Low‐risk factors for severe bacterial infection and acute chest syndrome in children with sickle cell disease

2019 ◽  
Vol 66 (6) ◽  
pp. e27667 ◽  
Author(s):  
Elena María Rincón‐López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández‐Sampelayo Matos ◽  
Jesús Saavedra‐Lozano ◽  
Yurena Aguilar de la Red ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Low Risk ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Severe Bacterial Infection

scholarly journals A Polymorphism in Toll-like Receptor 2 Gene Is Associated with Occurrence of Bacterial Infections in Sickle Cell Disease Patients

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3652-3652
Author(s):  
Karina Tozatto-Maio ◽  
Robert Girot ◽  
Indou Deme Ly ◽  
Vanderson Rocha ◽  
Ana Cristina Silva Pinto ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Bacterial Infections ◽  
Univariate Analysis ◽  
Genetic Models ◽  
Acute Chest Syndrome ◽  
Cell Transplant ◽  
Cell Disease ◽  
Functional Polymorphisms

Abstract Background: Sickle cell disease (SCD) patients (pts) are at increased risk of infection. This susceptibility is partially explained by the loss of spleen function, but other factors such as inflammatory status might also play a role. Despite adequate immunization and antimicrobial prophylaxis, bacterial infection remains a major cause of morbidity and mortality in SCD pts. Toll-like receptors (TLR), transmembrane proteins that recognize a wide range of pathogen molecular patterns, are key receptors in innate immune response. The association among TLR and infections in SCD has scarcely been explored. Objective: To analyze if functional polymorphisms in TLR genes can modulate the susceptibility to bacterial infections in SCD pts. Methods: Case-control retrospective study; 430 SCD pts followed in university hospitals in Brazil (n=196), France (n=180, mainly from Subsaharan Africa and French West Indies) and Senegal (n=54) were included. Sites of bacterial infection were lower respiratory system, abdominal, upper urinary system, bone/joints, central nervous system, blood stream, or any site for tuberculosis. For pts who underwent hematopoietic stem cell transplant (HSCT), only bacterial infections before HSCT were considered. All pts were tested for 7 single nucleotide polymorphisms (SNP) in TLR-1(rs4833095), TLR-2 (rs4696480, rs3804099, rs3804100), TLR-6 (rs5743810) and TLR-10 (rs11466653, rs11096957) using TaqMan 5'-nuclease assay. Univariate analysis was performed with chi-square or Mann-Whitney for categorical and continuous variables respectively. Associations were measured by odds ratio (OR). For SNP associations, significant P-value corrected by Bonferroni (cP) was 0.007; for genetic models, cP was 0.01. Results: Median age of cohort was 30 years (range: 2-70) and 87% were ≥18 years; 57% of pts were female. SCD genotype was SS in 81%, SC in 11%, SB in 7% and other in 1%. Fifty-eight percent received hydroxyurea and 79% received red blood cell transfusions; seven pts underwent HSCT. Eleven pts died during follow-up, mostly from acute chest syndrome and hemorrhagic stroke. All pts had access to immunization against encapsulated bacteria and to penicillin prophylaxis. One hundred ninety-five pts (45%) did not present any bacterial infection, whereas 235 (55%) pts presented at least one episode of bacterial infection, of which 20% had ≥ 3 episodes. Bacterial infections were mainly respiratory (51%), bone/joints (23%) and urinary (20%). In 106 cases, etiological agents were identified; most common agents were Escherichia coli, Streptococcuspneumoniae, Mycobacterium tuberculosis and Salmonella spp. In univariate analysis, there was no association among occurrence of infections and gender, origin and age distribution. Gene analysis: all tested SNPs had a typing rate >90% and minimum allele frequency >1%. The TLR-2 SNP rs4696480 (n=425) showed significant association with infection (p<0.001). Next, we tested genetic models for this association. Distribution of rs4696480 genotypes was: infected - AA=35%, TA=50%, TT=15%; non infected - AA=28%, TA=67%, TT=5%. In the overdominant model, the heterozygous genotype (TA) occurred significantly more in non infected (67%) than in infected pts (50%) compared with TT+AA (OR 0.5, 95%CI 0.34-0.75, P<0.001). Also, in the recessive model, genotype TT occurred significantly more in infected (15%) than in non infected pts (5%) compared with TA+AA (OR 3.18, 95%CI 1.53-6.61, P<0.001). Furthermore, in comparison with an African/African-American population described in the 1000 genomes database (n=661), TA genotype was more frequent in SCD pts, and TT genotype was significantly less frequent. Other TLR SNPs genotype and haplotype analysis did not show any significant association with occurrence of infections. Discussion: In SCD pts, TLR-2 rs4696480 TA genotype might be protective against bacterial infections, whereas TT genotype might increase risk of such infections. Previous reports demonstrated higher secretion of inflammatory factors in cells from AA individuals, lower occurrence and severity of immune diseases in T carriers. TA genotype might stand between deleterious effects of over inflammatory response (AA genotype) and under response (TT genotype) to infectious agents. Disclosures No relevant conflicts of interest to declare.

