Pediatric, renal, advances

BACKGROUND: Microphthalmia-associated transcription factor/transcription factor E (MiTF/TFE) translocation renal cell carcinoma (RCC) is a rare type of non-clear cell RCC (nccRCC), which is more common in females. Currently, there is no standardized treatment for advanced metastatic microphthalmia translocation RCC (MiT-RCC). The main treatment modalities include surgery, chemotherapy, immunotherapy, anti-vascular endothelial growth factor or vascular endothelial growth factor receptor (VEGFR) inhibitors, mammalian target of rapamycin (mTOR) inhibitors, and targeted therapy against the mesenchymal-epithelial transition (MET) factor signaling pathway...

Viral infections have been considered as a major cause of morbidity and mortality after kidney transplantation in pediatric cohort. Children are at high risk of acquiring virus-related complications due to immunological immaturity and the enhanced alloreactivity risk that led to maintenance of high immunosuppressive regimes. Hence, prevention, early detection, and prompt treatment of such infe ctions are of paramount importance. Among all viral infections, herpes viruses (herpes simplex virus, varicella zoster virus, Epstein-Barr virus, cytomegalovirus), hepatitis B and C viruses, BK polyomavirus, and respiratory viruses (respiratory syncytial virus, parainfluenza virus, influenza virus and adenovirus) are common in kidney transplant recipients...

Recent progress in glomerular immune complex and complement-mediated diseases have refined diagnostic categories and informed mechanistic understanding of disease development in pediatric patients. Herein, we discuss selected advances in 3 categories. First, membranous nephropathy antigens are increasingly utilized to characterize disease in pediatric patients and include phospholipase A2 receptor (PLA2R), Semaphorin 3B (Sema3B), neural epidermal growth factor-like 1 (NELL1), and protocadherin FAT1, as well as the lupus membranous-associated antigens exostosin 1/2 (EXT1/2), neural cell adhesion molecule 1 (NCAM1), and transforming growth factor beta receptor 3 (TGFBR3)...

Fetal Wilms tumor (WT) is extremely rare, but with advances in fetal imaging, more cases are being reported. The management of these cases remains challenging. Herein, we present the case of a full-term female infant diagnosed antenatally at 32 weeks of gestation with a right solid renal mass detected on routine prenatal ultrasound without polyhydramnios. At birth, the infant was healthy, with no evidence of dysmorphic features or abnormal laboratory tests to suggest a predisposition syndrome. Her family history was also unremarkable...

BACKGROUND: Dialysis disequilibrium syndrome (DDS) is a rare but significant concern in adult and pediatric patients undergoing dialysis initiation with advanced uremia or if done after an interval. It is imperative to gain insights into the epidemiological patterns, pathophysiological mechanisms, and preventive strategies aimed at averting the onset of this ailment. DESIGN: Prospective observational quality improvement initiative cohort study. SETTING AND PARTICIPANTS: A prospective single-center study involving 50 pediatric patients under 18 years recently diagnosed with chronic kidney disease stage V with blood urea ≥200 mg/dL, admitted to our tertiary care center for dialysis initiation from January 2017 to October 2023...

Surgery of locally advanced neuroblastoma with risk factors is one of the most difficult in pediatric surgery. Incidence of nephrectomy during subtotal or complete tumor resection is higher due to common involvement of renal vessels. We present a patient with locally advanced retroperitoneal neuroblastoma who underwent heterotopic kidney autotransplantation.

Many ventilator-dependent children have comorbid conditions including urinary tract disorders. We aimed to present a focused review of the literature describing the causes and management of urinary system problems in children with long-term home mechanical ventilation. We performed a literature search in PubMed/MEDLINE, Scopus, and Web of Science with keywords "children," "home mechanical ventilation," "urinary system," "urinary tract," "neurogenic bladder," "clean intermittent catheterization," "urinary tract infection," "urolithiasis," and "acute kidney injury...

Pediatric renal diseases encompass a diverse array of pathological conditions, often engendering enduring ramifications. Metabolomics, an emergent branch of omics sciences, endeavors to holistically delineate alterations in metabolite compositions through the amalgamation of sophisticated analytical chemistry techniques and robust statistical methodologies. Recent advancements in metabolomics research within the realm of pediatric nephrology have been substantial, offering promising avenues for the identification of robust biomarkers, the elaboration of novel therapeutic targets, and the intricate elucidation of molecular mechanisms...

Gastrointestinal (GI) sequelae, such as vomiting, hyperacidity, dysphagia, dysmotility, and diarrhea, are nearly universal among patients with nephropathic cystinosis. These complications result from disease processes (e.g., kidney disease, cystine crystal accumulation in the GI tract) and side effects of treatments (e.g., cysteamine, immunosuppressive therapy). GI involvement can negatively impact patient well-being and jeopardize disease outcomes by compromising drug absorption and patient adherence to the strict treatment regimen required to manage cystinosis...

Pediatric kidney transplant recipients will likely require a retransplant in their lifetime. Although the significant advances in clinical management and newer immunosuppressive agents have had a significant effect to improve short-term allograft function, it is apparent that long-term allograft function remains suboptimal. Therefore, it is likely that most pediatric renal allograft recipients will require 1 or more retransplants during their lifetime. In the West, an increasing number of patients on the deceased donor wait list are awaiting a retransplant; in the US, 15% of current annual transplants are retransplants...

