Seasonal Influenza-Related Neurologic Complications in Children from 2010 to 2015 / 대한소아신경학회지

PURPOSE: Influenza-associated neurologic complications in children are diverse. But there has been little long-term and large-scale research about neurologic complications of seasonal influenza. This study aimed to identify the incidence, characteristics, and risk factors for neurologic complications in children hospitalized with influenza. METHODS: Retrospective analysis was conducted on the clinical data of 940 children hospitalized with confirmed influenza infection from Oct, 2010 to May, 2016 in Kwangju Christian Hospital. RESULTS: A total of 940 children with influenza were hospitalized, of whom 96 (10.2%) had neurologic complications:81 children presented febrile seizures (8.6%) and some included 12 other seizures (1.3%),1 encephalitis (0.1%), 1 Guillain-Barré syndrome (0.1%), 1 aseptic meningitis (0.1%). They had good prognosis except the encephalitis child. The incidence of neurologic complications was significantly higher in influenza A than in influenza B (11.9% vs. 7.0%, P=0.036). The incidence of influenza A was highest in February, while that of influenza B was highest in March and April. The monthly distribution of neurological complications reflected the influenza incidence. The risk factors for influenza-associated neurologic complications were underlying neurologic disease and young age. No significant clinical differences were observed between influenza A and B in febrile seizure. CONCLUSION: Febrile seizures are the most common neurologic complication with good prognosis. Although encephalitis/encephalopathy is rare, it can be severe with sequelae, so prompt diagnosis and treatment should be initiated. And influenza vaccine should be encouraged to children with underlying neurologic disease.

Efficacy of early steroid use in Mycoplasma pneumoniae pneumonia

PURPOSE: The aim of this study was to evaluate whether or not the early use of steroid is useful for treating Mycoplasma pneumoniae pneumonia. METHODS: A prospective study was conducted on 85 patients with M. pneumoniae pneumonia admitted to Gwangju Christian Hospital between September 2015 and April 2016. A total of 85 patients were enrolled. Of these, 33 were treated with steroids (methyl-prednisolone 1 mg/kg/day), while 52 were not; both were treated with macrolides. The overall duration of fever was compared between the 2 groups and findings on chest radiographs were evaluated for their deterioration. RESULTS: The duration of fever after admission (1.36±0.92 days vs. 2.17±1.30 days, P=0.003) and the overall duration of fever (4.42±2.13 days vs. 6.07±2.59 days, P=0.003) were significantly lower in the steroid group. The duration of fever before admission was not different between the steroid and macrolide groups (3.06±1.74 days vs. 3.90±2.21 days, P=0.068). On chest radiographs taken 3 days later, 1 of 33 patients in the steroid group and 5 of 50 patients in the macrolide group worsened, although there was no statistically significant difference between the 2 groups (P=0.395). There was no significant difference in the duration of hospitalization between the 2 groups (6.72±1.54 days vs. 6.92±1.87 days, P=0.618). CONCLUSION: Early administration of steroids on patients with M. pneumoniae pneumonia reduced the duration of fever, but there was no difference in duration of admission and x-ray deterioration.

Alagille Syndrome Mimicking Biliary Atresia Confirmed by Jagged1 (JAG1) Gene Analysis in a Newborn: A Case Report

Alagille syndrome is a complex autosomal dominant disorder secondary to defects in the Notch signaling pathway, primarily caused by mutations in the Jagged1 (JAG1) gene. The liver, heart, skeleton, face and eyes are the body parts most commonly involved. Alagille syndrome may mimic other causes of high gamma-glutamyl transferase (GGT)-linked cholestasis, most notably biliary atresia in the neonatal period. Infants with Alagille syndrome are occasionally misdiagnosed as cases with biliary atresia due to variations in clinical features that might be expressed in early infancy. We describe a case of Alagille syndrome mimicking biliary atresia, identified by sequencing analysis of the JAG1 gene in a newborn. During counseling, family members of the patient have also been found to demonstrate various phenotypes and levels of disease severity of Alagille syndrome.

