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1.
J Clin Med ; 12(20)2023 Oct 13.
Artigo em Inglês | MEDLINE | ID: mdl-37892649

RESUMO

BACKGROUND: In neonates, the assessment of kidney function with serum creatinine is limited; therefore, more effective biomarkers are needed. AIM: The study aimed at analyzing the concentrations of renal biomarkers (osteopontin, cystatin C, and NGAL) in neonates. MATERIAL AND METHODS: The study included 80 term and 20 preterm neonates aged 28-33 weeks of gestation. Biomarkers were measured in urine. Term neonates' urine was collected on the 1st day of life. Preterm neonates' urine was collected on the 1st, 8th, 15th, 22nd day of life. Biomarkers' concentrations were normalized to urinary creatinine (cr.) and presented as urinary biomarker/cr. ratios. RESULTS: Median values of biomarker/creatine ratios in term and preterm neonates were the following: cystatin C/cr.: 7.26 and 439.49; osteopontin/cr.: 135.86 and 1633.37; NGAL/cr. in girls: 212.14 and 256.93; and NGAL/cr. in boys 27.123 and 65.29 ng/mg cr. In preterm neonates the cystatin C/cr. ratio was higher on the 1st than on the 8th day. The osteopontin/cr. ratio did not differ between the days. The NGAL/cr. ratio in girls was higher on the 8th than on the 22nd day, and in boys, the lowest was on the 22nd day. CONCLUSIONS: Prematurity in stable, Caucasian neonates might cause higher osteopontin and cystatin C excretion, but not NGAL. The excretion of NGAL and cystatin C, but not osteopontin, may change during first weeks of premature neonate's life.

2.
Cells ; 13(1)2023 12 27.
Artigo em Inglês | MEDLINE | ID: mdl-38201263

RESUMO

The mechanisms of immune tolerance of a mother against an antigenically foreign fetus without a concomitant loss of defense capabilities against pathogens are the factors underlying the success of a pregnancy. A significant role in human defense is played by killer immunoglobulin-like receptor (KIR) receptors, which regulate the function of the natural killer (NK) cells capable of destroying antigenically foreign cells, virus-infected cells, or tumor-lesioned cells. A special subpopulation of NK cells called uterine NK cells (uNK) is found in the uterus. Disruption of the tolerance process or overactivity of immune-competent cells can lead to immune infertility, a situation in which a woman's immune system attacks her own reproductive cells, making it impossible to conceive or maintain a pregnancy. Since the prominent role of the inflammatory response in infertility, including KIR receptors and NK cells, has been postulated, the process of antigen presentation involving major histocompatibility complex (MHC) molecules (HLA) appears to be crucial for a successful pregnancy. Proper interactions between KIR receptors on female uNK cells and HLA class I molecules, with a predominant role for HLA-C, found on the surface of germ cells, are strategically important during embryo implantation. In addition, maintaining a functional balance between activating and inhibitory KIR receptors is essential for proper placenta formation and embryo implantation in the uterus. A disruption of this balance can lead to complications during pregnancy. The discovery of links between KIR and HLA-C has provided valuable information about the complexity of maternal-fetal immune interactions that determine the success of a pregnancy. The great diversity of maternal KIR and fetal HLA-C ligands is associated with the occurrence of KIR/HLA-C combinations that are more or less favorable for reproductive success.


Assuntos
Antígenos HLA-C , Tolerância Imunológica , Infertilidade , Feminino , Humanos , Gravidez , Apresentação de Antígeno , Células Germinativas/imunologia , Infertilidade/imunologia
3.
J Clin Med ; 10(24)2021 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-34945104

RESUMO

In this study, we report the experience of our center with the prognosis of vesicoureteral reflux, depending on the indications for voiding cystography, during a 12-year period. Retrospective analysis included 4302 children who were analyzed according to the indication for voiding cystography: (1) a febrile urinary tract infection, (2) urinary tract malformations on ultrasonography and (3) lower urinary tract dysfunction. Vesicoureteral reflux was found in 917 patients (21.32%; 24.1% of girls and 17.9% of boys). In group (1), reflux was found in 437/1849 cases (23.63%), group (2) in 324/1388 cases (23.34%) and group (3) in 156/1065 cases (14.65%). A significantly lower prevalence of reflux and its lower degree was found in children from group (3) when compared to other groups (p < 0.01). VURs were confirmed in over 20% of children with urinary tract malformations on ultrasonography or after a febrile urinary tract infection, suggesting the need for voiding cystography in these children. Indications for this examination in children with lower urinary tract dysfunction should be limited.

