Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.
Adolescent ; Antibodies ; Antibodies, Antinuclear ; Blood Transfusion ; Female ; Hemorrhage* ; Humans ; Hypoprothrombinemias* ; Immunoglobulins ; Immunosuppressive Agents ; Lupus Coagulation Inhibitor* ; Methylprednisolone ; Pancytopenia ; Partial Thromboplastin Time ; Plasma ; Prothrombin ; Prothrombin Time ; Rare Diseases ; Steroids ; Vitamin K

Iatrogenic pseudoaneurysms are extremely rare in children. Conventional management of pseudoaneurysms in adults has included surgical repair, ultrasound-guided compression, and more recently, endovascular embolization. However, in infants and children, there is little information regarding the applicability of such treatment modalities, which have been effective in adults, because of its rarity. Here, we present the case of a 6-month-old infant who developed a postprocedural pseudoaneurysm of the external iliac artery, which was successfully treated with ultrasound-guided percutaneous thrombin injection.
Adult ; Aneurysm ; Aneurysm, False* ; Child ; Humans ; Iliac Artery* ; Infant* ; Thrombin*

PURPOSE: This study aimed to evaluate the autonomic imbalance in syncope by comparing the baseline heart rate variability (HRV) between healthy children and those with vasovagal syncope. METHODS: To characterize the autonomic profile in children experiencing vasovagal syncope, we evaluated the HRV of 23 patients aged 7-18 years and 20 healthy children. These children were divided into preadolescent (<12 years) and adolescent groups. The following time-domain indices were calculated: root mean square of the successive differences (RMSSD); standard deviation of all average R-R intervals (SDNN); and frequency domain indices including high frequency (HF), low frequency (LF), normalized high frequency, normalized low frequency, and low frequency to high frequency ratio (LF/HF). RESULTS: HRV values were significantly different between healthy children and those with syncope. Student t test indicated significantly higher SNDD values (60.46 ms vs. 37.42 ms, P=0.003) and RMSSD (57.90 ms vs. 26.92 ms, P=0.000) in the patient group than in the control group. In the patient group, RMSSD (80.41 ms vs. 45.89 ms, P=0.015) and normalized HF (61.18 ms vs. 43.19 ms, P=0.022) were significantly higher in adolescents, whereas normalized LF (38.81 ms vs. 56.76 ms, P=0.022) and LF/HF ratio (0.76 vs. 1.89, P=0.041) were significantly lower in adolescents. In contrast, the control group did not have significant differences in HRV values between adolescents and preadolescents. CONCLUSION: The results of this study indicated that children with syncope had a decreased sympathetic tone and increased vagal tone compared to healthy children. Additionally, more severe autonomic imbalances possibly occur in adolescents than in preadolescents.
Adolescent* ; Child* ; Heart Rate* ; Humans ; Syncope ; Syncope, Vasovagal*

PURPOSE: The prevalence of macrolide-resistant Mycoplasma pneumoniae (MRMP) has increased worldwide. The aim of this study was to estimate the proportion of MRMP in a tertiary hospital in Korea, and to find potential laboratory markers that could be used to predict the efficacy of macrolides in children with MRMP pneumonia. METHODS: A total of 95 patients with M. pneumoniae pneumonia were enrolled in this study. Detection of MRMP was based on the results of specific point mutations in domain V of the 23S rRNA gene. The medical records of these patients were reviewed retrospectively and the clinical course and laboratory data were compared. RESULTS: The proportion of patients with MRMP was 51.6% and all MRMP isolates had the A2063G point mutation. The MRMP group had longer hospital stay and febrile period after initiation of macrolides. The levels of serum C-reactive protein (CRP) and interleukin-18 in nasopharyngeal aspirate were significantly higher in patients who did not respond to macrolide treatment. CRP was the only significant factor in predicting the efficacy of macrolides in patients with MRMP pneumonia. The area under the curve for CRP was 0.69 in receiver operating characteristic curve analysis, indicating reasonable discriminative power, and the optimal cutoff value was 40.7 mg/L. CONCLUSION: The proportion of patients with MRMP was high, suggesting that the prevalence of MRMP is rising rapidly in Korea. Serum CRP could be a useful marker for predicting the efficacy of macrolides and helping clinicians make better clinical decisions in children with MRMP pneumonia.
Biomarkers ; C-Reactive Protein* ; Child* ; Drug Resistance, Microbial ; Genes, rRNA ; Humans ; Interleukin-18 ; Korea ; Length of Stay ; Macrolides* ; Medical Records ; Mycoplasma pneumoniae* ; Pneumonia* ; Pneumonia, Mycoplasma* ; Point Mutation ; Predictive Value of Tests ; Prevalence ; Retrospective Studies ; ROC Curve ; Tertiary Care Centers

