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1                                              HUS is responsible for most deaths associated with E. co
2                                              HUS is similar to TTP, but is associated with acute rena
3                                              HUS is usually categorized as typical, caused by Shiga t
4                                              HUS was the primary outcome.
5 oses (n = 11) exceeded the MTCD because of 2 HUS/TMA/HUS-like events.
6               Compared with waitlisted adult HUS patients on dialysis, 5-year mortality risks were 73
7             We retrospectively studied adult-HUS end-stage renal disease patients (n = 559) placed on
8 n the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking.
9  overall outcome of pediatric patients after HUS due to E. coli O104:H4 was equivalent to previous re
10 at provide cost-effective protection against HUS opens up new therapeutic approaches to managing dise
11              SpHUS accounts for 5-15% of all HUS cases.
12 ucture revealing that the N-terminal DCB and HUS regulatory domains of the Arf-GEF Sec7 form a single
13                          Bloody diarrhea and HUS were recorded as the most severe outcome for 44 and
14 ains, including strains isolated from HC and HUS cases.
15 57:H7 resulted in higher hospitalization and HUS rates than previous STEC outbreaks.
16 evelopment of thrombotic microangiopathy and HUS induced by EHEC Shiga toxins in these preclinical mo
17 idence of pediatric HUS, which is defined as HUS in children <18 years.
18 n dysregulation preceded HUS and worsened as HUS developed.
19                          Diarrhea-associated HUS accounts for more than 90% of cases and is usually c
20 et and fibrin thrombi in diarrhea-associated HUS.
21 ly no specific therapies for STEC-associated HUS, and the mechanism of Stx-induced renal injury is no
22                                     Atypical HUS (aHUS) can result from genetic or autoimmune factors
23                                     Atypical HUS (aHUS) is a disorder most commonly caused by inherit
24                                     Atypical HUS is frequently a diagnosis of exclusion.
25                                     Atypical HUS recurred after 43 (34.1%) of the transplants; in fou
26 pients among patients with ESKD and atypical HUS sharply increased between 2012 and 2016, from 46.2%
27 cherichia coli (STEC) infection, as atypical HUS (aHUS), usually caused by uncontrolled complement ac
28                       To assess how atypical HUS epidemiology in France in the eculizumab era evolved
29 o be at a high and moderate risk of atypical HUS recurrence, respectively.
30 or protein (MCP;CD46) predispose to atypical HUS (aHUS), which is not associated with exposure to Shi
31 ent and inflammation, predispose to atypical HUS, we assessed whether impaired TM function may advers
32 ulizumab to prevent post-transplant atypical HUS recurrence throughout the country.
33 ess complement activation underlies atypical HUS and is evident in Shiga toxin-induced HUS (STEC-HUS)
34 at included all adult patients with atypical HUS (n=397) between 2007 and 2016.
35 , enrolling all adult patients with atypical HUS who had undergone complement analysis and a kidney t
36 er-increasing number of associations between HUS and a variety of drugs.
37 clude some of the newer associations between HUS and a variety of infections, including, but not limi
38  aware that STEC, other than O157, can cause HUS, and 34% correctly interpreted a positive Shiga toxi
39 equently produced by STEC strains that cause HUS than is Stx1a.
40  Shiga toxin 2 are much more likely to cause HUS than are those that produce Shiga toxin 1 alone.
41 t contribute to an enhanced ability to cause HUS.
42 uman primates (Papio) recapitulated clinical HUS after Stx challenge and that novel therapeutic inter
43 tric series of Shiga toxin-producing E. coli HUS.
44 ame disease process, whereas others consider HUS and TTP to be distinct clinical and pathologic entit
45 at express Stx2 are more likely to cause D(+)HUS than are E coli expressing only Stx1.
46 ea-associated hemolytic uremic syndrome (D(+)HUS) is caused by the ingestion of Escherichia coli that
47 ible exception of Shiga toxin-mediated HUS(D+HUS), long-term outcome information is often limited by
48                            Renal damage in D+HUS is caused by Shiga toxin (Stx), which is elaborated
49 rhea-associated hemolytic uremic syndrome (D+HUS) is the most common cause of acute renal failure amo
50 ic purpura, a disease with similarities to D+HUS, in Adamts13(-/-) mice.
