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)

Bleeding involving the upper airway is a rare, highly emergent complication of hemophilia. This report describes the occurrence of two distinct episodes in a patient with severe factor IX deficiency. Clinical data from other cases described in the literature is summarized with regard to predisposing factors, age of incidence, presenting symptomatology, diagnosis, and management. Pertinent features of this bleeding complication include: high prevalence involving the pediatric population, presentation with non-specific symptoms (e.g., sore throat, dysphagia) early in the course of the bleeding episode, progression to complete upper respiratory obstruction if not recognized and treated, and the need for relatively high levels of the deficient coagulation factor for a period of at least 7 days to ensure resolution of the hematoma. The diagnosis is confirmed by observing retropharyngeal soft-tissue widening on lateral neck x-ray or alternatively, via cervical computed tomography.
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PMID:Hemorrhage involving the upper airway in hemophilia. 374 23

Endoscopic experience in patients with acquired immunodeficiency syndrome (AIDS) has rarely been reported in Taiwan. We present our experience in 9 AIDS patients (8 male and 1 female, age from 26 to 63 years) with 12 examinations. The risk factor of these patients were bisexual in 3, homosexual in 2, hemophilia in 1, drug abuse in 1, and paid-sex in 2. Odynophagia or dysphagia was the major complaints. Oral ulcers or/and thrush were noted in 8 patients. Endoscopic findings included negative (6/12), candidiasis (3/12), erosions (1/12), ulcers (1/12) and ulcer scar (1/12) in esophagus; negative (8/12), gastritis (1/12), erosions (1/12), ulcers (1/12) and Kaposi's sarcoma (1/12) in stomach; and negative (11/12) and duodenitis (1/12) in duodenum. Patients with esophageal candidiasis always had oral thrush. Dysphagia was highly correlated with positive endoscopic findings in esophagus. It is important for an endoscopist to identify clinical symptoms and to examine patient's oral cavity before an endoscopic examination. The endoscopist must keep himself from being infected by exposure to contaminated blood and secretion and avoid dissemination of this horrible disease by undisinfected instruments.
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PMID:Endoscopic examination in patients with acquired immunodeficiency syndrome: Taiwan experience. 840 71

We report the case of a 67 year old patient suffering from acute airway obstruction caused by hemorrhage localized to the tongue, mouth cavity and hypopharynx, with no evidence of bleeding in his history. The patient presented initially with a globus feeling of the neck, dysphagia and a sore throat. CT scan revealed a swelling of the lingual and sublingual areas and the pharyngeal wall. Next day, there was an immediate life-threatening event caused by progressive bleeding with airway obstruction and an inability to intubate requiring coniotomy. The etiology of the hemorrhage was confirmed by finding a depletion of factor VIII and the presence of auto-antibody directed against this factor. Based on this case report and a review of the literature, we discuss the diagnosis and treatment of acquired hemophilia.
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PMID:[Acute airway obstruction in acquired hemophilia A]. 1528 12

The aim of this paper is to review the literature and identify orofacial manifestations of hematological diseases, with particular reference to anemias and disorders of hemostasis. A computerized literature search using MEDLINE was conducted for published articles on orofacial manifestations of hematological diseases, with emphasis on anemia. Mesh phrases used in the search were: oral diseases AND anaemia; orofacial diseases AND anaemia; orofacial lesions AND anaemia; orofacial manifestations AND disorders of haemostasis. The Boolean operator "AND" was used to combine and narrow the searches. Anemic disorders associated with orofacial signs and symptoms include iron deficiency anemia, Plummer-Vinson syndrome, megaloblastic anemia, sickle cell anemia, thalassaemia and aplastic anemia. The manifestations include conjunctiva and facial pallor, atrophic glossitis, angular stomatitis, dysphagia, magenta tongue, midfacial overgrowth, osteoclerosis, osteomyelitis and paraesthesia/anesthesia of the mental nerve. Orofacial petechiae, conjunctivae hemorrhage, nose-bleeding, spontaneous and post-traumatic gingival hemorrhage and prolonged post-extraction bleeding are common orofacial manifestations of inherited hemostatic disorders such as von Willebrand's disease and hemophilia. A wide array of anemic and hemostatic disorders encountered in internal medicine has manifestations in the oral cavity and the facial region. Most of these manifestations are non-specific, but should alert the hematologist and the dental surgeon to the possibilities of a concurrent disease of hemopoiesis or hemostasis or a latent one that may subsequently manifest itself.
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PMID:Orofacial manifestations of hematological disorders: anemia and hemostatic disorders. 2204 88

Acquired haemophilia A and severe acquired achalasia are both very rare conditions with unknown aetiology. Haemophilia A is a haemorrhagic disease induced by deficiency or malfunction of coagulation factor VIII. Congenital haemophilia is an inherited disease transmitted by the mother through X-linked inheritance and primarily affects males. However, acquired haemophilia A is a serious, sudden-onset, autoimmune disease that affects either sex. In addition, achalasia is a disease of the oesophagus caused by abnormal function of the nerves and muscles. It causes swallowing difficulties due to the inability of the lower oesophageal sphincter to relax during swallowing, leading to dysphagia, regurgitation and chest pain. In this report, we describe the case of a patient with severe, newly diagnosed, acquired haemophilia A with long-standing, recurrent achalasia; the achalasia had recurred 3 times despite complete and proper surgical fixation. Acquired haemophilia A is treated with immunosuppressive therapy. High-dose steroid therapy was administered for 7 months, during which the patient responded well; moreover, the achalasia did not recur for more than 2 years. The response of the achalasia to immunosuppressive therapy suggests that achalasia may be an autoimmune disorder and that there may be an association between both diseases. The findings of the present case suggest that achalasia may favourably respond to steroid therapy as a first-line treatment prior to surgery.
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PMID:Severe recurrent achalasia cardia responding to treatment of severe autoimmune acquired haemophilia. 2313 52

BACKGROUND Massive tongue hemorrhage in patients with hemophilia is a medical emergency because it can lead to airway obstruction. However, managing bleeding in patients with inhibitors is more difficult than in patients without inhibitors. We report a case of life-threatening massive tongue and retropharyngeal hematoma in a patient with hemophilia A who had inhibitors. CASE REPORT The patient was a 71-year-old man with severe hemophilia A with high-responding inhibitors. He was admitted to our hospital with dysarthria and dysphagia secondary to a massive tongue hematoma. Although bypassing therapy was started immediately after admission, he rapidly developed an airway obstruction and cardiopulmonary arrest secondary to suffocation. Cardiopulmonary resuscitation and surgical cricothyrotomy were performed, which restored his pulse and breathing. On day 5 of hospitalization, he underwent tracheotomy under inhibitor-neutralizing therapy, and we began emicizumab on day 19 of hospitalization to prevent further bleeding events. He recovered and was transferred to another hospital for rehabilitation on day 64 of hospitalization. CONCLUSIONS Because tongue hematomas progress dramatically within a few days, prompt airway maintenance by tracheotomy under appropriate hemostatic therapy must be considered. Furthermore, emicizumab induction after primary hemostasis prevents further bleeding. We suggest that initiating emicizumab therapy is a good choice to prevent further bleeding after critical bleeding events if the patient has not received the drug previously.
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PMID:Life-Threatening Tongue and Retropharyngeal Hemorrhage in a Patient with Hemophilia A with Inhibitors. 3130 39