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Biomedical subjects

K Hirabayashi

Publications and source records attributed to K Hirabayashi.

At least 73 records · Page 4Linked to original sources

Linkage of Miyoshi myopathy (distal autosomal recessive muscular dystrophy) locus to chromosome 2p12-14.

Miyoshi myopathy (MM) is a young-adult-onset, autosomal recessive distal muscular dystrophy initially affecting the plantar flexors. We analyzed 12 MM families, five with consanguineous marriage, for chromosomal linkage using polymorphic microsatellite DNA markers to map the MM gene. A significant lod score was obtained with the 2p12-14 locus D2S291 (Zmax = 15.3 at theta = 0). Two additional 2p12-14 markers, D2S286 (Z = 10.7 at theta = 0) and D2S292 (Z = 7.2 at theta = 0.05), also gave significant lod scores. These markers will be useful for diagnosis of symptomatic and presymptomatic patients, prenatal and carrier diagnosis of family members carrying MM, and ultimately identification of a gene responsible for MM.

Adolescent↗

[A 86-year-old woman with dementia, gait and speech disturbance, and right hemiparesis].

We report a 86-year-old woman who developed dementia, gait disturbance, speech disturbance, and right hemiparesis. The patient was well until March of 1979 when upon wakening up on one morning she noted slurring of her speech and weakness in her left upper and lower extremities. These symptoms cleared up during the next several months, however, she noted weakness in her left leg again in May 1985. In 1988, her posture became stooped and she walked in small steps. In 1990, she developed memory disturbance and difficulty in naming. In March 1993, she developed weakness in her right hand; she was treated with aspirin and amantadine HCl, however, she deteriorated during the next two week period, and was admitted to our hospital on March 27, 1993. On admission, she appeared alert, however, she could not answer verbally to questions; she could only utter unintelligible sounds. Apparently she was markedly demented. Her blood pressure was 170/98 mmHg, and general physical examination was unremarkable. Cranial nerves were grossly normal except for marked non-fluency in her word expression. She could not stand or walk, and apparently her right upper and lower extremities were paralyzed with some contracture. Deep reflexes were normally active without asymmetry. Chaddock sign was positive bilaterally. Sensory examination was difficult. Pertinent laboratory examination included WBC 13,000/microliters, BUN 152mg/dl, creatinine 3.75mg/dl, CRP 20.1mg/dl; a chest X-ray film revealed pneumonic shadow in the upper and the middle right lung fields. Cranial CT scan revealed multiple lacunar infarctions in both basal ganglia and cerebral white matters; periventricular lucency was also noted. She was treated with antibiotics and intravenous fluid. Acid-fast bacilli were recovered from sputum, and she was transferred to another hospital for the treatment of pulmonary tuberculosis. After its treatment she returned to our hospital on July 8, 1993, when her condition was complicated with aspiration pneumonia. On admission, she was semicomatose, and no intelligible words were heard. Right facial paresis of the central type was noted. She was unable to stand or walk, and her right upper and lower extremities were paretic. Deep reflexes were increased with extensor toe sign on the right. She was treated with chemotherapy and intravenous fluid, however, her clinical course was complicated with respiratory as well as urinary tract infections. She developed cardiac as well as renal failure and expired on September 25, 1993.(ABSTRACT TRUNCATED AT 400 WORDS)

Aged↗

[Latent impairment of left ventricular filling in hypertension without left ventricular hypertrophy and improvement by dipyridamole treatment: pulsed Doppler echocardiography study].

Left ventricular (LV) filling impairment in patients with hypertension (HT) not necessarily associated with LV hypertrophy has not been sufficiently investigated. Therefore, we examined the response of LV filling to isometric exercise in patients with HT without LV hypertrophy and LV filling abnormality at rest. We studied 25 patients (aged 40 to 66 years, mean 51 +/- 7 years) and 13 age-matched normal subjects. The HT patients were selected by the following criteria: 1) systolic blood pressure (sBP) over 160 mmHg and/or diastolic BP over 90 mmHg was observed at least three times during the last 6 months, 2) LV wall thickness was under 11 mm, and 3) the ratio of peak atrial LV inflow velocity (A) to peak early diastolic LV inflow velocity (E) was within the mean +/- SD of normal subjects. LV inflow was measured by pulsed Doppler flowmetry before and during handgrip exercise (50% maximal effort for one minute and a half) in the patients before [HT-D (-)] and after [HT-D (+)] dipyridamole (D) administration (0.28 mg/kg/4 min) and in the normal subjects (N). Doppler-derived indices were A, E, A/E, DR (the deceleration rate from peak to half of the early diastolic inflow velocity), % delta A/E (% change in A/E from baseline), and % delta DR (% change in DR from baseline). There was no significant difference in LV wall thickness between the HT and N groups. There was also no significant difference in A/E at rest between the three groups. Increase of sBP and heart rate were similar in all groups during handgrip exercise.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Realignment of postoperative cervical kyphosis in children by vertebral remodeling.

