Differential Iron Loading of R2* Pancreas in Children with Thalassemia Major and Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 3817-3817
Author(s):  
Jhansi L. Papudesi ◽  
Shahriar Mokrian ◽  
Thomas D. Coates ◽  
John C. Wood
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pulse Sequence ◽  
Thalassemia Major ◽  
The Body ◽  
P Value ◽  
Cell Disease ◽  
Liver Iron ◽  
Patient Demographics ◽  
Control Subjects

Abstract MRI is a noninvasive method for detection of transfusional siderosis in the liver and heart of children with thalassemia major (TM) and sickle cell disease (SCD). Previous studies from our laboratory demonstrated that cardiac siderosis and cardiac failure are relatively uncommon in chronically transfused SCD, despite heavy somatic iron burdens. Others have reported correspondingly low rates of endocrinopathies. We used the MRI relaxation rate R2* as a surrogate for pancreatic iron to test the hypothesis that TM patients develop greater pancreatic iron load than SCD patients having comparable liver iron burdens. Methods We studied 70 TM and 31 chronically-transfused SCD patients. Pancreatic R2* was assessed in several mid pancreatic slices using a multi-echo, gradient echo pulse sequence with 8 echo times spaced from 2 to 16 ms. Pixelwise, monoexponential R2* reconstruction was performed with offset correction. Mean R2* was calculated from a region of interest (ROI) drawn around the body and tail of the pancreas. Control values for pancreatic R2* were derived from 14 normal volunteers, ages 19–31. Results Patient demographics are summarized in Table 1. TM patients were an average of 5 years older but less heavily iron loaded (liver iron 5 mg/g lower and ferritin 1800 ng/mL lower). Despite this, transferrin saturations trended higher in the TM patients (76% versus 58%). SCD patients had higher high sensitivity CRP and white blood cell count, consistent with greater inflammation. Figure 1 demonstrates pancreatic R2* distribution in TM, SCD, and control subjects; dotted line indicates the 95th confidence interval for the control subjects. Pancreatic R2* was elevated in 74% of the TM patients (R2* 148.9±200.9), compared with 48% of the SCD patients (R2* 39.6±37, p<0.021). In the TM patients, pancreatic R2* was correlated to cardiac R2*, but not to liver R2*. Discussion Pancreatic R2* is more common and more severe in TM compared to SCD, consistent with previously reported endocrinopathy rates. The younger age (decreased transfusion exposure) of the SCD patients and their lower transferrin saturations (secondary to inflammation) probably contribute to this observation. Prospective trials are necessary to determine the functional correlations and predictive value of pancreatic R2* measurements in these populations. Patient Demographics Parameter Thalassemia (n=70) Sickle Cell Disease (n=31) P Value Age 19.4 ± 10 14.5 ± 5.6 0.013 Gender 40M, 30F 17M, 14F NS Height(cm) 145 ± 29 147 ± 20 NS Weight(kg) 45.4±17 43.8 ± 19 NS Reticulocyte 2.0±1.9 (n=34) 9.6 ± 7.8 <0.001 Iron(mcg/dL) 193±99 (n=64) 146 ± 89 0.003 Ferritin(ng/mL) 4064±5661 5905 ± 6320 0.15 Transferrin (mg/dL) 174±70 169 ± 87 NS HIC(mg/g) 10.9 ± 12.2 (n=68) 16.1 ± 11.9 (n=30) 0.06 Iron % Sat(%) 76 ± 39 (n=38) 58 ± 29 (n=14) 0.11 TIBC(mcg/dL) 255 ± 119 219 ± 104 (n=13) 0.06 AST(U/L) 62.7 ± 63 (n=34) 72 ± 71 NS ALT(U/L) 77.6 ± 94.4 (n=34) 72 ± 99 NS hs-CRP(mg/L) 1.6 ± 1.9 (n=46) 6.2 ± 7 <0.001 WBC 10 ± 7 12.5 ± 7 0.04 Figure Figure

