[CIS PIDD] [cis-pidd] XLA and enterovirus

[Richard Wasserman]

I have cared for two XLA patients with Echo 11 encephalitis. The first
presented as a child and was treated with intraventricular IgG with an
Ommaya Reservoir until he developed obstruction and a lumbar reservoir was
added. This was 30 years ago and he was failing moderate dose IGIV. The
second presented as an asymptomatic adult and, based on the first
experience a Reservoir was placed but he had a bleed with the first dose
and was then treated IV. We were able to clear Echo from the spinal fluid
in both patients. If I saw such a patient today, I would probably start
with 1g/kg/w until there was a response. The second patient had done well
on 500mg/kg every other week and self weaned to every three weeks and
developed encephalopathy and seizure so we never actually cleared him even
though he was PCR negative.

Richard Wasserman
Dallas

Sent from my iPhone
Richard L. Wasserman, MD,PhD
214 697-7211

On Jun 24, 2015, at 9:40 AM, Gober, Laura M <GOBERL at email.chop.edu> wrote:

Hello,

I am currently caring for a 4 year-old boy with XLA who over the past year
has had progressive neurological deterioration including loss of
developmental milestones (no longer walking/talking).  Suspected etiology
has been enterovirus but prior testing has been negative (CSF, blood,
urine).  CSF has had elevated protein.  Has abnormal MIR results showing
progressive cerebral and cerebellar volume loss, subdural hemorrhages.



Had a brain biopsy yesterday that finally confirmed Enterovirus.  Besides
Pocapavir (already exploring this option), has anyone had any other
treatment strategies.  He has been therapeutic with Hizentra in past but
has been receiving higher doses of IVIg.  Has anyone used IVIg daily in
these patients with success?



And has anyone used Pocapavir in such a patient with chronic infection?



Thank you!

Laura

 Laura Gober, M.D.
Attending physician, Division of Allergy and Immunology
The Children's Hospital of Philadelphia
phone (215) 590-2549
fax (215) 590-4529

---

You are currently subscribed to cis-pidd as: drrichwasserman at gmail.com.

To unsubscribe click here:
http://cts.dundee.net/u?id=96396499.3449c9fd54f25f9fa0e022cb002b8c1e&n=T&l=cis-pidd&o=2932504

(It may be necessary to cut and paste the above URL if the line is broken)

or send a blank email to
leave-2932504-96396499.3449c9fd54f25f9fa0e022cb002b8c1e at lyris.dundee.net

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=2933862
or send a blank email to leave-2933862-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20150624/8e63adc1/attachment-0001.html>