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Original Article
Microdeletion of Chromosome 7 in Williams Syndrome and Supravalvular Aortic Stenosis
Ho Sung Kim, Yoon Sung Kang, Kyung Hyo Kim, Young Mi Hong, Yong Soo Yun, Kwang Ho Lee
Clin Exp Pediatr. 1999;42(1):47-59.   Published online January 15, 1999
Purpose : Williams syndrome is characterized by supravalvular aortic stenosis, mental retardation and peculiar facial appearance. Its genetic etiology is considered to be a hemizygotic deletion in Chromosome 7q11.23, which includes the elastin gene. We examined the hemizygotic deletion of Chromosome 7q11.23 in 12 Korean Williams syndrome patients and 8 patients with isolated supravalvular aortic stenosis and performed deletion mapping...
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)...
Anogenital Index of Normal Children and Its Clinical Significance in Children with Constipation
Kyung Rye Moon
Clin Exp Pediatr. 1998;41(12):1669-1674.   Published online December 15, 1998
Purpose : The purpose of this study was to find an objective criteria to determine the position of normal anus in Korean children and to evaluate the clinical significance of anterior displacement of anus in children with constipation. Methods : Three hundred and thirty-nine normal children and 24 children with idiopathic constipation represented the basis of this study. The position of the normal anus...
A Clinical Study of Histiocytosis Syndrome
Jong Jin Kim, Jae Kook Cha, Kon Hee Lee, Jong Young Park
Clin Exp Pediatr. 1998;41(11):1575-1582.   Published online November 15, 1998
Case Report
A Case of Ectopic Pancreas with Malrotation
Il Song Kang, Kil Soo Chung, Jae Kook Cha, Kon Hee Lee, Kwan Seop Lee, Byung Chun Kim, Eun Sook Nam
Clin Exp Pediatr. 1998;41(10):1438-1441.   Published online October 15, 1998
Ectopic pancreas is defined as pancreatic tissue lacking anatomical and vascular continuity with the main body of the pancreas. Common symptoms are epigastric pain, hemorrhage, chest pain, weight loss, nausea and vomiting. A 2-month-old male patient was hospitalized because of continuous bile stained vomiting for 2 days. Abdominal ultrasonography and colon study showed malrotation of the intestine. Surgical exploration revealed...
A Case of Inappropriate ADH Secretion Secondary to Vinblastine Therapy
Eun-Hye Park, Hyun-Sang Cho, Hae-Sook Yoo, Chuhl-Joo Lyu, Chang-Hyun Yang, Kir-Young Kim
Clin Exp Pediatr. 1998;41(10):1430-1432.   Published online October 15, 1998
Inappropriate secretion of antidiuretic hormone(ADH) secondary to vincristine therapy has been reported frequently. But there has been five previous reports of vinblastine associated SIADH in the world and in 4 reports, bleomycin and cis-platinum were used along with vinblastine together. Because penetration of vinka alkaloids into CSF of humans is known to be poor, it has been suggested that lowering...
A Case of Congenital Self-Healing Reticulohistiocytosis
Taek Sun Kim, Chang Wan Kim, Eun Ryoung Kim, You Mee Kang, Kye Yong Song
Clin Exp Pediatr. 1998;41(10):1424-1429.   Published online October 15, 1998
Congenital self-healing reticulohistiocytosis(CSHR) is a rare variant of cutaneous reticulohistiocytosis. We report a newborn who had about 25 reddish brown papulonodular lesions on whole body at birth. No extracutaneous involvement was found. All skin lesions involuted within 4 months spontaneously. Biopsy of a skin lesion showed marked aggregated histiocytic infiltration with some eosinophils. S-100 protein and lysozyme were positive. Electron...
Original Article
Thyroid Hormone Concentrations in Preterm Infants
Sung Keun Moon, Jeong Nyun Kim, Mi Jung Park, Myung Jae Choi, Chrul Young Chung
Clin Exp Pediatr. 1998;41(10):1324-1328.   Published online October 15, 1998
Purpose : To establish reference ranges for thyroid hormone concentrations in premature infants, we measured T4 and thyroid stimulating hormone(TSH) concentrations and analyzed the relation to gestational age and birth weight. Methods : Serum T4 and TSH concentrations were measured by radioimmunoassay for 391 premature infants born in Sanggye Paik Hospital for two years and eight months. Results : Gestational age of...
Case Report
A Case of Congenital Intestinal Lymphangiectasia
Pan Joo Lim, Jae Ock Park, Dong Hwan Lee, Sang Mann Shin, Dong Won Ki
Clin Exp Pediatr. 1998;41(9):1293-1298.   Published online September 15, 1998
Intestinal lymphangiectasia is a primary or secondary disorder of lymphatics which is associated with protein-losing enteropathy, hypoalbuminemia, edema, abdominal distension and growth failure. The patients with this disease lose albumin, immunoglobulins, and lymphocytes through the bowel. We have experienced one case of intestinal lymphangiectasia in 8 month-old boy, whose chief complaint was abdominal distension and watery diarrhea. He had hypoalbuminemia(1.7gm/dl)....
