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 pathogenic significance of SIX2 and CITED1 across WT, the Children's Oncology Group provided 40 WT specimens of stages I to IV (n = 10 per stage), which were enriched for unfavorable histology (n = 20) and treatment failure (relapse or death, n = 20). SIX2 and CITED1 protein expression was evaluated qualitatively (immunohistochemistry) and quantitatively (Western blot, or WB). Gene transcription was estimated using quantitative real-time polymerase chain reaction (qRT-PCR). RESULTS:SIX2 was visualized by immunohistochemistry in 36 (94.7%) of 38 specimens. Protein and messenger RNA expression of SIX2 were quantitatively similar across all stages of disease (P = .48 WB; P = 0.38 qPCR), in favorable or unfavorable histology (P = 0.51 WB; P = 0.58 qPCR), and in treatment failure or success (P = 0.86 WB; P = 0.49 qPCR). Although CITED1 expression paralleled SIX2 qualitatively, no quantitative correlation between SIX2 and CITED1 expression was observed (Spearman correlation coefficient, 0.28; P = 0.08). As in the fetal kidney, overlapping, but also distinct, WT cellular expression domains were observed between SIX2 and CITED1. CONCLUSION:SIX2 and CITED1 remain active across all disease characteristics of WT. Activity of these genes in WT potentially identifies a population of self-renewing cancer cells that exhibit an embryonic, stemlike phenotype. Taken together, these transcriptional regulators may be fundamental to WT cellular self-renewal and may represent targets for novel therapies that promote terminal differentiation.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Murphy AJ,Pierce J,de Caestecker C,Taylor C,Anderson JR,Perantoni AO,de Caestecker MP,Lovvorn HN 3rddoi
10.1016/j.jpedsurg.2012.03.034subject
Has Abstractpub_date
2012-06-01 00:00:00pages
1239-49issue
6eissn
0022-3468issn
1531-5037pii
S0022-3468(12)00274-6journal_volume
47pub_type
杂志文章abstract::Three children with testicular feminization syndrome have been seen in the past 4 yr. Each presented with at least one large inguinal hernia. The gonad was proven to be a testis by frozen section. Gonadectomy and herniorrhaphy were done. The plan is to remove the second gonad after puberty. The causation and genetics ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(81)80008-5
更新日期:1981-08-01 00:00:00
abstract:BACKGROUND/PURPOSE:This study aims to elucidate the clinical presentation, the effectiveness of investigations, and treatment of foreign body ingestion in children and to formulate an algorithm of management. METHODS:The records of children admitted to a single institution who had a history of foreign body ingestion o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(99)90106-9
更新日期:1999-10-01 00:00:00
abstract:PURPOSE:Chronic illness in infancy may influence parent-infant interaction. We assessed quality of mother-infant interaction in children with esophageal atresia (EA) and searched for predictors for impaired interaction. METHODS:The study group comprised 37 one-year-old infants with EA born in 1999 to 2002 and their mo...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2008.04.011
更新日期:2008-10-01 00:00:00
abstract::Bilateral fetal rhabdomyomatous nephroblastoma is a rare variant of Wilms' Tumor. The authors report the evolution over 48 months of a 10-month-old baby with bilateral nephroblastoma for which a left nephrectomy was initially performed. A right kidney tumor was enucleated preserving the kidney. The transformation of t...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1053/jpsu.2003.50151
更新日期:2003-04-01 00:00:00
abstract:INTRODUCTION:Non-central nervous system (non-CNS) rhabdoid tumors tend to present at a young age and have an extremely aggressive course, with dismal overall survival rates. Inactivation of the tumor suppressor gene SMARCB1 has been shown in rhabdoid tumors regardless of anatomic location, suggesting a common genetic b...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2016.08.017
更新日期:2017-03-01 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::Esophageal resection or replacement has become the standard therapy for severe esophageal strictures chiefly because less aggressive methods generally have failed. We hereby report our experience with 12 consecutive infants and children who have been managed successfully by means of Stamm gastrostomy and string-guided...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(89)80529-9
更新日期:1989-08-01 00:00:00
abstract:PURPOSE:The aim of this study was to determine how to manage children with recurrence of intussusception. METHODS:Medical records of patients treated for intussusception from 1976 to 2008 at the Queen Sirikit National Institute of Child Health were reviewed. Information on patients who developed recurrent intussuscept...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.07.029
更新日期:2010-11-01 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:RATIONALE:Recurrent tracheoesophageal fistulas (RTEFs) remain a therapeutic challenge because open surgical approaches have been associated with substantial rates of morbidity, mortality, and repeat recurrences. Recently, endoscopic techniques for the repair of RTEF have provided an alternative approach with the potent...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2007.08.062
更新日期:2008-01-01 00:00:00
abstract:BACKGROUND:Anal fissure in children usually is treated by sitz baths, stool softeners, and analgesic ointments. However, some cases are intractable to the treatment. In recent years, it has been reported that nitric oxide donors such as local glyceryl-trinitrate (GTN) ointment causes a reversible chemical sphincterotom...
