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.
First measurement of 𝚲+c production down to 𝑝T = 0 in pp and p–Pb collisions at √𝑠NN = 5.02 TeV
(2023)
The production of prompt Λ+c baryons has been measured at midrapidity in the transverse momentum interval 0<pT<1 GeV/c for the first time, in pp and p-Pb collisions at a centre-of-mass energy per nucleon-nucleon collision sNN−−−√=5.02 TeV. The measurement was performed in the decay channel Λ+c→pK0S by applying new decay reconstruction techniques using a Kalman-Filter vertexing algorithm and adopting a machine-learning approach for the candidate selection. The pT-integrated Λ+c production cross sections in both collision systems were determined and used along with the measured yields in Pb-Pb collisions to compute the pT-integrated nuclear modification factors RpPb and RAA of Λ+c baryons, which are compared to model calculations that consider nuclear modification of the parton distribution functions. The Λ+c/D0 baryon-to-meson yield ratio is reported for pp and p-Pb collisions. Comparisons with models that include modified hadronisation processes are presented, and the implications of the results on the understanding of charm hadronisation in hadronic collisions are discussed. A significant (3.7σ) modification of the mean transverse momentum of Λ+c baryons is seen in p-Pb collisions with respect to pp collisions, while the pT-integrated Λ+c/D0 yield ratio was found to be consistent between the two collision systems within the uncertainties.
First measurement of 𝚲+c production down to 𝑝T = 0 in pp and p–Pb collisions at √𝑠NN = 5.02 TeV
(2023)
The production of prompt Λ+c baryons has been measured at midrapidity in the transverse momentum interval 0<pT<1 GeV/c for the first time, in pp and p-Pb collisions at a centre-of-mass energy per nucleon-nucleon collision sNN−−−√=5.02 TeV. The measurement was performed in the decay channel Λ+c→pK0S by applying new decay reconstruction techniques using a Kalman-Filter vertexing algorithm and adopting a machine-learning approach for the candidate selection. The pT-integrated Λ+c production cross sections in both collision systems were determined and used along with the measured yields in Pb-Pb collisions to compute the pT-integrated nuclear modification factors RpPb and RAA of Λ+c baryons, which are compared to model calculations that consider nuclear modification of the parton distribution functions. The Λ+c/D0 baryon-to-meson yield ratio is reported for pp and p-Pb collisions. Comparisons with models that include modified hadronisation processes are presented, and the implications of the results on the understanding of charm hadronisation in hadronic collisions are discussed. A significant (3.7σ) modification of the mean transverse momentum of Λ+c baryons is seen in p-Pb collisions with respect to pp collisions, while the pT-integrated Λ+c/D0 yield ratio was found to be consistent between the two collision systems within the uncertainties.