Previous studies have demonstrated a strong association between carbamazepine（CBZ）-induced Stevens-Johnson syndrome（SJS） and HLA-B 1502 in Han Chinese. Here, we extended the study of HLA-B 1502 susceptibility to t...Previous studies have demonstrated a strong association between carbamazepine（CBZ）-induced Stevens-Johnson syndrome（SJS） and HLA-B 1502 in Han Chinese. Here, we extended the study of HLA-B 1502 susceptibility to two different antiepileptic drugs, oxcarbazepine（OXC） and phenobabital（PB）. In addition, we genotyped HLA-B 1511 in a case of CBZ-induced SJS with genotype negative for HLA-B 1502. The presence of HLA-B 1502 was determined using polymerase chain reaction with sequence-specific primers（PCR-SSP）. Moreover, we genotyped HLA-B 1502 in 17 cases of antiepileptic drugs（AEDs）-induced cutaneous adverse drug reactions（cADRs）, in comparison with AEDs-tolerant（n=32） and normal controls（n=38） in the central region of China. The data showed that HLA-B 1502 was positive in 5 of 6 cases of AEDs-induced SJS（4 CBZ, 1 OXC and 1 PB）, which was significantly more frequent than AEDs-tolerant（2/32, 18 CBZ, 6 PB and 8 OXC） and normal controls（3/38）. Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B 1502 with AEDs-induced SJS was 6.25（95% CI： 1.06–36.74） and 4.86（95% CI： 1.01–23.47）. The sensitivity and specificity of HLA-B 1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B 1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B 1502 was not found in 11 children with maculopapular exanthema（MPE）（n=9） and hypersensitivity syndrome（HSS）（n=2）. However, we also found one case of CBZ-induced SJS who was negative for HLA-B 1502 but carried HLA-B 1511. It was suggested that the association between the CBZ-induced SJS and HLA-B 1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B 1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B 1502.展开更多
Background： Stevens-Johnson syndrome （SJS） and toxic epidennal necrolysis （TEN） are life-threatening diseases with high mortality rates. This study was designed to analyze the pathogenic factors, clinical manifes...Background： Stevens-Johnson syndrome （SJS） and toxic epidennal necrolysis （TEN） are life-threatening diseases with high mortality rates. This study was designed to analyze the pathogenic factors, clinical manifestations, complications, treatment, and prognosis of SJS/TEN and to explore the differences between surviving and deceased patients. Methods： SJS/TEN patients admitted to Beijing Friendship Hospital from January 2006 to December 2015 were included in the study. Patients＇ data were retrospectively analyzed. Comparative studies were performed on the survival group and the deceased group, and Fisher＇s exact probability test was used for statistical analysis. Results： Among the 88 patients included, 40 （45.5%） were male with a mean age of 45 - 18 years. Forty-eight （54.5%） had SJS, 34 （38.6%） had SJS/TEN, and 6 （6.8%） had TEN. Fifty-three （60.2%） cases were caused by medications, mainly antibiotics （n = 24） followed by traditional Chinese medicines 97 - 7）. Forty-two cases （47.7%） developed visceral damage. Eighty-two patients improved or recovered and were discharged from hospital, and six patients died. Comparative studies on the survival group and the deceased group showed that the presence of malignant tumor （Z2 = 27.969, P 〈 0.001）, connective tissue diseases （x^2 - 9.187, P = 0.002）, previous abnormal liver/kidney functions （x^2 = 6.006, P = 0.014）, heart rate 〉100 times/rain （x^2 = 6.347, P = 0.012）, detached skin area 〉20% （x^2 = 5.594, P = 0.018）, concurrent mucosal involvement at the mouth, eyes, and external genitals （Z2 = 4.945, P = 0.026）, subsequent accompanying liver/kidney damage （x^ = 11.839, P = 0.001, and x^2 = 36.302, P 〈 0.00 l, respectively）, and SCORTEN score 〉2 （x^2 = 37.148, P 〈 0.001 ） increased the risk of death. Conclusions： SJS/TEN is mainly caused by medications, and nearly half of patients develop visceral damage. Multiple factors increase the mortality risk.展开更多