Original Article
Correlation Between Clinical and Pathological Prognositic Factors of IgA Nephropathy in Children
Hwang Jae Yoo, Bon Su Ku, Young Tak Lim, Su Yung Kim
Clin Exp Pediatr. 1998;41(8):1093-1101.   Published online August 15, 1998
Purpose : Clinical and pathological prognostic factors of idiopathic IgA nephropathy have been reported, but mostly in adults and a few in children. Especially studies about correlation between those factors are very rare. Methods : We studied 58 children patients who were hospitalized to our clinics and diagnosed as IgA nephropathy by renal biopsy from Jan. 1989 to Jun 1996. They...
Case Report
A Case of Neonatal Hyperthyroidism with Unilateral Ear Anomaly
Kwang Ok Chung, Na Yeon Kim, Sung Min Cho, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi
Clin Exp Pediatr. 1998;41(6):845-849.   Published online June 15, 1998
Neonatal hyperthyroidism is a very rare disorder occurring typically in the offspring of patients with Graves' disease or chronic thyroiditis. It is caused by the transplacental passage of thyroid stimulating antibodies(TSAb) from the mother to the fetus. There has been few reports of neonatal hyperthyroidism associated with congenital anomalies. We experienced a case of neonatal hyperthyroidism with unilateral microtia and agenesis of external auditory canal...
Original Article
Immunomodulation Therapy in Aplastic Anemia : Relapse Rate, Complications and Long-term Survival During Follow-up for More than 1 Year
Jun Ah Lee, Hyoung Jin Kang, Hyo Jeong Han, Hyoung Soo Choi, Eun Sun Yoo, Hee Young Shin, Joong Gon Kim, Hyo Seop Ahn
Clin Exp Pediatr. 1998;41(6):775-784.   Published online June 15, 1998
Purpose : We evaluated the response to immunomodulation therapy, long-term survival and relapse rate of aplastic anemia during follow-up for more than 1 year. Methods : Twenty-eight children, with moderate to severe aplastic anemia were followed for more than one year and 7 children expired after therapy, were analyzed. Antilymphocyte globulin(ALG) or antithymocyte globulin(ATG) by itself was given to 27 patients, and cyclosporine A(CsA)...
A Clinical Study of Survival Rate and Prognostic Factors in Advanced Neurobalstoma in Children
Seung Yang, Ho Joon Im, Nam Su Kim, Hahng Lee
Clin Exp Pediatr. 1998;41(5):684-694.   Published online May 15, 1998
Purpose : Despite the use of improved therapeutic modalities, long-term survival remains poor in patients in stage Ⅲ or Ⅳ of neurobalstoma and for over 1 year olds at diagnosis. We evaluated the long-term survival rate and related prognostic factors in advanced neuroblastoma. Methods : Twenty-nine children diagnosed with stage Ⅲ or Ⅳ neuroblastoma after 1 year of age, between May...
Etiological Analysis of Gynecomastia in Children
Jai Keun Kim, Ki Keun Oh, Choon Sik Yoon, Jae Hyun Park
Clin Exp Pediatr. 1998;41(2):224-229.   Published online February 15, 1998
Purpose : The purpose of this study is to analyze the etiology of gynecomastia confirmed by breast operation among children under the age of 15 exhibiting clinical symptoms. Methods : Twenty patients under the age of 15 were diagnosed as having gynecomastia in the past 10 years. The number of pathologically confirmed gynecomastia patients was 15. The causes or related...
Serum Pepsinogen Ⅰ, Ⅱ Levels and Upper Gastrointestinal Diseases in Children with H. pylori Infection
Je Woo Kim, Ki Sup Chung
Clin Exp Pediatr. 1998;41(2):200-208.   Published online February 15, 1998
Purpose : In H. pylori infection serum pepsinogen Ⅰ/Ⅱ ratio decreases and pepsinogen Ⅱ level is increased. Increased serum pepsinogen Ⅱ level correlates with pathologic severity of gastritis. We assayed serum pepsinogen Ⅰ, Ⅱ levels and pepsinogen Ⅰ/Ⅱ ratio to evaluate the relationship between the serum pepsinogen level and upper gastrointestinal diseases. Methods : One hundred and sixty-five children who had...
