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Biomedical subjects

H Ueyama

Publications and source records attributed to H Ueyama.

At least 55 records · Page 3Linked to original sources
PubMed1998

Immunohistochemical study of clathrin in distal myopathy with rimmed vacuoles.

Clathrin-coated vesicles are involved in three receptor-mediated intracellular transport pathways: export from the Golgi apparatus, transfer of lysosomal enzymes from the Golgi apparatus to lysosomes, and endocytosis at the plasma membrane. Seeking evidence of transport abnormalities in distal myopathy with rimmed vacuoles (DMRV), we performed immunohistochemistry for clathrin in muscle biopsy specimens from patients with this disorder or other neuromuscular disorders, and also in control muscle samples resected in orthopedic procedures. While most myofibers from control muscle did not stain for clathrin, some fibers revealed finely granular sarcoplasmic staining. In specimens from patients with Duchenne and Becker muscular dystrophy, amyotrophic lateral sclerosis, peripheral neuropathy, and DMRV, numerous clathrin-positive granules were often scattered through the sarcoplasm and seen to a lesser extent in subsarcolemmal regions. Quantitative immunohistochemical assessment showed more reactivity for clathrin in DMRV than in controls and other diseased muscles, particularly in atrophic fibers and type 2 fibers. Not all strongly clathrin-positive muscle fibers contained rimmed vacuoles, although most fibers with vacuoles were clathrin positive. The result suggests that the lysosome system is activated and receptor-mediated intracellular transport pathways function appropriately in the muscles of DMRV patients.

Adolescent↗
PubMed1998

Measurement of blood volume using indocyanine green measured with pulse-spectrophotometry: its reproducibility and reliability.

OBJECTIVE: To systematically investigate the reproducibility and reliability of a newly developed, less invasive approach of estimating blood volume (BV), using indocyanine green (ICG) measured with pulse-spectrophotometry. DESIGN: Prospective, clinical study. SETTING: Surgical unit at a university hospital. PATIENTS: Twenty-two patients undergoing general anesthesia for elective surgery and seven healthy volunteers. INTERVENTIONS: Catheters were inserted into the forearm veins of healthy volunteers for the administration of ICG and blood sampling for the measurement of hemoglobin concentration. MEASUREMENTS AND MAIN RESULTS: The distribution volumes of ICG in seven healthy volunteers were estimated repetitively following three or four consecutive intravenous administrations at 30-min intervals. A low intrasubject coefficient of variation of 3.94 +/- 2.03 (SEM) % and a reasonable intersubject coefficient of variation of 13.3 +/- 5.52% (in mL/kg) for the BV measurements were obtained. In addition, ICG was administered to 22 patients, first under general anesthesia by a bolus, and then by a bolus with a constant-rate infusion. The ICG blood concentration was noninvasively measured with pulse-spectrophotometry. The blood concentration time courses following both bolus and constant-rate infusion were well fitted by the one-compartment model, indicating that the distribution equilibrium of ICG is instantaneous. The distribution volumes estimated following bolus injection correlate closely with the distribution volume estimated based on constant-rate infusion administration (r2 = .90). CONCLUSIONS: The BV estimation with a bolus injection of ICG and pulse-spectrophotometry is reliable, as reflected by the reproducible BVs estimated in the same subject. The integrated pulse-spectrophotometry monitoring system offers a less invasive and useful tool for bedside estimation of BV.

Adolescent↗
PubMed1998

Clinical and genetic studies of spinocerebellar ataxia type 2 in Japanese kindreds.

OBJECTIVES: We report the results of clinical and genetic studies from 2 related Japanese kindreds with spinocerebellar ataxia type 2 (SCA2). MATERIAL AND METHODS: Family A showed 19 patients through 4 generations, while family B showed 6 patients, including dizygotic twin brothers, through 3 generations. We performed clinical, radiological, neurophysiological, and genetic analyses in the family members. RESULTS: Neurologic analysis of 13 affected patients revealed a mean age at onset of 43.5 years. The most common neurologic finding was cerebellar ataxia with deep sensory disturbance. Slow saccades was found only in the younger patients below age 35 years. Nerve conduction studies revealed subclinical sensory neuropathy. Brain MRI showed the presence of pontocerebellar atrophy. Genetic study using PCR revealed that all affected patients had an expanded CAG allele in the ataxin-2 gene, which led to a final diagnosis of SCA2. CONCLUSION: SCA2 may be more clinically heterogeneous than previously thought. PCR is useful in differentiating SCA2 from other types of inherited ataxia.

