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Flashcards in Conditions that results in haematuria Deck (21):
1

What conditions present primarily as glomerulonephritis?

Post-infections (including streptococcal)
SLE
Ig A nephropathy
HSP
Membranoproliferazive glomerulonephritis
Familiar nephritis
Immune complex nephritis
- infective endocarditis
- Hep B
Rapidly progressive glomerulonephritis
- Wegener
- Polyarteritis nodosa

2

What causes benign hemorrhagic cystitis that can produce bright red blood at end of micturition?

Adenovirus

3

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

4

What are clinical features of acute glomerulonephritis?

Micro / macroscopic haematuria
RBC casts
Proteinuria
Pyuria
Oedema
Oliguria
HTN

5

What conditions are assoc with low C3?

Post-streptococcal Glomerulonephritis, Lupus, Membranoproliferative, chronic infection

6

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

Renal failure
- Male presents in first 10 year with persistent haematuria and proteinuria
High tone nerve deafness
- progressive in second decade
- Renal transplant usually very successful

7

What is the inheritance pattern of benign microscopic haematuria?

Autosomal dominant
Occurs 1 in 200 children

8

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

Assoc w
- SLE, HSP, Polyarteritis nodosa, Wegener granulomatosis

Mx
- rates of survival and renal recovery improved with pulse methylpred, oral cyclophosphamide and plasmapheresis

9

What is the triad of HUS?

Microangiopathic haemolytic anaemia
- schistocytes (helmet cells), burr cells, fragmented RBC
- Coombs NEGATIVE
Thrombocytopenia
Acute renal impairment

Other features
- HTN can be severe
- Seizures is the most common manifestation

10

What is the most common cause of acute renal filature in children?

HUS
Most common under 3 yo

11

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

12

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

Pathology
- Focal proliferative glomerulonephritis with IgA in the mesangium
- similar to HSP

Serum Ig A raised

13

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'

14

What are the clinical features of PSGN?

Onset 2-3 weeks after GAS pharyngitis or 4-6 weeks after impetigo
- Fever, loin pain
- Oedema
- Oliguria
- HTN (60-80%) +/- CNS
- microscopic (all) +/- macroscopic haematuria (30-50%)

15

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%

16

What are the principles behind PSGN management?

Restrict protein, salt and potassium
HTN
- frusemide
- sometimes calcium channel blocker / ACE inhibitor but latter has SE of hyperkalaemia
- avoid B blocker if pul. oedema
Penicillin
- 10-day course

17

What are complications of PSGN?

ARF

Hypertensive encephalopathy
- seizures assoc with papillodema and temporary cortical blindness
- treat with diazoxide

LVF secondary to HTN / fluid overload

18

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

19

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

Cause includes:
- post-strep
- lupus
- membranoproliferateive
- goodpasture's
- HSP
- other forms of vasculitis

20

What condition has positive ANCA?

Wegner's granulomatosis

21

What are the classic signs of HSP?

The most common vasculitis in childhood (IgA mediated) .

Palpable purpura (100%)
arthralgia / arthritis (75%)
Abdo pain (70%)
Nephritis (20-100%)


**Intussusception is rare**
- The classic triad of colicky abdo pain, redcurrant jelly stools and vomiting is only seen in 2-6%