ライフサイエンスコーパス: sickle

1 ach share the goal of preventing erythrocyte sickling.

2 role of cell density in the kinetics of cell sickling.

3 Physiologically, sickling always occurs when some ligands are present; no

4 ne induces SphK1 activity in human and mouse sickle and normal erythrocytes in vitro.

5 sickle cell disease (SCD) and contributes to sickling and disease progression.

6 Repeated sickling and ongoing haemolytic anaemia, even when subcl

7 gle mutant allele of Kcc1 induces widespread sickling and tissue damage, leading to premature death.

8 Sickling arises from nucleation-controlled polymer forma

9 ell mice led to a substantial improvement of sickle-associated hemolytic anemia and reticulocytosis,

10 can predict the fraction of cells that will sickle at any given partial ligand saturations.

11 zero-thalassemia [Sbeta(0)], 495 SC, and 161 sickle beta(+)-thalassemia [Sbeta(+)]), aged 3 years old

12 ses human alleles encoding normal alpha- and sickle beta-globin.

13 d Ivory Coast, 2407 SCD patients (1751 SS or sickle beta-zero-thalassemia [Sbeta(0)], 495 SC, and 161

14 Analysis of lung tissues from Berkeley sickle (BK-SS) mice showed increased levels of ATF3 and

15 ts of intravenous PFCE therapy in transgenic sickle cell (HbSS) mice infected with S. pneumoniae.

16 His mother has Sickle cell anaemia (Hb SS) and his father is a carrier

17 In sickle cell anaemia (homozygous HBBE6V; HbSS), plasma EP

18 ere elevated (P < 0.05) in participants with sickle cell anaemia (n = 27) not receiving monthly blood

19 tic resonance imaging method were applied in sickle cell anaemia (n = 34) and healthy race-matched co

20 Sickle cell anaemia (SCA) is associated with structural

21 For high-risk children with sickle cell anaemia and abnormal TCD velocities who have

22 For children with sickle cell anaemia and high transcranial doppler (TCD)

23 graphic abnormalities in adult patients with sickle cell anaemia in steady state attending the Haemat

24 Sickle cell anaemia is a monogenetic disorder with a hig

25 l quadrant ultrasonography of 50 consecutive sickle cell anaemia patients were compared with those of

26 We enrolled children with sickle cell anaemia who were aged 4-16 years and had abn

27 ygen extraction fraction in individuals with sickle cell anaemia with higher levels of clinical impai

28 screening procedures exist for children with sickle cell anaemia, no accepted screening procedures ex

29 biliary ultrasound findings in patients with sickle cell anaemia.

30 ol for evaluating stroke risk in adults with sickle cell anaemia.

31 of cerebrovascular impairment in adults with sickle cell anaemia.

32 hepatobiliary abnormalities in patients with sickle cell anaemia.

33 hn Kendrew sought to understand mutations in sickle cell and other genetic diseases related to hemogl

34 subset of 2388 children and adolescents with sickle cell anemia (50%) was enrolled for 2 or more cons

35 ransplantation for a cohort of children with sickle cell anemia (SCA) and abnormal transcranial Doppl

36 nitric oxide (Feno) levels in children with sickle cell anemia (SCA) is unclear, but increased level

37 a treatment is recommended for children with sickle cell anemia (SCA) living in high-resource malaria

38 e mainstay of stroke prevention in pediatric sickle cell anemia (SCA), but the physiology conferring

39 Stroke risk in sickle cell anemia (SCA), predicted by high transcranial

40 Sickle cell anemia (SCA)-related cardiomyopathy is chara

41 ations are the leading cause of mortality in sickle cell anemia (SCA).

42 both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1%

43 income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated th

44 Hemolytic diseases, such as sickle cell anemia and thalassemia, are characterized by

45 Current therapeutic strategies for sickle cell anemia are aimed at reactivating fetal hemog

46 example of risk factors for complications of sickle cell anemia from a longitudinal study with repeat

47 Sickle cell anemia is an inherited blood disorder that i

48 Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiolo

49 lization among children and adolescents with sickle cell anemia using administrative claims data.

