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Biomedical subjects

H Fukunaga

Publications and source records attributed to H Fukunaga.

At least 37 records · Page 2Linked to original sources
PubMed1998

Different manners of sarcoglycan expression in genetically proven alpha-sarcoglycan deficiency and gamma-sarcoglycan deficiency.

We investigated the expression of alpha-sarcoglycan, beta-sarcoglycan, gamma-sarcoglycan, and delta-sarcoglycan immunohistochemically in three patients with mutations of the alpha-sarcoglycan gene and a patient with a mutation of the gamma-sarcoglycan gene. Although each of the four sarcoglycans were decreased on the muscle membranes of all the patients, different expression patterns for each were seen among the patients. In patients with mutations of the alpha-sarcoglycan gene, beta-, gamma- and delta-sarcoglycans were relatively preserved as compared to greatly reduced alpha-sarcoglycan. However, the patient with a mutation of the gamma-sarcoglycan gene showed marked reduction of gamma-sarcoglycan as compared to partially preserved alpha- and beta-sarcoglycans, and well-preserved delta-sarcoglycan. These results suggest that each sarcoglycan component in sarcoglycanopathy does not decrease in the same manner, and that mutations of the sarcoglycan gene can be predicted, at least in part, by means of sensitive immunohistochemistry for each sarcoglycan.

Child↗
PubMed1998

Morphological observation of laryngeal motoneurons by means of cholera toxin B subunit tracing technique.

Gross anatomical and histological studies and immunohistochemical demonstration of cholera toxin B subunit used as a retrograde tracer were performed to investigate distinct muscle fibre bundles (MFBs) in the feline intrinsic laryngeal muscles (ILMs) and to characterize the respective supplying motoneurons in the nucleus ambiguus (AM). Distinct bundles were observed in all ILMs except the lateral cricoarytenoid muscle. Motoneurons supplying MFBs related to phonation located more medially than others. Motoneuron size did not differ between distinct MFBs in the same muscle. Neurodendrites for the cricothyroid muscle (CT) and adductor motoneurons extended dorsomedially, ventrolaterally and ventromedially, while those for the posterior cricoarytenoid muscle (PCA) motoneurons stretched dorsomedially and ventrolaterally. We conclude that distinct MFBs in ILMs play different roles in laryngeal functions, and that corresponding motoneurons have their own specific visceroneural nature allowing regulation of complex laryngeal functions. To receive information from the central nervous system, laryngeal motoneuron dendrites extended to regions locating respiratory, phonatory and swallowing neurons.

Animals↗
PubMed1998

Parvalbumin distribution in the musculature of the pharyngo-oesophageal segment.

The composition and size of muscle fibre types in the hyopharyngeus (HP), thyropharyngeus (TP), cricopharyngeus (CP) and cervical oesophageal muscle (CE) from 6 normal adult cats were investigated using parvalbumin (PA) immunohistochemistry. Fibre types I, IIA and IIB were identified in all muscles. HP and TP revealed predominance of type II fibres (74.8% and 75.2%, respectively), whilst CP and CE showed predominance of type I fibres (72.6% and 82.2%, respectively). The mean diameter of narrow fibres was greater in type II (23.9 microm) than in type I fibres (21.7 microm). The results seem to reflect the physiological features of each muscle, i.e. short rapid contractions of HP and TP, sustained contraction of CP and slow peristaltic movements of CE.

Animals↗
PubMed1998

[Clinical efficacy of sulbactam/ampicillin in comparison with cefotiam in the treatment of elderly patients with pneumonia].

Clinical efficacy and safety of pareteral sulbactam/ampicillin (SBT/ABPC) was compared with cefotiam (CTM) in a randomized clinical trial of pneumonia in the elderly at 13 National Hospitals of Kyushu island. 37 patients received SBT/ABPC 3 g i.v., b.i.d., and 31 patients received CTM 1 g i.v., b.i.d. for 7 to 14 days. 1. 68 patients (37 for SBT/ABPC and 31 for CTM) were evaluated for safety. No statistical differences were noted in the patients' backgrounds of either group. 2. The clinical efficacy of SBT/ABPC was 96.3% (26/27 cases) while CTM was 75.2% (17/23 cases). This was found to be statistically significant (Fisher's exact test: p < 0.05). 3. 100% of evaluated cases (10 for SBT/ABPC and 4 for CTM) showed bacterial elimination. 4. No side effects were observed in the study. 5. Abnormal laboratory findings were noted in 10.8% (4/37 cases) for SBT/ABPC and 3.2% (1/31 cases) for CTM. The major adverse events were mild elevation of GOT, GPT and A1-P for SBT/ABPC, and mild platelets overproduction for CTM. No statistical differences were noted in both groups. These results are consistent with SBT/ABPC as a highly effective antibiotic in the treatment of elderly patients with pneumonia.

