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We present a case of Marshall-Smith syndrome, characterized by accelerated osseous maturation, craniofacial anomalies, failure to thrive, psychomotor delay, pulmonary dysfunction, and limited life expectancy. Recently, Marshall-Smith syndrome's clinical variability is becoming apparent and the phenotype is expanding. Our case had craniofacial anomalies, including prominent eyes, external strabismus, blue sclera, flat nasal bridge, and micrognatia. Moreover, she showed extension of the hip joint, hypotonia, and arachnodactyly. She was referred to our hospital due to dysmorphic facies at 7 mo and overgrowth was noticed with a height of 74.2 cm (+2.7 SD). Radiographic surveys demonstrated accelerated osseous maturation with a bone age of 1 yr 6 mo at the chronological age of 10 mo and broad proximal phalanges. We diagnosed this case as Marshall-Smith syndrome according to clinical features and radiographic findings. Except for one episode of broncho-pulmonitis which required hospitalization, she has not had other severe infections up to date. Presently, she is 1 yr 9 mo; 88.0 cm (+2.1 SD) and 10.4 kg (-0.3 SD). This case suggests that there are patients with Marshall-Smith syndrome without life-threatening complications.

Purpse: Patients with diffuse cortical dysplasia in one cerebral cortex, such as those with cortical hemihypertrophy, are well known to have intractable epileptic seizures and severe developmental delay of mental state and motor skills. We previously reported that the region of cortical dysplasia shows the histologic characteristics of neuronal hyperactivity with impaired γ-aminobutyric acid (GABA)-mediated transmission. Resections of one hemisphere have historically been performed to control such epileptic seizures, although often they also cause severe complications such as massive bleeding and hydrocephalus. To decrease the severity and incidence of such complications, a procedure known as modified hemispherectomy was recently developed. We performed functional hemispherectomies on two cases of intractable epilepsy caused by unilateral cortical dysplasia. The purpose of this study was to evaluate the long-term clinical effects of this surgery for epileptic seizure, including the effects on mental state and motor skills. Patients and Methods: Case 1 was a 4-year-old boy whose epilepsy first occurred 2 weeks after birth. The seizures were focal motor seizures and secondarily generalized seizures, which began from the region of the right face. The seizures occurred >l00 times every day and sometimes involved the pharyngeal region, leading to apneic episodes. The patient was severely retarded and had no head control, could not respond to his name being called, and could not track movements visually. Case 2 was a 2–year 10–month-old boy, whose epilepsy appeared at birth and whose seizures were simple motor and secondarily generalized seizures occumng 20–50 times a day. He was a floppy infant, could not sit, could crawl only on his elbows, and could not speak. The epileptic seizures of both cases were intractable to any anticonvulsant therapy. Case 1 had unilateral cortical hypertrophy of left hemisphere on magnetic resonance imaging (MRI), and case 2 had a large cortical hyperplasia occupying almost two thirds of the left hemisphere. The epileptic seizures were confirmed to originate from the region of the cortical dysplasia by both ictal and interictal physiological examinations, including EEG and single-photon-emission computed tomography (SPECT). Functional hemispherectomy with resection of the parietal cortex and fiber disconnection was performed in case 1. Case 2 received a modified hemispherectomy. which involved only a fiber-disconnection procedure, and was originally developed by Simizu. The clinical effects of the surgical treatment were evaluated in both cases with regard to epileptic seizures and mental and motor development. The follow-up period was 3.5 years in case 1, and 1.5 years in case 2. Results: Unfortunately, case 1 had a massive intracranial hemorrhage and hydrocephalus shortly after the operation. Thereafter, however, he became stable and could eat, respond to stimuli, such as the voice of his mother, and follow movements visually. His epileptic seizures disappeared after treatment with only phenytoin (PHT). In case 2, the seizures disappeared completely with no anticonvulsant therapy from the day of the operation. His right hemiplegia recovered remarkably, and he can now stand and walk, and he can use his right fingers. He can speak, sing a song, and seems happy. Although soon after operation, he was almost completely deaf in the right ear, he can now respond even to a whisper. Conclusions: Because patients with diffuse cortical dysplasia often have severe epileptic seizures and developmental delays, surgical treatment should be considered to control the epilepsy and allow developmental “catch-up”, and should be performed, if necessary, as soon as possible after determining its intractability to anticonvulsant medication.

