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H Fukunaga

Publications and source records attributed to H Fukunaga.

At least 55 records · Page 3Linked to original sources
PubMed1994

Pigmentary retinopathy with nephrotic syndrome, Ménétrier's disease, and diabetes mellitus.

A patient with pigmentary retinopathy, nephrotic syndrome, Ménétrier's disease, and diabetes mellitus is presented. Other complications were congestive heart failure, hypothyroidism, hypertension, and hypertriglyceridemia. Hypogenitalism was also suspected. Pigmentary retinopathy is known to associate with many systemic diseases, which are classified into several syndromes. This case superficially resembles Alström's disease due to the common characteristics of pigmentary retinopathy, diabetes mellitus, renal disease, and hypogenitalism. But clinically and histologically, there are distinct differences. To our knowledge, this association has never been reported.

Consanguinity↗
PubMed1994

[Detection of HTLV-I proviral DNA by PCR method from bronchoalveolar lavage cells of HTLV-I carriers].

To elucidate whether or not human T-lymphotropic virus type I (HTLV-I) infected cells exist in the lungs of HTLV-I carriers, we examined bronchoalveolar lavage (BAL) cells for the presence of HTLV-I proviral DNA by the polymerase chain reaction (PCR) method. The cells were harvested from HTLV-I seropositive individuals with or without various respiratory diseases. The HTLV-I pX region was detected from separated BAL cells by dot blot hybridization after PCR in all 14 HTLV-I seropositive individuals tested. Four out of these 14 individuals were asymptomatic HTLV-I carriers, while the other ten had symptoms, including those of bacterial pneumonia, chronic respiratory tract infection, interstitial pneumonia, atypical mycobacterial infection, bronchitis, mediastinal lymphadenitis and bronchial asthma. HTLV-I proviral DAN was detected in BAL cells from patients with a normal proportion of lavage lymphocytes and a normal CD4/CD8 ratio. These findings suggest that HTLV-I infected cells may commonly exist in the lower respiratory tract and alveolar space without producing characteristic symptoms/signs, and that any causal relation to the pulmonary lesion must therefore be carefully elucidated, in HTLV-I carriers.

Aged↗
PubMed1993

Long-term follow-up of patients with Duchenne muscular dystrophy receiving ventilatory support.

We, retrospectively, examined the clinical course, decline in pulmonary function, and requirements for ventilatory assistance in 54 patients with Duchenne-type muscular dystrophy (DMD) who were followed in the muscle disease ward of the National Hospital in Kagoshima, Japan, over the past 20 years. The percentage of the predicted vital capacity (%VC) declined in relation to age and stage of disease. Most patients required assisted ventilation when the %VC fell below 10%. Twenty patients were treated with a negative pressure chest respirator. Six of these died at the mean age of 23.2 years after being on the respirator for a mean period of 18 months. Fourteen patients are surviving at a mean age of 23.5 years after being on the respirator for a mean period of 39 months.

Adolescent↗
PubMed1993

Thalamic- and cerebellar-projecting interpolaris neuron responses to afferent inputs.

Thalamic- and cerebellar-projecting interpolaris neuron responses to afferent inputs from the temporomandibular joint (TMJ) and/or the masseter muscle (Mm) were examined in rats. Of 230 neurons tested, 24 could be antidromically stimulated from the contralateral ventral posteromedial thalamic nucleus (VPM), and 27 of 91 neurons tested were stimulated from the ipsilateral posteromedial part of crus II of the cerebellar cortex. None had dual projections. The thalamic-projecting neurons were recorded in the dorsomedial region of the interpolaris; most cerebellar-projecting neurons were at the medial border of the interpolaris. Ten of 24 thalamic- and 17 of 27 cerebellar-projecting neurons received nociceptive information. Afferent inputs from the TMJ and the Mm converged on 6 of 24 thalamic-projecting neurons and on 16 of 27 cerebellar-projecting neurons. In both the thalamic- and cerebellar-projecting neurons, there was no difference between the non-nociceptive and nociceptive neurons in mean antidromic latency. The results suggest that the interpolaris integrates and relays afferent inputs from deep oral structures.

