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)

We report the anesthetic management of esophagectomy for a patient with Alport-leiomyomatosis syndrome. A 23-year-old woman complained of dysphagia and severe chest pain. Her chest X-ray, computed tomography (CT), and magnetic resonance imaging (MRI) showed an enlarged esophagus, in contact with the trachea, heart, aorta, and large vessels. She frequently experienced severe asthma attacks. Because various risks in both respiration and circulation, especially in anesthesia induction, were of concern, her right femoral vessels were exposed, for the emergency use of percutaneous cardiopulmonary support (PCPS), prior to anesthesia induction. Anesthesia was induced and maintained with propofol, fentanyl, and vecuronium. Esophagectomy was performed uneventfully and no severe events were seen in anesthesia management. Alportleiomyomatosis syndrome is a very rare disease. When we are involved in the anesthetic management of a patient with this disease, evaluation of the influence of the enlarged esophagus on both respiration and circulation, and careful preparation for emergence, are very important.
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PMID:Anesthetic management of a patient with Alport-leiomyomatosis syndrome. 1968 35

Eagle syndrome is considered a rare disease. It is an aggregation of symptoms that includes neck, throat, and hemifacial pain; sensation of foreign body in the throat; dysphagia; otalgia; and change in voice as a direct result of an elongated styloid process or calcified stylohyoid ligament.The clinical diagnosis is generally difficult and must be confirmed by radiologic imaging. Our patient was a 37-year-old woman who had a 3 years' history of dysphagia and foreign body sensation. Computed tomographic scan showed a long left styloid process and calcification in the upper part of the stylohyoid ligament suggestive of Eagle syndrome. The patient underwent surgical removal of the elongated styloid process through an intraoral approach. The patient became symptom-free at 9 months after the surgery.
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PMID:Complete recovery after an intraoral approach for Eagle syndrome. 2009 99

Laryngeal tuberculosis is a rare disease. It is almost always associated with pulmonary tuberculosis. It occurs generally in adults without BCG vaccination or in cases of the acquired immune deficiency syndrome. On laryngoscopy and imaging, it often simulates laryngeal carcinoma, and confirmation is always histological. We report the case of a 36-year-old man who presented to our hospital with dysphonia and dysphagia. Laryngoscopy revealed a lesion of the left vocal cord and the ventricular strip. CT scan found focal, regular thickening of the left vocal cord, associated with irregular thickening of the posterior laryngeal wall. A biopsy confirmed the diagnosis of tuberculosis.
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PMID:Primary laryngeal tuberculosis mimicking laryngeal carcinoma: CT scan features. 2035 85

Mixed connective tissue disease (MCTD) is a rare disease that includes clinical and laboratorial manifestations of systemic lupus erythematosus, scleroderma and polymyositis that is associated with high titers of anti-U1RNP antibodies. In general, muscle involvement is subclinical, usually appearing as an increase in muscle enzyme levels that tends to be a characteristic of the initial phases of the disease. Severe clinical muscle weakness is not observed in this disease. The objective of this study is to report a rare case of a patient who presented a severe onset of myositis characterized by dysphagia, an increase in myopathy and a weakening of the cervical musculature. While there was no response to the administration of an initial dose of corticosteroids, improvement was observed after increasing the dose of corticosteroids, in addition to the initiation of pulse therapy with methylprednisolone accompanied by methotrexate treatment. The authors emphasize that there is only one previously reported case regarding a child with MCTD and severe clinical myopathy on electromyography and muscle biopsy, and they report in this article one adult female patient who presented severe myositis and was refractive to corticotherapy.
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PMID:Severe and refractory myositis in mixed connective tissue disease: a description of a rare case. 2066 43

Amyotrophic lateral sclerosis is a rare disease. It is a fatal neurodegenerative disease characterized by progressive muscular paralysis reflecting degeneration of motor neurons which leads to muscle weakness and muscle wasting. Respiratory failure limits survival to 2-5 years after disease onset. Several clinical manifestations including dysphagia can result in reductions in both the quality of life and life expectancy. Dysphagia occurs at onset in about one third of case, although generally it occurs in later stage of the disease. Evaluation of dysphagia includes video-fluoroscopic swallow study, radiological esophagogram, flexible endoscopic examination, ultrasound examination, conventional manometry and electromyography. We report a case of amyotrophic lateral sclerosis in a 54-year-old man presenting oropharyngeal dysphagia which was diagnosed by high resolution esophageal manometry presenting abnormality of the upper esophageal sphincter.
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PMID:A case of amyotrophic lateral sclerosis presented as oropharyngeal Dysphagia. 2068 Jan 72

Primary malignant melanoma of the esophagus (PMME) is a rare disease that is characterized by aggressive invasion, early metastasis, and poor prognosis. Treatment protocols are not well-established. To understand this condition more precisely, we performed a retrospective review of eight cases of PMME diagnosed at the Thoracic Department of the Cancer Center at Sun Yat-Sen University between 1985 and 2009. Eight PMME patients (five men and three women) with a mean age of 58 years (range: 48 to 72 years) were included. Dysphagia was the most common presenting symptom. All patients underwent an Ivor-Lewis esophagogastrectomy and lymph node dissection with postoperative adjuvant chemotherapy. One patient with stage III/pT4N0M0 underwent postoperative chemotherapy plus radiotherapy. Four patients died of distant metastases. The median survival time was 28 months (range: 11 months to 6 years). Our data confirm that PMME is a highly aggressive disease with a poor prognosis. If the diagnosis is suspected or confirmed as PMME, and the patients have no distal metastases or extensive lymph node enlargement, we suggest that surgery should be the first choice of treatment. With regard to adjuvant therapy, we recommend the addition of chemotherapy. The role of radiotherapy remains questionable and requires further investigation.
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PMID:Primary malignant melanoma of the esophagus: a study of clinical features, pathology, management and prognosis. 2104 Jan 50

