Materiały źródłowe

• #1 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Patients with recurrent infections may benefit from prophylactic antibiotic therapy given for 6 months-or given only during the winter months in those with seasonal patterns of infections. […] Patients with SIGAD who have significant recurrent upper respiratory tract infections may benefit from a 6-month course of prophylactic antibiotics. These antibiotics may be continued if the initial course is successful. […] Some children with SIGAD who have seasonal patterns of infections may benefit from receiving prophylactic antibiotics only during the winter months. […] A small number of patients with SIGAD continue to have recurrent infections despite antibiotic prophylaxis. These patients may be treated with immunoglobulin replacement therapy; however, use of this treatment remains controversial. […] All children with SIGAD should be immunized according to the same schedule used for children without SIGAD and should receive the annual influenza vaccine.

• #2 Low IgA and Selective IgA Deficiency

  https://www.geneticlifehacks.com/low-iga-and-selective-deficiency/

  Selective IgA deficiency (SIgAD) is the most common immunodeficiency worldwide, with an estimated prevalence of 1:400. However, the prevalence varies considerably by ethnicity, being more common in Caucasian populations (about 1%). […] IgA deficiency is a genetic immunodeficiency disorder characterized by the absence or very low levels of immunoglobulin A (IgA). An essential antibody, IgA plays a critical role in protecting mucosal surfaces in the body, such as the respiratory and gastrointestinal tracts, from infection. […] IgA helps prevent infection by complexing with bacterial antigens for removal. […] Most people with IgA deficiency don’t have any noticeable symptoms, but they may be prone to getting certain types of infections more often. […] Symptoms of IgA deficiency include increased gastrointestinal tract infections, recurrent respiratory tract infections, increased risk of allergies, more frequent urinary tract infections or skin infections, and increased risk of autoimmune diseases.

• #3 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  Selective IgA deficiency (SIgAD) is the most common human primary immune deficiency (PID). It is classified as a humoral PID characterized by isolated deficiency of IgA (less than 7 mg/dL but normal serum IgG and IgM) in subjects greater than 4 years of age. […] Patients with SIgAD can be either asymptomatic or symptomatic with clinical manifestations including allergy, autoimmunity and recurrent infections mainly of the respiratory and gastrointestinal tract. […] Studies analyzing allergy on SIgAD patients showed prevalence up to 84%, supporting in most cases the relationship between sIgAD and allergic disease. […] The aim of this review is to examine the association between SIgAD and atopic disease and to update readers on advances over time at this important interface between allergy and SIgAD.

• #4 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein called immunoglobulin A. […] Selective IgA deficiency is usually inherited, which means it is passed down through families. However, there are also cases of drug-induced selective IgA deficiency. […] No specific treatment is available. Some people gradually develop normal levels of IgA without treatment. Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections. […] Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.

• #5 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. Therefore, the suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations. […] Hypotheses on the pathogenesis of allergy in SIgAD include high penetration of allergens through the mucosa due to defective serum and mucosal IgA, facilitating the contact of allergens with the local respiratory and gastrointestinal mucosal immune system. […] The association between SIgAD and allergy was found for various allergic manifestations, the most commonly described of which are asthma, allergic rhinitis, allergic conjunctivitis, urticaria, atopic dermatitis and food allergy, even if the exact prevalence of each symptom and of one clinical manifestation or another varies among the studies reported.

• #6 Selective IGA Deficiency

  https://my.clevelandclinic.org/health/diseases/17887-selective-iga-deficiency-

  Because it is inherited, selective IgA deficiency cannot be prevented. […] Selective IgA deficiency cannot be eliminated or cured, but some young children can outgrow the condition within the first few years of life.

• #7 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #8 Selective immunoglobulin A deficiency – wikidoc

  https://www.wikidoc.org/index.php/Selective_immunoglobulin_A_deficiency

  Selective IgA deficiency is inherited with a variable inheritance pattern. There are no established measures for the primary prevention. […] Effective measures for secondary and tertiary prevention of selective IgA deficiency include: Patient education, Vaccination, Use of prophylactic antibiotics. […] Patient with severe IgA deficiency may have anaphylactic reaction secondary to blood transfusion or its products. It is specifically seen in patients with undetectable serum IgA levels. These patients develop anti IgA antibodies so they should be advised to wear medical alert bracelet. […] Pneumococcal vaccine is recommended in patients with SIgAD to reduce the risk of sinopulmonary infections. […] Prophylactic antibiotics can be given to patients with SIgAD to prevent infections and other complications.

