[Developmental abnormalities of the eye].
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Biomedical subjects
Publications and source records attributed to S Shirai.
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A case of angiomyolipoma arising from the renal capsule is reported. A 49-year-old female was admitted to our hospital with a complaint of abdominal mass pointed out by ultrasonography during her yearly health check. The tumor was 10 cm in size. On abdominal enhanced CT, the tumor was existed behind the left kidney. The capsule of the tumor was enhanced and the septums were seen inside of the tumor. MRI revealed a hemorrhage in the tumor and selective renal angiography showed the tumor was fed by the renal capsular artery. A tumor of the renal capsule was suspected and a laparotomy was done. Pathological results by the frozen section suggested malignancy and radical nephrectomy was performed. Angiomyolipoma was diagnosed histologically. Tumors of the renal capsule are uncommon and angiomyolipoma of the renal capsule is extremely rare. We have found only two cases in Japanese medical literature.
To clarify the relationship between neural crest cells and ocular anomalies, pregnant mice were treated intraperitoneally with 12.5 mg/kg retinoic acid suspended in corn oil on day 7 of pregnancy (RA group). Control mice were given an equal volume of corn oil (control group). Each group consisted of 5 mother mice, and the offsprings were removed on day 18 of gestation. The fetal mortality was 46.3% in the RA group and 2.2% in the control group. Twenty-two live fetuses of the RA group and 45 of the control group were grossly observed, and the eyes were examined histologically. In the RA group, gross malformations such as microphthalmos (95.5%), cleft lip and palate (36.4%), and central nervous system anomalies (31.8%) were observed, and in the control group, malformations such as microphthalmos (6.7%), central nervous system anomalies (2.2%), and low set ears (2.2%) were seen. Histological examination revealed microphthalmos (47.7%), anophthalmos (38.6%), faulty closure of the embryonic fissure (36.4%), developmental abnormalities of the vitreous (34.1%), aphakia (22.7%), goniodysgenesis (18.2%), and faulty separation of the lens vesicle (15.9%) in the RA group. These anomalies arose from abnormal neural crest cell migration induced by retinoic acid. They were detected in only 3.3, 1.1, 3.3, 8.9, 1.1, 2.2 and 2.2%, respectively of the control group.
We evaluated four cases of persistent hyperplastic primary vitreous (PHPV) encountered at Nagoya City University Hospital in 1995. PHPV was seen unilaterally in three cases and bilaterally in one. The series comprised two males and two females, ranging in age from three to eight months, with an average of 4.8 months. Case 1 had a white strand running from the optic disc to the posterior surface of the lens in the left eye. Case 2 showed leukocoria in the right eye and central corneal opacity in the left. Magnetic resonance imaging (MRI) revealed total retinal detachment in both eyes. Case 3 exhibited retinal folds running from the optic disc to the posterior surface of the lens in the left eye. Case 4 showed elongation of the ciliary processes and leukocoria in the right eye. Ipsilateral total retinal detachment was seen in MRI. Three eyes of two cases were microphthalmic. Associated ocular anomalies included, posterior embryotoxon, sclerocornea, hypoplasia of the iris stroma and peripapillary staphyloma. There were accompanying systemic anomalies such as arachnoidal cyst, syndactyly, microcephalus, heart anomalies, pulmonary atresia and asplenia. Patients with PHPV should be carefully examined for the possible presence of other ocular and systemic anomalies caused by neural crest disorders.
Vascular endothelial growth factor (VEGF) has been investigated as a potent mediator of brain tumor angiogenesis, vascular permeability, and glioma growth. Using a VEGF ELISA, we determined the concentration of VEGF in the sera and tumor extracts of 19 brain tumor patients including glioblastoma, anaplastic astrocytoma, low grade astrocytoma, meningioma, malignant lymphoma, and metastatic brain tumor as well as normal brain. Although VEGF concentration of the serum was not correlated with that of the tissue, VEGF concentrations of glioblastoma cyst fluid were 200-300-fold higher than those of serum in the patients. VEGF concentration in the tumors was significantly correlated with the vascularity measured by counting vessels stained with von Willebrand factor antibody. VEGF protein localized to the cytoplasm of tumor cells and vasculature in gliomas, predominantly in the peripheral microvessel "hot spots" as well as around the necrosis in glioblastomas. VEGF immunopositivities were well reflected with VEGF concentration determined by ELISA. VEGF ELISA demonstrated time-dependent increase of the VEGF concentration in the serum-free conditioned medium of various glioma cell lines. The conditioned medium with high VEGF concentration induced endothelial cell migration. These observations suggest that VEGF represents a useful marker and measurable element of glioblastoma angiogenesis. The measurement of VEGF concentration by ELISA in tumor and tumor cyst fluid may allow for the assessment of vascularity in gliomas.
