Short-Term Impact of Newly Imposed Legal Restriction on DSD Surgery in Children in Germany.

Background/objectives: In recent years, changing paradigms, both culturally and scientifically, have fundamentally altered the approach to the treatment of children with Disorders of Sexual Development (DSD) prior to reaching the age of legal consent. In Germany, the situation changed with the introduction of legislation that includes a partial ban on DSD surgery in children in 2021. This study aims to analyze the impact of this legislation on clinical practice.

Early postnatal high-dose fat-soluble enteral vitamin A supplementation for moderate or severe bronchopulmonary dysplasia or death in extremely low birthweight infants (NeoVitaA): a multicentre, randomised, parallel-group, double-blind, placebo-controlled, investigator-initiated phase 3 trial.

Background: Vitamin A plays a key role in lung development, but there is no consensus regarding the optimal vitamin A dose and administration route in extremely low birthweight (ELBW) infants. We aimed to assess whether early postnatal additional high-dose fat-soluble enteral vitamin A supplementation versus placebo would lower the rate of moderate or severe bronchopulmonary dysplasia or death in ELBW infants receiving recommended basic enteral vitamin A supplementation.

Methods: This prospective, multicentre, randomised, parallel-group, double-blind, placebo-controlled, investigator-initiated phase 3 trial conducted at 29 neonatal intensive care units in Austria and Germany assessed early high-dose enteral vitamin A supplementation (5000 international units [IU]/kg per day) or placebo (peanut oil) for 28 days in ELBW infants.

Does Accidental Hypothermia Increase Morbidity and Mortality in Mature Neonates?

Accidental hypothermia in mature neonates requiring hospitalization is a clinical complication that has not been studied in detail. In the present study, the effect of accidental hypothermia on common morbidity in neonates in the postnatal period was investigated. We did temperature measurements in 616 mature neonates after birth, after transfer to the maternity ward and at check-up at age of 48-72 hours of life.

"Multisystem Inflammatory Syndrome in Children" (MIS-C) after COVID-19 Infection in the Metropolitan Area of Nuremberg-Erlangen, Germany-Expectations and Results of a Two-Year Period.

Background: Multisystemic Inflammatory Syndrome in children (MIS-C) is a rare autoimmune disorder occurring after a latency period following acute SARS-CoV-2 infection. The therapeutic regime of MIS-C is adapted to the therapy of the Kawasaki disease, as clinical symptoms are similar. Since the Kawasaki disease can potentially result in severe symptoms, which may even affect long-term health, it is essential to gain further knowledge about MIS-C.

[Pediatric infectious emergencies-from febrile seizure to purpura fulminans].

Febrile seizures, which are relatively common in young children, are often triggered by an infection and resolve quickly. Prompt presentation to a pediatric department is mandatory after any first seizure and every time for children ≤ 12 months. Central nervous system (CNS) diseases in childhood are able to cause seizures or other neurological disorders.

[Breathing disorders in children].

Breathing disorders in children are one of the most common challenges for both parents and physicians. The first step should always consist of the initial clinical assessment concerning the potentially critically ill patient. Using the pediatric assessment triangle (PAT), the rapid evaluation of airway and breathing is crucial.

Umbilical venous catheter- and peripherally inserted central catheter-associated complications in preterm infants with birth weight < 1250 g : Results from a survey in Austria and Germany.

Background And Objective: Umbilical venous catheters (UVC) and peripherally inserted central catheters (PICC) are commonly used in preterm infants but have been associated with a number of serious complications. We performed a survey in Austria and Germany to assess the use of UVCs and PICCs in preterm infants with a birth weight < 1250 g and associated rates of catheter-related adverse events.

Methods: Electronic survey of participating centers of the NeoVitaA trial.

Pharmacokinetic/Pharmacodynamic Modelling of Allopurinol, its Active Metabolite Oxypurinol, and Biomarkers Hypoxanthine, Xanthine and Uric Acid in Hypoxic-Ischemic Encephalopathy Neonates.

