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- Original Article
- Neurology
- Long-term prenatal stress increases susceptibility of N-methyl-D-aspartic acid-induced spasms in infant rats
- Hyeok Hee Kwon, Taekwan Lee, Jinpyo Hong, Dong Woon Kim, Joon Won Kang
- Clin Exp Pediatr. 2018;61(5):150-155. Published online May 28, 2018
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Purpose Infantile spasms, also known as West syndrome, is an age-specific epileptic seizure. Most patients with this condition also exhibit delayed development. This study aimed to determine the effect of long-term prenatal stress on susceptibility to infantile spasms.
Methods We subjected pregnant rats to acute or chronic immobilization stress. Resulting offspring received N-methyl-D-aspartic acid (15 mg/kg, intraperitoneally) on postnatal day 15, and their...
- Review Article
- Nutritional strategy of early amino acid administration in very low birth weight infants
- Byong Sop Lee
- Clin Exp Pediatr. 2015;58(3):77-83. Published online March 20, 2015
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Relative to a fetus of the same gestational age, very low birth weight (VLBW) infants are more likely to be underfed and to undergo growth restriction during their early hospital stay. The current trend towards "early and aggressive" nutritional strategies in VLBW infants aims to overcome the early nutritional deficiency and thereby boost postnatal catch-up growth, simultaneously improving long-term neurodevelopmental...
- Original Article
- Lowe syndrome: a single center's experience in Korea
- Hyun-Kyung Kim, Ja Hye Kim, Yoo-Mi Kim, Gu-Hwan Kim, Beom Hee Lee, Jin-Ho Choi, Han-Wook Yoo
- Clin Exp Pediatr. 2014;57(3):140-148. Published online March 31, 2014
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Purpose Lowe syndrome is a rare, X-linked recessive disorder caused by mutations in the
OCRL gene. It involves multiple anatomic systems, particularly the eyes, central nervous system, and kidneys, and leads to profound growth failure and global developmental delay. This study evaluated the clinical and genetic characteristics of Korean patients with Lowe syndrome.Methods The clinical findings and results of genetic studies were...
- Kidney size estimation in Korean children with Technesium-99m dimercaptosuccinic acid scintigraphy
- Min Jung Lee, Mi Kyung Son, Byung Ok Kwak, Hye Won Park, Sochung Chung, Kyo Sun Kim
- Clin Exp Pediatr. 2014;57(1):41-45. Published online January 31, 2014
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Purpose Renal size is an important indicator to determine adequate organ growth in children. The aim of this study was to estimate renal size with Technesium-99m dimercaptosuccinic acid (DMSA) scan and propose a simple formula for predicting renal length in normal Korean children.
Methods This study included 346 children (148 boys and 198 girls; age range, 1 month to 17 years) in whom...
- Case Report
- Chronic intermittent form of isovaleric aciduria in a 2-year-old boy
- Jin Min Cho, Beom Hee Lee, Gu-Hwan Kim, Yoo-Mi Kim, Jin-Ho Choi, Han-Wook Yoo
- Clin Exp Pediatr. 2013;56(8):351-354. Published online August 27, 2013
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Isovaleric aciduria (IVA) is caused by an autosomal recessive deficiency of isovaleryl-CoA dehydrogenase (IVD). IVA presents either in the neonatal period as an acute episode of fulminant metabolic acidosis, which may lead to coma or death, or later as a "chronic intermittent form" that is associated with developmental delays, with or without recurrent acidotic episodes during periods of stress, such...
- 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
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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
- Discordant findings on dimercaptosuccinic acid scintigraphy in children with multi-detector row computed tomography-proven acute pyelonephritis
- Jeongmin Lee, Duck Geun Kwon, Se Jin Park, Ki-Soo Pai
- Clin Exp Pediatr. 2011;54(5):212-218. Published online May 31, 2011
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Purpose The diagnosis of acute pyelonephritis (APN) is often difficult, as its clinical and biological manifestations are non-specific in children. If not treated quickly and adequately, however, APN may cause irreversible renal damage, possibly leading to hypertension and chronic renal failure. We were suspecting the diagnostic value of 99mTc-dimercaptosuccinic acid (DMSA) scan by experiences and so compared the results of DMSA...
