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Case Report
Chronic Epstein-Barr virus infection causing both benign and malignant lymphoproliferative disorders
Yoojin Kwun, Soo-Jong Hong, Jin Seong Lee, Da Hye Son, Jong Jin Seo
Clin Exp Pediatr. 2014;57(9):420-424.   Published online September 30, 2014

The Epstein-Barr virus (EBV) is oncogenic and can transform B cells from a benign to a malignant phenotype. EBV infection is also associated with lymphoid interstitial pneumonia (LIP). Here, we report the case of a 14-year-old boy who was diagnosed with a latent EBV infection and underlying LIP, without any associated immunodeficiency. He had been EBV-seropositive for 8 years. The...

Original Article
Neurofibromatosis type 1: a single center's experience in Korea
Min Jeong Kim, Chong Kun Cheon
Clin Exp Pediatr. 2014;57(9):410-415.   Published online September 30, 2014
Purpose

Neurofibromatosis 1 (NF1) is an autosomal dominant condition caused by an NF1 gene mutation. NF1 is also a multisystem disorder that primarily affects the skin and nervous system. The goal of this study was to delineate the phenotypic characterization and assess the NF1 mutational spectrum in patients with NF1.

Methods

A total of 42 patients, 14 females and 28 males, were enrolled...

Case Report
Gastrointestinal hemangioma in childhood: a rare cause of gastrointestinal bleeding
Eon Chul Han, Soo-Hong Kim, Hyun-Young Kim, Sung-Eun Jung, Kwi-Won Park
Clin Exp Pediatr. 2014;57(5):245-249.   Published online May 31, 2014

Gastrointestinal (GI) hemangiomas are relatively rare benign vascular tumors. The choice of an appropriate diagnostic method depends on patient age, anatomic location, and presenting symptoms. However, GI hemangiomas are not a common suspected cause of GI bleeding in children because of their rarity. Based on medical history, laboratory results, and imaging study findings, the patient could be treated with either...

Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome
Hye Jin Chang, Hwa Young Kim, Jae Hong Choi, Hyun Jin Choi, Jae Sung Ko, Il Soo Ha, Hae Il Cheong, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2014;57(2):96-99.   Published online February 24, 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have...

Griscelli syndrome type 2: a novel mutation in RAB27A gene with different clinical features in 2 siblings: a diagnostic conundrum
Kirtisudha Mishra, Shilpy Singla, Suvasini Sharma, Renu Saxena, Vineeta Vijay Batra
Clin Exp Pediatr. 2014;57(2):91-95.   Published online February 24, 2014

Griscelli syndrome type 2 (GS2) is a rare autosomal recessive disease caused by mutations in the RAB27A gene. It is characterized by cutaneous hypopigmentation, immunodeficiency, and hemophagocytic lymphohistiocytosis. We describe 2 brothers who had GS2 with clinically diverse manifestations. The elder brother presented with a purely neurological picture, whereas the younger one presented with fever, pancytopenia, hepatosplenomegaly, and erythema nodosum....

Original Article
Early postictal electroencephalography and correlation with clinical findings in children with febrile seizures
Kyung A Jeong, Myung Hee Han, Eun Hye Lee, Sajun Chung
Clin Exp Pediatr. 2013;56(12):534-539.   Published online December 20, 2013
Purpose

Electroencephalography (EEG) is frequently ordered for patients with febrile seizures despite its unclear diagnostic value. We evaluated the prevalence of abnormal EEGs, the association between clinical findings and abnormal EEGs, and the predictive value of EEG for the recurrence of febrile seizures.

Methods

Data were collected on 230 children who were treated for febrile seizures at Kyung Hee University Medical Center from...

Case Report
Cystic fibrosis of pancreas and nephrotic syndrome: a rare association
Selvi Kelekçi, Müsemma Karabel, Aydın Ece, Velat Şen, Ali Güneş, İlyas Yolbaş, Cahit Şahin
Clin Exp Pediatr. 2013;56(10):456-458.   Published online October 31, 2013

Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy...

