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1.
Scand J Urol Nephrol ; 46(1): 58-64, 2012 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22017447

RESUMO

OBJECTIVE: The purpose of this cross-sectional study was to identify whether plasma symmetric dimethylarginine (pSDMA) is a useful marker of renal function in children. MATERIAL AND METHODS: The study group consisted of 35 patients with chronic kidney disease (CKD) stages 1-5 (median age 11.5 years), classified on the basis of estimated glomerular filtration rate (eGFR) and divided into three groups: group A, patients with CKD stages 1 and 2; group B, CKD stage 3; and group C, CKD stages 4 and 5. A control group included 42 age-matched healthy children. Commercial enzyme-linked immunosorbent assay kits were used to measure pSDMA and serum cystatin C (sCysC) concentrations. RESULTS: The pSDMA and sCysC levels were significantly elevated in all CKD patients in comparison with healthy controls (p < 0.05). The pSDMA level in children was increased in the mild CKD (group A) (p < 0.01). There were also a significant difference in pSDMA concentration between groups A and B (p < 0.01). No differences in pSDMA levels were found between groups B and C. Receiver operating characteristics analyses showed that pSDMA was a better diagnostic tool than sCysC for identifying CKD stage among all the examined children and for detecting patients from group A (eGFR >60 ml/min/1.73 m(2)). CONCLUSIONS: Increased pSDMA and sCysC levels were found in CKD children. Further studies are required to confirm potential applications of pSDMA and CysC as useful biomarkers for the diagnosis and progression of CKD.


Assuntos
Arginina/análogos & derivados , Nefropatias/sangue , Nefropatias/fisiopatologia , Rim/fisiopatologia , Adolescente , Arginina/sangue , Biomarcadores/sangue , Estudos de Casos e Controles , Criança , Pré-Escolar , Doença Crônica , Estudos Transversais , Cistatina C/sangue , Progressão da Doença , Feminino , Taxa de Filtração Glomerular/fisiologia , Humanos , Lactente , Masculino , Curva ROC , Sensibilidade e Especificidade , Adulto Jovem
2.
Pediatr Nephrol ; 26(4): 579-86, 2011 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21279810

RESUMO

Congenital obstructive nephropathy is the primary cause of chronic renal failure in children. Rapid diagnosis and initiation of the treatment are vital to preserve function and/or to slow down renal injury. The aim of our study was to determine whether urinary (u) kidney injury molecule-1 (KIM-1) and neutrophil gelatinase-associated lipocalin (NGAL) may be useful non-invasive biomarkers in children with congenital hydronephrosis (HN) caused by ureteropelvic junction obstruction. The study cohort consisted of 20 children with severe HN who required surgery (median age 2.16 years) and two control groups (control group 1: 20 patients with mild, non-obstructive HN; control group 2: 25 healthy children). All of the children had normal renal function. Immunoenzymatic ELISA commercial kits were used to measure uKIM-1 and uNGAL concentrations. The preoperative median uKIM-1/creatinine (cr.) and uNGAL levels were significantly greater in the children with severe HN than in both control groups. Three months after surgery, uNGAL had decreased significantly (p<0.05) in the children with severe HN, but was still higher than that in control group 2 children (p<0.05). Receiver operator characteristic analyses revealed a good diagnostic profile for uKIM-1 and uNGAL in terms of identifying a differential renal function of <40% in HN patients (area under the curve (AUC) 0.8 and 0.814, respectively) and <45% in all examined children (AUC 0.779 and 0.868, respectively). Based on these results, we suggest that increasing uNGAL and uKIM-1 levels are associated with worsening obstruction. Further studies are required to confirm a potential application of uKIM-1 and uNGAL as useful biomarkers for the diagnosis and progression of chronic kidney disease.


Assuntos
Proteínas de Fase Aguda/urina , Hidronefrose/diagnóstico , Hidronefrose/urina , Lipocalinas/urina , Glicoproteínas de Membrana/urina , Proteínas Proto-Oncogênicas/urina , Adolescente , Área Sob a Curva , Biomarcadores/urina , Estudos de Casos e Controles , Criança , Pré-Escolar , Ensaio de Imunoadsorção Enzimática , Feminino , Receptor Celular 1 do Vírus da Hepatite A , Humanos , Hidronefrose/congênito , Lactente , Recém-Nascido , Lipocalina-2 , Masculino , Curva ROC , Receptores Virais , Sensibilidade e Especificidade
3.
Eur J Pediatr ; 170(3): 389-91, 2011 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-20848129

RESUMO

Denys-Drash syndrome (DDS) is characterized by progressive glomerulopathy caused by diffuse mesangial sclerosis (DMS), genitourinary defects, and a higher risk of developing Wilms' tumor. It is commonly assumed that the DMS is unresponsive to any medications. In this report, we present a patient with Denys-Drash syndrome, in whom the cyclosporine A (CsA) was found to induce total remission. This observation and observations of other authors confirm that in genetic forms of nephrotic syndrome, the proteinuric effect of CsA may be due to a non-immunologic mechanism. We confirm the beneficial effect of CsA treatment in DDS; however, the potential nephrotoxicity of this drug will probably not allow long-term use.


