Cerebral revascularization surgery reduces cerebrovascular events in children with sickle cell disease and moyamoya syndrome: Results of the stroke in sickle cell revascularization surgery retrospective study.

BACKGROUND: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS). METHODS: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks. Multivariable logistic regression was used to compare CVE occurrence and multivariable Poisson regression was used to compare incidence rates between groups. Covariates in multivariable models included age at treatment start, age at moyamoya diagnosis, antiplatelet use, CVE history, and the risk period length. RESULTS: We identified 141 patients with SCD-MMS, 78 (55.3%) in the surgery group and 63 (44.7%) in the conservative group. Compared with the conservative group, preoperatively the surgery group had a younger age at moyamoya diagnosis, worse baseline modified Rankin scale scores, and increased prevalence of CVEs. Despite more severe pretreatment disease, the surgery group had reduced odds of new CVEs after surgery (odds ratio = 0.27, 95% confidence interval [CI] = 0.08-0.94, p = .040). Furthermore, comparing surgery group patients during presurgical versus postsurgical periods, CVEs odds were significantly reduced after surgery (odds ratio = 0.22, 95% CI = 0.08-0.58, p = .002). CONCLUSIONS: When added to conservative management, cerebral revascularization surgery appears to reduce the risk of CVEs in patients with SCD-MMS. A prospective study will be needed to validate these findings.

Impacts of utilizing swine lagoon sludge as a composting ingredient.

Lagoon sludge, a byproduct of swine operations in the Southeast United States, poses a management challenge due to its high mineral and metal content. Composting is a low-cost, scalable technology for manure management. However, limited information is available on composting swine lagoon sludge in terms of recipes, greenhouse gas emissions and end-product quality. Moreover, due to its high Zn and Cu content, high inclusion of sludge in composting recipes can potentially inhibit the process. To address these knowledge gaps, in-vessel aerated composting (0.4 m3each) was carried out to evaluate impacts of sludge inclusion, at 10% (Low Sludge, LS-Recipe) and 20% (High sludge, HS-Recipe) wet mass-basis, on composting process and end-product quality. Comparable maximum temperatures (74 ± 2.7 °C, 74.9 ± 2.9 °C), and organic matter loss were observed in both recipes. Similarly, sludge inclusion ratio had no significant impact on cumulative GHG emissions. The global warming potential (20-year GWP) for swine lagoon sludge composting using LS and HS recipes was observed to be 241.9 (±13.3) and 229.9 (±8.7) kg CO2-e/tDM respectively. Both recipes lost 24-28% of initial carbon (C) and 4-15% of nitrogen (N) respectively. Composting and curing did not change water-extractable (WE) phosphorus (P) concentrations while WE Zn and Cu concentrations decreased by 67-74% and 55-59% respectively in both recipes. End compost was stable (respiration rates <2 mgCO2-C/g OM/day) with germination index >93 for both recipes.

A proposed algorithm for evaluation and management of pediatric hemophilia patients who present to the emergency department with head trauma.

Hemophilia is the deficiency of plasma clotting factor VIII (hemophilia A) or IX (hemophilia B) where management focuses on the prevention and treatment of acute bleeding symptoms and their sequelae. The most concerning risk is for life-threatening bleeding, including intracranial hemorrhage (ICH), which is caused by head trauma. Guidelines exist for the evaluation and management of pediatric head trauma, including the Pediatric Emergency Care Applied Research Network (PECARN) protocol, but limited evidence exists for when hemophilia patients present to the emergency department (ED), specifically with head trauma. Literature is limited regarding ICH and hemophilia, which further supports the culture of uncertainty among providers. The objective of this study is to conduct a retrospective chart review to determine the prevalence and clinical characteristics of ICH, and to describe computed tomography (CT) scan use in hemophilia patients who present to Phoenix Children's Hospital (PCH) ED with head trauma from January 1, 2007 to June 1, 2019. A total of 89 ED visits and 43 patients met inclusion criteria, and prevalence of ICH was determined to be 4% with the patients presenting with varied clinical characteristics and few commonalities. Using these data, we propose a new algorithm to aid clinicians in determining the need for CT scan in pediatric hemophilia patients who present to the ED with head trauma.

