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Background: Patients with complex congenital heart disease frequently undergo a life-long ambulatory therapy with the need for repeated hospital interventions. To optimize this manifold interplay, we designed and implemented a tele-medical service, the Congenital Cardiology Cloud (CCC). This study aims to analyse the requirements for its implementation through the comprehensive assessment of design, installation and impact on patient´s care., Methods: CCC's development comprised the analysis of historically raised admission and discharge management and the definition of technical and organizational requirements. Elaboration of procedural flow charts, description of data formats and technical processes as well as distribution of patient structure formed part of this process., Results: Analysis of existing workflows uncovered a need for the rebuilding of admission and discharge process and decision making for further treatment. The CCC reduces conference-meetings in general and repetitive meetings up to less than a third. Real-time dispatch of discharge documents guarantees an instantaneous access to patient-related data. Comparative analyses show a more complex patient group to be involved in tele-medical services., Conclusions: The CCC enables the sharing of complex clinical information by overcoming sectoral barriers and improves mutual patient advice. Implementation of a tele-medical network requires willingness, perseverance and professional engagement. Future application analysis and possible introduction of refinancing concepts will show its long-term feasibility., Competing Interests: The authors declare that they have no conflict of interest., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Background: Allogeneic hematopoietic stem cell transplantation (allo-HSCT) has improved survival in high-risk childhood leukemia but is associated with long-term sequelae such as impaired pulmonary function and reduced exercise capacity impacting quality of life., Methods: A convenience sample of 17 patients after allo-HSCT (HSCT-12 male, age 15.7±6.7 years, time after HSCT 5.3±2.8 years) underwent pulmonary function testing, echocardiography, and an incremental exercise test on a bike. Physical activity and health-related quality of life were assessed by questionnaires (7-day physical activity recall, PEDS-QL). Seventeen healthy age- and gender-matched controls served as control group (CG) for results of pulmonary function and exercise testing., Results: HSCT showed reduced pulmonary function (HSCT vs. CG: FEV1 90.5±14.0 vs. 108.0±8.7%pred; FVC 88.4±19.3 vs. 107.6±6.9%pred, DLCO 75.3±23.6 vs. 104.9±12.8%pred) and exercise capacity (VO 2 peak 89±30.8%pred, CG 98±17.5%pred; Wmax 84±21.7%pred, CG 115±22.8%pred), but no relevant cardiac dysfunction and a good quality of life (PEDS-QL mean overall score 83.3±10.7). Differences in peak oxygen uptake between groups were mostly explained by 5 adolescent patients who underwent total body irradiation for conditioning. They showed significantly reduced diffusion capacity and reduced peak oxygen uptake. Patients reported a mean time of inactivity of 777±159min/day, moderate activity of 110±107 min/day, hard activity of 35±36 min/day, and very hard activity of 23±22 min/day. A higher amount of inactivity was associated with a lower peak oxygen uptake (correlation coefficient tau -0.48, p=0.023)., Conclusions: This pilot study shows that although patients after allo-HSCT reported a good quality of life, regular physical activity and exercise capacity are reduced in survivors of stem cell transplantation, especially in adolescents who are treated with total body irradiation for conditioning. Factors hindering regular physical activity need to be identified and exercise counseling should be part of follow-up visits in these patients., (© 2021. The Author(s).)

The neonatal bacterial infection is a potentially life-threatening condition that justifies intravenous antibiotic therapy. However, clinical symptoms are often unspecific. Particularly in the absence of a response to antibiotic therapy, various differential diagnoses can be considered. We report the clinical presentation, the diagnostic steps, the therapy as well as the long-term progression of a preterm infant who acquired a perinatal enterovirus infection including viral myocarditis. The case underlines the clinical relevance of enterovirus infections in newborns. The current literature, however, describes only single aspects and lacks, in particular, accurate data on both epidemiology and morbidity., Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht., (© Georg Thieme Verlag KG Stuttgart · New York.)

