ライフサイエンスコーパス: coagulopathy

1 CMV activation in the recipient (consumptive coagulopathy).

2 insic pathway of coagulation (sepsis-induced coagulopathy).

3 a (APL) is commonly complicated by a complex coagulopathy.

4 and improve thrombin generation in acquired coagulopathy.

5 ested that ANXII plays a pivotal role in APL coagulopathy.

6 orrhagic shock treatment for acute traumatic coagulopathy.

7 ransgenic mice, worsening their survival and coagulopathy.

8 infection, vasculopathy, cardioembolism, and coagulopathy.

9 s dysregulate hemostatic pathways, prompting coagulopathy.

10 road spectrum of cardiovascular diseases and coagulopathy.

11 must occur before laboratory confirmation of coagulopathy.

12 egrees C), acidosis (median pH <7.2), and/or coagulopathy.

13 sed for markers of leukocyte trafficking and coagulopathy.

14 esistance, impaired drug clearance, and mild coagulopathy.

15 tical factor that acts by inducing placental coagulopathy.

16 votal role in haemostasis and trauma-induced coagulopathy.

17 emostasis through prevention or treatment of coagulopathy.

18 l patients with the most aggravated forms of coagulopathy.

19 vicious cycle of hypothermia, acidosis, and coagulopathy.

20 leeding risk in critically ill patients with coagulopathy.

21 ying myosin might contribute to acute trauma coagulopathy.

22 d should not be used exclusively to evaluate coagulopathy.

23 generation due to disseminated intravascular coagulopathy.

24 nd provided guidance for limiting dilutional coagulopathy.

25 inflammation, and measures of sepsis-induced coagulopathy.

26 that Gas6 may be involved in cancer-induced coagulopathy.

27 synthetic function accounts for most of the coagulopathy.

28 wth retardation, without evidence of a gross coagulopathy.

29 ococcal sepsis invariably is associated with coagulopathy.

30 l management of patients with trauma-induced coagulopathy.

31 both patients had evidence of a consumptive coagulopathy.

32 ratio-based definitions for acute traumatic coagulopathy.

33 l and esthetic impairment, and intravascular coagulopathy.

34 a role for cryoprecipitate in reversing rtPA coagulopathy.

35 tant severe pathology (liver and spleen) and coagulopathy.

36 h a 66% lower risk of clinically significant coagulopathy.

37 apid and reliable method to assess traumatic coagulopathy.

38 reased risk of bleeding due to a consumption coagulopathy.

39 garding fluid resuscitation and treatment of coagulopathy.

40 instability, capillary leak, and consumptive coagulopathy.

41 a high mortality and is increased in case of coagulopathy.

42 ections are frequently complicated by severe coagulopathies.

43 eceive anticoagulation therapy or experience coagulopathies.

44 The most prevalent comorbidities were coagulopathy (36.0%), fluid and electrolyte disorders (3

45 eminated intravascular coagulation and other coagulopathies accompanying severe sepsis.

46 Acute traumatic coagulopathy affected 50% of subjects if defined as an i

47 r results suggest a new mechanism of anthrax coagulopathy affecting the levels and functional activit

48 Dilutional coagulopathy after resuscitation with crystalloids/collo

49 m identified bowel gangrene and peritonitis, coagulopathy, age, the use of stoma, and chronic kidney

50 trigger immune activation, inflammation, and coagulopathy, all of which are key factors that drive HI

51 national normalised ratio >1.5), and one had coagulopathy alone.

52 llowing a rapid and timely identification of coagulopathy along with enabling an individualized, goal

53 nsights into the pathogenesis of RVV-induced coagulopathies and indicate that DrKIn-I is a novel APC

54 Pros1-/- mice die in utero,from a fulminant coagulopathy and associated hemorrhages.

55 Coagulopathy and bleeding after severe injury is a commo

56 l injury markers (HMGB1, histones) confirmed coagulopathy and cell injury.

57 ased on the presence of clinical features of coagulopathy and elevated levels of proinflammatory cyto

58 This previously-well male developed profound coagulopathy and encephalopathy 6 weeks after the onset

59 period fulfilling standard criteria for ALF (coagulopathy and encephalopathy), from which 275 (42%) w

60 hepatocytes causes acute liver failure with coagulopathy and encephalopathy.

