ライフサイエンスコーパス: HAE

1 HAE is transcriptionally up-regulated in the floral absc

2 HAE should be considered in the differential diagnosis o

3 HAE-FXII and HAE-unknown differ in various respects, inc

4 dogenous MASP-1/C1-INH complex levels in 128 HAE patients and 100 controls.

5 ike protein kinases, HAESA and HAESA-like 2 (HAE/HSL2) that regulate a MAP kinase cascade that is req

6 Forty-nine percent of 521 HAE attacks only involved abdominal symptoms.

7 Analyzing 60 HAE-induced leaf transcriptomes from closely-related Nic

8 esent novel subcutaneous therapies for acute HAE exacerbations.

9 opment of ecallantide for treatment of acute HAE attacks included affected anatomic location, accompa

10 allantide was efficacious for treating acute HAE attacks.

11 intestinal angioedema in pediatric and adult HAE patients.

12 short-term and long-term prophylaxis against HAE exacerbations.

13 s to consider short-term prophylaxis for all HAE patients undergoing surgery.

14 The level of functional C1-INH in all HAE types I and II plasma tested was less than 40% of ou

15 t is recommended to follow up patients in an HAE comprehensive care center.

16 However, the risk of an HAE attack in patients without prophylaxis has not been

17 Both HAE-FXII and HAE-unknown are inherited as autosomal-dominant traits w

18 HAE-FXII and HAE-unknown differ in various respects, including gender

19 e to angioedema was similar for HAE-FXII and HAE-unknown.

20 arameters between patients with HAE-FXII and HAE-unknown.

21 come wilted in response to limited water and HAE continues to accumulate in the leaf abscission zones

22 edema, which includes hereditary angioedema (HAE types I, II and III), acquired C1-INH deficiency, an

23 but individuals with hereditary angioedema (HAE) are deficient in C1-inhibitor and consequently exhi

24 ically, treatment for hereditary angioedema (HAE) attacks has been administered by healthcare profess

25 , in the treatment of hereditary angioedema (HAE) attacks.

26 Hereditary angioedema (HAE) caused by a deficiency of functional C1-inhibitor (

27 Hereditary angioedema (HAE) caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-

28 agement of attacks of hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency (C

29 Hereditary angioedema (HAE) due to C1 inhibitor deficiency manifests as recurre

30 ) are associated with hereditary angioedema (HAE) in the presence of normal C1 esterase inhibitor act

31 Hereditary angioedema (HAE) is a disease characterized by recurrent tissue swel

32 Hereditary angioedema (HAE) is a heterozygous deficiency of first component of

33 Hereditary angioedema (HAE) is a rare genetic disease characterized by unpredic

34 Hereditary angioedema (HAE) is a rare genetic disease usually caused by mutatio

35 Hereditary angioedema (HAE) is characterized by unpredictable attacks of debili

36 Hereditary angioedema (HAE) types I and II were then tested.

37 for acute attacks of hereditary angioedema (HAE) with C1-inhibitor (C1-INH) deficiency.

38 Hereditary angioedema (HAE) with normal C1 inhibitor (C1Inh) associated with th

39 Hereditary angioedema (HAE) with normal C1-INH (HAEnCI) may be linked to specif

40 NH is associated with hereditary angioedema (HAE), an autosomal inherited disease characterized by sw

41 Hereditary angioedema (HAE), caused by deficiency in C1-inhibitor (C1-INH), lea

42 and understanding of hereditary angioedema (HAE), while integrating insights into pediatric subtleti

43 ment for treatment of hereditary angioedema (HAE).

44 t option for pKal-mediated diseases, such as HAE.

45 To provide evidence-based HAE treatment guidelines supported by the new studies, a

46 Because HAE cells are isolated directly from human airways, Vero

47 hout pre-event STP (in 39/89 patients before HAE was diagnosed), or after STP (in 3/55 cases after di

48 Both HAE-FXII and HAE-unknown are inherited as autosomal-domi

49 pleting a positive feedback loop controlling HAE expression.

50 caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-INH) is a potentially life-threatening rare disea

51 y angioedema due to C1 inhibitor deficiency (HAE-C1-INH) is characterized by relapsing skin swellings

52 ngioedema caused by C1 inhibitor deficiency (HAE-C1-INH).

