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

[CIS-PIDD]

Hi,
________________________________
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
9° piso, Torre de Investigacion,
Instituto Nacional de Pediatria
Coyoacan 04530 DF, Mexico
+52(55)10840900 ext. 1866
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"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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