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Original Article
Developmental mRNA Expression of Cellular Retinoic Acid Binding Protein I and Ⅱ in Rat
Young Yoo, Hyung Suk Kim, Chang Sung Son, Young Chang Tockgo, Young Hyuk Jeon
Clin Exp Pediatr. 1998;41(2):154-162.   Published online February 15, 1998
Purpose : Retinoic acid(RA) is well known as a potent teratogenic agent in both deficiency and excess. Cellular retinoic acid binding proteins(CRABPs) are involved in RA. We carried this study in order to determine the possible relations of CRABPs with RA-induced teratogenesis through observation of the expression patterns of CRABP Ⅰand Ⅱ in developing rats. Methods : 35S-labeled RNA probes were...
Case Report
A Case of Neuronal Heterotopia
Jeong Hae Joo, Eun Joo Seok, Min Jeong Kim, Son Sang Seo
Clin Exp Pediatr. 1997;40(8):1173-1177.   Published online August 15, 1997
Neuronal migrational disorders of the brain represent abnormalities in the formation of the neocortex caused by faulty migration of the subependymal neuroblasts. The neuroblasts normally migrate between the sixth and 15th gestational week and in doing so form the six-layered neocortex. When the migration does not occur in a normal fashion the resultant brain anomalies include lissencephaly, pachygyria, schizencephaly, hemimegalencephaly, heterotopia, and polymicrogyria. Neuronal heterotopia is...
Original Article
Clinical Study of Symptoms and Various Anomalies of Patients with Joubert Syndrome
Hang Bo Jeong, Se Hee Hwang, Ki Joong Kim, Yong Seung Hwang, Seung Cheol Kim, In-One Kim
Clin Exp Pediatr. 1997;40(3):385-392.   Published online March 15, 1997
Purpose : Joubert syndrome is known tobean autosomal recessive disorder characterized by cerebellar vermian dysgenesis with many symptoms and variety of other malformations. We studied the relevant symptoms and various malformations of seven patients diagnosed as Joubert syndrome at our institution. Methods : Seven children with cerebellarvermian dysgenesis consistent with Joubert syndrome were included in ourstudy. Each child was diagnosed at the Seoul National University Children's...
Case Report
An Autopsy Case of Tracheal Agenesis Type 2
Hyun Joo Lee, Eun Ae Park, Seung Joo Lee, Mi Jeong Kim, Soon Hee Seong
Clin Exp Pediatr. 1996;39(12):1759-1762.   Published online December 15, 1996
Tracheal agenesis is the rare and uniformly lethal anomaly that presents with severe respiratory distress and aphonia after birth. In this anomaly, the trachea is usually absent and air is reaching the bronchi through a communication with the esophagus. The diagnosis should be suspected in a nowborn infant with respiratory distress whose intubation is difficult. We report an autopy case of tracheal...
Original Article
Bacteriological Survey of beta-Hemolytic Streptococci from the Throats of Elementary School Children in Chinju -Compared with the Results of Children in Kangwon, Chungnam and Seoul-
Seon-Ju Kim, Kook-Young Maeng, Hyang-Im Lee, Yun-Kyong Cho, Hee-Sang Yun
Clin Exp Pediatr. 1996;39(2):238-245.   Published online February 15, 1996
Purpose : Throat culture for streptococcal pharyngitis is a gold standard to diagnose, but it may be misleading to interpret due to the carriers. The isolation rates of beta-hemolytic streptococci (BHS) and Streptococcus pyogenes in the school children in Chinju were investigated and compared with those of Kangwon, Chungnam and Seoul previously reported. Methods : Throat cultures were taken from the...
Case Report
A Case of Agenesis of the Right Lung Accompanied by Cleft Lip
Dong Joon Lee, Moon Soo Han, Won Bae Lee, Joon Sung Lee
Clin Exp Pediatr. 1995;38(2):252-256.   Published online February 15, 1995
This paper describes a case of agenesis of the right lung in a male neonate, who suffered respiratory difficulty. He was evaluated by plain X-ray, bronchography, lung perfusion scan, chest CT, and brain CT. After he died, autopsy was performed. The results revealed multiple anomalies which included cleft lip and palate, absence of left thumb and radius bone, hemivertebra between L1...
