Abstract:
PURPOSE:Hirschsprung disease (HSCR) has previously been associated with increased mortality. The aim of this study was to assess mortality in patients with Hirschsprung disease in a population-based cohort. METHODS:This was a nationwide, population-based cohort study. The study exposure was HSCR and the study outcome was death. The cohort included all individuals with HSCR registered in the Swedish National Patient Register between 1964 and 2013 and ten age- and sex-matched controls per patient, randomly selected from the Population Register. Mortality and cause of death were assessed using the Swedish National Causes of Death Register. RESULTS:The cohort comprised 739 individuals with HSCR (565 male) and 7390 controls (5650 male). Median age of the cohort was 19 years (range 2-49). Twenty-two (3.0%) individuals with HSCR had died at median age 2.5 years (range 0-35) compared to 49 (0.7%) controls at median age 20 years (0-44), p < 0.001. Hazard ratio for death in HSCR patients compared to healthy controls was 4.77 (confidence interval (CI) 95% 2.87-7.91), and when adjusted for Down syndrome, the hazard ratio was 3.6 (CI 95% 2.04-6.37). CONCLUSIONS:The mortality rate in the HSCR cohort was 3%, which was higher than in controls also when data were adjusted for Down syndrome.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Löf Granström A,Wester Tdoi
10.1007/s00383-017-4150-zsubject
Has Abstractpub_date
2017-11-01 00:00:00pages
1177-1181issue
11eissn
0179-0358issn
1437-9813pii
10.1007/s00383-017-4150-zjournal_volume
33pub_type
杂志文章,多中心研究abstract::The successful management of faecal incontinence can dramatically improve the quality of life of affected children. The introduction of the non-refluxing, catheterisable appendico-caecostomy provides the opportunity to treat previously resistant patients. Over a 6-year period, 29 children had a Malone antegrade contin...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050342
更新日期:1998-07-01 00:00:00
abstract::This article describes five cases of congenital epulis, a rare and benign swelling in the mouth of a newborn, which is not widely known. We present five cases: four cases presented as single pedunculated nodules of the gingiva and in one case two nodules were present. Of all, 50% were located at the maxilla. Excision ...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-008-2304-8
更新日期:2009-02-01 00:00:00
abstract::Reflux nephropathy (RN) is recognized as a major cause of end-stage renal failure in children and young adults. Inhibition of nitric oxide (NO) exacerbates and enhanced production ameliorates tubulointerstitial fibrosis (TIF) in experimental obstructive uropathy. NO is synthesised by NO synthase (NOS), three distinct ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0858-4
更新日期:2002-10-01 00:00:00
abstract::Three cases of neonatal chylous ascites (CCA) were managed in the neonatal unit, University Hospital, Kuala Lumpur, over the past 9 years. Fetal ascites and polyhydramnios were the sole abnormalities detected in all three babies by antenatal ultrasonography. They were born at 36 weeks' gestation and their birth weight...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/BF01372154
更新日期:1997-04-01 00:00:00
abstract:AIMS AND OBJECTIVES:Vascularized bio-engineered human dermo-epidermal skin substitutes (vascDESS) hold promise for treating burn patients, including those with severe full-thickness wounds. We have previously shown that vascDESS promote wound healing by enhanced influx of macrophages and granulocytes. Immediately follo...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4179-z
更新日期:2018-02-01 00:00:00
abstract:PURPOSE:Evaluate the management of hydrocephalus in pediatric patients in the Eastern Democratic Republic of Congo by a general surgeon. METHODS:Retrospective review of a single institution in the province of North Kivu. Patient charts and surgical notes were reviewed from 2003 to 2016. RESULTS:116 procedures were pe...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-018-4238-0
更新日期:2018-04-01 00:00:00
