Trends of Sensitization to Inhalant Allergens in Korean Children Over the Last 10 Years

Purpose@#Climate and lifestyle changes increase an individual’s susceptibility to various allergens and also the incidence of allergic diseases. We aimed to examine the changes in sensitization rate for aeroallergens over a 10-year period in Korean children. @*Materials and Methods@#We retrospectively reviewed the medical records of 4493 children who visited the allergy clinic at a tertiary hospital in Korea for allergic rhinitis or asthma from January 2009 to December 2018. The serum specific immunoglobulin E (IgE) levels were measured to confirm the sensitization against Dermatophagoides farinae (D. farinae), Alternaria, weed and tree pollen mixtures, as well as cat and dog dander through ImmunoCAP test. @*Results@#D. farinae was the most common sensitizing aeroallergen (45.9%) during the 10-year span. The sensitization rate for tree pollen mixture (p for trend <0.001), weed pollen mixtures (p for trend <0.001), dog dander (p for trend=0.025), and cat dander (p for trend=0.003) showed ascending trends during the 10-year study period. Furthermore, the sensitization rate for multiple allergens (≥2) in 2018 increased significantly compared to that in 2009 (p for trend=0.013).Compared with children without sensitization to D. farinae, those with sensitization to D. farinae showed higher sensitization rates to other aeroallergens (p for interaction <0.001). @*Conclusion@#Children’s sensitization rate to cat and dog dander and weed and tree pollen mixtures significantly increased during the 10-year period in Korea. Children with sensitization to D. farinae are likely to be sensitized to other aeroallergens as well.

A Case of Von Hippel-Lindau Disease Presented with Multiple Pancreatic Cysts and Medullary Hemangioblastoma / 임상소아혈액종양

Von Hippel-Lindau (VHL) disease is a rare inherited cancer predisposition syndrome characterized by benign and malignant tumors in multiple organs, especially cerebellar hemangioblastomas, retinal angiomas, renal-cell carcinoma, and pheochromocytomas. Clinically, VHL disease also presents an increased risk for developing multiple visceral cysts in the pancreas, liver, and kidneys. Regular surveillance for VHL disease-associated tumors after early diagnosis is necessary for better outcomes in VHL disease. An 11-year-old girl was admitted with prolonged fever lasting for more than 10 days and cervical lymphadenopathy. She did not have a family history of cysts or malignancy. Initial blood tests showed mild leukopenia and moderate elevation in aspartate aminotransferase, alanine aminotransferase, and lactate dehydrogenase, but with normal amylase and lipase. Hepatobiliary ultrasonography and magnetic resonance cholangiopancreatography were done and revealed multiple cysts involving the whole pancreas with cyst sizes up to 1.6 cm, indicating VHL disease. Direct sequencing of the VHL gene showed a heterozygous duplication at codon 384 (c.384dup), which is predicted to cause a frameshift of the reading frame (p.Leu129Serfs*3). This was a novel pathogenic variant VHL gene. We carried out the surveillance protocol for VHL disease-associated tumors, and found a hemangioblastoma in the medulla of the brainstem. We are reporting an 11-year-old female patient of VHL disease with brainstem hemangioblastoma who could be suspected and diagnosed of VHL disease in asymptomatic state due to incidentally found multiple pancreatic cysts.

Multisystem Inflammatory Syndrome in Children Related to COVID-19: the First Case in Korea

Since mid-April 2020, cases of multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 that mimics Kawasaki disease (KD) have been reported in Europe and North America. However, no cases have been reported in Korea.We describe an 11-year old boy with fever, abdominal pain, and diarrhea who developed hypotension requiring inotropes in intensive care unit. His blood test revealed elevated inflammatory markers, thrombocytopenia, hypoalbuminemia, and coagulopathy. Afterward, he developed signs of KD such as conjunctival injection, strawberry tongue, cracked lip, and coronary artery dilatation, and parenchymal consolidation without respiratory symptoms. Microbiological tests were all negative including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) reverse transcription polymerase chain reaction.However, serum immunoglobulin G against SARS-CoV-2 was positive in repeated tests using enzyme-linked immunosorbent assay and fluorescent immunoassay. He was recovered well after intravenous immunoglobulin administration and discharged without complication on hospital day 13. We report the first Korean child who met all the criteria of MIS-C with features of incomplete KD or KD shock syndrome.