A Case of Neonatal Isoimmune Hemolytic Disease due to Anti-Mi(a) Antibody with Massive Fetomaternal Hemorrhage / 대한주산의학회잡지

Authors experienced a newborn treated with severe anemia transferred to our hospital due to pulselessness and apnea shortly after birth. Laboratory analysis of the blood on admission revealed hemoglobin 3.1 g/dL, reticulocyte 11.0%. Kleihauer-Betke test for fetal hemoglobin from maternal blood was seen Hgb F 7%, then we suggested almost 180 ml fetomaternal hemorrhage. But, anemia was not improved despite repeated packed RBC transfusion. So, we evaluated the other cause of intractable anemia. The results were as follows; the Coombs' test was positive. The antibody identification test using mother's serum revealed anti-Mia antibody. The patient improved with supportive treatment, but got hypoxic brain injury due to massive fetomaternal hemorrhage. At day 29, the infant was doing well and was discharged. We report a case of neonatal isoimmune hemolytic disease due to anti-Mia with massive fetomaternal hemorrhage with a brief review of the related literatures.

A Case of Bronchiolitis Obliterans Organizing Pneumonia with a Solitary Pulmonary Nodule in a Child / 소아알레르기및호흡기학회지

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pulmonary disorder that exhibits various radiographic findings. It often shows bilateral, patched, or ground glass interstitial infiltrate, but a solitary nodular pattern rarely appears. We report a case of an 8-year-old boy suffering from BOOP that showed a single nodular pattern of the lung in the chest radiography. We conclude that when there is a solitary nodule discovered in the lungs of children or adolescence, the differential diagnosis must include BOOP.

Comparison of Clinical Manifestations of RSV, Rhinovirus and Bocavirus Infections in Children with Acute Wheezing / 소아알레르기및호흡기학회지

PURPOSE: The purpose of this study was to compare clinical manifestations of human bocavirus (hBoV), respiratory syncytial virus (RSV), and human rhinovirus (hRV) in children with acute wheezing. METHODS: We retrospectively investigated 549 virus-positive throat swabs obtained between January 2006 and December 2008 from pediatric inpatients with acute respiratory tract disease at Kwangju Christian Hospital. Among them, 109 patients, who had lower respiratory tract infections with wheezing, were enrolled in this study. The medical records of patients with positive results were reviewed for clinical data. RESULTS: The mean age of the patients with RSV was 7.15 months, 15.66 months in those with hRV, and 15.04 months in those with hBoV. The mean fever duration and frequency of patients with fever was 2.43 days and 47.9% for RSV, 2.86 days and 51.7% for hRV, and 3.75 days and 69.6% for hBoV. The frequency of patients with acute otitis media was 20.8% in the RSV, 20.7% in the hRV, and 13.0% in the hBoV groups. The frequency of lung infiltration on chest X-ray was 12.5% in the RSV, 20.7% in the hRV, and 47.8% in the hBoV groups. CONCLUSION: We compared the clinical manifestations of respiratory viral infections in infants and children with wheezing. However, further surveillance will be necessary to clarify the clinical manifestations of the viruses.

Clinical Features and Prognostic Factors of Neurologic Outcome in Group B Streptococcal Meningitis / 대한소아신경학회지

PURPOSE: Group B streptococcal (GBS) meningitis is the leading cause of mortality and morbidity in neonates. There are limited current data regarding outcomes from GBS meningitis and factors that predict an adverse outcome. METHODS: Twenty-three cases of GBS meningitis that were proven by cerebrospinal fluid culture from 2000 to 2010 were retrospectively reviewed. RESULTS: All GBS meningitis cases occurred in young infants less than 3 months and were late-onset (> or =7 days). Four infants (17.4%) died, and three (13.0%) were neurologically impaired at hospital discharge. Compared to the 16 infants with normal neurologic examinations, the 7 infants who died or had adverse outcomes at hospital discharge were more likely to present with seizures within hours of admission, have coma, require pressor support or ventilator support, have an initial peripheral blood leukocyte count less than 4,000/mm3 or neutrophil count less than 1,000/mm3, and have ampicillin resistance on culture. CONCLUSION: Despite advances in intensive care, 30.4% of infants with GBS meningitis die or have neurologic impairment at hospital discharge. Poor outcome can be predicted in cases that have seizures, coma, peripheral leukopenia, or require ventilator or pressor support on initial presentation. These cases show a tendency toward resistance to ampicillin, so vancomycin can be tried initially.

