[CIS PIDD] [cis-pidd] XLA, Bronchiectasis, Chronic sinsusitis

[CIS-PIDD]

Hi, 

Interventions I have found helpful in patients with XLA and bronchiectasis are
1/chronic azithromycin therapy (10 mg/kg 3x/week) plus physio twice daily with PEP mask - of course I don't know what sputum culture shows?  Even if the sinus Hemophilus is not sensitive to azi the anti-inflammatory effect can be useful. 
2/you could try to use soms scig preparation as eye drops but I think the antibiotics will be more efficaceous.

Best wishes,

Isabelle Meyts
UZ Leuven





> Op 13-apr.-2017 om 18:02 heeft CIS-PIDD <cis-pidd at lists.clinimmsoc.org> het volgende geschreven:
> 
> Hi Everyone,
> Any thoughts regarding this case is greatly appreciated.
>  I have a now 8-year-old male with XLA (BTK gene mutation confirmed) on IVIG replacement.  He has bronchiectasis in the lower lobes, chronic rhinosinusitis status post sinus surgery x 1. His trough IgG level have been >800mg/dl. 
> 
> My questions are:
> 1. Pt has had chronic cough likely related to his bronchiectasis and his chronic rhinosinusitis and we have been unsuccessful in treating it in spite of doing chest physical therapy, asthma controller medication, sinus surgery, saline rinse, Tobramycin rinse. Pt has been treated with multiple courses of antibiotics in the past with no significant relief of symptoms. ENT is recommending one more sinus surgery but I am hesitant as the first one did not help. His direct sinus cultures have not yielded much except for occasional scant growth of H.Influence.  Anything else we could do to help this little guy.
> 
> 2. He also has this weird history of yellow mucus coming from his eyes on and off with evidence of conjunctival injection. This discharge is more visible when he sneezes and coughs. He was checked by an ophthalmologist who ruled out lacrimal duct obstruction. ENT thinks this is related to his chronic sinusitis and the constant yellow drainage he has in his nose. Any thoughts?
> 
> Thank you,
> 
> Santhosh Kumar, MD, FAAAI
> Assistant Professor
> Children's Hospital of Richmond at VCU
> VCU Heath
> Richmond, VA
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