We report a male newborn who became symptomatic with supraventricular tachycardia on the first day of life. Neither adenosine nor electric cardioversion could terminate the tachycardia, therefore intravenous esmolol (β-receptor blocker) was initiated. Inspite of subsequent administration of various antiarrhythmic medications in increasingly higher doses, repeated supraventricular tachycardic episodes occurred. The electrocardiogram showed typical findings of a multifocal atrial tachycardia as the underlying cause. When tachycardic episodes occurred, they also presented as atrial flutter at 460 bpm and a 2:1 block. Finally, high dosage of amiodarone (10 mg/kgbw/d) led to continuous control of the heart rate without tachycardic episodes. To date our patient is mostly in sinus rhythm but without tachycardic episodes and doing well., (© Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Late vitamin K deficiency bleeding in young infants is a rare disorder which occurs almost exclusively in breast-fed infants who did not receive proper vitamin K prophylaxis at birth and who might additionally suffer from cholestasis. Its impact on morbidity is high since in 50% of the cases it presents with intracranial hemorrhage with a mortality rate of 20% and life-long neurologic sequelae in 30% of the affected infants., Case Reports: 2 male infants were both admitted to our unit at the age of 5 weeks with subdural hematoma with midline shift due to late vitamin K deficiency bleeding. Both infants did not receive the recommended Vitamin K prophylaxis in Germany. One patient presented with cholestatic jaundice on admission as an additional risk factor., Discussion: Parents who in the apparent best interest for their children refuse the recommended and well established vitamin K prophylaxis at birth leading to the reappearance of late vitamin K deficiency bleeding. These parents also tend to refuse routine immunizations of childhood in later life, which not only have an impact on their own child but might bear a risk for the whole community., Conclusion: It is the responsibility of health-care takers to show increased awareness to the growing number of parents refusing vitamin K prophylaxis at birth and educate them properly about the devastating consequences of late vitamin K deficiency bleeding., (© Georg Thieme Verlag KG Stuttgart · New York.)

Despite antibiotic therapy, infections with Neisseria meningitidis still demonstrate a high rate of morbidity and mortality even in developed countries. The fulminant septicaemic course, named Waterhouse-Friderichsen syndrome, with massive haemorrhage into the adrenal glands and widespread petechial bleeding suggest pathophysiological inhibition of platelet function. Our data show that N. meningitidis produces the important physiological platelet inhibitor and cardiovascular signalling molecule nitric oxide (NO), also known as endothelium-derived relaxing factor (EDRF). N. meningitidis -derived NO inhibited ADP-induced platelet aggregation through the activation of soluble guanylyl cyclase (sGC) followed by an increase in platelet cyclic nucleotide levels and subsequent activation of platelet cGMP- and cAMP- dependent protein kinases (PKG and PKA). Furthermore, direct measurement of horseradish peroxidase (HRP) passage through a vascular endothelial cell monolayer revealed that N. meningitidis significantly increased endothelial monolayer permeability. Immunfluorescence analysis demonstrated NO dependent disturbances in the structure of endothelial adherens junctions after co-incubation with N. meningitidis . In contrast to platelet inhibition, the NO effects on HBMEC were not mediated by cyclic nucleotides. Our study provides evidence that NO plays an essential role in the pathophysiology of septicaemic meningococcal infection.

Accidental ingestion of foreign bodies is a common problem in infants and childhood, but ingestion of magnetic construction toys is very rare. In the case of ingestion of multiple parts of these magnetic construction toys, they may attract each other through the intestinal walls, causing pressure necrosis, perforation, fistula formation or intestinal obstruction. A 20-month-old boy presented with a three-day history of abdominal pain and bilious vomiting. Physical examination revealed a slighted distended abdomen. The -white blood cell count was increased, but the C-reactive protein was normal. Ultrasound and X-ray of the abdomen showed a distended bowel loop in the right upper quadrant, a moderate amount of free intraperitoneal liquid and 4 foreign bodies. Emergency laparotomy was performed and 2 perforations in the ileum were detected. The perforations were caused by a magnetic construction toy and 2 iron globes. The fourth foreign body was a glass marble. The foreign bodies were removed, both perforations were primarily sutured. The child was discharged on postoperative day 10 after an uneventful recovery. Parents should be warned against the potential dangers of children's constructions toys that contain these kinds of magnets., (Georg Thieme Verlag Stuttgart, New York.)

