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

A case with tetanus presenting with progressive dysphagia due to lower esophageal dysfunction is described. A barium swallow at the onset of symptoms showed esophageal dilatation with a smooth tapering at the lower end suggestive of achalasia cardia. The patient recovered from tetanus over the ensuing three weeks; repeat barium swallow at this time was normal, suggesting that esophageal dysfunction was a manifestation of tetanus.
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PMID:Transient achalasia of esophagus in tetanus. 150 52

Between 1980 and 1989, 15 of 46 patients (11 children, 4 adults) who underwent suboccipital craniectomy and cervical laminectomy for symptomatic Chiari malformations presented with manifestations of neurogenic dysphagia. Each of these patients had normal swallowing function before the development of dysphagic symptoms. Dysphagia was progressive in all 15 and, in most cases, preceded the onset of other severe brain stem signs. The rate of symptom progression varied depending on the age of the patient. Whereas the six infants (all Chiari II) deteriorated rapidly after the onset of initial symptoms, the five older children (two Chiari I, three Chiari II) and four adults (all Chiari I) showed a more gradual deterioration. In 11 patients with severe dysphagia, barium video esophagograms, pharyngoesophageal motility studies, continuous esophageal pH monitoring, and appropriate scintigraphic studies were useful in defining the scope of the swallowing impairment and determining whether perioperative nasogastric or gastrostomy feedings, gastric fundoplication, and/or tracheostomy were needed to maintain adequate nutrition and avoid aspiration. These patients all had widespread dysfunction of the swallowing mechanism, with a combination of diffuse pharyngoesophageal dysmotility, cricopharyngeal achalasia, nasal regurgitation, tracheal aspiration, and gastroesophageal reflux. The pathophysiology of these swallowing impairments and their relation to the hindbrain malformation is discussed. Postoperative outcome with regard to swallowing function correlated with the severity of preoperative symptoms. The four patients with mild dysphagia showed rapid improvement in swallowing function after surgery. Seven patients with more severe impairment but without other signs of severe brain stem compromise, such as central apnea or complete bilateral vocal cord paralysis, also improved, albeit more slowly. In contrast, the outcome in the four patients who developed other signs of severe brain stem dysfunction before surgery was poor. Early recognition of neurogenic dysphagia and expeditious intervention are therefore crucial in ensuring a favorable neurological outcome.
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PMID:Neurogenic dysphagia resulting from Chiari malformations. 158 83

Achalasia of the cardia is an uncommon condition with an incidence of 0.1 cases per year per 100,000 population under 14 years. A review of experience at Booth Hall Children's Hospital over the past 10 years has revealed six cases of achalasia in children. This includes two cases of the rare syndrome of achalasia, alacrima and ACTH insensitivity (also known as Triple A syndrome) and its variants. There are 23 previously reported cases of Triple A syndrome and a further eight cases of its variants reported in the world literature. One-third of these cases (10 out of 31) have associated neurological abnormalities. The clinical and radiological features of achalasia are reviewed. The importance of barium studies in making the diagnosis is emphasized. However, early cases may show only spasm or incoordination, and manometry is required to confirm the diagnosis in these equivocal cases. In this series only three of the six barium studies were diagnostic and the remainder were diagnosed by manometry; all cases were subsequently confirmed by histology. Delay in diagnosis may result in severe lung damage due to repeated aspiration; this occurred in one of the six cases and was the cause of the child's death.
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PMID:Achalasia of the cardia in childhood and the syndrome of achalasia alacrima and ACTH insensitivity. 160 7

A 70-year-old woman with no previous gastroesophageal surgery gave a 6-month history of dysphagia. Barium studies suggested a diagnosis of achalasia. Esophageal manometry showed absence of peristalsis and a high lower esophageal sphincter pressure. Endoscopy showed a dilated esophagus with food residue, and Barrett's esophagus was present. The association of Barrett's esophagus and achalasia must be rare.
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PMID:Barrett's esophagus and achalasia. A case report. 174 94