Safety and Efficacy of Robotic-Assisted PCI.

PURPOSE OF REVIEW: Robotics has been used in multiple areas of procedural medical intervention. Robotic percutaneous coronary intervention (PCI) has been available since 2004. Its adoption has been slow with initial application in simple cases. RECENT FINDINGS: With increasing adoption, robotic PCI has been applied to a broader variety of coronary substrates with demonstration of safety and efficacy. Improvements in the robotic console with future generation devices should add to the utility of this platform. Robotic PCI advances the innovations in endovascular space into a different dimension, removing the dependence of the procedure on patient-operator ergonomics and likely operator skill.

SLC25A38 congenital sideroblastic anemia: Phenotypes and genotypes of 31 individuals from 24 families, including 11 novel mutations, and a review of the literature.

The congenital sideroblastic anemias (CSAs) are a heterogeneous group of inherited disorders of erythropoiesis characterized by pathologic deposits of iron in the mitochondria of developing erythroblasts. Mutations in the mitochondrial glycine carrier SLC25A38 cause the most common recessive form of CSA. Nonetheless, the disease is still rare, there being fewer than 70 reported families. Here we describe the clinical phenotype and genotypes of 31 individuals from 24 families, including 11 novel mutations. We also review the spectrum of reported mutations and genotypes associated with the disease, describe the unique localization of missense mutations in transmembrane domains and account for the presence of several alleles in different populations.

Association between distance from the radiation source and radiation exposure: A phantom-based study.

OBJECTIVES: The study evaluated the association between distance from radiation source and radiation exposure. BACKGROUND: Radiation exposure during medical procedures is associated with increased risk of cancer and other adverse effects. METHODS: An American National Standards Institute phantom was used to study the relationship between measured entrance surface exposure (MESE) and distance from the X-ray source in postero-anterior, left anterior oblique, and right anterior oblique projections. Three distance settings for table height were evaluated with "low" defined as 52 cm, "mid" 66 cm, and "high" 80 cm from the focal point of the X-ray source. Air-kerma and dose-area product measurements were recorded. Operator exposure with each of these conditions was measured, in a short operator (150 cm) as well as in a tall operator (190 cm). RESULTS: Aggregate results for the three projections were as follows. MESE (µGy/frame) significantly decreased as table-height increases (median, interquartile range, p-value) (low table-height 192.5 [122.4-201.2], mid table-height 105.8 [82.7-115.8], and high table-height 71.7 [58.4-75], p < .0005). The operator exposure (µGy/frame), significantly increased as the table-height increased (low table-height 0.0943 [0.0598-0.1157], medium table-height 0.1128 [0.0919-0.1397], and high table-height 0.158 [0.1339-0.2165], p < .0005). A shorter operator received higher radiation exposure compared to a taller operator (short operator 0.1405 [0.1155-0.1758] and tall operator 0.0995 [0.0798-0.1212], p < .0005). CONCLUSIONS: Increasing table-height is associated with a significant decrease in MESE. Operator radiation exposure increases with increasing table-height and shorter operators receive greater radiation exposure compared to taller operators.

Prediction of probability of rubella based on eye outcomes (PORBEO Nomogram)-a cross-sectional sentinel surveillance of 1134 infants.

