ライフサイエンスコーパス: SJS

1 SJS is an autosomal recessive skeletal dysplasia charact

2 SJS, SJS/TEN, and TEN pose a substantial health care bur

3 SJS/TEN mostly manifest as a reaction to new drug use, b

4 SJS/TEN serum induced significant MMP9 expression and co

5 SJS/TEN was associated with nonwhite race, particularly

6 The IHD cohort included 11 084 SJS/TEN survivors (mean [SD] age, 56.6 [18.6] years; 556

7 in, affected skin, and blister fluid from 15 SJS/TEN patients.

8 emic Symptoms (DRESS) were suspected, with 2 SJS and 3 DRESS cases considered likely.

9 A total of 20 SJS and 12 TEN cases were included.

10 The studies included 227 SJS or TEN cases, 602 matched control subjects, and 2949

11 ity (15 drug-induced liver injury [DILI], 33 SJS/TEN, 20 hypersensitivity syndrome, and 46 nevirapine

12 The CVA cohort included 10 571 SJS/TEN survivors (mean [SD] age, 56.1 [18.5] years; 535

13 nts, we calculated an incidence rate of 5.76 SJS/TEN cases per million person-years between 1995 and

14 re (SJS: 9.8 +/- 0.3 days, $21,437 +/- $807; SJS/TEN: 16.5 +/- 1.0 days, $58,954 +/- $5,238; TEN: 16.

15 Nine of 48 patients (18 eyes) had acute SJS/TEN from 2000 to 2007 and did not receive protocol c

16 irty-nine of 48 patients (78 eyes) had acute SJS/TEN from 2008 to 2017 and received protocol care (Gr

17 Early AMT is an effective treatment of acute SJS.

18 (CVA) or ischemic heart disease (IHD) after SJS/TEN survival.

19 of CVA and IHD morbidity and mortality after SJS/TEN survival.

20 cular mortality risks peaked at 1 year after SJS/TEN and persisted for 4 to 7 years.

21 3 weeks and recovered from both COVID-19 and SJS life-threatening complications but ocular complicati

22 lva, and blisters on the trunk appeared, and SJS was thus diagnosed.

23 in the phenotypic severity between DDSH and SJS.

24 apine-induced rash plus 3 with both DILI and SJS phenotype) and 155 age-, sex- and ethnicity-matched

25 Causality assessment for DILI and SJS/TEN was carried out with the Roussel Uclaf Causality

26 Prevalence of antibiotic-associated SJS/TEN was presented as pooled proportions with 95% CIs

27 Among antibiotic-associated SJS/TEN, the sulfonamide class was associated with 32% (

28 c review has described antibiotic-associated SJS/TEN.

29 vivors admitted to an intensive care unit at SJS/TEN diagnosis had significantly higher cardiovascula

30 The basis of the association between SJS and ECT is considered, as well as the role of plausi

31 atistically significant associations between SJS/TEN and pre-existing depression, lupus erythematosus

32 pare the acute ocular manifestations between SJS and TEN.

33 on was common during the acute phase in both SJS and TEN.

34 to identify patients with diagnoses of both SJS and ECT.

35 ients were identified with diagnoses of both SJS and ECT.

36 ased cytotoxicity in in vitro models of both SJS/TEN (elicited by drug-specific antigen) and GVHD (el

37 T lymphocytes of patients with carbamazepine-SJS/TEN, with its expression showing drug/phenotype-spec

38 ged length of stay and higher costs of care (SJS: 9.8 +/- 0.3 days, $21,437 +/- $807; SJS/TEN: 16.5 +

39 th caution because of its potential to cause SJS.

40 9 patients with ocular symptoms from chronic SJS who were referred for PROSE fitting evaluation.

41 uantify the tear fluid proteins from chronic SJS/TEN patients (n = 22 eyes) and age- and gender-match

42 ficantly differentially regulated in chronic SJS/TEN.