Radiographic evaluation of the lower esophagus was done immediately after pneumatic dilatation using the Rigiflex dilator in 34 patients (24 men, 10 women; mean age, 55 years) with achalasia. The dilator was positioned across the esophagogastic junction using fluoroscopy and the balloon was inflated for 1 min. The esophagus was intubated and injected sequentially with water-soluble and barium contrast materials. Radiographic analysis included changes in the appearance of the caliber and contour of the esophagogastric junction, rate of esophageal emptying, and presence of complications. In 23 patients with predilatation esophagrams, the mean esophagogastric junction caliber increased from 4.7-7.6 mm following dilatation. The postdilatation esophagrams in 33 patients showed a smooth contour in 22 (67%) and immediate esophageal emptying in 26 (79%). Esophageal perforation occurred in one (3%) patient and intramural hematoma in one (3%). Clinical follow-up (mean, 7 months) was available in 29 patients and 23 (79%) had symptomatic improvement. Five of the six patients who did not improve clinically all had previous Heller myotomy, pneumatic dilatation, or both.
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PMID:Radiographic evaluation of achalasia immediately after pneumatic dilatation with the Rigiflex dilator. 193 64

Calcium channel blockers have been previously shown to decrease lower esophageal sphincter (LES) pressure and improve symptoms in achalasia. We performed a placebo-controlled, double-blind, crossover study to assess the effects of oral nifedipine and verapamil on LES pressure, amplitude of esophageal body contraction, and clinical symptomatology in eight patients with symptomatic achalasia diagnosed by endoscopy, barium swallow, and manometry. Patients were randomized to receive up to 20 mg nifedipine, 160 mg verapamil, or placebo and underwent esophageal manometry before (baseline) and after four weeks on each drug. Diary cards were kept to record and grade symptoms and drug plasma level determinations were correlated with manometric and clinical findings. Both nifedipine and verapamil caused a statistically significant decrease in mean LES pressure, but only nifedipine caused a significant decrease in the amplitude of contractions of the smooth muscle portion of the esophagus. No statistically significant differences in the overall clinical symptomatology were noted with any of the drugs, although some individual improvements in dysphagia and chest pain were noted. We conclude that, despite the reduction in LES pressure and contraction amplitude of the distal esophageal body, oral nifedipine and verapamil do not significantly alter the clinical symptomatology of patients with achalasia.
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PMID:Medical treatment of esophageal achalasia. Double-blind crossover study with oral nifedipine, verapamil, and placebo. 199 57

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

Patients with a prominent cricopharyngeal bar visible on radiography are generally considered to have spasm of the cricopharyngeus, which is the major muscle component of the upper esophageal sphincter. This condition has been termed "cricopharyngeal achalasia." The aim of this study was to determine the pathogenesis of cricopharyngeal bars. Concurrent videofluoroscopic and manometric examinations of the pharynx and upper esophageal sphincter were performed in a cohort of six patients with prominent cricopharyngeal bars and in eight control volunteers. In each subject, swallows of 2-30-mL barium boluses were recorded. The patients with cricopharyngeal bars showed (a) normal peristaltic contraction in the pharynx, (b) normal axial upper esophageal sphincter pressure and relaxation, (c) normal flow rate across the upper esophageal sphincter, and (d) normal duration of upper esophageal sphincter opening for different bolus volumes. The major abnormalities in the patients with cricopharyngeal bars were (a) reduced maximal dimensions of the upper esophageal sphincter during the transsphincteric flow of barium and (b) increased intrabolus pressure upstream to the upper esophageal sphincter. Thus, the increase in intrabolus pressure preserved normal transsphincteric flow rates even though the upper esophageal sphincter did not open normally. Overall, the constellation of findings in the patients studied suggests that the underlying pathogenesis of their cricopharyngeal bar was reduced muscle compliance wherein the relaxed cricopharyngeus did not distend normally during swallowing.
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PMID:Biomechanics of cricopharyngeal bars. 221 Feb 35


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