[CIS PIDD] [cis-pidd] Recurrent bacteremia due to capsulated microorganisms

[Seppänen Mikko]

Dear Pere, 

Very interesting, I would possibly still try to exclude:

SGD:chemotaxis and PMN hyposegmentation, if suspect, EM or gene test? 
MHCI def??
CBC x3/week, for six weeks, to exclude cyclic neutropenia

If these neg, opsonization test of vaccine resp tubes? And of course H-J not completely reliable in hyposplenism dg, but both a bit far-fetched maybe? And tough to test.

Since I have once seen a very puzzling, yet unsolved case with everything else in lab ok, very similar story and low mitogen responses (???), You might want to paradoxically test that as well? 

mikko seppänen, finland

________________________________________
Lähettäjä: Pere Soler Palacin [psoler at vhebron.net]
Lähetetty: 14. tammikuuta 2013 18:38
Vastaanottaja: CIS-PIDD
Aihe: [cis-pidd] Recurrent bacteremia due to capsulated microorganisms

Dear all, we need your help with a new patient. Here's a brief report:



3yo girl born of non-consanguineous parents and with a healthy brother.

Vaccinal schedule including pneumococcus, meningococcus and HiB: Ok.

 -20/10/2010: bacteremia due to S. pneumoniae

-19/09/11 sepsis due to N. meningitidis

- 17/10/2011 osteomielitis of unknown aetiology.



Main studies:

- No lymphopenia or neutropenia.
- Normal Ig levels (A,G, M) and IgG subclass levels.
- Response to vaccines: tetanus, Hib and pneumococcus: normal.
- Isohemagglutinins and ASTO: normal.
- C3, C4,C3PA, AP50 and CH50: normal.
- HIV serologies: NEG.
- Abdominal ultrasound: normal spleen. No Howell-Jolly bodies.
- TLR pathway assay: normal.

In the last year the patient is doing well with normal growth and no other significant infections (occasional acute otitis media).

Any suggestion?

Thnx in advance.

Pere.



Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Assistant Professor. Universitat Autònoma de Barcelona.
Passeig de la Vall d'Hebron 119-129.
08035 Barcelona. Spain.
Tel: 0034934893140. Fax: 0034934893039.
E-mail: psoler at vhebron.net<mailto:psoler at vhebron.net>; 34660psp at comb.cat<mailto:34660psp at comb.cat>. Web: www.upiip.com<http://www.upiip.com/>.



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