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 SobrevidaRESUMO
BACKGROUND: Hypertension very often accompanies progression of chronic kidney disease (CKD) in children. A cross-sectional analysis of hypertension prevalence in dialyzed children in Poland was designed with a comparison with the data previously recorded 10 years earlier. METHODS: Two cohorts of children were analyzed: 59 subjects dialyzed in 2013, and 134 children from the previous study performed in 2003 that were reevaluated according to the current methodology. The incidence of hypertension (defined by SDS of sBP or dBP >1.64), clinical data, medical history, dialysis modalities and selected biochemical parameters of dialysis adequacy were analyzed. RESULTS: The prevalence of hypertension increased from 64% in 2003 to 78% in 2013. The efficacy of antihypertensive treatment remained unsatisfactory (61% proper BP control). Preservation of residual urine output and strict fluid balance may prevent development of hypertension in children on dialysis. CONCLUSIONS: Despite the higher awareness of hypertension and its complications in dialyzed children, the incidence of this entity has increased during the last decade, with the percentage of undertreated patients comparable to that observed 10 years ago. Thus, more attention should be paid to therapy efficacy in this population to prevent further damage to the cardiovascular system and to decrease morbidity.
Assuntos
Anti-Hipertensivos/uso terapêutico , Progressão da Doença , Hipertensão/tratamento farmacológico , Hipertensão/epidemiologia , Insuficiência Renal Crônica/complicações , Adolescente , Pressão Sanguínea , Criança , Pré-Escolar , Estudos Transversais , Feminino , Hidratação/métodos , Humanos , Incidência , Masculino , Pediatria , Polônia , Fatores de RiscoRESUMO
WT1 mutations cause a wide spectrum of renal and extrarenal manifestations. Here we evaluated disease prevalence, phenotype spectrum, and genotype-phenotype correlations of 61 patients with WT1-related steroid-resistant nephrotic syndrome relative to 700 WT1-negative patients, all with steroid-resistant nephrotic syndrome. WT1 patients more frequently presented with chronic kidney disease and hypertension at diagnosis and exhibited more rapid disease progression. Focal segmental glomerulosclerosis was equally prevalent in both cohorts, but diffuse mesangial sclerosis was largely specific for WT1 disease and was present in 34% of cases. Sex reversal and/or urogenital abnormalities (52%), Wilms tumor (38%), and gonadoblastoma (5%) were almost exclusive to WT1 disease. Missense substitutions affecting DNA-binding residues were associated with diffuse mesangial sclerosis (74%), early steroid-resistant nephrotic syndrome onset, and rapid progression to ESRD. Truncating mutations conferred the highest Wilms tumor risk (78%) but typically late-onset steroid-resistant nephrotic syndrome. Intronic (KTS) mutations were most likely to present as isolated steroid-resistant nephrotic syndrome (37%) with a median onset at an age of 4.5 years, focal segmental glomerulosclerosis on biopsy, and slow progression (median ESRD age 13.6 years). Thus, there is a wide range of expressivity, solid genotype-phenotype associations, and a high risk and significance of extrarenal complications in WT1-associated nephropathy. We suggest that all children with steroid-resistant nephrotic syndrome undergo WT1 gene screening.
Assuntos
Glomerulosclerose Segmentar e Focal/genética , Mutação , Síndrome Nefrótica/congênito , Insuficiência Renal Crônica/genética , Proteínas WT1/genética , Idade de Início , Criança , Pré-Escolar , Análise Mutacional de DNA , Progressão da Doença , Feminino , Estudos de Associação Genética , Predisposição Genética para Doença , Testes Genéticos/métodos , Glomerulosclerose Segmentar e Focal/diagnóstico , Glomerulosclerose Segmentar e Focal/epidemiologia , Glomerulosclerose Segmentar e Focal/terapia , Humanos , Incidência , Lactente , Masculino , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/epidemiologia , Síndrome Nefrótica/genética , Síndrome Nefrótica/terapia , Fenótipo , Prevalência , Prognóstico , Sistema de Registros , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/epidemiologia , Insuficiência Renal Crônica/terapia , Fatores de Risco , Fatores de TempoRESUMO
UNLABELLED: Slipped capital femoral epiphysis (SCFE) is the most common orthopedic hip disorder affecting otherwise healthy adolescents. The majority of SCFE cases are classified as idiopathic; rarely, it may be secondary to different endocrinopathies including hyperparathyroidism due to chronic renal failure (CRF). However, over the last decades, the association between SCFE and CRF has almost disappeared, probably due to better management of renal osteodystrophy. Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC, OMIM no. 248250) is a rare autosomal recessive tubulopathy characterized by renal wasting of calcium and magnesium leading to hypomagnesemia, hypercalciuria, nephrocalcinosis, and CRF. Patients usually show hyperparathyroidism before the onset of advanced CRF caused by FHHNC-related metabolic disturbances. We report on a 15-year-old patient with FHHNC and CRF who developed extreme hyperparathyroidism and high-grade bilateral SCFE after self-discontinuation of supportive treatment of underlying conditions. CONCLUSION: We believe that SCFE was caused not only by untreated CRF but also by metabolic disturbances related to FHHNC. To prevent this complication, careful management of disturbances of calcium, phosphate, and magnesium homeostasis seems to be crucial.
