bluuebunny/crossref_metadata_2025_split huggingface dataset:
Implementation and Assessment of a SmartZone Alert to Notify Clinicians of Critical Hyperbilirubinemia in Preterm Infants Less Than 35 Weeks Gestation
Authors: Xiao Yi , Palmucci Mandy , Carlin Lance , Lee Catherine , O'Gorman Maurice , Nair Srikumar , Yieh Leah , Leung Edward 
Published: November 2022

Abstract

Background
Neonatal jaundice, also known as hyperbilirubinemia in term and preterm infants, is treated with phototherapy when bilirubin results exceed gestational age- and age-specific medical decision levels (MDL) to prevent kernicterus and bilirubin-induced neurological damage. During phototherapy, unconjugated bilirubin is converted to water-soluble isomers that are excreted in the urine. Presently, the electronic medical record (EMR) at our hospital cannot use gestational age to stratify reference ranges thereby the associated flags and alerts would not be triggered, leading to delays in reviewing bilirubin results and placing phototherapy orders. The aim of this project is to replace the current manual assessment process for phototherapy with a newly designed alert to notify clinicians of elevated bilirubin results for preterm infants (&lt;35 weeks gestation) and to minimize prolonged delays in placing phototherapy orders. We hypothesized that the SmartZone alert will encourage consistent and timely consideration of phototherapy.

Method
A SmartZone alert, built with Cerner command language and Discern Expert rules, evaluates total or neonatal bilirubin results for patients in the Newborn and Infant Critical Care Unit (NICCU), calculates the patient’s age using the recorded gestational age and birth time, and utilizes the following rules: 1) bilirubin result must exceed the pre-defined gestational age-specific MDLs, and 2) calculated gestational age is &lt;35 weeks. Additionally, the SmartZone alert will not be triggered if there is an active phototherapy order. Once triggered, the alert will be visible for four hours to all caregivers who view the patient’s EMR. We compared the duration of time between verified bilirubin results and phototherapy orders before and after the implementation of the SmartZone alert.

Result
The SmartZone alert was implemented on 01/11/2022. Between 1/1/2021 - 1/11/2022, 95 preterm infants in the NICCU had neonatal and total bilirubin results, in which 19 met the criteria for the alert to be triggered, and 17 had phototherapy orders. Further analysis revealed that 12 orders were placed &lt;24 hours, four orders were placed 24 - 72 hours, and one order was placed &gt;72 hours after bilirubin results were verified. Between 1/12/2022 – 2/13/2022, 13 newly admitted preterm infants in the NICCU had neonatal and total bilirubin results, in which one triggered the alert, and phototherapy was ordered within 15 hours.

Conclusion
A SmartZone alert was implemented to encourage consistent and timely consideration of phototherapy for preterm infants &lt;35 weeks with critical hyperbilirubinemia. The alert is functioning as intended. This alert will be continuously monitored to further optimize its functionality and utilization.
Development of a Novel Algorithm Using Red Blood Cell Indices for Screening of Alpha Globin Gene Deletions
Authors: Zhang Xi , Parikh Bijal 
Published: November 2022

