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BACKGROUND: Evaluating glomerular filtration rate (GFR) remains challenging in pediatrics; new formulas were developed to increase performance of GFR estimation (eGFR). We aimed to evaluate the recently published formulas as applied to another pediatric population. METHODS: A retrospective study was conducted in a cohort of 307 patients with a "kidney risk" (mean age 12.1 ± 4.5 years, sex ratio 1/1) assessed in a tertiary pediatric nephrology center and a mean measured GFR (mGFR) using plasma iohexol clearance of 85.5 ± 25.3 mL/min/1.73 m2; creatinine levels were measured by IDMS-standardized enzymatic method and cystatin C by immunonephelometry. The following eGFRs were calculated: Schwartz2009, Schwartz-Lyon, CKiDU25creat, and EKFC for eGFR using creatinine (eGFR-creat), CKiDU25cys and FAScys for eGFR using cystatin (eGFR-cys) as well as combined SchwartzCreat-Cys, average (CKiDU25creat-CKiDU25cys), and average (EKFC-FAScys) for eGFR using both biomarkers. The performance of the different formulas was evaluated compared to mGFR by absolute bias measurement and accuracy (p10%, p30%). Results are expressed as mean ± SD. RESULTS: Creatinine-based formulas and especially the new CKiDU25 and EKFC overestimate GFR, even in children with normal kidney function. However, the bias is constant with these two formulas whatever the age group or gender, contrary to the previously published formulas. In contrast, cystatin C-based equations and combined formulas showed good performance in all age groups and all medical conditions with an acceptable bias and p30%. CONCLUSIONS: In our pediatric population, the performance of all creatinine-based formulas is inadequate with significant GFR overestimation, mainly in subjects with mGFR > 75 mL/min/1.73 m2. Conversely, cystatin C-based or combined formulas have acceptable performance in patients followed in a tertiary pediatric nephrology unit.
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Kidney failure has a negative impact on both children and families' quality of life (QOL). We evaluated the burden of home peritoneal dialysis (PD) using two local questionnaires and the French version of PedsQL3.0 end-stage kidney disease module and family impact module. Data are expressed as median (min-max). We reviewed the charts of 12 patients, at a median age of 8.8 (1.2-16.7) years, undergoing maintenance PD for 8 (1-42) months. Parathyroid hormone and haemoglobin levels were 215 (17-606) ng/L and 117 (104-141) g/L, respectively. Patients were taking 7 (3-10) different medications, corresponding to 9 (4-17) doses per day. The PD fluid volume per cycle was 1035 (723-1348) mL/m2 with a dwell duration of 75 (60-90) min and 6 (5-9) cycles per night. On a 2-week period, there were 2 (1-11) alarms per night resulting in 2 (0-8) times waking up and getting out of the bed for the parent(s); families were late 1 (0-11) times for school or parent's work. The time spent to connect and disconnect the cycler to the patient was 30 (12-46) min per day. QOL score on child self-report was correlated positively with weight percentile for age (R = 0.857; p = 0.014) and negatively with the number of siblings (R = -0.917; p = 0.004). The children QOL was evaluated higher by self-report scores: 77 (59-87) than by parent-proxy report scores: 53 (29-74), respectively (p = 0.028). PD children/teenagers and their caregivers can feel overwhelmed by the daily home therapy. Self-report and parent-proxy report QOL were significantly different, and it is questionable whether the parent-proxy report QOL relies rather on parents' own QOL.
