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

The effect of nifedipine (10-20 mg) on esophageal motility was tested in 18 patients with clinical esophageal syndrome and radiologic aspect suggesting esophageal spasm. The drug was administered 20-30 min prior to the second radiologic examination. In 3 cases of achalasia the drug was administered in doses of 10 mg/day for 10 days before the second X-ray examination. Fiber esophagoscopy was performed in all the cases. The initial radiologic aspect was favourably influenced by nifedipine administration in 12 cases confirmed as esophageal spasm. The other 6 patients who did not benefit by the test were cases of organic diseases: 2 post caustic stenoses and 4 cases of eso-cardio-tuberal neoplasm. In the 3 cases of achalasia, treatment with nifedipine led to clinical improvement with relaxation of inferior esophageal contraction on radiologic examination. The study demonstrated the favourable contribution of calcium channel inhibitors to the diagnosis and treatment of esophageal motility disturbances.
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PMID:The diagnostic and therapeutic contribution of calcium channel inhibitors (nifedipine) in esophageal spasm. 228 70

Although achalasia is usually of idiopathic origin, it may be secondary to another disease process such as neoplasia. The first description of a familial achalasia syndrome that appears to be secondary to diffuse esophageal leiomyomatosis with entrapment of nerve ganglia is presented. Documented in four generations of a family, the disease followed an autosomal dominant mode of inheritance. Long lower esophageal sphincter pressure zones and a high incidence of epiphrenic diverticula were interesting accompaniments of achalasia in this family. Many achalasia-affected family members have also had associated intestinal leiomyomas or neurofibromas. Affected family members also had urticaria pigmentosa, and some had systemic mast cell disease as well.
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PMID:Achalasia due to diffuse esophageal leiomyomatosis and inherited as an autosomal dominant disorder. Report of a family study. 232 26

Primary motility disorders of the esophagus require the exclusion of intramural tumors. The procedures currently used for the differential diagnosis of achalasia such as endoscopy with biopsy, esophageal and gastric radiography, abdomino-thoracic computed tomography and intraluminal esophageal manometry, are unsatisfactory when a tumor growing intramurally is suspected. A more recent method of studying the integrity of the gastrointestinal wall and its surrounding tissue is endoscopic ultrasonography. In 16 patients suspected of having achalasia, endosonography was performed in addition to the above-mentioned conventional examinations. Fifteen of them showed a normal ultrasonic structure of the wall of the gastro-esophageal junction, with no sign of hypertrophy of the smooth muscle layer. In the remaining case endoscopic ultrasonography was able to detect an intramural tumor, as evidenced by the inhomogeneous ultrasonic structure of the esophageal wall. Computed tomography and all the other conventional diagnostic procedures used failed to demonstrate this tumor. In conclusion, the findings presented strongly suggest that endosonography can contribute to the differential diagnosis of achalasia and intramural tumors.
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PMID:Endosonographic appearance of the esophagus in achalasia. 240 24

We present a case of secondary achalasia due to an adenocarcinoma of the stomach with no tumor infiltration of the esophagus. Immunohistochemical staining revealed a massive infiltration of activated eosinophils in the muscularis of the esophagus with secretion of the highly cytotoxic and neurotoxic eosinophil cationic protein (ECP). Immunohistochemical staining for the neuropeptides VIP and substance P, as well as the histochemical demonstration of AChE, revealed a nearly total absence of all three neurotransmitters/modulators compared to control. The hypothesis is advanced that eosinophil neurotoxicity is the cause of secondary achalasia.
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PMID:Severe destruction of esophageal nerves in a patient with achalasia secondary to gastric cancer. A possible role of eosinophil neurotoxic proteins. 246 64

Diffuse esophageal leiomyomatosis is characterized by diffuse muscular hypertrophy chiefly marked in the lower end of the esophagus looks at a large tumor. It involves the entire length of the esophagus and down to the upper part of the stomach (esogastric leiomyomatosis). Diffuse leiomyomatosis is a extremely rare pathology encountered in children (13 previous report cases) and young adults. Il may be isolated or found in association with other intrathoracic and genital localisations or Alport syndrome (nephropathy, sensorineural deafness, ocular lesions). Extensive leiomyomatosis, engulfing the trachea and stem bronchi, may involve acute respiratory insufficiency. Genital localisations, exclusively in women (clitoral hypertrophy, vulvar leiomyomatosis), in association with esophageal leiomyomatosis, realize the esophago vulvar syndrome. Recently, familial diffuse leiomyomatosis cases were described with, in association, esophageal and extra-esophageal leiomyomatosis and Alport syndrome. The majority of the patients developed esophageal or respiratory symptoms. The radiological appearance is that of a mediastinal tumor or achalasia. CT Scans findings can give evidence diffuse muscular thickening of esophageal wall. Prognosis depends on the associated lesions. In that reports, two young adults (27 and 39 years old) died of inhabitual carcinomas (esophageal and gallbladder carcinoma). The only surgical treatment for symptomatic esogastric leiomyomatosis is subtotal esophagectomy with proximal gastrectomy and esocoloplasty. Myotomy is ineffective (2 cases). Five cases of esophageal leiomyomatosis are described (3 children, 2 young adults). Among these, two are familial leiomyomatosis cases. An esophageal resection was performed in four patients.
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PMID:[Diffuse esophageal leiomyomatosis. Apropos of 5 cases with 2 familial cases]. 261 74

