The Value of Procalcitonin and Lipopolysaccharide Binding Protein to Differentiate the Fever In the Patients with Sickle Cell Anemia

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4818-4818
Author(s):  
Selma Unal ◽  
Ali Ertug Arslankoylu ◽  
Necdet Kuyucu ◽  
Gönül Aslan ◽  
Semra Erdogan

Abstract Abstract 4818 Objective: Differentional diagnosis of fever is very important in patients with sickle cell anemia (SCA) in order to prevent inappropriate antibiotic use, drug resistance and to shorten the hospitalization period. A reliable marker to be used in the differentional diagnosis of fever in these patients has not been defined yet. We aimed to evaluate the values of C Rective Protein (CRP), procalcitonin (PCT), and lipopolysaccharide binding protein (LBP) levels in the differentional diagnosis of fever in patients with SCA. Material and Methods: 86 children with SCA (40 males and 36 females, mean age of 9.6 ± 3.84, range: 1–18 years), (Group 1) and 49 healthy children as a control group (mean age: 8.8 ± 3.91, range: 1–18 years) (Group 2) were included in this study. Patients who had admitted to the emergency department for concurrently vasoocclusive crisis and fever (axillary temperature ≥38C°) were classified as Group 1A and who had vasoocclusive crisis but no fever were classified as Group 1B and the patients without fever and vasoocclusive crisis were classified as Group 1C. A detailed history was taken from every child, and a full physical examination was performed. The patients who had taken antibiotics in last one week were excluded. The type of vasoocclusive crisis were recorded in Group 1A and 1B patients. In Group 1A patients, the fever was evaluated with appropriate laboratory tests (WBC count, periferic blood smear, serum CRP level, urinary test, chest radiography, blood and urinary culture). No infection focus was identified in patient and conrol groups. The WBC count, serum CRP, PCT and LBP levels were evaluated in all the patient and control groups. Results: The median CRP level of all patient groups was significantly higher than the control group (0.78 mg/L; range 0.21–70.0) (p<0.0001). The median CRP level in Group 1A (7.42 mg/L) and Group 1B (6.94 mg/L) were significantly higher than group 1C patients (2.24 mg/L). There were no significant difference between the SCA groups considering median serum PCT levels (Group 1A: 0.18 ng/ml, Group 1B: 0.11 ng/ml, Group 1C: 0.13 ng/ml, p>0.05). These values were significantly higher than control group (0.08 ng/ml) (p<0.0001). LBP level in Group 1A (median:11.5 μg/ml) was significantly higher than Group 1B (median: 8.9μg/ml), Group 1C (median: 7.7μg/ml) and control group (median:5.9μg/ml) (p<0.0001). Also serum LBP in both Group 1B and Group 1C were higher than control group (p<0.0001). Conclusion: PCT level is not affected from the acute inflamation originates from the vasoocclusive crisis. Thus, serum PCT level can be considered a good marker in the differentional diagnosis of fever in patients with SCA. Disclosures: No relevant conflicts of interest to declare.

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 975-975
Author(s):  
Thais Helena Chaves Batista ◽  
Rodrigo Marcionilo Santana ◽  
Marcondes José de Vasconcelos Costa Sobreira ◽  
Gabriela da Silva Arcanjo ◽  
Diego Arruda Falcao ◽  
...  

