ライフサイエンスコーパス: sickle cell

1 d differences in the densities of normal and sickled cells.

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

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

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

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

6 Sickle cell anaemia (SCA) is associated with structural

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

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

9 19, 2013, we enrolled 18 adult patients with sickle cell anaemia and leg ulcers into our trial.

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

11 Sickle cell anaemia is a monogenetic disorder with a hig

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

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

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

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

16 biliary ultrasound findings in patients with sickle cell anaemia.

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

18 of cerebrovascular impairment in adults with sickle cell anaemia.

19 hepatobiliary abnormalities in patients with sickle cell anaemia.

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

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

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

23 avenous magnesium to saline in children with sickle cell anemia (SCA) admitted to the hospital for ac

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

25 Sickle cell anemia (SCA) is a hemoglobinopathy leading t

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

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

28 Hoban et al show in situ gene correction of sickle cell anemia (SCA), a prototypical hemoglobinopath

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

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

31 anial Doppler (TCD) screening of the Creteil sickle cell anemia (SCA)-newborn cohort, and rapid initi

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

33 nt cerebral infarcts (SCIs) in children with sickle cell anemia (SCA).

34 n (PHT) is associated with high mortality in sickle cell anemia (SCA).

35 lications result in mortality in adults with sickle cell anemia (SCA).

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

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

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

39 The thalassemias, together with sickle cell anemia and its variants, are the world's mos

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

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

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

43 Sickle cell anemia is a blood disorder, known to affect

44 s a frequently inherited blood disorder, and sickle cell anemia is a common type of hemoglobinopathy.

45 Sickle cell anemia is an inherited blood disorder that i

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

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

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

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

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

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

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

53 from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to

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

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

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

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

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

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

60 atopoietic progenitors from individuals with sickle cell anemia.

61 cus pneumoniae pathogenesis in patients with sickle cell anemia.

62 oglobinopathies such as beta-thalassemia and sickle cell anemia.

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

64 ractions among children and adolescents with sickle cell anemia.

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

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

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

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

69 ssion injury causing global ocular ischemia, sickle cell crisis, Purtscher's retinopathy, inflammator

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

71 I 2.82-7.55), prematurity (4.33, 2.47-7.58), sickle cell disease (3.46, 1.63-7.37), immunosuppression

72 onsecutive patients (3 men and 2 women) with sickle cell disease (4 patients with hemoglobin SS disea

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

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

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

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

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

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

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

80 Sickle cell disease (SCD) and thalassemias (Thal) are co

81 Human immunodeficiency virus (HIV) and sickle cell disease (SCD) are regarded as endemic in ove

82 and high counts of circulating HSC/P seen in sickle cell disease (SCD) as a result of vascular damage

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

84 Sickle cell disease (SCD) complications are associated w

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

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

87 Patients with sickle cell disease (SCD) have markers of chronic inflam

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

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

90 Sickle cell disease (SCD) is a genetic disorder that pos

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

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

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

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

95 Sickle cell disease (SCD) is a worldwide distributed her

96 Sickle cell disease (SCD) is caused by genetic defects i

97 Sickle cell disease (SCD) is characterized by a single p

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

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

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

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

102 potheses relating intravascular hemolysis to sickle cell disease (SCD) pathogenesis.

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

104 oses reduces platelet adhesion but increases sickle cell disease (SCD) red blood cell (RBC) adhesion

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

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

107 Sickle cell disease (SCD) results in vascular occlusions

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

109 ) induction is a well-validated strategy for sickle cell disease (SCD) treatment.

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

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

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

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

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

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

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

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

118 Hydroxyurea (HU), a common therapy for sickle cell disease (SCD), induces fetal Hb production a

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

120 tients after splenectomy or in patients with sickle cell disease (SCD), the number of circulating red

121 ts with hematologic disorders, most commonly sickle cell disease (SCD), there is significant concern

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

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

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

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

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

127 onary hypertension has been controversial in sickle cell disease (SCD).

128 , and survival advantage in a mouse model of sickle cell disease (SCD).

129 processes modulating the pathophysiology of sickle cell disease (SCD).

130 ular event that leads to hemolytic anemia in sickle cell disease (SCD).

131 diate vaso-occlusive events in patients with sickle cell disease (SCD).

132 a major cause of morbidity and mortality in sickle cell disease (SCD).

133 ion and injury, and promote vasoocclusion in sickle cell disease (SCD).

134 cations for efforts to prevent thrombosis in sickle cell disease (SCD).

