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Case Report
A Case of Cerebrocostomandibular Syndrome with Congenital Heart Disease
Sang Heui Song, Kook In Park, Hye Jung Joo, Ran Namgung, Chul Lee, Dong Gwan Han
Clin Exp Pediatr. 1993;36(4):574-578.   Published online April 15, 1993
Cerebrocostomandibular Syndrome is characterized by micrognathia, cleft palate, multiple thorax deformity and frequently, mental deficiency. Respiratory compromise is a common cause of death. We experienced a case of cerebrocostomandibular syndrome with congeniyal heart disease in a 2 day old female baby with the chief complaint of cyanosis and respiratory difficulty since birth, She was delivered by cesarian section due to delayed...
A Case of Congenital Neuroblastoma: Diagnosed at Antenatal Perio
Hye Young Kang, Chuhl Joo Lyu, Byung Soo Kim, Seung Kang Choi, Woo Hee Jung, Soon Ae Chun
Clin Exp Pediatr. 1992;35(12):1750-1755.   Published online December 15, 1992
Neuroblastoma is one of the most common tumors of childhood. It accounts for about 8% of all pediatric cancers. Because most children who present with this tumor are older than 1 year of age and have advanced disease, they have a poor prognosis in spite of intensive treatment. The patients with less than 1 year of age at the time of...
A Case of Congenital Pelger-Huet Anomaly
Soo Jung Lee, Young Yoo Kim, Seung Won Kang, Jong Wan Kim, Kyung Tai Whang, Sung Ho Cho
Clin Exp Pediatr. 1992;35(11):1578-1583.   Published online November 15, 1992
Congenital Pelger-Huet anomaly is an autosomal dominant disorder characterized by incomplete nuclear segmentation of neutrophils without functional disturbance. The morphology of these mature but hyposegmented neutrophils may be confused with that of immature neutrophils (bands), thereby suggesting bacterial infection. We experienced a case of congenital Pelger-Huët anomaly on routine hematologic examination in a 7-year old boy who was admitted because of...
A Case of Fukuyama Type Congenital Muscular Dystrophy
Young Eun Lee, Min Hyea Kim, Keun Lee, Eun Chul Chung, Hyea Soo Koo
Clin Exp Pediatr. 1992;35(10):1463-1468.   Published online October 15, 1992
Fukuyama type congenital muscular dystrophy is characterized by mental retardation, progressive muscle weakness and hypotonia, joint contracture and seizures in half of the reported cases. The usual CT abnormalities are dilated cerebral ventricles and Sylvian fissures and lucency of cortical white matter. The most consistent pathologic findings in the brain are polymicrogyria and pachygyria, caused by a disturbance of migration...
Original Article
A Clinical Study on Cardiovascular Disease of Children Taken Cardiac Catheterization and Cineangiography
Gi Yeon Song, Seog Beom Cho, Pyoung Han Hwang, Chan Uhng Joo, Jung Soo Kim
Clin Exp Pediatr. 1992;35(7):949-956.   Published online July 15, 1992
A clinical study was made on 533 patients with cardiovascular disease who were admitted to pediatric department of Chonbuk National University Hospital and performed cardiac catheterization and cineangiography from February 1984 to August 1991. The results were as follows; 1) The ratio of male to female was about 0.81:1; among 533 cases, male 283cases, male 283cases, female 295cases. 2)The age distribution of the...
Case Report
A Case of Congenital Hypopituitarism Presenting Ectopic Pituitary Gland and Pituitary Stalk Transection Identified by Magnetic Resonance Imaging
Do Seong Kim, Kyung Eun Oh, Duk Hi Kim, Myung Joon Kim
Clin Exp Pediatr. 1992;35(6):809-815.   Published online June 15, 1992
The syndrome of congenital hypopitutarism is a rare but well recognized entity. The typical clinical picture consists of presentation in infanncy with hypoglycemia, microgenitalia, and evidence of multiple anterior pituitary hormone deficiencies, but not diabetes inspidus. Recently, magnetic resonance imaging has disclosed a consistent abnormality in many patients with congenital hypopituitarism as well as idiopathic hypopituitarism characterized by an adenohypophysis...