Introduction: Leg ulcers (LUs) are a cutaneous complication of sickle cell anemia (SCA), whose etiology is considered multifactorial. In the search for new candidates for modulators of SCA clinical events, recent evidence suggests the significant role of mechanisms related to post-transcriptional regulation, especially microRNAs (miRNAs). Thus, the analysis of miRNAs miR-21 and miR-130a differential expression in patients with SCA becomes an interesting approach, since both act in the regulation of several biological mechanisms related to the pathophysiology of LU, especially the tissue repair process. In addition, these miRNAs have already been related to the regulation of serum leptin levels, a strong angiogenic pleiotropic hormone that acts in the healing process of skin lesions. Therefore, the aim of the study was to investigate the influence of miR-21 and miR-130a and serum leptin levels on the development of LUs in SCA patients. Methods: After analyzing medical records, 60 SCA patients were selected. Patients who presented some of the main clinical manifestations that may have etiology due to the underlying disease (for example: osteonecrosis, stroke, priapism and acute chest syndrome) were not included. Patients with a history of LU were considered cases, and those who did not develop this complication (n=20), were considered control (median age: 26 years, range: 19-61, 50% males). The control group was called "HbSS-Control" and the case group was divided into two subgroups: Active leg ulcer group, composed of 19 patients with active LU at the time of blood collection (median age: 35 years, range: 24-56, 68% males), and healed leg ulcer group, composed of 21 patients with healed LU at the time of blood collection (median age: 34 years, range: 22-52, 43% males). In addition, it was analyzed a group of 10 donors with normal hemoglobin profile (median age: 25 years, range: 20-30, 50% males), identified as "HbAA-Control". Expression levels of miRNAs extracted from peripheral blood, using mirVanaTM PARIS Kit (Invitrogen™) were evaluated by RT-qPCR technique utilizing TaqMan® probes. Serum leptin levels of the patients were evaluated employing the ELISA method (Human Leptin ELISA Kit, Millipore®). Mann-Whitney and Kruskal-Wallis tests were applied to compare continuous variables. Results: Up-regulation of both miRNAs was observed in the active leg ulcer group in contrast to the healed leg ulcer (miR-21: P&lt;0.0001, Figure 1A, Fold change [FC]=14,2; miR-130a: P=0.0004, FC=18,8, Figure 1B) and Control-HbSS groups (miR-21: P&lt;0.0001, FC=34,4, Figure 1A; miR-130a: P=0.0006, FC=15,3, Figure 1B) and the HbAA-Control group (miR-21: P&lt;0.0001, FC=5,8, Figure 1C; miR-130a: P=0.0009, FC=10,9, Figure 1D). However, there was no significant difference between the healed leg ulcer, HbSS-Control and HbAA-Control groups (miR-21: P=0.1829, Figure 1E; miR-130a: P=0.3537; Figure 1F). Furthermore, the active leg ulcer group had lower serum leptin levels when compared to the healed leg ulcer and Control-HbSS groups (P=0.0058; Figure 2A). The levels of leptin in the healed leg ulcer group did not differ from the Control-HbSS group (P=0.5929; Figure 2B). Conclusion: Our results demonstrated an inverse relation between the miRNAs miR-21 and miR-130a expression with serum leptin levels, suggesting that the up-regulation of these miRNAS may be related to the chronicity and healing of LUs in individuals with SCA through decreased of serum leptin levels. Disclosures No relevant conflicts of interest to declare.


2009 ◽  
Vol 55 (2) ◽  
pp. 295-304 ◽  
Author(s):  
Tanja Vollmer ◽  
Cornelia Piper ◽  
Knut Kleesiek ◽  
Jens Dreier

Abstract Background: Infectious endocarditis (IE) is a bacterial infection of the endocardium. Diagnosis is based on results obtained from echocardiography, blood cultures, and molecular genetic screening for bacteria and on data for inflammatory markers such as the leukocyte (WBC) count and the C-reactive protein (CRP) concentration. The aim of the present study was to evaluate lipopolysaccharide-binding protein (LBP) as a supportive biomarker for the diagnosis and therapeutic monitoring of IE. Methods: We measured LBP and CRP concentrations and WBC counts in 57 IE patients at hospital admission, 40 patients with noninfectious heart valve diseases (HVDs), and 55 healthy blood donors. The progression of these 3 markers and the influence of cardiac surgery on them were evaluated in 29 IE patients and 21 control patients. Results: Serum LBP concentrations were significantly higher in IE patients [mean (SD), 33.41 (32.10) mg/L] compared with HVD patients [6.67 (1.82) mg/L, P &lt; 0.0001] and healthy control individuals [5.61 (1.20) mg/L]. The progression in the LBP concentration during therapy of IE patients correlated with the changes in the CRP concentration. The 2 markers were equally influenced by antibiotic treatment and surgical intervention. Conclusions: Serial LBP measurement may provide an effective and useful tool for evaluating the response to therapy in IE patients. We found a strong correlation between LBP and CRP concentrations; LBP has a tendency to increase earlier in cases of reinfection.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1390-1390
Author(s):  
Ashutosh Lal ◽  
Kristen Yen ◽  
Lasandra Patterson ◽  
Alisa Goldrich ◽  
Anne M Marsh ◽  
...  

