[CIS PIDD] [cis-pidd] Enterovirus infection

Mon Mar 7 16:18:00 EST 2016

Agree with Mike...in my experience the TLR from ARUP frequently has low responses...even in controls...and the TLR3 response needs to be done on fibroblasts since PBMCs are not reliable.  My guess is that was a false positive.  Could be a transient hypogam with the low G...would consider titers (with revaccination) and possible Ig replacement for a short period...time will tell if the humoral response normalizes but you may have to treat empirically if she is not doing well with a plan to re-analyze.

Best

james

From: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Sent: Monday, March 07, 2016 3:12 PM
To: CIS-PIDD
Subject: RE:[cis-pidd] Enterovirus infection

Hi Kathleen & co,

We had a similar case last year in a child who was traveling to SE Asia and came down with encephalitis due to type 71.  We couldn't find any other form of immunodeficiency in her, but it was nonetheless a devastating infection.

Has she had any titers checked?  If they were abnormal, I would have a very low threshold to use immunoglobulin for this child given her degree of illness.  If they were not checked, you might want to draw them and start immunoglobulin empirically.

The TLR assay is unfortunately not reliable for looking at the TLR3 pathway. For workup of this pathway, you would really be stuck referring her to the groups that specialize in these disorders (JL Casanova and co are at Rockefeller, so very close to you).

Best regards,
Mike

-------------------------

Michael D. Keller MD

Assistant Professor, Division of Allergy / Immunology

Center for Cancer and Immunology Research

Children's National Health System

111 Michigan Ave NW, M7745A

Washington, DC 20010

Clinic: 202.476.3016

Office: 202.476.5843

Fax: 202.476.2280

www.childrensnational.org<http://www.childrensnational.org>

________________________________
From: CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
Sent: Monday, March 07, 2016 3:56 PM
To: CIS-PIDD
Subject: [cis-pidd] Enterovirus infection
Dear All:  Would like your opinion on a now-12 month old girl who was seen a month ago with enterovirus meningitis.

She was a full term baby with no neonatal problems.   At 6 months of age she began attending day care and since then she has had recurrent episodes of nasal congestion associated with some fever. In between these infections she is well and has continued to grow and develop appropriately.
At the end of December the family was in Punta Cana Resort in the Dominican Republic. She had a persistent fever on New Year's Eve which did not respond to anti-ppyretics. She was brought to her pediatrician when they arrived home the next day. A vesicular rash on her hands, feet, knees and in her mouth was noted and Coxackie virus was diagnosed clinically. She had fluid in her ears and was given Cefdinir for an otitis at that visit. This appeared to resolve.

Two weeks later she again was febrile to 103F and did not defervesce with anti-pyretic agents. She was then noted to have a staring spell and would not respond to external stimuli. She was sent to the HUMC ER and was felt to have signs of seizures and symptoms of meningitis. An LP was done which indicated meningitis with 905 WBC, 50% PMN, 46% Monocytes, 4% Lymphocytes  . Coxackie was identified in CSF.  CT and MRI only indicated mild mucosal thickening in the paranasal sinuses.

Labs done in hospital: Normal CBC and diff, IgA 36 mg/dl, IgM 46, IgG low at 260 mg/dL, IgE 16
ALC 6693, 30% atypical
CD3 5555
CD4 3079
CD 8 2476
CD 56 slightly low at 134 (nl 160-950)
CD19 1071

TLR were sent to ARUP.  She responded to all ligands except Poly I:C, which corresponds to low TLR3.   As I understand it, this is associated, in mouse models at least, with susceptibility to Herpes virus, she had an enterovirus.

I am trying to get them to come back for follow up, but would love everyone's opinion as to the abnormal TRL function test.

Kathy

Kathleen A. Haines, MD
Section Chief, Pediatric Immunology
Section of Pediatric Rheumatology & Immunology
Joseph M. Sanzari Children's Hospital
30 Prospect Ave.
Hackensack, NJ 07601

T:  551-996-5306
F: 201-996-9815
Email:  Khaines at HackensackUMC.org<mailto:Khaines at HackensackUMC.org>

About Hackensack University Medical Center HackensackUMC, a 775- bed nonprofit teaching and research hospital located in Bergen County, NJ, is the largest provider of inpatient and outpatient services in the state. Founded in 1888 as the county's first hospital, it is the flagship hospital of Hackensack University Health Network, one of the largest health networks in the state comprised of 1,717 beds, more than 10,000 team members and 3,300 credentialed physicians. HackensackUMC was listed as the number one hospital in New Jersey in U.S. News & World Report's 2015-16 Best Hospital rankings - maintaining its place atop the NJ rankings since the rating system was introduced. It was also named one of the top four New York Metro Area hospitals. HackensackUMC is the only hospital in New Jersey, New York and New England to be named one of Healthgrades America's 50 Best Hospitals(tm) nine consecutive years, and receive the Healthgrades Distinguished Hospital Award for Clinical Excellence(tm) 13 years in a row. The medical center is one of the top 25 green hospitals in the country according to Practice Greenhealth, and received 24 Gold Seals of Approval(tm) by The Joint Commission - more than any other hospital in the country. It was the first hospital in New Jersey and second in the nation to become a Magnet(r) recognized hospital for nursing excellence; receiving its fifth consecutive designation in 2014. HackensackUMC has created an entire campus of award-winning care, including: the John Theurer Cancer Center; the Heart & Vascular Hospital; and the Sarkis and Siran Gabrellian Women's and Children's Pavilion, which houses the Joseph M. Sanzari Children's Hospital and Donna A. Sanzari Women's Hospital, which was designed with The Deirdre Imus Environmental Health Center and listed on the Green Guide's list of Top 10 Green Hospitals in the U.S. HackensackUMC is the Hometown Hospital of the New York Giants and the New York Red Bulls and is Official Medical Services Provider to The Barclays PGA Golf Tournament. It remains committed to its community through fundraising and community events. To learn more, visit: http://www.hackensackumc.org/

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