Afferent Pathways↗
PubMed1992

Failure to detect HTLV-I by in situ hybridization in the biopsied muscles of viral carriers with polymyositis.

Direct infection of muscle fibers by human T-lymphotropic virus type I (HTLV-I) has recently been reported in a patient with polymyositis infected with both HTLV-I and human immunodeficiency virus (HIV). Coinfections of these viruses are frequently found in the United States. In Kagoshima, Japan, patients with polymyositis have a significantly increased incidence of seropositivity to HTLV-I alone, when compared with the general population of Kagoshima. In this study, we examined muscle tissue from 6 HTLV-I-positive patients with polymyositis from Kagoshima. To detect HTLV-I products, sensitive immunohistochemistry and in situ hybridization analysis were performed. These were compared with muscle fibers from a well-characterized transgenic mouse model which expressed HTLV-I tax. No specific signals were detected in the biopsied muscles of patients with polymyositis infected with HTLV-I alone. HIV co-infection may, therefore, augment HTLV-I expression through either immunosuppression or direct viral interactions.

Adult↗
PubMed1992

Lymphocyte subsets in muscle biopsies of human T-lymphotropic virus type I carriers with polymyositis.

Recent epidemiologic studies have shown that human T-lymphotropic virus type I (HTLV-I) is associated with polymyositis. To clarify the histological features of HTLV-I positive polymyositis, we studied muscle tissue from two groups of patients with polymyositis, those seropositive for HTLV-I (5 patients) and those seronegative (5 patients). We examined the lymphocyte subsets in muscle biopsies using monoclonal antibodies. In the endomysium, a variable number of T-lymphocytes and macrophages and a smaller proportion of B-lymphocytes were found in both groups. In both HTLV-I positive and negative patients a variable number of T-lymphocytes and macrophages, with a smaller number of B-lymphocytes, were found in the perimysium. As a whole, the T-lymphocytes were predominantly of the helper/inducer phenotype in both groups. We failed to find any specific phenotype distribution among T-cells infiltrating the muscle of HTLV-I carriers with polymyositis.

Adult↗
PubMed1992

[Clinical and histochemical findings in spinal muscular atrophy].

The childhood form of the spinal muscular atrophy (SMA) is classically subdivided into three groups on the basis of a combination of age of onset, milestones of development and age of survival: acute Werdning-Hoffmann (type I), intermediate Werdnig-Hoffmann (type II) and Kugelberg-Welander disease (type III). Now we examined 7 cases of type I and 9 cases of type II on clinical and histochemical ground. Of the total of 16 cases, 5 cases had a family history of the disease. (1) In type I, three were males and 4 females. The onset was within 30 days and the disease was manifest before or at delivery in 3 cases. The progression was so severe. All cases were dead by 10 months. They showed generalized hypotonia, abnormal respiration and could not sit without support. In type II, five were males and 4 females. The onset of the disease was between the age of 3 and 15 months. The progression was slow. All patients couldn't walk by themselves at all but 7 of them had abilities to sit without support. Clinically it was easy to classify type I from type II. (2) The most characteristic histochemical findings of both types were group atrophy, fiber hypertrophy, fiber type predominance and fibrosis. Though there was a slight difference between two types in histological pattern, the basis was so similar. There is controversy about the proper classification of recessive childhood SMA. Now it is suggested that the majority of both acute and chronic cases are allelic, similar to the patterns of Duchenne and Becker forms of muscular dystrophy.

Biopsy↗
PubMed1992

[Clinical significance of serum cardiac myosin light chain I in patients with Duchenne muscular dystrophy].