Assuntos
Hipercalciúria/complicações , Hiperparatireoidismo/complicações , Falência Renal Crônica/complicações , Deficiência de Magnésio/complicações , Nefrocalcinose/complicações , Escorregamento das Epífises Proximais do Fêmur/etiologia , Adolescente , Humanos , Hipercalciúria/diagnóstico , Hiperparatireoidismo/diagnóstico , Falência Renal Crônica/diagnóstico , Deficiência de Magnésio/diagnóstico , Masculino , Nefrocalcinose/diagnóstico , Escorregamento das Epífises Proximais do Fêmur/diagnósticoRESUMO
OBJECTIVE: Erythropoiesis-stimulating agents (ESAs) are applied as a standard therapy in children with anaemia in chronic kidney disease. The aim of this study was to describe the efficacy and details of ESA treatment in a population of dialysed children in Poland. MATERIAL AND METHODS: The study had a prospective observational design and was performed in 12 dialysis centres. The study group comprised 117 dialysed children with a mean age at enrolment of 165.33 (97.18-196.45) months. RESULTS: Dialysed children were treated mostly with epoietin beta and darbepoietin. The mean dose of ESA was 99 (68-147) U/kg/week with a significant difference between patients on peritoneal dialysis [83 (54-115)] and haemodialysis [134 (103-186)] (p < 0.0001). The mean haemoglobin of all the time-point tests during 6 months was 10.91 ± 1.18 g/dl. The efficacy of anaemia treatment was unsatisfactory in 52% of subjects. In multivariate analysis, initial haemoglobin level <10 g/l, any infection, younger age at first dialysis, malnutrition and inadequate ESA dosage remained significant predictors of anaemia. CONCLUSIONS: The study revealed that anaemia treatment in Polish children is unsatisfactory. Late commencement of the treatment, inadequate dosing, malnutrition and infections could constitute risk factors for therapy failure.
Assuntos
Anemia/tratamento farmacológico , Eritropoetina/análogos & derivados , Hematínicos/uso terapêutico , Falência Renal Crônica , Adolescente , Fatores Etários , Anemia/etiologia , Criança , Pré-Escolar , Estudos de Coortes , Darbepoetina alfa , Índices de Eritrócitos , Eritropoetina/uso terapêutico , Feminino , Humanos , Lactente , Falência Renal Crônica/complicações , Falência Renal Crônica/terapia , Masculino , Desnutrição/complicações , Análise Multivariada , Avaliação de Resultados em Cuidados de Saúde , Polônia , Proteínas Recombinantes/uso terapêutico , Diálise Renal , Resultado do Tratamento , Adulto JovemRESUMO
Unlike adults urolithiasis in children is a rare disease. For this reason medical treatment in this age group is still a serious therapeutic challenge. The aim of the study was to analyze the results of the applied treatment in the first incidences of urinary stones disease. A group of 143 patients aged 1.5 to 17 years was enrolled in this study. Spontaneous stone passage was obtained in case of 65 patients, which amounts to 63% of patients, who received conservative treatment. 17% were qualified to the ESWL treatment, 5.6% underwent the URS procedure, and in 2.8% of patients open surgery was performed. In 29% of cases stones were left for further observation. Conservative therapy was effective in the majority of children with small urinary stones. Preferred treatment in remaining patients were minimally invasive urological procedures.
