The Author's Response: Genetic Contributions to Childhood Obesity: Association of Candidate Gene Polymorphisms and Overweight/Obesity in Korean Preschool Children

No abstract available.

The influencing factors on procalcitonin values in newborns with noninfectious conditions during the first week of life / 소아과

PURPOSE: Although procalcitonin (PCT) level is useful for the diagnosis of neonatal sepsis, PCT reliability is inconsistent because of the varied conditions encountered in neonatal intensive care units. This study aimed to investigate PCT levels and factors influencing increased PCT levelin newborns without bacterial infection during the first week of life. METHODS: In newborns hospitalized between March 2013 and October 2015, PCT levels were measured on the first, third, and seventh days after birth. Newborns with proven bacterial (blood culture positive for bacteria) or suspicious infection (presence of C-reactive protein expression or leukocytosis/leukopenia) were excluded. Various neonatal conditions were analyzed to identify the factors influencing increased PCT level. RESULTS: Among 292 newborns with a gestational age of 35.2±3.0 weeks and a birth weight of 2,428±643 g, preterm newborns (n=212) had higher PCT levels than term newborns (n=80). Of the newborns, 7.9% had increased PCT level (23 of 292) on the firstday; 28.3% (81 of 286), on the third day; and 3.3% (7 of 121), on the seventh day after birth. The increased PCT level was significantly associated with prenatal disuse of antibiotics (P=0.004) and surfactant administration (P<0.001) on the first day after birth, postnatal use of antibiotics (P=0.001) and ventilator application (P=0.001) on the third day after birth, and very low birth weight (P=0.042) on the seventh day after birth. CONCLUSION: In newborns without bacterial infection, increased PCT level was significantly associated with lower gestational age and respiratory difficulty during the first week of life. Further studies are needed for clinical applications.

The influencing factors on procalcitonin values in newborns with noninfectious conditions during the first week of life / 소아과

PURPOSE: Although procalcitonin (PCT) level is useful for the diagnosis of neonatal sepsis, PCT reliability is inconsistent because of the varied conditions encountered in neonatal intensive care units. This study aimed to investigate PCT levels and factors influencing increased PCT levelin newborns without bacterial infection during the first week of life. METHODS: In newborns hospitalized between March 2013 and October 2015, PCT levels were measured on the first, third, and seventh days after birth. Newborns with proven bacterial (blood culture positive for bacteria) or suspicious infection (presence of C-reactive protein expression or leukocytosis/leukopenia) were excluded. Various neonatal conditions were analyzed to identify the factors influencing increased PCT level. RESULTS: Among 292 newborns with a gestational age of 35.2±3.0 weeks and a birth weight of 2,428±643 g, preterm newborns (n=212) had higher PCT levels than term newborns (n=80). Of the newborns, 7.9% had increased PCT level (23 of 292) on the firstday; 28.3% (81 of 286), on the third day; and 3.3% (7 of 121), on the seventh day after birth. The increased PCT level was significantly associated with prenatal disuse of antibiotics (P=0.004) and surfactant administration (P<0.001) on the first day after birth, postnatal use of antibiotics (P=0.001) and ventilator application (P=0.001) on the third day after birth, and very low birth weight (P=0.042) on the seventh day after birth. CONCLUSION: In newborns without bacterial infection, increased PCT level was significantly associated with lower gestational age and respiratory difficulty during the first week of life. Further studies are needed for clinical applications.

Individualized ibuprofen treatment using serial B-type natriuretic peptide measurement for symptomatic patent ductus arteriosus in very preterm infants / 소아과

