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


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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, Lung, Bone marrow, 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
Cystic kidney disease in a patient with systemic toxicity from long-term D-penicillamine use. (23796907)
2013
2
Renal graft outcome in autosomal dominant medullary cystic kidney disease type 1. (23475468)
2013
3
Microtubule modifications and stability are altered by cilia perturbation and in cystic kidney disease. (23124988)
2013
4
Cystic lymphangioma of the kidney: Diagnosis and management. (22963798)
2012
5
Bilateral multiple cystic kidney disease and renal cortical abscess in a Boerboel. (22135926)
2011
6
Clear cell papillary renal cell carcinoma and clear cell renal cell carcinoma arising in acquired cystic disease of the kidney: an immunohistochemical and genetic study. (20952286)
2011
7
Cystic kidney disease: the role of Wnt signaling. (20576469)
2010
8
Segmental cystic kidney tumours in children. (19968582)
2009
9
Adult cystic nephroma and mixed epithelial and stromal tumor of the kidney: clinical, radiographic, and pathologic characteristics. (18313107)
2008
10
Clinical manifestations of hereditary cystic kidney disease. (18508505)
2008
11
Chromophobe renal cell carcinoma and 'capsulomas' with acquired cystic disease of the kidney in a long-term hemodialysis patient. (18489645)
2008
12
Editorial comment on: cystic nephroma and mixed epithelial and stromal tumour of the kidney: opposite ends of the spectrum of the same entity? (18006142)
2008
13
Medullary cystic kidney disease type 1: mutational analysis in 37 genes based on haplotype sharing. (16738948)
2006
14
Fine needle aspiration cytology of cystic partially differentiated nephroblastoma of the kidney. (16719857)
2006
15
Cilia and centrosomes: a unifying pathogenic concept for cystic kidney disease? (16341073)
2005
16
Mating worms and the cystic kidney: Caenorhabditis elegans as a model for renal disease. (15947985)
2005
17
Medullary cystic kidney disease type 1 in a large Native-American kindred. (15384011)
2004
18
A genetic screen in zebrafish identifies cilia genes as a principal cause of cystic kidney. (15269167)
2004
19
Cystic embryonal sarcoma of the kidney. (12394750)
2002
20
The C. elegans homolog of the murine cystic kidney disease gene Tg737 functions in a ciliogenic pathway and is disrupted in osm-5 mutant worms. (11290289)
2001
21
Molecular analysis of uromodulin and SAH genes, positional candidates for autosomal dominant medullary cystic kidney disease linked to 16p12. (11730273)
2001
22
Synchronous occurrence of pleuropulmonary blastoma and cystic nephroma: possible genetic link in cystic lesions of the lung and the kidney. (10881016)
2000
23
CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease. (11095651)
2000
24
Association of cystic nephroma with angiomyolipoma in the same kidney. (10982023)
2000
25
Renal cell carcinoma in children with diffuse cystic hyperplasia of the kidneys. (10191351)
1999
26
Cystic kidney disease presenting in infancy. (10406337)
1999
27
Cellular pathophysiology of cystic kidney disease: insight into future therapies. (10535324)
1999
28
Cystic fibrosis transmembrane conductance regulator in the kidney: clues to its role? (10450015)
1999
29
Plasma erythropoietin level and iron reserves in haemodialysis patients with and without acquired cystic kidney disease. (9203047)
1997
30
Oral-facial-digital syndrome type I: an unusual cause of hereditary cystic kidney disease. (9198060)
1997
31
Late occurrence of cysts in autosomal dominant medullary cystic kidney disease. (9198059)
1997
32
Transcatheter embolization for retroperitoneal hemorrhage secondary to acquired cystic kidney disease. (9007814)
1996
33
Autosomal dominant primary hyperparathyroidism and jaw tumor syndrome associated with renal hamartomas and cystic kidney disease: linkage to 1q21-q32 and loss of the wild type allele in renal hamartomas. (8954016)
1996
34
Renal cortical neoplasm in a child with dialysis-acquired cystic kidney disease. (8607247)
1996
35
The PAX2 tanscription factor is expressed in cystic and hyperproliferative dysplastic epithelia in human kidney malformations. (8755656)
1996
36
Nephroblastoma and end-stage renal failure with bilateral cystic kidneys. (7794714)
1995
37
Solitary multilocular cyst of kidney (cystic nephroma). (7558107)
1995
38
Acquired cystic kidney disease in renal insufficiency: a multicentre study. Group of Nephrologists of the East of France. (8258045)
1993
39
Cerebro-reno-digital (Meckel-like) syndrome with Dandy-Walker malformation, cystic kidneys, hepatic fibrosis, and polydactyly. (8368252)
1993
40
Association of cyclosporin A with acquired cystic kidney disease of the native kidneys in renal transplant recipients. (8231035)
1993
41
Renal malignancy in peritoneal dialysis patients with acquired cystic kidney disease. (1361772)
1992
42
Dandy-Walker malformation (variant), cystic dysplastic kidneys, and hepatic fibrosis: a distinct entity or Meckel syndrome? (1867280)
1991
43
Nephronophthisis (medullary cystic disease of the kidney) with proteinuria and hypertension--an atypical presentation of a rare kidney disease. (2486778)
1989
44
Multilocular cyst of the kidney (cystic nephroma) and cystic, partially differentiated nephroblastoma. Terminology and criteria for diagnosis. (2544253)
1989
45
Cystic kidneys in a patient with oral-facial-digital syndrome type I. (7072712)
1982
46
Substitute ileal ureter in an infant with congenital hydronephrosis of the left kidney and congenital cystic hypoplasia of the right kidney. (13764561)
1961
47
A Case of Congenital Cystic Kidney, Associated with Several Other Abnormalities. (17104076)
1925
48
CYSTIC DISEASE OF KIDNEYS WITH PYURIA AND HAEMORRHAGES. (20764124)
1908
49
CONGENITAL CYSTIC KIDNEY AND LIVER WITH FAMILY TENDENCY. (19867040)
1906
50
VIII. Contribution to the Surgery of True Cystic Kidney. (17861063)
1901

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:00213310.4PKD1, PKD2
2primary ciliumGO:07237210.4PKHD1, NPHP3, UMOD, NEK8
3mitotic spindleGO:07268610.4PKD2, PKHD1
4ciliumGO:00592910.3NPHP3, NPHP4, PKD2, PKD1, INVS
5microtubule basal bodyGO:00593210.3B9D2, PKD2, PKHD1, NPHP4
6motile primary ciliumGO:03151210.3PKD1, PKD2
7membraneGO:01602010.1B9D2, NPHP1, NPHP4, EDNRA, INVS, TSC2
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:00496210.3EDNRB, EDNRA
2protein bindingGO:0055159.2HNF1B, B9D2, NPHP1, NPHP4, NPHP3, EDNRB

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