Effect of urine alkalization on urinary inflammatory markers in cystinuric patients.

Background: Cystinuria is associated with a high prevalence of chronic kidney disease (CKD). We previously described a urinary inflammatory-protein signature (UIS), including 38 upregulated proteins, in cystinuric patients (Cys-patients), compared with healthy controls (HC). This UIS was higher in Cys-patients with CKD.

How useful is an oral calcium load test for diagnosing recurrent calcium stone formers?

Hypercalciuria is the main risk factor for recurrent calcium urolithiasis. The goal of our study is to determinate how useful an oral calcium load test is for stone formers to classify different forms of hypercalciuria in pathogenetic categories defined as renal or absorptive according to the current knowledge. Between June 2013 and February 2016, a prospective study was carried out on 117 documented recurrent hypercalciuric stone formers undergoing an oral calcium load test modified from the original description by Pak.

How the diagnosis and the management of genetic renal phosphate leak impact the life of kidney stone formers?

Genetic renal phosphate leak is one of the rare disorders in recurrent stone formers with absorptive hypercalciuria. Diagnosis and appropriate management may change the life of patients. To provide answers on how and when to make the diagnosis of genetic renal phosphate leak and how medical management prevents the recurrences and changes patients' life, we conducted a retrospective study including nine patients with recurrent nephrolithiasis and a confirmed genetic mutation of a phosphate transporter between 2008 and 2019 in our multidisciplinary center at the Pitié Salpetriere Hospital, Paris, France.

Long-term health-related quality of life outcomes of adults with pediatric onset of frequently relapsing or steroid-dependent nephrotic syndrome.

Background: Long-term psychosocial outcomes and health-related quality of life (HRQOL) in adults with pediatric onset of frequently relapsing or steroid-dependent idiopathic nephrotic syndrome (FRNS or SDNS) remain to be determined.

Methods: In this prospective cohort study, 59 adults with pediatric onset of FRNS/SDNS and persistent active glomerular disease in adulthood completed the GEDEPAC-2 questionnaire exploring 11 well-being domains. Data were compared to the French general population (FGP) with standardized incidence ratio ([SIR]; adjusted for period, age, gender).

[Cystinuria].

Cystinuria is the most common monogenic nephrolithiasis disorder. Because of its poor solubility at a typical urine pH of less than 7, cystine excretion results in recurrent urinary cystine stone formation. A high prevalence of high blood pressure and of chronic kidney disease has been reported in these patients.

Adverse events associated with currently used medical treatments for cystinuria and treatment goals: results from a series of 442 patients in France.

Objective: To evaluate medical treatments, in terms of adverse events (AEs) and therapeutic goals, in a large series of patients with cystinuria.

Patients And Methods: Data from 442 patients with cystinuria were recorded retrospectively. Crystalluria was studied in 89 patients.

Impact of pH on Urine Chemistry Assayed on Roche Analyzers.

Background: The pH may impact the concentration of certain urinary parameters, making urine pre-treatment questionable.

Methods: 1) Determining the impact of pH in vitro on the urinary concentration of chemistry parameters assayed on Roche Modular analyzers. 2) Evaluating whether concentrations depended on pH in non-pretreated urines from patients.

Clinical and molecular characterization of cystinuria in a French cohort: relevance of assessing large-scale rearrangements and splicing variants.

Background: Cystinuria is an autosomal recessive disorder of dibasic amino acid transport in the kidney and the intestine leading to increased urinary cystine excretion and nephrolithiasis. Two genes, and , coding respectively for rBAT and b0,+AT, account for the genetic basis of cystinuria.

Methods: This study reports the clinical and molecular characterization of a French cohort including 112 cystinuria patients and 25 relatives from 99 families.

Spectrum of mutations in cystinuria patients presenting with prenatal hyperechoic colon.

Cystinuria is a heterogeneous, rare but important cause of inherited kidney stone disease due to mutations in 2 genes: SLC3A1 and SLC7A9. Antenatal hyperechoic colon (HEC) has been reported in some patients as a non-pathological consequence of the intestinal transport defect. We report 83 patients affected by cystinuria: 44 presented prenatally with a HEC (HEC group) and 39 with a classical postnatal form (CC group).

