Familial congenital lower urinary tract obstruction (LUTO) suggested by screening for lower urinary tract dysfunction in parents of patients: A descriptive study.

Background: Congenital lower urinary tract obstruction (LUTO) describes a heterogeneous group of congenital malformations. Posterior urethral valves (PUV) represent the most common entity. Familial occurrence has been described, suggestive of underlying genetic factors. LUTO can occur in various degrees of severity. In severe forms, oligohydramnios, pulmonary hypoplasia, and renal damage can occur resulting in high pre- and postnatal mortality. On the contrary, mild forms may become apparent through recurrent urinary tract infections. Such high phenotypic variability has been described even within the same family. Here, we systematically screened parents of affected children for symptoms of LUTO. Methods: The study population consisted of parents of LUTO patients. Fathers over 50 years of age were excluded, to avoid inclusion of male phenocopies due to early prostatic hypertrophy. Uroflowmetry, ultrasonography for residual urine and hydronephrosis, and laboratory examination of standard renal retention parameters were assessed, and a detailed patient history was taken, including the assessment of the International Prostate Symptom Score. Results: Twenty-nine of 42 LUTO families enrolled were found eligible for the present study. Of these, we identified five families in which the father had already been diagnosed with infravesical obstruction (17%). Of the remaining families, nine agreed to participate in our study. Of these nine families, eight families had a child affected with PUV and one family had a child with urethral stenosis. Here, we found two fathers and one mother with symptoms of LUTO suggestive of mild LUTO and one family, in which the unborn male fetal brother of the affected index patient was also diagnosed prenatally with LUTO. Conclusion: Our observations suggest that LUTOs have a higher heritability than previously thought and that first-degree relatives of the affected should be clinically assessed for symptoms of LUTO.

Right ventricular cardiomyocyte expansion accompanies cardiac regeneration in newborn mice after large left ventricular infarcts.

Cauterization of the root of the left coronary artery (LCA) in the neonatal heart on postnatal day 1 (P1) resulted in large, reproducible lesions of the left ventricle (LV), and an attendant marked adaptive response in the right ventricle (RV). The response of both chambers to LV myocardial infarction involved enhanced cardiomyocyte (CM) division and binucleation, as well as LV revascularization, leading to restored heart function within 7 days post surgery (7 dps). By contrast, infarction of P3 mice resulted in cardiac scarring without a significant regenerative and adaptive response of the LV and the RV, leading to subsequent heart failure and death within 7 dps. The prominent RV myocyte expansion in P1 mice involved an acute increase in pulmonary arterial pressure and a unique gene regulatory response, leading to an increase in RV mass and preserved heart function. Thus, distinct adaptive mechanisms in the RV, such as CM proliferation and RV expansion, enable marked cardiac regeneration of the infarcted LV at P1 and full functional recovery.

Implementation of 2D Barcode Medication Labels and Smart Pumps in Pediatric Acute Care: Lessons Learned.

BACKGROUND: In pediatric intensive care, prescription, administration, and interpretation of drug doses are weight dependent. The use of standardized concentrations simplifies the preparation of drugs and increases safety. For safe administration as well as easy interpretation of intravenous drug dosing regimens with standardized concentrations, the display of weight-related dose rates on the infusion device is of pivotal significance. OBJECTIVES: We report on challenges in the implementation of a new information technology-supported medication workflow. The workflow was introduced on eight beds in the pediatric heart surgery intensive care unit as well as in the pediatric anesthesia at the University of Bonn Medical Center. The proposed workflow utilizes medication labels generated from prescription data from the electronic health record. The generated labels include a two-dimensional barcode to transfer data to the infusion devices. METHODS: Clinical and technical processes were agilely developed. The reliability of the system under real-life conditions was monitored. User satisfaction and potential for improvement were assessed. In addition, a structured survey among the nursing staff was performed. The questionnaire addressed usability as well as the end-users' perception of the effects on patient safety. RESULTS: The workflow has been applied 44,111 times during the pilot phase. A total of 114 known failures in the technical infrastructure were observed. The survey showed good ratings for usability and safety (median "school grade" 2 or B for patient safety, intelligibility, patient identification, and handling). The medical management of the involved acute care facilities rated the process as clearly beneficial regarding patient safety, suggesting a rollout to all pediatric intensive care areas. CONCLUSION: A medical information technology-supported medication workflow can increase user satisfaction and patient safety as perceived by the clinical end-users in pediatric acute care. The successful implementation benefits from an interdisciplinary team, active investigation of possible associated risks, and technical redundancy.

Blood pressure and kidney function in neonates and young infants with intrauterine growth restriction.

BACKGROUND: Intrauterine growth restriction (IUGR) has been associated with changes in kidney anatomy, nephrogenesis and the vascular system, resulting in secondary arterial hypertension and kidney damage in adulthood. Here, we compare routine clinical and metabolic parameters between IUGR and non-IUGR study participants in the neonatal and early infant period. METHODS: A total of 39 IUGR and 60 non-IUGR neonates were included during an 18-month study period. We compared blood pressure, serum creatinine (SCr), urea nitrogen (BUN), urinary albumin, α-1-microglobulin, transferrin, immunoglobulin G and total protein excretion in spontaneous urine normalized by urine creatinine level during the hospital stay. RESULTS: There were no significant differences in mean values of blood pressure and urinary protein excretion between cases and controls. SCr and BUN levels were lower in the IUGR group compared to the non-IUGR group. CONCLUSIONS: The lower levels of SCr and BUN may be attributed to lower liver and muscle mass in IUGR neonates and young infants. Biomarkers currently used in routine clinical care do not allow early postnatal prediction of higher blood pressure or worse kidney function due to IUGR, so further studies are needed. A higher resolution version of the Graphical abstract is available as Supplementary information.