50 or identifying children and adolescents with sickle cell anemia was recently developed and validated

51 en and adolescents 2 to 16 years of age with sickle cell anemia were identified by the presence of 3

52 d adolescents 2 through 17 years of age with sickle cell anemia were randomly assigned to receive ora

53 e can identify children and adolescents with sickle cell anemia who are at the highest risk of stroke

54 or more Medicaid claims with a diagnosis of sickle cell anemia within a calendar year (2005-2010).

55 ders such as complex regional pain syndrome, sickle cell anemia, or fibromyalgia.

56 In children with sickle cell anemia, routine use of transcranial Doppler

57 red fifty-nine adults with HCV infection and sickle cell anemia, thalassemia, or hemophilia A/B or vo

58 Among children and adolescents with sickle cell anemia, the rate of vaso-occlusive crisis wa

59 sion and thrombosis during vaso-occlusion in sickle cell anemia, which suggests a role for antiplatel

60 ractions among children and adolescents with sickle cell anemia.

61 mutant form h-HbS, which is responsible for sickle cell anemia.

62 atopoietic progenitors from individuals with sickle cell anemia.

63 and mechanical properties of sickle RBCs in sickle cell anemia.

64 ty and define a set of functional regimes of sickle cell blood flow personalized for each patient tha

65 y not present with clinically apparent acute sickle cell crises, but these milder forms can provide a

66 Patients with sickle cell disease (n = 17) and malaria (n = 15) contri

67 e (ACS) is a common, serious complication of sickle cell disease (SCD) and a leading cause of hospita

68 Pain is a life-long symptom in sickle cell disease (SCD) and a predictor of disease pro

69 ene (HBB; which encodes beta-globin), mainly sickle cell disease (SCD) and beta-thalassemia, become s

70 tment for beta-hemoglobinopathies, including sickle cell disease (SCD) and beta-thalassemia.

71 erythrocyte SphK1 activity is upregulated in sickle cell disease (SCD) and contributes to sickling an

72 eness (AHR) affects 55%-77% of children with sickle cell disease (SCD) and occurs even in the absence

73 -independent EPO regulation, we assessed two sickle cell disease (SCD) cohorts for genetic associatio

74 Sickle cell disease (SCD) complications are associated w

75 CRISPR/Cas enhanced correction of the sickle cell disease (SCD) genetic defect in patient-spec

76 ce the frequency of vaso-occlusive episodes, sickle cell disease (SCD) has continued to be treated pr

77 globin expression and the pathophysiology of sickle cell disease (SCD) in a NRF2 knockout (SCD/NRF2(-

78 and sustainability of newborn screening for sickle cell disease (SCD) in sub-Saharan Africa and othe

79 Sickle cell disease (SCD) is a hematological disorder le

80 Sickle cell disease (SCD) is a highly complex genetic bl

81 Sickle cell disease (SCD) is a major global health conce

82 Sickle cell disease (SCD) is a severe genetic blood diso

83 entral nervous system (CNS) complications in sickle cell disease (SCD) is evolving.

84 ymptom of glomerular injury in patients with sickle cell disease (SCD) is microalbuminuria.

85 Although a blood genetic disease, sickle cell disease (SCD) leads to a chronic vasculopath

86 ective and more potent inhibitor, RN-1, in a sickle cell disease (SCD) mouse model.

87 gh hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North Ame

88 Effective medical management for sickle cell disease (SCD) remains elusive.

89 of vaso-occlusive crises (VOC) or events in sickle cell disease (SCD) remains limited to symptom rel

90 Patients with sickle cell disease (SCD) suffer from intense pain that

91 ll-free heme in plasma from 47 patients with sickle cell disease (SCD) was sequestered in circulating

92 Sickle cell disease (SCD), a congenital hemolytic anemia

93 ythrocyte arachidonic acid (AA) in mice with sickle cell disease (SCD), a prevalent hemolytic genetic

94 In sickle cell disease (SCD), abnormal adhesion of RBCs to

95 pportive therapy to prevent complications of sickle cell disease (SCD), access to care is not univers

96 ingosine 1-phosphate (S1P) is detrimental in Sickle Cell Disease (SCD), but the mechanistic basis rem

97 prophylaxis is recommended for persons with sickle cell disease (SCD), but the value of this has bee

98 ould benefit chronic pain conditions such as sickle cell disease (SCD), for which patients may requir

99 cts approximately 10% of adult patients with sickle cell disease (SCD), particularly those with the h

100 In sickle cell disease (SCD), treatment of recurrent vasooc

101 Sickle cell disease (SCD)-associated nephropathy is a ma

102 Cs) are in clinical trials for patients with sickle cell disease (SCD).

103 helin-1 (ET-1), a potent vasoconstrictor, in sickle cell disease (SCD).

