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1.  Necrotizing enterocolitis in newborns: update in pathophysiology and newly emerging therapeutic strategies 
Korean Journal of Pediatrics  2014;57(12):505-513.
While the survival of extremely premature infants with respiratory distress syndrome has increased due to advanced respiratory care in recent years, necrotizing enterocolitis (NEC) remains the leading cause of neonatal mortality and morbidity. NEC is more prevalent in lower gestational age and lower birth weight groups. It is characterized by various degrees of mucosal or transmural necrosis of the intestine. Its exact pathogenesis remains unclear, but prematurity, enteral feeding, bacterial products, and intestinal ischemia have all been shown to cause activation of the inflammatory cascade, which is known as the final common pathway of intestinal injury. Awareness of the risk factors for NEC; practices to reduce the risk, including early trophic feeding with breast milk and following the established feeding guidelines; and administration of probiotics have been shown to reduce the incidence of NEC. Despite advancements in the knowledge and understanding of the pathophysiology of NEC, there is currently no universal prevention measure for this serious and often fatal disease. Therefore, new potential techniques to detect early biomarkers or factors specific to intestinal inflammation, as well as further strategies to prevent the activation of the inflammatory cascade, which is important for disease progression, should be investigated.
PMCID: PMC4316593  PMID: 25653683
Necrotizing enterocolitis; Extremely premature infants; Pathophysiology; Risk factor; Prevention
2.  BK virus-associated hemorrhagic cystitis after pediatric stem cell transplantation 
Korean Journal of Pediatrics  2014;57(12):514-519.
Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem cell transplantation late-onset hemorrhagic cystitis, pediatricians outside the hemato-oncology and nephrology specialties tend to be unfamiliar with hemorrhagic cystitis and the BK virus. Moreover, no standard guidelines for the early diagnosis and treatment of BK virus-associated hemorrhagic cystitis after stem cell transplantation have been established. Here, we briefly introduce poststem cell transplantation BK virus-associated hemorrhagic cystitis.
PMCID: PMC4316594  PMID: 25653684
Cystitis; Stem cell transplantation; BK virus; Child
3.  Lifestyle modifications in an adolescent dormitory: a clinical trial 
Korean Journal of Pediatrics  2014;57(12):520-525.
Childhood obesity is an increasing public health issue worldwide. We examined dietary patterns among adolescents in a dormitory school, identified obese adolescents and tried to intervene to improve food habits and physical activity.
We conducted an experimental prospective longitudinal study based on 36 obese (body mass index [BMI]≥95th percentile) adolescents (aged 12-18 years) compared with controls (healthy children: normal age-appropriate BMI (BMI≤85th percentile). Six months' intervention included lifestyle-modification counseling (once a week by a clinical dietician), and an exercise regimen twice a week, 60 minutes each time, instructed by a professional pediatric trainer). Both groups underwent baseline measurements at the beginning of the study and 6 months later (arterial stiffness, blood pressure, pulse, weight and height, hemoglobin, creatinine, liver enzymes, highly sensitive C-reactive protein and complete lipid profile).
Twenty-one participants completed the study. Low compliance from participants, school staff and parents was observed (participation in planned meetings; 71%-83%). BMI significantly decreased from 32.46±3.93 kg/m2 to 30.32±3.4 kg/m2 (P=0.002) in the study group. Arterial stiffness was not significantly different between the 2 groups and did not change significantly after 6 months' intervention (P=0.494). No significant changes in CRP and lipid profile were observed after the intervention.
Making lifestyle modifications among adolescents in a dormitory school is a complex task. Active intervention indeed ameliorates BMI parameters. However, in order to maximize the beneficial effects, a multidisciplinary well-trained team is needed, with emphasis on integrating parents and the school environment.
PMCID: PMC4316595  PMID: 25653685
Life style; Obesity; Adolescent; Arterial stiffness
4.  Associations of matrix metalloproteinase (MMP)-8, MMP-9, and their inhibitor, tissue inhibitor of metalloproteinase-1, with obesity-related biomarkers in apparently healthy adolescent boys 
Korean Journal of Pediatrics  2014;57(12):526-532.
