Abstract：
:Persistent müllerian duct syndrome (PMDS) is characterized by the presence of a uterus, cervix, and fallopian tubes in an otherwise normally differentiated 46.XY male. During embryogenesis, regression of müllerian structures in normal males is mediated by antimüllerian hormone (AMH), also called müllerian inhibiting substance (MIS), produced by fetal Sertoli's cells. PMDS has been attributed to deficient AMH activity or to abnormalities in the AMH receptor. The authors report on two patients with PMDS in whom the abnormalities were discovered during surgery for inguinal hernia and cryptorchidism. During the initial operations in each case, testicular biopsies were obtained, and the gonads and müllerian elements were replaced in the pelvis. A second operative procedure, performed several months later, included proximal salpingectomies with dissection of the vasa deferentia on pedicles of myometrium. This permitted excision of the vestigial uterine corpus, leaving a tiny remnant of cervix with the vasa deferentia. The testes were further mobilized so that bilateral orchidopexies could be completed. In the first case, a molecular abnormality was present at position 377 of the first exon of the AMH gene. Thymine replaced cytosine, which altered a CGG arginine codon to a TGG tryptophan codon, rendering the AMH molecule unstable. The molecular abnormality in the first case differs from the first abnormality in AMH reported by Knebelmann et al, thus indicating heterogeneity in this condition. The molecular basis for deficient AMH activity in the second patient has not yet been defined. No molecular abnormalities were found in the exons of this patient's AMH gene.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Loeff DS,Imbeaud S,Reyes HM,Meller JL,Rosenthal IMdoi
10.1016/0022-3468(94)90525-8subject
Has Abstractpub_date
1994-01-01 00:00:00pages
61-5issue
1eissn
0022-3468issn
1531-5037pii
0022-3468(94)90525-8journal_volume
29pub_type
杂志文章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：BACKGROUND/PURPOSE:Although caudal and spinal blocks are modern and safe techniques, in many centers children still are operated on under general anesthesia (GA), or the blocks are used for postoperative analgesia after GA in infraumbilical operations. The authors aimed to document the results and the complications of ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.31619
更新日期：2002-04-01 00:00:00
abstract：BACKGROUND:Establishment of enteral nutrition is necessary after intestinal surgery. In resource-strained environments, it can be critical. This study examined the effect of early feeding in pediatric patients undergoing stoma closure in a country with mid-level socioeconomic indices. METHODS:With parenteral consent a...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2013.02.013
更新日期：2013-05-01 00:00:00
abstract：BACKGROUND:The management of giant omphaloceles (GO) can be quite difficult when there is absence of abdominal domain. Coverage with delayed closure has been described. We present a technique to create an adequate peritoneal domain. METHODS:This is a retrospective review of our experience using an intraperitoneal tiss...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.12.013
更新日期：2006-04-01 00:00:00
abstract：:Between January 1, 1969, and February 2, 1988, 331 pediatric renal transplants were performed at a single pediatric center. Of these 225 were first cadaveric allografts. Graft allocation was independent of the outcome of human lymphocyte antigen (HLA) matching. The results of class I antigen matching were analyzed in ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(89)80567-6
更新日期：1989-12-01 00:00:00
abstract：:The authors report a case of neonatal neuroblastoma mimicking Altman type III sacrococcygeal teratoma. A newborn male infant delivered after a normal pregnancy was found to have an extremely large sacrococcygeal mass. Imaging studies strongly suggested Altman type III sacrococcygeal teratoma. On the 10th day after the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2004.11.016
更新日期：2005-03-01 00:00:00
abstract：:Two infants with unusual bronchopulmonary malformations associated with congenital diaphragmatic hernia (CDH) are presented. One infant had extralobular sequestration and cystic adenomatoid malformation of the lower lobe, in addition to a left-sided CDH. The second infant had a laryngotracheoesophageal cleft extending...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(95)90772-6
