[CIS PIDD] [cis-pidd] FW: Neonate with septic arthritis and possible aortic fungal thombus.

[Daniel H. Conway]

For further work-up:

 

1.       TLR assay for identification of signaling defects.

2.      Some form of genome hybridization to identify two X chromosomes, any
regions of homology, and exclude DiGeorge (which the T cell number seems to
do also).  

 

"Normal immunity" should be considered if one can find a history of an
umbilical artery catheter which caused thrombosis and was seeded from E.
coli resulting in an intra-arterial focus for which likely antibiotic
duration for "mere" sepsis would have been inadequate.  Then one can explain
the appearance of metastatic E coli from an undertreated infected thrombus.

 

Dan Conway

 

From: Jason W. Caldwell [mailto:jcaldwel at wakehealth.edu] 
Sent: Wednesday, January 02, 2013 1:35 PM
To: CIS-PIDD
Subject: [cis-pidd] FW: Neonate with septic arthritis and possible aortic
fungal thombus.

 

 

 

Happy holidays to everyone.

 

I have a patient that I was consulted on that I need some help with. I will
try to be brief and concise as possible. 

 

This is a former 34 week infant who is now 2-1/2 months old. She was born in
West Virginia. She was large for gestational age secondary to gestational
diabetes. The mother's 35 and G3 P1 A1. The prenatal labs were unremarkable.
The birth was unremarkable and the pregnancy was only complicated with
gestational diabetes. The parents deny consanguinity.

 

Shortly after birth she was diagnosed with ESBL Escherichia coli sepsis. She
was placed on meropenem. At the outside hospital they were not seen marked
improvement with her clinical course and started Zosyn. She was noted to
have abdominal distention and an abdominal CT was done showing an occlusive
infrarenal abdominal aortic clot.  Prior to coming to our facility for some
unknown reason she was given 400 mg per kilogram of IV Ig on November 2.
She is also been noted to have thrombocytopenia with a platelet count of
37,000 and received to platelet transfusions although now she has
thrombocytosis with a maximal platelet counts of a little over 700,000.

 

She ended up having an MRI of her pelvis because of a suspicious collection
of fluid near her bladder and this should a gluteal abscess which was
culture positive for Escherichia coli and the left hip showed a significant
joint effusion and synovitis. This has subsequently been drained by
orthopedic surgery and the fluid was cloudy, but no cell count was done. The
fluid did not grow any organisms. The wound is healing without
complications.  Then this little girl was improving and then began to spike
fevers again and her CRP began to trend up as did her platelets and white
blood cell count. Her maximum whites of blood count was 39,000, but
currently is 11,000.  In her situation began to decline a repeat MRI was
done showing re\re collection of fluid in the hip as well as the gluteal
abscess. The thrombus in her aorta was reevaluated and the radiologist
remarked that the thrombus could be infectious and placed a fungal thrombus
on the differential. She was started on fluconazole without improvement and
is currently on micafungin and the addition of a fungal agents has trended
with a decline in her white blood cell count and platelet count as well as
CRP.

 

She has not had pneumonia, she is on room air, she is feeding currently
orally. She was never intubated. CSF cultures and cell counts were within
normal limits or negative. And she is currently relatively stable.  There
are no rashes, no diarrhea, no vomiting, no skin pigment changes noted, and
ophthalmology exam normal, and no unusual morphology. She was noted to have
a small ASD on echo, but no other heart defects. There is a thymic shadow on
her chest x-ray.

 

I was counseled to see her secondary to "hypogammaglobulinemia", thrush,
sepsis Escherichia coli and multiple abscesses as described above.

 

My workup has revealed:

Immunoglobulins: IgG 276, IgA 23, and IgM 54 all in milligrams per
deciliter. A repeat IgG one week later was 318 mg/deciliter.

T cell and B cell numbers:  13% B cells with an absolute of 1080
cells/microliter. 80.1% CD3 T cells with an absolute of 6025 cells per
microliter. 64.4% CD4 T cells with an absolute of 5020 cells/microliter.
15.1% CD8 T cells with an absolute of 1180 cells/microliter. CD4: CD8 ratio
4.3.

Neutrophil oxidative burst: I reviewed the flow cytometry which shows 93% of
the neutrophils  bursting.   The histograms were not indicative of a carrier
state or autosomal recessive disease.

Leukocyte adhesion deficiency type I: CD18 and CD 11 markers were normal.

Lymphocyte responses to mitogens are normal and robust.

Total IgE is 2.4

During this entire admission she has had an absolute monocytosis with a peak
at 5800 cells/microliter and a nadir of 1100 cells per microliter. There is
no eosinophilia.

 

Since I have any results that are pointing me in a specific direction and I
don't have any physical findings to suggest other immune deficiency, I am
wondering if anybody else has any ideas about further workup or diagnoses to
consider.

 

I thank you very much for any time taken to review this case.

 

 

Jason W Caldwell, DO
Assistant Professor Internal Medicine & Pediatrics
Wake Forest School of Medicine
Section Pulmonary, Critical Care, Allergic, and Immunological Diseases
Medical Center Boulevard  \  Winston-Salem, NC 27157
p 336.716.5166  \  f 336.716.4743  \  pager 336.806.8330
jcaldwel at wakehealth.edu  \  WakeHealth.edu

 

 

 

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