GU middle tier Flashcards
(45 cards)
nephritic syndrome pathophysiology
- inflammation of glomeruli
- glomerular capillary develop large pores, allowing blood to flow into the urine (rbc and wbc (sterile pyuria))
- podocytes also damaged (proteinuria)
- rapid decline in function
- low renal function – low urine output
- fluid overload –> hypertension, oedema
nephritic syndrome investigations/ symptoms
dipstick
- haematuria!
- proteinuria (little)
- sterile pyruria
- high creatinine
urinanalysis – red cell casts (microscopic haematuria- red cells clumped togeth)
cola coloured urine
oliguria
- low output volume
- decreased GFR
fluid overload , hypertension, oedema
uraemia
nephritic syndrome causes
AAV (ANCA associated vacilitis, small vessel)
goodpastures disease (acute, autoimmune)
SLE, systemic sclerosis
IE
upper resp tract infection
- IgA nephritis (days, common, )
- post streptococcal infection (weeks, often tonsilitis)
viral (hepB/C, malaria)
bacterial (MRSA)
Nephritic syndrome treatment
Treat underlying disease
Recovery happens spontaneously
nephrotic syndrome pathophysiology
- Damage to podocyte foot processes –> gaps in podocytes
- Glomerular permeability increased so protein lost as it filters through into urine
- Albumin lost → hypoalbuminemia (and proteinuria) → decreased intravascular oncotic pressure → fluid moves into surrounding tissue, causing oedema
- Hypoalbuminemia → liver compensation → increased lipid production → hyperlipidaemia/ Less of the protein enzymes that convert → storage or that catabolise (lipase)
- Increased risk of thromboembolism due to liver compensation
- Increased susceptibility to infection (Ig lost in urine)
nephrotic syndrome symtpoms
triad
- heavy proteinuria (frothy wee)
- hypoalbuminaemia
- peripheral oedema
- hyperlipidaemia/ hypercholesterolaemia (due to more production due to lipid compensation /lipases and storage conversion enzymes lost in urine)
- increased susceptibiltiy to infection (Ig lost in urine)
- increased risk of thromboembolism (liver compensation)
- potentially haematuria
causes of nephrotic syndrome with detail inc pathophsy, cause, epidem, treatment
minimal change
- fused podocytes –> loss of foot processes
- vacuolation
- microvilli appear in glomerulus
- see with electron microscopy (not light)
- often children
- related to atopy
- steroids and anti-oedema
membranous
- thickened basement membrane
- secondary to drugs, autoimmune, infection, malignancy
- asymptomatic but investigations same
- steroids and immunosup
focal segmented glomerulosclerosis (FSGS)
- unknown reasoning
- steroids and immunosup
what is the most common secondary cause to nephrotic syndrome
+ others
diabetes
hepB/C SLE drugs/toxins amyloidosis RA malignancy
nephrotic syndrome treatment
- loop diuretics for oedema
- BP control - for proteinuria
- statins - for hyperchol
- anticoag
- steroids (in children and ?)
if membranous, immunosup
glomerulonephritis = aka
nephritic syndrome!!
nephrotic vs nephritic
nephrotic has higher proteinuria !
nephritic also has capillary damage –> blood loss!
can be related though - eg nephritic syndrome with a nephrotic element
uraemia symptoms
uraemia = high serum urea
anorexia
pruritus (itchy) rash
lethargy
nausea
what does frothy urine indicate?
high protein
dominant polycystic kidney disease
- penetrance high/low
- commoness
- gender
high penetrance
common
m>F
is dominant/recessive polycystic kidney disease more common
dominant
dominant/recessive polycystic kidney disease age of presentation
dom - largely 20 onwards
rec - infancy
which genes are affected in dominant/recessive polycycstic kidney disease
dom - PKD1 (main) and PKD2
(- PKD1 encodes polycystin 1 - involved in cell-cell and cell-matrix interactions, regulates tubular and vascular development
- PKD2 encodes polycystin 2 - = calcium channel)
rec- PKHD1
dominant polycystic kidney disease pathophysiology
- PKD1 encodes polycystin 1 - involved in cell-cell and cell-matrix interactions, regulates tubular and vascular development
- PKD2 encodes polycystin 2 - = calcium channel
- cyst formation
- These increase in size with age → renal enlargement → progressive destruction of renal tissue
- PKD1 mutation has a quicker course, more reach ESRF end stage renal failure than PKD2 mutation
recessive polycystic kidney disease pathophysiology
Collecting duct dilation and elongation → Result in kidney enlargement and tissue destruction.
Loss of kidney function due to mechanical compression, apoptosis of healthy tissue (Atrophy) and reactive fibrosis.
dominant polycystic kidney disease progression
- Clinically silent for many years (family screening important)
- Bilateral kidney enlargement
- Hypertension
- Renal stones
- progressive renal failure
- loin/abdomen pain
- Renal colic (clots)
- cyst haemorrhage /infection /urinary tract stone
- – Haematuria
- – Acute loin pain
- Nocturia
- Excessive water and salt loss
- Cysts may become infected
where else is affected by PKD1/2 mutation (dominant)
- Liver/pancreas - have cysts but function unaffected
- Heart - valve disorders
- brain/vessels - berry aneurysms → subarach haem
recessive polycystic kidney disease symptoms
- Enlarged kidneys
- Renal cysts
- Hepatic fibrosis– Portal hypertension
- Kidney failure
polycystic kidney disease investigation including diagnosis
Ultrasound
- Liver and pancreas cysts in dominant
- Salt and pepper appearance for recessive
- Diagnosis:
15-39 y ≥2 or 3 cysts total (uni/bilateral)
40-59y ≥ 2 cysts each kidney
60y+ ≥4 cysts each kidney
Exclude if less than 2 cysts total above 40 y . cannot exclude diagnosis if less than 30y
PKD1/2 /PKHD1 genetic testing
BP- raised
Monitor liver (rec) -
CT/MRI
- MRI- to screen for intracranial aneurysms
polycystic cysts management
Family screening
- Ultrasound
Genetic testing
If early onset/prenatal diagnosis, atypical disease, reproductive choices, potential kidney donor available?
No treatment slows progression
Disease progression monitored with serum creatinine
Treat stones, UTIs, HTN (ACEi), pain (analgesia)
Renal replacement for end stage renal failure ESRF
Surgical
- removal of cysts laparoscopically - helps with pain
- Nephrectomy - removes whole kidney
Counselling, support
