Annual bibliography series of congenital muscular dystrophies--cobblestone lissencephalies: Series I (1997).
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Brain↗
Biomedical subjects
Y Fukuyama
Publications and source records attributed to Y Fukuyama.
Bibliography of congenital muscular dystrophies-cobblestone lissencephalies: Series II (1998).
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Animals↗
[A case of acute theophylline intoxication with repeated status convulsivus].
We report a 6-month-old female infant with status convulsivus which appeared during intravenous drip infusion of aminophylline. She had an extremely high serum theophylline concentration (79 micrograms/ml), which was effectively reduced by plasmapheresis and dialysis. Three days later, she developed status convulsivus again, though her serum theophylline was undetectable at that time. A CT on 14th day of illness revealed mild widening of frontal sulci and Sylvian fissure. The patient apparently recovered her healthy condition, but psychomotor developmental delay, especially in speech and social behavior, was noted at the age of 2 years 6 months (DQ = 55). A delay of myelination was observed on brain MRI at 4 year 1 months, suggesting an irreversible brain injury caused by theophylline intoxication.
Brain↗
[Visiting nurse for a terminal ALS patient].
The authors' hospital is a 585-bed hospital under the direct management of the National Health Insurance System. The hospital has been providing visiting nurses for the past 8 years, who work from local medical centers and the Visiting Nurses Department. Thirty-seven patients have received such home care, among whom 8 had intractable disease. Patient S was a 46-year-old woman who suffered from amyotrophic lateral sclerosis (ALS). The onset of the disease was in April, 1993, when the patient experienced muscular atrophy in both legs and deteriorating muscular strength. The diagnosis was definitive in 1995. On March 1, 1998, the patient received emergency hospitalization for breathing difficulties and aspiration pneumonia, and on March 5 underwent tracheotomy. A cannula had to be inserted for tubal feeding, and the physician in charge explained to her family that her prognosis was 3 months. Both the patient and her family desired home care, and the patient returned home on April 11. Respecting the wishes of the patient, the visiting nurse provided support so that home treatment could be continued. In the end, the patient lived at home while receiving home treatment for 7 months. Through the support provided by the visiting nurse, efforts were made to keep the patient's condition stable, and she was able to continue home treatment and living at home for a higher quality of life.
Amyotrophic Lateral Sclerosis↗
[Rethinking the visiting nurse system at a major regional hospital].
The authors' hospital is a National Health Insurance System Hospital near four cities. It serves as the central hospital for a region with a population of about 330,000 people. The hospital has 585 beds and sees an average of 1,500 outpatients each day. The average number of hospitalized patients each day is 500. Its visiting nurse department is located in a regional medical center and functions as a "Visiting Nurse Center". The purpose of the present study was to review the visiting nurse system at our hospital that has been operating for the past 8 years. The motivation for this review was our intention to actively increase the number of people advantage of such services, and attempt to provide continuous care for each individual patient. By looking back on the system as it has been practiced, its procedures, and results during the 8 years from 1990 to 1997, we can consider points for improvement from among the problem points discovered. The problems uncovered in our practice of home nursing care are listed below. 1. It is difficult to present a list with an estimated period for the release from the hospital. 2. Instructions for leaving the hospital are not sufficiently detailed. 3. Arranging the schedule and actual visits for diagnosis and treatment is complex. 4. The system for cooperation with the activities that are done in the hospital is insufficient. 5. The system for cooperation with local public health nurses is insufficient. 6. The system for managing equipment is insufficient. 7. The 24-hour support system for terminal patients is inadequate.
Community Health Nursing↗
Il-1, EGF, and HGF suppress the antiviral activity of interferon in primary monkey hepatic parenchymal cells.
The purpose of this study was to elucidate the mechanism behind low efficacy of interferon therapy to hepatitis C virus infected patients by using primary monkey hepatic parenchymal cells as a surrogate of primary human liver cells. The effects of various cytokines on the antiviral activity of IFNs in the monkey hepatic cells were studied to search for physiological inhibitors. Interleukin-1 alpha, EGF, and HGF showed suppressive effects on the antiviral activity of IFN-alpha, -beta in primary monkey hepatic cells when examined by the yield reduction method using vesicular stomatitis virus (VSV). In contrast, 50 ng/ml of TNF and IL-6 had no suppressive effect on the IFN-induced antiviral state in the hepatic cells.
Animals↗
[Outpatient chemotherapy with oral UFT (tegafur and uracil) and cisplatin against metastatic non-small cell lung carcinoma--an effective case].
