The aim of this study was to clarify the role of Kupffer cells in the mechanism of endotoxin-induced liver injury. The study on fine structure of Kupffer cells was performed after the injection of endotoxin. The endotoxin(Escherichia coli lipopolysaccharide 026 : B6. 1.5mg/100 g of body weight) was intraperitoneally injected in Sprague-Dewley rats. Animals were sacrificed at 1/4, 1/2, 1, 2, 4, 8, 16, 24, 72 and 120 hours after the injection of endotoxin. Livers were extirpated and processed to be examined by light and electron microscopy. The results obtained were summerized as follows: Early changes observed in liver after endotoxin injection included the increased number and hypertrophy of Kupffer cells, infiltration of neutrophils and presence of fibrin thrombi within the sinusoids. The continuous increase of the Kupffer cells in number with hypertrophy, congestion and infiltration of inflammatory cells within the sinusoids were observed. Hepatocytes showed fatty change and occasional necrosis. At 72 hours the congestion decreased. At 120 hours the number of Kupffer cells was increased, but the morphology of Kupffer cells became similar to that of the control group. The numbers and sizes of primary and secondary lysosomes and amount of euchromatin of Kupffer cells increased. Swellings and increase in number of mitochondria, Golgi complex, smooth endoplasmic reticulum, rough endoplasmic reticulum were evident. Microthrombi were present within the sinusoids. The swelling of rough endoplasmic reticulum and mitochondria, decrease of glycogen particles, fatty change, hypoxic vacuoles, pyknotic nuclei and occasional necrosis were observed in hepatocytes. At 72 hours the number of secondary lysosomes in Kupffer cells decreased. At 120 hours the morphology of Kupffer cells became similar to that of the control group. According to these results, it was postulated that the endotoxin was initially taken up by pinocytosis into Kupffer cells and degraded in secondary lysosomes of activated Kupffer cells. Kupffer cells may play an important role in the defense mechanism of liver during endotoxemia. The dysfunction of Kupffer cells and ischemia by sinusoidal microthrombi may cause liver injury.
Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.
Journal of The Korean Society of Inherited Metabolic disease
/
v.23
no.1
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pp.25-30
/
2023
Leigh syndrome is a rare progressive neurodegenerative mitochondrial disorder with clinical and genetic heterogeneity. Recently, balletic IARS2 variants have been identified in a number of patients presenting broad clinical phenotypes from Leigh and West syndrome to a rare syndrome CAGSSS characterized by cataracts, growth hormone deficiency, sensory neuropathy, sensorineural hearing loss, and skeletal dysplasia syndrome (OMIM#616007). We describe a child with Korean Leigh syndrome with urologic manifestations resulting from a compound heterozygote mutation in IARS2. A 5-year-old girl visited the emergency room with a complaint of abdominal pain accompanied by abdominal distension. Abdominal-pelvic CT showed a markedly distended urinary bladder without definite obstructive lesions. She was diagnosed with neurogenic bladder dysfunction based on a urodynamic study. She had global delayed development due to neurologic regression after 6 months of age and a history of bilateral cataract surgery at the age of 2 years. Her brain magnetic resonance imaging showed symmetrically increased signal intensities in the bilateral putamen and caudate nuclei with diffuse cerebral atrophy. No gene variants were identified through whole-mitochondrial genome analysis. Whole exome sequencing was performed for diagnosis, and compound heterozygous pathogenic variants were identified in IARS2: c.2446C>T (p. Arg816Ter) and c.2450G>A (p. Arg817His). To the best of our knowledge, this is the first case report of bladder dysfunction manifestation in a patient with IARS2-related Leigh syndrome. Thus, it broadens the clinical and genetic spectrum of IARS2-associated diseases.
