überlebensverbesserung eines unizentrischen Gesamtkollektivs aus 20 Jahren

PURPOSE: The development of overall survival of a DOSAK (German-Austrian-Swiss Cooperative Group on tumours of the maxillofacial region) clinic's overall population comprising a time period of more than 20 years (1983-2004) should be assessed. At a cutoff date (January 1st, 1997), a change from a primarily surgically based to a consequent multi-modality treatment regimen was implemented. The periods of time before and after that change should be compared. METHODS AND PATIENTS: The data of the DOSAK registry entries on 1038 patients suffering from primary untreated oral and oropharyngeal carcinomas were updated with respect to follow-up and mortality data to achieve a 100% quality of follow-up. The end point (death) was reached in 67% of the overall population. Statistical analysis was carried out by the Trium Analysis Online corporation, Munich. RESULTS: The portion of female and older tumor patients increased, more than half of all tumor patients were clearly in stage IV of the disease at first referral. The portion of patients operated on persisted approximately (80%), the portion of additional treatment modalities could be increased considerably. The fact of a bony infiltration by the tumor and the operability remained highly significantly relevant for survival in multivariate analysis, despite of multi-modality treatment. The survival rate of the patients remained significantly dependent on the clinical stage of the disease in multivariate analysis but could be improved by 10% in the clinical stages II and III and in the patients who could not be operated on. All in all, the cutoff date was statistically relevant for survival in multivariate analysis, i.[Symbol: see text]e. the change in the treatment regimen had a verifiable positive effect on the survival of a unicentric overall population. CONCLUSION: Survival improvement in an overall population via change in treatment strategy is possible in relatively short time; the clinical stages II and III and the non-operable patients have the greatest benefit from a multi-modality treatment.     

Pleural mesotheliomas and asbestos exposure in the pulp and paper industries: a new risk group identified by linkage of official registers

Analysis of data obtained by linking the 1960 Swedish Census and the Swedish Cancer Registry has demonstrated an increased risk of pleural mesothelioma among pulp and paper workers. The present study was undertaken with the aim of revealing possible environmental risk factors. The work histories of the 25 cases identified earlier were reviewed. "Certain" or "probable" exposure to asbestos was found among 70% of these workers. The study illustrates how linkage of official registers can be used to identify new risk environments and encourage the establishment of preventive measures.     

The Turner syndrome research registry: Creating equipoise between investigators and participants

To address knowledge gaps about Turner syndrome (TS) associated disease mechanisms, the Turner Syndrome Society of the United States created the Turner Syndrome Research Registry (TSRR), a patient‐powered registry for girls and women with TS. More than 600 participants, parents or guardians completed a 33‐item foundational survey that included questions about demographics, medical conditions, psychological conditions, sexuality, hormonal therapy, patient and provider knowledge about TS, and patient satisfaction. The TSRR platform is engineered to allow individuals living with rare conditions and investigators to work side‐by‐side. The purpose of this article is to introduce the concept, architecture, and currently available content of the TSRR, in anticipation of inviting proposals to utilize registry resources.     

Primary Immunodeficiency Diseases in Latin America: First Report from Eight Countries Participating in the LAGID

The Latin American Group for Primary Immunodeficiencies, formed in 1993, presently includes 12 countries. One goal was to study the frequency of primary immunodeficiencies in various regions of the American continent and to enhance knowledge about these diseases among primary-care physicians, as well as allergist–immunologists. Important for this purpose was the development of a registry of primary immunodeficiencies using a uniform questionnaire and computerized database. To date, eight countries have collected information on a total of 1428 patients. Predominantly antibody deficiencies were reported in 58% of patients, followed by cellular and antibody immunodeficiencies associated with other abnormalities in 18%, immunodeficiency syndromes associated with granulocyte dysfunction in 8%, phagocytic disorders in 9%, combined cellular and antibody immunodeficiencies in 5%, and complement deficiencies in 2% of patients. The information gathered from this initial analysis of data will serve to expand the patient database to more areas within participating countries and to new countries and to increase collaboration toward better diagnosis and treatment of these diseases.     

