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

Wed Jun 24 19:11:07 EDT 2015

We have one XLA patient who developed acute enteroviral encephalitis years ago just at the time of diagnosis - his brother had just been diagnosed and he got sick before we could evaluate him.  He was comatose - we treated with pleconaril under an emergency IRB/compassionate use agreement and high dose IVIG and he recovered.  He has not had any signs of CNS issues since that time.  Interestingly he also has undetectable MBL and developed Blastomyces pneumonia several years after being started on IVIG.

Unfortunately, as far as I know, pleconaril is no longer available.

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director

Division of Pediatric Allergy, Asthma & Immunology

University of Alabama at Birmingham

Tel: 205-939-9072

Fax: 205-975-7080

________________________________
From: Richard Wasserman [drrichwasserman at gmail.com]
Sent: Wednesday, June 24, 2015 4:30 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] XLA and enterovirus

Kate,
Negative PCRs. That patient was Diane Wara's who came to me with the reservoirs in place.
Richard

On Wed, Jun 24, 2015 at 4:00 PM, Sullivan, Kathleen <SULLIVANK at email.chop.edu<mailto:SULLIVANK at email.chop.edu>> wrote:
Rich- was the first patient you described completely cleared?

Kate Sullivan
Sent from my iPhone

On Jun 24, 2015, at 3:55 PM, Richard Wasserman <drrichwasserman at gmail.com<mailto:drrichwasserman at gmail.com>> wrote:

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<tel:214%20697-7211>

On Jun 24, 2015, at 9:40 AM, Gober, Laura M <GOBERL at email.chop.edu<mailto: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<tel:%28215%29%20590-2549>
fax (215) 590-4529<tel:%28215%29%20590-4529>

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--
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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