Nephrotic Syndrome and Recurrent Infection
,
Abstract
Nephrotic syndrome is characterized by the leakage of protein from the blood into the urine along with the triad of proteinuria, albuminuria, and peripheral edema. Loss of protein leads to the loss of immunoglobulin and complements. X-linked agammaglobulinemia (XLA), or Bruton disease, is a primary immunodeficiency disease caused by a defect in the development of B cells in the bone marrow and a low serum level of immunoglobulins. The present case involves a 12-year-old boy with nephrotic syndrome, osteomyelitis, and recurrent infections. We discovered that he had XLA. This report underscores the importance of considering inborn errors of immunity in cases of protein loss, such as nephrotic syndrome.
1. Veissi S, Smeets B, Lambertus P. Nephrotic syndrome in a dish: recent developments in modeling in vitro. Pediatric Nephrol. 2020;35(8):1363–72.
2. Noone DG, Iijima K, Parekh R. Idiopathic nephrotic syndrome in children. Lancet. 2018;392(14):61–74.
3. Wiggins B, Deliwala S, Banno F, Knight K, Minaudo M. Acute Renal Failure and Nephrotic Syndrome Secondary to Collapsing Glomerulopathy Associated With Hepatitis C. 2022;14(3):e23175.
4. Tan L, An Y, Yang Q, Yang H, Zhang G, Li Q, Wang M. A novel FOXP3 mutation in a Chinese child with IPEX-associated membranous nephropathy. Mol Genet Genomic Med. 2022;10(6):e1945.
5. Di Genova L, Ceppi S, Stefanelli M, Esposito S. IgA Deficiency and Nephrotic Syndrome in Children. Int J Environ Res Public Health. 2018;15(8):1702.
6. Aird A, Lagos M, Vargas-Hernández A. Novel Heterozygous Mutation in NFKB2 Is Associated With Early Onset CVID and a Functional Defect in NK Cells Complicated by Disseminated CMV Infection and Severe Nephrotic Syndrome. Front Pediatr. 2019;7:303.
7. Jiang X, Chen J, Gu FF, Li ZR, Song YS, Long JJ, et al. IgA nephropathy in a child with X-linked agammaglobulinemia: a case report. BMC Pediatrics. 2024;24(1):291.
8. Vivarelli M, Gibson K, Sinha A, Boyer O. childhood nephrotic syndrome. Lancet. 2023;402(10404):809-24.
9. Cardenas M, Vivian P, Trujillo H. Agammaglobulinemia: from X linked to Autosomal Forms of Disease. Clin Rev Allergy Immunol. 2022;63(1):22-35.
10. Lougaris V, Soresina A, Baronio M, Montin D, Martino S, Signa S, et al. Long-term follow-up of 168 patients with
X-linked agammaglobulinemia reveals increased morbidity and mortality. J Allergy Clin Immunol. 2020:148(2):429-37
11. Tamura H. Trends in pediatric nephrotic syndrome. World J Nephrol. 2021;10(5):88-100
12. El-Sayed ZA, Abramova I, Aldave JC. X-linked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world. World Allergy Organ J. 2019;12(3):100018.
13. Rawat A, Jindal AK, Suri D et al. Clinical and Genetic Profile of X-Linked Agammaglobulinemia: A Multicenter Experience From India. Front Immunol. 2021; 11:612323.
14. Youssef J, Novosad SA, Winthrop KL. Infection risk and safety of corticosteroid use. Rheum Dis Clin N Am. 2016;42(1):157-76.
15. Deng Y, Ou YY, Mo CJ, Huang L, Qin X, Li S. Peripheral blood lymphocyte subsets in children with nephrotic syndrome: a retrospective analysis. BMC Nephrology. 2023; 24(7):2-8.
16. Lavrador V, Correia F, Sampaio R, Cândido C, Sameiro-Faria M, Marques L, Mota C. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association. Case Rep Pediatr. 2014;2014:480947.
17. Abdelwahed Hussein M, Babiker M, Asim S. Membranoproliferative Glomerulonephritis Type I Associated with Intravenous Immunoglobulin Administration Arising in a Child with X-Linked Agammaglobulinemia: A Case Report and a Reappraisal. Saudi J Kidney Dis Transpl. 2023;34(6):660-5.
18. Takeguchi M, Korematsu S, Miyahara H, Kuga S, Izumi
T. IVIG-triggered tubulointerstitial nephritis in X-linked
agammaglobulinemia. Pediatr Int. 2017;59(8):945-6.
19. Jiang X, Chen J, Gu FF, Li ZR, Song YS, Long JJ, et al. IgA nephropathy in a child with X-linked agammaglobulinemia: a case report. BMC Pediatrics. 2024;24(1):291.
20. Lavrador V, Correia F, Sampaio R. Membranoproliferative glomerulonephritis and x-linked agammaglobulinemia: An uncommon association. Case Rep Pediatr. 2014;2014:480947.
