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Biomedical subjects

T Orii

Publications and source records attributed to T Orii.

At least 397 records · Page 22Linked to original sources
PubMedDate not supplied

Long-term follow up of patients with common variable immunodeficiency treated with intravenous immunoglobulin: reevaluation of intravenous immunoglobulin replacement therapy. IVIG therapy in CVID.

Five patients with common variable immunodeficiency treated in our hospital between December 1979 and December 1990 were given six kinds of intravenous immunoglobulin preparations (pepsin treated, S-sulfonated, polyethylene glycol treated, pH4 treated, alkylated, and pH4.25 formulation preparation) for replacement therapy. Duration of the therapy ranged from 7.6 to 11 years. Incidences of fever and acute infections were variable among patients, but no significant differences were seen in the incidences among periods given each preparation. Three cases revealed abnormal pulmonary functions in tests. Adverse reactions were rarely seen in our study periods, and no severe reactions were observed. No significant differences were seen in incidences of adverse reactions. Postinfusion levels of serum complement slightly decreased from preinfusion levels. However, the decrease in complement was not related to any adverse reaction. No long-term complications such as transmission of hepatitis have been observed. Our data suggest that no obvious differences exist between the efficacy and safety of each IVIG preparation. Differences of efficacy of IVIG replacement therapy may be due to the variable pathophysiology of each patient.

Adolescent↗
PubMedDate not supplied

Adrenoleukomyeloneuropathy presenting as cerebellar ataxia in a young child: a probable variant of adrenoleukodystrophy.

A child aged 5 years 7 months was diagnosed as suffering from adrenoleukomyeloneuropathy (ALMN). The first sign was ataxia, and high intensity lesions were observed in the cerebellar hemispheres on T2-weighted brain MRI. His condition progressed rapidly to a vegetative state in 1 year. When aged 7 years 3 months adrenal insufficiency supervened and his skin turned dark. Rectal biopsy revealed linear cytoplasmic inclusions in macrophages in the rectal membrane. At the age of 8 years 2 months, an analysis of very long-chain fatty acids of sphingomycin in plasma led to the final diagnosis. At the age of 10 years, cerebellar and cerebral atrophy were prominent and diffuse high intensity lesions were noted in the cerebellum and cerebrum. An onset below 9 years of age has not previously been documented in ALMN.

Adrenoleukodystrophy↗
PubMedDate not supplied

Effects of erucic acid therapy on Japanese patients with X-linked adrenoleukodystrophy.

Ten Japanese boys with childhood adrenoleukodystrophy (ALD), one adult patient with adrenomyeloneuropathy (AMN), and two presymptomatic ALD boys were treated with dietary erucic acid (C22:1) for more than 12 months; except in a case of childhood ALD patient who died 7 months after beginning erucic acid therapy. During erucic acid therapy, the serum levels of very long-chain fatty acid (VLCFA) (C24:0/C22:0) decreased within 1-2 months in all patients, and these levels in four of the patients decreased to the normal range. Neurological examination and MRI findings in all 10 of the childhood ALD patients showed progression of the disease while they were receiving the dietary therapy. However, the mean interval between the onset of awkward gait and a vegetative state in diet-treated patients was significantly longer than that in the untreated patients. One AMN patient showed slight improvement of spastic gait and lessened pain in the lower limbs due to spasticity. The two presymptomatic ALD boys remained intact on clinical examination and on MRI findings for 38 and 23 months, respectively, after starting the diet.

Adolescent↗
PubMedDate not supplied

Short latency somatosensory evoked potentials and 99mTc-HMPAO SPECT in a case of flunarizine-effective alternating hemiplegia in infancy.

Short latency somatosensory evoked potentials (SSEPs) and 99mTc-hexamethylpropylene amine oxime single photon emission computed tomography (99mTc-HMPAO SPECT) were examined in a patient with alternating hemiplegia in infancy (AHI) before and after flunarizine treatment. The low amplitude and elongation of the latency time at the C'4 level of the interictal SSEPs before treatment were ameliorated by flunarizine administration. The cerebral hypoperfusion in the right hemisphere observed on 99mTc-HMPAO SPECT before treatment improved, as seen by normal and symmetrical imaging, after flunarizine treatment. These observations suggest that AHI is caused by a functional disturbance in the cerebral area contralateral to the hemiplegia, and that SSEPs and 99mTc-HMPAO SPECT are useful for evaluating functional disturbance in the contralateral motoneurons and the effect of flunarizine treatment in AHI patients.