scholarly journals Acute Chest Syndrome in Children with Sickle Cell Disease in Italy: Results of a National Survey from the Italian Association of Pediatric Hematology Oncology (AIEOP)

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2301-2301
Author(s):  
Vania Munaretto ◽  
Raffaella Colombatti ◽  
Serena Ilaria Tripodi ◽  
Corti Paola ◽  
Simone Cesaro ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Exchange Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Italian Association ◽  
Pediatric Hematology ◽  
Frequent Event ◽  
Regional Hospitals

Background: Acute Chest Syndrome (ACS) is the second cause of hospitalization in Sickle Cell Disease (SCD), burdened by significant morbidity and mortality. The guidelines regarding management of ACS are sometimes difficult to follow in the real world and the prevention and treatment strategies of ACS are often applied in an uneven manner in the various settings (community care, regional hospitals, reference university centers). Moreover, epidemiology, clinical phenotype and outcomes as well as risk factors could vary in different populations according to ethnicity, genotype or health care system organization. Aims and Methods: A retrospective multicenter observational study was conducted to investigate the epidemiology of ACS and to the evaluate the diagnostic and therapeutic pathways of ACS in children with SCD (age 0-18 years) in the 2013-2018 period, after the publication of the Italian Association of Pediatric Hematology Oncology (AIEOP) Guidelines for the Management of SCD in Childhood in Italy in 2012. Results: 126 children were recruited and 122 included in the analysis, with 208 evaluable episodes of ACS (range: 1-6 episodes /patient) from 11 AIEOP Centers. 73 M, 49 F. Mean age was 10.9 years. 85% patients were of African origin, 92% were HbSS/SB°; mean age at diagnosis of SCD of the entire cohort was 25,3 months (range 0-16,8). 44.2% of patients had more than one episode of ACS during the study period; 37% had had a previous episode before 2013. 58% had comorbidities, mostly respiratory (asthma or allergy). 75% of the patients underwent disease modifying treatment during study period (73% hydroxyurea, 2% chronic transfusion). The seasonality of ACS episodes was important in our country: 75% of episodes occured between October and March. 95% of ACS episodes were secondary to a Vaso-Occlusive Crisis. 76% of the admissions occurred in SCD reference centers, 24% in regional hospitals, but 30% later required transfer to reference centers for worsening of clinical conditions or need of exchange transfusion. The mean length of hospitalization was 9.6 days (range 1-46); one patient died of pneumococcal sepsis; 6 episodes required transfer to the Intensive Care Unit, mechanical ventilation was required in one episode. A good adherence to the AIEOP Guidelines was documented for some aspects: 99% of the patients were hospitalized, 98% performed chest X-ray for the diagnosis of ACS and in 99% antibiotic therapy was started. Others aspects were less satisfactory and in need of improvement: incentive spirometry was only performed in 19% of admissions; oxygen therapy was performed only in 75% of patients even if SatO2 was<95%; transfer to reference centers was not always timely. During 75% of ACS episodes a simple red cell transfusion was required for Hb>8g/dl, while in 16% an exchange transfusion was performed for severe respiratory distress (of these 71% were performed in patients transfered from regional hospitals); 38% required inhaled bronchodilators, 6% steroids. A preliminay evaluation of risk factors for recurrent ACS showed that in our cohort allergy to inhaled allergens (p 0.02) and enuresis (p 0.01) were associated with increased prevalence of recurrent ACS; patients with asthma/wheezing also presented more recurrent ACS compared to patients wihout them (23% vs 13%) but this data did not reach statistical significance. Conclusion: This study represents the first analysis in Italy of ACS, which is confirmed as a frequent event in our cohort, with a significant proportion of patients who experience recurrent ACS. Steps need to be undertaken to improve management of ACS and adherence to the AIEOP guidelines at a national level: stimulate the application of early preventive measures that are still under-utilized, increase the appropriateness of multidisciplinary specialist approach (transfusion specialist, acute care physicians, pneumologists, hematologists) strengthen the dissemination of information through training events for all the Hospitals of the network. Disclosures Colombatti: AddMedica: Consultancy; Global Blood Therapeutics: Consultancy; Novartis: Consultancy.