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Introduction: Patients with short bowel syndrome (SBS) may exhibit enteric hyperoxaluria (EH), and the prevalence of oxalate nephropathy in SBS is likely underestimated. Plasma oxalate (POx) is a surrogate of systemic oxalate deposition and, consequently, may increase the risk of developing chronic kidney disease (CKD). The main objective of this study was to explore the distribution of POx levels in patients with SBS. Methods: Patients followed for SBS were recruited prospectively in the OXAGO study (NCT04119765) to assess POx during their annual renal follow-up including iohexol clearance. The inclusion criteria were age ≥18 years, and SBS type 2 and type 3 for more than 6 months. Results: A total of 47 patients were included but only 45 patients has a measured POx (55% males, 80% SBS type 2, 66% parenteral nutrition, 61% kidney stone history). POx levels were 6.8 ± 4.4 µmol/l, 29% of patients had POx ≥5 µmol/l. In the whole cohort, mean urinary oxalate (UOx) was 648±415 and 54% were >500 µmol/24h. In the group of patients with high POx levels (HPO), 24-hour urine oxalate was significantly higher than in the group with normal POx levels (NPO) (919 ± 566 vs. 526 ± 257 µmol/l; P = 0.003). Glomerular filtration rate (GFR) was 66 ± 22 ml/min per 1.73 m2, and 91% had CKD. GFR was significantly lower in the HPO than in the NPO group (49 ± 23 vs. 73 ± 18 ml/min per 1.73 m2; P = 0.0005. Conclusion: Patients with SBS can display increased POx levels even with GFR >30 ml/min per 1.73 m2. POx may be an interesting biomarker to assess the severity of EH.
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X-linked hypophosphatemia (XLH) is a rare, multi-systemic, invalidating disease requiring a multi-disciplinary approach. No specific action in XLH, neither for the patients' specific needs nor for the methodology for the evaluation of these were found. Thus, to identify the needs of XLH patients and their caregivers, we organised focus groups in our reference centre with a view to build educational sessions. Focus groups including either XLH children, XLH adults, or caregivers ran in parallel. Each group was led by a person trained in therapeutic education (nurse, paediatric nephrologist) with another healthcare provider specialised in XLH (rheumatologist, nephrologist). One additional person with knowledge of XLH (clinical research associate, paediatric resident) took minutes. The duration of each session was 1.5h; XLH patients/caregivers were asked to answer age-adapted "open questions" on their daily life and quality of life. At the end, a global restitution was made. The needs identified were later grouped and analysed, which allowed us to build the educational sessions. The XLH children group included 5 children, the XLH adults group included 10 adults, and the caregivers group included 6 parents or partners. Major needs were identified: knowledge of XLH, treatment, dental care and adapted physical activity, with additional questions on socio-professional adaptations and financial support in adults. Partner patients were also identified to co-build the support programme. The study allowed us to identify the needs of XLH patients and their caregivers using the focus group method and then, using these needs, to build educational sessions and a therapeutic education programme for XLH patients.
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Raquitismo Hipofosfatémico Familiar , Adulto , Niño , Humanos , Raquitismo Hipofosfatémico Familiar/terapia , Grupos Focales , Calidad de Vida , Cuidadores/educación , RetroalimentaciónRESUMEN
BACKGROUND: Lumasiran is the first RNA interference (RNAi) therapy of primary hyperoxaluria type 1 (PH1). Here, we report on the rapid improvement and even disappearance of nephrocalcinosis after early lumasiran therapy. CASE-DIAGNOSIS/TREATMENT: In patient 1, PH1 was suspected due to incidental discovery of nephrocalcinosis stage 3 in a 4-month-old boy. Bilateral nephrocalcinosis stage 3 was diagnosed in patient 2 at 22 months concomitantly to acute pyelonephritis. Urinary oxalate (UOx) and glycolate (UGly) were increased in both patients allowing to start lumasiran therapy before genetic confirmation. Nephrocalcinosis started to improve and disappeared after 27 months and 1 year of treatment in patients 1 and 2, respectively. CONCLUSION: These cases illustrate the efficacy of early lumasiran therapy in infants to improve and even normalize nephrocalcinosis. As proposed in the 2023 European guidelines, the interest of starting treatment quickly without waiting for genetic confirmation may have an impact on long-term outcomes.
