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Review Article
Neurology
Autoimmune encephalitis and epilepsy: evolving definition and clinical spectrum
Joo Hee Seo, Yun-Jin Lee, Ki Hyeong Lee, Elakkat Gireesh, Holly Skinner, Michael Westerveld
Clin Exp Pediatr. 2020;63(8):291-300.   Published online August 16, 2019
Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis...
Case Report
A case of Kikuchi-Fujimoto disease with autoimmune thyroiditis
Eun Ji Go, You Jin Jung, Seung Beom Han, Byung Kyu Suh, Jin Han Kang
Clin Exp Pediatr. 2012;55(11):445-448.   Published online November 23, 2012

Kikuchi-Fujimoto disease (KFD) is a benign self-limiting disease characterized by fever and lymphadenitis. The etiology and pathogenesis of KFD is unclear. However, two hypotheses have been suggested: a viral infection hypothesis and an autoimmune hypothesis. Several KFD patients with various types of autoimmune diseases have been reported, and these reports support the hypothesis for autoimmune pathogenesis of KFD. Here, we...

Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis
Jeana Hong, Mi Kyoung Song, Jae Sung Ko, Gyeong Hoon Kang, Woo Sun Kim, Jeong Kee Seo
Clin Exp Pediatr. 2009;52(4):504-507.   Published online April 15, 2009
Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of...
Autoimmune hepatitis and thyroiditis associated with antituberculous medications : A case report
Seong Keun Yu, Sara Kim, Jin Soo Moon, Han Seong Kim
Clin Exp Pediatr. 2008;51(5):528-532.   Published online May 15, 2008
Drug-induced toxic hepatitis is a relatively common hepatic disease in children, and it is usually self-limiting upon cessation of the offending drugs. Antituberculous drugs are well known for inducing hepatitis. Some cases of drug-induced hepatitis with autoimmune features have been reported; in these cases, the offending drugs were usually methyldopa, nitrofurantoin, minocycline, and interferon. The authors report the first case...
Review Article
Autoimmunity
Joong Gon Kim
Clin Exp Pediatr. 2007;50(12):1165-1172.   Published online December 15, 2007
Self/non-self discrimination and unresponsiveness to self is the fundamental properties of the immune system. Self-tolerance is a state in which the individual is incapable of developing an immune response to an individual's own antigens and it underlies the ability to remain tolerant of individual's own tissue components. Several mechanisms have been postulated to explain the tolerant state. They can be...
Hemolytic anemia in pediatrics
Jeong Ok Hah
Clin Exp Pediatr. 2007;50(6):511-518.   Published online June 15, 2007
To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types : RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea,...
Original Article
Clinical Characteristics of Autoimmune Thyroid Disease Developed in Patients with Type 1 Diabetes Mellitus
Se Min Lee, Hye Rim Chung, Su Young Hong, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2005;48(3):292-297.   Published online March 15, 2005
Purpose : It is known that 3-50 percent of type 1 diabetes mellitus(T1DM) patients develop autoimmune thyroid disease. We analyzed the clinical characteristics of autoimmune thyroid disease(AITD) developed in patients with T1DM in Korean. Methods : The medical records of 139 patients, who were followed up in Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1981 to Jul. 2004,...
Case Report
A Case of Rothmund-Thomson Syndrome with Pure Red Cell Aplasia, Autoimmune Hemolytic Anemia and Chronic Respiratory Infection
Jung Hyun Lee, Eun Seok Roh, Yoo Rah Hong, Jae Sun Park, Ghi Seok Seo, Bang Hur, Mi Hyang Kim
Clin Exp Pediatr. 2004;47(12):1351-1355.   Published online December 15, 2004
Rothmund-Thomson syndrome(RTS), or poikiloderma congenita, is a rare, multisystem disorder. It is inherited genetically as an autosomal recessive trait, occurring predominantly in females(1.4 : 1). The RTS is comprised of poikiloderma, short stature, sparse hair, juvenile cataracts, skeletal defects, dystrophic teeth and nails, photosensitivity, and hypogonadism. We report a case of RTS who died of bleeding from esophageal varices, pulmonary...
Original Article
Clinical Characteristic of Chronic Autoimmune Thyroiditis in Children
Hye Rim Chung, Choong Ho Shin, Sei Won Yang
Clin Exp Pediatr. 2004;47(1):76-80.   Published online January 15, 2004
Purpose : Although chronic autoimmune thyroiditis(AIT) is known to progress into overt hypothyroidism in adults, the outcomes of this disorder in pediatric patients are different from those in adults, so it is hard to predict its course. We reviewed clinical characteristics of chronic AIT in children. Methods : The medical records of 94 children, who were diagnosed as AIT, were analyzed,...
