[PAGID] Attached umbilical cord in 8 week old

[Dr. med. Jan Rohr]

Dear Chris,

just a thought: there are reports stating that patients suffering from 
defects in IRAK-4 also display a delay in the separation of the 
umbilical cord (which at least from a theoretical standpoint makes 
sense: if the leukocytes cannot sense the pathogen, they won´t migrate). 
These patients are extraordinarily susceptible to pneumococci and to a 
lesser extend to staphylococci. Did micobiological cultures grow any 
bugs during the pneumonias ?
IRAK-4 deficient patients usually don´t show any abnormalities in the 
routine immunological studies such as standard lymphocyte phenotyping, 
proliferation studies or immunoglobulins.

Regards,

Jan



Chris Seroogy schrieb:

> Dear Colleagues:

>

> I saw an 8 week old FT male in clinic this week with a solidly attached

> umbilical stump.  He has had two hospitalizations for pneumonia.  One at 3

> weeks (documented RSV) and the second bilateral infiltrates responsive to

> antibiotics 2 weeks ago with documented resolution.  His weight is at the

> 10%ile and down from birth (25-50%ile).  Normal immunoglobulins, normal T, B

> and NK cell numbers.  Initially with the second hospitalization he was

> slightly neutropenic (ANC 1000) and this has normalized.  He has never had a

> leukocytosis on numerous CBCs.  His flow for CD18 and CD11b is normal.  I

> intend to send DHR.  It seems he needs more PMN functional studies, but I am

> perplexed by the lack of leukocytosis.  I welcome any thoughts.  Regards,

> Chris

>

>

> Chris Seroogy, M.D.

>

> Assistant Professor

>

> Dept. of Pediatrics

>

> Mail:  H4/474 CSC, Mailstop 4108

>

> Shipping:  H4/431 CSC, Mailstop 4108

>

> 600 Highland Ave.

>

> Madison, WI  53792

>

> phone: 608- 263-2652

>

> fax: 608-265-0164

>

>

>   


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---------------------------------------------
Dr. med. Jan Rohr
Klinik I: Allgemeine Pädiatrie
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Zentrum für Kinderheilkunde und Jugendmedizin
Universitätsklinikum Freiburg
Mathildenstr. 1
79106 Freiburg
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Tel.:    +49 761-270-4300
E-mail:  Jan.Rohr at uniklinik-freiburg.de
Web:     http://www.uniklinik-freiburg.de