[Argininosuccinate synthetase (ASS)].
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Argininosuccinate Synthase↗
Biomedical subjects
H Ichiki
Publications and source records attributed to H Ichiki.
Participation of nitric oxide in mouse anaphylactic hypotension.
Mouse hen egg-white lysozyme-specific anaphylaxis was estimated by monitoring changes in blood pressure by using a tail-cuff method. Stimulation of histamine H1 receptors of the vascular endothelium was suggested to be critical for mouse anaphylactic hypotension, because pretreatment with diphenhydramine but not with cimetidine completely inhibited the hypotension. Nitric oxide (NO) was indicated to play an important role in mouse anaphylaxis, because NG-nitro-L-arginine methyl ester, a NO synthase inhibitor, significantly blocked the hypotension while a large amount of L-arginine, a precursor of NO synthesis, restored the hypotension.
Anaphylaxis↗
Generation of active oxygens, cell deformation and membrane potential changes upon UV-B irradiation in human blood cells.
The generation of peroxides (presumably hydrogen peroxide) by UV-B irradiation of human blood cells was detected. Non-fluorescent dihydrorhodamine 123 (DHR) is oxidized to fluorescent rhodamine 123 (R123) by H2O2 or peroxides with a stoichiometry of 1:1 in the presence of exogeneous peroxidase, and the fluorescence of R123 within the cells was measured using flow-cytometry. UV irradiation gave rise to changes in the cellular volume and the membrane potential, whose extent and direction were dependent on the type of blood cells. The production of peroxides (H2O2) in polymorphonuclear leukocytes is the largest among blood cells at the lower dose (< 0.1 J/cm2), and the production decreases with an increase in the dose, while the production in platelets is the smallest at the lower dose, but above 0.4 J/cm2 it increases suddenly so that at the higher dose (1.2 J/cm2) it amounts to 3.3 x 10(-16) mol/cell. For monocytes and lymphocytes, the production increases gradually with the increase in the dose.
Blood Cells↗
Pericarditis as the initial manifestation of malignant thymoma. Disappearance of pericardial effusion with corticosteroid therapy.
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Cardiac Tamponade↗
[A case of idiopathic pulmonary fibrosis associated with bilateral pulmonary arterial thrombosis found at autopsy].
A 73-year-old woman admitted to the hospital with dyspnea on exertion. Chest radiography revealed a diffuse interstitial shadow; PaO2 was 72 Torr, and PaCO2 was 41 Torr. Laboratory examination results were compatible with idiopathic pulmonary fibrosis (IPF). Prednisolone relieved the dyspnea, but tapering of the drug led to a recurrence of this symptom. Pulse therapy was started and azathioprine was added to the corticosteroid. Over the course of 6 months of treatment, the patient's respiratory function remained fairly stable. Then respiratory distress was induced by an attack of atrial fibrillation, with relief provided by anti-arrhythmic drugs and large doses of corticosteroids. The patient died suddenly 3 weeks later. An autopsy revealed large thrombi in both pulmonary arteries with 90% stenosis. Parts of the thrombi were organized, which suggests that 2-3 weeks had elapsed since initial thrombus formation. Histological examination of lung tissue showed usual interstitial pneumonia. Pulmonary thromboembolism should be considered in patients with IPF if respiratory distress suddenly and unexpectedly worsens.
Aged↗
[Evaluation of CEA, SLX and CA125 in active pulmonary tuberculosis].