Chronic intermittent form of isovaleric aciduria in a 2-year-old boy
Jin Min Cho, Beom Hee Lee, Gu-Hwan Kim, Yoo-Mi Kim, Jin-Ho Choi, Han-Wook Yoo
Clin Exp Pediatr. 2013;56(8):351-354.   Published online August 27, 2013

Isovaleric aciduria (IVA) is caused by an autosomal recessive deficiency of isovaleryl-CoA dehydrogenase (IVD). IVA presents either in the neonatal period as an acute episode of fulminant metabolic acidosis, which may lead to coma or death, or later as a "chronic intermittent form" that is associated with developmental delays, with or without recurrent acidotic episodes during periods of stress, such...

An unusual cause of duodenal perforation due to a lollipop stick
Mi Jin Kim, Jeong Meen Seo, Yoon Lee, Yoo Min Lee, Yon Ho Choe
Clin Exp Pediatr. 2013;56(4):182-185.   Published online April 22, 2013

Children have a natural tendency to explore objects with their mouths; this can result in the swallowing of foreign objects. Most ingested foreign bodies pass uneventfully through the gastrointestinal tract. However, some foreign bodies cause obstruction or perforation of the gastrointestinal tract, requiring surgical intervention. Perforation of the gastrointestinal tract may be associated with considerable morbidity and mortality. The most...

Original Article
Vascular endothelial dysfunction after anthracyclines treatment in children with acute lymphoblastic leukemia
Woo Jung Jang, Duk Yong Choi, In-Sang Jeon
Clin Exp Pediatr. 2013;56(3):130-134.   Published online March 18, 2013
Purpose

Anthracyclines have been utilized in the treatment of children with acute lymphoblastic leukemia (ALL). Recent studies have shown that anthracyclines may induce toxicity in the vascular endothelium. This study was performed using brachial artery reactivity (BAR) to evaluate vascular endothelial function in ALL patients who were treated with anthracycline chemotherapy.

Methods

We included 21 children with ALL who received anthracycline chemotherapy and...

Immune reconstitution after allogeneic hematopoietic stem cell transplantation in children: a single institution study of 59 patients
Hyun O Kim, Hyun Jin Oh, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung, Bin Cho, Hack-Ki Kim
Clin Exp Pediatr. 2013;56(1):26-31.   Published online January 29, 2013
Purpose

Lymphocyte subset recovery is an important factor that determines the success of hematopoietic stem cell transplantation (HSCT). Temporal differences in the recovery of lymphocyte subsets and the factors influencing this recovery are important variables that affect a patient's post-transplant immune reconstitution, and therefore require investigation.

Methods

The time taken to achieve lymphocyte subset recovery and the factors influencing this recovery were investigated...

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...

Original Article
Parental concerns about their premature infants' health after discharge from the neonatal intensive care unit: a questionnaire survey for anticipated guidance in a neonatal follow-up clinic
Ji-Yun Cho, Juyoung Lee, Young Ah Youn, Soon Ju Kim, So Young Kim, In Kyung Sung
Clin Exp Pediatr. 2012;55(8):272-279.   Published online August 23, 2012
Purpose

The aim of this study was to develop an appropriate nursing information guideline according to corrected age, after investigating parents' concerns about the growth, development, and diseases of their premature infants after discharge from the neonatal intensive care unit (NICU).

Methods

The parents of premature infants (birth weight, <2,500 g; gestational age, <37 weeks) who went to a neonatal follow-up clinic after...

Evaluation of changes in random blood glucose and body mass index during and after completion of chemotherapy in children with acute lymphoblastic leukemia
Kyong-Won Bang, Soo Young Seo, Jae Wook Lee, Pil-Sang Jang, Min Ho Jung, Nack-Gyun Chung, Bin Cho, Dae-Chul Jeong, Byung Kyu Suh, Hack-Ki Kim
Clin Exp Pediatr. 2012;55(4):121-127.   Published online April 30, 2012
Purpose

Improved survival of patients with childhood acute lymphoblastic leukemia (ALL) has drawn attention to the potential for late consequences of previous treatments among survivors, including metabolic syndrome. In this study, we evaluated changes in 3 parameters, namely, random blood glucose, body mass index (BMI), and Z score for BMI (Z-BMI), in children with ALL during chemotherapy and after completion of...

Outcome of allogeneic hematopoietic stem cell transplantation for childhood acute lymphoblastic leukemia in second complete remission: a single institution study
Eun-Jung Lee, Ji Yoon Han, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim
Clin Exp Pediatr. 2012;55(3):100-106.   Published online March 16, 2012
Purpose

The survival rate for childhood acute lymphoblastic leukemia (ALL) has improved significantly. However, overall prognosis for the 20 to 25% of patients who relapse is poor, and allogeneic hematopoietic stem cell transplantation (HSCT) offers the best chance for cure. In this study, we identified significant prognostic variables by analyzing the outcomes of allogeneic HSCT in ALL patients in second complete...

