• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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• pp.62-67
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• 2018

Hunter syndrome, also known as mucopolysaccharidosis Type II (MPS II), is one of the lysosomal storage diseases caused by a lack of the enzyme iduronate 2-sulfatase (I2S). Lack of the I2S enzyme activity leads to accumulation of the glycosaminoglycans (GAG), causing dysfunction of multiple organs and systems. MPS II is an X-linked recessive disease due to mutation of IDS gene located on long arm of the X chromosome (Xq28). To date, more than 350 mutations of IDS gene have been identified in Hunter syndrome. Phenotypes of MPS II are classified as either severe or attenuated depending on the degree of cognitive impairment. Because the phenotype of MPS II is related to the type of mutation, identifying mutations is useful in predicting prognosis. We recently had a case of MPS II diagnosed by exome sequencing in a 7 month old boy with infantile spasm uncontrolled by AED. He was diagnosed with hearing loss at 2 months of age, and he took vigabatrin and prednisolone to control infantile spasms diagnosed at 3 months of age. At 6 months of age, whole exome sequencing was performed to evaluate the infantile spasm and hearing loss in this patient, and the mutation c.851C>T (p.Pro284Leu) inherited from hemizygous mother was revealed. The results of urine Cetylpyridinium Chloride (CPC) precipitation test, which were negative until 8 months of age, were positive from 9 months of age. We report a case of MPS II diagnosed by exome sequencing and treated through enzyme replacement therapy from 9 months after birth.

• Journal of Mushroom
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• v.19 no.1
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• pp.23-32
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• 2021

This study investigated the anti-hyperlipidemic and anti-obesity effects of Sparassis latifolia (S. latifolia) fruiting body powder in rats fed with a high fat and cholesterol diet (HFD). Rats were fed a normal control diet (ND), an HFD, an HFD supplemented with 5% fruiting body powder of S. latifolia (HFD+SL), or an HFD supplemented with 0.03% simvastatin (HFD+SS), for 6 weeks. The HFD group demonstrated considerable increase in body weight gain, the food efficiency ratio (FER), and plasma cholesterol and triglyceride levels, compared to the ND group. In contrast, the HFD+SL and HFD+SS groups showed significantly reduced body weight gain, food intake, and plasma cholesterol and triglyceride levels compared to the HFD group. In particular, the HFD+SL and HFD+SS diets significantly suppressed the occurrence of non-alcoholic fat deposits in the liver. Taken together, these results suggest that dietary supplementation of the fruiting body powder of S. latifolia in an HFD could lower the risks of hyperlipidemia, atherogenesis, and obesity and may be used as a functional food to manage cardiovascular disease and fecal lipid and cholesterol levels.

• Journal of Mushroom
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• v.18 no.4
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• pp.357-364
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• 2020

The grade and price of Lentinula edodes largely differs in preference depending on the product area and seasonal factors. The product amount of autumn L. edodes was higher than that of spring L. edodes, but high quality, which is divided into "Hwago" is low in preference. Mostly, the autumn L. edodes is obtained as powder; hence, it is necessary to develop a processing method to utilize its flavor and aroma at an affordable price. Additionally, we estimated the content of 𝛽-glucan, ergosterol, vitamin D₂, reducing sugars, and free amino acids and evaluated the anti-inflammatory activity of saccharification powder of log-cultivated L. edodes. In the saccharification powders obtained via 7 min of UV irradiation of log-cultivated L. edodes, 𝛽-glucan and vitamin D₂ contents were found to be the highest, whereas ergosterol content was found to be the lowest. The content of reducing sugars ranged from 62.4 mg/L to 68.2 mg/L. The free amino acids were higher in these saccharification powders than in the control. Subsequently, RAW 264.7 cells were treated with different concentrations (10, 50, 100, 200, 300, and 500 ㎍/mL) of the saccharification powders of log-cultivated L. edodes obtained via different UV irradiation time applications. The cells showed good viability; the anti-inflammatory effect was found to be the highest at 7 min UV irradiation. Therefore, 7 min of UV irradiation was determined to be the optimum condition for manufacturing saccharification powders of log-cultivated L. edodes. Hence, saccharification powders of log-cultivated L. edodes may be used as a raw material for natural sweeteners, food additives, and in the food industry.

