Haemophilia A home therapy in the United Kingdom 1975-6.

Data on home treatment for patients with haemophilia A (factor VIII deficient haemophilia) were compiled for 1975 and 1976 from questionnaires answered by directors of haemophilia centres throughout the United Kingdom. There were 48 haemophilia centres in 1975 and 71 in 1976. The number of patients on or in training for home treatment increased from 267 to 488 in the two years, and a further 241 haemophiliacs were considered suitable for home therapy by the end of 1976. Apart from a small (but increasing) number of haemophiliacs on prophylactic treatment, most patients were on low-dose (250-500 units) on-demand regimens, using a mean of 20 000 factor VIII units per patient per year in 1976. An estimated 55% of the blood product used for home therapy in the UK in 1976 was imported from commercial sources. Despite the fact that the numbers of patients on home treatment have increased, so that about 60% of the potential population were receiving or being considered for home treatment in 1976, inadequacies in the service still remain. In some centres follow-up is clearly inadequate; about 15% of patients still rely on cryoprecipitate; and too little money has been invested in making the NHS self-sufficient in factor VIII production.     

Recommendations for a medical-meteorologic prognosis for hemophilia]

A one year lasting study about the relation between bleedings in haemophiliacs and weather was performed. There had been included 1802 bleeding events. The influence of meteorologic factors to bleeding was proved. The influx of warm air proved inopportune. The decrease in atmospheric pressure as well as winter cold fronts lead to increase of bleeding tendency. Haemophiliacs should avoid physical and psychical stress in such unconvenient atmospheric situations. The replacement therapy should be adapted to the weather situation.     

Trends in incidence of AIDS associated with transfusion of blood and blood products in Europe and the United States, 1985-93.

OBJECTIVE--To quantify patterns and trends in incidence of AIDS associated with transfusion of blood and its products in 14 European countries and the United States. DESIGN--Data were derived from the World Health Organisation''s European non-aggregate AIDS dataset and, for the United States, from the Centers for Disease Control AIDS public information dataset. Rates were standardised by using the world standard populations and adjusted for reporting delays in each country. SUBJECTS--Cases of AIDS in patients with haemophilia and recipients of transfusions. RESULTS--Overall, between 1985 and 1993 almost 6000 cases of AIDS associated with transfusions were registered in the 14 European countries considered and over 8000 in the United States between 1985 and 1992. Most European countries had annual age adjusted rates lower than 0.5 per million children aged 12 or less and between 1 and 2 per million adults. The United States had rates around 1 per million children and 5 per million adults in the most recent period. For children, the highest rates were generally reached in 1985-7, whereas in adults the highest rates were in the late 1980s. France had the highest overall incidence of AIDS related to transfusion in Europe (3.3 per million). Romania had a major epidemic in children (over 30 cases per million children in 1988-90). Incidence rates of AIDS associated with transfusion were still increasing in some southern European countries in the early 1990s. CONCLUSIONS--Apart from in France and Romania it is clear that rates of bloodborne AIDS in European countries are lower than those registered in the United States.     

Treatment of haemophilia and related disorders in Britain and Northern Ireland during 1976-80: report on behalf of the directors of haemophilia centres in the United Kingdom.

A five year survey of the treatment of patients in the United Kingdom suffering from haemophilia and related disorders was carried out on behalf of the directors of haemophilia centres. The survey showed an increase in the number of patients receiving treatment from the centres, a substantial increase in the total amount of therapeutic materials used, and an increase in the average amount of factor VIII or factor IX used yearly per patient. Home treatment became established for severely affected patients and accounted for roughly half of the total amount of material used. Study of the acquisition of factor VIII or factor IX antibodies (inhibitors) in patients with haemophilia A or haemophilia B showed no increase in antibodies during the survey period, despite the increased use of factor VIII and factor IX concentrates. The occurrence of acute hepatitis in treated patients was also studied and no increased incidence was observed. A near normal median expectation of life in patients with severe haemophilia A was found.     

