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

[CIS-PIDD]

I would perform directed cultures and aspiration for bacteria, fungi and mycobacteria, preferably sent to National Jewish or Mayo.  You may also want to look at the microbiome through Pathogenius in Dallas.  Even if the organism is sensitive to azithromycin, there may be resistant organisms under a biofilm.  You may want to consider IV antibiotics.  Is he using an electronic nasal wash device (Sinugator or Hydropulse) which will prevent backflow and recontamination?

Did they evaluate his adenoids before the FESS?  Current protocol in kids is to do an adenoidectomy and sinus aspiration prior to FESS.

Sincerely,

Wellington S. Tichenor, MD, FACP, FAAAI
642 Park Avenue
New York, New York 10065
212 517-6611
www.sinuses.com
wtichenor at sinuses.com
________________________________________
From: cis-pidd at lyris.dundee.net [cis-pidd at lyris.dundee.net] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Thursday, April 13, 2017 11:48 AM
To: CIS-PIDD
Subject: [cis-pidd] XLA, Bronchiectasis, Chronic sinsusitis

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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