[CIS PIDD] [cis-pidd] 13mth male with recurrent perirectal abscesses

Fri Jan 8 16:18:25 EST 2016

Dear Araceli, we have used oral immunoglobulin in both PID and solid transplant recipients with both chronic Campylobacter spp and norovirus infection with unconclusive results. However, some clinical benefit was observed in almost of all cases despite the lack of bacterial/viral clearance. 
Related to C.diff I'd consider the use of fidaxomicin that seems to be associated with better outcome in recurrent infections 

Best, 

Pere Soler Palacín, MD, PhD, MSc. 
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'He bron. 
Vall d'Hebron Research Institute (VHIR) 
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 
ORCID ID: 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 
Linked In : http://es.linkedin.com/pub/pere-soler-palac%C3%ADn/73/918/b16 

No imprimir aquest correu ajudarà a preservar el medi ambient. 
Si vostè no és el destinatari del missatge, o l'ha rebut per error, si us plau notifiqui-ho al remitent i destrueixi el missatge amb tot el seu contingut. Està prohibida la distribució no autoritzada del contingut d'aquest missatge. 

No imprimir este correo ayudará a preservar el medio ambiente. 
Si usted no es el destinatario del mensaje, o lo ha recibido por error, notifíquelo por favor al remitente y destruya el mensaje con todo su contenido. Está prohibida la distribución no autorizada del contenido de este mensaje. 

----- Missatge original -----

De: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> 
Per: "CIS-PIDD" <cis-pidd at lyris.dundee.net> 
Enviats: Divendres, 8 de Gener 2016 22:07:47 
Assumpte: Re: [cis-pidd] 13mth male with recurrent perirectal abscesses 

Dear professors, 
I would like to seek advice on a 7 year old male with XLA, crohn's disease, recurrent episodes of C. diff colitis (x7), chronic rhinosinusitis and recurrent conjunctivitis. 
The main problem is that the patient requires multiple courses of antibiotics to treat his sinus disease and this invariable result in recurrent episodes of c. diff colitis (even when not taking antibiotics with higher risk of c. diff such as doxycicline). C. diff episodes have been sometimes associated to crohn’s flares ending up in admission. We have tried giving him metronidazole or vancomycin concomitantly administered with po antibiotics (they were continued after finishing the antibiotics) but this approach has not prevented him from getting c. diff. He has stable IgG levels on hizentra (>1000 mg/dL most of the time). He has had sinus surgery in the past, last sinus culture on 12/14 grew H. flu susceptible to ceftriaxone and meropenem. Sinusitis prevention strategy with sinus rinses using ceftazidime + budesonide, mupiracine + budesonide has not been effective. His crohn's is currently treated with remicade infusions, pentasa and probiotics and symptoms are stable. 
Any thoughts/previous experience about using human breast milk to prevent enteric/sinus disease in XLA? 
Any advice/previous experience using oral immunoglobulin to prevent c. diff infections in PID? 
Any thoughts/previous experience about fecal microbiota transplant in XLA or other PID? 
Thank you, 
Araceli Elizalde, MD 
Assistant Professor of Clinical Pediatrics 
Children’s Hospital of San Antonio 
Baylor College of Medicine 
-----Original Message----- 
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org> 
To: CIS-PIDD <cis-pidd at lyris.dundee.net> 
Sent: Fri, Jan 8, 2016 9:09 am 
Subject: Re: [cis-pidd] 13mth male with recurrent perirectal abscesses 

Although not consanguineous, how about LRBA deficiency? 
http://www.jacionline.org/article/S0091-6749(15)01365-2/abstract 

