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Biomedical subjects

A Hoshika

Publications and source records attributed to A Hoshika.

At least 19 recordsLinked to original sources

Alkaline phosphatase, defensin gene expression and effect of myeloid cell growth factors in normal and leukemic cells.

In neutrophils, four different granules are defined, i.e. azurophil, specific, gelatinase and secretary vesicles. In these granules many neutrophil-specific constituents are identified. Some of these constituents have already been cloned and their gene expressions studied. In such constituents, alkaline phosphatase (ALP) and defensin are well known, although their functions are not yet fully clarified. ALP is present in secretary vesicles and has important roles in the diagnosis of some myeloid disorders. On the other hand, defensin is the most abundant functional peptide of neutrophils and is present in azurophillic granules. Which are subdivided into defensin-rich and defensin-poor granules. This review describes the expression of ALP and the defensin gene in normal and leukemic cells and the effect on these genes of myeloid growth factors, such as granulocyte colony-stimulating factor, granulocyte-macrophage colony-stimulating factor and interleukin 3.

Alkaline Phosphatase

Myeloperoxidase gene expression and regulation by myeloid cell growth factors in normal and leukemic cells.

Myeloperoxidase (MPO) is present in azurophilic granules which appear in the promyelocyte stage of differentiation, and is the most common functional protein of myeloid cells. With progress in molecular biology, the expression and regulation of MPO have been clarified in normal myeloid and leukemic cells, not only by enzymatical activity but at the gene level MPO expression is affected by the differentiation of myeloid cells, and has been suggested to be regulated by myeloid cell growth factors, such as granulocyte colony-stimulating factor, granulocyte-macrophage colony-stimulating factor and interleukin-3. In the past decade the signal transduction from their receptors has been clarified. This review describes the expression and regulation of the MPO gene in myeloid cells including myeloid disorders, such as myeloid leukemia or myelodysplastic syndromes, The effects on MPO by myeloid growth factors and signal transduction from their receptors are also presented.

Bone Marrow Cells

Usefulness of nested PCR and sequence analysis in a nosocomial outbreak of neonatal enterovirus infection.

BACKGROUND: Non-polio enterovirus infections are recognized in children during summer-fall seasons and they sometimes cause large outbreaks. We experienced a nosocomial infection in the neonatal nursery and echovirus type 7 was isolated from samples of four patients. OBJECTIVES: We diagnosed the horizontal infection of four neonates by reverse transcriptase-nested polymerase chain reaction (RT-nested PCR) and the nucleotide sequence. STUDY DESIGN: Total RNA was extracted from clinical isolates, serum samples and cerebrospinal fluid (CSF). We amplified enterovirus genome in the 5'-noncoding region by nested PCR and determined the nucleotide sequences. RESULTS: Enterovirus genome was detected in all isolates, in the acute-phase sera in all four patients and in the CSF in one patient by the first PCR. By using nested PCR, the genome was detected from convalescent-phase sera in two patients. All enterovirus genome obtained from the nursery outbreak showed the same sequences with 100% homology. CONCLUSION: We demonstrated the clinical advantages of RT-nested PCR from serum samples and the analysis of nucleotide sequencing gave the supportive evidence of identification of transmission pathway.

5' Untranslated Regions

Telomeric length and telomerase activity vary with age in peripheral blood cells obtained from normal individuals.

The telomerase activity and length of telomeres of peripheral blood mononuclear cells obtained from 124 healthy individuals aged 4-95 years was measured. Telomerase activity level was semiquantitatively assessed by a fluorescent-telomeric repeat amplification protocol (fluorescent-TRAP) using an internal telomerase assay standard, fluorescent primers and an automated laser fluorescent DNA sequencer. Telomeric length, measured by assay of terminal restriction fragments (TRFs), was determined in HinfI-digested DNA by Southern blot analysis using a (TTAGGG)4 probe. TRF length was determined in 80 individuals and age-related progressive reduction of size was observed. TRF length in peripheral blood mononuclear cells obtained from normal individuals (aged 4-39 years) decreased by approximately 84 bp per year, while in individuals aged > or = 40 years it decreased by 41 bp per year. In contrast, telomerase activity showed an apparent biphasic pattern with aging. Individuals aged 4-39 years showed a progressive decrease in telomerase activity, whereas 65% of those aged > or = 40 years showed relatively stable but very low telomerase activity, and the remaining individuals aged > or = 40 years had no detectable telomerase activity. These data obtained from normal individuals might in the future be of value to help risk stratify and manage the care of patients with leukemia.

