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Statements

Subject Item
n2:RIV%2F00216208%3A11110%2F13%3A10173664%21RIV15-MSM-11110___
rdf:type
n11:Vysledek skos:Concept
rdfs:seeAlso
http://dx.doi.org/10.1186/1471-2369-14-6
dcterms:description
Background: IgA nephropathy (IgAN) is the most common glomerulonephritis, which may also coexist with other diseases. We present two patients with an unusual coincidence of IgAN and Fabry disease (FD). Case presentation: A 26 year-old man underwent a renal biopsy in February 2001. Histopathology showed very advanced IgAN and vascular changes as a result of hypertension. Because of his progressive renal insufficiency the patient began hemodialysis in August 2001. By means of the blood spot test screening method the diagnosis of FD was suspected. Low activity of alpha-galactosidase A in the patient's plasma and leukocytes and DNA analysis confirmed the diagnosis of FD. Enzyme replacement therapy started in July 2004. Then the patient underwent kidney transplantation in November 2005. Currently, his actual serum creatinine level is 250 mu mol/l. Other organ damages included hypertrophic cardiomyopathy, neuropathic pain and febrile crisis. After enzyme replacement therapy, myocardial hypertrophy has stabilized and other symptoms have disappeared. No further progression of the disease has been noted. The other patient, a 30 year-old woman, suffered from long-term hematuria with a good renal function. Recently, proteinuria (2.6 g/day) appeared and a renal biopsy was performed. Histopathology showed IgAN with remarkably enlarged podocytes. A combination of IgAN and a high suspicion of FD was diagnosed. Electron microscopy revealed dense deposits in paramesangial areas typical for IgAN and podocytes with inclusive zebra bodies and myelin figures characteristic of FD. FD was confirmed by the decreased alpha-galactosidase A activity in plasma and leukocytes and by DNA and RNA analysis. Enzyme replacement therapy and family screening were initiated. Conclusions: Our results emphasize the role of complexity in the process of diagnostic evaluation of kidney biopsy samples. Background: IgA nephropathy (IgAN) is the most common glomerulonephritis, which may also coexist with other diseases. We present two patients with an unusual coincidence of IgAN and Fabry disease (FD). Case presentation: A 26 year-old man underwent a renal biopsy in February 2001. Histopathology showed very advanced IgAN and vascular changes as a result of hypertension. Because of his progressive renal insufficiency the patient began hemodialysis in August 2001. By means of the blood spot test screening method the diagnosis of FD was suspected. Low activity of alpha-galactosidase A in the patient's plasma and leukocytes and DNA analysis confirmed the diagnosis of FD. Enzyme replacement therapy started in July 2004. Then the patient underwent kidney transplantation in November 2005. Currently, his actual serum creatinine level is 250 mu mol/l. Other organ damages included hypertrophic cardiomyopathy, neuropathic pain and febrile crisis. After enzyme replacement therapy, myocardial hypertrophy has stabilized and other symptoms have disappeared. No further progression of the disease has been noted. The other patient, a 30 year-old woman, suffered from long-term hematuria with a good renal function. Recently, proteinuria (2.6 g/day) appeared and a renal biopsy was performed. Histopathology showed IgAN with remarkably enlarged podocytes. A combination of IgAN and a high suspicion of FD was diagnosed. Electron microscopy revealed dense deposits in paramesangial areas typical for IgAN and podocytes with inclusive zebra bodies and myelin figures characteristic of FD. FD was confirmed by the decreased alpha-galactosidase A activity in plasma and leukocytes and by DNA and RNA analysis. Enzyme replacement therapy and family screening were initiated. Conclusions: Our results emphasize the role of complexity in the process of diagnostic evaluation of kidney biopsy samples.
dcterms:title
The coincidence of IgA nephropathy and Fabry disease The coincidence of IgA nephropathy and Fabry disease
skos:prefLabel
The coincidence of IgA nephropathy and Fabry disease The coincidence of IgA nephropathy and Fabry disease
skos:notation
RIV/00216208:11110/13:10173664!RIV15-MSM-11110___
n5:aktivita
n17:I
n5:aktivity
I
n5:cisloPeriodika
6
n5:dodaniDat
n8:2015
n5:domaciTvurceVysledku
n6:2876361 n6:3281620 n6:7995962 n6:7168187 n6:5909627 n6:5401909 n6:7311982 n6:7469802 n6:7988818 n6:8180229 n6:3167232
n5:druhVysledku
n15:J
n5:duvernostUdaju
n19:S
n5:entitaPredkladatele
n10:predkladatel
n5:idSjednocenehoVysledku
65895
n5:idVysledku
RIV/00216208:11110/13:10173664
n5:jazykVysledku
n12:eng
n5:klicovaSlova
Alpha-galactosidase A; IgA nephropathy; Fabry disease
n5:klicoveSlovo
n13:Fabry%20disease n13:Alpha-galactosidase%20A n13:IgA%20nephropathy
n5:kodStatuVydavatele
DE - Spolková republika Německo
n5:kontrolniKodProRIV
[0BCD7887ED03]
n5:nazevZdroje
BMC Nephrology
n5:obor
n18:FE
n5:pocetDomacichTvurcuVysledku
11
n5:pocetTvurcuVysledku
11
n5:rokUplatneniVysledku
n8:2013
n5:svazekPeriodika
14
n5:tvurceVysledku
Reiterová, Jana Dvořáková, Lenka Kidorová, Jana Merta, Miroslav Neprašová, Michaela Goláň, Lubor Honsová, Eva Tesař, Vladimír Poupětová, Helena Ryšavá, Romana Maixnerová, Dita
n5:wos
000314167900001
s:issn
1471-2369
s:numberOfPages
7
n7:doi
10.1186/1471-2369-14-6
n9:organizacniJednotka
11110