Exploring systemic inflammation in children with chronic kidney disease: correlates of interleukin 6.

BACKGROUND: Systemic inflammation (SI) is linked to chronic kidney disease (CKD) progression and multiple complications. Data regarding SI biomarkers in pediatric patients are scarce. This case-control and cross-sectional study investigates the correlation of neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), total iron binding capacity (TIBC) and serum albumin to serum interleukin-6 (IL-6). METHODS: NLR and PLR were measured in 53 patients (median age: 12.9 years), including 17 on dialysis and 36 with a median glomerular filtration rate of 39 ml/min/1.73m2, and in 25 age and sex-matched healthy controls. Iron profile, serum albumin and IL-6 were measured in the patient group. IL-6 levels > 3rd quartile were classified as high. RESULTS: Patients presented higher NLR and PLR and particularly those on dialysis (p < 0.001 and p = 0.001). We observed a significant correlation between natural logarithm (ln) of IL-6 (lnIL-6) and NLR (rs = 0.344, p = 0.014), serum albumin (rs = -0.350, p = 0.011) and TIBC (rs = -0.345, p = 0.012) after adjustment for CKD stage, while the correlation between lnIL-6 and PLR was not significant (rs = 0.206, p = 0.151). Combination of NLR, serum albumin and TIBC predicted high IL-6 (13 patients) with an AUC of 0.771 (95% CI 0.608-0.943). Pairing of NLR ≥ 1.7 and TIBC ≤ 300 µg/dL exhibited the highest sensitivity (76.9%), while incorporating serum albumin ≤ 3.8 g/dL along with them achieved the highest specificity (95%) for detecting high IL-6 levels. CONCLUSION: Both NLR and PLR levels increase in CKD, especially in patients on chronic dialysis. NLR, rather than PLR, along with TIBC and serum albumin, are associated with IL-6 in pediatric CKD.

Fibroblast growth-factor 23 and vitamin D are associated with iron deficiency and anemia in children with chronic kidney disease.

BACKGROUND: This cross-sectional study investigates the association of fibroblast growth-factor 23 (FGF23) and other bone mineral parameters with iron status and anemia in pediatric chronic kidney disease (CKD). METHODS: Serum calcium, phosphorus, 25-hydroxyvitamin D (25(OH)D), intact parathormone, c-terminal FGF23, a-Klotho, iron (Fe), ferritin, unsaturated iron-binding capacity, and hemoglobin (Hb) were measured in 53 patients from 5 to 19 years old with GFR < 60 mL/min/1.73 m2. Transferrin saturation (TSAT) was calculated. RESULTS: Absolute (ferritin ≤ 100 ng/mL, TSAT ≤ 20%) and functional iron deficiency (ferritin > 100 ng/mL, TSAT ≤ 20%) were observed in 32% and 7.5% of patients, respectively. In CKD stages 3-4 (36 patients), lnFGF23 and 25(OH)D were correlated with Fe (rs = - 0.418, p = 0.012 and rs = 0.467, p = 0.005) and TSAT (rs = - 0.357, p = 0.035 and rs = 0.487, p = 0.003) but not to ferritin. In this patient group, lnFGF23 and 25(OH)D were correlated with Hb z-score (rs = - 0.649, p < 0.001 and rs = 0.358, p = 0.035). No correlation was detected between lnKlotho and iron parameters. In CKD stages 3-4, in multivariate backward logistic regression analysis, including bone mineral parameters, CKD stage, patient age, and daily alphacalcidol dose as covariates, lnFGF23 and 25(OH)D were associated with low TSΑΤ (15 patients) (OR 6.348, 95% CI 1.106-36.419, and OR 0.619, 95% CI 0.429-0.894, respectively); lnFGF23 was associated with low Hb (10 patients) (OR 5.747, 95% CI 1.270-26.005); while the association between 25(OH)D and low Hb did not reach statistical significance (OR 0.818, 95% CI 0.637-1.050). CONCLUSIONS: In pediatric CKD stages 3-4, iron deficiency and anemia are associated with increased FGF23, independently of Klotho. Vitamin D deficiency might contribute to iron deficiency in this population. A higher resolution version of the Graphical abstract is available as Supplementary information.

