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Ruthy Shaco-Levy, Kory W Jasperson, Katie Martin, N Jewel Samadder, Randall W Burt, Jian Ying, Mary P Bronner
The morphologic features of the gastrointestinal polyps in hamartomatous polyposis syndromes are poorly defined. Our aim was to better characterize the gastrointestinal hamartomas in these syndromes. A blinded review was performed regarding many histologic features for every polyp. The study included 15 Cowden syndrome, 13 Peutz-Jeghers (PJS), 12 juvenile polyposis (JuvPS) patients, and 32 cases of sporadic hamartomatous polyps. A total of 375 polyps were examined. Cowden syndrome polyps were characteristically colonic, sessile, small, without surface erosion, and showing mildly inflamed fibrotic lamina propria with smooth muscle proliferation and lymphoid follicles...
March 2016: Human Pathology
Kathrin Ludwig, Andrea Volpe, Vincenza Guzzardo, Luisa Santoro, Paola Midrio, Piergiorgio Gamba, Rita Alaggio
OBJECTIVES: Hirschsprung's disease (HSCR) results from a malformation of the enteric nervous system. A congenital absence of intrinsic ganglion cells from the distal rectum and a variable length of the contiguous bowel is the required diagnostic feature of Hirschsprung's disease and total colonic aganglionosis (TCA). We evaluated the utility of a monoclonal antibody directed against glypican 3 (GPC-3), a membrane bound protein involved in regulation of the signaling of Wingless-types (WNTs), Hedgehogs (Hh), Fibroblast Growth Factors (FGFs), and Bone Morphogenetic Proteins (BMPs), in the detection of ganglion cells in formalin-fixed, paraffin-embedded tissue sections...
May 2016: Journal of Pediatric Gastroenterology and Nutrition
David Coyle, Anne Marie O'Donnell, Nicolae Corcionivoschi, John Gillick, Prem Puri
BACKGROUND: Rho-kinase (ROCK) is the primary effector protein in the RhoA pathway, which regulates Ca(2+)-independent smooth muscle contraction in the human bowel. This pathway has been reported to be hyper-activated in the aganglionic bowel of EDNRB-null (-/-) rats compared to the ganglionic bowel from EDNRB (+/+) rats. We hypothesised that ROCK expression is up-regulated in human aganglionic bowel and designed this study to investigate ROCK 1 and ROCK 2 expression in Hirschsprung's disease (HSCR) and controls...
November 2015: Pediatric Surgery International
Iban Aldecoa, Judith Navarro-Otano, Nadia Stefanova, Fabienne S Sprenger, Klaus Seppi, Werner Poewe, Miriam Cuatrecasas, Francesc Valldeoriola, Ellen Gelpi, Eduard Tolosa
We aimed to compare immunoreactivity patterns of four different anti-α-syn antibodies in surgical specimens of the gastrointestinal tract of Parkinson disease and control cases. Surgical specimens from stomach, small and large bowel of 6 PD cases and 12 controls were studied. Primary antibodies: anti-α-syn clone KM51, anti-phosphorylated α-syn Ser129, anti-α-syn clone 15G7 and anti-nitrated α-syn505. We found different immunoreactivity patterns: (a) coarse, Lewy-body-like aggregates labelled by the 4 antibodies and detected in 4/6 PD cases and in 1/12 controls; (b) distinct punctate cytoplasmic staining of ganglion cells labelled by anti-phosphorylated-α-syn and detected in 3/6 PD cases and 3/12 controls; (c) fine diffuse, synaptic-type staining of neural structures labelled by anti-α-syn-15G7 and anti-nitrated-α-syn505 and detected in all subjects...
August 18, 2015: Neuroscience Letters
Sarin Yk, Raj P, Thakkar N
BACKGROUND: The purpose of this study is to review the cases of total colonic aganglionosis seen in the span of ten years at a pediatric surgery unit of a tertiary care public hospital in New Delhi. METHODS: Medical records of twelve patients with total colonic aganglionosis were retrieved. RESULTS: Ten out of the twelve patients were males; seven were of the Muslim community. Average recorded birth weight was 2.2 kg. Ten patients presented with features of intestinal obstruction, while two presented with perforation peritonitis...
July 2014: Journal of Neonatal Surgery
Jadwiga Małdyk, Jolanta Rybczyńska, Dariusz Piotrowski, Rafał Kozielski
Hirschsprung disease (HD) is a congenital malformation defined as the absence of myenteric and submucosal ganglion cells (GCs) in the distal rectum and variable length of the contiguous bowel. The aim of this study was to assess the utility of calretinin immunochemistry in comparison with that of standard histology complemented with acetylcholinesterase (AChE) histochemistry routinely employed at our institution to evaluate rectal biopsies carried out for suspicion of HD. Twenty-one rectal biopsies were reviewed, including 14 from patients with suspected HD, 6 from infants with necrotizing enterocolitis (NEC), and 1 from a patient diagnosed with spontaneous intestinal perforation (SIP)...
