Adjustment Difficulties of Adolescents With Sickle Cell Disease

TOPIC: The psychosocialfactors that affect adolescents with sickle cell disease (SCD). PURPOSE: To explore whether specific psychosocial factors can provide clues to the future adjustment of this population. SOURCES: Ovid Web, Medline, Psychinfo, and CINAHL databases for the years 1997 to 2001. CONCLUSIONS: Promoting effective psychosocial functioning is as important as managing the medical aspects of SCD, yet this is an area of care that is commonly overlooked. Nurses, therefore, have an opportunity to have a significant impact on the lives of adolescents with SCD if they intervene in ways to promote both biological and psychosocial adjustment.     

Improving Transition Readiness in Young Adults With Sickle Cell Disease

Transition of young adults with sickle cell disease is essential. This project examined the effectiveness of an educational intervention at a pediatric hematology practice to improve transition readiness in young adults with sickle cell disease. Interventions used were 2 Stepping Up videos and 5 questions from “Incorporating Health Care Transition Services Into Preventive Care for Adolescents and Young Adult.” Data was collected and results were analyzed preintervention, immediately postintervention, and at return office visit. Non–high school graduates showed greater improvement in total and subscale mean scores after the intervention at the return office visit.     

High Fetal Splenic Artery Peak Velocity in Fetuses With Hemoglobin Bart Disease

Objective. The purpose of this study was to evaluate the role of the splenic artery (SPA) peak systolic velocity (PSV) in identifying fetuses with hemoglobin (Hb) Bart disease among pregnancies at risk for the disease. Methods. Pregnancies at risk for fetal Hb Bart disease scheduled for cordocentesis at 18 to 25 weeks' gestation at Maharaj Nakorn Chiang Mai Hospital were recruited into the study. The SPA PSV was measured before cordocentesis, and the final fetal diagnosis of Hb Bart disease was based on fetal Hb typing using high‐performance liquid chromatography. Results. Seventy‐six singleton pregnancies at risk for fetal Hb Bart disease were sonographically evaluated for the SPA PSV and underwent cordocentesis for fetal blood analysis. Among the 76 recruited pregnancies, 17 fetuses with Hb Bart disease were finally diagnosed by fetal blood analysis with high‐performance liquid chromatography, and the remainder had no abnormalities or had the α‐thalassemia 1 trait and were defined as unaffected fetuses. The mean SPA PSVs ± SD for the unaffected and affected fetuses were significantly different: 21.17 ± 3.7 cm/s (range, 13.8–29.9 cm/s) and 26.12 ± 3.6 cm/s (range, 20.4–31.5 cm/s) respectively. The SPA PSV of the affected fetuses was higher than that of the unaffected ones (Wilcoxon signed rank test, P < .001). Conclusions. Splenic artery PSV assessment at mid pregnancy may have a potential role in identifying fetuses with Hb Bart disease. Further studies to evaluate the effectiveness of the SPA PSV in differentiating affected from unaffected fetuses among pregnancies at risk are desirable.     

Effect of Propranolol as Antiadhesive Therapy in Sickle Cell Disease

Sickle red blood cells (SSRBCs) adhere to both endothelial cells (ECs) and the extracellular matrix. Epinephrine elevates cyclic adenosine monophosphate in SSRBCs and increases adhesion of SSRBCs to ECs in a β‐adrenergic receptor and protein kinase A‐dependent manner. Studies in vitro as well as in vivo have suggested that adrenergic stimuli like epinephrine may contribute to vaso‐occlusion associated with physiologic stress. We conducted both animal studies and a Phase I dose‐escalation study in sickle cell disease (SCD) patients to investigate whether systemically administered propranolol inhibits SSRBC adhesion and to document the safety of propranolol in SCD. Systemically administered propranolol prevented SSRBC adhesion and associated vaso‐occlusion in a mouse model. In patients receiving a single oral dose of 10, 20, or 40 mg propranolol, SSRBC adhesion to ECs was studied before and after propranolol, with and without stimulation with epinephrine. Propranolol administration significantly reduced epinephrine‐stimulated SSRBC adhesion in a dose dependent manner (p = 0.03), with maximum inhibition achieved at 40 mg. Adverse events were not severe, did not show dose dependence, and were likely unrelated to drug. No significant heart rate changes occurred. These results imply that β‐blockers may have a role as antiadhesive therapy for SCD. Clin Trans Sci 2012; Volume 5: 437–444     