Abstract Background: Carbon monoxide (CO) produced during oxygen-dependent cleavage of porphyrin ring of heme is excreted in exhaled breath. The catabolism of heme is increased when red blood cells are destroyed at an accelerated rate. Thus, quantifying CO in exhaled breath could serve as an indicator of hemolysis. However, the requirement for forced breath sample has limited the measurement of exhaled CO in young children. Objective: To assess end-tidal CO concentration (ETCOc) in children with sickle cell anemia (SCA). Design/Methods: ETCOc was measured using the CoSense ETCO Monitor (Capnia Inc. Palo Alto, CA). Children between 5-14 years with SCA (Hb SS) who were not on chronic transfusions were eligible. Healthy children served as age-matched controls. Children with exposure to second-hand smoke, acute respiratory infection or symptomatic asthma were excluded. End-tidal breath samples were collected by placing the tip of a nasal cannula 5 mm into the nares. Up to 3 measurements were taken for each subject and the highest ETCOc value was used for analysis. (ClinicalTrials.gov: NCT01848691) Results: The mean (range) age of 16 children with SCA and 16 controls was 9.7 years (5-14 years) and 9.9 years (5-14 years), respectively. The mean (± s.d.) ETCOc for SCA was 4.85 ± 2.24 ppm versus 0.96 ± 0.54 ppm for control group (p<0.001). The ETCOc in the control group ranged from 0.2 to 2.3 ppm, but was ≤1.2 ppm in 14/16, which is suggested as the upper limit of normal for healthy children. In the SCA group, the ETCOc range was 1.8 to 9.7 ppm, with values ≥2.4 ppm in 15/16 subjects. A threshold ETCOc value of >2.1 ppm provided both sensitivity and specificity equal to 93.8% (69.8-99.8%) for distinguishing SCA from healthy children. Children with SCA who had higher absolute reticulocyte count also demonstrated higher ETCOc (r=0.62, p=0.011). Patients with severe anemia (hemoglobin <8 g/dL) had a higher mean ETCOc (5.43 ppm) than the rest (4.40 ppm) but the difference was not significant. ETCOc level tended to increase with age in SCA (r=0.45, p=0.08). Conclusions: Carbon monoxide in exhaled breath can be measured in young children in the clinic using a portable monitor. ETCOc may be a valuable tool for non-invasive monitoring of the severity of hemolysis in SCA. The mean ETCOc was 5-fold higher in SCA compared with controls, with little overlap seen between the groups. This suggests a potential use for ETCOc as a point-of-care screening test for SCA in children. Figure 1 Figure 1. Figure 2 Figure 2. Disclosures Lal: Capnia, Inc: Research Funding. Yen:Capnia, Inc. : Employment. Bhatnagar:Capnia, Inc: Employment.


2020 ◽  
Vol 6 (4) ◽  
pp. 304
Author(s):  
Sigrid Bülow ◽  
Robert Heyd ◽  
Martina Toelge ◽  
Katharina U. Ederer ◽  
Annette Schweda ◽  
...  