• Radiation Oncology Journal
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• v.24 no.4
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• pp.230-236
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• 2006

$\underline{Purpose}$: To analyze the response, toxicity, patterns of failure and survival rate of patients with locally advanced non-small cell lung cancer who were treated with concurrent chemoradiotherapy with weekly paclitaxel. $\underline{Materials\;and\;Methods}$: Twenty-three patients with locally advanced non-small cell lung cancer patients who received radical chemoradiotherapy from October 1999 to September 2004 were included in this retrospective study. Patients received total $55.4{\sim}64.8$ (median 64.8) Gy (daily 1.8 Gy per fraction, 5 days per weeks) over $7{\sim}8$ weeks. 50 or $60\;mg/m^2$ of paclitaxel was administered on day 1, 8, 15, 22, 29 and 36 of radiotherapy. Four weeks after the concurrent chemoradiotherapy, three cycles of consolidation chemotherapy consisted of paclitaxel $135\;mg/m^2$ and cisplatin $75\;mg/m^2$ was administered every 3 weeks. $\underline{Results}$: Of the 23 patients, 3 patients refused to receive the treatment during the concurrent chemoradiotherapy. One patient died of bacterial pneumonia during the concurrent chemoradiotherapy. Grade 2 radiation esophagitis was observed in 4 patients (17%). Sixteen patients received consolidation chemotherapy. During the consolidation chemotherapy, 8 patients (50%) experienced grade 3 or 4 neutropenia and one of those patients died of neutropenic sepsis. Overall response rate for 20 evaluable patients was 90% including 4 complete responses (20%) and 14 partial responses (70%). Among 18 responders, 9 had local failure, 3 had local and distant failure and 2 had distant failure only. Median progression-free survival time was 9.5 months and 2-year progression-free survival rate was 19%. Eleven patients received second-line or third-line chemotherapy after the treatment failure. The median overall survival time was 21 months. 2-year and 5-year survival rate were 43% and 33%, respectively. Age, performance status, tumor size were significant prognostic factors for progression-free survival. $\underline{Conclusion}$: Concurrent chemoradiotherapy with weekly paclitaxel revealed high response rate and low toxicity rate. But local failure occurred frequently after the remission and large tumor size was a poor prognostic factor. Further investigations are needed to improve the local control.

• The Korean Nurse
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• v.29 no.2
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• pp.48-65
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• 1990

The government has adopted a policy to introduce Home Health Care Program, and has established a three stage plan to implement it. The three stage plan is : First, to amend Article 54 (Nurses for Different Types of Services) of the Regulations for Implementing the Law of Medical Services; Second, to tryout the new system through pilot projects established in public hospitals and clinics; and third, to implement at all hospitals and equivalent medical institutions. In accordance with the plan, the Regulation has been amend and it was promulgated on January 9,1990, thus establishing a legal ground for implementing the policy. Subsequently, however, the Medical Association raised its objection to the policy, causing a delay in moving into the second stage of the plan. Under these circumstances, a study was conducted by collecting and evaluating the opinions of physicians, nurses, non-medical personnel and patients on the need and expected result from the home health care for the purpose of help facilitating the implementation of the new system. As a result of this study, it was revealed that: 1. Except the physicians, absolute majority of all other three groups - nurses, non-medical personnel and patients -gave positive answers to all 11 items related to the need for establishing a program for Home Health Care. Among the physicians, the opinions on the need for the new services were different depending on their field of specialty, and those who have been treating long term patients were more positive in supporting the new system. 2. The respondents in all four groups held very positive view for the effectiveness and the expected result of the program. The composite total of scores for all of 17 items, however, re-veals that the physicians were least positive for the- effectiveness of the new system. The people in all four groups held high expectation on the system on the ground that: it will help continued medical care after the discharge from hospitals; that it will alleviate physical and economic burden of patient's family; that it will offer nursing services at home for the patients who are suffering from chronic disease, for those early discharge from hospital, or those who are without family members to look after the patients at home. 3. Opinions were different between patients( who will receive services) and nurses (who will provide services) on the types of services home visiting nurses should offer. The patients wanted "education on how to take care patients at home", "making arrangement to be admitted into hospital when need arises", "IV injection", "checking blood pressure", and "administering medications." On the other hand, nurses believed that they can offer all 16 types of services except "Controlling pain of patients", 4. For the question of "what types of patients are suitable for Home Health Care Program; " the physicians, the nurses and non-medical personnel all gave high score on the cases of "patients of chronic disease", "patients of old age", "terminal cases", and the "patients who require long-term stay in hospital". 5. On the question of who should control Home Health Care Program, only physicians proposed that it should be done through hospitals, while remaining three groups recommended that it should be done through public institutions such as public health center. 6. On the question of home health care fee, the respondents in all four groups believed that the most desireable way is to charge a fixed amount of visiting fee plus treatment service fee and cost of material. 7. In the case when the Home Health Care Program is to be operated through hospitals, it is recommended that a new section be created in the out-patient department for an exclusive handling of the services, instead of assigning it to an existing section. 8. For the qualification of the nurses for-home visiting, the majority of respondents recommended that they should be "registered nurses who have had clinical experiences and who have attended training courses for home health care". 9. On the question of if the program should be implemented; 74.0% of physicians, 87.5% of non-medical personnel, and 93.0% of nurses surveyed expressed positive support. 10. Among the respondents, 74.5% of -physicians, 81.3% of non-medical personnel and 90.9% of nurses said that they would refer patients' to home health care. 11. To the question addressed to patients if they would take advantage of home health care; 82.7% said they would if the fee is applicable to the Health Insurance, and 86.9% said they would follow advises of physicians in case they were decided for early discharge from hospitals. 12. While 93.5% of nurses surveyed had heard about the Home Health Care Program, only 38.6% of physicians surveyed, 50.9% of non-medical personnel, and 35.7% of patients surveyed had heard about the program. In view of above findings, the following measures are deemed prerequisite for an effective implementation of Home Health Care Program. 1. The fee for home health care to be included in the public health insurance. 2. Clearly define the types and scope of services to be offered in the Home Health Care Program. 3. Develop special programs for training nurses who will be assigned to the Home Health Care Program. 4. Train those nurses by consigning them at hospitals and educational institutions. 5. Government conducts publicity campaign toward the public and the hospitals so that the hospitals support the program and patients take advantage of them. 6. Systematic and effective publicity and educational programs for home heath care must be developed and exercises for the people of medical professions in hospitals as well as patients and their families. 7. Establish and operate pilot projects for home health care, to evaluate and refine their programs.

