Leukocyte adhesion deficiency type 1

Common Name(s)

Leukocyte adhesion deficiency type 1

Leukocyte adhesion deficiency (LAD) is an autosomal recessive disorder of neutrophil function resulting from a deficiency of the beta-2 integrin subunit of the leukocyte cell adhesion molecule. The leukocyte cell adhesion molecule is present on the surface of peripheral blood mononuclear leukocytes and granulocytes and mediates cell-cell and cell-extracellular matrix adhesion. LAD is characterized by recurrent bacterial infections; impaired pus formation and wound healing; abnormalities of a wide variety of adhesion-dependent functions of granulocytes, monocytes, and lymphocytes; and a lack of beta-2/alpha-L, beta-2/alpha-M, and beta-2/alpha-X expression.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Leukocyte adhesion deficiency type 1" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Leukocyte adhesion deficiency type 1" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Leukocyte adhesion deficiency type 1" returned 10 free, full-text research articles on human participants. First 3 results:

Prenatal diagnosis of leukocyte adhesion deficiency type-1 (five cases from iran with two new mutations).
 

Author(s): Behnaz Esmaeili, Mohsen Ghadami, Mohammad Reza Fazlollahi, Shirin Niroomanesh, Lida Atarod, Zahra Chavoshzadeh, Zeinab Moradi, Zahra Alizadeh, Zahra Pourpak

Journal: Iran J Allergy Asthma Immunol. 2014 Feb;13(1):61-5.

 

Leukocyte adhesion deficiency type-1(LAD-1) is one of the immunodeficiency autosomal recessive diseases that results from mutation in integrin, beta 2 (complement component 3 receptor 3 and 4 subunit) ITGB2 gene. The aim of this study was to investigate molecular prenatal diagnosis ...

Last Updated: 16 Dec 2013

Go To URL
Allogeneic hematopoietic stem cell transplantation in leukocyte adhesion deficiency type 1: a single center experience.
 

Author(s): Hasan Al-Dhekri, Hamoud Al-Mousa, Mouhab Ayas, Saleh Al-Muhsen, Abdulaziz Al-Ghonaium, Ghanam Al-Ghanam, Bandar Al-Saud, Rand Arnaout, Amal Al-Seraihy, Ali Al-Ahmari, Abdullah Al-Jefri, Mohammed Al-Mahr, Hassan El-Solh

Journal: Biol. Blood Marrow Transplant.. 2011 Aug;17(8):1245-9.

 

Leukocyte adhesion deficiency type 1 is a rare autosomal recessive immunodeficiency disorder. The severe phenotype is fatal unless hematopoietic stem cell transplantation (HSCT) is performed. A retrospective analysis was performed in 11 patients with leukocyte adhesion deficiency ...

Last Updated: 18 Jul 2011

Go To URL
Successful treatment of Fusarium solani ecthyma gangrenosum in a patient affected by leukocyte adhesion deficiency type 1 with granulocytes transfusions.
 

Author(s): Fethi Mellouli, Habib Ksouri, Ridha Barbouche, Mongi Maamer, Leila Ben Hamed, Slama Hmida, Assia Ben Hassen, Mohamed BĂ©jaoui

Journal:

 

Ecthyma gangrenosum (EG) manifests as a skin lesion affecting patients suffering extreme neutropenia and is commonly associated with Pseudomonas aeruginosa in immunocompromised patients. Leukocyte adhesion deficiency I (LAD I) which count among primary immunodeficiency syndromes of ...

Last Updated: 19 Oct 2010

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Leukocyte adhesion deficiency type 1" returned 0 free, full-text review articles on human participants.

 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

Apheresis of Patients With Immunodeficiency
 

Status: Recruiting

Condition Summary: LAD-1; DOCK8; GATA2 Deficancy

 

Last Updated: 16 Jan 2015

Go to URL
Unrelated Hematopoietic Stem Cell Transplantation(HSCT) for Genetic Diseases of Blood Cells
 

Status: Recruiting

Condition Summary: Sickle Cell Disease; Thalassemia; Anemia; Granuloma; Wiskott-Aldrich Syndrome; Chediak Higashi Syndrome; Osteopetrosis; Neutropenia; Thrombocytopenia; Hurler Disease; Niemann-Pick Disease; Fucosidosis

 

Last Updated: 22 Jun 2011

Go to URL