Early diagnosis of invasive pulmonary aspergillosis (IPA) is crucial to prevent lethal disease in immunocompromized hosts. So far, lipopolysaccharide binding protein (LBP) and bactericidal/permeability-increasing protein (BPI) levels have not been evaluated as biomarkers for IPA. IL-8, previously introduced as a biomarker for IPA, was also included in this study. Bronchoalveolar lavage fluid (BALF) of IPA patients and control patients with non-infectious lung disease was collected according to clinical indications. Measurements in BALF displayed significantly higher levels of LBP (p < 0.0001), BPI (p = 0.0002) and IL-8 (p < 0.0001) in IPA compared to control patients. Receiver operating characteristic curve analysis revealed higher AUC for LBP (0.98, 95% CI 0.95–1.00) than BPI (0.84, 95% CI 0.70–0.97; p = 0.0301). Although not significantly different, AUC of IL-8 (0.93, 95% CI 0.85–1.00) also tended to be higher than AUC for BPI (p = 0.0624). When the subgroup of non-hematological patients was analyzed, test performance of LBP (AUC 0.99, 95% CI 0.97–1.00), BPI (AUC 0.97, 95% CI 0.91–1.00) and IL-8 (AUC 0.96, 95% CI: 0.90–1.00) converged. In conclusion, LBP and—to a lesser extend—BPI displayed high AUCs that were comparable to those of IL-8 for diagnosis of IPA in BALF. Further investigations are worthwhile, especially in non-hematological patients in whom sensitive biomarkers for IPA are lacking.


Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4628-4628
Author(s):  
Cem Kurt ◽  
Ilgen Sasmaz ◽  
Bulent Antmen ◽  
Yurdanur Kilinc ◽  
Sadi Kurdak ◽  
...  

Abstract Abstract 4628 Aim In this study we evaluated to pulmonary functions and determined relations of these findings with clinical parameters in children with sickle cell anemia (SCA) who were at follow up in our pediatric heamatology clinic. Materials and Methods 24 children with sickle cell anemia and 9 healthy children as control group include to the study. Complete blood count, hemoglobin electrophoresis and biochemical values were eveluated for both groups. At pulmonology department, the carbonmonoxide diffusion test performing for both groups. At the same day spirometric respiratory function evaluation and exercise test performed both groups at department of sports physiology. The data recieved are compared statistics. Results HbS, HbF, SGPT, ferritin, total bilirubine, direkt bilirubine and Fe++ values were high at patient group (p<0.05). Hemoglobin and hematocrit values were low at patient group according to control group as expected (p<0.05). The number of SCA patient who had one-three venoocclusive crises (VOC) were 14 (58.3%), patient who had three or more VOC were 7 (29.2%) and patient who had no VOC were 3 (12.5%). The number of patient who had acute chest syndrome (ACS) were 5 (20.9%) and 19 patients had no ACS (79.1%). Ýmpaired isole carbonmonoxide diffusion test was established at the 62.5% of the patient's. At patient group, spirometric FEV1 and MEF25 measurement were found lower than the control group (p<0.05). At exercise test VO2/HR rate were lower for patient group (p<0.05). VE/VO2 rate (p=0.023) and R (p=0.016) measurement were found higher. Conclusion Pulmonary gas transfer was found difficult in patients with SCA. Respiratory airways established obstructed in spirometric evaluation. Obstructive defficiensies have to be follow up. Oxygen pulse and respiratory exchange rates were determined low and more oxygen usage was observed for aerobic metabolic activity. With these results, ýt can be say that chronic inflamation process at lung due to oxygen radicals and hipoksemia in sickle cell patients, the aerobic respiratory load was increased. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2210-2210
Author(s):  
Adam M Bush ◽  
Matthew Borzage ◽  
Thomas Coates ◽  
John C. Wood

