Gastrointestinal Manifestations in Patients with Agammaglobulinemia
-
Kawthar Jasim Mohammad Rida Al-Hussieni
,
Erfan Rasouli
,
Gholamreza Azizi
,
Mehdi Mosavian
,
Rahman Matani
,
Marzieh Tavakol
Abstract
Background/objectives: The most common symptoms of agammaglobulinemia are recurrent respiratory and gastrointestinal problems. The purpose of this study was to define the prevalence and type of gastrointestinal (GI) manifestations in patients with agammaglobulinemia.
Methods: A total of 147 patients with agammaglobulinemia were entered into this study. For each patient clinical, immunological and laboratory data were documented.
Results: The GI manifestations as the first presentation of the immunodeficiency diseases were reported in 14.5% of patients mostly due to chronic and recurrent diarrhea. History of GI manifestations was evident in 67 patients (45.6%). Recurrent, chronic or bloody diarrhea (36.1%), vomiting (10.2%), and gastroenteritis (6.1%) were the most common GI manifestations in patients with agammaglobulinemia, respectively.
Conclusion: The bowel is exposed to multiple antigens including gut microbiota and pathogens from the environment. Mucosal antibodies play a major role in protection and homeostasis; hence, decreasing level of IgA can provide the opportunity for infectious diarrhea and more pathogen to attack.
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2. Tsukada S, Saffran DC, Rawlings DJ, Parolini O, Allen RC, Klisak I, et al. Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia. Cell. 1993;72(2):279-90.
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4. El-Sayed ZA, Abramova I, Aldave JC, AlHerz W, Bezrodnik L, Boukari R, et al. Xlinked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world. World Allergy Organization Journal. 2019;12(3):100018.
5. Hernandez-Trujillo VP, Scalchunes C, Cunningham-Rundles C, Ochs HD, Bonilla FA, Paris K, et al. Autoimmunity and inflammation in X-linked agammaglobulinemia. Journal of clinical immunology. 2014;34(6):627-32.
6. Pac M, Bernatowska EA, Kierkuś J, Ryżko JP, Cielecka-Kuszyk J, Jackowska T, et al. Gastrointestinal disorders next to respiratory infections as leading symptoms of X-linked agammaglobulinemia in children–34-year experience of a single center. Archives of medical science: AMS. 2017;13(2):412.
7. Bal SK, Haskologlu S, Serwas NK, Islamoglu C, Aytekin C, Kendirli T, et al. Multiple presentations of LRBA deficiency: a single-center experience. Journal of clinical immunology. 2017;37(8):790-800.
8. Agarwal S, Mayer L. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clinical gastroenterology and hepatology. 2013;11(9):1050-63.
9. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. Journal of clinical immunology. 2006;26(6):519-32.
10. Mahdaviani SA, Shirkani A, Chavoshzadeh Z. Gastrointestinal manifestations of Iranian patients with LRBA deficiency. Immunology and Genetics Journal. 2018;1(2):93-102.
11. Gupta D, Thakral D, Kumar P, Kabra SK, Lodha R, Kumari R, et al. Primary Immunodeficiency Disorders Among North Indian Children. The Indian Journal of Pediatrics. 2019:1-7.
12. Eren M, SALTIK‐TEMIZEL IN, YÜCE A, ÇAĞLAR M, Kocak N. Duodenal appearance of giardiasis in a child with selective immunoglobulin A deficiency. Pediatrics International. 2007;49(3):409-11.
13. Oksenhendler E, Gérard L, Fieschi C, Malphettes M, Mouillot G, Jaussaud R, et al. Infections in 252 patients with common variable immunodeficiency. Clinical Infectious Diseases. 2008;46(10):1547-54.
14. Parvaneh L, Sharifi N, Azizi G, Abolhassani H, Sharifi L, Mohebbi A, et al. Infectious etiology of chronic diarrhea in patients with primary immunodeficiency diseases. European annals of allergy and clinical immunology. 2019;51(1):32-7.
15. Ghoshal UC, Goel A, Ghoshal U, Jain M, Misra A, Choudhuri G. Chronic diarrhea and malabsorption due tohypogammaglobulinemia: a report on twelve patients. Indian Journal of Gastroenterology. 2011;30(4):170.
