Wegener's granulomatosis is characterized by necrotizing granulomatosis lesion of the respiratory tract, glomerulonephritis and frequently vasculitis involving other organs. The basic pathophysiologic mechanism of Wegener's granulomatosis is not defined yet. However, it may be suspected an autoimmune disease. We experienced a case of Wegener's granulomatosis which are associated with acute renal failure and pneumothorax. The patient suffered from hemoptysis, fever and cough. Despite antibiotic therapy, symtoms did not improved and multiple varying sized nodules were aggravated on chest roentogenogram and serum creatinine elevated 3.4mg/dl. After diagnosis using video associated thoracoscopic surgery, the patient was treated with cyclophsphamide, glucocorticoid and sulfamethoxasole-trimethoprime. With the combination therapy, the patient felt completely well and chest roentogenogram showed lungs were improved and serum creatinine was normal. The patient was readmitted because of right pleuritic pain and dyspnea 15 day after discharge. The patient was developed a right pneumothorax. The lung was easily expanded by intercostal tube drainage with a one way valve. The patient has been treated as an out- patient with immunosuppressive agents continously.
Acute Kidney Injury* ; Autoimmune Diseases ; Cough ; Creatinine ; Diagnosis ; Drainage ; Dyspnea ; Fever ; Glomerulonephritis ; Hemoptysis ; Humans ; Immunosuppressive Agents ; Lung ; Pneumothorax* ; Respiratory System ; Thoracoscopy ; Thorax ; Vasculitis ; Wegener Granulomatosis*

PURPOSE: IgA nephropathy(IgAN) and thin glomerular basement membrane disease(TGBMD) are common glomerular diseases that cause hematuria in childhood. IgAN has characteristics of IgA deposit as the sole or predominantly localized to the mesangium. Recently, it has been reported that thinning of glomerular basement membrane(GBM) is commonly accompanied with precipitation of electron dense deposits in IgAN. We performed this study to examine the frequency of thinning of GBM among children with IgAN and to analysis the correlation between urinary abnormalities and GBM thickness, and furthermore to conduct comparative analysis of the clinical and pathological features of IgAN and TGBMD. METHODS: This study summarizes data collected from Department of Pediatrics, Busan Paik Hospital, Inje Medical College. Data include 51 cases who were diagnosed as IgAN from 1995 to 2000, and 26 cases who were diagnosed as TGBMD from 1990 to 2000 by percutaneous renal biopsy. RESULTS: Males accounted for 29/51(56.9%) patients with IgAN and 8/26(30.8%) of those with TGBMD. The clinical and laboratory features between IgAN and TGBMD were significantly different regarding the incidence of proteinuria(IgAN vs TGBMD: 43.1% vs 3.8%, P=0.001), the incidence of co-appearance of proteinuria with hematuria (41.2% vs 3.8%, P=0.001), total amount of protein in 24 hours collected urine (808+/-188.5 mg vs 251+/-00.7 mg, P=0.001) and the incidence of proteinuria more than 1 gm in 24 hours collected urine (23.5% vs 3.8%, P=0.01). On the contrary, there were no significant differences in the levels of serum albumin, creatinine, BUN, and Ccr between two groups. The mean thickness of GBM in patients with IgAN was293.0+/-9.2 nm(139.7-461.9 nm) and 180.9+/-5.8 nm (110.5-229.5 nm) in patients with TGBMD. The mean GBM thickness revealed significantly thinner in TGBMD compared than those with IgAN (P=0.0001). The frequency of thickness being less than 250 nm was 37.4 +/-34.4% in IgAN and 93.0 +/-7.0% in TGBMD (P=0.0001). But there were no correlations between urinary abnormalities and GBM thickness in patients with IgAN. CONCLUSION: The thinning of GBM would be one of the common pathological findings in IgAN. Moreover, there is no significant correlations between urinary abnormalities and GBM thickness in patients with IgAN. However, patients with IgAN tend to have significantly higher possibilities of proteinuria, co-appearance of proteinuria with hematuria and higher total amount of protein in 24 hours collected urine compared those with TGBMD. These differences might be play an important role as progressive prognostic indicators in patients with IgAN.
Biopsy ; Busan ; Child ; Creatinine ; Glomerular Basement Membrane* ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Incidence ; Male ; Pediatrics ; Proteinuria ; Serum Albumin