Assuntos
Ciclosporina/uso terapêutico , Síndrome de Denys-Drash/tratamento farmacológico , Imunossupressores/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Síndrome de Denys-Drash/complicações , Síndrome de Denys-Drash/genética , Feminino , Genes do Tumor de Wilms , Humanos , Lactente , Mutação , Síndrome Nefrótica/etiologia , Indução de Remissão
4.
Scand J Urol Nephrol ; 45(1): 52-9, 2011 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-21034351

RESUMO

OBJECTIVE: The aim of the study was to examine the urinary levels and clinical significance of monocyte chemoattractant protein-1 (uMCP-1) in children according to histological diagnosis and degree of proteinuria. MATERIAL AND METHODS: Group I comprised 20 children with idiopathic nephrotic syndrome (INS), examined twice (A, during INS relapse; and B, after proteinuria subsided). Group II comprised 17 children with persistent proteinuria due to focal segmental glomerulosclerosis (FSGS). Group III included 12 children with immunoglobulin A nephropathy (IgAN). The control group (C) contained 22 healthy children. uMCP-1 was determined by enzyme-linked immunosorbent assay and expressed in pg/ml. RESULTS: The median uMCP-1/creatinine ratio (uMCP-1/cr) in children with minimal change disease in relapse (IA) was significantly higher than in controls (p < 0.05), but when controlling for cyclosporine A (CsA) treatment the median uMCP-1 in children with INS, who were not treated with CsA, was 12.01 pg/mg cr (range 1.82-261.56 pg/mg cr) and did not differ from healthy controls. In examination IB the uMCP-1/cr concentration decreased and did not differ from healthy controls (p > 0.05). Children from groups II and III also had higher uMCP-1/cr levels than groups I and C (p < 0.01). uMCP-1/cr positively correlated with serum total cholesterol, low-density lipoprotein and protein/creatinine ratio in relapse (IA), and with serum cholesterol level in group B. A positive correlation between uMCP-1/cr and protein/creatinine ratio was also confirmed in groups II and III. CONCLUSION: Increased uMCP-1 was found in children with IgAN and FSGS correlated with proteinuria. A slight increase in uMCP-1 in children with INS was probably associated with CsA treatment.


Assuntos
Quimiocina CCL2/urina , Glomerulonefrite por IGA/urina , Glomerulosclerose Segmentar e Focal/urina , Síndrome Nefrótica/urina , Proteinúria/urina , Índice de Gravidade de Doença , Adolescente , Biomarcadores/urina , Estudos de Casos e Controles , Criança , Pré-Escolar , Colesterol/sangue , Creatinina/urina , Ciclosporina/uso terapêutico , Feminino , Glomerulonefrite por IGA/complicações , Glomerulosclerose Segmentar e Focal/complicações , Humanos , Imunossupressores/uso terapêutico , Lactente , Glomérulos Renais/fisiopatologia , Masculino , Síndrome Nefrótica/complicações , Síndrome Nefrótica/tratamento farmacológico , Proteinúria/etiologia
5.
Pol Merkur Lekarski ; 31(184): 204-8, 2011 Oct.
Artigo em Polonês | MEDLINE | ID: mdl-22097175

RESUMO

UNLABELLED: Hyperhomocysteinemia is independent risk factor of cardiovascular diseases. Similarly to nephrotic syndrome (NS) predisposes to vein thrombosis. THE AIM OF THE STUDY: To evaluate serum and urinary total homocysteine (stHcy and utHcy) levels in children with the symptoms of SN, and to determine a correlation between its concentration and some parameters of hemostasis, as well as doses and the time of prednisone therapy and serum cortisol level. MATERIAL AND METHODS: The examined group consisted of 18 children with NS, aged 7.64 +/- 5.1 years, divided on two groups: A--in time o proteinuria; B--during treatment with prednisone after regression of proteinuria. Control group (C) consisted of 20 children, aged 8.5 +/- 3.6 years. Serum and urinary tHcy levels were assayed by enzyme-linked immunosorbent assay method using the Axis-Shield set. RESULTS: Serum total Hcy concentration in groups A and B did not differ from the control group (p > 0.05). Urinary total Hcy concentration in groups A and B was significantly higher than that of control (p < 0.05). A positive correlation was observed between stHcy and serum albumin as well as cortisol levels, and between utHcy and serum AT III level. CONCLUSIONS: In children with steroid-dependent NS, subclinical disturbances in hemostasis were independent of serum tHcy concentration. There was no correlation between serum tHcy and cumulated doses, as well as time of prednisone treatment, however positive correlation was found with serum cortisone. Urinary excretion of Hcy significantly increases, in comparison to control, and correlates with serum AT III level.


