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Original Article
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...
A Case of Potter Syndrome Type Ⅰ.
Jin Hong Park, Mi Jeong Koh, Yeon Kyun Oh, Chan Choi
Clin Exp Pediatr. 1989;32(8):1150-1154.   Published online August 31, 1989
We experienced a case of Potter syndrome type I characterized by polycystic kidney, pulmomnary hypoplasia and abnormal face in a 1 day-old male neonate with respiratory distress. Diagnosis was made with autopsy findings. A brief review of literature was made.
A Case of Peutz-Jeghers Syndrome.
Ick berm Park, Sung guen Oh, Jung suk Ko, Sang hyun Byun, Kyu chul Choeh
Clin Exp Pediatr. 1989;32(7):990-994.   Published online July 31, 1989
This is a report of our experience of Peutz-Jeghers syndrome in a 14 year old girl having family history. She was admitted with complaints of crampy abdominal pain and vomiting, and showed melanotic pigmentation on the lips, face around nose, oral mucosa around molar areas, fingers and toes. Colon study revealed multiple polyposis in colon and small bowel. In family, her father has melanotic...
A Case of Laurence-Moon-Biedl Syndrome Including Diabetic Mellitus.
Byoung Hoon Lee, Byung Rai Cho, Myoung Ik Lee, Keun Chan Sohn, Hyung Joon Yoo
Clin Exp Pediatr. 1989;32(6):857-861.   Published online June 30, 1989
Laurence-Moon-Biedl syndrome has a varialbe phenotype and includes obesity, mental deficiency, retinitis pigmentosa, polydactyly and hypogonadotrophic hypogonadism. This report concerns a rare case of a 13-year-old girl having diabetic mellitus with Laurence-Moon-Biedl syndrome. The relevant literature was reviewed.
A Case of Prune Belly Syndrome.
Myoung Suk Nam, Soon Soen Lim, Young Ha Kim, Moon Ki Joe
Clin Exp Pediatr. 1989;32(6):852-856.   Published online June 30, 1989
We have recently experienced a case of prune.belly syndrome in 1.day.old male with congenital defect of abdominal muscles, both cryptorchidism, ureter and bladder dilatation, left renal cyst. Imperforate anus and left club foot were associated. The diagnosis was confirmed by autopsy. A brief review of Iiterature was made.
3 Cases of Prune Belly syndrome.
Chong Sung Chung, Chong Woo Bae, Byung Soo Cho, Chang Il Ahn, Ju Hie Lee, Moon Ho Yang
Clin Exp Pediatr. 1989;32(6):845-851.   Published online June 30, 1989
The prune belly syndrome is a rare congenital anomaly characterized by laxed, wrinkled abdominal wall, cryptorchidism and urinary tract anomalies. But it has wide spectrum of clinical severity, clinical presentation and other anomalies such as pulmonary and skeletal anomalies. We suggested that we should examine the abdominal wall carefully in order to detect mild form (class III) of prune belly syndrome when we...
A case of Evans Syndrome Associated with A-V Malformation.
Keun Haeng Cho, Min Young Lee, Kwang Chul Lee, Young Sook Hong, Soon Kyum Kim
Clin Exp Pediatr. 1989;32(6):839-844.   Published online June 30, 1989
In 1951, Evans and associates first described a group of patients with primary thrombocytopenic purpura for a diagnosis of Evans syndrome, The criteria for a diagnosis of Evans syndrome were(l) hemolytic anemia with a positive direct coombs’ test and thrombocytopenia occuring either simultaneously or in succession and(2) the absence of any known underlying etiology. We experienced a case of Evans syndrome associated with...
Bone Age Determination and Hand Radiographic Findings in Children With Russell-Silver Syndrome.
Hun Kyu Lim, Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1989;32(6):823-833.   Published online June 30, 1989
Russell.Silver syndrome is a disease of unknown etiology characterized by prenatal onset dwarfism irrespective of full term gestation, growth retardation, body asymmetry, craniofacial dysproportion with a triangular face, short and incurved little finger, etc. Forty.two children were diagnosed as Russell.Silver syndrome by Silver’s criteria at SNU Children' s Hospital from Apr. 1981 till Aug. 1988, and we reviewed clinical data and hand radiographs of...