Adult↗
PubMed1998

High Fas ligand expression on lymphocytes in lesions of ulcerative colitis.

BACKGROUND: The pathogenesis of ulcerative colitis is unclear, but cytotoxic T lymphocytes infiltrating the mucosa have been implicated in mucosal damage. The Fas ligand (FasL), expressed on cytotoxic T lymphocytes, induces apoptosis in cells expressing Fas. AIM: To analyse FasL expression in affected colonic mucosa to ascertain Fas-FasL interaction in ulcerative colitis. METHODS: FasL mRNA was quantified in colonic mucosal specimens from healthy subjects and patients with ulcerative colitis or Crohn's disease, using the competitive reverse transcription polymerase chain reaction. FasL mRNA localisation was determined by in situ hybridisation. Expression of Fas in colonic mucosa was analysed immunohistochemically. Phenotypes of lamina propria lymphocytes that expressed FasL were analysed by flow cytometry. RESULTS: FasL mRNA was strongly expressed in active ulcerative colitis lesions, but not in those associated with active Crohn's disease or active proctitis-type ulcerative colitis. In situ hybridisation showed that FasL mRNA expression occurred in mononuclear cells infiltrating lesions. Fas was expressed in epithelial cells in ulcerative colitis and Crohn's disease, and in normal subjects. Cytometry showed that FasL was expressed in CD3 lymphocytes infiltrating the lamina propria in active lesions. CONCLUSIONS: FasL is expressed in CD3 lymphocytes infiltrating into ulcerative colitis but not Crohn's disease lesions, suggesting that Fas-FasL induced apoptosis participates in the mucosal damage of ulcerative colitis.

Acute Disease↗
PubMed1998

Proteasomes in distal myopathy with rimmed vacuoles.

In a previous report we suggested that muscle fibers in distal myopathy with rimmed vacuoles (DMRV) were degraded by both lysosomal proteolysis (cathepsins) and Ca2+-dependent, nonlysosomal proteolysis (calpain). Given recent evidence of abnormal ubiquitin accumulation in rimmed vacuoles, we examined the role of the ATP-ubiquitin-dependent proteolytic pathway (proteasomes) in myofiber degradation in this myopathy. Immunohistochemically, proteasomes (26S) were located in the cytoplasm in normal human muscle, but the staining intensity was weak. Quantitative analysis showed more reactivity for proteasomes in DMRV muscles and, to a lesser extent, in muscles from muscular dystrophy, polymyositis, and amyotrophic lateral sclerosis patients. In DMRV, proteasomes often were located within or on the rim of rimmed vacuoles, and in the cytoplasm of atrophic fibers. Ubiquitin accumulation was marked within rimmed vacuoles and was seen less extensively in the cytoplasm of atrophic fibers. The latter proteins colocalized well. In other diseased muscles, proteasomes and ubiquitin showed a positive reaction in the atrophic or necrotic fibers. The results indicate increased proteasome and ubiquitin in these muscle fibers as well as in other diseased muscle fibers. We suggest that the ATP-ubiquitin-proteasome proteolytic pathway as well as the nonlysosomal calpain and the lysosomal proteolytic pathway may participate in the muscle fiber degradation in DMRV.

Adenosine Triphosphate↗
PubMed1998

Estrogen receptor gene polymorphism and generalized osteoarthritis.

OBJECTIVE: Generalized osteoarthritis (OA) is a common disease, more often affecting women than men. Although genetic factors are known to be the major risk factor, the genes related to its development are as yet undetermined. We investigated the association between estrogen receptor gene polymorphisms and generalized OA. METHODS: Pvu II and Xba I restriction fragment length polymorphisms (RFLP) of estrogen receptor gene were analyzed in 65 Japanese patients with generalized OA and in 318 healthy control women. The RFLP were represented as Pp(Pvu II) and Xx(Xba I), with capital and small letters signifying the absence and the presence of restriction sites, respectively. RESULTS: Estrogen receptor genotype PpXx, with the combination of the Pvu II and Xba I RFLP, is a significant risk factor for the disease subset (odds ratio 1.86, 95% confidence interval 1.03-3.24; p = 0.039). The association of this genotype was more obvious in patients with severe radiographic changes and in younger patients. CONCLUSION: Some variant of the estrogen receptor gene is a genetic marker for generalized OA. How this genotype affects development of the disease remains to be elucidated.

Age Factors↗
PubMed1998

[A case of chronic progressive external ophthalmoplegia presenting as inflammatory myopathy].

