注射用右兰索拉唑治疗急性胃和/或十二指肠溃疡引起的上消化道出血的有效性及安全性

目的：以注射用兰索拉唑为对照,评价注射用右兰索拉唑15 mg q12 h治疗急性胃和/或十二指肠溃疡引起的上消化道出血的有效性及安全性。方法：选取全国31家研究中心的急性胃和/或十二指肠溃疡引起的上消化道出血患者共202例,按照1∶1随机分配至试验组（注射用右兰索拉唑组）和对照组（注射用兰索拉唑组）。主要疗效终点为72 h有效止血率。对主要疗效终点采用非劣效评价,非劣效性界值δ是10%。结果：有效性方面,全分析数据集分析结果显示：用药72 h后,注射用右兰索拉唑组有效止血率为96.08%(98/102);注射用兰索拉唑组有效止血率为98.00%(98/100),两组率差为-1.92%(95%CI-6.58,2.74)。两组72 h有效止血率差异无统计学意义（P=0.682 9）。两组率差的双侧界值均低于δ(10%),注射用右兰索拉唑非劣于注射用兰索拉唑。安全性方面,试验组的不良事件及不良反应发生率与对照组差异无统计学意义,无非预期不良反应和严重不良反应。主要的不良反应为白细胞计数降低、中性粒细胞计数降低等。结论：注射用右兰索拉唑15 mg q12 h在治疗急性胃和/或十二指肠溃疡引起的...     

Hidradenoma papilliferum associated with pregnancy: a case report

We present the case of a 33‐year‐old female who developed a cystic nodule on the vulva during pregnancy. Immediately following Cesarean section, the lesion was biopsied and histologic examination revealed a dermal tumor composed of glandular structures arranged in a labyrinth pattern. The glandular structures displayed cytoplasmic vacuolization, large atypical nuclei, prominent nucleoli and scattered eosinophilic luminal secretions. Immunohistochemistry showed the tumor cells to be diffusely positive for CK7 and progesterone receptor with focal expression of mammaglobin and GCDFP‐15. The tumor cells were negative for estrogen receptor and CK20. These histologic and immunophenotypic findings were consistent with hidradenoma papilliferum. Our unusual (and to our knowledge first reported) case demonstrates hidradenoma papilliferum in association with pregnancy and raises the possibility of cytologic atypia and lactational change being secondary to hormonal changes in pregnancy.     

Evidence for processing of dolichol-linked oligosaccharides in patients with neuronal ceroid-lipofuscinosis.

In agreement with reports from other laboratories, we have shown that patients with the juvenile or late infantile forms of neuronal ceroid-lipofuscinosis (NCL) have greatly increased levels (5-fold to 20-fold) of dolichyl pyrophosphoryl oligosaccharides in their cerebral gray matter. Oligosaccharides containing 2 GlcNAc residues and 3 to 9 mannose residues were liberated by mild acid hydrolysis. The oligosaccharide profile given by brain tissue from 2 patients with infantile NCL was markedly different from that of late infantile and juvenile NCL brain, with Man9GlcNAc2 as the most abundant component and decreasing amounts of Man8- Man7- and Man6GlcNAc2. By contrast, Man5GlcNAc2 was the most abundant oligosaccharide present in all juvenile NCL brain samples analyzed. Both the susceptibility of the isolated Man5GlcNAc2 to endoglucosaminidase H digestion and permethylation analysis clearly indicated that it is not an intermediate in the biosynthesis of Glc3Man9GlcNAc2-PP-dolichol but has undergone catabolism, probably either in the endoplasmic reticulum or in the Golgi apparatus. Treatment of cultured skin fibroblasts for 7 days with N-methyldeoxynojirimycin, a potent inhibitor of the endoplasmic reticulum processing enzymes glucosidase I and II, resulted in an accumulation of the same Man5GlcNAc2-PP-dolichol species that was elevated in juvenile NCL brain. The level in untreated fibroblasts was undetectable, suggesting that inhibition of processing glucosidases has interfered with the regulation and compartmentalization of lipid-linked oligosaccharides.