MCID: CYS016
MIFTS: 56

Cystic Kidney malady

Nephrological diseases category

Summaries for Cystic Kidney

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33MedlinePlus, 63Wikipedia, 32MalaCards
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MedlinePlus:33 A cyst is a fluid-filled sac. there are two types of kidney cysts. polycystic kidney disease (pkd) runs in families. in pkd, the cysts take the place of the normal tissue. they enlarge the kidneys and make them work poorly, leading to kidney failure. when pkd causes kidneys to fail - which usually happens after many years - people need dialysis or kidney transplantation. about half of people with the most common type of pkd end up with kidney failure. pkd also causes cysts in other parts of the body, such as the liver. symptoms of pkd include pain in the back and lower sides headaches urinary tract infections blood in the urine doctors diagnose pkd with imaging tests and family history. treatments include medications, and, when people with pkd develop kidney failure, dialysis or kidney transplants. acquired cystic kidney disease (ackd) usually happens in people who are on dialysis. unlike pkd, the kidneys are normal sized, and cysts do not form in other parts of the body. people with ackd already have chronic kidney disease when they develop cysts. ackd often has no symptoms. in most cases, the cysts are harmless and do not need treatment. nih: national institute of diabetes and digestive and kidney diseases

MalaCards: Cystic Kidney, also known as cystic kidney disease, is related to nephronophthisis and umod-associated kidney disease. An important gene associated with Cystic Kidney is UMOD (uromodulin), and among its related pathways are Selected targets of HNF1 and Endothelins. The compounds trp-p-2 and [125i]et-1 have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver and testes, and related mouse phenotypes are liver/biliary system and embryogenesis.

Wikipedia:63 Cystic kidney disease refers to a wide range of hereditary, developmental, and acquired conditions. With... more...

Aliases & Classifications for Cystic Kidney

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8Disease Ontology, 10DISEASES, 60UMLS, 44Novoseek, 33MedlinePlus, 34MeSH, 39NCIt, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Nephrological diseases


Aliases & Descriptions:

cystic kidney 8 10 60
cystic kidney disease 8 44
renal cyst 8 44
congenital cystic kidney disease 60
cystic disease of kidney 8
kidney cysts 33
kidney cyst 8


External Ids:

Disease Ontology8 DOID:2975
MeSH34 D052177
NCIt39 C3970
ICD1025 Q61

Related Diseases for Cystic Kidney

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17GeneCards, 18GeneDecks
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Diseases in the Renal Dysplasia, Cystic family:

cystic kidney

Diseases related to Cystic Kidney via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 159)
idRelated DiseaseScoreTop Affiliating Genes
1nephronophthisis30.9PKD1, UMOD, PKD2
2umod-associated kidney disease30.7UMOD, MCKD2
3polycystic kidney disease30.4PKD1, PKHD1
4kidney cancer30.4HNF1B
5uromodulin-associated kidney disease30.3UMOD, MCKD2
6polycystic kidney disease, autosomal dominant30.1PKD1, PKD2
7familial juvenile hyperuricemic nephropathy30.1UMOD, HNF1B
8polycystic kidney disease, autosomal recessive30.1PKD2, TSC2, PKD1, HNF1B, PKHD1
9polydactyly30.1B9D2
10tuberous sclerosis30.0PKD1, TSC2, PKD1P6, PKHD1
11renal dysplasia29.8HNF1B
12retinal degeneration29.8NPHP1
13hepatitis29.8EDNRA
14situs inversus29.8PKD2, INVS, NPHP1, NPHP3
15end stage renal failure29.8NPHP4
16hyperuricemia29.8UMOD, HNF1B
17gout29.8UMOD, HNF1B
18chronic kidney failure29.8PKD1, INVS, UMOD, NPHP1, HNF1B, TSC2
19cystic fibrosis29.8UMOD, PKD1
20polycystic liver disease29.8PKD1P6, PKD1, PKD2, PKHD1
21joubert syndrome29.8NPHP1
22glomerulocystic kidney disease with hyperuricemia and isosthenuria29.8UMOD, MCKD2
23intracranial aneurysm29.8PKD2, PKD1
24kidney disease11.0
25medullary cystic kidney disease type 110.7
26autosomal dominant disease10.5
27renal cell carcinoma10.5
28peritonitis10.4
29renal cysts and diabetes syndrome10.3
30chromosomal disease10.2
31nephronophthisis 1, juvenile10.2
32autosomal dominant medullary cystic kidney disease with hyperuricemia10.2
33infantile autosomal recessive medullary cystic kidney disease10.2
34juvenile autosomal recessive medullary cystic kidney disease10.2
35late-onset autosomal recessive medullary cystic kidney disease10.2
36nephrocalcinosis10.2
37hepatic fibrosis - renal cysts - intellectual deficit10.2
38joubert syndrome 210.2
39daneman davy mancer syndrome10.2
40meckel syndrome 110.2
41renal hypertension10.1
42hepatic fibrosis renal cysts mental retardation10.1
43multiloculated renal cyst10.1
44pyuria10.1
45urethritis10.1
46hajdu-cheney syndrome10.1
47papillary carcinoma10.1
48childhood multilocular cystic kidney neoplasm10.1
49autosomal recessive disease10.1
50hemorrhagic disease10.1

