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Original Article
Sudden Unexpected Death in Infancy (Analysis of 34 Cases Including 13 Autopsies)
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
Clin Exp Pediatr. 2002;45(9):1065-1074.   Published online September 15, 2002
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...
Case Report
A Case of Hereditary Motor and Sensory Neuropathy Type III
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
Clin Exp Pediatr. 2001;44(9):1057-1061.   Published online September 15, 2001
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...
A Case of Marden-Walker Syndrome
Hyun Jong Cho, Yoon Kyung Lee, Dong Un Kim, Byung Joon Choi, Jin Tack Kim, Ik Jun Lee, Je Geun Chi
Clin Exp Pediatr. 1999;42(10):1471-1474.   Published online October 15, 1999
Original Article
Clinical Presentations of Immotile Cilia Syndroms
Yong Han Sun, Myung Hyun Lee, Young Yull Koh, Je Geun Chi
Clin Exp Pediatr. 1997;40(1):69-79.   Published online January 15, 1997
Case Report
A Pediatric Case of MELAS Syndrome Associated with Insulin-dependent Diabetes Mellitus
Kyung Ran Park, Hye Won Park, Tae Sung Ko, Hae Il Cheong, Sei Won Yang, Young Seung Hwang, In Won Kim, Je Geun Chi
Clin Exp Pediatr. 1996;39(10):1461-1465.   Published online October 15, 1996
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...
A Case of Chronic Pancreatitis Complicated by Hemobilia
Sang Ook Nam, Jeong Kee Seo, Je Geun Chi, Kyung Mo Yeon
Clin Exp Pediatr. 1995;38(3):422-427.   Published online March 15, 1995
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,...
Isolated Unilateral Pulmonary Vein Atresia
Yun Ae Jeon, Chung Il Noh, Ho Sung Kim, Jung Yun Choi, Yong Soo Yun, Woo Sun Kim, Je Geun Chi
Clin Exp Pediatr. 1995;38(3):409-416.   Published online March 15, 1995
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...
A Case of Cardiac Tumor Associated with Tuberous Sclerosis in Newborn
Yong Myung Cho, Woo Seok Kim, Nam Soo Park, Un Jun Hyoung, Eun Ryyoung Kim, Je Geun Chi
Clin Exp Pediatr. 1995;38(2):275-279.   Published online February 15, 1995
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...
A Case of \Intractable Ulcerating Enterocolitis\" of Infant"
Ju Young Jeong, Jeong Kee Seo, Kwi Won Park, Je Geun Chi
Clin Exp Pediatr. 1995;38(2):264-270.   Published online February 15, 1995
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...
Original Article
Acute Hemorrhagic Cystitis(AHC) in Children -Etiology and Clinical Characteristics-
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
Clin Exp Pediatr. 1995;38(2):207-215.   Published online February 15, 1995
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...
Cystic Kidney Disease According to Potter's Classification
Kee Hyuck Kim, Sung Chul Shin, Soon Il Lee, Je Geun Chi
Clin Exp Pediatr. 1995;38(1):91-98.   Published online January 15, 1995
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...
Case Report
Candida Esophagitis in Infancy-A Report of 3 Cases
Ho Sung Kim, Youn Woo Kim, Jae Geon Sim, Beom Soo Park, Hoan Jong Lee, Joong Gon Kim, Jeong Kee Seo, Je Geun Chi
Clin Exp Pediatr. 1994;37(2):269-275.   Published online February 15, 1994
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...
A Case of Bart's Syndrome - Epidermolysis Bullosa and Congenital Localized Absence of Skin -
Hong Shin Jeon, Young Jin Hong, Don Hee Ahn, Hee Jun Yoo, Je Geun Chi
Clin Exp Pediatr. 1994;37(1):104-108.   Published online January 15, 1994
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....
Original Article
Chronic Idiopathic Intestinal pseudo-obstrucion Syndrome in Childhood
Jae Geon Sim, Jeong Kee Seo, Kui Won Park, Je Geun Chi
Clin Exp Pediatr. 1993;36(11):1583-1595.   Published online November 15, 1993
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...