We previously reported that mice lacking JSAP1 (jsap1-/-) were lethal and the brain of jsap1-/- at E18.5 exhibited multiple types of developmental defects, which included impaired axon projection of the corpus callosum and anterior commissures. In the current study, we examined whether the early telencephalic commissures were formed abnormally from the beginning of initial development or whether they arose normally, but have been progressively lost their maintenance in the absence of JSAP1. The early corpus callosum in the brain of jsap1+/+ at E15.5-E16.5 was found to cross the midline with forming a distinct U-shaped tract, whereas the early axonal tract in jsap1-/- appeared to cross the midline in a diffuse manner, but the lately arriving axons did not cross the midline. In the brain of jsap1-/- at E17.5, the axon terminals of lately arriving collaterals remained within each hemisphere, forming an early Probst's bundle-like shape. The early anterior commissure in the brain of jsap1+/+ at E14.5-E15.5 crossed the midline, whereas the anterior commissure in jsap1-/- developed, but was deviated from their normal path before approaching the midline. The axon tracts of the corpus callosum and anterior commissure in the brain of jsap1-/- at E16.5-E17.5 expressed phosphorylated forms of FAK and JNK, however, their expression levels in the axonal tracts were reduced compared to the respective controls in jsap1+/+. Considering the known scaffolding function of JSAP1 for the FAK and JNK pathways, these results suggest that JSAP1 is required for the pathfinding of the developing telencephalic commissures in the early brains.
Adaptor Proteins, Signal Transducing/genetics/*metabolism ; Animals ; Brain/*embryology/*metabolism ; Female ; Focal Adhesion Kinase 1/genetics/metabolism ; Immunohistochemistry ; In Situ Nick-End Labeling ; JNK Mitogen-Activated Protein Kinases/genetics/metabolism ; Mice ; Mice, Knockout ; Nerve Tissue Proteins/genetics/*metabolism ; Pregnancy ; Telencephalon/*embryology/*metabolism

PURPOSE: Each ethnic group has a unique life style, including diets. Life style affects bowel movement. The aim of this study is to describe the results of colon transit time (CTT) tests in Korean children who had chronic functional constipation based on highly refined data. METHODS: One hundred ninety (86 males) out of 415 children who performed a CTT test under the diagnosis of chronic constipation according to Rome III criteria at Konkuk University Medical Center from January 2006 through March 2015 were enrolled in this study. Two hundreds twenty-five children were excluded on the basis of CTT test result, defecation diary, and clinical setting. Shapiro-Wilk and Mann-Whitney U, and chi-square tests were used for statistical analysis. RESULTS: The median value and interquartile range (IQR) of CTT was 54 (37.5) hours in Encopresis group, and those in non-encopresis group was 40.2 (27.9) hours (p<0.001). The frequency of subtype between non-encopresis group and encopresis was statistically significant (p=0.002). The non-encopresis group (n=154, 81.1%) was divided into normal transit subgroup (n=84, 54.5%; median value and IQR of CTT=26.4 [9.6] hours), outlet obstruction subgroup (n=18, 11.7%; 62.4 [15.6] hours), and slow transit subgroup (n=52, 33.8%; 54.6 [21.0] hours]. The encopresis group (n=36, 18.9%) was divided into normal transit subgroup (n=8, 22.2%; median value and IQR of CTT=32.4 [9.9] hours), outlet obstruction subgroup (n=8, 22.2%; 67.8 [34.8] hours), and slow transit subgroup (n=20, 55.6%; 59.4 [62.7]hours). CONCLUSION: This study provided the basic pattern and value of the CTT test in Korean children with chronic constipation.
Academic Medical Centers ; Child* ; Colon* ; Constipation* ; Defecation ; Diagnosis ; Diet ; Encopresis ; Ethnic Groups ; Humans ; Korea ; Life Style

