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1.
Clin Kidney J ; 16(4): 745-755, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37007701

RESUMO

Background: Data on comorbidities in children on kidney replacement therapy (KRT) are scarce. Considering their high relevance for prognosis and treatment, this study aims to analyse the prevalence and implications of comorbidities in European children on KRT. Methods: We included data from patients <20 years of age when commencing KRT from 2007 to 2017 from 22 European countries within the European Society of Paediatric Nephrology/European Renal Association Registry. Differences between patients with and without comorbidities in access to kidney transplantation (KT) and patient and graft survival were estimated using Cox regression. Results: Comorbidities were present in 33% of the 4127 children commencing KRT and the prevalence has steadily increased by 5% annually since 2007. Comorbidities were most frequent in high-income countries (43% versus 24% in low-income countries and 33% in middle-income countries). Patients with comorbidities had a lower access to transplantation {adjusted hazard ratio [aHR] 0.67 [95% confidence interval (CI) 0.61-0.74]} and a higher risk of death [aHR 1.79 (95% CI 1.38-2.32)]. The increased mortality was only seen in dialysis patients [aHR 1.60 (95% CI 1.21-2.13)], and not after KT. For both outcomes, the impact of comorbidities was stronger in low-income countries. Graft survival was not affected by the presence of comorbidities [aHR for 5-year graft failure 1.18 (95% CI 0.84-1.65)]. Conclusions: Comorbidities have become more frequent in children on KRT and reduce their access to transplantation and survival, especially when remaining on dialysis. KT should be considered as an option in all paediatric KRT patients and efforts should be made to identify modifiable barriers to KT for children with comorbidities.

2.
Genes (Basel) ; 13(12)2022 11 24.
Artigo em Inglês | MEDLINE | ID: mdl-36553470

RESUMO

Alport syndrome (AS) is the most frequent monogenic inherited glomerulopathy and is also genetically and clinically heterogeneous. It is caused by semi-dominant pathogenic variants in the X-linked COL4A5 (NM_000495.5) gene or recessive variants in the COL4A3/COL4A4 (NM_000091.4/NM_000092.4) genes. The disease manifests in early childhood with persistent microhematuria and can progress to proteinuria and kidney failure in adolescence or early adulthood if left untreated. On biopsy, pathognomonic features include alternate thinning, thickening and lamellation of the glomerular basement membrane (GBM), in the presence of podocyte foot process effacement. Although previous studies indicate a prevalence of AS of about 1/50,000, a recent publication reported a predicted rate of pathogenic COL4A5 variants of 1/2320. We herewith present 98 patients (40 M/58 F) from 26 Greek families. We are selectively presenting the families segregating the X-linked form of AS with pathogenic variants in the COL4A5 gene. We found 21 different pathogenic variants, 12 novel: eight glycine and one proline substitutions in the collagenous domain, one cysteine substitution in the NC1 domain, two premature termination of translation codons, three splicing variants, one 5-bp insertion/frameshift variant, one indel-frameshift variant and four gross deletions. Notably, patients in six families we describe here and three families we reported previously, carried the COL4A5-p.G624D substitution, a founder defect encountered all over Europe which is hypomorphic with mostly milder symptomatology. Importantly, on several occasions, the correct genetic diagnosis reclassified patients as patients with AS, leading to termination of previous immunosuppressive/cyclosporine A therapy and a switch to angiotensin converting enzyme inhibitors (ACEi). With the understanding that all 98 patients span a wide range of ages from infancy to late adulthood, 15 patients (11 M/4 F) reached kidney failure and 11 (10 M/1 F) received a transplant. The prospects of avoiding lengthy diagnostic investigations and erroneous medications, and the advantage of delaying kidney failure with very early administration of renin-angiotensin-aldosterone system (RAAS) blockade, highlights the importance of timely documentation of AS by genetic diagnosis.