Relationship between Breastfeeding, Birth History, and Acute Pyelonephritis in Infants

BACKGROUND: Although the clinical importance of the immunological benefits of breastfeeding has been emphasized for decades, their direct relationship with acute pyelonephritis (APN) is still not clear. Our goal was to determine whether breastfeeding truly provides protection against APNs, while investigating the effects of other factors such as sex, age, mode of delivery, and birth weight on APN.METHODS: A total of 62 infants under 6 months of age who had both microbiologically and radiologically-confirmed APN were enrolled in the case group. Healthy infants (n = 178) who visited the hospital for scheduled vaccinations were enrolled in the control group. The following participant characteristics were compared between the case and control groups: age, sex, birth order among siblings, feeding methods, weight percentile by month, birth weight percentile by gestational age, gestational age at birth, and mode of delivery.RESULTS: Babies exclusively fed with manufactured infant formulae before 6 months of age had significantly higher risk for APN than breastfed or mixed-fed infants (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.687–7.031; P = 0.001). Firstborn babies had lower risk for APN than 2nd- or 3rd-born babies (OR, 0.43; 95% CI, 0.210–0.919). Other factors that increased the risk for APN were low birth weight percentiles (OR, 8.33; 95% CI, 2.300–30.166) and birth via caesarean section (OR, 2.32; 95% CI, 1.097–4.887). There were more preterm births in the case group (10.9% vs. 1.7%; P = 0.002), but this did not increase the risk for APN (OR, 4.47; P = 0.063).CONCLUSION: Feeding exclusively with formula before 6 months of age was related to higher risk for APN, which demonstrates that breastfeeding has a protective effect against APN. The other risk factors for APN were birth order (≥ 2nd-born), low birth weight, and birth via caesarean section.

Relationship between Breastfeeding, Birth History, and Acute Pyelonephritis in Infants

BACKGROUND@#Although the clinical importance of the immunological benefits of breastfeeding has been emphasized for decades, their direct relationship with acute pyelonephritis (APN) is still not clear. Our goal was to determine whether breastfeeding truly provides protection against APNs, while investigating the effects of other factors such as sex, age, mode of delivery, and birth weight on APN.@*METHODS@#A total of 62 infants under 6 months of age who had both microbiologically and radiologically-confirmed APN were enrolled in the case group. Healthy infants (n = 178) who visited the hospital for scheduled vaccinations were enrolled in the control group. The following participant characteristics were compared between the case and control groups: age, sex, birth order among siblings, feeding methods, weight percentile by month, birth weight percentile by gestational age, gestational age at birth, and mode of delivery.@*RESULTS@#Babies exclusively fed with manufactured infant formulae before 6 months of age had significantly higher risk for APN than breastfed or mixed-fed infants (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.687–7.031; P = 0.001). Firstborn babies had lower risk for APN than 2nd- or 3rd-born babies (OR, 0.43; 95% CI, 0.210–0.919). Other factors that increased the risk for APN were low birth weight percentiles (OR, 8.33; 95% CI, 2.300–30.166) and birth via caesarean section (OR, 2.32; 95% CI, 1.097–4.887). There were more preterm births in the case group (10.9% vs. 1.7%; P = 0.002), but this did not increase the risk for APN (OR, 4.47; P = 0.063).@*CONCLUSION@#Feeding exclusively with formula before 6 months of age was related to higher risk for APN, which demonstrates that breastfeeding has a protective effect against APN. The other risk factors for APN were birth order (≥ 2nd-born), low birth weight, and birth via caesarean section.

Relationship between Breastfeeding, Birth History, and Acute Pyelonephritis in Infants

BACKGROUND@#Although the clinical importance of the immunological benefits of breastfeeding has been emphasized for decades, their direct relationship with acute pyelonephritis (APN) is still not clear. Our goal was to determine whether breastfeeding truly provides protection against APNs, while investigating the effects of other factors such as sex, age, mode of delivery, and birth weight on APN.@*METHODS@#A total of 62 infants under 6 months of age who had both microbiologically and radiologically-confirmed APN were enrolled in the case group. Healthy infants (n = 178) who visited the hospital for scheduled vaccinations were enrolled in the control group. The following participant characteristics were compared between the case and control groups: age, sex, birth order among siblings, feeding methods, weight percentile by month, birth weight percentile by gestational age, gestational age at birth, and mode of delivery.@*RESULTS@#Babies exclusively fed with manufactured infant formulae before 6 months of age had significantly higher risk for APN than breastfed or mixed-fed infants (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.687–7.031; P = 0.001). Firstborn babies had lower risk for APN than 2nd- or 3rd-born babies (OR, 0.43; 95% CI, 0.210–0.919). Other factors that increased the risk for APN were low birth weight percentiles (OR, 8.33; 95% CI, 2.300–30.166) and birth via caesarean section (OR, 2.32; 95% CI, 1.097–4.887). There were more preterm births in the case group (10.9% vs. 1.7%; P = 0.002), but this did not increase the risk for APN (OR, 4.47; P = 0.063).@*CONCLUSION@#Feeding exclusively with formula before 6 months of age was related to higher risk for APN, which demonstrates that breastfeeding has a protective effect against APN. The other risk factors for APN were birth order (≥ 2nd-born), low birth weight, and birth via caesarean section.

