Unraveling chronic kidney disease in children: a surprising manifestation of celiac disease.

Celiac disease, firstly described in children, is a type of T-cell enteropathy that occurs in individuals genetically predisposed to gluten exposure. The estimated global prevalence of celiac disease is continuously increasing. Although, traditionally, celiac disease was diagnosed in children with failure to thrive and digestive issues, it is now recognized that may present with a wide range of symptoms beyond gastrointestinal ones. Celiac disease continues to pose significant challenges due to the continuous advancement of knowledge in understanding its pathophysiology, diagnosing the condition, managing its effects, and exploring potential therapeutic approaches. The prevalence of celiac disease is increased among individuals with chronic kidney disease, also. The most frequent associations are with diabetic nephropathy, IgA nephropathy and urolithiasis. A gut-kidney axis has been recognized to play a significant role in chronic kidney diseases. This literature review aims to review the chronic renal pathology associated with celiac disease, with emphasis on childhood.

Celiac disease - a pluripathological model in pediatric practice.

Being defined as an autoimmune, chronic pathology, frequently encountered in any age group, but especially in pediatrics, celiac disease (also called gluten enteropathy), is gaining more and more ground in terms of diagnosis, but also interest in research. The data from the literature of the last decades attest the chameleonic way of its presentation, there may be both classic onset symptoms and atypical symptoms. Given the impact played by celiac disease, especially in the optimal growth and development of children, the current narrative review aims to highlight the atypical presentation methods, intended to guide the clinician towards the inclusion of the pathology in the differential diagnosis scheme. To these we add the summary presentation of the general data and therapeutic lines regarding the underlying condition and the existing comorbidities. In order to place the related information up to date, we performed a literature review of the recent articles published in international databases. We bring forward the current theories and approaches regarding both classic celiac disease and its atypical manifestations. Among these we note mainly constitutional, skin or mucous, bone, neuro-psychic, renal, reproductive injuries, but also disorders of biological constants and association with multiple autoimmunities. Knowing and correlating them with celiac disease is the key to optimal management of patients, thus reducing the subsequent burden of the disease.

Neural network analysis of neutron and X-ray reflectivity data incorporating prior knowledge.

Due to the ambiguity related to the lack of phase information, determining the physical parameters of multilayer thin films from measured neutron and X-ray reflectivity curves is, on a fundamental level, an underdetermined inverse problem. This ambiguity poses limitations on standard neural networks, constraining the range and number of considered parameters in previous machine learning solutions. To overcome this challenge, a novel training procedure has been designed which incorporates dynamic prior boundaries for each physical parameter as additional inputs to the neural network. In this manner, the neural network can be trained simultaneously on all well-posed subintervals of a larger parameter space in which the inverse problem is underdetermined. During inference, users can flexibly input their own prior knowledge about the physical system to constrain the neural network prediction to distinct target subintervals in the parameter space. The effectiveness of the method is demonstrated in various scenarios, including multilayer structures with a box model parameterization and a physics-inspired special parameterization of the scattering length density profile for a multilayer structure. In contrast to previous methods, this approach scales favourably when increasing the complexity of the inverse problem, working properly even for a five-layer multilayer model and a periodic multilayer model with up to 17 open parameters.

Closing the loop: autonomous experiments enabled by machine-learning-based online data analysis in synchrotron beamline environments.

Recently, there has been significant interest in applying machine-learning (ML) techniques to the automated analysis of X-ray scattering experiments, due to the increasing speed and size at which datasets are generated. ML-based analysis presents an important opportunity to establish a closed-loop feedback system, enabling monitoring and real-time decision-making based on online data analysis. In this study, the incorporation of a combined one-dimensional convolutional neural network (CNN) and multilayer perceptron that is trained to extract physical thin-film parameters (thickness, density, roughness) and capable of taking into account prior knowledge is described. ML-based online analysis results are processed in a closed-loop workflow for X-ray reflectometry (XRR), using the growth of organic thin films as an example. Our focus lies on the beamline integration of ML-based online data analysis and closed-loop feedback. Our data demonstrate the accuracy and robustness of ML methods for analyzing XRR curves and Bragg reflections and its autonomous control over a vacuum deposition setup.

Dental Appliances for the Treatment of Obstructive Sleep Apnea in Children: A Systematic Review and Meta-Analysis.

