ライフサイエンスコーパス: sickle cell disease

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

2 ion and its pathophysiologic consequences in sickle cell disease.

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

4 maging of the perifoveal microvasculature in sickle cell disease.

5 Most children had either malaria or sickle cell disease.

6 comparative group of pregnant women without sickle cell disease.

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

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

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

10 ular vascular abnormalities in patients with sickle cell disease.

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

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

13 hesive targets for vasoocclusive episodes in sickle cell disease.

14 ivation, ischemia, and reperfusion injury or sickle cell disease.

15 e used to mitigate inflammatory processes in sickle cell disease.

16 in the pathogenesis of malaria, sepsis, and sickle cell disease.

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

18 ve crises are the main acute complication in sickle cell disease.

19 itical predictor of the likelihood of severe sickle cell disease.

20 isodes of vaso-occlusion are the hallmark of sickle cell disease.

21 e used in patients with beta-thalassemia and sickle cell disease.

22 eedback loop to drive the pathophysiology of sickle cell disease.

23 ly correlates swith the clinical severity of sickle cell disease.

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

25 y a central role in vaso-occlusive crisis in sickle cell disease.

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

27 stroke prevention strategy for children with sickle cell disease.

28 c induction of fetal hemoglobin in pediatric sickle cell disease.

29 d may correlate with hemolytic parameters in sickle cell disease.

30 HLA-haploidentical, donors for patients with sickle cell disease.

31 ed morbidity and mortality for patients with sickle cell disease.

32 dent predictor of mortality in patients with sickle cell disease.

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

34 or ACS) among a cohort of 942 children with sickle cell disease.

35 HK-beta-globin transgene for gene therapy of sickle cell disease.

36 ection of subclinical retinopathy related to sickle cell disease.

37 se models, X-linked adrenoleukodystrophy and sickle cell disease.

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

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

40 All 3 patients included had homozygous sickle cell disease.

41 cations among HPC transplant recipients with sickle cell disease.

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

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

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

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

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

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

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

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

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

51 transfusions are beneficial in patients with sickle-cell disease.

52 y also precipitate sickling in patients with sickle-cell disease.

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

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

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

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

57 r uncomplicated pain crises in patients with sickle cell disease; (3) do not perform baseline or surv

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

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

60 Sickle-cell disease affects millions of individuals worl

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

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

63 h is implicated in vasoocclusive episodes in sickle cell disease and activated through the cyclic ade

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

65 Sickle cell disease and beta-thalassemia are common gene

66 ates symptoms of beta-globinopathies such as sickle cell disease and beta-thalassemia, but current ga

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

68 tion can ameliorate the clinical severity of sickle cell disease and beta-thalassemia.

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

70 globin (HbF) holds therapeutic potential for sickle cell disease and beta-thalassemias.

71 s of topical sodium nitrite in patients with sickle cell disease and chronic leg ulcers.

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

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

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

75 ariant hemoglobin allele (HbS), which causes sickle cell disease and resists infection by the malaria

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

77 full donor-type hemoglobin for patients with sickle cell disease and transfusion independence for pat

78 The management of patients with sickle-cell disease and cardiac arrest presents special

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

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

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

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

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

84 The pathology of sickle cell disease arises from the occlusion of small b

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

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

87 None of the sickle cell disease baseline characteristics predicted a

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

89 ic pain is not well studied in patients with sickle cell disease but has been modeled in the transgen

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

91 (HSCT) is curative for children with severe sickle cell disease, but toxicity may be prohibitive for

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

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

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

95 site observational analysis of children with sickle cell disease, candidate single nucleotide polymor

96 nging patient populations such as those with sickle cell disease, congenital heart defects, neutropen

97 Sickle cell disease contributes substantially to mortali

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

99 n participants from the Cooperative Study of Sickle Cell Disease (CSSCD) for acute chest syndrome (AC

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

101 ember that renal failure in conjunction with sickle cell disease does carry a significant burden of m

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

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

104 dy, we show that RBC membrane alterations in sickle cell disease enhance the activation acid sphingom

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

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

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

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

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

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

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

112 Sickle cell disease impacts on multiple facets of an ado

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

114 a-3 fatty acids for patients with homozygous sickle cell disease in a randomized, placebo-controlled,

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

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

117 perioperative complications in patients with sickle-cell disease in this trial.

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

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

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

121 Sickle cell disease is a common hemolytic disorder with

122 Sickle cell disease is an inherited blood disorder chara

123 Sickle cell disease is associated with a decreased life

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

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

126 Sickle cell disease is fundamentally a kinetic disorder,

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

128 Sickle Cell Disease is the commonest monogenic haemoglob

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

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

131 e complications experienced by patients with sickle cell disease; its prevalence is likely to increas

132 Patients with sickle cell disease may develop various macular vascular

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

134 Then, we used beta-thalassemia and sickle cell disease mice as models of hemolytic diseases

135 Moreover, we showed that in sickle cell disease mice, endothelial activation and oxi

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

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

138 Children with sickle cell disease or lead toxicity were excluded.

139 clude other common causes of icterus such as sickle cell disease or malarial infection.

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

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

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

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

144 We aimed to describe the characteristics of sickle cell disease patients admitted to ICU and to iden

145 Sickle cell disease patients are at high risk of complic

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

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

148 The sickle-cell disease patients were grouped into those wit

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

150 iated with fetal hemoglobin in patients with sickle cell disease, primarily adults.

