[CIS-PAGID] Glycogen Storage Disease Type 1-b

[M CLAYTON]

There was a period of time several years ago when they missed appointments and struggled with supervising his medication, but recently they have been doing pretty well.  
Michael Clayton, MD, MPH
 



From: sullivak at mail.med.upenn.edu
Date: Tue, 14 Feb 2012 15:42:14 -0500
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Glycogen Storage Disease Type 1-b


Wow!  We used to see a bunch of these kids and generally they did pretty well on GCSF.  They do still have some dysfunction even with normal counts but this is definitely sicker than I have seen.  Are you sure of his compliance?


On Feb 14, 2012, at 3:33 PM, M CLAYTON wrote:


Dear Colleagues:
Does anyone have experience managing the neutropenia/impaired neutrophil function in Glycogen Storage Disease type 1-b?  I have a 16 year old boy with this disorder who seems to be having more frequent infections as he gets older despite maintaining normal ANC's with GCSF and/or Neulasta.  Metabolically, he has done OK with no major hypoglycemic episodes, and no discussion of liver transplant at this time.  He has had osteomyelitis and a cerbral abscess within the past 18 months, but has recovered from both.  Is there anything else that I should be doing for him?  Thanks in advance for your help.
 
Michael H. Clayton, MD, MPH
Assistant Clinical Professor of Pediatrics
University of New Mexico



Kate Sullivan, MD PhD
Professor of Pediatrics
ARC 1216 Immunology CHOP
3615 Civic Center Blvd.
Philadelphia, PA 19104
(p) 215-590-1697
(f) 267-426-0363


 		 	   		  
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20120215/4b554a31/attachment.htm>