Primary Immunodeficiency Syndrome in Spain: First Report of the National Registry in Children and Adults

The Spanish Registry for Primary Immunodeficiency Diseases (REDIP) was organized in 1993. One thousand sixty-nine cases of primary immunodeficiency diseases (PID) were registered in patients diagnosed between January 1980 and December 1995. PID diagnosis was made according to the World Health Organization criteria. The most frequent disorders were IgA deficiency (n = 394) and common variable immunodeficiency (n = 213), followed by severe combined immunodeficiency (n = 61), C1 inhibitor deficiency (n = 52), X-Iinked agammaglobulinemia (n = 49), IgG subclass deficiency (n = 48), and chronic granulomatous disease (n = 32). A comparative study between REDIP and data recently obtained from the European registry (ESID Report, 1995) revealed important differences between phagocytic disorders and complement deficiencies reported in both registries, 4.9 vs 8.7 and 6.0 vs 3.6, while percentages of predominantly antibody deficiencies and T cell and combined deficiencies concurred with those reported in the European registry, 69.3 vs 64.7 and 14.7 vs 20.2, respectively. The heterogeneous nature of the geographical distribution of cases submitted may indicate underdiagnosis of PID in some country areas; surprisingly, the interval between the onset of clinical symptoms and diagnosis was significant, even in immunodeficiency diseases, such as IgA deficiency, which are easy to diagnose.     

河南居民常见癌症死亡率及其流行趋势分析(1974～1999)

目的 :研究河南人口常见恶性肿瘤死亡率及其流行特征。方法 :收集河南省 1974～ 1976年全人口死亡回顾调查资料和 1983～ 1999年 1/ 10人口 ( 15市县 )居民死亡登记报告资料 ,按《中国恶性肿瘤登记报告试行规范》的统计方法 ,分析常见恶性肿瘤死亡率及动态特征并对未来趋势进行预测。结果 :近 2 6年来 ,河南居民总癌死亡率变化不大 ,而食管癌则呈下降趋势 ,胃癌相对稳定 ,肝癌、肺癌上升明显 ,子宫颈癌下降很快。结论 :建立以人群为基础的癌症登记报告系统 ,对于了解和掌握恶性肿瘤流行动态及其进行肿瘤控制是非常必要的     

A novel approach to delivery of extracorporeal support using a modified continuous flow ventricular assist device in a mid‐volume congenital heart program

Extracorporeal life support (ECLS) is an essential component of a modern congenital cardiac surgery program. The circuit components and bedside management team may, however, vary among institutions. Here, we evaluate our initial experience with a modified ventricular assist device—based ECLS circuit primarily managed by the bedside nurse. We hypothesize that our outcomes are comparable to Extracorporeal Life Support Organization (ELSO) registry data. All patients who received ECLS from January 1, 2016 to December 31, 2019 at a single institution were included. Primary outcomes were survival to ECLS decannulation and discharge or transfer. Secondary outcomes included complications from ECLS. Data were compared to available ELSO registry data. Thirty‐seven patients underwent 44 ECLS runs during the study period. Forty percent of patients had single ventricle physiology. Nearly 46% of patients received ECLS as part of extracorporeal cardiopulmonary resuscitation (eCPR). Survival to ECLS decannulation (68.2%) and survival to discharge or transfer (61.4%) did not differ from overall ELSO outcomes (69.7%, P = .870 and 50.7%, P = .136), as well as survival to discharge or transfer in a comparable cohort of ELSO centers (53.1%, P = .081). Patients with complications had a lower rate of survival to discharge or transfer but this did not reach statistical significance (47.7% vs. 75.0%, P = .455). Neurologic (50.0%), hemorrhagic (45.5%), and renal complications (31.8%) were most common in this cohort. A modified ventricular assist device‐based ECLS circuit with primary management by the bedside nurse can provide comparable support in a neonatal and pediatric cardiac surgery population. Cost analyses and further delineation of the complication profile are necessary for a complete characterization of this system.     

Lung transplant waitlist outcomes in the United States and patient travel distance

There is a broad range of patient travel distances to reach a lung transplant hospital in the United States. Whether patient travel distance is associated with waitlist outcomes is unknown. We present a cohort study of patients listed between January 1, 2006 and May 31, 2017 using the Scientific Registry of Transplant Recipients. Travel distance was measured from the patient's permanent zip code to the transplant hospital using shared access signature URL access to Google Maps, and assessed using multivariable competing risk regression models. There were 22 958 patients who met inclusion criteria. Median travel distance was 69.7 miles. Among patients who traveled > 60 miles, 41.2% bypassed a closer hospital and sought listing at a more distant hospital. In the adjusted models, when compared to patients who traveled ≤60 miles, patients who traveled >360 miles had a 27% lower subhazard ratio (SHR) for waitlist removal (SHR 0.73, 95% confidence interval [CI]: 0.60, 0.89, P = .002), 16% lower subhazard for waitlist death (SHR 0.84; 95% CI 0.73-0.95, P = .07), and 13% increased likelihood for transplant (SHR 1.13, 95% CI: 1.07, 1.20, P < .001). Many patients bypassed the nearest transplant hospital, and longer patient travel distance was associated with favorable waitlist outcomes.