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Biomedical subjects

A Imamura

Publications and source records attributed to A Imamura.

At least 91 records · Page 5Linked to original sources
PubMed1996

Prenatal diagnosis of adrenoleukodystrophy by means of mutation analysis.

Prenatal diagnosis of adrenoleukodystrophy (ALD) was performed by means of genetic and biochemical analysis using chorionic villi and amniocytes. The mother was a carrier of an exonic point mutation in the ALD protein gene (2154 C to T) which resulted in the premature formation of a termination codon (Q590STOP) and deletes the Pst I site. Two patients in this family were hemizygotes for this mutation. Pst I digestion of cDNA from chorionic villi revealed that the fetus was a heterozygote for this mutation, and sex determination using the polymerase chain reaction (PCR) indicated female. Lignocerate oxidation in cultured amniocytes was slightly decreased. These findings suggest that the fetus is a female carrier of ALD, and the resultant baby was female.

Adrenoleukodystrophy↗
PubMed1996

Inflammatory pseudotumor of the liver: report of a case and review of the literature.

Only 56 cases of inflammatory pseudotumor of the liver have been reported in the world literature since its first documentation in 1953. We report herein the case of a 68-year-old man incidentally found to have a lesion in the right lobe of the liver which closely resembled a neoplasm on imaging studies. Thus, partial hepatic resection was performed and histological examination of the resected specimen revealed a diagnosis of inflammatory pseudotumor. Surgical resection is the preferred treatment for inflammatory pseudotumor of the liver, especially in patients for whom a definite histologic diagnosis cannot be made preoperatively or by intraoperative frozen sections. In fact, most of the patients reported in the literature recovered uneventfully after local resection without any postoperative complications, as did our patient.

Aged↗
PubMed1996

Gallbladder cancer with a low junction of the cystic duct or an anomalous pancreaticobiliary junction.

OBJECTIVE: To evaluate conditions similar to those of carcinogenesis of the gallbladder between the gallbladder with a low junction of the cystic duct (LJCD) and an anomalous pancreaticobiliary junction (APBJ). DESIGN: Retrospective and clinicopathological analysis of patients with gallbladder carcinoma. SETTING: First Department of Surgery, Kansai Medical University. PATIENTS: Examination of 47 patients (7 men and 40 women; average age: 67.8 years) with gallbladder carcinoma revealed 7 patients (14.9%; 1 man and 6 women; average age: 67.8 years) with LJCD and 6 patients (12.8%; 6 women; average age: 60.3 years) with APBJ. METHODS: Clinical findings in both groups were compared with those of the 34 patients who remained after exclusion of the data of the above 7 patients with LJCD and 6 patients with APBJ. The data of the three groups were examined by the chi 2 test at the 5% level of significance. RESULTS: Most of the gallbladder cancer patients with LJCD or APBJ had gallstones. The biliary amylase levels determined in the gallbladder of patients with LJCD or APBJ were remarkably high. CONCLUSION: The results indicate that patients with LJCD or APBJ are more likely to develop carcinoma of the gallbladder. The factors responsible for carcinogenesis may be alteration of the bile content due to reflux of pancreatic enzymes through the LJCD or APBJ, and mechanical irritation due to gallstones. Therefore, these pathological conditions in patients with LJCD are similar to those experienced in patients with APBJ.

Aged↗
PubMed1996

Trial of docosahexaenoic acid supplementation on a Japanese patient with a peroxisome biogenesis defect.

A female Japanese patient diagnosed with peroxisome biogenesis defect (PBD), who had hypotonia and craniofacial dysmorphism, was given supplementation of docosahexaenoic acid (DHA). Accumulation of very long chain fatty acids was revealed, and a diagnosis of PBD was made at 2 months of age because of the absence of peroxisomes, a defect in peroxisomal beta-oxidation enzymes and a decreased level of DHA in the erythrocytes. Supplementation of DHA was introduced at 3 months of age. For the first several months, psychomotor development was fairly good. The patient could laugh, brush off a blanket and play with toys at 6 months of age. However, neurological regression and convulsions occurred after 7 months of age. After recurrent respiratory infections and disturbance of the circadian rhythm, the patient died of liver failure and disseminated intravascular coagulopathy at 20 months of age. DHA may have a favorable effect on the early development of patients with PBD, but neurological deterioration cannot be prevented. Patients with a milder phenotype would be better candidates for DHA supplementation.