A fecaloma refers to a mass of accumulated feces that is much harder than a mass associated with fecal impaction. Fecalomas are usually found in the rectosigmoid area. A 10-year-old male with chronic constipation was admitted because of increasing abdominal pain. An abdominal computed tomography scan and a simple abdominal x-ray revealed rapidly evolving mechanical obstruction in the small intestine. Most of the fecalomas are successfully treated by conservative methods such as laxatives, enemas and rectal evacuation. When conservative treatments have failed, surgical intervention may be needed. In this case, an emergency operation was performed and a 4x3x2.5 cm fecaloma was found in the distal ileum. We thus report a case of ileal fecaloma inducing small bowel obstruction in a patient with chronic constipation, who required surgical intervention. When symptoms of acute small intestinal obstruction develop in a patient with chronic constipation, a fecaloma should be considered in differential diagnosis.
Abdominal Pain ; Child ; Constipation ; Diagnosis, Differential ; Emergencies ; Enema ; Fecal Impaction ; Feces ; Humans ; Ileum ; Intestinal Obstruction ; Intestine, Small ; Laxatives ; Male

Objectives: The aim of this study was to investigate dietary intakes and nutritional status among Uganda primary school children from two selected schools in urban and rural areas. Methods: Data were collected from 350 pupils (6-14 years) in Mpigi district, Uganda. All participants were offered a school lunch meal (usually maize porridge and boiled beans). Dietary survey was conducted in October 2016. Data for dietary intake levels were collected by the 24-hour recall method with trained school staffs. The data were converted into nutrient intakes using the CAN-Pro 5.0 Program and compared with KDRIs to evaluate the nutritional status of the subjects. Diet quality indexes such as nutrient density, nutrient adequacy ratio (NAR), mean adequacy ratio (MAR), and index of nutritional quality (INQ) and a diet diversity index such as diet diversity score (DDS) were calculated to evaluate nutritional status among subjects. Data were analyzed using SPSS statistical programs. Results: Results show that the intakes of most nutrients were significantly different by schools. The nutritional status of micro-nutrients was very low in both schools according to analysis of nutritional indexes such as NARs and INQs. Students from both schools should improve intakes of micro-nutrients related to child growth such as calcium, Vitamin B6, zinc and folate. According to the analysis of dietary diversity, there was difference in dietary patterns by schools presumably due to their locations. Conclusions This suggests that current meals could not provide adequate nutrients for the subjects and urgent nutrition interventions for school food services are needed to improve their nutritional well-being. New foods supplements based on local cuisine are also needed to ensure dietary diversity and sustainable development plans.

BACKGROUND: Embryonic stem cells (ESCs) can be expanded infinitely in vitro and have the potential to differentiate into hematopoietic stem cells (HSCs); thus, they are considered a useful source of cells for HSC production. Although several technical in vitro methods for engineering HSCs from pluripotent stem cells have been developed, clinical application of HSCs engineered from pluripotent stem cells is restricted because of the possibility of xenogeneic contamination resulting from the use of murine materials. METHODS: Human ESCs (CHA-hES15) were cultured on growth factor-reduced Matrigel-coated dishes in the mTeSR1 serum-free medium. When the cells were 70% confluent, we initiated HSC differentiation by three methods involving (1) knockout serum replacement (KSR), cytokines, TGFb1, EPO, and FLT3L; (2) KSR, cytokines, and bFGF; or (3) cytokines and bFGF. RESULTS: Among the three differentiation methods, the minimal number of cytokines without KSR resulted in the greatest production of HSCs. The optimized method resulted in a higher proportion of CD34⁺CD43⁺ hematopoietic progenitor cells (HPCs) and CD34⁺CD45⁺ HPCs compared to the other methods. In addition, the HSCs showed the potential to differentiate into multiple lineages of hematopoietic cells in vitro. CONCLUSION: In this study, we optimized a two-step, serum-free, animal protein-free, KSR-free, feeder-free, chemically defined monolayer culture method for generation of HSCs and hematopoietic stem and progenitor cells (HSPCs) from human ESCs.
Animals ; Cytokines ; Embryonic Stem Cells ; Hematopoietic Stem Cells* ; Human Embryonic Stem Cells* ; Humans* ; In Vitro Techniques ; Methods* ; Pluripotent Stem Cells ; Stem Cells