March 2014: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
Fatemeh Elham Mahjoub, Nasim Zahedi, Bahar Ashjai, Mohammad Taghi Haghi Ashtiani, Fatemeh Farahmand, Maryam Monajemzadeh, Leila Kashi, Heshmat Iranikhah
BACKGROUND/AIMS: Calprotectin is a 36.5 kD calcium and zinc binding protein in the S100 protein family. Fecal calprotectin levels are elevated in patients with inflammatory bowel disease and some other gastrointestinal disorders such as colorectal carcinoma. We decided to evaluate the fecal calprotectin level to see if it was able to distinguish between functional and organic causes of constipation. METHODS: Seventy-six children aged 1 to 120 months that all underwent deep rectal mucosa biopsies at Children Medical Center from November 2010 till September 2011 were recruited...
November 2013: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
Woo Ick Yang, Jung-Tak Oh
BACKGROUND/PURPOSE: Identifying ganglion cells by rectal suction biopsy is a basic diagnostic tool for the diagnosis of Hirschsprung's disease (HD). However, the difficult interpretation of conventionally processed slides often necessitates ancillary staining methods. The aim of this study was to evaluate the usefulness of calretinin and microtubule-associated protein-2 (MAP-2) immunohistochemistry in the diagnosis of HD. METHODS: We analyzed 52 rectal suction biopsy specimens (37 from 15 HD patients and 15 from 7 non-HD patients) for ganglion cells with calretinin and MAP-2 immunohistochemistry...
October 2013: Journal of Pediatric Surgery
Andrea Volpe, Rita Alaggio, Paola Midrio, Loredana Iaria, Piergiorgio Gamba
OBJECTIVES: The aim of this study was to investigate calretinin and β-tubulin immunohistochemical expression together with submucosal nerve trunks morphology in differently innervated segments of Hirschsprung disease (HD) and total colonic aganglionosis (TCA). METHODS: A total of 25 cases (22 HD, 3 TCA) and 18 controls were processed for calretinin and β-tubulin immunohistochemistry. Sections representative of distal aganglionic, transition, and proximal ganglionic segments were evaluated by a visual grading score; β-tubulin was evaluated also by image analysis...
December 2013: Journal of Pediatric Gastroenterology and Nutrition
Katherine J Baxter, Amina M Bhatia
Hirschsprung's disease (HD), congenital absence of ganglion cells, is considered uncommon in preterm infants. The aim was to describe the frequency, presentation, and surgical outcomes of preterm infants with HD. A retrospective cohort study was conducted of all patients diagnosed with HD from 2002 to 2012 at a single children's hospital. Clinical presentation and surgical outcomes were obtained for term (37 weeks of gestation or greater) and preterm infants. One hundred twenty-nine subjects with HD were identified, 24 (19%) preterm and 105 (81%) term...
July 2013: American Surgeon
Weibing Tang, Junwei Tang, Jingjing Qin, Qiming Geng, Zhigang Zhou, Bo Li, Jie Zhang, Huan Chen, Yankai Xia, Xinru Wang
BACKGROUND/PURPOSE: Hirschsprung's disease (HSCR) is a common cause of neonatal bowel obstruction characterized by the absence of ganglion cells in the colon. Impaired migration of the neural crest cells (NCCs) has been implicated as one of the main causes of HSCR. E2F3, a member in the E2F family, which plays a crucial role in the control of the cell cycle is correlated with neuron migration. However, the function of E2F3 in the development of the enteric nervous system still remains unknown...
April 2013: Journal of Pediatric Surgery
S W Moore, D Sidler, P A W Schubert
INTRODUCTION: There is accumulating evidence that "skip" lesions or zonal aganglionosis do occur in HSCR disease, albeit rarely. They are of interest because it may cause confusion in interpreting surgical margins as well as understanding the pathophysiology of HSCR disease. Normally described as "a skip area" of normally ganglionated bowel, surrounded proximally and distally by aganglionosis with variations may occur. CASE REPORTS: We report two cases of infants with unusual types of "skip lesions", identified within the last 5 years...
May 2013: Pediatric Surgery International
Marty Zdichavsky, Hannes Gögele, Gregor Blank, Miriam Kraulich, Tobias Meile, Maximilian von Feilitzsch, Dörte Wichmann, Alfred Königsrainer
BACKGROUND: Acute appendicitis frequently needs acute surgical intervention. Laparoscopic appendectomy (LA) and conventional open appendectomy (OA) are well established procedures, but appendectomy for intraoperative inconspicuous or vascular injected appendixes remains under debate because of potential postoperative morbidity. The aim of this study was to correlate intraoperative nonacute appendixes with histological and clinical outcome. METHODS: Between 2005 and 2009, a total of 1,017 patients underwent OA or LA...
