Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The purpose of this paper is to report the experience acquired in pneumatic dilatation in achalasia of the esophagus up to 1990. Two hundred and six patients were studied in that period (X 50, 7 years, M/F 1:1). According to X Rays the distribution was: grade I 17.4%, grade II 54.8%, grade III 14% and grade IV 13.5%. The associated esophageal pathology was: hiatus hernia 9.7%, esophagitis 5.8%, benign stenosis 2.4%, cancer 1.4%, ulcer and diverticula 0.9% and Schatzki's ring and leiomyoma 0.4% respectively Serology for Chagas disease was positive in 23% Chagasic megacolon was more frequent than chagasic heart disease (4.3% Vs. 1.4%). Out of these, one hundred and twenty patients were treated by pneumatic dilatation. To this group we shall refer in more detail. One hundred ant two patients were dilated once and the remaining 18 twice. Esophageal manometry showed a vigorous pattern in 7.7%. The LES' pressure pre-treatment was 24.5 mm Hg and post-dilatation 13.7 mm Hg in 75.8% of the cases the result was good. The morbidity was 5% and the mortality 0.7%. Relapse was seen in 25.8% of the cases. The follow-up was X 38 months. We conclude that pneumatic dilatation is the election procedure in the treatment of achalasia since it offers good results with low morbimortality. Surgery is indicated after failure of 2 dilatations, in children, and association with esophageal neoplasms, hiatus hernia and esophageal diverticula.
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PMID:[Esophageal achalasia: 20 years' experience with non surgical treatment]. 181 99

In electromanometric investigation of the rectoanal region, in 103 of 108 patients with established diagnosis of functional megacolon, anal achalasia--rectoanal dyssynergy was diagnosed. In 12 patients, achalasia of the internal anal sphincter was revealed. Because of ineffectiveness of the conservative treatment, which included digital dilation of the external anal sphincter and rectoanal training with bioadaptive regulation, in 10 of 12 patients, myectomy of the internal anal sphincter was performed with a good result.
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PMID:[Diagnosis and treatment of anal achalasia in functional megacolon in children]. 234 55

The disturbance of defecation in the patient with Hirschsprung's disease and the pathophysiology of constipation in the constipated patient with simple megarectum were investigated. In Hirschsprung's disease, an existence of aganglionosis and the sphincter achalasia are two main factors which cause clinical symptoms such as chronic constipation and megacolon. As a surgical treatment, we have routinely performed our modified Duhamel's operation. Postoperative followup study indicated that the most cases in our series have gained a satisfactory defecatory function 1 to 3 years after operation. In chronically constipated patient with simple megarectum, on the other hand, it was found manometrically that the most of them had significantly high anal canal pressure and incomplete anal relaxation after rectal stimulation. These findings indicate that those patients have hypertonic and achalasic sphincter. Therefore, the authors propose "high anal pressure syndrome (HAPS)" for such constipated patient with simple megarectum. As to the surgical treatment, 6 patients with simple megarectum had a complete posterior internal sphincterotomy and the postoperative results were excellent or good in our series.
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PMID:[Pathophysiology of defecatory disturbance in the patient with Hirschsprung's disease and chronically constipated patients with simple megarectum]. 408 53

Destruction of the myenteric plexus impairs ongoing peristalsis and produces dilatation of the gut lumen with thickening of the gut wall. Achalasia of the cardia, hypertrophic pyloric stenosis and non-Hirschsprung megacolon are well-known examples of damage to the bowel nerve supply. There are many causes, some of which are unknown, but the common ones can be divided into three groups. Congenital developmental failure may be total and incompatible with life, or less severe, in which case resection may be possible. Acquired inflammatory damage, other than Chagas' disease, can occur anywhere in the gut. Autonomic neuropathy may involve either the neurons or the Schwann cells. The former is often due to drugs because the autonomic ganglia are outside the blood-brain barrier. The latter is frequently associated with metabolic disease, such as diabetes mellitus.
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PMID:The neuropathology of pseudo-obstruction of the intestine. 695 Dec 64

A case of achalasia coexistent with sigmoid megacolon in a 38-year-old man with known epilepsy is described. The patient was referred to the Ryukyu University Hospital with a 4-year history of dysphagia and heartburn and a 1-year history of abnormal bowel movement. On admission, upper gastrointestinal (GI) series demonstrated a dilated, tortuous thoracic esophagus with a flask-type configuration. Barium enema studies showed a dilated sigmoid colon from the rectosigmoid junction to the descending colon. Myotomy (modified Jekler-Lhotka's procedure) for achalasia and simple sigmoidectomy for sigmoid megacolon were carried out. The biopsied wall of the narrowed esophageal segment at operation showed decreased numbers of ganglion cells in Auerbach's plexus and atrophy of the muscle fibers. The resected dilated sigmoid colon revealed degeneration and markedly decreased numbers of ganglion cells in Auerbach's and Meissner's plexuses. The patient's postoperative course was uneventful and he has been doing well since surgery. The present case is very interesting and to our knowledge, such a case is rare in the literature. We believe that the abnormalities of the ganglion cells may be due to the same etiologic factor as the sigmoid megacolon. The association of the two pathologic processes is discussed, together with a brief review of the literature.
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PMID:A rare case of achalasia coexistent with sigmoid megacolon and associated with epilepsy. 800 May 13