The diagnosis of tyrosinase-negative oculocutaneous albinism (OCA) was made in a 19-week-old fetus by skin biopsy. Because the parents had an 11-year-old son with tyrosinase-negative OCA, they requested that the fetus be aborted at the 20th week of gestation. A histological analysis of the eyes was performed. Throughout the retina, the ganglion cell layer was separated from the inner neuroblastic layer by the inner plexiform layer. However, the number of ganglion cells was decreased and the nerve fiber layer was immature. Bipolar and horizontal cells had begun to segregate into the inner nuclear layer. Rods and cones were identifiable in the posterior, but not peripheral, retina. Cones were more numerous in the center of the retina, and no rod-free area was identifiable. In addition, the ciliary body (epithelial folds, blood vessels in the mesodermal connective tissue core, and ciliary muscle) was less developed than in a normal fetus. Melanosomes in the retinal pigment epithelium only contained filaments without melanization and were therefore classified as stage I or II melanosomes. However, the ciliary epithelium also contained some stage III melanosomes with melanin adherent to the filaments.
A right-handed 48-year-old female had clustered groups of seizures characterized by speech disturbance (attacks of verbal repetition and speech arrest) over a period of 7 years. Later, speech arrest was accompanied by perioral focal seizures. Magnetic resonance imaging and surgery found a low grade astrocytoma, centered in the left supplementary motor area. Postoperatively, no seizures or speech disturbances occurred for 3 years. This case confirms the main function of the supplementary motor area of the dominant hemisphere is connected with the initiation of speech and the setting into motion of the mechanism of speech.
We examined glycosaminoglycan molecular species in the corneal stroma and the sclera histochemically in two cases of Peters' anomaly. Paraffin-embedded sections were stained with either hematoxylin-eosin or sensitized high iron diamine combined with enzyme digestion and then examined with a light microscope. In the center of the cornea, the histological specimens revealed defects of the corneal endothelium and Descemet's membrane, together with a local defect of the corneal stroma. Disorganized lens materials were detected in the corneal stroma. In the peripheral cornea, the undifferentiated iris adhered to the corneal posterior surface. Based upon the findings mentioned above, these two cases were diagnosed as having Peters' anomaly caused by faulty separation of the lens vesicle. The sclera showed normal structures in the specimen stained with hematoxylin-eosin. In both cases, the corneal stroma involved nearly equal amounts of chondroitin sulfate A/C and B. The sclera, on the other hand, contained a large amount of chondroitin sulfate A/C and a relatively small amount of chondroitin sulfate B. Keratan sulfate was never detected in either the cornea or the sclera. Thus, disorders of glycosaminoglycan molecular species were detected in the sclera as well as in the corneal stroma.
A 2-month-old female presented with nonrhegmatogenous retinal detachment in Dandy-Walker syndrome. At the fist examination, coloboma involving the optic disc in both eyes was detected. The left eye showed microphthalmos with sclerocornea, persistent pupillary membrane, hypoplasia of the iris stroma, and bullous retinal detachment near the optic disc. Chromosomal analysis revealed a mosaic pattern: 46, XX/47, XXX. Increased intracranial pressure associated with Dandy-Walker syndrome was detected by a neurosurgeon at the age of 3 months. The patient was followed for several weeks, and then nonrhegmatogenous retinal detachment appeared in the right eye. Subretinal fluid alternately increased and decreased in both eyes. A ventriculo-peritoneal shunt was performed at the age of 6 months, and the retinal detachment was remarkably reduced in both eyes after lowering of intracranial pressure. Coloboma involving the optic disc, sclerocornea, persistent pupillary membrane, hypoplasia of iris stroma, and Dandy-Walker syndrome were thought to be caused by the abnormal development of neural crest cells. We surmised that the retinal detachment in this case might have resulted from a communicating pathway between the subarachnoid space and the subretinal space. We concluded that the etiology of retinal detachment associated with optic disc anomaly should be investigated to determine adequate treatment.