51                         We find that the DCB/HUS domain amplifies the ability of Sec7 to activate Arf
52 he 259 children analyzed, 36 (14%) developed HUS.
53  of the remaining 886, 126 (14.2%) developed HUS.
54 men, 22 (92%) were adults, 7 (29%) developed HUS, 5 (21%) developed bloody diarrhea, and 12 (50%) dev
55 the diarrhea phase more frequently developed HUS than those who did not (36% vs 12%; P = .001).
56         A case was an attendee who developed HUS or diarrhea between 8 and 24 June.
57  who received antibiotics, (3) who developed HUS, and (4) for whom data reported timing of antibiotic
58  diarrhea (odds ratio = 1.81) and developing HUS (odds ratio = 1.83) than did men.
59 ther established risk factors for developing HUS, such as Shiga toxin 2 and EHEC serotypes traditiona
60 nd/or individuals at high risk of developing HUS due to exposure to STEC.
61 lly protected against the risk of developing HUS.
62 ociated with an increased risk of developing HUS; however, after excluding studies at high risk of bi
63 nding of the pathogenetic mechanisms driving HUS has increased.
64 ion is profoundly affected before and during HUS, reflecting that subclinical endothelial dysfunction
65 ophils and monocytes as the key event during HUS development.
66 macrophages were evaluated in an established HUS mouse model.
67 for microvascular thrombosis in experimental HUS.
68 erve as a marker of a greater propensity for HUS, similar to the correlation between the absence of s
69 n the absence of stx(1) and a propensity for HUS.
70 s (Stx1, Stx2) are primarily responsible for HUS and the kidney and neurologic damage that ensue.
71 ation during STEC infections on the risk for HUS.
72         Currently, specific therapeutics for HUS are lacking, and therapy for patients is primarily s
73 argets and development of new treatments for HUS is described.
74 t produce only Stx1 are rarely isolated from HUS cases.
75 Of 927 STEC-infected children, 41 (4.4%) had HUS at presentation; of the remaining 886, 126 (14.2%) d
76                However, SNPs from the IL12A, HUS, CYP2C8 genes were associated with time to anemia, a
77 of p38 MAPK may be of therapeutic benefit in HUS.
78                               Differences in HUS frequency among E. coli O157:H7 outbreaks have been
79 /Ang-1 ratio were significantly different in HUS vs the pre-HUS phase of illness or uncomplicated inf
80 ed RBC-derived microvesicles are elevated in HUS patients and induced in vitro by incubation of RBCs
81 ar transplantation probability was higher in HUS than in DM and HTN patients.
82  neutrophil/platelet aggregates) involved in HUS pathogenesis.
83  associated with podocyte damage and loss in HUS mice generated by the coinjection of Stx2 and LPS.
84        Activated neutrophils are observed in HUS patients, yet it is unclear whether Stx exerts a dir
85    Shiga toxins (Stx) play a pivotal role in HUS by triggering endothelial damage in kidney and brain
86 al HUS and is evident in Shiga toxin-induced HUS (STEC-HUS).
87           E. coli O104:H4 caused the largest HUS outbreak in children reported in detail to date and
88                          Complement mediated HUS (aHUS) has a worse prognosis compared with shiga tox
89 prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease.
90 e possible exception of Shiga toxin-mediated HUS(D+HUS), long-term outcome information is often limit
91 ed to E. coli 0157:H7 (Shiga toxin-mediated) HUS, as well as the ever-increasing number of associatio
92                            Diarrhea-negative HUS is associated with complement dysregulation in up to
93 ic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous childhood onset; n = 216).
94  99% of the nonglomerular and glomerular-non-HUS groups were 42.5 years (95% confidence interval (CI)
95 uration (median, 13-14 days) compared to non-HUS patients (median, 33-34 days).
96        STEC O111 accounted for most cases of HUS and was also the cause of 3 of 7 non-O157 STEC outbr
97 oli O157:H7 remains the predominant cause of HUS in our institution.
