BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuAgammaglobulinemiaBruton's agammaglobulinemia; X-linked agammaglobulinemia; Immunosuppression - agammaglobulinemia; Immunodepressed - agammaglobulinemia; Immunosuppressed - agammaglobulinemiaAgammaglobulinemia is an inherited disorder in which a person has very low levels of protective immune system proteins called immunoglobulins. Immunoglobulins are a type of antibody. Low levels of these antibodies make you more likely to get infections.AntibodyAn antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...ImageRead Article Now Book Mark Article Causes This is a rare disorder that mainly affects males. It is caused by a gene defect that blocks the growth of normal, mature immune cells called B lymphocytes.As a result, the body makes very little (if any) immunoglobulins. Immunoglobulins play a major role in the immune response, which protects against illness and infection.Immune responseThe immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful....ImageRead Article Now Book Mark Article People with this disorder develop infections again and again. Common infections include ones that are due to bacteria such as Haemophilus influenzae, pneumococci (Streptococcus pneumoniae), and staphylococci. Common sites of infection include:Gastrointestinal tract Joints Lungs Skin Upper respiratory tract RespiratoryThe words "respiratory" and "respiration" refer to the lungs and breathing.ImageRead Article Now Book Mark Article Agammaglobulinemia is inherited, which means other people in your family may have the condition. Symptoms Symptoms include frequent episodes of:Bronchitis (airway infection) BronchitisAcute bronchitis is swelling and inflamed tissue in the bronchi, the main passages that carry air to the lungs. This swelling narrows the airways, w...ImageRead Article Now Book Mark Article Chronic diarrhea DiarrheaDiarrhea is when you pass loose or watery stool.ImageRead Article Now Book Mark Article Conjunctivitis (eye infection) ConjunctivitisThe conjunctiva is a clear layer of tissue lining the eyelids and covering the white of the eye. Conjunctivitis occurs when the conjunctiva becomes ...ImageRead Article Now Book Mark Article Otitis media (middle ear infection) Otitis mediaSuspected ear infections are one of the most common reasons parents take their children to the health care provider. The most common type of ear inf...ImageRead Article Now Book Mark Article Pneumonia (lung infection) PneumoniaPneumonia is a breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). ...ImageRead Article Now Book Mark Article Sinusitis (sinus infection) SinusitisSinusitis is present when the tissue lining the sinuses become swollen or inflamed. It occurs as the result of an inflammatory reaction or an infect...ImageRead Article Now Book Mark Article Skin infections Upper respiratory tract infectionsInfections typically appear in the first 4 years of life.Other symptoms include:Bronchiectasis (a disease in which the bronchial tubes in the lungs become damaged and enlarged) BronchiectasisBronchiectasis is a disease in which the large airways in the lungs are damaged. This causes the airways to become permanently wider. Bronchiectasis...ImageRead Article Now Book Mark Article Asthma without a known causeAsthmaAsthma is a chronic disease that causes the airways of the lungs to swell and narrow. It leads to breathing difficulty such as wheezing, shortness o...ImageRead Article Now Book Mark Article Exams and Tests The disorder is confirmed by blood tests that measure levels of immunoglobulins.Tests include:Flow cytometry to measure circulating B lymphocytes Immunoelectrophoresis - serum Immunoelectrophoresis - serumSerum immunoelectrophoresis is a lab test that measures proteins called immunoglobulins in the blood. Immunoglobulins are proteins that function as ...ImageRead Article Now Book Mark Article Quantitative immunoglobulins - IgG, IgA, IgM (usually measured by nephelometry) Quantitative immunoglobulinsQuantitative nephelometry is a lab test to quickly and accurately measure levels of certain proteins called immunoglobulins in the blood. Immunoglob...ImageRead Article Now Book Mark Article Treatment Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections.Immunoglobulins are given through a vein or by injection to boost the immune system.A bone marrow transplant may be considered. Support Groups More information and support for people with agammaglobulinemia and their families can be found at:Immune Deficiency Foundation -- primaryimmune.org National Organization for Rare Disorders -- rarediseases.org/rare-diseases/agammaglobulinemia NIH Genetic and Rare Diseases Information Center -- rarediseases.info.nih.gov Outlook (Prognosis) Treatment with immunoglobulins has greatly improved the health of those who have this disorder. Without treatment, most severe infections are deadly. Possible Complications Health problems