Serum tumor markers (CEA, SLX, CA125) were evaluated in 123 patients with active pulmonary tuberculosis. The results were as follows: Elevated serum CEA, SLX and CA125 levels were found in 16.9%, 39.5% and 44.4%, respectively, of patients with active pulmonary tuberculosis. Markedly elevated serum CA125 levels were found in some cases. On chest X-ray films, the factors associated with elevation of serum tumor marker levels were examined. Serum CEA and SLX levels were correlated with the extent of disease. Pleural effusion had no influence on serum tumor marker levels. Serum SLX levels were higher in patients without a tuberculous cavity than in those with a tuberculous cavity. After therapy with antituberculous drugs, the mean values of serum SLX and CA125 levels significantly decreased. The decrease in mean value of serum CEA levels was not significant, but in 4 of 8 cases, elevated serum CEA levels were normalized after therapy. On the other hand, two cases with increased serum CEA or SLX level died of respiratory failure. These results suggest that to follow up patients with elevated serum tumor markers is useful for the evaluation of disease activity of pulmonary tuberculosis. Further elevation of serum tumor marker levels after antituberculous therapy may indicate the presence of cancer or deterioration of the disease.
Adolescent↗
[A case of endobronchial chondroma in a patient with atypical mycobacterial infection].
A case of endobronchial chondroma in a 74-year-old man with atypical mycobacterial infection is reported. Bronchoscopy was performed in order to exclude the possibility of malignancy. At bronchoscopic examination, a hemispherical polypoid tumor with smooth surface was found at the orifice of left B3 and was removed by transbronchial biopsy forceps. Histologically the tumor was composed of cartilage tissue. About 1 year after forceps resection, no growth of the tumor was seen in the left upper lobe bronchus on bronchoscopic examination. Endobronchial chondroma is relatively rare. We discuss the clinical features of 19 cases of endobronchial chondroma so far reported in Japan, including our case.
Aged↗
[Three cases of lung cancer with calcification--demonstration by chest CT].
We report three cases of lung cancer with calcification shown on chest CT scans. Case 1 was a 63-year-old man with well differentiated squamous cell carcinoma of the lung. Chest CT scan showed pronounced central calcification within the tumor. Case 2 was a 57-year-old woman with small cell carcinoma of the lung. Chest CT scan showed diffuse amorphous calcification within the tumor. Case 3 was a 70-year-old man with lung cancer for which pathologic data was not available. Chest CT scan showed punctate and linear calcification of the cavity wall within the tumor. Microscopic examinations revealed dystrophic calcification in cases 1 and 2. Calcification has long been recognized as a distinguishing radiologic feature of a benign pulmonary mass, however, the presence of calcification within a tumor on chest CT scan does not exclude the diagnosis of lung cancer.
Aged↗
[Two cases of adult onset of Still's disease in the elderly].
Two cases of adult onset of Still's disease is in a 83-year-old and a 61-year-old women. Both cases complained of high fever, arthralgia, sore throat and maculae. Examinations on admission revealed leukocytosis and negative antinuclear antibody. Administration of prednisolone resulted in improvement of the clinical symptoms and laboratory data. In general, the onset of this disease is usually in early adulthood, but in these two cases the onset was at an advanced age. These results suggest that Still's disease of adult onset should be included in the differential diagnosis of fever of unknown origin, if the patient is elderly. In the treatment of the disease, proper attention to side effects of prednisolone and complications can be important.
Aged↗
[A case of idiopathic pulmonary fibrosis with histology of usual interstitial pneumonia that responded to pulse therapy followed by combined immunosuppression with prednisolone and azathioprine].
A 64-year-old woman who was admitted with cough and dyspnea showed severe hypoxemia and interstitial lung shadows. The clinical diagnosis was idiopathic interstitial pneumonia (synonymous with idiopathic pulmonary fibrosis in the United States), since there were no specific immunological or bacteriological findings. No clinical signs or laboratory data compatible with collagen disease were observed. Methylprednisolone pulse therapy was given followed by prednisolone (0.8 mg/kg) and azathioprine (15 mg/kg). Marked improvement of hypoxia, chest X-ray and spirometry results was observed after five weeks. Histological examination of an cases of residual interstitial shadow obtained by open lung biopsy revealed usual interstitial pneumonia. Tapering of the immunosuppressant drugs led to a recurrence 3 months later, which was controlled by reintroduction of the same regimen. Therefore, only prednisolone was tapered, and data obtained in an outpatient clinic 6 months after the recurrence were as follows: %VC 108%, %DLco 72%, PaO2 80 Torr. The value of this regimen for acute IPF or exacerbation of IPF is suggested because of its life-saving effects.