Graphical network of the top 20 diseases related to Cystic Kidney:



Diseases related to cystic kidney

Clinical Features for Cystic Kidney

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Drugs & Therapeutics for Cystic Kidney

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Cystic Kidney

Drug clinical trials:

Search ClinicalTrials for Cystic Kidney

Search NIH Clinical Center for Cystic Kidney

Search CenterWatch for Cystic Kidney

Genetic Tests for Cystic Kidney

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Anatomical Context for Cystic Kidney

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32MalaCards
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MalaCards organs/tissues related to Cystic Kidney:

32
Kidney, Liver, Testes, Pancreas, Bone marrow, Bone, Lung, Breast

Animal Models for Cystic Kidney or affiliated genes

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36MGI
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Publications for Cystic Kidney

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50PubMed
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Articles related to Cystic Kidney:

(show top 50)    (show all 293)
idTitleAuthorsYear
1
Survivin-Induced Abnormal Ploidy Contributes to Cystic Kidney and Aneurysm Formation. (24235270)
2013
2
Pilot study of non-contrast-enhanced MRI vs. ultrasound in renal transplant recipients with acquired cystic kidney disease: a prospective intra-individual comparison. (24118352)
2013
3
Unilateral localized cystic kidney. (20730454)
2011
4
Pioglitazone Attenuates Cystic Burden in the PCK Rodent Model of Polycystic Kidney Disease. (21052534)
2010
5
Cystic nephroma masquerading as hydatid cyst of the kidney. (21045463)
2010
6
Normal ciliogenesis requires synergy between the cystic kidney disease genes MKS-3 and NPHP-4. (20150540)
2010
7
New syndrome--situs inversus totalis with cystic dysplasia of kidneys, pancreas and bowing. (20530999)
2010
8
Autosomal dominant polycystic kidney disease with cystic haemorrhage and infection. (21268963)
2010
9
Long-term online hemodiafiltration does not reduce the frequency and severity of acquired cystic kidney disease in hemodialysis patients. (19839850)
2009
10
Lethal cystic kidney disease in Amish neonates associated with homozygous nonsense mutation of NPHP3. (19303681)
2009
11
Impaired Wnt-beta-catenin signaling disrupts adult renal homeostasis and leads to cystic kidney ciliopathy. (19718039)
2009
12
Inactivation of Pkd1 in principal cells causes a more severe cystic kidney disease than in intercalated cells. (19145237)
2009
13
Cystic kidney gene seahorse regulates cilia-mediated processes and Wnt pathways. (18539122)
2008
14
Adenoid cystic carcinoma metastatic to the kidney: a case report. (18499998)
2008
15
Histogenesis of the acquired cystic kidney disease: an immunohistochemical study. (16932028)
2006
16
Tamm-Horsfall protein knockout mice do not develop medullary cystic kidney disease. (16688192)
2006
17
Cystic angiomyolipoma of the kidney: a clinicopathologic description of 11 cases. (16528375)
2006
18
Bilateral laparoscopic radical nephrectomy for renal tumors in patients with acquired cystic kidney disease. (16366867)
2005
19
C-jun activation in acquired cystic kidney disease and renal cell carcinoma. (16006965)
2005
20
Ischiospinal dysostosis with cystic kidney disease: report of two cases. (12868471)
2003
21
Zebrafish pronephros: a model for understanding cystic kidney disease. (14579389)
2003
22
Dialysis-associated acquired cystic kidney disease imitating autosomal dominant polycystic kidney disease in a patient receiving long-term peritoneal dialysis. (11865101)
2002
23
