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Original Article
Clinical Analysis of Children with Nephrotic Syndrome.
Ki Young Cheong, Keon Su Rhee
Clin Exp Pediatr. 1990;33(7):959-969.   Published online July 31, 1990
A clinical analysis was made on 51 cases with nephrotic syndrome who were admitted to Pediatric Department of Chung Nam National University Hospital from January 1st, 1984 to December 31st, 1988. Clinical manifestation, response to the inital continuous steroid therapy, relapsing patterns, factors affecting response to steroid therapy, pathologic findings and difference in laboratory findings between relapser and nonrelapser were evaluated. Results were as follows; 1) Among...
A Case of Poland's Syndrome.
Seok Gyoung Kang, Joo Tae Choi, Wha Mo Lee, Young Seok Jeon
Clin Exp Pediatr. 1990;33(6):860-863.   Published online June 30, 1990
Poland’s syndrome is a congenital disorder associated with absence of the pectoralis muscle and ipsilateral defect of the upper extremity, usually syndactyly. The syndrome is not hereditary and is of unknown origin. It affects males more frequently than females. Recently, we experienced a 1 year old male, who had the absence of right pectoralis muscle and ipsilateral syndactyly with hypoplasia of fingers.
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,...
An Experience of High Dose Intravenous Immunoglobulin Therapy in Refractory Evans Syndrome.
Hee Sup Kim, Won Sup Shin, Sang Il Lee, Sang Woo Kim
Clin Exp Pediatr. 1990;33(5):701-707.   Published online May 31, 1990
Evans syndrome, a combination of autoimmune hemolytic anemia and idiopathic thrombocytopenic purpura, relapses freguently and is refractory to conventional treatment such as corticosteroid, immunosuppressive agents, splenectomy and plasma exchange. Recently Oda et al applied high dose immunoglobulin (400/kg/day for 4 days repeated 2 weeks later) to 5 month-old infant with refractory Evans syndrome, and was successful in maintaining remission for five years and nine...
A Case of Wilson-Mikity Syndrome.
Jung Suk Lee, Young Kyun Lee, Hyang Suk Yoon, Yeon Kyun Oh
Clin Exp Pediatr. 1990;33(5):675-679.   Published online May 31, 1990
Wilson-Mikity syndrome is a chronic lung disease in the premature infant characterized by respira- tory symptoms and became increasingly severe, and was associated with distinctive chest radiologic changes. We experienced a case of Wilson-Mikity syndrome in a premature infant. A brief review of related literature was made.
A Clinical Study on Reye's Syndrome.
Myeong Hee Cha, Seong Hee Shin
Clin Exp Pediatr. 1990;33(5):658-665.   Published online May 31, 1990
The auther analyzed the clinical features and prognosis of the 143 patients with Reye*s syndrome who were admitted at three hospital for 10 years from Jan. 1979 to Dec. 1988. The results were summarized as follows: 1) There was non significant difference in annual and seasonal incidence except slight higher incidence in 1981. 2) The male to female ratio was 1.4:1. The age distribution was from 3...
A case of Kleeblattschadel Syndrome; cloverleaf Skull.
J S kim, C H Koo, M S Eom, J S Park, H G Jung, Y S Kim, H G Lee, C Lee
Clin Exp Pediatr. 1990;33(4):559-563.   Published online April 30, 1990
We have recently experienced a case of Kleeblattschadel Syndrome in a 5 month old boy. The diagnosis was made by characteristic morphologic clinical features and radiologic findings. We report this case with brief review of some related literatures.
Two cases of Klippel-Treaunay-Weber Syndrome.
Chang Suk Seo, Jae In Rho, Young Su Kwon, Man Chul Ha, Jin Young Jung
Clin Exp Pediatr. 1990;33(4):553-558.   Published online April 30, 1990
We experienced two cases of Klippel-Trenaunay-Weber Syndrome in 8-year old girl and 7-month old girl. In two cases, plain X-ray revealed osteohypertrophy of femur and tibia without any periosteal reaction and unilateral soft tissue hypertrophy was also seen on left lower extremity. In case I, femoral and popliteal venography showed no varix. The review of literatures on this syndrome was presented and discussions were attempted...
Two cases of Goldenhar's Syndrome.
