Association Between Immune Dysfunction and Sepsis Severity in Children with Primary Immunodeficiency: A Retrospective Analysis

Sepsis continues to be one of the major morbidities and mortalities among paediatric patients, especially in children with a primary immunodeficiency (PID). These patients are characterised by high susceptibility to severe infections, atypical clinical manifestations, and high probability of rapid progression of disease due to congenital defects of the immune system. The current research intends to examine clinical and immunological characteristics of sepsis in children with PID in terms of retrospective data analysis of cases treated in the Tashkent City Children's Clinic. A retrospective study was performed through the medical records of children diagnosed with PID and sepsis within a fixed study period. Clinical data and laboratories, immu- nological parameters, causative pathogens, treatment methods and outcomes were assessed. The features of immune dysfunction, such as the defects in humoral and cellular immunity, and their correlation with the progression and severity of sepsis, were singled out. The review showed that children with PID tend to exhibit severe and frequent septic events, late diagnosis, and length of stay.The prevalent ones were the persistent leukopenia or lymphopenia, low levels of immunoglobulins, and the lack of an inflammatory response. Opponent pathogens and Gram-negative bac- teria were often verified as causative agents. Despite the intensive antimicrobial and supportive treatment, the threat of complications and the adverse outcomes were still very great in comparison with immunocompetent children. These results demonstrate the need to undertake early identification of PID in the septic child, timely immuno- logical evaluation, and adopt a personalised treatment plan. Awareness of diagnosis and optimal multidisciplinary care can help improve the prognosis and decrease mor- tality in the susceptible population of such patients.

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