Transferrin Saturation/Hepcidin Ratio Discriminates TMPRSS6-Related Iron Refractory Iron Deficiency Anemia from Patients with Multi-Causal Iron Deficiency Anemia.

Pathogenic TMPRSS6 variants impairing matriptase-2 function result in inappropriately high hepcidin levels relative to body iron status, leading to iron refractory iron deficiency anemia (IRIDA). As diagnosing IRIDA can be challenging due to its genotypical and phenotypical heterogeneity, we assessed the transferrin saturation (TSAT)/hepcidin ratio to distinguish IRIDA from multi-causal iron deficiency anemia (IDA). We included 20 IRIDA patients from a registry for rare inherited iron disorders and then enrolled 39 controls with IDA due to other causes. Plasma hepcidin-25 levels were measured by standardized isotope dilution mass spectrometry. IDA controls had not received iron therapy in the last 3 months and C-reactive protein levels were <10.0 mg/L. IRIDA patients had significantly lower TSAT/hepcidin ratios compared to IDA controls, median 0.6%/nM (interquartile range, IQR, 0.4-1.1%/nM) and 16.7%/nM (IQR, 12.0-24.0%/nM), respectively. The area under the curve for the TSAT/hepcidin ratio was 1.000 with 100% sensitivity and specificity (95% confidence intervals 84-100% and 91-100%, respectively) at an optimal cut-off point of 5.6%/nM. The TSAT/hepcidin ratio shows excellent performance in discriminating IRIDA from TMPRSS6-unrelated IDA early in the diagnostic work-up of IDA provided that recent iron therapy and moderate-to-severe inflammation are absent. These observations warrant further exploration in a broader IDA population.

The difference between the patients' initial and previously measured systolic blood pressure as predictor of mortality in older emergency department patients.

PURPOSE: To assess how often baseline systolic blood pressure (SBP) could be retrieved from the Electronic Health Record (EHR) in older Emergency Department (ED) patients. Second, to assess whether the difference between baseline SBP and initial SBP in the ED (ΔSBP) was associated with 30-day mortality. METHODS: A multicenter hypothesis-generating cohort study including patients ≥ 70 years. EHRs were searched for baseline SBPs. The association between ΔSBP and 30-day mortality was investigated. RESULTS: Baseline SBP was found in 220 out of 300 patients (73.3%; 95%CI 68.1-78.0%). In 72 patients with normal initial SBPs (133-166 mmHg) in the ED, fifteen (20.8%) had a negative ΔSBP with 20.0% mortality. A negative ΔSBP was associated with 30-day mortality (AHR 4.7; 1.7-12.7). CONCLUSION: Baseline SBPs are often available in older ED patients. The ΔSBP has prognostic value and could be used as an extra variable to recognize hypotension in older ED patients. Future studies should clarify whether the ΔSBP improves risk stratification in the ED.

Paediatric procedural sedation and analgesia by emergency physicians in a country with a recent establishment of emergency medicine.

OBJECTIVES: Paediatric patients receive less procedural sedation and analgesia (PSA) in the emergency department compared with adults, especially in countries where emergency medicine is at an early stage of development. The objectives of this study were to evaluate the adverse events and efficacy of paediatric PSA in a country with a recent establishment of emergency medicine and to describe which factors aided implementation. METHODS: This is a prospective, multicentre, observational study of paediatric patients undergoing PSA by the first trained emergency physicians (EPs) in The Netherlands. A standardized data collection form was used at all participating hospitals to collect data on adverse events, amnesia, pain scores, and procedure completion. A survey was used to interpret which factors had aided PSA implementation. RESULTS: We recorded 351 paediatric PSA. The mean age was 9.5 years (95% confidence interval: 9.1-10.0). Esketamine was most frequently used (42.4%), followed by propofol (34.7%). The adverse event rate was low (3.0%). Amnesia was present in 86.8%. The median pain score was 2 (out of 10) for patients without amnesia. Procedures were successfully completed in 93.9% of the cases. CONCLUSION: Paediatric PSA provided by the first EPs in The Netherlands showed appropriate levels of sedation and analgesia with a high rate of procedure completion and a low rate of adverse events. Our paper suggests that EPs provided with a proper infrastructure of mentorship, training and guidelines can implement effective paediatric PSA.