Abstract Neurovascular sequalae are a common occurrence in Sickle Cell Anemia (SCA), with 11% of patients suffering cerebral vascular accident (CVA), or overt stroke by their twentieth birthday and 40% of patients developing silent cerebral infarcts (SCI) by age 14. Elevated transcranial Doppler (TCD) velocity of the middle cerebral artery identifies patients at risk for stroke, and this risk can be reduced by chronic transfusion therapy. However, the specificity of TCD is low causing many patients to be transfused unnecessarily. To further refine cerebrovascular risk stratification, we are studying factors responsible for normal and pathophysiologic cerebral blood flow (CBF) in SCD patients. Cerebral blood flow is increased in SCA patients compared to controls, but is believed to be a compensatory mechanism for chronic anemia and systemic desaturation. In order to test this hypothesis, we studied whole CBF and oxygen delivery (DO2) at rest and in response to hyperoxia in subjects with SCA and sickle cell trait (SCT). All patients were recruited at Children's Hospital Los Angeles through an IRB approved protocol. Informed consent was obtained for all patients. Exclusion criteria included pregnancy, previous stroke, acute chest or pain crisis hospitalization within one month. EKG, peripheral arterial oxygen saturation (SaO2), and fractional inspired oxygen (fiO2) were measured continuously throughout the study. Imaging consisted of a survey, reference scan, angiography localization, and nine axial phase contrast (PC) images. PC slices were placed inferior to the Circle of Willis, perpendicular to the carotids and basilar arteries. Participants received room air and 100% O2 through a non rebreathing respiratory circuit at 10 L/m. Room air exposure 20 minutes with six PC images collected throughout. Oxygen was delivered for 5 minutes followed by three more PC images. Blood for hemoglobin (HGB) and hematocrit (HCT) were drawn prior to MRI testing. No adverse events were reported upon follow up. Nine patients with SCA (5 male, 22.5 ± 6.7 yo p<0.05) and 3 with sickle cell trait (2 male, 36.7 ± 8.7 yo p<0.05) were studied. Baseline HGB was 25% lower (9.7 ± 1.3 versus 12.9 ± 0.1) and baseline HCT was 41% lower (28.0 ± 3.6 versus 47.3 ± 0.14 SCT) in SCA patients (p<0.05). Baseline whole brain CBF was elevated in SCA (1398 ± 400 ml/min versus 700 ± 172 ml/min). After correcting for HGB and SaO2, DO2 remained higher in SCA (192 ± 75 ml/min versus 105 ± 1.4 ml/min, p<0.05). During hyperoxia, whole brain CBF decreased by 10-15%, but the change was proportional to increased oxygen carrying capacity such that there was no change in DO2 in either populations. The increase in CBF we observed in SCA patients has been described using other modalities. However, we are the first to demonstrate that the increased flow observed is almost double what can be explained by their anemia and arterial desaturation alone. There are several possibilities for this observation. 1) SCA patients could have increased cerebral metabolic rate. If so, their mixed cerebral venous saturation will be normal and the flow is appropriate; 2) SCA patients have a mismatch between cerebral perfusion and metabolic demand, whether much of the increased flow does not effectively unload oxygen. If so, cerebral venous saturation would be high. We are currently optimizing MRI and NIR's technologies to estimate cerebrovascular metabolic rate, mixed cerebral venous saturation, and local supply-demand balance. Figure 1 Baseline CBF and O2 delivery in SCA and control group. ** statically significant population difference Figure 1. Baseline CBF and O2 delivery in SCA and control group. ** statically significant population difference Figure 2 Responce to 100% oxygen exposure. *statistically significant change from baseline Figure 2. Responce to 100% oxygen exposure. *statistically significant change from baseline Disclosures: Coates: Novartis Inc.: Honoraria, Speakers Bureau; Apopharma: Honoraria, Speakers Bureau; Shire: Speakers Bureau. Wood:Shire: Consultancy, Research Funding; Apopharma: Honoraria, Patents & Royalties; Novartis: Honoraria.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4681-4681
Author(s):  
Anil Atmis ◽  
Ilgen Sasmaz ◽  
Bulent Ali Antmen ◽  