A 64-year-old female had slowly progressive bilateral external ophthalmoplegia, blepharoptosis and muscle weakness of the extremities since age 30. Laboratory examination showed an elevation of serum CK level. Biopsied specimens from the left biceps and the left orbicularis oculi muscles revealed myopathic change with infiltration of mononuclear cells. In addition, some ragged-red fibers and a few cytochrome c oxidase-negative fibers, which are characteristic of mitochondrial myopathy, were observed. Polymerase chain reaction analysis of mtDNA in the muscles showed multiple mtDNA deletions. On administration of prednisolone (initial dose, 60 mg/day), blepharoptosis and muscular strength improved transiently and serum CK level was normalized but external ophthalmoplegia was not improved. We diagnosed our case as chronic progressive external ophthalmoplegia (CPEO). This is the first report of CPEO presenting as inflammatory myopathy.

DNA, Mitochondrial↗
PubMed1997

Alanyl aminopeptidase from human seminal plasma: purification, characterization, and immunohistochemical localization in the male genital tract.

Alanyl aminopeptidase (AAP) was purified to homogeneity from human seminal plasma. The calculated molecular weight of the purified enzyme was approximately 137,000+/-5,000 from light scattering, 140,000 (main) and 137,000 (minor) from non-denatured PAGE and 153,000 from SDS-PAGE in the absence or presence of 2-mercaptoethanol (2-ME). These findings suggest that the enzyme is monomeric in form in human seminal plasma. The enzyme hydrolyzed several amino acid 4-methyl-coumaryl-7-amide (MCA) substrates. The order of Kcat/Km values of AAP at optimal pH (pH 7.5) was Ala- > Lys-Ala- > or = Met- > Leu- > Phe- > Arg- > or = Arg-Arg- > Lys- > Gly-MCAs. AAP was potently inhibited by bestatin, leuhistin, actinonin, amastatin, and 1,10-phenanthroline. These findings suggest that AAP is an aminopeptidase. We determined that the amino acid sequence of the first 22 residues of the enzyme was Ser1-Thr-Thr-Pro-Ser5-Ala-Ser-Ala-Thr-Thr10-Asn-Pro-Al a-Ser-Ala15-Thr-Thr-Leu-Asp-Gln20-Ser-Lys-. This sequence was completely coincident with that downstream of the transmembrane site of human intestinal alanyl aminopeptidase N (CD13). We also isolated cDNA encoding AAP from human prostate cDNA library, sequenced its structure, and confirmed human seminal plasma AAP to be identical with alanyl aminopeptidase N. We postulated that native human seminal plasma alanyl aminopeptidase is released into the seminal plasma after the specific site is cleaved by elastase or an elastase-like enzyme. The enzyme level in human seminal plasma determined by single radial immunodiffusion was 5.2+/-3.2 mg/100 ml (mean+/-SD, n=20) in individuals 20-47 years of age. AAP was immunohistochemically stained in the luminal site-cell membrane of epithelial cells in the prostatic gland and ductuli efferentes of the testis.

Adult↗
PubMed1997

Elevated soluble intercellular adhesion molecules-1 in inflammatory myopathy.

INTRODUCTION: We evaluated the serum level of soluble intercellular adhesion molecule-1 (sICAM-1) in patients with polymyositis (PM) and dermatomyositis (DM) and investigated the correlation between the serum level of sICAM-1 and clinical findings. MATERIAL AND METHODS: We measured the serum level of sICAM-1 using an enzyme-linked immunosorbent assay in 19 untreated patients with inflammatory myopathy (14 patients with PM and 5 patients with DM), 20 patients with other neuromuscular disorders in which immunological mechanisms are unlikely to be involved, and 14 normal healthy controls. RESULTS: The serum level of sICAM-1 was significantly higher in patients with PM/DM compared with patients with other neurological disorders and control subjects. The sICAM-1 level was, however, not correlated with the clinical characteristic including disease severity, the duration of illness, and the serum level of CK. CONCLUSION: These findings suggest that sICAM-1 is involved in the inflammatory process of PM and DM.

Adult↗
PubMed1997

Calpain and cathepsins in the skeletal muscle of inflammatory myopathies.