21. Ruth E, Joshua M.The Immune System and Idiopathic Nephrotic Syndrome. Clin J Am Soc Nephrol. 2022;17(12): 1823–34.
2. Noone DG, Iijima K, Parekh R. Idiopathic nephrotic syndrome in children. Lancet. 2018;392(14):61–74.
3. Wiggins B, Deliwala S, Banno F, Knight K, Minaudo M. Acute Renal Failure and Nephrotic Syndrome Secondary to Collapsing Glomerulopathy Associated With Hepatitis C. 2022;14(3):e23175.
4. Tan L, An Y, Yang Q, Yang H, Zhang G, Li Q, Wang M. A novel FOXP3 mutation in a Chinese child with IPEX-associated membranous nephropathy. Mol Genet Genomic Med. 2022;10(6):e1945.
5. Di Genova L, Ceppi S, Stefanelli M, Esposito S. IgA Deficiency and Nephrotic Syndrome in Children. Int J Environ Res Public Health. 2018;15(8):1702.
6. Aird A, Lagos M, Vargas-Hernández A. Novel Heterozygous Mutation in NFKB2 Is Associated With Early Onset CVID and a Functional Defect in NK Cells Complicated by Disseminated CMV Infection and Severe Nephrotic Syndrome. Front Pediatr. 2019;7:303.
7. Jiang X, Chen J, Gu FF, Li ZR, Song YS, Long JJ, et al. IgA nephropathy in a child with X-linked agammaglobulinemia: a case report. BMC Pediatrics. 2024;24(1):291.
8. Vivarelli M, Gibson K, Sinha A, Boyer O. childhood nephrotic syndrome. Lancet. 2023;402(10404):809-24.
9. Cardenas M, Vivian P, Trujillo H. Agammaglobulinemia: from X linked to Autosomal Forms of Disease. Clin Rev Allergy Immunol. 2022;63(1):22-35.
10. Lougaris V, Soresina A, Baronio M, Montin D, Martino S, Signa S, et al. Long-term follow-up of 168 patients with
X-linked agammaglobulinemia reveals increased morbidity and mortality. J Allergy Clin Immunol. 2020:148(2):429-37
11. Tamura H. Trends in pediatric nephrotic syndrome. World J Nephrol. 2021;10(5):88-100
12. El-Sayed ZA, Abramova I, Aldave JC. X-linked agammaglobulinemia (XLA): Phenotype, diagnosis, and therapeutic challenges around the world. World Allergy Organ J. 2019;12(3):100018.
13. Rawat A, Jindal AK, Suri D et al. Clinical and Genetic Profile of X-Linked Agammaglobulinemia: A Multicenter Experience From India. Front Immunol. 2021; 11:612323.
14. Youssef J, Novosad SA, Winthrop KL. Infection risk and safety of corticosteroid use. Rheum Dis Clin N Am. 2016;42(1):157-76.
15. Deng Y, Ou YY, Mo CJ, Huang L, Qin X, Li S. Peripheral blood lymphocyte subsets in children with nephrotic syndrome: a retrospective analysis. BMC Nephrology. 2023; 24(7):2-8.
16. Lavrador V, Correia F, Sampaio R, Cândido C, Sameiro-Faria M, Marques L, Mota C. Membranoproliferative Glomerulonephritis and X-Linked Agammaglobulinemia: An Uncommon Association. Case Rep Pediatr. 2014;2014:480947.
17. Abdelwahed Hussein M, Babiker M, Asim S. Membranoproliferative Glomerulonephritis Type I Associated with Intravenous Immunoglobulin Administration Arising in a Child with X-Linked Agammaglobulinemia: A Case Report and a Reappraisal. Saudi J Kidney Dis Transpl. 2023;34(6):660-5.
18. Takeguchi M, Korematsu S, Miyahara H, Kuga S, Izumi
T. IVIG-triggered tubulointerstitial nephritis in X-linked
agammaglobulinemia. Pediatr Int. 2017;59(8):945-6.
19. Jiang X, Chen J, Gu FF, Li ZR, Song YS, Long JJ, et al. IgA nephropathy in a child with X-linked agammaglobulinemia: a case report. BMC Pediatrics. 2024;24(1):291.
20. Lavrador V, Correia F, Sampaio R. Membranoproliferative glomerulonephritis and x-linked agammaglobulinemia: An uncommon association. Case Rep Pediatr. 2014;2014:480947.
21. Ruth E, Joshua M.The Immune System and Idiopathic Nephrotic Syndrome. Clin J Am Soc Nephrol. 2022;17(12): 1823–34.
| Files | ||
| Issue | Vol 23 No 5 (2024) | |
| Section | Case Report(s) | |
| DOI | https://doi.org/10.18502/ijaai.v23i5.16754 | |
| Keywords | ||
| Bruton type agammaglobulinemia Inborn errors of immunity Nephrotic syndrome Primary immunodeficiency diseases X-linked agammaglobulinemia | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Shahraki ghadimi Z, Sadeghi Bojd S, Parvane N, Atabaki M, Alijani E. Nephrotic Syndrome and Recurrent Infection. Iran J Allergy Asthma Immunol. 2024;23(5):594-599.
