[CIS PIDD] [cis-pidd] <no subject>

[CIS-PIDD]

This is a difficult problem indeed.
I would suggest to pursue all potential routes to prove that in fact there IS an infectious agent involved eg panmicrobial PCR, panfungal PCR (although less likely to be fungal in AT), PCRs for detection of viral genomes,  all necessary cultures etc etc.
Also: did the child receive live vaccines? (Cfr Bodemer C et al) or did he/she have chickenpox (De Somer L et al)?
To what extent is the child immunodeficient at this stage?
Any signs of bone marrow trouble?
Most therapies could do more harm than good but in my opinion after relevant cultures IVIG is a good idea as are empirical broadspectrum antibiotics. Personally would refrain from anti-TNF and opt for systemic steroids as first choice if necessary (short term)... Maybe then thalidomide or another immunomodulating drug?


Isabelle Meyts
UZ Leuven
Belgium




Op 24-mrt.-2016 om 18:39 heeft CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> het volgende geschreven:

I am seeing a 2 year old with with newly discovered ataxia telangiectasia and speeding non-infectious cutaneous granulomas, well described in AT but little success in therapy.
Reports of systemic steroids, high dose topical, anti- TNF not entirely beneficial.
Other thoughts?

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