2.Fabry disease previously diagnosed as Henoch-Schonlein purpura.
Ji Hyeong KIM ; Dong Hoon HAN ; Moo Yong PARK ; Soo Jeong CHOI ; Jin Kuk KIM ; Seung Duk HWANG ; So Young JIN
The Korean Journal of Internal Medicine 2015;30(6):925-927
No abstract available.
Biopsy
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DNA Mutational Analysis
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*Diagnostic Errors
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Enzyme Replacement Therapy
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Fabry Disease/complications/*diagnosis/enzymology/genetics
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Genetic Predisposition to Disease
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Glomerulonephritis, IGA/diagnosis/etiology
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Humans
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Male
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Middle Aged
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Mutation
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Phenotype
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Predictive Value of Tests
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Purpura, Schoenlein-Henoch/*diagnosis
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alpha-Galactosidase/genetics/therapeutic use
3.Urokinase, urokinase receptor, and plasminogen activator inhibitor-1 expression on podocytes in immunoglobulin A glomerulonephritis.
Ji Hye LEE ; Mee Hye OH ; Jae Seok PARK ; Gyoung Jae NA ; Hye Wook GIL ; Jong Oh YANG ; Eun Young LEE ; Sae Yong HONG
The Korean Journal of Internal Medicine 2014;29(2):176-182
BACKGROUND/AIMS: The purpose of this study was to investigate the expression of urokinase-type plasminogen activator (uPA), uPA receptor (uPAR), and plasminogen activator inhibitor (PAI)-1 on podocytes in immunoglobulin A (IgA) glomerulonephritis (GN). METHODS: Renal biopsy specimens from 52 IgA GN patients were deparaffinized and subjected to immunohistochemical staining for uPA, PAI-1, and uPAR. The biopsies were classified into three groups according to the expression of uPA and uPAR on podocytes: uPA, uPAR, and a negative group. The prevalences of the variables of the Oxford classification for IgA GN were compared among the groups. RESULTS: On podocytes, uPA was positive in 11 cases and uPAR was positive in 38 cases; by contrast, PAI-1 was negative in all cases. Expression of both uPA and uPAR on podocytes was less frequently accompanied by tubulointerstitial fibrosis. CONCLUSIONS: Our results suggest a possible protective effect of podocyte uPA/uPAR expression against interstitial fibrosis.
Adolescent
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Adult
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Aged
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Atrophy
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Biological Markers/analysis
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Biopsy
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Female
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Fibrosis
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Glomerulonephritis, IGA/diagnosis/*enzymology/immunology
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Humans
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Immunohistochemistry
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Male
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Middle Aged
;
Plasminogen Activator Inhibitor 1/*analysis
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Podocytes/*enzymology/immunology/pathology
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Receptors, Urokinase Plasminogen Activator/*analysis
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Urokinase-Type Plasminogen Activator/*analysis
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Young Adult