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1.
BackgroundPaper-based charts remain the principal means of documenting the vital signs of hospitalised pregnant and postnatal women. However, poor chart design may contribute to both incorrect charting of data and clinical responses. We decided to identify design faults that might have an adverse clinical impact.MethodsOne hundred and twenty obstetric early warning charts and escalation protocols from consultant-led maternity units in the United Kingdom and the Channel Islands were analysed using an objective and systematic approach. We identified design errors that might impede their successful use (e.g. generate confusion regarding vital sign documentation, hamper the recognition of maternal deterioration, cause a failure of the early warning system or of any clinical response).ResultsWe found 30% (n=36/120) of charts contained at least one design error with the potential to confuse staff, render the charts difficult to use or compromise patient safety. Amongst the most common areas were inadequate patient identification, poor use of colour, illogical weighting, poor alignment and labelling of axes, and the opportunity for staff to ‘game’ the escalation.ConclusionsWe recommend the urgent development of an evidence-based, standardised obstetric observation chart, which integrates ‘human factors’ and user experience. It should have a clear layout and style, appropriate colour scheme, correct language and labelling, and the ability for vital signs to be documented accurately and quickly. It should incorporate a suitable early warning score to guide clinical management.  相似文献   

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Study objectiveTo identify demographic, intraoperative, and parental factors that influence the postoperative pain experience in ambulatory surgery pediatric patients. We also monitored postoperative maladaptive behavior changes (PMBCs) to investigate the relationship between pain and PMBCs.DesignProspective cohort study.SettingDischarge period after ambulatory surgery.Patients204 patients ages 1–6 years undergoing ambulatory orthopedic, urology, general surgery, and otolaryngology surgical procedures who were American Society of Anesthesiologists (ASA) physical status I or II.InterventionsNone.MeasurementsWe administered telephone questionnaires to parents of ambulatory surgery patients 1–6 years old exploring pain ratings, behavior change ratings, and medication compliance at 2–3 days and 1–2 weeks after surgery. Pain and behavioral change scores were obtained using the Parents Postoperative Pain Measure (PPPM) and Post-Hospital Behavior Questionnaire (PHBQ). Parental medication compliance was defined as parents who followed the discharge instructions for pain medication administration.Main resultsFor our cohort, 69% of patients experienced pain after 2–3 days and 17% after 1–2 weeks post-discharge. PMBCs were reported in 55% after 2–3 days, and in 15% after 1–2 weeks. In addition, PMBCs occurred in the absence of pain (PPPM = 0) at rates of 20% and 5% at 2–3 days and 1–2 weeks after surgery, respectively. Female sex, anesthesia duration, and otolaryngology procedures correlated with higher postoperative pain (PPPM) scores in univariate and multivariate analysis. Intraoperative medications did not correlate with PPPM or PHBQ scores. Higher pain scores were associated with parents who were compliant with discharge instructions for pain medications.ConclusionsMany pediatric patients experienced short-term pain and PMBCs after ambulatory surgery, but these largely resolved by 1–2 weeks following discharge. Patient sex, anesthesia duration, and surgical procedure influenced postoperative pain and/or PMBCs. Furthermore, PMBCs were associated with, but not solely a manifestation of, postoperative pain.  相似文献   

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《Injury》2017,48(5):1006-1012
BackgroundMany scoring systems for the early prediction of the need for massive transfusion (MT) have been reported; in most of these, vital signs are regarded as important. However, the validity of these scoring systems in older patients remains unclear because older trauma patients often present with normal vital signs. In this study, we investigated the effectiveness of previously described scoring systems, as well as risk factors that can provide early prediction of the need for MT in older severe trauma patients.MethodsWe prospectively collected data from a cohort of severe trauma patients (ISS ≥16 and age ≥16 years) admitted from January 2007 to March 2015. Trauma Associated Severe Hemorrhage (TASH), Assessment of Blood Consumption (ABC), and Prince of Wales Hospital (PWH) scores were compared between a younger and an older group. Furthermore, the predictors associated with MT in older severe trauma patients were assessed using multivariable logistic regression analyses.ResultsThe area under the curve (AUC) was significantly smaller for older group than for younger group for all three scoring systems (p < 0.05). The most important risk factors to predict the need for MT were related to anatomical factors including FAST results (odds ratio (OR): 5.58, 95% confidence interval (CI): 2.10–14.99), unstable pelvic fracture (OR: 21.56, 95% CI: 6.05–90.78), and long bone open fracture of the lower limbs (OR: 12.21, 95% CI: 4.04–39.09), along with pre-injury anticoagulant agent use (OR: 5.22, 95% CI: 1.30–19.61), antiplatelet agent use (OR: 3.81, 95% CI: 1.57–9.04), lactate levels (OR: 1.20, 95% CI: 1.04–1.39) and shock index (OR: 2.67, 95% CI: 1.05–6.84). Traditional vital signs were not early risk factors.ConclusionWe suggest that MT in older trauma patients should be considered on the basis of anatomical factors, pre-injury anticoagulant or antiplatelet agent use, lactate level and SI even if traditional vital signs are normal.  相似文献   

