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Biomedical subjects

O Yamada

Publications and source records attributed to O Yamada.

At least 19 recordsLinked to original sources

A deletional frameshift mutation of the beta-spectrin gene associated with elliptocytosis in spectrin Tokyo (beta 220/216).

A novel spectrin variant carrying a truncated beta-chain and designated Spectrin Tokyo (beta 220/216) is presented. It was associated with elliptocytosis and moderate uncompensated hemolysis. The dimer self-association was reduced. An increase of the alpha I 74-Kd fragment was detected upon partial trypsin digestion. Analysis of cDNA and genomic DNA showed a 1-base deletion in codon 2059 (GCC AGC-->GCA GCT; Ala-Ser-->Ala-Ala) that belongs to exon X of spectrin beta-gene. A missense sequence extended down to (new) codon 2075. Serine 2060, a potential phosphorylation site, was replaced by alanine. The shortened beta-chain failed to undergo phosphorylation in vitro. Spectrin Tokyo shared the same stop codon, overlapping normal codons 2076 and 2077 (CTG AAA), as Spectrin Nice (beta 220/216), which is caused by a dinucleotide insertion in codon 2046 and contains 2076 amino acids. However, for some reason, Spectrin Tokyo had a lower incorporation level into the membrane than Spectrin Nice.

Base Sequence

Granular lymphocyte leukemia with pure red cell aplasia: usefulness of gene analysis in assessing therapeutic effect.

A patient with granular lymphocyte leukemia (GLL) of the CD3+, CD4-, CD8+ phenotype accompanied by pure red cell aplasia (PRCA) is described. Surface marker analysis, nonmajor histocompatibility complex (MHC)-restricted cytotoxicity assay, gene analysis, and in vitro colony assay were performed on the granular lymphocytes before and after treatment. Cyclophosphamide therapy was highly effective, and after remission clonal granular lymphocytes were no longer identified by T-cell antigen receptor (TCR) gene analysis or surface marker analysis. Lymphocytes obtained after remission did not exhibit elevated levels of non-MHC-restricted cytotoxicity, nor did they demonstrate a suppressive effect on erythroid colony formation. TCR gene analysis proved to be a sensitive parameter for evaluating the residual malignant granular lymphocytes. Gene analysis will be useful both for timing the discontinuation of treatment and for the early detection of relapse. Various factors possibly related to the development of PRCA in this patient were investigated and their significance is discussed.

Bone Marrow

Selective cerebral perfusion during operation for aneurysms of the aortic arch: a reassessment.

Thirty-two consecutive patients with thoracic aortic aneurysms who required aortic arch reconstruction were operated on with the aid of extracorporeal circulation and selective cerebral perfusion between January 1986 and August 1990. For selective cerebral perfusion, blood was infused into both the innominate and left common carotid arteries at a rate of 10 mL.kg-1.min-1 using a single roller pump separately from the systemic circulation. In 9 patients treated before March 1987, the operations were performed without open aortic anastomosis (group 1), whereas in 23 patients treated from March 1987 onward we used open aortic anastomosis (group 2). The extracorporeal circulation and cardiac arrest times were significantly longer in group 2, but there was no significant difference in the cerebral perfusion time. Early death occurred in 1 patient in group 1 and 2 in group 2. No serious cerebrospinal neurological complications occurred in either group, and there were similar rates of postoperative hepatic and renal dysfunction in both groups. The present data suggest that selective cerebral perfusion and open aortic anastomosis are useful methods for thoracic aortic aneurysm operation requiring complex repair of the aortic arch.

Adult

Hepatitis C virus RNA and hepatitis C virus antibody in the serum of patients with abnormal liver function.

