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Case Report
A Case of Reiter's Syndrome.
Y J Kim, Y K Suh, T C Kwon, C M Kang
Clin Exp Pediatr. 1983;26(12):1226-1230.   Published online December 31, 1983
Reiter’s syndrome is a rare disease and no case was reported in pediatric field in this country. We experienced a case of Reiter’ s syndrome, developed to 11 year old boy who presented cardinal symptoms and signs of this syndrome, urethritis, conjunctivitis and arthritis associated with high fever. HLA-B 27 antigen was positive. These conditions were preceded by Shigellosis like enteritis. Symptoms...
A Case of Familial Treacher-Collins Syndrome.
Sang Hee Cho, Hye Sun Chung, Gwi Jong Choi, Heung Jae Lee, Keun Soo Lee
Clin Exp Pediatr. 1983;26(12):1215-1219.   Published online December 31, 1983
The authors experienced a case of familial Treacher-Collins syndrome in 24 years old mother and 2 months old male baby confirmed by typical facial appearance such as antimongoloid slant, coloboma, macrostomia and micrognathia and X-ray findings. We reviewed the literatures briefly.
A Case of Histiocytosis X(Hand-Schuller-Christian Syndrome).
Yeong Bong Park, Jin Heon Kim, Jong Myeon Hong, Chang Soo Ra
Clin Exp Pediatr. 1983;26(12):1209-1214.   Published online December 31, 1983
A case of histiocytosis X (Hand-Schuller-Christian syndrome) in a 3 year old girl is presented. The patients has the triad of exophthalmos, diabetes insipidus and skeletal lesions, plus hepatomegaly, wide spread pulmonary infiltration and growth retardation. The patient was treated with chlorambucil, prednisone, radiation therapy and chlorpropamide therapy for diabetes insipidus with good response. Similar cases reported in literature are briefly...
A Case of Trensient Myeloproliferative Disorder with Down Syndrome.
Dong Gyoon Kim, Jae Ho Lee, Jung Il Noh, Hyo Seop Ahn, Chang Yee Hong
Clin Exp Pediatr. 1983;26(10):1024-1028.   Published online October 31, 1983
Transient myeloproliferative disorder has been reported in infants with Down syndrome. Infants with Down syndrome and this transient myeloproliferative disorder often present with signs and symptoms that are clinically and hematologically indistinguishable from congenital acute myelogenous leukemia. In contrast to congenital AML, complete clinical and hematological recovery occurs within weeks to months of diagnosis without any specific anti- leukemic treatment. A case of transient myeloproliferative disorder...
Original Article
Clinical Observation on Guillain-Barre Syndrome in Children.
Y J Kim, G J Kim, T C Kwon, Y D Kwon, C M Kang
Clin Exp Pediatr. 1983;26(10):986-995.   Published online October 31, 1983
55 cases of Guillain-Barre syndrome which were admitted to the Pediatric Department of Keimyung University, Dong San Hospital during the period of 10 years from January 1973 to December 1982 were reviewed and the clinical observations were attempted. The following results were obtained. There was no specific prevalence in annual incidence. The highest incidence was in summer season and especially low during the winter season....
Hyperlipidemia and Hyperlipoproteinemia in Focal Segmental Glomerulosclerosis Nephrotic Syndrome.
Heui Jeen Kim, Kwang Wook Ko
Clin Exp Pediatr. 1983;26(10):967-977.   Published online October 31, 1983
Sera obtained from 23 hospitalized patients with focal segmental glomerulosclerosis nephrotic syndrome which is confirmed by kidney biopsy, were analyzed for serum albumin, serum lipids, 24hr urine protein, creatinine clearance, HDL-cholesterol. In 19 of the patients lipoproteins were analyzed. the following results were obtained. 1) Serum phospholipid (PL) was increased whenever serum total cholesterol (TC) was increased but to a lesser degree. The ratio of TC/PL...
Case Report
Two Cases of Rotor Syndrome in Brothers.
Sonn Il Kwon, Kum Le Ko, Jong Hun Park, Young Soo Lim, Dong Heuck Kum
Clin Exp Pediatr. 1983;26(9):934-938.   Published online September 30, 1983
Two cases of Rotor syndrome were observed clinically. Both cases were observed as follows; 1. Both cases were observed in a same family; They were brothers. 2. Jaundice was appsirel fro.n aboat sscoai year of life without any clinical disturbance. 3. Oral cholangiography and Ultra sonogram on abdomsn showed normal Findings and functions except slight enlargement of liver. 4. Direct...
A Case of Cushing's Syndrome.
