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Biomedical subjects

S Hirose

Publications and source records attributed to S Hirose.

At least 541 records · Page 30Linked to original sources

[Xeroderma pigmentosum presenting clinical features of spinocerebellar degeneration].

A case of group D xeroderma pigmentosum is reported. This 26-year-old woman was normal delivery, and showed a normal psychomotor development. Her parents noted a patchy brownish pigmentation on her limbs, face and trunk soon after the birth. Gait disturbance appeared at 17 years old and progressed over 9 years. Neurological examination disclosed a severe loss of deep sensation, spastic weakness and mild cerebellar ataxia. Slight involvement of peripheral nerves was revealed by the elecrophysiological investigations. CT and MRI brain scans showed a cerebellar atrophy, however no atrophy was seen in the cerebrum. Adrenocortical hypo-function was noted by decreased 17 KS and 17 OHCS. Unscheduled DNA synthesis of the patient's skin fibroblast was 45% of normal controls, and the result was consistent with group D xeroderma pigmentosum. The neurological findings of this patient are well characterized by the degeneration of the posterior and lateral column of the spinal cord, cerebellum and peripheral nerves. We discussed symptomatological and etiological similarities of the present case to spinocerebellar degeneration. It may be necessary to consider the possibility of xeroderma pigmentosum in the differential diagnosis of spinocerebellar degeneration.

Adult↗

[Eosinophilic lymphofolliculosis (Kimura's disease) complicated with aortitis syndrome].

A 47 year-old-man was admitted because of mild congestive heart failure. A differential white blood cell count revealed eosinophilia and a biopsied inguinal lymph node showed well-developed lymphoid follicles with germinal centers, eosinophil infiltration and marked proliferating vessels with swollen endothelial cells. He was diagnosed as eosinophilic lymphfolliculosis (Kimura's disease). He also was hypertensive and vascular murmur was pointed out in his abdomen. Angiography revealed the complication of aortitis syndrome. Renal complication in eosinophilic lymphfolliculosis was reported in some cases and it was though that the disease had a clinical aspect of systemic diseases. The complication of aortitis syndrome in our case is interesting from a point of view of eosinophilic lymphfolliculosis as a systemic disease.

Angiolymphoid Hyperplasia with Eosinophilia↗

[A case report of ascending-abdominal aorta bypass in the treatment of atypical coarctation].

A 45-year-old woman, who had atypical coarctation of the aorta due to aortitis syndrome, underwent ascending-infrarenal abdominal aorta bypass successfully. A woven dacron prosthesis of 14 mm in diameter was anastomosed to the ascending aorta. The course of the graft arranged to pass through a hole cut into the diaphragm across the omental sac behind the pancreas to the infrarenal abdominal aorta. This operation is approached through a midline sternal-splitting and abdominal incision, accordingly pulmonary complication and injury to major collateral vessels associated with lateral thoracotomy can be avoided.

Anastomosis, Surgical↗

[A case of successful surgery of atrial septal defect combined with coronary artery bypass grafting].

A 66-year-old man, who had atrial septal defect (ASD) associated with severe coronary artery disease, underwent closure of ASD and concomitant coronary artery bypass grafting (LIMA-LAD & SVG-RCA). Response to surgery was excellent, with improvement of cardiac function and returning to NYHA functional class I, while, preoperatively, the patient had been limited due to symptoms related both to large left to right shunt flows (Qp/Qs 3.04) and to left ventricular dysfunction because of severe myocardial ischemia as well as previous inferior myocardial infarction. SAD is the most common form of congenital heart disease requiring surgery in adults, accordingly it should be remembered that some of the elderly patients with ASD might have obvious or occult coronary artery disease at the time of diagnosis and operative intervention.

Aged↗

T-cell colony formation in homosexual men with anti-human immunodeficiency virus.

