Renal dysplasia

Common Name(s)

Renal dysplasia

Autosomal recessive renal tubular dysgenesis is a severe disorder of renal tubular development characterized by persistent fetal anuria and perinatal death, probably due to pulmonary hypoplasia from early-onset oligohydramnios (the Potter phenotype) ({5:Gribouval et al., 2005}). Absence or paucity of differentiated proximal tubules is the histopathologic hallmark of the disorder and may be associated with skull ossification defects.
 

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Condition Specific Organizations

Following organizations serve the condition "Renal dysplasia" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Renal dysplasia" returned 99 free, full-text research articles on human participants. First 3 results:

Bilateral renal dysplasia, nephroblastomatosis, and bronchial stenosis. A new syndrome?
 

Author(s): Maria Matilde Rodriguez, Mayrin Correa-Medina, Elizabeth E Whittington

Journal: Fetal Pediatr Pathol. 2015 Jun;34(3):190-6.

 

Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or ...

Last Updated: 21 May 2015

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Insights into the renal pathogenesis in Schimke immuno-osseous dysplasia: A renal histological characterization and expression analysis.
 

Author(s): Sanjay Sarin, Ashkan Javidan, Felix Boivin, Iakovina Alexopoulou, Dusan Lukic, Bruno Svajger, Stephanie Chu, Alireza Baradaran-Heravi, Cornelius F Boerkoel, Norman D Rosenblum, Darren Bridgewater

Journal: J. Histochem. Cytochem.. 2015 Jan;63(1):32-44.

 

Schimke immuno-osseous dysplasia (SIOD) is a pleiotropic disorder caused by mutations in the SWI/SNF2-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like-1 (SMARCAL1) gene, with multiple clinical features, notably end-stage renal disease. Here we characterize ...

Last Updated: 27 Dec 2014

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A giant renal angiomyolipoma (AML) in a patient with septo-optic dysplasia (SOD).
 

Author(s): Marcin Cichocki, Marek Sosnowski, Zbigniew Jablonowski

Journal:

 

Angiomyolipoma (AML) is a rare benign renal tumor occurring in about 0.3 to 3% of the general population. Most frequently it takes the form of small single tumors occurring sporadically or accompanying tuberous sclerosis (Bourneville-Pringle disease). In some cases the tumor may reach ...

Last Updated: 28 Feb 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Renal dysplasia" returned 8 free, full-text review articles on human participants. First 3 results:

Fibromuscular dysplasia affecting a two-branched renal artery in a patient with a solitary kidney: case presentation.
 

Author(s): Xuanqi An, Xiongjing Jiang, Hui Dong, Meng Peng, Yubao Zou, Lei Song, Ting Guan, Xianliang Zhou, Yuejin Yang

Journal: Clin Cardiol. 2013 Aug;36(8):E7-10.

 

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease, commonly involving the renal arteries. Here we report a case of a 16-year-old Chinese male who was found to have severe hypertension with proteinuria for 2 years. Computed tomography showed absence ...

Last Updated: 16 Sep 2013

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Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: a systematic review and meta-analysis.
 

Author(s): Ludovic Trinquart, Claire Mounier-Vehier, Marc Sapoval, Nathalie Gagnon, Pierre-Fran├žois Plouin

Journal: Hypertension. 2010 Sep;56(3):525-32.

 

In patients with fibromuscular dysplasia and renal artery stenosis, renal artery revascularization has been used to cure hypertension or to improve blood pressure control. To provide an up-to-date assessment of the benefits and risks associated with revascularization in this condition, ...

Last Updated: 19 Aug 2010

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Genetic approaches to human renal agenesis/hypoplasia and dysplasia.
 

Author(s): Simone Sanna-Cherchi, Gianluca Caridi, Patricia L Weng, Francesco Scolari, Francesco Perfumo, Ali G Gharavi, Gian Marco Ghiggeri

Journal: Pediatr. Nephrol.. 2007 Oct;22(10):1675-84.

 

Congenital abnormalities of the kidney and urinary tract are frequently observed in children and represent a significant cause of morbidity and mortality. These conditions are phenotypically variable, often affecting several segments of the urinary tract simultaneously, making clinical ...

Last Updated: 27 Aug 2007

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Ambulatory Blood Pressure Measurement in Children With Congenital Urine Flow Obstruction
 

Status: Available

Condition Summary: Hydronephrosis; Multicystic-Dysplastic Kidney

 

Last Updated: 1 Oct 2008

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Assessment of Renal Artery Fibromuscular Dysplasia: From Diagnosis to Treatment
 

Status: Recruiting

Condition Summary: Renal Artery Fibromuscular Dysplasia

 

Last Updated: 2 Dec 2015

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