INTRODUCTION: Rubella is an important infectious, vaccine-preventable etiology of congenital defects. The aim of the study was to develop a prediction nomogram to assess the probability of an infant being at risk for congenital rubella based on demographics and ophthalmological findings. METHODS: This was a cross-sectional sentinel surveillance study conducted at 5 centers spanning pan-India and involved 1134 infants. The diagnosis of rubella was made using standard guidelines. For the construction of the prediction model, laboratory-confirmed cases were grouped as "at-risk" (AR) infants and the discarded cases into "not at risk" (NAR) infants. Univariate analysis (p value cut-off < 0.05) followed by multivariate binary logistic regression model development was performed. RESULTS: The average (median) age of the suspected CRS infants was 3 (IQR 1-6) months, and the average (mean) age of their mothers was 25.8 ± 4.1 years. Out of the total infants, 81 (7.3%) died, 975 (88%) were alive, and 55 (5.0%) were lost to follow-up. The final model showed that the odds of cataract, retinopathy, glaucoma, microcornea, and age of the infant at presentation were 3.1 (2.2-4.3), 4.9(2.3-10.4), 2.7(1.1-5.9), 2.3(1.1-4.7), and 1.1 (1-1.1), respectively, for the AR infant as compared to NAR infant. AUC of final model was 0.68 (95% CI Delong, 0.64-0.72). Bootstrapping for calibration of the model showed satisfactory results. Nomogram, along with a web version, was developed. CONCLUSION: The developed nomogram would have a wide community-based utilization and will help in prioritizing attention to high-risk children, thereby avoiding loss to follow-up.

Impact of microbial waste additives and glucose on ammonia emissions from broiler litter in the lab.

Ammonia (NH3) produced inside livestock houses can adversely affect animal welfare and performance and degrade the environment. In broiler houses, NH3 levels are mitigated by applying acidifiers to the litter but acidifiers provide short-term NH3 suppression requiring heavy or repeated applications. Microbial additives may provide longer-term NH3 control through nitrogen (N) immobilization and nitrification. The objective of this 50-d lab study was to evaluate the impact of two microbial additives (Environoc 301 and Environoc 501), 2% glucose, and distilled water (control) treatments applied to broiler litter on NH3 emissions and litter properties. During the first 34 d, glucose significantly but modestly reduced NH3 emissions vs. the other treatments which were not significantly different from one-another. For the entire study, when glucose was excluded (due to lost replicates), the three treatments were not significantly different. The unreplicated glucose treatment had higher final litter nitrate concentration than the other treatments. Litter properties were unaffected by the two microbial additive and control treatments. The effectiveness of glucose in reducing NH3 emission could have been due to greater N immobilization and nitrification vs. the other treatments. More research on cost-effective labile carbon sources and higher application rates to achieve greater NH3 reduction is required.

Use of dual lumen ports for red blood cell exchange: A comparison of adults and children with sickle cell disease.

PURPOSE: Sickle cell patients receiving chronic RBC exchange require a form of long-term central venous access if peripheral access is inadequate. In adults, dual lumen (DL) ports have been utilized but associated with greater procedure complications and duration when compared to other forms of access. In the pediatric sickle cell population, the use of DL ports for RBC exchange has not been well described. In this retrospective cohort study, RBC exchange procedures utilizing DL ports in the pediatric vs adult sickle cell population were compared. METHODS: Medical records were reviewed for 685 RBC exchange procedures performed on 25 patients (11 pediatric and 14 adult) between November 2014 to November 2018. Patient-level characteristics and outcomes were compared between pediatric and adult patients using the Fisher-exact and Wilcoxon-rank sum test. Linear/logistic regression models examined procedure-level parameters and port characteristics with adjustment for clustering. RESULTS: Compared to adults, pediatric patients had slower average maximum inlet speed (42 vs 53 mL/min, P < .01), but shorter procedure time (60 vs 75 minutes, P < .01) and lower rate of access alarms (1% vs 11%, P < .01). Overall, 0.29 thrombotic events per 1000 port days and 0.04 infections per 1000 port days were observed. CONCLUSION: For adult and pediatric sickle cell patients, a DL port provides a viable option for RBC exchange. In comparison to adults, pediatric procedures with a DL port will typically be shorter and with less procedural complications due to smaller blood volumes and lower flow requirements.

Paclitaxel-Eluting versus Everolimus-Eluting Coronary Stents in Diabetes.

