[CIS PIDD] [cis-pidd] Hypogammaglobulinemia

[CIS-PIDD]

Yes, she had a video capsule too that was normal.

On Tue, Sep 26, 2017 at 12:14 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Hi,
>
> Does normal endoscopy mean normal biopsies?
>
> I would also consider video capsule to rule out intestinal
> lymphangiectasia.
>
> David Hagin
>
>
> On Tue, Sep 26, 2017 at 8:34 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
>> 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>,
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>>
>> (Office): 503-494-0829 <(503)%20494-0829>  I  (Fax): 503-494-0714
>> <(503)%20494-0714>
>>
>>  (E-mail): shereck at OHSU.edu
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>>
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>>
>>
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