The Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is comprised of vaginal atresia with other variable Mullerian duct abnormalities. Its features include an absent or very short vagina and a uterus that can be absent or immaturely formed. Female with MRKH syndrome have functioning ovaries, normal external genitalia and the typical 46, XX, karyotype. MRKH syndrome is the second most common cause of primary amenorrhea, usually remains undetected until the patient presents with primary amenorrhea despite normal sexual female development. We report a case of MRKH syndrome, associated with ovarian follicular cyst, in a 14-years-old girl who visited for primary amenorrhea and sexual precosity.
Amenorrhea* ; Female ; Follicular Cyst ; Genitalia ; Humans ; Karyotype ; Ovary ; Uterus ; Vagina

PURPOSE: Intradermal BCG vaccine has not well been accepted by pediatric practitioners due to BCG lymphadenitis. Therefore, this study was undertaken to find out the incidence of lymphadenitis following intradermal BCG vaccination and its clinical outcome. METHODS: One thousand and fifty infants, who received intradermal BCG(French 1173 P2, Korea Tuberculosis Association) vaccination in the Well Baby Clinic of Hanyang University Hospital from July 2001 to January 2004, were included in the study. Severe local reactions at the injection site and any mass noted on surrounding areas were reported to, and evaluated by, pediatricians. Surgical procedures, either surgical resection or needle aspiration, were recommended when lymph nodes progressed to suppurate without regression. RESULTS: Twenty infants(1.9 percent) developed lymphadenitis 2 to 8 months following vaccination. The incidence of BCG lymphadenitis was significantly higher in infants born with intrauterine period of <38 weeks and birth weight of <2,700 g. The lymph nodes became suppurative in 7/17 infants (41.2 percent) and four infants required surgical procedures with which the rate for the requirement of surgical procedures among intradermal BCG vaccinnes approximated to be 0.45 percent. There was no correlation between the size of lymph nodes and suppuration, however surgical procedures were required significantly more often for lymph nodes of greater than 3 cm in diameter. CONCLUSION: The incidence of BCG lymphadenitis following intradermal BCG(French 1173 P2, Korea Tuberculosis Association) vaccinations would be more than 1.9 percent, when considering cases of lymphadenits not reported. More efforts need to be paid to decrease the incidence of BCG lymphadenitis in order to promote intradermal BCG vaccination in Korea.
BCG Vaccine ; Birth Weight ; Humans ; Incidence ; Infant ; Korea ; Lymph Nodes ; Lymphadenitis* ; Mycobacterium bovis* ; Needles ; Suppuration ; Tuberculosis ; Vaccination*

PURPOSE: This study assessed the association between the ratio of leukotriene E4 (LTE4) to fractional exhaled nitric oxide (FENO) in the response of children with exercise-induced bronchoconstriction (EIB) enrolled in a therapeutic trial with montelukast or inhaled corticosteroid (fluticasone propionate [FP]). METHODS: Children aged 6 to 18 years with EIB were randomized in a 4-week, placebo-controlled, double-blinded trial with montelukast or FP. Before and after treatment, treadmill exercise challenges were performed. The LTE4 levels in the induced sputum and urine and the FENO levels were measured in subjects before and 30 minutes after the exercise challenges. The same tests were conducted after treatment. RESULTS: A total of 24 patients completed the study: 12 in the montelukast group and 12 in FP group. Both study groups displayed a similar postexercise maximum decrease in forced expiratory volume in one second (FEV1) before treatment as well as after treatment. However, there were significant differences in the magnitude of change between the two (Delta; -18.38+/-14.53% vs. -4.67+/-8.12% for the montelukast and FP groups, respectively; P=0.021). The Delta logarithmic sputum baseline and postexercise LTE4/FENO ratio were significantly lower in the montelukast group than in the FP group (baseline; -0.09+/-0.21 vs. -0.024+/-0.03, P=0.045; postexercise, -0.61+/-0.33 vs. -0.11+/-0.28, P=0.023). CONCLUSIONS: These data indicate that the efficacy of montelukast for preventing a maximum decrease in FEV1 after exercise is significantly higher than that of FP, and the high LTE4/FENO ratio is associated with a greater response to montelukast than to FP for EIB therapy. These results suggest that LTE4 may play an important role in EIB.
Acetates ; Aged ; Bronchoconstriction ; Child ; Diethylpropion ; Forced Expiratory Volume ; Humans ; Leukotriene E4 ; Nitric Oxide ; Quinolines ; Sputum

