Rationale & Objective: Lumasiran reduces urinary and plasma oxalate (POx) in patients with primary hyperoxaluria type 1 (PH1) and relatively preserved kidney function. ILLUMINATE-C evaluates the efficacy, safety, pharmacokinetics, and pharmacodynamics of lumasiran in patients with PH1 and advanced kidney disease.
Study Design: Phase 3, open-label, single-arm trial.
Management of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in the pediatric population can be challenging. Kidney manifestations of liver failure, such as hepatorenal syndrome (HRS) and acute kidney injury (AKI), are increasingly prevalent and may portend a poor prognosis. The overall incidence of AKI in children with ALF has not been well-established, partially due to the difficulty of precisely estimating kidney function in these patients.
View Article and Find Full Text PDFIntroduction: Recently, prolonged intermittent renal replacement therapies (PIRRT) have emerged as cost-effective alternatives to conventional CRRT and their use in the pediatric population has started to become more prominent. However, there is a lack of consensus guidelines on the use of PIRRT in pediatric patients in an intensive care setting.
Methods: A literature search was performed on PubMed/Medline, Embase, and Google Scholar in conjunction with medical librarians from both India and the Cleveland Clinic hospital system to find relevant articles.
Sequential liver-kidney transplantation (SeqLKT) from the same living donor has shown excellent results in children with primary hyperoxaluria type 1 (PH1), yet its experience is limited due to the invasiveness of two major procedures for liver-kidney procurement in a single donor. Despite laparoscopic nephrectomy and hepatic left lateral sectionectomy (LLS) being considered standard procedures in living donation, the sequential use of the two laparoscopic approaches in the same living donor has never been reported. Herein, we present the first two case series of laparoscopic liver-kidney procurement in the same living donor for SeqLKT in children with PH1 and review of the current literature on this topic.
View Article and Find Full Text PDFTo evaluate the use and benefits of social networking sites (SNS) and mobile applications (MA) in young patients with kidney disease (KD). Pediatric KD is prevalent. The Internet is increasingly being used to communicate rapid healthcare information to children about acute and chronic diseases with greater medical care satisfaction.
View Article and Find Full Text PDFBackground: The past few decades have witnessed a tremendous development in the field of genetics. The implementation of next generation sequencing (NGS) technologies revolutionized the field of molecular biology and made the genetic information accessible at a large scale. However, connecting a rare genetic variation to a complex phenotype remains challenging.
View Article and Find Full Text PDFCytomegalovirus (CMV) remains one of the most important pathogen responsible for the morbidity and mortality of transplantation patients. The impact on recipients depends on the form of CMV infection knowing that 10% to 50% develop symptomatic disease while solid organ involvement if presumed (e.g.
View Article and Find Full Text PDFKidney transplantation is now accepted to be the best treatment for end-stage renal disease. Despite the improvement of immunosuppressive therapy, there is still actually substantial loss of allografts, in part due to uncontrolled humoral immunity. For many years, the primary technique for the detection of anti-HLA antibodies was the CDC (complement dependent cytotoxicity).
View Article and Find Full Text PDFKidney transplantation is the treatment of choice of end stage kidney disease. Over the years, kidney transplantation progressed tremendously, mainly by the improvement of immunosuppressive drugs used in the prevention of acute rejection. Since the introduction of cyclosporine in the 80s, many immunosuppressive protocols have been established.
View Article and Find Full Text PDFObjectives: This study is to compare the short and long-term renal function between adult recipients of living laparoscopic and open donors (LR vs OR) to highlight the effect of the surgical technique on graft function. Moreover, we chose to compare the organic (hypertension, proteinuria, serum creatinine) and psychological (aspect of libido, need for anxiolytics) long-term effect of the surgery between laparoscopic and open donors (LD vs OD).
Methods: After census of adult recipients and living donors between 2003 and 2012, and after application of exclusion criteria and selection of homogeneous groups of donors and recipients, a retrospective cohort study was conducted between October 2008 and January 2012.
Introduction: During the period from September 1993 to March 2011, 79 kidney transplants in children and adolescents less than 18 years old were performed in our department; fifty-six in the familial group and 13 in the cadaveric group. We report in this study: immunosuppression protocols, medical and surgical complications (including rejection episodes), graft and patient survival.
Material And Methods: Immunosuppression protocol has been modified throughout those eighteen years.
Hereditary defects of coenzyme Q10 biosynthesis cause steroid-resistant nephrotic syndrome (SRNS) as part of multiorgan involvement but may also contribute to isolated SRNS. Here, we report 26 patients from 12 families with recessive mutations in ADCK4. Mutation detection rate was 1.
View Article and Find Full Text PDFWe sought to ascertain the long-term outcome and genotype-phenotype correlations available for primary hyperoxaluria type 1 in a large retrospective cohort study. We examined the clinical history of 155 patients (129 families primarily from Western Europe, North Africa, or the Middle East) as well as the enzymatic or genetic diagnosis. The median age at first symptom was 4 years, and at diagnosis 7.
View Article and Find Full Text PDFSalmonella species are a rare cause of urinary tract infections in children. They have been associated with a higher incidence of structural abnormalities or immunosuppressive status. We report the case of an 11-year-old girl with urinary tract infection (UTI) secondary to Salmonella typhi and associated with urolithiasis.
View Article and Find Full Text PDFObjectives: Diagnosis, initial workup and immediate prognosis in congenital or acquired heart diseases revealed by cardiogenic shock (CS) in a Lebanese paediatric multicentric study.
Material And Methods: During a six-year period (1st January 1999 to 31 December 2004), 56 paediatric patients aged between 0 and 7 years have been admitted to the emergency departments or neonatal and intensive care units in ten different Lebanese hospitals, for high degree of CS suspicion with underlying cardiac disease. All diagnoses were confirmed by echocardiography.
Ciclosporine was launched in therapeutic use in 1978. It marked the transplantation era due to the success of organ transplantation. Ciclosporine is actually used in bone marrow transplantation and in the transplantation of slid organs such as kidney, liver, heart, lungs, lung-heart, pancreas and bowel.
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