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	Provedor de dados BJMBR (3) Autor Onuchic,L.F. (3) Balbo,B.E.P. (1) Bastos,A.P. (1) Castro,I. (1) Dettoni,J.B. (1) Jayanthi,S.K. (1) Menezes,L.F. (1) Ono,C.R. (1) Sapienza,M.T. (1) Mais... Palavra-chave Autosomal dominant polycystic kidney disease (2) Cystogenesis (2) Primary cilium (2) ARPKD (1) Autosomal recessive polycystic kidney disease (1) Cyst infection (1) Kidney volume (1) Liver volume (1) Mortality (1) PKD1 gene (1) PKD2 gene (1) PKHD1 gene (1) Polycystic kidney disease (1) Polycystins 1 and 2 (1) Polyductin/Fibrocystin (1) Positron-emission tomography/computed tomography (1) Mais... Tipo do documento Info:eu-repo/semantics/article (3) Ano 2014 (1) 2011 (1) 2006 (1) País Brazil (3) Idioma Inglês (3)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 3 Primeira ... 1 ... Última Molecular and cellular pathogenesis of autosomal dominant polycystic kidney disease BJMBR Bastos,A.P.; Onuchic,L.F.. Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common human life-threatening monogenic disorders. The disease is characterized by bilateral, progressive renal cystogenesis and cyst and kidney enlargement, often leading to end-stage renal disease, and may include extrarenal manifestations. ADPKD is caused by mutation in one of two genes, PKD1 and PKD2, which encode polycystin-1 (PC1) and polycystin-2 (PC2), respectively. PC2 is a non-selective cation channel permeable to Ca2+, while PC1 is thought to function as a membrane receptor. The cyst cell phenotype includes increased proliferation and apoptosis, dedifferentiation, defective planar polarity, and a secretory pattern associated with extracellular matrix remodeling. The two-hit... Tipo: Info:eu-repo/semantics/article Palavras-chave: Autosomal dominant polycystic kidney disease; PKD1 gene; PKD2 gene; Polycystins 1 and 2; Cystogenesis; Primary cilium. Ano: 2011 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2011000700001 Molecular and cellular pathogenesis of autosomal recessive polycystic kidney disease BJMBR Menezes,L.F.; Onuchic,L.F.. Autosomal recessive polycystic kidney disease (ARPKD) is an inherited disease characterized by a malformation complex which includes cystically dilated tubules in the kidneys and ductal plate malformation in the liver. The disorder is observed primarily in infancy and childhood, being responsible for significant pediatric morbidity and mortality. All typical forms of ARPKD are caused by mutations in a single gene, PKHD1 (polycystic kidney and hepatic disease 1). This gene has a minimum of 86 exons, assembled into multiple differentially spliced transcripts and has its highest level of expression in kidney, pancreas and liver. Mutational analyses revealed that all patients with both mutations associated with truncation of the longest open reading... Tipo: Info:eu-repo/semantics/article Palavras-chave: Autosomal recessive polycystic kidney disease; ARPKD; PKHD1 gene; Polyductin/Fibrocystin; Polycystic kidney disease; Primary cilium; Cystogenesis. Ano: 2006 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2006001200004 Cyst infection in hospital-admitted autosomal dominant polycystic kidney disease patients is predominantly multifocal and associated with kidney and liver volume BJMBR Balbo,B.E.P.; Sapienza,M.T.; Ono,C.R.; Jayanthi,S.K.; Dettoni,J.B.; Castro,I.; Onuchic,L.F.. Positron-emission tomography/computed tomography (PET/CT) has improved cyst infection (CI) management in autosomal dominant polycystic kidney disease (ADPKD). The determinants of kidney and/or liver involvement, however, remain uncertain. In this study, we evaluated clinical and imaging factors associated with CI in kidney (KCI) and liver (LCI) in ADPKD. A retrospective cohort study was performed in hospital-admitted ADPKD patients with suspected CI. Clinical, imaging and surgical data were analyzed. Features of infected cysts were evaluated by PET/CT. Total kidney (TKV) and liver (TLV) volumes were measured by CT-derived multiplanar reconstruction. CI was detected in 18 patients who experienced 24 episodes during an interval of 30 months (LCI in 12, KCI... Tipo: Info:eu-repo/semantics/article Palavras-chave: Autosomal dominant polycystic kidney disease; Cyst infection; Positron-emission tomography/computed tomography; Kidney volume; Liver volume; Mortality. Ano: 2014 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2014000700584 Registros recuperados: 3 Primeira ... 1 ... Última

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