Abstract:
:Rhizomelic chondrodysplasia punctata (RCP), a rare autosomal recessive disease characterized by a disorder of peroxisome metabolism, has been shown to affect multiple organ systems. A neonate presenting with a colonic perforation in the first few hours of life was subsequently diagnosed with RCP. A literature search revealed no previous reports of intestinal perforation associated with RCP. Intestinal perforation should be added to the list of medical complications associated with RCP.
journal_name
Pediatr Surg Intjournal_title
Pediatric surgery internationalauthors
Fairbanks T,Emil Sdoi
10.1007/s00383-005-1426-5subject
Has Abstractpub_date
2005-08-01 00:00:00pages
662-4issue
8eissn
0179-0358issn
1437-9813journal_volume
21pub_type
杂志文章abstract::A neonate with a constellation of unusual congenital deformities associated with pulmonary agenesis is reported. ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050603
更新日期:1999-07-01 00:00:00
abstract::Congenital obstructive uropathy is associated with significant morbidity and mortality in the human neonate. The pathophysiology of congenital obstructive uropathy is poorly understood. There are very few experimental models of prenatal obstruction of the urinary tract, except in the fetal lamb or inbred rats. Prenata...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1322-4
更新日期:2005-03-01 00:00:00
abstract:PURPOSE:Eosinophilic gastrointestinal disorders are rare in children and present with a broad spectrum of non-specific symptoms. To date, no guidelines for diagnosis, therapy and follow-up are validated. Aim of our study is to focus on eosinophilic colitis (EC), to determine a possible correlation between associated di...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-020-04832-8
更新日期:2021-01-07 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:PURPOSE:The need for clinically applicable skin substitutes continues to be a matter of fact. Hypothetically, a laboratory grown autologous skin analog with near normal architecture might be a suitable approach to yield both satisfactory functional and cosmetic long-term results. In this study, we explored the use of h...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3808-7
更新日期:2016-01-01 00:00:00
abstract::Interposing vascularized flaps between urethra and skin sutures is recommended in hypospadias surgery to decrease the rate of urethrocutaneous fistula. For this purpose nine patients with failed results after at least one hypospadias surgery and with insufficient foreskin were included in the study. Tubularized incise...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-003-0989-2
更新日期:2003-11-01 00:00:00
abstract::Obstruction of the ureterovesical junction is an uncommon but well-recognized complication of ureteral reimplantation that traditionally has been treated by surgical correction [1, 5-9]. We report our experience with antegrade balloon dilation (ABD) of these strictures in two children. Obstruction was confirmed by diu...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00626067
更新日期:1996-10-01 00:00:00
abstract:AIM:An experimental study was performed to investigate the efficacy of colchicine in the prevention of fibrosis in the alkaline burn of the esophagus in rats. METHOD:A standard esophageal alkaline burn was produced by the application of 37.5% NaOH for a period of 90 s to the distal esophagus followed by a water rinse....
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-006-1644-5
更新日期:2006-04-01 00:00:00
abstract:PURPOSE:The purpose of this study was to test the susceptibility of human hepatoblastoma and neuroblastoma cells to photodynamic diagnostics (PDD) and photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) as a photosensitizer. METHODS:Cell cultures of human hepatoblastoma (HuH6) and neuroblastoma (MHH-NB-11) ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-008-2275-9
更新日期:2008-12-01 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:PURPOSE:The aim of this study is to identify the risk factors for esophageal anastomotic stricture (EAS) and/or anastomotic leakage (EAL) after primary repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) in infants. METHODS:A retrospective chart review of 52 patients with congenital EA/TEF between Jan...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-016-3931-0
更新日期:2016-09-01 00:00:00
abstract:PURPOSE:Pyriform sinus fistula (PSF), which originates from the third or fourth branchial pouch remnant, is relatively rare. In young children and adults, it is characterized by lateral neck infections and suppurative thyroiditis, while neonatal PSF presents as a large cervical cystic mass and causes respiratory distre...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-3008-z
更新日期:2012-01-01 00:00:00
abstract::In neonates requiring operation for necrotizing enterocolitis (NEC), the complications due to enterostomy (ES) and the need for another operation to restore continuity have prompted several surgeons to employ primary anastomosis (PA) after resection as the operative strategy of choice. Our objective was to compare pri...
