Citations

572 Background: Renal medullary carcinoma (RMC) is an aggressive malignancy affecting predominantly young African Americans with sickle cell trait (SCT) or disease (SCD), while a pathologically similar collecting duct carcinoma (CDC) affects patients without sickle cell trait. Clinical responses to chemotherapy and IL-2 in RMC/CDC are poor and novel therapies are needed. Methods: 9 patients with RMC (ages 13-58 y. o., all male) and 15 patients (ages 26-74 y. o., M:F = 13:2) with collecting duct carcinoma (CDC) were studied. Expression of PD-L1 was evaluated with 2 monoclonal antibodies (SP142 and SP263) and tumor infiltrating lymphocytes (TIL) were evaluated for PD1 expression (MRQ-22 antibody) using immunohistochemistry (IHC). Additional studies included ALK protein expression (D5F3 antibody), gene translocation (break apart FISH), next generation sequencing (NGS), and microsatellite instability (MSI). Results: Cancer cell PD-L1 expression above the threshold ( ≥ 2+, ≥ 5%) was seen in 7/9 RMC and 5/13 CDC cases. Concordance between 2 PD-L1 antibodies was 94.4%. PD-1+ TIL were absent in 6/18 cases and variably present in 12/18 cases (from 1 to > 15 TIL/40x power field). No MSI was detected in any of the cases tested (0/6). No case expressed ALK protein, but one case of CDC showed ALK gene re-arrangement. Mutations were identified in SMARCB1, FH, TP53 (3x), ATM, BRCA2, CHEK2 (2x), NF2 (3x), SETD2, and CDKN2A. No mutations in VHL or KDR were detected. One patient with RMC (and SCT) achieved complete clinical remission after treatment with bevacizumab plus paclitaxel. Conclusions: RMC and CDC strongly express PD-L1 in the majority of cases (12/22), suggesting that these patients may benefit from targeting the PD-L1/PD1 interaction. The absence of MSI in these cancers indicates a different mechanism of PD-L1 upregulation from colorectal carcinomas. Consistent with our previous study that showed frequent activation of (pseudo)hypoxia-induced pathways in RMCs (Human Pathology 2011;42:1979), we describe a case of RMC successfully treated with anti-VEGF therapy.

Objective: No risk prediction model is currently available to measure patient’s probability for readmission to the pediatric intensive care unit (PICU). This retrospective case–control study was designed to assess the applicability of an adult risk prediction score (Stability and Workload Index for Transfer [SWIFT]) and to create a pediatric version (PRediction Of PICU Early Readmissions [PROPER]). Design: Eighty-six unplanned early (<48 hours) PICU readmissions from January 07, 2007, to June 30, 2014, were compared with 170 random controls. Patient- and disease-specific data and PICU workload factors were compared across the 2 groups. Factors statistically significant on multivariate analysis were included in the creation of the risk prediction model. The SWIFT scores were calculated for cases and controls and compared for validation. Results: Readmitted patients were younger, weighed less, and were more likely to be admitted from the emergency department. There were no differences in gender, race, or admission Pediatric Index of Mortality scores. A higher proportion of patients in the readmission group had a Pediatric Cerebral Performance Category in the moderate to severe disability category. Cases and controls did not differ with respect to staff workload at discharge or discharge day of the week; there was a much higher proportion of patients on supplemental oxygen in the readmission group. Only 2 of 5 categories in the SWIFT model were significantly different, and although the median SWIFT score was significantly higher in the readmissions group, the model discriminated poorly between cases and controls (area under the curve: 0.613). A 7-category PROPER score was created based on a multiple logistic regression model. Sensitivity of this model (score ≥12) for the detection of readmission was 81% with a positive predictive value of 0.50. Conclusion: We have created a preliminary model for predicting patients at risk of early readmissions to the PICU from the hospital floor. The SWIFT score is not applicable for predicting the risk for pediatric population.