104 een a long-standing goal in the treatment of sickle cell disease (SCD).

105 reportedly occurs in 10% of adolescents with sickle cell disease (SCD).

106 dhesion, correlate with clinical severity in sickle cell disease (SCD).

107 ons are of vital importance in patients with sickle cell disease (SCD).

108 natal health outcomes in pregnant women with sickle cell disease against a comparative group of pregn

109 adolescents with sickle cell disease aged 12-19 years, primary data on ad

110 The beta-haemoglobinopathies, such as sickle cell disease and beta-thalassaemia, are caused by

111 ion of fetal hemoglobin (HbF) in people with sickle cell disease and beta-thalassemia.

112 erentiating erythroid cells from people with sickle cell disease and in myeloma patients.

113 imilarities in the experience of living with sickle cell disease and living with other chronic illnes

114 ase that manifests clinical complications in sickle cell disease and other chronic hereditary or acqu

115 lobinopathies or globin disorders, including sickle cell disease and thalassemia.

116 Patients with sickle cell disease are at high risk for chronic hepatit

117 tion of the Glu6Val mutation responsible for sickle cell disease by using patient-derived stem and pr

118 Temporal macular involvement in sickle cell disease can now easily be detected by optica

119 Sickle cell disease contributes substantially to mortali

120 Living with a long-term condition such as sickle cell disease during adolescence constitutes a sig

121 The study population was all patients with sickle cell disease enrolled before March 31, 2015, in t

122 Three patients with sickle cell disease exhibiting preserved visual acuity b

123 Children with sickle cell disease experience organ damage, impaired qu

124 Sickle cell disease impacts on multiple facets of an ado

125 rations made in a single referral center for sickle cell disease in 2016.

126 Microvascular abnormalities in sickle cell disease involved both the superficial and th

127 Sickle cell disease is a common and life-threatening hae

128 The majority of morbidity and mortality in sickle cell disease is caused by vaso-occlusion: circula

129 Sickle cell disease is fundamentally a kinetic disorder,

130 own for more than 60 years that the cause of sickle cell disease is polymerization of a hemoglobin mu

131 Sickle Cell Disease is the commonest monogenic haemoglob

132 Although the root cause of sickle cell disease is the polymerization of hemoglobin

133 Patients with sickle cell disease may develop various macular vascular

134 Temporal macular atrophy in sickle cell disease may have direct consequences on visu

135 eme in pregnant women either due to malaria, sickle cell disease or other hemolytic diseases, will en

136 troversies include the role of haemolysis in sickle cell disease pathophysiology, optimal management

137 essel density was significantly lower in the sickle cell disease patients than in the control group i

138 avascular zone) was significantly larger in sickle cell disease patients than in the control group,

139 Sickle cell disease results from a homozygous missense m

140 For adolescents with sickle cell disease to be cared for and supported approp

141 ring adolescents' experiences of living with sickle cell disease to make recommendations for practice

142 Sickle cell disease was less common in children older th

143 years) with electrophoretic confirmation of sickle cell disease were included and analyzed.

144 assess the visual function of patients with sickle cell disease with no visual symptoms despite temp

145 res et Drepanocytose, ie, Heart Arteries and Sickle Cell Disease) study prospectively recruited pedia

146 ickle cell disease; 26,151,746 women without sickle cell disease) were eligible for inclusion.

147 f 41), and 89.4% (42 of 47) of patients with sickle cell disease, beta-thalassemia, and hemophilia A/

148 babies per year are thought to be born with sickle cell disease, but accurate data are not available

149 In patients with sickle cell disease, crizanlizumab therapy resulted in a

150 free iron during hemolytic diseases such as sickle cell disease, dengue fever, malaria, and sepsis.

