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Biomedical subjects

Y Fukuyama

Publications and source records attributed to Y Fukuyama.

At least 73 records · Page 4Linked to original sources
PubMed1997

Molecular genetic evidence of clinical heterogeneity in Fukuyama-type congenital muscular dystrophy.

Fukuyama-type congenital muscular dystrophy (FCMD) is an autosomal recessive severe muscular dystrophy associated with brain malformation. The gene responsible for FCMD was mapped to chromosome 9q31, a region in which convincing evidence of strong linkage disequilibrium between FCMD and mfd220 (D9S306) was recently found. FCMD is also characterized clinically by a peak motor function which, at best, allows patients to sit unassisted or slide on the buttocks. However, a small fraction of patients acquire the capacity to walk unassisted. Whether such ambulant cases belong to the FCMD spectrum or to a different disease entity has been a topic of considerable debate. We performed linkage analysis for ten families with ambulant cases using DNA markers flanking the FCMD locus. The mfd220 locus yielded a significant lod score of 3.09 for ambulant FCMD. We also found evidence for linkage disequilibrium between ambulant FCMD and mfd220. We further conducted haplotype analysis in FCMD siblings with different phenotypes, one of whom was ambulant while the other was not. The results indicate that the FCMD siblings share exactly the same haplotype at nine marker loci spanning 23.3 cM surrounding the FCMD locus. On the basis of these results, we conclude that, genetically, ambulant cases are, in fact, part of the FCMD spectrum.

Adolescent↗
PubMed1997

Diagnosis of visceral pleural invasion in resected lung cancer using a jet stream of saline solution.

BACKGROUND: Visceral pleural invasion by the tumor is an important prognostic factor in patients undergoing resection for lung cancer. We developed a method to detect more accurately the presence of visceral pleural invasion in resected lung cancer. METHODS: The surface of the visceral pleura over 90 resected peripheral tumors was irrigated twice with a jet stream of saline solution using a 20-mL syringe with a 21-gauge needle, and then the fluid, which contained desquamated cells, was collected for cytologic analysis. When cancer cells were found in the collected fluid, the tumor was judged to have invaded the visceral pleura. RESULTS: Thirty-eight (42%) resected tumors were identified as having visceral pleural invasion either by our new method or by pathologic examination. Twenty-four cases were detected by the jet stream of saline method alone, 5 by pathologic examination alone, and 9 by both techniques. The sensitivity and accuracy of the two approaches in the diagnosis of visceral pleural invasion were 87% and 94%, respectively, for our new method, and 37% and 73%, respectively, for pathologic examination (p < 0.0001). Furthermore, among 38 patients who had a tumor demonstrating visceral pleural invasion, 5 (13%) and 9 (24%) patients, respectively, had cancer cells in the pleural effusion and intrapleural lavage fluid. CONCLUSIONS: Our findings suggest that our method is useful in detecting cancer invasion of the visceral pleura, which is considered one of the causes of malignant effusion.

Adenocarcinoma↗
PubMed1997

Polymorphism analysis of Fukuyama type congenital muscular dystrophy (FCMD) siblings with different phenotypes.

Peak motor function in Fukuyama type congenital muscular dystrophy (FCMD) is generally considered to be no better than sitting without help or sliding on the buttocks. There are a few patients who acquire the ability to stand and a small fraction of our total congenital muscular dystrophy (CMD) population are able to walk at some point. These ambulant cases may reflect a broad spectrum of motor disabilities in the category of FCMD, or may represent another CMD entity, which closely resembles but is distinct from FCMD. Since the localization of the FCMD gene to chromosome 9q3 1 by Toda et al. in 1993 and 1994, polymorphism analysis of this disease has become possible. We describe correlations between clinical features and genetic analysis using microsatellite markers flanking the FCMD locus in two FCMD families each having two affected children with distinctly different motor abilities. The results demonstrate that two sets of FCMD siblings share exactly the same haplotype at nine marker loci spanning 23.3 cM, surrounding the FCMD locus. Our results provide genetic confirmation that some FCMD cases may acquire the ability to walk.

Adolescent↗
PubMed1997

The current status of postoperative complications and risk factors after a pulmonary resection for primary lung cancer. A multivariate analysis.