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《Injury》2018,49(1):82-85
BackgroundLarge studies which developed decision rules for the use of Computed tomography (CT) in children with minor head trauma excluded children with late presentation (more than 24 h).ObjectiveTo assess the prevalence of significant traumatic brain injury (TBI) on CT in infants with head trauma presenting to the emergency department (ED) more than 24 h from the injury.MethodsA retrospective chart review of infants less than 24 months old referred for head CT because of traumatic brain injury from January 2004 to December 2014 in Assaf-Harofeh medical center was conducted. We used the PECARN definitions of TBI on CT to define significant CT findings.Results344 cases were analyzed, 68 with late presentation.There was no significant difference in the age between children with late and early presentation (mean 11.4 (SD 5.6) month vs 10. 5 (SD 7.0) month, P = 0.27). There was no significant difference between the groups in the incidence of significant TBI (22% vs 19%, p = 0.61). Any TBI on CT (e.g. fracture) was found in 43 (63%) patients with late presentation compared with 116 (42%) patients with early presentation (p = 0.002, OR 2.37, 95% CI 1.37–4.1).ConclusionA similar rate of CT-identified traumatic brain injury was detected in both groups.‏ There was no significant difference in the incidence of significant TBI on CT between the groups.‏ Young children presenting to the ED more than 24 hours after the injury may have abnormal findings on CT.  相似文献   

6.
BackgroundThere is currently limited information regarding lung clearance index (LCI) and its response to treatment of pulmonary exacerbations in CF. We aimed to examine the utility of LCI for assessing short term clinical response to IV antibiotic therapy in school-age children with CF.MethodsSubjects experiencing exacerbations and hospitalised for IV antibiotics performed both multiple breath nitrogen washout (MBNW) and spirometry on admission to hospital and prior to discharge.Results27 patients (aged 6–20 years) had paired data for MBNW and spirometry. Mean LCI reduced from 12.18 to 11.65 (4.4%) by time of discharge and FEV1 z-score improved from − 3.05 to − 2.86 (6.2%). Overall, LCI improved in n = 15 (55%) patients compared with n = 18 (67%) where FEV1 improved.ConclusionsIn summary, these findings do not support the use of LCI (or indeed, FEV1) to gauge the short term clinical response to IV antibiotic therapy in school-age children with cystic fibrosis.  相似文献   

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BackgroundIn manual therapy, the ‘harmonic’ approach to inducing cyclical motion in different body regions has been described as distinct from rhythmic articulation, ‘harmonic’ techniques being further defined as those that bring about a state of resonance in body tissues. During application of ‘harmonic’ techniques, resonance is proposed to result from the technique operator ‘tuning in’ to the inherent frequency of the oscillating region. This study provides a quantitative analysis of the motion induced by a ‘harmonic’ technique and the notion of ‘tuning in’.ObjectivesTo provide a quantitative analysis of the oscillatory motion induced in the lumbar–pelvic complex by the application of a ‘harmonic’ technique and to compare the motion induced by two different operators.MethodsA ‘harmonic’ technique was applied to the lumbar–pelvic complex of 26 volunteers and the oscillatory motion filmed. An additional operator performed the technique on a subgroup (n = 12) of the sample to provide data for the comparisons of two operators.ResultsThe mean (SD) frequency of oscillation across all sessions was 1.17 Hz (0.10). The mean (SD) amplitude was 0.15 m (0.03). The mean (SD) inter-cycle variability of period was 2.45% (0.47). The mean (SD) inter-cycle variability of amplitude was 6.35% (2.06). There were no consistent intra-cycle relationship identified between period and amplitude. There was an inverse relationship between frequency and subject weight (Pearson's r = −0.66 (95% CI −0.84 to −0.32). The intra-class correlation coefficient (ICC) for inter-operator reliability of frequency was 0.93 (95% CI = 0.83 to 0.97). The ICC for inter-operator reliability of amplitude was 0.87 (95% CI = 0.69 to 0.94).ConclusionThe motion induced in the lumbar–pelvic complex using a modified harmonic technique displayed properties of harmonic motion. The inter-operator study demonstrated that two operators of different morphologies ‘tuned in’ to the same frequency of individual subjects. This supports the theory that motion arising from harmonic techniques is resonant.  相似文献   