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Hiperoxaluria Primaria , Nefrocalcinosis , Humanos , Nefrocalcinosis/genética , Nefrocalcinosis/diagnóstico , Nefrocalcinosis/terapia , Masculino , Lactante , Hiperoxaluria Primaria/genética , Hiperoxaluria Primaria/diagnóstico , Hiperoxaluria Primaria/terapia , Hiperoxaluria Primaria/orina , Hiperoxaluria Primaria/complicaciones , Tratamiento con ARN de Interferencia/métodos , Resultado del Tratamiento , Glicolatos/uso terapéutico , Glicolatos/orinaRESUMEN
CONTEXT: X-linked hypophosphatemia (XLH) is a rare genetic disorder that results in increased plasma levels of fibroblast growth factor 23 (FGF23). Several studies have demonstrated a direct association between FGF23 and cardiovascular mortality in cohorts of patients with chronic renal failure. However, in patients with XLH, studies on the cardiovascular impact of the disease are rare, with contradictory results. OBJECTIVE: The aim was to assess whether the disease led to an increased cardiovascular risk. METHODS: We conducted a single-center retrospective observational study on a local cohort of adult patients with XLH. The primary endpoint was a composite endpoint of the frequency of left ventricular hypertrophy (LVH) or presence of high blood pressure. Our secondary objectives were to assess echocardiographic, pulse wave velocity, and central blood pressure data as other markers of CV health. Independently of this cohort, tissue sodium content with magnetic resonance imaging was studied in 2 patients with XLH before and after burosumab. RESULTS: Twenty-two patients were included. Median serum phosphate was 0.57 (0.47-0.72) mmol/L and FGF23 94 pg/L (58-2226). Median blood pressure was 124 (115-130)/68 (65-80) mm Hg, with only 9% of patients being hypertensive. A majority of patients (69%) had no LVH, only 1 had a left ventricular mass >100 g/m² and 25% of patients had left ventricular remodeling. Pulse wave velocity was normal in all patients. No differences in skin and muscle sodium content were observed before and after burosumab in the 2 patients who underwent sodium magnetic resonance imaging. CONCLUSION: We found no elevated risk of developing hypertension or LVH in patients with XLH.
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Enfermedades Cardiovasculares , Raquitismo Hipofosfatémico Familiar , Hipertensión , Hipofosfatemia , Adulto , Humanos , Raquitismo Hipofosfatémico Familiar/complicaciones , Enfermedades Cardiovasculares/epidemiología , Enfermedades Cardiovasculares/etiología , Análisis de la Onda del Pulso , Factores de Riesgo , Hipertrofia Ventricular Izquierda/diagnóstico por imagen , Hipertrofia Ventricular Izquierda/epidemiología , Hipertrofia Ventricular Izquierda/etiología , Hipertensión/complicaciones , Hipertensión/epidemiología , Factores de Riesgo de Enfermedad Cardiaca , Sodio , Factores de Crecimiento de Fibroblastos , FosfatosRESUMEN
BACKGROUND: Shrunken pore syndrome (SPS) is defined as cystatin C-based-eGFR (eGFRcys)/creatinine-based-eGFR (eGFRcreat) <0.6 or 0.7 and is associated with an increased cardiovascular risk. SPS has been described in children, but no link to increased morbi-mortality was demonstrated. OBJECTIVES: Study the prevalence of SPS in a pediatric population using several glomerular filtration rate (GFR) estimating formulas and measured GFR and evaluate the potential link with cardiovascular risk. METHODS: In 307 renal risk pediatric patients, we studied prevalence of SPS either with CKiDU25creat and cyst or with FAScreat and cyst and EKFCcreat. The characteristics of patients with SPS (defined with Full-age spectrum equation (FAS) and/or European Kidney Function Consortium equation (EKFC)) were compared. RESULTS AND CONCLUSION: The prevalence of SPS varies widely depending on the threshold and the formulas used. Higher C-reactive protein (CRP) and phosphate levels and smaller size are observed in children with SPS defined with FAS and/or EKFC and might be associated with long-term increased cardiovascular risk. Further studies in wider general pediatric populations are warranted.