Association Between Adipokine Profile, Systemic Inflammation, Muscle and Protein Energy Wasting in Children With Chronic Kidney Disease.

OBJECTIVES: This cross-sectional study explores the association of adipokines and interleukin-6 (IL-6) with muscle and protein energy wasting (PEW) in children with chronic kidney disease (CKD). METHODS: We measured serum adiponectin, leptin, resistin and IL-6 in 53 patients with CKD stage 3-5. Lean tissue (LTI) and fat tissue index (FTI) were estimated by bioimpedance analysis spectroscopy. PEW was defined as muscle wasting [LTI adjusted to height age (LTI HA) z-score < -1.65 SD) and at least 2 of the following: reduced body mass [body mass index adjusted to height age (BMI HA) z-score < -1.65 SD), poor growth [height z-score < -1.88 SD], questionnaire-based decreased appetite, and serum albumin ≤3.8 g/dL. RESULTS: PEW, observed in 8 (15.1%) patients, was more prevalent in CKD stage 5 (P = .010). Among the adipokines, adiponectin, and resistin levels were significantly higher in CKD stage 5 (P < .001, P = .005). Adiponectin was correlated to LTI HA z-score (Rs = -0.417, P = .002), leptin to FTI z-score (Rs = 0.620, P < .001), while no correlation was observed between resistin and body composition parameters. Resistin was the only adipokine correlated to IL-6 (Rs = 0.513, P < .001). After adjustment for CKD stage and patient age, PEW was associated with adiponectin and IL-6 rise by 1 µg/mL and 10 pg/mL respectively (odds ratio (OR) 1.240, 95% confidence interval (CI) 1.040, 1.478 and OR 1.405, 95% CI 1.075-1.836) but not with leptin, while resistin association with PEW lost its significance. CONCLUSIONS: In pediatric CKD, adiponectin is associated with muscle wasting, leptin with adiposity and resistin with systemic inflammation. Adiponectin and cytokine IL-6 may serve as PEW biomarkers.

Impact of de novo donor-specific HLA antibodies on pediatric kidney transplant prognosis in patients with acute declined or stable allograft function.

BACKGROUND: This retrospective multicenter long-term cohort study investigates de novo donor-specific HLA antibodies (dnDSA) impact on allograft survival in pediatric kidney transplantation (KTx), depending on allograft function at dnDSA detection. METHODS: Seventy patients with dnDSA screening in the context of acute allograft dysfunction (AAD) (>50% serum creatinine increase) or routine follow-up were included during a 20-year period. Number of dnDSA specificities and HLA total mean fluorescence intensity (MFI-sum) were collected. RESULTS: Median follow-up time was 8.6 years. Among the 22 dnDSA+ patients, 8 patients presented AAD. Compared with dnDSA- patients, allograft survival was shorter only in dnDSA+/AAD+ patients, regardless of dnDSA detection during the 5-year post-transplant period (9 patients) or later (13 patients) (log rank p < .001 and p < .001, respectively). One dnDSA+/AAD-, 7 dnDSA+/AAD+, and 5 dnDSA- patients lost their allograft. Allograft survival was shorter in dnDSA+/AAD+ patients compared with the 16 dnDSA-/AAD+ patients (log rank p < .001) but did not differ between dnDSA+/AAD- and dnDSA-/AAD- patients (log rank p = .157). dnDSA+/AAD+ and dnDSA-/AAD+ patients presented higher risk of allograft failure compared with the other patient groups after adjustment for recipient age at KTx, donor type, and incidence of delayed graft function (HR 11.322, 95% CI 3.094-41.429, p < .001). Concurrent MFI-sum >10 000 and multiple dnDSA specificities were more significantly associated with AAD, compared with each factor separately (p < .001). CONCLUSIONS: In pediatric KTx, AAD shortens allograft survival in dnDSA+ patients, regardless of dnDSA time detection, and is commonly observed when high MFI-sum concurs with multiple dnDSA specificities. dnDSA without AAD incidence does not determinately affect allograft survival.