Assuntos
Homocisteína/sangue , Homocisteína/urina , Síndrome Nefrótica/sangue , Síndrome Nefrótica/tratamento farmacológico , Prednisona/uso terapêutico , Criança , Ensaio de Imunoadsorção Enzimática , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Síndrome Nefrótica/urina
6.
J Urol ; 184(5): 2103-8, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-20850811

RESUMO

PURPOSE: The Bonn Risk Index has been used to evaluate the risk of urinary calcium oxalate stone formation. According to the original method, risk should be determined based on 24-hour urine collection. We studied whether the Bonn Risk Index could be measured in spot urine samples and which part of the day is most suitable for this purpose. MATERIALS AND METHODS: We collected total and fractionated 24-hour urine (in a 6-hour nocturnal portion and 9 consecutive 2-hour diurnal samples) in 42 children and adolescents with calcium oxalate urolithiasis and 46 controls. Bonn Risk Index values determined from each of the urine fractions were compared to those obtained from related 24-hour urine collections. RESULTS: Both groups exhibited similar circadian patterns of Bonn Risk Index values. Median Bonn Risk Index for the nighttime portion of urine in the stone group was 1.4 times higher than that obtained from the total 24-hour urine. The morning hours between 08:00 and 10:00 showed the peak lithogenic risk, and this fraction had the highest sensitivity and selectivity regarding discrimination between stone formers and healthy subjects. The afternoon hours demonstrated lower and less fluctuating crystallization risk. Despite diurnal fluctuations in Bonn Risk Index, there was still a well-defined cutoff between the groups. CONCLUSIONS: Bonn Risk Index determined from urine samples collected between 08:00 and 10:00 appears optimal in separating stone formers from healthy subjects, and appears as useful as the value determined from 24-hour urine collection. Investigation of this diurnal sample simplifies diagnosis in pediatric stone disease without loss of clinical information.


Assuntos
Urolitíase/urina , Adolescente , Oxalato de Cálcio/análise , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Curva ROC , Medição de Risco
7.
Pediatr Nephrol ; 25(10): 2067-75, 2010 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-20602239

RESUMO

Receptor activator of NF-kB ligand (RANKL) and osteoprotegerin (OPG) play key roles in the pathogenesis of glucocorticoid-induced osteoporosis (GIO). The aim of our study was to determine whether the cumulative glucocorticoid dose (CGCS) in children with idiopathic nephrotic syndrome (INS) has any effect on the concentration of serum RANKL and OPG and the RANKL/OPG ratio. The study population consisted of 90 children with INS, aged 3-20 years, who were treated with GCS. These children were divided into two groups according to the CGCS: low (L)<1 g/kg body weight (BW) and high (H)>or=1 g/kg BW, respectively. The control group (C) consisted of 70 healthy children. RANKL concentration was observed to be significantly higher and OPG significantly lower in INS children than in the reference group: 0.21 (range 0.01-1.36) versus 0.15 (0-1.42) pmol/l (p<0.05), respectively, and 3.76 (1.01-7.25) versus 3.92 (2.39-10.23) pmol/l (p<0.05), respectively. The RANKL/OPG ratio was significantly higher in INS children (p<0.01). The concentration of RANKL, similar to the RANKL/OPG ratio, was significantly higher in Group H children than in Group L children: 0.46 (0.02-1.36 ) versus 0.19 (0.01-1.25) (p<0.01) and 0.14 (0.01-0.71) versus 0.05 (0.002-0.37) (p<0.01), respectively. The concentration of OPG was similar in both groups. There was a positive correlation between CGCS and the concentration of sRANKL as well as the RANKL/OPG ratio (in both cases r=0.33, p<0.05). Based on these results, we suggest that long-term exposure to GCS results in a dose-dependent increase in serum RANKL concentration and the RANKL/OPG ratio, but not in the level of serum OPG.


Assuntos
Densidade Óssea/efeitos dos fármacos , Glucocorticoides/efeitos adversos , Síndrome Nefrótica/sangue , Osteoprotegerina/sangue , Ligante RANK/sangue , Adolescente , Criança , Pré-Escolar , Humanos , Síndrome Nefrótica/tratamento farmacológico , Adulto Jovem
8.
Pediatr Nephrol ; 25(8): 1519-27, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20414684

