• Title/Summary/Keyword: Immune deficiency

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A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl

  • Lee, Eun;Oh, Seak-Hee;Kwon, Ji-Won;Kim, Byoung-Ju;Yu, Jin-Ho;Park, Chan-Jeoung;Hong, Soo-Jong
    • Clinical and Experimental Pediatrics
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    • v.53 no.6
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    • pp.722-726
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    • 2010
  • Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as $Aspergillus$ species, $Staphylococcus$ $aureus$, $Serratia$ $marcescens$, $Nocardia$ species and $Burkholderia$ $cepacia$. In case of a history of recurrent or persistent infections, immune deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for $Aspergillus$ antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN-${\gamma}$ initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.

Roles of Mannose-Binding Lectin on Innate Immunity and Disease (Mannose-binding lectin의 선천성 면역과 질병에 대한 역할)

  • Jang, Ho-Jung;Park, Jeong-Hae;Chung, Kyung-Tae
    • Journal of Life Science
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    • v.20 no.9
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    • pp.1420-1425
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    • 2010
  • Innate immunity is the first line of host defense consisting of various molecules against infectious challenges. Mannose-binding lectin (MBL) belongs to the collectin protein family which takes part of innate immunity and is able to recognize specific carbohydrates on the surface of a variety of infectious agents acting as a pattern recognition molecule. In this way, MBL differentiates self from non-self and interacts with other molecules of the immune system. MBL genotype shows various MBL2 polymorphisms which are responsible for MBL deficiency in a substantial portion of the entire human population and for susceptibility to infectious disease. Therefore, it has been highlighted in the relationship between genetic variants and clinical significance. Here we focus on presenting anoverview of our understanding of MBL structure and functions.

The Study about the Comparison of Korean-Western Medicine on Hair (모발에 대한 동서의학적 고찰)

  • Jang, In-Wook;Ko, Woo-Shin;Yoon, Hwa-Jung
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.29 no.4
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    • pp.1-13
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    • 2016
  • Objectives : The purpose of this study is to compare hair and alopecia of Korean Medicine with those of western medicine. Methods : We studied relationships between hair and essense(精), qi(氣), blood(血), five vicera(五臟) and meridians(經絡) through literature review about hair and alopecia. We compared Korean medicine with western medicine on physiology of hair and treatment of alopecia based on the study. Results & Conclusion : 1. Congenital essense(先天之精) is related with genetic factor and acquired essence(後天之精) is connected with nutritional factor. Defending function of Defense qi(衛氣) is related with immune reaction and qi stagnation(氣鬱) is associated with stress reaction. Atrophy of vascular tissues observed in alopecia scalp means deep relationship between blood(血) and alopecia, further deficiency of blood can cause telogen effluvium. 2. Kidney qi(腎氣) is related with inhibiting combination of Androgen receptor and Dihydrotestosterone(DHT) or activating hair growth factior. Pi(脾) is connected with alopecia seborrheica caused by damp-heat(濕熱) and alopecia areata caused by excessive prudence(思慮過度). Heart(心) is associated with atrophy in vascular tissue of scalp and liver(肝) is connected with metabolism. 3. Armpit hair and pubic hair as secondary sex characteristics are realated with Yangming Meridian(陽明經) and beard and hair at crown part where Type II $5{\alpha}$-reductase is activated much are associated with Taiyang Meridian(太陽經). 4. Juglandis Semen pharmacopuncture and Ganoderma lucidum pharmacopucture have better effects on inhibit $5{\alpha}$-reductase than Finasteride. Minoxidil and PRP are similar with promoting blood flow and removing stasis(活血祛瘀). Seven-star needling(七星針) is similar with microneedling. 5. Alopecia can be caused by due to lack of circulation Views we need solution to improve circulation coincide in opinion with Korean Medicine and Western medicine.

Various Pulmonary Manifestations of the Cryptococcal Pneumoniae in the Three Immunocompetent Patients (다양한 양상으로 발현된 폐크립토콕스증 3예)

  • Park, Jin-Chan;Kim, Hyung-Tae;Jeung, Hun;Park, Ji-Han;Choi, Jae-Hyuck;Kim, Hyeon-Tae;Park, Jae-Min;Lee, Yong-Hee;Kim, Jeung-Sook
    • Tuberculosis and Respiratory Diseases
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    • v.50 no.3
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    • pp.359-366
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    • 2001
  • More than half of the cryptococcal infections occur in acquired immune deficiency (AIDS) patients, and more than half of the non-AIDS patients with cryptococcosis are immunocompromised. Most immunocompromised patients have meningoencephalitis at the time of diagnosis. Without the appropriate therapy, this form of the infection is invariably fatal. Death can occur any time from 2 weeks to several years after the onset of symptoms. Pulmonary cryptococcosis in immunocompromised patients is usually asymptomatic, but coughing, chest pain, fever, or hemoptysis may occur in immunocompetent patients. Pulmonary cryptococcosis symptoms in immunocompetent patients tend to improve without treatment. Here, we describe the various pulmonary manifestations of cryptococcal pneumoniae in three immunocompetent patients.

