[CIS PIDD] [cis-pidd] C8 Deficiency

[Locke, Bradley]

Due to the autosomal recessive nature of the disease, you may want to consider a screening CH50 in the patient's siblings and children.
We have previously found asymptomatic/healthy siblings to also have late complement deficiencies after diagnosing the index case.
The resultant diagnosis may help with future infection management and also with future family planning.

Bradley Locke, DO
Medical College of Wisconsin
Allergy/Clinical Immunology

From: Sullivan, Kathleen [mailto:sullivak at mail.med.upenn.edu]
Sent: Tuesday, July 30, 2013 12:45 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] C8 Deficiency

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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