Agammaglobulinemia or Hypogammaglobulinemia is a rare congenital immunodeficiency disease. This type is characterized by lack of B cells that are mature and can lead to severe deficiency in antibody production. The presence of it could reflect an immediate effect after the maternal immunoglobulins fade away at about six months of age.
Epidemiology
Agammaglobulinemia is an inherited disorder that follows a pattern of X- shaped segregation. Since chimeric genes are often inherited, in cases where patients are affected, it is the mothers who are carriers. Around 50% of patients can be found to have a hereditary family history of the disease and 15%-20% of mutations emerge without any causative genetic background.
XLA, the incidence rate of which is about 1 in 200,000 newborns [in the United States], is an extremely rare inherited and sex-linked disorder.
Anatomy
Pathophysiology
BTK mainly functions via the stimulation of a pro B-cell differentiation to a pre-B cell. The mutation in BTK gene results in an arrest in B lymphocyte development which ultimately leaves a very short production of the fully developed type with reduced circulating mature B-cells. After that, B cells give rise to plasma cells which form IgG, IgA, IgE and IgD antibodies in large amounts, therefore providing complete immune protection, as defined by the numbers and types of these antibodies.
Immunoglobulins, including IgG, IgM, IgA and IgD, are responsible for handling extracellular infections, with encapsulated pyogenic bacteria especially. This group comprises Streptococcus pneumoniae, Haemophilus influenzae type B, Streptococcus pyogenes and a part of Pseudomonas species.
Etiology
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
Genetics
Prognostic Factors
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
Clinical History
Aged 0 to 2 years or even early childhood, agammaglobulinemia shows up to most cases which may cause continuous bacterial infections, especially in the respiratory system. It is not always that the onset of agammaglobulinemia is severe in adults, but they would also have complications that might have resulted from the condition having gone undiagnosed or untreated such as bronchiectasis, chronic lung disease or autoimmune disorders.
Physical Examination
Skin: Skin infections or chronic wound recurrences signify. Head and Neck: symptoms consistent with the upper respiratory infections. Oropharyngeal: Oral candidiasis or recurrent aphthous ulcers are just some of the symptoms. Chest: irregular breathing sounds commonly heard in patients with lung ailments are the basis of the criterion.
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Differential Diagnoses
XLA has SCID phenomenon characteristics with relatively lower T-cell counts while the latter typically maintain T-cell types number that is comparable to average type XLA.
On the contrary, such a condition must be accompanied by a small number of B cells than that which is the case with CVID, despite this being a common trend.
THI manifested by its typical feature of lowering the level of IgG with or without decreasing the IgA and IgM as well. This condition is usually a short one and improves with age. The children under 4 years of age may be suffering from the suppression of mother’s antibodies (IgG) on fetal immunoglobulin production.
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Immunoglobulin Replacement Therapy (IRT): Provides antibodies for life, thus securing the human body from diseases. infusion is either subcutaneous or intravenous every 3-4 weeks. Clinical response, serum immunoglobulin levels and the presence of infections are all the factors that play role in the dosage and the interval calculation. Antibiotic Prophylaxis: Use preventative antibiotics for instance, trimethoprim-sulfamethoxazole or off-the-peg penicillin, to avoid the bacterial infections. Infection Treatment: Antibiotics with both broad and myriad actions were given before the organism was identified. If the patient is battling a viral type or fungal infection, sometimes they may just need an antifungal drug, or an antiviral drug may be required. Complications Management: Consider arthritis as well as acid reflux and chronic pulmonary diseases. It may be mandatory to inject immunosuppressive medications, engage in pulmonary rehabilitation, or use supportive therapy. This often happens during the treatment which lasts until aspects of the respiratory system, either in health or disease, are restored back to their normal condition. Vaccination Strategies: Proceed with caution in case of live vaccinations since it may induce certain diseases.
Immunoglobulin Replacement Therapy (IRT): Alternative treatment for agammaglobulinemia is using injections either intravenously or subcutaneously.
Infection Prevention Measures: Hand hygiene of a daily basis, thorough distancing from persons actively infected as well as the application of inactivated vaccines.
Environmental Modifications: Avoidance of crowded or half-ventilated locality, particularly peak respiratory virus season, is a must, as also any contact with sick human or freshly received live vaccines.
Dietary and Lifestyle Measures: Bringing regular exercise into practice, keeping body intact nutrition and sound sleep as well is healthy as balanced diet in the form of fruits, vegetables, whole grains, lean proteins, etc. is helpful.
Role of subcutaneous immune globulin
Subcutaneous administration of immune globulin is another route of administering immune globulin (except in for patients treated intravenously).
