Mitochondrial leucine tRNA mutation in a mitochondrial encephalomyopathy.
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Biomedical subjects
Publications and source records attributed to T Sano.
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A 30 year old female was admitted with right upper abdominal pain and fever. Ultrasonography and computed tomography revealed a large cystic mass in the right lobe of the liver, and aspiration bacteriology was negative. A right hepatic lobectomy was performed for a suspected cystadenocarcinoma, however, the tumor was histologically diagnosed as a hemangiopericytoma with prominent venous invasion. The patient died within a short time of multiple pulmonary metastases. Primary hepatic hemangiopericytoma is extremely rare, and according to our research, this is only the 4th case reported in the literature.
In a 9-year-old girl with supravalvular aortic stenosis combined with severe left main coronary artery trunk obstruction (Williams syndrome), the repair for both lesions was performed successfully by simultaneous extended aortoplasty and direct coronary angioplasty using a single double-flanged pericardial patch.
Sixteen growth hormone (GH)-producing pituitary adenomas were studied for the expression of glycoprotein hormone subunits and cytokeratin by light microscopic immunohistochemistry. Cytokeratin immunoreactivity was demonstrated in all adenomas, but its intracytoplasmic distribution showed two distinct patterns; a prominent, dot-like pattern and a diffuse, perinuclear pattern. Seven adenomas (type 1) were exclusively composed of cells with cytokeratin in a dot-like pattern, whereas 9 adenomas (type 2) comprised of cells with cytokeratin of perinuclear distribution. The expression of alpha-subunit of glycoprotein hormone was significantly different between the two types of adenomas; 8 of 9 adenomas of type 2 contained many alpha-subunit immunoreactive cells but none of type 1 adenomas showed any immunoreactivity. Only a small number of adenoma cells were positive for beta-subunit of thyrotropin stimulating hormone in 3 adenomas of type 2. beta-subunits of follicle stimulation hormone and luteinizing hormone were negative in all adenomas. These findings suggest that the expression of glycoprotein hormone subunits in GH-producing adenomas may be closely linked to their types distinguishable by the cytokeratin distribution pattern.
We recently reported that six 1,4-dihydropyridine derivatives out of 57 screened effectively over-came vincristine (VCR)-resistance in VCR-resistant (P388/VCR) leukaemia-bearing mice when the dihydropyridines and VCR were administered intraperitoneally (i.p.). Furthermore, among the six dihydropyridine derivatives, two compounds, NK-250 and NK-252, most effectively overcame VCR-resistance while exhibiting relatively low calcium antagonistic activity and toxicity. In this study, we examined whether NK-250 and NK-252 could potentiate the antitumour activities of etoposide in mice with drug-sensitive (P388/S) or VCR-resistant (P388/VCR) leukaemia cells when the anticancer agents and tumour cells were administered by various routes. In both groups of mice inoculated i.p. with P388/S- and P388/VCR-leukaemia cells, the oral (p.o.) administration of NK-250 combined with i.p. or intravenously (i.v.) administration of etoposide (ip-po-ip trials and ip-po-iv trials) dramatically potentiated the antitumour activity of etoposide. Although etoposide alone was less effective in treating mice inoculated i.v. with P388/S- and P388/VCR-leukaemia cells, p.o. administration of NK-250 combined with i.p. or i.v. administration of etoposide (iv-po-ip trials and iv-po-iv trials) potentiated the antitumour activity of etoposide to similar levels as in treating mice inoculated i.p. with leukaemia cells. These 1,4-dihydropyridines were therefore highly effective in potentiating anticancer drugs against both drug-sensitive and drug-resistant tumours.
We have expressed a gene fusion of streptavidin and the two immunoglobulin G (IgG)-binding domains of protein A in Escherichia coli. The purified chimeric protein had full biotin-binding ability, and bound one IgG molecule per subunit (31.4 kD). The affinity of the chimeric protein both for biotin and IgG can be used to provide antibody molecules with additional recognition capabilities. For example, the chimera will allow any IgG molecule to be used for sensitive biotin-linked detection systems without the need for chemical modification.
We studied three cases of adrenocortical neoplasms that were detected incidentally after radiological examination of the abdomen. These cases did not demonstrate any clinical evidence of adrenocortical abnormalities, such as virilization. Macroscopically, the tumors were light to dark tan on cut surface. Light-microscopic examination revealed compact cells with abundant lipid-sparse eosinophilic cytoplasm and occasional enlarged nuclei. In one case, ultrastructural observation demonstrated abundant mitochondria. Immunohistochemical examination of all of the adrenocortical steroidogenic enzymes showed that none of the cases had immunoreactivity. No mitotic activity and no vascular invasion was observed. The postoperative course were uneventful. The follow-up interval varied from 8 to 27 months. These three neoplasms apparently represent the first reported cases of adrenocortical oncocytoma. They can be considered true nonfunctioning adrenocortical neoplasms because steroidogenic enzymes required for corticosteroid biosynthesis were not expressed in the tumor cells.
