Abstract：
BACKGROUND/PURPOSE:Down's syndrome (DS) is the most common chromosomal abnormality associated with Hirschsprung's disease (HD). The purpose of this study was to review the long-term clinical outcome in patients with HD and associated DS. METHODS:Between 1975 and 2003, 39 (15%) of the 259 patients with HD had been associated with DS. Follow-up was carried out by means of examination of patient's records and personal/telephone interviews with the patient's parents or guardians. RESULTS:Twenty-six (67%) patients presented in the newborn period and 13 (33%) after the neonatal period. Twenty-eight (72%) patients had rectosigmoid HD, 10 long segment, and 1 total colonic aganglionosis. Thirty-two patients had other associated anomalies, 24 of these having cardiac anomalies. Definitive pull-through operation was performed in 33 patients. Parents of 1 child refused surgical intervention and parents of 2 children decided against pull-through operation after colostomy. Three children died before pull through. Thirteen patients had one or more episodes of enterocolitis after pull-through operation. At the time of follow-up (6 months to 28 years), 3 patients were found to have reverted to stoma because of poor bowel control or recurrent enterocolitis. Of the remaining 30 patients, 3 were lost to follow-up and 4 were too young to be assessed for bowel control. Assessment of bowel function in 23 patients revealed normal control in 8 (4 of these soiled for 6-17 years after definitive surgery), soiling in 8, and constipation requiring enemas or laxatives in 7. CONCLUSIONS:The vast majority of patients with HD associated with DS continue to have disturbances of bowel function after definitive pull-through operation.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Menezes M,Puri Pdoi
10.1016/j.jpedsurg.2005.01.048subject
Has Abstractpub_date
2005-05-01 00:00:00pages
810-2issue
5eissn
0022-3468issn
1531-5037pii
S0022346805000862journal_volume
40pub_type
杂志文章abstract：BACKGROUND/PURPOSE:Prenatal tracheal occlusion (TO) has been shown to accelerate lung growth in animal models and models of pulmonary hypoplasia. However, these models may not mimic early events in human congenital diaphragmatic hernia (CDH). The authors previously have developed a model of TO in the rat. The purpose o...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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更新日期：2001-02-01 00:00:00
abstract：BACKGROUND:Serial transverse enteroplasty (STEP) lengthens and tapers dilated bowel. Redilation of the STEP segment occurs in some patients with intestinal failure. The feasibility of a repeat STEP procedure in a pig model is evaluated. METHODS:Six pigs underwent reversal of an intestinal segment distal to the ligamen...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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abstract：BACKGROUND/PURPOSE:This study describes the pediatric inguinal hernia and hydrocele experience of a tertiary care training hospital. METHODS:A total of 3776 patients who had been operated between January 2005 and June 2009 for inguinal hernia, cord hydrocele, and hydrocele were included. The surgeries and patient foll...
journal_title：Journal of pediatric surgery
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更新日期：2013-08-01 00:00:00
abstract：PURPOSE:The Malone appendicostomy, for antegrade enemas, has improved the quality of life for many children with fecal incontinence. In patients whose appendix has been removed, a neo-appendix can be created. We describe our approach and experience with this procedure as an option for surgeons managing children with fe...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2011.03.059
更新日期：2011-06-01 00:00:00
abstract：PURPOSE:We present our experience with CDH patients who required delayed abdominal closure following CDH repair. METHODS:A retrospective review of all CDH repairs from 2004 to 2014 was performed. RESULTS:233 patients underwent CDH repair, of which 21 required delayed abdominal closure defined as the inability to clos...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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更新日期：2016-02-01 00:00:00
abstract：:The successful endoscopic drainage of a posttraumatic pancreatic pseudocyst in a 9-year-old boy is described. This case study suggests that there is a role for endoscopic placement of stents in the treatment of pancreatic pseudocysts in children. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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更新日期：1999-10-01 00:00:00
abstract：BACKGROUND/PURPOSE:This study was aimed at characterizing the structure and function of engineered fetal cartilage in vitro. METHODS:Chondrocytes from ovine specimens of fetal elastic, fetal hyaline, and adult elastic cartilage were expanded in culture and their growth rates determined. Cells were seeded onto syntheti...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.36705
更新日期：2002-12-01 00:00:00
abstract：BACKGROUND/PURPOSE:Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.10.074
更新日期：2006-01-01 00:00:00
abstract：PURPOSE:A fecalith is a fecal concretion that can obstruct the appendix leading to acute appendicitis. We hypothesized that the presence of a fecalith would lead to an earlier appendiceal perforation. METHODS:Between January 2001 and December 2005, the charts of all patients younger than 18 years old who underwent app...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.12.034
更新日期：2008-05-01 00:00:00
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journal_title：Journal of pediatric surgery
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更新日期：1980-08-01 00:00:00
abstract：PURPOSE:Although occasionally difficult, distinguishing abdominal neuroblastoma (NBL) from Wilms tumor (WT) at presentation is important, as surgical management differs significantly. We reviewed our 20-year experience (1987-2006) treating patients with NBL, focusing on those with an initial diagnosis of WT, to determi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.02.047
更新日期：2008-06-01 00:00:00
abstract：BACKGROUND/PURPOSE:Although caudal and spinal blocks are modern and safe techniques, in many centers children still are operated on under general anesthesia (GA), or the blocks are used for postoperative analgesia after GA in infraumbilical operations. The authors aimed to document the results and the complications of ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.31619