Matrix metalloproteinases (MMPs) have been implicated in atherosclerosis, and therefore, are considered risk factors for metabolic dysfunction in adults. However, there is little data on circulating levels of MMPs and tissue inhibitors of MMPs (TIMPs) with regard to obesity-related biomarkers in the general adolescent population. In the present study, we determined the associations of MMP-8, MMP-9, and TIMP-1 levels and MMP-8/TIMP-1 and MMP-9/TIMP-1 ratios with obesity-related biomarkers in apparently healthy adolescent boys.
We measured MMP and TIMP concentrations in plasma samples using the enzyme-linked immunosorbent assay and analyzed their associations with obesity-related biomarkers, such as liver enzymes and lipid profiles, in a sample of 91 Korean boys aged 13-14 years who participated in a general health check-up.
The mean age of the boys was 13.8±0.3 years; 72 boys were normal weight and 19 were overweight/obese. The Pearson correlation coefficients revealed a significant correlation between MMP-8 and aspartate aminotransferase (r=0.217, P=0.039) and alanine aminotransferase (r=0.250, P=0.017) and between TIMP-1 and aspartate aminotransferase (r=0.267, P=0.011). In a multivariate linear regression analysis, serum alanine aminotransferase was positively associated with the MMP-8 level. There were no significant differences in the MMP-8, MMP-9, and TIMP-1 levels or MMP-8/TIMP-1 and MMP-9/TIMP-1 ratios between control and overweight/obese subjects.
We found a significant association between the MMP-8 level and alanine aminotransferase in the apparently healthy adolescent boys. These findings indicate that there may be a pathophysiological mechanism underlying the relationship between MMP-8 and liver enzymes in young adolescents.
PMCID: PMC4316596  PMID: 25653686
Adolescent; Alanine aminotransferase; Overweight; Matrix metalloproteinases; Tissue inhibitor of metalloproteinase
5.  The relationship between serum adiponectin and inflammatory cytokines in obese Korean juveniles 
Korean Journal of Pediatrics  2014;57(12):533-537.
Obesity is related to systemic inflammatory processes causing cardiovascular complications. Intercellular adhesion molecule-1 (ICAM-1), CD40 ligand (CD40L), P-selectin are newly described mediators of inflammation and have a significant effect in atherosclerosis. Adiponectin has shown anti-inflammatory effects in adults. The aim of this study was to evaluate the relationship between adiponectin and inflammatory mediators in children and adolescents.
Fifty children or adolescents, twenty two with a body mass index (BMI) over 95th percentile, and twenty eight with a BMI below 75th percentile were included in the study. Serum soluble ICAM-1 (sICAM-1), P-selectin, CD40L, lipid profiles, aspartate aminotransferase, alanine aminotransferase, glucose and insulin were measured to evaluate associations with adiponectin. Comparison of these variables was performed between the obese and the nonobese group.
We found a adiponectin to be significant lower and sICAM-1 significant higher in the obese group compared to the nonobese group, but there were no significant differences in P-selectin and soluble CD40L. Adiponectin was negatively associated with ICAM-1 and P-selectin in the obese group.
Negative associations of adiponectin with ICAM-1 and P-selectin in obese children and adolescents suggest that serum adiponectin level may represent the inflammatory status.
PMCID: PMC4316597  PMID: 25653687
Adiponectin; Intercellular adhesion molecule-1; CD40 ligand; P-selectin
6.  Necrotizing fasciitis and streptococcal toxic shock syndrome secondary to varicella in a healthy child 
Korean Journal of Pediatrics  2014;57(12):538-541.
Varicella is usually considered to be a benign disease in healthy children; however, serious complications can occur such as necrotizing fasciitis and toxic shock syndrome. We describe a 38-month-old girl with necrotizing fasciitis and streptococcal toxic shock syndrome following varicella. She was previously healthy and vaccinated against varicella at 12 months of age. She had been diagnosed with varicella three days prior to presenting at our facility; she developed fever, vomiting, and painful swelling on her left flank. Her skin lesions worsened, she became lethargic, and had episodes of hypotension and coagulopathy. Necrotizing fasciitis on the left abdominal wall, buttocks, and left thigh was diagnosed by magnetic resonance imaging, and group A Streptococcus was isolated from a tissue culture. She was diagnosed as necrotizing fasciitis and streptococcal toxic shock syndrome, and successfully treated with repeated surgical debridement and fasciotomy, in addition to intensive antibiotics. Our experience suggests that necrotizing fasciitis in patients with varicella should be considered to be a rare complication even with widespread vaccine use. Early diagnosis and intensive treatment are required to prevent a fatal outcome.
