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ASCC3 is a multi-functional helicase that contains two consecutive Ski2-like helicase units. Here, the authors show that ASCC3 can unwind DNA by threading one strand of a substrate duplex through both helicase units, supported by the TRIP4 protein.

Abstract Background There are persistent disparities in maternal and infant perinatal outcomes experienced by Black birthing persons compared with non-Hispanic white (NHW) individuals in the US. The differences in outcomes arise from not only socioeconomic factors and individual health behaviors but also structural racism. Recent research is beginning to elucidate the benefits of patient navigation to support underserved minoritized individuals who experience this constellation of barriers to equitable care. Qualitative research that utilizes both the experiences of Black birthing individuals and the expert opinion of healthcare providers working with them can serve to guide a patient navigation intervention to further decrease disparities in perinatal outcomes. Methods We conducted 30 interviews between August and December 2020 with Black birthing individuals in the Chicago metropolitan area and healthcare providers who care for this population both in Chicago and across the nation to explore their experiences, perceptions of barriers to care and ways to decrease inequities. Results Clinical care team members acknowledged the presence of health disparities experienced by Black pregnant individuals compared with their NHW counterparts stemming from racism, discrimination, and lack of resources. Patients similarly reported personal experiences with these disparities and barriers to care. The successful methods used by clinical care teams to help decrease these differences in the past included patient education on important topics such as breastfeeding and the use of patient advocates. Effectively screening for social determinants of health by someone the patient trusts was also cited as important. Regarding perinatal care practices, clinical care team members described the importance of patient education needs and care team cultural competency. Patients’ reported positive and negative experiences corroborated these findings, emphasizing the importance of trust, listening, education, access to care, support, and patient advocacy. Finally, the care team members and patients agreed that active trust-building can help the provider/patient relationship and ultimately improve outcomes. Conclusions These qualitative research findings improve the understanding of barriers to care and will help guide development of an intervention to reduce the health disparities experienced by Black pregnant persons.

While robotic surgery has shown clear utility and advantages in the adult population, its role in pediatrics remains controversial. Pediatric-sized robotic instruments and equipment are not readily available yet, so certain modifications can be made in order to make robotic surgery successful in children. While the cost of robotic surgery remains high compared to open procedures, patients experience greater satisfaction and quality of life with robotic surgery. Robotic pyeloplasty is a standard of care in older children, and has even been performed in infants and re-do surgery. Other robotic procedures performed in children include heminephroureterectomy, ureteroureterostomy, ureteral reimplantation, urachal cyst excision, bladder diverticulectomy, and bladder reconstructive procedures such as augmentation, appendicovesicostomy, antegrade continence enema, bladder neck reconstruction and sling, as well as other procedures. Robotic surgery has also been used in oncologic cases such as partial nephrectomy and retroperitoneal lymph node dissection. Future improvements in technology with production of pediatric-sized robotic instruments, along with increases in robotic-trained pediatric urologists and surgeon experience along each's learning curve, will help to further advance the field of robotic surgery in pediatric urology.

Abstract Background Older adults may find it problematic to attend hospital appointments due to the difficulty associated with travelling to, within and from a hospital facility for the purpose of a face-to-face assessment. This study aims to investigate equivalence between telephone and face-to-face administration for the Frenchay Activities Index (FAI) and the Euroqol-5D (EQ-5D) generic health-related quality of life instrument amongst an older adult population. Methods Patients aged >65 (n = 53) who had been discharged to the community following an acute hospital admission underwent telephone administration of the FAI and EQ-5D instruments seven days prior to attending a hospital outpatient appointment where they completed a face-to-face administration of these instruments. Results Overall, 40 subjects' datasets were complete for both assessments and included in analysis. The FAI items had high levels of agreement between the two modes of administration (item kappa's ranged 0.73 to 1.00) as did the EQ-5D (item kappa's ranged 0.67–0.83). For the FAI, EQ-5D VAS and EQ-5D utility score, intraclass correlation coefficients were 0.94, 0.58 and 0.82 respectively with paired t-tests indicating no significant systematic difference (p = 0.100, p = 0.690 and p = 0.290 respectively). Conclusion Telephone administration of the FAI and EQ-5D instruments provides comparable results to face-to-face administration amongst older adults deemed to have cognitive functioning intact at a basic level, indicating that this is a suitable alternate approach for collection of this information.