- The relationships between clinical variables and renal parenchymal disease in pediatric clinically suspected urinary tract infection
- Jung Lim Byun, Sang Taek Lee, Sochung Chung, Kyo Sun Kim
- Clin Exp Pediatr. 2010;53(2):222-227. Published online February 15, 2010
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Purpose : To evaluate the significance of clinical signs and laboratory findings as predictors of renal parenchymal lesions and vesicoureteral reflux (VUR) in childhood urinary tract infection (UTI). Methods : From July 2005 to July 2008, 180 patients admitted with a first febrile UTI at the Pediatric Department of Konkuk University Hospital were included in this study. The following were... -
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- Inherited metabolic diseases in the urine organic acid analysis of complex febrile seizure patients
- Hee Jeong Cheong, Hye Rim Kim, Seong Soo Lee, Eun Joo Bae, Won Il Park, Hong Jin Lee, Hui Chul Choi
- Clin Exp Pediatr. 2009;52(2):199-204. Published online February 15, 2009
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Purpose : Seizure associated with fever may indicate the presence of underlying inherited metabolic diseases. The present study was performed to investigate the presence of underlying metabolic diseases in patients with complex febrile seizures, using analyses of urine organic acids. Method : We retrospectively analyzed and compared the results of urine organic acid analysis with routine laboratory findings in 278... -
- Genotype and clinical features of Korean patients with methylmalonic aciduria and propionic aciduria
- Eun Hye Lee, Jung Min Ko, Jae-Min Kim, Han-Wook Yoo
- Clin Exp Pediatr. 2008;51(9):964-970. Published online September 15, 2008
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Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes.... -
- The neuroprotective effect of mycophenolic acid via anti-apoptosis in perinatal hypoxic-ischemic brain injury
- Ji Young Kim, Seung Ho Yang, Sun Hwa Cha, Ji Yeun Kim, Young Chae Jang, Kwan Kyu Park, Jin Kyung Kim, Hai Lee Chung, Eok Su Seo, Woo Taek Kim
- Clin Exp Pediatr. 2007;50(7):686-693. Published online July 15, 2007
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Purpose : Mycophenolic acid (MPA), the active metabolite of mycophenolate mofetil (MMF), is a potent inhibitor of inosine-monophosphate dehydrogenase (IMPDH), a new immunosuppressive drug used. It was reported that MPA protected neurons after excitotoxic injury, induced apoptosis in microglial cells. However, the effects of MPA on hypoxic-ischemic (HI) brain injury has not been yet evaluated. Therefore, we examined whether MPA... -
- Valproate-associated weight gain and potential predictors in children with epilepsy
- Gook Chan Jang, Eun Young Kim, Young Il Rho, Kyung Rye Moon, Sang Kee Park
- Clin Exp Pediatr. 2007;50(5):484-488. Published online May 15, 2007
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Purpose : The purpose of this study was to determine the incidence and potential predictors of weight gain in older children and teens treated with valproate (VPA) for epilepsy. Methods : Sixty-five subjects aged 8 to 17 years of age, who began VPA treatment between January 1, 2001, and December 31, 2004, and who had documented weight and height measurements... -
- Comparison of total parenteral nutrition-associated cholestasis according to amino acid mixtures in very low birth weight infants
- Jin-Sung Choi, Yun-Jin Bae, Young Ah Lee
- Clin Exp Pediatr. 2006;49(9):972-976. Published online September 15, 2006
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Purpose : The purpose of this study was to evaluate the effect of amino acid mixtures on incidence and severity of total parenteral nutrition associated-cholestasis(PNAC) in very low birth weight infants. Methods : Retrospective review of 63 very low birth weight infants(birth weight ≤1,500 g) who received total parenteral nutrition(TPN) in our neonatal intensive care unit from January 2000 to... -
- The diagnostic significance of serum bile acid on total parenteral nutrition induced cholestasis in premature infants
- Kyoung Soo Park, Myung Seok Shin, Mea Young Chang
- Clin Exp Pediatr. 2006;49(8):851-856. Published online August 15, 2006
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Purpose : The purpose of this study is to find out the diagnostic significance of serum bile acid on total parenteral nutrition induced cholestasis in premature infants. Methods : Infants without cholestasis were classified into postnatal days and each change of serum bile acid was measured and analyzed. Also, the serum direct bilirubin, serum bile acid, γ-glutamic acid transferase, and alkaline... -
- Homocysteine, insulin, vitamin B12 and folic acid levels in obese children
- Jee Yoon Park, Young Mi Hong
- Clin Exp Pediatr. 2006;49(5):475-481. Published online May 15, 2006
