A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes.

Familial hemophagocytic lymphohistiocytosis (FHL) is a life-threatening disorder of immune regulation caused by defects in lymphocyte cytotoxicity. Rapid differentiation of primary, genetic forms from secondary forms of hemophagocytic lymphohistiocytosis (HLH) is crucial for treatment decisions. We prospectively evaluated the performance of degranulation assays based on surface up-regulation of CD107a on natural killer (NK) cells and cytotoxic T lymphocytes in a cohort of 494 patients referred for evaluation for suspected HLH. Seventy-five of 77 patients (97%) with FHL3-5 and 11 of 13 patients (85%) with Griscelli syndrome type 2 or Chediak-Higashi syndrome had abnormal resting NK-cell degranulation. In contrast, NK-cell degranulation was normal in 14 of 16 patients (88%) with X-linked lymphoproliferative disease and in 8 of 14 patients (57%) with FHL2, who were identified by diminished intracellular SLAM-associated protein (SAP), X-linked inhibitor of apoptosis protein (XIAP), and perforin expression, respectively. Among 66 patients with a clinical diagnosis of secondary HLH, 13 of 59 (22%) had abnormal resting NK-cell degranulation, whereas 0 of 43 had abnormal degranulation using IL-2-activated NK cells. Active disease or immunosuppressive therapy did not impair the assay performance. Overall, resting NK-cell degranulation below 5% provided a 96% sensitivity for a genetic degranulation disorder and a specificity of 88%. Therefore, degranulation assays allow a rapid and reliable classification of patients, benefiting treatment decisions.

An audit of the adequacy of acute wound care documentation of surgical inpatients.

AIMS AND OBJECTIVES: This study examined the degree to which acute wound care documentation by doctors and nurses meets the standards set in the Australian Wound Management Association guidelines, focusing on clinical history with regard to the wound, wound characteristics, evidence of a management plan and factors such as wound pain. BACKGROUND: Wound care documentation is an important component of 'best practice' wound management. Evidence suggests that wound documentation by hospital staff is often ad hoc and incomplete. DESIGN: Survey. METHOD: An audit of acute wound care documentation of inpatients admitted to a surgical ward was conducted in 2006 using the progress notes of 49 acute inpatients in a regional Australian hospital. The audit focused on wound documentation on admission and during dressing changes. RESULTS: The findings demonstrated that, whereas doctors and nurses documented different aspects of the wound on admission, three quarters of patients had no documentation of wound margins and over half had no documentation of wound dimensions, exudate and wound bed. Whereas 122 dressing changes were documented by nurses and 103 by doctors, only 75 (60%) were reviewed by both medical and nursing staff. Doctors and nurses tended to document different aspects of dressing changes; however, in more than half the cases, there was no documentation about wound bed, margins, exudate and state of surrounding skin, whereas wound dimensions and skin sensation were recorded in less than 5%. CONCLUSION: Wound care documentation by doctors and nurses does not meet the Australian standard. The findings suggest there is ineffective communication about wound care in the multidisciplinary setting of the hospital. RELEVANCE TO CLINICAL PRACTICE: The article concludes that hospitals need to engage medical and nursing staff in collaborative processes to identify the issues that underpin poor wound documentation and to implement interventions to ensure best practice is achieved.