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

[Mel.Berger at cslbehring.com]

Can you determine in vitro if IVIG inhibits the activity of her nephritic factor (consumption of C3 in normal serum added to hers). If so, you will have a treatment which helps with the pathogenesis and helps to prevernt infection. If not, you could try suppressing the NeF production with Rituxin.  I have no personal clinical experience in this situation- just theoretical suggestions.

Mel Berger

________________________________________
From: João Farela Neves [jpfn13 at gmail.com]
Sent: Friday, October 19, 2012 10:18 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] symptomatic C3Nef in otherwise healthy child

Hi Esther

How is factor H/I? And AH50?

Elevated C3d with decreased C3 and normal C4 point to alternate
pathway activation with secondary C3 comsuption. This would go along
the clinical picture of recurrent infections, namely unusual
meningococcal serogroups.

Has, for sure, risk of atipical HUS and could benefit from early
eculizumab therapy.

Yours,
João Farela Neves
Primary Immunodeficiencies Unit
Hospital Dona Estefânia
Lisbon
Portugal

2012/10/19, Dr. Esther de Vries <esid at estherdevries.nl>:

> 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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