Serum IgE Level in Patients with Minimal Change Nephrotic Syndrome.
Sung Oh Kim, Hee Jung Ahn, Seong Ho Kim, Woo Gill Lee
Clin Exp Pediatr. 1989;32(6):789-796.   Published online June 30, 1989
It has been reported that many patients with minimal change nephrotic syndrome(MCNS) have high serum IgE level, but its significance has not been disclosed yet. In this study, serum levels of IgE were evaluated in different stages of the disease with or without treatment in 26 MCNS(proven by renal biopsy) patients admitted to the Department of Pediatrics Hanyang University Hospital from January, 1980 to...
Two Cases of Poland Syndrome.
Kul Ha Yoo, Ki Soo Pai, Byung Ju Chung, Chul Lee, Dong Gwan Han, Jong Doo Lee
Clin Exp Pediatr. 1989;32(5):713-717.   Published online May 31, 1989
Poland syndrome is characterized by an absence of the pectoralis major muscle, with ipsilateral defect of the upper extremity, usually syndactyly. The incidence of this syndrome has been estimated at 1 per 25,000 to 77,000 persons and more than 100 patients have been described worldwide. Recently, we experienced two patients, the one was 14 year old male, who showed absence of left pectralis maior...
A Case of Virus Associated Hemophagocytic Syndrome.
Yang Soo Kang, Ue Chong Yang, Hae Il Cheoung, Ho Jin Park, Mi Ja Shin, Je Geun Chi
Clin Exp Pediatr. 1989;32(4):567-575.   Published online April 30, 1989
Virus associated hemophagocytic syndrome is a rare hemophagocytic disorder, apparently associated with active viral infection, apparently associated with active viral infection, in which reactive histiocytes proliferate in the reticuloendothelial system, mainly bone marrow and medullary portions of lymph nodes, as a non-malignant, reversible but potentially constitutional symptoms with high fever and hepatosplenomegaly and by laboratory findings of pancytopenia, hemophagocytosis, abnormal liver function and coagulopathy. Inappropriate immunosuppressive...
A Case of Hypereosinophilic Syndrome.
Byoung Hoon Lee, Jae Wook Ko, Soon Wha Kim, Don Hee Ahn
Clin Exp Pediatr. 1989;32(4):561-566.   Published online April 30, 1989
Hypereosinophilic syndrome is a state of severe eosinophilia of unknown cause accomanied by multiple organ-system involvement. The criteria of hypereosinophilic syndrome includes. (1) A persistent eosinophil count of more than 1.5 X 10 9 /L for associated with the signs and symptoms of hypereosinohilic disease; (2) a lack of evidence for parasite, allergic, or other known causes of eosinophila; (3) presumptive signs and...
A Case of Edward Syndrom.
Youn Hong Choi, Hyun Joo Choi, Eun Hwa Shin, Ju Hong Cha, Kwang Jeon Kim
Clin Exp Pediatr. 1989;32(3):396-401.   Published online March 31, 1989
Edward syndrome is known as 18-trisomy with multiple congenital anomalies. Recently, we experienced a case of 18-trisomy syndrome in a new-born female baby who was born at Chung Gu Sung Sim Hospital. Grossly this baby had multiple anomalies which were characterized by prominent occiput, hypertelorism, small palpebral fissure, small oral opening, high arched palate, micrognathia, low-set malformed ear, rocker bottom feet, narrow pelvis, limited...
Clinical Application of Shake test od Gastric Aspiretes for the Prediction od Respiratory Distress Syndrome in the Newborn infants.
In Sang Jeon, Hann Tchah, Myoung Jae Choi, Beyng Il Kim, Jung Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1989;32(3):349-358.   Published online March 31, 1989
To study the predictive value of gastric aspirates shake test on respiratory distress syndrome (RDS) in the newborn infants, we carried out the shake test on gastric aspirates of all newborn who admitted to NICU of Seoul National University Hospital from July 1986 to December 1987. We successfully carried out gastric aspirates shake test on 96 newborn infants and analyzed the relation between gastric...
