[Coordination of unrelated bone marrow donors in the Tokai Bone Marrow Bank--psychological reactions of donors to donation].

The Tokai Bone Marrow Bank was established in 1989 and coordinated 1,415 patients with 3,000 HLA-A, B-typed donors. Of the 1,415 patients, 757 patients had HLA-A, B-identical donors, 206 patients had HLA-DR-identical donors, and 80 patients had MLC-compatible donors. Finally, 55 unrelated donor bone marrow transplantations were done.

[Familial amyloid polyneuropathy type IV (Finnish type)--a clinicopathological study].

Familial amyloid polyneuropathy type IV (Finnish type, FAP IV) is one form of hereditary generalized amyloidosis with autosomal dominant trait and is characterized clinically by a triad of corneal lattice dystrophy, caudal cranial neuropathy and various skin changes. The vast majority of the families with this disorder originated from Finland. We carried out a clinicopathological study of a large FAP IV kindred recently found in Japan.

[A case of transverse myelopathy caused by pyogenic vertebral osteomyelitis having extended from a pedal ulcer].

A 64-year-old man who had suffered from diabetes mellitus and a recurrent intractable ulcer of the foot developed subacute transverse myelopathy with severe inflammatory reactions. Magnetic resonance imaging (MRI) revealed destructive lesions in both upper thoracic and lumbar spines. In plain X-ray film, although intervertebral spaces of the lumbar spines were apparently normal on the supine position, they became narrow spaces on the sitting position.

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.

Amyloid precursor protein is found in lysosomes.

The major component of Alzheimer's disease amyloid is a small polypeptide referred as the amyloid beta protein, which is derived from a larger precursor, amyloid precursor protein (APP). Cell fractionation and immunological studies on the APP molecule indicate that APP is localized either in the neuron and astrocyte and that the molecule is recovered from lysosomal fraction.

[Bone marrow transplantation for patients with Philadelphia chromosome-positive acute lymphoblastic leukemia].

We reported the results of 6 allogenic bone marrow transplantation (BMT) and 3 autologous BMT for patients with Philadelphia chromosome (Ph1)-positive acute lymphoblastic leukemia (ALL) by Nagoya BMT group. Two of six patients who received allogenic BMT have continued complete remission (CR) on +639 days and +1,597 days. Four of six patients relapsed on +134, +203, +216, and +267 days.

[A comparison of the several methods for determination of specific IgE antibodies].

The accuracy of measurement using kits in the clinical laboratories is important for the patient diagnosis and treatment. In the present paper, the AL-18, AlaSTAT, CAP, FAST and RAST methods were investigated and were compared among kits the results obtained with serum sample, for determination of specific IgE antibodies. Significant differences among kits were observed from the results of those methods.

[Relationship between the total IgE levels and specific IgE antibodies].

In this study, nine allergens were selected; i.e. 1289 cases of d1, 1277 cases of e1, 1547 cases of f1, 1063 cases of t17 and others, for investigating the relationship between the total IgE level and RAST score.

Correlation of regional cerebral blood flow with performance on neuropsychological tests in schizophrenic patients.

Regional cerebral blood flow (rCBF) was determined by the 133Xe inhalation technique (Headtome II: ring detection SPECT) in 53 DSM-III schizophrenic patients. The rCBF values were corrected by using end-tidal carbon dioxide concentration values (PECO2). After rCBF measurement, neuropsychological tests--Word Fluency Test, Maze Test and Wisconsin Card Sorting Test--were performed.

Exclusion mapping of the hereditary dentatorubropallidoluysian atrophy gene from the Huntington's disease locus.

Hereditary dentatorubropallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder. Clinical and genetic findings in hereditary DRPLA are very similar to those of Huntington's disease (HD). However, it can be differentiated from HD by the pathological findings of dentatorubral and pallidoluysian atrophies and by a lack of prominent atrophy of the striatum at necropsy.

[Effect of breathing fluctuations on cerebral blood flow in demented patients and its correction method using end-tidal CO2 concentration].

During mouthpiece respiration of Xe-133 for a measurement of regional cerebral blood flow (rCBF), the breathing pattern of patients fluctuated and it caused a change of end-tidal CO2 concentration that had an excellent correlation with PaCO2 in patient without respiratory disease. The end-tidal CO2 concentration of demented patients varied within lower ranges than senile control group. The range of fluctuation on the end-tidal CO2 concentration was dependent on the type and the degree of dementia, and it fluctuated most widely at the middle stage of Alzheimer disease.

Increase of protein synthesis by uridine supplement in lectin-stimulated peripheral blood lymphocytes and EB virus-transformed B cell line of hereditary orotic aciduria type I.

A 2 month-old Japanese girl with hereditary orotic aciduria type I was treated with oral uridine supplement. The activities of orotate phosphoribosyltransferase (OPRT) and orotidine-5'-phosphate decarboxylase (ODC) in erythrocytes were 2.7 and 0.

A case of adult neuronal ceroid-lipofuscinosis with the appearance of membranous cytoplasmic bodies localized in the spinal anterior horn.

An autopsy case of adult neuronal ceroid-lipofuscinosis was examined. The clinical picture was characterized by gait disturbance, bulbar palsy and dementia. Histopathologically, diffuse neuronal loss was found throughout the central nervous system.

An autopsy case of adult neuronal ceroid lipofuscinosis.

This is a report of an autopsy case (45-year-old woman) of adult neuronal ceroid lipofuscinosis with a long-lasting course of 25 years. In the course of her illness, gait disturbance, bulbar palsy and dementia were observed. Pathologically severe neuronal loss and hemosiderin-like granules were found in the cortex, basal nuclei, thalamus and others.

Menkes' kinky hair disease: clinical and experimental study.

A pedigree of Menkes' kinky hair disease (MKHD) is reported. One patient of this family who underwent copper treatment was followed for three years with fundus examinations and ERG measurement. The blood copper level remained normal after six months of age, when intravenous treatment was switched from cupric acetate to cupric sulfate.

Clinicopathologic studies on neuro-Behçet's disease.

Nine cases of neuro-Behcet's disease were investigated clinicopathologically. Pathological pictures of the central nervous system were characterized as follows: the site of predilection was the brain stem, followed by the spinal cord, cerebrum and cerebellum. The pathognomonic changes were recurrent inflammations around small vessels, causing a softening of the tissue.

Copper-binding proteins in the liver and kidney from the patients with Menkes' kinky hair disease.

Copper-binding proteins in the tissues from the patients with Menkes' kinky hair disease were examined by gel filtration on a Sephadex G-75 column. In the kidney, major part of copper was found to bind to low molecular weight protein, which corresponded chromatographically to metallothionein. This copper-binding protein contained a large amount of copper and a small amount of zinc.

Gmi-gangliosidosis. A variant with high activity of hepatic neutral beta-galactosidase.

A case of GM1-gangliosidosis with high activity of hepatic neutral beta-galactosidase is reported. GM1-beta-galactosidase was deficient. Ganglioside GM1 was accumulated in the liver of this patient.