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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...
Original Article
Endocrinology
Screening of SHOX gene sequence variants in Saudi Arabian children with idiopathic short stature
Abdulla A. Alharthi, Ehab I. El-Hallous, Iman M. Talaat, Hamed A. Alghamdi, Matar I. Almalki, Ahmed Gaber
Clin Exp Pediatr. 2017;60(10):327-332.   Published online October 20, 2017
Purpose

Short stature affects approximately 2%–3% of children, representing one of the most frequent disorders for which clinical attention is sought during childhood. Despite assumed genetic heterogeneity, mutations or deletions in the short stature homeobox-containing gene (SHOX) are frequently detected in subjects with short stature. Idiopathic short stature (ISS) refers to patients with short stature for various unknown reasons. The goal...

Case Report
Idiopathic acute eosinophilic pneumonia in a 14-month-old girl
Ha Neul Park, Bo Hyun Chung, Jung Eun Pyun, Kwang Chul Lee, Ji Tae Choung, Choon Hak Lim, Young Yoo
Clin Exp Pediatr. 2013;56(1):37-41.   Published online January 29, 2013

Idiopathic acute eosinophilic pneumonia (IAEP), characterized by acute febrile respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, is rarely reported in children. Diagnosis is based on an association of characteristic features including acute respiratory failure with fever, bilateral infiltrates on the chest X-ray, severe hypoxemia and bronchoalveolar lavage fluid >25% eosinophils or a predominant eosinophilic infiltrate in lung...

Review Article
Juvenile idiopathic arthritis: Diagnosis and differential diagnosis
Ki Hwan Kim, Dong Soo Kim
Clin Exp Pediatr. 2010;53(11):931-935.   Published online November 30, 2010

Juvenile idiopathic arthritis (JIA) is comprised of a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and has symptoms lasting at least 6 weeks. The previous classification of JIA included seven different categories, whereas its current classification was compiled by the International League of the Association for Rheumatology,...

Original Article
Analysis of cytosine adenine repeat polymorphism of the IGF-I promoter gene in children with idiopathic short stature
Jae Hoon Moon, Woo Yeong Chun
Clin Exp Pediatr. 2009;52(3):356-363.   Published online March 15, 2009
Purpose : A polymorphism in the IGF-I gene promoter region is known to be associated with serum IGF-I levels, birth weight, and body length, suggesting that IGF-I gene polymorphism might influence postnatal growth. The present study aimed to investigate the role of this polymorphic cytosine-adenine (CA) repeat of the IGF-I gene in children with idiopathic short stature. Methods : The...
Case Report
A case of idiopathic pulmonary hemosiderosis with seasonal recurrence
Ga Young Kwak, Na Young Lee, Moon Hee Lee, Soo Young Lee, Seung Yun Chung, Jin Han Kang, Dae Chul Jeong
Clin Exp Pediatr. 2009;52(2):256-260.   Published online February 15, 2009
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been...
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
Change of neutrophil count after treatment of intravenous immunoglobulin in children with idiopathic thrombocytopenic purpura
Jun Young Park, Ji Ae Park, Seong Shik Park, Young Tak Lim
Clin Exp Pediatr. 2008;51(2):204-208.   Published online February 15, 2008
Purpose : The aim of this study was to investigate the incidence and course of neutropenia following intravenous immunoglobulin (IVIG) therapy in children with idiopathic thrombocytopenic purpura (ITP). Methods : From January 2001 to June 2006, fifty-four patients with ITP were enrolled in this study. Forty-two of 54 patients were treated with IVIG, while the other 12 were treated with...
Original Article
Change of absolute neutrophil count after intravenous immunoglobulin administration for the children with idiopathic thrombocytopenic purpura
Hyun Jung Shin, In Kug Bang, Byung Kyu Choe, Jin-Bok Hwang, Jun Sik Kim, Heung Sik Kim
Clin Exp Pediatr. 2007;50(10):982-986.   Published online October 15, 2007
Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels...