In order to elucidate the relation between hepatitis C virus (HCV) RNA and antibody to HCV (anti-HCV) in serum, we examined samples of serum collected from 228 HBsAg-negative patients, with abnormal alanine aminotransferase (ALT) values, for HCV-RNA by nested polymerase chain reaction (PCR) assay and for anti-HCV using C100 protein as the antigen. HCV-RNA was detected in 99 (92.5%) of 107 anti-HCV-IgG-positive samples, regardless of ELISA optical density cut-off value (ELISA ratio), and in 34 (28.1%) of 121 anti-HCV-IgG-negative samples in which the frequency of the presence of HCV-RNA became higher in proportion to the ELISA ratio. Among 42 discordant cases (34 anti-HCV-IgG-negative, RNA-positive cases and eight anti-HCV-IgG-positive, RNA-negative cases), 10 were positive for anti-HCV-IgM (8/34 and 2/8, respectively) irrespective of clinical status. These findings suggest that in patients with abnormal ALT values, even if they are anti-HCV-IgG negative, HCV infection cannot be excluded. Furthermore, PCR assay for detecting HCV-RNA may be more suitable for identifying patients with infectious virus than is detection of anti-HCV-IgG. Detection of anti-HCV-IgM may also be useful.

Alanine Transaminase

Rapid DNA diagnosis of herpes simplex virus serotypes.

The presence of nucleotide sequences specific for each of herpes simplex virus (HSV) serotypes was demonstrated. These sequences were applied for dot DNA-DNA hybridization and for PCR for rapid DNA diagnosis of HSV infections. These sequences were found by molecular cloning of HSV-DNA fragments after digestion of DNA by KpnI enzyme. The type 1-specific sequence was found around the 5' end of BamHI B-fragment in the L region of type 1 DNA (corresponds to alpha gene 27, promoter-regulatory region) and the type 2-specific sequence was around the junction region of the L and S of type 2 DNA (corresponds to a' sequence). Both simple dot blot hybridization and PCR of HSV DNA's, employing these type-specific nucleotide sequences, were proven to be much more useful than immunofluorescence in terms of type-specific diagnosis of HSV infections.

Base Sequence

Detection of HIV-1 RNA in heparinized plasma of HIV-1 seropositive individuals.

The interference of reverse transcription by heparin was removed by heparinase. When the HIV-1 RNA in the presence of heparin was detected by a combination of reverse transcription and the polymerase chain reaction (PCR), heparinase treatment followed by removal of Ca2+ before the reverse transcription step permitted the efficient detection of HIV-1 RNA. Prior treatment with heparinase revealed HIV-1 RNA in 68% (13/19) of heparinized plasma samples from HIV-1 carriers, whereas only 26% (5/19) of the same specimens were positive without the heparinase step. Heparinase removed the inhibition of reverse transcription by heparin and is highly recommended when detecting low levels of viral RNA in heparinized plasma.

HIV Reverse Transcriptase

A prospective study on correlation between the decrease in anti-P17 antibody level and progression to AIDS in asymptomatic carriers of HIV.

As the majority of human immunodeficiency virus (HIV) carriers are in asymptomatic stage for a long period of time, it is important to investigate the factors or surrogate markers for conversion from asymptomatic to symptomatic stage. Our study is designed to evaluate the relationship among virus isolation rate, anti-p17 antibody status and progression to AIDS. We studied anti-p17 antibody status along with virus isolation in 56 asymptomatic carriers and 46 AIDS cases. Progression to AIDS was markedly associated with high rate of virus isolation and loss of anti-p17 antibody. In order to know the meaning of loss of anti-p17 antibody during the clinical course, 15 anti-p17 antibody positive and 16 anti-p17 antibody negative cases were followed up prospectively for the development of AIDS. None of the anti-p17 antibody positive cases developed AIDS while 6 out of 16 anti-p17 negative cases developed AIDS during observation period (P < 0.05). Progression to AIDS was associated with loss of anti-p17 antibody. Identification of cases losing anti-p17 antibody in peripheral blood during asymptomatic period may help high-risk group who are in need of chemoprophylaxis. Moreover, study of anti-p17 antibody may be helpful in designing vaccine in future if it works as a neutralizing antibody to HIV in vivo.

Acquired Immunodeficiency Syndrome

Langerhans-cell histiocytosis in an adult patient with multiple myeloma.

A 44-year-old man who had suffered for 6 years from multiple myeloma developed multiple papules on the face and chest. Histological examination of these papules revealed the infiltration of predominantly histiocytic cells into the dermis and into parts of the epidermis. These cells were seen on electron-microscopic study to have Langerhans granules in the cytoplasm, which led to a diagnosis of Langerhans-cell histiocytosis concomitant with multiple myeloma. Possible explanations for this unusual association are discussed.

