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Review Article
Nephrology (Genitourinary)
Atypical hemolytic uremic syndrome and eculizumab therapy in children
Seong Heon Kim, Hye Young Kim, Su Young Kim
Clin Exp Pediatr. 2018;61(2):37-42.   Published online February 28, 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is...

Case Report
Shiga toxin-associated hemolytic uremic syndrome complicated by intestinal perforation in a child with typical hemolytic uremic syndrome
Hye Jin Chang, Hwa Young Kim, Jae Hong Choi, Hyun Jin Choi, Jae Sung Ko, Il Soo Ha, Hae Il Cheong, Yong Choi, Hee Gyung Kang
Clin Exp Pediatr. 2014;57(2):96-99.   Published online February 24, 2014

Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in childhood and is primarily diagnosed in up to 4.5% of children who undergo chronic renal replacement therapy. Escherichia coli serotype O157:H7 is the predominant bacterial strain identified in patients with HUS; more than 100 types of Shiga toxin-producing enterohemorrhagic E. coli (EHEC) subtypes have...

Original Article
Comparative study of typical and atypical benign epilepsy with centrotemporal spikes (Rolandic epilepsy)
Junhyuk Song, Kyuha Lee, Sajun Chung
Clin Exp Pediatr. 2008;51(10):1085-1089.   Published online October 15, 2008
Purpose : This study aims to examine and compare the features of rolandic epilepsy. Methods : Of 158 patients selected retrospectively, 116 had typical (group A) and 42 had atypical (group B) rolandic epilepsy, as defined by Worrall's criteria. Results : The age at onset of the seizures in group A was 8.6¡¾2.0 y and 6.2¡¾1.7 y in group B...
Case Report
A Case of Atypical Teratoid/Rhabdoid Tumor Arising from the Supratentorial Area
Kyeong Hun Jung, Young Se Kwon, Yong Hun Jun, Soon Ki Kim, Young Jin Hong, Byong Kwan Son, Eun Young Kim, In Suh Park
Clin Exp Pediatr. 2005;48(2):228-231.   Published online February 15, 2005
Atypical teratoid/rhabdoid tumor may arise at any central nervous system location, but it is most commonly located in the cerebellum(60 percent). The incidence of this tumor remains unclear but it occurs most commonly in children less than 2 years of age. This highly malignant tumor shows a rapid progression and nonspecific radiologic findings. We report a case of primary intracranial...
Atypical Hemolytic Uremic Syndrome Associated with Streptococcus pneumoniae Infection
Hyun Sug Lee, Ja Wook Koo, Sang Woo Kim, Hyun Soon Lee
Clin Exp Pediatr. 2004;47(2):217-222.   Published online February 15, 2004
Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease....
Original Article
Clinical Features of Atypical Kawasaki Disease
Mi Young Heo, Su Jung Choi, Hae Soon Kim, Sejung Sohn
Clin Exp Pediatr. 2002;45(3):376-382.   Published online March 15, 2002
Purpose : To identify clinical and laboratory features of atypical Kawasaki disease(KD), and to develop criteria for early diagnosis of atypical KD patients. Methods : All patients with KD treated at our hospital from January 1998 to June 2000 were reviewed retrospectively. Results : Among a total of 167 patients, 28(16.8%) were atypical KD of which seven(25%) were infants. Among the...
Case Report
A Case of Central Nervous System Atypical Teratoid/Rhabdoid Tumor of The 4th Ventricle : A Highly Malignant Tumor in Infancy and Childhood Frequently Mistaken for Medulloblastoma
So Hee Eun, Sung Hee Jang, Myoung Hee Han, Myoung Ja Chung, Pyoung Han Hwang
Clin Exp Pediatr. 2001;44(12):1475-1479.   Published online December 15, 2001
Central nervous system atypical teratoid/rhabdoid tumor(CNS rhabdoid tumor) is a rare malignancy of uncertain origin. It typically occurs in infants and young children and comprises only a small fraction of pediatrics CNS malignancies. The tumor contains a large spindled cell component as classical rhabdoid morphology and focal areas resembling primitive neuroectodermal tumor. The tumor is defined histopathologically by the presence...
Original Article
Clinical Analysis of Atypical Kawasaki Disease : Comparison of Kawasaki Disease Between Typical and Atypical Types
Eun Ju Jeong, Jae Hong Park, Hee Ju Park
Clin Exp Pediatr. 2001;44(12):1448-1453.   Published online December 15, 2001
Purpose : Kawasaki disease(KD) is known as one of the most important causes of acquired heart disease in children. But the incidence of acquired heart disease can be reduced by early diagnosis and treatment with large amounts of intravenous γ-globulin(IVGG). For early diagnosis and treatment of atypical KD, we analyzed and compared the clinical features, laboratory findings and coronary lesions in patients with typical...