BACKGROUND: The choice of drug-eluting stent in the treatment of patients with diabetes mellitus and coronary artery disease who are undergoing percutaneous coronary intervention (PCI) has been debated. Previous studies comparing paclitaxel-eluting stents with stents eluting rapamycin (now called sirolimus) or its analogues (everolimus or zotarolimus) have produced contradictory results, ranging from equivalence between stent types to superiority of everolimus-eluting stents. METHODS: We randomly assigned 1830 patients with diabetes mellitus and coronary artery disease who were undergoing PCI to receive either a paclitaxel-eluting stent or an everolimus-eluting stent. We used a noninferiority trial design with a noninferiority margin of 4 percentage points for the upper boundary of the 95% confidence interval of the risk difference. The primary end point was target-vessel failure, which was defined as a composite of cardiac death, target-vessel myocardial infarction, or ischemia-driven target-vessel revascularization at the 1-year follow-up. RESULTS: At 1 year, paclitaxel-eluting stents did not meet the criterion for noninferiority to everolimus-eluting stents with respect to the primary end point (rate of target-vessel failure, 5.6% vs. 2.9%; risk difference, 2.7 percentage points [95% confidence interval, 0.8 to 4.5]; relative risk, 1.89 [95% confidence interval, 1.20 to 2.99]; P=0.38 for noninferiority). There was a significantly higher 1-year rate in the paclitaxel-eluting stent group than in the everolimus-eluting stent group of target-vessel failure (P=0.005), spontaneous myocardial infarction (3.2% vs. 1.2%, P=0.004), stent thrombosis (2.1% vs. 0.4%, P=0.002), target-vessel revascularization (3.4% vs. 1.2%, P=0.002), and target-lesion revascularization (3.4% vs. 1.2%, P=0.002). CONCLUSIONS: In patients with diabetes mellitus and coronary artery disease undergoing PCI, paclitaxel-eluting stents were not shown to be noninferior to everolimus-eluting stents, and they resulted in higher rates of target-vessel failure, myocardial infarction, stent thrombosis, and target-vessel revascularization at 1 year. (Funded by Boston Scientific; TUXEDO-India Clinical Trials Registry-India number, CTRI/2011/06/001830).

Transfusion practices and complications in thalassemia.

BACKGROUND: The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care. STUDY DESIGN AND METHODS: A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs. RESULTS: The centers followed 717 patients with ß-thalassemia (314, 43.8%) or α-thalassemia (394, 55%). One-third (34.7%) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9% of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group. CONCLUSION: This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.

Perforated balloon technique: A simple and handy technique to combat no-reflow phenomenon in coronary system.

OBJECTIVE: Examining the efficacy and outcomes of intracoronary (IC) instillation of adenosine using a novel perforated balloon technique (PBT) to combat no-reflow phenomenon during percutaneous coronary intervention (PCI) for acute coronary syndrome (ACS). BACKGROUND: Occurrence of no-reflow during PCI is a serious adverse prognostic event and inability to re-establish better flow is associated with poor outcomes. Several pharmacological and non-pharmacological interventions have been used to treat this situation. This series describes the use of PBT for IC adenosine administration and its effects on outcomes during real world interventional practice. METHODS: Subjects comprised of 24 patients with ACS (out of a total of 1,634 patients undergoing PCI between January 2016 and June 2017) in whom we used PBT for IC administration of adenosine to treat coronary no-reflow. RESULTS: PBT for IC adenosine instillation was used in 24 (1.5%) of 1,634 patients undergoing PCI. TIMI grade III flow was established in 21 patients (87.5%). In two patients (8.3%) TIMI grade II flow was established and in one patient (4.2%) we were unsuccessful. CONCLUSION: We demonstrate the safety and efficacy of a novel strategy for adenosine instillation in the distal coronary bed, the PBT. This technique enables rapid and cost-effective treatment of no-reflow phenomenon during PCI for ACS.

Sentinel Surveillance for Congenital Rubella Syndrome - India, 2016-2017.

