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Biomedical subjects

Y Sakiyama

Publications and source records attributed to Y Sakiyama.

At least 91 records · Page 5Linked to original sources
PubMed1994

Good's syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis.

A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.

B-Lymphocyte Subsets↗
PubMed1994

[A clinical study of fluconazole in the treatment of systemic mycosis associated with immunocompromised children].

Efficacies of fluconazole (FLCZ) were evaluated in 8 cases of systemic mycosis (1 case each of candidemia, candiduria, 4 cases of pulmonary candidiasis, and 2 cases of suspected fungemia) complicated in immunocompromised children. Enrolled in the study were 3 patients with acute leukemia, 1 with malignant lymphoma, 2 with congenital immunodeficiency syndrome, 2 with other disorders. The clinical efficacies were good in 6 out of 7 cases. No side effects were observed. FLCZ is a very good and safe agent for the treatment of systemic mycosis complicated with immunocompromised children.

Candidiasis↗
PubMed1993

Fatal Epstein-Barr virus-associated hemophagocytic syndrome.

A virus-associated hemophagocytic syndrome is characterized by high fever, liver dysfunction, coagulation abnormalities, pancytopenia, and a benign histiocytic proliferation with prominent hemophagocytosis in bone marrow, lymph node, spleen, and liver. We describe six Japanese children with fatal Epstein-Barr virus (EBV)-associated hemophagocytic syndrome. Five of the six patients had serologic evidence of primary EBV infection at the onset of their diseases. EBV genomes were detected in all the patients by Southern blot hybridization or the polymerase chain reaction. Furthermore, clonality analysis of the EBV genome showed that EBV-infected cells proliferated monoclonally or biclonally in three examined patients. In situ hybridization study using EBV-encoded RNA 1 (EBER1) showed that EBER1 was detected in one of two examined liver tissues, which localized in hepatocytes.

Base Sequence↗
PubMed1993

Distinct clonotypic Epstein-Barr virus-induced fatal lymphoproliferative disorder in a patient with Wiskott-Aldrich syndrome.

BACKGROUND: Recently, reports of Epstein-Barr virus (EBV)-induced lymphoproliferative disorders (LPD) have increased in number among immunosuppressed recipients of organ transplants. The importance of analyzing both the immunoglobulin gene and EBV termini is advocated for the investigation of pathogenetic mechanisms for clonal proliferation in EBV-induced LPD; however, the oncogenic mechanisms of EBV-induced LPD remain unclear. Furthermore, there are very few clonotypic studies of EBV-induced LPD in patients with primary immunodeficiency diseases. The authors studied the clonality of an EBV-induced fatal LPD in a 20-year-old patient with Wiskott-Aldrich syndrome (WAS), an X-linked recessive primary immunodeficiency disease. METHODS AND RESULTS: An autopsy showed non-Hodgkin lymphoma of B-cell origin with diffuse large cells in both systemic lymph nodes and extranodal organs. Immunohistochemical and Southern blot analyses showed polyclonal rearrangement of immunoglobulin genes in most of the lesions except for the pulmonary hilar lymph node. Furthermore, the analysis of restriction fragment length polymorphism with several fragments from EBV genome indicated that EBV genomes in all lesions were identical; however, a single but different-sized EBV termini was detected in every EBV-positive lesion when probed with the EcoRI-Dhet spanning terminal repeat region of EBV. CONCLUSIONS: The EBV-induced fatal LPD in a patient with WAS showed the characteristic clonotype, polyclonal immunoglobulin gene rearrangement, and monoclonal EBV terminal configuration. Furthermore, EBV termini in each lesion varied in size. This particular clonotype implicates several unique pathogenetic mechanisms for clonal proliferation of EBV-induced LPD.

Adult↗
PubMed1993

A newly recognized point mutation in the cytochrome b558 heavy chain gene replacing alanine57 by glutamic acid, in a patient with cytochrome b positive X-linked chronic granulomatous disease.

