1 HAE by virtue of its infiltrative growth pattern with a
2 HAE is transcriptionally up-regulated in the floral absc
3 HAE results from variations in the SERPING1 gene that en
4 HAE should be considered in the differential diagnosis o
5 HAE was the most efficient method yielding 81 +/- 2 mg/g
6 HAE-FXII and HAE-unknown differ in various respects, inc
7 dogenous MASP-1/C1-INH complex levels in
128 HAE patients and 100 controls.
8 ike protein kinases, HAESA and HAESA-like
2 (
HAE/HSL2) that regulate a MAP kinase cascade that is req
9 Forty-nine percent of
521 HAE attacks only involved abdominal symptoms.
10 Analyzing
60 HAE-induced leaf transcriptomes from closely-related Nic
11 sed by reduced plasma C1-inhibitor
activity,
HAE has been linked to lysine/arginine substitutions for
12 opment of ecallantide for treatment of
acute HAE attacks included affected anatomic location, accompa
13 intestinal angioedema in pediatric and
adult HAE patients.
14 s to consider short-term prophylaxis for
all HAE patients undergoing surgery.
15 The level of functional C1-INH in
all HAE types I and II plasma tested was less than 40% of ou
16 t is recommended to follow up patients in
an HAE comprehensive care center.
17 However, the risk of
an HAE attack in patients without prophylaxis has not been
18 Both HAE-FXII
and HAE-unknown are inherited as autosomal-dominant traits w
19 HAE-FXII
and HAE-unknown differ in various respects, including gender
20 e to angioedema was similar for HAE-FXII
and HAE-unknown.
21 arameters between patients with HAE-FXII
and HAE-unknown.
22 ith C1-inhibitor deficiency (C1-INH-HAE)
and HAE with normal C1-INH activity (nl-C1-INH-HAE), due to
23 come wilted in response to limited water
and HAE continues to accumulate in the leaf abscission zones
24 edema, which includes hereditary
angioedema (
HAE types I, II and III), acquired C1-INH deficiency, an
25 but individuals with hereditary
angioedema (
HAE) are deficient in C1-inhibitor and consequently exhi
26 ically, treatment for hereditary
angioedema (
HAE) attacks has been administered by healthcare profess
27 fficacy in preventing hereditary
angioedema (
HAE) attacks in the phase 3 HELP Study.
28 , in the treatment of hereditary
angioedema (
HAE) attacks.
29 or the prophylaxis of hereditary
angioedema (
HAE) attacks.
30 Hereditary
angioedema (
HAE) caused by a deficiency of functional C1-inhibitor (
31 Hereditary
angioedema (
HAE) caused by C1-inhibitor (C1-INH) deficiency (HAE-C1-
32 Hereditary
angioedema (
HAE) comprises HAE with C1-inhibitor deficiency (C1-INH-
33 agement of attacks of hereditary
angioedema (
HAE) due to C1 esterase inhibitor (C1-INH) deficiency (C
34 Hereditary
angioedema (
HAE) due to C1 inhibitor deficiency manifests as recurre
35 Patients with hereditary
angioedema (
HAE) experience episodes of soft tissue swelling as a co
36 Patients with hereditary
angioedema (
HAE) have gained additional access to highly efficacious
37 ) are associated with hereditary
angioedema (
HAE) in the presence of normal C1 esterase inhibitor act
38 Hereditary
angioedema (
HAE) is a disease characterized by recurrent tissue swel
39 Hereditary
angioedema (
HAE) is a rare genetic disease characterized by unpredic
40 Hereditary
angioedema (
HAE) is a rare genetic disease usually caused by mutatio
41 Hereditary
angioedema (
HAE) is a rare genetic disorder primarily caused by muta
42 Hereditary
angioedema (
HAE) is an autosomal dominant disease characterized by r
43 Hereditary
angioedema (
HAE) is associated with recurrent, painful, and potentia
44 Hereditary
angioedema (
HAE) is characterized by unpredictable attacks of debili
45 Hereditary
angioedema (
HAE) types I and II were then tested.
46 Hereditary
angioedema (
HAE) with normal C1 inhibitor (C1Inh) associated with th
47 Hereditary
angioedema (
HAE) with normal C1-INH (HAEnCI) may be linked to specif
48 NH is associated with hereditary
angioedema (
HAE), an autosomal inherited disease characterized by sw
49 Hereditary
angioedema (
HAE), caused by deficiency in C1-inhibitor (C1-INH), lea
50 ment for treatment of hereditary
angioedema (
HAE).
