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Rare Clinical Entity (rare + clinical_entity)
Selected AbstractsEpsilon-Like Electrocardiographic Pattern in a Patient with Brugada SyndromeANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 3 2009Ozcan Ozeke M.D. Both Brugada syndrome (BrS) and arrhythmogenic right ventricle dysplasia/cardiomyopathy (ARVD/C) can cause repolarization abnormalities in right precordial leads and predispose to sudden cardiac death (SCD) due to ventricular arrhythmias. Although there is controversy over whether BrS is distinct from ARVD/C, it is believed that both are different clinical entities with respect to both the clinical presentation and the genetic predisposition. The coexistence of these two relatively rare clinical entities is also reported, but, some hypothesized that it is more possible that disease of the right ventricular muscle might accentuate the Brugada electrocardiographic pattern. In clinic practice, there may be cases where the dividing line is not so clear. We report a 33-year-old male presenting with recurrent syncope, who has a peculiar pattern of coved-type ST-segment elevation (ST-SE) with epsilon-like wave in right precordial leads. [source] BLUE RUBBER BLEB NEVUS SYNDROME: TREATMENT OF MULTIPLE GASTROINTESTINAL HEMANGIOMAS WITH ARGON PLASMA COAGULATORDIGESTIVE ENDOSCOPY, Issue 1 2009Enders K.W. Ng Blue rubber bleb nevus syndrome is a rare clinical entity characterized by the formation of multiple blue or purplish rubbery cavernous hemangiomas on the skin and other epithelial surfaces. Involvement of the gastrointestinal tract is common and often presents with crippling anemia as a result of chronic occult blood loss. While surgical extirpation is an option for symptomatic hemangiomas in the intestine, endoscopic therapy is more appealing for lesions found in the stomach and colon. Here we report the successful use of argon plasma coagulation in the management of an adult with multiple hemangiomas in her colon and terminal ileum. [source] A rare case of multiple myeloma initially presenting with pseudoachalasiaDISEASES OF THE ESOPHAGUS, Issue 6 2009Georgia Lazaraki SUMMARY Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia. [source] Outcome of sinonasal melanoma: Clinical experience and review of the literature,HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 10 2010Thomas N. Roth MD Abstract Background. Primary sinonasal malignant melanoma (SNMM) is a rare clinical entity. There is neither a classification nor a staging system nor an evidence-based treatment concept established. Our objective was to find potential risk factors predicting the outcome. Methods. Twenty-five patients with histologically confirmed SNMM were consecutively included and retrospectively analyzed. Staging methods were nasal endoscopy, CT, MRI, and positron emission tomography (PET) scan. Patients were selected for a curative or palliative concept. All patients had postoperative follow-up with control-MRI at 3 and 6 months. Restaging was performed when local recurrence occurred. Results. Nineteen patients underwent primary surgery with curative intention; in 16 cases with tumor free margins. Thirteen patients (68%) had transnasal endoscopic surgery, 4 lateral rhinotomy, and 2 transfacial approach with orbital exenteration. Six patients (32%) had palliative therapy and 7 patients (37%) had adjuvant radiotherapy. Despite radical operations, 6 patients (37%) showed local recurrence and 8 patients (50%) developed distant metastasis. In 2 patients with incomplete surgery, regional metastasis was noted. The median disease-free interval was 18 months, and the median overall survival rate was 23 months. Conclusion. SNMMs of the ethmoid and maxillary sinuses have a worse prognosis than other localizations in the nasal cavity; infiltration into the skull base, orbit, or facial soft tissue correlates with a very poor outcome corresponding to the palliative situations. Furthermore, local recurrence insinuates aggressive disease with short survival rate. A main difference from its cutaneous counterpart seems to be a primary tendency to hematogenic spread. Further research is needed to confirm these findings. © 2010 Wiley Periodicals, Inc. Head Neck, 2010 [source] Subdural empyema and cerebellar abscess due to chronic otitis mediaINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 2 2004K.S. Polyzoidis Summary The infratentorial variety of the subdural empyema, with or without coexisting cerebellar abscess, is a rare clinical entity that carries a high mortality rate. We briefly describe the case of a 49-year-old man presented with severe debility, fever and an obviously neglected chronic otitis media. The patient had refused surgical treatment several months ago. After admission, his level of consciousness began to deteriorate, and the radiological studies showed infratentorial subdural suppuration extending into the right cerebellar hemisphere, along with chronic pyogenic infection of the middle ear and the mastoid process. Radical mastoidectomy was performed first, followed by extensive right posterior fossa craniectomy. The two subdural collections and the cerebellar abscess were successfully evacuated. Subsequently, he received post-operative antibiotic treatment for 6 weeks. At follow-up, 10 months after surgery, his neurological recovery was complete except for a minor residual cerebellar dysfunction on the right. This unusual