[Results of the hypothermia registry of the University Hospital Dresden from 2010-2017. Application of hypothermia therapy in newborns in Germany]. / Ergebnisse des Hypothermienetzwerks des Universitätsklinikums Dresden von 2010­2017. Entwicklung der Hypothermietherapie bei Neugeborenen in Deutschland.

INTRODUCTION: Therapeutic hypothermia (TH) improves the outcome in newborns with hypoxic-ischemic encephalopathy (HIE) and should be used in case of perinatal asphyxia and signs of moderate/severe HIE. MATERIAL/METHODS: Frequency of HIE and the application of TH were extracted from the neonatal survey, a registry that collects data from all German hospitals, and from the hypothermia registry, established in 2010. The latter was also used to analyze short-term outcomes of the newborns. RESULTS: Between 2010 and 2017, 106 of Germany's 213 perinatal centers joined the registry. Response rates varied between 22 and 60%. The registry recorded 164 (IQR 115-224) TH cases per year in newborns with HIE. In the neonatal survey, 517 (382-664) TH and 543 (432-581) HIE cases were reported. Since 2014 there have been more cases of TH than HIE. After TH, 10.4% (8-13%) of the newborns died, 81% (78-82%) of the newborns were discharged home, 3.6% (3-5%) to a rehabilitation facility, and 5.4% (5-7%) transferred to another clinic. 89% (87-89%) were on complete oral feedings. DISCUSSION: After the introduction of TH in the clinical routine, the number of treated newborns increased continuously. Currently, the number of TH is higher than the number of children with HIE, which is difficult to explain, as the presence of a moderate or severe HIE is a mandatory requirement for TH. The data from the hypothermia registry showed no significant changes in mortality or neurological outcome over time.

Weaning Premature Infants from Nasal Continuous Positive Airway Pressure - Current Practice and Scientific Evidence.

Objective In the past decade, a number of trials have been conducted to determine the optimal strategy of weaning premature infants from nasal continuous airway pressure (nCPAP). However, a paucity of information exists on how weaning is actually performed in clinical routine. Aim of this study was to investigate the current practice of weaning premature infants from nCPAP in Germany. Methods An online survey was performed in German tertiary care neonatal units. Results All 160 German tertiary care units were contacted. Replies were retrieved from 85/160 (53%) units, of which 83/160 (52%) completed the questionnaire. 66/83 (80%) respondents indicated to wean without the use of formal written policies. In 44/83 (53%) units weaning decisions are made jointly between physicians and nurses, whereas physicians are the sole decision makers in 33/83 (40%) as are nurses in 6/83 (7%) units. Many units use more than one weaning strategy. 81/83 units (98%) gradually reduce nCPAP pressure as the initial step in the weaning process. 9/83 (11%) units stop nCPAP at standard criteria [CICADA (CeasIng nCpap At standarD criteriA) method] and 58/83 (70%) units use a cycling nCPAP on/off strategy. 52/83 (63%) of the responding units use nasal high flow at least at some point during the weaning process, either as a gradual weaning method or during nCPAP breaks. Conclusion Weaning strategies from nCPAP vary widely in German tertiary care neonatal units. It appears that evidence is still insufficient to promote a distinct weaning strategy which in turn highlights the urgent need for further adequately powered clinical trials.

Mucopolysaccharidosis VI: cardiac involvement and the impact of enzyme replacement therapy.

OBJECTIVE: To describe cardiac abnormalities in patients with mucopolysaccharidosis (MPS) VI and to evaluate the impact of enzyme replacement therapy (ERT) on cardiac structure and function. METHODS: Data from electrocardiographic and echocardiographic evaluations were retrospectively collected from patients with MPS VI who are followed up at the Children's Hospital of Mainz. RESULTS: The study included 44 (16 male and 28 female) patients. At baseline, valvular regurgitation (mainly aortic and mitral) and left ventricular (LV) volume overload were present in over half of patients. Other common cardiac manifestations were sinus tachycardia, LV hypertrophy, concentric LV remodelling, and pulmonary hypertension. One patient had left atrial dilation and one had congestive heart failure. Interventricular septal wall thickness and LV posterior wall thickness were above normal in most patients. Twenty five patients had a pre-ERT and at least one follow-up visit after ERT start. Mean follow-up after ERT start was 5.6 (SD 2.3) years. Despite the late onset (mean age 14.6 years) of treatment, ERT appeared to improve or arrest the progression of LV remodelling and LV hypertrophy and suspend the progression of cardiac valve disease. CONCLUSIONS: MPS VI is associated with an array of cardiac manifestations. ERT appears to have some impact on cardiac structure and function when started late in life, but may have better long-term results when started during early infancy.