Abstract
Distinguishing between different causes of microcytic anemia is essential for management of anemic patients. More than 20 different RBC indices have been used to screen thalassemia trait with inconsistent results. Differentiation between iron deficiency anemia (IDA) and alpha-thalassemia appears to be more challenging than differentiation between IDA and beta-thalassemia, based on RBC indices alone. Moreover, thalassemia and IDA may coexist. A highly sensitive screening algorithm is needed to select clinical cases for alpha globin gene analysis to reduce unnecessary testing and improve pre-test probabilities. A retrospective study was performed to evaluate the reliability of 22 RBC indices (RBC, MCV, RDW, MCH, Mentzer Index, RDW Index, etc) and formulae in differentiating cases with 1-2 alpha globin gene deletions from negative cases, in a cohort of 90 individuals with confirmatory alpha globin gene analysis results. The number of patients with each alpha globin gene deletion pattern were: 37 patients with alpha-thalassemia trait, 21 silent carriers (one gene deletion), 31 negative patients and 1 patient with triplicated alpha globin gene. The mean RBC count in each group were 4.79 +/- 0.65 (trait), 4.45 +/- 0.73 (carrier), and 4.21 +/- 0.84 (negative) (million/mm). The mean MCV in each group were 71.1 +/- 4.8 (trait), 76.1 +/- 5.9 (carrier), and 74.2 +/- 9.3 (negative) (fl). The mean RDW in each group were 16.5 +/- 3.0 (trait), 17.2 +/- 3.4 (carrier), and 18.3 +/- 4.9 (negative) (%). The mean MCH in each group were 22.0 +/- 1.8 (trait), 23.8 +/- 2.3 (carrier), and 23.5 +/- 3.7 (negative) (pg). None of the RBC indices showed significant difference between carrier and negative groups, while 20 RBC indices showed significant difference (p&lt;0.05) between trait and negative groups. The sensitivities of the RBC indices in detecting 1-2 alpha globin gene deletions based on published cutoffs ranged between 3.4% and 81.0%, while the specificities ranged between 41.9% and 100%. A novel scoring algorithm based on the combination of 12 RBC indices and formulae achieved a sensitivity of 96.6% in detecting 1-2 alpha globin gene deletions, with a negative likelihood ratio of 0.15. The negative likelihood ratio of the scoring algorithm outperformed each of the 12 RBC indices at similar levels of sensitivities (&gt;95%) based on revised cutoffs. The algorithm is a highly sensitive tool for screening of alpha globin gene deletions, including one gene deletion. It is more effective at identifying negative cases compared to individual RBC indices. Implementation of the algorithm could efficiently reduce 10.0% (9/90) of the test volume of alpha globin gene analysis. A more stringent version of the algorithm could achieve 100% sensitivity and reduce 5.6% (5/90) of the alpha globin gene analysis test volume.
Benign Multicystic Peritoneal Mesothelioma as a Rare Cause of Death
Authors: Webber M , Connor E , Singh A , Majari G , moroz K , craig R 
Published: November 2022

Abstract

Introduction/Objective
Benign multicystic peritoneal mesothelioma (BMPM) is a tumor arising from the serosa of the peritoneal cavity, often in the pelvis, and is comprised of multiple cysts lined by bland mesothelial cells. It is a rare entity with less than 200 cases reported. The clinical course is often indolent with nonspecific symptoms. It has a strong predilection for females (90%) and is commonly associated with endometriosis or prior surgeries. A high local recurrence rate (50%) following excision has been reported, but malignant transformation is rare.

Methods/Case Report
The decedent was a 53-year-old male with a history of multiple hernia repairs who presented following three days of diffuse abdominal pain and obstipation. Imaging showed large volume, complex ascites with multiple rim-enhancing fluid collections, diffuse peritoneal thickening, and a soft tissue density in the lower abdomen/pelvis. Exploratory laparotomy was congruent and revealed 2L of purulent ascites, numerous peritoneal cystic lesions, and a bulky tumor in the pelvis. Blood and ascites cultures later grew Bacteroides species, and Escerichia coli was also isolated from the blood. Postoperatively, the decedent continued to decline, and death occurred the following day. Autopsy demonstrated that the firm pelvic mass entrapped the bladder, rectum, and segments of the small intestine. Sectioning revealed numerous cystic spaces of varying size. Microscopically, they were lined by a single layer of benign cells with mild to focally moderate atypia. These cells stained positive for Calretinin, Pan-cytokeratin, and D2-40 and negative for CD31, which is characteristic of mesothelial cells. These findings are consistent with BMPM.

Results (if a Case Study enter NA)
NA.

Conclusion
The decedent’s death is the result of sepsis following acute peritonitis due to BMPM. Death resulting from this entity is exceedingly rare, and to our knowledge this case is only the second reported death due to BMPM.
Hemorrhagic Pancreatitis in Pediatric Heart Transplant Recipients
Authors: Schwietert M , Sanabria Nunez D , Petty D 
Published: November 2022

Abstract

Introduction/Objective
Acute pancreatitis is a known side effect of tacrolimus in solid organ transplant recipients, but can be difficult to detect as symptoms may be vague and poorly localized, or overlap with the underlying condition. This phenomenon has most frequently been described in adult kidney transplant recipients. We present two autopsy cases in which pediatric patients with history of orthotopic heart transplantation both developed hemorrhagic pancreatitis shortly following operative procedures. Both had received immunosuppressants including tacrolimus during therapy. Although the etiology of pancreatitis in these medically complex cases is likely multifactorial, tacrolimus may be an important contributor.

