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Volume 52(6); Jun 2009
Review Articles
Neutropenia in children
Eun Sun Yoo
Korean J Pediatr. 2009;52(6):633-643.   Published online June 15, 2009
Neutropenia is defined as an absolute neutrophil count (ANC) of <1,500/µL, and the severity of neutropenia generally can be graded as mild (1,000-1,500/µL), moderate (500-1,000/µL), or severe (<500/µL). This stratification aids in predicting the risk of pyogenic infection because the susceptibility to life-threatening infections is significantly increased in patients with prolonged episodes of severe neutropenia. Especially cancer-related neutropenia carry significant...
Eosinophil disorders
Sun Young Kim
Korean J Pediatr. 2009;52(6):643-648.   Published online June 15, 2009
Blood eosinophilia can be classified as either familial or acquired. Familial eosinophilia is a rare autosomal dominant disorder characterized by a stable eosinophil count. Acquired eosinophilia is classified further into a primary or secondary phenomenon depending on whether eosinophils are considered integral to the underlying disease. Primary eosinophilia is considered clonal in the presence of either a cytogenetic abnormality or...
Recent advance in primary immune deficiency disorders
Hyoung Jin Kan, Hee Young Shin, Hyo Seop Ahn
Korean J Pediatr. 2009;52(6):649-654.   Published online June 15, 2009
The immune system is comprised of cells and molecules whose collective and coordinated response to the introduction of foreign substance is referred to as the immune response. Defense against microbes is mediated by the early reaction (innate immunity) and the late response (adaptive immunity). Innate immunity consists of the epithelial barrier, phagocytes, complement and natural killer cells. Adaptive immunity, a...
Original Articles
Multicenter clinical study on birth weight and associated anomalies of single umbilical artery
Su-Nam Bae, Jae-Woo Lim, Kyong-Og Ko, Hyun-Seung Jin, Min-Hee Kim, Bo-Young Lee, Chun-Soo Kim, Eun-Ryoung Kim, Sang-Kee Park, Jung-Joo Lee
Korean J Pediatr. 2009;52(6):655-660.   Published online June 15, 2009
Purpose : To compare birth weight between infants with a single umbilical artery (SUA) and normal infants, investigate the associated anomalies of infants with SUA and isolated SUA (no abnormality of external appearance on birth, except SUA), and determine the prognosis of infants with isolated SUA. Methods : Live-born infants with SUA (n=59) detected by physical examination from among 15,193...
Clinical significance of codetection of the causative agents for acute respiratory tract infection in hospitalized children
Eui Jung Roh, Young Pyo Chang, Jae Kyung Kim, In Soo Rheem, Kwi Sung Park, Eun Hee Chung
Korean J Pediatr. 2009;52(6):661-666.   Published online June 15, 2009
Purpose : To determine the prevalence and clinical features of codetected respiratory etiological agents for acute respiratory infection in hospitalized children. Methods : Nasopharyngeal aspirates were obtained from hospitalized children with acute respiratory infection at Dankook University Hospital from September 2003 through June 2005. Immunofluorescent staining and culture were used for the detection of respiratory viruses (influenza virus [IFV] types...
Toll-like receptor 2 expression on monocytes from patients with BCG vaccine-associated suppurative lymphadenitis
Hyun Joo Oh, Kyung-Sue Shin
Korean J Pediatr. 2009;52(6):667-673.   Published online June 15, 2009
Purpose : Toll-like receptor 2 (TLR2) is critical in the immune response to mycobacterial infections. The purpose of this study was to analyze TLR2 surface expressions and TLR2-mediated tumor necrosis factor-alpha (TNF-α) and interleukin-6 (IL-6) production in patients with BCG vaccine-associated suppurative lymphadenitis. Methods : Peripheral monocytes were separated from 16 patients with BCG vaccine-associated suppurative lymphadenitis and 10 healthy...
Bilateral retinoblastoma: Long-term follow-up results from a single institution
Sang Yul Choi, Dong Hwan Kim, Kang Min Lee, Hyun Jae Lee, Mi-Sook Kim, Tai-Won Lee, Sang Wook Choi, Dong Ho Kim, Kyung Duk Park, Jun Ah Lee
Korean J Pediatr. 2009;52(6):674-679.   Published online June 15, 2009
Purpose : The authors aimed to analyze the long-term effects of treatments, especially external beam radiotherapy (EBRT), in bilateral retinoblastoma patients. Methods : This retrospective study analyzed the medical records of 22 bilateral retinoblastoma patients who were registered between October, 1987 and October, 1998 and followed-up for more than 10 years. They were treated by enucleation, EBRT, and systemic chemotherapy....
Association study of polymorphism in leukotriene C4 synthase and cysteinyl leukotriene receptor 1 genes with phenotype of asthma and clinical parameters in Korean children
Jung Yeon Shim, Byung-Joo Kim, Young Hwa Song, Mi-Jin Kang, So-Yeon Lee, Jinho Yu, Soo-Jong Hong
Korean J Pediatr. 2009;52(6):680-688.   Published online June 15, 2009
Purpose : Cysteinyl leukotrienes are important proinflammatory mediators in asthma. Recently, it was suggested that a promoter polymorphism in the genes encoding for leukotriene C4 synthase (LTC4S), a key enzyme in the leukotriene synthetic pathway, and cysteinyl leukotriene receptor 1 (CysLTR1) might be associated with aspirin-intolerant asthma. We investigated whether polymorphisms in LTC4S and CysLTR1 genes or their interactions were...