scholarly journals Procalcitonin to Reduce Antibiotic Exposure during Acute Chest Syndrome in Adult Patients with Sickle-Cell Disease

2020 ◽  
Vol 9 (11) ◽  
pp. 3718
Author(s):  
Keyvan Razazi ◽  
Ségolène Gendreau ◽  
Elise Cuquemelle ◽  
Mehdi Khellaf ◽  
Constance Guillaud ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Major Complication ◽  
Adverse Outcomes ◽  
Acute Chest Syndrome ◽  
Antibiotic Exposure ◽  
Cell Disease ◽  
Intervention Phase ◽  
Entire Cohort

Acute chest syndrome (ACS) is a major complication of sickle-cell disease. Bacterial infection is one cause of ACS, so current guidelines recommend the routine use of antibiotics. We performed a prospective before–after study in medical wards and an intensive-care unit (ICU). During the control phase, clinicians were blinded to procalcitonin concentration results. We built an algorithm using the obtained measurements to hasten antibiotic cessation after three days of treatment if bacterial infection was not documented, and procalcitonin concentrations were all <0.5 μg/L. During the intervention period, the procalcitonin algorithm was suggested to physicians as a guide for antibiotic therapy. The primary endpoint was the number of days alive without antibiotics at Day 21. One-hundred patients were analyzed (103 ACS episodes, 60 in intervention phase). Possible or proven lung infection was diagnosed during 13% of all ACS episodes. The number of days alive without antibiotics at Day 21 was higher during the intervention phase: 15 [14–18] vs. 13 [13,14] days (p = 0.001). More patients had a short (≤3 days) antibiotic course during intervention phase: 31% vs 9% (p = 0.01). There was neither infection relapse nor pulmonary superinfection in the entire cohort. A procalcitonin-guided strategy to prescribe antibiotics in patients with ACS may reduce antibiotic exposure with no apparent adverse outcomes.

Risk factors for acute chest syndrome in children with sickle cell disease undergoing abdominal surgery

2004 ◽  
Vol 39 (6) ◽  
pp. 848-850 ◽  
Author(s):  
E.R. Kokoska ◽  
K.W. West ◽  
D.E. Carney ◽  
S.E. Engum ◽  
M.E. Heiny ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Abdominal Surgery ◽  
Sickle Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease

scholarly journals Risk Factors for Adverse Maternal and Fetal Outcomes in Pregnant Patients with Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3117-3117
Author(s):  
Sherraine Della-Moretta ◽  
William Marshall ◽  
Rui Li ◽  
Erin Cleary ◽  
Philip Samuels ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Research Funding ◽  
Maternal Age ◽  
Acute Chest Syndrome ◽  
Hypertensive Disorders Of Pregnancy ◽  
Cell Disease ◽  
History Of ◽  
Maternal Bmi