Adolescent

[A case of multiple sclerosis with initial symptoms of narcolepsy].

We reported a 13-year-old boy diagnosed as multiple sclerosis associated with narcolepsy. He had suffered from retrobulbar optic neuritis at the age of 11 years which was improved gradually by prednisolone. Four months later he was admitted because of excessive somnolence. The diagnosis of narcolepsy was made based on hypnagogic hallucination, sleep paralysis, changes of personality and the sleep onset sREM (SOREM). The elevation of anti-measles antibody and a positive oligoclonal band in CSF, low density areas in the bilateral internal capsule on CT scan, and high signal areas in the same region on T2-weighted MRI confirmed the diagnosis of multiple sclerosis. An abnormal arousal response occurred occasionally in sleep stage 2 and 4, which started with electrical silence followed by a period with irregular high voltage slow waves and repetitive focal spike train. Those clinical symptoms and abnormalities of biochemical and electrophysiological studies normalized after treatment with prednisolone. However, abnormalities on MRI showed no improvement even after long term administration of prednisolone (2.5 mg/day).

Adolescent

Detection of mumps virus genome directly from clinical samples and a simple method for genetic differentiation of the Hoshino vaccine strain from wild strains of mumps virus.

A simple and sensitive method was developed for the differentiation of the Hoshino vaccine strain from wild strains with a restriction fragment length polymorphism (RFLP) analysis in the part of hemagglutinin-neuraminidase (HN) gene. The virus genome was amplified by using a reverse transcriptase-polymerase chain reaction (RT-PCR) directly from clinical samples. The PCR product of the Hoshino vaccine strain was cleaved into 2 fragments after digestion with Sca I and Afl II. All wild strains showed 2 RFLP profiles, A and B, different from that of vaccine strain. Wild A strains were cut into 2 fragments after digestion with Sca I only, while wild B strains were cleaved neither with Sca I nor Afl II. This molecular approach provides an effective method for differentiation of the Hoshino vaccine strain from wild strains of mumps virus in patients after vaccination.

Animals

Polymerase chain reaction detection of the hemagglutinin gene from an attenuated measles vaccine strain in the peripheral mononuclear cells of children with autoimmune hepatitis.

We examined the measles H gene using reverse transcriptase-polymerase chain reaction in peripheral mononuclear cells obtained from 4 pediatric and 2 adult patients with autoimmune hepatitis and 12 healthy children who had been infected with measles or vaccinated with an attenuated measles vaccine in the past. All patients were positive for the presence of the gene. Only one healthy control, who had been vaccinated two weeks before the study, was positive, while the other 11 controls were negative for the presence of the gene. The restriction enzyme patterns of the products in the pediatric patients were different from those observed in adults. The sequences of amplified products from pediatric patients coincided with the vaccine strain, whereas those from adults were different from the vaccine strain. The sequence of those from one of two adult patients was similar to those of the isolates in 1990 and later. Our results demonstrated that children with autoimmune hepatitis can have persistence of the vaccine strain in vivo for many years after vaccination.

Autoimmune Diseases

[A 10-year-old girl with anticardiolipin antibody, who showed bleeding tendency].