43 Forty-five eyes of 41 patients with chronic SJS sequelae were recruited and evaluated from 2013 to 2

44 Concomitantly, SJS/TEN displayed a reduced Treg signature compared with

45 tal and observational studies that described SJS/TEN risks since database inception to February 22, 2

46 n (UVR) exposures who subsequently developed SJS or TEN with photodistribution.

47 en identified as risk factors for developing SJS/TEN with severe ocular complications (SOC).

48 ciated effector molecules are known to drive SJS/TEN pathophysiology, but the contribution of innate

49 TNF-alpha inhibitor etanercept, an effective SJS/TEN treatment.

50 g ophthalmic records of patients with either SJS (<30% body surface area involvement) or TEN (> = 30%

51 nscripts in formalin-fixed paraffin-embedded SJS/TEN skin biopsies.

52 d analysing each particular clinical entity: SJS-TEN (62.5% vs 87.5%), MPE (15% vs 47.4%) and AGEP (3

53 justed mortality was 4.8% for SJS, 19.4% for SJS/TEN, and 14.8% for TEN.

54 Mean adjusted mortality was 4.8% for SJS, 19.4% for SJS/TEN, and 14.8% for TEN.

55 The most common inciting agent for SJS was oral medications (85%).

56 No eyes in Group I received AMT for SJS/TEN, compared to 87% of qualifying eyes in Group II

57 modulating therapies or supportive care) for SJS/TEN were selected.

58 The commonest cause for SJS, in 51 patients (85%), was drug reaction.

59 CARs were penicillins and cephalosporins for SJS/TEN and AGEP; glycopeptides for DRESS.

60 The most common reported etiologies for SJS/TENS were antibiotics (n = 25), ibuprofen (n = 15),

61 nternational experts, a case report form for SJS/TEN has been created to help standardize the collect

62 tion may represent the disease mechanism for SJS.

63 anguages looking for treatment proposals for SJS/TEN.

64 ents with epilepsy and gout, odds ratios for SJS/TEN were significantly increased only in the presenc

65 was found to markedly increase the risk for SJS (odds ratio [OR] = 17.52; 95% confidence interval, 3

66 oted differences in treatment strategies for SJS/TEN in Europe; the findings suggest the need for pro

67 sing systemic immunomodulating therapies for SJS/TEN.

68 on possible immunomodulating treatments for SJS/TEN and estimate their effects on mortality compared

69 sed systemic immunomodulating treatments for SJS/TEN, which is of great relevance for treating physic

70 those who have already suffered damage from SJS, but emphasis on the prevention of damage in the acu

71 lymphocytes (CTL) in blister cells from ICI-SJS/TEN skin lesions.

72 ion, Clinical Modification codes to identify SJS, SJS/TEN, and TEN (n = 2,591, n = 502, and n = 564,

73 In SJS/TEN, HLA class I-restricted oligoclonal CD8(+) T-cel

74 14 +/- 0.22 logMAR post-PROSE (P = .0007) in SJS patients.

75 0.05) comparing between the moderate (15% in SJS, 0% in TEN) and severe groups (20% in SJS, 33% in TE

76 in SJS, 0% in TEN) and severe groups (20% in SJS, 33% in TEN).

77 number of cases with mild involvement (5% in SJS, 42% in TEN, p = 0.01), while no statistically signi

78 rce of elevated enzymes, including MMP-9, in SJS and OCP tears.

79 nt of cytotoxic CD8+ T cells into skin as in SJS/TEN, yet a pro-Treg signature as in MDE.

80 A specific protocol for acute ocular care in SJS/TEN, including aggressive use of AMT, was highly suc

81 CD3(+) +CD4(+) T(h) 1 or CD3(+) +NK cells in SJS-TEN, CD3(+) +CD4(+) T(h) 1+NK cells in MPE and CD3(+

82 n and its associated ocular complications in SJS/TEN.

