Red Urine – a mystery

1. Red Urine – a mystery Shaila Sukthankar

2. Haematuria • Common presenting symptom of renal tract disorders • Prevalence 0.5 - 6% 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. 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, 50-100 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. 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. 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. 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. 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. 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. 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??