Aged↗
[Immunological reactions in patients with pulmonary aspergilloma].
A 52-year-old man with pulmonary aspergilloma is reported. In immunoassays, type I, III and IV hypersensitivity reactions of Gell & Coombs were observed. The finding of such immunological reactions, especially type I hypersensitivity, has rarely been observed in this disease. The present study was performed to investigate these reactions, especially to determine whether type I allergy participates in pulmonary aspergilloma. We reviewed 126 cases of pulmonary aspergilloma reported in the Japanese literature between January 1980 and April 1991. The following results were obtained: Elevation of serum IgE was observed in 49% of the subjects examined, positive IgE RAST score in 28%, precipitating antibody against A. fumigatus in 79%, and positive lymphocyte stimulation test using A. fumigatus antigen in 68%. In conclusion, type I, III and IV hypersensitivity of Gell & Coombs appears to participate in pulmonary aspergilloma. The occurrence of type I hypersensitivity is not rare in this disease.
Aspergillosis, Allergic Bronchopulmonary↗
Immunocytochemical localization of argininosuccinate synthetase in the rat brain.
The neuronal distribution of argininosuccinate synthetase (ASS) was mapped in the rat brain. Argininosuccinate synthetase is one of the enzymes of the arginine metabolic pathway and catabolizes the synthesis of argininosuccinate from aspartate and citrulline. Since arginine is the precursor of nitric oxide, argininosuccinate synthetase may act as part of the nitric oxide producing pathway. Argininosuccinate is also suggested to have a messenger function in the nervous system. Therefore, the localization of ASS is of great interest. Polyclonal antisera against purified rat liver argininosuccinate synthetase revealed a characteristic distribution pattern of argininosuccinate synthetase-like immunoreactivity: (1) many neurons with strong argininosuccinate synthetase-like immunoreactivity were observed in the septal area, basal forebrain, anterior medial and premammillary nuclei of the hypothalamus, anterior and midline thalamic nuclei, dorsal endopiriform nucleus of the amygdala, basal nucleus of Meynert, subthalamic nucleus, laterodorsal tegmental nucleus, raphe nuclei, nucleus ambiguus, and the area postrema, (2) neuropile staining was dense in the septal areas, hypothalamus, area postrema, nucleus of the solitary tract, and the laminae I and II of the caudal subnucleus of the spinal trigeminal nucleus and the spinal dorsal horn, (3) relay nuclei of the specific sensory systems such as the dorsal lateral geniculate nucleus and the ventral nuclei of the thalamus were devoid of argininosuccinate synthetase-like immunoreactivity, (4) no staining was seen in the large white matter structures such as the internal capsule, corpus callosum, and the anterior commissure, and (5) most of the neurons stained were small or medium in size and appeared to be interneurons. The results suggest that argininosuccinate synthetase affects the widely distributed, neuromodulatory system in the brain.
Animals↗
A case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase.
A male neonate, thus far healthy and fed with breast milk, developed rapidly increasing apathy on the third day of life. Sucking became poor and hyperhidrosis was present. Hyperammonemia (3,305 micrograms/dl) was noted. He became comatose and died on the fourth day. There was a profound derangement of amino acid concentration in the body fluids, with highly elevated citrulline levels (4.70 mumols/ml in serum and 8.47 mumols/ml in urine). Autopsy showed diffuse pulmonary bleeding, as the only noteworthy pathological finding. The liver contained no detectable argininosuccinate synthetase (ASS) activity. The defect of ASS in the present case was not due to a reduced amount of ASSmRNA, but its structure was found to be abnormal; it was approximately 1.57 kb in length due to a defect of about 0.1 kb near the 3' end of the coding region.
Amino Acid Metabolism, Inborn Errors↗
[A senile patient with Rendu-Osler-Weber disease who underwent resection of 4 multiple bilateral pulmonary arteriovenous fistulas].
