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Case Report
Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia
Yun-Jin Lee, Kee-Yang Chung, Hoon-Chul Kang, Heung Dong Kim, Joon Soo Lee
Clin Exp Pediatr. 2015;58(9):354-357.   Published online September 21, 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the...

Original Article
Applying the bacterial meningitis score in children with cerebrospinal fluid pleocytosis: a single center's experience
Jungpyo Lee, Hyeeun Kwon, Joon Soo Lee, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(7):251-255.   Published online July 22, 2015
Purpose

The widespread introduction of bacterial conjugate vaccines has decreased the risk of cerebrospinal fluid (CSF) pleocytosis due to bacterial meningitis (BM) in children. However, most patients with CSF pleocytosis are hospitalized and treated with parenteral antibiotics for several days. The bacterial meningitis score (BMS) is a validated multivariate model derived from a pediatric population in the postconjugate vaccine era and...

Case Report
Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses
Soo Jin Chang, Ji Hyun Lee, Shin Hye Kim, Joon Soo Lee, Heung Dong Kim, Joon Won Kang, Young Mock Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(5):194-198.   Published online May 22, 2015

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at...

Original Article
Screening for depression and anxiety disorder in children with headache
Sang Mi Lee, Jung-Rim Yoon, Yoon Young Yi, Soyong Eom, Joon Soo Lee, Heung Dong Kim, Keun-Ah Cheon, Hoon-Chul Kang
Clin Exp Pediatr. 2015;58(2):64-68.   Published online February 28, 2015
Purpose

The purpose of this study was to investigate the importance of initial screening tests for depression and anxiety disorders in children with headache. In addition, this study evaluated whether the Children's Depression Inventory (CDI) and Revised Children's Manifest Anxiety Scale (RCMAS) are suitable for screening symptoms of depression and anxiety.

Methods

A retrospective chart review was conducted of 720 children aged 7-17...

Case Report
Wernicke's encephalopathy in a child with high dose thiamine therapy
So Won Park, Yoon Young Yi, Jung Woo Han, Heung Dong Kim, Joon Soo Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2014;57(11):496-499.   Published online November 30, 2014

Wernicke's encephalopathy is an acute neurological disorder characterized by mental confusion, oculomotor dysfunction, and ataxia. It has been reported in individuals with alcohol dependence, hyperemesis gravidarum, and prolonged parenteral nutrition without vitamin supplementation. Here we present the case of a 13-year-old male patient with neuroblastoma and a history of poor oral intake and nausea for 3 months. After admission, he...

Congenital muscular dystrophy type 1A with residual merosin expression
Hyo Jeong Kim, Young-Chul Choi, Hyung Jun Park, Young-Mock Lee, Heung Dong Kim, Joon Soo Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2014;57(3):149-152.   Published online March 31, 2014

Congenital muscular dystrophy type 1A (MDC1A) is an autosomal recessive disorder characterized by hypotonia, elevated serum creatine kinase level, delayed motor milestones, white matter changes observed by brain magnetic resonance imaging, and normal intelligence. A mutation in the laminin α2 (LAMA2) gene, located at 6q22-23, is a genetic cause of MDC1A. Patients have merosin (laminin α2)-deficient skeletal muscles. However, the...

Review Article
Lower fat and better quality diet therapy for children with pharmacoresistant epilepsy
Jung-Rim Yoon, Heung Dong Kim, Hoon-Chul Kang
Clin Exp Pediatr. 2013;56(8):327-331.   Published online August 27, 2013

The ketogenic diet (KD) is an established, effective, nonpharmacologic treatment for children with pharmacoresistant epilepsy. Although the KD is the most well-established dietary therapy for epilepsy, it is too restrictive and is associated with serious complications; therefore, alternative lower-fat diets, including a modified Atkins diet and low-glycemic index diet, have been developed. Recent ongoing clinical evidence suggests that other dietary...

Original Article
Epilepsy in Korean patients with Angelman syndrome
Sung-Hee Park, Jung-Rim Yoon, Heung Dong Kim, Joon Soo Lee, Young-Mock Lee, Hoon-Chul Kang
Clin Exp Pediatr. 2012;55(5):171-176.   Published online May 21, 2012
Purpose

The aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea.

Methods

We retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric...

Review Article
Disease-specific pluripotent stem cells
Hoon-Chul Kang
Clin Exp Pediatr. 2010;53(8):786-789.   Published online August 31, 2010

Induced pluripotent stem (iPS) cells are generated by epigenetic reprogramming of somatic cells through the exogenous expression of transcription factors. Recently, the generation of iPS cells from patients with a variety of genetic diseases was found to likely have a major impact on regenerative medicine, because these cells self-renew indefinitely in culture while retaining the capacity to differentiate into any...

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...
A pilot study of neuroprotection with umbilical cord blood cell transplantation for preterm very low birth weight infants
Kyu Young Chae, Kyu Hyung Lee, So-Hee Eun, Byung Min Choi, Baik-Lin Eun, Hoon-Chul Kang, Myung Jae Chey, Nam Keun Kim, Doyeun Oh
Clin Exp Pediatr. 2007;50(9):882-890.   Published online September 15, 2007
Purpose : Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental...