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Original Article
Effects of coagulation factor concentrate prophylaxis in moderate and severe hemophilia A patients at a single hemophilia center in Korea
Byung Suk Moon, Jun Seok Choi, Chur Woo You
Clin Exp Pediatr. 2013;56(7):291-297.   Published online July 19, 2013
Purpose

The aim of this study was to investigate prophylactic treatment effects in Korean patients with severe hemophilia A.

Methods

A prospective study of 32 severe hemophilia A patients was conducted with the approval of the Institutional Review Board at the Eulji University Hospital. Two patients received primary prophylaxis; whereas, the other 30 patients were divided into 2 groups-secondary prophylaxis (n=15) and on-demand...

Case Report
Two cases of chronic pancreatitis associated with anomalous pancreaticobiliary ductal union and SPINK1 mutation
Eun Sam Rho, Earl Kim, Hong Koh, Han-Wook Yoo, Beom Hee Lee, Gu-Hwan Kim
Clin Exp Pediatr. 2013;56(5):227-230.   Published online May 28, 2013

Chronic pancreatitis is a progressive inflammatory disease resulting from repeated episodes of acute pancreatitis that impair exocrine function and eventually produce endocrine insufficiency. Some causes of chronic pancreatitis appear to be associated with alterations in the serine-protease inhibitor, Kazal type 1 (SPINK1), cationic trypsinogen (PRSS1), and cystic fibrosis-transmembrane conductance regulator (CFTR) genes, or with structural disorders in the pancreaticobiliary ductal...

Original Article
The hybrid perventricular closure of apical muscular ventricular septal defect with Amplatzer duct occluder
Soo Jin Kim, June Huh, Jin Young Song, Ji-Hyuk Yang, Tae-Gook Jun, I-Seok Kang
Clin Exp Pediatr. 2013;56(4):176-181.   Published online April 22, 2013
Purpose

Apical muscular ventricular septal defects (MVSDs), especially in small infants, can be difficult to manage using surgical and percutaneous closure. An intraoperative perventricular procedure is a good option for closing apical MVSDs in small children with or without associated cardiac anomalies. We evaluated the results of hybrid perventricular closure of apical MVSDs performed using an Amplatzer duct occluder (ADO).

Methods

We retrospectively...

p-Cresyl sulfate and indoxyl sulfate in pediatric patients on chronic dialysis
Hye Sun Hyun, Kyung Hoon Paik, Hee Yeon Cho
Clin Exp Pediatr. 2013;56(4):159-164.   Published online April 22, 2013
Purpose

Indoxyl sulfate and p-cresyl sulfate are important protein-bound uremic retention solutes whose levels can be partially reduced by renal replacement therapy. These solutes originate from intestinal bacterial protein fermentation and are associated with cardiovascular outcomes and chronic kidney disease progression. The aims of this study were to investigate the levels of indoxyl sulfate and p-cresyl sulfate as well as the...

Expression of peroxisome proliferator-activated receptor (PPAR)-α and PPAR-γ in the lung tissue of obese mice and the effect of rosiglitazone on proinflammatory cytokine expressions in the lung tissue
Seung Lok Ryu, Jae Won Shim, Duk Soo Kim, Hye Lim Jung, Moon Soo Park, Soo-Hee Park, Jinmi Lee, Won-Young Lee, Jung Yeon Shim
Clin Exp Pediatr. 2013;56(4):151-158.   Published online April 22, 2013
Purpose

We investigated the mRNA levels of peroxisome proliferator-activated receptor (PPAR)-α, PPAR-γ, adipokines, and cytokines in the lung tissue of lean and obese mice with and without ovalbumin (OVA) challenge, and the effect of rosiglitazone, a PPAR-γ agonist.

Methods

We developed 6 mice models: OVA-challenged lean mice with and without rosiglitazone; obese mice with and without rosiglitazone; and OVA-challenged obese mice with and...

Case Report
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...

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth
Ju Sun Heo, Ka Young Choi, Se Hyoung Sohn, Curie Kim, Yoon Joo Kim, Seung Han Shin, Jae Myung Lee, Juyoung Lee, Jin A Sohn, Byung Chan Lim, Jin A Lee, Chang Won Choi, Ee-Kyung Kim, Han-Suk Kim, Beyong Il Kim, Jung-Hwan Choi
Clin Exp Pediatr. 2012;55(11):438-444.   Published online November 23, 2012

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant...

Magnetic resonance imaging and spectroscopic analysis in 5 cases of Pelizaeus-Merzbacher disease: metabolic abnormalities as diagnostic tools
Eun Lee, Mi-Sun Yum, Hae-Won Choi, Han-Wook Yoo, Su Jeong You, Eun-Hye Lee, Tae-Sung Ko
Clin Exp Pediatr. 2012;55(10):397-402.   Published online October 29, 2012

Pelizaeus-Merzbacher disease (PMD) is a rare, X-linked recessive disorder characterized by dysmyelination in the central nervous system. PMD results from deletion, mutation, or duplication of the proteolipid protein gene (PLP1) located at Xq22, leading to the failure of axon myelination by oligodendrocytes in the central nervous system. PMD may be suspected when there are clinical manifestations such as nystagmus, developmental...

