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)

In children with asthma, routine chest X-ray typically shows bilaterally increased air volume, low diaphragms, wide diaphragmatic angles, and often a slender cardiac silhouette with a prominent pulmonic arch. Such an X-ray is not diagnostic of asthma itself, however, but rather of its complications: pneumonitis (particularly in toddlers with infectious asthma), atelectasis due to mucus obstruction, and, rarely, extra-alveolar air trapping (pneumomediastinum with or without cutaneous emphysema more often than pneumothorax). The differential diagnosis has to rule out "pseudo asthma" due to cystic fibrosis, alveolitis, achalasia, and foreign body aspiration.
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PMID:[Radiologic findings and differential diagnosis of bronchial asthma in childhood]. 220 86

Thirty-three operations for subtotal esophagectomy with one-stage plastics with a gastric pedicle without thoracotomy were carried out between 1985 and 1988. The indications for the operation were as follows: carcinoma of the esophagus (17) and of cardioesophageal localization (7), cicatricial stricture of the esophagus (6), IV degree cardiospasm (2), unspecific esophageal ulcer (1). The esophagus was resected through a laparotomo-transdiaphragmatic-cervical access, the graft formed from the greater curvature of the stomach was passed in the posterior mediastinum with the establishment of a cervical esophagogastroanastomosis. Postoperative complications occurred in 29 patients: incompetence of the anastomosis (26), mediastinitis and pyothorax, (4), peritonitis (2), pneumonia (4). Six patients died. With the performance of intrapleural esophagogastroplasty the mortality rate fell from 25 to 18.2%. The authors claim that subtotal esophagectomy with posteromediastinal gastroplasty without thoracotomy is a less traumatic and safer operative intervention.
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PMID:[Subtotal esophagectomy with simultaneous retromediastinal plastic surgery using gastric pedicled flap without thoracotomy]. 235 73

Arthrogryposis multiplex congenita (AMC) is a rare congenital disorder characterized by multiple fixed-joint deformities of the upper and lower extremities. Those afflicted with the more common neurogenic form of AMC can have otolaryngologic manifestations. Here, a case of AMC with severe otolaryngologic manifestations is reported. The clinically relevant findings included an expressionless face, micrognathia, poor suck reflex, high arched palate, and an omega-shaped epiglottis, but otherwise normal larynx, trachea, and esophagus by endoscopic examination. Airway compromise, achalasia, aspiration pneumonitis, and poor nutrition were constant problems. Tracheostomy and gastrostomy were subsequently placed, and the patient's clinical course eventually improved. Early involvement of the otolaryngologist and placement of a tracheostomy and gastrostomy are recommended in those patients with severe otolaryngologic manifestations of AMC.
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PMID:Severe otolaryngologic manifestations of arthrogryposis multiplex congenita. 240 63

Long-term results are presented in 60 patients (4 to 50 years old) who underwent a diaphragmatic graft procedure for relief of cardiospasm (achalasia) from 1962 through 1987. The operative technique involves construction of a pedicle flap of diaphragm. The muscular defect on the lower segment of the esophagus and the transplanted diaphragmatic pedicle that is sutured to the defect must be the same size. Immediate operative results were good. Only one complication developed, a case of pneumonia that was cured. The patients were followed up from 11 months to 25 years. Two patients were lost to follow-up, 55 had excellent results, and three patients still had nausea and heartburn but were better than before the operation. This procedure has three advantages: (1) It prevents the development of fistulas and diverticula at the site of the esophageal muscular defect; (2) it effectively eliminates both restenosis resulting from scar tissue and reflux esophagitis; and (3) it allows the cardia to recover its normal function and the esophagus to return to normal size at the site of the operation.
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PMID:Treatment of esophageal achalasia (cardiospasm) with diaphragmatic graft. Twenty-five years' experience. 292 62

In some cases of achalasia, lungs may show characteristic granulomatous pneumonia, following non-symptomatic spill-over and aspiration of fat. The lesion is characterised by fat-containing vacuoles, necrosis, granulocytes, macrophages, some of them with lipid content, epithelioid and giant cells and fibrosis rich in lymphocytes. The histological pattern is pathognomonic and represents an entity. These alterations may be mistaken for tuberculosis and be distinguished from mineral-oil pneumonia (paraffinoma). Affected lung regions may be colonised by nontuberculous (atypical) mycobacteria.
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PMID:[The histomorphology of granulomatous lipid pneumonia due to dietary fat aspiration in achalasia of the esophagus (megaesophagus)]. 324 52

Results with the use of a diaphragmatic graft in the surgical relief of achalasia are reported for 44 patients. The operative technique involves construction of a pedicle flap of diaphragm the size of the muscular defect on the lower segment of the esophagus and suture of the transplanted diaphragmatic pedicle to the site of the esophageal muscular defect. Immediate operative results were good; there was only one complication, a case of pneumonia that was cured. Patients were followed from 3 months to 19 years. Two patients were lost to follow-up. Excellent results were obtained in 39 patients; 3 patients still had nausea and heartburn, but were better than before operation. This procedure has three advantages: (1) it prevents occurrence of fistula and diverticulum at the site of the esophageal muscular defect; (2) it effectively eliminates formation of restenosis due to scar and reflux esophagitis; and (3) it allows the cardia to recover its normal function and the esophagus to return to normal size at the site of operation.
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PMID:Treatment of esophageal achalasia (cardiospasm) with diaphragmatic graft: report of 44 patients. 640 96

