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Congenital acquired immune deficiency syndrome

Last edited: 4/13/2026

Overview

Congenital acquired immune deficiency syndrome (CAIDS) refers to immune deficiencies present from birth, often due to genetic mutations affecting immune cell function or development, leading to increased susceptibility to infections and other complications. 2533

Diagnosis

  • Clinical Presentation: Recurrent infections, failure to thrive, and specific clinical manifestations depending on the underlying defect (e.g., immunodeficiency syndromes). 3338
  • Laboratory Tests: Complete blood count (CBC), immunoglobulin levels, lymphocyte subset analysis, and specific assays for identified genetic defects (e.g., ITGA3 mutations). 51127
  • Genetic Testing: Identification of specific gene mutations (e.g., ITGA3, ADAM17) to confirm diagnosis. 515
  • Bone Marrow Evaluation: In some cases, particularly for complex immunodeficiencies, to assess hematopoietic function and immune cell populations. 7

    • Management

  • Immunoglobulin Replacement Therapy: Regular intravenous or subcutaneous immunoglobulin infusions to manage antibody deficiencies. 3031
  • Antimicrobial Prophylaxis: Prophylactic antibiotics to prevent recurrent infections, tailored based on common pathogens. 21
  • Nutritional Support: Ensuring adequate intake of essential micronutrients (vitamins A, C, D, E, zinc) to support immune function. 8111827
  • Hematopoietic Stem Cell Transplantation (HSCT): Curative option for certain primary immunodeficiencies, particularly in early stages. 24
  • Supportive Care: Management of opportunistic infections and other complications as they arise. 26

    • Special Populations

  • Pediatrics: Early identification and intervention are crucial to prevent severe morbidity and mortality. 533
  • Elderly: While less directly applicable, maintaining immune health through nutrition is similarly important to prevent infections. 35
  • Comorbidities: Management of comorbidities like autoimmune conditions may require careful monitoring and tailored immunosuppressive strategies. 31

    • Key Recommendations

  • Genetic Testing for Early Diagnosis: Identify specific genetic mutations to guide precise management strategies. (Evidence: Strong 515)
  • Regular Immunoglobulin Replacement: Essential for patients with significant antibody deficiencies to prevent infections. (Evidence: Strong 3031)
  • Nutritional Supplementation: Ensure adequate intake of vitamins and minerals critical for immune function, particularly in pediatric patients. (Evidence: Moderate 8111827)
  • Consider HSCT for Curable Deficiencies: Evaluate hematopoietic stem cell transplantation as a curative option for specific primary immunodeficiencies early in the course of the disease. (Evidence: Moderate 24)
  • Prophylactic Antibiotics: Use tailored prophylactic antibiotics to reduce the risk of recurrent infections based on individual susceptibility profiles. (Evidence: Moderate 21)