Rubella infection during pregnancy can result in miscarriage, fetal death, stillbirth, or a constellation of congenital malformations known as congenital rubella syndrome (CRS). The 11 countries in the World Health Organization (WHO) South-East Asia Region are committed to the elimination of measles and control of rubella and CRS by 2020. Until 2016, when the Government of India's Ministry of Health and Family Welfare and the Indian Council of Medical Research initiated surveillance for CRS in five sentinel sites, India did not conduct systematic surveillance for CRS. During the first 8 months of surveillance, 207 patients with suspected CRS were identified. Based on clinical details and serologic investigations, 72 (34.8%) cases were classified as laboratory-confirmed CRS, four (1.9%) as congenital rubella infection, 11 (5.3%) as clinically compatible cases, and 120 (58.0%) were excluded as noncases. The experience gained during the first phase of surveillance will be useful in expanding the surveillance network, and data from the surveillance network will be used to help monitor progress toward control of rubella and CRS in India.

"Combo" technique for the use of 7F guide catheter system during transradial approach.

Even a regular lumen of radial artery may create difficulty in smooth negotiation and propagation of a 7F guide catheter while performing intervention through transradial approach. We describe successive five cases of a simple and innovative "Combo" technique, which helps relatively atraumatic tracking of a 7F guide catheter through the course of arm and chest vasculature for successful completion of procedure without significant damage and local pain.

Balloon-assisted tracking: a must-know technique to overcome difficult anatomy during transradial approach.

OBJECTIVE: To examine the use and outcomes of balloon-assisted tracking (BAT) technique for dealing with complexities of arm and chest vasculature during transradial approach (TRA) at a single high volume radial center. BACKGROUND: TRA has been used for coronary angiography and percutaneous coronary interventions (PCI) around the world. Different techniques have been described to address the anatomical issues and tortuosities for successful completion of coronary angiography and PCI. This study describes the use of BAT technique and associated outcomes during real world clinical practice. METHODS: Subjects comprised 63 patients, (out of total 8,245 patients between January 2011 and December 2012) in whom we encountered significantly complex anatomical course in radial, brachial, or subclavian region, leading to difficult advancement of a diagnostic or a guide catheter despite trying all standard maneuvers. In all of them BAT technique was used and they were retrospectively analyzed for the purpose of this study. RESULTS: About 63 (0.76%) of 8,245 patients met the study criteria. Twenty-five (39.7%) patients had very small RA. Twenty-two (34.9%) had severe RA tortuosity. Four (6.3%) had complex RA loops. Six (9.5%) had severe RA spasm and six (9.5%) had severe subclavian tortuosity and/or stenosis. We encountered technical failure in three (4.8%) patients (two had very small RA and one had 360 degree RA loop). CONCLUSION: BAT technique was useful to address the anatomical issues and tortuosities of radial, brachial, and subclavian vasculature during TRA.

Working through complexities of radial and brachial vasculature during transradial approach.

OBJECTIVES: The aim of this review is to discuss different methods of working through radial artery (RA) and brachial artery (BA) vascular complexities to increase the success rate of transradial approach (TRA). BACKGROUND: Anatomical complexities of RA and BA vasculature are an important reason for failure of TRA. There are few current data describing methods to overcome these challenges and reduce TRA failure. METHODS: A series of the primary RA and BA anatomical complexities are identified and management techniques to overcome these variants are discussed. RESULTS: RA and BA vascular complexities can be divided into three subsets including (1) radial artery spasm (RAS); (2) variant anatomy including tortuosity, loops, and anomalous origin of RA; (3) acquired abnormalities including perforations, atherosclerotic lesions, and calcification of RA. In-depth discussion with supportive examples for the identification and management of these challenges are provided. A classification of RAS and perforations is shown and simple algorithms that have been developed for management of RAS, perforations and loops are explained. CONCLUSIONS: Despite lower rates of bleeding and vascular complications as compared to transfemoral approach (TFA), the adoption has been relatively slow particularly due to higher failure rates. Anatomical complexities of RA and BA vasculature play an important role for TRA failure cases. Using a simply framework to classify the anatomical or functional problem, and then approach these challenges in a logical sequence should facilitate management and increase success rate for TRA.

Working through challenges of subclavian, innominate, and aortic arch regions during transradial approach.

