Obliterative portal venopathy: A comparative study of 184 cases of extrahepatic portal obstruction and 469 cases of idiopathic portal hypertension

Abstract A total of 184 cases of extrahepatic portal obstruction (EHPO), mostly demonstrated by intraoperative portography and studied at 17 institutes during the period 1957–1983, were compared with 469 cases of idiopathic portal hypertension (IPH) similarly studied. Of the cases of EHPO, there were 101 males and 83 females; 93 were under 20 years of age and the average age was 25.9 years (i.e. much younger than that of IPH cases). There were two age peaks, one before age 19 years and the other at age 40–49 years. One out of three adult cases had a history of abdominal surgery, but otherwise the aetiologic factor was difficult to elicit. Bleeding was the initial symptom in the majority, and splenectomy and haematological findings of hypersplenism were less pronounced compared with IPH. Liver function tests were almost always normal. The liver appeared normal macroscopically in 69% and histologically in 35%. The changes seen in the remainder were similar to those in IPH; they were less frequent in young patients than in cases above age 20 years. Compared with IPH, the wedged hepatic venous pressure in patients with EHPO was lower and the gradient from the portal venous pressure was greater. It is concluded that extrahepatic portal obstruction is less common compared with IPH in Japan, and that there are cases particularly among adults that present clinicopathological features very similar to those of IPH. It is unclear at present whether these two disorders represent two different disease entities, or whether they represent one disorder with differences in the site of involvement along the portal vein system.     

A randomized clinical trial of prophylaxis in children with hemophilia A (the ESPRIT Study)

Summary. Background: Prevention of arthropathy is a major goal of hemophilia treatment. While studies in adults have demonstrated an impact of prophylaxis on the incidence of joint bleeds and patients’ well‐being in terms of improved quality of life (QoL), it is unclear whether or not prophylaxis influences the outcome and perception of well‐ of children with hemophilia. Objective: This randomized controlled study compared the efficacy of prophylaxis with episodic therapy in preventing hemarthroses and image‐proven joint damage in children with severe hemophilia A (factor VIII <1%) over a 10‐year time period. Methods: Forty‐five children with severe hemophilia A, aged 1–7 years (median 4), with negative clinical‐radiologic joint score at entry and at least one bleed during the previous 6 months, were consecutively randomized to prophylaxis with recombinant factor VIII (25 IU kg^?1 3 × week) or episodic therapy with ≥25 IU kg^?1 every 12–24 h until complete clinical bleeding resolution. Safety, feasibility, direct costs and QoL were also evaluated. Results: Twenty‐one children were assigned to prophylaxis, 19 to episodic treatment. Children on prophylaxis had fewer hemarthroses than children on episodic therapy: 0.20 vs. 0.52 events per patient per month (P < 0.02). Plain‐film radiology showed signs of arthropathy in six patients on prophylaxis (29%) vs. 14 on episodic treatment (74%) (P < 0.05). Prophylaxis was more effective when started early (≤36 months), with patients having fewer joint bleeds (0.12 joint bleeds per patient per month) and no radiologic signs of arthropathy. Conclusion: This randomized trial confirms the efficacy of prophylaxis in preventing bleeds and arthropathy in children with hemophilia, particularly when it is initiated early in life.     

地塞米松对家兔失血性休克-再灌注损伤的防治作用

目的：探讨地塞米松对失血性休克－再灌注损伤的防治作用。方法：制备家兔失血性休克模型,随机分为地塞米松保护组（Ⅱ组）和未用地塞米松对照组（Ⅰ组）,检测血浆和组织一氧化氮代谢产物（NOP）、丙二醛（MDA）含量及平均动脉压（MAP）。结果：休克前2组动物NOP、MDA及MAP间均无统计学差异。休克90分钟时2组动物NOP、MDA均明显升高,MAP均显著下降,再灌注后,Ⅱ组NOP及MDA均逐渐下降,再灌注3小时后接近休克前水平,但明显低于休克90分钟和Ⅰ组同时间点水平;Ⅱ组MAP逐渐上升,再灌注3小时后接近休克前水平,但明显高于休克90分钟和Ⅰ组同时间点水平。此外,Ⅱ组心、肺、肝、肾、肠道组织NOP及MDA含量均明显低于Ⅰ组。结论：地塞米松可降低一氧化氮及氧自由基水平,减轻脂质过氧化反应,对休克－再灌注损伤起良好的防护作用。     

Mortality and cancer incidence in persons with Down's syndrome, their parents and siblings