Background and objectives: Obstructive sleep apnea (OSA) in children is a debilitating disease, difficult to treat. Dental appliances have been proposed as a valid therapy for improving functional outcomes with good compliance rates. Herein, we aimed to perform a meta-analysis comparing clinical outcomes between OSA children treated with dental appliances versus controls. Materials Methods: The study was registered with PROSPERO. A systematic search was performed for all comparative studies examining outcomes in pediatric patients who underwent treatment of OSA with oral appliances versus controls. Data was extracted and analyzed using a random effects model via Rev Man 5.3. Results: Six studies including 180 patients were analyzed split into two groups: patients treated with dental appliances (n = 123) and the controls (n = 119). Therapy with dental appliances was shown to significantly improve the apnea-hypopnea index (p = 0.009) and enlarge the superior posterior airway space (p = 0.02). Maxilla-to-mandible measurements were not significantly different between the two groups, nor was the mean SO2 (p = 0.80). Conclusions: This is the most updated meta-analysis assessing the role of dental appliances for OSA in children; it shows that such devices can improve functional outcomes by decreasing the apnea-hypopnea index.

Machine learning for scattering data: strategies, perspectives and applications to surface scattering.

Machine learning (ML) has received enormous attention in science and beyond. Discussed here are the status, opportunities, challenges and limitations of ML as applied to X-ray and neutron scattering techniques, with an emphasis on surface scattering. Typical strategies are outlined, as well as possible pitfalls. Applications to reflectometry and grazing-incidence scattering are critically discussed. Comment is also given on the availability of training and test data for ML applications, such as neural networks, and a large reflectivity data set is provided as reference data for the community.

An Up-to-Date Literature Review on Ventricular Assist Devices Experience in Pediatric Hearts.

Ventricular assist devices (VAD) have gained popularity in the pediatric population during recent years, as more and more children require a heart transplant due to improved palliation methods, allowing congenital heart defect patients and children with cardiomyopathies to live longer. Eventually, these children may require heart transplantation, and ventricular assist devices provide a bridge to transplantation in these cases. The FDA has so far approved two types of device: pulsatile and continuous flow (non-pulsatile), which can be axial and centrifugal. Potential eligible studies were searched in three databases: Medline, Embase, and ScienceDirect. Our endeavor retrieved 16 eligible studies focusing on five ventricular assist devices in children. We critically reviewed ventricular assist devices approved for pediatric use in terms of implant indication, main adverse effects, and outcomes. The main adverse effects associated with these devices have been noted to be thromboembolism, infection, bleeding, and hemolysis. However, utilizing left VAD early on, before end-organ dysfunction and deterioration of heart function, may give the patient enough time to recuperate before considering a more long-term solution for ventricular support.

The Impact of Water and Other Fluids on Pediatric Nephrolithiasis.

Pediatric nephrolithiasis cases have been on the rise in the past several years, resulting in increased healthcare costs and other burdens on the juveniles with this ailment. Recent research has shown that present trends in pediatric nephrolithiasis have changed as a result of fluid intake, including water consumption, nutrition, obesity prevalence, lifestyle, and imaging procedures. A specific cause, meanwhile, is still elusive. Trends in pediatric nephrolithiasis need to be thoroughly researched. Furthermore, variables specific to pediatric nephrolithiasis that could cause greater difficulties in an affected child elevate the level of worry with cumulative prevalence. Doctors should rigorously assess patients who present with kidney stones when they have dynamics such as varied clinical presentation, high recurrence of kidney stones linked to metabolic and urinary tract problems, and the potential existence of rare genetic kidney stone illnesses. This review aims to identify adaptive risk factors and anomalies that call for specialized treatment and prescription. More specifically, the major goals of medical and surgical treatments are to eliminate kidney stone risk and stop relapse while concurrently lowering interventional barriers. A dedicated nephrolithiasis clinic run by a pediatric nephrologist, nutritionist, urologist, and clinical nurse may sometimes be beneficial for patients in serious danger. Such a clinic offers significant chances to learn more about pediatric nephrolithiasis, which has been linked to water consumption and hence fosters urgently required study in this area.

Diagnosis Difficulties and Minimally Invasive Treatment for Ovarian Masses in Adolescents.

