Infectious diseases precede a significant proportion of acute ischemic strokes in children. Here, we report a case of acute ischemic stroke in a 3-year-old girl with a Mycoplasma pneumonia-associated respiratory tract infection. She developed an acquired prothrombotic state of protein S deficiency and had increased fibrinogen and fibrinogen degradation product levels and increased titer of antinuclear antibodies. However, these conditions were completely alleviated at the 1-month follow-up examination. Infection with M. pneumoniae may cause a transient prothrombotic state that can potentially cause a thrombus.
Antibodies, Antinuclear ; Child ; Communicable Diseases ; Fibrinogen ; Follow-Up Studies ; Humans ; Mycoplasma ; Mycoplasma pneumoniae ; Pneumonia ; Pneumonia, Mycoplasma ; Preschool Child ; Protein S Deficiency ; Respiratory Tract Infections ; Stroke ; Thrombosis

Klebsiella pneumoniae has emerged as a leading pathogen that causes pyogenic liver abscesses (PLAs) in Korea. K. pneumoniae liver abscess (KLA) is potentially life threatening, and the diagnosis is difficult. In developed countries, PLA is rarely observed in children and is frequently associated with disorders of granulocyte function and previous abdominal infection. We observed a case of KLA in a healthy 12-year-old boy. To our knowledge, this is the first reported case of KLA in an immunocompetent child without an underlying disease in Korea. The patient was treated with percutaneous catheter drainage and antibiotics. The catheter was placed in the intrahepatic abscess for 3 weeks and parenteral antibiotics (ceftriaxone and amikacin) were administered for 4 weeks, followed by oral antibiotics (cefixime) for 2 weeks. We reported this case to raise awareness of KLA in immunocompetent children among physicians, and to review the diagnosis, risk factors, potential complications, and appropriate treatment of KLA.
Abscess ; Anti-Bacterial Agents ; Catheters ; Child ; Developed Countries ; Drainage ; Granulocytes ; Humans ; Klebsiella ; Klebsiella pneumoniae ; Korea ; Liver ; Liver Abscess ; Liver Abscess, Pyogenic ; Pneumonia ; Risk Factors

PURPOSE: We performed a pilot study to determine the benefit of high-dose chemotherapy and autologous peripheral blood stem cell transplantation (HDCT/autoPBSCT) for patients with Ewing sarcoma family of tumors. METHODS: We retrospectively analyzed the data of patients who received HDCT/autoPBSCT at Korea Cancer Center Hospital. Patients with relapsed, metastatic, or centrally located tumors were eligible for the study. RESULTS: A total of 9 patients (3 male, 6 female), with a median age at HDCT/autoPBSCT of 13.4 years (range, 7.1 to 28.2 years), were included in this study. Patients underwent conventional chemotherapy and local control either by surgery or radiation therapy, and had achieved complete response (CR, n=7), partial response (n=1), or stable disease (n=1) prior to HDCT/autoPBSCT. There was no transplant-related mortality. However, the median duration of overall survival and event-free survival after HDCT/autoPBSCT were 13.3 months (range, 5.3 to 44.5 months) and 6.2 months (range, 2.1 to 44.5 months), respectively. At present, 4 patients are alive and 5 patients who experienced adverse events (2 metastasis, 2 local recur, and 1 progressive disease) survived for a median time of 2.8 months (range, 0.1 to 10.7 months). The 2-year survival after HDCT/autoPBSCT was 44.4%+/-16.6% and disease status at the time of HDCT/autoPBSCT tended to influence survival (57.1%+/-18.7% of cases with CR vs. 0% of cases with non-CR, P=0.07). CONCLUSION: Disease status at HDCT/autoPBSCT tended to influence survival. Further studies are necessary to define the role of HDCT/autoPBSCT and to identify subgroup of patients who might benefit from this investigational treatment.
Adolescent ; Child ; Disease-Free Survival ; Humans ; Korea ; Male ; Neoplasm Metastasis ; Peripheral Blood Stem Cell Transplantation ; Pilot Projects ; Retrospective Studies ; Sarcoma, Ewing ; Stem Cell Transplantation ; Therapies, Investigational

