Abstract：
:Hirschsprung disease (HSCR) is a fairly well understood congenital, genetically based functional obstruction due to the congenital absence of ganglion cells in the distal bowel. However, although over 90% of Hirschsprung cases conform to the normally accepted histological diagnostic criteria, it has become increasingly clear that in addition to HSCR, there is a group of functional disturbances relating to a number of other congenital neurodysplastic conditions causing some degree of gastrointestinal tract malfunction. Although these represent a variety of possibly separate conditions of the enteric nervous system, this spectrum it would appear to be also influenced by similar developmental processes. The term "variant Hirschsprung" is commonly used to describe these conditions, but ganglion cells are mostly present if abnormal in number and distribution. These conditions are a problem group being amongst the most difficult to diagnose and treat with possible practical and legal consequences. The problem appears to be possibly one of definition which has proven difficult in the relative paucity of normal values, especially when correlated to age and gestation. It is the purpose of this paper to review the current position on these conditions and to explore possible shared common pathogenetic and genetic mechanisms. This article explores those conditions where a similar pathogenetic mechanisms to HSCR can be demonstrated (e.g. hypoganglionosis) as well as other neural features, which appear to represent separate conditions possibly linked to certain syndromes.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Moore SWdoi
10.1007/s00383-016-4038-3subject
Has Abstractpub_date
2017-03-01 00:00:00pages
285-298issue
3eissn
0179-0358issn
1437-9813pii
10.1007/s00383-016-4038-3journal_volume
33pub_type
杂志文章,评审abstract：:We present the case of a 14-year-old male with a history of small bowel transplantation for long segment Hirschsprung's disease who underwent Duhamel ileorectal pull-through procedure. In post-transplant, the patient had no restrictions and was not TPN-dependent. To improve his quality of life, he and his family were ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-017-4216-y
更新日期：2018-01-01 00:00:00
abstract：:Adrenocortical tumors (ACT) are rare in childhood. Determination of malignant potential is difficult. To assess the presentation, clinical behaviour, and histology of these tumors in our center, the records of nine patients with ACTs between 1989 and 2000 were analyzed. The age, sex, clinical presentation, investigati...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-002-0727-1
更新日期：2003-08-01 00:00:00
abstract：:Mucosa-associated lymphoid tissue (MALT) lymphomas comprise a group of indolent B-cell non-Hodgkin lymphomas (NHL), which are rare in pediatric age. The clinical presentation of MALT lymphomas varies according to the location of the lymphoma. We report on a case of MALT lymphoma involving the appendix in a 6-year-old ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-007-1957-z
更新日期：2008-04-01 00:00:00
abstract：BACKGROUND:Children with intussusception require rapid and accurate diagnosis to enable timely intervention for satisfactory outcome. Ultrasonography is the recommended standard diagnostic modality; however, abdominal radiography (AR) is still used as an initial investigation. The aim of this study was to investigate t...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-015-3817-6
更新日期：2016-01-01 00:00:00
abstract：UNLABELLED:Vascular trauma is uncommon in children but may be complex. Arterial injury in children presents a challenge to the surgeon and may have long-term implications. Children have major advantage of a rich-collateral circulation but are still growing and developing. Decisions about when to operate remain an issue...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-011-3033-y
更新日期：2012-04-01 00:00:00
abstract：:Accessory hepatic tissue, unlike accessory splenic and pancreatic tissue, is rare and has a limited and almost exclusively sub diaphragmatic distribution. Fewer than ten cases of intrathoracic ectopic liver have been reported so far in the literature. This paper reports a case that caused a diagnostic dilemma. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章,评审
doi：10.1007/s003830000520
更新日期：2001-07-01 00:00:00
abstract：:The presentation of congenital diaphragmatic hernia (CDH) at birth may fall outside the typical features (cyanosis, tachypnea and respiratory failure), manifesting, instead, also with others pictures that make the diagnosis difficult or even impossible. We report a case of CDH presenting as a pneumothorax and a perfor...
journal_title：Pediatric surgery international
pub_type： 杂志文章,评审
doi：10.1007/s00383-007-2000-0
更新日期：2008-03-01 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：:A simple, quick, painless, and versatile dressing that fulfills most of the criteria of an ideal hypospadias dressing is described. ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-004-1206-7
更新日期：2004-05-01 00:00:00
abstract：:Evolution of the present-day policy of conservative management of ruptured spleen has been hailed as one of the most notable advances in pediatric surgery. Until 1971, routine splenectomy used to be the sacrosanct treatment for splenic trauma. It was universally believed that non-operative management carried a high mo...
