Sjs

SJS
  • 文章类型: Journal Article
    严重的皮肤不良反应(SCAR)是罕见的,但危及生命,抗生素是主要原因。这项来自单一中心的回顾性研究旨在分析罪魁祸首药物,抗生素诱导的SCAR的临床特征和治疗结果。
    我们分析了2013年1月至2024年1月间中国某三甲医院抗生素诱发的SCAR病例,包括史蒂文-约翰逊综合征(SJS)或史蒂文斯-约翰逊综合征-中毒性表皮坏死松解症(SJS-TEN)重叠,中毒性表皮坏死松解症(TEN),药物反应伴嗜酸性粒细胞增多和全身症状(DRESS)和急性全身性发疹性脓疱病(AGEP)。对人口特征的描述性分析,临床表现,进行治疗和预后。
    在354例SCAR中,纳入63例经过验证的抗生素相关病例。头孢菌素(31.7%),青霉素(25.4%),喹诺酮类药物(19.0%)是SCAR最常见的触发因素。总的来说,肝脏(50.8%),肺(31.7%),在SCAR病例中,肾脏(23.8%)是最常见的受累器官。SJS/SJS-TEN重叠组8例(28.6%)和TEN组8例(80.0%)接受糖皮质激素和IVIG联合治疗。由青霉素或头孢菌素引起的SCAR患者可以接受替代疗法,例如林可胺,喹诺酮类药物,还有四环素.TEN组死亡率最高,为20.0%,其次是SJS/SJS-TEN重叠组(7.1%),在DRESS和AGEP组中没有观察到死亡。
    识别罪魁祸首抗生素和应用替代抗生素疗法对于抗生素诱导的SCAR的管理至关重要。如果复杂的潜在疾病和并发症,如高龄,癌症和肺炎与SCAR并存,患者可能面临更大的死亡风险。
    UNASSIGNED: Severe cutaneous adverse reactions (SCARs) are rare but life-threatening, with antibiotics being the main cause. This retrospective study from a single center was designed to analyze the culprit drugs, clinical features and treatment outcomes of antibiotic-induced SCARs.
    UNASSIGNED: We analyzed cases of antibiotic-induced SCARs in a tertiary hospital in China between January 2013 and January 2024, including Steven-Johnson syndrome (SJS) or Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Descriptive analysis of the demographic characteristics, clinical manifestations, treatment and prognosis were carried out.
    UNASSIGNED: Among 354 cases of SCARs, 63 validated antibiotic-related cases were included. Cephalosporins (31.7%), penicillins (25.4%), and quinolones (19.0%) were the most common triggers for SCARs. Overall, liver (50.8%), lungs (31.7%), and kidneys (23.8%) were the most frequently affected organ in SCARs cases. Eight patients (28.6%) in the SJS/SJS-TEN overlap group and 8 patients (80.0%) in the TEN group received combination therapy of corticosteroids and IVIG. Patients with SCARs caused by penicillins or cephalosporins could receive alternative treatments such as lincomamides, quinolones, and tetracyclines. The mortality rate in the TEN group was the highest at 20.0%, followed by the SJS/SJS-TEN overlap group (7.1%), and no deaths were observed in the DRESS and AGEP groups.
    UNASSIGNED: The identification of the culprit antibiotics and the application of alternative antibiotic therapies are crucial for the management of antibiotic-induced SCARs. If complicated underlying conditions and complications like advanced age, cancer and pneumonia coexist with SCARs, patients might be more at risk for mortality.
