• Clinical and Experimental Pediatrics
• /
• 제52권6호
• /
• pp.649-654
• /
• 2009

The immune system is comprised of cells and molecules whose collective and coordinated response to the introduction of foreign substance is referred to as the immune response. Defense against microbes is mediated by the early reaction (innate immunity) and the late response (adaptive immunity). Innate immunity consists of the epithelial barrier, phagocytes, complement and natural killer cells. Adaptive immunity, a more complex defense reaction, consists of activation of later-developed lymphocytes that, when stimulated by exposure to infectious agents, increase in magnitude and defensive capabilities with each successive exposure. In this review we discuss recent advances in important primary immune deficiency disorders of innate immunity (chronic granulomatous disease, leukocyte adhesion deficiency) and adaptive immunity (severe combined immune deficiency, Wiskott- Aldrich syndrome).

• Tuberculosis and Respiratory Diseases
• /
• 제71권4호
• /
• pp.282-285
• /
• 2011

Common variable immune deficiency (CVID) is the most common primary immune deficiency, which is manifested as chronic recurrent respiratory infections and hypoglobulinemia. CVID usually presents in the second or third decade of life. A 33-year-old woman was admitted with recurrent pneumococcal pneumonia with bacteremia and had very low levels of serum immunoglobulin G, M and A. This case emphasized a high index of suspiciousness for diagnosis of CVID in a mid-adulthood patient presenting with recurrent pneumonia with hypoglobulinemia.

• Toxicological Research
• /
• 제33권4호
• /
• pp.283-290
• /
• 2017

The host immune system is the first line of host defense, consisting mainly of innate and adaptive immunity. Immunity must be maintained, orchestrated, and harmonized, since overactivation of immune responses can lead to inflammation and autoimmune diseases, while immune deficiency can lead to infectious diseases. We investigated the regulation of innate and adaptive immune cell activation by Artemisia capillaris and its components (ursolic acid, hyperoside, scopoletin, and scopolin). Macrophage phagocytic activity was determined using fluorescently labeled Escherichia coli, as an indicator of innate immune activation. Concanavalin A (ConA)- and lipopolysaccharide (LPS)-induced splenocyte proliferation was analyzed as surrogate markers for cellular and humoral adaptive immunity, respectively. Neither A. capillaris water extract (WAC) nor ethanol extract (EAC) greatly inhibited macrophage phagocytic activity. In contrast, WAC suppressed ConA- and LPS-induced proliferation of primary mouse splenocytes in a dose-dependent manner. Similarly, EAC inhibited ConA- and LPS-induced splenocyte proliferation. Oral administration of WAC in mice decreased ConA- and LPS-induced splenocyte proliferation, while that of EAC suppressed LPS-induced splenocyte proliferation. Repeated administration of WAC in mice inhibited ConA- and LPS-induced splenocyte proliferation. Ursolic acid, scopoletin, and scopolin reduced ConA- and LPS-induced primary mouse splenocyte proliferation, while hyperoside did not show such activity. These results indicate that A. capillaris and its components, ursolic acid, scopoletin, and scopolin, suppress ConA- and LPS-induced adaptive immune cell activation. The results suggest that A. capillaris is useful as a regulator of adaptive immunity for diseases involving excessive immune response activation.

• 대한후두음성언어의학회지
• /
• 제24권2호
• /
• pp.125-127
• /
• 2013

Infection by aspergillus, which is a kind of mold, or a filamentous fungus, occurs rarely in larynx. Furthermore, primary laryngeal aspergillosis without any other airway tract extension and without any generalized immune deficit is extremely rare. We present a case of primary aspergillosis on vocal cord in a 72-year-old male who had no history of immune deficiency, voice abuse or steroid use.

• Pediatric Infection and Vaccine
• /
• 제12권1호
• /
• pp.95-99
• /
• 2005

주폐포자충 폐렴은 면역 기능이 억제된 환자군에서 발병률이 높은 감염성 질환으로 특히, 후천성 면역 결핍증이나 백혈병 등의 혈액 종양 환자군, 심한 영양 결핍 환자군, 장기 이식 환자군, 고용량의 부신 피질 호르몬 사용 환자군 등이 고위험군으로 알려져 있으며, 일차성 면역 결핍증에 이환된 환아에서 주폐포자충 폐렴의 증례가 보고되어 있다. 저자들은 상기 위험군에는 속하지 않으나, 10여일 정도의 수유부진 후 빈호흡을 보인 영아에서 흉부 방사선 소견상, 폐양측에 간질성 침윤이 관찰되었고 기관지내 삽관으로 흡인한 객담의 Gomori-methenamine silver 염색 검사상 주폐포자충이 검출되어 주폐포자충 폐렴으로 확진 후 TMP-SMX 14일 투여로 호전을 보인 예를 경험하여 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제39권4호
• /
• pp.340-342
• /
• 2006

원발성 흉골 골수염은 드문 질환이다. 특히 소아에서 발생한 경우는 매우 드물어 영어로 보고된 문헌에 11예가 보고되었다. 원발성 흉골 골수염은 영양실조, 면역 저하, 흉부 둔상, 정맥을 통한 약물 주사, 낫 세포빈혈증 등의 환자에서 주로 발생하며, 배농 및 항생제 투여로 치료한다. 저자들은 기저질환이 없는 16세 남아에서 발생한 원발성 흉골 골수염 1예를 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제26권4호
• /
• pp.201-212
• /
• 2023

Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion: Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.

