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)

This report describes paraneoplastic visceral neuropathy including achalasia, gastroparesis, subileus and constipation in a 59 year old patient with metastasising atypical bronchial carcinoid. Achalasia was successfully treated by cardiomyotomy and fundoplication; additionally, extramucosal pylorectomy was undertaken to improve gastric emptying. Endoscopic papillotomy was necessary because of a functional stenosis of the sphincter of Oddi with development of obstructive jaundice. Symptoms of intestinal pseudoobstruction did not improve with cisapride or corticosteroid treatment. Histological examination of gastrointestinal specimens revealed a lymphocytic infiltration of the myenteric plexus associated with loss of neurones. The rheumatoid factor was positive, there was evidence of circulating immune complexes and antibodies to Sm-antigen were present, suggesting a possible autoimmune pathogenesis.
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PMID:Paraneoplastic chronic intestinal pseudoobstruction as a rare complication of bronchial carcinoid. 164 19

The association between upper gastrointestinal (GI) motility disorders and respiratory problems is reviewed. Upper GI motility disorders, such as gastroesophageal reflux disease, gastroparesis, and achalasia, have been associated with respiratory problems, including aspiration, airway obstruction, asthma, bronchospasm, chronic cough, and laryngitis. These associations, which had been based solely on clinical observation, have recently been supported by physiologic studies and treatment trials. The association of reflux disease with asthma has the most support. Up to 80% of persons with asthma have evidence of pathologic gastroesophageal reflux, and in several studies antireflux therapy with prokinetic agents, antisecretory drugs, or fundoplication surgery has been found to reduce asthma symptoms and the need for medication in some patients. Reflux has also been associated with chronic cough and laryngitis, and antireflux therapy can reduce respiratory symptoms. Gastroesophageal reflux, gastroparesis, and achalasia are all associated with aspiration. In addition, in rare instances, the megaesophagus associated with achalasia can produce mechanical airway obstruction. Effective therapy for these GI motility disorders can eliminate complicating respiratory problems.
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PMID:Upper gastrointestinal motility disorders and respiratory symptoms. 893 26

Type 1 antineuronal nuclear autoantibody (ANNA-1, also known as "anti-Hu") is a marker of neurologic autoimmunity that is highly associated with small-cell lung carcinoma (SCLC). To determine the spectrum of symptoms and signs as well as the frequency of cancer in adult patients who are seropositive for ANNA-1, we reviewed 162 sequential patients (67% female) identified as ANNA-1-positive in a comprehensive immunofluorescence screening test. In 21% of these patients, the antibody test requested by the physician was not ANNA-1. By the end of the follow-up period, cancer had been found in 142 patients (88%). Ten of these lacked evidence of SCLC (4 had prostate carcinoma, 3 breast carcinoma, 1 both prostate carcinoma and melanoma, 1 lymphoma, and 1 squamous-cell lung carcinoma). Of the 132 patients (81%) with proven SCLC, 17 had one or more coexisting malignant neoplasms (6 had renal carcinoma, 4 another lung primary carcinoma, 3 prostate carcinoma, 3 breast carcinoma, and 4 assorted neoplasms). The diagnosis of SCLC in 128 patients (97%) followed the onset of paraneoplastic symptoms. SCLC was identified in 10 patients by chest MRI after an equivocal chest radiograph or CT; in 28 by bronchoscopy, mediastinoscopy, or thoracotomy; and in 7 at autopsy. Neurologic signs in decreasing frequency were neuropathy (sensory > mixed somatic > autonomic > cranial [especially cranial nerve VIII] > motor), cerebellar ataxia, limbic encephalitis, polyradiculopathy, associated Lambert-Eaton myasthenic syndrome, myopathy, myelopathy, opsoclonus/myoclonus, motor neuronopathy, brachial plexopathy, and aphasia. Nineteen patients had a solely gastrointestinal initial presentation, including gastroparesis, pseudo-obstruction, esophageal achalasia, or other dysmotility. We conclude that seropositivity for ANNA-1 can expedite the diagnosis and treatment of otherwise occult cancer in patients, especially tobacco abusers, with varied neurologic and gastroenterologic presentations. The search for SCLC should not end on discovering a different neoplasm.
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PMID:Paraneoplastic and oncologic profiles of patients seropositive for type 1 antineuronal nuclear autoantibodies. 952 Dec 51

