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Original Article
A Case of Kasabach-Merritt Syndrome.
Eun Hwa shin, Youn Hong Choi, Ju Hong Cha, Kwang Jun Kim
Clin Exp Pediatr. 1988;31(7):935-941.   Published online July 31, 1988
Kasabach-Merritt Syndrome is a combination of a rapidly enlarging hemangioma and throm- bocytopenia. A 5 day old male newborn who had giant hemangioma associated with thrombocytopenia and anemia is reported with review of literatures. He was placed on prednisolone, blood transfusion, and compression with the elastic bandage over hemangioma. Hemangioma has been somewhat regressed, bleeding tendency has been improved, however, platelt counts are still low.
Study on Diagnostic Methods for Spleen in Complex Heart Disease.
Jung Yun Choi, Yong Soo Yun, Chung Il Noh, Chang Yee Hong, Kyung Mo Yeon, In One Kim
Clin Exp Pediatr. 1988;31(7):833-840.   Published online July 31, 1988
Thirty one cases of congenital heart disease with suspicious splenic anomaly were prospectively studied at Seoul National University Children’s Hopsital from October, 1986 to February, 1988 in order to determine the sensitivity and the specificity of various non-invasive diagnostic methods for splenic status. In most cases, splenic status was confirmed by abdominal aortography. Each case had chest X-ray, abdominal X-ray, bronchus tomography, electrocardiogram, echocardiography,...
A Case of Mucocutaneous Lymph Node Syndrome Complicated by Partial Intestinal Obstruction.
Dong Won Choi, Ki Sup Chung, Jin Suk Suh
Clin Exp Pediatr. 1988;31(6):796-802.   Published online June 30, 1988
Mucocutaneous lymph node syndrome was first described as a new clinical disease by Kawasaki in Japan in 1967. Recently, there have been numerous reports of this disease throughout the world and mucocutaneous lymph node syndrome has become familiar to the pediatrician. Complications previously described include coronary arteritis, carditis and gall bladder hydrops, aseptic meningitis and arthritis. We experienced 1 case of mucocutaneous lymph node syndrome...
Hypoplastic Left Heart Syndrome : Report of a Unique Survivor.
Hang Bo Cho, Seong Ho Kim, Ha Baik Lee, Kyu Hwang Rhee, Chong Moo Park
Clin Exp Pediatr. 1988;31(6):790-795.   Published online June 30, 1988
Hypoplastic left heart syndrome is a rare, and fatal congenital heart disease. Most of the patients with this syndrome die within the 1st month of life. Recently we experienced a hypoplastic left heart syndrome in a child who was unusually living without surgical intervention for 3 and half years. The unique clinical course might result from a number of favorable hemodynamic factors and intact...
Waardenburg's Syndrome in a Boy and Mother.
D H Kim, M Y Kim, C S Suh, S Y Kim, J Y Jung
Clin Exp Pediatr. 1988;31(5):655-659.   Published online May 31, 1988
Waardenburg's syndrome is a rare autosomal dominant disorder which is characterized by (1) lateral displacement of the medial canthi of the eyes and the inferior lacrimal puncta. (2) a broad, prominent root of the nose (3) hyperplasia of the medial portions of the eyebrows (4) white or grey forelock (5) partial or total heterochromia of the irides and (6) deaf-mutism. The authors experienced 2...
A Case of Congenital Nephrotic Syndrome.
Choong Sun Kang, Chang Hee Hwang, Pyung Kil Kim, Hyeon Joo Jeong, In Joon Choi, Jung Hye Choi
Clin Exp Pediatr. 1988;31(5):627-634.   Published online May 31, 1988
Congenital nephrotic syndrome is an uncommon disorder, which is caused by a heterogenous group of renal diseases with variable etiology and natural history. It may be inherited, sporadic, acquird or a part of a general malformation syndrome. This disorder has no single or pathological feature for pathognomonic finding, and there is no ultimate curative treatment except renal transplantation. We experienced a case of congenital...
Burkitt Lymphoma in Children.
Soo Jong Hong, Dong Kyu Jin, Hee Young Shin, Hyo Seop Ahn, Chang Yee Hong, Je G Chi
Clin Exp Pediatr. 1988;31(4):482-491.   Published online April 30, 1988
Seventeen cases of Burkitt lymphoma under 15 years of age who were admitted to Dept, of Ped., Seoul National University Hospital from Jan. 1979 to Dec. 1986 were reviewed and analysed. One hundred and six cases of non-Hodgkin lymphoma were seen during the same period. 1) Burkitt lymphoma accounted for 16% of non-Hodgkin lymphoma. 2) The age ranged from 10/12 year to 15 5/12 years, with...
Physical Features, Karyotypes and Dermatoglyphics of 113 Children with Down Syndrome.
