Right-To-Left Shunts (Extra-Cardiac Arterial-Venous Malformations) Are Highly Common In Adults With Sickle Cell Disease

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1004-1004
Author(s):  
Nathan Langer ◽  
MaryAnn O'Riordan ◽  
Santosh K. Rao ◽  
Jane A. Little ◽  
Robert Schilz
Keyword(s):  
Sickle Cell Disease ◽  
General Population ◽  
Oxygen Saturation ◽  
Sickle Cell ◽  
Morbidity And Mortality ◽  
Acute Chest Syndrome ◽  
Venous Malformations ◽  
Cell Disease ◽  
Group I ◽  
Group Ii

Abstract Introduction Cardiopulmonary complications are a major cause of morbidity and mortality in sickle cell disease (SCD) as shown by worse prognosis in patients who have experienced acute chest syndrome or who have an elevated tricuspid regurgitant jet velocity (TRV) on echocardiogram at clinical baseline. Here we describe an unexpected and novel cardio-pulmonary complication in HbSS, right-to-left shunting through extra-cardiac arterial-venous malformations (AVMs), which may contribute to pathophysiology. Extracardiac AVMs are rare in the general population, with an estimated incidence of 1/5000. Of 2111 shunt evaluation echocardiograms performed at our institution over 12 months only 81 (3.8%) of individual studies were positive. Methods We evaluated 36 HbSS patients who presented with subjective dyspnea or hypoxia with clinical exam and with echocardiogram utilizing agitated saline to assess for vascular right-to-left shunts. We compared this group with the remaining 81 HbSS patients in our database. 19 of 36 symptomatic patients were found to have an extracardiac right-to-left shunt. We then compared these 19 patients with the 17 symptomatic HbSS patients who did not have a shunt. 10 HbSC and 5 S-beta-thalassemia patients were also studied and did not have a right-to-left shunt; only HbSS are included in comparative analyses. Results Patients with symptoms did not differ in age (32.7±10.3 years vs 31.7±11.7 years) from patients who did not present with hypoxia or subjective dyspnea (n=81). Symptomatic patients were more likely to have macroalbuminuria (>300 mg/g albumin-to-creatinine, 9/36 vs 8/63 evaluable, p=0.05), more likely to have a TRV ≥3 meters/second (9/36 vs 11/74 evaluable, p=0.09), and were more hypoxic at rest (96 ±5% vs 98±2% oxygen saturation, p=0.07). We found delayed left-sided bubble visualization in 19/36 symptomatic HbSS subjects (53%) consistent with extra-cardiac AVMs. HbSS subjects with (n=19, Group I) or without (n=17, Group II) a positive bubble study were clinically and demographically similar (age, gender, WBC, total Hgb, HgbF%, LDH, eGFR, proportion with macroalbuminuria, baseline oxygen saturation, and elevated TRV). However, group I patients had a higher reticulocyte count (15.4±5.5% vs 9.8±6.7% p<0.005). Of Group I patients, 42.1% had history of acute chest while 70.6% of Group II had such history (p=0.09). Group I patients were less likely to be on hydroxyurea (52.78% vs 47.22% p<0.05). Conclusion Extra-cardiac AVMs are observed in 16% of all subjects with HbSS, compared with 3.8% of general medical patients at a tertiary center undergoing shunt evaluation and .02% in the general population. In HbSS, symptomatic subjects are more likely to have evidence for vasculopathy (macroalbuminuria, elevated TRV) and hypoxia; one-half of these symptomatic patients have extracardiac AVMs. We speculate that this finding is unlikely to be clinically silent, and a bubble-echocardiogram may be an important additional clinical evaluation for symptomatic dyspnea or hypoxia. The impact of this novel clinical finding on morbidity and mortality in this disease remains under investigation. Disclosures: No relevant conflicts of interest to declare.

