[CIS PIDD] [cis-pidd] <no subject>

[CIS-PIDD]

He has had several courses and remains on azithromycin.
Thank you for your interest.

Erwin W. Gelfand, M.D.
Department of Pediatrics
National Jewish Health
1400 Jackson Street
Denver, CO 80206
Ph: 303-398-1196
Fax: 303-270-2105
E-mail: gelfande at njhealth.org<mailto:gelfande at njhealth.org>


From: <cis-pidd at lyris.dundee.net> on behalf of CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Reply-To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Date: Saturday, 9 December 2017 at 20:22
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Subject: Re: [cis-pidd] <no subject>

Dr. Gelfand,
I apologize for my ignorance and admit that I just looked up MST1, of which until now I knew nothing. Still, I might be able to help with an empiric approach to treatment.

I have had the same scenario in 3 of my patients. One of those had intestinal lymphangiectasia, and in the other two I have not been able to pinpoint the underlying defect that resulted in their susceptibility to pseudomonas or tie it to any concrete defect in their immune function. I attempted everything that could offer a promise extrapolating from CF. Unfortunately, that approach wasn't fruitful and chronic pseudomonas bronchitis with recurrent asthma exacerbations led to steroid dependence. As a last resort I attempted suppressive treatment with macrolides (azithromycin) based on the reports of their effectiveness in diffuse panbronchiolitis after all else had failed. This approach led to a dramatic response
in the first subject, who had IL, and could be replicated in the other 2 patients, who as of yet have no defined PID. I hope this will also help your patient, and would appreciate your feedback.


Best regards,
Soheil Chegini
Exton, PA





On Sat, Dec 9, 2017 at 6:01 PM, CIS-PIDD
<cis-pidd at lists.clinimmsoc.org> wrote:

I am following a 48 year old with extensive AD and asthma, who suffers from recurrent oral thrush (even off ICS) and pseudomonas in the lungs but without bronchiectasis. Immune studies have all been normal. Of interest he is a compound het for MST1 (Macrophage stimulation 1) with  C183F and P19SP mutations. Very little in the literature. This gene should not be confused with the other MST1 or STK4, which has been associated with immunodeficiency/lymphoproliferation.

Any thoughts most welcome.







Erwin W. Gelfand, M.D.

Department of Pediatrics

National Jewish Health

1400 Jackson Street

Denver, CO 80206

Ph: 303-398-1196

Fax: 303-270-2105

E-mail: gelfande at njhealth.org<mailto:gelfande at njhealth.org>



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