51 te tissue swelling in hereditary
angioedema (
HAE).
52 QoL) in patients with hereditary
angioedema (
HAE).
53 lasminogen (PLG-HAE), angiopoietin 1 (
ANGPT1-
HAE), kininogen 1 genes (KNG1-HAE), or angioedema of unk
54 43 FXII-HAE patients, 58 U-HAE, and 4
ANGPT1-
HAE.
55 The levels of these mediators in
ANGPT1-
HAE patients were not altered.
56 t option for pKal-mediated diseases, such
as HAE.
57 Because HAE cells are isolated directly from human airways, Vero
58 Both HAE-FXII and HAE-unknown are inherited as autosomal-domi
59 ts with type I HAE, mutated C1INH encoded
by HAE-causing SERPING1 acts upon wildtype (WT) C1INH in a
60 T), FXII-W268R, and FXII-T309R (which
causes HAE), as well as other FXII variants in HEK293 freestyle
61 Hereditary angioedema (HAE)
comprises HAE with C1-inhibitor deficiency (C1-INH-HAE) and HAE wi
62 point was the rate of investigator-
confirmed HAE attacks during the 24-week treatment period.
63 ciency and at least 2 investigator-
confirmed HAE attacks in the first 56 days of a prospective run-in
64 pleting a positive feedback loop
controlling HAE expression.
65 caused by C1-inhibitor (C1-INH)
deficiency (
HAE-C1-INH) is a potentially life-threatening rare disea
66 f airway epithelial cells from human
donors (
HAE), MV infectious centers form rapidly and become larg
67 d airway epithelial cells from human
donors (
HAE).
68 Lanadelumab efficacy was
durable-
HAE attack rate was consistently lower vs placebo, from
69 e demonstrated that ECs are activated
during HAE attacks.
70 tudied whether EC function is altered
during HAE attacks in comparison with attack-free intervals.
71 helin-1) were significantly increased
during HAE attacks.
72 bradykinin, have not yet been studied
during HAE attacks.
73 erception of herbivore associated
elicitors (
HAE) that includes transient accumulations of jasmonic a
74 The genes that
encode HAE/HAESA-LIKE2, IDA, NEVERSHED, and MAPK KINASE4 and 5
75 fferentiated primary human airway
epithelia (
HAE) in vitro In human embryonic kidney HEK293 cells, th
76 well-differentiated human airway
epithelia (
HAE).
77 ng primary human ciliated airway
epithelial (
HAE) cell cultures, the only in vitro replication model
78 well-differentiated human airway
epithelial (
HAE) cell cultures.
79 ted more rapidly in human airway
epithelial (
HAE) cells than did the first-wave virus.
80 In ciliated human airway
epithelial (
HAE) cells, NAbs induced by wt G, but not by wt F, compl
81 clearly observed in human airway
epithelial (
HAE) culture.
82 RS-CoV-2 culture in human airway
epithelial (
HAE) cultures replicated the in vivo antiviral host resp
83 based extensions in human airway
epithelial (
HAE) tissues than RSV, suggesting HMPV may promote cell-
84 on of human cartilaginous airway
epithelium (
HAE) and a hamster model of disease with recombinant res
85 s polarized primary human airway
epithelium (
HAE) cultured at an air-liquid interface (HAE-ALI).
86 ith icatibant in adult patients
experiencing HAE attacks during the FAST-2 open-label extension (OLE)
87 io was 35 : 14 for HAE-FXII and 109 : 12
for HAE-unknown.
88 paternal transmission ratio was 35 : 14
for HAE-FXII and 109 : 12 for HAE-unknown.
89 To develop a potential therapeutic agent
for HAE and other pKal-mediated disorders, we used phage dis
90 ntact G protein would be more infectious
for HAE cell cultures.
91 in Vero cells was 5-fold more infectious
for HAE cells in culture, confirming our hypothesis and indi
92 phyxiation due to angioedema was similar
for HAE-FXII and HAE-unknown.
93 novel mechanism justifying gene therapy
for HAE.
94 d safety of repeated icatibant treatment
for HAE attacks.
95 osphorylation of AGL15 is necessary for
full HAE expression, thus completing a positive feedback loop
96 ake of quinapril or enalapril had no
further HAE-FXII attacks after discontinuation of those drugs.