PMCID: PMC4316598  PMID: 25653688
Chickenpox; Necrotizing fasciitis; Streptococcus pyogenes; Child
7.  A pediatric case of Bickerstaff's brainstem encephalitis 
Korean Journal of Pediatrics  2014;57(12):542-545.
Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.
PMCID: PMC4316599  PMID: 25653689
Encephalitis; Miller Fisher syndrome; Ophthalmoplegia; Ataxia
8.  Current issues of pediatric inflammatory bowel disease in Korea 
Korean Journal of Pediatrics  2014;57(11):465-471.
Inflammatory bowel disease (IBD) is a chronic relapsing disorder of unknown etiology, which is believed to be multifactorial. Recently, the incidence of pediatric IBD has steeply increased in Korea since 2000. Poorly controlled disease activity can result in complications such as intestinal fistulae, abscess, and stricture, as well as growth retardation and delayed puberty in children. Because of a lack of confirmative tests, various diagnostic modalities must be used to diagnose IBD. Onset age, location, behavior, and activity are important in selecting treatments. Monogenic IBD must be excluded among infantile and refractory very-early-onset IBD. Early aggressive therapy using biologics has recently been proposed for peripubertal children to prevent growth failure and malnutrition.
PMCID: PMC4279006  PMID: 25550700
Inflammatory bowel disease; Child; Crohn disease; Ulcerative colitis
9.  Vascular health late after Kawasaki disease: implications for accelerated atherosclerosis 
Korean Journal of Pediatrics  2014;57(11):472-478.
Kawasaki disease (KD), an acute vasculitis that primarily affects young children, is the most common acquired paediatric cardiovascular disease in developed countries. While sequelae of arterial inflammation in the acute phase of KD are well documented, its late effects on vascular health are increasingly unveiled. Late vascular dysfunction is characterized by structural alterations and functional impairment in term of arterial stiffening and endothelial dysfunction and shown to involve both coronary and systemic arteries. Further evidence suggests that continuous low grade inflammation and ongoing active remodeling of coronary arterial lesions occur late after acute illness and may play a role in structural and functional alterations of the arteries. Potential importance of genetic modulation on vascular health late after KD is implicated by associations between mannose binding lectin and inflammatory gene polymorphisms with severity of peripheral arterial stiffening and carotid intima-media thickening. The changes in cholesterol and lipoproteins levels late after KD further appear similar to those proposed to be atherogenic. While data on adverse vascular health are less controversial in patients with persistent or regressed coronary arterial aneurysms, data appear conflicting in individuals with no coronary arterial involvements or only transient coronary ectasia. Notwithstanding, concerns have been raised with regard to predisposition of KD in childhood to accelerated atherosclerosis in adulthood. Until further evidence-based data are available, however, it remains important to assess and monitor cardiovascular risk factors and to promote cardiovascular health in children with a history of KD in the long term.
PMCID: PMC4279007  PMID: 25550701
Kawasaki disease; Blood vessels; Atherosclerosis
10.  A comparison of the efficacy of amoxicillin and nasal irrigation in treatment of acute sinusitis in children 
Korean Journal of Pediatrics  2014;57(11):479-483.
The efficacy of antibiotic therapy for acute sinusitis is controversial. This study aimed to compare the efficacies of amoxicillin with nasal irrigation and nasal irrigation alone for acute sinusitis in children.
This randomized, double-blind, controlled study included 80 children aged 4-15 years with a clinical presentation of acute sinusitis. Patients were randomly assigned to receive either amoxicillin (80 mg/kg/day) in 3 divided doses orally for 14 days with saline nasal irrigation (for 5 days) and 0.25% phenylephrine (for 2 days) or the same treatment without amoxicillin. Clinical improvements in their initial symptoms were assessed on days 3, 14, 21, and 28.
