Altered expression of KCNG3 and KCNG4 in Hirschsprung's disease.

Abstract:

PURPOSE:Voltage-gated potassium ion channels have long been implicated in gastrointestinal motility. Recent studies have highlighted the role of voltage-gated channel subfamily G member 3 (KCNG3) and 4 (KCNG4) genes in the electrical functioning of interstitial cells of Cajal and PDGFRα+ cells of the mouse colon. We designed this study to investigate KCNG3 and KCNG4 expression in the normal human colon and in Hirschsprung's disease (HSCR). METHODS:HSCR tissue specimens (n = 6) were collected at the time of pull-through surgery, while control samples were obtained at the time of colostomy closure in patients with imperforate anus (n = 6). qRT-PCR analysis was undertaken to quantify KCNG3 and KCNG4 gene expression, and immunolabelling of KCNG3 and KCNG4 proteins was visualized using confocal microscopy. RESULTS:qRT-PCR analysis revealed significant downregulation of the KCNG3 and KCNG4 genes in both aganglionic and ganglionic HSCR specimens compared to controls (p < 0.05). Confocal microscopy revealed KCNG3 and KCNG4 expression within neurons, ICC and PDGFRα+ cells of the myenteric plexus and smooth muscle layers, with a reduction in both proteins in aganglionic and ganglionic HSCR colon compared to controls. CONCLUSION:KCNG3 and KCNG4 gene expression is significantly downregulated in HSCR colon, suggesting a role for these genes in colonic motility. KCNG3 and KCNG4 downregulation within ganglionic specimens highlights the physiologically abnormal nature of this segment in HSCR patients.

journal_name

Pediatr Surg Int

authors

O'Donnell AM,Nakamura H,Tomuschat C,Marayati NF,Puri P

doi

10.1007/s00383-018-4394-2

subject

Has Abstract

pub_date

2019-02-01 00:00:00

pages

193-197

issue

2

eissn

0179-0358

issn

1437-9813

pii

10.1007/s00383-018-4394-2

journal_volume

35

pub_type

杂志文章
  • An in vitro study of silicone migration from intravenous fluid tubing.

    abstract::Migration of particulate matter from plastic tubing and solid plastic implants has been documented in a number of studies, including some with the use of cardiac bypass, haemodialysis, and pump-assisted intravenous infusions. In order to ascertain whether silicone embolisation occurs when children have an Ivac 560 pum...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/BF01194802

    authors: Dewan PA,Owen AJ,Ashwood PJ,Terlet J,Byard RW

    更新日期:1997-01-01 00:00:00

  • The vacuum bell for conservative treatment of pectus excavatum: the Basle experience.

    abstract:OBJECTIVE:Surgical repair of pectus excavatum (PE) in childhood is a well-established procedure. Previously used operative techniques to correct PE were largely based on the Ravitch technique. Since about 10 years, the minimally invasive repair (MIRPE) by Nuss is well established. Conservative treatment with the vacuum...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-010-2843-7

    authors: Haecker FM

    更新日期:2011-06-01 00:00:00

  • The outcomes of a frenulotomy on breastfeeding infants followed up for 3 months at Thammasat University Hospital.

    abstract:PURPOSE:To study the effects of frenulotomy on nipple pain, latch and the success in exclusive breastfeeding (EBF) at 3-month follow-up. METHODS:A prospective cross-sectional study of 328 mother-infant pairs with both tongue-tie and breastfeeding problems was performed. Nipple pain and latch were evaluated using numer...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3952-8

    authors: Wakhanrittee J,Khorana J,Kiatipunsodsai S

    更新日期:2016-10-01 00:00:00

  • Protocol-based management of 154 cases of pediatric liver abscess.

    abstract:AIM:Although liver abscess (LA) is prevalent worldwide, especially in developing countries, there is a paucity of data regarding the protocol for management of LA in children. The aim of this study was to analyze the outcome of a protocol-based management of pediatric LA from a single institution. MATERIALS AND METHOD...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-4009-8

    authors: Roy Choudhury S,Khan NA,Saxena R,Yadav PS,Patel JN,Chadha R

    更新日期:2017-02-01 00:00:00

  • Giant-cell fibroblastoma in a patient with a bone-marrow transplant.

