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…     

Chemotherapy for malignant mixed Müllerian tumors of the ovary

OBJECTIVE: The aim of this study was to review the chemotherapy experience at Magee-Womens Hospital for malignant mixed müllerian tumor (MMMT) of the ovary. Patients were treated with either paclitaxel/carboplatin (PC) outpatient chemotherapy or platinum/ifosfamide (PI) inpatient chemotherapy as first- or second-line therapy. METHODS: Thirteen patients diagnosed with MMMT of the ovary after complete surgical staging from 1990 to 1999 were studied retrospectively. Six patients received PC combination chemotherapy, of which 3 patients received PC as first-line treatment. The other 3 patients received PC as second-line therapy. Eight patients were treated with PI. Demographic data, pathology, cytoreductive surgery, treatment, and survival rates were reviewed. Complete clinical response (CR) was defined as the disappearance of all measurable disease or normalization of elevated CA 125 level after chemotherapy. Kaplan-Meier analysis was used for survival analysis. RESULTS: The median survival time of patients receiving PC was 19 months. One patient, after receiving PC as first-line treatment, demonstrated a CR and is free of disease beyond 33 months. The median survival time of patients managed with PI was 23 months. Three patients with suboptimal disease demonstrated CR after receiving PI. CONCLUSIONS: Optimal chemotherapy regimen for MMMT of ovary remains to be determined. Platinum-based chemotherapy in combination with ifosfamide or paclitaxel may be active against this rare malignancy.     

A bile salt hydrolase of Brucella abortus contributes to the establishment of a successful infection through the oral route in mice

Choloylglycine hydrolase (CGH), a bile salt hydrolase, has been annotated in all the available genomes of Brucella species. We obtained the Brucella CGH in recombinant form and demonstrated in vitro its capacity to cleave glycocholate into glycine and cholate. Brucella abortus 2308 (wild type) and its isogenic Deltacgh deletion mutant exhibited similar growth rates in tryptic soy broth in the absence of bile. In contrast, the growth of the Deltacgh mutant was notably impaired by both 5% and 10% bile. The bile resistance of the complemented mutant was similar to that of the wild-type strain. In mice infected through the intragastric or the intraperitoneal route, splenic infection was significantly lower at 10 and 20 days postinfection in animals infected with the Deltacgh mutant than in those infected with the wild-type strain. For both routes, no differences in spleen CFU were found between animals infected with the wild-type strain and those infected with the complemented mutant. Mice immunized intragastrically with recombinant CGH mixed with cholera toxin (CGH+CT) developed a specific mucosal humoral (immunoglobulin G [IgG] and IgA) and cellular (interleukin-2) immune responses. Fifteen days after challenge by the same route with live B. abortus 2308 cells, splenic CFU counts were 10-fold lower in mice immunized with CGH+CT than in mice immunized with CT or phosphate-buffered saline. This study shows that CGH confers on Brucella the ability to resist the antimicrobial action of bile salts. The results also suggest that CGH may contribute to the ability of Brucella to infect the host through the oral route.     

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。