12.1 Congenital Renal Diseases Flashcards
What is the most common renal congenital abnormality?
Horseshoe Kidney
Horseshoe Kidney
- Kidneys are conjoined as a single unit
- Abnormally located kidney in the lower abdomen
Renal Agenesis
Absence of kidney formation - can be uni/bilateral
What happens in unilateral renal agenesis?
Leads to hypertrophy of the single kidney that leads to increased chance of likelihood of renal failure later in life
What happens in bilateral renal agenesis?
Oligohydramnios - little amniotic fluid
Lung Hypoplasia - due to too little fluid to stretch out the lung’s growth
Potter Sequence - flattened face with extremity defects
-> Incompatible with life
Dysplastic Kidney Defects
Noninherited congenital defects that is characterized by cysts
Polycystic Kidney Disease
Inherited bilateral defect that will have cysts in the renal cortex and medulla
Autosomal Recessive PKD
Presents in infants with renal failure - newborns may present with Potter Sequence
What is ARPKD associated with?
Hepatic fibrosis and hepatic cysts
Autosomal Dominant PKD
Presents in young adults due to a mutation in APKD1 or 2
How do ADPKD patients present?
HTN
Hematuria
Renal Failure
What are the associations of Autosomal Dominant PKD?
- Berry Aneurysms
- Hepatic Cysts
- Mitral Valve Prolapse
Medullary Cystic Kidney Disease
Inherited disease (AD) with cysts in the medullary CDs of the kidneys that results in shrunken kidneys
