A case of Creutzfeldt-Jakob disease with a point mutation at codon 232: correlation of MRI and neurologic findings.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to H Goto.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
BACKGROUND: This study was carried out to ascertain the clinical usefulness and problems of three-dimensional (3D) images obtained with the ultrasound probe. METHODS: Eighteen resected specimens and 21 patients were examined. After the images were recorded on video, 3D displays were produced using a workstation. RESULTS: In the in vitro study the surface images were quite consistent with the macroscopic findings in 17 cases. In 2 esophageal cancers, 7 of 10 gastric cancers, and 2 colonic cancers the depth of tumor invasion was assessed accurately from the reconstructed images. In the in vivo study although 3 D display had some limitations, it was useful in esophageal and rectal lesions. CONCLUSIONS: This new diagnostic method could visualize the 3D extent of gastrointestinal lesions and appears to have a useful clinical application.
To assess the oxygen transport capacity and safety of Neo Red Cells (NRC) with the enzymatic reduction system of methemoglobin in vitro and in experimental animals. Stroma free hemoglobin (SFH) prepared without damage of enzymes from outdated human red blood cells, together with inositol hexaphosphate as an allosteric effector, NAD as a coenzyme and glucose, adenine and inosine as a substrate was encapsulated within liposomes composed of hydrogenated soy phosphatidylcholine, cholesterol, myristic acid and alpha-tocopherol in the ratio of 7:7:2:0.28 respectively. NRC thus prepared with a mean diameter of 220 nm, encapsulation efficiency of 1.3 g-Hb:1 g-lipid and P50O2 of 50-60 mmHg were then coated with polyethylene glycol bound to hydrogenated soy phosphatidylethanolamine as a surface modifier to prevent aggregation of NRC in plasma. The methemoglobin formation of the NRC with enzymatic reduction system were evaluated by in-vitro examination and exchange transfusion with rats as in-vivo examination, then the methemoglobin formation was reduced from 1%/hr to 0.4%/hr by the addition of methemoglobin reduction system. The generation of the pyruvate and the lactate were observed within the NRC with enzymatic reduction system, then the activation of the Embden-Meyerhof pathway was confirmed. And we concerned about the availability of the NRC as a perfusate for the cardiopulmonary bypass during moderate or profound hypothermia, then we evaluated the oxygen transporting efficiency and capacity of the NRC under the using of the artificial lung system in vitro examination. The present investigation suggest that the effectiveness of the NRC with enzymatic reduction system, they restrained the formation of methemoglobin and they are efficient oxygen carriers as a perfusate of the artificial lung, and we suggest the new extracorporeal circulation system using of the NRC as a perfusate for the cardiopulmonary bypass.
Lesch-Nyhan syndrome is caused by the complete deficiency of hypoxanthine guanine phosphoribosyl-transferase (HPRT). By the analysis of genomic DNA and mRNA using the polymerase chain reaction (PCR) technique coupled with direct sequencing, five independent mutations in HPRT genes have been identified in Korean Lesch-Nyhan families. Two novel mutations and three previously reported mutations have been found in five independent families. Heterozygous carriers were detected in all the families, and prenatal diagnosis was carried out in two families.
The purpose of this study is to examine the usefulness of intraductal ultrasonography at a frequency of 20 or 30 MHz in the diagnosis of mucin-producing tumor. The subjects were 66 patients with mucin-producing tumor (10 with main pancreatic duct type tumor and 56 with branch type tumor) who had also undergone endoscopic ultrasonography. In main pancreatic duct type tumors, we could diagnose the extent of the tumor and evaluate the invasion of the tumor into the pancreatic parenchyma in all seven patients who had undergone resection (three invasive tumors, four noninvasive tumors). Sixteen of 32 resected branch type tumors showing mural nodules with intraductal sonography were carcinoma or adenoma. On comparing intraductal and endoscopic ultrasonography in the rate of detecting nodules, in five tumors (one carcinoma and four adenomas), nodules were detected only with intraductal ultrasonography. In conclusion, intraductal ultrasonography is very useful for the diagnosis in the main pancreatic duct type tumor and in the detection of mural nodules in the branch type tumor.
Explore the source record for details and available documents.
We previously reported that ATP, but not adenosine, administered i.v. attenuates the baroreflex-mediated increase in sympathetic nerve activity in response to arterial hypotension by a vagal afferent mechanism. It was not elucidated in that study which vagal afferent endings are involved. Mongrel dogs were anesthetized with alpha-chloralose, thoracotomy was performed and a 27-gauge hypodermic needle was inserted into the left circumflex coronary artery. The left renal sympathetic nerves were isolated and placed on a bipolar silver electrode for measurement of renal sympathetic nerve activity (RSNA). Dose-response effects of intracoronary or i.v. infusion of ATP (100, 200 or 400 microg/kg/min) on RSNA and mean arterial pressure were studied in neuraxis-intact and cervically vagotomized dogs. RSNA was increased dose-dependently with decreasing mean arterial pressure during the i.v. ATP infusion. Elevation of RSNA was attenuated by higher intracoronary ATP infusion rates, despite the fact that mean arterial pressure was decreased dose-dependently. Left ventricular end-diastolic pressure, however, remained unchanged. This suppression of RSNA by the intracoronary ATP infusion was completely abolished by bilateral cervical vagotomy. Our data suggest that ATP attenuates reflex increases in sympathetic nerve activity by possibly stimulating ventricular chemoreceptors with cardiac vagal afferents.