• Pediatric Infection and Vaccine
• /
• 제14권1호
• /
• pp.116-119
• /
• 2007

범저감마글로불린혈증은 X 염색체에 의해 유전되는 질환으로 주로 1세 이전에 진단이 가능하지만, 진단이 늦어질 경우 반복된 감염 및 중증 감염으로 인한 심각한 후유증을 남길 수 있으므로 조기 진단과 치료가 매우 중요하다. 증상이 다양하게 나타날 수 있으므로 진단을 위한 각별한 주의가 요구된다. 저자들은 반복적이고 심각한 감염의 병력에도 8세가 돼서야 범저감마글로불린혈증 진단을 받은 환아와 그로 인해 함께 진단 받은 친형의 사례를 경험하였기에 보고하는 바이다.

• Journal of Ginseng Research
• /
• 제43권4호
• /
• pp.676-683
• /
• 2019

Background: Korean Red Ginseng (KRG) is widely used for strengthening the immune system and fighting fatigue, especially in people with deficiency syndrome. However, there is concern that the long-term application or a high dose of KRG can cause "fireness" (上火 in Chinese) because of its "dryness" (燥性 in Chinese). The aim of this study was to assess the safety and efficacy of a 4-week treatment with KRG in participants with deficiency syndrome. Methods: This was a 4-week, randomized, double-blind, placebo-controlled clinical trial. A total of 180 Chinese participants were randomly allocated to three groups: placebo control group, participants were given a placebo, 3.6 g/d; KRG 1.8 g and 3.6 g groups. The primary outcomes were the changes in fireness and safety evaluation (adverse events, laboratory tests, and electrocardiogram). The secondary outcomes were the efficacy of KRG on fatigue, which include the following: traditional Chinese medicine (TCM) symptom scale and fatigue self-assessment scale. Results: Of the 180 patients, 174 completed the full study. After 4 weeks of KRG treatment, the Fire-heat symptoms score including Excess fire-heat score and Deficient fire-heat score showed no significant change as compared with placebo treatment, and no clinically significant changes in any safety parameter were observed. Based on the TCM syndrome score and fatigue self-assessment score, TCM symptoms and fatigue were greatly improved after treatment with KRG, which showed a dose- and time-dependent effect. The total effective rate was also significantly increased in the KRG groups. Conclusion: Our study revealed that KRG has a potent antifatigue effect without significant adverse effects in people with deficiency syndrome. Although a larger sample size and longer treatment may be required for a more definite conclusion, this clinical trial is the first to disprove the common conception of "fireness" related to KRG.

• 대한한의학방제학회지
• /
• 제25권2호
• /
• pp.223-251
• /
• 2017

Objectives : A lot of experiment results of Yugmijihwangtang(YM) are reported in various kinds of journals. Many of them report on the new effects that are not recorded in the traditional medical texts. So it is necessary to take it into consideration that newly reported effects could be of help to clinical practice, because this process of comparison of Donguibogam and scientific experiment results will have basis to lead into the evidence based medicine. Methods : We compared the effects of in Donguibogam and the experiment results of YM. Results : The effects of YM in Donguibogam are to replenish essence and marrow, and to treat red wen, fatigue, treat hypouresis, urinary sediment, urinary urgency, hematuria, hydrocephalus, speech and movement retardation, yin-deficiency, diabetes mellitus, nonalcoholic fatty liver, melanoma, disability to see near and far sight, tinnitus, hearing loss, alopecia, angiogenesis, cough, cough at night, trachyphonia, and, infantile convulsion. The experiment results of YM since 2000 in both Korea and China are to inhibit atopic dermatitis, renal interstitial fibrosis, anti-oxidant, emphysema, stress, glomerulosclerosis, diabetic nephropathy, chronic glomerulonephritis, hemorrhage, plantar sweating, dermal aging, kidney aging, bone loss, breast cancer, pathological myocardial cell, primary liver cancer, thrombosis, osteoporosis, intrauterine growth retardation, chronic renal failure, IgA nepropathy, slow cerebral development, and hippocampal tissue lesions on the one hand, and to help bone formation, renin-angiotensin- aldosterone system, cerebral recovery, cognitive function and expression, osteoblast proliferation and differentiation, learning and memory, cold-tolerance and oxygen deficit-tolerance and anti-fatigue, endometrial formation, humoral and cell-mediated immunity, immune regulation effect, Hypothalamus-Pituitary-Ovary Axis, and spermatogenesis, on the other hand. Conclusion : When we compared the effects of YM with the experiment results of YM, there existed a considerable gap between them. So, from now on, it is expected that a great effort and consideration are needed to solve these gaps from an academic and clinical point of view.