Methods/Case Report
Case 1: A 6-year-old male with hypoplastic left heart syndrome was admitted for two years for medical management while awaiting transplant. Prior to and following transplantation he received immunosuppressants including tacrolimus to reduce risk of transplant rejection. Two weeks post-operatively, he developed abdominal pain and elevated pancreatic enzymes, leading to a diagnosis of pancreatitis. Imaging revealed hemorrhage. Tacrolimus was suspected to have caused this, and was discontinued. Despite medical management and multiple attempts to embolize vessels around the pancreas, the decedent remained unstable and ultimately went into hypotensive bradycardic cardiac arrest. Autopsy revealed a large hemorrhagic cavity confluent with a necrotic pancreas, as well as coagulum adherent to bowel loops throughout the abdomen. Case 2: A 13-year-old male status post two orthotopic heart transplants presented for a dental procedure. Following this, he developed an anaphylactic-type reaction to medication, with hives and bradycardia followed by cardiac arrest. He was resuscitated, however, his cardiac function steadily declined throughout the remainder of his stay. Heart biopsy revealed no evidence of cell-mediated transplant rejection. Pancreatic enzymes were mildly elevated, and abdominal imaging was unremarkable. His tacrolimus levels were noted to be supratherapeutic, and this was discontinued. Shortly before passing, he developed severe abdominal pain with distension, nausea and vomiting. At autopsy, he was found to have nearly two liters of hemorrhage and clot within the peritoneum emanating from a necrotic and hemorrhagic pancreas.

Results (if a Case Study enter NA)
NA.

Conclusion
Close clinical monitoring of pancreatic function should be considered in solid organ transplant recipients receiving tacrolimus.
Histologic Features of a Solitary Fibrous Tumor in the Region of Oral Cavity and Maxillofacial Complex with Recurrence and Distant Metastasis: a Rare Case Report
Authors: Johnson A , Eltahir M , Xu Y 
Published: November 2022

Abstract

Introduction/Objective
Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm, and may involve many anatomic locations. Approximately 0.6% of SFTs in the oral cavity and maxillofacial complex may recur and/or develop distant metastasis. Here we report a case of SFT in oral cavity with local recurrence and distant metastasis.

Methods/Case Report
The patient was a 66-year-old male with a past medical history of left buccal mass status post excision with an unclear diagnosis in a foreign country 6 years ago. He presented with a recurrence of the left buccal mass. Computerized tomography (CT) of the head and chest demonstrated a 9 cm expansile mass centered in the left pterygopalatine fossa and a 1.7 cm mass in left upper lobe of the lung, respectively. Biopsy of the lung mass was performed first and displayed a hypercellular mesenchymal tumor, composed of spindle cells arranged in vague short fascicles, and scant fibrous stroma with thin-walled and staghorn-like vasculature. The tumor cells had mild nuclear atypia with 5 mitoses/10 high power fields. No necrosis is identified. There were trapped benign pneumocytes and bronchial epithelial cells in the tumor. The tumor is positive for CD34, CD99, Bcl-2 and STAT6 immunostains. The large left buccal mass was biopsied later and showed similar morphology. The diagnoses of metastatic SFT in the lung and recurrent SFT were rendered, respectively.

Results (if a Case Study enter NA)
NA.

Conclusion
The histologic findings of the recurrent SFT and the pulmonary metastasis are similar in our case. Although marked cellular atypia and necrosis are not observed in either tumor, other features including the large size of recurrent tumor, hypercellularity, and increased mitotic activity are associated with malignant behavior in this report, and warrant the need for long-term follow-up.
Giant Cell Tumor of Soft Tissue Arising from Breast with Distant Metastases Post-therapy: A Rare Entity
Authors: Platero Portillo T M , Benzi E , Dhamne S 
Published: November 2022

Abstract

Introduction/Objective
Giant-cell tumor of soft tissue (GCT-ST) is rare and indistinguishable from giant cell tumor of bone. GCT-ST arising in the soft tissue of breast is exceedingly rare, with only ten cases previously reported.

