[CIS PIDD] a case of recurrent pneumonias and culture negtive endocarditis

[Richard Wasserman]

Keeping in mind that IgG production is in reaction to infection, the
marginal IgG concentrations in the context of a lifetime of infections is
quite striking. The pneumococcal antibody titers are virtually nil. At this
time, the nature of the immunodeficiency is unclear but an antibody
production defect is certainly a major component. These data, in my view,
support my previous recommendation.
Richard Wasserman

On Wed, Jun 20, 2012 at 5:33 PM, Mahboobeh Mahdavinia <
mahboobeh-mahdavinia at fsm.northwestern.edu> wrote:


> Thank you Dr Wasserman;

> The IgG levels were 782 and 685 , IgA was 113 and IgM 60  .

>

> Strep Pneumo titers:

>

>  0.8 ug/mL Type 1 Abs

>  0.2 ug/mLType 3 Abs

> < 0.2 ug/mLType 4 Abs

>  0.8 ug/mL Type 5 Abs

>  0.5 ug/mL Type 6B

>

> 0.5 ug/mL Type 7F Abs

>

>  0.9 ug/mL Type 8 Abs

>  0.5 ug/mLType 9N Abs

>

> Type 9V Abs 0.4 ug/mL L

> Type 12F < 0.2 ug/mL Abs

> Type 14 Abs 0.8 ug/mL

>

> Type 18C 0.6 ug/mL Abs

> Type 19F 0.2 ug/mL Abs

> Type 23F 0.2 ug/mL

>

>

>

> On Wed, Jun 20, 2012 at 4:53 PM, Richard Wasserman <

> drrichwasserman at gmail.com> wrote:

>

>> You didn't provide the actual Ig values nor the pneumococcal antibody

>> values; they would be interesting. The complement abnormalities are a

>> reflection of active infection and consumption. This lady sounds quite ill.

>> I don't think 500mg/kg/mo is adequate in this setting. I would have given

>> her 500mg/kg/dose on two consecutive days, repeat an IgG at 48-72 hours and

>> follow her levels and clinical response. Good luck.

>> Richard Wasserman

>> Dallas

>>

>>

>> On Wed, Jun 20, 2012 at 4:46 PM, Mahboobeh Mahdavinia <

>> mahboobeh-mahdavinia at fsm.northwestern.edu> wrote:

>>

>>> Dear Colleagues,

>>> We appreciate your input with suggestions for possible diagnosis, work

>>> up and treatment for the following case.

>>>

>>> Regards,

>>>

>>> Maha Mahdavinia,MD

>>> Allergy/Imuunolog fellow

>>> Northwestern University

>>>

>>> Pt is a 37 y/o Female with Hx of multiple pneumonias and otitis since

>>> age 2 months old. By age 18 years she had documented bronchiectasis and

>>> continues to have multiple hard to treat otitis, frequent pneumonias

>>> documented by

>>> CXR despite prophylactic clarithromycin.

>>>

>>>

>>>

>>> Her w/u so far showed normal immunoglobulin levels, normal lymphocyte

>>> numbers and function, negative workup for cystic fibrosis (both sweat test

>>> and mutation analysis), negative ANCA, ANA of 1:80  (speckled) and

>>> negative nRNP, SS-A, SS-B, Sm and Scl-70 Autoantibodies.

>>>

>>>

>>>

>>> She has been in hospitals for the past 4 months with recurrent and

>>> refractory culture negative endocarditis .She has no history of valve

>>> disease in past. During this period, she has already undergone 3 cardiac

>>> surgeries for MVR twice, AVR, and repairs of aortic-atrial fistula. She

>>> is currently intubated due to respiratory failure, is in renal failure and

>>> has had prolonged thrombocytopenia (improved after IVIG in last 3 days).

>>>

>>>  All cultures during her hospitalization and throughout her life,

>>> including bronchoalveolar  lavages , blood cultures, and biopsy

>>> cultures have been negative (fungal, TB, special cultures and viral PCRs).

>>>

>>>

>>>

>>> During this endocarditis admission, her C3 was 38 (NL:90-180), C4 was 17

>>> (NL:10-40), and CH50 was 54 and 48  (NL:63-145).

>>>

>>> Pneumococcus Ab titers showed minimal levels to all 14 serotypes despite

>>> Pneumovax  in 2008. We started IVIG 500mg/kg IV last Friday 6/15.

>>>

>>> * *

>>>

>>> Pending labs include: AH50 level ,Dihydrorhodamine (DHR) , NK function,

>>> C1 and C2.

>>>

>>>

>>>

>>>

>>>

>>

>>

>>

>> --

>> Richard L. Wasserman, MD, PhD

>> DallasAllergyImmunology

>> 7777 Forest Lane, Suite B-332

>> Dallas, Texas 75230

>> Office (972) 566-7788

>> Fax (972) 566-8837

>> Cell (214) 697-7211

>>

>

>



-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20120620/208e416b/attachment.htm>