Aim: To evaluate the Baerveldt glaucoma implant (BGI) in paediatric glaucoma treatment. Methods: In a retrospective non-comparative case series 55 eyes of 40 consecutive paediatric patients (≤ 16 years) with primary ...Aim: To evaluate the Baerveldt glaucoma implant (BGI) in paediatric glaucoma treatment. Methods: In a retrospective non-comparative case series 55 eyes of 40 consecutive paediatric patients (≤ 16 years) with primary or secondary glaucoma underwent Baerveldt (350 mm2) implantation. Surgical out come was evaluated by Kaplan-Meier table analysis. Results: The overall success rate was 80% at last follow up, with a mean follow up of 32 (range 2- 78) months. Cumulative success was 94% at 12 months and 24 months, 85% at 36 months, 78% at 48 months, and 44% at 60 months. 11 eyes (20% )-failed postoperatively because of an IOP > 21 mm Hg (eight eyes), persistent hypotony (two eyes), and choroidal haemorrhage following cataract surgery (one eye). The most frequent complication needing surgery was tube related (20% ). A new observation was mild to moderate dyscoria in 22% of the eyes, all buphthalmic, caused by entrapment of a tuft of peripheral iris in the tube track. Conclusions: The BGI is effective and safe in the management of primary and secondary glaucoma. When angle surgery has proved to be unsuccessful or inappropriate in paediatric patients, a BGI is a good treatment option. One must be prepared to deal with the tube related problems.展开更多
Background: Restrictive dermopathy is a very rare autosomal recessive skin disorder. The typical pathologic findings are striking: microstomia, micrognathia, thin but very tight translucent skin that tears spontaneous...Background: Restrictive dermopathy is a very rare autosomal recessive skin disorder. The typical pathologic findings are striking: microstomia, micrognathia, thin but very tight translucent skin that tears spontaneously, and arthrogryposis multiplex. The mechanisms behind this disease are unknown. Observations: We describe for the first time a newborn girl with restrictive dermopathy, transposition of the great vessels, and microcolon. She had thin shiny skin with nearly no compliance indicating restrictive dermopathy. Additional dysmorphic findings included enlarged fontanelle, hypertelorism, absent eyelashes,smallpinchednose,microstomia,micrognathia,dysplastic ears, pterygium colli. dyplastic fingers and toes with upper- and partial lower- limb flexion contractures, dysplastic genitalia, and muscular hypotonia. She also had left transposition of the great artery with small atrial septal defect, bilateral hypoplasia of the first rib, and congenital stenosis of the small bowel with microcolon. Conclusions: The pathogonomic diagnostic features remain reduced dermal thickness and nearly complete absence of elastic fibers in the dermis. In mice, a defective fatty acid transport protein 4 gene (Fatp4) leads to clear signs of restrictive dermopathy by influencing the arrangement of the lipids in the epidermis. Whether the left transposition of the great artery is associated with restrictive dermopathy or represents an additional malformation of multifactorial, polygenetic, or monogenetic cause remains open.展开更多
目的探讨盆底肌功能康复训练方法,如Kegel训练和阴道康复器辅助训练,对于改善初产妇产后盆底肌功能的效果,评估阴道康复器辅助训练,对于初产妇产后盆底肌功能的恢复作用。方法选取2016年1月至6月,在江苏省淮安市妇幼保健院足月经阴道分...目的探讨盆底肌功能康复训练方法,如Kegel训练和阴道康复器辅助训练,对于改善初产妇产后盆底肌功能的效果,评估阴道康复器辅助训练,对于初产妇产后盆底肌功能的恢复作用。方法选取2016年1月至6月,在江苏省淮安市妇幼保健院足月经阴道分娩,并于产后第42天返回医院接受盆底肌功能评估的60例盆底肌肌力下降初产妇为研究对象。按照随机数字表法,将其分为研究组( n =30)与对照组( n =30)。对研究组受试者采用阴道康复器辅助训练至产后第18周,再改为Kegel训练至产后1年。对对照组受试者采用Kegel训练至产后1年。2组受试者在产后第6、10、18周及1年时,盆底Ⅰ、Ⅱ类肌纤维不同级别肌力发生率比较,采用Cochran-Mantel-Haenszel检验。受试者产后盆底Ⅰ、Ⅱ类肌纤维肌力影响因素分析,分别以其产后盆底Ⅰ、Ⅱ类肌纤维肌力为因变量,治疗措施、时间、治疗措施与时间交互效应为自变量,进行非线性混合效应模型(NLMIXED)分析。本研究遵循的程序符合江苏省淮安市妇幼保健院人体试验委员会制定的标准,并经过该伦理委员会批准(批准文号:2014016),并与所有受试者签订临床研究知情同意书。结果①2组受试者年龄、孕次、人体质量指数(BMI)、新生儿出生体重及治疗前盆底Ⅰ、Ⅱ类纤维的肌力分级构成比等一般临床资料比较,差异均无统计学意义( P >0.05)。②控制治疗措施因素后,受试者产后盆底Ⅰ、Ⅱ类肌纤维肌力变化,均具有显著时间趋势,即随着训练时间延长,产后盆底肌功能不断恢复,并且差异均有统计学意义(χ^ 2 =133.204、116.344, P <0.001);控制时间因素后,研究组治疗措施对受试者产后盆底Ⅰ类肌纤维肌力的恢复情况,显著优于对照组,差异亦有统计学意义(χ^2 =22.239, P <0.001),但是2组治疗措施对受试者产后盆底Ⅱ类肌纤维肌力的恢复情况比较,差异无统计学意义( P >0.0展开更多
