Combined use of late phase dimercapto-succinic acid renal scintigraphy and ultrasound as first line screening after urinary tract infection in children.

PURPOSE: The aim of this study was to evaluate the diagnostic accuracy of dimercapto-succinic acid renal scintigraphy and renal ultrasound in identifying high grade vesicoureteral reflux in children after a first episode of urinary tract infection. MATERIALS AND METHODS: A total of 533 children following a first urinary tract infection were included in the analysis. Patients were assessed by 3 diagnostic imaging studies, renal ultrasound, dimercapto-succinic acid scan and voiding cystourethrography. The main event of interest was the presence of high grade (III to V) vesicoureteral reflux. The combined and separate diagnostic accuracy of screening methods was assessed by calculation of diagnostic OR, sensitivity, specificity, positive predictive value, negative predictive value and likelihood ratio. RESULTS: A total of 246 patients had reflux, of whom 144 (27%) had high grade (III to V) disease. Sensitivity, negative predictive value and diagnostic OR of ultrasound for high grade reflux were 83.3%, 90.8% and 7.9, respectively. Dimercapto-succinic acid scan had the same sensitivity as ultrasound but a higher negative predictive value (91.7%) and diagnostic OR (10.9). If both tests were analyzed in parallel by using the OR rule, ie a negative diagnosis was established only when both test results were normal, sensitivity increased to 97%, negative predictive value to 97% and diagnostic OR to 25.3. Only 9 children (6.3%) with dilating reflux had an absence of alterations in both tests. CONCLUSIONS: Our findings support the idea that ultrasound and dimercapto-succinic acid scan used in combination are reliable predictors of dilating vesicoureteral reflux.

Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis.

Renal histological features of focal segmental glomerulosclerosis (FSGS) are found in 75% of pediatric patients with steroid-resistant nephrotic syndrome. In order to evaluate the predictive factors of chronic kidney disease (CKD), we retrospectively reviewed the records of 110 children with biopsy-proven FSGS admitted between 1972 and 2004. Renal survival was analyzed by the Kaplan-Meier method and Cox's regression model. Two multivariate models were developed: (1) from the onset of symptoms to the occurrence of CKD and (2) from the time of renal biopsy to CKD. Mean follow-up time was 10 years [standard deviation ((SD) 5.5], and 24 patients (21.8%) progressed to CKD. At baseline, after adjustment three variables remained as independent predictors of CKD: age >6.5 years (RR=3.3, 95% CI=1.3-7.8), creatinine >1 mg/dl (RR=2.5, 95% CI=0.97-6.5), and non-response to steroids (RR=7.3, 95% CI=2.7-19.7). In a model with continuous variables only age and non-response to steroids were associated with CKD. At the time of renal biopsy, after adjustment two variables remained as independent predictors of CKD: hematuria (RR=3.0, 95% CI=1.2-7.3) and creatinine >0.8 mg/dl (RR=4.3, 95% CI=1.7-10.6). In a model with continuous variables four factors predicted CKD: age, creatinine, hematuria, and percentage of global sclerosis.

Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis.

The purpose of this retrospective cohort study was to report the clinical course of children and adolescents with primary focal segmental glomerulosclerosis (FSGS). The records of 110 patients with biopsy-proven FSGS admitted between 1972 and 2004 were retrospectively reviewed. Demographic, clinical and laboratory data were recorded and histopathological data were reanalyzed by one pathologist who had no information about the outcome of the patients. Renal survival analysis was performed using the Kaplan-Meier method. Differences between subgroups (response to corticosteroids) were assessed by the two-sided log rank test. The median age at admission was 5 years (range: 1-15 years). Forty-two patients (38.2%) presented with hematuria at admission, and 55 (50%) presented blood pressure levels above the 95th percentile. Mean follow-up time was 10 years (SD 5.5). Twenty-four patients (21.8%) presented chronic kidney disease (CKD). It was estimated that the probability of CKD was 8% at 5 years, 17% at 10 years, and 32% at 15 years after diagnosis of nephrotic syndrome. In conclusion, on the basis of the clinical and histological characteristics observed, apparently our cohort of idiopathic FSGS is comparable with other published series. However, the long-term overall renal survival seems to be better in our cohort.

