RESUMO
UNLABELLED: Tubulointerstitial fibrosis and tubular atrophy play a crucial role in the pathogenesis of chronic kidney disease (CKD). They are also major determinants in chronic kidney disease development and progression in patients with primary renal diseases characterized by persistent or recurrent proteinuria. The purpose of the study was to assess urinary excretion of alpha-glutathione S-transferase (alpha-GST), pi-glutathione S-transferase (pi-GST), neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and serum NGAL level in children with idiopathic nephrotic syndrome (INS). PATIENTS AND METHODS: the study group comprised of 39 children with INS and the control group consisted of 20 healthy children. A total of 23 patients were affected with steroid-dependent nephrotic syndrome (SDNS) and 16 with steroid-resistant nephrotic syndrome (SRNS). In the majority of patients, a histopathologic examination revealed minimal change disease (MCD)-25 (64%). Focal segmental glomerulosclerosis (FSGS), mesangioproliferative glomerulonephritis (MesPGN), membranoproliferative glomerulonephritis (MPGN), and membranous glomerulonephritis (MGN) were diagnosed in 4 (10.3 %), 6 (15.5%), 2 (5.1%), and 2 (5.1%) children, respectively. Urinary alpha-GST, urinary pi-GST, urinary KIM-1, and urinary and serum NGAL concentrations were measured using specific enzyme-linked immunosorbent assay. The urinary results were expressed in nanograms per milligram of creatinine (ng/mg). RESULTS: The authors observed significantly higher levels of urinary alpha-GST/creatinine ratio (P = 0.03), urinary KIM-1/creatinine ratio (P < 0.02), serum NGAL level (P < 0.01), and urinary NGAL/creatinine ratio (P = 0.02) in children with INS compared with controls. The median values of urinary pi-GST/creatinine ratio in children with INS and controls did not differ significantly. In children with SRNS, the median values of urinary NGAL/creatinine ratio (P = 0.02) and urinary KIM-1/creatinine ratio (P = 0.02) were significantly higher compared with children with SDNS. The authors noted significant positive correlation between KIM-1/creatinine ratio and proteinuria (r = 0.56, P < 0.05). The analysis of alpha-GST/creatinine ratio, pi-GST/creatinine ratio, sNGAL, and uNGAL/creatinine ratio concerning the histopathologic examination, the duration of the disease, and number of relapses did not show any significant differences. CONCLUSIONS: 1. Both children with SDNS and those with SRNS were characterized by increased tubular injury marker levels. 2. Patients with SRNS and higher proteinuria are more susceptible to early kidney damage.
Assuntos
Nefropatias/etiologia , Túbulos Renais/patologia , Síndrome Nefrótica/complicações , Adolescente , Biomarcadores/análise , Criança , Pré-Escolar , Diagnóstico Precoce , Feminino , Fibrose/diagnóstico , Fibrose/etiologia , Humanos , MasculinoRESUMO
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/citologiaRESUMO
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
Growth references are useful in monitoring a child's growth, which is an essential part of child care. The aim of this paper was to provide updated growth references for Polish school-aged children and adolescents and show the prevalence of overweight and obesity among them. Growth references for height, weight, and body mass index (BMI) were constructed with the lambda, mu, sigma (LMS) method using data from a recent, large, population-representative sample of school-aged children and adolescents in Poland (n = 17,573). The prevalence of overweight and obesity according to the International Obesity Taskforce definition was determined with the use of LMSGrowth software. Updated growth references for Polish school-aged children and adolescents were compared with Polish growth references from the 1980s, the Warsaw 1996-1999 reference, German, and 2000 CDC references. A positive secular trend in height was observed in children and adolescents from 7 to 15 years of age. A significant shift of the upper tail of the BMI distribution occurred, especially in Polish boys at younger ages. The prevalence of overweight or obesity was 18.7% and 14.1% in school-aged boys and girls, respectively. The presented height, weight, and BMI references are based on a current, nationally representative sample of Polish children and adolescents without known disorders affecting growth. Changes in the body size of children and adolescents over the last three decades suggest an influence of the changing economical situation on anthropometric indices.
