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Original Article
Observation on neurocysticercosis in childhood.
Hae Jung Park, Seong Hee Jang, Se Hee Hwang, Jae Il Sohn, Yong Seung Hwang, Kyu Chang Wang, Byug Kyu Cho, In One Kim, Kyung Mo Yeun, Je Geun Chi, Seung Yull Cho
Clin Exp Pediatr. 1991;34(6):877-889.   Published online June 30, 1991
A clinical study was performed on 5 patients of neurocysticercosis in childhood who admitted to the Seoul National University Hospital for the last two years. The conclusions were as follows: 1) Boys were more than girls with the ratio of 4:1. The average age was 9.5. 2) Three out of five lived in Jeju Island. Three had familial history of taeniasis. 3) Three out of five were...
A case of 4p- syndrome with oligomeganephronia.
Ri Sa Lee, Bong Sik Kong, Beyong Il Kim, Sang Kyu Park, Ho Jin Park, Soong Deok Lee, Je Geun Chi
Clin Exp Pediatr. 1991;34(4):558-565.   Published online April 30, 1991
The 4p_ syndrome results from structural deficiency of chromosome 4. We experienced a patient of 4p~ syndrome who all features of already described Wolf syndrome, i.e., prominent glabella, hypertelorism, broad beak nose, cleft lip and palate, kyphoscoliosis, hypospadia, etc.. Postmortem examination revealed multiple visceral anomalies, including large atrial septal defect, diaphragmatic eventration, intestinal malrotation, ankyloglossia, and hemivertebrae. The kidneys showed a marked simple hypoplasia weighing...
A case of Caroli's disease.
Seong Hee Jung, Cheol Ho Chang, Han Tchah, Jeong Kee Seo, Kwi Won Park, In One Kim, Kyung Mo Yeon, Je Geun Chi
Clin Exp Pediatr. 1991;34(2):261-266.   Published online February 28, 1991
Caroli’s disease was first described by Caroli in 1958 and characterized by segmental cystic dilatation of the intrahepatic bile ducts. There is stasis of bile with stone formation in the dilatated ducts, giving rise to frequent attacks of pain and cholangitis. In the past, diagnosis of the Caroli’s disease is rarely established prior to operation and subsequent cholangiography. Recently, with ultrasonography, computerized tomography, radioisotope...
A Case of MELAS Syndrome.
Ki Joong Kim, Yong Seung Hwang, Young In Choi, Sung Hye Park, Je Geun Chi
Clin Exp Pediatr. 1990;33(11):1586-1592.   Published online November 30, 1990
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode) syndrome is a rare but clinicopathologically distinctive mitochondrial disorder. We experienced a case of MELAS syndrnmp in A 11 year-old girl who showed repeated attacks of stroke-like episodes, vomitng, cortical blindness, hearing deficit and alternating hemiplegia. Serum lactic acid level was elevated (6.4 mmol/Z). The diagnosis was confirmed by muscle biopsy which revealed dispersed ragged-red fibers.
A Case of Neuroepithelial(Colloid) Cyst.
Min Sik Kim, Yo Han Kim, Mu Young Song, Soon Jai Lee, Young Bae Lee, Je Geun Chi
Clin Exp Pediatr. 1990;33(5):695-700.   Published online May 31, 1990
The authors experienced a case of neuroepithelial cyst which was found deep inside the brain, involving the ventricular septum. A 2 months old female patient was presented with convulsion. Characteristic CT findings showing multiloculated cystic structures were seen. Operation was carried out to confirm multiple intraventricular cysts containing yellowish fluid. Histologically the cyst wall consisted of simple cuboidal epithelium and fibrous connective tissue. A brief...
A case of Second Malignant Neoplasm Complicating Hodgkin's Disease in Remission.
Hong Hoe Koo, Jong Woon Choi, Sang Oh Na, Il Soo Ha, Hee Young Shin, Hyo Seop Ahn, Yeon Lim Suh, Chul Woo Kim, Je Geun Chi
Clin Exp Pediatr. 1990;33(4):564-572.   Published online April 30, 1990
Recent advances in the treatment of cancer have resulted in increased survival and possible cure for many malignant disorders, particularly childhood cancers. There has been a marked improvement in the treatment of childhood Hodgkin’s disease, and five-year survival rates for even advanced stages of Hodgkin’s disease approach or exceed more than 75% with modern treatment policies. However, successful therapy in childhood cancer is frequently...
