Hereditary angioedema (HAE) is a rare autosomal dominant disease that usually occurs in adolescence and early adulthood. It is characterized by recurrent non-pitting edema involving the skin and intestinal tract, especially the extremities and face. It is not associated with urticaria and pruritus. The cause is known to be the deficiency of C1 inhibitor. We herein report a 7-year-old girl with HAE who had recurrent episodes of swelling of the extremities and face without urticaria and pruritus. Her great grandmother had suffered from the same symptoms. The level of serum C4 was 8.01 mg/dL (normal: 10-40 mg/dL). The level of C1 inhibitor was 5.0 mg/dL (normal: 18-40 mg/dL). To our knowledge, this is the first pediatric case with typical clinical symptoms of HAE and C1 esterase inhibitor deficiency in Korea.
Adolescent ; Angioedema ; Angioedemas, Hereditary ; Complement C1 Inhibitor Protein ; Edema ; Extremities ; Humans ; Korea ; Pruritus ; Skin ; Urticaria

Egg allergy is one of the most common food allergies in children and has a wide spectrum of clinical presentation including anaphylaxis. Many studies suggested egg-specific IgE cutoff values (diagnostic decision point) and skin prick test size that predict a clinical allergic reaction without oral food challenges. Some patients may react to all forms of egg including raw egg, but many egg-allergic patients tolerate baked egg products. A few studies reported that a high concentration of ovomucoid-specific IgE antibody indicates a high risk of reacting to heated egg white. Recently it has been suggested that regular ingestion of baked egg products may hasten tolerance development. Egg allergy may be more persistent than previously thought. The treatment of egg allergy still relies on dietary avoidance of egg-containing foods until tolerance has developed. In recent years there has been increasing success in clinical trials of egg oral immunotherapy, and oral immunotherapy can be a promising treatment modality for providing protection from reactions caused by accidental egg exposure. However, concerns regarding the safety and long-term efficacy still preclude the general use of oral immunotherapy in clinical practice. In this article, the recent literature regarding egg allergens, clinical presentation, diagnosis, management and natural history of egg allergy will be reviewed.
Allergens ; Anaphylaxis ; Child* ; Diagnosis ; Eating ; Egg Hypersensitivity* ; Egg White ; Food Hypersensitivity ; Hot Temperature ; Humans ; Hypersensitivity ; Immunoglobulin E ; Immunotherapy ; Natural History ; Ovomucin ; Ovum ; Skin

No abstract available.
Prevalence* ; Urticaria*

PURPOSE: The present study was performed to determine the factor, either duration or the temperature of heat treatment, exerting maximal and significant influence on the composition and allergenicity of egg white (EW) proteins. METHODS: Raw EW and 4 kinds of heated EW (fried EW, boiled EW for 10 minutes, boiled EW for 30 minutes, and baked EW for 20 minutes at 170degrees C) were prepared, and subsequently protein extraction was carried out. The proteins were separated by SDS-PAGE, and then immunoglobulin E (IgE) immunoblots were performed with the sera of 7 egg-allergic patients. Furthermore, the antigenic activities of ovalbumin (OVA), ovomucoid (OM), and ovotransferrin (OT) in different EW samples were measured by inhibition enzyme-linked Immuno-sorbent assay (ELISA). RESULTS: In SDS-PAGE analysis, the intensity of the protein band at 45 kD (corresponding to OVA) decreased significantly in boiled EW (30 minutes) and baked EW, but no change was observed in the case of boiled EW for 10 minutes. In IgE immunoblots, the IgE response to 34-50 kD (OM and OVA) in boiled EW for 30 minutes decreased significantly, when compared with raw EW and other heated EWs. In inhibition ELISA, a significant decrease in the OVA antigenic activity was observed in boiled EW for 30 minutes amongst other heated EW samples. However, OM antigenic activity in all kinds of heated EW including boiled EW for 30 minutes did not reduce after heat treatment. The OT antigenic activity nearly disappeared in heated EWs except in the case of boiled EW for 10 minutes. CONCLUSIONS: Amongst 4 kinds of heated EWs, the boiled EW for 30 minutes showed the most significant changes both in composition and reduction in allergenicity. Our results revealed that the duration of heat treatment had more influence on the composition and allergenicity of EW proteins than the temperature.
Conalbumin ; Egg Hypersensitivity ; Egg Proteins ; Egg White ; Electrophoresis, Polyacrylamide Gel ; Enzyme-Linked Immunosorbent Assay ; Hot Temperature ; Humans ; Immunoglobulin E ; Immunoglobulins ; Ovalbumin ; Ovomucin ; Ovum ; Proteins