Autosomal-dominant medullary cystic kidney disease type 1: clinical and molecular findings in six large Cypriot families. (12234310)
2002
24
Comparison of acquired cystic kidney disease between hemodialysis and continuous ambulatory peritoneal dialysis. (10714092)
2000
25
Acquired cystic kidney disease in ESRD. (10838969)
1999
26
The diagnostic dilemma of the unilateral cystic kidney-ADPKD with aplasia of one kidney. (10534805)
1999
27
Coordinate expression of the autosomal dominant polycystic kidney disease proteins, polycystin-2 and polycystin-1, in normal and cystic tissue. (10362797)
1999
28
Medullary cystic kidney disease with hyperuricemia and gout in a large Cypriot family: no allelism with nephronophthisis type 1. (9605289)
1998
29
Chromosome 1 localization of a gene for autosomal dominant medullary cystic kidney disease. (9536096)
1998
30
Recent Advances in Investigation of Cystic Kidney Disease. Proceedings of a conference. Heidelberg, Germany, 27 September 1995. (9148234)
1996
31
Acquired cystic kidney disease. (8784391)
1996
32
Unravelling the pathogenesis of cystic kidney diseases. (7702367)
1995
33
Cystic kidney disease in Hajdu-Cheney syndrome. (7747781)
1995
34
An unusual cystic lesion histologically similar to autosomal dominant polycystic kidney disease in a child with aniridia and del 11p13. (8072106)
1994
35
Renal cancer complicating acquired cystic kidney disease. (7919148)
1994
36
Renal cancer complicating acquired cystic kidney disease. (7894008)
1994
37
Acquired cystic kidney disease before and after renal transplantation. (1625094)
1992
38
Acquired cystic kidney disease. (1921151)
1991
39
Sonographic, clinical and genetic aspects of prenatal diagnosis of cystic kidney disease. (1781072)
1991
40
Acquired cystic kidney disease in renal insufficiency. (1868477)
1991
41
Metastatic renal cell carcinoma associated with acquired cystic kidney disease 15 years after successful renal transplantation. (1962659)
1991
42
Anemia in dialysis: its relation to acquired cystic kidney disease and serum levels of erythropoietin. (2048573)
1991
43
Robinow syndrome: report of two patients with cystic kidney disease. (2200625)
1990
44
Case report of a primary cystic sarcoma of the kidney, demonstrating fibrohistiocytic, osteoid, and cartilaginous components (malignant mesenchymoma). (7127261)
1982
45
Cystic kidney in an 8-week human embryo. (7148379)
1982
46
Genetics in cystic kidney disease. (1264570)
1976
47
Multilocular cystic dysplasia of half of a horseshoe kidney. (4844002)
1974
48
Congenital unilateral cystic kidney: report of two cases. (13567631)
1956-1957
49
CONGENITAL CYSTIC KIDNEY WITH LOCAL DIFFUSE PERITONITIS: SURGICAL DESTRUCTION OF PART OF KIDNEY: RECOVERY. (20768182)
1916
50
Case of Cystic Disease of the Kidney simulating Ovarian Disease. (20896314)
1868

Genetic Variations for Cystic Kidney

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Expression for genes affiliated with Cystic Kidney

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Cystic Kidney

Search GEO for disease gene expression data for Cystic Kidney.

Pathways for genes affiliated with Cystic Kidney

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12EMD Millipore, 37NCBI BioSystems Database
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Pathways related to Cystic Kidney according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
110.3PKD2, UMOD, HNF1B
210.2BCAR1, EDNRA, EDNRB

Compounds for genes affiliated with Cystic Kidney

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44Novoseek, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB
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Compounds related to Cystic Kidney according to GeneCards/GeneDecks:

