Search PubMed⌕ Search

Biomedical subjects

H Terasaki

Publications and source records attributed to H Terasaki.

At least 37 records · Page 2Linked to original sources
PubMed2001

Primary synovial sarcoma of the lung: a case report confirmed by molecular detection of SYT-SSX fusion gene transcripts.

We report a rare case of primary synovial sarcoma of the lung. The patient was a 49-year-old woman who presented with a well-defined oval-shaped mass in the left upper lobe on a chest radiograph. A malignant pulmonary tumor was suspected and consequently a left upper lobectomy was performed. Grossly, the tumor measured 5 x 4 cm, was whitish-yellow in color and soft in consistency. Histologically, the tumor showed a dense proliferation of short spindle cells, partly arranged in interlacing fascicles. In some areas a hemangiopericytoma-like pattern, stromal myxoid change and necrosis of various sizes were noted. Numerous mitotic figures were also seen. Immunohistochemically, the tumor cells were positive for epithelial markers such as cytokeratin and epithelial membrane antigen. As these features suggested a monophasic fibrous type of synovial sarcoma, we examined for the presence of SYT-SSXfusion gene transcripts using RNA samples from the frozen tumor tissue. A reverse transcription polymerase chain reaction amplified a single 583-base pair fragment characteristic of synovial sarcoma. As no other tumorous lesions were found during a follow-up period of 1 year, primary synovial sarcoma of the lung was our final diagnosis. This tumor should be considered in the differential diagnosis of round to short spindle cell tumors arising in the lung.

Female↗
PubMed2001

Histopathologic findings of cornea verticillata in a woman heterozygous for Fabry's disease.

PURPOSE: To report the histopathologic findings of the cornea verticillata observed in a woman who was heterozygous for Fabry's disease. METHOD: A 67-year-old woman was found to have a whorl-like corneal opacity on her visit to the Department of Ophthalmology. Aichi Saiseikai Hospital. Her visit was because of a sudden loss of vision in her right eye owing to a central retinal artery occlusion in association with an ophthalmic artery occlusion. The patient died suddenly of an acute heart failure; with family consent, an autopsy was performed and the right eye was removed for histopathologic examination by light and electron microscopy. RESULTS: Low levels of alpha-galactosidase in the leukocytes together with the corneal finding led to the diagnosis of heterozygous Fabry's disease. Light microscopy revealed a 0.3- to 0.5-microm thick layer between the epithelial and Bowman's layers. Oil red O positive deposits were accumulated in the subepithelial layer, and the density varied in different regions. Electron microscopy showed that subepithelial layer differed in thickness, and the basal lamina reduplicated regionally. We were not able to determine the structure that correlated with the "ridge" in the central part of the cornea. CONCLUSION: The oil red O positive deposits and their variation in density in the subepithelial area of the cornea may have caused the characteristic whorl-like corneal opacity in this woman who was heterozygous for Fabry's disease.

Aged↗
PubMed2001

Focal macular ERGs in eyes after removal of macular ILM during macular hole surgery.

PURPOSE: The removal of the internal limiting membrane (ILM) for traction maculopathy has recently been advocated. However, it is generally believed that the ILM plays an important role in retinal function, because it is the basal lamina of the Müller cells that are involved in the generation of the electroretinogram (ERG) b-wave. To date, there has been no objective assessment of retinal function on removing the ILM. In this study, the changes of each component of the focal macular electroretinograms (FMERGs) were investigated in eyes before and after the ILM was removed in the macular area during surgery for idiopathic macular holes (IMHs). METHODS: FMERGs were elicited by a 15 degrees stimulus centered on the fovea and monitored by an infrared fundus camera. FMERGs were recorded from 49 eyes of 48 patients with IMHs before and 6 weeks after anatomically successful macular hole surgery. Whether an eye had or did not have the ILM removed was randomly determined. The ILM was removed in 30 eyes (ILM-off group) and was not removed in 19 eyes (ILM-on group). Six months after surgery, the same examination was performed in 27 eyes of the ILM-off group and in 15 eyes of the ILM-on group. The amplitudes and implicit times of the a- and b-waves and the mean amplitudes and implicit times of the first three oscillatory potentials (OP1 to OP3) were compared before and after surgery within and between the groups. RESULTS: Visual acuity increased significantly after surgery in both groups. In the ILM-on group, the amplitude of the a- and b-waves and the OPs increased significantly 6 months after surgery (P: = 0.0093, P: = 0.0019, P: = 0.0024, respectively, paired t-test). In the ILM-off group, the a-wave amplitude and mean OP amplitudes were significantly larger 6 months after surgery (P: = 0.0077, P: = 0.0030, respectively, paired t-test). The b-wave amplitude, however, did not change significantly. The percentage increase in the b-wave amplitude 6 months after surgery was significantly higher in the ILM-on group (44.0%) than in the ILM-off group (15.0%; P: = 0.037, t-test). CONCLUSIONS: The removal of the ILM had no adverse effect on visual acuity. However, the selective delay of recovery of the FMERG b-wave 6 months after surgery suggests an alteration of retinal physiology in the macular region.

