Although rare, antihistamines can cause adverse effects, including drug-induced eruptions or anaphylaxis. A 4-year-old child visited the pediatric department of a hospital for skin eruptions after administration of antihistamines, (e.g., ucerax [hydroxyzine] or leptizine [levocetirizine]), for cholinergic rashes; he did not have pruritus. Skin prick, intradermal, and drug provocation tests were performed to determine the relationship between the antihistamines and eruptions. Levocetirizine induced wheals in the skin prick test and a rash in the oral drug provocation test. In contrast, ketotifen induced no reaction in the skin prick test but showed a positive reaction in the oral provocation test. Our case report highlights that children can experience the same types of adverse reactions as seen in adults, and cross-reactivity between various antihistamines can occur.
Adult ; Anaphylaxis ; Child ; Child, Preschool ; Drug Eruptions ; Exanthema ; Histamine Antagonists ; Humans ; Ketotifen ; Pruritus ; Skin ; Urticaria

PURPOSE: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. METHODS: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. RESULTS: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. CONCLUSION: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.
Christianity ; Congenital Abnormalities ; Humans ; Incidence ; Magnetic Resonance Imaging ; Neural Tube Defects ; Neurosurgery ; Physical Examination ; Prevalence ; Retrospective Studies ; Ultrasonography

PURPOSE: We aimed to determine the prognostic significance of lymphocyte counts and the lymphocytemonocyte ratio (LMR) in pediatric patients with osteosarcoma. METHODS: We retrospectively reviewed the medical records of 27 pediatric patients with localized extremity osteosarcoma, treated at the Korea Cancer Center Hospital between May 2002 and March 2016. Leukocyte counts and LMR before treatment and on day 14 (LMR14) of the first cisplatin-doxorubicin chemotherapy round were evaluated. Patients were dichotomized according to the median value of these parameters, and survival rates were compared. RESULTS: The median age of the 27 patients was 9.9 years (range, 3.2–14.1 years) and tumor sites were: distal femur (n=14), proximal humerus (n=7), proximal tibia (n=2), proximal fibula (n=2), and elsewhere (n=2). Patients were followed up on for a median of 76.4 months (range, 4.5–174.7 months), and 5-year overall (OS) and event-free survival (EFS) rates were 66.0%±9.8% and 60.9%±9.7%, respectively. Patients with a higher pretreatment lymphocyte count (≥2,320/μL) had better OS (90.9% vs. 46.2%, P=0.04) and EFS (83.9% vs. 38.5%, P=0.02). However, the day 14 lymphocyte count was not associated with survival. While no survival difference was observed between patients grouped according to pretreatment LMR (median value, 6.3), patients with a higher LMR14 (≥5) fared better than those with lower LMR14 (5-year OS: 83.3% vs. 46.3%, P=0.04). CONCLUSION: Pretreatment lymphocyte count and LMR during chemotherapy had prognostic significance in pediatric osteosarcoma patients. Further studies involving larger cohorts are necessary to validate our findings.
Cohort Studies ; Disease-Free Survival ; Drug Therapy ; Extremities ; Femur ; Fibula ; Humans ; Humerus ; Korea ; Leukocyte Count ; Lymphocyte Count ; Lymphocytes ; Medical Records ; Monocytes ; Osteosarcoma ; Retrospective Studies ; Survival Rate ; Tibia ; Treatment Outcome

