Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations ...Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations have been described. In addition to these noncutaneous manifestations, about 15% of patients have central neurological symptoms such as psychosis, convulsions and encephalopathy. Successful therapy is difficult but high- dose intravenous immunoglobulin (IVIg) has been reported to be a successful treatment. We describe a patient with scleromyxoedema who presented with novel central nervous system manifestations of chronic cognitive impairment and dementia (Folstein Mini Mental State test score 8/30), which improved within a week after treatment with high- dose IVIg, with full restoration (Folstein Mini Mental State test score 27/30) at 2 months.展开更多
目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据...目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据有无冠脉病变将患儿分为无冠脉病变组和冠脉病变组。对两组患者的发热时间、IVIG治疗时的病程时间、白细胞、血红蛋白、血小板、C反应蛋白、降钙素原、离子、肝及肾功能等临床资料进行分析。结果KD合并冠脉病变组患者血白细胞、血小板计数高于无冠脉病变组,而白蛋白、羟基酸脱氢酶浓度低于无冠脉病变组,差异均有统计学意义(P<0.05)。KD发热时间<8 d患儿的冠脉病变发生率低于发热时间>10 d的患儿,差异有统计学意义(P<0.05)。病程第5天前应用IVIG治疗患者冠脉病变发生率与病程第7天、第8天及10天后治疗患者比较,差异有统计学意义(P<0.05)。病程第5天及第6天应用IVIG治疗患者的冠脉病变发生率低于病程第10天后应用的患者,差异有统计学意义(P<0.05)。结论应用IVIG时间过晚,白细胞、血小板计数升高,发热时间较长是KD患儿并发冠脉病变的危险因素,而白蛋白、羟基酸脱氢酶浓度的降低也可作为KD并发冠脉病变的预测指标。热程少于8 d,病程7 d内应用IVIG治疗,可减少冠脉病变发生率。展开更多
目的:系统评价静脉注射免疫球蛋白(intravenous immunoglobulins, IVIG)治疗复发性流产(recurrent spontaneous abortion, RSA)的临床疗效。方法:检索Cochrane图书馆、PubMed、Embase、中国知网和维普数据库,查找有关IVIG治疗RSA的随机...目的:系统评价静脉注射免疫球蛋白(intravenous immunoglobulins, IVIG)治疗复发性流产(recurrent spontaneous abortion, RSA)的临床疗效。方法:检索Cochrane图书馆、PubMed、Embase、中国知网和维普数据库,查找有关IVIG治疗RSA的随机对照试验(randomized controlled trials, RCT),检索范围为建库至2021年12月。按照纳入排除标准,由两名作者独立进行文献筛选并提取所需信息,采用Review Manager 5.3软件对所提取的数据进行分析。结果:本研究纳入10篇RCT,共496名RSA患者。IVIG治疗组活产率高于安慰剂组(60.57% vs 55.2%),差异无统计学意义(RR = 0.88, 95% CI = 0.72~1.08, P = 0.23)。相对于原发性RSA患者,IVIG治疗对提高继发性RSA患者活产率有积极作用(RR = 1.05, 95% CI = 0.90~1.22, P = 0.03)。按照用药时机将RSA患者分为妊娠前与确定妊娠后IVIG治疗两个亚组进行分析,活产率在两种给药方案间无统计学差异(RR = 1.09, 95% CI (0.95~1.27), P = 0.06)。相比于妊娠后给药组(103/172 vs 101/168, RR = 1.00, 95% CI (0.84~1.18), P = 0.97),在妊娠前给予IVIG治疗RSA患者活产率更高(46/74 vs. 37/82;RR = 1.38, 95% CI (1.03~1.86), P = 0.03)。将原发性RSA患者分为妊娠前与妊娠后给予IVIG治疗两个亚组进行分析,活产率在两种方案之间无统计学差异(RR = 0.88, 95% CI = 0.71~1.07, P = 0.45)。将继发性RSA患者分为妊娠前与妊娠后给予IVIG治疗两个亚组进行分析,活产率在两种方案之间无统计学差异(RR = 1.24, 95% CI = 0.98~1.57, P = 0.94)。结论:IVIG治疗RSA疗效尚不确定。相对于原发性RSA,IVIG治疗对提高继发性RSA患者活产率有积极作用。RSA患者在妊娠前给予IVIG治疗中获益。对于继发性RSA患者,妊娠前与妊娠后给予IVIG治疗,活产率无统计学差异。展开更多
文摘Scleromyxoedema is a rare skin disease, characterized by deposition of acid mucopolysaccharides in the dermis. Although the disease primarily affects the skin, cardiovascular, renal and rheumatological manifestations have been described. In addition to these noncutaneous manifestations, about 15% of patients have central neurological symptoms such as psychosis, convulsions and encephalopathy. Successful therapy is difficult but high- dose intravenous immunoglobulin (IVIg) has been reported to be a successful treatment. We describe a patient with scleromyxoedema who presented with novel central nervous system manifestations of chronic cognitive impairment and dementia (Folstein Mini Mental State test score 8/30), which improved within a week after treatment with high- dose IVIg, with full restoration (Folstein Mini Mental State test score 27/30) at 2 months.
