[PAGID] Unequal Lynonization in a CGD daughter

[Richard Wasserman]

Nine year old daughter of a patient with x-linked CGD.

At age three had post-streptococcal glomerulonephritis. At age five
she presented with a new diagnosis of membranoproliferative
glomerulonephritis and was treated with prednisone. She had no
infection problems until several months after the diagnosis of MPGN
but was then referred for evaluation because of three episodes of
otitis, conjunctivitis and strep throat and one pneumonia in a four
month period. At that time IgM: 147 mg/dL (49-196) IgA: 236 mg/dL
(34-194) IgG: 431 mg/dL (551-1301). Hypogammaglobulinemia was felt to
be due to proteinuria. Further evaluation was started but she was lost
to follow up.

She was treated with prednisone for 3 years for MPGN. About one year
ago prednisone was stopped and Cellcept was started. Within 24 hours
of starting Cellcept she developed tender cervical, submandibular and
axillary adenopathy and fever. Cellcept was stopped and the nodes
slowly improved taking one month to normalize. This occurred three
times (restarting Cellcept) with the same result. Was seen by her
pediatrician and treated with unknown antibiotics without benefit.
Episodes were associated with worsening of MPGN. One year after the
last dose of Cellcept intermittent, painful adenopathy has persisted.
Not unexpectedly, there is worsening of MPGN with every infection. She
is currently getting prednisone 120mg/day.

Neutrophil oxidative burst assay was 32 and 25 on two separate
occasions, normal >73 (test performed at IBT Laboratories)

Paternal grandmother of this child has diminished oxidative burst and
a history of abscesses and pneumonia.

I would appreciate suggestions on further evaluation and
recommendations for management.

-- 
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