A Case of Congenital Megakaryoblastic Leukemia Accompanied by Down Syndrome Which was Diagnosed by Autopsy Findings.
Il Kyung Kim, Dong Beom lee, Jung Sik Min, Chang Hee Choi, Je Geun Chi
Clin Exp Pediatr. 1989;32(2):262-269.   Published online February 28, 1989
Many cases of acute megakaryonlastic leukemia have been reported in childhood or adults, but congenital form or the cases occuring in neonatal period is very rare throughout the world. In Korea only one case was reported previously by Chung et al in 1987. The authors experienced a case of congenital megakaryoblastic leukemia accompanied by Down' s syndrome, which was confirmed by autopsy and positive...
11 q Deletion Syndrome.
Y B Im, S B Park, B Y Pyun, J O Park, S J Lee, S Y Moon
Clin Exp Pediatr. 1989;32(2):239-243.   Published online February 28, 1989
llq deletion syndrome is a rare chromosomal anomaly. The authors experienced a case of llq deletion syndrome with congenital glaucoma and infantile spasm in a female child. She showed delayed psychomotor development, flat occiput, hypertelorism, low set malformed ears, flat nasal bridge, short neck, simian line on right palm and throm. bocytopenia. Chromosomal study showed the deletion of the long arm of chromosme 11, karyotypically...
A Case of Neonatal Short Bowel Syndrome.
Gill Hyoun Kim, In Chul Yoo, Dong Hwan Lee, Sang Jhoo Lee
Clin Exp Pediatr. 1989;32(1):81-86.   Published online January 31, 1989
We experienced a case of neonatal short bowel syndrome due to multiple jejuno-ileal atresia with microcolon. On the 1st day of her life, there were symptoms of vomiting and abdominal distension. The radiological study show multiple dilated intestinalloops and malrotation. On the 4th day of her life, the extensive enterectomy was done. The remaining small bowel consisted of 30 cm of proximal jejunum and distal...
Clinical Observation on Guillain-Barre Syndrome in Children.
Byung Ran Yun, Hye Kyung Han, Yong Seung Hwang
Clin Exp Pediatr. 1989;32(1):52-61.   Published online January 31, 1989
Clinical observation was performed on 65 cases of Guillain -Barré syndrome which were en at the Department of Pediatrics, Seoul National University Hospital, during the past 10 years from January 1977 to December 1986. The results were as follows: 1) The ratio of male to female was 2:1 and 56.9% of the total cases were seen between the ages of 1-5 years. 2) The highest seasonal...
A Case of Beckwith-Wiedemann Syndrome.
Jeong Wi Wook, Eun Young Choi, Min Shik Kim, Hey Sun Lee, Young Min Ahn
Clin Exp Pediatr. 1988;31(12):1685-1688.   Published online December 31, 1988
We experienced one case of Beckwith-Wiedemann syndrome. This 5 day aged female neonate manifested gigantism, facial nevus flammeus, macroglossia, Promi- nent occiput, unusual linear fissures in lobule of external ear, omphalocele, gall bladder hydrops, and mild hypoglycemia. A brief review of related literature was done.
A case of Marfan Syndrome.
T U Kim, S H Bae, H S Yoon, S H Kim
Clin Exp Pediatr. 1988;31(12):1680-1684.   Published online December 31, 1988
We experienced a case of Marfan syndrome in 10 year-old girl with dolichomorphism, myopia, dilatation of ascending aorta, PDA, mitral valve prolapse. Diagnosis was established by clinical characteristics with echocardiography and cardiac catheter- ization. And also, Pertinent literatures on Marfan svndrome were reviewed briefly.
A Case of Kartagener's Syndrome.
Young Jin Min, Chang Il Ahn, Kyung Sook Cho, Jong Dae Cho
Clin Exp Pediatr. 1988;31(11):1522-1526.   Published online November 30, 1988
We experienced a case of Kartagener’s syndrome in a 15 year-old male patient who showed bronchiectasis, sinusitis, and situs inversus by X-ray and physical examination. We report a case of Kartagener's syndrome and a brief review of literliture was done.
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