PURPOSE: The present study investigates the long-term effects of intravenous immunoglobulin (IVIg) therapy for the treatment of moderate to severe childhood atopic dermatitis (AD). Previous research indicates that IVIg can treat severe AD; however, the effectiveness of IVIg has not been confirmed in prospective, blinded clinical trials. METHODS: Forty eligible children with moderate to severe AD, as defined by the criteria of Hanifin and Rajka, were enrolled in a randomized, placebo-controlled study. After the completion of an initial screening visit (V0), the patients were randomly allocated into therapy (n=30) and control (n=10) groups (V1). Thirty children were each treated with three injections of 2.0 g/kg IVIg at 1-month intervals over a 12-week period. Ten children were treated with placebo. Assessments were conducted after each injection (V2, V3, and V4) and at 3 (V5) and 6 months (V6) after completed treatment. RESULTS: The disease severity index was significantly decreased at V5 compared with the value at V1 (P<0.05). There were no significant changes in the total IgE level or total eosinophil count in peripheral blood at the last injection (V4) compared with the value at V1. The interleukin (IL)-5/interferon (IFN)-gamma ratio was assessed in T-helper 1 (Th1) and Th2 cells. The ratio significantly decreased between V1 and V5, after which it increased, such that the ratio at V6 was not significantly different from that at V1. Compared with the level at V1, the intercellular cell adhesion molecule-1 level at V4 did not differ significantly, but the level at V5 was lower. CONCLUSIONS: This study suggests that IVIg therapy may clinically improve AD in patients after 3 months of therapy, but the improvement may decline by 6 months after therapy.
Cell Adhesion ; Child ; Dermatitis, Atopic ; Eosinophils ; Humans ; Immunization, Passive ; Immunoglobulin E ; Immunoglobulins ; Immunoglobulins, Intravenous ; Interleukins ; Mass Screening ; Prospective Studies ; Th2 Cells

PURPOSE: The social and economic impact of asthma is remarkable worldwide. To date, there have been many unanswered questions about factors related to asthma progression and persistence. This study focused on possible risk factors for persistent asthma that had developed between infancy and late childhood. METHODS: Sixty-seven children with persistent mild-to-moderate asthma were enrolled in this study. They were classified into 2 groups according to the Global Initiative for Asthma (GINA) guideline 2006: early-onset (<3 years, n=28) and late-onset (>3 years, n=39) asthmatics. All patients were interviewed on the personal and familial history of atopy, breast feeding, parental smoking and the recent use of inhaled corticosteroids. We performed spirometry, and skin prick tests and measured body mass index, serum allergen-specific IgE, serum eosinophil counts and serum ECP in asthmatics. All asthmatics underwent the bronchial challenge by methacholine inhalation and outdoor free running. RESULTS: Risk factors such as eczema and frequent wheezing were more common in early-onset asthmatics than in late-onset asthmatics (P<0.05). However, there was no difference between the 2 groups in the overall incidence of airway hyperresponsiveness (AHR) determined by the PC20 and postexercise decrease of FEV1 (P>0.05). Inhaled corticosteroids were more frequently used in early-onset asthmatics than in late-onset asthmatics (P<0.0001). A reciprocal relationship between FEV1/FVC and the duration of asthma was also detected in persistent asthmatics (n=57, r=-0.398, P=0.002). CONCLUSION: The results of this study suggest that atopic dermatitis and frequent wheezing may be important risk factors for the persistence of asthma and lung function decline from early to late childhood.
Adrenal Cortex Hormones ; Asthma ; Body Mass Index ; Breast Feeding ; Bronchial Provocation Tests ; Child ; Dermatitis, Atopic ; Eczema ; Eosinophils ; Humans ; Immunoglobulin E ; Incidence ; Inflammation ; Inhalation ; Lung ; Methacholine Chloride ; Parents ; Respiratory Sounds ; Risk Factors ; Running ; Skin ; Smoke ; Smoking ; Spirometry

