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

Sat May 2 05:13:34 EDT 2015

Hi Klaus,

This is probably the patient from Bonn?

J Clin Microbiol. 2010 Dec; 48(12): 4672–4676. 
Published online 2010 Sep 29. doi:  10.1128/JCM.01350-10

And I am sure you have read this? Which only tells that it will be very resistant... And often by unknown mechanisms...
Have you attempted WGS of the organism? 

Genome Announc. 2014 Sep-Oct; 2(5): e00857-14. 
Published online 2014 Sep 11. doi:  10.1128/genomeA.00857-14

T: Mikko Seppanen
Finland

> Prof. Dr. Klaus Warnatz <klaus.warnatz at uniklinik-freiburg.de> kirjoitti 1.5.2015 kello 23.39:
> 
> 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 / FAX -71000 / Pager 12-7100
> klaus.warnatz at uniklinik-freiburg.de
>  
> www.uniklinik-freiburg.de/cci
>  
> Von: Prof. Dr. Stephan Ehl [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
> Sekretariat +49(0)761.270-77550  fax +49(0)761.270-77600
> e-mail: stephan.ehl at uniklinik-freiburg.de
>  
> Von: Pere Soler Palacin <psoler at vhebron.net>
> Antworten an: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Datum: Donnerstag, 30. April 2015 11:55
> An: CIS-PIDD <cis-pidd at lyris.dundee.net>
> Cc: Oscar Segarra Canton <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  /  34660psp at comb.cat
> Web: 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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