Abstract Background Most studies of men undergoing treatment for prostate cancer examine physical symptoms as predictors of Quality of Life (QOL). However, symptoms vary by treatment modality in this population, and psychosocial variables, shown to be important to QOL, have rarely been examined. Litwin noted a need for analysis of QOL data in men treated for prostate cancer with different modes of therapy, as studies focusing on specific treatments will increase the homogeneity of research findings. Methods This cross-sectional study explored physical and psychosocial predictors of QOL in men receiving one of two types of radiation treatment for prostate cancer: Intensity Modulated Radiation Therapy (IMRT) + High Dose Rate (HDR) Brachytherapy or IMRT + seed implantation. Subjects completed a biographic questionnaire; quality of life measures, which were the eight subscales of the Medical Outcome Study Short Form Health Survey (SF-36); measures of physical symptoms including the Radiation Therapy Oncology Group and the European Organization for Research and Treatment of Cancer (RTOG/EORTC) and the Prostate Symptom Self-Report (PSSR); and measures of psychological factors, the Ways of Coping Scale (WOC), Perceived Stress Scale, the Anxiety Subscale of the SCL-90, and Strategies Used by Patients to Promote Health (SUPPH). Eight regression models including both physical and psychosocial variables were used to predict quality of life. Results Sixty-three subjects with complete data on all variables were studied. Treatment effect sizes were medium to large in predicting each of the quality of life subscales of the SF-36. Psychosocial variables were related to physical function, role function, bodily pain, general health, social function, emotional role, and mental health. Physical symptoms were related to subjects' perceived general health and mental health. Discussion The number of significant relationships among psychosocial variables and indicators of QOL exceeded the number of relationships among symptoms and QOL suggesting that psychosocial variables associate strongly with prostate cancer patients' reports of quality of life. Findings of the study may provide patients and families with knowledge that contributes to their understanding of quality of life outcomes of IMRT+ HDR and IMRT + seed implantation and their ability to make more informed treatment choices.

Objective: Emphysematous cystitis (EC) is a rare urinary tract infection (UTI) typically associated with severe diabetes in older women. We present a unique case of this gas-forming infection in a man with type 2 diabetes mellitus (T2DM) treated with empagliflozin. To the best of our knowledge, this is the first case report of EC associated with the use of a sodium-glucose cotransporter 2 inhibitor (SGLT2i). Case Report: A 62-year-old man with T2DM treated with an SGLT2i developed EC. His moderately controlled T2DM was treated for over 20 years with metformin, saxagliptin/metformin, and pioglitazone to which empagliflozin was added due to his consistently elevated hemoglobin A1c level, slightly reduced estimated glomerular filtration rate, and proteinuria. Four months after initiation of the SGLT2i, he reported lower urinary tract symptoms and was found to have EC radiographically. His urine cultures were positive for Klebsiella pneumonia and was found to have asymptomatic urinary retention. He was treated conservatively, and his outcome was favorable. Discussion: EC is commonly seen in patients with diabetes mellitus, and symptoms range from asymptomatic to severe sepsis. Most urine cultures grow Escherichia coli and K. pneumonia. The association of increased UTIs in susceptible patients with T2DM with the use of SGLT2i is yet to be determined. Most cases of EC are diagnosed radiographically and treated conservatively, although some cases require surgical intervention. Conclusion: Initially, our patient was considered a good candidate for treatment with an SGLT2i. The subsequent development of EC precluded its further use. The role of SGLT2i in patients with T2DM susceptible to UTI is controversial.