151 In patients with sickle cell disease, donor and recipient red cell phenot

152 y be warranted when evaluating patients with sickle cell disease, even if asymptomatic with 20/20 vis

153 will help to inform national strategies for sickle cell disease, including neonatal screening.

154 e uveitis, systemic lupus erythematosus, and sickle cell disease, respectively, as well as in 41 age-

155 In several genetic conditions, including sickle cell disease, thalassemia, and G6PD deficiency, e

156 y disorders of erythrocyte hydration include sickle cell disease, thalassemia, hemoglobin CC, and her

157 ities in RBCs, including altered hematocrit, sickle cell disease, thalassemia, hemolytic anemias, and

158 literature about how adolescent's experience sickle cell disease, this body of research has not been

159 c nephropathy in European Americans and with sickle cell disease-associated nephropathy.

160 cations among HPC transplant recipients with sickle cell disease.

161 ogenitor cell (HPC) transplantation can cure sickle cell disease.

162 bin (HbS) is the primary pathogenic event of sickle cell disease.

163 ar atrophy, cystic fibrosis, haemophilia and sickle cell disease.

164 ng the subcellular and cellular phenomena in sickle cell disease.

165 emolytic anemia, anemia of inflammation, and sickle cell disease.

166 me excess alters the macrophage phenotype in sickle cell disease.

167 ads to formation of HbS (alpha2beta(S)2) and sickle cell disease.

168 or a clinical trial application for treating sickle cell disease.

169 eased in patients with autoimmune uveitis or sickle cell disease.

170 of the most important pathologic process in sickle cell disease.

171 ion and its pathophysiologic consequences in sickle cell disease.

172 rovide a unique view of the kidney injury in sickle cell disease.

173 maging of the perifoveal microvasculature in sickle cell disease.

174 Most children had either malaria or sickle cell disease.

175 comparative group of pregnant women without sickle cell disease.

176 n a variety of diseases including cancer and sickle cell disease.

177 molytic diseases and conditions, sepsis, and sickle cell disease.

178 P-selectin, were evaluated in patients with sickle cell disease.

179 pt clinical trial in 12 subjects with stable sickle cell disease.

180 or a severe cerebral vasculopathy related to sickle cell disease.

181 on and acute lung injury in murine models of sickle cell disease.

182 increase muscle blood flow in patients with sickle cell disease.

183 nd doubled muscle perfusion in patients with sickle cell disease.

184 All 3 patients included had homozygous sickle cell disease.

185 nty-one studies (including 26,349 women with sickle cell disease; 26,151,746 women without sickle cel

186 The findings were compared by sickle cell genotype and retinopathy stage and correlate

187 ssociation was observed between Sl genotype, sickle cell genotype, alpha+thalassaemia genotype, gende

188 Sickle cell genotypes included 27 patients with hemoglob

189 n case series of adult patients with various sickle cell genotypes.

190 lar flow are common in patients with various sickle cell genotypes.

191 angiography (OCT-A) in patients with various sickle cell genotypes.

192 an malaria patients who are heterozygous for sickle cell hemoglobin occupy a small area of RBCs by re

193 hy is a common complication in patients with sickle cell hemoglobinopathies.

194 n of gene-modified murine HSCs from Berkeley sickle cell mice led to a substantial improvement of sic

195 g symptoms, lung function measures, or prior sickle cell morbidity but were associated with markers o

196 Sickle cell nephropathy can occur in patients with homoz

197 Sickle cell nephropathy is a common complication in pati

198 ell injury in the medulla that contribute to sickle cell nephropathy.

199 Sickle cell numbers ranged from 631 (4.6%) for trait and

200 Sickle cell patients often require monthly transfusions

201 The prevalence of both the sickle cell trait (10/218 [4.6%]) and homozygous alpha+t

202 OL1 alleles that were directly genotyped and sickle cell trait (hemoglobin subunit beta gene [HBB] va

203 sk genotypes, hemoglobin variants, including sickle cell trait (SCT) and hemoglobin C trait, have a r

204 s relationship may differ between those with sickle cell trait (SCT) and those without it.