97 We identified 43
FXII-
HAE patients, 58 U-HAE, and 4 ANGPT1-HAE.
98 normal C1 esterase inhibitor activity (
FXII-
HAE).
99 We report two Brazilian
FXII-
HAE families segregating the mutation c.983 C>A (p.Thr32
100 essed a prevalence of 1:1.4 x 10(6) for
FXII-
HAE and 1:1.0 x 10(6) for U-HAE.
101 (44-76) and 49 (35-59) nmol/ml/min for
FXII-
HAE asymptomatic and symptomatic carriers, respectively,
102 3) nmol/ml/min and 38.5 (32.8-45.6) for
FXII-
HAE asymptomatic and symptomatic carriers, respectively,
103 and c.983C>G mutations of the F12 gene (
FXII-
HAE) is a rare condition, and presents with highly varia
104 ygous mutations in the factor XII gene (
FXII-
HAE).
105 dings therefore suggest that homozygous
FXII-
HAE mutation status leads to a severe phenotype in femal
106 In
FXII-
HAE patients, cHK levels, in the absence of PIC, were si
107 In
FXII-
HAE, only VEGF-C levels were increased.
108 it is the first such report for a male
FXII-
HAE mutation carrier.
109 To predict
FXII-
HAE disease severity, we analyzed the biological phenoty
110 We here report that
FXII-
HAE mutations collectively introduce new sites that are
111 The
FXII-
HAE is associated with modifiers, for example kinin cata
112 Meaningful variables contributed to
FXII-
HAE, with the kinin catabolism enzymes ACE and CPN exhib
113 lates disease activity in patients with
FXII-
HAE.
114 bradykinin production in patients with
FXII-
HAE.
115 H-HAE), due to mutations in factor XII (
FXII-
HAE), plasminogen (PLG-HAE), angiopoietin 1 (ANGPT1-HAE)
116 ase inhibitor and mutations in the F12
gene (
HAE-FXII) is associated with skin swellings, abdominal p
117 ns in the coagulation factor 12 (FXII)
gene (
HAE-FXII) or functional mutations in other genes that ar
118 ns in the coagulation factor 12 (FXII)
gene (
HAE-FXII) or mutations in genes that are still unknown (
119 ine-rich repeat receptor-like kinases
HAESA (
HAE) and HAESA-LIKE2 (HSL2).
120 elated receptor-like protein kinases,
HAESA (
HAE) and HAESA-LIKE2 (HSL2).
121 Gene expression of
HAESA (
HAE) and INFLORESCENCE DEFICIENT IN ABSCISSION (IDA) is
122 A)-derived peptide and its receptors,
HAESA (
HAE) and HAESA-LIKE2 (HSL2).
123 H levels (20%-30% of normal) in
heterozygous HAE type I patients remain obscure.
124 how that in a subset of patients with type
I HAE, mutated C1INH encoded by HAE-causing SERPING1 acts
125 Here we show that this process requires
IDA,
HAE, and HSL2.
126 aken together, our results suggest that
IDL6-
HAE/HSL2 facilitates the ingress of Pst DC3000 by promot
127 hance its infection by manipulating the
IDL6-
HAE/HSL2-ADPG2 signaling pathway.
128 s conducted in 18 patients with type I or
II HAE who received two of twice-weekly 1500, 3000, or 6000
129 ratio was 1 : 68 in HAE-FXII and 1 : 6.3
in HAE-unknown.
130 The male to female ratio was 1 : 68
in HAE-FXII and 1 : 6.3 in HAE-unknown.
131 and also provided a replicative advantage
in HAE cells.
132 two segments replicated more efficiently
in HAE cells.
133 d not account for increased virus fitness
in HAE cells.
134 ange of gene expression (27-fold increase
in HAE) observed in flowers when the abscission program is
135 2 and NS4 underwent an abortive infection
in HAE-ALI.
136 duces progeny virions that are infectious
in HAE.
137 mechanisms affecting C1INH plasma levels
in HAE type I patients, and may pave the way for new treatm
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 n of the NS2 protein in HBoV1 replication
in HAE-ALI.
144 ctively inhibited entry and spread of RSV
in HAE tissues, with nirsevimab displaying significantly hi
145 gly, neither RSV nor HMPV formed syncytia
in HAE tissues.
146 rious infections may have a role in
inducing HAE attacks.
