[CIS PIDD] [cis-pidd] Chronic norovirus enteritis, update and Q

Sat Jan 3 21:02:53 EST 2015

Dear Mikko:

Indeed very interesting case, I hope soon you can help your patient to
improve.

Some things you mentioned [i.e. ITP + AINP (Evans) + splenomegaly + a non
specific inflammatory bowel disease + lung inflammation with mild
follicular bronchiolitis] fit well into ALPS or ALPS-like syndrome. So i
wanted to ask if DNT (CD3+TCRab+CD4-CD8-) had been determined, as well as
vitamin B12, sFASL, IL-10 levels, IL-18 levels, lymphocyte apoptosis tests,
etc. I know ALPS or ALPS-like syndrome is a diagnosis not easy to fit in,
but considering the difficult scenario, just wanted to mention it.

And as previos mails have mentioned, I think rapamycin should be tried. A
previous report, mentions on a simiar case:

Pleconaril-resistant chronic parechovirus-associated enteropathy in
agammaglobulinaemia. by
van de Ven AA
<http://www.ncbi.nlm.nih.gov/pubmed/?term=van%20de%20Ven%20AA%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
1, Douma JW
<http://www.ncbi.nlm.nih.gov/pubmed/?term=Douma%20JW%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, Rademaker C
<http://www.ncbi.nlm.nih.gov/pubmed/?term=Rademaker%20C%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, van Loon AM
<http://www.ncbi.nlm.nih.gov/pubmed/?term=van%20Loon%20AM%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, Wensing AM
<http://www.ncbi.nlm.nih.gov/pubmed/?term=Wensing%20AM%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, Boelens JJ
<http://www.ncbi.nlm.nih.gov/pubmed/?term=Boelens%20JJ%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, Sanders EA
<http://www.ncbi.nlm.nih.gov/pubmed/?term=Sanders%20EA%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
, van Montfrans JM
<http://www.ncbi.nlm.nih.gov/pubmed/?term=van%20Montfrans%20JM%5BAuthor%5D&cauthor=true&cauthor_uid=21685550>
.
Antivir Ther.
<http://www.ncbi.nlm.nih.gov/pubmed/?term=norovirus+AND+pleconaril#>
2011;16(4):611-4.
doi: 10.3851/IMP1792.

reported quote: "pleconaril did not have any effect on viral replication.
Symptoms improved on immunosuppressive therapy, suggesting
infection-related immune dysregulation in an immunocompromised host."

Salutes.

On Sat, Jan 3, 2015 at 12:19 PM, Seppänen Mikko <Mikko.Seppanen at hus.fi>
wrote:

