[CIS PIDD] [cis-pidd] Hypogammaglobulinemia

Tue Sep 26 19:25:16 EDT 2017

Yes, stool alpha-1 anti-trypsin was normal too.

On Tue, Sep 26, 2017 at 12:05 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Hi Evan:  This still sounds like a protein-losing enteropathy even though
> endoscopy was normal.  Did you get a stool alpha-1-antitrypsin?
>
>
>
> Prescott
>
>
>
> T. Prescott Atkinson, MD PhD, Professor and Director
>
> Division of Pediatric Allergy, Asthma & Immunology
>
> University of Alabama at Birmingham
>
> Tel: 205-638-6457 <(205)%20638-6457>
>
> Fax: 205-975-7080 <(205)%20975-7080>
>
>
>
>
>
>
>
> *From:* cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] *On
> Behalf Of *CIS-PIDD
> *Sent:* Tuesday, September 26, 2017 1:20 PM
> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Subject:* RE: [cis-pidd] Hypogammaglobulinemia
>
>
>
>
>
> Dr. Shereck:
>
>
>
> A few questions/suggestions –
>
>
>
> (1) has the child been evaluated for chronic infections?  EBV, CMV, HTLV,
> HIV, and mycobacteria (both TB and non) would be on my D/Dx … and if she
> had any exotic exposures (animals, forest/farm, recreational water), the
> list would get longer.  Specific IgG could certainly have a transient
> activity against some occult viral infection … and even some bacterial
> infections.
>
>
>
> (2) has she had an evaluation of antiviral titers pre-IVIG initiation?
>
>
>
> (3) has the abdominal pain been evaluated?  If she’s thin enough, superior
> mesenteric artery (Wilkie’s) syndrome would be part of my D/Dx.
>
>
>
> I think most people would agree that re-starting IVIG would be a prudent
> route.  The question is what are we treating / missing.
>
>
>
> Good luck with the case.
>
>
>
> Karl
>
>
>
> Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
>
> Scientist II and Assistant Director, Center for Infectious Diseases and
> Immunology
>
> RGH Research Institute | Rochester General Hospital | Rochester Regional
> Health
>
> 1425 Portland Ave., Room R
> <https://maps.google.com/?q=1425+Portland+Ave.,+Room+R&entry=gmail&source=g>-403,
> Rochester, NY   14621
>
> Tel  585-922-3709 <(585)%20922-3709>  |  Fax  585-922-2415
> <(585)%20922-2415>
>
>
>
>
>
> *From:* cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net
> <cis-pidd at lyris.dundee.net>] *On Behalf Of *CIS-PIDD
> *Sent:* Tuesday, September 26, 2017 2:04 PM
> *To:* CIS-PIDD
> *Subject:* Re: [cis-pidd] Hypogammaglobulinemia
>
>
>
> Yes, both were done and were normal.
>
>
>
> -Evan
>
> _____________________
>
> On Tue, Sep 26, 2017 at 10:54 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> If not done, fecal alpha-1-antitrypsin and calprotectin may be helpful.
>
>
>
> Joe Church
>
> Children’s Hospital Los Angeles
>
> _____________________
>
> *From:* cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] *On
> Behalf Of *CIS-PIDD
> *Sent:* Tuesday, September 26, 2017 10:34 AM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] Hypogammaglobulinemia (EXTERNAL EMAIL)
>
>
>
> All,
>
>
>
> I am seeking advice about a 10 year old female with low IgG. who
> first came to my attention 3 years ago. At that time, she had low energy,
> abdominal pain and low-grade fevers (99.9-100.4). She also has
> asthma. There were no obvious findings on physical exam other than a
> skinny, sad, child.
>
>
>
> Her immune work-up revealed an IgG level of 260 with normal IgM and IgA
> levels. She has vaccine responses to strep pneumo, tetanus, and diphtheria.
> She had a normal total B cell count with switch to memory cells.
>
>
>
> She was started on IVIG and her symptoms disappeared right away. She had
> to be maintained on IVIG Q2 weeks to keep her level > 600 to keep her
> asymptomatic. Her teachers even commented on how much better she was at
> school after starting the IVIG.
>
>
>
> Last year, the family decided to come off the IVIG. Again her symptoms
> have returned. She is missing 2-3 days of school/week. She is having
> abdominal pain daily. She has no energy and feels the need to nap all the
> time (even at school).
>
>
>
