Do you see this patient frequently?

  • Fatigue and pallor
  • Swelling (especially legs/face)
  • Decreased urine output
  • Unexplained bruising or small red spots (petechiae)
  • Elevated blood pressure

Do you encounter children with acute kidney injury, unexplained anemia, and low platelets?

Early referral could make all the difference— how can you help?

Timely diagnosis of aHUS is vital for improving outcomes1

aHUS could be a fatal disorder if not timely and appropriately treated5, aHUS has a high degree of morbidity and mortality3 Time is of the essence when diagnosing and selecting appropriate therapies for this life-threatening condition3

Round 1:

Recognise TMA early6

TMA
=
THOMBOCYTOPENIA6
Platelet count < 150 × 109/L or > 25% decrease from baseline
MAHA6 Schistocytes and/or elevated LDH and/or decreased haptoglobin and/or decreased haemoglobin

And one or more of the following:7

Neurological symptoms

  • Confusion1
  • Seizures1
  • Stroke1
  • Coma1

Visual symptoms

  • Pain and blurred vision1
  • Ocular occlusion1

Renal impairment

  • Elevated creatinine level3
  • Decreased eGFR3
  • Abnormal urinalysis results3
  • Elevated blood pressure1

Pulmonary symptoms

  • Dyspnoea7
  • Pulmonary haemorrhage7
  • Pulmonary oedema7

Cardiovascular symptoms

  • Myocardial infarction7
  • Hypertension7
  • Diffuse Vasculopathy7
  • Peripheral gangrene7

Gastrointestinal symptoms

  • Diarrhoea ± blood1
  • Nausea/vomiting1
  • Abdominal pain1
  • Gastroenteritis / pancreatitis1

Don’t miss the signs

The symptoms of TMA are non-specific rendering the diagnosis is very challenging.2 Recognising them early helps ensure you can beat the clock in this life-threatening disease.3

Round 2:

Rapidly determine the cause of TMA6

aHUS is a diagnosis of exclusion1

≤ 5%
ADAMTS13 activity
TTP
Shiga toxin/EHEC
positive
STEC-HUS
> 5%
ADAMTS13 activity
aHUS

While awaiting ADAMTS13 results, a platelet count >30 × 109/L and/or a serum creatinine of >1.7 to 2.3 mg/dL are unusual in a diagnosis of TTP7

  • CBC with platelet count
  • Peripheral smear for schistocytes
  • LDH
  • Haptoglobin
  • Bilirubin
  • Creatinine/eGFR
  • Urinnalysis (protein/haematurial)
  • PT/INR-aPTT

Round 3:

Treat aHUS immediately6

In aHUS, TMA is driven or exacerbated through overactivation of the complement pathway3 Early clinical intervention is crucial to help patients with aHUS beat the clock and to enhance their outcomes1
Take immediate and appropriate action1

Request Medical Information Watch Expert Series

References:

  • Azoulay E, Knoebl P, Garnacho-Montero J, Rusinova K, Galstian G, Eggimann P, Abroug F, Benoit D, von Bergwelt-Baildon M, Wendon J, Scully M. Expert statements on the standard of care in critically ill adult patients with atypical hemolytic uremic syndrome. Chest. 2017 Aug 1;152(2):424-34.
  • Blasco M, Guillén-Olmos E, Diaz-Ricart M, Palomo M. Complement mediated endothelial damage in thrombotic microangiopathies. Frontiers in Medicine. 2022 Apr 25;9:811504.
  • Raina R, Krishnappa V, Blaha T, Kann T, Hein W, Burke L, Bagga A. Atypical hemolyticusuremic syndrome: an update on pathophysiology, diagnosis, and treatment. Therapeutic Apheresis and Dialysis. 2019 Feb;23(1):4-21.
  • Kavanagh D, Goodship TH, Richards A. Atypical hemolytic uremic syndrome. InSeminars in nephrology 2013 Nov 1 (Vol. 33, No. 6, pp. 508-530). WB Saunders.
  • Tseng MH, Lin SH, Tsai JD, Wu MS, Tsai IJ, Chen YC, Chang MC, Chou WC, Chiou YH, Huang CC. Atypical hemolytic uremic syndrome: Consensus of diagnosis and treatment in Taiwan. Journal of the Formosan Medical Association. 2023 May 1;122(5):366-75.
  • McFarlane PA, Bitzan M, Broome C, Baran D, Garland J, Girard LP, Grewal K, Lapeyraque AL, Patriquin CJ, Pavenski K, Licht C. Making the correct diagnosis in thrombotic microangiopathy: a narrative review. Canadian Journal of Kidney Health and Disease. 2021 Apr;8:20543581211008707.
  • Yerigeri K, Kadatane S, Mongan K, Boyer O, Burke LL, Sethi SK, Licht C, Raina R. Atypical Hemolytic-Uremic Syndrome: Genetic Basis, Clinical Manifestations, and a Multidisciplinary Approach to Management. Journal of Multidisciplinary Healthcare. 2023 Dec 31:2233-49.
  • Hofer J, Rosales A, Fischer C, Giner T. Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Frontiers in pediatrics. 2014 Sep 8;297.
  • LDH, lactate dehydrogenase
  • MAHA, microangiopathic haemolytic anaemia
  • eGFR, estimated glomerular filtration rate
  • TMA, thrombotic microangiopathy
  • ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13
  • EHEC, enterohemorrhagic E. coli
  • STEC-HUS, shiga toxin-producing E. coli haemolytic uremic syndrome
  • TMA, thrombotic microangiopathy
  • TTP, thrombotic thrombocytopenic purpura