A Case of 22q13 Deletion Syndrome / 대한소아신경학회지

The deletion 22q13 syndrome (Phelan-McDermid syndrome) is a rare microdeletion syndrome characterized by prominent neurobehavioral deficits including neonatal hypotonia, developmental delay, language delay, autism, and minor dysmorphic features. Due to nonspecific facial features and difficulties in detection in routine chromosome analysis, this chromosome deletion syndrome has gone under-diagnosed. Fluorescence in situ hybridization (FISH) is required to confirm the presence of this deletion. Here we report the first case of 22q13 deletion syndrome in Korea. An 18-month-old girl was admitted to a pediatric clinic due to severe developmental delay and hypotonia from the neonatal period. She was diagnosed as 22q13 deletion syndrome through a chromosomal analysis and FISH using arylsulfatase A probe.

Recurrent Wheezing After Human Bocavirus Lower Respiratory Tract Illnesses in Early Life / 소아알레르기및호흡기학회지

PURPOSE: Excluding RSV, the relationship between bronchiolitis caused by viruses and the development of wheezing and atopy in childhood has not been well studied. We studied this relationship in children who had bronchiolitis caused by human bocavirus before 2 years of age. METHODS: We retrospectively investigated 2,430 throat swab obstained between January 2005 and December 2007 from pediatric in-patients with acute respiratory tract disease at the Kwangju Christian Hospital. Human bocavirus was detected in 112 patients. A total of 61 patients less than 2 years of age were finally enrolled in this study. Patients were followed up between April and June of 2008. We measured the frequency of wheezing and atopic status using (allergy skin-prick tests, CAP tests and MAST tests). RESULTS: Of the 61 patients, 16 (26.2%) had recurrent wheezing. Of these 16 patients, 8 (13.1%) had Infrequent wheezing (1-2 wheezing episodes) and 8 (13.1%) had frequent wheezing (3 and over wheezing episodes). Of the total 61 patients, 18 (29.5%) completed allergy tests. Of the 18 patients, 10 (55.6%) were sensitized to at least 1 allergen. Recurrent wheezing was significantly associated with the severity of bronchiolitis (mild vs. moderate vs. severe; 9.1% vs. 22.2% vs. 66.7%). CONCLUSION: Human bocavirus-induced bronchiolitis in childhood are an independent risk factor for development of wheezing in childhood and may be associated with an increased risk of allergic sensitization. The most important risk factor for recurrent wheezing is the severity of lower respiratory tract illnesses.

Effect of Otitis Media and Cerumen Occlusion on Body Temperature Measured by Thermometers / 소아감염

PURPOSE: To examine the effect of unilateral otitis media and unilateral cerumen occlusion of the ear canal on thermometers. METHODS: One hundred eighty six children with unilateral otitis media, fifty children with unilateral cerumen occlusion, and fifty children with neither otitis media nor cerumen were enrolled. Temperature was measured in both ear canals using thermometers. After 15 minutes, second temperature was measured again in both ears. Unilateral otitis media was graded by video otoscope for 7 grades. Differences in temperatures between affected ears and unaffected ears were analyzed. RESULTS: No temperature difference between the normal and cerumen groups was observed. The mean temperature of the otitis media ear canal was 0.13+/-0.20degrees C higher than that of the intact ear canal (36.99+/-0.54degrees C vs 36.86+/-0.52degrees C; P<0.001). There was no statistically significant temperature difference between grades. CONCLUSION: Unilateral otits media can affect estimation of body temperature measured by thermometers.