Consequences and management of iron overload in sickle cell disease

Hematology ◽  
2013 ◽  
Vol 2013 (1) ◽  
pp. 447-456 ◽  
Author(s):  
John Porter ◽  
Maciej Garbowski
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Iron Overload ◽  
Sickle Cell ◽  
Iron Concentration ◽  
Thalassemia Major ◽  
Iron Loading ◽  
Cell Disease ◽  
Liver Iron Concentration ◽  
Liver Iron

Abstract The aims of this review are to highlight the mechanisms and consequences of iron distribution that are most relevant to transfused sickle cell disease (SCD) patients and to address the particular challenges in the monitoring and treatment of iron overload. In contrast to many inherited anemias, in SCD, iron overload does not occur without blood transfusion. The rate of iron loading in SCD depends on the blood transfusion regime: with simple hypertransfusion regimes, rates approximate to thalassemia major, but iron loading can be minimal with automated erythrocyte apheresis. The consequences of transfusional iron overload largely reflect the distribution of storage iron. In SCD, a lower proportion of transfused iron distributes extrahepatically and occurs later than in thalassemia major, so complications of iron overload to the heart and endocrine system are less common. We discuss the mechanisms by which these differences may be mediated. Treatment with iron chelation and monitoring of transfusional iron overload in SCD aim principally at controlling liver iron, thereby reducing the risk of cirrhosis and hepatocellular carcinoma. Monitoring of liver iron concentration pretreatment and in response to chelation can be estimated using serum ferritin, but noninvasive measurement of liver iron concentration using validated and widely available MRI techniques reduces the risk of under- or overtreatment. The optimal use of chelation regimes to achieve these goals is described.

scholarly journals Sickle Cell Disease: An Overview of Oral Health Considerations for Pediatric Patients

2021 ◽  
Vol 2 (3) ◽  
pp. 9-17
Author(s):  
Dafni Eleftherou ◽  
Aristidis Arhakis ◽  
Sotiria Davidopoulou
Keyword(s):  
Oral Health ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Genetic Disorder ◽  
The Body ◽  
Common Finding ◽  
Cell Disease ◽  
Preventive Dental Care ◽  
Periodontal Inflammation

Aim: This literature review aims to update the evidence for orofacial manifestations and current treatment recommendations for children and adolescents with sickle cell disease. Background: Sickle cell disease is a frequent hemoglobinopathy and a life-threatening genetic disorder. The lifelong condition is characterized by chronic hemolytic anemia and vaso-occlusive crisis that may occur in a variable range of clinical presentations in different regions of the body, including the oral cavity. Review results: This review explored the most common orofacial alterations of pediatric patients with SCD. Dental caries is a common finding in SCD pediatric patients, especially in those who are socio-economically vulnerable. Moreover, malocclusions occur in high prevalence in SCD pediatric patients. Other oral health complications seen in SCD patients include periodontal inflammation, bone changes, infections, mental nerve neuropathy, facial overgrowth, delayed tooth eruption, dental anomalies, pulp necrosis, soft tissue alterations and salivary changes. Dental infections may trigger a vaso-occlusive crisis leading the patient to a higher probability on arriving in hospital emergency departments and in need for further hospital admission to deal with the correlated complications. Thus, preventive dental care and non-invasive dental procedures are the principal focus in SCD patients in order to avoid possible subsequent complications. Conclusion: The review showed that in pediatric patients with SCD the risk for orofacial manifestations and complications depends not only on the presence of SCD but also on other confounding factors such as oral hygiene, diet habits and social conditions. Moreover, more well-designed epidemiological studies are necessary to assess the real link between SCD disease and its impact on stomatognathic health.

Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease

2002 ◽  
Vol 140 (6) ◽  
pp. 681-687 ◽  
Author(s):  
Dorothy J. VanderJagt ◽  
Paul Harmatz ◽  
Ajovi B. Scott-Emuakpor ◽  
Elliot Vichinsky ◽  
Robert H. Glew
Keyword(s):  
Body Composition ◽  
Sickle Cell Disease ◽  
Children And Adolescents ◽  
Sickle Cell ◽  
Bioelectrical Impedance Analysis ◽  
Bioelectrical Impedance ◽  
Impedance Analysis ◽  
The Body ◽  
Cell Disease

scholarly journals Immune regulation in chronically transfused allo-antibody responder and nonresponder patients with sickle cell disease and β-thalassemia major

2011 ◽  
Vol 86 (12) ◽  
pp. 1001-1006 ◽  
Author(s):  
Weili Bao ◽  
Hui Zhong ◽  
Xiaojuan Li ◽  
Margaret T. Lee ◽  
Joseph Schwartz ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Immune Regulation ◽  
Thalassemia Major ◽  
Cell Disease

Prevalence of ocular abnormalities in relation to sickle cell disease severity among children in South-western, Nigeria

2020 ◽  
pp. 112067212095761
Author(s):  
Oluwatoyin I. Oladimeji ◽  
Oluwagbemiga O. Adeodu ◽  
Oluwatoyin H. Onakpoya ◽  
Samuel A. Adegoke
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
The Body ◽  
University Teaching ◽  
Teaching Hospitals ◽  
Cell Disease ◽  
Cross Sectional ◽  
Ocular Abnormalities

Introduction: Sickle cell disease (SCD) ranks high among genetic disorders worldwide. It is characterised by repeated vaso-occlusion with resultant end-organ damage. This process can occur in all vascular beds in the body, including ocular blood vessels and may cause irreversible blindness in advanced stages. Little is known of the relationship between the prevalence of ocular abnormalities among children with SCD and their disease severity. Methods: A descriptive cross-sectional study was carried out at the Paediatric Haematology Clinics and the Eye Centre of the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. Children with SCD in steady state were recruited from the Haematology Clinics and examined for ocular abnormalities at the Eye Centre of the hospital. The subjects SCD severity grade was determined using a previously validated scoring system. Results: One hundred and twenty (120) children aged 5 to 15 years were examined. Of these, 72 had one or more ocular abnormalities giving the prevalence of ocular abnormalities among them to be 60.0%. Though a higher proportion of children with moderate disease, 23 (65.7%) of 35, compared to those with mild disease, 49 (57.6%) of 85 had ocular abnormalities, this difference was not statistically significant, p = 0.412. Conclusion: Ocular abnormalities among Nigerian children with SCD are common even in steady-state, but not significantly associated with disease severity. Periodic screening for ocular abnormalities should thus be done on them irrespective of disease severity.

scholarly journals 3458 Temporal Trends and Outcomes of Opioid Abuse among Adolescents & Young Adult Sickle Cell Disease Patients

2019 ◽  
Vol 3 (s1) ◽  
pp. 54-55
Author(s):  
Nnaemeka E Onyeakusi ◽  
Adebamike Oshunbade ◽  
Fahad Mukhtar ◽  
Adeyinka Adejumo ◽  
Semiu Gbadamosi ◽  
...  
Keyword(s):  
Young Adults ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Temporal Trends ◽  
Opioid Abuse ◽  
P Value ◽  
Total Charge ◽  
Inpatient Mortality ◽  
Cell Disease ◽  
Sickle Cell Crisis