    abstract::A case of an epigastric giant-cell fibroblastoma is reported in a 6-year-old girl who had undergone a bone-marrow transplant for severe combined immunodeficiency secondary to adenosine deaminase deficiency. A small subcutaneous nodule had been excised from the epigastrium at age 12 months. ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-002-0827-y

    authors: Carroll D,Ramani P,Lander AD

    更新日期:2003-08-01 00:00:00

  • Surgical closure of patent ductus arteriosus in very-low-birth-weight infants.

    abstract::In many very-low-birth-weight (VLBW) infants the ductus arteriosus fails to close spontaneously, and they subsequently develop signs and symptoms of poor tissue perfusion and heart failure. This study evalutes the results of early surgical closure of patent ductus arteriosus (PDA). We retrospectively reviewed the reco...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830000515

    authors: Niinikoski H,Alanen M,Parvinen T,Aantaa R,Ekblad H,Kero P

    更新日期:2001-07-01 00:00:00

  • Congenital hepatic cyst with antenatal diagnosis: a case report and literature review.

    abstract::Congenital hepatic cysts are rare lesions of infancy. While operative management and outcomes have been extensively studied in adult patients with hepatic cysts, data in pediatric patients are limited. We discuss our experience in an infant and review relevant literature regarding operative technique and surgical outc...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-013-3312-x

    authors: Nordin AB,Fallon SC,Carter BA,Brandt ML

    更新日期:2013-08-01 00:00:00

  • Robotic fundoplication in children.

    abstract::Since January 2006, robotic assistance has been used for performing minimal invasive laparoscopic fundoplications in children. These patients were compared with those operated on with either the open surgical technique or the laparoscopic procedure. The first six children operated on with a fundoplication using the op...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-006-1817-2

    authors: Anderberg M,Kockum CC,Arnbjörnsson E

    更新日期:2007-02-01 00:00:00

  • Bianchi high scrotal approach revisited.

    abstract::Cryptorchidism is a common paediatric complaint and is traditionally managed surgically by orchidopexy. We aimed to prospectively review the success of the Bianchi high scrotal incision technique for performing orchidopexy in boys with palpable non-descended testis. A total of 206 orchidopexies were performed in 85 pa...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-008-2149-1

    authors: Samuel DG,Izzidien AY

    更新日期:2008-06-01 00:00:00

  • Reduction of incarcerated inguinal hernia in infants using caudal epidural anaesthesia.

    abstract::Incarcerated inguinal hernias in infants are commonly encountered. Reduction with intravenous sedation using morphine and related drugs carries risks of apnoea and/or respiratory arrest, especially in small, ex-premature babies who have an increased incidence of incarceration and thus opiate use is best avoided. Cauda...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-005-1534-2

    authors: Brindley N,Taylor R,Brown S

    更新日期:2005-09-01 00:00:00

  • Prophylactic efficacy of enteral miconazole administration for neonatal intestinal perforation and its potential mechanism.

    abstract:PURPOSE:Intestinal perforation (IP) is a fatal complication in extremely low birth weight infants (ELBWI). We started administrating enteral miconazole (MCZ) to ELBWI in 2002. Since then, the incidence of IP has significantly decreased. The aim of this study was to elucidate the prophylactic effect of MCZ for the treat...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3946-6

    authors: Torikai M,Ibara S,Ieiri S,Hamada T,Noguchi H,Sueyoshi K,Fukuda T,Abeyama K

    更新日期:2016-10-01 00:00:00

  • The vulnerable stomach in babies born with pure oesophageal atresia.

    abstract::Nine babies with pure oesophageal atresia were treated in our institution in the years 1979-1996. All received a feeding gastrostomy as their initial operation. After initiation of gastrostomy feeds seven (78%) developed gastric complications, including two posterior gastric perforations (one fatal). We propose that t...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050640

    authors: Kimble RM,Harding JE,Kolbe A

    更新日期:1999-01-01 00:00:00

  • T-cryptantigen (TCA) activation in surgical neonates: a hidden problem.

    abstract::The Thomsen-Friedenreich cryptantigen (TCA) is located on the surface of all red cells, but is concealed by a layer of neuraminic acid. When bacteria that produce neuraminidase disrupt this coating, the TCA can be exposed and activated. If blood products containing antibody to the TCA are subsequently administered, ha...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050488

    authors: Grant HW,Hadley GP,Adhikari M,Fernandes-Costa F

    更新日期:1998-12-01 00:00:00

  • Polypoid disease of colon in children.