STUDY DESIGN: This study analyzed radiographically change in the sagittal curvature of the cervical spine after atlantoaxial (C1-C2) posterior fusion in children. OBJECTIVES: This study clarified the process of spinal remodeling after postoperative cervical deformation in children. SUMMARY OF BACKGROUND DATA: Postoperative spinal deformations in children are observed frequently. However, there have been only a few reports on postoperative changes in the sagittal curvature of the cervical spine and spinal remodeling after those changes. METHODS: Between 1979 and 1991, there was a total of 12 children who underwent C1-C2 posterior fusions. The average age at the time of surgery was 9.8 years. The alignment of the cervical spine was classified into four groups (lordosis, straight, kyphosis, and swan-neck deformity). Radiographic findings suggestive of the remodeling were as follows: 1) new bone formation on the anterior vertebral cortex, and 2) increase in body/canal ratio (BCR). The follow-up period averaged 6.2 years. RESULTS: Postoperative cervical malalignment (kyphosis or swan-neck deformity) occurred in four patients. In all four patients, new bone formation and increase in BCR at the apex of kyphosis were observed. Therefore, there was gradual improvement of the malalignment by vertebral remodeling. This phenomenon was not observed in eight patients with normal alignment. CONCLUSION: Realignment of postoperative cervical kyphosis by vertebral remodeling was observed in children. The results of this study suggested that remodeling occurred even in the spine, which was similar to the remodeling in long bones.

Atlanto-Axial Joint↗

Long-term follow-up studies of open-door expansive laminoplasty for cervical stenotic myelopathy.

Follow-up at an average time of 7.8 years postoperatively on open-door expansive laminoplasty (EL) was carried out to determine the long-term results of surgery. Thirty-three patients had ossification of the posterior longitudinal ligament and 18 had cervical spondylotic myelopathy. The average age at operation was 54.7 years. Japanese Orthopaedic Association scores and recovery rates increased during the 3 years after surgery and then plateaued. Radiographically, average spinal canal diameter remained enlarged past 5 years' follow-up. Factors leading to worsening of clinical symptoms included age greater than 60 years (4 patients), loss of sagittal canal diameter (2 patients), progression of ossification (4 patients), and minor trauma (1 patient). Postoperative motor paresis due to C5 and C6 root damage recovered to 4 (manual muscle testing) in all patients within 6 years. The conclusion is that open-door EL is safe and leads to good results that are maintained for over 5 years.

Cervical Vertebrae↗

Von Willebrand factor in the rat kidney: its localization and the effects of its in vivo interaction with specific antibodies.

Antiendothelial cell antibodies and increased von Willebrand factor (vWF) levels are observed in many renal diseases. We studied renal morphology after administration of rabbit anti-human factor VIII-von Willebrand factor (FVIII-vWFc) gamma-globulin (IgG) in rats. Isolated perfusion of normal rat kidney with the antibodies disclosed rabbit IgG along glomerular endothelium and in mesangial areas. Systemic administration resulted in an identical antibody distribution. C3 deposits and few electron dense deposits were transiently noted. After 1 week the mesangial deposition of rabbit IgG had increased significantly, most likely reflecting increased release of vWF by the endothelium. Thereafter, the deposits gradually relocated into mesangial stalk and into the glomerular vascular pole, and they had vanished after 6 weeks. Except for a mild influx of leukocytes, no renal injury occurred. In conclusion, although FVIII-vWFc antibodies cause mesangial immune deposits, antibodies against other endothelial antigens are apparently needed to inflict renal damage.

Animals↗

An acidic polysaccharide with immunological activities from the root of Paeonia lactiflora.

An acidic polysaccharide, called peonan PA, was isolated from the root of Paeonia lactiflora PALLAS. It was homogeneous on electrophoresis and gel chromatography, and its molecular mass was estimated to be 6.0 x 10(4). Peonan PA is composed of L-arabinose: D-galactose: D-galacturonic acid in the molar ratio of 2:1:10, in addition to small amounts of O-acetyl groups and peptide moieties. About forty percent of the hexuronic acid residues in peonan PA exist as methyl esters. Reduction of carboxyl groups, methylation analysis, lithium degradation and nuclear magnetic resonance studies indicated that its main structural features involve both alpha-1,5-linked L-arabino-beta-3,6-branched D-galactan type and alpha-1,4-linked D-galacturonan type structural units. The polysaccharide exhibited remarkable reticuloendothelial system-potentiating activity in a carbon clearance test and considerable anti-complementary activity.

Animals↗

The core structure of ginsenan PA, a phagocytosis-activating polysaccharide from the root of Panax ginseng.

