Surgical treatment of moyamoya syndrome in patients with sickle cell anemia: outcome following encephaloduroarteriosynangiosis

2008 ◽  
Vol 1 (3) ◽  
pp. 211-216 ◽  
Author(s):  
Todd C. Hankinson ◽  
Leif-Erik Bohman ◽  
Geoffrey Heyer ◽  
Maureen Licursi ◽  
Saadi Ghatan ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Significant Risk ◽  
Moyamoya Syndrome ◽  
Effective Treatment Option ◽  
Left Lower Extremity ◽  
Cerebrovascular Complications ◽  
Period 2 ◽  
Ischemic Attack

Object Children with sickle cell anemia (SCA) and moyamoya syndrome carry a significant risk of ischemic stroke. Given the success of encephaloduroarteriosynangiosis (EDAS) or pial synangiosis in the treatment of moyamoya disease, the purpose of this study was to examine whether it reliably and durably protected children with SCA and moyamoya syndrome against cerebrovascular complications. Methods The authors retrospectively reviewed a series of 12 patients with SCA who developed clinical and/or radiological evidence of moyamoya syndrome and underwent EDAS. Results Eleven patients (92%) presented following a cerebrovascular accident (CVA), transient ischemic attack (TIA), or seizure. Magnetic resonance (MR) imaging or angiography suggested moyamoya vascular changes, and cerebral angiography confirmed the diagnosis in all 12 patients. At the time of surgery, the median age was 12.3 years (range 6.8–19.4 years). Ten (83%) of 12 patients had a history of CVA, and 4 of these patients were compliant with a transfusion protocol at the time of their CVA. Bilateral (7 patients) or unilateral (5 patients) EDAS was performed without complications. The mean follow-up period was 46.8 months (range 8.1–106 months). During the follow-up period, 2 patients (16.7%) suffered cerebrovascular events. One patient, who was stroke-free preoperatively, suffered a CVA 3 weeks after the procedure. The other patient suffered a single left lower-extremity TIA 18 months following right-sided EDAS. She returned to her neurological baseline condition and remains stable 53 months postoperatively. Seven patients underwent follow-up angiography or MR angiography, and evidence of revascularization was noted in all cases. At this time, no patient has developed progressive disease requiring a contralateral procedure after unilateral EDAS. Conclusions The EDAS procedure is a safe and effective treatment option in patients with SCA who develop moyamoya syndrome.

Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases

2014 ◽  
Vol 36 (1) ◽  
pp. E12 ◽  
Author(s):  
Benjamin C. Kennedy ◽  
Michael M. McDowell ◽  
Peter H. Yang ◽  
Caroline M. Wilson ◽  
Sida Li ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Brain Ischemia ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Pediatric Patients ◽  
Significant Risk ◽  
Moyamoya Syndrome ◽  
Transfusion Therapy ◽  
Comprehensive Review

Object Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature. Methods The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes. Results The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003). Conclusions Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.

scholarly journals Outcomes in Children with Severe Vasculopathy from Sickle Cell Anemia after Treatment with Chronic Transfusion, Surgical Revascularization and/or Allogenic Hematopoetic Stem Cell Transplantation

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1098-1098
Author(s):  
Courtney W. Johnson ◽  
Suvankar Majumdar ◽  
Andrew D. Campbell ◽  
Suresh Magge ◽  
Deepika S. Darbari ◽  
...  
Keyword(s):  
Stem Cell ◽  
Board Of Directors ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Research Funding ◽  
Moyamoya Syndrome ◽  
Surgical Revascularization ◽  
Advisory Committees ◽  
Time Of Intervention