53 H (63 cases) than in patients with diagnosed HAE-C1-INH (7 cases).

54 f airway epithelial cells from human donors (HAE), MV infectious centers form rapidly and become larg

55 d airway epithelial cells from human donors (HAE).

56 e demonstrated that ECs are activated during HAE attacks.

57 tudied whether EC function is altered during HAE attacks in comparison with attack-free intervals.

58 helin-1) were significantly increased during HAE attacks.

59 bradykinin, have not yet been studied during HAE attacks.

60 erception of herbivore associated elicitors (HAE) that includes transient accumulations of jasmonic a

61 The genes that encode HAE/HAESA-LIKE2, IDA, NEVERSHED, and MAPK KINASE4 and 5

62 fferentiated primary human airway epithelia (HAE) in vitro In human embryonic kidney HEK293 cells, th

63 well-differentiated human airway epithelia (HAE).

64 well-differentiated human airway epithelial (HAE) cell cultures 600-fold less efficiently than did HE

65 ng primary human ciliated airway epithelial (HAE) cell cultures, the only in vitro replication model

66 well-differentiated human airway epithelial (HAE) cell cultures.

67 cle of infection in human airway epithelial (HAE) cells cultured in vitro, the L-226-containing H9N2

68 SMase activated in human airway epithelial (HAE) cells following exposure to oxidative stress (ox-st

69 f icSZ16-S K479N on human airway epithelial (HAE) cells produced two viruses (icSZ16-S K479N D8 and D

70 ted more rapidly in human airway epithelial (HAE) cells than did the first-wave virus.

71 on of human cartilaginous airway epithelium (HAE) and a hamster model of disease with recombinant res

72 res, a model of the human airway epithelium (HAE) in which primary HAE cells are cultured at an air-l

73 o model of human ciliated airway epithelium (HAE), a useful tool for studying respiratory virus-host

74 udostratified mucociliary airway epithelium (HAE).

75 The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical

76 ith icatibant in adult patients experiencing HAE attacks during the FAST-2 open-label extension (OLE)

77 io was 35 : 14 for HAE-FXII and 109 : 12 for HAE-unknown.

78 paternal transmission ratio was 35 : 14 for HAE-FXII and 109 : 12 for HAE-unknown.

79 To develop a potential therapeutic agent for HAE and other pKal-mediated disorders, we used phage dis

80 ed consensus about management approaches for HAE in adult/adolescent patients.

81 , a plasma kallikrein inhibitor approved for HAE attack treatment.

82 The evolving standard of care for HAE management involves not only treatment of acute exac

83 ntact G protein would be more infectious for HAE cell cultures.

84 in Vero cells was 5-fold more infectious for HAE cells in culture, confirming our hypothesis and indi

85 This reduced infectivity for HAE cell cultures is not likely to be due to the loss of

86 phyxiation due to angioedema was similar for HAE-FXII and HAE-unknown.

87 d safety of repeated icatibant treatment for HAE attacks.

88 Current guidelines for the treatment for HAE were released before the new trials and before the n

89 trolled studies of ecallantide treatment for HAE: EDEMA3-DB and EDEMA4.

90 osphorylation of AGL15 is necessary for full HAE expression, thus completing a positive feedback loop

91 ake of quinapril or enalapril had no further HAE-FXII attacks after discontinuation of those drugs.

92 normal C1 esterase inhibitor activity (FXII-HAE).

93 We report two Brazilian FXII-HAE families segregating the mutation c.983 C>A (p.Thr32

94 (44-76) and 49 (35-59) nmol/ml/min for FXII-HAE asymptomatic and symptomatic carriers, respectively,

95 3) nmol/ml/min and 38.5 (32.8-45.6) for FXII-HAE asymptomatic and symptomatic carriers, respectively,

96 and c.983C>G mutations of the F12 gene (FXII-HAE) is a rare condition, and presents with highly varia

97 ygous mutations in the factor XII gene (FXII-HAE).

98 dings therefore suggest that homozygous FXII-HAE mutation status leads to a severe phenotype in femal

99 it is the first such report for a male FXII-HAE mutation carrier.

