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Anne-Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Voltage-dependent K(+) channels (Kv channels) participate in electrical rhythmicity and smooth muscle responses and are regulated by excitatory and inhibitory neurotransmitters. Kv channels also participate in the interstitial cell of Cajal (ICC) and smooth muscle cell (SMC) responses to neural inputs. The Kv family consists of 12 subfamilies, Kv1-Kv12, with five members of the Kv7 family identified to date: Kv7.1-Kv7.5. A recent study identified the potassium channel Kv7.5 as having a role in the excitability of ICC-IM in the mouse colon...
January 3, 2017: Journal of Pediatric Surgery
Bindey Kumar, Amit Kumar Sinha, Prem Kumar, Anil Kumar
Currarino syndrome (CS) is a rare clinical condition. The classical presentation includes a triad of sacral anomaly, anorectal malformations, and presacral mass. This syndrome belongs to the group of persistent neuroenteric malformations. This article presents two cases of Currarino syndrome, where there was rare clinical variants such as rectal atresia in the first case and rectal stenosis in the second case. The clinical presentations were very deceptive as the first case presented as high anorectal malformation and the second case was simulating Hirschprung's disease...
October 2016: Journal of Indian Association of Pediatric Surgeons
N R Chevalier, E Gazguez, L Bidault, T Guilbert, C Vias, E Vian, Y Watanabe, L Muller, S Germain, N Bondurand, S Dufour, V Fleury
Neural crest cells (NCCs) are a population of multipotent cells that migrate extensively during vertebrate development. Alterations to neural crest ontogenesis cause several diseases, including cancers and congenital defects, such as Hirschprung disease, which results from incomplete colonization of the colon by enteric NCCs (ENCCs). We investigated the influence of the stiffness and structure of the environment on ENCC migration in vitro and during colonization of the gastrointestinal tract in chicken and mouse embryos...
February 18, 2016: Scientific Reports
Parul Jain, S M Rajeshwari, Jagjit Singh, Tarun Kumar, Sandeep P Agarwal, Prasenjit Das
BACKGROUND: Although the most common cause of neonatal intestinal pseudoobstruction (IP) is Hirschprungs disease, rarely myofibrillar myopathy can cause the same. CASE REPORT: This 31+4/7 male infant at autopsy had marked narrowing of the jejunum (0.9 cm long), and colon (7.0 cm long) markedly narrowed segments of the jejunum and large intestine, were noted respectively. Sections from these segments showed eosinophilic periodic acid Schiff stain and desmin positive cytoplasmic inclusion bodies in the myocytes...
2016: Fetal and Pediatric Pathology
Mansi Shah, Jared Gallaher, Nelson Msiska, Sean E McLean, Anthony G Charles
BACKGROUND: Intestinal obstruction (IO) is a common pediatric surgical emergency in sub-Saharan Africa with high morbidity and mortality, but little is known about its etiopathogenesis in Malawi. METHODS: Retrospective analysis of patients seen from February 2012 to June 2014 at Kamuzu Central Hospital in Lilongwe, Malawi (n = 3,407). Pediatric patients with IO were analyzed (n = 130). RESULTS: Overall, 57% of patients were male with a mean age of 3...
April 2016: American Journal of Surgery
Cristina Elena Singer, Carmen Simona Coşoveanu, Mircea Ovidiu Ciobanu, George Alin Stoica, Ileana Puiu, Corina Lavinia Gruia, Liliana Streba, Cristian Constantin, Carmen Daniela Neagoe
Failure of neural crest cells to migrate from neural crests during intrauterine development result in partial or total aganglionosis of the colon in newborn. Hirschprung's disease (HD) represents the clinical manifestation of this pathogenic process, currently accounting for the majority of lower intestinal obstruction in the first period of life. Our aim was to present a series of three cases presenting to our tertiary care center with a range of symptoms, all benefiting from surgery and consequent pathology examination of biopsy or resection pieces...
2015: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Xue Xia, Ning Li, Jia Wei, Wen Zhang, Donghai Yu, Tianqi Zhu, Jiexiong Feng
INTRODUCTION: This study aims to describe laparoscopic reoperation (LSR) and compare its outcomes with transabdominal reoperation (TAR) for treating Hirschsprung's disease (HD). PATIENTS AND METHODS: Eighteen patients with HD underwent reoperation for recurring constipation due to residual aganglionosis and transition zone pathology after an initial transanal procedure (LSR, n=10; TAR, n=8). Preoperative, operative and postoperative data were collected through patient follow-ups ranging from 13 to 75months to compare operative characteristics and postoperative outcomes between the two groups...