Assuntos
Gráficos de Crescimento , Adolescente , Estatura , Índice de Massa Corporal , Peso Corporal , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Modelos Estatísticos , Obesidade/epidemiologia , Sobrepeso/epidemiologia , Polônia/epidemiologia , Adulto JovemRESUMO
BACKGROUND AND AIM: Oxalobacter formigenes is an intestinal bacterium that utilizes oxalate as the only source of energy. It has been suggested that the lack of colonization with this organism may be a risk factor for calcium oxalate urolithiasis. Because this problem was not investigated in pediatric stone formers, we decided to assess it in our patients. METHODS: The presence of O. formigenes in stool samples of 76 children and adolescents (aged 4.1-18 years) with idiopathic calcium urolithiasis (36 with chemically confirmed calcium oxalate stones and 40 children with a strong clinical suspicion of this type of urolithiasis) was assessed using PCR method. Simultaneously, urinary oxalate excretion was measured in this group. Fifty healthy, age- and sex-matched subjects served as controls. RESULTS: O. formigenes was found in 21/76 patients (27.6%). In controls, frequency of colonization was similar (26%). The median 24h urinary oxalate excretion in patients colonized with O. formigenes was significantly lower in comparison with non-colonized patients, 0.319 (range 0.141-0.546) and 0.437 (range 0.198-0.967) mmol/1.73 m(2)/24h, respectively. CONCLUSIONS: Higher urinary oxalate excretion in children with calcium urolithiasis may be a result of the absence of O. formigenes. The reasons for similarly low intestinal colonization with this bacterium in normal subjects and stone formers remain speculative. Thus, further studies are necessary to clarify this issue.
Assuntos
Intestinos/microbiologia , Oxalatos/metabolismo , Oxalobacter formigenes/metabolismo , Urolitíase/microbiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Mucosa Intestinal/metabolismo , Masculino , Oxalatos/urina , Urolitíase/metabolismo , Urolitíase/urinaRESUMO
The pathogenesis of calcium urolithiasis involves complex interactions of urinary promoters and inhibitors of crystallization. A variety of risk formulas have been established to approximate these interactions for clinical evaluation, and the aim of our study was to determine their usefulness as predictors of stone formation. The study cohort comprised 126 patients (63 boys and 63 girls) aged 6.7-18 years (mean age 14.1 +/- 2.9 years) with calcium urolithiasis (61 with chemically confirmed calcium oxalate stones and 65 children with a strong clinical suspicion of this type of urolithiasis). Of these, 36 children were classified as recurrent stone-formers, whereas the remaining 90 had experienced only one stone episode. The values obtained were compared to those of a control group of 60 age- and gender- matched healthy children. A number of crystallization risk indices were calculated from analytes obtained in 24-h urine: calcium/magnesium ratio (Ca/Mg), calcium/citrate ratio (Ca/Cit), (calcium x oxalate)/(magnesium x citrate) ratio (CaOx/MgCit), relative urinary CaOx supersaturation (RS(CaOx)), CaOx activity product index (AP(CaOx)), and standardized CaOx activity product index (AP(CaOx stand)). All indices, except for the AP(CaOx) index, were significantly higher in stone-formers than in the controls. The Ca/Mg, Ca/Cit, CaOx/MgCit, AP(CaOx), and AP(CaOx stand) indices were significantly higher in recurrent stone-formers than in first-episode ones. However, the determination of precise cutoffs between pathological and non-pathological values was problematic due to a considerable overlap of individual values. Based on our results, we conclude that calculation of the majority of risk indices may play a rather supplementary role in the evaluation of children with calcium urolithiasis.
