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Review Article
Double-negative T cells in pediatric rheumatic diseases
Dimitri Poddighe, Tilektes Maulenkul, Kuanysh Dossybayeva, Gulsamal Zhubanova, Zaure Mukusheva, Lyudmila Akhmaltdinova
Double-negative (CD4-CD8-) T (DNT) cells have been implicated in Autoimmune Lymphoproliferative Syndrome (ALPS), where their expansion inside the circulating pool of T cells represents a diagnostic criterion. Recent experimental evidence has supported the immunomodulatory roles of DNT cells, and studies in adult patients have suggested that they may be altered in some immune-mediated conditions. This study aimed to retrieve available...
Genetics and Metabolism
Understanding the genetics of systemic lupus erythematosus using Bayesian statistics and gene network analysis
Seoung Wan Nam, Kwang Seob Lee, Jae Won Yang, Younhee Ko, Michael Eisenhut, Keum Hwa Lee, Jae Il Shin, Andreas Kronbichler
Clin Exp Pediatr. 2021;64(5):208-222.   Published online July 15, 2020
Bayesian false-discovery probability and false-positive report probability are the 2 major Bayesian methods used to evaluate noteworthiness of a genetic variant.
Application of stricter P value is needed to confirm statistical significance in meta-analyses.
Gene network analysis of noteworthy genetic variants shows a blueprint of the genetic background in complex diseases.
Case Report
Moyamoya syndrome occurred in a girl with an inactive systemic lupus erythematosus
Yun-Jin Lee, Gyu Min Yeon, Sang Ook Nam, Su Yung Kim
Clin Exp Pediatr. 2013;56(12):545-549.   Published online December 20, 2013

We report the case of a 17-year-old Korean girl with systemic lupus erythematosus (SLE) who presented with sudden weakness of the right-sided extremities and dysarthria. Oral prednisolone was being taken to control SLE. Results of clinical and laboratory examinations did not show any evidence of antiphospholipid syndrome or thromboembolic disease nor SLE activity. Cerebral angiography showed stenosis of the left...

Familial systemic lupus erythematosus in two Korean male siblings
Hyun Sik Kang, Hyun Ju Oh, Young Ree Kim, Jae-Wang Kim, Kyung-Sue Shin
Clin Exp Pediatr. 2009;52(5):611-614.   Published online May 15, 2009
Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease characterized by the production of a wide range of autoantibodies, resulting in tissue damage. Although the susceptibility to SLE has been attributed to complex interactions between genetic and environmental factors, the influence of a genetic predisposition to SLE is supported by observations of familial aggregations. Family studies have found that siblings...
Original Article
Prevalence and clinical significance of the positive antinuclear antibody in children with idiopathic thrombocytopenic purpura
So Eun Jun, Seong Sik Park, Young Tak Lim
Clin Exp Pediatr. 2008;51(11):1217-1221.   Published online November 15, 2008
Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children...
Case Report
Intestinal pseudo-obstruction as the initial presentation of systemic lupus erythematosus in a 13-year-old girl
Ky Young Cho, Tae Young Khil, Hye Mi Ahn, Sun Wha Lee, Jeong Wan Seo
Clin Exp Pediatr. 2008;51(6):655-659.   Published online June 15, 2008
Intestinal pseudo-obstruction (IPO) is a rare and poorly understood manifestation of systemic lupus erythematosus (SLE), especially in children. The characteristic clinical feature of IPO is obstruction without an identifiable obstructive lesion. The authors a 13-year-old girl whose first symptom of SLE was IPO. The patient presented with a 3-day history of nausea, bilious vomiting, abdominal distention, and no bowel movement....
Review Article
Systemic lupus erythematosus
Kwang-Nam Kim
Clin Exp Pediatr. 2007;50(12):1180-1187.   Published online December 15, 2007
Systemic lupus erythematosus (SLE) is an episodic, multi-system, autoimmune disease characterized by widespread inflammation of blood vessels and connective tissues and by the presence of antinuclear antibodies (ANAs), especially antibodies to native (double-stranded) DNA (dsDNA). Its clinical manifestations are extremely variable, and its natural history is unpredictable. Untreated, SLE is often progressive and has a significant fatality rate. The most...
Original Article
A clinical study of systemic lupus erythematosus in children
Ji Tae Kim, Young Mee Nam, Jae Seung Lee, Dong Soo Kim
Clin Exp Pediatr. 2007;50(1):74-78.   Published online January 15, 2007
Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance...