OBJECTIVE: The purpose of the present study is to identify the current postoperative complications after a pulmonary resection for primary lung cancer and the associated risk factors. METHODS: From 1988 to 1992, 291 patients with primary lung cancer, excluding T4 diseases, consecutively underwent a pulmonary resection at our institute. The observed postoperative complications were divided into non-life-threatening ones (simple arrhythmia, atelectasis, liver dysfunction, etc.) and life-threatening ones (respiratory failure, pyothorax, pneumonia, bronchopleural fistula, cardiac failure, cerebral infarction, myocardial infarction, etc.). Using logistic regression procedures, both univariate and multivariate analyses of the association between various perioperative factors and the incidence of postoperative complications were performed. RESULTS: Non-life-threatening complications occurred in 60 patients (20.6%) while life-threatening ones occurred in 36 (12.4%), and resulted in five in-hospital deaths (1.75%). Multivariate analyses showed that an age of 70 or older, a combined resection, an abnormality on preoperative ECG, and diffusing capacity of carbon monoxide (%DLco) below 70 were all independently associated with an increased non-life-threatening morbidity. On the other hand, an age of 70 or older, the need for a pneumonectomy, and a %DLco below 70 were also independently predominant risk factors for life-threatening morbidity. CONCLUSION: The risk factors for life-threatening morbidity therefore did not completely correspond to those for non-life-threatening morbidity. Since the mortality was quite low, even risk factors for life-threatening morbidity were not solely considered to be a contraindication for a major pulmonary resection.

Adult↗
PubMed1997

Failure in resection of multiple pulmonary metastases from colorectal cancer.

BACKGROUND: To clarify whether or not multiple pulmonary metastases from colorectal cancer are contraindicated for a surgical resection, we retrospectively evaluated the influence of the number of pulmonary metastases on both the postthoracotomy survival and the pattern of the first failure. METHODS: From 1981 to 1993, 36 patients underwent a complete resection for pulmonary metastases from colorectal cancer. RESULTS: Of the various factors investigated including gender, primary site, disease-free interval, tumor size, the number of metastases, type of resection, and the history of hepatic metastases, only the number of pulmonary metastases was found to be significantly related to postthoracotomy survival. The rate of disease-free survival at 5 years was 62% for solitary metastasis (n = 17), 35% for two metastases (n = 8), and 0% for four or more metastases (n = 11). The pattern of failure also differed according to the number of pulmonary metastases. In particular, the incidence of local recurrence at the primary site increased with the number of pulmonary metastases (ie, 1 of 17 patients with a solitary metastasis, 3 of 8 with two metastases, and 6 of 11 with four or more metastases). CONCLUSIONS: These results suggest that multiple metastases might indicate the presence of local recurrence at the primary site; therefore, in cases of multiple pulmonary metastases, the primary site should be thoroughly explored.

Colorectal Neoplasms↗
PubMed1997

K-ras and p53 mutations are an independent unfavourable prognostic indicator in patients with non-small-cell lung cancer.

We examined 159 consecutive cases of non-small-cell lung cancer (NSCLC) for a mutation at codon 12 of the K-ras gene and for a mutation of the p53 gene occurring in exons 5-8. Eleven (6.9%) had mutations of the K-ras (ras+) and 57 (35.8%) had mutations of the p53 (p53+). There were 95 cases (59.7%) with ras- p53-, seven cases (4.4%) with ras+/p53-, 53 cases (33.3%) with ras-/p53+ and four cases (2.5%) with ras+/p53+. The ras+ group had a worse prognosis than the ras group in all cases and in 107 early-stage cases (stage I-II, P<0.05). The p53+ group had a worse prognosis in 107 early-stage cases (P<0.01), but there was no statistically significant difference when 52 advanced-stage cases (stage III-IV) or all patients were considered. Both ras and p53 mutations were unfavourable prognostic factors in 94 cases with adenocarcinoma, but there was no statistical significance in 57 cases with squamous cell carcinoma. According to Cox's model, the pathological stage, ras mutation and p53 mutation were found to be independent prognostic factors. Our results suggest that ras and p53 mutations were independent unfavourable prognostic markers especially in the early stage of NSCLC or in adenocarcinoma.

Adult↗
PubMed1997

Bicycloillicinone asarone acetal: a novel prenylated C6-C3 compound increasing choline acetyltransferase (ChAT) activity from Illicium tashiroi.

Bicycloillicinone asarone acetal (1), a novel bicyclic prenylated C6-C3 compound, has been isolated from the woods of Illicium tashiroi and its structure has been elucidated by spectroscopic analyses and chemical degradation. Compound 1 was found to increase choline acetyltransferase (ChAT) activity in culture of P10 rat septal neurons.

Allylbenzene Derivatives↗
PubMed1997

Prospective study of nesidioblastosis in newborns and infants: hypoglycemic seizures, epileptogenesis and the significance of the C-peptide suppression test in pancreatectomy.

