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

[Mahboobeh Mahdavinia]

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

>

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