[CIS PIDD] [cis-pidd] Recurrent hand, foot and mouth disease with normal immune work up.

Thu Feb 2 00:16:10 EST 2017

TLR3 you can ask from prof J-L Casanova, Rockefeller

T differential: at least Mayo/ prof Roshini Abrahams, I believe. But I think a number of more close by flow labs will be able to do that as well.

STAT2, ask Seattle if they can test?

?

Oyl Mikko Seppänen
Harvinaissairauksien yksikkö (HAKE)
[X]

Head, Rare Disease Center,
Helsinki University Hospital (HUH)
FINLAND

phone +358 947180201
GSM +358 50 4279606
fax +358 9 47174703

CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>> kirjoitti 2.2.2017 kello 3.44:

Thanks Dr. Seppanen
Problem is where to send TLR assays?
Also, anything in particular for advance T cell analysis( other than Navieve/Memory cells)?
Regards
Ejaz

-----Original Message-----
From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Wednesday, February 01, 2017 5:35 AM
To: CIS-PIDD
Subject: [cis-pidd] VS: Recurrent hand, foot and mouth disease with normal immune work up.

To add to Karl's list, a.o. STAT2 comes to mind as well, wondering also about the theoretical (animal data) possibility of a TLR3 pathway defect....

...and 4 relapses make different strains from the environment and able to cause hand and foot phenotype rather unlikely of course, like the question already implies.

Also: T cell subtyping, who deep an analysis was made? Are all mostly naives? RTE? Tg/d? (Here the lack of sufficient age-matched control group data causes a bit of difficulty.)

Mikko

BTW Karl:  In our rather old material, recurrent HSV1 stomatitis seemed to be associated with HLA homozygosity...( Clin Infect Dis. 2001 Nov 1;33(9):1604-7.) Have you ever been able to check for that? Would love to know if true or coincidental...

Mikko Seppänen
Head
Hospital District of Helsinki and Uusimaa, Children's Hospital, Rare Disease Center P.O.Box 280, 00029 HUS |Lastenlinnantie 2, Helsinki Senior Consultant Hospital District of Helsinki and Uusimaa, Inflammation Center, Adult Primary Immunodeficiency Outpatient Clinic mikko.seppanen at hus.fi<mailto:mikko.seppanen at hus.fi>
+358 9 47180201| +358 50 4279606
faxi +358 9 47174703

-----Alkuperäinen viesti-----
Lähettäjä: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Lähetetty: 1. helmikuuta 2017 11:56
Vastaanottaja: CIS-PIDD <cis-pidd at lyris.dundee.net<mailto:cis-pidd at lyris.dundee.net>>
Aihe: RE:[cis-pidd] Recurrent hand, foot and mouth disease with normal immune work up.

Dr. Yousef --

Sounds very similar to the usual case I get of recurrent HSV-1 stomatitis.  Difference is, of course, you do not have the option of standing antiviral prophylaxis.

I would suggest that the workup is not normal.  I would recommend checking antiviral titers (rubella and varicella would be the easiest, if the child's vaccinated), the  as well as doing the formal pneuomococcal polysaccharide vaccine challenge.

I leave it to the more experienced members of the group to give a proper ranked differential.   Though, of course, recurrent viral infection would lead one to look at the pure B cell defects, plus some SCIDs, NK defects, WHIM, and STAT1.  If the patient actually does not mount a proper polysaccharide response (which may be the reason he needed the tympanostomy tubes to begin with), then it's a wider problem.

 - K

Karl O. A. Yu, M.D., Ph.D., F.A.A.P.
Section of Infectious Diseases  |  Department of Pediatrics  |  Comer Children's Hospital  |  University of Chicago
5841 S Maryland Ave,  MC 6054,  Chicago  IL  60637
Office phone: 773-702-9281  |  Pager: 773-702-6800 x1744  |  Fax: 773-702-1196

________________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>]
Sent: Tuesday, January 31, 2017 3:28 PM
To: CIS-PIDD
Subject: [cis-pidd] Recurrent hand, foot and mouth disease with normal immune work up.

Dear colleagues and experts: I would like to get your help on the following case:

2-1/2-year-old with history of 4 episodes of hand, foot and mouth disease (in August 2015, September 2015, 08/01/2016 and 09/03/2016). Each episode presented with vesicles on the hands, feet and in the mouth associated with fever. The patient had a nasal swab on 09/01/2016 that showed enterovirus. The patient was evaluated in this clinic on 09/19/2016 and had immune work up that came back normal. Since last visit, John has done well without any medical problems till 01/07/2017, when he developed fever and vesicular rash on the hands, feet and the tongue.

Past Medical History: The patient was born prematurely at 33 weeks' gestation and he stayed in the NICU for 8 weeks and he was diagnosed with apnea and bradycardia and gastroesophageal reflux. He has asthma and allergic rhinitis and he had recurrent illnesses and had tympanostomy tubes placement in March of 2016.

Laboratory evaluation: On 09/19/2016, IgE 6, IgG: 598, IgA: 53, IgM: 74, IgG 1: 372 , IgG2: 85, IgG3: 42, IgG4: 3.7, white blood cell count: 10.6, poly 31%, lymph 57%, mono 8%, hemoglobin: 12.4, hematocrit: 36.6, platelets count: 422, Tand B cell subsets normal, pneumococcal titer with no response and tetanus antibody 1.56.

Impression: Recurrent hand, foot and mouth disease with normal immune work up.

Any suggestion regarding  any further work up would be appreciated

Ejaz Yousef MD
Nemours Children Specialty Care
Jacksonville, FL

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