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Quistes , Insuficiencia Renal Crónica , Humanos , Niño , Riñón , Tasa de Filtración Glomerular , Proteína C-Reactiva , Síndrome , Creatinina , Insuficiencia Renal Crónica/epidemiologíaRESUMEN
Nephrolithiasis affects around 10% of the population and is frequently associated with impaired dietary factors. The first one is insufficient fluid intake inducing reduced urine volume, urine supersaturation, and subsequently urinary lithiasis. Kidneys regulate 24 h urine volume, which, under physiological conditions, approximately reflects daily fluid intake. The aim of this study is to synthesize and highlight the role of hydration in the treatment of nephrolithiasis. Increasing fluid intake has a preventive effect on the risk of developing a first kidney stone (primary prevention) and also decreases the risk of stone recurrence (secondary prevention). Current guidelines recommend increasing fluid intake to at least at 2.5 L/day to prevent stone formation, and even to 3.5-4 L in some severe forms of nephrolithiasis (primary or enteric hyperoxaluria or cystinuria). Fluid intake must also be balanced between day and night, to avoid urinary supersaturation during the night. Patients should be informed and supported in this difficult process of increasing urine dilution, with practical ways and daily routines to increase their fluid intake. The liquid of choice is water, which should be chosen depending on its composition (such as calcium, bicarbonate, or magnesium content). Finally, some additional advice has to be given to avoid certain beverages such as those containing fructose or phosphoric acid, which are susceptible to increase the risk of nephrolithiasis.
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Cistinuria , Hiperoxaluria , Cálculos Renales , Adulto , Humanos , Cálculos Renales/prevención & control , Riñón , Calcio de la Dieta , Cistinuria/complicacionesRESUMEN
BACKGROUND: From 2006 to 2020, 24% of children starting haemodialysis in France weighed < 20 kg. Most new-generation long-term haemodialysis machines do not propose paediatric lines anymore but Fresenius has validated two devices for use in children above 10 kg. Our aim was to compare the daily use of these two devices in children < 20 kg. METHODS: Retrospective single-center evaluation of daily practice with Fresenius 6008® machines, and low-volume paediatric sets (83 mL), as compared to 5008® machines with paediatric lines (108 mL). Each child was treated randomly with both generators. RESULTS: A total of 102 online haemodiafiltration sessions were performed over 4 weeks in five children (median body weight 12.0 [range 11.5-17.0] kg). Arterial aspiration and venous pressures were maintained respectively over - 200 mmHg and under 200 mmHg. For all children, blood flow and volume treated per session were lower with 6008® vs. 5008® (p < 0.001), median difference between the two devices being 21%. In the four children treated in post-dilution mode, substituted volume was lower with 6008® (p < 0.001, median difference: 21%). Effective dialysis time was not different between the two generators; however, the difference between total duration of session and dialysis effective time was slightly higher (p < 0.05) with 6008® for three patients, due to treatment interruptions. CONCLUSION: These results suggest that children between 11 and 17 kg should be treated with paediatric lines on 5008® if possible. They advocate for modification of the 6008 paediatric set to decrease resistance to blood flow. The possibility to use 6008® with paediatric lines in children below 10 kg deserves further studies.