Assuntos
Urolitíase/terapia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Litotripsia , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos , Urolitíase/cirurgiaRESUMO
UNLABELLED: Lower urinary tract dysfunction plays significant role in patogenesis of vesicoureteral reflux (VUR). Thus, urodynamic assessment is very useful in diagnosis and treatment of VUR. AIM: Assessment of the incidence of urodynamic disturbances among children with VUR. MATERIAL AND METHODS: Retrospective assessment of 125 children with VUR including 93 assessed urodymically. RESULTS: Urodynamic disturbances were found in 58 patients (70.9% of examined children), of which--detrussor hyperactivity in 25 (43.1% of children with urodynamic disturbances), detrussor hyperactivity with subvesical obstruction or dysfunctional voiding in 6 (10.3%), dysfunctional voiding in 17 (29.3%), anatomical subvesical obstruction in 9 (15.5%), detrussor hypotony in 1 (1.7%). No disturbances were found in 27 (29.1% of examined children). Children with detrussor overactivity were the prevalent group among all children with lower urinary tract dysfunction. CONCLUSIONS: Children with lower urinary tract dysfunction constitute a significant part of children with VUR.
Assuntos
Urodinâmica , Refluxo Vesicoureteral/diagnóstico , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Refluxo Vesicoureteral/fisiopatologiaRESUMO
In the article, 17-year-old girl with iatrogenic severe hipercalcemia was presented. The girl was treated since the age of 12 years for steroid-sensitive minimal change disease. Due to steroid therapy osteoporosis developed and intensive treatment with active form of vitamin D and high doses of calcium was started. She was admitted to our clinic in severe general state with abdominal pain, vomiting, dehydration, muscle weakness, hypertension and mental confusion. Severe hipercalcemia with nephrocalcinosis was diagnosed. The history revealed that the girl had increased the doses of drugs intentionally. The authors emphasized the need for careful monitoring of prophylaxis and treatment for osteoporosis due to steroid therapy.
Assuntos
Conservadores da Densidade Óssea/efeitos adversos , Hidroxicolecalciferóis/efeitos adversos , Hipercalcemia/induzido quimicamente , Nefrose Lipoide/tratamento farmacológico , Osteoporose/induzido quimicamente , Esteroides/efeitos adversos , Adolescente , Conservadores da Densidade Óssea/uso terapêutico , Cálcio/efeitos adversos , Cálcio/uso terapêutico , Feminino , Humanos , Hidroxicolecalciferóis/uso terapêutico , Doença Iatrogênica , Cálculos Renais/induzido quimicamente , Esteroides/administração & dosagem , Vitamina D/efeitos adversos , Vitamina D/uso terapêuticoRESUMO
UNLABELLED: Nephrocalcinosis (NC) is defined by increased calcium content of the kidney. To be identified radiologically it must be substantially gross accumulation of calcium within renal tissue. The incidence of NC in children is very low. The main causes of NC are hypercalcemic states with hypercalciuria and various tubular disorders. Although renal calcification may induce interstitial and tubular damage, NC is relatively rare associated with renal failure. We present three children with such an association. In those patients, primary distal tubular acidosis, hyperoxaluria type I and familial hypomagnesemia with hypercalciuria and nephrocalcinosis were diagnosed. The former two patients developed terminal renal failure in the school age whereas in the latter one, only moderate renal insufficiency was observed. CONCLUSIONS: The coexistence of nephrocalcinosis and renal failure is caused by extremely rare metabolic disorders. Therefore, this condition requires careful examinations.
Assuntos
Injúria Renal Aguda/etiologia , Cálculos Renais/complicações , Injúria Renal Aguda/diagnóstico , Adolescente , Adulto , Criança , Feminino , Humanos , Hipercalcemia/complicações , Hipercalciúria/complicações , Hiperoxalúria/complicações , Cálculos Renais/diagnóstico por imagem , Masculino , RadiografiaRESUMO
The purpose of the study was to analyze treatment approaches in children with malformations of urinary system. The retrospective study comprised 557 children (196 boys and 361 girls) aged 1-18 years with malformations of urinary system hospitalized in 2000-2007 in the Department of Pediatric Nephrology, University Children's Hospital, Lublin. Primary and secondary vesicoureteral reflux (269 children), ureteral obstruction (102 children), urethral obstruction (91 children) and renal duplication (83 children) were the most common malformations observed. In 26 children, multiple urinary system malformations were diagnosed. 332 children required surgery including 113 with vesicoureteral reflux. However, in the majority of children with vesicoureteral reflux, on an average 18-month pharmacologic treatment resulted in complete recovery.
