Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with hyperinflammation and multiple organ dysfunction. It has many causes, symptoms, and outcomes. Early recognition is critical for treatment. Fever, cytopenias, coagulopathy, and hepatosplenomegaly are hallmark findings. Identifying the trigger event is crucial but challenging because of the varied presentations and infrequent provider experience. Diagnostic features include anemia, thrombocytopenia, neutropenia, elevated ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis (in bone marrow, spleen, or lymph nodes), low or absent natural killer cells, and elevated soluble interleukin 2 receptor assay. Primary treatment goals are eliminating the underlying trigger and suppressing hyperinflammation with steroids, immunoglobulins, or immunomodulators. Specific treatment includes corticosteroids, etoposide, and antithymocyte globulin followed by hematopoietic stem cell transplantation in patients with refractory or relapsing disease. Prompt immunochemical therapy is essential but often complicated by a high risk of treatment-related morbidity and disease recurrence. Despite these challenges, improvements in diagnostic technology and treatment have enhanced survival.
Intestinal obstruction, a disorder that afflicts both children and adults, is associated with significant morbidity. Early recognition and appropriate management can prevent life-threatening complications; however, considerable controversies exist regarding the ideal method of diagnosis and treatment. This article provides a comprehensive overview of risk factors for small and large bowel obstruction. The pathophysiologic process is related to the clinical findings. A summary of diagnostic methods with an evaluation of their relative merit is presented. Preventive, and medical, surgical, and palliative therapies are described, with an emphasis on the actions that an advanced practice nurse can implement.
Evaluation of implementation of the protocol showed improved timeliness and adherence to sepsis practice guidelines. Postintervention adherence to threshold times for obtaining blood cultures and blood lactate and start of antibiotics showed improvement. All recommended interventions were completed within the target time frame for the majority of patients.
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