About 1% of childhood tumors can be malignant ovarian tumors and differential diagnosis with benign ones is sometimes difficult before surgery. Concerning the management of such tumors in adolescents for which future fertility is a concern, there is specific interest in their malignant potential and the possible use of ovarian-sparing operative techniques, as well the suitability of chemotherapy. To exemplify the difficulties of preoperative differential diagnosis, personalized approach and the difficulties of deciding to preserve the affected ovary, we report a rare case of a 14-year-old female adolescent with a growing abdominal painless mass and without any other chronic diseases. After physical examination and imaging investigations, laparoscopic surgical procedure is performed, the peritoneal cavity is explored and the well-delimited gigantic tumor is removed. Considering the normally looking pelvis and absence of adenopathy, as well as the patient's age and wish to have children, both ovaries are preserved. Laparoscopy has become the gold standard in the management of this condition, although there are few studies that report this approach in children; the differential diagnosis between a benign and a malignant tumor cannot be established exactly until after the histological examination, which revealed in our case a cystic teratoma with mature tissues. A better understanding of clinical features and evolution of giant ovarian masses in adolescents could help clinicians better diagnose and treat such lesions.

Neural network analysis of neutron and X-ray reflectivity data: automated analysis using mlreflect, experimental errors and feature engineering.

The Python package mlreflect is demonstrated, which implements an optimized pipeline for the automated analysis of reflectometry data using machine learning. The package combines several training and data treatment techniques discussed in previous publications. The predictions made by the neural network are accurate and robust enough to serve as good starting parameters for an optional subsequent least-mean-squares (LMS) fit of the data. For a large data set of 242 reflectivity curves of various thin films on silicon substrates, the pipeline reliably finds an LMS minimum very close to a fit produced by a human researcher with the application of physical knowledge and carefully chosen boundary conditions. The differences between simulated and experimental data and their implications for the training and performance of neural networks are discussed. The experimental test set is used to determine the optimal noise level during training. The extremely fast prediction times of the neural network are leveraged to compensate for systematic errors by sampling slight variations in the data.

Unusual Case of Masseter Muscle Hypertrophy in Adolescence-Case Report and Literature Overview.

Unilateral hypertrophy of the masseter muscle is a very rare pathological entity in children. Its etiology is uncertain and it requires a high degree of suspicion, as it must be differentiated from other conditions of the masseter area. As there are few pathological studies to elucidate this condition, we report a rare case of unilateral masseter muscle hypertrophy in a 16-year-old female patient with gradual onset of a painless swelling in the posterior left cheek which caused facial asymmetry with repercussions on the patient's self-image. The diagnosis of unilateral masseter muscle hypertrophy was suggested by clinical examination, ultrasound scanning, and nuclear magnetic resonance, and was confirmed by histologic examination two years later when the patient returned for the surgical correction. The pathological findings report showed fragments of skeletal muscle with hypertrophic fibers associated with normal-sized muscle fibers in both longitudinal and transverse sections. The postoperative evaluation was favorable as both the adolescent and her family were satisfied with her look on the 14th day, 1st year, and 3rd year follow-ups. In conclusion, unilateral masseter muscle hypertrophy in adolescence is a sensitive problem due to the psychological implications of facial appearance. Definite diagnosis and treatment of the hypertrophied muscle is the ideal solution.

Statistical Analysis of the Main Risk Factors of an Unfavorable Evolution in Gastroschisis.

Gastroschisis is a congenital abdominal wall defect that presents an increasing occurrence at great cost for the health system. The aim of the study is to detect the main factors of an unfavorable evolution in the case of gastroschisis and to find the best predictors of death. METHODS: we conducted a retrospective cohort study of neonates with gastroschisis treated in a tertiary pediatric center during the last 30 years; 159 patients were eligible for the study. Logistic regression was used to determine the risk of death, estimated based on independent variables previously validated by the chi-square test. RESULTS: if the birth weight is below normal, then we find an increased risk (4.908 times) of evolution to death. Similarly, the risk of death is 7.782 times higher in the case of developing abdominal compartment syndrome, about 3 times in the case of sepsis and 7.883 times in the case of bronchopneumonia. All four independent variables contributed 47.6% to the risk of death. CONCLUSION: although in the past 30 years in our country we have seen transformational improvements in outcome of gastroschisis, survival rates increasing from 26% to 52%, some factors may still be ameliorated for a better outcome.

Brown tumors of the femur and pelvis secondary to a parathyroid carcinoma: report of one case.