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  • 文章类型: Journal Article
    Stevens-Johnson综合征(SJS)和中毒性表皮坏死松解症(TEN)是严重的皮肤不良反应,因其死亡率高而备受关注。SJS和TEN的预后使用SCORTEN(TEN的SCORe)进行广泛评估。虽然,这是一个非常有用的量表,预测能力仍然是可变的。
    本研究旨在评估SJS和TEN的临床病因和结局,并评估SCORTEN在评估南印度人群预后中的有效性。
    这项前瞻性观察性研究是在皮肤科进行的,2016年1月至2017年6月,三级医院的性病和麻风病。详细的历史,检查结果,记录治疗和SCORTEN评分.在入院第1、3和5天评估SCORTEN预测死亡率的准确性。
    其他药物反应中SJS/TEN的发生率为29.5%。受影响最常见的年龄组是30-49岁(41.1%),男性占优势(76.5%)。患者的年龄范围为6岁和67岁。TEN(64.7%)是主要的光谱,其次是SJS和SJS-TEN重叠,各占17.6%。抗惊厥药(47%)是最常见的致病药物,其次是镇痛药(35%)和抗生素(11%)。SCORTEN的有效性在第1、3和5天是相同的。在所有三天的实际死亡率和预测死亡率之间存在良好的一致性。本研究记录的死亡率为17.6%(3例)。我们的研究中有3名患者(17.6%)死亡。所有幸存者的得分为4分或更低。在SJS中,预测死亡率分别为0.417、1.836和2.574,观察死亡率分别为0、2和1。SJS-TEN重叠,分别为十。单日SCORTEN分析,发现第1天,第3天或第5天与系列分析一样有用.
    SCORTEN对SJS-TEN重叠患者的死亡率进行了重大估计,而它高估了TEN患者的死亡率。在现有的SCORTEN中,血液尿素氮(BUN)升高的个体得分增加,并纳入了新的参数,例如肝酶升高,血小板减少症,和肺浸润有助于为南印度人口提出改良的SCORTEN。更大规模的进一步研究,需要验证我们提出的修改后的SCORTEN。
    UNASSIGNED: Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe cutaneous adverse reactions of major concern because of its high mortality. The prognosis of SJS and TEN is widely assessed with SCORTEN (SCORe of TEN). Although, it is a largely useful scale, the predictive ability is still variable.
    UNASSIGNED: This study was conducted to assess the clinicoetiological profile and outcome of SJS and TEN and to evaluate the validity of SCORTEN in assessing the prognosis in South Indian population.
    UNASSIGNED: This prospective observational study was conducted in the Department of Dermatology, Venereology and Leprosy in a Tertiary care hospital from January 2016 to June 2017. Detailed history, examination findings, treatment and SCORTEN scores were recorded. SCORTEN\'s accuracy in predicting the mortality was assessed on day 1, 3 and 5 of admission.
    UNASSIGNED: The incidence of SJS/TEN among other drug reactions was 29.5%. The most common age group affected was 30-49years (41.1%), with male preponderance (76.5%). The age range of patients was 6 and 67 years. TEN (64.7%) was the predominant spectrum followed by SJS and SJS-TEN overlap in 17.6% each. Anticonvulsants (47%) were the commonest causative drug, followed by analgesics (35%) and antibiotics (11%). The validity of SCORTEN was the same on days 1, 3, and 5. There was good agreement between the actual and predicted mortality on all three days. A mortality of 17.6% (3 cases) was recorded in this study. Three patients (17.6%) died in our study. All survivors had a score of 4 or less. The predicted mortalities were 0.417, 1.836, and 2.574 and the observed mortalities were 0, 2, and 1 in SJS, SJS-TEN overlap, and TEN respectively. Analysis of SCORTEN on a single day, either day 1, 3, or 5 was found to be as useful as the serial analysis.
    UNASSIGNED: SCORTEN gave a significant estimation of mortality in SJS-TEN overlap patients, whereas it overestimated mortality in TEN patients. An increase in individual scores for the elevation of blood urea nitrogen (BUN) in existing SCORTEN and the inclusion of new parameters like raised liver enzymes, thrombocytopenia, and pulmonary infiltrates aided in proposing a modified SCORTEN for the South Indian population. Further studies on a larger scale, are needed to validate the modified SCORTEN proposed by us.
