- Original Article
- Sudden Unexpected Death in Infancy
(Analysis of 34 Cases Including 13 Autopsies)
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Yeo Ok Moon, Hee Kyoung Choi, Jeoung-A Her, Woo Jong Shin, Myoung-A Kim, Seong Yong Lee, Seong Hee Jang, Eun Sil Dong, Chong Jae Kim, Young Min Ahn, Je Geun Chi
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Clin Exp Pediatr. 2002;45(9):1065-1074. Published online September 15, 2002
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Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy.
Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical... |
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- Case Report
- A Case of Hereditary Motor and Sensory Neuropathy Type III
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Su Hyun Cho, Soo-Jin Kim, Young-Hoon Kim, Yun Tae Kim, Yoon-Kyung Lee, Dong-Un Kim, Seung-Hoon Han, Seung-Yun Chung, In-Goo Lee, Kyung-Tai Whang, Je Geun Chi
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Clin Exp Pediatr. 2001;44(9):1057-1061. Published online September 15, 2001
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Hereditary motor and sensory neuropathy type III, which is also known as Dejerine-Sottas disease,
is a severe demyelinating polyneuropathy which presents from birth or infancy, and is
sometimes presented as a hypotonic or floppy infant. The disease is inherited autosomal recessively
and includes clinical findings of generalized muscle weakness and atrophy, with the
greatest severity in distal limb muscles, areflexia, and sensory loss. The... |
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- A Case of Marden-Walker Syndrome
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Hyun Jong Cho, Yoon Kyung Lee, Dong Un Kim, Byung Joon Choi, Jin Tack Kim, Ik Jun Lee, Je Geun Chi
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Clin Exp Pediatr. 1999;42(10):1471-1474. Published online October 15, 1999
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- Case Report
- A Pediatric Case of MELAS Syndrome Associated with Insulin-dependent Diabetes Mellitus
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Kyung Ran Park, Hye Won Park, Tae Sung Ko, Hae Il Cheong, Sei Won Yang, Young Seung Hwang, In Won Kim, Je Geun Chi
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Clin Exp Pediatr. 1996;39(10):1461-1465. Published online October 15, 1996
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MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and sroke like episodes) is a
major subgroup of heterogeneous mitochondrial encephalopathy. Recent advances in molecular
genetics revealed specific mutations in the mitochondrial DNA causing MELAS. We described
clinical and molecular genetic findings of a 13-year-old boy with MELAS syndrome associated
IDDM(insulin dependent diabetes mellitus). For molecular genetic studies, DNAs from peripheral
blood nucleated cells were used. And the A→G... |
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- Isolated Unilateral Pulmonary Vein Atresia
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Yun Ae Jeon, Chung Il Noh, Ho Sung Kim, Jung Yun Choi, Yong Soo Yun, Woo Sun Kim, Je Geun Chi
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Clin Exp Pediatr. 1995;38(3):409-416. Published online March 15, 1995
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We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and... |
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- A Case of Chronic Pancreatitis Complicated by Hemobilia
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Sang Ook Nam, Jeong Kee Seo, Je Geun Chi, Kyung Mo Yeon
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Clin Exp Pediatr. 1995;38(3):422-427. Published online March 15, 1995
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Hemobilia caused by pancreatic disease is very rare. The most common cause is a splenic artery pseudoaneurysm caused by acute and chronic inflammation of the pancreas.
We experienced a case regarding as hemobilia as a complication of chronic relapsing pancreatitis in a 14 year-old boy. He was admitted with chief complaints of abdominal pain and hematemesis. Two years prior to admission,... |
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- Original Article
- Acute Hemorrhagic Cystitis(AHC) in Children -Etiology and Clinical Characteristics-
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Jin Won Pyo, Eun Hwa Choi, Jin Young Park, Hoan Jong Lee, Hae Il Cheong, Il Soo Ha, Yong Choi, Kwang Myung Kim, Hwang Choi, Je Geun Chi
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Clin Exp Pediatr. 1995;38(2):207-215. Published online February 15, 1995
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Purpose : AHC characterized by sudden onset of gross hematuria, dysuria and frequency oc curs in children and young adults as a self-limited disease that should be differentiated from se rious renal disorders. We have performed this study to establish the cause and characterize the clinical features of this illness in Korean children.