100 To predict FXII-HAE disease severity, we analyzed the biological phenoty

101 We here report that FXII-HAE mutations collectively introduce new sites that are

102 The FXII-HAE is associated with modifiers, for example kinin cata

103 Meaningful variables contributed to FXII-HAE, with the kinin catabolism enzymes ACE and CPN exhib

104 lates disease activity in patients with FXII-HAE.

105 bradykinin production in patients with FXII-HAE.

106 ase inhibitor and mutations in the F12 gene (HAE-FXII) is associated with skin swellings, abdominal p

107 ns in the coagulation factor 12 (FXII) gene (HAE-FXII) or functional mutations in other genes that ar

108 ns in the coagulation factor 12 (FXII) gene (HAE-FXII) or mutations in genes that are still unknown (

109 in, the receptor-like protein kinases HAESA (HAE) and HAESA-like 2 (HSL2), the Mitogen-Activated Prot

110 ine-rich repeat receptor-like kinases HAESA (HAE) and HAESA-LIKE2 (HSL2).

111 Gene expression of HAESA (HAE) and INFLORESCENCE DEFICIENT IN ABSCISSION (IDA) is

112 A)-derived peptide and its receptors, HAESA (HAE) and HAESA-LIKE2 (HSL2).

113 eat receptor-like kinases (LRR-RLKs), HAESA (HAE) and HAESA-LIKE2 (HSL2), have been shown to activate

114 s suggest that the sequential action of IDA, HAE and HSL2, and a MAP kinase cascade regulates the pro

115 Here we show that this process requires IDA, HAE, and HSL2.

116 aken together, our results suggest that IDL6-HAE/HSL2 facilitates the ingress of Pst DC3000 by promot

117 hance its infection by manipulating the IDL6-HAE/HSL2-ADPG2 signaling pathway.

118 s conducted in 18 patients with type I or II HAE who received two of twice-weekly 1500, 3000, or 6000

119 In HAE, RSV spreads over time to form focal collections of

120 In HAE, type I interferon (IFN) is induced following infect

121 ratio was 1 : 68 in HAE-FXII and 1 : 6.3 in HAE-unknown.

122 The male to female ratio was 1 : 68 in HAE-FXII and 1 : 6.3 in HAE-unknown.

123 and also provided a replicative advantage in HAE cells.

124 ient SARS-CoV infection of ciliated cells in HAE provides a useful in vitro model of human lung origi

125 a higher proportion of nonciliated cells in HAE than a 1968 pandemic-era human virus, which infected

126 two segments replicated more efficiently in HAE cells.

127 All human viruses replicated efficiently in HAE, leading to accumulation of nascent virus released f

128 ing mutant C proteins were also evaluated in HAE.

129 d not account for increased virus fitness in HAE cells.

130 tations, were highly restricted in growth in HAE at permissive (32 degrees C) and restrictive (37 deg

131 ll, the vaccine candidates exhibit growth in HAE that parallels their level of attenuation in childre

132 ange of gene expression (27-fold increase in HAE) observed in flowers when the abscission program is

133 triggering prohibits successful infection in HAE cells suggests that antiviral strategies targeted to

134 2 and NS4 underwent an abortive infection in HAE-ALI.

135 duces progeny virions that are infectious in HAE.

136 S: Major advances have recently been made in HAE treatment.

137 ma-producing Vbeta19(+) T cell population in HAE(574-586)-primed mice that appears to be the "public

138 and CPN, different from those recognized in HAE with C1Inh deficiency.

139 1 and MASP-1/C1-INH complexes are reduced in HAE patients compared with controls.

140 P-1 and C1-INH were significantly reduced in HAE patients compared with matched controls (p < 0.0001)

141 t does not express NS3 and NS4 replicated in HAE-ALI as effectively as the wild-type virus; however,

142 y HBoV1 and AAV2 rescued AAV2 replication in HAE cells.

143 The level of replication in HAE correlated with that previously observed for African

144 During multiple cycles of replication in HAE cultures, L-226-containing H9N2 isolates grew consis

145 n of the NS2 protein in HBoV1 replication in HAE-ALI.

146 RSV grows to moderate titers in HAE, though they are significantly lower than those in a

147 l tropism and replication of H9N2 viruses in HAE cells and may have implications for the abilities of

148 ckground have abscission defects, indicating HAE and HSL2 are epistatic to IDA.

