Congenital Kidney Quiz Flashcards
A rare kidney diseases characterized by tubular damage and interstitial fibrosis.
SHRUNKEN Kidneys
Medullary Cystic Kidney
AD
Which 2 congenital renal diseases are inherited?
PKD
Medullary Cystic Kidney
Which congenital renal disease is associated with Potter Syndrome
AR-PKD
Bilateral Renal Agenesis
Which congenital renal disease has lower fused poles?
Horeshoe Kidney
Which congenital renal disease causes kidney Hypertrophy/Hypersecretion?
Unilateral Renal Agenesis?
What 3 associations are found in ADPKD?
BERRY ANEURYSM
Mitral valve prolapse
Hepatic/Cystic Fibrosis
What two gene mutations lead to AD-PKD?
PKD1
PKD2
Which congenital renal disease has Kidney fibrosis?
Medullary Cystic Kidney
List two Autosomal Dominant congenital renal diseases?
AD-PKD
Medullary Cystic Kidney
Which congenital renal disease has cartilage (or abnormal tissue) in the kidneys?
Dysplastic Kidney disease
non-inherited
Which 2 congenital renal diseases lead to HTN?
AR-PKD
AD- PKD
Young adult with hematuria, flank pain, recurrent UTIs, and/or kidney stones
Possible h/o or FHx of:
murmur (MVP)
Hepatic problems due to cysts
Enlarged Kidneys Bilaterally
AD–Polycystic Kidney Disease
Horseshoe kidney gets caught where?
IMA
Increased Renin
Hematuria
young adult
AD-PKD
Portal HTN → ascities, paraumbilical/epigastric veins dilated, esophageal varices, Hepato and/or splenomegaly
Hepatic Fibrosis → anechoic cysts/spots on US, multiple cysts
Bilaterally enlarged cystic kidneys → proteinuria, hematuria, or oligouria
PULMONARY HYPOPLASIA → respiratory distress
AR-PKD
oligohydraminos
lung hypoplasia
flat face
low ears
Bilateral Renal Agenesis
AR-PKD
PKHD1 codes for
Fibrocystin (in kidney and liver)
*AR-PKD
Clinical findings associated with
Cysts in the medullary collecting tube
Medullary cystic kidney
Uretic bud fails to induce differentiation of metanephric mesenchyme
Consists of cyst and connective tissue
Unilateral/bilateral
POTTERS
Dysplastic Kidney (Multicystic)
Associated with Hydronephrosis Renal stones Turner syndrome Trisonomies 13, 18, 21
Horseshoe Kidney
↑ Risk of Renal Cancer
Horseshoe Kidney
Posterior urethral valves only seen in
MALES
never females
Bilateral hydronephrosis
THICK or DILATED wall bladder on US
Bladder outlet obstruction MOST COMMON
(+/- oligohydraminos in fetus)
Posterior urethral valves
Reccurent UTIs in a young male with a thick bladder wall
Posterior urethral valves
Bifurcation of one uretic bud or 2 uretic buds connecting to blastema.
Y shaped bi-fid ureter
Duplex collecting system
*causes vesicuroectal reflux & Pyelonephritis
Highly associated with vesicoureteral reflux
uretera, obstruction, ↑ risk of UTIs
Duplex collecting system
Little girl with reccurrent UTIs and flank pain
vesicoureteral reflux
POTTER is associated with
AR-PKD &
Dysplastic Kidney disease
It stands for
Pulmonary hypoplasia Oligohydraminos Twisted face Twisted skin (Low ears, Flat face) Extremity defects Renal failure
Inadequate canalization of the ___ is the most common cause of unilateral fetal hydronephrosis.
ureteropelvic junction
the connection site between the kidney and the ureter,