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BackgroundData from the Wisconsin newborn screening (NBS) study show that neonatally diagnosed infants are at risk of early Pseudomonas aeruginosa (PsA) acquisition. We have had NBS since 1981 and in 2003, introduced PsA-free ‘segregation’ from older patients for children ≤ 5. This study investigated the effect of simple ‘segregation' on acquisition of respiratory pathogens.MethodsSputum culture results (n = 2814) and details of antibiotic use before (1999–2002) and after (2004–2007) ‘segregation’ were collected.ResultsEach year each child provided an average of 4.6 samples for culture. There was a significant decrease (p  0.001 Chi2) in the acquisition of mucoid (from 5.9% of children to 1.0%) but not non-mucoid PsA (22.3% and 22.7%, respectively) after ‘segregation’. There was no significant change in other respiratory pathogens.ConclusionsYoung children with CF diagnosed via NBS can be protected from the acquisition of mucoid PsA by ‘segregation’ and the acquisition of non-mucoid PsA is likely to be from environmental sources outside the hospital.  相似文献   

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ObjectiveThe aim of this study was to assess the effect of baseline inflammation on Magnetic Resonance Imaging (MRI) on the change in Bone Mineral Density (BMD) over 5 years in patients with early spondyloarthritis (SpA).MethodsFrom the patients of the DESIR cohort (an early axial SpA cohort), patients with BMD data at both baseline and 5 years, and baseline spine and sacroiliac joints MRI were included. Inflammation was assessed with the SpondyloArthritis Research Consortium of Canada (SPARCC) spine score. Significant BMD loss was defined by a change of > 0.03 g/cm2. No patients had received TNF blockers before inclusion in the cohort. Univariate and multivariable prognostic analyses were performed. An inverse propensity score weighting method was used to handle confounders.ResultsOne hundred and eighty-three patients were included (mean age 33.9 ± 8.7 years, 58.5% men). A significant bone loss was reported in 51% (n = 92) of patients at either lumbar spine or hip. Fourteen (7%) patients had low BMD (Z-score < −2) at the end of the follow-up vs. 28 (15%) at baseline. In multivariable analysis, age was a protective factor of 5 year-BMD loss at any site (OR = 0.96, 95% CI [0.93–0.99]). Baseline MRI inflammation has no significant effect on BMD change at any site (OR= 0.84, 95% CI [0.46–1.53]).ConclusionHalf of patients with early SpA have a significant bone loss at either lumbar spine or hip over 5 years. Baseline MRI inflammation is not a determinant of this bone loss.  相似文献   

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BackgroundThe effectiveness of current treatment recommendations for vitamin D insufficiency in children with CF is unknown. Therefore, we assessed the effectiveness of vitamin D2 50,000 IU once daily for 28 days for vitamin D insufficiency.MethodsRetrospective chart review of pediatric CF patients from 2006–2008. Vitamin D2 50,000 IU daily for 28 days was given to patients with 25-OHD < 30 ng/mL and repeat 25-OHD levels were obtained after completion of therapy.ResultsOne hundred forty-seven levels from 97 individuals were assessed. Success of treatment was 54% (n = 80/147). Seventeen of 39 patients (43%) followed for an additional 6–18 months were able to maintain levels of ≥ 30 ng/mL.ConclusionsVitamin D2 50,000 IU daily for 28 days was effective in correcting vitamin D insufficiency in approximately 50% of subjects. However, almost half of successfully treated patients were unable to maintain normal 25-OHD levels > 6 months after completion of therapy, implying that this effect is transient.  相似文献   