PURPOSE: Plasma level of B-type natriuretic peptide (BNP), an emerging, sensitive, and specific biomarker of hemodynamically significant patent ductus arteriosus (PDA), rapidly decreases in infants receiving cyclooxygenase inhibitors for ductal closure. We investigated the usefulness of serial BNP measurement as a guide for individual identification of early constrictive responses to ibuprofen in preterm infants with symptomatic PDA (sPDA). METHODS: Before March 2010, the standard course of pharmacological treatment was initiated with indomethacin (or ibuprofen) and routinely followed by 2 additional doses at intervals of 24 hours. After April 2010, individualized pharmacological treatment was used, starting with the first dose of ibuprofen and withholding additional ibuprofen doses if the BNP concentration was <600 pg/mL and clinical symptoms of PDA improved. RESULTS: The BNP-guided group received significantly fewer doses of ibuprofen than the standard group did during the first course of treatment and the entire study period. The need for further doses of cyclooxygenase inhibitors and for surgical ligation was not significantly different between the 2 groups. No significant differences were seen in clinical outcomes and/or complications related to sPDA and/or pharmacological treatment. CONCLUSION: Individualized BNP-guided pharmacological treatment may be used clinically to avoid unnecessary doses of cyclooxygenase inhibitors without increasing the ductal closure failure and the short-term morbidity related to sPDA.

Genetic Contributions to Childhood Obesity: Association of Candidate Gene Polymorphisms and Overweight/Obesity in Korean Preschool Children

This study was aimed to investigate the association of candidate gene polymorphisms and obesity or overweight in young Korean children. A total of 190 Korean preschool children (96 control, 48 overweight, and 46 obese children) were genotyped for the angiotensin converting enzyme (ACE) insertion (I)/deletion (D), angiotensin II type 2 receptor (AT2) C3123A, transforming growth factor (TGF)-β1 T869C, vascular endothelial growth factor (VEGF) T460C, and tumor necrosis factor (TNF)-α G308A polymorphisms. No differences were found among the groups with respect to age, sex, birth weight, blood pressure levels, and serum concentrations of glucose and total cholesterol. Obese children showed a higher incidence of ACE DD genotype and D allelic frequency compared to the controls (odds ratio [OR], 2.7, 95% confidence interval [CI], 1.01–7.21; OR, 2.5, 95% CI, 1.49–4.19; all P < 0.05). The frequency of TC genotype and C allele in the TGF-β1 T869C polymorphism (OR, 2.08, 95% CI, 1.01–4.27; OR, 1.93, 95% CI, 1.15–3.21) and that in the VEGF T460C polymorphism (OR, 2.5, 95% CI, 1.19–5.28; OR, 2.15, 95% CI, 1.26–3.68) was also higher in obese children than in control subjects (all P < 0.05). Overweight children exhibited a higher frequency of the A allele in the AT2 C3123A polymorphism compared to the controls (OR, 1.72, 95% CI, 1.03–2.88, P < 0.05). There were no differences in the TNF-α G308A polymorphism among the groups. The ACE I/D, AT2 C3123A, TGF-β1 T869C, and VEGF T460C polymorphisms can affect susceptibility to obesity or overweight in Korean children.

Impaired angiogenesis in the enalapril-treated neonatal rat kidney / 소아과

PURPOSE: Nephrogenesis is normally accompanied by a tightly regulated and efficient vascularization. We investigated the effect of angiotensin II inhibition on angiogenesis in the developing rat kidney. METHODS: Newborn rat pups were treated with enalapril (30 mg/kg/day) or vehicle (control) for 7 days after birth. Renal histological changes were checked using Hematoxylin & Eosin staining. We also investigated the intrarenal expression of vascular endothelial growth factor (VEGF)-A, VEGF receptor 1 (VEGFR1), VEGFR2, platelet-derived growth factor (PDGF)-B, and PDGF receptor-beta with Western blotting and immunohistochemical staining at postnatal day 8. Expression of the endothelial cell marker CD31 was examined to determine glomerular and peritubular capillary density. RESULTS: Enalapril-treated rat kidneys showed disrupted tubules and vessels when compared with the control rat kidneys. In the enalapril-treated group, intrarenal VEGF-A protein expression was significantly higher, whereas VEGFR1 protein expression was lower than that in the control group (P<0.05). The expression of VEGFR2, PDGF-B, and PDGF receptor-beta was not different between the 2 groups. The increased capillary CD31 expression on the western blots of enalapril-treated rat kidneys indicated that the total endothelial cell protein level was increased, while the cortical capillary density, assessed using CD31 immunohistochemical staining, was decreased. CONCLUSION: Impaired VEGF-VEGFR signaling and altered capillary repair may play a role in the deterioration of the kidney vasculature after blocking of angiotensin II during renal development.