A 70-year-old female patient with multiple bilateral pulmonary arteriovenous fistulas was referred to our hospital for its treatment. She had experienced frequent epistaxis and had nasopharyngeal telangiectasia. Her mother, sister and all four children also suffered from epistaxis. We diagnosed this patient as Rendu-Osler-Weber disease combined with multiple bilateral pulmonary arteriovenous fistulas. Her chest roentgenogram revealed 4 arteriovenous fistulas in the left lung and 2 arteriovenous fistulas in the right. On admission arterial blood gas analysis while breathing room air showed a PaO2 of 39.8 torr and an SaO2 of 75.4%. The percent of R-L shunt was calculated to be 56.5%. Because of severe hypoxemia, she underwent resection of four large fistulas in segments 3, 4, 5, and 8 of the left lung. Twenty-eight days after the operation, the PaO2 was 76.4 torr and SaO2 was 95.7%. There was no sign of enlargement in size of the remaining arteriovenous fistulas.
Aged↗
Antibody responses and protective immunity in rats receiving repeated inoculations of Strongyloides ratti.
The relationship between specific antibody responses and protective immunity against Strongyloides ratti was examined in rats receiving 10, 50, or 500 infective larvae (L3) at weekly intervals. No specific IgG response was detected in rats receiving 10-L3 inoculations for 7 wk. Fifty- and 500-L3 inoculations induced an IgG response by weeks 2 and 3, respectively, and a higher IgG response was induced in rats receiving the higher doses. All 3 inoculation doses induced high IgE responses, but the kinetics were different. IgE in the 10-L3 group continued to rise from weeks 4 to 7. In the 50- and 500-L3 groups, IgE was detected first at week 3 and increased until week 5. It then declined in the 500-L3 group and the titer at week 7 was significantly lower than that at week 5, whereas it remained the same in the 50-L3 group. The number of larvae recovered from the head 40 hr after a challenge inoculation (1,000 L3) significantly declined by weeks 7, 3, and 2 in rats receiving 10, 50-, and 500-L3 inoculations, respectively. Intestinal worm burdens increased for 7 wk in the 10-L3 group, 5 wk for the 50-L3 group, and 2 wk for the 500-L3 group. These findings indicate that repeated inoculations of low doses of L3 induce delayed and limited protective immunity to a heavy challenge and worm expulsion from the intestine. There was a temporal correlation between the levels of protection and serum IgG, whereas circulating IgE level did not seem to affect directly either the level of the resistance or expulsion of intestinal worms.
Animals↗
[A case of malignant pericardial mesothelioma detected by gallium-67 scintigraphy].
A case of malignant mesothelioma originated from pericardium was reported. A 76 year-old male who complained of dyspnea showed cardiomegaly (CTR = 68%) and bilateral pleural effusion in chest plain X-ray film. Gallium-67 (67Ga) scintigraphy revealed doughnut-like accumulation on cardiac apical site and mediastinal accumulation. Cytological examination of pericardial effusion revealed class V. Autopsy confirmed diagnosis of malignant pericardial mesothelioma. 67Ga scintigraphy may be useful in diagnosis of malignant pericardial mesothelioma.
Aged↗
Clearance of argininosuccinate synthetase from the circulation in acute liver disease.
Argininosuccinate synthetase is an enzyme which has been found to be a specific marker for liver damage. In patients with acute hepatitis, the concentration in serum increases at the onset of the disease, but later decreases more quickly, so that the time required for normalization is shorter than that of alanine aminotransferase. This is probably caused by rapid clearance of argininosuccinate synthetase from the serum. Rapid clearance was demonstrated in experimental animals given purified enzymes intravenously. Argininosuccinate synthetase disappeared from the serum with a half life of about 15 min, while the half lives of alanine aminotransferase and aspartate aminotransferase were 4 and 5 h, respectively, under the same conditions.
Acute Disease↗
[Clinical significance of argininosuccinate synthetase analysis in biochemical laboratory tests].
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Acute Disease↗