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Purpose : As the prevalence of childhood obesity is increasing, hypertension, hyperlipidemia, insulin resistance and diabetes mellitus have become problems. High homocysteine levels and low vitamin B12 supplementation are acknowledged to have a role in coronary artery disease, but there are few studies on homocysteine, insulin and vitamin B12 levels in obese children. We aimed to study whether homocysteine, insulin,... -
- Reye syndrome after acute enteritis during the neonatal period
- Jun Suck Bang, Sang Jung Nam, Kyung Hwa Lee, Eun Joo Bae, Won Il Park, Hyun Sook Lee, Bae Young Son, Hwan Suck Choi, Hong Jin Lee
- Clin Exp Pediatr. 2006;49(3):273-277. Published online March 15, 2006
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Purpose : The reinfection rate of H. pylori reported before 13C-urea breath test(13C-UBT) era was higher than that of the post 13C-UBT era. Children are usually reluctant to receive invasive endoscopic evaluation for the reinfection of H. pylori, particularly when they are asymptomatic. The aim of the study is to discover the reinfection rate by different diagnostic tests, and to... -
- Organic acidemias in Korea - Eight years experience of organic acid analysis -
- Jun Suck Bang, Si Hong Lim, Kyung Hwa Lee, Eun Joo Bae, Won Il Park, Hong Jin Lee
- Clin Exp Pediatr. 2006;49(3):258-267. Published online March 15, 2006
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Purpose : We have done this retrospective study to know the relative incidence and clinical manifestations of organic acidopathies in Korea during 8 years(from Jul. 1997 to May 2005). This results of organic acid analysis of 1,787 patients were compared with the results of organic acid analysis that were published three years ago. Methods : The results of quantitative organic acid... -
- Medical Lecture Course
- New Nutritional Concepts of Vitamins and Minerals
- Hee-Shang Youn
- Clin Exp Pediatr. 2005;48(12):1295-1309. Published online December 15, 2005
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Nowadays, the nutritional deficits are rarely seen in Korea. However, an increased availability of the highly palatable energy dense, nutrient-poor foods increases the risks of obesity and deficits of vitamins and minerals in the general population. Also, optimum intake of vitamins and minerals, which varies with age and genetic back ground, might not suffice the poor, young, obese, and elderly... -
- Original Article
- Allogeneic Hematopoietic Stem Cell Transplantation in Juvenile Myelomonocytic Leukemia
- Keon Hee Yoo, Dong Kil You, Soo Hyun Lee, Ki Woong Sung, Eun Joo Cho, Hong Hoe Koo
- Clin Exp Pediatr. 2005;48(2):178-185. Published online February 15, 2005
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Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo- HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ?13-cis-retinoic acid(CRA) before... -
- Urine Organic Acid Analysis of Patients with Febrile Seizures
- Dong Soo Shin, Gun Jun Lee, Kyeung Ja Lee, Won Il Park, Eun Joo Bae, Hong Gin Lee
- Clin Exp Pediatr. 2004;47(11):1205-1209. Published online November 15, 2004
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Purpose : Febrile seizure, the most common seizure disorder in children between 6 months and 5 years of age, is mostly caused by viral infections, except severe CNS infection. It can also be caused by disorders in organic acid metabolism, especially accompanied by fever. Therefore we decided to investigate the relationship between children with febrile seizures and disorders in organic... -
- Quantification of Organic Acids in Amniotic Fluid for Prenatal Diagnosis of Organic Acidemia
- Chang Bum Koh, Dong Soo Shin, Eun Joo Bae, Won Il Park, Kyeung Ja Lee, Hong Jin Lee, Young Jo, Seung Won Lee, Ui Sun Noh
- Clin Exp Pediatr. 2004;47(10):1047-1052. Published online October 15, 2004
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Purpose : Since 1997, the quantification of organic acids in urine has become possible in Korea. This helped to diagnose a great variety of inborn errors of metabolism. However, we still don't know the normal value of organic acids in amniotic fluid, therefore it is impossible for doctors to make a correct diagnosis of inborn errors of metabolism in prenatal... -
- Case Report
- A Case of Partial Hypoxanthine-Guanine Phosphoribosyltransferase Deficiency Presenting as Uric Acid Lithiasis
- Ji Hae Kim, Mi Jung Kim, Kee Hwan Yoo, Young Sook Hong, Joo Won Lee, Soon Kyum Kim
- Clin Exp Pediatr. 2004;47(9):1020-1023. Published online September 15, 2004
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The deficiency of enzyme hypoxanthine-guanine phosphoribosyltransferase(HPRT) results in hyperuricemia and subsequently manifests in diverse symptoms. Lesch-Nyhan syndrome is a disorder characterized by hyperuricemia, mental retardation, choreoathetosis, spasticity and self-mutilation, resulting from complete deficiency of the enzyme, whereas partial deficiency of the enzyme shows symptoms of milder forms more often without abnormal neurologic signs. A 7-year-old boy with normal growth and... -
- Original Article
