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

Wed Dec 31 11:29:32 EST 2014

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>
Antworten an:  CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
Datum:  Wednesday 31 December 2014 13:42
An:  CIS-PIDD <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

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
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