Polycystic Kidney, Autosomal Recessive
"Polycystic Kidney, Autosomal Recessive" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
A genetic disorder with autosomal recessive inheritance, characterized by multiple CYSTS in both KIDNEYS and associated LIVER lesions. Serious manifestations are usually present at BIRTH with high PERINATAL MORTALITY.
Descriptor ID |
D017044
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MeSH Number(s) |
C12.777.419.403.875.510 C13.351.968.419.403.875.510 C16.320.793
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Concept/Terms |
Polycystic Kidney, Autosomal Recessive- Polycystic Kidney, Autosomal Recessive
- Polycystic Kidney Disease, Infantile, Type I
- ARPKD
- Polycystic Kidney Disease, Autosomal Recessive
- Autosomal Recessive Polycystic Kidney Disease
- Kidney, Polycystic, Autosomal Recessive
- Polycystic Kidney and Hepatic Disease 1
- Polycystic Kidney and Hepatic Disease 1 (Autosomal Recessive)
- Polycystic Kidney Disease, Infantile, Type 1
- Autosomal Recessive Polycystic Kidney
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Below are MeSH descriptors whose meaning is more general than "Polycystic Kidney, Autosomal Recessive".
Below are MeSH descriptors whose meaning is more specific than "Polycystic Kidney, Autosomal Recessive".
This graph shows the total number of publications written about "Polycystic Kidney, Autosomal Recessive" by people in this website by year, and whether "Polycystic Kidney, Autosomal Recessive" was a major or minor topic of these publications.
To see the data from this visualization as text, click here.
Year | Major Topic | Minor Topic | Total |
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2002 | 3 | 0 | 3 | 2003 | 2 | 0 | 2 | 2004 | 1 | 0 | 1 | 2005 | 2 | 0 | 2 | 2006 | 2 | 0 | 2 | 2007 | 2 | 0 | 2 | 2008 | 1 | 0 | 1 | 2009 | 2 | 0 | 2 | 2011 | 0 | 1 | 1 | 2012 | 1 | 0 | 1 | 2013 | 2 | 0 | 2 | 2014 | 4 | 0 | 4 | 2015 | 1 | 1 | 2 |
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Below are the most recent publications written about "Polycystic Kidney, Autosomal Recessive" by people in Profiles.
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Mrug M, Zhou J, Yang C, Aronow BJ, Cui X, Schoeb TR, Siegal GP, Yoder BK, Guay-Woodford LM. Genetic and Informatic Analyses Implicate Kif12 as a Candidate Gene within the Mpkd2 Locus That Modulates Renal Cystic Disease Severity in the Cys1cpk Mouse. PLoS One. 2015; 10(8):e0135678.
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Cramer MT, Guay-Woodford LM. Cystic kidney disease: a primer. Adv Chronic Kidney Dis. 2015 Jul; 22(4):297-305.
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Lambie L, Amin R, Essop F, Cnaan A, Krause A, Guay-Woodford LM. Clinical and genetic characterization of a founder PKHD1 mutation in Afrikaners with ARPKD. Pediatr Nephrol. 2015 Feb; 30(2):273-9.
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Hartung EA, Guay-Woodford LM. Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics. 2014 Sep; 134(3):e833-45.
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Guay-Woodford LM, Bissler JJ, Braun MC, Bockenhauer D, Cadnapaphornchai MA, Dell KM, Kerecuk L, Liebau MC, Alonso-Peclet MH, Shneider B, Emre S, Heller T, Kamath BM, Murray KF, Moise K, Eichenwald EE, Evans J, Keller RL, Wilkins-Haug L, Bergmann C, Gunay-Aygun M, Hooper SR, Hardy KK, Hartung EA, Streisand R, Perrone R, Moxey-Mims M. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: report of an international conference. J Pediatr. 2014 Sep; 165(3):611-7.
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Hartung EA, Matheson M, Lande MB, Dell KM, Guay-Woodford LM, Gerson AC, Warady BA, Hooper SR, Furth SL. Neurocognition in children with autosomal recessive polycystic kidney disease in the CKiD cohort study. Pediatr Nephrol. 2014 Oct; 29(10):1957-65.
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Wu M, Yang C, Tao B, Bu S, Guay-Woodford LM. The ciliary protein cystin forms a regulatory complex with necdin to modulate Myc expression. PLoS One. 2013; 8(12):e83062.
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Mrug M, Zhou J, Guay-Woodford LM, Smythies LE. Renal macrophages in autosomal recessive polycystic kidney disease. Nephrology (Carlton). 2013 Nov; 18(11):746.
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Gunay-Aygun M, Font-Montgomery E, Lukose L, Tuchman Gerstein M, Piwnica-Worms K, Choyke P, Daryanani KT, Turkbey B, Fischer R, Bernardini I, Sincan M, Zhao X, Sandler NG, Roque A, Douek DC, Graf J, Huizing M, Bryant JC, Mohan P, Gahl WA, Heller T. Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease. Gastroenterology. 2013 Jan; 144(1):112-121.e2.
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Raynaud P, Tate J, Callens C, Cordi S, Vandersmissen P, Carpentier R, Sempoux C, Devuyst O, Pierreux CE, Courtoy P, Dahan K, Delbecque K, Lepreux S, Pontoglio M, Guay-Woodford LM, Lemaigre FP. A classification of ductal plate malformations based on distinct pathogenic mechanisms of biliary dysmorphogenesis. Hepatology. 2011 Jun; 53(6):1959-66.
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