PURPOSE: Myotonic dystrophy, also known as dystrophia myotonica (DM), is an autosomal dominant disorder with 2 genetically distinct forms. DM type 1 (DM1) is the more common form and is caused by abnormal expansion of cytosine/thymine/guanine (CTG) repeats in the DM protein kinase (DMPK) gene. Our study aimed to determine whether the age of onset is correlated with CTG repeat length in a population of pediatric patients with DM1. METHODS: We retrospectively identified 30 pediatric patients with DM1 that underwent DMPK testing, of which the clinical data of 17 was sufficient. The cohort was divided into 2 subgroups based on the clinical phenotype (congenital-onset vs. late-onset) and number of CTG repeats ( < 1,000 vs. ≥1,000). RESULTS: We found no significant difference between the age of onset and CTG repeat length in our pediatric patient population. Based on clinical subgrouping, we found that the congenital-onset subgroup was statistically different with respect to several variables, including prematurity, rate of admission to neonatal intensive care unit, need for respiratory support at birth, hypotonia, dysphagia, ventilator dependence, and functional status on last visit, compared to the late-onset subgroup. Based on genetic subgrouping, we found a single variable (poor feeding in neonate) that was significantly different in the large CTG subgroup than that in the small CTG subgroup. CONCLUSION: Clinical variables exhibiting statistically significant differences between the subgroups should be focused on prognosis and designing tailored management approaches for the patients; our findings will contribute to achieve this important goal for treating patients with DM1.
Age of Onset ; Cohort Studies ; Deglutition Disorders ; Genetic Association Studies ; Genotype ; Humans ; Infant, Newborn ; Intensive Care, Neonatal ; Muscle Hypotonia ; Myotonic Dystrophy ; Myotonin-Protein Kinase ; Parturition ; Phenotype ; Prognosis ; Retrospective Studies ; Ventilators, Mechanical

PURPOSE: Liver metabolism plays a pivotal role in the development of metabolic disorders. We aimed to investigate the clinical and laboratory risk factors associated with alanine aminotransferase (ALT) levels in young adolescents from an urban population in Korea. METHODS: A population of 120 apparently healthy adolescents aged 12–13 years was included in the cross-sectional design study; 58 were overweight or obese and 62 were of normal weight. We estimated anthropometric and laboratory measurements, including waist-to-height ratio, blood pressure, insulin sensitivity, aspartate aminotransferases (AST), ALT, and lipid profiles. RESULTS: The mean ages of the overweight or obese and normal weight participants were 12.9±0.3 and 13.0±0.3 years, respectively. Height, weight, body mass index, waist circumference, waist-to-height ratio, systolic and diastolic blood pressure, AST, ALT, total cholesterol, low-density lipoprotein-cholesterol, triglyceride, insulin, and the homeostatic model assessment of insulin resistance (HOMA-IR) score were significantly higher and the high-density lipoprotein-cholesterol and quantitative insulin-sensitivity check index were significantly lower in the overweight/obese participants in comparison to the normal-weight participants (all P < 0.05). In multivariate linear regression analysis, waist-to-height ratio, systolic blood pressure, and HOMA-IR score were independently and positively associated with serum ALT levels. CONCLUSION: Screening for ALT levels in adolescents may help to differentiate those at risk of metabolic abnormalities and thus prevent disease progression at an early age.
Adolescent ; Alanine Transaminase ; Alanine ; Aspartate Aminotransferases ; Blood Pressure ; Body Weight ; Cholesterol ; Disease Progression ; Humans ; Insulin ; Insulin Resistance ; Korea ; Linear Models ; Liver ; Mass Screening ; Metabolism ; Obesity ; Overweight ; Risk Factors ; Triglycerides ; Urban Population ; Waist Circumference

Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates
Ammonia ; Astrocytes ; Brain Edema ; Coma ; Diagnosis ; Edema ; Emergency Treatment ; Humans ; Hyperammonemia ; Infant, Newborn ; Metabolism ; Metabolism, Inborn Errors ; Peritoneal Dialysis ; Plasma ; Prescriptions ; Prognosis ; Renal Dialysis ; Renal Replacement Therapy ; Urea

