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Original Article
Doppler Echocardiographic Assessment of the Changes in Pulmonary Arterial Pressure in Preterm Infants with Respiratory Distress Syndrome
Sejung Sohn, Eun Ae Park
Clin Exp Pediatr. 1999;42(6):790-799.   Published online June 15, 1999
Purpose : The ratio of time to peak velocity(AT) and right ventricular ejection time(ET) as measured from the pulmonary artery Doppler waveform showed a close inverse correlation with pulmonary arterial pressure. The purpose of this study was to see the pattern of change in pulmonary arterial pressure assessed by AT/ET in preterm infants with respiratory distress syndrome (RDS). Methods : The...
Case Report
A Case of 18q-Deletion Syndrome with Hydronephrosis and Anhydrosis
Mun Hee Kim, Kee Hwan Yoo, Young Sook Hong, Soon Kyum Kim
Clin Exp Pediatr. 1999;45(5):711-715.   Published online May 15, 1999
The 18q-syndrome is a deletion disorder that occurs in humans. Clinical symptoms are mental retardation, craniofacial anomalies, skeletal deformity, seizure, and hearing loss. 18q- deletion occurs over a broad region, spanning the interval from 18q22.2 to 18qter rather than a single critical region containing 18q. We experienced a case of 18q-syndrome in a male child. It was diagnosed by clinical...
A Case of Idiopathic Hypoparathyroidism Associated with Graves' Disease
Eun Young Jang, Hwang Min Kim, Baek Keun Lim, Jong Soo Kim
Clin Exp Pediatr. 1999;42(5):716-721.   Published online May 15, 1999
Graves' disease is a relatively rare endocrinologic disorder in childhood age and often associated with other endocrinologic disorders such as type I diabetes mellitus and Addison's disease etc. Also, it is associtated with non-endocrinologic autoimmune diseases such as systemic lupus erythematosus, myasthenia gravis, idiopathic thrombocytopenic purpura, vitiligo and pernicious anemia. However, idiopathic hypoparathyroidism associated with Graves' disease is very...
Levamisole-Induced Reversible Agranulocytosis in Children with Steroid Dependent Nephrotic Syndrome
Su Jin Lee, Su Ja Hwan, Eun Seon Yoo, Seung Joo Lee
Clin Exp Pediatr. 1999;42(4):576-579.   Published online April 15, 1999
Levamisole has been used for nephrotic syndrome due to its immunostimulating, immunomodulating, and steroid-sparing effects. Agranulocytosis, a serious side effect of levamisole, was rare and mostly associated with autoimmune disease, neoplastic disease and HLA B27 except one case in a nephrotic syndrome who was treated with high-dose(5mg/kg QOD) levamisole. This 15 year-old girl with steroid dependent nephrotic syndrome, who was...
Original Article
Clinical Trial of Domestically Developed Bovine Lung Surfactant YY-38 in Neonatal Respiratory Distress Syndrome
Chul Lee, Jeong Nyun Kim, Min-Soo Park, Moon Sung Park, Shin Won Yoon, Wook Chang, Ran Namgung, Kook In Park, Dong-Gwan Han
Clin Exp Pediatr. 1999;42(4):472-483.   Published online April 15, 1999
Purpose : We previously reported modified bovine lung surfactant YY-38(NewfactanⓇ) had a low surface tension, good hysteresis, and exhibited good pressure-volume curve in animal experiment (J Korean Pediatr Asso 1997;40:771-85). We performed multicenter clinical trial of NewfactanⓇ in neonatal RDS. Methods : Seventy-seven infants with RDS(GA 31.8¡¾2.9 wks and BW 1,809¡¾592 gm) in 4 NICU were enrolled. After administration of...
Case Report
Two Cases of Oculocerebrorenal Syndrome of Lowe
So Won Kim, Young Suk Yu, In-One Kim, Hae Il Cheong, Yong Seung Hwang, Yong Choi
Clin Exp Pediatr. 1999;42(3):419-423.   Published online March 15, 1999
Oculocerebrorenal syndrome of Lowe(OCRL) is a rare X-linked disorder characterized by congenital cataract(oculo-), hypotonia, developmental delay, cognitive impairment(cerebro-), renal tubular dysfunction(renal), and growth retardation. Recently, the defective gene, OCRL-1 gene encoding [PtdIns(4,5)P2] 5-phosphatase, was cloned with mutations identified in patients. Although there have been about 200 cases of OCRL reported in English literature, only three reports have been published in...