Children with sickle cell disease (SCD) are at increased risk of invasive pneumococcal disease (IPD). Over 25 years, the Georgia Emerging Infections Program/Centers for Disease Control and Prevention Active Bacterial Core Surveillance network identified 104 IPD episodes among 3707 children with hemoglobin SS (HbSS) or HbSC aged <10 years, representing 6% of IPD in Black or African American children residing in Metropolitan Atlanta (reference population). Children with IPD and HbSS/SC were older than those with IPD in the reference population (P < .001). From 1994-1999 to 2010-2018, IPD declined by 87% in children with HbSS aged 0 to 4 years, and by 80% in those aged 5 to 9 years. However, IPD incidence rate ratios when comparing children with SCD with the reference population increased from 20.2 to 29.2 over these periods. Among children with HbSS and IPD, death declined from 14% to 3% after 2002, and meningitis declined from 16% to 8%. Penicillin resistance was more prevalent in children with SCD before 7-valent pneumococcal conjugate vaccine (PCV7) licensure. After 2010, all IPD serotypes were not included in the 13-valent PCV (PCV13). Within 3 years of vaccination, the effectiveness of the 23-valent pneumococcal polysaccharide vaccine (PPSV23) against non-PCV13 serotypes included in PPSV23 plus 15A/15C was 92% (95% confidence interval, 40.8- 99.0, P = .014; indirect-cohort effect adjusted for age and hydroxyurea). PPSV23 would cover 62% of non-PCV13 serotype IPD in children with SCD, whereas PCV15, PCV20, and PCV21/V116 (in development) could cover 16%, 51%, and 92%, respectively. Although less frequent, IPD remains a life-threatening risk in children with SCD. Effective vaccines with broader coverage could benefit these children., (© 2023 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Introduction/objective: Acute pain episodes are a major cause of health care utilization (HCU) in sickle cell disease (SCD), and adolescence is associated with increased pain frequency. We sought to determine whether there were differences in acute pain trajectories by sex and frequency of pain episodes among adolescents with SCD who presented to the emergency department (ED)., Methods: Retrospective review of electronic health records from a large, multicampus, pediatric SCD program., Results: Of the 113 adolescents included, the mean age was 16.6 (SD 0.9), 41.6% (n = 47) were female, 77.9% (n = 88) had HbSS or a similarly severe genotype, and 43.4% (n = 49) had ≥3 episodes of HCU for pain, which we defined as having history of high HCU for pain. Those with a history of high HCU for pain had higher mean pain intensity scores at presentation, were more likely to receive either intravenous or intranasal opioids, and were more likely to be hospitalized. In a model considering the 3-way interaction between sex, history of high HCU for pain, and follow-up time from the initial pain intensity score, adjusted for opioid per kilogram body weight, and prescription of hydroxyurea, adolescent female patients with high HCU for pain had the slowest decline in pain intensity during treatment for acute pain in the ED., Conclusion: Sex and history of high HCU for pain are associated with acute pain trajectories in adolescents with SCD presenting to the ED. These novel findings should be confirmed in future prospective studies., Competing Interests: CRM is the Executive Director of Food as Medicine Therapeutics, LLC; and is on the scientific advisory Board for Trility. CRM is the inventor or co-inventor of several UCSF-Benioff Children's Hospital Oakland patents/patent-pending applications that include nutritional supplements for autism/apraxia (receiving royalties); is an inventor or co-inventor of several Emory University School of Medicine patent application for nutritional supplements for autism, and coronaviruses, and kidney disease and is a consultant for Roche and CSL Behring, and an editor for the sickle cell disease-fever reference for UpToDate.Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The International Association for the Study of Pain.)

