Lipoprotein glomerulopathy with a new apolipoprotein E phenotype.
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Biomedical subjects
Publications and source records attributed to H Ochi.
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The patients with a hematopoietic neoplasm can develop myelodysplastic syndrome following chemotherapy. We report herein a case of non-Hodgkin B-cell lymphoma and refractory anemia with ringed sideroblasts. Absence of the preceding chemotherapy establishes the true coincidence of the two disorders. We review 25 similar cases reported in the last 11 years and discuss the possible etiology of coexistence.
The aim of the present study was to simulate the pathological uteroplacental circulation observed in complicated human pregnancies in the pregnant ewe and to analyze its velocimetric changes. Four pregnant ewes at 16-17 weeks of pregnancy were used in the study. Micro-beads (Gelfoam) were administered stepwise into the uterine artery and changes in the uterine circulation were assessed by Doppler velocimetry. Gelfoam administration successfully embolized the uterine spiral arteries located in the decidual segment. The Gelfoam embolization decreased the uterine blood flow dose-dependently from 550 +/- 48 ml/min (mean +/- SD) to 142 +/- 12 ml/min and reciprocally increased the uterine vascular resistance from 139 +/- 12 mmHg min l-1 to 540 +/- 46 mmHg min l-1 at 0 mg and 30 mg Gelfoam, respectively. It dose-dependently attenuated the pregnancy-related physiological elevation in diastolic flow velocity, while the systolic flow velocity was unaffected, resulting ina dose-dependent increase in the pulsatility index from 0.5 +/- 0.2 to 3.2 +/- 0.7 at 0 mg and 30 mg Gelfoam, respectively. The pulsatility index linearly correlated with the uterine vascular resistance, giving a high correlation coefficient of r = 0.947. It could be concluded that the uterine arterial pulsatility index is an indicator of uterine vascular resistance.
Lysophosphatidylcholine (lyso-PC), a polar phospholipid product increased in atherogenic lipoproteins and atherosclerotic lesions, has been shown to differentially induce functional intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 and mRNA for platelet-derived growth factor (PDGF)-A and -B chains and heparin-binding epidermal growth factor-like growth factor in various cultured endothelial cells. In this study, we have demonstrated increased expression of cell- and matrix-associated forms of PDGF-B chain (PDGF-B) protein elicited by lyso-PC and further characterized potential signal transduction mechanisms responsible for lyso-PC-induced gene expression, focusing on PDGF-B and ICAM-1 genes in cultured human umbilical vein endothelial cell models. Cycloheximide almost completely inhibited PDGF-B but not ICAM-1 mRNA induction elicited by lyso-PC, suggesting that dependence on de novo protein synthesis for PDGF-B is different from that for ICAM-1. Prolonged exposure to phorbol myristate acetate (PMA), which depletes protein kinase C (PKC), or staurosporine, a PKC inhibitor, did not block lyso-PC-induced increases in PDGF-B or ICAM-1 mRNA. Forskolin and dibutyryl cAMP, which elevate intracellular cAMP levels, blocked both PDGF-B and ICAM-1 upregulation elicited by lyso-PC; however, these cAMP-elevating agents did not suppress ICAM-1 upregulation by PMA. Taken together, PDGF-B and ICAM-1 gene induction by lyso-PC may involve different signaling mechanisms; however, both appear to be independent of PMA-regulatable PKC activation but are suppressed by increased levels of intracellular cAMP.
The analgesic mechanism of Y-23023, a new non-steroidal anti-inflammatory drug, was investigated in the writhing response induced by intraperitoneal injection of kaolin and captopril in mice. Y-23023 (0.1-1 mg/kg, p.o.) suppressed the writhing frequency in a dose-dependent manner. Y-23023 also significantly reduced the increased levels of prostaglandin (PG) and bradykinin (BK) in the peritoneal cavity. In contrast, indomethacin, diclofenac sodium, loxoprofen sodium and mefenamic acid inhibited the writhing response, but their efficacies were lower than that of Y-23023. The peritoneal PG levels were dose-dependently reduced to the same extent as Y-23023, whereas the BK levels were not. M1, an active metabolite of Y-23023, inhibited the cyclooxygenase from sheep vesicular gland in a concentration-dependent manner, and its potency was similar to that of indomethacin. These results suggest that in addition to the suppressive effect on PG production via inhibition of cyclooxygenase, the inhibitory effect on BK production is involved in the analgesic action of Y-23023, unlike indomethacin and diclofenac sodium.
