Danon病是一种罕见的X连锁显性遗传病,由Danon在1981年首次报道[1]。因Danon病的特征性病理改变为骨骼肌和心肌细胞内存在自噬空泡和糖原颗粒增多,酸性麦芽糖酶活性正常,曾被称为X连锁的空泡性骨骼肌心肌病或酸性麦芽糖酶正常的溶酶体...Danon病是一种罕见的X连锁显性遗传病,由Danon在1981年首次报道[1]。因Danon病的特征性病理改变为骨骼肌和心肌细胞内存在自噬空泡和糖原颗粒增多,酸性麦芽糖酶活性正常,曾被称为X连锁的空泡性骨骼肌心肌病或酸性麦芽糖酶正常的溶酶体糖原贮积症。2000年Nishino等[2]首次报道编码溶酶体相关膜蛋白2(lysosomal-associated membrane protein 2,LAMP2)的LAMP2基因突变可导致该病。目前关于Danon病的报道已超过500例[3],其临床表现各不相同,但以肥厚型心肌病、骨骼肌病、智力障碍为主要临床表现。本文回顾性分析1例以抽搐起病的Danon病患者的临床资料,报道如下。展开更多
Danon病(Danon Disease,DD)是一种罕见的X连锁溶酶体贮积症。本文报道了一个DD家系,共2人携带溶酶体相关膜蛋白2(Lysosomal Associated Membrane Protein 2,LAMP2)基因的致病突变c.928G>C:p.V310L。两名患者表现出不同的临床表型。...Danon病(Danon Disease,DD)是一种罕见的X连锁溶酶体贮积症。本文报道了一个DD家系,共2人携带溶酶体相关膜蛋白2(Lysosomal Associated Membrane Protein 2,LAMP2)基因的致病突变c.928G>C:p.V310L。两名患者表现出不同的临床表型。先证者接受了两次经导管心脏射频消融术,分别消融了左、右两侧旁道。先证者母亲以反复心力衰竭为主要表现。在母亲进行心脏移植之前,尝试使用左束支区域起搏(Left Bundle Branch Area Pacing,LBBAP)结合左室电极进行再同步化改善心功能。据我们所知,这是首例被报道接受LBBAP进行心脏再同步化治疗的DD患者。左束支区域起搏在原起搏模式基础上可能进一步延缓了该患者心衰的进展,心脏移植术后标本展示了这种罕见病中LBBAP电极的位置及与起搏图形的关系。证实在该类患者中,左束支区域起搏技术能够成功实施。展开更多
Social business is becoming popular worldwide as a sustainable and innovative solution to tackling pressing social problems. The Grameen Danone Food Ltd. (GDFL) Shokti+ yogurt business was chosen as a social business ...Social business is becoming popular worldwide as a sustainable and innovative solution to tackling pressing social problems. The Grameen Danone Food Ltd. (GDFL) Shokti+ yogurt business was chosen as a social business case study. The study described GDFL Shokti+ yogurt production, pricing, and distribution process, elucidated consumers’ perception of the Shokti+ yogurt, identified the major problems faced by the consumers, and proposed probable solutions to popularize the yogurt among the consumers. The study’s objectives were achieved using descriptive statistics based on a quantitative survey of 60 consumers. The study found that Shokti+ yogurt is distributed primarily through four channels: modern model, urban trade, rural trade, and direct trade. The study revealed that non-poor households consume more Shokti+ yogurt than poor households. The findings showed that about 78% and 95% of respondents were satisfied with the yogurt quality and availability, respectively. About 17% of respondents perceived that Shokti+ yogurt is superior to regular yogurt. However, about 58% of respondents claimed that the price is higher than the amount and quality offered. Excessive liquidity, increased price, and lack of trust in the nutritional quality of yogurt were some major problems identified by the consumers. The study proposed certain recommendations such as maintaining the consistency of the yogurt, increasing the amount of yogurt sold, making the yogurt more affordable for poor consumers, and raising awareness about its nutritional benefits for popularizing Shokti+ yogurt in Bangladesh.展开更多
Background: In 1996, Wahaha, Danone and BNP (Hong Kong) jointly invested in and incorporated 5 companies to manufacture Wahaha brand products, including purified water and eight-treasure porridge. Wahaha holds 49% equ...Background: In 1996, Wahaha, Danone and BNP (Hong Kong) jointly invested in and incorporated 5 companies to manufacture Wahaha brand products, including purified water and eight-treasure porridge. Wahaha holds 49% equity interests in these companies, while Danone later became the dominant shareholder with a 51% stake after acquiring shares held by BNP in the aftermath of the Asian financial crisis. Danone then proposed to transfer the Wahaha brand to a joint venture but did not succeed. Subsequently, a trademark usage contract was signed by both parties instead. In the ensuing years, the Wahaha Group established non-JV companies in west China, central China and the Three Gorges reservoir area. These companies had RMB 5.6 billion of total assets as of December 31st, 2006 and made a net income of RMB 1.04 billion in that year. On April 3rd, 2007, Danone intended to force a deal by which it could acquire 51% equity interests in those non-JV companies for a sum of RMB 4 billion. Wahaha Chairman Zong Qinghou later complained that he and Wahaha fell into a trap set up by Danone. Both parties have since engaged in a lengthy "war of words", thereby arousing a grand debate on China's FDI policies.展开更多