PURPOSE: Prognostic factors of coronary aneurysms in Kawasaki disease have been investigated in many studies. The aim of this study was to identify risk factors associated with early and late coronary artery outcomes in treated patients with Kawasaki disease. METHODS: A total of 392 patients diagnosed with Kawasaki disease from January 2012 to December 2015 in Pusan National University Children’s Hospital were retrospectively selected as subjects of the present study to determine risk factors for coronary aneurysms and persistence of coronary aneurysms after a 1-year follow-up. RESULTS: Coronary aneurysms were detected in 30 of 392 patients within 1 month after the occurrence of Kawasaki disease. Coronary aneurysms persisted in 5 of 30 patients after a 1-year follow-up. A long duration of fever (adjusted odds ratio [OR], 1.47; 95% confidence interval [CI], 1.06–2.02; P=0.018) and high platelet count (adjusted OR, 1.00; 95% CI, 1.00–1.01; P=0.009) were found to be independent factors to predict the development of coronary aneurysms in the early phase. Initial coronary severity (adjusted OR, 46.0; 95% CI, 2.01–1047.80; P=0.016) and a high white blood cell count (adjusted OR, 1.17; 95% CI, 1.01–1.36; P=0.028) were found to be significant factors for the persistence of late coronary aneurysms in univariate analysis. However, no significant factors were found in multivariate analysis. CONCLUSION: These data are from early and late follow-up of coronary aneurysms in our unit. Further studies are needed to determine the mechanisms involved in the disappearance of coronary aneurysms and related factors.
Busan ; Coronary Aneurysm ; Coronary Vessels ; Fever ; Follow-Up Studies ; Humans ; Leukocyte Count ; Mucocutaneous Lymph Node Syndrome ; Multivariate Analysis ; Odds Ratio ; Platelet Count ; Prognosis ; Retrospective Studies ; Risk Factors

PURPOSE: Respiratory syncytial virus (RSV) infection can cause various neurological complications. This study aimed to investigate the RSV-associated neurologic manifestations that present with seizures. METHODS: We retrospectively reviewed the medical records of patients aged less than 15 years with laboratory-confirmed RSV infections and seizures between January 2011 and December 2016 in a regional hospital in South Korea. RESULTS: During this period, 1,193 patients with laboratory-confirmed RSV infection were identified. Of these, 35 (35 of 1,193, 2.93%; boys, 19; girls, 16; mean age: 20.8±16.6 months) presented with seizure. Febrile seizure was the most common diagnosis (27 of 35, 77.1%); simple febrile seizures in 13 patients (13 of 27, 48.1%) and complex febrile seizures in 14 (14 of 27, 51.9%). Afebrile seizures without meningitis or encephalopathy were observed in 5 patients (5 of 35, 14.3%), seizures with meningitis in 2 (2 of 35, 5.7%), and seizure with encephalopathy in 1 (1 of 35, 2.9%) patient. Lower respiratory symptoms were not observed in 8 patients. In a patient with encephalopathy, brain diffusion-weighted magnetic resonance imaging revealed transient changes in white matter, suggesting cytotoxic edema as the mechanism underlying encephalopathy. Most patients recovered with general management, and progression to epilepsy was noted in only 1 patient. CONCLUSION: Although febrile seizures are the most common type of seizure associated with RSV infection, the proportion of patients with complex febrile seizures was higher than that of those with general febrile seizures. Transient cytotoxic edema may be a pathogenic mechanism in RSV-related encephalopathy with seizures.
Brain ; Brain Diseases ; Child ; Diagnosis ; Edema ; Epilepsy ; Female ; Humans ; Korea ; Magnetic Resonance Imaging ; Medical Records ; Meningitis ; Neurologic Manifestations ; Respiratory Syncytial Viruses ; Retrospective Studies ; Seizures ; Seizures, Febrile ; White Matter

PURPOSE: In this meta-analysis, relevant case-control and cohort studies were pooled to evaluate the association between preeclampsia and the risk of autism spectrum disorders (ASDs) in children. METHODS: A search for relevant studies in major databases, including Web of Science, PubMed, and Scopus, was performed up to May 2018. The odds ratios (ORs) or rate ratios (RRs) with 95% confidence intervals (CIs) were extracted from eligible studies to determine the association among studies. RESULTS: The pooled estimates of ORs and RRs indicated a significant association between preeclampsia and ASD [(OR, 1.36; 95% CI, 1.12–1.60) and (RR, 1.30; 95% CI, 1.20–1.41)]. CONCLUSIONS: Despite existing controversy, our findings indicated that preeclampsia was associated with an increased risk of ASD among children.
Autism Spectrum Disorder ; Autistic Disorder ; Case-Control Studies ; Child ; Cohort Studies ; Humans ; Odds Ratio ; Pre-Eclampsia ; Pregnancy

No abstract available.
Aneurysm ; Coronary Vessels ; Mucocutaneous Lymph Node Syndrome ; Risk Factors