The analgesic and anti-inflammatory activities of Y-23023, a new nonsteroidal analgesic and anti-inflammatory compound, were investigated in acute, subacute and chronic pain models in rats. In the carrageenin paw edema test, Y-23023 (0.3-3 mg/kg, p.o.) dose-dependently inhibited hyperalgesia assessed by the Randall-Selitto method and paw edema. Y-23023 (1 mg/kg, p.o.), when administered at 2 hr after carrageenin injection, significantly elevated the reduced pain threshold without affecting paw edema. Therefore, the antinociception activity induced by Y-23023 was not the result of its anti-inflammatory activity. In addition, Y-23023-induced antinociception was resistant to post-treatment with naloxone (2 mg/kg, s.c.), but was antagonized by intraplantar injection of prostaglandin E2 (1 microgram/paw). These results suggest that Y-23023 produces a peripheral analgesic effect mediated by inhibition of prostaglandin production. Y-23023 (0.3-10 mg/kg, p.o.) also had a potent inhibitory effect on the silver nitrate-induced arthritic pain. In suppressing acute and subacute pain, Y-23023 was more potent than diclofenac sodium, indomethacin and loxoprofen sodium. The analgesic and anti-inflammatory activities of Y-23023 (0.1-1 mg/kg/day, p.o.) on the adjuvant-induced hyperalgesia and the paw swelling were nearly equipotent to diclofenac sodium and indomethacin, but were more potent than loxoprofen sodium. Therefore, Y-23023 would be regarded to show predominantly strong analgesic activity in acute and subacute pain when compared with reference drugs. The above results suggest that Y-23023 is a novel analgesic compound with an anti-inflammatory activity in the clinical field.
We have evaluated bone scans, parathyroid hormone (PTH) levels and bone mineral densities (BMDs) of hemodialysis (HD) patients. The bone scans were grouped into 4 types (type 1-4). Type 1 showed a diffusely high activity of the tracer in the whole skeleton especially in the skull: secondary hyperparathyroidism pattern. Type 2 showed high background activity, the same pattern as osteomalacia. Type 3 was a mixture of types 1 and 2. Type 4 was almost normal. The PTH levels of type 1 were higher and the radius BMDs were lower than those of the other types. The radius BMDs in one third of type-2 patients were lower than the normal range. A comparison of bone scans, PTH and BMDs helps to evaluate bone changes in HD patients.
Yolk sac tumor is one of the histological types of germ cell tumors. Primary retroperitoneal germ cell tumors are extremely rare neoplasms. We report a case of peritoneal metastasis after resection of a primary retroperitoneal germ cell tumor. The peritoneal metastases were detected by 67Ga-scan when CT did not suggest metastatic lesions while the level of alphafetoprotein was still elevated. The 67Ga-scan showed multiple hot spots in the abdomen and pelvis 72 hours after intravenous injection of the tracer and no changes were observed in serial scan taken 6 hours after the first scan. These findings were strongly suggested the dissemination of the malignant tumors. A PET study with 18F-fluorodeoxyglucose was performed and showed extensive uptake in the intraabdominal and intrapelvic cavity. It was also useful for understanding the extension and distribution of the peritoneal metastatic lesions. The sites of the accumulation of 18F-fluorodeoxyglucose corresponded to the results found in postmortem.
A 31-year-old man was admitted to our hospital because of a sudden onset of thirst, polyposia, and polyuria. Five years previously he had been admitted to our hospital because of a dry cough. On the first admission, the chest X-ray film had shown reticular shadows and bullous changes in both upper lung fields. Histological examination of a transbronchial lung biopsy specimen had revealed that the nodular lesion in the interstitium of the alveolar lesion consisted of an aggregate of many Langerhans cells with pale cytoplasm and partly convoluted nuclei. In addition, immunoperoxidase stain for S-100 protein had been strongly positive in numerous Langerhans cells in a bone biopsy specimen from a left mandibullar lesion, which is the same histological appearance as the lung lesion. A diagnosis of pulmonary eosinophilic granuloma had been made. The course after discharge was not progressive without treatment for 5 years, but the patient suddenly began to have thirst, polyposia, and polyuria. Dehydration, vasopressin tests, and the findings of MRI indicated diabetes insipidus due to a pathological change in the pituitary gland. Although diabetes insipidus is known to be a common complication of pulmonary eosinophilic granuloma, only 9 cases have been reported in Japan.