In vitro differentiation and proliferation of precursor T-cells was examined in four homosexual men positive for anti-human immunodeficiency virus (HIV), one of whom had acquired immunodeficiency syndrome (AIDS). Peripheral mononuclear cells from the men were cultured with phytohemagglutinin-P in semi-solid culture media containing methylcellulose for 7 days. Colony formation was significantly reduced in all subjects, even in the presence of IL-2. Moreover, numerous colony component cells lacked T-cell specific surface markers. These results suggest that the impaired T-cell differentiation and proliferation occurs at the precursor T-cell level in HIV infection.

Acquired Immunodeficiency Syndrome↗

[Antibiotics-induced agranulocytosis. Patient's IgG inhibits a GM colony formation].

The pathogenesis of Cefmetazole (CMZ) induced agranulocytosis was investigated in the case of a 40-year-old man who developed agranulocytosis while he was under treatment with CMZ. Although concentration of CMZ in serum is not detected, patient's serum drawn at the time of diagnosis suppressed normal allogeneic marrow GM colonies in vitro. Normal IgG purified from serum had no effect on GM colony formation in vitro. However, patient's IgG purified from serum drawn at the time of diagnosis suppressed normal allogeneic marrow GM colony in vitro without CMZ. These studies suggest that CMZ-induced agranulocytosis is immunologically mediated through an IgG inhibitor which seems to exert its effect on GM colony formation.

Adult↗

[A seven-year course after Fontan operation in an older adult with tricuspid atresia].

Tricuspid atresia (TA) is a uncommon cardiac anomaly. Most patients with TA will develop severe hypoxia due to insufficient pulmonary flow at an early age and long-term survival without any surgical intervention may not be expected. A 38-year-old male with TA (type Ib) was referred with the complaints of increasing cyanosis and easy fatigability. After diagnosis was confirmed through the cardiac catheterization, modified Fontan operation was performed. In the procedure atrial septal defect was repaired by a patch, stenotic pulmonary valve was closed and prosthetic graft was inserted between the right atrium and the main pulmonary arterial trunk. In literatures about adult with TA, left ventricular dysfunction was generally reported. However, in our case postoperative course was uneventful and his symptoms improved dramatically. After the discharge from hospital, he has done well. But in the cardiac catheterization performed 7 years after operation, the cardiac index was slightly decreased and a significant stenosis along the left anterior descending coronary artery was incidentally discovered. In each laboratory examination after operation slight polycythemia was disclosed.

Cardiac Catheterization↗

[A case of cancer of the esophagus accompanied by postoperative fulminant hepatitis].

A 60-years-old male subject, who had complained of swallowing difficulties, was diagnosed as having cancer of the esophagus. No allergic reaction was noted in his history. Mechanical anastomosis connecting the gastric tract with the cervical esophagus was performed satisfactorily. During the operation, haemodynamics remained stable and in the absence of excessive bleeding, 5 bags of fresh frozen plasma were used. One day after the operation, however, marked abnormalities in liver function were observed and by the 11th day GOT and GPT levels had increased to 783 and 1,108, respectively. In addition, redness suggestive of drug rash was observed. Although steroid therapy, followed by plasma exchange therapy, was repeated four times, the patient died on day 21. Autopsy revealed that the patient suffered an acute form of fulminant hepatitis. Viral infection, drug treatment or surgical aggression is often considered to be the cause of this condition. In this subject, it is thought that the hepatic disorder was evoked by either the operation or drug treatment and that subsequently allergic reaction or immune abnormality caused the fulminant hepatitis.

Alanine Transaminase↗

DNA supercoiling facilitates formation of the transcription initiation complex on the fibroin gene promoter.

Transcription of the Bombyx mori fibroin gene in a posterior silk gland extract can be separated into three functional steps on the basis of sensitivity to Sarkosyl: 1) formation of an initiation complex, which is blocked by 0.025% Sarkosyl; 2) conversion of the initiation complex to an elongation complex, a step sensitive to 0.05% Sarkosyl; 3) the subsequent elongation of RNA chain which occurs in the presence of 0.05% Sarkosyl. Whereas the last two steps are rapid and unaffected by template topology, the first step is slow and affected by DNA conformation. In the posterior silk gland extract, closed circular DNA forms a superhelical state and supports more rapid assembly of the initiation complex than linear DNA does. Both DNA supercoiling and rapid assembly of the initiation complex require ATP and are abolished by the addition of a topoisomerase II inhibitor VP16. These results suggest that DNA supercoiling enhances the fibroin gene transcription by facilitating formation of the initiation complex.

