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Chronic Granulomatous Disease on Jeju Island, Korea

  • Cho, Moonjae;Shin, Kyung-Sue
    • Journal of Genetic Medicine
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    • v.10 no.1
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    • pp.1-6
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    • 2013
  • Chronic granulomatous disease (CGD) is a rare inherited disorder of a defective NADPH oxidase enzyme, resulting in very low or no production of superoxide and subsequent reactive oxygen species. Consequently, patients with CGD are highly susceptible to severe bacterial and fungal infections. CGD is a genetically heterogeneous disease caused by defects in any one of the genes encoding the NADPH oxidase components. CGD generally affects about 3-4 per 1,000,000 individuals; thus, it is surprising that the prevalence of CGD on Jeju Island is 34.3 per 1,000,000 individuals. At present, 20 patients with CGD from 14 unrelated families on Jeju Island have been identified; nine males and 11 females. All patients with CGD tested on Jeju Island had an identical and homozygous mutation (c.7C>T in CYBA, p.Q3X in $p22^{phox}$). Therefore, all patients were autosomal recessive form of CGD. This strongly suggests that the unique and identical mutation in CYBA may be inherited from a common proband. Using mutation-specific primers to detect the mutated allele in CYBA, the frequency of subjects carrying a mutated allele was 1.3% of enrolled subjects from Seogwipo City. Further studies are necessary to elucidate how frequently this mutant allele occurs in the population on Jeju Island. Additionally, it is important to construct a national registry system to understand the pathophysiology of CGD and develop a strategy for long-term therapy.

Analysis of Nursing Interventions and Nursing Outcomes of the Patients with Liver Transplantation (간호중재와 간호결과 분석- 간이식 환자를 중심으로 -)

  • Yoo, Je-Bog;Jang, Hee-Jung;Kim, Nam-Cho
    • Korean Journal of Adult Nursing
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    • v.15 no.4
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    • pp.509-519
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    • 2003
  • Purpose: This study was designed to investigate the survey of analyzing nursing interventions and nursing outcomes of the patients with liver transplantation. Method: The subjects of this study consisted of 83 patients and fifteen nurses who took care of them. The methods of this study were retrospective and descriptive survey. Result: The mean age of patients was 42.7 years and the subjects were 62.7% males. 56.7% of these patients were diagnosed with liver cirrhosis of B type. The mean duration of hospitalization was 48.6 days. Ninety-five nursing interventions were performed at least daily. The most frequent used interventions were "environmental management: comfort", "medication administration: parenteral", "cough enhancement", and " oxygen therapy". Even though SGOT level to identify nursing outcomes was decreased, SGOT was not within normal limits. Therefore, even after discharge of patients, There is a need to take care of them carefully. Conclusion: These findings revealed the significance and need of nurse practitioners who performed professional nursing intervention for the patients with liver transplantation. Especially, it is necessary needs to develop the nursing intervention programs for comfort.

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Cardiopulmonary Symptoms, Quality of Sleep, and Depression in the Hospitalized Patient with Pneumoconiosis (요양병원 진폐 환자의 심폐증상, 수면의 질 및 우울에 관한 연구)

  • Jang, Ju-Yeon;Lee, Young-Hee;Choi, Mi-Kyoung
    • Korean Journal of Adult Nursing
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    • v.23 no.2
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    • pp.135-145
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    • 2011
  • Purpose: This study was to examine the cardiopulmonary symptoms, quality of sleep, and depression, and to identify the influencing factors in the hospitalized coal worker's pneumoconiosis patient. Methods: 137 hospitalized patients who received pneumoconiosis treatment were conveniently selected. A descriptive correlational study was conducted. Data were collected using structured questionnaires and were analyzed using descriptive statistics, t-test, ANOVA, Pearson's correlation coefficients and multiple regression analysis. Results: The mean score of cardiopulmonary symptoms was relatively high ($3.97{\pm}0.56$, out of 5). The 5 and over score of quality of sleep, sleep disturbance was 94.2%, and the reported depression was 95.6%. The levels of depression depended on the oxygen therapy and family/acquaintance visiting. The depression was significantly correlated with the cardiopulmonary symptoms and quality of sleep. The influential factors affecting depression were cardiopulmonary symptoms and quality of sleep, which explained about 34.1% of the variance. Conclusion: The results of this study indicate that nursing interventions are needed to reduce depression, and to improve cardiopulmonary symptoms and quality of sleep in the hospitalized pneumoconiosis patient. These results can provide for nursing intervention to facilitate reduction of depression.

