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

[CIS-PIDD]

Hi Dr Kumar, 
I would increase IVIG to have troughs > 1000 mg/dl. 
Best, 

Yesim Demirdag
Pediatric Allergy and Immunology
Columbia University Medical Center
New York 

Sent from my iPhone

> On Apr 13, 2017, at 11:48 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
> 
> 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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