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A Case of Atypical Hemolytic Uremic Syndrome

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Stanley Medical College, Department of Medicine
Gesundheit & Medizin
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PROF DR K H NOOR UL AMEEN M5 UNIT DR.RAKESH PINNINTI CASE OF JAUNDICE
Chief complaints ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Presentation ,[object Object],[object Object],[object Object],[object Object],[object Object]
Presentation ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
History ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Examination ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
GPE ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Systemic examination ,[object Object],[object Object],[object Object],[object Object]
CNS ,[object Object],[object Object],[object Object],[object Object]
List of problems ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],PERIPHERAL SMEAR : MACROCYTIC ANEMIA URINE FOR HB : POSITIVE 24HR URINARY PROTIEN : 100 mg%
Investigations ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],USG ABDOMEN : FATTY LIVER XRAY CHEST : NORMAL ECG : SINUS TACHY`
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
GH HEMATOLOGY FOR RESCUE ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
KIDNEY BIOPSY (Apollo)
ONLINE REFERENCE Fibrin thrombi & RBC in renal capillaries Subintimal fibrin, but no inflammation Healing from prior fibrinoid injury, occluding vessel
 
FINAL DIAGNOSIS ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS
TTP vs HUS
D+ vs. D-
Oklahoma TTP-HUS Registry ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.
Treatment
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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A Case of Atypical Hemolytic Uremic Syndrome

  • 1. PROF DR K H NOOR UL AMEEN M5 UNIT DR.RAKESH PINNINTI CASE OF JAUNDICE
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  • 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
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  • 16. ONLINE REFERENCE Fibrin thrombi & RBC in renal capillaries Subintimal fibrin, but no inflammation Healing from prior fibrinoid injury, occluding vessel
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  • 18. FINAL DIAGNOSIS ATYPICAL HEMOLYTIC UREAMIC SYNDROME / D-HUS
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  • 23. Disseminated intravascular coagulation is not typically present, but may be seen when there is diffuse tissue ischemia.
  • 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