[CIS PIDD] [cis-pidd] 14yo girl with MPGN and C5 deficiency

[Church, Joseph]

Colleagues:

I have been asked to consult on a 14yo girl with membranoproliferative glomerulonephritis.  Complement studies show

-          CH50 0

-          C5 15mcg/ml (normal 55-113)

-          C5 function 28u/ml (normal 13,700-37,900)

-          Other complement levels were normal.

This appears consistent with a primary C5 deficiency and secondary MPGN.  However, MPGN has not to my knowledge been reported with C5 deficiency.

Has anyone seen this association?

Regarding therapy, any thoughts other than Meningococcal vaccine and prophylactic antibiotics?

Joe Church
Children's Hospital Los Angeles


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