A case of cytomegalovirus-negative Ménétrier's disease with eosinophilia in a child
Keun Hyung Son, Jeong Ja Kwak, Jae Ock Park
Clin Exp Pediatr. 2012;55(8):293-296.   Published online August 23, 2012

Ménétrier's disease is a rare form of acquired gastropathy characterized by giant rugal folds in the stomach and protein-losing gastropathy. Children with Ménétrier's disease tend to follow a benign self-limited course with symptoms typically completely resolving within 2 to 10 weeks in contrast to the chronic course in adults. A 9-year-old girl presented with a history of gradually worsening abdominal...

Original Article
Neuroprotective effects of L-carnitine against oxygen-glucose deprivation in rat primary cortical neurons
Yu Jin Kim, Soo Yoon Kim, Dong Kyung Sung, Yun Sil Chang, Won Soon Park
Clin Exp Pediatr. 2012;55(7):238-248.   Published online July 17, 2012
Purpose

Hypoxic-ischemic encephalopathy is an important cause of neonatal mortality, as this brain injury disrupts normal mitochondrial respiratory activity. Carnitine plays an essential role in mitochondrial fatty acid transport and modulates excess acyl coenzyme A levels. In this study, we investigated whether treatment of primary cultures of rat cortical neurons with L-carnitine was able to prevent neurotoxicity resulting from oxygen-glucose deprivation...

Review Article
Effects of probiotics on the prevention of atopic dermatitis
Nam Yeun Kim, Geun Eog Ji
Clin Exp Pediatr. 2012;55(6):193-201.   Published online June 21, 2012

Atopic dermatitis (AD) is an immune disorder that is becoming increasingly prevalent throughout the world. The exact etiology of AD remains unknown, and a cure for AD is not currently available. The hypothesis that appropriate early microbial stimulation contributes to the establishment of a balanced immune system in terms of T helper type Th1, Th2, and regulatory T cell (Treg)...

Original Article
Successful and safe treatment of hemangioma with oral propranolol in a single institution
Sun Hee Chung, Dong Hyuk Park, Hye Lim Jung, Jae Won Shim, Deok Soo Kim, Jung Yeon Shim, Moon Soo Park, Hong Hoe Koo
Clin Exp Pediatr. 2012;55(5):164-170.   Published online May 21, 2012
Purpose

Dramatic improvement of hemangioma to propranolol has been recently reported; however, details on dose and duration of treatment, potential risks, and monitoring have not been determined. The objective of this study is to describe and analyze the use of propranolol as a first-line treatment or as a single therapy in management of complicated hemangioma.

Methods

A retrospective chart review of eight patients...

Correlation between glomerular filtration rate and urinary N acetyl-beta-D glucosaminidase in children with persistent proteinuria in chronic glomerular disease
Jeong Deok Hong, In Seok Lim
Clin Exp Pediatr. 2012;55(4):136-142.   Published online April 30, 2012
Purpose

Urinary excretion of N acetyl-beta-D glucosaminidase (NAG) and β2-microglobulin (β2-M) was increased in the presence of proximal tubular damage. Based on these urinary materials, we investigated the ability of expecting renal function in chronic glomerular diseases. In this study, we evaluated the relationship between glomerular filtration rate (GFR) urinary NAG, and urinary β2-M.

Methods

We evaluated 52 children with chronic kidney disease...

Comparison of the accuracy of neutrophil CD64 and C-reactive protein as a single test for the early detection of neonatal sepsis
Young Kwang Choo, Hyun-Seok Cho, In Bum Seo, Hyeon-Soo Lee
Clin Exp Pediatr. 2012;55(1):11-17.   Published online January 31, 2012
Purpose

Early identification of neonatal sepsis is a global issue because of limitations in diagnostic procedures. The objective of this study was to compare the diagnostic accuracy of neutrophil CD64 and C-reactive protein (CRP) as a single test for the early detection of neonatal sepsis.

Methods

A prospective study enrolled newborns with documented sepsis (n=11), clinical sepsis (n=12) and control newborns (n=14). CRP,...

Validation study of the Dinamap ProCare 200 upper arm blood pressure monitor in children and adolescents
Chong Guk Lee, Hyang Mi Park, Hye Jung Shin, Jin Soo Moon, Yeong Mi Hong, Nam Soo Kim, Il Soo Ha, Myeong Jin Chang, Kyeong Won Oh
Clin Exp Pediatr. 2011;54(11):463-469.   Published online November 30, 2011
Purpose

To validate the Dinamap ProCare 200 blood pressure (BP) monitor against a mercury sphygmomanometer in children 7 to 18 years old in accordance with the 2010 International Protocol of European Society of Hypertension (ESH-IP2) and the British Hypertension Society (BHS) protocol.