Assuntos
Sistema Urinário/anormalidades , Sistema Urinário/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Rim/anormalidades , Masculino , Estudos Retrospectivos , Obstrução Ureteral/terapia , Obstrução Uretral/terapia , Neoplasias Urológicas , Procedimentos Cirúrgicos Urológicos , Refluxo Vesicoureteral/terapiaRESUMO
Children with nephrotic syndrome (NS) are at increased risk of Streptococcus pneumoniae infections. Streptococcus pneumoniae carrier state in the nasopharynx is an early stage of this infections. The aim of the study was to assess carriage rate and characterise bacterial isolates of Streptococcus pneumoniae among children with NS in a prospective multicentre study involving 10 nephrologic centres in Poland. We studied 95 children including 56 boys (59%) i 39 girls (41%) with NS in mean age 3 i 7/12 +/- 11 miesiecy. All children had throat and nasopharynx swabs taken in two epidemiological seasons (Oct-Dec 2006 and Feb-Apr 2007). Streptococcus pneumoniae isolates were serotyped with PCR. Carriage of Streptococcus pneumoniae was diagnosed in 13 children (13.7%) including 13 boys (100%). Following serotypes were found: 6B (38.5%), 9V (15.3%), 23F (15.3%), 19F (7.6%). There were 23.3% isolates without identifiable serotype. Carriage of Streptococcus pneumoniae was diagnosed mostly in boys with NS. Serotype 6B was dominant in this age group.
Assuntos
Nasofaringe/microbiologia , Síndrome Nefrótica/complicações , Infecções Pneumocócicas/microbiologia , Streptococcus pneumoniae/isolamento & purificação , Feminino , Humanos , Lactente , Masculino , Sorotipagem , Streptococcus pneumoniae/classificaçãoRESUMO
BACKGROUND: By now, two-dimensional contrast-enhanced voiding urosonography (ceVUS) has become a well-established method for the diagnosis and treatment monitoring of vesicoureteral reflux in children, particularly after the recent approval for this application in children in the USA and in Europe. The introduction of three-dimensional static (3D) and real-time (4D) techniques with ultrasound contrast agents opens up new diagnostic opportunities for this imaging modality. OBJECTIVE: To analyze whether 3D and 4D ceVUS is a superior technique compared to standard 2D ceVUS in diagnosing vesicoureteral reflux in children. MATERIAL AND METHODS: The study included 150 patients (mean age 3.7 years) who underwent 2D and 3D/4D ceVUS for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2D ceVUS and 3D/4D ceVUS diagnosed the same number of vesicoureteral refluxes, however, there was a statistically significant difference in grading between the two methods. Performing 3D/4D ceVUS resulted in changing the initial grade compared to 2D ceVUS in 19 out of 107 refluxing units (17.76%) diagnosed. The 4D technique enabled a more conspicuous visualization of vesicoureteral reflux than the 3D technique. CONCLUSIONS: 2D ceVUS and 3D/4D ceVUS diagnosed the same number of vesicoureteral refluxes, however, there was a statistically significant difference in grading between the two methods. Thus 3D/4D ceVUS appears at least a valid, if not even a more conspicuous technique compared to 2D ceVUS.