Aged↗
PubMed2001

[Anesthetic management of a patient with pheochromocytoma and cerebral ischemic attacks].

A 15-year-old girl with history of two cerebral ischemic attacks possibly caused by cerebral vascular spasm was diagnosed as having a pheochromocytoma, and was scheduled for laparoscopic removal of the adrenal tumor. Epidural catheterization was performed at Th 12/L 1. General anesthesia was induced with thiamylal and vecuronium, and maintained with nitrous oxide-oxygen-isoflurane and continuous epidural infusion of 1.5% lidocaine. Phentolamine and thiamylal were continuously administrated into the vein. While operating on the tumor, abnormal hypertension did not occur. Just after removal of the tumor, the systolic blood pressure fell to 50-70 mmHg. The hypotension continued for about 75 min, despite administration of dopamine, norepinephrine and epinephrine. After the emergence from anesthesia, the blood pressure recovered to normal level. No neurocerebral abnormality was found. In patients like this one, we have to prevent cerebral vascular spasm and cerebral infarction caused by excessive secretion of catecholamines.

Adolescent↗
PubMed2001

Multifocal ERG findings in complete type congenital stationary night blindness.

PURPOSE: To study the multifocal electroretinogram (mfERG) in patients with the complete type of congenital stationary night blindness (cCSNB), which is thought to be due to a defect in neurotransmission from the photoreceptors to the ON-bipolar cells. METHODS: mfERGs were recorded with the VERIS recording system from four patients with cCSNB, none of whom had nystagmus. The stimulus array consisted of 61 hexagons, and the total recording time was approximately 4 minutes. The amplitudes and implicit times of the first- and second-order kernels of the local responses were compared with those from 20 myopic controls. Waveforms of the summed response from all locations were also compared between the two groups. RESULTS: The first-order kernels of the mfERGs of cCSNB patients had normal amplitudes but delayed implicit times for nearly the whole field tested. The second-order kernel was severely attenuated in amplitude in cCSNB patients. The ratios of the second- to first-order kernel amplitudes were significantly reduced in cCSNB and clearly separated the cCSNB group from the control group without any overlap of the values. CONCLUSIONS: The second-order kernel, which is involved in adaptative mechanism of the retina to repeated flashes, is selectively reduced in cCSNB. The delay of the implicit times of the first-order kernel in patients with cCSNB may be related to the severe amplitude reduction of the second-order kernel.

Adolescent↗
PubMed2001

Novel CACNA1F mutations in Japanese patients with incomplete congenital stationary night blindness.

PURPOSE: Although it was reported that congenital stationary night blindness (CSNB) could be divided into complete and incomplete CSNB clinically in 1986, it was not until 1998 that the two types were found to be distinct clinical diseases by molecular genetic analysis. The purpose of this article is to report mutations in the retina-specific calcium channel alpha1-subunit gene (CACNA1F) in Japanese patients with incomplete CSNB and to describe the clinical features of these patients. METHODS: Seven patients from five separate Japanese families with incomplete CSNB were examined. Genomic DNA was extracted from leukocytes of the peripheral blood, and all 48 exons of the CACNA1F were amplified by polymerase chain reaction and directly sequenced. A complete ophthalmic examination was performed, including best corrected visual acuity, slit lamp and fundus examinations, fundus photography, and electroretinography. RESULTS: A mutation in the CACNA1F was identified in all the patients. The identified mutations were a missense mutation (Gly609Asp); a nonsense mutation (Arg913stop); a splice donor site mutation of G to C at nucleotide 2571+1; a G insertion at nucleotide 709, resulting in a frame shift with a predicted stop codon at codon 247; and a 4-bp deletion at nucleotides 271 to 274, with a replacement by an abnormal 34-bp sequence. Clinically, each patient had essentially normal fundi, mildly reduced corrected visual acuity, and slight myopia or hyperopia with astigmatism. Electrophysiologically, the mixed rod-cone ERG showed a negative configuration with recordable oscillatory potentials. The rod ERG was recordable but subnormal, and the cone and 30-Hz flicker ERGs were markedly depressed. CONCLUSIONS: Five novel mutations were identified in the CACNA1F in five Japanese families with incomplete CSNB, leading to the conclusion that in most Japanese patients, incomplete CSNB is caused by a CACNA1F mutation.

