[Report of a case of fused pelvic cake kidney].
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Biomedical subjects
Publications and source records attributed to K Kameda.
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We have investigated serum lipids, lipoproteins and apolipoprotein levels in 97 survivors of acute myocardial infarction in order to clarify the characteristics of lipid and lipoprotein disorders in coronary artery disease among Japanese. Although the HDL cholesterol level was lower and the atherogenic index was higher in the myocardial infarction (MI) group than in the control group, in agreement with previous papers, there was no significant difference in total serum cholesterol level between the MI and the control groups. On the other hand, the triglyceride level was significantly higher in the MI group. The MI group also had higher levels of both cholesterol and triglyceride in the VLDL fraction (d less than 1.006) with a statistically significant increase in the ratio of cholesterol to triglyceride compared with the control group. Levels of both lipids in the IDL fraction (1.006 less than d less than 1.019) were higher in the MI group than in the control group. Lipoprotein analysis with PAG disc electrophoresis revealed a more frequent occurrence of a "midband", which appeared as an additional band between the LDL and VLDL positions, in the MI group than in the control group (MI:51% versus control: 25%, P less than 0.02). The midband-positive subgroup had a higher ratio of cholesterol to triglyceride in the VLDL fraction and higher levels of both lipids in the IDL fraction. Isoelectric focusing of apo VLDL revealed that the incidence of the suspected apo E-ND (E3/2 or E4/2) constitution, which was determined by a low ratio (less than 1.1) of the peak area of E3 to that of E2, was three times higher in the MI group than in the control group (MI:25% versus control: 7%).(ABSTRACT TRUNCATED AT 250 WORDS)
A patient with mucopolysaccharidosis type IIA (MPS IIA) and progressive gait disturbance is described. The histopathology of biopsied muscle was studied; Dorling's method revealed muscle fibers and interstitial cells containing metachromatic granules which suggested the storage of sulfated acidic glycosaminoglycans. Electron microscopy demonstrated that the membrane-bound vacuoles were present in muscle fibers, subsarcolemmal area, vascular endothelial cells, satellite cells, and endomysial fibroblasts. Besides clinical features, this ultrastructural pathology in MPS IIA muscles of MPS IIA was more severe than MPS IIB muscles. The accumulation of glycosaminoglycans in muscle tissue may be an additional factor contributing to gradual motor impairment of patients with MPS IIA.
We report the histopathologic findings of 3 sural nerve biopsies and 1 muscle biopsy from 3 patients with Rett syndrome. The 3 sural nerve biopsies demonstrated a few ultrastructural abnormalities, including the presence of many Pi-granules and mitochondrial changes in the cytoplasm of Schwann cells, occasional bands of Büngner and onion-bulb formations, and mitochondrial alterations in myelinated axons. Morphometric analysis disclosed reduction in the number of large myelinated fibers with normal densities in comparison to those of an age-matched normal control. Light microscopic examination of the biopsied muscle from a 6-year-old patient with Rett syndrome revealed the existence of many small, dark, angulated fibers with NADH-TR staining. Ultrastructural investigation of the muscle confirmed the presence of the dumbbell-shaped mitochondria. Peripheral nerve involvement and the possibility of mitochondrial abnormalities in Rett syndrome were suggested by the results.
Using the latencies of M and F responses, we assessed motor nerve conduction velocity along the entire course of the median and ulnar nerves from the spinal cord to the muscle in 14 patients with the less severe forms of Werdnig-Hoffmann disease. In these forms, the motor nerve conduction velocities were decreased significantly over both proximal (cord-to-elbow) and distal (elbow-to-wrist) segments in both the nerves as compared with normal values; however, the mean motor nerve conduction velocities in the proximal segments were faster than those in the distal segments by about the same amount as the normal controls. These findings indicate that motor conduction abnormalities in Werdnig-Hoffmann disease are diffuse over the entire course of the nerve and appear to eliminate a dying-back process in which the affected axons are severely damaged, beginning with the more distal sites.
Five siblings with hereditary spastic paraplegia of autosomal-dominant inheritance were studied with somatosensory evoked potentials. Somatosensory evoked potentials were recorded from Cz', T12, and the left popliteal fossa following left posterior tibial nerve stimulation. The latency and amplitude of the corresponding potentials (i.e., P37, N20, and N7) were compared with normal values obtained from age- and height-matched controls. There was no significant difference in the values of N7, suggesting an intact afferent peripheral pathway; in contrast, the amplitudes of P37 and N20 were decreased with normal latencies. The degree of amplitude decrease correlated with the severity of vibration sense impairment in the lower limbs. These results appear to support selective axonopathy of the centrally directed axons of the dorsal root ganglion cells. Furthermore, our results suggest that different degrees of dorsal column involvement in hereditary spastic paraplegia can cause different types of somatosensory evoked potential abnormalities, namely, attenuated amplitudes with no latency shifts, as recorded in this family, and the prolonged latencies, as reported previously.
