[CIS PIDD] [cis-pidd] Chest imaging in Bloom syndrome

[Sullivan, Kathleen]

I like MRI in my AT patients.  It is actually quite nice.

On Feb 28, 2013, at 11:36 AM, Jason Raasch wrote:


> Good Morning,

> 

> I am looking for advice from those of you who have experience with Bloom syndrome.

> 

> The question is what chest imaging modality is reasonable in the following patient:

> 

> 18 year old female who was seen last week to establish immunology care as it relates to Bloom syndrome.

> 

> Her diagnosis was made at 3 years of age; I have yet to confirm how this by what criteria.  Previous immunologic evlatuion notable only for modestly decreased IgM.  She was on IVIG for six months for recurrent respiratory disease and according to her mother this therapy helped a great deal.  It is unclear to me why it was discontinued.

> 

> She has not had immunology follow-up since age 10.

> 

> PFTs (spirometry only) one year ago revealed FEV1 64% (with 12% improvement after bronchodilator); FVC 61% and [pre-bronchodilator] FEV1/FVC 105%.

> 

> She has a history of recurrent upper and lower respiratory illness.

> 

> I will obtain PFTs to include lung volume and diffusion.

> 

> However, assessement for bronchiectasis, pulmonary nodules, interstial disease, etc. will be important.

> 

> ** Does the clinical picture justify chest CT (with attempts to minimize radiation) or are there MRI protocols that might provide just as useful information? **

> 

> Of note, preliminary immune assessment is fairly unremarkable.  Notable for normal quantitative immunoglobuilins, etc.  Pneumococcal titers arguably 'low' (baseline) but has not had pneumococcal vaccination in at least 10 years.  Evaluation ongoing.

> 

> Thank you for your input.

> 

> Regards,

> 

> Jason

> 

> Jason Raasch, MD

> 

> Midwest Immunology Clinic

> 15700 37th Ave N, Ste 110

> Plymouth, MN 55446

> 

> TEL: (763) 577-0008

> FAX: (763) 577-0192

> 

> 

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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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