KID syndrome: congenital ichthyosiform dermatosis with keratitis and deafness--report of the first case in Japan.
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Biomedical subjects
Publications and source records attributed to K Sakamoto.
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The vegetative cells and toxic filtrates of Clostridium perfringens type C strains were injected into ligated rabbit ileal loops and the responses were observed. Out of 12 strains examined, 2 strains showed positive reaction in this test, when the vegetative cells were injected. One of these 2 strains was an enterotoxigenic and beta-toxigenic and the other was beta- and delta-toxigenic but not enterotoxigenic. Culture filtrates containing beta or delta toxin also showed fluid accumulation in the rabbit ileal loop. Histological findings of loops injected with culture filtrates containing beta toxin showed separated and effaced villi, hemorrhage in the mucosa, engorged vessels, inflammatory cell infiltration, and hyperplasia of the lymphoid tissue.
Systemic sclerosis-like lesions developed in a 14-year-old boy with Wilson's disease who had been treated with D-penicillamine for 11 years. Clinical and laboratory manifestations included proximal scleroderma, pulmonary restrictive defects, positive antinuclear antibodies, and the deposition of C3 at the dermal-epidermal junction of the lesional skin. This is the first case reported in which long-term administration of penicillamine was followed by the development of systemic sclerosis-like lesions.
Even in a Schneider-oriented university clinic, the majority of the diagnoses "schizophrenia" are based on non-first-rank symptoms. About one-fifth of the cases diagnosed as having schizophrenia showed nonproductive symptoms such as disturbances of thought, of affect and of behavior. But the nonproductive forms showed a relatively high density of symptoms. Two-thirds of the patients are women, one-third men. Schizophrenia is most frequently diagnosed in the third decade of life. Single people with a low education are more commonly represented. But all this is not valid any longer if we separate schizoaffective psychoses and other atypical psychoses from "pure" schizophrenia.
The clinical features of refractory epilepsy were studied in comparison between 135 patients in a refractory group and 103 in a controlled group. All the children were Japanese. The clinical features of the refractory group were the onset of epilepsy during the first year of life, absence of family history, retarded development before the onset, phakomatoses, daily or weekly seizures, secondarily generalized epilepsy, and marked EEG abnormalities at the initial visit, a change of types in epilepsy, no improvement in EEG findings, mental deterioration or severe retardation during the follow-up. The number of drugs was increased and relatively new drugs such as carbamazepine, valproic acid or clonazepam were frequently administered. The side effects, including gum hypertrophy, drowsiness, hypertrichosis, ataxia or increased serum-GTP, were more frequent in the refractory group.
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Serum concentrations of CA 19-9, CA 125 and carcinoembryonic antigen (CEA) in 145 patients with gastrointestinal carcinomas and 89 with non-neoplastic diseases were determined to compare the clinical usefulness of these tumour markers. Significantly fewer positive cases were obtained with serum CA 19-9 (9%) and CA 125 (8%) tests than the CEA test (22%) (both p less than 0.05) in patients with benign diseases, while comparable sensitivities were achieved with the CA 19-9 (44%) test, the CA 125 (41%) test and the CEA test (47%) in those with a carcinoma. High incidences of raised concentrations of serum CA 19-9 and CA 125 were observed in case of cancer of the pancreas (CA 19-9: 87%, CA 125: 67%) and biliary tract (CA 19-9: 63%, CA 125: 48%). Combined tests of CA 19-9 and CA 125 revealed increments in the sensitivity (61%) and provided a higher specificity (87%) than that of the single CEA test (78%). These combined tests were most useful for a differential diagnosis of pancreatic carcinoma (97% positive) and biliary tract carcinoma (74%) from chronic pancreatitis (4%) and cholelithiasis (0%), respectively. Studies on the relations of clinical staging and serum concentrations of CA 19-9 and CA 125 revealed significant rises in cases of disseminated carcinoma. These results clearly show that serum CA 19-9 and CA 125 tests are most pertinent for diagnosing advanced carcinomas of organs in the digestive system.