A Case of Severe Pituitary Dwarfism due to Agenesis of Anterior Pituitary Gland with Pituitary Stalk Transection
Myoung Ju Yoo, Dong Ki Han, Jeh Hoon Shin, In Jun Seul, Seung Ro Lee
Clin Exp Pediatr. 1994;37(9):1305-1311.   Published online September 15, 1994
We experienced one case of severe pituitary dwarfism in a 10 years old female girl. Magnetic resonance image(MRI) revealed transection of the pituitary stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland. The serum Growth Hormone(GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient...
Original Article
Rapid Detection Test for Streptococcus pyogenes in Normal Carriers of Group A Beta-Hemolytic Streptococci
Sung Ho Cha, Seon Ju Kim
Clin Exp Pediatr. 1994;37(6):812-815.   Published online June 15, 1994
Streptococcus pyogenes has been regarded very importantly because of its severe sequelae, namely rheumatic fever and acute glomerulonephropathy. Rapid tests to identify this organism have been studied widely in developed countries in 1980s. We proceeded the rapid test(A Strept ADTM, Denka Seiken Co., Japan) of S. pyogenes on 141 children who were known to be carriers of this organism to...
Case Report
Three Cases of Listeria Infection in the Immunocompromised Children
Mee Ran Kim, Bo Young Yun, Hye Young Kang, Sung Hee Jang, Hoan Jong Lee, Yong Seung Hwang, Eui Chong Kim
Clin Exp Pediatr. 1992;35(10):1419-1426.   Published online October 15, 1992
Infection by Listeria monocytogenes, an uncommon pathogen in immunocompromised host, has been reported in neonates, pregnant women and adults with defective cell mediated immunity but not in children in Korea. It can cause sepsis, meningitis, abortion, premature labor, intrauterine fetal infection. etc. We have experienced L. monocytogenes sepsis and/or meningitis in three children under immunosuppresive therapy for lymphoma-leukemia, cytophagic histiocytic...
A Case of Unilateral Absence of Pulmonary Artery
Joon Ho Bang, Sang Nyen Kim, Jong In Byun, Won Bae Lee, Byung Churl Lee, Kyong Su Lee, Sung Hoon Cho
Clin Exp Pediatr. 1992;35(6):873-878.   Published online June 15, 1992
A Unilateral Absence of Pulmonary Artery(UAPA), which develops occasionally as one of the associated anomaly in congenital cardiovascular defect like as tetralogy of Fallot, is rare anomaly as an isolated congenital defect. We have recently experienced a case of UAPA in a 9 years old boy, which was diagnosed by perfusion scan, digital subtraction angiography. So we report this case...
A Case of Agenesis of the Right Lung with H-type Tracheoesophageal Fistula
Yong Seok Choi, Kyung Hee Kim, Young Chul Ahn, Baik Lin Eun, Kwang Je Beck
Clin Exp Pediatr. 1992;35(6):816-821.   Published online June 15, 1992
Agenesis of the lung is a developmental defect with complete absence or profound hypoplasia of one or both lung : absence of one lung is more common. Absence of both lung is very rare and is incompatible with life. Unilateral or bilateral agenesis of the lung often associated with the skeletal, cardiovascular, gastrointestinal and genitourinary anomalies. H-type tracheoesophageal fistula in...
A Case of Cloacal Deformity
Young Mi Jee, Seung Jin Lee, In Kyung Sung, Byung Churl Lee
Clin Exp Pediatr. 1992;35(5):667-670.   Published online May 15, 1992
We experienced a case of cloacal deformity. This one day-old female neonate had hydrometrocolpos with vaginal atresia, imperforated anus, cloaca associated with hemivertebrae, left renal agenesis and right hydronephrosis. A brief review of related literature was made.
