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 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.

Moyamoya syndrome associated with neurofibromatosis Type 1: perioperative and long-term outcome after surgical revascularization

2013 ◽  
Vol 11 (4) ◽  
pp. 417-425 ◽  
Author(s):  
McKenzie Koss ◽  
R. Michael Scott ◽  
Mira B. Irons ◽  
Edward R. Smith ◽  
Nicole J. Ullrich
Keyword(s):  
Perioperative Complications ◽  
Neurofibromatosis Type ◽  
Neurological Status ◽  
Neurological Deficits ◽  
Moyamoya Syndrome ◽  
Surgical Revascularization ◽  
Radiographic Evidence

Object Children with neurofibromatosis Type 1 (NF1) can present with progressive arteriopathy of the branches of the internal carotid artery consistent with moyamoya syndrome. Clinical symptoms, radiographic evidence of ischemia, and the potential for disease progression may necessitate surgical revascularization to minimize the risk of stroke and progressive neurological deficits. This study aims to evaluate the presentation and surgical outcomes of these patients by reviewing clinical, radiographic, and angiographic data. Methods A retrospective review was conducted of clinical and radiographic records of all children with NF1 who were diagnosed with moyamoya syndrome and underwent surgical revascularization between January 1988 and April 2012 at Boston Children's Hospital. Results During this period, 39 patients (27 female and 12 male, ages 0.2–19.3 years) had both NF1 and moyamoya syndrome, of whom 32 underwent surgical revascularization with pial synangiosis. Of the 32 patients treated by surgical revascularization, 21 (66%) manifested ischemic symptoms and 18 (56%) had radiographic evidence of prior stroke at the time of moyamoya diagnosis. In total, 25 of 32 patients developed neurological symptoms prior to surgical intervention. Only 1 patient presented with hemorrhage. The average age at first surgery was 8.1 years (range 0.5–15.6 years). Perioperative complications in the first 7 days included stroke (n = 2), transient ischemic attack (n = 1), and infection (n = 1). Twenty-two patients had more than 6 months of follow-up, with an average clinical and radiographic postoperative follow-up period of 80.2 months (range 9.4–257.1 months). Of those patients with long-term follow-up, 21 (95%) of 22 demonstrated stable or improved neurological status despite radiographic evidence of moyamoya progression in 48% of patients. Conclusions Children with NF1-associated moyamoya syndrome are often diagnosed prior to development of fixed neurological deficits as a consequence of imaging studies obtained for other manifestations of NF1. The clinical, radiographic, and angiographic features in this population are comparable to primary moyamoya disease, with the exception of patients treated with cranial irradiation, who may be at greater risk for both stroke as well as perioperative complications. Despite radiographic evidence of progressive stenosis in 48% of patients, nearly all demonstrated stable or improved neurological status after surgical revascularization. Surgical revascularization for children with NF1 appears safe and is protective against further ischemic and neurological damage, with a 27-fold reduction in stroke rate.

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.

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.

Pilonidal Sinus: How to Choose Between Excision and Open Granulation Versus Excision and Primary Closure?

Swiss Surgery ◽  
2002 ◽  
Vol 8 (6) ◽  
pp. 255-258 ◽  
Author(s):  
Perruchoud ◽  
Vuilleumier ◽  
Givel
Keyword(s):  
Hospital Stay ◽  
Pilonidal Sinus ◽  
Primary Closure ◽  
Average Length ◽  
Length Of Hospital Stay ◽  
Healing Time ◽  
Incision And Drainage ◽  
The Mean ◽  
Primary Healing

Aims: The purpose of this study was to evaluate excision and open granulation versus excision and primary closure as treatments for pilonidal sinus. Subjects and methods: We evaluated a group of 141 patients operated on for a pilonidal sinus between 1991 and 1995. Ninety patients were treated by excision and open granulation, 34 patients by excision and primary closure and 17 patients by incision and drainage, as a unique treatment of an infected pilonidal sinus. Results: The first group, receiving treatment of excision and open granulation, experienced the following outcomes: average length of hospital stay, four days; average healing time; 72 days; average number of post-operative ambulatory visits, 40; average off-work delay, 38 days; and average follow-up time, 43 months. There were five recurrences (6%) in this group during the follow-up period. For the second group treated by excision and primary closure, the corresponding outcome measurements were as follows: average length of hospital stay, four days; average healing time, 23 days; primary healing failure rate, 9%; average number of post-operative ambulatory visits, 6; average off-work delay, 21 days. The average follow-up time was 34 months, and two recurrences (6%) were observed during the follow-up period. In the third group, seventeen patients benefited from an incision and drainage as unique treatment. The mean follow-up was 37 months. Five recurrences (29%) were noticed, requiring a new operation in all the cases. Discussion and conclusion: This series of 141 patients is too limited to permit final conclusions to be drawn concerning significant advantages of one form of treatment compared to the other. Nevertheless, primary closure offers the advantages of quicker healing time, fewer post-operative visits and shorter time off work. When a primary closure can be carried out, it should be routinely considered for socio-economical and comfort reasons.