PURPOSE: This study was performed to investigate the epidemiologic and clinical features of human metapneumovirus (hMPV). METHODS: We performed real-time reverse transcription polymerase chain reaction with nasopharyngeal samples from 2,403 children who were hospitalized with acute respiratory infection. Then medical records of 120 children, who were diagnosed with hMPV respiratory infection between 2011 and 2014, were retrospectively analyzed retrospectively and compared to epidemiologic data on respiratory virus infection reported by Korea Centers for Disease Control and Prevention. RESULTS: We detected 120 hMPV-positive cases out of 1,723 virus-positive specimens (7.0%), which prevailed mostly in spring between March and May. Respiratory infection with hMPV was more common in female patients (55.0%) and hPMV infection was prevalent among children aged <5 years (80.8%). Coinfections with other respiratory viruses were observed in 34 patients of 120 hMPV-positive cases (28.3%), mostly with rhinovirus (52.9%). In addition, hPMV infection mostly presented with pneumonia (71.7%). Among 120 hMPV-positive patients during the recent 4 years, most (68.3%) were diagnosed in 2014. During 2011–2014, influenza virus infection was prevalent mainly from January to March, and hMPV infection started to appear just after the end of influenza virus outbreak. CONCLUSION: In Korean children, hMPV was a common causative organism of viral pneumonia during the spring season. The hMPV infection pandemic was observed in 2014 and the clinical importance of hMPV has recently been increasing. Therefore, additional studies are required to define the epidemiology, disease characteristics caused by hMPV, and the cause of recent outbreak.
Centers for Disease Control and Prevention (U.S.) ; Child* ; Coinfection ; Epidemiology* ; Female ; Humans* ; Korea ; Medical Records ; Metapneumovirus* ; Orthomyxoviridae ; Pandemics ; Pneumonia ; Pneumonia, Viral ; Polymerase Chain Reaction ; Respiratory Tract Infections ; Retrospective Studies ; Reverse Transcription ; Rhinovirus ; Seasons

Laryngomalacia, the most common cause of stridor in infants, is characterized by the inward collapse of soft and immature upper laryngeal cartilages during inspiration, resulting in airway obstruction at the supraglottic level. Acquired laryngomalacia is a rare condition that mainly occurs following significant neurological dysfunctions associated with cerebrovascular disease, head and neck surgery, or cervical trauma. We present a case of acquired idiopathic laryngomalacia in a 12-year-old adolescent caused by the prolapse of redundant arytenoid mucosa. The patient exhibited no neurological dysfunctions or laryngeal deformities. However, he had allergic rhinitis accompanied by high serum immunoglobulin E levels. His symptoms worsened after being infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Furthermore, allergic rhinitis or SARS-CoV-2 infection may have worsened preexisting asymptomatic congenital or acquired laryngomalacia through neurological damage. Acquired idiopathic laryngomalacia is rale in children. In cases where children and adolescents present with a sudden onset of inspiratory stridor, it is essential to perform a laryngoscopic examination for identifying potential cases of acquired laryngomalacia.

PURPOSE: This study was conducted to evaluate the difference of clinical characteristics of pneumonia in children caused by Mycoplasma pneumoniae, according to their chest radiographic patterns. METHODS: We analyzed medical records of 921 children who were admitted to Soonchunhyang University Buchon Hospital due to M. pneumoniae pneumonia from January 2008 to December 2011. Enrolled children were divided into 2 groups by radiological patterns: lobar/lobular pneumonia group (group 1) and broncho/interstitial pneumonia group (group 2). RESULTS: The number of patients in group 1 was 295 (32%) and in group 2, 626 (68%). Lobar/lobular pneumonia occurred in older children compared to broncho/interstitial pneumonia (mean age, 6.4 years vs. 4.2 years; P=0.00). Group 1 had significantly longer durations of fever and hospitalization than group 2. The frequency of pleural effusion was significantly higher in group 1. Erythrocyte sedimentation rate and C-reactive protein values were higher in group 1. Coinfections with respiratory viruses were more frequent in group 2. The history of allergic diseases were more common in group 2 (P=0.006). In 2011, lobar/lobular pneumonia was more frequent and the duration of fever was longer compared with 2008-2010. CONCLUSION: In M. pneumoniae pneumonia, patients with lobar/lobular pneumonia were more older and had more severe clinical features and laboratory findings. Because there was an outbreak with severe clinical course in 2011, we wonder that the outbreak was related to the macrolide resistant M. pneumoniae. Careful attention about clinical course and consequences of patients with lobar/lobular pneumonia is required.
Blood Sedimentation ; C-Reactive Protein ; Child ; Coinfection ; Fever ; Hospitalization ; Humans ; Medical Records ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pleural Effusion ; Pneumonia* ; Pneumonia, Mycoplasma* ; Radiography, Thoracic