Ah Young Yun, Nam Sun Baik, Young Ah Lee, Hyung Ro Moon
Clin Exp Pediatr. 1990;33(3):380-385.   Published online March 31, 1990
Goldenhar’s syndrome is a syndrome consisting of (1) epibulbar dermoids and/or lipodermoids (2) preauricular skin tags and blind-ended fistulas and (3) vertebral anomalies. Two cases of Goldenhar’s syndrome which showed asymmetric facies, epibubar dermoids, preaur- icular skin tags blind-ended fistulas in 7 month-old girl and 2 month-old boy were reported. In one of our cases cleft of vertebrae, hypoplasia of left lung and cyanotic...
A case of Beckwith-Wiedemann Syndrome.
Nam Joo Hwang, Soo Mi Back, Yang Suk Choi, Son Sang Soe, Hye Kyoung Yoon
Clin Exp Pediatr. 1990;33(3):373-379.   Published online March 31, 1990
We experienced on case of Backwith-Wiedemann syndrome. This female neonate manifested omphalocele, gigantism, facial nevus flammeus, Macroglossia, and ear lobe grooves. A brief review of related literature was done.
A Case of Cryptophthalmos Syndrome.
Ho Seek Ahn, Gyu Ha Lee, Eui Bon Koo, Sung Won Kim, Kil Hyen Kim
Clin Exp Pediatr. 1990;33(2):274-277.   Published online February 28, 1990
Cryptophthalmos Syndrome is a rare congenital disease charaterized by unilateral or bilateral absence of the palphbral fissure, other dyscephaly, partial cutaneous syndactyly and malformation of the genitalia. It is presumably a nonuniform syndrome and its etiology is not certain. But, a type transmitted by autosomal recessive inheritance may be considered certain. We experienced a characteristic case of cryptophthalmos syndrome in newborn female infant who died...
A Case of Transient Myeloproliferative Disorder in Down's Syndrome.
Ji Hye Kang, Young Mi Hong, Kyung Hee Kim, Seung Joo Lee, Ki Sook Hong, Ok Kyung Kim, Je Geun Chi
Clin Exp Pediatr. 1990;33(2):252-258.   Published online February 28, 1990
Transient myeloproliferative disorder has been reported in infant with Down's syndrome during neonatal period. This is clinically and hematologically indistinguishable from congenital acute leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. A neonate presented with jaundice, hepatosplenomegaly and mongoloid face was diagnosed as Transient myeloproliferative disorder. We report this case with a review of...
A Case of Cornelia de Lange syndrome.
Byoung Hong Ahn, Song Nyeon Choi, Young Wook Kim, Ki Bok Kim
Clin Exp Pediatr. 1990;33(2):234-240.   Published online February 28, 1990
We have experienced a case of Cornelia de Lange syndrome in a 3-year-old boy. The patient showed most of the typical clinical features of the syndrome: microcephaly, bushy eyebrows and synophys, antimongolian slant, small nose and anteverted nostrils, thin protruding lips with downturning of the comer, micrognathia, low set ears, generalized hirsuitism, and growth redardation. The patient also had skeletal abnormalities of hands. Chromosomal...
2 Case of Scimitar syndrome.
Jae Kon Ko, Nam Su Kim, Woong Heum Kim, Heung Jae Lee, Shi Joon Yoo
Clin Exp Pediatr. 1990;33(2):229-233.   Published online February 28, 1990
The scimitar syndrome, a rare complex anomaly, consists essentially of an anomalous pulmonary vein draining whole or part of right lung into inferior vena cava and its curved Turkish sword (scimitar) like appearance has provided the name of this syndrome. Associated anomalies are frequent, such as hypoplasia of the right lung and right pulmonary artery, abnormalities of bronchial architecture and lobation, rightward displacement of...
A Case of Nonimmunologic Hydrops Fetalis Associated with Down Syndrome.
Sang Eun Lee, Meen Jai Lee, Dong Hwan Lee, Sang Jhoo Lee
Clin Exp Pediatr. 1990;33(2):205-211.   Published online February 28, 1990
We experienced a case of nonimmunologic hydrops fetalis associated with Down syndrome. The patient had generalized edema with severe scrotal swelling and abdominal distension. A roentgenogram showed bilateral pleural effusion and ascites. The chromosomal study revealed 21 trisomy. On autopsy, there were pleural fluid and ascites as well as pericardial fluid. Small ASD and incomplete lobation of the right lung were detected. Chromosomal abnormalities should always...
The Clinical Observation on Mucocutaneous Lymph Node Syndrome.
