[CIS PIDD] MBL deficiency

[Anete S Grumach]

For additional diagnosis on lectin pathway and therapy, you may contact
"jens jensenius" <j.c.jensenius at immunology.au.dk>,

Anete

2012/6/21 Berger, Melvin <Melvin.Berger at uhhospitals.org>


> There are lots of reports of MBL deficiency as a modifier gene which has

> negative impact on other diseases (CF for sure, and I think XLA as well),

> also that MBL deficiency other things like chemo or sickle cellhave more

> infections. The Danish StaensSerum Instituet has preparations of MBL they

> have tested in people. I don't know the availability of that at present. I

> don't think i would use FFP for prophylaxis, but might try it for a deep

> infection responding poorly. I have seen patients with MBL deficiency with

> very slowly resolving abscesses.

>

> Melvin Berger, M.D., Ph.D.

> Adjunct Professor of Pediatrics and Pathology

> Case Western Reserve University

> Cleveland, OH 44106

>

> ________________________________

>

> From: pagid-bounces at list.clinimmsoc.org on behalf of Richard Wasserman

> Sent: Thu 6/21/2012 3:40 PM

> To: PAGID

> Subject: [CIS PIDD] MBL deficiency

>

>

>  I am seeing a 43 year old female physician with a lifelong history of

> recurrent sinusitis, two episodes of pneumonia in the past 10 years that

> required ICU care (one of which sounds like septic shock), significant

> post-op MRSA infection after sinus surgery and a large cellulitis after

> stingray envenomation. Immunoglobulins are normal (IgG >1000), pneumococcal

> antibody titers (14 serotypes) are strongly positive (12/14 >1ug/ml, 8/14

> >5ug/ml). Mannose binding lectin was not detectable.

>

> I know that Dr. Kirkpatrick reported a family treated successfully with

> IGIV. Are there other suggestions for treatment? Antibiotic prophylaxis?

> Richard Wasserman

> Dallas

>

> --

> Richard L. Wasserman, MD, PhD

> DallasAllergyImmunology

> 7777 Forest Lane, Suite B-332

> Dallas, Texas 75230

> Office (972) 566-7788

> Fax (972) 566-8837

> Cell (214) 697-7211

>

>

>

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