> Thanks for all of Your kind replies!
>
> Philipp: no I have not, a good suggestion. I have tried to find info on
> newer agents and their activity against norovirus, seems like not much has
> been published..
>
> Klaus: certainly we did check for monoallelic CTLA4 mutations.
>
> My thanks to Drs Bleesing and Sokolic also for invaluable information on
> colostrum and p.o. IgG dosing! If needed I will try all of these if
> possible, in the patient's case this seems vital... including Entocort or
> even rapamycin...
>
> Mikko Seppänen, Finland
>
>
> ________________________________________
> Lähettäjä: philipp.henneke at uniklinik-freiburg.de [
> philipp.henneke at uniklinik-freiburg.de]
> Lähetetty: 31. joulukuuta 2014 19:57
> Vastaanottaja: CIS-PIDD
> Kopio: CIS-PIDD
> Aihe: Re: [cis-pidd] Chronic norovirus enteritis, update and Q
>
> Have you already tried nitazoxanide, which has good activity against
> norovirus? It may be worth a try.
> Regards
> Philipp
>
> Philipp Henneke, MD
> Professor
> Head Ped. Infectious Dis & Rheumatology
> UNIVERSITY MEDICAL CENTER FREIBURG
> Center for Chronic Immundefiency (CCI)
> Center for Pediatrics & Adolesc. Medicine
> phone assist/ office + 49(0)761270 77640
> mobil +49 (0)162 285 2481
> fax +49 (0) 761 270 77600
> Breisacherstr. 117-2.OG, 79106 Freiburg
> Germany
> philipp.henneke at uniklinik-freiburg.de
> http://www.cci.uniklinik-freiburg.de
>
>
>
> -----Bodo Grimbacher <bodo.grimbacher at uniklinik-freiburg.de> schrieb:
> -----
> An: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org>
> Von: Bodo Grimbacher <bodo.grimbacher at uniklinik-freiburg.de>
> Datum: 31.12.2014 17:34
> Betreff: Re: [cis-pidd] Chronic norovirus enteritis, update and Q
>
> When I was an MD student in Freiburg (so many years ago:),
> our Hospital's “Milk-unit” also offered human colostrum.
> I wonder whether this is still available?
> Best wishes for the New Year 2015!
> Yours, 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
>
> Von: "Bleesing, Jacob" < Jack.Bleesing at cchmc.org<mailto:
> Jack.Bleesing at cchmc.org>>
> Antworten an: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:
> cis-pidd at lists.clinimmsoc.org> >
> Datum: Wednesday 31 December 2014 13:42
> An: CIS-PIDD < cis-pidd at lists.clinimmsoc.org<mailto:
> cis-pidd at lists.clinimmsoc.org>>
> Betreff: RE: [cis-pidd] Chronic norovirus enteritis, update and Q
>
> Just an FYI to consider setting up in Europe (if not already underway).
>
> https://clinicaltrials.gov/ct2/show/NCT02025478
>
> We are expanding this trial to use breastmilk for norovirus – both in the
> context of BMT and PID (based on promising results in the MILK study).
>
> Regards,
>
> Jack
>
> From: Klaus Warnatz [mailto:klaus.warnatz at uniklinik-freiburg.de ]
> Sent: Wednesday, December 31, 2014 6:40 AM
> To: CIS-PIDD
> Subject: Re: [cis-pidd] Chronic norovirus enteritis, update and Q
>
> Hi Mikko,
>
> this is a problem that many of us face. After failing all your attempts:
> I do think that it is worth keeping her on gluten-free diet with the DQ8+,
> add immunosuppression. It was not clear to me whether she was on steroids
> or not.
> Immunosuppressive therapy doesn't clear the norovirus infection but
> improves the diarrhea in several patients.
> We usually start with budesonide 9mg/day (1x3mg capsule opened and
> supplied with smashed apple) if not sufficient we continue with systemic
> steroids and if that fails rapamycine.
> Did you check her for CTLA4 deficiency (decreasing B cells, GI etc) but
> could be others.
> Do you think she had NRH? portal hypertension?
>
> greetings and a happy new year to all
>
> klaus
>
> Prof. Dr. med. Klaus Warnatz
>
> UNIVERSITÄTSKLINIKUM FREIBURG
> University Medical Center Freiburg
> Center for Chronic Immunodeficiency
> Division of Rheumatology and Clinical Immunology
>
> Tel: +49-761-270-77640 / FAX -71000 / Pager: 12-7100
>
> Breisacher Str. 117, 79106 Freiburg, Germany
> klaus.warnatz at uniklinik-freiburg.de<mailto:
> klaus.warnatz at uniklinik-freiburg.de>
> http://www.uniklinik-freiburg.de/cci
>
> Am 31.12.2014 um 07:33 schrieb Mikko Sepp �nen:
>
>
> Dear all,
>
> we have now attempted to our most affected patient all other in
> Listserve-suggested means of therapy (IFN, IFN+ escalated riba, the latter
> has caused further diarrhea and vomiting) except daily p.o. IgG , to no
> avail. IFN+ escalated riba for 2.5 weeks did not clear norovirus. Due to
> the side effects and further weight loss caused by it, the patient cannot
> continue this approach.
>
> Q: Robert Sokolic suggested 5ml/day of Gamunex, mixed with orange juice.
> Was a 5% or 10% prep used at the time?
> I seem to recall that someone had recently used this approach successfully
> as well. Their dose?
>
> Patient case in more detail:
>
> The patient is 31-y young recently married female, 174 cm tall, weighs
> only 40 kg now (!!!), has CVID of unknown genetic origin (WES: no known
> genes).
>
> CVID noted after ITP --> ITP+AINP (Evans)and  S.aureus sepsis 2004,
> recurrent LRTIs since 1998. Bronchiectasis already when diagnosed, HRCT
> mild follicular bronchiolitis.
>
> EGD- and IC-scopy performed a year ago, after following celiac diet no