Case Report
A case of idiopathic renal hypouricemia
Moon Hee Han, Sang Uk Park, Deok-Soo Kim, Jae Won Shim, Jung Yeon Shim, Hye Lym Jung, Moon Soo Park
Clin Exp Pediatr. 2007;50(5):489-492.   Published online May 15, 2007
Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. This disease usually produces no symptoms, but hematuria, uric acid nephrolithiasis or acute renal failure may develop. A defect in the SLC22A12 gene, which encodes the human urate transporter, is the known major cause of this disorder. We describe a 10-month-old boy with idiopathic renal...
Review Article
Giant platelet syndrome
Hoon Kook
Clin Exp Pediatr. 2006;49(8):833-838.   Published online August 15, 2006
Giant platelet syndrome is a group of unique disorders characterized by the presence of abnormally large platelets, and usually accompanied by thrombocytopenia. Most cases of giant platelets are encountered in idiopathic thrombocytopenic purpura(ITP). In contrast, inherited giant platelet disorders, a group of heterogeneous diseases, are rare. Bernard-Soulier syndrome and its variants, and MYH9 related diseases have been defined at the...
Immune thrombocytopenic purpura(ITP)
Hee Young Shin
Clin Exp Pediatr. 2006;49(8):830-832.   Published online August 15, 2006
Immune thrombocytopenic purpura(ITP) is an autoimmune disease characterized by increased peripheral platelet destruction due to antibody to platelet, which results in thrombocytopenia and cutaneous or mucosal bleeding. Bleeding generally occurs when platelet counts fall to less than 20,000/µL. Children affected with ITP are usually healthy prior to the onset of the disease and typically present suddenly after a viral infection...
Original Article
Effects of growth hormone treatment on glucose metabolism in idiopathic short stature
Seung Yeon Kwon, Duk-Hee Kim, Ho-Seong Kim
Clin Exp Pediatr. 2006;49(6):665-671.   Published online June 15, 2006
Purpose : To study the effects of growth hormone(GH) treatment on glucose metabolism and insulin resistance in children with idiopathic short stature(ISS). Methods : Glucose and insulin concentrations were measured during oral glucose tolerance test (OGTT) before and after GH treatment(0.6-0.7 IU/kg/week) in 20 patients with ISS. Insulin resistance was assessed by homeostasis model assessment(HOMA). Results : During OGTT, the mean...
Effects of Growth Hormone Therapy in Children with Idiopathic Short Stature
Kyong A Lee, Heon Seok Han
Clin Exp Pediatr. 2005;48(8):865-870.   Published online August 15, 2005
Purpose : The use of growth hormone(GH) to promote growth in normal short children without classical GH deficiency is controversial. Numerous foreign studies have shown the effects of GH therapy in children with idiopathic short stature(ISS) whereas few has been interested in Korea. Therefore, this study is designed to investigate the effects of GH therapy on ISS by observing correlations...
Pulmonary Function of Patients with Adolescent Idiopathic Scoliosis
Yu Sok Han, Hyun Ah Kim, Uee Ryoung Chung
Clin Exp Pediatr. 2004;47(10):1076-1080.   Published online October 15, 2004
Purpose : To evaluate the patterns of preoperative pulmonary function tests(PFTs) and their correlation with postoperative pulmonary impairment in patients with adolescent idiopathic scoliosis(AIS), who had orthopaedic operation with or without rib resection thoracoplasty. Methods : The case records and radiographs of 79 patients(12, boys and 67, girls) of a mean age of 13.3 years(7-15 years) with AIS who underwent orthopaedic...
Case Report
A Case of Teratocarcinoma with Central Diabetes Insipidus
Jong Hoon Kim, In Seok Lim, Eung Sang Choi, Byoung Hoon Yoo
Clin Exp Pediatr. 2004;47(4):453-457.   Published online April 15, 2004
Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in...
Original Article
Study on the Correlation between Clinical Classification according to Hemorrhagic Symptoms and Platelet Counts in Childhood Idiopathic Thrombocytopenic Purpura
Ho Il Bang, Sae Ron Shin, Du Young Choi
Clin Exp Pediatr. 2004;47(4):412-416.   Published online April 15, 2004
Purpose : Idiopathic thrombocytopenic purpura(ITP) is a relatively common hematologic disease in children. The optimal strategy for treating ITP during childhood is a matter of controversy. In general, platelet count has been the primary, if not the sole measure, on which treatment decisions have been made and outcomes determined. In this study, we tried to find out the correlation between...