83 three SJS/TEN patients and was confirmed in SJS/TEN tears and eyelid margins by ELISA and IHC, respe

84 The TIMP-1 levels were decreased in SJS and OCP patients when compared with those in control

85 used approaches to identify culprit drugs in SJS/TEN are associated with overlabeling patients allerg

86 9-to-TIMP-1 ratios were markedly elevated in SJS and OCP tears (SJS>OCP) when compared with those of

87 scoring system of chronic ocular features in SJS/TEN sequelae is a useful tool to grade all levels of

88 for improving visual acuity and function in SJS patients who failed conventional treatment.

89 MMP9 expression was significantly higher in SJS/TEN skin (70.6%) than in healthy control skin (0%) (

90 The MMP activity was highest in SJS patients, whereas OCP patients and controls showed l

91 ut their effects on systemic inflammation in SJS/TEN.

92 Ocular involvement in SJS, TEN, and Overlap syndrome is common and the ocular

93 ivity are a putative pathogenic mechanism in SJS/TEN, which is limited by etanercept.

94 wann cell physiology and suggest that PNH in SJS originates distally from synergistic actions of peri

95 Serum inflammatory profiles in SJS/TEN patients were notably characterized by massive u

96 cts on the systemic inflammatory response in SJS/TEN, which may not correlate with clinical outcome d

97 l aspects of the meibomian gland are seen in SJS.

98 th MPO, but it did not reach significance in SJS patients.

99 nd MPO levels were elevated significantly in SJS and OCP tears (SJS>OCP) when compared with controls.

100 d and nonexpanded cytotoxic CD8+ skin TRM in SJS/TEN.

101 were 69 cases of PPI-related DHR, including SJS/TEN (n=27) and DRESS (n=10).

102 d specialist-adjudicated allopurinol-induced SJS/TEN or DRESS (collectively allopurinol-induced sever

103 eactive T cells in patients with ALP-induced SJS or TEN and, in particular, there are no reports exam

104 BMCs isolated from both resolved ALP-induced SJS/TEN cases and drug-naive healthy donors, we show tha

105 notypic profile in both resolved ALP-induced SJS/TEN cases and drug-naive healthy donors.

106 between HLA-B*1502 and carbamazepine-induced SJS and TEN and that reported sufficient data for calcul

107 HLA-B*1502 allele and carbamazepine-induced SJS and TEN in Han-Chinese, Thai, and Malaysian populati

108 between HLA-B*1502 and carbamazepine-induced SJS and TEN was 79.84 (95% CI, 28.45-224.06).

109 he primary outcome was carbamazepine-induced SJS and TEN.

110 ocytes (CTLs) from patients with CBZ-induced SJS/TEN and analyzed the interaction between HLA-B and C

111 al correlations for CBZ-related drug-induced SJS/TEN.

112 e examine 6 cohorts including 25 ICI-induced SJS/TEN patients and conduct single-cell RNA sequencing

113 temic corticosteroids treatment, ICI-induced SJS/TEN patients treated with biologic TNF blockade show

114 iage as a risk factor for nevirapine-induced SJS/TEN in a Malawian HIV cohort.

115 Patients who survive the initial SJS/TEN episodes are affected by various sequelae.

116 son syndrome and toxic epidermal necrolysis (SJS/TEN) and drug reaction with eosinophilia and systemi

117 Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and drug reaction with eosinophilia and systemi

118 son Syndrome and Toxic Epidermal Necrolysis (SJS/TEN) are part of a disease continuum of vesiculobull

119 son syndrome and toxic epidermal necrolysis (SJS/TEN) are rare but severe adverse reactions with high

120 son syndrome and toxic epidermal necrolysis (SJS/TEN) are severe cutaneous adverse drug reactions cha

121 nson syndrome or toxic epidermal necrolysis (SJS/TEN) group, whereas glaucoma was found not to signif

122 son syndrome and toxic epidermal necrolysis (SJS/TEN) is a rare but life-threatening cutaneous drug r

123 Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe hypersensitivity reaction.

