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

[Verbsky, James]

DIRA would be highly unusual without a rash


James W Verbsky MD/PhD
Associate Professor of Pediatrics
Medical College of Wisconsin
Milwaukee WI, 53226
jverbsky at mcw.edu<mailto:jverbsky at mcw.edu>
414-266-6701



From: Jason W. Caldwell [mailto:jcaldwel at wakehealth.edu]
Sent: Thursday, January 03, 2013 10:42 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] FW: Neonate with septic arthritis and possible aortic fungal thombus.

The CH50 is normal.  There have been no vaccines given at this point.
I will consider sub classes.

I will look into DIRA.

Thank you for the input.


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 336.716.7277  \  pager 336.806.8330
jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu>  \  WakeHealth.edu



From: Sabiha Anis [mailto:sabiha_anis at hotmail.com]
Sent: Thursday, January 03, 2013 1:53 AM
To: CIS-PIDD
Subject: RE: [cis-pidd] FW: Neonate with septic arthritis and possible aortic fungal thombus.



Have you looked at the IgG subsets, besides complement proteins and also functional antibody status? if the child has received  any childhood vaccination?

Sabiha Anis
Asst Professor
Department of Molecular Diagnostics and Immunology
Sindh Institute of Urology and Transplantation
Karachi, Pakistan
________________________________
From: safabaris at hotmail.com
To: cis-pidd at lists.clinimmsoc.org
Subject: RE: [cis-pidd] FW: Neonate with septic arthritis and possible aortic fungal thombus.
Date: Wed, 2 Jan 2013 21:08:58 +0200
It like DIRA deficiency. the disease chracterized by neonatal osteomyelitis.

Dr.Safa Bar¹º
Çocuk Sa»l¹»¹ ve Hastal¹klar¹ Uzman¹
Çocuk Allerji-©mmünoloji


________________________________
CC: cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>
From: dsuez at dsallergy.com<mailto:dsuez at dsallergy.com>
Subject: Re: [cis-pidd] FW: Neonate with septic arthritis and possible aortic fungal thombus.
Date: Wed, 2 Jan 2013 13:04:47 -0600
To: cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>
Any complement evaluation results including CH50 and AH50?
Daniel suez, MD

On Jan 2, 2013, at 12:35 PM, "Jason W. Caldwell" <jcaldwel at wakehealth.edu<mailto:jcaldwel at wakehealth.edu>> wrote:


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
<image001.gif>
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<mailto:jcaldwel at wakehealth.edu>  \  WakeHealth.edu<http://WakeHealth.edu>



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