• Journal of the Korean Society of Child Welfare
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• no.36
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• pp.101-132
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• 2011

Using data collected from Korean mothers (N＝429) with preschool children (aged 3 to 5 years) in 12 day care centers in Seoul, this study aims to empirically demonstrate the different pathways acute and chronic stress affect parenting efficacy of mothers according to the income group to which they belong. Analytic results show mothers in the low-income group show higher levels of depressive symptoms than mothers in the middle-income group, and the former also show more frequent and higher levels of acute and chronic stress than the latter. The mediation model results show that acute stress and chronic stress did not directly affect parenting efficacy, but rather indirectly affect parenting efficacy through maternal depressive symptoms. Acute stress and chronic stress are both significant factors affecting depressive symptoms with chronic stress exerting greater influence. When the total effect was analyzed, chronic stress has a greater effect on parenting efficacy than acute stress. The effect of chronic stress on maternal depressive symptoms is greater on low-income mothers than middle-income mothers, while the effect of acute stress on maternal depressive symptoms and the effect of those symptoms on parenting efficacy are greater on middle class families than low-income families. In order to maximize effective parenting in high-risk situations, the psychological welfare of mothers needs to be protected from the environmental difficulties they face. Based on the results, policies to support women and parents at the national and social levels are discussed.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.610-615
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• 2008

Purpose : Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematological disease in children. It generally occurs after exposure to a common viral infection episode; however, it may occasionally follow immunization with measles, measles-mumps-rubella (MMR), hepatitis B (HBV), influenza, diphtheria-tetanus-pertussis (DTP), or chickenpox vaccines. In this study, the incidence, clinical characteristics, and treatment outcome of vaccination-associated ITP were investigated and compared with non-vaccination-associated ITP. Methods : The admission records of 105 pediatric ITP patients between 0-14 years of age admitted to Department of Pediatrics, Wonkwang University Hospital from January 1994 to July 2007 were retrospecitively reviewed. Patients were grouped into a vaccination-associated group and a non-vaccination-associated group according to vaccination history within the previous 1 month, and various clinical features between the two groups were statistically analyzed. Results : Thirteen patients (12%) had a preceding vaccination. Eight had received DTP vaccination, 2 had received hepatitis B, and 1 each had received influenza, MMR, and Japanese B encephalitis vaccination. However, none of the patients had a recurrent thrombocytopenia after subsequent vaccinations. In the vaccination-associated group, the age was significantly lower, anemia was more common, and the risk period with blood platelet count $<20{\times}10^9/L$ was significantly shorter than for the in non-vaccination-associated group. Also, wet purpura was less prominent and the remission within 1month was more frequently achieved in the vaccination-associated ITP group. Conclusion : Vaccination-associated ITP patients showed mild symptoms with a more benign and shorter lasting course than non-vaccination-associated ITP patients. Moreover, platelet count assessment at the time of the next immunization may not be necessary.

• Genomics & Informatics
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• v.16 no.2
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• pp.36-41
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• 2018

Kawasaki disease (KD) is an acute febrile vasculitis predominately affecting infants and children. The dominant incidence age of KD is from 6 months to 5 years of age, and the incidence is unusual in those younger than 6 months and older than 5 years of age. We tried to identify genetic variants specifically associated with KD in patients younger than 6 months or older than 5 years of age. We performed an age-stratified genome-wide association study using the Illumina HumanOmni1-Quad BeadChip data (296 cases vs. 1,000 controls) and a replication study (1,360 cases vs. 3,553 controls) in the Korean population. Among 26 candidate single nucleotide polymorphisms (SNPs) tested in replication study, only a rare nonsynonymous SNP (rs4365796: c.1106C>T, p.Thr369Met) in the lymphoid enhancer binding factor 1 (LEF1) gene was very significantly associated with KD in patients younger than 6 months of age (odds ratio [OR], 3.07; $p_{combined}=1.10{\times}10^{-5}$), whereas no association of the same SNP was observed in any other age group of KD patients. The same SNP (rs4365796) in the LEF1 gene showed the same direction of risk effect in Japanese KD patients younger than 6 months of age, although the effect was not statistically significant (OR, 1.42; p = 0.397). This result indicates that the LEF1 gene may play an important role as a susceptibility gene specifically affecting KD patients younger than 6 months of age.

