Coronavirus disease 2019 (COVID-19) in patients with underlying diseases, is associated with high infection and mortality rates, which may result in acute respiratory distress syndrome and death. Mucopolysaccharidosis (MPS) type II is a progressive metabolic disorder that stems from cellular accumulation of the glycosaminoglycans, heparan, and dermatan sulfate. Upper and lower airway obstruction and restrictive pulmonary diseases are common complaints of patients with MPS, and respiratory infections of bacterial or viral origin could result in fatal outcomes. We report a case of COVID-19 in a 16-year-old adolescent with MPS type II, who had been treated with idursulfase since 5 years of age. Prior to infection, the patient’s clinical history included developmental delays, abdominal distension, snoring, and facial dysmorphism. His primary complaints at the time of admission included rhinorrhea, cough, and sputum without fever or increased oxygen demand. His heart rate, respiratory rate, and oxygen saturation were within the normal biological reference intervals, and chest radiography revealed no signs of pneumonia. Consequently, supportive therapy and quarantine were recommended. The patient experienced an uneventful course of COVID-19 despite underlying MPS type II, which may be the result of an unfavorable host cell environment and changes in expression patterns of proteins involved in interactions with viral proteins. Moreover, elevated serum heparan sulfate in patients with MPS may compete with cell surface heparan sulfate, which is essential for successful interaction between the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spike protein and the host cell surface, thereby protecting against intracellular penetration by SARS-CoV-2.

PURPOSE: This study was to develop evidence-based clinical practice guideline in order to prevent contrast-induced nephropathy (CIN) for patients undergoing percutaneous coronary intervention (PCI). METHODS: The guideline was developed based on the “Scottish Intercollegiate Guidelines Network (SIGN)”. The first draft of guideline was developed through 5 stages and evaluated by 10 experts.(1) Clinical questions were ensured in PICO format.(2) Two researchers conducted a systematic search through electronic database, identifying 170 studies. We selected 27 full text articles including 16 randomized clinical trials, 7 systematic reviews, and 4 guidelines. Quality of each studies were evaluated by the Cochran's Risk of Bias, AMSTAR, K-AGREEII. Among the studies, 11 studies were excluded.(3) The strength of recommendations were classified and quality of recommendations were ranked.(4) Guideline draft was finalized.(5) Content-validation was conducted by an expert group. All contents were ranked above 0.8 in CVI. RESULTS: Evidence-based clinical practice guideline to prevent CIN was dveloped.(1) The guideline for preventing CIN recommends using 0.9% saline.(2) Standardized rate of fluid therapy is 1 to 1.5ml/kg/hr.(3) Execute hydration for 6~12hrs before PCI and after PCI. CONCLUSION: This study suggests evidence-based clinical practice guideline for preventing CIN which can be more efficiently used in clinical practice.
Acute Kidney Injury ; Bias (Epidemiology) ; Contrast Media ; Evidence-Based Practice ; Fluid Therapy ; Humans ; Percutaneous Coronary Intervention

PURPOSE: The aim of this study is to investigate the changing prevalence rate of Helicobacter pylori infection in children, of different age groups, with recurrent abdominal pain over a 10-year period. METHODS: Children with recurrent abdominal pain who visited the pediatric outpatient clinic at university hospital were screened for H. pylori. Children were divided into 3 age categories of 4-5, 6-11, and 12-16 years. To study the changes in the annual prevalence rates of H. pylori infection, the study period was divided into 3 time periods: 2004-2007, 2008-2010, and 2011-2014. Urea breath test was performed for all children aged 4-16 years, with a cut-off value of 4.0per thousand for children aged > or =6 years and 7per thousand for children aged <6 years. RESULTS: A total of 2,530 children (1,191 boys) with a mean age of 10.0+/-3.0 years (range, 4.0-16.9 years) were included in the study. The total prevalence of H. pylori infection was 7.4% (187/2,530). The prevalence rate of H. pylori infection in children with recurrent abdominal pain was 8.0% (70/873) in 2004-2007, 7.7% (51/666) in 2008-2010, and 6.7% (66/991) in the 2011-2014. Nevertheless, a significant difference was observed in the prevalence rate between children <12 years old and > or =12 years of age (p=0.018). CONCLUSION: The prevalence of H. pylori infection in Korean children with recurrent abdominal pain was 7.4%, showing no significant decrease in the last 11 years; however, the prevalence rate in children <12 years old was significantly lower than that in those > or =12 years old.
Abdominal Pain* ; Ambulatory Care Facilities ; Breath Tests ; Child* ; Endoscopy ; Gastrointestinal Diseases ; Helicobacter ; Helicobacter pylori* ; Humans ; Prevalence* ; Urea