文摘Aim: To evaluate the Baerveldt glaucoma implant (BGI) in paediatric glaucoma treatment. Methods: In a retrospective non-comparative case series 55 eyes of 40 consecutive paediatric patients (≤ 16 years) with primary or secondary glaucoma underwent Baerveldt (350 mm2) implantation. Surgical out come was evaluated by Kaplan-Meier table analysis. Results: The overall success rate was 80% at last follow up, with a mean follow up of 32 (range 2- 78) months. Cumulative success was 94% at 12 months and 24 months, 85% at 36 months, 78% at 48 months, and 44% at 60 months. 11 eyes (20% )-failed postoperatively because of an IOP > 21 mm Hg (eight eyes), persistent hypotony (two eyes), and choroidal haemorrhage following cataract surgery (one eye). The most frequent complication needing surgery was tube related (20% ). A new observation was mild to moderate dyscoria in 22% of the eyes, all buphthalmic, caused by entrapment of a tuft of peripheral iris in the tube track. Conclusions: The BGI is effective and safe in the management of primary and secondary glaucoma. When angle surgery has proved to be unsuccessful or inappropriate in paediatric patients, a BGI is a good treatment option. One must be prepared to deal with the tube related problems.
文摘Background: Restrictive dermopathy is a very rare autosomal recessive skin disorder. The typical pathologic findings are striking: microstomia, micrognathia, thin but very tight translucent skin that tears spontaneously, and arthrogryposis multiplex. The mechanisms behind this disease are unknown. Observations: We describe for the first time a newborn girl with restrictive dermopathy, transposition of the great vessels, and microcolon. She had thin shiny skin with nearly no compliance indicating restrictive dermopathy. Additional dysmorphic findings included enlarged fontanelle, hypertelorism, absent eyelashes,smallpinchednose,microstomia,micrognathia,dysplastic ears, pterygium colli. dyplastic fingers and toes with upper- and partial lower- limb flexion contractures, dysplastic genitalia, and muscular hypotonia. She also had left transposition of the great artery with small atrial septal defect, bilateral hypoplasia of the first rib, and congenital stenosis of the small bowel with microcolon. Conclusions: The pathogonomic diagnostic features remain reduced dermal thickness and nearly complete absence of elastic fibers in the dermis. In mice, a defective fatty acid transport protein 4 gene (Fatp4) leads to clear signs of restrictive dermopathy by influencing the arrangement of the lipids in the epidermis. Whether the left transposition of the great artery is associated with restrictive dermopathy or represents an additional malformation of multifactorial, polygenetic, or monogenetic cause remains open.
文摘目的探讨盆底肌功能康复训练方法,如Kegel训练和阴道康复器辅助训练,对于改善初产妇产后盆底肌功能的效果,评估阴道康复器辅助训练,对于初产妇产后盆底肌功能的恢复作用。方法选取2016年1月至6月,在江苏省淮安市妇幼保健院足月经阴道分娩,并于产后第42天返回医院接受盆底肌功能评估的60例盆底肌肌力下降初产妇为研究对象。按照随机数字表法,将其分为研究组( n =30)与对照组( n =30)。对研究组受试者采用阴道康复器辅助训练至产后第18周,再改为Kegel训练至产后1年。对对照组受试者采用Kegel训练至产后1年。2组受试者在产后第6、10、18周及1年时,盆底Ⅰ、Ⅱ类肌纤维不同级别肌力发生率比较,采用Cochran-Mantel-Haenszel检验。受试者产后盆底Ⅰ、Ⅱ类肌纤维肌力影响因素分析,分别以其产后盆底Ⅰ、Ⅱ类肌纤维肌力为因变量,治疗措施、时间、治疗措施与时间交互效应为自变量,进行非线性混合效应模型(NLMIXED)分析。本研究遵循的程序符合江苏省淮安市妇幼保健院人体试验委员会制定的标准,并经过该伦理委员会批准(批准文号:2014016),并与所有受试者签订临床研究知情同意书。结果①2组受试者年龄、孕次、人体质量指数(BMI)、新生儿出生体重及治疗前盆底Ⅰ、Ⅱ类纤维的肌力分级构成比等一般临床资料比较,差异均无统计学意义( P >0.05)。②控制治疗措施因素后,受试者产后盆底Ⅰ、Ⅱ类肌纤维肌力变化,均具有显著时间趋势,即随着训练时间延长,产后盆底肌功能不断恢复,并且差异均有统计学意义(χ^ 2 =133.204、116.344, P <0.001);控制时间因素后,研究组治疗措施对受试者产后盆底Ⅰ类肌纤维肌力的恢复情况,显著优于对照组,差异亦有统计学意义(χ^2 =22.239, P <0.001),但是2组治疗措施对受试者产后盆底Ⅱ类肌纤维肌力的恢复情况比较,差异无统计学意义( P >0.0