基金supported by a grant from Clinical Scientific Research of Wuhan Sanitary Bureau(No.WX11C26)research fund from Janssen Research Council of China(No.JRCC2011-01)
文摘Previous studies have demonstrated a strong association between carbamazepine（CBZ）-induced Stevens-Johnson syndrome（SJS） and HLA-B 1502 in Han Chinese. Here, we extended the study of HLA-B 1502 susceptibility to two different antiepileptic drugs, oxcarbazepine（OXC） and phenobabital（PB）. In addition, we genotyped HLA-B 1511 in a case of CBZ-induced SJS with genotype negative for HLA-B 1502. The presence of HLA-B 1502 was determined using polymerase chain reaction with sequence-specific primers（PCR-SSP）. Moreover, we genotyped HLA-B 1502 in 17 cases of antiepileptic drugs（AEDs）-induced cutaneous adverse drug reactions（cADRs）, in comparison with AEDs-tolerant（n=32） and normal controls（n=38） in the central region of China. The data showed that HLA-B 1502 was positive in 5 of 6 cases of AEDs-induced SJS（4 CBZ, 1 OXC and 1 PB）, which was significantly more frequent than AEDs-tolerant（2/32, 18 CBZ, 6 PB and 8 OXC） and normal controls（3/38）. Compared with AEDs-tolerant and normal controls, the OR for patients carrying the HLA-B 1502 with AEDs-induced SJS was 6.25（95% CI： 1.06–36.74） and 4.86（95% CI： 1.01–23.47）. The sensitivity and specificity of HLA-B 1502 for prediction of AEDs-induced SJS were 71.4%. The sensitivity and specificity of HLA-B 1502 for prediction of CBZ-induced SJS were 60% and 94%. HLA-B 1502 was not found in 11 children with maculopapular exanthema（MPE）（n=9） and hypersensitivity syndrome（HSS）（n=2）. However, we also found one case of CBZ-induced SJS who was negative for HLA-B 1502 but carried HLA-B 1511. It was suggested that the association between the CBZ-induced SJS and HLA-B 1502 allele in Han Chinese children can extend to other aromatic AEDs including OXC and PB related SJS. HLA-B 1511 may be a risk factor for some patients with CBZ-induced SJS negative for HLA-B 1502.
文摘Background： Stevens-Johnson syndrome （SJS） and toxic epidennal necrolysis （TEN） are life-threatening diseases with high mortality rates. This study was designed to analyze the pathogenic factors, clinical manifestations, complications, treatment, and prognosis of SJS/TEN and to explore the differences between surviving and deceased patients. Methods： SJS/TEN patients admitted to Beijing Friendship Hospital from January 2006 to December 2015 were included in the study. Patients＇ data were retrospectively analyzed. Comparative studies were performed on the survival group and the deceased group, and Fisher＇s exact probability test was used for statistical analysis. Results： Among the 88 patients included, 40 （45.5%） were male with a mean age of 45 - 18 years. Forty-eight （54.5%） had SJS, 34 （38.6%） had SJS/TEN, and 6 （6.8%） had TEN. Fifty-three （60.2%） cases were caused by medications, mainly antibiotics （n = 24） followed by traditional Chinese medicines 97 - 7）. Forty-two cases （47.7%） developed visceral damage. Eighty-two patients improved or recovered and were discharged from hospital, and six patients died. Comparative studies on the survival group and the deceased group showed that the presence of malignant tumor （Z2 = 27.969, P 〈 0.001）, connective tissue diseases （x^2 - 9.187, P = 0.002）, previous abnormal liver/kidney functions （x^2 = 6.006, P = 0.014）, heart rate 〉100 times/rain （x^2 = 6.347, P = 0.012）, detached skin area 〉20% （x^2 = 5.594, P = 0.018）, concurrent mucosal involvement at the mouth, eyes, and external genitals （Z2 = 4.945, P = 0.026）, subsequent accompanying liver/kidney damage （x^ = 11.839, P = 0.001, and x^2 = 36.302, P 〈 0.00 l, respectively）, and SCORTEN score 〉2 （x^2 = 37.148, P 〈 0.001 ） increased the risk of death. Conclusions： SJS/TEN is mainly caused by medications, and nearly half of patients develop visceral damage. Multiple factors increase the mortality risk.
