[CIS PIDD] [cis-pidd] Pt with salmonella bacteremia and adenopathy

[Conley, Mary Ellen]

I would think about CGD. We recently diagnosed CGD in a 3 year old who presented with Salmonella bacteremia and pleural effusion and a history of diarrhea. Your patient is a little old for X-CGD but he might have a hypomorphic mutation, or he might have AR-CGD.
Mary Ellen

Mary Ellen Conley, MD
West Research Tower
LeBonheur Children's Hospital
50 N. Dunlap St.
Memphis TN  38103-2800
Tel     901-287-4657
FAX  901-287-4551
mconley at uthsc.edu<mailto:mconley at uthsc.edu<mailto:mconley at uthsc.edu%3cmailto:mconley at uthsc.edu>>

From: Federly, Tara, J [mailto:tjfederly at cmh.edu]
Sent: Friday, May 24, 2013 9:12 AM
To: CIS-PIDD
Subject: [cis-pidd] Pt with salmonella bacteremia and adenopathy

Any suggestions for diagnosis/further work-up....

We saw a 14 year old Caucasian boy, previously healthy except one episode of gingivitis 6 months ago and pneumonia at age 2, presenting with salmonella enteritidis bacteremia and adenopathy (cervical and inguinal confirmed by excisional lymph node biopsy) and thrush. He and his family had a history of diarrheal illness 4 months prior to presentation, but he has no exposure to well water, raw eggs/meat, reptiles or amphibians. He was treated by ID with ciprofloxacin and fluconazole with resolution of his thrush and improvement in his lymphadenopathy. Laboratory data included normal CBC with diff, HIV antibody screen and oxidative burst. T&B cell subunits showed total T cells 63% (abs 1518), CD4 20% (482), CD8 28% (675) and total B cells 15% (361). ALPS flow testing was negative. His IgG was elevated at 4,930 and improved to 3, 010 after treatment. Immunofixation electrophoresis showed no monoclonal protein and his high IgG level was presumed to be overstimulation due to infection.

We considered an isolated episode of disseminated salmonella in an otherwise healthy boy versus immunodeficiency including complement disorder or MSMD. We have obtained further labs including CH50, extended immunodeficiency panel and MSMD screen by flow (pSTAT1/4 in response to IFNgamma, IL-12 stimulation - Seattle).

Thanks!

Tara Federly, MD
Fellow, Allergy/Asthma/Immunology
Children's Mercy Hospital
2401 Gillham Rd
Kansas City, MO 64108
(816)-960-8885


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