In this paper, noise analysis of the optical fiber sensor array system based on the phase generated carrier (PGC) technique with time division multiplexing (TDM) will be discussed. In this system, the laser beam with sinusoidal frequency modulation from a laser diode is gated by a LN(LiNbO3) intensity modulator, amplified by a booster erbium‐doped fiber amplifier (EDFA) sent into array. The returned pulses from the sensor array are amplified by pre‐EDFA, converted to electric pulses, and demultiplexed into each channel by the analog switch followed by the interpolation filter, and finally demodulated. Results of both noise analysis and experimental data will also be discussed.

Using two radioimmunoassays (RIAs) for endothelin-1 (ET-1) with and without a substantial cross-reactivity with ET-3, we have measured the plasma ET-1-like immunoreactivity (-LI) level in rat plasma. ET-1-LI was detected in plasma from male Wistar rats. ET-1-LI in rat plasma consisted of three components with molecular weights of 6K, 4K and 2.5K daltons by gel permeation chromatography. Two of the components were eluted at positions of big ET (4K) and synthetic ET-1 (2.5K). The remaining component was eluted at the preceding fraction (6K). No difference was observed in ET-1-LI of the small molecular form of ET (2.5K) between the two RIAs. Thus, there is little or no ET-3 in rat plasma, which has the sequence found originally in the rat genome. The concentration of the small molecular form of ET, presumably ET-1, in rat plasma was about 4 pg/ml.

We have developed monoclonal (KY-ET-1-I) and polyclonal (ET-F5) antibodies against endothelin-1 (ET-1) and established sensitive radioimmunoassays (RIAs) with different specificities. The RIA with KY-ET-1-I detected ET-1, ET-2 and ET-3, while the RIA with ET-F5 recognized ET-3 very weakly. Using these RIAs, we have investigated the concentration and molecular forms of ET-1-like immunoreactivity (-LI) in culture medium of bovine aortic endothelial cells and human plasma. Culture medium of endothelial cells contained two major components compatible with big ET and ET-1. ET-1-LI was also detected in human plasma. ET-1-LI in human plasma consisted of apparent two components, the small molecular form emerging at the position of ET-1 and the large form with the peak eluting at the preceding fraction of the elution position of big ET. The concentration of the small form of ET in human plasma was about 5 pg/ml.

The effects of intracerebroventricular (i.c.v.) administration of ANP on blood pressure and intakes of water and salt were examined, using conscious, unrestrained normotensive Wistar rats and spontaneously hypertensive rats (SHR). In normotensive rats, i.c.v. administration of alpha-rat ANP (alpha-rANP), alpha-human ANP (alpha-hANP), alpha-rANP (4-28) and alpha-rANP (5-28) at the dose of 1.5 nmol significantly attenuated water intake induced by i.c.v. injection of 0.1 nmol of angiotensin II (AII). Centrally administered alpha-hANP (5 micrograms) also attenuated AII-induced pressor response. Centrally injected alpha-hANP (1 microgram) produced a greater reduction of water intake after 24-hour water deprivation in SHR compared to control Wistar Kyoto rats (WKY). Central infusion of alpha-hANP for 1 week also reduced the salt appetite of SHR, as shown by two bottle preference test with 0.3 M NaCl solution and tap water, while it had no effect on drinking behavior of WKY. These results suggest the central antagonistic relationship of the ANP and renin-angiotensin systems and the possible involvement of brain ANP in the pathophysiology of genetically hypertensive rats.

Using a radioimmunoassay for alpha-rat atrial natriuretic polypeptide (alpha-rANP), tissue levels of alpha-rANP-like immunoreactivity (-LI) in the rat spinal cord and autonomic ganglia were investigated. The alpha-rANP-LI level was higher in the more caudal parts of the spinal cord and the highest in the sacral spinal cord. alpha-rANP-LI was also detected in the superior cervical and coeliac ganglia. Gel permeation chromatographic analysis showed that the major peak of alpha-rANP-LI in the spinal cord was a low molecular weight form co-eluted with synthetic alpha-rANP. Reverse-phase high performance liquid chromatographic analysis revealed that alpha-rANP-LI with a low molecular weight in the spinal cord consisted of several components, two major components of which co-migrated with synthetic alpha-rANP (4-28) and alpha-rANP (5-28), whereas little immunoreactivity was eluted at the position of alpha-rANP. These findings suggest the involvement of ANP in the function of the spinal cord and autonomic nervous system.