CIRRHOSIS OF THE LIVER—Clinical Course in 2,377 Patients at San Francisco General Hospital

The records of 2,377 patients with Laennec''s cirrhosis were reviewed for the period 1947-1957. The chief presenting symptom was ascites in 46 per cent, bleeding in 23 per cent, coma in 18 per cent, jaundice in 9 per cent, and both jaundice and ascites in 4 per cent. Nearly half of the patients died during the period under study—one-third from hepatic failure, one-third from gastrointestinal bleeding, and one-third from other causes, most of which were related to alcoholism.Massive gastrointestinal bleeding occurred in 21 per cent of the patients at some time in their clinical course, and in the 10 per cent of these in whom ulcer was demonstrated, one-fifth died as a result of the hemorrhage. Of those presumed to be bleeding from esophageal varices, 64 per cent died at the first hemorrhage and 10 per cent at subsequent hemorrhages; 85 per cent of all those who bled from varices were dead at the end of one year, and 91 per cent were dead at the end of three years.The survival curve of a group of patients who bled once and were good operative risks but had received no operative treatment was compared to the survival curve for entire group who survived the first hemorrhage. The three-year survival in the good risk group was 47 per cent; for the group as a whole it was 30 per cent. The difference in mortality rate was primarily due to an increased number of deaths from hepatic failure in the combined group, whereas 60 per cent of the good risk group died of recurrent gastrointestinal hemorrhage.As 86 per cent of those who were to die of gastrointestinal bleeding did so at the first hemorrhage, it was concluded that any decided improvement in the salvage rate achievable by operation must come from some means of diagnostic forecast of the likelihood of bleeding, with resort to prophylactic operation in such cases.     

LEEP治疗宫颈上皮内瘤变疗效的临床研究

目的：探讨LEEP在宫颈上皮内瘤变（CIN）临床应用的安全性及有效性。方法：回顾性分析45例CIN患者治疗的临床资料。结果：45例LEEP手术过程中,患者无特殊不适,手术时间平均10±5 min,术中出血平均15±5 mL。术后病理报告与术前诊断相比完全符合18例,一致率为40%;病理升级13例,比率为28.89%;病理降级14例,比率为31.11%。其中16例LEEP患者再次行筋膜外子宫全切术,术后病检均未见到CIN的残留。1年后随访发现,16例经LEEP及筋膜外子宫全切术后,复查无异常,达到治愈。29例经单纯LEEP治疗后,27例治愈,2例治疗无效,再次行LEEP手术及药物治疗好转。结论：LEEP治疗CIN,安全有效,价格实惠,手术时间短,患者疼痛小,出血少,并且能够保留完整、连续的标本进行病检,明显降低CIN的漏诊率,值得临床应用。     

磺达肝癸钠应用于冠心病合并肾功能不全患者的临床研究

目的:观察磺达肝癸钠应用于冠心病急性冠脉综合征合并肾功能不全患者抗凝治疗的疗效及安全性。方法:216例急性冠脉综合征患者分为肾功能正常组及肾功能不全组,皮下注射磺达肝癸钠2.5毫克,一日一次,连用8天,比较两组治疗9天内主要不良心脏事件(MACE)及出血并发症的发生率和30天及180天的随访情况。结果:治疗9天内及随访30天、180天期间,两组患者主要不良心脏事件发生率无统计学差异(P0.05),治疗及随访期间两组均未出现严重出血及中度出血,肾功能不全组轻微出血8例,肾功能正常组轻微出血5例,两组间比较差异无统计学意义(P0.05)。结论:磺达肝癸钠对于冠心病合并肾功能不全患者的抗凝治疗是一种安全有效的药物。     

Whole blood transfusion in common marmosets: a clinical evaluation

In veterinary medicine, blood transfusion is commonly performed on companion animals. The common marmoset is a small nonhuman primate with increasing popularity as an animal model in biomedical research. Because of its small whole blood volume, the marmoset is at high risk of exsanguination, and blood transfusion is required to care for life-threatening bleeding. However, few clinical evaluations exist on transfusions for marmosets. This study performed whole blood transfusion with cross-matching on nine marmosets and surveyed the therapeutic effects. Recipients included clinical cases with persistent bleeding, anemia, and coma, as well as animals subjected to postoperative bleeding prophylaxis. Donors were selected from healthy marmosets, including littermates. Cross-match assay before transfusion were all negative, and recipients showed no visible signs of transfusion-related adverse reactions. Whole blood transfusions caused hemostasis and successful recovery in bleeding marmosets, including long-term improvement of anemia cases. Our results indicated that blood transfusion is effective for marmosets with severe anemia and persistent hemorrhage from both non-experimental and surgical causes. Furthermore, DNA sequencing for blood-group classification revealed that all subject marmosets were type A, suggesting that the risk of blood type mismatch may be low in this species.     

Incidence of hepatitis B in hemophilia patients

Hepatitis represents a serious complication in the transfusion of blood and blood products. In examining patients from the haemophilia centre in Jena HBsAg was identified in no case, HBsAK, however, in 80% of them. The rate of hepatitis infection increased here with the increasing frequency of transfusion. It should be determined by regularly checking HBsAG, HBsAK and transferases within the scope of prophylactic examinations.     

