[CIS PIDD] [cis-pidd] SCID?

[CIS-PIDD]

Dear colleagues,

I have been following a 5-month old male who had a positive newborn TREC screen for SCID (8 TREC and 16,600 actin), the following is a brief history:

Negative family of SCID; parents are non-consanguineous.
Initial results at one-month: CD3 = 322; CD4 = 283; CD8 = 31; NK = 527; B cells = 1274; IgG = 808; IgM = 35; IgA = 18.
At 5-month: CD3 = 338; CD4 = 297; CD8 = 31; NK = 145; B cells = 610; IgG = 185; IgM = 19; IgA < 7.
PHA blastogenesis normal.
RBC PNP/ADA levels normal.
Microarray normal.

Genetic testing at Genedx for T-B-NK+ panel (IL7R, CD3D, CD3E, CD3Z, IL2RG, JAK3, PNP, PTPRC, RMRP, ZAP70) negative; TBX gene also negative.

Cardiac echo normal.


Patient was given vaccinations Prevnar13, HIB and DTaP at 2-month and 4-month with great response: pneumococcal titers (went from zero to 10 serotypes > 1.5, ranging from 1.8 to 41), HIB (from < 0.15 to > 9); tetanus = 6.4.

Patient had cleft lip surgery without complication, seborrheic dermatitis on scalp and cow's formula intolerance currently on hydrolyzed formula, otherwise, continues to be healthy.

Any thoughts? Would you keep child on antibiotic prophylaxis and in isolation?


Thanks,

Peck Y. Ong, MD
Associate Professor
Division of Clinical Immunology and Allergy
Children's Hospital Los Angeles
pyong at chla.usc.edu<mailto:pyong at chla.usc.edu>





---------------------------------------------------------------------
CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, 
is for the sole use of the intended recipient(s) and may contain confidential
or legally privileged information. Any unauthorized review, use, disclosure
or distribution is prohibited. If you are not the intended recipient, please
contact the sender by reply e-mail and destroy all copies of this original message.  

---------------------------------------------------------------------


---
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=3196056
or send a blank email to leave-3196056-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20151010/986bb87c/attachment-0001.html>