Abstract：
:Testicular feminization (TF) is a syndrome due to androgen insensitivity. It occurs in a complete (CTF) and an incomplete (ITF) form. We have treated 21 patients with TF over the last 24 years. Eight patients presented because of ambiguous genitalia, seven presented as "females" with inguinal hernia and testes were found at surgery, five were diagnosed by karyotyping performed for a family history of TF, and one presented with an incarcerated hernia and primary amenorrhea. Two patients had prior surgery for inguinal hernia but the diagnosis was not recognized. All patients had a 46,XY karyotype. Patients with CTF were phenotypically female while those with ITF had a variable appearance of the external genitalia depending on the degree of androgen insensitivity. Seventeen patients underwent gonadectomy and one patient planned for delayed gonadectomy was lost to follow-up. Seventeen patients had been raised as females since birth. One patient with ambiguous genitalia, who was initially raised as a male, was reassigned female gender at 1 year of age when the diagnosis of ITF was made. Three patients were raised as males even after the diagnosis of ITF was made. Patients raised as males underwent multiple genital reconstructive procedures with poor results. In view of the poor anatomic and functional results of genital reconstructive surgery and the consequent psychological problems, patients with TF should be raised as female. Careful evaluation of infants with ambiguous genitalia and documentation of absent fallopian tubes in "females" presenting with inguinal hernia will lead to early diagnosis of TF, correct sex assignment, and early gonadectomy.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Shah R,Woolley MM,Costin Gdoi
10.1016/s0022-3468(05)80110-1subject
Has Abstractpub_date
1992-06-01 00:00:00pages
757-60issue
6eissn
0022-3468issn
1531-5037pii
S002234689200263Xjournal_volume
27pub_type
杂志文章abstract：BACKGROUND/PURPOSE:Multisystem organ failure (MSOF) is a major cause of morbidity and mortality in the critically ill patient. Animal models of endotoxin-induced sepsis were used to develop therapeutic regimens, which thus far have failed in clinical trials. Because multiple etiologies of MSOF affect the intestine, the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.11.045
更新日期：2005-03-01 00:00:00
abstract：:A lateral skin-flap perineoplasty has been used in 15 patients born with an absent anal canal. The aim behind the introduction of this modification was the prevention of mucosal prolapse. On early assessment, this has been achieved in 11 of the cases. This represents a 50% improvement on our previous results. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(84)80108-6
更新日期：1984-10-01 00:00:00
abstract：:Since ulcerative colitis is primarily a mucosal disease, total colectomy with rectal mucosectomy and ileo-endomuscular pull-through (Soave) offers many theoretical advantages. The diseased colon is removed, the anal sphincteric mechanism is preserved, and permanent ileostomy is avoided, bladder and sexual function are...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(81)80346-6
更新日期：1981-04-01 00:00:00
abstract：:Experimental aganglionosis was produced successfully in a colonic segment proximal to the peritoneal reflection by intraluminal filling under tension of the colorectum of rats with 0.01% corrosive sublimate in normal saline solution. Where the length of aganglionic segment was more than 3 cm, ileus appeared and megaco...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(75)80089-3
更新日期：1975-12-01 00:00:00
abstract：:The informed consent process for emergency surgery in children poses a challenge for pediatric surgeons because the child and his/her surrogates (usually parents) must make medical decisions in a relatively short period. The unique circumstances of a surgical emergency create potential barriers to achieving the centra...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/j.jpedsurg.2005.05.019
更新日期：2005-08-01 00:00:00
abstract：:A 3-mo-old female presented with growth retardation, vomiting, reflux esophagitis, recurrent aspiration pneumonias, and was found to have megaesophagus and microgastria. After the failure of conservative therapy a double-lumen jejunal (Hunt-Lawrence) pouch with distal Roux-en-Y anastomosis was anastomosed to the stoma...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(80)80298-3
更新日期：1980-12-01 00:00:00
abstract：:A newborn boy was brought to us, 2 hours after birth, with a mucosal-lined left hemiperineal lesion associated with classical bladder exstrophy and an anterolaterally displaced anus. Perineal anatomy was restored by excising the mucosa lined lesion. The bladder closure for classical bladder exstrophy was done at the s...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.03.012