Cogan's syndrome can accompany a variety of systemic vasculitides including aortitis. A 45-year-old woman with a history of typical Cogan's syndrome presented with orthopnea and exertional dyspnea. Echocardiography demonstrated severe aortic valve insufficiency. Computed tomographic angiography demonstrated active vasculitis affecting the ascending and descending aortas and also showed stenosis of the left subclavian artery, both renal arteries, the celiac axis, the superior mesenteric artery, and the right common iliac artery. She received high dose corticosteroid and then underwent an aortic valve replacement. This is the first case of Cogan's syndrome with aortitis in Korea.
Angiography ; Aorta, Thoracic ; Aortic Valve ; Aortic Valve Insufficiency ; Aortitis ; Arthritis ; Axis, Cervical Vertebra ; Cogan Syndrome ; Constriction, Pathologic ; Dyspnea ; Echocardiography ; Female ; Humans ; Iliac Artery ; Korea ; Mesenteric Artery, Superior ; Middle Aged ; Renal Artery ; Subclavian Artery ; Systemic Vasculitis ; Vasculitis

Poststreptococcal glomerulonephritis (PSGN) is one of the most well-known and important infectious renal diseases resulting from a prior infection with group A beta-hemolytic streptococcus. The typical clinical characteristics of the disease reflect acute onset with gross hematuria, edema, hypertension and moderate proteinuria after the antecedent streptococcal infection. In children, usually PSGN is healed spontaneously but if it combines with fast progressing glomerulonephritis, it would be developed to chronic renal failure. Therefore, it is important to make a fast diagnosis and treatment by simple tools to predict the course and the prognosis of disease. Sonography is a simple tool for diagnosis but there is no typical renal sonographic finding in PSGN, so it is difficult to predict the course and the prognosis of disease by sonographic findings. In comparison between two cases of renal sonographic findings in PSGN, a patient who showed more increased echogenicity in more extended area of renal sonography had the severe results of renal pathology, prolonged treatment period and low serum C3 level. Here, we report the different findings of renal sonography and pathology depending on the degree of severity between two patients. Thus, it is necessary to gather more information from further studies to make a consensus about the relationship between the renal sonography and the prognosis of disease in PSGN.
Child ; Consensus ; Diagnosis ; Edema ; Glomerulonephritis* ; Hematuria ; Humans ; Hypertension ; Kidney Failure, Chronic ; Pathology ; Prognosis ; Proteinuria ; Streptococcal Infections ; Streptococcus ; Ultrasonography

BACKGROUND: We studied the association of sagittal abdominal diameter (SAD) and metabolic syndrome and insulin resistance in Korean adults. METHODS: This is a cross-sectional study by 190 patients who visited a Health Promotion Center of National University of Pusan from 11 November to 14 November, 2008. We analyzed the association of anthropometry (waist circumference, BMI, SAD), insulin resistance (homeostatic model assessment-insulin resistance, HOMA-IR), insulin sensitivity (quantitative insulin sensitivity check index, QUICKI), and plasma concentrations of glucose, insulin, total cholesterol, LDL, HDL, triglyceride. SAD was categorized into quartiles and assessed odds ratio of metabolic syndrome adjusted for age, sex, lifestyle factors. RESULTS: SAD showed significance correlation to HOMA-IR than BMI. Quartiles of SAD showed a positive trend with metabolic risk factors including abdominal obesity, high blood pressure, HOMA-IR and obesity but hypertriglyceridemia, low HDL-cholesterolemia did not show significant association. In men QUICKI was significantly high. A multivariate model, adjusted for age, sex, smoking status, physical activity, heavy drinking, HOMA-IR and QUICKI, revealed a progressively increased odds ratio of metabolic syndrome, 3rd quartile (odds ratio [OR]; 9.467; 95% confidence interval [CI], 3.225 to 27.789; P < 0.001) and 4th quartile (OR, 7.253; 95% CI, 2.437 to 21.586; P < 0.001), with increasing SAD. CONCLUSION: As shown above, SAD was a strong anthropometric marker of insulin resistance, risk of metabolic syndrome and decreased insulin sensitivity in Korean adults.
Adult ; Anthropometry ; Cholesterol, LDL ; Cross-Sectional Studies ; Drinking ; Glucose ; Health Promotion ; Humans ; Hypertension ; Hypertriglyceridemia ; Insulin ; Insulin Resistance ; Life Style ; Male ; Motor Activity ; Obesity ; Obesity, Abdominal ; Odds Ratio ; Plasma ; Risk Factors ; Smoke ; Smoking