In this study, we evaluated the changes of fruit quality indices during fruit development and ripening in Korean new pear cultivar 'Changjo', developed from a cross between 'Tama' and '81-1-27' ('Danbae' ${\times}$ 'Okusankichi') in 1995 and named in 2009, to determine appropriate harvest time and to enhance the market quality and broaden the cultivation area. The fruits of 'Changjo' pears harvested from 132 days after full bloom (DAFB) to 160 DAFB. Fruit growth and quality indices were monitored at 1 week interval by measuring fruit weight, length, diameter, firmness, and taste related quality indices. The calculated fruit fresh weight increased continuously with fruit development and reached to an average of 594g on Sep. 20 (160 DAFB). The ratio of length to diameter declines as fruit maturation progress, resulting in 0.898 for ripe fruit stage as a round oblate shape. Flesh firmness of 'Changjo' pears showed over 30N until 153 DAFB and then decreased abruptly with fruit ripening, reaching a final level of about 26.44N on 160 DAFB. Starch content of fruit sap was also decreased abruptly after 146 DAFB which decreased almost half of the fruits harvested at 139 DAFB. In parallel with the decrease of flesh firmness, ethanol insoluble solids (EIS) content decreased sharply with fruit ripens, only 50% of EIS was detected on the fruits harvested on 160 DAFB when compared to that of the fruits harvested on 139 DAFB (Aug. 30). The maximum value of soluble solids contents was observed in the fruits harvested on 153 DAFB, resulting in $14.2^{\circ}Brix$. The changes of skin color difference $a^*$ which means loss of green color occurred only after 139 DAFB, coincide with the decrease of SPAD value of the fruit skin. The sugars of the 80% ethanol soluble fraction consisted mainly of fructose, sorbitol, glucose and sucrose, also increased during maturation and ripening. Fructose and sucrose contents were larger than those of glucose and sorbitol in flesh tissues. These results were explained that stored starch is converted to soluble sugars during fruit maturation, mainly in fructose and sucrose increasing the sweetness of this cultivar. Total polyphenols were increased up to middle of fruit maturation (146 DAFB) and then decreased continuously until the end of fruit maturation. Consequently, our results suggested that the commercial harvest time of 'Changjo' pears should not be passed 153 DAFB and late harvest of this cultivar would not good for quality maintenance during shelf-life. As a result of the post-harvest low-temperature acclimation experiment during the short-term transportation period, fruits harvested at 146 DAFB tended to maintain higher firmness after 14 days of simulated marketing at $25^{\circ}C$ compared to fruits harvested at 153 DAFB regardless of temperature set. And, the slower the rate of decrease to the final transport temperature of $5^{\circ}C$, the higher the incidence of internal browning and ethylene production. Therefore, in order to suppress the physiological disorder and to maintain the fruit quality when exporting to Southeast Asia in the 'Chanjo' pears, it is desirable to lower the temperature of the fruits within a short time after harvest and to set the harvest time before 146 days after full bloom.
Noise and air pollution, which accompany the development of industry and the increase of population, contribute to the deterioration of urban environment. The air pollution level of Seoul has gradually increased and the city residents are suffering from a high pollution of noise. If no measures were taken against pollution, the amount of emission of pollutant into air would be 36.7 thousand tons per year per square kilometer in 1975, three times more than that of 1970, and it would be the same level as that of United States in 1968. The main sources of air pollution in Seoul are the exhaust has from vehicles and the combustion of bunker-C oil for heating purpose. Thus, it is urgent that an exhaust gas cleaner should be instaled to every car and the fuel substituted by less sulfur-contained-oil to prevent the pollution. Transportation noise (vehicular noise and train noise) is the main component of urban noise problem. The average noise level in downtown area is about 75㏈ with maximum of 85㏈ and the vehicular homing was checked 100㏈ up and down. Therefore, the reduction of the number of bus-stop the strict regulation of homing in downtown area and a better maintenance of car should be an effective measures against noise pollution in urban areas. Within the distance of 200 metres from railroad, the train noise exceeds the limit specified by the pollution control law in Korea. Especially, the level of noise and steam-whistle of train as measured by the ISO evaluation can adversely affect the community activities of residents. To prevent environmental destruction, many developed countries have taken more positive action against worsening pollution and such an action is now urgently required in this country.