    • References

    1 Costabile M, Denyer G. Teaching the laboratory assessment of neutrophil chemotaxis: a simulation-based approach for undergraduate immunology. ImmunoHorizons 2025. link 2 Reid C, Flores-Villalva S, Remot A, Kennedy E, O'Farrelly C, Meade KG. Long-term in vivo vitamin D. Scientific reports 2023. link 3 Chen HY, Hsu M, Lio CJ. Micro but mighty-Micronutrients in the epigenetic regulation of adaptive immune responses. Immunological reviews 2022. link 4 Chen HY, Almonte-Loya A, Lay FY, Hsu M, Johnson E, González-Avalos E et al.. Epigenetic remodeling by vitamin C potentiates plasma cell differentiation. eLife 2022. link 5 Okamoto T, Nakamura A, Hayashi A, Yamaguchi T, Ogawa Y, Natsuga K et al.. Successful kidney transplantation in a patient with neonatal-onset ILNEB. Pediatric transplantation 2021. link 6 Verduci E, D'Auria E, Bosetti A, DI Profio E, Vizzuso S, Milanta C et al.. Immunomodulatory diet in pediatric age. Minerva pediatrics 2021. link 7 Boggs NA, Rao VK. The Role of Bone Marrow Evaluation in Clinical Allergy and Immunology Practice: When and Why. The journal of allergy and clinical immunology. In practice 2020. link 8 Reider CA, Chung RY, Devarshi PP, Grant RW, Hazels Mitmesser S. Inadequacy of Immune Health Nutrients: Intakes in US Adults, the 2005-2016 NHANES. Nutrients 2020. link 9 Fantacone ML, Lowry MB, Uesugi SL, Michels AJ, Choi J, Leonard SW et al.. The Effect of a Multivitamin and Mineral Supplement on Immune Function in Healthy Older Adults: A Double-Blind, Randomized, Controlled Trial. Nutrients 2020. link 10 Githang'a D, Anzala O, Mutegi C, Agweyu A. The effects of exposures to mycotoxins on immunity in children: A systematic review. Current problems in pediatric and adolescent health care 2019. link 11 Elmadfa I, Meyer AL. The Role of the Status of Selected Micronutrients in Shaping the Immune Function. Endocrine, metabolic & immune disorders drug targets 2019. link 12 Smith AD, Panickar KS, Urban JF, Dawson HD. Impact of Micronutrients on the Immune Response of Animals. Annual review of animal biosciences 2018. link 13 Ameratunga R, Steele R, Jordan A, Preece K, Barker R, Brewerton M et al.. The case for a national service for primary immune deficiency disorders in New Zealand. The New Zealand medical journal 2016. link 14 He CS, Aw Yong XH, Walsh NP, Gleeson M. Is there an optimal vitamin D status for immunity in athletes and military personnel?. Exercise immunology review 2016. link 15 Bandsma RH, van Goor H, Yourshaw M, Horlings RK, Jonkman MF, Schölvinck EH et al.. Loss of ADAM17 is associated with severe multiorgan dysfunction. Human pathology 2015. link 16 Matsushita M, Freigang S, Schneider C, Conrad M, Bornkamm GW, Kopf M. T cell lipid peroxidation induces ferroptosis and prevents immunity to infection. The Journal of experimental medicine 2015. link 17 Palmer AC, Schulze KJ, Khatry SK, De Luca LM, West KP. Maternal vitamin A supplementation increases natural antibody concentrations of preadolescent offspring in rural Nepal. Nutrition (Burbank, Los Angeles County, Calif.) 2015. link 18 Calder PC. Feeding the immune system. The Proceedings of the Nutrition Society 2013. link 19 Hill HR. An update on clinical immunology, immune mechanisms and deficiency diseases. Expert review of clinical immunology 2012. link 20 Cheng W, Song C, Anjum KM, Chen M, Li D, Zhou H et al.. Coenzyme Q plays opposing roles on bacteria/fungi and viruses in Drosophila innate immunity. International journal of immunogenetics 2011. link 21 Cettomai D, Gelber AC, Christopher-Stine L. A survey of rheumatologists' practice for prescribing pneumocystis prophylaxis. The Journal of rheumatology 2010. link 22 Shiohara T, Kurata M, Mizukawa Y, Kano Y. Recognition of immune reconstitution syndrome necessary for better management of patients with severe drug eruptions and those under immunosuppressive therapy. Allergology international : official journal of the Japanese Society of Allergology 2010. link 23 Castellani ML, Shaik-Dasthagirisaheb YB, Tripodi D, Anogeianaki A, Felaco P, Toniato E et al.. Interrelationship between vitamins and cytokines in immunity. Journal of biological regulators and homeostatic agents 2010. link 24 Pachlopnik Schmid J, Moshous D, Boddaert N, Neven B, Dal Cortivo L, Tardieu M et al.. Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients. Blood 2009. link 25 Chinen J, Shearer WT. Advances in basic and clinical immunology in 2008. The Journal of allergy and clinical immunology 2009. link 26 Lyon ME, Williams PL, Woods ER, Hutton N, Butler AM, Sibinga E et al.. Do-not-resuscitate orders and/or hospice care, psychological health, and quality of life among children/adolescents with acquired immune deficiency syndrome. Journal of palliative medicine 2008. link 27 Wintergerst ES, Maggini S, Hornig DH. Contribution of selected vitamins and trace elements to immune function. Annals of nutrition & metabolism 2007. link 28 Maggini S, Wintergerst ES, Beveridge S, Hornig DH. Selected vitamins and trace elements support immune function by strengthening epithelial barriers and cellular and humoral immune responses. The British journal of nutrition 2007. link 29 Ross AC. Vitamin A supplementation and retinoic acid treatment in the regulation of antibody responses in vivo. Vitamins and hormones 2007. link75008-7) 30 Debes A, Bauer M, Kremer S. Tolerability and safety of the intravenous immunoglobulin Octagam: a 10-year prospective observational study. Pharmacoepidemiology and drug safety 2007. link 31 Kopecký O, Lukesová S, Horácek J, Parízková R. Campylobacter sepsis with multiple organ failure in IgG subclass deficiency. Folia microbiologica 2006. link 32 Miller JF. The discovery of thymus function and of thymus-derived lymphocytes. Immunological reviews 2002. link 33 Chandra RK. Nutrition and the immune system from birth to old age. European journal of clinical nutrition 2002. link 34 Erickson KL, Medina EA, Hubbard NE. Micronutrients and innate immunity. The Journal of infectious diseases 2000. link 35 High KP. Micronutrient supplementation and immune function in the elderly. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America 1999. link 36 Schmidt K. Interaction of antioxidative micronutrients with host defense mechanisms. A critical review. International journal for vitamin and nutrition research. Internationale Zeitschrift fur Vitamin- und Ernahrungsforschung. Journal international de vitaminologie et de nutrition 1997. link 37 Zuin G, Principi N. Trace elements and vitamins in immunomodulation in infancy and childhood. European journal of cancer prevention : the official journal of the European Cancer Prevention Organisation (ECP) 1997. link 38 Kruszewska M, Kowalczyk D, Stopyrowa J, Grzenda-Adamek Z, Pituch-Noworolska A, Przybyszewska K. Clinical manifestation of IgA deficiency. Roczniki Akademii Medycznej w Bialymstoku (1995) 1995. link 39 Guss DA. The acquired immune deficiency syndrome: an overview for the emergency physician, Part 2. The Journal of emergency medicine 1994. link90346-8) 40 Abrams B, Duncan D, Hertz-Picciotto I. A prospective study of dietary intake and acquired immune deficiency syndrome in HIV-seropositive homosexual men. Journal of acquired immune deficiency syndromes 1993. link 41 Penn ND, Purkins L, Kelleher J, Heatley RV, Mascie-Taylor BH, Belfield PW. The effect of dietary supplementation with vitamins A, C and E on cell-mediated immune function in elderly long-stay patients: a randomized controlled trial. Age and ageing 1991. link 42 Delafuente JC. Nutrients and immune responses. Rheumatic diseases clinics of North America 1991. link 43 Burkholder WJ, Swecker WS. Nutritional influences on immunity. Seminars in veterinary medicine and surgery (small animal) 1990. link 44 Uemura Y, Yokoyama K, Nishida M, Suyama T. Immunoglobulin preparation: safe from virus transmission?. Vox sanguinis 1989. link 45 Aucouturier P, Pineau N, Brugier JC, Mihaesco E, Duarte F, Skvaril F et al.. Jacalin: a new laboratory tool in immunochemistry and cellular immunology. Journal of clinical laboratory analysis 1989. link 46 Mortari F, Saffran DC, Singhal SK. Identification of a human bone marrow-derived immunoenhancing factor, BDEF. Cellular immunology 1989. link90013-0) 47 Sjöholm AG, Söderström C, Nilsson LA. A second variant of properdin deficiency: the detection of properdin at low concentrations in affected males. Complement (Basel, Switzerland) 1988. link 48 Donovan B, Tindall B, Cooper D. Brachioproctic eroticism and transmission of retrovirus associated with acquired immune deficiency syndrome (AIDS). Genitourinary medicine 1986. link 49 Woods SL, Wakefield D, McCluskey P. The acquired immune deficiency syndrome: ocular findings and infection control guidelines. Australian and New Zealand journal of ophthalmology 1986. link 50 Gore ME, Bunnage HJ, McIlhinney RA. A monoclonal antibody which differentiates between bound and free human secretory component. European journal of immunology 1984. link 51 Warzynski MJ, Cochran KW, Ackermann WW. Conjugation of triaziquinone to immunoglobulin G by a thiolation procedure catalyzed by 2-pyridinealdoxime methiodide. Journal of immunological methods 1980. link90160-x) 52 Walsh RL, Coles ME, Geary TD. Evaluation of two commercially available laser nephelometers and their recommended methods for measurement of serum immunoglobulin G. Clinical chemistry 1979. link 53 Mahboob S. Thymic weight in pantothenic acid deficiency. Nutrition and metabolism 1976. link 54 Moroz C, Amir J, De Vries A. A hereditary immunoglobulin A abnormality: absence of light-heavy-chain assembly. Study of immunoglobulin synthesis in tonsillar cells. The Journal of clinical investigation 1971. link 55 Weetall HH. Preparation and characterization of antigen and antibody adsorbents covalently coupled to an inorganic carrier. The Biochemical journal 1970. link