IgG antibodies prevent various kinds of pathogen that includes myelin antibodies, the proinflammatory cytokines, Fc receptors, suppressor T and B cells, and so on, from going on their complement cascade. They cause a drop in peak serum IgG levels and an increase in trough levels alongside the administration of doses. Administration of SC on a weekly basis is done for maintenance of the SC steady-state IgG levels which are offered at a concentration of 160 mg/mL. as a SC intramuscular injection.
Role of Antibodies
An antibody for the blockage of the respiratory syncytial virus (RSV) in immunodefficient patients can be obtained with the passive immunization of a huMAb (humanized rat monoclonal IgG).
Palivizumab: A humanized mouse IgG monoclonal monovalent which identifies a Respiratory syncytial virus.
Immunoglobulin replacement therapy (IRT) is the main treatment of agammaglobulinemia with an aim to compensate for a lack of this protein in the blood. The infusions of IgG subcutaneously (SCIG) or intravenously (IVIG) can be used as well. On the other hand, SCIG involves the subcutaneous administration of immunoglobulins (at least weekly) into subcutaneous fatty tissue under the skin, IVIG is performed by injecting immunoglobulins directly into the circulation, which typically occurs every three to four weeks. The patients’ choice, tolerance, and convenience are the criteria that contribute to the choice of IVIG over SCIG.
IVIG is a procedure that is usually administered in clinic or admission in a hospital and infusion pump is connected to a vein where a needle is put into a vein. The well that contains the immunoglobulin media is plunged subcutaneously, usually into adipose tissue (belly; thighs; upper arms) through the subcutaneous injection process SCIG.
use-of-phases-in-managing-agammaglobulinemia
Agammaglobulinemia is a medical condition which shall be fully investigated by taking a history, doing a physical exam and drawing laboratory test results in order to verify the lack of immunoglobulins. The diagnosis step is used to do the investigations that lead to finding out the base of immunoglobulin deficiency and discriminate it from the other primary immunodeficiency disorders.
The consequent phase would comprise IRT initiation to compensate the losing antibodies, consequently, to offer renovated passive immunity against infections. IRT can be given internal injection (IVIG) or through the skin (SCIG) depending on patient case. It is basically a prevention of recurrent bacterial infections, and at the same time, it improves quality of life by giving protective antibodies to maintain those levels.
Frequent assessment of therapy treatment is required so we can evaluate the response, change the dose, and check for any adverse effects or complications. The maintenance phase is to be focused on the optimal management of agammaglobulinemia in the long run whereby the triggering of any infections and their complications may be avoided.
Preventive measures are a very important part of management of agammaglobulinemia – washing hands regularly, trying to stay away from people with similar diseases and keeping all recommended vaccination schedules. All this helps to reduce the risk of infection. Complications management is the process of timely recognition and adequate management of complications by medical care provision that includes supporting therapies, pulmonary rehabilitation, immuno- suppressive agents, or surgery as the last resort where necessary.
Agammaglobulinemia or Hypogammaglobulinemia is a rare congenital immunodeficiency disease. This type is characterized by lack of B cells that are mature and can lead to severe deficiency in antibody production. The presence of it could reflect an immediate effect after the maternal immunoglobulins fade away at about six months of age.
Agammaglobulinemia is an inherited disorder that follows a pattern of X- shaped segregation. Since chimeric genes are often inherited, in cases where patients are affected, it is the mothers who are carriers. Around 50% of patients can be found to have a hereditary family history of the disease and 15%-20% of mutations emerge without any causative genetic background.
XLA, the incidence rate of which is about 1 in 200,000 newborns [in the United States], is an extremely rare inherited and sex-linked disorder.
BTK mainly functions via the stimulation of a pro B-cell differentiation to a pre-B cell. The mutation in BTK gene results in an arrest in B lymphocyte development which ultimately leaves a very short production of the fully developed type with reduced circulating mature B-cells. After that, B cells give rise to plasma cells which form IgG, IgA, IgE and IgD antibodies in large amounts, therefore providing complete immune protection, as defined by the numbers and types of these antibodies.
Immunoglobulins, including IgG, IgM, IgA and IgD, are responsible for handling extracellular infections, with encapsulated pyogenic bacteria especially. This group comprises Streptococcus pneumoniae, Haemophilus influenzae type B, Streptococcus pyogenes and a part of Pseudomonas species.
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
Aged 0 to 2 years or even early childhood, agammaglobulinemia shows up to most cases which may cause continuous bacterial infections, especially in the respiratory system. It is not always that the onset of agammaglobulinemia is severe in adults, but they would also have complications that might have resulted from the condition having gone undiagnosed or untreated such as bronchiectasis, chronic lung disease or autoimmune disorders.