RESUMO
BACKGROUND: Blood pressure in pediatric dialyzed patients is under poor control. OBJECTIVES: The aim of the study was to assess the strategy and efficacy of antihypertensive drugs used for the treatment of hypertension in pediatric dialyzed patients in 2013 in comparison with the data collected in 2003/2004. The results have been viewed against present strategies of antihypertensive treatment in children. There is still limited data concerning the treatment of hypertension in dialyzed pediatric patients. MATERIAL AND METHODS: The study embraced 10 of 12 pediatric dialysis units in Poland treating 59 pediatric patients (mean age - 132 months). Collected information included present antihypertensive treatment with regard to drug classes and the dose of antihypertensive agent. The treatment was regarded as effective if both systolic and diastolic values of blood pressure were below 1.64 SDS. The results from 2013 were juxtaposed with previously analyzed data from a similar study on hypertension in dialyzed children conducted in 2003/2004. RESULTS: Forty subjects have been provided with antihypertensive treatment. In monotherapy and polytherapy 50% of the subjects were treated with ACEI (enalapril and ramipril), 67.5% with amlodipine, 50% with beta-blockers. Only 10% of the subjects were treated with angiotensin II receptor blocker (losartan). Thirty percent of the subjects received furosemide, whereas 5% were given doxazosin. Antihypertensive drugs regarded as the 2nd and 3rd choice in treating high blood pressure (doxazosin, beta-blockers and furosemide) were applied as monotherapy in 46% of the patients. Satisfactory control of treated blood pressure was reached in 45% of them. CONCLUSIONS: Antihypertensive treatment in dialyzed children did not change significantly during the last decade with regard to the groups of drugs being used. Despite a wider feasibility of antihypertensive substances, the effectiveness of this therapy was still unsatisfactory.
Assuntos
Anti-Hipertensivos/uso terapêutico , Hipertensão/tratamento farmacológico , Diálise Renal , Adolescente , Antagonistas de Receptores de Angiotensina/uso terapêutico , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Criança , Pré-Escolar , Feminino , Humanos , MasculinoRESUMO
Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is a rare autosomal recessive tubulopathy resulting from mutation in the gene encoding paracelin 1. The main symptoms of FHHNC include excessive urinary calcium and magnesium excretion, nephrocalcinosis, and chronic renal failure. We present 16-year old girl in whom symptoms of FHHNC were accidentally recognized during therapy of acute lymphoblastic leukemia. In our patient, some symptoms of FHHNC were initially taken for the adverse effects of cytostatic therapy that delayed an adequate diagnosis. To the best of our knowledge, this is the first report of FHHNC associated with acute lymphoblastic leukemia. However, in our opinion this association is accidental.
Assuntos
Hipofosfatemia Familiar/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Adolescente , Feminino , Humanos , Hipercalciúria/complicações , Hipofosfatemia Familiar/complicações , Nefrocalcinose/complicaçõesRESUMO
UNLABELLED: Citrate is thought to be one of the most important inhibitor of calcium salts crystallization in the urine. Therefore, an assessment of urinary citrate excretion is an integral element of metabolic evaluation in urolithiasis. The reported incidence of urolithiasis associated with hypocitraturia varies from 10% to 63%. The purpose of the study was to assess urinary citrate excretion in children with calcium urolithiasis living in region of Lublin. The study comprised 60 children (34 boys and 26 girls) aged 4.3-18 years. In 36 of them, calcium oxalate urolithiasis was diagnosed by spectrophotometry. In the remaining children, an assessment of stone composition was impossible. However, in all children, stones were radiopaque. Hypercalciuria (HC) and mild hyperoxaluria (HOx) were diagnosed in 23 and 16 children, respectively. In the remaining 21 children urolihiasis was classified as idiopathic. The controls were 35 healthy age- and gender-matched children. Urinary citrate excretions were assessed by enzymatic method in 24-hour urine specimens and expressed as citrate/creatinine ratios (Cit/Cr). Decreased Cit/Cr were observed in 9 (15%) children with calcium urolithiasis. However, there was no significant difference between the mean Cit/ Cr in children with calcium urolithiasis and controls (410 +/- 207 mg/g vs 385 +/- 144 mg/g). There was also no significant difference between the mean Cit/ Cr in children with HC and controls. Similarly, the mean Cit/Cr did not differ significantly between children with HOx and controls. However, the lowest mean Cit/Cr was revealed in children with HOx (306 +/- 161 mg/g). CONCLUSIONS: In most children with calcium urolithiasis urinary citrate excretion was normal. However, in some children with urolithiasis, detection of hypocitraturia allows to explain pathogenesis of stone formation and to carry on a causative prophylaxis.