Abstract Background Approximately 100,000 Americans are affected by sickle cell disease (SCD), an inherited hematologic disorder. In women with sickle cell disease, pregnancy is associated with increased maternal and fetal adverse outcomes (Elenga et al). However, there is a paucity of data on risk factors for adverse events in this population. This retrospective study seeks to add to the deficient repertoire of information regarding maternal and fetal outcomes in patients with sickle cell disease and their children. Methods We retrospectively evaluated pregnancy outcomes of women with SCD who had previously undergone echocardiography from the year 2000-2021. The associations between clinical variables and adverse hematologic (AHE), cardiac (ACE), obstetric (AOE) and fetal/neonatal (ANE) events were evaluated by the Generalized Linear Model (GLM). The adverse hematologic events were vaso-occlusive crisis (VOC) antepartum and postpartum, acute chest syndrome, venous thromboembolism antepartum and postpartum, and transfusion antepartum. Results We identified 43 women/59 pregnancies with a median maternal age 27 years old (interquartile range [IQR] 20), pre-pregnancy BMI 25 kg/m2 (IQR 16). Maternal sickle cell genotype was SS in 31 (72%) women/37 (63%) pregnancies, SC in 8 (18%) women/18 (31%) pregnancies, and other genotype in 4 (9%) women/4 (7%) pregnancies. Prior venous thromboembolism was present in 12 (27%) women/15 (25%) pregnancies and prior acute chest syndrome (ACS) in 33 (80%) women/41 (75%) pregnancies. In the year before pregnancy, 24 (56%) women were admitted at least once for VOC. There were no maternal deaths during pregnancy or up to 1 year postpartum. AHE (n = 171) occurred in 43 (73%) pregnancies (Figure A), with a median of 2 (range 0-13) AHE per pregnancy. AHE were more common with genotype SS, history of ACS, history of ³ 10 lifetime transfusions, admission for VOC in the year before pregnancy, tricuspid regurgitation velocity (TRV) &gt;2.5 m/s on echocardiogram, and increased maternal age, and less common with increased hemoglobin (Figure B). ACE were rare (n = 3) (Figure A) and weakly associated with increased maternal age (Figure C). AOE (n = 37) occurred in 27 (45%) pregnancies (Figure A) and were associated with lower pre-pregnancy maternal BMI (Figure D). ANE (n = 54) occurred in 27 (46%) of pregnancies, and were associated with maternal hypertensive disorders of pregnancy (Figure E). Conclusions We found that AHE during pregnancy in women with SCD were associated with genotype SS, history of ACS, ³ 10 lifetime transfusions, admission for VOC in the year before pregnancy, higher maternal age, and inversely related to hemoglobin. In addition, AHE during pregnancy were associated with TRV &gt;2.5 m/s on echocardiogram, which has not been previously shown in women with SCD. These data may be useful to identify women at increased risk during pregnancy. Data show that patients with sickle cell disease who have more disease-related complications including history of acute chest syndrome, frequent pain crisis, elevated TRV on echocardiogram, and lower hemoglobin are at greater risk for AHE. This suggests that disease severity is directly related to outcomes. The association between increased maternal age and ACE has been demonstrated in the past in women without SCD (DeViti et al). The same can be noted for the association of AOE with lower maternal BMI (Verma et al), and ANE being associated with maternal hypertensive disorders of pregnancy (Lugobe et al). In the future, prevention of these complications will be key. Future directions include determining the effect of disease-modifying therapy on these outcomes, though safety during pregnancy has not yet been demonstrated for more novel agents such as voxelotor and crizanlizumab. With more information on these risk factors, we hope that modification and treatment can result in better outcomes. Figure 1 Figure 1. Disclosures Desai: Pfizer: Other: Publication Fee, Research Funding; Foundation for Sickle Cell Research: Honoraria; Forma: Consultancy; Novartis: Research Funding, Speakers Bureau; Global Blood Therapeutics: Honoraria, Research Funding.

scholarly journals Interleukin 6 as a marker of severe bacterial infection in children with sickle cell disease and fever: a case–control study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Elena María Rincón-López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández-Sampelayo Matos ◽  
David Aguilera-Alonso ◽  
Eva Dueñas Moreno ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Case Control Study ◽  
Case Control ◽  
Cell Disease ◽  
Severe Bacterial Infection ◽  
Tnf Α ◽  
Significant Difference ◽  
Control Study

Abstract Background Etiological diagnosis of fever in children with sickle cell disease (SCD) is often challenging. The aim of this study was to analyze the pattern of inflammatory biomarkers in SCD febrile children and controls, in order to determine predictors of severe bacterial infection (SBI). Methods A prospective, case–control study was carried out during 3 years, including patients younger than 18 years with SCD and fever (cases) and asymptomatic steady-state SCD children (controls). Clinical characteristics and laboratory parameters, including 10 serum proinflammatory cytokines (IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70, IL-17a, IFN-γ and TNF-α) and comparisons among study subgroups were analyzed. Results A total of 137 patients (79 cases and 58 controls) were included in the study; 78.5% males, median age 4.1 (1.7–7.5) years. Four cases were diagnosed with SBI, 41 viral infection (VI), 33 no proven infection (NPI) and 1 bacterial-viral coinfection (the latter excluded from the subanalyses). IL-6 was significantly higher in patients with SBI than in patients with VI or NPI (163 vs 0.7 vs 0.7 pg/ml, p < 0.001), and undetectable in all controls. The rest of the cytokines analyzed did not show any significant difference. The optimal cut-off value of IL-6 for the diagnosis of SBI was 125 pg/mL, with high PPV and NPV (PPV of 100% for a prevalence rate of 5, 10 and 15% and NPV of 98.7%, 97.3% and 95.8% for those prevalences rates, respectively). Conclusion We found that IL-6 (with a cut-off value of 125 pg/ml) was an optimal marker for SBI in this cohort of febrile SCD children, with high PPV and NPV. Therefore, given its rapid elevation, IL-6 may be useful to early discriminate SCD children at risk of SBI, in order to guide their management.

