[Case of pure progressive autonomic failure with sleep apnea syndrome].
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Autonomic Nervous System Diseases↗
Biomedical subjects
S Nagasawa
Publications and source records attributed to S Nagasawa.
Polymorphism and proteolytic fragments of granulocyte membrane cofactor protein (MCP, CD46) of complement.
Human granulocytes (polymorphonuclear leucocytes, PMN) possess a membrane cofactor protein (MCP, CD46), which is structurally and functionally distinct from the MCPs of other cell types: it shows a single broad band of 56-80 kDa (without the doublet pattern characteristic of MCP) on SDS/PAGE and has less affinity for complement component C3b. We purified PMN MCP using monoclonal antibodies in order to study the molecular differences between it and other MCPs. Several forms of PMN MCP with size heterogeneity were noted on SDS/PAGE and by immunoblotting. O-Glycanase treatment decreased this heterogeneity, yielding a fast-migrating component identical in position on SDS/PAGE to the O-glycanase-treated MCP of other cells. The cell-specific variation of MCP, therefore, arises from post-translational glycosylation and not from a difference in primary structure. The Factor I cofactor activity of PMN MCP was more efficient in cleaving the methylamine-treated complement components C4/C3 than was MCP from other cells, which shared a similar potency of cofactor activity on a weight basis. Two types of small-form PMN MCP were identified during purification. These were 42 kDa and 30 kDa in size; the former was recognized by M177 (a monoclonal antibody against the active site marker), possessed N-linked sugars [located on the short consensus repeats (SCRs)] but not O-linked ones (on the Ser/Thr-rich region), and retained cofactor activity for C3b/C4b cleavage, similar in potency to that of other MCPs. The functionally active soluble form of MCP was observed specifically in PMN. Protease inhibitors did not inhibit liberation of the fragments, although the generated fragments became susceptible to serine proteases. The findings show that the SCRs are the functional domain of MCP and that the MCP proteolysis found only in PMN may modulate the properties of PMN MCP. In conclusion, the structural features of PMN MCP largely reflect a variability in the O-linked sugars, and the decreased affinity for C3b may be in part attributable to proteolysis.
Antigens, CD↗
A novel sensor-regulator protein that belongs to the homologous family of signal-transduction proteins involved in adaptive responses in Escherichia coli.
Expression of the Escherichia coli outer membrane porins, OmpC and OmpF, is regulated in response to changes in the medium osmolarity through the functions of the regulatory factors, EnvZ and OmpR. A 3.0 kilobase pair DNA fragment cloned from E. coli is able phenotypically to suppress the defect in ompC and ompF expression caused by an envZ deletion mutation, provided that a certain gene located in this fragment is expressed on a high copy-number plasmid. Nucleotide sequencing revealed that the putative gene encodes a protein of 102,452 Da. The deduced amino acid sequence of the protein shows a high degree of homology to those of both EnvZ and OmpR, i.e. it contains both a 'sensory kinase domain' and a 'response regulator domain' in its primary amino acid sequence. The protein identified in this study is probably a novel member of the homologous family of proteins involved in bacterial adaptive responses. Hence, the gene encoding this novel sensor-regulator protein was designated as barA (bacterial adaptive responses) and mapped at 60 min on the E. coli genetic map. The BarA protein in isolated membranes was demonstrated in vitro to undergo phosphorylation in the presence of ATP.
Adaptation, Biological↗
Selective cooling of brain using profound hemodilution in dogs.
A new method of selective cooling of the brain was studied under profound hemodilution in 17 dogs. The carotid and vertebral arteries were bilaterally exposed, and the right vertebral artery was destroyed to provide an infusion route for cold solution for brain cooling. After the other three cerebral arteries were clamped simultaneously in the neck under low-dose heparinization, cold Ringer's lactate solution was immediately perfused into the right vertebral artery. Brain temperatures fell gradually in two dogs, and the experiments were terminated. In 10 dogs, the brain temperature fell to 28 degrees C within 4.4 +/- 1.5 minutes and was maintained at 27.0 +/- 1.0 degrees C for 60 minutes. During this interval, the body temperature was 33.9 +/- 1.6 degrees C, the stump pressure of the vertebral artery was 58 +/- 15 mm Hg, and the hematocrit value of cerebral venous blood was 7.2 +/- 4.2%. Inspection of the brain during infusion revealed paleness of the cortical vessels and no evidence of swelling. All animals survived in good condition until the time of death at 10 weeks. Histological examination of the brain revealed no evidence of ischemic injury. In a control study of five dogs, Ringer's solution at 38 degrees C was infused in the same manner as the cold solution. None of these dogs recovered from anesthesia. It is concluded that selective cooling of the brain under profound hemodilution has a protective effect on cerebral ischemia and provides a relatively bloodless operative field.