Case Report
A Case of Kikuchi-Fujimoto Disease, Subsequently Evolving to Systemic Lupus Eyrthematosus
Soon Hwa Yoon, Myoung Hoon Song, Seon Hee Shin, Sung Koo Kim, Kon Hee Lee, Hae Sun Yoon, Young Ok Jung, Hye Kyung Ahn
Clin Exp Pediatr. 2004;47(8):904-907.   Published online August 15, 2004
Kikuchi-Fujimoto disease(KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limited systemic illness and it has the pathognomonic histological appearance of lymph nodes. KFD is rarely associated with systemic lupus erythematosus (SLE). The diagnosis of KFD can precede, postdate or coincide with the diagnosis of SLE. Our case describes a young woman, originally diagnosed as having Kikuchi's disease by lymph...
Original Article
Association of Angiotensin-converting Enzyme Gene Polymorphism with the Disease Activity of Systemic Lupus Erythematosus in Korean Children
Kyung-Sue Shin
Clin Exp Pediatr. 2004;47(6):672-676.   Published online June 15, 2004
Purpose : Several recent studies have shown a significant association of angiotensin converting enzyme(ACE) gene polymorphism with systemic lupus erythematosus(SLE). The association has not been consistently confirmed; moreover, the association of ACE genotype with SLE in children has never been evaluated. The aim of this study is to evaluate the association of ACE gene polymorphism with SLE in Korean children. Methods...
Case Report
A Case of Hemophagocytic Lymphohistiocytosis in a Child with Systemic Lupus Erythematosus
Ja Young Hwang, Suk Man No, Jin Lee, Pil Sang Jang, Young Hoon Kim, Jin Tack Kim, Joon Sung Lee
Clin Exp Pediatr. 2003;46(10):1029-1031.   Published online October 15, 2003
Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment...
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...
Original Article
A Clinico-pathologic Study of Childhood Lupus Nephritis
Youn-Sook Rho, Sung-Hoo Cha, Byoung-Soo Cho
Clin Exp Pediatr. 2000;43(2):236-240.   Published online February 15, 2000
Purpose : Systemic lupus erythematosus(SLE) is a disease of immunologic origin, affecting multiple organs including joints, skin, kidneys, nervous system, heart and blood, with rare involvement of gut and liver. The incidence of renal disease in children with SLE was approximately 70 percent and tended to be more severe than in adults. Accordingly we have retrospectively studied on the prognostic...
Erratum
A Case of Pancreatitis associated with Systemic Lupus Erythematosus
Su Eun Park, Ju Suk Lee, Sang Ook Park, Jae Hong Park, Su Young Kim
Clin Exp Pediatr. 1998;41(7):989-993.   Published online July 15, 1998
Systemic lupus erythematosus(SLE), an autoimmune disease with multisystemic involvement, has been reported to be associated with a number of gastrointestinal complications. But pancreatitis is an unusual complication of SLE, occuring in only 3-4% of lupus cohort. Multiple mechanisms contributing to pancreatitis associated with SLE have been demonstrated which include vasculitis, necrotizing pancreatitis, corticosteroid administration, and vascular thrombosis. We experienced a...
Original Article
A Case of Successful Treatment of Pulmonary Alveolar Hemorrhage with Plasmapheresis in Child with Systemic Lupus Erythematosus
Jae Hong Min, Kyung Hoon Paek, Kyung Mi Park, Jin Soon Hwang, Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Kyou Seup Han, Yong Choi
Clin Exp Pediatr. 1998;41(7):974-978.   Published online July 15, 1998
Seoul National University College of Medicine, Seoul, Korea Pulmonary hemorrhage is a rare but possibly fatal complication of systemic lupus erythematosus(SLE). We report a case of massive pulmonary hemorrhage in a 14-year-old boy recently diagnosed as SLE. He developed massive pulmonary hemorrhage during the courses of i.v. methylprednisolone pulse therapy, and did not respond to i.v. cyclophosphamide. However, he rapidly...
Case Report
A Case of Transverse Myelitis associated with Systemic Lupus Erythematosus
Yeong Sim Han, Sun Yeong Park, Min Seop Song, Yeong Sim Han
Clin Exp Pediatr. 1998;41(6):831-836.   Published online June 15, 1998
Transverse myelitis is a rare complication of systemic lupus erythematosus(SLE) and its prognosis is very poor including death or severe neurologic sequelae. We report a 14-year-old girl with transverse myelitis who was not exactly diagnosed as SLE before the onset of neurologic symptoms. Transverse myelitis was diagnosed based on the clinical presentations, cerebrospinal fluid analysis and MRI findings. We employed aggressive treatment with pulse...