149 rious infections may have a role in inducing HAE attacks.

150 Avian influenza A viruses also infected HAE, but spread was limited compared to that of human vi

151 DNA in both transfected HEK293 and infected HAE cells.

152 SARS-CoV specifically infected HAE via the apical surface and replicated to titers of 1

153 otease IV protein of Haemophilus influenzae (HAE(574-586)).

154 to the deficiency of C1-inhibitor (C1-INH) (HAE-C1-INH).

155 ecruited from the Italian Network for C1-INH-HAE (ITACA), we selected a large multiplex family with U

156 mens contributed by 139 patients with C1-INH-HAE at the annual control visits were studied retrospect

157 mer levels were associated with acute C1-INH-HAE attacks, particularly with submucosal involvement.

158 Patients with C1-INH-HAE experiencing more than 12 angioedema attacks per yea

159 All neonates/infants with an affected C1-INH-HAE family member should be screened for C1-INH deficien

160 and Ang2 were higher in patients with C1-INH-HAE in remission than in healthy controls.

161 iatric patients should always carry a C1-INH-HAE information card and medicine for emergency use.

162 ls of VEGFs and Angs in patients with C1-INH-HAE may prompt the investigation of VEGFs and Angs as bi

163 The symptoms of C1-INH-HAE often present in childhood.

164 We studied 258 C1-INH-HAE patients from 113 European families, and we explored

165 s for the diagnosis and management of C1-INH-HAE patients was created.

166 ge acts as an independent modifier of C1-INH-HAE severity.

167 on of VEGFs and Angs as biomarkers of C1-INH-HAE severity.

168 ealthy controls and 128 patients with C1-INH-HAE were studied.

169 ioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients.

170 terase inhibitor (C1-INH) deficiency (C1-INH-HAE) includes therapy with exogenous C1INH.

171 ioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare inherited genetic disease characterized b

172 angioedema due to C1-INH deficiency (C1-INH-HAE) represent one of the oldest unsolved problems of th

173 abdominal pain is common in pediatric C1-INH-HAE, but also commonly occurs in the general pediatric p

174 of acute attacks in 74 patients with C1-INH-HAE.

175 management of pediatric patients with C1-INH-HAE.

176 ns of VEGFs and Angs in patients with C1-INH-HAE.

177 ia in the urinalysis of patients with C1-INH-HAE.

178 ng to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease.

179 uman airway epithelium air-liquid interface (HAE-ALI) cultures, HBoV1 infection initiates a DNA damag

180 thelium cultured at an air-liquid interface (HAE-ALI).

181 nt of cutaneous, abdominal, and/or laryngeal HAE attack(s) severe enough to warrant treatment.

182 angioedema with normal C1 inhibitor levels (HAE-N) is associated with a Factor XII mutation in 30% o

183 ions of plasma hsCRP, MCP-1, VCAM-1, and MDA+HAE after 6 mo of supplementation.

184 osis factor-alpha, IL-1beta, VCAM-1, and MDA+HAE and the activation of nuclear transcription factor k

185 and malondialdehyde and hydroxyalkenals (MDA+HAE) in elderly subjects.

186 ors of abscission, potentially by modulating HAE/HSL2 activity.

187 on in both well-differentiated (nondividing) HAE and dividing HEK293 cells.

188 Hereditary angio-oedema (HAE) with normal C1 inhibitor is associated with heteroz

189 nt clinical reviews have raised awareness of HAE.

190 ema, and ANGPT1 variants can be the basis of HAE.

191 Diagnosis of HAE types I and II can be ascertained by inhibition of e

192 f patients with and without the diagnosis of HAE-C1-INH and analyzed fatal laryngeal attacks.

193 me-wide duplications shaped the evolution of HAE-induced EDS in Nicotiana.

194 Three women with exacerbation of HAE-FXII during intake of quinapril or enalapril had no

195 of AGL15 results in decreased expression of HAE as well as a delayed abscission phenotype.

196 issues addressed include special features of HAE-C1-INH treatment in female patients, genetic counsel

197 rhC1INH appeared to reduce the frequency of HAE attacks and were generally safe and well tolerated.