Surgical management of Encapsulating Peritoneal Sclerosis (EPS) in children: international case series and literature review.

BACKGROUND: Encapsulating Peritoneal Sclerosis (EPS) is a rare phenomenon in paediatric patients with kidney failure treated with peritoneal dialysis (PD). This study highlights clinical challenges in the management of EPS, with particular emphasis on peri-operative considerations and surgical technique. METHODS: Retrospective analysis of all paediatric patients with EPS treated at the Manchester Centre for Transplantation. RESULTS: Four patients were included with a median duration of 78 months on PD. All patients had recurrent peritonitis (> 3 episodes), and all had symptoms within three months of a change of dialysis modality from PD to haemodialysis or transplant. In Manchester, care was delivered by a multi-disciplinary team, including surgeons delivering the adult EPS surgical service with a particular focus on nutritional optimisation, sepsis control, and wound management. The surgery involved laparotomy, lavage, and enterolysis of the small bowel + / - stoma formation, depending on intra-abdominal contamination. Two patients had a formal stoma, which were reversed at three and six months, respectively. Two patients underwent primary closure of the abdomen, whereas two patients had re-look procedures at 48 h with secondary closure. One patient had a post-operative wound infection, which was managed medically. One patient's stoma became detached, leading to an intra-abdominal collection requiring re-laparotomy. The median length of stay was 25 days, and patients were discharged once enteral feeding was established. All patients remained free of recurrence with normal gut function and currently two out of four have functioning transplants. CONCLUSIONS: This series demonstrates 100% survival and parenteral feed independence following EPS surgery. Post-operative morbidity was common; however, with individualised experience-based decision-making and relevant additional interventions, patients made full recoveries. Health and development post-surgery continued, allowing the potential for transplantation. A higher resolution version of the Graphical abstract is available as Supplementary information.

Block copolymers based on poly(butylene adipate) and poly(L-lactic acid) for biomedical applications: synthesis, structure and thermodynamical studies.

This work describes the synthesis of poly(butylene adipate) (PBAd), by melt polycondensation, poly(l-lactic acid) (PLLA), by ring opening polymerization, and the new block copolymer PLLA/PBAd in ratios 90/10, 95/5, 75/25 and 50/50. Due to the biocompatibility and low toxicity of neat PBAd and PLLA, these copolymers are suitable to be used in biomedical applications. The 1H and 13C nuclear magnetic resonance spectroscopy techniques were employed for structural characterization. The thermal transitions, with an emphasis on crystallization, were assessed by differential scanning calorimetry, supplemented by X-ray diffraction and polarized optical microscopy. Molecular mobility studies were conducted using two advanced techniques, broadband dielectric spectroscopy and thermally stimulated depolarization currents. The results from the structural techniques, in combination with each other, provided proof of the presence of PLLA and PBAd blocks and, moreover, the successful copolymer synthesis. The overall data showed that the different co-polymer compositions result directly in severe changes in the polymer crystal distribution and, indirectly, the formation of PBAd micro/nano domains surrounded by PLLA. Furthermore, it was demonstrated that both the continuity of the two polymers throughout the copolymer volume and the semicrystalline morphology can be tuned to a wide extent. The latter makes these systems quite promising envisaging biomedical applications, including the encapsulation of small molecules, e.g. drug solutions. The molecular mobility map was constructed for these systems for the first time, revealing the local (short scale) and segmental (larger nm scale) mobility of PBAd and PLLA, as well as intermediate behaviors of the copolymers.

