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 enteric nervous system (ENS) is an important neural network that integrates, modulates and coordinates various motor functions of the digestive tract. Chagas' disease, to a variable extent and in a nonsystematic way, causes destruction of ENS neurons throughout the digestive tract. The studies carried out on the pathophysiology of the digestive form of this disease have provided, as a corollary, a better understanding of the participation of the ENS in the regulation of the normal motor activities of the digestive tract and have led to the conclusion that the disease is a true model of intrinsic denervation of the human digestive tract. Among the motor alterations caused by impairment of the digestive tract in Chagas' disease are: absence of peristalsis in the esophagus, achalasia of the cardia, loss of the accommodation reflex in the proximal stomach, gastric emptying disorders, changes in the migrating motor complex (MMC), disappearance of postprandial motor phenomena in the colon and rectum, lack of relaxation of the internal sphincter of the anus, and anomalous responses of the gall bladder to cholekinetic stimuli.
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PMID:Chagas' disease: a model of denervation in the study of digestive tract motility. 393 3

A prospective study was performed in 17 patients with achalasia of the esophagus determining the manometric characteristics of the gastroesophageal sphincter, correlating it with histological analysis by biopsies taken during surgery at the distal narrowed segment of the esophagus, at the location of the sphincter. The histological findings were compared to 10 control cases. Presence or absence of ganglion cells at the Auerbach's plexuses and appearance of smooth muscle fibers were evaluated. Only one case (6%) had Chagas' disease. The mean sphincter pressure was 41 mm Hg, with incomplete relaxation in all patients. Histological analysis showed a complete disappearance of ganglion cells in 94% of the cases and a decrease in the number of neurons with marked chronic inflammatory cells in one case (6%). In all control cases, the ganglion cells were normal. Smooth muscle fibers were normal on light microscopy. No relationship was found between resting gastroesophageal sphincter pressure, length and relaxation, and histological findings at the distal esophagus. These findings suggest that the denervation in the majority of cases is located in the Auerbach plexus, with complete absence of ganglion cells and, therefore, absence of postganglionic nerve fibers.
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PMID:Gastroesophageal sphincter pressure and histological changes in distal esophagus in patients with achalasia of the esophagus. 402 10

The physiologic similarities between the megaesophagus of Chagas' disease and idiopathic achalasia are well documented. Therefore, it would seem reasonable that comparisons of controlled trials of therapy for the more common Chagas' megaesophagus could be applied to idiopathic achalasia, where the paucity of cases makes such a controlled comparison difficult. We had the opportunity to study 18 patients with achalasia secondary to Chagas' disease. All of the patients were from the mid central states of Brazil, all had symptoms of dysphagia and radiographic documentation of dilated esophagus and abnormal peristalsis (Rezende Groups II and III), as well as positive serologic evidence of Chagas' disease. Perfused intraluminal manometric studies were performed on all patients. Resting sphincter pressures ranged from 20-35 mm./Hg., mean of 25 (normal 5-12 mm./Hg.) with aperistalsis. Patients were randomly dilated with either bouginage (44-55 ff catheter) or pneumatic dilator (4-4.5 kg./6.5 cm.2 x 2 min.). Although all patients reported symptomatic improvement several days after either procedure, repeat manometric tracings demonstrated no change in the sphincter pressure in the bouginage group. The pneumatically dilated group, however, demonstrated a decrease in sphincter pressure to normal levels (mean 12 mm./Hg.). Follow-up studies one year after the procedure confirmed the persistence of normal sphincter pressure in the pneumatically dilated group but no change, as well as return of initial symptoms, in the bouginage group.
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PMID:A clinical trial with pre- and post-treatment manometry comparing pneumatic dilation with bouginage for treatment of Chagas' megaesophagus. 678 91