RESUMO

The purpose of the study was to investigate serum uric acid (SUA), high-sensitivity C-reactive protein (hs-CRP) and mean platelet volume (MPV) in pre-hypertensive (PH) and hypertensive (HT) children and adolescents. The study group consisted of 80 patients aged 10-19 years subdivided into PH and HT groups according to mean daytime or night-time systolic or diastolic blood pressure (BP) levels (> 90th percentile, but < 95th percentile and > or = 95th percentile, respectively). The control group (C) contained 25 normotensive subjects. Serum hs-CRP level was determined by a nephelometric method (Behring); platelets (PLTs) were counted, and MPV was assessed by a Coulter Analyzer MAXM. SUA was measured with an Hitachi instrument. The median SUA and hs-CRP levels in PH and HT subjects were significantly higher than those of the controls (P < 0.01) and were higher in the HT group than in the PH group (P < 0.05). An increase in SUA above 5.5 mg/dl was associated with an increase in hs-CRP [odds ratio (OR) 4.8; confidence interval (CI) 1.3-17.4; P < 0.01]. MPV values in the PH group did not differ from those of the controls (P > 0.05), but it was significantly higher in HT patients (P < 0.01). Serum hs-CRP and MPV concentrations were positively correlated with all BP measurements except night-time diastolic blood pressure (DBP). We demonstrated that, in HT children and adolescents, increased SUA with a parallel increase in hs-CRP and PLTs with MPV is observed. Although large, multicentre, prospective studies are needed to confirm this observation, hyperuricaemia seems to be associated with an increase in hs-CRP in PH and HT patients.


Assuntos
Proteína C-Reativa/metabolismo , Hipertensão/sangue , Adolescente , Plaquetas/metabolismo , Pressão Sanguínea , Criança , Eritrócitos , Feminino , Humanos , Masculino , Contagem de Plaquetas , Estudos Prospectivos , Testosterona , Ácido Úrico
9.
Pediatr Nephrol ; 25(5): 889-97, 2010 May.
Artigo em Inglês | MEDLINE | ID: mdl-20072790

RESUMO

The aim of work was to investigate whether serum and urinary neutrophil gelatinase-associated lipocalin(sNGAL and uNGAL, respectively) are potential biomarkers of early cyclosporine A (CsA) nephrotoxicity in steroid-dependent nephrotic children (SDNS). The study group (I) consisted of 19 children with SDNS aged 9.46+/-5.52 years treated with CsA. The children were examined four times: at proteinuria relapse, prior to CsA treatment,then after 3, 6, and 12 months of CsA treatment. The control group (II) consisted of 18 healthy children aged 3-15 years. A commercial enzyme-linked immunosorbent assay method was used to measure NGAL concentration.The sNGAL level in SDNS children prior to the administration of CsA was similar to that in the healthy controls (p>0.05), but it increased significantly during the course of treatment (p<0.01). The uNGAL/creatinine (cr) ratio in SDNS patients was higher before the withdrawal of CsA therapy (p<0.05), and was also increased at the consecutive examinations (p<0.01). There was a positive correlation between both sNGAL and uNGAL levels and CsA serum level. However, based on the serum and urinary NGAL/cr receiver operating characteristic curve and area under the curve (AUC) analysis, it remains uncertain whether uNGAL is a good predictor of cyclosporine nephropathy. Both sNGAL and uNGAL concentrations increased during the course of CsA treatment. Further studies in larger groups of patients are therefore necessary to confirm our experimental data that increased NGAL levels may be a non-invasive marker for the early detection of tubulointerstitial damage in CsA nephrotoxicity.


Assuntos
Proteínas de Fase Aguda , Ciclosporina/efeitos adversos , Monitoramento de Medicamentos/métodos , Imunossupressores/efeitos adversos , Nefropatias/induzido quimicamente , Lipocalinas , Síndrome Nefrótica/tratamento farmacológico , Proteínas Proto-Oncogênicas , Esteroides/uso terapêutico , Proteínas de Fase Aguda/urina , Adolescente , Biomarcadores/sangue , Biomarcadores/urina , Estudos de Casos e Controles , Criança , Pré-Escolar , Creatinina/sangue , Ciclosporina/sangue , Quimioterapia Combinada , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Imunossupressores/sangue , Nefropatias/sangue , Nefropatias/urina , Lipocalina-2 , Lipocalinas/sangue , Lipocalinas/urina , Masculino , Síndrome Nefrótica/sangue , Síndrome Nefrótica/complicações , Síndrome Nefrótica/urina , Valor Preditivo dos Testes , Proteinúria/tratamento farmacológico , Proteinúria/etiologia , Proteínas Proto-Oncogênicas/sangue , Proteínas Proto-Oncogênicas/urina , Curva ROC , Resultado do Tratamento
10.
Pol Merkur Lekarski ; 29(170): 88-92, 2010 Aug.
Artigo em Polonês | MEDLINE | ID: mdl-20842819