Flacherie, as one of the most prevalent silkworm diseases, causes severe economic damage to sericultural industry and its pathogens have been proved to be flacherie virus (FV) and densonucleosis virus (DNV). Multiplications of the viruses in the larvae of the silkworm, Bombyx mori, were studied by the sucrose density gradient centrifugation and electron microscopy. The quantitative and qualitative changes of nucleic acids and proteins were investigated from the midgut and hemolymph in the silkworm larvae infected separately with FV and DNV. The histopathological changes of epithelial cells of infected midgut also were examined by an electron microscope. 1. Purified fractions of FV or DNV in a sucrose density gradient centrifugation yielded one homogenous and sharp peak without a shoulder, suggesting no heterogenous materials in the preparation. Electron microscopy also revealed that FV and DNV were spherical particles, 27nm and 21nm in diameter, respectively. 2. Silkworm larvae showed a decrease in body weight on the 6th day and in midgut weight on the 3rd day after inoculation with FV or DNV. 3. DNA content was higher in the midgut when infected with FV or DNV, but the hemolymph of the infected larvae showed no difference during first 6 days after inoculation, after which DNA concentration declined rapidly. 4. RNA synthesis of silkworm larvae infected separately with FV and DNV was stimulated in the midgut, but RNA content was reduced in the hemolymph at the early stage of virus multiplication. At the late stage of virus multiplication, however, it was extremely reduced in both midgut and hemolymph. 5. The concentration of protein in the midgut and hemolymph of silkworm larvae infected separately with FV and DNV showed no difference from that of the healthy larvae at the early stage of virus multiplication, but it was significantly reduced at the late stage of virus multiplication. 6. There was no difference in the electrophoretic patterns of RNAs extracted from the midgut of healthy or virus-infected larvae. 7. The electrophoresis of proteins extracted from the midgut infected with FV or DNV, when carried out on the 1st and 5th day after virus inoculation, showed no difference from that of the healthy larvae. But, there was an additional band with medium motility in the proteins on the 8th day after virus inoculation, while a band with low mobility shown in the proteins of healthy larvae disappeared in the infected larvae. However, a band with high mobility in the healthy larvae was separated into two fractions in the infected larvae. 8. The electrophoretic pattern of hemolymph proteins of the silkworm larvae infected separately with FV and DNV was similar to that of the healthy larvae, but the concentration of hemolymph proteins in the infected larvae was lower than that of the healthy larvae at the late stage. 9. Two types of inclusion bodies were shown by the double staining of pyronin-methyl green in the columnar cell of the midgut on the 8th day after FV inoculation. 10. Electron microscopy of the infected midgut revealed that the 'cytoplasmic wall' of the goblet cell thickened on the 5th day after FV inoculation and several types of the cytopathogenic structures, such as virus$.$specific vesicles, virus particles, linear structures, tubular structures, and high electron-dense matrices were observed in the cytoplasm of the goblet cell. The virus particles were also observed in the microvilli and the structures similar to spherical virus particles were observed around the virus-specific vesicles, suggesting the virus assembly in the cytoplasm. 11. Fluorescence micrograph of the infected midgut stained with acridine orange showed that the nucleus, the site of DNV multiplication in the columnar cell, enlarged on the 5th day after virus inoculation. 12. Electron microscopic examination of DNV infected midgut revealed that the nucleolus of the columnar cell was broken into granules and those granules dispersed into apical region of the nucleus on the 5th day after virus inoculation. On the 8th day after inoculation, it was also observed that the nucleus of the columnar cell was full with the high electron-dense virogenic stroma which were similar to virus particles. These facts suggest that the virogenic stroma were the sites of virus assembly in the process of DNV multiplication.
Journal of the Korean Society of Food Science and Nutrition
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v.39
no.11
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pp.1595-1603
/
2010
The present study was conducted to investigate the effect of dietary grape pomace on lipid metabolism and hepatic morphology of rats fed a high fat diet. The high fat diet contained additional 15% lard to AIN 93-based diet. Male Sprague-Dawley rats were fed experimental diets containing 5% grape pomace for 4 weeks. Serum activities of glutamic-oxaloacetic transaminase (GOT) and glutamic-pyruvic transaminase (GPT) were not changed by high fat and grape pomace feeding. Serum concentration of triglyceride in rats fed a high fat diet was decreased significantly by dietary grape pomace. Hepatic concentrations of total lipid, total cholesterol and triglyceride were reduced in grape pomace groups with a high fat diet. Fecal concentrations of total cholesterol and triglyceride were increased in grape pomace groups with a high fat diet. The fecal content of coprostanol was not different among the groups. Dietary grape pomace increased the fecal excretion of cholesterol and coprostanone in rats fed a high fat diet. The fecal excretion of bile acid was not affected by feeding grape pomace in rats fed a high fat diet. Light micrographs of liver tissue revealed lipid droplets were increased by a high fat diet, but dietary supplementation of grape pomace tended to alleviate such changes.