The Effect of Growth Hormone on Patients with Growth Hormone Deficiency and Idiopathic Short Stature
Jeong Cheol Kang, Yoon Suk Choi, In Kyong Choi, Ho Sung Kim, Duk Hee Kim
Clin Exp Pediatr. 2004;47(3):310-318.   Published online March 15, 2004
Purpose : This study was designed to evaluate the effect of growth hormones on children with growth hormone deficiency(GHD) or idiopathic short stature(ISS). Methods : Between January 1988 to July 2003, 45 patients(M26, F19) with GHD and 24 patients (M13, F11) with ISS were enrolled in this study. Height standard deviation score(Ht SDS) for chronological age(CA) and Ht SDS for bone...
Overnight Growth Hormone Secretions and Sleep Patterns in Idiopathic Short Stature Children
Sang Young Seo, Kee Hyoung Lee, Baik Lin Eun, Chang Sung Sohn, Young Chang Tockgo, Chol Shin, Baek-Hyun Kim
Clin Exp Pediatr. 2003;46(4):363-369.   Published online April 15, 2003
Purpose : Pharmacologic provocation test of growth hormone(GH) is a non-physiologic method and has several limitations for diagnosing growth hormone(GH) deficiency. Spontaneous GH release studies could be important in understanding the pathophysiology of children with poor growth but normal responses to GH provocation tests. Also, the relationship between nocturnal GH secretions and sleep patterns in short stature children is poorly...
Comparison of Effectiveness between Two Different Protocols of Treatment of IV γ-globulin in Idiopathic Thrombocytopenic Purpura
Jae Ho Jang, Moonsouk Lee, Mira Park, Kyuchul Choeh
Clin Exp Pediatr. 2003;46(4):358-362.   Published online April 15, 2003
Purpose : Several methods of IV γ-globulin(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. Methods : Forty seven...
Changes in Mean Platelet Volume and Platelet Distribution Width after Therapy in Childhood Idiopathic Thrombocytopenic Purpura
Jong Tai Kim, Kyung Won Lee, Soon Nam Kim, Moon Kyu Kim
Clin Exp Pediatr. 2002;45(4):505-511.   Published online April 15, 2002
Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may...
Clinical Study of the Correlation of Tumor Necrosis Factor Alpha and the Proteinuria of Henoch-Schönlein Nephritis and Idiopathic Nephrotic Syndrome
Dong-Ho Jeong, Jeong-Hyun Park, Hye-Cheon Jeong, Hyun-Hoe Koo, Jun-Ho Lee, Tae-Sun Ha
Clin Exp Pediatr. 2002;45(2):240-246.   Published online February 15, 2002
Purpose : It is not clear that the development of glomerular injury and aggravation by tumor necrosis factor alpha(TNF-α) is related to intrarenal or serum concentration of TNF-α. So, we studied the relationship between the concentration of TNF-α and aggravation of glomerular damage in the Henoch-Schönlein nephritis(HSN) and idiopathic nephrotic syndrome(INS). Methods : We collected the sera and urines of...
Case Report
Two Cases of Growth Hormone Therapy in Idiopathic Dilated Cardiomyopath
Jeong Won Shin, Hong Ryang Kil, Jae Hong Yu
Clin Exp Pediatr. 2001;44(8):942-947.   Published online August 15, 2001
Growth hormone receptor gene is expressed in the myocardium and growth hormone(GH) administration has been shown to increase cardiac insulin-like growth factor-I(IGF-1) contents. The cardiac myocyte express IGF-I receptors and IGF-I promotes cardiac hypertrophy. Furthermore GH therapy has been reported to have beneficial effects in the treatment of dilated cardiomyopathy in adults. We have studied the effect of short term...
