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Orphanet Rare Disease Ontology
Last uploaded:
July 3, 2024
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| Preferred Name | Multicystic dysplastic kidney | |
| Synonyms |
MCDK Multicystic renal dysplasia |
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| Definitions |
A rare congenital anomaly of the kidney and urinary tract (CAKUT) in which one or both kidneys (unilateral or bilateral MCDK respectively) are large, distended by multiple cysts, and non-functional. Unilateral MCDK is typically asymptomatic if the other kidney is fully functional but may occasionally present with abdominal obstructive signs when the cysts become too large. Bilateral MCDK is considered a lethal entity and neonates present with features of the Potter sequence, severe pulmonary hypoplasia and severe renal failure, and generally die shortly after birth. |
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| ID |
http://www.orpha.net/ORDO/Orphanet_1851 |
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| alternative_term |
MCDK Multicystic renal dysplasia
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| definition |
A rare congenital anomaly of the kidney and urinary tract (CAKUT) in which one or both kidneys (unilateral or bilateral MCDK respectively) are large, distended by multiple cysts, and non-functional. Unilateral MCDK is typically asymptomatic if the other kidney is fully functional but may occasionally present with abdominal obstructive signs when the cysts become too large. Bilateral MCDK is considered a lethal entity and neonates present with features of the Potter sequence, severe pulmonary hypoplasia and severe renal failure, and generally die shortly after birth.
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| definition_citation |
Orphanet
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| expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=1851 |
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| has_age_of_onset |
Infancy Neonatal
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| has_inheritance |
Not applicable
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| hasDbXref |
ICD-10:Q61.4 UMLS:C3714581 ICD-11:LB30.9 MeSH:D021782
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| label |
Multicystic dysplastic kidney
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| notation |
ORPHA:1851
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| part_of | ||
| prefixIRI |
ORDO:Orphanet_1851
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| prefLabel |
Multicystic dysplastic kidney
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| present_in |
Worldwide AND has_birth_prevalence_average_value : 23.26 AND has_birth_prevalence_range : 1-5 / 10 000 Worldwide AND has_point_prevalence_range : Unknown
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| treeView | ||
| subClassOf |
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