Byung Ho Lim, Kyoung Sim Kim, Ki Bok Kim
Clin Exp Pediatr. 1988;31(4):474-481.   Published online April 30, 1988
The physical features including dermatoglyphic patterns as well as the karyotype patterns were assesed in 113 children with Down Syndrome who had been cytogenetically confirmed in the Department during past 5 years from August 1981. The results are summarized as follows: 1) The sex ratio of the patients was 1.2 male to one female. 2) Cytogenetic examination revealed 21-trisomy in 79% and translocation in 21% of all...
A Case of Kasabach-Merritt Syndrome.
S H Lee, S J Kim, H D Cha, T W Paik, C M Kang
Clin Exp Pediatr. 1988;31(3):398-403.   Published online March 31, 1988
Kasabach-Merritt syndrome (Giant hemangioma associated with thrombo-cytopenia) is a rare disease in infants and ifs hemorrhgic manifestations in these patients are frequently severe enough to threaten life. We experienced a case of this syndrome in a 19 days old boy who had a huge hemangioma on the left upper arm, left anterior chest and back, associated with thrombocytopenia at birth. The patient also had pneumonia...
A Case of Fetal Alcohol Syndrome.
Kee Don Hong, In Chul Yoo, Hung Sub Choi, Dong Hwan Lee, Snag Jhoo Lee
Clin Exp Pediatr. 1988;31(3):375-380.   Published online March 31, 1988
We experienced a case of fetal alcohol syndrome in 9 day old male neonate who delivered at term. On maternal history, his mother had been drunk as much as 64 to 96 cc of alcohol everyday during first trimester and third trimester. The baby had a small for gestational age, microcephaly, bilateral micro-ophthalmia, short palpebral fissure, shallow philtrum, posterior rotation of helix, vocal...
A Case of 18P-Syndrome with Frequent Intection.
Ellen A Kim, Jong Sung Eum, Hee Ju Kim, Sung Ill Ahn, Kyung Yu Park
Clin Exp Pediatr. 1988;31(3):370-374.   Published online March 31, 1988
We herein reported a case of newborn with round face, low nasal bridge, micrognathia, down turning comers of mouth, large protruding ears, high arched palate with webbed short neck: This odd looking newborn was confirmed to have deltion of short arm of chromosome 18 (18P- syndrome). Frequent episodes of pneumonia developed until recently at 6 months of age. The cause of this frequent infection...
Acute Tumor Lysis Syndrome in Burkitt Lymphoma.
Dong Kyu Jin, Soo Jong Hong, Il Soo Ha, Hyo Seop Ahn, Yong Choi, Chang Yee Hong
Clin Exp Pediatr. 1988;31(3):362-369.   Published online March 31, 1988
To assess the tumor lysis syndrome in Burkitt lymphoma, we analyzed the clinical and laboratory findings of 17 patients with Burkitt lymphoma who were admitted to the Department of Pediatrics, Seoul National University Hospital from January 1979 to December 1986. The acute tumor lysis syndrome was diagnosed when two of the five following metabolic abnormal- ities were met: ① hyperuricemia (≥8.0mg/dl), ② hyperphosphatemia (≥5.5 mg/dl),...
Clinical Study on 19 Cases of Neurocutaneous Syndrome in Children.
S H Lee, S J Kim, H D Cha, H S Kim, T C Kwon, C M Kang
Clin Exp Pediatr. 1988;31(3):355-361.   Published online March 31, 1988
The clinical study on 19 cases of neurocutaneous syndrome which were diagnosed at Pediatric Department of Dong San hospital, Keimyung University for last 10 years from Mar. 1975 to Feb. 1985 were performed and following results were obtained. Out of 19 cases, 11 cases were neurofibromatosis, 4 were Tuberous sclerosis and 4 were Sturge- Weber syndrome. 9 cases were male and 10 cases were...
A Case of Cornelia de Lange Syndrome.
Jung Han, Soo Taek Bae, Soon Ok Byun, Ji Sub Oh
Clin Exp Pediatr. 1988;31(2):282-287.   Published online February 28, 1988
The Cornelia de Lange syndrome is characterized by severe growth and mental retardation, typical face, and low-pitched, weak, growling cry, as was first described by Cronelia de Lange in 1933. We have recognized a case of Cornelia de Lange syndrome in a 4 month old Korean girl. The patient showed typical appearance of face with low forehead, bushy eyebrows and synophrys, long curly eyelases,...
A Case of Wilson-Mikity Syndrome.
Jae You Choi, Sang Ho Choi, Byung Hak Lim, Im Ju Kang
Clin Exp Pediatr. 1988;31(2):241-245.   Published online February 28, 1988
We experienced a male newborn patient with Wilson-Mikity Syndrome who showed characteristic X-ray findings and clinical manifestation. We managed the patient conservatively. During in the course, a congestive heart failure with cor pulmonale was developed. He was gradually recovered clinically and radiologically with management. We report one case of Wilson-Mikity Syndrome with review of literature.