Original Article
Growth Hormone Receptor Mutation and Partial Growth Hormone Insensitivity in Children with Idiopathic Short Stature
So Chung Chung, Ran Namgung, Duk Hee Kim
Clin Exp Pediatr. 2001;44(8):922-935.   Published online August 15, 2001
Purpose : Children with idiopathic short stature(ISS) are classified on the basis of exclusion criteria. Short stature with normal or increased circulating growth hormone(GH) and low IGF-I levels indicates that partial growth hormone insensitivity(GHI) may play a role in ISS. The present study was performed to investigate whether partial GHI is observed in children with idiopathic short stature and whether...
Case Report
Two Cases of Congenital Paraesophageal Hiatal Hernia in Infancy
Ji Yeon Hong, Kih Yeon Song, Woo Ki Lee, Kwang Woo Kim, Jong Gon Ha, Soon Ok Choi
Clin Exp Pediatr. 2000;43(12):1613-1620.   Published online December 15, 2000
Herniation of the stomach through the esophageal hiatus into the posterior mediastinum is a commom affliction of humans. The incidence of hiatal hernia is difficult to determine because of the absence of symptoms in a large number of patients. Hiatal hernias are classified into two major types; type I sliding hiatal hernia and type II paraesophageal hiatal hernia. Sliding hernia...
Original Article
Study of Autoantibodies Against Platelet GP IIb/IIIa and GP Ib/IX in Childhood Chronic Idiopathic Thrombocytopenic Purpura
Chanwook Woo, Junghwa Lee, Kwangchul Lee, Soonkyum Kim
Clin Exp Pediatr. 2000;43(3):395-401.   Published online March 15, 2000
Purpose : Chronic idiopathic thrombocytopenic purpura(CITP) is an autoimmune disease caused by autoantibodies reacting to certain antigens, and platelet glycoprotein(GP) IIb/IIIa and GP Ib/IX complexes are thought to be some of those antigens. However, the clinical significance of anti-GP autoantibodies in CITP patients is unknown. In this study, we investigated the clinical correlation between the presence of circulating autoantibodies against...
Case Report
Medical Treatment of Infantile Hypertrophic Pyloric Stenosis Using Intravenous Atropine Sulfate
Kun Hee Lim, Son Moon Shin, Han Ku Moon, Mi Soo Hwang
Clin Exp Pediatr. 1999;42(6):874-878.   Published online June 15, 1999
Infantile hypertrophic pyloric stenosis(IHPS) is one of the most common causes of nonbilious vomiting in early infancy, and is caused by hypertrophied pyloric muscle but its exact etiology and pathogenesis are still unknown. Fredet-Ramstedt pyloromyotomy has been accepted as the treatment of choice for IHPS. Atropine is a cholinergic blocking agent with potent antimuscarinic activity that decreases peristaltic contractions...
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...
Original Article
Congenital Esophageal Stenosis : with Special Reference to Diagnosis and Postoperative Complications
Ju Young Jang, Jae Seong Ko, Kwi Won Park, Woo Seon Kim, In Won Kim, Ja Jun Jang, Jeong Kee Seo,
Clin Exp Pediatr. 1999;42(4):535-544.   Published online April 15, 1999
Purpose : Congenital esophageal stenosis(CES) is one of the rare causes of recurrent vomiting during infancy and childhood. We studied the diagnostic and therapeutic tools and postoperative complications for early diagnosis and adequate management of CES. Methods : Fourteen cases of CES were evaluated for clinical manifestations, findings of esophagogram and esophagoscopy, classification of pathologic findings and postoperative complications. Results : Most...
Correlation Between Clinical and Pathological Prognositic Factors of IgA Nephropathy in Children
Hwang Jae Yoo, Bon Su Ku, Young Tak Lim, Su Yung Kim
Clin Exp Pediatr. 1998;41(8):1093-1101.   Published online August 15, 1998
Purpose : Clinical and pathological prognostic factors of idiopathic IgA nephropathy have been reported, but mostly in adults and a few in children. Especially studies about correlation between those factors are very rare. Methods : We studied 58 children patients who were hospitalized to our clinics and diagnosed as IgA nephropathy by renal biopsy from Jan. 1989 to Jun 1996. They...