The effects of phenol, guaiacol and m-cresol on erythrocytes, hepatocytes, dipalmitoyl phosphatidylcholine (DPPC)-liposomes and surface tension were studied at various concentrations. Phenol at 10 mM caused a slight inhibition of hypotonic hemolysis in rat erythrocytes. Guaiacol at 4 and 10 mM and m-cresol at 0.6 to 10 mM caused a significant inhibition of hypotonic hemolysis. In the enzyme leakage from isolated rat hepatocytes, phenol at 0.001 to 0.4 mM and 2 to 10 mM, guaiacol at 2 to 10 mM and m-cresol at 0.001 to 4 mM caused an inhibition in GOT leakage. The leakage of GPT from hepatocytes was inhibited by phenol at 0.4 to 10 mM, guaiacol at 2 to 10 mM, and m-cresol at 0.001 to 4 mM. m-Cresol at 10 mM caused increases in GOT and GPT leakage. The inhibition of phenol and m-cresol on the LDH leakage in hepatocytes were observed at a concentration of 0.001 mM and 0.1 to 1 mM, respectively. Guaiacol or m-cresol at 10 mM caused an increase in LDH leakage. Phase-transition temperature of DPPC-liposomes was depressed by phenol and m-cresol at 1 to 10 mM and by guaiacol at 5 and 10 mM. Guaiacol at 1 and 10 mM and m-cresol at 10 mM caused a depression of surface tension, but phenol caused no change in surface tension. The order of effects on erythrocyte, hepatocyte and DPPC-liposome membranes was m-cresol greater than phenol greater than or equal to guaiacol. In the present study, phenol and its related compound showed a positive correlation between their effects on various membranes and germicidal effects as evaluated by the phenol coefficient, but the effects were not related to a depression of surface tension.
The hypolipidemic and antiatherosclerotic effects of NIP-200 (3,5-dimethyl-4,6-diphenyl-tetrahydro-2H-1,3,5-thiadiazine-2-th ion e) were studied in experimental models of 1% cholesterol diet (HCD)-fed rabbits. The increases of total lipids, total cholesterol (TC), free cholesterol (FC) and phospholipid (PL) in plasma by HCD-feeding were inhibited by the treatment of NIP-200. The plasma contents of TC in high density lipoprotein (HDL-TC) and HDL-PL in the treatment of NIP-200 were increased more than that of the control and the HCD-fed group, but the former was not significantly different compared to the HCD-fed group. Both atherogenic index using TC-HDL-TC/HDL-TC and PL-HDL-PL/HDL-PL were reduced by NIP-200. There was a slight deposition of fatty plaques on the lumen surface of the thoracic aorta in the HCD-fed and NIP-200-treated rabbits. In the scanning electron microscopic study on the lumen surface of the aortic arch, NIP-200 reduced fatty plaques. NIP-200 also prevented the loss of endothelial fissures and swelling of the nuclei. It is suggested that the hypolipidemic action of NIP-200 may be the inhibition of lipid absorption in the intestine and the acceleration of lipid excretion in the liver. The antiatherosclerotic action of NIP-200 may be due to the improvement of lipid metabolism by the increases of HDL-TC and HDL-PL in plasma.
Hypolipidemic effects of the usual gamma-oryzanol (gamma-OZ) and a new gamma-OZ (N-gamma-OZ) with a different sterol composition from gamma-OZ were investigated on the hyperlipidemia induced by ingestion of a high cholesterol diet (HCD) containing 1% cholesterol for 12 days in male Sprague-Dawley rats. Treatment with gamma-OZ for 6 days significantly inhibited the increase in serum total cholesterol (TC) and phospholipids (PL) induced by HCD, while the treatment with gamma-OZ for 12 days did not inhibit the increase of TC and PL. Treatment with N-gamma-OZ at 100 or 1000 mg/kg for 6 days slightly inhibited the increase of TC by HCD. The decrease of TC in high density lipoprotein (HDL-TC) was markedly inhibited by treatment with N-gamma-OZ for 12 days, but N-gamma-OZ for 6 days and gamma-OZ for 6 and 12 days did not inhibit the decrease of HDL-TC. Treatment with N-gamma-OZ for 12 days significantly inhibited the increase of PL and free cholesterol (FC) by HCD. gamma-OZ at 1000 mg/kg for 12 days also inhibited the increase of FC. N-gamma-OZ significantly reduced the atherogenic index using TC and HDL-TC by affecting the HDL-TC increase. gamma-OZ at 100 mg/kg and N-gamma-OZ at 100 mg/kg for 6 days reduced the atherogenic index using TC and HDL-TC by the inhibition of TC increase. The atherogenic index using PL and HDL-PL was only reduced by the treatment with N-gamma-OZ at 1000 mg/g for 12 days. The increase of triglyceride (TG) by HCD was inhibited by the treatment of N-gamma-OZ for 6 days (all doses) and 12 days (500, 1000 mg/kg), and gamma-OZ at 500 mg/kg for 6 and 12 days also inhibited the increase of TG by HCD. gamma-OZ and N-gamma-OZ had no effects on liver lipid contents. The hypolipidemic effect of N-gamma-OZ was slightly more potent than that of gamma-OZ.(ABSTRACT TRUNCATED AT 250 WORDS)