Adolescent↗
PubMed2001

Rescue of retinal function by macular translocation surgery in age-related macular degeneration and other diseases with subfoveal choroidal neovascularization.

Age-related macular degeneration (AMD) is the leading cause of legal blindness among the elderly. In AMD and some other macular diseases, subfoveal choroidal neovascularization (CNV) damages the underlying retinal pigment epithelium (RPE), and because retinal function is dependent on a healthy RPE, vision is markedly reduced by a subfoveal CNV. To treat such CNVs, macular translocation surgery has been performed to move the sensory retina from the damaged RPE to healthier RPE. At present, this surgery is the only possible treatment to improve the visual acuity of patients with subfoveal CNV. Macular translocation surgery involves the detachment of the entire retina from the RPE by a subretinal infusion of fluid and creating a 360 degrees circumferential retinotomy followed by the rotation of the retina. Severe postoperative complications such as recurrent retinal detachment have been reported in about 30% of the cases after macular translocation. To determine the efficacy of this surgery, it is necessary to demonstrate an improvement in macular and overall retinal function objectively as well as subjectively. To this end, we have assessed the changes in visual function by measuring the visual acuity subjectively, and the macular function objectively by focal macular ERGs (FERGs). We shall show that there is an improvement in the FERGs in most patients after retinal translocation surgery but the full-field ERGs were reduced by about 30%. Thus, macular translocation surgery with 360-degree retinotomy may be feasible for macular function, although some degree of peripheral retinal function is lost.

Aging↗
PubMed2001

[Propofol-fentanyl anesthesia for a 13-year-old patient with Beckwith-Wiedemann syndrome].

We report anesthetic management of a 13-year-old boy with Beckwith-Wiedemann syndrome for urethral fistulectomy using propofol-fentanyl anesthesia. The syndrome has three major symptoms; exomphalos, macroglossia and gigantism. Other complications including neonatal hypoglycemia, visceromegaly, metabolic disorders, malignant tumors, and cardiovascular abnormalities may also be seen. This patient was complicated with gigantism, exomphalos, hypospadias, cryptorchidism, hepatoblastoma, accessory mammae, and left axis deviation with left anterior fascicular block. A comprehensive preoperative examination should include assessment of the airway, cardiovascular conditions, endocrine status, blood glucose, and electrolytes. In this patient, airway management was easy by laryngeal mask airway and the anesthetic course was uneventful.

Adolescent↗
PubMed2000

Structure, expression, and chromosomal localization of the human gene encoding a germinal center-associated nuclear protein (GANP) that associates with MCM3 involved in the initiation of DNA replication.

A 210kDa protein named GANP is upregulated in germinal center (GC)-B cells in the spleen of antigen-immunized mouse. We studied a human ganp gene (hganp) encoding a putative polypeptide of 1980 amino acids. The carboxyl-terminal 721-amino-acid sequence of hGANP is identical to Map80, that is presumably generated by alternative splicing of hganp/Map80 gene. The genomic segment carrying hganp and Map80 genes was isolated, and the chromosomal location was determined on 21q22.3. Northern blot analysis with RNAs from various organs demonstrated a single band of 7kb hganp mRNA, which suggests a preferential transcription of hganp gene from the hganp/Map80 locus. The hGANP expression was upregulated in GCs of the tonsil, as demonstrated by in-situ RNA hybridization and immunohistochemical analyses. The hGANP, with the domain (Map-box) capable of binding to MCM3 in B cells, might be involved in regulation of cell-cycle progression and DNA replication of GC-B cells.

Acetyltransferases↗
PubMed2000

Mother and daughter with 45,X/46,X,r(X)(p22.3q28) and mental retardation: analysis of the X-inactivation patterns.

We report on a mother and daughter both with a 45,X/46,X,r(X)(p22. 3q28) karyotype and mental retardation. Fluorescence in situ hybridization (FISH) and microsatellite analyses for 14 loci/region at Xp22.3 and seven loci/region at Xq28 indicated that the ring X chromosome was missing a roughly 12-Mb region from Xp22.3 with the breakpoint between DXS85 and DXS9972, and another region of less than 100 kb from Xq28 with the breakpoint distal to the region defined by the FISH probe c8.2/1. X-inactivation analysis, using the methylation status of the AR gene (exon 1) as an indicator, showed that the normal and ring X chromosomes in the X,r(X)(p22.3q28) cell lineage were randomly inactivated. The Xp22.3 deleted region partially overlaps with the regional intervals of MRX19, MRX21, MRX24, MRX37, MRX43, and MRX49 associated with heterozygote manifestation. Therefore, it is likely that one or more of these MRX genes, subject to X-inactivation, are lost from the ring X chromosome, and that reduced expression of the MRX gene(s) caused by random X-inactivation has resulted in mental retardation in the mother and daughter.

