[PAGID] Unequal Lynonization in a CGD daughter

[Prof. Dr. Antonio Condino Neto]

Prednisone interferes with oxidative burst and we have to be careful  
about interpretation of these data.

Is that a DHR test? Only neutrophils? What about the monocytes?

There are specific tests to assess lynonization and if this is the  
case, the patient will bahave as a CGD like and should be treated as  
CGD.

-- 
Antonio Condino-Neto
Professor, Department of Immunology
Institute of Biomedical Sciences, University of São Paulo
1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil
Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando Richard Wasserman <drrichwasserman at gmail.com>:


> 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

>




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