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Case Report
A Case of Stevens-Johnson Syndrome.
Jang Kwun Yang, Byeung Kuk Jung, Jeong Bok Lee, In Sil Lee, Won Jae Park, Won Ho Lee
Clin Exp Pediatr. 1985;28(8):812-816.   Published online August 31, 1985
We have experienced a case of Stevens-Johnson Syndrome in a 10 yr 11 mo old girl with unknown etiology. The pertineiit literatures are reviewed briefly.
Original Article
A Clinical Study on Reye's Syndrome.
Hye Sun Jung, Seong Ryong Hyon, Ha Baik Lee, Soo Jee Moon, Chong Moo Park
Clin Exp Pediatr. 1985;28(8):783-794.   Published online August 31, 1985
Ninety three cases of Reye's Syndrome were admitted to pediatric ward of Hanyang University Hospital during 6 year period from January 1979 to December 1984. The following results were obtained; 1)The peak age incidence was under 1 year of age. The sex incidence revealed male predominance with sex ratio 1.6 to 1(M : F). 2)The peak seasonal incidence was noted in...
A Clinicopathological Study on Renal Biopsies in Children.
Chung Hye Chu, Byoung Soo Cho, Chang Il Ahn
Clin Exp Pediatr. 1985;28(7):705-713.   Published online July 31, 1985
A clinical and histopathological study was made on 53 cases of ultrasonoguided renal biopsies in children who were admitted to pediatric ward of Kyung Hee University Hospital, from February 1, 1979 to March 31, 1984. The result were as follows: 1)The final diagnosis was confirmed by ultrasonoguided renal biopsy in this 53 cases and there was no severe complication (perirenal hematoma, renal...
Primary Peritonitis in Children with Nephrotic Syndrome.
Hae Il Cheong, Whan Jong Lee, Jeong Kee Seo, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1985;28(7):695-704.   Published online July 31, 1985
Thirty episodes of primary peritonitis in 22 children with nephrotic syndrome who had admitted in the Department of Pediatrics, Seoul National University Hospital from Jan. *81 to Sept. ’84 were studied, and the results were as follows; 1)Among 22 children, male to female ratio was 16 : 6 and the mean age at the onset of peritonitis was 7.80 years (average...
A Clinical Study on Cardiofacial Syndrome (Asymmetric Cry Syndrome).
Chung Il Noh, Chang Yee Hong
Clin Exp Pediatr. 1985;28(7):637-641.   Published online July 31, 1985
Seven patients with cardiofacial syndrome were observed for twenty months from Oct. 1983 to May. 1985 at the Pediatric Department of Seoul National University. Age ranged from 1 months to 10 years and six patients were male. Right-sided facial palsy occurred in 4 patients and left-sided in 3 patients. Cardiac defects were TOF, VSD(membranous), Criss-Cross heart with large VSD, pulmonary...
Case Report
A Case of Peutz-Jeghers Syndrome.
Sang Kie Kim, Choon Ho Park, Jin Heon Kim, Keun Chul Myung, Chang Soo Ra
Clin Exp Pediatr. 1985;28(6):622-626.   Published online June 30, 1985
Peutz-Jeghers syndrome, also known as intestinal polyposis II, is a familial condition characterized by the triad of (1) mucocutaneous pigmentation, (2) benign polyps occuring in any part of the intestinal tract but mainly in the jejunum, and (3) autosomal dominant inheritance. This syndrome was first reported by Peutz in 1929. Following reemphasis by Jegers in 1949, it became a definite...
Two Cases of the Rud Syndrome in Siblings.
Mi Jeong Lee, Myung Geul Yum, Jung Hwan Choi, Chong Ku Yoon, Hyung Ro Moon
Clin Exp Pediatr. 1985;28(6):610-615.   Published online June 30, 1985
The Rud syndrome, which is characterized by ichthyosis, mental retardation, seizure, dwarfism, sexual infantilism, etc., was described by Rud in 1927 at first. It is hereditary disorder whose mode of inheritance is autosomal recessive. Because the incidence of this syndrome is very rare, the Rud syndrome has not been reported till now in Korea. We experienced two cases of the...
Original Article
Associated Ventilation with Mechanical Aids in Newborn Infants.
Eue Cho Yang, Jung Hwan Choi, Chong Ku Yun
Clin Exp Pediatr. 1985;28(5):435-447.   Published online May 31, 1985
Clinical features of 85 neonates who treated with assisted ventilation in Neonatal Intensive Care Unit at Seoul National University Hospital from April ’80 to Dec. 5 84 were analyzed with special respect to mechanical aids. The results were as follows; 1)According to diagnostic categories, 56 cases (66%) were hyaline membrane disease (HMD), 14 cases (16%) were postasphyxia syndrome (PAS), 9 cases...