Increased procalcitonin level is a risk factor for prolonged fever in children with Mycoplasma pneumonia / 소아과

PURPOSE: Macrolide-resistant Mycoplasma pneumoniae pneumonia (MPP) is characterized by prolonged fever and radiological progression despite macrolide treatment. Few studies have examined serum procalcitonin (PCT) level in children with MPP. We aimed to investigate the association of acute inflammation markers including PCT with clinical parameters in children with MPP. METHODS: A total of 147 children were recruited. The diagnosis of MPP relied on serial measurement of IgM antibody against mycoplasma and/or polymerase chain reaction. We evaluated the relationships between C-reactive protein (CRP), PCT, and lactate dehydrogenase (LDH) levels and white blood cell (WBC) counts, and clinical severity of the disease. We used multivariate logistic regression analysis to estimate the odds ratio for prolonged fever (>3 days after admission) and hospital stay (> 6 days), comparing quintiles 2–5 of the PCT levels with the lowest quintile. RESULTS: The serum PCT and CRP levels were higher in children with fever and hospital stay than in those with fever lasting ≤ 3 days after admission and hospital stay ≤ 6 days. CRP level was higher in segmental/lobar pneumonia than in bronchopneumonia. The LDH level and WBC counts were higher in children with fever lasting for >3 days before compared to those with fever lasting for ≤ 3 days. The highest quintile of PCT levels was associated with a significantly higher risk of prolonged fever and/or hospital stay than the lowest quintile. CONCLUSION: Serum PCT and CRP levels on admission day were associated with persistent fever and longer hospitalization in children with MPP.

Mutation Analysis of X-linked Sideroblastic Anemia in a 12-Month-Old Boy by Massively Parallel Sequencing

No abstract available.

Relationship of serum vitamin D and interleukin-31 levels to allergic or nonallergic rhinitis in children

PURPOSE: Serum vitamin D (25-hydroxyvitamin D, 25[OH] D) and interleukin-31 (IL-31) are related to atopic dermatitis, but their relationship with allergic rhinitis is unclear. The purpose of this study was to compare the levels of serum IL-31 and 25 (OH) D between the allergic rhinitis (AR), nonallergic rhinitis (NAR), and control groups and to investigate the relationship between IL-31 and 25 (OH) D. METHODS: We recruited 59 children with only rhinitis and 33 controls without any allergic diseases. Serum IL-31 and 25(OH) D levels were assayed using an enzyme-linked immunosorbent assay and high-performance liquid chromatography, respectively. The patients were considered to have atopic sensitization if the levels of serum specific IgE to inhalant allergens as assessed using immunoCAP were ≥0.35 IU/mL or if they tested positive for one or more allergens by the skin prick test. RESULTS: Of children with rhinitis, 25 had nonatopy (NAR), and 34 children had atopy (AR). Serum 25(OH) D levels were significantly lower in the rhinitis group than in the control group, while there was no significant difference serum 25(OH) D levels between the AR and NAR groups. Children with rhinitis demonstrated higher serum IL-31 levels than controls; however, there was no difference in serum IL-31 levels between the AR and NAR groups. Serum 25(OH) D levels were inversely correlated with serum IL-31 levels and blood eosinophil counts. On the other hand, serum 25(OH) D levels were not correlated with total serum IgE levels. CONCLUSION: Serum 25(OH) D and IL-31 may play a role in the pathogenesis of rhinitis via mechanisms other than IgE-related pathway.

