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.

We conclude that stabilization of exogenous surfactant by adding imipramine to create a "fortified surfactant preparation" improves lung function in a clinically relevant piglet model, and that this effect can be attributed to the inhibition of a-SMase as evidenced in the mouse model.

Natriuresis and polyuria are the main diagnostic criteria for CSW. The fluid balance in CSW is negative, in contrast to a positive fluid balance in SIADH. The length of the disease is self-limited and generally ceases within 2 weeks, while Na+, K+, and fluid turnover should be monitored carefully. Only a minority of our children showed elevated ANP/BNP levels. A dose/effect relationship for natriuretic peptide levels and increased Na+ turnover could not be established.

The competitive inhibition of P. aeruginosa lectins by the lectin specific sugars galactose and fucose may overcome particular mechanisms of bacterial resistance in patients with P. aeruginosa airway infection. This underlying biochemical mechanism and the outcome of our patient suggest a clinical benefit of this novel therapeutic approach for immunocompromised patients or patients with cystic fibrosis suffering from infection with P. aeruginosa.

The emergence of the novel influenza A(H1N1)pdm09 virus may have been influenced the epidemiology of other epidemic viruses, such as the RSV and hMPV. No epidemic of seasonal influenza was observed in the 2009/2010 influenza season.

Persistent lobar atelectasis in pediatric patients on mechanical ventilation results in impaired gas exchange and lung mechanics and contributes to a further need for mechanical ventilation. The most common types of atelectasis in children are resorption atelectasis following airway obstruction, and atelectasis due to surfactant deficiency or dysfunction. We aimed to determine whether bronchoscopic suctioning and surfactant application to atelectatic lung segments would result in improved oxygenation, ventilation, chest X-ray scoring, and early extubation. Five children with heterogeneous lung diseases (aged between 7 months and 15 years) were treated with a diluted surfactant preparation (Curosurf^TM) in a concentration of 5–10 mg/ml (total dose 120–240 mg) which was instilled into the affected segments. Outcome parameters were gas exchange, radiographic resolution of atelectasis and extubation. All mechanically ventilated patients could be extubated within 24 h following the intervention. Bronchoscopic surfactant application could be carried out without adverse effects and brought improvements in oxygenation, respiratory rate, and partial or complete resolution of atelectases without recurrence.

The m-RT-PCR-ELISA evaluated here improves the spectrum for diagnosing respiratory infections and is a feasible instrument for individual diagnostic and epidemiological studies.

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