• #9 Selective ig a deficiency | PPT

  https://www.slideshare.net/slideshow/selective-ig-a-deficiency/15560176

  Selective IgA deficiency is the most common primary antibody deficiency, where there is an absence of IgA antibodies in the blood and secretions. Treatment focuses on preventing infections and managing any associated conditions. The prognosis is generally good, though there is a small risk of developing other immune deficiencies. […] There is no means of prevention of Selective IgA Deficiency. Because IgA Deficiency does not become detectable until approximately six months of age, prenatal and neonatal detection of this disorder is currently not possible. […] Management patients who do not have any symptoms do not need any treatment. prevent anaphylactic reaction secondary to blood transfusion treatment of associated diseases.

• #10 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein called immunoglobulin A. […] Selective IgA deficiency is usually inherited, which means it is passed down through families. However, there are also cases of drug-induced selective IgA deficiency. […] No specific treatment is available. Some people gradually develop normal levels of IgA without treatment. Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections. […] Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.

• #11 FloridaHealthFinder | Selective deficiency of IgA | Health Encyclopedia | FloridaHealthFinder

  https://quality.healthfinder.fl.gov/health-encyclopedia/HIE/1/001476

  Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.

• #12 Selective IgA deficiency – Humanitas.net

  https://www.humanitas.net/diseases/selective-iga-deficiency/

  While there is no known prevention for IgA deficiency, genetic counseling has been proven helpful for prospective parents with a family history of selective IgA deficiency. […] No specific treatment is available for Selective IgA deficiency. Most individuals who have bacterial infections of the ears, sinuses, lungs or gastrointestinal tract are given antibiotics to treat the infections and prevent their reoccurrence. Selective IgA deficiency that results from taking a drug usually resolves if the drug is stopped. […] Individuals with selective IgA deficiency are advised to wear a medical identification bracelet to prevent inadvertent plasma or IVIG administration, which could lead to anaphylaxis. Anaphylaxis is a severe, potentially life-threatening allergic reaction.

• #13 Is familial screening useful in selective immunoglobulin A deficiency? | Anales de Pediatría

  https://www.analesdepediatria.org/es-is-familial-screening-useful-in-articulo-resumen-S2341287915002094

  Selective immunoglobulin A deficiency (SIgAD), the most common primary immunodeficiency, is often asymptomatic. High rates of familial clustering have been described in SIgAD, but the causative genetic defect and mechanism of inheritance are unknown. […] The symptoms were not more severe in familial than sporadic SIgAD cases. Nonetheless, the elevated prevalence of affected FDRs with significant morbidity may justify routine screening of close family members of these patients. […] The elevated prevalence of SIgAD in FDRs of patients with this pathology associated with a greater clinical severity, measured indirectly through admission rates and the need for chronic treatment, shows FDRs could benefit from screening programmes. Since few relatives will need to be screened to find one positive case, this could be a cost-effective prevention strategy. This would enable individuals with this type of immunodeficiency to start treatment or prophylaxis measures to prevent hospitalisation, or to simply attend regular check-ups due to the risk of evolution to CVID.

• #14 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #15 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #16 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Patients with recurrent infections may benefit from prophylactic antibiotic therapy given for 6 months-or given only during the winter months in those with seasonal patterns of infections. […] Patients with SIGAD who have significant recurrent upper respiratory tract infections may benefit from a 6-month course of prophylactic antibiotics. These antibiotics may be continued if the initial course is successful. […] Some children with SIGAD who have seasonal patterns of infections may benefit from receiving prophylactic antibiotics only during the winter months. […] A small number of patients with SIGAD continue to have recurrent infections despite antibiotic prophylaxis. These patients may be treated with immunoglobulin replacement therapy; however, use of this treatment remains controversial. […] All children with SIGAD should be immunized according to the same schedule used for children without SIGAD and should receive the annual influenza vaccine.

• #17 Immunoglobulin A Deficiency

  https://phoenixchildrens.staywellsolutionsonline.com/134,124

  IgA deficiency is a problem that may be passed down through your family, so you cant do anything to prevent it. […] But you can limit the spread of germs and sickness by washing your hands often and staying away from large crowds. This is especially important to do during cold and flu season. Also talk with your healthcare provider about vaccines that may help prevent illness and when you should get them.

