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 Dor-Gavriliu procedure was utilized in six children with achalasia of the cardia from 1983 to 1991. Four boys and two girls (mean age, 10.1 years; range, 3 months to 16 years) presented with symptoms of weight loss (83%), emesis (83%), dysphagia (67%), recurrent respiratory infections (67%), and nocturnal regurgitation (33%). The diagnosis of achalasia was established by barium swallow in all patients; esophageal manometry was used in four patients to confirm the diagnosis. Follow-up ranged from 10 months to 8 years (mean, 3.5 years). Four neurologically normal patients had excellent results with complete resolution of their preoperative symptoms. Two neurologically impaired children, both afflicted with Down's syndrome, had less than excellent results. One moderately impaired child had a good result (required three postoperative bougie dilations over 8 years without demonstration of gastroesophageal reflux); the second, more severely impaired child, had only a fair result (persistent failure to thrive with the development of grade II gastroesophageal reflux). The Dor-Gavriliu procedure uses a transabdominal, anterior esophageal myotomy with incorporation of an effective, nonobstructing, antireflux mechanism that should prevent myotomy reapposition.
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PMID:Surgery for achalasia of the cardia in children: the Dor-Gavriliu procedure. 147 2

In a patient with progressive dysphagia, postprandial vomiting, and a history of Alport syndrome, barium and manometric studies had been interpreted as consistent with achalasia, but a subsequent computed tomographic (CT) scan of the thorax was suggestive of a lower esophageal intramural mass. Multiple leiomyomas of the esophagus were later proved at thoracotomy. Differences between adult and pediatric leiomyomas and the association of leiomyomas with Alport syndrome are discussed.
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PMID:Diffuse esophageal leiomyomatosis in a patient with Alport syndrome: CT demonstration. 200 73

A 4 month old girl with oesophageal achalasia was successfully treated with a single pneumatic dilatation. A subsequent barium swallow showed almost normal oesophageal caliber. During the following eighteen months, vomiting and feeding problems did not recur.
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PMID:Infantile achalasia treated by pneumatic dilatation. 213 54

The case of a young women with dysphagia, regurgitation, and weight loss, who was diagnosed as having anorexia nervosa but in whom reevaluation showed that achalasia was causing the symptoms, is presented together with related observations. Misinterpretation of esophageal symptoms may occur not only as a consequence of inadequate history taking and of being biased by a patient's emaciation, age, and gender, which leads to view certain aspects of the patient's history and behavior as suggesting a pathologic attitude towards eating and body weight, but also as a consequence of a misinterpretation of the symptoms as indicative of an eating disorder by the patients themselves. In some cases a disordered attitude toward eating and body weight may develop together or coexist with achalasia. The clinical evaluation of patients with symptoms suggestive of anorexia nervosa but also of bulimia nervosa should include the taking of a thorough history regarding swallowing and vomiting in order to recognize a possible esophageal motor disorder.
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PMID:Symptoms of achalasia in young women mistaken as indicating primary anorexia nervosa. 227 21

When esophageal disruption occurs in the presence of preexisting esophageal disease or is associated with sepsis or fluid and electrolyte imbalance, aggressive and definitive therapy often provides the only chance for patient salvage. Twenty-four adults (average age, 59 years) with intrathoracic esophageal perforations underwent esophagectomy: 15, transhiatal esophagectomy without thoracotomy; and 9, transthoracic esophagectomy. Restoration of alimentary continuity with an immediate cervical esophagogastric anastomosis was carried out in 13 patients. Eleven underwent a cervical or anterior thoracic esophagostomy, and 10 of them had a subsequent colonic (7) or gastric (3) interposition from 4 to 32 weeks (average time, 8.6 weeks) later. The perforations were due to esophageal instrumentation (9 patients), acute caustic ingestion (2), emesis (2), intrathoracic esophagogastric anastomotic disruption (2), and other causes (9). Preexisting esophageal disease in 20 patients included chronic strictures (10 patients), reflux esophagitis (3), esophageal cancer (3), achalasia (2), diffuse spasm (2), and monilial esophagitis (1 patient). Ten patients were operated on within 12 hours after the injury; 3, within 12 to 24 hours; and 11, within three to 45 days (average interval, 6.6 days). There were three hospital deaths (13%). Nineteen of the 21 survivors were able to swallow comfortably until the time of death or latest follow-up. Aggressive diagnosis and aggressive treatment of life-threatening esophageal perforations are advocated. Conservative procedures (repair, diversion, or drainage) for a perforation with preexisting esophageal disease often inflict more morbidity than esophageal resection, which eliminates the perforation, the source of sepsis, and the underlying esophageal disease. The decision to restore alimentary continuity in a single stage must be individualized.
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PMID:Esophagectomy for esophageal disruption. 229 75