The Use Of Transcranial Doppler Ultrasonography In Adult Patients With Sickle Cell Disease: A Retrospective Analysis

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4692-4692 ◽  
Author(s):  
Sofia Galli ◽  
Soundrie Padayachee ◽  
Jo Howard
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Stroke Risk ◽  
Cell Disease ◽  
Carotid Duplex ◽  
Group I ◽  
Ultrasound Findings ◽  
Group Ii ◽  
Ica Stenosis ◽  
Extracranial Ica

Background Unlike in children, Transcranial Doppler Ultrasound (TCD) can not currently be used to predict stroke risk in adults with Sickle Cell Disease, and this is confounded by an age related decline in TCD and lack of an established normal TCD velocity range for adults. In addition abnormalities in the extracranial circulation have been associated with increased incidence of stroke in children with SCD, but this has not been shown in adults. Aims To retrospectively review TCD and carotid duplex ultrasound results from adults with SCD and to correlate with neurological outcome. Methods TCD and carotid duplex data were collected from 112 patients from January 2003 to December 2012 and analysed retrospectively. Imaging and non-imaging TCD and carotid duplex were performed as a routine out-patient investigation as part of an annual review in the comprehensive adult sickle cell clinic in our unit. STOP velocity criteria were used for stroke risk stratification. Carotid disease was defined as non-significant (<50%), moderate stenosis (50-69%) and severe disease (≥70%). Results The mean age of patients was 35.4 (range 17-79) years; 65% were female and 78% had HbSS. There were 5 patient exclusions based on the TCD findings; two were non-diagnostic and three had abnormal scans as part of childhood screening. The remaining 107 patients were divided into two groups; Group I patients had a normal neurological examination (n = 95) and Group II had an abnormal neurological examination with a clinical diagnosis of stroke that preceded TCD/carotid duplex examination (n = 12). In Group I there were 4 abnormal TCD scans according to STOP criteria; two showed significant asymmetry (>25%) and two showed abnormally low velocities (<50cm/s), one of whom subsequently developed a silent stroke. Although the remaining 91 patients had normal TCD findings; a limited TCD examination was noted in 6 patients and asymmetrical velocities that fell below the 25% threshold were observed in 7 patients, one of whom subsequently developed a symptomatic lacunar infarct. A further eight patients showed extracranial (ICA) disease and one of these also had evidence of a silent stroke on MRI. In Group II, 5 patients showed abnormal TCD findings; four had low velocities (<50cm/sec) and one had low velocities and significant asymmetry. Six patients had severe extracranial ICA stenosis or occlusion on carotid duplex. There was a significant increase in the incidence of abnormal TCD (C2=19.4, p<0.001) and duplex findings (C2=16.2, p<0.001) in patients in Group II compared to Group I. Conclusions Routine screening of adults with SCD using TCD did not show any asymptomatic patients with elevated TCD flow-velocities (>170cm/s), confirming previous studies. However, other abnormal ultrasound findings were observed including; abnormally low or asymmetric TCD velocities and extracranial ICA disease. These abnormalities were associated with stroke in three cases; TCD asymmetry preceded a lacunar infarct by five years, abnormally low velocities preceded a silent infarct by two years and severe extracranial ICA stenosis was associated with a silent infarct. There was a significantly increased incidence of abnormal ultrasound findings in patients who had neurological symptoms (91.6% versus 12.6%). Further investigation of the incidence and implications of abnormal TCD and extracranial carotid duplex findings in adults with sickle cell disease is required. Longitudinal follow-up of patients with low or asymmetric velocities and ICA disease will clarify if these abnormalities are associated with increased neurological morbidity; if so, intervention may be justified. The traditional STOP criteria of raised TCD velocities are inadequate in the adult sickle population, new velocity criteria are required to stratify the risk of stroke in this population. Disclosures: Howard: Sangart: Membership on an entity’s Board of Directors or advisory committees.

