Publications by authors named "Burek C"

Bone Morphogenetic Protein (BMP) signaling is essential for craniofacial development, though little is known about the mechanisms that govern BMP secretion. We show that depolarization induces calcium-dependent BMP4 release from mouse embryonic palate mesenchyme. We show endogenous transient changes in intracellular calcium occur in cranial neural crest cells, the cells from which embryonic palate mesenchyme derives.

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Purpose: To report our experience and results in terms of complications, reoperation rate and urinary continence with the Salvage Continent Vesicostomy (SCV) technique in pediatric patients with history or need of enterocystoplasty and absent appendix.

Methods: Retrospective review of all patients with a history of a continent catheterizable channel surgery performed in our institution between June 2016 and January 2022. Only patients with a SCV surgery with a minimum 6-month post operative follow up were included in this cohort and divided in group 1 (history of previous bladder augmentation) and group 2 (simultaneous bladder augmentation and SCV).

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In April 2018, rotted 'Lamoka' tubers were received from a commercial storage facility (<1% incidence) in St. Joseph County, Michigan by the MSU Potato & Sugar Beet Pathology and Plant & Pest Diagnostics programs. Dense circular colonies of white fungal-like growth were observed on the surface of the tubers, and internal tissues were watery and spongy with gray to brown discoloration (Supplemental Figure 1).

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Introduction: The proactive management of spina bifida (SB), especially of its severe form, myelomeningocele (MMC), has contributed to decreasing chronic kidney disease (CKD). The objective of this study is to present the evolution of 5-year-old patient with MMC followed from birth with a proactive approach.

Material And Methods: This retrospective study included 55 cases with MMC of up to 5 years of age.

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Objective: To analyze the outcomes of patients undergoing upper-pole heminephrectomy surgery and to assess the different variables that may have an impact on outcome, specifically regarding morbidity and the need for further surgeries.

Materials And Methods: A retrospective study of patients who underwent laparoscopic upper-pole heminephrectomy for a nonfunctional moiety between August 2007 and December 2019 was conducted at 3 centers. A total of 130 patients met the inclusion criteria.

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Objective: To define the accuracy of ultrasound to determine bladder volume in pediatric patients with neurogenic bladder (NB).

Methods: Retrospective analysis of children with NB in treatment with urethral clean intermittent catheterization.

Exclusion Criteria: bladder surgeries, and catheterization through a channel different than urethra.

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Introduction: When patients with neurogenic bladder become refractory, there are different alternatives, such as the use of β3-adreceptor agonists. The aim of the present study is to evaluate efficacy and safety of Mirabegron as adjuvant treatment.

Material And Methods: 37 patients under 18 years of age who underwent Mirabegron were retrospectively studied.

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Objective: To prove that incidence of UTI after a pediatric urodynamic study (UDS) is low, and that patients without urine culture (UC) analysis prior to a UDS will not have a significant increase in the incidence of UTI (post-UDS UTI).

Methods: Prospective cohort study including consecutive pediatric patients undergoing UDS in a single center for 1 year. Patients were divided in 2 groups: (G1) UDS with a previous negative UC and (G2) UDS without a previous UC analysis.

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Purpose: Preserving renal function and achieving urinary continence are the most important urological goals in the management of cloaca patients. Many prognostic factors have been described, such as the length of the common channel (CC) and urethra, the presence of spinal dysraphism, and associated urological anomalies. The aim of this study was to analyze urinary continence status and need for reconstructive procedures and their correlation with urological risk factors in a series with a long-term follow-up.

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Objetive: Reninoma, or juxtaglomerular cell tumor, is a benign neoplasm which causes severe hypertension with elevated plasma renin activity. Usually, it is well localized on computed tomography scan or magnetic resonance images, and complete resection of the tumor with renal function preservation is the optimal treatment. To describe the technique of a transperitoneal nephron-sparing laparoscopic surgery in a pediatric patient.

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The purpose of this paper is to render an account of the experience of a group of healthcare providers and to propose innovative solutions for patients with Spina Bifida (SB) in Argentina. Based on our practice, patients reach specialists too late (40% already undergoing chronic kidney disease stage 1). However, several strategies were implemented in order to reverse this trend, among which were: the setting up of a professional network (via emails and Google groups), team training at different locations, videoconferences, and calling the Ministry of Health to action.

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Introduction: The proactive management of children with myelomeningocele (MMC) has contributed to decreasing their progression to end-stage renal disease, thanks to early urological evaluation and timing implementation of treatments.

Objective: To demonstrate that early urological evaluation of the urinary tract in MMC shows functional alterations in most cases, and that it requires medical intervention, even when in some cases the complementary imaging studies do not show any abnormalities.