Review Article
Changes in the neonatal and infant mortality rate and the causes of death in Korea
Sung-Hoon Chung, Yong-Sung Choi, Chong-Woo Bae
Clin Exp Pediatr. 2011;54(11):443-455.   Published online November 30, 2011

Neonatal mortality rate (NMR) or infant mortality rate (IMR) are the rate of deaths per 1,000 live births at which babies of either less than four weeks or of one year of age die, respectively. The NMR and IMR are commonly accepted as a measure of the general health and wellbeing of a population. Korea's NMR and IMR fell significantly...

Case Report
Testicular adrenal rest tumors in a patient with untreated congenital adrenal hyperplasia
Hye Young Jin, Jin Ho Choi, Gu Hwan Kim, Chung Sik Lee, Han Wook Yoo
Clin Exp Pediatr. 2011;54(3):137-140.   Published online March 31, 2011

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of...

Review Article
Philadelphia chromosome-positive acute lymphoblastic leukemia in childhood
Hong Hoe Koo
Clin Exp Pediatr. 2011;54(3):106-110.   Published online March 31, 2011

In pediatric patients with acute lymphoblastic leukemia (ALL), the Philadelphia chromosome translocation is uncommon, with a frequency of less than 5%. However, it is classified as a high or very high risk, and only 20-30% of Philadelphia chromosome-positive (Ph+) children with ALL are cured with chemotherapy alone. Allogeneic hematopoietic stem cell transplantation from a closely matched donor cures 60% of...

Original Article
Prognostic significance of minimal residual disease detected by a simplified flow cytometric assay during remission induction chemotherapy in children with acute lymphoblastic leukemia
Kyung Nam Koh, Meerim Park, Bo Eun Kim, Ho Joon Im, Chan-Jeoung Park, Seongsoo Jang, Hyun Sook Chi, Jong Jin Seo
Clin Exp Pediatr. 2010;53(11):957-964.   Published online November 30, 2010
Purpose

Our study attempted to determine the prognostic significance of minimal residual disease (MRD) detected by a simplified flow cytometric assay during induction chemotherapy in children with B-cell acute lymphoblastic leukemia (B-ALL).

Methods

A total of 98 patients were newly diagnosed with precursor B-ALL from June 2004 to December 2008 at the Asan Medical Center (Seoul, Korea). Of those, 37 were eligible for...

Multiple births conceived by assisted reproductive technology in Korea
Young Sil Park, Sun Hee Choi, Kye Shik Shim, Ji Young Chang, Won Ho Hahn, Yong Sung Choi, Chong-Woo Bae
Clin Exp Pediatr. 2010;53(10):880-885.   Published online October 31, 2010
Purpose

The recent trends of multiple births (MBs) conceived by assisted reproductive technology (ART) in Korea were analyzed as well as the relationship with maternal age, especially advanced maternal age.

Methods

Data were obtained from the Korean Statistical Information Service and annual ART reports from the ART committee of the Korean Society of Obstetrics and Gynecology.

Results

MBs increased from the early 1990s; there was...

Case Report
Testicular torsion in the inguinal region in an extremely low birth weight infant
Yu Jin Jung, Jae Min Chung
Clin Exp Pediatr. 2010;53(9):852-854.   Published online September 13, 2010

Testicular torsion is rare in newborn infants. However, its frequency has increased, most of which are reported in full-term infants. We diagnosed and treated testicular torsion in an extremely low birth weight infant (ELBWI). A 2×2 cm red mass was palpable in the left groin of a 24-week-old, 745 g, male newborn at 23 days of age. Left testicular torsion...

Review Article
General considerations and updates in pediatric gastrointestinal diagnostic endoscopy
Yong Joo Kim
Clin Exp Pediatr. 2010;53(9):817-823.   Published online September 13, 2010

Gastrointestinal and colonic endoscopic examinations have been performed in pediatric patients in Korea for 3 decades. Endoscopic procedures are complex and may be unsafe if special concerns are not considered. Many things have to be kept in mind before, during, and after the procedure. Gastrointestinal endoscopy is one of the most frequently performed procedure in children nowadays, Since the dimension...