    abstract::Polyps in gastrointestinal tract are mostly benign and result from hamartomas or lymphoid hyperplasia of submucosa. They usually occur as isolated lesions in children and are referred to as juvenile polyps. Multiple polyps with inherited origin are called polyposis and can be seen as a part of the syndrome. The polyps...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-020-04621-3

    authors: Soyer T

    更新日期:2020-04-01 00:00:00

  • Unilateral spermatic vessel ligation in prepubertal rats decreases microvascular blood flow to ipsilateral testis postpubertally.

    abstract::High ligation of spermatic vessels (HLSV) and low ligation of spermatic vessels (LLSV) have been described in the surgical treatment of high undescended testis. Testicular capillary blood flow utilizing laser Doppler flowmetry after these maneuvers has not been studied thus far; hence, this study was designed. Thirty ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-003-1018-1

    authors: Srinivas M,Kilmartin B,Basham P,Puri P

    更新日期:2003-07-01 00:00:00

  • Congenital epulis of the jaw: a series of five cases and review of literature.

    abstract::This article describes five cases of congenital epulis, a rare and benign swelling in the mouth of a newborn, which is not widely known. We present five cases: four cases presented as single pedunculated nodules of the gingiva and in one case two nodules were present. Of all, 50% were located at the maxilla. Excision ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-008-2304-8

    authors: Küpers AM,Andriessen P,van Kempen MJ,van der Tol IG,Baart JA,Dumans AG,van der Waal I

    更新日期:2009-02-01 00:00:00

  • Neuroblastoma: treatment outcome after incomplete resection of primary tumors.

    abstract:PURPOSE:For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-009-2417-8

    authors: Moon SB,Park KW,Jung SE,Youn WJ

    更新日期:2009-09-01 00:00:00

  • Increased pulmonary vascular expression of Krüppel-like factor 5 and activated survivin in experimental congenital diaphragmatic hernia.

    abstract:AIM OF THE STUDY:The high morbidity and mortality in congenital diaphragmatic hernia (CDH) is attributed to pulmonary hypoplasia and persistent pulmonary hypertension (PH). PH is characterized by increased pulmonary artery smooth muscle cell (SMC) proliferation, suppressed apoptosis as well as endothelial dysfunction. ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-014-3606-7

    authors: Hofmann AD,Takahashi T,Duess JW,Gosemann JH,Puri P

    更新日期:2014-12-01 00:00:00

  • A review of congenital lung malformations with a simplified classification system for clinical and research use.

    abstract:PURPOSE:Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practi...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章,评审

    doi:10.1007/s00383-017-4062-y

    authors: Seear M,Townsend J,Hoepker A,Jamieson D,McFadden D,Daigneault P,Glomb W

    更新日期:2017-06-01 00:00:00

  • Evaluating the socioeconomic and cultural factors associated with pediatric burn injuries in Maputo, Mozambique.

    abstract:BACKGROUND:Pediatric burn injuries are one of the leading causes of preventable morbidity and mortality in Sub-Saharan Africa. Research on the complex system of social, economic and cultural factors contributing to burn injuries in this setting is much needed. METHODS:We conducted a prospective questionnaire-based ana...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-015-3761-5

    authors: Karan A,Amado V,Vitorino P,Kulber D,Taela A,DeUgarte DA

    更新日期:2015-11-01 00:00:00

  • Elective laparoscopic gastrostomy in children: potential for an enhanced recovery protocol.

    abstract:PURPOSE:Gastrostomy tube placement is one of the most commonly performed pediatric surgical procedures and discharge is possible as early as the first postoperative day with early initiation of feeds postoperatively. We examined a national database to determine hospital length of stay (LOS) after elective laparoscopic ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-019-04472-7

    authors: Williams K,Baumann L,Abdullah F,Hendrickson RJ,Oyetunji TA

    更新日期:2019-06-01 00:00:00

  • A national survey on the use of screening tools to detect physical child abuse.

    abstract:PURPOSE:Recognition of physical child abuse is imperative for ensuring children's safety. Screening tools (ST) may increase identification of physical abuse; however, the extent of their use is unknown. This study assessed use of STs for physical abuse in children's hospitals and determined attitudes regarding STs. ME...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3916-z

    authors: Crichton KG,Cooper JN,Minneci PC,Groner JI,Thackeray JD,Deans KJ

    更新日期:2016-08-01 00:00:00

  • Is barium enema reduction safe and effective in patients with a long duration of intussusception?