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

152 All nine deaths (7%) were sickle cell disease related.

153 ICU admission was mainly indicated for sickle cell disease-related events, especially acute che

154 herocytosis, elliptocytosis, and especially, sickle cell disease requires the development of a detail

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

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

157 Sickle cell disease results from a homozygous missense m

158 Chronic anemia in sickle cell disease results in cardiac chamber dilation

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

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

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

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

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

164 In hematologic diseases, such as sickle cell disease (SCD) and hemolytic uremic syndrome

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

166 Although it has long been recognized that sickle cell disease (SCD) and other hemoglobinopathies a

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

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

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

170 d CBT) for severe beta thalassemia (SBT) and sickle cell disease (SCD) as experienced by the Eurocord

171 nsfusion is a key treatment of patients with sickle cell disease (SCD) but remains complicated by RBC

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

173 tive issues will take higher priority in the sickle cell disease (SCD) community.

174 Sickle cell disease (SCD) complications are associated w

175 Increased platelet activation in sickle cell disease (SCD) contributes to a state of hype

176 We postulated that the hypoxic response in sickle cell disease (SCD) contributes to altered gene ex

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

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

179 Sickle cell disease (SCD) has evolved into a debilitatin

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

181 Children with sickle cell disease (SCD) have significantly increased r

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

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

184 Sickle cell disease (SCD) is a devastating genetic disor

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

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

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

188 Sickle cell disease (SCD) is a life-threatening genetic

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

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

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

192 Sickle cell disease (SCD) is associated with a complex v

193 Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early morta

194 Sickle cell disease (SCD) is caused by a mutation in bot

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

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

197 Sickle cell disease (SCD) is characterized by recurring

198 Sickle cell disease (SCD) is characterized by vaso-occlu

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

200 Treatment of sickle cell disease (SCD) is hampered by incomplete unde

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

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

203 ct Red blood cells (RBCs) from patients with sickle cell disease (SCD) lyse in deoxygenated isosmotic

204 cell activation and decrease inflammation in sickle cell disease (SCD) mice.

205 n in diminishing vaso-occlusive processes in sickle cell disease (SCD) mice.(1)

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

207 Patients with sickle cell disease (SCD) often require transfusions to

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

209 Sickle cell disease (SCD) patients are at high risk of c

210 ctivity in plasmas from control subjects and sickle cell disease (SCD) patients confirms previous rep

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

212 cell gene therapy is an approach to treating sickle cell disease (SCD) patients that may result in lo

213 e to screen for mitochondrial dysfunction in sickle cell disease (SCD) patients, a population with ab

214 Patients with sickle cell disease (SCD) present with a wide range of c

215 l study of alloimmunization in patients with sickle cell disease (SCD) receiving blood transfusions f

216 485 patients with thalassemia major (TM) or sickle cell disease (SCD) receiving HLA-identical siblin

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

218 ability modulates the clinical expression of sickle cell disease (SCD) remain elusive.

219 risis (VOC) bone infarction in children with sickle cell disease (SCD) remains challenging for clinic

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

221 tions exist, child mortality attributable to sickle cell disease (SCD) remains high in low-resource a

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

223 Sickle cell disease (SCD) results in vascular occlusions

224 Men with sickle cell disease (SCD) risk developing priapism.

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

226 yte hydration status play important roles in sickle cell disease (SCD) through unresolved mechanisms.

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

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

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

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

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

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

233 In adults with sickle cell disease (SCD), an increased tricuspid regurg

234 ls and platelets isolated from patients with sickle cell disease (SCD), an inherited hematological di

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

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

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

238 rovide a comprehensive review of wheezing in sickle cell disease (SCD), including epidemiology, patho

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

240 In adults with sickle cell disease (SCD), markers of iron burden are as

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

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

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

244 in red blood cells (RBCs) from patients with sickle cell disease (SCD), thereby increasing oxygen aff

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

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

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

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

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

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

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

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

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

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

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

256 rtant role in pulmonary hypertension (PH) in sickle cell disease (SCD).

257 duced venular inflammation with relevance to sickle cell disease (SCD).

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

259 mportant cause of morbidity and mortality in sickle cell disease (SCD).

260 eir role in the pathology of inflammation of sickle cell disease (SCD).

261 e erection and is prevalent among males with sickle cell disease (SCD).

262 cular inflammation are prominent features of sickle cell disease (SCD).

263 cell transplant (HSCT) is the only cure for sickle cell disease (SCD).

264 yndrome (ACS) is a major clinical concern in sickle cell disease (SCD).

265 rged as an important pathogenic mechanism in sickle cell disease (SCD).

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

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

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

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

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

271 status is prevalent in children with type SS sickle cell disease (SCD-SS) and is associated with hosp

272 ributor to increased infectious mortality in sickle-cell disease (SCD), this relationship has not bee

273 ions that are as diverse as RBC transfusion, sickle cell disease, sepsis, and extracorporeal circulat

274 atients with a sickle cell hemoglobinopathy (sickle-cell disease, sickle-cell hemoglobin C disease, a

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

276 , had haemoglobin SS or Sbeta(0)thalassaemia sickle-cell-disease subtypes, and were scheduled for low

277 iple beneficial effects for the treatment of sickle cell disease symptoms and provides an alternative

278 heir potential uses in the oral treatment of sickle cell disease symptoms.

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

280 ellular volume and volume regulation include sickle cell disease, thalassemia, and hereditary spheroc

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

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

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

284 In the context of sickle cell disease, the blockade of both FcgammaRIIB an

285 uman gamma-globin, a pharmacologic target in sickle cell disease therapy.

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

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

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

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

290 Sickle cell disease was less common in children older th

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

292 th baseline fetal hemoglobin levels in adult sickle cell disease were examined in children at baselin

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

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

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

296 bstructive pulmonary disease, pneumonia, and sickle cell disease, were risk factors with an opposite

297 he beta-globinopathies, beta-thalassemia and sickle cell disease, which begin to manifest symptoms ar

298 979-1998), a disparity likely due in part to sickle cell disease, which leads to a high risk of child

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

300 Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease With Sildenafil Therapy cohort (alle