Six patients with known achalasia were examined by endoscopic ultrasonography before dilatation therapy. At the level of the lower esophageal sphincter, a typical enlargement of the echolayer corresponding to the muscularis propria was observed in 5 cases. Endoscopic ultrasonography is a complementary procedure to manometry and x-ray for diagnosing achalasia. It helps differentiate achalasia from pseudoachalasia. In pseudoachalasia there is tumor infiltration.
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PMID:Endoscopic ultrasonography in achalasia. 240 30

Endoscopic ultrasonography (EUS) was performed prospectively in 38 patients with various esophageal disorders. Twenty-four had a histologically proven carcinoma and EUS was done to assess its ability in preoperative staging. In 9 of 24 patients (37.5%), tumor stenosis could be passed with the endoscope and EUS preoperative findings regarding tumor extension and the presence of enlarged periesophageal lymph nodes were confirmed in those operated on (n = 4). In five patients with achalasia, a periesophageal tumor was reliably excluded by EUS. In one of four patients with Barrett's esophagus, EUS demonstrated a small (less than 1 cm) carcinoma that could not be visualized with any conventional technique. For differentiation of cancer recurrence after operation from periesophageal scar tissue EUS-guided transmural biopsies are needed. Our experience shows that at the present time EUS is the most reliable method to demonstrate small (less than or equal to 1 cm) intra- and extramural esophageal lesions and that it should therefore be applied early in the work-up of patients with dysphagia.
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PMID:Endoscopic ultrasonography in esophageal diseases. 266 1

In a simple radiologic test, amyl nitrite inhalation seems to distinguish patients with pseudoachalasia from those with idiopathic achalasia. In three pseudoachalasia patients with tumor infiltration of the distal esophagus, the narrow lower esophageal sphincter segment was unaffected by the smooth-muscle relaxant effect of amyl nitrite. In contrast, amyl nitrite caused a measurable increase of 2 mm or more in sphincter diameter in nine patients with idiopathic achalasia. The amyl nitrite test may be a useful method for distinguishing patients with pseudoachalasia from those with idiopathic achalasia.
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PMID:Radiologic amyl nitrite test for distinguishing pseudoachalasia from idiopathic achalasia. 286 1

Secondary or "pseudo" achalasia of the esophagus can mimic idiopathic achalasia radiographically and can be difficult to diagnose. Typically, it is due to invasive carcinoma involving the gastroesophageal junction, usually gastric adenocarcinoma. Occasionally, an achalasialike condition can be produced by tumors not involving the gastroesophageal junction. We report 2 cases, 1 of lung carcinoma and the other of hepatoma, in which the patients had radiographic and endoscopic changes compatible with achalasia. However, the onset of symptoms was abrupt and the patients were elderly; these are unusual features for primary achalasia. There have been several other reports of nongastrointestinal neoplasms producing a clinical and radiographic picture similar to achalasia. Although there are several theories as to the cause, our cases would support the concept that direct tumor involvement of the gastroesophageal junction is not necessary to produce significant esophageal dysmotility.
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PMID:Achalasia secondary to nongastrointestinal malignancies. 299 89

Vasoactive intestinal peptide (VIP), first isolated from the gut, was originally considered a candidate gastrointestinal hormone. Since about 1975, however, it has become increasingly clear that it is primarily a neurotransmitter or neuromodulator and that it exerts its functions mainly by local release from nerve endings. VIP plays a hormonal role only when it is released in large amounts from a tumor, with a consequent overflow into the circulation and grossly elevated plasma concentrations of the peptide. Moderately increased VIP plasma and tissue concentrations that cause mainly local effects are found in intestinal ischemia. Crohn's disease and some other chronic inflammatory diseases of the bowel. VIP is also measured in increased amounts in the normal fetus and neonate, where it may play an important physiological role. Such an increase of VIP levels in the circulation could enhance perfusion and metabolic activity of tissues during their rapid-growth period. On the other hand, disorders with a disturbed VIP function such as achalasia and Hirschsprung's disease and possibly also asthma and cystic fibrosis seem to be characterized mainly by a derangement of smooth muscle activity and/or exocrine secretion. Considering this list of disorders where VIP has either a proven or suspected role, it is easy to imagine the significance of this peptide in pediatric pathophysiology.
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PMID:[Vasoactive intestinal polypeptide (VIP)--possible importance in diseases of childhood]. 299 82


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