PURPOSE: It is known that ovomucoid, an egg allergen, is heat resistant and remains soluble after heating. However, a recent study showed that the antigenic activity of ovomucoid could be reduced by heating when egg white (EW) was mixed with wheat flour. This study was performed to determine the influence of wheat flour on the antigenic activities of EW proteins when EW is heated, and the influence of the duration of heat treatment. METHODS: A mixture of EW and wheat flour was kneaded for 10 minutes and then baked at 180degrees C for 10 minutes and 30 minutes. The EW without wheat flour was also heated at 180degrees C for 10 minutes and 30 minutes. The proteins were separated by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE), and IgE immunoblotting was performed with the pooled sera of 5 egg-allergic patients. The antigenic activities of ovomucoid in different EW samples were measured by inhibition enzyme-linked immunosorbent assay (ELISA). RESULTS: 1) SDS-PAGE: the intensity of the 37-50 kD bands (overlapped bands of ovomucoid and ovalbumin) decreased significantly in the mixture of EW and wheat flour baked for 30 minutes, compared with the mixture baked for 10 minutes, heated EW and raw EW. 2) IgE immunoblot: in the mixture of EW and wheat, a remarkable decrease of IgE reactivity to 37-50 kD was observed when baked for 30 minutes. 3) Inhibition ELISA: the antigenic activity of ovomucoid decreased significantly in the mixture of EW and wheat baked for 30 minutes, but not in the heated pure EW. CONCLUSIONS: This study showed that the antigenic activity of ovomucoid can be reduced by baking EW with wheat flour. The decrease in ovomucoid antigenicity in the baked mixture of EW and wheat flour was dependent on the time of heat treatment, indicating that heating should be prolonged to achieve a reduction in ovomucoid antigenic activity.
Acrylic Resins ; Egg Proteins ; Egg White ; Electrophoresis, Polyacrylamide Gel ; Enzyme-Linked Immunosorbent Assay ; Flour ; Heating ; Hot Temperature ; Humans ; Immunoblotting ; Immunoglobulin E ; Ovomucin ; Ovum ; Proteins ; Sodium Dodecyl Sulfate ; Triticum

Purpose: Recent clinical trials have successfully used oral immunotherapy (OIT) to treat food allergies. Probiotics have immunomodulatory effects by balancing Th1/Th2 immunity and enhancing regulatory T-cell activity. In this study, we analyzed the effects of OIT, probiotics alone, and probiotics administered simultaneously with OIT in a mouse model of egg allergy. Methods: C3H/HeJ mice were sensitized by intragastric administration of ovomucoid (OM) with cholera toxin. For the OIT regime, increasing doses of OM were administered orally to sensitized mice. Lactobacillus casei variety ramnosus (Lcr35) was also administered. The mice were divided into 4 groups: control (no OIT), OIT, Lcr35, and OIT plus Lcr35 (OIT + Lcr35). The effects of OIT and Lcr35 treatment were estimated based on the symptom score, rectal temperature, serum levels of OM-specific immunoglobulin (Ig)E, IgA, IgG1, and IgG2a immediately after and 2 weeks after ceasing treatment and histological staining of the small intestine. Results: The severity of anaphylaxis decreased in all treatment groups. Simultaneous administration of Lcr35 and OIT decreased the severity of anaphylaxis compared to controls and the OIT group. The protective effects were sustained 2 weeks after ceasing treatment in all treatment groups. A significant decrease in OM-specific IgA, IgG1, and IgG2a levels was observed in both the OIT and OIT plus Lcr35 groups. However, a significant decrease in the OM-specific IgE level was observed only in OIT plus Lcr35 treated mice and was sustained 2 weeks after ceasing treatment. Mucin amounts in the small intestine decreased after OIT, OIT plus Lcr35, and Lcr35 treatment with the lowest in the OIT plus Lcr35 group. Conclusions Lcr35 treatment during OIT had some synergic effect for protection against anaphylaxis in a mice model of egg allergy. These findings should be confirmed in future animal studies including more detailed immunological profiles and human studies.

PURPOSE: This report describes the results of a survey of the characteristics of pertussis in children from a single institution and compares it to data from the Korea Centers of Disease Control (KCDC). METHODS: We retrospectively evaluated the medical records of 17 and 6 patients diagnosed with pertussis and parapertussis, respectively, at Soonchunhyang University Bucheon Hospital from January 2005 to January 2017. RESULTS: Of the 17 patients with pertussis, 9 were under 1 year of age (52.9%), 3 were aged between 1 and 10 years (17.6%), and 5 were over 10 years of age (29.4%). Seven patients (41.2%) had never received diphtheria-tetanus-acellular pertussis vaccines, of which 5 were infants below 2 months of age and 2 were 10 years old and lived in China. Four patients showed the initial symptoms of cough in China. The sources of infection were the parents (2 cases) and the siblings (8 cases). All patients showed prolonged severe cough and the average duration of cough was 26 days. Severe symptoms, including dyspnea, cyanosis, apnea, and seizures, were observed in the children under 2 months of age. According to the recent 10-year KCDC data, the highest rate of pertussis diagnosis was noted in infants (47.8%), followed by adolescents (18.7%). Six patients with parapertussis also presented with prolonged severe cough without any other severe symptoms. Lymphocytosis was not found, unlike the patients with pertussis. CONCLUSION: The possibility of pertussis and parapertussis should be considered among patients with prolonged severe cough, especially in infants and adolescents.
Adolescent ; Apnea ; Bordetella parapertussis ; Bordetella pertussis ; Child ; China ; Cough ; Cyanosis ; Diagnosis ; Diphtheria-Tetanus-acellular Pertussis Vaccines ; Dyspnea ; Gyeonggi-do ; Humans ; Infant ; Korea ; Lymphocytosis ; Medical Records ; Parents ; Retrospective Studies ; Seizures ; Siblings ; Whooping Cough