A Case of Retroperitoneal Lymphangioma.
Gyu Jin Oh, Jin Hyeon Park, Hee Jung Kwon, In Sil Lee, Kui Won Park, Je Geun Chi
Clin Exp Pediatr. 1990;33(3):422-428.   Published online March 31, 1990
Cystic lymphangioma, or cystic hygroma, is a true, benign, congenital multic-ystic tumor arising from sequestration of embryonic lymphatic tissue. Most often it occurs at birth or early in life, and it was found most commonly in the neck, but rarely in the retroperitoneum and its distribution coincides with that of the primitive lymph sac. We have experienced a case of retroperitoneal cystic lymphangioma in...
A Case of Transient Myeloproliferative Disorder in Down's Syndrome.
Ji Hye Kang, Young Mi Hong, Kyung Hee Kim, Seung Joo Lee, Ki Sook Hong, Ok Kyung Kim, Je Geun Chi
Clin Exp Pediatr. 1990;33(2):252-258.   Published online February 28, 1990
Transient myeloproliferative disorder has been reported in infant with Down's syndrome during neonatal period. This is clinically and hematologically indistinguishable from congenital acute leukemia. In contrast to congenital leukemia, complete clinical and hematological recovery occurs within weeks to months of diagnosis without antileukemic treatment. A neonate presented with jaundice, hepatosplenomegaly and mongoloid face was diagnosed as Transient myeloproliferative disorder. We report this case with a review of...
A Case of Mucinous Cystadenocarcinoma in a Premenarchal Girl.
Hong Hoe Koo, Sang Oh Na, In Sang Jeon, Hyo Seop Ahn, Wan Suk Park, Suk Koo Lee, Kwi Won Park, Chong Jai Kim, Je Geun Chi
Clin Exp Pediatr. 1990;33(1):124-128.   Published online January 31, 1990
In childhood, cancer of the ovary is highly unusual. It is responsible for only 1—2% of cancers found in patients under the age of 17. The frequency with which the various histologic types of ovarian neoplasm occur in childhood differs from that in adults. Epithelial tumors account for 70% to 80% of ovarian neoplasms in adults but only 20% in patients less than...
Congenital Systemic Cytomegalic Inclusion Disease.
Dong Beom Lee, Dong Hyun Kim, Jung Sik Min, Chang Hee Choi, Je Geun Chi
Clin Exp Pediatr. 1990;33(1):100-106.   Published online January 31, 1990
The authors experienced a case of congenital systemic cytomegalic inclusion disease in a newborn baby. This case showed the clinical features of low birt wight (2,200 gm), numerous petechiae, severe jaundice, hepatosplenomegaly, hypotonia and respiratory difficulty with cyanosis and Apgar score of 3 at 1 minute at birth. In the clinical course, he showed hypotonia, repeated spell of apnea. These symptoms progressively worsened and he...
A Case of Mixed Gonadal Dysgenesis.
Byung Ran Yun, Jae Il Sohn, Sei Won Yang, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1989;32(12):1757-1762.   Published online December 31, 1989
Mixed gonadal dysenesis is a disorder of sexual differentiation which is characterized by streak gonad associated with contralateral tesis, persistent Mullerian duct structures and/or sex chromosomal mosaicism most commonly with 45, X/46, XY. The authors experienced a case of mixed gonadal dysgensis in a 6-year old child who was reared as female and admitted due to clitoral enlargement. The patient had asymmetrical gonads consisted with...
A case of hypertophic cardiomyopathy in newborn infant: An autopsy case.
Min Shik Kim, Eun Yong Choi, Hey Sun Lee, Young Min Ahn, Je Geun Chi
Clin Exp Pediatr. 1989;32(11):1560-1567.   Published online November 30, 1989
Hypertrophic cardiomyopathy is defined as a disorder of heart muscle of unknown cause or association, and is functionally characterized by a diastolic failure of the left ventricle due to loss of its normal distensibility. We subsequently experienced an 18 day old female newborn infant who had suffered from dyspnea, cyanosis and congestive heart failure since a few days after birth. She died of progressive...
Angiolymphoid Hyperplasia with Eosinophilial: Kiruma's disease.