Seong Ja Jeon, Oh Kyung Lee, Keun Chull Choi, Myung Ho Lee, Jong Deok Kim
Clin Exp Pediatr. 1983;26(9):928-933.   Published online September 30, 1983
We experienced a Cushing’s syndrome which was caused by adrenocortical carcinoma in 8-year-old boy. He has suffered from obesity, moonface and growth retardation for 2 years. The diagnosis was confirmed by clinical symptoms, biochemical studies, X-rays and pathologic examinations. A total adrenalectomy was impossible because of far extended mass to adjacent organ, so only tissue biopay was done. After surgery, he received radiation...
A Case of Sturge-Weber Syndrome.
Jun Taek Park, Geom Hyun Jang, Jae Kyu Lee, Kyu Eun Lee, Jung Hyup Oh
Clin Exp Pediatr. 1983;26(8):823-828.   Published online August 31, 1983
We experienced a case of Sturge-Weber Syndrome in 6 month old male. The diagnosis was established by charecteristic features of Sturge-Weber Syndrome such as facial port-wine nevus, focal seizure, hemiparesis, glaucoma and typical calcification & brain atrophy as on brain CT. Plain radiographys of the skull revealed no evidence of calcification, But the brain C.T. clearly showed calcific densities. We have presented this case...
A Case of Edward Syndrome.
Hyun Hwa Kim, Hyun Sook Park, Young Hee Yu, Hyun Sook Lee
Clin Exp Pediatr. 1983;26(7):712-716.   Published online July 31, 1983
A case of 18 trisomy syndrome in a male baby who was born at our Korea Veterans Hospital was presented. Thisbaby has grossly multiple anomalies those were characterized by elongated skull, hypertelorism, low-set malformed ears, high arched palate, micrognathia, microphallus, limited abduction of hips, flexon deformity of fingers, rocker-bottom, feet and short big toes. Chromosomal study showed the 3rd. chromosome in No. 18 chromosome...
A Case of Weismann-Netter Stuhl Syndrome.
Kwang Ho Kim, Hong Ku Lee, Dong Hwan Cha, Jung Sim Choi, Chie Ja Cho, Jung Sook Lee
Clin Exp Pediatr. 1983;26(6):622-625.   Published online June 30, 1983
Weismann-Netter-Stuhl syndrome is congenital anterior bowing of the diaphyses of the tibia and fibula usually bilateral and symmetical, which is clinically resembles the sabre shin of syphilis. A case of Weismann-Netter-Stuhl syndrome in a 5 years oldboy was presented with a brief review of literature. This boy showed stunted growth, anterior bowing of legs and moderate degree of dyphosis, clear intelligence. X-ray showed...
A Case of Cornelia De Large Syndrome.
Do Seung Lee, Dae Young Hwang, Jeong Sick Min, Jae Sun Park
Clin Exp Pediatr. 1983;26(6):616-621.   Published online June 30, 1983
We have experienced a case of1 Cornelia de Lange Syndrome in a male infant. At first visit, the patient showed typical appearance of microcephaly, cleft palate, generalized hirsutism and growth retardation due to poor sucking since birth. The face of the patient was characterized by micrognathia, antirrongolism slant of eyes, synorphys of eye brows, bushy forehead and bushy lateral aspect of both cheeks, all...
A Case of Bartter'S Syndrome.
Yung Suk Song, Moon Ho Chung, Ha Baik Lee, Chong Moo Park
Clin Exp Pediatr. 1983;26(6):611-615.   Published online June 30, 1983
Bartter’s syndrome is rare disease which is characterised by hypokalemic, hypochloremic metabolic alkalosis, hyperreninemia, secondary aldosteronism, hyperplasia of juxtaglomerular apparatus without hypertension. We had experienced a case of Barker's syndrome in 4 months old girl. The diagnosis was made by clinical, hematologic, and biochemical studies. Literatures are reviewed briefly
Original Article
A Clinical Observational on Stenens-Johnson Syndrome.
T W Paik, H Y Chung, T C Kwon, Y D Kwon, C M Kang
Clin Exp Pediatr. 1983;26(5):482-488.   Published online May 31, 1983
A clinical study of 17 cases of Stevens-Johnson Syndrome in infancy and childhood, which were admitted to the Pediatric Department of Keimyung University, School of Medicine during the period of 6 and half years from January 1975 through July 1981 was made. The results are summarized as follows: 1) Stevens-Johnson syndrome has tendency of increasing incidence in recent years. 64.4% of the cases were seen during...
Study of Serum Lipids and Lipoprotein in Patients with Nephrotic Syndrome.
Eui Soo Park, Jeh Hoon Shin, Woo Gill Lee, Chong Moo Park
Clin Exp Pediatr. 1983;26(4):349-357.   Published online April 30, 1983
The nephrotic syndrome is characterized by generalized edema, hypoproteinemia (serum albumin; below 2.5 gm/dl), hyperlipidemia (serum cholesterol; above 220 mg/dl), massive proteinuria (2.0 gm/M2/24 hrs or more). For causes of hyperlipidemia, decreased plasma lipoprotein lipase activity and decreased serum albumin have been postulated as contributing factors. So the serum lipids and lipoproteins were studied in 24 patients with nephrotic syndrome...