更新日期：2010-07-01 00:00:00
abstract：:Mesenchymal hamartoma of the liver is the second most common benign liver tumor of childhood. Ultrasonography demonstrates a cystic lesion that may be very large. Computed tomographic scan will further establish the location and its relationship to vascular anatomy. Most will be amenable to gross total resection with ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.09.029
更新日期：2009-02-01 00:00:00
abstract：BACKGROUND/PURPOSE:This study was performed to assess the long-term follow-up of five patients who underwent one-stage ileoendorectal pull-through with right colon onlay patch for total colonic aganglionosis (TCA) at Kaiser Permanente Medical Center. METHODS:A retrospective review of inpatient and outpatient charts an...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(97)90447-4
更新日期：1997-11-01 00:00:00
abstract：PURPOSE:"Early on-ECMO" repair of CDH entails repair within 48-72 h of cannulation in an effort to optimize pulmonary physiology, shorten ECMO duration, and, ultimately, improve survival. This study evaluated the effect of early on-ECMO repair as compared to leaving patients unrepaired during ECMO. METHODS:The CDH Stu...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.01.063
更新日期：2019-10-01 00:00:00
abstract：OBJECTIVES:The objectives of the study were to provide a review of patients with giant omphalocele managed in a single institution (2001-2006), focusing on medical management in the neonatal period, and to evaluate short-term outcomes. METHODS:Data from 14 neonates with giant ompholocele (abdominal wall defect >5 cm a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.04.011
更新日期：2010-08-01 00:00:00
abstract：:A 21-year-old female underwent a Kasai hepatoportoenterostomy with Roux-en-Y reconstruction for typeIII biliary atresia at age 63 days. At the age of 19 years, she developed cholangitis and CT scan revealed hepatolithiasis. She presented for treatment of the intrahepatic stone and the hepatportoenterostomy was directl...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2016.06.019
更新日期：2016-09-01 00:00:00
abstract：:DiGeorge Syndrome (DGS) is a congenital disorder that affects the thymus, parathyroid glands, and heart and brain. Thymus involvement in DGS may vary between absence/hypoplasia of thymus to various forms of reduced T cell function. TBX1 deficiency causes a number of distinct vascular and heart defects, suggesting mult...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(03)00295-1
更新日期：2003-08-01 00:00:00
abstract：BACKGROUND:Long-term dysphagia occurs in up to 50% of repaired esophageal atresia and tracheoesophageal fistula (EA/TEF) patients. The underlying factors are unclear and may include stricture, esophageal dysmotility, or associated anomalies. Our purpose was to determine whether structural airway abnormalities (SAA) are...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2017.12.025
更新日期：2018-09-01 00:00:00
abstract：BACKGROUND/PURPOSE:The aim of the study was to explore long-term orthopedic outcomes in patients with epispadias and bladder exstrophy (BE). METHODS:Sixty-three adult patients with epispadias or BE were mailed the Modified Nordic Musculoskeletal Questionnaire, of which 33 (52%) responded. The patients were reviewed fo...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2012.04.023
更新日期：2012-10-01 00:00:00
abstract：:The occurrence of malignant schwannoma in children is rare. A search of English language literature did not reveal involvement of cranial nerve among children. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(86)80090-2
更新日期：1986-11-01 00:00:00
abstract：PURPOSE:The aim of the study was to report the experience with renal cell carcinoma (RCC) in children. MATERIALS AND METHODS:We conducted a retrospective study on renal adenocarcinoma cases treated in our center between 1993 and 2008. The age limit was established at 15 years. We collected data on personal history, di...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2009.02.073
更新日期：2009-09-01 00:00:00
abstract：BACKGROUND:The increasing prevalence of obesity has necessitated the increasing use of bariatric surgery in the adolescent population. Outcomes following laparoscopic sleeve gastrectomy (LSG) among adolescents, however, have not been well-studied. We report outcomes following LSG as a first-line surgical therapy in pat...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2016.08.023
更新日期：2017-04-01 00:00:00
