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Case Report
A Case of Bronchogenic Cyst with Nausea and Epigastric Pain
Ji-Hyun Kim, Kang-Won Rhee, In-Seok Lim, Byung-Hoon Yoo, Eung-Sang Choi
Clin Exp Pediatr. 2005;48(3):333-336.   Published online March 15, 2005
Bronchogenic cysts are rare congenital anomalies that arise early in gestation from abnormal budding of the developing respiratory system. Mediastinal bronchogenic cysts account for 10-15 percent of all primary mediastinal masses; 63.7 percent of patients are symptomatic. Common symptoms are fever, chest pain, cough, dyspnea, and dysphagia. Gastrointestinal symptoms except dysphagia are rare. It can be life threatening with compression,...
A Case of Choledochal Cyst Complicated by Liver Cirrhosis on Pathology in a 20-Month-Old-Girl
Sun Hwan Bae, Sung Yun Choi, Tae Seok Lee, Ho Jeong Lee
Clin Exp Pediatr. 2005;48(1):104-107.   Published online January 15, 2005
Choledochal cyst is considered to be congenital anomalies of the biliary tract, characterized by varying degrees of cystic dilatation at various segments of the biliary tract. A 20-month-old girl was admitted to Eul-Ji general hospital because of abdominal distension. Physical examination revealed marked splenomegaly and hepatomegaly with nodular surface and hard consistency. Laboratory examination showed elevated transaminase level, alkaline phosphatase...
Original Article
Intrauterine Intraventricular Hemorrhage in Premature Infants
Hyun-Seung Jin, Kyeng-Ah Park, Hyun-Woo Goo, Jong-Hyun Yoon, Ellen Ai-Rhan Kim, Ki-Soo Kim, Soo-Young Pi
Clin Exp Pediatr. 2005;48(1):27-33.   Published online January 15, 2005
Purpose : To determine incidence, characteristics and risk factors associated with intrauterine intraventricular hemorrhage(IU-IVH) among premature infants. Methods : The medical records of infants with intraventricular hemorrhage(IVH) admitted to the neonatal intensive care unit of Asan Medical Center from January 1999 to June 2003 were reviewed retrospectively. Infants whose IVH with cystic change were detected within five days of life were...
Case Report
A Case of Congenital Megacystis Due to Non-Obstructive Urinary Retention Associated with Intestinal Malrotation and Hypoperistalsis
Tai Young Ham, Jeong Hoon Kim, Ji Hong Kim, Jae Seung Lee, Seok Ju Han, Choon Sik Yoon, Soon Won Hong
Clin Exp Pediatr. 2004;47(1):95-99.   Published online January 15, 2004
Congenital megacystis with bilateral hydroureteronephrosis is most commonly associated with posterior urethral valves, prune-belly syndrome, vesicoureteral reflux, or nonrefluxing, nonobstructive megaureters. Among the other cause of congenital megacystis, megacystis-microcolon-intestinal hypoperistalsis syndrome(MMIHS), which is characterized by megacystis, microcolon and hypoperistalsis of the intestines with resultant abdominal distension, is a rare cause of functional obstruction of urinary tracts in childhood. It was...
A Case of Cystinosis
Min Ho Jung, Seung On Keun, Soon Ju Lee, Byung Churl Lee
Clin Exp Pediatr. 2003;46(6):615-619.   Published online June 15, 2003
Cystinosis, an autosomal recessively inherited lysosomal storage disease, results from impaired transport of the amino acid cystine out of cellular lysosomes. The consequent accumulation and crystallization of cystine destroys tissues, causing growth retardation, Fanconi syndrome, renal failure, eye problems, and endocrinopathies. The gene for cystinosis, CTNS, was mapped to chromosome 17p13. The diagnosis of cystinosis was made by measuring the...
Original Article
The Usefulness of MRCP in the Evaluation of Pancreaticobiliary Diseases in Children
Ji Hyun Uhm, Seung Yeon Lee, Ki Sup Chung
Clin Exp Pediatr. 2002;45(11):1381-1388.   Published online November 15, 2002
Purpose : Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. Methods : From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and...
Case Report
A Case of Klinefelter Syndrome associated with Unilateral Multicystic Dysplastic Kidney in a Newborn Infant
Kyung A Ha, Sun Mi Chung, Eun Jin Choi, Jin Kyung Kim, Un Seok Nho, Jae Shin Park, Woo Taek Kim, Young Dae Kwon
Clin Exp Pediatr. 2002;45(9):1141-1145.   Published online September 15, 2002
Klinefelter syndrome is the most common chromosomal abnormality, with a 47, XXY karyotype and typical clinical findings of infertility, hypogonadism, reduced body hair, gynecomastia, tall stature, and incresed gonadotropins and decreased testosterone levels. In addition to this classic description, several other diseases have been discribed in Klinefelter syndrome such as unilateral renal aplasia, autoimmune disease, diabetes mellitus, sexual precoxity, renal...