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Dental Treatment of an AIDS Patient with Cerebral Infarction History and Hepatitis Type B Under General Anesthesia (뇌경색 및 B형 간염을 동반한 후천성 면역 결핍증 환자의 전신 마취 하 치과치료)

  • Ji, Sang-Eun;Kim, Jong-Soo;Kim, Chul-Hwan;Kim, Seung-Oh
    • Journal of The Korean Dental Society of Anesthesiology
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    • v.14 no.4
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    • pp.237-241
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    • 2014
  • Acquired Immune Deficiency Syndrome is the state which develops after complications with the infection from HIV. Irrespective of their state, all HIV infections have infectivity. According to a 2013 U.N. AIDS global report, the number of reported newly infected with HIV is constantly falling, while that of Korea has been increasing over recent years and it surpassed 10,000 in 2013. This phenomenon might be attributed to the unusualness of the blood test for early detection of HIV infection. From this fact, we can assume that we have strong possibilities for encounters with infections in the office. But many misconceptions about the disease makes patients try to hide their medical history, which can lead to a nationwide spread of the infection without proper management. Even though it may be difficult to take care of HIV patients in smaller dental offices, large scale hospitals have the means to arrange protocols to treat them. We present a case about dental treatment of a patient with AIDS that has a history of cerebral infarction and hepatitis type B under general anesthesia. The purpose of this case report was to discuss the special considerations of dental care for patients with HIV.

Nutrient Supplementation in the Elderly

  • Meydani, Mohsen
    • Journal of Community Nutrition
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    • v.2 no.1
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    • pp.5-11
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    • 2000
  • Populations both in the US and worldwide are aging. It is projected that by the year 2030 the population of Americans over 65 will increase to 70 million, more than twice their number in 1998. About one-third of elderly over the age of 65 have debilitating chronic health conditions that greatly impede their activities. Age-associated chronic diseases are believed to be associated with free radicals and the imbalance in antioxidant and oxidative stress contributes to development of several chronic health conditions. Diet and nutrients can have great impact in the health status of elderly. Several factors may contributor to the inadequate consumption of nutrients in elderly, including an inability to chew food adequately, polypharmacy, living along and limited income. Low intake of energy and several micronutrients are common among the elderly. Although overt deficiency of nutrients among the elderly is not common. a recent study showed that while elderly consume more fat, the total energy intake is low among the elderly. Inadequate intake of antioxidant nutrients such as vitamin E in elderly may contribute to the development of chronic health problems. Intake of higher than normal RDA levels of vitamin E for long periods of time has been shown to reduce the risk of many degenerative diseases, such as cardiovascular diseases(CVD) and cancer, and improve immune response in elderly. High intake of other antioxidants is also associated with reduced risk chronic diseases. Dietary antioxidants are abundant in fruits and vegetables. However, due to variety of factors, the elderly may not be able to receive these and other micronutrients through diet. Therefore, supplemental intake of micronutrients in the form of multi vitamins/minerals and/or specific micronutrients is a more practical approach to the maintenance of health status in the elderly.

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Cloning and Expression of Lactadherin Gene from Korean Women (한국 여성의 Lactadherin 유전자 Cloning과 발현 연구)

  • Yom, Heng-Cherl
    • Development and Reproduction
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    • v.11 no.3
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    • pp.253-261
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    • 2007
  • Lactadherin is a glycoprotein of human milk fat globule membrane that binds to mucin and butyrophilin forming the protein complex. Especially, mucin and lactadherin in human milk efficiently protect infants with poor immune functions right after birth from infections by microorganisms and play important roles for their early survival, growth and development. Lactadherin inhibits the propagation and growth of rotavirus that is a global pathogen causing infants' diarrhea. Recently this protein was known to promote neovascularization and its deficiency related to develop Alzheimer's disease. In this study, the basic biochemical and physiological aspects of lactadherin were investigated. Messenger RNAs were isolated from mammary tissues from Korean women patients to clone a 1.2 kb cDNA and sequenced its DNA to determine its amino acid sequences. The cDNA was cloned to express its 43 kD protein in E. coli, which was confirmed by Western blot. The recombinant protein was purified and injected to 2 rabbits to raise antibodies against it. The semi-purified milk fat globule membrane proteins from Korean women was analyzed by Western blot using the rabbit antibody to give 70, 55, 46, 30 kD bands. Also several polymorphism and SNPs of lactadherin gene from Korean women were observed compared with those of Caucasian women.

