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Review Article
Current insights into inherited bone marrow failure syndromes
Nack-Gyun Chung, Myungshin Kim
Clin Exp Pediatr. 2014;57(8):337-344.   Published online August 25, 2014

Inherited bone marrow failure syndrome (IBMFS) encompasses a heterogeneous and complex group of genetic disorders characterized by physical malformations, insufficient blood cell production, and increased risk of malignancies. They often have substantial phenotype overlap, and therefore, genotyping is often a critical means of establishing a diagnosis. Current advances in the field of IBMFSs have identified multiple genes associated with IBMFSs...

Original Article
Effect of early postnatal neutropenia in very low birth weight infants born to mothers with pregnancy-induced hypertension
Yang Hee Park, Gyung Min Lee, Jung Min Yoon, Enn Jung Cheon, Kyung Ok Ko, Yung Hyuk Lee, Jae Woo Lim
Clin Exp Pediatr. 2012;55(12):462-469.   Published online December 20, 2012
Purpose

In this study, we aimed to investigate the perinatal clinical conditions of very low birth weight (VLBW) infants born to mothers with pregnancy-induced hypertension (PIH) focusing on the effects of early postnatal neutropenia.

Methods

We reviewed the medical records of 191 VLBW infants who were born at Konyang University Hospital, between March 2003 and May 2011. We retrospectively analyzed the clinical characteristics...

Case Report
Candida tropicalis arthritis of the elbow in a patient with Ewing's sarcoma that successfully responded to itraconazole
Seung Youn Kim, Jung Sub Lim, Dong Hwan Kim, Hyeon Jeong Lee, Joong Bum Cho, Jun Ah Lee, Dong Ho Kim
Clin Exp Pediatr. 2011;54(9):385-388.   Published online September 30, 2011

Fungal infections are rarely responsible for arthritis. Few cases of fungal arthritis have been reported, even in immunocompromised hosts susceptible to low-virulence organisms. Herein, the authors report the first case of Candida tropicalis arthritis in a child with a solid tumor. A 13-year-old boy with Ewing's sarcoma developed arthritis in his elbow during the neutropenic period after chemotherapy. Despite treatment...

