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

Wed Feb 1 06:02:40 EST 2017

Are monocytes/ DCs present (GATA2 deficiency)?
Philipp

*************
Philipp Henneke, MD

Professor, Head of Pediatric Infectious Diseases and Rheumatology
Center for Pediatrics &
Center for Chronic Immunodeficiency
University Medical Center Freiburg
Phone +49 (0)761 270-77640
Mobile +49 (0) 162 285 2481
Fax      +49 (0)761 270-77600

philipp.henneke at uniklinik-freiburg.de
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Postal address:
Center for Chronic Immunodeficiency in
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Am 01.02.17 11:35 schrieb "CIS-PIDD" unter <cis-pidd at lists.clinimmsoc.org>:

>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
>+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>
>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]
>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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