Clinical Review of Pulmonary Tuberculosis in Teenagers According to the Involved Lung / 소아감염

PURPOSE: The purpose of this study was to investigate clinical features and culture-positive rates according to the involved lung in adolescent pulmonary tuberculosis (TB). METHODS: We retrospectively reviewed the medical records of adolescents who ranged in age from 10 to 20 years and who had been hospitalized with a diagnosis of TB at Kwangju Christian Hospital from 2000 to 2008. RESULTS: Sixty-six patients were identified with pulmonary TB: median age 16.82 years; 48.5% males. Among them, 90.9% of patients were between 15 and 20 years of age. Most patients presented with multiple symptoms, and the most common included cough (74.2%), sputum (60.6%), fever (39.5%), and night sweating (18.2%). Sputum samples were smear-positive in 28 (42.4%), culture-positive in 40 (60.6%), and PCR-positive in 46 (69.7%). The most common radiological patterns included cavitation in 18 (27.3%), pleural effusion in 18 (27.3%), lymphadenopathy in 10 (15.2%), and tuberculoma in 5 (7.6%). The prevalence of smear, culture, and PCR positive rates increased as the number of involved lobes increased (P<0.05, P<0.01, P<0.05). The median treatment duration was 7 months. Twelve patients (18.2%) had lower lung field TB (Group A) and forty-four patients (66.7%) had other areas involving TB, except for Group A (Group B), and ten patients (15.1%) had only TB pleurisy (Group C). The difference of clinical characteristics and culture rates between group A and group B was not significant. CONCLUSION: Pulmonary TB toward late adolescence is increasing. We need to pay more attention to lower lung field TB, which is difficult to detect with specific radiographic findings.

Clinical Spectrum of Norovirus Gastroenteritis Compared to Rotavirus Gastroenteritis at a Single Center in Gwanju, Korea during 2005-2006; Compared to Rotaviral Gastroenteritis / 소아감염

PURPOSE:We evaluated the clinical features of Norovirus gastroenteritis compared with Rotavirus gastroenteritis in hospitalized children. METHOD:We detected causative agents in 3,261 samples of children hospitalized with gastroenteritis symptoms at a single center of pediatrics between 2005 and 2006. Among 266 and 303 samples which tested positive for Norovirus and Rotavirus, we selected 73 and 182 samples of children with relatively pure gastroenteritis symptoms and retrospectively analyzed the corresponding medical records. RESULTS:The male-to-female ratio of the Norovirus (+) and Rotavirus (+) groupswas 1.43:1 and 1.56:1 both groups were predominantly in males. The mean age of the Norovirus (+) and Rotavirus (+) groups was 36.7 and 24.4 months, respectively the children in the former group were older than the children in the latter group. The incidence in the Norovirus (+) group was more concentrated in the winter. The symptoms in the Norovirus (+), in decreasing order, included vomiting, diarrhea, and fever. The duration of vomiting, diarrhea, and fever was 2.1, 1.2, and 1.2 days. The maximum number of episodes of vomiting and diarrhea per day was 3.5 and 4.5, respectively. The severity score was 10.16. The symptoms inthe Rotavirus (+) group, in decreasing order, included diarrhea, vomiting, and fever. The duration of diarrhea, vomiting, and fever was 2.2, 4.3, and 2.2 days, respectively. The maximum number of episodes of vomiting and diarrhea per day was 3.3 and 6.5, respectively. The severity score was 11.9. The severity in the Norovirus (+) group was somewhat lower than the Rotavirus (+) group. The younger the child, the more severe the symptoms in the Norovirus (+) group. There was no difference between mono-and co-infection in severity and between the two groups regarding the hematologic findings. CONCLUSION:Based on the findings reported herein, additional studies about prophylaxis, as well as the epidemiology and clinical features of pediatric Norovirus gastroenteritis, are required.

A Case of Miller-Dieker Syndrome with Infantile Spasm and Lennox-Gastaut Syndrome / 대한소아신경학회지

Miller-Dieker syndrome is a contiguous gene deletion syndrome involving chromosome 17p13.3, which is characterized by type 1(classical) lissencephaly and typical craniofacial abnormalities. Children with Miller-Dieker syndrome have profound psychomotor retardation, seizures that often are intractable, chronic feeding problems that lead to recurrent pneumonia, and shortened lifespan. We have experienced a Miller-Dieker syndrome female who has lived to 8years, showing severe mental and motor retardation and intractable epilepsy. She was diagnosed as Miller-Dieker syndrome in the neonatal period, showing typical facial features, type 1 lissencephaly, and chromosome 17p13.3 microdeletion in fluorescence in situ hybridization. Infantile spasm occurred at 4 months of age and progressed to Lennox-Gastaut syndrome at 3 years and 6 months, both of which were not controlled by antiepileptic drugs.