We present a case of a patient with achalasia who developed symptomatic gastroparesis after botulinum toxin injection therapy. Symptoms responded to prokinetics. Pathophysiology of gastric motility disturbances in patients with achalasia is discussed.
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PMID:Symptomatic gastroparesis in a patient with achalasia. 975 85

Gastric function is finely modulated by a series of neurological mechanisms, so that gastric digestion is normally not perceived. Alteration of these control mechanisms may lead to different situations, which are frequently associated with symptoms. An impaired tonic contraction of the proximal stomach, that is, an impaired gastric tone, results in gastroparesis. Patients with functional dyspepsia, and also patients with achalasia, have impaired meal accommodation of the stomach. Interestingly, patients with functional dyspepsia may also have a sensory dysfunction, and both dysfunctions could play a synergistic role. However, the sensory dysfunction in dyspepsia, particularly the types of afferent fibres affected, and the mechanisms of impaired accommodation, still remain to be characterized. Evaluation of gastric function has been approached using the barostat. However, the barostat has limitations and potential technical pitfalls that require proper attention. Meal ingestion induces a variety of reflexes and the net result is a relaxation of the stomach. However, gastric reflexes can be best evaluated with the stomach empty, when the stimuli are applied at a different site. Nevertheless, altered reflex responses may be difficult to interpret. For instance, absent or decreased relaxatory responses may equally correspond to a gastroparetic stomach without tone or to a dyspeptic stomach unable to relax. In this context, it may be important to measure basal tone. Distension of the stomach by means of the barostat has been also used to test gastric sensitivity. However, recent studies have shown that perception of gastric distension relays on stimulation of tension receptors; since wall tension depends on both pressure and volume, distension with the barostat may be difficult to standardize. Hopefully, a battery of tests may become available in the near future for a complete neuromuscular evaluation of the gut.
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PMID:Gastric neurology: evolving concepts and techniques. 983 Dec 67

Dysphagia and chest pain are the two commonest symptoms of abnormalities of oesophageal motility. Dysphagia is to be distinguished into high or oropharyngeal and low or oesophageal dysphagia. Oropharyngeal dysphagia pertains to dysfunction of the pars cricopharyngea of the M. constrictor pharyngis inferior (M. cricopharyngeus), which is frequently associated with a Zenker diverticulum. Treatment consists of endoscopical or surgical myotomy and diverticulectomy. In achalasia there is incomplete relaxation of the lower esophageal sphincter with aperistalsis. The main treatment modalities are endoscopic pneumodilation and surgical myotomy of this sphincter. In dysphagia or non-cardiac chest pain spastic or hypocontractile abnormalities of the oesophageal motility can be involved, these are often difficult to treat. Disorders of gastric motility are mainly gastroparesis and functional dyspepsia. In diabetic gastroparesis, adequate monitoring of the blood sugar level is also necessary. New insights into the pathophysiology of functional dyspepsia concern abnormal visceral sensitivity and reduced adaptive relaxation of the stomach during intake of food.
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PMID:[Gastrointestinal surgery and gastroenterology. VII. Proximal motility disorders in the digestive tract]. 1074 45