Successful Peritoneal Dialysis in an Extremely Preterm Infant

Peritoneal dialysis can be considered renal supportive therapy, even in an extremely low birth weight infant with acute kidney injury not responding to general supportive measures. Although there have been several reports of successful peritoneal dialysis in extremely low birth weight infants, general practice guidelines and commercially available optimal peritoneal dialysis catheters have not been introduced. We report a successful case of peritoneal dialysis in an extremely low birth weight infant born at 25 weeks gestational age, with birth weight 790 g, with uncontrollable metabolic acidosis, hyperkalemia, progressive azotemia and continued anuria.

Intraspinal and Urogenital Abnormalities in Infants with Sacral Cutaneous Lesions

PURPOSE: Sacral cutaneous lesions, such as dimples and hairy patches, may be associated with occult spinal dysraphism and urogenital abnormalities. This study aim to delineate high risk infants who need early screening for intraspinal and urogenital abnormalities by identifying the association between cutaneous lesions and combined abnormalities. METHODS: Sacral ultrasonography was performed in 777 infants with sacral cutaneous lesions from January 2010 to July 2014. Of these, 317 infants underwent abdominal ultrasonography for urogenital abnormalities. We reviewed the patient's medical records and radiographic findings retrospectively. RESULTS: Of the 777 infants, abnormal intraspinal findings such as tethered cord or meningocele were reported in 26 (3.4%). Sixteen of these 26 patients with abnormal findings underwent follow-up ultrasonography or MRI; 4 infants were diagnosed with lipomeningomyelocele through MRI, and 1 infant underwent a neurosurgical procedure. Among the 317 infants who underwent abdominal ultrasonography, 78 infants (24.6%) had congenital hydronephrosis and 8 infants (2.5%) had other urogenital abnormalities including duplication of kidney, vesicoureteral reflux, horseshoe kidney, renal cyst, or multicystic dysplastic kidney. Urogenital abnormalities were more common in patients with sacral dimples associated with hair or deviated gluteal folds than in those with simple dimples (OR 3.24 and 8.88; P=0.007 and P=0.001, respectively). CONCLUSION: Midline sacral cutaneous lesions may be associated with occult spinal dysraphism and urogenital abnormalities. To detect intraspinal lesions, ultrasonography is recommended for infants with sacral cutaneous lesions. Likewise, infants with sacral dimples associated with either hair or deviated gluteal folds, should be evaluated via abdominal ultrasonography to assess for combined urogenital abnormalities.

A case of CHARGE syndrome featuring immunodeficiency and hypocalcemia

CHARGE syndrome (coloboma, heart defects, atresia choanae, retarded growth and development, genital hypoplasia, and ear abnormalities) is characterized by multiple malformations and is diagnosed using distinct consensus criteria. Mutations in the gene encoding chromodomain helicase DNA-binding protein 7 (CHD7) are the major cause of CHARGE syndrome. Clinical features of CHARGE syndrome considerably overlap those of 22q11.2 deletion syndrome. Of these features, immunodeficiency and hypocalcemia are frequently reported in patients with 22q11.2 deletion syndrome but are rarely reported in patients with CHARGE syndrome. In this report, we have described the case of a patient with typical phenotypes of 22q11.2 deletion syndrome but without the proven chromosome microdeletion. Mutation analysis of CHD7 identified a pathogenic mutation (c.2238+1G>A) in this patient. To our knowledge, this is the first case of CHARGE syndrome with immunodeficiency and hypocalcemia in Korea. Our observations suggest that mutation analysis of CHD7 should be performed for patients showing the typical phenotypes of 22q11.2 deletion syndrome but lacking the proven chromosome microdeletion.

Application of Array-Based Comparative Genomic Hybridization to Pediatric Neurologic Diseases