Sang Yun Ahn, Ja Wook Koo, Ha Baik Lee, Soo Jee Moon, Hahng Lee
Clin Exp Pediatr. 1990;33(2):196-204.   Published online February 28, 1990
The one-hundred-forty nine children with mucocutaneous lymph node syndrome who were diagnosed and treated through admission at the Department of Pediatrics, Hanyang University Hospital from July 1977 to January 1989, were investigated for clinical patterns and laboratory , correlations. The results are as follows: 1) Most patients (84.5%) were under 4 years of the age. 2) Boys were more prevalent than girls, as a ratio of 1.9:1. 3) Seasonal incidence...
A Case of Cornelia de Lange Syndrome.
Hwa Kyoung Oh, Soo Jung Yoo, Myung Jin Kim, Mi Na Lee
Clin Exp Pediatr. 1990;33(1):94-99.   Published online January 31, 1990
We have experienced a case of Cornelia de Lange syndrome in a girl of 4.5/12 years old. She showed typical features of a microbrachycephaly, hirsuitism and characteristic face with bushy eyebrows, antimongolian slant depressed nasal bridge, thin protruding lips with downtuming of the comers and the microtia. She also had severe retardation in growth and mental development. The diagnosis was established by typical clinical...
A case of Guillain-Barré syndrome in a child treated with plasmapheresis.
Hae Won Lee, Hoo Jae Han, Seung Joo Lee, Keun Lee
Clin Exp Pediatr. 1989;32(11):1592-1597.   Published online November 30, 1989
We experienced a case of Guillain -Barré syndrome in a 12-year-old female patient who had progressive motor weakness and showed rapid recovery with plasmapheresis and supportive therapy in acute stage. A brief review of the related literature was added.
A case of Dandy-Walker syndrome.
Kyung Hee Kim, Jong Leam Choi, Hyo Sook Hong, Wan Seop Kim
Clin Exp Pediatr. 1989;32(11):1586-1591.   Published online November 30, 1989
Dandy-Walker syndrome is a developmental disorder of the brain associated with hydrocephalus, possterior fossa cyst and maldevelopment of the cerebellar vermis and is a result of outlet obstruction of the fourth ventricle. We experienced a case of a 6 day old male neonate who presented macrocephaly which revealed marked dilated fourth & lateral ventricle, large posterior fossa cyst and hypoplastic cerebellum, no vermis on Brain...
A case of Kostmann syndrome.
Won Sup Shin, Sang Woo Kim, In Kee Paik
Clin Exp Pediatr. 1989;32(11):1568-1573.   Published online November 30, 1989
Kostmann Syndrome is an uncommon disorder, which is characterized by recurring infections, begining soon after birth, arid by abscence of granulocyte in the peripheral blood. The authors experienced a case of Kostmann Syndrome in a 5 months old boy who had suffered from recurrent infection since 7 days of age. We diagnosed this case by history, physical examina- tions, culture of bone marrow stem...
Role of aldosterone on the minimal change nephrotic syndrome in children.
Soon Wha Kim, Myung Ik Lee, Don Hee Ahn
Clin Exp Pediatr. 1989;32(11):1526-1532.   Published online November 30, 1989
We studied 11 cases of minimal change nephrotic syndrome randomly selected among 16 cases of minimal change nephrotic syndrome who were proved by kidney biopsy at department of pediatrics, National Medical Center during the period of January 1985 through December 1987. These patients were studied serially at 3 different stages in their clinical course. These were stages of edema formation, diuresis and remission, respectively. The...
Caudal Regression Syndrome Associated with Potter Syndrome.
Hyun Joo Jeong, Sung Sook Bang, Hae Ran Lee, Choon Myung Ro, Yeon Lim Suh
Clin Exp Pediatr. 1989;32(10):1448-1454.   Published online October 31, 1989
Caudal egression syndrome is a term applied to a syndrome comprising complete or partial agenesis of sacrum and coccyx, often associated with malformation of the lower extremities, congenital heart disease, urologic abnormalities and malformation of the upper extremities. The primary defect may occur in the mid-posterior axis mesoderm of the embryo prior to the 4th week of development, resulting in other defects of the...
A Case of Anomalous Origin of the Left Coronary Artery from The Pulmonary Artery(Bland-White-Garland Syndrome).
Jae Sook Ma, Kwang Ok Lee, Tae Ju Hwang
Clin Exp Pediatr. 1989;32(10):1428-1434.   Published online October 31, 1989
The anomalous origin of the left coronary artery from the pulmonary artery (Bland-White -Garland Syndrome) is a relatively rare congenital heart disease, in which the right coronary artery arises normally while the left comoary artery arises from the pulmonary artery. We experienced a case of this anomaly without any other cardiac defect in a 4 month-old female infant. The diagnosis was suspected on the...
A Case of Cockayne Syndrome.
