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Volume 43(6); Jun 2000
Clinical Lecture
Recent Advance in Congenital Heart Disease
Heung Jae Lee
J Korean Pediatr Soc. 2000;43(6):733-737.   Published online June 15, 2000
Original Articles
A Prospective Epidemiological Study on Birth Defects : A Community Based Pilot Study
Hyon Ju Kim, Yeon Kyeoung Kim, Dae Kyun Koh, Jong Hyun Kim, Bo Won Choi
J Korean Pediatr Soc. 2000;43(6):738-745.   Published online June 15, 2000
Purpose : This prospective pilot study is a part of the Korean NIH’s effort to characterize congenital anomalies and genetic disorders in Korea and to establish a National Genetic Database. Methods : This population-based study was conducted at all hospitals that deliver in the province of Suwon; a total of 39 hospitals from May 1, 1997 to April 30, 1999 for...
Protective Effect of Probucol against Adriamycin-Induced Apoptosis in Cultured Rat Cardiac Myocytes
Sun-Ju Lee, Dong-Seok Lee, Yong-Wook Jung
J Korean Pediatr Soc. 2000;43(6):746-754.   Published online June 15, 2000
Purpose : In adriamycin(ADR)-induced cardiomyopathy, several different mechanisms are suggested. However, little information is available regarding the role of apoptosis. In the present study, we examined the induction of apoptosis on ADR treatment and anti-apoptotic effects of probucol, a lipid-lowering drug, and we also studied the changes of bcl-2 expression in order to see the molecular mechanisms underlying the effect...
Red Blood Cell Indices, Serum Iron and Total Iron Binding Capacity in Children with Helicobacter pylori Infection
Soo Joon Park, Chang Han Lee, Ki Sup Chung
J Korean Pediatr Soc. 2000;43(6):755-762.   Published online June 15, 2000
Purpose : H. pylori infection was recently reported to be associated with unexplained iron-deficiency anemia(IDA) in children and adolescents. H. pylori-related IDA was thought to occur due to GI blood loss, scavenging of iron by H. pylori and iron malabsorption. The aim of this study was to examine how the status of H. pylori infection and age of children affected...
Infantile Hypertrophic Pyloric Stenosis Treated with Intravenous Atropine Sulfate
Jae Woo Lim, Hee Sook Son, Kye Shik Shim, Kyu Chul Choeh, Tae Il Han
J Korean Pediatr Soc. 2000;43(6):763-768.   Published online June 15, 2000
Purpose : The pharmacologic effect of atropine on HPS can be considered to control pyloric muscle spasm. Therefore, we studied the effects of intravenous atropine sulfate on the clinical course of HPS, and periodically observed the ultrasonographic appearance of the pyloric muscles after atropine treatment. Methods : From April 1998 to May 1999, 14 infants who were diagnosed with HPS were...
Changes of Endothelin-1 after Pulmonary Venous Stenosis in Dog Model
Young Mi Hong, Yong Soon Won, Seung Suk Kim
J Korean Pediatr Soc. 2000;43(6):769-778.   Published online June 15, 2000
Purpose : Endothelin-1(ET-1) is a potent vasoconstrictor peptide. It has potent contractile and proliferative effects on vascular smooth muscle cells. Congenital heart diseases are often accompanied by pulmonary hypertension, and the severity of pulmonary hypertension is important in the prognosis. The aim of this study was to elucidate changes of ET-1 after pulmonary venous stenosis in a dog model, and...
Mutations in Hepatitis B Virus Precore and Core Promotor in Children with Chronic Hepatitis B Infection - Comparison Between Vertical and Non-vertical Transmission -
Jin Kuk Kim, Yeong Hong Park, Woo Yeong Chung, Chul Ho Kim
J Korean Pediatr Soc. 2000;43(6):779-791.   Published online June 15, 2000
Purpose : The aims of this study were to investigate the frequencies and role of hepatitis B virus(HBV) precore and core promotor mutations in children with chronic hepatitis B infection. Methods : Sera from 46 children with chronic hepatitis B infection were analyzed by direct sequencing of polymerase chain reaction product of HBV DNA. In this study, the patients were divided...