205 The contribution of sickle cell trait (SCT) to racial disparities in cardiop

206 um malaria in its heterozygous state, called sickle cell trait (SCT).

207 Prevalence of sickle cell trait and disease were high in Uganda, with

208 of the study was to calculate prevalence of sickle cell trait and disease.

209 th sickle cell trait except for the cases of sickle cell trait associated with systemic arterial hype

210 Studies have suggested that sickle cell trait elevates the risks of exertional rhabd

211 an uncommon complication in individuals with sickle cell trait except for the cases of sickle cell tr

212 We conducted a study of sickle cell trait in relation to these outcomes, control

213 rhabdomyolysis and death varied according to sickle cell trait status among 47,944 black soldiers who

214 -matched control (n = 11) volunteers without sickle cell trait to assess whole-brain oxygen extractio

215 The overall number of children with sickle cell trait was 12,979 (13.3%) and with disease wa

216 Sickle cell trait was not associated with a higher risk

217 Sickle cell trait was seen in all districts.

218 nce in the risk of death among soldiers with sickle cell trait, as compared with those without the tr

219 llow for real-time transfusion monitoring in sickle cell treatment settings and therefore improve wor

220 valuable therapeutic intervention for acute sickle cell vasoocclusive crises.

221 The secondary end points were the rate of sickle cell-related pain and the intensity of pain, whic

222 py resulted in a significantly lower rate of sickle cell-related pain crises than placebo and was ass

223 The primary end point was the annual rate of sickle cell-related pain crises with high-dose crizanliz

224 ed in the pathogenesis of vaso-occlusion and sickle cell-related pain crises.

225 ment of donor cells and full correction of a sickle-cell anemia model.

226 demic sub-Saharan Africa, where the greatest sickle-cell burden exists, remain unknown.

227 on, with symptoms defining the acute painful sickle-cell crisis.

228 Sickle-cell disease affects millions of individuals worl

229 ardiovascular complications in patients with sickle-cell disease and discuss how screening and interv

230 Asthma is common in children with sickle-cell disease and is associated with increased inc

231 s increased about asthma as a comorbidity in sickle-cell disease and its effects on morbidity, substa

232 Although the molecular cause of sickle-cell disease has been known for more than half a

233 have reduced the morbidity and mortality of sickle-cell disease in developed countries.

234 The burden of sickle-cell disease is expected to continue to rise over

235 ion of fetal haemoglobin to the forefront of sickle-cell disease research.

236 pport the design of innovative therapies for sickle-cell disease that are based on fetal haemoglobin.

237 In sickle-cell disease, a point mutation in the beta-globin

238 globin disorders, including thalassaemia and sickle-cell disease, are the most common monogenic disea

239 cause of acute lung disease in children with sickle-cell disease.

240 is a promising strategy for the treatment of sickle-cell disease.

241 cleation of ordered solids such as crystals, sickle-cell hemoglobin polymers, and amyloid fibrils.

242 ed to investigate the dynamics of individual sickle cells in a capillary-like microenvironment in ord

243 provide unique insights into how individual sickle cells move through capillaries under transient hy

244 id pathological cells such as thalassemic or sickle cells, and even to interactions of soft/stiff can

245 hemoglobin F, hemoglobin polymerization and sickling, coagulation, and platelet activation.

246 ta-like-globin chains) without recurrence of sickle crises and with correction of the biologic hallma

247 e heme/iron accumulation in a mouse model of sickle disease trigger similar proinflammatory phenotypi

248 al link phosphatidylserine (PS) exposure in sickle erythrocytes to a physiological event in reticulo

249 receive monthly transfusions to maintain 30% sickle haemoglobin or lower, while those assigned to the

250 reased erythrocyte S1P binds to deoxygenated sickle Hb (deoxyHbS), facilitates deoxyHbS anchoring to

251 rstanding of intracellular polymerization of sickle hemoglobin (HbS) and subsequent interaction with

252 y-free paper-based test capable of detecting sickle hemoglobin (HbS) in newborn blood samples with a

253 lity, is driven by polymerization of mutated sickle hemoglobin (HbS) in red blood cells (RBCs).