abstract：OBJECTIVE:The repair of pectus excavatum with bar placement is associated with substantial postoperative pain. Optimal pain control strategy has not been addressed with level 1 or substantial level 2 evidence. Many institutions operate under the assumption that a thoracic epidural offers the best pain control for these...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.09.024
更新日期：2008-01-01 00:00:00
abstract：PURPOSE:The purpose of this report is to detail the nutritional sequelae seen in survivors of congenital diaphragmatic hernia (CDH) followed in a multidisciplinary clinic. METHODS:Data on 121 surviving CDH patients seen between 1990 and 2000 were collected. Regression analysis was used to determine the impact of facto...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.25746
更新日期：2001-08-01 00:00:00
abstract：:The authors report an unusual case of bilateral short term acute orchitis in a patient with recurrent polyserositis (familial Mediterranean fever). ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90428-x
更新日期：1995-10-01 00:00:00
abstract：BACKGROUND/PURPOSE:Synoptic, or standardized, reporting of surgery and pathology reports has been widely adopted in surgical oncology. Patients with Hirschsprung disease may experience morbidity related to surgical factors or underlying pathology and often undergo multiple operations. Our aim is to improve the postoper...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/j.jpedsurg.2019.03.010
更新日期：2019-10-01 00:00:00
abstract：:There is evidence that gastrostomy can induce gastroesophageal reflux (GER). We used pH monitoring in piglets to evaluate GER after gastrostomy and to assess the effect of anterior gastropexy. Oesophageal pH studies were performed before and after gastrostomy with (8) and without (9) anterior gastropexy. The reflux sc...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(91)90706-y
更新日期：1991-05-01 00:00:00
abstract：PURPOSE:This communication is an analysis of the experience with a new type of gastrostomy. It was developed for patients with pronounced epigastric anatomical abnormalities, such as dense adhesions, in whom the conventional "open" gastrostomy could prove difficult and the percutaneous endoscopic, the imaging-guided, a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.08.043
更新日期：2008-12-01 00:00:00
abstract：PURPOSE:Though growth in children's surgical expenditures has been documented, procedure-specific differences in volume and costs at children's hospitals (CH) and non-hildren's hospitals (NCH) have not been explored. Our purpose was to compare trends in volume and costs of common pediatric surgical procedures between C...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2017.11.053
更新日期：2018-08-01 00:00:00
abstract：OBJECTIVE:Our objective was to analyze performance of noninvasive markers for significant esophageal varices in relation to outcomes of endoscopic surveillance and primary prophylaxis in biliary atresia (BA). METHODS:This was a prospective follow-up study of a national cohort of BA patients born between 1989 and 2017,...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2020.06.017
更新日期：2020-12-01 00:00:00
abstract：:Intrapericardial diaphragmatic hernia is a very rare phenotype of neonatal diaphragmatic hernia which is thought to be caused by the developmental failure of the septum transversum. There have been only 10 cases reported since 1980, and among them, only 2 cases were diagnosed in fetal life. We herein report a new case...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.07.044
更新日期：2005-11-01 00:00:00
abstract：BACKGROUND/PURPOSE:The aim of this study was to review evidence-based literature addressing pertinent questions about venous thromboembolism (VTE) after traumatic injury in children. METHODS:Data were obtained from English-language articles identified through Pubmed published from 1995 until November 2012, and from bi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/j.jpedsurg.2013.02.059
更新日期：2013-06-01 00:00:00
abstract：:The leading cause of death in the neonatal period is respiratory failure, and extracorporeal membrane oxygenation (ECMO) is an effective means of improving survival in select patients. Only neonates with severe hypoxia and acidosis are treated with ECMO. To determine the developmental outcome of the neonates we suppor...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(05)80162-9
更新日期：1990-01-01 00:00:00
abstract：BACKGROUND:Pancreatic islet cell tumors are rare in adolescents, and most studies published to date focus on older patients. We utilized a national database to describe the histology and clinical pattern of pancreatic islet cell tumors in adolescent and young adult (AYA) patients, and to compare AYAs to older adults. W...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.01.060
更新日期：2019-10-01 00:00:00
