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Original Article
A Clinicopathological Study on Re nal Biopsies in Children.
Soon Wha Kim, Keun Chan Sohn
Clin Exp Pediatr. 1986;29(8):868-879.   Published online August 31, 1986
A clinical and histopathologic study was made on 80 cases of renal biopsies in children who were admitted to National Medical Center from January 1976 to June 1985. The results are as follows: 1) Among 80 cases, 60 cases were male and 20 cases female. The sex ratio of male to female was 3 : 1. The age distribution was from...
Case Report
Six Cases of Edward Syndrome.
Hye Yun Kang, Hee Bong Park, Mi Na Lee
Clin Exp Pediatr. 1986;29(7):797-802.   Published online July 31, 1986
Six cases of Edward syndrome who were born at Cheil Hospital was presented. These babies had grossly multiple anomalies that were characterized by promint occiput, micrognathia, small palpebral fissures, small oral opening, narrow high palatal arch, low set ear, overlapping fingers, prominent labia minora and majora, narrow pelvis, and Rocker bottom feet. We have been confirmed by chromosome culture and their chromosomal analysis...
Adrenal Cortical Adenoma Associated with Spinal Epidural Lipomatosis and Paraplegia.
Il Whan Kim, Chul Hoe Koo, Jae Sun Park, Sung Uhn Baek, Sung Do Lee, Kyu Ho Lee, Jae Do Kim, Bang Hur, In Sun Jun
Clin Exp Pediatr. 1986;29(7):784-790.   Published online July 31, 1986
A 14 year old girl was admitted because of severe obesity, respiratory difficulty and weakness of legs with edema. Diagnosis of Cushing* s syndrome by left adrenal adenoma was made by hormonal assay, abdominal C-T and surgery. 1 week after adrenalectomy, she developed paraplegia which was caused by massive deposition of fat in the spinal epidural space and compression of thoracic spinal cord. She was...
Original Article
Russell-Silver Syndrome.
Sei Won Yang, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(7):715-722.   Published online July 31, 1986
Seventeen cases(12 boys and 4 girls) with Russell-Silver syndrome were evaluated for weight, height and bone age at initial presentation and during follow up. 1) Distribution of patient` s age at initial presentation was between 1 day and 75/12 year of age. 2) In 14 cases, the birth weight was below 3 percentile except for one case(3 percentile). In 15 cases, the...
Case Report
A case of Hutchinson Gilford Progeria Syndrome.
Myung Ho Cho, Yong Woo Choi, Wan Seob Kim, Oh Kyung Lee, Myung Ho Lee
Clin Exp Pediatr. 1986;29(5):570-574.   Published online May 31, 1986
An extremely rare syndrome originally described by Hutchinson in 1886 and Gilford in 1904 is associated with a characteristic aged appearance very early in life. Although numerous clinical. metabolic and pathologic studies had been carried out and several theories advanced , the cause and pathogenesis of progeria remain obscure. The patient of this syndrome develope widespread atherosclerosis and often...
A Case of the Dubowitz Syndrome.
Kyung Ae Lee, Chi Ho Yun, Jae Sun Jung, Hee Ju Kim, Sung Ill Ahn, Hyung Ro Moon
Clin Exp Pediatr. 1986;29(4):436-440.   Published online April 30, 1986
The Dubowitz syndrome is an autosomal recessive condition characterized by intrauterine growth retardation, postnanal growth retardation, peculiar facial appearance, short stature, low birth weight, microcephaly and borderline intelligence or mild mental retardation. We reported a typical case of the Dubowitz syndrome in a full term male infant together with a brief review of pertinent literatures since the incidence of the...
Original Article
Superoxide Dismutase Activity in Down Syndrom.
Moon Sook Lee, Hae Woon Chang, Kun Soo Lee, Ja Hoon Koo, Kun Young Sohn
Clin Exp Pediatr. 1986;29(4):361-374.   Published online April 30, 1986
Superoxide dismutase activity of erythrocyte in 20 cases of 21-trisomy Down syndrome between 2 months and 15 years old age and 13 cases of age-matched normal children were checked at Department of Pediatrics and Department of Biochemistry, School of Medicine, Kyung-Pook National University. The results were following: Mean superoxide dismutase activity in normal children was 202.25 U/gmHb and in Down syndrome 291.69 U/gmHb, and the ratio...
