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

Two rare cases with surgically treated vascular ring associated with complex congenital cardiac anomaly are reported. Each case showed vascular ring due to right aortic arch, right descending aorta, aberrant left subclavian artery and left ductus arteriosus. Case 1 was a 2-year-old boy associated with ligamentum arteriosum and total anomalous pulmonary venous connection (TAPVC), and presented dyspnea and dysphagia. Case 2 was a 3-year-old girl associated with patent ductus arteriosus (PDA) and tetralogy of Fallot (TOF). In case 1, the two-staged operation was performed because of the necessity of mechanical ventilation and of recurrent respiratory infection. Division of the ligamentum, dissection of bronchus and esophagus was performed at the first operation through left thoracotomy, and the intracardiac repair of TAPVC was done three months later. In case 2, PDA division and the intracardiac repair of TOF was done simultaneously through median sternotomy because of the mild symptom of vascular ring and technical feasibility. Each case was successfully treated and became completely asymptomatic.
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PMID:[Surgery of vascular rings associated with complex intracardiac anomaly]. 156 3

Retrospective study of ten infants and young children in whom an unsuspected foreign body was found in the esophagus revealed that all had had wheezing of difficulty swallowing solid food. In four, stridor or wheezing had been ascribed to respiratory infection. All foreign bodies were in the upper esophagus. Their presence can cause prolonged, severe morbidity. They can be identified by radiography of the neck and chest, occasionally supplemented by barium studies.
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PMID:Unsuspected esophageal foreign bodies. 721 59

A retrospective study covering December 1978 to December 1989 at Booth Hall Children's Hospital, Manchester, revealed that 12 colon interposition were performed in 11 children to reconstruct their oesophagus. All were for wide-gap oesophageal atresia with or without fistula. One patient underwent colon interposition twice due to ischaemia of the initial transplant. Waterston's transthoracic procedure was used mostly with a modification by Freeman performed in one patient. Main early postoperative complications included proximal anastomosis leak (45.5%) and respiratory infection (36.4%). Late complications were proximal anastomotic stricture (22.2%); gastrointestinal problems (66.7%); and recurrent respiratory infection (11.1%). Although colon redundancy radiologically was noted in two patients, they were well with no dysphagia. Mortality was 18.2%, the two patients dying suddenly two weeks after colon transplant from pulmonary complications. At follow-up (mean: 6.2 years) growth rate had improved slightly, and 11.1% weighed above the 10th percentile. However, 77.8% could swallow satisfactorily within one year of surgery.
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PMID:Colon interposition for wide gap oesophageal atresia. 803 68

We describe two patients of right aortic arch associated with aberrant retroesophageal innominate artery. One case was associated with tetralogy of Fallot and a tortuous left common carotid artery originating from the retroesophageal innominate artery. Tetralogy of Fallot associated with this kind of aortic arch anomaly has never before been reported. The other case was associated with a perimembranous ventricular septal defect. Symptoms such as dyspnea, dysphagia and recurrent respiratory infection due to the compression by the vascular anomaly were not noted in these two cases. The morphological findings are indicative of a double aortic arch with an interruption of the left arch proximal to the common carotid artery.
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PMID:Right aortic arch with aberrant retroesophageal innominate artery: a report of 2 cases and review of the literature. 846 5

Leigh disease is a subacute neurodegenerative disorder characterized by symmetric necrotic lesions in the basal ganglia, cerebellum, thalamus, brain stem, and optical nerves and caused by altered oxidative phosphorylation. We describe the clinical, biochemical, neuroimaging, and molecular studies of a 19-year-old boy with early-onset Leigh disease manifesting as severe extrapyramidal disorder with generalized dystonia and choreoathetosis. He was born of healthy parents after an uneventful pregnancy and delivery. At the age of 2 1/2 years, after a minor respiratory infection, he developed unstable, broad-based gait and tremor of the hands. These symptoms persisted for the next several years, when ataxia became more prominent. Difficulty in swallowing, dysarthria, trunk dystonia, and marked dyskinesia of the arms and hands gradually developed. Nystagmus, transient ptosis, and strabismus also appeared. Abnormal laboratory findings included elevated plasma and cerebrospinal fluid lactate and pyruvate, with an abnormal lactate/pyruvate ratio. Cranial computed tomography and magnetic resonance imaging demonstrated signs of cerebellar atrophy, bilateral and symmetric hypodensities in the lentiform nucleus and thalamus, and transient hyperintensities of cerebral peduncles in T2-weighted sequences suggestive of Leigh disease. Muscle biopsy revealed isolated fiber atrophy, necrotic fibers undergoing phagocytosis, and no ragged-red fibers. The measured catalytic activity of cytochrome c oxidase in skeletal muscle homogenates demonstrated a partial cytochrome c oxidase deficiency No abnormalities in the mitochondrial genome and in the SURF-1 gene were found. The boy is currently receiving levodopa therapy, creatine monohydrate, and a high dosage of thiamine and lipoic acid, his condition is stabilized, and extrapyramidal symptoms are less pronounced.
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PMID:Cytochrome c oxidase partial deficiency-associated Leigh disease presenting as an extrapyramidal syndrome. 1151 Sep 39

