• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제23권2호
• /
• pp.180-187
• /
• 2020

Giant cell hepatitis with autoimmune hemolytic anemia (AHA) is a rare disease of infancy characterized by the presence of both Coombs-positive hemolytic anemia and progressive liver disease with giant cell transformation of hepatocytes. Here, we report a case involving a seven-month-old male infant who presented with AHA followed by cholestatic hepatitis. The clinical features included jaundice, pallor, and red urine. Physical examination showed generalized icterus and splenomegaly. The laboratory findings suggested warm-type AHA with cholestatic hepatitis. Liver biopsy revealed giant cell transformation of hepatocytes and moderate lobular inflammation. The patient was successfully treated with four doses of rituximab. Early relapse of hemolytic anemia and hepatitis was observed, which prompted the use of an additional salvage dose of rituximab. He is currently in clinical remission.

• Biomolecules & Therapeutics
• /
• 제27권2호
• /
• pp.152-159
• /
• 2019

Multiple sclerosis (MS) is an autoimmune disease characterized by progressive neuronal loss, neuroinflammation, axonal degeneration, and demyelination. Previous studies have reported that 6-shogaol, a major constituent of ginger (Zingiber officinale rhizome), and its biological metabolite, 6-paradol, have anti-inflammatory and anti-oxidative properties in the central nervous system (CNS). In the present study, we investigated whether 6-shogaol and 6-paradol could ameliorate against experimental autoimmune encephalomyelitis (EAE), a mouse model of MS elicited by myelin oligodendrocyte glycoprotein ($MOG_{35-55}$) peptide immunization with injection of pertussis toxin. Once-daily administration of 6-shogaol and 6-paradol (5 mg/kg/day, p.o.) to symptomatic EAE mice significantly alleviated clinical signs of the disease along with remyelination and reduced cell accumulation in the white matter of spinal cord. Administration of 6-shogaol and 6-paradol into EAE mice markedly reduced astrogliosis and microglial activation as key features of immune responses inside the CNS. Furthermore, administration of these two molecules significantly suppressed expression level of tumor necrosis $factor-{\alpha}$, a major proinflammatory cytokine, in EAE spinal cord. Collectively, these results demonstrate therapeutic efficacy of 6-shogaol or 6-paradol for EAE by reducing neuroinflammatory responses, further indicating the therapeutic potential of these two active ingredients of ginger for MS.

• IMMUNE NETWORK
• /
• 제24권2호
• /
• pp.12.1-12.17
• /
• 2024

Exosomes are double phospholipid membrane vesicles that are synthesized and secreted by a variety of cells, including T cells, B cells, dendritic cells, immune cells, are extracellular vesicles. Recent studies have revealed that exosomes can play a significant role in under both physiological and pathological conditions. They have been implicated in regulation of inflammatory responses, immune response, angiogenesis, tissue repair, and antioxidant activities, particularly in modulating immunity in autoimmune diseases (AIDs). Moreover, variations in the expression of exosome-related substances, such as miRNA and proteins, may not only offer valuable perspectives for the early warning, and prognostic assessment of various AIDs, but may also serve as novel markers for disease diagnosis. This article examines the impact of exosomes on the development of AIDs and explores their potential for therapeutic application.

• Neonatal Medicine
• /
• 제28권3호
• /
• pp.139-142
• /
• 2021

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.