• #18 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Per the CDC, patients with IgAD are recommended to receive the pneumococcal Hib (for children aged 12-59 months) and recombinant zoster vaccines given their specific risks for infection. Vaccine contraindications in IgAD patients include the OPV, BCG, DEN4CYD, and yellow fever vaccines. Other live vaccines appear to be safe.

• #19 Immunoglobin A Deficiency and Squamous Cell Carcinoma With a Rare Presentation as Anal Cancer | Kasi | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/3804/3189

  Selective immunoglobin A deficiency (IgAD) is the most common immunodeficiency disorder in the western world. […] This case highlights the association between IgAD and malignancy. […] Patients with IgAD and other immunodeficiencies are at higher risk for HPV infection and therefore may be at a higher risk of SCC. […] With widespread use of the HPV vaccine, the medical community should be aware of its importance in cancer prevention for these patients. […] Further studies are needed to evaluate relationships between IgAD, HPV infections, SCC cancer, and the role that the HPV vaccine has in cancer prophylaxis. […] Given the evidence in the literature linking HPV infections to cancer and the reports of increased risk of HPV infection shown in immunosuppressed patients, it is clear these patients should receive prophylaxis with the 9-valent HPV vaccine.

• #20 Immunoglobin A Deficiency and Squamous Cell Carcinoma With a Rare Presentation as Anal Cancer | Kasi | Journal of Medical Cases

  https://journalmc.org/index.php/JMC/article/view/3804/3189

  Selective immunoglobin A deficiency (IgAD) is the most common immunodeficiency disorder in the western world. […] This case highlights the association between IgAD and malignancy. […] With widespread use of the HPV vaccine, the medical community should be aware of its importance in cancer prevention for these patients. […] Further studies are needed to evaluate relationships between IgAD, HPV infections, SCC cancer, and the role that the HPV vaccine has in cancer prophylaxis. […] Given the evidence in the literature linking HPV infections to cancer and the reports of increased risk of HPV infection shown in immunosuppressed patients, it is clear these patients should receive prophylaxis with the 9-valent HPV vaccine. […] The medical community should be made aware of its importance in cancer prevention for IgA-deficient individuals as well as other individuals with immunosuppression. […] Further studies should be pursued to evaluate the relationship between IgA deficiency, HPV infections, SCC cancer, and the role that the HPV vaccine has in cancer prophylaxis for these patients.

• #21 Immunodeficiency Search

  https://www.immunodeficiencysearch.com/iga-deficiency

  Selective IgA deficiency is defined as the isolated undetectable serum IgA (with normal IgG and IgM levels) in a patient older than 4 years of age. […] For patients with recurrent bacterial infections, prophylactic antibiotic therapy may be utilized. […] The addition of prophylactic antibiotics can be considered in certain patients who continue to have infections despite appropriate treatment of contributing conditions such as allergic rhinitis.

• #22 Selective Immunoglobulin A (IgA) Deficiency – Immune Disorders – MSD Manual Consumer Version

  https://www.msdmanuals.com/home/immune-disorders/immunodeficiency-disorders/selective-immunoglobulin-a-iga-deficiency

  Most people with selective IgA deficiency have few or no symptoms, but some have chronic lung infections, sinusitis, and other disorders. […] Antibiotics are used to treat or sometimes to prevent infections. […] Antibiotics are given to people who have an infection. If the disorder is severe, antibiotics are given in advance to prevent infections from developing.

• #23 Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463416/

  Selective IgA deficiency is the most common type of primary immunodeficiency, but there is not yet a specific effective treatment. […] Currently, there is no specific treatment for selective IgA deficiency. The therapeutic approach is individualized according to the clinical status of the patient. The use of antibiotic prophylaxis in patients with a history of recurrent respiratory infections may be established in specific cases of higher severity or characterized by high frequency of infectious episodes. […] In the present study, we report the use of sublingual immunotherapy (SLIT) combined activated whole-cell bacterial extract and Derp1-specific immunotherapy in a patient with partial IgA deficiency and severe persistent rhinitis and moderate persistent asthma. […] Our results suggest that the long-term therapeutic regimen with a high cumulative total load of total body extract of inactivated bacteria, similar to that proposed for specific allergen immunotherapy, may be an effective strategy to promote prophylaxis against respiratory tract infections in selective IgA deficiency.