Material And Methods: A retrospective study including 60 patients aged <1 year with MMC who were followed by a multidisciplinary team.

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Background/purpose: It is still under discussion which is the best tissue augmenting substance for the endoscopic treatment of children with vesicoureteral reflux (VUR). We describe our preliminary experience (September 2009-November 2011) with polyacrylate-polyalcohol copolymer hydrogel (PPCH).

Methods: This is an observational, descriptive, prospective study which included 81 female and male patients (age 1-14 years) diagnosed with unilateral (n=45) and bilateral (n=36) primary VUR comprising a total of 117 refluxing renal units (RRU).

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Objective: To evaluate an outcome of endoscopic correction of vesicoureteral reflux (VUR) using Vantris (Promedon, Cordoba, Argentina) in terms of its effectiveness and morbidity in a multicenter study.

Materials And Methods: From 2009 to 2013, 611 patients (210 boys and 401 girls) with a mean age of 3.56 years (range, 1 month-18 years) were treated at 7 centers worldwide endoscopically with Vantris injection.

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A study of IgG4 autoantibody levels in juvenile thyroid disease patients showed evidence of heritability using the ROMP screening method. These levels increased with time despite the fact that total IgG antibody decreased with time. Evidence of heritability was demonstrated only in patients with high titers of autoantibodies to both thyroglobulin (Tg) and thyroperoxidase (TPO) unlike family members who may show high titers of one or the other and be asymptomatic at the time of sampling.

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Aim: To evaluate the effects of repeated injections of onabotulinumtoxinA in children with neurogenic bladders refractory to conventional treatment.

Patients And Methods: A total of 26 children with neurogenic bladder were prospectively studied. Some patients received up to four repeat injections.

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High content omic techniques in combination with stable human in vitro cell culture systems have the potential to improve on current pre-clinical safety regimes by providing detailed mechanistic information of altered cellular processes. Here we investigated the added benefit of integrating transcriptomics, proteomics and metabolomics together with pharmacokinetics for drug testing regimes. Cultured human renal epithelial cells (RPTEC/TERT1) were exposed to the nephrotoxin Cyclosporine A (CsA) at therapeutic and supratherapeutic concentrations for 14days.

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Objective: to describe and incorporate a modification of the fully laparoscopic Mitrofanoff (LM) procedure with the aim of diminishing operative time and technical difficulties, and to reproduce functional results.

Patients And Methods: Retrospective review of six patients with voiding dysfunction with indication for clean intermittent catheterization who did not require bladder augmentation and who underwent a fully LM procedure between January and November 2011.

Results: The mean operative time was 183.

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When the neurogenic bladder is refractory to anticholinergics, botulinum toxin type A is used as an alternative. The neurotoxin type A reduces bladder pressure and increases its capacity and wall compliance. Additionally, it contributes to improving urinary continence and quality of life.

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Lysosome-related organelles (LROs) are synthesized in specialized cell types where they largely coexist with conventional lysosomes. Most of the known cellular transport machinery involved in biogenesis are ubiquitously expressed and shared between lysosomes and LROs. Examples of common components are the adaptor protein complex-3 (AP-3) and biogenesis of lysosome-related organelle complex (BLOC)-2.

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Ureteral necrosis is an uncommon complication following RT which can adversely affect outcome. Even though authors agree that the native ureter ought to be preserved, there are cases in which graft function can only be salvaged by ureteral substitution. The scant references in the literature on the use of the appendix for left ureteral replacement in children prompted us to report the following two cases in whom the technique was employed and to assess the evolution of graft function in these patients.

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Objective: To retrospectively assess early postoperative complications in augmentation cystoplasty without preoperative mechanical bowel preparation (MBP).

Material And Methods: Between May 1987 and May 2006, 162 cystoplasties were performed in 158 children. The segments used were: sigmoid colon (81.

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To study the role of invariant Natural Killer T cell ( iNKT) cells in autoimmune thyroiditis, we derived two iNKT cell lines from the spleens of NOD· H2(h4) mice, a strain that develops spontaneous autoimmune thyroiditis exacerbated by excess dietary iodine. The two lines were CD1d-restricted and expressed CD4(+), DX5(+), and the Vα4Jα281 gene segment, of the T-cell receptor α locus. Upon stimulation with α-galactosyl-ceramide (α-GalCer), both lines rapidly produced IL-2, IL-4, IFN-γ, IL-10, and TNF-α.

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Purpose: To present three cases of arterial high flow priapism (HFP) and propose a management algorithm for this condition.

Materials And Methods: We studied three children with post-traumatic arterial HFP (two patients with perineal trauma and one with penis trauma).

Results: Spontaneous resolution was observed in all the patients.

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