Variable treatment response to lumasiran in pediatric patients with primary hyperoxaluria type 1.

Background: Primary hyperoxaluria type 1 (PH 1) is a rare genetic condition due to mutations in the AGXT gene. This leads to an overproduction of oxalate in the liver. Hyperoxaluria often causes kidney stones, nephrocalcinosis, and chronic kidney disease.

Immunoadsorption is equally effective as plasma exchange in paediatric neuroimmunological disorders - A retrospective multicentre study.

Background: Therapeutic apheresis (TA) are promising treatment option for neuroimmunological disorders. In paediatrics, the available data is limited, particularly for the use of IA. The aim of this study was to analyse the use of PE and IA in children and adolescents, with emphasis on outcome and neurological course after treatment as well as the safety of the two modalities.

National and International Comparisons of Gynecological Research in Germany Based on a Bibliometric Analysis of Publications.

Background: Recent years have seen a considerable shift from male doctors to female doctors in the field of gynecology. Female doctors are traditionally more involved with planning and maintaining their family. For gynecology, this could be associated with a risk that research activities will decrease, particularly if results are published in scientific journals.

Time-reversal and parity-time symmetry breaking in non-Hermitian field theories.

We study time-reversal symmetry breaking in non-Hermitian fluctuating field theories with conserved dynamics, comprising the mesoscopic descriptions of a wide range of nonequilibrium phenomena. They exhibit continuous parity-time (PT) symmetry-breaking phase transitions to dynamical phases. For two concrete transition scenarios, exclusive to non-Hermitian dynamics, namely, oscillatory instabilities and critical exceptional points, a low-noise expansion exposes a pretransitional surge of the mesoscale (informatic) entropy production rate, inside the static phases.

Entropy production in the nonreciprocal Cahn-Hilliard model.

We study the nonreciprocal Cahn-Hilliard model with thermal noise as a prototypical example of a generic class of non-Hermitian stochastic field theories, analyzed in two companion papers [Suchanek, Kroy, and Loos, Phys. Rev. Lett.

Volume expansion mitigates Shiga toxin-producing E. coli-hemolytic uremic syndrome in children.

Background: Shiga toxin-producing E. coli-hemolytic uremic syndrome (STEC-HUS) is associated with high morbidity and relevant mortality. Previous small studies showed that volume expansion could improve the course and outcome of STEC-HUS.

Irreversible Mesoscale Fluctuations Herald the Emergence of Dynamical Phases.

We study fluctuating field models with spontaneously emerging dynamical phases. We consider two typical transition scenarios associated with parity-time symmetry breaking: oscillatory instabilities and critical exceptional points. An analytical investigation of the low-noise regime reveals a drastic increase of the mesoscopic entropy production toward the transitions.

Case report: Early onset FSGS in a child after kidney transplantation-a successful treatment.

Background: Early onset focal segmental glomerular sclerosis (FSGS) in the kidney allograft in patients without FSGS in the native kidney is a rare disorder in children. It usually occurs mostly beyond the first year after kidney transplantation and often leads to graft loss. Standardized treatment protocols have not yet been established.

Perspectives on adaptive dynamical systems.

Adaptivity is a dynamical feature that is omnipresent in nature, socio-economics, and technology. For example, adaptive couplings appear in various real-world systems, such as the power grid, social, and neural networks, and they form the backbone of closed-loop control strategies and machine learning algorithms. In this article, we provide an interdisciplinary perspective on adaptive systems.

Long-Range Order and Directional Defect Propagation in the Nonreciprocal XY Model with Vision Cone Interactions.

We study a two-dimensional, nonreciprocal XY model, where each spin interacts only with its nearest neighbors in a certain angle around its current orientation, i.e., its "vision cone.

Long-term outcome after combined or sequential liver and kidney transplantation in children with infantile and juvenile primary hyperoxaluria type 1.

Introduction: Combined or sequential liver and kidney transplantation (CLKT/SLKT) restores kidney function and corrects the underlying metabolic defect in children with end-stage kidney disease in primary hyperoxaluria type 1 (PH1). However, data on long-term outcome, especially in children with infantile PH1, are rare.

Methods: All pediatric PH1-patients who underwent CLKT/SLKT at our center were analyzed retrospectively.

Nonreciprocal forces enable cold-to-hot heat transfer between nanoparticles.

We study the heat transfer between two nanoparticles held at different temperatures that interact through nonreciprocal forces, by combining molecular dynamics simulations with stochastic thermodynamics. Our simulations reveal that it is possible to construct nano refrigerators that generate a net heat transfer from a cold to a hot reservoir at the expense of power exerted by the nonreciprocal forces. Applying concepts from stochastic thermodynamics to a minimal underdamped Langevin model, we derive exact analytical expressions predictions for the fluctuations of work, heat, and efficiency, which reproduce thermodynamic quantities extracted from the molecular dynamics simulations.

