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Case Report
Alveolar rhabdomyosarcoma with massive disseminated intravascular coagulopathy treated with systemic chemotherapy
Byung Gyu Yoon, Hee Jo Baek, Burm Seok Oh, Dong Kyun Han, Yoo Duk Choi, Hoon Kook
Clin Exp Pediatr. 2015;58(12):505-508.   Published online December 22, 2015

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy...

Graves disease following rabbit antithymocyte globulin treatment of severe aplastic anemia in a Korean child
In Su Choi, Han Kyul Kim, Dong Kyun Han, Hee Jo Baek, Hae In Jang, Chan Jong Kim, Hoon Kook
Clin Exp Pediatr. 2015;58(7):267-269.   Published online July 22, 2015

Antithymocyte globulin (ATG) is used as an immunosuppressive treatment (IST) to deplete clonal suppressor T cells in patients with severe aplastic anemia (SAA). The depletion of suppressor T cells by ATG may affect the activation of B cells, which results in an increased risk for autoimmune conditions. A 12-year-old boy was diagnosed with idiopathic SAA. As he did not have...

Original Article
Long-term follow-up of Fanconi anemia: clinical manifestation and treatment outcome
Byung Gyu Yoon, Hee Na Kim, Ui Joung Han, Hae In Jang, Dong Kyun Han, Hee Jo Baek, Tai Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2014;57(3):125-134.   Published online March 31, 2014
Purpose

The aim of this study was to characterize Korean patients with Fanconi anemia (FA), which is a rare but very challenging genetic disease.

Methods

The medical records of 12 FA patients diagnosed at Chonnam National University Hospital from 1991 to 2012 were retrospectively reviewed.

Results

The median age at diagnosis was 6.2 years. All patients showed evidence of marrow failure and one or more...

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
Perivascular epithelioid cell tumor (PEComa) of the ascending colon: the implication of IFN-α2b treatment
Sun Ju Park, Dong Kyun Han, Hee Jo Baek, Sang Young Chung, Jong Hee Nam, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(11):975-978.   Published online November 30, 2010

A 7-year-old boy presented with hematochezia and abdominal pain. A 3.7-cm-sized mass was identified in the ascending colon by abdominal computed tomography and colonoscopy. The patient underwent surgical resection. Pathological examination revealed a low-grade perivascular epithelioid cell tumor (PEComa). PEComa in the colon is very rare. Only a few cases have been reported so far. An effective treatment method for...

Original Article
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,...
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...
Case Report
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...
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
Clin Exp 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...
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
Clin Exp 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,...
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome: Successful treatment of the first case with bilateral Wilms` tumors in Korea
Kyung Sun Min, Hee Jo Baek, Dong Kyun Han, Ju Hee You, Tai Ju Hwang, Dong Deuk Kwon, Hoon Kook
Clin Exp Pediatr. 2008;51(12):1355-1358.   Published online December 15, 2008
Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms` tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had...
A case of megalencephalic leukoencephalopathy with subcortical cysts
Eun Young Park, Young Ok Kim, Ji Youn Kim, Chae Young Yeo, Hee Jo Baek, Chan Jong Kim, Eun Young Kim, Young Jong Woo
Clin Exp Pediatr. 2008;51(12):1342-1345.   Published online December 15, 2008
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare white matter disorder, first described in the early 1990s. The brain in patients with MLC appears swollen on MRI, with diffuse white matter abnormalities; in addition, there is an invariable presence of subcortical cysts, primarily in the anterior temporal region sparing the deep white matter, basal ganglia, thalami, and cerebellum. Patients...
The clinical phenotype of the derivative (8)t(7;8)(q22;p23.3) in two siblings
Young Ok Kim, Young Kuk Cho, En Song Song, Dong Kyun Han, Ic Sun Choi, Hee Jo Baek, Chan Jong Kim, Young Jong Woo, Young Youn Choi
Clin Exp Pediatr. 2008;51(11):1241-1244.   Published online November 15, 2008
We report on 2 siblings with a partial trisomy of 7q (7q22→qter) and concomitant partial monosomy of 8p (8p23.3→pter), which were shown by FISH using probes located at the telomere region of each chromosome. All the balanced translocation carriers (father and a sister) in this family had a normal phenotype. The 2 siblings with the same abnormal karyotype had similar...
Original Article
Diamond-Blackfan anemia: long-term follow-up of six cases
Young Jun Son, Hee Jo Baek, Hoon Kook
Clin Exp Pediatr. 2008;51(11):1211-1216.   Published online November 15, 2008
Purpose : Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. Methods :...
Comparison of growth and neuropsychological function after treatment for hematologic and oncologic diseases in monozygotic twins
Gi Hwan Kim, Hoon Kook, Hee Jo Baek, Dong Kyun Han, Eun Song Song, Young Kook Jo, Ic Sun Choi, Young Ok Kim, Chan Jong Kim, Young Jong Woo, Soo Jin Yang
Clin Exp Pediatr. 2007;50(2):182-189.   Published online February 15, 2007
Purpose : The primary purpose of this study was to evaluate the growth and neuropsychologic function following treatments for pediatric hematologic and oncologic diseases. Healthy monozygotic twins served as ideal controls for comparison to exclude possible confounding factors. Methods : Seven children treated with various hematologic and oncologic diseases were included in the study: acute lymphoblastic leukemia (ALL; n=2), Diamond-Blackfan anemia...
Second allogeneic hematopoietic stem cell transplantation in children to overcome graft failure or relapse after initial transplant
Dong-Yeon Kim, Do Kyun Kim, Soo Young Kim, Seok Joo Kim, Dong Gyun Han, Hee Jo Baek, Hoon Kook, Tai-Ju Hwang
Clin Exp Pediatr. 2006;49(12):1329-1339.   Published online December 15, 2006
Purpose : Failure of hematopoietic stem cell transplantation(HSCT) may be encountered in practice because of either relapse of the malignancy or dysfunction of the graft. Second HSCT may be the only option for some patients whose initial HSCT failed. Methods : From May, 1991 to December, 2004, 115 HSCTs were performed at the Pediatric Blood & Marrow Transplantation Center, Chonnam National...
Case Report
Pyridoxine responsive sideroblastic anemia in a boy with mitral valve prolapse
June Seung Sung, Ki Hwan Kim, Dong Gyun Han, Mi Jeong Kim, Young Kook Cho, Hae Yul Chung, Hee Jo Baek, Jae Sook Ma, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2006;49(11):1223-1226.   Published online November 15, 2006
Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of...
Two Cases of Paragonimiasis in Young Siblings Presenting with Pleural Effusion and Subcutaneous Nodules
Moon Young Jeong, Hee Jo Baek, Duck Cho, Jin Kim, Chan Kyoo Hwang, Dong Kyun Han, Jae Sook Ma, Hoon Kook
Clin Exp Pediatr. 2005;48(12):1385-1388.   Published online December 15, 2005
Paragonimiasis is the infestation of lung flukes of the trematode genus Paragonimus. This disease is common in Asia, and the southern part of Korea has been known as one of the endemic areas of Paragonimiasis westermanii in Korea. Human infection is associated with specific dietary habits, such as eating freshwater crawfish or crabs. In a 6 1/2-year-old boy with pleural...