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Haemolytic Uraemic Syndrome. David V Milford. Paediatric Nephrology for the General Paediatrician 2012 Manchester. Overview. Presentation Diagnosis Management Prognosis and follow-up Atypical HUS. Haemolytic uraemic syndrome. s. in childhood. Syndrome comprising.

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Haemolytic Uraemic Syndrome

David V Milford

Paediatric Nephrology for the General Paediatrician 2012

Manchester


Overview

  • Presentation

  • Diagnosis

  • Management

  • Prognosis and follow-up

  • Atypical HUS


Haemolytic uraemic syndrome

s

in childhood

Syndrome comprising

acute renal failure of varying severity

microangiopathic anaemia

thrombocytopenia of varying severity

Multiple aetiologies


BPSU National HUS study 1985-1988

D+

D-

reported

282 (94.6%)

16 (5.4%)

dialysed

165 (59%)

9 (56%)

followed for > 4

259

15

months

lone hypertension

3

2

CRF

17

2

78%

13.6%

ESRF

0

5

died

15

3

Arch. Dis. Child 1990; 65:716‑721


Arch. Dis. Child 1990; 65:716‑721


Evolution of lab results

in HUS – outbreak in an

institution


Diagnosis

  • Diarrhoea (often bloody)

  • Haematological – microangiopathic haemolytic

  • anaemia

  • - thrombocytopenia

Fragmented red cells

Absence of platelets


Acute Kidney Injury


Shiga toxin producing E.coli (STEC) isolates BAPN/CDC study 1985-88

isolates

VTEC

O157

HUS

185

60

39

Bloody diarrhoea

48

4

3

Diarrhoea

54

3

1

Controls

46

2

0

Arch. Dis. Child. 1990; 65:722‑727


D+ HUS and EHEC

  • EHEC colonise cattle

  • Transmission – contaminated meat, milk, water, fruit, vegetables

  • Exposure to EHEC → diarrhoea in ≈ 10% children

  • HUS develops in ≈ 15% of children with EHEC diarrhoea

  • O157:H7 predominant serotype in the UK

    • O26:H11, O103:H2, O111:NM, O121:H19, O145:NM


Annual STEC cases in the UK

Emerging Infectious Diseases 2005; 11: 590-6


Non-renal complications

  • Seizures

    • hyponatraemia

    • neurotoxicity (STx receptors – neurones, endothelium)

  • Hypertension

  • Gut

    • rectal prolapse

    • toxic megacolon, perforation, intussception

  • Cardiomyopathy

  • Diabetes mellitus


Acute CNS changes

6 months

Even in patients with severe CNS involvement on

acute imaging studies, prognosis can be favourable for clinical outcome and resolution of pathological imaging

findings

Pediatr Radiol (2004) 34: 805–810

Clin J Am Soc Nephrol 2010; 5:1218–1228,


Rectal prolapse

Air in bowel

Late change - stricture


Emerging Infectious Diseases 2005; 11: 590-6


395 D+ HUS

18 D- HUS

Infections in HUS 1997-2001

1 O26

65

329 (83%) O157 +ve

culture/serology

No infection identified 59

S pneumonae 7

Campylobacter 2

CKD 1

Died 2

(out of 8 cases)

Shigella Sonnei 1

S pneumonae 1

Staph aureus 1

Emerging Infectious Diseases 2005; 11: 590-6


Northern German outbreak May-July 2011

  • Source: bean sprout farm in Lower Saxony

  • Sprouted from batch of seeds from Egypt

  • 3793 cases of diarrhoea – O104:H4

  • Delay in symptoms, ingestion → diarrhoea 8 days

  • 827 (22%) developed HUS, 88% in adults

  • 53 deaths

  • 2010 European data

    • 4000 STEC cases reported, 5.5% developed HUS

    • O157 (41%), 026 (7%), O103 (2.5%)


Management

  • Conservative

    • Monitor fluid balance, sodium, potassium, H+,BP

    • Furosemide may be useful early

    • Sodium, protein restriction; high calorie intake

    • Transfuse with caution

    • Avoid antibiotics/anti-motility agents/NSAID

  • Transfer to regional centre

    • Oliguria +

      • Fluid overload, need for transfusion, high K

    • Anuria

    • Complications of D+ HUS


Prognosis and follow-up

  • BCH (n=250) 56% required acute dialysis

  • Prognostic markers

    • Neutrophils >20 at presentation

    • Dialysis > 2weeks

  • Mortality

    • 5% (BPSU 1985-88)

    • 1.8% (BPSU 1997-2001)

  • Long term: HBP, reduced GFR, proteinuria

    • Variable in studies, probably 20-30%

    • BCH n=201 19% poor outcome at 5,10,0r 15 yrs


Proteinuria at 1 year and outcome

Poor outcome

Good outcome

J Pediatr 1991; 118:191‑4


  • Follow-up

    • Frequently until Hb and creatinine normal

    • BP, PCr and EMU protein at 1 year after illness

    • BP, EMU protein, formal GFR, renal USS at 5 years and every 5 years until post pubertal

    • BP, EMU protein by GP at intervals once discharged

  • Lifestyle advice

    • Avoid overweight, high sodium intake

    • Avoid smoking

    • Girls need renal function/proteinuria monitoring during pregnancy


Level 1: aetiology advanced

1.i Infection induced

(a) Shiga and shiga-like toxin-producing bacteria;

enterohaemorrhagic Escherichia coli, Shigella

dysenteriae type 1, Citrobacter freundii

(b) Streptococcus pneumoniae, neuraminidase and

T-antigen exposure

1.ii Disorders of complement regulation

(a) Genetic disorders of complement regulation

(b) Acquired disorders of complement regulation, e.g.

anti-factor H antibody

1.iii von Willebrand proteinase, ADAMTS13, deficiency

(a) Genetic disorders of ADAMTS13

(b) Acquired ADAMTS13 deficiency; autoimmune,

drug induced

1.iv Defective cobalamin metabolism

1.v Quinine induced

Level 2: aetiology unknown

2.i Human immunodeficiency virus (HIV)

2.ii Malignancy, cancer chemotherapy and ionising radiation

2.iii Calcineurin inhibitors and transplantation

2.iv Pregnancy, HELLP syndrome and oral contraceptive pill

2.v Systemic lupus erythematosus and antiphospholipid

antibody syndrome

2 vi Glomerulopathy

2.vii Familial, not included in part 1

2.viii Unclassified

Typical/diarrhoeal/

D+ HUS

Atypical/

non-diarrhoeal/

D- HUS


Alternative complement pathway

Johnson, Eur J Pediatr 2008:167;965–971


Non-diarrhoeal HUS

  • Requires urgent referral to a nephrology centre

  • Associated with

    • High risk of death, CKD, hypertension, CNS events, recurrent episodes, familial

  • Therapies used

    • Plasmapheresis

    • Plasma infusion (especially ADAMTS13)

    • Eculizumab (binds to C5 and blocks C5 convertase)

    • Liver, liver/kidney transplantation


QUESTIONS?


Glomerular size in HUS patients with proteinuria

3.3-7 years after illness

n=7

J Pediatr 1998; 133:220-3


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