Abstract：
:Fourteen patients with Mixed Gonadal Dysgenesis who presented as infants or children are discussed. Gonadal asymmetry, and/or sex chromosomal mosaicism, as well as retained Mullerian Ducts characterize the anomaly. The dysgenetic testis may occur as the result of a cascade of development mishaps stemming from abnormalities of H-Y antigen expression or function that lead to abnormal differentiation of the indifferent urogenital ridge and, in turn, to aberrant production of Mullerian inhibiting Substance and testosterone. The latter two cause retention of Mullerian ducts and incomplete masculinization of the external genitalia. Absence of a second X chromosome may lead to the formation of a streak ovary, in which the dysgenetic testis may invoke formation of hilar and medullary cords. Neoplastic transformation, so characteristic of this group of patients, may result from unprotected germ cells and abnormally high and prolonged gonadotropin stimulation. Gonadoblastoma and seminoma-dysgerminomas are the tumors found in the gonads with the risk exceeding 50% as the third decade is approached. Laterality of the gonads in this anomaly remains an enigma. The gonads should be removed at birth if possible and the external genitalia repaired soon thereafter. These patients should be raised as females. The risk of neoplastic transformations must be considered at all stages of management.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Donahoe PK,Crawford JD,Hendren WHdoi
10.1016/s0022-3468(79)80486-8subject
Has Abstractpub_date
1979-06-01 00:00:00pages
287-300issue
3eissn
0022-3468issn
1531-5037pii
S0022346879000531journal_volume
14pub_type
杂志文章abstract：:The location anomaly of the verumontanum has not been described in the literature. We confirmed that the verumontanum was located either in the sphincteric or bulbous urethra in 61 of 477 patients with hypospadias (12.8%) through endoscopic examination and urethorgraphy. There was a patient with bilateral cryptorchidi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(91)90157-o
更新日期：1991-07-01 00:00:00
abstract：BACKGROUND:Single visit surgery (SVS) consists of same-day pre-operative assessment and operation with telephone post-operative follow-up. This reduces family time commitment to 1 hospital trip rather than 2-3. We began SVS for ambulatory patients with clear surgical indications in 2013. We sought to determine family s...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2017.10.021
更新日期：2017-10-10 00:00:00
abstract：:The ideal surgical closure for large omphaloceles remains controversial. Most techniques described advocate removal of the amniotic sac prior to repair of the abdominal wall defect. Herein, the authors describe a surgical technique preserving the amniotic sac, which has distinct advantages in achieving safe and rapid ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(86)90013-8
更新日期：1986-12-01 00:00:00
abstract：BACKGROUND:Inguinal hernia repair in the small infant is often technically difficult. An alternative operative approach has been developed that can simplify troublesome repairs, while decreasing the potential risks of damage to cord structures and of recurrence. METHODS:Thirteen small infants (weight 1400-3000 grams) ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(00)90034-4
更新日期：2000-02-01 00:00:00
abstract：PURPOSE:The goal of this study was to identify the proportion of sequestrations that were atypical or associated with other entities, such as congenital cystic adenomatoid malformations, communicating bronchopulmonary foregut malformations, bronchogenic cyst, and scimitar syndrome. METHODS:All charts of patients with ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.22961
更新日期：2001-05-01 00:00:00
abstract：BACKGROUND/PURPOSE:Symptomatic pancreatic pseudocysts have traditionally been managed with surgical, percutaneous, and, more recently, endoscopic drainage. Although the role of the latter is well defined in the adult population, its utility in children needs to be clarified. The authors reviewed their experience with e...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.03.071
更新日期：2004-07-01 00:00:00
abstract：PURPOSE:Despite the reported value of early video-assisted thoracoscopic surgery (VATS) for empyema, many children are still referred to the surgeon late in the disease process. The authors wished to determine the optimal management strategy for this group of children. METHODS:Medical records of all children (n = 70) ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.30828
更新日期：2002-03-01 00:00:00
abstract：PURPOSE:SIX2 and CITED1 are transcriptional regulators that specify self-renewing nephronic progenitor cells of the embryonic kidney. We hypothesized that SIX2, which promotes and maintains this stem cell population, and CITED1 remain active in Wilms' tumor (WT). METHODS:To evaluate expression domains and the pathogen...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2012.03.034
更新日期：2012-06-01 00:00:00
abstract：BACKGROUND/PURPOSE:Colonic injuries are rare in childhood, but when they do occur, they are mostly associated with penetrating abdominal injuries. The primary repair of colon injuries without stoma is still controversial within surgical experience, and the potential risk factors affecting morbidity and mortality is not...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2000.19262
更新日期：2000-12-01 00:00:00
abstract：:Pulmonary hydatid disease is prevalent in many parts of world including India. In our small series of 5 pediatric patients, all patients were diagnosed with clinical and radiological findings on plain X-ray chest and CT (Computed Tomography) chest. All of them received oral albendazole 10 mg/kg/day soon after diagnosi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2008.11.029
更新日期：2009-04-01 00:00:00
abstract：BACKGROUND:The Nuss procedure corrects pectus excavatum by forceful displacement of the sternum with metal bars. Optimal pain management remains a challenge. Mutimodal anesthesia alleviates pain through blockade of multiple nociceptive and inflammatory pain receptors. METHODS:A retrospective chart review of anesthetic...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2015.10.084
更新日期：2016-04-01 00:00:00
abstract：:During the period between 1974 and 1988 44 patients were treated for congenital intrinsic duodenal obstruction using a diamond-shaped anastomosis (35 atresias, including two multiple atresias and nine stenoses). Neither gastrostomy nor transanastomotic tube was used. Postoperatively, oral feeding was started on days 2...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(90)90241-z
更新日期：1990-09-01 00:00:00
abstract：BACKGROUND:Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to i...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2016.04.021
更新日期：2016-11-01 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：:The purpose of this study was to analyze the preliminary results obtained with endoscopic treatment of children with chronic idiopathic pancreatitis. The disease appears to be caused by a deficit of pancreatic stabilizing proteins that leads to precipitation of solutes contained in the pancreatic secretions; these pre...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(96)90837-4
更新日期：1996-10-01 00:00:00
abstract：:This paper reviews the past 25 years of experience with these lesions at The Hospital for Sick Children, Toronto, during which 19 infants and children were treated. There were 8 females and 11 males. Nine of these patients were under 1 year of age at presentation, 5 were between the ages of 1 and 10 years, 5 were olde...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(84)80096-2
更新日期：1984-10-01 00:00:00
abstract：PURPOSE:This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome. METHODS:An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.12.022
更新日期：2008-05-01 00:00:00
abstract：:Neuroblastomas located in the apex of the hemithorax or in the lower cervical region may make complete resection via a cervical or a thoracic approach difficult. The authors recently managed two patients with cervicothoracic neuroblastomas through an approach using the trap-door incision often applied in the setting o...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90127-2
更新日期：1995-04-01 00:00:00
abstract：BACKGROUND/PURPOSE:Over the last 20 years, hepatocyte transplantation (HcTx) has advanced from the experimental to the clinical stage. To date, HcTx has been performed in 30 patients in the United States. Regardless whether hepatocytes are transplanted into the spleen and migrate to the liver or are injected directly i...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.04.020
更新日期：2004-08-01 00:00:00
abstract：:The most common site of teratomas in neonates is in the sacrococcygeal region. Herein the authors describe a congenital teratoma, and to their knowledge it is the first reported case of vulvar site in a newborn. Tumor was removed on the fourth day of life, and she was completely normal 18 months after the operation. B...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.22302
更新日期：2001-04-01 00:00:00
abstract：:Two newborns with intestinal obstruction of the terminal ileum without mucoviscidosis were cured by Gastrografin enema. A further case of a premature infant showed, at autopsy, a large intestine containing normal meconium, but an ileum which was plugged with tenacious meconium containing PAS-positive and alcian blue-n...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(78)80308-x
更新日期：1978-10-01 00:00:00
abstract：INTRODUCTION:When creating a continent catheterizable channel (CCC) the choice of bowel segment used as the conduit should be tempered with the morbidity associated with it. The split-appendix technique allows the creation of both a urinary and fecal CCC without the need for a bowel anastomosis. However, there is conce...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2020.11.002
更新日期：2020-11-13 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：BACKGROUND/PURPOSE:The authors have shown previously that surgical specimens from infants with acute necrotizing enterocolitis (NEC) show upregulation of inducible nitric oxide (NO) synthase (iNOS) and interferon-gamma mRNA. However, the contribution of other inflammatory cytokines such as interleukin-8 (IL-8), IL-11, ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2001.25726
更新日期：2001-08-01 00:00:00
abstract：:Since 1978, six boys with prostatic rhabdomyosarcoma have been treated at our institution. Three had localized disease and were managed by initial biopsy, vincristine, actinomycin-D, and cyclophosphamide (VAC) chemotherapy, and bladder-sparing surgery with or without irradiation. Further combination chemotherapy ("pul...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80196-4
更新日期：1989-10-01 00:00:00
abstract：:Solid pseudopapillary tumors of the pancreas (SPTP) are very rare, and an SPTP arising in a pancreatic rest has been reported only 4 times previously and never in association with the jejunum. We report this unusual case of a 16 year old girl who presented with 4 days of intermittent, crampy abdominal pain and was fou...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2009.01.074
更新日期：2009-04-01 00:00:00
abstract：:A 95% pancreatectomy became the treatment of choice for persistent hyperinsulinemic hypoglycemia of the neonate (PHHN, Nesidioblastosis) at the author's institution, when lesser resections failed to prevent hypoglycemia in 25% to 50% of cases. With few outcome data available in the literature, the authors reviewed the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(97)90207-4
更新日期：1997-02-01 00:00:00
abstract：:From 1973 to 1988, 105 children with renal failure (average age, 6.5 years; range, 5 days to 16.2 years) were treated with hemodialysis via surgically implanted Scribner shunts (n = 120). The average duration of dialysis was 16 days (range, 1 to 194 days). Twenty-three patients died during treatment or during the hosp...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90153-8
更新日期：1994-11-01 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：PURPOSE:Beginning in 2003, the pediatric emergency medicine (PEM) physician replaced the surgeon as the team leader for all level II trauma resuscitations at a busy pediatric trauma center. The purpose was to review the outcomes 10 years after implementing this practice change. METHODS:Trauma registry data for all lev...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2014.10.041
更新日期：2015-01-01 00:00:00