A Study on Minimal Change Nephrotic Syndrome in Children.
Yong Choi, Hong Jin Lee, Hae Il Cheong, Kwang Wook Ko, Hyun Soon Lee, Yong Il Kim
Clin Exp Pediatr. 1988;31(2):220-226.   Published online February 28, 1988
To assess the clinical significance of histopathologic variants of minimal change nephrotic syn- drome (MCNS) by light microscopic findings, 148 biopsy proven MCNS patients, who were admitted to Seoul National University Hospital between the period from January 1976 to December 1986, were analyzed according to clinical findings, steroid responsiveness, and follow up status in relation to the pathologic variants. The followings are summary and conclusion. 1) Significantly...
A Case of DiGeorge's Syndrome.
Jong Sik Kim, Dae Young Kim, Kang Ho Kim, Choon Ho Park, Young Bong Park, Kwang Rhun Koo, Chang Soo Ra
Clin Exp Pediatr. 1988;31(1):100-105.   Published online January 31, 1988
DiGeorge’s syndrome is characterized by partial or complete absence of the parathyroid gland and thymus gland and often associated with the cardiovascular and craniofacial anomalies. A-22-days old male infant, delivered without complications at term after a normal pregnancy, developed irritability, feeding difficulties, dyspnea and convulsion. He was characterized by a low-set, malformed right ear, short philtrum of the upper lip, absence of the thymic...
A Case of Congenital Myeloblastic Leukemia Associated with Down's Syndrome.
Ho Jin Oh, Kee Hyoung Lee, Chang Sung Son, Hyun Keum Lee
Clin Exp Pediatr. 1987;30(12):1468-1474.   Published online December 31, 1987
A 4 day old male patient was admitted to our hospital due to jaundice, poor feeding and abdominal distention. His clinical features and peripheral blood smear revealed congenital myeloblastic leuke- mia which confirmed by autopsy. Chromosome study showed 47, XY, +21 in Karyotype. We report this case with review of literatures.
A Case of Short Rib-Polydactyly Syndrome, Type 3.
Yong Woo Park, Jun Ho Kim, Yo Han Kim, Eun Ryong Kim, Soon Jai Lee, Young Jin Kim
Clin Exp Pediatr. 1987;30(12):1439-1445.   Published online December 31, 1987
A case of the Naumoff type (type 3) of short rib-polydactyly syndrome is reported. The skeletal changes included bulging forehead, flat occiput, short cranial base, extremely short horizontal ribs, abnormal vertebrae, and post-axial polydactyly. Microscopically, cartilage showed markedly stunted and disorganized endochondrial ossification. Extraskeletal manifestation were cleft palate, pulmonary hypoplasia, hydropelvis and hydroureter with narrowing of ureterovesical junction. Differentiation from other type of short rib-polydactyly syndrome...
A Case of Larsen Syndrome.
Dong Chul Park, Chan Jun Coe, Duk Jin Yun
Clin Exp Pediatr. 1987;30(11):1305-1310.   Published online November 30, 1987
An infant is descirbed who has multiple joint dislocations, unusual facies, and bony abnormalities, which constitute a syndrome first reported by Larsen. This report expands Larsen syndrome with additional findings such as apparent diminshed rigidity of cartilage in infancy, short nails, unusual juxtacalcaneal “accessory” bone, and mental retardation. The joint lesions and mental retardation require prolonged observation and treatment is limited mainly symptomatic therapy and...
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...
A Case of Potter Syndrome with Bilateral Polycystic Kidneys.
Chun Hwa Lee, Jung Hwan Choi, Yong Choi, Chong Ku Yun, Yeon Lim Suh, Je G Chi, Son Moon Shin
Clin Exp Pediatr. 1987;30(11):1282-1286.   Published online November 30, 1987
In 1946, Potter described a series of 20 cases of infants in whom bilateral absence of the kidneys was associated with hypoplasia of the lungs and characteristic faces. Afterwards it has been known that any condition that causes significant reduction in urine formation or excretion will result in oligohydramnios accounts for many of the nonrenal features of Potter syndorme. We report a case...
A Case of Mucocutaneous Lymph Node Syndrome Complicated by Aseptic Meningitis, Hydrops of the Gallbladeder and Dilatation of the Coronary Arteries.