A case of glycogen storage disease type Ib
Moon-Sun Kim, Jae-Bok Park, Chang-Seok Ki, Jin-Kyung Kim
Clin Exp Pediatr. 2009;52(12):1383-1387.   Published online December 15, 2009
We report a case of an 18-month-old girl with glycogen storage disease type Ib (GSD Ib). Her neutrophil counts had gradually decreased to less than 500/µL by the age of 3 years. However, there were no recurrent bacterial infections. Mutation analysis of the glucose-6-phosphate translocase (G6PT) gene revealed a compound heterozygous missense mutation (Ala148Val/Gly273Asp).
Review Article
Neutropenia in children
Eun Sun Yoo
Clin Exp 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...
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
Outcome of typhlitis in children with cancer
Jae Min Lee, Kwang Hae Choi, Jeong Ok Hah
Clin Exp Pediatr. 2008;51(2):156-161.   Published online February 15, 2008
Purpose : Neutropenic enterocolitis is an acute, life-threatening inflammation of the small and large bowel, often seen in children with malignancies during periods of prolonged or severe neutropenia. The optimal management for typhlitis in pediatric oncology patients has been debateful between operative and nonoperative approaches. The purpose of this study was to review the outcome of medical management of patients...
Etiological agents isolated from blood in children with hemato-oncologic diseases (2002-2005)
So-Hee Kim, Young-Ah Lee, Byung-Wook Eun, Nam-Hee Kim, Jin-A Lee, Hyoung Jin Kang, Eun-Hwa Choi, Hee Young Shin, Hoan-Jong Lee, Hyo Seop Ahn
Clin Exp Pediatr. 2007;50(1):56-64.   Published online January 15, 2007
Purpose : This study was performed to identify the etiologic agents and antimicrobial susceptibility patterns of organisms responsible for bloodstream infections in pediatric cancer patients for guidance in empiric antimicrobial therapy. Methods : One hundred and ninety-seven episodes of bloodstream infections that developed in 128 pediatric cancer patients were analyzed, which were identified at the Seoul National University Children's Hospital during...
Case Report
Two Pediatric Patients with Neutropenia and Thrombocytopenia Associated with Parvovirus B19 Infection
Dong Koo Kim, Young Dai Kim, Ho Jun Lim, Sung Hee Oh, Hahng Lee
Clin Exp Pediatr. 2004;47(3):338-342.   Published online March 15, 2004
Parvovirus B19, a member of the Erythrovirus genus, is the only member of the Parvoviridae family known to be pathogenic in humans. Haematological consequences of B19 infection arise due to a direct cytotoxic effect on erythroid progenitors in bone marrow, resulting in interruption of erythrocyte production. The physiology of host haematopoiesis and competence of the immune response each determines clinical...
Original Article
Peripheral Neutrophil Count and Respiratory Failure in Preterm Infant
Kum Joo Lee, Soo Young Yun, Ran Lee, Jae Ho Hean, Ghee Young Jung, Jin Hee Park, Young Sun Park
Clin Exp Pediatr. 2002;45(5):596-602.   Published online May 15, 2002
Purpose : The purpose of this study was to analyze the association of peripheral neutrophil count with the development of respiratory failure in preterm infants. Methods : A retrospective study was conducted from January 1993 to December 1999 on 44 preterm infants, who were admitted to the neonatal intensive care unit of St. Francisco hospital. Preterm infants(birth weight 500 to 1,350...
Therapeutic Effect of Different Doses of Recombinant Human Granulocyte Colony-Stimulating Factor(rhG-CSF) on Neonatal Sepsis Complicated by Neutropenia
Moon Young Choi, Yeon Sook Jung, Dong Woo Son, Hyo Seop Ahn
Clin Exp Pediatr. 2002;45(4):439-448.   Published online April 15, 2002
Purpose : The aim of this study is to determine and compare the effects of adjunctive therapy with different doses of recombinant human granulocyte-colony stimulating factor(rhG-CSF) on reversing sepsis-associated neonatal neutropenia, and their survival rate in a group I/II-type trial. Methods : RhG-CSF was injected subcutaneously to 10 septic-neutropenic neonates with doses of 10 μg/kg from Oct. 1995 to Sep. 1996,...
Serum Level of G-CSF and GM-CSF and CFU-GM Response to Hematopoietic Growth Factors in a Patient with Cyclic Neutropenia
Youn-Kyung Lee, Seok-Hun Kim, Kwang-Woo Kim, Soon Lee, Heung-Sik Kim, Dong-Seok Chun, Kun-Soo Lee
Clin Exp Pediatr. 2001;44(10):1168-1175.   Published online October 15, 2001
Purpose : Serum levels of G-CSF and GM-CSF were measured and CFU-GM assay using G- CSF, GM-CSF and SCF was conducted to evaluate the influence of hematopoietic growth factor on the precursor cells of cyclic neutropenia. Methods : A 7-year-old male with cyclic neutropenia was studied. Marrow mononuclear cells were isolated at neutrophil nadir and recovery and cultured in methylcellulose media...
The Effect of Empirical Antibiotics in Febrile Neutropenia
Ju Suk Lee, Young Tak Lim
Clin Exp Pediatr. 1998;41(9):1209-1215.   Published online September 15, 1998
