Pediatrics international : official journal of the Japan Pediatric Society
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A significant proportion of cases of tracheobronchial foreign body aspiration due to life-threatening condition is observed during childhood. The aim of the present study was to describe our experience with the diagnosis and treatment of foreign body aspirations during childhood and review published literature. ⋯ Tracheobronchial foreign body aspiration is a significant cause of childhood morbidity and mortality. Early diagnosis and treatment is of utmost importance. Rigid bronchoscopy under general anesthesia should be performed in all patients suspected of foreign body aspiration, which could minimize mortality and morbidity if performed by experienced personnel with safe methods.
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Hemophagocytic lymphohistiocytosis (HLH) is a multisystem inflammatory disorder due to cytokine overproduction from excessively activated lymphocytes and macrophages. HLH has been divided into two subgroups: primary HLH and secondary HLH. Primary HLH includes PRF1, UNC13D, STX11, STXBP2, RAB27A, LYST, SH2D1A and XIAP gene mutations; and secondary HLH is associated with infections, malignancies and autoimmune diseases. ⋯ Analysis of clinical phenotypes of XLP patients suggests that XLP predominantly shows familial HLH phenotypes, whereas some XLP patients present sporadic HLH. For many decades, clinicians and investigators have been concerned with possible XLP in young boys presenting with Epstein-Barr-virus-associated HLH. This review aims to describe the new knowledge about XLP and to draw the attention of the pediatrician to XLP, which should be differentiated from other forms of HLH.