OBJECTIVES/SPECIFIC AIMS: In this study, we aim to describe temporal trends in opioid abuse among adolescents and 11-21years and young adults 22-35years with Sickle cell disease hospitalized for sickle cell crisis. We also aim to evaluate clinical and healthcare utilization outcomes of opioid abuse in the same population. In addition, we hope to assess for difference in effect by age category. METHODS/STUDY POPULATION: Our study is a cross-sectional study of data secondarily sourced from the 2007-2014 National Inpatient Sample(NIS), a component of the Healthcare Utilization Project (HCUP). Variables were identified using ICD-9-CM codes. We selected inpatient stays for patients aged 11-35 years admitted for sickle cell crisis. Opioid abuse was the primary outcome of interest. Secondary outcomes were inpatient mortality, total charge, length of stay and select clinical outcomes. We analyzed our data for trends and outcomes. We performed trend analysis of prevalence rates between 2007-2014 by age categories. Propensity-Matched Score regression models were deployed to assess for associations between opioid abuse and outcomes while adjusting for relevant covariates. Sub-group analysis of opioid abuse by age was assessed for outcomes of interest. Trend analysis was performed on Joinpoint Software v4.6.0, (National Cancer Institute, NIH, Bethesda, MD). Outcome analysis was performed on SAS v9.4 (SAS Institute, Cary, NC). Statistical significance was set at 95% and p-value of 0.05, two-tailed. RESULTS/ANTICIPATED RESULTS: Of 86,827 inpatients admitted for sickle cell crisis, 2,363 (2.73%) had a diagnosis of opioid abuse while 84,464 (97.27%)did not abuse opioids. 27,004 (31.01%) of admitted patients were 11-21 years while 59,823 (68.99%) were 21-35 years. We found statistically significant APCs (Annual Percentage Change) showing increasing trends in both age categories for years under review, (18.47% [95% CI 15.39-21.63]; p-value <0.001 in young adults vs. 10.31% [95% CI 3.58-17.49]; p-value 0.009 in adolescents). The difference in APCs between both age categories were also significant (−8.16% [95% CI [−14.26-2.05]; p-value 0.009). There were no parallelism or coincidence in the trend lines. Opioid abuse was found to be associated with significantly longer length of stay (7.74 vs 6.05 days), higher total charge ($40,797 vs $32,164), (aOR 1.44; 95% CI [1.19-1.75]) seizures, sepsis (aOR 1.62; 95% CI [1.35-1.94]) and pulmonary hypertension (aOR 1.36; 95% CI [1.12-1.66]). No significant association was found for inpatient mortality, transfusion, cardiac dysrhythmias, pulmonary embolism and acute kidney injury. Significant interaction existed between opioid abuse and age for total charge (for $41,869 vs $29,371 among adolescents & $40,632 vs $32,550 among young adults; interaction p-value 0.03). DISCUSSION/SIGNIFICANCE OF IMPACT: Trends show a significant increase in the prevalence of opioid abuse among adolescents and an increasingly higher prevalence when adolescents transition to young adults. Opioid abuse among sickle cell patients is associated with significant poor healthcare resource utilization and clinical outcomes. Public health interventions to prevent worsening opioid abuse prevalence are expected to improve patient outcomes.

Genetic Polymorphisms Associated with Priapism in Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 789-789
Author(s):  
Laine Elliott ◽  
Allison E. Ashley-Koch ◽  
Jude Jonassaint ◽  
Jennifer Price ◽  
Jason Galloway ◽  
...  
Keyword(s):  
Genetic Variation ◽  
Linkage Disequilibrium ◽  
Sickle Cell Disease ◽  
Candidate Genes ◽  
Sickle Cell ◽  
P Value ◽  
Cell Disease ◽  
P Values ◽  
History Of ◽  
Male Patients