Methods : 19 cases collected prospectively for 30... |
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- Case Report
- A Case of \Intractable Ulcerating Enterocolitis\" of Infant"
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Ju Young Jeong, Jeong Kee Seo, Kwi Won Park, Je Geun Chi
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Clin Exp Pediatr. 1995;38(2):264-270. Published online February 15, 1995
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Intractable ulcerating enterocolitis of infancy is uncommon, inhereditary disease characterized by ulcerating stomatitis, severe perianal disease, affecting the whole gastrointestinal tract, mainly colon with flask shaped large ulcer. It was first described by Sanderson et al in 5 cases of infant with intractable diarrhea having above clinical manifestation. It should be differentiated with Crohn's disease and Behcet's disease.
We experienced a... |
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- A Case of Cardiac Tumor Associated with Tuberous Sclerosis in Newborn
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Yong Myung Cho, Woo Seok Kim, Nam Soo Park, Un Jun Hyoung, Eun Ryyoung Kim, Je Geun Chi
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Clin Exp Pediatr. 1995;38(2):275-279. Published online February 15, 1995
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Symptomatic primary heart tumors are rare in newborn. Cardiac rhabdomyoma is the most common lesion of the primary heart tumors and over half of them are related to kthe tuberous s clerosis. We described a case of cardiac rhabdomyoma, in a 2 day-old female newborn who presented with dyspnea and cyanosis. A 2D Echocardiogram demonstrated two masses; one is in... |
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- Original Article
- Cystic Kidney Disease According to Potter's Classification
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Kee Hyuck Kim, Sung Chul Shin, Soon Il Lee, Je Geun Chi
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Clin Exp Pediatr. 1995;38(1):91-98. Published online January 15, 1995
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The cystic disease of the kidney include a heterogeneous group of developmental, hereditary, and acquired disorders. Based on extensive microdissection studies, Potter concluded all renal cystic diseases could be categorized into four types.
We have experienced 5 cases of cystic kidney disease which were confirmed by aoutopsy and classified as Type I, Type II, Type III, Boderline between types II and... |
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- Case Report
- Candida Esophagitis in Infancy-A Report of 3 Cases
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Ho Sung Kim, Youn Woo Kim, Jae Geon Sim, Beom Soo Park, Hoan Jong Lee, Joong Gon Kim, Jeong Kee Seo, Je Geun Chi
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Clin Exp Pediatr. 1994;37(2):269-275. Published online February 15, 1994
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We experienced 3 cases of Candida esophagitis in infancy which were diagnosed by esophageal endoscopy, First case, 10 month-old boy with combined immune deficiency had suffered from oral thrush and poor feeding for more than 4 months. Esophageal endoscopy revealed multiple whitish creamy patches on the friable erythematous and necrotic mucosa of the esophagus, He was firstly treated with amphotericin-B... |
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- A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -
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Hong Shin Jeon, Young Jin Hong, Don Hee Ahn, Hee Jun Yoo, Je Geun Chi
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Clin Exp Pediatr. 1994;37(1):104-108. Published online January 15, 1994
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A female newborn had the following characteristics: a congenital localized absence of skin over the lower extremities; blistering of the skin or mucous membrane, incited by trauma, which heals without scarring; and congenital absence or deformity of the nails. In respect to the characteristic manifestationhj, clinical course and electron microscopic features, our patient seems to fit well into Bart's syndrome.... |
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- Recurrent Rhinocerebral Mucormycosis - A Case Report
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Ho Sung Kim, Jin Young Park, Bo Young Yun, Eun Sil Dong, Hee Young Shin, Hoan Jong Lee, Hyo Seop Ahn, Je Geun Chi, Myung Whun Sung
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Clin Exp Pediatr. 1993;36(4):589-595. Published online April 15, 1993
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A case of recurrent rhinocerebral mucormycosis that has occurred during an induction chemotherapy for acute megakaryocytic leukemia in a 10 year-old boy is reported. He had suffered from high fever, proptosis, right eye ball pain and necrotic inflammation of hard palate during the chemotherapy of leukemia. CT scan of the paranasal sinus showed inflammatory change of right ethmoid and maxillary... |
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- Original Article
- Chronic Idiopathic Intestinal pseudo-obstrucion Syndrome in Childhood
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Jae Geon Sim, Jeong Kee Seo, Kui Won Park, Je Geun Chi
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Clin Exp Pediatr. 1993;36(11):1583-1595. Published online November 15, 1993
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Chronic idiopathic intestinal pseudo-obstruction syndrome is a clinical condition induced by an impaired function of intestinal motility. Although its clinical symptoms are those of intestinal obstruction, mechanical obstruction of the intestine cannot be found by vigorous studies, even by operative exploration.