abstract::Oesophageal atresia (OA) is often associated with anomalies of other systems. The genetic contribution to the formation of the VACTERL association is not clear. The objective of this study was to evaluate the incidence of associated anomalies in two different racial populations. The associated anomalies in neonates wi...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830100692
更新日期:2002-05-01 00:00:00
abstract:PURPOSE:Reactive oxygen species (ROS) are thought to contribute to the pathogenesis of necrotizing enterocolitis (NEC). Mitochondria as a major source of intracellular ROS and apoptotic signaling during oxidative stress in NEC have not been investigated. We sought to determine: (1) the effects of oxidative stress on in...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2880-x
更新日期:2011-08-01 00:00:00
abstract:BACKGROUND:Intestinal adaptation in short bowel syndrome (SBS) consists of increased epithelial cells (ECs) proliferation as well as apoptosis. Angiotensin-converting enzyme (ACE) has been shown to regulate ECs apoptosis. In this study, we investigated the effect of ACE inhibition on intestinal adaptation after small b...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3075-9
更新日期:2012-05-01 00:00:00
abstract::Nonoperative management is acceptable treatment for minor pancreatic injuries. However, management of major pancreatic duct injury in children remains controversial. We present our experience in treating isolated pancreatic duct injury. We describe the cases of three male patients treated for complete pancreatic duct ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3570-2
更新日期:2014-09-01 00:00:00
abstract::Primary hydatid disease of the pancreas is rare. The authors report a 3-year-old girl who had a cystic mass in the head of the pancreas with no other viscera involved. At laparatomy the entire endocyst was totally extracted from the surrounding pancreatic parenchyma. The postoperative course was uneventful. She is cur...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050677
更新日期:1999-01-01 00:00:00
abstract:PURPOSE:The aim of the German Network for Congenital Uro-REctal malformations (CURE-Net) is to collect data of affected patients with anorectal malformation (ARM) to investigate molecular causes, clinical implications and psychosocial outcome. The current issue was to examine the transition to adulthood in adults with ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3120-8
更新日期:2012-08-01 00:00:00
abstract::A case of isolated Caroli's disease in a 2-year-old female presented as an exophytic cystic mass of the liver. A palpable mass below the right costal margin and displacement of the right colon were the main points of clinical presentation. Preoperatively, sonographic signs of intraluminal vascular tracts were identifi...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050281
更新日期:1998-03-01 00:00:00
abstract:PURPOSE:In 2006, The Japanese Society of Pediatric Endoscopic Surgeons devised a plan to develop a pediatric endoscopic surgical skill qualification (ESSQ) system. This system is controlled by The Japan Society for Endoscopic Surgery. The standard requirement for skills qualification is the ability of each applicant to...
journal_title:Pediatric surgery international
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00383-011-2871-y
更新日期:2011-07-01 00:00:00
abstract::Since January 2006, robotic assistance has been used for performing minimal invasive laparoscopic fundoplications in children. These patients were compared with those operated on with either the open surgical technique or the laparoscopic procedure. The first six children operated on with a fundoplication using the op...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1817-2
更新日期:2007-02-01 00:00:00
abstract:PURPOSE:The goal of this study was to determine the degree of consensus in the management of spinal cord tethering (TC) in patients with anorectal malformation (ARM) in a large cohort of European pediatric centers. METHODS:A survey was sent to pediatric surgeons (one per center) members of the ARM-Net Consortium. RES...
journal_title:Pediatric surgery international
pub_type: 杂志文章,多中心研究
doi:10.1007/s00383-017-4105-4
更新日期:2017-08-01 00:00:00
abstract:PURPOSE:Adnexal torsion is a gynecologic emergency, requiring intervention for tissue preservation. At our institution, torsion is managed by pediatric surgeons or gynecologists. We evaluated differences between specialties to streamline evaluation for children with gynecological emergencies, develop a clinical pathway...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-020-04782-1
更新日期:2021-01-01 00:00:00
abstract:INTRODUCTION:Applicants in the NRMP for pediatric surgery have little objective data available regarding factors predicting successful matching. We analyzed data from applicants at our institution to attempt to identify parameters correlated with three outcomes: successfully matching, or attaining either a top ten or t...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2912-6
更新日期:2011-11-01 00:00:00
abstract::Hirschsprung disease is the result of aganglionosis of a variable length of the terminal bowel, which arises from the incomplete colonisation of the embryonic gut by vagal neural crest-derived cells (NCC) that migrate caudally from the pharyngeal gut to the rectum. We have previously shown that a very small group of N...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0868-2
更新日期:2002-10-01 00:00:00
abstract:PURPOSE:Historically, the majority of patients with colonic atresia have been managed by ostomy creation at the time of initial operation; conversely, we have tended to treat the atresia with resection and anastomosis. The purpose of this study is to determine the rate of concomitant Hirschsprung's disease and to ident...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2401-3
更新日期:2009-07-01 00:00:00
abstract::The factors that affect survival in patients with stage 4 neuroblastoma vary. Several prospective and retrospective studies have provided conflicting conclusions regarding the benefit of combining aggressive chemotherapy with complete surgical resection. We analyzed our experience to evaluate the effect of complete su...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1353-x
更新日期:2005-02-01 00:00:00
abstract:INTRODUCTION:Hepatoportoenterostomy (HPE) with the Kasai procedure is the treatment of choice for biliary atresia (BA) as the initial surgery. However, the appropriate level of dissection level of the fibrous cone (FC) of the porta hepatis (PH) is frequently unclear, and the procedure sometimes results in unsuccessful ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3799-4
更新日期:2015-12-01 00:00:00
abstract::Colonic atresia and choledochal cyst are rare congenital abnormalities and there are few cases of their coexistence reported in the literature. We report a case of choledochal cyst and colonic atresia in a term neonate. Our prenatal ultrasonographic and intraoperative findings support the idea that these two entities ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2280-z
更新日期:2009-01-01 00:00:00
abstract:PURPOSE:To date, no detailed study of the changes in the image-defined risk factors (IDRFs) after neoadjuvant chemotherapy has been performed. The aim of this study was to investigate the effect of chemotherapy on IDRFs for stage L2 neuroblastomas. METHODS:Fifteen stage L2 patients treated by neoadjuvant chemotherapy ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3858-5
更新日期:2016-03-01 00:00:00
abstract::Liver transplantation (LT) for malignancy has had disappointing long-term results due to tumour recurrence. Ex-vivo dissection and auto-transplantation have had poor results when the tumor was obstructing bile ducts. Advances in liver surgery have made extensive liver resection safer, but cases of "unresectable" tumou...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000531
更新日期:2001-07-01 00:00:00
abstract::The Malone antegrade enema (MACE) and the caecostomy button (CB) are two methods of achieving colonic lavage in constipated children with faecal soiling. We reviewed our experience with the MACE and CB, aiming to compare results, complications, and outcomes. Between June 1998 and August 2002, 37 children (15 boys) und...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1220-9
更新日期:2004-07-01 00:00:00
abstract::Perineal-mound (PMD) and genital-fold defects cause anorectal malformations (ARM) in both sexes. They are common in females and usually present as a low anomaly (except rectovestibular fistula). They are rare in males and present as an intermediate anomaly. A common embryological explanation for these defects with var...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s003830050562
更新日期:1999-01-01 00:00:00
abstract::Polyorchidism is defined as the presence of more than two testes. We report the case of a 3-year-old boy and review the embryology and surgical management of the condition. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050247
更新日期:1998-01-01 00:00:00
abstract:PURPOSE:The loss of normal anatomic barriers in neonates with congenital diaphragmatic hernia (CDH) can predispose children to gastroesophageal reflux (GER). In an attempt to improve post-operative feeding, we have added a modified anterior fundoplication to restore natural gastric and esophageal positioning. METHODS:...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2411-1
更新日期:2009-08-01 00:00:00
abstract::A boy with a history of esophageal atresia repair received an esophagoesophagostomy at 14 months for a severe refractory anastomotic stricture, which had already required repeated balloon dilation with intralesional dexamethasone injection. Anastomotic leakage and stricture was again evident after the second surgery, ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2367-1
更新日期:2009-06-01 00:00:00