[CIS-PAGID] case

[Juan Carlos Aldave Becerra]

Dear Dr. Routes,
I am also worried about congenital neutropenias. I had a newborn patient with congenital neutropenia and sepsis by Klebsiella.I think bone marrow smear would help.
All the best,

Juan AldaveLima-Peru

> Date: Fri, 6 Jan 2012 08:31:45 -0200

> From: dmvascon at usp.br

> To: pagid at list.clinimmsoc.org

> Subject: Re: [CIS-PAGID] case

> 

> Hi Jack, good morning

> 

> What about Autosomal dominant congenital neutropenias? ELANE and GFI1  

> are possible.

> How are her monocytes, are they elevated?

> In the event of decreasing neutrophil counts may be interesting to  

> evaluate response to G-CSF.

> 

> All the best,

> 

> Dewton Vasconcelos

> University of São Paulo

> 

> Citando "Routes, John" <jroutes at mcw.edu>:

> 

> > Appreciate your thoughts on an difficult case.

> >

> >

> > We were asked to evaluate a 6 week old female with past history of  male

> > sibling who died at age 2 months from sudden "infant death syndrome".  Later

> > autopsy demonstrated few klebsiella on culture from spleen but was otherwise

> > normal.  Thymus and spleen were normal. Our patient also had a male cousin

> > who died at age 6 months of strep pneumo sepsis. No autopsy on that child.

> > No immune studies or cbcs on dead infants.

> >

> > Our infant has a completely normal history  and physical examination.

> > So far evaluation shows-

> > CBC-Neutropenic with serial values ranging from 500s to 1100.

> > Flow--CD4-3714, CD8-1130, CD56-215, CD19 215 (low numbers of B cells,

> > everything else normal, including normal numbers of naïve T cells).

> > Normal IgG (maternal), IgA   <6 (1-40), IgM 9 (11-68).  Normal CH50,  TLR 4

> > assay-normal induction TNF.   Started on amoxicillin prophylaxis.

> >

> > We will be getting dna from brother¹s autopsy.

> >

> > Other  testing?  Treatment? Management long term?

> >

> >

> > thanks

> > Jack

> >

> > John M. Routes, MD

> > Chief, Section of Allergy and Clinical Immunology

> > Co-Director, Clinical and Translational Science Institute of Southeast WI

> > Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics

> > Department of Pediatrics

> > Children's Hospital of Wisconsin

> > Medical College of Wisconsin

> > 9000 W. Wisconsin Ave.

> > Milwaukee, WI  53226-4874

> >

> > Phone: 414-456-4802; 414-266-6997

> > Fax: 414-456-6487 (Clinical)

> > Fax: 414-456-6323 (Laboratory)

> > Email: jroutes at mcw.edu

> >

> >

> >

> >

> 

> 

> 

 		 	   		  
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