Purpose: To report a multi-institutional international experience in performing robotic pyeloplasty for complicated cases of ureteropelvic junction obstruction (UPJO) in the pediatric population and assess feasibility and outcomes. Materials and Methods: Retrospective chart review of all pediatric patients who underwent robotic-assisted laparoscopic pyeloplasty (RALP) for UPJO in challenging cases between 2013 and 2021 was included. Demographics, perioperative surgical data, complications, and results are described...

OBJECTIVE: The purpose of our study is to investigate the laboratory and clinical features of tumor lysis syndrome (TLS) and acute kidney injury (AKI) in childhood non-Hodgkin lymphomas (NHL) and to reveal their impact on long term kidney function in survivors. METHODS: Our single-center retrospective study included 107 patients (0-18 years old) with NHL who were admitted and treated at our hospital between 1998 and 2020. The relationship between TLS and age, gender, histopathological subgroup, tumor stage, lactate dehydrogenase (LDH) level at presentation, bone marrow and kidney involvement were assessed...

Congenital anomalies of the kidneys and urinary tract encompass the spectrum of disorders that include the kidneys, ureters, bladder, and urethra. These abnormalities often lead to altered renal size and location, dysplastic changes in the kidney parenchyma, and anomalies in the collecting system. Though the etiology of each of these conditions can be variable, it is known that the collection of these defects represent 40% to 50% of all pediatric end-stage renal disease worldwide. The multifaceted management of these conditions is aimed at preserving kidney function and ultimately delaying the need for transplantation...

INTRODUCTION: The etiology of chronic kidney disease (CKD) is varied and complex. Diabetes and hypertension comprise 2/3 of cases and rare conditions, including inherited genetic diseases, comprise 1/3. We previously reported a 54% increase in clinical studies in CKD in the last 10 years. Hypothesizing a greater increase in rare renal disease studies, we undertook further analysis of metadata from Clinicaltrials.gov. METHODS: CT.gov was searched for 49 conditions determined to be rare renal diseases posted between Jan-2003 and Dec-2022...

BACKGROUND AND OBJECTIVE: Osteomyelitis, a severe bone infection caused mainly by pyogenic organisms, poses diagnostic challenges due to its non-specific magnetic resonance imaging (MRI) manifestations. Conventional MRI, though the imaging modality of choice, often exhibits signal abnormalities with overlapping differential diagnoses, potentially leading to overestimation of infection extent and duration. To address these limitations, advanced MRI sequences, including dynamic contrast-enhanced (DCE) MRI, 1H magnetic resonance spectroscopy (MRS), diffusion-weighted imaging (DWI), and Dixon techniques have emerged as promising alternatives...

The primary function of the kidneys is to maintain systemic homeostasis (disruption of renal structure and function results in multilevel impairment of body function). Kidney diseases are characterized by a chronic, progressive course and may result in the development of chronic kidney disease (CKD). Evaluation of the composition of the proteome of urinary small extracellular vesicles (sEVs) as a so-called liquid biopsy is a promising new research direction. Knowing the composition of sEV could allow localization of cellular changes in specific sections of the nephron or the interstitial tissue before fixed changes, detectable only at an advanced stage of the disease, occur...

BACKGROUND: Wilms' tumor (WT) is the most common paediatric renal tumor and is one of the most treatment-responsive solid tumours. Survival from Wilms tumour (WT) in sub-Saharan Africa remains dismal as a result of late presentation, treatment abandonment and infrastructure deficit. The purpose of this study was to analyze the clinical outcome of children with Wilms tumour managed in a Nigerian referral centre over a 15-year period. METHODS: This is a retrospective study of children with WT (nephroblastoma) who were treated at our institution between January 2006 and December 2020...

BACKGROUND: This study investigated the clinical characteristics and kidney outcomes of childhood-onset lupus nephritis (LN), and risk factors associated with prognosis. METHODS: We enrolled 216 patients with histologically diagnosed LN during childhood. The Korean Society of Pediatric Nephrology organized a retrospective cohort study of childhood-onset LN in 13 major pediatric nephrology centers in South Korea. RESULTS: The mean age at kidney biopsy was 13...

Allogeneic hematopoietic cell transplantation (HCT) can be complicated by life-threatening organ toxicity and infection necessitating intensive care. Epidemiologic data have been limited by single-center studies, poor database granularity, and a lack of long-term survivors. To identify contemporary trends in ICU utilization and long-term outcomes, we merged data from the Center for International Blood and Marrow Transplant Research and the Virtual Pediatric Systems databases. We identified 6,995 pediatric HCT patients age ≤21 years who underwent 1st allogeneic HCT between 2008-2014 across 69 centers in the United States or Canada and followed patients until the year 2020...

Infants with congenital bilateral renal agenesis are at significant risk for morbidity and mortality, despite substantial and continuing advances in fetal and neonatal therapeutics. Infants with bilateral renal agenesis may episodically develop severe hypotension that can be refractory to traditional vasopressors. Synthetic angiotensin-II has been successfully used in adult and a few pediatric patients with refractory hypotension but has not been extensively studied in infants. We describe the use of angiotensin-II in treating refractory hypotension in a premature infant with congenital bilateral renal agenesis admitted to the NICU...