No abstract available.
Gaucher Disease*

To evaluate characteristic CT findings of orbital pseudotumor and to define differentialpoints from other pathology, the authors retrospectively reviewed CT of 19 patients who were prooen to have orbital pseudotumor by clinical course and, in some cases, biopsy. A variety of CT findings including extraocular muscle thickening(11 cases), streaky infiltration of retroorbital fat(11 cases), mass formation(10 cases), optic nerve thickening (6 cases), conjunctival thickening (5 cases), scleral thickening(4cases), enlarged lacrimal gland(4 cases) and destruction of orbital bone (2 cases) were observed. Thickening of the anterior portion and irregular margin were characteristic findings of extraocular muscle and optic nerve lesions. Mass formation predominantly occurs in the anterior portion of the orbit. In most cases more than two orbital structures are involved by lesion.
Biopsy ; Humans ; Optic Nerve ; Orbit* ; Orbital Pseudotumor* ; Pathology ; Retrospective Studies

Simulated three-dimensional (3D) imaging represents reformation of conventional sectional imaging data into a series of images that closely resemble the original studied structure. We tried to make 3D mucosal surface images of the sinonasal cavities, pharynx and larynx, and evaluated the feature of normal anatomy in 28 subjects. In the sinonasal cavities, 3D imaging was capable of demonstrating the inner wall of paranasal sinuses and its openings, and general status status of the nasal cavity. In the nasal cavity. In the nasopharynx, 3D imaging provided an easy concept of sectional images as 3D picture and displayed anatomic subsites and lesions comparable to that in fiberscope. In addition, 3D imaging had advantages in overcoming the technical limitations in fiberscope. In the larynx and hypopharynx, 3D imaging gave a 3D concept of the laryngeal structures and presented additive information not seen in axial iamges thus enabling access to regions beyond the scope of fiberscope. In conclusion, 3D imaging allows an easy conceptualization of transaxial CT images in complex anatomic areas and provides additional in formations undetectable in transaxial CT. We believe that the spaces of the sinonasal cavities, pharynx and larynx would be a new field of application of 3D image.
Hypopharynx ; Larynx* ; Nasal Cavity ; Nasopharynx ; Paranasal Sinuses ; Pharynx*

Simulated three-dimensional (3D) imaging represents reformation of conventional sectional imaging data into a series of images that closely resemble the original studied structure. We tried to make 3D mucosal surface images of the sinonasal cavities, pharynx and larynx, and evaluated the feature of normal anatomy in 28 subjects. In the sinonasal cavities, 3D imaging was capable of demonstrating the inner wall of paranasal sinuses and its openings, and general status status of the nasal cavity. In the nasal cavity. In the nasopharynx, 3D imaging provided an easy concept of sectional images as 3D picture and displayed anatomic subsites and lesions comparable to that in fiberscope. In addition, 3D imaging had advantages in overcoming the technical limitations in fiberscope. In the larynx and hypopharynx, 3D imaging gave a 3D concept of the laryngeal structures and presented additive information not seen in axial iamges thus enabling access to regions beyond the scope of fiberscope. In conclusion, 3D imaging allows an easy conceptualization of transaxial CT images in complex anatomic areas and provides additional in formations undetectable in transaxial CT. We believe that the spaces of the sinonasal cavities, pharynx and larynx would be a new field of application of 3D image.
Hypopharynx ; Larynx* ; Nasal Cavity ; Nasopharynx ; Paranasal Sinuses ; Pharynx*

Sjogren syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands resulting in dry mouth and eyes. Approximately one-third of patients present with systemic manifestations, but respiratory muscle involvements have been rarely reported. We report a case of acute respiratory failure complicated by primary Sjogren syndrome. Muscle biopsy revealed perivascular lymphocytic infiltrations. Corticosteroid therapy improved respiratory muscle weakness. Sjogren syndrome should be considered as one of the underlying diseases causing acute respiratory failure.
Autoimmune Diseases ; Biopsy ; Exocrine Glands ; Humans ; Mouth ; Muscular Diseases ; Respiratory Insufficiency ; Respiratory Muscles* ; Respiratory Paralysis ; Sjogren's Syndrome*

Current digital radiography systems are rapidly growing in clinical applications. The purpose of this study was to evaluate the characteristics of a mobile digital radiographic system. The performance of the mobile DR system was evaluated by measuring the modulation transfer function (MTF), noise power spectrum (NPS), and detective quantum efficiency (DQE). Measurements were made on a LISTEM Mobix-1000 generator and a Teleoptic PRA Alpha-R4000 detector. Imaging characteristics were measured for these two systems using the IEC-61267 defined RQA5 (kVp: 74, additional filtration: 21 mmAl) radiographic condition. The MTF at 10% was measured as 2.4 cycles/mm and the DQE(0) values for radiation exposure 0.19, 0.5, and 1.3 mR were measured as 54%, 55%, and 76%, respectively. The NPS curves gradually decreased at high spatial frequencies. This high DQE at low frequencies, may be useful for low frequency information. The results suggested that mobile DR system could be integrated with emergency ambulance system in teleradiologic imaging applications.
Ambulances ; Emergencies ; Filtration ; Noise ; Radiographic Image Enhancement