scholarly journals Why do frail patients with chronic heart failure die and become hospitalised?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Sze ◽  
P Pellicori ◽  
J Zhang ◽  
J Weston ◽  
A.L Clark
Keyword(s):  
Heart Failure ◽  
Cause Of Death ◽  
Hospital Admissions ◽  
Average Length ◽  
Significant Proportion ◽  
Length Of Hospital Stay ◽  
Clinical Frailty Scale ◽  
Frail Patients ◽  
Increased Risk

Abstract Background Frailty is common in patients with heart failure (HF) and is associated with increased morbidity and mortality. A better understanding of the causes of hospitalisations and death in frail patients might help to tailor interventional strategies for these at-risk patients. Purpose We studied the cause of death and hospitalisations in ambulatory patients with HF and frailty. Methods We assessed frailty using the clinical frailty scale (CFS) in consecutive HF patients attending a routine follow-up visit. Those with CFS ≥5 were classified as frail. Mortality and hospitalisations were ascertained from medical records (updated systematically using an NHS electronic database), discharge letters, autopsy reports and death certificates. We studied the primary cause of death and hospitalisations within one year of enrolment. Results 467 patients (67% male, median (IQR) age 76 (69–82) years, median (IQR) NT-proBNP 1156 (469–2463) ng/L) were enrolled. 206 (44%) patients were frail. Frail patients were more likely to not receive or receive suboptimal doses of ACEi/ARB and Beta-blockers; while non-frail patients were more likely to be treated with optimal doses. At 1-year follow up, there were 56 deaths and 322 hospitalisations, of which 46 (82%) and 215 (67%) occurred in frail patients. Frailty was associated with an increased risk of all-cause mortality (HR (95% CI): 4.27 (2.60–7.01)) and combined mortality/ hospitalisation (HR (95% CI): 2.85 (2.14–3.80)), all p<0.001. 57% (n=26) of frail patients died of cardiovascular causes (of which 58% were due to HF progression); although deaths due to non-cardiovascular causes (43%, n=20), especially severe infections, were also common (26%, n=12). (Figure 1) The proportion of frail patients who had non-elective hospital admissions within 1 year was more than double that of non-frail patients (46% (n=96) vs 21% (n=54); p<0.001). Compared to non-frail patients, frail patients had more recurrent (≥2) hospitalisations (28% (n=59) vs 9% (n=24); p<0.001) but median (IQR) average length of hospital stay was not significantly different (frail: 6 (4–11) vs non-frail: 6 (2–12) days, p=0.50). A large proportion of hospitalisations (64%, n=137) in frail patients were due to non-cardiovascular causes (of which 34%, 30% and 20% were due to infections, falls and comorbidities respectively). Of cardiovascular hospitalisations (36%, n=78), the majority were due to decompensated HF (67%, n=46). (Figure 1) Conclusion Frailty is common in patients with HF and is associated with an increased risk of mortality and recurrent hospitalisations. A significant proportion suffered non-cardiovascular deaths and hospitalisations. This implies that interventions targeted at HF alone can only have limited impact on outcomes in frail patients. Figure 1 Funding Acknowledgement Type of funding source: None

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 Case Report: Acute Stroke in Young Adult Patient with Moyamoya Syndrome Secondary to Sickle Cell Disease

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 13-13
Author(s):  
Oladipo Cole ◽  
Asia Filatov ◽  
Javed Khanni ◽  
Patricio Espinosa
Keyword(s):  
Case Report ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Moyamoya Disease ◽  
Conflicts Of Interest ◽  
Acute Chest Syndrome ◽  
African American Female ◽  
Moyamoya Syndrome ◽  
Cell Disease ◽  
Radiographic Findings

Moyamoya disease, well described in literature, is a chronic cerebrovascular occlusive disorder. It is characterized by progressive stenosis/occlusion of the terminal portions of the internal carotid arteries (ICA) and the proximal portions of the middle cerebral arteries (MCA). Less frequently described is Moyamoya syndrome, the name given to radiographic findings consistent with Moyamoya disease, but with an identifiable cause. The diseases associated with Moyamoya Syndrome include Sickle Cell Disease (SCD), Thalassemias, and Down's Syndrome to name a few. Common complications of Moyamoya include both ischemic and hemorrhagic strokes. Upon literature review, Moyamoya syndrome caused by SCD is not well described. When it is, the discussion is centered around the pediatric patient population and surgical management. Our case report describes a 22-year-old African American female with SCD who initially presented with Acute Chest Syndrome. Her hospital course was complicated by development of overt debilitating neurologic deficits. Subsequently, she was found to have Moyamoya Syndrome on neuroimaging. She was successfully treated with medical management without any surgical intervention. This case highlights the necessity of thorough examination, differential diagnosis, imaging findings, and consideration of predisposing syndromes in the work-up for Moyamoya syndrome; especially individuals with Sickle Cell Disease. Disclosures No relevant conflicts of interest to declare.

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