The cardiac myosin light chain I (LCI) is one of the cardiac muscle structural proteins. A sensitive immunoradiometric assay kit for LCI by using LCI monoclonal antibodies is developed. We estimated LCI in the patients with Duchenne muscular dystrophy (DMD) and Kugelberg-Welander disease (KW). The results suggested that LCI has close relationships with the functional disturbances of skeletal muscles, especially disturbances of pulmonary ventilation. Therefore we studied properties and localizations of LCI in the skeletal muscles by Western blotting and immunohistochemical methods. In Western blotting method LCI monoclonal antibodies have a band of 27 KD proteins of skeletal muscles. LCI has also found to be localized in type 1 fibers in frozen sections of biopsied of human skeletal muscles. LCI was measured from 47 patients with DMD and 8 patients with KW. The average serum LCI levels in the patients with DMD were 11.79 ng/dl and its levels in the patients with KW were in the normal range (under 2.5 ng/dl). Among 12 patients receiving negative pressure chest respirator, the levels of LCI were also under 2.5 ng/dl. Serum LCI decreased with increasing age and reduced physical activity. The levels of LCI has obvious positive correlations with CK and myoglobin. These results suggested that the measurements of serum LCI are useful as one of the markers of disease severity and the determination of suitable time of using respirator.

Adolescent↗
PubMed1992

[Clinical study on myocardial imaging with beta-methyl-p-(123I)-iodophenyl-pentadecanoic acid in patients with mitochondrial myopathy].

Myocardial imaging with beta-methyl-p-(123I)-iodophenyl-pentadecanoic acid (123I-BMIPP), a new radiopharmaceutical designed to evaluate myocardial fatty acid metabolism, was performed in 7 patients with mitochondrial myopathy to detect their myocardial damages in comparison with 201Tl myocardial imaging. These patients were divided into 4 chronic progressive external ophthalmoplegia (CPEO) cases, 2 mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) cases and 1 myoclonus epilepsy with ragged-red fibers (MERRF). In visual assessment, we observed more myocardial segments with decreased uptake of 123I-BMIPP compared to 201Tl in MELAS cases than in CPEO cases. The mean myocardial uptake of 123I-BMIPP was higher than that of 201Tl in CPEO cases. On the other hand, in MELAS and MERRF cases, the mean myocardial uptake of 123I-BMIPP was lower than that of 201Tl. Abnormal findings suggesting myocardial damages were observed in echocardiogram and/or in electrocardiogram in MELAS and MERRF cases, while no such abnormal findings were observed in CPEO cases. Along with the previously reported experimental result that the impairment of rat myocardial mitochondria decreased myocardial uptake of 125I-BMIPP, these results suggest that 123I-BMIPP may be useful to detect myocardial damages in patients with mitochondrial myopathy.

Adolescent↗
PubMed1991

Vacuolar myositis with expression of both MHC class I and class II antigens on skeletal muscle fibers.

We describe here a 10-year-old patient with high levels of serum IgE and inflammatory myopathy whose muscle fibers exhibit excessive autophagy. Previous studies have demonstrated surface expression of class I MHC antigens on muscle fibers from patients with inflammatory myopathy. The muscle fibers of this patients showed marked expression of both class I and class II MHC antigens. The reaction products were demonstrated not only on sarcolemma but also in and around some vacuoles. Both CD4-positive and CD8-positive T-lymphocytes were noted in inflammatory exudates surrounding these fibers but B-lymphocytes were rare. We hypothesize that myocyte expression of both class I and class II antigens may play a role in the pathogenesis of this new type of inflammatory myopathy.

Autoimmune Diseases↗
PubMed1991

[Advanced stage of giant axonal neuropathy].