198 Patients with a history of HAE attacks occurring >/=every 2 weeks received a once w

199 d-range neuraminidase abolished infection of HAE by human parainfluenza virus type 3, this treatment

200 Infection of HAE cells with wild-type HPIV3 and variant viruses close

201 e syncytia do not form after MV infection of HAE, the cytoplasm of an infected cell suddenly flows in

202 e primary receptor for SARS-CoV infection of HAE.

203 The management of HAE consists of avoiding the triggering factors, prophyl

204 ferent paraclinical and clinical outcomes of HAE.

205 evising the knowledge on the pathogenesis of HAE-C1-INH and for reconsidering the role of ECs as a po

206 ctin-lectin pathway in the pathomechanism of HAE-C1-INH.

207 unrecognized role in the pathophysiology of HAE.

208 infection, we confirmed that pretreatment of HAE cells with HE but not the enzymatically inactive mut

209 scription factor) as a putative regulator of HAE expression.

210 rmaceutical companies and representatives of HAE patients' associations.

211 , is not detectable at the apical surface of HAE cell cultures where RSV enters.

212 nd DCs applied to the basolateral surface of HAE grown on large-pore (3.0-mum) membranes successfully

213 or DCs applied to the basolateral surface of HAE grown on small-pore (0.4-mum) support membranes did

214 ly deliver MeV to the basolateral surface of HAE.

215 ished controlled studies in the treatment of HAE attacks suggests that efficacy of C1INH therapy is o

216 nin have been developed for the treatment of HAE types I and II and are also being evaluated in other

217 rolled randomized trials in the treatment of HAE with either plasma-derived or recombinant C1INH prod

218 Family studies revealed that this type of HAE was transmitted as an autosomal dominant trait.

219 and characterize a hitherto unknown type of HAE with normal C1-INH and without mutation in the F12 g

220 mutation in the PLG gene is a novel type of HAE.

221 The unpredictability of HAE episodes supports current international treatment re

222 Recurrence or worsening of HAE symptoms (22 of 97) was the most commonly reported A

223 heless, each case should be treated based on HAE-C1-INH symptoms during pregnancy and previous labors

224 -S K479N D8 and D22) with enhanced growth on HAE cells and on delayed brain tumor cells expressing th

225 nancies had a significantly higher impact on HAE-FXII than on HAE-unknown.

226 nificantly higher impact on HAE-FXII than on HAE-unknown.

227 As an example, we used the IDA peptide HAE/HSL2 receptor signaling system known to regulate flo

228 Following a 2-week run-in period, HAE patients received 8 weekly rhC1INH administrations a

229 man airway epithelium (HAE) in which primary HAE cells are cultured at an air-liquid interface and re

230 patients were successfully able to recognize HAE attacks and decide when to self-administer icatibant

231 observed in more patients with HAE-FXII than HAE-unknown.

232 nimal model, the cotton rat, suggesting that HAE cells provide an ideal system for assessing the inte

233 The HAE and HSL2 receptors are redundant in function during

234 on experiments indicate that AGL15 binds the HAE promoter in floral receptacles.

235 Efficacy was evaluated by comparing the HAE attack incidence during the treatment period to the

236 he establishment of ongoing infection in the HAE, and these HN functions independently modulate the p

237 ic acids, respectively, were detected on the HAE cell surface, and their distribution accurately refl

238 r African green monkeys, suggesting that the HAE model has potential as a tool for the preclinical ev

239 k (M4 module) which is co-activated with the HAE-induced JA accumulations but is elicited independent

240 electively infects ciliated cells within the HAE and that progeny virus is released from the apical s

241 vates the ADPG2 expression partially through HAE and HSL2.

242 formally that transfer from immune cells to HAE occurs in a nectin-4-dependent manner.

243 function of HBoV1 for AAV2 is not limited to HAE cells but also includes HEK293 and HeLa cells.

244 or DCs were incapable of transferring MeV to HAE when applied to the apical surface.

245 We report our experience treating HAE-FXII with discontinuation of potential trigger facto

246 ngioedema with yet unknown genetic defect [U-HAE]).

247 or an additional 20 patients with familial U-HAE, 22 patients with sporadic U-HAE, and 200 control su

248 familial U-HAE, 22 patients with sporadic U-HAE, and 200 control subjects.

249 in all patients with familial or sporadic U-HAE.

250 we selected a large multiplex family with U-HAE and performed whole-exome sequencing.

