Biomaterial Database

Predictors of progression in autosomal dominant and autosomal recessive polycystic kidney disease. 2021

Eric G Benz, and Erum A Hartung

Division of Nephrology, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA, 19104, USA.

Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are characterized by bilateral cystic kidney disease leading to progressive kidney function decline. These diseases also have distinct liver manifestations. The range of clinical presentation and severity of both ADPKD and ARPKD is much wider than was once recognized. Pediatric and adult nephrologists are likely to care for individuals with both diseases in their lifetimes. This article will review genetic, clinical, and imaging predictors of kidney and liver disease progression in ADPKD and ARPKD and will briefly summarize pharmacologic therapies to prevent progression.

UI	MeSH Term	Description	Entries
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D016891	Polycystic Kidney, Autosomal Dominant	Kidney disorders with autosomal dominant inheritance and characterized by multiple CYSTS in both KIDNEYS with progressive deterioration of renal function.	Autosomal Dominant Polycystic Kidney,Kidney, Polycystic, Autosomal Dominant,ADPKD,Adult Polycystic Kidney Disease,Adult Polycystic Kidney Disease Type 1,Adult Polycystic Kidney Disease Type 2,Polycystic Kidney Disease 2,Polycystic Kidney Disease, Adult,Polycystic Kidney Disease, Adult Type 2,Polycystic Kidney Disease, Adult, Type II,Polycystic Kidney Disease, Autosomal Dominant,Polycystic Kidney Disease, Type 2,Polycystic Kidney, Type 1 Autosomal Dominant Disease,Polycystic Kidney, Type 2 Autosomal Dominant Disease
D017044	Polycystic Kidney, Autosomal Recessive	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.	Autosomal Recessive Polycystic Kidney,Autosomal Recessive Polycystic Kidney Disease,Kidney, Polycystic, Autosomal Recessive,Polycystic Kidney Disease, Infantile, Type I,ARPKD,Polycystic Kidney Disease, Autosomal Recessive,Polycystic Kidney Disease, Infantile, Type 1,Polycystic Kidney and Hepatic Disease 1,Polycystic Kidney and Hepatic Disease 1 (Autosomal Recessive)
D018450	Disease Progression	The worsening and general progression of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.	Clinical Course,Clinical Progression,Disease Exacerbation,Exacerbation, Disease,Progression, Clinical,Progression, Disease