Congenital Renal Pathology Flashcards
What is the most common renal congenital anomaly?
Horseshoe Kidney.
What is horseshoe kidney?
Conjoined kidneys usually connected at the lower pole
Where are kidney’s located in Horseshoe kidney?
In the lower abdomen; horseshoe kidney gets caught on the inferior mesenteric artery root during its ascent from the pelvis to the abdomen
What is Renal Agenesis?
Absent kidney formation, may be unilateral or bilateral.
What are the consequences of unilateral renal agenesis?
- Hypertrophy of the existing kidney
2. Hyperfiltration increases risk of renal failure later in life.
What does Bilateral renal agenesis lead to?
- Oligohydramnios with lung hypoplasia
- Flat Face with low set ears
- Developmental defects of the extremities Incompatible with LIFE
What is dysplastic Kidney?
Noninherited congenital malformation of the renal parenchyma characterized by cysts and abnormal tissue (e.g cartilage)
How is Dysplastic Kidney normally seen?
Usually unilateral
What is (PKD) Polycystic Kidney Disease?
Inherited defect leading to bilateral enlarged kidneys with cysts in the renal cortex and medulla
How is PKD inherited?
There are two forms
- Autosomal recessive
- Autosomal Dominant
How / when does the Autosomal recessive form present?
Presents in infants as worsening renal failure and hypertension; newborns may present with Potter’s sequence.
What is one of the primary associations with Autosomal recessive PKD
Associated with congenital hepatic fibrosis (leads to portal HTN) and hepatic cysts
How / when does the Autosomal dominant form present?
Presents in young adults as hypertension (due to increased renin), hematuria, and worsening renal failure.
What is the underlying cause of AD form of PKD?
Due to mutation in the APKD1 or APKD2 gene
What develops overtime with AD PKD?
Cysts!