更新日期：1995-06-01 00:00:00
abstract：BACKGROUND:Primary hyperparathyroidism (PHPT) is uncommon in children. The surgical management of PHPT in children has evolved over the past two decades. METHODS:A retrospective study of patients who underwent parathyroidectomy for PHPT diagnosed at age < 18 years and managed at a tertiary referral center for endocrin...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2013.11.059
更新日期：2014-04-01 00:00:00
abstract：BACKGROUND:Gastroesophageal reflux (GER) and dysmotility are frequent in patients treated for esophageal atresia (EA). This aim of this study is to evaluate GER and dysmotility in young EA patients using pH-multichannel intraluminal impedance (pH-MII). METHODS:Fifteen patients with a mean age of 7.5 years (group 1) ha...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.08.012
更新日期：2011-03-01 00:00:00
abstract：:The management of pediatric empyema remains controversial. An experimental study was undertaken to evaluate the role of bacteria in the evolution and severity of empyema, using specific bacteria that are pathogens of empyema in children. A rabbit model was used. The groups were Haemophilus influenzae (n = 9), Bacteroi...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90737-4
更新日期：1994-05-01 00:00:00
abstract：:Recognition of the buried penis is necessary both to ensure its proper management and to avoid performance of a standard circumcision, which can result in penile degloving. We describe a simple technique of unfurling the prepuce which provides adequate functional and cosmetic replacement for the penile shaft skin. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(86)90007-2
更新日期：1986-12-01 00:00:00
abstract：:Ten children among 221 with congenital cardiac anomalies developed urinary symptoms after removal of urethral catheters, which were routinely inserted for monitoring purposes during and after cardiac surgery. Six children developed urethral strictures. Poor tissue perfusion concomitant with serious congenital cardiac ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(85)80396-1
更新日期：1985-02-01 00:00:00
abstract：BACKGROUND/PURPOSE:Intestinal damage (ID) is closely related to morbidity and mortality in gastroschisis. This study was performed to determine the intraamniotic substances that may correlate ID and also to verify their time course levels that would be useful for determining when ID starts in gastroschisis. METHODS:In...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2007.05.027
更新日期：2007-10-01 00:00:00
abstract：PURPOSE:Despite advances in neonatal care of congenital diaphragmatic hernia (CDH), a significant variation exists in the mortality rates reported by individual centers. Center experience (reflected by case volume) may contribute to this variation in outcome. The aim of the study was to determine whether CDH mortality ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jpedsurg.2009.01.023
更新日期：2009-05-01 00:00:00
abstract：BACKGROUND/PURPOSE:The purpose of the study was to determine variables associated with attending postoperative clinic follow-up (POFU) in pediatric surgical patients, predictors of clinical value, and visit cost estimates. METHODS:POFU patterns of children undergoing eight common pediatric surgical procedures over one...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2019.01.045
更新日期：2019-05-01 00:00:00
abstract：BACKGROUND:The management of malrotation in patients with congenital abdominal wall defects has varied among surgeons. We were interested in investigating the risk of midgut volvulus in patients with gastroschisis and omphalocele to help determine if these patients may benefit from undergoing a Ladd procedure. METHODS...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jpedsurg.2016.10.014
更新日期：2017-01-01 00:00:00
abstract：:A prospective, randomized, double-blind clinical trial was undertaken comparing gentamicin, ampicillin, and clindamycin (GAC) to gentamicin, ampicillin, and placebo (GAP) in children with complicated appendicitis. Of the 64 patients enrolled in this study, 33 were assigned to the GAC group and 31 received GAP. A singl...
