Abstract:
BACKGROUND/PURPOSE:VACTERL syndrome is a common surgical condition affecting the development of many midaxial organs. The etiology, embryology, and pathogenesis of the VACTERL syndrome are not known. The authors report here new mouse models of VACTERL syndrome involving the Sonic hedgehog (Shh) signaling pathway. METHODS:Mutant mice involving Shh signaling, the Shh transcription factors Gli2-/- and Gli3-/-, Gli2-/-;Gli3+/- double heterozygotes, and Shh-/- were analyzed. RESULTS:In addition to reported vertebral, anal, tracheoesophageal, and limb anomalies, mutant mice display cardiac, renal, and associated anomalies, namely congenital diaphragmatic hernia and omphalocele, known to be associated in VACTERL syndrome. The Shh transcription factors Gli2 and Gli3 have specific and overlapping roles in the induction of VACTERL phenotypes in a gene-dose dependent manner in these mutants. CONCLUSION:To the authors' knowledge, these mutant mice represent the first animal model that mimics the human VACTERL syndrome, and suggests that aberrations in Shh signaling might be involved in the VACTERL syndrome.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Kim PC,Mo R,Hui Cc Cdoi
10.1053/jpsu.2001.20722subject
Has Abstractpub_date
2001-02-01 00:00:00pages
381-4issue
2eissn
0022-3468issn
1531-5037pii
S0022346801580756journal_volume
36pub_type
杂志文章abstract:BACKGROUND:If creating an obstructive uropathy early in glomerulogenesis produces MCDK (Multicystic Dysplastic Kidney), then a very early obstruction may produce Potter's Syndrome (PS) with oligohydramnios. METHODS:Fetal lambs at 50 days' gestation underwent urethral and urachal ligation using fine SILASTIC (Dow Corni...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2000.18305
更新日期:2000-11-01 00:00:00
abstract::Successful portal decompression presents a unique challenge in children. The meso-caval shunt, utilizing the large caliber iliac vein in constructing the anastomosis, has been accepted as the standard operative procedure for treating these patients. Technical and anatomic difficulties, however, often prevent the succe...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(79)80572-2
更新日期:1979-02-01 00:00:00
abstract::Solid pseudopapillary tumors of the pancreas (SPTP) are very rare, and an SPTP arising in a pancreatic rest has been reported only 4 times previously and never in association with the jejunum. We report this unusual case of a 16 year old girl who presented with 4 days of intermittent, crampy abdominal pain and was fou...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2009.01.074
更新日期:2009-04-01 00:00:00
abstract:BACKGROUND:Delaying surgery for infants with CDH until they achieve clinical stability is common practice. Stability, however, is inconsistently defined, and many infants fail to reach pre-established criteria. We sought to determine if infants undergoing surgery without meeting pre-established criteria could achieve m...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2013.02.002
更新日期:2013-05-01 00:00:00
abstract::An 11-day-old neonate presents with worsening nonbilious emesis and radiographic findings suggestive of pyloric stenosis. Using a laparoscopic approach, a slightly asymmetric and hypertrophied pylorus was visualized. The unusual age of presentation and appearance of the pylorus led to the decision to perform an open e...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.02.045
更新日期:2011-07-01 00:00:00
abstract::The joining of widely separated proximal and distal esophageal segments in esophageal atresia remains a challenging problem. Livaditis introduced the operation of circular myotomy as an effective means of bridging such a wide gap. Three babies with esophageal atresia were successfully treated in this manner at the Hos...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(78)80453-9
更新日期:1978-08-01 00:00:00
abstract::Agenesis of the dorsal mesentery in association with jejunal atresia is a well-recognized congenital anomaly. Only few reports exist in the English literature of children with agenesis of the dorsal mesentery without small bowel atresia. Herein, we report a child with total mesenteric agenesis presenting with midgut v...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2006.05.011
更新日期:2006-09-01 00:00:00
abstract:BACKGROUND:Rhabdoid tumors (RTs) of the liver are rare, aggressive and nonsecreting malignancies occurring mainly during the first year of life. Definition of RT relies on characteristic morphology and on the inactivation of the SMARCB1 tumor suppressor gene. The aim of this study was to analyze clinical data, treatmen...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.09.005