61 in the form of microparticles contribute to coagulopathy and further organ dysfunction.

62 ary outcome was drug-induced ALF (defined as coagulopathy and hepatic encephalopathy without underlyi

63 Massive hemorrhage is associated with coagulopathy and high mortality.

64 e found that FXI-deficient mice have reduced coagulopathy and increased survival relative to FXI-expr

65 cations of perioperative hypothermia include coagulopathy and increased transfusion requirement, surg

66 llows for rapid and timely identification of coagulopathy and individualized, goal-directed transfusi

67 t appear to influence baseline biomarkers of coagulopathy and inflammation or disease severity, with

68 agement of patients with warfarin-associated coagulopathy and intracerebral hemorrhage.

69 Whether this early coagulopathy and later propensity to infection, multiple

70 point-of-care device to detect the onset of coagulopathy and monitor response following therapeutic

71 allows 'point-of-care' testing of postinjury coagulopathy and monitoring of transfusion strategies.

72 ment with RA101295 also improved consumptive coagulopathy and preserved endothelial anticoagulant and

73 theless, massive transfusion always leads to coagulopathy and so is at best an adjunct to good surgic

74 Coagulopathy and systemic inflammation are almost univer

75 t changes and advancement of early traumatic coagulopathy and the important role of substantial bleed

76 clinical hallmarks of EHF, including severe coagulopathy and uncontrolled host immune responses.

77 e showed signs of disseminated intravascular coagulopathy and were lost because of proteinuria or inf

78 his finding suggests that FXI contributes to coagulopathy and/or inflammation during sepsis and that

79 fibrinogen replacement therapy for acquired coagulopathies, and additional studies are underway.

80 es bowel rest, correction of cytopathies and coagulopathies, and broad spectrum antibiotics and antif

81 The major limitations are infection, coagulopathies, and device dysfunction.

82 detection and intervention for hypothermia, coagulopathy, and acidosis, to avoid extreme pathophysio

83 ory host responses, severe thrombocytopenia, coagulopathy, and death.

84 ete spectrum of HLH, including splenomegaly, coagulopathy, and decreased NK cell cytotoxicity, indica

85 ent, potentiating the inflammatory response, coagulopathy, and depressed fibrinolysis.

86 patic diseases, obesity, anemia, malignancy, coagulopathy, and depression and other psychiatric illne

87 els of hepatic fibrin, decreased evidence of coagulopathy, and diminished cytokine production (interl

88 is is associated with systemic inflammation, coagulopathy, and disrupted protein C (PC) pathway funct

89 o" of RBC:FFP leads to earlier correction of coagulopathy, and earlier and prolonged repletion of som

90 ypothermia and acidosis, direct treatment of coagulopathy, and early transfusion in trauma patients.

91 ur), and is associated with MT requirements, coagulopathy, and hemorrhage-related death.

92 issue perfusion and for preventing acidosis, coagulopathy, and hypothermia, referred to as the 'letha

93 h the anti-NS1-DR4 Ig led to plasma leakage, coagulopathy, and morality in mice with warfarinized ant

94 variables indicative of severe inflammation, coagulopathy, and muscle damage including less bacterial

95 ly infected with P. coatneyi develop anemia, coagulopathy, and renal and metabolic dysfunction.

96 ent sepsis-induced inflammation, consumptive coagulopathy, and subsequent organ failure and death.

97 use deformity, pain, and local intravascular coagulopathy, and they expand with time.

98 early in severe shock, leading to postinjury coagulopathy, and ultimately hemorrhage-related death.

99 in multiple ways to endothelial activation, coagulopathy, and vascular leakage in sepsis.

100 ic syndrome resulting from thrombocytopenia, coagulopathy, and vasculopathy.

101 Since coagulopathies are a hallmark of T2D, we wondered whethe

102 These data imply that coagulopathies are an important feature of T2D, and may

103 We conclude that whereas coagulopathies are more exacerbated in LPS-treated TF(-/

104 Conventional concepts of traumatic coagulopathy as a late occurring condition in response t

105 gulation inhibitor activity triggered severe coagulopathy as indicated by prolonged coagulation times

106 ads to increased clot firmness in dilutional coagulopathy as measured with thromboelastometry.