Extremely immature preterm infants rarely present with a leukocytosis exceeding 30,000/microL. The pathogenetic sequence leading to leukemoid reactions in non-malignant diseases remains to be elucidated. Potential triggers for leukemoid reactions in premature infants include prenatal corticosteroids, chorioamnionitis and funisitis or systemic infection. In the two-year period from 2006 to 2007 all infants with a gestational age of less than 26 weeks were screened for leukocytosis. Among our cases, one preterm infant presented with a leukocyte count of 229,300/microL at the age of 48 hours, lasting throughout the first three weeks of life. Impairment of microcirculation and resulting organ dysfunction were not observed. Thus, invasive therapeutic procedures, which are routinely initiated in hyperleukocytosis in accompanying malignant diseases, may not have the same significance in extremely immature preterm infants and should be executed in these patients on an individual basis and with extreme caution.

Infection with group B streptococcus (GBS) is the most common cause of early onset neonatal sepsis in many countries, leading to neonatal morbidity and mortality. There is much evidence for a direct involvement of platelets in the pathogenesis of inflammation and sepsis. Several bacteria are known to directly interact with platelets leading to activation and aggregation, a phenomenon also observed with GBS. Here, we demonstrate that GBS rapidly bound to platelets; however, only strains isolated from septic patients bound fibrinogen on their surface and induced platelet thromboxane synthesis, platelet aggregation, and P-selectin (CD62P) expression. In contrast, GBS strains isolated from healthy newborns or healthy pregnant women induced only shape change, but not platelet thromboxane synthesis, platelet aggregation, or CD62P expression. All GBS strains investigated were able to activate FcgammaRIIA receptor signaling pathways including phospholipase C gamma2 (PLCgamma2), as well as calcium/calmodulin-dependent myosin kinase II (CaMKII) and phosphorylation of myosin light chain (MLC). In contrast, protein kinase C (PKC) was exclusively activated by GBS strains isolated from septic patients, and p38 mitogen activated protein kinase (p38 MAP kinase) was preferentially activated by septic GBS strains. Furthermore, stress signaling kinase SEK1/MKK4 and focal adhesion kinase (FAK) were activated by all tested GBS strains in a FcgammaRIIA-independent way. This study demonstrates that septic, but not colonizing, GBS strains bind fibrinogen on their surface, and that septic GBS strains influence platelet function not only via the FcgammaRIIA receptor, but also via pathways distinct from IgG-mediated signalling. These mechanisms lead to platelet aggregation and secretion, thereby possibly modulating the pathophysiologic course of GBS infections.

The fetal lung secretes significant quantities of surfactant during late gestation to prepare for initiation of respiration at birth. However, the mechanism by which this occurs has not been determined. Since Ca2+-phosphatidylserine (PS)-dependent protein kinase C has been implicated in surfactant secretion in adult lung, the present study was done to determine whether this enzyme is also involved in the initiation of surfactant release from fetal type II cells. Type II cells isolated from gestational day-24 fetal rabbits were used. Cells were prelabelled with [32P] and [3H]choline and exposed to 4beta phorbol ester (10(-5) M) for 2 h. Secretion product and subcellular fractions were isolated by removing the culture medium, mixing with homogenate from adult rabbit lung, and subfractionating by centrifugation on a sucrose gradient. Samples of secretion product were also prepared for electron microscopy. Ca2+-PS-dependent protein kinase C was also assayed in some samples, and an add-back technique was used to determine whether enzyme activity in the intracellularly stored surfactant fraction was due to contamination. The results showed that material released by fetal type II cells after exposure to phorbol ester coprecipitated with adult rabbit lung lamellar bodies and microsomes. Morphologically, a range of forms, including lamellar-body-like structures, was detected. The released material originated largely from the lamellar body compartment of the fetal type II cells and displayed immunoreactivity with antibody to surfactant protein A (SP-A) at 35 and 70 kDa apparent molecular mass. Assay of protein kinase C in fetal type II cells showed that exposure to conditioned medium, which induces differentiation, increased activity. Incubation with phorbol ester induced translocation of activity to the microsomal fraction. Add-back assays suggested that protein kinase C activation by treatment with phorbol ester induced translocation of enzyme activity to the lamellar body fraction; none was detected prior to treatment. These results support a role for Ca2+-PS-dependent protein kinase C in initiation of surfactant release by interaction with the developing lamellar body compartment in fetal type II cells.