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Biomedical subjects

H Arimura

Publications and source records attributed to H Arimura.

At least 19 recordsLinked to original sources

SET binding factor 2 (SBF2) mutation causes CMT4B with juvenile onset glaucoma.

The authors report a Japanese family segregating autosomal recessive Charcot-Marie-Tooth disease (CMT) with focally folded myelin, juvenile-onset glaucoma, and a nonsense mutation of SET binding factor 2 (SBF2). The consistent phenotypic features associated with SBF2 mutations are early-onset demyelinating neuropathy, myelin folding, and markedly decreased motor nerve conduction velocities; glaucoma associates with SBF2 nonsense mutations.

Adolescent↗

[Development of computerized method for automated classification of the body parts in digital radiographs].

PURPOSE: In picture archiving and communication systems (PACS), the information on the body parts included in radiographs is often not or incorrectly recorded in an image header. In order to apply the computer-aided diagnosis (CAD) system in the PACS environment, the body parts in radiographs need to be recognized correctly by computer. The purpose of this study is to develop a computerized method for correctly classifying the body parts in digital radiographs based on a template matching technique. METHODS/MATERIALS: The image database used in this study was 1032 digital radiographs (14 x 17 inches) obtained with a computed radiography, and included 505 chest of postetroanterior view, 39 chest of lateral view, 241 abdomen, 108 pelvis, 10 upper limbs, 125 lower limbs, and 4 thoracic spine. In this method, test images were classified into four body parts, i.e., (1) chest, (2) abdomen, (3) pelvis, and (4) upper/lower limbs and thoracic spine. This computerized method was tested with 852 images, since 180 images were employed for creation of 98 templates, which represented the average radiographs for various body parts. Our approach was to examine the similarity of a given test image with templates by use of the cross-correlation values as the similarity measures. The body part of the test image was identified as the body part in the template yielding the maximum correlation value. Our method consisted of the following five steps. First, test images were classified into one of three groups; i.e. 1) chest and abdomen, 2) pelvis, and 3) upper/lower limbs and thoracic spine by using the templates obtained from images with the average size and position. Second, the remaining uncertain images were classified by using additional templates in various directions. Third, the chest and abdomen group was separated into two subgroups; i.e.chest and abdomen. Fourth, in order to classify some uncertain images, templates were shifted horizontally and vertically. Fifth, outer pixels of templates were eliminated to avoid the misclassification due to x-ray collimation. RESULTS: Our preliminary results indicated that the body parts for 850 cases (99.8%) were correctly classified with our method. CONCLUSIONS: This method would be useful for automated identification of the body parts in radiographs when various CAD systems would be implemented in the PACS environment.

Diagnosis, Computer-Assisted↗

Expression and purification of cytokine receptor homology domain of human granulocyte-colony-stimulating factor receptor fusion protein in Escherichia coli.

Direct expression of the cytokine receptor homology (CRH) domain of granulocyte-colony-stimulating factor (G-CSF) receptor is lethal to Escherichia coli. For the efficient and stable production of an active CRH domain in E. coli, we fused the CRH domain with different proteins, such as maltose-binding protein (MalE), glutathione S-transferase, and thioredoxin (Trx). Among these, Trx appeared to be the best in terms of the protein expression level, purification efficiency by affinity chromatography, and binding activity to its ligand, G-CSF. The yield of active Trx-CRH fusion protein increased about 200-fold compared to that of previously reported MalE-CRH fusion.

ATP-Binding Cassette Transporters↗

Hereditary motor and sensory neuropathy with myelin folding and juvenile onset glaucoma.