The patient was a 19-year-old female student who purchased a puppy from a pet shop four weeks earlier. At the time of her first examination, an annular edematous erythema with adherent scales and vesicles surrounding its margin was seen on the left forearm. On direct examination of the vesicles, fungal elements were detected, and Microsporum canis was isolated. The puppy was a Pomeranian and was kept in the house at all times. No clinical lesions were seen on the puppy, and the Wood's lamp test was negative. However, M. canis was isolated from the animal by the hairbrush method. Symptoms disappeared after the patient was treated topically with terbinafine cream for three weeks. Although the dog received no treatment whatsoever, there was no evidence of the disease on the pet. Results of the hairbrush method performed on the pet two and three weeks later were negative, but, at five weeks, it was again positive. Human infection with M. canis from an asymptomatic dog was demonstrated in this case. Attention should be paid to preventing infections from animals without lesions.
Massive intestinal protein loss was demonstrated and the site of loss determined by abdominal scintigraphy with 99mTc-labeled human serum albumin in a 9-year-old girl following the Fontan operation for pulmonary atresia with intact ventricular septum. Significant activity accumulating in the lower small intestine and moving with its contents into the colon were shown. Her condition may have resulted from intestinal lymphangiectasia, caused by increased central venous pressure. Abdominal scintigraphy with 99mTc-human serum albumin is useful in the diagnosis of protein-losing enteropathy.
All samples of cyanobacterial blooms collected from 1986 to 1989 from Lake Kasumigaura, Ibaraki Prefecture, Japan, were hepatotoxic. The 50% lethal doses (LD50s) of the blooms to mice ranged from 76 to 556 mg/kg of body weight. Sixty-eight Microcystis cell clones (67 Microcystis aeruginosa and 1 M. viridis) were isolated from the blooms. Twenty-three strains (including the M. viridis strain) were toxic. However, the ratio of toxic to nontoxic strains among the blooms varied (6 to 86%). Microcystins were examined in six toxic strains. Five toxic strains produced microcystin-RR, -YR, and -LR, with RR being the dominant toxin in these strains. Another strain produced 7-desmethylmicrocystin-LR and an unknown microcystin. This strain showed the highest toxicity. Establishment of axenic strains from the Microcystis cells exhibiting extracellularly mucilaginous materials was successful by using a combination of the agar plate technique and two-step centrifugation.
Cyclothiazomycin is a novel renin inhibitor produced by Streptomyces sp. NR0516. It was isolated from fermentation broth by extraction with butyl alcohol, QAE-Toyopearl column chromatography and preparative HPLC. Cyclothiazomycin, which was determined to be a unique polythiazole-containing bicyclic peptide, exhibited inhibitory activity against human plasma renin with IC50 being 1.7 microM.
A 56-year-old man, tentatively diagnosed as having cardiomegaly, was found to have a large cyst in the left anterior mediastinum. At thoracotomy a multilocular cystic mass containing a colorless serous fluid was found and resected. Histologically, the cyst was lined with flattened epithelial cells, and thymic tissue was present in the cyst's wall; a diagnosis of thymic cyst was made. There were no malignant components or inflammatory changes. Sialylated Lewis X-i (SLX), carbohydrate antigen 19-9 (CA19-9) and tissue polypeptide antigen (TPA) in the cystic fluid were markedly elevated, while the serum concentrations of these tumor markers were within the normal ranges. Immunohistochemical studies showed the epithelial cells lining the cyst to contain CA19-9 and SLX. The findings suggest the tumor markers to have originated in the thymic epithelium lining the cyst.
Experimental tractional retinal detachments in rabbits' eyes was produced several weeks following aspiration of vitreous gels through a posterior small window of the eye wall. Histological examination showed that intravitreal proliferative tissues contained many fibroblasts, derived from the optic disc a small amount of migrated RPE, macrophages and other cells associated with proliferative tissues, Glial cells also grew especially at the epi-retina, causing shrinkage of the sensory retina. Vitreous neovascularization deriving from the retinal vessels was also found in these proliferative tissues. Our experimental data indicated that the extent of proliferative tissues mainly depend upon the amount of vitreous loss and vitreous hemorrhage, and the duration of retinal detachment. This experiment may be useful as an animal model of vitreous neovascularization.
We investigated immunoreactivity for galanin, a 29-amino acid peptide, in formalin-fixed, paraffin-embedded sections of 123 neuroendocrine tumors. Galanin-immunoreactive cells were found in one of 12 hypothalamic gangliocytomas, nine of 18 adrenal pheochromocytomas, nine of 14 pituitary corticotroph adenomas, and one of two thymic endocrine tumors. In pheochromocytomas, galanin-immunoreactive cells were seen either singly or in clusters. In corticotroph adenomas, many tumor cells were positive for galanin, indicating colocalization of corticotropin and galanin in the same tumor cells. No galanin-immunoreactive cells were noted in four extra-adrenal paragangliomas; 10 medullary carcinomas of the thyroid; 35 endocrine tumors arising in the lung, pancreas, and gastrointestinal tract; and 28 pituitary adenomas composed of cells other than corticotrophs. In nontumorous counterparts of these neuroendocrine tumors, galanin immunoreactivity was observed in nerve cells of the hypothalamus, nerve fibers of the duodenum, and adenohypophyseal cells corresponding to corticotrophs. These findings indicate that galanin expression in neuroendocrine tumors is uncommon and restricted to some tumor types.