• Tuberculosis and Respiratory Diseases
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• v.44 no.2
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• pp.379-390
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• 1997

Background : It has long been suggested that neutrophils and their products are implicated as the central mediators of the acute lung injuries. Contrary to the dominant role of neutrophils in ARDS, many cases of ARDS has occurred in the setting of severe neutropenia without pulmonary neutrophil infiltration. Therefore it is certain that effector cell(s) other than neutrophil play an important role in the pathogenesis of ARDS. This experiment was performed to define the mechanism of ARDS in the setting of neutropenia, 1) by comparing the severity of endotoxin-induced lung injury, 2) by measurement of hydrogen peroxide production and cytokine concentration in the bronchoalveolar lavage cells and fluids obtained from different rats with and without cyclophosphamide-pretreatment. Method : The male Sprague-Dawleys were divided into the normal control (NC)-, endotoxin (ETX)-, and cyclophosphamide (CPA)-group in which neutropenia was induced by injecting cyclophosphamide intraperitoneally. Acute lung injury was evoked by injecting lipopolysaccharide (LPS) into a tail vein. The bronchoalveolar lavage (BAL) was performed at 3 and 6 hour after administration of LPS to measure the change of cell counts and concentrations of protein and cytokines, tumor necrosis factor-alpha (TNF-alpha) and interleukin-6 (IL-6). Hydrogen peroxide (HPO) production from BAL cells was measured at 6 hour after LPS administration by phenol red microassay with and without zymosan stimulation. Results : The results were as follows. A change of leukocyte counts in the peripheral blood after treatment with CPA : More than 95% of total leukocytes and neutrophils were reduced after CPA administration, resulting in severe neutropenia. A change of BAL cells : In the ETX-group, the number of total cells (p < 0.01) and of macrophage and neutrophil (p < 0.05) were increased at 3 and 6 hour after LPS administration compared to those of NC-group. In the CPA-group, the number of total leukocyte and macrophage were not changed after LPS administration, but neutrophil counts were significantly reduced and it took part in less than 0.1% of total BAL cells (p < 0.01 vs NC-group). BAL cells in this group were almost all macrophages (99.7%). A change of protein concentration in the BALF : In the ETX-group, protein concentration was increased at 3 hour and was more increased at 6 hour after LPS administration (p < 0.05 and < 0.01 vs NC-group, respectively). In the CPA-group, it was also significantly elevated at 3 hour after LPS administration (p < 0.05 vs NC-group), but the value was statistically not different from that of ETX-group. The value measured at 6 hour after LPS administration in the CPA-group became lower than that of ETX-group (p < 0.05), but showed still a higher value compared to that of NC-group (p < 0.05). A change of cytokine concentration in the BALF : TNF -alpha and IL-6 were elevated in the ETX - and CPA-group compared to those of NC-group at both time intervals. There was no statistical difference in the values of both cytokines between the ETX- and CPA-groups. Measurement of hydrogen peroxide production from BAL cells : There was no intergroup difference of HPO production from resting cells. HPO production after incubation with opsonized zymosan was significantly elevated in all groups. The percent increment of HPO production was highest in the ETX-group (89.0%, p < 0.0008 vs NC-group), and was 42.85 in the CPA-group (p = 0.003 vs NC-group ). Conclusion : Acute lung injury in the setting of neutropenia might be caused by functional activation of resident alveolar macrophages.