A 64-year-old man underwent a left pneumonectomy for squamous cell carcinoma of the left lung in July 1995. In May 1996, right pelvic bone metastasis occurred and was well controlled by the concurrent chemoradiotherapy; UFT (600 mg/body, day 1-14) and cisplatin (80 mg/m2, day 8) with a total 50.4 Gy of irradiation. In August 1996, left renal metastasis occurred, but regressed after two cycles of combination chemotherapy with UFT and cisplatin. In January 1997, multiple lung metastasis occurred. In accordance with the patient's request, combination chemotherapy was performed at the outpatient clinic. UFT (250 mg/m2) was given orally every day while cisplatin (60-80 mg/m2), fractionated in 3 or 5 days, was intravenously administered at an interval of 4 weeks or longer. The patient has continued the treatment for one year without serious (G3, 4) adverse events. During the treatment, the tumor growth was slow with a repeated cycle of progression and regression. The outpatient chemotherapy using UFT and cisplatin is considered to be useful, especially for the better quality of life of patients.
Administration, Oral↗
Prenatal diagnosis of Fukuyama type congenital muscular dystrophy in eight Japanese families by haplotype analysis using new markers closest to the gene.
We conducted prenatal diagnosis by haplotype analysis, using newly developed microsatellite markers, in eight Fukuyama type congenital muscular dystrophy (FCMD) families. In addition to six new families, two previously reported families were reexamined by haplotype analysis including detection of an ancestral founder haplotype (138-183-301) for 3 microsatellite markers closest to the FCMD gene, designated D9S2105-D9S2107-D9S172, the distances of which from the FCMD gene are presumed to be approximately 140, approximately 20, and approximately 280 kb, respectively. Five fetuses from five families were diagnosed as nonaffected, and were subsequently confirmed to be healthy. Three fetuses of the other three families were diagnosed as having a high probability of being affected by FCMD. In the prenatal diagnosis conducted for these eight families, the ancestral founder allele was observed in 13 of 16 (81%) FCMD-bearing chromosomes. Detection of the ancestral haplotype facilitated achieving accurate prenatal diagnosis of FCMD. The brains of all three fetuses prenatally diagnosed as FCMD-affected showed the initial stage of cortical dysplasia, strong evidence of FCMD.
Brain↗
beta-Carboline alkaloids as matrices for UV-matrix-assisted laser desorption/ionization time-of-flight mass spectrometry in positive and negative ion modes. Analysis of proteins of high molecular mass, and of cyclic and acyclic oligosaccharides.
We report that commercially available beta-carbolines (nor-harmane (9H-pyrido[3,4-b]indole), harmane (1-methyl-9H-pyrido[3,4-b]indole), harmine (7-methoxy-1-methyl-9H-pyrido[3,4-b]indole), harmol (1-methyl-9H-pyrido[3,4-b]indol-7-ol), harmaline (3,4-dihydro-7-methoxy-1-methyl-9H-pyrido[3,4-b]indole) and harmalol (3,4-dihydro-1-methyl-9H-pyrido[3,4-b]indol-7-ol)), are useful MALDI matrices at 337 nm, for cyclic oligosaccharides (cyclodextrins, range 972-1290 Da), acyclic oligosaccharides (range 342-828 Da) and high molecular mass proteins (range 23,290-66,525 Da) in both positive and negative modes. This was investigated by using time-of-flight (TOF) mass spectrometers of different sensitivities, equipped with and without pulse extraction facilities. A comparison with conventional matrices for carbohydrates (DHB and DHB/HIC) indicates that beta-carbolines provide the same level of sensitivity and resolution in the positive mode, but offer the advantage of high levels of sensitivity and resolution in the negative mode. Harmaline has been found to be specially effective for the analysis of high-mass proteins in both modes, and also exhibits excellent experimental reproducibility of the results owing to the homogeneous crystallization of the analyte-matrix mixture over the entire sample surface area. Harmane and nor-harmane are both excellent matrices for high-mass proteins also. As MALDI matrices, beta-carbolines permit measurement of sulfated sugars in the negative ion mode as ([M-H]), and of neutral sugars and proteins as both [M + H]+ and [M-H]- in appropriate modes.
Carbolines↗
HLA class I and class II expression of pulmonary adenocarcinoma cells and the influence of interferon gamma.