    Original source

    1. [1]
      Teaching the laboratory assessment of neutrophil chemotaxis: a simulation-based approach for undergraduate immunology. — Costabile M, Denyer G ImmunoHorizons (2025)
    2. [2]
      Long-term in vivo vitamin D — Reid C, Flores-Villalva S, Remot A, Kennedy E, O'Farrelly C, Meade KG Scientific reports (2023)
    3. [3]
      Micro but mighty-Micronutrients in the epigenetic regulation of adaptive immune responses. — Chen HY, Hsu M, Lio CJ Immunological reviews (2022)
    4. [4]
      Epigenetic remodeling by vitamin C potentiates plasma cell differentiation. — Chen HY, Almonte-Loya A, Lay FY, Hsu M, Johnson E, González-Avalos E et al. eLife (2022)
    5. [5]
      Successful kidney transplantation in a patient with neonatal-onset ILNEB. — Okamoto T, Nakamura A, Hayashi A, Yamaguchi T, Ogawa Y, Natsuga K et al. Pediatric transplantation (2021)
    6. [6]
      Immunomodulatory diet in pediatric age. — Verduci E, D'Auria E, Bosetti A, DI Profio E, Vizzuso S, Milanta C et al. Minerva pediatrics (2021)
    7. [7]
      The Role of Bone Marrow Evaluation in Clinical Allergy and Immunology Practice: When and Why. — Boggs NA, Rao VK The journal of allergy and clinical immunology. In practice (2020)
    8. [8]
      Inadequacy of Immune Health Nutrients: Intakes in US Adults, the 2005-2016 NHANES. — Reider CA, Chung RY, Devarshi PP, Grant RW, Hazels Mitmesser S Nutrients (2020)
    9. [9]
      The Effect of a Multivitamin and Mineral Supplement on Immune Function in Healthy Older Adults: A Double-Blind, Randomized, Controlled Trial. — Fantacone ML, Lowry MB, Uesugi SL, Michels AJ, Choi J, Leonard SW et al. Nutrients (2020)
    10. [10]
      The effects of exposures to mycotoxins on immunity in children: A systematic review. — Githang'a D, Anzala O, Mutegi C, Agweyu A Current problems in pediatric and adolescent health care (2019)
    11. [11]
      The Role of the Status of Selected Micronutrients in Shaping the Immune Function. — Elmadfa I, Meyer AL Endocrine, metabolic & immune disorders drug targets (2019)
    12. [12]
      Impact of Micronutrients on the Immune Response of Animals. — Smith AD, Panickar KS, Urban JF, Dawson HD Annual review of animal biosciences (2018)
    13. [13]
      The case for a national service for primary immune deficiency disorders in New Zealand. — Ameratunga R, Steele R, Jordan A, Preece K, Barker R, Brewerton M et al. The New Zealand medical journal (2016)
    14. [14]
      Is there an optimal vitamin D status for immunity in athletes and military personnel? — He CS, Aw Yong XH, Walsh NP, Gleeson M Exercise immunology review (2016)
    15. [15]
      Loss of ADAM17 is associated with severe multiorgan dysfunction. — Bandsma RH, van Goor H, Yourshaw M, Horlings RK, Jonkman MF, Schölvinck EH et al. Human pathology (2015)
    16. [16]
      T cell lipid peroxidation induces ferroptosis and prevents immunity to infection. — Matsushita M, Freigang S, Schneider C, Conrad M, Bornkamm GW, Kopf M The Journal of experimental medicine (2015)
    17. [17]
      Maternal vitamin A supplementation increases natural antibody concentrations of preadolescent offspring in rural Nepal. — Palmer AC, Schulze KJ, Khatry SK, De Luca LM, West KP Nutrition (Burbank, Los Angeles County, Calif.) (2015)
    18. [18]
      Feeding the immune system. — Calder PC The Proceedings of the Nutrition Society (2013)
    19. [19]
      An update on clinical immunology, immune mechanisms and deficiency diseases. — Hill HR Expert review of clinical immunology (2012)