Abstract Priapism, a painful and prolonged erection, has been reported to occur in 30–45% of male patients with sickle cell disease (SCD). However, little is known about the pathological processes and genetic risk factors that contribute to the occurrence of priapism. The identification of genetic variables that are associated with priapism may therefore help define both critical pathophysiologic mechanisms not otherwise apparent, as well as patients at increased risk. We examined genetic variation in our sample of 199 unrelated, adult (&gt;18 years), male patients with Hb SS and Hb Sβ0-thalassemia, 83 (42%) of whom reported a history of priapism. Candidate genes for association with priapism were identified based on their involvement in adhesion, coagulation, inflammation, and cell signaling. Additionally, we examined genes involved in NO biology (NOS2, NOS3, SOD1, SLC4A1). Finally, we also examined polymorphisms in the KLOTHO gene, which has previously been associated with priapism. We examined a total of 389 SNPs in 48 candidate genes. Except for the gene encoding the β2 adrenergic receptor, SNP genotyping was performed by TaqMan, using Assays-on-Demand or Assays-by-Design genotyping products (Applied Biosystems). Allele tests were used to detect genetic associations with priapism. Strong evidence of association was found for SNP rs7526590 in the transforming growth factor-β receptor, type III (TGFBR3) gene (p=.00058), SNP rs10244884 in the aquaporin (AQP1) gene (p=.00068), and SNP rs3768780 in the integrin αV (ITGAV) gene (p=0.00090). A second ITGAV SNP (rs3768778), in linkage disequilibrium (r2=.59) with the first, also showed association with priapism (p=.00888). The A1 subunit of coagulation factor XIII (F13A1) had four SNPs (hcv1860621, rs1032045, rs1674074, rs381061) with p-values less than 0.010 (p-values = 0.00156, 0.00415, 0.00648, and 0.00712, respectively). The linkage disequilibrium among these F13A1 SNPs is negligible (r2 &lt;.15). We also adjusted for multiple testing using the Benjamini-Hochberg procedure (significance threshold &lt;.10). SNP rs7526590 in TGFBR3, SNP rs10244884 in AQP1 and SNP rs3768780 in ITGAV each had a false discovery rate (FDR) p-value of .09834. SNP rs1674074 in F13A1 had an FDR p-value of .12733. The other SNPs in F13A1 had large FDR p-values, close to .30. We did not detect an association between priapism and genetic variation in the Klotho gene, as was previously reported by Nolan et al. (2005). Specifically, SNPs rs2249358, rs211234 and rs211239 showed a virtually identical distribution of genotypes for individuals with and without a history of priapism. However, our population is not identical to the previous study, which included patients as young as 10 years old. In conclusion, our data support the hypothesis that genetic variation is associated with risk for priapism among males with SCD and suggest that genes involved in the TGFβ pathway, coagulation, cell adhesion and cell hydration pathways may be important.

Impact of Hydroxyurea on Thrombin Antithrombin Complex and Vaso-Occlusive Crisis in Pediatric Sickle Cell Disease.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5525-5525
Author(s):  
Mohsen Saleh Elalfy ◽  
Ashraf M. Abdelmonem ◽  
Soha Youssef ◽  
Heba Ismail
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Coagulation System ◽  
P Value ◽  
Cell Disease ◽  
Significant Difference ◽  
Healthy Control ◽  
Pain Crisis

Abstract Background: Several studies suggest that increased activity of the coagulation system may be important in the pathogenesis of vascular occlusion in sickle cell disease. Hydroxyurea (HU) has been shown to reduce the frequency of vaso-occlusive manifestations in both adults and children with sickle cell disease (SCD). Aim: To analyze the effect of HU on Thrombin-Antithrombin (TAT) as a marker of thrombin generation and hypercoagualbility in SCD and to find out the relation between TAT level and vaso-occusive crisis. Subjects and Method: we evaluated 37 child with sickle cell hemoglobinopathy (mean age 10.92±5.39 years) and 15 normal control children (mean age 9.75±6.34 years). Informed consent was obtained from patients and/or guardians and study approval by local IRB was obtained. Twenty-two patients (59.5%) were on HU, 15 (41.5%) patients did not receive HU, 7 (46.7%) of them were transfusion dependant. TAT assay was done in vitro using a sandwich enzyme immunoassay. Results: Mean patients’ age at institution of HU was 8.54± 3.85 years with median treatment duration of 4.5 years. Causes for initiating HU therapy were frequent blood transfusion in 11 patients (50%), frequent pain crisis (≥ 3/year) in 9 patients (41%), severe anemia and parents refusing blood transfusion in 1 patient (4.5%) and stroke in another patient (4.5%). HU dose was 20.82±4.95 mg/kg/day. We measured TAT in all patients and compared them to healthy control. There was significant difference in TAT level in sickle cell patients (198.86±185.7) compared to healthy control 2.91±0.94, [P value < 0.0001]. When the level of TAT was compared between the HU and non-HU groups we found that patients on HU had statistically significant lower TAT level (172.36 vs.225.37) [P=0.039]. There was also a significant negative correlation between HU dose and TAT level (p=0.03). A significant positive correlation between number of vaso-occlusive crisis/year [P=0.03], frequency of pain crisis/year [P=0.04], duration of pain crisis [P=0.03] and TAT level was observed. Conclusion: Hydroxyurea has significant inhibitory effect on thrombogenesis in sickle cell patients, which may be another mechanism for reducing vaso-occlusive crisis. Sickle cell children with higher TAT level had more frequent and severe vaso-occlusive crisis.

