ライフサイエンスコーパス: sickle cell anemia

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

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

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

4 enous circulation underlies the debilitating sickle cell anemia.

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

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

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

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

9 circulating endothelial cells in humans with sickle cell anemia.

10 atopoietic progenitors from individuals with sickle cell anemia.

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

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

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

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

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

16 n attractive intervention point for treating sickle cell anemia.

17 xperimental models of tumor angiogenesis and sickle cell anemia.

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

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

20 One patient had alpha-thalassemia sickle cell anemia.

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

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

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

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

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

26 arterial oxygen saturation in patients with sickle cell anemia.

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

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

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

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

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

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

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

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

35 rculating endothelial cells in patients with sickle cell anemia.

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

37 mechanisms of fetal hemoglobin modulation in sickle cell anemia.

38 ysiology of the vaso-occlusive phenomenon in sickle cell anemia.

39 ay be related in a model of vasoocclusion in sickle cell anemia.

40 cus pneumoniae pathogenesis in patients with sickle cell anemia.

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

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

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

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

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

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

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

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

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

50 ractions among children and adolescents with sickle cell anemia.

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

52 reventing recurrent strokes in children with sickle cell anemia.

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

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

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

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

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

58 complexity of the clinical manifestations of sickle cell anemia.

59 g was observed in samples from patients with sickle cell anemia (2.18% +/- 1.21%, n = 13).

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

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

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

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

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

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

86 Sickle cell anemia and thalassemia constitute the most c

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

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

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

90 Sickle cell anemia and the thalassemias are globally the

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

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

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

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

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

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

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

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

99 zed to contribute to vasoocclusive crises in sickle cell anemia are increased sickle red blood cell-e

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

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

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

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

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

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

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

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

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

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

110 In sickle cell anemia, different fetal hemoglobin levels ar

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

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

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

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

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

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

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

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

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

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

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

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

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

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

125 In red cells from patients with sickle cell anemia, hemoglobin S denatures and forms Hei

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

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

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

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

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

131 Sickle cell anemia is a common autosomal recessive disor

132 Sickle cell anemia is a common genetic disorder in Afric

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

134 Sickle cell anemia is a debilitating genetic disease tha

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

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

137 Sickle cell anemia is an inherited blood disorder that i

138 Sickle cell anemia is associated with the mutant hemoglo

139 Sickle cell anemia is characterized by chronic hemolysis

140 Sickle cell anemia is characterized by painful vaso-occl

141 Sickle cell anemia is characterized by periodic vasooccl

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

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

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

145 Sickle cell anemia is the first monogenic disease ever d

146 Sickle cell anemia is the most common heritable hematolo

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

163 Sickle cell anemia patients have significant LV dilatati

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

197 Much of the morbidity associated with sickle cell anemia (SCA) is due to ongoing infarction re

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

239 Sickle cell anemia (SS) is highly phenotypically variabl

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

266 From 295 individuals with sickle cell anemia, we chose for detailed studies 53 pat

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

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

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

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

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

272 % (P <.001 v normal) of CEC from donors with sickle cell anemia were apoptotic.

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

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

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

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

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

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

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

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

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

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

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

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

285 We treated five subjects who have sickle cell anemia with oral clotrimazole, a specific Ga

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

287 ruct in the same vector, in gene therapy for sickle cell anemia, with the objective of modifying a la

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