254 Polymerization of sickle hemoglobin (HbS) is the primary pathogenic event

255 sing blood samples from 25 SCD patients with sickle hemoglobin (HbS) levels varying from 64 to 90.1%,

256 d comparatively little toxicity, and reduced sickle hemoglobin (HbS) synthesis as well as sickling of

257 and thus increases deoxyHbS polymerization, sickling, hemolysis and disease progression.

258 oglobin S; HbS) will cause occlusion if they sickle in the microvasculature, but have minimal (or no)

259 ut have minimal (or no) consequences if they sickle in the venous return.

260 induced LysoPC production directly promotes sickling in cultured mouse and human SCD erythrocytes.

261 affinity, one of them (GBT440) also inhibits sickling in the absence of oxygen by two additional mech

262 ent and circulating AA levels and attenuated sickling, inflammation and tissue damage in SCD chimeras

263 mediators both peripherally and centrally in sickle mice correlative to the antinociceptive response.

264 Importantly, in sickle mice, the administration of human exogenous hemop

265 nd neuromodulation, and how pain affects the sickle microenvironment and pathophysiology.

266 Subsequently, the sickle mutation was corrected by delivering CRISPR/Cas9

267 sickle hemoglobin (HbS) synthesis as well as sickling of SCD erythroid cells under hypoxic conditions

268 pain, pathways specific to chronic and acute sickle pain, perception-based targets of "top-down" mech

269 ps in knowledge are identified regarding how sickle pathobiology evokes pain, pathways specific to ch

270 red that 20% DMC is necessary to reverse the sickle phenotype.

271 ce of oxygen is the key to understanding how sickling proceeds in a physiological context.

272 In particular, the dense and rigid sickle RBCs are obstructed thereby clogging blood flow w

273 ed morphologies and mechanical properties of sickle RBCs in sickle cell anemia.

274 the morphologies and membrane stiffening of sickle RBCs.

275 Abnormal sickle red blood cell (sRBC) biomechanics, including pat

276 vaso-occlusion induced by integrin-dependent sickle-red blood cell-leukocyte adhesion.

277 Management of adults with sickle-related pulmonary hypertension is based on antico

278 Sickle retinopathy defined by biomicroscopic changes, Go

279 These findings suggest that pediatric sickle retinopathy may be more prevalent than previously

280 Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescen

281 eyes with adequate UWFA studies demonstrated sickle retinopathy.

282 e approaches may enhance early screening for sickle retinopathy.

283 y of consanguinity in the parents revealed a sickle S trait associated to heterozygous alpha thalasse

284 parafoveal subfields of the deep plexus with sickle SC or proliferative retinopathy.

285 nopathy stage but may be more extensive with sickle SC or proliferative retinopathy.

286 proposed framework can successfully classify sickle shape RBCs in an automated manner with high accur

287 Abnormal sickle-shaped erythrocytes disrupt blood flow in small v

288 ATOR7 promoter yielded a novel allele of the SICKLE (SIC) gene.

289 ing pathway and ETHYLENE INSENSITIVE2 (EIN2)/SICKLE (SKL), but acts independently of SUPER NUMERIC NO

290 uman healthy (wild-type (WT)) and homozygous sickle (SS) red blood cells (RBCs) express a large numbe

291 We did the cross-sectional Uganda Sickle Surveillance Study to assess the burden of diseas

292 mploy a kinetic model for cell morphological sickling that invokes parameters derived from patient-sp

293 t the precise time at which fibers distort ("sickle") the cells.

294 SC (30.4%), 4 beta-thalassemia (8.7%), and 1 sickle trait (2.2%).

295 is based on laser-induced polymerization in sickle trait cells and robust, automated image analysis

296 l samples to identify those from babies with sickle trait or disease.

297 nce of heterozygous alpha + -thalassemia and sickle trait.

298 ncrease of reactive oxygen species (ROS) and sickling under hypoxic conditions.

299 nflammatory, proadhesive state that promotes sickle vaso-occlusion and acute lung injury in murine mo

300 However, the hypoxia-affected cells undergo sickling which significantly alters cell dynamics.