A Case of Congenital Giant Pigmented Nevus
Seung Wan Lee, Wu Ha You, Chang Bin Im, Chang Woo Lee, Chan Kum Park
Clin Exp Pediatr. 1992;35(5):723-728.   Published online May 15, 1992
The congenital giant pigmented nevus, also known as bathing trunk nevus, is a severely disfiguring lesion covering large areas of the body surface. Although the occurrence of giant nevi in childhood is uncommon, it is frequent enough to represent a source of guilt for parents, and psychic trauma, and is advised. For those patients who do not choose to undergo...
Two Cases of Congenital Hepatic Fibrosis
Cue Jung Hwang, Young Hun Kim, Dae Kyun Koh, Byung Churl Lee
Clin Exp Pediatr. 1992;35(4):569-574.   Published online April 15, 1992
The congenital hepatic fibrosis is a rare condition defined pathologically by the presence within the line of bands of fibrous tissue which after contain linear or circular spaces lined by bile duct cells, It is commonly associated with intrahepatic portal hypertension but hepatocellular function is almost always preserved. The prinicipal clinical features of this disease are abdominal distension, firm hepatomegaly,...
A Case of Congenital Dyserythropoietic Anemia
Il Tae Whang, Young Sook Ko, Kyeung Hee Kim, Gyeung In Lee, Han Ik Cho
Clin Exp Pediatr. 1992;35(4):539-544.   Published online April 15, 1992
Congenital dyserythropoietic anemia(CDA) is a rare disease characterized by ineffective erythropoiesis, specific cytopathology of the nucleated red cells in the bone marrow and secondary hemochromatosis. CDA is classified into 4 types on the basis of results of electron microscopic and serologic studies. We experienced a case of congenital dyserythropoietic anemia in a 5/12 year-old boy. Diagnosis was established by macrocytosis, anisopoikilocytosis in PB...
Original Article
Clinical Study on Congenital Anomalies
Young Soo Kwon, Heung Keun Oh, Jin Jae Kim, Chang Ok Soh, Jin Hee Jung, Jin Young Jung
Clin Exp Pediatr. 1992;35(3):315-321.   Published online March 15, 1992
Congenital malformations are known as a major cause of perinatal death. But the causes and Mechanisms of malformations ae still not understood in many cases. So, a clinical stduy was one about congenital malformaions on 8,584 deliveries a Haesung Hospital during the period of 5 and 7/12years from Jan. 1, 1985 to Jul. 31, 1990. The results were as follows: 1) The incidence...
Case Report
A Case of Beals Syndrome
Sung Kee Park, Tae Woo Kim, Young Soo Baik, Chang Yeon Lee, Suk Kang, Moon Jib Yoo, Jae Hwy Lee, Moon Whan Im
Clin Exp Pediatr. 1992;35(2):282-285.   Published online February 15, 1992
Beals syndrome, congenital contractural arachnodactyly (CCA), is an autosomal dominant disorder of connective tissue sharing many of the clinical manifestations of Marfan's syndrome, characterized by congenital joint contractures, crumpled ears, arachnodactyly (dolichostenomelia), kyphoscoliosis, and autosomal dominant inheritance. Differential diagnosis of CCA includes Marfan syndrome, Achard syndrome, homocystinuria, osteogenesis imperfecta, Stickler syndrome, and arthrogryposis multiplex congenita. Especially, the major consideration is Marfan...
Analysis of 5,653 Cases with Congental Heart Disease (CHD) Catheterized at Yonsei Medical Center - A 28-Year Review -
Dong Shik Chin, Jong Kyun Lee, Jun Hee Sul, Sung Kyu Lee, Bum Koo Cho
Clin Exp Pediatr. 1992;35(2):143-156.   Published online February 15, 1992
This is a 28-year analysis of 5,653 cases with congenital heart disease who underwent cardiac catheterization at Yonsei Medical Center from 1964 to 1991. Form 1964, there was a steady increment in the number of catheterized cases with CHD until the late 1970's when the patients population began to increase rapidlly. This was undoubtedly set off by the introduction of the...