RESUMO

UNLABELLED: Vesicoureteral reflux (VUR) in children may lead to the renal fibrosis and scarring due to the overproduction and accumulation of extracellular matrix proteins (ECM) in interstitial tissue. Metalloproteinases produced in the kidneys are called biological markers of fibrosis. THE AIM OF THE STUDY was to assess if the presence of VUR in children disturb the balance between the serum and urinary concentrations of matrix metalloproteinases 2 and 9 and their tissue inhibitors 1 (TIMP-1) and 2 (TIMP-2) and predispose to excessive renal fibrosis. MATERIAL AND METHODS. The study was performed in 88 children, median aged 5.5 years (0.08-16 yrs) with VUR confirmed by voiding cystouretrography (VCUG). In 95% of estimated children the pyelonephritis indicated for VCUG performance. Control group consisted of 30 healthy children at similar age. Concentrations of MMP-2, MMP-9, TIMP-1 and TIMP-2 were estimated using immunoenzymatic ELISA method in urine of all examined children, additionally all the mentioned parameters in children with high (ll-V) grade of VUR were assessed in serum. RESULTS revealed that the urinary and serum concentrations of TIMP-1 and TIMP-2 were higher in healthy controls (p < 0.05). MMP-9 levels were higher only in the urine (p < 0.05) and MMP-2 in serum (p < 0.05). Increase in TIMP concentrations was connected with parallel increase in MMP levels in children with I-V grades of VUR, what was confirmed by the normal values of MMP-2/TIMP-2 and MMP-9/TIMP-1 ratios (p > 0.05). Only children with Ill-rd grade of VUR revealed reduced values of MMP/TIMP ratios (p < 0.05). Children's with Ill-V grade VUR revealed higher increase in serum concentrations of TIMP than in MMP, it was also seen in decrease in MMP/TIMP ratios (p < 0.05). No correlation was found between serum and urinary results of estimated parameters (p > 0.05). CONCLUSION: MMP-2 and MMP-9 and TIMP-1 and TIMP-2 play role in pathogenesis of VUR disturbances, what was confirmed by the change in their serum and urinary concentrations. In serum and urine of children with high (Ill-V) grade VUR the biggest disturbances were observed in MMPs: TIMPs system with the TIMP levels higher than MMP values, what indirectly indicated ECM degradation disturbances and increase in renal fibrosis.


Assuntos
Metaloproteinase 2 da Matriz/sangue , Metaloproteinase 9 da Matriz/sangue , Inibidor Tecidual de Metaloproteinase-1/sangue , Inibidor Tecidual de Metaloproteinase-2/sangue , Refluxo Vesicoureteral/sangue , Refluxo Vesicoureteral/urina , Adolescente , Biomarcadores/sangue , Biomarcadores/urina , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Metaloproteinase 2 da Matriz/urina , Metaloproteinase 9 da Matriz/urina , Valores de Referência , Inibidor Tecidual de Metaloproteinase-1/urina , Inibidor Tecidual de Metaloproteinase-2/urina
11.
Pediatr Nephrol ; 24(9): 1705-10, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19350280

RESUMO

Idiopathic hypercalciuria is the most important predisposing risk factor for calcium oxalate (CaOx) renal stone formation. We assessed the associations between spontaneous CaOx crystallization based on the Bonn Risk Index (BRI), urinary pH, calciuria, oxaluria, and citraturia in 140 Caucasian patients with hypercalciuria, aged 4-17 years, and compared the findings with those in 210 normocalciuric controls. Of the 140 hypercalciuric patients, 58 had renal stones, and 82 had recurrent erythrocyturia, renal colic, or urinary obstructive symptoms-but without stones. Urinary ionized calcium ([Ca(2+)]) levels were measured using a selective electrode, while the onset of crystallization was determined using a photometer and titration with 40 mmol/L ammonium oxalate (Ox(2-)). The calculation of the BRI was based on the [Ca(2+)]:Ox(2-) ratio. The BRI values were 12-fold higher in hypercalciuric children than in healthy controls, but no differences were found in the BRI between subjects with urinary stones and those with urolithiasis-like symptoms. An increased BRI suggested an association with hypercalciuria, lower urinary pH, hypocitraturia, and hypooxaluria. These data indicate that hypercalciuria is an important factor associated with increased urinary CaOx crystallization, although the causal pathways need further investigation. Determination of the BRI in children with hypercalciuria may improve the risk assessment of kidney stones.


Assuntos
Oxalato de Cálcio/urina , Hipercalciúria/urina , Cálculos Renais/urina , Adolescente , Criança , Pré-Escolar , Comorbidade , Cristalização , Feminino , Humanos , Concentração de Íons de Hidrogênio , Hipercalciúria/diagnóstico , Hipercalciúria/epidemiologia , Cálculos Renais/diagnóstico , Cálculos Renais/epidemiologia , Masculino , Polônia/epidemiologia , Fatores de Risco , Urinálise
12.
Pol Merkur Lekarski ; 26(156): 642-4, 2009 Jun.
Artigo em Polonês | MEDLINE | ID: mdl-19711733

RESUMO

Frasier syndrome is an uncommon genetic disorder featuring progressive glomerulopathy, male pseudohermaphroditism and gonadal dysgenesis. It is caused by mutations in intron 9 of the WT1 gene. Because of its rarity there is limited literature available on the diagnosis and treatment of this syndrome. The aim of the study was to present the clinicopathological findings and molecular analysis of phenotypically female adolescent presenting with severe proteinuria and primary amenorrhea. The significance of early recognition of Frasier syndrome and its differentiation from Denys-Drash syndrome was discussed. WT1 mutation analysis should be routinely done in females with steroid-resistant nephritic syndrome.