BACKGROUND: The clinico-biological significance of HLA (both class I antigen and class II one) expressed on tumor cells still remains controversial. METHODS: Tumor cells were freshly separated from 33 surgical specimens of pulmonary adenocarcinoma. The tumor cells were incubated for 24 h in the presence or absence of IFN-gamma (130 International Units/ml). After incubation, the cells were cytocentrifuged onto glass slides and immunostained with either an anti-HLA class I (A, B, C) monoclonal antibody or anti-HLA class II (DR) one. RESULTS: In 22 of 33 cases (66.7%), the HLA class I were individually expressed by more than 60% of tumor cells while so were the HLA class II in 15 (45.4%). No significant correlation was observed between the HLA class I expression and the HLA class II one. The proportion of HLA class I-positive tumor cells correlated with neither the grade of histological differentiation nor the stage of disease. In contrast, the proportion of HLA class II-positive tumor cells correlated with both the grade of histological differentiation and the stage. In most cases, IFN-gamma was found to increase the proportion of class II-positive tumor cells as well as that of class I-positive cells. CONCLUSIONS: The above findings thus suggested that the HLA class II expression might therefore represent a manifestation of cellular differentiation and that IFN-gamma may, as a result, have the potential to differentiate cancer cells.
Adenocarcinoma↗
A new method for evaluation and dietary therapy of congenital: deficiencies of amino acid metabolic enzymes. Linear system analysis and optimization of feedback inputs for the metabolic pathways of lysine, methionine and isoleucine.
We intended to elucidate an integrated mathematical model of amino acid metabolism and we propose a system for optimization treatment of disturbed metabolic states caused by congenital enzyme deficiencies. Our analysis focused on the metabolic pathway starting at asparaginic acid proceeding to isoleucine, methionine and lysine. The rate of change in the concentration of the biochemical species was expressed as 21 linear rate equations. We obtained the rate constants and the magnitude of feedback from reported experimental data. Linear systems analysis revealed that the metabolic system under study was stable but uncontrollable. These properties were insensitive to changes in the magnitude of feedback. To show the effect of optimizing the feedback so that it minimizes the square of the concentration of the species and the control input, we analyzed the impulse response of the species, transient response and the singular value of the system for four cases; (1) at the physiological state without optimizing the feedback, (2) at the physiological state attained after optimizing the feedback, (3) at the pathophysiological state attained with enzyme deficiency states for lysine and methionine metabolism without optimizing the feedback, and (4) at the pathophysiological state attained after optimizing the feedback for enzyme deficiencies. In the enzyme deficient model, the impulse response oscillated and lasted longer than that in the physiological state. These changes appeared even in the species on other branched pathways. The singular value was elevated in the enzyme deficient state. By optimizing the feedback, all the impulse responses in the enzyme deficient state recovered to nearly those in the normal physiological state. Similarly, the transient response and the singular value in the enzyme deficient state recovered to nearly the normal physiological values. We elucidated the numerical value of the feedback gain for this optimization. The present analysis is useful for the evaluation of the integrated properties of amino acid metabolism and the optimization technique is potentially of use for determining a treatment course for congenital metabolic enzyme deficiencies.
Amino Acid Metabolism, Inborn Errors↗
Proceedings of the First National Meeting of the Study Group on Seizures in Infancy and Early Childhood Tokyo, April 4, 1998.
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Animals↗
Long-term outcomes of pediatric moyamoya disease monitored to adulthood.
The long-term outcomes of 25 patients with childhood moyamoya disease (18 with the transient ischemic attack [TIA] type and seven with the non-TIA type), who were monitored to adulthood (older than 20 years of age), were evaluated in terms of residual clinical symptoms, intellectual development, and activities of daily living. Surgical treatment was performed in ten patients, encephaloduroarteriosynangiosis in nine, and superficial temporal artery to middle cerebral artery anastomosis plus encephalomyosynangiosis in one. Only seven with the TIA type (three surgically and four medically treated) demonstrated good activities of daily living without TIA or headache and normal IQ. Two patients with the TIA type and three with the non-TIA type demonstrated poor outcomes. Three of these patients with poor outcomes had renal artery stenosis. Surgery was effective in nine. Since the long-term outcomes of patients with childhood moyamoya disease are generally poor, surgical treatment is believed to be an effective procedure for preventing the progression of clinical symptoms.
Activities of Daily Living↗
Long-term survivors with pN2 non-small cell lung cancer after a complete resection with a systematic mediastinal node dissection.
OBJECTIVE: A substantial number of surgical patients with pN2 disease have survived longer than 5 years without any evidence of recurrence, although the surgical indications for those patients remain controversial. The present study was performed in order to clarify the clinical characteristics of the long-term survivors with pN2 disease. METHODS: We retrospectively reviewed the cases of 111 patients with pN2 disease who had undergone a complete resection with a systematic mediastinal lymph node dissection from 1974 through 1991. RESULTS: Of the 111 patients with pN2 disease, 20 survived longer than 5 years after a surgical resection. When both the pre- and post-operative conditions were compared between the long-term survivors and the others, the long-term survivors were characterized by significantly higher proportions of cN0 disease (P = 0.031), pT1 disease (P = 0.004), skip metastasis without hilar node metastasis (P = 0.028), and metastasis of a single mediastinal station (0.044). Of those characteristics, only the likelihood of having cN0 disease could be pre-operatively determined. The survival rate of such a population with cN0-pN2 disease was 34.5% at 5 years and 29.6% at 10 years after a complete resection, respectively. CONCLUSIONS: Pathologic N2 patients with some favorable prognostic factors can survive long-term after a complete resection combined with a systematic mediastinal lymph node dissection. At present, due to the lack of any effective adjuvant therapy, a systematic mediastinal node dissection should be routinely performed even in patients with cN0 disease.