Patterns of bleeding in adolescents with severe haemophilia A.

Eighty-two boys with severe haemophilia A who spent some time at Lord Mayor Treloar College during 1973-7 were studied. All episodes of bleeding that occurred during term time were recorded, along with the number of transfusions. The bleeding frequency among these boys, most of them aged 10-17 years, increased steadily from 8,31 episodes/100 days in 1973 to 12,63 episodes/100 days in 1977. At the same time there was a steady fall in bleeding frequency with age. Altogether 24% of bleeding episodes were into the elbow joint, 22% into the knee, and 15% into the ankle. As the boys grew older the proportion of bleeding episodes in the legs declined and that in the arms increased. The overall results reflect the fact that special schools now see only the severest cases of haemophilia. The pattern of bleeding during adolescence suggests that concepts of management of arm bleeding need modifying.     

Post‐marketing observational study on 5% intravenous immunoglobulin therapy in patients with secondary immunodeficiency and recurrent serious bacterial infections

Secondary hypogammaglobulinemia is one of the factors responsible for the increased susceptibility to infection in patients with chronic lymphocytic leukemia (CLL). This study assessed the therapeutic results, concomitant medication and tolerance of administering 5% intravenous immunoglobulin, secondary immunodeficiency and recurrent serious bacterial infections. A single center, post‐marketing, observational clinical study was performed on 10 patients with a variety of hematological malignancies (CLL, follicular non‐Hodgkin lymphoma, IgM‐secreting immunocytoma, IgA plasmacytoma and myelodysplastic syndrome/non‐Hodgkin lymphoma) who had been infused with IVIG from June 1994 to May 2009. The clinical benefit of IVIG was assessed by comparing the incidence of bacterial infections before and after starting this therapy. Plasma immunoglobulin concentrations and relevant hematological variables were recorded. For safety assessment, adverse events were monitored. The standard IVIG dosage was approximately 0.35 g/kg body weight every 3–4 weeks. Most patients had normal IgG trough values of >600 mg/dL during the IVIG treatment period. The rate of bacterial infections was reduced from 2.4 per patient in the 3 months before IVIG to 0.7 (0–1.5) per patient per year during IVIG treatment. All patients received concomitant medication, mainly anticancer and anti‐anemia therapy (100%). No serious adverse events related to IVIG were observed. The frequency of at least one minor adverse reaction was 1.44% (8/556 infusions). In conclusion, the investigated IVIG preparation was well tolerated and clinically beneficial in reducing the long term rate of serious bacterial infections in patients receiving concomitant treatment for malignant diseases.     

Cutaneous malignant melanoma in Scotland: incidence,survival, and mortality, 1979-94. The Scottish Melanoma Group.

OBJECTIVE: To determine the changing incidence of and mortality from cutaneous malignant melanoma in Scotland from 1979 to 1994. DESIGN: Detailed registration of clinical and pathological features, surgical and other treatment, and follow up of all cases of cutaneous malignant melanoma diagnosed from 1979 to 1994 and registered with specialist database for Scotland. SETTING: Scotland. SUBJECTS: 6288 patients with invasive primary cutaneous malignant melanoma diagnosed between 1 January 1979 and 31 December 1994. RESULTS: The annual age standardised incidence of cutaneous malignant melanoma rose significantly from 3.5 to 7.8 per 100,000 per year in men and from 6.8 to 12.3 per 100,000 per year in women (P < 0.001 for both). World standardised rates increased from 2.7 to 6.0 per 100,000 per year in men and 4.6 to 8.50 per 100,000 in women. The incidence of melanoma continued to increase significantly in men of all ages during the study, but the rate stabilised in women after 1986. Mortality from cutaneous malignant melanoma was 1.3 per million per annum in men in 1979, rising to 2.3 per million per annum in 1994 (P < 0.01); it was 2.4 per million per annum in women in 1979, falling to 1.9 per million per annum in 1994 (P = 0.09). The underlying mortality trends showed a continuing rise for men but a downward trend for women that was not significant (P = 0.09). In men, melanoma free survival was 69% at 5 years and 61% at 10 years; in women the corresponding rates were 82% and 75%. Younger patients had higher survival rates, which were not entirely explained by thinner tumours. Over the 15 year period, survival rates improved by 12% overall, only partly owing to thinner tumours. CONCLUSIONS: In Scotland the incidence of melanoma in women has stabilised, while mortality associated with melanoma in women shows a downward trend.     