文摘目的探讨静脉注射人免疫球蛋白(intravenous infusion of human immunoglobulin,IVIG)对川崎病(Kawasaki disease,KD)患儿冠状动脉(冠脉)病变发生的影响。方法选择2019年1月至2022年5月在东莞市妇幼保健院住院确诊并治疗的KD患儿,根据有无冠脉病变将患儿分为无冠脉病变组和冠脉病变组。对两组患者的发热时间、IVIG治疗时的病程时间、白细胞、血红蛋白、血小板、C反应蛋白、降钙素原、离子、肝及肾功能等临床资料进行分析。结果KD合并冠脉病变组患者血白细胞、血小板计数高于无冠脉病变组,而白蛋白、羟基酸脱氢酶浓度低于无冠脉病变组,差异均有统计学意义(P<0.05)。KD发热时间<8 d患儿的冠脉病变发生率低于发热时间>10 d的患儿,差异有统计学意义(P<0.05)。病程第5天前应用IVIG治疗患者冠脉病变发生率与病程第7天、第8天及10天后治疗患者比较,差异有统计学意义(P<0.05)。病程第5天及第6天应用IVIG治疗患者的冠脉病变发生率低于病程第10天后应用的患者,差异有统计学意义(P<0.05)。结论应用IVIG时间过晚,白细胞、血小板计数升高,发热时间较长是KD患儿并发冠脉病变的危险因素,而白蛋白、羟基酸脱氢酶浓度的降低也可作为KD并发冠脉病变的预测指标。热程少于8 d,病程7 d内应用IVIG治疗,可减少冠脉病变发生率。
文摘目的:系统评价静脉注射免疫球蛋白(intravenous immunoglobulins, IVIG)治疗复发性流产(recurrent spontaneous abortion, RSA)的临床疗效。方法:检索Cochrane图书馆、PubMed、Embase、中国知网和维普数据库,查找有关IVIG治疗RSA的随机对照试验(randomized controlled trials, RCT),检索范围为建库至2021年12月。按照纳入排除标准,由两名作者独立进行文献筛选并提取所需信息,采用Review Manager 5.3软件对所提取的数据进行分析。结果:本研究纳入10篇RCT,共496名RSA患者。IVIG治疗组活产率高于安慰剂组(60.57% vs 55.2%),差异无统计学意义(RR = 0.88, 95% CI = 0.72~1.08, P = 0.23)。相对于原发性RSA患者,IVIG治疗对提高继发性RSA患者活产率有积极作用(RR = 1.05, 95% CI = 0.90~1.22, P = 0.03)。按照用药时机将RSA患者分为妊娠前与确定妊娠后IVIG治疗两个亚组进行分析,活产率在两种给药方案间无统计学差异(RR = 1.09, 95% CI (0.95~1.27), P = 0.06)。相比于妊娠后给药组(103/172 vs 101/168, RR = 1.00, 95% CI (0.84~1.18), P = 0.97),在妊娠前给予IVIG治疗RSA患者活产率更高(46/74 vs. 37/82;RR = 1.38, 95% CI (1.03~1.86), P = 0.03)。将原发性RSA患者分为妊娠前与妊娠后给予IVIG治疗两个亚组进行分析,活产率在两种方案之间无统计学差异(RR = 0.88, 95% CI = 0.71~1.07, P = 0.45)。将继发性RSA患者分为妊娠前与妊娠后给予IVIG治疗两个亚组进行分析,活产率在两种方案之间无统计学差异(RR = 1.24, 95% CI = 0.98~1.57, P = 0.94)。结论:IVIG治疗RSA疗效尚不确定。相对于原发性RSA,IVIG治疗对提高继发性RSA患者活产率有积极作用。RSA患者在妊娠前给予IVIG治疗中获益。对于继发性RSA患者,妊娠前与妊娠后给予IVIG治疗,活产率无统计学差异。