Abstract Background: Cerebral vasculopathy is a frequent complication of sickle cell anemia (SCA) and is associated with a high risk for stroke. This vasculopathy seen in SCA can be progressive and severe. Sickle cell patients with severe vasculopathy, including Moyamoya syndrome are at increased risk for neurological disabilities and death. While chronic transfusions decrease the risk of stroke in SCA; unfortunately, progression of vasculopathy can occur despite treatment. Limited data exists regarding long term outcomes for this population. We evaluated effectiveness of three treatment approaches at our center, namely chronic transfusions, surgical revascularization plus chronic transfusions and allogenic hematopoietic stem cell transplant (HSCT). Methods: A retrospective chart review was preformed to identify patients with SCA (hemoglobin SS, Sβ0) and severe vasculopathy including Moyamoya syndrome between 1986 to 2017. Severe vasculopathy was defined as having at least one cerebral artery with > 70% stenosis and/or occlusion as seen on MR angiogram (MRA), CT angiogram (CTA) or conventional angiogram (DSA) as determined by a neuroradiologist at our institution. Patients were identified from an institutional stroke database. Patients were included for analysis if they received at least one of the following: chronic transfusions, surgical revascularization (i.e. encephalo-duro-arterio-synagiosis (EDAS) plus chronic transfusions or HSCT. For HSCT, all graft types (bone marrow, peripheral blood stem cells, umbilical cord blood), conditioning regimens and donor types (related, unrelated and haploidentical) were included. Time to event analyses were performed from the time of intervention (transfusion, HSCT, EDAS/chronic transfusions) using overt clinical stroke, new silent infarcts, progression of vasculopathy or new vasculopathy. Survival curves were analyzed using the log-rank (Mantel-Cox) test. Results: Of 35 patients identified, 54% (n =19) underwent chronic transfusions, 23% (n=8) of patients underwent HSCT after being on chronic transfusions, 23% (n=8) underwent EDAS with chronic transfusions and 1 patient underwent each of the above three modalities (Table 1). Median age at time of intervention was similar for all three cohorts (Table 1). Males were overrepresented in all treatment arms (62.5-79% of patients). Average hemoglobin level prior to intervention was also similar: 7.6 g/dL (IQR 7.1-8.3) for the chronic transfusion cohort, 7.3 gm/dL (IQR 6.3-8.2) for the HSCT cohort, and 7.5 gm/dL (IQR 7.2-8) for the EDAS/chronic transfusion cohort. Absolute reticulocyte count was 492.9 K/ul (IQR 358.4-550) for the chronic transfusion group, 389.4 (IQR 174.3-449) for HSCT, and 250.2 (IQR 107.3-393) for EDAS/chronic transfusions (p=0.08). One patient died of overt stroke in the chronic transfusion cohort. The median follow-up times for the transfusion, HSCT and EDAS plus transfusion groups were 4.4, 2.4 and 6 years respectively. Time from date of intervention (transfusion, HSCT, EDAS) to overt clinical or silent stroke was evaluated (Fig 1). Two of the nineteen patients in the chronic transfusion cohort suffered an overt stroke, while one of eight and two of eight had strokes in the post-HSCT and EDAS plus chronic transfusion cohorts respectively. Fourteen of nineteen (74%) in the chronic transfusion cohort had progression of severe vasculopathy after being on transfusions while two of eight (25%) in the HSCT and four of the eight (50%) patients in the EDAS plus chronic transfusion cohorts had progression. The one patient with all three different interventions did not have additional infarction (clinical or silent) or vasculopathy progression during 1.5 years of follow-up. Conclusions: The risk for cerebral infarction and/or vasculopathy progression after initiation of treatment with either chronic transfusion, HSCT or EDAS is still a major concern. Our data suggest HSCT and surgical revascularization with chronic transfusion provide the greatest benefit in reducing stroke risk and HSCT reduces risk for progression of a severe vasculopathy. Additional, large population studies are needed to clarify the risk. Disclosures Majumdar: NIMHD: Research Funding. Campbell:Functional Fluitics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding.

scholarly journals Pial synangiosis in patients with moyamoya syndrome and sickle cell anemia: perioperative management and surgical outcome

2009 ◽  
Vol 26 (4) ◽  
pp. E10 ◽  
Author(s):  
Edward R. Smith ◽  
Craig D. McClain ◽  
Matthew Heeney ◽  
R. Michael Scott
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Average Length ◽  
Length Of Hospital Stay ◽  
Multidisciplinary Care ◽  
Neurological Status ◽  
Moyamoya Syndrome ◽  
Radiographic Evidence ◽  
Radiographic Findings