Original Article
MELAS Syndrome Confirmed by Mitochondrial DNA Analysis in Siblings
Young Ghil Rah, Soo Ahn Chae, In Suk Lim, Dong Keun Lee, Byoung Hun Yoo, Tae Sung Ko, Han Wook Yoo
Clin Exp Pediatr. 1999;42(3):412-418.   Published online March 15, 1999
MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome is a major subgroup of mitochondrial myopathy. Recent advances in molecular genetics revealed specific mutations in mitochondrial DNA which cause MELAS. We described here clinical and molecular genetic findings of sister and brother with MELAS syndrome. For molecular genetic studies, DNAs from peripheral blood nucleated cells were used. And the substitution...
Case Report
A Case of Type Ⅱ Ehlers-Danlos Syndrome
Dong Hwan Oh, Jin Kuk Kim, Beuyng Do Nam, Ju Eun Lee, Je Hong Park
Clin Exp Pediatr. 1999;42(2):268-273.   Published online February 15, 1999
Ehlers-Danlos syndrome represents a group of inherited connective tissue disorders characterized by varying degrees of joint laxity, skin fragility and hyperextensibility, and a bleeding tendency. The essential defect is a quantitative deficiency of collagen. At present, 10 clinical forms have been recognized on the basis of extent and severity of the principal features, in combination with other abnormalities and the...
A Case of Acute Myeloblastic Leukemia Transformed from Transient Myeloproliferative Disorder with Down Syndrome
Jae Eun Lee, Seung Woo Baeck, Wan Seob Kim, Chun Hee Lee
Clin Exp Pediatr. 1999;42(1):128-132.   Published online January 15, 1999
Individuals with Down syndrome have a high incidence of hematologic diseases such as transient myeloproliferative disorder(TMD) & acute leukemia. Because it is difficult to distinguish TMD from acute myeloblastic leukemia, the diagnosis in neonate, who have Down syndrome, should be made with extreme caution. TMD usually undergoes spontaneous remission within a few months, but acute leukemia can develope after remission...
Original Article
The Effect of Long-term Steroid Therapy on Cardiac Function in Nephrotic Syndrome
Jae-Goo Lee, Hong-Ryang Gil, Jae-Ho Lee, Yong-Hun Chung
Clin Exp Pediatr. 1999;42(1):102-107.   Published online January 15, 1999
Purpose : The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. Methods : The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6...
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...
A Nationwide Clinical Study of Acute Respiratory Distress Syndrome in Children
Soo Jung Choi, Do Suck Jeong, Jae Won Oh, Ha Baik Lee, Im Ju Kang, Kwang Woo Kim, Kyu Earn Kim, Ki Bok Kim, Young Ho Rah, Kang Seo Park, Byong Kwan Son, Ki Young Lee, Sang Il Lee, Ji Tai Choung, Young Mi Hong
Clin Exp Pediatr. 1999;42(1):23-31.   Published online January 15, 1999
Purpose : Acute respiratory distress syndrome(ARDS) is the final course of acute lung injury. It results from various etiological origins and pathophysiologic mechanisms, and has a mortality rate of approximately 60-70%. Although the confirmative incidence of ARDS in children is yet unknown, the increasing incidence of ARDS has been reported in Korea. In the present study, we report ARDS diagnosed...
Case Report
A Case of VURD Syndrome
Sun Young Kim, Eu Jun Yang, Jae Hong Park, Su Young Kim, Sang Don Lee
Clin Exp Pediatr. 1998;41(12):1722-1725.   Published online December 15, 1998
VURD syndrome is a disorder characterized by a posterior urethral valve, unilateral reflux, and ipsilateral renal dysplasia. This syndrome is not uncommon, with an incidence in the range of 15 to 20% in patients with posterior urethral valve. Etiology is not clear, but there are a few reports that renal dysplasia is either a result of hydrostatic pressure transmitted to...
A Case of FGFR2 Exon Ⅲc Mutation in Crouzon Syndrome
Seon Chan Bae, Eun Ha Lee, Moon Sung Park, Si Houn Hah, Chang Ho Hong
Clin Exp Pediatr. 1998;41(12):1717-1721.   Published online December 15, 1998
Crouzon syndrome, an autosomal dominant disorder, has characteristic features of craniosynostosis, hypertelorism, exophthalmos, maxillary hypoplasia and relative mandibular prognathism. Mutations of fibroblast growth factor receptor 2(FGFR2) gene are associated with craniosynostotic conditions, such as Crouzon syndrome, Jackson-Weiss syndrome, Pfeiffer syndrome, Apert syndrome and Beare-Stevenson cutis gyrata. We found one child with common morphological features of Crouzon syndrome. Interestingly, she...