Classification and Risk Factors for Chronic Lung Disease(CLD) of Prematurity : Classical CLD Versus Atypical CLD
Chang Won Choi, Beyong Il Kim, Heui Seung Jo, Jun Dong Park, Chong Jae Kim, Bo Hyun Yoon, Jung-Hwan Choi
Clin Exp Pediatr. 2001;44(11):1222-1232.   Published online November 15, 2001
Purpose : We tried to classify the different type of CLD and assess the risk factors for classical CLD and atypical CLD. Methods : Retrospective cohort analysis was done in 120 preterm infants with birth weights less than 1,500 g who were admitted to NICU in Seoul National University Children's Hospital between Jan. 1993 and Dec. 1998 and survived more...
Case Report
A Case of Atypical Benign Partial Childhood Epilepsy
Hae Jung Park, In Joon Seol
Clin Exp Pediatr. 2001;44(2):211-215.   Published online February 15, 2001
We report a case of atypical benign partial childhood epilepsy in a 11 years old male child whose case has been followed up for 6 years. His first symptom was focal seizure of the left side of his face during a drowsy state, followed by focal seizures of left fingers and legs. At that time he had been on phenobarbital...
Original Article
A Clinical Study of Atypical Kawasaki Disease - A Rate of Coronary Artery Involvement -
Jin-Sook Kim, Young-Yoo Kim, Jong-Wan Kim, Won-Bae Lee, Jin-Han Kang, Kyung-Tai Whang
Clin Exp Pediatr. 2000;43(1):70-77.   Published online January 15, 2000
Purpose : Without a specific diagnostic method, the clinical diagnostic criteria for atypical Kawasaki disease is known as the presence of coronary artery changes in a patient who did not meet the already estabilished clinical criteria. With this criteria, we are put into dilemma because atypical Kawasaki disease should exhibit coronary artery disease. So, we demonstrated the rate of coronary artery involvement in atypical...
Erratum
A Case of Joubert Syndrome
Hye-Young Kim, Sun-Jun Kim, Jung-Soo Kim
Accepted January 1, 1970  
Joubert syndrome is frequently associated with developmental delay and mental retardation, neonatal tachypnea and apnea, abnormal eye movements, and ataxia. It is inherited by an autosomal recessive trait. The most significant and constant neuropathological finding is partial or total agenesis of cerebellar vermis. Absence of the vermis results in a triangular-shaped mid-fourth ventricle and a "bat-wing" shaped fourth ventricle superiorly. The superior cerebellar peduncles...
Case Report
Abstract= A Case of Right Coronary Arterial Occlusion with Normal Electrocardiogram in Atypical Kawasaki Diseases
Yoon Kyung Lee, Jong Wan Kim, Kyung Tai Whang
Clin Exp Pediatr. 1996;39(3):411-416.   Published online March 15, 1996
Kawasaki disease is an acute febrile illness of unknown etiology that occurs predominantly in infants and early childhood. It had aroused intense interest because of the sequelae of coronary arteritis accompanied by coronary artery aneurysms and thrombotic occlusion, which may lead to ischemic heart disease or sudden death. Atypical Kawasaki disease is coined to describe patients who have coronary abnormalities,...
Two Cases of Tetrahydrobiopterin Deficiency
Jee Won Choi, Sei Won Yang, Hyung Ro Moon, Se Chin Cho
Clin Exp Pediatr. 1995;38(3):397-403.   Published online March 15, 1995
Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4 deficiency in brother and sister, confirmed by biochemical study of blood and urine. They had suffered from a progressive neurologic illness such as mental retardation, severe hypotonia, seizure, and athetotic movements...
Original Article
Unusual Presentation of Kawasaki Disease Complicated by Coronary Aneurysms
Young-Ah Lee, In-Sook Park, Young-Hwue Kim, Hyung-Nam Moon, Chang-Yee Hong
Clin Exp Pediatr. 1994;37(7):890-899.   Published online July 15, 1994
Patients with atypical or incomplete Kawasaki disease are at same risk for development of coronary artery complications as typical Kawasaki disease. In this communication we report six patients with unusual presentation of Kawasaki disease complicated by coronary artery aneurysms, in whom correct diagnosis were not made in time for proper treatment. One of these patients died from massive myocardial ischemia...
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