We described a 10-year-old girl with SLE complicated with anticardiolipin antibody, who showed bleeding tendency since infant. Her platelet was 6 x 10(3)/microliter. The APTT was remarkably prolonged. The platelet count was normalized after steroid treatment. A false positive Venereal Disease Research Laboratory (VDRL) test, lupus anticoagulants and anticardiolipin antibodies (IgG and IgM) were positive. Her symptoms and the laboratory data fulfilled the criteria of antiphospholipid syndrome. Renal biopsy was performed because of proteinuria, hematuria and the positive of anti-nuclear antibody. The pathology of kidney showed that of lupus nephritis (diffuse membranous glomerulonephritis), and she was diagnosed SLE. She showed no laboratory findings of hyperfibrinolysis and no symptoms of thrombosis under the steroid treatment for 3 years.

Antibodies, Anticardiolipin

Non-convulsive status epilepticus.

The clinical, electrographic and reported neuropsychological features of 50 children with non-convulsive status epilepticus (NCSE) were reviewed and the children's progress followed for one to five years. NCSE occurred in a variety of epilepsies, especially the Lennox-Gastaut syndrome. Clinical manifestations ranged from obvious mental deterioration to subtle changes. The condition had often been overlooked or misinterpreted and many children had experienced repeated episodes over long periods. Following diagnosis, immediate treatment was often not attempted or was not successful. Further episodes of NCSE occurred in the majority of children during the follow up period. Failure to recognise NCSE and to treat episodes promptly, and the high rate of recurrence, is of particular concern in view of fears that repeated exposure to this condition might be brain damaging. At least 28 children in the present series showed evidence of intellectual or educational deterioration over the period during which NCSE had occurred, although the exact cause was difficult to determine.

Adolescent

[Lennox-Gastaut syndrome with series of spasms intermixed with tonic seizures].

A patient with Lennox-Gastaut syndrome developed an unusual feature of periodic spasms (PS), intermixed with tonic seizures. At age 14, she began to have unilateral seizures on awaking, followed by series of spasms Each spasm consisted of rotation of the head to the left and global muscle contraction of the whole body lasting for 0.5-1 second. Ictal EEGs revealed diffuse high voltage slow waves (HVSW), sometimes superimposed upon by diffuse low voltage fast activity, which was related to the increase of muscle activity following the spasms. When the duration of the fast activity on EEG became longer than 2 seconds, the peculiar seizures consisting of combination of asymmetrical spasms and tonic seizures occurred. It is speculated that the fast activity superimposed on HVSW seen in PS represents a transition from PS to tonic seizures.

Adolescent

Establishment of HIV-1-producing cells from peripheral mononuclear cells cultured with normal human serum.

Normal human serum (NHS) contributed to the establishment of cells producing HIV-1 under the conditions of coculture of peripheral mononuclear cells (PMC) from HIV-1 seropositive patients and of PHA-prestimulated or -non-stimulated PMC from seronegative healthy donors. No addition of IL-2 and Polybrene was necessary. Since, in the case 90101, the mitochondrial displacement-loop DNA showed identical sequences in the established cells and the HIV-1 seropositive patient's cells, it can be asserted that the HIV-1-producing cells originated from the patient. These cells are still releasing HIV-1-virion more than one year after their establishment.

Adult

[Study on fluctuation of several parameters in varicella].

Specific immunological responses (varicella-specific IgG and IgM) and various parameters (interferon, C3, C4, platelet count, erythrocyte count, erythrocyte CR1) in varicella infection were determined over the course of the disease (days) and their relationships to severity and immunological significance evaluated. Defining the day varicella appeared as disease day 0, IgG appeared on the disease day 4 and IgG on day 5. The preceding disease days were dominated by non-specific immunological mechanisms. Interferon appearance preceded that of these antibodies but did not correlate with severity. In serious cases, C3 and C4 increased in the acute stage while platelet count declined. Erythrocyte count decreased in severe cases after disease day 5. Three patients with lower erythrocyte CR1 activity values remained critical. These findings suggested that determining platelet count is expedient in determining severity in the early stage and that the complement pathway is a major component of early immunological response.

Adult