Estudo retrospectivo de coorte de 739 crianças com refluxo vesicoureteral primário / Retrospective cohort study of 739 children with primary

O b j e t i v o : Descrever as características clínicas, laboratoriais e radiológicas de pacientes com refluxo vesicoureteral primário (RVU) e avaliar o curso clínico. M é t o d o s : Dados foram obtidos retrospectivamente dos prontuários dos pacientes portadores de RVU encaminhados entre 1969 a 2004. Foram registrados os seguintes dados: sexo, idade do diagnóstico da infecçãourinária (ITU) e do RVU, pressão arterial, surtos de ITU, níveis de uréia e creatinina, tempo de seguimento e a evolução do refluxo. Os seguintes exames de imagem foram obtidos: ultra-sonografia (US), uretrocistografia miccional (UCM) e cintilografia renal. Para a comparação de freqüências foi utilizado o teste do qui-quadrado. Foram calculados odds ratio (OR) e intervalode confiança a 95 (IC 95) para a comparação de risco. R e s u l t a d o s : Um total de 739 pacientes foi incluído na análise. Houve predomínio do sexo feminino (71) e da cor branca (80). A apresentação clínica inicial mais freqüente foi ITU (92,4). O RVU foi bilateral em 385 (52). Assim, um total de 1.124 unidades renais apresentava refluxo; 373 apresentavamcicatrizes renais na admissão. Do total de 739 pacientes, 677 (91,6) foram abordados com tratamento conservador e 62 (8,4) submetidos a procedimento cirúrgico. Os pacientes foram acompanhados por um tempo médio de 76 meses (DP= 55 meses). Durante o acompanhamento, 319 (43) pacientes não apresentaram surtos de ITU. Hipertensão arterial foi detectada em 21 (2,8) e insuficiência renal em 22 (3,0) pacientes. C o n c l u s ã o : O RVU é uma entidade clínica heterogênea. Nossa casuística mostra que a conduta conservadora é eficaz, mas independentemente da abordagem inicial esses pacientes devem ser acompanhados até a idade adulta, especialmente aqueles com nefropatia do refluxo.

The renin angiotensin system in childhood hypertension: selective increase of angiotensin-(1-7) in essential hypertension.

OBJECTIVE: We examined the activity of the renin-angiotensin system (RAS) in normotensive and hypertensive children. STUDY DESIGN: Hypertensive patients (12 with renovascular hypertension and 15 with essential hypertension) and 32 normotensive subjects were evaluated at a Pediatric Nephrology Center. Blood samples for plasma renin activity (PRA) and angiotensin peptides measurements were obtained once from normotensive subjects and before and after treatment from hypertensive patients. Plasma samples were extracted using Bond-Elut cartridges (Analytichem International, Harbor City, Calif), and peptide concentrations were determined by radioimmunoassay (RIA). RESULTS: PRA, Angiotensin I (Ang I), Angiotensin II (Ang II), and Angiotensin-(1-7) [Ang-(1-7)] levels were significantly higher in renovascular hypertensive patients than in normotensive children (3.3 +/- 1.2 vs 0.40 +/- 0.22 ng Ang I/mL/hour, 81.4 +/- 24.8 vs 26.4 +/- 13.4 pg/mL, 59.3 +/- 17.0 vs 21.4 +/- 8.7 pg/mL, 41.0 +/- 10.5 vs 16.2 +/- 7.9 pg/mL, respectively). The surgical treatment normalized blood pressure, PRA, and angiotensins levels. In contrast with renovascular disease, only Ang-(1-7) levels were significantly increased in essential hypertensive patients compared with normotensive (78.8 +/- 22.8 vs 16.2 +/- 7.9 pg/mL). Treatment with calcium channel blockers did not alter the RAS measurements. CONCLUSION: Our data show different RAS profiles in childhood hypertension and suggest a blood pressure-independent change of Ang-(1-7) in essential hypertension.

Aspectos atuais na abordagem do refluxo vésico-ureteral / Advances in management of vesicoureteral reflux

The advances of knowledge in the clinical course of vesicoureteral reflux and its consequences have allowed an improvement in the management of this common disease in children. In the last years, several facts have contributed for a better understanding of the etiology of reflux and of the associated renal damage. Among them, it is important to emphasize the neonatal diagnosis of the reflux in the investigation of fetal hydronephrosis, the precise detection of renal damage by renal scintigraphy and the advances of the genetic of primary vesicoureteral reflux. The aim of this study is review these aspects and the recent clinical trials...

Os avanços no conhecimento do curso clínico do refluxo vésico-ureteral e de suas consequências têm permitido um aperfeiçoamento na abordagem dessa doença freqüente na faixa etária pediátrica. Nos últimos anos, vários fatores têm contribuído para uma maior compreensão da genêse do refluxo e do dano renal associado. Entre esses destacam-se o diagnóstico neonatal do refluxo na investigação da hidronefrose fetal, a detecção mais precisa do dano renal através da cintilografia renal e os avanços no conhecimento da genética do refluxo vésico-ureteral primário. Esta revisão enfatiza esses avanços e os mais recentes estudos prospectivos controlados abordando a questão do tratamento do refluxo...

Outcome of apparent ureteropelvic junction obstruction identified by investigation of fetal hydronephrosis.

