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)

Myasthenia gravis (MG) is a rare complication of allogeneic bone marrow transplantation (BMT). We present the 11th case in the medical literature, a 23-year-old female 100 months post-allogeneic bone marrow transplantation for acute myelogenous leukemia (AML). After discontinuation of immunosuppression for chronic graft-versus-host disease (GVHD) involving skin, gastrointestinal tract and lacrimal glands, the patient developed severe, progressive dysphagia initially attributed to esophageal candidiasis. With the development of muscle weakness, ptosis, and dysphonia the diagnosis of generalized myasthenia gravis was suspected, and confirmed by elevated anti-acetylcholine receptor antibody titer and a positive edrophonium challenge. Prednisone and pyridostigmine produced improvement, and thymectomy was performed without pathologic evidence of thymoma. Recurrent post-operative respiratory distress required transient mechanical ventilation. Twenty-seven months after diagnosis, the patient requires maintenance prednisone to control symptoms of myasthenia gravis. The clinical features of all reported cases of MG post-allogeneic BMT are reviewed, and universal features include an association with decreasing immunosuppression, the presence of other manifestations of chronic GVHD, anti-acetylcholine receptor antibodies, and the absence of an associated thymoma. HLA Cw1, Cw7 and DR2 were identified at frequencies significantly above that expected from HLA antigen prevalance studies, and may be markers for increased risk of developing MG post-allogeneic BMT. No statistically significant associations with HLA A2, B7, B35 or donor-recipient sex mismatch were present. Reinstitution of immunosuppression and standard therapies for myasthenia gravis were effective in the majority of cases. The role of thymectomy in this population remains unclear.
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PMID:Myasthenia gravis in association with allogeneic bone marrow transplantation: clinical observations, therapeutic implications and review of literature. 915 70

Cervical aortic arch is a rare type of aortic arch anomaly that is presumed to result from persistence of the third aortic arch and regression of the normal fourth arch. Most of the patients with this anomaly are asymptomatic, but symptoms of dysphagia and respiratory distress due to the compression by the vascular ring have been reported. Other findings such as a supraclavicular pulsatile mass, blood pressure discrepancies between the upper limbs, and loss of femoral or opposite-upper-limb pulses with compression of the cervical mass may also be present. In this article a twenty-two-year-old woman with symptomatic cervical aortic arch is presented. The patient had a left cervical pulsatile mass and elevated blood pressure on her right upper limb and was treated surgically with reanastomosis of the aorta.
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PMID:Cervical aortic arch. A case report. 924 66

Tracheobronchial ruptures are rare but potentially lifethreatening events. We report on the case of a 34-year-old suicidal unrestrained car driver, who developed subcutaneous and mediastinal emphysema and right-sided haematothorax following blunt thoracic trauma. Fibreoptical inspection of the tracheobronchial system revealed a rupture (approximately 2 cm in length) of the pars membranacea of the trachea ending shortly above the carina. CT-scan confirmed the diagnosis of mediastinal emphysema, tracheal rupture and, in addition, left-sided pulmonary contusion. A repair of the tracheal tear was performed by right-sided thoracotomy using a double-lumen tube. The left-sided double-lumen tube was used postoperatively to achieve respirator ventilation with low pressure on the tracheal lumen and on the suture of the tracheal tear. On the other hand, sufficient airway pressure with PEEP for the left lung showing contusion could be provided, using the endobronchial tube. The postperative course was without complications. The patient was on respiratory support for three days due to his-pulmonary contusion. Following final endoscopic control of the trachea he was discharged from the ICU one week after the trauma. The clinical and radiological signs of tracheobronchial ruptures are discussed (respiratory distress, haemoptysis, cyanosis, localised pain, hoarseness, coughing, dysphagia, stridor, subcutaneous emphysema and pneumothorax, tension pneumothorax, mediastinal emphysema). Fibreoptic bronchoscopy is the present gold standard for confirming the diagnosis. The surgical and anaesthesiological approach to the management of tracheobronchial ruptures is described reviewing the current literature.
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PMID:[Diagnosis and therapy of tracheal rupture after blunt thoracic trauma]. 928 31