scholarly journals Presentation, Management and Outcomes of COVID-19 Patients with Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 29-30
Author(s):  
Nwabundo Anusim ◽  
Ruby Gupta ◽  
Hycienth O Ahaneku ◽  
Candace Franklin ◽  
Savitha Balaraman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Respiratory Symptoms ◽  
Bone Pain ◽  
Globin Gene ◽  
Morbidity And Mortality ◽  
Polymerase Chain Reaction Test ◽  
Acute Chest Syndrome ◽  
Shortness Of Breath ◽  
Cell Disease

Background Sickle cell disease (SCD) is an inherited disorder of red blood cell (RBC) caused by a mutation in the beta-globin gene resulting in abnormal hemoglobin known as hemoglobin S (HbS) or the sickle hemoglobin. Several clinical variants of SCD have been elucidated, all driven by two fundamental pathophysiologic processes: RBC hemolysis and intermittent vaso-occlusive vasculopathy resulting in tissue ischemia/infarction. These two processes underscore the many complications and eventual multi-organ damage that may develop in patients with the most severe types of SCD. Cardiopulmonary complications including heart failure, pulmonary hypertension and acute chest syndrome (ACS) are major drivers of morbidity and mortality among patients with SCD. With regards to ACS, patients often present with fever, cough and shortness of breath caused by vaso-occlusive crisis affecting the lungs. This is particular concerning in view of its similar features to symptomatic COVID-19 infection. Methods We retrospectively identified SCD patients with COVID-19 infection admitted to Beaumont hospitals in Michigan between March 1st 2020 and July 1st 2020. Data was abstracted using the ICD 10 code of U07. 1 for COVID-19, ICD 9 and 10 codes of 282.60 and D57 for sickle cell disease. We excluded patients with sickle cell trait. Data regarding the demographics, presentation, management and outcomes were abstracted. Results A total of eleven patients with sickle cell disease were identified as having a positive SARS-Cov19 polymerase chain reaction test (Table I). All were African American and predominantly female (64%) with a mean age of 44 (22-60) years and mean BMI of 30.2 kg/m2. Genotypes identified were HbSS in 5 (45%) patients, HbSC in 4 (36%), HbS/beta-thalassemia in 1 (9%) and HbS/alpha-thalassemia in 1 (9%). All of the patients had seen a haematologist since their diagnosis but none of the patients were on hydroxyurea, voxeloter, L-glutamine or crizanlizumab at admission. The predominant clinical presentation was fever, chest pain, chills, exertional shortness of breath and cough but this was not consistent across all patients. All the patients were managed with intravenous hydration, pain management as well as hydroxychloroquine/azithromycin per institutional guideline at that time. Three patients (cases 1-3) had recurrent visits to the hospital for similar symptoms and new bone pain crises. Case 1 had a pulmonary embolus which was evident on re-admission. Two patients (cases 3 and 10) succumbed to COVID-19. Two patients (cases 5 and 7) presented with bone pain crisis and no respiratory symptoms, but chest imaging was suggestive of COVID-19 infection necessitating treatment with antibiotics, possibly indicating that the virus can trigger vaso-occlusive crises without respiratory symptoms. Case 8 had a high Charlson comorbidity index and age over 60, had the lengthiest hospital stay complicated by renal failure and polyneuropathy, and was discharged to a long-term acute care facility: an outcome which is consistent with current data showing that the elderly and unfit patients are more likely to have a higher morbidity and mortality with COVID-19. Conclusion To date, there no compelling evidence to provide guidelines for the management of SCD patients with COVID-19. However, following existing recommendations in managing acute chest syndrome and those for COVID-19 symptomatic infection, is a good place to start. We continue to seek to improve management of these patients as new evidence of successful treatment emerges, and also encourage patients to participate in clinical trials. Disclosures No relevant conflicts of interest to declare.

scholarly journals Sickle Cell Disease and COVID-19 Infection: A Single-Center Experience