Animals↗
Plasma high density lipoprotein in anemic cattle infected with Theileria sergenti.
Changes in plasma concentration of lipid composition were analysed in cattle with anemia due to Theileria sergenti infection. Plasma levels of phospholipids, cholesterol, free cholesterol, high density lipoprotein cholesterol, and vitamin E decreased to 40-67% of the pre-infection levels, corresponding to the decrease of PCV due to the infection. However, no definite changes were detected in plasma level of triglyceride. By gradient centrifugation, it was confirmed that lipid components, other than triglyceride, occur in high density lipoprotein (HDL) and these decreases lowered the HDL value. There was no correlation between this phenomenon and liver function. As similar changes in lipid composition were also observed in phlebotomized calves, it was considered that this phenomenon might partially depend on the acceleration of erythropoiesis as a reaction to anemia caused by T. sergenti infection.
Anemia↗
[Basilar artery occlusion therapy for giant aneurysm: hemodynamic analysis by hydraulic vascular model].
Therapeutic occlusion of the basilar artery has been one of the alternative treatments for surgically or intravascularly inaccessible basilar bifurcation giant aneurysms. However, several problems have been reported, such as incomplete thrombosis of the aneurysms, their growth or rupture, and cerebral embolism originating from their cavities. Since hemodynamic changes after occlusion therapy are suspected to be responsible for these phenomena, they were investigated by a hydraulic vascular model. A hydraulic vascular model of the vertebrobasilar artery was constructed with silicone and glass tubes. A glass-made sphere of 2.5 cm in diameter was attached to the model and was regarded as a basilar head aneurysm. A 40% glycerol solution at 25 degrees C was found to be of similar viscosity and specific gravity to those of human whole blood at 37 degrees C and was perfused in the model. A device to measure intra-aneurysmal clearance was made from a stable luminous source and a Cds photocell. Good correlation was found between the output and an intra-aneurysmal dye concentration. The dye was injected into the aneurysm and its half-life was calculated from clearance curves. It was then regarded as an index of stagnation in an aneurysmal cavity. The flow volumes were estimated as: 60ml/min to the territory of one posterior cerebral artery (PCA) and 80ml/min to the cerebellum and the brain stem. Half-life was recorded in the following conditions: 120ml/min of flow in the basilar artery (BA) into bilateral PCAs stimulating the condition before BA occlusion, and various flow values (60ml/min to 10ml/min) of P1 segment simulating the conditions after BA occlusion distal to the superior cerebellar artery.(ABSTRACT TRUNCATED AT 250 WORDS)
Basilar Artery↗
[Bilateral distal anterior cerebral artery aneurysms associated with polycystic kidney and liver disease; a case report].
A patient who had bilateral distal anterior cerebral artery aneurysms and a right middle cerebral artery aneurysm in association with polycystic kidney and liver disease is reported. A 57-year-old woman was referred to our center with headache and disturbance of consciousness. On admission, her level of consciousness as evaluated by the Japan Coma Scale was 10. CT revealed subarachnoid hemorrhage, especially in the interhemispheric fissures. Right carotid angiography demonstrated bilateral distal anterior cerebral artery aneurysms and a right middle cerebral artery aneurysm. All three aneurysms were clipped in a one-stage procedure. The patient was discharged without any neurological deficits two weeks after the operation. Bilateral distal anterior cerebral artery aneurysms are extremely rare. This is the first report of such aneurysms and a right middle cerebral artery aneurysm in association with polycystic kidney and liver disease. The etiology of these aneurysms is discussed.
Cerebral Angiography↗
[Determination of approach angle to intra- and peri-ventricular lesions; ultrasonography with reference to MRI].
