mortality both due to the stenosis itself and to the high incidence of other associated congenital malformations. Success...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2016.02.007
更新日期:2016-06-01 00:00:00
abstract::Identification of CDH infant populations at high risk for mortality postnatally may help to develop targeted care strategies, guide discussions surrounding palliation and contribute to standardizing reporting and benchmarking, so that care strategies at different centers can be compared. Clinical prediction rules are ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2017.04.010
更新日期:2017-06-01 00:00:00
abstract::Hirschsprung (HSCR) disease is a relatively common neonatal developmental disorder of the enteric nervous system and is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the distal intestine. This results in absent peristalsis in the affected bowel, and the development of a fun...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2004.10.027
更新日期:2005-02-01 00:00:00
abstract::Pediatric heart transplantation has undergone major changes over the past two decades, marked by a substantial improvement in survival, reduction in posttransplant complications, and enhancement in quality of life for transplant recipients. Actuarial survival has improved substantially in the last decade. Indications ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2006.03.011
更新日期:2006-08-01 00:00:00
abstract::When a female is born and has only a single perineal orifice on the newborn clinical examination, a diagnosis of cloaca type of anorectal malformation is made. Along with associated malformations which may initiate the ordering of radiologic imaging, there are a finite number of radiologic tests that are performed to ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2015.11.007
更新日期:2016-04-01 00:00:00
abstract::Adult and pediatric urologists, gynecologists, and surgeons are often faced with primary reconstruction of complex congenital defects of the vagina as seen in cloacal anomalies, disorders of sexual development (DSD), and vaginal agenesis as well as with the secondary repair of post-surgical or radiation-induced vagina...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.sempedsurg.2019.150843
更新日期:2019-10-01 00:00:00
abstract::Central venous catheters (CVC) are commonplace in the management of critically ill patients and serve a variety of purposes. Venous access is important for the administration of fluid and medications as well as blood procurement needed for lab analyses. However, not every critically ill patient requires a CVC. This ar...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2019.01.005
更新日期:2019-02-01 00:00:00
abstract::Colorectal disease profiles for children in low- and middle-income settings (LMIC) are characterized by late presentation, increased complications and limited follow-up in many cases. There is a high prevalence of infectious conditions causing secondary colorectal disease such as Mycobacterium Tuberculosis(TB), Human ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.sempedsurg.2020.150989
更新日期:2020-12-01 00:00:00
abstract::Children with anorectal malformations (ARMs) constitute a significant group within a pediatric surgery practice. In females, the most common ARM is an imperforate anus with a rectovestibular fistula, followed by an imperforate anus with a rectoperineal fistula and then cloacal anomalies. Some malformations, such as an...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2009.11.019
更新日期:2010-05-01 00:00:00
abstract::Traditional surgical attitude regarding error and complications has focused on individual failings. Human factors research has brought new and significant insights into the occurrence of error in healthcare, helping us identify systemic problems that injure patients while enhancing individual accountability and teamwo...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2015.08.003
更新日期:2015-12-01 00:00:00
abstract::Prune belly is a lax, wrinkled abdominal wall and frequently is associated with other anomalies. The prune belly syndrome is a specific constellation of anomalies consisting of an abdominal wall deficient in muscular tissue, dilated urinary tract, and bilateral cryptorchidism. This group of anomalies is also called th...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/spsu.2000.7556
更新日期:2000-08-01 00:00:00
abstract::Focal developmental malformations of the lung are a heterogeneous group of lesions that display a variety of features. They often are thought to be quite distinct entities, but there is considerable overlap among these lesions. Both standard nomenclature and common usage do not identify clearly the components of these...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/spsu.2003.00001
更新日期:2003-02-01 00:00:00
abstract::Traditionally, pediatric surgical education consisted of exposure to patients, textbooks, lectures, team-based education, congresses, and workshops. Over the last decades, however, new information technology (IT) and the internet revolutionized the sharing of information and communication. IT has become relevant in pa...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2015.02.012
更新日期:2015-06-01 00:00:00
abstract::The field of intestinal transplantation has experienced dramatic growth since the first reported cases 3 decades ago. Improvements in operative technique, donor assessment and immunosuppressive protocols have afforded children who suffer from life-threatening complications of intestinal failure a chance at long-term s...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2017.07.007
更新日期:2017-08-01 00:00:00
abstract::Disorders of sexual development (DSDs) are relatively rare congenital conditions in which the development of the chromosomal, gonadal, or anatomic sex is atypical. Some conditions may not manifest until puberty or adulthood. The examination and workup of either an infant or an older patient with suspected DSD should b...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2015.01.009
更新日期:2015-04-01 00:00:00