Recurrent mutations in the factor IX gene: founder effect or repeat de novo events

The investigation of 114 unrelated patients, representing about half the sample of the German haemophilia B population, enabled us to delineate the causative mutation in 103 (90.4%) haemophilic factor IX genes. Of these 103 cases 84 (81.6%) turned out to be unique molecular events, the remainder being repeats. Haplotype analysis revealed that the great majority, if not all, of these recurrent observations occurred independently. This conclusion is supported by our finding that three de novo mutations could be demonstrated at two sites of frequent mutation. A further 20 de novo events could be established in an unselected sample of 37 families with sporadic haemophilia B and 37 families with a history of the disease. Altogether, the germ line of origin could be determined in 21 of these 23 cases, thereby indicating a ratio of male to female mutation rates close to 2. On the basis of the data available, it is becoming clear that rearrangements in the factor IX gene (35.4% of de novo cases) are responsible for haemophilia B at a higher frequency than has been observed today (12.3%). More than two-thirds of the de novo cases cause the severe form of the disease, thereby reflecting the deficit of these haemophilic genes in the actual gene pool because of excess mortality in the past. In addition 40% (12/30) of the de novo single-base mutations were transitions at CpG dinucleotides. Compared with the expected at-random frequency, this observation indicates an 83-fold enhancement of mutation at CpG.     

内镜下喷洒蛇毒血凝酶与金属钛夹治疗不明原因消化道出血的临床疗效比较

目的:探讨内镜下喷洒蛇毒血凝酶与金属钛夹治疗不明原因消化道出血的临床疗效。方法:选取2013年8月至2015年12月本院收治的不明原因消化道出血患者82例,随机分为对照组和实验组,每组41例。对照组给予内镜下喷洒蛇毒血凝酶治疗,实验组给予金属钛夹止血治疗。观察两组患者经止血治疗后即时止血时间、外科手术率、输血量、症状消失时间和临床疗效、再出血率和出血量。结果:实验组即时止血率明显高于对照组,输血量低于对照组(P0.05);实验组呕血消失时间、潜血转阴时间和引流液变清时间均小于对照组(P0.05);实验组痊愈率、总有效率高于对照组(P0.05);两组之间再出血率和出血量比较差异无统计学意义(P0.05)。结论:金属钛夹较内镜下喷洒蛇毒血凝酶对不明原因消化道出血具有更好的临床疗效,且再出血率较低。     

Joint status after prophylactic substitution. An investigation in hemophilic children and adolescents in the DDR]

The joint condition in 98 children and adolescents with severe or moderate types of haemophilia participating in a summer camp between 1987 and 1989 had been analyzed. This was done by the centers' report and on the other hand with testing the patients by aid of the score system recommended by the World Federation of Hemophilia. According to the centers' reports more than 50% of all patients seem to be free of joint contractures. But according to the more sensitive score system more patients are affected by discrete symptoms of the chronic arthropathy. There had been discovered big differences between the centers and an increase of the patient's score by age. Not seldom an impairment of the findings within a single year was recognized. The joint problems in haemophiliacs are solved by the prophylactic treatment in an incomplete manner only. Lacks in the sophisticated evaluation of joints, with the management of prophylactic treatment as well as adjustment to prevention lead to avoidable damage crippling the patient on later age.     

Regional transfusion centre preoperative autologous blood donation programme: the first two years.

OBJECTIVE--To assess the efficacy of a regional autologous blood donation programme. DESIGN--Clinical and laboratory data were collected and stored prospectively. Transfusion data were collected retrospectively from hospital blood bank records. SETTING--Northern Region Blood Transfusion Service and 14 hospitals within the Northern Regional Health Authority. SUBJECTS--505 patients referred for autologous blood donation before elective surgery. MAIN OUTCOME MEASURES--Patient eligibility, adverse events from donation, autologous blood units provided, and autologous and allogeneic blood units transfused within 10 days of operation. RESULTS--Of 505 patients referred, 354 donated at least one unit. 78 of 151 referred patients who did not donate were excluded at the autologous clinic, mostly because of anaemia or ischaemic heart disease. In 73 cases the patient, general practitioner, or hospital consultant decided against donation. 363 autologous procedures were undertaken. In 213 (59%) cases all requested units were provided. The most common reasons for incomplete provision were late referral or anaemia. Adverse events accompanied 24 of 928 donations (2.6%). Transfusion data were obtained for 357 of the 363 procedures. 281 donors were transfused; autologous blood only was given to 225, autologous and allogeneic blood was given to 52, and allogeneic blood only was given to four. 648 of 902 (72%) units of autologous blood were transfused. Complete provision of requested autologous units was followed by allogeneic transfusion in 12 of 208 procedures (5.8%). Incomplete provision was followed by allogeneic transfusion in 44 of 149 procedures (30%). CONCLUSIONS--This study shows the feasibility of a regional autologous transfusion programme. Autologous donors only infrequently received allogeneic transfusion. Patients should be appropriately selected and referred early.     