PURPOSE: Blood culture is the most important tool for detecting bacteremia in children with fever. However, blood culture contamination rates range from 0.6% to 6.0% in adults; rates for young children have been considered higher than these, although data are limited, especially in Korea. This study determined the contamination rate and risk factors in pediatric patients visiting the emergency room (ER) or being admitted to the ward. METHODS: We conducted a retrospective chart review of blood cultures obtained from children who visited Yonsei Severance Hospital, Korea between 2006 and 2010. Positive blood cultures were labeled as true bacteremia or contamination according to Centers for Disease Control and Prevention/National Healthcare Safety Network definitions for laboratory-confirmed bloodstream infection, after exclusion of cultures drawn from preexisting central lines only. RESULTS: Among 40,542 blood cultures, 610 were positive, of which 479 were contaminations and 131 were true bacteremia (overall contamination rate, 1.18%). The contamination rate in the ER was significantly higher than in the ward (1.32% vs. 0.66%, P<0.001). The rate was higher in younger children (2.07%, 0.94%, and 0.61% in children aged <1 year, 1-6 years, and >6 years, respectively). CONCLUSION: Overall, contamination rates were higher in younger children than in older children, given the difficulty of performing blood sampling in younger children. The contamination rates from the ER were higher than those from the ward, not accounted for only by overcrowding and lack of experience among personnel collecting samples. Further study to investigate other factors affecting contamination should be required.
Adult ; Bacteremia ; Centers for Disease Control and Prevention (U.S.) ; Child ; Delivery of Health Care ; Emergency Service, Hospital ; Fever ; Humans ; Korea ; Retrospective Studies ; Risk Factors

PURPOSE: Eosinophilia is common in premature infants, and its incidence increases with a shorter gestation period. We investigated the clinical significance of eosinophilia in premature infants born at <34 weeks gestation. METHODS: We analyzed the medical records of premature infants born at <34 weeks gestation who were admitted to the neonatal intensive care unit at Ewha Womans University Mokdong Hospital between January 2003 and September 2010. Eosinophilia was defined as an eosinophil percentage of >3% of the total leukocytes. Perinatal parameters and clinical parameters were also analyzed. RESULTS: Of the 261 infants born at <34 weeks gestation, 22.4% demonstrated eosinophilia at birth. The eosinophil percentage peaked in the fourth postnatal week at 7.5%. The incidence of severe eosinophilia increased after birth up to the fourth postnatal week when 8.8% of all patients had severe eosinophilia. Severity of eosinophilia was positively correlated with a lower gestational age, birth weight, and Apgar score. Respiratory distress syndrome, bronchopulmonary dysplasia, nephrocalcinosis, intraventricular hemorrhage, and sepsis were associated with a higher eosinophil percentage. The eosinophil percentage was significantly higher in infants with bronchopulmonary dysplasia from the first postnatal week and the percentage was the highest in the fourth postnatal week, with the maximal difference being 4.1% (P<0.001). CONCLUSION: Eosinophilia is common in premature infants and reaches peak incidence and severity in the fourth postnatal week. The eosinophil percentage was significantly higher in bronchopulmonary dysplasia patients from the first postnatal week. Severe eosinophilia was significantly associated with the incidence of bronchopulmonary dysplasia even after adjusting for other variables.
Apgar Score ; Birth Weight ; Bronchopulmonary Dysplasia* ; Eosinophilia* ; Eosinophils ; Female ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infant, Premature* ; Intensive Care, Neonatal ; Leukocytes ; Medical Records ; Nephrocalcinosis ; Parturition ; Pregnancy* ; Sepsis

Influenza causes acute respiratory infections and various complications. Children in the high-risk group have higher complication and hospitalization rates than high-risk elderly individuals. Influenza prevention in children is important, as they can be a source infection spread in their communities. Influenza vaccination is strongly recommended for high-risk children with chronic underlying circulatory and respiratory disease, immature infants, and children receiving long-term immunosuppressant treatment or aspirin. However, vaccination rates in these children are low because of concerns regarding the exacerbation of underlying diseases and vaccine efficacy. To address these concerns, many clinical studies on children with underlying respiratory diseases have been conducted since the 1970s. Most of these reported no differences in immunogenicity or adverse reactions between healthy children and those with underlying respiratory diseases and no adverse effects of the influenza vaccine on the disease course. Further to these studies, the inactivated split-virus influenza vaccine is recommended for children with underlying respiratory disease, in many countries. However, the live-attenuated influenza vaccine (LAIV) is not recommended for children younger than 5 years with asthma or recurrent wheezing. Influenza vaccination is contraindicated in patients with severe allergies to egg, chicken, or feathers, because egg-cultivated influenza vaccines may contain ovalbumin. There has been no recent report of serious adverse events after influenza vaccination in children with egg allergy. However, many experts recommend the trivalent influenza vaccine for patients with severe egg allergy, with close observation for 30 minutes after vaccination. LAIV is still not recommended for patients with asthma or egg allergy.
Aged ; Animals ; Aspirin ; Asthma ; Chickens ; Child* ; Egg Hypersensitivity ; Feathers ; Hospitalization ; Humans ; Hypersensitivity* ; Infant ; Influenza Vaccines ; Influenza, Human* ; Ovalbumin ; Ovum ; Respiratory Sounds ; Respiratory Tract Infections ; Seasons* ; Vaccination*

