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

[Giclas, Patsy]

I haven't seen a C5 induced MPGN.  What proteins were in the biopsy?  
Did you look for C3 nephritic factor?  AH50?   What was C3 level and function?

best regards,
Patsy Giclas

Patricia C. Giclas, PhD
Professor, Pediatric Allergy and Immunology
Director, Diagnostic Complement Laboratory
Advanced Diagnostic Laboratories
National Jewish Health
Denver, CO, 80206

office:  303-398-1217
fax:      303-270-2128
giclasp at njhealth.org
________________________________________

From: Church, Joseph [JChurch at chla.usc.edu]
Sent: Thursday, June 27, 2013 7:19 PM
To: CIS-PIDD
Subject: [cis-pidd] 14yo girl with MPGN and C5 deficiency

Colleagues:e sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution 
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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