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《Cirugía espa?ola》2022,100(5):288-294
IntroductionThe paradoxical benefit of obesity, the ‘obesity paradox’, has been analyzed in lung surgical populations with contradictory results. Our goal was assessing the relationship of body mass index (BMI) to acute outcomes after minimally invasive major pulmonary resections.MethodsRetrospective review of consecutive patients who underwent pulmonary anatomical resection through a minimally invasive approach for the period 2014–2019. Patients were grouped as underweight, normal, overweight and obese type I, II and III. Adjusted odds ratios regarding postoperative complications (overall, respiratory, cardiovascular and surgical morbidity) were produced with their exact 95% confidence intervals. All tests were considered statistically significant at p < 0.05.ResultsAmong 722 patients included in the study, 37.7% had a normal BMI and 61.8% were overweight or obese patients. When compared with that of normal BMI patients, adjusted pulmonary complications were significantly higher in obese type I patients (2.6% vs 10.6%, OR: 4.53 [95%CI: 1.86–12.11]) and obese type II–III (2.6% vs 10%, OR: 6.09 [95%CI: 1.38–26.89]). No significant differences were found regarding overall, cardiovascular or surgical complications among groups.ConclusionsObesity has not favourable effects on early outcomes in patients undergoing minimally invasive anatomical lung resections, since the risk of respiratory complications in patients with BMI  30 kg/m2 and BMI  35 kg/m2 is 4.5 and 6 times higher than that of patients with normal BMI.  相似文献   

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《Injury》2017,48(7):1662-1669
AimsTo evaluate the associations of timing of surgery with postoperative length of stay (LOS), complications, and functional outcomes 3–6 years after open reduction and internal fixation (ORIF) in closed ankle fractures.Patients and methodsHistorical cohort study by chart review of 1011 patients for postoperative LOS and complications; 959 individuals were invited to participate in a postal survey with functional outcomes questionnaires. Complications were classified as perioperative, early, or late. The associations with time from trauma to surgery (<8 h, 8 h to 6 days, >6 days) were assessed with (1) postoperative LOS using multivariable random-effects negative binomial regression, (2) complications using multivariable binary and multinomial logistic regression, and (3) three different functional outcomes using multivariable linear regression.ResultsThe mean patient age was 51.4 (range 18–94) years, 556 (55%) were female, and 567 individuals (59%) responded to the questionnaire. There were no statistically significant associations between time to surgery and either postoperative LOS or complications after adjusting for several patient and fracture characteristics. Patients operated on >6 days after the trauma had significantly worse scores on the Olerud and Molander Ankle Score (OMAS) (p = 0.039) and somewhat worse, but non-significant, scores on the Lower Extremity Functional Scale (LEFS; p = 0.573) and the Self-Reported Foot and Ankle Score (SEFAS) scale (p = 0.161) than those operated on <8 h after trauma.ConclusionIn ankle-fracture surgery, there was no apparent association between timing of surgery and postoperative LOS or complications. A delay of surgery for 8 h to 6 days resulted in similar functional outcomes after 3–6 years suggesting there may be a safe window of time for surgery of up to 6 days after trauma that can be used to plan and perform the final ORIF.  相似文献   

15.
BackgroundPalonosetron is a new, potent, and long-acting 5HT3-receptors antagonist that had been approved by the FDA for use in postoperative nausea and vomiting (PONV) prophylaxis. This study is designed to evaluate its efficacy combined with dexamethasone in PONV prophylaxis in highrisk patients scheduled for laparoscopic surgeries.MethodsIn this double-blind, active-controlled study, 150 patients aged 20–55 years, ASA I–II, and with Apfel’s PONV score 2–4 were equally randomized to receive dexamethasone 8 mg before anesthesia induction and saline 30 min before the end of surgery (group D + S), dexamethasone 8 mg before anesthesia induction and metoclopramide 25 mg 30 min before the end of surgery (group D + M), or dexamethasone 8 mg combined with palonosetron 0.075 mg before anesthesia induction and saline 30 min before the end of surgery (group D + P). Incidences of early and late PONV, complete response, adverse events from antiemetics used, and overall patients’ satisfaction were recorded.ResultsThe incidence of PONV was comparable in the three groups 0–6 h postoperatively. Palonosetron–dexamethasone and dexamethasone–metoclopramide combination therapies significantly reduced the incidence of PONV at 6–12 h postoperatively compared to dexamethasone monotherapy (12% and 16%, vs. 36%, respectively, with P < 0.05). Moreover, palonosetron–dexamethasone combination therapy significantly reduced the incidence of PONV at 12–24 h postoperatively compared to both dexamethasone monotherapy (16% vs. 48%, P < 0.01), and dexamethasone–metoclopramide combination therapy (16% vs. 40%, P < 0.05). The incidence of adverse drug effects was comparable in the three groups. The overall patients’ satisfaction was significantly higher in palonosetron–dexamethasone combination therapy compared to other groups.ConclusionPalonosetron–dexamethasone combination is effective and safe in PONV (early and late) prophylaxis in high-risk patients undergoing laparoscopic surgeries with known high-risk of PONV.  相似文献   