• #24 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Patients with recurrent infections may benefit from prophylactic antibiotic therapy given for 6 months-or given only during the winter months in those with seasonal patterns of infections. […] Patients with SIGAD who have significant recurrent upper respiratory tract infections may benefit from a 6-month course of prophylactic antibiotics. These antibiotics may be continued if the initial course is successful. […] Some children with SIGAD who have seasonal patterns of infections may benefit from receiving prophylactic antibiotics only during the winter months. […] A small number of patients with SIGAD continue to have recurrent infections despite antibiotic prophylaxis. These patients may be treated with immunoglobulin replacement therapy; however, use of this treatment remains controversial. […] All children with SIGAD should be immunized according to the same schedule used for children without SIGAD and should receive the annual influenza vaccine.

• #25 Mayo Clinic Health Library – Selective IgA deficiency | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20256724

  Selective IgA deficiency may increase the risk of other conditions related to the immune system, such as allergies, asthma, rheumatoid arthritis, inflammatory bowel diseases and others. […] There is no treatment specifically for selective IgA deficiency. Treatments focus on addressing the frequent, repeat or long-lasting conditions that develop with this immune system disorder. […] Antibiotic treatments are prescribed as needed to treat bacterial disease. If you have had a long-term illness, such as chronic bronchitis, you may receive antibiotics as a preventive treatment. This therapy is called antibiotic prophylaxis (pro-fuh-LAK-sis).

• #26 AAAAI & ACAAI GUIDELINES Bundle (free trial) – Primary Immunodeficiency

  https://eguideline.guidelinecentral.com/i/802140-primary-immunodeficiency/27

  Selective IgA deficiency (SIGAD) SS 96. Subjects older than 4 years with a serum IgA level <7 mg/dL and normal serum IgG and IgM levels and in whom other causes of hypogammaglobulinemia have been excluded should be given a diagnosis of SIGAD. (C) [...] SS 98. Patients with SIGAD should be monitored over time for the occurrence of complications. (C) [...] SS 100. Aggressive antimicrobial therapy, prophylaxis, or both should be used in patients with SIGAD and recurrent sinopulmonary infections. (C) [...] SS 101. Atopic disease should be treated aggressively in patients with SIGAD. (C) [...] SS 102. Rare patients with SIGAD might benefit from IVIG replacement therapy. (C)

• #27 Selective immunoglobulin A deficiency – wikidoc

  https://www.wikidoc.org/index.php/Selective_immunoglobulin_A_deficiency

  Selective IgA deficiency is inherited with a variable inheritance pattern. There are no established measures for the primary prevention. […] Effective measures for secondary and tertiary prevention of selective IgA deficiency include: Patient education, Vaccination, Use of prophylactic antibiotics. […] Patient with severe IgA deficiency may have anaphylactic reaction secondary to blood transfusion or its products. It is specifically seen in patients with undetectable serum IgA levels. These patients develop anti IgA antibodies so they should be advised to wear medical alert bracelet. […] Pneumococcal vaccine is recommended in patients with SIgAD to reduce the risk of sinopulmonary infections. […] Prophylactic antibiotics can be given to patients with SIgAD to prevent infections and other complications.

• #28 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #29 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #30 Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463416/

  Selective IgA deficiency is the most common type of primary immunodeficiency, but there is not yet a specific effective treatment. […] Currently, there is no specific treatment for selective IgA deficiency. The therapeutic approach is individualized according to the clinical status of the patient. The use of antibiotic prophylaxis in patients with a history of recurrent respiratory infections may be established in specific cases of higher severity or characterized by high frequency of infectious episodes. […] In the present study, we report the use of sublingual immunotherapy (SLIT) combined activated whole-cell bacterial extract and Derp1-specific immunotherapy in a patient with partial IgA deficiency and severe persistent rhinitis and moderate persistent asthma. […] Our results suggest that the long-term therapeutic regimen with a high cumulative total load of total body extract of inactivated bacteria, similar to that proposed for specific allergen immunotherapy, may be an effective strategy to promote prophylaxis against respiratory tract infections in selective IgA deficiency.