April 2016: Journal of Pediatric Surgery
Maryam Khazdouz, Majid Sezavar, Bahareh Imani, Hossein Akhavan, Alireza Babapour, Gholamreza Khademi
BACKGROUND: Bowel function has been reported to be adversely affected following surgery in cases of Hirschsprung. We retrospectively studied both the clinical outcome and bowel function status following surgery in patients diagnosed with Hirschprung's disease (HD). 161 cases, who underwent pull-through operations for HD in Sheikh Pediatric Tertiary Centre, Mashhad, Iran. The specified time bracket spanned between 2006 and 2011. MATERIALS AND METHODS: Data was extracted from Health Information System with the aim of investigating patients for both short and long-term gastrointestinal (GI) complications after surgery bases in addition to the concurrence of any associated anomalies...
April 2015: African Journal of Paediatric Surgery: AJPS
Ambreen Asim, Ashok Kumar, Srinivasan Muthuswamy, Shalu Jain, Sarita Agarwal
Down syndrome (DS) is one of the commonest disorders with huge medical and social cost. DS is associated with number of phenotypes including congenital heart defects, leukemia, Alzeihmer's disease, Hirschsprung disease etc. DS individuals are affected by these phenotypes to a variable extent thus understanding the cause of this variation is a key challenge. In the present review article, we emphasize an overview of DS, DS-associated phenotypes diagnosis and management of the disease. The genes or miRNA involved in Down syndrome associated Alzheimer's disease, congenital heart defects (AVSD), leukemia including AMKL and ALL, hypertension and Hirschprung disease are discussed in this article...
2015: Journal of Biomedical Science
David J Wilkinson, George S Bethell, Rajeev Shukla, Simon E Kenny, David H Edgar
Enteric nervous system progenitor cells isolated from postnatal human gut and cultured as neurospheres can then be transplanted into aganglionic gut to restore normal patterns of contractility. These progenitor cells may be of future use to treat patients with Hirschprung's disease, a congenital condition characterized by hindgut dysmotility due to the lack of enteric nervous system ganglia. Here we demonstrate that progenitor cells can also be isolated from aganglionic gut removed during corrective surgery for Hirschsprung's disease...
2015: PloS One
Ayşe Karaaslan, Ahmet Soysal, Abdurrahman Sarmış, Eda Kepenekli Kadayifci, Kıvılcım Cerit, Serkan Atıcı, Güner Söyletir, Mustafa Bakır
Lactococcus lactis is a gram-positive coccus that is nonpathogenic in humans. Herein, we present the case of a 1-year-old boy with Down syndrome and Hirschprung's disease (HD) who developed a catheter-related bloodstream infection with L. lactis after gastrointestinal surgery. The patient had been hospitalized in the pediatric surgery unit from birth because of HD, and had undergone the Duhamel-Martin procedure which caused recurrent diarrhea episodes and feeding intolerance. On the infant's 430th day of life, he had an episode of gastroenteritis and feeding intolerance...
2015: Japanese Journal of Infectious Diseases
Leyla Cinel, Bahar Ceyran, Berrin Güçlüer
In this study we aimed to evaluate the usability of calretinin staining in the diagnosis and exclusion of HD in 36 rectal biopsies. Through immunohistochemical examination, in of a total of 21 pediatric patients in whom ganglion cells were detected in first rectal biopsies and in re-biopsies, ganglion cells were seen through nuclear and cytoplasmic staining. In the lamina propria and superficial submucosa, staining of nerve fibers was detected in a granular pattern in varying intensities. Out of a total of 5 biopsies (including one re-biopsy) of non-HD patients, where ganglion cells could not be seen, the nerve fibers were all stained...
January 2015: Pathology, Research and Practice
Tingting Gao, Haijiao Chen, Mei Liu, Wenliang Ge, Qiyou Yin
Hirschprung's disease (HD), a very common congenital abnormality in children, occurs mainly due to the congenital developmental defect of the enteric nervous system. The absence of enteric ganglia from the distal gut due to deletion in gut colonization by neural crest progenitor cells may lead to HD. The capacity to identify and isolate the enteric neuronal precursor cells from developing and mature tissues would enable the development of cell replacement therapies for HD. However, a mature method to culture these cells is a challenge...