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BackgroundCombination opioid-acetaminophen drugs are commonly used for pain management after cesarean delivery. The aim of this study was to determine if scheduled acetaminophen decreases opioid use compared to as-needed combination acetaminophen-opioid administration.MethodsWe performed a retrospective chart review of women who underwent cesarean delivery before and after a clinical practice change. All patients received spinal anesthesia containing intrathecal morphine 200 μg and scheduled non-steroidal anti-inflammatory drugs for 48 h postoperatively. The first group (As-Needed Group, n=120) received combination oral opioid-acetaminophen analgesics as needed for breakthrough pain. The second group (Scheduled Group, n=120) received oral acetaminophen 650 mg every 6 h for 48 h postoperatively with oral oxycodone administered as needed for breakthrough pain. The primary outcome was opioid use, measured in intravenous morphine mg equivalents, in the first 48 h postoperatively.ResultsThe Scheduled Group used 9.1 ± 2.1 mg (95% CI 5.0–13.2) fewer intravenous morphine equivalents than the As-Needed Group (P <0.0001) over the study period. Fewer patients in the Scheduled Group exceeded acetaminophen 3 g daily compared to the As-Needed Group (P=0.008). Pain scores were similar between study groups.ConclusionsAfter cesarean delivery, scheduled acetaminophen results in decreased opioid use and more consistent acetaminophen intake compared to acetaminophen administered as needed via combination acetaminophen-opioid analgesics, without compromising analgesia.  相似文献   

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ObjectiveTo assess the safety of peripherally inserted central venous catheter (PICC) placement in patients with altered and uncorrected coagulation parameters or receiving antiplatelet therapy.Materials and methodsMedical charts of all patients with major primary and secondary hemostasis disorders, combined hemostasis disorders or on antiplatelet therapy and who had undergone non-tunneled PICC placement from December 2009 to December 2013, were retrospectively reviewed. A hemostatic disorder was defined as a platelet count (PC)  50 × 109/L, an international normalized ratio (INR) ≥ 2, or an activated partial thromboplastin time (aPTT)  66 s, alone or in combination. Underlying hemostasis disorders were not corrected and antiplatelet therapy was not interrupted before PICC placement in any patient. 4, and 5-Fr single and dual lumen PICCs were used.ResultsA total of 378 PICCs were placed in 271 patients (180 men and 91 women; mean age = 62 ± 13.4 years; range, 18  93 years)) with coagulation disorders. Eighty-nine (23%) PICCs were placed in patients who were receiving antiplatelet therapy (aspirin, clopidogrel, rivaroxaban). Thrombocytopenia was noted in 269 PICC placements (71%). Among these patients, 23 had disseminated intravascular coagulation. Prolonged INR and aPTT were observed in 42 procedures (11.1%). PICC placement was achieved in all patients, with a mean number of 1.14 attempts. Peripheral venous access was obtained through the basilic and the brachial vein respectively in 295 (79.1%) and 83 (20.9%) of patients. The placements were performed by residents and fellows in 108 (28.5%) and 270 (71.5%) procedures, respectively. No early or late complications were reported after any procedure. No accidental puncture of the brachial artery occurred.ConclusionIn patients with severe primary and secondary hemostasis disorders, combined hemostasis disorders or on antiplatelet therapy, PICC placement is a feasible and safe procedure and does not require correction of coagulation parameters or discontinuation of antiplatelet therapy.  相似文献   