251 ed in all members of the index family with U-HAE but not in asymptomatic family members or an additio

252 cation of causative genes in patients with U-HAE is valuable for understanding the cause of the disea

253 families and unrelated index patients with U-HAE recruited from the Italian Network for C1-INH-HAE (I

254 d genetic studies in Italian patients with U-HAE to identify novel causative genes.

255 NGPT1, c.807G>T, p.A119S) in a family with U-HAE.

256 tion was higher in patients with undiagnosed HAE-C1-INH (63 cases) than in patients with diagnosed HA

257 high mortality in patients with undiagnosed HAE-C1-INH underscores the need to identify these patien

258 pan of asphyxiated patients with undiagnosed HAE-C1-INH was on average approximately 31 years shorter

259 years shorter than patients with undiagnosed HAE-C1-INH who died of other causes.

260 r mutations in genes that are still unknown (HAE-unknown).

261 r at multiple sites during an attack and why HAE attacks respond well to modest increases of circulat

262 y, we have detected interactions of CST with HAE and EVR at the plasma membrane of Arabidopsis protop

263 ohort of 728 patients from 182 families with HAE-C1-INH was evaluated for death cases by analyzing pe

264 In families with HAE-FXII, the number of female offspring with F12 mutati

265 ds functional C1-INH levels in patients with HAE (types I and II) that are clearly abnormal with less

266 wever, several observations in patients with HAE are difficult to explain from a pathogenic model cla

267 ates of perioperative edema in patients with HAE not receiving prophylaxis.

268 f records of randomly selected patients with HAE type I or II treated at the Frankfurt Comprehensive

269 of perioperative angioedema in patients with HAE type I or II without prophylaxis undergoing surgical

270 self-administered icatibant in patients with HAE type I or II.

271 INH or attenuated androgens in patients with HAE undergoing surgery.

272 outcome study of SC CSL830 in patients with HAE warrants further investigation.

273 ay for bradykinin formation in patients with HAE, in which FXII is cleaved and activated by plasmin.

274 However, in a series of patients with HAE, no causative variants have been described, and the

275 odel for angioedema attacks in patients with HAE, which assumes a systemic, fluid-phase activation of

276 s for the management of female patients with HAE-C1-INH is presented.

277 btained during attacks from 18 patients with HAE-C1-INH were compared with inter-attack samples of th

278 Thirty-five patients with HAE-C1-INH, who have experienced severe attacks on 106 o

279 gic/obstetric events in female patients with HAE-C1-INH.

280 cal symptoms was 20.3 years in patients with HAE-FXII and 29.6 years in patients with HAE-unknown.

281 laboratory parameters between patients with HAE-FXII and HAE-unknown.

282 Sixty-nine patients with HAE-FXII from 23 unrelated families and 196 patients wit

283 ntration were observed in more patients with HAE-FXII than HAE-unknown.

284 For patients with HAE-FXII, various treatment options are available which

285 The study included 72 patients with HAE-FXII.

286 was abnormally high in 8 of 17 patients with HAE-N (4 in each subcategory) and could be corrected by

287 s markedly abnormal in 1 of 23 patients with HAE-N and normal in the remaining 22 patients.

288 n degradation in the plasma of patients with HAE-N both with and without the mutation.

289 was normal in all but 1 of 23 patients with HAE-N studied.

290 abnormality in PAI-2 levels in patients with HAE-N that is not seen in patients with C1 inhibitor def

291 2 ng/mL (mean, 0.54 ng/mL) in patients with HAE-N with a Factor XII mutation (12 samples), and from

292 25 ng/mL (mean, 4.3 ng/mL) in patients with HAE-N with or without the Factor XII mutation.

293 .7 ng/mL (mean, 1.03 ng/mL) in patients with HAE-N without a Factor XII mutation (11 samples).

294 23 unrelated families and 196 patients with HAE-unknown from 65 unrelated families were studied.

295 ith HAE-FXII and 29.6 years in patients with HAE-unknown.

296 s; 20 pediatric and 117 adult) patients with HAE.

297 in acute angioedema attacks in patients with HAE.

298 ing their therapeutic value in patients with HAE.

299 cted for further evaluation in patients with HAE.

300 C1-INH has not been studied in patients with HAE.