98 o [OR] with 95% confidence interval [CI]) of HUS included younger age (0.77 [.69-.85] per year), leuk
99 ases platelet aggregation, in the context of HUS.
100                In addition, a time course of HUS disease progression that will be useful for identifi
101 venous fluid administration up to the day of HUS diagnosis was associated with a decreased risk of re
102 those employing an appropriate definition of HUS yielded an OR of 2.24 (95% CI, 1.45-3.46; I(2) = 0%)
103 t did not employ an acceptable definition of HUS, there was a significant association.
104 tify features associated with development of HUS (primary outcome) and need for renal replacement the
105 antibiotic administration and development of HUS was 1.33 (95% confidence interval [CI], .89-1.99; I(
106                               Development of HUS, complications (ie, oligoanuric renal failure, invol
107 independently associated with development of HUS.
108 tory response involved in the development of HUS.
109 tric patients with the clinical diagnosis of HUS were registered in Austria and Germany, and a subset
110 uid administration prior to establishment of HUS and (2) a higher hematocrit value at presentation.
111            Renal failure is a key feature of HUS and a major cause of childhood renal failure worldwi
112 or presumed STEC infection, and some form of HUS that developed.
113 g the pathogenesis of the different forms of HUS may prove helpful in clinical practice.
114 iga toxin mediated and the atypical forms of HUS, with a focus on genetic variations in the complemen
115 inach, there was a notably high frequency of HUS.
116                                Management of HUS remains supportive; there are no specific therapies
117 nfected outpatients without manifestation of HUS, were investigated between May 15 and July 26, 2011,
118 osystem can function as an in vitro model of HUS and showed that shear stress influences microvascula
119           We have developed a mouse model of HUS by administering endotoxin-free Stx2 in multiple dos
120                             A mouse model of HUS designed to mirror human mutations in FH has now bee
121 at this C57BL/6 mouse is a complete model of HUS that includes the thrombocytopenia, hemolytic anemia
122 he contribution of CCR1 in a murine model of HUS.
123  the frequency of bloody diarrhea but not of HUS and the length of the incubation period depended on
124 dy their efficacy in preventing the onset of HUS during the systemic blood phase of Stx.
125 type have been linked to the pathogenesis of HUS.
126 nd their contribution to the pathogenesis of HUS.
127  the understanding of the pathophysiology of HUS and could have an important effect on the developmen
128 apheresis (n = 13) during the acute phase of HUS had comparable outcomes.
129 e that, when performed during progression of HUS, passive immunization of mice with anti-Stx2 antibod
130                           The higher rate of HUS was observed across all antibiotic classes used.
131 e farm prior to the first clinical report of HUS.
132 uraged because it might increase the risk of HUS development.
133 trongly associated with an increased risk of HUS, and eae was strongly associated with an increased r
134                                      Sera of HUS patients, but not healthy individuals, recognized Hc
135 fice, moribund animals demonstrated signs of HUS: increased blood urea nitrogen and serum creatinine
136 accharide (LPS) caused signs and symptoms of HUS in mice, but the mechanism leading to renal failure
137                                 This type of HUS is characterized by obstruction of the glomeruli and
138 per limit of normal for age) and oligoanuric HUS.
139 sociated with the development of oligoanuric HUS (OR, 2.38 [95% CI, 1.30-4.35]; I2 = 2%), renal repla
140 sociated with the development of oligoanuric HUS.
141 104:H4 was equivalent to previous reports on HUS due to other types of Shiga toxin-producing E. coli
142  (HUS), thrombotic microangiopathy (TMA), or HUS-like events, exceeding the MTCD.
143              During 2000-2007, 627 pediatric HUS cases were reported.
144 ho had been included in the German Pediatric HUS Registry during the 2011 outbreak.
145 s population-based surveillance of pediatric HUS to measure the incidence of disease and to validate
146 nce rate for all reported cases of pediatric HUS was 0.78 per 100,000 children <18 years.
147           The overall incidence of pediatric HUS was affected by key characteristics of the surveilla
148         We report the incidence of pediatric HUS, which is defined as HUS in children <18 years.
149 re significantly different in HUS vs the pre-HUS phase of illness or uncomplicated infection.