The long-term follow-up of chronic hyperinsulinemic seizures, epileptogenesis and other neurological complications in five patients who were treated with conservative therapy followed by pancreatectomy during the neonatal period and infancy, who were confirmed to have diffuse nesidioblastosis are described. The reaction pattern of the C-peptide (CPR) suppression test and its relation to the final extent of pancreatectomy was examined in four patients. The chronological change in electro-encephalography (EEG) and its epileptogenesis was also examined in each patient during hyperinsulinemic hypoglycemia, and during normoglycemia in a long-term post-pancreatectomy follow-up. All patients demonstrated several types of hypoglycemic seizures, ranging from apnea, erratic seizures, evolving to generalized/unilateral tonic-clonic or tonic seizures, myoclonic seizures and EEG abnormalities. Four of five patients still suffered from epilepsy at the age of 4-22 years. The reaction pattern of the CPR suppression test showed dichotomy, with a hyper-reactive pattern in two patients who required total pancreatectomy to control hypoglycemia, and a suppression pattern in two other patients treated with 90-95% pancreatectomy. Neonatal onset and subsequent myoclonic seizures were ominous signs of epileptogenesis to various types of intractable epilepsy and other neurological sequelae. A prompt diagnosis and pancreatectomy of a sufficient extent at the first operation are essential. The CPR suppression test may be useful for a prompt diagnosis and selection of the extent of pancreatectomy.

Blood Chemical Analysis↗
PubMed1997

An impedance matching of femoral-popliteal arterial grafts: a theoretical study.

We have proposed a mathematical method to investigate the matching conditions for an arterial graft in the femoral-popliteal region from a mechanical stand-point. Pulsatory blood flow, arterial wall motions, and conservation law are expressed by linear dynamical equations based on strict mechanical and constitutional considerations. To express the physiological blood flow in an actual arterial system, the tethering effects from the surrounding tissue and wall tensions were incorporated. The physiological parameters of arterial wall and tethering were utilized from reported experimental data. By complex analysis, mathematical expressions for the local impedance and reflection coefficient were obtained. They include not only blood properties such as viscosity and density, but also arterial properties including elastic modulus, radius, Poisson ratio, wall thickness, wall tension, frequency, and tethering effects from surrounding tissue. A matching condition was defined for minimizing the local impedance and reflection coefficient. The biophysical background was to reduce any mechanical mismatches, thus minimizing the disturbance of the flow velocity profile and shear stress distribution within the artery. Impedance matching in turn diminishes the negative factors for graft substitution represented by intimal hyperplasia and thrombosis. The calculated impedance and reflection coefficient inversed parabolically to functions of the resistance of the host artery, and there was one host arterial resistance that minimized the impedance and reflection coefficient. The present analysis revealed that for matching host artery with an elevated resistance, the dynamic elastic modulus of the wall of the graft that minimizes the impedance and reflection coefficient was increased. This indicates that for a host artery with a high resistance, an impedance matched stiff wall graft is preferable. For a large radius and a compliant host artery on the other hand, a large compliant graft should be linked. The present theoretical matching conditions will prevent anastomotic hyperplasia and thrombosis in graft substitution.

Blood Vessel Prosthesis↗
PubMed1997

Aggressive pulmonary metastasectomies for synovial sarcoma.

A 51-year-old woman underwent a bilateral wedge resection of lung metastases through a median sternotomy 10 months after an initial operation for synovial sarcoma of the left lower extremity. Since then, five right and two left posterolateral thoracotomies have been performed over a 6-year period. The patient is presently doing well 7 years after the initial operation of the left lower extremity without any evidence of recurrence.

Adult↗
PubMed1997

Stereoselective reductive metabolism of metyrapone and inhibitory activity of metyrapone metabolites, metyrapol enantiomers, on steroid 11 beta-hydroxylase in the rat.

Pharmacokinetics of metyrapone and metyrapol enantiomers was studied in the rat to determine the stereoselective reductive metabolism of metyrapone. The HPLC method using a chiral column was developed for the stereoselective analysis of metyrapol enantiomers in rat plasma. The AUC ratio of (-)- and (+)-metyrapol appeared in rat plasma after i.v. administration of metyrapone was about 3:1. The interconversion of (-)- or (+)-metyrapol to its antipode was negligible, and the reverse reaction from metyrapol to metyrapone was insignificant. There were similar kinetic parameters of (-)-metyrapol to those of (+)-metyrapol after i.v. administration of racemic metyrapol. These results indicate metyrapone displays product-stereoselective reductive metabolism in the rat. The inhibition of steroid 11 beta-hydroxylase by metyrapone, racemic metyrapol, (-)-metyrapol or (+)-metyrapol was analyzed in rat adrenal homogenates. Metyrapol was equally as potent as metyrapone in the inhibition of steroid 11 beta-hydroxylase and each enantiomer of metyrapol showed similar inhibitory activity on the rat adrenal steroid 11 beta-hydroxylase. These results indicate there is an insignificant difference in the inhibitory effects on steroid 11 beta-hydroxylase of metyrapol enantiomers, and that the inhibitory effects of metyrapol may be involved in the pharmacological activity of metyrapone in vivo.