4.
J Clin Med ; 10(22)2021 Nov 13.
Artigo em Inglês | MEDLINE | ID: mdl-34830560

RESUMO

The objectives of this study were to evaluate urinary beta-2-microglobulin (ß2M) levels in long-term childhood cancer survivors and to establish its association with anticancer drug-induced nephrotoxicity. The study consisted of 165 childhood cancer survivors (CCS) who were in continuous complete remission. We reported that CCS had a significantly higher level of ß2M (p < 0.001) and ß2M/Cr. ratio (p < 0.05) than healthy peers. Among all participants, 24 (14.5%) had decreased eGFR (<90 mL/min/1.73 m2). A significant positive correlation between ß2M/Cr. ratio and body mass index (coef. 14.48, p = 0.046) was found. Furthermore, higher levels of urinary ß2M were detected among CCS with a longer follow-up time (over 5 years) after treatment. Subjects with decreased eGFR showed statistically higher urinary ß2M levels (20.06 ± 21.56 ng/mL vs. 8.55 ± 3.65 ng/mL, p = 0.007) compared with the healthy peers. Twelve survivors (7.2%) presented hyperfiltration and they had higher urinary ß2M levels than CCS with normal glomerular filtration (46.33 ± 93.11 vs. 8.55 ± 3.65 ng/mL, p = 0.029). This study did not reveal an association between potential treatment-related risk factors such as chemotherapy, surgery, radiotherapy, and the urinary ß2M level. The relationship between treatment with abdominal radiotherapy and reduced eGFR was confirmed (p < 0.05). We demonstrated that urinary beta-2-microglobulin may play a role in the subtle kidney injury in childhood cancer survivors; however, the treatment-related factors affecting the ß2M level remain unknown. Further prospective studies with a longer follow-up time are needed to confirm the utility of urinary ß2M and its role as a non-invasive biomarker of renal dysfunction.

5.
J Clin Med ; 10(19)2021 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-34640422

RESUMO

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children.

6.
J Clin Med ; 10(9)2021 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-34066698

RESUMO

The aim of this study was to evaluate the galectin-3 (Gal-3) level in children with a congenital solitary functioning kidney (cSFK) and determine its association with common renal function parameters. The study consisted of 68 children (49 males) with cSFK. We demonstrated that children with cSFK had a lower level of galectin-3 than that of healthy subjects (p < 0.001). No significant differences in serum cystatin C (Cys C) levels between the cSFK children and the reference group were found. The subjects with cSFK and reduced estimated glomerular filtration rate (eGFR) had significantly higher levels of Gal-3 and Cys C compared to those with normal eGFR (p < 0.05). Children with eGFR <60 mL/min/1.73 m2 showed significant statistical differences between the values of area under ROC curve (AUC) for Gal-3 (AUC 0.91) and Cys C (AUC 0.96) compared to that for creatinine level (AUC 0.76). Similar analyses carried out among cSFK children with eGFR <90 mL/min/1.73 m2 revealed an AUC value of 0.69 for Gal-3, 0.74 for Cys C, and 0.64 for creatinine; however, no significant superiority was shown for any of them. The receiver operating characteristic (ROC) analyses for identifying the SFK children among all participants based on the serum levels of Gal-3 and Cys C did not show any diagnostic profile (AUCs for Gal-3 and Cys C were 0.22 and 0.59, respectively). A positive correlation between the Gal-3 and Cys C concentrations was found (r = 0.39, p = 0.001). We demonstrated for the first time that Gal-3 might play an important role in the subtle kidney damage in children with cSFK. However, further prospective studies are required to confirm the potential applicability of Gal-3 as an early biomarker for kidney injury and possible progression to CKD.

