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Table 1 aHUS Cases Associated with Inflammatory Bowel Disease

From: Successful use of eculizumab to treat atypical hemolytic uremic syndrome in patients with inflammatory bowel disease

Reference #

n

Pt age

Pt gender

UC/CD

Creatinine improvement (Y/N)

TMA resolution (Y/N)

Ab/Mutation found?

Case Reports

 Beers [6] [abstract]

1

19

F

IBD**

Y

Y

CFH Auto-Ab & HZ CF1/CFH3 Del

 Webb [7]

1

16

M

UC

Y

Y

HZ MTHFR mutation***

 Viada Bris [8, 9]*

1

15

F

UC

NT

NT

HZ CFH, HZ MCP

 Green H [10]

1

27

F

UC

Y

Y

CFH Auto-Ab

 Hanna et.al. Case 1

(1/2)

19

M

CD

Y

Y

No clear mutation found****

 Hanna et.al. Case 2

(2/2)

49

F

UC

Y

Y

Testing not completed

  1. Ab antibody, CD Crohn’s disease, CF1 complement factor 1, CF3 complement factor 3, CFH complement factor H, HZ heterozygous, MCP membrane cofactor protein, MTHFR methylenetetrahydrofolate reductase gene/protein product, n number, N no, NT not treated, Pt patient, UC ulcerative colitis, Y yes. *8, 9 describe same case. ** IBD is only stated, it is not otherwise specified whether UC/CD.***Heterozygous MTHFR mutations are not generally thought to cause aHUS, therefore a known causative mutation was not found in Webb et.al. [7]. ****Early on in Case 1 a borderline positive C3 nephritic factor antibody was found and complement testing revealed a high level of soluble membrane attack complex
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Thrombosis Journal

ISSN: 1477-9560

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