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

[CIS-PIDD]

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 responsein 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 CheginiExton, PA

 
 
  On Sat, Dec 9, 2017 at 6:01 PM, CIS-PIDD<cis-pidd at lists.clinimmsoc.org> wrote:    <!--#yiv7771276144 _filtered #yiv7771276144 {font-family:"Cambria Math";panose-1:2 4 5 3 5 4 6 3 2 4;} _filtered #yiv7771276144 {font-family:Calibri;panose-1:2 15 5 2 2 2 4 3 2 4;}#yiv7771276144 #yiv7771276144 p.yiv7771276144MsoNormal, #yiv7771276144 li.yiv7771276144MsoNormal, #yiv7771276144 div.yiv7771276144MsoNormal {margin:0cm;margin-bottom:.0001pt;font-size:12.0pt;font-family:"Calibri", sans-serif;}#yiv7771276144 a:link, #yiv7771276144 span.yiv7771276144MsoHyperlink {color:#0563C1;text-decoration:underline;}#yiv7771276144 a:visited, #yiv7771276144 span.yiv7771276144MsoHyperlinkFollowed {color:#954F72;text-decoration:underline;}#yiv7771276144 span.yiv7771276144EmailStyle17 {font-family:"Calibri", sans-serif;color:windowtext;}#yiv7771276144 span.yiv7771276144msoIns {text-decoration:underline;color:teal;}#yiv7771276144 .yiv7771276144MsoChpDefault {font-family:"Calibri", sans-serif;} _filtered #yiv7771276144 {margin:72.0pt 72.0pt 72.0pt 72.0pt;}#yiv7771276144 div.yiv7771276144WordSection1 {}-->
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 
 
  
 
NOTICE: This email message is for the sole use of the intended recipient(s) and may contain confidential and privileged information. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply email and destroy all copies of the original message.  ­­  
 ---
 
 You are currently subscribed to cis-pidd as: schegini at yahoo.com.
 
 To unsubscribe click here: http://cts.dundee.net/u?id=96396879.1c4ba390e5cba8b441ad14c59c207858&n=T&l=cis-pidd&o=4695163
 
 (It may be necessary to cut and paste the above URL if the line is broken)
 
 or send a blank email to leave-4695163-96396879.1c4ba390e5cba8b441ad14c59c207858 at lyris.dundee.net
  

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=4695241
or send a blank email to leave-4695241-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20171210/5ab431f7/attachment-0001.html>