7.
J Clin Med ; 10(3)2021 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-33535375

RESUMO

This study was performed to explore serum tumor necrosis factor-like weak inducer of apoptosis (TWEAK) and its dependent cytokines urinary excretion: monocyte chemoattractant protein-1 (MCP-1) and regulated on activation, normal T cell expressed and secreted chemokine (RANTES) with their relation to the kidney function parameters in children with solitary functioning kidney (SFK). The study included 80 children and adolescents (median age 9.75 year) with congenital and acquired (after surgical removal) SFK. Serum TWEAK and urinary MCP-1 and RANTES levels were significantly higher in SFK patients (p < 0.05). The serum TWEAK was positively related to serum creatinine (r = 0.356; p < 0.001). Moreover, in SFK the receiver operating characteristic analyses revealed good diagnostic profile for serum TWEAK with AUC (Area Under The Curve)-0.853, uRANTES-0.757, and for RANTES/cr.: AUC-0.816. Analysis carried out to identify children with impaired renal function (albuminuria and/or decreased estimated glomerular filtration rate < 90 mL/min/1.73 m2 and/or hypertension) showed good profile for TWEAK (AUC-0.79) and quite good profile for uRANTES and RANTES/cr. (AUC 0.66 and 0.631, respectively). This is the first study investigating serum TWEAK and urinary excretion of MCP-1 and RANTES together in children with SFK. Obtained results indicate that TWEAK and RANTES may serve as potential markers of renal impairment.

8.
J Clin Med ; 10(3)2021 Jan 21.
Artigo em Inglês | MEDLINE | ID: mdl-33494327

RESUMO

The deterioration of renal function after childhood solid tumors treatment is the result of using the intensive multimodal therapy. In recent years, urinary kidney injury molecule-1 (KIM-1) and urinary neutrophil gelatinase-associated lipocalin (NGAL) have been introduced as potential promising biomarkers of early kidney damage. The aim of the present study was to determine whether anticancer treatment has any effect on the concentration of KIM-1 and NGAL and its association with renal impairment in survivors of childhood solid tumors. Sixty patients previously treated for solid tumors were involved in this study. The median time after end of treatment was 8.35 years. Urine KIM-1 and NGAL levels were measured using immunoenzymatic ELISA commercial kits. Higher levels of urine NGAL, KIM-1/cr. (creatinine), and NGAL/cr. ratios were found in comparison with healthy controls (p < 0.0001). Among all subjects, 23% were found to have decreased estimated glomerular filtration rate (eGFR). A strong correlation between KIM-1/cr. and a cumulative dose of ifosfamide was observed (r = 0.865, p < 0.05). In addition, a moderate correlation between NGAL/cr. and a cumulative dose of cisplatin was identified (r = 0.534, p < 0.05). The AUC for KIM-1/cr. was 0.52, whereas NGAL/cr. showed a diagnostic profile describing the AUC of 0.67. Univariable regression showed significant associations between NGAL/cr. ratio and subjects after unilateral nephrectomy (coeff. 63.8, p = 0.007), cumulative dose of cisplatin (coeff. 0.111, p = 0.033), and age at diagnosis (coeff. 3.75, p = 0.023). The multivariable model demonstrated only cumulative dose of cisplatin as an independent factor influence on NGAL/cr. ratio. The results of our study showed increased levels of urine KIM-1 and NGAL many years after completion of the childhood solid tumors treatment, which correlated positively with a cumulative dose of ifosfamide and cisplatin. This study also suggests that unilateral nephrectomy could affect the concentration of the studied biomarkers.