To clarify the significance of intracellular lysosomal (cathepsins B, L and H) and extralysosomal (calpain) proteolytic systems in the process of muscle fiber degradation in inflammatory myopathies, biopsied muscle specimens were examined from patients with polymyositis (PM) and dermatomyositis (DM). Generally, in specimens from patients with PM and DM, but not in those from normal controls, muscle fibers surrounding inflammatory infiltrates or in the perifascicular regions, and occasionally mononuclear cell infiltrates demonstrated positive immunostaining for calpain and cathepsins B, L, and H. In addition, enzyme activities of cathepsins B and L increased in specimens with inflammatory myopathy. These results suggest that calpain and cathepsins play a significant role in the process of muscle fiber destruction in inflammatory myopathy.

Adult↗
PubMed1997

Multiple hemorrhagic gastric ulcers due to polyarteritis nodosa.

A 73-year-old man developed refractory multiple hemorrhagic gastric ulcers. Emergent gastrectomy was performed, and the diagnosis of polyarteritis nodosa was made by histologic evaluation of the resected stomach. Although gastric ulceration is a relatively rare complication of polyarteritis nodosa, it should be considered in the differential diagnosis of refractory hemorrhagic gastric ulcers. Emergent surgery may be indicated in such cases.

Aged↗
PubMed1997

Severity of neurological disorders in elderly residents of a farming town in Kyushu, Japan; prevalence of disability in activities of daily living.

Activities of daily living (ADL) were evaluated in 421 elderly people, aged 60-95, who resided in 27 randomly selected communities in the town of Mifune, Japan. The disability of ADL increased with age, and was particularly rapid after age 80. Most of the elderly were independent; the prevalence of dependent elderly ranged from 0.7% (in feeding) to 9.5% (in working). The prevalence of dependent elderly increased markedly after age 80 in all ADL items, especially those related to behavior such as ambulation, stair climbing, transfers, and working (i.e., employment, housekeeping, etc.). These results emphasize the importance of community health care of the elderly for preventing and treating neurological disorders which comprised the main cause of disabilities of behavioral ADL.

Activities of Daily Living↗
PubMed1997

[Effects of polychlorinated biphenyls on regenerating myelin of peripheral nerve in rats].

The effects of polychlorinated biphenyls (PCB) on regenerating myelin of the peripheral nerves were investigated in rats. The sciatic nerves were crushed at the mid-thigh level on the last of 32 days of oral administration of PCB. The sciatic nerves were biopsied from the crushed regions at 4, 8 and 12 weeks after crushing. The nerves were fixed in 2.5% glutaraldehyde buffered with 0.1 M sodium cacodylate and embedded in epon 812. The ultrathin sections were analysed by JEOL JEM-1210 with EDAXDX-4. The chloride which was composed of PCB was increased in myeline on 4 weeks after crushing. Freeze fracture study of myelines of sciatic nerves on 8 and 12 weeks after crushing showed an increase of intramembranous particle on 12 weeks and an increase of caveolae on 8 weeks. These results suggest that PCB may affect the plasmamembrane of shwann cell and induce the disturbance of myeline.

Animals↗
PubMed1997

[Elevation of serum creatine kinase in the patients with Kanemi Yusho].

18.9% of the patients with Kanemi Yusho showed an elevation of serum creatine kinase, however, the cause is still unknown. The relation between exercise, dehydration, thyroid hormone and concentration of PCB was studied. Dehydration, hyperexercise and PCB affected the elevation of creatine kinase. No relation between PCB and thyroid hormone or creatine kinase and thyroid hormone was observed. PCB may change the permeability of muscle plasma membrane.

Creatine Kinase↗
PubMed1997

[A case report of Bickerstaff brainstem encephalitis with attention to electronystagmographic findings].

We evaluated brainstem function by using electronystagmography in a 43-year-old woman with typical Bickerstaff brainstem encephalitis. She developed fever, drowsiness, total opthalmoplegia, swallowing disturbance, areflexia, and muscle weakness. On electronystagmography, eye-tracking test showed ataxic pattern with impaired smooth pursuit eye movement. The horizontal optokinetic nystagmus test showed diminished pattern. Caloric test revealed the vestibular hypofunction and the increase of caloric nystagmus on eye fixation. We considered these findings were attributed to the disturbance of pontine tegmentum, especially reticular formation. These findings were similar to those observed in patients with Fisher syndrome.

Adult↗
PubMed1997

Isolated third nerve palsy due to sarcoidosis.

We present a 28-year-old man with isolated third nerve palsy as the initial manifestation of sarcoidosis. MRI demonstrated abnormal enhancement of the cisternal portion of the right third nerve. One month after steroid therapy, he showed almost complete recovery with resolution of the contrast enhancement on MRI. This patient illustrates the usefulness of enhanced MRI for detection of cranial nerve lesions of sarcoidosis.

Adult↗