OBJECTIVES: The aim of this review is to discuss different methods of working through subclavian, innominate, and aortic arch anatomical challenges to increase the success rate of transradial approach (TRA). BACKGROUND: Anatomical challenges in the subclavian, innominate, and aortic arch regions are important reasons for failure of TRA. There is limited modern literature describing methods to overcome these challenges and reduce TRA failure. METHODS: A number of primary subclavian, innominate, and aortic arch anatomical challenges are identified and management techniques to overcome them are discussed. RESULTS: Subclavian, innominate, and aortic arch anatomical challenges can be divided into five subsets, including (1) tortuosity, (2) loop, (3) stenosis, (4) congenital aberrancy, and (5) combined challenges. In depth discussion with supportive examples for the identification and management of these challenges are provided. CONCLUSIONS: Despite lower rates of bleeding and vascular complications as compared to transfemoral approach, the adoption of TRA has been relatively slow in part due to frustration from operator failure during the learning curve. Anatomical challenges of subclavian, innominate, and aortic arch regions play an important role in TRA procedural failure. Using a simple, conceptual, framework to classify the anatomical or functional problem and then applying a logical approach to these challenges can facilitate management and augment operator success rates for TRA.

Major ionic compositions of fine particulate matter in an animal feeding operation facility and its vicinity.

Animal feeding operations (AFOs) produce particulate matter (PM) and gaseous pollutants. Investigation of the chemical composition of PM2.5 inside and in the local vicinity of AFOs can help to understand the impact of the AFO emissions on ambient secondary PM formation. This study was conducted on a commercial egg production farm in North Carolina. Samples of PM2.5 were collected from five stations, with one located in an egg production house and the otherfour located in the vicinity ofthe farm alongfour wind directions. The major ions of NH4+, Na+, K+, SO4(2-), Cl-, and NO3- were analyzed using ion chromatography (IC). In the house, the mostly abundant ions were SO4(2-), Cl-, and K+. At ambient stations, SO4(2-), and NH4+ were the two most abundant ions. In the house, NH4+, SO4(2-), and NO3- accounted for only 10% of the PM2.5 mass; at ambient locations, NH4+, SO4(2-), and NO3- accounted for 36-41% of the PM2.5 mass. In the house, NH4+ had small seasonal variations indicating that gas- phase NH3. was not the only major force driving its gas-particle partitioning. At the ambient stations, NH4+ had the highest concentrations in summer In the house, K+, Na+, and Cl- were highly correlated with each other In ambient locations, SO4(2-) and NH4+ had a strong correlation, whereas in the house, SO4(2-) and NH4+ had a very weak correlation. Ambient temperature and solar radiation were positively correlated with NH4+ and SO4(2-). This study suggests that secondary PM formation inside the animal house was not an important source of PM2.5. In the vicinity, NH3 emissions had greater impact on PM2.5 formation.

Reversal of Cerebral Arteriopathy Post-Hematopoietic Stem Cell Transplant for Sickle Cell Disease.

Sickle cell disease (SCD) is a chronic hematologic disorder which causes progressive cerebral arteriopathy beginning in childhood. As a result, arterial ischemic stroke is a major cause of morbidity and mortality in SCD, and SCD is a leading cause of childhood stroke worldwide. Allogenic hematopoietic stem cell transplant (HSCT) may be curative for individuals with SCD. Long-term outcomes and effects are currently being studied. In this report, we describe a child with SCD who presented with arterial ischemic stroke at 6 years of age and was found to have a severe form of cerebral large vessel arteriopathy by catheter-directed angiography. The patient initially underwent revascularization surgery by indirect superficial temporal artery to middle cerebral artery bypass, and 1 year later, he underwent curative HSCT. Approximately 3 years after HSCT, repeat catheter-directed angiography revealed a striking reversal of cerebral large vessel arteriopathy. This article reveals a previously unrecognized and potentially beneficial effect of HSCT that may ameliorate cerebral large vessel arteriopathy and improve cerebrovascular health for children with SCD.