Case Report
A Case of Roberts Syndrome
Young Choi, Yo Han Chung, In Seok Lim, Chul Ha Kim, Dong Keun Lee, Seong Nam Kim, Sang Yong Song, Je Geun Chi
Clin Exp Pediatr. 1993;36(10):1447-1451.   Published online October 15, 1993
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...
Recurrent Rhinocerebral Mucormycosis - A Case Report
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
Clin Exp Pediatr. 1993;36(4):589-595.   Published online April 15, 1993
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...
A Case of Asphyxiating Thoracic Dystrophy Type II
Sang Mi Ha, Soon Il Lee, Moon Chul Kim, Je Geun Chi
Clin Exp Pediatr. 1992;35(12):1756-1761.   Published online December 15, 1992
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...
A Case of Intestinal Lymphangiectasia
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
Clin Exp Pediatr. 1992;35(12):1737-1743.   Published online December 15, 1992
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...
Original Article
Gastrofiberscopic Findings and Helicobacter pylori Gastritis in Children with Recurrent Abdominal Pain
Jeong Kee Seo, Je Geun Chi, Eui Chong Kim
Clin Exp Pediatr. 1992;35(12):1646-1656.   Published online December 15, 1992
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...
Case Report
A Case of Chronic Intestinal Pseudo-obstruction Syndrome
Hye Won Park, Chul Ho Chang, Bum Soo Park, Jeong Kee Seo, Sung Hye Park, Je Geun Chi, Kyung Mo Yeon, Kui Won Park
Clin Exp Pediatr. 1992;35(10):1427-1434.   Published online October 15, 1992
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...
Acquired Persistent Cytomegalovirus Infection : An Association with Common Variable Immunodeficiency
Min Hyea Kim, Young Mi Hong, Sung Joo Lee, Je Geun Chi, Dong Soo Kim
Clin Exp Pediatr. 1992;35(9):1272-1279.   Published online September 15, 1992
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...
A Case of Kugelberg-Welander Syndrome
Sun Young Lee, Myung Ik Lee, Soon Wha Kim, Don Hee Ahn, Je Geun Chi
Clin Exp Pediatr. 1992;35(9):1263-1266.   Published online September 15, 1992
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...
Three Cases of Post-transfusion Hepatitis C
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
Clin Exp Pediatr. 1992;35(9):1255-1262.   Published online September 15, 1992
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...
Transfusion-Acquired Cytomegalovirus Infections in Two Premature Infants
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
Clin Exp Pediatr. 1992;35(8):1141-1147.   Published online August 15, 1992
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...
Seven Cases of Immotile Cilia Syndrome
Sun Young Lee, Myung Ik Lee, Don Hee Ahn, Keun Chan Sohn, Je Geun Chi
Clin Exp Pediatr. 1992;35(8):1127-1134.   Published online August 15, 1992
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...
A Case of WDHA(Watery Diarrhea Hypokalemia Achiorhydria) Syndrome
Nam Seon Beck, Jun Jai Kim, Beom Soo Park, Jeong Kee Seo, Hyo Seop Ahn, Hyung Ro Moon, Je Geun Chi
Clin Exp Pediatr. 1992;35(4):575-580.   Published online April 15, 1992
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...
Three Cases of Brain Tumors with Bone Metastasis
Eun Joo Kim, Kyung Duk Park, Eun Sil Dong, Hye Jung Park, Hong Hoe Koo, Hee Young Shin, Hyo Seop Ahn, Je Geun Chi
Clin Exp Pediatr. 1992;35(3):381-389.   Published online March 15, 1992
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...
Original Article
A case of pulmonary vascular air embolism in the newborn.
Won Soon Park, Beom Soo Park, Hye Kyung Han, Jung Hwan Choi, Chong Ku Yun, Kyoung Mo Yeon, Je Geun Chi
Clin Exp Pediatr. 1991;34(10):1422-1427.   Published online October 31, 1991
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...
Weight and morphologic development of prenatal human thymus.
Il Soo Ha, Kwang Wook Ko, Je Geun Chi
Clin Exp Pediatr. 1991;34(8):1057-1069.   Published online August 31, 1991
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...
Morphological study on the development of human fetal kidney.
Hee Young Shin, Kwang Wook Ko, Je Geun Chi
Clin Exp Pediatr. 1991;34(8):1045-1056.   Published online August 31, 1991
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...