Animals↗

Rapid and sensitive on-line precolumn purification and high-performance liquid chromatographic assay for bile acids in serum.

A simple and rapid technique for the simultaneous isolation and analysis of fifteen kinds of bile acid was developed using reversed-phase high-performance liquid chromatography with an automatic dual-precolumn switching system. The serum samples were directly injected onto a first precolumn (hydroxyapatite), which was flushed with 1 mM phosphate buffer. Serum proteins were strongly retained on the hydroxyapatite column, but bile acids were unretained. The bile acids were absorbed on a second precolumn (Serumout-25) and eluted onto the analytical column with solvent B (acetonitrile-methanol-30 mM ammonium acetate, 20:20:60, v/v/v). For the separation of each bile acid, the gradient elution technique was used (solvent A was acetonitrile-methanol-30 mM ammonium acetate, 30:30:40). After separation of the bile acids, NADH was produced by use of immobilized 3 alpha-hydroxysteroid dehydrogenase column and then determined fluorimetrically (gamma em = 460 nm, gamma ex = 350 nm). The recoveries of bile acids in serum generally approached 100%.

Bile Acids and Salts↗

DNA blotting analysis of human retroviruses in cerebrospinal fluid of spastic paraparesis patients: the viruses are identical to human T-cell leukemia virus type-1 (HTLV-1).

The structure of the integrated provirus in cell lines established from the cerebrospinal fluid (CSF) of patients with human T-cell leukemia virus type-1 (HTLV-1)-associated myelopathy (HAM) was analyzed. The digestion patterns with 3 restriction endonucleases, Sac I, Eco RI and PstI, of the proviruses integrated in T-lymphoid cell lines derived from the CSF of 4 HAM patients were similar to those of HTLV-1 from adult T-cell leukemia (ATL) patients. Integrated proviruses in one of these cell lines derived from the CSF were further analyzed in detail with 2 more restriction enzymes, BamHI and Sma I. The results indicate that the retrovirus found in lymphocytes of the CSF from patients with HAM are very similar, if not identical, to HTLV-1 found in the leukemic lymphocytes of ATL patients.

Cell Line↗

Tyrosine O-sulfation of the fibrinogen gamma B chain in primary cultures of rat hepatocytes.

Sulfation of fibrinogen was studied in a primary culture of rat hepatocytes. After cells were incubated with [35S]sulfate, 35S-labeled fibrinogen was obtained from the medium by immunoprecipitation and analyzed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis/fluorography. It was demonstrated that [35S]sulfate is exclusively incorporated into the gamma B chain, which is a minor variant form found in rat fibrinogen, in addition to a major gamma A chain. When the purified 35S-gamma B chain was digested with carboxypeptidase Y, the radioactivity was almost completely released from the protein, and the labeled product released was identified as tyrosine O-sulfate. Based on the available primary structure of the gamma B chain, the results suggest that sulfation occurs on the tyrosine residue at the second position from its COOH terminus. Pulse-chase experiments using both [3H]leucine and [35S]sulfate showed that 35S-labeled fibrinogen is secreted into the medium much faster than the 3H-labeled molecule. Incubation of cells with monensin, an inhibitor of Golgi function, strongly inhibited the sulfation of fibrinogen. In addition, in vitro sulfation experiments demonstrated that sulfotransferase activity is localized in the Golgi fraction. These results indicate that the sulfation of fibrinogen takes place in the Golgi complex, especially in the trans Golgi region, just before its secretion.

Animals↗

Lack of atrial natriuretic peptide receptors in human aldosteronoma.

