AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The rel...AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The relationship between the AL/TCRP ratio and the diagnosis of MFS for 192 eyes in 97 children were evaluate.The biological characteristics,including age,sex,AL,and TCRP,were collected from medical records.Receiver operating characteristic(ROC)curve analysis was performed to investigate whether the AL/TCRP ratio effectively distinguishes MFS from other subjects.The Youden index was used to re-divide the whole population into two groups according to an AL/TCRP ratio of 0.59.RESULTS:Of 96 subjects(mean age 7.46±3.28 y)evaluated,56(110 eyes)had a definite diagnosis of MFS in childhood based on the revised Ghent criteria,41(82 eyes)with diagnosis of congenital ectopia lentis(EL)were included as a control group.AL was negatively correlated with TCRP,with a linear regression coefficient of-0.36(R2=0.08).A significant correlation was found between age and the AL/TCRP ratio(P=0.023).ROC curve analysis showed that the AL/TCRP ratio distinguished MFS from the other patients at a threshold of 0.59.MFS patients were present in 24/58(41.38%)patients with an AL/TCRP ratio of≤0.59 and in 34/39(87.18%)patients with an AL/TCRP ratio of>0.59.CONCLUSION:An AL/TCRP ratio of>0.59 is significantly associated with the risk of MFS.The AL/TCRP ratio should be measured as a promising marker for the prognosis of children MFS.Changes in the AL/TCRP ratio should be monitored over time.展开更多
Marfan’s syndrome(MS) is an inherited disease of connective tissue. The most characteristic ocular change is dislocation of lens, but myopia and retinal detachment also can occur. We examined 11 MS patients using ele...Marfan’s syndrome(MS) is an inherited disease of connective tissue. The most characteristic ocular change is dislocation of lens, but myopia and retinal detachment also can occur. We examined 11 MS patients using electroretinography (ERG). 6 cases showed normal ERG. 5 cases had abnormal ERG. One had retinal detachment. 3 had my- opia and 1 case suffered from secondary glaucoma.The defects of visual function were shown by electrophysiological test in those MS patients.展开更多
Background: Acute aortic dissection is a common life-threatening disorder affecting the aorta. The immediate mortality rate in aortic dissection is as high as 1% per hour over the first several hours, making early dia...Background: Acute aortic dissection is a common life-threatening disorder affecting the aorta. The immediate mortality rate in aortic dissection is as high as 1% per hour over the first several hours, making early diagnosis and treatment critical for survival. Case presentation: We are presenting a case of Stanford Type A aortic dissection in a 58-year-old male patient with a history of hypertension. He arrived at the emergency department (ED) with diagnosed acute coronary syndrome a few hours after a sudden and severe worsening of his epigastric pain. Interesting case where the dissection starts from the orifice of the right coronary artery, occupies the aortic valve. Conclusion: Predictors of follow-up this cause mortality reflect patient history variables as opposed to in-hospital parameters or in-hospital complications, which may be explained by the successful in-hospital treatment of the acute dissection.展开更多
基金Supported by the National Natural Science Foundation of China(No.81770908)the Shanghai Science and Technology Commission(Scientific Innovation Project,No.20Y11911000)。
文摘AIM:To investigate whether the axial length(AL)/total corneal refractive power(TCRP)ratio is a sensitive and simple factor that can be used for the early diagnosis of Marfan’s syndrome(MFS)in children.METHODS:The relationship between the AL/TCRP ratio and the diagnosis of MFS for 192 eyes in 97 children were evaluate.The biological characteristics,including age,sex,AL,and TCRP,were collected from medical records.Receiver operating characteristic(ROC)curve analysis was performed to investigate whether the AL/TCRP ratio effectively distinguishes MFS from other subjects.The Youden index was used to re-divide the whole population into two groups according to an AL/TCRP ratio of 0.59.RESULTS:Of 96 subjects(mean age 7.46±3.28 y)evaluated,56(110 eyes)had a definite diagnosis of MFS in childhood based on the revised Ghent criteria,41(82 eyes)with diagnosis of congenital ectopia lentis(EL)were included as a control group.AL was negatively correlated with TCRP,with a linear regression coefficient of-0.36(R2=0.08).A significant correlation was found between age and the AL/TCRP ratio(P=0.023).ROC curve analysis showed that the AL/TCRP ratio distinguished MFS from the other patients at a threshold of 0.59.MFS patients were present in 24/58(41.38%)patients with an AL/TCRP ratio of≤0.59 and in 34/39(87.18%)patients with an AL/TCRP ratio of>0.59.CONCLUSION:An AL/TCRP ratio of>0.59 is significantly associated with the risk of MFS.The AL/TCRP ratio should be measured as a promising marker for the prognosis of children MFS.Changes in the AL/TCRP ratio should be monitored over time.
文摘Marfan’s syndrome(MS) is an inherited disease of connective tissue. The most characteristic ocular change is dislocation of lens, but myopia and retinal detachment also can occur. We examined 11 MS patients using electroretinography (ERG). 6 cases showed normal ERG. 5 cases had abnormal ERG. One had retinal detachment. 3 had my- opia and 1 case suffered from secondary glaucoma.The defects of visual function were shown by electrophysiological test in those MS patients.
文摘Background: Acute aortic dissection is a common life-threatening disorder affecting the aorta. The immediate mortality rate in aortic dissection is as high as 1% per hour over the first several hours, making early diagnosis and treatment critical for survival. Case presentation: We are presenting a case of Stanford Type A aortic dissection in a 58-year-old male patient with a history of hypertension. He arrived at the emergency department (ED) with diagnosed acute coronary syndrome a few hours after a sudden and severe worsening of his epigastric pain. Interesting case where the dissection starts from the orifice of the right coronary artery, occupies the aortic valve. Conclusion: Predictors of follow-up this cause mortality reflect patient history variables as opposed to in-hospital parameters or in-hospital complications, which may be explained by the successful in-hospital treatment of the acute dissection.