Skin: Skin infections or chronic wound recurrences signify. Head and Neck: symptoms consistent with the upper respiratory infections. Oropharyngeal: Oral candidiasis or recurrent aphthous ulcers are just some of the symptoms. Chest: irregular breathing sounds commonly heard in patients with lung ailments are the basis of the criterion.
XLA has SCID phenomenon characteristics with relatively lower T-cell counts while the latter typically maintain T-cell types number that is comparable to average type XLA.
On the contrary, such a condition must be accompanied by a small number of B cells than that which is the case with CVID, despite this being a common trend.
THI manifested by its typical feature of lowering the level of IgG with or without decreasing the IgA and IgM as well. This condition is usually a short one and improves with age. The children under 4 years of age may be suffering from the suppression of mother’s antibodies (IgG) on fetal immunoglobulin production.
Immunoglobulin Replacement Therapy (IRT): Provides antibodies for life, thus securing the human body from diseases. infusion is either subcutaneous or intravenous every 3-4 weeks. Clinical response, serum immunoglobulin levels and the presence of infections are all the factors that play role in the dosage and the interval calculation. Antibiotic Prophylaxis: Use preventative antibiotics for instance, trimethoprim-sulfamethoxazole or off-the-peg penicillin, to avoid the bacterial infections. Infection Treatment: Antibiotics with both broad and myriad actions were given before the organism was identified. If the patient is battling a viral type or fungal infection, sometimes they may just need an antifungal drug, or an antiviral drug may be required. Complications Management: Consider arthritis as well as acid reflux and chronic pulmonary diseases. It may be mandatory to inject immunosuppressive medications, engage in pulmonary rehabilitation, or use supportive therapy. This often happens during the treatment which lasts until aspects of the respiratory system, either in health or disease, are restored back to their normal condition. Vaccination Strategies: Proceed with caution in case of live vaccinations since it may induce certain diseases.
Pediatrics, General
Immunoglobulin Replacement Therapy (IRT): Alternative treatment for agammaglobulinemia is using injections either intravenously or subcutaneously.
Infection Prevention Measures: Hand hygiene of a daily basis, thorough distancing from persons actively infected as well as the application of inactivated vaccines.
Environmental Modifications: Avoidance of crowded or half-ventilated locality, particularly peak respiratory virus season, is a must, as also any contact with sick human or freshly received live vaccines.
Dietary and Lifestyle Measures: Bringing regular exercise into practice, keeping body intact nutrition and sound sleep as well is healthy as balanced diet in the form of fruits, vegetables, whole grains, lean proteins, etc. is helpful.
Pediatrics, General
Subcutaneous administration of immune globulin is another route of administering immune globulin (except in for patients treated intravenously).
IgG antibodies prevent various kinds of pathogen that includes myelin antibodies, the proinflammatory cytokines, Fc receptors, suppressor T and B cells, and so on, from going on their complement cascade. They cause a drop in peak serum IgG levels and an increase in trough levels alongside the administration of doses. Administration of SC on a weekly basis is done for maintenance of the SC steady-state IgG levels which are offered at a concentration of 160 mg/mL. as a SC intramuscular injection.
Pediatrics, General
An antibody for the blockage of the respiratory syncytial virus (RSV) in immunodefficient patients can be obtained with the passive immunization of a huMAb (humanized rat monoclonal IgG).
Palivizumab: A humanized mouse IgG monoclonal monovalent which identifies a Respiratory syncytial virus.
Pediatrics, General
Immunoglobulin replacement therapy (IRT) is the main treatment of agammaglobulinemia with an aim to compensate for a lack of this protein in the blood. The infusions of IgG subcutaneously (SCIG) or intravenously (IVIG) can be used as well. On the other hand, SCIG involves the subcutaneous administration of immunoglobulins (at least weekly) into subcutaneous fatty tissue under the skin, IVIG is performed by injecting immunoglobulins directly into the circulation, which typically occurs every three to four weeks. The patients’ choice, tolerance, and convenience are the criteria that contribute to the choice of IVIG over SCIG.
IVIG is a procedure that is usually administered in clinic or admission in a hospital and infusion pump is connected to a vein where a needle is put into a vein. The well that contains the immunoglobulin media is plunged subcutaneously, usually into adipose tissue (belly; thighs; upper arms) through the subcutaneous injection process SCIG.
Agammaglobulinemia is a medical condition which shall be fully investigated by taking a history, doing a physical exam and drawing laboratory test results in order to verify the lack of immunoglobulins. The diagnosis step is used to do the investigations that lead to finding out the base of immunoglobulin deficiency and discriminate it from the other primary immunodeficiency disorders.
