[CIS PIDD] [cis-pidd] Fucose anyone? LAD2 patient with draining wound

[Saul Oswaldo Lugo Reyes]

    Dear Elena,

     Have you tried using intralesional filgastrim?

     I believe Richard Stiehm and Marco Yamazaki have reported good
experiences in patients with defective wound healing by applying GCSF
locally.

      Best wishes,

Saul.



2015-05-27 14:56 GMT-05:00 Perez, Elena Elizabeth <e.perez13 at med.miami.edu>:

>
> Dear immunology colleagues around the world,
>
> I am caring for a 14yo patient with LAD2 (the patient described in
> Hidalgo, et al Blood 2003;101:1705-1712), who was transferred to our
> university hospital for a chronically draining wound from site of former
> G-tube.
>
> Abdominal ultrasound shows, "3.9 x 0.6 x 3.1 cm irregular heterogeneous
> hyperemic collection in the left abdomen wall with apparent track to the
> skin similar to the prior exam. There is no demonstration of track between
> this collection and the peritoneal cavity." Upper GI with small bowel
> follow through shows, "  Abnormally positioned duodenum and small bowel, as
> expected in view of the patient's history of malrotation, 2.  No evidence
> of intestinal obstruction,  3.  A gastrocutaneous fistula was not
> identified on this examination."
>
> This child has been in and out of several area hospitals for at least half
> a year, on a range of antibiotics (including trial of long term antibiotics
> via PICC line which was eventually discontinued), to no avail.
>
> He spiked temps to 40C several days ago, with WBCs in the 50k range. Fever
> resolved with addition of vancomycin to his regimen of tobramycin and
> cefepime. His cultures have been negative at our hospital, but there are
> reports of previous recent cultures with pseudomonas at the outside
> hospital.
>
> Surgeons do not want to operate, stating that it is already draining and
> there is no discreet abscess nor fistula. Mom reports pretty good wound
> healing from surgery in the past, while not on fucose.
>
> He has severe failure to thrive (weighs 14kg). He is otherwise stable on
> room air and NG feeding tube, although mom reports recent weight loss.
>
> Unfortunately, he's been off of fucose since 2007. I have contacted the
> Pfanstiehl company (USA side) who says that their fucose lot is currently
> expired, and not available again until mid-July, but they are looking for
> any left over GMP grade fucose they might have. When it is available, I am
> not sure that I will be able to get it unless the insurance covers it or
> they give it to him compassionately (apparently super expensive).
>
> I have a couple of questions:
>
> 1. any immunologist out there (Dr. Etzioni?) have access to fucose that we
> might be able to somehow arrange compassionately for this patient? (this
> might be complicated)...
>
> 2. the possibility of granulocyte infusion +/- plasmapheresis +/- ritux
> has been considered, but blood bank is hesitant, (and this is also probably
> even more complicated). Anyone that can share their experience with
> granulocyte infusion in Bombay blood group patient?
>
> I'm "between a rock and a hard place" as the saying goes...
>
> Thanks for your thoughts, ideas, suggestions, and fucose if you have any...
> Elena
>
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-- 
Saúl Oswaldo Lugo Reyes

Investigador en Ciencias Médicas
Unidad de Investigacion en Inmunodeficiencias
9° piso, Torre de Investigacion,
Instituto Nacional de Pediatria
Coyoacan 04530 DF, Mexico
+52(55)10840900 ext. 1866
--------------------------------------
"La penicilline guérit les humains; le vin les rend heureux"
"If penicillin cures sicknesses, Jerez resuscitates dead people". -Sir
Alexander Fleming

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