On day 3, patients in the amoxicillin with nasal irrigation group showed significant clinical improvement (P=0.001), but there was no significant difference in the degree of improvement between the amoxicillin with nasal irrigation and nasal irrigation alone groups during follow-up (P>0.05). In addition, no significant differences were seen in age, sex, and degree of improvement between groups (P>0.05).
High-dose amoxicillin with saline nasal irrigation relieved acute sinusitis symptoms faster and more often than saline nasal irrigation alone. However, antibiotic treatment for acute sinusitis confers only a small therapeutic benefit over nasal irrigation.
PMCID: PMC4279008  PMID: 25550702
Sinusitis; Acute; Amoxicillin; Child; Nasal lavage
11.  Effect of adenotonsillar hypertrophy on right ventricle function in children 
Korean Journal of Pediatrics  2014;57(11):484-488.
Chronic upper airway obstruction causes hypoxemic pulmonary vasoconstriction, which may lead to right ventricle (RV) dysfunction. Adenotonsillar hypertrophy (ATH) is the most common cause of upper airway obstruction in children. Therefore, we aimed to evaluate RV function in children with ATH.
Twenty-one children (male/female, 15/6; mean age, 92.3¡¾39.0 months; age range, 4-15 years) with ATH and 21 healthy age- and gender-matched controls were included in this study. Tricuspid annular plane systolic excursion and RV myocardial performance index were measured by transthoracic echocardiography. Further, the plasma level of N-terminal of probrain natriuretic peptide (NT-proBNP), an indicator of RV function, was determined.
The snoring-tiredness during daytime-observed apnea-high blood pressure (STOP) questionnaire was completed by the patients' parents, and loud snoring was noted in the ATH group. The plasma NT-proBNP level was significantly higher in the ATH group than that in the controls (66.44±37.63 pg/mL vs. 27.85±8.89 pg/mL, P=0.001). The echocardiographic parameters were not significantly different between the groups.
We were unable to confirm the significance of echocardiographic evidence of RV dysfunction in the management of children with ATH. However, the plasma NT-proBNP level was significantly higher in the ATH group than that in the control, suggesting that chronic airway obstruction in children may carry a risk for cardiac dysfunction. Therefore, more patients should be examined using transthoracic echocardiography. In addition, pediatricians and otolaryngologists should consider cardiologic aspects during the management of children with severe ATH.
PMCID: PMC4279009  PMID: 25550703
Hypertrophy; Obstructive sleep apnea; Airway obstruction; Echocardiography; Probrain natriuretic peptide
12.  Clinical features and short-term outcomes of pediatric acute fulminant myocarditis in a single center 
Korean Journal of Pediatrics  2014;57(11):489-495.
The aims of this study were to document our single-center experience with pediatric acute fulminant myocarditis (AFM) and to investigate its clinical features and short-term outcomes.
We performed a retrospective chart review of all children <18 years old who were diagnosed with AFM between October 2008 and February 2013. Data about patient demographics, initial symptoms, investigation results, management, and outcomes between survivors and nonsurvivors were collected.
Seventeen of 21 patients (80.9%) with myocarditis were diagnosed with AFM. Eleven patients (64.7%) survived to discharge, and 6 (35.3%) died. Electrocardiography on admission revealed dysrhythmia in 10 patients (58.8%); of these, all 7 patients with a complete atrioventricular block survived. Fractional shortening upon admission was significantly different between the survivors (16%) and nonsurvivors (8.5%) (P=0.01). Of the serial biochemical markers, only the initial brain natriuretic peptide (P=0.03) and peak blood urea nitrogen levels (P=0.02) were significantly different. Of 17 patients, 4 (23.5%) required medical treatment only. Extracorporeal membrane oxygenation (ECMO) was performed in 13 patients (76.5%); the survival rate in these patients was 53.8%. ECMO support was initiated >24 hours after admission in 4 of the 13 patients (30.7%), and 3 of those 4 patients (75%) died.