Significant amounts of fresh and fresh-cut fruits and vegetables are wasted every year due to enzymatic browning. Polyphenol oxidase (PPO) is the key enzyme involved in the enzymatic browning. In the past decades, various methods have been developed to inhibit browning of various fresh produce items. However, for most fresh horticultural produce, ideal measures accepted by industries and consumers are still scarce. This review provides up-to-date knowledge of browning control technologies, including physical methods, chemical methods such as natural inhibitors, molecular biotechnology, and nanotechnology. In addition, we propose some ideas to improve the efficacies of these strategies with fewer side effects. To better inhibit tissue browning, new research directions are also discussed, for example, regulation of PPO substrate techniques., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objectives: To determine the acceptability, feasibility and safety of yoga for chronic pain in sickle cell disease., Design and Setting: In Part A of this two-part study, adolescents with SCD and chronic pain (Group 1) and their parent (Group 2) completed a survey designed to capture pain characteristics, attitudes and practices related to yoga, and potential acceptability of a yoga program. In Part B, the study assessed the feasibility and safety of an instructor-led group yoga program. The study was registered on clinicaltrials.gov (NCT03694548)., Intervention: Eight instructor-led group yoga sessions., Main Outcome Measures: Feasibility and safety outcomes were chosen a priori, as follows: 1) Proportion of adolescent patients with SCD and chronic pain approached that consent to participate in Part A, 2) Proportion of adolescent participants enrolled in Part A that consent to participate in Part B, 3) Proportion of participants enrolled in Part B that attend at least 6 of 8 yoga sessions, 4) Proportion of participants enrolled in Part B with an ED visit or a hospitalization for pain within 24 h of completion of each yoga session, 5) Proportion of participants in Part B who complete all study assessments before, and at the end of the yoga program, 6) Adherence to submission of pain diary., Results: The median age of 15 patient participants in Part A was 16 (IQR 14-17), and 14 parents was 43.5 (IQR 42-51). Most participants were female. Most participant responses indicated a positive opinion of yoga. Nine adolescents (60 %) from Part A participated in Part B of the study. The median age of 9 participants in Part B was 17 (IQR 15-18), and 5 of the 9 participants were female (53.3 %). Only one participant was able to attend 3 of the 8 yoga sessions offered, and did not experience any ED visits or hospitalizations following the yoga sessions. None of the other feasibility endpoints were met in this study., Conclusions: Patients with SCD and chronic pain overall have a positive opinion of yoga, but there are challenges with recruitment and retention of participants in a clinical trial of yoga, and barriers to feasibility of an in-person group yoga intervention., (Copyright © 2021. Published by Elsevier Ltd.)

Altered mitochondrial function occurs in sickle cell disease (SCD), due in part to low nitric oxide (NO) bioavailability. Arginine, the substrate for NO production, becomes acutely deficient in SCD patients with vaso-occlusive pain episodes (VOE). To determine if arginine improves mitochondrial function, 12 children with SCD-VOE (13.6 ± 3 years; 67% male; 75% hemoglobin-SS) were randomized to 1 of 3 arginine doses: (1) 100 mg/kg IV 3 times/day (TID); (2) loading dose (200 mg/kg) then 100 mg/kg TID; or (3) loading dose (200 mg/kg) followed by continuous infusion (300 mg/kg per day) until discharge. Platelet-rich plasma mitochondrial activity, protein expression, and protein-carbonyls were measured from emergency department (ED) presentation vs discharge. All VOE subjects at ED presentation had significantly decreased complex-V activity compared to a steady-state cohort. Notably, complex-V activity was increased at discharge in subjects from all 3 arginine-dosing schemes; greatest increase occurred with a loading dose (P < .001). Although complex-IV and citrate synthase activities were similar in VOE platelets vs steady state, enzyme activities were significantly increased in VOE subjects after arginine-loading dose treatment. Arginine also decreased protein-carbonyl levels across all treatment doses (P < .01), suggesting a decrease in oxidative stress. Arginine therapy increases mitochondrial activity and reduces oxidative stress in children with SCD/VOE. This trial was registered at www.clinicaltrials.gov as #NCT02536170., (© 2020 by The American Society of Hematology.)

