Chordoid glioma is a rare, low-grade brain neoplasm typically located in the third ventricle. Herein, we report an unusual case of histologically confirmed chordoid glioma located in the pituitary fossa and suprasellar region, not attached to the third ventricle. A 57-year-old woman presented with a 2-month history of headache and visual disturbance. Magnetic resonance imaging revealed an ovoid mass in the pituitary fossa and suprasellar region, compressing the optic chiasm without involvement of the third ventricle. The tumor showed low signal intensity on T1-weighted images and iso- to high signal intensity on T2-weighted images, with strong and homogenous contrast enhancement. Subtotal resection was performed via the transcranial approach, and the patient subsequently received adjuvant gamma knife radiosurgery. However, the residual mass showed disease progression 5 months after the initial surgery.
Brain Neoplasms ; Disease Progression ; Female ; Glioma* ; Headache ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Optic Chiasm ; Radiosurgery ; Third Ventricle

Peritoneal dialysis can be considered renal supportive therapy, even in an extremely low birth weight infant with acute kidney injury not responding to general supportive measures. Although there have been several reports of successful peritoneal dialysis in extremely low birth weight infants, general practice guidelines and commercially available optimal peritoneal dialysis catheters have not been introduced. We report a successful case of peritoneal dialysis in an extremely low birth weight infant born at 25 weeks gestational age, with birth weight 790 g, with uncontrollable metabolic acidosis, hyperkalemia, progressive azotemia and continued anuria.
Acidosis ; Acute Kidney Injury ; Anuria ; Azotemia ; Birth Weight ; Catheters ; General Practice ; Gestational Age ; Humans ; Hyperkalemia ; Infant ; Infant, Extremely Low Birth Weight ; Infant, Extremely Premature* ; Infant, Low Birth Weight ; Infant, Newborn ; Peritoneal Dialysis*

Aortic aneurysms infected by Klebsiella pneumoniae are rarely seen. We describe a 50-year-old man with infected aortic aneurysm that was successfully treated with endovascular aneurysm repair (EVAR). Diagnosis was confirmed using blood culture and computed tomography (CT). Intravenous antibiotics were immediately administered, with improvements in clinical findings and negative blood cultures before the procedure. Twenty-four months after the procedure, the patient was stable and serial CT revealed regression of the infected aortic aneurysm. Therefore, after controlling bacteremia and fever with targeted antibiotic therapy, EVAR can be considered as an alternative for patients who have serious comorbidities and are ineligible for conventional surgery.

Pneumatosis cystoides intestinalis (PCI) is a relatively rare disease which is characterized by multiple gas-filled cysts in the wall of gastrointestinal tract. It is associated with a number of clinical conditions including organ transplantation. We report a 8-year-girl of PCI without definite symptom who was taken liver transplantation after Kasai operation due to biliary atresia. On radiologic finding revealed the multiple clustered localized collection of gas in the ascending and transverse colon. She was asymptomatic and pneumatosis is discovered incidentally on abdominal X-ray at 3 months after liver transplantation. She was taken no specific treatment and discharged. The patient is currently well 6 months post- discharge with the improved abdominal radiographs.
Biliary Atresia ; Colon, Transverse ; Gastrointestinal Tract ; Humans ; Liver Transplantation* ; Liver* ; Organ Transplantation ; Pneumatosis Cystoides Intestinalis* ; Rare Diseases ; Transplants

PURPOSE: Children with asthma frequently have allergic rhinitis (AR) as a comorbidity. Asthmatic children with AR have a higher exhaled nitric oxide (eNO) level and bronchial hyperresponsiveness (BHR) than those without. The purpose of this study is to investigate the difference in lung function, eNO, and BHR between atopic asthma with and without AR, and the association of eNO and BHR with atopic intensity in total asthmatics. METHODS: We recruited 69 atopic asthmatic children with AR, 19 atopic asthmatic children without AR, 38 children with AR, and 43 nonatopic controls. We measured forced expiratory volume in one second (FEV1) and forced expiratory flow at 25% to 75% of forced vital capacity (FEF(25%-75%)), dose response slope (DRS) of bronchial challenge with methacholine and adenosine 5'-monophosphate (AMP), the levels of eNO, and the ratio of sum of allergen wheal diameter to histamine using skin prick tests. RESULTS: Atopic asthmatic children with AR had a higher eNO level compared to those without AR (P<0.05). However, there was no difference in FEV1 %predicted, FEF(25%-75%) %predicted, methacholine DRS, and AMP DRS between asthmatic children with and without AR. In total asthmatics, methacholine DRS and AMP DRS significantly correlated with eNO levels (r=0.338, P<0.001; r=0.365, P<0.001), but not with total IgE levels. However, eNO significantly correlated with total IgE levels (r=0.479, P<0.001). CONCLUSION: These results suggest that AR may enhance airway inflammation but may not lead to enhanced BHR in children with asthma.
Adenosine ; Asthma ; Bronchial Hyperreactivity ; Child* ; Comorbidity ; Forced Expiratory Volume ; Histamine ; Humans ; Immunoglobulin E ; Inflammation ; Lung ; Methacholine Chloride ; Nitric Oxide* ; Rhinitis* ; Skin ; Vital Capacity