AFM outcomes may be associated with complete atrioventricular block upon hospital admission, left ventricular fractional shortening at admission, time from admission to the initiation of ECMO support, initial brain natriuretic peptide level, and peak blood urea nitrogen level.
PMCID: PMC4279010  PMID: 25550704
Myocarditis; Extracorporeal membrane oxygenation; Child; Outcomes
13.  Wernicke's encephalopathy in a child with high dose thiamine therapy 
Korean Journal of Pediatrics  2014;57(11):496-499.
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 showed gait disturbances, nystagmus, and excessive dizziness; his mental state, however, indicated he was alert, which did not fit the classical triad of Wernicke's encephalopathy. A diagnosis of Wernicke's encephalopathy was made only after brain magnetic resonance imaging and serum thiamine level analyses were performed. The patient's symptoms remained after 5 days of treatment with 100-mg thiamine once daily; thus, we increased the dosage to 500 mg 3 times daily, 1,500 mg per day. His symptoms then improved after 20 days of replacement therapy. This case report describes a pediatric patient who was promptly diagnosed with Wernicke's encephalopathy, despite only 2 suspicious symptoms, and who completely recovered after high doses of thiamine were given intravenously.
PMCID: PMC4279011  PMID: 25550705
Wernicke encephalopathy; Thiamine deficiency; Parenteral nutrition
14.  Multifocal kaposiform hemangioendothelioma of soft tissue with bilateral pulmonary involvement in an adolescent 
Korean Journal of Pediatrics  2014;57(11):500-504.
Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.
PMCID: PMC4279012  PMID: 25550706
Kaposiform hemangioendothelioma; Bone; Pulmonary neoplasm; Multifocal; Adolescent
15.  Adrenal and thyroid function in the fetus and preterm infant 
Korean Journal of Pediatrics  2014;57(10):425-433.
Adrenal and thyroid hormones are essential for the regulation of intrauterine homeostasis, and for the timely differentiation and maturation of fetal organs. These hormones play complex roles during fetal life, and are believed to underlie the cellular communication that coordinates maternal-fetal interactions. They serve to modulate the functional adaptation for extrauterine life during the perinatal period. The pathophysiology of systemic vasopressor-resistant hypotension is associated with low levels of circulating cortisol, a result of immaturity of hypothalamic-pituitary-adrenal axis in preterm infants under stress. Over the past few decades, studies in preterm infants have shown abnormal clinical findings that suggest adrenal or thyroid dysfunction, yet the criteria used to diagnose adrenal insufficiency in preterm infants continue to be arbitrary. In addition, although hypothyroidism is frequently observed in extremely low gestational age infants, the benefits of thyroid hormone replacement therapy remain controversial. Screening methods for congenital hypothyroidism or congenital adrenal hyperplasia in the preterm neonate are inconclusive. Thus, further understanding of fetal and perinatal adrenal and thyroid function will provide an insight into the management of adrenal and thyroid function in the preterm infant.
PMCID: PMC4219944  PMID: 25379042
Premature infant; Thyroid; Adrenal glands
16.  Advancements in the treatment of pediatric acute leukemia and brain tumor - continuous efforts for 100% cure 
Korean Journal of Pediatrics  2014;57(10):434-439.
Treatment outcomes of pediatric cancers have improved greatly with the development of improved treatment protocols, new drugs, and better supportive measures, resulting in overall survival rates greater than 70%. Survival rates are highest in acute lymphoblastic leukemia, reaching more than 90%, owing to risk-based treatment through multicenter clinical trials and protocols developed to prevent central nervous system relapse and testicular relapse in boys. New drugs including clofarabine and nelarabine are currently being evaluated in clinical trials, and other targeted agents are continuously being developed. Chimeric antigen receptor-modified T cells are now attracting interest for the treatment of recurrent or refractory disease. Stem cell transplantation is still the most effective treatment for pediatric acute myeloid leukemia (AML). However, in order to reduce treatment-related death after stem cell transplantation, there is need for improved treatments. New drugs and targeted agents are also needed for improved outcome of AML. Surgery and radiation therapy have been the mainstay for brain tumor treatment. However, chemotherapy is becoming more important for patients who are not eligible for radiotherapy owing to age. Stem cell transplant as a means of high dose chemotherapy and stem cell rescue is a new treatment modality and is often repeated for improved survival. Drugs such as temozolomide are new chemotherapeutic options. In order to achieve 100% cure in children with pediatric cancer, every possible treatment modality and effort should be considered.
