ライフサイエンスコーパス: sickle cell anemia

1 t types of sickle-shaped RBCs as observed in sickle cell anemia.

2 capacity in a large cohort of patients with sickle cell anemia.

3 of HbF in patients with beta-thalassemia or sickle cell anemia.

4 fier of the severity of beta-thalassemia and sickle cell anemia.

5 and treatment of the chronic vasculopathy of sickle cell anemia.

6 stating complication affecting children with sickle cell anemia.

7 complexity of the clinical manifestations of sickle cell anemia.

8 alassemia and other genetic diseases such as sickle cell anemia.

9 andidate genes and the many subphenotypes of sickle cell anemia.

10 enous circulation underlies the debilitating sickle cell anemia.

11 f the basic events in the pathophysiology of sickle cell anemia.

12 e sickle beta-globin gene (beta(S)) leads to sickle cell anemia.

13 underlie the beta-globinopathies, including sickle cell anemia.

14 lusion in hypoxic tissues is the hallmark of sickle cell anemia.

15 circulating endothelial cells in humans with sickle cell anemia.

16 n 6 of the beta-globin gene, responsible for sickle cell anemia.

17 ormation is the primary pathogenic event for sickle cell anemia.

18 in patients with severe end-organ effects of sickle cell anemia.

19 BSC use for transplantation in patients with sickle cell anemia.

20 lotor (500-1000 mg per day) in patients with sickle cell anemia.

21 he clinical severity of beta-thalassemia and sickle cell anemia.

22 n attractive intervention point for treating sickle cell anemia.

23 xperimental models of tumor angiogenesis and sickle cell anemia.

24 potentially fatal end-organ complication of sickle cell anemia.

25 on, currently the only curative approach for sickle cell anemia.

26 One patient had alpha-thalassemia sickle cell anemia.

27 in vivo in a well-established mouse model of sickle cell anemia.

28 ortant adjunct strategy for the treatment of sickle cell anemia.

29 sis of acute chest syndrome in subjects with sickle cell anemia.

30 ximeter-measured saturation in patients with sickle cell anemia.

31 arterial oxygen saturation in patients with sickle cell anemia.

32 usion for stroke prevention in patients with sickle cell anemia.

33 s is of therapeutic benefit in patients with sickle cell anemia.

34 in the pathophysiology of vaso-occlusion in sickle cell anemia.

35 ogies with reported vascular damage, such as sickle cell anemia.

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

37 y play an important role in vasoocclusion in sickle cell anemia.

38 with hydroxyurea (HU) therapy in adults with sickle cell anemia.

39 e therapy for the treatment of patients with sickle cell anemia.

40 eption of those with unstable hemoglobins or sickle cell anemia.

41 rculating endothelial cells in patients with sickle cell anemia.

42 generation of the transgenic mouse model for sickle cell anemia.

43 ractions among children and adolescents with sickle cell anemia.

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

45 atopoietic progenitors from individuals with sickle cell anemia.

46 vestigations into cerebrovascular disease in sickle cell anemia.

47 cus pneumoniae pathogenesis in patients with sickle cell anemia.

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

49 the major modifier of the clinical course of sickle cell anemia.

50 these models to predict HbF in patients with sickle cell anemia.

51 urrence of cerebral infarct in children with sickle cell anemia.

52 in (HbF) within erythrocytes of persons with sickle cell anemia.

53 by Microbacterium binotii in a patient with sickle cell anemia.

54 lar transfusions to prevent complications of sickle cell anemia.

55 Vasoocclusion crisis is a key hallmark of sickle cell anemia.

56 effective, safe, and affordable therapy for sickle cell anemia.

57 fants (beginning at 9-18 months of age) with sickle cell anemia.

58 oxyurea therapy for very young children with sickle cell anemia.

59 reventing recurrent strokes in children with sickle cell anemia.

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

61 aluated spleen function in 193 children with sickle cell anemia 8 to 18 months of age by (99m)Tc sulf

62 Most children with sickle cell anemia (93.9%) and nearly all children with

63 l cells (CEC) from normals and patients with sickle cell anemia, a disease associated with activation

64 inishes the severity of beta-thalassemia and sickle cell anemia, a strategy using autologous, stem ce

65 owth endothelial cells from 20 subjects with sickle cell anemia (age, 4-19 years) shown to be either

66 Children with sickle cell anemia, age 5 to 15 years, were eligible for

67 oxyurea represents an approved treatment for sickle cell anemia and a number of cancers.

