Disease Information for Agammaglobulinemia, acquired

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Clinical Manifestations
Signs & Symptoms
Chronic Diarrhea in a Child
Diarrhea, chronic
Diarrhea, recurrent
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Children/all
Laboratory Tests
Microbiology & Serology Findings
Common vaccination antibodies absent/low
Abnormal Lab Findings - Decreased
C4, serum (Lab)
Gamma globulin (Lab)
Globulin, serum (Lab)
IGE/Immunoglobulin E (Lab)
IGG/Immunoglobulin G (Lab)
Associated Diseases & Rule outs
Associated Disease & Complications
Agammaglobulinemia
Agammaglobulinemia, acquired
Epstein-Barr disease/chronic
Hypogammaglobulinemia
Hypogammaglobulinemia, acquired
Immune deficiency , acquired (AIDS/HIV) disease/assn
Immunodeficiency
Infections
Pneumonia, pneumococcal
Pneumonia, recurrent
Pneumonia/Bronchopneumonia
Disease Mechanism & Classification
Class
CLASS/Humoral Immune System Disorder (ex)
CLASS/Immune System Disorder (ex)
CLASS/Pediatric disorders (ex)
CLASS/Hematologic (category)
CLASS/Spleen/thymus/RES/immune system (category)
Pathophysiology
Pathophysiology/Infection resistant to usual treatments
Pathophysiology/Proteins/blood disorder (ex)
Pathophysiology/Immune physiology/defective
Pathophysiology/Poor immune resp/encapsulated bacteria
Pathophysiology/Poor slow recovery/ bacteria infection
Process
PROCESS/INCIDENCE/Acquired disorder
PROCESS/Immune system disorder (ex)
PROCESS/Immunodeficiency disorder/Primary
Treatment
Drug Therapy - Indication
RX/Gamma globulin/prophylaxis/low dose
Definition

Agammaglobulinemias, Primary

Antibody Deficiency

Gammaglobulin Deficiency

Immunoglobulin Deficiency

Disorder Subdivisions

Acquired Immunodeficiency Syndrome (AIDS)

Adenosine Deaminase Deficiency, Severe Combined Antibody ns

Autosomal Recessive Agammaglobulinemia

Bruton"s Agammaglobulinemia Common

Chronic Mucocutaneous Candidiasis

DiGeorge Syndrome

Duncan"s Disease

Dysgammaglobulinemia, Janeway

hypogammaglobulinemia Transient of Infancy

Idiopathic Immunoglobulin Deficiency

IgG Subclass, Selective Deficiency of

IgG Subclasses, Selective Deficiency of

Immunodeficiency with Hyper-IgM

Janeway I

Lymphoproliferate X-Linked Syndrome

Severe Combined Immunodeficiency (SCID)

Thymic Hypoplasia, Agammaglobulinemias, Primary Included

X-Linked Agammaglobulinemia

X-Linked Agammaglobulinemia with Growth Hormone Deficiency

X-Linked Infantile Agammaglobulinemia

Primary Agammaglobulinemias are a group of inherited immune deficiencies characterized by insufficient antibodies; Antibodies are composed of certain proteins immunoglobulins that are essential to the immune system; They are produced by specialized cells (ie, B lymphocytes) that circulate in the lymphatic fluid and blood; Antibodies fight off bacteria, viruses, and other foreign substances that threaten the body; Agammaglobulinemias are also characterized by the abnormal function of specialized white blood cells called B lymphocytes; The B lymphocytes are supposed to search out and identify bacteria, viruses, or other foreign substances in the body; T lymphocytes, also known as the "killer cells," assist B lymphocytes to respond to infection and other antigens; However, in some forms of Primary Agammaglobulinemias, neither the B nor the T lymphocytes function normally;There are three types of Primary Agammaglobulinemias: X-linked Agammaglobulinemia (XLA), X-linked Agammaglobulinemia with growth hormone deficiency, and autosomal recessive Agammaglobulinemia; All of these disorders are characterized by a weakened immune system treated with hyperimmune gamma globulin injections.

[NORD 2005]

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NEJM (The New England Journal of Medicine)
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