• Pediatric Infection and Vaccine
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• v.15 no.2
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• pp.152-161
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• 2008

Purpose : Staphylococcal scalded skin syndrome (4S) is uncommon, but reports of 4S are on the increase during the recent years. The purpose of this study is to determine the clinical features and associated factors of 4S during the recent 10 years. Methods : We retrospectively reviewed the medical records and microbiologic results of 63 patients (27 neonates and 36 children) from January 1998 to December 2007. Results : Since 2003, the incidence of 4S has increased. The mean age of the patients was 16.3 months and the gender ratio was 1:1. The clinical types of 4S were 38 cases of the abortive type (60%), 19 cases of the intermediate type (30%) and 6 cases of the generalized type (10%). The culture results were 36 cases of Methicillin resistant S. aureus (MRSA), 4 cases of Methicillin sensitive S. aureus and 17 cases of no growth. The patients were treated with semi-synthetic penicillin. For the 9 patients who had MRSA isolated and who didn't improve with penicillin, they were treated with vancomycin instead of penicillin. All the patients had no complications. 4S abruptly increased in 2005, and especially in neonates, due to an MRSA outbreak at a local nursery room. The associated factors of 4S in neonates were hospitalization (27 cases), including nursery infection in 2005 (18 cases) and dermatitis (1 case). There was an unknown origin for some children, and the suggested factors for their infection were community acquired infection (24 cases), atopic dermatitis (9 cases) and hospitalization (3 cases). Conclusion : 4S has recently been increasing. The major associated factors of 4S are a history of hospitalization, an outbreak in a nursery room, atopic dermatitis and community acquired infection.

• Pediatric Infection and Vaccine
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• v.5 no.1
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• pp.69-78
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• 1998

Purpose : The risk of severe tuberculous disease such as meningitis or miliary tuberculosis increases as younger is the child at the time of infection. Therefore, the early diagnosis and prompt treatment is mandatory for infants with tuberculosis. This study was undertaken to describe the epidemiology, clinical and radiographic manifestations, and response to therapy in infants with tuberculous disease. Methods : Medical records of 29 infants with tuberculosis diagnosed at the Seoul National University Children's Hospital from July, 1985, to April, 1997, were reviewed, retrospectively. A case of tuberculosis was confirmed if M. tuberculosis was isolated from any body site or if there was histologic proof of tuberculosis. Otherwise, the diagnoses were individualized considering history of contact with contagious adult case, clinical manifestations, chest X-ray findings, result of a Mantoux test reaction with 5 tuberculin unit of PPD, and the response to therapy. Results : The mean age at diagnosis was $7.00{\pm}2.65$ months (range, 3 to 12 months). Twelve cases had isolated pulmonary diseases, and the rest had pulmonary disease and meningitis, 5 cases; pulmonary disease and cervical lymphadenitis, 3; isolated meningitis, 3; and miliary tuberculosis, 6. Source case was identified in 19 cases, 7 of which were detected with retrograde manner. Twenty seven of 29 were symptomatic at their initial visit. The presenting symptoms were mainly respiratory or neurologic, and respiratory difficulty was accompanied in 7 cases. Physical examination revealed wheezing in 7 cases and decreased breath sounds in 9. Hepatomegaly or hepatosplenomegaly were frequent. Chest radiographs showed lung parenchymal disease with hilar lymphadenopathy in 18 cases, and focal or generalized emphysematous change in 7 cases. Conclusion : Most of the infants with tuberculosis are symptomatic at diagnosis, and many of infants with intrathoracic tuberculosis presented with symptoms of bronchial obstruction. When tuberculosis is suspected in an infant, the adult source case should be vigorously investigated to aid in diagnosis and for the prevention of further transmission of tuberculous disease. Almost half of infant tuberculosis are preventable if prophylaxis were given when adult cases were diagnosed.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1253-1259
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• 2003

Backgroud : Seizures in the neonate are relatively common and their clinical features are different from those in children and adults. The study aimed to provide the clinical profiles of neonatal seizure in our hospital. Methods : A total of 41 newborns with seizures were enrolled in this study over a period of three years. They were evaluated with special reference to risk factors, neurologic examinations, laboratory data, neuroimaging studies, EEG findings, seizure types, response to treatment, and prognosis, etc. Results : The average age at onset of seizures was $6.1{\pm}4.6days$ and the majority of patients(42%) had multifocal clonic seizure and 24% had subtle seizure. Factors that are known to increase risk of neonatal seizures include abnormal delivery history, birth asphyxia, and electrolyte imbalance, etc. However, they remain obscure in about 20% of cases. More than 50 percent showed abnormal lesions on neuroimaging studies such as brain hemorrhage, periventricular leukomalacia, brain infarction, cortical dysplasia, hydrocephalus, etc. and 17 out of 32 patients showed abnormal electroencephalographic patterns. Phenobarbital was tried as a first line antiepileptic drug and phenytoin was added if it failed to control seizures. The treatments were terminated in the majority of patients during the hospital stay. The overall prognosis was relatively good except for those with abnormal EEG background or congenital central nervous system malformations. Conclusion : Neonatal seizures may permanently disrupt brain development. Better understanding of their clinical profiles and appropriate management may lead to a reduction in neurological disability in later childhood.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1342-1347
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• 2005