PURPOSE: Asthma is a chronic airway inflammatory disease characterized by bronchial hyperresponsiveness and reversible airway obstruction. Bronchial challenge with methacholine or adenosine-5'-monophosphate (AMP) has been used to diagnose asthma. Recently, measurement of exhaled nitric oxide (eNO) can also be used for the diagnosis of asthma. The aim of this study was to compare the diagnostic value for asthma between challenge with methacholine or AMP and eNO in children with chronic nonspecific respiratory symptoms. METHODS: One hundred thirty-three children who have chronic nonspecific respiratory symptoms were enrolled. Bronchial challenge with methacholin and AMP were performed, and eNO was measured in all subjects. Subjects were defined as asthma based on the clinical symptoms and bronchodilator response during follow-up of at least 3 months after test. RESULTS: Thirty-three subjects (34%) were finally diagnosed as asthma among 97 patients after 3-month follow-up. The area under the receiver operating characteristic curves for the diagnosis of asthma were 0.903 (95% confidence interval [CI], 0.838-0.969; P<0.001) for methacholline challenge, 0.867 (95% CI, 0.783-0.950; P<0.001) for AMP challenge, and 0.588 (95% CI, 0.467-0.709, P=0.156) for eNO measurement. The cutoff values of these tests were methacholine PC20 (provocative concentration of methacholine causing a 20% fall in forced expiratory volume in one second) 12.0 mg/mL (sensitivity, 87.9%; specificity, 82.8%), AMP PC20 566.2 mg/mL (sensitivity, 84.8%; specificity, 85.9%), and eNO 18.5 ppb (sensitivity, 45.5%; specificity, 71.9%). CONCLUSION: Measurement of eNO may be inferior to challenge with methacholine and AMP for the diagnosis of asthma in children.
Airway Obstruction ; Asthma* ; Bronchial Provocation Tests ; Child ; Diagnosis* ; Follow-Up Studies ; Forced Expiratory Volume ; Humans ; Methacholine Chloride* ; Nitric Oxide* ; ROC Curve ; Sensitivity and Specificity

The study was carried out to evaluate and compare the immune responses in mice experimentally infected with either wild-type or isogenic mutants of Salmonella enterica serovars Enteritidis (SE), Salmonella Typhimurium (ST) and Gallinarum (SG). The mutant strains were constructed by allelic replacement of some virulence-associated genes in the wild-type strains. Seven-week-old female BALB/c mice were orally or intraperitoneally inoculated by injecting bacterial suspension. To evaluate the immune responses, enzyme-linked immunosorbent assay (ELISA) and enzyme-linked immunospot (ELISPOT) assay were conducted with serum and fecal samples. As a result, the mice group infected orally with the SE mutant strain showed the highest level of specific IgA-secreting splenocytes, compared to the other groups. The peritoneally injected groups showed the greater levels of IgG1 than the orally injected groups, which was in a good agreement with the previous studies. In addition, the mutant infected groups had the similar secretion levels of antibodies with the wild-type infected groups. These results demonstrated that the SE mutant strain elicited humoral immune response as much as wild-type, implying that it can be useful as a delivery vehicle as well as a candidate of a live attenuated vaccine.
Animals ; Antibodies ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Immunity, Humoral ; Immunity, Mucosal ; Immunoglobulin G ; Mice ; Salmonella ; Salmonella enterica ; Salmonella typhimurium ; Sprains and Strains