Muscle-bone axis in children with chronic kidney disease: current knowledge and future perspectives.

Bone and muscle tissue are developed hand-in-hand during childhood and adolescence and interact through mechanical loads and biochemical pathways forming the musculoskeletal system. Chronic kidney disease (CKD) is widely considered as both a bone and muscle-weakening disease, eventually leading to frailty phenotype, with detrimental effects on overall morbidity. CKD also interferes in the biomechanical communication between two tissues. Pathogenetic mechanisms including systemic inflammation, anorexia, physical inactivity, vitamin D deficiency and secondary hyperparathyroidism, metabolic acidosis, impaired growth hormone/insulin growth factor 1 axis, insulin resistance, and activation of renin-angiotensin system are incriminated for longitudinal uncoordinated loss of bone mineral content, bone strength, muscle mass, and muscle strength, leading to mechanical impairment of the functional muscle-bone unit. At the same time, CKD may also interfere in the biochemical crosstalk between the two organs, through inhibiting or stimulating the expression of certain osteokines and myokines. This review focuses on presenting current knowledge, according to in vitro, in vivo, and clinical studies, concerning the pathogenetic pathways involved in the muscle-bone axis, and suggests approaches aimed at preventing bone loss and muscle wasting in the pediatric population. Novel therapeutic targets for preserving musculoskeletal health in the context of CKD are also discussed.

Association between insulin growth factor-1, bone mineral density, and frailty phenotype in children with chronic kidney disease.

BACKGROUND: This cohort study investigates the association between insulin growth factor-1 (IGF-1), bone mineral density, and frailty phenotype in children with chronic kidney disease (CKD). METHODS: Forty-six patients (median age 14.5 years) were prospectively enrolled. Frailty phenotype was defined as the presence ≥ 3 of the following indicators: suboptimal growth/weight gain (body mass index height age < 5th percentile or height < 3rd percentile or loss of ≥ 10 percentiles/year in at least one parameter), low muscle mass (lean tissue mass height age < 5th percentile or loss of ≥ 10 percentiles/year), general fatigue reported by parent or child, and C-reactive protein > 3 mg/l. Lumbar bone mineral apparent density (LBMAD) was measured by dual-energy X-ray absorptiometry, body composition by bioimpedance spectroscopy, and IGF-1 by enzyme-labeled chemiluminescent immunometric assay. RESULTS: Frailty phenotype (seven patients) was more frequent in advanced CKD (estimated glomerular filtration rate < 30 ml/min/1.73m2) (p = 0.014). IGF-1 and LBMAD z-scores were lower in patients with suboptimal growth/weight gain (14 patients) (p = 0.013, p = 0.012), low muscle mass (nine patients) (p = 0.001, p = 0.009), and general fatigue (eight patients) (p < 0.001, p = 0.004). IFG-1 and LBMAD z-scores were associated with frailty phenotype (OR 0.109, 95% CI 0.015-0.798 and OR 0.277, 95% CI 0.085-0.903) after adjustment for CKD stage. IGF-1 z-score was associated with LBMAD < 5th percentile (six patients) (OR 0.020, 95% CI 0.001-0.450) after adjustment for CKD stage. The association between LBMAD and frailty phenotype lost significance after adjustment for IGF-1. CONCLUSION: Frailty phenotype is more frequent in advanced pediatric CKD. IGF-1 is negatively associated with frailty phenotype and interferes in the association between frailty and LBMAD.

Association between relative fat mass, uric acid, and insulin resistance in children with chronic kidney disease.