A woman has appeared normal during her neonatal and childhood period except for a short stature. Her parents were healthy and non-consanguineous. At the age of 8, she noticed difficulty in climbing stairs and had tendency to fall. In her 13th year, she developed marked scoliosis and genu valgum. Physical examination at 14 years of age revealed a gentle and shy child of short stature with brown-black kinky hair. Neurological examinations revealed progressive mental retardation, optic nerve atrophy, moderate and coarse nystagmus on lateral and vertical gaze, atrophic tongue with fasciculations, slow and scanning speech, distal muscular weaknesses with diffuse atrophies in the four extremities and sensory deficiencies in all modalities with a glove-stocking type distribution. At the age of 15, she was unable to walk without a wheelchair. During the course she showed slowly progressive muscular weakness, ataxia and decreasing sensation especially in the lower extremities. She died of infection of the respiratory and urinary tracts at the age of 25. Pathologically the abnormalities in the biopsied and autopsied sural nerve were characterized by an advanced stage of nerve fiber degeneration without giant axons. The phrenic nerve obtained at autopsy at 1 to 10 cm from axon terminal revealed the presence of several large focal axonal swelling of 15-20 microns in diameter. On the other hand, sections of the phrenic nerve at 15 cm from axon terminal displayed a mild to moderate reduction in the number of myelinated fibers without giant axons. The difference of pathological findings among these specimens seems to depend on the time as well as the site of the examination.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

[A case of scapuloperoneal atrophy with rigid spine having lobulated fibers in muscle biopsy].

The patient, a 52-year-old male, noticed abnormalities on walking at about 20 years of age, followed by slowly progressive muscle weakness of arms and neck. The family history was negative. He showed muscular atrophy and weakness with a preferential involvement of the scapular, arms and peroneal muscles. Deep tendon reflexes were absent. He had a limited range of motion in the spine, but the onset was unclear. Creatine kinase (CK) was elevated (324 IU/L) and the EMG study showed myogenic pattern. Muscle biopsy was obtained from the biceps brachii muscle; on NADH dehydrogenase stain, there was subsarcolemmal increase in the oxidative enzyme activity showing "lobulated fiber" mostly seen in type 1 fibers. On electron-microscopy, the sub-sarcoplasmic areas which had high NADH activity, contained many mitochondria and glycogen particles. However, iodine-glycogen complex spectrum analysis pattern and debranching enzyme activity were normal. CT scan revealed low density in the paravertebral muscles, suggesting degeneration. This is a rare type of scapuloperoneal atrophy different from Emery-Dreifuss syndrome, rigid spine syndrome and FSH type muscular dystrophy.

Adult↗
PubMed1991

[Respiratory failure and its care in Duchenne muscular dystrophy].

Respiratory failure is the major cause of death in patients with Duchenne muscular dystrophy (DMD). In this report we retrospectively examined the clinical courses, pulmonary function, progression of scoliosis and the time of assisted ventilation. Forty nine patients, aged 2 to 27 years were studied and thirty eight patients of them were examined twice in some intervals. The subjects of examinations included pulmonary function studies consisting of spirometry, measurement of the thoracic scoliosis measured according to the method of Cobb and room air arterial blood gas values. The results were the following; 1) The decline in percentage values of vital capacity (%VC) began at age 8 or 9 and decreased consistently with age. Finally %VC reached under 10 percent at mean age of 21.53 with respiratory failure. 2) Thoracic scoliosis measured according to the method of Cobb increased further with advancing age. The greater the angle of curvature, the more likely the development of respiratory failure. 3) Sixteen patients received negative pressure ventilator (chest respirator: CR) between 16.5 and 25.1 years of age. Twelve patients used part-time (mainly night-time) ventilator and four patients used it in full-time. The average PaCO2 and PaO2 before administration of ventilator were 67.49 mmHg and 71.46 mmHg, respectively. The levels after ventilation were 58.01 mmHg (PaCO2) and 82.09 mmHg (PaO2).

Adolescent↗
PubMed1990

Differentiation of bronchogenic carcinoma from secondary changes, obstructive pneumonitis and/or collapse by I-123 IMP lung imaging.

