[CIS PIDD] [cis-pidd] 13mth male with recurrent perirectal abscesses
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Wed Jan 13 11:07:05 EST 2016
Thank you for your input, it is truly appreciated
We will try fidaxomicin.
Do you have a preference on gammaglobulin formulation and dosage?
I reviewed the literature and found different ways to administer oral immunoglobulin including 150-300 mg/Kg as a single dose or 150 mg/kg q 5 hours for 5 doses.
Thank you,
Araceli
-----Original Message-----
From: CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
To: CIS-PIDD <cis-pidd at lyris.dundee.net>
Sent: Sun, Jan 10, 2016 3:14 am
Subject: VS: [cis-pidd] 13mth male with recurrent perirectal abscesses
Agree with Pere on fidaxomicin.
However, in our adult PID patients no discernible effect on nutritional status or symptoms with per oral IgG, sadly.
Worth trying anyway, since there are positive reports as well (and I personally have not needed to try on XLA patients)?
Human breast milk have not been able to attempt due to lack of it. Would seem worth trying.
Fecal transplant from next of kin:
C diff if nothing else works yes, noro and Campylo doubtful to have effect (gallbladder and biliary tract: normal in imaging?)
Best of luck!
Mikko
oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE), HUS
Mikko Seppänen, MD, PhD, Associate professor
Specialist in Internal Medicine and Infectious Diseases
Head, Rare Disease Center, Helsinki University Hospital (HUH)
Children’s Hospital, P.O.Box 280
FI-00029 HUS
FINLAND
&
Senior Consultant (PIDD)
Adult Immunodeficiency Unit
Inflammation Center, HUH
Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 8. tammikuuta 2016 23:18
Vastaanottaja: CIS-PIDD
Aihe: Re: [cis-pidd] 13mth male with recurrent perirectal abscesses
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'Hebron.
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
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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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
>
>
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