• #31 Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463416/

  The immunotherapy practice guidelines state that Immunotherapy can be considered in patients with immunodeficiency and autoimmune disorders. […] In the present case report of a patient with partial IgA deficiency, immunotherapy with inactivated whole-cell bacterial extract concurrently with Der p1, clinically monitored for an 18-month period, promoted a significant decrease in the number of episodes of respiratory infections as well as allergic rhinitis and asthma control.

• #32 Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463416/

  Selective IgA deficiency is the most common type of primary immunodeficiency, but there is not yet a specific effective treatment. […] Currently, there is no specific treatment for selective IgA deficiency. The therapeutic approach is individualized according to the clinical status of the patient. The use of antibiotic prophylaxis in patients with a history of recurrent respiratory infections may be established in specific cases of higher severity or characterized by high frequency of infectious episodes. […] In the present study, we report the use of sublingual immunotherapy (SLIT) combined activated whole-cell bacterial extract and Derp1-specific immunotherapy in a patient with partial IgA deficiency and severe persistent rhinitis and moderate persistent asthma. […] Our results suggest that the long-term therapeutic regimen with a high cumulative total load of total body extract of inactivated bacteria, similar to that proposed for specific allergen immunotherapy, may be an effective strategy to promote prophylaxis against respiratory tract infections in selective IgA deficiency.

• #33 Prophylaxis of Upper Airway Infections in a Patient with Partial IgA Deficiency: Concurrent Use of Sublingual Immunotherapy with Inactivated Whole-Cell Bacterial Extract and Der p1

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463416/

  The immunotherapy practice guidelines state that Immunotherapy can be considered in patients with immunodeficiency and autoimmune disorders. […] In the present case report of a patient with partial IgA deficiency, immunotherapy with inactivated whole-cell bacterial extract concurrently with Der p1, clinically monitored for an 18-month period, promoted a significant decrease in the number of episodes of respiratory infections as well as allergic rhinitis and asthma control.

• #34 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  It has been estimated that allergies can be the first manifestation of SigAD in up to 40% of cases. Therefore, the suspicion of SIgAD should heighten not only in patients with recurrent infections but also in those with atopic manifestations. […] Hypotheses on the pathogenesis of allergy in SIgAD include high penetration of allergens through the mucosa due to defective serum and mucosal IgA, facilitating the contact of allergens with the local respiratory and gastrointestinal mucosal immune system. […] The association between SIgAD and allergy was found for various allergic manifestations, the most commonly described of which are asthma, allergic rhinitis, allergic conjunctivitis, urticaria, atopic dermatitis and food allergy, even if the exact prevalence of each symptom and of one clinical manifestation or another varies among the studies reported.

• #35 AAAAI & ACAAI GUIDELINES Bundle (free trial) – Primary Immunodeficiency

  https://eguideline.guidelinecentral.com/i/802140-primary-immunodeficiency/27

  Selective IgA deficiency (SIGAD) SS 96. Subjects older than 4 years with a serum IgA level <7 mg/dL and normal serum IgG and IgM levels and in whom other causes of hypogammaglobulinemia have been excluded should be given a diagnosis of SIGAD. (C) [...] SS 98. Patients with SIGAD should be monitored over time for the occurrence of complications. (C) [...] SS 100. Aggressive antimicrobial therapy, prophylaxis, or both should be used in patients with SIGAD and recurrent sinopulmonary infections. (C) [...] SS 101. Atopic disease should be treated aggressively in patients with SIGAD. (C) [...] SS 102. Rare patients with SIGAD might benefit from IVIG replacement therapy. (C)

• #36 Selective IgA Deficiency and Allergy: A Fresh Look to an Old Story

  https://www.mdpi.com/1648-9144/58/1/129

  It is worth mentioning that in many cases allergy is the first symptom of SIgAD, and, in some cases, it is the only symptom of disease. […] In light of the association between allergy/asthma and recurrent infections in SIgAD, early detection and management of respiratory disorders is essential to prevent severe complications.