• Asia Marketing Journal
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• v.13 no.4
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• pp.55-76
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• 2012

Most people prefer to choose zero-cost products they may get without paying any money. The 'zero-cost effect' can be explained with a 'zero-cost model' where consumers attach special values to zero-cost products in a different way from general economic models (Shampanier, Mazar and Ariely 2007). If 2 different products at the regular prices of ￦200 and ￦400 simultaneously offer ￦200 discounts, the prices will be changed to ￦0 and ￦200, respectively. In spite of the same price gap of the two products after the ￦200 discounts, people are much more likely to select the free alternative than the same product at the price of ￦200. Although prior studies have focused on the 'zero-cost effect' in isolation of other factors, this study investigates the moderating effects of a self-regulatory resource and a construal level on the selection of free products. Self-regulatory resources induce people to control or regulate their behavior. However, since self-regulatory resources are limited, they are to be easily depleted when exerted (Muraven, Tice, and Baumeister 1998). Without the resources, consumers tend to become less sensitive to price changes and to spend money more extravagantly (Vohs and Faber 2007). Under this condition, they are also likely to invest less effort on their information processing and to make more intuitive decisions (Pocheptsova, Amir, Dhar, and Baumeister 2009). Therefore, context effects such as price changes and zero cost effects are less likely in the circumstances of resource depletion. In addition, construal levels have profound effects on the ways of information processing (Trope and Liberman 2003, 2010). In a high construal level, people tend to attune their minds to core features and desirability aspects, whereas, in a low construal level, they are more likely to process information based on secondary features and feasibility aspects (Khan, Zhu, and Kalra 2010). A perceived value of a product is more related to desirability whereas a zero cost or a price level is more associated with feasibility. Thus, context effects or reliance on feasibility (for instance, the zero cost effect) will be diminished in a high level construal while those effects may remain in a low level construal. When people make decisions, these 2 factors can influence the magnitude of the 'zero-cost effect'. This study ran two experiments to investigate the effects of self-regulatory resources and construal levels on the selection of a free product. Kisses and Ferrero-Rocher, which were adopted in the prior study (Shampanier et al. 2007) were also used as alternatives in Experiments 1 and 2. We designed Experiment 1 in order to test whether self-regulatory resource depletion will moderate the zero-cost effect. The level of self-regulatory resources was manipulated with two different tasks, a Sudoku task in the depletion condition and a task of drawing diagrams in the non-depletion condition. Upon completion of the manipulation task, subjects were randomly assigned to one of a decision set with a zero-cost option (i.e., Kisses ￦0, and Ferrero-Rocher ￦200) or a set without a zero-cost option (i.e., Kisses ￦200, and Ferrero-Rocher ￦400). A pair of alternatives in the two decision sets have the same price gap of ￦200 between a low-priced Kisses and a high-priced Ferrero-Rocher. Subjects in the no-depletion condition selected Kisses more often (71.88%) over Ferrero-Rocher when Kisses was free than when it was priced at ￦200 (34.88%). However, the zero-cost effect disappeared when people do not have self-regulatory resources. Experiment 2 was conducted to investigate whether constual levels influence the magnitude of the 'zero-cost effect'. To manipulate construal levels, 4 different 'why (in the high construal level condition)' or 'how (in the low construal level condition)' questions about health management were asked. They were presented with 4 boxes connected with downward arrows. In a box at the top, there was one question, 'Why do I maintain good physical health?' or 'How do I maintain good physical health?' Subjects inserted a response to the question of why or how they would maintain good physical health. Similar tasks were repeated for the 2nd, 3rd, and 4th responses. After the manipulation task, subjects were randomly assigned either to a decision set with a zero-cost option, or to a set without it, as in Experiment 1. When a low construal level is primed with 'how', subjects chose free Kisses (60.66%) more often over Ferrero-Rocher than they chose ￦200 Kisses (42.19%) over ￦400 FerreroRocher. On contrast, the zero-cost effect could not be observed any longer when a high construal level is primed with 'why'.

