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Case Report
A Case of Citrullinemia Diagnosed at the Neonatal Period
Seung Kyu Song, Kyung Chang Oh, Mi Ae Hong, Hee Taeg Kim, Hye Jung Shin, Soon Young Kim, Jin Keun Chang, Heui Seung Jo, Beyong Il Kim, Sei Won Yang, Jung-Hwan Choi
Clin Exp Pediatr. 2002;45(4):524-528.   Published online April 15, 2002
Citrullinemia is a rare inborn error of metabolism of the urea cycle, and was first reported by McMurray, et al. in 1962. It is inherited as an autosomal recessive trait. The normal synthesis of argininosuccinic acid is blocked in this disease due to a deficiency of argininosuccinic acid synthetase(AS), which has been demonstrated in liver cells and fibroblasts. The clinical...
A Case of Maple Syrup Urine Disease
Dong Hyun Cho, Hyun Mi Lee, Soon Young Kim, Chang Soo Ra
Clin Exp Pediatr. 1997;40(9):1297-1302.   Published online September 15, 1997
A Case Report of Autoimmune Hepatitis Associated with Choledochal Cyst and Pancreatitis
Kyung Ran Park, Sun Young Lee, Soon Young Kim, Hyoung Shim Chang, Nam Su Back, Chong Jai Kim, Joong Gon Kim, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(8):1146-1150.   Published online August 15, 1996
Autoimmune hepatitis in children is a rare and severe inflammatory disease of unknown etiology, and progress to cirrohosis and liver failure, generally is responsive to immunosuppressive therapy. It is more prevalent in women than men, and characterized by the presence of circulating autoantibodies, a high serum globulin. Extrahepatic manifestations such as thyroiditis, ulcerative colitis, glomerulonephritis and autoimmune hemolytic anemia, are associated. We report, to our...
A Case of Neonatal Cholestasis with Arthrogryposis Multiplex Congenita and Renal Tubular Insufficiency(ARC Syndrome)
Hi Soo Rhee, Soon Young Kim, Nam Sun Baik, Il Soo Ha, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(1):126-130.   Published online January 15, 1996
We report a case of ARC syndrome with arthrogryposis multiplex congenita, renal tubular insufficiency and cholestasis. The Patient presented in the early neonatal period with micrognathia, low set ears, high arched palate, multiple joint contracture, conjugated hyperbilirubinemia and failure to thrive. He died at the age of 1 month despite medical therapy. Findings of renal tubular insufficiency included persistent renal...
Original Article
Choleithiasis in Childhood
Soon Young Kim, Jeong Kee Seo
Clin Exp Pediatr. 1994;37(1):70-77.   Published online January 15, 1994
Nineteen children found to have gallstones at Seoul National University of Children's Hospital form Jun. 1982 to Jun. 1992 wee reviewed. The results were as follows: 1) The mean age was 10 years, with 9 boys and 10 girls. Most of the cases (63%) were over 10 years of age. 2) Clinical manifestations were abdominal pain (14 cases), naused and/or vomiting (5 cases), fever...
Esophageal Varix in Children - Endoscopic Evaluation and Clinical characteristics-
Soon Young Kim, Jeong Kee Seo
Clin Exp Pediatr. 1993;36(12):1691-1700.   Published online December 15, 1993
Fifty five children with encoscopically proven esophageal varices form Jul. 1987 to Dec. 1992 were analyzed for endoscopic and clinical characteristics. The results were as follows : 1) According to etiological classification of portal hypertension in 55 cases, 35 cases showed intrahepatic causes (64%) and cases had extrahepatic causes (36%). The most common clinical manifestation on admission was hematemesis (42%) and abdominal mass...
Case Report
Hunter Syndrome
Soon Young Kim, Aaera Shin, Sung Won Park, Sang Hyup Kim, Han Woong Choi
Clin Exp Pediatr. 1973;16(4):330-334.   Published online April 30, 1973
The cases of Hunter syndrome in two brothers were reported, the first cases in Korea. The diagnosis was confirmed by physical findings and radiological characteristics. The review of literature is made briefly.
Two Cases of Neurofibromatosis
Esook Oh, Soon Young Kim, Heung Jae Lee, Jong Ku Yoon
Clin Exp Pediatr. 1973;16(3):248-252.   Published online March 31, 1973
Presenting two cases of neurofibromatosis, one^with generalized neurofibromatosis and typical family history, and the other with generalized cafe au lait spots and bony defect of the skull which were thought to be early signs of neurofibromatosis, the authors reviewed the referential literatures on the Von Recklinghausen' s disease
A Case of Total Anomalous Pulmonary Venous Return
Hyo Seop Ahn, Soon Young Kim, Tae Woon Kim, Jong Ku Yoon, Hyung Ro Moon, Chang Yee Hong, Hye Soon Chang, Man Cheong Han
Clin Exp Pediatr. 1973;16(2):156-160.   Published online February 28, 1973
A case of total anomalous pulmonary venous return in a 10 year and 8 month-old female was presented. The patient was admitted with the chief complaints. of palpitation and growth retardation. The diagnosis was confirmed by right heart catheterization and angiocardiography. The review of literature was made briefly.
A Case of Tuberculous Pericarditis
Yae Talk Han, Min Ja Pai, Soon Young Kim, Sang Hyup Kim
Clin Exp Pediatr. 1972;15(6):568-573.   Published online June 30, 1972
A case of tubsrculous pericarditis is reported with a brief review of the literature. The patient was a 13-year-old female who was admitted to the Department of Pediatrics, Seoul National University Hospital because of dyspnea and mild fever. Although a tuberculin test was negative on her, the triple chemotherapy for tuberculosis was instituted and resulted in a marked improvement. However, on discontinuation of the...
Original Article
Leukemia in Childhood with Mediastinal Masses
Min Ja Pai, Soon Young Kim, Don Hee Ahn, Chang Yee Hong
Clin Exp Pediatr. 1972;15(4):319-325.   Published online April 30, 1972
Six cases o£ acute leukemia with mediastinal masses are reported with a review of relevant literature.. Out of 105 patients wi h leukemia who had been admitted to the Department of Pediatrics, Seoul. National University Hospital for the last 6 years, six cases had huge mediastinal masses on the chest x-ray examination. In 3 of them the masses were located in the anterior...