[An adult case with clinical features similar to Shwachman syndrome].
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Biomedical subjects
Publications and source records attributed to K Miyazaki.
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A case of infantile neuropathy with signs of respiratory distress and impaired abdominal movements from the neonatal period was reported. Muscle biopsy revealed type II fiber predominance and atrophy, and the sural nerve biopsy demonstrated the absence of myelin in almost all of the axons. Onion bulb formation or myelin debris was not observed. The features of myelin deficit without active myelin breakdown and onion bulb formation, and the finding of muscle pathology, indicating the absence of spinal muscular atrophy, suggested that the case under study was identical to that of hypomyelination neuropathy.
A surgical case of an aggressive form of adenoid squamous cell carcinoma of the penis was studied. The histological features of the tumor originating from the coronal region of the penis showed a well differentiated keratinized squamous cell carcinoma in the superficial area, which transformed into, with a zone of transition in between, an alveolar structure in the deep invading portion and in the metastatic tumor in the inguinal lymph nodes. The alveolar lining cells exhibited an undifferentiated appearance with prominent nucleoli, frequent mitotic figures, and vascular invasion. These cells were neither dyskeratotic nor acanthotic as described in the literatures. Mucin was negative in the tumor cells and angiosarcoma was ruled out with a silver impregnation technique. The patient took a rapid downhill course for the ordinary squamous cell carcinoma of the penis, and he expired eight months after penile amputation with a radiological evidence of lung metastasis. These unusual pathological features and an aggressive behavior of this tumor which were not hitherto described for adenoid squamous cell carcinomas necessitated this report. Autopsy was not performed.
We report on hormonal regulation of protein kinases in the rabbit myometrium. The injection of estrogen or progesterone produced 5.2 +/- 0.7- or 4.7 +/- 0.4-fold increases, respectively, of the total activity when histone was used as the substrate in the cytosol fraction of the tissue. The analysis of the activity by DEAE-cellulose column chromatography revealed that estrogen had no effect on increases in the levels in both types I and II cAMP-dependent protein kinases, on the basis of mg protein, while progesterone produced a 50% decrease in type I cAMP-dependent protein kinase and a 50% increase in type II cAMP-dependent protein kinase. Thus, the activity ratio of types II to type I did not change by treatment with estrogen and increased from 2.8 to 7.9 with progesterone administration. As the activity patterns by treatment with human chorionic gonadotropin closely resembled those seen with progesterone, our findings in case of the latter hormone are probably valid. The results indicate that the levels of protein kinases in the myometrium are altered in response to estrogen and progesterone and may possibly be involved in the physiological functions of the tissue.
Myosin light chain kinase activity and the amount of calmodulin were measured in rabbit myometrium after the rabbits had been treated with oestrogen, progesterone and human chorionic gonadotrophin, and also during pregnancy. Injections of oestrogen and progesterone produced significant increases of of 2.8- and 2.1-fold in the enzyme activity/mg protein and of 1.4- and 1.2-fold in the calmodulin content/mg protein respectively. The increases seen with oestrogen treatment were inhibited by cycloheximide administration, suggesting that protein synthesis is involved in the process. Calmodulin increased about twofold in the particulate fraction under conditions of oestrogen and progesterone treatments and the raised level of calmodulin detected in the cytosol fraction was not due to translocation from the particulate fraction. During pregnancy the specific enzyme activity significantly increased in comparison with the values seen in samples from the non-treated rabbits. These findings suggest that myosin light chain kinase and calmodulin in the myometrium are under hormonal regulation and may play a role in expulsion of the fetus at the latter phase of pregnancy.