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  • 文章类型: Journal Article
    OBJECTIVE: This study aimed to examine the ultrastructural features of the lacrimal glands of patients with severe dry eye due to Stevens-Johnson syndrome (SJS).
    METHODS: Biopsies form orbital lobes of six lacrimal glands obtained from fresh body donors (n=3) and patients with SJS (n=3; absolute tear deficiency; 2-6 months of disease duration) were examined using transmission electron microscopy (TEM).
    RESULTS: On TEM, normal lacrimal glandular tissue shows columnar acinar epithelial cells containing basally located rounded or oval euchromatic nuclei with cytoplasmic electron dense and -moderately electron-dense secretory granules. Histologically, the lacrimal gland biopsies showed varied degree of acinar atrophy with lymphocytic infiltration in SJS patients. While on TEM, the lacrimal glands in SJS patients showed homogenous appearance of the nuclear chromatin of acinar cells with no differentiation into hetero-or euchromatin and loss of nucleoli. The cytoplasm lacked any electron dense material and showed significant loss of endoplasmic reticulum, mitochondria and Golgi bodies. The myoepithelial cells shared similar characteristics as in acinar epithelial cells along with loss of spindle shaped processes.
    CONCLUSIONS: Transmission electron microscopy revealed ultrastructural changes in terms of nuclear composition, secretory vesicles, lysosomal vacuolation and myoepithelial cell distribution in the lacrimal glands of SJS patients.
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  • 文章类型: Journal Article
    HIV-infected patients have a higher risk of developing cutaneous reactions to drugs than the general population. Severe cutaneous adverse reactions (SCARs) are not uncommon in patients taking antiretroviral therapy (HAART]. To evaluate HLA class I and II allele frequencies in HIV patients on HAART who develop SCARs due to nevirapine (NVP] or efavirenz (EFZ] containing regime and compare this genotype composition with HAART tolerant patients and healthy organ donors. A case-control study for 4 years was conducted with four subsets of patients hailing from north-east India:Cohort 1- HIV seropositive patients who developed SCARs due to EFZ (n = 8];Cohort 2 - HIV seropositive patients who developed SCARs due to NVP (n = 15]; Cohort 3 -HIV seropositive NVP/EFZ-tolerant patients (n = 18]; Cohort 4 - Healthy HIV seronegative organ donors (n = 169].Cohort 3 & 4 acted as control-group. These patients were genotyped for the HLA-A, HLA-B, HLA-C, HLA-DRB1, HLA-DQB1, and HLA-DPB1 by a sequence-based HLA typing method. HLA-DRB1*03:01 allele revealed a significant association with EFZ regimen-induced SCARs in 62.5% patients compared with only 5.56% observed in HAART-tolerant patients and 4.14% in healthy organ. HLA-B*3505was found to be significantly associated with NVP induced SCARs. We found significant novel association of HLA-DRB1*03:01 with EFZ induced SCARs in North-East Indian HIV patients. Thus, HLA-DRB*03:01 may be useful as a genetic marker to avoid EFZ induced serious cutaneous rashes. The molecular HLA characterization of these alleles may provide a novel insight into the immunological basis of the antiretroviral drug reactions.
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  • 文章类型: Journal Article
    To detect the immunohistochemical changes in the main lacrimal glands of patients suffering from chronic ocular sequelae of Stevens-Johnson syndrome (SJS).
    Histological sections of biopsies from the lacrimal gland of three chronic SJS patients (mean age, 33 years; 2 males) with severe dry eye disease (Schirmer = 0 mm) were assessed using double immunofluorescence techniques. Changes in the expression of secretory proteins lactoferrin (Lf), lysozyme (Ly), aquaporin 5 (AQP5), S-100, and early apoptosis marker (Annexin V) were studied.