May 2016: Cytotechnology
O B Bodnar, L I Vatamanesku, B M Bodnar, R I Sydorchuk
OBJECTIVE: To analyze our ten year experience of surgical treatment of children with chronic colonostasis of different origin. MATERIAL AND METHODS: The study covers 353 children aged from 1 day to 17 years with colonic stasis who undergo treatment at the Department of Children's Surgery, Bukovinian State Medical University, Cernăuţi, Ukraine. Study design included case history, physical examination, routine clinical and biochemical laboratory tests, endoscopy (sigmoidoscopy, colonoscopy), X-ray (irrigoscopy, irrigography, other radiopaque techniques) methods, and sphincterometry...
July 2014: Revista Medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti Din Iaş̧i
S B Patíl, S Hugar, S Patíl
BACKGROUND: Green stain is an uncommon clinical condition associated with deposition of bilirubin in the dental hard tissues. The staining of the teeth is due to hyperbilirubinemia caused by systemic conditions. CASE REPORT: The purpose of this report is to present a case of green teeth of the primary dentition in a 17-month old girl associated with hydrocephalus and Hirschprung's disease. CONCLUSION: The clinical characteristics of the teeth may help in the diagnosis of current or past systemic diseases...
July 2014: European Archives of Paediatric Dentistry: Official Journal of the European Academy of Paediatric Dentistry
John Massie, Lynn Gillam
Congenital central hypoventilation syndrome (CCHS) is a well-recognized disorder of the autonomic nervous system caused by mutations in the PHOX2B gene. The most characteristic feature is failure of ventilatory control, resulting in the need for respiratory support while asleep, and in some cases when awake also. Most cases present in infancy or early childhood. Technological advances allow patients with mild to moderate phenotypesto receive adequate support by non-invasive ventilation (NIV), or diaphragm pacing (or combination of the two) avoiding the need for long-term ventilation by tracheostomy...
May 2015: Pediatric Pulmonology
Jonathan Randall, Peter Coyne, Bruce Jaffray
PURPOSE: Antegrade continent enema (ACE) procedures have been used as a treatment of constipation and soiling. Little is known about the long-term results of these procedures, particularly as patients progress into adulthood. This study presents the long-term outcomes of ACE in children, with follow up into adulthood, over a fifteen-year period. METHODS: A prospective database of all consecutive procedures performed from 1998 to 2013 by a single surgeon in a regional centre was analysed...
September 2014: Journal of Pediatric Surgery
V V Kholostova, A F Dronov, A N Smirnov, D V Zalikhin, A G Mannanov, E Iu Ermolenko, L Iu Tikhomirova
It was analyzed the treatment results of 24 patients under the age of 2 years with total aganglionosis for the period from 2000 to 2013. Each of these patients underwent several surgical interventions (on the average 7.8±2.1). All children were operated radically. It was performed ileojejunorectal anastomosis at transitional fold of peritoneum in patients with concomitant short bowel syndrome. Soave's operation was done in 14 patients including by using of laparoscopic technique in 3 cases. Inflammatory complications (paraproctitis) developed in 2 children on maceration background...
2014: Khirurgiia
S B Patil, S Hugar, S Patil
BACKGROUND: Green stain is an uncommon clinical condition associated with deposition of bilirubin in the dental hard tissues. The staining of the teeth is due to hyperbilirubinemia caused by systemic conditions. CASE REPORT: The purpose of this report is to present a case of green teeth of the primary dentition in a 17-month old girl associated with hydrocephalus and Hirschprung's disease. CONCLUSION: The clinical characteristics of the teeth may help in the diagnosis of current or past systemic diseases...
July 2014: European Journal of Paediatric Dentistry: Official Journal of European Academy of Paediatric Dentistry
Girish D Bakhshi, Sachin S Shenoy, Kavita V Jadhav, Mukund B Tayade, Sunil R Patil, Chintan B Patel
The number of ileostomies created for benign diseases such as familial adenomatous polyposis and ulcerative colitis is increasing. Long-term ileostomies are prone to develop various complications over time. Ileostomy site carcinoma is a well-established complication in ulcerative colitis and familial adenomatous polyposis that have undergone total colectomy. However, no case of ileostomy site carcinoma has been described in a patient with Hirschprung's disease. We present the first case of adenocarcinoma at an ileostomy site in a patient with Hirschprung's disease with retroviral disease...
August 2, 2013: Clinics and Practice
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