[CIS PIDD] [cis-pidd] C8 Deficiency

[Sullivan, Kathleen]

He does not need any genetic testing.  It is sufficient to say he is C8 deficient.

The inheritance is always AR.

Prophylactic antibiotics are controversial and somewhat dependent on how easy it is for him to access health care and how risk averse your patient is.  Hypervacciantion with the meningococcal vaccine (every 5 years) can cut the risk to about 33% of the non-hypervaccinated risk

Kate
On Jul 30, 2013, at 1:02 PM, Mark Tucker wrote:


> Hello,

>  

> I have a 31 y/o male patient who had recent admission for meningococcal

> meningitis (serotype Y by PCR).  He has no history of recurrent

> sinopulmonary infections and no history of previous meningitis.  He had been

> vaccinated with meningococcal polysaccharide vaccine in 2010. 

>  

> On lab work up, during admission, he had low CH50 x 2, C3 and C4 were

> normal.  Subsequent labs demonstrated low C8 level of 4.8mg/dl performed at

> Quest Diagnostics.

>  

> He has since been immunized with pneumovax, prevnar and meningococcal

> vaccines as has his immediate family members.  He has been receiving

> prophylactic bicillin injections monthly.

>  

> My questions are:

> 1) Is further testing required in this individual concerning current

> diagnosis of C8 def?

> 2) Does anyone know of any common inheritance pattern?  Should children be

> screened?

> 3) Should he continue prophylactic antibiotics?

>  

> Appreciate any thoughts/guidance.

>  

> V/R,

>  

> Mark Tucker, MD

> CDR MC USN

> NMCSD Allergy / Immunology

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Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363



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