INTRODUCTION: This cross-sectional study investigates the association between insulin resistance (IR) and serum uric acid (sUA) and relative fat (RFM) and lean mass (RLM) profiles in children with chronic kidney disease (CKD). MATERIAL AND METHODS: RLM and RFM were assessed by bioimpedance spectroscopy in 41 children and adolescents. Normal weight obesity (NWO) was defined as normal height-age body mass index and RFM >85th percentile, according to age and sex. Homeostatic model assessment of insulin resistance (HOMA-IR) level >95th percentile, according to sex and pubertal stage, and sUA >7 mg/dl were used to define IR and hyperuricemia, respectively. RESULTS: High RFM (15 patients) and NWO (7 patients) were associated with higher HOMA-IR in total (p < 0.001) and normal-weight patients (p = 0.004), respectively. RFM was positively and RLM negatively correlated to HOMA-IR (rs = 0.500, p = 0.001 and rs = -0.539, p < 0.001, respectively) and sUA (rs = 0.370, p = 0.017 and rs = -0.325, p = 0.038, respectively), while sUA was positively correlated to HOMA-IR (rs = 0.337, p = 0.031). Hyperuricemia (16 patients) was positively associated with higher RFM and HOMA-IR (p = 0.001 and p = 0.010, respectively). The correlation between sUA and HOMA-IR lost significance after adjustment for RFM. In logistic regression analysis, a 5% increase in RFM was associated with IR (11 patients) independently of the age, sex, sUA, and CKD stage in both total (OR 2.174, 95% CI 1.115-4.225) and normal-weight (OR 3.504, 95% CI 1.110-11.123) patients. CONCLUSION: Children with high RFM, including those presenting NWO, are at risk for IR regardless of CKD stage. RFM is probably the mediator of the link between sUA and IR.

Antibody-mediated rejection with the presence of glomerular crescents in a pediatric kidney transplant recipient: A case report.

Glomerular crescents in kidney transplantation are indicative of severe glomerular injury and constitute a hallmark of RPGN. Their concurrence with ABMR has been rarely described only in adult patients. We report a case of 10-year-old boy with compound heterozygous Fin-major Finnish-type congenital nephrotic syndrome, who had received a deceased-donor kidney transplant 5 years before onset of acute kidney injury and nephrotic range proteinuria without hematuria. Kidney allograft biopsy illustrated 6 glomeruli with global sclerosis and 6 with remarkable circumferential or segmental cellular crescents. Negative glomerular immunofluorescence for immune-complex deposits and the absence of serum ANCA eliminated the presence of immune-mediated and ANCA-positive pauci-immune crescentic glomerulonephritis. Diagnosis of ABMR was based on the high levels of HLA class II DSA and the histological evidence of glomerulitis, peritubular capillaritis, and acute tubular injury with positive linear peritubular capillary C4d staining. The patient despite plasmapheresis and enhanced immunosuppressive treatment progressed to end-stage renal disease. We conclude that glomerular crescents may represent a finding of AMBR and possibly a marker of poor allograft prognosis in pediatric patients.

Ultrasound dilution and thermodilution versus color Doppler ultrasound for arteriovenous fistula assessment in children on hemodialysis.

BACKGROUND: This study is aimed at comparing ultrasound dilution (UD) and thermodilution (TD) with color Doppler ultrasound (CDU) for arteriovenous fistula (AVF) assessment in children on hemodialysis (HD). MATERIAL AND METHODS: All patients were dialysed with the Fresenius 5008 HD machine. UD was performed using the Transonic device. The two methods were compared with CDU performed on a non-HD day. AVF flow rate was expressed as ml/min/1.73 m2. RESULTS: Sixteen measurements of AVF flow rate and recirculation with UD and TD were compared with CDU in 16 patients with a median weight of 39 kg. Both UD and TD overestimated AVF flow rate when compared with CDU (+437 (95% CI + 200, + 674) and + 476 (95% CI + 80, + 871) ml/min/1.73 m2 for UD and TD, respectively). CDU flow rate was significantly correlated to UD flow rate (r2 = 0.761, p < 0.001), but not to TD flow rate (r2 = 0.164, p = 0.120). Although recirculation in all AVF was estimated to be 0% and < 15% with UD and TD, respectively, 7 significant stenoses were diagnosed by CDU. AVF with stenosis had lower flow rates when measured by CDU, UD or TD, but only CDU measurements reached statistical significance (p = 0.008, p = 0.142 and p = 0.174, respectively). CONCLUSION: UD and TD overestimate AVF flow rate when compared with CDU, which is the most reliable non-invasive method for screening vascular access for stenosis. UD seems more accurate than TD for AVF flow rate assessment. Recirculation via UD or TD should not be used for early screening of AVF stenosis in children on HD.