135 revalent and debilitating hemolytic disorder sickle cell disease (SCD).

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

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

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

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

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

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

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

143 dult erythrocytes can reduce the severity of sickle cell disease and beta-thalassemia.

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

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

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

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

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

149 and the development of a device to identify sickle cell disease based on aqueous multiphase systems

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

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

152 Sickle cell disease contributes substantially to mortali

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

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

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

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

157 Sickle cell disease impacts on multiple facets of an ado

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

159 onducted to quantify the association between sickle cell disease in pregnancy and adverse maternal an

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

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

162 Pregnancy in women with sickle cell disease is associated with adverse maternal

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

164 Sickle cell disease is fundamentally a kinetic disorder,

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

166 Sickle Cell Disease is the commonest monogenic haemoglob

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

168 Patients with sickle cell disease may develop various macular vascular

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

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

171 th HIV or malaria, or who are compromised by sickle cell disease or severe malnutrition.

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

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

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

175 Sickle cell disease results from a homozygous missense m

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

177 mutant mice with a humanized mouse model of sickle cell disease to directly explore the relevance of

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

179 Sickle cell disease was less common in children older th

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

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

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

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

184 the hemoglobinopathies (beta-thalassemia and sickle cell disease); rare genetic disorders of RBC prod

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

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

187 a variety of important pathologies including sickle cell disease, cancer, inflammation, and fibrosis.

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

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

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

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

192 ort the inclusion of neurological disorders, sickle cell disease, immunosuppression, diabetes, and ag

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

194 Despite advances in the management of sickle cell disease, obstetrics, and neonatal medicine,

195 ren (aged 6-60 months), most with malaria or sickle cell disease, presenting February 2013 through Ma

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

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

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

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

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

201 h SCA, followed in the Cooperative Study for Sickle Cell Disease, was constructed using the first pul

202 h HbSS genotype, compared with women without sickle cell disease, were at increased risk of maternal

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

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

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

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

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

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

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

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

211 ion and its pathophysiologic consequences in sickle cell disease.

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

213 maging of the perifoveal microvasculature in sickle cell disease.

214 Most children had either malaria or sickle cell disease.

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

216 comparative group of pregnant women without sickle cell disease.

217 end-organ damage and diminished survival in sickle cell disease.

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

219 adult beta-hemoglobin: beta-thalassemia and sickle cell disease.

220 ular vascular abnormalities in patients with sickle cell disease.

221 maternal and neonatal outcomes in women with sickle cell disease.

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

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

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

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

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

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

228 All 3 patients included had homozygous sickle cell disease.

229 cations among HPC transplant recipients with sickle cell disease.

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

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

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

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

234 maternal and neonatal outcomes in women with sickle cell disease; however, the evidence stems from a

235 Sickle-cell disease affects millions of individuals worl

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

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

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

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

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

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

242 flammation-related organ damage in models of sickle-cell disease or endotoxin-induced septic shock.

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

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

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

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

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

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

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

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

251 Sickle cell genotypes included 27 patients with hemoglob

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

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

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

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

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

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

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

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

260 hrocytes, as well as fewer reticulocytes and sickle cells, in the peripheral blood of treated SCD mic

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

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

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

264 Sickle cell nephropathy can occur in patients with homoz

265 Sickle cell nephropathy is a common complication in pati

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

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

268 QL) for pediatric patients hospitalized with sickle cell pain crises.

269 reliever, could alter the pathophysiology of sickle cell pain crises.

270 quality of life in children hospitalized for sickle cell pain crisis.

271 Sickle cell patients often require monthly transfusions

272 gical research, the sickle cell trait (HbAS) sickle cell polymorphism, ABO blood group, and other hem

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

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

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

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

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

278 , and occurred at the following frequencies: sickle cell trait (HbAS) 220 (14%), HbC heterozygosity (

279 des of genetic epidemiological research, the sickle cell trait (HbAS) sickle cell polymorphism, ABO b

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

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

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

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

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

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

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

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

288 in status (healthy control subjects, n = 10; sickle cell trait carriers, n = 10; and SCA patients, n

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

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

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

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

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

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

295 Sickle cell trait was not associated with a higher risk

296 Sickle cell trait was seen in all districts.

297 -American patients, 542 (10.2%) patients had sickle cell trait, and 129 (2.4%) patients had hemoglobi

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

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

300 valuable therapeutic intervention for acute sickle cell vasoocclusive crises.