We present a case of a 13-year-old boy with Gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (GI) tract, which was complicated by recurrent chylothorax and GI bleeding. The presenting symptoms were intermittent abdominal pain, back pain, and melena. Esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a D2-40 immunohistochemical marker. In cases of GI bleeding with osteolysis, the expression of a D2-40 marker in the lymphatic endothelium of the GI tract may help to diagnose GI involvement in Gorham's disease. To the best of our knowledge, this is the first case report to pathologically demonstrate intestinal lymphatic malformation as a cause of GI bleeding in Gorham's disease.
Abdominal Pain ; Adolescent ; Back Pain ; Biopsy ; Child ; Chylothorax ; Colonoscopy ; Duodenum* ; Endoscopy, Digestive System ; Endothelium, Lymphatic ; Femur ; Gastrointestinal Tract* ; Hemorrhage ; Humans ; Male ; Melena ; Mucous Membrane ; Occult Blood ; Osteolysis ; Osteolysis, Essential ; Spine

PURPOSE: The spontaneous seroconversion rate of hepatitis B e antigen (HBeAg)-positive chronic hepatitis B (CHB) virus infection in children is lower than that in adults. However, few studies have investigated the rate of transition from the immune-tolerant to the early immune-clearance phase in children. METHODS: From February 2000 to August 2011, we enrolled 133 children aged <18 years who had visited the Department of Pediatrics, Kyungpook National University Hospital. All subjects were in the immune-tolerant phase of HBeAg-positive CHB virus infection. The estimated transition rate into the early immune-clearance phase was calculated using the Kaplan-Meier method. RESULTS: Among the 133 enrolled pediatric CHB virus infection patients in the HBeAg-positive immune-tolerant phase, only 21 children (15.8%) had converted to the early immune-clearance phase. The average age at entry into active hepatitis was 10.6+/-4.8 years. The incidence of transition from the immune-tolerant to the early immune-clearance phase in these children was 1.7 episodes/100 patient-years. When analyzed by age, the estimated transition rate was 4.6%, 7.1%, and 28.0% for patients aged <6, 6-12, >12 years, respectively. CONCLUSION: In children with CHB virus infection, the estimated rate of entry into the early immune-clearance phase was 28.0% for patients aged 12-18 years, which was significantly higher than that observed for children aged <12 years (11.7%; p=0.001).
Adult ; Child* ; Gyeongsangbuk-do ; Hepatitis ; Hepatitis B ; Hepatitis B virus ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Incidence ; Natural History ; Pediatrics ; Phase Transition