Incidence of advanced chronic renal failure and the need for end stage renal replacement treatment.

OBJECTIVE--To determine the age related incidence of advanced chronic renal failure in two areas of England. DESIGN--Prospective study of patients newly identified as having advanced chronic renal failure within a two year period; subsequent monitoring of patients'' clinical course for a further 26 months. SETTING--Devon and Blackburn. SUBJECTS--Those patients in a population of 708,997 who developed advanced chronic renal failure (serum creatinine concentration greater than 500 mumol/l) for the first time during a two year period. MAIN OUTCOME MEASURES AND RESULTS--210 Patients (148 per million population per year) developed advanced chronic renal failure, 117 (51%) of whom were over 70. The age related incidence rose from 58 per million per year in those aged 20-49 to 588 per million per year in those aged 80 or over. Only 54% (113) of patients were referred to a nephrologist; 120 patients (57%) needed dialysis or died within three months of presenting without receiving dialysis, and 187 (89%) died or needed dialysis within three years. After those unsuitable for further treatment had been excluded, 78 patients per million population per year aged under 80 needed to start long term renal replacement treatment. CONCLUSIONS--Many patients suitable for renal replacement treatment are still not referred for nephrological opinion and are denied treatment. If the treatment rate in the United Kingdom rose from the 1988 rate of 55.1 per million per year to 78 per million per year then the number of patients receiving treatment would rise to about 800 per million. This is double the present number and has considerable but predictable resource implications for the NHS.     

微创经皮肾穿刺碎石术（MPCNL）治疗上尿路结石的临床分析

目的：探讨微创经皮肾穿刺碎石术（MPCNL）用于治疗尿路结石的临床效果。方法：选取120例单侧上尿路结石病患者,随机分为两组,每组60人。一组应用微创经皮肾穿刺碎石术（MPCNL）进行一期单通道上尿路取石,另一组采用开放手术治疗。比较并分析两组患者的临床疗效。结果：应用MPCNL治疗的60名患者中,结石清除的有55名,清除率为91．17％,手术时间平均为77分钟,住院时间平均为5-3天。术中平均出血100mL,术后发生大出血者一例,经输血后好转,术后发热者38例,发热比例为63-3％,尿液转清时间平均为2．5天。应用开放式手术的60名患者中,结石清除的有39例,清除率为65．0％,平均手术之间为112分钟,住院时间平均为18．1天,术中平均出血380mL,术后发生大出血者9例,经输血后好转,术后发热者43例,发热比例为71．2％,尿液转清时间平均为8．6天。结论：MPCNL方法治疗上尿路结石的效果明显比开放式手术好,具有清除率高、手术时间短、术后并发症少、术后感染少以及患者恢复快的优点。     

Clinical outcomes of long coronary stents: a single-center experience

BACKGROUND: Coronary artery stenting is particularly useful during percutaneous coronary intervention for long lesions previously associated with a low procedural success rate and a high complication rate of dissection and occlusion. Current treatment options include implantation of a single long stent, multiple contiguous stents, or 'spot' stenting. However, multiple stent implantation may result in sections of overlapping stent or gaps of unstented segments and is an independent predictor of restenosis. The early and intermediate clinical outcome of single and multiple long stent (>/= 30 mm) implantation is not established. METHODS AND RESULTS: The authors retrospectively identified 123 consecutive patients who had undergone stenting using one or more long coronary stents. Baseline clinical data, procedural outcomes and completed clinical follow-up to 52 weeks were obtained by case-note review. The majority (69%) required intervention for stable coronary disease. Seventy-seven per cent of lesions were either type B2 or C and only 2% were in saphenous vein grafts. The procedural success rate was 94%. A total of 15 major events occurred in 13 patients (11%). Ten acute events occurred and five events were during the follow-up period from 30 days to 52 weeks. Two patients died, one from uncontrolled bleeding secondary to the use of antithrombotic agents and one at four weeks due to sudden death. One patient had a postprocedural infarct. Two patients required in-hospital repeat revascularization for acute vessel closure and eight required revascularization during follow-up (three cases of occlusion/thrombosis and five cases of restenosis). CONCLUSIONS: The use of long coronary stents (>/= 30 mm) for the treatment of long diffuse native vessel disease, saphenous vein graft disease and long coronary dissections is associated with a reasonable procedural success rate and acceptable early and intermediate-term clinical outcomes.