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 985-985
Author(s):  
Sindhu Devarashetty ◽  
Kimberly Le Blanc ◽  
Udhayvir Singh Grewal ◽  
Jacqueline Walton ◽  
Tabitha Jones ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Sickle Cell Disease ◽  
Mortality Rate ◽  
Sickle Cell ◽  
Adult Patients ◽  
Living Alone ◽  
Morbidity And Mortality ◽  
Acute Chest Syndrome ◽  
Single Center ◽  
Cell Disease

Abstract Background and Objectives: The COVID-19 (CO19) pandemic caused by SARS-CoV-2 remains a significant issue for global health, economics, and society. Several reports have shown that African Americans (AA) have been disproportionately affected by the CO19 pandemic. Limited data have suggested that sickle cell disease (SCD) could be one of the several reasons for higher morbidity and mortality related to CO19 among AA. Recent reports have suggested higher-than-average morbidity and mortality related to CO19 among patients with SCD. We conducted a retrospective, single-institution study in adult patients with SCD who were diagnosed with CO19 infection and their outcomes. Methods: After IRB approval, we conducted a chart review of adult patients (greater than 18 years) with SCD who were diagnosed with CO19 infection between March 1st, 2020, and March 31st, 2021. We recorded demographic data including age, gender, social factors (the type of insurance, availability of primary care provider (PCP), living alone/not), clinical parameters (type of SCD, co-morbidities), outpatient management of SCD, and how CO19 infection was managed like inpatient admission and complications. In patients who were admitted or seen in the emergency department (ED), we collected additional data including vitals, labs, the severity of illness, complications, length of stay, and outcomes. Computations were performed using statistical software SAS 9.4 for Windows. Results: We found a total of 51 patients with SCD diagnosed with CO19 infection in the above period. The median age of patients was 30 years. 61% were females and 39 % were males. All of them were AA. 11.76% were living alone, 49.02% were living with family, 1.96% (1 patient) was institutionalized, and the living situation was unknown in 37.25%. Most of the patients had Medicaid Insurance (52.94%), Medicare in 33.3%, private insurance in 13.73 % and 2% were uninsured. Only 64.71% of patients had a PCP. 60% had HbSS disease, 32% had HbSC disease, 4% had HbS-beta thalassemia, one patient each had HbSS with hereditary persistence of HbF and HbS/HbD. Comorbidities and previous history included acute chest syndrome in 65.96%, avascular necrosis in 36.96%, leg ulcers in 8.7%, hypertension in 8.7%, sickle cell retinopathy in 14.57%, cerebrovascular disease in 26.19%, chronic kidney disease in 7.69%, venous thromboembolism (VTE) in 20.41%, 10.41% were on anticoagulation, history of HIV and hepatitis C infection in 6.38%. 28.21% of patients were maintained on partial exchange transfusions as an outpatient for various indications. 72.73% were on hydroxyurea, 7.5% were on crizanlizumab, 5.26% were on voxelotor and 26.83% were on iron chelation. Vitals and pertinent lab values on initial assessment were recorded and many patients had missing data. On presentation, 25.53% were febrile, 29.17% of patients were tachycardic, 31.25% were hypoxic (SpO2 &lt; 95%), 38.46% were tachypneic, 59.18% had a body mass index (BMI) of &gt; 24.9. Median hemoglobin and hematocrit were 8.9/27.4 g/dL. The median white blood cell count was 9490/uL and platelets were 315,000/uL. Median ferritin was 1573 ug/L. Median bilirubin and creatinine were 2.05 mg/dL and 0.86 mg/dL. The patients were further stratified based on the clinical location where CO19 infection was managed (Table 1). 39.3% were diagnosed in the outpatient setting/ED and 60.3% in the inpatient setting. Among 51 patients, 5.71% (n=2) required ICU admission and was mechanically ventilated. 17.5% received dexamethasone, 7.69% received remdesivir, 2.76% received convalescent plasma, 17.07% had infections and 47% received antibiotics. Only one patient received an exchange transfusion during admission. One patient developed a new VTE after CO19 infection. On statistical analysis, the only factor which impacted the clinical location of management was tachycardia (P=0.007). Of the 51 patients, only 3.9% (2 patients) died of complications of CO19 infection, one with hypoxic respiratory failure, disseminated intravascular coagulation, shock, and the other one with pulmonary embolism. 13% were readmitted within a month, one of them was admitted with a new pulmonary embolism and the others were admitted for acute painful episodes. Conclusion: We found a mortality rate of 3.9% in our single-center study of patients with SCD and CO19 infection. This mortality rate is lower than other published experiences in patients with SCD and CO19 infection. Figure 1 Figure 1. Disclosures Master: Blue Bird Bio: Current holder of individual stocks in a privately-held company.

