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

[CIS-PIDD]

Hi, I usually do consider bisphophonates for these patients in  whom there is both osteoporosis and recurrent fractures.  We have used pamidronate (in the kids mostly), zoledronate and aledronate.  I do involved a pediatric endocrinologist with the children though as there are some risks if you continue the medications for too long as they need the normal bone remodeling as well. In addition, for girls, there is a risk of the bisphophoate coming out of the bone in later pregnancies.  For these reasons, i do like to involve endocrine.  It is hard to know how much impact there is from the bisphophonates, but for some of these with the really low BMD and fractures, seems to help.
Alexandra Freeman
NIH
________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Friday, June 24, 2016 1:53 AM
To: CIS-PIDD
Subject: Re: [cis-pidd] Recurrent Fx in AD HIES - how to manage?


  Dear Dr. Klemann,

    Tamara Staines and Luz Sanchez, in Monterrey, treated a school-age boy with STAT3-LOF who had sustained 5 low-energy fractures and developed osteonecrosis of the hip, with intravenous zoledronate (plus hip surgery), 0.05mg/kg, 3 doses over 18 months, with no adverse effects and very good response:3 years later, the patient has not suffered another fracture and walks unaided.

      I know this is not exactly first level evidence, but maybe you should try it.  Their case report is about to appear on Pediatric Blood & Cancer.

      I remain at your service for further details, or I can get you in touch with them.

    Best regards,

Saul.





2016-06-22 12:33 GMT-05:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>:
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/24402620 or 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<tel:%2B49%280%29761%20270%2043010>
Fax: +49(0)761 270 45990<tel:%2B49%280%29761%20270%2045990>
www.cci.uniklinik-freiburg.de<http://www.cci.uniklinik-freiburg.de/>



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--
Saúl Oswaldo Lugo Reyes

Investigador en Ciencias Médicas
Unidad de Investigacion en Inmunodeficiencias
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"If penicillin cures sicknesses, Jerez resuscitates dead people". -Sir Alexander Fleming

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