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

[Nacho Gonzalez]

Dear Jason,

I think this paper may help you decide:

Lung MRI as a possible alternative to CT scan for patients with primary
immune deficiencies and increased radiosensitivity.Serra G, Milito C,
Mitrevski M, Granata G, Martini H, Pesce AM, Sfika I, Bonanni L, Catalano
C, Fraioli F, Quinti I.Chest. 2011 Dec;140(6):1581-9. doi:
10.1378/chest.10-3147. Epub 2011 May 26.

Best regards,

Luis Ignacio Gonzalez Granado
Pediatrics, Immunodeficiencies.
Hospital 12 octubre. Madrid. Spain

2013/2/28 Jason Raasch <jraasch at midwestimmunology.com>


> 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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