Controlled Smith degradation and limited hydrolysis of ginsenan PA, the main phagocytosis-activating polysaccharide isolated from the root of Panax ginseng C. A. Meyer, were performed. The reticuloendothelial system-potentiating and anti-complementary activities of the degradation products were investigated. Methylation analysis of the primary and secondary Smith degradation products indicated that the core structural features of ginsenan PA include a backbone chain mainly composed of beta-1,3-linked D-galactose. Almost half of the galactose units in the backbone carry side-chains composed of beta-1,6-linked D-galactosyl residues at position 6. Further 3,6-branching of D-galactose units was observed in a part of the side-chains. alpha-L-Arabinose units are connected mainly to the core galactose moieties via position 6. Removal of most of the arabinose units had a considerable effect on immunological activity.

Animals↗

[Histological and radiobiological study on adult cases with ataxia telangiectasia].

Ataxia telangiectasia (AT) is a hereditary disease that involves both the immune and nervous system; its pathogenesis is believed to be due to disturbances in DNA repair system. Because children are usually affected, reports concerning adult patients are rare. In the present study, we performed comprehensive clinical, histological, and radiobiological examinations on two adult female siblings, currently 23 and 19 years old. The initial symptom was an ataxic gait, which began in the elder sister at the age of 20 and the younger sister at the age of 18. Both had mild telangiectasia in the bulbar conjunctiva and auricula. The neurological manifestations of both were gaze nystagmus, cerebellar ataxia, hyporeflexia, and vibratory sensation disturbance. Only the elder sister showed stocking type sensory disturbance. The MRI of both patients revealed cerebellar atrophy, especially in vermis. After either X-ray irradiation or bleomycin treatment, fibroblast survival curve showed a marked, dose-dependent declined in both patients compared with normal controls. Similar patterns were observed in both patients. After bleomycin treatment, no differences in the survival ratio of cultured fibroblasts could be found between these adult patients and those observed in younger children with classic AT. Biopsied sural nerves showed a decrease in the number of large myelinated fibers, with a few myelinated axons having relatively thin myelin. There were severe atrophic changes, with regenerative myelinated fibers in the elder sister. In conclusion, both adult patients with delayed onset AT displayed similar bleomycin hypersensitivity in fibroblasts as would be expected in children with AT. Radiobiological examinations and sural nerve biopsy suggest that both disturbed cellular regeneration and atrophic process may play a role in the neural involvement in this disease.

Adult↗

[Phase II clinical study of RP56976 (docetaxel) in patients with carcinoma ovarii or carcinoma colli uteri].

An early phase II clinical study of RP56976 (docetaxel), a new semisynthetic agent, in patients with carcinoma ovarii or carcinoma colli uteri was undertaken by a cooperative study group of 23 institutes. Docetaxel was administered at an initial intravenous dose of 60 mg/m2 with dose-free intervals of 3-4 weeks, and its efficacy and safety were evaluated. Of the 47 patients with carcinoma ovarii enrolled, 44 patients were eligible and 36 patients completed the scheduled course of treatment. Of the 23 patients with carcinoma colli uteri enrolled, 20 patients were eligible and 15 patients completed the scheduled course of treatment. For antitumor efficacy in patients with carcinoma ovarii, 1 patient showed partial response (PR), 10 showed no changes (NC) (2 showed minor response (MR)), and 25 had progressive disease (PD). The overall response rate was 2.8% (1/36). Of patients with carcinoma colli uteri, 7 patients showed no changes (NC) (1 patient showed minor response (MR)), 8 patients had progressive disease (PD). Major adverse reactions included 64/65 (98.5%) leukopenia, 56/59 (94.9%) neutropenia, 40/60 (61.5%) decrease of hemoglobin, 12/64 (18.8%) thrombocytopenia, 30/65 (46.2%) anorexia, 23/65 (35.4%) nausea/vomiting, 37/65 (56.9%) alopecia, and 26/65 (40.0%) fatigue, all of which were mild.

Adenocarcinoma, Clear Cell↗

[Ovarian cancer].

Primary aggressive cyto-reduction followed by adjuvant chemotherapy has so far been the standard treatment modality for advanced ovarian cancer. However, as cisplatin-containing regimens have been shown to generate high efficacy rates, a new modality involving extirpation of the tumor after chemotherapy, rather than surgery as the first treatment, has become available for patients with stage III or IV ovarian cancer in whom tumor cells may remain after surgery. In our examination of 56 cases, higher efficacy rates, higher tumor-debulking rates, lower frequency of extended surgery and longer median survival time were obtained with preoperative chemotherapy in comparison with postoperative chemotherapy. These results were almost equivalent to those shown in the recent reports on preoperative chemotherapy for ovarian cancer: the efficacy rates have exceeded 80% against ovarian cancers except for mucinous adenocarcinoma and clear cell adenocarcinoma (70% of all ovarian cancers). In addition, preoperative chemotherapy has an advantage over postoperative chemotherapy in that the higher debulking rate may result in an improved quality of life. Moreover, the preoperative chemotherapy per se can be the best sensitivity testing, since chemotherapeutic effects can be determined during the following surgery. However, a large randomized trial is needed to definitely confirm the higher efficacy of preoperative chemotherapy over postoperative chemotherapy. Thus, it is considered that intensive induction chemotherapy followed by aggressive cyto-reduction can produce optimal cyto-reduction, which seems to offer a good prognosis. Thus, it should be the standard treatment modality for advanced ovarian cancer.

