[CIS PIDD] [cis-pidd] Hypogammaglobulinemia

[CIS-PIDD]

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://maps.google.com/?q=3181+SW+Sam+Jackson+Park+Rd&entry=gmail&source=g>,
> 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://mail.ohsu.edu/owa/14.3.266.1/scripts/premium/redir.aspx?SURL=Lo7EaJgNr7dm-91Vzq29a6AsFozcU6HcZxr1gNGTZFW2xPIy05TTCG0AYQBpAGwAdABvADoAcwBoAGUAcgBlAGMAawBAAE8ASABTAFUALgBlAGQAdQA.&URL=mailto%3ashereck%40OHSU.edu>
>
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