Characterization of Pain, Vasculature and Innervation in Sickle Cell Disease.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2248-2248
Author(s):  
Kalpna Gupta ◽  
Chunsheng Chen ◽  
Marna E. Ericson ◽  
Robert P. Hebbel ◽  
Yunfang Li
Keyword(s):  
Sickle Cell Disease ◽  
Blood Vessels ◽  
Sickle Cell ◽  
Thermal Hyperalgesia ◽  
Cell Disease ◽  
Morphine Treatment ◽  
Group I ◽  
Group Ii ◽  
And Control

Abstract Pain in sickle cell disease (SCD) is characterized by chronic vasculopathy. Characterization of pain and vasculature may be critical to improve the analgesic ability of opioids in treating pain in SCD. Therefore, we examined the association between vasculature, innervation and pain in a transgenic mouse model of SCD (BERK) and control mice (HbABERK). Pain behavior was analyzed using paw withdrawal latencies (PWL) using Hargreave’s device for thermal hyperalgesia. A radiant heat stimulus was applied to the plantar surface of each hind paw from underneath the glass floor with a projector lamp bulb (CXL/CXR, 8 V, 50 W). PWL to the nearest 0.1 s was automatically recorded upon paw withdrawal. Paws were alternated randomly to preclude “order” effects. We observed that 5 mo old sickle mice showed significantly lower thresholds to noxious heat than controls (p=0.002 for females; and p=0.04 for males; n=5–7 and 15 observations/mouse). Females showed a significantly shorter latency than their respective males (control, p=0.03 and sickle, p=0.01), i.e., they were more sensitive to thermal stimuli. No significant difference was observed between sickle and control nor male and female mice (p &gt; 0.05, n = 5–7) for mechanical allodynia using von Frey filaments, suggesting that sickle mice show increased sensitivity to thermal hyperalgesia. BERK mice were subcutaneously injected with 0.7 mg morphine/Kg mice/day (equivalent to 50 mg/70 kg /day/ human adult) with an added increment of 0.14 mg/day/Kg for each week for three different time periods; Group I, treated for 6 weeks, Group II treated for 6 weeks followed by withdrawal for 6 weeks and Group III treated for 12 weeks. Morphine treatment for 6 weeks as well as 12 weeks resulted in a ∼50–75% increase in PWL vs PBS, suggesting that chronic morphine treatment decreased hyperalgesia in sickle mice. The anti-hyperalgesic effect of morphine was antagonized by simultaneous co-administration of naloxone (2 mcg/Kg/day), suggesting an opioid receptor mediated effect. Naloxone treatment alone did not show any significant effect on PWL. In Group II, withdrawal of morphine for 6 weeks following 6 wks of treatment showed about 35% increase in PWL vs PBS (p&lt;0.005), suggesting that the anti-hyperalgesic effect of morphine continues after its withdrawal. The continued anti-hyperalgesic effect of morphine following withdrawal could be secondary to its vasoregulatory effect. Indeed, confocal microscopy of skin sections revealed disorganized and stringy blood vessels, nerves and lymphatics in sickle mice vs. control. Deep blood vessels and nerve plexus, as well as the skin, were appreciably thicker in controls vs sickle. The diameter of lymphatics and densities of both blood vessels and nerves were significantly lower in sickle vs HbA controls (p= 0.0001 and 0.002, for sub-epidermal and dermal blood vessels, respectively; p=0.04 for lymphatic diameter; p=0.0001 for sub-epidermal, dermal and deep dermal nerve fibers). Functionally, we observed about 30% decrease in subcutaneous O2 measured by Laser Doppler (in real-time) in BERK vs HbA control (p&lt;0.05). Thus, complementary alteration in vasculature and innervation may underlie the chronic pain in SCD. Given that morphine promotes angiogenesis and vasodilation, its prolonged anti-hyperalgesic activity could be due to its vasoregulatory function. Together, these data suggest that intermittent therapy with morphine in SCD may provide analgesia similar to continued therapy and may even be devoid of side-effects.