A Case of Right Lung Agenesis
Seung Hyun Seo, Yu Sub Shin, Ki Sik Min, Jong Wan Kim, Kwang Nam Kim, Ki Yang Ryoo
Clin Exp Pediatr. 1992;35(3):428-433.   Published online March 15, 1992
Lung agenesis is the very rare disease which is defined as total absence of the pulmonary parenchyma, its vascular supporting structure, and bronchi beyond the carina. In many cases, there are coexisting anomalies involving the skeletal, cardiovascular, gastrointestinal, and genitourinary systems. Also, severe respiratory infections are common in infancy and may lead to pneumonia and death. When associated anomalies are...
A Case of Agenesis of the Right Lung
Hyun Soon Lee, Gyeong Cheol Yoon, Se Wook Oh, Ho Seek Ahn, Sung Won Kim, Chung Hee Chi
Clin Exp Pediatr. 1992;35(2):240-244.   Published online February 15, 1992
Unilateral lung agenesis is defined with absence of parenchyme, supporting structures and airway below the level of the carina. Lung agenesis is associated with other anomaly of cardiovascular system, musculoskeletal system and genitourinary system in about 50% cases. In most cases, the diseases is found due to respiratoru problems. Prognosis is variable but largely depends on the associated anomaly and...
Agenesis of Corpus Callosum-Two Cases Report
Soo Young Kweon, Jeoung Wean Seo, Gyung Hee Kim, Eun Chul Chung, Hea Soo Koo
Clin Exp Pediatr. 1992;35(1):113-121.   Published online January 15, 1992
Agenesis of corpus callosum is a relatively comon malformation that may occur in isolation or in combination with other cerebral malformations. A male newborn who presented generalized convulsion showed agenesis of the corpus callosum and septum pellusidum, gyral malformation, and diffuse leukomalacia on postmortem examinations. Also a female newborn with a cystic lesion in brain by antenatal neurosonography showed partial...
Original Article
A case of agenesis of corpus callosum.
Su Min Kim, Chang Hee Bae, In Hee Park, Chin Sam Ro, Yun Jung Kim, Hyo Jin Lee
Clin Exp Pediatr. 1991;34(10):1433-1438.   Published online October 31, 1991
Agenesis of the corpus callosum is a form of dorsal midline dysgenesis Malformation of the corpus callosum may vary from partial absence of the corpus callosum and surrounding structure. We experienced a case of corpus callosum agenesis in a one day old neonate who showed facial anom- alies. Brain CT demonstrated a typical picture of corpus callosum agenesis and then we confirmed by autopsy. A brief...
Two cases of ectopic sublingual thyroid with hypothyroidism to be appeared in fetal life.
Dong Sik Kim, Mee Kyung Namgoong, Hae Yong Lee, Hwang Min Kim, Baek Keun Lim
Clin Exp Pediatr. 1991;34(3):426-434.   Published online March 31, 1991
Infants & children with hypothyrodism usually manifest variable skeletal abnormality. These features are the delay in longitudinal bone growth, the delay in epiphyseal maturation (=delay in bone age), the disturbances in bone mineralization and charateristic multiple stippled epiphysis (cretenoid epiphyseal dysgenesis). But many pediatrician have only concerned about the delay in epiphyseal maturation. Epiphyseal dysgenesis can be used a marker to find out the begining...
A Case of Right Lung Agenesis Associated with Right Hemifacial Atrophy.
Jung Tak Kim, Young Mi Chung, Dong Goo Lee, Byeong Ju Jeoung, Kyu Earn Kim, Ki Young Lee, Jin Suk Suh
Clin Exp Pediatr. 1990;33(12):1723-1728.   Published online December 31, 1990
Unilateral lung agenesis is a rare disease with associated anomalies of soft tissue, the musculos- keletal system, cardiovascular system and gastrointestinal system in 50-60% of cases. When associat- ed anomalies are absent, the disease may not be discovered during a lifetime, but in most of the cases, frequent respiratory infection or associated anomalies make it possible for diagnosis of the disease. Since early diagnosis...