Choong Won Lee, Young Sik Kim, Soon Ki Kim, Il Soo Ha, Hyo Seop Ahn, Je Geun Chi, Il Han Kim
Clin Exp Pediatr. 1989;32(10):1416-1421.   Published online October 31, 1989
Kimura’s disease is a rare, benign disease characterized by single or multiple subcutaneous nodules, eosinophilia and distinctive histopathologic features. Seven cases of Kimura’s disease under 15 yeaps of age who were admitted to the Dept, of Pediatrics, Seoul National University Hospital from Apr. 1983 to July 1987 were reviewed. The results were as follows: 1) The lesions were noted between the ages of 28 months and...
Juvenile Nephronophyjisis in An Infant.
Young Seo Park, Chang Youn Lee, Hae Il Cheong, Yong Choi, Kwang Wook Ko, Je Geun Chi, Chang Bin Im
Clin Exp Pediatr. 1989;32(8):1155-1160.   Published online August 31, 1989
Juvenile nephronophthisis is a chronic tubulo-interstitial renal disease characterized by an early onset of polyuria and polydipsia, decreased urinary concentrating ability, anemia, growth retardation and progressive renal disease. It is recognized as an important cause of chronic renal failure in the childhood. However only one case was reported in the Korean literatures. In this paper we have reported the second documented case of juvenile...
Alobar Holotelencephaly Associated with Microphthalmia and Choanal Atresia.
Hyon Sook Seo, Gun Tae Yi, Jung Hye Choi, Fan Chen Mong, Hee Dae Park, Soo Nam Rhee, Je Geun Chi
Clin Exp Pediatr. 1989;32(7):1007-1011.   Published online July 31, 1989
Holotelencephaly is a congenital malformation of the telencephalon which is characterized by developmental failure of the cerebral hemispheres and frequently associated with midline facial defect. We experienced a case of holotelencephaly, alobar type, associated with microphthalmia and choanal atresia. Diagnosis was confirmed by autopsy. A brief review of literature was made.
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 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...
Two Cases of Idiopathic pulmonary Hemosiderosis.
Sung Ky You, Ki Soo Kim, Young Jee Kim, Young Yull Koh, Yeon Lim Suh, Je Geun Chi
Clin Exp Pediatr. 1988;31(9):1209-1216.   Published online September 30, 1988
Idiopathic pulmonary hemosiderosis is an uncommon disease of undetermined cause that is char- acterized by periodic episodes of cough, hemoptysis, widespread pulmonary infiltrates and hypo- chromic anemia. Two cases of idiopathic pulmonary hemosiderosis were diagnosed after open lung biopsy. It revealed hemosiderin and hemosiderin-laden macrophage. There were no specific causes for pulmo- nary hemorrhage. We report 2 cases of idiopathic pulmonary hemosiderosis with brief review...
A Case of Acute Megakaryoblastic Leukemia in Infant with Down's Syndrome.
Jung Soon Whang, Soon hee Kim, Moon Ja Kim, Keun Lee, Je Geun Chi
Clin Exp Pediatr. 1988;31(8):1064-1070.   Published online August 31, 1988
Acute megakaryoblastic leukemia is an uncommonly recognized disorder that is characterized by rapidly progressive proliferation of atypical megakaryocytes and their precursor cells, and fatal course. Abnormalities in chromosome 21 may have a more than random relationship to acute megakaryoblastic leukemia. The authors reports a case of acute megakaryoblastic leukemia in a 2 month old female patient who was admitted for evaluation of hepatosplenomgaly. Multiple organ infiltration...
A Case of Familial Dysautonomia.
Hong Jin Lee, Dong Kyu Jin, Sei Won Yang, Jeong Kee Seo, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1988;31(5):648-654.   Published online May 31, 1988
Few documented cases of Familial dysautonomia fulfilling current diagnostic criteria have been recognized in non-Jews especially in orientals. In our case diagnosis was established in 8 year old Korean girl. She fulfilled 8 out of 9 essential diagnostic criteria of Riley. It represents a report of this syndrome with achalasia and improved with modified Heller’s myotomy.
A Case of Pulmonary Candidiasis.
