[CIS PIDD] [cis-pidd] Question: Immune Work up

[Kiehm, Jamie H]

I was hoping to get some insight about any further diagnostic testing or thoughts you may have on the following patient.



55 day old full term male presented at 15 days old with shortness of breath and fever.  Patient was found to have coarctation of the aorta.  Sepsis work up was remarkable for an urine culture positive for MRSA.  Patient was treated for MRSA UTI and underwent coarctation repair.  Post-operation course has been complicated by RSV infection, ARDS/Pneumonitis and failed extubation x 2.  He was also found to have congenital lobar emphysema, bilateral bronchomalacia, and left vocal cord paralysis.  BAL cultures were positive for MRSA, Klebsiella oxytoca, Acinetobacter baumannii, Stenotrophomonas maltophilia.  All four of these organisms are catalase positive.  Patient was treated with a 10 day course of antibiotics - vancomycin and ciprofloxacin.



Immune work up thus far:

FISH negative for DiGeorge Chromosome 22q11.2 and 22q13

Cytogenetics: Normal Male Karyotype

CD4 1271

CD8 3909

CD19 328

CD1656 862

Helper/Suppressor Ratio 0.33

Normal and robust lymphocyte proliferative responses to PHA and PWM.

IgA 37

IgG 602

IgM 78

G6PD 9.0

CH50 19



We are awaiting CGD assay and MPO stain.



Thank you,

Jamie Kiehm, MD
Fellow, Division of Allergy & Immunology
North Shore LIJ Health System
865 Northern Boulevard
Great Neck, NY 11201




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