Assuntos
Nefrite Hereditária , Insuficiência Renal , Adolescente , Humanos , Pré-Escolar , Adulto , Nefrite Hereditária/genética , Grécia , Colágeno Tipo IV/genética , Hematúria
3.
Perit Dial Int ; 41(2): 217-225, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32783508

RESUMO

BACKGROUND: This 6-month prospective longitudinal study investigates the association between hydration status changes using bioimpedance spectroscopy (BIS) and systolic blood pressure (SBP), pulse pressure (PP), and serum albumin (sAlb) changes in children on peritoneal dialysis (PD). METHODS: Thirteen patients (median age: 12.58 years) were enrolled. Normal hydration, moderate hydration, severe overhydration, and dehydration were defined as -7% ≤ relative overhydration (Re-OH) < +7%, +7% ≤ Re-OH < +15%, Re-OH ≥ +15%, and Re-OH < -7%, respectively. Automated office blood pressure z-score, sAlb, and weight z-score were recorded. RESULTS: Fifty-two Re-OH measurements were recorded: three in five, four in five, five in two, and seven in one patient, respectively. SBP was higher and sAlb lower in cases with severe overhydration (9 readings) (p < 0.001, p < 0.001), but distribution of these parameters did not differ between normal hydration/dehydration (28 readings) and moderate overhydration (15 readings) cases. In patients with hydration status change, SBP and PP were higher while sAlb lower in cases with higher hydration status level (p = 0.026, p = 0.05, and p = 0.109, respectively). In all patients, visit-to-visit SBP, PP, and sAlb changes were correlated to Re-OH changes (rs = 0.693, p < 0.001; rs = 0.643, p < 0.001; rs = -0.444, p = 0.008, respectively) but not to weight changes (rs = 0.052, p = 0.754; rs = 0.034, p = 0.838; rs = -0.156, p = 0.378, respectively). Visit-to-visit Re-OH changes, which were >+4% or <-4%, were linearly correlated to SBP (r = 0.858, p < 0.001), PP (r = 0.757, p < 0.001), and sAlb (r = -0.699, p = 0.002) changes. CONCLUSION: In children on PD, longitudinal Re-OH changes are superior to weight changes in assessing volume-dependent variations of SBP, PP, and sAlb. Routine BIS application, rather than single BIS measurements, seems useful in the intra-patient monitoring of hydration status.


Assuntos
Diálise Peritoneal , Desequilíbrio Hidroeletrolítico , Criança , Impedância Elétrica , Humanos , Estudos Longitudinais , Estudos Prospectivos , Diálise Renal , Análise Espectral , Desequilíbrio Hidroeletrolítico/diagnóstico , Desequilíbrio Hidroeletrolítico/etiologia
5.
Pediatr Nephrol ; 36(2): 425-434, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-32767109

RESUMO

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.


Assuntos
Hiperuricemia , Resistência à Insulina , Insuficiência Renal Crônica , Ácido Úrico/metabolismo , Adolescente , Índice de Massa Corporal , Estudos Transversais , Humanos , Hiperuricemia/diagnóstico , Hiperuricemia/epidemiologia , Obesidade , Insuficiência Renal Crônica/diagnóstico
7.
J Proteome Res ; 19(6): 2294-2303, 2020 06 05.
Artigo em Inglês | MEDLINE | ID: mdl-32351114

RESUMO

Ureteropelvic junction obstruction (UPJO) constitutes the predominant cause of obstructive nephropathy in both neonates and infants. Fundamental questions regarding UPJO's mechanism, assessment, and treatment still remain unanswered. The aim of the present study was to elucidate potential differences through serum metabolic profiling of surgical cases of infants with UPJO compared to both nonsurgical cases and healthy age-matched controls. Early diagnosis of renal dysfunction in this cohort based on highlighted biomarkers was the ultimate goal. Thus, serum samples were collected from 20 patients preoperatively, 19 patients with mild stenosis treated conservatively, and 17 healthy controls. All samples were subjected to targeted metabolomics analysis by hydrophilic interaction liquid chromatography coupled to mass spectrometry (HILIC LC-MS/MS). Both univariate and multivariate statistical analyses were performed. Principal component analysis (PCA) and orthogonal partial least squares-discriminant analysis (OPLS-DA) score plots showed that the studied groups differed significantly, with a panel of metabolites, including creatinine, tryptophan, choline, and aspartate, distinguishing patients who required surgery from those followed by systematical monitoring as well as from healthy controls, showing high performance as indicators of UPJO disease.


