[CIS PIDD] [cis-pidd] Baby with intestingal lymphangiectasia

[Richard Wasserman]

Joe,
Why is there no IgA? That concerns me a little. Most of the PLE's do pretty
well without supplementation. Does your lab normalize mitogen result for
lymphocyte count? I'd hate to give her two problems but while most dogs
have ticks OR fleas some have ticks AND fleas.
Richard Wasserman
Dallas

On Wed, Jul 22, 2015 at 1:28 PM, Nacho Gonzalez <nachgonzalez at gmail.com>
wrote:

> Dear Dr. Church,
>
> the last 2 XLA patients I have seen recently were under the first year of
> life. They had poor vascular access... every IVIG infusion was a pain. Now
> they are under SCIG and doing fine. But going back to your patient, I do
> not think she needs IgG replacement therapy if she is free of infections.
>
> Best regards,
>
> Luis Ignacio Gonzalez-Granado
> Immunodeficiencies Unit
> Pediatric Hematology & Oncology Unit
> Hospital 12 octubre
> Madrid. Spain
>
> 2015-07-22 20:19 GMT+02:00 Verbsky, James <jverbsky at mcw.edu>:
>
>>  Not sure if this will work as well in teliangiectasia but in other PLE
>> states subQ has been beneficial…might be tough in an 18mo..but it’s a
>> thought
>>
>>
>>
>> james
>>
>>
>>
>> *From:* Church, Joseph [mailto:JChurch at chla.usc.edu]
>> *Sent:* Wednesday, July 22, 2015 1:08 PM
>> *To:* CIS-PIDD
>> *Subject:* [cis-pidd] Baby with intestingal lymphangiectasia
>>
>>
>>
>> Colleagues:
>>
>>
>>
>> I have consulted on an 18 month-old girl with intestinal lymphangiectasia
>> and unilateral lymphedema.  She has experienced *no infectious
>> complications*.
>>
>>
>>
>> However, labs demonstrate
>>
>>             Serum albumin 2.3 g/dL
>>
>>             IgG      83 mg/dL
>>
>>             IgA      <7 mg/dL
>>
>>             IgM      27 mg/dL
>>
>>             Tetanus antibody 0.49 IU/mL (considered protective)
>>
>>             Haemophilus influenzae antibody 0.48 mcg/mL (considered
>> unprotective)
>>
>>             Pneumococcal antibody response to Prevnar 13 positive (>1.3
>> mcg/ml) for 10 of 12 serotypes tested.
>>
>>             CD3+ 46% (1399/mcL)
>>
>>             CD4+ 14% (328/mcL)
>>
>>             CD8+ 46% (1113/mcL)
>>
>>             CD19+ 24% (590/mcL)
>>
>>             NK 16% (391/mcL)
>>
>>             PHA and PWM responses decreased (~ 25% of concurrent control)
>>
>>
>>
>> The child is clearly losing protein, immunoglobulins and T-cells (CD4+ >
>> CD8+), but has fair circulating antibody responses to tetanus toxoid and
>> conjugated pneumococcal vaccine.
>>
>>
>>
>> I would appreciate your opinions regarding immunoglobulin replacement
>> therapy.
>>
>>
>>
>> Joe Church, MD
>>
>> Children’s Hospital Los Angeles
>>
>>
>>
>>
>>
>>
>> ---------------------------------------------------------------------
>> CONFIDENTIALITY NOTICE: This e-mail message, including any attachments,
>> is for the sole use of the intended recipient(s) and may contain
>> confidential
>> or legally privileged information. Any unauthorized review, use,
>> disclosure
>> or distribution is prohibited. If you are not the intended recipient,
>> please
>> contact the sender by reply e-mail and destroy all copies of this
>> original message.
>>
>> ---------------------------------------------------------------------
>>
>> ---
>>
>> You are currently subscribed to cis-pidd as: jverbsky at mcw.edu.
>>
>> To unsubscribe click here:
>> http://cts.dundee.net/u?id=96396710.777d26b6da96d84a8b0d5e2d208337f2&n=T&l=cis-pidd&o=3000839
>>
>> (It may be necessary to cut and paste the above URL if the line is broken)
>>
>> or send a blank email to
>> leave-3000839-96396710.777d26b6da96d84a8b0d5e2d208337f2 at lyris.dundee.net
>>
>> ---
>>
>> You are currently subscribed to cis-pidd as: nachgonzalez at gmail.com.
>>
>> To unsubscribe click here:
>> http://cts.dundee.net/u?id=96396812.66aedac07f1044b5f6e7c94e3e51e2ac&n=T&l=cis-pidd&o=3000852
>>
>> (It may be necessary to cut and paste the above URL if the line is broken)
>>
>> or send a blank email to
>> leave-3000852-96396812.66aedac07f1044b5f6e7c94e3e51e2ac at lyris.dundee.net
>>
>>
> ---
>
> You are currently subscribed to cis-pidd as: drrichwasserman at gmail.com.
>
> To unsubscribe click here:
> http://cts.dundee.net/u?id=96396499.3449c9fd54f25f9fa0e022cb002b8c1e&n=T&l=cis-pidd&o=3000873
>
> (It may be necessary to cut and paste the above URL if the line is broken)
>
> or send a blank email to
> leave-3000873-96396499.3449c9fd54f25f9fa0e022cb002b8c1e at lyris.dundee.net
>
>


-- 
Richard L. Wasserman, MD, PhD
Allergy Partners of North Texas
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

---
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=3000981
or send a blank email to leave-3000981-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20150722/053ed968/attachment-0001.html>