Docosahexaenoic Acids↗
PubMed1996

[A case of empyema caused by Salmonella enteritidis in a patient with sequela of pulmonary tuberculosis].

A 69-year-old male was admitted to our hospital on Jan. 10, 1995 complaining of bloody sputum, left back pain and fever of 39 degrees C. He had a history of pulmonary tuberculosis 45 years ago. His chest X-ray and CT showed the presence of air-fluid level in the left pleural cavity with thickening and calcification of the pleura. Salmonella Enteritidis was isolated from the sputum, bronchoalveolar lavage fluid and stool. He was diagnosed as suffering from Salmonella-empyema with an internal fistula. Based on the in vitro sensitivity test, sulfamethoxazole/trimethoprim was started. However, the efficacy of the antimicrobia was not sufficient. He then underwent left pleuropneumonectomy. Salmonella was cultured also from the specimen obtained at the operation. His course after operation was uneventful. Thus, although Salmonella Enteritidis is known as a common pathogen of food poisoning, it can cause empyema, especially in a case with an impaired host defence system.

Aged↗
PubMed1996

Effect of dietary protein and enalapril on proximal tubular delivery and absorption of albumin in nephrotic rats.

In passive Heymann nephritis (PHN), angiotensin-converting enzyme inhibition (ACEI) or a low dietary protein intake decreases albuminuria (UAlbV). Although this reduction in albuminuria appears to result from an improvement in glomerular permselectivity, the effect of these treatments on albumin permeation and absorption by the nephron has not been clarified. This study used micropuncture techniques to examine the effect of these two treatments on albumin permeation (by measuring the delivery of albumin to the proximal tubule) and the tubular absorption of albumin. PHN rats (12-18 days after injection of FX1A) were switched from 23% to either 40% protein diet (HP), 40% protein diet and concomitantly treated with enalapril (40 mg.kg-1.day-1) (HPE), or to 8% (LP) protein diet for 4-6 days. Although left kidney glomerular filtration rate (GFR) did not differ among the groups, UAlbV from the left kidney in LP and HPE was only 20-40% of that observed for the HP group. In protocol 1, the fractional recovery of albumin (FRAlb) in urine was calculated following injection of artificial tubular fluid containing [14C]inulin and 125I-labeled albumin into the earliest identifiable proximal loops. There were no differences in FRAlb among the three groups. In protocol 2, timed quantitative collections of tubular fluid were obtained from proximal tubular loops. The rate of albumin delivery to the earliest accessible loops of the proximal tubule was significantly lower for the LP and HPE groups compared with the HP group. For each group, albumin concentration corrected for water absorption was not altered along the proximal tubule. The data indicate that alterations of dietary protein intake or ACEI treatment results in large changes in the delivery of albumin at the proximal tubule that could singularly account for the changes in urinary albumin excretion.

Absorption↗
PubMed1996

[Cognitive function and MRI findings in very low birth weight infants].

Twenty-two very low birth weight infants at preschool ages of 5-6 years were studied to clarify the correlation between cognitive function and MRI findings. Cognitive function was evaluated by the Wechsler Intelligence Scale for Children-Revised (WISC-R) and the Frostig developmental test of visual perception. Ventricular enlargement, assessed by the bioccipital index (B. I.) measured on MRI, was correlated to cognitive disorders. Children with periventricular high intensity areas (T2-weighted images) extending from the posterior periventricular region to the parietal lobe tend to highly suffer from cerebral palsy and visuo-perceptual impairment. These results indicate that the disorders of cognitive function in very low birth weight infants were caused by a damage of association fibers in periventricular areas which was detectable by MRI.

Brain↗
PubMed1996

[Benign infantile mitochondrial myopathy caused by reversible cytochrome c oxidase deficiency].

A 2-month-old girl had generalized weakness, profound muscular hypotonia, hepatomegaly and severe lactic acidosis. She needed ventilatory support. Muscle specimen taken at 2 months showed ragged-red fibers, abnormal mitochondria, and reduced cytochrome c oxidase (CCO) staining Biochemical analysis showed CCO activity to be reduced to about 16% of the normal mean. She received carnitine and coenzyme Q10 supplementation from the age of 3 months and abnormal blood lactate values declined to near normal values during the first three weeks. Gradually her condition started to improved: she held her head at 9 months, and walked alone at 15 months. The second biopsy specimen at 3 years and 8 months showed almost normal CCO staining and she was free of clinical signs. This case is an example of a rare benign infantile mitochondrial myopathy caused by CCO deficiency. Early diagnosis is crucial to provide intensive treatment until spontaneous clinical improvement appears. We concluded that carnitine and coenzyme Q10 supplementation was a useful treatment for clinical improvement in patients with a benign infantile mitochondrial myopathy caused by CCO deficiency.

