[CIS PIDD] Making Diagnosis of LAD -leukocyte adhesion defect- 1

[Ochs, Hans]

Hi, Oner
If this was indeed an abscess, e.g. a collection of pus, a diagnosis of LAD is not likely
hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

206-987-7450 OFFICE
206-987-7310  FAX
allgau at u.washington.edu<mailto:allgau at u.washington.edu>, hans.ochs at seattlechildrens.org<mailto:hans.ochs at seattlechildrens.org>

OFFICE   1900 Ninth Avenue, Seattle, WA 98101
MAIL      M/S C9S-7
WWW     seattlechildrens.org<http://seattlechildrens.org/>

Assistant:
Dawn Marie Pares
206-884-7418   OFFICE
dawnmarie.pares at seattlechildrens.org<mailto:dawnmarie.pares at seattlechildrens.org>

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Oner Ozdemir
Sent: Wednesday, July 11, 2012 12:46 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] Making Diagnosis of LAD -leukocyte adhesion defect- 1

Dear Dr Ochs;

Sterile means no growth in the culture. This could be due different reasons other than being sterile. We might not be able to culture the microorganism and / or antibiotic interference etc.

Thanks for your input.

Oner





On Wed, Jul 11, 2012 at 9:21 PM, Ochs, Hans <hans.ochs at seattlechildrens.org<mailto:hans.ochs at seattlechildrens.org>> wrote:
If your patient had a "sterile abscess" (with pus), the diagnosis of LAD 1 is unlikely
hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington

206-987-7450<tel:206-987-7450> OFFICE
206-987-7310<tel:206-987-7310>  FAX
allgau at u.washington.edu<mailto:allgau at u.washington.edu>, hans.ochs at seattlechildrens.org<mailto:hans.ochs at seattlechildrens.org>

OFFICE   1900 Ninth Avenue, Seattle, WA 98101
MAIL      M/S C9S-7
WWW     seattlechildrens.org<http://seattlechildrens.org/>

Assistant:
Dawn Marie Pares
206-884-7418<tel:206-884-7418>   OFFICE
dawnmarie.pares at seattlechildrens.org<mailto:dawnmarie.pares at seattlechildrens.org>

From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> [mailto:pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org>] On Behalf Of Amos Etzioni
Sent: Wednesday, July 11, 2012 5:02 AM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: Re: [CIS PIDD] Making Diagnosis of LAD -leukocyte adhesion defect- 1

Dear Oner
First of all we need to know what is the leukocyte count and the clinical symptoms ( delay separation of the cord?). If indeed the WBC is very high and there are symptoms characteristic for LAD I, I would go for genetic analysis. There are rare case ( Hogg et al. JCI 1999) in which the mutation caused a nonfunctional but expressed CD18.
As far as I know there is no fix number for CD18 expression, but one can say that normally we see more than 80% expression
Amos

Amos Etzioni MD
Professor of Pediatrics and Immunology
Director - Meyer Children's Hospital
Bat- Galim, Haifa, Israel 31096
Tel- 972-4-854 2936
Fax- 972-4-854 1870
Email - etzioni at rambam.health.gov.il<mailto:etzioni at rambam.health.gov.il>



From: pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org> [mailto:pagid-bounces at list.clinimmsoc.org<mailto:pagid-bounces at list.clinimmsoc.org>] On Behalf Of Oner Ozdemir
Sent: Wednesday, July 11, 2012 2:20 PM
To: pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>
Subject: [CIS PIDD] Making Diagnosis of LAD -leukocyte adhesion defect- 1

Dear All,

We are dealing a patient suggestive of leukocyte adhesion defect -1.

The patient is borne with C/S, G3P3, pair of twins, with sterile cerebral abscesses. And there is a sibling to this twins is 5-year-old and healthy.

CBC: normal, Ig GAME: normal, lymphocyte subpopulations: normal in the patient.

On this patient, flow cytometry revealed that borderline low CD18 expression (80 %) on lymphocytes, but neutrophil and monocyte CD18 expression within normals (above 90 %).

And the lymphocyte expressions of CD11a /CD18: 19 %; CD11b /CD18: 2 %; CD11c /CD18: 11 % seemed to be low.


My questions are:

1- Even though total CD18 expression on lymphocytes is borderline low; can we make a diagnosis of LAD with one of the lower expresions of CD11a /CD18, CD11b /CD18, CD11c /CD18 on just lymphocytes?

2- Should these low expressions of CD11a /CD18, CD11b /CD18, CD11c /CD18 with CD18 epression be on the monocytes and neutrophils in addition to lymphocytes at the same time to make a diagnosis?

3- Any normal reference values for CD11a /CD18, CD11b /CD18, CD11c /CD18 expressions in children?


Best Regards;

Oner Ozdemir, MD














--


Öner Özdemir, MD
Chief and Assoc. Prof. Pediatrics
Republic of Turkey, Ministry of Health, İstanbul Medeniyet University,
Göztepe Research and Training Hospital
Göztepe, Kadıköy, İSTANBUL -TÜRKİYE.








************************************************************************************
This footnote confirms that this email message has been scanned by
PineApp Mail-SeCure for the presence of malicious code, vandals & computer viruses.
************************************************************************************




************************************************************************************
This footnote confirms that this email message has been scanned by
PineApp Mail-SeCure for the presence of malicious code, vandals & computer viruses.
************************************************************************************


CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information protected by law. Any unauthorized review, use, disclosure or distribution is prohibited. If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message.



--
Öner Özdemir, MD
Chief and Assoc. Prof. Pediatrics
Republic of Turkey, Ministry of Health, İstanbul Medeniyet University,
Göztepe Research and Training Hospital
Göztepe, Kadıköy, İSTANBUL -TÜRKİYE.







CONFIDENTIALITY NOTICE:  This e-mail message, including any attachments, is for the sole use of the intended recipient(s) and may contain confidential and privileged information protected by law.  Any unauthorized review, use, disclosure or distribution is prohibited.  If you are not the intended recipient, please contact the sender by reply e-mail and destroy all copies of the original message.

-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20120711/799c6fe2/attachment-0001.htm>