A Result of Treatment of Malignant Germ Cell Tumors in Childhood
Hyoung Jin Kang, Jun Ah Lee, Hyo Jeong Han, Hyoung Su Choi, Hyeon Jin Park, Ki Woong Sung, Eun Sun Yoo, Hee Young Shin, Hyo Seop Ahn, Kwi Won Park, Byung Keu Cho, Hwang Choi, Chul Woo Kim
Clin Exp Pediatr. 1997;40(12):1707-1718.   Published online December 15, 1997
Purpose : Germ cell tumors have variable clinical characteristics according to the age, sex, primary site, and pathologic diagnosis. To provide the therapeutic principle, the clinical characteristics, response to the treatment, and prognostic factors were analyzed. Methods : Medical records of 57 children who were diagnosed as malignant germ cell tumor in Seoul National University Children' s Hospital from January 1986 till June 1996 were...
Case Report
A Case of Spontaneouse Hemothorax Associated with a Posterior Mediastinal Endodermal Sinus Tumor in a Female Patient
Eui Seok Park, Il Tae Hwang, Ghee Young Jung, Du Bong Lee, Young Sook Han, Hyo Seon Choi, Mi Kyeong Ji
Clin Exp Pediatr. 1997;40(10):1465-1469.   Published online October 15, 1997
Primary endodermal sinus tumor(yolk sac tumor) of the mediastinum is rare. Most patients are young and male, and the great majority of tumors are found in the anterior mediastinum, and spontaneous hemothorax is a rare presentation of intrathoracic tumors. We report a case of spontaneous hemothorax associated with a posterior mediastinal endoderml sinus tumor occuring in a female patient. Brief review and related literatures...
A Case of Intestinal Amebiasis with Protein Losing Enteropathy
Chan Young Pak, Hee Taeg Kim, Soo Young Choi, Yun Jong Kang, Yeon Chung Chung, Jin Keun Ghang, Jeong Kee Seo
Clin Exp Pediatr. 1997;40(10):1458-1464.   Published online October 15, 1997
Amebiasis is an infectious disease caused by Entameba histolytica. Amebiasis remains an extremely important consideration in the differential diagnosis of diarrhea, especially when there is associated bleeding. It is imperative that appropriate studies to establish or exclude the diagnosis of amebiasis be carried out in all patients who present with a clinical and sigmoidoscopic picture of colitis, and that patients treated with metronidazole for amebiasis...
Original Article
Constipation in Children-Colonic Transit Time and the Barr-Score
Nam-Seon Beck, I Sok Kang, Dong Kyu Jin, Munhyang Lee, Sang Il Lee, Heung Jae Lee, Bokyung Kim, Tae Sung Park
Clin Exp Pediatr. 1997;40(10):1427-1434.   Published online October 15, 1997
Purpose : We have assessed the clinical usefulness of solid maker transit technique and Barr-score in 31 patients with idiopathic constipation (male:female; 17:14, mean age; 6.7 years, range 1.4-12 years). Methods : All patients underwent full history taking and physical examination including rectal examination. On first visit to gastroenterology clinic a plain abdominal film was taken for Barr-score which evaluated by two observers without the...
Crying Pattern of Infants by Crying Pattern Questionnaire
Keun Lee
Clin Exp Pediatr. 1997;40(8):1091-1097.   Published online August 15, 1997
Purpose : Studies done in Western societies have described characteristics of infant crying behavior. These include:crying peak in the second month, diurnal rhythm(typified by clustering during evening hours) and crying that is robust to differences in caretaking styles. This survey was undertaken to compare the crying patterns of Korean babies with those of their Western counterparts. Methods : From June 1995 to March 1996, well...
Case Report
A Case of Congenital Acute Megakaryoblastic Leukemia with Down Syndrome
Nan Yee Kye, Kon Hee Lee, Jae Kook Cha, Hye Sun Yoon, Won Keun Song
Clin Exp Pediatr. 1997;40(4):578-583.   Published online April 15, 1997
We experienced a case of congenital acute megakaryoblastic leukemia with Down syndrome. The patient was admitted due to characteristic facial figure of Down syndrome and abdominal distension. Acute megakaryoblastic leukemia was diagnosed with abundant megakaryoblast in peripheral blood smear, severe myelofibrosis in bone marrow biopsy and positive platelet glycoprotein Ⅲa receptor. On third hospital day, the patient expired due to DIC...
A Case of Lymphocytic Interstitial Pneumonia
Ho Young Lee, Hwang Min Kim, Mee Kyung Namgoong, Jong Soo Kim, Soon Hee Jung, Dong Jin Kim
Clin Exp Pediatr. 1997;40(4):572-577.   Published online April 15, 1997
Lymphoid interstitial pneumonia(LIP) is an uncommon process in childhood, and is characterized by interstitial accumulation of mature lymphocytes, plasma cells and reticuloendothelial cells. LIP is believed to have autoimmune or immunologic pathogenesis, because it is frequently associated with acquired immune deficiency syndrome, Epstein-Barr virus infection, common variable immunodeficiency. The patient described in this report had idiopathic thrombocytopenic purpura, Evans syndrome...
