[CIS PIDD] [cis-pidd] AT and rubella lesions

Fri Dec 2 10:00:45 EST 2016

Mikko:  you indicate IFNG does not work.  Kate:  did you try IFNG or IFNA?

Were these given systemically or via local injection?

Alternatively, how about topical imiquimod or imiquimod + steroid?

Joe Church
Children's Hospital Los Angeles

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Friday, December 02, 2016 6:36 AM
To: CIS-PIDD
Subject: Re: AW: [cis-pidd] AT and rubella lesions

Hi Hans!

Like Kate already replied, we suspect that nitazoxanide may work, but we have very limited experience (mostly from my colleague Timo Hautala's patient).

If anyone attempts, I suggest first contact Kate to test this (with Ludmila Perelygina, Stanley Plotkin).

We do need formal proof...

IFNG does not work. Again reiterating what Kate already noted.

ATB,

Mikko

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)
[X]

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 2.12.2016 kello 16.19:
Dear Colleagues,

thank you very much for your important considerations and suggestions. I will keep you posted !

Kind regards, Fabian

Von: Ochs, Hans [mailto:hans.ochs at seattlechildrens.org]
Gesendet: Donnerstag, 1. Dezember 2016 01:21
An: CIS-PIDD
Cc: Ochs, Hans (UW-External); Hauck, Fabian Dr. Dr.med.
Betreff: RE: [cis-pidd] AT and rubella lesions

Dear Fabian,

You must have read Kate Sullivan's paper just published: Perelygina L, Plotkin S, Russo P, Hautala T, Bonilla F, Ochs HD, Joshi A, Routes J, Patel K, Wehr C, Icenogle J, Sullivan KE.

J Allergy Clin Immunol. 2016 Nov;138(5):1436-1439.e11

We had an AT patient with extensive lesions, identified by the pathologist as "Sweets syndrome". She died of a malignancy at the age of 7 years.

Her immune deficiency was severe, affecting both cellular and humoral immunity. She had pronounced elevation of IgM, low IgG and IgA.

Very poor antibody to phage immunization.

We found out about the presence of Rubella  in the skin lesions years after hear death.

We tried high dose IVIG, steroids locally and for short intervals systemically, antibiotics,

But never gave antivirals (which were not available in limited types in the early 2000th.

Since IVIG does not make it well into the skin, I would now, knowing the etiology, try soaking

a lesion with IgG, e.g. IVIG or Hizentra, using an occlusion wrap, just to see if neutralizing antibodies in

sufficient concentration would make a difference. If improvements, use local treatment for the rest of the lesions,

if possible.

I do not know if any of the known antivirals would work.

HSCT is not the treatment of choice for obvious reasons, but the Munich group has moe experience with NBS

Than anybody else, except perhaps the Moscow group.

The best

hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

206-987-7450 OFFICE
206-987-7310  FAX
allgau at u.washington.edu<mailto:allgau at u.washington.edu>, hans.ochs at seattlechildrens.org<mailto:hans.ochs at seattlechildrens.org>

OFFICE   1900 Ninth Avenue, Seattle, WA 98101
MAIL      M/S C9S-7
WWW     seattlechildrens.org<http://seattlechildrens.org/>

Assistant:
Dawn Marie Pares
206-884-7418   OFFICE
dawnmarie.pares at seattlechildrens.org<mailto:dawnmarie.pares at seattlechildrens.org>

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Tuesday, November 29, 2016 10:33 PM
To: CIS-PIDD
Subject: AW: [cis-pidd] C4 deficiency, Cryoglobulinemia and Hypogammaglobulinemia

Dear colleagues,
I am following a seven year old patient with ataxia teleangiectasia and skin granuloma positive for rubella vaccine virus. Granuloma slowly increase in size and significantly contribute to reduced quality of life. Parents are at present not considering hematopoetic stem cell transplantation as a therapeutical option. Does anyone have experience in treating these granulomas with e.g. antiviral drugs or interferons?
Yours sincerely,
Fabian Hauck