Adrenal Glands↗
PubMed1996

Prenatal diagnosis of Fukuyama type congenital muscular dystrophy by polymorphism analysis.

Fukuyama type congenital muscular dystrophy (FCMD) is an autosomal recessive disorder characterized by a combination of primary muscular dystrophy of early infantile onset and brain malformation (lissencephaly type II). The identification of the FCMD gene locus at 9q31 opened the theoretical possibility of prenatal diagnosis. The authors conducted prenatal diagnosis in two unrelated FCMD families by analysis using nine microsatellite CA-repeat polymorphic markers flanking the FCMD locus, and calculated phenotype probabilities in fetuses with a computer program, LINKAGE. The fetus in family 1 showed a 99% probability of being healthy either as a normal homozygote or a heterozygote carrier and was born without signs of FCMD. In family 2, the fetus was diagnosed to have FCMD with at least 86% probability. The parents of this family decided to terminate the pregnancy and an abortus showed brain malformations characteristic of an FCMD fetus.

Brain↗
PubMed1996

Tumor-induced hypercalcemia and parathyroid hormone-related protein in lung carcinoma.

BACKGROUND: Although lung carcinoma is the most common cause of tumor-induced hypercalcemia (TIH), the precise incidence of TIH remains obscure. Furthermore, the role of parathyroid hormone-related protein (PTHrP) has not been clearly elucidated. METHODS: This study included 690 consecutive patients who were newly diagnosed as having lung carcinoma between 1989 and 1994 (379 adenocarcinomas, 207 squamous cell carcinomas, 75 small cell carcinomas, and 29 large cell carcinomas). All patients were treated for lung carcinoma and were also periodically monitored for their serum level of calcium (Ca). Hypercalcemia was defined as a serum Ca concentration higher than 11 mg/dL. The serum levels of PTHrP (109-141) were measured by a C-terminal-region-specific radioimmunoassay. RESULTS: TIH was observed in 17 of 690 patients (2.5%). All 17 patients demonstrated an advanced stage of lung carcinoma (Stage III or IV), 10 squamous cell carcinomas, 5 adenocarcinomas, 1 small cell carcinoma, and 1 large cell carcinoma. In 15 patients, the serum level of C-PTHrP (109-141) was substantially high, ranging from 99 pmol/L to 890 pmol/L (normal range, 21-50.7 pmol/L). There was no significant difference in the serum PTHrP level between patients with or without bone metastasis. The reduction of tumor burden decreased both the serum level of PTHrP and that of Ca in parallel. The median survival time after diagnosis of TIH was only 27 days. CONCLUSIONS: TIH in lung carcinoma was most likely attributable to PTHrP, and its occurrence appears to be an ominous prognostic sign.

Adult↗
PubMed1996

Kinetic analysis of recurrence and survival after potentially curative resection of nonsmall cell lung cancer.

BACKGROUND: About two-thirds of the patients with nonsmall cell lung cancer (NSCLC) who undergo a potentially curative resection eventually suffer from recurrent disease. However, it has yet to be elucidated as to how survival after recurrence is influenced by different variables, including timing, type of recurrence, or other clinicopathological features. There have been few studies concentrating on the kinetics of growth of occult micrometastatic tumor cells that eventually manifest as tumor recurrence. METHODS: We retrospectively reviewed the charts of 197 patients who developed recurrence after a potentially curative resection for NSCLC. RESULTS: The median disease-free interval was a little over 1 year (395 days), as was the median postrecurrence survival-383 days. We created a model for the kinetics of recurrence by assuming that: (1) a tumor of 10(9) cells is the usual limit of detection, (2) patients generally die before the tumor reaches 10(12) cells, and (3) it takes 1 year for average lung cancer cells to show a 10-fold increase. The model indicated that as much as 10(9) tumor cells should have been present immediately after the operation. Alternatively, the residual tumor cells should have an accelerated growth after the surgery. CONCLUSIONS: These models indicate the importance of developing a sensitive detection method for occult metastatic cells and to understand the tumor dormancy mechanism.

Adult↗