Interleukin 6 as a Marker of Severe Bacterial Infection in Children with Sickle Cell Disease and Fever: A Case-Control Study

2021 ◽  
Author(s):  
Elena María Rincón-López ◽  
María Luisa Navarro Gómez ◽  
Teresa Hernández-Sampelayo Matos ◽  
David Aguilera-Alonso ◽  
Eva Dueñas Moreno ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Bacterial Infection ◽  
Sickle Cell ◽  
Case Control Study ◽  
Case Control ◽  
Cell Disease ◽  
Severe Bacterial Infection ◽  
Tnf Α ◽  
Significant Difference ◽  
Control Study

Abstract BackgroundEtiological diagnosis of fever in children with sickle cell disease (SCD) is often challenging. The aim of this study was to analyze the pattern of inflammatory biomarkers in SCD febrile children and controls, in order to determine predictors of severe bacterial infection (SBI).MethodsA prospective, case-control study of febrile and steady-state SCD children was carried out during 3 years. Clinical characteristics and laboratory parameters, including 10 serum proinflammatory cytokines (IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70, IL-17a, IFN-γ and TNF-α) and comparisons among study subgroups were analyzed.ResultsA total of 137 patients (79 cases and 58 controls) were included in the study; 78.5% males, median age 4.1 (1.7-7.5) years. Four cases were diagnosed with SBI, 41 viral infection (VI) and 33 no proven infection (NPI). IL-6 was significantly higher in patients with SBI than in patients with VI or NPI (163 vs 0.7 vs 0.7 pg/ml, p < 0.001), and undetectable in all controls. The rest of the cytokines analyzed did not show any significant difference. The optimal cut-off value of IL-6 for the diagnosis of SBI was 125 pg/mL, with high PPV and NPV (PPV of 100% for a prevalence of 5, 10 and 15% and NPV of 98.7%, 97.3% and 95.8% for those prevalences, respectively).ConclusionWe found that IL-6 (with a cut-off value of 125 pg/ml) was an optimal marker for SBI in this cohort of febrile SCD children, with high PPV and NPV. Therefore, given its rapid elevation, IL-6 may be useful to early discriminate SCD children at risk of SBI, in order to guide their management.

Antecedent Transfusion and Primary Hemorrhagic Stroke in Adults with Sickle Cell Disease.

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1437-1437
Author(s):  
John J. Strouse ◽  
Joshua Field ◽  
Regina D. Crawford ◽  
Sophie Lanzkron
Keyword(s):  
Risk Factors ◽  
Ischemic Stroke ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Hemorrhagic Stroke ◽  
Neurological Deficits ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Elevated Systolic Blood Pressure

