重症肾血管炎的辅助疗法——血浆置换或大剂量甲泼尼龙随机试验

Wegener肉芽肿和显微镜下多血管炎是原发性系统性血管炎,与循环中对蛋白酶3(PR3)或髓过氧化物酶(MPO)有特异性的中性粒细胞胞质抗原(ANCA)自身抗体密切相关.70%的患者肾组织学上主要表现为以中性粒细胞为主的强烈的炎性浸润;表现出肾小球节段性坏死的肾小球毛细管炎;肾小球内单核细胞增生导致寡免疫,聚集和坏死,新月体肾小球肾炎.80%的急进性肾炎发生血管炎并进展为终末期肾功能衰竭的病例,可以通过类固醇和免疫治疗来预防.     

Stricture of the common bile duct secondary to blunt abdominal trauma

A 3-year- 8-month-old girl developed obstructive jaundice associated with stricture of the distal end of the common bile duct 7 months after blunt abdominal trauma. Ultrasonography, computerised tomography, and percutaneous trans-hepatic cholangiography made the anatomical diagnosis before surgical reconstruction. Histological examination of the retroperitoneal tissue near the site of the stricture showed evidence of previous bile leakage due to rupture of the duct. Relief of obstruction was achieved by a Roux-en-Y choledochojejunostomy with complete resolution of symptoms and restoration of normal liver function. Offprint requests to: H. C. O Martin     

Assessment of an abbreviated odorant identification task for children: a rapid screening device for schools and clinics

To validate the level of olfactory performance of children, we tested 825 volunteers, aged 4–17 years, with an abbreviated form of our pediatric odorant identification task. The test consisted of sniffing and identifying five odorants (baby powder, bubble gum, candy cane, licorice and peach). Mean olfactory scores increased as a function of age, reaching a plateau of about 94–95% correct at 8 years of age. In general, girls out–performed boys. Physicians require a test instrument such as the one we have devised to allow them to diagnose olfactory dysfunction in children. The present task is particularly applicable in screening large numbers of children in clinics or schools because it can be administered easily and rapidly. Adult subjects with olfactory dysfunction also performed poorly on this odorant identification task designed for children. Therefore, we expect that our odorant identification task will also detect children with severe olfactory dysfunction.     

HLA class II specificities in vasculitis with antibodies to neutrophil cytoplasmic antigens.

HLA class II genes were examined in patients with small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) using restriction fragment length polymorphism and allele specific oligonucleotide typing. Fifty-nine patients were studied, 34 with Wegener's granulomatosis and 25 with microscopic polyarteritis, and their results were compared with those from 1103 British Caucasoid controls. The frequency of HLA-DQw7 was significantly increased in patients with vasculitis (patients 53%; controls 27.8%, chi 2 17.8, Pc less than 0.0025, relative risk 2.9), and all the DQw7 bearing haplotypes commonly found in Caucasoid populations contributed to the increase. By contrast, the frequency of HLA-DR3 bearing haplotypes was decreased in the patients (patients 6.8%; controls 21.6%, chi 2 6.7, P less than 0.01). HLA specificities were similar in the groups of patients presenting with Wegener's granulomatosis and microscopic polyarteritis and with different types of ANCA assessed by indirect immunofluorescence. However, patients with the DQw7, DR4 haplotype were significantly more likely to have transiently positive tests for ANCA than patients with other DQw7 bearing haplotypes, whereas patients with DR2 bearing haplotypes were more likely to have persistently positive ANCA. These results show that HLA class II genes are associated with small vessel vasculitis and may influence the duration of the associated autoimmune response.