Carnitine↗
PubMed1996

[Selective thermocoagulation of unresectable malignant tumors using radiofrequency].

Based on our experimental findings on porcine liver, we have been conducting a clinical trial of selective hyperthermia by radiofrequency (RF) capacitive heating with laparotomy for patients with unresectable malignant tumors. In 10 patients with malignant tumors (8 carcinoma of the pancreas, 2 carcinoma of the gallbladder), laparotomy and RF heating were performed after informed consent. The local heat coagulation was produced by heating equipment using 13.56 MHz radiofrequency produced by Omron Corporation, Japan. Four 2-cm electrode needles were placed in the tumor in a square array at intervals of 2.0 cm. Hyperthermia was given for 30 min with a controlled temperature of 50 degrees C in the RF field (2 x 2 x 2 cm3). That of the surrounding area was maintained at less than 40 degrees C. The calculated volume treated by RF ranged between (2 x 2 x 2 cm3) x 1 and (2 x 2 x 2 cm3) x 6. We followed all patients by computed tomographic (CT) scan 2 weeks after coagulation. Tumor markers in the blood were assayed before and 14 days after heating. Follow-up CT scans demonstrated that after the tumor mass had been heterogeneously enhanced, it changed to a homogeneous low-density area in 6 of 10 patients. The levels of tumor markers decreased to lower than the pre-treatment values in 9 of 10 patients. In all patients, the changes in CT scans and/or decrease in the markers were confirmed. Complications such as bleeding or abscess formation were not observed. It was suggested that the selective hyperthermia was safely produced by this equipment. The encouraging results in these patients justify further clinical trials.

Biomarkers, Tumor↗
PubMed1995

Immunohistochemistry for a bifunctional protein in patients with peroxisomal disorders.

Immunohistochemical studies using antisera against bifunctional protein, a beta-oxidation enzyme, were performed on liver, kidney, and brain tissue specimens from patients with peroxisomal disorders and from controls to investigate the distribution and development of peroxisomes. Bifunctional protein-positive granules were not found in patients with Zellweger syndrome or neonatal adrenoleukodystrophy, whereas positive immunoreactivity was observed from 8 and 6 weeks gestation in the liver and kidney, respectively, and in the brain, from 23-25 weeks in the brainstem neurons and from 12-14 weeks in the white matter glia, in controls. Bifunctional protein immunoreactivity then increased with gestation in the brain. These results suggest that bifunctional protein immunohistochemistry is useful for the detection of peroxisomes, which are closely related to neuronal maturation and gliogenesis in premyelination in human brain development.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1995

Analysis of auditory brainstem response waveforms derived ipsilaterally and contralaterally to monaural stimulation.

The auditory brainstem responses (ABRs) obtained from 28 normal healthy adults were studied using the ipsilateral and contralateral recordings from the positions of vertex and each mastoid to monaural stimulation. Each wave of ABRs recorded by ipsilateral and contralateral derivations to stimulation site showed slightly significantly differences. Comparing the ipsilateral and contralateral data, the latencies of waves II and V showed a slightly small reduction in the ipsilateral recording, while these of waves III and IV showed a small increase. Next, the distributions of potentials and latencies of waves II to V were investigated from ABRs situating different electrodes in mid-coronal array of the scalp and non-cephalic reference electrode on the seventh cervical vertebra (CVII). The results of latencies showed the reverse relation to the data obtained from the ipsilateral and contralateral recordings using the reference electrodes on each mastoid. These facts suggest that the comparable differences of latencies in the bilaterally recorded ABRs are explained by the pseudo-phenomena of differential recordings, which the phase delayed or advanced potential propagated to each mastoid being reference electrode position.

Acoustic Stimulation↗
PubMed1995

Effects of chronic treatment with angiotensin converting enzyme inhibitor or an angiotensin receptor antagonist in two-kidney, one-clip hypertensive rats.