Case Report
A Case of Hutchinson-Gilford Progeria Syndrome.
Seon Ock Khang, Jung Hee Lee, In Joon Seol, Gwi Jong Choi, Keun Soo Lee
Clin Exp Pediatr. 1985;28(4):405-410.   Published online April 30, 1985
Hutchinson-Gilford progeria syndrome is a rare genetic disease of unknown etiology with some features suggestive of accelerated aging. Patients with this disorder are usually of average intelligence and appear clinically normal at birth. However, in early childhood they develop severe growth retardation and a senescent phenotype, including balding, aged-appe-aring skin, generalized atherosclerosis, and strokes. They die prematurely as a result...
One Case of Pendred Syndrome.
D.H. Song, C.S. Son, J.W. Lee, S.K. Kim
Clin Exp Pediatr. 1985;28(4):400-404.   Published online April 30, 1985
We presented one case of Pendred syndrome who had characteristic sporadic goiter and deafmutism, in 12 years old boy. A review of related literatures was made.
Adrenaogenital Syndrome with Congenital Adrenal Hyperplasia.
Kyeong Rae Moon, Yeong Bong Park, Sang Gi Kim, Jin Heon Kim, Chang Soo Ra, Jae Hong Seo, Ho Won Hwang
Clin Exp Pediatr. 1985;28(1):78-84.   Published online January 31, 1985
Adrenogenital syndrome is caused by a defect in biosynthesis of adrenal corticoids as a result of deficiency in one of the essential enzymes, most commonly 21-hydroxylase. About 50% of 21-hydroxylase deficiency have salt losing type as a result of aldosterone deficiency. We have experienced a casa of adrenogenital syndrome associated with congenital adrenal hyperplasia (salt losing form) in a 3...
Original Article
Spontaneous Pneumothorax in the Newborn.
Dong Gwan Han
Clin Exp Pediatr. 1985;28(1):16-24.   Published online January 31, 1985
A retrospective 13 medical records of symptomatic and spontaneous pneumothorax of newborn has been reviewed, which occurred during last 13 years from Jan. 1971 through Dec. 1983 at Pediatric Department of Yonsei Medical School. Spontaneous pneumothorax was defined one with pneumothorax which was not given positive pressure or intubation. And following results W6re obtained; 1)The incidence of spontaneous pneumothorax in...
Case Report
A Case of Rubinstein-Taybi Syndrome.
Won Hee Baik, Mee Ran Roh, Young Chang Kim, Hyung Jin Choi, Sang Jhoo Lee
Clin Exp Pediatr. 1984;27(12):1244-1249.   Published online December 31, 1984
Rubinstein-Taybi syndrome is a new complex of physical findings consisting primarily of broad thumbs, broad great toes and facial abnormalities, associated with mental retardation. We have experienced a case of Rubinstein-Taybi syndrome in a 5month-old male infant who had two recurrent pneumonia a竹acks. The diagnosis was easily established by clinical features and radiologic examinations. So a brief review of the pertinent literatures is given.
A Case of Acute Febrile Mucocutaneous Lymph Node Syndrom Complicated with Ileus.
Yang Ho Park, Jin Keun Chang, Sung Sook Cho, Keun Chan Sohn
Clin Exp Pediatr. 1984;27(12):1218-1222.   Published online December 31, 1984
We experienced a case of acute febrile mucocutaneous lymph node syndrome accompanied by ileus in a 2 10/12-year-old boy. His symptoms and signs sufficed the diagnostic criteria of acute febrile mucocutaneous lymph node syndrome, and included two peaks of fever and ileus. Related literatures were reviewed briefly.
Original Article
A Clinical Study of Reye`s Syndrome.
Young Seo Park, Hwan Jong Lee, Sang Pok Suk, Jeong Kee Seo, Kwang Wook Ko
Clin Exp Pediatr. 1984;27(11):1088-1096.   Published online November 30, 1984
Seventy-four cases of Reye’s syndrome, diagnosed during the period of 1978-1983 at the Pediatric ward of Seoul National University Hospital, were studied on the clinical features and laboratory findings. The following results were obtained; 1) The annual incidence was increasing year by year and the monthly incidence was highest in September. 2) The sex ratio of male to female was 1.2:1. The highest incidence was showed between...
A Clinical Observation of Meconium Aspiration Syndrome.