Methods

Forty-five children were recruited for the study. A validation procedure was performed following the protocol based on the ESH-IP2 and...

Review Article
Complications of nephrotic syndrome
Se Jin Park, Jae Il Shin
Clin Exp Pediatr. 2011;54(8):322-328.   Published online August 31, 2011

Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and...

Original Article
Usefulness of drug provocation tests in children with a history of adverse drug reaction
Hye Ran Na, Jeong Min Lee, Jo Won Jung, Soo-Young Lee
Clin Exp Pediatr. 2011;54(7):304-309.   Published online July 31, 2011
Purpose

There are very few reports of adverse drug reactions (ADR) and almost no study of drug provocation test (DPT) in Korean children. We aimed to assess the role of DPT in children with unpredictable ADRs, and compare the causative drugs and clinical characteristics between detailed history of ADRs and result of DPTs.

Methods

We included 16 children who were experienced ADRs referred...

Novel influenza A (H1N1) 2009 infection in the pediatric patients with hematologic and oncologic diseases in the Yeungnam region
Seok Jeong Kang, Jae Min Lee, Jeong Ok Hah, Ye Jee Shim, Kun Soo Lee, Hyun Jung Shin, Heung Sik Kim, Eun Jin Choi, So Eun Jeon, Young Tak Lim, Ji Kyeong Park, Eun Sil Park
Clin Exp Pediatr. 2011;54(3):117-122.   Published online March 31, 2011
Purpose

Natural history and consequences of the novel 2009 influenza A H1N1 (2009 H1N1) infection in immunocompromised pediatric patients are not yet fully understood. In this study, we investigated the clinical features and outcomes of the 2009 H1N1 infection in pediatric patients with hematological and oncological diseases.

Methods

We retrospectively reviewed the medical records of 528 patients who had hematological and oncological diseases...

Review Article
Recent advances in the diagnosis and management of childhood acute promyelocytic leukemia
Eun Sun Yoo
Clin Exp Pediatr. 2011;54(3):95-105.   Published online March 31, 2011

Since the successful introduction of all-trans-retinoic acid (ATRA) and its combination with anthracycline-containing chemotherapy, the prognosis for acute promyelocytic leukemia (APL) has markedly improved. With ATRA and anthracycline-based-chemotherapy, the complete remission rate is greater than 90%, and the long-term survival rate is 70-89%. Moreover, arsenic trioxide (ATO), which was introduced for APL treatment in 1994, resulted in excellent remission rates...

Original Article
Multiple births conceived by assisted reproductive technology in Korea
Young Sil Park, Sun Hee Choi, Kye Shik Shim, Ji Young Chang, Won Ho Hahn, Yong Sung Choi, Chong-Woo Bae
Clin Exp Pediatr. 2010;53(10):880-885.   Published online October 31, 2010
Purpose

The recent trends of multiple births (MBs) conceived by assisted reproductive technology (ART) in Korea were analyzed as well as the relationship with maternal age, especially advanced maternal age.

Methods

Data were obtained from the Korean Statistical Information Service and annual ART reports from the ART committee of the Korean Society of Obstetrics and Gynecology.

Results

MBs increased from the early 1990s; there was...

Case Report
A case of regression of atypical dense deposit disease without C3 deposition in a child
Min Sun Kim, Pyoung Han Hwang, Mung Jae Kang, Dae-Yeol Lee
Clin Exp Pediatr. 2010;53(7):766-769.   Published online July 31, 2010

Dense deposit disease (DDD) is a rare disorder characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidneys. The diagnosis is made in most patients between 5 and 15 years of age, and within 10 years, approximately half of the affected patients progress to end-stage renal disease. We report a rare case of regressive...