March 2013: Surgical Endoscopy
Tariq Burki, Liina Kiho, Irene Scheimberg, Simon Phelps, Devesh Misra, Harry Ward, Isabel Colmenero
PURPOSE: We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells. METHODS: Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded. RESULTS: We identified 73 patients born at term...
May 2011: Pediatric Surgery International
Masamichi Shinoda, Jun-Ho La, Klaus Bielefeldt, G F Gebhart
Irritable bowel syndrome (IBS) is a functional gastrointestinal disorder characterized by pain and hypersensitivity in the relative absence of colon inflammation or structural changes. To assess the role of P2X receptors expressed in colorectal dorsal root ganglion (c-DRG) neurons and colon hypersensitivity, we studied excitability and purinergic signaling of retrogradely labeled mouse thoracolumbar (TL) and lumbosacral (LS) c-DRG neurons after intracolonic treatment with saline or zymosan (which reproduces 2 major features of IBS-persistent colorectal hypersensitivity without inflammation) using patch-clamp, immunohistochemical, and RT-PCR techniques...
December 2010: Journal of Neurophysiology
Salvatore Arena, Giuseppina Cutroneo, Angelo Favaloro, Maria Teresa Sinatra, Fabio Trimarchi, Silvia Scarvaglieri, Agostino Mallamace, Francesco Arena, Giuseppe Anastasi, Vincenzo Di Benedetto
Hirschsprung's disease (HD) is a development disorder of the enteric nervous system in which the altered innervation explains the inability of the aganglionic segment to relax. Impairment of cytoskeleton in SMC of aganglionic bowel has been shown. Sarcoglycan subcomplex (SG) may support the development and maintenance of muscle cells. We examined the SG subunit expression in colonic aganglionic and ganglionic specimens obtained from patients with HD. Full-thickness bowel specimens were obtained from six patients with HD...
March 2010: International Journal of Molecular Medicine
Béla Veress, Björn Nyberg, Hans Törnblom, Greger Lindberg
AIMS: Visceral inflammatory neuropathies are enteric disorders underlying various forms of bowel dysmotility. The aim was to analyse the microscopic characteristics of a unique combination of intraepithelial lymphocytosis and myenteric ganglioneuritis. METHODS AND RESULTS: Paraffin sections of full-thickness proximal jejunal biopsy specimens from 28 patients, with proven disorders of gastrointestinal motility, were analysed following conventional and immunohistochemical staining...
April 2009: Histopathology
G Bassotti, V Villanacci, C A Maurer, S Fisogni, F Di Fabio, M Cadei, A Morelli, T Panagiotis, G Cathomas, B Salerni
BACKGROUND: Idiopathic slow transit constipation is one of the most severe and often intractable forms of constipation. As motor abnormalities are thought to play an important pathogenetic role, studies have been performed on the colonic neuroenteric system, which rules the motor aspects of the viscus. AIMS: We hypothesised that important neuropathological abnormalities of the large bowel are present, that these are not confined to the interstitial cells of Cajal and ganglion cells, and that the previously described reduction of enteric neurones, if confirmed, might be related to an increase in programmed cell death (apoptosis)...
January 2006: Gut
Vonny Tunru-Dinh, Mark Li-cheng Wu
It generally is believed that perikarya of ganglion cells in the human colorectum are confined to plexuses that lie deep to the mucosa, and that intramucosal perikarya are rare. We retrospectively reviewed 100 specimens from biopsies of normal and abnormal mucosa to further characterize intramucosal perikarya. The presence of intramucosal perikarya, their number, location, and grouping were recorded. Twenty-one specimens (21.0%) contained intramucosal perikarya. Intramucosal perikarya occurred throughout the colorectum in the muscularis mucosae or lamina propria and in normal mucosa, acute self-limited colitis, inflammatory bowel disease, cytomegalovirus-associated colitis, hyperplastic polyps, tubular adenomas, and high-grade intraepithelial neoplasia...
August 2005: American Journal of Clinical Pathology
Caroline P Daly, Richard H Cohan, Isaac R Francis, Elaine M Caoili, James H Ellis, Bin Nan
OBJECTIVE: The purpose of our study was to determine the incidence of acute appendicitis in patients for whom the CT interpretation is deemed equivocal. MATERIALS AND METHODS: Of 1,344 patients referred for CT with suspected appendicitis between January 1998 and December 2002, 172 patients were identified in whom the radiographic findings were equivocal. Two radiologists reviewed the equivocal CT cases, reassessing appendiceal size and the presence of right lower quadrant stranding, fluid, or an appendicolith...
June 2005: AJR. American Journal of Roentgenology
2016-03-12 14:18:36
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