> Repeat immune testing is consistent with her previous testing with the low
> IgG being the only abnormality. In addition, she had B-cell
> immunophenotyping which was normal. Inflammatory markers (ESR and CRP were
> normal). CBC is normal. She does have a mildly low albumin that fluctuates
> from 2.9-3.
>
>
>
> She has had an endoscopy, colonoscopy which were normal. Stool studies are
> normal. Urine micro and  urinalysis were normal.
>
>
>
> It does not seem like a primary production issue. So, is she losing her
> IgG somewhere and if so, where? Where else should I be looking?
>
>
>
> In the meantime, she is going to re-start her IVIG because it does seem to
> make a big difference in her overall health and quality of life.
>
>
>
> Thank you for your thoughts.
>
>
>
> -Evan
>
>
>
> *Evan Shereck, MD, MEd*
>
> Associate Professor of Pediatrics
>
> Director, Pediatric Hematology/Oncology Fellowship
>
> Block Co-Director, Blood and Host Defense
>
> Oregon Health & Science University I  3181 SW Sam Jackson Park Rd
> <https://urldefense.proofpoint.com/v2/url?u=https-3A__maps.google.com_-3Fq-3D3181-2BSW-2BSam-2BJackson-2BPark-2BRd-26entry-3Dgmail-26source-3Dg&d=DwMFaQ&c=ZcS_IThVDLRgSnibLQVJ9vwqRPpc3RkFqvJL1VfvJu0&r=SS5WX_zJKFGQt9gnWxXM2zj3mGuaCrOGfogIF4rsPm06T2ggGCIyqTpu8I8GvD7y&m=D5mKjcT6ogU16E86TBOSrZTFoOkhxW40hetNIWH3k4E&s=vDNCWJ9Sm287m9YknFjmsT5ngdUTMgkLU-AXPGSgkwM&e=>,
> Mail Code: CDRCP  I  Portland, OR 97239
>
> (Office): 503-494-0829 <(503)%20494-0829>  I  (Fax): 503-494-0714
> <(503)%20494-0714>
>
>  (E-mail): shereck at OHSU.edu
> <https://urldefense.proofpoint.com/v2/url?u=https-3A__mail.ohsu.edu_owa_14.3.266.1_scripts_premium_redir.aspx-3FSURL-3DLo7EaJgNr7dm-2D91Vzq29a6AsFozcU6HcZxr1gNGTZFW2xPIy05TTCG0AYQBpAGwAdABvADoAcwBoAGUAcgBlAGMAawBAAE8ASABTAFUALgBlAGQAdQA.-26URL-3Dmailto-253ashereck-2540OHSU.edu&d=DwMFaQ&c=ZcS_IThVDLRgSnibLQVJ9vwqRPpc3RkFqvJL1VfvJu0&r=SS5WX_zJKFGQt9gnWxXM2zj3mGuaCrOGfogIF4rsPm06T2ggGCIyqTpu8I8GvD7y&m=D5mKjcT6ogU16E86TBOSrZTFoOkhxW40hetNIWH3k4E&s=zmngEWzRLj6tHZEq_r8lNiuf8oYTxKBCniz8-BTPVTE&e=>
>
> Visit us on Facebook: https://www.facebook.com/OHSUPHO
> <https://urldefense.proofpoint.com/v2/url?u=https-3A__mail.ohsu.edu_owa_14.3.266.1_scripts_premium_redir.aspx-3FSURL-3DWyRPz0SA5Ekc-5Frc2B-5FAotiWctzbv9uoiWKt3Q3jWNgm2xPIy05TTCGgAdAB0AHAAcwA6AC8ALwB3AHcAdwAuAGYAYQBjAGUAYgBvAG8AawAuAGMAbwBtAC8ATwBIAFMAVQBQAEgATwA.-26URL-3Dhttps-253a-252f-252fwww.facebook.com-252fOHSUPHO&d=DwMFaQ&c=ZcS_IThVDLRgSnibLQVJ9vwqRPpc3RkFqvJL1VfvJu0&r=SS5WX_zJKFGQt9gnWxXM2zj3mGuaCrOGfogIF4rsPm06T2ggGCIyqTpu8I8GvD7y&m=D5mKjcT6ogU16E86TBOSrZTFoOkhxW40hetNIWH3k4E&s=e7wAF3r_paNJwWCCNnSsCJgAQnhBLmXhj9Ubw37BcC8&e=>
>
>
>
>
>
>
>
>
> *Rochester Regional Health Email Disclaimer*
> This email and any files transmitted with it are confidential and intended
> solely for the use of the individual or entity to whom they are addressed.
> If you have received this email in error please delete it from your system.
> This footnote also confirms that this email message has been swept for the
> presence of computer viruses.
>
> ---
>
> You are currently subscribed to cis-pidd as: patkinson at peds.uab.edu.
>
> To unsubscribe click here: http://cts.dundee.net/u?id=96396838.
> 952f64cd9dda04997031bb8889426d86&n=T&l=cis-pidd&o=4591617
>
> (It may be necessary to cut and paste the above URL if the line is broken)
>
> or send a blank email to leave-4591617-96396838.
> 952f64cd9dda04997031bb8889426d86 at lyris.dundee.net
>
> ---
>
> You are currently subscribed to cis-pidd as: evan.shereck at gmail.com.
>
> To unsubscribe click here: http://cts.dundee.net/u?id=103087178.
> 39df88cd7b664007cf977ccbb4f6115b&n=T&l=cis-pidd&o=4591677
>
> (It may be necessary to cut and paste the above URL if the line is broken)
>
> or send a blank email to leave-4591677-103087178.
> 39df88cd7b664007cf977ccbb4f6115b at lyris.dundee.net
>

---
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=4591981
or send a blank email to leave-4591981-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20170926/8b67f8e4/attachment-0001.html>