Renal Flashcards
Nephrotic Syndrome:
- there is massively increased what?
- due to abnormalities of what?
- what are the main 3 features?
- increased filtration of macromolecules across the glomerular capillary wall
- due to structural/functional abnormalities of the glomerular podocytes
- hypoalbuminaemia, oedema and hypercholesterol/triglycerid-aemia
Podocyte dysfunction in nephrotic syndrome causes the triad of -hypoalbuminaemia, oedema and hypercholesterol/triglycerid-aemia, explain each.
- hypoalbuminaemia: (<30g/L) due to heavy proteinuria (>3.5g/24hrs) and increased renal catabolism of filtered protein
- oedema: due to sodium retention in CDs + increased capillary permeability, reduced circulating volume
- high choles/TGs: common due to increased synthesis and impaired catabolism
Nephrotic Syndrome with ‘bland urine sediments’
-most common causes?
-membranous nephropathy and FSGS or minimal change in children
Nephrotic Syndrome with ‘bland urine sediments’ - caused by Membranous nephropathy
- what is membranous n?
- what are the most common causes?
- IgG and C3 deposition along outer side of glomerular BM, BM expands as deposits slowly are resorbed
- usually idiopathic or SLE/thyroiditis related, neoplasitc related, infection of sarcoid/sickle cell related or drug induced
Nephrotic Syndrome with ‘bland urine sediments’ - caused by Membranous nephropathy
-membranous (IgG and C3 deposition around BM) can be secondary to drugs or infections name 3 examples
- drugs: penicillamine, gold, NSAIDs
- infections: hepatitis B and C, schistosomiasis, plasmodium malariae
Nephrotic Syndrome with ‘bland urine sediments’
- is often caused by focal segmental glomerulosclerosis, what population is it more common in?
- a similar histological appearance occurs in what?
- black adults
- HIV
Nephrotic Syndrome with ‘bland urine sediments’
- minimal change is a common cause in children (boys <5yrs mostly)
- findings on light microscopy vs EM?
-glomeruli normal on light microscopy but on EM can see fusion of the podocytes (foot processes of epithelial cells)
NB: increase in glomerular permeability is suspected to be immunologically mediated
Nephrotic Syndrome with ‘bland urine sediments’
-Name 2 Causes that are NOT immune mediated?
- associated with renal amyloid
- associated with diabetes
Nephrotic Syndrome with ‘active’ urine sediments (mixed nephrotic/nephritic)
- can be primary or secondary glomerular disease causing this
- suggest one primary cause that occurs with chronic infection (/idiopathic)
- mesangiocapillary membranoproliferative glomerulonephritis (renal failure develops over years)
- mesangial proliferative glomerulonephritis
Nephrotic Syndrome with ‘active’ urine sediments (mixed nephrotic/nephritic)
- can be primary or secondary glomerular disease causing this
- one primary cause is mesangial proliferative glomerulonephritis-describe it’s findings?
- presents with heavy proteinuria, minimal changes on light microscopy
- deposits of IgM and complement (IgM or C1q nephropathy) in the glomerular mesangium
- some pts respond to steroids, others progress to renal failure
Podocyte dysfunction in nephrotic syndrome causes what clinical features and hence what 2 ddx’s may arise?
- hypoalbuminaemia, oedema and hypercholesterol/triglycerid-aemia
- ankle, genitals, abdo wall oedema (face/arms rarely)
- ddx: CCF-but JVP would be raised whereas is low in/normal in nephrotic, cirrhosis
Investigations in Nephritic Syndrome Glomerular Disease:
- baseline (4)
- for diagnosis: -urine__?,-culture? -bloods?-
- serum ix: what Igs and antigens
- imaging?
baseline: eGFR, urine protein, U&Es, serum albumin
- diagnosis: urine microscopy, culture, serum U&E, albumin
- serum: ANA, anti-DNA Ig, ANCA, anti-GBM Ig, hepBsAg, hepCIg, HIV Ig, Cryoglobulins
- imaging: CXR, US kidneys, renal biopsy
What will you see on urine microscopy in a pt with nephrotic syndrome glomerulonephritis?
What may a throat swab come back positive with?
And blood glucose is checked because?
- red cell casts
- recent streptococcal infection + present
- in case diagnosis of diabetes mellitus (RF for these renal diseases)
Wegener’s granulomatosis/Granulomatosis with polyangiitis can cause blood vessel inflamm. in nose, sinuses, throat, lung and kidney, what may be seen on CXR?
-Cavities
Complications of Nephrotic Syndrome name 3
- Venous thrombosis
- Sepsis
- AKI
Why is there a predisposition for venous thrombosis in nephrotic syndrome? Presentation/diagnosis of this?
- loss of clotting factors, in peripheral and renal veins
- presents with renal pain, haematuria, and deteriorating renal function.
- diagnose by US
Why can sepsis arise as complication of nephrotic syndrome?
Why can AKI?