The consequent phase would comprise IRT initiation to compensate the losing antibodies, consequently, to offer renovated passive immunity against infections. IRT can be given internal injection (IVIG) or through the skin (SCIG) depending on patient case. It is basically a prevention of recurrent bacterial infections, and at the same time, it improves quality of life by giving protective antibodies to maintain those levels.
Frequent assessment of therapy treatment is required so we can evaluate the response, change the dose, and check for any adverse effects or complications. The maintenance phase is to be focused on the optimal management of agammaglobulinemia in the long run whereby the triggering of any infections and their complications may be avoided.
Preventive measures are a very important part of management of agammaglobulinemia – washing hands regularly, trying to stay away from people with similar diseases and keeping all recommended vaccination schedules. All this helps to reduce the risk of infection. Complications management is the process of timely recognition and adequate management of complications by medical care provision that includes supporting therapies, pulmonary rehabilitation, immuno- suppressive agents, or surgery as the last resort where necessary.
Agammaglobulinemia or Hypogammaglobulinemia is a rare congenital immunodeficiency disease. This type is characterized by lack of B cells that are mature and can lead to severe deficiency in antibody production. The presence of it could reflect an immediate effect after the maternal immunoglobulins fade away at about six months of age.
Agammaglobulinemia is an inherited disorder that follows a pattern of X- shaped segregation. Since chimeric genes are often inherited, in cases where patients are affected, it is the mothers who are carriers. Around 50% of patients can be found to have a hereditary family history of the disease and 15%-20% of mutations emerge without any causative genetic background.
XLA, the incidence rate of which is about 1 in 200,000 newborns [in the United States], is an extremely rare inherited and sex-linked disorder.
BTK mainly functions via the stimulation of a pro B-cell differentiation to a pre-B cell. The mutation in BTK gene results in an arrest in B lymphocyte development which ultimately leaves a very short production of the fully developed type with reduced circulating mature B-cells. After that, B cells give rise to plasma cells which form IgG, IgA, IgE and IgD antibodies in large amounts, therefore providing complete immune protection, as defined by the numbers and types of these antibodies.
Immunoglobulins, including IgG, IgM, IgA and IgD, are responsible for handling extracellular infections, with encapsulated pyogenic bacteria especially. This group comprises Streptococcus pneumoniae, Haemophilus influenzae type B, Streptococcus pyogenes and a part of Pseudomonas species.
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
A single mutation in the BTK gene, located on the X chromosome, is responsible for the pre-B cell maturation alteration that results in X-linked agammaglobulinemia. 544 mutations under the splicing, missense, insertion, and deletion subtypes of which much have been associated with the disease. Genes related to B cell development that affect the occurrence of autosomal recessive agammaglobulinemia up to 15% in cases are known. ARAG is the more complex gene of this gene family spanning over relatively many chromosomes.
T and B cells perform essential immunological functions. Any viral infection or medication that affects their functionality may lead to a form of complex immune deficiency known as secondary immunodeficiency. A class of viral infections that include HIV (AIDS), impairing activity of CD4+T cells and weakening the cellular immune responses, is one example of the viral infection that causes immunodeficiency.
Aged 0 to 2 years or even early childhood, agammaglobulinemia shows up to most cases which may cause continuous bacterial infections, especially in the respiratory system. It is not always that the onset of agammaglobulinemia is severe in adults, but they would also have complications that might have resulted from the condition having gone undiagnosed or untreated such as bronchiectasis, chronic lung disease or autoimmune disorders.
Skin: Skin infections or chronic wound recurrences signify. Head and Neck: symptoms consistent with the upper respiratory infections. Oropharyngeal: Oral candidiasis or recurrent aphthous ulcers are just some of the symptoms. Chest: irregular breathing sounds commonly heard in patients with lung ailments are the basis of the criterion.
XLA has SCID phenomenon characteristics with relatively lower T-cell counts while the latter typically maintain T-cell types number that is comparable to average type XLA.
On the contrary, such a condition must be accompanied by a small number of B cells than that which is the case with CVID, despite this being a common trend.
THI manifested by its typical feature of lowering the level of IgG with or without decreasing the IgA and IgM as well. This condition is usually a short one and improves with age. The children under 4 years of age may be suffering from the suppression of mother’s antibodies (IgG) on fetal immunoglobulin production.