The Effect of Single Dose Imipramine on Nocturnal Urine Output in Patients with Nocturnal Enuresis
Won Jung Kim, Min Jung Kim, Byeong Hee Son, Sung Won Kim
J Korean Pediatr Soc. 2000;43(6):792-797.   Published online June 15, 2000
Purpose : The aim of the present study was to investigate the effect of single dose imipramine on nocturnal urine output in patients with nocturnal enuresis. Methods : A total of 6 monosymptomatic enuretic patients of more than 5 years of age were enrolled in this study. We measured nocturnal urine output, urine osmolality, creatinine clearance, osmolal clearance, excretion rate of...
Correlation between the Grade of Brain MRI and Clinical Features of Periventricular Leukomalacia
Ju Eun Lee, Su Eun Park, Sang Ook Nam, Hak Jin Kim
J Korean Pediatr Soc. 2000;43(6):798-805.   Published online June 15, 2000
Purpose : Periventricular leukomalacia(PVL) is associated with various neurologic sequelae such as cerebral palsy and cortical blindness. The aim of this study was to analyse the correlation between the degree of PVL on MRI and clinical features or its severity. Methods : Thirty-eight children with PVL on MRI among children brought to Pusan National University Hospital between January 1996 and August...
Clinical Manifestation of Childhood Acute Leukemia with Bone Involvement
Kyoung Eun Jeong, Hee Jung Lee, Kwang Soon Song, Heung Sik Kim, Chin Moo Kang
J Korean Pediatr Soc. 2000;43(6):806-813.   Published online June 15, 2000
Purpose : Bone involvement is known to develop in 40-70% of pediatric acute leukemia. We aimed to analyze the clinical course and result of therapy in pediatric acute leukemia with bone involvement. Methods : Twenty-seven patients diagnosed as pediatric acute leukemia at Dong San Medical Center from Jan. 1996 to Aug. 1998 were evaluated. According to bone X-ray and whole body...
Hemorrhagic Shock and Encephalopathy Syndrome as a Cause of Sudden Death in Infants
Jong Won Lee, Chang Han Lee, Ki Sup Chung
J Korean Pediatr Soc. 2000;43(6):814-819.   Published online June 15, 2000
Purpose : To evaluate the clinical characteristics, treatments and outcome of patients with hemorrhagic shock and encephalopathy(HSE) syndrome. Methods : We performed a clinical study on 14 patients who were diagnosed as hemorrhagic shock and having encephalopathy syndrome in the Department of Pediatrics, from 1984 to 1998. Age, sex, clinical symptoms and physical findings at admission, the most deranged laboratory findings,...
Case Reports
The Management of Three Infants with Critical Valvular Aortic Stenosis by Transcarotid Balloon Aortic Balvuloplasty
Kie-Young Park, Young-Hwue Kim, Ji-Yeon Min, Jae-Kon Ko, In-Sook Park, Chang-Yee Hong, Tae-Jin Yun, Dong-Man Seo
J Korean Pediatr Soc. 2000;43(6):820-827.   Published online June 15, 2000
Critical valvular aortic stenosis(AS) is a life threatening congenital heart anomaly in neonates or infants. The main cause of death is rapidly progressing left ventricular failure. The treatment goal of critical AS is to save left ventricular function early. Before the 1980s, the preferred treatment was surgical valvotomy, however, that operative method had a high mortality rate and risk of...
Isovaleric Acidemia in Siblings Diagnosed by Organic Acid Analysis
Kyung Soo Cheon, Dong Hwan Lee
J Korean Pediatr Soc. 2000;43(6):828-831.   Published online June 15, 2000
Isovaleric acidemia is an inborn error in metabolism due to a defect in isovaleryl-CoA dehydrogenase. Accumulation of serum isovaleric acid causes poor feeding, vomiting, lethargy, hypothermia, convulsion, mental retardation, etc. It is inherited as an autosomal recessive trait. Since the first reports of isovaleric acidemia by Tanaka et al in 1966, more than 60 cases have been reported. There are...
