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Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune dysregulation that encompasses a broad range of underlying genetic diseases and infectious triggers. Monogenic conditions, autoimmune diseases, and infections can all drive the phenotype of HLH and associated immune hyperactivation with hypercytokinemia. A diagnosis of HLH usually requires a combination of clinical and laboratory findings; there is no single sensitive and specific diagnostic test, which often leads to "diagnostic dilemmas" and delays in treatment initiation. Ferritin levels, one of the most commonly used screening tests, were collected across a large tertiary care pediatric hospital to identify the positive predictive value for HLH. Herein, we present several cases that illustrate the clinical challenges of confirming an HLH diagnosis. Additionally, we report on the utility of establishing a formal multi-disciplinary group to aid the prompt diagnosis and treatment of patients presenting with HLH-like pathophysiologies., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Radiofrequency ablation (RFA) of the medial branch nerves that innervate the facet joints is a well-established treatment modality; however, studies to determine the optimal radiofrequency ablation temperature are lacking. A wide range (70 to 90°C) has been used. This study aimed to compare outcomes with two set temperatures for the lumbar facet medial branch ablation, 90 and 80°C., Methods: This retrospective study compared the degree of patient self-reported functional improvement relief, postoperative opioid dose changes, as well as duration among lumbar facet medial branch (RFA) patients who had the procedures performed at 80 or 90°C., Results: Patients who underwent the procedure at 90°C had 3.1 (95% CI 1.7, 6.5) times the odds (P = 0.0004) of reporting functional improvement of at least 50% when compared to those who underwent neurotomy at 80°C. For self-reported functional improvement greater or equal to 75%, the results were sustained with an odds ratio of 2.8 (95% CI 1.2, 5.7) favoring those with 90°C temperature neurotomy (P = 0.002)., Conclusion: There seems to be significant functional improvement associated with temp of 90°C compared to 80°C, with no added risk of complications. Randomized controlled studies are warranted., (© 2015 World Institute of Pain.)

Background: We analyzed a large number of demographic and biochemical variables to identify predictors of hospitalization in subjects on peritoneal dialysis (PD)., Methods: All patients initiated on PD at our center from January 1990 through December 1999 were included. The following variables at the initiation of PD were included: demographics, clinical data, nutritional and adequacy parameters, transport characteristics, and various co-morbidities. Co-morbidities were graded for severity using a modified version of the Index of Coexistent Disease. Variables included during the course of PD consisted of weighted time average of a number of laboratory, adequacy, and nutritional parameters along with the number of peritonitis episodes per year. Stepwise linear regression was used following a univariate screening procedure to identify independent predictors of the outcome of hospitalization days per month on PD., Results: The subject population consisted of 191 subjects (105 men, 86 women; 180 Caucasians, 10 African-American, 1 Asian). The mean age was 61 +/- 13 (SD) years and mean duration of follow-up was 21 +/- 18 months. The baseline variable analysis revealed that the presence of partner to perform PD predicted increased hospitalization (p < 0.0001). Additionally, the presence and severity of peripheral vascular disease and residual renal Kt/V at baseline (negative association) predicted increased hospitalization. In the analyses of ongoing variables, stepwise linear regression solely identified weighted time average albumin as a strong negative predictor of hospitalization (p < 0.0001)., Conclusion: A comprehensive analysis of a large number of variables revealed that serum albumin during the course of PD (negative association) and the need for partner to perform PD strongly predicted increased hospitalization in PD subjects., (2007 S. Karger AG, Basel)

Background: The study was designed to identify predictors of death in subjects on peritoneal dialysis (PD)., Methods: The population consisted of patients initiated on PD at the University of Missouri-Columbia and Dialysis Clinic Incorporated from January 1, 1990, through December 31, 1999. Baseline variables included demographics, clinical data, initial measures of nutritional status, adequacy, and transport characteristics. Co-morbidities were scored using a modified version of the Index of Coexistent Disease. Ongoing (during the course of PD) variables consisted of clinical characteristics and weighted time average of a number of laboratory, adequacy, and nutritional variables. The variables were screened using a univariate procedure, and then analyzed using stepwise logistic regression to evaluate their independent relation to death., Results: There were 105 men and 86 women--180 Caucasians, 10 African-American, 1 Asian, mean age 61 +/- 13 (SD) years, and mean duration of follow-up 21 +/- 18 months. Eighty-two patients suffered the outcome of death. Lean body mass (LBM) at the initiation of PD was negatively associated with the risk of death (p < 0.01). In addition, the need for a partner to perform PD, total morbidity count, and the summated severity score of all co-morbidities were associated with an increased risk of death. The analysis of ongoing variables revealed that serum phosphate (negative association, p = 0.02) and number of hospitalization days per month on PD (p = 0.0006) were associated with an increased risk of death., Conclusion: Phosphate levels and LBM are strong negative predictors of death in PD subjects. Further, patients who need the assistance of a partner to perform PD have decreased survival., (Copyright (c) 2005 S. Karger AG, Basel.)

Background: The present study was designed to determine the reliability of the current practice of random pre-dialysis phosphate testing in subjects receiving hemodialysis therapy, since phosphate levels exhibit a significant variation in a 24-hour period under usual physiologic conditions., Methods: Subjects receiving chronic hemodialysis (HD) were invited to participate during an incidental hospitalization. In Study A subjects (n=31) had serum phosphate tested three times on a single non-dialysis day, between 6 to 7 am (A1), between 11 am to 12:30 pm (A2), and 3:45 pm to 4:45 pm (A3). In study B subjects (n=25) had serum phosphate tested just before (B1) and 2 hours after lunch (B2), on a non-dialysis day. For Study A the collection times coincided with the start times of the out-patient dialysis shifts at our institution. All patients continued their usual phosphate binder therapy, if any. For study A the results were analyzed using one-way repeated measures analysis of variance or Friedman repeated measures analysis of variance on ranks, as opposite. Paired t-test was used for Study B. Results are expressed as mean+/- standard deviation., Results: Twenty-three men and eight women (mean age 62.2+/-15.2 years) were enrolled in Study A (24 Caucasian, 7 African-American). Nineteen men and six women (mean age 65.5+/-12.8 years) participated in study B (19 Caucasian, 6 African-American). In Study A, there was no significant difference in the mean serum phosphate levels in the samples collected through the day A1 (4.5+/-1.3 mg/dL), A2 (4.5+/-1.3 mg/dL) and A3 (4.7+/-1.5 mg/dL) (p=0.19 for comparison of the three). The mean amplitude of circadian variation (peak minus trough) was 0.64+/- 0.37 mg/dL. Similarly, there was no significant difference in the mean serum phosphate before (4.4+/-1.4 mg/dL) and two hours after lunch (4.4+/-1.5 mg/dL) (p =0.6). The mean of the difference in serum phosphate (post-prandial minus pre-prandial) was 0.2+/-0.4 mg/dL., Conclusion: Our results in hospitalized HD subjects indicate there is no significant difference in phosphate levels at different times of a single day or in relation to meals.