abstract::Nutritional management of infants and children differs from that of adults because of the extra requirements for growth and the limitations of physiological immaturity. Although parenteral nutrition (PN) is an accepted practice and a potentially life-saving therapy for pediatric patients who cannot be fed through thei...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1992-08-01 00:00:00
abstract::In dealing with conjoined twins, we have adopted a pragmatic approach in balancing what is possible, parental wishes and the likely outcome for the children. We believe that separation where feasible should be undertaken but appreciate that a few parents may wish their children to remain joined. The advice that we giv...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2015.06.008
更新日期:2015-10-01 00:00:00
abstract:OBJECTIVE:Exploring pros and cons of bridging long-gap esophageal atresia with an orthotopic jejunal pedicle graft. Retrospective series of 19 patients. METHODS:From 1988 through 2005, 19 patients with long-gap esophageal atresia received a jejunal graft. Median age at reconstruction was 76 days. The technique involve...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/j.sempedsurg.2008.10.007
更新日期:2009-02-01 00:00:00
abstract::Survey methodologies enable researchers to obtain information not routinely captured in electronic medical records, including how delivery of surgical care is perceived through the lens of a child, a parent or caregiver, or a surgeon. Here we describe the approach to using surveys to aid pediatric surgery practice. Th...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2018.10.007
更新日期:2018-12-01 00:00:00
abstract::During the past 10 years, the minimally invasive technique has become the standard approach for many thoracic, abdominal, and pelvic procedures in adults and children. We now know there are well-defined benefits to this approach. However, when it was introduced, the laparoscopic technique was quickly adopted without a...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2011.05.008
更新日期:2011-11-01 00:00:00
abstract::Adhesive small bowel obstruction is a significant cause of short- and long-term morbidity in infants and children. Unfortunately, the majority of scientific literature relative to adhesive obstructions continues to be dominated by adult studies. In this article, the existing literature for infant and pediatric adhesiv...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2014.06.006
更新日期:2014-12-01 00:00:00
abstract::Sternal clefts, ectopia cordis, and Cantrell's pentalogy continue to be very rare congenital anomalies in pediatric surgery. Unfortunately, these conditions present as neonatal emergencies and demand early surgical intervention. This article reviews the embryological development of the chest wall, specific sternal def...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2008.03.004
更新日期:2008-08-01 00:00:00
abstract::Enterocolitis remains a relatively common complication of Hirschsprung's disease with significant morbidity and mortality. The etiology of Hirschsprung's enterocolitis (HEC) is multifactorial and remains poorly understood. Preventative measures and better treatment modalities will evolve out of a better understanding ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2004.10.014
更新日期:2004-11-01 00:00:00
abstract::In the United States nearly 2 million people are burned every year; about 100,000 burns are moderate to severe and require hospitalization and about 5,000 deaths occur because of burns. The overall improvement in mortality and outcome of patients with severe burn trauma over the last decades can be attributed to the f...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1053/spsu.2001.19389
更新日期:2001-02-01 00:00:00
abstract::This review of parathyroid surgery in children will briefly discuss parathyroid gland embryology and anatomy before focusing on the pathophysiology, clinical presentation, and treatment of hyperparathyroidism in children. Hyperparathyroidism (HPT) is the overproduction of PTH and it is rare in children, with an incide...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2014.03.003
更新日期:2014-04-01 00:00:00
abstract::The spectrum of complications associated with congenital lung malformation is wide. They can range from fetal hydrops in utero to postnatal problems of ventilation, obstruction and infection; presentation may occur from the neonatal period to adulthood. Many lesions will remain asymptomatic while at the other end of t...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2015.02.002
更新日期:2015-08-01 00:00:00
abstract::Caring for children with colorectal and pelvic reconstructive needs is complex and requires a lifelong commitment from clinicians devoted to the field. There has been a myriad of advances that have improved care and it has become clear that an integrated, multi-disciplinary approach maximizes the goal of improving the...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.sempedsurg.2020.150992
更新日期:2020-12-01 00:00:00
abstract::Congenital obstructive uropathy is a relatively common developmental malformation, and severely affected fetuses die soon after birth from oligohydramnios-induced pulmonary hypoplasia or renal failure. Prenatal ultrasonography can reliably diagnose the specific anatomic defect, and using fetal urine sampling we can de...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1993-05-01 00:00:00
abstract::The optimal surgical care of patients with imperforate anus begins with appropriate decision making in the critical newborn period. In most cases the decision to create a colostomy should be delayed until the infant is 18 to 24 hours old. Except in cases of a rectoperineal fistula, most neonates are best treated with ...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:
更新日期:1997-11-01 00:00:00
abstract::Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features...
journal_title:Seminars in pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/j.sempedsurg.2018.08.007
更新日期:2018-10-01 00:00:00