1. Red Urine – a mystery
Shaila Sukthankar

2. Common presenting symptom of renal tract disorders
Haematuria
Common presenting symptom of renal tract disorders
Prevalence % on population screening in children

3. Haematuria - Definition
Urine microscopy
RBC > 5/uL in a fresh uncentrifuged specimen
RBC > 5 -10/high power field in a midstream sample
RBC morphology & presence of casts

4. Case Presentation - May 09
5 years, male
Painless gross haematuria – frequent episodes 1 week
Initially red, later pink – no clots
No history of
Fever, dysuria, back/ abdo pain
rashes, joint pains
Swelling
Trauma
Bleeding diathesis
Recent medication
No family h/o renal disease/ deafness/ renal stones/ haematuria
Tonsillitis 6 weeks before

5. Normal vitals, BP 110/68, apyrexial No pallor or oedema
Examination
Normal vitals, BP 110/68, apyrexial
No pallor or oedema
No bruises or rash
Systems review NAD
ENT normal

6. Macroscopic haematuria with no features of glomerulonephritis
Painless
IgA nephropathy
Benign familial nephropathy/ Alport’s syndrome
Exercise induced
Coagulopathy
Painful
Infection
Trauma
Malignancy

7. Haematuria with features of glomerulonephritis
Primary renal diseases
IgA nephropathy
MPGN 1 and 2
Anti GBM disease
Secondary renal diseases
Postinfectious GN
HSP nephritis
SLE

8. Initial Investigations
FBC, coagulation – normal
Urea 6.5, creatinine 40, Albumin 46
Electrolytes, bone profile normal
crp <3
Urine microscopy (X2) - <10 WCC, RBC, no bacterial growth, trace to 1+ proteinuria
Renal USS - NAD

9. Subsequent Investigations
C3 and C4 normal
ANA, dsDNA negative
Immunoglobulins normal
ASOT 100 U/mL
antiDNASe B 600 U/mL
Urine calcium/ creatinine ratio 0.45
Intermittent 3+ blood on dipstick, no proteinuria and well with normal BP over next 4 weeks

10. Urine dipstick
Useful screening tool
Very sensitive

11. Haematuria - Diagnosis
Do not use urine dipstick to diagnose haematuria

12. Recurrence of painless gross haematuria for 1 week
12 weeks later (Aug 09)…
Recurrence of painless gross haematuria for 1 week
Always towards the end of the day
Clear in the morning
Bright red or cola coloured in the evening
Worse with exercise and vigorous activity
Some discomfort with micturition
No other significant positive history
Urine microscopy confirmed RBCs in some but not all red urine samples

13. Causes of red or pink urine
Haemoglobinuria
Myoglobinuria
Porphyrins
Urates (pink)
Foods – beetroot, blackberries
Drugs
Rifampicin (orange)
Chloroquine, desferoxamine

14. ? Bladder pathology (polyp, interstitial cystitis)
Possibilities - 1
Recurrent gross haematuria - ? Alport’s/ IgA nephropathy/ thin basement membrane disease
? Bladder pathology (polyp, interstitial cystitis)
Exercise induced haematuria
? Not blood (Hburia or myoglobinuria)
? Renal AV malformation

15. Repeat haematology, biochemistry and immunology normal
Management
Repeat haematology, biochemistry and immunology normal
Presence of blood without RBCs on some urine samples
Myoglobin screen positive on one occasion
No infection
MR renal angiogram (limited views) – normal
Cystoscopy – NAD
Family members’ urine microscopy – NAD
Review by haematology – no e/o intravascular hemolysis
Intermittent painless asymptomatic gross haematuria continues

16. Possibilities - 2
Exercise induced haematuria – exercise test with urine microscopy before and after
Nutcracker syndrome – Repeat MR/ direct renal angiogram under GA – parents not keen for further invasive procedures/ GA
Evolving nephropathy (IgA/ Alport’s/ TBM) – no indication for biopsy as asymptomatic, normotensive, no proteinuria and normal renal function

17. Compression of L renal vein between the aorta and sup mesentric artery
Nutcracker syndrome
Compression of L renal vein between the aorta and sup mesentric artery
40% of children with unexplained haematuria

18. Investigations in a child with haematuria
Urine microscopy and culture
Urine protein creatinine ratio
FBC, coagulation
U&E, creatinine, albumin
Urine calcium creatinine ratio
ASOT, C3 and C4
US renal tract

19. Haematuria - Indications for renal biopsy
Associated proteinuria
Persistent low C3
Impaired renal function
Systemic disease with proteinuria
SLE, HSP, ANCA associated vasculitis
Family history suggestive of Alport’s syndrome
Recurrent gross haematuria of unknown aetiology with extreme parental anxieties

20. Haematuria - cystoscopy
Seldom useful
Consider
Negative preliminary investigations
Suspected bladder or urethral pathology
Vascular malformations
Bladder mass on US
To lateralise the source of bleeding

21. Progress – June 10 (12 months on)
Well
Normally active
Occasional brown urine (once in 2-3 months)
Lasts for a day, resolves spontaneously
Occurs with activity
Occurs towards the end of the day
Normotensive
Parents and child opted for non-invasive observation for now

22. Investigation are to be guided by presentation and likely diagnosis
Haematuria - Summary
In the absence of proteinuria is not usually indicative of serious pathology
Investigation are to be guided by presentation and likely diagnosis
In asymptomatic children, ensure serious conditions are not missed and guidelines for further investigations are in place if change in clinical course

23. DID NOT SEE GP, COMMUNITY NURSES OR HOSPITAL TEAM
Latest update (March 11)
Well until 3 weeks before review!
Febrile coryzal illness with sore throat and recurrence of haematuria
Initially bright red, subsequently cola coloured
Lasted for 7-10 days, progressively cleared over 2-3 days thereafter
Asymptomatic (no headaches, oedema, oliguria etc)
DID NOT SEE GP, COMMUNITY NURSES OR HOSPITAL TEAM
When attended clinic, back to normal self, urine NAD!!
Repeat haematology, biochemistry and immunology normal.
ΔΔ??