scholarly journals Timed Average Mean Maximum Velocity (TAMMV) of Cerebral Blood Flow of Children and Adolescents with Sickle cell Disease: correlation with clinical and hematological profiles in country

2021 ◽  
Vol 33 (3) ◽  
pp. 169-177
Author(s):  
Bartholomew Chukwu ◽  
Lyra Menezes ◽  
Thiago Fukuda ◽  
Jamary Filho ◽  
Marilda Goncalves
Keyword(s):  
Sickle Cell Disease ◽  
Oxygen Saturation ◽  
Sickle Cell ◽  
Previous History ◽  
White Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Platelet Counts ◽  
Increased Risk ◽  
History Of

BackgroundDetection of abnormal TAMMV with transcranial Doppler is fundamental in primary stroke prevention in children with sickle cell disease (SCD). The study aimed at evaluating TAMMV and correlating it with clinical and hematological profiles of children and adolescent with SCD. MethodsTranscranial Doppler was performed on subjects aged 2-16 years, using a 2 MHz probe placed over the transtemporal windows. Pulse oximetry was used to determine the peripheral oxygen saturation while clinical and hematological profiles were retrieved from their medical records.Results One hundred and thirty five patients were recruited. The mean TAMMV was 125cm/s. Patients with HbSS had a significantly higher TAMMV (131cm/s) than those with HbSC (107cm/s). Only one (0.74%) patient had abnormal TAMMV. TAMMV correlated inversely with oxygen saturation, Hct and patient’s age, and positively with white cell and platelet counts. Previous history of acute chest syndrome (ACS) and recurrent painful crises increased the risk of development of abnormal and conditional velocity.Conclusion Frequency of abnormal TAMMV in this study was low. Younger children and those with HbSS had higher TAMMV. Age, oxygen saturation and haematocrit correlated negatively while white cell and platelet counts correlated positively with TAMMV. Previous history of ACS and recurrent bone pain were associated with increased risk of having abnormal and conditional TAMMV.

Von Willebrand Factor in Sickle Cell Disease - Relationship to Sleep Hypoxia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1240-1240
Author(s):  
Suba Krishnan ◽  
Gene Pullen ◽  
Megan Hevelov ◽  
Jamie Siegel ◽  
B.N.Y. Setty ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Mean Values ◽  
Group I ◽  
Platelet Aggregometry ◽  
Endothelial Response ◽  
Group Ii ◽  
Student’S T