PURPOSE: Soluble ST2 (sST2) has been reported to regulate Th2 response. In this study, serum levels of sST2 and other cytokines were measured in recurrent early wheezers and asthmatic children. We aimed to investigate if there are any differences or similarities in Th1 or Th2 response between those two patient groups. METHODS: Fifty-nine patients admitted with exacerbation of wheezing or asthma were enrolled. Two patient groups were defined: children with atopic asthma (> or =6 years, n=21) and recurrent early wheezers (< or =2 years, n=38). Recurrent early wheezers were divided based on their atopic status: 19 were atopic and 19 were nonatopic. sST2, interleukin (IL) 33, IL-5, and interferon (IFN)-gamma were measured in serum samples collected on admission. Cytokine levels in both patient groups were compared with their age-matched controls and evaluated the relationship with blood eosinophils, serum IgE levels, and also with the severity of symptom. RESULTS: sST2 and IL-5 were significantly increased both in asthmatic children (P=0.02, P=0.004) and recurrent early wheezers (P=0.01, P=0.001) compared to their age-matched controls. IL-5 was significantly higher in atopic wheezers compared with non-atopic wheezers (P=0.04). Severity score showed a positive correlation with sST2 and IFN-gamma in asthmatic children, but only with IFN-gamma in early wheezers. There was an inverse correlation between sST2 and blood eosinophil counts both in asthmatic children and atopic recurrent wheezers. CONCLUSION: Our study suggests that sST2 might regulate allergic inflammation by suppressing eosinophilia and play an important role in pathophysiology of acute exacerbation of wheezing or asthma both in asthmatic children and early wheezers.
Asthma ; Child* ; Cytokines ; Eosinophilia ; Eosinophils ; Humans ; Immunoglobulin E ; Inflammation ; Interferons ; Interleukin-5 ; Interleukins ; Respiratory Sounds

PURPOSE: Collectin family is an important component of innate immunity, of which surfactant protein (SP)-D and mannose-binding lectin (MBL) are the most characterized. We examined SP-D and MBL in young children with acute respiratory syncytial virus (RSV) bronchiolitis. METHODS: Sixty-three children (< or =24 months of age) admitted with the first RSV bronchiolitis during 2 epidemics and followed for 1 year after discharge were enrolled. The patients were defined as severe group when they had 2 of followings during admission: hypoxemia (<92% oxygen saturation), rapid breathing (and/or lower chest wall indrawing), and >7 days of hospital stay. All children were evaluated if they had recurrent wheezing during follow-up. SP-D and MBL were measured using enzyme-linked immunosorbent assay in serum collected on admission and compared with controls. Their levels were evaluated in relation to the symptom severity during admission and recurrence of wheezing after discharge. RESULTS: Serum SP-D increased significantly in the patients (P<0.01), but MBL showed no difference compared to the controls. SP-D levels were significantly higher in severe group compared with nonsevere group (P<0.05). SP-D levels in the patients with recurrent wheezing after discharge were significantly higher than in those without (P<0.05). MBL showed no difference in relation to the symptom severity or recurrence of wheezing. CONCLUSION: Our study showed that serum SP-D was associated with the severity of RSV bronchiolitis and suggests that it might be a biomarker of lung injury and recurrence of wheezing illnesses in the young children admitted with their first RSV bronchiolitis.
Anoxia ; Bronchiolitis ; Child ; Collectins ; Enzyme-Linked Immunosorbent Assay ; Follow-Up Studies ; Humans ; Immunity, Innate ; Infant ; Length of Stay ; Lung Injury ; Mannose-Binding Lectin ; Oxygen ; Pulmonary Surfactant-Associated Protein D ; Recurrence ; Respiration ; Respiratory Sounds ; Respiratory Syncytial Viruses ; Thoracic Wall

PURPOSE: Interleukin (IL)-33, a member of the IL-1 cytokine family, is considered to be important for innate-type mucosal immunity of the lung and also has been suggested to induce Th2-type immune responses. We aimed to investigate if IL-33 is involved in airway inflammation due to respiratory syncytial virus (RSV) infection in young children. METHODS: Thirty-eight infants (< or =24 months of age) admitted with their first episode of RSV bronchiolitis were enrolled in the study. Atopy was defined by having at least 1 allergen-specific immunoglobulin E (IgE), positive result to skin prick test, or high serum IgE levels. The patients were assessed to have severe symptoms when they had > or =2 of the following clinical findings: hypoxemia (<92% oxygen saturation), rapid breathing (and/or lower chest wall indrawing), and >7 days of hospital stay. The levels of IL-33 and the IL-33 receptor (sST2) were measured using enzyme-linked immunosorbent assay in nasal secretion samples collected from the patients on admission and compared with 20 age-matched controls. We also investigated the levels of IL-33 and sST2 in relation to the atopic status and symptom severity of the patients. RESULTS: Nasal IL-33 levels in the patients with acute RSV bronchiolitis were significantly increased (P<0.05), but sST2 showed no difference compared to the controls. Neither IL-33 nor sST2 showed significant difference in relation to the atopic status or severity of symptoms. CONCLUSION: Our study showed significantly increased IL-33 in the nasal secretions of the young infants admitted with acute RSV bronchiolitis and suggests that IL-33 is involved in the pathogenesis of RSV-induced airway inflammation.
Anoxia ; Bronchiolitis ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunity, Mucosal ; Immunoglobulin E ; Immunoglobulins ; Infant ; Inflammation ; Interleukin-1 ; Interleukins ; Length of Stay ; Lung ; Oxygen ; Respiration ; Respiratory Syncytial Viruses ; Skin ; Thoracic Wall

