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Case Report
A case of hemolytic uremic syndrome preceded by intussusception
Eun Young Ko, Joo Young Kim, Hye Jin Lee, Hyun Seung Lee, Ji Whan Han, Young Hoon Kim, Jin Tack Kim, Hae Il Cheong, Pil Sang Jang
Clin Exp Pediatr. 2011;54(4):176-178.   Published online April 30, 2011

Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in young children. It is classically characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and uremia. Further, not only is intussusception one of the differential diagnoses of HUS but it may also become a complication during disease progression. We report a case of HUS preceded by intussusception...

Original Article
Selective embolization of the internal iliac arteries for the treatment of intractable hemorrhage in children with malignancies
Sul Hee Bae, Dong Kyun Han, Hee Jo Baek, Sun Ju Park, Nam Kyu Chang, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2011;54(4):169-175.   Published online April 30, 2011
Purpose

Acute internal hemorrhage is an occasionally life-threatening complication in pediatric cancer patients. Many therapeutic approaches have been used to control bleeding with various degrees of success. In this study, we evaluated the efficacy of selective internal iliac artery embolization for controlling acute intractable bleeding in children with malignancies.

Methods

We retrospectively evaluated the cases of 6 children with various malignancies (acute lymphoblastic...

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...

Recent advances in the diagnosis and management of childhood acute promyelocytic leukemia
Eun Sun Yoo
Clin Exp Pediatr. 2011;54(3):95-105.   Published online March 31, 2011

Since the successful introduction of all-trans-retinoic acid (ATRA) and its combination with anthracycline-containing chemotherapy, the prognosis for acute promyelocytic leukemia (APL) has markedly improved. With ATRA and anthracycline-based-chemotherapy, the complete remission rate is greater than 90%, and the long-term survival rate is 70-89%. Moreover, arsenic trioxide (ATO), which was introduced for APL treatment in 1994, resulted in excellent remission rates...

Case Report
A case of pseudohypoaldosteronism type 1 with a mutation in the mineralocorticoid receptor gene
Se Eun Lee, Yun Hye Jung, Kyoung Hee Han, Hyun Kyung Lee, Hee Gyung Kang, Il Soo Ha, Yong Choi, Hae Il Cheong
Clin Exp Pediatr. 2011;54(2):90-93.   Published online February 28, 2011

Pseudohypoaldosteronism type 1 (PHA1) is a rare form of mineralocorticoid resistance characterized in newborns by salt wasting with dehydration, hyperkalemia and failure to thrive. This disease is heterogeneous in etiology and includes autosomal dominant PHA1 owing to mutations of the NR3C2 gene encoding the mineralocorticoid receptor, autosomal recessive PHA1 due to mutations of the epithelial sodium channel (ENaC) gene, and...

Traumatic ventricular septal defect in a 4-year-old boy after blunt chest injury
Yun Mi Kim, Byung Won Yoo, Jae Young Choi, Jun Hee Sul, Young Hwan Park
Clin Exp Pediatr. 2011;54(2):86-89.   Published online February 28, 2011

Traumatic ventricular septal defect (VSD) resulting from blunt chest injury is a very rare event. The mechanisms of traumatic VSD have been of little concern to dateuntil now, but two dominant theories have been described. In one, the rupture occurs due to acute compression of the heart; in the other, it is due to myocardial infarction of the septum. The...

Review Article
Systematic review of the clinical and genetic aspects of Prader-Willi syndrome
Dong Kyu Jin
Clin Exp Pediatr. 2011;54(2):55-63.   Published online February 28, 2011

Prader-Willi syndrome (PWS) is a complex multisystem genetic disorder that is caused by the lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. This syndrome has a characteristic phenotype including severe neonatal hypotonia, early-onset hyperphagia, development of morbid obesity, short stature, hypogonadism, learning disabilities, behavioral problems, and psychiatric problems. PWS is an example of a genetic condition caused...

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...