The effects of chronic angiotensin II (Ang II) receptor blockade (losartan) or converting enzyme inhibition (enalapril) on blood pressure (BP), urinary albumin excretion (Ualb V), renal histology and the hemodynamic and excretory function of the clipped and nonclipped kidneys were studied in two-kidney, one-clip (2-K 1-C) rats. One day after clipping the right renal artery, male Wistar rats were divided into three groups receiving: (1) losartan, 20 mg/kg/day (N = 7), (2) enalapril, 20 mg/kg/day (N = 8), or (3) no treatment (controls, N = 9) for three weeks. Both losartan and enalapril treatments maintained conscious BP at comparably lowered levels compared to control animals (116 +/- 6 mm Hg and 113 +/- 2 mm Hg vs. 188 +/- 11 mm Hg, respectively, P < 0.01). Treatment also prevented the increase in Ualb V, observed for the untreated group, three weeks after clipping (1.7 +/- 0.5 and 0.7 +/- 0.1 mg/24 hr vs. 17.8 +/- 7 mg/24 hr, respectively, P < 0.01). After three weeks of treatment, acute study of renal function during pentobarbital anesthesia revealed higher values of GFR and RPF and lowered vascular resistance for nonclipped kidneys from the losartan and enalapril groups compared to the corresponding kidneys from control animals. Despite the lower BP of both treated groups, clipped kidney GFR and RPF were unchanged compared to the control group. Ualb V for nonclipped kidneys from untreated rats was approximately 5- to 10-fold higher than in the nonclipped kidneys from the treated groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Albuminuria↗
PubMed1994

Developmental immunohistochemistry of bifunctional protein in human brain.

Immunohistochemical studies of a peroxisomal enzyme, bifunctional protein, were performed on human brains (occipital cortex, cerebellum, pons) from fetus to young adult. Bifunctional protein-positive neurons appeared at 23-25 weeks of gestation in the facial nuclei of pons, at 27-28 weeks in the occipital cortex and Purkinje cells of vermis, and at 36-38 weeks in the Purkinje cells of the cerebellar hemisphere and pontine nuclei. They then increased in number with gestational age. However, bifunctional protein-positive glia appeared early in the occipital deep white matter at 17-20 weeks of gestation, their appearance shifting from the deep to the superficial white matter with increasing age. These results suggest that bifunctional protein is closely related to neuronal maturation and gliogenesis of premyelination in the human brain during development as other peroxisomal enzymes.

3-Hydroxyacyl CoA Dehydrogenases↗
PubMed1994

Identification of a nonsense mutation in ALD protein cDNA from a patient with adrenoleukodystrophy.

The molecular basis of X-linked adrenoleukodystrophy (ALD) was investigated. Six (A to 50) fragments of cDNA for ALD protein (Mosser et al. Nature 361: 726-730, 1993) from an adult patient with adrenomyeloneuropathy were amplified by PCR and mutations were screened by Mutation Detection Enhancement gel electrophoresis. A single base substitution (2154 C-->T, which resulted in the formation of a termination codon for glutamine (Q590STOP) and deletes Pst I site (CTGCAG-->CTGTAG), was detected. Eight other ALD patients did not have this mutation. A family study revealed the presence of both the mutant and normal alleles in the mother, a sister and a niece, indicating that these individuals were carriers. A nephew with childhood ALD who died 10 years earlier had the same mutant allele as detected by Pst I restriction assay. This report is the first description of a mutant allele for ALD, at the cDNA level, and presents confirmatory evidence of ALD protein as the primary etiology of ALD.

ATP Binding Cassette Transporter, Subfamily D, Mem↗
PubMed1994

High-intensity proton and T2-weighted MRI signals in the globus pallidus in juvenile-type of dentatorubral and pallidoluysian atrophy.

Dentatorubral and pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder. An expanded CAG trinucleotide repeat sequence motif in a gene on the short arm of chromosome 12 has recently been identified in patients with DRPLA. Juvenile-type DRPLA is characterized by childhood onset and progressive myoclonic epilepsy (PME). According to the pathological study, the degeneration of the globus pallidus is more marked in this than in other types. We observed high-intensity signals in the globus pallidus on proton and T2-weighted magnetic resonance imaging (MRI) in a patient clinically diagnosed as juvenile-type DRPLA who had the expanded CAG trinucleotide repeat motif in the DRPLA gene. The globus pallidus may be affected in the early stages of this type of DRPLA, and MRI may be useful for the early diagnosis of DRPLA.

Atrophy↗