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common inherited kidney diseases and a frequent cause of end-stage renal failure. Generally accepted indications of nephrectomy in ADPKD cases are recurrent pyelonephritis, cyst hemorrhage, pain refractory to medical management and massively enlarged kidneys. Although pretransplant nephrectomy has been applied in these patients, evidence of the benefits of this strategy is lacking. METHODS: ADPKD patients transplanted from cadaveric or living donors were reviewed retrospectively regard to posttransplant complications and outcomes. We compared operative time, hospital day, serum creatinine at discharge and at 1year after transplantation, complication, rejection and graft loss. RESULTS: Between February 1995 and March 2008, 24 patients with end stage ADPKD received kidney transplantation at our hospital. 14 patients underwent simultaneous bilateral nephrectomy (Group I), 10 patients underwent pretransplant sequential nephrectomy (Group II). The mean operative time was 5.87+/-1.17, 9.52+/-2.67 hours in Group I and II, respectively (P=0.001). The mean hospital stay was different between Group I and II at 20.8+/-6.0, 42.8+/-17.6 days, respectively (P<0.001). Overall posttransplant complications were more frequent in the sequential nephrectomy (43% vs 80%); however, the difference was not statistically significant. Also about rejection, graft loss, there are no statistically significant differences. CONCLUSIONS: Our data imply that there is no higher morbidity or mortality when performing simultaneous bilateral nephrectomy during renal transplantation in patients with renal failure due to ADPKD. Therefore if bilateral nephrectomy is performed as an adjunct to transplantation, it is tolerable to be done at the same time as renal grafting.
Cadaver ; Creatinine ; Graft Rejection ; Hemorrhage ; Humans ; Kidney ; Kidney Diseases ; Kidney Failure, Chronic ; Kidney Transplantation ; Length of Stay ; Living Donors ; Nephrectomy ; Operative Time ; Polycystic Kidney, Autosomal Dominant ; Pyelonephritis ; Rejection (Psychology) ; Renal Insufficiency ; Retrospective Studies ; Transplants

Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the case of a 12-year-old girl with respiratory failure due to CF who underwent lung transplantation. She had been diagnosed with CF 8 years previously after being treated for recurrent Pseudomonas aeruginosa pneumonia and malnutrition based on sweat chloride concentrations and the CFTR protein gene mutation test. Progression to end-stage lung disease and respiratory failure led to registration with the Korean Network for Organ Sharing. She underwent successful double lung transplantation in 2014. Although she has diabetes mellitus and chronic kidney disease, she has a better quality of life and a prolonged life expectancy.
Child* ; Cystic Fibrosis Transmembrane Conductance Regulator ; Cystic Fibrosis* ; Diabetes Mellitus ; Female ; Humans ; Korea ; Life Expectancy ; Lung Diseases ; Lung Transplantation* ; Lung* ; Malnutrition ; Mortality ; Pneumonia ; Pseudomonas aeruginosa ; Quality of Life ; Renal Insufficiency, Chronic ; Respiratory Insufficiency ; Sweat

PURPOSE: Atopic dermatitis (AD) is a common and chronic inflammatory skin disease affecting up to 20% of children and 3% of adults worldwide. Although previous reports including genome-wide association study (GWAS) approaches have identified several risk factors that appear to be associated with AD development, replication studies are lacking. In our current study, we replicated the associations between candidate susceptibility loci and AD. METHODS: A total of 885 Korean subjects (425 AD patients and 460 unaffected controls) were genotyped for 17 single nucleotide polymorphisms (SNPs) from previous GWASs and meta-analyses of AD and from immune-related genes. RESULTS: Several SNPs showed significant associations with AD in the case-control analysis (minimum P=0.005 at rs17389644), suggesting that these polymorphisms may be related to this disease. In addition, several SNPs showed significant signals (minimum P=0.004 at rs6473227) in severe AD compared to unaffected controls. In additional linear regression analysis, a few genotypes appeared to have potential effects on the SCORing AD (SCORAD) values (minimum P=0.003 at rs13361382 on TMEM232) and immunoglobulin E (IgE) levels (minimum P < 0.0001 at rs4713555 near HLA-DRB1 and HLA-DQA1) in AD patients. CONCLUSIONS: Our replication and extended study provide additional supporting information on the genetic associations (especially, variants in TMEM232 and nearby to IL21 and HLA-DRB1/HLA-DQA1) related to AD, its clinical severity and IgE involvement.
Adult ; Case-Control Studies ; Child ; Dermatitis, Atopic* ; Genome-Wide Association Study ; Genotype ; HLA-DRB1 Chains ; Humans ; Immunoglobulin E* ; Immunoglobulins ; Linear Models ; Polymorphism, Genetic* ; Polymorphism, Single Nucleotide ; Risk Factors ; Skin Diseases