Flashcards in Conditions that results in haematuria Deck (21):
What conditions present primarily as glomerulonephritis?
Post-infections (including streptococcal)
Ig A nephropathy
Immune complex nephritis
- infective endocarditis
- Hep B
Rapidly progressive glomerulonephritis
- Polyarteritis nodosa
What causes benign hemorrhagic cystitis that can produce bright red blood at end of micturition?
What is the pathology of glomerulonephritis?
Inflammation of glomeruli resulting in proliferation of one or more glomerular cell types including mesangial cells, endothelial cells and epithelial cells
What are clinical features of acute glomerulonephritis?
Micro / macroscopic haematuria
What conditions are assoc with low C3?
Post-streptococcal Glomerulonephritis, Lupus, Membranoproliferative, chronic infection
What is alport syndrome?
Proliferative glomerulonephritis and high tone nerve deafness
Caused by mutation in gene coding for type IV collagen
- 80% x-linked dominant
- Rest AR / AD
- Electromicroscopy causes splitting of internal elastic lamina in basement membrane
- Male presents in first 10 year with persistent haematuria and proteinuria
High tone nerve deafness
- progressive in second decade
- Renal transplant usually very successful
What is the inheritance pattern of benign microscopic haematuria?
Occurs 1 in 200 children
What's Goodpasture disease?
Clinical picture of pulmonary haemorrhage and glomerulonephritis associated with antibodies against lung and glomerular basement membrane.
Immunofluorescent microscopy shows continuous linear patter of IgG along GBM
- SLE, HSP, Polyarteritis nodosa, Wegener granulomatosis
- rates of survival and renal recovery improved with pulse methylpred, oral cyclophosphamide and plasmapheresis
What is the triad of HUS?
Microangiopathic haemolytic anaemia
- schistocytes (helmet cells), burr cells, fragmented RBC
- Coombs NEGATIVE
Acute renal impairment
- HTN can be severe
- Seizures is the most common manifestation
What is the most common cause of acute renal filature in children?
Most common under 3 yo
What are poor prognostic features of HUS?
Atypical (Diarrhoea negative) type
Onset > 5y
Anuria > 2w
Initial neutrophilia > 20
Recurrence in diarrhoea +ve cases is rare with 90% making full recovery whereas diarrhoea -ve cases often relapses with high risk of hypertension, chronic renal failure and mortality
What is Berger's disease?
Ig A nephropathy
- most common cause of chronic glomerulonephritis
- present in 50% of children with recurrent episode of macroscopic haematuria
- occurs with intercurrent viral infection and flank pain
- usually normotensive
- very few develop chronic renal failure
- Focal proliferative glomerulonephritis with IgA in the mesangium
- similar to HSP
Serum Ig A raised
What is the pathological of PSGN?
Antigen-antibody complexes depositing into glomeruli
- proliferation of mesangial cells and endothelial cell with neutrophil infiltration
- immunofluorescence show IgG and C3 (alternative complement activated rather than classical)
- EM show sub endothelial 'humps'
What are the clinical features of PSGN?
Onset 2-3 weeks after GAS pharyngitis or 4-6 weeks after impetigo
- Fever, loin pain
- HTN (60-80%) +/- CNS
- microscopic (all) +/- macroscopic haematuria (30-50%)
What ix helps dx PSGN?
Mild anaemia with low grade haemolysis
Low C4 initially then normalises
Low C3 in 90% and returns w/in 6-12weeks
Antistreptolysin O titre and antistreptococcal DNAse B are elevated in 90%
What are the principles behind PSGN management?
Restrict protein, salt and potassium
- sometimes calcium channel blocker / ACE inhibitor but latter has SE of hyperkalaemia
- avoid B blocker if pul. oedema
- 10-day course
What are complications of PSGN?
- seizures assoc with papillodema and temporary cortical blindness
- treat with diazoxide
LVF secondary to HTN / fluid overload
What's the natural outcome of PSGN?
Acue phase resolve w/in 2m
CRF in 1%
Proteinuria clears w/in 6m
Microscopic haematuria may continue for 2y
What's rapidly progressive glomerulonephritis?
Clinical cause of several forms of glomerulonephritis with presence of crescents in majority of glomeruli as unifying abN.
Crescents are seen in Bowmans capsule.
Progression to ESRF follows w/in wks to months
- other forms of vasculitis
What condition has positive ANCA?