Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Esophageal myotomy is the standard primary therapy for achalasia. However, reports on long-term results of myotomy have suggested a deterioration of outcome over time with many patients presenting with end stage disease several years after esophagomyotomy. Eight patients who had previously undergone esophagomyotomy for achalasia presented with recurrent or worsening symptoms, and after preoperative evaluation, were treated by esophagectomy via laparotomy and right thoracotomy. The mean age at the time of myotomy was 52 years (range 18 to 62 years), and the mean time until esophagectomy was 12.5 years (range 2 to 18 years) after the initial myotomy. The median time until esophagectomy was performed after myotomy was 14 years. All patients in this series gained weight (mean, 23 pounds; range, 9 to 42 lbs) following esophagectomy, and none of the patients complained of dysphagia at follow-up or developed stricture. There were no major complications (including anastomotic leak) or deaths in this series. Five of the patients have been followed a mean of six years and remain well. Esophagectomy is a safe and appropriate treatment option in the setting of recurrent and end stage achalasia.
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PMID:Esophagectomy for end stage achalasia. 1762 58

Esophago-respiratory fistulas, evolving as a result of esophageal tumors, are serious and lethal complications on account of the constant respiratory contamination and the inability to swallow. They can develop either as the complication of the end stage disease or sometimes even in the first stage of the malignancy. The objective was to reveal the characteristics of the disease. In a prospective single-center study in the period between 1984 and 2004, 243 fistulas were diagnosed. Their data were analyzed using multivariate analysis. The mean age of patients with fistula was 56.9 years, the male-to-female ratio was 4.3:1. The average time of the complaints was 5.2 months, while the time of manifestation of the fistula was 7.5 months. Dysphagia was diagnosed in 97.5% of the patients, fever in 36.9%, and cachexia in 59.5%, respectively. The average loss of weight was 10.4 kg and the average size of the tumor was 7.7 cm. Endoscopic intubation was performed in 176 cases. The average survival was 3.4 months. Patients with fistula were divided into two groups, where the characteristics of the disease were significantly different. Only in 66.3% was the fistula a late complication. In the other 33.7% of the cases the fistula was diagnosed in younger patients at the early stage of the disease, with a more aggressive, less differentiated histology. In these patients the weight loss, the grade of dysphagia and the size of the tumor were smaller, the possibilities of treatment were fewer, and survival were shorter.
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PMID:[Pathological characteristics of esophago-respiratory fistulas of esophageal tumor origin]. 1864 Aug 92

Although SPG11 is the most common complicated hereditary spastic paraplegia, our knowledge of the long-term prognosis and life expectancy is limited. We therefore studied the disease course of all patients with a proven SPG11 mutation as tested in our laboratory, the single Dutch laboratory providing SPG11 mutation analysis, between 1 January 2009 and 1 January 2011. We identified nine different SPG11 mutations, four of which are novel, in nine index patients. Eighteen SPG11 patients from these nine families were studied by means of a retrospective chart analysis and additional interview/examination. Ages at onset were between 4 months and 14 years; 39% started with learning difficulties rather than gait impairment. Brain magnetic resonance imaging showed a thin corpus callosum and typical periventricular white matter changes in the frontal horn region (known as the 'ears-of the lynx'-sign) in all. Most patients became wheelchair bound after a disease duration of 1 to 2 decades. End-stage disease consisted of loss of spontaneous speech, severe dysphagia, spastic tetraplegia with peripheral nerve involvement and contractures. Several patients died of complications between ages 30 and 48 years, 3-4 decades after onset of gait impairment. Other relevant features during the disease were urinary and fecal incontinence, obesity and psychosis. Our study of 18 Dutch SPG11-patients shows the potential serious long-term consequences of SPG11 including a possibly restricted life span.
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PMID:Rapidly deteriorating course in Dutch hereditary spastic paraplegia type 11 patients. 2344 22