OBJECTIVES: The purpose of the study was to evaluate the outcome of prenatally detected ureteropelvic junction obstruction (UPJO) managed with a more conservative protocol. METHODS: The records and imaging studies of 77 consecutive neonates with UPJO identified by fetal hydronephrosis were reviewed. A nonoperative approach was attempted in patients with mild/moderate pelvic dilatation, renal units with good function as ascertained by DMSA scan and a non-obstructed pattern on DTPA. Otherwise, the patients were managed surgically by pyeloplasty. Both groups were prospectively followed and the imaging studies were performed before and after the initial approach and at one-year intervals thereafter. RESULTS: Of the 77 infants (85 units), 39 were submitted to surgery (33 pyeloplasty and 7 nephrectomy) and 38 were conservatively managed. During follow-up, 9 (24%) of 38 patients in the non-operative group presented renal function deterioration and 3 presented with urinary infections and were submitted to pyeloplasty. Of the 39 patients surgically managed, 76% presented improvement of hydronephrosis and 90% showed a non-obstructed pattern on diuretic renography. The differential renal uptake, as measured by DMSA scan, remained stable in the three groups analyzed (conservative, initial pyeloplasty, and delayed pyeloplasty). There was a minimal improvement in those units submitted to pyeloplasty with impaired renal function at baseline (< 40%). Mean renal uptake was 28.6% at admission and 33.9% at the end of follow-up. CONCLUSION: There was a wide spectrum of ureteropelvic junction stenosis. Surgical intervention in a subgroup of patients with severe hydronephrosis and impaired function may possibly improve or preserve renal parenchyma. Conversely, conservative management and clinical follow-up are safe and desirable for the subgroup with mild/moderate pelvic dilatation and preserved renal function.

Síndrome de Liddle: diagnóstico tardio de causa rara de hipertensão arterial / Liddle's syndrome: late diagnosis of a rare cause of arterial hypertension

Objetivo: a finalidade desse relato de caso é chamar a atenção para uma doença que, embora rara, é causa de hipertensão arterial grave em crianças. O relato procura chamar a atenção dos pediatras para a importância do diagnóstico precoce de hipertensão arterial, e do valor de se estabelecer uma investigação apropriada a cada caso.Relato de caso: neste artigo é relatado uma caso de um paciente de treze anos, do sexo masculino, com quadro clínico e laboratorial típico dessa doença. O diagnóstico foi estabelecido a partir do quadro clínico associado à presença de hipocalemia crônica, aumento da excreção urinária de potássio acoplado à retenção de sódio, e dimi- nuição da atividade de renina plasmática e dos níveis circulantes de Angiotensina II e aldosterona. Apesar da resposta inicial ao uso de triamtereno, o paciente evoluiu com perda da função renal, secundária ao retardo no diagnóstico e ao mau controle da hipertensão. Discussão: a Síndrome de Liddle consiste numa forma de pseudo-aldosteronismo caracterizado por hipertensão arterial, hipocalemia, alcalose metabó1ica e retardo no crescimento. Alguns aspectos da fisiopatologia, do diagnóstico e do tratamento são discutidos no estudo

Prognostic factors in prenatally-detected posterior urethral valves: a multivariate analysis.

To identify prognostic factors associated with chronic renal insufficiency in children with posterior urethral valves (PUV), 22 children with PUV were submitted to a systematic protocol and prospectively followed. Prognostic factors associated with fetal echography and clinical and laboratory findings were studied on admission. Median follow-up was 76 months. The analysis was conducted in two steps: in univariate analysis, variables associated with adverse outcome were identified by the Kaplan-Meier method. The variables that were significantly associated with adverse outcome were then included in a multivariate analysis using the Cox model. Eleven patients (50%) developed chronic renal failure (CRF) during follow-up. After adjustment by the multivariate model, four factors were identified as independent predictors of adverse outcome: oligohydramnios (relative risk [RR] = 10.6, 95% CI = 2.7 - 77, P = 0.02), ventilatory support (RR = 6, 95% CI = 2 - 24, P = 0.01), urea higher than 40 mg/dl (RR = 3.7, 95% CI = 0.92 - 15.0, P = 0.06), and bilateral vesicoureteral reflux (VUR) (RR = 6.1, 95% CI = 1.25 - 30, P = 0.02). On the other hand, the presence of unilateral VUR was a protective factor against the development of CRF or death during follow-up (RR = 0.92, 95% CI = 0.87 - 0.98, P = 0.05).

[Liddle's syndrome: late diagnosis of a rare cause of arterial hypertension]. / Síndrome de Liddle: diagnóstico tardio de causa rara de hipertensão arterial.