Evoked Potentials, Somatosensory↗
PubMedDate not supplied

Prenatal diagnosis of peroxisomal disorders. Biochemical and immunocytochemical studies on peroxisomes in human amniocytes.

Prenatal diagnoses of peroxisomal disorders, including peroxisome-deficient Zellweger syndrome, isolated deficiency of peroxisomal beta-oxidation enzyme and rhizomelic type chondrodysplasia punctata were investigated by means of the lignoceric acid oxidation assay, indirect immunofluorescence staining and pulse-chase experiments, using cultured amniocytes. Assessment of peroxisomal beta-oxidation activity by means of [1-14C]lignoceric acid oxidation is essential for the diagnosis of a single enzyme deficiency of peroxisomal beta-oxidation with detectable enzyme protein. For the diagnosis of Zellweger syndrome, the absence of peroxisomes was readily determined by immunofluorescence staining of only a few amniocytes. Evidence for abnormal processing of 3-ketoacyl-CoA thiolase leads to the diagnosis of rhizomelic chondrodysplasia punctata. All the fetuses were considered to be normal and the neonates were normal. Use of these methods requires only a small number of amniocytes and will facilitate the prenatal diagnosis of peroxisomal disorders.

Acetyl-CoA C-Acyltransferase↗
PubMedDate not supplied

Evaluations of the prescription order entry system for outpatient clinics by physicians in the 80 university hospitals in Japan.

We investigated the evaluations of the prescription order entry system by the physicians who are the direct users of this system. Questionnaires were sent to 400 staff physicians of 80 medical school affiliated hospitals, out of which 76% responded. Among the respondents, 48 per cent are making use of the computerized outpatient prescription order entry system, and 52 per cent are not. The result shows 78% of the physicians are in favor of the prescription order entry system; the most dissatisfied opinion of the physicians toward the system was that the input took too much time thus reduced the communication with the patient. Physicians who are using the system seemed to favor the system more than the physicians who are not using it.

Attitude to Computers↗
PubMedDate not supplied

Primary hepatic carcinoid tumor: a case report.

A case of primary hepatic carcinoid tumor is reported. A 57-year-old woman had no endocrine symptoms. Light microscopic findings revealed tumor cells forming trabecular or insular structures and demonstrating diffuse positive staining by the Grimelius method. Mucin stained with Periodic acid-Schiff (PAS) and alcian-blue was partially present in the tumor cells. Immunohistochemically, most tumor cells stained positive with chromogranin A and cytokeratin stains. Intensive and careful investigation before and after the operation revealed no other origin of the tumor. We present this rare case, describe the clinical features of hepatic carcinoid, and discuss its diagnosis.

Biomarkers, Tumor↗
PubMedDate not supplied

Evaluation of theophylline clearance in children with bronchial asthma.

The total body clearance of theophylline was examined in 95 children aged 6 months to 15 years with chronic bronchial asthma. Each patient received sustained-release theophylline preparations and was evaluated at steady state. Blood samples were obtained 1-8 hours after a morning dose, and individual total body clearance was determined with a Bayesian forecasting computer model. Changes in theophylline clearance were evaluated within age groups, with the age determined at the time of blood sampling. Total body clearance tended to increase with age up to 10 years. In children over 10 years old, total body clearance significantly decreased with age. The interpatient variability of theophylline clearance was most noticeable at 2-4 years of age. Changes in volume of distribution were similar to those in clearance. In conclusion, there are pronounced age-dependent differences in theophylline clearance that require individual adjustment of dosage regimens on the basis of serum concentrations and clinical response in children. Individualization of the oral dose based on frequent serum measurements is necessary to maintain theophylline levels in the therapeutic range.

Adolescent↗
PubMedDate not supplied

IgE production by B-cells stimulated with interleukin-4 and Epstein-Barr virus in patients with elevated serum IgE levels.

