Disease Information for Hypogammaglobulinemia/Agammaglobulinemia

Ads
Clinical Manifestations
Signs & Symptoms
Depigmented patches, skin
Chronic Diarrhea in a Child
Diarrhea
Diarrhea and Weight Loss
Diarrhea in Children
Diarrhea, chronic
Diarrhea, recurrent
Fatty food intolerance
Poor weight gain/child
Recurrent chest infections/bronchitis/sign
Recurrent URI/Colds/LRI infections
Short stature
Short stature Child
Weight Loss
Clinical Presentation & Variations
Presentation/Recurrent Infections
Presentation/Recurrent respiratory infections
Presentation/Agressive pneumonia Immuncompromised
Disease Progression
Course/Chronic disorder
Onset/Childhood
Onset/Infancy
Onset/Six months
Demographics & Risk Factors
Established Disease Population
Patient/Chronic lymphocytic leukemia
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Boy patient
Population/Child
Population/Children/all
Population/Older baby 6-12 months
Population/Toddler
Laboratory Tests
Microbiology & Serology Findings
Common vaccination antibodies absent/low
Abnormal Lab Findings (Non Measured)
Absent/Low B-cells on Flow Cytometry
Abnormal Lab Findings - Decreased
C4, serum (Lab)
Gamma globulin (Lab)
IGE/Immunoglobulin E (Lab)
IGG/Immunoglobulin G (Lab)
Associated Diseases & Rule outs
Rule Outs
Cystic fibrosis (mucoviscidosis)
Associated Disease & Complications
Aseptic meningitis syndrome
Asthma
Autoimmune disease
Bacterial infection, general
Bronchiectasis
Coxacki virus infection
Giardia infection/intestinal
Growth retardation/failure
Hemolytic anemia/autoimmune
Hemophilus influenza infection/Hemophilus B
Hypogammaglobulinemia
Hypogammaglobulinemia, acquired
Immune deficiency , acquired (AIDS/HIV) disease/assn
Immune suppressed status
Immunodeficiency
Infections
Malabsorption/Steatorrhea syndrome
Megaloblastic anemia/disease
Meningitis, pneumococcal
Meningococcal arthritis
Meningococcal meningitis
Meningococcemia/Meningococcus
Otitis media, chronic
Pneumococcus infection
Pneumonia
Pneumonia, bacterial
Pneumonia, pneumococcal
Pneumonia, recurrent
Poliomyelitis, acute
Rheumatoid arthritis
Sinusitis
Sinusitis, chronic
Vaccine associated paralytic poliomyelitis
Vitiligo
Asthma Children
Disease Mechanism & Classification
Class
CLASS/Humoral Immune System Disorder (ex)
CLASS/Pediatric disorders (ex)
CLASS/Hematologic (category)
CLASS/Spleen/thymus/RES/immune system (category)
Pathophysiology
Pathophysiology/Gene locus Xq21.3-q22
Pathophysiology/Infection resistant to usual treatments
Pathophysiology/Proteins/blood disorder (ex)
Pathophysiology/Fat Malabsorption
Pathophysiology/Fat soluble Vitamin Malabsorption
Pathophysiology/Chemotaxis failure/phagocytes/effect
Pathophysiology/Immune physiology/defective
Pathophysiology/Poor immune resp/encapsulated bacteria
Pathophysiology/Poor slow recovery/ bacteria infection
Process
PROCESS/Biochemical (category)
Treatment
Drug Therapy - Indication
RX/Gamma globulin/prophylaxis/low dose
Definition

Hypogammaglobulinemia is a disorder that is caused by a lack of B-lymphocytes and a resulting low level of immunglobulins (antibodies) in the blood; Immunoglobulins play a dual role in the immune response by recognizing foreign antigens and triggering a biological response that culminates in the elimination of the antigen; Antibody deficiency is associated with recurrent infections with specific types of bacteria; In pure B-cell disorders, cellular immunity generally is intact and the frequency of viral, fungal, and mycobacterial ( tuberculosis) infections is not increased; There are 5 major types of immunoglobulins: immunoglobulin G, immunoglobulin M (IgM), immunoglobulin A (IgA), immunoglobulin D (IgD), and immunoglobulin E (IgE); The most common congenital abnormalities of B lymphocyte production include:

Hypogammaglobulinemia (Common Variable Immunodeficiency)

Ig A Deficiency X-linked Agammaglobulinemia (Bruton Disease), Transient hypogammaglobulinemia of infancy,

Hypogammaglobulinemia or Common Variable Immunodeficiency (CVID); CVID is the second most common cause of hypogammaglobulinemia and affects both sexes equally; The incidence is about 1 in 50,000 people; In most patients there is a reduced amount of the immunoglobulins IgG, IgA, and IgM in the blood; It is an immune deficiency disorder that can be acquired or inherited; In most cases, there is no family history of immunodeficiency; However, in instances where more than one family member is affected, an autosomal recessive mode of inheritance is suggested; In about 5% of the cases, one or more of the family members can be found to be IgA deficient; IgA deficiency is the most common antibody deficiency syndrome, about 1 case in 700 persons; Recurrent infections may occur in as many as 50% of IgA-deficient patients, but most of these individuals are healthy; Recurrent or chronic upper and lower respiratory tract infections is common as is Giardia lamblia infections of the gastrointestinal tract; Patients with undetectable levels of IgA antibodies may develop severe allergic reactions if they receive blood products

---------[NORD 2005]---------------

(Edit)

External Links Related to Hypogammaglobulinemia/Agammaglobulinemia
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
Ads