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Biomedical subjects

K Iinuma

Publications and source records attributed to K Iinuma.

At least 145 records · Page 8Linked to original sources
PubMed1990

Reduced regional cerebral metabolic rate for glucose at the terminal stage in a case of late infantile neuronal ceroid lipofuscinosis.

Regional cerebral metabolic rate for glucose was determined for six different areas of the gray matter in an 8-year-old girl with late infantile neuronal ceroid lipofuscinosis. In all regions, the rates were almost half of the control values. The regional cerebral metabolic rate for glucose was relatively preserved in the striatal region and severely reduced in the frontal cortex.

Blood Glucose↗
PubMed1990

[The development of a highly sensitive IgE immunoradiometric assay using monoclonal antibodies].

We have developed two-site immunoradiometric assay (IRMA) for quantitation of human serum IgE, using two different anti-IgE monoclonal antibodies which recognize different epitopes on IgE molecule. We obtained 25 different monoclonal antibodies classified seven groups, and then two different monoclonal antibodies were selected for bead coater and tracer after checking the titer and specificity of each antibody. IgE IRMA we developed here showed good performance in terms of specificity, sensitivity and reproducibility. No cross-reactivity to IgG, IgM and albumin in the level of normal range was observed. The sensitivity for IgE assay was 0.6 x 10(-3) IU/tube and measurable range was 0.01-20.0 IU/tube (0.5-10(3) IU/ml). Coefficient of variations of Intra assay were 2.2-4.3% and average recovery yield was 92.5-108%. Good correlations with Phadebas IgE PRIST and Pharmacia IgE RIA were obtained i.e., y = 1.12x-0.19 (r = 0.96) and y = 0.97x-5.9 (r = 9.7), respectively. These results indicate that the assay system will contribute much to the routine diagnosis for atopic allergy disease and parasitic infections.

Antibodies, Monoclonal↗
PubMed1990

[Special application of EEG and evoked potential studies for practice of childhood epilepsy].

The patients whose EEG demonstrates spikes in the midline-parietal, left and/or right centro-parietal areas can be belonged to a category of benign epilepsy in childhood. In these patients, an extremely high voltage somatosensory evoked potential (SEP) mimicking a spike is elicited by electric stimulation to both upper and lower extremities. The peaks of this giant SEP show extraordinarily high amplitude at N32 and at N50 by stimulation to median and posterior tibial nerves, respectively. It is thought that these spikes originate from the somatosensory cortex in the light of the latencies of the giant SEP. It is not uncommon that the infants with perinatal problems show insufficient staring and tracking. Among these infants, those who have occipital irregular polyspikes and slow waves later develop infantile spasms. This unique combination may be important as the high risk signs of infantile spasms. The visual outcome of these patients relates to persistence of seizures and discharges rather than the pattern of visual evoked potential.

Electroencephalography↗
PubMed1990

[A typical cases of X-linked anhidrotic ectodermal dysplasia and a diagnosis of carriers].

A typical case of X-linked anhidrotic ectodermal dysplasia (AED) aged 19 year-old-male was discussed in detail, and a diagnosis of carriers of AED with his elder sister aged 24 years just before her marriage and mother was carried out. It seemed that the presence of 6 items of manifestation were important for diagnosis of carriers of AED without any evident symptoms. These were (1) abnormal teeth, (2) dissociation of dermatoglyphics, (3) hypohidrosis, (4) anhidrotic patchy distribution, (5) the lines of Blaschko and (6) expansion of distance between sweat pores. His mother was able to be diagnosed as a carrier of AED by having all of the 6 items. It was confirmed that his sister was not a carrier of AED, since she had none of the 6 items and yet her probability of being a carrier of AED was 1.5625% due to Lyon's hypothesis.

Adult↗
PubMed1990

[Clinical evaluation of serum biotin levels and biotinidase activities in patients with various liver diseases].

In order to evaluate the clinical significance of serum biotin and biotinidase in liver disease, serum biotin levels and biotinidase activities were determined in 83 patients with various liver diseases and 10 healthy controls. Serum biotin levels and biotinidase activities were determined by a simplified lactobacillus plantarum bioassay and liquid chromatography with fluorimetric detection respectively. Serum biotin levels in decompensated liver cirrhosis, hepatoma and fulminant hepatitis were found to be significant low compared with healthy controls, while it was significant high in autoimmune hepatitis. There was no significant difference between serum biotin levels in the other liver diseases and healthy controls. In various liver diseases except for both acute hepatitis and alcoholic liver disease biotinidase activities were significantly reduced than in healthy controls. Serum biotinidase activities were correlated with serum albumin, prothrombin time, ChE and total cholesterol respectively, suggesting that biotinidase activities may reflect the degree of liver damage. These results seem that biotin deficiency may occur in some cases of severe liver diseases.

Adult↗
PubMed1989

Hydranencephaly and maximal hydrocephalus: usefulness of electrophysiological studies for their differentiation.