PMCID: PMC4219945  PMID: 25379043
Pediatrics; Neoplasms; Survival rate; Leukemia; Brain neoplasms
17.  Serum interleukin-1beta and tumor necrosis factor-alpha in febrile seizures: is there a link? 
Korean Journal of Pediatrics  2014;57(10):440-444.
Febrile seizures are induced by fever and are the most common type of seizures in children. Although numerous studies have been performed on febrile seizures, their pathophysiology remains unclear. Recent studies have shown that cytokines may play a role in the pathogenesis of febrile seizures. The present study was conducted to identify potential links between serum interleukin-1beta (IL-1β), tumor necrosis factor-alpha (TNF-α), and febrile seizures.
Ninety-two patients with simple or complex febrile seizures (46 patients per seizure type), and 46 controls with comparable age, sex, and severity of temperature were enrolled.
The median concentrations of serum IL-1β in the simple, complex febrile seizure, and control groups were 0.05, 0.1, and 0.67 pg/mL, respectively (P=0.001). Moreover, the median concentrations of TNF-α in the simple, complex febrile seizure, and control groups were 2.5, 1, and 61.5 pg/mL, respectively (P=0.001). Furthermore, there were significant differences between the case groups in serum IL-1β and TNF-α levels (P<0.05).
Unlike previous studies, our study does not support the hypothesis that increased IL-1β and TNF-α production is involved in the pathogenesis of febrile seizures.
PMCID: PMC4219946  PMID: 25379044
Interleukin-1beta; Tumor necrosis factor-alpha; Febrile seizures
18.  The large-conductance calcium-activated potassium channel holds the key to the conundrum of familial hypokalemic periodic paralysis 
Korean Journal of Pediatrics  2014;57(10):445-450.
Familial hypokalemic periodic paralysis (HOKPP) is an autosomal dominant channelopathy characterized by episodic attacks of muscle weakness and hypokalemia. Mutations in the calcium channel gene, CACNA1S, or the sodium channel gene, SCN4A, have been found to be responsible for HOKPP; however, the mechanism that causes hypokalemia remains to be determined. The aim of this study was to improve the understanding of this mechanism by investigating the expression of calcium-activated potassium (KCa) channel genes in HOKPP patients.
We measured the intracellular calcium concentration with fura-2-acetoxymethyl ester in skeletal muscle cells of HOKPP patients and healthy individuals. We examined the mRNA and protein expression of KCa channel genes (KCNMA1, KCNN1, KCNN2, KCNN3, and KCNN4) in both cell types.
Patient cells exhibited higher cytosolic calcium levels than normal cells. Quantitative reverse transcription polymerase chain reaction analysis showed that the mRNA levels of the KCa channel genes did not significantly differ between patient and normal cells. However, western blot analysis showed that protein levels of the KCNMA1 gene, which encodes KCa1.1 channels (also called big potassium channels), were significantly lower in the membrane fraction and higher in the cytosolic fraction of patient cells than normal cells. When patient cells were exposed to 50 mM potassium buffer, which was used to induce depolarization, the altered subcellular distribution of BK channels remained unchanged.
These findings suggest a novel mechanism for the development of hypokalemia and paralysis in HOKPP and demonstrate a connection between disease-associated mutations in calcium/sodium channels and pathogenic changes in nonmutant potassium channels.
PMCID: PMC4219947  PMID: 25379045
Channelopathies; Hypokalemic periodic paralysis; Potassium channels
19.  Serum procalcitonin as a diagnostic marker of neonatal sepsis 
Korean Journal of Pediatrics  2014;57(10):451-456.
We evaluated serum procalcitonin (PCT) as a diagnostic marker of neonatal sepsis, and compared PCT levels with C-reactive protein (CRP) levels.