68 studies including fetal hemoglobin levels in sickle cell anemia and a sample of centenarians and show

69 oxyurea represents an approved treatment for sickle cell anemia and acts as a nitric oxide donor unde

70 es have been used to create mouse models for sickle cell anemia and all of the clinically relevant th

71 st common neurologic injury in children with sickle cell anemia and are associated with the recurrenc

72 ession approaching the therapeutic range for sickle cell anemia and beta thalassemia.

73 pressing fetal hemoglobin synthesis to treat sickle cell anemia and beta thalassemia.

74 Human [beta] globin disorders such as sickle cell anemia and beta-thalassemia are very common

75 rate beta-type globin gene disorders such as sickle cell anemia and beta-thalassemia through activati

76 Human beta-globin disorders, such as sickle cell anemia and beta-thalassemia, are relatively

77 reatment of the common hemoglobin disorders, sickle cell anemia and beta-thalassemia.

78 sted in a replication set of 305 blacks with sickle cell anemia and in subjects with hemoglobin E or

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

80 e validated in a cohort of 283 patients with sickle cell anemia and known pediatric cerebrovascular o

81 quire multiple blood transfusions, including sickle cell anemia and myelodysplasia.

82 To enter the study, children with sickle cell anemia and no history of stroke had to have

83 erythroid progenitors from individuals with sickle cell anemia and normal hemoglobin genotypes.

84 roid progenitors isolated from patients with sickle cell anemia and patients with beta-thalassemia.

85 uld be present in about 10% of children with sickle cell anemia and represent a genetic risk factor t

86 the understanding of the pathophysiology of sickle cell anemia and should be tested in further work.

87 Red blood cells (RBCs) from patients with sickle cell anemia and thalassemia carry abnormal accumu

88 Sickle cell anemia and thalassemia constitute the most c

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

90 tal gamma-globin expression in patients with sickle cell anemia and thalassemia.

91 link between the single molecular defect in sickle cell anemia and the extensive pathology of this d

92 Sickle cell anemia and the thalassemias are globally the

93 The models were trained in 841 patients with sickle cell anemia and were tested in 3 independent coho

94 y of human hemoglobinopathies in general and sickle-cell anemia and beta-thalassemia in particular.

95 HU is used to treat HIV, sickle-cell anemia and some cancers.

96 Plasma arginine levels are low in sickle cell anemia, and it is reported here that low pla

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

98 rt disease, infection, head and neck trauma, sickle cell anemia, and prothrombotic abnormalities.

99 utes to the vasoocclusive pathophysiology of sickle cell anemia, and that the phenotypic variation in

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

101 The beta-thalassemias and sickle cell anemia are severe congenital anemias for whi

102 revent chronic organ damage in children with sickle cell anemia are warranted.

103 ith Pauling's seminal work, which recognized sickle-cell anemia as a molecular disease, and with Ingr

104 ould prevent initial stroke in children with sickle-cell anemia at high risk as determined by transcr

105 ainful crisis episodes are poorly treated in sickle cell anemia, both in timeliness and appropriatene

106 Hydroxyurea is an effective treatment for sickle cell anemia, but few studies have been conducted

107 ute to exercise intolerance in patients with sickle cell anemia, but little information exists regard

108 ns prevent recurrent stroke in children with sickle cell anemia, but the value of transfusions in pre

109 hemoglobin (HbF) modulates the phenotype of sickle cell anemia by inhibiting deoxy sickle hemoglobin

110 We hypothesize that individuals with sickle cell anemia demonstrate increased intertrabecular

111 unaffected pregnancy resulting from PGD for sickle cell anemia demonstrates that the technique can b

112 onditional mutualism is analogous to chronic sickle cell anemia enhancing the resistance to malaria a

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

114 pared ES cells from blastocysts that had the sickle cells anemia genotype and carried out homologous

115 nt assumption central to most treatments for sickle cell anemia has been that replacement of sickle h

116 cacy of hydroxyurea (HU) in the treatment of sickle cell anemia has mainly been attributed to increas

117 acy of this agent in pediatric patients with sickle cell anemia has not been determined.