Original Article
Congenital cystic diseases of the lung.
Won Soon Park, Young Pyo Chang, Hee Ju Kim, Young Yull Koh, Jung Hwan Choi, Chong Ku Yun, Joo Hyun Kim
Clin Exp Pediatr. 1991;34(12):1671-1677.   Published online December 31, 1991
Congenital cystic diseases of the lung (CCDL) is a clinical group of disorders characterized by a cystic appearance of pulmonary tissue. It can be classified into four distinct categories: 1) Congenital lobar emphysema (CLE), 2) Congenital cystic adenomatoid malformation (CCAM), 3) Pulmonary sequeestration (PS), and ^Bronchogenic cyst (BC). 24 patients with CCDL admitted to the Seoul National University Children’s Hospital from January 1984 to June...
Blade atrial septostomy in infants with cyanotic congenital heart diseases.
In Sook Park, Young Hwue Kim, Chang Yee Hong
Clin Exp Pediatr. 1991;34(12):1655-1663.   Published online December 31, 1991
In patients with certain types of congenital heart malformations, an adequate interatrial communi- cation is essential for survival and balloon atrial septostomy (BAS) is a life saving procedure in these infants. However, BAS alone is not successful in older infants and children with thickened atrial septum, and blade septostomy is indicated in these situations. This procedure so far has not been reported in Korean literature...
The clinical review of congenital magacolon.
Myung Goo Min, Ki Sup Chung
Clin Exp Pediatr. 1991;34(10):1373-1380.   Published online October 31, 1991
This clinical study presents an anlaysis of 102 patients who underwent definitive surgery for Hirschsprung’s disease from January 1979 to April 1990 at Department of Pediatrics, Yonsei Univer- sity College of Medicine. The results were summarized briefly as follows: 1) The sex ratio was 2.6:1, of male preponderence. 2) The first symptom appeared within 1 month after birth in majority of cases, in 82 cases (80.4%) and diagnosis...
Congenital cytomegalovirus infection: incidence and clinical outcome.
Young Mo Sohn, Kook In Park, Chul Lee, Dong Kwan Han, Won Young Lee
Clin Exp Pediatr. 1991;34(10):1365-1372.   Published online October 31, 1991
In order to assess the rate of congenital cytomegalovirus (CMV) infection in newborns, CMV IgG antibody was studies at about 37.5 weeks gestation in 309 mothers and a positive rate of 96.4% was obtained. Virus culture for CMV from urine of 514 neonates less than 1 week after birth resulted in CMV growth in 6 neonates showing a 1.2% congenital infection rate. In...
Balloon atrial septostomy through umbilical vein approach using flexible guide wire and sheath technique.
In Sook Park, Ki Soo Kim, Chang Yee Hong, Shi Joon Yoo
Clin Exp Pediatr. 1991;34(9):1294-1298.   Published online September 30, 1991
Balloon atrial septostomy is accepted as the best initial palliative treatment in infants with certain types of cyanotic congenital heart disease and the importance of prompt creation of atrial communi- cation can not be overemphasized in these patients. Balloon atrial septostomy is performed through femoral vein using percutaneous technique in most cases and is rarely done by cut down approach. However, percutaneous femoral vein...
A case of Rubinstein-Taybi syndrome.
Cheol Hee Hwang, Dong Gue Lee, Myung Sug Nam, Moon Kee Cho
Clin Exp Pediatr. 1991;34(8):1146-1150.   Published online August 31, 1991
Rubinstein-taybi syndrome is characterized by typical skeletal abnormalities such as broad thumb and large first toe, mental and motor retardation, and facial abnormalities. In addition, defects of the cardiovascular system and the urinary tract may be present. We have experienced a case of Rubinstein-Taybi syndrome which a five-year-old boy had broad thumb and large first toe, mental and motor retardation, and facial abnormaiities (palpebral...
