scholarly journals Morphometric Evaluation of PGP9.5 and NCAM Expressing Nerve Fibers in Colonic Muscle of Patients with Hirschsprung's Disease

2002 ◽  
Vol 43 (1) ◽  
pp. 31 ◽  
Author(s):  
Jung Tak Oh ◽  
Ai Ri Han ◽  
Woo Ick Yang ◽  
Seok Joo Han ◽  
Seung Hoon Choi ◽  
...  
2001 ◽  
Vol 16 (1) ◽  
pp. 13-20 ◽  
Author(s):  
Yuji Nirasawa ◽  
Yasuo Ito ◽  
Tomonori Fujiwara ◽  
Nobuo Seki ◽  
Hiroyuki Tanaka ◽  
...  

2017 ◽  
Vol 9 (02) ◽  
pp. 076-080 ◽  
Author(s):  
Bedabrata Mukhopadhyay ◽  
Moumita Sengupta ◽  
Chhanda Das ◽  
Madhumita Mukhopadhyay ◽  
Shibsankar Barman ◽  
...  

Abstract BACKGROUND: Hirschsprung’s disease (HD) is the major cause of pediatric intestinal obstruction with a complex pattern of inheritance. The absence of ganglion cells along with an analysis of hypertrophy and hyperplasia of nerves in the nerve plexus of submucosa and muscularis mucosae is regarded as a potential hallmark for its diagnosis. AIMS AND OBJECTIVES: This study was undertaken to ascertain the (1) clinical profile, (2) mode of presentation, and (3) to compare the role of calretinin immunostaining with acetylcholinesterase in the diagnosis of HD. MATERIALS AND METHODS: This prospective and observational study was conducted in the Department of Pathology, IPGME & R from June 2014 to May 2015. One hundred and four patients clinically and radiologically diagnosed with HD underwent surgery were included in the study. The data of every patient including age, sex, and presenting symptoms were recorded. Eventually, histopathological, calretinin, and acetylcholinesterase immunohistochemical examination were done. RESULTS: Total numbers of cases studied were 104, which aged between 0 days and 365 days. Male preponderance (76.92%) was noted. The overall sensitivity, specificity, positive, and negative predictive value of acetylcholinesterase were 100%, 86.44%, 84.91%, and 100%, respectively. The concordance of detection of ganglion cells and nerve fibers, and thereby diagnosis of Hirschsprung’s and non-HD using calretinin and the gold standard was statistically in strong agreement (κ = 0.749, 95% confidence interval: 0.635–0.863). CONCLUSIONS: Calretinin stands out as the single and indispensable tool that differentiates HD from other mimickers.


2009 ◽  
Vol 15 (12) ◽  
pp. 1493 ◽  
Author(s):  
Amit Kumar Yadav ◽  
Kiran Mishra ◽  
Anup Mohta ◽  
Sarla Agarwal

2011 ◽  
Vol 30 (6) ◽  
pp. 405-413 ◽  
Author(s):  
Iva Gasparovic ◽  
Drazen Kovac ◽  
Mladen Persic ◽  
Irena Slavic ◽  
Harry Nikolic ◽  
...  

2019 ◽  
Vol 23 (2) ◽  
pp. 127-131
Author(s):  
Fernanda Cordeiro-Rudnisky ◽  
Sangtae Ahn ◽  
Natallia Sheuka ◽  
Christine Whyte ◽  
Ann Boguniewicz ◽  
...  

The aganglionic segment of bowel in Hirschsprung’s disease (HD) varies in length. It is not clear whether total colonic aganglionosis (TCA) merely represents a long form of HD or a different phenotype of the disease. Animal model studies suggest that TCA may have a longer transition zone (TZ) than conventional colorectal HD. We compared mucosal innervation of TZ in 2 TCA cases and 10 conventional colorectal HD cases by quantifying calretinin-positive mucosal nerve fibers using image processing and analysis. One TCA was associated with esophageal atresia-tracheoesophageal fistula, the other with trisomy 21. The gradients of calretinin-stained pixel count increase per distance from the beginning of TZ (slope) for TCA were not significantly different from those for the conventional HD group. Given this observation, it is speculated that the length of TZ in TCA may fall within the range of and may not be much longer than conventional colorectal HD.


2020 ◽  
Author(s):  
Simone Keck ◽  
Virginie Galati-Fournier ◽  
Urs Kym ◽  
Michèle Moesch ◽  
Jakob Usemann ◽  
...  

Background & AimsHirschsprung’s disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric nervous cells (ganglia). The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates extramural innervation of acetylcholine-secreting (cholinergic) nerve fibers. Cholinergic signals have been reported to control excessive inflammation, but the impact on HSCR-associated enterocolitis is unknown.MethodsWe enrolled 44 HSCR patients in a prospective multicenter study and grouped them according to their degree of colonic mucosal cholinergic innervation using immunohistochemistry. The fiber phenotype was correlated with the tissue cytokine profile as well as immune cell frequencies using quantitative reverse-transcribed real-time polymerase chain reaction (qRT-PCR) of whole colonic tissue and fluorescence-activated cell sorting (FACS) analysis of isolated colonic immune cells. Fiber-associated immune cells were identified using confocal immunofluorescence microscopy and characterized by RNA-seq analysis. Microbial dysbiosis was analyzed in colonic patient tissue using 16S rDNA gene sequencing. Finally, the fiber phenotype was correlated with postoperative enterocolitis manifestation.ResultsWe provided evidence that extrinsic mucosal innervation correlated with reduced interleukin (IL)-17 cytokine levels and T-helper-17 (Th17) cell frequencies. Bipolar CD14high macrophages colocalized with neurons and expressed significantly less interleukin-23, a Th17-promoting cytokine. HSCR patients lacking mucosal cholinergic nerve fibers showed microbial dysbiosis and had a higher incidence of postoperative enterocolitis.ConclusionThe mucosal fiber phenotype might serve as a new prognostic marker for enterocolitis development in HSCR patients and may offer an approach to personalized patient care and new future therapeutic options. (www.clinicaltrials.gov accessing number NCT03617640)


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