Fabian Hauck, MD, PhD

Attending physician / Head Immunodeficiency Unit and Immunological Diagnostics Laboratoy
Pediatrics / Pediatric Hematology and Oncology / Immunology (DGfI)

Dr. von Hauner Children's Hospital
Klinikum der Universität München
Lindwurmstr. 4, 80337 München
Germany

Tel.: +49 89 4400-53931
Fax: +49 89 4400-53964
E-Mail: fabian.hauck at med.uni-muenchen.de<mailto:fabian.hauck at med.uni-muenchen.de>

www.klinikum.uni-muenchen.de<https://urldefense.proofpoint.com/v2/url?u=http-3A__www.klinikum.uni-2Dmuenchen.de_&d=DgMGaQ&c=aBkXpkKi7gN5fe5MqrMaN-VmRugaRb1IDRfSv2xVRy0&r=dqvYsOKBGq1fSB6Xe4T-fVy9dlGu-SpUWxzWv4ubA8k&m=aa1X__sR-DCv7qfOegu5CjTr4paVBg4UjknTea4YFig&s=nQYyByvV14DqEpZRZyJrD8E-gs_b2zxbTRWTWA3iu14&e=>

<image001.png>

Das Klinikum der Universität München ist eine Anstalt des öffentlichen Rechts (AöR)
The Klinikum der Universität München is an Institution under Public Law

---

You are currently subscribed to cis-pidd as: allgau at u.washington.edu<mailto:allgau at u.washington.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396380.ac5fb155d16e66ae9ff84c9ecd80407d&n=T&l=cis-pidd&o=4030870<https://urldefense.proofpoint.com/v2/url?u=http-3A__cts.dundee.net_u-3Fid-3D96396380.ac5fb155d16e66ae9ff84c9ecd80407d-26n-3DT-26l-3Dcis-2Dpidd-26o-3D4030870&d=DgMGaQ&c=aBkXpkKi7gN5fe5MqrMaN-VmRugaRb1IDRfSv2xVRy0&r=dqvYsOKBGq1fSB6Xe4T-fVy9dlGu-SpUWxzWv4ubA8k&m=aa1X__sR-DCv7qfOegu5CjTr4paVBg4UjknTea4YFig&s=U-f2hGqkGtS-rvTG27CqlYvMaOqHcAsxFmoFRlvWzQw&e=>

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

or send a blank email to leave-4030870-96396380.ac5fb155d16e66ae9ff84c9ecd80407d at lyris.dundee.net<mailto:leave-4030870-96396380.ac5fb155d16e66ae9ff84c9ecd80407d at lyris.dundee.net>
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information protected by law. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message.

---

You are currently subscribed to cis-pidd as: mikko.seppanen at hus.fi<mailto:mikko.seppanen at hus.fi>.

To unsubscribe click here: http://cts.dundee.net/u?id=99266512.00d254228cd4b291924022bf56fac1f0&n=T&l=cis-pidd&o=4036881

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

or send a blank email to leave-4036881-99266512.00d254228cd4b291924022bf56fac1f0 at lyris.dundee.net<mailto:leave-4036881-99266512.00d254228cd4b291924022bf56fac1f0 at lyris.dundee.net>

---

You are currently subscribed to cis-pidd as: jchurch at chla.usc.edu<mailto:jchurch at chla.usc.edu>.

To unsubscribe click here: http://cts.dundee.net/u?id=96396658.df9245d5219d19fc82ac9f3bbf52c836&n=T&l=cis-pidd&o=4036918

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

or send a blank email to leave-4036918-96396658.df9245d5219d19fc82ac9f3bbf52c836 at lyris.dundee.net<mailto:leave-4036918-96396658.df9245d5219d19fc82ac9f3bbf52c836 at lyris.dundee.net>

---------------------------------------------------------------------
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, 
is for the sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution is prohibited. If you are not the intended recipient, please
contact the sender by reply e-mail and destroy all copies of this original message.  

---------------------------------------------------------------------

---
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=4037001
or send a blank email to leave-4037001-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20161202/cb108c7a/attachment-0001.html>