150          Angiopoietin dysregulation preceded HUS and worsened as HUS developed.
151                      The best way to prevent HUS is to prevent primary infection with Shiga-toxin-pro
152 erstanding of bacterial factors that promote HUS is incomplete.
153     Both patients had a history of recurrent HUS after transplantation.
154 t to index visit was associated with reduced HUS risk (OR, 0.70 [95% CI, .54-.90]).
155 s are hampered by the inability to reproduce HUS with thrombotic microangiopathy, hemolytic anemia, a
156                          Herein are reviewed HUS and TTP along with recent progress shedding new ligh
157 athogenesis of STEC-HUS, aHUS, and secondary HUS are discussed.
158 ic features in STEC-HUS, aHUS, and secondary HUS are simultaneous damage to endothelial cells, intrav
159 olled complement activation, or as secondary HUS with a coexisting disease.
160                          Some have secondary HUS with a coexisting disease or trigger such as autoimm
161 and some patients with STEC-HUS or secondary HUS.
162 xin-free Stx challenge exhibit full spectrum HUS, including thrombocytopenia, hemolytic anemia, and A
163 cting data for the use of eculizumab in STEC HUS.
164 associated haemolytic uraemic syndrome (STEC HUS) is also provided.
165 isits in patients are recommended after STEC-HUS.
166  the hemolytic process occurring during STEC-HUS.
167 ent and future choices of therapies for STEC-HUS.
168  is evident in Shiga toxin-induced HUS (STEC-HUS).
169                        Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whe
170     The common pathogenetic features in STEC-HUS, aHUS, and secondary HUS are simultaneous damage to
171 and similarities in the pathogenesis of STEC-HUS, aHUS, and secondary HUS are discussed.
172 erived microvesicles from patients with STEC-HUS (n = 25) were investigated for the presence of C3 an
173 psilon mutation, and some patients with STEC-HUS or secondary HUS.
174  identified in 1 patient each with fatal Stx-HUS, the HELLP (hemolysis, elevated liver enzymes, and l
175 ogy of renal microvascular thrombosis in Stx-HUS is still ill-defined.
176 ding to podocyte dysfunction and loss in Stx-HUS.
177 nction may adversely affect evolution of Stx-HUS.
178 for using soluble TM in the treatment of Stx-HUS.
179  associated with a higher rate of subsequent HUS and should be avoided.
180 hoea-associated haemolytic uraemic syndrome (HUS) are caused by Shiga-toxin-producing bacteria; the p
181 ng 855 cases with hemolytic uremic syndrome (HUS) and 53 deaths.
182 or development of hemolytic uremic syndrome (HUS) and acute kidney injury (AKI).
183 e major causes of hemolytic uremic syndrome (HUS) and acute renal failure in children.
184  life-threatening hemolytic uremic syndrome (HUS) and are the main virulence factors of enterohemorrh
185 ngiopathy are the hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP), the
186 similarities with hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP).
187 purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's diffe
188 hagic colitis and hemolytic uremic syndrome (HUS) by colonizing the gut mucosa and producing Shiga to
189 are isolated from hemolytic-uremic syndrome (HUS) cases more frequently than are strains that produce
190                   Hemolytic uremic syndrome (HUS) caused by intestinal Shiga toxin-producing Escheric
191                   Hemolytic-uremic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli in
192                   Hemolytic-uremic syndrome (HUS) features episodes of small-vessel thrombosis result
193 STEC) can lead to hemolytic-uremic syndrome (HUS) in 5 to 10% of patients.
194 he development of hemolytic-uremic syndrome (HUS) in a small percentage of infected humans.
195  life-threatening hemolytic uremic syndrome (HUS) in any of 6 closed cohorts from 4 countries (1 coho
196 ith >800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children.