By the administration of carcinogens to the fetal membrane of pregnant rats, yolk sac tumors producing AFP was induced. The process of production of such tumors and AFP was studied at the ultrastructural level. Electron microscopy revealed that the structure of the tumor induced extended radiately around the tubal cavity, and the cubical cells were arranged in rows. Microvilli were observed on the cellular surface toward the tubal cavity. The adjacent cells were relatively diffused and an interdigitation was partly observed, but formation of incomplete desmosome was also apparence. The nuclei were generally large, some being irregular in shape. Nucleoli with a thread-like nucleolonema were also observed. There was rich organella in the cytoplasm and mitochondria and r-ER was particularly noted. In the r-ER the intracavity was enlarged containing some substances with moderate density. Some granules with the same density as said substance were present in the cytoplasm. This substance was recognized to be AFP. As the source of the material, rich free-ribosome and glycogen granules were observed around the r-ER with an amorphous substance. Due to the effect of functional collaboration, AFP was produced and accumulated in the r-ER according to the same process as protein synthesis.
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We conducted a randomized double blind comparison of cefamandole (CMD) and cefmetazole (CMZ) in the treatment of 193 patients with complicated urinary tract infections. The patients received 1 gram of CMD or CMZ twice a day intravenously by drip infusion over 1 hour for 5 days. Pretreatment urinary leukocyte counts and urinary bacterial counts were at least 5 cells/hpf and 10(4) bacteria/ml, respectively. Each patient was randomly allocated either to CMD or CMZ group. There were 93 patients in CMD group and 100 patients in CMZ group. Clinical efficacy was evaluated based on the effect of treatment on bacteriuria and pyuria according to the criteria set by the UTI Committee, Japan. The response to CMD treatment was excellent in 18 cases (19.4%), moderate in 38 cases (40.9%) and poor in 37 cases (39.8%) with an overall effectiveness of 60.2%, whereas the response to CMZ was excellent in 19 cases (19.0%), moderate in 40 cases (40.0%) and poor in 41 cases (41.0%) with an overall effectiveness of 59.0%. No statistical significant difference was found between 2 treatment groups. Comparison of the bacteriological response between 2 groups showed that the eradication rate for strains of Gram-positive cocci were significantly higher in those patients treated with CMD. Gram-negative rods were eradicated from 68.4% of cases treated with CMD, and 78.0% of those with CMZ, but the difference was not significant. Adverse reactions were observed in 3 patients receiving CMD 1 case each of diarrhea, eruption and epigastric pain. Abnormality in laboratory tests was found in 6 patients in each treatment group. The results indicate that CMD is effective, safe and useful in the treatment of patients with complicated urinary tract infections, and its efficacy, safety and usefulness are comparable with those of CMZ.
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A boric acid and zinc oxide ointment (J.P. VIII) is an unique preparation in Japan, which consist of boric acid (5%), zinc oxide (10%), vegetable oil (usually soybean oil or sesame oil) and yellow wax. The ointment is widely used in the area of Hokkaido, because not only of the customary prescription but also of the characteristic clinical efficiency. However, boric acid has been recognized to be harmful in these days. Therefore, a zinc oxide ointment consisting 10% zinc oxide, soybean oil and white beeswax was tentatively made and evaluated. The zinc oxide ointment presented the same consistency as the boric acid and zinc oxide ointment, measured with penetrometer. The clinical efficiency was also confirmed on six patients with chronic eczema and seven patients with psoriasis vulgaris. The effect on wound healing of the donor site of skin graft was as good as the previous preparation.
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An extremely rare case of malignant degeneration arising separately in benign cystic teratoma of the bilateral ovaries is reported in a 51-year-old woman. The solid part of the right tumour consisted basically of squamous cell carcinoma, but had a small area of adenocarcinoma, whereas the solid part of the left tumour consisted of squamous cell carcinoma. It was found that in both the cystic teratomas, squamous cell carcinoma had developed separately from the columnar epithelium by a process of squamous metaplasia and malignant change in situ. It is suggested that these malignant degenerations of benign cystic teratoma of the bilateral ovaries had occurred independently due to local carcinogenic agents in the constituents of each cyst content.
Uptake and conjugation of acetaminophen were studied in isolated rat hepatocytes. Acetaminophen was taken up by the cells and the conjugates formed in the cells were rapidly released from the cells at a constant rate. The kinetic analysis demonstrated that uptake was due to a saturable process as well as a simple diffusion. Decrease in uptake of acetaminophen by metabolic inhibitors suggested that uptake of acetaminophen by hepatocytes might be, at least in part, due to an energy dependent process.
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