A cohort study of 1425 persons with Down's syndrome (DS), and of their parents (447 mothers, 435 fathers) and siblings (1176), was set up to investigate death rates from various causes and cancer incidence patterns. In individuals with DS the all-cause death rate was six times that of the national population (SMR = 622: 95% CI 559–693), the excess being attributable to many different causes. These included: leukaemia (SMR = 1304: 95% CI 651–2334); diabetes mellitus (SMR = 982: 95% CI 267–2515); Alzheimer's disease (SMR = 22028: 95% CI 7137–51326); epilepsy (SMR = 1727: 95% CI 744–3403); and congenital anomalies (SMR = 4987: 95% CI 4175–5955). The overall survival showed marked improvements for successive birth cohorts, particularly at young ages. For mothers and fathers of persons with DS, all-cause death rates were 20% lower than national rates and there were no significant excesses from any specific cause. For siblings, all-cause death rates were similar to national rates; the only condition with a significantly raised mortality ratio was colo-rectal cancer (SMR = 793: 95% CI 216–2031), but this may well be a chance finding.     

Reduction of immunosuppression for transplant-associated skin cancer: expert consensus survey

BACKGROUND: Reduction of immunosuppression is considered a reasonable adjuvant therapeutic strategy in solid-organ transplant recipients experiencing multiple or high-risk skin cancers. However, the literature provides no guidance about what threshold of cancer development would warrant initiation of reduction of immunosuppression. OBJECTIVES: To develop expert consensus guidelines for initiation of reduction of transplant-associated immunosuppression for solid-organ transplant recipients with severe skin cancer. METHODS: An expert consensus panel was convened by the International Transplant Skin Cancer Collaborative and Skin Care for Organ Transplant Patients Europe Reduction of Immunosuppression Task Force. Thirteen hypothetical patient scenarios with graduated morbidity and mortality risks were presented and mean and mode expert opinions about appropriate level of reduction of systemic immunosuppression (mild, moderate, severe) were generated. RESULTS: Mild reduction of transplant-associated immunosuppression was considered warranted once multiple skin cancers per year developed or with individual high-risk skin cancers. Moderate reduction was considered appropriate when patients experienced > 25 skin cancers per year or for skin cancers with a 10% 3-year risk of mortality. Severe reduction was considered warranted only for life-threatening skin cancers. CONCLUSIONS: Reduction of immunosuppression is considered a reasonable adjuvant management strategy for transplant recipients with numerous or life-threatening skin cancers. Proposed guidelines are presented for the graduated reduction of immunosuppression coincident with the increasing skin cancer risks.     

开腹及超声引导下深植入法和浅植入法建立兔肝VX2肿瘤模型的比较

目的比较开腹和超声引导下深植入法和浅植入法建立兔肝VX2肿瘤模型的成功率和异位种植率。方法选取80只新西兰白兔,按植入组织块或其悬液及穿刺深、浅分为4组,超声引导下将VX2肿瘤组织块或其悬液穿刺接种于兔左肝内（浅≤20mm,深〉20mm）,接种后第1、2、3周行超声检查。第3周检查后处死实验兔,行原位、异位肿瘤肉眼及病理检查。结果开腹及超声引导下运用深植入法及浅植入法造模,成模率均为100%。开腹组及超声组织块组肿瘤体积明显大于组织块悬液组。组织块深植入法,开腹组及超声组无异位种植。组织块浅植入法,超声组1例腹腔种植,开腹组未见腹腔种植。组织块悬液深植入法,开腹组无腹腔种植,超声组2例腹腔种植。组织块悬液浅植入法,开腹组2例腹腔种植;超声组5例腹腔种植,其中1例同时腹腔和腹壁种植。结论超声引导下穿刺组织块及组织块悬液接种制作兔肝VX2瘤模型方法简单,创伤小,成功率高。组织块穿刺植入法优于组织块悬液穿刺植入法,深植入法优于浅植入法。     

老年人慢性肾功能衰竭的临床特点─—附53例回顾分析

我院在１９８５年１月－１９９４年１２月共收治慢性肾功能衰竭（ＣＲＦ）患者１８５例，其中老年患者（≥６０岁）５３例（占２８．６％），与同期住院的非老年患者（＜６０岁）１３２例（占７１．４％）进行对比分析，发现老年人ＣＲＦ在其基础疾病，临床表现，实验室检查，并发症及伴发病方面均有许多特点，并对老年人ＣＲＦ的治疗及与预后有关的因素略加讨论。     

经皮肾镜取石术的研究现状及并发症防治策略

回顾分析了近几年有关经皮肾镜取石术(PCNL)的文献,对其在通道大小,体位摆置,定位方法,碎石方式等方面不同选择的优劣进行了总结和评价,同时从导致PCNL并发症的原因出发,探讨其防治策略的有效性。