Brown tumors result from excess osteoclast activity and consist of collections of osteoclasts intermixed with fibrous tissue and poorly mineralized woven bone. They are secondary to hyperparathyroidism (HPT). Their incidence is higher in primary than in secondary hyperparathyroidism. We report a 69 years-old male, admitted in a state of confusion, lethargy and bedridden, with a pathological fracture of the femur caused by a brown tumor. The laboratory examination revealed a hypercalcemia (8.85 mEq/L), with high levels of ionized Ca (5.48 mEq/L), serum alkaline phosphatases (416 U/L) and serum parathormone (120 pg/mL). Ultrasound examination of the neck showed a large parathyroid tumor, probably corresponding to a carcinoma. A primary HPT was diagnosed. The patient was hydrated and high doses of diuretics and bisphosphonates were administered. After correction of serum calcium and neurologic symptoms, the patient was operated, performing an extensive resection of the tumor. The pathology report confirmed the diagnosis of parathyroid carcinoma.

Brown tumors of the femur and pelvis secondary to a parathyroid carcinoma: Report of one case / Tumores pardos de fémur y pelvis secundarios a carcinoma de paratiroides: Informe de un caso

Brown tumors result from excess osteoclast activity and consist of collections of osteoclasts intermixed with fibrous tissue and poorly mineralized woven bone. They are secondary to hyperparathyroidism (HPT). Their incidence is higher in primary than in secondary hyperparathyroidism. We report a 69 years-old male, admitted in a state of confusion, lethargy and bedridden, with a pathological fracture of the femur caused by a brown tumor. The laboratory examination revealed a hypercalcemia (8.85 mEq/L), with high levels of ionized Ca (5.48mEq/L), serum alkaline phosphatases (416 U/L) and serum parathormone (120 pg/mL). Ultrasound examination of the neck showed a large parathyroid tumor, probably corresponding to a carcinoma. A primary HPT was diagnosed. The patient was hydrated and high doses of diuretics and bisphosphonates were administered. After correction of serum calcium and neurologic symptoms, the patient was operated, performing an extensive resection of the tumor. The pathology report confirmed the diagnosis of parathyroid carcinoma.

Los tumores pardos son una consecuencia de una actividad osteoclástica excesiva y consisten en osteoclastos mezclados con tejido fibroso y tejido óseo mal mineralizado. Son secundarios a hiperparatiroidismo y más comunes en hiperparatiroidismo primario. Informamos de un hombre de 69 años que ingresa confuso y letárgico con una fractura patológica del fémur causada por un tumor pardo. El laboratorio mostró hipercalcemia de 8,85 mEq/L, fosfatasas alcalinas de 416 U/L y parathormona de 120 pg/mL. La ecografía del cuello mostró un tumor paratiroideo sospechoso de carcinoma. Se diagnosticó un hiperparatiroidismo primario. El paciente se hidrató y estabilizó con diuréticos y bifosfonatos. Una vez estabilizado, se operó efectuando una extensa resección del tumor. El estudio anatomopatológico confirmó el diagnóstico de cáncer de paratiroides.

Ex-vivo perfusion machines in kidney transplantation. The significance of the resistivity index.

INTRODUCTION: With a growing shortage of organs for transplantation, finding ways of increasing the donor organ pool remains of utmost importance. Perfusion machines (PM) have been proven to enhance the potential for kidney transplants to function sooner, last longer, giving patients the opportunity for a better life quality. OBJECTIVE: The aim of this study is to evaluate the relation between the resistance index provided by the PM, the postoperative resistance index measured by Doppler ultrasound and the initial graft outcome. MATERIAL AND METHOD: Between January 2012-December 2012, clinical data obtained from 82 consecutive renal transplants from brain death donors (BDD) which underwent PM maintenance were analyzed in a transversal study. Prior transplantation we recorded the solution temperature, filtration rate and the resistance index provided by PM. After the surgical intervention, each patient had standard follow-up. Doppler ultrasound resistivity index (RI) was recorded on the first postoperative day. RESULTS: Out of 115 renal transplants, 98 (85.21%) were performed with grafts from BDD. The PM was used for 82 renal grafts. The Doppler resistance index in relation to the resistance index shows a highly statistical correlation by linear regression (R=0.813, p<0.0001). Primary graft function was recorded in 74 patients (90.24%) and it was highly statistically significant correlated with the resistance index measured by PM. Out of 8 patients with primary non-function, 6 patients recovered with normal graft function at one year. CONCLUSION: The resistivity index recorded by the life-port machine is correlated with the vascular resistivity index measured by Doppler ultrasound and thus it may predicts the primary graft outcome.

Heterotopic bone formation in gastric carcinoma. Case report and discussion of the literature.

Osseous metaplasia is a rare finding in the gastrointestinal tract. We report the case of a 75-year-old woman with a gastric adenocarcinoma showing heterotopic ossification with bone marrow formation. The pathogenetic theories are briefly discussed.