Assuntos
Metabolômica , Espectrometria de Massas em Tandem , Biomarcadores , Cromatografia Líquida , Análise Discriminante , Humanos , Lactente , Recém-Nascido , Análise de Componente Principal
8.
Pediatr Nephrol ; 35(1): 163-170, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31606750

RESUMO

BACKGROUND: The ideal management of ureteropelvic junction obstruction (UPJO) remains debatable. This prospective case-control study aimed to investigate if urinary levels of Neutrophil Gelatinase-Associated Lipocalin (NGAL) and serum levels of cystatin C could distinguish surgical from non-surgical cases of UPJO and if they could detect earlier impairment of renal function. METHODS: Biomarkers were measured in the following age-matched groups: (a) 22 infants with surgical UPJO, at initial diagnosis and 12 months postoperatively (groups A1 and A2, respectively); (b) 19 infants with non-surgical UPJO (group B); and (c) 17 controls (group C). Based on serum cystatin C levels, estimated glomerular filtration rate (eGFR) was calculated. RESULTS: Urinary NGAL (uNGAL) was significantly higher in group A1 vs. group A2 (p = 0.02) and in group A1 vs. group C (p = 0.03), whereas there was no statistically significant difference between groups A2 and C (p = 0.77). Likewise, cystatin C levels were significantly higher in group A1 vs. group A2 and in group A1 vs. group C (p = 0.004 and p = 0.02, respectively), but no statistically significant difference between groups A2 and C (p = 0.82). uNGAL and serum cystatin C did not differ between groups B and A, nor did they differ between groups B and C. Cystatin C levels and eGFR of group A1 were significantly higher than those of group A2 and group C (p = 0.0001 and p = 0.02, respectively). CONCLUSION: It seems that NGAL and cystatin C are able to distinguish patients who were treated surgically from healthy controls, and their levels appear to improve significantly following surgery.


Assuntos
Cistatina C/sangue , Hidronefrose/diagnóstico , Lipocalina-2/urina , Obstrução Ureteral/diagnóstico , Biomarcadores/sangue , Biomarcadores/urina , Estudos de Casos e Controles , Pré-Escolar , Feminino , Taxa de Filtração Glomerular/fisiologia , Humanos , Hidronefrose/sangue , Hidronefrose/cirurgia , Hidronefrose/urina , Lactente , Pelve Renal/diagnóstico por imagem , Pelve Renal/patologia , Pelve Renal/fisiopatologia , Masculino , Período Pós-Operatório , Período Pré-Operatório , Estudos Prospectivos , Índice de Gravidade de Doença , Resultado do Tratamento , Ultrassonografia , Ureter/patologia , Obstrução Ureteral/sangue , Obstrução Ureteral/cirurgia , Obstrução Ureteral/urina , Procedimentos Cirúrgicos Urológicos
9.
Pediatr Nephrol ; 34(7): 1253-1260, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30927128

RESUMO

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.


Assuntos
Adiposidade , Artérias/fisiopatologia , Sobrepeso/fisiopatologia , Insuficiência Renal Crônica/fisiopatologia , Magreza/fisiopatologia , Rigidez Vascular , Adolescente , Pressão Sanguínea , Índice de Massa Corporal , Estudos de Casos e Controles , Criança , Estudos Transversais , Impedância Elétrica , Feminino , Taxa de Filtração Glomerular , Humanos , Falência Renal Crônica/complicações , Falência Renal Crônica/fisiopatologia , Masculino , Sobrepeso/complicações , Análise de Onda de Pulso , Insuficiência Renal Crônica/complicações , Albumina Sérica/metabolismo , Magreza/complicações
10.
Sleep Med ; 55: 33-39, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30743208