The effect of synthetic alpha-human atrial natriuretic peptide (ANP) on aldosterone secretion was studied in human aldosterone producing adrenocortical adenoma obtained surgically from a patient with primary aldosteronism and in human apparently normal adjacent adrenal cortical tissues obtained from a patient with pheochromocytoma, in vitro. Apparently normal adrenal cortical tissue responded to ANP with the known inhibition of aldosterone secretion. In contrast, the aldosterone producing adenoma did not respond to ANP. When stimulated by either ACTH or angiotensin II, there is no inhibition by ANP in the adenoma tissue, whereas normal tissue was inhibited. Immunohistochemical examination utilizing an ANP-receptor antiserum demonstrated that there was no evidence of binding site in the cortical adenoma, in contrast, zona glomerulosa cells in the cortical tissues adjacent to either aldosterone producing adenoma or pheochromocytoma were densely stained. This apparent lack of ANP-receptors is an associated finding with the hypersecretion of aldosterone in the aldosterone producing adenoma.

Adenoma↗

Biosynthesis, assembly and secretion of fibrinogen in cultured rat hepatocytes.

The biosynthesis, assembly and secretion of fibrinogen were investigated in cultured rat hepatocytes which were incubated with [35S]methionine. When initial rates of the synthesis of three fibrinogen subunits were compared, the A alpha-subunit was found to be synthesized significantly slower than the B beta- and gamma-subunits. Pulse-chase experiments revealed that the secreted fibrinogen contained different proportions of the newly synthesized subunits, depending upon the chase times. Radioactivity in the A alpha subunit, which initially had the highest level of the three, was rapidly decreased in parallel with the chase time. The gamma-subunit had an increasing amount of the radioactivity in the secreted molecule during the chase periods, whereas that in the B beta-subunit was gradually decreased at the later stages of chase. Analysis of intracellular components of fibrinogen confirmed that the nascent A alpha-subunit was most rapidly exhausted, and the gamma-subunit occupied the largest proportion among the non-assembled subunits at later stages of chase. Taken together, these results suggest that the synthesis of A alpha-subunit, which has the lowest rate, could be the rate-limiting step in the production and secretion of fibrinogen in cultured rat hepatocytes, in contrast with what has been proposed for human and rabbit fibrinogen, namely that the synthesis of B beta-subunit is the rate-limiting step. The results also indicate that there is a large intracellular pool of gamma-subunit.

Animals↗

GTP induces knotting, catenation, and relaxation of DNA by stoichiometric amounts of DNA topoisomerase II from Bombyx mori and HeLa cells.

Type II DNA topoisomerases isolated from posterior silk glands of Bombyx mori and HeLa cells utilize ATP for unknotting of knotted DNA, relaxation of super-coiled DNA, and catenation/decatenation of circular duplex DNA under catalytic conditions. In these reactions, ATP cannot be replaced by GTP. However, GTP induces knotting, catenation, relaxation, but decatenation of circular duplex DNA by stoichiometric amounts of these enzymes. Only a limited round of the reactions proceeds with a concomitant hydrolysis of GTP and then pauses. The GTP-dependent reactions may be employed for maintenance of the knotted and/or catenated state they formed.

Animals↗

Pre-column derivatization of sisomicin with o-phthalaldehyde-beta-mercaptopropionic acid and its application to sensitive high-performance liquid chromatographic determination with fluorimetric detection.

The stability of the o-phthalaldehyde (OPA) derivatives of sisomicin obtained using beta-mercaptopropionic acid was investigated by reversed-phase high-performance liquid chromatography. One of the fluorescent derivatives of sisomicin was stable at least for 6 h in 50% methanol under the optimal conditions used (OPA concentration, pH and temperature). When plasma samples spiked with sisomicin were analysed, the response was linear in the calibration range 136-900 pg of sisomicin per injected volume (40 microliters). As little as 0.06 micrograms of sisomicin per 1 ml of plasma could be detected with signal-to-noise ratio greater than or equal to 2. For plasma samples spiked with 0.2 micrograms/ml sisomicin, the recovery was 97.1 +/- 6.6% (mean +/- S.D., n = 5) with a within-run coefficient of variation of 6.8% and a day-to-day coefficient of variation of 7.2%. The method was also applied to plasma samples from rabbit after a subcutaneous injection of 1 mg/kg sisomicin.

3-Mercaptopropionic Acid↗