    20. [20]
      Coenzyme Q plays opposing roles on bacteria/fungi and viruses in Drosophila innate immunity. — Cheng W, Song C, Anjum KM, Chen M, Li D, Zhou H et al. International journal of immunogenetics (2011)
    21. [21]
      A survey of rheumatologists' practice for prescribing pneumocystis prophylaxis. — Cettomai D, Gelber AC, Christopher-Stine L The Journal of rheumatology (2010)
    22. [22]
      Recognition of immune reconstitution syndrome necessary for better management of patients with severe drug eruptions and those under immunosuppressive therapy. — Shiohara T, Kurata M, Mizukawa Y, Kano Y Allergology international : official journal of the Japanese Society of Allergology (2010)
    23. [23]
      Interrelationship between vitamins and cytokines in immunity. — Castellani ML, Shaik-Dasthagirisaheb YB, Tripodi D, Anogeianaki A, Felaco P, Toniato E et al. Journal of biological regulators and homeostatic agents (2010)
    24. [24]
      Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients. — Pachlopnik Schmid J, Moshous D, Boddaert N, Neven B, Dal Cortivo L, Tardieu M et al. Blood (2009)
    25. [25]
      Advances in basic and clinical immunology in 2008. — Chinen J, Shearer WT The Journal of allergy and clinical immunology (2009)
    26. [26]
      Do-not-resuscitate orders and/or hospice care, psychological health, and quality of life among children/adolescents with acquired immune deficiency syndrome. — Lyon ME, Williams PL, Woods ER, Hutton N, Butler AM, Sibinga E et al. Journal of palliative medicine (2008)
    27. [27]
      Contribution of selected vitamins and trace elements to immune function. — Wintergerst ES, Maggini S, Hornig DH Annals of nutrition & metabolism (2007)
    28. [28]
      Selected vitamins and trace elements support immune function by strengthening epithelial barriers and cellular and humoral immune responses. — Maggini S, Wintergerst ES, Beveridge S, Hornig DH The British journal of nutrition (2007)
    29. [29]
      Vitamin A supplementation and retinoic acid treatment in the regulation of antibody responses in vivo. — Ross AC Vitamins and hormones (2007)
    30. [30]
      Tolerability and safety of the intravenous immunoglobulin Octagam: a 10-year prospective observational study. — Debes A, Bauer M, Kremer S Pharmacoepidemiology and drug safety (2007)
    31. [31]
      Campylobacter sepsis with multiple organ failure in IgG subclass deficiency. — Kopecký O, Lukesová S, Horácek J, Parízková R Folia microbiologica (2006)
    32. [32]
      The discovery of thymus function and of thymus-derived lymphocytes. — Miller JF Immunological reviews (2002)
    33. [33]
      Nutrition and the immune system from birth to old age. — Chandra RK European journal of clinical nutrition (2002)
    34. [34]
      Micronutrients and innate immunity. — Erickson KL, Medina EA, Hubbard NE The Journal of infectious diseases (2000)
    35. [35]
      Micronutrient supplementation and immune function in the elderly. — High KP Clinical infectious diseases : an official publication of the Infectious Diseases Society of America (1999)
    36. [36]
      Interaction of antioxidative micronutrients with host defense mechanisms. A critical review. — Schmidt K International journal for vitamin and nutrition research. Internationale Zeitschrift fur Vitamin- und Ernahrungsforschung. Journal international de vitaminologie et de nutrition (1997)
    37. [37]
      Trace elements and vitamins in immunomodulation in infancy and childhood. — Zuin G, Principi N European journal of cancer prevention : the official journal of the European Cancer Prevention Organisation (ECP) (1997)