Case Report
A Case of Silver Russel Syndrome.
Kee Hyuck Kim, Chang Jun Coe, Duk Jin Yun
Clin Exp Pediatr. 1986;29(3):318-321.   Published online March 31, 1986
Recently, we have experienced a case of Silver-Russel syndrome, representing short sature, asymmetric skeletal development causing hemihypertrophy, macrocephaly and clinodactily. We are reporting this case with brief review of literatures.
Two Cases of Patau Syndrome.
Eun Hee Cho, Byung Ho Lim, Ki Bok Kim
Clin Exp Pediatr. 1986;29(2):225-231.   Published online February 28, 1986
Two cases of Patau syndrome which had been confirmed by cytogenetic analysis at Kwangju Christian Hospital was presented. Two babies were low-birth-weight infants born to mothers aged 30 and 27, respectively. No history of drugs, nor any exposure to radiation or other teratogenic agents were recalled by their mothers. No familial history of congenital anomalies were noted. They expired immediately after birth. On...
Original Article
A Study on Changes of Serum Prootein, Immunoglobulins, and Electrolyte Metabolism in Childhood Nephrotic Syndrome after Steroid Therapy.
Kyung Rae Moon, Chang Soo Ra
Clin Exp Pediatr. 1986;29(1):32-44.   Published online January 31, 1986
The author determined serum and 24hr urine protein(total protein and albumin), immunoglobulins(lgG, IgA, and IgM) and serum electrolytes(Na, K, Ca, P, Mg, and Cl) in 5 cases of childhood nephrotic syndrome before and after steroid therapy, until urinary excretion of protein was negative (remission).The results observed are summarized as follows; 1) Serum protein; Before treatment, the mean serum total protein...
Case Report
Myelodysplastic syndrome Transformed into Erythroleukemia.
Sun Hee Kim, Don Hee Ahn, Keun Chan Sohn, Jung Il Suh, Jung Hee Um, Hea Ran Park
Clin Exp Pediatr. 1985;28(12):1231-1237.   Published online December 31, 1985
Myelodysplastic syndrome is a disorder of the marrow stem cell characterized initially by ineffective erythropoiesis, an abnormal bone marrow, and peripheral blood cytopjenia. Recently MDS was classified into 5 subtypes by the F rench-American-British (FAB) Cooperative Group: (1) Refractory anemia(R.A.), (2) RA with ring sideroblast, (3) RA with excess of blast(RAEB), (4) Chronic myelomonocytic leukemia (CMML) and (5) RAEB in transformation. We...
Original Article
A Clinical Study on Reye Syndrome.
Yong Hae Lee, Jong Chul Yu, Jun Taek Park, Chang Hee Choi
Clin Exp Pediatr. 1985;28(11):1089-1096.   Published online November 30, 1985
Fifty-five cases of Reye’s syndrome, diagnosed during the period of October 1 st, 1980-September 31st, 1984 at the Pediatric of Seoul Adventist Hospital, were studied on the clinical features and laboratory finding. The following results were obtained; 1)There was no much indifference in annual incidence but highest incidence was showed September by 9 cases (16.4%). 2)The sex ratio of male to...
Case Report
Two Case of Watson-Alagille Syndrome.
Heon Seok Han, Soo Heum Lim, Young Sun Kim, Jeong Kee Seo, Hyung Ro Moon
Clin Exp Pediatr. 1985;28(10):1047-1051.   Published online October 31, 1985
Watson-Alagille syndrome (Arteriohepatic dysplasia, Syndromatic paucity of interlobular bile duct), a disease characterized by chronic liver disease, characteristic facies, cardiova scular abnormalities, and vertebral arch abnormalities has paucity of interlobular bile ducts on histologic examination of liver. We experienced two cases of Watson-Alagille syndrome in a 7-month-old male infant and a 12-month-old male infant who had the chief complaint of...