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《Injury》2016,47(1):19-25
Study objectiveWe sought to (1) define the high-risk elderly trauma patient based on prognostic differences associated with different injury patterns and (2) derive alternative field trauma triage guidelines that mesh with national field triage guidelines to improve identification of high-risk elderly patients.MethodsThis was a retrospective cohort study of injured adults ≥65 years transported by 94 EMS agencies to 122 hospitals in 7 regions from 1/1/2006 through 12/31/2008. We tracked current field triage practices by EMS, patient demographics, out-of-hospital physiology, procedures and mechanism of injury. Outcomes included Injury Severity Score  16 and specific anatomic patterns of serious injury using abbreviated injury scale score ≥3 and surgical interventions. In-hospital mortality was used as a measure of prognosis for different injury patterns.Results33,298 injured elderly patients were transported by EMS, including 4.5% with ISS  16, 4.8% with serious brain injury, 3.4% with serious chest injury, 1.6% with serious abdominal-pelvic injury and 29.2% with serious extremity injury. In-hospital mortality ranged from 18.7% (95% CI 16.7–20.7) for ISS  16 to 2.9% (95% CI 2.6–3.3) for serious extremity injury. The alternative triage guidelines (any positive criterion from the current guidelines, GCS  14 or abnormal vital signs) outperformed current field triage practices for identifying patients with ISS  16: sensitivity (92.1% [95% CI 89.6–94.1%] vs. 75.9% [95% CI 72.3–79.2%]), specificity (41.5% [95% CI 40.6–42.4%] vs. 77.8% [95% CI 77.1–78.5%]). Sensitivity decreased for individual injury patterns, but was higher than current triage practices.ConclusionsHigh-risk elderly trauma patients can be defined by ISS  16 or specific non-extremity injury patterns. The field triage guidelines could be improved to better identify high-risk elderly trauma patients by EMS, with a reduction in triage specificity.  相似文献   

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《Injury》2016,47(9):1919-1923
IntroductionThere is a translational gap between physicians who document in the medical record and coders, who ultimately determine which codes are submitted. This gap exists because physicians are never formally educated about documentation strategies despite the fact that the quality of physician documentation directly affects revenue, outcomes and public profiling. We evaluated the effect of a formal model of focused documentation improvement (FDI) on the trauma/critical care division. We hypothesized that FDI would improve physician documentation, resulting in revenue recovery and a shift in the case mix index (CMI) to more accurately reflect the clinical complexity of trauma patients.MethodsFDI is defined as targeted physician education followed by concurrent inpatient chart review for documentation improvement opportunities by a clinical documentation specialist (CDS). All trauma surgeons (n = 9) at our Level 1 trauma center first completed three hours of mandatory training on documentation improvement. A CDS was subsequently assigned to the trauma service. They reviewed the charts of Medicare patients (n = 776) from January–December 2014 to identify opportunities for documentation improvement, participated in ICU rounds and provided ongoing education. Requests to clarify documentation (queries) were posted in the electronic medical record (EMR) and physicians were required to respond within 48 h. Data was collected on physician response rate, CMI and revenue recovery.Results411 of 776 (57%) charts were reviewed. Opportunities for FDI were identified in 177 (43%) cases. The physician response rate to queries was 100%. The CMI for reviewed cases increased (1.80 (SD 0.15) vs. 2.11 (SD 0.19); p < 0.001) after FDI. Overall revenue recovery was $1,132,581 with an average of $154,092 in revenue recovery/clinical full time equivalent. The total cost for administration of FDI was $353,265 resulting in a 220% return on investment (ROI).ConclusionFDI is an effective strategy to engage physicians in documentation improvement. It provides an infrastructure to assist physicians and yields a significant ROI.  相似文献   

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IntroductionCystic fibrosis (CF) is the most common genetic disorder in Caucasians. Presentation of CF in non-Caucasians is less well studied.ObjectiveThis audit was undertaken to determine the phenotypic expression of the 3120 + 1G > A mutation in black and mixed race children in South Africa.MethodsA multi-centre retrospective chart review of clinical, laboratory and spirometry data of non-Caucasian CF patients in four CF centres in South Africa was collected. Data was collected at diagnosis and after a five-year follow-up period. Ethical approval was granted for the study.ResultsA total of 30 participants were enrolled of whom 14 (47%) were homozygous and 16 (53%) heterozygous for the 3120 + 1G > A mutation. The mean age of diagnosis was 13 months. Twenty-four (80%) patients had malnutrition (mean weight z-score ? 3.6) or failure to thrive (77%) at presentation. Twenty (67%) presented with non-specific abdominal symptoms, whilst fifteen (50%) had recurrent respiratory tract infections. Pseudomonas aeruginosa was detected at a mean age of 21 months. The mean FEV1 was 73% predicted (95% CI 54.0–91.1) at study entry and 68% predicted (95% CI 49.74–87.06) at follow-up.ConclusionFailure to thrive and a diagnosis of protein energy malnutrition (kwashiorkor) are the common presenting features of CF in children with the 3120 + 1G > A mutation. Meconium ileus is a rare presenting feature of CF in black and mixed race children with this deletion in South Africa.  相似文献   

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