The leaves of the persimmon Diospyros kaki, have been traditionally used for treatment of hypertensive diseases in Japan. We have studied the inhibitory effects of four flavonoids isolated from the leaves of the persimmon on angiotensin-converting enzyme activity. The four flavonoids astragalin [1], kaempferol-3-O-(2"-O-galloyl)-glucoside [2], isoquercitrin [3], and quercetin-3-O-(2"-O-galloyl)-glucoside [4] inhibited the angiotensin-converting enzyme activity in a dose-dependent fashion. Compounds 1-4 produced 67%, 53%, 33%, and 48% inhibition at a concentration of 300 micrograms/ml, respectively. The 50% inhibitory concentrations (IC50) of 1 and 2 for the angiotensin-converting enzyme were 180 micrograms/ml and 280 micrograms/ml, respectively. On the other hand, 2 and 4 were shown to have tannin activities, but 1 and 3 had no tannin activities. These results suggest that there is no relationship between the inhibition for angiotensin converting enzyme activity and the tannin activity for the four flavonoids.
Effects of citalopram on dopamine D2 receptor expression in the rat brain striatum were studied. Repeated administration of citalopram increased the amount of dopamine D2 receptors, the level of dopamine D2 receptor mRNA, and the transcription rate of the dopamine D2 receptor gene. Single administration of citalopram also increased the level of dopamine D2 receptor mRNA with a maximum effect in 2-4 h after the treatment, and the transcription rate of the dopamine D2 receptor gene. The administration of 5-hydroxytryptophan (5-HTP) also increased the level of dopamine D2 receptor mRNA. These results suggest that the increase in the dopamine D2 receptor expression induced by citalopram may be owing, at least partially, to the stimulation of the dopamine D2 receptor gene transcription, and that serotonin (5-HT) may mediate the effects of citalopram in the induction of dopamine D2 receptor expression.
BACKGROUND AND PURPOSE: Retrograde cortical venous drainage (RCVD) is the most major risk factor for aggressive behavior of intracranial dural arteriovenous fistulas (DAVF). The purpose of this study was to assess the efficacy of relative cerebral blood volume (rCBV) map for RCVD in patients with DAVF. METHODS: Ten patients with angiographically proven DAVF with RCVD, 2 reference patients with DAVF without RCVD, and 10 control subjects underwent examinations with dynamic susceptibility contrast (DSC)-MR imaging. Four patients with DAVF with unilateral RCVD were evaluated, before and after treatment. The calculation of mean rCBV ratio was performed on a hemispheric basis. The mean rCBV ratio was defined as the value on one side (higher value side) divided by that on the other side (lower value side). RESULTS: In all patients with DAVF with RCVD, the rCBV map showed an increase in rCBV of the angiographically proved affected hemisphere. In 2 reference patients with DAVF without RCVD and all control subjects, the rCBV map showed no increase of rCBV. The mean rCBV ratio in patients with DAVF with RCVD was significantly higher than that of control subjects (P = .0002). Treatment response for RCVD was indicated by a decrease of CBV on the rCBV map and by a decrease of 22% in the mean rCBV ratio. CONCLUSIONS: Increased rCBV by DSC-MR correlated with RCVD in patients with DVAF. The assessment with rCBV for RCVD may be more quantitative than that with angiogram.
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BACKGROUND/AIMS: We present our new laparoscopic technique for treating incarcerated stones in either the cystic duct or the neck of the gallbladder. PATIENTS AND METHODS: One hundred sixteen patients who underwent laparoscopic cholecystectomy were studied. RESULTS: In all, 18 patients (16%) were found to have incarcerated stones in gallbladder or cystic duct. Eleven patients were drip infusion cholangiography (DIC) negative. The length of the operative time for these patients was significantly longer than that in non-incarcerated stone cases. We present our new laparoscopic technique for treating incarcerated stones in either the cystic duct or neck of the gallbladder and this procedure includes a needle aspiration of the bile juice, the transsection and removal of the incarcerated stone and an extracorporeal ligation. CONCLUSIONS: This above procedure was successfully performed on a series of 4 patients. Our procedure is therefore considered to allow us to complete a laparoscopic cholecystectomy even in extremely hypertrophic cases with incarcerated stones either in the cystic duct or Hartmann's pouch.