Effectiveness of Polyvinyl Acetate Sheeted with Carboxymethyl Cellulose as a Packing Material after Septoplasty

BACKGROUND AND OBJECTIVES: Although polyvinyl acetate (Merocel®) has been widely used as a packing material after septoplasty, removable nasal packing can increase patient discomfort, local pain, and pressure. Furthermore, the removal of nasal packing has been described as the most uncomfortable and distressing feature associated with septoplasty. The purpose of this study was to investigate the efficacy of polyvinyl acetate with carboxymethyl cellulose sheet (Rhinocel®) nasal packing on patient subjective symptoms, degree of bleeding, hemostasis, and wound healing following septoplasty. SUBJECTS AND METHOD: Forty patients with nasal septum deviation requiring septoplasty were included. Following surgery, one nasal cavity was packed with Rhinocel® and the other one with Merocel®. Patient subjective symptoms while the packing was in situ, hemostatic properties, pain on removal, degree of bleeding on removal, duration of hemostasis after removal, postoperative wound healing, and the cost of the pack were evaluated. RESULTS: Although the two types of packing materials were equally effective in controlling postoperative bleeding after septoplasty, Rhinocel® was significantly more comfortable while in situ and less painful on removal than Merocel®, which was associated with significantly more bleeding on removal and so more time was needed to control hemorrhage. There was no significant difference in postoperative wound healing or pack cost. CONCLUSIONS: The use of Rhinocel® after septoplasty has less discomfort, greater patient satisfaction, and less bleeding on removal with no adverse reactions compared to Merocel® packing. Therefore, Rhinocel® may be a useful packing material after septoplasty.

Effect of Doctor and Device Manager on Adherence with Positive Airway Pressure Therapy in Obstructive Sleep Apnea Patients

BACKGROUND AND OBJECTIVES: Although many studies have assessed factors related to adherence of PAP therapy, there were no studies about factors related to doctors or device managers. The purpose of this study was to investigate the relationship between PAP therapy adherence and doctor or device manager. SUBJECTS AND METHODS: Between February 2013 and June 2015, 163 patients newly diagnosed with moderate to severe OSA in one of five hospitals were enrolled in this study. All patients received 4 weeks of PAP treatment with intervention consisting of mechanical support and motivation by doctor and device manager. Data from the PAP device were obtained following a minimum of an initial 30 days, with adherence defined as >4 h/night on 70% of nights. RESULTS: After 30 days, total adherence rate to PAP therapy was 35.6% (n=58). The adherence rate of device manager ① was 26.3% (31/118), and that of device manager ② was 60% (27/45), and there was statistically significant difference between the two device managers. Furthermore, there were statistically significant differences in the adherence to PAP therapy ranging from 85.6% to 0.0% according to doctor. CONCLUSION: Our study demonstrates that the device manager and doctor may be important factors for good adherence to PAP therapy in patients with OSA.

Acute Gastritis and Splenic Infarction Caused by Epstein-Barr Virus / 대한소아소화기영양학회지

Epstein-Barr virus (EBV) infection can be presented with various clinical manifestations and different levels of severity when infected. Infectious mononucleosis, which is most commonly caused by EBV infection in children and adolescents, is a clinical syndrome characterized by fatigue, malaise, fever, sore throat, and generalized lymphadenopathy. But rarely, patients with infectious mononucleosis may present with gastrointestinal symptoms and complicated by gastritis, splenic infarction, and splenic rupture. We encountered a 16-year-old girl who presented with fever, fatigue, and epigastric pain. Splenic infarction and EBV-associated gastritis were diagnosed by using esophagogastroduodenoscopy and abdominal computed tomography. Endoscopy revealed a generalized hyperemic nodular lesion in the stomach, and the biopsy findings were chronic gastritis with erosion and positive in situ hybridization for EBV. As splenic infarction and acute gastritis are rare in infectious mononucleosis and are prone to be overlooked, we must consider these complications when an infectious mononucleosis patient presents with gastrointestinal symptom.

Clinical characteristics of Mycoplasma pneumoniae pneumonia in Korean children during the recent 3 epidemics

