Congenital Diseases Flashcards
What is the most common congenital renal anomaly?
Horseshoe kidney
Where is a horseshoe kidney typically found and why?
Located in lower abdomen because gets caught on inferior mesenteric artery during its ascent from pelvis to abdomen
What are the consequences of unilateral renal agenesis?
- Hypertrophy of existing kidney
2. Increased risk of renal failure later in life bc of hyperfiltration
What are the consequences of bilateral renal agenesis?
Oligohydramnios = decreased amniotic fluid –> Potter Sequence: Pulmonary hypoplasia, Oligohydramnios, Twisted face, Twisted skin, Extremity defects, Renal failure (in utero)
*Incompatible with life
What would a dysplastic kidney look like on biopsy? How is it attained?
NONINHERITED but CONGENITAL malformation of renal parenchyma:
Cysts & abnormal tissue (cartilage)
*Usually unilateral
In Polycystic Kidney Disease, what are the sizes of the kidneys? Where are the cysts located?
Bilateral enlarged kidneys + cysts in renal cortex and medulla
What are the two inherited forms of Polycystic Kidney Disease?
(1) Autosomal Recessive
(2) Autosomal Dominant
Which population does the autosomal recessive type of Polycystic Kidney Disease show up in? What is it associated with?
Infants: worsening renal failure & HTN
Newborns: Potter sequence
Associated w/ congenital hepatic fibrosis (portal HTN) + hepatic cysts
*Cysts in kidney, liver
Which population does the autosomal dominant type of Polycystic Kidney Disease show up in? What is it associated with?
Young adults: HTN (increased renin), hematuria, pain, UTIs, worsening renal failure
Associated w/ hepatic cysts, berry aneurysm, mitral valve prolapse
*Cysts in kidney, liver, brain
How do you treat adult dominant PKD?
Supportive treatment!
- Control HTN
- Restrict protein
- Cyst drainage
What are the sizes of the kidneys in medullary cystic kidney disease?
Shrunken kidneys because of parenchymal fibrosis –> worsening renal failure
What is the inheritance pattern of medullary cystic kidney disease?
Autosomal dominant
Horseshoe kidney is associated with which disease?
Turner syndrome