Body composition and arterial stiffness in pediatric patients with chronic kidney disease.

BACKGROUND: This study investigated the impact of body composition in the arterial stiffness of children with chronic kidney disease (CKD). METHODS: Fat mass (FM), fat tissue index (FTI), fat-free mass (FFM), fat-free tissue index (FFTI), and FFTI/FTI were measured in 26 patients and 25 healthy controls by bio-impedance analysis. Data on patient's body mass index (BMI) for height-age, serum albumin, glomerular filtration rate (GFR), blood pressure status, and pulse wave velocity (PWV) were collected in patients. RESULTS: Patients presented lower levels of FM and FFM compared to healthy controls (p = 0.04 and p = 0.055 respectively). In patient group, BMI height-age z-score was positively correlated to FTI (r2 = 0.574, p < 0.001) and FFTI (r2 = 0.338, p = 0.001) and negatively correlated to FFTI/FTI (r2 = 0.263, p = 0.007). Serum albumin was positively correlated only with FFM among body composition data (r2 = 0.169, p = 0.037). PWV z-score was positively correlated to FFTI (r2 = 0.421, p = 0.006) and inversely correlated to FFTI/FTI ≥ 2.5 (r2 = 0.317, p = 0.003). Patients with FFTI/FTI ≥ 2.5 presented lower levels of PWV regardless the need for antihypertensive treatment. Serum albumin ≥ 3.8 mg/dl and FFTI/FTI ≥ 2.5 were independently associated with a lower risk for high PWV, after adjustment for age, sex, and GFR (OR 0.009, 95% CI 0.000-0.729 and OR 0.039, 95% CI 0.002-0.680). All underweight [2 (7.7%)] and overweight [4 (15.4%)] patients presented high PWV. Among normal weight patients, FFTI/FTI ratio ≥ 2.5 was significantly associated with lower PWV z-score (p = 0.013). CONCLUSIONS: Both underweight and overweight are associated with arterial stiffness. Targeting FFTI/FTI ≥ 2.5 could be protective against cardiovascular disease in normal weight children.

Interdialytic weight gain and vasculopathy in children on hemodialysis: a single center study.

BACKGROUND: Increased interdialytic weight gain (IDWG) has been associated with poor outcomes in adults, but its impact on hemodialysis vasculopathy in children is unknown. METHODS: Nineteen patients (age 9 to 19 years old) with a median hemodialysis duration of 10.4 months were enrolled. Cardiovascular evaluation included left ventricular mass index (LVMI), pulse wave velocity (PWV), and carotid intima-media thickness (cIMT) measurements. PWV and cIMT were expressed as z-scores based on reference values in healthy children. Blood pressure (BP) evaluation consisted in a 24-h ambulatory BP monitoring. Mean IDGW and residual urine output during the 6 months prior to cardiovascular examination were calculated. RESULTS: Increased cIMT, LVMI, and PWV was observed in 11 (57.9%), 7 (36.8%), and 5 (26.3%) patients respectively, while BP was normal in all patients. Median IDWG was 3.5% (1.8-6.7). Residual urine output and BP status did not significantly differ between patients with IDWG ≥ or < 4%. After linear regression, IDWG was correlated to cIMT z-score (r2 = 0.485, p = 0.001), but not to PWV z-score (r2 = 0.04, p = 0.415) and LVMI (r2 = 0.092, p = 0.206). After univariate logistic regression, IDWG ≥ 4% was significantly associated to increased cIMT (above 1.65 SDS) (odds ratio 12.25, 95% confidence interval 1.08-138.988). The trend toward an increased cIMT with IDWG ≥ 4% was observed in both patients with short and long dialysis vintage. CONCLUSIONS: High IDWG is associated with increased cIMT in hemodialyzed children independently of BP control and dialysis vintage. This observation reinforces the importance of interventions to avoid IDWG in hemodialyzed children.