case highlights that in patients presented with severe intracranial complications of chronic otitis media, early diagnosis and radical surgical intervention may be life saving. [source] Middle ear cancer: A population-based study,,THE LARYNGOSCOPE, Issue 10 2009Richard K. Gurgel MD Abstract Objectives/Hypothesis: Primary carcinoma of the middle ear is a rare clinical entity, best suited for evaluation using a population-based database. The objective of this study was to utilize the Surveillance, Epidemiology, and End Results (SEER) database to determine the incidence, treatment, and survival of middle ear carcinoma. Study Design: Analysis of national cancer database. Method: Using SEER*Stat software, records for patients diagnosed with middle ear carcinoma between 1973 and 2004 were extracted from the SEER database. Five-year, observed survival was analyzed, with significant differences determined by the Wilcoxon statistic. Results: The 5-year observed survival rate for the 215 patients in this study was 36.4%. Histologic subtypes included squamous cell carcinoma (62.8%), adenocarcinoma (18.2%), other carcinomas (13.0%), and noncarcinomas (6.0%), with 5-year survival rates of 23.9%, 65.0%, 60.0%, and 38.6%, respectively (P = .003). Of the 123 patients with known stage, 23.6% had local, 69.1% had regional, and 7.3% had distant disease, with their 5-year survival rates being 64.9%, 34.2%, and 0%, respectively (P < .001). Treatment included surgery (31.2%), radiation (16.3%), surgery and radiation (38.6%), or no treatment (8.4%) with 5-year survival of 69.2%, 14.6%, 26.4%, and 0%, respectively (P < .001). Conclusions: Patients with primary middle ear carcinoma have a relatively poor prognosis. However, subsets of patients, such as those with adenocarcinomas and with localized tumors, demonstrated significantly better survival. Surgery alone had significantly better survival than the other treatment groups, presumably due to less advanced disease in this treatment group. These data are useful in counseling patients and understanding the natural history of middle ear carcinoma. Laryngoscope, 2009 [source] Incidence and clinical characteristics of symptomatic choroidal metastasis from lung cancerACTA OPHTHALMOLOGICA, Issue 5 2008Klaus-Martin Kreusel Abstract. Purpose:, To determine the clinical characteristics of symptomatic choroidal metastasis (CM) resulting from metastatic lung cancer. Methods:, Twenty-two consecutive patients with symptomatic CM resulting from lung cancer were retrospectively reviewed for ocular findings, medical history and systemic disease. All patients underwent a complete screening for further organ metastasis by computed tomography (CT) and bone scintigraphy. Annual frequency of CM was determined and compared with the incidence predicted from ocular screening studies. Results:, In eight of 22 (36%; 95% confidence interval [CI] 17,59) patients, lung cancer had been diagnosed before occurrence of CM, with a median interval of 13 months. In 14 patients lung cancer was detected after diagnosis of CM, with a median interval of 1 month. Choroidal metastasis was unilateral, solitary and located close to or at the posterior pole in the majority of patients. Further organ metastasis with a median number of three affected organ systems was present in 19 (86%; 95% CI 65,97) patients. Median survival after diagnosis of symptomatic CM was 13 months, by contrast with 2 months in lung cancer patients with CM identified in an ocular screening study. The mean number of patients in Berlin diagnosed with symptomatic CM was 1.4 per year, which was two orders of magnitude less than predicted from screening studies. Conclusions:, Symptomatic choroidal lung cancer metastasis in the majority of patients presents as a solitary tumour before diagnosis of lung cancer in patients with multiple organ systems affected by metastatic disease. Contrary to predictions from ocular screening studies, it is a rare clinical entity. [source] Orofacial granulomatosis in childhood,a clinical entity that may indicate Crohn's disease as well as food allergyACTA PAEDIATRICA, Issue 7 2009Robert Saalman Abstract Aim:, Orofacial granulomatosis (OFG) is a rare clinical entity with orofacial swelling in association with oral lesions such as mucosal oedema, ulcerations and mucosal tags. The aim of this prospective study was to evaluate the connection between OFG in childhood and systemic disease. Methods:, During a 3-year period, eight children (9,16 years old) who had been referred to the clinic of oral medicine were diagnosed solely with OFG. Thus, none of them had any known systemic disease or gastrointestinal symptoms at the time of referral. The children were then medically examined and followed up for 6,8 years at the department of paediatrics for systemic disease with specific emphasis on inflammatory disorders elsewhere in the gastrointestinal tract. Results:, During follow-up, four patients were diagnosed with Crohn's disease (CD). Further, one girl was found to have a food allergy-induced OFG, with delayed-type hypersensitivity to oats. One boy developed both diabetes and celiac disease during the follow-up. Only two patients had no diagnosis of systemic disease at the end of the observation period. Conclusion:, OFG in childhood seems to be frequently related to systemic disease, and children with OFG should be referred to a paediatrician for examination and follow-up. [source] | ||||||||||