journal_title:Pediatric surgery international
pub_type: 杂志文章,评审
doi:10.1007/s00383-017-4126-z
更新日期:2017-11-01 00:00:00
abstract::Internal anal sphincter achalasia (IASA) is a condition with a clinical presentation similar to Hirschsprung's disease, but with the presence of ganglion cells on rectal biopsy. The diagnosis of IASA is made on anorectal manometry, which demonstrates the absence of a rectosphincteric reflux on rectal balloon inflation...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830050552
更新日期:1999-01-01 00:00:00
abstract::A reproducible technique was developed experimentally in pigs for construction of a 'composite bowel tube' (CBT) made up of ileal mucosa that was grafted to a mucosally denuded colonic muscle surface vascularised by the colonic mesentery. Macroscopic and microscopic studies at terminal laparotomy revealed a viable, mu...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/BF00178430
更新日期:1996-04-01 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::An infant with a large-bowel obstruction due to Salmonella B enterocolitis is presented. The clinical and radiologic findings were suggestive of Hirschsprung's disease with total colonic aganglionosis. Due to further deterioration, an ileostomy was performed. Pathologic examination disclosed ganglia in the colon. At l...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003839900332
更新日期:2000-01-01 00:00:00
abstract::Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Ca...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-005-1401-1
更新日期:2005-04-01 00:00:00
abstract::We review a single surgeon and surgical centre's experience with congenital cystic adenomatoid malformation of the lung (CCAML) in relation to clinical spectrum, operative experience, and postoperative course. A retrospective hospital record review was done on surgically treated cases of CCAML over a 10-year period, f...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-004-1225-4
更新日期:2004-07-01 00:00:00
abstract::Our presentation of four cases demonstrates the essential features of limb-body wall complex (LBWC), representing a compound anomaly pattern in body-wall defects. The diagnosis of this entity is based on two of the three following characteristics: (1) exencephaly/encephalocele and facial clefts; (2) thoraco- and/or ab...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s003830000497
更新日期:2001-07-01 00:00:00
abstract:AIM:The aim of this study was to examine the relationship between birth weight, pulmonary artery (PA) size, and outcome in congenital diaphragmatic hernia (CDH) to establish if PA size has prognostic value. METHODS:The subjects for this study were 39 consecutive left-sided CDH patients treated at our institution accor...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2899-z
更新日期:2011-08-01 00:00:00
abstract::The purpose of this study is to evaluate the efficacy of intraoperative radiation therapy (IORT) and the problem of securing the IORT field in advanced pediatric neuroblastoma. Between 1996 and 2005, 12 children received IORT for advanced pediatric neuroblastoma patients. Electron beam energies ranged from 10 to 12 Me...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-007-2065-9
更新日期:2007-12-01 00:00:00
abstract::Congenital arteriovenous fistulas are a rare cause of neck mass in children, but should be considered in the differential diagnosis of paediatric patients presenting with pulsatile neck masses even when there is no history of trauma. Balloon embolisation is the treatment of choice because of the high risk of surgical ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2918-0
更新日期:2011-12-01 00:00:00
abstract::Interstitial cells of Cajal (ICCs) have been reported to play the role of a pacemaker in regulating bowel motility. The relationship between neurons and ICCs, however, remains unclear. Hirschsprung's disease (HD) is an ideal model for investigating this relationship. The operated specimens obtained from 6 short and 3 ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-002-0931-z
更新日期:2003-06-01 00:00:00
abstract:PURPOSE:Although the precise pathogenesis of ventral body wall (VBW) defects is not clearly understood, it has recently postulated that disruption of somite development during early embryogenesis may cause failure of proper VBW formation. The administration of cadmium (Cd) after 60 h of incubation induces omphalocele s...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-011-2994-1
更新日期:2012-02-01 00:00:00
abstract:PURPOSE:Human pigmented tissue-engineered skin substitutes represent an advanced therapeutic option to treat skin defects. The inflammatory response is one of the major factors determining integration and long-term survival of such a graft in vivo. The aim of the present study was to investigate the spatiotemporal dist...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3616-5
更新日期:2014-12-01 00:00:00
abstract:BACKGROUND:Pediatric surgeons and patient organisations agree that fewer centers for anorectal malformations with larger patient numbers are essential to reach better treatment. The European Union transacts a political process which aims to realize such centers of expertise for a multitude of rare diseases. All the cen...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-015-3752-6
更新日期:2015-08-01 00:00:00
abstract:PURPOSE:Ghrelin was recently reported to promote recovery from hepatic injury. We hypothesized that it could also be associated with clinical recovery of the transplanted liver from ischemia and reperfusion injury. Our aims were to investigate perioperative ghrelin changes following pediatric living donor liver transpl...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-019-04463-8
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:Retinoids are essential for fetal and lung development. Beta-carotene(BC) is the main dietary retinoid source and beta-carotene-15,15'-oxygenase-1 and 2 (Bcmo1,2) is the primary enzyme generating retinoid from BC in adult mammalian tissues. Placenta has a major role in the retinol homeostasis in fetal life: ...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-014-3621-8
更新日期:2015-01-01 00:00:00
abstract:OBJECTIVE:For esophagus tissue engineering, isolation and proliferation of esophageal epithelial cells (EEC) is a pre-requisite for scaffold seeding to create constructs. The aim of this study was to sort EEC expressing cytokeratin markers and their proliferative subpopulations; also, to investigate the viability of di...
journal_title:Pediatric surgery international
pub_type: 杂志文章
doi:10.1007/s00383-009-2512-x
更新日期:2010-01-01 00:00:00