A Case of Candida Endocarditis with Vegetation on the Tricuspid Valve in a Preterm Infant
Hye Young Han, Mi Jin Jung, Kwang Hoon Lee, Gil Hyun Kim, Hak Soo Lee, Guk Yang Park, Young Ha Oh
J Korean Pediatr Soc. 2000;43(6):832-836.   Published online June 15, 2000
Fungal endocarditis is a rare disease in infants, but it has been reported with increasing frequency among premature infants requiring neonatal intensive care. Congenital heart disease, prolonged intravenous catheterization, the use of intravenous alimentation, broad-spectrum antibiotics administration and narcotic addiction are risk factors. Candida endocarditis is an unusual but severe complication of systemic candidiasis. Its occurrence has been related to...
Mauriac Syndrome in a Patient with Type I Diabetes Mellitus
Ji Yeoun Kang, Pil Soon Yang, Hyung Su Kim, Ok Young Kim, Chul Hoi Koo, Wha Mo Lee
J Korean Pediatr Soc. 2000;43(6):837-841.   Published online June 15, 2000
Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed...
A Case of Diffuse Aspiration Bronchiolitis in a Dysphagic Infant
Ok Ja Choi, Bong Seong Kim, ung Hye Park, Soo-Jong Hong
J Korean Pediatr Soc. 2000;43(6):842-845.   Published online June 15, 2000
Diffuse aspiration bronchiolitis is defined as a clinical entity characterized by a chronic inflammation of bronchioles caused by recurrent aspiration of foreign particles. Clinical symptoms are bronchorrhea, bronchospasm, and dyspnea, and chest radiographs show the presence of regional or disseminated small nodular shadows and hyperlucency. Chest CT should help in detecting diffuse nodular shadows of bronchiolitis. Pathologic findings of diffuse...
A Case of Chronic Granulomatous Disease in which Enterococci were Observed in Culture
Kyunglae Cho, Jeonghee Kim, Eunjeong Kim, Churwoo Yoo, Chunsoo Kim, Unseok Nho, Chulkyu Kim
J Korean Pediatr Soc. 2000;43(6):846-850.   Published online June 15, 2000
Chronic granulomatous disease(CGD) is a genetically inherited disorder caused by the failure of phagocytic cells to produce superoxide. It is characterized by frequent and uncontrollable infection which often lead to death in early childhood. The first clinical signs may be confined to skin and manifest themselves as abscesses, pyoderma, eczema or draining sinuses. The disease was first reported in 1957...
A Case of Shaken Baby Syndrome
Jin Choi, Hyun Hee Kim, Won Bae Lee
J Korean Pediatr Soc. 2000;43(6):851-855.   Published online June 15, 2000
Shaken baby syndrome refers to the group of nonaccidental injuries occuring in infants and children as a consequence of violent shaking. The characteristic injuries include subdural and subarachnoid hemorrhages, retinal hemorrhages, traction-typed metaphyseal fracture of long bone and fractures of the ribs. General physical findings may include bruising and burns, but sometimes no extracranial injuries are detected. Affected children are...
A Case of Transient Hyperphosphatasemia
Sung Kee Kim, Yong Won Park, Chong Guk Lee
J Korean Pediatr Soc. 2000;43(6):856-860.   Published online June 15, 2000
We examined a male infant with transient increase in alkaline phosphatase(ALP) activity. The 25-month-old infant was admitted to a local hospital because of pneumonia of unknown etiology. Initial laboratory investigation revealed a serum ALP of 11,260U/L, which was comparable to that of bone isoenzyme on the electrophoresis pattern. There was no evidence of hepatitis, skeletal or intestinal diseases. The hyperphosphatasemia...
A Case of Neonatal Alloimmune Thrombocytopenia due to Anti-HLA A2
Sung Young Kim, Eun Ryoung Kim, Young Jin Kim, Myoung Hee Park, Eun Young Song, Kyou Sup Han
J Korean Pediatr Soc. 2000;43(6):861-865.   Published online June 15, 2000
Neonatal alloimmune thrombocytopenia(NAIT) is a rare disease caused by maternal alloimmunization against fetal platelet surface antigen, which is mainly platelet specific alloantigen or human leukocyte antigen(HLA). During routine hemotology, we accidentally discovered thrombocytopenia in a female fullterm newborn admitted due to jaundice. We excluded NAIT due to human platelet alloantigen(HPA), because the HPA of the mother and baby were the...
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