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Case Report
A Case of Toxic Epidermal Necrolysis in Early Infancy
Min Seong Kim, Jeong Nyun Kim, Bang Soon Kim, Hong Joo Kim, Myung Jae Chey, Churl Young Chung
Clin Exp Pediatr. 1999;42(11):1594-1598.   Published online November 15, 1999
Toxic epidermal necrolysis(TEN) is a bullous disorder affecting mainly basal layers of epidermis by hypersensitive reaction. It is rarely reported in infants under six months of age. It can be developed by drug, infection, and vaccination, which makes it difficult to differentiate from staphylococcal scalded skin syndrome(SSSS) especially in early infancy. We report a case of TEN in a 6-week-old...
A Case of Parry-Romberg Syndrome in Neonate
Chun Hyuk Chang, Jin Hwa Jung, Sung Min Cho,, Dae Seop Choi
Clin Exp Pediatr. 1999;42(11):1589-1593.   Published online November 15, 1999
Parry-Romberg syndrome(Progressive hemifacial atrophy), described in the last century by Parry(1825) and Romberg(1846), is a very rare disorder characterized by a slowly progressive and self-limited unilateral(rarely bilateral) atrophy of the faces affecting variably the skin, subcutaneous fat tissues, musculature, connective tissue, cartilage and bones. And this disorder is usually accompanied by contralateral Jacksonian epilepsy, trigerminal neuralgia, and changes in the...
A Case of Infantile Marfan Syndrome
Sun Young Kim, Hee Ju Park
Clin Exp Pediatr. 1999;42(11):1583-1588.   Published online November 15, 1999
Marfan syndrome is infrequently diagnosed early in infancy. The morphologic characteristics and prognosis in infantile Marfan syndrome may be quite different from those in older patients. Characteristic cardiac findings in early life include mitral valve prolapse, valvular regurgitation, and aortic root dilation. Morbidity and mortality may be high for infants diagnosed with Marfan syndrome. Cardiac surgery for cardiovascular complication in...
A Case of Marden-Walker Syndrome
Hyun Jong Cho, Yoon Kyung Lee, Dong Un Kim, Byung Joon Choi, Jin Tack Kim, Ik Jun Lee, Je Geun Chi
Clin Exp Pediatr. 1999;42(10):1471-1474.   Published online October 15, 1999
Original Article
Dysmenorrhea and Premenstrual Syndrome of School Girls in Ansan
Yong Kyu Sohn, Yo Ahn Rho, Young Kyoo Shin, Baik Lin Eun, Sang Hee Park, Young Chang Tockgo
Clin Exp Pediatr. 1999;42(10):1434-1440.   Published online October 15, 1999
Purpose : Menstrual disorders are very common problems in adolescence. Most of them are minor, including mild dysmenorrhea, premenstrual syndrome(PMS) and minor variations in cycle, length, or amount of flow. Dysmenorrhea is the most prevalent among them while PMS is a nebulous condition characterized by a broad spectrum of symptoms. The purpose of this study is to investigate the correlation...
Correlation between Changes of Intrapulmonary Right to Left Shunt and Improvements in Oxygenation after Exogenous Surfactant Administration in Preterm Respiratory Distress Syndrome
Sei Woo Chung
Clin Exp Pediatr. 1999;42(10):1373-1380.   Published online October 15, 1999
Purpose : This study was aimed to investigate the relationship between changes in intrapulmonary right to left shunt(QSP/QT) and improvements in oxygenation during the 2-hr study period after Surfactant-TA(S-TA) administration. Methods : Umbilical arterial blood gases and corresponding respiratory indexes of 27 preterm neonates with respiratory distress syndrome were studied. The neonates were separated into group A(n=20) and group B(n=7) according...
Case Report
A Case of Guillain-Barré Syndrome Coinciding with Bronchial Asthma associated with Mycoplasma Pneumonia
Kyung-Yil Lee, Dong-Joon Lee, Ji-Whan Han, Sang-Won Ch, John-Sung Lee, Kyung-Tai Whang
Clin Exp Pediatr. 1999;42(8):1165-1169.   Published online August 15, 1999
Mycoplasma pneumoniae is the most common etiologic agent of pneumonia in school-aged children and young adults. It involves not only the respiratory system but includes extrapulmonary complications such as exanthem, hemolysis, arthritis, hepatic dysfuction, cardiac disease, and central nervous system disease. The pathogenesis of extrapulmonary involvements may be an autoimmune phenomena. Recent studies suggest that bronchial asthma can be initiated...
A Case of Catel Manzke Syndrome
Sang Hyun Oh, Eun Young Kim, Young Bong Park, Sang Kee Park, Jeong Ryoul Yang
Clin Exp Pediatr. 1999;42(8):1154-1158.   Published online August 15, 1999
Catel Manzke syndrome is characterized by hyperphalangy of the index fingers associated with Robin malformation sequence. The etiology of Catel Manzke syndrome is still uncertain, with the majority of cases being sporadic. Careful observation to recognize upper airway obstruction secondary to the Robin sequence should be made a part of routine care for newborns with this disorder. Failure to thrive...
