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Case Report
Neurology
A neonate with Joubert syndrome presenting with symptoms of Horner syndrome
Narae Lee, Sang-Ook Nam, Young Mi Kim, Yun-Jin Lee
Clin Exp Pediatr. 2016;59(Suppl 1):S32-S36.   Published online November 30, 2016

Joubert syndrome (JS) is characterized by the “molar tooth sign” (MTS) with cerebellar vermis agenesis, episodic hyperpnea, abnormal eye movements, and hypotonia. Ocular and oculomotor abnormalities have been observed; however, Horner syndrome (HS) has not been documented in children with JS. We present the case of a 2-month-old boy having ocular abnormalities with bilateral nystagmus, left-dominant bilateral ptosis, and unilateral...

X-linked recessive myotubular myopathy with MTM1 mutations
Young-Mi Han, Kyoung-Ah Kwon, Yun-Jin Lee, Sang-Ook Nam, Kyung-Hee Park, Shin-Yun Byun, Gu-Hwan Kim, Han-Wook Yoo
Clin Exp Pediatr. 2013;56(3):139-142.   Published online March 18, 2013

X-linked recessive myotubular myopathy (XLMTM) is a severe congenital muscle disorder caused by mutations in the MTM1 gene and characterized by severe hypotonia and generalized muscle weakness in affected males. It is generally a fatal disorder during the neonatal period and early infancy. The diagnosis is based on typical histopathological findings on muscle biopsy, combined with suggestive clinical features. We...

Review Article
Brain death and organ transplantation
Sang-Ook Nam
Clin Exp Pediatr. 2009;52(8):856-861.   Published online August 15, 2009
Cardiopulmonary arrest has long been accepted as an unquestionable definition of death. An advent of cardiopulmonary resuscitation and artificial ventilation along with the development of organ transplantation has prompted the emergence of the concept of brain death. The criteria for brain death are based mainly on the clinical examination of coma, apnea and total loss of brain stem function. Although organ transplantation by...
Original Article
Multicenter clinical study of childhood periodic syndromes that are common precursors to migraine using new criteria of the International Classification of Headache Disorders (ICHD-II)
Jae Yong Park, Sang-Ook Nam, So-Hee Eun, Su Jeong You, Hoon-Chul Kang, Baik-Lin Eun, Hee Jung Chung
Clin Exp Pediatr. 2009;52(5):557-566.   Published online May 15, 2009
Purpose : To evaluate the clinical features and characteristics of childhood periodic syndromes (CPS) in Korea using the new criteria of the International Classification of Headache Disorders (ICHD)-II. Methods : The study was conducted at pediatric neurology clinics of five urban tertiary-care medical centers in Korea from January 2006 to December 2007. Patients (44 consecutive children and adolescents) were divided into...
Case Report
A Case of Wernicke's Encephalopathy which Occurred During Chemotherapy in a Child with Acute Myeloblastic Leukemia
Sang-Nam Bae, Bu-Jin Kim, Seong-Shik Park, Sang-Ook Nam, Young-Tak Lim, Hak-Jin Kim
Clin Exp Pediatr. 2001;44(11):1320-1325.   Published online November 15, 2001
Wernicke,s encephalopathy(WE), a neurological disorder caused by thiamine deficiency, is characterized by the triad of ocular symptoms, ataxia, and mental confusion. More than 90% of the cases are observed in chronic alcoholics. Other conditions less frequently associated with WE are anorexia nervosa, prolonged parenteral nutrition, hemodialysis, uremia, hyperemesis gravidarum, gastroplasty for morbid obesity, and acquired immunodeficiency syndrome(AIDS). One of the...
Original Article
Factors Related to Weight Changes in Epileptic Children Treated with Vigabatrin
Sang-Nam Bae, Bong-Seok Choi, Sang-Ook Nam, Hee-Ju Park
Clin Exp Pediatr. 2001;44(11):1269-1277.   Published online November 15, 2001
Purpose : This study was undertaken to investigate factors related to weight changes in children treated with vigabatrin(VGB). Methods : We have analyzed mean weight standard deviation scores(SDS) retrospectively and interviewed 35 childhood epileptic patients attending Pusan National University Hospital on VGB monotherapy followed over 36 months. Putative risk factors including sex, age, duration of VGB administration, mean weight SDS...
Effect of Growth Hormone Secretion on Serum Concentration of Leptin
Se-Eun Kim, Jeong-Nyeo Lee, Sang-Ook Nam, Woo-Yeong Chung
Clin Exp Pediatr. 2000;43(7):959-966.   Published online July 15, 2000
Purpose : To investigate the effect of growth hormone(GH) secretion on serum concentration of leptin, we evaluated the differences in serum concentration of leptin between patients with growth hormone deficiency(GHD) and GH normal short stature in prepubertal male children, and the relationship between GH and serum leptin level. Also, we analyzed the correlation between serum concentrations of leptin and insulin-like growth factor-I(IGF-I), insulin-like growth...
Side Effects of Antiepileptic Drug in Children
Ick-Jin Song, Sang-Ook Nam
Clin Exp Pediatr. 1999;42(11):1559-1566.   Published online November 15, 1999
Purpose : The purpose of this study is to estimate the side effects of antiepileptic drug(AED) in children. Methods : Subjects were 267 children who had received AED for at least 1 month during January 1995 to July 1998. We reviewed their sex, age at start of seizure onset, age at medication, class and number of AED, developmental delay, type and...
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