RESUMO

OBJECTIVE: The impact of sleep-related disorders in children and adolescents with chronic kidney disease on executive function performance has been scarcely studied. The aims of the present study were to assess the prevalence of sleep-related disorders in chronic kidney disease pediatric patients, and to examine possible correlations with measures of executive function. METHODS: We performed a case-control study including 51 children with chronic kidney disease stages 2-5, aged 5-18 years, and 51 healthy controls. The parents of both patients and controls completed the Pediatric Seep Questionnaire (PSQ), and the Behavior Rating Inventory of Executive Function (BRIEF) questionnaire. RESULTS: Children with chronic kidney disease presented lower executive performance with higher scores in scales of executive function, and an increased risk of presenting a sleep-related disorder (odds ratio (OR) = 7.58, 95% confidence interval (CI) 2.36-24.31, p < 0.005) compared to controls. In the patient group, age of chronic kidney disease onset correlated with Behavior Regulation Index T-score (r = 0.32, p < 0.05). In multivariate analysis, differences between patients and controls for Metacognition Index T-scores did not persist after adjustment for sleep-related breathing disorder and insomnia scores, while differences in Behavior Regulation Index T-scores remained significant (54.15 vs 49.14, p < 0.02). Moreover, mediational analyses showed that sleep-related disorders mediated the effect of chronic kidney disease on metacognition, but not on behavioral regulation. CONCLUSION: Sleep-related disorders may in part explain the lower executive function performance during everyday life in chronic kidney disease pediatric patients. However, additional disease-related factors may influence executive function, especially in the domains of behavior regulation.


Assuntos
Função Executiva/fisiologia , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Transtornos do Sono-Vigília/diagnóstico , Transtornos do Sono-Vigília/epidemiologia , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Insuficiência Renal Crônica/fisiopatologia , Transtornos do Sono-Vigília/fisiopatologia
11.
J Hum Hypertens ; 33(4): 277-285, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30664657

RESUMO

This school-based screening study assessed the prevalence of high blood pressure (BP) levels according to the European Society of Hypertension (ESH) 2016 guidelines. Moreover, risk factors for BP elevation, and the effect of geographic and seasonal factors on BP screening were investigated. BP and anthropometric measurements were obtained from 2832 children and adolescents, aged 6-18 years, during the period 2013-2016. Three BP measurements were performed using a mercury sphygmomanometer, and the mean of the last two was used for the analysis. Obesity was defined according to the International Obesity Task Force (IOTF) criteria. The prevalence of high-normal BP/hypertension and overweight/obesity was 3.7%/0.9%, and 22.9%/8.5%, respectively. The majority of the participants presenting high BP (≥90th percentile) were overweight or obese. Increased prevalence of high BP was observed during spring (5.5%) and winter (5%) compared with 2.5% in autumn (P<0.05). SBP z scores were higher in males, during spring and summer, and in urban areas. In conclusion, a low rate of high-normal and hypertensive BP levels was found despite the high prevalence of overweight and obesity. Overweight and obesity were associated with higher BP levels, but there was also a seasonal difference in the prevalence of high BP levels.


Assuntos
Pressão Sanguínea , Hipertensão/epidemiologia , Obesidade Infantil/epidemiologia , Serviços de Saúde Escolar , Estações do Ano , Adolescente , Fatores Etários , Determinação da Pressão Arterial , Criança , Estudos Transversais , Feminino , Grécia/epidemiologia , Humanos , Hipertensão/diagnóstico , Hipertensão/fisiopatologia , Masculino , Obesidade Infantil/diagnóstico , Obesidade Infantil/fisiopatologia , Prevalência , Fatores de Risco
13.
J Hypertens ; 37(1): 30-36, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-29939943