• #37 Transfusion Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28851

  Individuals with selective IgA deficiency may develop anti-IgA antibodies causing allergic reactions when transfused with blood products containing IgA. […] Patients with selective IgA deficiency are at a significantly higher risk of transfusion reactions compared to the general population, making careful blood product selection crucial to prevent complications. […] Experts recommend screening patients with severe selective IgA deficiency and those who have experienced a reaction to blood products in the past for anti-IgA antibodies. […] Methods to prevent anaphylactic reactions in patients with anti-IgA antibodies include exclusively using products from IgA-deficient donors and transfusing washed platelets and red blood cells. […] Patients with severe selective IgA deficiency should wear medical alert identification that notifies healthcare professionals of their condition, the potential risk of anaphylactic reaction to plasma-containing blood products, and the need for anti-IgA testing before receiving transfusions.

• #38 Transfusion Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28851

  Individuals with selective IgA deficiency may develop anti-IgA antibodies causing allergic reactions when transfused with blood products containing IgA. […] Patients with selective IgA deficiency are at a significantly higher risk of transfusion reactions compared to the general population, making careful blood product selection crucial to prevent complications. […] Experts recommend screening patients with severe selective IgA deficiency and those who have experienced a reaction to blood products in the past for anti-IgA antibodies. […] Methods to prevent anaphylactic reactions in patients with anti-IgA antibodies include exclusively using products from IgA-deficient donors and transfusing washed platelets and red blood cells. […] Patients with severe selective IgA deficiency should wear medical alert identification that notifies healthcare professionals of their condition, the potential risk of anaphylactic reaction to plasma-containing blood products, and the need for anti-IgA testing before receiving transfusions.

• #39 Transfusion Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28851

  Individuals with selective IgA deficiency may develop anti-IgA antibodies causing allergic reactions when transfused with blood products containing IgA. […] Patients with selective IgA deficiency are at a significantly higher risk of transfusion reactions compared to the general population, making careful blood product selection crucial to prevent complications. […] Experts recommend screening patients with severe selective IgA deficiency and those who have experienced a reaction to blood products in the past for anti-IgA antibodies. […] Methods to prevent anaphylactic reactions in patients with anti-IgA antibodies include exclusively using products from IgA-deficient donors and transfusing washed platelets and red blood cells. […] Patients with severe selective IgA deficiency should wear medical alert identification that notifies healthcare professionals of their condition, the potential risk of anaphylactic reaction to plasma-containing blood products, and the need for anti-IgA testing before receiving transfusions.

• #40 Anaphylactic transfusion reactions and IgA deficiency | Professional Education

  https://profedu.blood.ca/en/transfusion/publications/anaphylactic-transfusion-reactions-and-iga-deficiency

  Individuals with a history of an anaphylactic transfusion reaction or two severe allergic reactions may benefit from further IgA level and anti-IgA testing. […] Given the high frequency of anti-IgA antibodies and the low frequency of anaphylactic transfusion reactions attributable to anti-IgA, it is estimated that approximately 1 in 100 patients with IgA deficiency and anti-IgA antibodies develop transfusion reactions. […] For patients with a history of anaphylactic transfusion reactions, ideally, an investigation would be done to determine if anti-IgA antibodies are present. […] If the patient is not IgA deficient and/or no anti-IgA has been detected, and the patient has experienced only a single anaphylactic reaction, a trial transfusion of unwashed blood components may be performed under controlled conditions, including patient consent, premedication, and close medical supervision. […] If haptoglobin deficiency is determined to be the etiology of the anaphylactic (or 2 severe) allergic transfusion reaction, specialized products may be required for transfusion support.

• #41

  https://step1.medbullets.com/immunology/105013/selective-iga-deficiency

  Prognosis, Prevention, and Complications […] Prevention […] avoid blood transfusions unless from IgA-deficient individual

• #42

  https://step2.medbullets.com/pediatrics/120735/iga-deficiency

  Prevention: avoid blood transfusions unless treated with saline wash or from IgA deficient individual […] Blood transfusions: wash RBCs with saline or obtain blood from IgA-deficient donor.

• #43 Transfusion Selective IgA Deficiency | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/28851

  Individuals with selective IgA deficiency may develop anti-IgA antibodies causing allergic reactions when transfused with blood products containing IgA. […] Patients with selective IgA deficiency are at a significantly higher risk of transfusion reactions compared to the general population, making careful blood product selection crucial to prevent complications. […] Experts recommend screening patients with severe selective IgA deficiency and those who have experienced a reaction to blood products in the past for anti-IgA antibodies. […] Methods to prevent anaphylactic reactions in patients with anti-IgA antibodies include exclusively using products from IgA-deficient donors and transfusing washed platelets and red blood cells. […] Patients with severe selective IgA deficiency should wear medical alert identification that notifies healthcare professionals of their condition, the potential risk of anaphylactic reaction to plasma-containing blood products, and the need for anti-IgA testing before receiving transfusions.