Epstein-Barr virus (EBV) and recombinant interleukin-4 (rIL-4) induce IgE production by normal B-cells. We demonstrate here the difference in IgE synthesis in patients with elevated IgE levels and normal control subjects. EBV and rIL-4 induced the synthesis of IgE by surface IgE-negative B-cell precursors isolated by cell sorting. IgE synthesis in normal subjects became apparent in the second week with increasing rIL-4 concentrations, whereas in patients with elevated IgE levels, there was a marked elevation in IgE synthesis without rIL-4 stimulation. We did not observe the presence of DNA rearrangement occurring between IgM and IgE switch regions (S mu and S epsilon, respectively) during switching to IgE isotype in patients with elevated IgE levels. We found that with the limited sensitivity of Southern blotting, this method may not be useful for the study of B-cell populations in which only a small percentage of cells have undergone switching.

B-Lymphocytes↗
PubMedDate not supplied

Evaluation of lymphocyte proliferative responses to casein hydrolysate formula in cow's milk-sensitive children: response of peripheral blood mononuclear cells to hydrolysate formula.

The proliferative responses of peripheral blood mononuclear cells (PBMCs) to cow's milk proteins and a casein hydrolysate formula were investigated in 10 cow's milk-sensitive patients. The casein hydrolysate formula reduced the symptoms in seven children but not in three. The proliferative responses of PBMCs to cow's milk proteins and the formula were higher in the three patients whose symptoms were not reduced by casein hydrolysate formula. However, the proliferative responses of PBMCs to hydrolysate formula were lower than those to cow's milk proteins in patients whose symptoms were reduced by casein hydrolysate formula. These results suggest that an elimination diet with casein hydrolysate formula may not be as effective in some patients with cow's milk allergy whose PBMCs proliferate to casein hydrolysate formula.

Caseins↗
PubMedDate not supplied

Responses of lymphocytes to Epstein-Barr virus in patients with primary immunodeficiencies.

Several reports have demonstrated that the responses of B-cells to Epstein-Barr virus (EBV) are variable in common variable immunodeficiency (CVID). In this study in patients with selected primary immunodeficiencies, i.e., Bloom's syndrome, Wiskott-Aldrich syndrome or IgA deficiency, the responses of peripheral blood mononuclear cells (PBMCs) to EBV were investigated. In the two patients with Bloom's syndrome, PBMCs stimulated with EBV showed decreased proliferation and immunoglobulin production, suggesting a mild abnormality of B-cells. In patients with Wiskott-Aldrich syndrome, the responses were variable. In the patient with IgA deficiency, PBMCs responded normally to EBV in proliferation, whereas PBMCs responded poorly to EBV in IgA production, suggesting an abnormality only in the IgA production mechanism.

B-Lymphocytes↗
PubMedDate not supplied

Suppression of IgA production by lymphocytes induced by diphenylhydantoin.

In this study, the mechanisms for suppression of immunoglobulin production by lymphocytes induced by diphenylhydantoin (DPH) were investigated using peripheral blood mononuclear cells from one patient 3 months after discontinuation of DPH, when IgA deficiency was improved. Immunoglobulin production in PBMCs was dose-dependently suppressed by DPH and the suppressive effect of DPH on IgA production tended to be greater than the effect on IgM or IgG production. Moreover, cell separation experiments indicated that DPH suppresses immunoglobulin production by PBMCs through a suppression of B cell, but not T cell, function. The finding that the suppressive effect of DPH on IgA production tended to be greater than the effect on IgM or IgG production might be related to the fact that the alpha-chain gene is located in the 3' end of the gene coding the constant regions of human heavy chains.

B-Lymphocytes↗
PubMedDate not supplied

Interleukin-2 and interferon-gamma production by peripheral blood lymphocytes of patients with common variable immunodeficiency.

We evaluated the production of interleukin-2 (IL-2) and interferon-gamma (IFN-gamma) in peripheral blood lymphocytes (PBLs) from six patients with common variable immunodeficiency (CVID). IL-2 production and IFN-gamma concentrations were not reduced in these patients. As the production of IL-2 and IFN-gamma secreted from TH1 clones was within the normal range in patients with CVID, we concluded that TH1 function was normal in these patients. Our previous report showed B-cell dysfunction in CVID patients. Therefore, we concluded that the immune defect in these patients is caused by B-cell dysfunction rather than T-cell dysfunction.

Adult↗