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Case Report
A Case of Glutaric Aciduria Type I with Macrocephaly
Woo Jong Shin, Yeo Ok Moon, Hye Ran Yoon, Eun Sil Dong, Young Min Ahn
Clin Exp Pediatr. 2003;46(3):295-301.   Published online March 15, 2003
Glutaric aciduria type 1(GA1) is an autosomal recessive disorder of the lysine, hydroxylysine and tryptophan metabolism caused by the deficiency of mitochondrial glutaryl-CoA dehydrogenase. This disease is characterized by macrocephaly at birth or shortly after birth and various neurologic symptoms. Between the first weeks and the 4-5th year of life, intercurrent illness such as viral infections, gastroenteritis, or even routine...
Original Article
Sudden Unexpected Death in Infancy (Analysis of 34 Cases Including 13 Autopsies)
Yeo Ok Moon, Hee Kyoung Choi, Jeoung-A Her, Woo Jong Shin, Myoung-A Kim, Seong Yong Lee, Seong Hee Jang, Eun Sil Dong, Chong Jae Kim, Young Min Ahn, Je Geun Chi
Clin Exp Pediatr. 2002;45(9):1065-1074.   Published online September 15, 2002
Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical...
Case Report
A Case of Marshall-Smith Syndrome
Yeo Ok Moon, Woo Jong Shin, Youn Jeong Shin, Eun Sil Dong, Young Min Ahn
Clin Exp Pediatr. 2002;45(7):906-911.   Published online July 15, 2002
Marshall-Smith syndrome is characterized by a triad of facial dysmorphism, failure to thrive and accelerated osseous maturation. We report a one-month-old male infant with of this rare syndrome, with laryngeal anomalies who died at 6 months of age with pneumonia. This is the first case of Marshall-Smith syndrome in Korea.
Original Article
Clinical Manifestations of Group A β-Hemolytic Streptococcal Pharyngits and Usefulness of Rapid Antigen Test
Youn Jeong Shin, Seong Hee Jang, Eun Sil Dong, Young Min Ahn, Myung Suk Ku
Clin Exp Pediatr. 2001;44(7):732-741.   Published online July 15, 2001
Purpose : Accurate diagnosis of group A streptococcal(GAS) pharyngitis and appropriate antimicrobial therapy are important, particularly to prevent nonsuppurative sequelae and to reduce the improper use of antibiotics. Because the clinical presentation of pharyngitis does not reliably predict the etiologic agent, when GAS infection is suspected, diagnosis should be based on the result of a throat swab culture or antigen-detection...
Case Report
A Case of Wolf-Hirschhorn Syndrome with Long Term Survival Diagnosed by Fluorescent In-situ Hybridization(FISH)
Eun Sil Dong, Mee Jeong Kim, Young Min Ahn, Myoung Sook Koo, Hwan Keuk Yong, Anna Lee
Clin Exp Pediatr. 2000;43(3):438-443.   Published online March 15, 2000
Wolf-Hirschhorn syndrome is a multiple malformation syndrome associated with mental and developmental retardation, resulting from a deletion at the short arm of chromosome 4(4p16.3). We report a 11-year-old girl with Wolf-Hirschhorn syndrome, who was presented with severe growth and mental retardation along with characteristic features-frontal bossing, hypertelorism, downslanting of the palpebral fissures and fishlike lips. The diagnosis was confirmed by...
A Case of Newborn Sepsis Caused by Streptococcus pneumoniae
Eun Ha Choi, Seong Hee Jang, Eun Sil Dong, Young Min Ann
Clin Exp Pediatr. 1995;38(12):1690-1693.   Published online December 15, 1995
Systemic infection due to Streptococcus pneumoniae is a serious, albeit rare disease in the neonatal period. There is no case of pneumococcal sepsis reported in Korea. The results of epidemiologic studies suggest that the majority of infants with pneumococcal septicemia acquire the microorganisms either by the ascending route or during passage throught the birth canal. The mortality is high in...
Original Article
A Prospective Study of Totally Implanted Venous Access System in 19 Children with Cancer
Kyung Duk Park, Eun Sil Dong, Seong Hoon Ha, Hong Hoe Koo, Hee Young Shin, Hyo Seop Ahn, Seong Eun Jun, Gui Won Park
Clin Exp Pediatr. 1993;36(5):687-692.   Published online May 15, 1993
A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drug, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter day), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter...
Case Report
Recurrent Rhinocerebral Mucormycosis - A Case Report
Ho Sung Kim, Jin Young Park, Bo Young Yun, Eun Sil Dong, Hee Young Shin, Hoan Jong Lee, Hyo Seop Ahn, Je Geun Chi, Myung Whun Sung
Clin Exp Pediatr. 1993;36(4):589-595.   Published online April 15, 1993
A case of recurrent rhinocerebral mucormycosis that has occurred during an induction chemotherapy for acute megakaryocytic leukemia in a 10 year-old boy is reported. He had suffered from high fever, proptosis, right eye ball pain and necrotic inflammation of hard palate during the chemotherapy of leukemia. CT scan of the paranasal sinus showed inflammatory change of right ethmoid and maxillary...
Original Article
Acute Megakaryoblastic Leukemia in Children
Hong Hoe Koo, Sang Kyu Park, Eun Sil Dong, Hye Jung Park, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 1993;36(1):9-16.   Published online January 15, 1993
We analyzed the clinical and laboratory features of ten children with acute megakaryoblastic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination of platelet peroxidase or immunophenotyping for glycoprotein Iib/IIIa. Of the ten children, five were girls and five were boys. The median age at diagnosis was 13 months. Two...
Case Report
Three Cases of Brain Tumors with Bone Metastasis
Eun Joo Kim, Kyung Duk Park, Eun Sil Dong, Hye Jung Park, Hong Hoe Koo, Hee Young Shin, Hyo Seop Ahn, Je Geun Chi
Clin Exp Pediatr. 1992;35(3):381-389.   Published online March 15, 1992
Medulloblastoma and primitive neuroectodermal tumor(PNET) are relatively common intracranial neoplasms in childhood. Their extracranial metastasis were thought to be rare occurrences. But they are more frequent compared to other intracranial tumors. The most frequent site of metastasis deposits in medulloblastoma is bone, and metastasis to lymph node, peritoneum, liver or lung have been reported infrequently. The authors experienced three cases...
Original Article
Clinical considerations of acute leukemia or transient myeloprolifo- rative disorder in Down syndrome.
Eun Sil Dong, Sung Hee Jang, Hong Hoe Koo, Hye Lim Jung, Hee Young Shin, Hyo Seop Ahn
Clin Exp Pediatr. 1991;34(1):74-82.   Published online January 31, 1991
Children with Down syndrome have an increased incidence of acute leukemia. Infants with Down syndrome are also at risk of developing a transient myeloproliferative disorder indistinguishable from acute nonlymphocytic leukemia (ANLL) except by its eventual clinical recovery. We observed 11 patients with acute leukemia or transient myeloproliferative disorder in Down syndrome who had admitted to the Departmetn of Pediatrics, Seoul National University Children’s Hospital,...
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