• #44 Selective IgA deficiency – Humanitas.net

  https://www.humanitas.net/diseases/selective-iga-deficiency/

  While there is no known prevention for IgA deficiency, genetic counseling has been proven helpful for prospective parents with a family history of selective IgA deficiency. […] No specific treatment is available for Selective IgA deficiency. Most individuals who have bacterial infections of the ears, sinuses, lungs or gastrointestinal tract are given antibiotics to treat the infections and prevent their reoccurrence. Selective IgA deficiency that results from taking a drug usually resolves if the drug is stopped. […] Individuals with selective IgA deficiency are advised to wear a medical identification bracelet to prevent inadvertent plasma or IVIG administration, which could lead to anaphylaxis. Anaphylaxis is a severe, potentially life-threatening allergic reaction.

• #45

  https://scholars.duke.edu/publication/642461

  Sera from three hundred five patients with immunoglobulin deficiencies were analyzed for the presence of anti-IgA antibodies by using indirect agglutination and enzyme-linked immunosorbent assay (ELISA). Anti-IgA antibodies were observed in 15 of 68 (22%) patients with hypogammaglobulinemia and 53 of 185 (29%) patients with selective IgA deficiency, both groups having serum IgA less than 0.05 g/liter. […] Of eight patients with anti-IgA antibodies, seven tolerated Ig prophylaxis with a commercial immunoglobulin preparation low in IgA when given either intramuscularly or intravenously. The titers of anti-IgA in the sera of these patients did not rise in relation to the prophylaxis. Only one of the eight patients had a history of previous anaphylactic reactions to IgA-containing blood products. He tolerated six Ig infusions during 5 months with the IgA-depleted preparation without any adverse effects but showed increasing levels of anti-IgA antibodies and ultimately experienced a near-fatal reaction at the seventh infusion.

• #46 Immunoglobulin A Deficiency

  https://phoenixchildrens.staywellsolutionsonline.com/134,124

  IgA deficiency is a problem that may be passed down through your family, so you cant do anything to prevent it. […] But you can limit the spread of germs and sickness by washing your hands often and staying away from large crowds. This is especially important to do during cold and flu season. Also talk with your healthcare provider about vaccines that may help prevent illness and when you should get them.

• #47 Immunoglobulin A Deficiency | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/i/immunoglobulin-a-deficiency.html

  IgA deficiency is a problem that may be passed down through your family, so you cant do anything to prevent it. But you can limit the spread of germs and sickness by washing your hands often and staying away from large crowds. This is especially important to do during cold and flu season. […] Also talk with your healthcare provider about vaccines that may help prevent illness and when you should get them.

• #48 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #49 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #50 Selective IgA Deficiency in Children: Clinical Manifestations, Evaluation, and Management

  https://www.contemporarypediatrics.com/view/selective-iga-deficiency-children-clinical-manifestations-evaluation-and-management

  Selective IgA deficiency (SIGAD) is the most common immunodeficiency disorder; it affects about 1 in 200 to 900 persons. Most affected children are asymptomatic. […] Patients with recurrent infections may benefit from prophylactic antibiotic therapy given for 6 months-or given only during the winter months in those with seasonal patterns of infections. […] Patients with SIGAD who have significant recurrent upper respiratory tract infections may benefit from a 6-month course of prophylactic antibiotics. These antibiotics may be continued if the initial course is successful. […] Some children with SIGAD who have seasonal patterns of infections may benefit from receiving prophylactic antibiotics only during the winter months. […] A small number of patients with SIGAD continue to have recurrent infections despite antibiotic prophylaxis. These patients may be treated with immunoglobulin replacement therapy; however, use of this treatment remains controversial. […] All children with SIGAD should be immunized according to the same schedule used for children without SIGAD and should receive the annual influenza vaccine.

