ABSTRACT
Primary immune regulatory disorders (PIRDs) constitute a spectrum of inborn errors of immunity (IEIs) that are primarily characterized by autoimmunity, lymphoproliferation, atopy, and malignancy. In PIRDs, infections are infrequent compared to other IEIs. While susceptibility to infection primarily stems from antibody deficiency, it is sometimes associated with additional innate immune and T or NK cell defects. The use of immunotherapy and chemotherapy further complicates the immune landscape, increasing the risk of diverse infections. Recurrent sinopulmonary infections, particularly bacterial infections such as those associated with staphylococcal and streptococcal organisms, are the most reported infectious manifestations. Predisposition to viral infections, especially Epstein-Barr virus (EBV)-inducing lymphoproliferation and malignancy, is also seen. Notably, mycobacterial and invasive fungal infections are rarely documented in these disorders. Knowledge about the spectrum of infections in these disorders would prevent diagnostic delays and prevent organ damage. This review delves into the infection profile specific to autoimmune lymphoproliferative syndrome (ALPS), Tregopathies, and syndromes with autoimmunity within the broader context of PIRD. Despite the critical importance of understanding the infectious aspects of these disorders, there remains a scarcity of comprehensive reports on this subject.
ABSTRACT
BACKGROUND: Influenza is a seasonal acute respiratory tract infection with different strains in circulation at different time periods with varying spectrum of clinical presentation. OBJECTIVES: To study the clinical presentation, morbidity, and mortality patterns associated with different strains of influenza virus, to identify the predominant strains related to hospitalization, and to identify the seasonal trend in hospitalization and risk factors for mortality in children aged 1-59 months hospitalized with influenza. MATERIALS AND METHODS: The records of children hospitalized with influenza were analyzed retrospectively (June 2013 to June 2018). Anonymized data from the Medical Records Department of our Institute, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), were used for the study and waiver of consent was obtained from the Institute Ethics Committee for Human Studies (JIPMER), which also approved the study. The data from the medical records were extracted as per the proforma and entered into Microsoft excel and summary statistics was obtained. Categorical data were expressed as proportion and analyzed using chi square test. Association was tested using odds ratio. RESULTS: Of the 693 children tested for influenza during the study period, 91 were found to be positive for influenza infection and out of which 68 (74.7%) were hospitalized. Infection was seen during both the summer and winter months. The predominant strain being A (H1N1) pdm09 (63.2%). The other strains found were A (H3N2) and Influenza B. The predominant diagnosis was pneumonia. The need for mechanical ventilation was more common with influenza B infection (p = 0.035). No significant risk factors for mortality could be found in our study. CONCLUSION: An absence of seasonal predilection for the disease was noted with influenza A (H1N1) pdm09 being the predominant strain with influenza B seen as an emerging strain and an important contributor towards morbidity.