Ultrasonography using a 5-MHz sector-scanning probe was performed in 4 patients with intraventricular or periventricular lesions. All patients underwent craniotomy, and the lesions included an intraventricular cyst (Case 1, Fig. 1), a subependymoma (Case 2, Figs. 2 and 3), a hypothalamic glioma (Case 3, Fig. 4) and a periventricular cavernoma (Case 4, Fig. 5). The lesion was approached in the former 3 cases via the transcallosal route and in the last via the transcortical route. Preoperative MRI was performed in such a way that both the lesion and the anticipated operative route to it were shown on the image, so that the approach angle and surrounding structures could be assessed. During craniotomy, intraoperative ultrasonographic examination was performed while the dura was still closed. By frequently changing the probe direction, we were able to obtain an image similar to that shown on the preoperative MRI, and we then initiated intradural operative procedures at an angle parallel to the axis of the probe. Since MRI provides detailed anatomical information about the approach to the lesion and the tissue around it, it has become an indispensable tool in the planning of neurosurgical operations. Although ultrasonography generally provides poorer resolution than does MRI, especially for deep structures, it does provide convenient, real-time images of any slice. Because the interhemispheric fissure, lateral ventricle, and choroid plexus have distinctive shapes and echogenicity and, hence, can be readily recognized, we found that they can be good landmarks in the imaging of periventricular or intraventricular lesions.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Correlation of serum concentration of alpha 1-acid glycoprotein with lymphocyte blastogenesis and development of experimentally induced or naturally acquired hepatic abscesses in cattle.
Changes in serum alpha 1-acid glycoprotein (alpha 1AG) concentration in cattle with hepatic abscesses were observed, and function of alpha 1AG was evaluated, particularly its influence on cellular immune response. Test cattle (n = 4) were inoculated with Fusobacterium necrophorum, control cattle (n = 2) were inoculated with inactivated bacteria, and naturally affected cattle (n = 11) were found in a slaughterhouse. Determination of alpha 1AG was made by use of a single radial immunodiffusion method. The action on lymphocyte blastogenesis was determined by [3H]thymidine incorporation. Cultured lymphocytes from healthy cattle were treated with variable concentrations of alpha 1AG purified from serum obtained from cattle with hepatic abscesses and suppression of blastogenesis stimulated by each of 3 mitogens was measured. In cattle with experimentally induced abscesses, serum alpha 1AG concentration increased for 7 to 10 days after F necrophorum inoculation, its change being parallel to that of sialic acid. High concentration of alpha 1AG was found in naturally affected cattle and was highly correlated to sialic acid concentration. Suppression of lymphocyte blastogenesis in cattle with experimentally induced hepatic abscesses was highly correlated to serum alpha 1-AG concentration.
Animals↗
[Assessment of left ventricular function with ESS-ESVI relationship in patients undergoing aortic valve replacement for aortic regurgitation].
Aortic valve replacement (AVR) for aortic regurgitation (AR) results in the reduction of left ventricular dimensions. But postoperative death or congestive heart failure may occur in patients with left ventricular dysfunction. Pre- and postoperative stress (ESS)-volume (ESVI) relationship by M-mode echocardiography was examined in 30 patients undergoing AVR. Postoperatively, 23 patients (Group A) achieved a normal left ventricular dimension (LVDd less than 55 mm, LVDs less than 45 mm) and 7 patients (Group B) had persistent left ventricular dilation (LVDd greater than or equal to 55 mm, LVDs greater than or equal to 45 mm). Correlation between preoperative ESS and ESVI was significant (r = 0.92, p less than 0.001), and the ESS/ESVI was greater in Group A of 1.62 +/- 0.29 kdy/cm2/ml/m2 than in Group B of 1.18 +/- 0.19 kdy/cm2/ml/m2 (p less than 0.001). Three patients with ESVI greater than 180 ml/m2 and ESS/ESVI less than 1.2 kdyn/cm2/ml/m2 died after operation. Echocardiographic variables correlated with ESS/ESVI were ESVI (r = -0.57), FS (r = -0.53) and CSA (r = -0.47). The changes in LVDs (delta Ds) after AVR correlated with ESS/ESVI in 12 patients with severe left ventricular dilatation. Postoperative left ventricular function could be predicted by ESS-ESVI relationship by preoperative DBcAMP infusion test. ESS/ESVI is sensitive to changes in the left ventricular contractility. The patients with ESVI greater than 180 ml/m2 and ESS/ESVI less than 1.2 kdyn/cm2/ml/m2 may result in poor prognostic outcome.
Adolescent↗
Urinary excretion of terminal complement complexes in glomerular disease.
