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

[Federly, Tara, J]

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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