197                   Hemolytic uremic syndrome (HUS) is a potentially life-threatening condition.
198                   Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by in
199                   Hemolytic-uremic syndrome (HUS) is a thrombotic microangiopathy that is characteriz
200               The hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, th
201          Atypical hemolytic uremic syndrome (HUS) is associated with high recurrence rates after kidn
202 157:H7-associated hemolytic-uremic syndrome (HUS) is characterized by profound prothrombotic abnormal
203                   Hemolytic uremic syndrome (HUS) is the life-threatenig sequela of intestinal infect
204     Postdiarrheal hemolytic uremic syndrome (HUS) is the most common cause of acute kidney failure am
205               The hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in
206   Brain injury in hemolytic-uremic syndrome (HUS) may be enhanced by inflammatory cytokine up-regulat
207                   Hemolytic uremic syndrome (HUS) occurred in 12 patients (10 infected with STEC O157
208 high incidence of hemolytic uremic syndrome (HUS) occurred in Germany in May 2011.
209 known outbreak of hemolytic uremic syndrome (HUS) occurred in northern Germany.
210 , 8 patients with hemolytic uremic syndrome (HUS) or bloody diarrhea were reported in France.
211 nfection leads to hemolytic uremic syndrome (HUS) or other complications.
212                   Hemolytic-uremic syndrome (HUS) results from infection by Shiga toxin (Stx)-produci
213       Identifying hemolytic uremic syndrome (HUS) risk factors is needed to guide care.
214 ls with diarrheal hemolytic uremic syndrome (HUS) seen at our institution during the study period, 16
215  infection called hemolytic-uremic syndrome (HUS) than isolates that make Stx1a only or produce both
216 arrhea-associated hemolytic uremic syndrome (HUS), a disorder of glomerular ischemic damage and wides
217 arrhea-associated hemolytic uremic syndrome (HUS), a disorder of thrombocytopenia, microangiopathic h
218 s and can lead to hemolytic-uremic syndrome (HUS), a life-threatening condition that principally affe
219 agent that causes hemolytic uremic syndrome (HUS), a microangiopathic disease characterized by hemoly
220 ction can lead to hemolytic-uremic syndrome (HUS), a severe disease characterized by hemolysis and re
221 t can progress to hemolytic uremic syndrome (HUS), a systematic microvascular syndrome with predomina
222 ns that can cause hemolytic-uremic syndrome (HUS), a thrombotic microangiopathy, following infections
223 t and severity of hemolytic uremic syndrome (HUS), and adverse outcomes in STEC-infected individuals.
224                   Hemolytic-uremic syndrome (HUS), caused by Shiga toxin (Stx)-producing Escherichia
225  colitis (HC) and hemolytic uremic syndrome (HUS), due to the expression of one or more Shiga toxins
226 ations, including hemolytic-uremic syndrome (HUS), in animal models of disease.
227 e associated with hemolytic uremic syndrome (HUS), membranoproliferative glomerulonephritis (dense de
228  postenteropathic hemolytic uremic syndrome (HUS), most commonly caused by Shiga toxin (Stx)-producin
229 otentially lethal hemolytic uremic syndrome (HUS), particularly in children.
230 disease (SCD) and hemolytic uremic syndrome (HUS), pathological biophysical interactions among blood
231                   Hemolytic-uremic syndrome (HUS), the life-threatening complication following infect
232 0 doses developed hemolytic uremic syndrome (HUS), thrombotic microangiopathy (TMA), or HUS-like even
233                   Hemolytic uremic syndrome (HUS), which is caused by Shiga toxin-producing Escherich
234  colitis (HC) and hemolytic-uremic syndrome (HUS), which is the most common cause of acute renal fail
235 nfection, such as hemolytic-uremic syndrome (HUS), zinc might be capable of preventing severe sequela
236 isted adults with hemolytic uremic syndrome (HUS).
237 coli (STEC) cause hemolytic uremic syndrome (HUS).
238 equela called the hemolytic uremic syndrome (HUS).
239 c colitis and the hemolytic-uremic syndrome (HUS).
240  including 4 with hemolytic uremic syndrome (HUS).
241 viduals developed hemolytic-uremic syndrome (HUS).
242 ease consequence, hemolytic-uremic syndrome (HUS).
243 uses diarrhea and hemolytic uremic syndrome (HUS).