CKD and Its Risk Factors among Patients with Cystinuria.

Background And Objectives: Cystinuria is an autosomal recessive disorder affecting renal cystine reabsorption; it causes 1% and 8% of stones in adults and children, respectively. This study aimed to determine epidemiologic and clinical characteristics as well as comorbidities among cystinuric patients, focusing on CKD and high BP.

Design, Setting, Participants, & Measurements: This retrospective study was conducted in France, and involved 47 adult and pediatric nephrology and urology centers from April 2010 to January 2012.

[Urinary stone management at the time of its discovery].

Circumstances of diagnosis of urinary stones vary. Medical management is performed either in emergency for acute or serious symptoms, or delayed. Renal colic is the most typical clinical form.

[Simultaneous bilateral percutaneous nephrolithotomy: Series of 60 cases].

Objective: Retrospective evaluation of the efficacy and morbidity of simultaneous bilateral percutaneous nephrolithotomy (SB-PCNL).

Methods: From January 1993 to July 2009, 60 patients have undergone SB-PCNL over a series of 1709 PCNL. Thirty men and 30 women, mean age 45 years old (13-78), were treated for bilateral renal stones (120 kidneys) of 1177 mm(2) (268-4972 mm(2)); 25 were complete staghorn stones.

[Kidney diseases in HIV-infected patients].

HIV infection has become a global disease, both in geographic terms, since it has spread worldwide, and at the individual level since it affects every organ of the human body. Antiretroviral treatments, in countries where they are easily available, have modified HIV infection into a systemic chronic disease, the life expectancy of which is yet to be determined precisely. Treatments have dramatically changed the pattern of the disease and clinicians now have to face a number of new challenges.

Advanced glycation end products regulate extracellular matrix protein and protease expression by human glomerular mesangial cells.

Advanced glycation end products (AGEs) may play a role in the pathogenesis of diabetic nephropathy, by modulating extracellular matrix turnover. AGEs are known to activate specific membrane receptors, including the receptor for AGE (RAGE). In the present study, we analyzed the various receptors for AGEs expressed by human mesangial cells and we studied the effects of glycated albumin and of carboxymethyl lysine on matrix protein and remodelling enzyme synthesis.

[New ethical issues around dialysis. To dialyze or not to dialyze, that is not the question].

Although a physician's first ethical duty is to master the relevant techniques, it is not enough for kidney specialists to know the major principles of dialysis and apply them to all patients with kidney failure. Historically a truly ethical promise, dialysis revolutionized the management of chronic kidney disease by sparing life for the time needed to wait for renal transplantation. Constrained by a supply considerably lower than demand, the nephrologists of that time selected patients, treating only the young and relatively healthy.

Antiretroviral drug dosing errors in HIV-infected patients undergoing hemodialysis.

Background: Several studies have revealed the frequency of antiretroviral (ARV) drug prescription errors. We analyzed highly active antiretroviral therapy (HAART) prescribing practices for human immunodeficiency virus (HIV)-infected patients undergoing hemodialysis in France.

Methods: Prescribed ARV drug doses in our cohort (consisting of all HIV-infected patients who underwent hemodialysis from 1 January 2002 and were prospectively followed up until 1 January 2004) were compared with the recommended doses for patients undergoing hemodialysis.

Outcome and prognosis factors in HIV-infected hemodialysis patients.

HIV-infected patients who are on hemodialysis have a worse prognosis than noninfected patients who are on hemodialysis. Their outcome in the highly active antiretroviral therapy (HAART) era remains unclear. Outcomes in patients who were enrolled in the French Dialysis in HIV/AIDS (DIVA) cohort were determined in a 2-yr prospective follow-up.

Renal outcome after ciclosporin-induced nephrotoxicity.

Background: Renal outcome after ciclosporin (CsA) is not clear in most studies involving patients with many renal comorbid conditions. We first report on renal function recovery after CsA in previously healthy kidney patients.

Methods: Uveitis patients, enroled in a unique single centre cohort follow-up study initiated in 1987, were prospectively evaluated for plasma creatinine and glomerular filtration rate (GFR) before, during (>2 years) and after (>6 months) CsA therapy.