Chul Lee, Kook In Park, Jean Young Lee, Dong Gwan Han
Clin Exp Pediatr. 1984;27(10):958-964.   Published online October 31, 1984
The Authors observed 51 cases of meconium aspiration syndrome in newborns who were born at Severance Hospital during the period of Jan. 1980 through Jun. 1983. The following results were obtained: 1) The incidencs of meconium aspiration syndrome was 0.6%(51 cases). 2)88.2% of newborn with meconium aspiration syndrome was weighing more than 2,500 gm and 93.1% of that was with a...
Case Report
Four Cases of Gianotti-Crosti Syndrome.
Yong Gab Kwon, Ki Sup Chung, Kir Yong Kim
Clin Exp Pediatr. 1984;27(8):820-826.   Published online August 31, 1984
The Gianotti-Crosti syndrome, papular acrodermatitis of childhood(PAC), is an infrequently recognized disorder with distinctive characteristics. At present hepatitis B virus is thought to be an etiologic gent. The disease is very rare in Korea, in spite of the high frequency of hepatitis B surface antigen(HBsAg) in the general population. It is known that subtype ayw of the HBsAg may influence...
A Case of Congenital Renal Anomalies in Tuner's Syndrome.
Jeong Bok Lee, Sook Kyeung Jung, Dong Hwan Cho, Won Jae Park, Dong Sun Park
Clin Exp Pediatr. 1984;27(6):616-620.   Published online June 30, 1984
We have experienced a case of Turner’s Syndrome with hydronephrosis and partial duplication of renal pelvis in 71 days old female. The patient manifested the clinical finding of loose skin fold at the nape and palpable mass in the left flank. The chromosomal analysis was 45, XO. She was shown by operation to be a left hydronephrosis, shown by intravenous...
Adrenogenital Syndrome with Congenital Adrenal Hyperplasia.
Myoung Sung Moon, Kwang Nam Kim, Woo Gill Lee
Clin Exp Pediatr. 1984;27(5):511-515.   Published online May 31, 1984
When the adrenogenital syndrome is associated with congenital adrenal hyperplasia, it is caused by an inborn defect in the biosynthesis of adrenal corticoids. Especially, infant with congenital adrenal hyperplasia caused by a 21-hydroxylation. defect in steroidogenesis often present with salt-losing crisis, hyponatremia and dehydration. Vomiting is prominent and disturbances in cardiac rate and rhythm may occur. Without treatment, collapse and death...
Rifampin Induced Nonresponsiveness to Steroid Therapy in Children with Minimal Change Nephrotic Syndrome .
Hae Il Cheong, Sang Bok Suk, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1984;27(5):506-510.   Published online May 31, 1984
We experienced three children with the biopsy proven minimal change nephrotic syndrome who had failed to respond to prednisolone, 60 mg/m2/d for 4 to 9 weeks due to concomittent administration of rifampin. Because rifampin is a known enzyme inducer of the liver like phenobarbital and dilantin which can increase the clearance of the other drugs such as steroid etc., rifampin were...
A Case of Pierre Robin Syndrome.
Hyun Hwa Kim, Hae Sook Cha, Byoung Hai Ahn, Young Hee You, Hyun Sook Lee
Clin Exp Pediatr. 1984;27(5):488-492.   Published online May 31, 1984
A case ef Pierre Robin Syndrome in a male baby who was born at the Korea Veterans Hospital was presented. After birth, dyspnea and feeding difficulty were developed. Physical examination revealed micrognathia, glossoptosis and cleft plate. Chromosomal analysis was normal pattern. This finding was compatible with Pierre Robin Syndrome. A brief review of literature was presented.
Original Article
Reye's Syndrome in Children.
Dong Jin Lee, Sook Hee Lee, Ja Hoon Koo, Doo Hong Ahn, Yong Jin Kim
Clin Exp Pediatr. 1984;27(5):457-466.   Published online May 31, 1984
A clinical observation has been made on 44 children with Reys's syndrome who were admitted to pediatric department of KNU hospital during 6 year period from September 1977 to August 1983. Male to female ratio was 1.3 : 1. Cases under 6 years of age comprised 40(90.9%) and cases under 1 year 13(29.6%). The most common antecedent illness was URI...
Case Report
Adrenal Cortical Carcinoma in a Nineteen Month Old Girl.
Sei Weon Yang, Jong Jin Seo, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(4):413-418.   Published online April 30, 1984
Adrenal carcinoma is a rare disease both in adults and in children. Most of these tumors are functional, especially in children, producing endocrine syndromes such as Cushing syndrome, virilization, feminization or precocious puberty. The authors report a nineteen month old girl who exhibited features of Cushing syndrome and virilization caused by adrenal cortical carcinoma measuring 5.5X5.0X2.8 cm. She was successfully...
