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

Focus Groups to Increase the Cultural Acceptability of a Contingency Management Intervention for American Indian and Alaska Native Communities

Introduction Many American Indian and Alaska Native (AI/AN) people seek evidence-based, cost-effective, and culturally acceptable solutions for treating alcohol use disorders. Contingency management (CM) is a feasible, low-cost approach to treating alcohol use disorders that uses “reinforcers” to promote and support alcohol abstinence. CM has not been evaluated among AI/AN communities. This study explored the cultural acceptability of CM and adapted it for use in diverse AI/AN communities. Methods We conducted a total of nine focus groups in three AI/AN communities: a rural reservation, an urban health clinic, and a large Alaska Native healthcare system. Respondents included adults in recovery, adults with current drinking problems, service providers, and other interested community members (n = 61). Focus group questions centered on the cultural appropriateness of “reinforcers” used to incentivize abstinence and the cultural acceptability of the intervention. Focus groups were audio-recorded, transcribed, and coded independently by two study team members using both a priori and emergent codes. We then analyzed coded data. Results Across all three locations, focus group participants described the importance of providing both culturally specific (e.g., bead work and cultural art work supplies), as well as practical (e.g., gas cards and bus passes) reinforcers. Focus group participants underscored the importance of providing reinforcers for the children and family of intervention participants to assist with reengaging with family and rebuilding trust that may have been damaged during alcohol use. Respondents indicated that they believed CM was in alignment with AI/AN cultural values. There was consensus that Elders or a well-respected community member implementing this intervention would enhance participation. Focus group participants emphasized use of the local AI/AN language, in addition to the inclusion of appropriate cultural symbols and imagery in the delivery of the intervention. Conclusions A CM intervention for alcohol use disorders should be in alignment with existing cultural and community practices such as alcohol abstinence, is more likely to be successful when Elders and community leaders are champions of the intervention, the intervention is compatible with counseling or treatment methodologies, and the intervention provides rewards that are both culturally specific and practical

Assessing the Predictive Validity of the Stages of Change Re... : Journal of Addiction Medicine

Objectives: The objective of this study was to examine the predictive validity of the Stages of Change Readiness and Treatment Eagerness Scale (SOCRATES) among Alaska Native and American Indian (ANAI) people with an alcohol use disorder. Methods: The sample was 170 ANAI adults with an alcohol use disorder living in Anchorage, Alaska who were part of a larger alcohol intervention study. The primary outcome of this study was alcohol use as measured by mean urinary ethyl glucuronide (EtG). EtG urine tests were collected at baseline and then up to twice a week for four weeks. We conducted bivariate linear regression analyses to evaluate associations between mean EtG value and each of the three SOCRATES subscales (Recognition, Ambivalence, and Taking Steps) and other covariates such as demographic characteristics, alcohol use history, and chemical dependency service utilization. We then performed multivariable linear regression modeling to examine these associations after adjusting for covariates. Results: After adjusting for covariates, mean EtG values were negatively associated with the Taking Steps (P¼0.017) and Recognition (P¼0.005) subscales of the SOCRATES among ANAI people living in Alaska. We did not find an association between mean EtG values and the Ambivalence subscale (P¼0.129) of the SOCRATES after adjusting for covariates. Conclusions: Higher scores on the Taking Steps and Recognition subscales of the SOCRATES at baseline among ANAI people predicted lower mean EtG values. This study has important implications for communities and clinicians who need tools to assist ANAI clients in initiating behavior changes related to alcohol use