Methods/Case Report
We present a 46-year-old female with history of lupus, presenting with an enlarging mass in her left breast. Needle core biopsy showed a hypercellular proliferation of spindle cells arranged in fascicles and sheets with infiltrative borders. A primary diagnosis of high-grade spindle cell lesion was rendered. The patient’s clinical workup was negative for lesions elsewhere. The patient subsequently underwent a left segmental mastectomy. Histopathology revealed a tumor composed predominantly of high-grade spindled and epithelioid cells with tumor necrosis, a high mitotic rate, and several scattered multinucleated giant cells. Immunohistochemical stains showed positivity for CD68 and CD34 in giant cells only, while the tumor cells were positive for Vimentin and CD99. Pancytokeratin, cytokeratin 5/6, HMB-45, smooth muscle actin, myogenin, myo-D1, and desmin were all negative. Based on the morphology and immunohistochemistry, the tumor was diagnosed as a malignant GCT-ST. Adjuvantly, she underwent chemoradiation; however, within four months, she developed new nodules in the lung, left ventricle, and liver, radiologically consistent with metastases.

Results (if a Case Study enter NA)
NA

Conclusion
Although GCT-ST possesses a low malignant potential, its unpredictable behavior poses a challenge. Metastases may develop even after excision with adequate surgical margins. To our knowledge, this is the first case of primary malignant GCT-ST of the breast with distant metastases post-therapy. This entity, with its clinical complexities, remains a diagnostic and therapeutic challenge.
Hyperglycemia Alters the CD27:CD70 Axis of Human Immune Cells
Authors: Lerret N , Owaja C , Schulz M , Roback-Navarro E 
Published: November 2022

Abstract

Introduction/Objective
The exact mechanism that inflammation plays in the pathogenesis from obesity to Type 2 diabetes is unclear, however, activated immune cells and pro-inflammatory cytokines have been found in the adipose tissue of Type 2 diabetics, implicating their role in the disease process. While the CD27-CD70 axis is being explored in other models of chronic inflammation, such as rheumatoid arthritis and colitis, the role played in Type 2 diabetes remains unknown.

Objective
Evaluate whether hyperglycemia alters immune cells phenotype and effector function, specifically of the CD27:CD70 axis on human immune cells.

Methods/Case Report
Human peripheral blood from donors was divided into three groups. n=40 total those that have a healthy blood glucose value and are without any co-morbidities (normoglycemic), patients identified as having pre-diabetes per their hemoglobin A1c (HgbA1c) value (5.6-6.5%), and patients with diabetes, having an elevated blood glucose and HgbA1c value (&gt; 6.5%). The blood was then stained with monoclonal antibodies towards cell surface markers associated with inflammation, including CD27/CD70 and analyzed via flow cytometry. Previous data using cell cultures of human PBMC-derived T cells and autologous dendritic cells exposed to varing concentrations of glucose to simulate hyperglycemia and pre-diabetes was compared to this data.

Results (if a Case Study enter NA)
1) T cells directly analyzed from pre-diabetic and hyperglycemia individuals down-regulate their CD27 expression. CD70 is up-regulated on immune cells directly analyzed from pre-diabetic individual when compared to immune cells from normoglycemic individuals. In accordance with the above resutls, CD4 T cells have an activated effector phenotype after co-culture with glucose stimulated dendritic cells. This includes down-regulation of CD27 on T cells and up-regulation of CD70 on dendritic cells.

Conclusion
Our data shows that certain markers of inflammation are up-regulated on the surface of immune cells from pre-diabetic and/or diabetic patients. Specifically, we demonstrate novel evidence that the CD27-CD70 axis is activated in diabetes or hyperglycemic conditions. These molecules may offer a potential target for therapeutics. Alternatively, our findings would allow a further way to characterize where a patient with an elevated blood glucose value is on the spectrum between pre-diabetes and full-blown diabetes.
Performance Evaluation of Two FDA-Approved Fentanyl Immunoassays against LC-MS/MS
Authors: Militello L , Zhang V 
Published: November 2022

Abstract

Introduction/Objective
The increased number of deaths caused by Fentanyl in recent years created the need for accurate, automated assays for fentanyl detection. Many products have been brought to market but there is concern over the sensitivity and specificity of the available immunoassays. Here we evaluate the performance of our current assay, the ARK Fentanyl II Assay (ARK), against the newly FDA-approved Fentanyl Immunoassay for Roche Cobas (Roche), using liquid chromatography-tandem mass spectrometry (LC-MS/MS) as the gold standard. To our knowledge, this is the first study to evaluate the performance of the Roche fentanyl immunoassay.