Thymidylate synthase-negative mutant mouse cells starved of thymidine or their parental FM3A cells treated with 5-fluoro-2′-deoxyuridine produced DNA fragments ranging from 50 to 200 kilobase pairs with a peak at 100 kb in length as determined by pulsed-field agarose gel electrophoresis. Accumulation of the DNA fragments following such thymidylate stress was time-dependent but their size distribution did not change in either case. Regions of the chromosomal DNA breaks seemed to be restricted to those where DNA replication was in progress as shown by pulse-labeling of the DNA synthesis. Emetine, an inhibitor of protein synthesis, blocked the accumulation of the DNA fragments when present during thymidylate stress. Cell-free extracts prepared from the thymidylate-stressed cells derived by either of the above means were capable of degrading DNA in chromatins prepared from normally growing cells in vitro. The resulting DNA fragments were similar but with a somewhat broader size distribution compared to those produced in vivo. The broader distribution of the fragments produced in the in vitro reaction became closer to the pattern obtained in vivo when ATP and 4 deoxyribonucleotides were added to the reaction.

To clarify the role of endogenous opioid peptides in the control of vasopressin (AVP) secretion, the effects of an endogenous kappa-agonist, leumorphin, derived from proenkephalin B and an opioid antagonist, naloxone, on AVP secretion were examined in conscious and freely moving rats. Intraperitoneal injection of nicotine markedly increased AVP secretion in rats. The nicotine-induced AVP secretion was significantly suppressed by intracerebroventricular (i.c.v.) pretreatment with leumorphin. Intravenous injection of naloxone significantly increased the basal AVP level and carbachol-induced AVP secretion. These results indicate that endogenous opioid peptides have an inhibitory effect on AVP secretion in rats.

The syntheses of 5-hydroxyl-4'-methoxy-7-methyl-6-azaflavanone (Ia) and 5-hydroxy-4'-methoxy-7-methyl-6-azaflavone (IIa) from 4-hydroxy-6-methyl-2 (1H)-pyridone (IVa), and the syntheses of Ib and IIb, N-methyl analogues of Ia and IIa respectively, and of IIc, N-methyl-4'-demethoxy analogue of IIa, starting with N-methyl analogue (IVb) of IVa, were described.

The nature of the refractoriness of C6 glioblastoma cells to herpes simplex virus type I (HSV-I) infection has been studied. The cells were restricted in susceptibility to HSV-I since only a small proportion of the cells could be infected by HSV-I and the virus yield per cell was low. The susceptibility to infection was increased by treating the cells with trypsin-EDTA prior to infection. The cells so treated recovered resistance to the virus when incubated at 37 degrees C, their resistance being restored to the initial level in 2 days. This restoration was inhibited by addition of cycloheximide or puromycin. Trypsin-EDTA treatment of C6 cells increased the efficiency of adsorption of HSV-I and the formation of stable cell-virus complexes from which the virus could not be dissociated by heparin.

Long life corrosion fatigue tests were carried out using web-gusset-type fillet welded joints and 3% NaCl solution. In corrosive environment, fatigue cracks initiated from the bottom of corrosion pits formed along the weld toe and led specimens to failure at such a low stress range as half of the fatigue limit in air and the Sr vs Nf relationship had a linearity with a slope of about -1/3 in the range of Nf=5×105-107 cycles on a logarithmic graphpaper. Corrosion fatigue life of fillet welded joints could be accurately estimated by assuming that corrosion pits start to grow from the facet-like initial crack and by using da/dN vs ΔK relationship including corrosion pit growth rate vs ΔK relationship.

Using radio-immunoassays for the C-terminal sequence of alpha-atrial natriuretic polypeptide [atrial natriuretic factor (ANF)-(99-126)], alpha-ANP (17-28) [ANF-(115-126)], which corresponds to gamma-human ANP [115-126; gamma-hANP-(115-126)] and for the N-terminal sequence of gamma-hANP [human ANF-(1-126)], gamma-hANP-(1-25) [human ANF-(1-25)], we detected the parallel distribution of alpha-hANP-like immunoreactivity and gamma-hANP-(1-25)-like immunoreactivity in the human and monkey brains, with the highest concentrations in the midbrain and the pons. High performance gel permeation chromatography coupled with the two radio-immunoassays and also with another radio-immunoassay using a monoclonal antibody against the N-terminal sequence of alpha-ANP revealed that gamma-ANP is synthesized in the brain and cleaved into N-terminally deleted form(s) of alpha-ANP and 10-K N-terminal fragment(s) of gamma-ANP, which coexist within the neuron. These results suggest that the post-translational processing of gamma-ANP in the brain is different from that in the heart.