Original Article
Long-term Follow-up Study of Children with Minimal Change Nephrotic Syndrome
Jung Sue Kim, Hae Il Cheong, Hyun Soon Lee, Yong Choi
Clin Exp Pediatr. 1998;41(12):1675-1684.   Published online December 15, 1998
Purpose : Most of childhood MCNS has a long disease course with frequent relapses. This study was designed to analyze the long-term clinical course of childhood MCNS, focusing at relapsing pattern, treatment response and complications. Mothods : The medical records of 137 children with biopsy-proven MCNS observed during 1976 ti 1996 were analyzed retrospectively. They were classified as initial responders(111 patients,...
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
Relationship of Recurrent Abdominal Pain Syndrome and Gastric Emptying Time
Eui Jun Yang, Jae Hong Park, In Ju Kim
Clin Exp Pediatr. 1998;41(11):1538-1548.   Published online November 15, 1998
Purpose : Recurrent abdominal pain syndrome(RAPS) is not uncommon in school-aged children, but the etiology and pathogenesis are not understood well. But recent studies suggest that motility disorder makes up a majority of the pathogenesis. The aim of this study was to investigate gastric emptying time(GET) which is a method to evaluate gastrointestinal motility in patients with recurrent abdominal pain...
Case Report
A Case of Williams Syndrome Diagnosed by FISH
Hyun Kim, Won Bae Lee, Won Bae Lee
Clin Exp Pediatr. 1998;41(10):1433-1437.   Published online October 15, 1998
Williams syndrome was first reported in 1961, it is characterized by distinct facial changes, growth deficiency, mental retardation, congenital heart defect(particularly the supravalvular aortic stenosis), associated at times with infantile hypercalcemia. The diagnosis of this disease relied on phenotype, after recent acknowledgement of its genetic basis on the 7th chromosomal locus q11.23 involving elastin gene on the PCR. Recently, commercial...
Original Article
The Clinical Study on Marfan Syndrome
Jung Sim Kim, Dong Kyu Jin, See Hwan Ko, Jee Yeon Min, I-Seok Kang, Kye Won Jeon, Yung Lan Choi, Heung Jae Lee, Seung Woo Park, Won Ro Lee, Chong Suh Lee, Sei Yeul Oh, Chang Won Ki, Han Wook Yoo, In Sook Park, Jae Kon Ko
Clin Exp Pediatr. 1998;41(10):1411-1416.   Published online October 15, 1998
Purpose : The aim of this study was to assess the involvement of several organs patients with Marfan syndrome in Korea. Also the clinical features in childhood patients with Marfan syndrome were assessed. Methods : Thirty-eight cases of Marfan syndrome were enrolled in this study. Clinical evaluations of the musculoskeletal, cardiovascular and occular system were performed in all cases. Results : The...
The Effect of Steroid Therapy on Growth and Bone Density in Children with Nephrotic Syndrome
Seong Hoi Jeon, Ae Yeon Lim, Young Kook Kim, Hae Won Cheon, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 1998;41(10):1396-1402.   Published online October 15, 1998
Purpose : Long-term steroid therapy for various glomerular disorders in children has been known to decrease serum Vit D3 level and develop osteomalacia. The aim of this study was to observe the effect of long-term steroid therapy on growth and bone density in children with nephrotic syndrome. Methods : Bone density of 17 steroid-treated nephrotic syndrome was compared with that of...
Case Report
A Case of Polyglandular Autoimmune Syndrome
Kyung Ah Choi, Ji Yeoun Kang, Chul Hoe Koo, Wha Mo Lee, Young Suk Jeon
Clin Exp Pediatr. 1998;41(9):1299-1303.   Published online September 15, 1998
When dysfunction of two or more endocrine glands occurs in association with circulating organ specific antibodies directed against the involved glands, the term polyglandular autoimmune(PGA) syndrome is applied. This syndrome is usually classified into three groups. The autoimmune nature of this disease has been based on the presence of lymphocytic infiltration of the affected glands, organ specific autoantibody in serum,...
Original Article
Prevalence and Clinical Features of Wolff-Parkinson-White(WPW)- Type Ventricular Preexcitation Syndrome in Children
Byung Ju Kim, Jae Sook Ma
Clin Exp Pediatr. 1998;41(9):1243-1249.   Published online September 15, 1998
Purpose : This study was conducted to establish the prevalence and clinical features of WPW- type ventricular preexcitation syndrome in children. Methods : Eighteen patients were discovered by the mass heart disease screening using ECG on 47,691 elementary school children in Kwangju from September 1992 to December 1994(group 1), and 14 patients were diagnosed at the Department of Pediatrics, Chonnam...
Prognostic Factors for Mortality in Neonates with Respiratory Distress Syndrome After Surfactant Replacement Therapy
Nam Soo Kang, Byung Min Choi, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
Clin Exp Pediatr. 1998;41(9):1188-1197.   Published online September 15, 1998
Purpose : To evaluate the association between neonatal mortality and the initial response to surfactant replacement therapy, and to examine the correlation between the response type and other prognostic factors. Methods : Sixty-seven neonates with respiratory distress syndrome(RDS) were divided into two groups; group I(n=51) who survived and group II(n=16) who died within the first 28 days of life after one...