• Tuberculosis and Respiratory Diseases
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• v.44 no.2
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• pp.360-378
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• 1997

Background : Severe acute lung injury(ALI), also known as the adult respiratory distress syndrome(ARDS), is a heterogenous nature of dynamic and explosive clinical synrome that exacts a mortality of approximately 50%. Endotoxin(ETX) is an abundant component of the outer membrane of gram-negative bacteria capable of inducing severe lung injury in gram-negative sepsis and gram-negative bacterial pneumonia, which are among the most common predisposing causes of ARDS. The influx of PMNs into airway tissue is a pathological hallmark of LPS-induced lung injury. And there is a substantial evidence suggesting that cytokines are important mediators of lung injury in gram-negative sepsis. However, the kinetics of phagocytes and cytokines by an exact time sequence and their respective pathogenic importance remain to be elucidated. This study was performed to investigate the role of phagocytes and proinflammatory cytokines in ETX-induced ALI through a time course of changes in the concentration of protein, $TNF{\alpha}$ and IL-6, and counts of total and its differential cells in BALF. The consecutive histologic findings were also evaluated. Method : The experimental animals, healthy male Sprague-Dawley, weighted $200{\pm}50g$, were divided into control- and ALI- group. ALI was induced by an intravenous administration of ETX, 5mg/kg. Above mentioned all parameters were examined at 0(control), 3, 6, 24, 72 h after administration of ETX. $TNF{\alpha}$ and IL-6 cone. in BALF were measured by a bioassay. Results : The protein concentration and total leukocyte count(TC) in BALF was significantly increased at 3h compared to controls(p < 0.05). The protein conc. was significantly elavated during observation period, but TC was significantly decreased at 72h(p < 0.05 vs. 24h). There was a close relationship between TC and protein cone. in BALF(r = 0.65, p < 0.001). The PMN and monocyte count was well correlated with TC in BALF, and the correlation of PMN(r = 0.97, p < 0.001) appeared to be more meaningful than that of monocyte(r = 0.61, p < 0.001). There was also a significant correlation between protein cone. and PMN or monocyte count in BALF(PMN vs. monocyte : r = 0.55, p < 0.005 vs. r = 0.64, p < 0.001). The count of monocyte was significantly elavated during observation period though a meaningful reduction of PMN count in BALF at 72h, this observation suggested that monocyte may, at least, partipate in the process of lung injury steadly. In this study, there was no relationship between IL-6 and $TNF{\alpha}$ cone., and $TNF{\alpha}$ but not IL-6 was correlated with TC(r = 0.61, p < 0.05) and monocyte(r = 0.67, p < 0.05) in BALF only at 3, 6h after ETX introduced. In particular, the IL-6 cone. increased earlier and rapidly peaked than $TNF{\alpha}$ cone. in BALF. In histologic findings, the cell counts of lung slices were increased from 3 to 72h(p < 0.001 vs. NC). Alveolar wall-thickness was increased from 6 to 24h(p < 0.001 vs. NC). There was a significant correlation between the cell counts of lung slices and alveolar wall-thickness(r= 0.61, p < 0.001). This result suggested that the cellular infiltrations might be followed by the alterations of interstitium, and the edematous change of alveolar wall might be most rapidly recovered to its normal condition in the process of repair. Conclusion : We concluded that although the role of PMN is partly certain in ETX-induced ALI, it is somewhat inadequate to its known major impact on ALL Alveolar macrophage and/or non-immune cells such as pulmonary endothelial or epithelial cells, may be more importantly contributed to the initiation and perpetual progression of ETX-induced ALI. The IL-6 in ETX-induced ALI was independent to $TNF{\alpha}$, measured by a bioassay in BALF. The early rise in IL-6 in BALF implies multiple origins of the IL-6.