Abstract:
:Epidermolysis bullosa letalis has been associated with congenital pyloric atresia in an autosomal recessive syndrome. This report documents the second occurrence in premature siblings. Our review indicates that it is a lethal condition with death due to the septic complications of epidermolysis bullosa letalis. Surgical management of pyloric atresia should be withheld until the severity of the epidermolysis is known. Skin biopsy to document the type of epidermolysis bullosa is imperative as only the letalis form is documented to be lethal. The risk for subsequent siblings is 25% and parents should undergo genetic counseling. Prenatal diagnosis of epidermolysis bullosa letalis can be made by fetoscopic skin biopsy. We suggest nonoperative management of pyloric atresia unless the skin disease is responsive to treatment.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Rosenbloom MS,Ratner Mdoi
10.1016/s0022-3468(87)80248-8subject
Has Abstractpub_date
1987-04-01 00:00:00pages
374-6issue
4eissn
0022-3468issn
1531-5037pii
S0022346887001787journal_volume
22pub_type
杂志文章abstract::Major rectal bleeding may occur in children and adults who have extensive hemangiomatous involvement of the pelvis and rectosigmoid colon, as in the Kleppel-Trenaunay syndrome (KTS). Conventional surgical techniques such as bowel resection and colostomy have often been associated with large blood loss and/or incontine...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(93)90235-d
更新日期:1993-03-01 00:00:00
abstract::Histologic studies of the human chorionic gonadotropin (hCG) producing hepatoblastoma were made for seven cases encountered in Japan. It was found in all cases that multinucleated tumor giant cells in several figures were admixed in usual hepatoblastoma tissue and one of them was confirmed to be hCG positive immunohis...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80258-0
更新日期:1987-05-01 00:00:00
abstract:BACKGROUND/PURPOSE:Kasai portoenterostomy (KP) is regarded as first-line treatment for biliary atresia, although its postoperative course is often unpredictable. Hepatobiliary scintigraphy using technetium-labeled iminodiacetic acid derivatives offers a dynamic, objective assessment both of parenchymal liver function a...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2007.01.063
更新日期:2007-06-01 00:00:00
abstract::A primary lung abscess due to non type B Hemophilus influenzae was diagnosed in three children who failed to improve after prolonged antibiotic treatment for dense, pneumonic infiltrates. In each instance percutaneous aspiration yielded pure cultures of Hemophilus influenzae, one of which was ampicillin resistant. Bec...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(82)80013-4
更新日期:1982-06-01 00:00:00
abstract:PURPOSE:The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS). METHODS:A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.02.039
更新日期:2019-06-01 00:00:00
abstract::The fifteenth case of neonatal adrenal abscess is reported. It is the first case successfully diagnosed with needle aspiration and treated with percutaneous drainage. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(88)80026-5
更新日期:1988-11-01 00:00:00
abstract:PURPOSE:We conducted a nationwide survey in Japan, to identify recent trends in the diagnosis and treatment of patients who had total colonic aganglionosis with or without small bowel involvement (TCSA). These data were compared with 2 similar studies conducted within the last 30 years. METHODS:Patient data were colle...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2008.08.049
更新日期:2008-12-01 00:00:00
abstract::Six cases of prepyloric antral diaphragm were encountered at Children's Hospital Medical Center of Akron between 1965 and 1985, with approximately 150 cases reported in the literature during this time. In our series, a delay from onset of symptoms to treatment existed for a period of 4 months to 6 years. We suggest th...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80597-3
更新日期:1987-10-01 00:00:00
abstract:PURPOSE:Expandable metallic stents (Palmaz stents) have been used in the treatment of tracheobronchial obstruction in children and adults. The authors investigated their utility in the management of acute airway stenosis in a growing animal model. METHODS:A model for tracheal stenosis was developed in young lambs (mea...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(98)90527-9
更新日期:1998-07-01 00:00:00
abstract:BACKGROUND:The Malone antegrade continence enema (MACE) procedure has been previously described as a safe and effective option for the treatment of children with chronic defecation disorders when maximal medical therapy and conventional treatment have failed. PURPOSE:To evaluate clinical success, complications, and qu...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.04.050
更新日期:2011-08-01 00:00:00
abstract:AIM:The aim of the study is to report feasibility and safety of endoscopic esophageal substitution in infants with pure esophageal atresia and wide gap tracheoesophageal fistula with a minimum one year follow-up. MATERIALS AND METHODS:This prospective study was conducted from January 2012 for twenty four consecutive m...