118 Several transgenic murine models for sickle cell anemia have been developed that closely repr

119 SNPs) in 49 candidate genes in patients with sickle cell anemia (Hb SS) who had been screened for pHT

120 Forty-three patients with homozygous sickle cell anemia (HbSS) and 1 with HbSO(Arab) (16 fema

121 Children with sickle cell anemia (HbSS) are at high risk for neurologi

122 f PAH in plasma specimens from 27 homozygous sickle cell anemia (HbSS) patients with PAH and 28 witho

123 ients with SCD including 106 with homozygous sickle cell anemia (HbSS), 7 with sickle hemoglobin C (H

124 (HU) can increase fetal hemoglobin (HbF) in sickle cell anemia (HbSS).

125 ar disease is a common cause of morbidity in sickle cell anemia (HbSS): approximately 10% of patients

126 Most participants had sickle cell anemia (homozygous hemoglobin S or hemoglobi

127 enrolled children 1 to 10 years of age with sickle cell anemia in four sub-Saharan countries.

128 Among children with sickle cell anemia in sub-Saharan Africa, hydroxyurea wi

129 , feasibility, and efficacy in children with sickle cell anemia in sub-Saharan Africa, with studies s

130 essentially everyone, such as patients with sickle cell anemia, in need of the procedure.

131 s have been identified in vascular injury in sickle cell anemia, in vascular occlusion following the

132 athies, including thalassemia intermedia and sickle cell anemia, in which a different spectrum of car

133 The vascular pathobiology of sickle cell anemia involves inflammation, coagulation, v

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

135 Sickle cell anemia is a common autosomal recessive disor

136 Sickle cell anemia is a common genetic disorder in Afric

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

138 Sickle cell anemia is a debilitating genetic disease tha

139 ed by identical mutations in a single gene - sickle cell anemia is a prime example - can have clinica

140 These data suggest the hypothesis that sickle cell anemia is a state of enhanced anti-apoptotic

141 Sickle cell anemia is a unique disease dominated by hemo

142 Sickle cell anemia is an inherited blood disorder that i

143 Sickle cell anemia is associated with the mutant hemoglo

144 Sickle cell anemia is characterized by chronic hemolysis

145 Sickle cell anemia is characterized by painful vaso-occl

146 Sickle cell anemia is characterized by periodic vasooccl

147 Renal involvement in sickle cell anemia is common, and in some cases, can pre

148 Nevertheless, at the phenotypic level, sickle cell anemia is not a monogenic disease; it is a m

149 Sickle cell anemia is one of the most common genetic dis

150 Sickle cell anemia is the first monogenic disease ever d

151 Sickle cell anemia is the most common heritable hematolo

152 The primary pathogenic event of sickle cell anemia is the polymerization of the mutant h

153 sduction of CD34(+) cells from patients with sickle cell anemia led to erythroid-specific expression

154 tment was feasible and safe in children with sickle cell anemia living in sub-Saharan Africa.

155 Sickle cell anemia may expand marrow spaces in the jaws.

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

157 By using a humanized sickle cell anemia mouse model, we show that mice can be

158 Using a transgenic/knockout sickle cell anemia mouse model, which harbors 240 kb of

159 the 1996 Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) Patients' Follow-up Study.

160 lls and enabled A*T-to-G*C base editing of a sickle cell anemia mutation using a previously inaccessi

161 Forty-three patients (sickle cell anemia, n = 32; beta-thalassemia major, n =

162 c activity in two transgenic mouse models of sickle cell anemia (NY1DD and hBERK1) and not in their r

163 s a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, tr

164 h stiffening of red blood cells (RBCs; e.g., sickle cell anemia or malaria), the mechanical propertie

165 e asymptomatic patients aged 2-16 years with sickle cell anemia or sickle cell-beta thalassemia were

166 nts who received the diagnosis of homozygous sickle cell anemia or sickle cell-Beta(0)-thalassemia be

167 positive HIV status, history of hemophilia, sickle cell anemia or thalassemia, history of blood tran

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

169 which occurs in diseases such as malaria and sickle cell anemia, or following blood transfusions.

170 ochimerism to treat such diseases as cancer, sickle cell anemia, or thalassemia.

171 ther study in other forms of thalassemia and sickle-cell anemia, particularly when splenic function i

172 Sickle cell anemia patients have significant LV dilatati

173 ee heme concentration in the erythrocytes of sickle cell anemia patients may be a significant factor

174 n ability of hydroxyurea to ease the pain of sickle cell anemia patients may be the result of vasodil

175 ifferent fetal hemoglobin levels among adult sickle cell anemia patients suggest genetic modulation o

176 globin level and markers of RBC hemolysis in sickle cell anemia patients.