The EEG was evaluated in three cases of hydranencephaly and two cases of maximal hydrocephalus, and the visual evoked potential was studied in one case each of hydranencephaly and maximal hydrocephalus. The EEG tracing of hydranencephaly typically showed a flat pattern in most bipolar derivations because of the differential amplification of the same activity from remote generators. However, the EEG of maximal hydrocephalus did not demonstrate a flat pattern in bipolar as well as referential derivation, and the activities were different from various electrodes in referential derivation. The visual evoked potential of maximal hydrocephalus showed a normal pattern, while that of hydranencephaly showed no response. Electrophysiological examinations (such as EEG and visual evoked potential) are useful for differentiation of hydranencephaly and maximal hydrocephalus in cases whose computed tomographic scans do not provide clear differentiation.

Anencephaly↗
PubMed1989

Computed tomography, magnetic resonance imaging, positron emission tomography and evoked potentials at early stage of adrenoleukodystrophy.

An 8 year-old-boy with adrenoleukodystrophy (ALD) was subject to an x-ray computed tomography (CT) scan, magnetic resonance imaging (MRI), positron emission tomography (PET) using 18F-fluorodeoxy glucose and evoked potential studies, about 3 months after the onset of neurologic symptoms. A CT showed decreased radiodensity over the white matter of both occipital lobes, and high density areas on the periventricular space. No contrast enhancement was observed around the low dense white matter, while occipital grey matter seemed to be almost normal. MRI demonstrated a larger and more obvious lesion of prolonged T2 relaxation time which was 170-250 msec than that detected by CT scan. The PET demonstrated wide and severe hypometabolism for glucose over the grey and the white matter of bilateral occipital lobes. The visual evoked potential (VEP) was scarcely elicitable, while the auditory brainstem response (ABR) and short latency somatosensory evoked potential (SSEP) was normal. These imaging studies suggest that extensive and profound demyelination exists in the occipital white matter. For detection of demyelinizing process, MRI may be more effective than CT in this disorder. The PET study revealed that there may have been an abnormality of the grey matter as well as the white matter in the occipital lobe. The VEP disappeared in the relatively early stage. The brainstem may not be involved at such early stage in light of normal ABR and SSEP.

Adrenoleukodystrophy↗
PubMed1989

[Two siblings of Leber's congenital amaurosis with an increase in very long chain fatty acid in blood: relationship between peroxisomal disorders and Leber's congenital amaurosis].

We reported two siblings of Leber's congenital amaurosis associated with increased level of very long chain fatty acid (VLCFA) in blood. Case 1, a 3 1/2-year-old boy had congenital blindness, severe psychomotor retardation, hepatomegaly, profound hypotonia, loss of deep tendon reflexes, muscular atrophy and weakness, and non-convulsive status epilepticus characterized by a sudden respiratory failure, and also showed a flat electroretinogram, non-pigmentary retinal degeneration, severe atrophy of the brain stem and cerebellum, hepatic fibrosis, decreased motor and sensory conduction velocities and atlanto-axial instability. Sural nerve biopsy revealed severely decreased number of total myelinated fibers without remarkable demyelination or remyelination. Case 2, an elder sister of case 1, with pigmentary retinal degeneration, hepatomegaly and pericarditis had died at 3 months. Autopsy revealed hypomyelination and heterotopy of the cerebral white matter, hepatic fibrosis, renal microcysts and normal adrenal cytoarchitecture. In case 1, the level of VLCFA was increased twofold and sevenfold of controls in serum and in red cell membrane, respectively. Phytanic or trihydroxycholestanoic acid was not detected in the serum and bile. Normal shaped peroxisomes were definitely recognized in biopsied liver by means of electronmicroscopic histochemistry. From the above findings, these patients was thought to be a new variant of peroxisomal disorders relating to degradation of VLCFA, other than Zellweger syndrome, infantile Refsum disease and infantile adrenoleukodystrophy. It was concluded that peroxisomal functions should be studied in cases of Leber's congenital amaurosis.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1988

Hyperamino-acidaemia and hyperammonaemia in epileptic children treated with valproic acid.

Serum amino acid pattern, blood ammonia, and serum valproic acid (VPA) levels were estimated in 75 epileptic children with and without VPA therapy. The incidence of patients with hyperammonaemia (greater than 60 mumol/l) in patients treated with only VPA or with a combination of VPA and other antiepileptic drugs was 19% and 20% respectively. Hyperammonaemia was not observed in patients receiving other drugs. Elevated levels of serum glycine, alanine and serine were more common in epileptic patients treated with VPA alone and with VPA in combination with other drugs than in patients receiving other drugs. A significant positive correlation was found between the serum levels of glycine and alanine, of glycine and serine, and of serine and alanine. These findings may indicate a mitochondrial dysfunction caused by VPA.

Adolescent↗
PubMed1988

Sex chromatin analysis of lymphocytes invading host organs in transfusion-associated graft-versus-host disease.

We report a method of sex chromatin analysis of lymphocytes separated from host organs in transfusion-associated graft-versus-host disease (GVHD), which enabled the demonstration of invasion by donor lymphocytes. The lymphocytes examined were separated from deparaffinized tissue blocks of skin, spleen and bone marrow from two female patients with transfusion-associated GVHD by incubation in 0.5% pepsin. The tissues had been removed at autopsy and fixed in formalin. Sex chromatin analysis was performed by fluorescence microscopy on separated lymphocytes stained with 0.005% quinacrine dihydrochloride. By this means Y chromatin-positive (i.e. male) lymphocytes were demonstrated in the skin, spleen and bone marrow of both female patients.

Aged↗