Assuntos
Cálculos Renais/urina , Urolitíase/urina , Adolescente , Cálcio/urina , Oxalato de Cálcio/urina , Criança , Ácido Cítrico/urina , Cristalização , Feminino , Humanos , Magnésio/urina , Masculino , Oxalatos/urina , Fatores de RiscoRESUMO
Plasma oxalate (POx) concentration is significantly elevated in primary hyperoxaluria, severe renal failure or ethylene glycol poisoning. In these conditions, the degree of hyperoxalemia correlates with the severity of systemic calcium oxalate (CaOx) deposition and should be therefore carefully monitored. Although secondary hyperoxaluria (secHyOx) is a common finding in pediatric patients with kidney stone disease, very little is known about POx in this condition. We therefore evaluated POx level in 59 children and adolescence with calcium urolithiasis (34 confirmed by CaOx stone analysis and 25 children with a strong clinical suspicion of this type of urolithiasis), with or without "mild" secHyOx. A control group consisted of 41 healthy sex- and age-matched children. We found that POx was significantly increased in children with calcium urolithiasis and secHyOx compared to healthy children (9.16 +/- 3.60 vs. 6.42 +/- 2.53 micromol/l), but that was not the case in children with calcium urolithiasis but with normal urinary oxalate excretion (7.12 +/- 3.33 micromol/l). We conclude that POx may be slightly increased in some pediatric calcium stone formers with secHyOx, probably related to intestinal oxalate hyperabsorption.
Assuntos
Hiperoxalúria/sangue , Oxalatos/sangue , Urolitíase/sangue , Adolescente , Oxalato de Cálcio/urina , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Humanos , Hiperoxalúria/metabolismo , Hiperoxalúria/urina , Absorção Intestinal , Masculino , Oxalatos/metabolismo , Urolitíase/metabolismo , Urolitíase/urinaRESUMO
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: Hyperoxaluria is one of the crucial risk factors for calcium stone formation. AIM: Estimation of own reference range for 24-hour urinary oxalate (OX) excretion and evaluation of incidence of primary and secondary hyperoxaluria in children with calcium urolithiasis. MATERIALS AND METHODS: The study comprised 128 healthy children aged 4-17,9 years and 137 children aged 3.7-18 years with calcium urolithiasis. RESULTS: In healthy children, the mean 24-hour urinary OX excretion was 0.305 +/- 0.109 mmol/ 1.73 m2/ 24 h. An upper normal limit for urinary OX excretion was assumed at 0.474 mmol/1, 73 m2 / 24 h (95th percentile). Patients with stones had significantly higher urinary OX excretion (0.381 +/- 0.173 mmol / 1.73 m/ 24 h) in comparison to healthy children. Secondary hyperoxaluria was revealed in 37 (27%) children, whereas primary hyperoxaluria type I was diagnosed in only 1 (0.7%). CONCLUSIONS: Secondary hyperoxaluria occurred in a significant percentage of children with calcium urolithiasis, predominantly in males, whereas primary hyperoxaluria is casuistic.
Assuntos
Hiperoxalúria/urina , Oxalatos/urina , Cálculos Urinários/urina , Adolescente , Cálcio/análise , Criança , Pré-Escolar , Comorbidade , Feminino , Humanos , Hiperoxalúria/epidemiologia , Masculino , Valores de Referência , Cálculos Urinários/química , Cálculos Urinários/epidemiologiaRESUMO
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
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
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
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
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
We present a 5-years old boy with acquired Fanconi-de Toni-Debre syndrome being a effect of therapy for Ewing's sarcoma. At the age of 3 years, this boy was diagnosed as suffering from Ewing sarcoma of his right femur. The boy received a course of 8-month pre-surgery (6 VIDE--Vincristine, Ifosfamide, Doxorubicin, Etoposide cycles and 2 VAI--Vincristine, Actinomycin, Ifosfamide cycles) and 6-month post-surgery (6 VAI--Vincristine, Actinomycin, Ifosfamide cycles) cytostatic therapies according to EWING, EURO 99 protocol. In forth month of post-surgery cytostatic therapy, progressive malaise, polyuria, polydypsia, and recurrent vomiting occurred. The association between those symptoms and malignancy was excluded. Laboratory studies revealed hypokaliemia, hypophosphatemia, proximal tubular acidosis, proteinuria, glucosuria, aminoaciduria, hyperkaliuria and hyperphosphaturia. Acquired Fanconi-de Toni-Debre syndrome due to toxic effect of cytostatic therapy on renal proximal tubules was diagnosed. At present, two years after the time the diagnosis was made, despite constant substitution of potassium, phosphates and bicarbonates, deficit of body mass and height, and bone mineral density abnormalities are observed.