Left ventricular contractile function was compared between primary and two-stage arterial switch operation (ASO) in neonates and infants with simple transposition of the great arteries (TGA). Eight patients (group 1) underwent primary ASO at 13-42 (23 +/- 10 [mean +/- SD]) days of age, and five patients (group 2) underwent two-stage ASO after pulmonary artery banding and Blalock-Taussig shunting at 11-26 (15 +/- 3) months of age. Postoperative echocardiographic studies were undertaken at 6-48 (22 +/- 16) months of age in group 1 and 25-45 (32 +/- 7) months of age in group 2. The results were compared with those obtained from nine age-matched normal children (group N). To compare left ventricular contractile state among the patients, the deviations from the mean normal values in relations of end-systolic wall stress to fractional shortening and to rate-adjusted mean velocity of circumferential shortening (the stress-shortening index and stress-velocity index, respectively) were obtained for each patient. The stress-shortening index was significantly lower in group 2 (-3.1 +/- 3.1) than in group 1 (1.3 +/- 1.7, p less than 0.05); the stress-velocity index was significantly lower in group 2 (-3.3 +/- 1.8) than in group 1 (1.5 +/- 1.6, p less than 0.01) or in group N (-0.4 +/- 0.5, p less than 0.05). These results suggest that postoperative left ventricular contractility can be well preserved in patients after primary ASO for simple TGA in neonates, but there is a possibility of depressed contractility in patients after two-stage ASO.
A 52-year-old woman presented slight fever, diffuse papular skin rash and painful cervical lymph node swelling. Her lymph node swelling generally up to 3 cm in diameter, with petechiae on the lower legs and hepato-splenomegaly within a few weeks. ESR was 45 mm/h, Hb 10.0 g/dl, RBC 345 x 10(4)/microliter, WBC 22,600/microliter (atypical lymphocyte 47%), PLT 1.0 x 10(4)/microliter, GPT 91 U/L, gamma-globulin 34.3%, EBV-VCA x 2,560, EBNA x 20, and anti-rubella antibody x 512. The biopsied cervical lymph node showed histologic features of effacement of nodal architecture by an exuberant vascular proliferation accompanied with infiltration of the immunoblasts, and was diagnosed as immunoblastic lymphadenopathy (IBL)-type lymphadenopathy. The pulse therapy of methylprednisolone and high dose of gamma-globulin improved lymphadenopathy, thrombocytopenia and anemia. IBL-type lymphadenopathy after infection of rubella virus may be different from true IBL, but is important to discuss the pathogenesis of IBL.
An 86-year-old man was admitted to our hospital with complaints of dyspnea and productive cough, and diagnosed as small cell lung cancer with sputum cytology. We concluded that he was unsuitable for standard aggressive intravenous chemotherapy because of an old age, a poor performance status and cardiac complication. He was treated with oral etoposide (100 mg/day x 16.5 days), and showed a good partial response and improvement of clinical symptoms. Toxicities were leukopenia, alopecia, and anorexia. Daily oral administration of etoposide is considered to be effective and useful for elderly patient with small cell lung cancer.
A case of sphenoid ridge meningioma and pituitary adenoma adjacent in the brain is reported. A 70-year-old female was admitted to our hospital with headache. She had no neurological deficit but did have acromegalic change. Hormonal examination showed elevation of plasma levels of HGH (19.0 ng/ml), with normal levels of the other hormones. CT examination revealed a tumor with calcification in the inner third of the sphenoid ridge and another in the pituitary fossa with suprasellar expansion. MRI showed flow void of ICA between these tumors. Intensity of the T1-weighted image of the tumor in the sphenoid ridge was homogeneously iso intensity, and low intensity in the pituitary fossa. The diagnosis of adjacent tumors in the sphenoid ridge meningioma and pituitary adenoma had been made preoperatively. Left front-temporal craniotomy and removal of these tumors were performed. These tumors were close to each other, but were separated by the internal carotid artery and anterior cerebral artery. Pathological examination demonstrated meningotheliomatous meningioma in the sphenoid ridge and sparsely granulated somatotroph adenoma in the pituitary fossa. Fourteen cases showing association of meningioma and pituitary adenoma, which had no history of radiation and trauma, have been reported previously. Although GH producing pituitary adenoma may stimulate adjacent dura and arachnoid cells resulting in the formation of meningioma, the possibility of coincidental occurrence of the two tumors cannot be ignored.