2. Tsukada S, Saffran DC, Rawlings DJ, Parolini O, Allen RC, Klisak I, et al. Deficient expression of a B cell cytoplasmic tyrosine kinase in human X-linked agammaglobulinemia. Cell. 1993;72(2):279-90.
3. Vetrie D, Vořechovský I, Sideras P, Holland J, Davies A, Flinter F, et al. The gene involved inX-linked agammaglobulinaemia is a member of the src family of protein-tyrosine kinases. Nature. 1993;361(6409):226.
4. El-Sayed ZA, Abramova I, Aldave JC, AlHerz W, Bezrodnik L, Boukari R, et al. Xlinked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world. World Allergy Organization Journal. 2019;12(3):100018.
5. Hernandez-Trujillo VP, Scalchunes C, Cunningham-Rundles C, Ochs HD, Bonilla FA, Paris K, et al. Autoimmunity and inflammation in X-linked agammaglobulinemia. Journal of clinical immunology. 2014;34(6):627-32.
6. Pac M, Bernatowska EA, Kierkuś J, Ryżko JP, Cielecka-Kuszyk J, Jackowska T, et al. Gastrointestinal disorders next to respiratory infections as leading symptoms of X-linked agammaglobulinemia in children–34-year experience of a single center. Archives of medical science: AMS. 2017;13(2):412.
7. Bal SK, Haskologlu S, Serwas NK, Islamoglu C, Aytekin C, Kendirli T, et al. Multiple presentations of LRBA deficiency: a single-center experience. Journal of clinical immunology. 2017;37(8):790-800.
8. Agarwal S, Mayer L. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clinical gastroenterology and hepatology. 2013;11(9):1050-63.
9. Rezaei N, Aghamohammadi A, Moin M, Pourpak Z, Movahedi M, Gharagozlou M, et al. Frequency and clinical manifestations of patients with primary immunodeficiency disorders in Iran: update from the Iranian Primary Immunodeficiency Registry. Journal of clinical immunology. 2006;26(6):519-32.
10. Mahdaviani SA, Shirkani A, Chavoshzadeh Z. Gastrointestinal manifestations of Iranian patients with LRBA deficiency. Immunology and Genetics Journal. 2018;1(2):93-102.
11. Gupta D, Thakral D, Kumar P, Kabra SK, Lodha R, Kumari R, et al. Primary Immunodeficiency Disorders Among North Indian Children. The Indian Journal of Pediatrics. 2019:1-7.
12. Eren M, SALTIK‐TEMIZEL IN, YÜCE A, ÇAĞLAR M, Kocak N. Duodenal appearance of giardiasis in a child with selective immunoglobulin A deficiency. Pediatrics International. 2007;49(3):409-11.
13. Oksenhendler E, Gérard L, Fieschi C, Malphettes M, Mouillot G, Jaussaud R, et al. Infections in 252 patients with common variable immunodeficiency. Clinical Infectious Diseases. 2008;46(10):1547-54.
14. Parvaneh L, Sharifi N, Azizi G, Abolhassani H, Sharifi L, Mohebbi A, et al. Infectious etiology of chronic diarrhea in patients with primary immunodeficiency diseases. European annals of allergy and clinical immunology. 2019;51(1):32-7.
15. Ghoshal UC, Goel A, Ghoshal U, Jain M, Misra A, Choudhuri G. Chronic diarrhea and malabsorption due tohypogammaglobulinemia: a report on twelve patients. Indian Journal of Gastroenterology. 2011;30(4):170.
| Files | ||
| Issue | Vol 2, No 3 (2019) | |
| Section | Original Article | |
| DOI | https://doi.org/10.22034/igj.2019.200932.1025 | |
| Keywords | ||
| Gastrointestinal Agammaglobulinemia Diarrhea Vomiting Gastroenteritis | ||
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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Jasim Mohammad Rida Al-Hussieni K, Rasouli E, Azizi G, Mosavian M, Matani R, Tavakol M. Gastrointestinal Manifestations in Patients with Agammaglobulinemia. Immunol Genet J. 2019;2(3):104-113.
