Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. In December, she felt difficulty in walking due to muscle weakness. In January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. In middle of February, she complained of high fever over 39.0 degrees C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases.
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PMID:[A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide]. 1555 24

A 65-year-old man was admitted to our hospital because of dyspnea on exertion. He had oculocutaneous albinism innately and his parents were consanguineous. His chest roentgenogram on admission showed reticulo-nodular infiltrates and cystic changes throughout both lung fields, and 7 cm mass in the left middle field. Cytology of bronchoalveolar lavage fluid (BALF) revealed macrophages containing ceroid. The diagnosis of HPS was made clinically and the tumor was diagnosed as poorly differentiated adenocarcinoma of the lung. He died of respiratory failure. By autopsy, additional well-differentiated adenocarcinoma was detected. Cytology of BALF was useful to confirm ceroid accumulation in the lung.
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PMID:Diagnostic usefulness of bronchoalveolar lavage in Hermansky-Pudlak syndrome: a case with double lung cancers. 1557 50

The performance requirements prescribed in IEC 62387-1, 2007 recommend linear, additive algorithms for external dosimetry [IEC. Radiation protection instrumentation--passive integrating dosimetry systems for environmental and personal monitoring--Part 1: General characteristics and performance requirements. IEC 62387-1 (2007)]. Neither of the two current standards for performance of external dosimetry in the USA address the additivity of dose results [American National Standards Institute, Inc. American National Standard for dosimetry personnel dosimetry performance criteria for testing. ANSI/HPS N13.11 (2009); Department of Energy. Department of Energy Standard for the performance testing of personnel dosimetry systems. DOE/EH-0027 (1986)]. While there are significant merits to adopting a purely linear solution to estimating doses from multi-element external dosemeters, differences in the standards result in technical as well as perception challenges in designing a single algorithm approach that will satisfy both IEC and USA external dosimetry performance requirements. The dosimetry performance testing standards in the USA do not incorporate type testing, but rely on biennial performance tests to demonstrate proficiency in a wide range of pure and mixed fields. The test results are used exclusively to judge the system proficiency, with no specific requirements on the algorithm design. Technical challenges include mixed beta/photon fields with a beta dose as low as 0.30 mSv mixed with 0.05 mSv of low-energy photons. Perception-based challenges, resulting from over 20 y of experience with this type of performance testing in the USA, include the common belief that the overall quality of the dosemeter performance can be judged from performance to pure fields. This paper presents synthetic testing results from currently accredited function-based algorithms and new developed purely linear algorithms. A comparison of the performance data highlights the benefits of each approach and demonstrates that, at least for some dosemeter designs, it is possible for a single purely linear algorithm to satisfy both US and IEC performance requirements.
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PMID:Linear vs. function-based dose algorithm designs. 2119 27