We have experienced nine cases of chronic idiopathic intestinal pseudo-obstruction syndrome. It will help in diagnosis and treatment of the... |
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- Case Report
- A Case of Roberts Syndrome
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Young Choi, Yo Han Chung, In Seok Lim, Chul Ha Kim, Dong Keun Lee, Seong Nam Kim, Sang Yong Song, Je Geun Chi
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Clin Exp Pediatr. 1993;36(10):1447-1451. Published online October 15, 1993
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Roberts syndrome is an autosomal recessive disorder accompanied by limb defects, craniofacial abnormalities, pre-and postnatal growth retardation. Patients with Roberts syndrome have characteristic premature separation of heterochromatin of many chromosomes and abnormalties in celldivision cycle.
We have experienced a case of Roberts syndrome in an immature neonate. The patients showed characteristic clinical features of multiple encephaloceles. Severe facial mid-line clefts, and... |
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- Three Cases of Post-transfusion Hepatitis C
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Kyung Un No, Ho Seong Kim, Ji Won Choi, Dong Wook Kim, Cheol Ho Jang, Beom Su Park, Jeong Kee Seo, Gyeong Hoon Kang, Je Geun Chi
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Clin Exp Pediatr. 1992;35(9):1255-1262. Published online September 15, 1992
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Hepatitis C virus has been known to be the main cause of post-transfusion non-A, non-B hepatitis. The authors experienced three cases of hepatitis C. All were associated with blood transfusions. They had no specific symptoms, and the levels of aminotransferases were markedly elevated and showed severe fluctuations in two cases. Anti-HCV tested two or three times were all positive, and... |
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- A Case of Kugelberg-Welander Syndrome
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Sun Young Lee, Myung Ik Lee, Soon Wha Kim, Don Hee Ahn, Je Geun Chi
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Clin Exp Pediatr. 1992;35(9):1263-1266. Published online September 15, 1992
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We have experienced a 6 year-old female patient who had suffered from progressive muscular atrophy with weakness and loss of voluntary motor action since 3 years of age. She was diagnosed as Kugelberg-Welander syndrome by EMG and muscle bio. Multiple polyphasic motor unit potentials were revealed on EMG and grouped muscle atrophy without fibrosis or degenerative change was found on... |
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- Acquired Persistent Cytomegalovirus Infection : An Association with Common Variable Immunodeficiency
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Min Hyea Kim, Young Mi Hong, Sung Joo Lee, Je Geun Chi, Dong Soo Kim
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Clin Exp Pediatr. 1992;35(9):1272-1279. Published online September 15, 1992
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Acquired persistent cytomegalovirus infection was diagnosed by anti CMV antibody and lymph node biopsy and persisted in a 3-year-old boy with recurrent episode of high fever, lymphadenopathy hepatosplenomegaly and pneumonia.
Initial immunologic abnormalities including low serum IgG, high IgM decreased T4 and T4/T8 ratio finally progressed to immunologic paralysis which was compatible to common variable immunodeficiency.
This is the first case which... |
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- Seven Cases of Immotile Cilia Syndrome
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Sun Young Lee, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn, Je Geun Chi
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Clin Exp Pediatr. 1992;35(8):1127-1134. Published online August 15, 1992
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Immotile cilia syndrome is a disease caused by congenital abnormality of the cilia and symptom complex composed by chronic rhinitis, sinusitis, otitis, male infertility etc.
We have experienced 7 cases of immotile cilia syndrome diagnosed by electron microscopic finding of nasal or bronchial mucosal biopsies since 1984. Six cases were girls and type Id was most common (4 cases) on... |
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- Transfusion-Acquired Cytomegalovirus Infections in Two Premature Infants
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Dong Wook Kim, Kyung Un No, Mi Jung Kim, Soon Mee Park, Hee Sup Kim, Young Pyo Chang, Hoan Jong Lee, Jung-Hwan Choi, Chong Ku Yun, Je Geun Chi
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Clin Exp Pediatr. 1992;35(8):1141-1147. Published online August 15, 1992
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Transfusion-acquired perinatal cytomegalovirus (CMV) infection can cause significant morbidity and mortality, particularly in premature infants with a birth wight of less than 1,500 gm. This recognizable syndrome consisted of deterioration of respiratory function, hepatosplenogaly, unusual gray pallor with disturbing septic appearance, lymphocytosis, thrombocytopenia and hemolytic anemia.