Adult↗
PubMed2000

Jugular vein temperature reflects brain temperature during hypothermia.

PURPOSE: The neuroprotective properties of mild to moderate hypothermia are well recognized but may not be employed correctly because brain temperature cannot usually be measured directly. This study investigated the jugular vein as a more accessible site that accurately reflects the actual brain temperature during mild, induced hypothermia. METHODS: We selected ten mongrel dogs (mean weight 12 +/- 2 kg) and measured temperatures of the brain, jugular vein, cisterna magna, pulmonary artery and rectum during hypothermia, including cooling and rewarming. The brain temperature needle probe was inserted 2.0 cm into the parenchyma. A temperature probe was placed in the cisterna magna with an epidural needle. Swan-Ganz thermistor probes measured the jugular venous and pulmonary artery blood temperatures. RESULT: The brain temperature decreased from 37.5 +/- 0.3 to 33.0 +/- 0.3 degrees C over an average 150 +/- 45 min cooling period. Stable cool was maintained for 245 +/- 32 min, followed by 165 +/- 50 min for rewarming from 33.5 +/- 0.3 to 37.5 +/- 0.3 degrees C. Jugular, cisterna magna and pulmonary arterial blood (PAB), but not rectal temperature, were close to brain temperature during stable cool. The mean jugular and cisterna magna temperatures were near the brain temperature at 0.1 degrees C higher and 0.1 degrees C lower, respectively. No significant effects of hypothermia were noted on hemodynamics in any phase. CONCLUSION: Jugular vein temperature, along with cisterna magna and pulmonary artery blood and rectal temperature, reflected brain temperature during hypothermia. The jugular vein and cisterna magna sites more sensitively reflected brain temperature than other sites.

Animals↗
PubMed2000

Comparative effect of amrinone, aminophylline and diltiazem on rat airway smooth muscle.

We investigated the effect of amrinone, a phosphodiesterase III inhibitor, on rat airway smooth muscle, and thereafter, compared its activity with aminophylline and diltiazem. Amrinone produced relaxation of the acetylcholine-induced airway contraction in a dose-related manner. This bronchodilatory activity of amrinone was similar to that of aminophylline, but smaller than that of diltiazem. The 50% relaxant effect (ED50) of amrinone, aminophylline and diltiazem were 3.6 x 10(-4) M, 1.4 x 10(-4) M and 1.4 x 10(-5) M, respectively. Diltiazem was the most potent airway relaxant, and amrinone was less potent in these experiments. Taken together in its positive inotropic and chronotropic effects and anti-inflammatory activity, however, amrinone could be beneficial for treatment of patients suffering from asthma or heart failure with cardiac asthma.

Acetylcholine↗
PubMed2000

Total and prolonged filling of the lungs with Ringer's solution under extracorporeal lung assist (ECLA) in dogs.

BACKGROUND: Massive alveolar lavage has been used clinically to remove materials accumulated in the alveoli. Recently, filling the lungs with oxygenated perfluorochemical (total liquid ventilation) has been investigated. However, effects of complete and prolonged filling of bilateral lungs with aqueous fluid, such as saline or Ringer's solution, has not been evaluated, although it is possible to sustain gas exchange without the natural lung by using extracorporeal circulation and an artificial lung (extracorporeal lung assist: ECLA). It is also not known whether the lung can recover gas exchange ability after prolonged fluid filling. METHODS: Normal mongrel dogs were endotracheally intubated under general anesthesia and mechanically ventilated. After initiation of venoarterial ECLA, warmed lactated Ringer's solution was instilled into the lungs through the endotracheal tube, and the lungs were completely filled at a hydrostatic pressure of 15 cmH2O (fluid-filled group: group F). After the lungs were filled for 4 h, the fluid was drained and ventilation was re-instituted. ECLA, then mechanical ventilation was gradually weaned within 24 h after fluid drainage. In control group (group C), dogs were kept apneic for 4 h with their lungs inflated at an airway pressure of 15 cmH2O with air. RESULTS: Transient hypoxemia occurred during fluid filling but every dog could be weaned from ECLA and mechanical ventilation to spontaneous respiration. The average rate of fluid absorption from the lung during fluid filling was 4.2+/-1.8 ml kg(-1) h(-1). After fluid drainage and restart of mechanical ventilation, bilateral lungs were expanded and well aerated. Total static respiratory system compliance (static compliance) remained unchanged even after fluid filling, and the weight of the lung water did not increase significantly compared to that in group C. Total urine volume was significantly increased in group E Histologically, alveolar structures were preserved and no interstitial edema or bleeding was seen in either group. CONCLUSION: Complete filling of the bilateral lungs for 4 h with lactated Ringer's solution under ECLA causes no deterioration in gas exchange or static compliance in normal dogs, although transient hypoxemia occurs during fluid filling.