• #51 SciELO Brazil – Treatment of patients with immunodeficiency: Medication, gene therapy, and transplantation Treatment of patients with immunodeficiency: Medication, gene therapy, and transplantation

  https://www.scielo.br/j/jped/a/nBQBqFTBZmGFdgKFr5C6SRd/

  In addition to the above mentioned PID pictures, isolated antibiotic prophylaxis is often offered to patients with mild hypogammaglobulinemia, selective immunoglobulin A (IgA) deficiency or deficiency of IgG subclasses, who are not receiving immunoglobulin, despite the lack of evidence to support the use of antibiotics in this population. […] There is no indication for immunoglobulin replacement in patients with selective IgA deficiency, unless there is an association with deficiency of IgG subclasses or a qualitative defect in antibody production.

• #52 Selective deficiency of IgA: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001476.htm

  Selective IgA deficiency is the most common immune deficiency disorder. People with this disorder have a low or absent level of a blood protein called immunoglobulin A. […] Selective IgA deficiency is usually inherited, which means it is passed down through families. However, there are also cases of drug-induced selective IgA deficiency. […] No specific treatment is available. Some people gradually develop normal levels of IgA without treatment. Treatment involves taking steps to reduce the number and severity of infections. Antibiotics are often needed to treat bacterial infections. […] Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.

• #53 Selective IgA deficiency – Humanitas.net

  https://www.humanitas.net/diseases/selective-iga-deficiency/

  While there is no known prevention for IgA deficiency, genetic counseling has been proven helpful for prospective parents with a family history of selective IgA deficiency. […] No specific treatment is available for Selective IgA deficiency. Most individuals who have bacterial infections of the ears, sinuses, lungs or gastrointestinal tract are given antibiotics to treat the infections and prevent their reoccurrence. Selective IgA deficiency that results from taking a drug usually resolves if the drug is stopped. […] Individuals with selective IgA deficiency are advised to wear a medical identification bracelet to prevent inadvertent plasma or IVIG administration, which could lead to anaphylaxis. Anaphylaxis is a severe, potentially life-threatening allergic reaction.

• #54 Low IgA and Selective IgA Deficiency

  https://www.geneticlifehacks.com/low-iga-and-selective-deficiency/

  IgA deficiency is often diagnosed when testing for autoimmune diseases. […] IgA deficiency may also be autoimmune or caused by certain medications. […] Research on the gut microbiome and IgA levels also shows that there is an interaction. Probiotics can also increase IgA levels. […] You may be wondering if your frequent respiratory infections mean that you have a partial IgA deficiency? The only way to know is to have an IgA blood test done.

• #55 Immunoglobulin A Deficiency Treatment & Management: Medical Care, Surgical Care, Consultations

  https://emedicine.medscape.com/article/136580-treatment

  Primary prevention for immunoglobulin A deficiency (IgAD) has not been developed. If a medication is under consideration as the cause of the IgAD, it should be discontinued. […] Secondary prevention relies primarily on vaccination to increase specific IgG and IgM. In situations in which purified and decontaminated water cannot be guaranteed, precautions such as boiling drinking water may help prevent GI infections like giardiasis or cryptosporidiosis. The role of prophylactic antibiotics is controversial because they may increase the hazard of infection with resistant bacteria or fungi. […] Tertiary prevention includes (1) prompt antibiotic treatment for respiratory tract infections, (2) microbial identification of diarrheal pathogens and specific treatment, (3) dietary modification for malabsorption syndromes, and (4) use of washed cells and/or IgA-poor blood if whole blood is needed, and screening for anti-IgA antibodies if reactions to blood products occur (not routinely performed). It has been shown in a systematic review that inhaled antibiotics resulted in a slight reduction in exacerbations, a probable reduction in severe exacerbations, and a likely slight improvement in symptoms and quality of life in adults with bronchiectasis, a common complication of IgAD.

• #56 Relationship between Selective IgA Deficiency and COVID-19 Prognosis

  https://www.jstage.jst.go.jp/article/yoken/75/3/75_JJID.2021.281/_article

  The prevalence and mortality rates of coronavirus disease 2019 (COVID-19) widely vary among populations. Mucosal immunity is the first barrier to the pathogens entry into the body. Immunoglobulin A (IgA) is the primary antibody responsible for mucosal immunity. We explored the relationship between selective IgA deficiency (SIgAD) and COVID-19 severity. […] In patients with SIgAD, the number of severe acute respiratory coronaviruses 2 that pass through mucosal membranes may be increased, leading to complications such as cytokine storm syndrome and acute respiratory distress syndrome.

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