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)

A 7-month-old dystrophic infant weighing 3.8 kg was investigated endoscopically in relation to recurrent pneumonia. A tracheo-esophageal cleft was demonstrated. The situation was complicated by the coexistence of achalasia of the cardia. There was also hypertelorism and malformation of the urinary tract, so that this would appear to be a case of the G-Syndrome. A Heller's operation with fundoplication and gastrostomy was performed and only after this was the tracheooesophageal cleft closed. A flap of sternomastoid muscle was interposed between the trachea and oesophagus. Stenosis was treated by dilatation.
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PMID:[Laryngo-tracheo-oesophageal cleft and G-syndrome--interposition of a flap from the sternocleidomastoid muscle (author's transl)]. 728 28

The technique of 24 hour esophageal pH monitoring (24 hour pH test) is described. Experience with the 24 hour pH test in 393 patients with suspected esophageal disease has shown the clinical usefulness of the test in objectively determining the presence of gastroesophageal reflux. The test was effective in evaluating atypical symptoms of gastroesophageal reflux such as respiratory symptoms and chest pain and, in children, failure to thrive and recurrent pneumonia. The 24 hour pH test was particularly useful in evaluating patients who were referred with other abdominal or thoracic disease and had, in addition, symptoms suggestive of gastroesophageal reflux on history. The test helped to unsnarl the cause of recurrent symptoms after an esophageal myotomy for achalasia or an antireflux procedure. Of 179 patients with typical symptoms of gastroesophageal reflux, 27% had normal 24 hour test results and were subsequently diagnosed as having another cause for their symptoms. Of 146 patients who had normal findings on esophagoscopy, 54% were shown to have abnormal gastroesophageal reflux on 24 hour pH monitoring, indicating lack of sensitivity of endoscopy to detect reflux. In addition, the 24 hour pH test identified patterns of abnormal reflux and indicated those patients most at risk for development of stricture. The test is well tolerated by the patients, simple to use, and dependable when performed and read as described. The clinical use of the 24 hour pH test brings objectivity to the evaluation of exophageal disease that has hitherto not been available.
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PMID:Technique, indications, and clinical use of 24 hour esophageal pH monitoring. 736 33

We reviewed our experience in the diagnosis and management of esophageal achalasia in 33 children over a 25-year period at a single center by a retrospective chart review of all patients diagnosed with achalasia between December 1, 1975 and January 30, 2001. There were 33 cases ranging from 5 months to 16 years of age at the time of presentation (17 boys and 16 girls). Although dysphagia and vomiting were the commonest presenting symptoms, weight loss, chest pain, coughing, and recurrent pneumonia also occurred in many patients. Barium contrast study of the esophagus was the initial diagnostic modality followed by esophageal manometry. An upper endoscopy was also performed in 78.7% of cases. Management was predominantly surgical; however, seven recently diagnosed patients opted for botulinum toxin (botox) injection as the first line of treatment. The follow-up duration varied from 10 months to 10 years (mean 4.71 +/- 3.2 years). Postsurgical complications included gastroesophageal reflux disease in five patients who had not received a simultaneous antireflux procedure and "residual achalasia" in two patients, who both responded to a single botox injection.
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PMID:A review of achalasia in 33 children. 1245 92

In this article, the literature regarding the effects of achalasia cardiae on pulmonary disorders is reviewed. We specifically focused on the issue how achalasia may be associated with: airway obstruction, stridor, cough and recurrent pneumonia. Authors suggested it is necessary to carry out full differential diagnosis considering achalasia cardiae in patients with unsatisfactory treatment of recurrent respiratory tract diseases.
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PMID:[Pulmonary masks of achalasia cardiae]. 1652 70

We report on the case of a 3-year-old child presenting bilateral bronchiectasis due to recurrent pneumonia with esophageal achalasia. The final diagnosis was triple A syndrome. This presentation is particularly atypical and rare at this age.
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PMID:[Bronchiectasis revealing triple A syndrome]. 2604 40