A Case of Wolf-Hirschhorn Syndrome Resulting from Familial Translocation
So Yeon Yoon, Jae Kyun Hur, Dae Chul Jeong, Won Bae Lee, Chang Kyu Oh
Clin Exp Pediatr. 1999;42(8):1149-1153.   Published online August 15, 1999
Wolf-Hirschhorn syndrome is caused by a partial loss of the distal short arm of chromosome 4. Characteristic clinical features are severe growth retardation, mental retardation, seizures, congenital cardiac defects, urogenital abnormalities, microcephaly, hypertelorism, prominent glabella, cleft lip and palate and micrognathia. In 87% of cases, chromosome 4 deletion arises as a de novo event, whereas in the remaining cases it...
A Childhood Case of Nephrotic Syndrome Complicated with Cerebral Venous Sinus Thrombosis Followed by Left Popliteal Artery Thrombosis
Si-Eun Lee, Sue-Jin Lee, Yang-Suk Jung, Jae-Young Lim, Chan-Hoo Park, Hyang-Ok Woo, Jin-Jong Yoo, Hee-Shang Youn
Clin Exp Pediatr. 1999;42(7):1019-1025.   Published online July 15, 1999
We report a 10-year-old male with steroid-responsive nephrotic syndrome which was complicated by cerebral venous sinus thrombosis including superior sagittal sinus and left popliteal artery thrombosis following 5 months later. At each thrombotic episode, he suffered from relapse of massive proteinuria with hypovolemic crisis, and from hemoconcentration with dehydration due to vomiting or diarrhea. Brain MR angiography confirmed thromboses of...
Original Article
Role of Polymorphism in HLA DQ-α and -β Chain Loci in the Pathophysiology of Autoimmune Thyroid Disease in Children with and without Turner Syndrome
Kye Shik Shim, Kyu Chul Choeh, Sei Won Yang, Sa Jun Chung, Jin Sung Lee, Kyung Tae Lee, Sung Ho Goh, Yong Sung Kim
Clin Exp Pediatr. 1999;42(7):980-990.   Published online July 15, 1999
Purpose : About 10% of girls with Turner syndrome may have autoimmune thyroid disease(AIT), but the disease's pathophysiology has not yet been elucidated. Accordingly, this study was performed to observe whether the pathogenesis of AIT in children with Turner syndrome and without Turner syndrome correlate with special loci of DQ α and β chain in HLA. Methods : Blood samples were...
Changes in GFAP Immunoreactivity of Astrocytes in Rats with Reye's Syndrome Induced by Valproic Acid and the Effects of Carnitine Supplementation
Young-Yoo Kim, Kyung-Tai Whang
Clin Exp Pediatr. 1999;42(7):966-979.   Published online July 15, 1999
Purpose : This study was designed to investigate whether valproic acid(VPA) induces Reye's syndrome by analyzing blood chemistry, carnitine(CARN) concentration, and the morphologic changes of hepatocytes. The changes of GFAP immunoreactivity of astrocytes were examined in rats with Reye's syndrome induced by VPA. And the effects of CARN supplementation on Reye's syndrome were also investigated. Methods : Ten rats were assigned...
Antenatal Thyrotropin-Releasing Hormone to Prevent Respiratory Distress Syndrome in Preterm
Ai-Rhan Kim, Sung-Jong Park, Hye-Sun Yoon, Ki-Soo Kim, Hye-Sung Won, In-Sik Lee, Ahm Kim, Soo-Young Pi
Clin Exp Pediatr. 1999;42(7):911-920.   Published online July 15, 1999
Purpose : The purpose of this study was to assess the efficacy of antenatal thyrotropin-releasing hormone(TRH) given to mothers at risk for preterm delivery and assess various neonatal outcomes in infants born to these mothers. Methods : Sixty-one mothers(TRH＋Dexamethasone(D):30, D:31) with preterm labor at 26-34 weeks of gestational age were randomized into a study group which received 400μg of TRH at...
Case Report
A Case of Nonsyndromic Paucity of Interlobular Bile Ducts in Down Syndrome
Chun Hyuk Chang, Jun Ho Kim, Sun Ju Le, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi, Woo Taek Kim, Tae Jung Jang
Clin Exp Pediatr. 1999;42(6):858-862.   Published online June 15, 1999
The nonsyndromic paucity of interlobular bile ducts, which belongs to intrahepatic biliary atresia, is characterized by conjugated hyperbilirubinemia, suggesting cholestasis in newborn infants it has little relationship with extrahepatic congenital abnormalities. Pathologic findings through percutaneous liver biopsy show portal changes(duct paucity and fibrosis) and lobular changes(cholestasis, giant cell transformation, extramedullary hematopoiesis and perisinusoidal fibrosis). The overall incidence of intrahepatic biliary...
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
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