Peroxynitrite Scavenging Activity of Sabohwanin Lipopolysaccharide-Induced Oxidatively-Stressed Mice (Lipopolysaccharide로 산화 스트레스를 유도한 Mouse에서 사보환(四補丸)의 Peroxynitrite 억제 효과)

  • Kweon, Youl;Jeong, Ji-Cheon
    • The Journal of Internal Korean Medicine
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    • v.28 no.1
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    • pp.80-91
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    • 2007
  • Objectives : Peroxynitrite (ONOO-), superoxide anion radical (?O2-) and nitric oxide (NO) are cytotoxic because they can oxidize several cellular components such as proteins, lipids and DNA. They have been implicated in the aging process, and age-related diseases such as Alzheimer's disease, rheumatoid arthritis, cancer and atherosclerosis. The aim of this study was to investigate Sabohwan's activity for scavenging ONOO- and its precursors. NO and ?02-. Methods : For this study, the fluorescent probes, namely 2',7'-dichlorodihydrofluorescein diacetate (DCFDA), 4.5-diaminofluorescein (DAF-2) and dihydrorhodamine 123 (DHR 123) were used. Results : Sabohwanblocked tert-butylhydroperoxide (t-BHP)-induced cell death in a dose-dependent fashion. It scavenged t-BHP-induced ONOO-, NO and ?O2- in YPEN cells. Sabohwan inhibited the generation of ONOO-, NO and ?O2- in the lipopolysaccharide (LPS)-treated mouse kidney postmitochondria both in vitro and in vivo. The lipid peroxide level increased and glutathione level decreased in the LPS-treated mice, whereas the ones in the Sabohwanadministered group among the LPS-treated mice reversed toward their natural levels. Conclusions : These results suggest that Sabohwanis an effective ONOO-, ?O2- and NO scavenger, and thereby it might have a potential role as a therapy against the aging process and age-related diseases.

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Clinical Evaluation of Spontaneous Pneumothorax -A Review of 244 Cases- (자연 기흉의 임상적 고찰 -244례-)

  • 김병린
    • Journal of Chest Surgery
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    • v.27 no.4
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    • pp.292-296
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    • 1994
  • From March, 1985, to June, 1993, 244 patients with 345 episodes of spontaneous pneumothorax treated at Koryo General Hospital were reviewed. Most of the patients were male, and the ratio of male to female was 8:1. The average age of the patients with spontaneous pneumothorax was 32.8 years old. The site of pneumothorax was revealed left side in 53.3%, right side in 42.6%, and bilateral in 4.1%. The cause of pneumothorax were shown primary spontaneous pneumothorax in 73.4%, and secondary spontaneous pneumothorax in 26.6%. The underlying pathologic lesion in secondary spontaneous pneumothorax showed pulmonary tuberculosis in 56patients[86.1%], COPD in 4patients[6.2%], bronchial asthma in 2patients[3.1%], lung cancer in 2patients[3.1%], and pneumoconiosis in a patient[1.5%]. The usual clinical symptomes were dyspnea, chest pain and chest discomfort. Recurrence rate was as follow; 2nd episode 33.6%, 3rd episode in 26.8%, and above in 4th episode in 18.2%. All the patient of pneumothorax was treated as following; Closed thoracostomy tube drainage in 127patients, bullectomy in 88patients, lobectomy in 5patients, wedge resection in 2patients, conservative treatment with oxygen therapy in 21patients, and video assisted thoracoscopic bullectomy in a patient. The course of treatment of all of the patients were smooth and uneventful.

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Oxidative Stress and Skin Diseases: Possible Role of Physical Activity

  • Kruk, Joanna;Duchnik, Ewa
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.2
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    • pp.561-568
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    • 2014
  • Background: The skin is the largest body organ that regulates excretion of metabolic waste products, temperature, and plays an important role in body protection against environmental physical and chemical, as well as biological factors. These include agents that may act as oxidants or catalysts of reactions producing reactive oxygen species (ROS), reactive nitrogen species (RNS), and other oxidants in skin cells. An increased amount of the oxidants, exceeding the antioxidant defense system capacity is called oxidative stress, leading to chronic inflammation, which, in turn, can cause collagen fragmentation and disorganization of collagen fibers and skin cell functions, and thus contribute to skin diseases including cancer. Moreover, research suggests that oxidative stress participates in all stages of carcinogenesis. We report here a summary of the present state of knowledge on the role of oxidative stress in pathogenesis of dermatologic diseases, defensive systems against ROS/RNS, and discuss how physical activity may modulate skin diseases through effects on oxidative stress. The data show duality of physical activity actions: regular moderate activity protects against ROS/RNS damage, and endurance exercise with a lack of training mediates oxidative stress. These findings indicate that the redox balance should be considered in the development of new antioxidant strategies linked to the prevention and therapy of skin diseases.

Effect of Tooth-Cut Induced Dental Malocclusion on Mouse Model of Ischemic Stroke (생쥐의 하악 치아 절단으로 인한 부정교합이 뇌경색에 미치는 영향)

  • Lee, Young-Jun;Lee, Byoungho;Cho, Suin
    • Journal of TMJ Balancing Medicine
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    • v.9 no.1
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    • pp.4-11
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    • 2019
  • Objectives: Although intraoral balancing appliance therapy has been used effective to several diseases, verification studies through cerebral diseases are poorly reported so far. Thus we investigated the effect of tooth-cut induced dental malocclusion against mouse model of ischemic stroke. Methods: Tooth-cut and 90 min middle cerebral artery occlusion (MCAO) were loaded to C57BL/6 male mice, and total infarct area, neurological deficit scores (NDS), histological change of hippocampal region were observed. Production levels of reactive oxygen species (ROS) and inducible nitric oxide synthase (iNOS) in cerebral tissue were also measured. Results: The longer the tooth-cut period, the greater the area of cerebral infarction caused by MCAO, and NDS began to increase as the tooth was cut, and the results were more negative when MCAO was loaded. Histological change of hippocampal cells was significant when tooth-cut was maintained for 7 days. Those damages were thought to depend on the generation of ROS and iNOS in brain tissue. Conclusions: Since tooth-cut increased total area of cerebral infarction due to MCAO in mice, it is able to be confirmed that anomaly of the temporomandibular occlusion can affect neurological diseases.