Original Article
Long-term safety of PEG 4000 in children with chronic functional constipation: A biochemical perspective
Sun Hwan Bae
Clin Exp Pediatr. 2010;53(7):741-744.   Published online July 31, 2010
Purpose

To evaluate the long-term safety of polyethylene glycol (PEG) 4000 in children with constipation, particularly the biochemical aspects of safety.

Methods

Medical records were evaluated, and 100 children, who had been taking PEG 4000 for more than 6 months, and who had been under clinical and biochemical monitoring, were enrolled. Ages; 6.11±3.12 years, Duration of therapy; 16.93±7.02 months, dose of PEG 4000;...

Analysis of reports on orphan lung diseases in Korean children
Sun Jung Jang, Hyun Kyung Seo, Sung Jae Yi, Kyong Min Kim, Hye Mi Jee, Man Yong Han
Clin Exp Pediatr. 2010;53(6):711-717.   Published online June 23, 2010
Purpose

Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50...

Case Report
A case of paraneoplastic limbic encephalitis due to ovarian mature teratoma
Seong Heon Kim, hye Young Kim, Young Tak Im, Sang Ook Nam, Young Mi Kim
Clin Exp Pediatr. 2010;53(4):603-606.   Published online April 15, 2010
Paraneoplastic limbic encephalitis, a remote effect of cancer without nervous system metastasis, is rare, especially in childhood. Here, we report a case of paraneoplastic limbic encephalitis associated with an ovarian mature teratoma in an adolescent girl. The 15-year-old girl developed neuropsychiatric symptoms, memory loss, seizures, and unconsciousness. Cerebrospinal fluid analysis and brain magnetic resonance imaging (MRI) findings were normal, while...
Original Article
The natural history and prognostic factors of Graves' disease in Korean children and adolescents
Seung Min Song, Ji-Seok Youn, Jung Min Ko, Chong Kun Cheon, Jin-Ho Choi, Han Wook Yoo
Clin Exp Pediatr. 2010;53(4):585-591.   Published online April 15, 2010
Purpose : Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. Methods : One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed...
Spectrum of patients with hypermethioninemia based on neonatal screening tests over 14 years
Se Jung Oh, Yong Hee Hong, Yong Wha Lee, Dong Hwan Lee
Clin Exp Pediatr. 2010;53(3):329-334.   Published online March 15, 2010
Purpose : The neonatal screening test for homocystinuria primarily measures methionine by using a dried blood specimen. We investigated the incidence and clinical manifestations of homocystinuria, isolated hypermethioninemia, and transient hypermethioninemia among patients with hypermethioninemia on a neonatal screening test. Methods : We performed a retrospective study of 58 patients transferred to Shoonchunhyang Hospital because of hypermethioninemia on a neonatal...
Review Article
Genetic testing in clinical pediatric practice
Han Wook Yoo
Clin Exp Pediatr. 2010;53(3):273-285.   Published online March 15, 2010
Completion of the human genome project has allowed a deeper understanding of molecular pathophysiology and has provided invaluable genomic information for the diagnosis of genetic disorders. Advent of new technologies has lead to an explosion in genetic testing. However, this overwhelming stream of genetic information often misleads physicians and patients into a misguided faith in the power of genetic testing....
Case Report
A case of postoperative nasopharyngeal reflux associated with retropharyngeal lymphangioma in newborn infant
Kyo Yeon Koo, Jun Seok Lee, Soon Min Lee, Min Soo Park, Ran Namgung, Kook In Park, Chul Lee, Choon Sik Yoon, Woo Hee Jung, Hong Shik Choi
Clin Exp Pediatr. 2010;53(2):258-261.   Published online February 15, 2010
Lymphangioma is a rare benign congenital tumor of the lymphatic system, which is commonly diagnosed before 2 years of age. In the natronal report, cystic lymphangioma was usually reported as a huge translucent mass located in the head and neck area. It's occurrence in retropharyngeal space with respiratory obstruction and swallowing difficulty in neonate is extremely rare and postoperative nasopharyngeal...
A case of atypical hemolytic uremic syndrome as an early manifestation of acute lymphoblastic leukemia
Dong Kyun Han, Hee Jo Baek, Young Kuk Cho, Chan Jong Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(2):253-257.   Published online February 15, 2010
Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a...


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