We experienced transfusion-acquired CMV infections in 2 premature infants with a birth weight of... |
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- A Case of WDHA(Watery Diarrhea Hypokalemia Achiorhydria) Syndrome
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Nam Seon Beck, Jun Jai Kim, Beom Soo Park, Jeong Kee Seo, Hyo Seop Ahn, Hyung Ro Moon, Je Geun Chi
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Clin Exp Pediatr. 1992;35(4):575-580. Published online April 15, 1992
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VIP secreting tumors are rare in children but they produce a dramatic clinical picture, the most prominent feature of which is profuse, watery diarrhea and hypokalemia. A 5-year-old girl was brought to Seoul National University Children's Hospital for evaluation of profuse watery diarrhea. She presented with watery diarrhea, hypokalemia, and stunted growth, and had experienced these problems for four years... |
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- Three Cases of Brain Tumors with Bone Metastasis
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Eun Joo Kim, Kyung Duk Park, Eun Sil Dong, Hye Jung Park, Hong Hoe Koo, Hee Young Shin, Hyo Seop Ahn, Je Geun Chi
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Clin Exp Pediatr. 1992;35(3):381-389. Published online March 15, 1992
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Medulloblastoma and primitive neuroectodermal tumor(PNET) are relatively common intracranial neoplasms in childhood. Their extracranial metastasis were thought to be rare occurrences. But they are more frequent compared to other intracranial tumors. The most frequent site of metastasis deposits in medulloblastoma is bone, and metastasis to lymph node, peritoneum, liver or lung have been reported infrequently. The authors experienced three cases... |
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- Original Article
- Gastrofiberscopic Findings and Helicobacter pylori Gastritis in Children with Recurrent Abdominal Pain
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Jeong Kee Seo, Je Geun Chi, Eui Chong Kim
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Clin Exp Pediatr. 1992;35(12):1646-1656. Published online December 15, 1992
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Gastrofiberscopic findings and the presence of Helicobacter pylori (H. pylori) were studied prospectively in children with recurrent abdominal pain (RAP). Endoscopic findings in 707 children with RAP revealed that 243 children (34.3%) showed abnormal findings including gastritis in 16.9%, duodenitis in 10.4%, esophagitis in 5.7%, duodenal ulcer in 5.7%, and gastric ulcer in 1.1% of children with RAP.
Endoscopic biopsy of... |
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- Case Report
- A Case of Intestinal Lymphangiectasia
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Yae Kyung Suh, Kyung Hee Park, Chul Ho Jang, Bum Soo Park, Jeong Kee Seo, Sung Hae Park, Je Geun Chi, Kyung Mo Yeon, Kwi Won Park
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Clin Exp Pediatr. 1992;35(12):1737-1743. Published online December 15, 1992
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Intestinal lymphangiectasia is a primary or secondary disorder of the gastrointestinal tract, which is associated with lymphatic dysfuction and protein-losing enteropathy. It's clinical manifestations vary widely, but the main symptoms are abdominal distention, edema, abdominal pain and growth failure.
We described a 15-yr-old boy, who suffered from above symptoms since third year of his age and diagnosed by duodenal endoscopy which... |
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- A Case of Asphyxiating Thoracic Dystrophy Type II
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Sang Mi Ha, Soon Il Lee, Moon Chul Kim, Je Geun Chi
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Clin Exp Pediatr. 1992;35(12):1756-1761. Published online December 15, 1992
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Asphyxiating thoracic dystrophy (ATD) is a rare inherited malformation first described in 1954 by Jeune et al. ATD is a clinical and radiological entity characterized by disturbance of in utero endochondral bone formation; it appears with a small thoracic cage and pelvic and phalangeal abnormalities.
The authors experienced a case of ATD in a 1 day old boy with the chief... |
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- A Case of Chronic Intestinal Pseudo-obstruction Syndrome
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Hye Won Park, Chul Ho Chang, Bum Soo Park, Jeong Kee Seo, Sung Hye Park, Je Geun Chi, Kyung Mo Yeon, Kui Won Park
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Clin Exp Pediatr. 1992;35(10):1427-1434. Published online October 15, 1992
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Chronic intestinal pseudo-obstruction is a clinical condition in which impaired intestinal propulsion causes recurrent symptoms of bowel obstruction in the absence of mechanical occlusion.