177 mechanism by which hypoxia may influence the sickle cell anemia phenotype.

178 In certain pathologic states, such as sickle cell anemia, phospholipid asymmetry is altered.

179 f circle of Willis disease in the child with sickle cell anemia predominantly involves inflammation b

180 In persons with sickle cell anemia, preoperative transfusion therapy to

181 al administration of HU for the treatment of sickle cell anemia produced detectable nitrosyl hemoglob

182 We describe a population of sickle cell anemia red cells (SS RBCs) ( approximately 4

183 In patients with sickle cell anemia, regardless of clinical status, the c

184 ar endothelium is activated in patients with sickle cell anemia, regardless of the patients' clinical

185 perience with dual organ transplantation for sickle cell anemia-related complications.

186 cation of the biophysical characteristics of sickle cell anemia remains an open issue.

187 pted importance of circulatory impairment to sickle cell anemia remains to be verified by in vivo exp

188 nuates leukocyte-endothelial interactions in sickle cell anemia, resulting in protection against leth

189 Trabecular structures in individuals with sickle cell anemia revealed increased intertrabecular di

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

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

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

193 fer major clinical benefits in patients with sickle cell anemia (SCA) and beta thalassemia, diseases

194 as many characteristics of an ideal drug for sickle cell anemia (SCA) and provides therapeutic benefi

195 Sickle cell anemia (SCA) and thalassemia are among the m

196 Children with sickle cell anemia (SCA) carry a 200-fold increased risk

197 ctic blood transfusion with standard care in sickle cell anemia (SCA) children aged 2 to 16 years sel

198 ariate analysis showed that in patients with sickle cell anemia (SCA) genotypes, older age (95% confi

199 ment of prolonged priapism for patients with sickle cell anemia (SCA) has not been established.

200 fetal hemoglobin (HbF) expression to correct sickle cell anemia (SCA) in the Berkeley "humanized" sic

201 Sickle cell anemia (SCA) is a chronic illness causing pr

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

203 Sickle cell anemia (SCA) is a paradigmatic single gene d

204 Sickle cell anemia (SCA) is a well characterized severe

205 Sickle cell anemia (SCA) is an inherited disorder associ

206 Sickle cell anemia (SCA) is an inherited disorder of bet

207 The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (

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

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

210 atients receiving long-term transfusions for sickle cell anemia (SCA) matched for age, sex, and liver

211 Patients with sickle cell anemia (SCA) may develop a glomerulopathy wi

212 utations, 14 beta-thalassemia mutations, two sickle cell anemia (SCA) mutations, three Tay-Sachs muta

213 tial fiber shortening (VCFc) relationship in sickle cell anemia (SCA) patients compared with a simila

214 most common surgical procedure performed in sickle cell anemia (SCA) patients.

215 requirements are higher in adolescents with sickle cell anemia (SCA) than in healthy control subject

216 nd effect on spleen function in infants with sickle cell anemia (SCA) was reported (HUSOFT trial).

217 nd effect on spleen function in infants with sickle cell anemia (SCA) were reported (Hydroxyurea Safe

218 Stroke is a devastating complication of sickle cell anemia (SCA) with high recurrence if untreat

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

220 Stroke is a devastating complication of sickle cell anemia (SCA), affecting 5% to 10% of patient

221 Stroke is a devastating complication of sickle cell anemia (SCA), affecting up to 30% of childre

222 lobinopathies, such as beta-thalassemias and sickle cell anemia (SCA), are among the most common inhe

223 rea has hematologic and clinical efficacy in sickle cell anemia (SCA), but its effects on transcrania

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

225 In children with sickle cell anemia (SCA), high transcranial Doppler (TCD

226 Stroke is a devastating complication of sickle cell anemia (SCA), occurring in 11% of patients b

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

228 shown to be efficacious for the treatment of sickle cell anemia (SCA), primarily through the inductio

229 ischemic brain injury are known to occur in sickle cell anemia (SCA), resulting in overt stroke and

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

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

232 ory vaso-occlusive processes associated with sickle cell anemia (SCA).

233 is associated with diastolic dysfunction in sickle cell anemia (SCA).

234 form of neurologic disease in children with sickle cell anemia (SCA).