(show all 22)
idCompoundScoreTop Affiliating Genes
1trp-p-24410.7PKD1, PKD2
2[125i]et-12810.7EDNRA, EDNRB
3pd 1450654410.7EDNRB, EDNRA
4sitaxsentan44 2811.7EDNRB, EDNRA
5pd 1428934410.7EDNRB, EDNRA
6bq6104410.6EDNRB, EDNRA
7bq 302044 2811.6EDNRB, EDNRA
8ambrisentan44 2811.6EDNRB, EDNRA
9irl 162044 2811.6EDNRA, EDNRB
10tezosentan4410.6EDNRA, EDNRB
11Sitaxentan1110.6EDNRA, EDNRB
12fr13931744 2811.6EDNRB, EDNRA
13sarafotoxin4410.6EDNRB, EDNRA
14atrasentan4410.5EDNRB, EDNRA
15darusentan44 2811.5EDNRB, EDNRA
16bq78844 2811.5EDNRB, EDNRA
17bq12344 2811.4EDNRA, EDNRB
18toxin a4410.3EDNRB, EDNRA
19sodium44 2411.3PKD1, PKD2, EDNRA, EDNRB, UMOD
20bosentan44 1111.2EDNRA, EDNRB
21phosphatidylinositol4410.1BCAR1, TSC2, PKD1, EDNRA, EDNRB
22calcium44 49 11 2412.7UMOD, EDNRB, EDNRA, PKHD1, PKD2, PKD1

GO Terms for genes affiliated with Cystic Kidney

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16Gene Ontology
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Cellular components related to Cystic Kidney according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1polycystin complexGO:00213310.4PKD2, PKD1
2primary ciliumGO:07237210.4PKHD1, NEK8, UMOD, NPHP3
3mitotic spindleGO:07268610.4PKD2, PKHD1
4ciliumGO:00592910.3NPHP4, NPHP3, PKD2, PKD1, INVS
5microtubule basal bodyGO:00593210.3PKD2, PKHD1, NPHP4, B9D2
6motile primary ciliumGO:03151210.3PKD2, PKD1
7membraneGO:01602010.1TSC2, INVS, EDNRA, NPHP4, NPHP1, B9D2
8cell-cell junctionGO:00591110.0PKD2, NPHP4, NPHP1

Biological processes related to Cystic Kidney according to GeneCards/GeneDecks:

(show all 22)
idNameGO IDScoreTop Affiliating Genes
1mesonephric tubule developmentGO:07216410.7PKD2, PKD1
2regulation of cAMP metabolic processGO:03081410.7PKD2, NPHP3
3visual behaviorGO:00763210.7NPHP1, NPHP4
4mesonephric duct developmentGO:07217710.7PKD1, PKD2
5metanephric ascending thin limb developmentGO:07221810.7UMOD, PKD2, PKD1
6determination of liver left/right asymmetryGO:07191010.7NPHP3, PKD2
7genitalia developmentGO:04880610.6PKD1, HNF1B
8positive regulation of cyclin-dependent protein serine/threonine kinase activity involved in G1/S transition of mitotic cell cycGO:03165910.6PKD2, PKD1
9placenta blood vessel developmentGO:06067410.6PKD1, PKD2
10regulation of epithelial cell proliferationGO:05067810.6EDNRB, EDNRA
11heart developmentGO:00750710.6TSC2, PKD1, PKD2, EDNRA
12detection of mechanical stimulusGO:05098210.6PKD2, PKD1
13cytoplasmic sequestering of transcription factorGO:04299410.5PKD1, PKD2
14kidney developmentGO:00182210.5INVS, PKD1, PKHD1, NPHP3, HNF1B
15cell-cell adhesionGO:01633710.5PKHD1, NPHP4, NPHP1
16vasoconstrictionGO:04231010.5EDNRA, EDNRB
17spinal cord developmentGO:02151010.5PKD2, PKD1
18enteric nervous system developmentGO:04848410.4EDNRA, EDNRB
19embryonic placenta developmentGO:00189210.4PKD1, PKD2
20cell cycle arrestGO:00705010.3TSC2, PKD1, PKD2
21calcium ion transmembrane transportGO:07058810.3PKD2, PKD1
22neural tube developmentGO:02191510.1PKD2, PKD1

Molecular functions related to Cystic Kidney according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1endothelin receptor activityGO:00496210.3EDNRA, EDNRB
2protein bindingGO:0055159.2BCAR1, HNF1B, B9D2, NPHP1, NPHP4, NPHP3

Products for genes affiliated with Cystic Kidney

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Sources for Cystic Kidney

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet