Abstract:
:"Variants of Hirschsprung's disease" are conditions that clinically resemble Hirschsprung's disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Friedmacher F,Puri Pdoi
10.1007/s00383-013-3351-3subject
Has Abstractpub_date
2013-09-01 00:00:00pages
855-72issue
9eissn
0179-0358issn
1437-9813journal_volume
29pub_type
杂志文章,评审abstract::Negative pressure wound therapy (NPWT) is an accepted form of treatment in managing complex wounds in adults and children. It is not as widely used in the neonatal population due to limited clinical experience. We describe the application of the RENASYS™ system (Smith and Nephew, UK) in delivering NPWT to four neonate...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2868-6
更新日期:2011-08-01 00:00:00
abstract:PURPOSE:The clinical application of autologous tissue-engineered skin analogs is an important strategy to cover large skin defects. Investigating biological dynamics, such as reinnervation after transplantation, is essential to improve the quality of such skin analogs. Previously, we have examined that our skin substit...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3978-y
更新日期:2016-12-01 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::The advances in science have resulted in the emergence of nanotechnology, which deals with the design and use of tools and devices of size 1-100 nm. The application of nanotechnologies to medicine is thus termed nanomedicine. Significant research has been focused on this new and exciting field and this review article ...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-012-3162-y
更新日期:2012-10-01 00:00:00
abstract:OBJECTIVE:To compare the safety and efficacy between laparoscopic and open cyst excision with hepaticojejunostomy for children with choledochal cysts using meta-analysis. METHODS:Studies comparing the laparoscopic and the open choledochal cyst excision that met the inclusion criteria for data extraction were identifie...
journal_title:Pediatric surgery international
pub_type: 杂志文章,meta分析
doi:10.1007/s00383-015-3705-0
更新日期:2015-06-01 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::Macrophages (MPs) produce increased levels of proinflammatory cytokines in Crohn's disease; these cytokines are thought to play a central role in the occurrence of the disease. Biologics are currently available for anti-cytokine therapy, but treating intestinal inflammation through direct suppression of proinflammator...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1767-8
更新日期:2006-11-01 00:00:00
abstract:PURPOSE:Pancreatic neoplasms are uncommon in children. This study sought to analyze the clinical and pathological features of surgically resected pancreatic tumors in children and discuss management strategies. METHODS:We conducted a retrospective review of patients ≤21 years with pancreatic neoplasms who underwent su...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3925-y
更新日期:2016-08-01 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::Non-accidental trauma (NAT) represents a significant cause of morbidity and mortality in the pediatric population. The management of these patients often involves many care providers including the surgeon. Victims of NAT often present with multiple injuries and as such should be treated as trauma patients with complet...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-015-3688-x
更新日期:2015-07-01 00:00:00
abstract:BACKGROUND:The role of thymectomy in the treatment of juvenile myasthenia gravis (JMG) is poorly defined. The objective of this systematic review was to evaluate the effect of thymectomy on survival, disease severity, and peri-operative complications for patients with JMG. METHODS:A search of MEDLINE, EMBASE, and the ...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-017-4086-3
更新日期:2017-06-01 00:00:00
abstract:PURPOSE:To study the gender assignment and hormonal treatment advocated for disorders of sexual differentiation (DSD). METHODS:A study was done on patients who were reviewed in the Pediatric Intersex Clinic to evaluate the pattern of gender assignment and hormonal treatment advocated. RESULTS AND CONCLUSION:The patie...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2232-7
更新日期:2008-10-01 00:00:00
abstract::The n-3 and n-6 polyunsaturated fatty acids (PUFAs) are essential dietary constituents. They are important as a source of energy, as structural components of cell membranes, and as signalling molecules. They have been demonstrated to be potent modulators of the immune response, and research has endeavoured to optimise...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-005-1385-x
更新日期:2005-05-01 00:00:00
abstract:PURPOSE:The purpose of this study was to review the clinical characteristics and prognosis of children with adrenocortical tumors (ACT). METHODS:We retrospectively reviewed the medical records of 28 patients with ACT at our hospital between March 2010 and March 2017. RESULTS:The main clinical presentations were sexua...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-018-4409-z
更新日期:2019-03-01 00:00:00
abstract::Ovarian involvement of acute lymphoblastic leukemia (ALL) without relapse in the bone marrow or other extramedullary site is rare. We report a case of ovarian relapse in a 13-year-old girl who was diagnosed with ALL at the age of 11. A complete remission was induced with the Children Cancer Group 1882 protocol for hig...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-008-2253-2
更新日期:2008-11-01 00:00:00
abstract::A 45,X/46,Xidic(Y)(q11.2) mosaicism was found in a 4-year-old boy. The clinical appearance was characterized by bilateral cryptorchidism, penoscrotal hypospadias, short penis, and coarctation of the aorta. The latter is the only abnormality also seen in Turner syndrome. A biopsy of the gonads revealed normal prepubert...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050731