Objective: Several states are building infrastructure and data collection methods for longitudinal, population-based surveillance systems for selected hemoglobinopathies. The objective of our study was to improve an administrative case definition for sickle cell disease (SCD) to aid in longitudinal surveillance., Methods: We collected data from 3 administrative data sets (2004-2008) on 1998 patients aged 0-21 in Georgia who had ≥1 encounter in which an SCD International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) code was recorded, and we compared these data with data from a laboratory and medical record review. We assessed performance (sensitivity, specificity, positive predictive value [PPV], and negative predictive value [NPV]) of case definitions that differed by number and type of SCD-coded encounters; addition of SCD-associated treatments, procedures, and complications; and length of surveillance (1 vs 5 years). We identified correct diagnoses for patients who were incorrectly coded as having SCD., Results: The SCD case definition of ≥3 SCD-coded encounters in 5 years simplified and substantially improved the sensitivity (96.0% vs 85.8%) and NPV (68.2% vs 38.2%) of the original administrative case definition developed for 5-year, state-based surveillance (≥2 encounters in 5 years and ≥1 encounter for an SCD-related treatment, procedure, or complication), while maintaining a similar PPV (97.4% vs 97.4%) and specificity (76.5% vs 79.0%)., Conclusions: This study supports an administrative case definition that specifies ≥3 ICD-9-CM-coded encounters to identify SCD with a high degree of accuracy in pediatric patients. This case definition can be used to help establish longitudinal SCD surveillance systems.

The clinical efficacy of hydroxyurea in patients with sickle cell anemia (SCA) has been well established. However, data about its clinical effectiveness in practice is limited. We evaluated the clinical effectiveness of hydroxyurea in a large pediatric population using a retrospective cohort, pre-post treatment study design to control for disease severity selection bias. The cohort included children with SCA (SS, Sβ 0 thalassemia) who received care at Children's Healthcare of Atlanta (CHOA) and who initiated hydroxyurea in 2009-2011. Children on chronic transfusions, or children with inadequate follow up data and/or children who had taken hydroxyurea in the 3 years prior were excluded. For each patient healthcare utilization, laboratory values, and clinical outcomes for the 2-year period prior to hydroxyurea initiation were compared to those 2 years after initiation. Of 211 children with SCA who initiated hydroxyurea in 2009-2011, 134 met eligibility criteria. After initiation of hydroxyurea, rates of hospitalizations, pain encounters, and emergency department visits were reduced by 47% (<0.0001), 36% (P = 0.0001) and 43% (P < 0.0001), respectively. Average hemoglobin levels increased by 0.7 g/dl (P < 0.0001). Hydroxyurea effectiveness was similar across gender, insurance types and age, although there was a slightly greater reduction in hospitalizations in younger children. Am. J. Hematol. 92:77-81, 2017. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

Malignant gliomas, including glioblastoma (GBM), are the most common primary brain tumors. Despite extensive research only modest gains have been made in long-term survival. Standard of care involves maximizing safe surgical resection followed by concurrent chemoradiation with temozolomide. Immunotherapy for GBM is an area of intense research in recent years. New immunotherapies, although promising, have not been integrated into standard practice. Human cytomegalovirus (HCMV) is a DNA virus of the family Herpesviridae. Human seroprevalence is approximately 80%, and in most cases, is associated with asymptomatic infection. HCMV may be an important agent in the initiation, promotion and/or progression of tumorigenesis. Regardless of a possible etiologic role in GBM, interest has centered on exploiting this association for development of immunomodulatory therapies.

Pigs are evidently more resistant to avian than swine influenza A viruses, mediated in part through frontline epithelial cells and alveolar macrophages (AM). Although porcine AM (PAM) are crucial in influenza virus control, their mode of control is unclear. To gain insight into the possible role of PAM in the mediation of avian influenza virus resistance, we compared the host effects and replication of two avian (H2N3 and H6N1) and three mammalian (swine H1N1, human H1N1 and pandemic H1N1) influenza viruses in PAM. We found that PAM were readily susceptible to initial infection with all five avian and mammalian influenza viruses but only avian viruses caused early and extensive apoptosis (by 6 h of infection) resulting in reduced virus progeny and moderated pro-inflammation. Full length viral PB1-F2 present only in avian influenza viruses is a virulence factor that targets AM for mitochondrial-associated apoptotic cell death. With the use of reverse genetics on an avian H5N1 virus, we found that full length PB1-F2 contributed to increased apoptosis and pro-inflammation but not to reduced virus replication. Taken together, we propose that early apoptosis of PAM limits the spread of avian influenza viruses and that PB1-F2 could play a contributory role in the process.