PURPOSE: Mycoplasma pneumoniae (MP) is a major cause of community-acquired pneumonia in children. Since 2000, emerging macrolide-resistant MP has been reported. Three epidemics of MP pneumonia have occurred in Korea during the past 10 years: 2006–2007, 2011, and 2015. We investigated the differences in MP pneumonia of each epidemic in terms of clinical, laboratory, and radiologic perspectives. METHODS: We retrospectively analyzed 529 medical records of children (1–18 years of age) who were admitted and diagnosed with MP pneumonia at Kangbuk Samsung Hospital during the past 3 epidemic periods. We compared the clinical, laboratory, and radiologic characteristics of MP pneumonia among individual epidemics and between children younger and older than 6 years of age. RESULTS: The mean age of the patients was 5.7 years old, which had increased by each epidemic and showed the highest (6.3 years old) in 2015 compared to previous epidemics. Among 3 epidemics, there were no sex differences. The duration of fever after admission and hospitalization, and the percentage of lobar pneumonia and use of systemic steroids increased significantly in 2015 epidemic. Since 2006, the mean levels of erythrocyte sedimentation rate and lactate dehydrogenase had increased and in 2015 it marked the highest. Children older than 6 years showed a higher proportion of lobar pneumonia and pleural effusion as well as longer duration of fever (before and after admission) and hospitalization days than those younger than 6 years. CONCLUSION: This study suggests an increasing incidence of refractory MP pneumonia which required a more frequent use of systemic steroids over the past 10 years, and children older than 6 years were found to have more severe pneumonia than those younger than 6 years.

Changes in Etiologies and Clinical Characteristics of Operated Unilateral Sinus Diseases: Comparison Study between 2005 and 2015

BACKGROUND AND OBJECTIVES: A considerable proportion of sinus diseases is associated with a unilateral lesion. Unilateral paranasal pathological lesions require precise preoperative diagnosis and histopathological assessment. This study aimed to analyze the changes in etiologies and clinical characteristics of operated unilateral sinus diseases between 2005 and 2015. SUBJECTS AND METHODS: Two hundred eighteen operated cases with unilateral sinus disease in 2005 and 2015 were reviewed to retrospectively analyze the etiologies and clinical characteristics. RESULTS: In 2015 compared with 2005, the proportion of unilateral sinus disease and patient age were increased. The proportions of patients with allergic rhinitis and hypertension were significantly increased in 2015. The leading cause of unilateral sinus lesions was nasal polyp, followed by fungus, tumor, mucocele, anatomical variation, odontogenic sinusitis, and foreign body. This ranking did not change between 2005 and 2015. The number of patients with fungal sinusitis was significantly increased in 2015. The direction of nasal septal deviation had no statistically significant relation with unilateral sinus disease. CONCLUSION: This comparison study of unilateral sinus disease between 2005 and 2015 showed that the proportion of fungal sinusitis, patient age, and patients with underlying disease were increased.

A Case of Myosin-heavy-chain-9 (MYH9) Gene Mutation Confirmed May-Hegglin Anomaly: 11-year Follow-up / 임상소아혈액종양

May-Hegglin anomaly (MHA) is a myosin-heavy-chain-9 (MYH9)-related disorder characterized by thrombocytopenia with giant platelets and inclusion bodies in leukocytes. MHA does not require treatment, but it may be misdiagnosed as immune thrombocytopenic purpura (ITP) and inappropriately managed. Reported herein is a case of a 12 year old female patient diagnosed as MHA with laboratory findings of severe thrombocytopenia and giant platelets in peripheral blood morphology, and followed up until 23 years of age. The patient had been diagnosed with ITP and treated with intravenous gamma-globulin therapy at another hospital, and showed no improvements in platelet count. She was then referred to our hospital for further diagnostic workup and followed up for 11 years, showing platelet count of 6,000-20,000/µL and prolonged platelet function test. She was occasionally treated with iron therapy due to iron-deficiency anemia. In 2014, we conducted a DNA analysis that revealed c.4339G>T(p.Asp1447Tyr), a known mutation of MYH9 gene.

A Case of Myosin-heavy-chain-9 (MYH9) Gene Mutation Confirmed May-Hegglin Anomaly: 11-year Follow-up / 임상소아혈액종양

May-Hegglin anomaly (MHA) is a myosin-heavy-chain-9 (MYH9)-related disorder characterized by thrombocytopenia with giant platelets and inclusion bodies in leukocytes. MHA does not require treatment, but it may be misdiagnosed as immune thrombocytopenic purpura (ITP) and inappropriately managed. Reported herein is a case of a 12 year old female patient diagnosed as MHA with laboratory findings of severe thrombocytopenia and giant platelets in peripheral blood morphology, and followed up until 23 years of age. The patient had been diagnosed with ITP and treated with intravenous gamma-globulin therapy at another hospital, and showed no improvements in platelet count. She was then referred to our hospital for further diagnostic workup and followed up for 11 years, showing platelet count of 6,000-20,000/µL and prolonged platelet function test. She was occasionally treated with iron therapy due to iron-deficiency anemia. In 2014, we conducted a DNA analysis that revealed c.4339G>T(p.Asp1447Tyr), a known mutation of MYH9 gene.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

