[CIS PIDD] 3 month old Pneumocystis pneumonia, normal labs

[Ochs, Hans]

The rash you describe does not excite me as one typical for Job syndrome. You are correct, the NIH scoring system suggests that the typical rash for AD-HIES is eczematous and starts in the newborn period in most patients - and this is in fact true.
The elevated IgG, M, A may tell us something. What if this was not really a PCP, but the PJ found by PCR was just "normal colonization" not disease causing, since PCR picks up a bug or 2. It just happened that CGD was discovered serendipitously, some 60 years ago, in boys with hypergammaglobulinemia.
hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

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-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Karin Chen
Sent: Wednesday, September 05, 2012 10:56 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] 3 month old Pneumocystis pneumonia, normal labs


Good to know that rash can be minimal in AD HIES. I had always thought there would be at least some obvious eczematous rash presenting in the neonatal period.  The ulcerative lesion on the foot did not require antibiotics and on my exam her skin was totally clear, so I thought there was low likelihood of hyper IgE. I will check IgE and consider STAT3 testing. Karin

________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Freeman, Alexandra (NIH/NIAID) [E] [freemaal at mail.nih.gov]
Sent: Wednesday, September 05, 2012 11:37 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] 3 month old Pneumocystis pneumonia, normal labs

Although you described only rash to one foot, STAT3 mutated HIES can present with PCP in this age range and could be a consideration, and rash may be minimal.  I didn't see an IgE listed, but may not be that elevated at this age.
Alexandra

--
Alexandra Freeman MD
Laboratory of Clinical Infectious Diseases NIAID, NIH NIH, Bldg 10,Room 12C103 9000 Rockville Pike Bethesda, MD  20892 Phone 301-594-9045 Fax 301-496-0773 freemaal at mail.nih.gov

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On 9/5/12 1:21 PM, "Karin Chen" <karin.chen at hsc.utah.edu> wrote:



Dear Thomas,

Thank you for your suggestion. I should have highlighted that HIV 1/2 antibodies were negative, HIV1 RNA was negative.  Mother had negative HIV prenatal testing. Both mother and father got retested for HIV after the child's diagnosis, both were negative by mother's report.

Karin
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Boyce, Thomas G., M.D. [Boyce.Thomas at mayo.edu]
Sent: Wednesday, September 05, 2012 11:13 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] 3 month old Pneumocystis pneumonia, normal labs

Karin,

It is not "normal" for 3 month olds to develop PCP, but it can happen.
It is the peak age of incidence in children with perinatal HIV infection, perhaps because of initial acquisition of the organism at that time.  However, I would keep looking.  We just saw a very similar pt, 4 month old boy with confirmed PCP, and normal apparent T cell numbers and function.  However, in a new polyfunctional T cell assay in which our lab looks at mitogen stimulated T cell production of IFN, IL2 and TNF, all were markedly reduced compared with controls.

Thomas G. Boyce, MD, MPH
Pediatric Infectious Diseases and Immunology Mayo Clinic Rochester, MN 55905
phone: 507-255-8464
fax: 507-255-7767


-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Karin Chen
Sent: Wednesday, September 05, 2012 12:07 PM
To: pagid at list.clinimmsoc.org
Subject: [CIS PIDD] 3 month old Pneumocystis pneumonia, normal labs


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