[CIS PIDD] [cis-pidd] IgG Levels dropping in XLA patient

[CIS-PIDD]

We have seen mycoplasmas other than M pneumoniae causing invasive disease including M salivarium and animal species (M arginini).  Severe lung infection with ureaplasma in a STAT3 deficient HIES pt was just reported by a French group.  There is a relatively new species called M amphoriforme that has been reported in Europe but not yet in the U.S., mainly from PID patients. IVIG may offer little protection from exotic species of bacteria including veterinary species.  What I'm getting at is you cannot exclude atypicals by anything but unbiased NGS.  Since that isn't available yet for general clinical use, I'd suggest starting with the 16S PCR/NGS assay offered by the Univ of Washington.

Prescott

Sent from my iPhone

On Jun 19, 2017, at 12:06 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

Dear all
I am managing a 4 years old with XLA. Who has started with chronic cough .Seen by pulmonary who did bronchoscopy which came out normal except some minor growth of H influenza( Fungi, Atypical and Typical TB negative)
He has been treated with several antibiotics including Augmentin, clinda+ cefopodoxime  without any significant response . Finally started on Bactrim with some improvement however was stopped due to urticarial reaction and angioedema  . Pulmonologist does not feel that a CT scan is required at this time
CT scan done prior to diagnosing XLA ( In 2014 ) showed  multiple  opacities( as per history  I don’t have access to that CT
Another significant trend  noted is that he is dropping his pre IVIG  IgG levels . He is on currently on about 700 mg/kg of IVIG however his last 3 IgG levels have been dropped from 832 to 784 and now 634( lowest he ever had)
Stool for  alpha one antitrypsin is negative
Albumin level is 3.9
Stool for Giardia is negative
Gaining weight and height
No fevers except recently after bronch

Any suggestions why is he dropping his IgG level  and any other suggestions for managing his chronic cough

Thanks

Ejaz Yousef MD

Nemours Children Specialty Care

Jacksonville, FL




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