PURPOSE: Neonatal lupus is characterized by congenital complete heart block(CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with systemic lupus erythematosus(SLE). This study aims to examine the incidence of CCHB and clinical outcome in neonates born to mothers with SLE. METHODS: The study group consisted of 49 neonates, born from 57 pregnancies of 55 women with SLE, diagnosed at Hanyang University Hospital for the period between January 1997 and January 2005. Clinical and laboratory data were retrospectively identified from medical record. RESULTS: There were 5(8.8 percent) spontaneous abortions and one(1.8 percent) still births among 57 pregnancies of 55 mothers. Of 49 live births, 15(26.3 percent) were premature and eight(12.3 percent) were small for their gestational age. There was one(1.8 percent) CCHB suspected during pregnancy on fetal echocardiograpy in a fetus of mother with systemic lupus erythematosus and the fetus was not born by artificial abortion because of mother. There was no CCHB among EKG findings of 49 newborns. Laboratory testing showed hematologic abnormalities among 25.6 percent(10/39) of the babies. 5.1 percent(2/39) and 7.7 percent(3/39) of them were diagnosed as neutropenia, and thrombocytopenia was seen respectively. Anti-SSA(Ro) and antiphospholipid antibodies were predictive factors for prematurity(P=0.003, P=0.049). Anticardiolipin antibodies were predictive factors for ventilatory care(P=0.018). CONCLUSION: The incidence of CCHB among neonates born to mothers with SLE, which was measured in this study, was lower than that in earlier studies. A high incidence of hematologic abnormalities was found in our study. It is suggested that careful examination should be made of skin for the diagnosis of neonatal lupus.
Abortion, Spontaneous ; Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Diagnosis ; Electrocardiography ; Exanthema ; Female ; Fetus ; Gestational Age ; Heart* ; Humans ; Incidence ; Infant ; Infant, Newborn ; Live Birth ; Lupus Erythematosus, Systemic* ; Medical Records ; Mothers* ; Neutropenia ; Parturition ; Pregnancy ; Retrospective Studies ; Skin ; Thrombocytopenia

PURPOSE: The prevalence of asthma and obesity is increasing concomitantly, but the link between asthma and obesity is unclear. We sought to address possible roles of leptin and adiponectin in the development of asthma, and changes in pulmonary function in overweight children. METHODS: Four study groups of 61 children aged 6 to 18 years (mean age, 9.69+/-2.16) were enrolled: (1) 14 mild-to-moderate asthmatics with overweight, (2) 16 mild-to-moderate asthmatics with normal weight, (3) 16 obese subjects without asthma, and (4) 15 healthy controls. We measured biomarkers in blood, including total and allergen-specific IgE, eosinophil, eosinophilc cationic protein (ECP), leptin, adiponectin, interleukin (IL)-6, tumor necrosis factor-alpha (TNF-alpha), lipid profiles, insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein 3 (IGF-BP3). Body mass index (BMI), antioxidants and micronutrients in a daily diet were evaluated by the questionnaire. We performed the bronchial challenge test by methacholine inhalation and free running, respectively. RESULTS: The leptin levels was apparently high, and the adiponectin level was low in the over-weight children, as depicting a significant inverse correlation between the 2 variables (R=-0.479; P<0.001). The FEV(1)/FVC ratio was low in the overweight children regardless of the presence of asthma. However, the effect of IL-6, TNF-alpha, nutrients, and other variables on asthma development in the overweight children with asthma was not verified. CONCLUSION: In this study, the levels of leptin, adiponectin or other obesity-related biomarkers were not independently associated with asthma. Therefore, it is concluded that obesity may not be an important factor in pulmonary function impairment.
Adiponectin ; Aged ; Aluminum Hydroxide ; Antioxidants ; Asthma ; Biomarkers ; Body Mass Index ; Bronchial Provocation Tests ; Carbonates ; Child ; Diet ; Eosinophils ; Humans ; Immunoglobulin E ; Inhalation ; Insulin-Like Growth Factor Binding Protein 3 ; Interleukin-6 ; Interleukins ; Leptin ; Methacholine Chloride ; Micronutrients ; Obesity ; Overweight ; Prevalence ; Surveys and Questionnaires ; Running ; Tumor Necrosis Factor-alpha