Case Report
Two Cases of Di Guglielmo Syndrome.
Seok Ku Kim, Seong Ja Jeon, Chul Lee, Myung Ho Lee, Sook Ja Park
Clin Exp Pediatr. 1983;26(3):289-284.   Published online March 31, 1983
Di Guglielmo Syndrome is a relatively rare disease in childhood and refers to an acute or chronic myeloproliferative disorder in which erythrocytes and their precursors are the cells predominantly involved in the leukemic process. We experienced two cases of Di Guglielmo syndrome, an llyear-old girl and a 14 year-old boy, who had the chief complaints of severe anemia, fever, dizziness and general...
A Case Report Kostmann Syndrome.
Han Wook Yoo, Son Moon Shin, Hyo Seop Ahn, Yong Choi, Chang Yee Hong
Clin Exp Pediatr. 1983;26(3):284-288.   Published online March 31, 1983
Kostmann syndrome (Infantile genetic agranulocytosis), a disease characterized by recurrent infections leading to death in infancy, shows an agranulocytosis with variable monocytosis, eosinophilia in the peripheral blood, accompanied by the maturation arrest of the myelocytic series at the promyelocyte-myelocyte level. A 2 years 3 month old female patient with Kostmann syndrome is reported, who has suffered from recurrent infections since 1 month of age. During...
Original Article
Lipoprotein Profiles in Minimal Change Nephrotic Syndrome.
Yong Choi, Heui Jeen Kim, Hae Il Cheong, Jeong Kee Seo, Kwang Wook Ko
Clin Exp Pediatr. 1983;26(3):237-246.   Published online March 31, 1983
Data obtained from 59 hospitalized patients with minimal change nephrotic syndrome which was confirmed by kidney biopsy, were analyzed for serum albumin, serum lipids, 24hr urine protein, creatinine clearanee, HDL-eholesterol. In 38 of the patients, lipoproteins were analyzed. The following results were obained 1. Serum phospolipid (PL) was increased whenever serum total cholesterol (TC) was increased but to a lesser degree. The ratio of TC/PL were...
Case Report
9 Case of Klinefelter Syndrome.
Hack Joo Cha, Jong Sub Lee, Ki Bok Kim
Clin Exp Pediatr. 1983;26(2):203-208.   Published online February 28, 1983
A cytogenetic study was carried out on 9 cases of Klinefelter Syndrome referred to the Department during a-3-year period from February, 1979 through February, 1982. The following results were obtained. 1) The distribution of age at the time of definite diagnosis was between the age of 17 and 31, average being 23.8 years. 2) Mean height was 173.2 cm. 3) Chief complaints were small genitalia...
A Case of Hypereosinophilic Syndrome.
Chang Moo Lee, Chull Kwon Chung, Soon Ok Byun, Ji Sub Oh
Clin Exp Pediatr. 1983;26(2):193-197.   Published online February 28, 1983
We experienced a case of Hypereosinophilic syndrome with congestive heart failure in 15 month old girl. The clinical, hematologic and E.C.G. findings were compatible with hype- reosinophilic syndrome. She was treated with oxygen, digoxine, antibiotics and mebendazole. But on 8th day of admission she suddenly died. A brief review of the related literature was made on this subject.
Original Article
A Clinical Study on Reye Syndrome.
N C Kim, C H Kim
Clin Exp Pediatr. 1983;26(2):158-164.   Published online February 28, 1983
We analyzed 15 infants and children who were admitted and diagnosed as Reye syndrome. at Gu Mi Soon Chun Hyang Hospital during the period of 9 months from Feb. 1981 to Oct. 1981 The results were as follows. 1) The cases of Reye syndrome were 15 patients (1. 6%) among 579 hospitaHzed patients and 4 patients (26.7%) died of Reye syndrome. 2) It affected male...
Respiratory Distress Syndrome of the New born Infants and Complications of Pregnancy.
Yoon Joo Choi, Eun Ai Lee, Seung Joo Lee, Keun Lee
Clin Exp Pediatr. 1983;26(2):118-123.   Published online February 28, 1983
A group of 514 newborn infants with gestational ages between 28 weeks and 36 weeks were studied for the influence of pronolonged rupture of amniotic membranes and maternal hype- rtension. during pregnancy on. the incidence of Idiopathic Respiratory Distress Syndrome (IRDS). The incidence of IRDS in. complicated pregnancy is that significantly lower than in the control groups. (8.9% vs 18.8%, p<0.05) The occurence of...
Case Report
Eleven Cases of Turner Syndrome.