Object Many children with sickle cell anemia (SCA) also have clinical and radiographic findings of an arteriopathy suggestive of moyamoya syndrome. These patients may continue to experience strokes despite optimal medical management. The authors wished to define features of moyamoya syndrome associated with SCA and determine the results of surgical revascularization in these patients at early and late follow-up. Methods The authors reviewed the clinical and radiographic records of all patients with moyamoya syndrome and SCA who underwent cerebral revascularization surgery using a standardized surgical procedure—pial synangiosis—from 1985 to 2008. Results Twelve patients had SCA and moyamoya syndrome. Six patients were female and 6 were male. The average patient age at surgery was 11.3 years (range 3–22 years). All patients presented with ischemic symptoms, 11 (92%) with previous transient ischemic attacks, and 10 (83%) with completed strokes. Eleven patients (92%) had radiographic evidence of previous stroke at presentation. None presented with hemorrhage. Surgical treatment included pial synangiosis in all patients. Complications included 1 perioperative stroke, 1 wound infection, and 1 perioperative pneumonia. The average length of hospital stay was 5.7 days (including a 24-hour preoperative admission for hydration) and average blood loss was 92.5 ml/hemisphere (in a total of 19 hemispheres). Clinical and radiographic follow-up with an average of 49 months (range 9–144 months) demonstrated no worsening in neurological status in any patient. No clinical or radiographic evidence of new infarcts was observed in any patient at late follow-up, despite disease progression in 13 (68%) of 19 hemispheres. Conclusions The clinical and radiographic features of moyamoya syndrome associated with SCA appear comparable to primary moyamoya disease. Successful treatment of these patients requires multidisciplinary care involving hematologists, anesthesiologists, and neurosurgeons. Operative treatment of moyamoya syndrome using pial synangiosis appears to be safe and confers long-lasting protection against further stroke in this population, and provides an alternative for failure of optimal medical therapy in patients. This study underscores the potential merit of screening patients with SCA for moyamoya syndrome.

scholarly journals Ischemic Stroke in Children and Young Adults with Sickle Cell Disease (SCD) in the Post-STOP Era

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 68-68 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
Julie Kanter ◽  
Heather J. Fullerton ◽  
Jenifer Voeks ◽  
Ellen Debenham ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Clinical Series ◽  
The Mean

Abstract Background: The Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) established routine transcranial Doppler ultrasound (TCD) screening with indefinite chronic red cell transfusions (CRCT) for children with abnormal TCD as standard of care. To identify children at high-risk of stroke, annual TCD screening is recommended from ages 2 to 16 years, with more frequent monitoring if the result is not normal. A reduction in stroke incidence in children with SCD has been reported in several clinical series and analyses utilizing large hospital databases when comparing rates before and after the publication of the STOP study in 1998. We sought to determine the rate of first ischemic stroke in a multicenter cohort of children who had previously participated in the STOP and/or STOP 2 trials and to determine whether these strokes were screening or treatment failures. Subjects and Methods: Between 1995 and 2005, STOP and STOP 2 (STOP/2) were conducted at 26 sites in the US and Canada. These studies included 3,835 children, ages 2 to 16 y with SCD type SS or S-beta-0-thalassemia. Participation in STOP/2 ranged from a single screening TCD to randomization. STOP 2 also had an observational arm for children on CRCT for abnormal TCD whose TCD had not reverted to normal. The Post-STOP study was designed to follow-up the outcomes of children who participated in one or both trials. 19 of the 26 original study sites participated in Post-STOP, contributing a total of 3,539 (92%) of the STOP/2 subjects. After exit from STOP/2, these children received TCD screening and treatment according to local practices. Data abstractors visited each clinical site and obtained retrospective data from STOP/2 study exit to 2012-2014 (depending on site) including follow-up TCD and brain imaging results, clinical information, and laboratory results. Two vascular neurologists, blinded to STOP/2 status and prior TCD and neuroimaging results, reviewed source records to confirm all ischemic strokes, defined as a symptomatic cerebral infarction; discordant opinions were resolved through discussion. For the first Post-STOP ischemic stroke, prior TCD result and treatment history subsequently were analyzed. Results: Of the 3,539 subjects, follow-up data were available for 2,850 (81%). Twelve children who had a stroke during STOP or STOP2 were excluded from these analyses resulting in data on 2,838 subjects. The mean age at the start of Post-STOP was 10.5 y and mean duration of follow-up after exiting STOP/2 was 9.1 y. A total of 69 first ischemic strokes occurred in the Post-STOP observation period (incidence 0.27 per 100 pt years). The mean age at time of stroke was 14.4±6.2 (median 13.8, range 3.5-28.9) y. Twenty-five of the 69 patients (36%) had documented abnormal TCD (STOP/2 or Post-STOP) prior to the stroke; 15 (60%) were receiving CRCT and 9 (36%) were not (treatment data not available for 1 subject). Among the 44 subjects without documented abnormal TCD, 29 (66%) had not had TCD re-screen in the Post-STOP period prior to the event; 7 of these 29 (24%) were 16 y or older at the start of Post-STOP, which is beyond the recommended screening age. Four of the 44 (9%) patients had inadequate TCD in Post-STOP (1 to 10.7 y prior to event). Six (14%) had normal TCD more than a year before the event (1.2 - 4 y); all but one of these children were younger than 16 y at the time of that TCD. Only 5 (11%) had a documented normal TCD less than 1 year prior to the event. Conclusions: In the Post-STOP era, the rate of first ischemic stroke was substantially lower than that reported in the Cooperative Study of Sickle Cell Disease, prior to implementation of TCD screening. Many (39%) of the Post-STOP ischemic strokes were associated with a failure to re-screen according to current guidelines, while only 11% occurred in children who had had recent low-risk TCD. Among those known to be at high risk prior to stroke, treatment refusal or inadequate treatment may have contributed. While TCD screening and treatment are effective at reducing ischemic stroke in clinical practice, significant gaps in screening and treatment, even at sites experienced in the STOP protocol, remain to be addressed. Closing these gaps should provide yet further reduction of ischemic stroke in SCD. Disclosures No relevant conflicts of interest to declare.