Carcinoma, Non-Small-Cell Lung↗
Antioxidative bromoindole derivatives from the mid-intestinal gland of the muricid gastropod Drupella fragum.
Three new bromoindoles, 6-bromo-5-hydroxyindole (1), 6-bromo-4, 5-dihydroxyindole (2), and 6-bromo-4,7-dihydroxyindole (3), were isolated from the midintestinal gland of the muricid gastropod Drupella fragum. The structures of 2 and 3 were elucidated mainly by NMR spectroscopic analyses of their acetyl derivatives, whereas the structure of 1 was established by spectroscopic methods and total synthesis. Antioxidative activity for compounds 1-3 was evaluated by the POV method, and compound 1 was found to have as strong an antioxidative potency as BHT.
Acetates↗
Demographic features of patients seeking cosmetic surgery.
The demographic features of 415 patients seeking cosmetic surgery were investigated from a psychiatric point of view. Of the 415 patients, 198 (47.7%) were found to have mental disorders according to ICD-10 including: 17 with schizophrenia, 20 with other persistent delusional disorders, 33 with depressive episode, 47 with neurotic disorders, 42 with hypochondriacal disorder, five with paranoid personality disorder and 14 with histrionic personality disorder. The rate of subjects with poor social adjustment was 56.0%. It was noteworthy that such a considerable number of patients with mental disorders or with poor social adjustment had sought cosmetic surgery. Distinct gender differences were found: male subjects were characterized to have a greater number of mental disorders, especially dysmorphophobia (other persistent delusional disorders plus hypochondriacal disorder) and showed the narrow age range between teenage and young adult age when they were preoccupied with their 'deformity', and poor social function. A history of frequent operations was not considered to be an indicator for mental abnormality. The diagnostic issue in dysmorphophobia is briefly described.
Adolescent↗
Intraoperative intrapleural hypotonic cisplatin treatment for carcinomatous pleuritis.
BACKGROUND AND OBJECTIVES: We recently developed a new intraoperative intrapleural hypotonic cisplatin treatment for carcinomatous pleuritis found at thoracotomy in non-small cell lung cancer patients. In the present study, the efficacy and adverse events of this treatment as well as the pharmacokinetics of cisplatin in the blood after the treatment were evaluated. PATIENTS AND METHODS: Twenty-one patients received the treatment for 15 minutes after completing the intrathoracic surgical procedures. The total and free platinum levels in the blood of five patients were then measured. As a control, 29 patients without such treatment were reviewed retrospectively. RESULTS: The survival rates in the treatment and non-treatment groups were similar. The pleural disease free survival of the treated patients was, however, significantly higher than that of the non-treated patients. Such pleural disease as effusion and the growth of the pleural disseminated tumors only appeared in three of the 21 (14%) treated patients while 26 of 29 (90%) non-treated patients had clinically detected pleural disease. The blood platinum levels after the treatment were extremely low and such low levels probably induced no systemic adverse events after the treatment. The only adverse event of this treatment was an increase in the postoperative drainage volume. CONCLUSIONS: These observations seem to suggest that intraoperative intrapleural hypotonic cisplatin treatment for carcinomatous pleuritis found at thoracotomy can, at least, delay the appearance of the pleural disease without any adverse events.
Adult↗
Alpha-Interferon for the treatment of idiopathic sudden sensorineural hearing loss.
We have employed alpha-interferon (IFN-alpha), an anti-viral agent, in the treatment of severe idiopathic sudden sensorineural hearing loss (ISSHL). Forty-two patients were studied and had an average hearing ability of > or = 70 dB before treatment. We also examined 2'-5' oligoadenylate synthetase (2,5A-S) activity, one of the parameters indicating anti-viral activity of IFN, to investigate the relationship between the suppression of viral proliferation and prognosis and explain the pathogenesis of ISSHL. Complete recovery was found in 27 patients (64.3%) after IFN therapy. Increased 2,5A-S activity was observed on the 3rd day of IFN therapy in 24 of the 27 patients who completely recovered. No severe adverse events were reported after IFN therapy. Findings suggest that IFN therapy may be effective and safe in the treatment of ISSHL and calls for further investigation.
2',5'-Oligoadenylate Synthetase↗