Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.1.7 (acetylcholinesterase)
 28,390 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study is based on 17 neonates suffering from total colonic aganglionosis. The male:female ratio was 2:3 and there was a significant familial occurrence. The ages on admission varied between 1 and 90 days. The clinical presentation was extremely variable. Early diagnosis depends on clinical awareness of the condition in neonates who have intestinal obstruction or diarrhea, or both. The most important radiologic indication was retention of barium for 24 hours. Results of manometric studies were misleading. Suction biopsy of the rectum provided the only sure method of diagnosis, although determination of cholinesterase activity in the biopsy specimen and in the patient's serum was of some value. Two patients died before operation and two died from total colonic and small intestinal aganglionosis. Eight patients survived both the initial ileostomy and subsequent pull through operation. Of the various procedures utilized, the Lester Martin operation has proved to be the most satisfactory.
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PMID:Total colonic aganglionosis. 71 62

Rectal suction biopsy with acetylcholinesterase (AChE) histochemistry has gained increased acceptance as the means of definitely diagnosing Hirschsprung's disease (HD) as well as of excluding this diagnosis when evaluating children with low intestinal obstruction or chronic constipation since the report of Meier-Ruge et al. in 1972. But this AChE histochemical study has not been reported yet in Korea. During the 14-month period from April, 1991 through June, 1992, 37 children, aged 3 days to 17 years had rectal suction biopsies for the diagnosis or exclusion of HD. In this study, AChE histochemistry (N = 37) was compared with hematoxylin & eosin (H&E) staining of same suction biopsy specimens (N = 35) for diagnostic accuracy. The histochemical criterion used for the diagnosis of Hirschsprung's disease was that of Chow et al. (1977), i.e., the presence of many coarse discrete cholinergic fibers in the muscularis mucosae and in the immediately subjacent submucosa regardless of an infiltration of cholinergic fibers in the lamina propria. Of 13 biopsies from the patients with Hirschsprung's disease (N = 13), there were 12 positive reactions, and one false negative reaction in a neonate with total colonic aganglionosis. All biopsies from 24 unaffected children demonstrated negative reactions with no false positive reaction. In comparison, of the 35 specimens examined by H&E staining, ganglion cells were present in the submucosal Meissner's plexus only in 15 of these 24 unaffected children. In conclusion, a 97% diagnostic accuracy was achieved with AChE histochemistry compared with a 74% accuracy with H&E staining (P < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Acetylcholinesterase histochemistry of rectal suction biopsies in the diagnosis of Hirschsprung's disease. 129 39

Rectal mucosal biopsies were performed in 146 patients suspected of having Hirschsprung's disease. The biopsies were serially sectioned and screened for the presence of ganglion cells. In the 101 cases in which ganglion cells were found, the diagnosis of Hirschsprung's disease was ruled out. In 45 patients, histologic examination revealed the typical features of Hirschsprung's disease, i.e., the absence of ganglion cells and the presence of numerous hypertrophied nerve bundles. The varied morphologic features of Meissner's plexus in patients of different ages and the diagnostic pitfalls are described in detail. Serial frozen sections stained with hematoxylin-eosin were examined in eight cases of neonatal intestinal obstruction and enterocolitis. This method was found to be useful and reliable in emergencies. In three of these cases ganglion cells were found and major surgical procedures were avoided. In 73 cases, staining for acetylcholinesterase activity was performed. The results matched the microscopic findings in all but three false-positive cases.
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PMID:Rectal mucosal biopsy in aganglionosis and allied conditions. 619 84

Motility disturbances following prolonged intestinal obstruction have been attributed to secondary effects. This study aimed to demonstrate the effects of incomplete obstruction on the enteric nervous system (ENS) of a rat model. Surgical placement of a nonstrangulating ligature encircling the distal bowel was performed in 41 freshly weaned rats. Anesthetic protocol included Ketamine, ether, or Xylazine (an alpha 2-adrenergic agonist). Histological evaluation was by ganglion cell morphology, histochemical staining for acetylcholinesterase (AChE) and tyrosine hydroxylase (TOH) immunocytochemistry. Forty-one freshly weaned LE rats were divided into controls (8), sham procedures (8), intestinal obstruction (16), and a group of rats with colonic biopsy performed prior to and following experimental obstruction (9). The rats were sacrificed at periods varying between 14 and 45 days post experimental obstruction (median survival, 27 days). Histological changes included elongation of ganglion cells and a decrease in the number per 5-mm slide in obstructed animals. No other obstruction specific differences were detected. A significant (P < .01) increase in AChE in the submucous plexus was recorded in Xylazine-anesthetized animals. No obstruction-specific effects could be demonstrated in the ENS, suggesting that prolonged obstruction without ischemia does not result in any significant alterations in the ENS. Pharmacological stimulation of the alpha 2-adrenergic receptor appeared to result in an increase in AChE. This mechanism may help to explain a possible role for the adrenergic system in the increased AChE levels in affected bowel in patients with Hirschsprung's disease.
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PMID:Secondary effects of prolonged intestinal obstruction on the enteric nervous system in the rat. 790 22

