Congenital Flashcards Preview

Pathoma - Kidney and Urinary Tract Pathology > Congenital > Flashcards

Flashcards in Congenital Deck (21):
1

What is a Horseshoe Kidney?

Conjoined kidneys usually connected at the lower pole

2

What is the most common congenital renal anomaly?

Horseshoe Kidney

3

Where are Horseshoe kidney's located? What do they get caught on?

-Kidney is abnormally located in lower abdomen
-Get caught on the inferior mesenteric artery root during ascent from the pelvis to the abdomen

4

What is Renal Agenesis?

Absent kidney formation
-May be unilateral or bilateral

5

What does Unilateral Renal Agenesis cause?

-Hypertrophy of the existing kidney
-Hyperfiltration (doing the work of two kidneys) increases risk of renal failure later in life

6

What does Bilateral Renal Agenesis cause?

-It leads to oligohydraminos with lung hypoplasia, flat face, low set ears and developmental defects of extremities (Potter Sequence)

7

Is Bilateral Renal Agenesis compatible with life?

NO

8

What is a Dysplasic Kidney?

NONINHERITED, congenital malformation of the renal parenchyma

9

What is a dysplastic kidney characterized by?

Cysts and abnormal tissue

10

Is a Dysplastic kidney usually unilateral or bilateral?

-Usually unilateral
-When bilateral, must be distinguished from inherited polycystic kidney disease (often on exams!)

11

What is polycystic kidney disease (PKD)?

Inherited defect leading to bilateral enlarged kidneys with cysts in renal cortex and medulla

12

What are the two genetic forms of PKD?

1. Autosomal recessive
2. Autosomal dominant

13

What does autosomal recessive PKD present with? What populations is it seen in?

-Infants with worsening renal failure and hypertension
-Newborns may present with Potter sequence --> it's like they don't have any kidneys because the kidneys aren't functional

14

What pathological findings are associated with autosomal recessive PKD?

Congenital hepatic fibrosis (leads to portal hypertension _ and hepatic cysts
[So overall: cysts in liver --> fibroblasts & cysts in kidneys]

15

What does autosomal dominant PKD present with? What populations is it seen in?

-Young adults with hypertension (due to increased renin), hematuria, and worsening renal failure.

16

What features are associated with autosomal dominant PKD?

-Berry aneurysm
-Hepatic cysts
-Mitral valve prolapse

17

What mutation cases autosomal dominant PKD?

Mutation in APKD1 or APKD2 gene

18

How do the cysts develop in autosomal dominant PKD?

Over time

19

Where are the "cysts" in autosomal dominant PKD?

-Kidney
-"Brain"
-Liver

20

What is Medullary Cystic Kidney Disease?

Inherited (autosomal dominant) defect leading to cysts in the medullary collecting ducts

21

What pathology is associated with Medullary Cystic Kidney Disease?

Parenchymal fibrosis which results in shrunken kidneys and worsening renal failure.