107 sting aids anesthesiologists in diagnosis of coagulopathy as well as therapeutic optimization of anti

108 s in patients with cirrhosis and significant coagulopathy (as defined by routine coagulation tests) a

109 ecent work has improved understanding of the coagulopathy associated with acidosis and provided guida

110 Antifibrinolytics are used to attenuate the coagulopathy associated with cardiopulmonary bypass.

111 o include chronic disseminated intravascular coagulopathy associated with microangiopathy, verrucous

112 near-normal prothrombin time can reverse the coagulopathy associated with superwarfarins.

113 omboelastometry can identify acute traumatic coagulopathy at 5 mins and predict the need for massive

114 ology and pathophysiology of acute traumatic coagulopathy (ATC).

115 In patients with cirrhosis and significant coagulopathy before invasive procedures, TEG-guided tran

116 ejection or the development of a consumptive coagulopathy, biopsy specimens were obtained for studies

117 e treatment of sepsis-associated consumptive coagulopathy, but its application is limited because of

118 Acidosis is an important predictor of coagulopathy, but the underlying contributing mechanisms

119 We are moving toward avoiding coagulopathy by individualized, goal-directed transfusio

120 Thus, in 2005, a strategy aiming at avoiding coagulopathy by proactive resuscitation with blood produ

121 Defining acute traumatic coagulopathy by using an international normalized ratio

122 n, a potentially interfering molecule in the coagulopathy cascade.

123 brinolysis, therapeutic anticoagulation, and coagulopathies caused by dilution and consumption in the

124 Hemophilia B is an X-linked coagulopathy caused by absence of functional coagulation

125 Hepatic transplantation may result in coagulopathy caused by the release of mast-cell-derived

126 idly progressive disorder termed consumptive coagulopathy (CC) has been observed frequently in pig-to

127 ssibly an increased incidence of consumptive coagulopathy (CC).

128 vation of the PC which was associated with a coagulopathy characterized by inactivation of the coagul

129 ecause of the perception that the coexistent coagulopathy could promote bleeding.

130 Early identification of shock and coagulopathy coupled with damage control resuscitation a

131 isease, hypothyroidism, liver disease, AIDS, coagulopathy, deficiency anemia, obesity, alcohol abuse,

132 Acute traumatic coagulopathy defined as an international normalized rati

133 t for potential confounders, acute traumatic coagulopathy defined as an international normalized rati

134 ime ratio was calculated and acute traumatic coagulopathy defined as laboratory prothrombin time rati

135 n in patients with cirrhosis and significant coagulopathy (defined in this study as INR >1.8 and/or p

136 the expression of tissue factor, consumptive coagulopathy developed irrespective of histopathologic f

137 r understanding of the process by which this coagulopathy develops and how it may be inhibited.

138 Our understanding of perioperative coagulopathy, diagnostic tools, and therapeutic approach

139 treatments may not be sufficient to reverse coagulopathy early enough to prevent hematoma expansion

140 ry to acute liver failure and include severe coagulopathy, encephalopathy, adult respiratory distress

141 n of findings, including hyperbilirubinemia, coagulopathy, encephalopathy, and ascites formation.

142 y state induced by sepsis, the potential for coagulopathy exists because of up-regulation of natural

143 nt acidosis, hypothermia and the progressive coagulopathy following injury, trauma victims the world

144 Coagulopathy following major trauma is conventionally at

145 on recent literature regarding treatment of coagulopathy for patients with life-threatening bleeding

146 ach for fluid resuscitation and treatment of coagulopathy for trauma patients.

147 cytopenia, elevated aminotransferase levels, coagulopathy, graft dysfunction, and either fever or leu

148 Coagulopathy has a significant impact on survival after

149 Our understanding of hemostasis and coagulopathy has improved, leading to a change in the pr

150 derstanding of the hemostatic process and of coagulopathy has improved.

151 on with prolonged fever, hepatosplenomegaly, coagulopathy, hematologic cytopenias, and evidence of he

152 An understanding of the coagulopathy, hemostatic considerations, and therapeutic

153 clerosis, insulin resistance, dyslipidemias, coagulopathies, hypertension, and a pro-inflammatory sta

154 d lead to stunted growth, liver dysfunction, coagulopathy, hypoglycemia, and intestinal abnormalities

155 s of fibrin and display evidence of systemic coagulopathy (i.e., thrombocytopenia, fibrinogen depleti

156 o have neurological symptoms associated with coagulopathies, immune dysfunction with or without infec

157 mboelastography, demonstrated development of coagulopathies in LPS-treated mice, which were more seve

158 implicated in the development of consumptive coagulopathies in severely envenomed patients.