Purpose : Among perinatal risk factors, neonatal seizures are one of the strongest independent discriminators of adverse outcome, representing high risks of mortality and neurologic morbidity. This study was undertaken to evaluate the neurologic outcome of neonatal status epilepticus according to underlying etiology, seizure pattern, onset time, and duration. Methods : We reviewed retrospectively 36 neonates(19 males, 17 females) with status epilepticus who were admitted to the neonatal intensive care unit, Inha Hospital between July, 1988 and June, 2003. They were evaluated with neurologic examination, laboratory data, EEG findings, and neuroimaging studies etc. Results : The mean gestational period of the patients was $37.0{\pm}3.6$ weeks and birth weight was $2.70{\pm}0.82$ kilogram. Fifty two point eight percent of the neonates were male and 66.7 percent were born at term. The most common cause of neonatal status epilepticus was hypoxic-ischemic encephalopathy. In preterm babies, intracranial hemorrhages showed an especially high frequency(P=0.034). Gestational age and birth weight did not show a correlation with neurologic complications. The incidence of neurological sequelae were significantly related to prolonged seizures lasting more than 1 hour(P=0.002). Neonates with seizures within the first 72 hours tended to be more frequent among those who developed adverse outcomes(P=0.016). Generalized tonic seizures had the worst prognosis, whereas those children who had subtle seizures had better outcomes than any other type(P<0.05). Generalized tonic seizures were primarily represented on EEG by abnormal background, whereas subtle seizure showed a significantly more normal EEG than any other seizures(P<0.05). Conclusion : Our results indicate that neonatal status epilepticus with early onsets, prolonged durations. And generalized tonic types can predict an increased risk for neurologic sequelae. So, those seizures must be perceived as medical emergencies and treated aggressively with antiepileptic drugs.

• Journal of Genetic Medicine
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• v.8 no.1
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• pp.71-75
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• 2011

A male infant was diagnosed with obstetric brachial plexus injury, congenital muscular torticollis and cleft palate 17 days after birth. His mother presented with gestational diabetes and premature rupture of membranes. Although it is possible that these three disorders arose independently, it is very likely that all three have the same etiologic cause, and we propose that a possible mechanism for this concurrence is related to maternal gestational diabetes. Maternal hyperglycemia mostly affects fetal structures deriving from the neural crest, including the palatine bone, and may have caused the cleft palate observed in this case. Gestational diabetes is also associated with increased frequency of large for gestational age infants and, by extension, with increased risk of birth injuries such as obstetric brachial plexus injury or congenital muscular torticollis associated with large for gestational age infants. Since the children of mothers with gestational diabetes are at increased risk for congenital defects such as cleft palate as well as being large for gestational age, precautions indicated for each respective disorder must be taken during prenatal testing and during birth. However, further studies of more cases are required to evaluate whether the concurrence of obstetric brachial plexus injury, congenital muscular torticollis and cleft palate in this case are complications specifically associated with gestational diabetes or just a simple coincidence.

• Pediatric Infection and Vaccine
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• v.30 no.3
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• pp.129-138
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• 2023

Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haploinsufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection.

• Neonatal Medicine
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• v.18 no.2
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• pp.310-319
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• 2011

Purpose: The aim of this study was to investigate the effect of perinatal risk factors on brain maturation and the relationship of brain maturation and neurodevelopmental outcomes with brain maturation scoring system in brain MRI. Methods: ELBWI infants born at the Seoul National University Children's Hospital from January 2006 to December 2010 were included. A retrospective analysis was performed with their medical record and brain MR images acquired at near full term. We read brain MRI and measured maturity with total maturation score (TMS). TMS is a previously developed anatomic scoring system to assess brain maturity. The total maturation score was used to evaluate the four parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. Results: Images from 124 infants were evaluated. Their mean gestational age at birth was 27.1${\pm}$2.1 weeks, and mean birth weight was 781.5${\pm}$143.9 g. The mean TMS was 10.8${\pm}$2.0. TMS was significantly related to the postmenstrual age (PMA) of the infant, increasing with advancing postmenstrual age (P<0.001). TMS showed no significance with neurodevelopmental delay, and with brain injury, respectively. Conclusion: TMS was developed for evaluating brain maturation in conventional brain MRI. The results of this study suggest that TMS was not useful for predicting neurodevelopmental delay, but further studies are needed to make standard score for each PMA and to re-evaluate the relationship between brain maturation and neurodevelopmental delay.