Aged↗
PubMed1997

New missense mutation in the alpha-sarcoglycan gene in a Japanese patient with severe childhood autosomal recessive muscular dystrophy with incomplete alpha-sarcoglycan deficiency.

A new homozygous alpha-sarcoglycan (adhalin) gene mutation was found in a Japanese patient with severe childhood autosomal recessive muscular dystrophy (SCARMD). Muscle biopsy specimens from the patient showed marked reduction but not complete deficiency of alpha-sarcoglycan. The sequence of part of exon 3 of the alpha-sarcoglycan gene exhibited a cytosine to thymidine substitution at nucleotide position 220. Since the same mutation was not found in 100 normal control samples, this new alpha-sarcoglycan gene mutation is not a polymorphism but is presumed to be responsible for the marked reduction of alpha-sarcoglycan in skeletal muscle. Most patients with homozygous alpha-sarcoglycan gene mutation were reported to show complete alpha-sarcoglycan deficiency. Present case showed the homozygous missense mutation of alpha-sarcoglycan and associated with incomplete alpha-sarcoglycan deficiency and severe clinical phenotype.

Action Potentials↗
PubMed1997

Clinical findings, status of care, comprehensive quality of life, daily life therapy and treatment at home in patients with Parkinson's disease.

We analyzed patients with Parkinson's disease from various aspects such as clinical findings, the degree of independence in daily life, care environment, quality of life (QOL) and treatment at home. The subjects were 104 in- and outpatients (47 males and 57 females) seen at our hospital. The mean age was 69.85 years in the males and 70.35 years in the females. The disease most frequently developed at the age of 60-69 years and the disease duration was 5 years or more in 52 patients. Rigidity was the most common symptom (91 patients), followed by gait disturbance (n = 87) and tremor (n = 86). Levodopa/carbidopa was the drug most frequently used (77 patients). The number of patients treated by combination drug therapy increased with the duration of disease. Concerning the degree of independence in daily life, assistance was often necessary in bathing, dressing and undressing, toileting and walking. In particular, total assistance was necessary in patients with Hoehn-Yahr stage-IV and V disease. The comprehensive QOL was the lowest in terms of social activities, hobbies and leisure activities, followed in order by work and subjective QOL. QOL decreased in each item with the severity of the disease. Treatment at home was performed for 19 patients, of whom 11 are still being treated by our staff. Treatment at home combined with persons who care for the patient and in cooperation with other welfare resources may improve the patient's QOL. In diseases that require long-term care such as Parkinson's disease, a comprehensive care management system should be established from the aspect of the patient's QOL.

Activities of Daily Living↗
PubMed1997

Facioscapulohumeral muscular dystrophy: clinical diversity and genetic abnormalities in Japanese patients.

We studied 71 Japanese individuals, 42 patients (30 familial and 12 sporadic) suspected to have facioscapulohumeral muscular dystrophy (FSHD) and 29 family members, clinically and genetically using the chromosome 4qter DNA marker p13E-11. Early onset FSHD was detected in 7 patients, tortuosity of retinal arterioles and hearing impairment in 3 patients, progressive respiratory failure in 3 patients and limb-girdle type muscular weakness in 6 patients. Thirty-six (85.7%) FSHD patients, 3 asymptomatic family members and 1 of 35 healthy volunteers showed EcoRI digestion fragments shorter than 28kb. New mutations were detected in 25% of the patients with shorter EcoRI fragment. The age of disease onset appeared younger with successive generations in 6 parent-child pairs in FSHD families. We confirmed the existence of phenotypic and genetic diversities in Japanese patients with FSHD. It is still difficult to explain the phenotypic diversity merely by the size of the abnormal EcoRI fragment detected with the p13E-11 probe.

Arterioles↗
PubMed1997

Combination chemotherapy for malignant paraganglioma.

Treatment with a combination chemotherapeutic regimen consisting of cyclophosphamide, vincristine, and dacarbazine for malignant paraganglioma with hepatic metastasis is reported. A 51-year-old male presented with tumors in the retroperitoneal space and liver. The patient was diagnosed as having paraganglioma based on elevated levels of serum neuron-specific enolase, urinary catecholamine and vanillylmandelic acid, and on histological findings of the liver specimen. The patient was treated with this combination chemotherapy in repeated 21-day cycles. Temporary improvement in laboratory findings and a 20% reduction in the size of the hepatic masses were observed without severe adverse effects.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

[A case of tuberculous abscess in the chest wall close to the thickening pleural lesion following tuberculous pleuritis].