    abstract::The aim of this study was to determine whether barium enema reduction (BER) is safe and effective in patients with a long duration of intussusception. Over the last 17 years, 104 patients were admitted to our hospital with a diagnosis of intussusception. All except 1 with peritonitis underwent BER primarily. Of the 10...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050526

    authors: Okuyama H,Nakai H,Okada A

    更新日期:1999-01-01 00:00:00

  • Evidence-based operations in paediatric surgery.

    abstract::It has been assumed that only 10% of medical interventions are supported by solid scientific evidence. The aim of this study was to determine the type of research evidence supporting operations in a tertiary referral paediatric surgical unit. All patients admitted over a 4-week period to two surgical firms were enroll...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050332

    authors: Baraldini V,Spitz L,Pierro A

    更新日期:1998-07-01 00:00:00

  • Sibling vesicoureteral reflux in twins.

    abstract:AIM OF STUDY:Few studies have evaluated the incidence of familial vesicoureteral reflux (VUR) among multiple gestation births. The purpose of this study was to determine the incidence and characteristics of VUR in twins in a large cohort of families with VUR. METHODS:Between 1998 and 2010, data were collected on 251 f...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-010-2821-0

    authors: Giannotti G,Menezes M,Hunziker M,Puri P

    更新日期:2011-05-01 00:00:00

  • Pulmonary artery size has prognostic value in low birth weight infants with congenital diaphragmatic hernia.

    abstract:AIM:The aim of this study was to examine the relationship between birth weight, pulmonary artery (PA) size, and outcome in congenital diaphragmatic hernia (CDH) to establish if PA size has prognostic value. METHODS:The subjects for this study were 39 consecutive left-sided CDH patients treated at our institution accor...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-011-2899-z

    authors: Takahashi T,Koga H,Tanaka T,Shoji H,Takeda S,Shimizu T,Lane GJ,Yamataka A,Okazaki T

    更新日期:2011-08-01 00:00:00

  • Self-assessed physical health among children with congenital diaphragmatic hernia.

    abstract:PURPOSE:The aim of this long-term follow-up study was to investigate the current self assessed physical health in a CDH birth cohort at a single center. METHODS:Between 1990 and 2009, 195 children born with CDH were treated at Astrid Lindgren Children's Hospital. The primary survival rate was 85 %, and in 2010, 78 % w...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-016-3879-0

    authors: Öst E,Joelsson MÖ,Burgos CM,Frenckner B

    更新日期:2016-05-01 00:00:00

  • Comparison between ultrasonography and X-ray as evaluation methods of central venous catheter positioning and their complications in pediatrics.

    abstract:PURPOSE:This study evaluates the capacity of ultrasonography as a diagnostic method to confirm the proper positioning of central venous catheter (CVC) when compared to the current gold standard, chest radiography (CR). METHODS:A prospective study was performed including children from 0 to 14 incomplete years, who unde...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s00383-020-04642-y

    authors: Oliveira L,Pilz L,Tognolo CM,Bischoff C,Becker KA,Oliveira GG,Neves PJF,Fachin CG,Agulham MA,Dias AIBS

    更新日期:2020-05-01 00:00:00

  • Preoperative imaging of a choledochal cyst in children: non-breath-holding magnetic resonance cholangiopancreatography.

    abstract::Magnetic resonance cholangiopancreaticography (MRCP) was used to visualize the biliary tract in two children, aged 7 weeks and 10 years, with a choledochal cyst. MRCP was successful in both cases and the findings were confirmed by intraoperative cholangiography. ...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/s003830050667

    authors: van Heurn-Nijsten EW,Snoep G,Kootstra G,Greve JW,Forget P,van Heurn LW

    更新日期:1999-01-01 00:00:00

  • A new method for removal of metallic-ferromagnetic foreign bodies from the tracheobronchial tree.

    abstract::Foreign-body (FB) aspirations account for a reasonable percentage of respiratory emergencies in young children. Whereas 67% of aspirated FBs are of organic origin, metallic-ferromagnetic FBs are observed less frequently. We report the use of a wire with a magnetic tip fitting into the working channel of a rigid bronch...

    journal_title:Pediatric surgery international

    pub_type: 杂志文章

    doi:10.1007/BF01076970

    authors: Mayr J,Dittrich S,Triebl K

    更新日期:1997-07-01 00:00:00