scholarly journals HMOX1 and Acute Kidney Injury in Sickle Cell Anemia

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 686-686
Author(s):  
Santosh L. Saraf ◽  
Maya Viner ◽  
Ariel Rischall ◽  
Binal Shah ◽  
Xu Zhang ◽  
...  
Keyword(s):  
Chronic Kidney Disease ◽  
Acute Kidney Injury ◽  
Risk Factor ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Kidney Injury ◽  
Ckd Progression ◽  
Kdigo Guidelines

Abstract Acute kidney injury (AKI) is associated with tubulointerstitial fibrosis and nephron loss and may lead to an increased risk for subsequently developing chronic kidney disease (CKD). In adults with sickle cell anemia (SCA), high rates of CKD have been consistently observed, although the incidence and risk factors for AKI are less clear. We evaluated the incidence of AKI, defined according to Kidney Disease Improving Global Outcomes (KDIGO) guidelines as a rise in serum creatinine by ≥0.3mg/dL within 48 hours or ≥1.5 times baseline within seven days, in 158 of 299 adult SCA patients enrolled in a longitudinal cohort from the University of Illinois at Chicago. These patients were selected based on the availability of genotyping for α-thalassemia, BCL11A rs1427407, APOL1 G1/G2, and the HMOX1 rs743811 and GT-repeat variants. Median values and interquartile range (IQR) are provided. With a median follow up time of 66 months (IQR, 51-74 months), 137 AKI events were observed in 63 (40%) SCA patients. AKI was most commonly observed in the following settings: acute chest syndrome (25%), an uncomplicated vaso-occlusive crisis (VOC)(24%), a VOC with pre-renal azotemia determined by a fractional excretion of sodium <1% or BUN-to-creatinine ratio >20:1 (14%), or a VOC with increased hemolysis, defined as an increase in serum LDH or indirect bilirubin level >1.5 times over the baseline value at the time of enrollment (12%). Compared to individuals who did not develop AKI, SCA adults who developed an AKI event were older (AKI: median and IQR age of 35 (26-46) years, no AKI: 28 (23 - 26) years; P=0.01) and had a lower estimated glomerular filtration rate (eGFR) (AKI: median and IQR eGFR of 123 (88-150) mL/min/1.73m2, no AKI: 141 (118-154) mL/min/1.73m2; P=0.02) by the Kruskal-Wallis test at the time of enrollment. We evaluated the association of a panel of candidate gene variants with the risk of developing an AKI event. These included loci related to the degree of hemolysis (α-thalassemia, BCL11A rs1427407), to chronic kidney disease (APOL1 G1/G2 risk variants), and to heme metabolism (HMOX1) . Using a logistic regression model that adjusted for age and eGFR at the time of enrollment, the risk of an AKI event was associated with older age (10-year OR 2.6, 95%CI 1.4-4.8, P=0.002), HMOX1 rs743811 (OR 3.1, 95%CI 1.1-8.7, P=0.03), and long HMOX1 GT-repeats, defined as >25 repeats (OR 2.5, 95%CI 1.01-6.1, P=0.04). Next, we assessed whether AKI is associated with a more rapid decline in eGFR and with CKD progression, defined as a 50% reduction in eGFR, on longitudinal follow up. Using a mixed effects model that adjusted for age and eGFR at the time of enrollment, the rate of eGFR decline was significantly greater in those with an AKI event (β = -0.51) vs. no AKI event (β = -0.16) (P=0.03). With a median follow up time of 66 months (IQR, 51-74 months), CKD progression was observed in 21% (13/61) of SCA patients with an AKI event versus 9% (8/88) without an AKI event. After adjusting for age and eGFR at the time of enrollment, the severity of an AKI event according to KDIGO guidelines (stage 1 if serum creatinine rises 1.5-1.9 times baseline, stage 2 if the rise is 2.0-2.9 times baseline, and stage 3 if the rise is ≥3 times baseline or ≥4.0 mg/dL or requires renal replacement therapy) was a risk factor for CKD progression (unadjusted HR 1.6, 95%CI 1.1-2.3, P=0.02; age- and eGFR-adjusted HR 1.6, 95%CI 1.1-2.5, P=0.03). In conclusion, AKI is commonly observed in adults with sickle cell anemia and is associated with increasing age and the HMOX1 GT-repeat and rs743811 polymorphisms. Furthermore, AKI may be associated with a steeper decline in kidney function and more severe AKI events may be a risk factor for subsequent CKD progression in SCA. Future studies understanding the mechanisms, consequences of AKI on long-term kidney function, and therapies to prevent AKI in SCA are warranted. Disclosures Gordeuk: Emmaus Life Sciences: Consultancy.

