Nefropatía C1q en un paciente de 17 años: Caso clínico

Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reponed. It is defined by a pattern of immunofluorescense (IF) with dominant or co-dominant complement Clq with electrondense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. Th...

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Autores principales:	Panuncio,Ana, Alba,Andrea, Bacigalupe,Claudia, Caorsi,Hena, Schwedt,Emma, Otatti,Gabriela, Saralegui,Patricia, Marino,Ana
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2008
Materias:	Complement Clq Kidney diseases Proteinuria
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001000011
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spelling	oai:scielo:S0034-988720080010000112009-01-15Nefropatía C1q en un paciente de 17 años: Caso clínicoPanuncio,AnaAlba,AndreaBacigalupe,ClaudiaCaorsi,HenaSchwedt,EmmaOtatti,GabrielaSaralegui,PatriciaMarino,Ana Complement Clq Kidney diseases Proteinuria Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reponed. It is defined by a pattern of immunofluorescense (IF) with dominant or co-dominant complement Clq with electrondense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. The most common histopathological findings of ClqN are focal segmental glomerulosclerosis and Minimal Change Disease. We repon a 17 year-old male patient with an isolated selective proteinuría found in a routine study. He had normal renal function and uriñe culture was negative. Serum lipids, liver enzymes an complement were all normal. Serum antinuclear and anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), HIV, Hepatitis B and C serology, were negative. Renal and abdominal ultrasonography was normal. The histopathological study revealed segmental glomerular sderosis, modérate increase of mesangial matrix, Bowmann capsule adhesions and fucsinophil deposits in mesangium. The IF was positive (dominant) for Clq (+++) and IgA, IgG, IgM, C3++, all of them with a granular mesangial distribution. Ultrastructural findings were pedicelar effacement and paramesangial electrondense deposits. Tubular reticular inclusions (TRI) were not found. Remission of proteinuría was reached after 18 months of treatment with enalapril and losartan. The patient remains with normal renal function. Clinical findings, negative serology for Lupus, light microscopy IFwith dominant positivity for Clq, absence of TRI and paramesangial electrondense deposits in electron microscopy lead us to the diagnosis of ClqN. A poor response to steroid therapy was described in ClqN. Thus it was worthwhile to differentiate it from lupus nephritis, that is responsive to steroids.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.136 n.10 20082008-10-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001000011es10.4067/S0034-98872008001000011
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Complement Clq Kidney diseases Proteinuria
spellingShingle	Complement Clq Kidney diseases Proteinuria Panuncio,Ana Alba,Andrea Bacigalupe,Claudia Caorsi,Hena Schwedt,Emma Otatti,Gabriela Saralegui,Patricia Marino,Ana Nefropatía C1q en un paciente de 17 años: Caso clínico
description	Clq nephropathy (Clq N) is an infrequent disease and only about 100 cases have been reponed. It is defined by a pattern of immunofluorescense (IF) with dominant or co-dominant complement Clq with electrondense deposits in the mesangium, without clinical or serological features of Lupus Nephritis. The most common histopathological findings of ClqN are focal segmental glomerulosclerosis and Minimal Change Disease. We repon a 17 year-old male patient with an isolated selective proteinuría found in a routine study. He had normal renal function and uriñe culture was negative. Serum lipids, liver enzymes an complement were all normal. Serum antinuclear and anti-DNA antibodies, antineutrophil cytoplasmic antibodies (ANCA), HIV, Hepatitis B and C serology, were negative. Renal and abdominal ultrasonography was normal. The histopathological study revealed segmental glomerular sderosis, modérate increase of mesangial matrix, Bowmann capsule adhesions and fucsinophil deposits in mesangium. The IF was positive (dominant) for Clq (+++) and IgA, IgG, IgM, C3++, all of them with a granular mesangial distribution. Ultrastructural findings were pedicelar effacement and paramesangial electrondense deposits. Tubular reticular inclusions (TRI) were not found. Remission of proteinuría was reached after 18 months of treatment with enalapril and losartan. The patient remains with normal renal function. Clinical findings, negative serology for Lupus, light microscopy IFwith dominant positivity for Clq, absence of TRI and paramesangial electrondense deposits in electron microscopy lead us to the diagnosis of ClqN. A poor response to steroid therapy was described in ClqN. Thus it was worthwhile to differentiate it from lupus nephritis, that is responsive to steroids.
author	Panuncio,Ana Alba,Andrea Bacigalupe,Claudia Caorsi,Hena Schwedt,Emma Otatti,Gabriela Saralegui,Patricia Marino,Ana
author_facet	Panuncio,Ana Alba,Andrea Bacigalupe,Claudia Caorsi,Hena Schwedt,Emma Otatti,Gabriela Saralegui,Patricia Marino,Ana
author_sort	Panuncio,Ana
title	Nefropatía C1q en un paciente de 17 años: Caso clínico
title_short	Nefropatía C1q en un paciente de 17 años: Caso clínico
title_full	Nefropatía C1q en un paciente de 17 años: Caso clínico
title_fullStr	Nefropatía C1q en un paciente de 17 años: Caso clínico
title_full_unstemmed	Nefropatía C1q en un paciente de 17 años: Caso clínico
title_sort	nefropatía c1q en un paciente de 17 años: caso clínico
publisher	Sociedad Médica de Santiago
publishDate	2008
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001000011
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Nefropatía C1q en un paciente de 17 años: Caso clínico

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