The role of inhaled and/or nasal corticosteroids on the bronchodilator response
Ju Kyung Lee, Dong In Suh, Young Yull Koh
Clin Exp Pediatr. 2010;53(11):951-956.   Published online November 30, 2010
Purpose

To compare the profiles of the bronchodilator response (BDR) among children with asthma and/or allergic rhinitis (AR) and to determine whether BDR in these children is reduced by treatment with inhaled and/or nasal corticosteroid.

Methods

Sixty-eight children with asthma (mean age, 10.9 years), 45 children with comorbid asthma and AR (mean age, 10.5 years), and 44 children with AR alone (mean age,...

Review Article
Treatment of juvenile rheumatoid arthritis
Kwang Nam Kim
Clin Exp Pediatr. 2010;53(11):936-941.   Published online November 30, 2010

The systematic approach to pharmacologic treatment is typically to begin with the safest, simplest, and most conservative measures. It has been realized that the more rapidly inflammation is under control, the less likely it is that there will be permanent sequelae. Nonsteroidal anti-inflammatory drugs (NSAIDs) are the mainstay of initial treatment for inflammation. In addition, the slow-acting antirheumatic drugs (SAARDs)...

Original Article
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
Transient splenial lesion of the corpus callosum in a case of benign convulsion associated with rotaviral gastroenteritis
Yoon Young Jang, Kye Hyang Lee
Clin Exp Pediatr. 2010;53(9):859-862.   Published online September 13, 2010

Transient magnetic resonance (MR) signal changes in the splenium of the corpus callosum (SCC) arise from many different conditions, including encephalopathy or encephalitis caused by infection, seizures, metabolic derangements, and asphyxia. Few case reports exist on reversible SCC lesions associated with rotavirus infection. A benign convulsion with mild gastroenteritis (CwG) is frequently associated with rotaviral infections. This entity is characterized...

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...

Original Article
Korean speech sound development in children from bilingual Japanese-Korean environments
Jeoung Suk Kim, Jun Ho Lee, Yoon Mi Choi, Hyun Gi Kim, Sung Hwan Kim, Min Kyung Lee, Sun Jun Kim
Clin Exp Pediatr. 2010;53(9):834-839.   Published online September 13, 2010
Purpose

This study investigates Korean speech sound development, including articulatory error patterns, among the Japanese-Korean children whose mothers are Japanese immigrants to Korea.

Methods

The subjects were 28 Japanese-Korean children with normal development born to Japanese women immigrants who lived in Jeonbuk province, Korea. They were assessed through Computerized Speech Lab 4500. The control group consisted of 15 Korean children who lived in...

Needle aspiration as therapeutic management for suppurative cervical lymphadenitis in children
Mee Young Baek, Kyung Hee Park, Ju Hee We, Su Eun Park
Clin Exp Pediatr. 2010;53(8):801-804.   Published online August 31, 2010
Purpose

This study aimed to evaluate the usefulness of the needle aspiration alternative to open surgical drainage of children with suppurative cervical lymphadenitis requiring surgical drainage.

Methods

From January 1998 to June 2008, we retrospectively reviewed 38 children treated with needle aspiration as management with suppurative cervical lymphadenitis instead of open surgical drainage.

Results

All 38 children underwent only 1 puncture. Two patients (5.2%) out...

Effect of p16 on glucocorticoid response in a B-cell lymphoblast cell line
Sun-Young Kim, Kyung-Yil Lee, Dae-Chul Jeong, Hak-Ki Kim
Clin Exp Pediatr. 2010;53(7):753-758.   Published online July 31, 2010
Purpose

It has been suggested that p16 has a role in glucocorticoid (GC)-related apoptosis in leukemic cells, but the exact mechanisms have yet to be clarified. We evaluated the relationship between the GC response and p16 expression in a lymphoma cell line.

Methods

We used p16 siRNA transfection to construct p16-inactivated cells by using the B-cell lymphoblast cell line NC-37. We compared glucocorticoid...