journal_title:Journal of pediatric surgery
pub_type: 临床试验,杂志文章
doi:10.1016/j.jpedsurg.2015.08.004
更新日期:2016-03-01 00:00:00
abstract:PURPOSE:An artificial placenta (AP) utilizing extracorporeal life support (ECLS) could avoid the harm of mechanical ventilation (MV) while allowing the lungs to develop. METHODS:AP lambs (n = 5) were delivered at 118 days gestational age (GA; term = 145 days) and placed on venovenous ECLS (VV-ECLS) with jugular draina...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.06.001
更新日期:2018-10-01 00:00:00
abstract::In the past 6 years, 50 patients with interstitial emphysema in our newborn nursery were treated with open operative procedures to remove interstitial air. Thirty-one were critically ill newborns with severe progressive pulmonary interstitial emphysema treated with pleurotomies. Five underwent pleurotomies on both sid...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80010-6
更新日期:1987-01-01 00:00:00
abstract:BACKGROUND:Hyperparathyroidism (HPT) in children is rare and surgical management is supported only by limited evidence. METHODS:Retrospective case series of all children under the age of 16 years who underwent parathyroidectomy (PTx) between 1978 and 2012. RESULTS:We identified 29 children who had surgery for HPT. Si...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2014.05.032
更新日期:2014-11-01 00:00:00
abstract::A brief resume of the highlights in the history of oesophageal atresia is presented. This is followed by research into the etiology, ontogeny and embryology, and microbiological studies. A revised classification of risk factors with consequent survival statistics is presented. Lessons learned in the management of the ...
journal_title:Journal of pediatric surgery
pub_type: 历史文章,杂志文章,评审
doi:10.1016/j.jpedsurg.2006.07.004
更新日期:2006-10-01 00:00:00
abstract:BACKGROUND:In addition to trauma center levels and types, trauma volume may be an important factor impacting outcomes in severe pediatric trauma. METHODS:All severely injured pediatric patients treated at adult trauma centers were identified from the National Trauma Data Bank. All qualifying centers were stratified in...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2017.01.066
更新日期:2017-11-01 00:00:00
abstract:BACKGROUND:Several factors are implicated in transabdominal testicular descent, including insulinlike factor 3 (INSL3) hormone and Müllerian-inhibiting substance (MIS). A transgene insertional mutation found on chromosome 5 in the mouse, known as crsp, causes deletion of a transmembrane G protein-coupled receptor gene,...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2003.08.047
更新日期:2003-12-01 00:00:00
abstract::Pediatric surgeons brought forth non-operative treatment for children with blunt spleen injury more than 50 years ago. At the time, this proposal was deemed reckless by many adult surgeons, and debate ensued for decades. Despite criticisms, pediatric surgeons refined the clinical pathways for children with spleen inju...
journal_title:Journal of pediatric surgery
pub_type: 古典文章,历史文章,杂志文章,评审
doi:10.1016/j.jpedsurg.2018.10.026
更新日期:2019-01-01 00:00:00
abstract::The management of giant omphalocele remains a major surgical challenge. A staged approach is mandatory to achieve an uncomplicated reduction. The case of a child conservatively and successfully treated by progressive external compression of the herniated organs and viscera using an elastic bandaging is described here....