We report a 65-year-old male with progressive supranuclear palsy (PSP) who developed bilateral vocal cord abductor paralysis (VCAP). The patient was admitted to our hospital because of impaired gait. He was well until two years earlier, when he began to walk unsteadily. During the next two years, dysarthria and dysphagia developed and his gait worsened gradually. On admission, neurological examination showed impaired vertical and incomplete lateral gaze. His speech was slow and monotonous. Contractures were found in the neck muscles and elbows. The deep tendon reflexes were increased in the upper and decreased in the lower extremities. Babinski sign was negative. Snout and forced grasping reflexes were elicited. He showed marked bradykinesia. Magnetic resonance imaging revealed a midbrain tectum atrophy. Single photon emission tomography showed severe hypoper-fusion in the frontal cortex. No improvement was provided by the administration of levodopa-carbidopa, bromocriptine, droxydopa and amitriptyline. One month after admission, inspiratory stridor developed at night. The laryngofiberscopic examination demonstrated VCAP. An emergency tracheostomy relieved his respiratory distress. Although VCAP rarely occurs in neurodegenerative disorders other than multiple system atrophy, attention to VCAP should be required in PSP patients.
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PMID:[A case of progressive supranuclear palsy associated with bilateral vocal cord abductor paralysis]. 936 83

Advanced forms of diffuse idiopathic skeletal hyperostosis or Forestier's disease can induce dysphagia and significant respiratory symptoms such as hoarseness, dyspnea, snoring and laryngeal stridor. Severe respiratory distress is very rare. Two identical cases of chronic respiratory distress due to bilateral vocal cord adduction-fixation in conjunction with skeletal pathology in the cervical area are presented. The clinical, diagnostic and therapeutic work-up and the prognostic outcome of the laryngeal pathology are discussed.
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PMID:Vocal cord immobilisation in diffuse idiopathic skeletal hyperostosis (DISH). 958 Dec 3

In order to evaluate complications due to cervical spine surgery using the anterior cervical approach a prospective study was conducted on 125 patients. ENT examination with the fibroscope was employed for all the patients before the procedure. The patients were operated on under general anesthesia and were intubated with an armoured tube, and then were placed in an intensive care unit for 24 hours. Assessment of deglutition and an ENT examination were performed the day after surgery. Before surgery, two cases of vocal cord paralysis were noted. 111 patients (88.8%) presented with subjective disorders: problems such as sore throat, odynophagia, dysphagia, dysphagia with overspill and hoarseness were respectively noted in 55 (44%), 34 (27.2%), 32 (25.6%), 11 (8.8%) and 13 (10.4%) cases. Dyspnoea was found in 2 cases (1.6%). 117 patients (93.6%) presented postoperative anomalies which were found on the posterolateral pharyngeal wall, on the arytenoids and on posterior third of the vocal cords. Inflammatory and/or swollen lesions were slight, moderate, significant or very significant in respectively 22.4%, 22.4%, 15.2% and 1.6% of cases. Very significant circumferential swelling of the pharyngeal wall and of the arytenoids was responsible for two cases of respiratory distress, and the patients required reintubation and return to theatre. Severe pharyngeal lesion correlated with duration of surgery (r = 0.20; p < 0.05), with the number levels of fusion (r = 0.02; p < 0.02) and with the age of the patient (p < 0.02). Six patients presented problems of mobility of the vocal cords: 3 had a right vocal cord paresis which was temporary and 3 had paralysis, also on the right but which persisted. There were no other complications. It is concluded that (i) ENT complications are frequently found in postoperative cervical spine surgery using the anterior cervical approach, some of them being severe. An ENT examination must be performed before the procedure for legal reasons. It is also recommended in the postoperative period in the case of discomfort; (ii) patients need to be placed in an intensive care unit during for the first 24 hours (iii). This study needs to be attended over more patients (iv) comparison with a control group of patients having non cervical surgery and intubated in the same way is needed to differentiate lesions related to surgery or intubation.
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PMID:[A prospective study of ENT complication following surgery of the cervical spine by the anterior approach (preliminary results)]. 977 50