Amyotrophic lateral sclerosis (ALS) is a rare disease causing degeneration of the upper and lower motor neuron. Involvement of the bulbar motor neurons often results in fast progressive dysphagia. While cortical compensation of dysphagia has been previously shown in stroke patients, this topic has not been addressed in patients suffering from ALS. In the present study, we investigated cortical activation during deglutition in two groups of ALS patients with either moderate or severe dysphagia. Whole-head MEG was employed on fourteen patients with sporadic ALS using a self-paced swallowing paradigm. Data were analyzed by means of time-frequency analysis and synthetic aperture magnetometry (SAM). Group analysis of individual SAM data was performed using a permutation test. We found a reduction of cortical swallowing related activation in ALS patients compared to healthy controls. Additionally a disease-related shift of hemispheric lateralization was observed. While healthy subjects showed bilateral cortical activation, the right sensorimotor cortex was predominantly involved in ALS patients. Both effects were even stronger in the group of patients with severe dysphagia. Our results suggest that bilateral degeneration of the upper motor neuron in the primary motor areas also impairs further adjusted motor areas, which leads to a strong reduction of 'swallowing related' cortical activation. While both hemispheres are affected by the degeneration a relatively stronger activation is seen in the right hemisphere. This right hemispheric lateralization of volitional swallowing observed in this study may be the only sign of cortical plasticity in dysphagic ALS patients. It may demonstrate compensational mechanisms in the right hemisphere which is known to predominantly coordinate the pharyngeal phase of deglutition. These results add new aspects to our understanding of the pathophysiology of dysphagia in ALS patients and beyond. The compensational mechanisms observed could be relevant for future research in swallowing therapies.
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PMID:Cortical processing of swallowing in ALS patients with progressive dysphagia--a magnetoencephalographic study. 2162 45

Bacterial thyroiditis is a rare disease, and one of which the clinical symptoms and signs are frequently misleading. On the other hand, prompt diagnosis is crucial for successful treatment. We report the case of an 82 year-old man with diabetes mellitus type 2 and a history of steroid treatment who presented with severe odynophagia and dysphagia associated with fever, chills, sore throat and right ear pain. Based on the clinical picture, radiological studies, thyroid cytology, blood and thyroid aspirate culture, suppurative thyroiditis caused by Salmonella enteritidis was diagnosed. The patient was successfully treated with antibiotics and surgical drainage.
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PMID:Suppurative thyroiditis caused by Salmonella enteritidis. 2206 8

Esophageal intramural pseudodiverticulosis (EIPD) is a rare disease of unknown etiology that displays multiple pseudodiverticula radiologically, leading to benign esophageal stricture. Dysphagia, which sometimes slowly progresses, is the main symptom in the majority of cases. We here report a 59-year-old male EIPD patient who suffered from severe dysphagia. Radiography and endoscopy of this patient disclosed a severe constriction in the upper thoracic esophagus. Although we tried several endoscopic procedures including frequent endoscopic balloon dilatation (EBD), the effect was very limited and his dysphagia relapsed shortly after the treatments. During the procedures, we noticed some white, thick, creamy liquid emerging from the orifices of EIPD, and PAS staining of biopsy specimens revealed infection with Candida albicans. Hence, the patient was given anti-fungal medicine in addition to EBD. The additional treatment with anti-fungal medicine dramatically improved his symptoms and the esophageal constriction. This case suggests that anti-fungal treatment is an effective first-line therapy even against a severe form of esophageal constriction in EIPD.
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PMID:A case of severe esophageal intramural pseudodiverticulosis whose symptoms were ameliorated by oral administration of anti-fungal medicine. 2261 54

A 74-year-old woman was admitted to our hospital due to severe nuchal pain and occipitalgia. Neurological examination found neck stiffness but no throat pain or dysphagia. Blood examination showed slight elevation of white blood cell count, but C-reactive protein level was normal. Cerebrospinal fluid examination found no abnormalities. Computed tomography (CT) and magnetic resonance (MR) imaging demonstrated no abnormalities in the brain. Cervical CT showed a small calcification in front of the C1 body. Cervical T(2)-weighted MR imaging showed a high intensity area in front of the upper cervical vertebral body from C1 to C4, suggesting inflammation of the longus colli muscles. We diagnosed acute calcific prevertebral tendonitis. She was administered nonsteroidal anti-inflammatory drugs. Her symptoms gradually improved and she was discharged without neurological deficit 8 days after admission. It is important to be aware of the possibility of this rare disease in a patient with severe occipitalgia but no sign of intracerebral lesion.
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PMID:Acute calcific prevertebral tendonitis mimicking tension-type headache. 2300 73


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