نتایج جستجو برای: arpkd

تعداد نتایج: 183  

2017
William E Sweeney Philip Frost Ellis D Avner

AIM To investigate the therapeutic potential of tesevatinib (TSV), a unique multi-kinase inhibitor currently in Phase II clinical trials for autosomal dominant polycystic kidney disease (ADPKD), in well-defined rodent models of autosomal recessive polycystic kidney disease (ARPKD). METHODS We administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk mod...

2005
A M Sharp L M Messiaen G Page C Antignac M-C Gubler L F Onuchic S Somlo G G Germino L M Guay-Woodford

A utosomal recessive polycystic kidney disease (ARPKD; MIM 263200) is an important childhood nephropathy, occurring in 1 in 20 000 live births. The clinical phenotype is dominated by dilatation of the renal collecting ducts, biliary dysgenesis, and portal tract fibrosis. Affected children often present in utero with enlarged, echogenic kidneys, as well as oligohydramnios secondary to poor urine...

Journal: :Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2011
Catharina Renken Dagmar-Christiane Fischer Günther Kundt Norbert Gretz Dieter Haffner

BACKGROUND Activation of the mTOR pathway has been implicated in the mediation of the progression of polycystic kidney disease (PKD). Whereas targeted inhibition of mTOR has been proven to be effective in various animal models of autosomal dominant PKD, its efficacy in autosomal recessive PKD (ARPKD) remains to be elucidated. We examined the effects of sirolimus in PCK rats, an orthologous anim...

Journal: :Jornal brasileiro de nefrologia : 'orgao oficial de Sociedades Brasileira e Latino-Americana de Nefrologia 2010
Natasha Favoretto Dias Vivian Lanzarini Luiz Fernando Onuchic Vera Hermina Kalika Koch

INTRODUCTION Autosomal Recessive Polycystic Kidney Disease (ARPKD) is an important pediatric cause of morbidity and mortality, with a variable clinical spectrum. METHODS The clinical presentation and evolution of 25 patients (Pts) were analyzed by clinical record review, according to the forms proposed by Guay-Woodford et al. Morbidities associated with the disease were evaluated with respect...

Journal: :Journal of the American Society of Nephrology : JASN 2003
Laszlo Furu Luiz F Onuchic Ali Gharavi Xiaoying Hou Ernie L Esquivel Yasuyuki Nagasawa Carsten Bergmann Jan Senderek Ellis Avner Klaus Zerres Gregory G Germino Lisa M Guay-Woodford Stefan Somlo

Autosomal recessive polycystic kidney disease (ARPKD; MIM 263200) is a hereditary and severe form of polycystic disease affecting the kidneys and biliary tract with an estimated incidence of 1 in 20,000 live births. The clinical spectrum is widely variable: up to 50% of affected neonates die shortly after birth, whereas others survive to adulthood. Mutations at a single locus, polycystic kidney...

Journal: :Journal of the American Society of Nephrology : JASN 1995
T Nadasdy Z Laszik G Lajoie K E Blick D E Wheeler F G Silva

Increased proliferative activity of the renal tubular epithelium is thought to be a prerequisite for renal cyst formation by many investigators. However, in humans, the exact in vivo proliferation rate of epithelial cells lining these cysts is not known. In this study, which used immunohistochemical methods with an antibody to proliferating cell nuclear antigen (PCNA), the proliferation index (...

پایان نامه :وزارت علوم، تحقیقات و فناوری - دانشگاه شهید چمران اهواز - دانشکده علوم 1392

کلید واژه ها: adpkd,arpkd، ژن pkd1، ژن pkhd1، کلیه پلی کیستیک ، جهش چکیده: بیماری کلیه ی پلی کیستیک (pkd) به دو صورت غالب اتوزوم ( adpkd) و مغلوب اتوزوم ( arpkd) به ارث می رسد و میزان شیوع آن در جهان به ترتیب 1 در1000 و 1 در 40000 تولد زنده است. بیماری pkd بوسیله ی حضور تعداد زیادی کیست در یک یا هردو کلیه تشخیص داده می شود و وجه تمایز دو نوع بیماری بر اساس الگوی توارث، سایر علائم خارج کلیوی و ...

Background Autosomal recessive polycystic kidney disease (ARPKD; OMIM number 263200) is a severe early onset hereditary form of polycystic kidney and liver disease. Case Report In the current study, we present a consanguineous couple with a history of an affected son with polycystic kidney disease (PKD), hepatic failure and epileptic seizures who died at the age of 8 months. Both parents were h...

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