L4: Pathology 1 Flashcards
Patients with chronic renal failure/renal disease usually die from what cause?
HEART FAILURE
- when kidneys aren’t working properly, need to increase blood flow to them
- heart needs to pump harder
- this can lead to heart disease + failure
e.g. can probably see LV hypertrophy in heart
What are the function of podocytes?
- prevent protein from entering the urinary filtrate
- foot processes extend from podocytes creating filtration slits
What is the value of hydrostatic pressure that allows filtration at the glomerulus?
50 - 60 mmHg
Where is the juxtaglomerular apparatus located?
- distal convoluted tubule
- beside the afferent arteriole to measure blood flow to kidney
- renin produced here
What are the 2 types of injury that can occur in the kidney tubules?
A. Ischaemic
B. Toxic
What are urinary casts?
- result from solidification of material in the renal tubule
- excreted via urine
- different materials (e.g. RBC, WBC, hyaline…)
There are 2 types of injury that can occur in the kidney tubules. Which type is more likely in the proximal renal tubule?
- proximal renal tubule has more mitochondria
- prone to ischaemic injury
What is bilateral renal agenesis and what is a common ultrasound finding for this?
- fetus with no kidneys
- no amniotic fluid in womb (oligohydramnios)
- a/w Potter Syndrome
What is Oligohydramnios and what is genetic kidney condition is it a sign of?
- no amniotic fluid in uterus
- indicative of bilateral renal agenesis
What is Potter Syndrome and what is the most common condition it is a/w?
- physical characteristics of a fetus that develop when there is too little amniotic fluid in the uterus (in utero) during pregnancy
- a/w bilateral renal agenesis
List some congenital anomolies of the kidneys
- bilateral renal agenesis
- unilateral renal agenesis
- horseshoe kidney
- hypoplasia (lack of nephrons)
- ectopia (ectopic kidney tissue)
- dysplastic kidney (abnormally formed)
In a patient with a horseshoe kidney, when ascending from the pelvis, which blood vessel may it get stuck underneath?
Inferior Mesenteric Artery
What does ADPKD stand for?
Autosomal Dominant Polycystic Kidney Disease
What genes are involved in ADPKD?
90% - Chromosome 16 (PKD1, PKD2)
10% - Chromosome 4
What does ARPKD stand for?
Autosomal Recessive Polycystic Kidney Disease
What gene (and chromosome) is involved in ARPKD?
PCKD1 gene (Chromosome 6P12)
What are the main differences between ARPKD and ADPKD?
ARPKD:
- affects at young age (stillborn babies)
- more severe
- cysts in kidney ± liver
ADPKD:
- can appear in adults
- less severe
- cysts only in kidneys
What is Nephronophthisis?
- autosomal recessive
- cystic kidney disease
What is Medullary Cystic Disease?
- autosomal dominant
- cystic kidney disease
Cysts in the kidney have a predisposition for what?
renal cell carcinoma (due to increased cell proliferation)
What is Von Hippel-Lindau Syndrome?
- autosomal dominant
- presents with kidney cysts
- a/w haemangioblastomas of retina, cerebellum and spine
What genetic abnormality is a/w Von Hippel-Lindau Syndrome?
VHL gene (tumour suppressor) on chromosome 3p25
What is the major risk/consequence in patients with Von Hippel-Lindau Syndrome?
50% develop renal cell carcinoma
What is the treatment of those with Von Hippel-Lindau Syndrome?
prophylactic bilateral nephrectomy