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...

Review Article
Pulmonary hypertension in infants with bronchopulmonary dysplasia
Gi Beom Kim
Clin Exp Pediatr. 2010;53(6):688-693.   Published online June 23, 2010

An increase in the number of preterm infants and a decrease in the gestational age at birth have resulted in an increase in the number of patients with significant bronchopulmonary dysplasia (BPD) and secondary pulmonary hypertension (PH). PH contributes significantly to the high morbidity and mortality in the BPD patients. Therefore, regular monitoring for PH by using echocardiography and B-type...

Case Report
A case of pyogenic liver abscess in a 10-year-old girl
Jung Lim Byun, Sun Hwan Bae, Sang Woo Park
Clin Exp Pediatr. 2010;53(5):666-668.   Published online May 31, 2010

Pyogenic liver abscesses are rare in children. In pediatric patients, altered host defences seem to play an important role. However, pyogenic liver abscess also occurs in healthy children. We experienced a case of pyogenic liver abscess in a healthy immunocompetent 10-year-old-girl. The patient presented two distinct abscesses: one subphrenic and the other intrahepatic. The intrahepatic abscess resolved with percutaneous drainage...

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...
Phonetic analysis of Korean elementary students who had overseas study at early ages
Mee Heun Ryu, Chang Woo Lee
Clin Exp Pediatr. 2010;53(4):579-584.   Published online April 15, 2010
Purpose : The number of the repatriated Korean students who had overseas study at early ages is increasing. They received foreign education, they can speak international languages, but they have many difficulties in articulation and intonation of the Korean language. This study aims to measure closure and aspiration duration, length of consonants, length of subsequent vowels, and ratio of consonants...
Change of interictal epileptiform discharges after antiepiletic drug treatment in childhood epilepsy
Mun Ju Kim, Sang Ook Nam
Clin Exp Pediatr. 2010;53(4):560-564.   Published online April 15, 2010
Purpose : Electroencephalography (EEG) findings can play a critical role in a variety of decisions, including initiation and withdrawal of antiepileptic drugs (AEDs) therapy. Interictal epileptiform discharges (IEDs) are predictor of recurrent seizures. We investigated IEDs in EEG after AED therapy and related factors in epileptic children. Methods : The subjects were 257 children [151 males and 106 females; age,...
Comparison of total body irradiation-based or non-total body irradiation-based conditioning regimens for allogeneic stem cell transplantation in pediatric leukemia patients
Sang Jeong Kim, Dong Kyun Han, Hee Jo Baek, Dong Yeon Kim, Taek Keun Nam, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2010;53(4):538-547.   Published online April 15, 2010
Purpose : This study aims to compare the outcome of total body irradiation (TBI)- or non-TBI-containing conditioning regimens for leukemia in children. Methods : We retrospectively evaluated 77 children conditioned with TBI (n=40) or non-TBI (n=37) regimens, transplanted at Chonnam National University Hospital between January 1996 and December 2007. The type of transplantation, disease status at the time of transplant, conditioning regimen,...
Case Report
A case of steroid-induced psychosis in a child having nephrotic syndrome with toxic epidermal necrolysis
Sae Yoon Kim, Jae Min Lee, Yong Hoom Park
Clin Exp Pediatr. 2010;53(3):437-441.   Published online March 15, 2010
Toxic epidermal necrolysis (TEN) and Stevens–Johnson syndrome (SJS) are rare, life-threatening mucocutaneous diseases, usually attributable to drugs and infections. Corticosteroids have been used in the management of TEN for the last 30 years. This remains controversial and is still much debated. TEN can occur despite administration of high doses of systemic corticosteroids. The psychiatric side effects of corticosteroids can include...
Original Article
Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent
Eun Mi Yang, Dong Kyun Han, Hee Jo Baek, Young Ok Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(3):428-431.   Published online March 15, 2010
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other...
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...