Background: Allopurinol, an xanthine oxidase (XO) inhibitor, is a promising intervention that may provide neuroprotection for neonates with hypoxic-ischemic encephalopathy (HIE). Currently, a double-blind, placebo-controlled study (ALBINO, NCT03162653) is investigating the neuroprotective effect of allopurinol in HIE neonates.

Objective: The aim of the current study was to establish the pharmacokinetics (PK) of allopurinol and oxypurinol, and the pharmacodynamics (PD) of both compounds on hypoxanthine, xanthine, and uric acid in HIE neonates.

Glomerular Filtration Rate in Asphyxiated Neonates Under Therapeutic Whole-Body Hypothermia, Quantified by Mannitol Clearance.

Background: Therapeutic hypothermia (TH) is an established intervention to improve the outcome of neonates with moderate-to-severe hypoxic-ischemic encephalopathy resulting from perinatal asphyxia. Despite this beneficial effect, TH may further affect drug elimination pathways such as the glomerular filtration rate.

Objectives: The objective of this study was to quantify the effect of TH in addition to asphyxia on mannitol clearance as a surrogate for the glomerular filtration rate.

Acute urinary retention in children.

Background: Acute urinary retention is a common emergency in adult patients, foremost in older men. In childhood urinary retention is a rare entity with only sparse literature on the etiology.

Objective: To assess the etiology and treatment of acute urinary retention in the pediatric population and assess age and sex distribution.

[Is the Quality Guideline of Care Delivery of Preterm and Mature Infants (QFR-RL) of the Federal Joint Committee (G-BA) a Quality Risk? - An Analysis of the Fulfillment Rate and the Consequences for Care Delivery to Other IC Patients and Imminent Premature Infants at a Regional Network of Perinatal Centers].

Background: The quality guideline for care delivery to preterm and mature infants (QFR-RL) places high demands on perinatal centers. In this analysis, the degree of fulfillment was determined. Additionally, care delivery to further patient groups and sufficient nursing staff capacity for care delivery to imminent preterm infants (FG) were evaluated.

Small direct right ventricular cardiac metastasis of osteosarcoma in a 10-year-old boy affirmed by cardiac MRI.

Background: Metastatic osteosarcoma with direct cardiac involvement is an exceptionally rare finding. Reliable detection of cardiac metastases is known to be crucial for patients therapy and prognosis.

Case Summary: In a 10-year-old boy affected by osteosarcoma of the left femur, a baseline Fluorine-18-fluorodeoxy-glucose positron emission tomography/computed tomography (F-FDG PET/CT) was performed to assess the full extent of disease.

Improved visualization of peripherally inserted central catheters on chest radiographs of neonates using fractional multiscale image processing.

Background: Peripherally inserted central catheters (PICCs) provide secure intravenous access for the delivery of life-sustaining medications and nutrition. They are commonly used in pediatrics. Confirmation of correct central catheter tip position is crucial.

Influence of factor XIII activity on post-operative transfusion in congenital cardiac surgery-A retrospective analysis.

Objectives: Coagulation factor XIII (FXIII) plays a key role in fibrin clot stabilization-an essential process for wound healing following cardiothoracic surgery. However, FXIII deficiency as a risk for post-operative bleeding in pediatric cardiac surgery involving cardiopulmonary bypass (CPB) for congenital heart disease (CHD) is controversially discussed. Thus, as primary outcome measures, we analyzed the association of pre-operative FXIII activity and post-operative chest tube drainage (CTD) loss with transfusion requirements post-operatively.

Assessment of continuous pain in newborns admitted to NICUs in 18 European countries.

Aim: Continuous pain occurs routinely, even after invasive procedures, or inflammation and surgery, but clinical practices associated with assessments of continuous pain remain unknown.

Methods: A prospective cohort study in 243 neonatal intensive care units (NICUs) from 18 European countries recorded the frequency of pain assessments, use of mechanical ventilation, sedation, analgesia or neuromuscular blockade for each neonate for up to 28 days after NICU admission.