RESUMO

BACKGROUND: Vascular phenotype by assessing carotid-femoral pulse wave velocity (cf-PWV) and central SBP (cSP) in the young could be used as an intermediate cardiovascular outcome measure. Tonometry is considered the gold-standard technique, but its use is challenging in clinical practice, especially when used in children. The purpose of this study was to validate cf-PWV and cSP assessment with novel oscillometric device (SphygmoCor XCEL) in children and adolescents. METHODS: cf-PWV and cSP were measured in 72 children and adolescents aged 6-20 years. Measurements were performed by applanation tonometry and by the SphygmoCor XCEL device at the same visit under standardized conditions. Regression analysis and Bland-Altman plots were used for comparison of the tonometer-based with oscillometric-based method. RESULTS: Mean cf-PWV measured by applanation tonometry was 4.85 ±â€Š0.81 m/s and measured by SpygmoCor XCEL was 4.75 ±â€Š0.81 m/s. The mean difference between the two devices was 0.09 ±â€Š0.47 m/s (P = NS). cSP measured by SpygmoCor XCEL was strongly correlated with cSP measured by applanation tonometry (R = 0.87, P < 0.001). Mean cSP measured by applanation tonometry was 103.23 ±â€Š9.43 mmHg and measured by SpygmoCor XCEL was 103.54 ±â€Š8.87 mmHg. The mean cSP difference between the two devices was -0.30 ±â€Š3.34 mmHg (P = NS), and fulfilled the AAMI criterion 1. The estimated intersubject variability was 2.17 mmHg. CONCLUSION: The new oscillometric SphygmoCor XCEL device provides equivalent results for cf-PWV and cSP values to those obtained by tonometry in children and adolescents. Thus, the SphygmoCor XCEL device could be appropriate for assessing cf-PWV and cSP in the pediatric population.


Assuntos
Determinação da Pressão Arterial , Pressão Sanguínea/fisiologia , Adolescente , Adulto , Determinação da Pressão Arterial/métodos , Determinação da Pressão Arterial/normas , Determinação da Pressão Arterial/estatística & dados numéricos , Velocidade da Onda de Pulso Carótido-Femoral , Criança , Humanos , Manometria , Oscilometria , Adulto Jovem
14.
Rheumatol Int ; 38(7): 1241-1250, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29845429

RESUMO

To assess longitudinally the course and outcome of juvenile idiopathic arthritis (JIA) in patients diagnosed and followed-up exclusively in the biologic era; also, to define possible predictors of the disease progression and need for early implementation of biologicals. Prospective and retrospective, monocentric cohort study of 120 JIA patients, diagnosed between 2001 and 2010, and followed-up for ≥ 4 years (median 8.04). Disease activity, cumulative articular/extra-articular damage and quality of life were evaluated by the assessment tools Juvenile Arthritis Disease Activity Score (JADAS71), Juvenile Arthritis Damage Index (JADI) and Childhood Health Assessment Questionnaire (CHAQ), respectively. Moreover, potential predictors of the disease progression and their relation to biologic therapy were investigated. High JADAS71 score (> 9) at diagnosis was indicative of progression to polyarticular course and the need for early introduction of biologic treatment. Other independent predictors of progression to polyarthritis, were: involvement of upper limb, hip and ankle within 6 months following JIA diagnosis and percentage of cumulative time with active disease > 35% within the first year. At the end of the study, both the median JADAS71 score and the Disability Index were significantly lower than the initial (p < 0.001) and remission off medication was achieved in 25% of the patients. Articular and extra-articular (only ocular) cumulative damage was demonstrated only in 5 and 7.5% of patients, respectively. Physical functional ability was found normal/mildly restricted in 93.3% and moderately restricted in 6.7% of the patients. We believe that these findings, fit in with a picture of JIA course and outcome under current conditions of objective "disease status" evaluation and of tightly controlled follow-up. Predictors emerged from our study could contribute to the identification of patients who will need early implementation of biologic treatment.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/terapia , Produtos Biológicos/uso terapêutico , Adolescente , Criança , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Masculino , Estudos Prospectivos , Qualidade de Vida , Estudos Retrospectivos , Índice de Gravidade de Doença
16.
Pediatr Nephrol ; 31(12): 2309-2316, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27677977

