A 30-year-old male presented with jaundice, vomiting, fatigue and oliguria for 4 days. On examination, he had pallor, icterus, hepatomegaly and altered sensorium. Investigations revealed macrocytic anemia, elevated liver enzymes and kidney dysfunction. A kidney biopsy showed fibrin thrombi in renal capillaries. He was diagnosed with atypical hemolytic uremic syndrome (aHUS) based on the clinical features and biopsy findings. aHUS has a poor prognosis with mortality rates up to 25% and 50% of patients progressing to end-stage renal disease.

1. PROF DR K H NOOR UL AMEEN M5 UNIT DR.RAKESH PINNINTI CASE OF JAUNDICE

13. RFT PROGRESSION Hemo-Dialysis started on 16/5/2011 14/5 15/5 17/5 20/5 21/5 22/5 24/5 26/5 Date 104 96 148 180 99 89 179 71 Urea 4.8 4.9 5.2 13.0 8.8 6.7 8.2 7.2 Creat

15. KIDNEY BIOPSY (Apollo)

16. ONLINE REFERENCE Fibrin thrombi & RBC in renal capillaries Subintimal fibrin, but no inflammation Healing from prior fibrinoid injury, occluding vessel

18. FINAL DIAGNOSIS ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS

19. TTP vs HUS

20. D+ vs. D-

23. Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.

24. Treatment

25. Prognosis Index contains three adverse prognostic factors (age >40, hemoglobin <9 g/dL, temperature >38.5ºC). Subjects with zero, one, two, or all three of these adverse factors had 6-month mortality rates of 12, 14, 31, and 62 percent, respectively. Atypical hemolytic–uremic syndrome has a poor prognosis, with death rates as high as 25% and progression to end-stage renal disease in half the patients

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