9.
Cancer Chemother Pharmacol ; 86(6): 741-749, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33052454

RESUMO

INTRODUCTION: Nephrotoxicity is a potential adverse effect of anticancer treatment in childhood. Cytostatics, abdominal radiotherapy, total body irradiation (TBI) and some agents used in supportive care may induce acute kidney injury (AKI) or lead to chronic kidney disease (CKD). The aim of this study was to test the hypothesis whether urinary kidney injury molecule-1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) are increased in acute lymphoblastic leukemia (ALL) survivors. METHOD: The study cohort consisted of 86 patients (42 females) previously treated for ALL. The median time after cessation of treatment was 6.55 (IQR: 1.96-9.93) years and median age at the time of study: 12 (IQR: 6.76-16.00). The control group included 53 healthy peers. Immunoenzymatic ELISA commercial kits were used to measure urine KIM-1 and NGAL levels. RESULTS: The median levels of urine uNGAL (p < 0.05), uNGAL/creatinine (cr.) ratio (p < 0.0001) and uKIM-1/creatinine ratio (p < 0.0001) were significantly higher in ALL survivors in comparison with healthy controls. Female patients had significantly higher levels of NGAL and NGAL/cr. than males (mean 8.42 ± 7.1 vs. 4.59 ± 4.5 ng/mL and 86.57 ± 77 vs. 37.7 ± 37 ng/mg, respectively; p < 0.01). Of all the study participants, 11 (13%) presented eGFR below 90 mL/min/1.73 m2. The NGAL/cr. ratio seemed to be the best predictor of decreased eGFR (AUC = 0.65). The cumulative dose of methotrexate and cyclophosphamide did not predict the values of the urine NGAL, NGAL/cr., KIM-1/cr. and eGFR. Five years after the end of treatment, the patients had higher levels of uKIM-1 (1.02 ± 0.8 vs. 0.62 ± 0.6 ng/mL, p < 0.01), uNGAL (7.9 ± 6.7 vs. 4.6 ± 5 ng/mL, p < 0.01) and lower eGFR (114 ± 29 vs. 134 ± 35 mL/min/1.73 m2, p < 0.01) in comparison with ALL survivors with the observation period of less than 5 years. CONCLUSION: We demonstrated that ALL survivors have higher levels of urine NGAL, NGAL/cr. and uKIM-1/cr. ratio as compared to the control group. Further long-term follow-up studies are necessary to assess the significance of the NGAL and KIM-1 and their relationship to kidney damage after anticancer treatment in childhood.


Assuntos
Injúria Renal Aguda/diagnóstico , Antineoplásicos/administração & dosagem , Receptor Celular 1 do Vírus da Hepatite A/análise , Lipocalina-2/urina , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Insuficiência Renal Crônica/diagnóstico , Injúria Renal Aguda/induzido quimicamente , Injúria Renal Aguda/epidemiologia , Injúria Renal Aguda/urina , Adolescente , Biomarcadores/urina , Sobreviventes de Câncer/estatística & dados numéricos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Insuficiência Renal Crônica/induzido quimicamente , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/urina , Adulto Jovem
10.
Orphanet J Rare Dis ; 15(1): 36, 2020 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-32007091