The effect of essential oils, eugenol, thymol and menthol, on erythrocytes, hepatocytes, dipalmitoyl phosphatidylcholine (DPPC)-liposomes and surface tension were studied at various concentrations. Maximal inhibition of eugenol, thymol and menthol on the hypotonic hemolysis in rat erythrocytes were observed at a concentration of 2 mM, 1 mM and 1 mM, respectively. Eugenol at 4 mM and thymol at 2 mM caused an acceleration of hypotonic hemolysis. In isolated rat hepatocytes, thymol caused an increase in GOT leakage, but eugenol at 4 mM and menthol at 0.1 and 0.4 mM inhibited the GOT leakage. The leakage of GPT from hepatocytes was inhibited by eugenol at 0.1 mM and 0.4 to 4 mM and menthol at 0.1 to 0.6 mM. The inhibition of eugenol and menthol on the LDH leakage in hepatocytes were observed at a concentration of 0.001 to 4 mM and 0.1, 0.4 and 0.6 mM, respectively. Thymol caused no change in GPT and LDH leakage. Eugenol, thymol and menthol indicated a depression of surface tension at a concentration of 0.1 mM. The rank by order of surface activity was eugenol greater than thymol. Eugenol, thymol and menthol depressed the phase-transition temperature of DPPC-liposomes. The depression of phase-transition temperature by thymol was greater than that by eugenol and menthol. These results suggest the periapical tissue damage produced by essential oils may be related to membrane lysis and surface activity and that their tissue penetration may be related to membrane affinity and lipid solubility.
Phagocytic activity as a function of the reticuloendothelial system (RES) has been studied in ethionine induced liver injury by using the carbon clearance test. Liver damage in male and female mice was induced by DL- and L-ethionine injections (1000 mg/kg/day, i.p.). In both female and male mice, a single dose or three injections of DL- or L-ethionine caused increases in liver/body weight ratio, A/G ratio, GOT and GPT levels, and BSP retention. There was the decrease of the total protein levels in the serum. The degree of liver injury was more severe after three injections of DL- and L-ethionine than after a single injection of them. After a single injection of ethionine, the L-isomer induced a slightly greater response than the racemic mixture, except for BSP retention. On the other hand, phagocytic activities by the carbon clearance test were increased after a single injection or three injections of DL- and L-ethionine. That is, the K value was increased in all ethionine treated mice except for females with three injections of DL-ethionine. The alpha value was increased after three injections in DL- and L-ethionine treated males and DL-ethionine treated females. In addition, the increase in carbon uptake by Kupffer cells can be seen by light microscopy after a single injection or three injections of DL- or L-ethionine. These findings indicate that ethionine injections induce the enhancement of RES phagocytosis, although the biochemical parameters indicating liver injury are changed severely. These results support the data indicating no correlation between the alteration of RES activity and the degree of liver injury.
Hypolipidemic effects of gamma-oryzanol (OZ) and cycloartenol ferulic acid ester (CAF) on the hyperlipidemia induced by ingestion of a high cholesterol diet (HCD) in male Sprague-Dawley rats were investigated. The test drugs were given orally and intravenously, daily for 12 days with the HCD feeding. The oral administration with OZ and CAF at 100 mg/kg daily for 6 or 12 days did not apparently prevent the hyperlipidemia induced by HCD-feeding. The intravenous administrations with OZ and CAF at 10 mg/kg for 6 days significantly inhibited the increases in serum total cholesterol (TC), phospholipid (PL) and free cholesterol by HCD. OZ and CAF did not inhibit the decreases of TC in high density lipoprotein (HDL-TC) and HDL-PL by HCD. The increases of atherogenic index [( TC-HDL-TC]/[HDL-TC] and [PL-HDL-PL]/[HDL-PL]) with the HCD feeding were reduced by the intravenous administrations of OZ and CAF. Triglyceride, nonesterified fatty acid, lactate dehydrogenase and transaminase (GOT and GPT) markedly decreased below the control level by the intravenous administrations of OZ and CAF for 12 days. These results suggest that the intravenous administrations of OZ and CAF may have accelerated the excretion of lipids in the blood.
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A streptothricin-like antibiotic, A-269A (referred to as A-269A hereafter) was isolated from the culture broth of Streptomyces sp., strain No. A-269. In this paper, the characterization of the producer, and the production, isolation, physico-chemical properties and biological properties of A-269A are reported. The structure of the antibiotic was also examined by 1H NMR, 13C NMR and fast atom bombardment mass spectra studies. From the spectroscopic data, A-269A was assumed to be a mixture of antibiotic LL-BL 136 and an isomeric compound in which the carbamoyl group is substituted on C-12' hydroxyl instead of C-10 hydroxyl.