[CIS PIDD] [cis-pidd] Agammaglobulinemia and suspected Helicobacter canadensis like induced infection of the calf

Edgar, David david.edgar at belfasttrust.hscni.net
Wed May 6 08:05:53 EDT 2015


Dear Jasmeen
Our patient had had a very prolonged course over several years, with repeated admissions, multiple courses of antibiotics and surgical debridement. The patient was at the point of requesting lower limb amputation because of persisting pain and debility. Commencement of hyperbaric oxygen had  a dramatic effect within days and led to complete healing of what had been a very large necrotic sloughing wound. Healing was complete within a period of weeks and there has been no further breakdown over 5 years now. The case seems to confirm that wound healing is dependent on multiple oxygen dependent processes in the microenvironment, and that perhaps active infection is not always the sole/dominant factor preventing healing. It would be great to know if this is reproducible in other cases.
Thanks
David


From: Jasmeen Dara [mailto:jasmeen.dara at gmail.com]
Sent: 05 May 2015 23:05
To: CIS-PIDD
Subject: Re: [cis-pidd] Agammaglobulinemia and suspected Helicobacter canadensis like induced infection of the calf

We are also treating a Helicobacter (H. cinaedi) lower extremity soft tissue infection in a patient with Bruton's.

He had 2 recurrences after being treated with meropenem and levofloxacin for 6 weeks. He is currently improving on doxycycline, rifampin, and amoxicillin. I think he likely needs a very prolonged course (6 months to a year).

What duration have others had success with? Alternatively, what duration have others had treatment failures with?

I like the idea of hyperbarics, especially for our patient since he has a left lower extremity neuromyelitis, muscle atrophy and also had 2 debridements and a skin graft over the last 2 years. (likely poor perfusion to that area)

Thanks!

On Sat, May 2, 2015 at 5:21 AM, Edgar, David <david.edgar at belfasttrust.hscni.net<mailto:david.edgar at belfasttrust.hscni.net>> wrote:
Hi Klaus
Is this now a chronic wound?
We have had very dramatic success with hyperbaric oxygen in similar aged XLA patient with a chronic lower leg wound. This may be worth considering?
Best Regards
David
Steele CL, Cridge C, Edgar JDM. A novel treatment in X Linked agammaglobulinaemia - Hyperbaric Oxygen Therapy in refractory chronic wounds. Journal of Clinical Immunology 2014; doi 10.1007/s10875-014-0078-4
------------------ via Blackberry

From: Prof. Dr. Klaus Warnatz [mailto:klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>]
Sent: Friday, May 01, 2015 09:39 PM
To: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Subject: [cis-pidd] Agammaglobulinemia and suspected Helicobacter canadensis like induced infection of the calf

I would appreciate your input on  a 36 y/o patient with XLA who acquired a soft tissue infection of the right calf in 2004 after a visit to Corfu. The biopsy revealed a lymphocytic panniculitis no signs of T cell lymphoma. An initial therapeutic attempt with steroids was without effect so that Tacrolimus was started. Under this therapy he developed a sepsis and one blood culture revealed a Helicobacter Canadensis like bacterium. After different attempts of antibiotic treatment he clearly improved under imipinem/fosfomycin but several attempts to stop the therapy even after years failed and so again this January. In the MRI of the calf from 12/2014 there were increased signals in the tibia in the T2 weighted images but no contrast medium enhancement, so that the radiologists did not find evidence of an ongoing osteomyelitis, there was no affection of the neighboring muscles. PET scan did not reveal additional sites.
Otherwise infections are well controlled under IGRT with serum trough levels of 12g/l IgG.
Is there any way to improve diagnosis? All additional attempts to culture the pathogen failed in the past therefore there is very limited information on the resistance profile.
Is there any alternative treatment regimen which more likely would allow the eradication of the pathogen?
With best regards

klaus

Prof. Dr. med. Klaus Warnatz

MEDICAL CENTER – UNIVERSITY OF FREIBURG
Center for Chronic Immunodeficiency – CCI
Department of Rheumatology and Clinical Immunology

Breisacher Str. 117, 79106 Freiburg, Germany
Tel. +49 761 270 77640<tel:%2B49%20761%20270%2077640> / FAX -71000 / Pager 12-7100
klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>

www.uniklinik-freiburg.de/cci<http://www.uniklinik-freiburg.de/cci>

Von: Prof. Dr. Stephan Ehl [mailto:stephan.ehl at uniklinik-freiburg.de<mailto:stephan.ehl at uniklinik-freiburg.de>]
Gesendet: Donnerstag, 30. April 2015 12:18
An: CIS-PIDD
Cc: Oscar Segarra Canton
Betreff: Re: [cis-pidd] NEMO and BID

Capucine picard is currently collecting the international experience on this and should have the best overview. Best wishes, St.

Beste Grüße

Prof. Dr. Stephan Ehl
Medizinischer Direktor

UNIVERSITÄTSKLINIKUM FREIBURG
CCI - Center for Chronic Immunodeficiency

Breisacher Str. 117 - 2. OG, 79106 Freiburg i. Brsg., Germany
phone: +49(0)761.270-77300<tel:%2B49%280%29761.270-77300>
Sekretariat +49(0)761.270-77550<tel:%2B49%280%29761.270-77550>  fax +49(0)761.270-77600<tel:%2B49%280%29761.270-77600>
e-mail: stephan.ehl at uniklinik-freiburg.de<mailto:stephan.ehl at uniklinik-freiburg.de>

Von: Pere Soler Palacin <psoler at vhebron.net<mailto:psoler at vhebron.net>>
Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Datum: Donnerstag, 30. April 2015 11:55
An: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Cc: Oscar Segarra Canton <osegarra at vhebron.net<mailto:osegarra at vhebron.net>>
Betreff: [cis-pidd] NEMO and BID

Dear all, I'd appreciate your inputs on a new case we have. He's a 15 yo boy who was followed by our gastroenterologists due to inflammatory bowel disease (diagnosis was made at 13 years of age due to arthritis and diarrhoea) that was unresponsive to steroids and azathioprine. Then, infliximab was started and PID screening was performed yielding a mutation in the X-linked NEMO gene both mother and sister are carriers of the mutation). My questions are:
- Do you have any experience in NEMo deficiency patients presenting only with Crohn-like phenotype?
- I'm worried about the risk of mycobacteriosis in a patient with NEMO-def receiving infliximab. Would you consider any other therapeutic option?
- The patient is doing clinically well, should SCT be considered in this case?

Thanks in advance and best regards from Barcelona,

P.

Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron
Assistant Professor. Universitat Autònoma de Barcelona (UAB)
Pg. de la Vall d'Hebron, 119-129
08035 Barcelona. Spain.
Tel. 0034934893140 /  Fax 0034934893039

psoler at vhebron.net<mailto:psoler at vhebron.net>  /  34660psp at comb.cat<mailto:34660psp at comb.cat>
Web: www.upiip.com<http://www.upiip.com/>
ORCIDID:http://orcid.org/0000-0002-0346-5570
Scopus Author ID:http://www.scopus.com/authid/detail.url?authorId=55923378300
ResearchGate:http://www.researchgate.net/profile/Pere_Soler-Palacin
LinkedIn: http://es.linkedin.com/pub/pere-soler-palac%C3%ADn/73/918/b16


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--
Jasmeen S. Dara, MD MSc
Fellow in Allergy & Immunology
Albert Einstein College of Medicine
Children's Hospital at Montefiore

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