A Case of Corpus Callosun Agenesis with Situs Ambiguus.
Kiyoung Cheong, Jaehong Yoo, Jongjin Seo, Keonsu Rhee, Younghun Chung
Clin Exp Pediatr. 1990;33(6):854-859.   Published online June 30, 1990
In agenesis of the corpus callosum, the major nerve fiber tracts that connect the two carebral hemisphere are absent, and various associated anomalies involve almost all organs. We experienced a case of corpus callosum agenesis associated with situs ambiguus that has not been reported until now. A brief review of related literatures was done.
A Case of Mixed Gonadal Dysgenesis.
Byung Ran Yun, Jae Il Sohn, Sei Won Yang, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1989;32(12):1757-1762.   Published online December 31, 1989
Mixed gonadal dysenesis is a disorder of sexual differentiation which is characterized by streak gonad associated with contralateral tesis, persistent Mullerian duct structures and/or sex chromosomal mosaicism most commonly with 45, X/46, XY. The authors experienced a case of mixed gonadal dysgensis in a 6-year old child who was reared as female and admitted due to clitoral enlargement. The patient had asymmetrical gonads consisted with...
A Case of Mixed Gonadal Dysgenesis.
Eun Young Choi, Min Sik Kim, Hey Sun Lee, Young Min Ahn, Kyung Joon Min
Clin Exp Pediatr. 1989;32(7):1023-1029.   Published online July 31, 1989
The syndrome of Mixed Gonadal Dysgenesis is characterized by a unilateral testis, usually intra- abdominal a streak gonad on contralateral side, persistent Mullerian structures and ambiguous genitalia. These patients are chromatin negative and exhibit XO/XY mosaicism, probably resulting from a cytogenetic error very early in embryogenesis. The testis and the streak gonad should be removed because of the potential devlopement of a gonadoblastoma and the virilization...
Clinical Study of Partial Agenesis of Callosum.
Seung Hwan Oh, Chang Jun Coe, Jung Ho Suh
Clin Exp Pediatr. 1989;32(4):511-517.   Published online April 30, 1989
26 cases of partial agenesis of the corpus callosum diagnosed at Pediatric Department of Yonsei Medical Center for 8 years from Jan. 1980 to Dec. 1987 and clinical information has been analized and we got following results. 1) The sex ratio of male to female was 1:1.9. 2) Among the 26 cases, 23 cases (88.5%) were diagnosed under 6 years old. 3) Clinical findings were...
Two Cases of Osteogenesis Imperfecta Congenita.
Soon Soen Lim, Byeung Ho Lee, Young Ha Kim, Moon Ki Joe
Clin Exp Pediatr. 1988;31(8):1085-1090.   Published online August 31, 1988
Osteogenesis imperfecta is an inherited disorder of connective tissue that affects the skeleton, ligament, skin, sclerae and dentin. It is thought to be characterized by the abnormal maturation of collagen in both mineralized and nonmineralized tissues. The three major clinical criteria are osteoporosis with abnormal fragility of the skeleton, blue sclerae and dentinogenesis imperfecta. The presence of two of these abnormalities confirms the diagnosis. We have...
A Case of Potter Syndrome Syndrome Accompanied with Partial Agenesis of Corpus Callosum.
Soon Sup Jang, Byung Chun Suh, Kyoo Hwan Rhee, Soo Jee Moon, Keun Soo Lee, Byung Tae Park, Eun Kyung Hong
Clin Exp Pediatr. 1987;30(11):1287-1293.   Published online November 30, 1987
We have experienced a case of Potter syndrome recently. He was a newborn infant who suffered from severe respiratory distress soon after birth. His face was characterized by facies renalies and chest X-ray film revealed bilateral pneumothorax. Anuria since birth and findings of abdominal ultrasonography strongly suggested bilateral renal agenesis. Severe respiratory acidosis was progressed in spite of proper management and died at 11...
