Purpose: It has been suggested that patients with posterior urethral valves (PUV) and a solitary functioning kidney (SFK) have poorer mid-term and long-term renal function than those with 2 functioning kidneys. We compared the mid-term estimated glomerular filtration rate (eGFR) of PUV patients with and without solitary kidneys.
Materials And Methods: We used the CIRCUP randomized controlled trial database, which included only patients with prenatally suspected, postnatally confirmed PUV.
Introduction: Denys-Drash syndrome (DDS) is a rare disease typically associated with a triad of early onset nephrotic syndromes (NS), susceptibility to Wilms tumor (WT), and genitourinary structural defects. DDS is caused by Wilms' tumor suppression gene () variants, with the most frequent variants in exons 8 and 9. This study aimed to evaluate the long-term clinical outcomes and genotype-to-phenotype correlations in a large, multicenter cohort of children with typical DDS.
View Article and Find Full Text PDFRectoperineal fistula (RPF) and rectovestibular fistula (RVF) are the most common forms of low anorectal malformations (ARMs) in girls, and lead to difficult stooling, thus demanding early surgical correction. This study's aim was to assess early outcomes associated with one-step ventral and dorsal proctoplasty in RPF/RVF.All female infants who consecutively underwent one-step proctoplasty for RPF/RVF at our institution (2012-2022) were retrospectively included.
View Article and Find Full Text PDFIntroduction: The diagnosis of ovarian torsion is made with delay among pre-pubertal girls and the management after the surgical detorsion is poorly codified. We interested in the French experience during the last two decades.
Methods: Retrospective study in French pediatric surgery centers from January 2000 to December 2022 concerning girls who presented with at least 1 episode of ovarian torsion in the prepubertal period.
Eur J Surg Oncol
December 2024
Background: Minimally invasive surgery for paediatric adrenal tumours has evolved, but robot-assisted laparoscopic adrenalectomy (RALA) in children remains poorly studied. The current prospective study aims to demonstrate the safety and efficacy of RALA in treating children with adrenal tumours.
Methods: A prospective institutional analysis of children presenting with neuroblastic and endocrine tumours treated with RALA was undertaken over a six year-period.
Background: Robot-assisted pyeloplasty is the most frequently performed robotic procedure in children. A retroperitoneal approach limits surgical trauma and avoids peritoneal irritation. This led to the establishment of the criteria for day surgery (DS) and a related clinical care pathway.
View Article and Find Full Text PDFBackground & Aims: As the composition of the bile acid (BA) pool has a major impact on liver pathophysiology, we studied its regulation by the BA receptor Takeda G protein coupled receptor (TGR5), which promotes hepatoprotection against BA overload.
Methods: Wild-type, total and hepatocyte-specific TGR5-knockout, and TGR5-overexpressing mice were used in: partial (66%) and 89% extended hepatectomies (EHs) upon normal, ursodeoxycholic acid (UDCA)- or cholestyramine (CT)-enriched diet, bile duct ligation (BDL), cholic acid (CA)-enriched diet, and TGR5 agonist (RO) treatments. We thereby studied the impact of TGR5 on: BA composition, liver injury, regeneration and survival.
During liver repair after injury, bile secretion has to be tightly modulated in order to preserve liver parenchyma from bile acid (BA)-induced injury. The mechanisms allowing the liver to maintain biliary homeostasis during repair after injury are not completely understood. Besides their historical role in lipid digestion, bile acids (BA) and their receptors constitute a signalling network with multiple impacts on liver repair, both stimulating regeneration and protecting the liver from BA overload.
View Article and Find Full Text PDFIntroduction: The use of interventional radiology (IR) in the treatment of pediatric solid tumors has markedly increased over the last three decades. However, data on effectiveness of IR-techniques, such as embolization/ablation, are scarce. In this systematic review and meta-analysis, we examined the outcomes of IR-procedures in the treatment of solid tumors in children.
View Article and Find Full Text PDFMol Aspects Med
August 2017
Tissue repair is orchestrated by a finely tuned interplay between processes of regeneration, inflammation and cell protection, allowing organisms to restore their integrity after partial loss of cells or organs. An important, although largely unexplored feature is that after injury and during liver repair, liver functions have to be maintained to fulfill the peripheral demand. This is particularly critical for bile secretion, which has to be finely modulated in order to preserve liver parenchyma from bile-induced injury.
View Article and Find Full Text PDFTriple-negative breast cancer (TNBC) is a subgroup of breast cancer that is negative for estrogen and progesterone receptor and ERBB2 protein expression. It is characterized by its aggressive behavior and by the lack of targeted therapies. To identify new therapeutic targets in TNBC, we used real-time quantitative RT-PCR to analyze 63 TNBC samples in terms of their mRNA expression of 26 genes coding for the major proteins currently targeted by drugs used to treat other cancers or undergoing clinical trials in breast cancer.
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