Abstract：
BACKGROUND/PURPOSE:The genetic, embryological, and pathogenetic aspects of hindgut development remain poorly understood. Recently, the morphogenetic pathway involving the Sonic hedgehog (Shh) gene has been shown essential to the normal development of many midaxial organs, including the foregut. This study reports genetically based murine models of congenital anorectal malformations (CAM) involving the Shh-responsive transcription factors, Gli2 and Gli3. Its purpose is to show the necessity of these 2 factors to normal hindgut development. METHODS:Gli2-/- mutants were generated by a targeted deletion. Gli3-/- mutants are spontaneous mutants involving the Gli3 gene. Gli2-/- Gli3+/- mutants were generated by intercrossing double heterozygotes. Whole-mount midsagittal sections of the embryos were analyzed on embryonic days (E) 11.5 and E13.5. RESULTS:Gli3-/- mutants had anal stenosis and ectopic anus, and Gli2-/- mutants showed imperforate anus and rectourethral fistula. Gli2-/- Gli3+/- mutants had a cloacal abnormality. CONCLUSIONS:The phenotypic abnormalities observed in these mutant mice are identical to the spectrum of human CAM. The severity of the phenotype appears to reflect the gene dose. Gli2 and Gli3 play an important role in the normal development of murine hindgut. The results of this study provide, for the first time, a molecular basis for CAM.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Kimmel SG,Mo R,Hui CC,Kim PCdoi
10.1016/s0022-3468(00)90014-9subject
Has Abstractpub_date
2000-02-01 00:00:00pages
227-30; discussion 230-1issue
2eissn
0022-3468issn
1531-5037pii
S0022-3468(00)90014-9journal_volume
35pub_type
杂志文章abstract：:A rare case of ileal duplication in a 6-year-old boy is presented. The duplicated area is just like an additional ectopic stomach both in radiologic and pathologic examination. The patient was operated on because of intestinal obstruction. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.29441
更新日期：2002-01-01 00:00:00
abstract：:An 8-month-old boy was found to have a solitary hepatic mesenchymal hamartoma. Histologically, the lesion appeared as a large island of loose mesenchymal tissue with few cystic bile ducts and liver cells. Electronmicroscopy showed microvilli on the surface of tumor cells and desmosomes between the cells. Immunohistoch...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(84)80197-9
更新日期：1984-06-01 00:00:00
abstract：:Solitary nonparasitic cyst of the liver (SNPCL) is rare in children. Although there are several hypotheses regarding the pathogenesis, the true origin of SNPCL remains unknown. The authors present an infant with a huge SNPCL in whom the epithelial markers, CA19-9, DU-PAN-2, and SPan-1, were elevated in the serum and c...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.08.039
更新日期：2005-12-01 00:00:00
abstract：BACKGROUND:Single-incision laparoscopic surgery (SILS) has been successfully performed in children using 5-mm reticulating instruments. There are, however, few reports investigating the use of conventional instruments in SILS in the pediatric population. METHODS:We conducted a retrospective review of all consecutive c...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.08.036
更新日期：2010-12-01 00:00:00
abstract：BACKGROUND/PURPOSE:Biliary anastomotic strictures (BAS) after left lateral segment liver transplantation (LLST) may cause graft dysfunction, sepsis, and patient mortality. A review of the authors' experience was performed to better characterize the risk factors and corrective management. METHODS:The medical records of...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2000.6932
更新日期：2000-06-01 00:00:00
abstract：:In infants and children requiring prolonged and multiple central venous catheterizations, conventional cannulation sites may become thrombosed or stenotic, making inability to gain vascular access a life-threatening problem. The technique we use for the percutaneous placement of inferior vena caval tunneled silastic c...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(90)90341-6
更新日期：1990-06-01 00:00:00
abstract：:The cumulative evidence supports nonoperative management of hepatic trauma in children who are hemodynamically stable and who require less than 50% blood volume replacement to maintain a stable blood count. This management approach, based on the use of either serial computed tomography scans, liver/spleen scans, or ul...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(92)90503-y
更新日期：1992-12-01 00:00:00
abstract：:Nine infants underwent operations for perforation of a Meckel's diverticulum over a 21-yr period. All of the infants were acutely ill when initially seen by the surgical service. Lethargy, irritability, anorexia, fever, abdominal tenderness, and passage of blood in the stools were common clinical manifestations. The p...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(75)90277-8
更新日期：1975-04-01 00:00:00
abstract：:Seventy-five years ago, a 7-year-old girl underwent the first successful ligation of a patent ductus arteriosus. This procedure transformed her health and was a milestone in the development of cardiac surgery. The operation was performed by Dr. Robert E. Gross, then the surgical chief resident at Boston Children's Hos...