Congenital bile duct cyst.
Myung Hee Yoon, Mi Sung Jeong, Hyung Ja Park, Shinna Kim, Doo Sun Lee
Clin Exp Pediatr. 1991;34(5):707-714.   Published online May 31, 1991
A case of congenital bile duct cyst in a 15-year-old female patient was diagnosed by clinical symptoms, laboratory data, ultrasonography, endoscopic retrograde cholangiopancreatography. Excision with Roux-en-Y choledochocystojejunostomy was performed with good result. We report one case of congenital bile duct cyst type IV-A (by Todani classification) with brief review of all 277 cases reported so far in Korean literature and present comparisons with Flanigan’ s...
The value of 2-D echocardiography in diagnosis of CHD.
Sang Kyung Yun, Young Woon Baek, Hyun Ki Jung
Clin Exp Pediatr. 1991;34(5):662-667.   Published online May 31, 1991
Cardiac catheterization, cineangiography and surgical analysis of 220 cases of congenital heart disease diagnosed by 2-D echocardiography was made, retrospectively from mar. 1985 to sep. 1989, who were admitted to the department of pediatrics Kosin Medical College Hospital. The results were analysed as follows; 1) 22 cases of 23 were correctly diagnosed in ASD, sensitivity and specificity of subxiphoid 2-D echocardiogram were 95.7% and 99.6% respectively. 2) 109...
A clinical study of congenital hypertropic pyloric stenosis.
Eun Chin Mun, Hae Jin Lee, Son Sang Seo, Jung Woo Yang
Clin Exp Pediatr. 1991;34(5):637-644.   Published online May 31, 1991
This study was a review and clinical analysis of 77 cases of congenital hypertrophic pyloric stenosis at II Sin Christian Hospital from Jan. 1980. to Mar. 1990. The result was as follows. 1) At age distribution, the most prevalent age group was between 4 weeks and 6 weeks, and ratio of male to female was about 3.8:1. 2) Among 77 cases, the first bom was most affected...
Accuracy of anorectal manometry in the diagnosis of congenital megacolon.
Jong Yul Jeon, Byeong Gie Yeo, Chong Woo Bae, Yong Mook Choi, Chang Il Ahn, Young Woon Chang
Clin Exp Pediatr. 1991;34(5):614-620.   Published online May 31, 1991
Thirty-seven patients with clinical impression of congenital megacolon were studied with anorectal manometry for the evaluation of its diagnostic accuracy. Including 12 neonates, their age ranged form 3 days to 8 years and barium enema was performed to all of them except one. The results of rectosphincteric reflex (RSR) of anorectal manometry was compared with the final diagnosis, which was obtained by clinical course,...
A case of congenital lactic acidosis.
Suk Min Choi, Myeong Ku Cho, Dong Hwan Lee, Sang Jhoo Lee, Kikumaro Aoki, Shuichi Yamaguchi
Clin Exp Pediatr. 1991;34(1):95-100.   Published online January 31, 1991
The congenital lactic acidosis represent a sizable group of metabolic disorders. They are in all likelihood genetically determined, and they are clearly heterogenous. They may be considered broadly as disorders of pyruvate metabolism in which there are two general categories: defects in gluconeogenesis and defects in pyruvate oxidation. We experienced a case of congenital lactic acidosis in neonate who was diagnosed by urinary organic acid...
Clinical Observation on isolated TRH deficient Congenital Hypothyroidism.
Heon Seok Han, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(10):1388-1393.   Published online October 31, 1990
Clinical features and endocrine function of 3 children with isolated TRH deficient congenital hypothyroidism followed at Seoul National University Children’s Hospital from Aug, 1986 to Aug. 1990 were reviewed. During above period 262 congenital hypothyroid patients were followed at endocrine clinic, number of congenital primary hypothyroidism was 218 cases (83.2%), and that of congenital secondary hypothyroidism was 44 cases (16.8%). Of the congenital primary hypothyroidism,...
A Clinical Observation of Congenital Syphilis.