Ho Jin Lee, Sung Sook Cho, Hyung Sik Moon, Hong Kue Lee, Soon Il Lee, Young Seok Lee
Clin Exp Pediatr. 1987;30(11):1259-1264.   Published online November 30, 1987
We experienced 1 case of mucocutaneous lymph node syndrome complicated by aseptic meningitis, hydrops of the gallbladder and dilatation of the coronary arteries in 2 year 8 month old female infant. Her clinical synptoms, laboratory data and clinical courses were presented with brief review of the literatures. This baby admitted because of high fever and vomiting for 6 days. She had injection of...
Factors Influencing the Prognosis of Reye Syndrome.
Chang Ho Hong, Heung Dong Kim, Pyung Kil Kim
Clin Exp Pediatr. 1987;30(11):1252-1258.   Published online November 30, 1987
While the etiology of Reye syndrome is still unknown, wide variations in both the clinical spectrum and the therapeutic outcome of patients have been noted. We reviewed the cases of 42 Reye syndrome patients who were admitted to Young Dong Severance Hospital during the period from April, 1983 to July, 1986 and analyzed factors influencing the prognosis of Reye syndrome. The patients were divided...
A Clinical Studey on Reye Syndrome.
Myung Ho Oh, Hung Sub Choi, Chang Hwi Kim, Sang Jhoo Lee
Clin Exp Pediatr. 1987;30(11):1243-1251.   Published online November 30, 1987
We took clinical study for 47 cases of Reye syndrome at S.C.H. University Hospital during last 8 years from Jan:, 1979 to Dec., 1986. The results obtained were as follows; 1) There was no significant difference in annual incidence and the seasonal incidence was highest in sumer and autumn. 2) The sex ratio of male to female was...
Plasma Renin Acivity in Children with Acute Glomerulonephritis and Nephrotic Syndrome.
Oh Kyun Kwon, Young Hun Chung
Clin Exp Pediatr. 1987;30(11):1213-1220.   Published online November 30, 1987
This is a study on plasma renin acitivity (PRA) which was obtanined from the patients with acute glomerulonephritis or nephrotic syndrome and normotensive control group admitted in Chungnam National University Hospital from Oct. 1985 to Aug. 1986 to confirm any differences of PRA between groups and to establish their influencing factors. The results were as follows; 1) PRA of noimal children in preschool age (3.49 ±0.98...
Case Report
A Case of Beckwith-Wiedemann Syndrome.
Hyo Sook Hong, Hong Chul Lee, Oh Kyung Lee, Myung Ho Lee
Clin Exp Pediatr. 1987;30(10):1166-1169.   Published online October 31, 1987
Beckwith-Wiedemann syndrome constitutes a clinicopathologic entity characterized macroglossia, omphalocele or umblical hernia, visceromegaly, postnatal gigantism, microcephaly, Nevus flammeus, ear lobe grooves, hemihypertrophy and other abnormalities. The anomalies of the syndrome are facultative and not obligatory. We experience one case of Beck with-Wiedemann syndrome. This 1-day-aged female neonate showed macroglossia, gigantism, omphalocele and hypoglycemia. Review of literature was done briefly.
Case of Peripheral Neuroepithelioma with Ectopic ACTH Syndrome.
O Y Lee, K S Lee, H I Chung, H J Park, M J Shin, K J Cho, J G Chi
Clin Exp Pediatr. 1987;30(10):1152-1160.   Published online October 31, 1987
Peripheral neuroepithelioma is a rare and controversial neoplasm that may occur at any age. The authors observed the course of a 1-year-old Korean female infant who presented with an enlarging mass in the right hand and manifested hepatic and pulmonary metastases at the time of diagnosis. She also presented with characteristic Cushingoid features, such as moon face, buffalo hump, and truncal obesity. Laboratory...
Original Article
A Clinical Studies in Patients with Turner's Syndrome.
Chang Hyun Yang, Duk Hi Kim, Kir Young KIm, Young Ho Yang
Clin Exp Pediatr. 1987;30(10):1143-1151.   Published online October 31, 1987
This study is systematical cytogenetic and clinico-hormonal analysis of 42 cases who were diagnosed as Turner’s syndrome at the Yonsei Medical Center from Jan. 1971 to Dec. 1985. The careful history taking, physical examination, cytogenetic studies, radiologic and hormonal evalua- tion, and GnRH stimulation test were performed. The results were as follows. 1) The patients ranged in age from 14 months to 30 years at the...
Case Report
A Case of Alport's Syndrome.
Jung Bae Lee, Jong Kyun Lee, Pyung Kil Kim, Hyeon Joo Jeong, In Jun Choi
Clin Exp Pediatr. 1987;30(9):1040-1048.   Published online September 30, 1987
We have experienced a case of Alporfs syndrome. The patient was suspicious to make the dignosis of Alporfs syndrome at first examination when he was five years and seven months old. At that time diagnostic work-up were included audiogram and kidney biopsy. His family history was more impressive. His elder brother has died with undetermined cause of nephrotic syndrome one month ago and his...
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