To investigate the role of leumorphin, a kappa-opioid agonist derived from proenkephalin B (neoendorphin/dynorphin precursor) in the central cardiovascular control, the effects of intracerebroventricular (ICV) administration of leumorphin on basal blood pressure and angiotensin II (AII)-stimulated pressor response were examined in conscious unrestrained rats. The ICV injection of 0.06 and 0.6 nmol of leumorphin caused a significant decrease in basal blood pressure (delta mean arterial pressure (MAP): -6.5 +/- 2.7 and -7.2 +/- 1.7 mm Hg, respectively). The ICV injection of AII (0.1 nmol) elicited a pressor response (delta MAP: 21.4 +/- 1.1 mm Hg). This pressor response was significantly reduced by the simultaneous administration of leumorphin, and furthermore, the blood pressure was lowered below the basal level. These depressor actions of leumorphin were partially antagonized by the preadministration of naloxone, an opiate antagonist. These results together with our previous works on the potent inhibitory actions of leumorphin on drinking and vasopressin secretion suggest the possible involvement of leumorphin in the central regulation of blood pressure and body fluid homeostasis.

Analyse du murissement d'Ostwald des particules dispersees sur la base d'un equilibre thermodynamique entre les particules dispersees et la matrice. Systematisation des theories pour expliquer la suppression du grossissement des particules, et prevenir ainsi la deterioration de la resistance a haute temperature. Application a des aciers refractaires

To elucidate the involvement of the brain renin–angiotensin system and the brain atrial natriuretic polypeptide (ANP) system in the regulation of ANP secretion from the heart, the effects of intracerebroventricular administration of angiotensin II and ANP on the plasma ANP level were examined in conscious unrestrained rats. The intracerebroventricular administration of angiotensin II at doses of 100 ng and 1 μg significantly enhanced ANP secretion induced by volume-loading with 3-mL saline infusion (peak values of the plasma ANP level: control, 220 ± 57 pg/mL; 100 ng angiotensin II, 1110 ± 320 pg/mL, p 1-receptor antagonist of vasopressin or with the intracerebroventricular administration of phentolamine. The intracerebroventricular administration of α-rANP(4–28) (5 μg) had no significant influence on the basal plasma ANP level; however, it significantly attenuated central angiotensin II potentiating effect of volume-loading induced ANP secretion. These results indicate that the brain rennin–angiotensin system regulates ANP secretion via the stimulation of vasopressin secretion and (or) via the activation of the central α-adrenergic neural pathway, and that the brain ANP system interacts with the brain renin–angiotensin system in the central modulation of ANP secretion from the heart. The result further supports the proposed antagonistic relationship between the brain ANP system and the brain renin–angiotensin system in body fluid and blood pressure homeostasis.

The effects of naloxone, an opioid antagonist, on arginine vasopressin (AVP) secretion were examined in conscious unrestrained rats under both basal and stimulated conditions. Intravenous injection of naloxone in a dose of 0.1 mg/kg did not significantly affect the basal plasma AVP level. However, 0.5 or 2.5 mg/kg naloxone significantly raised the basal AVP level in euhydrated rats. Naloxone (0.5 mg/kg) significantly enhanced AVP secretion after 72-h water deprivation. However, the enhancement was more prominent in euhydrated rats than in dehydrated rats. Pretreatment with naloxone (0.5 mg/kg) also significantly prolonged AVP secretion induced by intracerebroventricular injection of angiotensin-II (100 ng). Moreover, naloxone (0.5 mg/kg) significantly increased AVP secretion induced by intracerebroventricular injection of carbachol (10 ng). Naloxone (0.5 mg/kg) altered neither basal blood pressure nor the angiotensin-II-induced pressor response, but augmented the carbachol-induced pressor response. This suggests that facilitation of AVP secretion by naloxone is not due to a reflex mechanism resulting from decreased blood pressure. These results indicate that endogenous opioid peptides exert a tonic inhibitory control on AVP secretion in rats.