A 33-year-old woman with a history of right tuberculous pleuritis was successfully treated in December 1992 by administration of anti-tuberculous drugs, she demonstrated residual localized pleural thickening on chest computed tomography (CT) and gradually developed a subcutaneous mass in the right chest which became apparent in March 1993. In September, chest CT revealed a periocostal abscess in the right anterior chest wall close to the localized pleural thickening. The patient was diagnosed with tuberculous abscess in the right chest wall on confirmation of acid-fast bacilli in a needle aspiration material of the abscess, and was referred to our hospital. Anti-tuberculous chemotherapy was continued but the chest abscess grew, so on January 28, 1994 she underwent a resection of the abscess, the third costal cartilage and bone, and the parietal pleural lesion connected to the abscess. Histopathological examination showed that the abscess and parietal pleural lesion were compatible with tuberculosis, i.e. both lesions consisted of caseous necrosis and epitheloid cell granuloma, but acid-fast bacilli were not demonstrated in both lesions. After one year of postoperative anti-tuberculous chemotherapy, she was followed without any therapy for 3 years and there has been no recurrence to date. When a localized thickening pleural lesion remains after tuberculous pleuritis, complication of tuberculous abscess in the chest wall should be considered.

Abscess↗
PubMed1996

Characteristic expression of cell adhesion molecules in adhalin deficiency.

We have reported the reduction of the B1 subunit of laminin and that of heparan sulfate proteoglycan (HSPG) in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of cell adhesion molecules, including intercellular adhesion molecule-1 (ICAM-1), neural cell adhesion molecule (NCAM), and CD44 (HCAM), in four Japanese patients with adhalin deficiency, compared to other types of muscular dystrophy. We found that NCAM was upregulated in a fair number of muscle fibers, regardless of the type of muscular dystrophy. ICAM-1 was detected on the rare muscle cell membrane in all patients. CD44 was barely detected on the muscle cell membrane in adhalin deficiency, in contrast to the strong expression of CD44 which was observed in other types of muscular dystrophy. These findings suggest that a different degenerative or regenerative process is involved in adhalin deficiency compared to other types of muscular dystrophy.

Adult↗
PubMed1996

Multicenter evaluation of a colorimetric microplate antimycobacterial susceptibility test: comparative study with the NCCLS M24-P.

A colorimetric test method using the microplate culture technique for the determination of susceptibility of Mycobacterium tuberculosis against antimycobacterial agents was developed and evaluated by the multicenter study. The test method utilizes an oxidation-reduction dye, 2,3-diphenyl-5-thienyl-(2)-tetrazolium chloride (STC), as an indicator of mycobacterial growth. When compared to the presently available test method, some modifications were also included; lower inoculum density (10-fold dilution), inclusion of an inoculum diluted 1:100 as a growth control, and the preparation of inoculum preincubated in Middlebrook 7H9 broth and spectrophotometrically adjusted to McFarland #1 turbidity. The test method evaluated was highly precise and reliable to detect antimycobacterial resistances when the ATCC reference strains were tested. Also, the interpretations of the test result were highly comparable to those determined by the method of NCCLS M24-P, the % agreements ranging from 76.1% (ethambutol) to 91.3% (streptomycin). The test results were also comparable to those determined by Ogawa media; > 90% agreed with susceptible, intermediate, or resistant. The appearance of mycobacterial colonies on the test media was easily read, and the test results were more comparable to those of NCCLS M24-P. With these results, it can be concluded that the colorimetric microplate susceptibility test method described will be more suitable for clinical mycobacteriology laboratories.

Antitubercular Agents↗
PubMed1995

Abnormal expression of heparin sulfate proteoglycan on basal lamina of muscle fibers in two Japanese patients with adhalin deficiency.

We recently reported the selective reduction of the B1 subunit of laminin in two Japanese patients with adhalin deficiency. We here investigated immunohistochemically the expression of other components of the extracellular matrix (ECM), including collagen type IV, heparan sulfate proteoglycan can (HSPG), chondroitin-4-sulfate proteoglycan, decorin, and fibronectin in adhalin deficiency, compared with other types of muscular dystrophy. We found a reduction of HSPG on the basal lamina surrounding each muscle fiber in adhalin deficiency compared with HSPG in other diseases. This finding may be characteristic evidence of the disturbance of the sarcolemma-ECM interaction and the sarcolemmal instability in adhalin deficiency. Recently, a direct role of HSPG in fibroblast growth factor (FGF) signal transduction was demonstrated. Further investigation is required to determine if the dysfunction of FGF is relevant to the pathogenesis of adhalin deficiency.