• Clinical and Experimental Pediatrics
• /
• 제48권3호
• /
• pp.292-297
• /
• 2005

대 상 : 1981년 1월부터 2004년 7월까지 서울대학교병원 소아과에서 추적 중인 1형 당뇨병 환자 일부에서 후향적으로 의무기록 분석을 시행하였다. 방 법 : Anti-TG Ab, anti-TPO Ab를 방사면역방법으로 측정하였으며, 둘 중 하나라도 양성인 경우에는 자가면역성 갑상선염이 있는 군(갑상선 질환군)으로 정의하였다. 두 항체 모두 음성이며, 갑상선 수용체 항체(TSHRAb)가 음성인 경우는 자가면역성 갑상선 질환이 없는 군(갑상선 비질환군)으로 정의하였다. TSHRAb가 양성인 Graves병 환자는 갑상선 질환군에 포함시켰다. 결 과 : 총 139명에서 검사를 시행하였으며, 남아 44명(연령 $14.4{\pm}4.8$, 5.9-26.6세), 여자 95명(연령 $13.6{\pm}5.4$세, 3.6-30세)이었다. 갑상선 질환군과 비 갑상선 질환군에서 성비, 당화혈색소, 체질량지수, 당뇨병의 발병연령, 갑상선 질환의 가족력에서 두 군 간에 통계적으로 유의한 차이는 없었다. 갑상선 질환은 남자에서는 역연령 5.0-9.9세 사이에서 검사한 환자의 42.8%에서 발견되었고 여자에서는 역연령 10-14.9세, 15-19.9세에서 각각 56.5%와 50.0%에서 발견되어 남자가 여자에 비해 상대적으로 낮은 연령에서 흔하게 발생하였다. 자가면역성 갑상선 질환은 갑상선종이 있는 경우에서 없는 경우보다 흔하게 발견되었고 갑상선종이 동반된 경우에 남녀간의 발생빈도의 차이는 없었다. 갑상선 기능은 정상 기능을 유지하는 경우가 74%였고, 기능저하와 기능항진은 각각 13%로 동일한 비율을 보였다. 결 론 : 1형 당뇨병 환자에서는 10세 이상의 연령에서 갑상선 기능검사와 자가항체검사가 필요한데 특히, 갑상선종이 발견되거나 남아에서는 최소 8세가 되면 갑상선 기능검사와 항체검사를 시행하고 지속적으로 추적하는 것이 바람직할 것으로 생각된다.

• 혜화의학회지
• /
• 제20권1호
• /
• pp.11-23
• /
• 2011

Systemic Lupus Erythematosus(SLE) is an autoimmune disease invading the skin, joint, kidney, intestinal membrane, neurosystem and other organs. SLE is an autoimmune disease characterized by immune dysregulation resulting in the production of antinuclear antibodies(ANA), generation of circulating immune complexes, and activation of the complement system. In Korean medicine, lupus can be classified as acute arthritis, reddish butterfly erythema, asthenic disease, edema and so on. The cause and procedure of the diseases are flourishing noxious heat, excessive fire due to deficiency of yin, blood stasis due to stagnation of qi, internal movement of the liver-wind, congenital deficiency, exhausted vital-qi, which are treated by clearing away heat and cooling the blood, nourshing yin and extinguishing fire, treating flatulence and activating blood circulation, nourishing the blood to expel wind, invigorating the liver and kidney, invigorating qi and replenishing the blood. To experimentally examine the influence of Insam-Buja-Tang (Ginseng & Aconiti Extract, IBT) on the outbreak and development of lupus, lupus induce MRL/MpJ-Faslpr lupus-prone mice model was used. As IBT was orally administrated to a lupus model mouse, various tests such as the weight, urine protein, renal function, Lymph cell test of the spleen, Cytokine expression, histopathological analysis of kideny were performed to see the influence on the kidney and whether it work effectively on the immune function. The main purpose of this study is to evaluate the effect of IBT on MRL/MpJ-Faslpr lupus-prone mice model. The effect of IBT on MRL/MpJ-Faslpr lupus-prone mice that can have autoimmune disease similar to SLE in human was evaluated after IBT per oral in the present study.