RESUMO

BACKGROUND: Progressive chronic kidney disease (CKD), irrespective of the underlying etiology, affects the quality of life (QoL) of children due to the need for regular follow-up visits, a strict medication program and diet intake. METHODS: The Greek version of the KIDSCREEN-52 multidimensional questionnaire was used in children with CKD, renal transplantation (RT) and in a control group (CG) of healthy children. RESULTS: Fifty-five patients between 8 and 18 years, with CKD (n = 25), RT (n = 16) and with end-stage renal disease (ESRD) on peritoneal dialysis (PD) (n = 14) were included. Each group of studied children was compared with the CG (n = 55), the validation sample (VS) (n = 1200) and the parent proxy scores. Physical well-being of all studied children was significantly lower compared to CG (p = 0.004). In contrast, all studied children between 8 and 11 years showed better social acceptance compared to VS (p = 0.0001). When QoL of children with CKD was compared with parent proxy QoL, conflicting opinions were observed in several dimensions, such as self-perception (p = 0.023), autonomy (p = 0.012), school environment (p = 0.012) and financial resources (p = 0.03). CONCLUSIONS: QoL and mainly the dimension of physical well-being, may be affected dramatically in children with CKD unrelated to disease stage. In early school years children with CKD seem to feel higher social acceptance than the healthy controls, exhibiting better score in this dimension. Optimal care requires attention not only to medical management, but also to an assessment of QoL factors, that may help promote pediatric patient's health.


Assuntos
Qualidade de Vida , Insuficiência Renal Crônica/psicologia , Adolescente , Fatores Etários , Criança , Estudos Transversais , Feminino , Grécia/epidemiologia , Humanos , Falência Renal Crônica/psicologia , Transplante de Rim/psicologia , Masculino , Autonomia Pessoal , Insuficiência Renal Crônica/epidemiologia , Autoimagem , Autorrelato , Comportamento Social , Inquéritos e Questionários
17.
Ren Fail ; 38(7): 1031-5, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27185324

RESUMO

Peritoneal dialysis (PD) constitutes the preferred dialysis modality for children requiring renal replacement therapy with peritonitis being one of the most common complications of PD. This study was performed to evaluate the epidemiology, microbiology, and outcomes of PD-associated peritonitis in Greek children for a 10-year period. A total of 27 patients (16 males) with a mean age 121.8 ± 57.2 months were retrospective analyzed. Patients were on PD therapy for a mean duration of 45.2 ± 26.1 months. We found 23 episodes of PD-associated peritonitis occurred in 9 out of 27 patients (0.23 episodes/patient-year), with four patients experienced two or more peritonitis episodes. Gram-positive bacteria were responsible for 15 (65.2%) peritonitis episodes, with Staphylococcus aureus being the predominant specie isolated in 30.4% of cases. A total of seven episodes of exit-site infections (ESIs) were identified in five patients (0.069 episodes/patient-year) with the most common bacteria isolated being S. aureus (57.4%). Initial antibiotic treatment included intraperitoneal vancomycin plus ceftazidime in the majority of cases (82.6%). At the end of study, 12 (44.4%) patients remained on PD, 11 (41.8%) underwent renal transplantation, 2 (7.4%) shifted to hemodialysis and unfortunately, two patients (7.4%) died. Conclusively, our study revealed a noticeable low peritonitis and ESIs rate as compared to international data and represents the first evaluation of the characteristics and outcomes of peritonitis in the Greek pediatric PD population.


Assuntos
Falência Renal Crônica/terapia , Diálise Peritoneal/efeitos adversos , Peritonite/epidemiologia , Peritonite/microbiologia , Adolescente , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Ceftazidima/administração & dosagem , Ceftazidima/uso terapêutico , Criança , Pré-Escolar , Feminino , Grécia/epidemiologia , Humanos , Incidência , Lactente , Injeções Intraperitoneais , Falência Renal Crônica/tratamento farmacológico , Masculino , Peritonite/tratamento farmacológico , Estudos Retrospectivos , Infecções Estafilocócicas/tratamento farmacológico , Infecções Estafilocócicas/epidemiologia , Infecções Estafilocócicas/etiologia , Staphylococcus aureus/isolamento & purificação , Resultado do Tratamento , Vancomicina/administração & dosagem , Vancomicina/uso terapêutico , Adulto Jovem
18.
Pediatr Transplant ; 20(4): 500-6, 2016 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27029484