journal_title：Journal of pediatric surgery
pub_type： 传,历史文章,杂志文章
doi：10.1016/j.jpedsurg.2013.06.008
更新日期：2013-08-01 00:00:00
abstract：:A preterm infant's immature brain is susceptible to both anoxic and hemorrhagic injury during periods of physiological stress. The advent of in utero surgery has created a new population of premature patients at risk for central nervous system (CNS) injury. The aim of this study was to evaluate the frequency and natur...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90009-8
更新日期：1995-08-01 00:00:00
abstract：:The authors report on two adults who had jejunal dilatation after having had resection for jejunal atresia in the neonatal period. Both patients presented 20 years after the initial procedure, with severe iron deficiency anemia, marked jejunal dilatation proximal to the old anastomotic site (which was not narrowed), a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90238-0
更新日期：1994-12-01 00:00:00
abstract：AIM:Extrahepatic portal vein obstruction (EPVO) is a common cause of portal hypertension in children and can lead to life-threatening bleeding, thrombocytopenia, and coagulation disorders. Mesoportal bypass (MPB) restores normal physiologic portal flow to the liver and corrects portal hypertension. There is, however, l...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2009.08.019
更新日期：2010-01-01 00:00:00
abstract：:In this report a rare case of mediastinal abscess secondary to purulent tonsilitis is reported. Generally this condition is consequent to systemic, cervical, thoracic, or abdominal infections. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80090-9
更新日期：1989-11-01 00:00:00
abstract：PURPOSE:We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. METHODS:BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or olde...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2017.11.016
更新日期：2018-02-01 00:00:00
abstract：BACKGROUND:Investigation of the value of scrotal Doppler ultrasound (DUS) in the diagnosis of acute scrotum and its impact on the treatment strategy was undertaken. METHOD:Seventy-nine children (mean age, 9.2 years) with acute scrotum were prospectively studied. The protocol included history, clinical examination, scr...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.04.026
更新日期：2006-08-01 00:00:00
abstract：:In 25 years, from 1959 to 1984, esophageal substitution was performed in 32 patients. In most, the transverse colon was used, brought through the left chest on a vascular pedicle of the left colic artery. Indications for operation included: 21, esophageal atresia; 5, caustic injury; 3, peptic stricture; 2, esophageal ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(85)80051-8
更新日期：1985-12-01 00:00:00
abstract：:Translational research (TR) bridges discovery to clinical delivery. All TR also requires the development of an intervention. Classical 'bench to bedside' TR is responsible for many important advances, but cannot account for many others, which begin with clinical observations. My personal involvement in TR has ranged f...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2014.10.023
更新日期：2015-01-01 00:00:00
abstract：:Hypertension is a known complication after renal trauma. The cause of posttraumatic hypertension can be renal scarring, infarction, hydonephrosis, infection, vascular injury, and parenchymal compression. The authors report on the case of a 16-year-old boy who experienced hypertension after blunt renal trauma. He had a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(97)90630-8
更新日期：1997-06-01 00:00:00
abstract：PURPOSE:To assess the diagnostic accuracy of limited abdominal ultrasound (US) examination for midgut volvulus (MGV) and to evaluate how clinical practice has changed in a free-standing children's hospital leading to the near obsolescence of upper GI (UGI) studies for the diagnosis of MGV. MATERIALS AND METHODS:All pa...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2020.04.012
更新日期：2020-12-01 00:00:00
abstract：BACKGROUND:Many chronic liver diseases lead to progressive hepatic fibrosis, a condition that can ultimately result in loss of organ function and severe portal hypertension necessitating hepatic transplantation. Within the last few decades, studies have been conducted to demonstrate the possibility of drug modulation o...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2009.05.020
