[CIS PIDD] [cis-pidd] AW: SCIG or IVIG in Netherton syndrome?

Tue Jan 10 00:58:02 EST 2017

Dear Hans,

thank you for your response. I certainly appreciated tha 2009 JACI article of your group.
We have the same impression that IVIG and SCIG ameliorate the disease severity. However, at least to me the mechanism behind this is not clear.
As Netherton syndrome tends to improve with age I am wondering how to proceed in adolescents and AYAs. Should we just stop adjusting the dosage to their body weight?

Regards, Fabian

Von: Ochs, Hans [mailto:hans.ochs at seattlechildrens.org]
Gesendet: Montag, 9. Januar 2017 22:16
An: CIS-PIDD
Cc: Hauck, Fabian Dr. Dr.med.; Seppänen Mikko
Betreff: RE: SCIG or IVIG in Netherton syndrome?

Fabian,
The immune aberrations in Netherton syndrome were evaluated by Ellen Renner, while here in Seattle.
JACI 2009
While IVIG had clearly a positive effect on the management of Netherton, it did not cure the disease.
We recommend IVIG (or SCIG) for affected children which makes the management of these patients much easier.
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

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From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Monday, January 09, 2017 5:23 AM
To: CIS-PIDD
Subject: [cis-pidd] VS: SCIG or IVIG in Netherton syndrome?

Netherton belongs to Finnish Disease Heritage PIDs like CHH, APECED, AICDA deficiency.
We have started it now to I think three individuals (the last I heard). We yet have no long-term follow up information.

Patients thus far seem to subjectively benefit from it, but they are actually mainly followed up by our dermatologists.

Fabian, I will connect you with our dermatologists in charge of this, outside Listserv, you may get more precise info from them. AFAIK, all get IVIG, Netherton skin makes one hesitant to use SCIG.

ATB

Mikko

Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 9. tammikuuta 2017 14:56
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Aihe: [cis-pidd] SCIG or IVIG in Netherton syndrome?

Dear All,

I would like to ask your opinion and/or experience in treating patients with Netherton syndrome with s.c. and/or i.v. immunoglobulin. Published data is scarce and I can not find strong evidence to do so.

Thank you in advance, Fabian

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

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