Primary Immune Deficiency Disorder Clinical Trial
Official title:
Intensive Care Unit and Secondary and Primary Immune Deficiency
Primary immune deficiencies (PID) are characterized by a failure of the immune system that is not explained by any infectious, neoplastic, or iatrogenic cause. In 2015, more than 300 different inherited rare disorders were described. The occurrence of PID in adult is rare and diagnosis may be supported by the 6 ESID signs for adult. However these warnings signs are based only on expert recommendations and do not include comprehensive symptoms of PIDs. Recurrent infections, more aggressive, are the most common mode of revelation of the PID. Less frequently, autoimmune manifestation, solid tumor, lymphoproliferation tumor, chronic granulomatosis or hemophagocytic lymphohistiocytosis syndrome (HLS) may also revealed a PID. The objective of this study was to evaluate the occurrence of unknown PID in adult admitted in critical care unit and to determinate if the investigation of PID in patients with severe infections or HLS should be routinely performed in MCIU.
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