Background: An experiment was conducted in Vietnam to test the hypothesis that total dry matter (DM) intake and liveweight (LW) gain would increase in a curvilinear manner with increasing amounts of concentrate offered., Method: There were five treatments: a basal diet of Guinea grass fed at 1 % of LW and rice straw fed ad libitum (T0), or this diet supplemented with concentrate at 0.6 (T1), 1.2 (T2), 1.8 (T3), or 2.4 % of LW (T4). The concentrate comprised locally available ingredients, namely cassava chips, rice bran, crushed rice grain, fishmeal, salt, and urea, mixed manually., Results: Concentrate intake increased from T0 to T3, but there was no difference in concentrate intake between T3 and T4. Total feed intake increased in a curvilinear manner from 4.0 to 6.4 kg DM/d as the quantity of concentrate consumed increased. The substitution of concentrate for grass and rice straw increased with increasing consumption of concentrate and was as high as 0.49 kg DM reduction per kg of concentrate consumed. LW gain increased curvilinearly, with significant differences between T0 (0.092 kg/d), T1 (0.58 kg/d) and T2 (0.79 kg/d); but there were no significant differences in LW gain between T2, T3 (0.83 kg/d) and T4 (0.94 kg/d).With increasing amount of concentrate in the diet, the digestibilities of dry matter, organic matter, crude protein, and crude fat increased, but NDF digestibility decreased., Conclusion: Based on these results, young Vietnamese Brahman-cross growing cattle will respond to a locally-sourced concentrate mix offered at a level of up to 1.2 % of LW.

Photocurrent in an organic solar cell is generated by a charge transfer reaction between electron donors and acceptors. Charge transfer is expected to proceed from thermalized states, but this picture has been challenged by recent studies that have investigated the role of hot excitons. Here we show a direct link between excess excitation energy and photocarrier mobility. Charge transfer from excited donor molecules generates hot photocarriers with excess energy coming from the offset between the lowest unoccupied molecular orbital of the donor and that of the acceptor. Hot photocarriers manifest themselves through a short-lived spike in terahertz photoconductivity that decays on a picosecond timescale as carriers thermalize. Different dynamics are observed when exciting the acceptor at its absorption edge to a thermalized state. Charge transfer in this case generates thermalized carriers described by terahertz photoconductivity dynamics consisting of an instrument-limited rise to a long-lived signal.

The objective of this study was to evaluate the predictions of dry matter intake (DM) and average daily gain (ADG) of Vietnamese Yellow (Vang) purebred and crossbred (Vang with Red Sindhi or Brahman) bulls fed under Vietnamese conditions using two levels of solution (1 and 2) of the large ruminant nutrition system (LRNS) model. Animal information and feed chemical characterization were obtained from five studies. The initial mean body weight (BW) of the animals was 186, with standard deviation ±33.2 kg. Animals were fed ad libitum commonly available feedstuffs, including cassava powder, corn grain, Napier grass, rice straw and bran, and minerals and vitamins, for 50 to 80 d. Adequacy of the predictions was assessed with the Model Evaluation System using the root of mean square error of prediction (RMSEP), accuracy (Cb), coefficient of determination (r(2)), and mean bias (MB). When all treatment means were used, both levels of solution predicted DMI similarly with low precision (r(2) of 0.389 and 0.45 for level 1 and 2, respectively) and medium accuracy (Cb of 0.827 and 0.859, respectively). The LRNS clearly over-predicted the intake of one study. When this study was removed from the comparison, the precision and accuracy considerably increased for the level 1 solution. Metabolisable protein was limiting ADG for more than 68% of the treatment averages. Both levels differed regarding precision and accuracy. While level 1 solution had the least MB compared with level 2 (0.058 and 0.159 kg/d, respectively), the precision was greater for level 2 than level 1 (0.89 and 0.70, respectively). The accuracy (Cb) was similar between level 1 and level 2 (p = 0.8997; 0.977 and 0.871, respectively). The RMSEP indicated that both levels were on average under- or over-predicted by about 190 g/d, suggesting that even though the accuracy (Cb) was greater for level 1 compared to level 2, both levels are likely to wrongly predict ADG by the same amount. Our analyses indicated that the level 1 solution can predict DMI reasonably well for this type of animal, but it was not entirely clear if animals consumed at their voluntary intake and/or if the roughness of the diet decreased DMI. A deficit of ruminally-undegradable protein and/or a lack of microbial protein may have limited the performance of these animals. Based on these evaluations, the LRNS level 1 solution may be an alternative to predict animal performance when, under specific circumstances, the fractional degradation rates of the carbohydrate and protein fractions are not known.