In this paper a female neonate was presented with vomiting and abdominal distension in the first few days of life but passed normal meconium. Barium enema showed a microcolon and an abnormaly sited cecum. Malrotation of bowel... |
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- Original Article
- Weight and morphologic development of prenatal human thymus.
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Il Soo Ha, Kwang Wook Ko, Je Geun Chi
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Clin Exp Pediatr. 1991;34(8):1057-1069. Published online August 31, 1991
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Thymus is a primary lymphoid organ which has an important role in maturation of immune
competence. This organ matures earlier than the other organs, and the informations about its
prenatal development are essential to understand the maturation of human immunity. To delineate
the weight and morphologic development of prenatal thymus, the authors measured the weight of 221,
and observed gross and histologic features of... |
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- Morphological study on the development of human fetal kidney.
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Hee Young Shin, Kwang Wook Ko, Je Geun Chi
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Clin Exp Pediatr. 1991;34(8):1045-1056. Published online August 31, 1991
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A total of 293 human fetal kidneys, including those of 42 embryos are studied for the elucidation
of temporal development of the kidney. These specimens were interpreted as normal by thorough
postmortem examinations. The age of the specimens was determined by developmental horizon
criteria in embryos and by correlating crown-rump length in fetuses. Embryos were embedded in toto,
cut in various planes and reconstructed... |
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- Observation on neurocysticercosis in childhood.
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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
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Clin Exp Pediatr. 1991;34(6):877-889. Published online June 30, 1991
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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... |
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- A case of 4p- syndrome with oligomeganephronia.
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Ri Sa Lee, Bong Sik Kong, Beyong Il Kim, Sang Kyu Park, Ho Jin Park, Soong Deok Lee, Je Geun Chi
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Clin Exp Pediatr. 1991;34(4):558-565. Published online April 30, 1991
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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... |
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- A case of Caroli's disease.
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Seong Hee Jung, Cheol Ho Chang, Han Tchah, Jeong Kee Seo, Kwi Won Park, In One Kim, Kyung Mo Yeon, Je Geun Chi
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Clin Exp Pediatr. 1991;34(2):261-266. Published online February 28, 1991
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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... |
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- A case of pulmonary vascular air embolism in the newborn.
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Won Soon Park, Beom Soo Park, Hye Kyung Han, Jung Hwan Choi, Chong Ku Yun, Kyoung Mo Yeon, Je Geun Chi
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Clin Exp Pediatr. 1991;34(10):1422-1427. Published online October 31, 1991
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Pulmonary vascular air embolism is a rare, and almost invariably fatal complication of positive
pressure ventilation of newborn infants. There have only been 53 cases described in the world
literature to date.
We have experienced a case of pulmonary vascular air embolism in a premature newborn as a
complication of mechanical ventilation during the course of respiratory distress syndrome.
The pathophysiology and clinical characteristics of... |
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- A Case of Neuroepithelial(Colloid) Cyst.
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Min Sik Kim, Yo Han Kim, Mu Young Song, Soon Jai Lee, Young Bae Lee, Je Geun Chi
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Clin Exp Pediatr. 1990;33(5):695-700. Published online May 31, 1990
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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... |
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- A case of Second Malignant Neoplasm Complicating Hodgkin's Disease in Remission.
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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
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Clin Exp Pediatr. 1990;33(4):564-572. Published online April 30, 1990
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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... |
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- A Case of Retroperitoneal Lymphangioma.
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Gyu Jin Oh, Jin Hyeon Park, Hee Jung Kwon, In Sil Lee, Kui Won Park, Je Geun Chi
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Clin Exp Pediatr. 1990;33(3):422-428. Published online March 31, 1990
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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... |
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- A Case of Transient Myeloproliferative Disorder in Down's Syndrome.
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Ji Hye Kang, Young Mi Hong, Kyung Hee Kim, Seung Joo Lee, Ki Sook Hong, Ok Kyung Kim, Je Geun Chi
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Clin Exp Pediatr. 1990;33(2):252-258. Published online February 28, 1990
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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... |
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- A Case of MELAS Syndrome.