Jong Sub Lee, Hack Joo Cha, Ki Bok Kim
Clin Exp Pediatr. 1983;26(1):91-96.   Published online January 31, 1983
Among the patients who have been referred to our Cytogenetic Laboratory with the suspicion of Turner Syndrome during the past 8 years, eleven were confirmed by cytogenetic analyses. Clinical features and the results of cytogenetic analyses of those cases are summarized as follows: 1) The patients, all females, ranged in age from 16 to 25 years at the time of first consultation, and their...
Two Case of Ginaotti-Crosti Syndrome.
Jae Ho Lee, Soon Ung Kang, Jeong Kee Seo, Hyo Seop Ahn, Kwng Wook Ko, Won Suk Kim
Clin Exp Pediatr. 1982;25(12):1289-1294.   Published online December 31, 1982
The Gianotti-Crosti syndrome is an. infrequently recognized disorder with distinctive clinical features. We have experienced two cases of Gianotti-Crosti syndrome diagnosed by clinical manifestation, laboratory data and histopathologic finding of skin. Case one; An 18-month-old girl was admitted to the pediatric ward with the chief complaint of erythematous rash. Its onset was preceded by an. upper respiratory tract infection. She had a wide spread erythematous...
A Case of Acardi's Syndrome.
Bok Lyun Park, Hee Jung Chung, Chang Joon Coe, Young Sook Seo
Clin Exp Pediatr. 1982;25(12):1285-1288.   Published online December 31, 1982
A case of Aicardi’s syndrome is experienced at the Department of Pediatrics, College of Medicine, Yonsei University recently. This patient was 50 days of female infant, who had infantile spasm, and her development was deteriorated recently. Her CT scan showed partial absence of corpus callosum and whose EEG was a pattern of modified hypsarrhythmia limited only on left hmisphere. She also had hemivertebra at...
A Case of Potter Syndrome.
Young Mi Hong, Hae Seung Kim, Jung Gon Kim, Keun Lee
Clin Exp Pediatr. 1982;25(11):1181-1184.   Published online November 30, 1982
In 1946 Potter described a series of 20 cases in infants in whom bilateral absence of the kidneys was associated with, hypoplasia of the lung and characteristic face. The main facial features she described were increased space between the eyes, a prominent fold which arises at inner canthus and sweeps downward and laterally below the eyes, unusual flattening of the nose, excessive recession of...
Original Article
Physical Features of Korean Children of Down Syndrome.
Hack Joo Cha, You Nam Kim, Ki Bok Kim
Clin Exp Pediatr. 1982;25(10):994-1006.   Published online October 31, 1982
Ths physical features characteristic of Down Syndrome in Korean children were analyzed in 81 patients confirmed by cytogenetic examination in the Department of Pediatrics, Kwangju Christian Hospital, during the period from March, 1974 through July, 1981, and results are- summarized as follows: 1) The sex ratio was 2 males to one female. 2) The frequency of most stigmata found among Down cases were signifi cantly higher than, among...
A case of Chedia-Higashi Syndrome.
D K Moon, S H Shin, T C Kwon, C M Kang
Clin Exp Pediatr. 1982;25(8):843-847.   Published online August 31, 1982
A case of Chedia-Higashi syndrome in a seven months old male was presented. The diagnosis was suspected by albinism, nystagmus, photophobia, cervical lymphadenopathy, hepatosplenomegaly, past history of frequent infection and was confirmed by the peripheral and bone marrow findings of large purplish inclusion bodies in the cytoplasms of granulocytes and lymphocytes. A brief review of related literature was attempted.
A Case of Infantile Cortical Hyperostosis.
Won Bae Lee, Hyun Koo Oh, Jae Chung Lee, Sung Hoon Cho
Clin Exp Pediatr. 1982;25(8):839-842.   Published online August 31, 1982
Infantile cortical hyperostosis occurs in the first few months of life, and is characterized by irritability, fever, soft tissue swelling and radiological evidence of a periosteal reaction, particularly of the mandible. It's cause is unknown. The disease has been reported over 130 cases worldwidely, with only 2 cases in Korea sofar. Recently we experienced a case of infantile cortical hyperostosis....
Changes in Serum Complement and Immunglonulins following Cardiopulmonary Bypass.
Hong Jin Kim, Jun Hee Sul, Sung Kyn Lee, Dong Shik Chin, Kwang Ho Kim, Pill Whoon Hong
Clin Exp Pediatr. 1982;25(8):781-786.   Published online August 31, 1982
Clinical problem occuring in the early post operative period after cardiopulmonary bypass may ne related to the altered function of capillary bed which was affected by activation of complement system. This is so called post perfusion syndrome. We selected 20 patients with confirmed congenital heart disease by cariac catheterization. We studied the changes in the C3 , immunolglobulins, total protein,...
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