Adult

[A case of chronic myelogenous leukemia with pulmonary aspergillosis diagnosed by the detection of circulating Aspergillus antigen].

Immunocompromised hosts usually develop invasive mycotic disease. Among many pathogenic fungi. Aspergillus spp, is the most common pathogen of respiratory infection. Early diagnosis of invasive type pulmonary aspergillosis is still difficult, and the treatment is usually difficult. Many investigations have recently suggested that detection of Aspergillus antigen from sera of the patients is useful for early diagnosis to save their lives. We have experienced a case diagnosed by the detection of circulating Aspergillus antigen by Pastorex Aspergillus, who was a 64-year-old female with the blastic crisis chronic myelogenous leukemia. After anti-leukemic chemotherapy, she suffered from pneumoniae with pleural effusions and severe hypoxia, which did not respond to antibiotics. At this point, her serum sample showed positive Aspergillus antigen by Pastorex Aspergillus. She was treated by intensive antifungal chemotherapy, and thereafter improved quickly. Titers of Pastorex Aspergillus were well correlated with her clinical course. The sensitivity of the test requires further improvement, but the specificity of the test is considered to be high enough for clinical use.

Antigens, Fungal

[The evaluation of pre and intraoperative factors influencing the false lumen after graft replacement surgery to the extended dissecting aneurysm].

We evaluated the residual false lumen of type I and IIIb dissecting aneurysm by CT, MRI and angiography postoperatively. The 19 patients with type I dissecting aneurysm were included eleven men and eight women, the average age was 55.8 +/- 10.2 years old. The 20 patients with type IIIb dissecting aneurysm were included sixteen men and four women, the average age was 56.2 +/- 8.5 years old. The rate of distal patent false lumen was 52.6% of type I and 35% of type IIIb dissecting aneurysm after graft replacement surgery. In type I dissecting aneurysm, the rate of distal patent false lumen was 40% of acute stage vs 66.7% of chronic stage, 66.7% of ascending and partial arch replacement vs 46.2% of ascending and total arch replacement, and 90% of graft inclusion technique vs 11.1% of graft exclusion technique. The distal patent false lumen was the lowest (12.5%) with type I dissecting aneurysm of ascending and total arch replacement using graft exclusion technique. In type IIIb dissecting aneurysm, the rate of patent false lumen was 66.7% of acute stage vs 29.4% of chronic stage, 30% of graft exclusion technique vs 40% of graft inclusion technique. The size of false lumen preoperatively were larger (11.1 +/- 4.5 cm2) in patients with distal patent false lumen than that (6.7 +/- 3.2 cm2) of in patients with distal occlusive false lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

[Effects of blood-diltiazem-nitroglycerin-cardioplegia in coronary artery bypass grafting].

Forty-seven patients who underwent coronary artery bypass grafting using blood cardioplegia (BCP) were studied clinically. They were divided into 3 groups based on concentration of diltiazem (DTZ) and nitroglycerin (NTG). Group I (n = 12); DTZ 5 mg plus NTG 5 mg in BCP 1,000 ml, Group II (n = 10); NTG 25 mg in BCP 1,000 ml, Group III (n = 25); DTZ 5 mg plus NTG 25 mg in BCP 1,000 ml. From the standpoint of serum enzyme analysis, there was no significant difference between 3 groups, however, the incidence of perioperative myocardial infarction (PMI) and perioperative coronary spasm (PCS) were significantly higher in Group I than that in other groups. The present data suggests that intramyocardial concentration of NTG in Group II and III were 5 times higher than Group I. Increased dose of NTG in BCP would be benefit for myocardial protection in CABG.

Adult

[Cardiac performance in total anomalous pulmonary venous connection].