> active cellular inflammation which You could treat with immunomodulators,
> however severe malabsorption of protein, calories, lipid soluble vitamins
> continues....
>
> Ciprofloxacin a.s.f. attempted ex juvantibus to no avail, bacterial
> overgrowth seems unlikely. Further causes for diarrhea tested and not found
> (We are actually preparing a manuscript on GI manifestations in CVIDs in
> our cohort, this lady has been tested for all known and unknown alternative
> causes...).
>
> We are left with - in biopsies after celiac diet very moderate -
> enteropathy resembling coeliac disease with only subtotal villus atrophy
> (HLA-DQ8+), mild chronic PBC-like cholangiopathy: sinus dilation with mild
> sinusoidal fibrosis in biopsy, in ERC mild cholangipathy (Adursal), chronic
> norovirus, severe diarrhea and severe malabsorption. She also has
> gastroparesis of completely unknown origin (no SFN, no diabetes...).
>
> She further has asthma, lymphatic hyperplasia (spleen 13 cm). She receives
> s.c. Gammanorm 16.5% 120ml /week (SIC!), P-IgG before norovirus therapy
> attempts 12.9-13.1, now 10.9 g/l...(more diarrhea)
>
> She has no switched-memory B cells, during follow up loses all B cells
> from blood (no thymoma), CD4 and CD8 counts normal, NKs 0.04 (low). Her
> Treg counts are normal, though function seemed somewhat but mildly
> impaired. Rapamycin not attempted.
>
> Any further suggestions?
>
> I appreciate Your time spent, thanks in advance,
>
> Mikko Seppänen, MD, PhD
> Helsinki, Finland
> ---
> The CIS-PIDD listserv is supported by the Clinical Immunology Society
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>
>
> Not a member of CIS? Please visit www.clinimmsoc.org<
> http://www.clinimmsoc.org> to join!
>
> You are currently subscribed to cis-pidd as:
> klaus.warnatz at uniklinik-freiburg.de<mailto:
> klaus.warnatz at uniklinik-freiburg.de>.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=204020768.f427698a4bfd5db811f00eb2c8c003f5&n=T&l=cis-pidd&o=45894551
> or send a blank email to
> leave-45894551-204020768.f427698a4bfd5db811f00eb2c8c003f5 at lists.clinimmsoc.org
> <mailto:
> leave-45894551-204020768.f427698a4bfd5db811f00eb2c8c003f5 at lists.clinimmsoc.org
> >
>
>
> ---
>
> The CIS-PIDD listserv is supported by:
>
> [http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>
>
> Not a member of CIS? Please visit www.clinimmsoc.org<
> https://cis.execinc.com/edibo/Signup> to join!
>
> You are currently subscribed to cis-pidd as: jack.bleesing at cchmc.org
> <mailto:jack.bleesing at cchmc.org>.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=183824396.60ab2025d9ab67496282f5c977f33e12&n=T&l=cis-pidd&o=45894929
>
> ---
>
> The CIS-PIDD listserv is supported by:
>
> [http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org<mailto:info at clinimmsoc.org>
>
> Not a member of CIS? Please visit www.clinimmsoc.org<
> https://cis.execinc.com/edibo/Signup> to join!
>
> You are currently subscribed to cis-pidd as:
> bodo.grimbacher at uniklinik-freiburg.de<mailto:
> bodo.grimbacher at uniklinik-freiburg.de>.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=205786708.ca2828e401b5b29dc95bd08570b2b3a9&n=T&l=cis-pidd&o=45895038
>
> ---
>
> The CIS-PIDD listserv is supported by:
>
> [http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org
>
> Not a member of CIS? Please visit www.clinimmsoc.org<
> https://cis.execinc.com/edibo/Signup> to join!
>
> You are currently subscribed to cis-pidd as:
> philipp.henneke at uniklinik-freiburg.de<mailto:
> philipp.henneke at uniklinik-freiburg.de>.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=183824502.5eac8d321b098e5cb58b60e96d0a260e&n=T&l=cis-pidd&o=45895845
>
> ---
>
> The CIS-PIDD listserv is supported by:
>
> [http://www.clinimmsoc.org/UserFiles/image/cis-pidd-list-logo_v1.jpg]
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org
>
> Not a member of CIS? Please visit www.clinimmsoc.org<
> https://cis.execinc.com/edibo/Signup> to join!
>
> You are currently subscribed to cis-pidd as: mikko.seppanen at hus.fi<mailto:
> mikko.seppanen at hus.fi>.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=183824751.2106f30c0050b88ca85ab6b5148641fa&n=T&l=cis-pidd&o=45896104
>
>
> ---
> The CIS-PIDD listserv is supported by the Clinical Immunology Society
> The science & practice of human immunology
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org
>
> Not a member of CIS? Please visit www.clinimmsoc.org to join!
>
> You are currently subscribed to cis-pidd as: e.diuxe at gmail.com.
> To unsubscribe click here:
> http://lm.clinimmsoc.org/u?id=208053827.b5e84d67385b33fe7069350ba7ea6399&n=T&l=cis-pidd&o=45904899
> or send a blank email to
> leave-45904899-208053827.b5e84d67385b33fe7069350ba7ea6399 at lists.clinimmsoc.org

-- 
Armando Partida Gaytán
e.diuxe at gmail.com
dr.partida.g at gmail.com

*Alergia e Inmunología Clínica Pediátrica*
*Hospital Infantil de México Federico Gómez*
*Inmunodeficiencias Primarias*
*Instituto Nacional de Pediatría*
*Maestría en Ciencias Médicas*
*Universidad Nacional Autónoma de México*

---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=45906327
or send a blank email to leave-45906327-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20150103/3ef7e414/attachment-0001.html>