Molecular genetic analysis was performed in a patient with cytochrome b positive X-linked chronic granulomatous disease. A previous Southern blot study, using a cytochrome b heavy chain cDNA as probe, revealed a Pst I restriction fragment pattern for the cytochrome b heavy chain gene (CYBB) different to that of normal individuals. Since restriction length polymorphism with Pst I has never been observed in control individuals and no abnormal restriction fragment patterns in the patient's CYBB was detected with seven other enzymes used, we focussed on the single Pst I site in the CYBB cDNA as being the only mutation site responsible for his disease. A fragment of the patient's cDNA which included the Pst I site was amplified by reverse polymerase chain reaction, and loss of the Pst I site in the fragment was confirmed by incubation with Pst I. Subsequent sequence analysis of the fragment revealed a point mutation in the Pst I site (cytosine to adenine), substituting glutamic acid for alanine at position 57.

Alanine↗
PubMed1993

A Japanese family of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy.

Three cases of X-linked auto-immune enteropathy with haemolytic anaemia and polyendocrinopathy are described from one related Japanese kindred. Two boys had died due to severe diarrhoea accompanied by total or subtotal intestinal villous atrophy. In contrast, although one patient showed the same symptoms and had circulating IgG antibodies against enterocytes, his condition improved dramatically and he developed well following the use of cyclosporin A (CSA). CSA may be beneficial in patients with this rare disorder. Auto-immune enteropathy should be considered as a cause of protracted diarrhoea with unknown aetiology.

Anemia, Hemolytic↗
PubMed1993

Detection of Epstein-Barr virus DNA in cardiac and aortic tissues from chronic, active Epstein-Barr virus infection associated with Kawasaki disease-like coronary artery aneurysms.

We describe three patients with chronic, active Epstein-Barr virus infection associated with Kawasaki disease-like coronary artery aneurysms. The Epstein-Barr virus genome was detected in three cardiac tissue samples and one aortic tissue sample examined by means of the polymerase chain reaction. These findings suggest that chronic Epstein-Barr virus infection may play a pathogenic role in the development of coronary artery aneurysms.

Antibodies, Viral↗
PubMed1993

A study of the binding of immunoglobulin G and immunoglobulin E from children with bronchial asthma to peptides derived from group II antigen of Dermatophagoides pteronyssinus.

The peptides that were produced by the treatment of the purified group II antigen of Dermatophagoides pteronyssinus with cyanogen bromide, lysylendopeptidase, Staphylococcus aureus V8 protease, or N-tosyl-L-phenylalanylchloromethyl ketone-treated trypsin were examined for their binding with IgG and IgE from children with bronchial asthma by immunoblotting analysis. Both IgG and IgE bound to the peptides from the amino terminus to the 76th residue and the carboxyl terminal side from the 13th or 15th residue; on the other hand, both Ig failed to bind to the carboxyl terminal side from the 39th, 56th, or 77th residue. The data demonstrated that peptides lacking residues 1 to 15 possessed the ability to bind Ig, whereas those lacking residues 1 to 39 had lost it. The present study indicates that the amino terminal half of the group II antigen of Dermatophagoides pteronyssinus is more important for the binding of IgG and IgE than the carboxyl terminal half and suggests that the residues 15 to 39 may be directly related to the binding of both IgG and IgE.

Adolescent↗
PubMed1993

[A clinical study of fluconazole-granules and -injectable in pediatric patients with deep-seated mycoses].

Fluconazole (FLCZ) is an antifungal agent of triazole class developed by Pfizer, Inc. Its oral and injectable forms have been available on the market since June 1989 in Japan. FLCZ exhibits potent antifungal activities against Candida spp., Aspergillus spp. and Cryptococcus spp. and, as orally or intravenously administered, is widely distributed into organs and tissues. For its low protein binding rate of about 10 per cent and long serum half life of about thirty hours in adults, FLCZ has been proved highly effective and useful in the treatment of deep-seated mycosis in adult patients. In the present study, we have investigated the clinical effectiveness and antifungal activities of FLCZ granules, a new dosage form of the drug, and of intravenous form in pediatric patients with deep mycosis. A total of 72 patients were treated either with granules orally or with intravenous injection and 47 patients among them were evaluable on the clinical efficacy of the drug. Also, a study on the pharmacokinetics of pediatric patients including premature/new born babies was conducted employing multiple dose regimens in a total of 27 patients. The clinical efficacy rates were 79.5% (35 patients out of 44) in candidiasis and 100.0% (3 of 3) in aspergillosis. The safety of the drug was assessed in 63 patients. No side effects were observed. Clinical laboratory test abnormalities were observed in some patients with an incidence of 9.7% (6 patients out of 62) but most of the abnormalities were only mild and transient. The pharmacokinetics at repeated doses indicated that a steady-state is reached in 4 days after the initial administration of either granules or intravenous form. From these results, it may be concluded that FLCZ is a very useful medication in the treatment of deep mycosis in pediatric patients.