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(96)90061-5
更新日期:1996-12-01 00:00:00
abstract:BACKGROUND:Rapamycin can inhibit tumor growth and angiogenesis in various human cancers. Cyclooxygenase 2 (COX-2) is involved in the angiogenic process. We hypothesized that the antiangiogenic effect of rapamycin may be mediated by suppression of COX-2. METHODS:Ewing sarcoma (ES) cells were implanted in athymic mice. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2009.02.037
更新日期:2009-06-01 00:00:00
abstract:BACKGROUND:No consensus exists among surgeons regarding the optimal treatment of complicated acute appendicitis in children (CAA). Existing studies present heterogeneity of data and only few studies analyzed free perforated appendicitis (FPA) separately from appendicular abscess (AAb) and appendicular phlegmon (AP). M...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,meta分析
doi:10.1016/j.jpedsurg.2019.01.065
更新日期:2019-11-01 00:00:00
abstract:BACKGROUND:Phyllodes tumors are fibroepithelial breast lesions that are uncommon in women and rare among children. Due to scarcity, few large pediatric phyllodes tumor series exist. Current guidelines do not differentiate treatment recommendations between children and adults. We examined national guideline adherence fo...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2018.02.070
更新日期:2018-06-01 00:00:00
abstract:PURPOSE:The aim of this study was to investigate the prenatal courses and management of abdominal surgical diseases. METHODS:Of the 327 patients registered with our fetal treatment board since March 2002, 83 fetuses referred to the surgical department were enrolled for the current study. The prenatal diagnosis, sequen...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2004.08.039
更新日期:2004-12-01 00:00:00
abstract::Fifty-six patients with a pathological lesion at the leadpoint of their intussusception were encountered at the Royal Children's Hospital, Melbourne, over a 16-year period. The most common lesions were Meckel's diverticula, followed by small bowel polyps, lymphosarcomas, and duplication cysts. Age at presentation was ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(90)90353-b
更新日期:1990-06-01 00:00:00
abstract:BACKGROUND:Minimally invasive bar repair for pectus patients produces substantial pain which dictates the post-operative hospital course. We have data from 2 randomized trials comparing epidural catheter placement to patient controlled analgesia. The purpose of this study was to compare the outcomes of patients who wer...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jpedsurg.2015.05.009
更新日期:2015-11-01 00:00:00
abstract::Wandering spleen usually causes clinical symptoms by torsion. Cases of torsion or hemorrhage after blunt trauma are reported. The authors experienced avulsion of wandering spleen after traumatic torsion in the victim of pedestrian injury. The spleen had been located in the left upper quadrant on the time of torsion, b...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1053/jpsu.2003.50136
更新日期:2003-04-01 00:00:00
abstract:INTRODUCTION:We recommend orchiopexy between 9 and 18 months of age for surgical, testicular, and psychological reasons. However, in practice, we observed many patients coming to orchiopexy at a later age. To understand this difference better, we reviewed our experience with patients undergoing late orchiopexy. METHOD...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2008.03.029
更新日期:2008-09-01 00:00:00
abstract::We present the first case of Wilms' tumor, pancreatic islet cell carcinoma, and pheochromocytoma affecting the same individual. This case underscores the importance of repeat biopsies in patients with multiple neoplasms to confirm the diagnosis and guide management. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/j.jpedsurg.2007.11.006
更新日期:2008-03-01 00:00:00
abstract::Giant cavernous hemangiomas in newborns and infants can present with life-threatening complications. Although some modes of therapy are effective, there is a significant associated morbidity and mortality. The author reports two cases of complicated giant cavernous hemangiomas treated successfully with intermittent pn...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(87)80004-0
更新日期:1987-01-01 00:00:00
abstract:BACKGROUND/PURPOSE:The optimal management of neonates with gastroschisis is unclear, and there is a significant morbidity. We performed a review of neonates with gastroschisis treated at our center of pediatric surgery over the last 21 years to determine predictive factors of outcome. METHODS:Single-center retrospecti...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.07.012
更新日期:2011-11-01 00:00:00