[CIS PIDD] [cis-pidd] Recurrent Fx in AD HIES - how to manage?

[CIS-PIDD]

I know of patients (incl. children) put on bisphosphonates who tolerated this well, but I am not aware of the outcome... and I do not know of any controlled trial.
Maybe Alexandra Freeman from the NIH knows?
Best, Bodo

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

Scientific-Director
CCI-Center for Chronic Immunodeficiency
UNIVERSITÄTSKLINIKUM FREIBURG
Tel.: 0761 270-77731  Fax: -77744
Breisacherstraße 115, 79106 Freiburg
bodo.grimbacher at uniklinik-freiburg.de
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
www.centreforimmunodeficiency.com

Von: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Wednesday 22 June 2016 19:33
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Betreff: [cis-pidd] Recurrent Fx in AD HIES - how to manage?

Dear colleagues,

I'd appreciate your advice on a 8 yr old girl with AD HIES (w/ STAT3 LOF mutation in SH2 domain) and recurrent bone fractures (5 in the past 3 yrs including olecranon, femur, several metatarsal bones) following minimal trauma. Calcium and Vitamin D are supplemented and (as well as phosphorus) within normal range. Densitometry scan showed a z-score of -2,4.

In general, how do you manage recurrent bone Fx in your AD HIES patients?
Does anyone have experience exceeding Sowerwine et al. http://www.ncbi.nlm.nih.gov/pubmed/24402620or Schuermann et al. http://www.ncbi.nlm.nih.gov/pubmed/23606327?
Would you recommend bisphosphonates in this case?

Best regards,
   Christian Klemann



Dr. med. Christian Klemann
UNIVERSITÄTSKLINIKUM FREIBURG
Zentrum für Kinderheilkunde und Jugendmedizin CCI - Centrum für Chronische Immundefizienz
Mathildenstr. 1
79106 Freiburg
Telefon: +49(0)761 270 43010
Fax: +49(0)761 270 45990
www.cci.uniklinik-freiburg.de<http://www.cci.uniklinik-freiburg.de/>



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