Background and Objectives: Sickle cell nephropathy begins in childhood and may progress to renal failure. Albuminuria is a sensitive marker of glomerular damage that may indicate early chronic kidney disease (CKD)., Design, Setting, Participants, & Measurements: The aims of this study were to determine the cross-sectional prevalence and clinical correlates of albuminuria and CKD among children with sickle cell disease (SCD). Over a 10-year period (1995 to 2005) 410 pediatric SCD patients ages 2 to 21 years were enrolled: 261 with hemoglobin SS (HbSS) or HbSβ(0) thalassemia (HbSβ(0)) and 149 with HbSC or HbSβ(+) thalassemia (HbSβ(+)). The albumin/creatinine ratio (ACR) of spot-urine specimens and serum creatinine were measured; abnormal albuminuria was defined as urinary ACR ≥ 30 mg/g., Results: The prevalence of abnormal albuminuria was 20.7% (23.0% in HbSS/HbSβ(0), 16.8% in HbSC/HbSβ(+)). Among HbSS/HbSβ(0), abnormal albuminuria was associated with increasing age and lower baseline hemoglobin. GFR, estimated in 189 patients using the updated Schwartz formula, correlated negatively with age (r = -0.27, P = 0.0002). CKD defined according to the Kidney Disease: Improving Global Outcomes study was present in 26.5% (50 of 189) of patients: stage 1 in 27 (14.8%) and stage 2 in 22 (11.6%). In multivariate analysis, age and HbSC/HbSβ(+) genotype were associated with CKD., Conclusions: This is the first study to stage CKD in children with SCD and highlights a high prevalence of albuminuria and glomerular injury early in life. Detecting CKD in childhood could allow for earlier intervention and prevention of renal failure in adulthood.

We evaluated spleen function in 193 children with sickle cell anemia 8 to 18 months of age by (99m)Tc sulfur-colloid liver-spleen scan and correlated results with clinical and laboratory parameters, including 2 splenic biomarkers: pitted cell counts (PIT) and quantitative Howell-Jolly bodies (HJB) enumerated by flow cytometry. Loss of splenic function began before 12 months of age in 86% of infants in association with lower total or fetal hemoglobin and higher white blood cell or reticulocyte counts, reinforcing the need for early diagnosis and diligent preventive care. PIT and HJB correlated well with each other and liver-spleen scan results. Previously described biomarker threshold values did define patients with abnormal splenic function, but our data suggest that normal spleen function is better predicted by PIT of ≤1.2% or HJB ≤55/10(6) red blood cells and absent function by PIT ≥4.5% or HJB ≥665/10(6). HJB is methodologically advantageous compared with PIT, but both are valid biomarkers of splenic function. This trial was registered at www.clinicaltrials.gov as #NCT00006400., (© 2011 by The American Society of Hematology)

The design, fabrication, and properties of one of a new class of gradient-index lenses are reported. The lens is an f/2.25 GRIN singlet based on a nanolayered polymer composite material, designed to correct for spherical aberration. The light gathering and focusing properties of the polymer lens are compared to a homogeneous BK7 glass singlet with a similar f-number. The modulation transfer function of the polymer GRIN lens exceeded that of the homogeneous glass lens at all spatial frequencies and was as much as 3 times better at 5 cyc/mm. The weight of the polymer lens was approximately an order of magnitude less than the homogeneous glass lens.