RESUMO

BACKGROUND: Sensenbrenner syndrome, which is also known as cranioectodermal dysplasia (CED), is a rare, autosomal recessive ciliary chondrodysplasia characterized by a variety of clinical features including a distinctive craniofacial appearance as well as skeletal, ectodermal, liver and renal anomalies. Progressive renal disease can be life-threatening in this condition. CED is a genetically heterogeneous disorder. Currently, variants in any of six genes (IFT122, WDR35, IFT140, IFT43, IFT52 and WDR19) have been associated with this syndrome. All of these genes encode proteins essential for intraflagellar transport (IFT) a process that is required for cilium assembly, maintenance and function. Intra- and interfamilial clinical variability has been reported in CED, which is consistent with CED's genetic heterogeneity and is indicative of genetic background effects. RESULTS: Two male CED patients from two unrelated Polish families were included in this study. Clinical assessment revealed distinctive clinical features of Sensenbrenner syndrome, such as dolichocephaly, shortening of long bones and early onset renal failure. Ectodermal anomalies also included thin hair, short and thin nails, and small teeth in both patients. Next generation sequencing (NGS) techniques were performed in order to determine the underlying genetic cause of the disorder using whole exome sequencing (WES) for patient 1 and a custom NGS-based panel for patient 2. Subsequent qPCR and duplex PCR analysis were conducted for both patients. Genetic analyses identified compound heterozygous variants in the IFT140 gene in both affected individuals. Both patients harbored a tandem duplication variant p.Tyr1152_Thr1394dup on one allele. In addition, a novel missense variant, p.(Leu109Pro), and a previously described p.(Gly522Glu) variant were identified in the second allele in patients 1 and 2, respectively. Segregation analysis of the variants was consistent with the expected autosomal recessive disease inheritance pattern. Both patients had severe renal failure requiring kidney transplantation in early childhood. CONCLUSION: The finding of compound heterozygous IFT140 mutations in two unrelated CED patients provide further evidence that IFT140 gene mutations are associated with this syndrome. Our studies confirm that IFT140 changes in patients with CED are associated with early onset end-stage renal disease. Moreover, this report expands our knowledge of the clinical- and molecular genetics of Sensenbrenner syndrome and it highlights the importance of multidisciplinary approaches in the care of CED patients.


Assuntos
Craniossinostoses , Displasia Ectodérmica , Falência Renal Crônica , Osso e Ossos/anormalidades , Proteínas de Transporte/genética , Pré-Escolar , Displasia Ectodérmica/genética , Humanos , Falência Renal Crônica/genética , Masculino , Mutação/genética , Polônia
11.
Neonatology ; 115(2): 169-174, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30513528

RESUMO

BACKGROUND: Intrauterine growth restriction (IUGR) is a poorly understood complication of pregnancy. It may be associated with various diseases in adulthood, such as hypertension, cardiovascular disease, insulin resistance, and end-stage renal disease. OBJECTIVES: The aim of this study was to check whether IUGR affects the function of renal tubules, as assessed by the tubular damage markers neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury molecule 1 (KIM-1). METHODS: The study included 126 term neonates. Thirty-eight newborns were the result of pregnancies complicated by IUGR. Eighty-eight healthy newborns were the result of normal pregnancies with no prenatal or perinatal complications. The concentrations of urinary NGAL and KIM-1 were determined with a commercially available ELISA kit and were normalized for urinary creatinine (Cr) concentration. RESULTS: We found a significantly higher urinary concentration of NGAL and NGAL/Cr ratio in newborns from pregnancies complicated by IUGR when compared to the reference group. We found that female gender was associated with a higher concentration of urinary NGAL and also urinary NGAL/Cr. CONCLUSIONS: This is the first work that demonstrates that urinary NGAL concentration and urinary NGAL/Cr are significantly higher in infants that are small for gestational age than in appropriate-for-gestational-age infants. This might indicate subclinical kidney damage in newborns with IUGR.


Assuntos
Injúria Renal Aguda/diagnóstico , Injúria Renal Aguda/urina , Retardo do Crescimento Fetal/urina , Lipocalina-2/urina , Injúria Renal Aguda/etiologia , Biomarcadores/urina , Estudos de Casos e Controles , Creatinina/urina , Feminino , Receptor Celular 1 do Vírus da Hepatite A/análise , Humanos , Recém-Nascido , Falência Renal Crônica , Masculino
12.
Cent Eur J Immunol ; 43(2): 162-167, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30135628