Background : Bronchial anthracofibrosis (BAF) is a dark black or brown pigmentation of multiple large bronchi associated with a fibrotic stenosis or obliteration that is incidentally found during a diagnostic bronchoscopy some reporters have suggested endobronchial tuberculosis or tuberculous lymphadenitis as a possible cause of BAF. However, some BAF patients do not have any medical history of tuberculosis. The aim of this study was to elucidate the clinical features of simple BAF patients, which were not associated with tuberculosis. Methods : We reviewed the patients' charts retrospectiely and interviewed all BAF patients who were followed up for 1 year or more. Among the 114 BAF patients, 43 patents (38 %) had no associated tuberculosis, cancer and pneumoconiosis. The clinical characteristics, radiological findings and associated pulmonary diseases of these patients were evaluated. Results : Most patients were non-smokers, old aged, housewifes who resided in a farming village. The common respiratory symptoms were dyspnea, cough and hemoptysis. The predominant X-ray findings were a multiple bronchial wall thickening(89%), bronchial narrowing or atelectasis (76%) and a mediastinal lymph node enlargement with/without calcification (78%). Pulmonary function test usually showed mild obstructive ventilatory abnormalities but no patient showed a restrictive ventilatory pattern and the patients were frequently affected with chronic bronchitis(51%), post-obstructive pneumonia(40%) and chronic asthma(4%). Conclusion : Because BAF is frequently associated with chronic bronchitis and obstructive pneumonia as well as tuberculosis, a careful clinical evaluation and accurate differential diagnosis is more essential than empirical anti-tuberculous medication.
This study was carried out to investigate characteristics of new garlic cultivar 'Alkae' and to identify the effect of scape removal on bulb characteristics and growth of garlic cultivar 'Alkae' and 'Namdo'. Bolting time and harvesting time were earlier about 20 days in 'Alkae' than those in 'Namdo'. 'Alkae' was inferior to 'Namdo' in top growth as plant height, number of leaves, leaf area, SPAD value, diameter of leaf sheath, and so on. but root developing was better in 'Alkae'. Bulb weight of' Alkae' was 17% more than that of 'Namdo', harvested on proper time. The ratio of bulb weight over 40 g was almost double in 'Namdo' as 48% with 'Alkae' as 25%. Occurrence of bulb cracking was more considerable in 'Alkae' and the number of clove per bulb was 1.9 more in 'Alkae' than 'Namdo', but the ratio of bulb rot during storage was higher in 'Namdo' than 'Alkae'. In treatments of scape removal, SPAD value did not show any difference between two cultivars. Leaf dryness was earlier and harder than control and leaf area showed the same tendency as leaf dryness, and there were no significant differences among the methods of scape removal. The bulb weight and the ratio of bulb weight over 40 g at 50 days after the harvest of 'Alkae', 'Namdo' were more 10%, 12% and 27%, 44% in scape removal treatment than control. and there were no significant differences among the methods of scape removal. So, scape removal was very effective to increase large bulb production in the two garlic cultivals and the effect was higher in 'Namdo' than 'Alkae'. The ratio of bulb cracking and the occurrence of bulb rot were almost same in with the scape removal and non removal groups And also there were no significant differences among the methods of scape removal.
Park, Shin-I;Kwon, Hae Oak;Lee, Jun Ho;Jung, Su Jin
Clinical and Experimental Pediatrics
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v.48
no.10
/
pp.1121-1125
/
2005
Purpose : Rotavirus is the main cause of infantile diarrheal disease worldwide, especially in patients 3-24 months of age. Infants younger than 3 months of age are relatively protected by transplacental antibody. So the purpose of this study is to evaluate the clinical features and severity of neonatal rotaviral gastroenteritis less than 1 month of age. Methods : A retrospective chart review was established of 62 neonates less than 1 month of age and with a diagnosis of rotaviral gastroenteritis who had been admitted to Pochon CHA University between June 2002 through July 2004. The rotavirus was examined by stool latex agglutination. Results : During 2 years, the total number of admitted patients for rotaviral gastroenteritis was 688 and among these, less than 1 month of age accounted for 9%(62). The occurrence was generally even distribution from January to July($7.14{\pm}1.0$) but since then decreased($2.4{\pm}1.8$). The most common chief complaint was mild fever(46%) when admitted which subsided within 1 hospital day in most patients. 4 patients had seizure and cyanosis with no typical symptoms of rotaviral gastroenteritis. During admission, all the patients had diarrhea. 17% of the patients had leukocytosis and positive C-reactive protein. In one patient, stool occult blood test was positive but there was no necrotizing gastroenteritis evidence. The mean period of hospital day was $5.8{\pm}2.5$ and breast-milk feeding was 62.9%. Conclusion : Neonatal rotaviral gastroenteritis is not a rare disease. Most patients have fever and diarrhea and improve through conservative therapy but a few patients may have severe complications so we must be more cautious about the hygiene for prevention.
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