We retrospectively reviewed the medical records of 269 neonates with a suspected infection, admitted to Wonkwang University School of Medicine & Hospital between January 2011 and December 2012, for whom PCT and CRP values had been obtained. Neonates were categorized into 4 groups according to infection severity. CRP and PCT values were analyzed and compared, and their effectiveness as diagnostic markers was determined by using receiver operating characteristic (ROC) curve analysis. We also calculated the sensitivity, specificity, and positive, and negative predictive values.
The mean PCT and CRP concentrations were respectively 56.27±81.89 and 71.14±37.17 mg/L in the "confirmed sepsis" group; 15.64±32.64 and 39.23±41.41 mg/L in the "suspected sepsis" group; 9.49±4.30 and 0.97±1.16 mg/L in the "mild infection" group; and 0.21±0.12 and 0.72±0.7 mg/L in the control group. High concentrations indicated greater severity of infection (P<0.001). Five of 18 patients with confirmed sepsis had low PCT levels (<1.0 mg/L) despite high CRP levels. In the ROC analysis, the area under the curve was 0.951 for CRP and 0.803 for PCT. The cutoff concentrations of 0.5 mg/L for PCT and 1.0 mg/L for CRP were optimal for diagnosing neonatal sepsis (sensitivity, 88.29% vs. 100%; specificity, 58.17% vs. 85.66%; positive predictive value, 13.2% vs. 33.3%; negative predictive value, 98.6% vs. 100%, respectively).
PCT is a highly effective early diagnostic marker of neonatal infection. However, it may not be as reliable as CRP.
PMCID: PMC4219948  PMID: 25379046
Sepsis; Procalcitonin; C-reactive protein
20.  Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus 
Korean Journal of Pediatrics  2014;57(10):457-460.
A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barré syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.
PMCID: PMC4219949  PMID: 25379047
Bickerstaff brainstem encephalitis; Guillain-Barré syndrome; Epstein-Barr virus
21.  Inhaled iloprost for the treatment of patient with Fontan circulation 
Korean Journal of Pediatrics  2014;57(10):461-463.
Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.
PMCID: PMC4219950  PMID: 25379048
Fontan operation; Vascular resistance; Iloprost
22.  Efficacy and safety of growth hormone treatment for children born small for gestational age 
Korean Journal of Pediatrics  2014;57(9):379-383.
Recombinant growth hormone (GH) is an effective treatment for short children who are born small for gestational age (SGA). Short children born SGA who fail to demonstrate catch-up growth by 2-4 years of age are candidates for GH treatment initiated to achieve catch-up growth to a normal height in early childhood, maintain a normal height gain throughout childhood, and achieve an adult height within the normal target range. GH treatment at a dose of 35-70 µg/kg/day should be considered for those with very marked growth retardation, as these patients require rapid catch-up growth. Factors associated with response to GH treatment during the initial 2-3 years of therapy include age and height standard deviation scores at the start of therapy, midparental height, and GH dose. Adverse events due to GH treatment are no more common in the SGA population than in other conditions treated with GH. Early surveillance in growth clinics is strongly recommended for children born SGA who have not caught up. Although high dose of up to 0.067 mg/kg/day are relatively safe for short children with growth failure, clinicians need to remain aware of long-term mortality and morbidity after GH treatment.
PMCID: PMC4198952  PMID: 25324863
Infant; Small for gestational age infant; Growth hormone; Treatment outcome; Safety
23.  Febrile seizures 
Korean Journal of Pediatrics  2014;57(9):384-395.
Febrile seizure (FS) is the most common seizure disorder of childhood, and occurs in an age-related manner. FS are classified into simple and complex. FS has a multifactorial inheritance, suggesting that both genetic and environmental factors are causative. Various animal models have elucidated the pathophysiological mechanisms of FS. Risk factors for a first FS are a family history of the disorder and a developmental delay. Risk factors for recurrent FS are a family history, age below 18 months at seizure onset, maximum temperature, and duration of fever. Risk factors for subsequent development of epilepsy are neurodevelopmental abnormality and complex FS. Clinicians evaluating children after a simple FS should concentrate on identifying the cause of the child's fever. Meningitis should be considered in the differential diagnosis for any febrile child. A simple FS does not usually require further evaluation such as ordering electroencephalography, neuroimaging, or other studies. Treatment is acute rescue therapy for prolonged FS. Antipyretics are not proven to reduce the recurrence risk for FS. Some evidence shows that both intermittent therapy with oral/rectal diazepam and continuous prophylaxis with oral phenobarbital or valproate are effective in reducing the risk of recurrence, but there is no evidence that these medications reduce the risk of subsequent epilepsy. Vaccine-induced FS is a rare event that does not lead to deleterious outcomes, but could affect patient and physician attitudes toward the safety of vaccination.
