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Ten new species belonging to three new genera (Atlantisina gen. nov., Bathycyclopora gen. nov., Calvetopora gen. nov.) of umbonulomorph bryozoans from northeastern Atlantic seamounts, islands, and the continental slope are introduced. We furthermore erect the new family Atlantisinidae fam. nov. for these genera. Eight new species belong to the new genus Atlantisina: Atlantisina atlantis gen. et sp. nov. (type species), A. acantha gen. et sp. nov., A. gorringensis gen. et sp. nov., A. inarmata gen. et sp. nov., A. lionensis gen. et sp. nov., A. meteor gen. et sp. nov., A. seinensis gen. et sp. nov., and A. tricornis gen. et sp. nov. The genus Bathycyclopora gen. nov. is introduced for ?Phylactella vibraculata Calvet from the Azores, and also includes Bathycyclopora suroiti gen. et sp. nov. The type species of Calvetopora gen. nov. is Lepralia inflata Calvet from the Gulf of Cadiz; this genus also includes Calvetopora otapostasis gen. et sp. nov. and another species left in open nomenclature. Of the 13 species described herein, 11 occur on seamounts and islands, and nine species are endemic to a single seamount, island or station. The present results show that bryozoans provide striking examples of the function of seamounts as areas of endemism, most likely intrinsically linked to the low dispersal abilities of bryozoan larvae.
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.