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Hemodiafiltración , Riñones Artificiales , Niño , Humanos , Hemodiafiltración/métodos , Hemodinámica , Diálisis Renal/métodos , Estudios RetrospectivosRESUMEN
PURPOSES OF REVIEW: With chronic kidney disease (CKD) progression, secondary hyperparathyroidism (sHPT) and mineral and bone metabolism disease (MBD) almost inevitably develop and result in renal osteodystrophy and cardiovascular disease (CVD). Together with active vitamin D, calcimimetics are the main therapy for sHPT in CKD. This review provides an overview of the therapeutic effects of oral cinacalcet and intravenous etelcalcetide on CKD-MBD and vascular disease, with a focus on pediatric dialysis patients. RECENT FINDINGS: Randomized controlled trials in adults and children demonstrate efficient lowering of parathyroid hormone (PTH) by the calcimimetics together with a reduction in serum calcium and phosphate when combined with low-dose active vitamin D, while therapy with active vitamin D analogs alone increases serum calcium and phosphate. Cinacalcet and etelcalcetide both improve bone formation and correct adynamic bone, i.e., have a direct bone anabolic effect. They decrease serum calciprotein particles, which are involved in endothelial dysfunction, atherogenesis, and vascular calcification. Clinical trials in adults suggest a modest slowing of the progression of cardiovascular calcification with cinacalcet. Calcimimetic agents represent a major pharmacological tool for improved control of CKD-MBD, by efficiently counteracting sHPT and allowing for better control of calcium/phosphate and bone homeostasis. Albeit definite evidence is lacking, the beneficial effects of calcimimetics on CVD are promising. Routine use of cinacalcet has been suggested in children.
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Enfermedades Cardiovasculares , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica , Hiperparatiroidismo Secundario , Insuficiencia Renal Crónica , Adulto , Humanos , Niño , Cinacalcet/uso terapéutico , Diálisis Renal , Calcio/uso terapéutico , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/tratamiento farmacológico , Trastorno Mineral y Óseo Asociado a la Enfermedad Renal Crónica/complicaciones , Hiperparatiroidismo Secundario/tratamiento farmacológico , Hiperparatiroidismo Secundario/etiología , Calcimiméticos/uso terapéutico , Hormona Paratiroidea , Vitamina D/uso terapéutico , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/terapia , Minerales , Fosfatos/metabolismoRESUMEN
OBJECTIVE: To evaluate whether urine output (UO), rarely assessed in the literature, is associated with relevant neonatal outcomes in very preterm infants, and which UO threshold may be the most clinically relevant. DESIGN: Retrospective cohort study. SETTING: Two Level IV neonatal intensive care units. PATIENTS: Very preterm infants born between 240/7 and 296/7 weeks of gestation documented with eight UO measurements per day between postnatal day 1 and day 7. MAIN OUTCOME MEASURES: Composite outcome defined as death before discharge, or moderate to severe bronchopulmonary dysplasia, or severe brain lesions. The association between this outcome and UO was studied using several UO thresholds. RESULTS: Among 532 infants studied, UO <1.0 mL/kg/hour for at least 24 consecutive hours was measured in 55/532 (10%) infants and the primary outcome was recorded in 25 patients. The association between a UO threshold <1.0 mL/kg/hour and the primary outcome was found marginally significant (crude OR 1.80, 95% CI 1.02 to 3.16, p=0.04). The primary outcome was recorded in 112/242 (46%) patients with a UO <2.0 mL/kg/hour and only 64/290 (22%) patients with a UO ≥2.0 mL/kg/hour (p<0.001). This UO threshold was found significantly associated with the primary outcome (crude OR 3.1, 95% CI 2.1 to 4.7, p<0.001), an association confirmed using a multivariate logistic regression model including baseline covariates (adjusted OR 3.7, 95% CI 2.2 to 6.4, p<0.001). CONCLUSION: A UO <2 mL/kg/hour over 24 hours between postnatal day 1 and day 7 strongly predicts neonatal mortality or severe morbidities in very preterm infants.