Assuntos
Síndrome de Frasier/diagnóstico , Síndrome de Frasier/genética , Genes do Tumor de Wilms , Mutação , Adolescente , Criança , Síndrome de Denys-Drash/diagnóstico , Síndrome de Denys-Drash/genética , Diagnóstico Diferencial , Feminino , Humanos , Fenótipo
13.
Pol Merkur Lekarski ; 26(154): 315-7, 2009 Apr.
Artigo em Polonês | MEDLINE | ID: mdl-19580196

RESUMO

UNLABELLED: Laminin (LN) and fibronectin (FN) are important extra cellular matrix (ECM) proteins. Disturbance between production and degradation of ECM proteins contributes to renal scarring. The aim of the study was evaluation the levels of urinary LN and FN in children with proteinuria in nephrotic syndrome (NS). MATERIALS AND METHODS: Examinations were conducted on 71 children, 3-15 years old: (A)--44 children with NS (proteinuria above 50 mg/kg b.v./24 hours); (B)--27 children without proteinuria (remission NS). Control group (K)--30 healthy children. Concentration of LN and FN were determined by EIA. RESULTS: In urine of children with NS (A) urinary concentration of LN significantly increased, in comparison to control (K) (p<0.05), but FN was normal (p>0.05). In children with remission of NS (B) urinary concentration of LN was unchanged (p>0.05), but concentration of FN significantly decreased (p<0.05). In renal biopsies majority children of A group presented minimal changes, but majority children of B group presented hyalinization of renal tubules. CONCLUSION: Nephrotic proteinuria disturbs production of LN and increases its urinary excretion, but did not influence on urinary excretion of FN.


Assuntos
Fibronectinas/urina , Laminina/urina , Síndrome Nefrótica/urina , Proteinúria/urina , Adolescente , Biópsia , Criança , Pré-Escolar , Feminino , Humanos , Rim/patologia , Masculino , Síndrome Nefrótica/complicações , Síndrome Nefrótica/patologia , Proteinúria/etiologia
14.
Pol Merkur Lekarski ; 27(157): 10-3, 2009 Jul.
Artigo em Polonês | MEDLINE | ID: mdl-19650421

RESUMO

UNLABELLED: In small children, pyelonephritis (PN) is an important cause of scarring in the renal and disturbed in the production and degradation of extracellulare matrix proteins (ECM). Aim of the study was to assess the urinary levels metalloproteinases 2 and 9 (MMP-2 and MMP-9) and their inhibitors 1 and 2 (TIMP-1 and TIMP-2) in children with pyelonephritis (PN). MATERIALS AND METHODS: Study group (I) consisted of 42 children with PN, aged 1-15 years, examined twice: A--prior to treatment (1-3 days of fever), B--after antibacterial treatment (10-14 days). The control group (K) consisted of 30 healthy children. Enzyme-linked immunosorbent assay kits were used for measurements of total human MMP-2, MMP-9, TIMP-1 and TIMP-2 in first morning urine. RESULTS: In children with PN (I) prior to treatment (A), urinary concentration of all parameters were increased as compared to the control (K) (p<0.05). After treatment (B), only the levels of TIMP-1 was still elevated (p = 0.02). In PN before (A) and after (B) treatment MMP-9/TIMP-1 ratio. However MMP-2/TIMP-2 ratio was normal. CONCLUSION: In children with PN the balance MMP-9/TIMP-1 is disturbed, with the predominance of TIMP-1 production over MMP-9. It may lead to renal fibrosis.


Assuntos
Metaloproteinase 2 da Matriz/urina , Metaloproteinase 9 da Matriz/urina , Pielonefrite/urina , Inibidor Tecidual de Metaloproteinase-1/urina , Inibidor Tecidual de Metaloproteinase-2/urina , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
15.
Przegl Lek ; 66(9): 485-90, 2009.
Artigo em Polonês | MEDLINE | ID: mdl-21033407

RESUMO

Metalloproteinases (MMP) and their tissue inhibitors (TIMP) play a crucial role to keep the balance between the synthesis and degradation of extracellular matrix protein. Balance disturbances of those two systems lead to abnormal tissue remodeling. There is evidence that matrix metalloproteinases activity changes in many pathological conditions, including inflammatory, degenerative disorders as well as tumor progression. Recent investigations indicate that MMPs and TIMPs play a pivotal role in pathogenesis of most of kidney diseases. Studies describing dysregulated activity of MMPs and/or their tissue inhibitors in various experimental and clinical models of kidney disease, including chronic kidney disease, glomerulonephritis, pyelonephritis, diabetic and hypertensive nephropathy, polycystic kidney disease and renal cancer are reviewed.