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Urologic complications after pediatric renal transplantation can adversely effect the outcome and may result in decreased graft survival. Efforts to prevent these complications are worthwhile. This study investigates the incidence of these complications in a clinical transplant program and reports on an anim...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(99)90654-1
更新日期:1999-11-01 00:00:00
abstract:PURPOSE:In October 2000, our institution implemented a clinical practice guideline (CPG) utilizing selective computed tomography (CT) and ultrasound scan (US) for the evaluation of children with suspected appendicitis. The authors sought to determine surgical outcomes and diagnostic accuracy in the CPG period. METHODS...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2003.11.015
更新日期:2004-03-01 00:00:00
abstract:PURPOSE:The aim of this study was to identify the most appropriate timing for surgery in newborns with choledochal cysts. METHODS:The clinical and histological data of 8 newborn cases of choledochal cysts (newborn group) were compared with 45 cases that were diagnosed later than the newborn period (late group). RESUL...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(99)90308-1
更新日期:1999-12-01 00:00:00
abstract:PURPOSE:The objective of this study is to use anorectal manometry for functional assessment of early postoperative results after corrective surgery for anorectal malformations (ARMs) in children and compare manometric observations with age-matched controls. Parents were counseled and management strategies were planned ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.04.012
更新日期:2010-09-01 00:00:00
abstract:PURPOSE:The purpose of the study is to study the natural course of undescended testes after inguinoscrotal surgery. METHODS:From 2003-2010, 24 boys were observed with 26 undescended testes after inguinoscrotal surgery; 12 had previously undergone inguinal hernia repair and 12 orchidopexy. Spontaneous descent was await...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2013.06.016
更新日期:2013-12-01 00:00:00
abstract:PURPOSE:Feeding tube access with an antireflux procedure is frequently necessary in children with severe neurological deficits. Fundoplication in this particular group of patients has many complications and a reported failure rate of 40% to 50%. Recently, the use of a feeding Roux-en-Y jejunostomy has been advocated in...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(97)90713-2
更新日期:1997-04-01 00:00:00
abstract::Tracheoesophageal fistula (TEF) without atresia is rare and usually presents with symptoms from birth. In this report, a 9-year-old boy presented with productive cough of 4 month's duration and was shown to have a right lung abscess seen on chest radiograph. His parents denied earlier respiratory symptoms or illnesses...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(98)90294-9
更新日期:1998-12-01 00:00:00
abstract:BACKGROUND:There is ongoing debate concerning the use of peritoneal irrigation in the setting of complicated appendicitis (CA) in children. Our aim was to conduct a prospective randomized controlled trial for the treatment of CA during a laparoscopic appendicectomy in children. METHODS:Following ethical approval (REC1...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jpedsurg.2019.08.039
更新日期:2019-12-01 00:00:00
abstract:INTRODUCTION:Perforated appendicitis is a common condition in children, which, in a small number of patients, may be complicated by a well-formed abscess. Initial nonoperative management with percutaneous drainage/aspiration of the abscess followed by intravenous antibiotics usually allows for an uneventful interval ap...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jpedsurg.2009.10.039
更新日期:2010-01-01 00:00:00
abstract::Recently, interest has been expressed in the correction of diaphragmatic hernia in utero as a solution to the problem of high mortality from this condition. Before such a procedure can be adopted in the human, studies aimed at establishing the contribution such advanced techniques would make in reducing mortality are ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(84)80010-x
更新日期:1984-02-01 00:00:00
abstract:PURPOSE:The purpose of this study was to quantify pathologic lesions of the kidney found incidentally during the workup of a blunt renal trauma. METHODS:A retrospective review of the medical records of 103 patients ages 0 to 18 years with blunt renal injuries admitted to a level 1 pediatric trauma center between Janua...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2002.32286