Nonurgent Visits to the Pediatric Emergency Department before and during the First Peak of the COVID-19 Pandemic.

BACKGROUND: Nonurgent visits in pediatric Emergency Departments are a growing burden. In order to find predictors for those nonurgent visits, we performed a retrospective analysis of unscheduled visits at the Pediatric Emergency Department of the University Hospital of Bonn, Germany, in the year 2017. Additionally, we compared these findings to unscheduled visits during the first peak of the worldwide pandemic of the Coronavirus disease 2019, to see if there would be an effect on nonurgent pediatric Emergency Department attendances. METHODS: For our retrospective cohort study, we analyzed more than 5.000 visits at the pediatric Emergency Department of the University Hospital of Bonn, Germany, before and during the first peak of the ongoing worldwide pandemic of the Coronavirus disease 2019, particularly with regard to their urgency. Data included gender, age, zip code, urgency, and preexisting conditions. RESULTS: Our study shows that more than half of unscheduled pediatric Emergency Department visits (69%) at the University Hospital in Bonn are for nonurgent reasons, with short living distance being a factor to present children to a pediatric Emergency Department, even with minor complaints. During the first peak of the pandemic of the Coronavirus disease 2019, nonurgent visits decreased significantly, potentially due to hesitation to attend a pediatric Emergency Department with minor issues, fearing an infection with SARS-CoV-2 at the hospital. CONCLUSION: Many people use pediatric Emergency Departments for nonemergency complaints. In order to address the reasons for nonurgent visits to pediatric Emergency Departments and to prevent parents from doing so, further studies and targeted education concepts for parents are needed.

Exploratory Assessment of Levosimendan in Infants With Congenital Diaphragmatic Hernia.

OBJECTIVES: Infants with congenital diaphragmatic hernia frequently suffer from cardiac dysfunction and pulmonary hypertension during the postnatal course. With the use of the inodilator levosimendan, a therapeutic approach is available in situations with catecholamine-refractory low-cardiac-output failure and severe pulmonary hypertension. DESIGN: Retrospective single-center cohort study. SETTING: University-based, tertiary-care children's hospital neonatal ICU. PATIENTS: Cohort of 24 infants with congenital diaphragmatic hernia and levosimendan therapy, without underlying major cardiac defect, treated at the University Children´s Hospital Bonn, Germany, between January 2017 and December 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty-four infants with congenital diaphragmatic hernia were treated with levosimendan (41% of hospitalized congenital diaphragmatic hernia infants in the study period). In 88%, the congenital diaphragmatic hernia was left-sided. The median observed-to-expected lung-to-head ratio was 36%. About 60% of the infants were supported with venovenous extracorporeal membrane oxygenation and the mortality was 38% (9/24 infants). Levosimendan administration was associated with improvement of pulmonary hypertension severity (p = 0.013 and p = 0.000) and right ventricular dysfunction (p = 0.011 and p = 0.000) at 24 hours and 7 days after treatment. Similarly, the prevalence of left ventricular dysfunction decreased from 50% at baseline to 10% after 7 days (p = 0.026). A significant reduction in the peak inspiratory pressure was observed after drug application (p = 0.038) and a significant decrease of the Vasoactive-Inotropic Score was apparent (p = 0.022). A relevant arterial hypotension as a drug-related adverse event occurred in one patient. CONCLUSIONS: This is the first study exploring clinical and hemodynamic changes after levosimendan treatment in a cohort of infants with congenital diaphragmatic hernia. An association of levosimendan application and an improvement in pulmonary hypertension, right ventricular, and left ventricular dysfunction were observed within 7 days after drug infusion. However, due to the retrospective design of this study, the results should be interpreted carefully.

Typical epidemiology of respiratory virus infections in a Brazilian slum.

Host population size, density, immune status, age structure, and contact rates are critical elements of virus epidemiology. Slum populations stand out from other settings and may present differences in the epidemiology of acute viral infections. We collected nasopharyngeal specimens from 282 children aged ≤5 years with acute respiratory tract infection (ARI) during 2005 to 2006 in one of the largest Brazilian slums. We conducted real-time reverse transcription-polymerase chain reaction (RT-PCR) for 16 respiratory viruses, nested RT-PCR-based typing of rhinoviruses (HRVs), and collected clinical symptoms. Viruses were common causes of respiratory disease; with ≥1 virus being detected in 65.2% of patients. We detected 15 different viruses during 1 year with a predominance of HRV (33.0%) and human respiratory syncytial virus (hRSV, 12.1%) infections, and a high rate of viral coinfections (28.3%). We observed seasonality of hRSV, HRV and human coronavirus infections, more severe symptoms in hRSV and influenza virus (FLU) infections and prolonged circulation of seven HRV clusters likely representing distinct serotypes according to genomic sequence distances. Potentially unusual findings included the absence of human metapneumovirus detections and lack of typical FLU seasonal patterns, which may be linked to the population size and density of the slum. Nonetheless, most epidemiological patterns were similar to other studies globally, suggesting surprising similarities of virus-associated ARI across highly diverse settings and a complex impact of population characteristics on respiratory virus epidemiology.

Human parvovirus 4 in nasal and fecal specimens from children, Ghana.

Nonparenteral transmission might contribute to human parvovirus 4 (PARV4) infections in sub-Saharan Africa. PARV4 DNA was detected in 8 (0.83%) of 961 nasal samples and 5 (0.53%) of 943 fecal samples from 1,904 children in Ghana. Virus concentrations ≤ 6-7 log(10) copies/mL suggest respiratory or fecal-oral modes of PARV4 transmission.