The digestive system is one of the major sources of nitric oxide, which, due to its smooth muscle-relaxing and vasodilating properties, appears to play a key role in the regulation of gastrointestinal motility, mucosal blood flow and gastroprotection. In addition nitric oxide takes part in the control of pancreatic secretion and liver functions. Recent studies suggest that the substance may be involved in the pathogenesis of achalasia, toxic megacolon, Hirschsprung's disease and portal hypertension.
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PMID:Nitric oxide: an ubiquitous actor in the gastrointestinal tract. 820 Jan 22

The regulation of gastrointestinal function is known to involve elements of the enteric nervous system. Processes such as secretion, motility, blood flow, and immune function are all influenced by a complex network of neurons whose cell bodies lie in the gut. These neurons use a wide spectrum of substances as neurotransmitters, although the majority use peptides once thought to function only as gut hormones. It has been increasingly recognized that abnormalities of this neuroendocrine regulatory system underlie many gastrointestinal disorders. The most obvious are states of peptide excess found in patients with gut endocrine tumors such as carcinoid, gastrinoma, and somatostatinoma. Conversely, other disorders appear to be related to deficiency states. Examples include both achalasia and Hirschsprung's disease (congenital megacolon), where the loss of inhibitory neural action leads to abnormalities of peristalsis and sphincter function. Evidence for abnormal neuroendocrine regulation leading to disease states is increasing for many other gastrointestinal disorders.
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PMID:Neuroendocrine disorders of the gut. 853 22

We report a rare case of achalasia coexistent with megacolon. The patient, a 25-year-old woman, presented at our hospital with a history of abdominal pain with distension, and was finally operated on for a megacolon. Five months later she presented symptoms of progressive dysphagia and heartburn. Oesophageal manometry of the upper and lower oesophageal sphincter and X-ray studies showed images compatible with achalasia. Oesophagomyotomy of the oesophagogastric junction (Heller procedure with Dor haemifundoplication technique) was performed. In the specimens taken for biopsy, neither pathology of the myenteric plexuses, nor atrophy of the muscle fibres was evident. Chagas' disease serological diagnosis for Trypanosoma cruzii, neurological disease, diabetes and all the pathological events related with neuromuscular disorders of the gastrointestinal tract proved negative. We believe that the pathological findings are related to a dysfunction of the physiological mediators of the upper and lower digestive tract motility. The present case is extraordinary and, to our knowledge, extremely rare. The association of the two pathological diseases is questionable, and the literature is reviewed.
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PMID:Idiopathic megacolon associated with oesophageal achalasia. 958 91

Inflammatory neuropathy of the enteric nervous system is emerging as an important topic in the field of neurogastroenterology. Enteric ganglionitis can be either primary or secondary to a wide array of diseases (i.e., paraneoplastic, infectious, and neurological disorders) and is characterized by a dense infiltrate of inflammatory/immune cells mainly confined to the neural microenvironment. The clinical picture reflects the involved segment of the gastrointestinal tract (achalasia, gastroparesis, pseudo-obstruction, and megacolon). In these settings, symptoms may develop either acutely (frequently after a flulike episode in otherwise previously healthy individuals) or more slowly (e.g., in paraneoplastic syndromes). The inflammatory/immune response in enteric ganglionitis leads to neuronal dysfunction and degeneration over time and sometimes results in a complete loss of enteric neurons. The diagnosis of enteric ganglionitis is supported by detection of circulating antineuronal antibodies against select molecular targets, including Hu and Yo proteins, neurotransmitter receptors, and ion channels. Potential mechanisms involved in neuronal dysfunction include viral antigen expression in the enteric neural environment, molecular mimicry (onconeural antigens), and the role exerted by cellular and humoral autoimmunity. A short course of steroid or other immunosuppressive therapy has been shown to be helpful in the treatment of these conditions. This feature reinforces the concept of a cause/effect relationship of the immune-mediated insult damaging the enteric innervation. An increased awareness of the clinical features and the immunologic and neurodegenerative mechanisms of these forms of peripheral neuropathy is important to correctly diagnose this problem during the early stages of the disease process and to provide appropriate immunosuppressive therapies.
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PMID:Inflammatory neuropathies of the enteric nervous system. 1518 82

The aim of this study is to review current understanding of the molecular and morphological pathology of the enteric neuropathies affecting motor function of the human gastrointestinal tract and to evaluate the described pathological entities in the literature to assess whether a new nosology may be proposed. The authors used PUBMED and MEDLINE searches to explore the literature pertinent to the molecular events and pathology of gastrointestinal motility disorders including achalasia, gastroparesis, intestinal pseudo-obstruction, colonic inertia and megacolon in order to characterize the disorders attributable to enteric gut neuropathies. This scholarly review has shown that the pathological features are not readily associated with clinical features, making it difficult for a patient to be classified into any specific category. Individual patients may manifest more than one of the morphological and molecular abnormalities that include: aganglionosis, neuronal intranuclear inclusions and apoptosis, neural degeneration, intestinal neuronal dysplasia, neuronal hyperplasia and ganglioneuromas, mitochondrial dysfunction (syndromic and non-syndromic), inflammatory neuropathies (caused by cellular or humoral immune mechanisms), neurotransmitter diseases and interstitial cell pathology. The pathology of enteric neuropathies requires further study before an effective nosology can be proposed. Carefully studied individual cases and small series provide the basic framework for standardizing the collection and histological evaluation of tissue obtained from such patients. Combined clinical and histopathological studies may facilitate the translation of basic science to the clinical management of patients with enteric neuropathies.
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PMID:Human enteric neuropathies: morphology and molecular pathology. 1550 May 8


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