A case of neonatal major burn by electrical heating pad / 대한주산의학회잡지

Burn in neonates have been reported following the use of pulse oximeters, phototherapy blanket, infrared heating lamp, laryngoscope, and warming bottle. This case reports a newborn who had major burn injuries of 45% total body surface area (TBSA) including 3rd degree burns of 20% TBSA on her back, buttocks, both thighs and heels by exposure to an electrical heating pad for 3 hours. She developed significant systemic response, showing disseminated intravascular coagulation, electrical imbalance, jaundice, hypoalbuminemia, acute renal failure, and persistent pulmonary hypertension of the newborn. The potential hazard of the electric heating pad is reported in order to alert clinicians to this specific risk, to stimulate concern about other similar problems with materials in contact with skin, and to prevent burn of newborns in the neonate unit.

Characterization of Respiratory Viral Infection in Children in Gwangju / 감염과화학요법

BACKGROUND: This investigation was to perform the epidemiological surveillance and genetic analysis on respiratory viral agents from children with acute respiratory infections in Gwangju. MATERIALS AND METHODS: For this study, 3,695 specimens obtained from patients with acute respiratory infections were collected by collaboration with pediatric hospitals in Gwangju between 2005 and 2007. Specimens were screened for 8 respiratory viruses including influenza viruses (IFV), human rhinoviruses (HRV), human coronaviruses (HCoV), adenoviruses (ADV), parainfluenza viruses (PIV), human enteroviruses (HEV), respiratory synthitial viruses (RSV) and human bocaviruses (HBoV). Respiratory viruses were detected using multiplex (RT) PCR with viral specific primers. RESULTS: Out of 3,695 specimens, the ratio of virus detection was 24.9% (919). Overall, HRV (35.5%) and IFV (34.9%) were the most common viruses found, followed by HBoV (14.8%), HCoV (10.6%), RSV (3.7%), ADV (3.4%), PIV (3.2%) and HEV (3.0%). In addition, multiple infections were detected in 80 patients (8.7%). When the prevalence was analyzed according to season, HBoV, IFV and HCoV showed two epidemic points in late spring and early winter. ADV, HRV, RSV PIV and HEV, however, were all found to have only one epidemic point, with RSV being most common during winter and the others being most prominent during spring. CONCLUSIONS: Through this epidemiological surveillance, the respiratory viruses prevalent in children in Gwangju area were investigated. We strongly recommend the development of nationwide policy for the management of prevalent respiratory virus that includes long term collection of data and samples, vaccine development and prevention education of the misuse of antibiotics.

Characterization of Respiratory Viral Infection in Children in Gwangju / 감염과화학요법

BACKGROUND: This investigation was to perform the epidemiological surveillance and genetic analysis on respiratory viral agents from children with acute respiratory infections in Gwangju. MATERIALS AND METHODS: For this study, 3,695 specimens obtained from patients with acute respiratory infections were collected by collaboration with pediatric hospitals in Gwangju between 2005 and 2007. Specimens were screened for 8 respiratory viruses including influenza viruses (IFV), human rhinoviruses (HRV), human coronaviruses (HCoV), adenoviruses (ADV), parainfluenza viruses (PIV), human enteroviruses (HEV), respiratory synthitial viruses (RSV) and human bocaviruses (HBoV). Respiratory viruses were detected using multiplex (RT) PCR with viral specific primers. RESULTS: Out of 3,695 specimens, the ratio of virus detection was 24.9% (919). Overall, HRV (35.5%) and IFV (34.9%) were the most common viruses found, followed by HBoV (14.8%), HCoV (10.6%), RSV (3.7%), ADV (3.4%), PIV (3.2%) and HEV (3.0%). In addition, multiple infections were detected in 80 patients (8.7%). When the prevalence was analyzed according to season, HBoV, IFV and HCoV showed two epidemic points in late spring and early winter. ADV, HRV, RSV PIV and HEV, however, were all found to have only one epidemic point, with RSV being most common during winter and the others being most prominent during spring. CONCLUSIONS: Through this epidemiological surveillance, the respiratory viruses prevalent in children in Gwangju area were investigated. We strongly recommend the development of nationwide policy for the management of prevalent respiratory virus that includes long term collection of data and samples, vaccine development and prevention education of the misuse of antibiotics.