Autologous arteriovenous fistulas for hemodialysis using microsurgery techniques in children weighing less than 20 kg.

BACKGROUND: This study aimed to describe the efficiency and longevity of arteriovenous fistula (AVF) for hemodialysis (HD) in children weighing ≤20 kg. METHODS: We collected data of all AVFs created using microsurgery techniques between 1988 and 2015. Success was considered as the ability to use the AVF for HD. Primary and secondary patency rates were measured. RESULTS: Forty-eight AVFs (35 forearm, 13 upper arm) were created in 41 children with a median weight of 13.5 kg (range 5.5-20). The need for a second AVF was significantly higher in younger and thinner children at the time of AVF creation (p = 0.046 and p = 0.019, respectively). Successful use for HD occurred in 42 AVFs (87.5%), while six (12.5%) resulted in failure for early thrombosis or nonmaturation. Median time to first cannulation was 18.8 weeks (range 2-166.3). Primary and secondary patency rates at 1, 5, and 10-year follow-ups were 54.2%, 29.2%, and 13.7%; and 85.4%, 57.7%, and 33%, respectively. Almost one third of thromboses after first AVF cannulation were observed at kidney transplantation (KT) perioperatively. At the end of the follow-up (median duration 5.07 years, range 0-17.95), one patient was still on HD via AVF, two died of unrelated reason, and 38 were transplanted-one of whom returned to HD with a new AVF. CONCLUSIONS: AVF using microsurgery techniques is feasible in young children, showing an early failure rate of 12.5%. Time to first cannulation may be rather long, but secondary patency is excellent. Thrombosis rate is high during KT.

Early cardiovascular manifestations in children and adolescents with autosomal dominant polycystic kidney disease: a single center study.

BACKGROUND: This study aims to describe the cardiovascular manifestations in children with autosomal dominant polycystic kidney disease (ADPKD) and detect their relation with kidney disease and type of gene mutation. METHODS: Twenty-one patients (7 to 19 years old) were included. Cardiovascular evaluation involved blood pressure (BP), indexed left ventricular mass (LVMI), pulse wave velocity (PWV), and carotid intima media thickness (cIMT) measurement. Patients were classified according to percentile reference values of these parameters in healthy children. The 95th percentile was the highest level of normal values. Glomerular filtration rate (GFR) and microalbuminuria were also measured. RESULTS: Antihypertensive treatment, large LVMI, high PWV, and increased cIMT were observed in 6 (28.6%), 2 (9.5%), 4 (19%), and 8 (38.1%) patients respectively. Antihypertensive treatment was not associated with either high PWV or increased cIMT. Linear correlation was noticed between LVMI and PWV (r2 = 0.243, p = 0.023) and also between LVMI and cIMT (r2 = 0.203, p = 0.041). The median age of patients with high PWV, increased cIMT, and large LVMI was 9.5, 13, and 18 years old. GFR was normal in all patients. Patients with increased cIMT presented higher levels of urine microalbumin to creatinine ratio (p = 0.025). Genetic mutation was available in 18 patients. Antihypertensive treatment was more frequent in patients without PKD1 missense mutation (p = 0.044). CONCLUSIONS: High PWV and increased cIMT indicating arterial stiffness and hypertrophic vasculopathy may be present in children with ADPKD regardless BP status, and prior to GFR decline, suggesting that vascular disease precedes chronic kidney disease in ADPKD.