Original Article
The natural history and prognostic factors of Graves' disease in Korean children and adolescents
Seung Min Song, Ji-Seok Youn, Jung Min Ko, Chong Kun Cheon, Jin-Ho Choi, Han Wook Yoo
Clin Exp Pediatr. 2010;53(4):585-591.   Published online April 15, 2010
Purpose : Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. Methods : One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed...
Apoptosis and upregulation of TNF-α and TRAIL receptor 1 (DR4) in the pathogenesis of food protein-induced enterocolitis syndrome
Jin-Bok Hwang, Sang Pyo Kim, Yu Na Kang, Seong-Ryong Lee, Seong-Il Suh, Taeg Kyu Kwon
Clin Exp Pediatr. 2010;53(4):525-531.   Published online April 15, 2010
Purpose : Expression levels of tumor necrosis factor (TNF)-α expression on the mucosa of the small intestine is increased in patients with villous atrophy in food protein-induced enterocolitis syndrome (FPIES). TNF-α has been reported to induce apoptotic cell death in the epithelial cells. We studied the TNF family and TNF-receptor family apoptosis on the duodenal mucosa to investigate their roles in the pathogenesis...
NT-proBNP as a useful tool in diagnosing incomplete Kawasaki disease
Dong Won Lee, Yeo Hyang Kim, Myung Chul Hyun, Tae Chan Kwon, Sang Bum Lee
Clin Exp Pediatr. 2010;53(4):519-524.   Published online April 15, 2010
Purpose : To determine the efficacy of the N-terminal fragment of B-type natriuretic peptide (NT-proBNP) as a useful diagnostic method in children with incomplete Kawasaki disease (KD). Methods : Ninety-six patients who were diagnosed as having KD between January 2008 and June 2009 were enrolled in the study. American Heart Association recommendations for diagnosis were used, and patients were divided into...
Clinical significance of matrix metalloproteinase 9 and tissue inhibitor of metalloproteinase 1 and 2 in Kawasaki disease
Ki Wook Yun, Sin Weon Sin, Jung Ju Lee, Soo Ahn Chae, In Seok Lim, Eung Sang Choi, Byoung Hoon Yoo, Mi-Kyung Lee
Clin Exp Pediatr. 2010;53(4):510-518.   Published online April 15, 2010
Purpose : Kawasaki disease (KD) is a systemic vasculitis, a leading cause of pediatric acquired heart disease. Histopathological findings of coronary artery lesion (CAL) in KD indicate destruction of the coronary artery wall with diffuse vasculitis. Matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs) might play central roles in this process. Special attention to MMP-9 has recently been emerging....
The risk factors and prognosis associated with neonatal pulmonary hemorrhage
Su Jin Park, Ki Tae Yun, Won Duck Kim, Sang Geel Lee
Clin Exp Pediatr. 2010;53(4):503-509.   Published online April 15, 2010
Purpose : Although neonatal pulmonary hemorrhage is rare, it is associated with high mortality. We aimed to evaluate the risk factors associated with pulmonary hemorrhage in preterm infants and to describe the clinical course, including neonatal morbidity, of infants who developed pulmonary hemorrhage. Methods : We performed a retrospective case-control study of 117 newborn infants aged less than 37 gestational weeks admitted...
Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent
Eun Mi Yang, Dong Kyun Han, Hee Jo Baek, Young Ok Kim, Myung Geun Shin, Hoon Kook, Tai Ju Hwang
Clin Exp Pediatr. 2010;53(3):428-431.   Published online March 15, 2010
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other...
Predictable risk factors and clinical courses for prolonged transient tachypnea of the newborn
Ji Young Chang, Chang Ryul Kim, Ellen A Kim, Ki Soo Kim
Clin Exp Pediatr. 2010;53(3):349-357.   Published online March 15, 2010
Purpose : Transient tachypnea of the newborn (TTN) is usually benign and improves within 72 hours. However, it can also progress to prolonged tachypnea over 72 hours, profound hypoxemia, respiratory failure, and even death. The aim of this study is to find predictable risk factors and describe the clinical courses and outcomes of prolonged TTN (PTTN). Methods : The medical records...
Analysis of prognostic factors of laparotomy for necrotizing enterocolitis in extremely low birth weight infants
Jin Kyu Kim, Yi Sun Kim, Hye Soo Yoo, So Yoon Ahn, Hyun ju Seo, Seo Heui Choi, Soo Kyung Park, Yu Jin Jung, Myo Jing Kim, Ga Won Jeon, Soo Hyun Koo, Kyung-Hoon Lee, Yun Sil Chang, Won Soon Park
Clin Exp Pediatr. 2010;53(2):167-172.   Published online February 15, 2010
Purpose : With improved survival of extremely low birth weight infants (ELBWI), there is an increase in the incidence of necrotizing enterocolitis (NEC) requiring laparotomy, and the risk of morbidity and mortality in these ELBWI is increased. Thus, we determined the prognostic factors in ELBWI who underwent laparotomy for NEC. Methods : We retrospectively reviewed the medical records of 35 ELBWI...
Case Report
The effect of perioperative inhaled iloprost on congenital heart disease with severe pulmonary arterial hypertension
Su Nam Kim, Deok Young Choi
Clin Exp Pediatr. 2010;53(1):93-96.   Published online January 15, 2010
A 47-year-old male patient in whom atrial septal defect (ASD) had been diagnosed 15 years previously was admitted for cardiac catheterization. He had definite cyanotic lips and nail beds and severe pulmonary arterial hypertension (PAH). He had received medical treatment only for the last few years after being diagnosed with Eisenmenger syndrome. After cardiac catheterization, he received iloprost inhalation therapy...