GENERAL PRINCIPLES: The management of dysphagia requires multi-disciplinary interventions, implying various procedures, the choice of which depends on the results of the global prior assessment of the patient. General measures for oral hygiene, dental care and the organization of conditions in which meals are taken are essential, particularly for dependent patients. A change in food texture or in viscosity of liquids is a strategy commonly used. However, such changes must depend on objective bases and not unnecessarily penalize patients or expose them to further risks of complications. COMPENSATION AND RE-EDUCATION TECHNIQUES: Various strategies can help to counteract deficient deglutition mechanisms. Among these, adopting a particular position while swallowing is helpful in many dysphagic patients, but may be limited by severe cervical stiffness. The swallow reflex can be enhanced by sensorial stimulation techniques, although they are only effective short-term. Execution of voluntary maneuvers improves efficacy and safety when swallowing, but learning these maneuvers can be difficult or even impossible, even when accepted by the patients and compliance is inconsistent. Re-education techniques are intended to lastingly improve swallowing, but it is difficult to obtain the active participation of many elderly patients. Furthermore, these commonly used techniques lack seriousness and require validation. MISCELLANEOUS PROCEDURES: The efficacy of pharmacological intervention is not clear, other than in certain particular etiological contexts and, as with procedures enhancing the stimulating properties of food and liquids, will obviously be developed in the future. Surgery, or related techniques, provides help in certain specific conditions, notably when an anatomical disorder is responsible for the disorder. Gastrostomy is still controversial, not only with regard to its optimal practical use and its capacity to decrease respiratory infection risks and improve nutritional prognosis, but also with regard to its impact on patients' survival and quality of life.
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PMID:[Deglutition disorders in the elderly. Management]. 1175 44

Dysphagia in stroke is linked with increased risk of pneumonia, increased length of stay and poorer outcomes. This study followed a cohort of 88 acute ischaemic stroke patients admitted to hospitals in Perth, Western Australia, over 30 days. There were 8/88 deaths (9%). Infections were treated in 25/80 survivors (31%). Presence and severity of dysphagia were measured at 2 and 7 days post-stroke. Respiratory tract infections occurred at significantly higher rates for dysphagics (p<0.05). At 2 days post-stroke, the odds ratio (OR) of chest infection for dysphagics was 1.45 (95% CI=1.07-1.98). Survivors who were "nil by mouth" 2 days post-stroke were significantly more likely to develop pneumonia (p=0.01). At 7 days post-stroke, dysphagics were again more likely to develop pneumonia (p=0.014) with OR=1.77 (95% CI=1.26-2.49). The total anterior circulation infarcts demonstrated more severe and prolonged dysphagia than other stroke subtypes.
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PMID:Dysphagia in acute ischaemic stroke: severity, recovery and relationship to stroke subtype. 1743 10

Tracheal diverticulum is a rarely encountered entity which is frequently an incidental finding in the postmortem examination, reported in 1% of patients in an autopsy series. Most cases are asymptomatic, but when symptoms are present they usually have airway symptoms with cough or recurrent respiratory infection. We herein report a case of a tracheal diverticulum, which had cervical dysphagia and sensation of friction.
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PMID:Tracheal diverticulum: a rare cause of dysphagia. 1870 25

Tracheal diverticula are rarely encountered, and only a handful of cases have been described in the literature. In most of these cases the patient was asymptomatic. When symptoms have occurred, they usually had an airway component such as cough, dyspnea, and recurrent respiratory infection. Imaging techniques-particularly computed tomography-are beneficial for diagnosing a tracheal diverticulum because bronchoscopy can sometimes miss the point of communication with the trachea. We report the case of a 62-year-old woman with a tracheal diverticulum that manifested as an intermittent, painful right neck mass with associated cough, dysphonia, dysphagia/odynophagia, and an occasional strangulation/choking sensation.
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PMID:Intermittently symptomatic tracheal diverticulum: a case of a rare clinical phenomenon. 2193 86

The authors present the clinical case of an 87-year-old Caucasian male admitted to the emergency room with hematemesis. He had a history of intermittent dysphagia during the previous month. Endoscopic evaluation revealed an eccentric, soft esophageal lesion located 25-35 cm from the incisors, which appeared as a protrusion of the esophagus wall, with active bleeding. Biopsies were acquired. Tissue evaluation was compatible with a melanoma. After excluding other sites of primary neoplasm, the definitive diagnosis of Primary Malignant Melanoma of the Esophagus (PMME) was made. The patient developed a hospital-acquired respiratory infection and died before tumor-directed treatment could begin. Primary malignant melanoma represents only 0.1% to 0.2% of all esophageal malignant tumors. Risk factors for PMME are not defined. A higher incidence of PMME has been described in Japan. Dysphagia, predominantly for solids, is the most frequent symptom at presentation. Retrosternal or epigastric discomfort or pain, melena or hematemesis have also been described. The characteristic endoscopic finding of PMME is as a polypoid lesion, with variable size, usually pigmented. The neoplasm occurs in the lower two-thirds of the esophagus in 86% of cases. PMME metastasizes via hematogenic and lymphatic pathways. At diagnosis, 50% of the patients present with distant metastases to the liver, the mediastinum, the lungs and the brain. When possible, surgery (curative or palliative), is the preferential method of treatment. There are some reports in the literature where chemotherapy, chemohormonotherapy, radiotherapy and immunotherapy, with or without surgery, were used with variable efficacy. The prognosis is poor; the mean survival after surgery is less than 15 mo.
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PMID:Primary malignant melanoma of the esophagus: a case report. 2218 Jul 18


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