Abstract Introduction: Despite the intriguing in vitro data implicating vWF in sickle-RBC-endothelial adhesion and in animal models of vasoocclusion, there are few clinical studies characterizing the role of vWF in SCD pathophysiology. Rationale: Plasma vWF is mainly endothelial-derived, upto 95% of newly synthesized vWF being secreted constitutively. Since in vitro studies have shown that hypoxia mediates release of ultra-large vWF multimers stored in endothelial Weibel Palade bodies, we hypothesized that changes in the plasma vWF profile of patients with sickle cell disease (SCD) could potentially be related to their oxygenation status and subsequent clinical events. Patient groups: Following informed consent blood samples were obtained from 20 children with SCD who were enrolled in an investigation of sleep hypoxia. After an awake blood oxgygen saturation by pulse oximetry (SaO2), an overnight recording was performed and a histogram generated showing proportions of time spent at various SaO2s. On the basis of mean sleeping SaO2s, the study population was divided into Group I (Normoxia, sleeping SaO2>94%, n=10) and Group II (Hypoxia, sleeping SaO2 <94%, n=10). Methods: VWF antigen level (vWF antigen) was determined by a latex enhanced immunoassay and Ristocetin Cofactor activity (RCof) was evaluated by platelet aggregometry. VWF multimer analysis was performed on SDS- 1.6% agarose gels and visualized using a polyclonal rabbit anti-human VWF antibody (Dako) followed by chemiluminescent detection using horse radish peroxidase-conjugated anti-rabbit IgG (Amersham). The presence of high molecular weight VWF multimers had to fulfill the following three criteria: (a) vWF multimers larger than the largest vWF multimers present in pooled normal plasma;(b)vWF multimers similar to the largest multimers present in a post-DDAVP infusion plasma sample analyzed on the same gel;(c) assessment by three independent investigators. Student’s t-test was performed to compare the mean values of vWFAg, vWF:RCof, and vWF RCof:Ag ratio of Gps I and II. Results: We observed significant differences between normoxic vs. hypoxic children with SCD as depicted in the table below where results are shown as Mean values(1SD). vWF antigen levels correlated inversely (r2=0.28) and vWF RCof:Ag ratios correlated positively (r2=0.2) with sleeping SaO2s. vWF:Ag* vWF RCof vWF RCof:Ag Ratios** HMW-vWF *p <0.003; **p <0.006 Group I >/94% 120.8(30.9) 116.7 (36.8) 0.96(0.1) 0/7 (performed to date) Group II <94% 192.2 (69.4) 147.3 (71.3) 0.77(0.14) 4/5 (performed to date) Conclusions: Children with SCD and sleep hypoxia showed abnormalities in their vWF profiles when compared to normoxic children with SCD including elevated vWF antigen and qualitatively increased HMW multimers suggestive of hypoxia-mediated endothelial response. In addition, there is an unexpected discordance between these findings and decreased vWF RCof:Ag ratios. Since children with SCD and nocturnal hypoxemia have an increased rate of pain-associated vasoocclusion and CNS complications, our findings of vWF-related perturbations could provide an explanation for hypoxia-induced modulation of the proadhesive and prothrombotic state in these children.

scholarly journals ECMO Therapy in Acute Chest Syndrome for Patients with Sickle Cell Disease: a Case Report and Literature Review

2021 ◽  
Author(s):  
Soi Avgeridou ◽  
Ilija Djordjevic ◽  
Anton Sabashnikov ◽  
Kaveh Eghbalzadeh ◽  
Laura Suhr ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Scientific Data ◽  
Acute Chest Syndrome ◽  
Data Sets ◽  
Limited Data ◽  
Cell Disease ◽  
Life Saving ◽  
Institutional Experience ◽  
Ecmo Therapy

AbstractExtracorporeal membrane oxygenation (ECMO) plays an important role as a life-saving tool for patients with therapy-refractory cardio-respiratory failure. Especially, for rare and infrequent indications, scientific data is scarce. The conducted paper focuses primarily on our institutional experience with a 19-year-old patient suffering an acute chest syndrome, a pathognomonic pulmonary condition presented by patients with sickle cell disease. After implementation of awake ECMO therapy, the patient was successfully weaned off support and discharged home 22 days after initiation of the extracorporeal circulation. In addition to limited data and current literature, further and larger data sets are necessary to determine the outcome after ECMO therapy for this rare indication.

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