124 Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is known to cause multiple end-organ complicati

125 son syndrome and toxic epidermal necrolysis (SJS/TEN), although a detailed description is lacking in

126 ohnson syndrome, toxic epidermal necrolysis (SJS/TEN), and drug reaction with eosinophilia and system

127 Johnson syndrome/toxic epidermal necrolysis (SJS/TEN), hindering continuous ICI therapy.

128 son syndrome and toxic epidermal necrolysis (SJS/TEN), which are the most severe types of drug hypers

129 Johnson syndrome/toxic epidermal necrolysis (SJS/TEN).

130 SJS/TEN were identified and matched with non-SJS/TEN participants by age, sex, and Charlson Comorbidi

131 gression model showed that compared with non-SJS/TEN participants, patients with SJS/TEN had higher r

132 ty-five patients (50 eyes) with acute ocular SJS who presented within 4 weeks of onset of symptoms we

133 able ocular surface in cases of acute ocular SJS.

134 red eyes of 200 patients with chronic ocular SJS/toxic epidermal necrolysis (TEN) were included in th

135 To date, ocular SJS/TEN risk altering alleles have not been widely inves

136 The use of AMTs in severe ocular SJS/TEN greatly mitigates long-term complications and im

137 43 patients underwent AMT for severe ocular SJS/TEN.

138 Overall, 40% of SJS and 75% of TEN patients had acute ocular surface inf

139 While cases of SJS and TEN caused by glaucoma eye drops are documented,

140 ically and histologically confirmed cases of SJS/TEN overlap and TEN.

141 opriate and useful" for documenting cases of SJS/TEN, making it more reliable and valuable for future

142 skeletal disease phenotype characteristic of SJS patients.

143 he medical record revealed that diagnosis of SJS preceded that of ECT.

144 l observational study on the epidemiology of SJS/TEN contributes to the understanding of this still u

145 e observational study on the epidemiology of SJS/TEN using data from the UK-based Clinical Practice R

146 Demographics, etiology of SJS/TEN, age at treatment milestones, best-corrected vis

147 32Y mutation as the sole causative factor of SJS in the human family harboring this alteration and im

148 h 2015, 36 (4.8%) had associated features of SJS/TEN.

149 -expected rate in patients with a history of SJS.

150 ary 1992 and December 2016 with a history of SJS/TEN were reviewed.

151 luded 86 patients (167 eyes) with history of SJS/TEN who underwent PROSE treatment from January 1, 20

152 ed 35 males and 51 females with a history of SJS/TENS; median age was 36 years.

153 By contrast, the incidence of SJS/TEN with SOC in European countries is relatively low

154 The mean estimated incidences of SJS, SJS/TEN, and TEN were 9.2, 1.6, and 1.9 per million

155 gement of the acute ocular manifestations of SJS and TEN.

156 bserved in the keratinized eyelid margins of SJS/TEN to validate the presence of neutrophils.

157 We therefore studied a mouse model of SJS to determine whether a role for perlecan in these fu

158 gth of stay, comorbidities, and mortality of SJS and TEN in US adults.

159 a quality based on an international panel of SJS/TEN experts who performed a Delphi consensus-buildin

160 n suggestive of resorption among patients of SJS was 36.7 months and among patients of chemical injur

161 o the eyes and eyelids in the acute phase of SJS can prevent the devastating scarring and visual prob

162 riggered inflammation during early phases of SJS/TEN.

163 the chondrodystrophic myotonia phenotype of SJS is unknown.

164 ging, the severe, chronic ocular problems of SJS can be at least partially alleviated with autologous

165 he systemic and cutaneous immune profiles of SJS/TEN patients and to investigate whether/how intraven

166 ality findings to improve prognostication of SJS/TEN.