journal_title：Pediatric surgery international
pub_type： 历史文章,杂志文章,评审
doi：10.1007/s00383-003-0972-y
更新日期：2003-11-01 00:00:00
abstract：:Transanal one-stage endorectal pull-through (TOSEPT) has been presented as the most recent progression in treatment of Hirschsprung's disease (HD), which may be able to replace various previous techniques. This prospective study was designed to compare the efficacy and probable complications of the commonly used open ...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-008-2186-9
更新日期：2008-08-01 00:00:00
abstract：INTRODUCTION:Recent advances in laparoscopic surgery as well as increasing experience with these techniques have led to the selection of laparoscopic surgery for hemi/partial nephroureterectomy in children with a non/poorly functioning moiety in a duplex kidney. There is very little data on the long term follow-up of s...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-011-2883-7
更新日期：2011-07-01 00:00:00
abstract：:The Thomsen-Friedenreich cryptantigen (TCA) is located on the surface of all red cells, but is concealed by a layer of neuraminic acid. When bacteria that produce neuraminidase disrupt this coating, the TCA can be exposed and activated. If blood products containing antibody to the TCA are subsequently administered, ha...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050488
更新日期：1998-12-01 00:00:00
abstract：:Pseudoexstrophy is a rare variant of the exstrophy/epispadias complex, comprised of the musculoskeletal defects of classic exstrophy but with an intact bladder and urethra. We present a case of a neonate with left-sided gastroschisis and pseudoexstrophy, the first reported combination of these two anomalies. The anoma...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-004-1217-4
更新日期：2004-07-01 00:00:00
abstract：BACKGROUND/AIM:Esophageal dilatation, gastroesophageal reflux, and intestinal obstruction have been demonstrated in CDH survivors. Abnormal esophageal and intestinal innervations were recently found in rats and babies with this disease. Our aim was to further characterize these malformations in embryos and fetal rats e...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-010-2788-x
更新日期：2011-02-01 00:00:00
abstract：PURPOSE:Hepatopulmonary syndrome (HPS) is a progressive, deteriorating complication of end-stage liver disease (ESLD) that occurs in 13-47% of liver transplant candidates. Although LT is the only therapeutic option for HPS, it has a high morbidity and mortality, especially in patients with severe hypoxemia before trans...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-011-2866-8
更新日期：2011-08-01 00:00:00
abstract：PURPOSE:We investigated the associations between hsMAD2 mRNA expression in tumor cells and sensitivity to paclitaxel or patient prognosis in neuroblastoma. METHODS:Fifty-one formalin-fixed paraffin-embedded tumor samples were manually microdissected to collect tumor cells, and RNA was purified. Nineteen clinical sampl...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-010-2780-5
更新日期：2011-02-01 00:00:00
abstract：PURPOSE:We evaluated the effectiveness of prophylactic mesenteric lymphatic ligation in preventing iatrogenic chylous fistulae in abdominal neuroblastoma surgery. METHOD:All patients who underwent resection of abdominal neuroblastoma between years 2002 and 2013 were included. A change in surgical strategy commenced fr...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-014-3581-z
更新日期：2014-10-01 00:00:00
abstract：:Foreign-body (FB) aspirations account for a reasonable percentage of respiratory emergencies in young children. Whereas 67% of aspirated FBs are of organic origin, metallic-ferromagnetic FBs are observed less frequently. We report the use of a wire with a magnetic tip fitting into the working channel of a rigid bronch...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/BF01076970
更新日期：1997-07-01 00:00:00
abstract：:A 7-year-old female with a type I choledochal cyst underwent an exploratory laparotomy. A Roux-en-Y choledochojejunostomy was done following cyst excision. The patient was re-explored 8 days later for an anastomotic bile leak and high fever. A round-worm was found obstructing the jejunal lumen at the porta hepatis, re...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830050394
更新日期：1998-09-01 00:00:00
abstract：:In severe vaginal malformations, when the distance between the upper vaginal pouch and perineum is too long (6 cm or more), reconstruction of the vagina can be performed by colonic interposition or by long cutaneous flaps obtained by the tissue expansion technique. Two female adolescents were treated using expanded la...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：
更新日期：1997-02-01 00:00:00
abstract：:In spite of advances in the treatment of childhood bladder and prostate rhabdomyosarcoma (RMS), the ability to detect minimal residual disease correlates imperfectly with the ultimate outcome. We report the long-term follow-up of a child with microscopic residual RMS after chemotherapy. The correct interpretation of t...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s003830200017
更新日期：2002-01-01 00:00:00
abstract：BACKGROUND:There is a shortage of surgeons in Africa, and this shortage is particularly acute in paediatric surgery with most paediatric patients being cared for by general surgeons. The use of information technology to augment teaching in paediatric surgery in Africa is appealing but often unsuccessful due to the cost...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-010-2806-z
更新日期：2011-04-01 00:00:00
abstract：:New development in the vascular network is a significant process for the proliferation, as well as metastatic expand, of cancer cells that depends on a sufficient provider of oxygen and nutrients and the removal of waste products. New blood and lymphatic vessels form via step called angiogenesis and lymphangiogenesis....
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-013-3398-1
更新日期：2013-12-01 00:00:00
abstract：:This study deals with the advancement process of neuroblastoma through clinical observations and circulating tumor cell exploration. Clinical feature, tumor biology, and circulating tumor cell detected by the previously described polymerase chain reaction (PCR) method were analyzed in 31 patients with advanced neurobl...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-005-1503-9
更新日期：2005-11-01 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：:The Nuss procedure is the most popular technique for correction of pectus excavatum recently. Life-threatening complications associated with the procedure are very rare. We report a 13-year-old boy who developed late-onset bilateral hemothorax with hypovolemic shock 5 months after the Nuss procedure. In literature rev...
journal_title：Pediatric surgery international
pub_type： 杂志文章
doi：10.1007/s00383-011-2936-y
更新日期：2012-01-01 00:00:00
abstract：:In the published version, the Acknowledgements section was missing a funding note of co-author Dr C Verrill. The corrected version should read as follows. ...
journal_title：Pediatric surgery international
pub_type： 已发布勘误
doi：10.1007/s00383-020-04624-0
更新日期：2020-04-01 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：:Recently, most studies reported magnesium as a N-methyl-D-aspartate receptor antagonist and its analgesic and perioperative anaesthetic effects have been discussed with central desensitization pathway. We investigated the effects of caudal ropivacaine plus magnesium and compared with ropivacaine alone on postoperative...
journal_title：Pediatric surgery international
pub_type： 杂志文章,随机对照试验
doi：10.1007/s00383-006-1779-4
更新日期：2007-02-01 00:00:00