To elucidate the secretory form of atrial natriuretic polypeptide from the atrium, the molecular form of atrial natriuretic polypeptide in the perfusate from the isolated beating rat heart and in plasma taken at the coronary sinus of 10 patients during cardiac catheterization has been investigated using high performance gel permeation chromatography and reverse phase high performance liquid chromatography coupled with radioimmunoassay for atrial natriuretic polypeptide. Atrial natriuretic polypeptide in the perfusate from the rat heart showed a single peak eluting at the position of a low molecular weight form of atrial natriuretic polypeptide, without any detectable amounts of atrial natriuretic polypeptide with high molecular weights. The major component of atrial natriuretic polypeptide in the rat heart perfusate co-migrated with rat α-atrial natriuretic polypeptide in reverse phase high performance liquid chromatography. In 9 out of 10 patients atrial natriuretic polypeptide in plasma taken at the coronary sinus revealed a single peak of atrial natriuretic polypeptide emerging at the position of human α-atrial natriuretic polypeptide in gel filtration. Only one plasma sample had a small quantity of high molecular weight forms with the predominant low molecular weight form of atrial natriuretic polypeptide. The major component of atrial natriuretic polypeptide in the plasma extract from the coronary sinus was identified with human α-atrial natriuretic polypeptide. These results indicate that α-ANP, a 28-amino acid polypeptide, is secreted as a cardiac hormone into the coronary blood stream from the atrium.

Using RIAs for the N- and C-terminal fragments of the human atrial natriuretic polypeptide (ANP) precursor gamma ANP, that is gamma ANP-(1-25), and alpha ANP [gamma ANP-(99-126)], we studied the secretion of gamma ANP-derived peptides from the heart in normal subjects and patients with heart disease, chronic renal failure, and cirrhosis. We detected gamma ANP-(1-25)-like immunoreactivity (-LI) in plasma from normal subjects (n = 17) in considerable amounts [mean, 510 +/- 62 (+/- SE) pg/mL (174 +/- 21 pmol/L)]; the mean plasma alpha ANP-LI level at the same time in these subjects was 32.8 +/- 4.4 pg/mL (10.7 +/- 1.4 pmol/L). Gel permeation chromatographic analysis of plasma samples from normal subjects and patients with heart disease and chronic renal failure revealed two major components; one was alpha ANP, and the other was the 10K N-terminal gamma ANP fragment (N-peptide) resulting from the removal of alpha ANP (3K) from gamma ANP (13K). In addition, gamma ANP (13K), which possessed both gamma ANP-(1-25)-LI and alpha ANP-LI, and beta ANP, an antiparallel dimer of alpha ANP, were detected in some patients as minor components. A significant positive correlation between plasma levels of the N-terminal gamma ANP fragment and alpha ANP (P less than 0.01) and almost equal step-ups in the coronary sinus plasma levels of the N-terminal gamma ANP fragment and alpha ANP suggest that they are cosecreted in equimolar amounts. The high molar ratio of plasma gamma ANP-(1-25)-LI to alpha ANP-LI (17.4 +/- 1.4) in normal subjects and the significantly higher ratio in patients with chronic renal failure (36.9 +/- 7.1; P less than 0.01) suggest the slower clearance of the N-terminal gamma ANP fragment than alpha ANP and a role for the kidney in its degradation. Since the molar ratio of plasma gamma ANP-(1-25)-LI to alpha ANP-LI in patients with cirrhosis (20.7 +/- 2.7) was similar to that in normal subjects, it is unlikely that the N-terminal gamma ANP fragment is metabolized by the liver. In patients with heart disease, plasma gamma ANP-(1-25)-LI and alpha ANP-LI levels were higher in those with cardiac decompensation and were positively correlated with right atrial pressure, pulmonary arterial pressure, and pulmonary capillary wedge pressure, indicating cosecretion of the N-terminal gamma ANP fragment and alpha ANP in response to atrial stretch.(ABSTRACT TRUNCATED AT 400 WORDS)

To suppress the wheel/rail roar noise effectively by use of a resilient wheel, the detailed dynamic characteristics of the wheel must be clarified. The resilient wheel is regarded as a ring with T-section supported discretely on a rigid circular cylinder, a method of analysis on its radial dynamic characteristics is proposed and the accuracy of analysis is improved by consideration of shearing deformation and rotatory inertia.

Using Northern blot analysis and radioimmunoassay, we measured atrial natriuretic polypeptide (ANP) messenger RNA and ANP levels in the ventricle in patients with old myocardial infarction and investigated the relationship between ventricular ANP synthesis and hemodynamic parameters. Levels of ANP messenger RNA and ANP were markedly increased in the left ventricular aneurysm, and a positive correlation was observed between the ANP level in the left ventricular aneurysm and pulmonary capillary wedge pressure (r = 0.85, p less than 0.05). These findings indicate that ANP synthesis in the ventricle is increased in accordance with the severity of congestive heart failure.