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 Granular Acute Lymphoblastic Leukemia with t(5p;5p) Arising in Down Syndrome Infant
Soon Ja Lee, Young Ho An, Hai Lee Chung
Clin Exp Pediatr. 1997;40(3):417-422.   Published online March 15, 1997
Granular lymphoblast which is characterized by the presence of clearly defined azurophilic cytoplasmic granules are a relatively uncommon finding and indicate a negative impact on prognosis of childhood ALL. Granular ALL is more common in FAB L2 cases but there is no significant difference by immunophenotype and no specific cytogenetic abnormality correlated with clinical significance of granular ALL has been reported. We present a case...
Original Article
Risk factors for Pulmonary Interstitial Emphysema(PIE) in Mechanically Ventilated Neonates with Hyaline Membrane Diseas
Shin Won Yoon, Chul Lee, Jeong Nyun Kim, Ran Namgung, Dong Gwan Han, Myung Joon Kim
Clin Exp Pediatr. 1997;40(3):318-326.   Published online March 15, 1997
Purpose : Pulmonary interstitial emphysema(PIE) is a common and serious complication of mechanical ventilation in infants with hyaline membrane disease. This abnormal collection of gases has two basic roentgenographic features; linear and cyst-like radiolucencies. High positive inspiratory pressure was found to be the most significant parameter associated with development of fatal pulmonary interstitial emphysema. Without prompt conservative management such as lowering peak inspiratory...
Caustic Esophagitis and Gastrofiberoscopy in Children
Hyeon Jeong Kim, Jeong Kee Seo
Clin Exp Pediatr. 1996;39(11):1556-1564.   Published online November 15, 1996
Purpose : There have been many accidental ingestions of strong acid or alkali in early childhood. If the patient survives the acute effects of caustic ingestion, the reparative response can result in esophageal and gastric stenosis. However there have been few endoscopic studies on caustic esophagitis in children. The aim of this study was to review the contribution of the endoscopy to the diagnosis...
Clinical Course of the Ostium Secundum Atrial Septal Defect(ASD) and Its Rate of Spontaneous Closure
Dae Yong Kim, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 1996;39(11):1538-1543.   Published online November 15, 1996
Purpose : To know the natural course of the ostium secundum ASD including its rate of spontaneous closure. Methods : We studied 259 cases who were diagnosed as ostium secundum ASD by 2D-echocardiography at Department of Pediatrics, Kyungpook National University Hospital between Jan. 1985 and Dec. 1994. According to the age at diagnosis, we divided them into two groups, group I (those diagnosed before 1...
Case Report
A Case of Infantile Acute Hemorrhagic Edema
Hyun Jeong Lee, Dong Won Lee, Si Yong Kim, Baik Kee Cho, Chang Kyu Oh
Clin Exp Pediatr. 1996;39(8):1168-1172.   Published online August 15, 1996
Infantile acute hemorrhagic edema(IAHE) is a variant of leukocytoclastic vasculitis which is characterized by painful edematous purpura of cockade pattern on the head and distal extremities. The lesions are limited to the skin and complete recovery occurs within 1 to 3 weeks. Recently, it is accepted as a separate entity from Henoch-Schönlein purpura. A nine month-old boy presented multiple cockade ecchymotic plaques and inflammatory...
A Case of Intestinal Neuronal Dysplasia
Se Young Kim, Yon Ho Choe, Jeong Kee Seo, In One Kim, Chong Jai Kim
Clin Exp Pediatr. 1996;39(8):1151-1157.   Published online August 15, 1996
Intestinal neuronal dysplasia(IND) is a disease characterized clinically by symptoms of intestinal obstruction and pathologically by hyperplasia of the submucosal and myenteric plexuses with formation of giant ganglia. Chronic intestinal pseudo-obstruction is a clinical diagnosis, composed of myopathic form and neuropathic form, and normal intestinal histology. Intestinal neuronal dysplasia is a neuropathic form of chronic intestinal pseudo-obstruction. The clinical presentation and the course of IND...
Original Article
Cytodiagnostic Urinalysis in the Differentiation of Leukocyturias of Children with Bacterial Urinary Tract Infection (UTI) from Those of Children without UTI
Il Soo Ha, Hae Il Cheong, Young Joon Hong, Jin Q Kim, Yong Choi
Clin Exp Pediatr. 1996;39(8):1122-1129.   Published online August 15, 1996
Purpose : Although routine urinalysis can detect leukocyturia, difficulties remain in differentiating subpopulations of leukocytes and various epithelial cells in urinary sediments. This study was performed to appreciate the value of cytodiagnostic urinalysis in the discrimination of leukocyturias of bacterial urinary tract infection (UTI) from those without UTI. Methods : Urine from 12 children with UTI (UTI group) and from 11 children without UTI (non-UTI...


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