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

With aim of determining if there are predictive factors in the treatment of achalasia of the esophagus, we analyzed in a group of 119 patients variables such as age, gender, X-Rays, Chagas' disease serology and esophageal manometry before and after treatment. The only significant factor was the LES pressure post-treatment but, since the sampling was heterogeneous, and the differences where the same between bad and good results, we believe it has no predictive value in the treatment of achalasia of the esophagus.
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PMID:[Predictive factors in the treatment of esophageal achalasia]. 778 99

A symptomatic megaesophagus was demonstrated in a three months old boy with Chagas disease. He had a difficult evolution with frequent aspiration pneumonias and progressive undernutrition. At 14 years old a cardiomyotomy (Heller's procedure) was performed, with good results that allowed a near normal life. At 27 years old, the patient started with progressive dysphagia due to an epidermoid esophageal carcinoma and died after surgery. Since a higher frequency of esophageal carcinoma is observed in achalasia, periodic endoscopic examinations are mandatory.
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PMID:[A case of congenital chagasic megaesophagus: evolution until death caused by esophageal neoplasm, at 27 years of age]. 780 23

We present a case of a 19 year old female patient with dysphagia for 4 months. Radiologic, endoscopy and manometric examinations were compatible with the diagnosis of idiopathic achalasia. Clinical, epidemiologic and serologic investigation was negative for Chagas' disease. When she was three years old she had acute poliomyelitis that left muscular atrophy in her left leg. It is possible that lower esophageal sphincter achalasia was the consequence of lesion in the dorsal motor nucleus of the vagus nerve caused by poliomyelitis. The association between poliomyelitis and achalasia supports the infective hypothesis as the cause of achalasia.
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PMID:Achalasia occurring years after acute poliomyelitis. 814 35

Pseudoachalasia or secondary achalasia is commonly recognized and sought for in the context of an elderly patient presenting with weight loss and brief duration of symptoms. The majority of cases are caused by adenocarcinomas of the fundus or the cardia. It is accepted by gastroenterologists that endoscopy with "routine retroflexion" and biopsy are necessary in any newly diagnosed case of achalasia. Benign causes of pseudoachalasia are extremely rare in this country. In developing countries and South America, Chagas' Disease may mimic achalasia. Herein, we present a case of secondary achalasia linked to an unrecognized mediastinal pancreatic pseudocyst that resolved with appropriate treatment of the underlying cause.
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PMID:Pancreatic pseudocyst mimicking idiopathic achalasia. 944 85

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

Evidence accumulated by our investigations over the years give adequate proof for the existence of circulating antibodies in Chagas disease which bind to beta adrenergic and muscarinic cholinergic receptor of myocardium. The interaction of agonist-like antibodies with neurotransmitter receptors, triggers in the cells intracellular signal transductions that alter the physiological behaviour of the target organs. These events convert the normal cells into pathologically active cells. The interaction of antibodies with heart beta adrenergic and cholinergic receptors triggers physiologic, morphologic, enzymatic and molecular alterations, leading to tissue damage. The analysis of the prevalence and distribution of these antibodies reveals a strong association with cardiac and esophageal autonomic dysfunction in seropositive patients in comparison with those without alteration of the heart and esophagus autonomic disorders: therefore, the presence of these antibodies may partially explain the cardiomyoneurophathy and achalasia of Chagas disease, in which the sympathetic and parasympathetic systems are affected. The deposit of autoantibodies behaving like an agonist on neurotransmitter receptors, induceds desensitization and/or down regulation of the receptors. This in turn, could lead to a progressive blockade of neurotransmitter receptors, with sympathetic and parasympathetic dennervation, a phenomenon that has been described during the course of Chagas cardioneuropathy and achalasia. The clinical relevance of these findings is the demonstration, using biomolecules, of a strong association between the existence of circulating autoantibodies against peptides corresponding to the second extracellular loop of the human heart beta, adrenoceptor and M2 cholinoceptor in chagasic patients, and the presence of dysautonomic symptoms, making these autoantibodies a proper early marker of heart and digestive autonomic dysfunction.
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PMID:Overview of molecular mechanisms in chagasic cardioneuromyopathy and achalasia. 1066 47


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