Adenocarcinoma, Clear Cell↗

Predictability of operative results of cervical compression myelopathy based on preoperative computed tomographic myelography.

The transverse area and flattening ratio of the spinal cord were determined with preoperative computed tomographic myelography in 103 patients with cervical compression myelopathy: cervical spondylotic myelopathy (n = 44); ossification of the posterior longitudinal ligament (n = 39); and cervical disc herniation (n = 20). With these values and other clinical items (eg, age, duration of symptoms, preoperative severity), a linear model to predict postoperative recovery was attempted by multiple regression analysis. In cervical spondylotic myelopathy and ossification of the posterior longitudinal ligament, the transverse area of the spinal cord and the duration of symptoms were accepted as effective explanatory variables to predict recovery. In cervical disc herniation, regardless of the transverse area or duration, the recovery was good, and pathologic state was considered essentially different.

Cervical Vertebrae↗

Neutral proteinases in human intervertebral disc. Role in degeneration and probable origin.

Neutral proteinases were partially purified from human intervertebral disc by extraction with guanidine hydrochloride followed by Sephadex G-75 column chromatography. They showed gelatinolytic and elastolytic activities at neutral pH. The apparent molecular weights of these enzymes were 70 KD and 25 KD, the former being the complexed form of the latter. The sensitivity to various synthetic inhibitors indicated these enzymes to be serine elastases. Furthermore, they were considered to be leukocyte elastases because anti-leukocyte elastase antibody inhibited the proteinase activity of these enzymes. Proteinase activity was not detected in normal discs, except for some slight activity in the end-plate. In contrast, the degenerated discs showed high activity, especially in the nucleus pulposus and end-plate. Similar high levels of proteinase activity were found in the vertebral body adjacent to normal as well as degenerated discs. These findings suggest that leukocyte elastase, which is normally present in the vertebral body, flows into and degrades the matrix of the intervertebral disc under pathologic conditions.

Adolescent↗

Combination chemotherapy with 254-S, ifosfamide, and peplomycin for advanced or recurrent cervical cancer.

BACKGROUND: Based on the fact that combination chemotherapy with cisplatin, ifosfamide, and bleomycin generated a 69% response rate in patients with recurrent cervical cancer; that 254-S (a cisplatin analogue) monotherapy generated a 46.3% response rate for cervical cancer, which was higher than those generated by cisplatin and carboplatin in historic comparison; and that peplomycin is a bleomycin analogue with improved pulmonary toxic effects, a combination regimen with 254-S, ifosfamide, and peplomycin was evaluated in an animal experiment and a clinical study in patients with advanced or recurrent cervical cancer with an expectation that the regimen might show a higher efficacy than 254-S monotherapy and the combination regimen including cisplatin. METHODS: In the clinical testing, 254-S was administered intravenously (IV) at 80-100 mg/m2, ifosfamide was administered IV at 1500 mg/patient for 5 days, and peplomycin was administered intramuscularly at 5 mg/patient for 6 days. This treatment was repeated every 4 weeks. RESULTS: As a result, this regimen showed additive or synergistic antitumor effects in mice receiving B16 melanoma transplants. In the clinical study, 83.8% and 60.9% response rates were obtained in 37 previously untreated patients with Stage III or IV cervical cancer and 23 with recurrent cervical cancer, respectively. The dose-limiting factor was bone marrow toxic effects, which were tolerable. The other toxic effects were mild, and there were no deaths. CONCLUSIONS: From these results, this combination regimen was thought worthy of evaluation in a Phase III comparative study in patients with advanced or recurrent cervical cancer.

Adult↗

Accumulation of thallium-201 in medullary thyroid cancer with negative serum calcitonin and carcinoembryonic antigens: a case report.

Thallium-201 (201Tl) scintigraphy has been used in the evaluation of thyroid neoplasm. Even though several authors have shown the usefulness of 201Tl scintigraphy in evaluating recurrent tumor of medullary thyroid cancer, the number of reports on primary thyroid cancer is limited. We presented a case of medullary thyroid cancer which showed 201Tl uptake in both early and delayed scans. This case had normal serum levels of calcitonin and carcinoembryonic antigen.

Calcitonin↗