PURPOSE: Array comparative genomic hybridization (array-CGH) is a technique used to analyze quantitative increase or decrease of chromosomes by competitive DNA hybridization of patients and controls. This study aimed to evaluate the benefits and yield of array-CGH in comparison with conventional karyotyping in pediatric neurology patients. MATERIALS AND METHODS: We included 87 patients from the pediatric neurology clinic with at least one of the following features: developmental delay, mental retardation, dysmorphic face, or epilepsy. DNA extracted from patients and controls was hybridized on the Roche NimbleGen 135K oligonucleotide array and compared with G-band karyotyping. The results were analyzed with findings reported in recent publications and internet databases. RESULTS: Chromosome imbalances, including 9 cases detected also by G-band karyotyping, were found in 28 patients (32.2%), and at least 19 of them seemed to be causally related to the abnormal phenotypes. Regarding each clinical symptom, 26.2% of 42 developmental delay patients, 44.4% of 18 mental retardation patients, 42.9% of 28 dysmorphic face patients, and 34.6% of 26 epilepsy patients showed abnormal array results. CONCLUSION: Although there were relatively small number of tests in patients with pediatric neurologic disease, this study demonstrated that array-CGH is a very useful tool for clinical diagnosis of unknown genome abnormalities performed in pediatric neurology clinics.

Partial Retraction: Difference of Anxiety of Parents: before & after the VCUG

No abstract available.

Neonatal Transport and Regionalization of Neonatal Intensive Care: The Perspective of Transferred Preterm Infants in a Single Neonatal Intensive Care Unit of the South-western Area of Gyeonggi-do / 대한주산의학회잡지

PURPOSE: Not only regionalization of neonatal care for high risk newborn, but also safe neonatal transport system of newborn were not completely established in Korea. The aim of this study was to compare the clinical outcomes of preterm infants less than 35 week gestational age with regard to inborn and outborn status, to understand the problems of regionalization of neonatal care and neonatal transport system and to provide the basis to solve the potential problems. METHODS: This retrospective study included 40 outborn and 40 inborn preterm infants less than 35 week gestational age admitted to the neonatal intensive care unit of Korea University Ansan Hospital during the period between January 2006 and June 2013. RESULTS: Compared with those in the inborn group, the incidences of hypoglycemia and respiratory distress were significantly more frequent in the outborn group at admission. The uses of surfactant, ventilator, and inotrope were significantly more frequent in the outborn during hospitalization. Mortality occurred only in the outborn group. Most of infants were transferred by a nurse alone, not a team with doctor. CONCLUSION: Transferred preterm infants may not be sufficiently stabilized before transport, according to the result of more frequent hypoglycemia and respiratory distress in the outborn group. In order to reduce mortality and morbidity of transferred newborn from level I, the national policy about neonatal intensive care unit level guideline (manpower, equipment, and facility), regionalization of neonatal intensive care, and neonatal transport system are needed in Korea.

Myocardial Injury in Newborn Infants with Severe Metabolic Acidosis at the First Day of Life

PURPOSE: Severe acidosis by various causes can lead to multi-organ dysfunction including cardiac dysfunction (CD) due to myocardial injury. The aims of this study are to investigate CD in newborn infants with severe acidemia at the first day of life and to evaluate the effect on myocardial injury according to the type and the severity of acidosis. METHODS: This was a retrospective study of infants > or =34 weeks of gestational age with severe acidemia (pH60 mmHg) and non-RA group (pCO2 or =-10) were classified based on the degree of BE. RESULTS: The levels of CK-MB and myoglobin had significant negative relation with BE. CD group had higher incidences of seizure and mortality and higher levels of CK-MB and cTnI than those of non-CD group. Severe BE group had higher incidences of CD and seizure and higher levels of CK-MB, myoglobin and cTnI than those of mild BE group. Non-RA group had higher levels of CK-MB and myoglobin than those of RA group. CONCLUSION: At the first day of life, the newborn infants with severe metabolic acidosis have high incidences of CD and myocardial injury. Aggressive monitoring and appropriate treatment for CD according to myocardial injury should be recommended in the newborn infants with severe metabolic acidosis.

The Clinical Comparison between Monomicrobial and Polymicrobial Urinary Infection in Febrile Pediatric Acute Pyelonephritis

