[CIS PIDD] [cis-pidd] AW: Dock8 and IFN treatment

[CIS-PIDD]

Sorry for this translational error:

It might be an aciclovir-resistant H.simplex (lacking thymidinkinase)
Foscarnet should be tried
Best, Bernd

Bernd H.Belohradsky
University Childrens Hospital Muenchen

Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Gesendet: Donnerstag, 1. Oktober 2015 10:17
An: CIS-PIDD
Betreff: [cis-pidd] AW: Dock8 and IFN treatment

Lieber Bodo,
es könnte ein Aciclovir-resistenter H.simplex sein (Speziallaboratorien bestimmen in diesen Fällen das Fehlen der viralen Thymidinkinase, die das Aciclovir in die Viruszelle transportieren soll));
dann mußt Du Foscarnet nehmen; wir hatten das gleiche Problem bei einem DOCK8-Kind vor KMT; Foscarnet hat das akute Problem gelöst
Kinderdosisschema:  2 Wochen 120 mg/kg/Tag in 3 ED; dann Erhaltungstherapie 60-120 mg/kg/Tag in 1 ED

Beste Grüsse, Bernd

Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Gesendet: Donnerstag, 1. Oktober 2015 09:26
An: CIS-PIDD
Cc: Dr. Julius Wehrle
Betreff: [cis-pidd] Dock8 and IFN treatment

Dear all,
Does somebody have experience with IFN treatment in Dock8 deficiency?
I follow a 47 yr-old patient with Dock8 deficiency.
I know, he is probably one of the eldest Dock8 patients alive, possibly as he has some very little residual Dock8 protein expression due to his specific splice site mutation in Dock8.
However, he has severe skin eczema and completely  lost vision on his left eye and has only some residual visibility on his right eye following herpes keratitis and CMV retinitis.
But are barely controlled under aciclovir plus valganciclovir treatment, giving him elevated liver values.
So with his eye-sight, we are with the back to the wall
My question is now:
Does somebody have experience with IFN treatment in Dock8 deficiency?
I know it has been successfully tried for skin lesions, but has anybody ever used it for viral infections of the eye?
Would you expect a worsening of the inflammation and thereby loss of vision?
He also has a cornea transplant on his right eye...
Any other idea?
Best,  Bodo

****************************************
Univ.-Prof. Dr. med. B. Grimbacher

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Engesserstraße 4, 79108 Freiburg
bodo.grimbacher at uniklinik-freiburg.de<mailto:bodo.grimbacher at uniklinik-freiburg.de>
www.uniklinik-freiburg.de/cci<http://www.uniklinik-freiburg.de/cci>

and

Consultant Immunologist
Institute of Immunity & Transplantation
Dept of Immunology
Royal Free Hospital
UNIVERSITY COLLEGE LONDON
Pond Street
London NW3 2QG
b.grimbacher at ucl.ac.uk<mailto:b.grimbacher at ucl.ac.uk>
www.centreforimmunodeficiency.com<http://www.centreforimmunodeficiency.com>


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