244  life-threatening hemolytic uremic syndrome (HUS).
245  leading cause of hemolytic uremic syndrome (HUS).
246  of children with hemolytic uremic syndrome (HUS).
247 purpura (TTP) and hemolytic uremic syndrome (HUS).
248  leading cause of hemolytic-uremic syndrome (HUS).
249 n associated with hemolytic-uremic syndrome (HUS).
250 t common cause of hemolytic-uremic syndrome (HUS).
251 spose to atypical hemolytic uremic syndrome (HUS).
252 oody diarrhea and hemolytic-uremic syndrome (HUS).
253 arrhea-associated hemolytic uremic syndrome (HUS).
254  colitis (HC) and hemolytic uremic syndrome (HUS).
255 th development of hemolytic uremic syndrome (HUS).
256  650), glomerular-hemolytic uremic syndrome (HUS; n = 49), or glomerular-non-HUS (heterogeneous child
257                                       Of the HUS cases in which STEC was isolated, 28 (90%) were attr
258 n the dialysis-maintained cohorts within the HUS (HR, 0.56; 95% CI, 0.35-0.91), HTN (HR, 0.50; 95% CI
259 = 11) exceeded the MTCD because of 2 HUS/TMA/HUS-like events.
260 mined, suggesting that several approaches to HUS surveillance can be used to track trends.
261 he inflammatory response that contributes to HUS development.
262               The vascular injury leading to HUS is likely to be well under way by the time infected
263 sis and direct endothelial injury leading to HUS phenotype.
264  toxin-producing Escherichia coli leading to HUS was suspected following histology obtained at colono
265 re elevated in individuals who progressed to HUS.
266  of antibiotic administration in relation to HUS.
267  a significant proportion of non-shiga toxin HUS.
268                             The Oklahoma TTP-HUS (hemolytic uremic syndrome) Registry enrolled 70 con
269  <10%) in women enrolled in the Oklahoma TTP-HUS Registry from 1995 to 2012.
270                                      Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection
271 iopoietins 1 and 2 (Ang-1/2), could underlie HUS pathophysiology.
272 risk-of-bias studies employing commonly used HUS criteria.
273 ransplantation probability in the waitlisted HUS cohort was 60% versus 42% to 49% (P < 0.001) in the
274      The primary and secondary outcomes were HUS (hematocrit <30% with smear evidence of hemolysis, p
275 efit over dialysis in waitlisted adults with HUS.
276 tx2a only were significantly associated with HUS (odds ratio, 14.2; 95% confidence interval, 7.9-25.6
277 iotic use were independently associated with HUS and, additionally, these variables were each associa
278  strains are more frequently associated with HUS than Stx1-producing strains.
279 of Stx2a, the major Stx type associated with HUS, on human renal glomerular endothelial cells (HRGEC)
280 ophage to the kidney disease associated with HUS.
281 ntribute to the renal damage associated with HUS.
282 promote the renal thrombosis associated with HUS.
283                               Two cases with HUS died.
284 ation and adverse outcomes for children with HUS.
285 s (GN) were analyzed, and then compared with HUS patients.
286 ted by the bacteria, is directly linked with HUS.
287 ed by the bacterium, is directly linked with HUS.
288                        TM(LeD/LeD) mice with HUS had a higher mortality rate than TM(wt/wt) mice.
289 associated with bloody diarrhea but not with HUS.
290  be an effective treatment for patients with HUS and/or individuals at high risk of developing HUS du
291 with STEC infection, including patients with HUS as well as STEC-infected outpatients without manifes
292                                Patients with HUS receive only supportive treatment as the benefit of
293        Among the 30% to 50% of patients with HUS who have no detectable complement defect, some have
294 ed in vitro and as observed in patients with HUS.
295  IF mutations in a panel of 76 patients with HUS.
296 ure of hydration status at presentation with HUS was associated with the development of oligoanuric H
297     Two hundred nine patients presented with HUS.
298                     Patients presenting with HUS had a significantly shortened shedding duration (med
299 has the strongest association worldwide with HUS.
300 y outcome) in STEC-infected children without HUS at initial presentation.

 
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