159 This study defines evolution of coagulopathy in 10 pigs with acetaminophen (APAP)-induce

160 ged graft survival and prevented consumptive coagulopathy in all recipients.

161 PURPOSE OF REVIEW: Coagulopathy in an ICU setting is multifactorial, but ne

162 on algorithm would improve the management of coagulopathy in cardiac surgery and thereby reduce blood

163 to best diagnose and manage acute traumatic coagulopathy in children.

164 diagnosis, and management of acute traumatic coagulopathy in children.

165 All anticoagulation agents may contribute to coagulopathy in critically ill patients.

166 , and APAP hepatotoxicity is associated with coagulopathy in humans.

167 The management of coagulopathy in patients with acute and chronic liver di

168 coagulation pathway effectively reduces the coagulopathy in sepsis.

169 , little is known regarding the mechanism of coagulopathy in StrepTSS.

170 S and host cells contributes to the observed coagulopathy in StrepTSS.

171 onsidered in the prevention and treatment of coagulopathy in surgical patients.

172 increasing recognition of the importance of coagulopathy in the evolution of this disease.

173 n therapy, need to be considered in managing coagulopathy in the ICU setting.

174 ine) and Mg on hypotensive resuscitation and coagulopathy in the rat model of severe hemorrhagic shoc

175 iopathy in the graft or systemic consumptive coagulopathy in the recipient.

176 on complicated by disseminated intravascular coagulopathy in the teriflunomide 14 mg group).

177 s incompatible with prompt correction of the coagulopathy in the trauma setting.

178 responsible for persistent ICH and post-TBI coagulopathy in this model and offer a novel approach to

179 r VII (rFVIIa) as an adjunct for reversal of coagulopathy in trauma patients, and numerous other publ

180 limitations in the management of postinjury coagulopathy include the lack of a uniform definition of

181 evelopment of this drug in sepsis-associated coagulopathy including disseminated intravascular coagul

182 examines the current approach to postinjury coagulopathy, including identification of patients at ri

183 The coagulopathy induced in this study resulted in a reprodu

184 nked to the development of sequelae, such as coagulopathy, infection, morbid myocardial events, and d

185 ed bleeding is often ascribed to consumptive coagulopathy initiated by exposed brain tissue factor.

186 We postulated that the coagulopathy initiated by the inflammatory response to s

187 The coagulopathy injury resulted in short-term hypoxia in th

188 ation and extent of ischemia induced after a coagulopathy injury to the optic nerve of adult rats.

189 Tissues were hypoxic within 15 min of the coagulopathy injury, but normoxic by 24 h as measured by

190 We identified 115 patients with coagulopathy (INR of > or = 1.5) but without active blee

191 alpha-fetoprotein alone, including four with coagulopathy (international normalised ratio >1.5), and

192 ytopenia (platelet count < 150x10(3)/mm(3)), coagulopathy (International Normalized Ratio>2.0), and r

193 Traumatic coagulopathy is a complex process that leads to global d

194 orrhage in patients with warfarin-associated coagulopathy is an increasingly common life-threatening

195 Acute traumatic coagulopathy is associated with adverse outcomes includi

196 he emergency department, and the presence of coagulopathy is associated with increased morbidity and

197 We have previously reported that this coagulopathy is due to the association of severe injury

198 We hypothesized that early coagulopathy is due to tissue hypoperfusion, and investi

199 Acute traumatic coagulopathy is functionally characterized by a reductio

200 cornerstone of supportive treatment of this coagulopathy is management of the underlying condition.

201 A lethal triad of hypothermia, acidosis, and coagulopathy is the direct result of trauma and secondar

202 Lack of PS in mice causes lethal coagulopathy, ischemic/thrombotic injuries, vascular dys

203 rmia did not further aggravate shock-related coagulopathy, it caused a transitory attenuation of kidn

204 emangioendothelioma finds that an associated coagulopathy (Kasabach-Merritt phenomenon) occurs in 72%

205 ommon than IH but more often associated with coagulopathy (Kasabach-Merritt phenomenon).