Enlargement of the thyroid is common, especially in areas of endemic iodine deficiency. Substernal enlargement of a goitre can cause compression of several mediastinal structures. As a consequence of tracheal compression and tracheomalacia, syndromes of chronic respiratory distress occur and intercurrent upper respiratory infections may lead to acute respiratory failure. Superior vena cava syndrome secondary to compression by a substernal goitre may be complicated by venous thrombosis. Although dysphagia is the most frequent oesophageal symptom of a substernal goitre, upper gastrointestinal bleeding from 'downhill' oesophageal varices may be an initial presentation. Arterial compression or thyrocervical steal syndrome by large substernal goitres occasionally cause cerebral hypoperfusion and stroke. Recurrent and phrenic nerve palsies, as well as Horner's syndrome, occur secondary to non-malignant mediastinal goitres and may resolve after surgery. Substernal goitres rarely cause therapy-resistant pleural effusions, chylothorax and pericardial effusion. In conclusion, although cervical goitres are easily recognised, the initial presentation of mainly substernal goitres may be unusual.
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PMID:Compression syndromes caused by substernal goitres. 1019 9

Two cases of combined tracheal and oesophageal stenting for carcinoma of the upper oesophagus invading the tracheo-bronchial tree are described. Case 1 describes the complication of respiratory distress following insertion of a high oesophageal stent. This caused severe stridor which required tracheal stenting. In case 2 prophylactic stenting of the airway prior to oesophageal stenting was performed as a staging CT demonstrated severe compromise of the distal trachea/bronchus in a patient who was experiencing both dysphagia and dyspnoea. In both cases the respiratory and dyspnoeic symptoms were relieved. These cases illustrate the effective use of tracheal/bronchial and oesophageal metal stents in palliating patients with combined respiratory and dysphagic symptoms secondary to oesophageal malignancy. When treating high oesophageal tumours tracheal compromise should be considered and prophylactic stenting of the airway prior to oesophageal stenting performed to avoid further airway compromise when the oesophageal stent expands.
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PMID:Tracheal and oesophageal stenting for carcinoma of the upper oesophagus invading the tracheo-bronchial tree. 981 95

A female newborn presented with a huge mass protruding from the mouth. She had no respiratory distress but had difficulty swallowing. The mass originated from the tongue and was completely extirpated on the day of birth. The histologic diagnosis was mature teratoma. Three days later, another mass measuring 1 cm in diameter was found attached to the upper pharyngeal wall and was also completely extirpated 20 days after birth. The histologic diagnosis was also mature teratoma. The infant had a complete cleft palate, but no other malformation was found. Two years after surgery there is no sign of recurrence.
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PMID:Teratoma of the tongue in neonates: report of a case and review of the literature. 988 Jul 3

A 61-year-old woman in excellent health and taking no medications presented with progressive dyspnea on exertion. She did not exhibit diplopia, dysphagia, dysarthria or muscle weakness. Her condition deteriorated, and respiratory distress developed. The patient was intubated and placed on mechanical ventilation. A computed tomogram of her chest revealed a moderately enlarged thymus gland unsuspected on a plain x-ray film. Myasthenia gravis was diagnosed on the basis of a high acetylcholine receptor antibody titer. Treatment started with prednisolone, anticholinesterase agent, and plasma exchange. The patient underwent a thymectomy 3 weeks after her diagnosis. Her symptoms were brought under control by anticholinesterase agent and prednisolone. This case illustrates the need to consider myasthenia gravis as well as other motor-neuron disorders when evaluating individuals presenting acute respiratory failure of unknown origin.
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PMID:[Myasthenia gravis presenting as isolated respiratory failure]. 989 33


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