Case Report
A Case of Successful Danazol Therapy in Autoimmune Thrombocytopenia Associated with Systemic Lupus Erythematosus
Kyung Min Kim, Byeong Seon Lee, Young Seo Park
Clin Exp Pediatr. 2001;44(11):1330-1333.   Published online November 15, 2001
Autoimmune thrombocytopenia and autoimmune hemolytic anemia occur in 10-26% of patients with systemic lupus erythematosus(SLE). These hematological manifestations may be the sole presenting sign and can precede the appearance of diagnosable SLE in 5 to 23% of cases. The conventional treatment for SLE associated with these disorders includes corticosteroid therapy and splenectomy, but autoimmune thrombocytopenia or autoimmune hemolytic anemia may...
A Case of Common Variable Immunodeficiency with Autoimmune Hemolytic Anemia
Kyung-Yil Lee, Yon-Joo Lee, Sang-Won Cha, Dong Joon Lee, Ji-Whan Han, Kyung-Tae Whang, Joong-Gon Kim
Clin Exp Pediatr. 2000;43(1):117-122.   Published online January 15, 2000
Common variable immunodeficiency(CVID) is a heterogeneous collection of disorders with hypogammaglobulinemia with recurrent bacterial infections and high incidence of autoimmune disorders as its hallmark. We report a 7-year-old girl suffering from CVID with Coombs' test positive hemolytic anemia. She had been relatively well until 23-months old when she was admitted to Taejon St. Mary' s Hospital with pneumonia 5 years ago. Afterwards, she had...
Original Article
Role of Polymorphism in HLA DQ-α and -β Chain Loci in the Pathophysiology of Autoimmune Thyroid Disease in Children with and without Turner Syndrome
Kye Shik Shim, Kyu Chul Choeh, Sei Won Yang, Sa Jun Chung, Jin Sung Lee, Kyung Tae Lee, Sung Ho Goh, Yong Sung Kim
Clin Exp Pediatr. 1999;42(7):980-990.   Published online July 15, 1999
Purpose : About 10% of girls with Turner syndrome may have autoimmune thyroid disease(AIT), but the disease's pathophysiology has not yet been elucidated. Accordingly, this study was performed to observe whether the pathogenesis of AIT in children with Turner syndrome and without Turner syndrome correlate with special loci of DQ α and β chain in HLA. Methods : Blood samples were...
Case Report
A Case of Idiopathic Hypoparathyroidism Associated with Graves' Disease
Eun Young Jang, Hwang Min Kim, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1999;42(5):716-721.   Published online May 15, 1999
Graves' disease is a relatively rare endocrinologic disorder in childhood age and often associated with other endocrinologic disorders such as type I diabetes mellitus and Addison's disease etc. Also, it is associtated with non-endocrinologic autoimmune diseases such as systemic lupus erythematosus, myasthenia gravis, idiopathic thrombocytopenic purpura, vitiligo and pernicious anemia. However, idiopathic hypoparathyroidism associated with Graves' disease is very...
A Case of Polyglandular Autoimmune Syndrome
Kyung Ah Choi, Ji Yeoun Kang, Chul Hoe Koo, Wha Mo Lee, Young Suk Jeon
Clin Exp Pediatr. 1998;41(9):1299-1303.   Published online September 15, 1998
When dysfunction of two or more endocrine glands occurs in association with circulating organ specific antibodies directed against the involved glands, the term polyglandular autoimmune(PGA) syndrome is applied. This syndrome is usually classified into three groups. The autoimmune nature of this disease has been based on the presence of lymphocytic infiltration of the affected glands, organ specific autoantibody in serum,...
Erratum
A Case of Type I Autoimmune Hepatitis in Children
Soeng Hun Kim, Su Eun Park, Jeong Hwa Choi, Jae Hong Park, Su Yung Kim
Clin Exp Pediatr. 1998;41(7):979-983.   Published online July 15, 1998
Autoimmune hepatitis is an inflammatory liver disease characterized histologically by a dense mononuclaear cell infiltration of the portal tract, serologically by the presence of non-organ and liver specific autoantibodies and increased concentrations of IgG in the absence of a known etiology. Two types of autoimmune hepatitis are classified in the peripheral blood of antinuclear antibody and/or in antismooth muscle antibody(ANA/ASMA)...
