[CIS PIDD] [cis-pidd] Sick infant with multiple septic arthritis and osteomyelitis

[CIS-PIDD]

Hi All!

I am posting a case for my colleagues in Chicago:

3 month old ex 25 week premature infant  who presented with multiple septic arthritis and osteomyelitis growing MSSA (including the skull, spine, hips, knees, and long bones). He is not responding to antibiotic therapy and is now growing pathogenic serratia from the lung.  Prior to 2 weeks ago, the child had been largely healthy aside from some possible joint swelling which was mild in the weeks prior.
Labs are as follows:
He has low platelets but is septic, and the platelets are large in size. He does not have eczema.
Flow cytometry was within normal limits, and a neutrophil oxidative burst was normal. TLR function was also normal except for IL-6 response was low for poly I:C stimulation but ok for other stimulants.
IgM is high at 216 and IgG high at 273, IgE elevated at 13.8
The Th17 cell number was normal. CRP has been elevated at times which is inconsistent with the report of IL-6 neutralizing antibodies.
At this point, we do not think it is CGD, MyD88/ IRAK4, or IL-17 pathway defects.
PID genotyping panel is pending
Any help in terms of suggestions for other diagnostic tests or empiric therapy would be much appreciated.

Thanks,

Ramsay

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