Assuntos
Citratos/urina , Creatinina/urina , Hipercalciúria/urina , Cálculos Urinários/química , Urolitíase/diagnóstico , Urolitíase/urina , Adolescente , Biomarcadores/urina , Oxalato de Cálcio/urina , Criança , Pré-Escolar , Feminino , Humanos , Hiperoxalúria/urina , Masculino , Fatores de Risco , Urolitíase/metabolismoRESUMO
Reflux nephropathy is a progressive renal scarring due to chronic pyelonephritis developing in patients with vesico-ureteral reflux. TGF-beta1 is thought to be one of the most important factor that initiates reflux nephropathy and stimulates its progression. The purpose of the study was to assess serum TGF-beta1 level in children with reflux nephropathy. The study comprised 36 children (12 boys and 24 girls) aged 1-17 years with vesicoureteral reflux and renal scarring confirmed by scintigraphic evaluation. In most children recurrent urinary tract infections were observed. In 13 (27.8%) children, a first episode of urinary tract infection occurred in infancy. Primary vesico-ureteral reflux was diagnosed in 18 (50.0%) children. Secondary vesico-ureteral reflux due to anatomic or functional subvesical obstruction were recognized in 11 (30.6%) and 7 (19.4%) children, respectively. Serum TGF-beta1 levels were measured by ELISA. The mean serum TGF-beta1 level in children with reflux nephropathy was significantly lower than that in controls (92.04 +/- 33.14 ng/ml vs 141.73 +/- 45.38 ng/ml). This might be a result of increased influx of serum TGF-beta1 into renal parenchyma and/or increased urinary TGF-beta1 excretion due to tubular damage in children with reflux nephropathy.
Assuntos
Nefrite/sangue , Nefrite/etiologia , Fator de Crescimento Transformador beta1/sangue , Refluxo Vesicoureteral/sangue , Refluxo Vesicoureteral/complicações , Adolescente , Criança , Pré-Escolar , Regulação para Baixo , Feminino , Humanos , Lactente , Masculino , Nefrite/fisiopatologia , Valores de ReferênciaRESUMO
Since January 26th of 1996, when our center was established, 34 children (19 boys and 15 girls) with end-stage renal disease (ESRD) were treated with renal replacement therapy. Maintenance hemodialysis (HD) and peritoneal dialysis (PO) were received by 28 and 6 patients, respectively. In our patients, the most common cause of ESRD were congenital urinary system malformations (61.7%) and glomerulonephritides (20.6%). Duration of renal replace-ment therapy varied from 6 months to 8 years. In 71.5% of children on HD, vascular access was established by arteriovenous fistula, and in the remaining--by permanent venous catheter. Adequacy of renal replacement therapy was estimated by Kt/V ratio. In patients on HD and PO, Kt/V ratios were 1.27 and 1.9 per 7 days, respectively. In 82.4% of patients, pre-dialysis arterial hypertension was observed. During renal replacement therapy arterial hypertension was diagnosed in 73.5% of children. Anemia resulting from ESRD was treated with recombinant human erythropoietin and erythropoietic medicines. In all children, recommended hemoglobin and hematocrit levels were achieved. In our patients, parameters of calcium and phosphate handling were also monitored. The mean serum intact parathormone levels in HD and PO children were 304 pg/ml and 302.2 pg/ml, respectively. We also summarized the occurrence of hepatotropic viral infections and endocrine disturbances including hypothyreosis and short stature in our patients. 3 children died due to ESRD complication. 22 (64,7%) children received cadaver-donor renal transplants.
Assuntos
Hospitais Universitários/estatística & dados numéricos , Falência Renal Crônica/terapia , Terapia de Substituição Renal/estatística & dados numéricos , Adolescente , Causalidade , Criança , Pré-Escolar , Feminino , Seguimentos , Transtornos do Crescimento/epidemiologia , Transtornos do Crescimento/terapia , Humanos , Hipertensão/epidemiologia , Hipertensão/terapia , Lactente , Falência Renal Crônica/epidemiologia , Transplante de Rim/estatística & dados numéricos , Masculino , Diálise Peritoneal/estatística & dados numéricos , Polônia/epidemiologia , Diálise Renal/estatística & dados numéricosRESUMO
The purposes of the study were a) to assess the incidence of urinary system malformations in children with the nephrotic syndrome (NS) and b) to estimate their effect on clinical course of the NS. A retrospective analysis comprised medical records of patients aged 2-18 years hospitalized in the Department of Pediatric Nephrology, University Children's Hospital, Lublin. Urinary tract infections (UTI) occurred in 28% of children with the NS. Imaging evaluation revealed urinary system malformations in 21.2% of children with the NS and UTI. Urinary system malformations constituted 5.9% of all children hospitalized because of the NS. Urinary system malformations included vesico-ureteral reflux (42.8%), renal duplication (28.6%), renal agenesis (14.3%) and diverticulum of the bladder (14.3%). In 48% of children with the NS and urinary system malformations, frequent recurrences of the syndrome were observed.
