Congenital Kidney Pathology - Pathoma Flashcards
What is the most common congenital renal anomaly?
Horseshoe Kidney
What is a horseshoe kidney?
Conjoined kidneys
-usually at lower pole
Where are horseshoe kidneys located? Why is the location important?
- Lower abdomen
- Importance:
- develops in pelvis => ascends into abdomen
- gets stuck on root of inferior mesentary artery => blocks ascension
- Importance:
What is renal agenesis?
Absence of kidney formation.
-may be unilateral or bilateral
What complication arises in Unilateral Renal Agenesis?
- patient lacking one kidney
- consequence:
- existing kidney undergoes hypertrophy
- problem later in life when hyperfiltration leads to greater risk of renal failure
- consequence:
What does oligohydramnios mean?
- Deficiency in amniotic fluid
- no kidneys in fetus => no amniotic fluid (urine)
What are the complications in bilateral renal agenesis?
- Lung hypoplasia
- no amniotic fluid to stretch lungs
- Flat face with low set ears
- face gets pressed up against wall of uterus with no amniotic fluid
- Developmental defects of the extremeties
***Sequence called POTTER SEQUENCE
(incompatible with life)
What is Dysplastic Kidney?
-
Non-inherited congenital malformation of renal parenchyma
- Characterized by:
- cysts
- abnormal tissue (e.g. cartilage)
- Characterized by:
Is Dysplastic Kidney usually unilateral or bilateral?
Usually unilateral!
-when bilateral => must be distinguished from inherited Polycystic Kidney Disease
How is Polycystic Kidney Disease different from Dysplastic Kidney?
- Inherited defect
-
Always bilateral
- enlarged kidneys
- cysts in BOTH the renal cortex and medulla
What are the two subtypes of Polycystic Kidney Disease?
- Autosomal Recessive
- Autosomal Dominant
What patient population does Autosomal Recessive Polycystic Kidney Disease usually present in?
- Presents in infants
- used to be called Juvenille PKD
How does Autosomal Recessive PKD present?
- HTN
- Worsening renal failure
- cysts in kidneys
- Newborns may present with Potter Sequence
- as if baby had oligohydramninos such as bilateral agenesis
- kidneys not producing much amniotic fluid
- Associated with congenital hepatic fibrosis and hepatic cysts
- kidneys in liver => portal hypertension
How does Autosomal Domninant PKD usually present?
- Presents in young adults
- cysts develop over time
- HTN (due to increased renin)
- increased plasma renin
- Hematuria
- Worsening renal failure
***Mnemonic: ADult
A mutation in which gene causes Autosomal Dominant PKD?
APKD1 or APKD2
