No abstract available.
Catheters*

Krabbe disease is an autosomal recessive neurodegenerative disorder that affects both the central and peripheral nerve system due to an enzymatic defect of galactocerebroside bata-galactosidase. The patient had typical clinical features of Krabbe disease, with irritability, hypertonicity, failure to thrive, and opisthotonic posturing. A brain MRI demonstrates profound white matter demyelination. The diagnosis of Krabbe disease is suspected on the basis of clinical pictures and confirmed by finding markedly reduced galactocerebroside bata-galactosidase activity in leukocyte or cultured fibroblast. Here we present the first reported case of Krabbe disease in Korea confirmed by decreased activity of galactocerebroside bata-galactosidase enzyme in leukocyte.
Brain ; Demyelinating Diseases ; Diagnosis ; Failure to Thrive ; Fibroblasts ; Humans ; Korea ; Leukocytes ; Leukodystrophy, Globoid Cell* ; Magnetic Resonance Imaging ; Neurodegenerative Diseases ; Peripheral Nerves

PURPOSE: In this paper we tried to evaluate the most appropriate surface for rhBMP-2 coating among 4 rough titanium surfaces. MATERIALS AND METHODS: We used machined surface as a control group and anodized, RBM and SLA surfaces as test groups. We coated rhBMP-2 on the 4 surfaces and with uncoated surfaces for each case, we cultured human mesenchymal stem cells on all 8 surfaces. 24 hours after we measured the stem cell'attachment with SEM, and on 3rd, 7th, and 14th days, we checked the cell proliferation and differentiation by using MTT and ALP activity assay. And on the 7th day after the culture, we performed RT-PCR assay to determine whether the expression levels of Type I collagen, osteocalcin, osteopontin were changed. RESULTS: We observed with SEM that 4 rhBMP-2 coated surfaces exhibited wider and tighter cell attachment and more cell process spreading than uncoated surfaces. The anodized rhBMP-2 surface caused robustest effects. In MTT assay we could not find any meaningful difference. In ALP assay there was a significant increase (P<.05) in the ALP activity of anodized rhBMP-2 coated surface compared with that of the control (3rd and 14th days) and with that of the RBM rhBMP-2 coated surface (14th day). In RT-PCR assay there was increased expressions in the anodized rhBMP-2 coated surface for osteocalcin, and osteopontin. CONCLUSION: We found that the anodized rhBMP-2 coated surface were most prominent stem cell attachment and differentiation in compared to control and Machined rhBMP-2 coated, RBM rhBMP-2 coated surface.
Cell Proliferation ; Collagen Type I ; Humans ; Mesenchymal Stromal Cells ; Osteocalcin ; Osteopontin ; Stem Cells ; Titanium

PURPOSE: Spirometry, because of its simplicity and reproducibility, has been used to evaluate pulmonary function in childhood asthma. We investigated whether FEF25-75% is the most sensitive parameter among other spirometric parameters in the diagnosis of pulmonary function and airway obstruction. METHODS: This study included 32 children with asthma. We recorded values of spirometry(FVC, FEV1, PEFR, and FEF25-75%) before and after symptomatic improvement and compared them. The values were recorded as a percent of the predicted normal value. RESULTS: The FEV1 value before and after symptomatic improvement was 71.7+/-15.6% and 83.8+/-12.8%, respectively; PEFR was 75.4+/-18.3% and 85.0+/-16.0%; and FEF25-75% was 60.8+/-17.9% and 81.5+/-16.3%. According to symptomatic improvement, FEF25-75% improved more than the other spirometric parameters. Of the 40 cases of spitometric measurement before symptomatic improvement, abnormal FEV1, PEFR, FEF25-75% values were observed in 25, 17 and 28 cases, respectively. There was statistically significant difference between FEF25-75% and PEFR(P=0.005), but not between FEF25-75% and FEV1(P=0.091). After symptomatic improvement, abnormal FEV1, PEFR, and FEF25-75% values were observed in 14, 12 and 9 cases, respectively. There was statistically significant difference between FEF25-75% and FEV1(P=0.044) but not between FEF25-75% and PEFR (P=0.097). The cases of normalized values with symptomatic improvement, 11 in FEV1, 7 in PEFR and 19 in FEF25-75%. CONCLUSION: FEF25-75% showed the most improvement rate before and after symptomatic improvement. FEF25-75% was helpful in the assessment of treatment effectiveness and reversible bronchial responsiveness. However, this study did not suggest that FEF25-75% is the most sensitive measurement of airway obstruction.
Airway Obstruction ; Asthma* ; Child ; Diagnosis ; Humans ; Peak Expiratory Flow Rate ; Reference Values ; Spirometry ; Treatment Outcome

E-cadherin is a cell adhesion molecule that plays an important role in maintaining renal epithelial polarity and integrity. The purpose of this study was to determine the exact cellular localization of E-cadherin in pig kidney. Kidney tissues from pigs were processed for light and electron microscopy immunocytochemistry, and immunoblot analysis. E-cadhedrin bands of the same size were detected by immunoblot of samples from rat and pig kidneys. In pig kidney, strong E-cadherin expression was observed in the basolateral plasma membrane of the tubular epithelial cells. E-cadherin immunolabeling was not detected in glomeruli or blood vessels of pig kidney. Double-labeling results demonstrated that E-cadherin was expressed in the calbindin D28k-positive distal convoluted tubule and H(+)-ATPase-positive collecting duct, but not in the aquaporin 1-positive, N-cadherin-positive proximal tubule. In contrast to rat, E-cadherin immunoreactivity was not expressed at detectable levels in the Tamm-Horsfall protein-positive thick ascending limb of pig kidney. Immunoelectron microscopy confirmed that E-cadherin was localized in both the lateral membranes and basal infoldings of the collecting duct. These results suggest that E-cadherin may be a critical adhesion molecule in the distal convoluted tubule and collecting duct cells of pig kidney.
Animals ; Blotting, Western/veterinary ; Cadherins/*genetics/metabolism ; Cell Membrane/*metabolism/ultrastructure ; *Gene Expression Regulation ; Kidney/*metabolism ; Male ; Microscopy, Electron, Transmission/veterinary ; Sus scrofa/*genetics/metabolism

Ectopic pancreas is defined as an abnormally located pancreatic tissue not sufficiently connected with the normal pancreas, which rarely occurs in neonates. To our knowledge, only a few cases of ectopic pancreas have been reported in newborns in South Korea. We report a case of ectopic pancreas as the cause of intussusception and jejunal atresia in a newborn. This clinical association is extremely rare, and this is the first report in South Korea.

Ectopic pancreas is defined as an abnormally located pancreatic tissue not sufficiently connected with the normal pancreas, which rarely occurs in neonates. To our knowledge, only a few cases of ectopic pancreas have been reported in newborns in South Korea. We report a case of ectopic pancreas as the cause of intussusception and jejunal atresia in a newborn. This clinical association is extremely rare, and this is the first report in South Korea.

Since Winslow and Edwards24) reviewed 20 cases of the arteriovenous fistulas involving temporal artery described in the world wide literatures up to 1934, several authors have added the cases sporadically, but the arteriovenous fistula of the superficial temporal artery is unusual to the head and neck surgeons. Recently we experienced one case of huge traumatic arteriovenous fistula involving the superficial temporal artery and present it here.
Arteriovenous Fistula* ; Head ; Neck ; Temporal Arteries*