Methods/Case Report
We collected discarded patient urine samples screened for fentanyl using ARK over 3 months to get a total of 100 positive and 100 negative results. All samples were stored at -20 °C and later tested again using ARK, Roche and in-house LC-MS/MS assays. Both immunoassays were performed on Roche Cobas c501 chemistry analyzer. In-house LC-MS/MS assay gave quantitative measurements for both fentanyl and norfentanyl with a limit of quantification of 1 ng/mL for both. Samples with results above 1 ng/mL of either analyte were considered positive for the presence of fentanyl. The upper limit of quantification of the LC-MS/MS assay was 2000 ng/mL. The results obtained from LC-MS/MS were considered as reference values to determine the sensitivity and specificity of the immunoassays, which were calculated by dividing the number of positive and negative samples correctly identified by immunoassay by total positive and negative samples based on LC-MS/MS results, respectively. ARK is designed to detect fentanyl with a cutoff of 1 ng/mL. Roche is designed to detect norfentanyl with a cutoff of 5 ng/mL.

Results (if a Case Study enter NA)
Analytical sensitivity and specificity were 91.9% and 99.3% for ARK and 96.8 and 98.6% for Roche. Fentanyl and norfentanyl levels by LC-MS/MS ranged from &lt;1 to &gt;2000 ng/mL with a mean of 57 and 232 and standard deviation of 235 and 578, respectively. False positives were seen in 1 sample for ARK and 2 samples for Roche. False negatives were seen in 5 samples for ARK and 2 samples for Roche. Roche accurately identified 6 positive samples that had norfentanyl concentrations less than the cutoff of 5 ng/mL, indicating a potential greater sensitivity than stated.

Conclusion
Both immunoassays performed reasonably well, showing great specificity. Roche displayed greater sensitivity.
Extramedullary Hematopoiesis Identified in Pleural Fluid of a Patient with Bilateral Pleural Effusions and Associated Myelofibrosis
Authors: Marshall C , Liu Y 
Published: November 2022

Abstract

Introduction/Objective
Extramedullary hematopoiesis (EMH) is the formation of blood cells outside of the bone marrow medullary spaces. EMH is typically seen in the spleen or liver and in association with bone marrow replacement disorders, hemoglobinopathies, and other hematological disorders. Rarely EMH can occur in the pleural cavity of patients resulting in subsequent pleural effusions. These cases can accurately be diagnosed with identification of megakaryocytes and other hematopoietic elements in the pleural fluid.

Methods/Case Report
We present a 67 year old male with a past medical history of pericardial effusion, Jak 2 positive Myelofibrosis (Post PV/ET), and type II diabetes mellitus, who presented with complaint of shortness of breath for 10 days. The patient did not require oxygen at home and denied any cough or hemoptysis. In the emergency department a Chest X ray was ordered which showed bilateral pulmonary effusions. These effusions were attributed to worsening congestive heart failure, and diuretic therapy was started. With no resolution of the pleural effusions, the decision was made to conduct a thoracentesis and remove the fluid. Interventional radiology then performed a thoracentesis removing 1000 mL from each the left and right pleural spaces. Cytological preparation of the fluid demonstrated myeloid and erythroid precursors as well as occasional megakaryocytes. The diagnosis of extramedullary hematopoiesis was made and the effusion did not recur.

Results (if a Case Study enter NA)
NA.

Conclusion
Extramedullary hematopoiesis of the pleural space is rare. However it must be considered in the differential diagnosis of a patient with persistent effusions and concomitant bone marrow replacement disease such as myelofibrosis. Prompt removal and cytological evaluation of the pleura fluid for hematopoietic elements should be performed in order to diagnose and manage the patient most effectively.