147 DNA in both transfected HEK293 and
infected HAE cells.
148 to the deficiency of C1-inhibitor (C1-
INH) (
HAE-C1-INH).
149 The Italian network for C1-
INH-
HAE (ITACA) created a registry including different forms
150 ecruited from the Italian Network for C1-
INH-
HAE (ITACA), we selected a large multiplex family with U
151 mens contributed by 139 patients with C1-
INH-
HAE at the annual control visits were studied retrospect
152 mer levels were associated with acute C1-
INH-
HAE attacks, particularly with submucosal involvement.
153 Patients with C1-
INH-
HAE experiencing more than 12 angioedema attacks per yea
154 All neonates/infants with an affected C1-
INH-
HAE family member should be screened for C1-INH deficien
155 a cohort of Italian subjects with nl-C1-
INH-
HAE followed by ITACA to identify specific biomarkers.
156 and Ang2 were higher in patients with C1-
INH-
HAE in remission than in healthy controls.
157 iatric patients should always carry a C1-
INH-
HAE information card and medicine for emergency use.
158 ls of VEGFs and Angs in patients with C1-
INH-
HAE may prompt the investigation of VEGFs and Angs as bi
159 The symptoms of C1-
INH-
HAE often present in childhood.
160 We studied 258 C1-
INH-
HAE patients from 113 European families, and we explored
161 s for the diagnosis and management of C1-
INH-
HAE patients was created.
162 A total of 105 nl-C1-
INH-
HAE patients were studied.
163 on of VEGFs and Angs as biomarkers of C1-
INH-
HAE severity.
164 ge acts as an independent modifier of C1-
INH-
HAE severity.
165 ealthy controls and 128 patients with C1-
INH-
HAE were studied.
166 ses HAE with C1-inhibitor deficiency (C1-
INH-
HAE) and HAE with normal C1-INH activity (nl-C1-INH-HAE)
167 ioedema with C1 inhibitor deficiency (C1-
INH-
HAE) have focused on adult patients.
168 terase inhibitor (C1-INH) deficiency (C1-
INH-
HAE) includes therapy with exogenous C1INH.
169 ioedema with C1 inhibitor deficiency (C1-
INH-
HAE) is a rare inherited genetic disease characterized b
170 angioedema due to C1-INH deficiency (C1-
INH-
HAE) represent one of the oldest unsolved problems of th
171 d HAE with normal C1-INH activity (nl-C1-
INH-
HAE), due to mutations in factor XII (FXII-HAE), plasmin
172 abdominal pain is common in pediatric C1-
INH-
HAE, but also commonly occurs in the general pediatric p
173 management of pediatric patients with C1-
INH-
HAE.
174 ns of VEGFs and Angs in patients with C1-
INH-
HAE.
175 ia in the urinalysis of patients with C1-
INH-
HAE.
176 of acute attacks in 74 patients with C1-
INH-
HAE.
177 uman airway epithelium air-liquid
interface (
HAE-ALI) cultures, HBoV1 infection initiates a DNA damag
178 thelium cultured at an air-liquid
interface (
HAE-ALI).
179 m (HAE) cultured at an air-liquid
interface (
HAE-ALI).
180 (SERK) family of coreceptor protein
kinases,
HAE and HSL2 are activated when bound by INFLORESCENCE D
181 etin 1 (ANGPT1-HAE), kininogen 1 genes (
KNG1-
HAE), or angioedema of unknown origin (U-HAE).
182 nt of cutaneous, abdominal, and/or
laryngeal HAE attack(s) severe enough to warrant treatment.
183 angioedema with normal C1 inhibitor
levels (
HAE-N) is associated with a Factor XII mutation in 30% o
184 Least-squares mean
monthly HAE attack rate for lanadelumab was compared with placeb
185 Although
most HAE cases are caused by reduced plasma C1-inhibitor acti
186 on in both well-differentiated (
nondividing)
HAE and dividing HEK293 cells.
187 Hereditary angio-
oedema (
HAE) with normal C1 inhibitor is associated with heteroz
188 Knowledge of varied imaging appearances
of HAE is essential to suspect the condition and to make an
189 ema, and ANGPT1 variants can be the basis
of HAE.
190 gs in 23 histopathologically proven cases
of HAE with emphasis on the appearance and extent of diseas
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 Ultrasonography features
of HAE included heterogeneous, hyperechoic hepatic mass wit
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 ons that activate signaling independently
of HAE/HSL2.
