Red Urine – a mystery

Shaila Sukthankar

Haematuria

Common presenting symptom of renal tract disorders

Prevalence 0.5 - 6% on population screening in children

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

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

Examination

Normal vitals, BP 110/68, apyrexial

No pallor or oedema

No bruises or rash

Systems review NAD

ENT normal

Macroscopic haematuria with no features of glomerulonephritis

Painless– IgA nephropathy– Benign familial nephropathy/ Alport’s syndrome– Exercise induced– Coagulopathy

Painful– Infection– Trauma– Malignancy

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

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

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

Urine dipstick

Useful screening tool

Very sensitive

Haematuria - Diagnosis

Do not use urine dipstick to diagnose haematuria

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

Causes of red or pink urine

Haemoglobinuria Myoglobinuria Porphyrins Urates (pink) Foods – beetroot, blackberries Drugs

– Rifampicin (orange)– Chloroquine, desferoxamine

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

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

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

Nutcracker syndrome

Compression of L renal vein between the aorta and sup mesentric artery

40% of children with unexplained haematuria

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

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

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

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

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

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.

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