RESUMO

Pediatric kidney Tx has critically altered the outcome in ESRD pediatric patients. The aims of this study were to determine long-term graft and patient survival in a homogeneous ethnic population. We reviewed the medical charts of pediatric kidney Tx performed between 1990 and 2012 in Greece. Seventy-five kidney Txs were performed from LRD and 62 from DD. The 10- and 20-yr graft survival was higher in LRD Tx compared with DD Tx. Both patient and graft survival at 10 and 20 yr after Tx were similar in LRD Tx from grandparents compared with parents (92.9% vs. 93.4% 20-yr patient survival, 71.4% vs. 78.7% and 57.1% vs. 72.1%, 10- and 20-yr graft survival, respectively). However, there was a decreasing tendency in LRD Tx rates in period 2001-2012 compared with period 1990-2000 (47.1% vs. 62.7%). Risk factors for poor five-yr graft survival were DD Tx, and induction treatment with ALG compared with basiliximab, but their effect attenuated at 10 yr after Tx. In conclusion, Tx from LRD may offer efficient survival outcomes irrespective of donor age, suggesting that even older LRD could be an excellent option for the 1st kidney Tx in children and adolescents.


Assuntos
Sobrevivência de Enxerto , Falência Renal Crônica/mortalidade , Falência Renal Crônica/cirurgia , Transplante de Rim , Adolescente , Fatores Etários , Criança , Pré-Escolar , Feminino , Seguimentos , Grécia , Humanos , Doadores Vivos , Masculino , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida
19.
Urology ; 97: 194-196, 2016 11.
Artigo em Inglês | MEDLINE | ID: mdl-27079129

RESUMO

Lesch-Nyhan syndrome is an X-linked recessive inborn error of purine metabolism, due to deficiency of the enzyme HPRT (hypoxanthine-guanine phosphoribosyl transferase) and underlying HPRT gene mutations (over 300 mutations identified up to date). It is characterized by a wide range of neurological symptoms and signs (mainly a combination of spastic diplegia with choreoathetosis and an overall psychomotor redardation). Herein, we report of two cousins with Lesch-Nyhan syndrome and a confirmed novel HPRT gene mutation: c.65T>C, who both developed nephrocalcinosis and renal failure, findings not been previously published in children with HPRT deficiency.


Assuntos
Síndrome de Lesch-Nyhan/complicações , Síndrome de Lesch-Nyhan/diagnóstico , Nefrocalcinose/etiologia , Insuficiência Renal/etiologia , Pré-Escolar , Humanos , Hipoxantina Fosforribosiltransferase/genética , Síndrome de Lesch-Nyhan/genética , Masculino
20.
Am J Hypertens ; 29(7): 860-5, 2016 07.
Artigo em Inglês | MEDLINE | ID: mdl-26657420

RESUMO

BACKGROUND: Hypertension presents high prevalence rates following kidney transplantation (Tx). The aims of the present study were to investigate the prevalence and possible risk factors for hypertension and blood pressure (BP) control over time after pediatric kidney Tx, as well as to assess possible effects of hypertension on graft survival. METHODS: We reviewed the medical records of all pediatric kidney recipients followed up in our pediatric nephrology department. Hypertension was defined as systolic and/or diastolic BP greater than the 95th percentile for age and sex, or as being on antihypertensive medication. BP control was defined as normotension while on antihypertensive medication. RESULTS: The study population included 74 pediatric kidney recipients (median age 11 years). The prevalence of hypertension was found 77% before Tx, 82.4%, 71.7%, and 61% at 1, 5, and 10 years after Tx, respectively. Deceased donor Tx and pre-transplant hypertension on antihypertensive medication were significant risk factors for hypertension after kidney Tx over the follow-up period. BP control among patients on antihypertensive treatment was 16.7% before Tx, 43.8%, 66.7%, and 42.9% at 1, 5, and 10 years post-Tx, respectively. Hypertensive patients at 10 years post-Tx had 8.079 times higher hazard of graft loss compared to normotensives (95% CI 1.561-41.807, P < 0.05). CONCLUSIONS: Hypertension remains a frequent complication in pediatric kidney recipients even years after kidney Tx. BP control by antihypertensive treatment is unsatisfactory in about half of the patients. The adverse effects of hypertension on graft survival may appear in the long-term.


Assuntos
Pressão Sanguínea , Hipertensão/epidemiologia , Transplante de Rim/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Adolescente , Criança , Feminino , Sobrevivência de Enxerto , Grécia/epidemiologia , Humanos , Hipertensão/etiologia , Masculino , Complicações Pós-Operatórias/etiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco
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