Adhesions in the peritoneal cavity have been implicated in the cause of intestinal obstruction and infertility, but their role in the aetiology of chronic pelvic pain is unclear. Nerves have been demonstrated in human pelvic adhesions, but the presence of pain-conducting fibres has not been established. The purpose of this study was to use an animal model to examine the growth of nerves during adhesion formation at various times following injury and to characterize the types of fibres present. Adhesions were generated in mice by injuring the surface of the caecum and adjacent abdominal wall, with apposition. At 1-8 weeks post-surgery, adhesions were processed and nerve fibres characterized histologically, immunohistochemically, and ultrastructurally. Peritoneal adhesions had consistently formed by 1 week after surgery and from 2 weeks onwards, all adhesions contained some nerve fibres which were synaptophysin, calcitonin gene-related peptide, and substance P-immunoreactive, and were seen to originate from the caecum. By 4 weeks post-surgery, nerve fibres were found to originate from both the caecum and the abdominal wall, and as demonstrated by acetylcholinesterase histochemistry, many traversed the entire adhesion. Ultrastructural analysis showed both myelinated and non-myelinated nerve fibres within the adhesion. This study provides the first direct evidence for the growth of sensory nerve fibres within abdominal visceral adhesions in a murine model and suggests that there may be nerve fibres involved in the conduction of pain stimuli.
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PMID:Growth of nerve fibres into murine peritoneal adhesions. 1105 24

Acute colonic pseudo-obstruction (Ogilvie's syndrome) can be defined as a clinical condition with symptoms, signs and radiological appearance of acute large bowel obstruction unrelated to any mechanical cause. Recent reports of the efficacy of cholinesterase inhibitors in relieving acute colonic pseudo-obstruction have fuelled interest in the pharmacological treatment of this condition. The aim of the present review is to outline current perspectives in the pharmacological treatment of patients with acute colonic pseudo-obstruction. The best documented pharmacological treatment of Ogilvie's syndrome is intravenous neostigmine (2-2.5 mg), which leads to quick decompression in a significant proportion of patients after a single infusion. However, the search for new colokinetic agents for the treatment of lower gut motor disorders has made available a number of drugs that may also be therapeutic options for Ogilvie's syndrome. Among these agents, the potential of 5-hydroxytryptamine-4 receptor agonists and motilin receptor agonists is discussed.
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PMID:Review article: the pharmacological treatment of acute colonic pseudo-obstruction. 1168 85

The incidence of Hirschsprung's disease (HD) was determined in children who presented with constipation to a specialist paediatric surgical unit. During a 5-year period, 355 rectal biopsies were performed on 182 neonates, infants and children presenting with chronic constipation or intestinal obstruction: 25 (14%) were diagnosed HD. One hundred and four patients had suction and 78 had full-thickness rectal biopsies. Haematoxylin-eosin (HE) staining and acetylcholinesterase (AChE) histochemistry was used. In 13 cases (8%) of suction and 2 cases (2.5%) of full thickness rectal biopsies, specimens were inadequate to diagnose HD. The mean age of all patients was 2.9 years and that of patients diagnosed with HD was 3.64 months. Nineteen patients with HD were diagnosed in the first month, 5 in 1-12 months and 1 at 4 years of age (Fig. 1). The authors found that along with onset of constipation convincing indications for rectal biopsy to exclude HD were as follows: those infants and children who do not pass meconium within 48 hours, have low intestinal obstruction of unknown cause, severe constipation, chronic abdominal distension and failure to thrive. A diagnostic accuracy of 94% was achieved with AChE histochemistry for suction rectal biopsy. After this review, referring paediatricians were advised that screening of other common organic causes of constipation with the least invasive investigations, including laboratory, dietary and paediatric gastroenterology advice, should be undertaken to avoid unnecessary rectal biopsy to exclude HD and related disorders.
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PMID:The constipated child: how likely is Hirschsprung's disease? 1269 67