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(99)90318-4
更新日期:1999-12-01 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::The authors report on two adults who had jejunal dilatation after having had resection for jejunal atresia in the neonatal period. Both patients presented 20 years after the initial procedure, with severe iron deficiency anemia, marked jejunal dilatation proximal to the old anastomotic site (which was not narrowed), a...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(94)90238-0
更新日期:1994-12-01 00:00:00
abstract::Most appendiceal carcinoids (ACs) in children present without lymph node metastasis. Lymph node metastasis is rarely present when primary tumor diameter exceeds 1 cm. We present the extraordinary case of an AC with a primary tumor diameter of 0.7 cm and infiltration of the mesentery, as well as 1 positive lymph node o...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2010.07.025
更新日期:2010-11-01 00:00:00
abstract::The murine neuroblastoma appears to be a useful model for elucidating the mechanism of cellular differentiation. In tissue culture, MNB cells were induced to "irreversibly" differentiate into neuronal-like cells by DBcAMP alone or in combination with cAMP phosphodiesterase inhibitors: papaverine (Pap) and theophylline...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(76)90113-5
更新日期:1976-10-01 00:00:00
abstract::A 10-month-old boy was noted to have hepatomegaly, and hepatic angiography showed a huge tumor of the medial segment of the left lobe, located close to the hepatic hilum and involving the anterior segment of the right lobe in contiguity. We considered at first that resection would not be possible, but at operation the...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(89)80109-5
更新日期:1989-11-01 00:00:00
abstract:BACKGROUND/PURPOSE:Laparoscopic Appendectomy (LA) is a safe procedure in adults resulting in shorter hospitalization and sooner return to activity. The relative merits of LA and open appendectomy (OA) are evaluated in this first prospective and randomized study in children. METHODS:A total of 129 children with appendi...
journal_title:Journal of pediatric surgery
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1053/jpsu.2002.30841
更新日期:2002-03-01 00:00:00
abstract:OBJECTIVE:Intercellular adhesion molecule (ICAM) expression in liver and bile duct remnant is a feature of the inflammatory component of biliary atresia (BA). Circulating levels of such soluble adhesion molecules (SAM) should reflect intrahepatic disease and would prove a useful adjunct in the evaluation of BA. STUDY ...
journal_title:Journal of pediatric surgery
pub_type: 临床试验,杂志文章
doi:10.1016/j.jpedsurg.2005.05.050
更新日期:2005-09-01 00:00:00
abstract::The value of performing Nissen fundoplication in neurologically impaired children is a controversial issue. To evaluate the benefit of fundoplication in these children, hospital records were reviewed for 77 children who underwent fundoplication for gastroesophageal reflux (GER). Fifty-two children were neurologically ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(91)90013-j
更新日期:1991-06-01 00:00:00
abstract::Four adolescent girls, aged 12 to 14 years, were seen for evaluation of spontaneous nipple discharge, two of whom had associated breast lumps in the ipsilateral breast. Clinical examination showed the discharge to be arising from one or several of Montgomery's areolar tubercles, with the breast lumps localized to the ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(88)80409-3
更新日期:1988-08-01 00:00:00
abstract:OBJECTIVE:To characterize referrals to a vascular anomalies center to determine which lesions should be treated by specialists. STUDY DESIGN:Patients sent to our center between 1999 and 2010 were reviewed. Type of anomaly, anatomical location, sex, accuracy of referral diagnosis, and the geographic origin of the refer...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2011.05.006
更新日期:2011-09-01 00:00:00
abstract::In 31 cases of Hirschsprung's disease, the mean value of erythrocyte AChE activity was discovered to be 91.24 +/- 10.24 u/mL, being significantly higher than that from 127 normal children of 73.51 +/- 9.36 u/mL (P less than 0.001); whereas serum ChE mean value of 14.49 +/- 4.04 u/mL showed no difference from normal co...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(84)80187-6
更新日期:1984-06-01 00:00:00
abstract::Marine envenomations are the statistical price that humankind pays for intruding into the aquatic domain. Individual risk can be reduced by knowledge and prudence. If envenomations do occur, lives can be saved, complications prevented, and convalescence facilitated by knowledge of and interest in these fascinating cre...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/0022-3468(95)90622-3
更新日期:1995-01-01 00:00:00
abstract::A case of physiologic hypothyroidism caused by the topical application of povidone-iodine (PVPI) in a newborn with an omphalocele is presented. The literature on systemic absorption and effects of PVPI is reviewed. A management strategy is offered. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(88)80207-0
更新日期:1988-04-01 00:00:00
abstract::The case of a 12-year-old girl having cholestatic syndrome, due to the presence of gastric tissue in the gallbladder and extrahepatic biliary tract, is reported here. ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(90)90088-q
更新日期:1990-03-01 00:00:00
abstract::The distribution and localization of neurofilament (NF) and microtubule-associated tau protein (Tau) in the colon from Hirschsprung's disease were examined by immunohistochemistry. Specimens of the normoganglionic, oligoganglionic, and aganglionic segments of colons from nine patients with Hirschsprung's disease were ...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(93)90688-h
更新日期:1993-07-01 00:00:00
abstract:BACKGROUND:The management of nonparasitic splenic cysts (NPSC) remains controversial. Surgical resection is indicated for symptomatic or complicated forms, but no guidelines are available for asymptomatic NPSC. The aims of this study were to evaluate the management of NPSC in French hospitals and to analyze the results...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jpedsurg.2017.01.054
更新日期:2017-09-01 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::A 10-year-old boy presented with features of Cushing's syndrome. Investigations, including ultrasound scan and computed tomography scans, demonstrated a right adrenal tumor. The tumor was excised and histologically found to be an encapsulated adrenal adenoma. Surgical excision is the treatment of choice for adrenal tu...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章,评审
doi:10.1016/s0022-3468(98)90335-9
更新日期:1998-04-01 00:00:00
abstract:PURPOSE:The aim of this study is to determine what factors predict spontaneous passage of ≤1 cm ureteral stones in children. METHODS:Files of the patients diagnosed with a single ureteral stone on a given side between 2008 and 2017 were retrospectively reviewed. Patients with congenital obstructive uropathy, neurogeni...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2019.05.012
更新日期:2020-07-01 00:00:00