更新日期：2009-11-01 00:00:00
abstract：:Central venous occlusion in children is a challenging problem that can occur after a central venous catheter insertion. Long-term catheter-related complications include sepsis and venous thrombosis with consequent loss of central access. We describe 2 cases of children younger than 1 year who were dependent on a centr...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.01.076
更新日期：2008-06-01 00:00:00
abstract：BACKGROUND:Previous studies of recombinant human thrombin (rThrombin) enrolled adult and adolescent patients. This phase 4, open-label, single-group study was conducted in pediatric patients undergoing synchronous burn wound excision and skin grafting to provide information regarding the safety and immunogenicity of rT...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2011.05.022
更新日期：2011-10-01 00:00:00
abstract：BACKGROUND/PURPOSE:The purpose of this study is to describe the prenatal findings and postnatal outcomes of fetuses with axillary lymphatic malformations. METHODS:Retrospective chart review of fetuses with the prenatal diagnosis of isolated axillary lymphatic malformation detected between 2009 and 2013. RESULTS:There...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2015.03.066
更新日期：2015-10-01 00:00:00
abstract：PURPOSE:The purpose of this study was to audit the surgical training experience of a Higher Surgical Trainee in the United Kingdom during the tenure of the posts of Career Registrar, Research Fellow, and Senior Registrar from August 1992 to July 1997. Although the Report of the Working Party on the UK Specialist Medica...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90164-6
更新日期：1998-08-01 00:00:00
abstract：:Gastroesophageal reflux (GER) often develops in children who have undergone prior repair of esophageal atresia/tracheoesophageal fistula (EA/TEF). Fundoplication is necessary in many of these children. The complete wrap (Nissen) fundoplication is often used in this setting. However, poor results have been noted, with ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(97)90405-x
更新日期：1997-07-01 00:00:00
abstract：:Penile agenesis is a rare condition requiring gender reassignment and staged perineal reconstruction. This report describes two children reconstructed by taking advantage of the posterior sagittal approach. This approach allows a precise anatomic dissection, construction of a neovagina and accurate positioning of all ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(87)80712-1
更新日期：1987-12-01 00:00:00
abstract：:Surgical experience with 9 patients with chronic pancreatitis is reviewed. Vague, atypical abdominal pain and obstructive jaundice were the most common form of presentation. Serum amylase was elevated in less than half the patients. Ultrasound, ERCP, and operative cholangiopancreatography were the most helpful studies...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(82)80468-5
更新日期：1982-12-01 00:00:00
abstract：BACKGROUND:Perioperative hypothermia has been shown to increase surgical site infection (SSI) rates in adults. We sought to characterize whether intraoperative hypothermia or hyperthermia is associated with postoperative infections in infants. METHODS:We conducted a retrospective review of patients ≤6 months old who u...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.09.060
更新日期：2020-01-01 00:00:00
abstract：PURPOSE:Pediatric surgeon performed bedside ultrasound (PSPBUS) is a targeted examination that is diagnostic or therapeutic. The aim of this paper is to review literature involving PSPBUS. METHODS:PSPBUS practices reviewed in this paper include central venous catheter placement, physiologic assessment (volume status a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/j.jpedsurg.2018.04.040
更新日期：2018-11-01 00:00:00
abstract：PURPOSE:The aim of this study is to evaluate and compare the effectiveness of animated vs. non-animated biofeedback therapy in the treatment of dysfunctional voiding (DV) in the pediatric age group. METHODS:In this study, children with DV were randomly assigned for animated and non-animated biofeedback therapy. Age, v...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.jpedsurg.2017.06.002
更新日期：2018-04-01 00:00:00