Best, Bodo 

**************************************** 
Univ.-Prof. Dr. med. B. Grimbacher 

Scientific-Director 
CCI-Center for Chronic Immunodeficiency 
UNIVERSITÄTSKLINIKUM FREIBURG 
Tel.: 0761 270-77731 Fax: -77744 
Engesserstraße 4, 79108 Freiburg 
bodo.grimbacher at uniklinik-freiburg.de 
www.uniklinik-freiburg.de/cci 

and 

Consultant Immunologist 
Institute of Immunity & Transplantation 
Dept of Immunology 
Royal Free Hospital 
UNIVERSITY COLLEGE LONDON 
Pond Street 
London NW3 2QG 
b.grimbacher at ucl.ac.uk 

www.centreforimmunodeficiency.com 

Am 06/01/16 05:03 schrieb "CIS-PIDD" unter < cis-pidd at lists.clinimmsoc.org >: 

>Thank you for your input. In response to your questions: 
> 
>- the patient is growing well. 
>- sequencing for IL-10 deficiency is pending. 
>- prior endoscopy did not extend beyond the duodenum so the terminal 
>ileum was not assessed (need to f/u with GI) 
>- had not considered XIAP 
>- where can we send neutrophil phagocytosis assay? NJH? 
> 
>Ben 
> 
>________________________________________ 
>From: CIS-PIDD [ cis-pidd at lists.clinimmsoc.org ] 
>Sent: Tuesday, January 05, 2016 8:28 PM 
>To: CIS-PIDD 
>Subject: Re: [cis-pidd] 13mth male with recurrent perirectal abscesses 
> 
>IL10 mutations, XIAP, IPEX, CD25 would be unusual if no evidence of 
>growth failure 
> 
>James 
> 
>James Verbsky MD/PhD 
>Associate Professor of Pediatrics 
>Departments of Pediatrics and Microbiology 
>Medical Director, Clinical Immunology Research Laboratory 
>Medical Director, Clinical and Translational Research 
>Medical College of Wisconsin/Children's Hospital of Wisconsin 
>Milwaukee, WI 53226 
> 
> 
>From: CIS-PIDD 
>< cis-pidd at lists.clinimmsoc.org < mailto:cis-pidd at lists.clinimmsoc.org >> 
>Reply-To: CIS-PIDD 
>< cis-pidd at lyris.dundee.net < mailto:cis-pidd at lyris.dundee.net >> 
>Date: Tuesday, January 5, 2016 at 4:28 PM 
>To: CIS-PIDD < cis-pidd at lyris.dundee.net < mailto:cis-pidd at lyris.dundee.net >> 
>Subject: [cis-pidd] 13mth male with recurrent perirectal abscesses 
> 
>Hello all, 
> 
>I have difficult case that I would appreciate your thoughts on. 13mth 
>male with recurrent perirectal abscesses and anal fistulas beginning at 
>2mths of life. Other infections include recurrent URI's, 1 episode of 
>thrush at 2 months, AOM x 10. No history of pneumonia. No abscesses 
>elsewhere. No IBD on endoscopy/colonoscopy. No known endocrinopathies. 
> 
>PMHx: 
>Eczema (mild) 
>Food allergy (eggs, milk) 
>Chronic diarrhea 
> 
>Surgeries: OR 11 times for surgical management of perirectal 
>abscesses/anal fistulas. 
> 
>FHx: no PID, no consanguinity, parents are both from Mexico. 
> 
>Labs/workup: 
>10/22/2015-bacterial culture from wound positive for fecal flora, fungal 
>culture negative 
>01/24/2015- bacterial culture from wound positive for mixed enteric flora 
>02/02/2015-wound culture positive for mixed enteric flora 
>All prior blood cultures negative 
>01/19/2015 heme occult positive 
>02/01/2015 CBC: White blood cell count 9.8, hemoglobin 9.5, platelet 
>count 381, absolute neutrophil count 4500, absolute lymphocyte count 
>4200, absolute 
>eosinophil count 400 
>09/04/2015 colon fecal calprotectin elevated at 205 
>09/17/2015: Endoscopy and colonoscopy negative for gross endoscopic 
>findings. Focal chronic inflammation noted in the stomach by pathology. 