PMCID: PMC4198953  PMID: 25324864
Febrile seizures; Classification; Child; Epilepsy
24.  Outbreaks of mumps: an observational study over two decades in a single hospital in Korea 
Korean Journal of Pediatrics  2014;57(9):396-402.
The introduction of the mumps vaccine has dramatically reduced the number of mumps cases, but outbreaks have recently occurred among highly vaccinated populations in developed countries. Epidemiological and clinical characteristics of patients with mumps admitted between 1989 and 2012 in a single hospital in Korea are described in the present study.
We retrospectively evaluated inpatients with mumps between 1989 and 2012 and outpatients and inpatients with mumps in 2011-2012.
A total of 152 patients with mumps were admitted between 1989 and 2012, and 163 patients were recorded in 2011-2012. The highest number of admitted cases occurred in 1998 and 2012 (35 and 34 cases, respectively). Among the patients admitted in 2011-2012, the highest frequency was observed among people aged 15-19 years, and low frequency was observed in those aged <4 years and >20 years, compatible to the city data and national data. In patients admitted to our department in 1998 (35 cases) and in 2010-2012 (27 cases), there were significant differences in the mean age and the rate of secondary measles-mumps-rubella (MMR) vaccination, but had similar clinical features, including complications, except aseptic meningitis. Antimumps immunoglobulin (Ig) G was positive in 83% and 100%, and IgM was positive in 67% and 41%, respectively, in the two periods.
In Korea, recent mumps outbreaks have occurred mainly among secondary school students who received two doses of the MMR vaccine. The vaccinees might have a modified immune reaction to viral insults, manifesting modified epidemiological and clinical features.
PMCID: PMC4198954  PMID: 25324865
Mumps; Disease outbreaks; Measles-mumps-rubella vaccine; Antibodies; Immnoglobulin G; Immunoglobulin M
25.  Correlation between the morning hypertension on ambulatory blood pressure monitoring and the left ventricular mass in children 
Korean Journal of Pediatrics  2014;57(9):403-409.
Although high morning blood pressure (BP) is known to be associated with the onset of cardiovascular events in adults, data on its effects in children with hypertension are limited. Our retrospective study aimed to define the clinical characteristics of children with morning hypertension (MH) and to determine its associated factors.
We reviewed 31 consecutive patients with hypertension, confirmed by the ambulatory blood pressure monitoring (ABPM). We divided these patients into 2 groups: the MH group (n=21, 67.7%), morning BP above the 95th percentile for age and height (2 hours on average after waking up) and the normal morning BP group (n=10, 32.3%). We compared the clinical manifestations, laboratory results, and echocardiographic findings including left ventricular hypertrophy (LVH) between the groups.
The early/atrial (E/A) mitral flow velocity ratio in the MH group was significantly lower than that in the normal morning BP group. In addition, LV mass was higher in the MH group than in the normal morning BP group, although the difference was not statistically significant. The age at the time of hypertension diagnosis was significantly higher in the MH group than in the normal morning BP group (P=0.003). The incidence of hyperuricemia was significantly higher in the MH group than in the normal morning BP group.
Older patients and those with hyperuricemia are at higher risk for MH. The rise in BP in the morning is an important factor influencing the development of abnormal relaxation, as assessed by echocardiography. Clinical trials with longer follow-up periods and larger sample sizes are needed to clarify the clinical significance of MH.
PMCID: PMC4198955  PMID: 25324866
Ambulatory blood pressure monitoring; Hypertension; Left ventricular hypertrophy

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