Case Report
A Case Report of Autoimmune Hepatitis Associated with Choledochal Cyst and Pancreatitis
Kyung Ran Park, Sun Young Lee, Soon Young Kim, Hyoung Shim Chang, Nam Su Back, Chong Jai Kim, Joong Gon Kim, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(8):1146-1150.   Published online August 15, 1996
Autoimmune hepatitis in children is a rare and severe inflammatory disease of unknown etiology, and progress to cirrohosis and liver failure, generally is responsive to immunosuppressive therapy. It is more prevalent in women than men, and characterized by the presence of circulating autoantibodies, a high serum globulin. Extrahepatic manifestations such as thyroiditis, ulcerative colitis, glomerulonephritis and autoimmune hemolytic anemia, are associated. We report, to our...
Original Article
Clinical Review of Positive Antinuclear Antibody(ANA) Test in Pediatric Patients
Dong-Jin Choi, Kye-Sik Shim, Hyok Choi, Byoung-Soo Cho, Sung-Ho Cha, Jin-Tae Suh
Clin Exp Pediatr. 1994;37(10):1397-1404.   Published online October 15, 1994
The antinuclear antbody (ANA) test have been used to screen the patients with systemic lupus erythematosus (SLE) and other autoimmune diseases. We had retrospectively reviewed the 263 records of pediatric patients with doing ANA tests who admitted at Department of Pediatrics, Kyung Hee University Hospital, from January 1988 to May 1993. The following results were obtained. 1) The positive rate of ANA...
Inteleukin-6 Level in Systemic Lupus Erythematosus
Young Nae Yim, Dong Soo Kim
Clin Exp Pediatr. 1993;36(3):386-393.   Published online March 15, 1993
Systemic lupus erythematousus(SLE) is a disease affecting blood vessels and connective tissue, which are damaged by deposition of pathogenic autoantibodies and immune complexes.Although a complex disease, SLE provides a number of insights into autoimmune pathogenesis. Autoimmune disease, in general, is characterized by B cell hyperactivity which results in hypergammaglobulinemia and production of a variety of autoantibodies reactive to organ-nonspecific antigens...
A Case of Autoimmune Hemolytic Anemia Associated with Chronic Hepatitis in Children.
Kwan Mo Choi, Kyeong Hun Cha, Eun Yeong Kwak, Kyung Rae Moon, Yeong Bong Park
Clin Exp Pediatr. 1990;33(9):1288-1293.   Published online September 30, 1990
Recently, We experienced a case of autoimmune hemolytic anemia in a 13 year old female patient associated with chronic hepatitis. This female child manifested severe pallor, jaundice, abdominal pain and fever. This diagnosis was made on the bases of typical clinical features, C.B.C., blood chemistry, coombs test, warm antibody, immunoelectrophoresis, peripheral blood smear and bone marrow examination. The patient improved with steroid therapy. We report this...
Case Report
A Case of Autoimmune Hemolytic Anemia Associated with Acute Viral Hepatitis B.
Chong Kyu Woo, Eun Kyung Choi, Hae Ryung Chung, Dong Heuk Keum
Clin Exp Pediatr. 1987;30(9):1034-1039.   Published online September 30, 1987
We experienced a case of autoimmune hemolytic anemia in 3-year-old male patient associated with acute viral hepatitis B. Characteristic findings of this disease are pallor, fever, tachycardia, jaundice, hemoglobinuria, of which the onset is acute or chronic. Although the pathogenic mechanism of this disease is uncertain, it can be associated with pneumonia, measles, varicella, viral hepatitis (50%) and underlying autoinunune deficiency (32%). Diagnosis...
A Case of Autoimmune Hemolytic Anemia Caused by Warm Antibody.
Sei Hee Choi, Mee Kyung Namgoong, Baek Keun Lim
Clin Exp Pediatr. 1986;29(8):898-901.   Published online August 31, 1986
We reviewed a case of autoimmune hemolytic anemia caused by warm antibody in 7years old male patient. Diagnosis was established by clinical features, physical findings, laboratory findings, such as, CBC, Coombs’ test, fragility test, and bone marrow aspiration. Literatures were briefly reviewed.
Two Cases of Autoimmune Hemolytic Anemic.
Taik Kill Kim, Soo Jee Moon, Chong Moo Park
Clin Exp Pediatr. 1981;24(1):75-79.   Published online January 15, 1981
Two cases of autoimmune hemolytic anemia, 8 years old female patient and 9 years old female patient were presented. Dignosis was established by clinical features, CBC, urine analysis, Coombs?test, fragility test, liver function study, and bone marrow aspiration. Pertinent literatures on hemolytic anemia also reviewed briefly.