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

[Oner Ozdemir]

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>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****

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> Center for Immunity and Immunotherapies****

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> *Seattle Children's Research Institute | University of Washington** *

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> *From:* 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

> *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****

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> Email - etzioni at rambam.health.gov.il****

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> ** **

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> ** **

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> ** **

>

> *From:* 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

> *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 ****

>

>  ****

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>

> -- ****

>

>

>  ****

>

> 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.****

>

>  ****

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-- 
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.
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