3-year Follow-up of a Menkes Disease Patient / 대한소아신경학회지

Menkes disease is a rare fatal X-linked recessive disorder characterized by a generalized defect in intracelluar copper transport. The clinical features which arise from copper deficiency include progressive neurologic deterioration, epilepsy, hair and connective tissue abnormalities. Menkes disease is caused by mutations in the gene encoding the Menkes protein(ATP7A, copper transporting P-type ATPase), which is located on the long arm 13 of the X-chromosome. ATP7A mutations are found in 60 to 70% of the patients. We have experienced a case of Menkes disease in a 6-month-old male who showed developmental delay, myoclonic seizures and kinky hair. The serum copper and ceruloplasmin levels were low and the missense mutation(c.3352G>A, resulting in p.G1118S) in exon 17 of ATP7A gene was found. During 3-year follow-up, he regressed developmentally and showed brain atrophy, multiple bladder deverticula, and bony deformities.

A Case of Prader-Willi Syndrome with an Unusually Large 15q Deletion Due to an Unbalanced Translocation to Chromosome 2 45,XX,-15, der(2) t(2;15)(q37:q13)

Prader-Willi syndrome is a disease of chromosome 15, which is characterized by severe hypotonia and feeding difficulty in neonates, followed by development of obesity, mental retardation, and hypogonadism. Approximately 70% of the patients have a paternal deletion on chromosome 15q11-13, which is mainly a microdeletion, and a large deletion due to an unbalanced structural translocation of the proximal long arm of chromosome 15 to several other chromosomes is rarely found. We encountered a neonatal case with Prader-Willi syndrome who had sustained hypotonia and feeding difficulty. On high-resolution chromosome analysis, deletion of the short arm and the proximal part of the long arm of chromosome 15, with unbalanced translocation of the remaining part of chromosome 15(q13-qter) to the terminal part q37 of chromosome 2, was shown to be . Through FISH (Fluorescence in situ hybridization) and methylation-specific DNA PCR, we confirmed the deleted q11-13 was derived from the father.

Two Cases of Rickets that Developed as a Result of by Diet Restriction due to Atopic Dermatitis / 대한소아소화기영양학회지

Nutritional rickets, which is caused by deficiency of calcium or vitamin D, is a rare disease in developed countries. However some cases have been reported recently, that developed as a result of a restricted and nutritionally imbalanced diet due to atopic dermatitis and related food allergy. We treated two infant cases of nutritional rickets. The infants had suffered from atopic dermatitis, and were fed "Sun-sik" (a powdery mixture of several grains and fruits) without receiving cow's milk or any milk products in their diet. After an adequate supply of calcium and nutritional management, they were markedly improved.

A Case of Right Coronary Artery to Left Ventricle Fistula with Progression to Spontaneous Closure: Echocardiographic Findings

Coronary artery fistula (CAF) is a rare congenital cardiac anomaly, which mainly drains to the right side heart. As the progression to spontaneous closure in CAF was rare, it needed surgery or catheteric embolization in asymptomatic patients due to the future risk of congestive heart failure, myocardiac infarction or sudden death. With the advent of high resolution two-dimensional and color doppler echocardiography during the past decade, more cases of CAF have been diagnosed and spontaneously closed cases have been increased. Here we report a healthy neonate except heart murmur, who showed a right coronary to left ventricle fistula with progression to spontaneous closure at 11months of age. As the natural course of CAF is still poorly defined and spontaneous closure is increasing, it will be desirable to be followed up regularly by echocardiography in asymptomatic cases, rather than to be corrected surgically.