The German National Registry of Primary Immunodeficiencies (2012-2017)

Introduction: The German PID-NET registry was founded in 2009, serving as the first national registry of patients with primary immunodeficiencies (PID) in Germany. It is part of the European Society for Immunodeficiencies (ESID) registry. The primary purpose of the registry is to gather data on the epidemiology, diagnostic delay, diagnosis, and treatment of PIDs. Methods: Clinical and laboratory data was collected from 2,453 patients from 36 German PID centres in an online registry. Data was analysed with the software Stata® and Excel. Results: The minimum prevalence of PID in Germany is 2.72 per 100,000 inhabitants. Among patients aged 1–25, there was a clear predominance of males. The median age of living patients ranged between 7 and 40 years, depending on the respective PID. Predominantly antibody disorders were the most prevalent group with 57% of all 2,453 PID patients (including 728 CVID patients). A gene defect was identified in 36% of patients. Familial cases were observed in 21% of patients. The age of onset for presenting symptoms ranged from birth to late adulthood (range 0–88 years). Presenting symptoms comprised infections (74%) and immune dysregulation (22%). Ninety-three patients were diagnosed without prior clinical symptoms. Regarding the general and clinical diagnostic delay, no PID had undergone a slight decrease within the last decade. However, both, SCID and hyper IgE- syndrome showed a substantial improvement in shortening the time between onset of symptoms and genetic diagnosis. Regarding treatment, 49% of all patients received immunoglobulin G (IgG) substitution (70%—subcutaneous; 29%—intravenous; 1%—unknown). Three-hundred patients underwent at least one hematopoietic stem cell transplantation (HSCT). Five patients had gene therapy. Conclusion: The German PID-NET registry is a precious tool for physicians, researchers, the pharmaceutical industry, politicians, and ultimately the patients, for whom the outcomes will eventually lead to a more timely diagnosis and better treatment

Older patients' perceived burdens of their health problems: a cross-sectional analysis in 74 German general practices

BACKGROUND Older patients often experience the burden of multiple health problems. Physicians need to consider them to arrive at a holistic treatment plan. Yet, it has not been systematically investigated as to which personal burdens ensue from certain health conditions. OBJECTIVE The objective of this study is to examine older patients' perceived burden of their health problems. PATIENTS AND METHODS The study presents a cross-sectional analysis in 74 German general practices; 836 patients, 72 years and older (mean 79±4.4), rated the burden of each health problem disclosed by a comprehensive geriatric assessment. Patients rated each burden using three components: importance, emotional impact, and impact on daily activities. Cluster analyses were performed to define patterns in the rating of these components of burden. In a multilevel logistic regression analysis, independent factors that predict high and low burden were explored. RESULTS Patients had a median of eleven health problems and rated the burden of altogether 8,900 health problems. Four clusters provided a good clustering structure. Two clusters describe a high burden, and a further two, a low burden. Patients attributed a high burden to social and psychological health problems (especially being a caregiver: odds ratio [OR] 10.4, 95% confidence interval [CI] 4.4-24.4), to specific symptoms (eg, claudication: OR 2.3, 95% CI 1.3-4.0; pain: OR 2.3, 95% CI 1.6-3.1), and physical disabilities. Patients rated a comparatively low burden for most of their medical findings, for cognitive impairment, and lifestyle issues (eg, hypertension: OR 0.2, 95% CI 0.2-0.3). CONCLUSION The patients experienced a relatively greater burden for physical disabilities, mood, or social issues than for diseases themselves. Physicians should interpret these burdens in the individual context and consider them in their treatment planning

Disclosure of New Health Problems and Intervention Planning Using a Geriatric Assessment in a Primary Care Setting

Aim To determine the prevalence of health problems uncovered by a Standardized Assessment for Elderly Patients in a Primary Care Setting (STEP), to explore how often STEP uncovered conditions new to general practitioners (GP) and ascertain how often STEP results led GPs to plan further interventions. Methods This descriptive, interim analysis was based on the data of 189 elderly patients (median age, 78 years; interquartile range [IQ], 74-81) and their 20 GPs collected in Hannover region, Germany, between June 2008 and April 2009. Study nurses in the practice setting applied the 44- item STEP instrument, based mainly on self-reporting, as well as a standardized patient interview. Subsequently, GPs indicated whether the problems were new to them, and whether they planned further action or health interventions on the basis of the problems identified by STEP. Results A median of 11 health problems (IQ, 8-14) were uncovered per patient, of which a median of 2 (IQ, 1-4) were new to the GP and interventions were planned for a median of 2 problems (IQ, 0-4). Many of the identified health problems are typical of old age. The following health problems uncovered by STEP were often new to the GPs (percentages differ to numbers due to missing GP ratings): cognitive impairment (33 of 64 affected by this problem, 73%), missing or unknown immunization status (84 of 160, 55%), and recent chest pain (19 of 37, 53%). Alcohol misuse was new in all 4 affected patients (100%) and recent falls were new in 5 of 7 patients (83%). Interventions for affected patients were frequently planned for problems of immunization (for 83 patients of 160 reporting the problem, 57%), current anxiety (4 of 9, 50%), and chest pain (14 of 37, 44%). Moreover, further management was frequently planned for depression (10 of 29, 39%) and cognitive impairment (16 of 64, 38%). Conclusion Using a geriatric assessment in primary care discloses relevant heath problems and treatment needs that GPs may otherwise overlook

