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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,...
A case of mosaic ring chromosome 13 syndrome
Soo Young Kim, Soo Min O, Mi Jeong Kim, Eun Song Song, Young Ok Kim, Young Youn Choi, Young Jong Woo, Tai Ju Hwang
Clin Exp Pediatr. 2009;52(2):242-246.   Published online February 15, 2009
The clinical features of ring chromosome 13 include mental and growth retardation, CNS anomalies, facial dysmorphism, cardiac defects, genital malformations, limb anomalies, skeletal deformities and anal malformations. Although many cases of ring chromosome 13 have been reported worldwide, only 6 cases have been reported in Korea, and the latter cases were not mosaic but pure ring chromosome 13. Here we...
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...
Congenital hernia of the lung through the azygoesophageal recess
Young Seok Choi, Young Jun Son, Si Young Bae, Kyung Sun Min, Young Kuk Cho, Woo Yeon Choi, Young Youn Choi, Jae Sook Ma, Tai Ju Hwang
Clin Exp Pediatr. 2008;51(10):1123-1126.   Published online October 15, 2008
A lung hernia, defined as the protrusion of pulmonary tissue and pleural membranes through a defect in the thoracic wall, is a rare event. It can be congenital or acquired, and cervical, thoracic, or diaphragmatic in location. We report the rare occurrence of a congenital atraumatic lung herniation through the azygoesophageal recess. An 8-month-old male infant, who was born...
A case of subcutaneous paragonimiasis presented with pleural effusion
Soo Young Kim, Sun Ju Park, Si Young Bae, Young Kuk Cho, Chan Jong Kim, Young Jong Woo, Young Youn Choi, Jae Sook Ma, Tai Ju Hwang
Clin Exp Pediatr. 2008;51(7):760-765.   Published online July 15, 2008
Paragonimiasis is a parasitic infection that occurs following the ingestion of infectious Paragonimus metacercariae from crabs or crayfish. Pulmonary paragonimiasis is the most common clinical manifestation of this infection, but several ectopic paragonimiasis cases have also been reported. Among them, cases of subcutaneous paragonimiasis are rare, especially in children. We report a case of subcutaneous paragonimiasis of the right abdominal...
Review Article
Hematopoietic stem cell transplantation : overview for general pediatrician
Tai Ju Hwang
Clin Exp Pediatr. 2007;50(7):613-621.   Published online July 15, 2007
Hematopoietic stem cell transplantation (HSCT) has expanded and evolved substantially in the last decades to treat various malignant and nonmalignant diseases. However, the conditioning regimen can lead to transplantation related death by major organ dysfunction, severe infection and bleeding. In the allogeneic setting, graft versus host disease may also develop, making post-transplant management complex. To overcome these problems, new stem...
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...
Original Article
Frequency and clinical characteristics of prenatally diagnosed congenital hydronephrosis and outcomes of ureteropelvic junction stenosis
Hyun Soo Kang, June Seung Sung, Sun Hui Kim, Hee Jo Back, Young Ok Kim, Chan Jong Kim, Young Youn Choi, Tai Ju Hwang
Clin Exp Pediatr. 2006;49(8):870-874.   Published online August 15, 2006
Purpose : Popular use of fetal ultrasonography has increased to detect congenital hydronephrosis(CH) which is the most common anomaly prenatally detected. We'd like to determine the frequency and clinical characteristics of prenatally diagnosed CH and outcome of ureteropelvic junction stenosis(UPJS). Methods : The records of births between January 1994 and June 2003 in Chonnam National University Hospital(CNUH), and the records of...
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