Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber disease, is a rare autosomal dominant vascular disorder involving arteriovenous malformation (AVM) due to angiodysplasia. HHT is characterized by mucocutaneous hemorrhagic telangiectasia and AVM of various different organs. The mutated genes in HHT encode proteins that mediate signaling by the transforming growth factor-beta superfamily, leading to migration and proliferation of endothelial cells to finally result in AVMs of various organs. Amongst various affected organs, pulmonary AVM is the most common. This 11-year-old female patient with a past and familial history of frequent epistaxis visited the hospital for abdominal pain and vomiting which developed on the same day. Nodular opacity was found in chest X-ray. Subsequently, AVMs in the lungs and the spleen were recognized in chest computed tomography along with detection of ENG gene mutation. Here we report a case of hereditary hemorrhagic telangiectasia diagnosed by incidental chest X-ray findings followed by genetic and radiologic studies.
Abdominal Pain ; Angiodysplasia ; Arteriovenous Malformations ; Endothelial Cells ; Epistaxis ; Female ; Humans ; Lung ; Proteins ; Spleen ; Telangiectasia, Hereditary Hemorrhagic ; Telangiectasis ; Thorax ; Vomiting

Bronchiolitis obliterans organizing pneumonia (BOOP) is a pulmonary disorder that exhibits various radiographic findings. It often shows bilateral, patched, or ground glass interstitial infiltrate, but a solitary nodular pattern rarely appears. We report a case of an 8-year-old boy suffering from BOOP that showed a single nodular pattern of the lung in the chest radiography. We conclude that when there is a solitary nodule discovered in the lungs of children or adolescence, the differential diagnosis must include BOOP.
Adolescent ; Bronchiolitis ; Bronchiolitis Obliterans ; Child ; Cryptogenic Organizing Pneumonia ; Diagnosis, Differential ; Glass ; Humans ; Lung ; Solitary Pulmonary Nodule ; Stress, Psychological ; Thorax

Congenital bronchoesophageal fistula (BEF), congenital communication between esophagus and bronchus, is a rare malformation. Benign BEF shows only nonspecific symptoms that may delay a correct diagnosis and proper treatment. This is a case of a 3-year-old boy presenting congenital BEF with imperforate anus and atrial septal defect. He had recurrent pneumonia in the right lower lobe since he was 5 months old. A barium swallow examination showed a communicating fistula between the lower esophagus and the right lower lobe bronchus. Computed tomography scan supported to delineate the course of the fistula. He underwent resection surgery of the fistula and lobectomy of the right lower lobe. He recovered without any postoperative complications.
Anus, Imperforate ; Barium ; Bronchi ; Child ; Esophagus ; Fistula ; Heart Septal Defects, Atrial ; Humans ; Pneumonia ; Postoperative Complications

A 29-month-old boy presented with fever, dyspnea, and paleness. He was initially diagnosed with pneumonia and severe sepsis. Although he was treated with intravenous antibiotics and high dose methylprednisolone, dyspnea and paleness recurred two times. Under suspicion of pulmonary hemosiderosis, we performed video-assisted thoracoscopic lung biopsy and bronchoalveolar lavage on him and found hemosiderin-laden macrophages in both specimens. Despite thorough history and laboratory examination, we could not find any pathologic or serologic evidence for primary and secondary causes of pulmonary hemosiderosis except for one that indicating Heiner's syndrome. After taking oral prednisolone he showed improvement of anemia and dyspnea, which was maintained by milk avoidance. Based on the history and the existence of immunoglobulin G antibodies against milk components, we are considering it as the case of Heiner's syndrome.
Anemia ; Anti-Bacterial Agents ; Antibodies ; Biopsy ; Bronchoalveolar Lavage ; Dyspnea ; Fever ; Hemosiderosis ; Immunoglobulin G ; Lung ; Lung Diseases ; Macrophages ; Methylprednisolone ; Milk ; Milk Hypersensitivity ; Pneumonia ; Prednisolone ; Sepsis