Assuntos
Proteínas da Matriz Extracelular/metabolismo , Nefropatias/metabolismo , Metaloproteinases da Matriz/metabolismo , Humanos , Neoplasias Renais/metabolismo , Inibidor Tecidual de Metaloproteinase-1/metabolismo , Inibidor Tecidual de Metaloproteinase-2/metabolismo
16.
Pediatr Dermatol ; 25(3): 408-9, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18577065

RESUMO

IgA nephropathy is one of the most common forms of glomerulonephritis, but the coexistence of IgA nephropathy and psoriasis is very rare, especially in children. Herein we report the case of an 8-year-old girl with both psoriasis and IgA nephropathy who responded to treatment with cyclosporine A for both conditions.


Assuntos
Artrite Psoriásica/complicações , Glomerulonefrite por IGA/complicações , Psoríase/complicações , Criança , Feminino , Glomerulonefrite por IGA/diagnóstico , Humanos , Imunoglobulina A/análise
17.
Pol Merkur Lekarski ; 24(141): 206-11, 2008 Mar.
Artigo em Polonês | MEDLINE | ID: mdl-18634285

RESUMO

UNLABELLED: A small number of reports evaluating early effects of vesicoureteral reflux conservative therapy in children, based on antibacterial prophylaxis combined with correction of the of lower urinary tract function inspired us to perform the study. THE AIM OF THE STUDY was to assess the effects of vesicoureteral reflux (VUR) management in children according to grade, sex and way of treatment. MATERIAL AND METHODS: The study group consisted of 108 children, who were treated in 2000-2007 due to VUR. There were 162 refluxing ureters (grades I-V) diagnosed by voiding cystourethrography (VCU). In 82 children cystometry and (or) uroflowmetry were additionally performed, which revealed lower urinary tract disorders in 41 (60 refluxing ureters), mostly detrusor hyperactivity or detrusor-sphincter dyscoordination. All children had conservative treatment, A - in 67 (102 refluxing ureters) only antibacterial prophylaxis, B - in 41 of children (60 refluxing ureters) in combination with pharmacological treatment of urodynamic abnormalities. RESULTS: The check-up VCU was performed after 23+/-15 months on average. VUR was observed to subside in 44/162 (28%) of refluxing ureters, including 22/108 (22%) of those treated managed with method A and 22/60 (37%) with method B. Fifty three of refluxing ureters were qualified for further conservative therapy and 65/162 (40%) for surgery (especially endoscopic). Following 2-3 years medical and surgical treatment, 87/162 (54%) refluxing ureters resolved. CONCLUSION: In the diagnostics of VUR in children we should take into consideration the assessment of lower urinary tract function, as treatment of these abnormalities increases the effects of VUR conservative management. However it should be confirmed on a larger group of patients.


Assuntos
Refluxo Vesicoureteral/fisiopatologia , Refluxo Vesicoureteral/terapia , Fatores Etários , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Resultado do Tratamento
18.
Pol Merkur Lekarski ; 24 Suppl 4: 56-60, 2008.
Artigo em Polonês | MEDLINE | ID: mdl-18924504

RESUMO

UNLABELLED: The reason for our search was various investigations about urinary tract dysfunctions in enuretic children. AIM: The aim of our study was estimation of lover urinary tract function in children with monosymptomatic primary nocturnal enuresis without positive reaction for a long non pharmacological therapy. MATERIAL AND METHODS: 54 children after 9-12 months behavioral therapy and short pharmacological treatment (desmopresin) was undergoing urodynamic investigation (uroflowmetry and cystometry). RESULTS: Urodynamic disorders was found in 44/54 of estimated children. In 34 of children it was overactive bladder, in 6 patients we found detrusor-sphincter discoordination. Five children had decreased bladder capacity. Next to non pharmacological treatment we used anticholinergic or Baclofen depending on the results of urodynamic tests. The response to the treatment (non bedwetting at all) we observed in 34 children (in 9 of them after 3 months of therapy, in 16 after 6 months of therapy and in 12 after 12 months of therapy). The rest of children had decreased number of wet night per month. CONCLUSION: The pharmacological treatment of urodynamic disorders helps to children with monosymptomatic primary nocturnal enuresis to lost this symptom.


