BACKGROUND Left-sided accessory pathways(APs)can be accessed with either a transaortic(TA)or transseptal approach(TS).For children with Marfan syndrome(MFS)who have aortic disease,the use of TA can aggravate the disea...BACKGROUND Left-sided accessory pathways(APs)can be accessed with either a transaortic(TA)or transseptal approach(TS).For children with Marfan syndrome(MFS)who have aortic disease,the use of TA can aggravate the disease,making TS the best choice for these patients.CASE SUMMARY A 10-year-old girl was hospitalized because of intermittent heart palpitations and chest tightness.She was diagnosed with MFS,supraventricular tachycardia,Wolff-Parkinson-White syndrome,and left-sided AP was detected by cardiac electrophysiological.Catheter ablation was successfully performed via TS under the guidance of the Ensite system.During the follow-up,no recurrence or complications occurred.CONCLUSION The TS for catheter ablation of left-sided APs can be considered in children with MFS.Adequate evaluation and selection of the appropriate puncture site are particularly important.展开更多
AIM:To compare the clinical outcomes between two approaches for sutureless scleral-fixated intraocular lens(SFIOL)in children with Marfan syndrome(MFS).METHODS:The study included 15 children(26 eyes)with lens subluxat...AIM:To compare the clinical outcomes between two approaches for sutureless scleral-fixated intraocular lens(SFIOL)in children with Marfan syndrome(MFS).METHODS:The study included 15 children(26 eyes)with lens subluxation due to MFS.These children underwent lensectomy,anterior vitrectomy,and sutureless SFIOL.According to the position of placement of intraocular lens(IOL)haptics,two study groups were reviewed for best corrected visual acuity(BCVA)and postoperative complications:group A,14 eyes with haptics fixated at 2.0 mm from the limbus;group B,12 eyes with the haptics fixated at 2.5 mm from the limbus.RESULTS:The mean axial length for all patients was 25.66±2.35 mm.Postoperative BCVA in logMAR were significant improved in both groups(0.77±0.32 to 0.17±0.12 in group A,0.66±0.25 to 0.24±0.12 in group B,both P<0.001)while no significant difference between two groups(P>0.05).Pupillary capture was main postoperative complication,occurring between 3d and 18mo.It occurred in 7 eyes in group A and one eye in group B(P=0.02).CONCLUSION:Sutureless SFIOL is an effective treatment approach for lens subluxation in children with MFS.Pupillary capture is the main postoperative complication.Fixated IOL haptics at 2.5 mm from the limbus can reduce the occurrence of pupillary capture.展开更多
Marfan syndrome(MFS)includes a serious organic heart disease,for which no effective treatment methods are currently available.Most available drugs are only for symptoms management and have significant side effects.Thi...Marfan syndrome(MFS)includes a serious organic heart disease,for which no effective treatment methods are currently available.Most available drugs are only for symptoms management and have significant side effects.This study reported on the efficacy of a combination of Chinese and Western medicines to treat arrhythmia in a patient with MFS.The patient was a young woman who was frequently treated with amiodarone owing to MFS-related organic changes in her heart.Considering the negative side effects of amiodarone in the patient,a combination of traditional Chinese medicine decoction,Wenxin granule,and metoprolol was used to gradually replace amiodarone treatment.After three months of treatment the patient’s premature ventricular beats were controlled,and follow-up results were satisfactory.This case study provides a reference for the treatment of premature ventricular beats in patients with organic changes due to MFS.展开更多
Marfan syndrome is a rare genetic disease,and the condition of most patients deteriorates with age.The lesions are mainly cardiovascular,skeletal,and ocular lesions.Its clinical manifestations are characterized by con...Marfan syndrome is a rare genetic disease,and the condition of most patients deteriorates with age.The lesions are mainly cardiovascular,skeletal,and ocular lesions.Its clinical manifestations are characterized by congenital heart disease,ectopic lens,slender body,slender limbs,spider fingers(toes),and general muscle dysplasia.In the early stage of this disease,when the symptoms are not yet clearly manifested,some patients came to the hospital for treatment due to decreased vision.According to the particularity of crystal dislocation patients with Marfan syndrome,this article provides home nursing education in terms of physiological and psychological aspects,and builds an effective home nursing model to promote the physical and mental recovery of patients.展开更多
文摘BACKGROUND Left-sided accessory pathways(APs)can be accessed with either a transaortic(TA)or transseptal approach(TS).For children with Marfan syndrome(MFS)who have aortic disease,the use of TA can aggravate the disease,making TS the best choice for these patients.CASE SUMMARY A 10-year-old girl was hospitalized because of intermittent heart palpitations and chest tightness.She was diagnosed with MFS,supraventricular tachycardia,Wolff-Parkinson-White syndrome,and left-sided AP was detected by cardiac electrophysiological.Catheter ablation was successfully performed via TS under the guidance of the Ensite system.During the follow-up,no recurrence or complications occurred.CONCLUSION The TS for catheter ablation of left-sided APs can be considered in children with MFS.Adequate evaluation and selection of the appropriate puncture site are particularly important.