Advances in the Treatment of Sickle Cell Disease

Sickle cell disease (SCD) is a monogenic disorder that afflicts approximately 100,000 Americans and millions of people worldwide. It is characterized by hemolytic anemia, vaso-occlusive crises, relentless end-organ injury, and premature death. Currently, red blood cell transfusion and hydroxyurea are the major disease-modifying therapies available for SCD. Hematopoetic stem cell transplant is curative, but barriers to treatment are substantial and include a lack of suitable donors, immunologic transplant rejection, long-term adverse effects, prognostic uncertainty, and poor end-organ function, which is especially problematic for older patients. Gene therapy to correct the β^s point mutation is under investigation as another curative modality. Deeper insights into the pathophysiology of SCD have led to the development of novel agents that target cellular adhesion, inflammation, oxidant injury, platelets and/or coagulation, vascular tone, and hemoglobin polymerization. These agents are in preclinical and clinical trials. One such agent, L-glutamine, decreases red blood cell oxidant injury and is recently US Food and Drug Administration approved to prevent acute pain episodes of SCD in patients 5 years of age or older. The purpose of this review is to describe the currently established therapies, barriers to curative therapies, and novel therapeutic agents that can target sickle cell hemoglobin polymerization and/or its downstream sequelae. A PubMed search was conducted for articles published up to May 15, 2018, using the search terms sickle cell disease, novel treatments, hematopoietic stem cell transplantation, and gene therapy. Studies cited include case series, retrospective studies, prospective clinical trials, meta-analyses, online abstracts, and original reviews.     

双功彩色多普勒显像对Ⅱ型糖尿病患者肾动脉血流的研究

本文检测３０例正常人，６６例Ⅱ型糖尿病患者的肾动脉主于和段间动脉的血流频谱。结果表明：第一组肾主动脉血流频谱各项参数与正常对照组比较，均未见统计学差异。段间动脉的ＲＩ与对照组比较有显著性差异，（Ｐ＜０．０１）。第二组肾动脉和段间动脉的血流动力学参数与对照组比较有显著性差异。第二组处于收缩期正常流速，舒张末期低流速，肾血管高阻状态。我们认为根据多普勒定量参数（Ｖｄｍｉｎ、ＰＩ、ＲＩ），特殊的频谱图形和彩流图改变，可对肾血管受损作出判断。     

正常人肾动脉内径和血流参数的超声测量

本文报告用二维超声和多普勒超声测量３０６只正常肾的肾动脉内径和肾动脉、弓形动脉的血流参数。结果表明；正常成人肾动脉、弓形动脉的收缩期峰速、舒张末期流速、脉动指数和阻力指数与性别、年龄、左右侧肾无相关，而随体表面积的增大，肾动脉内径增大；随年龄的增长，肾动脉内径有所增宽，但后者变化无统计学意义。     

Evaluating Microalbuminuria in Children with Sickle Cell Disease: Review of the Literature

Microalbuminuria, an early marker of renal injury, may begin early in sickle cell disease (SCD). We conducted a retrospective chart review of 205 patients with SCD. Clinical data and treatment regimens were reviewed. Data were analyzed to determine prevalence and correlates of microalbuminuria and proteinuria. Thirty-one patients (15.2%) had microalbuminuria. Proteinuria, hematuria, age, height, weight, and systolic blood pressure were significantly associated with microalbuminuria. Age was the strongest predictor. We propose that all children with SCD be screened by annual urinalysis and careful blood pressure monitoring at each clinic visit for early identification of children at risk for renal injury.     

