[Giant cell tumors of the skull. Report of three cases].
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Biomedical subjects
Publications and source records attributed to S Fujiwara.
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We experienced two cases of "Osteosclerosis" who were 12 and 15 year old sisters. Previous reported cases of this disease are 50 cases and among them only one patient was reported in Japan. Osteosclerosis seems to be inherited as an autosomal recessive trait. Parental consanguinity is also observed. The peculiar facies are evident in infancy, characterized by broad, flat nasal bridge, ocular hypertelorism and prognathic, broadened mandible. Commonly, they have cutaneous syndactylies in bilateral hands and feet, especially between the second and third finger and toe. Roentgenographically, hyperostosis with osteosclerosis can be observed in systemic bones, particularly the calvarium is greatly thickened. Since such a bony change occurs most severely at the base of the skull, important clinical symptoms of this disease are cranial nerve palsies resulting from obliterations of unilateral or bilateral several cranial nerve foramina. In many cases deafness due to progressive encroachment upon the middle ear cavities and auditory nerve canals appears early in infancy. Transient palsy of the facial nerve occurs somewhat later, and bilateral facial paralyses are usually permanent in adulthood. In some cases optic atrophy and visual field defect due to compression of the optic nerves are late complications. Other ocular symptoms are strabismus, nystagmus and exophthalmos. Anosmia and trigeminal nerve palsy are less common. Lower cranial nerve symptoms can not be noted but the reason is unclear. Chronic headache, convulsion and mental retardation are occasionally present. They are considered as a result from increased intracranial pressure due to progressive diminution of the cranial capacity. By same mechanism, several patients have died suddenly from impaction of the medulla oblongata in the foramen magnum in early adulthood. Then, some reporter puts emphasis on prophylactic opening of the foramen magnum in all adult cases.
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A basement membrane collagen has been isolated from limited pepsin digests of bovine lung alveolar tissue by differential salt fractionation and DEAE-cellulose chromatography. Fractionation and characterization of the peptide chain fragments of the collagen have revealed the presence of two classes of collagen chains: one is 160K-1, 100K-1, and 80K-1 and the other is 100K-2 and 80K-2. Apparent isoelectric points of these chain fragments were 8.4, 8.6, 8.35-8.45, 8.9-9.0 and 8.9-9.0, respectively. Electrophoretic analysis on temperature dependent reductions of the collagen and its denaturation products has revealed the presence of disulfide bonds between collagen chain fragments of 160K-1-80K-2 and possibly 160K-1-160K-1 which require elevated temperatures for reduction and the bonds within 100K-2 fragments (80K-2-small fragments such as 10K-20K) and possibly between 80K-2-80K-2 which are reducible without elevated temperatures.
Type I collagen was prepared from bovine renal cortices by pepsin digestion followed by differential salt fractionation, and was identified by SDS-polyacrylamide gel electrophoresis, CM-cellulose chromatography, and by the analysis of CNBr-cleavage products of the alpha 1 chain. About 61-87% of total collagen in the tissue was solubilized by this procedure and type I collagen represents about 40% of the collagen solubilized. Renal cortex type I collagen is characteristic in that the extent of hydroxylation of the prolyl residues is high, but that of the lysyl residues is at the same level as in skin. Tissue-specific differences in the hydroxylation of prolyl residues of type I collagen are also discussed.
Type specific rabbit antibodies to bovine type I, II, III, and IV (basement membrane) collagens showing no cross-reaction with other types of collagen were prepared by cross-adsorption and diethylaminoethyl-cellulose chromatography. The antibodies to bovine type I and III collagens showed a high cross-reaction with the corresponding human collagens, but those to type II and IV collagens did moderate and no cross-reactions with human type II and IV collagens, respectively. By using these antibodies, tissue distribution of various types of collagen in normal bovine lung was examined by indirect immunofluorescence microscopy. both type I and III collagens were found to distribute widely in the interstitium of bronchial tree, bronchial lamina propria and of interlobules as well as alveolar nipples and adventitia of pulmonary arteries. Type II collagen was located only in bronchial cartilage. The tissues mainly stained for type III collagen were the alveolar interstitium (also stained faintly for type I collagen) and the intima and media of the arteries. Type IV collagen was located in a membranous fashion in alveolar septa and bronchial smooth muscles and subepithelial layers as well as capillaries and the intima and media of arteries.
Chronic toxicity and recovery tests of labetalol hydrochloride, alpha-and beta-adrenoceptor blocking agent, were carried out using male and female Wistar strain rats. The drug was orally administered at 50, 100 or 200 mg/kg/day for 9 months. In all the drug-treated groups, increase in salivation was observed from immediately after to 15 minutes after dosing through treatment period. Suppression of body weight gain was observed in male rats in the 200 mg/kg/day group. Food comsumption tended to be higher in all the drug-treated groups than in the control group. Similar trend was seen also in water comsumption, and increase in urine volume was noted in the groups treated with 100 and 200 mg/kg/day. In the serum biochemical examination, dose-dependent elevation in potassium level was noted in all drug-treated groups, but the values were within the range of physiological variation. In the 100 and 200 mg/kg/day groups, an increase was observed in the absolute heart weight and in its relative weight against body weight. Major abnormalities found in histopathological findings were; swelling of parenchymatous cells in liver and kidney, swelling of fibers and swelling or proliferation of interstitial cells in cardiac and skeletal muscles, and congestion in spleen. No notable abnormality was found in any examination item in the recovery test.
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We have experienced a case of a 35-year-old man with a lesion located on the left lateral surface of the floor of the 4th ventricle and showing high density on CT, part of which showed positive contrast enhancement. The patient was successfully treated by complete excision of the lesion. Pathological study indicated arteriovenous malformation.
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Two inbred strains of guinea pigs, Strain 13 and JY-1, were compared with regard to their susceptibility to the elicitation of experimental allergic encephalomyelitis (EAE) by passively transferred sensitized lymph node cells. Recipient guinea pigs were injected with more than 2 X 10(8) of viable lymph node cells from donors that had been immunized for the elicitation of EAE 8 days prior to the cell transfer. 1) Clinical course of EAE in all adult guinea pigs passively transferred with adult and juvenile sensitized lymph node cells was acute. 2) Sensitized cells required for successful passive transfer of EAE was less in JY-1 guinea pigs than in Strain 13 guinea pigs. 3) The sensitized cells from juvenile Strain 13 guinea pigs were less competent to transfer EAE than the adult ones whereas in JY-1 guinea pigs the juvenile cells were as effective as the adult ones.
The electrochemical behavior of iron storage protein, ferritin (molecular weight 700 000) was studied by pulse polarography, cyclic voltammetry and 'potentiometry' monitored by colorimetry. Comparison between in vivo and voltammetric redox reactions of the ferritin Fe(III) core and thermodynamic E'0 values for native ferritin are described.