Neuropathic Vs. Nociceptive Pain in Adolescent Sickle Cell Disease (SCD) Evaluated by a Computer-Based Self-Assessment Pain Tool.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2576-2576 ◽  
Author(s):  
Richard J. Labotka ◽  
Robert E. Molokie ◽  
A. Kyle Mack ◽  
Alexis A Thompson ◽  
Young Ok Kim ◽  
...  
Keyword(s):  
Neuropathic Pain ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
The Body ◽  
Nociceptive Pain ◽  
Mcgill Pain Questionnaire ◽  
Cell Disease ◽  
Self Assessment ◽  
Body Segments ◽  
Computer Based

Abstract Abstract 2576 Poster Board II-553 Pain remains the most frustrating and debilitating symptom of sickle cell disease (SCD). Yet, because pain is a perception of a sensation, objective assessment of pain remains elusive. In order to achieve adequate pain control, it is imperative that the patient be able to effectively communicate with the physician regarding the extent, severity and quality of the pain, as well as the response of the pain to the therapeutic intervention. Moreover, SCD pain may have two components, nociceptive (due to organ/tissue injury) and neuropathic (due to somatosensory system lesion or disease), with differing pathophysiologies, and differing responses to pain medications. PAINReportIt® is a multi-dimensional computerized, self-assessment pain reporting tool based on the McGill Pain Questionnaire (MPQ). The MPQ has been validated for adolescents and adults. However, the PAINReportIt®, which was developed originally for the evaluation of cancer pain, has not previously been used in adolescents, nor in the study of adolescents' SCD pain. The purpose of this study was to examine the frequency with which pain experienced in various body areas by adolescents and young adults had characteristics consistent with nociceptive and neuropathic pain. Methods. Inclusion criteria included an SCD diagnosis (SS, SC or S-beta thal) and age at least 14 years. Patients attending two pediatric/adolescent comprehensive sickle cell clinics were invited to participate. Consenting subjects were first instructed and then allowed to complete the PAINReportIt® tool, in which they marked their painful sites on a graphic body outline, selected pain quality descriptors from word lists provided by the computer, and for each site they matched each site to the pain quality descriptors that represented the site. The descriptors included sensory descriptors that are known to be characteristic of either neuropathic pain or nociceptive pain. Results. PAINReportIt® tools were completed by 49 SCD subjects, whose ages ranged from 14 to 27 years (mean, 18 +/− 2.6 years). For analysis, the body was divided into nine segments, and the computer analyzed the marked painful body outline sites and identified the body segments involved. Descriptive statistics were used to determine the frequency for which each body segment included a painful site, and the frequencies for which that site was characterized as having neuropathic or nociceptive pain qualities, or both. These results are summarized in the Table below. As the Table shows, virtually all body segments were frequently involved with pain, the upper back and legs being the most frequently reported, and the lower back the least (Column 1). For body segments reported as painful, few were reported as having only nociceptive (Column 2) or neuropathic (Column 3) pain qualities. The majority of painful sites were described by subjects as having mixed pain qualities (Column 4). As Column 5 shows, from 76% to 100% of all painful sites were characterized as having a neuropathic pain component. When the number of descriptors associated with each site was reviewed (data not shown), the right leg was matched to the largest number of neuropathic descriptors (Aching, Burning, Cold, Drilling, Flickering, Numb, Penetrating, Radiating, Shooting, Spreading, Tight, Tingling). The upper back was matched to the largest number of nociceptive descriptors (Beating, Cramping, Crushing, Gnawing, Hurting, Piercing, Pounding, Pressing, Pulsing, Sharp, Sore, Splitting, Squeezing, Tender, Throbbing). Conclusions. When utilizing a computer-based self-reporting pain tool, SCD patients overwhelmingly describe a neuropathic component to their pain as well as a nociceptive component. The high frequency of neuropathic pain has been underappreciated, and this may contribute to the difficulty in managing sickle cell pain, since this pain component is not well controlled by opioid analgesics. Disclosures: No relevant conflicts of interest to declare.

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