235 ry and clinical efficacies for children with sickle cell anemia (SCA).

236 ive impairment are frequent complications of sickle cell anemia (SCA).

237 n causes of hospitalization in children with sickle cell anemia (SCA).

238 aso-occlusive complications in patients with sickle cell anemia (SCA).

239 T) prevents stroke in selected patients with sickle cell anemia (SCA).

240 n occurs in one quarter of all children with sickle cell anemia (SCA).

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

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

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

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

245 Although sickle-cell anemia (SCA) is common in black Americans, S

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

247 iven disease processes in conditions such as sickle cell anemia, sepsis, transfusion reactions, medic

248 exercise capacity and long-term outcomes in sickle cell anemia should be considered.

249 splants have been performed in patients with sickle cell anemia since the late 1960s and a number of

250 tes to endothelium, children with homozygous sickle cell anemia (SS disease) were studied, using this

251 The higher expressors of S-Oman have a sickle cell anemia (SS) clinical syndrome of moderate in

252 Children with sickle cell anemia (SS) have an increased risk for cereb

253 Sickle cell anemia (SS) is highly phenotypically variabl

254 cidence (0.61 per 100 patient-years) were in sickle cell anemia (SS) patients, but CVA occurred in al

255 at high concentration in the circulation of sickle cell anemia (SS) patients.

256 tal hemoglobin (HbF) levels in patients with sickle cell anemia (SS) who did not respond to hydroxyur

257 defined an inception cohort of newborns with sickle cell anemia (SS), sickle-beta degrees -thalassemi

258 In sickle cell anemia (SS), some red blood cells dehydrate,

259 e severity of the clinical manifestations of sickle cell anemia (SSA), including renal involvement.

260 ublication of the Stroke Prevention Trial in Sickle Cell Anemia (STOP trial) in 1998 demonstrating th

261 Although the Stroke Prevention Trial in Sickle Cell Anemia (STOP) demonstrated the efficacy of b

262 The Stroke Prevention Trial in Sickle Cell Anemia (STOP) was a randomized multicenter c

263 The Stroke Prevention Trial in Sickle Cell Anemia (STOP) was a randomized trial to eval

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

265 globin silencer sequence and the severity of sickle cell anemia, suggesting a possible role for BP1 i

266 for sibling recipients included malignancy, sickle cell anemia, thalassemia major, nonmalignant hema

267 ial to correct genetic hemological diseases (sickle cell anemia, thalassemia) and eliminate chronic i

268 to various clinical diseases of man such as sickle cell anemia, thalassemia, and autoimmune disorder

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

270 ctive method of identifying individuals with sickle cell anemia than strut analysis.

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

272 In sickle cell anemia, the initiation, progression, and res

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

274 al trial, we randomly assigned children with sickle cell anemia to receive regular blood transfusions

275 ncept that toxic erythrocyte MPs may connect sickle cell anemia to vascular disease.

276 aluation of blood samples from patients with sickle cell anemia under oxygenated conditions demonstra

277 Seventeen adult women with sickle cell anemia underwent symptom-limited maximal CPE

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

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

280 Although sickle cell anemia was the first hereditary disease to b

281 In RBCs from patients with sickle cell anemia, we demonstrate in the present study

282 lobin (HbS), the primary pathogenic event of sickle cell anemia, we explore the role of free heme, wh

283 tion that could be used in a mouse model for sickle cell anemia, we have expressed recombinant double

284 of deoxygenated red cells from patients with sickle cell anemia, we have measured the formation rate

285 me-wide association study of 848 blacks with sickle cell anemia, we identified single nucleotide poly

286 % (P <.001 v normal) of CEC from donors with sickle cell anemia were apoptotic.

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

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

289 ws that HU therapy is safe for children with sickle cell anemia when treatment was directed by a pedi

290 sive crisis is the major clinical feature of sickle cell anemia, which is believed to be initiated or

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

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

293 The ability to identify infants with sickle cell anemia who are likely to have severe complic

294 effective in increasing HbF in patients with sickle cell anemia who failed to increase HbF with HU.

295 the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcra

296 Patients with sickle cell anemia who presented with acute painful epis

297 We describe the case of a patient with sickle cell anemia who underwent successful combined liv

298 er ultrasonography to identify children with sickle cell anemia who were at high risk for stroke and

299 We studied 168 children (55% male, 97% sickle cell anemia) with a mean follow-up of 7.1 years w

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