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Chronic Obstructive Pulmonary Disease with Severe Pulmonary Hypertension - A Case Report - (중증 폐동맥고혈압이 동반된 만성폐쇄성폐질환 1 예)

  • Park, Chan-Soh;Chin, Hyun-Jung;Kim, Seok-Min;Son, Chang-Woo;Yu, Sung-Ken;Chung, Jin-Hong;Lee, Kwan-Ho
    • Journal of Yeungnam Medical Science
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    • v.25 no.1
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    • pp.50-57
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    • 2008
  • Pulmonary hypertension is an increase in blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries. Depending on the cause, pulmonary hypertension can be a severe disease with markedly decreased exercise tolerance and right-sided heart failure. Pulmonary hypertension can present as one of five different types: arterial, venous, hypoxic, thromboembolic, or miscellaneous. Chronic obstructive pulmonary disease with severe pulmonary hypertension is a rare disease. A 52-year-old man presented with a complaint of aggravating dyspnea. The mean pulmonary arterial pressure was 61.5 mmHg by Doppler echocardiogram. The patient was prescribed diuretics, digoxin, bronchodilator, sildenafil, bosentan and an oxygen supply. However, he ultimately died of cor pulmonale. Thus, diagnosis and early combination therapy are important.

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Protective Effects of In Vitro Gastrointestinal Digests of Abalone (Haliotis discus hannai) Intestines against Oxidative Stress in RAW264.7 Macrophage Cells

  • Nguyen, Phuong-Hong;Kim, Sun-Ae;Choi, Il-Whan;Jung, Won-Kyo
    • Fisheries and Aquatic Sciences
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    • v.13 no.3
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    • pp.216-223
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    • 2010
  • Abalone (Haliotis discus hannai), mostly distributed and maricultured in southwestern coastal areas of South Korea, is recognized as an economically important species in the fishery industry. Abalone intestines are one of the by-products of abalone processing. To investigate abalone intestines as bioactive substances, abalone intestine gastrointestinal digests (AIGIDs) of various molecular weights (MWs) were prepared using in vitro gastrointestinal digestion and an ultrafiltration system, and tested for inhibitory effects against reactive oxygen species (ROS) and oxidative stress in macrophage cells treated with hydrogen peroxide ($H_2O_2$). In our results, among AIGIDs, AIGID-III (MW=5-10 kDa) showed potent inhibitory activities for lipid peroxidation and free radicals. Additionally, the results clearly indicated that AIGID-III treatment could prevent cytotoxic damage of macrophages by $H_2O_2$-induced oxidative stress due to its potent scavenging ability against cellular ROS. These results suggest that AIGIDs may have protective and therapeutic potential for oxidative stress syndromes and immune diseases through ROS inhibition in macrophage cells.

Clinical Evaluation of Neonatal Pneumothorax (신생아 기흉의 임상적 고찰)

  • Lee, Seok-Gi;Im, Jin-Su;Choe, Hyeong-Ho
    • Journal of Chest Surgery
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    • v.28 no.12
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    • pp.1132-1138
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    • 1995
  • From 1991 to 1994, we experienced 24 cases of neonatal pneumothorax who were admitted to the Neonatal Intensive Care Unit[NICU , Chosun University Hospital. The Following results were obtained.1 The incidence of neonatal pneumothorax was 0.70%, and there were 8 spontaneous pneumothoraces and 16 secondary pneumothoraces. 2 The clinical manifestation of neonatal pneumothorax was as followed. Male infant was dominant[M:F=2:1 , the onset was within 24 hours in the majority[83% , and the right side[62% was more frequent than the left side. The gestation duration and birth weight show no correlation with underlying neonatal pneumothorax. The pulmonary diseases were meconium aspiration syndrome and hyaline membrane disease, and the incidence of those was 58%. Meconium aspiration syndrome occurred earlier than hyaline membrane disease. Symptoms and signs were tachypnea[46% , cyanosis[21% , irritability[13% , chest retraction[8% and apnea[8% .3 The treatments performed were oxygen therapy[17% , thoracentesis[4% and closed thoracostomy with underwater seal drainage[79% . The Mean duration of air leakage was 11.7 hours, and the mean drainage time was 4.35$\pm$1.3day. 4 The overall hospital mortality was 33%, and the rate of complication was 46%. The complications were metabolic acidosis, atelectasis, pleural effusion, pulmonary hemorrhage and pneumonia. We concluded that the prognosis was related to the underlying pulmonary disease.

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