It has been demonstrated that nitric oxide (NO) is a major inhibitory nonadrenergic, noncholinergic (NANC) neurotransmitter in the gastrointestinal (GI) tract. NO released in response to nerve stimulation of the myenteric plexus causes relaxation of the smooth muscle. NO is synthesized by the activation of neuronal NO synthase (nNOS) in the myenteric plexus. Released NO plays an important physiological role in various parts of the GI tract. NO regulates the muscle tone of the sphincter in the lower esophagus, pylorus, sphincter of Oddi, and anus. NO also regulates the accommodation reflex of the fundus and the peristaltic reflex of the intestine. Previous studies have shown that NOS inhibitors delay gastric emptying and colonic transit. The reduction of nNOS expression, associated with impaired local production of NO, may be responsible for motility disorders in the GI tract. There is accumulated evidence that dysfunction of NO neurons in the myenteric plexus may cause various GI diseases. These reports are reviewed and possible mechanisms of altered nNOS expression are discussed in this article. In particular, impaired nNOS synthesis of the myenteric plexus seems to be an important contributing factor to the pathogenesis of achalasia, diabetic gastroparesis, infantile hypertrophic pyloric stenosis, Hirschsprung's disease, and Chagas' disease. Reduced NO release and/or nNOS expression are suspicious in a subset of patients with functional dyspepsia. Although the etiology of intestinal pseudo-obstruction remains unknown, it is conceivable that extrinsic denervation may upregulate nNOS expression, resulting in enhanced muscular relaxation and disturbed peristalsis. An animal model of colitis showed impaired nNOS expression in the colonic myenteric plexus. Antecedent infection may be associated with the impaired NO pathways observed in functional dyspepsia, colitis, and Chagas' disease.
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PMID:Pathophysiological significance of neuronal nitric oxide synthase in the gastrointestinal tract. 1276 83

Cholangiocarcinoma most commonly presents as painless progressive jaundice. We report a case occurring in a 56-year-old Chinese woman with an unusual presentation of progressive dysphagia and vomiting. Oesophageal manometric and barium studies were indicative of achalasia, and computed tomography confirmed the presence of cholangiocarcinoma extending to the gastroesophageal junction and proximal lesser curve of the stomach. In this case, a constricting tumour at the gastroesophageal junction with probable invasion of the vagus nerves led to features of achalasia and gastroparesis.
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PMID:Cholangiocarcinoma presenting as pseudoachalasia and gastroparesis. 1290 20

Despite the extensive impact of autonomic function on the gastrointestinal system, there is little understanding of the mechanisms by which specific autonomic abnormalities translate into particular gastrointestinal complaints. Three logical alternatives include: (1) the underlying disorder affects the autonomic and gastrointestinal systems independently; (2) autonomic dysfunction alters gastrointestinal processing directly; (3) gastrointestinal manifestations arise as a delayed, indirect consequence of autonomic dysfunction. The major gastrointestinal manifestations of dysautonomia include esophageal dysmotility such as achalasia, gastroparesis, and small bowel bacterial overgrowth in the upper tract. Lower tract disorders include diarrhea, fecal incontinence, and constipation. Sorting through the varied causes of these disorders requires a careful history and examination in each patient. Supportive diagnostic studies may include radionuclide imaging, motility examination, and electrogastrography. Autonomic studies can (1) distinguish a purely enteric from a more generalized dysautonomia; (2) provide surrogate information about motility; (3) differentiate primary (e.g., multiple system atrophy) from secondary (e.g., irritable bowel syndrome) dysautonomias as the etiology of gastrointestinal symptoms. Several new strategies are available for the treatment of gastroparesis, constipation, irritable bowel, and sphincteric incontinence.
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PMID:Evaluation and treatment of autonomic disorders of the gastrointestinal tract. 1508 66

Botulinum toxin type A is used extensively for the management of gastrointestinal smooth muscle disorders. This review is a comprehensive summary of the current status of this therapy. It includes English-language research from 1966 to 2003 and relevant abstracts from subspecialty meetings from the past 3 years. Botulinum toxin appears to be beneficial for achalasia, gastroparesis, sphincter of Oddi dysfunction, anal fissure and anismus. Very few placebo-controlled trials have been performed despite widespread use of toxin for the past 10 years. Botulinum toxin appears to be safe and side effects are uncommon. Despite uncontrolled data, botulinum toxin is now used for a variety of spastic disorders of GI smooth muscle. In some instances this therapy may preclude the need for more invasive treatments. Controlled trials are needed.
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PMID:The use of botulinum toxin for the treatment of gastrointestinal motility disorders. 1510 53


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