Pulmonary hemorrhage as an unusual initial manifestation of systemic lupus erythematosus

Pulmonary hemorrhage as the initial manifestation of systemic lupus erythematosus (SLE) has been rarely reported in children. We present the case of a 10-year-old girl who was admitted to Kangbuk Samsung Hospital with hemoptysis. She had a 5-day history of cough with dyspnea. On physical exam, breath sound was significantly decreased combined with rales on both lung fields. Blood tests revealed pancytopenia, decreased complement levels (C3, 21.28 mg/dL; C4, 3.10 mg/dL), positive antinuclear antibody (>1:640) and anti-double-stranded DNA antibody (262.5 IU/mL). Chest computed tomography revealed patchy ground glass opacity on both lung fields. She had proteinuria and diffuse lupus nephritis (International Society of Nephrology/Renal Pathology Society class IV-G(A)) confirmed by renal biopsy. High-dose methylprednisolone pulse therapy (30 mg/kg/day) was given for 3 days and then switched to a maintenance dose (1 mg/kg/day). Initially hemoptysis resolved after administration of methylprednisolone, but recurred on the 14th day of treatment. She was then treated with cyclophosphamide pulse therapy and hemoptysis subsided without recurrence. She was discharged on the 31st day of admission. She continued to receive monthly cyclophosphamide pulse therapy until the occurrence of leukopenia and then her regimen was switched to mycophenolate and hydroxychloroquine. SLE continues to be well controlled after 18 months of treatment. Recognition of pulmonary hemorrhage as a possible initial manifestation of SLE is crucial for early diagnosis. SLE was successfully treated with good outcome.

A Case of Kasabach-Merritt Syndrome Successfully Treated with Interferon-alpha 2b and Propranolol / 임상소아혈액종양

Kasabach-Merritt syndrome (KMS) is a rare, life-threatening disease characterized by rapidly enlarging hemangioma and consumptive coagulopathy. We report a case of KMS in a 28-day-old female neonate with a huge mixed type hemangioma on her right thigh with muscle involvement and severe venous engorgement, who was refractory to prednisone therapy, but was successfully managed with the interferon (IFN)-alpha 2b and propranolol combination therapy. By the third week of IFN-alpha 2b treatment, hematological parameters had normalized and the hemangioma size had dramatically decreased, and after 5 months of the treatment, complete resolution was observed visually. We also measured serum levels of cytokines including vascular endothelial growth factor (VEGF), basic fibroblast growth factor (bFGF), monocyte chemoattractant protein-1 (MCP-1) and platelet-derived growth factor-BB (PDGF-BB), at diagnosis and serially during treatment but the levels did not correlate with the clinical response. The patient has not shown relapse after 20 months of follow up.

Case of seropositive allergic bronchopulmonary aspergillosis in a 10-year-old girl without previously documented asthma / 소아과

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity lung disease due to bronchial colonization of Aspergillus fumigatus that occurs in susceptible patients with asthma or cystic fibrosis. A 10-year-old girl was referred to the Department of Pediatric Pulmonology for persistent consolidations on chest radiography. Pulmonary consolidations were observed in the right upper and left lower lobes and were not resolved with a 4-week prescription of broad-spectrum antibiotics. The patient had a history of atopic dermatitis and allergic rhinitis but no history of asthma. She had no fever but produced thick and greenish sputum. Her breathing sounds were clear. On laboratory testing, her total blood eosinophil count was 1,412/mm3 and total serum IgE level was 2,200 kU/L. Aspergillus was isolated in the sputum culture. The A. fumigatus-specific IgE level was 15.4 kU/L, and the Aspergillus antibody test was also positive. A chest computed tomography scan demonstrated bronchial wall thickening and consolidation without bronchiectasis. An antifungal agent was added but resulted in no improvement of pulmonary consolidations after 3 weeks. Pulmonary function test was normal. Methacholine provocation test was performed, revealing bronchial hyperreactivity (PC20=5.31 mg/mL). Although the patient had no history of asthma or bronchiectasis, ABPA-seropositivity was suspected. Oral prednisolone (1 mg/kg/day) combined with antifungal therapy was started. Pulmonary consolidations began decreasing after 1 week of treatment and completely resolved after 1 month. This is the first observed and treated case of seropositive ABPA in Korean children without previously documented asthma.