Renal 7.5 Flashcards
(290 cards)
Options of renal replacement therapy
Tx: living/deceased; always has better outcomes than any dialysis, and more effective when pre-emptive
Haemodialysis
Haemofilltration
Haemodiafiltration (in a Rx centre more effective than HD in a Rx centre)
PD - continuous ambulatory, & automated PD
Indications for considering starting RRT?
- Sx of uraemia on ADLs
- biochemical measures
- uncontrollable fluid overload
- eGFR 5-7 if no Sx
When to start assessment for RRT/conservative Rx?
At least 1yr before therapy e.g. Tx/HD is likely to be needed, inc for those with a failing Tx
What are AV fistulas?
= Direct connections between arteries & veins, may occur pathologically but gene formed surgically to allow access for HD - now regarded as preferred method of access due to lower rates of complications
What are the potential complications of AV fistulas?
- infection
- thrombosis
- stenosis
- steal syndrome
How to calculate anion gap?
(Na + K) - (HCO3 + Cl)
normal 8-14
Causes of a normal anion gap or hyperchloraemic metabolic acidosis?
- GI bicarbonate loss: diarrhoea, fistula, ureterosigmoidostomy
- renal tubular acidosis
- drugs e.g. Acetazolamide
- Ammonium chloride injection
- Addison’s disease
Causes of a raised anion gap metabolic acidosis?
- lactate: shock, hypoxia, burns, metformin (type B)
- ketones: DKA, etoh
- urate: renal failure
- acid poisoning: salicylates, methanol
What is Alport’s syndrome? what is the inheritance?
X-linked dominant
- defect in the gene which codes for type IV collagen -> abnormal glomerular-basement membrane
- more severe in males (females rarely develop renal failure)
Pt with Alport’s syndrome has a renal Tx that starts to fail - what is the cause?
presence of anti-GBM antibodies, leading to a Goodpasture’s syndrome like picture
When does Alport's syndrome present? what are the features? renal ear eye
- microscopic haematuria
- progressive renal failure
- BL SNHL
- lenticonus: protrusion of the lens surface into the anterior chamber
- retinitis pigmentosa
What is on renal biopsy in Alport’s syndrome?
Splitting of lamina densa on electron microscopy
What is amyloidosis?
Extracellular deposition of an insoluble fibrillar protein amyloid
- accumulation of amyloid fibrils leads to tissue/organ dysfunction
What are the component of amyloid?
- many different precursor proteins
- fibrillar component, insoluble amyloid
- non-fibrillary components inc: amyloid-P (derived from acute phase protein serum amyloid P), apolipoprotein E & heparan sulphate proteoglycans
How to classify amyloidosis?
systemic vs localised
- further characterised by precursor protein e.g. AL in myeloma - A amyloid, L Light chain Ig fragments
3 ways to Dx amyloidosis?
- Congo red staining of tissue: apple-green birefringence
- SAP: serum amyloid precursor scan
- Biopsy of rectal tissue
What are the 3 main types of amyloidosis?
- AL amyloid (Light chain Ig fragment)
- AA amyloid (precursor serum amyloid A protein, acute phase reactant)
- Beta-2 microglobulin amyloidosis (precursor protein, part of the major histocompatibility complex)
AL amyloid
- what are the causes?
- what are the features?
- myeloma, MGUS, Waldenstom’s
- cardiac & neuro involvement, macroglossia, periorbital eccymoses
AA amyloid
- when is it seen?
- what are the features?
- chronic infection/inflammation eg TB, RA, bronchiectasis
- fenal involvement is most common feature
Beta-2 microglobulin amyloidosis (precursor that is a part of the MHC) - what is it associated with?
pts on renal dialysis
ARPKD: what is the genetic defect?
- defect in gene on chr 6 which encodes fibrocystin (protein important for normal renal tubular development)
How to Dx ARPKD?
What are the features?
- prenatal US or in early inference with abdo masses & renal failure
- newborns may have features consistent with Potter’s syndrome 2ry to oligohydramnios
- ESRF develops in childhood
- pts also typically have liver involvement e.g. portal & interlobular fibrosis
What is on renal biopsy in ARPKD?
multiple cylindrical lesions at right angles to the cortical surface
What is the genetics of ADPKD type 1?
PKD1 loci, which codes for polycystin-1
chr 16
85% of cases, presents with renal failure earlier