Original Article
Clinical Study of Renal Cystic Diseases in Children
Ja Hyung Kim, You Jeong Kim, Byeong Seon Lee, Tae Sung Ko, Young Seo Park
Clin Exp Pediatr. 2002;45(2):232-239.   Published online February 15, 2002
Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with...
Case Report
49, XXXXY Syndrome with Multicystic Kidney in a Neonate
Eun Young Park, Soo Jee Moon
Clin Exp Pediatr. 2001;44(6):714-717.   Published online June 15, 2001
The 49, XXXXY Klinefelter variant is distinctive; classic findings include mental retardation, hypogonadism and radioulnar synostosis. Its incidence is estimated to be 1/80,000 to 1/100,000 male births. We report a case of 49, XXXXY syndrome confirmed by chromosomal study in neonatal period, whose clinical features included a coarse face, short neck, micropenis, and other multiple minor anomalies including simian creases....
Original Article
A Clinical Study for Risk Factors of Cystic Periventricular Leukomalacia
Ji Eun Kim, Su Jin Kim, Woo Taek Kim
Clin Exp Pediatr. 2001;44(6):627-634.   Published online June 15, 2001
Purpose : It is very important to study the individual risk factors which cause periventricular leukomalacia(PVL) and those which affect the process of cystic formation from liquefied periventricular white matter, in order to prevent and permit early diagnosis of cystic PVL that contributes to mortality and neurologic sequelaes in preterm infants. Therefore, the present study was designed to assess the...
Risk Factors for Cardiovascular Disease in Obese Children
Su Jin Cho, Se Jin Park, Il Tae Hwang, Young Mi Hong
Clin Exp Pediatr. 2001;44(5):493-500.   Published online May 15, 2001
Purpose : Obesity is known to be associated with complications such as hypertension, dyslipidemia, and insulin resistance. The importance of insulin resistance and hyperinsulinemia have been emphasized in obesity. Homocysteine is an emerging important risk factor for cardiovascular disease in adult, but there are less reports about homocysteine in children. Several risk factors such as blood pressure, lipid profiles, serum...
Case Report
Langerhans` Cell Histiocytosis Associated wjth Secondary Aneurysmal Bone Cyst, One Case Report
Yong-Koo Park, Kyung Nam Ryu, Won Leem
Clin Exp Pediatr. 2000;43(12):1626-1629.   Published online December 15, 2000
Seconnday aneurysmal bone cyst is the disease that occurrs in the primary bone tumor and/or tumorus conditions Meticulous search is needed to make a correct diagnosis which dose not overlook the primary diseases. We have experinced an osteolytic lesion on the 7-year-old boy on his skull bone. Plain radiography of the skull revealed osteolytic lesion in the parieto-occipital bone. On...
Original Article
Natural History and Clinical Feature of Multicystic Dysplastic Kidney detected with Prenatal Ultrasonography
Young Lim Shin, Hye Sung Won, Jong Hyun Yoon, Young Seo Park
Clin Exp Pediatr. 2000;43(7):926-932.   Published online July 15, 2000
Purpose : Multicystic dysplastic kidney(MCDK) is characterized by the presence of multiple cysts of varying sizes. It is associated with ureteral or pelviureteral atresia, and a non-functioning kidney. The purpose of this study was to examine the clinical feature and the natural history of MCDK. Methods : A study was performed in 36 children with prenatal diagnosis of MCDK from 1989 to 1999. All...
Case Report
A Case of Megacystis Microcolon Intestinal Hypoperistalsis Syndrome
Jae Woo Lim, Jong Su Shin, Kyuchul Choeh, Chang Su Na, Tae Il Han
Clin Exp Pediatr. 2000;43(2):278-282.   Published online February 15, 2000
Megacystis Microcolon Intestinal Hypoperistalsis(MMIH) Syndrome is a rare cause of functional neonatal bowel obstruction, characterized by hypoperistalsis, narrow distal ileum and colon, and bladder distension. We report a case of MMIH syndrome and review the literature. The patient was born after 34 weeks of gestation with marked abdominal distension. Her birth weight was 2,830g with 276ml of urine drained by...
A Case of Retroperitoneal Cystic Lymphangioma
Chang Ho Lee, Jeong Hwa Choi, Eun Ju Han, Jin Gook Kim, Bung Do Nam, Jae Won Back, Mun Kyu Park
Clin Exp Pediatr. 2000;43(1):111-116.   Published online January 15, 2000
Cystic lymphangiomas are comparatively rare, benign tumors of lymphatic system and their histogenesis are uncertain. About 75% of theses lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region or groin. But retroperitoneal cysticlymphangiomas are very rare. Retroperitoneal cystic lymphangiomas are usually found incidentally during diagnostic procedures performed for unrelated clinical...