Administration, Oral↗
PubMed1992

Cytochrome b positive X-linked chronic granulomatous disease: a normal cell surface expression of cytochrome b.

The polymorphonuclear (PMN) cells from a patient with cytochrome b positive X-linked chronic granulomatous disease (Xb+ CGD) were studied using flow cytometry. Both the cell surface expression of monoclonal antibody defined cytochrome b and the superoxide production (intracellular 2',7'-Dichlorofluorescin Diacetate oxidation) were investigated at a single cell level. Flow cytometry clearly demonstrated the complete absence of superoxide production in the patient's PMN cells, the mosaicism in his mother's PMN cells and also indicated the normal cell surface expression of cytochrome b. The results obtained by Western blot analysis and reduced-minus-oxidized spectra confirmed the presence of functional and normal amounts of cytochrome b. We concluded that this is a case of Xb+ CGD with a normal cell surface expression of cytochrome b.

Adolescent↗
PubMed1992

The effect of benzodiazepines on the binding of [3H]SCH 23390 in vivo.

The effect of flunitrazepam upon the binding of [3H]SCH 23390 in vivo was investigated. Acute treatment with flunitrazepam decreased the binding of [3H]SCH 23390 in the striatum in a dose-dependent manner. The time course of radioactivity in the striatum, cerebral cortex and cerebellum in controls and flunitrazepam (1 mg/kg)-treated mice was measured after intravenous injection of [3H]SCH 23390. The binding kinetics were calculated, using the cerebellum as a reference region for the estimation of the amount of free ligand in the brain. Flunitrazepam significantly decreased the input rate constant to the receptor compartment and the dissociation rate constant in vivo. An in vivo displacement study, using carrier SCH 23390, also showed significant reduction in the dissociation rate constant of [3H]SCH 23390 in vivo. The drug Ro 15-1788 reversed the effect of flunitrazepam, suggesting that this reduction in binding of [3H]SCH 23390 was mediated by benzodiazepine receptors. To evaluate the relationship between the reduction in binding of [3H]SCH 23390 in vivo and in vivo occupancy of benzodiazepine receptors, in vivo occupancy of benzodiazepine receptors was measured using [3H]Ro 15-1788. A non-linear relationship was found between the reduction in dopamine D1 receptor binding in vivo and the occupancy of benzodiazepine receptors in vivo, indicating that benzodiazepines exerted the maximum change in dopamine receptor binding at a low fractional occupancy of receptors.

Animals↗
PubMed1992

Progress in primary immunodeficiency.

Clinically, primary immunodeficiencies can be grouped into several well-defined syndromes. This consistent clinical picture, however, belies the enormous complexity of lymphocyte maturation and activation, and it has long been suspected that numerous distinct underlying defects give rise to primary immunodeficiencies. At a recent workshop, the molecular and genetic characterization of a handful of such defects provided tacit support for such suspicions.

Adenosine Deaminase↗
PubMed1992

Eosinophil cationic protein in peripheral blood of pediatric patients with allergic diseases.

We have studied the levels of eosinophil cationic protein (ECP) and tumour necrosis factor (TNF) in peripheral blood obtained from 68 children with bronchial asthma and 11 children with atopic dermatitis. The ECP mean concentrations of the patients were 23.7 +/- 21.4 micrograms/l and 21.2 +/- 18.7 micrograms/l for bronchial asthma and atopic dermatitis respectively, which were significantly higher than the control value, 5.8 +/- 2.3 micrograms/l (P less than 0.005). TNF was unmeasurable in almost all the samples and no significant difference was observed between normal controls and asthmatic children. A significant correlation was observed between serum levels of ECP and blood eosinophil counts in both diseases (r = 0.873; P less than 0.01 and r = 0.740; P less than 0.01, respectively). However, no obvious correlation was observed between serum levels of ECP and IgE levels. ECP levels were significantly reduced by treatment and normalized in parallel with blood eosinophil counts in the patients with total IgE levels less than 800 U/ml. Irrespective of the total IgE levels, the reduction of serum ECP levels was correlated with a decrease in the number of asthmatic attacks and/or improvement of pulmonary function. These results suggest that the ECP levels in peripheral blood indicate an increased activity of eosinophil and would be a more useful marker than eosinophil counts for making clinical analyses and estimating treatment efficacy in paediatric patients with allergic diseases.

