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

[CIS-PIDD]

Many thanks !

Sent from my iPhone

On Jun 19, 2017, at 1:25 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> wrote:

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This young fellow needs at least a chest x-ray and likely a CT.



Regarding dropping IgG trough levels:

  *   Look for protein-losing enteropathy: Fecal alpha-1-antitrypsin and calprotectin (for inflammation), and serum albumin
  *   Proteinuria severe enough to cause loss of IgG would likely be causing nephrotic syndrome.
  *   Rule-out gut infections  including H. pylori (fecal H. pylori antigen) that has been reported to cause PLE.  We had a patient with AR-agammaglobulinemia whose IgG trough dropped steadily.  We identified H. pylori antigen in the stool; treated him; and his IgGs returned to pre-H. pylori levels.

Joe Church

Children's Hospital Los Angeles

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From: cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net> [cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>] on behalf of CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Monday, June 19, 2017 10:05 AM
To: CIS-PIDD
Subject: [cis-pidd] IgG Levels dropping in XLA patient (EXTERNAL EMAIL)

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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