Assuntos
Enurese Noturna/etiologia , Urodinâmica , Doenças Urológicas/complicações , Doenças Urológicas/diagnóstico , Antidiuréticos/uso terapêutico , Baclofeno/uso terapêutico , Criança , Antagonistas Colinérgicos/uso terapêutico , Desamino Arginina Vasopressina/uso terapêutico , Feminino , Humanos , Masculino , Enurese Noturna/terapia , Resultado do Tratamento , Doenças Urológicas/tratamento farmacológico , Doenças Urológicas/fisiopatologia
19.
Pol Merkur Lekarski ; 25(150): 451-4, 2008 Dec.
Artigo em Polonês | MEDLINE | ID: mdl-19205372

RESUMO

UNLABELLED: Pyelonephritis (PN) is frequent bacterial infections in young infants and very important because may cause parenchymal scarring. Early confirmation of bacterial infection and application the appropriate treatment before obtaining result of urine culture, reduce probability of parenchymal scarring. THE AIM OF THE STUDY: To evaluate the useful of inflammatory and renal injury markers: serum procalcitonin (PCT), tumor necrosis factor alpha (TNF-alpha) and injury renal marker alpha1--microglobulin (A1M) measurement, in comparison with C-reactive protein concentration and abnormal urinary tract, in neonates and young infants with pyelonephritis. MATERIAL AND METHODS: Investigation was performed in two groups: I group--23 children with PN (1 to 24 weeks of age), and K group--30 healthy children aged from 1 to 24 weeks. Serum concentration of CRP was measured by immunonephelometric assay, PCT by immunoluminometric assay, TNF alpha by ELISA method, and urinary A1M by nephelometric assay. RESULTS: In control group (K) medians of all investigated markers were below minimum of detection. PN patients (I) had the highest PCT TNF-alpha, A1M and CRP concentration before treatment and normal results after antibiotic treatment. Using a cut-off: of 0.5 mg/dl for CRP, 0.5 ng/ml for PCT 15 pg/ml for TNF-alpha and 10 mg/g cr for A1M, sensitivity and specificity in children with pyelonephritis were: for CRP 100% and 62.5%, for PCT 81.8% and 87.2%, for TNF alpha 77.1% and 93.1% and A1M 70.4% and 56.1%, respectively. A positive correlation between serum PCT and CRP and TNF alpha was found. Very high concentration all markers were in patients with vesicoureteral reflux and 1 patient with hydronephrosis. CONCLUSION: In early diagnostics of PN (before obtaining results of urine culture) in youngest children, determination of concentration PCT and TNF alpha, has higher value than determination of CRP, taking into concentration high sensitivity and specificity for bacterial infection.


Assuntos
Pielonefrite/sangue , Pielonefrite/diagnóstico , Adolescente , Biomarcadores/sangue , Proteína C-Reativa/metabolismo , Calcitonina/sangue , Peptídeo Relacionado com Gene de Calcitonina , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Precursores de Proteínas/sangue , Pielonefrite/urina , Fator de Necrose Tumoral alfa/sangue , alfa-Macroglobulinas/urina
20.
Pol Merkur Lekarski ; 20(116): 159-63, 2006 Feb.
Artigo em Polonês | MEDLINE | ID: mdl-16708630

RESUMO

UNLABELLED: The aim of work was the assessment of plasma anion oxalate (Ox) concentration in children during antibacterial treatment depending on way and time of antibiotic administration. MATERIAL AND METHODS: The examinations were carried out in 80 children, without nephrolithiasis, aged 10.1 +/- 4.3 years with bronchopneumonia, treated with beta-lactame antibiotics. The children were divided in two groups: I--children treated with oral amoxicillin + clavulanic acid or cefuroxime axetil (n=40), II--children treated with the same antibiotics intravenously (n=40). The Ox concentration in plasma and urine was measured using an enzymatic method with oxalate oxidase, four times. (0)--before treatment, (a)--in third day and (b)--in last day of administration (10 to 14 day), (c)--3 weeks after finishing treatment with antibiotics. RESULTS: The result showed that in children before treatment (0) mean plasma Ox concentration was 2.439 +/- 0.645 micromol/l. In 3rd day (a) the Ox concentration increased to 7.848 +/- 0.999 micromol/l (p < 0.01), in last day of treatment (b) decreased to 5.681 +/- 0.871 micromol/l, and after 3 weeks (c) came back to initial values (p > 0.05). Intravenous antibiotics administration did not influence plasma Ox concentration. CONCLUSIONS: Plasma oxalate concentration increases during oral administration of beta-lactame antibiotics caused by increased intestinal absorption, as a result of saprophytic microflora deterioration. However intravewous administration of the same antibiotics does not change the concentration of plasma oxalate.


Assuntos
Combinação Amoxicilina e Clavulanato de Potássio/farmacologia , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Ânions/sangue , Antibacterianos/farmacologia , Antibacterianos/uso terapêutico , Bronquite/tratamento farmacológico , Oxalatos/sangue , Pneumonia/tratamento farmacológico , beta-Lactamases/metabolismo , Adolescente , Cefuroxima/farmacologia , Cefuroxima/uso terapêutico , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino
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