Hye Ran Byun, Kung Ha Ryu, Moon Ja Kim, Keun Lee, Yeon Lim Suh, Je Geun Chi
Clin Exp Pediatr. 1988;31(5):621-626.   Published online May 31, 1988
A case of fatal pulmonary candidiasis was diagnosed at autopsy of 5-month-old female infant. Patient had mild coughing, poor weight gain and marked hepatosplenomegaly. She had no sign of immune dysfunction but developed fever, wheezing and intermittent respiratory distress. Chest P-A showed infiltrations of multiple lobar pneumonia. Postmortem examination revealed the whitish necrotic mass like lesion with a feature of lobar pneumonic consolidation in...
Case Report
A Case of Gaucher's Disease.
Hyo Nam Cho, Myung Cheol Cho, Hyung Ro Moon, Je Geun Chi, Hyo Min Kim
Clin Exp Pediatr. 1987;30(7):784-790.   Published online July 31, 1987
We experienced a case of Gaucher’s disease of acute infantile type in a 12 month old male boy. The patient showed hepatosplenomegaly with anemia and thrombocytopenia, developmental delay and frequent infection. There were characteristic Gaucher’s cells in bone marrow aspiration and biopsy of liver, spleen, & lymph node. Splenectomy was done at 25 month old. He expired 3 days after splenectomy. Autopsy was done....
A Cases of Retroperitoneal Immature Teratoma(Grade III).
A Ellen Kim, Hee Ju Kim, Jae Sun Jung, Sung Ill Ahn, Hye Kyung Lee, Je Geun Chi
Clin Exp Pediatr. 1987;30(3):327-334.   Published online March 31, 1987
Je Geun Chi, M.D. Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea We herein presented a case of retroperitoneal immature teratoma in a 4 month-old boy who had a huge intra abdominal mass with hypertension, sweating and loose stool. The pre-op diagnosis as well as a post-op diagnosis was a intrarenal mass (R/0 Wilms' tumor) which was later confirmed to be a...
A Case of Potter Syndrome Type I.
Jong Cheol Ryu, Jae Kwang Hong, Jun Taek Park, Jung Sik Min, Chang Jee Choi, Je Geun Chi
Clin Exp Pediatr. 1986;29(10):1152-1156.   Published online October 31, 1986
We have experienced a case of Potter syndrome type I in a newborn infant who presented with severe respiratory distress soon after birth. The diagnosis was made with autopsy findings consisted of bilateral polycystic renal dysplasia, bilateral hypoplastic lung , facies renalis, fibrocystic change of liver, patent ductus arteriosus, patent foramen ovale, ureter and urinary bladder hypoplasia and calcaneovarus. Brief...
A Case of Subdiaphragmatic Total Anomalous Pulmonary Venous Return.
Ho Jin Lee, Sung Sik Lee, Soon Il Lee, Young Seok Lee, Je Geun Chi
Clin Exp Pediatr. 1986;29(7):791-796.   Published online July 31, 1986
One case of subdiaphragmatic total anomalous pulmonary venous return in a newborn male baby under the impression of respiratory distress syndrome was presented. Isolated form of subdiaphragmatic total anomalous pulmonary venous return was confirmed by autopsy. The pulmonary veins joined a common trunk which was connected to the hepatic vein below the diaphragm for the venous circulation. There were two associated small...
Hepatoblastoma with Rupture and Hemorrhage.
Kwang Wook Ko, Hyung Ro Moon, Je Geun Chi, Kwi Won Park
Clin Exp Pediatr. 1985;28(12):1261-1261.   Published online December 31, 1985
Asplenia Syndrome
Kwang Wook Ko, Je Geun Chi
Clin Exp Pediatr. 1985;28(11):1163-1163.   Published online November 30, 1985
Tracheal Foreign Body Associated with Esophageal Duplication Cyst.
Kwang Wook Ko, Je Geun Chi, Kwi Won Park
Clin Exp Pediatr. 1985;28(10):1052-1052.   Published online October 31, 1985
Papillary low grade carcinoma of pancreas.
Kwang Wook Ko, Je Geun Chi, G. W. Park
Clin Exp Pediatr. 1985;28(9):945-945.   Published online September 30, 1985
Juvenile Hyalin Fibromatosis.
Kwang Wook Ko, Je Geun Chi
Clin Exp Pediatr. 1985;28(8):841-841.   Published online August 31, 1985