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

[Sullivan, Kathleen]

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


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