We analyzed the bone changes at each site of secondary hyperparathyroidism (2 degrees HPT) on hemodialyzed patients using both single photon absorptiometry (SPA) and dual photon absorptiometry (DPA). The subjects were 35 hemodialysis patients (12 male, 24 female) with 2 degrees HPT. The bone mineral densities (BMD) of 1/3 radius, head, total body and 3rd lumbar spine were measured before and after parathyroidectomy (PTX). Before PTX, the BMD of 2 degrees HPT patients were lower than the normal range of all sites, especially at the radius which mainly consisted of cortical bone. After PTX, the BMD at all sites increased especially in the head. The BMD of the radius also continued to increased up two years after PTX, but it was not as increased as the BMD of head. The BMD of the lumbar spine which consisted of cancellous bone mainly increased after PTX for one year and decreased thereafter. This decrease was influenced by age. There was a difference with the site of BMD after PTX. The determination of changes of BMD of head and radius was considered useful for the judgment of treatment.
UNLABELLED: We investigated the possibility that fuzzy reasoning might be used to standardize diagnosis of liver disease based on scintigraphic results and compared the results with those obtained when scintiscans were scored conventionally. METHODS: Seventy-five patients with chronic liver disease (11 patients had chronic persistent hepatitis, 26 had chronic aggressive hepatitis and 38 had cirrhosis) and 25 controls were studied. Another 75 patients with hepatitis or cirrhosis were examined to test the effectiveness of the membership functions. Liver scintiscans were taken 20 min after the intravenous injection of 111 MBq of 99mTc-phytate. Fuzzy reasoning was used to evaluate the following five items: the ratio of the sizes of the left and right lobes, splenomegaly, radioactivity in the bone marrow, deformity of the liver and distribution of radioactivity in the liver. The degree of conformity to each of the three liver diseases being investigated was substituted into the membership function for the conclusion. The center of gravity for each patient's results was calculated. Conventional scoring was made with three levels for each of the five items examined by fuzzy reasoning. RESULTS: Distinctions between chronic persistent hepatitis and chronic aggressive hepatitis were difficult to assess with fuzzy reasoning and conventional scoring. The diagnostic accuracy was 95% for patients with cirrhosis and 88% for patients with chronic hepatitis with fuzzy reasoning. With conventional scoring the accuracy was 86% for patients with cirrhosis and 75% for patients with chronic hepatitis. When fuzzy reasoning was used to examine the other 75 patients with chronic liver diseases, the accuracy was 93% for patients with cirrhosis and 86% for patients with chronic hepatitis. CONCLUSION: The method is simple and can be used routinely in clinical settings.
In order to evaluate portal venous hemodynamics before and after TIPS, transrectal portal scintigraphy was performed in 8 patients. In all 8 patients, scintigram before TIPS demonstrated hepatofugal flow in the portal vein and less accumulation of radioisotope in the liver. In the scintigrams for follow-up of TIPS, the flow direction in the portal vein changed to be hepatopedal. In 3 of the patients in whom stent shunt was occluded after TIPS, the portal blood flow was turned to be hepatofugal again. It is concluded that transrectal portal scintigraphic studies of the TIPS patients provide noninvasive evaluation of portal hemodynamics.
OBJECTIVE: Portal circulation, in particular the contribution of the inferior mesenteric vein, can be evaluated in a relatively noninvasive way by per rectal portal scintigraphy (J Nucl Med 1988; 29:460-5). The clinical usefulness of the method was evaluated. METHODS: A solution containing technitium-99m pertechnetate was instilled into the rectum, and serial scintigrams were taken while radioactivity curves for the liver and heart were recorded sequentially. By analyses of the curves, the per rectal portal shunt index (SI) was calculated. RESULTS: The SI was higher for disorders that were more severe, increasing in the order of chronic persistent hepatitis, chronic aggressive hepatitis, and cirrhosis, and the SI was higher in cirrhotic patients than in patients with chronic hepatitis or in healthy subjects. The SI was significantly higher when a complication (varices, ascites, or encephalopathy) was present. Correlation between the SI and classic indicators for functional reserve was significant. The SI was significantly related to survival according to results of regression analysis by Cox's proportional hazards model. On the basis of the SI when patients were first examined, the patients with cirrhosis were divided into three groups of roughly equal size: group A, SI under 30%; group B, SI between 30 and 70%; and group C, SI over 70%. The survival rate was lower in group B than in A, lower in group C than in A, and lower in group C than in B. CONCLUSIONS: This method is clinically useful, especially in establishing the prognosis.