    Different morphological expressions of secretory proteins were present in the three samples. One sample had maintained the immunoreactivity for Lf, Ly, S-100, similar to healthy controls. Two samples had significantly reduced immunoreactivity for anti-Lf, anti-Ly, and S-100, the weakest being in the sample with distorted lobular architecture and mild interlobular fibrosis. AQP5 had a distinct vesicular intracytoplasmic immunoreactivity suggesting defective trafficking and integration of the protein to the apical membrane. There was no S-100 immunostaining in the acinar or ductal epithelium, whereas interstitial nerve fibers scattered in the periacinar region showed reduced immunoreactivity for S-100. There was strong Annexin V immunoreactivity in the nuclei of epithelial cells in the majority of acinar and ductal epithelia of all the samples, with distorted nuclear morphology in one sample.
    Defective trafficking of AQP5 and variable expression of Ly, Lf, S-100 are the notable findings in the lacrimal glands of chronic SJS patients along with signs of early apoptosis. It suggests that the palpebral lobe of the lacrimal gland is involved in the pathological processes occurring in the conjunctiva of SJS patients.
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  • 文章类型: Journal Article
    Multiple studies have been published on toxic epidermal necrolysis (TEN) and Stevens-Johnsen syndrome (SJS). Nursing care is an important part of the treatment of TEN patients. Unfortunately, limited information on nursing in TEN/SJS patients has been published in the current literature. Nursing research is needed to improve the complex nursing care required for these rare patients. Therefore, the objective was to assess nursing problems in TEN patients in a burn centre setting over a 30-year period.
    The data for this study were gathered retrospectively from nursing records of all patients with TEN/SJS admitted to Burn Centre Rotterdam between January 1987 and December 2016. Dutch burn centres were recently accepted as expertise centres for TEN patients. Nursing problems were classified using the classification of nursing problems of the Dutch Nursing Society.
    A total of 69 patients were admitted with SJS/TEN. Fifty-nine patient files were available. The most frequently reported nursing problems (>20% of the patients) were wounds, threatened or disrupted vital functions, dehydration or fluid imbalance, pain, secretion problems and fever. Furthermore, TEN-specific nursing problems were documented, including oral mucosal lesions and ocular problems. The highest number of concomitant nursing problems occurred during the period between days three and 20 after onset of the disease and varied by nursing problem.
    The most frequently reported nursing problems involved physical functions, especially on days three to 20 after onset of the disease. With this knowledge, we can start nursing interventions early in the treatment, address problems at the first sign and inform patients and their families or relatives of these issues early in the disease process. A next step to improve nursing care for TEN patients is to acquire knowledge on the optimal interventions for nursing problems.
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  • 文章类型: Journal Article
    Antiepileptic drugs (AEDs) can cause hypersensitivity reactions during childhood. Studies report a wide clinical spectrum of reactions with AED use, ranging from a mild rash to severe cutaneous reactions.
    To determine the prevalence and clinical features of AED hypersensitivity reactions during childhood.
    Patients in our pediatric neurology clinic who were prescribed an AED for the first time between November 2015 and November 2016 were monitored and those who developed skin rash during this period were evaluated.
    A total of 570 patients were evaluated. The median age of the patients was 8.86 (interquartile range, 4.2-13.7) years, and 55.8% (318) of patients were male. The most frequently used AEDs were valproic acid (42%, n = 285) and carbamazepine (20.4%, n = 116). Hypersensitivity reactions to AEDs developed in 5.4% of patients. Of these patients, 71% (29) had cutaneous drug reactions and 29% (9) had severe cutaneous drug reactions; 61.3% (19) were using aromatic AEDs, and the leading suspected AED was carbamazepine (45.2%). Comparison of patients who did and did not develop AED hypersensitivity showed that hypersensitivity was more frequent among patients who were younger than 12 years, who used aromatic AEDs, or who used multiple AEDs. In addition, according to regression analysis results, aromatic AED use significantly increased the risk of AED hypersensitivity (P < .001).
    Although allergic reactions to AEDs are rare, they are of significance because they can cause life-threatening severe cutaneous drug reactions. Therefore, patients receiving AEDs, especially aromatic AEDs, must be monitored closely.
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