Original Article
A Clinical Study of Childhood Systemic Lupus Erythematosus
Young-Jun Kim, Young Don Kim, Jae Hong Park, Su Young Kim, Hee-Ju Park
Clin Exp Pediatr. 1994;37(9):1235-1244.   Published online September 15, 1994
To Evaluate the clinical characteristics of childhood-onset systemic lupus erythemoatosus (SLE) and analyse the factors related to outcome of renal function in lupus nephritis, we reviewed medical records of 18 cases of SLE diagnosed at the Department of Pediatrics, Pusan Nationsl University Hospital from January 1981 to December 1990. The results were as was 1:2.6 1)Male...
A Clinical Observation in 14 Children with SLE
Jin Nyoung Park, Jai Kyoung Choi, Ha Baik Lee, Hahng Lee
Clin Exp Pediatr. 1994;37(4):510-519.   Published online April 15, 1994
To elucidate the clinical characteristics of childhood onset systemic lupus erythematosus (SLE), we carried out a retrospective reivew on medical records of fourteen patients who were diagnosed as SLE on the base of 뱓he 1982 revised criteria for the SLE?(by american Rheumatism Association) at the Department of pediatrics, Hanyang University Hospital from January 1980 through August 1992. The results were summerized...
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...
Four Cases of Childhood Systemic Lupus Erythematosus.
Jun Ah Jeong, Young Mi Kim, Eun Sook Suh, Sung Ho Kim, Chin Moo Kang, Sang Sook Lee, Chai Hong Chung
Clin Exp Pediatr. 1990;33(2):264-273.   Published online February 28, 1990
Systemic Lupus Erythematosus (SLE) is a rare entity in pediatric age and prognosis is poor due to higher incidence of renal involvement in children. Authors experienced four cases of SLE, aged between 9-13 years. All cases were female. The diagnosis was based on “The 1982 revised criteria for SLE (by American Rheumatism Association). All cases had fever on admission and the butterfly facial rash was...
A Case of Systemic Lupus Erythematosus Accompanied with Nephritis and Arteritis.
Oh Kyun Kwon, Hong Yang Khil, Jae Ho Lee, Young Hun Chung
Clin Exp Pediatr. 1987;30(11):1311-1317.   Published online November 30, 1987
The systemic lupus erythematosus (SLE) is a chronic inflammatory disease involving skin, kidney, serous membrane, nervous system and other organs. We experienced a case of SLE accompanied with nephritis and arteritis on the region of the left thigh, diagnosed by clinical, serologic, immunologic, radiologic and pathologic evaluations. A 15 years old female patient was admitted to pediatric ward due to sustained pain on left...
A Clinical Observation on Systemic Lupus Erythematosus in Children.
Han Wook Yoo, Hae Il Cheong, Hoan Jong Lee, Yong Choi, Kwang Wook Ko, Yong Il Kim
Clin Exp Pediatr. 1987;30(5):527-535.   Published online May 31, 1987
To elucidate the clinical characteristics of childhood onset systemic lupus erythematosus (SLE), we carried out a retrospective review on medical records of sixteen patients who were diagnosed as SLE on the base of “the 1982 revised criteria for the SLE” (by American Rheumatism Association) at the Department of Pediatrics, Seoul National University Children’s Hospital between January 1976 and July 1986. The results were summarized...
Case Report
A Case of systemic Lupus Erythematosis.
Keum Yang Son, Young Wook Kim, Ki Bok Kim, Jang Sihn Sohn
Clin Exp Pediatr. 1985;28(12):1250-1255.   Published online December 31, 1985
A Case of systemic lupus erythematosus in a 15-year old girl was presented with a brief review of the literature. She was admitted with the chief complaints of fever, anorexia, general malaise and hematuria. Ths diagnosis was made on the basis of typical clinical features and characteristic laboratory findings. Inspite of every effort including prednisolone and imuran therapy, the patient...
A Case of Systemic Lupus Erythematosus.
Dong Hwan Cho, Sook Kyeung Zung, Zun Ho Kim, Byung Yun Chung, Won Jae Park
Clin Exp Pediatr. 1982;25(11):1185-1189.   Published online November 30, 1982
A Case of Systemic Lupus Erythematosus was presented concerning the clinical wanifesta- tions and laboratory findings with reference to literatures. The patient was a 15-years old Korean High school girl who was admitted to this hospital on May, 19th, 1981, with the chief complaints of pain of both knee joints, skin rashes, paticularly characteristic butterfly rashes on malar area of face and fever. She was...