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Displasia Broncopulmonar , Enfermedades del Prematuro , Lactante , Femenino , Recién Nacido , Humanos , Estudios Retrospectivos , Recien Nacido Prematuro , Enfermedades del Prematuro/diagnóstico , Recién Nacido de muy Bajo PesoRESUMEN
BACKGROUND AND OBJECTIVES: The current threshold used for oliguria in the definition of neonatal AKI has been empirically defined as 1 ml/kg per hour. Urine output criteria are generally poorly documented, resulting in uncertainty in the most accurate threshold to identify AKI in very preterm infants with known tubular immaturity. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a bicentric study including 473 very preterm infants (240/7-296/7 weeks of gestation) born between January 2014 and December 2018 with urine output measurements every 3 hours during the first 7 days of life and two serum creatinine measurements during the first 10 days of life. AKI was defined using the neonatal Kidney Disease Improving Global Outcomes (KDIGO) definition. We tested whether higher urine output thresholds (1.5 or 2 ml/kg per hour) in modified AKI definitions may better discriminate neonatal mortality compared with the current definition. RESULTS: Early-onset AKI was developed by 101 of 473 (21%) very preterm infants. AKI was diagnosed on the basis of urine output criteria alone (no rise in creatinine) for 27 of 101 (27%) participants. Early-onset AKI was associated with higher risk of death before discharge (adjusted odds ratio, 3.9; 95% confidence interval, 1.9 to 7.8), and the AKI neonatal KDIGO score showed good discriminative performance for neonatal mortality, with an area under the receiver operating characteristic (ROC) curve of 0.68 (95% confidence interval, 0.61 to 0.75). Modified AKI definitions that included higher urine output thresholds showed significantly improved discriminative performance, with areas under the ROC curve of 0.73 (95% confidence interval, 0.66 to 0.80) for the 1.5-ml/kg per hour threshold and 0.75 (95% confidence interval, 0.68 to 0.81) for the 2-ml/kg per hour threshold. CONCLUSIONS: Early-onset AKI was diagnosed on the basis of urine output exclusively for a quarter of the cases. Furthermore, modified AKI definitions that included higher urine output improved the discriminative performance for predicting mortality.
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Lesión Renal Aguda , Recien Nacido Prematuro , Creatinina , Humanos , Lactante , Recién Nacido , Recién Nacido de muy Bajo Peso , Oliguria/diagnóstico , Oliguria/etiologíaRESUMEN
Although extracorporeal cardiopulmonary resuscitation (ECPR) is increasingly utilized in the pediatric critical care environment, our understanding regarding pediatric candidacy for ECPR remains unknown. Our objective is to explore current practice and indications for pediatric ECPR. Scenario-based, self-administered, online survey, evaluating clinical determinants that may impact pediatric ECPR initiation with respect to four scenarios: postoperative cardiac surgery, cardiac failure secondary to myocarditis, septic shock, and chronic respiratory failure in a former preterm child. Responders are pediatric critical care physicians from four societies. 249 physicians, mostly from North America, answered the survey. In cardiac scenarios, 40% of the responders would initiate ECPR, irrespective of CPR duration, compared with less than 20% in noncardiac scenarios. Nearly 33% of responders would consider ECPR if CPR duration was less than 60 minutes in noncardiac scenarios. Factors strongly decreasing the likelihood to initiate ECPR were out-of-hospital unwitnessed cardiac arrest and blood pH <6.60. Additional factors reducing this likelihood were multiple organ failure, pre-existing neurologic delay, >10 doses of adrenaline, poor CPR quality, and lactate >18 mmol/l. Pediatric intensive care unit location for cardiac arrest, good CPR quality, 24/7 in-house extracorporeal membrane oxygenation (ECMO) team moderately increase the likelihood of initiating ECPR. This international survey of pediatric ECPR initiation practices reveals significant differences regarding ECPR candidacy based on patient category, location of arrest, duration of CPR, witness status, and last blood pH. Further research identifying prognostic factors measurable before ECMO initiation should help define the optimal ECPR initiation strategy.