PURPOSE: The purpose of this study was to share our experience of transcatheter closure of small patent ductus arteriosus (PDA) by using an Amplatzer vascular plug (AVP). METHODS: We reviewed the medical records of 20 patients who underwent transcatheter closure at Samsung Medical Center and Sejong General Hospital from January 2008 to August 2012. The size and shape of the PDAs were evaluated by performing angiograms, and the PDA size and the AVP devices size were compared. RESULTS: The mean age of the patients was 54.9+/-45.7 months old. The PDAs were of type C (n=5), type D (n=12), and type E (n=3). The mean pulmonary end diameter of the PDA was 1.7+/-0.6 mm, and the aortic end diameter was 3.6+/-1.4 mm. The mean length was 7.3+/-1.8 mm. We used 3 types of AVP devices: AVP I (n=5), AVP II (n=7), and AVP IV (n=8). The ratio of AVP size to the pulmonary end diameter was 3.37+/-1.64, and AVP size/aortic end ratio was 1.72+/-0.97. The aortic end diameter was significantly larger in those cases repaired with AVP II than in the others (P=0.002). The AVP size did not significantly correlate with the PDA size, but did correlate with smaller ratio of AVP size to aortic end diameter (1.10+/-0.31, P=0.032). CONCLUSION: Transcatheter closure of small PDA with AVP devices yielded satisfactory outcome. AVP II was equally effective with smaller size of device, compared to others.
Cardiac Catheterization ; Ductus Arteriosus ; Ductus Arteriosus, Patent ; Hospitals, General ; Humans ; Medical Records ; Vascular Access Devices

PURPOSE: This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. METHODS: This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. RESULTS: The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. CONCLUSION: The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions.
Child ; Coronary Vessels ; Humans ; Immunoglobulins ; Incidence ; Length of Stay ; Medical Records ; Mucocutaneous Lymph Node Syndrome ; Retreatment ; Retrospective Studies

No abstract available.

Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as Aspergillus species, Staphylococcus aureus, Serratia marcescens, Nocardia species and Burkholderia cepacia. In case of a history of recurrent or persistent infections, immune deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for Aspergillus antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN-gamma initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.
Amphotericin B ; Aspergillosis ; Aspergillus ; Bacteria ; Bronchoalveolar Lavage Fluid ; Burkholderia cepacia ; Catalase ; Follow-Up Studies ; Fungi ; Granulomatous Disease, Chronic ; Homicide ; Humans ; Infant ; Invasive Pulmonary Aspergillosis ; Lung ; NADPH Oxidase ; Nocardia ; Phagocytes ; Pneumonia ; Pulmonary Aspergillosis ; Pyrimidines ; Serologic Tests ; Serratia marcescens ; Staphylococcus aureus ; Triazoles

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the CREB binding protein (CREBBP) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.
Arnold-Chiari Malformation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; CREB-Binding Protein ; Heart Defects, Congenital ; Humans ; Intellectual Disability ; Keloid ; Rubinstein-Taybi Syndrome ; Thumb ; Toes

PURPOSE: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. METHODS: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. RESULTS: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. CONCLUSION: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.
Aged ; Bronchiolitis Obliterans ; Child ; Child, Orphaned ; Disease Management ; Humans ; Hypersensitivity ; Incidence ; Korea ; Lung ; Lung Diseases ; Pediatrics ; Prevalence ; Publications

PURPOSE: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. METHODS: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. RESULTS: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37(+2) weeks and 2.5+/-0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. CONCLUSION: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.
Anal Canal ; Birth Weight ; Cause of Death ; Dilatation ; Duodenostomy ; Esophageal Atresia ; Esophageal Stenosis ; Esophagus ; Extremities ; Gastroesophageal Reflux ; Gastrostomy ; Gestational Age ; Heart Defects, Congenital ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Kidney ; Limb Deformities, Congenital ; Prenatal Diagnosis ; Retrospective Studies ; Sepsis ; Spine ; Trachea ; Tracheoesophageal Fistula