[CIS PIDD] [cis-pidd] symptomatic C3Nef in otherwise healthy child

[Nicole Chase]

Hi Esther,

During fellowship, I helped care for a patient who developed meningococcal
meningosepsis at age 18 (also with serogroup Y), with no prior history of
infection, renal disease, or lipodystrophy. He was ultimately found to have
a C4Nef. To my knowledge, he has done very well in the 2+ years since his
initial presentation, with no new symptoms or serious infections. He has
antibiotics with him to begin for signs/symptoms of illness.

His case is currently in press in Clinical Immunology. Drs. Elizabeth
Miller and John Atkinson from Washington University in St. Louis performed
the diagnostic experiments, and are cc'd on this reply. I believe that they
did find evidence of a C3-NeF (low titer positive), and this was attributed
to the presence of the C4Nef. (It is my understanding that patients who are
diagnosed with a C3Nef may actually have a C4Nef, as this is not routinely
screened for.)

Interestingly, our patient also had evidence of poor antibody response to
polysaccharide antigens (eg, 2/23 pneumococcal serotypes post-vaccination,
1/4 meningococcal serotypes post-vaccination). I believe he is currently
receiving yearly PPV23, MCV4, and HiB vaccines. (I cannot recall if we ever
gave him PCV-13, based on his poor polysaccharide response.) It would be
interesting to know if your patient had any similar findings.

Cheers,
Nicki Chase

Nicole Chase, MD
Midwest Immunology Clinic
Plymouth, MN

On Fri, Oct 19, 2012 at 3:18 AM, Dr. Esther de Vries
<esid at estherdevries.nl>wrote:


> I follow a 7-year-old girl who since 1.5 years has had serious infectious

> problems (meningitis meningococcus serogroup Y, 2x pneumonia, recurrent

> otitis media), but was previously healthy. Investigation showed strongly

> reduced C3, normal C4 and strongly increased C3d on several occasions. C3

> nephritic factor (C3Nef) was repeatedly found. She has no renal disease,

> she also did not develop this during follow-up, no lipodystrophy, and no

> other signs of auto-immunity. Her family history is unremarkable. She is

> now stable after vaccinations and being on antibiotic prophylaxis.

>

> My question is: do you have patients like this? Any ideas as to prognosis?

>

> Happy to receive your input! Thanks, Esther de Vries, Jeroen Bosch

> Hospital, 's-Hertogenbosch, the Netherlands.

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