journal_title：Journal of pediatric surgery
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/s0022-3468(83)80051-7
更新日期：1983-12-01 00:00:00
abstract：:We here report the clinical findings and management of 9 consecutive cases of chylothorax, 5 of which occurred spontaneously. One cases followed cardiac surgery. Three cases occurred in low-birth-weight, premature infants concurrently with other symptoms of the superior vena cava syndrome secondary to central intraven...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(80)80133-3
更新日期：1980-06-01 00:00:00
abstract：PURPOSE:A prenatal diagnosis of congenital diaphragmatic hernia (CDH) enables therapeutic decision making during the intrapartum period. This study seeks to identify the gestational age and delivery mode associated with optimal outcomes. PATIENTS AND METHODS:A national data set was used to study CDH babies born betwee...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2010.08.026
更新日期：2010-12-01 00:00:00
abstract：:Solitary nonparasitic cyst of the liver (SNPCL) is rare in children. Although there are several hypotheses regarding the pathogenesis, the true origin of SNPCL remains unknown. The authors present an infant with a huge SNPCL in whom the epithelial markers, CA19-9, DU-PAN-2, and SPan-1, were elevated in the serum and c...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2005.08.039
更新日期：2005-12-01 00:00:00
abstract：:Necrotizing fasciitis occurs when the inflammation (cellulitis) spreads beyond the umbilicus to include the subcutaneous tissue and underlying fascia. Presently, omphalitis is relatively uncommon because of aseptic delivery techniques and antimicrobial therapy. One hundred three neonates aged 7 to 28 days, with varyin...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90133-3
更新日期：1994-11-01 00:00:00
abstract：BACKGROUND/PURPOSE:Best results in experimental tracheal allotransplantation are obtained when metachronous revascularization by omentopexy and immunosuppression are used. Nevertheless, this method of revascularization implies in a 4-day period of ischemia to the graft. The aim of this study was to assess the influence...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(99)90156-2
更新日期：1999-08-01 00:00:00
abstract：:Injuries of the popliteal artery have the highest rate of limb loss compared with other peripheral vascular injuries. Particularly, blunt popliteal artery trauma is known to be associated with a high rate of amputation. Traumatic vascular injuries are usually associated with dislocations and fractures. We describe the...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/j.jpedsurg.2012.03.046
更新日期：2012-06-01 00:00:00
abstract：:Cardiopulmonary resuscitation (CPR) occasionally is necessary in the operating-room setting. In such instances, it may be difficult to perform CPR if the patient is in the prone position. Although the supine position is optimal for CPR, it may not be feasible because of the risks of damage to craniospinal structures. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90208-9
更新日期：1994-12-01 00:00:00
abstract：:Epigastric heteropagus refers to unequal and asymmetric conjoined twins in which the dependent component (parasite) is smaller and usually attached to the epigastrium of the dominant component (autosite). Epigastric heteropagus is a very rare type of conjoined twins. It is generally assumed that conjoined twins repres...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章,评审
doi：10.1016/s0022-3468(96)90857-x
更新日期：1996-10-01 00:00:00
abstract：BACKGROUND/PURPOSE:Aggressive neuroblastomas avoid apoptosis and have increased expression of the antiapoptotic protein, Bcl-2. Insulin-like growth factor-I (IGF-I) is mitogenic and may promote tumor survival by inhibiting apoptosis. The authors hypothesize that IGF-I may protect neuroblastoma cells from apoptosis by u...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1053/jpsu.2002.30869
更新日期：2002-03-01 00:00:00
abstract：:Only three primary peritoneal mesotheliomas in children have been previously reported. We describe a 6-wk-old girl with a papillary peritoneal mesothelioma of low grade malignancy. This is probably a congenital mesothelioma and is the youngest patient reported to date. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(83)80093-1
更新日期：1983-06-01 00:00:00
abstract：:Two cases of infantile dumping syndrome which developed following Nissen fundoplication for gastroesophageal reflux are described. Both infants were fed postoperatively via a gastrostomy and showed the typical clinical picture of dumping with failure to thrive, intermittent diarrhea, lethargy and pallor postprandially...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/s0022-3468(84)80436-4
更新日期：1984-04-01 00:00:00
abstract：:Three newborn boys presented with features suggestive of classic low anorectal abnormality. However, during surgery they were found to have an intermediate anorectal abnormality and a rectoperineal fistula rather than an anocutaneous fistula. The surgical treatment of these infants is discussed. ...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90085-x
更新日期：1994-04-01 00:00:00
abstract：:Central venous access devices have become important tools in the management of pediatric patients with malnutrition, malignancy, and infections requiring long-term antibiotic treatment. Hemophilia presents a lifetime challenge for venous access and at times can be an urgent or life-threatening situation. Since 1986, t...
journal_title：Journal of pediatric surgery
pub_type： 杂志文章
doi：10.1016/0022-3468(94)90806-0
更新日期：1994-09-01 00:00:00