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Reanimación Cardiopulmonar , Oxigenación por Membrana Extracorpórea , Paro Cardíaco , Reanimación Cardiopulmonar/efectos adversos , Reanimación Cardiopulmonar/métodos , Niño , Oxigenación por Membrana Extracorpórea/efectos adversos , Oxigenación por Membrana Extracorpórea/métodos , Paro Cardíaco/terapia , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico , Estudios RetrospectivosRESUMEN
INTRODUCTION: Neutrophil gelatinase-associated lipocalin (NGAL) has been proposed as an early acute kidney injury (AKI) biomarker in the neonatal population. Our goal is to describe this biomarker behavior in this high-risk population, in absence of AKI as confirmed by inulin clearance. MATERIALS AND METHODS: Prospective study including 42 preterm newborns (mean gestational age: 30.7 ± 2.3 weeks) with a urinary NGAL collection between day 1 and 6 of life. RESULTS: Median urinary neutrophil gelatinase-associated lipocalin (uNGAL) value is 122.8 ng/ml (7-1981.5 ng/ml). Statistically significant higher uNGAL values are found in female. uNGAL median values are decreasing when comparing extremely, very, and late preterm groups (812.2 ng/ml [75.8-1453.9] vs. 124.4 ng/ml [31.4-1981.5] vs. 65.3 ng/ml [7.1-1091]). There is a statistically significant inverse correlation between gestational age and uNGAL values (Pearson's coefficient r= -0.37). uNGAL median values are higher in groups exposed to gentamicin, neonatal asphyxia, early onset sepsis, or patent ductus arteriosus. Median inulin clearance is 18.8 ml/min/1.73 m2 [14.8-25.5 ml/min/1.73 m2]. There is no correlation between uNGAL values and inulin clearance results (Pearson's coefficient r=-0. 29, p: .06). CONCLUSIONS: In this preterm newborn's series without AKI, the median uNGAL and its high variability are in accordance with published reference ranges. Correlation between uNGAL and gestational age exists, as well as gender impact. Newborns exposed to different renal insults present higher uNGAL values, suggesting potential undetected tubular toxicity or reflecting NGAL production in case of inflammatory or ischemic processes.
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Lesión Renal Aguda , Inulina , Femenino , Humanos , Recién Nacido , Lesión Renal Aguda/diagnóstico , Biomarcadores , Lipocalina 2 , Estudios Prospectivos , Recien Nacido PrematuroRESUMEN
Tacrolimus is a calcineurin inhibitor characterized by a narrow therapeutic index and high intra- and inter-individual pharmacokinetic variability. Therapeutic drug monitoring in whole-blood is the standard monitoring procedure. However, tacrolimus extensively binds to erythrocytes, and tacrolimus whole-blood distribution and whole-blood trough concentrations are strongly affected by hematocrit. High whole-blood tacrolimus concentrations at low hematocrit may result in high unbound plasma concentrations and increased toxicity. We present the case of a 16-year-old girl with kidney and liver transplant in whom low concentrations of tacrolimus in the context of low hematocrit led to significant increase in the dosage of tacrolimus and participate, along with a genetic polymorphism of ABCB1, in nephrotoxicity.
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There is growing evidences of long-term renal and cardiovascular consequences of prematurity, intra-uterine growth restriction, and neonatal acute kidney injury (AKI). We performed an online survey to describe current pediatric management in this population, sent to 148 ambulatory pediatricians in Geneva. Among the 40% of pediatricians who completed the survey, 43% modify their blood pressure measurement practice in case of neonatal acute kidney injury, 24% and 19% in a context of prematurity or intra-uterine growth restriction, respectively. Twenty-five percent provide information about cardiovascular risk factors or catch up growth. In case of prematurity or intra-uterine growth restriction, renal tests (ultrasound, serum creatinine, micro albuminuria) or referral to nephrologist were realized by less than 5% of the pediatricians. For neonatal acute kidney injury, renal tests, and referral to specialists are performed by 30 and 60% of pediatricians, respectively. When prematurity or intra-uterine growth restriction was associated with abnormal blood pressure or abnormal renal tests, the referral to the specialist reached 80%.Conclusion: Ambulatory renal and cardio-vascular follow-up in case of neonatal medical history can be enhanced, with necessity to raise awareness and to edict guidelines available to pediatricians. What is Known: ⢠There is a compelling evidence of long-term renal and cardiovascular consequences of prematurity and low birth weight. ⢠Specific cardiovascular and renal follow-up guidelines, coming from professional organizations, are currently not available for these patients. What is New: ⢠Pediatricians in ambulatory setting do not adapt their renal and cardiovascular follow-up in case of neonatal medical history. ⢠There is a necessity to raise awareness about these long-term consequences among pediatricians and to edict guidelines available to them.