scholarly journals Silent infarct is a risk factor for infarct recurrence in adults with sickle cell anemia

Neurology ◽  
2018 ◽  
Vol 91 (8) ◽  
pp. e781-e784 ◽  
Author(s):  
Lori C. Jordan ◽  
Adetola A. Kassim ◽  
Manus J. Donahue ◽  
Meher R. Juttukonda ◽  
Sumit Pruthi ◽  
...  
Keyword(s):  
Risk Factor ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cox Regression ◽  
Brain Mri ◽  
Treatment Strategies ◽  
Significant Risk ◽  
Rank Test ◽  
Event Analysis ◽  
Log Rank Test

ObjectiveBecause of the high prevalence of silent cerebral infarcts (SCIs) in adults with sickle cell anemia (SCA) and lack of information to guide treatment strategies, we evaluated the risk of recurrent SCIs and overt stroke in adults with SCA with preexisting SCI.MethodsThis observational study included adults with SCA (HbSS or Sβ0 thalassemia) aged 18 to 40 years. Participants received 3-tesla brain MRI and a detailed neurologic examination. Time-to-event analysis assessed those with or without baseline SCI and with new or progressive infarcts. The incidence rate of new events was compared by log-rank test. Univariable Cox regression assessed the association of SCI with infarct progression.ResultsAmong adults with SCA with 2 MRIs and at least 6 months between MRIs (n = 54, mean interval = 2.5 years), 43% had SCI at baseline. Of participants with baseline SCI, 30% had new or progressive SCI over 2.5 years compared to 6% with no SCI at baseline; no participant had an overt stroke. New SCIs at follow-up were present in 12.9 per 100 patient-years with existing SCI compared with 2.4 per 100 patient-years without prior SCI (log-rank test, p = 0.021). No statistically significant differences were seen among those with or without baseline SCI in use of hydroxyurea therapy, hydroxyurea dose, or other stroke risk factors. The presence of SCI was associated with increased hazard of a new or progressive infarct (hazard ratio 5.27, 95% confidence interval 1.09–25.51, p = 0.039).ConclusionsSilent infarcts in adults with SCA are common and are a significant risk factor for future silent infarcts.

scholarly journals Barriers to Pediatric Sickle Cell Disease Guideline Recommendations