Introduction Sickle cell anemia is a disease which is characterized with hemolytic anemia, hypercoagulopathy and painful crisis. Microparticles are 0,1-1 µm sized little membrane particles which are derived during activation or apoptotic phase of cell cycle. It is reported that microparticles are increased in many systemic disease including sickle cell anemia. Aim In this study we aimed to investigate the role of microparticles on during crisis and non-crisis periods in sickle cell anemia patients. Materials and method Twenty nine patients, following by Cukurova University, Department of Pediatric Hematology, are included in this study. Blood samples were collected in 26 of these patients in non-crisis period. Control group formed with 18 healthy children without any systemic disease. Complete blood count, hemoglobin electrophoresis and biochemical parameters were studied in both groups. Also patients’ total microparticle levels, erythrocyte (CD235a), endothelial (CD106), monocyte (CD14) particle levels and tissue factor expressing (CD142) microparticle levels were studied by flow cytometry and whole data was statistically analyzed. Findings Hemoglobin and hematocrit levels were significantly low in sickle cell anemia patients (p<0,001). Levels of HbS were significantly high during crisis period comparing with mean HbS levels during non-crisis period (p<0,001). Total microparticle levels were significantly high in sickle cell anemia patients with painful crisis comparing with control group (p<0,05). Erythrocyte and monocyte microparticle levels were significantly high in patients with painful crisis comparing with non-crisis periods (p<0,05). Endothelial and tissue factor expressing microparticle levels were high in patiens with crisis comparing to non-crisis period but this was not statistically significant (p>0,05). There was not any significant relation with frequency of crisis and microparticle levels (p>0,05). Microparticle levels were low in patients whose were taking hydroxiurea treatment comparing with non-hydroxiurea treatment but this data was not statistically significant (p>0,05). Result As a result we found high levels of total microparticle, erythrocyte and monocyte microparticles in sickle cell anemia patients during painful crisis period. This important clue of crises was need further studies in order to understand the effect of microparticles on pathophysiology of sickle cell anemia. Disclosures: No relevant conflicts of interest to declare.


2014 ◽  
Vol 6 (1) ◽  
pp. e2014044 ◽  
Author(s):  
Adel Abd elhaleim Hagag ◽  
Ghada Elmashad ◽  
Aml Ezzat Abd El-Lateef

Abstract: Background: Sickle cell disease has a worldwide distribution. Vaso-occlusive crisis (VOC) is one of the most important clinical features of the disease. Thrombospondin (TSP1) and Placenta growth factor (PlGF) have been reported to be involved in sickle cell diseases (SCD). Objective: The aim of this study is to assess TSP1 and PlGF levels in SCD patients. Patients and methods: This study was carried out in forty patients with sickle cell anemia who were attendants to Hematology units, Pediatric Departments, Tanta and Elmenofia University Hospital in the period between December 2011 and December 2013 including twenty patients with sickle cell anemia during vaso-occlusive crisis (twelve males and eight females) and twenty patients with sickle cell disease out of crisis (ten males and ten females). Also this study included twenty healthy children of matched age and sex as a control group. Serum TSP1 and PlGF levels were analyzed by ELISA. Results: Mean serum TSP1 levels were significantly higher in SCA patients with crisis than SCA patients out of crisis and were significantly higher in SCA patients with or without crisis compared to controls. Mean serum Placenta growth factor levels were significantly higher in SCA patients with crisis than SCA patients out of crisis and were significantly higher in SCA patients with or without crisis compared to controls. Conclusions: TSP1and PlGF concentration may be useful VOC markers in SCD patients. Recommendation: Further studies should be conducted to determine the exact point before VOC, when serum TSP1 and PIGF levels begin to increase. This requires monitoring of the TSP1 and PIGF levels in sickle cell patients out of crisis, showing how rapidly these levels increase just before VOC development.