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Ki Joong Kim, Yong Seung Hwang, Young In Choi, Sung Hye Park, Je Geun Chi
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Clin Exp Pediatr. 1990;33(11):1586-1592. Published online November 30, 1990
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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. |
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- A Case of Mucinous Cystadenocarcinoma in a Premenarchal Girl.
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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
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Clin Exp Pediatr. 1990;33(1):124-128. Published online January 31, 1990
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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... |
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- Congenital Systemic Cytomegalic Inclusion Disease.
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Dong Beom Lee, Dong Hyun Kim, Jung Sik Min, Chang Hee Choi, Je Geun Chi
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Clin Exp Pediatr. 1990;33(1):100-106. Published online January 31, 1990
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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... |
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- Juvenile Nephronophyjisis in An Infant.
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Young Seo Park, Chang Youn Lee, Hae Il Cheong, Yong Choi, Kwang Wook Ko, Je Geun Chi, Chang Bin Im
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Clin Exp Pediatr. 1989;32(8):1155-1160. Published online August 31, 1989
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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... |
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- Alobar Holotelencephaly Associated with Microphthalmia and Choanal Atresia.
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Hyon Sook Seo, Gun Tae Yi, Jung Hye Choi, Fan Chen Mong, Hee Dae Park, Soo Nam Rhee, Je Geun Chi
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Clin Exp Pediatr. 1989;32(7):1007-1011. Published online July 31, 1989
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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. |
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- A Case of Virus Associated Hemophagocytic Syndrome.
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Yang Soo Kang, Ue Chong Yang, Hae Il Cheoung, Ho Jin Park, Mi Ja Shin, Je Geun Chi
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Clin Exp Pediatr. 1989;32(4):567-575. Published online April 30, 1989
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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... |
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- A Case of Congenital Megakaryoblastic Leukemia Accompanied by Down Syndrome Which was Diagnosed by Autopsy Findings.
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Il Kyung Kim, Dong Beom lee, Jung Sik Min, Chang Hee Choi, Je Geun Chi
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Clin Exp Pediatr. 1989;32(2):262-269. Published online February 28, 1989
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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... |
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- A Case of Mixed Gonadal Dysgenesis.
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Byung Ran Yun, Jae Il Sohn, Sei Won Yang, Hyung Ro Moon, Je Geun Chi
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Clin Exp Pediatr. 1989;32(12):1757-1762. Published online December 31, 1989
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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... |
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- A case of hypertophic cardiomyopathy in newborn infant: An autopsy case.
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Min Shik Kim, Eun Yong Choi, Hey Sun Lee, Young Min Ahn, Je Geun Chi
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Clin Exp Pediatr. 1989;32(11):1560-1567. Published online November 30, 1989
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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... |
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- Angiolymphoid Hyperplasia with Eosinophilial: Kiruma's disease.
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Choong Won Lee, Young Sik Kim, Soon Ki Kim, Il Soo Ha, Hyo Seop Ahn, Je Geun Chi, Il Han Kim
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Clin Exp Pediatr. 1989;32(10):1416-1421. Published online October 31, 1989
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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... |
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- Two Cases of Idiopathic pulmonary Hemosiderosis.
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Sung Ky You, Ki Soo Kim, Young Jee Kim, Young Yull Koh, Yeon Lim Suh, Je Geun Chi
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Clin Exp Pediatr. 1988;31(9):1209-1216. Published online September 30, 1988
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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... |
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- A Case of Acute Megakaryoblastic Leukemia in Infant with Down's Syndrome.
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Jung Soon Whang, Soon hee Kim, Moon Ja Kim, Keun Lee, Je Geun Chi
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Clin Exp Pediatr. 1988;31(8):1064-1070. Published online August 31, 1988
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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... |
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- A Case of Familial Dysautonomia.
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Hong Jin Lee, Dong Kyu Jin, Sei Won Yang, Jeong Kee Seo, Hyung Ro Moon, Je Geun Chi
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Clin Exp Pediatr. 1988;31(5):648-654. Published online May 31, 1988
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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. |
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- A Case of Pulmonary Candidiasis.
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Hye Ran Byun, Kung Ha Ryu, Moon Ja Kim, Keun Lee, Yeon Lim Suh, Je Geun Chi
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Clin Exp Pediatr. 1988;31(5):621-626. Published online May 31, 1988
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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... |
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- Case Report
- A Case of Gaucher's Disease.