Animals↗
PubMed2000

Echocardiographic and pathological evaluation of atherosclerosis in the ascending aorta during coronary artery bypass grafting.

UNLABELLED: We performed intraoperative echocardiography with an epiaortic probe to assess the correlation between echocardiographic appearance and pathological findings of the aorta and to examine the effect of cross-clamping on the aortic wall in 276 patients who underwent coronary artery bypass grafting. The ascending aorta was divided into three segments as follows: lower (L), upper (U), and innominate. The anterior (ant) and posterior (post) intimal thicknesses of each of the three segments were measured. The echogenicity at each of the six locations was examined and was classified as isoechoic or nonisoechoic (hyperechoic, hypoechoic, or mixed type). Tissue punched from the ant L wall of the ascending aorta for vein anastomosis was examined for the presence of atheroma. At the ant L, the prevalence of atheroma was significantly higher in nonisoechoic walls than in isoechoic walls (P = 0.049). We divided patients into two groups according to echogenicity at the U segments. Group A (n = 213) consisted of patients whose echogenicities at both ant U and post U were isoechoic. Group B (n = 63) consisted of patients with nonisoechoic echogenicity at ant U and/or post U. The intimal thicknesses at all six locations in Group B patients were greater than those of Group A (P < 0.01). Deformities at the clamp site after cardiopulmonary bypass were observed significantly more often in Group B than in Group A (P < 0.01). Our data suggest that a nonisoechoic aortic wall indicates more advanced atheroma and a higher risk of deformities at the clamp site. Examination of the echogenicity of the ascending aorta may be one method to reduce perioperative neurological complications. IMPLICATIONS: We performed epiaortic echocardiography during coronary artery bypass grafting and found that the presence of atheroma and deformities at the cross-clamping site were significantly more prevalent in nonisoechoic walls than isoechoic walls.

Aged↗
PubMed2000

Sex-determining gene(s) on distal 9p: clinical and molecular studies in six cases.

We report on clinical and molecular findings in five karyotypic males (cases 1-5) and one karyotypic female (case 6) with distal 9p monosomy. Cases 1-3 and 6 had female external genitalia, case 4 showed ambiguous external genitalia, and case 5 exhibited male external genitalia with left cryptorchidism and right intrascrotal testis. Gonadal explorations at gonadectomy in cases 3 and 4 revealed that case 3 had left streak gonad and right agonadism, and case 4 had bilateral hypoplastic testes. Endocrine studies in cases 1-4 and 6 showed that cases 1, 3, and 6 had definite primary hypogonadism, with basal FSH levels of 54, 39, and 41 IU/L, respectively, whereas case 2 with severe malnutrition was unremarkable for the baseline values, and case 4 had fairly good testicular function. Fluorescence in situ hybridization and microsatellite analyses demonstrated that all cases had hemizygosity of the 9p sex-determining region distal to D9S1779, with loss of the candidate sex-determining genes DMRT1 and DMRT2 from the abnormal chromosome 9. Sequence analysis in cases 1-4 and 6 showed that they had normal sequences of each exon of DMRT1 and the DM domain of DMRT2 on the normal chromosome 9, and that cases 1-4 had normal SRY sequence. The results provide further support for the presence of a sex-determining gene(s) on distal 9p and favor the possibility of DMRT1 and/or DMRT2 being the sex-determining gene(s). Furthermore, as hemizygosity of the 9p sex-determining region was associated with a wide spectrum of gonadogenesis from agonadism to testis formation in karyotypic males and with primary hypogonadism regardless of karyotypic sex, it is inferred that haploinsufficiency of the 9p sex-determining gene(s) primarily hinders the formation of indifferent gonad, leading to various degrees of defective testis formation in karyotypic males and impaired ovary formation in karyotypic females.

Adult↗