OBJECTIVE: The purpose of this study was to demonstrate the ultrasonographic (US) findings of rupture and the healing process of the medial head of the gastrocnemius ("Tennis Leg"). MATERIALS AND METHODS: Twenty-two patients (age range: 30 to 45 years) with clinically suspected ruptures of the medial head of the gastrocnemius were referred to us for US examination. All the patients underwent US of the affected limb and the contralateral asymptomatic limb. Follow-up clinical evaluation and US imaging of all patients were performed at two-week intervals during the month after injury and at one-month intervals during the following six months. RESULTS: Of the 22 patients who had an initial US examination after their injury, partial rupture of the medial head of the gastrocnemius muscle was identified in seven patients (31.8%); the remaining 15 patients were diagnosed with complete rupture. Fluid collection between the medial head of the gastrocnemius and the soleus muscle was identified in 20 patients (90.9%). The thickness of the fluid collection, including the hematoma in the patients with complete rupture (mean: 9.7 mm), was significantly greater than that seen in the patients with partial tear (mean: 6.8 mm) (p < 0.01). The primary union of the medial head of the gastrocnemius with the soleus muscle in all the patients with muscle rupture and fluid collection was recognized via the hypoechoic tissue after four weeks. CONCLUSION: Ultrasonography is a useful imaging modality for the diagnosis and follow-up examination for the patients suffering with rupture of the medial head of the gastrocnemius.
Ultrasonography/*methods ; Tendon Injuries/*ultrasonography ; Rupture/ultrasonography ; Prognosis ; Muscle, Skeletal/*injuries/*ultrasonography ; Middle Aged ; Male ; Leg Injuries/*ultrasonography ; Humans ; Follow-Up Studies ; Female ; Adult

Primary aldosteronism is characterized by hypokalemic metabolic alkalosis, low plasma renin activity, elevated plasma aldosterone level and can be suspected in the patients with hypertension and unexplained hypokalemia. Small adrenal cortical adenomas are responsible for this syndrome in most cases. The incidence of thyrotoxic periodic paralysis ranges from 1.9 to 6.2 % in Japan. Thyrotoxic periodic paralysis usually subsides following treatment of hyperthyroidism and has good prognosis. A 56 year-old man presented with hyperthyroidism, hypertension and recurrent hypokalemia. During the treatment of hyperthyroidism, he repeatedly experienced weakness of both lower extremities. Hormonal evaluation was performed and he was found to have a 2*2*1.5 cm sized right adrenal tumor by abdominal computerized topography(CT). After right adrenalectomy, hypokalemic periodic paralysis was improved. Both thyroid and adrenal function should be comprehensively investigated in periodic paralysis. In conclusion, physicians must be aware of the possibility of primary aldosteronism in hyperthyroid patients with hypokalemic periodic paralysis. We report a case of aldosteronoma complicated with hyperthyroidism and literatures are reviewed.
Adrenalectomy ; Adrenocortical Adenoma ; Aldosterone ; Alkalosis ; Humans ; Hyperaldosteronism ; Hypertension ; Hyperthyroidism* ; Hypokalemia ; Hypokalemic Periodic Paralysis ; Incidence ; Japan ; Lower Extremity ; Middle Aged ; Paralysis ; Plasma ; Prognosis ; Renin ; Thyroid Gland

Serine protease activity of high temperature requrement 2 (HtrA2) is essential for promoting cell death, as well as for protecting against cellular stresses. An X-ray crystallographic study described the formation of a pyramid shaped homotrimer that is a proteolytically competent form of HtrA2; however, little is known about effects of the trimeric structure of HtrA2 on the natural substrates. In this study, we generated the HtrA2 protein that has a single point mutation at the homotrimerization motif to assess relationship between structure and the proteolytic activity of HtrA2 on its substrates. Using gel filtration, a native gel electrophoresis system, and a co-precipitation assay, we confirm that phenylalanine 149 in HtrA2 is a crucial determinant for the formation of the HtrA2 homotrimeric structure. Moreover, we described that the HtrA2 monomeric form abolished not only autoproteolytic activity, but also the proteolytic activity against XIAP (X-linked inhibitor of apoptosis protein) known as the HtrA2 substrate. Taken together, the results indicate that the homotrimeric structure of HtrA2 is required for executing its serine protease activity.
Alanine/metabolism ; Amino Acid Motifs ; Amino Acid Sequence ; Amino Acid Substitution ; Cell Line ; Chromatography, Gel ; Crystallography, X-Ray ; Escherichia coli/genetics ; Glutathione Transferase/metabolism ; Hydrolysis ; Molecular Sequence Data ; Phenylalanine/metabolism ; Point Mutation ; Precipitin Tests ; Protein Structure, Tertiary ; Recombinant Fusion Proteins/metabolism ; Research Support, Non-U.S. Gov't ; Sequence Homology, Amino Acid ; Serine Endopeptidases/*chemistry/genetics/isolation & purification/*metabolism ; Structure-Activity Relationship ; Transfection

Pilomatricoma is a benign adnexal tumor, differentiating towards elements of the hair matrix and shaft. The tumor typically presents as a solitary, deep-seated, firm nodule with overlying normal epidermis. Herein, we report three cases of pilomatricomas with the dermal bullae overlying tumors. Histopathological examination of the lesions showed marked lymphatic dilatations, edema, and attenuated collagen and elastic fibers above the pilomatricomas in the upper dermis. We diagnosed them as lymphangiectatic pilomatricomas.
Collagen ; Dermis ; Dilatation ; Edema ; Elastic Tissue ; Epidermis ; Hair ; Pilomatrixoma*