Immunoglobulin Replacement Therapy (IRT): Provides antibodies for life, thus securing the human body from diseases. infusion is either subcutaneous or intravenous every 3-4 weeks. Clinical response, serum immunoglobulin levels and the presence of infections are all the factors that play role in the dosage and the interval calculation. Antibiotic Prophylaxis: Use preventative antibiotics for instance, trimethoprim-sulfamethoxazole or off-the-peg penicillin, to avoid the bacterial infections. Infection Treatment: Antibiotics with both broad and myriad actions were given before the organism was identified. If the patient is battling a viral type or fungal infection, sometimes they may just need an antifungal drug, or an antiviral drug may be required. Complications Management: Consider arthritis as well as acid reflux and chronic pulmonary diseases. It may be mandatory to inject immunosuppressive medications, engage in pulmonary rehabilitation, or use supportive therapy. This often happens during the treatment which lasts until aspects of the respiratory system, either in health or disease, are restored back to their normal condition. Vaccination Strategies: Proceed with caution in case of live vaccinations since it may induce certain diseases.
Pediatrics, General
Immunoglobulin Replacement Therapy (IRT): Alternative treatment for agammaglobulinemia is using injections either intravenously or subcutaneously.
Infection Prevention Measures: Hand hygiene of a daily basis, thorough distancing from persons actively infected as well as the application of inactivated vaccines.
Environmental Modifications: Avoidance of crowded or half-ventilated locality, particularly peak respiratory virus season, is a must, as also any contact with sick human or freshly received live vaccines.
Dietary and Lifestyle Measures: Bringing regular exercise into practice, keeping body intact nutrition and sound sleep as well is healthy as balanced diet in the form of fruits, vegetables, whole grains, lean proteins, etc. is helpful.
Pediatrics, General
Subcutaneous administration of immune globulin is another route of administering immune globulin (except in for patients treated intravenously).
IgG antibodies prevent various kinds of pathogen that includes myelin antibodies, the proinflammatory cytokines, Fc receptors, suppressor T and B cells, and so on, from going on their complement cascade. They cause a drop in peak serum IgG levels and an increase in trough levels alongside the administration of doses. Administration of SC on a weekly basis is done for maintenance of the SC steady-state IgG levels which are offered at a concentration of 160 mg/mL. as a SC intramuscular injection.
Pediatrics, General
An antibody for the blockage of the respiratory syncytial virus (RSV) in immunodefficient patients can be obtained with the passive immunization of a huMAb (humanized rat monoclonal IgG).
Palivizumab: A humanized mouse IgG monoclonal monovalent which identifies a Respiratory syncytial virus.
Pediatrics, General
Immunoglobulin replacement therapy (IRT) is the main treatment of agammaglobulinemia with an aim to compensate for a lack of this protein in the blood. The infusions of IgG subcutaneously (SCIG) or intravenously (IVIG) can be used as well. On the other hand, SCIG involves the subcutaneous administration of immunoglobulins (at least weekly) into subcutaneous fatty tissue under the skin, IVIG is performed by injecting immunoglobulins directly into the circulation, which typically occurs every three to four weeks. The patients’ choice, tolerance, and convenience are the criteria that contribute to the choice of IVIG over SCIG.
IVIG is a procedure that is usually administered in clinic or admission in a hospital and infusion pump is connected to a vein where a needle is put into a vein. The well that contains the immunoglobulin media is plunged subcutaneously, usually into adipose tissue (belly; thighs; upper arms) through the subcutaneous injection process SCIG.
Agammaglobulinemia is a medical condition which shall be fully investigated by taking a history, doing a physical exam and drawing laboratory test results in order to verify the lack of immunoglobulins. The diagnosis step is used to do the investigations that lead to finding out the base of immunoglobulin deficiency and discriminate it from the other primary immunodeficiency disorders.
The consequent phase would comprise IRT initiation to compensate the losing antibodies, consequently, to offer renovated passive immunity against infections. IRT can be given internal injection (IVIG) or through the skin (SCIG) depending on patient case. It is basically a prevention of recurrent bacterial infections, and at the same time, it improves quality of life by giving protective antibodies to maintain those levels.
Frequent assessment of therapy treatment is required so we can evaluate the response, change the dose, and check for any adverse effects or complications. The maintenance phase is to be focused on the optimal management of agammaglobulinemia in the long run whereby the triggering of any infections and their complications may be avoided.
Preventive measures are a very important part of management of agammaglobulinemia – washing hands regularly, trying to stay away from people with similar diseases and keeping all recommended vaccination schedules. All this helps to reduce the risk of infection. Complications management is the process of timely recognition and adequate management of complications by medical care provision that includes supporting therapies, pulmonary rehabilitation, immuno- suppressive agents, or surgery as the last resort where necessary.
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