Type IX Ehlers-Danlos syndrome. A new variant with pathognomonic radiographic features

The authors describe 7 male patients from two unrelated families who presented with what appears to be a heretofore undiagnosed X-linked variant of Ehlers-Danlos syndrome. Unlike the eight previously reported types, this variant is manifested by specific skeletal abnormalities, including occipital exostoses, widening and bowing of multiple long bones at tendinous and ligamentous insertion sites, and deformed clavicles. Major clinical complications include genitourinary problems, chronic diarrhea with malabsorption, and/or syncopal episodes. Laboratory tests suggest that this variant may represent the true lysyl-oxidase-deficient form of Ehlers-Danlos syndrome.     

Acne-associated spondylarthropathy: radiographic features

Experience with six patients with severe acne and associated axial and peripheral arthritis is described. Four of the patients had a dermatologic triad of severe acne, hidradenitis suppurativa, and dissecting cellulitis of the scalp, the so-called follicular occlusion triad. All were black men with episodic peripheral arthropathy and low back pain. One had inflammatory bowel disease. Rheumatoid factor and HLA-B27 were absent in five patients who had these determinations. An erosive and proliferative arthritis of the axial and appendicular skeleton is described. The radiographic findings were indistinguishable from those of the seronegative spondylarthropathies. We found no previous reports in the radiologic literature describing this articulocutaneous entity.     

Baby boomer pharmacists: ageing and projections of retirement

Objective To identify ageing and retirement patterns of the pharmacy workforce since 1986 and the implications of those changes for future workforce planning. Method Australian Bureau of Statistics census data from 1986 to 2001 were used to examine ageing of the pharmacy workforce and attrition of pharmacists aged 50 years and over. The number of pharmacists to retire was projected over the next 20 years. Key findings The Australian pharmacy workforce has aged significantly since 1986 (P < 0.01). Forty‐one per cent of pharmacists practising in 2001 are predicted to retire by 2026. Baby boomer pharmacists were more likely to work long hours (49+ per week) in 2001 than in 1986, and than generation X pharmacists in 2001. The proportion of women in pharmacy has increased from 39% in 1986 to over 50% in 2001. Women are still more likely to work part‐time than males, although they are less likely to do so than they were in 1986. Conversely, the proportion of male pharmacists working part‐time is rising. Conclusions With baby boomer and older cohorts currently making up 65% of the pharmacy workforce, their retirement will place increased pressure on a profession already facing severe shortages. The growing proportion of female pharmacists and their generally lower workforce participation mean the potential for continued pharmacy shortages.     

新型抗流感药物有望问世

H5N1流感病毒所致的禽流感在世界范围内引起了大流行,因此大家更加关注这种病毒是否会获得在人类之间传染的能力并引起禽流感大流行。目前用于治疗这种病毒感染引起的人禽流感的药物有两种,即奥司他韦(Tamiflu)和扎那米韦(Relenza),两者都是作用于病毒的神经氨酸酶。抗药性的出现需要研发新的抗流感药物。A型流感病毒的神经氨酸酶可分为遗传上不同的两种类型:一类包括N1神经氨酸酶,另一类包含N2、N9神经氨酸酶。N2、N9神经氨酸酶已经被作为当前药物设计的结构基础。用X射线衍射晶体分析法发现这两类酶结构上是不同的。前者在其活性位点…     

珍珠层／聚乳酸人工骨植入家犬颈椎椎间的融合效果

目的:观察珍珠层/聚乳酸人工骨植入家犬颈椎椎间的融合效果。方法:实验于2004-09/2005-06在南方医科大学附属南方医院实验动物中心及脊柱骨病科实验室完成。选择成年家犬14只,随机数字表法分为4组,实验组(n=4):在颈椎5/6或者6/7椎间植入珍珠层/聚乳酸人工骨;异体骨组(n=4):在相同部位植入体积分数为0.75的乙醇浸泡的新鲜同种异体髂骨;聚乳酸组(n=4):植入经环氧乙烷消毒的高分子聚乳酸材料;空白对照组(n=2):未植入任何材料,上述各组均用手外科微型钢板内固定。分别于术后4,8,12,16周行X射线片及组织病理检查。结果:纳入动物14只,均进入结果分析。①通过X射线及组织病理检查对椎间融合进行判断,在术后16周,实验组的融合程度差于异体骨组,优于聚乳酸组。②病理检查显示复合材料前方的软组织量极少,为非特异性的纤维结缔组织,在周围正常骨组织内可见珍珠层粉微粒,细小的颗粒被异物巨细胞吞噬,复合材料中可见大量类骨质,材料中的聚乳酸部分降解。③骨磨片显示新骨组织长入复合材料的小孔内,珍珠层粉微粒对骨组织无明显影响。结论:珍珠层/聚乳酸人工骨在体内有较好的植骨融合效果,并可以被缓慢降解吸收。     

Quantitation of human in vitro megakaryocytopoiesis by radioimmunoassay

The isolation and characterization of human megakaryocyte growth factors has been hampered because evaluation of megakaryocyte growth in semisolid medium requires both lengthy incubation and visual quantitation. In addition, colony formation requires cell division, while most regulation of platelet production may involve individual, nonproliferating differentiating megakaryocytes. We have developed a radioimmunoassay (RIA) that makes use of an iodinated murine monoclonal antibody (MoAb) specific for platelet/megakaryocyte glycoprotein IIb/IIIa (GPIIb/IIIa) to measure megakaryocyte production in liquid marrow culture. This assay is sensitive to 3 X 10(3) platelets (roughly 30 megakaryocytes) and linear up to 1 X 10(6) platelets, and thus it provides a useful range for quantitating megakaryocyte production in in vitro marrow culture. Significant differences (threefold to fivefold) in megakaryocyte/platelet-specific GPIIb/IIIa complex are detected between stimulated and unstimulated marrow cultures by day 7, although antigen accrual in stimulated cultures continues through at least day 16. Conditions that promote megakaryocyte growth in semisolid medium (ie, aplastic plasma and PHA-LCM) have also been facilitory in liquid culture. This rapid and sensitive assay for cell-bound GPIIb/IIIa should facilitate recognition and isolation of megakaryocyte and platelet growth factors.