A Case of Allergic Bronchopulmonary Aspergillosis During Childhood
Kyoung Ah Ha, Chang Keun Kim, Churl Young Chung, Jung Sook Kim
Clin Exp Pediatr. 1999;42(2):284-288.   Published online February 15, 1999
Allergic bronchopulmonary aspergillosis(ABPA) is an inflammatory disease which causes a hypersensitivity to Aspergillus spores growing in the bronchi. The clinical syndrome is characterized by asthma, recurrent pulmonary infiltrations or mucoid impaction, eosinophilia, and central bronchiectasis. A 12-year-old boy was admitted to our hospital because of right lower chest pain and cystic mass-like shadows on a chest X-ray film. He had...
Original Article
Longterm Follow-up of Autosomal Recessive Polycystic Kidney Disease
June Huh, Il Soo Ha, Hae Il Cheong, Jeong Kee Seo, Yong Choi
Clin Exp Pediatr. 1998;41(12):1693-1699.   Published online December 15, 1998
Purpose : This study was aimed to assess the clinical manifestations and courses of autosomal recessive polycystic kidney disease(ARPKD). Methods : The medical records of 10 children diagnosed as infantile or juvenile ARPKD at Seoul National University Children's Hospital between January, 1984 and December, 1996, were reviewed, retrospectively. Results : The average age at diagnosis was 3 8/12 years(4months-7 3/12 years)...
Case Report
A Case of Choledochal Cyst Type IVa Complicated by Multiple Choledocholithiasis and Recurrent Cholangitis : Therapeutic Endoscopic Retrograde Cholangiopancreatography and Endoscopic Nasobiliary Drainage
Yong Joo Kim, Eon Woo Shin, Soo Jung Choi, Ho Soon Choi, Jeh Hoon Shin
Clin Exp Pediatr. 1998;41(1):115-119.   Published online January 15, 1998
A 5-year-old male patient was admitted due to fever, and right upper abdominal pain for 2 weeks. He showed severe right upper quadrant tenderness on palpation, hepatomegaly 5 cm below the right costal margin, no mass, and no splenomegaly. On biochemical studies, ALT was 380IU/ml, AST 462IU/ml, alkaline phosphatase 1,069IU/ml, γGTP 239IU/ml, and total bilirubin 2.1mg/dl. Endoscopic retrograde cholangiopancreatography (ERCP) showed cylindrical dilatations of...
Original Article
A Study of Homocysteine Metabolism related Neural Tube Defect
Baeck Hee Lee
Clin Exp Pediatr. 1998;41(1):110-114.   Published online January 15, 1998
Purpose : Methylenetetrahydrofolate reductase is an important enzyme in homocysteine metabolism. Since the identification of the gene locus and mutation of methylenetetrahydrofolate reductase, an increasing number of reports have suggested that elevated levels of homocysteine were associated with various kinds of arteriovascular disease and neural tube defect. Our research plans to investigate whether elevated levels of homocysteine are more common with neural tube defect and...
Cerebral palsy, Periventricular echodensity, Cyst, Intraventricular hemorrhage
Kyeung Ho Park, Sang Hee Kim, Hyung Won Lee, Kil Hyun Kim, Hak Soo Lee, Ji Hye Kim, Young Seok Lee
Clin Exp Pediatr. 1997;40(9):1210-1218.   Published online September 15, 1997
Purpose : Although neonatal intensive care and development of obstetrics play a role in improving survival rate in prematurity, cerebral palsy(CP) is still one of the neurologic sequelae. We tried to find what kinds of risk factors in the patients with abnormal neurosonographic findings who developed CP later. This study was performed to predict early enough who will develop CP later and to treat...
Case Report
A Case of Choledochal Cyst with Anomalous Pancreaticobiliary Ductal Union
Je Woo Kim, Hyun Chul Bae, Ki Sup Chung, Seok Joo Han, Eui Ho Hwang
Clin Exp Pediatr. 1997;40(7):1010-1014.   Published online July 15, 1997
Choledochal cysts are congenital or acquired lesions of the biliary tree with a cystic dilatation of the bile duct. We experienced a choledochal cyst accompanying acute pancreatitis caused by anomalous pancreaticobiliary ductal union(APBDU) in a 2 year old boy. The choledochal cyst associated with APBDU is rarely encountered in the clinical field, and thus we report this case with the presentation of abdominal ultrasonogram, computed...