2019 ◽  
Vol 6 ◽  
pp. 2333794X1984702 ◽  
Author(s):  
Michael D. Cabana ◽  
Julie Kanter ◽  
Anne M. Marsh ◽  
Marsha J. Treadwell ◽  
Michael Rowland ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Support Staff ◽  
Cell Disease ◽  
National Guidelines ◽  
Convenience Sample ◽  
Different Types ◽  
Pediatric Sickle Cell Disease

National guidelines recommend that providers counsel all patients with sickle cell anemia about hydroxyurea (HU) therapy and screen children with sickle cell anemia annually for the risk of stroke with transcranial Doppler (TCD). We surveyed a national convenience sample of sickle cell disease clinicians to assess factors associated with low adherence. Adherence was 46% for TCD screening. Low adherence was associated with a lack of outcome expectancy (eg, a belief that there would be poor patient follow-up to TCD testing; P < .05). Adherence was 72% for HU counseling. Practice barriers (eg, lack of support staff or time) and a lack of agreement with HU recommendations were associated with low adherence ( P < .05). This study demonstrates that different types of strategies are needed to improve TCD screening (to address follow-up and access to testing) versus HU counseling (to address physician agreement and practice barriers).

scholarly journals An objective sign in painful crisis in sickle cell anemia: the concomitant reduction of high density red cells

Blood ◽  
1984 ◽  
Vol 64 (2) ◽  
pp. 559-563 ◽  
Author(s):  
ME Fabry ◽  
L Benjamin ◽  
C Lawrence ◽  
RL Nagel
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
State Level ◽  
Red Cells ◽  
Average Decrease ◽  
Concomitant Reduction ◽  
Objective Sign ◽  
The Stability ◽  
Painful Crisis

Abstract The etiopathologic basis of painful crisis in sickle cell anemia is largely unknown, and no objective criteria for its diagnosis and follow- up exist at present. We have studied 11 patients through 14 painful crises and observed a significant decrease of the densest fraction of red cells in 12 of the 14 crises as determined by isopycnic Percoll- Stractan continuous density gradients. If the first observation is normalized to 100%, the average decrease in dense cells was 77% with a range of 36% to 94%. The time needed for the percentage of dense cells to return to the steady-state level varied from seven to more than 30 days. These findings were in sharp contrast to the stability of the density pattern observed in another group of sickle cell patients, who were studied during crisis-free periods. The mechanism of the disappearance of dense cells could involve selective destruction by the reticuloendothelial (RE) system, selective sequestration in the areas of vasoocclusion, or a combination of both factors.

scholarly journals ALLOIMMUNIZATION IN PATIENTS WITH SICKLE CELL DISEASE AND THALASSAEMIA: EXPERIENCE OF SINGLE CENTRE FROM OMAN

2017 ◽  
Vol 9 (1) ◽  
pp. e2017013 ◽  
Author(s):  
Anil Pathare ◽  
Salam Alkindi
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Supportive Care ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Significant Risk ◽  
Red Cell ◽  
Cell Disease ◽  
Single Centre ◽  
Screening And Identification

Background: Blood transfusion is an integral part of the supportive care for patients with sickle cell anemia and thalassaemia. The hazard of red cell alloimmunization, however, is one of the main complications of this therapy. Objectives: The aim of this study was to evaluate the incidence of red cell alloimmunization in Omani patients with sickle cell anemia and thalassaemia. Methods: This study included 262 patients whose historical transfusion records were available. One hundred and twenty-nine patients with thalassaemia who were attending the day care unit for regular transfusions, and 133 sickle cell anemia patients admitted at our hospital were included in this study. The Diamed gel system was used for the screening and identification of atypical antibodies. Results: The rate of alloimmunization in sickle cell anemia patients was 31% (n=41), whereas in thalassaemia patients it was 20% (n=26). Antibodies to E, e, C, c, D, K, S, Fyª, Kpª, Jkª and Cw were observed. Among the two groups, 8 developed nonspecific antibodies, and 12 developed more than one antibody; however, 85% of patients were also immunized to Rh and Kell antigens. Conclusions: Red cell transfusions are associated with a significant risk of alloimmunization. It is, therefore, imperative to perform an initial extended red cell phenotyping for both donors and recipients, and carefully select ABO, Rh and Kell matched donors.

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