Adolescent↗
PubMed1995

Inflammatory response in facioscapulohumeral muscular dystrophy (FSHD): immunocytochemical and genetic analyses.

To investigate the nature of the inflammatory response in facioscapulohumeral muscular dystrophy (FSHD), we analyzed mononuclear cells in muscle sections obtained from 18 FSHD patients and 8 controls. Monoclonal antibodies reactive for T cells, T cell subsets, B cells, and NK cells were used for cell typing. Macrophages were identified by acid phosphatase reaction. The localization of perforin, granzyme A, MHC-I and -II, dystrophin, and alpha-actinin antigens was also examined. We found that all FSHD patients, both familiar and sporadic cases, had greater amounts of mononuclear cellular infiltrates in muscle than controls, in whose specimens only few extra vascular mononuclear cells were counted. Seventy-two percent (13 of 18) of the patients had more than 50 inflammatory mononuclear cells per 1000 muscle fibers, and 33% (6 of 18) patients had numerous inflammatory cells exceeding 600 per 1000 muscle fibers (1835 +/- 482 SE). Nonnecrotic fibers invaded by mononuclear cells with either T8+, perforin+, or granzyme A+ were not observed in FSHD, while a few degenerating fibers were superficially invaded by T cells and macrophages. Occasional T cells were observed moving through the blood vessel wall. The increased number of necrotic fibers was paralleled by an increased number of inflammatory cells (r = 0.783, P = 0.0001). Genetic analysis, using the probes p13E-11, pFR-1, D4S139, and D4S163, was done in 6 patients (3 familiar, 3 sporadic) who had numerous inflammatory infiltrates. These 6 patients had small (< 28 kb) EcoRI fragments associated with the disease, and the disease was linked to 4q35. These results suggest that, in chromosome 4-linked FSHD: (1) inflammatory changes in muscle are a common histological feature; (2) mononuclear cellular infiltrates may enhance muscle fiber damage; but (3) T-cell-mediated cytotoxicity directed against muscle fibers is unlikely. We speculate that the immune effector mechanism in FSHD is different from that in previously reported inflammatory myopathies and Duchenne muscular dystrophy.

Adolescent↗
PubMed1994

Abnormal expression of laminin suggests disturbance of sarcolemma-extracellular matrix interaction in Japanese patients with autosomal recessive muscular dystrophy deficient in adhalin.

Dystrophin is associated with several novel sarcolemmal proteins, including a laminin-binding extracellular glycoprotein of 156 kD (alpha-dystroglycan) and a transmembrane glycoprotein of 50 kD (adhalin). Deficiency of adhalin characterizes a severe autosomal recessive muscular dystrophy prevalent in Arabs. Here we report for the first time two mongoloid (Japanese) patients with autosomal recessive muscular dystrophy deficient in adhalin. Interestingly, adhalin was not completely absent and was faintly detectable in a patchy distribution along the sarcolemma in our patients. Although the M and B2 subunits of laminin were preserved, the B1 subunit was greatly reduced in the basal lamina surrounding muscle fibers. Our results raise a possibility that the deficiency of adhalin may be associated with the disturbance of sarcolemma-extracellular matrix interaction leading to sarcolemmal instability.

Adult↗
PubMed1994

Combination of ascorbic acid and methylprednisolone pulse therapy in the treatment of idiopathic thrombocytopenic purpura.

Ascorbic acid, reported in 1988 to be effective for idiopathic thrombocytopenic purpura (ITP), is an attractive drug because of its lack of toxicity. Further studies are necessary in order to improve its effectiveness without increasing secondary effects. We present a chronic ITP patient treated with a combination of ascorbic acid and methylprednisolone pulse (MP) therapy who was previously treated with MP therapy alone. The effect of this combination therapy seems to be better than MP therapy alone. This therapy is worth further examination as another therapeutic choice due to its fewer secondary effects than the usual regimen of corticosteroids, splenectomy, and other immunosuppressive drugs.

Aged↗
PubMed1994

Dystrophin-related protein in Becker muscular dystrophy.

A benign Becker muscular dystrophy (BMD) patient with a marked decrease in dystrophin exhibited remarkable expression of dystrophin-related protein (DRP) on most of the muscle cell membrane. A phenotypic Duchenne muscular dystrophy patient with a truncated form of dystrophin exhibited no DRP expression on the muscle cell membrane except for the neuromuscular junction. Increased DRP expression might compensate for a lack of dystrophin in some BMD patients.

Adolescent↗