Results: Only 2113 of 6648 (31.

Sedation and analgesia practices in neonatal intensive care units (EUROPAIN): results from a prospective cohort study.

Background: Neonates who are in pain or are stressed during care in the intensive care unit (ICU) are often given sedation or analgesia. We investigated the current use of sedation or analgesia in neonatal ICUs (NICUs) in European countries.

Methods: EUROPAIN (EUROpean Pain Audit In Neonates) was a prospective cohort study of the management of sedation and analgesia in patients in NICUs.

Autoselection of cytoplasmic yeast virus like elements encoding toxin/antitoxin systems involves a nuclear barrier for immunity gene expression.

Cytoplasmic virus like elements (VLEs) from Kluyveromyces lactis (Kl), Pichia acaciae (Pa) and Debaryomyces robertsiae (Dr) are extremely A/T-rich (>75%) and encode toxic anticodon nucleases (ACNases) along with specific immunity proteins. Here we show that nuclear, not cytoplasmic expression of either immunity gene (PaORF4, KlORF3 or DrORF5) results in transcript fragmentation and is insufficient to establish immunity to the cognate ACNase. Since rapid amplification of 3' ends (RACE) as well as linker ligation of immunity transcripts expressed in the nucleus revealed polyadenylation to occur along with fragmentation, ORF-internal poly(A) site cleavage due to the high A/T content is likely to prevent functional expression of the immunity genes.

Improved closure of patent ductus arteriosus with high doses of ibuprofen.

The patent ductus arteriosus (PDA) is associated with various complications of prematurity. Cyclooxygenase-inhibitors are the first-line intervention for closure of the PDA. However, the rates of PDA closure still are unsatisfactory.

Epstein-Barr virus triggered rejection after kidney allograft transplantation.

Pediatric allograft recipients are at increased risk for Epstein-Barr virus (EBV)-associated disorders. We report on a 4-year-old boy who received a cadaver kidney transplant. The donor's viral status was not known.

Intrauterine use of hyperconcentrated platelet concentrates collected with Trima Accel in a case of neonatal alloimmune thrombocytopenia.

Background: Due to the threat of serious or fatal bleedings, fetuses with neonatal alloimmune thrombocytopenia (NAIT) may need intrauterine platelet (PLT) transfusions. To prevent a volume overload or an ABO minor mismatch, standard PLT concentrates need to be washed to increase the PLT concentration and to reduce the plasma content. Hyperconcentrated single-donor PLT concentrates (HCPs) are a therapeutic alternative.

Hypertonic-hyperoncotic solutions improve cardiac function in children after open-heart surgery.

Objectives: Hypertonic-hyperoncotic solutions are used for the improvement of micro- and macrocirculation in various types of shock. In pediatric intensive care medicine, controlled, randomized studies with hypertonic-hyperoncotic solutions are lacking. Hypertonic-hyperoncotic solutions may improve cardiac function in children.

Pediatric renal allograft transplantation does not normalize the increased cortisol/cortisone ratios of chronic renal failure.

Objective: The conversion of cortisol (F) to cortisone (E) is catalyzed by 11beta-hydroxysteroid dehydrogenase type 2 (11beta-HSD2). Children suffering from chronic renal failure (CRF) have a decreased activity of 11beta-HSD2 contributing to increased arterial blood pressure. The objective was to investigate whether a normal conversion of F to E is achieved after renal transplantation (TX) in children.

Anesthetic management of patients with ornithine transcarbamylase deficiency.

Ornithine transcarbamylase deficiency (OTCD) is the most common inborn error of the urea cycle. Several specific factors require care during anesthesia in patients with this condition to avoid metabolic decompensation with acute hyperammonemia and encephalopathy. We report monozygous twins with severe neonatal-onset OTCD undergoing general anesthesia twice each, with midazolam, s-ketamine, fentanyl and isoflurane in combination with surgical field infiltration with ropivacaine.