New AKI – Acute glomeurolnephritis

You are asked to see a patient in ED by the nurse in charge. 16y old female became unwell 1 week ago with diarrhoea. She noted some blood in her stool. On exam, BP 120/60. Conjunctival pallor noted. Systems exam normal.

Bloods:

• Creatinine: 259
• Urea: 23
• K+: 4.4
• Hb: 117 (normal MCV)
• WCC: 12.2 x 10^9/L
• Platelets: 29 x 10^9/L
• CRP: 2 mg/L (1-5 mg/L)
• Bilirubin: 59 (1-20 umol/L)
• LDH: 1778 iu/L (130-250 iu/L)
• Haptoglobin: <0.08 g/L
• Direct Coombs test: Negative
• Fibrinogen: 3.3 g/L (2.0-4.5 g/L)

Peripheral smear: Marked red cell fragmentation noted on blood film. Suggestive of MAHA.

What advice would you give the medical registrar?

Find out if she is stable. If unstable, involve ICU. Ask regarding neuro symptoms (stroke-like symptoms, confusion, seizures). Rule out immediately life-threatening causes of MAHA first – TTP (send ADAMTS13, calculate PLASMIC score and discuss with Haematology), DIC, consider severe infections (Meningococcal, malaria, etc.). Likelihood is D+ HUS. Send stool for MCS + specifically test for Shigatoxin producing E Coli.

How would you manage D+ HUS?

Supportive care only. Dialysis if indicated. Antibiotics are advised against as they may worsen HUS.

If this patient gave you a family history of her mother and grandmother being affected by renal failure and having had renal transplants, what additional considerations might you have in this particular case?

Consider atypical HUS (complement-mediated HUS), genetic forms particularly. Contact the Newcastle HUS service and discuss the case. Send a sample to Newcastle for genetic testing.