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OBJECTIVES: To present a case of human monocytic ehrlichiosis (HME) that was complicated by macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis (sHLH). METHODS: Data was collected from patient's electronic medical records at the University of Alabama at Birmingham. The patient is a part of a larger cohort of patients with all-cause MAS treated at our center. CASE: A 63 year old renal transplant recipient male on maintenance immunosuppressive therapy presented with high grade fever, leukopenia, thrombocytopenia and elevated transaminases and initially met clinical criteria for severe sepsis. On further investigation, clinical and laboratory criteria for MAS were met. He was treated with a combination of doxycycline for HME and a novel combination of anakinra (interleukin-1 receptor antagonist), and high dose corticosteroids. The discussion focuses on clinical presentation, pathogenesis and treatment of MAS with an emphasis on MAS secondary to HME. CONCLUSION: Macrophage activation syndrome or sHLH is a dysfunctional, hyperactive and potentially fatal immune system response that results in multi-organ dysfunction. With increasing incidence of Ehrlichia chaffeensis as an emerging pathogen, clinicians should be aware of this fulminant and potentially fatal complication of HME.
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BACKGROUND: The diagnostic yield of capsule endoscopy is vulnerable to inadequate visualization related to residual bile or chyme remaining in the lumen despite intestinal lavage. It has been challenging to determine the optimal lavage preparation of the bowel and patient diet before capsule endoscopy, as well as the timing of the procedure, because no well-accepted, validated grading system for assessing the quality of intestinal lavage before capsule endoscopy is available. There remains no consensus on the reliability of qualitative, quantitative, or computer-derived assessments of the quality of preparation for capsule endoscopy. This study evaluates intra-observer and interobserver agreement for a previously validated scale. MATERIALS AND METHODS: The digital images of 34 patients who underwent capsule endoscopy were independently reviewed by two blinded physicians according to a previously validated grading scale. One of the physicians reviewed and graded the patients a second time. The quality of the bowel luminal preparation was assessed with a qualitative parameter (fluid transparency) and a more quantitative parameter (mucosal invisibility) for each of three small-intestinal segments, and an overall small-bowel score for each parameter was assigned as well. A weighted kappa coefficient was used to calculate intra-observer (observer 1A and 1B) and interobserver (observer 1A and observer 2) agreement. A kappa value of 0.60 or more suggests strong agreement, 0.40 to 0.60 moderate agreement, and less than 0.40 poor agreement. RESULTS: The intra-observer weighted kappa index for both fluid transparency and mucosal visibility was 0.52, which is consistent with moderate agreement. The interobserver weighted kappa indices for fluid transparency and mucosal invisibility were 0.29 and 0.42, respectively, demonstrating suboptimal interobserver agreement. The individual segment interobserver kappa indices were better for mucosal visibility (0.52, 0.39, and 0.47 for small-bowel segments 1, 2, and 3, respectively) than for fluid transparency (0.18, 0.38, and 0.31). CONCLUSIONS: The proposed grading scale for assessing the quality of preparation for capsule endoscopy has inadequate interobserver and intra-observer agreement. Capsule endoscopy preparation grading scales that focus more on quantitative than on qualitative assessment may demonstrate more reliable performance characteristics. Optimizing the quality of preparation and diagnostic yield of capsule endoscopy will first require the development of a well-validated grading scale.