167 Initiation and progression of SJS/TEN keratinocyte necroptosis appear to involve a cas

168 ere associated with more than one-quarter of SJS/TEN cases described worldwide, and sulfonamide antib

169 After remission of SJS/TEN, a complete ENT mucosal healing occurred in 36 p

170 The OR for absolute risk of SJS/TEN associated with carriage of HLA-C*04:01 was 5.17

171 and Asian patients were at a 2-fold risk of SJS/TEN when compared with white patients.

172 mprovement, in eyes with chronic sequelae of SJS.

173 in patients with chronic ocular sequelae of SJS.

174 or dyspnea is observed at the acute stage of SJS/TEN.

175 Survivors of SJS/TEN were identified and matched with non-SJS/TEN par

176 le of these alleles in the susceptibility of SJS/TEN with or without severe ocular complications in p

177 rted therapeutic target for the treatment of SJS/TEN.

178 ture and may represent a distinct variant of SJS/TEN.

179 ong signal for an increased risk of DRESS or SJS requires particular vigilance in telaprevir-treated

180 (SJS), toxic epidermal necrolysis (TEN), or SJS/TEN was performed.

181 eview of published cases of photodistributed SJS/TEN.

182 toprosthesis surgery in dry keratinized post-SJS eyes.

183 SJS, SJS/TEN, and TEN pose a substantial health care burden.

184 Clinical Modification codes to identify SJS, SJS/TEN, and TEN (n = 2,591, n = 502, and n = 564, respe

185 The mean estimated incidences of SJS, SJS/TEN, and TEN were 9.2, 1.6, and 1.9 per million adul

186 In this cohort study, SJS/TEN had a lasting association with cardiovascular fu

187 he relatively mild Schwartz-Jampel syndrome (SJS) and severe neonatal lethal dyssegmental dysplasia,

188 Schwartz-Jampel syndrome (SJS) is a form of PNH that is due to hypomorphic mutatio

189 developed to model Schwartz-Jampel syndrome (SJS), a skeletal disease resulting from decreased perlec

190 een HLA-B*1502 and Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) among carbamaz

191 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threa

192 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threa

193 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but l

194 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but l

195 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but p

196 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe dru

197 Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) cause diffuse

198 f photodistributed Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) have been infr

199 (SCAR), including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), could be life

200 is (EN), including Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), varies across

201 stering conditions Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).

202 reactions such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).

203 crolysis (TEN) and Stevens-Johnson Syndrome (SJS) are rare, acute, life-threatening dermatologic diso

204 eatments for acute Stevens-Johnson syndrome (SJS) as well as the emerging treatment options for patie

205 12 laminae) to the Stevens-Johnson syndrome (SJS) group.

206 To report Stevens-Johnson syndrome (SJS) in a patient with acute pneumonia secondary to SARS

207 By comparison, Stevens-Johnson syndrome (SJS) is a life-threatening severe cutaneous adverse reac

208 injury (DILI) and Stevens-Johnson syndrome (SJS) or toxic epidermal necrosis (TEN) is even rarer and

209 Three cases of Stevens-Johnson Syndrome (SJS), and 11 cases of Drug Reaction with Eosinophilia an

210 Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and graft-versus

211 f SCARs, including Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), drug rash with e

212 ed by PPI, such as Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or drug rash wit

213 for severe ocular Stevens-Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS/TEN was p

214 male patient with Stevens-Johnson syndrome (SJS), which was suspected to be caused by treatment with

215 ith a diagnosis of Stevens-Johnson syndrome (SJS).

216 pine (CBZ)-induced Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) as a model to stud

217 elevated significantly in SJS and OCP tears (SJS>OCP) when compared with controls.

218 were markedly elevated in SJS and OCP tears (SJS>OCP) when compared with those of controls.

219 Unlike DDSH, the SJS mutations result in different forms of perlecan in r

220 ury group and 8 out of the 12 laminae in the SJS group.

