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*

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

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

Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus, which involves only the extensor surfaces of the hands, wrists and the distal part of forearms. Some subtypes of lichen myxedematosus including APPM have been reported with increased frequency in a patient with human immunodeficiency virus (HIV) infection. We describe a 31 year-old male patient with an HIV infection who presented with discrete, flesh-colored papules on the extensor surfaces of the wrists and the distal part of the forearms. Histopathologic findings revealed focal deposition of mucin in papillary and mid dermis that spared a small grenz zone. The lesions showed spontaneous regression after starting highly active antiretroviral therapy (HAART). We report a rare case of APPM in a patient with HIV infection which showed spontaneous regression with HAART, suggesting that APPM may represent one of the cutaneous markers of HIV infection.
Acquired Immunodeficiency Syndrome ; Adult ; Antiretroviral Therapy, Highly Active ; Dermis ; Forearm ; Hand ; HIV Infections* ; HIV* ; Humans ; Male ; Mucins ; Scleromyxedema* ; Wrist

The effect of anesthetic volume on the spread of hypobaric tetracaine were sutdied after intrathecal injection in thirty patients with prone jackknife and 15 degree Trendelenburg position for perinal surgery. The patients were assigned randomly into the one of three groups divided by the 3, 4, or 5 ml of volume of anesthetic solution. The results show that the volume of tetracaine in distilled water with hypobaric spinal anesthesia in prone jackknife and l5 degree Trendelenburg position had a important effect on the anesthetic dermatomal levels in spite of slightly rapid onset with large. volume. Therefore, we concluded that for the perianal surgery in prone jackknife position, as the volume of the anes- thetic solution with hypobaric spinal anesthesia, 3 or 4 ml of the volume are sufficient to get the adequate anesthetic levels.
Anesthesia, Spinal* ; Head-Down Tilt ; Humans ; Injections, Spinal ; Tetracaine ; Water

PURPOSE: To evaluate the clinical usefulness of a home-made histographic analysis system using a lung volume controller. MATERIALS AND METHODS:Our study involved ten healthy volunteers, ten emphysema patients, and two idio-pathic pulmonary fibrosis (IPF) patients. Using a home-made lung volume controller, images were obtained in the upper, middle, and lower lung zones at 70%, 50%, and 20% of vital capacity. Electron beam tomography was used and scanning parameters were single slice mode, 10-mm slice thickness, 0.4-second scan time, and 35 -cm field of view. Using a home-made semi-automated program, pulmonary parenchyma was isolated and a histogram then obtained. Seven histographic parameters, namely mean density (MD), density at maximal frequency (DMF), maximal ascending gradient (MAG), maximal ascending gradient density (MAGD), maxi-mal descending gradient (MDG), maximal descending gradient density (MDGD), and full width at half maxi-mum (FWHM) were derived from the histogram. We compared normal controls with abnormal groups includ-ing emphysema and IPF patients at the same respiration levels. RESULTS: A normal histographic zone with +/-1 standard deviation was obtained. Histographic curves of normal controls shifted toward the high density level, and the width of the normal zone increased as the level of inspi-ration decreased. In ten normal controls, MD, DMF, MAG, MAGD, MDG, MDGD, and FWHM readings at a 70% inspiration level were lower than those at 20% (p<0.05). At the same level of inspiration, histograms of emphysema patients were located at a lower density area than those of normal controls. As inspiration status decreased, histograms of emphysema patients showed diminished shift compared with those of normal con-trols. At 50% and 20% inspiration levels, the MD, DMF, and MAGD readings of emphysema patients were significantly lower than those of normal controls (p<0.05). Compared with those of normal controls, his-tograms of the two IPF patients obtained at three inspiration levels were located in an area of higher density. CONCLUSION: Using a home-made histographic analysis system which included a lung volume controller, pa-tients with diffuse parenchymal lung disease could be distinguished from normal controls. The method may be useful for the diagnosis and follow up of diffuse parenchymal lung diseases.
Diagnosis ; Emphysema ; Healthy Volunteers ; Humans ; Lung Diseases* ; Lung Diseases, Interstitial ; Lung* ; Pilot Projects* ; Pulmonary Emphysema ; Pulmonary Fibrosis ; Reading ; Respiration ; Tomography, X-Ray Computed ; Vital Capacity