A 67 year old male caucasian clerical worker with a background of long-standing gastro-oesophageal reflux-like dyspepsia and bronchiectasis presented to a tertiary hospital gastroenterology unit with a recent onset of dysphagia. An initial diagnosis of achalasia was made and within 1 year an established verrucous carcinoma of the upper oesophagus had developed. The tumour was inoperable due to tracheal invasion and therefore palliative treatment was given. The patient developed a tracheo-oesophageal fistula and died of pneumonia. Thus, verrucous squamous cell carcinoma of the oesophagus can occur with achalasia.
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PMID:Verrucous carcinoma of the oesophagus and achalasia. 843 56

Esophageal achalasia (EA) has been historically treated by esophageal dilatation or myotomy with or without fundoplication. Botulinum toxin (Botox-Allergan) use in pediatric EA has not been previously described. The authors' objective was to observe the efficacy of botulinum toxin injection into the lower esophageal sphincter (LES) for EA. An 11-year-old boy presented with a 9-month history of frequent pneumonia, productive cough, and a 1-year history of chest discomfort and odynophagia. Chest radiograph showed changes compatible with aspiration. Upper gastrointestinal (UGI) series showed typical narrowing of the LES, and 24-hour pH study showed no reflux. Esophageal manometry showed classic findings of achalasia. An upper gastrointestinal endoscopy was performed showing a huge volume of retained food. A direct four-quadrant injection was performed with a total of 100 U of botulinum toxin into the LES. UGI series showed improvement in esophageal emptying. Esophageal manometry showed impressive improvement in LES pressure (preinjection, 44.1 mm Hg to postinjection mean of 16.6 mm Hg), percent relaxation (preinjection, 30% to postinjection, 58.8%), and duration of relaxation (preinjection, 1.9 seconds to postinjection, 11 seconds). The patient has not had any further respiratory symptoms, chest pain, or odynophagia in 8 months of follow-up. Botulinum toxin injection is simple and effective for EA and merits its study in a prospective manner in the pediatric population.
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PMID:Botulinum toxin use in pediatric esophageal achalasia: a case report. 920 Jan

Esophageal or other swallowing disorders complicated by lipoid pneumonia are reported to be associated with pulmonary infections caused by rapidly growing mycobacteria. Herein we describe a 63-year-old woman with achalasia of the esophagus complicated by lung infection with Mycobacterium chelonae and a 47-year-old man in whom long-term ingestion of mineral oil was complicated by lipoid pneumonia and M. fortuitum lung infection. A MEDLINE search of English language publications from 1966 to 1997 revealed 18 cases of lung infections caused by rapidly growing mycobacteria in patients with esophageal disorders. Of these 18 patients and our 2 patients, 11 were men and 9 were women (mean age, 50 years). Achalasia was present in 11 patients, and 6 had lipoid pneumonia without evidence of esophageal disorders. Three patients had lipoid pneumonia caused by lipoid ingestion in the setting of achalasia or another swallowing disorder. In 14 patients, lung infection was caused by M. fortuitum; in 5, M. chelonae; and in 1, a non-M. fortuitum rapidly growing mycobacterial infection. The most common clinical feature was fever, and the most common roentgenologic abnormality was the presence of unilateral or bilateral and patchy or dense infiltrates. The sputum was the most common source of isolation of rapidly growing mycobacteria. Achalasia and lipoid pneumonia are important risk factors for the development of lung infections caused by rapidly growing mycobacteria. Treatment of the esophageal disease might prevent occurrence of and facilitate recovery from these infections.
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PMID:Rapidly growing mycobacterial lung infection in association with esophageal disorders. 1040 12

A 9-year-old girl presents for continuing weight loss of 10 kg over the course of 1 year. Medical history showed three episodes of pneumonia requiring hospital admission in the 6 months before presentation and 4 months of weekly psychotherapy for anorexia nervosa. A thorough history of eating behavior and a review of systems revealed not only typical aspects of prepubertal anorexia nervosa but also vomiting at night while asleep, difficulty drinking liquids, epigastric pain, and a frequent experience of "a lump in the throat"; these symptoms were not suggestive of a diagnosis of anorexia nervosa but rather of esophageal achalasia. The patient was transferred to the Department of Pediatrics, and a diagnosis of esophageal achalasia was made by chest x-ray and barium swallow. After dilatation and botulinum toxin application, the patient regained weight easily and was discharged in stable condition. In this case, esophageal achalasia mimicked prepubertal anorexia nervosa.
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PMID:Achalasia mimicking prepubertal anorexia nervosa. 1265 33


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