Cardiac performance in 54 patients with total anomalous pulmonary venous connection was investigated by cardiac catheterization before and after surgery. 51 patients underwent intracardiac repair, and 17 of them died during or immediately after operation. According to the preoperative study, the left ventricular ejection fraction (LVEF) of surviving patients was significantly higher than that of patients who died, and the pulmonary arterial mean pressure of surviving patients was significantly lower than that of patients who died. However, there was no significant difference between the left ventricular end-diastolic volume (LVEDV), right ventricular ejection fraction (RVEF), and right ventricular end-diastolic volume (RVEDV) in surviving patients and those who died. Post-operative catheterization studies showed significant increases of LVEF and LVEDV compared to pre-operative figures. RVEF and RVEDV and pulmonary arterial mean pressure decreased significantly after surgery. It was concluded that preoperative cardiac performance of surviving patients was better than that of those who died, and post-operative cardiac performance of surviving patients was basically normal.

Blood Pressure

[Concomitant graft replacement of the total aortic root and the transverse aortic arch for type A aortic dissection associated with annuloaortic ectasia].

Between April 1988 and February 1992, eight patients with type A aortic dissection associated with annuloaortic ectasia (AAE) underwent the concomitant graft replacement of the total aortic root and the transverse aortic arch at our institution. The acuity of the aortic dissection was acute stage in 3 patients and chronic stage in 5 including 3 cases of re-do operation. All operations were performed with an aid of extracorporeal circulation, blood cardioplegia, selective cerebral perfusion and open distal anastomosis. The operative techniques employed in this series consisted of total aortic root replacement using a composite graft (Bentall, Cabrol or Piehler's technique), and total arch replacement using en bloc arch reconstruction or three vessels graft replacement. One patients underwent re-do operation for coronary anastomotic false aneurysm following Bentall operation and aneurysmal dilatation of the false lumen at the aortic arch, and died of LOS because of the prolonged myocardial ischemia. Other seven patients survived the operation, and lead the normal life at the present time. The present data suggests that type A aortic dissection associated with AAE involving aortic arch could be treated by concomitant graft replacement of the total aortic root and the transverse aortic arch.

Adult

[Surgical treatment of Marfan's syndrome with annulo aortic ectasia and mitral regurgitation].

This report is concerned with results of surgical treatment for Marfan's syndrome combined with annulo-aortic ectasia (AAE) and mitral regurgitation (MR). Of the 23 patients with Marfan's syndrome who received Bentall's procedure during 14 year period, seven (30%) of these patients had both AAE and MR. The MR grade of seven patients by cardiac Doppler or left ventriculographic studies were grade 1 in 2, 2 in 1, 3 in 1, and 4 in 3. Atrial fibrillation was present in 4 patients. New York Heart Association Functional Class on admission in these 7 patients were II in 1, III in 4, and IV in 2. The mitral valve was replaced with mechanical valve in 4 patients by left atrial approach whose MR grade were over 3. In the 4 patients the mitral annuli were extremely dilated, both valve leaflets were massively redundant, and all chordae were elongated and turned chordae and vegetation were detected due to infective endocarditis. Only Bentall's procedure was performed in 3 patients whose MR were minimal. There were no early death, but two late deaths. One of them died of cardiac failure 2.3 years after Bentall's procedure because grade 2 MR was increased. Another one died from ventricular arrhythmia 1.6 years after MVR and Bentall's procedure. The remaining 5 patients are doing well for 3 months to 11.5 years after operation. For Marfan's syndrome combined with AAE and MR, early operation is recommended before left ventricular impairment. Mitral valve repair was not performed, both to save time and because anticoagulant therapy was need for aortic valve replacement. Concomitant MVR was to be done for moderate to severe MR.

Adult

[Abnormalities of beta spectrin with hereditary elliptocytosis in mother and child].

It is generally considered that abnormality of the erythrocyte membrane skeleton co elliptocytes. There are, however, few reports of beta spectrin variants. We found a new variant of beta spectrin in a child and her mother. This report is the first case of abnormality of beta spectrin in Japan. The propositus was an 8 month-old girl who was first examined by us in 1988. On laboratory findings, she showed anemia, increased reticulocyte count and decreased haptoglobin concentration. Both peripheral blood smears of patient and her mother showed typical elliptocytosis and they were diagnosed as hereditary elliptocytosis. SDS-PAGE patterns of the red cell membranes of the propositus and her mother were characterized by the presence of an abnormal component migrating immediately below the spectrin chains. We confirmed that the abnormal spectrin appeared clearly at the expense of normal beta chain. The abnormal spectrin (M.W. 216,000d) makes up 16% of the total beta chain. The inheritance of our case was autosomal dominant. The present case is considered as a new spectrin variant.