Most guidelines for chronic urticaria (CU) in infants and children are based on limited pediatric evidence. Current evidence used to guide treatment in children is extrapolated from data focusing on older age groups. CU in children is a different and complex condition than that in adults. Furthermore, there is little published information regarding urticaria in Korean children. The aim of the present article is to review recent research on chronic childhood urticaria and improve the current understanding of its pathogenesis and management. The classification and definition of urticaria in adults also applies to children. CU is defined as a daily occurrence of spontaneous wheals, angioedema, or both for >6 weeks. The precise pathophysiology of CU is unknown and the rates of successful identification of a cause in children with CU vary from 20%-50%. There is no established laboratory test to evaluate the presence of urticaria. The natural course of childhood CU is undetermined, with limited reports discussing long-term outcomes. Second-generation H1 antihistamines are the cornerstone of management, while limited therapeutic drugs are available for adults.
Adult ; Angioedema ; Child* ; Classification ; Diagnosis* ; Histamine Antagonists ; Humans ; Infant ; Urticaria*

Purpose: Respiratory virus infection is a common cause of hospitalization in children. Rapid testing for respiratory viruses, such as the FilmArray method, can be clinically useful. However, insufficient evidence exists to support its use in standard clinical care. Methods: We retrospectively analyzed data from children under 18 years old who received the multiplex real-time polymerase chain reaction array (multiplex RT-PCR) method in 2017 and by FilmArray respiratory panel (FilmArray RP) in 2018. Results: Between January, 2017 and December, 2018, we reviewed data from 1,480 hospitalized children. The number of children with virus detection in respiratory viral PCR was 523 in the multiplex RT-PCR method and 419 in the FilmArray method. Seasonal virus outbreak patterns were similar to those of Korea Centers for Disease Control and Prevention in both groups. There was no difference between the 2 groups in the mean length of hospital stay. The time from admission to isolation by influenza infection was significantly shorter in the FilmArray group than in the multiplex RT-PCR group among patients who were not diagnosed with influenza infection by rapid antigen test at the time of admission. Conclusion The use of FilmArray method for respiratory viruses did not diminish length of hospital stay. However, the FilmArray method may quickly detect the prevalence of respiratory infection and aid in clinical treatment. In addition, it was related with a reduced time from admission to isolation by influenza infection in hospitalized children who were not identified with influenza infection by rapid antigen test at the time of admission.

Congenital laryngeal atresia is a rare cause of airway obstruction that is almost always lethal within a few minutes after birth. Therefore congenital laryngeal atresia should be diagnosed in the prenatal period. If not, it should be considered in newborn infant with life-threatening symptoms at birth such as cyanosis and dyspnea that need emergent procedures like a tracheotomy. We report a case of congenital laryngeal atresia with single umbilical artery diagnosed in a one-day-old neonate who required a tracheotomy and ventilator care after intubation failure.
Airway Obstruction ; Cyanosis ; Dyspnea ; Humans ; Infant, Newborn ; Intubation ; Parturition ; Single Umbilical Artery* ; Tracheotomy* ; Ventilators, Mechanical