A Case of Multicystic Encephalomalacia in a Liveborn Twin with a Stillborn Co-twin
Mi Ya Park, Kyung Hee Kim, Jae Seung Yang, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1997;40(3):429-433.   Published online March 15, 1997
Multicystic encephalomalacia is the condition defined anatomically by the presence of multiple cavities in the great part of both cerebral hemispheres. The most common cause of the condition was regarded as the circulatory disturbance during the perinatal period. Also,neonatal asphyxia was the most important cause of the circulatory disturbance. But we experienced a case of multicystic encephalomalacia in a liveborn twin with a stillborn...
A Case of Infected Urachal Cyst Mimicking Acute Peritonitis
Myoung-Bum Choi, Yoon-Ok Kim, Ge-Woo Yi, Yun-Kyeong Cho, Jae-Young Lim, Hyang-Ok Woo, Hee-Shang Youn
Clin Exp Pediatr. 1996;39(10):1466-1468.   Published online October 15, 1996
Infected urachal cyst is a rare disorder. While a urachal cyst is usually asymptomatic, the infected cyst may mimic a variety of acute intraabdominal infection. We have experienced a case of infected urachal cyst in a 3 year old boy complaining of high fever and diarrhea. During hospitalization, symptoms and sings of acute peritonitis dominated the clinical feature. Abdominal sonogram, abdominal computerized tomography and cavitogram...
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 Cystic Pancreatoblastoma Associated with Congenital Hemihypertrophy
Hyun Joo Lee, Kyuong Ha Yoo, Young Mi Hong, Kyung hee Kim, Kum Ja Choi, Joung Hyun Yoo, Sung Sook Kim
Clin Exp Pediatr. 1996;39(6):861-865.   Published online June 15, 1996
We experenced a case of cystic pancreatoblastoma associated with congenital hemihypertrophy in a 4months old male. The mass was located on the anterior side of pancreatic head without any connection to the pancreas. After exision of cystic pancreatoblastoma, chemotherapy(FAM regimen) was performed 15 times due to capsular tumor invasion. Until this time there was no drug side effect and metastasis....
Original Article
Evaluation of Severity of Childhood Pancreatitis with Multiple Factor Scoring Systems
Young In Choi, Jeong Kee Seo
Clin Exp Pediatr. 1995;38(12):1653-1663.   Published online December 15, 1995
Purpose : Pancreatitis in children is not common but can be associated with severe morbidity rates. Early prognostic evaluation of acute pancreatitis assists in selecting those patients who should be closely monitored. We studied the clinical characteristics and prognostic factors in patients with acute pancreatitis. Methods : Twenty five children with pancreatitis were included ranging in age from 1.8 to 14.9...
Infantile Choledochal Cyst Presenting with Neonatal Cholestasis; Review of Anatomical and Clinical Aspect
Jae Won Jeong, Jeong Kee Seo, Kwi Won Park, In Won Kim
Clin Exp Pediatr. 1995;38(12):1629-1637.   Published online December 15, 1995
Purpose : Extrahepatic biliary atresia, neonatal hepatitis, Allagille syndrome are most common primary causes of the neonatal cholestasis. Infantile choledochal cyst is another primary disease presenting with neonatal cholestasis but its incidence is so low that it has been reported rarely. Choledochal cyst in infancy has been known to have clinically and anatomically different characteristics from ones after infancy. Thus...
Case Report
A Case of Eosinophilic Cystitis in Child
So Young Park, Seung Joo Lee
Clin Exp Pediatr. 1995;38(8):1155-1159.   Published online August 15, 1995
Since Brown first descirbed the eosinophilic granuloma in 1960, 45 cases of eosinophilic cystitis have been reported in the literature, including 10 pediatric cases. In Korea, only four adult cases were reported. Eosinophilic cystitis is characterized by severe infiltration o eosinophils into the mucosa, submucosa and muscularis of bladder wall which causes irritative voiding symptoms. This lesions have been frequently...
Cystinuria 3 Cases
Ji Eun Choi, Bo Young Yun, Hae Won Park, Jae Hong Park, Il Sue Ha, Hae Il Jeong, Yong Choi, Hwang Choi, In Won Kim
Clin Exp Pediatr. 1995;38(2):245-251.   Published online February 15, 1995
We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed...
Original Article
Acute Hemorrhagic Cystitis(AHC) in Children -Etiology and Clinical Characteristics-
Jin Won Pyo, Eun Hwa Choi, Jin Young Park, Hoan Jong Lee, Hae Il Cheong, Il Soo Ha, Yong Choi, Kwang Myung Kim, Hwang Choi, Je Geun Chi
Clin Exp Pediatr. 1995;38(2):207-215.   Published online February 15, 1995
Purpose : AHC characterized by sudden onset of gross hematuria, dysuria and frequency oc curs in children and young adults as a self-limited disease that should be differentiated from se rious renal disorders. We have performed this study to establish the cause and characterize the clinical features of this illness in Korean children. Methods : 19 cases collected prospectively for 30...
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