BACKGROUND AND OBJECTIVES: Ineffective and inefficient patient transfer processes can increase the chance of medical errors. Improvements in such processes are high-priority local institutional and national patient safety goals. At our institution, nonintubated postoperative pediatric patients are first admitted to the postanesthesia care unit before transfer to the PICU. This quality improvement project was designed to improve the patient transfer process from the operating room (OR) to the PICU. METHODS: After direct observation of the baseline process, we introduced a structured, direct OR-PICU transfer process for orthopedic spinal fusion patients. We performed value stream mapping of the process to determine error-prone and inefficient areas. We evaluated primary outcome measures of handoff error reduction and the overall efficiency of patient transfer process time. Staff satisfaction was evaluated as a counterbalance measure. RESULTS: With the introduction of the new direct OR-PICU patient transfer process, the handoff communication error rate improved from 1.9 to 0.3 errors per patient handoff (P = .002). Inefficiency (patient wait time and non–value-creating activity) was reduced from 90 to 32 minutes. Handoff content was improved with fewer information omissions (P &lt; .001). Staff satisfaction significantly improved among nearly all PICU providers. CONCLUSIONS: By using quality improvement methodology to design and implement a new direct OR-PICU transfer process with a structured multidisciplinary verbal handoff, we achieved sustained improvements in patient safety and efficiency. Handoff communication was enhanced, with fewer errors and content omissions. The new process improved efficiency, with high staff satisfaction.

Background— Current guidelines recommend surgical revascularization (coronary artery bypass graft [CABG]) over percutaneous coronary intervention (PCI) in patients with diabetes mellitus and multivessel coronary artery disease. Few data are available describing revascularization patterns among these patients in the setting of non–ST-segment–elevation myocardial infarction. Methods and Results— Using Acute Coronary Treatment and Intervention Outcomes Network Registry–Get with the Guidelines (ACTION Registry–GWTG), we compared the in-hospital use of different revascularization strategies (PCI versus CABG versus no revascularization) in diabetes mellitus patients with non–ST-segment–elevation myocardial infarction who had angiography, demonstrating multivessel coronary artery disease between July 2008 and December 2014. Factors associated with use of CABG versus PCI were identified using logistic multivariable regression analyses. A total of 29 769 patients from 539 hospitals were included in the study, of which 10 852 (36.4%) were treated with CABG, 13 760 (46.2%) were treated with PCI, and 5157 (17.3%) were treated without revascularization. The overall use of revascularization increased over the study period with an increase in the proportion undergoing PCI (45% to 48.9%; P trend =0.0002) and no change in the proportion undergoing CABG (36.1% to 34.7%; p trend =0.88). There was significant variability between participating hospitals in the use of PCI and CABG (range: 22%–100%; 0%–78%, respectively; P value <0.0001 for both). Patient-level, but not hospital-level, characteristics were statistically associated with the use of PCI versus CABG, including anatomic severity of the disease, early treatment of adenosine diphosphate receptor antagonists at presentation, older age, female sex, and history of heart failure. Conclusions— Among patients with diabetes mellitus and multivessel coronary artery disease presenting with non–ST-segment–elevation myocardial infarction, only one third undergo CABG during the index admission. Furthermore, the use of PCI, but not CABG, increased modestly over the past 6 years.

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease characterized by infiltration of the lung, pleura and mediastinum with thin-walled lymphangiomas. DPL can result in mass effect from infiltrative disease, restrictive and obstructive pulmonary physiology, chylous effusions and respiratory failure. The present article discusses clinical, radiographic and pathological features, and treatment options for DPL.

Consensus diagnostic recommendations to distinguish GORD from eosinophilic oesophagitis (EoE) by response to a trial of proton pump inhibitors (PPIs) unexpectedly uncovered an entity called ‘PPI-responsive oesophageal eosinophilia’ (PPI-REE). PPI-REE refers to patients with clinical and histological features of EoE that remit with PPI treatment. Recent and evolving evidence, mostly from adults, shows that patients with PPI-REE and patients with EoE at baseline are clinically, endoscopically and histologically indistinguishable and have a significant overlap in terms of features of Th2 immune-mediated inflammation and gene expression. Furthermore, PPI therapy restores oesophageal mucosal integrity, reduces Th2 inflammation and reverses the abnormal gene expression signature in patients with PPI-REE, similar to the effects of topical steroids in patients with EoE. Additionally, recent series have reported that patients with EoE responsive to diet/topical steroids may also achieve remission on PPI therapy. This mounting evidence supports the concept that PPI-REE represents a continuum of the same immunological mechanisms that underlie EoE. Accordingly, it seems counterintuitive to differentiate PPI-REE from EoE based on a differential response to PPI therapy when their phenotypic, molecular, mechanistic and therapeutic features cannot be reliably distinguished. For patients with symptoms and histological features of EoE, it is reasonable to consider PPI therapy not as a diagnostic test, but as a therapeutic agent. Due to its safety profile, ease of administration and high response rates (up to 50%), PPI can be considered a first-line treatment before diet and topical steroids. The reasons why some patients with EoE respond to PPI, while others do not, remain to be elucidated.