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Lesión Renal Aguda , Neonatología , Niño , Creatinina , Estudios de Seguimiento , Humanos , Recién Nacido , Alta del Paciente , PediatrasRESUMEN
To improve survival rates during cardiopulmonary resuscitation (CPR), some patients are put on extracorporeal life support (ECLS) during active resuscitation (ECPR). Our objective was to assess the clinical outcomes after pediatric ECPR in Switzerland and to determine pre-ECPR prognostic factors for mortality. The present study is a retrospective analysis. The study setting included three pediatric intensive care units in Switzerland that use ECPR. All patients (<16 years old) undergoing ECPR from 2008 to 2016 were included in the study. There were no interventions. Data before ECLS initiation and clinical outcomes were collected. An ECPR score was designed to predict mortality, based on variables significantly different between survivors and non-survivors. Fifty-five patients were included, with a median age of 13.5 months. Eighty percent were cardiac patients. The mortality rate was 75%. Mortality was significantly associated with CPR duration ( p = 0.02), last lactate ( p = 0.05), and last pH ( p = 0.01) before ECLS initiation. Based on these three variables, an ECPR score was designed as follows: CPR duration (in minutes): 1 point if < 40; 2 points if ≥ 40; 3 points if ≥ 60; 6 points if ≥ 105. Lactate (in mmol/L): 1 point if < 8; 2 points if ≥ 8; 3 points if ≥ 14; 6 points if ≥ 18. pH: 1 point if > 7.00; 2 points if ≤ 7.00; 3 points if ≤ 6.85; 6 points if ≤ 6.60. The area under the receiver-operating characteristic curve was 0.74. The positive predictive value of a score ≥ 9 was 94%. In our population, a score based on three variables easily available prior to ECLS initiation had good discrimination and could appropriately predict mortality. This score now needs validation in a larger population.
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BACKGROUND AND OBJECTIVES: In 2009, the pandemic influenza A/H1N1 accounted for worldwide recommendations about vaccination. There are few data concerning the immunogenicity or the security of the adjuvanted-A/H1N1 vaccine in transplanted and hemodialyzed patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Sera from 21 controls, 53 hemodialyzed (HD) patients, and 111 renal transplant recipients (RT) were sampled before (T0) and 1 month after (T1) a single dose of Pandemrix® vaccine (GSK Biologicals, AS03-adjuvanted). We measured the neutralizing antibodies against A/H1N1/2009, the geometric mean (GM) titers, the GM titer ratios (T1/T0) with 95% confidence intervals, and the seroconversion rate (responders: ≥4-fold increase in titer). The HLA and MICA immunization was determined by Luminex technology. RESULTS: The GM titer ratio was 38 (19 to 78), 9 (5 to 16), and 5 (3 to 6) for controls, HD patients, and RT patients, respectively (P < 0.001). The proportion of responders was 90%, 57%, and 44%, respectively (P < 0.001). In RT patients, the prevalence of histocompatibility leukocyte antigen (HLA) class I, histocompatibility leukocyte antigen class II, and MHC class I-related chain A immunization, was, respectively, 15%, 14%, and 14% before and 14%, 14%, and 11% after vaccination (P = 1, 1, and 0.39). CONCLUSIONS: The influenza A/H1N1-adjuvanted vaccine is of limited efficacy but is safe in renal disease populations. The humoral response is lower in transplanted versus hemodialyzed patients. Further studies are needed to improve the efficacy of vaccination in those populations.