更新日期:2000-01-01 00:00:00
abstract::Port-site recurrence (PSR) following laparoscopic procedures has been an unpredictable complication in adult cancer patients; however, no data exist about this phenomenon in the pediatric field. The aim of this study was to determine whether PSR, following endosurgical procedure for malignancies, is a typical complica...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0918-9
更新日期:2003-05-01 00:00:00
abstract::Recent advances in culturing of intestinal stem cells and pluripotent stem cells have led to the development of intestinal organoids. These are self-organizing 3D structures, which recapitulate the characteristics and physiological features of in vivo intestinal epithelium. Intestinal organoids have allowed the develo...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-019-04581-3
更新日期:2020-01-01 00:00:00
abstract::The aim of this study was to determine the effects of a synthetic serine protease inhibitor, gabexate mesilate (GM), in rats with ischemia-reperfusion (I-R) damage due to unilateral testicular torsion. Thirty male Sprague-Dawley rats were separated into three groups, each containing ten rats. A sham operation was perf...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1667-y
更新日期:2006-05-01 00:00:00
abstract::Migration of particulate matter from plastic tubing and solid plastic implants has been documented in a number of studies, including some with the use of cardiac bypass, haemodialysis, and pump-assisted intravenous infusions. In order to ascertain whether silicone embolisation occurs when children have an Ivac 560 pum...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF01194802
更新日期:1997-01-01 00:00:00
abstract::Phylloides tumor is an uncommon breast tumor in children. Only a few cases have been reported in the literature. A case of borderline phylloides tumor in an 11-year-old girl is described. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050623
更新日期:1999-07-01 00:00:00
abstract::Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for almost 3 % of pediatric renal tumors. Cavoatrial tumor thrombosis is very rare and because of poor response to chemotherapy, invasive surgical interventions such as open heart surgery may be indicated. A 6-year-old girl with CCSK of righ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-012-3160-0
更新日期:2012-11-01 00:00:00
abstract::70 cases of gastroschisis (GS) were surgically treated at the Pediatric Surgical University Clinic, Münster, from 1984 through 1998. The defect occurred more frequently in males (44) than females (26). The average birth weight was 2,383 g and mean gestational age 36.8 weeks. 9 infants (12.9%) were delivered vaginally ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0799-y
更新日期:2002-09-01 00:00:00
abstract:PURPOSE:Acute respiratory distress syndrome, with the need for invasive mechanical ventilation (MV) remains a major cause of neonatal mortality and morbidity. Although venovenous extracorporeal lung support (VV-ECLS) has become a standard of care procedure in neonatal patients with acute pulmonary failure there are no ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3815-8
更新日期:2016-01-01 00:00:00
abstract:BACKGROUND:Sacrococcygeal teratoma (SCT) is a rare childhood malignancy. Although overall survival is favorable, recurrent tumors are associated with poor outcomes. As most recurrences occur within 3 years of presentation, the utility of long-term surveillance is uncertain. METHODS:Patients with SCTs evaluated and man...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-017-4132-1
更新日期:2017-11-01 00:00:00
abstract::A case of retrocecal appendicitis is described in a 6-year-old male child who presented with thigh abscess. The presence of a positive psoas stretch test, feculent discharge, an enteric growth on bacteriological examination, and intraabdominal fluid collection on abdominal ultrasound provided clues to the presence of ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1356-7
更新日期:2005-04-01 00:00:00
abstract:BACKGROUND/PURPOSE:The volume-outcome relationship and optimal surgical volumes for repair of congenital anomalies in neonates is unknown. METHODS:A retrospective study of infants who underwent diaphragmatic hernia (CDH), gastroschisis (GS), and esophageal atresia/tracheoesophageal fistula (EA/TEF) repair at US hospit...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-019-04525-x
更新日期:2019-11-01 00:00:00
abstract::We report the case of a female adolescent who had an ectopic ovary in the inguinal canal without an associated hernia, a unicornuate uterus, and ipsilateral renal agenesis. The incidental discovery of the ectopic ovary and other Mullerian anomalies, as well as the surgical correction that followed, highlights importan...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-014-3582-y
更新日期:2014-10-01 00:00:00
abstract::In the pediatric population, rectal injuries usually occur as a result of motor vehicle collisions. There has been an increased interest in selective diversion of rectal injuries in adults and increased utilization of laparoscopy both as a diagnostic and therapeutic adjunct. The aim of the study was to review our inst...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-007-1996-5
更新日期:2007-11-01 00:00:00
abstract::A testis is termed ectopic when it lies outside the normal line of descent. A case of testicular ectopia in which the testicle was located cranial and lateral to the internal ring dorsal to the external oblique muscle is reported in a 2-month-old boy. This highly aberrant ectopic testis was treated successfully by orc...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00178440
更新日期:1996-04-01 00:00:00