2002 ◽  
Vol 9 (2) ◽  
pp. 440-445 ◽  
Author(s):  
Reinhard Berner ◽  
Birgitt Fürll ◽  
Felix Stelter ◽  
Jana Dröse ◽  
Hans-Peter Müller ◽  
...  

ABSTRACT No data on lipopolysaccharide-binding protein (LBP) in newborns with sepsis have been available up to now. We therefore determined levels of LBP and soluble CD14 (sCD14) in plasma of healthy and septic neonates in order to evaluate their potential diagnostic role. The study included prospectively collected patient samples of two recently published studies on cytokine expression in neonatal sepsis. Twenty-nine septic patients were enrolled in the present analysis. Samples—either cord blood or peripheral blood—from patients admitted within the first 24 h of life for suspicion of sepsis and cord blood samples of a control group of 40 healthy mature infants delivered spontaneously were analyzed. For seven patients of the septic group, a second sample collected between 24 and 48 h of life was available. Levels of sCD14 and LBP in plasma were determined by an enzyme immunoassay using recombinant CD14 and LBP as standards. LBP and sCD14 were correlated to cytokine plasma levels. In septic neonates, LBP (median, 36.6 versus 7.8 μg/ml; P < 0.001) and sCD14 (median, 0.42 versus 0.28 μg/ml; P < 0.001) levels were highly elevated when compared to those of healthy neonates and strongly correlated to granulocyte colony-stimulating factor (G-CSF), interleukin-1β (IL-1β), IL-6, and IL-8 levels. LBP levels in septic neonates analyzed between 24 and 48 h of life even increased when compared to samples obtained at or shortly after delivery (median, 36.6 versus 60 μg/ml; P = 0.038). In summary, levels of LBP in plasma of neonates with early-onset sepsis are significantly elevated; the elevated plasma levels seem to persist for more than 24 h, which could provide the clinician with a prolonged time period to identify the newborn with bacterial sepsis.


2021 ◽  
Vol 20 (3) ◽  
pp. 31-35
Author(s):  
S. Ragab ◽  
E. Badr ◽  
H. El-Kholy ◽  
M. El-Hawy

To assess the impact of our transcranial Doppler (TCD) screening program on the incidence of a first stroke in children with sickle cell anemia and to study the role of elevated serum endothelin-1 (an inflammatory mediator) in these children. Background: stroke is a major complication of sickle cell disease (SCD), even in very young children. About 11% of children with homozygous sickle cell anemia (SS) develop stroke by the end of the second decade of life. The underlying etiology in most cases is an ischemic stroke caused by large-vessel stenosis or occlusion. Transcranial Doppler (TCD) recommended as a routine screening test to identify children at high risk of developing a stroke, measures flow velocities within large intracranial arteries. TCD should be routinely performed in children between 2 and 16 years as this age group is at the highest risk of sickle cerebral vasculopathy. We carried out a prospective case-control study which included 2 groups: a patient group consisted of 30 children with sickle cell anemia and sickle thalassemia and a group of 30 healthy children of matched age and sex. Each group included 11 males (36.5%) and 19 females (63.5%); the age range was 2 to 17 years. Both groups underwent a thorough clinical examination and laboratory tests (CBC, liver and renal function, serum ferritin and endothelin-1). Additionally, TCD was performed in all children included in the patient group. According to the results of TCD, time-averaged mean of the maximum velocity (TAMMX) was < 170 cm/s (normal), 170–200 cm/s (conditional), ≥ 200 cm/s (high risk) in 20 (66.7%), 4 (13.3%) 6 (20%) patients, respectively. The level of endothelin-1 was significantly higher in the patients (57.1 ± 91.3) than in the controls (21.9 ± 14.8). Hemoglobin concentration was significantly lower in the patient group than in the control group, but the levels of reticulocytes, WBCs and serum ferritin were significantly higher in the patients than in the healthy controls. Serum Endotheline-1 level was higher in patients with sickle cell anemia than control group.


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