Eine Untersuchung zum Erziehungsverhalten (Permissivitaet) Einstellungs-Verhaltens- und Verhaltens-Verhaltens-Konsistenz in Abhaengigkeit von Self-Monitoring, sozialem Einfluss und Einstellungs-Verfuegbarkeit

UuStB Koeln(38)-850106569 / FIZ - Fachinformationszzentrum Karlsruhe / TIB - Technische InformationsbibliothekSIGLEDEGerman

Versorgung von rheumatologischen Patienten während des Lockdowns im Frühjahr 2020

Background!#!Telemedicine was implemented in outpatient care during the lockdown between March and May 2020. The aim of the study was to assess patients from a private practice and the university outpatient department with respect to patient satisfaction with telemedicine, COVID-19 worries and vaccination behavior and to compare the teleconsultation by a medical assistant for rheumatology (RFA) and a physician.!##!Methods!#!Patients with rheumatoid arthritis, psoriatric arthropathy or spondylarthritis without treatment modifications since the previous presentation were offered a telemedical replacement appointment within the framework of this study in the case of appointment cancellation by the treating center. Participants were randomized to a telemedicine appointment by a physician or an RFA (RFA university only). The patient history was carried out by telephone and standardized using a questionnaire. The disease activity was determined using the modified clinical disease activity score (CDAI) and the BASDAI. Subsequently, all patients received a pseudonymized evaluation questionnaire.!##!Results!#!In total 112/116 (96%) patients participated. Of these 88/112 (79%) returned the questionnaire. The RFAs conducted 19/112 (17%) of the telephone calls. The treatment was modified in 19/112 (17%) patients. Concerns about contracting COVID-19 correlated with high disease activity (p = 0.031) including the presence of painful joints (p = 0.001) and high pain levels (VAS ≥7, p = 0.009). These patients would have also cancelled their appointment themselves (p = 0.015). Patient satisfaction with the consultation was good (mean 4.3/5.0 modified FAPI) independent of the institution, the duration of the consultation and the consultation partner. Patients with a high pain intensity were the least satisfied (p = 0.036). Only 42/100 (38.2%) of the patients had been vaccinated against pneumococci and 59/100 (53.6%) against influenza.!##!Conclusion!#!Telemedical care within the framework of a telephone consultation is well-suited for selected patients. With respect to patient satisfaction the delegation of a telemedical consultation to an RFA is possible. There is a need for improvement with respect to the vaccination behavior

CT or Invasive Coronary Angiography in Stable Chest Pain.

Background: In the diagnosis of obstructive coronary artery disease (CAD), computed tomography (CT) is an accurate, noninvasive alternative to invasive coronary angiography (ICA). However, the comparative effectiveness of CT and ICA in the management of CAD to reduce the frequency of major adverse cardiovascular events is uncertain. Methods: We conducted a pragmatic, randomized trial comparing CT with ICA as initial diagnostic imaging strategies for guiding the treatment of patients with stable chest pain who had an intermediate pretest probability of obstructive CAD and were referred for ICA at one of 26 European centers. The primary outcome was major adverse cardiovascular events (cardiovascular death, nonfatal myocardial infarction, or nonfatal stroke) over 3.5 years. Key secondary outcomes were procedure-related complications and angina pectoris. Results: Among 3561 patients (56.2% of whom were women), follow-up was complete for 3523 (98.9%). Major adverse cardiovascular events occurred in 38 of 1808 patients (2.1%) in the CT group and in 52 of 1753 (3.0%) in the ICA group (hazard ratio, 0.70; 95% confidence interval [CI], 0.46 to 1.07; P = 0.10). Major procedure-related complications occurred in 9 patients (0.5%) in the CT group and in 33 (1.9%) in the ICA group (hazard ratio, 0.26; 95% CI, 0.13 to 0.55). Angina during the final 4 weeks of follow-up was reported in 8.8% of the patients in the CT group and in 7.5% of those in the ICA group (odds ratio, 1.17; 95% CI, 0.92 to 1.48). Conclusions: Among patients referred for ICA because of stable chest pain and intermediate pretest probability of CAD, the risk of major adverse cardiovascular events was similar in the CT group and the ICA group. The frequency of major procedure-related complications was lower with an initial CT strategy. (Funded by the European Union Seventh Framework Program and others; DISCHARGE ClinicalTrials.gov number, NCT02400229.)