• Biomolecules & Therapeutics
• /
• 제2권4호
• /
• pp.376-382
• /
• 1994

Repeated injections of low-doses of mercuric chloride in rats or mice induce polyclonal activation which includes the induction of anti-glomerular basement membrane (GBM) antibodies and circulating immune complex and it results in nephritis. Because this disease is autoimmune mediated disease resulted from immune dysfunction, immunomodulators are used to control the symptoms or to cure the disease. Irpex lacteus Fr. is a kind of new medicinal fungus. The polysaccharide fraction extracted from submerged fermentation of Irpex lacteus Fr. decreased the serum agglutinin, serolysin and IgM plaque forming cells in normal mice. The hitherto obtained clinical results suggested that it significantly improved the oligourea, edema, and hypertension in patients who have nephritis. To elucidate the action-mechanisms of Irpex lacteus Fr., we established the experimental model of HgCl$_2$induced polyclonal activation by intraperitoneal administrations of HgCl$_2$to mice. To assess the immunomodulating effect of Irpex lacteus fraction, we Investigated its effects on the mitogen induced proliferation and IgM PFC counts of splenic lymphocytes in mice during the treatment of HgCl$_2$. The Irpex lacteus polysaccharide reduced the abnormally increased mitogen induced Iymphocyte proliferation and IgM PFCs to almost normal levels. And the Irpex lacteus polysaccharides prevented the increasement of serum immunoglobulin level induced by HgCl$_2$. These data suggested that the Irpex lacteus polysaccharides might have the immunomodulating activity to prevent and /or improve the HgCl$_2$ induced autoimmune disease.

• Advances in Traditional Medicine
• /
• 제5권4호
• /
• pp.251-261
• /
• 2005

Rheumatoid arthritis (RA) is an autoimmune/inflammatory disorder with a complex genetic component. RA is characterized by chronic inflammation of the synovial membrane in the joint, which leads to the progressive destruction of articular cartilage, ligament and bone. Several cytokines such as tumor necrosis $factor-{\alpha}\;TNF-{\alpha}\;and\;interleukin-1{\beta}\;(IL-1{\beta})$ and interleukin-6 (IL-6) have been implicated in the pathological mechanisms of synovial tissue proliferation, joint destruction and programmed cell death in rheumatoid joint. Genome wide screening of subjects suffering from autoimmune diseases especially arthritis revealed linkage to inflammatory molecules like $TNF-{\alpha},\;IL-1{\beta}$ and IL-6, inducible nitric oxide synthase (iNOS), cyclooxygenase-2 (COX-2), nuclear factor-kappaB $(NF-{\kappa}B)$ and human leucocyte antigen/major histocompatibility complex (HLA/MHC) locus. The status of the pharmacological mechanism of herbal drugs in the light of genome wide screening results has been discussed to reinforce the therapeutic potential and the pharmacological basis of the herbal drugs.

• Journal of Oral Medicine and Pain
• /
• 제46권2호
• /
• pp.63-68
• /
• 2021

Mucous membrane pemphigoid (MMP) is a heterogeneous group of chronic, autoimmune subepithelial blistering diseases, predominantly involving oral mucosal membrane. Because of its pathophysiology of autoimmune reaction, MMP-related gingivitis would not respond to conventional periodontal treatments. We present a case of a 65-year-old female with a chief complaint of chronic generalized buccal gingivitis, unimproved after periodontal treatment for four months. Based on the clinical findings, histological examination, and direct immunofluorescence microscopy, it was diagnosed as MMP. The symptoms were relieved with immunomodulatory therapy using corticosteroids and the supportive management of professional plaque control. MMP can cause pathological involvement throughout the oral and other mucosae of the body, thus leading to functional impairment through repeated inflammatory cascades. Therefore, accurate diagnosis is essential to properly manage local and possible systemic complications of MMP.

• IMMUNE NETWORK
• /
• 제20권6호
• /
• pp.47.1-47.13
• /
• 2020

Tetracyclines, which have long been used as broad-spectrum antibiotics, also exhibit a variety of nonantibiotic activities including anti-inflammatory and immunomodulatory properties. Tetracyclines bind to the 30S ribosome of the bacteria and inhibit protein synthesis. Unlike antimicrobial activity, the primary molecular target for the nonantibiotic activity of tetracycline remains to be clarified. Nonetheless, the therapeutic efficacies of tetracyclines, particularly minocycline and doxycycline, have been demonstrated in various animal models of autoimmune disorders, such as multiple sclerosis, rheumatoid arthritis, and asthma. In this study, we summarized the anti-inflammatory and immunomodulatory activities of tetracyclines, focusing on the mechanisms underlying these activities. In addition, we highlighted the on-going or completed clinical trials with reported outcomes.