[CIS PIDD] [cis-pidd] A case of chronic cough

Tue May 8 15:06:06 EDT 2018

Hi Kelli,

Nasal nitric oxide might be something to consider if you are suspecting PCD, sweat test?
Joud

Joud Hajjar MD, MS.
Assistant Professor of Medicine
Service Chief, Adult Allergy and Immunology
Baylor College of Medicine and Texas Children's Hospital

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Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Tuesday, May 8, 2018 at 1:51 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: [cis-pidd] A case of chronic cough

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Looking for input on a 7yo Chinese girl that I saw last month for chronic cough. Highlights of the case:
-Adopted from China at age 2.5 years, unknown family history, patient had tetralogy repair at age 13 months
-FTT with normal metabolic and endo w/u
-Chronic wet cough started at age 6, just after starting kindergarten. Prior to me, she had Pulm, GI, ENT, Speech and Allergy evaluations. Within the last year she was treated with albuterol without improvement, inhaled corticosteroid without improvement, 5-10 day courses of multiple antibiotics without significant improvement. Only thing that “helped a little” was a 21 day course of augmentin with prednisolone. Cough returned 3 weeks after off antibiotics
-Chest CT revealed focal airway trapping in the LLL with some linear scarring nearby
-Cough persisted so she was bronched and had + H. Flu, S. pneumo, and moraxella. Lipid laden macrophages were also detected
-MBSS that identified laryngeal penetration without aspiration, retained esophageal products
-We just did a ciliary biopsy due to concern for PCD and there were very few cilia identified and the ones that were there had complete degeneration with evidence of autolysis, unable to comment on dynein arm structure
-Normal cytogenetics and microarray
-Igs normal with adequate protection for Diphtheria, Hib and Tetanus. I just boosted her with pneumovax (7/23 prevaccine)
-CD3, CD4, CD8, CD19 normal with markedly elevated CD56/16 (>1000)
-EBV and CMV negative
-No history of ear infections, pneumonias, bronchitis, sinusitis, skin infections, skin abscesses, fungal infections or cutaneous viral infections
-She is up to date on her vaccines, received multiple live virus vaccines without any adverse reactions or subsequent infection

Not sure what to make of the markedly elevated NK cells in the context of her chronic cough. At CIS it was mentioned to look for a clonal population. I am planning to get more expanded flow when she comes back to see me later in the week. Planning to send genetics (including for PCD) once I get a better handle on her immune phenotype. Anyone have other thoughts re: her rare and degenerated cilia? Just chronic inflammation? Or should I be worried about something else when I hear this.

Thanks for your input!
Kelli
--
Kelli W. Williams, M.D., M.P.H.
MUSC Carolina Family Care
Allergy & Immunology

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