Urinary Tract Congenital Anomalies, Cystic Disease, Urolithiasis and Obstructive Diseases Flashcards
What percentage of renal failure in children is caused by congenital kidney malformations?
20%
What percentage of people are born with potentially significant malformation of the kidney/urinary tract?
10%
What percentage of chronic renal failure is due to ADPKD?
10%
What are the types of malformation of the kidneys?
Agenesis of the kidney
Hypoplasia
Ectopic kidneys Horseshoe kidneys
What is kidney hypoplasia?
Formed kidneys but very small
What is ectopic kidneys?
Kidneys that are misplaced
What is horseshoe kidney?
malformed kidneys that join together and never separate forming a horseshoe shape
What causes agenesis of the kidney?
Major disruption of metanephric development at an early stage
What is potter’s syndrome?
Bilateral agenesis of kidneys.
What happens to babies without kidneys?
They develop without much amniotic fluid around it. This condition is called oligohydramnios.
They also get hypoplastic lungs if there is not enough amniotic fluid to breath in for them to open
What does potter’s facies refer to?
A particular appearance associated with low exposure to amniotic fluid.
What does potter’s facies look like?
Large low set ears
Wide set eyes
Flattened nose
Receding chin
Prominent skin fold below the eyes
What does unilateral agenesis do to foetus?
Usually asymptomatic
What happens to the present kidney in unilateral agenesis?
It undergoes compensatory hypertrophy.
What happens to the ureter of the abscent kidney?
It disappears completely
What causes ectopic kidneys?
Kidneys usually ascend during development to their position and end up staying around the pelvis.
What are the cystic diseases of the kidney?
Cystic renal disease
Polycystic kidney disease (autosomal dominant PKD and autosomal recessive PKD)
What are simple renal cysts?
Very common in normal kidneys especially people older than 50 years old.
Usually they cause no symptoms or signs
Occasionally they can rupture, cause haematuria, pain, abdominal mass, infection, and hypertension.