OBJECTIVE: We describe three patients from a family with motor and sensory neuropathy accompanied by open-angle glaucoma. BACKGROUND: Autosomal recessive demyelinating hereditary motor and sensory neuropathies (HMSN) include different disorders. To our knowledge, autosomal recessive HMSN has not been associated with juvenile onset glaucoma. METHODS: Sural nerve pathology of the three patients were examined, and genetic analysis of the family was performed. RESULT: - The most prominent pathologic finding was a highly unusual myelin abnormality consisting of irregular redundant loops and folding of the myelin sheath. The family survey supports autosomal recessive inheritance. The molecular analysis failed to demonstrate either linkage of the disease to MPZ gene, PMP22 gene, Cx32 gene, orEGR2 gene. Analysis did not establish linkage of the disease to the locus of CMT4A, 4B, and 4C genes. CONCLUSION: The present cases may represent a new type of HMSN accompanied by juvenile onset glaucoma.

Adult↗

Proportionality between Wiener spectra of quantum mottle and the squares of modulation transfer functions.

Rossmann proposed that the Wiener spectra of the quantum mottle of radiographs made using screen-film systems were proportional to the squares of the modulation transfer functions (MTFs) of the screen-film systems. On the other hand, Lubberts theoretically pointed out that the shape of the Wiener spectrum of the quantum mottle depended on the sum of the squares of the MTFs for different depths in the screen phosphor layer, rather than the square of the sum of the MTFs for the different depths, i.e. the square of the MTF of the screen-film systems. The purpose of this study is to experimentally investigate the proportionality between the Wiener spectra of the quantum mottle and the squares of the MTFs of screen-film systems using two screen-film systems having different screen thicknesses. For this purpose, we determined correction factors for the square of the MTF of the screen-film system in the Wiener spectrum of the quantum mottle at each spatial frequency when the Wiener spectral values of the screen mottle were separated into those of the quantum mottle and structure mottle. The correction factor is the ratio of the normalized Wiener spectrum of the quantum mottle to the square of the MTF of the screen-film system. As a result, for a thin screen, the correction factors were unity for all spatial frequencies; on the contrary, for a thick screen, the factor increased with spatial frequency. By calculating the theoretical correction factors using the models for the MTF and Wiener spectrum of the quantum mottle of Nishikawa and Yaffe based on Lubberts' theory, we verified that our experimental results agreed with Lubberts' theory. Furthermore, by obtaining the screen thickness dependence of the theoretical correction factors for the two screens, we showed that, for screens thinner than 0.02 mm, Rossmann's theory can be applied to the relationship between the Wiener spectrum of the quantum mottle and the MTF of the screen-film system, whereas for screens thicker than 0.02 mm, Lubberts' theory should be applied.

Biophysical Phenomena↗

[A family with probable autosomal dominant bulbospinal muscular atrophy with gynecomastia].

We reported a 52-year-old man and his family with bulbospinal muscle atrophy (BSMA) and gynecomastia. The propositus presented with the clinical picture of late onset progressive bulbospinal muscular atrophy including postural tremor, general hyporeflexia, mild maturity onset diabetes, gynecomastia and sexual impotence. One of his brother and his two sons had gynecomastia. His elder son had ocular movement abnormality, associated movement of facial muscle and finger tremor. One of his brothers showed tongue fasciculation without gynecomastia. None of members examined had abnormal expansion of CAG repeats in the androgen receptor gene. We speculate that this family has a new clinical entity characterized by bulbospinal muscular atrophy with an autosomal dominant inheritance.

Aged↗

Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome.

Crow-Fukase or POEMS syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes is a rare multisystem disorder of obscure pathogenesis that is associated with microangiopathy, neovascularization, and accelerated vasopermeability. We examined the levels of the vascular endothelial growth factor/vascular permeability factor (VEGF) in the serum and cerebrospinal fluid (CSF) from 10 patients with this syndrome. Serum VEGF levels were about 15-30 times those in control subjects or patients with Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), and other neurological disorders. The CSF VEGF levels, however, were similar to those found in GBS and CIDP. Elevated VEGF levels in the serum decreased in 7 patients with Crow-Fukase syndrome after conventional therapy. The principal isoform of VEGF in Crow-Fukase syndrome was VEGF165. Elevated VEGF was independent of M-protein. Our results suggest that the overproduction of VEGF is important in the pathogenesis of this disorder.

Adult↗

Relation between radiographic mottle for double and single emulsions.