PURPOSE: We investigated the clinical presentation of febrile pediatric patients with acute pyelonephritis (APN) with a mixed urine culture from an aseptic urine sample, and compared with that of those with a single culture. METHODS: We retrospectively reviewed the medical charts of 95 patients diagnosed as APN with fever between January 2008 and October 2010 at Korea University Medical Center. We classified the patients with APN into two groups with a positive single culture (S group) and a positive mixed culture (M group) from an aseptic urine sample of suprapubic bladder aspiration or urethral catheterization and compared the fever duration, laboratory markers such as serum white blood cell (WBC) counts and C-reactive protein (CRP) values in peripheral blood, and the presence of hydronephrosis, renal scar and vesicoureteral reflux (VUR) between the two groups (If presence of hydronephrosis, scar and VUR=1 and no=0). RESULTS: Total pediatric patients with febrile APN were 95 patients, a positive S group was 89 patients and a positive M group was 6 patients. Fever duration (S vs. M, 4.7+/-3.1 vs. 6+/-5.7 days), serum WBC (S vs. M, 18,630+/-6,483 vs. 20,153+/-7,660/microL) and CRP (S vs. M, 100.6+/-2.46 vs. 81.1+/-0.09 mg/L) values, and the presence of hydronephrosis, renal scar and VUR were not different between the two groups. CONCLUSION: Our data shows that there were no specific differences of clinical manifestation between a positive single urine culture and a positive mixed urine culture in pediatric APN. A mixed urine culture from an aseptic urine sample should be interpreted cautiously.

Febrile Urinary Tract Infection in Infants Less than Two Months of Age : Characteristics and Factors Related to the Recurrence

PURPOSE: The aim of this study is to characterize and to investigate the factors related to the recurrence of febrile urinary tract infection (UTI) in infants less than 2 months of age. METHODS: We performed a retrospective study in 60 infants, who were treated for the first febrile UTI. Among them, 27 infants were followed for 12 months, and were reviewed concerning the factors related to the recurrence. The factors compared for the recurrence included sex, age at diagnosis, laboratory and radiologic findings like degree of the reflux and renal cortical defect. RESULTS: Among the 60 infants studied, 52 were male (86.7%). Age at diagnosis was 39+/-13 days. The most common pathogen was E.coli (71.7%). The infants underwent an ultrasonography (n=59), DMSA scan (n=55), and voiding cystourethrography (VCUG). Further hydronephrosis, renal cortical defect, vesicoureteral reflux (VUR) were found in 28 (47.4%), 12 (21.8%) and 11 (20.4%) infants, respectively. The hydronephrosis was found frequently in the recurrent UTI group (P=0.012). The VUR was found frequently, but not significantly, in therecurrent UTI group (44.4%) than that of the non-recurrent UTI group (16.7%). CONCLUSION: During the 12 months after the first febrile UTI, hydronephrosis increased the risk of recurrent UTI. The ultrasonography is needed during the follow up of UTI in infants less than two months of age.

Seasonal Variations of Respiratory Syncytial Virus Infection among the Children under 60 Months of Age with Lower Respiratory Tract Infections in the Capital Area, the Republic of Korea, 2008-2011

PURPOSE: Palivizumab prophylaxis has been used in the high risk groups of respiratory syncytial virus (RSV) infections, especially with the prematures, infants with chronic lung diseases or hemodynamically significant congenital heart disease. Substantial variations in timing of RSV outbreaks presents a challenge for the optimized use of palivizumab prophylaxis. This study investigates the epidemiologic characteristics of RSV associated lower respiratory tract infections (LRTI) in children, to help guide in the application of palivizumab prophylaxis in the Republic of Korea. METHODS: This was a retrospective observational study. We performed RSV culture or multiplex RT-PCR from children under 60 months of age admitted for LRTI at three hospitals in the capital area of Korea from May 2008 to April 2011. The study identified RSV infection and analyzed the RSV detection rates. RESULTS: RSV detection rate was 18.8% (1,721/9,178). The RSV season of 2008-2009 is from the second week of August to the fourth week of March and, that of 2009-2010 is from the first week of October to the third week of Apirl and that of 2010-2011 is from the third week of September to the third week of March. The RSV detection rate in preterms and low birth weight infants were significantly higher during the RSV season and non-RSV season. CONCLUSION: The RSV seasons were shown to have variations in onset, offset, and durations in each year. Physicains should determine the timing of the first and final doses of palivizumab on the basis of information about the RSV season in their own area. The real-time surveillance systems to analyze the variations of RSV seasons are necessary for the effective and economical preventions of RSV infections in high risk groups.