Explore the source record for details and available documents.
We investigated the transmigrating activity of peripheral blood T cells of patients with human T-lymphotropic virus type I-associated myelopathy (HAM) through reconstituted basement membrane. The transmigrating activity of CD4+ T cells of HAM patients was increased significantly in comparison to that of anti-HTLV-I-seropositive carriers and HTLV-I-seronegative controls. However, the migrating activity of CD8+ T cells was not significantly different in HAM patients and controls. The activity of aminopeptidase-N in peripheral blood T cells of HAM patients also was increased significantly, as compared to that of controls. In addition, HTLV-I proviral load in transmigrating CD4+ T cells of HAM patients was increased significantly (two- to eight-fold), compared to that in nontransmigrating CD4+ cells. By contrast, no significant difference in HTLV-I proviral load was found between transmigrating and nontransmigrating CD4+ cells of HTLV-I-seropositive carriers, although copy numbers of HTLV-I proviral load were very low in them. The heightened transmigrating activity of CD4+ cells from HAM patients through reconstituted basement membrane is based on the increased activity of aminopeptidase-N. Collectively, these findings suggest that HTLV-I-infected CD4+ T cells play an important role in the early stage of the pathogenesis of HAM.
A thread of surgical sponge was found by colonoscopy at the sigmoid colon wall in the tip of a granuloma. The patient was a 50-year-old woman who complained of diarrhea and had a history of Cesarean section and complete hysterectomy. A radiopaque thread that used to be part of the surgical sponge was demonstrated with plain pelvic X-ray films. Abdominal CT study revealed the gossypibioma including a radiopaque thread in front of the sacrum. The patient was admitted for endoscopic extraction. Upon endoscopic observation, no thread was found and the fistula had also disappeared. The surgical sponge was considered to have been spontaneously expelled from the fistula during the intervening 22-day period.
Arterial-venous carbon dioxide tension difference (Pv-aCO2) is known to become high after severe hemorrhage shock and resuscitation. We hypothesized that Pv-aCO2 might be high after cardiac surgery because of the oxygen debt occurred during hypothermic cardiopulmonary bypass (CPB). Blood pressure, cardiac index, hemoglobin, the arterial and mixed venous blood gases were repeatedly measured every 6 hours for 24 hours following cardiac surgery in 60 adult patients who underwent hypothermic CPB. Immediately after the surgery, Pv-aCO2 was extremely high, then gradually decreased to within normal ranges 12 hours later (8.0 +/- 2.9 mmHg vs 5.9 +/- 3.1 mmHg. p < 0.01). Factors which significantly correlated to Pv-aCO2 were cardiac index, oxygen delivery, minimum rectal temperature and duration of CPB. Oxygen debt during hypothermic CPB might cause significantly high Pv-aDO2. At least 12 hours were necessary to recover from anaerobic status to physiological condition.
The effects of polychlorinated biphenyls (PCB) on regenerating myelin of the peripheral nerves were investigated in rats. The sciatic nerves were crushed at the mid-thigh level on the last of 32 days of oral administration of PCB. The sciatic nerves were biopsied from the crushed regions at 4, 8 and 12 weeks after crushing. The nerves were fixed in 2.5% glutaraldehyde buffered with 0.1 M sodium cacodylate and embedded in epon 812. The ultrathin sections were analysed by JEOL JEM-1210 with EDAXDX-4. The chloride which was composed of PCB was increased in myeline on 4 weeks after crushing. Freeze fracture study of myelines of sciatic nerves on 8 and 12 weeks after crushing showed an increase of intramembranous particle on 12 weeks and an increase of caveolae on 8 weeks. These results suggest that PCB may affect the plasmamembrane of shwann cell and induce the disturbance of myeline.
OBJECTIVE: The purpose of this study was to assess and compare inflammatory reactions to two types of sealed vascular prostheses used in the repair of abdominal aortic aneurysm. EXPERIMENTAL DESIGN: Randomized prospective study with a follow-up period of 21 days. SETTING: University hospital study. PATIENTS: Sixty patients admitted for elective abdominal aortic aneurysm repair. INTERVENTION: Each patient underwent repair of abdominal aortic aneurysm with either a gelatin-sealed knitted Dacron prosthesis (Gelseal, Vascutek, n = 30) or a collagen-sealed woven Dacron prosthesis (Hemashield, Meadox Medicals, n = 30). MEASURES: As indicators of the presence of inflammation, the peripheral blood cell count, platelet count, and plasma C-reactive protein (CRP) concentration were determined in each patient on postoperative days (PODs) 1, 7, 14 and 21. Body temperature was measured every 8 hours. RESULTS: Patients in both groups revealed a low-grade fever and leukocytosis during the first 7 PODs. Significantly higher leukocyte counts were demonstrated in the Gelseal group on PODs 7 and 14. However, no difference in the leukocyte count, CRP concentration, or body temperature existed between the two groups on POD 21. Biphasis changes in the platelet count were observed in both groups. No complications were encountered. CONCLUSION: The Gelseal prosthesis was associated with higher inflammatory response during the second week following implantation, but this difference had resolved by POD 21. This finding may reflect an inflammatory reaction against impregnated gelatin.