200 e syncytia do not form after MV infection
of HAE, the cytoplasm of an infected cell suddenly flows in
201 ferent paraclinical and clinical outcomes
of HAE.
202 evising the knowledge on the pathogenesis
of HAE-C1-INH and for reconsidering the role of ECs as a po
203 ctin-lectin pathway in the pathomechanism
of HAE-C1-INH.
204 unrecognized role in the pathophysiology
of HAE.
205 infection, we confirmed that pretreatment
of HAE cells with HE but not the enzymatically inactive mut
206 scription factor) as a putative regulator
of HAE expression.
207 we showed that C1INH encoded by a subset
of HAE-causing SERPING1 alleles affected secretion of norma
208 nd DCs applied to the basolateral surface
of HAE grown on large-pore (3.0-mum) membranes successfully
209 or DCs applied to the basolateral surface
of HAE grown on small-pore (0.4-mum) support membranes did
210 ly deliver MeV to the basolateral surface
of HAE.
211 nin have been developed for the treatment
of HAE types I and II and are also being evaluated in other
212 , and may pave the way for new treatments
of HAE.
213 Family studies revealed that this type
of HAE was transmitted as an autosomal dominant trait.
214 and characterize a hitherto unknown type
of HAE with normal C1-INH and without mutation in the F12 g
215 mutation in the PLG gene is a novel type
of HAE.
216 The unpredictability
of HAE episodes supports current international treatment re
217 Recurrence or worsening
of HAE symptoms (22 of 97) was the most commonly reported A
218 nancies had a significantly higher impact
on HAE-FXII than on HAE-unknown.
219 nificantly higher impact on HAE-FXII than
on HAE-unknown.
220 As an example, we used the IDA
peptide HAE/HSL2 receptor signaling system known to regulate flo
221 Following a 2-week run-in
period,
HAE patients received 8 weekly rhC1INH administrations a
222 s in factor XII (FXII-HAE), plasminogen (
PLG-
HAE), angiopoietin 1 (ANGPT1-HAE), kininogen 1 genes (KN
223 patients were successfully able to
recognize HAE attacks and decide when to self-administer icatibant
224 -mg and 150-mg doses of berotralstat
reduced HAE attack rates compared with placebo and were safe and
225 observed in more patients with HAE-FXII
than HAE-unknown.
226 The HAE and HSL2 receptors are redundant in function during
227 on experiments indicate that AGL15 binds
the HAE promoter in floral receptacles.
228 Efficacy was evaluated by comparing
the HAE attack incidence during the treatment period to the
229 k (M4 module) which is co-activated with
the HAE-induced JA accumulations but is elicited independent
230 vates the ADPG2 expression partially
through HAE and HSL2.
231 formally that transfer from immune cells
to HAE occurs in a nectin-4-dependent manner.
232 function of HBoV1 for AAV2 is not limited
to HAE cells but also includes HEK293 and HeLa cells.
233 or DCs were incapable of transferring MeV
to HAE when applied to the apical surface.
234 We report our experience
treating HAE-FXII with discontinuation of potential trigger facto
235 We identified 43 FXII-HAE patients, 58
U-
HAE, and 4 ANGPT1-HAE.
236 t that pathogenesis of FXII-, ANGPT1-, and
U-
HAE moves through an unbalanced control of kallikrein ac
237 ngioedema with yet unknown genetic defect [
U-
HAE]).
238 or an additional 20 patients with familial
U-
HAE, 22 patients with sporadic U-HAE, and 200 control su
239 x 10(6) for FXII-HAE and 1:1.0 x 10(6) for
U-
HAE.
240 ease of VEGF-A, VEGF-C, and Ang1 levels in
U-
HAE patients compared to controls.
241 cHK levels in
U-
HAE patients were similar to controls in plasma collecte
242 Ang1 participate in the pathophysiology of
U-
HAE increasing the basal vascular permeability.
243 NG1-HAE), or angioedema of unknown origin (
U-
HAE).
244 familial U-HAE, 22 patients with sporadic
U-
HAE, and 200 control subjects.
245 in all patients with familial or sporadic
U-
HAE.
246 we selected a large multiplex family with
U-
HAE and performed whole-exome sequencing.