Clinical Characteristics, Including Endocrinological Changes, of Optic Glioma in Children
Jee Suk Yu, Se Young Kim, Choong Ho Shin, Ki Joong Kim, Sei Won Yang, Yong Seung Hwang
Clin Exp Pediatr. 1998;41(8):1111-1119.   Published online August 15, 1998
Erratum
Perinatal Factors Influencing Clinical Response to Surfactant Replacement Therapy in Neonates with Respiratory Distress Syndrome
June Dong Park, Beyong Il Kim, Jung-Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1998;41(8):1023-1032.   Published online August 15, 1998
Purpose : Surfactant replacement therapy significantly decreases neonatal complications and mortality in neonates with respiratory distress syndrome(RDS), but clinical responses to the treatment is not consistent. An analysis of the perinatal factors influencing the clinical response to the therapy is important for early detection of high risk and prognosis. The purpose of this study is to analyze the clinical...
Case Report
A Case of Fetal Alcohol Syndrome
Eun Jung Kim, Sun Mi Yang, Jun Yun, Hea Kyoung Lee, Young Hee Yu, Hyun Sook Lee
Clin Exp Pediatr. 1998;41(7):1001-1005.   Published online July 15, 1998
Fetal alcohol syndrome is characterized by distinctive facial appearance, prenatal onset growth deficiency, an increased frequency of development and mental retardation, cardiac anomaly and genitourinary anomaly. Complete abstinence during pregnancy is recommended, since alcohol consumption in each trimester has been associated with abnormalities. We experienced a case of fetal alcohol syndrome in a 11-month-old female infant. Her mother had drunk...
A Case of Superior Mesenteric Artery Aneurysm associated with Marfan Syndrome
Ick Ho Sung, Sang Hee Kim, Min Seop Song, Chul Ho Kim
Clin Exp Pediatr. 1998;41(7):984-988.   Published online July 15, 1998
Superior mesenteric artery aneurysm is very rare complication of Marfan syndrome, especially in children. A 11 years old male patient was admitted to the hospital because of fluctuating high fever and diagnosed as infective endocarditis and Marfan syndrome. During antibiotics therapy, fever was slowly decreased but abdominal pain was developed and pulsatile abdominal mass was palpable in the midline of...
Original Article
Molecular Genetic Diagnosis in Korean Patients with Myoclonic Epilepsy with Ragged Red Fiber(MERRF) Syndrome
Tae-Sung Ko, Sang-Ahm Lee, Gheeyoung Choe, Han-Wook Yoo
Clin Exp Pediatr. 1998;41(7):941-952.   Published online July 15, 1998
Purpose : Myoclonic epilepsy with ragged red fiber(MERRF) syndrome is a disease of the mitochondrial encephalomyopathies, characterized by progressive myoclonus(action), epilepsy, cerebellar ataxia, intention tremor, muscle weakness, progressive dementia, sensorineural hearing loss and optic atrophy. Its inheritance is maternally inherited mitochondrial mutation, and its pathologic finding is characterized by ragged red fibers(RRF). Biochemically its defects are diverse. This study was...
Developmental Morphology of the Atrial Venous Component in the iv/iv Mouse
Young-Hwue Kim, Yong-Soo Yun, Jeong-Wook Seo
Clin Exp Pediatr. 1998;41(7):931-940.   Published online July 15, 1998
Purpose : Developmental processes of atria in embryos or fetuses of the iv/iv mouse were studied to find the differences between right and left atrial chambers and to identify the characteristics of atrial isomerism in the abnormal laterality syndrome. Methods : Seventy-three embryos from SI/Col iv/iv mice(Jackson's laboratory, USA) or fetuses at the 10th-13th day were used. They were examined using...
Case Report
Meckel-Gruber syndrome
Jung Seo Park, Ji Yoen Lee, Sun Chan Bae, Moon Sung Park, Jung Seon Kim, Si Houn Hahn, Chang Ho Hong
Clin Exp Pediatr. 1998;41(6):856-860.   Published online June 15, 1998
Meckel-Gruber syndrome is a multiple malformation syndrome featuring occipital meningoencephalocele, multicystic dysplasia of kidney, cystic and fibrotic change of liver, polydactyly, and other characteristics inherited by the autosomal recessive trait. We exprienced a case of Meckel-Gruber syndrome in a newborn male diagnosed clinically and confirmed pathologically. Abnormalities of the fetus were found prenataly by ultrasonogram, and subsequently the baby was...