To evaluate renal terminal complement activation in patients with glomerular diseases, we measured terminal complement complexes (TCCs) in plasma and urine with sandwich enzyme-linked immunosorbent assay (ELISA) using a monoclonal antibody against a C9 neoepitope expressed on TCC and a polyclonal antihuman C7 antibody. TCCs were detectable in plasma but not in urine in most of normal controls. In plasma, TCC levels were elevated in 4 of 22 patients with lupus nephritis and in 6 of 12 with membranoproliferative glomerulonephritis. However all patients with IgA nephritis, focal glomerulosclerosis, idiopathic membranous nephritis and idiopathic minimal change nephrotic syndrome (MC) showed normal values. In urine, TCCs were detectable in almost all patients with heavy proteinuria (greater than or equal to 100 mg/ml) except MC. The TCCs present in urine were partially purified by gel filtration using Sepharose 6B and were found to contain C5, C6, C7, C8, C9 and S protein by ELISA. Although the molecular weight of TCC is similar to that of IgM, the fractional excretion rate of TCC was about 100 times higher than that of IgM. These results suggest that TCCs detectable in urine contain SC5b-9 complexes and are mostly of renal origin.
Adolescent↗
Regulation of pancreastatin release from a human pancreatic carcinoid cell line in vitro.
The objective of these experiments was to investigate the influence of activation of three second messenger systems (protein kinase-C, adenylate cyclase-cAMP, and calcium mobilization) on the secretion of pancreastatin (PST) and chromogranin-A (CGA) by a human pancreatic carcinoid cell line (BON) in tissue culture. Stimulation of protein kinase-C by a phorbol ester (0.025-7.5 microM) caused a significant dose-related release of PST (186 +/- 22-4271 +/- 228% over controls). Treatment of BON cells with graded doses of 8-bromo-cAMP (0.14-3.0 mM) and isobutylmethylxanthine (IBMX; 0.01-1.0 mM) also stimulated a dose-related release of PST (107 +/- 22-284 +/- 28 and 16 +/- 12-1076 +/- 100% over controls, respectively). Incubation of BON cells with ionomycin (0.134-13.4 microM) increased the release of PST (102 +/- 15-554 +/- 21% over controls) in a dose-related manner. A combination of IBMX and ionomycin resulted in an additive effect, whereas treatment with a phorbol ester plus IBMX resulted in a synergistic effect on PST release. Pretreatment of BON cells with monensin, an agent that prevents processing of precursors to smaller peptides, significantly decreased PST, but not CGA, secretion in response to phorbol ester or ionomycin. These findings indicate that protein kinase-C, cAMP, and Ca2+ mobilization participate in CGA and PST secretion. Although the observation that secretions of PST and CGA in response to theophylline are quantitatively associated, the absence of a quantitative relationship in the release patterns of PST and CGA in response to phorbol ester and ionomycin do not support a simple precursor-product relationship between CGA and PST. The monensin experiments are consistent with the notion that PST is derived from CGA in BON cells.
1-Methyl-3-isobutylxanthine↗
A novel HPLC method for glutathione-insulin transhydrogenase assay using fluorescence labeled insulin.
The activity of rat liver glutathione-insulin transhydrogenase (GIT) was measured by HPLC. The degradation of fluorescein isothiocyanate-I (FITC-I)-labeled insulin is separated into several peaks, which are bound different amount of FITC-I. We selected mono-fluorescein-thiocarbamylated insulin to estimate the decrease of insulin content and it became possible to assay GIT activity. This novel method was time-saving and simple, and this system could utilize instead of previous method.
Animals↗
Hemodynamic study of posterior circulation using hydraulic vascular model--critical stenosis of the vertebrobasilar artery and tolerance to occlusion therapy.
A hydraulic vascular model with glass and silicone tubes of the intracranial portion of the vertebro-basilar artery was used to determine the critical stenosis causing vertebrobasilar insufficiency, and the minimum diameter of the posterior communicating arteries (PComAs) necessary to tolerate therapeutic vertebrobasilar occlusion for unclippable aneurysms. The critical stenosis of one vertebral artery (VA) or basilar artery (BA) differed greatly depending upon the anatomical variations of the PComA and the posterior cerebral artery (PCA): 1.14 mm diameter when the artery supplies 80 ml/min to the cerebellum and brainstem only, 1.33 mm when 140 ml/min to these structures and one PCA, and 1.56 mm when 200 ml/min to these structures and both PCAs. The minimum PComA diameter to tolerate therapeutic occlusion depended largely upon the occlusion site: one PComA with 1.54 mm diameter for bilateral VA occlusion and 1.25 mm for BA occlusion distal to the branching of the superior cerebellar arteries. The total volume of collateral flow through both PComAs can be estimated by summing the squares of the diameters. These values cannot be applied rigidly to clinical cases, but are useful standards to evaluate the stenotic lesion or tolerance to occlusion.
Blood Flow Velocity↗
Intracranial and spinal germinomas occurring four years after spinal cord germinoma--case report.