Original Article
Two Cases of Hemolytic Uremic Syndrome.
Sook Hee Lee, Dong Jin Lee, Ja Hoon Koo, Doo Hong Ahn, Young Jin Kim
Clin Exp Pediatr. 1984;27(4):384-389.   Published online April 30, 1984
The hemolytic uremic syndrome is a clinical state characterized by uremia, thrombocytopenia and microangiopathic hemolytic anemia. Since the first description of this illness by Gasser in 1955, it has been recognized with increasing frequency. Recently, we have experienced 2 cases of severe hemolytic uremic syndrome occurring in boys in 5 and 6 years of age. Diagnosis was established by typical...
Case Report
Acute Tumor Lysis Syndrome.
Sun Oak Park, In Sil Lee, Hyp Seop Ahn, Yong Choi
Clin Exp Pediatr. 1984;27(3):303-312.   Published online March 31, 1984
To assess the incidence and the effectiveness of preventive measures for tumor lysis syndrome, we analyzed the serial electrolytes levels in leukemia (Group A, 15 patients) and in lymphoma (Group B, 8 patients) patients. The results were 1)Serum LDH were elevated in all of the patients before the chemotherapy. 2)Serum uric acid were also elevated in 8 patients (A 5, B...
Original Article
Clinical Observation about complications(Especially about Mortality Cases) in Childhood Primary Nephrotic Syndrome.
Hae ll Cheong, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1984;27(3):246-252.   Published online March 31, 1984
Among 350 children who had been admitted to the Ward of Pediatrics, Seoul National University Hospital during the period from 1975.1.1. to 1983.12.31. and had been diagnosed as idiopathic nephrotic syndrome, total 21 cases (6%) had expired or had progressed into end stage renal disease during follow-up. Among these, 13 cases(61.9%； Group A) had expired due to chronic renal failure...
Case Report
A Case of Treacher Collins Syndrome.
Hee Shang Youn, Koo Soo Kim, Hyung Ro Moon
Clin Exp Pediatr. 1984;27(2):207-212.   Published online February 28, 1984
The authors report a 5-year old boy who showed the abnormalities comprising relatively complete from of Treacher Colilns syndrome. These include hypoplasia of malar and mandibular bones, antimongoloid slant of palpebral fissures, colobomata of lower eyelids with defective eyelashes on medial sides, projection of scalp hair onto lateral cheeks, malformed auricles, complete atresia of both external auditory canals with defective...
A Case of Mucocutaneous Lymph Node Syndrome complicated by Coronary Aneurysm.
Phil Joun Song, Chung Hye Chu, Kyoo Hwan Rhee, Yong Mook Choi, Chang Il Ahn
Clin Exp Pediatr. 1984;27(2):197-201.   Published online February 28, 1984
We have recently experienced a case of mucocutaneous lymph node syndrome complicated by coronary aneurysm. He was suspected by two-dimensional echocardiographic examination and confirmed by the angiography of aorta. A brief review of related literatures was made.
2 Cases of Congenital Asplenia Syndrome.
Hyo Sup Joo, Chung Hye Chu, Byoung Soo Cho, Kyoo Hwan Rhee, Sa Jun Chung, Chang Il Ahn
Clin Exp Pediatr. 1984;27(2):191-196.   Published online February 28, 1984
The asplenia syndrome is rare, characterized by congenital abscence of spleen, cardiovascular anomalies and other malformations or malpositions such as total or partial situs inversus, symmetrical lobulation of the liver and/or lung. Associated multiple complex cardiovascular anomalies include common atrioventricular canal, pulmonary stenosis or atresia, anomalous venous drainage and transposition of great vessels. Two-dimensional echocardiography allows the accurate non invasive...
Original Article
Clinical Significance of Selectivity Index in Nephrotic Syndrome.
Hoan Jong Lee, Hee Jin Kim, Yong Choi, Kwang Wook Ko
Clin Exp Pediatr. 1984;27(1):68-73.   Published online January 31, 1984
Slectivity I nd ices (CIgG/Ctransf erri n) of 114 nephrotic children, who were admitted to the Pediatric ward of SNUH from January, 1979 to December 1982, were, analyzed in relation to pathology, response to steroid treatment and the variability in the individual patients. In Summary 1)83% of MPGN, and 74% of FSGS showed S.I of more than 0.2 while 36%...
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