BACKGROUND: Detection of differences in color is necessary for an automatic diagnostic system for skin lesions. OBJECTIVE: The purpose of this study was to examine whether color analysis of an image obtained with a videomicroscope can be used as a key to distinguish basic skin lesions. METHODS: We carried out quantitative color analyses of nine kinds of natural or artificial skin lesions. After images of the lesion and adjacent normal skin were obtained, the difference in the mean quasiabsorbance (logarithm of the inverse reflectance) values between the two were examined in each color band of red, green, and blue by an image analytic method. RESULTS: We could determine a set of thresholds of quasiabsorbance values to distinguish between basic lesion colors. On the basis of these results we developed a rudimentary system that automatically offers possible diagnoses of a test site. CONCLUSION: The algorithm that we developed may be of value in an initial screening of skin lesions in an automatic diagnosis system.
Diffusion-weighted magnetic resonance imaging was performed to determine the changes in water diffusion and to investigate the detectability of diffusion anisotropy in patients with intracranial disorders. Diffusion maps of the apparent diffusion coefficient (ADC) were created of 19 patients with cerebral infarction, five with intracerebral hematoma, four with glioma, four with meningioma, four with hydrocephalus, and five with subdural hematoma. ADC was increased in chronic cerebral infarction and glioma, and decreased in acute cerebral infarction, meningioma, and the marginal area of glioma compared with the ADC of the normal gray matter. There was a significant difference in ADC between the marginal and internal areas of glioma. Increased ADC may be due to increased vasogenic edema in infarction and a lack of significant restriction of diffusion within glioma. Decreased ADC can be attributed to restricted diffusion caused by cytotoxic edema in infarction and the underlying histological pattern of densely packed tumor cells in glioma. Diffusion anisotropy of the internal capsule was less detectable in pathological than normal hemispheres. Diffusion anisotropy was less detectable in patients with hydrocephalus and subdural hematoma. Intracranial lesions were thought to have influenced the compression of the brain structures and cells, resulting in decreased diffusion. The measurement of ADC by diffusion-weighted magnetic resonance imaging has the potential for greater understanding of the biophysical changes in various intracranial disorders, including correct diagnosis of cerebral infraction, and histological diagnosis of brain tumor.
A postmarketing surveillance of cefminox sodium (Meicelin, CMNX) for intravenous injection was conducted for about 4 years from August 1987 through June 1991, and 13,431 patients were followed up to evaluate the safety of the drug. The incidence of side-effects was 1.76%. By organ, the most frequently observed were hepatic and of the bile duct system (0.87%) followed by those on leukocytes and reticuloendothelial system (0.24%), skin and adnexa (0.24%) and digestive tract (0.16%) indicating a tendency similar to that of other injectable cephalosporins. The incidence of the side-effects among elderly patients (65 years old or older) was 2.12%, whereas among patients 64 years old or younger it was 1.58% with no significant differences between the two groups. No side-effects specific to the elderly were observed. Among children 15 years old or younger the incidence was 0.59%, which was lower than that for patients 16 years old or older (1.90%). Potential side-effects on pregnant women (n = 101) and their babies were also checked. No side-effects occurred among the 52 pregnant women evaluated and no abnormalities were detected in their babies who were followed up for up to 4 years. Cefminox is thus considered to be a highly safe cephalosporin antibiotic.