Intracranial and thoracic tumors developed in a 31-year-old male 4 years after irradiation therapy for a cervical tumor. He received irradiation for the pineal, suprasellar, and thoracic tumors. All tumors disappeared by neuroradiological imaging after treatment. The previous and present tumors were probably germinomas, possibly with multifocal origins.
Adult↗
[A case of C2 neurinoma suffering syncopal episodes].
A 32-year-old male had a 4-year history of episodes of loss of consciousness. These episodes occurred several seconds after he would turn his head 60 degrees to the left, and had increased in frequency at the time previous to the operation he underwent. MRI revealed a round mass on the right side between the atlas and the axis. Cerebral angiography demonstrated that the right vertebral artery (VA) was occluded at the transverse foramen of the axis with the head rotated toward the left. The left VA was hypoplastic and no posterior communicating artery was visualized. A lateral surgical approach was performed between the left sternocleidomastoid muscle and the internal jugular vein. A yellowish and elastic but hard tumor was found to compress the VA. Postoperative MRI and vertebral angiography revealed neither residual tumor nor stenosis. Although the VA is frequently compressed by cervical tumors, only a few cases have been reported with tumors whose initial symptom was vertebro-basilar insufficiency. On the other hand, symptomatic stenotic change in the VA due to head movement can occur at the atlanto-axial joint and is named "bow hunter's stroke". In this case, flow of the VA was measured with an ultrasonic flowmeter after the tumor was removed. As the flow proved to change little during head rotation, additional decompression procedures such as removal of the transverse processes were judged unnecessary. Intraoperative arterial flow measurement has been found to be quite useful for evaluating the actual effect of surgical procedures.
Adult↗
Effect of sodium chloride concentration on fluid-phase assembly and stability of the C3 convertase of the classical pathway of the complement system.
The assembly of the classical-pathway C3 convertase from C4 and I2-treated C2 by the action of C1s is an Mg2(+)-dependent reaction. The Mg2+ concentration necessary for the assembly of C3 convertase in the fluid phase was found to be dependent on NaCl concentration. In the absence of NaCl more than 5 mM-MgCl2 was found to be required, whereas 0.5 mM-MgCl2 was adequate for the assembly of C3 convertase in the presence of 150 mM-NaCl. The C3 convertase assembled in a low-ionic-strength buffer was extremely labile compared with that assembled in buffer of physiological ionic strength, and the stability of C3 convertase was improved with the increase in NaCl concentration. It was found that the stabilizing effect of NaCl on C3 convertase was due to inhibition of the dissociating activity of C2b, which was formed during the assembly of C3 convertase. In addition to the dissociation-accelerating effect, C2b inhibited the assembly of C3 convertase in low-ionic-strength buffer, and this effect also was diminished with increase in NaCl concentration. An increase in NaCl concentration to more than 200 mM resulted in a decrease in the assembly of C3 convertase. This effect was not due to the lability of the assembled C3 convertase but due rather to the inhibition of C2 cleavage by C1s. Purified C3 convertase itself is stable in dilute medium or high-ionic-strength medium such as 500 mM-NaCl, suggesting that the interactions between C4b and C2a are hydrophobic. In these respects C2b seemed to be functionally similar to C4bp, but C2b failed to act as a cofactor for the Factor I-catalysed C4b cleavage.
Complement C2↗
Regulatory system of guinea-pig complement C3b: two factor I-cofactor proteins on guinea-pig peritoneal granulocytes.
Complement factor I is a plasma protease serving for proteolytic inactivation of C3b together with its cofactor. We have identified two factor I-cofactor activities in solubilized extracts of guinea-pig peritoneal granulocytes using guinea-pig factor I (Igp) and fluorescent-labeled methylamine-treated guinea-pig C3 (f-C3(MA)gp). One of these eluted from a chromatofocusing column between pH 7.6-7.1, and the other at about pH 5.7. These two cofactor fractions both interacted with Igp and, to a lesser degree, with human factor I (Ihu) on C3(MA)gp cleaving it into an inactive C3bi analogue, but did not cleave methylamine-treated human C3 (C3(MA)hu) together with Igp or Ihu. These factors are therefore species specific. The neutral and acidic fractions with cofactor activity contained C3(MA)gp-binding proteins with a doublet of 55 kDa and 42 kDa, and a singlet of 160 kDa, respectively, on SDS-PAGE. These proteins may be membrane cofactor protein (MCP) and C3b/C4b receptor (CR1) of guinea-pigs.
Animals↗