更新日期：2002-04-01 00:00:00
abstract：BACKGROUND:The organogenesis of esophageal atresia with tracheoesophageal fistula remains unclear. We have previously demonstrated that the fistula tract develops from a trifurcation of the embryonic lung bud and displays pulmonary lineage traits. Unlike the lung, the fistula grows without branching. Bone morphogenetic...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.03.052
更新日期：2006-07-01 00:00:00
abstract：:Several factors suggested to predict mortality in congenital diaphragmatic hernia (CDH) have not always been applicable in different centers. A retrospective review was conducted of 19 consecutive neonates in Singapore in whom CDH was diagnosed within 12 hours of birth to identify factors associated with mortality. Of...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(97)90104-4
更新日期：1997-01-01 00:00:00
abstract：:The purpose of this study was to determine if propranolol (0.5 mg/kg and 1 mg/kg), administered intravenously (IV) at the height of the postburn hypermetabolic response, would decrease myocardial oxygen requirements, without adversely affecting overall oxygen delivery or total body oxygen consumption. To test this hyp...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80541-x
更新日期：1989-08-01 00:00:00
abstract：PURPOSE:Small bowel transplantation (SBT) is the ultimate treatment for intestinal failure. It remains unclear as to which intestinal segment is more suitable for use in segmental SBT. The current study aims to assess the susceptibility of various parts of small intestine to ischemia and reperfusion injury and their ca...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90614-5
更新日期：1998-11-01 00:00:00
abstract：:The authors report on 2 patients with congenital chylous ascites who underwent successful lymphatic duct ligation after a laparoscopic lymphoid dye test. Fetal ascites had been detected in both cases, and both babies were born with marked abdominal swelling. Given that conservative treatment by medium-chain triglyceri...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.27973
更新日期：2001-11-01 00:00:00
abstract：:Chest wall hamartoma is a very rare mass lesion of the chest wall that is manifest at birth or in early infancy with deformity of the thoracic wall and/or varying grades of respiratory distress. The authors report on an 11-month-old infant presenting with chest wall hamartoma who was treated by en bloc excision of the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(98)90199-3
更新日期：1998-05-01 00:00:00
abstract：:Varicella (chickenpox) affects approximately 90,000 children each year. Although most cases resolve, some develop necrotizing soft tissue infections secondary to group A streptococcus and staphylococcus. Delay in diagnosis is common. At the time of initial presentation, the need for surgical intervention is not always...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(96)90103-7
更新日期：1996-08-01 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：PURPOSE:Pulmonary hypoplasia remains the principal cause of high morbidity and mortality in patients with congenital diaphragmatic hernia (CDH). The precise mechanisms causing lung hypoplasia remains unclear. Aquaporins (AQPs) are reported to constitute a family of water channels that facilitate membrane water permeabi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.10.029
更新日期：2007-02-01 00:00:00
abstract：:The authors describe the case of an inhaled foreign body unusually located in the pericardium. An initial chest skiagram was misinterpreted leading to a negative bronchoscopy. Correct anatomic localization of the foreign body only was established at surgical exploration, despite preoperative computerized tomography sc...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.23980
更新日期：2001-06-01 00:00:00
abstract：BACKGROUND:Resuscitative endovascular balloon occlusion of the aorta (REBOA) has not been studied in children. We hypothesized that REBOA was feasible and would improve hemorrhage control and survival time, compared to no aortic occlusion, in a pediatric swine liver injury model. METHODS:Pediatric swine were randomize...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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更新日期：2020-02-01 00:00:00
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journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2006.06.049
更新日期：2006-11-01 00:00:00
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journal_title：Journal of pediatric surgery
pub_type： 杂志文章
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更新日期：1995-03-01 00:00:00
abstract：:The authors report the case of a 7-month-old Japanese infant with congenital intrahepatic portosystemic shunts. He had hypergalactosaemia and hyperammonemia at age 1 month. The diagnosis was made by ultrasonography and angiography. Coil embolization was performed successfully, and then hyperammonemia and hypergalactos...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(99)90785-6
更新日期：1999-06-01 00:00:00
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journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.10.091
更新日期：2006-01-01 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
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更新日期：2013-08-01 00:00:00
abstract：BACKGROUND/PURPOSE:This study was aimed at applying fetal tissue engineering to chest wall reconstruction. METHODS:Fetal lambs underwent harvest of elastic and hyaline cartilage specimens. Once expanded in vitro, fetal chondrocytes were seeded onto synthetic scaffolds, which then were placed in a bioreactor. After bir...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(03)00265-3
更新日期：2003-08-01 00:00:00
abstract：PURPOSE:This communication is an analysis of the experience with a new type of gastrostomy. It was developed for patients with pronounced epigastric anatomical abnormalities, such as dense adhesions, in whom the conventional "open" gastrostomy could prove difficult and the percutaneous endoscopic, the imaging-guided, a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.08.043
更新日期：2008-12-01 00:00:00