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The effects of elimination diet on nutritional status in subjects with atopic dermatitis

  • Kim, Jungyun;Kwon, Jaryoung;Noh, Geunwoong;Lee, Sang Sun
    • Nutrition Research and Practice
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    • v.7 no.6
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    • pp.488-494
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    • 2013
  • A food allergy is an adverse health effect arising from a specific immune response that occurs reproducibly upon exposure to a given food. In those with food allergies that are thought to cause aggravation of eczema, food avoidance is important. The objective of this study was to research the nutritional status of patients with food allergies. A total of 225 subjects diagnosed with atopic dermatitis underwent a skin prick test as well as measurement of serum immunoglobulin E. Food challenge tests were conducted using seven food items: milk, eggs, wheat, soybeans, beef, pork, and chicken. At post-food challenge visits to the test clinic, participants completed a three-day dietary record, which included two week days and one weekend day, in order to evaluate energy intake and diet quality during the challenge. We analyzed nutrient intake based on differential food allergens. Subjects with a food allergy to milk showed lower intake of Ca, Zn, and vitamin B2, and subjects with a food allergy to egg showed lower intake of vitamin A, B1, B2, niacin, and cholesterol. Subjects with a food allergy to wheat and soybean showed lower intake of Ca, P, Fe, K, Zn, vitamin B2, vitamin B6, and niacin; and subjects with a food allergy to beef, pork, and chicken showed lower intake of Fe and higher intake of K, vitamin A, B2. Subjects with atopic dermatitis were lacking in several nutrients, including vitamin A and vitamin C. A greater number of food allergies showed an association with a greater number of nutrient intake deficiencies. Allergen avoidance is the basic treatment for atopic dermatitis. However, when the allergen is food, excessive restriction can lead to nutrition deficiency. Findings of this study suggest the necessity for enhanced nutritional education in order to provide substitute foods for patients with food allergies who practice food restriction.

Surgical Treatment of Ulcerative Colitis in Children (소아 궤양성 대장염의 수술적 치료 성적)

  • Kim, Ji-Hoon;Kim, Hyun-Young;Jung, Sung-Eun;Park, Kwi-Won;Kim, Woo-Ki
    • Advances in pediatric surgery
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    • v.11 no.2
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    • pp.141-149
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    • 2005
  • Ulcerative colitis, an inflammatory bowel disease, is primarily managed medically with a combination of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, indications and outcome of surgical management of 21 patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were reviewed. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management after diagnosis and 8 patients (38 %) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53 %) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure as a result of sclerosing cholangitis. In 8 patients who received surgical management, the indications for operation were, 1 patient sigmoid colon perforation and 7 patients intractability despite medical management. The perforated case had a segmental colon resection and the other 7 patients underwent total colectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. and 1 is still on medical management because of mild persistent hematochezia after surgery. Six other operated patients are doing well without medical therapy. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation are present. Total colectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.

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A case of CHARGE syndrome featuring immunodeficiency and hypocalcemia

  • Son, Yu Yun;Lee, Byeonghyeon;Suh, Chae-Ri;Nam, Hyo-Kyoung;Lee, Jung Hwa;Hong, Young Sook;Lee, Joo Won
    • Journal of Genetic Medicine
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    • v.12 no.1
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    • pp.57-60
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    • 2015
  • CHARGE syndrome (coloboma, heart defects, atresia choanae, retarded growth and development, genital hypoplasia, and ear abnormalities) is characterized by multiple malformations and is diagnosed using distinct consensus criteria. Mutations in the gene encoding chromodomain helicase DNA-binding protein 7 (CHD7) are the major cause of CHARGE syndrome. Clinical features of CHARGE syndrome considerably overlap those of 22q11.2 deletion syndrome. Of these features, immunodeficiency and hypocalcemia are frequently reported in patients with 22q11.2 deletion syndrome but are rarely reported in patients with CHARGE syndrome. In this report, we have described the case of a patient with typical phenotypes of 22q11.2 deletion syndrome but without the proven chromosome microdeletion. Mutation analysis of CHD7 identified a pathogenic mutation (c.2238+1G>A) in this patient. To our knowledge, this is the first case of CHARGE syndrome with immunodeficiency and hypocalcemia in Korea. Our observations suggest that mutation analysis of CHD7 should be performed for patients showing the typical phenotypes of 22q11.2 deletion syndrome but lacking the proven chromosome microdeletion.