The radiographic density fluctuations produced by using dual screen-film systems are designated as the radiographic mottle. The density fluctuation of the radiographic mottle for the double emulsions at a density of the double emulsions consists of those for the front and back emulsions on a radiograph. However, the relation between the Wiener spectra of the radiographic mottle for the double and single emulsions had not been studied. Hence we compared the Wiener spectra of the radiographic mottle for the double emulsions with the sum of those for the front and back emulsions on the same radiographs and with the sum of those for the emulsions at the same densities. At all densities of more than 0.62 for lower spatial frequencies (< or = 1 mm-1), the Wiener spectral values of the radiographic mottle for the double emulsions were greater than the sum of those for the front and back emulsions for both comparisons on the same radiographs and at the same densities. In order to investigate the reason of the above phenomena, we separated the Wiener spectral values of the radiographic mottle for various densities into those of the three factors, i.e., quantum mottle, structure mottle, and film granularity, and performed the same comparisons as the radiographic mottle. Also, to explain the results for the three factors, we obtained the Wiener spectral values of the spatial fluctuations of the light exposure or the fluorescence intensity and the gradients of the characteristic curves of the film for the double and single emulsions of the x-ray film. As a result of the investigation, we found that the phenomena on the radiographic mottle were caused by that (1) on the same radiographs the squares of the gradients of the characteristic curves for the double emulsions were about 5.3 times as great as those for the single emulsion at densities of more than 0.62 of the double emulsions, and (2) at the same density of more than 0.62 those were more than about 2.2 times as great as those for the single emulsion.

Biophysical Phenomena↗

Angiosarcoma of the heart presenting as fatal pulmonary hemorrhage.

A 47-year-old man died from fatal pulmonary hemorrhage. Cardiac angiosarcoma with lung metastases was found at postmortem examination. His chest radiograph showed bilateral, diffuse nodular infiltrates without cardiomegaly. No cardiac signs and symptoms were observed. The clinical outcome was rapidly fatal. Angiosarcoma of the heart should be suspected in patients with hemoptysis and nodular chest radiograph abnormalities, even in the absence of cardiac signs and symptoms.

Fatal Outcome↗

[Two cases of POEMS syndrome with increased vascular endothelial growth factor (VEGF)].

We report a greatly increased amount of vascular endothelial growth factor (VEGF) in sera of two cases with POEMS syndrome. They had solitary myelomas in the cervical (case 1) and lumbar vertebrae (case 2). In both cases, serum VEGF decreased after steroid therapy. Sural nerve biopsy in case 1 showed marked subperineurial edema. The pathomechanism of polyneuropathy in POEMS syndrome may be due to involvement of the blood-nerve barrier by way of increased microvascular permeability induced by VEGF. VEGF is therefore not only a useful diagnostic marker for POEMS syndrome, but may also be a marker of clinical improvement.

Adult↗

Development of an integrated radiotherapy network system.

PURPOSE: To introduce the process of developing an integrated radiotherapy network. METHODS AND MATERIALS: We developed a new radiotherapy treatment-planning system in 1987 that we named the Computer Tomography (CT) simulator. CT images were immediately transported to multiimage monitors and to a planning computer, and treatment planning could be performed with the patient lying on the CT couch. The results of planning were used to guide a laser projector, and radiation fields were projected onto the skin of the patient. Since 1991, an integrated radiotherapy network system has been developed, which consists of a picture archiving and communicating system (PACS), a radiotherapy information database, a CT simulator, and a linear accelerator with a multileaf collimator. RESULTS: Clinical experience has been accumulated in more than 1,000 patients. Based on our 7 years of experience, we have modified several components of our original CT simulator and have developed a second generation CT simulator. A standard protocol has been developed for communication between the CT scanner, treatment planning computer, and radiotherapy apparatus using the Ethernet network. As a result, treatment planning data can be transported to the linear accelerator within 1 min after completion of treatment planning. CONCLUSION: This system enables us to make optimal use of CT information and to devise accurate three-dimensional (3D) treatment-planning programs. Our network also allows for the performance of fully computer-controlled dynamic arc conformal therapy.