Physicians' Perceptions of Factors Influencing Adherence to Antibiotic Prophylaxis in Children with Sickle Cell Disease

Background: Children with sickle cell disease (SCD) aged <5 years are at great risk for invasive infection with Staphylococcus pneumoniae and Haemophilus influenzae due to the inability of their spleen to protect against infection.Objectives: This study examined (1) physicians' perceptions of factors associated with adherence to antibiotic prophylaxis in children with SCD and (2) how physician characteristics are associated with these perceptions.Methods: A MEDLINE search of the years 1996 to 2002, using the terms sickle cell disease, compliance, children, physician perceptions, and antibiotic prophylaxis, was done. A survey was developed using existing literature to assess physicians' perceptions of factors associated with adherence to antibiotic prophylaxis for SCD. The survey was sent to a stratified random sample of 375 pediatricians and all 125 practicing hematologists in North Carolina. They were given a Likert-type scale to assess their perceptions of factors that influence patients' adherence to antibiotic prophylaxis for SCD. Physician demographic information was collected using the North Carolina Health Professions Data Book. The demographic information was matched to the survey respondent and correlated with his or her responses.Results: The response rate was 56.9%. Of the respondents, 60.9% were pediatricians, and 56.5% were in a practice with at least 1 patient aged <5 years with SCD. Physician race and practice specialty were significantly associated with factors physicians considered very important to adherence.Conclusions: Most physicians agree on many issues that affect adherence; however, significant and important differences exist, based on physician ethnicity. Physicians' perceptions of factors that affect adherence in this study did not always agree with factors demonstrated to actually affect adherence in SCD patients. Therefore, this study indicates a need for physician continuing-education programs that focus on factors that actually influence adherence of antibiotic prophylaxis and the racial/ethnic backgrounds of the providers in relation to the patient.     

Psychological Characteristics and Pain Frequency Are Associated With Experimental Pain Sensitivity in Pediatric Patients With Sickle Cell Disease

Sickle cell disease (SCD) is associated with episodes of severe vaso-occlusive pain beginning in infancy with a subset of patients with SCD transitioning to chronic pain. Response to experimental pain using quantitative sensory testing in these patients suggests altered pain processing. The objectives of this study were to characterize sensitivity to multiple modalities of experimental pain stimuli and to interrogate the relationship of psychological covariates, clinical pain burden, and pain-related outcomes to experimental pain sensitivity in children with SCD compared with healthy individuals of similar age and sex. Cross-sectional assessments of psychological characteristics were performed, and quantitative sensory testing methods were used to measure experimental pain sensitivity in children age 8 to 21 years. Anxiety, depressive symptoms, catastrophizing, and somatization were found to be associated with increased sensitivity to experimental pain stimuli. Increased frequency of painful episodes in SCD was associated with decreased sensitivity to heat pain and decreased mechanical temporal summation. These data suggest that careful consideration be given to psychological factors, age, sex, and clinical burden of pain when studying response to experimental pain in SCD.