文摘Danon病是一种罕见的X连锁显性遗传病,由Danon在1981年首次报道[1]。因Danon病的特征性病理改变为骨骼肌和心肌细胞内存在自噬空泡和糖原颗粒增多,酸性麦芽糖酶活性正常,曾被称为X连锁的空泡性骨骼肌心肌病或酸性麦芽糖酶正常的溶酶体糖原贮积症。2000年Nishino等[2]首次报道编码溶酶体相关膜蛋白2(lysosomal-associated membrane protein 2,LAMP2)的LAMP2基因突变可导致该病。目前关于Danon病的报道已超过500例[3],其临床表现各不相同,但以肥厚型心肌病、骨骼肌病、智力障碍为主要临床表现。本文回顾性分析1例以抽搐起病的Danon病患者的临床资料,报道如下。
文摘Danon病(Danon Disease,DD)是一种罕见的X连锁溶酶体贮积症。本文报道了一个DD家系,共2人携带溶酶体相关膜蛋白2(Lysosomal Associated Membrane Protein 2,LAMP2)基因的致病突变c.928G>C:p.V310L。两名患者表现出不同的临床表型。先证者接受了两次经导管心脏射频消融术,分别消融了左、右两侧旁道。先证者母亲以反复心力衰竭为主要表现。在母亲进行心脏移植之前,尝试使用左束支区域起搏(Left Bundle Branch Area Pacing,LBBAP)结合左室电极进行再同步化改善心功能。据我们所知,这是首例被报道接受LBBAP进行心脏再同步化治疗的DD患者。左束支区域起搏在原起搏模式基础上可能进一步延缓了该患者心衰的进展,心脏移植术后标本展示了这种罕见病中LBBAP电极的位置及与起搏图形的关系。证实在该类患者中,左束支区域起搏技术能够成功实施。
文摘Social business is becoming popular worldwide as a sustainable and innovative solution to tackling pressing social problems. The Grameen Danone Food Ltd. (GDFL) Shokti+ yogurt business was chosen as a social business case study. The study described GDFL Shokti+ yogurt production, pricing, and distribution process, elucidated consumers’ perception of the Shokti+ yogurt, identified the major problems faced by the consumers, and proposed probable solutions to popularize the yogurt among the consumers. The study’s objectives were achieved using descriptive statistics based on a quantitative survey of 60 consumers. The study found that Shokti+ yogurt is distributed primarily through four channels: modern model, urban trade, rural trade, and direct trade. The study revealed that non-poor households consume more Shokti+ yogurt than poor households. The findings showed that about 78% and 95% of respondents were satisfied with the yogurt quality and availability, respectively. About 17% of respondents perceived that Shokti+ yogurt is superior to regular yogurt. However, about 58% of respondents claimed that the price is higher than the amount and quality offered. Excessive liquidity, increased price, and lack of trust in the nutritional quality of yogurt were some major problems identified by the consumers. The study proposed certain recommendations such as maintaining the consistency of the yogurt, increasing the amount of yogurt sold, making the yogurt more affordable for poor consumers, and raising awareness about its nutritional benefits for popularizing Shokti+ yogurt in Bangladesh.
文摘Background: In 1996, Wahaha, Danone and BNP (Hong Kong) jointly invested in and incorporated 5 companies to manufacture Wahaha brand products, including purified water and eight-treasure porridge. Wahaha holds 49% equity interests in these companies, while Danone later became the dominant shareholder with a 51% stake after acquiring shares held by BNP in the aftermath of the Asian financial crisis. Danone then proposed to transfer the Wahaha brand to a joint venture but did not succeed. Subsequently, a trademark usage contract was signed by both parties instead. In the ensuing years, the Wahaha Group established non-JV companies in west China, central China and the Three Gorges reservoir area. These companies had RMB 5.6 billion of total assets as of December 31st, 2006 and made a net income of RMB 1.04 billion in that year. On April 3rd, 2007, Danone intended to force a deal by which it could acquire 51% equity interests in those non-JV companies for a sum of RMB 4 billion. Wahaha Chairman Zong Qinghou later complained that he and Wahaha fell into a trap set up by Danone. Both parties have since engaged in a lengthy "war of words", thereby arousing a grand debate on China's FDI policies.