Histological and histochemical studies were carried out in Jcl:ICR mice to determine the changes in microscopic structures and glycosaminoglycan molecular species in the tissues involved in normal or faulty closure of the embryonic fissure. The purpose of this study was to elucidate the mechanism underlying the faulty closure of the embryonic fissure and to identify the key substances involved in normal and faulty closure. To obtain mice with an appropriate faulty closure of the embryonic fissure, ochratoxin A was employed as a teratogenic agent. Serial sections from tissues undergoing normal and faulty closure of the embryonic fissure were cut at a right angle to the fissure. As the staining procedures, a hematoxylin-eosin procedure and a sensitized high iron diamine method were used. A chemical modification (nitrous acid treatment) or an enzyme digestion procedure (chondroitinase ABC digestion procedure) was employed in combination with the sensitized high iron diamine method to identify the glycosaminoglycan molecular species in the tissues. The results obtained in the present study have substantiated the histophysiological importance of glycosaminoglycan molecular species during the course of histogenesis in the normal and the faulty closure of the embryonic fissure of developing murine eyes.
To examine whether it is possible to evaluate the degree of ultraviolet B (UVB)-induced inflammation by measuring the degree of hyperpigmentation, we investigated the relationship between UVB-induced erythema and the subsequent pigmentation quantitatively. At 24 h and 7 d after irradiation with erythemogenic doses of UVB to the backs of 16 Japanese subjects, the degree of induced erythema (delta erythema index) and that of pigmentation (delta melanin index) were examined by an image analytic method using a videomicroscope interfaced with a computer. The relationship between two indices was linear in each subject, and the correlation coefficient was 0.83 when evaluated using whole data. The slope of the regression line for the delta melanin index against delta erythema index tended to become steeper as non-irradiated skin color became darker (r = 0.63), suggesting that more efficient melanogenesis takes place after the same level of inflammation in the subject with darker skin. Both erythema and hyperpigmentation were suppressed significantly and in a parallel manner by corticosteroids and indomethacin applied topically immediately after UVB irradiation. These results imply that the post-inflammatory hyperpigmentation correlates closely with the severity of the prior inflammation and that chemical mediators released in the inflammatory process have considerable influence on the melanogenesis. We conclude that the measurement of UVB-induced hyperpigmentation can be utilized for the assessment of topical anti-inflammatory agents, unless these have direct actions on the tyrosinase activity of melanocytes.
We report a method for quantitative analysis of erythema and pigmentation using a videomicroscope interfaced with a computer. The analysis was carried out by examining the brightness intensity of every picture element, composed of an image picked up from each band of red, green, and blue, and by deriving the quasi-absorbance value (absorbance index) from the mean brightness for each band. In assessments of UV-induced erythema and tanning, excellent linear correlations were found between the results obtained with our system and those with a narrow-band reflectance spectrometer. Moreover, the absorbance indices of haemoglobin and melanin solutions showed linear relationships with their concentrations in in vitro examination. As the monitored picture becomes out of focus if incorrect pressure is exerted on the skin, and as regions of interest can be chosen from a magnified image, this system offers excellent interobserver reproducibility, and is suitable for the evaluation of erythema or pigmented lesions which are too small or irregular to quantify by conventional methods such as colorimetry.
A 64-year-old female presented with hypertensive thalamic hemorrhage concurrent with subarachnoid hemorrhage (SAH) due to a ruptured aneurysm manifesting as sudden onset of right hemiparesis followed by severe headache. The aneurysm was located in the basilar artery at the origin of the superior cerebellar artery, remote from the thalamic hematoma. The aneurysm was clipped 3 weeks after SAH. She was discharged with slight right hemiparesis. The method and timing of surgery for such patients depend on hematoma size, location of the aneurysm and hematoma, and neurological status. The intracerebral hemorrhage remote from the ruptured aneurysm should be treated initially if necessary, and the aneurysm clipped after the brain swelling has reduced.
In order to clarify the ultrastructural consequence of the VX2 cancer cell exposed to underwater shock waves. The VX2 cancer implanted to the urinary bladder of rabbit was received the shock waves (1000 shots, 100 MPa, 5/sec.) generated by the piezo-ceramics. Damage to the cancer cell were manifested by the three kind of findings: the one was the cell junction failure which caused the isolated cells; the second was the destruction of organelle which seemed to form the vacuole in cytoplasm; and the last finding was the emergence of moth eaten appearance in the exposed cell. We conclude that underwater shock wave exposure induces lethal injury in VX2 implanted cancer.