Effect of endothelin receptor blockade on monocrotaline-induced pulmonary hypertension in rats
Kyoung Ah Lim, Jung Yun Shim, Sang Ho Cho, Kwan Chang Kim, Jae Jin Han, Young Mi Hong
Korean J Pediatr. 2009;52(6):689-695.   Published online June 15, 2009
Purpose : To examine the effect of bosentan, a dual endothelin receptor (ER) antagonist, on the development of monocrotaline (MCT)-induced pulmonary hypertension in rats by especially focusing on the pulmonary vascular morphology changes. Methods : Sprague-Dawley rats were treated as follows: controls received a subcutaneous saline injection, MCT-treated rats received a subcutaneous MCT injection, and bosentan-treated rats received a MCT...
Anti-tumor effect of new compound, 127, through the induction of apoptosis
Ki Hwan Baek, A Lum Han, Sae Ron Shin, Chun Mae Jin, Young Wook Yoon, Seung Taek Yu, Jong Duk Kim, Du Young Choi
Korean J Pediatr. 2009;52(6):696-700.   Published online June 15, 2009
Purpose : We screened more than 350 compounds with an endoperoxide ring structure in search of an anti-leukemic drug and found that compound 127 (c-127) could induce significant cytotoxicity in HL-60 cells. In this study, we investigated the molecular mechanisms of compound 127-induced antitumor activity on HL-60 cells. Methods : HL-60 cells were cultured in Rosewell Park Memorial Institute 1640...
Case Reports
Two cases of Smith-Magenis syndrome
Seong Kwan Jung, Kyu Hee Park, Hae Kyung Shin, So Hee Eun, Baik-Lin Eun, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Sook Young Bae
Korean J Pediatr. 2009;52(6):701-704.   Published online June 15, 2009
SmithMagenis syndrome (SMS) is a rare disorder with multiple congenital anomalies caused by a heterozygous interstitial deletion involving chromosome 17p11.2, where the retinoic acid-induced 1 (RAI1) gene is located, or by a mutation of RAI1. Approximately 90% of the patients with SMS have a detectable 17p11.2 microdeletion on fluorescence in-situ hybridization (FISH). SMS is characterized by mental retardation, distinctive behavioral...
A case of acute aseptic meningitis associated with herpes zoster
Myong A Kim, Rita Miyoung Yu, Kee Hyuck Kim, Hee Jung Chung
Korean J Pediatr. 2009;52(6):705-709.   Published online June 15, 2009
Herpes zoster is caused by the varicella-zoster virus (VZV), which affects nerve ganglions. VZV infection may be associated with neurologic complications, which are usually observed after vesicular exanthem. Acute aseptic meningitis is a rare complication of VZV reactivation. We report the case of a previously healthy 14-year-old boy who suffered from aseptic meningitis that was attributed to reactivated VZV infection...
A case of congenital syphilis mistaken for possible child abuse
Soon Ju Kim, Seung Woo Lee, Jung Woo Rhim, You Sook Youn, Jun Sung Lee, Kyung Yil Lee, Ja Young Hwang
Korean J Pediatr. 2009;52(6):710-712.   Published online June 15, 2009
We describe the case of a 4-month-old male infant diagnosed with early congenital syphilis during evaluation of a left distal humerus fracture. This report emphasizes the importance of screening for syphilis among pregnant women and newborns, and is a reminder of the continued existence of congenital syphilis.
Facial palsy as the presenting symptom of acute myeloid leukemia in children: Three cases with stem cell transplantatio
Hee Jo Baek, Dong Kyun Han, Young Ok Kim, Ic Sun Choi, Tai Ju Hwang, Hoon Kook
Korean J Pediatr. 2009;52(6):713-716.   Published online June 15, 2009
Facial palsy as the presenting symptom of leukemia is very rare, especially in acute myeloid leukemia. A review of the medical literature identified reports on 8 children with AML who had facial paralysis as the presenting sign. Whole brain irradiation (WBI) has been applied in most cases. We present the cases of 3 such children. Achieving a remission without WBI,...
A case of newly developed pulmonary lesion during the antitubercular agents in tuberculous pleurisy : A paradoxical response
Jee Min Park, Youn Ho Shin, Gyu Rak Chon, Hyun Joon Shin, Young Chil Choi
Korean J Pediatr. 2009;52(6):717-720.   Published online June 15, 2009
Paradoxical response refers to the enlargement of old lesions or unexpected appearance of new lesions after initial improvement following treatment with antitubercular agents. Various types of paradoxical responses have been reported in the world, but they are rarely reported in Korean children. We report the case of a 17-year-old boy who was diagnosed with tuberculous pleurisy and was treated appropriately....
A Korean familial case of hereditary complement 7 deficiency
Moon Kyu Ki, Kyung Yul Lee, Jun Hwa Lee
Korean J Pediatr. 2009;52(6):721-724.   Published online June 15, 2009
Meningococcal infections can be associated with abnormalities of the complement system, which contains 5 terminal complement proteins. Furthermore, deficiencies in 1 of these 5, complement component 7 (C7), leads to the loss of complement lytic function, and affected patients show increased susceptibility to recurrent meningococcal meningitis and systemic Neisseria gonorrhoeae infection. In September 2003, an 11-year-old female patient presented at...
Unrelated stem cell transplantation after reduced-intensity conditioning plus rituximab for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with CNS involvement
Hee Jo Baek, Hoon Kook, Dong Kyun Han, Min-Cheol Lee, Tae Woong Jeong, Tai Ju Hwang
Korean J Pediatr. 2009;52(6):725-729.   Published online June 15, 2009
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with central nervous system (CNS) involvement is usually fatal unless stem cell transplant (SCT) is offered. However, SCT with conventional intensity conditioning is associated with high transplant-related mortality. We describe our experience with unrelated SCTs after reduced-intensity conditioning (RIC) for patients with EBV-HLH with progressive CNS disease. This approach was associated with minimal toxicities and...
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