Previous studies showed that symptoms of oesophageal motor disorders can be misinterpreted as indicating anorexia nervosa and that in primary anorexia nervosa gastric motility is frequently impaired. We investigated in 32 women with bulimia nervosa whether symptoms of oesophageal motor disorders could be obscured by or be mistaken as forming part of bulimic behaviour, and whether impaired gastric motility was frequent as well. Oesophageal motility was normal in 18 of 26 patients studied, another four had incomplete lower oesophageal sphincter relaxation. Two patients had vigorous achalasia and each one achalasia and diffuse oesophageal spasm, all of whom experienced two types of vomiting: one self-induced and one involuntary, in which the vomit was non-acidic and tasted as the preceding meal. Gastric emptying of a semisolid meal was studied in all patients except of the eight with oesophageal motor abnormalities. Emptying was significantly slower than in healthy controls and grossly delayed in nine of 24 patients. Antral contraction amplitudes were lower and increased less postcibally than in controls. In conclusion (i) bulimic behaviour can obscure symptoms of oesophageal motor disorders and (ii) gastric emptying is frequently delayed in bulimia nervosa.
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PMID:Oesophageal and gastric motor activity in patients with bulimia nervosa. 232 85

Leiomyoma of the esophagus is not uncommon in the adult population but is rarely seen in children; only 20 cases have been reported in the pediatric population. In this paper we describe two cases of esophageal leiomyoma in female patients aged 6 and 13 years and review previous reports. Several differences were noted between the pediatric and adult population. The mean age in children is 14 years (range, 4 to 20 years). Leiomyoma appears 1.71 times more often in females than in males. Localized lesions are found in only 9%, whereas the diffuse form predominates in 91%. The entire esophagus may be involved 35% of the time, and encroachment on the cardia or upper stomach occurs in 70%. Leiomyomas associated with familial syndromes (familial leiomyoma and Alport's syndrome) occur in 22% of the cases. Major symptoms include dysphagia (86%), dyspnea (36%), vomiting (27%), retrosternal pain (27%), and coughing (22%). The initial diagnosis following contrast studies is most often achalasia. The diagnosis of leiomyoma is made only with subsequent endoscopy. Enucleation was performed in only 11% of the cases; surgical resection (including part of the stomach) was necessary in 78% with a 21% postoperative mortality. Esophageal leiomyoma should be considered in the differential diagnosis of mediastinal masses and esophageal obstruction. Accurate preoperative diagnosis is desirable in order to plan proper surgical treatment.
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PMID:Esophageal leiomyoma in children: two case reports and review of the literature. 268 58

Thirty-one cases of esophageal achalasia were admitted to Chang Gung Memorial Hospital between 1981 and 1986. Eighteen male patients and 13 female patients, aged from 12 to 84 years old with an average of 39 years old, were included in this series. Their chief complaints were dysphagia (83.9%), postprandial vomiting (12.9%), and food regurgitation (3.2%). The symptoms are present for an average of 2.8 years (mostly between 0.5 and 2 years) before the diagnosis is made. The clinical signs and symptoms included dysphagia, postprandial vomiting, loss of body weight, food regurgitation, abdominal fullness, cough, chest pain, belching, and choking. The tentative diagnoses at admission were achalasia, esophageal stricture R/O achalasia, achalasia R/O esophageal cancer, and esophageal cancer. Laboratory examinations showed 90.3% with absence of the gastric air shadow in chest P-A view X-ray film. Typical birds-beat deformity in barium-meal esophagogram was seen in 100%, and during esophagoscopic examination, 25% (6/24) were without abnormal findings, 66.7% (16/24) had liquid and food stasis, 8.3% (2/24) had esophagitis. Manometry of esophagus was performed in 5 cases, all had positive abnormal patterns detected, such as aperistalsis of esophageal body and incomplete relaxation of lower esophageal sphincter, but only 60% showed hypertensive lower esophageal sphincter. In these 31 cases, 3 cases refused any treatment, 9 cases received medical therapy including drug therapy(9) and pneumatic esophageal dilatation(8), and 19 cases received surgical operations. Better swallowing improvement was obtained in the surgically treated group than in the medically treated patients during follow up period.
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PMID:[A clinical analysis of esophageal achalasia]. 277 66

A 7-year-old boy presented with a history of postprandial vomiting, failure to thrive, hematuria, proteinuria and decreased renal function. Electron microscopy of a renal biopsy specimen demonstrated the typical glomerular basement membrane changes associated with Alport's syndrome. Audiometry revealed a moderate bilateral high-tone sensorineural hearing loss. Bilateral anterior lenticonus and a unilateral cataract were also diagnosed. Achalasia diagnosed radiologically and confirmed by biopsy was corrected by surgery. Evaluations of the parents and three siblings were negative. The patient subsequently developed end-stage renal failure. This case report and a review of the literature suggest that achalasia may be part of Alport's syndrome in some patients.
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PMID:Alport's syndrome and achalasia. 315 32

A 12-year-old girl presented with cough, emesis, and weight loss of 18-month duration. She was diagnosed as having achalasia of the esophagogastric junction. Flow-volume loops done because of the cough revealed a configuration consistent with variable intrathoracic tracheal obstruction. A barium swallow revealed the cause of obstruction to be massive dilatation of the esophagus. Balloon dilatation of the esophageal sphincter resolved all symptoms and normalized the flow-volume loops. This case illustrates that some pulmonary symptoms in achalasia can be due to direct tracheal obstruction in addition to the usual aspiration of esophageal contents.
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PMID:Tracheal obstruction secondary to esophageal achalasia. 318 82


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