>History of elevated inflammatory markers CRP 20.3, ESR 19 
> 
>09/21/2015: Respiratory burst normal 
>12/10/15: normal titers to tetanus/diphtheria, IgM normal, IgG elevated 
>at 1141, IgA normal, IgE elevated 599 
>12/10/15: functional testing for IL-10 receptor normal 
>12/10/15: Flow (attached): He has mild lymphopenia (ALC 3485) with 
>moderately decreased B cells (592 absolute). CD4, CD8, NK numbers are 
>all normal. Normal expression of CD11b and CD18. 
> 
> [cid: image001.png at 01D143D8.4897A630 ] 
> 
>We plan to send genetic sequencing as the functional IL-10R testing does 
>not rule out IL-10 deficiency, but I know this is quite rare and wanted 
>to query to listserve to see what else we should consider (CD25 def, 
>IPEX). 
> 
>Ben 
> 
>Benjamin L. Wright, MD| Allergy, Asthma & Clinical Immunology 
>Office Tel: 480.301.4284<tel:480.301.4284> | Fax: 
>480.301.9066<tel:480.301.9066>| Pager 127 or (79)1-5302 | 
> wright.benjamin at mayo.edu < mailto:wright.benjamin at mayo.edu > 
>Mayo Clinic | 13400 East Shea Boulevard | Scottsdale, AZ 85259 
> 
> 
>--- 
> 
>You are currently subscribed to cis-pidd as: 
> jverbsky at mcw.edu < mailto:jverbsky at mcw.edu >. 
> 
>To unsubscribe click here: 
> http://cts.dundee.net/u?id=96396710.777d26b6da96d84a8b0d5e2d208337f2&n=T&l 
>=cis-pidd&o=3396095 
> 
>(It may be necessary to cut and paste the above URL if the line is broken) 
> 
>or send a blank email to 
> leave-3396095-96396710.777d26b6da96d84a8b0d5e2d208337f2 at lyris.dundee.net <m 
>ailto: leave-3396095-96396710.777d26b6da96d84a8b0d5e2d208337f2 at lyris.dundee 
>.net> 
> 
>--- 
>You are currently subscribed to cis-pidd as: wright.benjamin at mayo.edu . 
>To unsubscribe click here: 
> http://cts.dundee.net/u?id=100174428.03717261b42ecd5bacefbb09c948dec5&n=T& 
>l=cis-pidd&o=3396458 
>or send a blank email to 
> leave-3396458-100174428.03717261b42ecd5bacefbb09c948dec5 at lyris.dundee.net 
>--- 
>You are currently subscribed to cis-pidd as: 
> bodo.grimbacher at uniklinik-freiburg.de . 
>To unsubscribe click here: 
> http://cts.dundee.net/u?id=96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a&n=T&l 
>=cis-pidd&o=3396491 
>or send a blank email to 
> leave-3396491-96396431.4f2a33ac30bfa58d3a76b5fb5b03d33a at lyris.dundee.net 

--- 
You are currently subscribed to cis-pidd as: culmenbe at aol.com . 
To unsubscribe click here: http://cts.dundee.net/u?id=96396475.2fd7b4a09abc22f35c4e114e0494359e&n=T&l=cis-pidd&o=3401387 
or send a blank email to leave-3401387-96396475.2fd7b4a09abc22f35c4e114e0494359e at lyris.dundee.net 

--- 

You are currently subscribed to cis-pidd as: psoler at vhebron.net . 

To unsubscribe click here: http://cts.dundee.net/u?id=96396854.89ed69440aad1943e77ddad00dfd36f7&n=T&l=cis-pidd&o=3401959 

(It may be necessary to cut and paste the above URL if the line is broken) 

or send a blank email to leave-3401959-96396854.89ed69440aad1943e77ddad00dfd36f7 at lyris.dundee.net 

---
You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://cts.dundee.net/u?id=96396833.5a9591ccd1e327fe6bc4d1543298c482&n=T&l=cis-pidd&o=3401986
or send a blank email to leave-3401986-96396833.5a9591ccd1e327fe6bc4d1543298c482 at lyris.dundee.net
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20160108/d4ca9950/attachment-0001.html>