Local cerebral blood flow and glucose metabolism were examined in spontaneously epileptic El mice using autoradiography with 125I-IMP and 14C-DG in the interictal phase and during seizure. El (+) mice that developed generalized tonic-clonic convulsions and El (-) mice that received no stimulation and had no history of epileptic seizures were examined. The seizure non-susceptible, maternal strain ddY mice were used as control. Uptake ratios for IMP and DG in mouse brain were calculated using the autoradiographic density. In the interictal phase, the pattern of local cerebral blood flow of El (+) mice was similar to that of ddY and El (-) mice, and glucose metabolism in the hippocampus was higher in El (+) mice than in El (-) and ddY mice, but flow and metabolism were nearly matched. During seizure, no significant changed blood flow and increased glucose metabolism in the hippocampus, the epileptic focus, and no markedly changed blood flow and depressed glucose metabolism in other brain regions were observed and considered to be flow-metabolism uncoupling. These observations have never been reported in clinical or experimental studies of epilepsy. Seizures did not cause large regional differences in cerebral blood flow. Therefore, only glucose metabolism is useful for detection of the focus of secondary generalized seizures in El mice, and appeared possibly to be related to the pathophysiology of secondary generalized epilepsy in El mice.
We report here a Japanese family with paramyotonia congenita. The proband was a 42-year-old woman (case 1), who noticed muscle stiffness and weakness in the cold since the age of 7 years. These symptoms were alleviated by warming. Her eldest son (case 2) also experienced similar symptoms, while her younger son and daughter were healthy. Neurological examination in case 1 revealed mild weakness in facial and neck muscles. Cold-induced muscle stiffness and weakness were present. Electromyography showed myotonic discharges, intensified by cooling or repetitive exercise. The amplitude of the compound muscle action potentials was also reduced by the repetitive exercise and cooling. Serum chemistry including potassium and CK was normal. Molecular analysis of SCN4A (exon22-24) by SSCP and nucleotide sequencing revealed a C-to-T transition at nucleotide 3,938, causing a substitution of 1313methionine of threonine in case 1. This mutation was confirmed by PCR-RFLP with a mismatched primer; the proband (case 1) and her eldest son (case 2) had a heterozygous mutation, while the other family members did not. This is the first report that a mutation in SCN4A was identified in a Japanese family with paramyotonia congenita.
To clarify factors involved in the formation of cholesterol gallstones, we studied the relationship between the degree of fatty acyl chain unsaturation of biliary lecithin and bile metastability. We used supersaturated model bile solutions (molar taurocholate/lecithin/cholesterol ratio (73:19.5:7.5), total lipid concentration 9 g/dl) that contained equimolar egg yolk or soybean lecithins or a sn-1 palmitoyl, sn-2 linoleoyl phosphatidylcholine. Gel permeation chromatographic studies showed that the vesicular cholesterol distribution and dimension were inversely related to the degree of unsaturation of the lecithin species, estimated by reverse phase, high-performance liquid chromatography. Differential interference contrast microscopy and assay of cholesterol crystal growth showed that a higher degree of fatty acyl chain unsaturation of the lecithin species was associated with a faster nucleation time and rate of crystal growth. Our results suggest that vesicular lecithins containing more unsaturated fatty acyl chains bind less tightly to cholesterol than lecithins containing predominantly saturated fatty acids, and that the biliary lecithin species dictates, in part, the nucleation and growth of cholesterol crystals in bile.
Simultaneous determination of biliary lipids was performed by alkaline hydrolysis, the formation of the methyl ester derivatives of fatty acids that are constituents of phospholipids and of the acetylated methyl ester derivatives of bile acids, and subsequent analysis by capillary column gas chromatography. Complete separation and satisfactory recovery of cholesterol, bile acids, and fatty acids were achieved. Also, the accuracy of the calculation of the bile cholesterol saturation index was enhanced by computation. Since the degree of acyl chain unsaturation affects the cholesterol-holding capacity in vesicles, this method provides a unique insight into bile metastability by the quantitative assessment of fatty acids in lecithin.