Respiratory distress syndrome (RDS) among preterm infants is typically due to a quantitative deficiency of pulmonary surfactant. Aside from the degree of prematurity, diverse environmental and genetic factors can affect the development of RDS. The variance of the risk of RDS in various races/ethnicities or monozygotic/dizygotic twins has suggested genetic influences on this disorder. So far, several specific mutations in genes encoding surfactant-associated molecules have confirmed this. Specific genetic variants contributing to the regulation of pulmonary development, its structure and function, or the inflammatory response could be candidate risk factors for the development of RDS. This review summarizes the background that suggests the genetic predisposition of RDS, the identified mutations, and candidate genetic polymorphisms of pulmonary surfactant proteins associated with RDS.
Genetic Predisposition to Disease ; Humans ; Infant, Newborn ; Infant, Premature ; Polymorphism, Genetic ; Pulmonary Surfactant-Associated Proteins ; Pulmonary Surfactants ; Risk Factors* ; Twins

PURPOSE: This study was conducted to survey the preliminary data on risk behaviors and to identify the factors that prevent risk-behaviors in late adolescence. METHODS: Freshmen(n=1,297) beginning the first semester in Korea University, Seoul, Korea completed self-administered risk behavior questionnaires, comprising 5 domains : demographics, smoking, drinking, drug abuse and sexual behavior. RESULTS: The rate of smoking experience was higher in people having friends who smoke and the predictors of transition to current smoking were male gender, urban residence, friends' smoking, and nicotine dependence. The rate of high risk drinking was higher among students who are male and who had experience of heavy episodic drinking. The study group showed a low prevalence of narcotic users, but two-thirds of students could get medicine easily without prescriptions. The prevalence of sexual experience was 6.5 percent, and the sexual education was not a predictor of contraceptive behavior. The prevalence of homosexuality was 1.6 percent, and the rate of mostly heterosexuality was higher in female students. CONCLUSION: The main targets of youth health education should be campaigns aimed at atcessation of reinforcing risk behaviors and the development of a surveillance system for the prevention of chronic disease. These results can be used to find risk factors of health-risk behaviors among late adolescents.
Adolescent ; Alcohol Drinking ; Chronic Disease ; Contraception Behavior ; Demography ; Drinking ; Education ; Female ; Friends ; Health Education ; Heterosexuality ; Homosexuality ; Humans ; Korea ; Male ; Prescriptions ; Prevalence ; Surveys and Questionnaires ; Risk Factors ; Risk-Taking* ; Seoul ; Sexual Behavior ; Smoke ; Smoking ; Substance-Related Disorders ; Tobacco Use Disorder

PURPOSE: The prevalence of obesity and nonalcoholic steatohepatitis(NASH) was increased in adolescents. This study was performed to observe the prevalence of elevated aminotransferase levels in adolescents and to assess the correlations between aminotransferase levels and obesity related parameters(body mass index, waist circumference, hip circumference, waist to hip ratio and insulin level). METHODS: We obtained weight, height, waist circumference and hip circumference from 2,417 male and 1,219 female adolescents. Mean age was 15.7+/-0.7 years old. We measured fasting insulin, aspartate aminotransferase(AST) and alanine aminotransferase(ALT). Obese and overweight were defined as body mass indices(BMI) of more than the 95th, and 85th-94th percentiles, respectively, for age and sex. RESULTS: The number of adolescents with obesity is 324(8.9 percent). 414(11.4 percent) subjects belonged to the overweight group. The average ALT level of obese, overweight and control groups were significantly different(obese : 32.1+/-34.3 U/L, overweight : 19.6+/-13.6 U/L, control : 12.7+/-6.7 U/L, P<0.001). The average AST level was also different according to group(obese : 27.8+/-16.5 U/L, overweight : 22.8+/-8.6 U/L, control : 20.8+/-8.5 U/L, P<0.001). AST and ALT were correlated with anthropometric parameters and insulin level. After multiple regression analysis, waist circumference was the significant predictive value for AST(r=0.234, P<0.001). Waist circumference, BMI and insulin levels were significant predictive values for ALT(r=0.435, P<0.001). CONCLUSION: The prevalence of abnormal aminotransferase was higher in the obese and overweight groups than control group. Waist circumference was useful to predict abnormal aminotransferase le
Adolescent* ; Alanine ; Aspartic Acid ; Fasting ; Hip ; Humans ; Insulin ; Male ; Obesity ; Overweight ; Pediatric Obesity* ; Prevalence ; Waist Circumference