that may result include:Arthritis Chronic sinus or pulmonary disease ChronicChronic refers to something that continues over an extended period of time. A chronic condition is usually long-lasting and does not easily or quick...ImageRead Article Now Book Mark Article Pulmonary diseaseLung disease is any problem in the lungs that prevents the lungs from working properly. There are three main types of lung disease:Airway diseases -...ImageRead Article Now Book Mark Article Eczema EczemaAtopic dermatitis is a long-term (chronic) skin disorder that involves scaly and itchy rashes. It is a type of eczema. Other forms of eczema include...ImageRead Article Now Book Mark Article Intestinal malabsorption syndromesMalabsorptionMalabsorption involves problems with the body's ability to take in (absorb) nutrients from food.ImageRead Article Now Book Mark Article When to Contact a Medical Professional Contact your health care provider for an appointment if:You or your child has experienced frequent infections. You have a family history of agammaglobulinemia or another immunodeficiency disorder and you are planning to have children. Ask the provider about genetic counseling. Prevention Genetic counseling should be offered to prospective parents with a family history of agammaglobulinemia or other immunodeficiency disorders.Immunodeficiency disordersImmunodeficiency disorders occur when the body's immune response is reduced or absent.ImageRead Article Now Book Mark Article Open ReferencesReferencesCunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 236.Pai SY, Notarangelo LD. Congenital disorders of lymphocyte function. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 51.Sullivan KE, Buckley RH. Primary defects of antibody production. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 150.AllVideoImagesTogAntibodies - illustration Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens. AntibodiesillustrationAntibodies - illustration Antigens are large molecules (usually proteins) on the surface of cells, viruses, fungi, bacteria, and some non-living substances such as toxins, chemicals, drugs, and foreign particles. The immune system recognizes antigens and produces antibodies that destroy substances containing antigens. AntibodiesillustrationRelated Information Sex-linked recessive(Special Topic)Immune response(Special Topic)Respiratory(Special Topic)Autoimmune disorders(Condition)Atopic dermatitis(Condition)Malabsorption(Condition)Chronic(Special Topic)Lung disease(Condition) Review Date: 1/23/2022 Reviewed By: Stuart I. Henochowicz, MD, FACP, Clinical Professor of Medicine, Division of Allergy, Immunology, and Rheumatology, Georgetown University Medical School, Washington, DC. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. © 1997- All rights reserved. A.D.A.M. content is best viewed in IE9 or above, Firefox and Google Chrome browser.Content is best viewed in IE9 or above, Firefox and Google Chrome browser.
AgammaglobulinemiaBruton's agammaglobulinemia; X-linked agammaglobulinemia; Immunosuppression - agammaglobulinemia; Immunodepressed - agammaglobulinemia; Immunosuppressed - agammaglobulinemiaAgammaglobulinemia is an inherited disorder in which a person has very low levels of protective immune system proteins called immunoglobulins. Immunoglobulins are a type of antibody. Low levels of these antibodies make you more likely to get infections.AntibodyAn antibody is a protein produced by the body's immune system when it detects harmful substances, called antigens. Examples of antigens include micr...ImageRead Article Now Book Mark Article Causes This is a rare disorder that mainly affects males. It is caused by a gene defect that blocks the growth of normal, mature immune cells called B lymphocytes.As a result, the body makes very little (if any) immunoglobulins. Immunoglobulins play a major role in the immune response, which protects against illness and infection.Immune responseThe immune response is how your body recognizes and defends itself against bacteria, viruses, and substances that appear foreign and harmful....ImageRead Article Now Book Mark Article People with this disorder develop infections again and again. Common infections include ones that are due to bacteria such as Haemophilus influenzae, pneumococci (Streptococcus pneumoniae), and staphylococci. Common sites of infection include:Gastrointestinal tract Joints Lungs Skin Upper respiratory tract RespiratoryThe words "respiratory" and "respiration" refer to the lungs and breathing.ImageRead Article Now Book Mark Article Agammaglobulinemia is inherited, which means other people in your family may have the condition. Symptoms Symptoms include frequent episodes of:Bronchitis (airway infection) BronchitisAcute bronchitis is swelling and inflamed tissue in the bronchi, the main passages that carry air to the lungs. This swelling narrows the airways, w...ImageRead Article Now Book Mark Article