PURPOSE: In the present study, we investigated the clinical characteristics of tuberculosis in school-age children and adolescents, which is important as a reservoir for future disease burden. METHODS: Ninety patients, aged from 6 to 18 years, who were diagnosed and treated with tuberculosis during the period from January 2005 to July 2011, were enrolled. We retrospectively analyzed the medical records and investigated clinical characteristics of the patients. RESULTS: Eight patients were 6 to 12 (9%) and 82 were over 13 years of age (91%). There was a significantly higher percentage of males than females in the latter age group (P<0.01). Route of infection was not confirmed in 74 patients, and 16 patients were diagnosed through the school or military medical examinations with no clinical symptoms. Seventy patients (78%) were presented with pulmonary tuberculosis. Chronic persistent coughing was the most common presenting symptom, and both upper lobes were most frequently involved. Nineteen patients over 13 years of age had adult-type cavitary tuberculosis. The positive results for acid-fast smears or cultures were not high, and the rate of positive tuberculin skin test was 88%. The most frequent adverse effects of anti-tuberculosis treatment were hepatotoxicity, hyperuricemia, and gastrointestinal disorders. The duration of the treatment was much prolonged in 8 patients who had drug-resistant tuberculosis. CONCLUSION: Our study showed that pulmonary tuberculosis should be suspected in the adolescents who have prolonged respiratory symptoms. It also indicates that pulmonary tuberculosis in adolescents has similar characteristics to tuberculosis in adults, which suggests the potential important role of adolescent tuberculosis in community disease transmission.
Adolescent ; Adult ; Aged ; Child ; Cough ; Female ; Humans ; Hyperuricemia ; Male ; Medical Records ; Military Personnel ; Retrospective Studies ; Skin Tests ; Tuberculin ; Tuberculosis ; Tuberculosis, Pulmonary

PURPOSE: This study evaluated the efficiency and safety of the Foley catheter for esophageal removal of coins in children, compared to standard endoscopic extraction with respect to success rate, sedation, promptness and cost. METHODS: Twenty four children with coin lodgement in esophagus were managed with either a Foley catheter (n=14) or endoscopic extraction (n=10) from January 2007 through August 2010 at Kyungpook National University Hospital. A retrospective review of medical records and radiological findings was performed. RESULTS: Of the 14 patients who underwent Foley catheter extraction, successful and complication-free removal was achieved in 10 cases (71.4%). Of the 10 patients who underwent endoscopic extraction, all cases were successful (p=0.114). Sedation rate in the Foley catheter and endoscopic extraction group was 6/14 and 10/10 (p=0.006). The average wait time before the procedure and average hospital charge (US$) were 2.0+/-1.1 hours and 18.1+/-13.7 hours, and $113 and $428 for Foley catheter extraction and endoscopic extraction, respectively. CONCLUSION: Foley catheter extraction may be tried for the removal of esophageal coins in uncomplicated children. The technique is effective, safe, inexpensive and free of general anesthesia.
Anesthesia, General ; Catheters ; Child ; Endoscopy ; Esophagus ; Hospital Charges ; Humans ; Medical Records ; Numismatics ; Retrospective Studies