RESUMO

INTRODUCTION: GDIgA1 (galactose deficient IgA1) plays a significant role in the pathogenesis of IgA nephropathy (IgAN) and Henoch-Schönlein nephritis (HSN). AIM OF THE STUDY: The aim of this study was to assess the relevance of serum GDIgA1 level as a prognostic marker in children with IgAN and HSN. MATERIAL AND METHODS: 41 children were included to the study group (15 IgAN, 26 HSN) and 22 to the control group. The following parameters were evaluated at baseline and endpoint: proteinuria, erythrocyturia, serum creatinine, serum IgA, GFR. A kidney biopsy was performed in all patients and evaluated according to the Oxford Classification (1 - present, 0 - absent: M - mesangial hypercellularity; E- endocapillary hypercellularity; S - segmental sclerosis/adhesion; T - tubular atrophy/interstitial fibrosis), and was calculated as the total score (sum of M, E, S, T). At the end of follow-up, the serum GDIgA1 concentration was measured. RESULTS: The serum GDIgA1 concentration in patients with IgAN and HSN was significantly higher than in the control group. No significant differences in mean proteinuria, erythrocyturia, GFR, MEST score, or GDIgA1 in serum, as well as the duration of follow-up between IgAN and HSN were observed. Baseline serum IgA concentration and time to kidney biopsy were significantly higher in children with IgAN than in children with HSN. We observed a positive correlation between GDIgA1 and IgA levels (r = 0.53), and GDIgA1 and serum creatinine levels (r = 0.5), as well as negative correlation between GDIgA1 and GFR (r = -0.37). CONCLUSIONS: Serum GDIgA1 level may have a prognostic value in children with IgAN and HSN; however, to fully elucidate its clinical potential further studies performed in larger patient cohorts are required.

13.
Pol Merkur Lekarski ; 44(262): 177-182, 2018 Apr 23.
Artigo em Polonês | MEDLINE | ID: mdl-29775444

RESUMO

IgA nephropathy is the most common glomerulonephritis in the world. For diagnosis kidney biopsy is necessary. AIM: The aim of the study was assessment the significance of IgA, C3 and IgG deposits intensity and location in kidney childhood IgA nephropathy (IgAN) for the symptoms of the disease and the follow up. MATERIALS AND METHODS: Study population consisted of 81 children, average 11,45±3,99 years. IgAN was recognized based on renal biopsy, performed 1,2±1,84, median 0,5 years after the onset. We used Oxford classification (OC) to assess the severity of histopatological lesions. In renal biopsy IgA and C3 deposits were found in immunofluorescence in mesangium or in vessels of glomeruli or both, and intensity was defined 0 to +4. We analyzed: proteinuria (mg/kg/day), hematuria, creatinine, GFR (according to Schwartz formula) two times, at the onset of the disease (OOD) and at the follow up (FU). Patients were treated with: ACEI/ARB or steroids alone or with imunossupresion drugs: azathioprine (AZA), cyclophosphamide (CYC), cyclosporine A (CsA), mycopnenolate mophetil (MMF). The follow up was 3,31±2,88 years. We divided the patients into two groups, depending on the intensity of IgA deposits: G1 n=29 (+1/+2), G2 n=52 (+3/+4); depending on the localizations of these deposits, we analyzed 3 groups: A n= 39 (mesangium), B n= 15 (glomeruli vessels), C n=27 (both) and depending on the kind of deposits we analyzed 4 groups: gr. a - n=30 (only IgA), gr. b - n=37 (IgA+C3), gr. c - n=5 (IgA+IgG) gr. d - n= 9 (IgA+IgG+C3). RESULTS: At OOD and FU we not found any differences in G1 vs G2 for: age, proteinuria, GFR and OC in renal biopsy; at FU GFR<90 ml/ min/1,73 m2 FU was observed more frequently in G2 vs G1 (p=0,02). The differences in groups A,B,C and groups a,b,c,d were not found. CONCLUSIONS: Poor prognosis in childhood IgAN may also depend on the intensity of the deposits, irrespective of their location.


Assuntos
Glomerulonefrite por IGA/patologia , Imunoglobulina A/análise , Rim/patologia , Adolescente , Biópsia , Criança , Pré-Escolar , Feminino , Seguimentos , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/metabolismo , Humanos , Imunoglobulina G/análise , Rim/química , Rim/metabolismo , Masculino , Proteinúria , Estudos Retrospectivos
15.
J Am Soc Nephrol ; 28(10): 3055-3065, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28566477

RESUMO

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.