Mycoplasma pneumoniae is the most common pathogen of the respiratory tract among school-aged children and adolescents. It can also cause extrapulmonary manifestation that involves the skin, nervous system, digestive system, and hematopoietic system. A 12-year old girl was admitted to Asan Medical Center Children's Hospital with M. pneumoniae pneumonia. Her respiratory symptoms and signs improved after the treatment with macrolide antibiotics, and she was discharged. However, pancytopenia developed 16 days after the first admission date. She was readmitted, and treated successfully with intravenous immunoglobulin. We reported her as the first case of pancytopenia asscociated with M. pnuemoniae pneumonia in Korea.
Adolescent ; Anti-Bacterial Agents ; Child ; Digestive System ; Hematopoietic System ; Humans ; Immunoglobulins ; Korea ; Mycoplasma ; Mycoplasma pneumoniae ; Nervous System ; Pancytopenia ; Pneumonia ; Pneumonia, Mycoplasma ; Respiratory System ; Skin

PURPOSE: Tracheal bronchus is an aberrant bronchus arising from tracheal wall above the carina. Most cases of tracheal bronchus are asymptomatic, so they are diagnosed incidentally. Tracheal bronchus may be associated with other anomalies. The aim of this study is to evaluate the clinical characteristics of tracheal bronchus. METHODS: This study was conducted on 19 children who were diagnosed as tracheal bronchus by the chest computed tomography from January 2000 to December 2011. Based on the medical record, clinical features, such as symptoms, radiologic findings, combined anomalies were evaluated retrospectively. RESULTS: The age at diagnosis was ranged from 2 day to 14 years (mean, 51 months). Among the 19 children, twelve children (63.2%) were boys and seven children (36.8%) were girls. Eighteen children (94.7%) had right-side tracheal bronchus and one child (5.3%) had left-side tracheal bronchus. Displaced type were fourteen children (73.7%), supernumerary type were five children (26.3%). Thirteen children (68.4%) had no respiratory symptoms, but five children (26.3%) had persistent cough and four children (21.1%) had recurrent wheezing. Combined congenital abnormalities were present in seventeen children (89.5%), including congenital cardiovascular anomaly (n=14, 73.7%), trachea-esophageal fistula (n=3, 15.8%), Down syndrome (n=2, 10.5%). CONCLUSION: The patients with tracheal bronchus had not severe respiratory symptoms, but had many combined anomalies. So, it is necessary to consider the presence of tracheal bronchus in children with respiratory symptom, like recurrent wheezing, and to evaluate clinical significance, like combined anomaly, in tracheal bronchus patients. The limitation of this study is that the study group includes many cardiovascular disease patients (84.2%).
Bronchi ; Cardiovascular Diseases ; Child ; Congenital Abnormalities ; Cough ; Down Syndrome ; Fistula ; Humans ; Medical Records ; Respiratory Sounds ; Thorax

PURPOSE: Determination of adenosine deaminase (ADA) in pleural fluid has been suggested as another tool to establish early diagnosis of tuberculous pleural effusion. However, there are few studies concerning its usefulness in children. The objective of this study was to evaluate the utility of the determination of ADA level in pleural fluid for the differential diagnosis between tuberculous pleural effusion (TPE) and Mycoplasma pneumonia with pleural effusion (MP) in children. METHODS: We retrospectively reviewed the clinical records of 13 TPE patients and 21 MP patients with pleural effusion. Also, we analyzed ADA levels, and clinical, biochemical, microbiologic and cytologic findings in the pleural fluid. RESULTS: The pleural fluid of all the subjects revealed exudative rather than transudate characteristics. The mean ADA level in the TPE group was significantly higher than that in the MP group (106.27+/-43.71 IU/L vs. 65.28+/-26.27 IU/L, P=0.003). The area under the curve in receiver operating characteristic analysis was 0.810. With a cut-off level for ADA of 60 U/L, the sensitivity, specificity, positive predictive value, and negative predictive value were 92.3%, 61.9%, 60.0%, and 92.9%, respectively. As many as 38.9% of patients with MP were false-positive with this ADA cut-off setting. CONCLUSION: Although the measurement of ADA activity in pleural fluid can help TPE diagnosis, we should consider that some cases of MP with pleural effusion showed high ADA activities. Accordingly, the utility of the ADA level in pleural fluid for the differentiation of TPE from MP declines and additional relevant studies are required.
Adenosine ; Adenosine Deaminase ; Child ; Diagnosis, Differential ; Early Diagnosis ; Exudates and Transudates ; Humans ; Mycoplasma ; Pleural Effusion ; Pneumonia, Mycoplasma ; Retrospective Studies ; ROC Curve ; Sensitivity and Specificity