Case Report
A Case of Unilateral Renal Agenesis Associated with Acute Renal Failure.
M J Kim, S Y Chung, H S Kim, S H Kim, C M Kang
Clin Exp Pediatr. 1986;29(11):1245-1250.   Published online November 30, 1986
The authors experienced a case of unilateral renal agenesis associated with hydronephrosis resulted from U.P.J. obstruction of contralateral kidney. The patient was a 7 year old girl who was hospitalized with sudden onset of renal failure and the diagnosis was made by intravenous pyelography, abdominal ultrasonogram and C-T scan. Patient was almost completely recovered with peritoneal dialysis and surgical operation. A brief review...
A case of Congenital Osteogenesis Imperfecta.
Suck Young Lee, Yoon Ho Kang, Sang Keun Oh, Mi Sook Park, Hee Dae Park, Dong Hwan Cha
Clin Exp Pediatr. 1986;29(5):575-578.   Published online May 31, 1986
The authors experienced a case of osteogenesis imperfecta in a 1 st day of life male patient. Characteristic findings of this disease are spontaneous fractures of bones, hypermobility of joints, otosclerosis, deafness and blue sclera. Diagnosis was confirmed by clinical symptoms and radiologic findings. Related literature was briefly reviewed.
A Case of Osteogenesis Imperfecta.
Kwang Wook An, Chull Zoo Jung, Hyo Seop Joo, Seung Woo Moon
Clin Exp Pediatr. 1986;29(1):84-87.   Published online January 31, 1986
Osteogenesis imperfecta is an inherited disorders of connective tissue that affects the skeleton, ligament, sclera, and dentin. Characterized clinical manifestation are multiplefracture, blue sclera, deafness, joint laxity, thin skin, and odontogenesis imperfecta. We experienced acase of osteogenesis imperfecta in a one day old male newborn who chiefly complain respiratory distress, triangular shaped head and fracture and deformity of lower extremity....
A Case of Sacral Agenesis.
Cheol Lee, Byung Uk Park, Tae Gyu Hwang, Soon Yong Lee
Clin Exp Pediatr. 1986;29(1):79-83.   Published online January 31, 1986
Sacral agenesis is a rare congenital anomaly of the lower vertebral column which usually produces lower urinary tract dysfunction. The authors have reported a case of sacral agenesis which was found in a l-year-4-month old girl, who was born of a diabetic mother and had been suffered from recurrent urinary tract infection. A brief review of literatures was made.
A Case of Agenesis of the Corpus Callosum.
Jung Hee Lee, Seong Ryong Hyun, In Joon Seol, Ha Baik Lee, Keun Soo Lee
Clin Exp Pediatr. 1985;28(8):836-840.   Published online August 31, 1985
Agenesis of the corpus callosum is a relatively common malformation that may occur in isolation or in combination with other cerebral malformation. We experienced a case of agenesis of the corpus callosum in a 5/12 year old boy who showed developmental retardation. Cerebral brian CT and sonography demonstrated a typical picture of agenesis of the corpus callosum without any further...
A Case of Unilateral Lung Agenesis (Right) Associated with Skeletal Anomalies.
Woan Chul Suh, Dong Youl Lee, Kyung Ae Lee, Hee Ju Kim, Sung Ill Ahn, Bom Woo Yeom
Clin Exp Pediatr. 1985;28(8):795-800.   Published online August 31, 1985
Lung agenesis is defined as total absence of pulmonary parenchyma with its supporting vasculature, as well as the main stem bronchi on one or both sides. This anomaly is usually associated with extrapulmonary anomalies such as cardiac, vascular, gastrointestinal, skeletal, genitourinary, and miscellaneous anomalies. We experienced a case of unilateral lung agenesis(Right) in a three days old female premature baby, who...
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