Emergency Department Management of Acute Pain Episodes in Sickle Cell Disease

Objectives To characterize the initial management of patients with sickle cell disease and an acute pain episode, to compare these practices with the American Pain Society Guideline for the Management of Acute and Chronic Pain in Sickle-Cell Disease in the emergency department, and to identify factors associated with a delay in receiving an initial analgesic.
Methods This was a multicenter retrospective design. Consecutive patients with an emergency department visit in 2004 for an acute pain episode related to sickle cell disease were included. Exclusion criteria included age younger than 18 years. A structured medical record review was used to abstract data, including the following outcome variables: analgesic agent and dose, route, and time to administration of initial analgesic. Additional variables included demographics, triage level, intravenous access, and study site. Mann–Whitney U test or Kruskal–Wallis test and multivariate regression were used to identify differences in time to receiving an initial analgesic between groups.
Results There were 612 patient visits, with 159 unique patients. Median time to administration of an initial analgesic was 90 minutes (25th to 75th interquartile range, 54–159 minutes). During 87% of visits, patients received the recommended agent (morphine or hydromorphone); 92% received the recommended dose, and 55% received the drug by the recommended route (intravenously or subcutaneously). Longer times to administration occurred in female patients (mean difference, 21 minutes; 95% confidence interval = 7 to 36 minutes; p = 0.003) and patients assigned triage level 3, 4, or 5 versus 1 or 2 (mean difference, 45 minutes; 95% confidence interval = 29 to 61 minutes; p = 0.00). Patients from study sites 1 and 2 also experienced longer delays.
Conclusions Patients with an acute painful episode related to sickle cell disease experienced significant delays to administration of an initial analgesic.     

犬失血性休克及补液过程中肾动脉多普勒血流速度频谱形态、阻力指数、搏动指数的变化

为探讨失血性休克及补液治疗过程中肾动脉多普勒血流速度频谱形态、RI、PI的变化规律及应用价值，本研究将10条太经股静脉放血至休克状态。然后行补液治疗。在实验过程中按失血量定时检测犬肾动脉血流速度频谱，计算RI、PI值，并记录同步血压。结果：（1）失血过程中．肾动脉RI、PI值随着失血量的增加，血压的下降．总体呈现上升趋势。随着补液的进行、MAP逐渐回升，RI、PI逐渐下降；（2）频谱形态出现一些特民改变，如重度失血时频谱舒张期出现小峰及凹陷．补液早期频谱舒张期凸起等。结论：多普勒指标RI、PI能较好地反映肾动脉阻力情况，肾动脉RI、PI可作为临床大出血患者病情观察和疗效判断的实用指标。     

Doppler Waveforms and Blood Flow Parameters of the Superior and Inferior Mesenteric Arteries in Patients Having Behçet Disease With and Without Gastrointestinal Symptoms

Objective . To evaluate hemodynamic changes in mesenteric arteries in patients with Behçet disease with and without gastrointestinal symptoms. Methods . Doppler sonography of mesenteric arteries was performed in 25 symptomatic and 15 asymptomatic patients having Behçet disease and in 25 healthy control subjects. The peak systolic, minimal, and mean velocities, resistive and pulsatility indexes, inner diameter, cross‐sectional area, and blood flow volume of mesenteric arteries were evaluated. The results were compared between patient groups and controls. Results . The mesenteric artery flow was significantly greater in patients in the symptomatic group than in those in the asymptomatic group or in controls. In the superior mesenteric artery, mean velocity and mean blood flow volume (0.35 ± 0.18 m/s and 711 ± 404 mL/min, respectively; P < .0001) in the symptomatic group were significantly higher than in the asymptomatic group (0.16 ± 0.07 m/s and 305 ± 168 mL/min, respectively) or in controls (0.15 ± 0.07 m/s and 290 ± 123 mL/min, respectively). The mean peak systolic velocity (1.23 ± 0.47 m/s; P < .005) in the symptomatic group was significantly higher than in controls (0.93 ± 0.23 m/s). In the inferior mesenteric artery, mean velocity and mean blood flow volume (0.25 ± 0.10 m/s and 139 ± 79 mL/min) in the symptomatic group were significantly higher than in the asymptomatic group (0.16 ± 0.07 m/s; P < .006; and 78 ± 26 mL/min; P < .007) or in controls (0.17 ± 0.07 m/s; P < .003; 83 ± 48 mL/min; P < .004). Conclusions . In this study, symptomatic patients with gastrointestinal Behçet disease were associated with a significant increase in mesenteric artery flow that could be evaluated easily on spectral patterns of arteries during Doppler sonography. The Doppler sonographic findings also revealed that intestinal involvement in patients with Behçet disease without gastrointestinal symptoms is not significantly different from that of healthy controls.