Chronic diarrhea DiarrheaDiarrhea is when you pass loose or watery stool.ImageRead Article Now Book Mark Article Conjunctivitis (eye infection) ConjunctivitisThe conjunctiva is a clear layer of tissue lining the eyelids and covering the white of the eye. Conjunctivitis occurs when the conjunctiva becomes ...ImageRead Article Now Book Mark Article Otitis media (middle ear infection) Otitis mediaSuspected ear infections are one of the most common reasons parents take their children to the health care provider. The most common type of ear inf...ImageRead Article Now Book Mark Article Pneumonia (lung infection) PneumoniaPneumonia is a breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). ...ImageRead Article Now Book Mark Article Sinusitis (sinus infection) SinusitisSinusitis is present when the tissue lining the sinuses become swollen or inflamed. It occurs as the result of an inflammatory reaction or an infect...ImageRead Article Now Book Mark Article Skin infections Upper respiratory tract infectionsInfections typically appear in the first 4 years of life.Other symptoms include:Bronchiectasis (a disease in which the bronchial tubes in the lungs become damaged and enlarged) BronchiectasisBronchiectasis is a disease in which the large airways in the lungs are damaged. This causes the airways to become permanently wider. Bronchiectasis...ImageRead Article Now Book Mark Article Asthma without a known causeAsthmaAsthma is a chronic disease that causes the airways of the lungs to swell and narrow. It leads to breathing difficulty such as wheezing, shortness o...ImageRead Article Now Book Mark Article Exams and Tests The disorder is confirmed by blood tests that measure levels of immunoglobulins.Tests include:Flow cytometry to measure circulating B lymphocytes Immunoelectrophoresis - serum Immunoelectrophoresis - serumSerum immunoelectrophoresis is a lab test that measures proteins called immunoglobulins in the blood. Immunoglobulins are proteins that function as ...ImageRead Article Now Book Mark Article Quantitative immunoglobulins - IgG, IgA, IgM (usually measured by nephelometry) Quantitative immunoglobulinsQuantitative nephelometry is a lab test to quickly and accurately measure levels of certain proteins called immunoglobulins in the blood. Immunoglob...ImageRead Article Now Book Mark Article Treatment Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections.Immunoglobulins are given through a vein or by injection to boost the immune system.A bone marrow transplant may be considered. Support Groups More information and support for people with agammaglobulinemia and their families can be found at:Immune Deficiency Foundation -- primaryimmune.org National Organization for Rare Disorders -- rarediseases.org/rare-diseases/agammaglobulinemia NIH Genetic and Rare Diseases Information Center -- rarediseases.info.nih.gov Outlook (Prognosis) Treatment with immunoglobulins has greatly improved the health of those who have this disorder. Without treatment, most severe infections are deadly. Possible Complications Health problems that may result include:Arthritis Chronic sinus or pulmonary disease ChronicChronic refers to something that continues over an extended period of time. A chronic condition is usually long-lasting and does not easily or quick...ImageRead Article Now Book Mark Article Pulmonary diseaseLung disease is any problem in the lungs that prevents the lungs from working properly. There are three main types of lung disease:Airway diseases -...ImageRead Article Now Book Mark Article Eczema EczemaAtopic dermatitis is a long-term (chronic) skin disorder that involves scaly and itchy rashes. It is a type of eczema. Other forms of eczema include...ImageRead Article Now Book Mark Article Intestinal malabsorption syndromesMalabsorptionMalabsorption involves problems with the body's ability to take in (absorb) nutrients from food.ImageRead Article Now Book Mark Article When to Contact a Medical Professional Contact your health care provider for an appointment if:You or your child has experienced frequent infections. You have a family history of agammaglobulinemia or another immunodeficiency disorder and you are planning to have children. Ask the provider about genetic counseling. Prevention Genetic counseling should be offered to prospective parents with a family history of agammaglobulinemia or other immunodeficiency disorders.Immunodeficiency disordersImmunodeficiency disorders occur when the body's immune response is reduced or absent.ImageRead Article Now Book Mark Article Open ReferencesReferencesCunningham-Rundles C. Primary immunodeficiency diseases. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 236.Pai SY, Notarangelo LD. Congenital disorders of lymphocyte function. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 51.Sullivan KE, Buckley RH. Primary defects of antibody production. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 150.