Soo Mi Baek, Eun Chin Moon, Ock Seung Jeong, Son Sang Seo
Clin Exp Pediatr. 1990;33(10):1326-1332.   Published online October 31, 1990
The authors studied 75 cases of congenital syphilis in the newborn treated at II Sin Christian Hospital from January 1983 to December 1987. The results were as follows; 1) The incidence of syphilis among the admitted pregnant women for delivery was 0.62%. 2) The incidence of neonatal syphilis from the 208 syphilis mothers was 36.1%. 3) The incidence of prematurity and low birth weight neonate among congenital syphilitic...
Congenital Cytomegalovirus infection.
Kang Woo park, Ho Seek Ahn, Eui Bon Koo, Sung Won Kim, Kil Hyun Kim
Clin Exp Pediatr. 1990;33(9):1271-1275.   Published online September 30, 1990
We report a case of congenital cytomegalovirus infection that was associated with microcephaly, hydrocephalus and periventricular calcification. The male baby was born after 39wks gestation to 28 year-old mother who had no prenatel problem except for active pulmonary tuberculosis and pre- mature rupture of membrain. Hydrocephalus was recognized by antenatal ultrasonography at 8 weeks of geststional age and microcephaly was noted at birth. This enable...
A Case of Lamellar Ichthyosis.
Yeon Seung Lim, Sang Joo Han, Won Il Park, Kyung Ja Lee
Clin Exp Pediatr. 1990;33(7):1018-1023.   Published online July 31, 1990
We observed a case of lamellar ichthyosis who was 4 day-old boy with the chief complaints of poor feeding, exessive lamellated scale covering entire body. Diagnosis of lamellar ichthyosis was estab- lished by clinical features and histopathological study. A brief review of literature was made.
A Case of Congenital Hypofibrinogenemia.
Ji In Park, In Seok Lim, Chul Ha Kim, Byoung Hoon Yoo
Clin Exp Pediatr. 1990;33(7):1009-1011.   Published online July 31, 1990
Congenital hypofibrinogenemia is a rare congenital bleeding disorder, inherited by autosomal dominant or recessive trait. We experienced one case of congenital hypofibrinogenemia in 4 year-old female patient with chief complaints of headache and ecchymosis. History of continuous umbilical cord bleeding and frequent bleeding tendency against minor trauma were revealed on her past medical history. On laboratory data, bleeding time, platelet aggregation test were normal and...
A Case of Dandy-Walker Syndrome Associated with Multiple Congenital Anomalies.
Gye Weon Shin, Chul Ho Lee, Bo Kyung Cho, Chung Sik Chun, Sung Hun Cho
Clin Exp Pediatr. 1990;33(6):848-853.   Published online June 30, 1990
The Dandy-Walker Syndrome is a developmental disorders of the brain characterized by cystic deformity of fourth ventricle, agenesis or hypoplasia of the cerebellar vermis, and hydrocephalus. This syndrome is frequently associated with central nervous system malformation and systemic anomalies. We experienced a case in a 2-day-old female who has multiple congenital anomalies. On physical examinations, macrocephaly with prominent occiput, encephalocele in the occipital area, microphth- almia,...
Transsphenoidal Meningoencephalocele in Association with Hypopituitarism ans Congenital Dysplastic Optic Disc: A Case Report.
Chang Gee Kang, Jung Wan You, Sung Chul Shin, Myung Goo Min, Duk Hi Kim, Jin Guk Kim, Oh Wong Kwon, Tae Sub Chung
Clin Exp Pediatr. 1990;33(6):842-847.   Published online June 30, 1990
Transsphenoidal meningoencephalocele is congenital defects in the sphenoid bone through which meninges and brain tissue have herniated; the defect, located in the midline of the base of the skull, was first reported by Richter in 1813. The nine cases of transsphenoidal meningoencephalocele have been reported. Patients with transsphenoidal meningoencephalocele also may have other midline craniocerebral and midfacial anomalies, including additional meningoencephalocele hypertelorism, cleft palate, and abnormalities...
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