206 Patients (n = 121) with acquired coagulopathy, largely due to liver disease, including he

207 Clinical pathology findings included coagulopathy, leukocytosis, and profound liver destructi

208 Higher bilirubin, coagulopathy, leukocytosis, and thrombocytopenia were in

209 loendothelial organs, immunostimulation, and coagulopathies, limit their application as therapeutics.

210 ve heart failure, chronic pulmonary disease, coagulopathy, liver disease, lymphoma, fluid and electro

211 fever, splenomegaly, neurologic dysfunction, coagulopathy, liver dysfunction, cytopenias, hypertrigly

212 d-stage liver disease, vitamin K-independent coagulopathy, low-to-normal serum gamma-glutamyl transfe

213 sion in critically ill medical patients with coagulopathy may not be favorable.

214 Preparation should include correcting coagulopathy, minimizing preexisting encephalopathy, pre

215 n invasive infection characterized by marked coagulopathy, multiple organ failure, and rapid tissue d

216 es mellitus, chronic kidney disease, anemia, coagulopathy, obesity, major bleeding, acute myocardial

217 mechanisms are also likely to contribute to coagulopathies observed in pig-to-primate xenotransplant

218 ation of these findings implicates the mixed coagulopathy observed in cirrhosis.

219 inical or laboratory features of consumptive coagulopathy occurred in 7 of 12 baboons.

220 ue factor expression; in vivo, a consumptive coagulopathy occurred when there was xenoreactive antibo

221 om these classic descriptions to any kind of coagulopathy occurring in the setting of any kind of mal

222 Acute traumatic coagulopathy occurs early in hemorrhagic trauma and is a

223 Coagulopathy occurs frequently in critically ill patient

224 Coagulopathy occurs in ICU patients because of multiple

225 Acute traumatic coagulopathy occurs in the presence of tissue hypoperfus

226 Early traumatic coagulopathy occurs only in the presence of tissue hypop

227 5% confidence interval, 2.25-5.36; P<0.001), coagulopathy (odds ratio, 2.19; 95% confidence interval,

228 usion protocols standardize treatment of the coagulopathy of massive bleeding, leading to rapid resto

229 in the bloodstream and mitigates the lethal coagulopathy of sepsis.

230 In addition to the expected coagulopathy of the f12(-/-) phenotype, mice homozygous

231 d our obligation to recognize and manage the coagulopathy of trauma better than in past years.

232 and a defined mechanistic link to the "Acute Coagulopathy of Trauma" is controversial.

233 udes imbalanced inflammatory response, acute coagulopathy of trauma, and endovascular glycocalyx degr

234 n the early recognition and treatment of the coagulopathy of trauma, as well as ongoing work to defin

235 administered from the outset to prevent the coagulopathy of trauma.

236 hat demonstrates the utility of managing the coagulopathy of trauma.

237 icance of recognizing the main influences in coagulopathy of trauma.

238 ns must anticipate, recognize and manage the coagulopathy of trauma.

239 trategy for liver transplant recipients with coagulopathy or hemodynamic instability after allograft

240 oid arthritis (OR, 1.19; 95% CI, 1.10-1.29); coagulopathy (OR, 1.19; 95% CI, 1.08-1.32); hypertension

241 95% confidence interval [CI]: 1.58 to 2.52), coagulopathy (OR: 2.35; 95% CI: 1.88 to 2.94), and cardi

242 1, 95% CI: 1.4 to 3.2 per mg/dl creatinine), coagulopathy (OR: 3.1, 95% CI: 1.7 to 5.8 per internatio

243 then ensues, resulting in graft destruction, coagulopathy, or both within weeks.

244 s to the xenograft and the induction of AVR, coagulopathy, or both.

245 Exclusion criteria were infection, coagulopathy, or emergency cases.

246 as not associated with hypertriglyceridemia, coagulopathy, or essential fatty acid deficiency.

247 uding altered clotting factor processing and coagulopathy, organ level effects such as hemorrhage, or

248 but not uPA(-/-), mice developed a systemic coagulopathy post-TBI.