更新日期:2002-05-01 00:00:00
abstract:BACKGROUND/PURPOSE:Burns involving the genitalia and perineum are commonly seen in the context of extensive total body surface area (TBSA) burns and rarely as isolated injuries because of protection provided by the thighs and the abdomen. Genital burns usually result in extended hospital stays and are accompanied by se...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.02.050
更新日期:2011-08-01 00:00:00
abstract::Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the ri...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s0022-3468(99)90644-9
更新日期:1999-11-01 00:00:00
abstract::From 1973 to 1988, 105 children with renal failure (average age, 6.5 years; range, 5 days to 16.2 years) were treated with hemodialysis via surgically implanted Scribner shunts (n = 120). The average duration of dialysis was 16 days (range, 1 to 194 days). Twenty-three patients died during treatment or during the hosp...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(94)90153-8
更新日期:1994-11-01 00:00:00
abstract::To minimize the risk of exsanguinating hemorrhage, the authors describe a technique of laparoscopic ligation of the median sacral artery before posterior sagittal resection of type I sacrococcygeal teratoma (SCT). Two female infants with antenatally diagnosed SCT underwent postnatal evaluation and preoperative imaging...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2004.04.042
更新日期:2004-08-01 00:00:00
abstract::Cytokines are immunoregulatory molecules that are important mediators of the host response to stress and infection. Infants and children undergoing major surgery are particularly at risk of developing sepsis and have altered metabolic responses to surgical stress compared to adults. We have investigated the temporal s...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(92)90553-j
更新日期:1992-08-01 00:00:00
abstract::Cystic lesions of the spleen are uncommon, about 600 cases being reported in the world literature. This report concerns three pediatric patients treated by partial splenectomy for benign cyst. In the world literature most of the cystic lesions of the spleen are treated by splenectomy and more recently by partial splen...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(86)80357-8
更新日期:1986-09-01 00:00:00
abstract:BACKGROUND:Patients with repaired esophageal atresia often show persistent motility disorders of the esophagus. METHODS:In this study, the authors used a newly developed method, videomanometry, to study intraluminal pressures and function of the pharynx and upper esophagus in such a group of patients and compared the ...
journal_title:Journal of pediatric surgery
pub_type: 临床试验,杂志文章
doi:10.1016/s0022-3468(98)90154-3
更新日期:1998-08-01 00:00:00
abstract::Sixteen patients with biliary atresia had 44 steroid courses for treatment of cholangitis or diminution of bile flow following Kasai hepatic portoenterostomy operations. A "blast" type (high dose/short duration) steroid administration was employed to potentiate the choleretic and anti-inflammatory effect. There was a ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(85)80026-9
更新日期:1985-12-01 00:00:00
abstract::A simple technical detail in placing the upper pouch holding suture to facilitate proximal segment mobilization in esophageal atresia is described. The suture is passed through both walls of the upper segment and the tip of the Foley catheter. If circular myotomy is needed, the Foley catheter balloon is inflated to fi...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80422-0
更新日期:1987-02-01 00:00:00
abstract::There is a significant incidence of inguinal hernia in premature infants and the optimal timing of repair is controversial. A high rate of postoperative respiratory complications has been reported in this group. In this study, the records of 47 premature infants (mean gestational age, 30.3 weeks) who underwent hernior...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(05)80285-4
更新日期:1993-02-01 00:00:00
abstract:PURPOSE:Although gastroschisis infants usually have a good outcome, there remains a cohort of babies who fare poorly. We inquired whether the presence of bowel dilatation in utero is predictive of postnatal course in infants with gastroschisis. METHODS:We compared the clinical course of infants who had bowel dilatatio...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.03.033
更新日期:2011-06-01 00:00:00