A case of acute aortic dissection without intimal tear is reported. A 68-year-old woman with acute onset of Stanford type-B aortic dissection was admitted. Because of progressive anemia and pleural leakage, an operation was performed. False lumen of the descending thoracic aorta was occupied with fresh hematoma, but no sign of an intimal defect was found. Although aortic dissection without intimal tear has been reported via angiography, CT scan, MRI, and autopsy, surgically confirmed cases of this type of dissection have been rarely reported.
Polysaccharides extracted from human tubercle bacilli (specific substance of Maruyama [SSM]) have been clinically applied with satisfactory results. Thymidylate synthetase (TS) and thymidine kinase (TK) are key enzymes in de novo and salvage pathways for pyrimidine nucleotide synthesis. Well- and moderately well differentiated adenocarcinomas induced with 1,2-dimethylhydrazine (DMH) are widely distributed throughout the colorectal tract with high TK activity, and the poorly differentiated type is mainly restricted in the proximal colon and the cecum with high TS activity in rats. Subcutaneously injecting the rats with SSM reduced TS activity in colonic nontumorous regions, but in the tumorous regions it reduced TK activity compared with that of the DMH-treated rats without SSM treatment. SSM is suggested to reduce the colorectal carcinogenesis induced with DMH by inhibiting DNA synthesis in a de novo pathway, and to suppress the development of the tumors by decreasing DNA synthesis in the salvage pathway in rats.
The effects of histamine (CAS 51-45-6) on cell growth, collagen synthesis of fibroblasts derived from human foreskin, and on fibroblast-mediated collagen remodelling were studied. The cellmat DNA content was measured 2 days after human fibroblasts were plated at a split ratio of 1:10. Effect of histamine (10(-9)-10(-4) mol/l) on the increase of DNA content was not observed. Fibroblasts at confluence were cultured with histamine only, and with pyrilamine or cimetidine in addition to histamine for 2 h. Type I procollagen C-peptide in the medium was measured by enzyme immunoassay and was corrected by DNA content. Type I collagen synthesis was stimulated by histamine (10(-6)-10(-4) mol/l) and its stimulation was inhibited by cimetidine, but not by pyrilamine. Collagen solution containing fibroblast was incubated until gelation. It was incubated with histamine only, and with pyrilamine or cimetidine in addition to histamine. The gel contraction was stimulated by histamine (10(-4) mol/l) and its stimulation was inhibited by pyrilamine, but not by cimetidine. These facts suggests that histamine stimulates type I collagen synthesis of fibroblast and collagen remodeling via H2 and H1 receptors, respectively.
Lesch-Nyhan syndrome is associated with complete deficiency of hypoxanthine-guanine phosphoribosyltransferase (HPRT), characterized by hyperuricemia and severe neurological signs. The HPRT gene has been mapped to the q26 region on the long arm of the X-chromosome. We are taking care of a family of Lesch-Nyhan syndrome. A 14-year-old male was noted the growth disturbance at the age of 7 months and self-mutilation behavior characterized by compulsive biting of his lip and fingers at the age of 18 months. In 1987, at the age of 4, he was diagnosed as Lesch-Nyhan syndrome from neurologic signs and hyperuricemia (9.8 mg/dl). Neurological examination revealed mild mental and growth retardation, spasticity and hyperreflexia of lower extremities, choreoathetoid movements of extremities, and compulsive self-mutilation. The HPRT activity in erythrocytes of this patient was 0.02 nmol/min/mg hemoglobin (control value 1.76 +/- 0.06), and adenine phosphoribosyltransferase (APRT) activity was 1.08 nmol/min/mg hemoglobin (control value 0.43 +/- 0.06). Using polymerase chain reaction (PCR) method coupled with direct sequencing, we analyzed the nucleotide sequences of each exon from the genomic DNA as well as the entire HPRT coding region of the cDNA by RT-PCR method. In the HPRT gene from the patient, a guanine to adenine substitution at base position 209 in exon 3 was identified, which resulted in a single amino acid substitution of glycine with glutamic acid at codon 70. The family studies indicated that his mother, sister and grandmother were heterozygotes. PCR-restriction fragment length polymorphism (RFLP) utilizing Mnl I site which created by the mutation, was useful for detection of the mutant gene. We have identified a new missense mutation of the HPRT gene in a Japanese patient. This mutation was reported at the same codon as foreign mutants and mighty be indicative of a location of mutation activity in the HPRT gene.