Preview

Pediatric pharmacology

Advanced search

Clinical Case of Agammaglobulinemia Late Diagnosis in Preschool Child

https://doi.org/10.15690/pf.v19i2.2376

Abstract

Background. Agammaglobulinemia is a disease from the group of primary immune deficiencies with impaired antibodies production characterized by significant decrease or complete absence of B-cells. Due to the absence of pathogenetic treatment, severe bacterial lesions of various organs and tissues can develop, and they can lead to the patient death. Timely history taking and clinical and laboratory data analysis will allow to reveal this pathology early and start the therapy that can save patient's life. Clinical case description. This article demonstrates clinical case of agammaglobulinemia late diagnosis with development of severe complications and reversal of clinical manifestations in response to pathogenetic therapy. Conclusion. Despite the disease onset in the second half-year period of life, the presence of recurrent purulent processes resistant to standard treatment and the significant decrease of all immunoglobulin levels, agammaglobulinemia was suspected only at preschool age. The outcomes were disabled patient, and considerable aggravation of the quality of life.

About the Authors

Elena S. Kolevatova
Privolzhsky Research Medical University
Russian Federation

MD, Resident at the Department of Hospital Pediatrics, Privolzhsky Research Medical University.

10/1, Minin and Pozharsky square, 603005 Nizhny Novgorod.


Disclosure of interest:

No



Alla Yu. Shutkova
Privolzhsky Research Medical University; Children's City Clinical Hospital № 1 of Prioksky district in Nizhny Novgorod
Russian Federation

MD, PhD, Associate Professor at the Department of Hospital Pediatrics, Privolzhsky Research Medical University.

10/1, Minin and Pozharsky square, 603005 Nizhny Novgorod.

tel.: +7 (831) 465-66-72

eLibrary SPIN: 1172-6732


Disclosure of interest:

No



Elena V. Tush
Privolzhsky Research Medical University; Children's City Clinical Hospital № 1 of Prioksky district in Nizhny Novgorod
Russian Federation

MD, PhD, Associate Professor at the Department of Hospital Pediatrics, Privolzhsky Research Medical University.

10/1, Minin and Pozharsky square, 603005 Nizhny Novgorod.

eLibrary SPIN: 4655-6099


Disclosure of interest:

No



Olga V. Khaletskaya
Privolzhsky Research Medical University; Children's City Clinical Hospital № 1 of Prioksky district in Nizhny Novgorod
Russian Federation

MD, PhD, Professor, the Head of the Department of Hospital Pediatrics, Privolzhsky Research Medical University.

10/1, Minin and Pozharsky square, 603005 Nizhny Novgorod.

eLibrary SPIN: 9342-9261


Disclosure of interest:

No



References

1. Pervichnye immunodefitsity s preimushchestvennoi nedostatochnost'yu sinteza antitel: Clinical guidelines. Russian Association of Allergists and Clinical Immunologists; National Association of Experts in Primary Immunodeficiencies; 2021. (In Russ).

2. Pervichnyi immunodefitsit: tyazhelaya kombinirovan-naya immunnaya nedostatochnost': Clinical guidelines. Russian Association of Allergists and Clinical Immunologists; 2019. (In Russ).

3. Kuvschinova EV, Kurtasova LM, Borisov AG, et al. Clinical case of primary immunodeficiency: X-linked agammaglobulinemia. Medical Immunology (Russia) = Meditsinskaya Immunologiya. 2020;22(2):379-382. (In Russ). doi: https://doi.org/10.15789/1563-0625-CCO-1837

4. Kondratenko IV, Bologov AA. Pervichnye immunodefitsity. Moscow: ID MEDPRAKTIKA-M; 2005. pp. 35-43. (In Russ).

5. Marcdante KJ, Kliegman RM. Nelson Essentials of Pediatrics. Kokareva EV, transl. Moscow: Eksmo; 2021. pp. 313334. (In Russ).

6. Lackey AE, Ahmad F. X-linked Agammaglobulinemia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022.

7. Schwartz RA. X-Linked (Bruton) Agammaglobulinemia Questions & Answers. In: MedScape; 2021. Available online: https://emedicine.medscape.com/article/1050956-questions-and-answers. Accessed on February 21, 2022.

8. Tangye SG, Al-Herz W, Bousfiha A, et al. Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2020;40(1):24-64. doi: https://doi.org/10.1007/s10875-019-00737-x

9. Bousfiha A, Jeddane L, Picard C, et al. Human Inborn Errors of Immunity: 2019 Update of the IUIS Phenotypical Classification. J Clin Immunol. 2020;40(1):66-81. doi: https://doi.org/10.1007/s10875-020-00758-x

10. Memorandum on the launch of a pilot project of neonatal screening to identify patients with spinal muscular atrophy (SMA) and primary immunodeficiencies (PID). In: Register of Medicinal Products of Russia. — June 10, 2021. (In Russ). Доступно по: https://www.rlsnet.ru/news_memorandum-o-zapuske-pilotnogo-proekta-neonatalnogo-skrininga-na-sma-i-pervicnye-immunodeficity.htm. Ссылка активна на 22.02.2022.


Review

For citations:


Kolevatova E.S., Shutkova A.Yu., Tush E.V., Khaletskaya O.V. Clinical Case of Agammaglobulinemia Late Diagnosis in Preschool Child. Pediatric pharmacology. 2022;19(2):123-126. (In Russ.) https://doi.org/10.15690/pf.v19i2.2376

Views: 474


ISSN 1727-5776 (Print)
ISSN 2500-3089 (Online)