RENAL - CONGENITAL DISORDERS Flashcards
What is renal agenesis?
When the kidneys don’t form. This can be unilateral or bilateral.
What causes renal agenesis?
The ureteric bud fails to induce development of the metanephric blastema. This is thought to be a combination of genetic factors and environmental agents.
Ho does renal agenesis present?
Asymptomatic if unilateral
If bilateral it causes oligohydramnios which leads to lung hypoplasia and potters sequence
What can unilateral renal agenesis put you at risk for?
Overtime it causes hypertrophy of the kidney which can increase the risk of hypertension and renal failure
Whats the prognosis of renal agenesis?
Ulitareal can live a healthy life
Bilateral is usually fatal in the first few days but some can have dialysis until strong enough to have. A kidney transplant
What is horseshoe kidney?
Congenital disorder where the 2 kidneys fuse together, resulting in 1 large horseshoe kidney.
What are the 2 main theories for how the kidneys fuse i. Horseshoe kidney?
• mechanical fusion - during the metonephros stage (wk 5), the kdineys are close together in the pelvis and the inferior poles touch and fuse together, forming a fibrous isthmus
• Teratogenic event - posterior nephrogenic cells migrate to the wrong area, form a parenchymal isthmus between the 2 kidneys (i.e. made of kidney cells)
Why are the kidneys lower than usual in horseshoe kidneys?
During weeks 7-8, the kidneys ascend but it hooks around the inferior mesenteric artery; this is why horseshoe kidneys are lower in the body than usually.
Who’s more likely to get horseshoe kidneys?
Twice as common in men
more common in those with chromosomal disorders e.g. Turner syndrome, trisomy 13,18,21 and neural tube defects.
What does horseshoe kidney predispose you to?
hydronephrosis, kidney stones, infections. It’s also associated with an increased risk of kidney cancer (especially wilms tumour and carcinoid tumour)
Do you need to treat horseshoe kidney?
No people can live a normal lifespan
You should keep it in mind for surgical interventions
What is potter sequence?
A rare disorder where there is little amniotic foetus which causes pulmonary hypoplasia and developmental abnormalities such as flat face, wrinkly skin, epicanthal folds, low set ears and limb abnormalities like club feet.
What can cause potter sequence?
renal agenesis, cystic dysplasia, obstructive uropathy, atresia or ureter or urethra, amniotic rupture, uteroplacental insufficiency
How does amniotic fluid help the lungs develop?
• physically stretching the airways and contributing proline which helps form connective tissue and collagen in the lungs
Why is potter sequence more likely in boys?
they have a higher rate of urinary tract obstructions e.g. posterior urethral valves.