- Fat Content in Stool of Children with Rotaviral Enteritis
- Joon Sup Song, So Chung Chung, Kyo Sun Kim
- Clin Exp Pediatr. 2003;46(12):1212-1216. Published online December 15, 2003
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Purpose : Rotavirus is a leading cause of severe gastroenteritis in infants and young children around the world. The aim of this study is to investigate the fat content in stools of patients with rotaviral enteritis compared to the stools of children who had no gastroenteritis. Methods : Seventy two patients who were admitted to Konkuk University Hospital, College of... -
- Case Report
- A Case of Acute Pancreatitis in a Neuroblastoma Patient after Retinoic Acid Therapy
- Yoo Jin Jeong, Yeon Kyong Seo, Heung Sik Kim, Hee Jung Lee
- Clin Exp Pediatr. 2003;46(11):1128-1130. Published online November 15, 2003
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Retinoic acid has been used successfully as a differentiating agent in acute promyelocytic leukemia and neuroblastoma. However, some adverse effects have been recognized, such as headaches, dry skin and retinoic acid syndrome, a life threatening acute cardiorespiratory disorder. Acute pancreatitis with hyperlipidemia has rarely been reported. We experienced a case of acute pancreatitis with hyperlipidemia in a neuroblastoma patient after... -
- A Case of Canavan Disease
- Young Ho Son, Tae Gyu Hwang, Jong Beom Sinn
- Clin Exp Pediatr. 2003;46(9):934-938. Published online September 15, 2003
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Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups.... -
- A Case of ARCI Syndrome with Hypoplasia of Corpus Callosum and Heart Anomaly
- Eo-Jin Kim, Young-Ran Yoon, Min-Hae Lee, Ki-Su Kang, Jae-Young Lim, Myoung-Bum Choi, Chan-Hoo Park, Hyang-Ok Woo, Hee-Shang Youn
- Clin Exp Pediatr. 2003;46(8):826-830. Published online August 15, 2003
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ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized... -
- A Case of Glutaric Aciduria Type I with Macrocephaly
- Woo Jong Shin, Yeo Ok Moon, Hye Ran Yoon, Eun Sil Dong, Young Min Ahn
- Clin Exp Pediatr. 2003;46(3):295-301. Published online March 15, 2003
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Glutaric aciduria type 1(GA1) is an autosomal recessive disorder of the lysine, hydroxylysine and tryptophan metabolism caused by the deficiency of mitochondrial glutaryl-CoA dehydrogenase. This disease is characterized by macrocephaly at birth or shortly after birth and various neurologic symptoms. Between the first weeks and the 4-5th year of life, intercurrent illness such as viral infections, gastroenteritis, or even routine... -
- Original Article
- The Morphologic Changes of Parvalbumin- Immunoreactive Interneurons of the Dentate Gyrus in Kainate-Treated Mouse Hippocampal Slice Culture Epilepsy Model
- Hee Sun Chung, Mi-Young Shin, Young-Hoon Kim, In-Goo Lee, Kyung-Tai Whang, Myung-Suk Kim
- Clin Exp Pediatr. 2002;45(12):1551-1558. Published online December 15, 2002
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Purpose : Loss of hippocampal interneurons in dentate gyrus has been reported in patients with severe temporal lobe epilepsy and in animals treated with kainic acid(KA). Interneurons contain Ca2＋- binding protein parvalbumin(PV). The effects of kainic acid on parvalbumin-immunoreactive (PV-IR) interneurons in dentate gyrus were investigated in organotypic hippocampal slice cultures. Methods : Cultured hippocampal slices from postnatal day nine... -
- Organic Acidemias in Korea - Three Years Experience of Organic Acid Analysis -
- You Kyoung Choi, Eung Seok Kim, Eun Joo Bae, Won Il Park, Kyung Ja Lee, Hong Jin Lee
- Clin Exp Pediatr. 2002;45(11):1346-1358. Published online November 15, 2002
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Purpose : We have done this retrospective study to know the relative incidences and clinical manifestations of organic acidopathies in Korea. Methods : The results of quantitative organic acid analysis of 1,125 samples of 712 patients, referred from Jul. 1997 to Jun. 2000, were analyzed retrospectively according to four age groups (-2 mon, 3 mon-2 year, 3 years-12 years, over... -
- Case Report
- A Case of Glutaric Aciduria Type 1
- Joon Young Song, Cheol Min Kim, Young Lim Shin, Han Wook Yoo
- Clin Exp Pediatr. 2002;45(10):1278-1282. Published online October 15, 2002
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Glutaric aciduria(type 1) is characterized clinically by progressive dystonia and dyskinesia in childhood, pathologically by degeneration of caudate and putamen, biochemically by tissue deficiency of glutaryl-CoA dehydrogenase(GCDH), and is transmitted as an autosomal recessive traits. Mutations of the GCDH gene on chromosome 19 have been implicated in the causation of glutaric aciduria(type 1). Macrocephaly in infancy and crossing of percentiles for head circumference are real... -
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6.52022CiteScore92nd percentilePowered by
