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

[CIS-PIDD]

Hi Dr. Kumar,

I would consider doing sputum cx looking for Campylobacter species such Helicobacter and Flexispira (although more in GI, they have been reported in the lung in XLA), they require selective media ( blood/blood products or egg yolk emulsion) to grow on, and it grows slowly .Sometimes it will be only detected by PCR. Below is a reference for your consideration
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3165598/

Good luck!
Joud

Joud Hajjar MD
Assistant Professor of Medicine
Service Chief, Adult Allergy and Immunology
Baylor College of Medicine and Texas Children's Hospital
Section of Immunology, Allergy & Rheumatology
joud.hajjar at bcm.edu
 
 
1102 Bates FC 330
Houston, TX 77303
Office: 832-824-2595
Fax :   832-825-1260
 

On 4/13/17, 10:48 AM, "cis-pidd at lyris.dundee.net on behalf of CIS-PIDD" <cis-pidd at lyris.dundee.net on behalf of 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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