ORIGINAL RESEARCH
Pediatrics
doi: 10.25005/2074-0581-2026-28-3-696-703
IMMUNOLOGICAL ALTERATIONS AND SUSCEPTIBILITY TO INFECTIONS IN CHILDREN WITH ALPORT SYNDROME: IMPACT ON DISEASE SEVERITY

M. BOLTABOEVA1, M. GANIEVA1, Z. URUMBOEVA1, N. MADJIDOVA1, A. HAYDAROV1, L. RAKHMANOVA2

1Department of Hospital Pediatrics, Andijan State Medical Institute, Andijan, Republic of Uzbekistan
2Department of Pediatrics Diseases in Family Medicine, Tashkent State Medical University, Tashkent, Republic of Uzbekistan

Objective: To investigate the levels of interleukin-2 (IL-2), complement components C3 and C4, and antigen-binding lymphocytes (ABLs) in children with AS and to assess their associations with the clinical course of the disease and the frequency of infectious diseases.

Methods: A total of 30 children diagnosed with AS were examined: 14 had AS alone, and 16 had AS combined with nephrotic syndrome (AS+NS). The control group consisted of 25 healthy children aged 3-18 years. ABLs were determined using the rosette formation assay, complement components C3 and C4 – by immunoturbidimetry, and IL-2 – by enzyme-linked immunosorbent assay (ELISA). Categorical variables were analyzed using Fisher’s exact test. Quantitative data (M±SD) were analyzed using one-way analysis of variance (one-way ANOVA) followed by Tukey’s HSD test. Associations between variables were assessed using Pearson’s correlation analysis.

Results: Data analysis demonstrated that IL-2 and ABL levels were significantly higher, whereas C3 and C4 levels were lower, in both the AS+NS and AS groups (p<0.001). Correlation analysis in children with AS revealed a statistically significant (p=0.01) moderate inverse correlation between IL-2 levels and glomerular filtration rate (GFR). In the AS+NS group, a statistically significant (p=0.01) strong inverse correlation between IL-2 and GFR was observed. In the AS+NS group, biomarker levels were strongly correlated with the annual number of various infectious episodes, including acute respiratory viral infections, bronchitis, pneumonia, and streptoderma. Specifically, the annual number of infectious episodes positively correlated with IL-2 levels and blood urea concentrations and negatively correlated with complement component C3 levels.

Conclusion: Immunological differences between the AS and AS+NS groups, including elevated IL-2 and ABL levels and reduced concentrations of complement components C3 and C4, were associated with disease severity. These findings suggest that immunological markers serve as potential biomarkers for assessing AS progression. This study is the first to demonstrate an association between immune dysfunction and the frequency of infectious diseases in patients with AS.

Keywords: Alport syndrome, interleukin-2, antigen-binding lymphocytes, immune imbalance, glomerular filtration rate, complement.

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Authors' information:

Boltaboeva Muqaddas,
Candidate of Medical Sciences, Assistant Professor of the Department of Hospital Pediatrics, Andijan State Medical Institute
ORCID ID: 0009-0000-8421-9592
E-mail: boltaboyeva.mukaddas@mail.ru

Ganieva Marifat,
Candidate of Medical Sciences, Head of the Department of Hospital Pediatrics, Andijan State Medical Institute
ORCID ID: 0000-0001-5662-5390
E-mail: ganieva.marifat@mail.ru

Urumboeva Zamira,
Candidate of Medical Sciences, Assistant Professor of the Department of Hospital Pediatrics, Andijan State Medical Institute
ORCID ID: 0000-0003-2997-7226
E-mail: umarovazamirahon6@gmail.com

Madjidova Nilufar,
Assistant Professor of the Department of Hospital Pediatrics, Andijan State Medical Institute
ORCID ID: 0009-0001-0509-0303
E-mail: magjidovanilufar87@gmail.com

Haydarov Abdulhamid,
Master's Student, Department of AnaesthesiologyReanimatology and Emergency Medicine, Andijan State Medical Institute
ORCID ID: 0009-0007-7195-2542
E-mail: a_xaydarov@icloud.com

Rakhmanova Lola,
Doctor of Medical Sciences, Senior Researcher of the Department of Pediatrics Diseases in Family Medicine, Tashkent State Medical University
ORCID ID: 0000-0001-7361-9953
E-mail: lola.rahmanova61@mail.ru

Information about support in the form of grants, equipment, medications

The authors did not receive financial support from manufacturers of medicines and medical equipment

Conflicts of interest: No conflict

Address for correspondence:

Boltaboeva Muqaddas
Candidate of Medical Sciences, Assistant Professor of the Department of Hospital Pediatrics, Andijan State Medical Institute

170100, Republic of Uzbekistan, Andijan, Yu. Otabekova str., 1

Tel.: +998 (882) 725055

E-mail: doctormeeeee1999@gmail.com


This work is licensed under a Creative Commons Attribution 4.0 International License.

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