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

[CIS-PIDD]

  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>:

> 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
>
> Fax: +49(0)761 270 45990
>
> www.cci.uniklinik-freiburg.de
>
>
>
>
>
> ---
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-- 
Saúl Oswaldo Lugo Reyes

Investigador en Ciencias Médicas
Unidad de Investigacion en Inmunodeficiencias
9° piso, Torre de Investigacion,
Instituto Nacional de Pediatria
Coyoacan 04530 DF, Mexico
+52(55)10840900 ext. 1866
--------------------------------------
"La penicilline guérit les humains; le vin les rend heureux"
"If penicillin cures sicknesses, Jerez resuscitates dead people". -Sir
Alexander Fleming

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