Abstract Primary hemorrhagic stroke is an uncommon but serious complication of sickle cell disease (SCD) with mortality from 20 to 65%. Proposed risk factors include previous ischemic stroke, aneurysms, low steady-state hemoglobin, high steady-state leukocyte count, acute chest syndrome, and transfusion. We performed a retrospective case-control study to evaluate risk factors for primary hemorrhagic stroke in adults (age &gt;18 years) with SCD from Johns Hopkins and Barnes- Jewish Hospitals and Duke University Medical Center from January 1989 to April 2008. Cases had SCD and intraparenchymal (IPH), subarachnoid (SAH), or intraventricular (IVH) hemorrhage confirmed by neuroimaging or analysis of cerebrospinal fluid; traumatic hemorrhages and hemorrhagic conversion of ischemic strokes were excluded. Controls had SCD and ischemic stroke (focal neurological deficits with corresponding cerebral infarcts by neuroimaging). Both were identified by searching the hospital discharge database using ICD-9 codes for acute stroke and SCD and reviewing divisional records. We compared continuous variables by Student’s t-test and calculated odds ratios with exact methods. We identified 19 cases (mean age 29 years, range 18 – 66, 42% male) and 18 controls (mean age 34 years, range 19 – 61, 39% male). Most cases (14/18) had sickle cell anemia (HbSS) and 22% had a prior overt stroke; controls had HbSS (9/17), HbSB0thalassemia (1), or HbSC (7) and 41% had a history of overt stroke. Cases presented with headache (89%) and seizure (37%) and less frequently hemiparesis (27%). Controls presented with hemiparesis (78%), headache (57%), and rarely seizure (11%). Twelve cases had IPH including those with extension to the ventricles (1), subarachnoid (2) or both (2); six had SAH including ventricular extension (1). Potential causes of hemorrhagic stroke included moyamoya (4), aneurysms (3), anticoagulation (1) and ateriovenous malformation (1). Four cases (21%) and no controls died during the initial hospitalization. More cases (82%) than controls (44%, P&lt;0.05) had elevated systolic blood pressure at the time of stroke. At steady-state, cases had lower hemoglobin (mean ± SEM 8.5 ± 0.6 g/dl vs. 9.7 ± 0.6 g/dl), lower blood pressures (systolic 121 ± 4 vs. 127 ± 6 mm Hg, diastolic 71 ± 4 vs. 72 ± 9 mm Hg) and higher platelet counts (399,231 ± 74,024/ul vs. 362,200/ul ± 39,927/ul) than controls, but these differences were not statistically significant. Mean hemoglobin concentration at the time of stroke increased 1.3 g/dl (19%) from steady-state in cases and 0.01 g/dl (2%) in controls (p&lt;0.05). Seven cases had simple transfusions (between 1 and 11 days before their primary hemorrhagic stroke) in preparation for surgery (3), and for aplastic crisis (1), bacteremia (1), acute renal failure (1), or suspected acute chest syndrome (1). Only 1 control was transfused; and 1 with HbSS had a hemoglobin of 14.5 g/dl at the time of stroke (from excessive erythropoietin administration). In this group of adults with SCD, primary hemorrhagic stroke was associated with antecedent transfusion. Identifiable causes include moyamoya from obstructive cerebral vasculopathy, aneurysms and other vascular malformations, and rarely coagulopathy. Mortality was similar to that previously described. The association of recent transfusion and cerebral vasculopathy with hemorrhagic stroke suggests caution in the use of simple transfusion in adults with SCD and moyamoya or cerebral aneurysms. Table 1: Associations with Primary Hemorrhagic Stroke Variable Odds Ratio (95% CI) P-value Genotype (HbSS vs. other) 3 (0.6 – 17) NS Moyamoya 5 (0.4 – 260) NS Transfusion in the last 14 days 13 (1.3 – 630) &lt;0.02 NSAID in the last 14 days 2.9 (0.3 – 36) NS

scholarly journals Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients

2019 ◽  
Vol 245 (1) ◽  
pp. 79-83
Author(s):  
Salam Alkindi ◽  
Said S Almufargi ◽  
Anil Pathare
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cardiovascular Response ◽  
Rare Complication ◽  
Acute Chest Syndrome ◽  
Mean Corpuscular Hemoglobin ◽  
Cell Disease ◽  
Laboratory Parameters ◽  
Blood Exchange

Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records. Specifically, the demographical, hematological, biochemical, and clinical parameters including complications attributable to priapism were studied. Means for continuous measures and independent t-test were used to evaluate the association between the parameter studied and the occurrence of priapism. Priapism occurrence was associated with low basal hemoglobin (Hb), along with an increase in other parameters such as white blood cell count (WBC) ( P = 0.010), platelet count ( P = 0.001), reticulocyte count ( P < 0.001), mean corpuscular volume (MCV) ( P < 0.001), and mean corpuscular hemoglobin (MCH) ( P < 0.001). Biochemical laboratory parameters revealed an association with an increased total bilirubin ( P < 0.001). Patients with priapism were more likely to have acute chest syndrome, stroke, pulmonary hypertension, asplenia ( P = 0.006), gallbladder stones, and consequently cholecystectomy. Blood exchange and Penile aspiration were the most frequent medical therapies ( P = 0.010 and P < 0.001 respectively). Patients with sickle cell disease who presented with priapism were young adults with low Hb, high WBC’s, platelets, reticulocytes, MCV, MCH, bilirubin, and LDH. These findings suggest an association of priapism with increased hemolysis. Impact statement Sickle cell disease is prevalent throughout the world with a 5.8% incidence of sickle cell trait among the Omani population, yet priapism was a relatively rare complication. Penile erection and detumescence are complex physiological processes, which require a delicate neurohormonal and cardiovascular response. In this cohort, young SCD patients developed priapism with a low incidence of complications. These patients had high WBCs, platelets, bilirubin, LDH, and reticulocytes with low hemoglobin as compared to controls, suggesting an increased hemolytic process. Favorable outcome was dependent on early intervention, with almost half of these patients needing surgical intervention.

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