221 Fatality was more frequent in the SJS/TEN group.

222 IL-36gamma (p < .01) was expressed in three SJS/TEN patients and was confirmed in SJS/TEN tears and

223 descriptively analyzed potential culprits to SJS/TEN, patients' allergy lists, and currently used app

224 es of highly cited manuscripts pertaining to SJS/TEN.

225 th chronic ocular surface disease related to SJS/TEN and results in significant improvement in vision

226 th chronic ocular surface disease related to SJS/TEN, PROSE treatment offers sustained and significan

227 ducation, and long-term sequelae relevant to SJS/TEN.

228 ggesting that this was a process specific to SJS/TEN.

229 Among 551 validated SJS/TEN patients, we calculated an incidence rate of 5.7

230 h mortality after hospital discharge (TEN vs SJS: AHR, 0.95; 95% CI, 0.60-1.47), but acute complicati

231 95% CI, 1.24-2.64), and EN severity (TEN vs SJS: AHR, 2.14; 95% CI, 1.49-3.07).

232 stern and South East Asian populations where SJS/TEN is prevalent, numerous human leukocyte antigen (

233 for Sick Children between 2001 and 2011 with SJS, TEN, and Overlap syndrome were reviewed.

234 A total of 212 adults with SJS/TEN were included between January 1, 2015, and Decem

235 1 patients (81 children and 320 adults) with SJS who presented with chronic lid-related keratopathy b

236 ssociation of HLA -A, -B and -C alleles with SJS/TEN in 33 patients residing in the UK with age match

237 harboring only the mutation associated with SJS, displayed a mild phenotype, inconsistent with SJS.

238 DILI associated with SJS/TEN is rare and associated with a high death rate, p

239 ed proportion of antibiotics associated with SJS/TEN was 28% (95% CI, 24%-33%), with moderate certain

240 alence of single antibiotics associated with SJS/TEN.

241 ients who developed DILI in association with SJS/TEN from a registry of DILI patients from a single c

242 rience of DILI occurring in association with SJS/TEN including the etiologic agents, clinical and bio

243 HLA class I associations with SJS/TEN and drug reaction with eosinophilia and systemic

244 h mild ocular involvement when compared with SJS, but no significant difference between the number of

245 n the US evaluated 121 adults diagnosed with SJS/TEN by inpatient consultive dermatologists between J

246 preserving and improving vision in eyes with SJS-induced lid-related keratopathy.

247 study recruited 60 patients (120 eyes) with SJS and chronic ocular sequelae.

248 sharing the histopathological features with SJS.

249 isplayed a mild phenotype, inconsistent with SJS.

250 Thus, it is crucial that all patients with SJS be evaluated by an ophthalmologist familiar with the

251 Heterozygous mutations in two patients with SJS either produced truncated perlecan that lacked domai

252 or Japanese race/ethnicity, no patients with SJS or TEN were carriers of the HLA-B*1502 allele.

253 onia and chondrodysplasia, and patients with SJS survive.

254 s study included 30 eyes of 30 patients with SJS-induced dry keratinized ocular surfaces; the patient

255 of perlecan in three unrelated patients with SJS.

256 l was noted to be higher among patients with SJS.

257 with non-SJS/TEN participants, patients with SJS/TEN had higher risks of cardiovascular morbidity (CV

258 Retrospective study of 49 patients with SJS/TEN hospitalized in a referral care center from 2005

259 NA chimera on CTL responses in patients with SJS/TEN or GVHD.

260 systemic inflammation seen in patients with SJS/TEN or GVHD.

261 All patients with SJS/TEN with chronic (more than 1 year) ocular sequelae

262 sitivity, such as that seen in patients with SJS/TEN, as well as the alloreactivity seen in patients

263 In patients with SJS/TEN, higher mortality was associated with old age an

264 We included 16 patients with SJS/TEN, treated with high-dose IVIG (n = 8), CSA (n = 4

265 anxiety appear to be common in patients with SJS/TEN, with implications for health and well-being.

266 re, counseling, and support to patients with SJS/TEN.