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Disease Information for Leukocyte adhesion deficiency

Clinical Manifestations

Clinical Presentation & Variations

Presentation/Lymphopenia Candidal diarrhea

Disease Progression

Course/Chronic disorder

Course/Chronic only

Course/Prognosis bad/usually

Demographics & Risk Factors

Population Group

Child

Population/Pediatrics population

Sex & Age Groups

Population/Child

Population/Child-Infant Only

Population/Children/all

Laboratory Tests

Abnormal Lab Findings (Non Measured)

Chromosomal abnormality (Lab)

Chronic lymphopenia

Abnormal Lab Findings - Decreased

Lymphocytes (Lab)

Abnormal Lab Findings - Increased

Lymphocytes (Lab)

Associated Diseases & Rule outs

Associated Disease & Complications

Candida albicans infection

Cellular immunity defect/deficiency

Immunodeficiency

Infections

Leukocyte adhesion deficiency disease

Lymphocytopenia

Lymphocytosis

Disease Mechanism & Classification

Class

CLASS/Humoral Immune System Disorder (ex)

CLASS/Leukocyte involvement/disorder (ex)

CLASS/Spleen/thymus/RES/immune system (category)

Pathophysiology

Pathophysiology/Gene locus chromosome 21

Pathophysiology/Gene Locus Identified/OMIM database

Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)

Pathophysiology/Decreased T Cells

Pathophysiology/Immune/cellular system disorder (ex)

Process

PROCESS/Congenital/developmental (category)

PROCESS/Immunodeficiency disorder/Primary

Treatment

Drug Therapy - Indication

SX/Stem cell transplant/allogenic

SX/Stem-cell transplant

Definition

Also MAC-1/LFA-1/CR3 Deficiency; An autosomal recessive disorder of WBC function characterized by recurrent or progessive necrotic soft tissue infection, periodontitis, poor wound healing, leukocytosis, and delayed (>3wk) umbilical cord detachment [merck manual 17th ed]

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External Links Related to Leukocyte adhesion deficiency

PubMed (National Library of Medicine)

NGC (National Guideline Clearinghouse)

Medscape (eMedicine)

Harrison's Online (accessmedicine)

NEJM (The New England Journal of Medicine)

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