249 emarkably, even in patients with significant coagulopathy, postprocedure bleeding was rare, indicatin

250 alcium level may be associated with a subtle coagulopathy predisposing to increased bleeding and migh

251 colleagues advance our understanding of the coagulopathy present in acute promyelocytic leukemia (AP

252 Recent studies have identified an acute coagulopathy present on admission that is independent of

253 tal role in mediating cytoprotection against coagulopathy, proinflammatory cytokines, and vascular pe

254 Progressive postinjury coagulopathy remains the fundamental rationale for damag

255 Coagulopathy represents one of the major challenges in t

256 tions include cytopenias, liver dysfunction, coagulopathy resembling disseminated intravascular coagu

257 Trauma-induced coagulopathy results from a complex interplay between sh

258 Previous studies examining acute traumatic coagulopathy's relation with mortality are limited by in

259 result of the inconsistent manifestation of coagulopathy seen in EHF.

260 hreatening hemorrhage at risk for postinjury coagulopathy should receive component therapy in rations

261 Uncontrolled bleeding from coagulopathy signals imminent death in severely injured

262 rted hemostasis in the treatment of acquired coagulopathy similarly to conventional FFP.

263 ng abnormal haematology, blood chemistry and coagulopathy, siRNA-treated animals had milder clinical

264 The presence of bowel gangrene and coagulopathy strongly predicts mortality, suggesting tha

265 rotect against histone-induced cytotoxicity, coagulopathy, systemic inflammation, and organ damage du

266 zi et al challenge the view that consumptive coagulopathy that accompanies traumatic brain injury (TB

267 The severe coagulopathy that followed combined "knockdown" of these

268 this will progress rapidly to an endogenous coagulopathy that is independently associated with worse

269 t studies have identified an acute traumatic coagulopathy that is present on admission to the hospita

270 roducts, often fail to effectively treat the coagulopathy that is present on arrival in these casualt

271 s not available or not adequately correcting coagulopathy, the risk:benefit ratio of warm fresh whole

272 In acute traumatic coagulopathy, the rotational thromboelastometry clot amp

273 vere restrictive lung disease, and one had a coagulopathy; the other five patients had nonpulmonary p

274 ay premature death due to thrombosis-related coagulopathy, thereby precluding their use in gene funct

275 Control patients with coagulopathies, thrombocytopenia, cancer, and hyperglyce

276 DCR aims at preventing coagulopathy through permissive hypotension, limiting cr

277 nd aggressive treatment of trauma-associated coagulopathy through transfusion of high plasma to packe

278 e, predefined risk factors, and treatment of coagulopathy throughout intensive care unit admission.

279 ding, commonly referred to as trauma-induced coagulopathy (TIC), affects a quarter of all trauma pati

280 laboratory-based evidence of trauma-induced coagulopathy (TIC), which is associated with poorer outc

281 tality, 30-day mortality, and trauma-induced coagulopathy [(TIC), admission international normalized

282 Thrombomodulin-associated coagulopathy (TM-AC) is a newly recognized dominant blee

283 rgeting future therapies for acute traumatic coagulopathy to patients with an international normalize

284 With the recognition of early coagulopathy, trauma resuscitation has shifted toward li

285 Resolved HBV infection, coagulopathy types and severity, types of clotting facto

286 eria were terminal disease, pregnancy, known coagulopathy, uncontrolled bleeding, temperature on admi

287 ring sustained diuretics or paracentesis, or coagulopathy unresponsive to vitamin K requiring fresh-f

288 ek-old African-American girl presenting with coagulopathy, vitamin D and E deficiencies, and mild cho

289 Coagulopathy was attenuated, and plasma interleukin-6, i

290 Coagulopathy was defined as r-TEG clot strength = G < 5.

291 The coagulopathy was prevented by dabigatran etexilate treat

292 tients over 14 months at risk for postinjury coagulopathy were stratified by transfusion requirements

293 ation, glucose monitoring, and correction of coagulopathy when there is overt bleeding or an invasive

294 a detection rate of 77% for acute traumatic coagulopathy with a false-positive rate of 13%.

295 dysfunction, neutrophil infiltration, airway coagulopathy with cast formation, ventilation-perfusion

296 aminotransferases >200 U/L, (2) severe ALI (coagulopathy with hyperbilirubinemia), and (3) death, al

297 Coagulopathy with sepsis or other variables, such as typ

298 time ratio in patients with acute traumatic coagulopathy, with 29% false-negative results.

299 focused on early and aggressive treatment of coagulopathy, with higher ratios of plasma and platelets

300 e viable as adults and displayed less-severe coagulopathy without vascular dysgenesis.