- sepsis as loss of Ig in urine increases susceptibility to infection (common cause of death)
- AKI usually arises as consequence of hypovolaemia (esp. after diuretic therapy) or renal vein thrombosis
Acute nephritic glomerulonephritis is characterised by what? (4)
NB: process is caused by an immune response that is triggered by disease/infection
e.g. post-streptococcal (group A beta-heamolytic) is a trigger in children
then bacterial antigen is trapped in glomerulus –> acute diffuse proliferative glomerulonephritis
- haematuria (+red cell casts on microscopy)
- proteinuria (<2g in 24hrs)
- hypertension and oedema (periorbital/leg/sacral) caused by salt/water retention
- oligouria
- uraemia
Acute nephritic glomerulonephritis
NB: process is caused by an immune response that is triggered by disease/infection
Suggest 5 diseases commonly associated?
- e.g. post-streptococcal (group A beta-haemolytic) is a trigger in children - antigen is trapped in glomerulus –> acute diffuse proliferative glomerulonephritis
- non-streptococcal post-infectious e.g. staphylococcus, mumps, legionella, hep B, hep C, schistosomiasis, malaria
- Infective endocarditis
- shunt nephritis
- visceral abscess
- SLE
- Henloch-Schonlein syndrome
- Cryoglobulinaemia
Rx/management for nephritic syndrome:
e. g. post-streptococcal
- hypertension rx:
- fluid balance:
- in SLE/systemic vasculitis related:
- e.g. post-streptococcal - supportive measures, spontaneously recovers
- hypertension: treat with salt restriction, loop diuretics, and vasodilators
- fluid balance: daily weights, input/output charts (if oliguric and overloaded must fluid restrict)
- in SLE/systemic vasculitis related: immunosuppression e.g. prednisolone, cyclophosphamide, azathioprine or rituximab
Rapidly progressive glomerulonephritis has 3 main causes:
-on background of acute nephritic syndrome
-anti-glomerular basement membrane disease (+lung involvement = Goodpasture’s)
-antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis
(treat the AKI and specific rx vs the cause)
Reflux nephropathy aka chronic/atrophic pyelonephritis arises from what?
leading to what?
-childhood UTIs in combo with vesicourethral reflux (incompetent valve between bladder and ureter)
leading to progressive renal scarring presenting as HT or CKD in childhood/adult life
Renal cysts are common with age, usually asymptomatic and incidentally found, occasionally what symptoms may occur?
- pain
- haematuria
ADPKD (autosomal dominant polycystic kidney disease) is a common 1:1000 condition due most commonly to a mutation in the ____ gene (short arm of chromosome __) which encodes for a protein “______1” - an integral membrane protein that regulates t____ and v____ development in kidneys and other organs.
(PKD2 mutation on chrsm 4 makes up rest of cases cause).
- PKD1 gene (chrsm 16)
- polycystin 1
- regulates tubular and vascular development
ADPKD (autosomal dominant polycystic kidney disease), due most commonly to a PKD1 mutation (chrsm 16) affecting tubular/vascular development causes what sort of disease/progression?
- multiple cysts develop in both kidneys
- cysts increase in size with age –> renal enlargement and progressive destruction of normal renal tissue
- with gradual loss of renal function
ADPKD (autosomal dominant polycystic kidney disease) 5 clinical features:
clue: pain-why?, 50% by 50yrs have …, cysts where else?, brain ? heart?
NB: presents any age from 20s
- acute loin pain due to cyst haemorrhage/infection or urinary tract stone formation (uric acid)
- abdo discomfort caused by renal enlargement
- hypertension
- progressive renal impairment (ESRF in 50% pts by 50yrs)
- liver cysts, rarely cyst in pancreas/spleen/ovaries
- subarachnoid haemorrhage - intercranial aneurysms common
- mitral valve prolapse (20%)
Diagnosis of ADPKD (autosomal dominant polycystic kidney disease) is definitively established by ?
Clinical exam demonstrates what?
- US
- clinical exam: large irregular kidneys, hypertension +/- hepatomegaly
- in adults with a family hx of ADPKD (autosomal dominant polycystic kidney disease), diagnosis criteria by age of patient:
- <30
- 30-60
- 60+
2+ unilateral renal cysts in pts <30yrs,
2 cysts in each kidney 30-60yrs and
4 cysts in each kidney 60yrs+
Management or monitoring of polycystic kidney disease:
who should be offered screening in their 20s?
- no rx slows progression or reduces cyst size
- BP should be controlled
- monitor disease progression by regular serum creatinine check ups
- many will eventually need RRT by dialysis/transplant
- offer children and siblings of pts with the disease screening with renal ultrasound
What is medullary sponge kidney ? Dilatation of ? in the ? +/- \_\_\_ change What forms within the \_\_\_? Pts present with renal \_\_\_ and \_\_\_\_\_ 20% get h\_\_\_\_\_ or \_\_\_\_ \_\_\_\_ a\_\_\_ NB: Diagnosed by excretion urography
- rare condition with dilatation of the CDs in the papillae +/- cystic change
- in severe cases, the medullary area has a sponge-like appearance
- small calculi form within the cysts
- pts present with renal colic / haematuria
- 20% get hypercalciuria or renal tubular acidosis