Assuntos
Imunossupressores/uso terapêutico , Falência Renal Crônica/etiologia , Síndrome Nefrótica/congênito , Adolescente , Criança , Pré-Escolar , Seguimentos , Humanos , Lactente , Síndrome Nefrótica/complicações , Síndrome Nefrótica/tratamento farmacológico , Análise de Sobrevida
16.
Acta Paediatr ; 105(9): 1105-8, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27359090

RESUMO

UNLABELLED: Neutrophil gelatinase-associated lipocalin (NGAL) is one of the most extensively examined biological markers for early prediction of acute kidney injury, but there is a lack of data on normal NGAL values in healthy term-born infants. This encouraged us to established serum and urine levels using samples collected from 38 girls and 50 boys, born at a median age of 39 weeks, during the first 48 hours after birth. CONCLUSION: Our findings showed that urine NGAL, but not serum levels, were significantly higher in girls than in boys.


Assuntos
Recém-Nascido/urina , Lipocalina-2/urina , Caracteres Sexuais , Feminino , Humanos , Recém-Nascido/sangue , Lipocalina-2/sangue , Masculino , Estudos Prospectivos
17.
Biomarkers ; 21(2): 123-8, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26631256

RESUMO

AIM: We aimed to investigate whether urine intercellular adhesion molecule-1 (ICAM-1) might serve as a marker of renal disorder in children with ureteropelvic junction obstruction. MATERIAL AND METHODS: Twenty-nine children with severe hydronephrosis (HN) were compared with 23 participants with mild HN and with 19 healthy peers. RESULTS: Urine ICAM-1/uCre levels were significantly higher in HN children than healthy controls (P<0.01), and in severe HN when compared with mild HN (p<0.05). CONCLUSIONS: It seemed to us that uICAM-1 is a biomarker of renal disorder, and might have the potential to predict which patients will require surgery.


Assuntos
Biomarcadores/urina , Molécula 1 de Adesão Intercelular/urina , Nefropatias/urina , Obstrução Ureteral/urina , Análise de Variância , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Hidronefrose/diagnóstico , Hidronefrose/urina , Lactente , Nefropatias/diagnóstico , Masculino , Prognóstico , Curva ROC , Índice de Gravidade de Doença , Obstrução Ureteral/diagnóstico
18.
Urol Int ; 95(2): 146-52, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26067248

RESUMO

INTRODUCTION: Myelomeningocele (MMC) is a congenital central nervous system malformation caused by a failure of the neurulation process in early pregnancy. Patients with MMC present many abnormalities and the nervous, skeletal and urinary systems are the most affected. The aim of this study was to clinically evaluate patients with MMC, estimate renal and lower urinary tract (LUT) function and to ascertain whether urodynamic findings can predict the deterioration of urinary tract function. MATERIALS AND METHODS: Medical records of 112 patients were gathered from a database and evaluated retrospectively. The data included age, sex, BMI Z-score WHO, physical activity, urodynamic parameters and diagnosis and renal function. RESULTS: A total of 112 patients with MMC were enrolled in the study. There were no differences in age, sex, BMI Z-score WHO, physical activity, renal function and urodynamic findings (apart from cystometric capacity) between boys and girls. Detrusor overactivity was the most frequent urodynamic diagnosis in all groups of physical activity, level of lesion and in catheterized and non-catheterized children. The correlations between urodynamic findings and renal function tests were found. CONCLUSIONS: Patients with neurogenic bladder after MMC most often present detrusor overactivity. LUT function is disturbed in all MMC patients independent of lesion level and physical activity.


