[CIS PIDD] [cis-pidd] 14yo with recurrent Staph aureus sepsis

Tue Jun 10 10:27:58 EDT 2014

Dear Joe and Mikko, 

The clinical picture is not compatible with IRAK4/MYD88 deciciency.
These two disorders present seriously in the early ages and the
infections became less frequent and less severe with advancing age. 

Yours, 

nima 

n 2014-06-10 18:19, Church, Joseph wrote: 

> Thanks, Mikko. Great suggestions. JC
> 
> -----Original Message-----
> From: Seppänen Mikko [mailto:Mikko.Seppanen at hus.fi] 
> Sent: Monday, June 09, 2014 9:26 PM
> To: CIS-PIDD
> Subject: [cis-pidd] VS: 14yo with recurrent Staph aureus sepsis
> 
> Dear Joe, 
> 
> I have not noticed any responses suggesting IRAK4/MyD88 (was cellulitis necrotizing?), nor congenital or acquired asplenia/ hyposplenism, I would also consider hypomorphic SGD (segmentation of neutrophils on blood smear?). And due to a mystery patient of ours, I suggest You check mitogen stimulation tests if everything else fails, however crazy it may sound. And cyclic neutropenia seemes a bit far-fetched due to Staph always and late-onset??
> 
> I would do the IRAK4/MYD88 functionals in JL Casanova's lab, 
> 
> .... and if neutrophils hyposegmented, please contact me as well. 
> 
> Outside PIDDs, are the strains checked against PVL production, a carrier? 
> 
> Yours, back from holiday
> 
> Mikko Seppänen, Finland
> 
> ________________________________________
> Lähettäjä: Church, Joseph [JChurch at chla.usc.edu]
> Lähetetty: 3. kesäkuuta 2014 2:54
> Vastaanottaja: CIS-PIDD
> Aihe: [cis-pidd] 14yo with recurrent Staph aureus sepsis
> 
> Colleagues:
> 
> I was asked to evaluate a previously very healthy 14yo boy who experienced the following:
> 
> · 2012 Cellulitis (likely Staph) after an insect bite
> 
> · 2013 Staph aureus, methicillin sensitive, septic shock after minor skin trauma and possible local infection
> 
> · 2014 Staph aureus, methicillin sensitive, sepsis.
> 
> He has a past history of a 4mm VSD, but echocardiography on two occasions failed to reveal a focus of infection.
> 
> Neutrophil counts were repeatedly normal.
> 
> Physical exam is normal except for a III/VI ejection murmur.
> 
> I have never read that CGD or LAD presents like this. I plan to suggest a closer look at his heart valves. Any other suggestions?
> 
> Joe Church
> Children's Hospital Los Angeles
> 
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-- 

Nima Parvaneh, MD.
Assistant Professor
Department of Pediatrics
Division of Allergy and Clinical Immunology
Tehran University of Medical Sciences
62 Gharib St, 14194 Tehran, Iran
Tel: +98-21-66920982
Fax: +98-21-66923054
Email: nparvaneh at tums.ac.ir

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