The etiology of neuronal intestinal dysplasia remains largely unknown. There is, however, supporting evidence of the existence of Hirschprung's disease or chronic intestinal obstruction associated with neuronal intestinal dysplasia. With the aim of investigating the possible development of neuronal intestinal dysplasia linked to chronic intestinal obstruction, we have examined the enteric nervous system response to long-term obstruction in a rat model. Three different surgical techniques were tested in Wistar male rats. In animals that survived longer than the cutoff chronic intestinal obstruction point (6 weeks), full-thickness biopsies and acetylcholinesterase (AChE), NADH, hematoxylin-eosin, and anti-S100 protein stainings were performed. The results of our model indicate that chronic intestinal obstruction induced different degrees of enteric nervous system dysplasia, including histological features of neuronal intestinal dysplasia. The relationship between chronic intestinal obstruction and anomalies of the enteric nervous system, including neuronal intestinal dysplasia, needs to be further studied.
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PMID:Evidence of secondary neuronal intestinal dysplasia in a rat model of chronic intestinal obstruction. 1476 26

The aim of this study was to analyze the short- to mid-term outcome of primary modified Georgeson's laparoscopy-assisted endorectal pull-through (PMGLEPT) for Hirschsprung's disease (HD). HD patients treated by PMGLEPT were evaluated prospectively by a single surgeon using a standard structured questionnaire to assess complications, incidence of enterocolitis, and evaluate continence (CE). CE involved scoring five parameters (frequency of motions, severity of staining/soiling, severity of perianal erosions, anal shape, and requirement for medications) on a 3-point scale (0, 1, and 2 for each parameter). Thus, scores for CE were: 10 = normal, 8-9 = good, 6-7 = fair, and 0-5 = poor. Our modifications include transanal rectal dissection starting below or on the dentate line, near total excision of the posterior rectal cuff, and intraoperative acetylcholinesterase staining to accurately identify normal colon. Patients with total colon aganglionosis or trisomy-21 were excluded, leaving 33 cases of PMGLEPT performed between 1997 and 2004. Mean operative age was 11.0 months. Follow-up ranged from 8 months to 7 years (mean 4.0 years). There were no intraoperative complications. Post-PMGLEP, bowel obstruction occurred in 1 subject who required middle colic division for pull-through (PT), and enterocolitis occurred in 3 (9.1%) of 33 patients. In 20 subjects aged over 3 years with a follow-up period of more than 12 months, final CE was normal in 5, good in 10, fair in 4, and poor in 1, despite staining/soiling being present in 12 (60%) of 20 subjects. None of the 33 had constipation. Our results suggest that PMGLEPT is safe with acceptable outcome in the short- to mid-term. However, careful long-term follow-up is mandatory as there appears to be a relatively high incidence of staining/soiling on short- to mid-term follow-up.
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PMID:Prospective analysis of primary modified Georgeson's laparoscopy-assisted endorectal pull-through for Hirschsprung's disease: short- to mid-term results. 1613 14

Isolated Intestinal neuronal dysplasia is a disease characterized by symptoms of diarrhea or intestinal obstruction along with histopathologic diagnosis based on hyperplasia of submucous plexus with giant ganglia containing more than seven nerve cells, increased acetylcholinesterase activity in the lamina propria and around the submucosal blood vessels and heterotopic ganglia in the lamina propria. The aims of this study have been to determine the incidence of the congenital malformations associated with the isolated intestinal dysplasia type B (not associated to another anomaly of the enteric nervous system) and to correlate them with the severity of the symptoms and their possible familial involvement. We have retrospectively reviewed all the patients diagnosed with IND type B nonassociated to Hirschsprung's disease in our hospital from 1981 to 2002. Our serie consists of 44 cases, 1 for every 7500 newborn. 35% of the patients presented associated congenital anomalies, with digestive malformations being the most commonly found (20% of the total). 40% of the patients studied had previous history of constipation in the family. Onset of symptoms was detected in 75% of children during newborn period.
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PMID:[Intestinal neuronal dysplasia: association with digestive malformations]. 1801 45


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