Assuntos
Síndrome Nefrótica/epidemiologia , Sistema Urinário/anormalidades , Sistema Urinário/patologia , Doenças Urológicas/epidemiologia , Doenças Urológicas/fisiopatologia , Adolescente , Causalidade , Criança , Pré-Escolar , Comorbidade , Divertículo/epidemiologia , Feminino , Humanos , Incidência , Rim/anormalidades , Rim/patologia , Nefropatias/epidemiologia , Masculino , Polônia/epidemiologia , Prevalência , Recidiva , Estudos Retrospectivos , Bexiga Urinária/anormalidades , Bexiga Urinária/patologia , Doenças Urológicas/congênito , Refluxo Vesicoureteral/epidemiologiaRESUMO
BACKGROUND: Two-dimensional (2DUS) contrast enhanced voiding urosonography has been used in the diagnosis and treatment monitoring of the vesicoureteral reflux in children for over 15 years. The opportunity of performing this examination with the use of three-dimensional static (3DUS) and real-time (4DUS) techniques opens up new diagnostic horizons. OBJECTIVE: To analyze if 3DUS/4DUS bring additional information leading to an increased detection rate or change in the grading of reflux compared to 2DUS and voiding cystouretrography. MATERIAL AND METHODS: We evaluated 69 patients (mean 4.1 years) who underwent 2DUS/3DUS/4DUS contrast enhanced voiding urosonography (ceVUS) and voiding cystourethrography (VCUG) for the diagnosis and grading of vesicoureteral reflux. RESULTS: 2DUS and 3DUS/4DUS urosonography diagnosed 10 more refluxes (7.25%) than cystourethrography and in 3 refluxes (2.17%) detected a higher grade. In 9 refluxes (6.52%) 3DUS/4DUS urosonography and cystourethrography diagnosed a higher grade than 2DUS. There was a statistically significant difference between cystourethrography and 3DUS/4DUS urosonography when the number of detected refluxes and differences in grading were compared. 4DUS enabled a better visualization of reflux than 3DUS. CONCLUSIONS: 3DUS/4DUS techniques bring additional information leading to a change in reflux grading compared to 2DUS and a detect higher number of refluxes compared to cystourethrography.
Assuntos
Meios de Contraste , Aumento da Imagem/métodos , Imageamento Tridimensional/métodos , Ultrassonografia/métodos , Urografia/métodos , Refluxo Vesicoureteral/diagnóstico por imagem , Adolescente , Criança , Pré-Escolar , Cistografia/métodos , Feminino , Humanos , Lactente , Masculino , Reprodutibilidade dos Testes , MicçãoRESUMO
INTRODUCTION: Vesicoureteral reflux (VUR) occurs in 20-50% of children suffering from recurrent urinary tract infections (UTIs) and is associated with an increased risk of renal scarring and impaired renal function. Early detection of renal perfusion deterioration would allow for the implementation of more aggressive treatment and potentially prevent further damage to the renal parenchyma. The aim of the study was to assess renal parenchymal perfusions in children with recurrent UTIs with and without coexisting VUR, and compare the findings with the results of healthy patients. MATERIAL AND METHODS: Color Doppler sonographic dynamic renal parenchymal perfusion measurements were performed with PixelFlux (Chameleon-Software, Germany) software in 77 children with recurrent UTIs and coexisting VUR and in 30 children with UTIs without VUR. The findings were compared with the results of 53 healthy children. RESULTS: Cortical parenchymal perfusion of children suffering from UTIs and VUR was significantly reduced when compared to the control group. Statistically significant differences (p < 0.05) were found in all perfusion parameters (i.e. mean velocity (v mix ), mean perfused area (A mix ), mean perfusion intensity (I mix ), tissue pulsatility index (TPI), and tissue resistance index (TRI)) between the control group and children suffering from UTIs and VUR, particularly VUR grades III and IV. There were no significant differences between the UTI group and the control group. No differences were found between the controls and VUR grade II. CONCLUSIONS: Renal parenchymal perfusion decreases significantly with higher grades of VUR.