Internal medicine
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Case Reports
A Japanese family with multiple lung cysts and recurrent pneumothorax: a possibility of Birt-Hogg-Dubé syndrome.
We herein report a Japanese family lineage, possibly demonstrating Birt-Hogg-Dubé (BHD) syndrome. A 29-year-old nonsmoking woman was admitted to our hospital due to spontaneous pneumothorax. A chest CT showed multiple lung cysts, and breast cancer was simultaneously detected that needed priority surgical treatment. ⋯ In a genetic analysis of her half brother, the mutation of the BHD gene was identified. BHD syndrome is a rare autosomal and dominantly inherited disorder, which has three characteristics: multiple lung cysts that may be associated with pneumothorax, skin fibrofolliculomas, and renal neoplasm. For multiple-cystic disease of the lungs with an unknown etiology, or pneumothorax, as seen in a family history, it is necessary to consider the possibility of BHD syndrome.
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Comparative Study
Age- and gender-related differences in correlations between abdominal obesity and obesity-related metabolic risk factors in Japanese.
There have been conflicting data regarding the relationship between obesity and coronary heart disease (CHD) in Japan. Obesity was reported to be a risk factor of CHD in young and middle-aged males, but not in females or older males. The age- and gender-related differences in the correlations between obesity and obesity-related metabolic risk factors might contribute to these conflicting data. ⋯ The correlations between abdominal obesity and obesity-related metabolic risk factors were stronger in younger men than in older men and women among Japanese.
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Case Reports
The middle meningial artery during a migraine attack: 3T magnetic resonance angiography study.
We performed 3T magnetic resonance angiography (MRA) during a spontaneous migraine attack. The patient was a 42-year-old woman migraineur diagnosed by the IHS criteria. ⋯ There were no dramatic changes of vasodilation in the MMA during the attack (2.0 mm), attack-free period (diameter 1.9 mm), or period after medication (1.7 mm), resembling extrapolations of observations in experimental animal models. This finding suggests that the dramatic vasomotion might not be associated with migraine pathophysiology.
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Real-time endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) with a dedicated EBUS bronchoscope has been reported as a minimally invasive and accurate method for sampling specimens from mediastinal and hilar lesions. Using this method, not only cytologic but also histologic specimens, which provide valuable information for a definitive diagnosis, can often be obtained. We report a case of an enlarged metastatic mediastinal lymph node from an unidentified primary papillary thyroid carcinoma that was accurately diagnosed by histological and immunohistochemical examination of tissue obtained by EBUS-TBNA.
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We present the case of a 23-year-old man with steroid-resistant nephrotic syndrome due to minimal change disease who was treated with rituximab. The patient was resistant to conventional therapy. We therefore treated him with a single dose of rituximab (375 mg/m(2)). ⋯ However, six months later, the patient was administered a second dose of rituximab as the peripheral B cell counts began to recover. Thereafter, at present, that is, one year after the first rituximab administration, complete remission has been maintained. We conclude that rituximab may be an effective treatment agent for resistant nephrotic syndrome and the peripheral B cell count may be a useful marker in such patients for preventing disease relapse.