A multicenter retrospective study of calcineurin inhibitors in nephrotic syndrome secondary to podocyte gene variants.

While 44-83% of children with steroid-resistant nephrotic syndrome (SRNS) without a proven genetic cause respond to treatment with a calcineurin inhibitor (CNI), current guidelines recommend against the use of immunosuppression in monogenic SRNS. This is despite existing evidence suggesting that remission with CNI treatment is possible and can improve prognosis in some cases of monogenic SRNS. Herein, our retrospective study assessed response frequency, predictors of response and kidney function outcomes among children with monogenic SRNS treated with a CNI for at least three months.

Electrolysis in reduced gravitational environments: current research perspectives and future applications.

Electrochemical energy conversion technologies play a crucial role in space missions, for example, in the Environmental Control and Life Support System (ECLSS) on the International Space Station (ISS). They are also vitally important for future long-term space travel for oxygen, fuel and chemical production, where a re-supply of resources from Earth is not possible. Here, we provide an overview of currently existing electrolytic energy conversion technologies for space applications such as proton exchange membrane (PEM) and alkaline electrolyzer systems.

LungMAP Portal Ecosystem: Systems-level Exploration of the Lung.

An improved understanding of the human lung necessitates advanced systems models informed by an ever-increasing repertoire of molecular omics, cellular imaging, and pathological datasets. To centralize and standardize information across broad lung research efforts, we expanded the LungMAP.net website into a new gateway portal.

Euthanasia of a person with a psychiatric disorder does not violate the European Convention on Human Rights ( [no. 78017/17]).

For more than 20 years, euthanasia in Belgium and The Netherlands is allowed for unbearable suffering caused by terminal or non-terminal illnesses, including psychiatric disorders. Although euthanasia numbers have been increasing over the years, the percentage of cases involving people with a primary psychiatric diagnosis has remained stable (between 1 and 2%). For these cases, the Belgian and Dutch Euthanasia Laws operate similar due care criteria: a well-considered, repeated, and voluntary request from a legally competent adult; a medical condition without prospect of improvement; constant and unbearable suffering that cannot be alleviated; consultation of two independent physicians, including a psychiatrist; and evaluation and control [1-3].

The effect of penis size on partner sexual satisfaction: a literature review.

Larger penis size has been associated with a sign of strength, masculinity, and social standing. Little study has examined the relationship between men's penis size and their partners' sexual satisfaction. The purpose of the present study was to detail and evaluate the effect of penis size on partner sexual satisfaction with a design of narrative literature review.

Improving control over euthanasia of persons with psychiatric illness: Lessons from the first Belgian criminal court case concerning euthanasia.

Background: Belgium is one of very few countries that legally allow euthanasia for suffering caused by psychiatric illness. In the first criminal trial in Belgium of physicians involved in euthanasia, three physicians recently faced the accusation of "murder by poisoning," for allegedly having failed to comply with several requirements of the Belgian Euthanasia Law in granting the euthanasia request a woman suffering from psychiatric illness. Although all three physicians were acquitted, the case generated much debate among policy makers, medical professionals, and the general public.

A link between Krüppel-like factor 4, complement activation, and kidney damage.

Krüppel-like factors (KLFs) are transcription factors with important roles in tissue homeostasis. KLF4 possesses antithrombotic and anti-inflammatory properties. In this issue, Estrada et al.

Safety of Therapeutic Apheresis in Children and Adolescents.

Background: Therapeutic apheresis (TA) is based on the principles of either removing dissolved pathogenic substances (e.g., antibodies) from the blood plasma or replacing plasma factors.

IgG Binds Serine Protease EspP and Protects Mice From O157:H7 Infection.

Shiga toxin-producing O157:H7 is a virulent strain causing severe gastrointestinal infection, hemolytic uremic syndrome and death. To date there are no specific therapies to reduce progression of disease. Here we investigated the effect of pooled immunoglobulins (IgG) on the course of disease in a mouse model of intragastric O157:H7 inoculation.

Finite-Size Scaling at the Edge of Disorder in a Time-Delay Vicsek Model.

Living many-body systems often exhibit scale-free collective behavior reminiscent of thermal critical phenomena. But their mutual interactions are inevitably retarded due to information processing and delayed actuation. We numerically investigate the consequences for the finite-size scaling in the Vicsek model of motile active matter.