文摘AIM:To compare the clinical outcomes between two approaches for sutureless scleral-fixated intraocular lens(SFIOL)in children with Marfan syndrome(MFS).METHODS:The study included 15 children(26 eyes)with lens subluxation due to MFS.These children underwent lensectomy,anterior vitrectomy,and sutureless SFIOL.According to the position of placement of intraocular lens(IOL)haptics,two study groups were reviewed for best corrected visual acuity(BCVA)and postoperative complications:group A,14 eyes with haptics fixated at 2.0 mm from the limbus;group B,12 eyes with the haptics fixated at 2.5 mm from the limbus.RESULTS:The mean axial length for all patients was 25.66±2.35 mm.Postoperative BCVA in logMAR were significant improved in both groups(0.77±0.32 to 0.17±0.12 in group A,0.66±0.25 to 0.24±0.12 in group B,both P<0.001)while no significant difference between two groups(P>0.05).Pupillary capture was main postoperative complication,occurring between 3d and 18mo.It occurred in 7 eyes in group A and one eye in group B(P=0.02).CONCLUSION:Sutureless SFIOL is an effective treatment approach for lens subluxation in children with MFS.Pupillary capture is the main postoperative complication.Fixated IOL haptics at 2.5 mm from the limbus can reduce the occurrence of pupillary capture.
基金supported by grants from the Scientific Research Fund of Shanghai Municipal Health and Family Planning Commission(20164Y0072).
文摘Marfan syndrome(MFS)includes a serious organic heart disease,for which no effective treatment methods are currently available.Most available drugs are only for symptoms management and have significant side effects.This study reported on the efficacy of a combination of Chinese and Western medicines to treat arrhythmia in a patient with MFS.The patient was a young woman who was frequently treated with amiodarone owing to MFS-related organic changes in her heart.Considering the negative side effects of amiodarone in the patient,a combination of traditional Chinese medicine decoction,Wenxin granule,and metoprolol was used to gradually replace amiodarone treatment.After three months of treatment the patient’s premature ventricular beats were controlled,and follow-up results were satisfactory.This case study provides a reference for the treatment of premature ventricular beats in patients with organic changes due to MFS.
基金Xi’an People’s Hospital(Xi’an Fourth Hospital)Scientific Research Incubation Fund Project:Analysis of Failure Factors and Selection of ICL Intraocular Lens Implantation based on Statistical Analysis.Project number:FZ-72Xi’an Science and Technology Plan Project:Failure Mode Analysis of ICL Implantation Within One Year and Improvement of Artificial Lens Microstructure Based on Biomechanics.Project number:21YXYJ0047。
文摘Marfan syndrome is a rare genetic disease,and the condition of most patients deteriorates with age.The lesions are mainly cardiovascular,skeletal,and ocular lesions.Its clinical manifestations are characterized by congenital heart disease,ectopic lens,slender body,slender limbs,spider fingers(toes),and general muscle dysplasia.In the early stage of this disease,when the symptoms are not yet clearly manifested,some patients came to the hospital for treatment due to decreased vision.According to the particularity of crystal dislocation patients with Marfan syndrome,this article provides home nursing education in terms of physiological and psychological aspects,and builds an effective home nursing model to promote the physical and mental recovery of patients.