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Hyo Nam Cho, Myung Cheol Cho, Hyung Ro Moon, Je Geun Chi, Hyo Min Kim
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Clin Exp Pediatr. 1987;30(7):784-790. Published online July 31, 1987
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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.... |
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- A Cases of Retroperitoneal Immature Teratoma(Grade III).
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A Ellen Kim, Hee Ju Kim, Jae Sun Jung, Sung Ill Ahn, Hye Kyung Lee, Je Geun Chi
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Clin Exp Pediatr. 1987;30(3):327-334. Published online March 31, 1987
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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... |
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- A Case of Subdiaphragmatic Total Anomalous Pulmonary Venous Return.
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Ho Jin Lee, Sung Sik Lee, Soon Il Lee, Young Seok Lee, Je Geun Chi
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Clin Exp Pediatr. 1986;29(7):791-796. Published online July 31, 1986
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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... |
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- A Case of Potter Syndrome Type I.
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Jong Cheol Ryu, Jae Kwang Hong, Jun Taek Park, Jung Sik Min, Chang Jee Choi, Je Geun Chi
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Clin Exp Pediatr. 1986;29(10):1152-1156. Published online October 31, 1986
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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... |
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- Asplenia Syndrome
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Kwang Wook Ko, Je Geun Chi
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Clin Exp Pediatr. 1985;28(11):1163-1163. Published online November 30, 1985
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- Four Cases of Papillary Thyroid Cancer in Childhood.
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Sei Weon Yang, Sei Won Park, In Sil Lee, Hyo Seup Ahn, Hyung Ro Moon, Chang Yee Hong, Kwi Won Park, Je Geun Chi
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Clin Exp Pediatr. 1984;27(3):282-287. Published online March 31, 1984
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Papaillary thyroid cancer is a rare lesion in children. It progresses slowly and its prognosis is relatively good. A painless nodule in the thyroid or in the neck is the usual first evidence of disease. Cervical lymph node involvement is usually present at the time of the initial diagnosis and is often bilateral. The lungs are the most common site... |
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- Hypothyroid Myopathy.
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Kawng Wook Ko, Je Geun Chi, Hyung Ro Moon
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Clin Exp Pediatr. 1984;27(11):1147-1150. Published online November 30, 1984
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- Congenital biliary atresia.
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Kwang Wook Ko, Je Geun Chi, Kwi Won Park
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Clin Exp Pediatr. 1983;26(1):106-106. Published online January 31, 1983
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- Congenital Systemic Cytomegalic Inclusion Disease: An Autopsy Case Report.
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Seung Bok Cho, Boc Lyul Park, Mi Na Lee, Hea Soo Koo, Je Geun Chi
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Clin Exp Pediatr. 1981;24(9):865-871. Published online September 15, 1981
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A case of systemic cytomegalovirus infection in a newborn baby is reported. This female baby was born after 36 weeks gestation to a 30 year-old mother who had no prenatal problem except for breech presentation and placenta previa for which Caesarian section was given. The mother has two healthy children. The clinical course of this baby was characterized by repeat... |
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- A Case of Congenital Hepatic Fibrosis.
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Son Moon Shin, Sang Il Lee, Joong Gon Kim, Hyo Seop Ahn, Hyung Ro Moon, Kwang Wook Ko, Je Geun Chi
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Clin Exp Pediatr. 1981;24(6):577-583. Published online June 15, 1981
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Congenital hepatic fibrosis is a relatively rare liver disease in children and young adults,that is characterized by stony hard hepatomegaly and portal hypertension with relative preservation of liver function and underlying architecture. Since this Condition was first delineated by Kerr et al in 1961, approximately over 150 cases have been reported in the literature. However, congenital hepatic fibrosis was not... |
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- Malignant Choroid Plexus Tumor in an Infant.
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Jung Yean choi, Bong Sik Kim, Hyo Sup Ahn, Chang Yee Hong, Je Geun Chi, Shin Kwang Khang, Kae Yong Song
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Clin Exp Pediatr. 1978;21(7):552-556. Published online July 31, 1978
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A 42 day-old male infant visited to our emergency room with chief complaints of convulsion. Prenatal and birth histories were unremarkable. The patient appeared well except persistent jaundice and had no signs of increased intracranial pressure. The head circumference at birth was not measured. The head circumference at 42 days was 39cm(50th percnntile) At the age of 42 days, tonic... |
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