Assuntos
Sintomas do Trato Urinário Inferior/fisiopatologia , Meningomielocele/complicações , Bexiga Urinaria Neurogênica/complicações , Urodinâmica , Adolescente , Índice de Massa Corporal , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Lactente , Testes de Função Renal , Masculino , Qualidade de Vida , Estudos Retrospectivos , Bexiga Urinária/fisiopatologia , Bexiga Urinária Hiperativa/diagnóstico , Bexiga Urinária Hiperativa/fisiopatologia
19.
Acta Paediatr ; 104(11): e518-23, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26095925

RESUMO

AIM: Hydronephrosis caused by ureteropelvic junction obstruction (UPJO) is an important problem in children and young adults. The aim of this pilot study was to determine the urine profiles of a number of lysosomal exoglycosidases to see whether they indicated tubular renal damage in children with UPJO. METHODS: We measured lysosomal exoglycosidases urine activities in 32 patients with UPJO, dividing them into three groups. The surgical group comprised 16 children with severe hydronephrosis who required surgery, the nonsurgical group comprised 16 patients with mild hydronephrosis, and the reference group comprised 42 healthy children. The following indicators were measured: N-acetyl-ß-hexosaminidase and its A and B isoenzymes, α-fucosidase, ß-galactosidase, α-mannosidase and ß-glucuronidase. RESULTS: The urine activities of all exoglycosidases were significantly higher in children with UPJO than children in the reference group (p < 0.01). A strong positive correlation was also found between most of the urine exoglycosidases and the urine albumin/creatinine ratio (p < 0.01). CONCLUSION: Our findings demonstrated that children with UPJO showed increased renal activities of assessed exoglycosidases, which correlated positively with the urine albumin/creatinine ratio. A larger multicentre study is required to confirm the clinical applications of these observations.


Assuntos
Glicosídeo Hidrolases/urina , Nefropatias/etiologia , Nefropatias/urina , Pelve Renal , Túbulos Renais , Obstrução Ureteral/complicações , Obstrução Ureteral/urina , Biomarcadores/urina , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Testes de Função Renal , Masculino , Projetos Piloto , Obstrução Ureteral/terapia
20.
Pediatr Nephrol ; 30(7): 1113-20, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25549975

RESUMO

BACKGROUND: The aim of this study was to evaluate the usefulness of serum immunoglobulin A/complement factor 3 (IgA/C3) ratio for predicting histological severity of kidney lesions in children with IgA nephropathy (IgAN) based on World Health Organization (WHO) and the Oxford classification (OC). METHODS: We studied 89 children with IgAN with a mean age of 11.38 ± 4.1 years (range 2-18 years). Based on available medical records, we retrospectively evaluated clinical data, IgA/C3 ratio, and kidney biopsy findings using the five-grade WHO classification and the OC The mesangial hypercellularity (M), endocapillary hypercellularity (E), segmental sclerosis (S), tubular atrophy/interstitial fibrosis (T) (MEST) score (absent = 0, present = 1) calculated as the sum of M+E+S+T ranging from 0 to 4. RESULTS: Mean IgA/C3 ratio values were significantly higher (P < 0.05) in patients with M1, S1, and T1 compared with M0, S0, and T0, respectively (P < 0.05); there were no differences in the WHO classification. We found a significant positive correlation between the IgA/C3 ratio and proteinuria (r = 0.24) and determined optimal cutoff values of the IgA/C3 ratio, with a corresponding confidence interval for specific MEST scores. CONCLUSIONS: The IgA/C3 ratio in children with IgAN may be a useful marker of the severity of lesions found in kidney biopsy as evaluated using the OC.


Assuntos
Complemento C3/análise , Glomerulonefrite por IGA/patologia , Imunoglobulina A/sangue , Adolescente , Idade de Início , Atrofia , Biomarcadores/análise , Biópsia , Criança , Pré-Escolar , Feminino , Fibrose , Mesângio Glomerular/patologia , Glomerulonefrite por IGA/sangue , Glomerulonefrite por IGA/classificação , Glomerulosclerose Segmentar e Focal/patologia , Humanos , Lactente , Rim/patologia , Masculino , Valor Preditivo dos Testes , Proteinúria/metabolismo , Fatores de Risco , Urina/citologia
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