TY - JOUR A1 - von Lukowicz, Hannah A1 - Schlegel, Paul-Gerhardt A1 - Härtel, Christoph A1 - Morbach, Henner A1 - Haubitz, Imme A1 - Wiegering, Verena T1 - ESPED survey on newly diagnosed immune thrombocytopenia in childhood: how much treatment do we give? JF - Molecular and Cellular Pediatrics N2 - Background Immune thrombocytopenia (ITP) is an autoimmune disease associated with isolated thrombocytopenia, which is caused by an imbalance between platelet production and platelet destruction. Petechial and mucous membrane hemorrhages are characteristic of ITP, but life-threatening bleeding rarely occurs. Depending on the bleeding symptoms, ITP can be treated with glucocorticoids (GC), intravenous immunoglobulins (IVIG), or in severe cases, platelet transfusions. Mild bleeding does not necessarily require therapy. Using the German Surveillance Unit for rare Pediatric Diseases (ESPED) we conducted a prospective survey on ITP patients in all German Children's Hospitals between September 2018 and August 2019. We collected data on ITP, including the clinical course, therapy implementation recommendations (according to the Association of German Scientific Medical Societies guidelines), outcome, and influence of treatment regimens depending on the treating physician´s experience with ITP patients. Results Of the 287 recorded cases of children with ITP, 268 questionnaires were sent to the authors. Two hundred seventeen of the questionnaires fulfilled the inclusion criteria. ITP affected boys and girls similarly, and the median age of manifestation was 3.5 years. The main reasons for hospitalization were thrombocytopenia, bleeding signs, hematomas, and/or petechiae. Bleeding scores were ≤ 3 in 96% of children, which corresponded to a low-to-moderately low risk of bleeding. No life-threatening bleeding was documented. The most common therapies were IVIG (n = 59), GC (n = 33), or a combination of these (n = 17). Blood products (i.e., red blood cells, platelet concentrate, and fresh frozen plasma) were given to 13 patients. Compared to the established guidelines, 67 patients were over-treated, and 2 patients were under-treated. Conclusions Adherence to German ITP treatment guidelines is currently limited. To improve patient safety and medical care, better medical training and dissemination of the guidelines are required in line with targeted analyses of patients with serious bleeding events to identify potential risk constellations. KW - pediatric immune thrombocytopenia KW - acute ITP KW - newly diagnosed ITP KW - ESPED KW - autoimmunity KW - treatment guidelines KW - intravenous immunoglobulins KW - bleeding score KW - children Y1 - 2021 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-261832 VL - 8 ER - TY - JOUR A1 - Augustin, Anne Marie A1 - Kertels, Olivia A1 - Wiegering, Verena A1 - Thurner, Annette A1 - Kickuth, Ralph T1 - Percutaneous implantation of peripherally inserted totally implantable venous access systems in the forearm in adolescent patients JF - Pediatric Radiology N2 - Background Children with different underlying malignant diseases require long-term central venous access. As for port systems in a pectoral position, peripherally implanted port systems in the forearm revealed high levels of technical and clinical success in adult cohorts. Objective To investigate the technical and clinical outcomes of percutaneous central venous port implantation in the forearm in adolescents. Materials and methods Between April 2010 and August 2020, 32 children ages 9 to 17 years with underlying malignancy received 35 totally implantable venous access ports (TIVAPs) in the forearm. All venous port systems were peripherally inserted under ultrasound guidance. Correct catheter placement was controlled by fluoroscopy. As primary endpoints, the technical success, rate of complications and catheter maintenance were analyzed. Secondary endpoints were the side of implantation, vein of catheter access, laboratory results on the day of the procedure, procedural radiation exposure, amount of contrast agent and reasons for port device removal. Results Percutaneous TIVAP placement under sonographic guidance was technically successful in 34 of 35 procedures (97.1%). Procedure-related complications did not occur. During the follow-up, 13,684 catheter days were analyzed, revealing 11 complications (0.8 per 1,000 catheter-duration days), Of these 11 complications, 7 were major and 10 occurred late. In seven cases, the port device had to be removed; removal-related complications did not occur. Conclusion Peripheral TIVAP placement in the forearms of children is a feasible, effective and safe technique with good midterm outcome. As results are comparable with standard access routes, this technique may be offered as an alternative when intermittent venous access is required. KW - adolescents KW - central venous catheter KW - children KW - forearm KW - interventional radiology KW - totally implantable venous access port KW - vascular access Y1 - 2022 U6 - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-324947 VL - 52 IS - 8 ER -