Update From The XLHED Dog World!

Margret Casal, D.V.M., Ph.D.
University of Pennsylvania

In previous treatment trials in the ectodermal dysplasia (XLHED) dogs, we had shown that a one-time treatment at birth with recombinant EDA (Fc:EDA) resulted in restoration of the adult teeth and some improvement of the clinical signs.  However, the majority of clinical problems associated with XLHED in humans is caused by the lack of eccrine, sweat, bronchial, and lacrimal glands resulting in hyperthermia, frequent pneumonia and dry-eye.  Therefore, the recovery of these glands is the primary goal of treatment.  We had proposed to use higher doses of Fc:EDA in affected dogs and were subsequently able to show that dogs thus treated had correction of dentition, normal respiratory function, normal tear production, and the ability to sweat.   We were also able to show that treatment instituted earlier in life resulted in better correction.  MORE

The generous funding provided by the NFED has been supporting the long term evaluation and maintenance of the treated dogs.  Overall, there is significant correction of dental, ocular, and respiratory disease in most of the dogs.  The oldest is now four years old and there has been no change in the corrected features of XLHED, indicating a long lasting stable cure in all of the dogs thus far treated. Interestingly, some of the treated dogs did not show improvement in dentition but still developed the ability to sweat, had normal tear production, and developed respiratory glands.  Even partially effective treatment can improve the quality of life as none of these dogs developed pneumonia or any of the other clinical signs associated with XLHED.

Together with Pascal Schneider and Olivier Gaide, we are currently working with Edimer Pharmaceuticals to bring this treatment one step closer to humans with XLHED.

 

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