Adult

Physiologic and anatomic assessment of patients with rectocele.

Clinical, physiologic, and anatomic assessments were carried out in 22 female patients with symptomatic rectocele (Group A), 15 patients with asymptomatic rectocele (Group B), and 14 subjects having no rectocele (Group C). Resting and pressure, rectal pressure, rectal compliance, anorectal inhibitory reflex, and rectal sensation did not differ among the groups. Proctography revealed that the lengths of the rectocele during attempted defecation in groups A (1.6 [1.0-3.5] cm) (median and range) and B (1.6 [1.0-3.0] cm) were significantly greater than that in Group C (0.4 [0.1-0.9] cm) (P less than 0.001 in both groups). Median pelvic floor descent at rest in Groups A (4.3 [1.6-7.5] cm) (median and range) and B (4.3 [1.3-6.9] cm) were significantly greater than that in Group C (2.5 [1.2-5.0] cm) (P less than 0.001 and P less than 0.02, respectively). These results indicate that rectocele is not associated with any physiologic change apart from a significant increase of pelvic floor descent.

Adult

Aortic valve replacement with omniscience and omnicarbon valves.

Clinical results achieved in 100 cases of aortic valve replacement with the Omniscience (O-S) valve during the period from 1980 to 1985 as well as 100 cases of aortic valve replacement with the Omnicarbon (O-C) valve during the period from 1985 to 1989 were studied. Concomitant surgical procedures including mitral valve replacement were performed in 63 patients in the O-S group and 67 patients in the O-C group. Cumulative follow-up in the two groups was carried out for a total of 559 and 273 patient-years, respectively. The overall 4-year actuarial survival rate was 82% +/- 3.8% in the O-S group and 89.5% +/- 3.2% in the O-C group, the corresponding rates for patients undergoing isolated aortic valve replacement being 82.9% +/- 4.2% in the O-S group and 91.9% +/- 3.5% in the O-C group. The overall 4-year actuarial event-free rate with respect to thromboembolic complications was 88.8% +/- 3.3% in the O-S group and 94.4% +/- 2.8% in the O-C group, as compared with the corresponding rates of 89.2% +/- 3.6% in the O-S group and 95.9% +/- 2.8% in the O-C group for patients undergoing isolated aortic valve replacement. The overall rate of valve-related complications, including thromboembolism, anticoagulant-related hemorrhage, perivalvular leak, infection, and structural failure, was 78.8% +/- 4.2% in the O-S group and 89.3% +/- 3.5% in the O-C group (p less than 0.05), and for isolated aortic valve replacement, 79.7% +/- 4.5% in the O-S group and 89.6% +/- 4.1% in the O-C group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Contrasuppressor T cell leukaemia: clonal proliferation of contrasuppressor T cells in a patient with granular lymphocyte-proliferative disorder.

In 12 patients with granular lymphocyte-proliferative disorders (GLPD), we studied the capacity of patient peripheral blood mononuclear cells (PBMC) to promote or suppress polyclonal IgG synthesis by normal non-T cells in pokeweed mitogen-containing medium using an enzyme-linked immunosorbent assay. During the experiments we found a patient whose PBMC possessed contrasuppressor function. The patient was a 27-year-old female with anaemia and lymphocytosis of CD3+CD8+ granular lymphocytes (GL). Reconstitution experiments using normal donor non-T cells and CD4+ and CD8+ T cells showed that addition of the patient's CD8+ cells abrogated the suppressor cell function of normal CD8+ T cells. The patient's PBMCs were CD3+, CD8+, Ia+, and Vicia villosa lectin-adherent characteristics which are consistent with those of normal blood contrasuppressor T cells. The T cell receptor beta and gamma genes were found to be monoclonally rearranged. Ultrastructurally, this patient's GLs exhibited clusters of dense cytoplasmic bodies, which were not detected in the GL of other patients with GLPDs. These results indicate that the clonal proliferation of contrasuppressor T lymphocytes had occurred in this patient.

Adult