Computer Communication Networks↗

Serum concentrations of IL-5, GM-CSF, and IL-3 and the production by lymphocytes in various eosinophilia.

The concentrations of interleukin-5 (IL-5), granulocyte-macrophage colony-stimulating factor GM-CSF, and interleukin-3 (IL-3) in serum and in IL-2-stimulated lymphocyte culture medium (L-IL2-CM) prepared from patients with reactive eosinophilia were measured by enzyme-linked immunosorbent assay (ELISA). Serum IL-5 levels were increased in 16 out of 42 cases. GM-CSF and IL-3 were below the detectable levels in all sera examined. The concentrations of IL-5 and GM-CSF in L-IL2-CM were increased in 10 out of 29 patients. IL-3 was below the detectable levels in all L-IL2-CM.

Angioedema↗

Immunological abnormality in patients with lysinuric protein intolerance.

Lysinuric protein intolerance (LPI) is a rare hereditary disorder manifesting hyperammonemia induced by low levels of basic amino acids, these low levels being due to the impaired transport of these acids in the intestinal mucosa and the renal tubules. Low serum arginine levels and probably the consequently low in vivo levels of nitric oxide (NO), which against acts as a physiological and immunological mediator/modulator, are thought to influence the immunological status in patients with LPI. Accordingly, this study was conducted to. We found that patients with LPI had leukocytopenia, high serum IgG levels, a high ratio of CD44B4-positive lymphocytes (helper inducer) to CD42H4-positive lymphocytes (suppressor inducer), low levels of leukocyte phagocytic, cytotoxic, and natural killer cell activity, and increased spontaneous proliferation of lymphocytes. These results were probably the consequence of persistent low NO levels in vivo.

Adult↗

A hemophiliac with human immunodeficiency virus (HIV)-1-associated dementia complex.

We report a 29-year-old male hemophiliac with human immunodeficiency virus (HIV)-1-associated dementia complex, who died 2.5 months after the onset of dementia. The patient's cognitive abnormalities including forgetfulness, loss of concentration and slowing of thought appeared about 7 years after HIV infection. His neurological symptoms were characterized as progressive dementia, episodic consciousness loss, transverse myelopathy and peripheral neuropathy. He had generalized slow waves in electroencephalogram (EEG), progressive cerebral atrophy and a diffuse high intensity lesion in the white matter as shown by T2-weighted brain magnetic resonance imaging (MRI). We emphasize the significance of neurological complications, especially acute progressive dementia, in Japanese patients with acquired immunodeficiency syndrome (AIDS).

AIDS Dementia Complex↗

Concurrent use of granulocyte colony-stimulating factor with low-dose cytosine arabinoside and aclarubicin for previously treated acute myelogenous leukemia: a pilot study.

We used a new chemotherapy regimen for the treatment of 18 consecutive patients with relapsed AML (median age 44 years, range 18-74). The regimen consisted of low-dose cytosine arabinoside (10 mg/m2/12 h, usually day 1 to 14), low-dose aclarubicin (10-14 mg/m2/day, day 1 to 4), and concurrent use of G-CSF (200 micrograms/m2/day) (CAG regimen). Overall, 15/18 patients (83%) achieved complete remission (CR) after one or two courses, including eight out of ten refractory patients with early relapse, second or subsequent relapses, and/or resistant relapse. Two of three patients who relapsed, achieved CR again after reinduction with a modified CAG regimen. Fourteen of the 15 complete remitters received consolidation therapy with the CAG regimen modified, followed by oral busulfan in eight cases, and by allogeneic bone marrow transplantation in two cases. At a median follow-up of 12 months, median CR duration and survival were 6 months and 17 months, respectively. Myelosuppression in the first course of induction therapy was moderate to severe. However, severe non-hematologic toxicity (WHO grade > or = 3) was characteristically rare. Although this is a preliminary study, the CAG combination seems promising for the treatment of relapsed AML, with its low toxicity contributing to a higher quality of life for the patient.

Aclarubicin↗