PURPOSE: Bronchiolitis obliterans (BO) is a type of chronic obstructive lung disease. In children, the most common presentation is a post infectious origin. The aim of this study was to determine the clinical features, etiology, and radiologic findings of post infectious BO, which was diagnosed by high resolution computed tomography (HRCT). The results were then compared to those of the control group. METHODS: We diagnosed 52 children with BO by a review of all performed HRCT findings at Severance Children's Hospital between January 2007 and March 2011. A retrospective study was conducted to define the clinical features, etiology, symptoms, and radiologic findings of post infectious BO. Sixty-five children who were diagnosed with lower respiratory infection confirmed by HRCT were defined as the control group for comparison with post infectious BO patients. RESULTS: The mean age of the patients at the time of diagnosis with BO was 41.3+/-5.9 months, and that of the control group was 57.1+/-5.1 months. The initial insult age of BO patients was 14.6+/-2.8 months, and that of the control group was 50.9+/-5.0 months. The frequency of previous admissions due to lower respiratory infection was relatively higher in BO patients than the control group. CONCLUSION: The results suggest that the development of post infectious BO should be early suspected and HRCT should be performed in children with a recurrent admission history of lower respiratory infection, especially in those younger than 2 years of age. Furthermore, continued management of prevent additional infection should be conducted.
Bronchiolitis ; Bronchiolitis Obliterans ; Child ; Humans ; Pulmonary Disease, Chronic Obstructive ; Retrospective Studies

PURPOSE: Serum screening test to detect specific immunoglobulin E (IgE) is an important step for the assessment of potential allergenicity of genetically modified (GM) food. The purpose of this study was to evaluate the usefulness of pooled serum for serum screening instead of individual serum. METHODS: Children with allergic disease were recruited and those who were sensitized to peanut or egg white were selected to obtain their sera. Sensitization to these foods was determined when the level of specific IgE was over 0.35 kU/L by ImmunoCAP. The patients were divided into subgroups according to their level of specific IgE. Raw proteins were extracted and immunoblot analysis was performed to compare the immunoreactivity between individual serum and pooled serum. RESULTS: Pooled serum from peanut-sensitized allergic children showed all the bands which were shown in immunoblot analysis by using individual serum and peanut protein extract. These findings were demonstrated both in pooled serum with low level of peanut-specific IgE and in those with high level of peanut-specific IgE. Likewise, there was no difference in the immunoreactivity between individual serum and pooled serum from egg white-sensitized allergic children. CONCLUSION: Pooled serum can be used as an alternative to individual serum for the serum screening in the allergenicity assessment of GM food.
Child ; Egg White ; Food, Genetically Modified ; Humans ; Immunoglobulin E ; Immunoglobulins ; Mass Screening ; Ovum ; Proteins

PURPOSE: Influenza virus is one of the most important viruses that cause the respiratory infection seasonally. In April 2009, H1N1 was detected in America and Mexico and then there was pandemic in Korea. We investigated the difference of clinical and laboratory findings between the infections of H1N1 and Influenza B. METHODS: We have retrospectively studied the patients under age of 15 years who visited Inje University Sanggye Paik Hospital from August 2009 to April 2010. Evaluation for influenza infection was performed by rapid antigen test or multiplex reverse transcriptase polymerase chain reaction. Complete blood count with differential counts, C-reactive protein and chest X-ray were checked. RESULTS: Enrolled patients were 2,226 in H1N1-infected group and 288 in influenza B-infected group. Seasonal variation was that H1N1 in autumn and winter but influenza B in spring. The male-to-female sex ratio was same as 1.23 in each group. The mean age of H1N1-infected group was higher than influenza B-infected group (P<0.001). Fever was developed similarly in both groups (P=0.114). However, cough, sputum, rhinorrhea, vomiting, diarrhea, and headache were more prevalent in influenza B infection compared to H1N1 infection (P<0.001). Pneumonia development and admission rate were higher in influenza B infection compared to H1N1 infection (P<0.001, respectively). CONCLUSION: Although H1N1 infection spread rapidly, H1N1 caused not so severe symptoms than influenza B. Because of the possibility that influenza epidemic will develop repeatedly in the future, we need to evaluate more about different characteristics depending on the virus subtype and prepare for them.
Americas ; Blood Cell Count ; C-Reactive Protein ; Cough ; Diarrhea ; Fever ; Headache ; Humans ; Influenza, Human ; Korea ; Mexico ; Orthomyxoviridae ; Pandemics ; Pneumonia ; Retrospective Studies ; Reverse Transcriptase Polymerase Chain Reaction ; Seasons ; Sex Ratio ; Sputum ; Thorax ; Viruses ; Vomiting