A Case of 4p-(Wolf-Hirschhorn) Syndrome.
Young Hun Jeon, Toung Ill Park
Clin Exp Pediatr. 1985;28(10):1042-1046.   Published online October 31, 1985
A case of 4p-syndrome in female newborn was presented. She had multiple anomalies such as craniofacial asymmetry, microcephaly, prominant glabella, hypertelorism, ocular anomalies, preauricular skin tag and sinus, malformed ear, beaked nose, cleft lip and palate, ASD and so on. Chromosomal study showed 46, XX, del(4) (P 13.) A brief review of literature was also presented.
A Case of Pierre Robin Syndrome.
Eung Deok Choi, Ae Sook Kim, Kwan Hwooy Cho, Jong Soo Kim
Clin Exp Pediatr. 1985;28(10):1023-1026.   Published online October 31, 1985
We experienced a case of Pierre Robin syndrome in newborn consisted of micrognathia, cleft palate and glossoptosis. It may accompany with cardiac malformations and ocular anormalies, which were not found in this baby. Brief review of literature was made.
Superior Mesenteric Artery Syndrome.
J H Kim, Y B Shin, H S Song, I S Choi, H S Lee, J S Kim
Clin Exp Pediatr. 1985;28(10):1017-1022.   Published online October 31, 1985
Vomiting, indigestion and recurrent epigastric pain are common in pediatric practice. Inspite of extensive clinical and laboratory investigation, definite cause can not be identified frequently, And furthermore many of those are considered as neurotic. The purpose of this report is to call attention to this syndrome as a cause of abdominal pain and to discuss its pathogenesis and treatment.
A case of Congenital Nephrotic Syndrome.
Kwang Nam Kim, Kwang Hyun Kim, In Joo Seol, Ha Baik Lee, Chong Moo Park
Clin Exp Pediatr. 1985;28(9):931-935.   Published online September 30, 1985
The congenital nephrotic syndrome is characterized by large placenta, low birth weight, early clinical findings, delayed growth and development and resistance to treatment. Authors experienced a case of characteristic of congenital nephrotic syndrome in a girl, who was admitted to the Pediatric Department of HUH at 28 days of age because of generalized edema, abdominal distension with vomiting and diarrhea....
A case of hypereosinophilic syndrome.
Sung Hun Eun, Joo Won Lee, Se Jin Kang, Soon Kyum Kim
Clin Exp Pediatr. 1985;28(9):922-925.   Published online September 30, 1985
Hypereosinophilic syndrome is a disorder characterized by peripheral blood & bone marrow eosinophilia associated with single or multiple organ system dysfunctions. The diagnostic criteria includes a peripheral blood eosinophilia of 1,500 eosinophils/cu mm for 6 Months or longer, signs and symtoms of organ involvement, and a lack of evidence for parasitic, allergic, or other known causes of eosinophilia. We have...
Original Article
The clinical Study on Mucocutaneous Lymphnode Syndrome.
H D Cha, B Ahn, S H Kim, T C Kwon, C M Knag
Clin Exp Pediatr. 1985;28(9):861-868.   Published online September 30, 1985
Mucocutaneous lymphnode syndrome (Kawasaki Disease) is a newly described acute symptom complex of children that was first recognized and described by Dr. Tomisuku Kawasaki in Japan. This syndrome recently was reported from various areas other than Japan, mainly U.S.A and Korea. Authors observed the clinical and epidemiological features on 68 cases of mucocutaneous lymphnode syndrome which were admitted to Pediatric...
Case Report
Three Cases of Congenital Hypoplastic Anemia.
Heui Jeong Kwon, Myung Hyun Lee, Jung Hwan Choi, Hyo Seop Ahn, Chang Yee Hong
Clin Exp Pediatr. 1985;28(8):829-835.   Published online August 31, 1985
Congenital hypoplastic anemia is a rare disease characterized by aplasia or severe hypoplasia of the erythron, with onset usually at birth or during early infancy. This report presented three cases of congenital hypoplastic anemia in 3 infants with pallor and irritability which were noticed since one to three months of age. Three cases were treatsd with prednisolone and the courses of...
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.
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