Jong Leam Choi, Kyung Hee Kim, Hyo Sook Hong, Wan Soeb Kim
Clin Exp Pediatr. 1989;32(10):1422-1427.   Published online October 31, 1989
We experienced a case of Cockayne syndrome in a 12 10/12 year-old male who had characteristic manifestations with cachectic dwarfism, severe mental retardation, intracranial calcifications, an unusal face, photo-sensitive dermatitis and joint flexion contractures. Laboratory investigations have not revealed any consistent abnormality. We report this case with brief review of some related literatures.
A Comparative Study of Therapeutic Effect of Intravenous Gammaglobulin plus Aspirin Versus Aspirin Alone in Kawasaki Syndrome.
Boung Yul Leem, Chong Sung Chung, Sung Ho Cha, Yong Mook Choi, Chang Il Ahn
Clin Exp Pediatr. 1989;32(10):1408-1415.   Published online October 31, 1989
We compared the efficacy of intravenous gamma globulin plus aspirin with that of aspirin alone in reducing the frequency of coronary artery abnormahties and changes of clinical coures in children with acute kawasaki syndrome in the children of 44 cases who were admitted in K.H.U.H from Mar. 1986 to Fab. 1988. Group A: High dose aspirin (60〜90 mg/kg/D) during febrile stage and then switched...
Cord Blood Insulin Concentration in Premature Neonates with Respiratory Distress Syndrome.
Soo Jung Keum, Rak Won Choi, Mi Youn Chung, Dong Hyuck Kum
Clin Exp Pediatr. 1989;32(10):1402-1407.   Published online October 31, 1989
Insulin seems to exert an inhibitory effect on lung maturation. The incidence of respiratory distress syndrome in neonates of diabetic mothers is significantly greater than that observed in age-and weight-matched controls. Thus hyperinsulinemia may be an important factor in the developemnt of RDS. We investigated the relationship between cord blood insulin concentration and subsequent develop- ment of RDS in 32 premature neonates born at Koryo...
Study on Antithyroid Antibody and Thyroid Functional Status in Patients with Turner Syndrome.
Bo Young Lee, Duk Hi Kim, Kir Young Kim
Clin Exp Pediatr. 1989;32(10):1397-1401.   Published online October 31, 1989
This study is a systematic cytogenetic and clinico-hormonal analysis of 26 cases who were diagnosed as Turner syndrome at the Yonsei Medical Center from Jan. 1978 to Jan. 1988. Antithyroid antibody and thyroid function tests were performed in Turner syndrome(3 groups according to karyotypes) and control group. The results were as follows: 1) The incidence of positive antithyroid antibody in the 26 cases was 42.3% and...
Selectivity Index of Proteinuria in Childhood Minimal Change Nephrotic Syndrome.
Cheol Ho Lee, Young Seo Park, Kwang Wook Ko
Clin Exp Pediatr. 1989;32(10):1384-1390.   Published online October 31, 1989
The selectivity indices (S.I.) of proteinuria in 120 biopsy proven minimal change nephrotic syn- drome (MCNS) children who were admitted to Seoul National University Childern’s Hospital from January 1976 to June 1988 and followed up for more than 1 year from onset of disease, were analyzed in relation to clinical findings, histopathologic features and steroid responsiveness. The results were as follows: 1) The mean age at...
Plasma Amino Acid Patterns in Reye's Syndrome.
Han Wook Yoo, Hee Shang Youn
Clin Exp Pediatr. 1989;32(10):1377-1383.   Published online October 31, 1989
The metabolic disturbances of Reye’s syndrome are many and involve carbohydrates, amino acids, free fatty acids, proteins, ammonia and acid-base balance. It has been suggested that the derangement of mitochondrial integrity by an agent or agents unknown leads to a loss of enzyme activity in many metabolic pathways in which t노e mitochondria are involoved. Of primary interests in this study is the abnormalities regarding...
A Case of Myelodysplatic Syndrome.
Sun Bok Suh, Chang Ok Soh, Sang Hoon Lee, Chul Ho Kim, Soon Yong Lee
Clin Exp Pediatr. 1989;32(9):1314-1319.   Published online September 30, 1989
We have experienced a case of myelodysplastic syndrome (RAEB) in an 11-year-old boy with the complaints of pallor and petechiae for 1 month. After diagnosis, improvement was remarkable with chemotherapy of low dose cytosine arabinoside but the RAEB was transformed to acute myeloblastic leukemia 4 months after treatmemt. Anti-leukemic therapy was tried but induction of remission was failed and he was died of sepsis...
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