[CIS-PAGID] case

[Routes, John]

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