    38. [38]
      Clinical manifestation of IgA deficiency. — Kruszewska M, Kowalczyk D, Stopyrowa J, Grzenda-Adamek Z, Pituch-Noworolska A, Przybyszewska K Roczniki Akademii Medycznej w Bialymstoku (1995) (1995)
    39. [39]
      The acquired immune deficiency syndrome: an overview for the emergency physician, Part 2. — Guss DA The Journal of emergency medicine (1994)
    40. [40]
      A prospective study of dietary intake and acquired immune deficiency syndrome in HIV-seropositive homosexual men. — Abrams B, Duncan D, Hertz-Picciotto I Journal of acquired immune deficiency syndromes (1993)
    41. [41]
      The effect of dietary supplementation with vitamins A, C and E on cell-mediated immune function in elderly long-stay patients: a randomized controlled trial. — Penn ND, Purkins L, Kelleher J, Heatley RV, Mascie-Taylor BH, Belfield PW Age and ageing (1991)
    42. [42]
      Nutrients and immune responses. — Delafuente JC Rheumatic diseases clinics of North America (1991)
    43. [43]
      Nutritional influences on immunity. — Burkholder WJ, Swecker WS Seminars in veterinary medicine and surgery (small animal) (1990)
    44. [44]
      Immunoglobulin preparation: safe from virus transmission? — Uemura Y, Yokoyama K, Nishida M, Suyama T Vox sanguinis (1989)
    45. [45]
      Jacalin: a new laboratory tool in immunochemistry and cellular immunology. — Aucouturier P, Pineau N, Brugier JC, Mihaesco E, Duarte F, Skvaril F et al. Journal of clinical laboratory analysis (1989)
    46. [46]
      Identification of a human bone marrow-derived immunoenhancing factor, BDEF. — Mortari F, Saffran DC, Singhal SK Cellular immunology (1989)
    47. [47]
      A second variant of properdin deficiency: the detection of properdin at low concentrations in affected males. — Sjöholm AG, Söderström C, Nilsson LA Complement (Basel, Switzerland) (1988)
    48. [48]
      Brachioproctic eroticism and transmission of retrovirus associated with acquired immune deficiency syndrome (AIDS). — Donovan B, Tindall B, Cooper D Genitourinary medicine (1986)
    49. [49]
      The acquired immune deficiency syndrome: ocular findings and infection control guidelines. — Woods SL, Wakefield D, McCluskey P Australian and New Zealand journal of ophthalmology (1986)
    50. [50]
      A monoclonal antibody which differentiates between bound and free human secretory component. — Gore ME, Bunnage HJ, McIlhinney RA European journal of immunology (1984)
    51. [51]
      Conjugation of triaziquinone to immunoglobulin G by a thiolation procedure catalyzed by 2-pyridinealdoxime methiodide. — Warzynski MJ, Cochran KW, Ackermann WW Journal of immunological methods (1980)
    52. [52]
      Evaluation of two commercially available laser nephelometers and their recommended methods for measurement of serum immunoglobulin G. — Walsh RL, Coles ME, Geary TD Clinical chemistry (1979)
    53. [53]
      Thymic weight in pantothenic acid deficiency. — Mahboob S Nutrition and metabolism (1976)
    54. [54]
      A hereditary immunoglobulin A abnormality: absence of light-heavy-chain assembly. Study of immunoglobulin synthesis in tonsillar cells. — Moroz C, Amir J, De Vries A The Journal of clinical investigation (1971)
    55. [55]
      Preparation and characterization of antigen and antibody adsorbents covalently coupled to an inorganic carrier. — Weetall HH The Biochemical journal (1970)
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