Serial lung images with N-isopropyl-p-[I-123]-iodoamphetamine (I-123 IMP) were obtained to assess the imaging findings and to clarify the lesion to uptake relationships in 74 lesions in 73 patients with various histological types of bronchogenic carcinoma. A decreased uptake area was observed in all 74 lesions in the initial one or two-min I-123 IMP image. The initial image was analogous to a Tc-99m MAA lung perfusion image in 70 patients in whom both lung imaging procedures were performed. The imaging findings changed following this initial phase. At 4 hr, the lesion was depicted as either areas of decreased uptake or increased uptake or a combination of the two. Comparison between the lesion findings in the 4-hr I-123 IMP images, radiograms and removed specimens revealed that areas of decreased uptake corresponded to the cancerous portions of the lung mass or pleural effusion and areas of increased uptake corresponded to inflammatory portions including obstructive pneumonitis and/or collapse. Thus, the 4-hr I-123 IMP lung images can be used to discriminate the cancerous portion from associated secondary changes, obstructive pneumonitis and/or collapse.

Adenocarcinoma↗
PubMed1990

Increased number of beta-adrenoceptors in cultured heart cells after propranolol withdrawal.

We have studied the effects of propranolol on the number and function of beta-adrenoceptors of cultured heart cells from mice. After 24 hrs in culture, heart cells were incubated for 48 hrs with either standard medium or medium containing propranolol. After a washing period of 30 min, beta-adrenoceptors were measured with a receptor binding assay. The dissociation constant (Kd) did not differ between control and cultured heart cells pretreated with propranolol. By contrast, the maximum number of binding sites (Bmax) was 2.5 times higher in propranolol-pretreated than control cultured heart cells. The increase in the rate of beating and the elevation of cyclic AMP content induced by 10(-5) M isoproterenol were significantly higher for cultured cells pretreated with propranolol than for control cells. These results indicate a correlation between an increased number of beta-adrenoceptors and increased physiological responses of cultured heart cells after propranolol withdrawal.

Animals↗
PubMed1990

[Asymptomatic infection of Legionella pneumophila in four cases with pulmonary diseases].

In view of the wide-spread existence of legionellae in cooling-tower and other environmental water, asymptomatic infection of this organism could occur. In order to verify the possibility of colonization of legionellae at lower respiratory tract of patients with various pulmonary diseases, a total of 22,036 sputum samples from in- and out-patients at National Sanyoso Hospital were examined during a five-year period from September, 1984 to August, 1989. Four (0.073%) out of 5,502 cases were culture-positive for L. pneumophila. L. pneumophila strains were isolated from expectorated, subsequently washed sputum samples of two male and two female patients with respiratory tract diseases. The identification of the isolates was genetically confirmed by the fluorometric microplate DNA-DNA hybridization method. The serogroup (SG) and viable counts of L. pneumophila per ml of sputum of each patient were as follows: 73 y/o female K.H., SG-6, 10(3) CFU; 75 y/o male H.J., SG-5, 10(4) CFU; 61 y/o female M.S., SG-5, 10(5) CFU; and 77 y/o male M.G., not-agglutinable against SG-1-6 antisera, 10(4) CFU. None of the four patients was clinically suspected of legionellosis and antibody titer of paired sera remained 1:64 or lower than 1:32. From these findings, we concluded that L. pneumophila can cause, though quite rarely, asymptomatic infection in human respiratory tract. None of the environmental samples obtained from in- and out-side of the Hospital was culture-positive for legionellae. Thus, the source of infection has remained unknown.

Aged↗
PubMed1990

[Database for patients with Duchenne muscular dystrophy].

We had a plan to arrange database for patients with Duchenne muscular dystrophy in Japan. The purpose of this projects will make it possible to design accurate and serial evaluation of patients and conduct therapeutic trial. The items of input are composed of two cards. One card is basic card which fills out mainly patients' basic and genetic informations, the other is course card which records symptoms, muscle strength, joint range of motion, laboratory studies including pulmonary function testing and therapeutic trials. These cards are written by physicians of dystrophic wards in national hospital twice a year and are sent to the statistical coordinating center of database. On the other hand outputs include annual report and optional output which are requested by each investigator. Now 519 basic cards and 1026 course cards have completed. In this paper we tried to output about some results of the data obtained. These outputs include the age of initial gait and difficulty in walking, mean muscle strength by functional grade and the change of muscle strength during the administration of some drugs.

Adolescent↗