Nephrotic syndrome Flashcards
What typically presents with nephrotic syndrome
Non proliferative glomerulonephritis - glomerular damage + sclerosis
What is nephrotic syndrome
Heavy proteinuria >3.5g/day
Hypoabuminaemia 35g/L
Oedema
Non classical features typical of nephrotic syndrome that are still typical
Hyperlipidemia
Inreased risk of VTE/arterial thrombosis
Higher risk of infection
All due to protein loss
Primary nephrotic syndrome causes
Minimal change disease
Focal segmental glomeruloscelrosis
Membranous nephropathy
Secondary nephrotic syndrome causes
Diabetes mellitus
Amyloidosis
HIV
What causes the symptoms of nephrotic syndrome
Loss of protein principally albumin across lomeruli -> loss oncotic pressure + increased fluid in interstitial space
Symptoms of nephrotic syndrome
Fatigue
Poor appetite
Peripheral oedema
Periorbital oedema
SOB - effusions/oedema pulm
Foamy urine - excess protein
Signs of nephrotic syndrome
Oedena
Ascites
Effusions
Can AKI occurin nephrotic syndrome?
Yes but less common than in nephritic
Principal investigation for sus glomerular disease
Renal biopsy
What is normal for protein:creatinine urine ratio
<30mg/mmol
What is normal protein content over 24 hour urinary colelction
<0.2g
Blood tests for glomerular disease
FBC, U+Es. bone profile, HbA1c, ABG, lipid profile
What causes majority of nephrotic syndromes in children
Minimal change disease
What disease wuld show fusion of podocyte foot processes in electron micrscopy
minimal change disease
Diagnosis of minimal change disease
Children with nephrotic sydnrome - presumptive - initiate treatment and monitor response
Adults - renal biopsy
Management of nephrotic syndroem
Systemic glucocorticoids eg prednisolone
Unresponsive -> further prednisolone causes or more intense immunosupressives
What is focal segmental glomerulosclerosis
Sclerosis in at least one part of glomerulus
Primary, secondary or genetic
what happens in focal segmental glomerulosclerosis - primary
circulating factor causes Foot process effacement - loss of podocyte foot process structure -> spread out, reduces filtration barrier efficacy
Secondary focal segmental glomerulosclerosis what happens histologically
Adaptive response to renal injury usually ass with less significant proteinuria and renal impairment
Combo of glomerular hypertrophy (cell proliferation)+ hyperfiltration (abnormally high glomerular filtration rate)
Cuases of secodnary focal segmental glomeruloscerosis
Severe obesity
Reflux nephopathy
Reduction in kidney mass 0 due to comensatory hyperfiltration in remaining kidney mass
Diagnosis of focal segmental glomerulosclerosis
Renal biopsy
In childhood suggests genetic cause 22
What is membranous nephropathy?
Glomerular basement membrane thickening in absence of significant cellular proliferation
Common in adults
Primary membranous nephropathy cause
AI reaction against important antigens in filtration barrier
=> autoantibody deveolpment. immune deposits and thickening of GBM
PLA2R antibodies major cause
Secondary membranous nephropahty
Underlying infection, drug use, systemic disorder context
SLE, viral hepatitis, prostate cancer, NSAID use
Diagnosis of membranous nephropathy
Anti-PALR2 on bloods - primary with nephrotic syndrome
Renal biopsy
Management membranous nephropathy
High risk disease progression - immunosupressants in primary MN
Secondary = underlying cause
Some may spontaneously resolve
Renal amyloidosis what is
Deposition of amyloid fibrils in glomerulus -> nephrotic syndrome
AL or AA
AL amyloidosis
Excess light chains - plasma cell disorders often malignancies
AA amyloidosis
Excess precursor protein due to chronic inflammation
Diagnosis of amyloidosis
Renal biopsy using congo red staining
Can be taken from anywhere as systemic
How does amyloidosis look in congo red staining
apple green birefringence under polarised light
Management of amyloidosis
AA, AL, hereditary
Supportive theraoy where renal involvement -> nephrotic syndrome
Severe end stage renal disease -> dialysis