Adolescent↗
PubMed1992

Hyper-response of serum IgG1 to Staphylococcus aureus peptidoglycan in patients with hyper-IgE syndrome.

The hyper-IgE (HIE) syndrome is characterized by high IgE serum levels, chronic dermatitis and recurrent infections. To determine whether an impairment of the antibody response to Staphylococcus aureus contributes to infections in this syndrome we measured total serum IgG subclass, specific IgG1 and IgG2 levels against peptidoglycan (PG), the immunodominant cell wall component of S. aureus and serum opsonic activity to PG. Of the 14 patients with HIE syndrome, nine had increased level of serum IgG1 and six had IgG2 subclass deficiency. In regard to specific response of IgG1 and IgG2 antibodies to PG, patients were divided into five groups related to ages and compared with 10 control subjects for each age cohort. Patients with HIE syndrome had significant high levels of serum-specific IgG1 to PG and significant decreased levels of serum-specific IgG2 to PG in all five groups. Additionally, serum opsonic activity in patients was significantly higher than that in normal control subjects. It is concluded that IgG2 deficiency or poor IgG2 antibody response to S. aureus is not the explanation of the abnormal susceptibility to S. aureus infections of HIE patients.

Adolescent↗
PubMed1992

Primary immunodeficiency diseases and Epstein-Barr virus-induced lymphoproliferative disorders.

Increased incidence of malignant disorders is noted in patients with both primary and acquired immunodeficiency diseases. The pathogenetic mechanism(s) for these disorders remain unclear. Defective immunosurveillance of these patients, however, is mainly postulated to be responsible for the increased risk of these malignant disorders. Of the malignant disorders, Epstein-Barr virus (EBV)-induced lymphoproliferative disorders (LPD) have been increasingly reported, possibly due to improved therapeutic management techniques such as bone marrow transplantation, which results in prolonged survival periods for the primary immunodeficiency; the dramatic development of immunosuppressive treatments for transplant recipients; and the growing numbers of acquired immunodeficiency syndrome (AIDS) patients. This review focuses on the primary immunodeficiency diseases and EBV-induced LPD, and discusses pathogenetic mechanism(s) for the increased incidence of these malignant disorders.

Adolescent↗
PubMed1992

Phenytoin-induced IgG2 and IgG4 deficiencies in a patient with epilepsy.

A five-year-old girl with epilepsy and recurrent respiratory infections was investigated for serum IgG subclass concentrations. She was diagnosed as having a combined deficiency of IgG2 and IgG4 with a decreased serum concentration of IgA and IgG3 and was given replacement therapy with i.v. immunoglobulins. Since then, she has been free from respiratory infections. After phenytoin therapy was stopped, IgG subclass deficiency improved. This case describes the further action of phenytoin on the immune system, adding IgG subclass deficiency to the list.

Child, Preschool↗
PubMed1992

Genetic heterogeneity in patients with X-linked recessive chronic granulomatous disease.

Genetic heterogeneity in 12 patients from 11 different families with X-linked recessive chronic granulomatous disease was studied by Southern blot analysis using cytochrome b heavy-chain cDNA as a probe. We found the abnormal restriction length fragment patterns of the cytochrome b heavy-chain gene in three families, which were not observed in healthy controls. DNA from one patient showed the abnormal patterns after digestion with several restriction enzymes. The DNA of two other patients showed the abnormality only with TaqI and PstI. Analysis of the same family members indicated that these abnormal patterns cosegregated with the disease. The other nine patients from eight families did not have any abnormalities detectable by Southern blot analysis. Although further experimentation should be done to study the molecular genetic heterogeneity in most X-linked chronic granulomatous disease families (eight of 11), we were able to demonstrate at least three different types of mutations in the cytochrome b heavy-chain gene responsible for the disease.

Adolescent↗