Clinical Significance of Extended-Spectrum beta-Lactamase Producing Escherichia coli in Pediatric Patients with Febrile Urinary Tract Infection

PURPOSE: The incidence of community-acquired urinary tract infection (UTI) due to extended-spectrum beta-lactamase producing Escherichia coli (ESBL(+) E. coli) has increased worldwide. ESBL causes resistance to various types of the newer beta-lactam antibiotics, including the expanded spectrum cephalosporins and monobactams. We aimed to investigate the severity of UTI and associated genitourinary malformations in children with febrile UTI caused by ESBL(+) E. coli. METHODS: We retrospectively reviewed the medical records of 290 patients diagnosed as febrile UTI caused by E. coli between January 2008 and October 2010 at Korea University Medical center. We classified the patients into two groups with ESBL(+) and ESBL(-) E. coli group according to the sensitivity of urine culture. Fever duration, admission period, white blood cell (WBC) counts and C-reactive protein (CRP) in peripheral blood, the presence of hydronephrosis, cortical defects, vesicoureteral reflux (VUR) and renal scar were compared between the two groups. RESULTS: Patients with ESBL(+) E. coli were 32, and those with ESBL(-) E. coli were 258. If we excluded those tested with a sterile urine bag, patients with ESBL(+) E. coli were 22, and those with ESBL(-) E. coli were 212. Whether the results of sterile urine bag tests were included or not, there was no significant difference in all parameters between the two groups statistically. CONCLUSION: Our data shows that ESBL(+) E. coli may not be related to the severity of UTI and associated genitourinary malformations.

Acute Kidney Injury Accompanied by Acute Pyelonephritis and Renal Abscess in a Child with Vesicoureteral Reflux

Acute pyelonephritis (APN) is a relatively common bacterial infection in children. In previously healthy children, acute kidney injury (AKI) is very rare in the course of APN without urinary tract obstruction, renal hypoperfusion due to hypotension or exposure to nephrotoxic agents. We describe a case of AKI secondary to APN and renal abscess in a child with vesicoureteral reflux. With antibiotic treatment and adequate hydration, the patient was improved. APN should be included in the differential diagnosis of AKI and adequate treatment by proper antibiotics is crucial for full recovery of renal function.

A Case of Neurofibromatosis with Invasion of Bladder

Neurofibromatosis is a rare systemic disease, and genitourinary tract involvement is especially uncommon. Bladder is the most frequently involved organ in the genitourinary tract. Bladder neurofibromatosis may present as a diffuse infiltrative process or an isolated neurofibroma. The symptoms vary, ranging from urinary incontinence to retention. Treatment is usually conservative. The patient should be worked up to rule out other manifestation of tumor enlargement and followed to evaluate the development of new lesion. We report a case of the development of invasion of bladder in a patient with neurofibromatosis.

Analysis of Interictal Epileptiform Discharges in the Benign Childhood Epilepsy with Centrotemporal Spikes: Prediction of Seizure Outcome

PURPOSE: The benign childhood epilepsy with centrotemporal spikes (BECTS), commonly affecting children between 3 and 13 years of age, is focal epilepsy with the presence of centrotemporal spikes on electroencephalography (EEG). The aim of our study was to assess the relationship between EEG findings and clinical prognosis in patients with BECTS. METHODS: From 2005 to 2010, patients with a diagnosis of BECTS with a follow-up period of over one year after the diagnosis were included in our study. We analyzed their first interictal EEG: frequency of interictal discharges and average voltage in each waking and sleep phase. In addition, multiple factors were reviewed from the medical records: the age at the time of seizure onset, frequency of seizures, use of antiepileptic drugs (AEDs), duration of AED therapy, age at the time of EEG normalization and age at the time of last seizure. RESULTS: Seventy-four patients were included in our study. Frequent interictal epileptiform discharges during sleep correlated with younger age of seizure onset (P=0.040), earlier onset of AED therapy (P=0.022), younger age at the time of seizure cessation (P=0.027), greater frequency of interictal epileptiform discharges during wakefulness (P=0.006), higher average voltage (P=0.001) and higher frequency of seizures after AED initiation (P=0.043). CONCLUSION: In BECTS patients, frequent interictal epileptiform discharges during sleep could be correlated with higher seizure frequency, even after cessation of AED. These findings would be helpful in predicting the prognosis of children with BECTS.