Purpose : This study was designed to evaluate the safety and efficacy of early cessation of antibiotic treatment regardless of absolute neutrophil count in children with febrile neutropenia and no identifiable infectious source. Methods : A prospective randomized clinical trial was performed in 93 episodes of fever in 37 neutropenic children with cancer, who were admitted to the Department of...
Erratum
Association of Early Postnatal Neutropenia and Development of Bronchopulmonary Dysplasia in Preterm Infants
Hee Seok Kim, Jun Dong Park, Beyong Il Kim, Jung-Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1998;41(8):1033-1040.   Published online August 15, 1998
Purpose : To see if a similar relationship exists between the decreased number of circulating neutrophils and the development of bronchopulmonary dysplasia(BPD) in preterm infants, we tried to test the hypothesis that claims that preterm infants, who develop BPD, have decreased number of circulating neutrophils than those who do not develop BPD. Methods : A retrospective cohort study was conducted in...
Original Article
The Risk Factor of Bacteremia in Children with Febrile Neutropenia due to Chemotherapy
Tae-Hong Kim, Young-Ho Lee
Clin Exp Pediatr. 1998;41(5):677-683.   Published online May 15, 1998
Purpose : We evaluated the easily-assessable risk factors to predict bacteremia in children with febrile neutropenia, who received anticancer chemotherapy. Methods : We retrospectively reviewed 46 children who had febrile neutropenia caused by anticancer chemotherapy between March, 1993 and February, 1997. The patients with localized infection on presentation were not eligible for this study. We evaluated the correlation between bacteremia and...
The Characteristics of Febrile Neutropenia Induced by Chemotherapy
Hyung Joo Sohn, Kee Hwan Yoo, Guang Chul Lee, Sun Gyum Kim
Clin Exp Pediatr. 1997;40(10):1443-1452.   Published online October 15, 1997
Purpose : The development of fever in neutropenic cancer patients is frequently a sign of infection. Infection remains the major cause of death in the patient with cancer, primarily as a consequence of the profound alterations of normal host defences which result from the malignancy, its treatment or both. Fever in the patient with neutropenia is generally recognized as an indication of the need...
Infections in Children with Neoplastic Disease
Eun Hwa Choi, Jin Won Pyo, Jin Young Park, Kyung Bae Kwon, Bo Young Yun, Hee Young Shin, Hoan Jong Lee, Hyo Seop Ahn
Clin Exp Pediatr. 1995;38(3):366-377.   Published online March 15, 1995
Purpose : Infection is a major complication in patients with malignant disease. This study was performed to identify the causes and the etiologic agents of febrile infections and to characterize the clinical courses including the response to antimicrobial agents inpediatric cancer patients. Methods : This study reviewed 274 febrile episodes occurring in 163 children with neoplastic disease which were indentified prospectively...
Case Report
A Case of Cyclic Neutropenia
Yoon Hwa Cha, Hye Sun Lee, Young Min Ahn, Myoung Sook Koo
Clin Exp Pediatr. 1993;36(7):1009-1015.   Published online July 15, 1993
Cyclic Neutropenia is a benign, unusual hematologic disorder characterized by regularly recurring episodes of severe neutropenia occurring approximately every 21 days. Beside neutrophils, monocytes, lymphocytes, platelets and reticulocytes all cycle with strict periodicity suggest that this disease should be viewed as cyclic hematopoiesis, not merely as cyclic neutropenia. During neutropenic periods, patients regularly experience aphthous stomatitis, fever, malaise, cervical lymphadenitis, cutaneous...
Original Article
Septicemia in Children with Neoplastic Disease
Bo Young Yun, Hoan Jong Lee
Clin Exp Pediatr. 1992;35(11):1481-1492.   Published online November 15, 1992
This study reviewed 66 episodes of septicemia occurring in 56 children with neoplastic disease at Seoul National University Children뭩 hospital over 31 month period. The most frequent organisms causing 45 episodes in neutropenic patient were Escherichia coli (28%), Klebsiella species (18%), Pseudomonas species (9%), polymicrobic (9%,), and Staphylococcus aureus (7%). The overal mortality was 13% (5/39). The most frequent organisms...
Clinical Characteristics and Pathogenesis of Typhlitis in Childhood Non-Lymphocytic Leukemia-Considerations on Clinical Mangement with Report of Two Cases and Literature Review.
Do Hyun Kim, Sung Oh Kim, Soo Yup Lee, In Joon Seol, Hahng Lee, Chong Moo Park, Poong Man Jung, Seok Chol Jeon, Young Hyeh Ko, Jung Dal Lee
Clin Exp Pediatr. 1988;31(5):607-620.   Published online May 31, 1988
The treatment of acute leukemia in childhood has been increasingly successful due to progression of chemotherapeutics and other supportive care. Improved survival rates in acute childhood leukemia have been associated with an increasing number of complications in the gastrointestinal tract. Typhlitis also known as ileocecal syndrome is a necrotizing inflammation of the cecum in leukemic patients on chemotherapy in the terminal stage of the...
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