[PAGID] CGD and bladder wall thickening

[Jack Bleesing]

Morning Folks (on the western hemisphere at least).

Unfortunately, long-term steroids often cannot be avoided in CGD even if you wanted to and should likely not be feared so much (at the low doses that seem to work in many patients). In addition to chest disease, all CGD patients seem to have IBD if you look hard enough. As we do extensive GI evaluations in the CGD patients that are evaluated prior to BMT, all biopsies are essentially abnormal even if the clinial picture is "quiet".

Now...........BMT will fix this horrible disease.

Many regards,

JB

---------------------------------------------------------------------------
Jack J.H. Bleesing, M.D., Ph.D.
Cincinnati Children's Hospital Medical Center
Division of Hematology/Oncology
3333 Burnet Avenue, MLC 7015
Cincinnati, OH 45229
513-636-4266 (phone)
513-636-3549 (fax)
Jack.Bleesing at CCHMC.org
http://www.cincinnatichildrens.org/immunodeficiencies/



>>> "John B. Ziegler" <j.ziegler at unsw.edu.au> 3/6/2008 7:26 AM >>>

I would not agree that long term steroids should be avoided in CGD. 
We have had to do this in CGD paitnets with inflammatory bowel or 
chest disease.

John Ziegler

At 02:38 AM 6/03/2008, you wrote:

>Our experience has been different.  We have sought the source of the 

>infection (urine culture).  This is not uncommon in adult urology 

>with individuals with cystostomy tubes.  Bladder wall infections 

>occur.  The granulomatous piece is the child's particular responses 

>to the infection.  We have tried various antibiotics to gain control 

>of the infections (first a trial of vanco, then 

>cephalosporins).  We've even tried macrolides with success when 

>these more routine antibiotics have failed (targetting 

>what?  ureaplasma? nontuberculous mycobacterium?  Admittedly we're 

>not sure).  We used ultrasound evaluations of bladder wall thickness 

>to determine length of therapy.  Symptoms (dysuria, hematuria, 

>frequency) resolved quickly with antibiotics.

>

>We struggle to avoid long term steroids.

>

>Additionally, we have found that with that one patient (at this 

>particular age) was a "retainer" in the words of our urologist at 

>the same age.  He urinated infrequently, all of a sudden with 

>extreme urgency (as is not uncommon for boys of age 4).  He was 

>constipated also.  We worked on increasing frequency of attending to 

>urination, and used antibiotics.  He improved.  It has recurred, 

>usually by November because elementary school results in less 

>frequent ability to attend to toiletting, but he still responds to 

>antibiotics.

>

>As far as the risk of biopsy, the lesions we've seen are very 

>vascular (or neovascular) and the one biopsy we did not only failed 

>to yield an organism but resulted in a lot of bleeding.

>

>Best of luck.

>

>Sincerely,

>Daniel H. Conway, MD

>St. Christopher's Hospital for Children

>Drexel University College of Medicine

>215-427-5284

>

>

> > Date: Mon, 3 Mar 2008 16:41:43 -0600

> > From: cmseroogy at pediatrics.wisc.edu 

> > To: pagid at list.clinimmsoc.org 

> > Subject: [PAGID] CGD and bladder wall thickening

> >

> >

> > We have a 4y/o boy with X-linked CGD and a right wall bladder thickening,

> > dysuria and hematuria. Culture negative, ESR normal and afebrile.

> >

> > The imaging consisted of U/S and CT with contrast. It does not have the

> > characteristics of a rhabdo and given his age in situ carcinoma is rare. I

> > am inclined to assume this is non-infectious granulomatous 

> disease and treat

> > with oral steroids. His history is notable for gastric antrum granulomas

> > this past Fall responsive to oral steroids and aspergillus pneumonia at

> > presentation 3 years ago.

> >

> > My questions:

> >

> >

> > 1. Would people be comfortable with empiric steroid treatment or is the

> > risk/invasive nature of cytoscopy with bx/culture warranted?

> >

> > 2. Should empiric antibiotics be started? If so, what regimen?

> >

> > Thank you for your thoughts! Chris

> >

> >

> >

> > Chris Seroogy, M.D.

> >

> > Assistant Professor

> >

> > Dept. of Pediatrics

> >

> > Mail:  H4/474 CSC, Mailstop 4108

> >

> > Shipping:  H4/431 CSC, Mailstop 4108

> >

> > 600 Highland Ave.

> >

> > Madison, WI  53792

> >

> > phone: 608- 263-2652

> >

> > fax: 608-265-0164

> >

> >

>

>

>

>----------

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A/Prof John B. Ziegler
Department of Immunology and Infectious Diseases
Sydney Children's Hospital
High Street, RANDWICK NSW 2031
Australia
Tel: (02) 93821515
Fax: + 61 + 2 + 93821580
Email: j.ziegler at unsw.edu.au