247 ed in all members of the index family with
U-
HAE but not in asymptomatic family members or an additio
248 cation of causative genes in patients with
U-
HAE is valuable for understanding the cause of the disea
249 families and unrelated index patients with
U-
HAE recruited from the Italian Network for C1-INH-HAE (I
250 d genetic studies in Italian patients with
U-
HAE to identify novel causative genes.
251 NGPT1, c.807G>T, p.A119S) in a family with
U-
HAE.
252 high mortality in patients with
undiagnosed HAE-C1-INH underscores the need to identify these patien
253 r mutations in genes that are still
unknown (
HAE-unknown).
254 rd in patients with drug allergy,
urticaria,
HAE, and anaphylaxis.
255 r at multiple sites during an attack and
why HAE attacks respond well to modest increases of circulat
256 a W268R substitution, is not associated
with HAE.
257 In families
with HAE-FXII, the number of female offspring with F12 mutati
258 Patients aged 12 years or older
with HAE due to C1 inhibitor deficiency and at least 2 invest
259 tudy was conducted in Japanese patients
with HAE (NCT02865720).
260 ds functional C1-INH levels in patients
with HAE (types I and II) that are clearly abnormal with less
261 wever, several observations in patients
with HAE are difficult to explain from a pathogenic model cla
262 ates of perioperative edema in patients
with HAE not receiving prophylaxis.
263 olerability of berotralstat in patients
with HAE over a 24-week treatment period (the phase 3 APeX-2
264 f records of randomly selected patients
with HAE type I or II treated at the Frankfurt Comprehensive
265 of perioperative angioedema in patients
with HAE type I or II without prophylaxis undergoing surgical
266 self-administered icatibant in patients
with HAE type I or II.
267 Eligible patients
with HAE type I/II received lanadelumab 150 mg every 4 weeks
268 INH or attenuated androgens in patients
with HAE undergoing surgery.
269 outcome study of SC CSL830 in patients
with HAE warrants further investigation.
270 ay for bradykinin formation in patients
with HAE, in which FXII is cleaved and activated by plasmin.
271 However, in a series of patients
with HAE, no causative variants have been described, and the
272 odel for angioedema attacks in patients
with HAE, which assumes a systemic, fluid-phase activation of
273 Patients
with HAE-1/2 experienced significant and clinically meaningfu
274 Patients
with HAE-1/2 received either lanadelumab 150 mg every 4 weeks
275 s for the management of female patients
with HAE-C1-INH is presented.
276 btained during attacks from 18 patients
with HAE-C1-INH were compared with inter-attack samples of th
277 Thirty-five patients
with HAE-C1-INH, who have experienced severe attacks on 106 o
278 gic/obstetric events in female patients
with HAE-C1-INH.
279 cal symptoms was 20.3 years in patients
with HAE-FXII and 29.6 years in patients with HAE-unknown.
280 laboratory parameters between patients
with HAE-FXII and HAE-unknown.
281 Sixty-nine patients
with HAE-FXII from 23 unrelated families and 196 patients wit
282 ntration were observed in more patients
with HAE-FXII than HAE-unknown.
283 For patients
with HAE-FXII, various treatment options are available which
284 The study included 72 patients
with HAE-FXII.
285 was abnormally high in 8 of 17 patients
with HAE-N (4 in each subcategory) and could be corrected by
286 s markedly abnormal in 1 of 23 patients
with HAE-N and normal in the remaining 22 patients.
287 n degradation in the plasma of patients
with HAE-N both with and without the mutation.
288 was normal in all but 1 of 23 patients
with HAE-N studied.
289 abnormality in PAI-2 levels in patients
with HAE-N that is not seen in patients with C1 inhibitor def
290 2 ng/mL (mean, 0.54 ng/mL) in patients
with HAE-N with a Factor XII mutation (12 samples), and from
291 25 ng/mL (mean, 4.3 ng/mL) in patients
with HAE-N with or without the Factor XII mutation.
292 .7 ng/mL (mean, 1.03 ng/mL) in patients
with HAE-N without a Factor XII mutation (11 samples).
293 23 unrelated families and 196 patients
with HAE-unknown from 65 unrelated families were studied.
294 ith HAE-FXII and 29.6 years in patients
with HAE-unknown.
295 ing their therapeutic value in patients
with HAE.
296 cted for further evaluation in patients
with HAE.
297 in this population of Japanese patients
with HAE.
298 C1-INH has not been studied in patients
with HAE.
299 ble safety profile in Japanese patients
with HAE.
300 roviders optimize therapies in patients
with HAE.