OBJECTIVE: The aim of this article is to highlight the importance of a rare disease that causes severe arterial hypertension in children. It is important to advise pediatricians to measure arterial pressure in children in order to avoid late diagnosis and renal insufficiency. DESCRIPTION: We report a case of a 13-year-old patient that presented typical clinical and laboratorial features of Liddle's syndrome. The diagnosis was established based on the clinical picture associated with the presence of chronic hypokalemia, increase of urinary potassium excretion with sodium retention and reduction of renin plasma activity, and circulating levels of angiotensin II and aldosterone. In a spite of the initial therapeutic response to triamterene, the patient developed progressive renal failure due to the delay in the diagnosis and the poorly controlled hypertension. COMMENTS: Liddle's syndrome consists of a form of pseudoaldosteronism characterized by arterial hypertension, hypokalemia, metabolic alkalosis and failure to thrive. Some aspects regarding physiopathology, diagnosis and treatment are discussed.

Revisäo: Hidronefrose fetal - abordagem pós-natal, avanços e controvérsias / Fetal hydronephrosis - advances and controversies in the post natal approach

Avanços no diagnóstico pré-natal nas últimas duas décadas têm permitido o aprimoramento da detecçäo e o manejo das anormalidades do trato urinário. A ultra-sonografia pré-natal permite o reconhecimento de anormalidades urológicas que somente seriam identificadas tardiamente, quando surgissem sintomas de pielonefrite, dor abdominal e cólica renal. O estudo consistiu de uma revisäo da literatura atual sobre o diagnóstico pré-natal das anomalias do trato urinário. Os dados obtidos foram confrontados com a experiência da Unidade de Nefrologia Pediátrica do HC/UFMG na conduta e no segmento de crianças portadoras de uropatias detectadas na investigaçäo de hidronefrose fetal. Uma anormalidade envolvendo o trato urinário pode ser suspeitada, dependendo do critério ecográfico adotado, em uma a cada 100 gestaçöes. Na conduta pré-natal devem ser considerados: o bem-estar fetal global; a idade gestacional; se a hidronefrose é unilateral ou bilateral; e o volume de líquido amniótico. Após o nascimento, deve ser iniciada a profilaxia antibiótica e obtidos exame ultra-sonográfico e uretrocistografia miccional. No caso hidronefrose moderada ou grave, cintilografia renal também é recomendada. As uropatias mais frequentes identificadas na investigaçäo de hidronefrose fetal säo obstruçäo de junçäo de ureteropélvica, refluxo vesicoureteral, rim displásico multicístico, megaureter primário e válvula de uretra posterior. A pesquisa da literatura e os dados do presente estudo mostram que a maioria dessas anormalidades pode ser conduzida de modo conservador, com a exceçäo clara da válvula de uretra posterior.(au)

Curso clínico da válvula de uretra posterior detectada intra-útero: seguimento a longo prazo / Clinical course of posterior urethra detected intra-uterus : long follow-up

Com o objetivo de avaliar o curso clínico da VUP identificada na vida fetal, foram estudadas 15 crianças prospectivamente com o objetivo de avaliar o curso clínico da válvula de uretra posterior (VUP). Pacientes com hidronefrose detectada no pré-natal foram avaliados no período pós-natal, submetidos a um protocolo sistemático e seguidos prospectivamente. Após início daquimioprofilaxia, a avaliaçäo de imagens consistiu de ultrasonografia (US), uretrocistografia miccional (UCM) e cintilografia estática por DMSA. Nos casos confirmados de VUP, as crianças foram submetidas a vesicostomia e seguidas com avaliaçöes clínicas e laboratoriais periódicas. Quinze meninos foram identificados como portadores de VUP entre 1989 e 1998. A média de idade do diagnóstico pré-natal foi de 30 meses. O tempo médio de seguimento foi de 40 meses. Das 15 crianças acompanhadas, 7 (47 porcento) evoluíram com isuficiência renal crônica (IRC) e 6 (40 porcento) faleceram. O óbito esteve associado à perda da funçäo renal (RR = 7, p=0,001). Apesar do diagnóstico precoce, a VUP continua a representar um desafio para a área da nefrologia pediátrica, e os riscos de perda da funçäo renal e de óbito se mantêm elevado

Diabetes insipidus como uma manifestação clínica inicial de tumor da região pineal / Diabetes insipidus as an early clinical manisfestation of pineal

Objetivos: Relatar o diagnóstico de tumor da pineal identificado após longa evolução de um quadro de diabetes insipidus. Relato do caso: Criança de 10 anos de idade, com sintomas de poliúria, polidipsia e noctúria de evolução há 18 mese, foi admitido na Unidade de Nefrologia com suspeita diagnóstica de diabetes insipidus nefrogênico. Já havia sido submetida a tomografia computadorizada do encéfalo, que foi considerada inconclusiva. Após um teste de privação de água e estímulo com desmopressina, foi obtido o diagnóstico de diabetes insipidus central. a ressonância magnética revelou a presença de duas massas intracraninas sugestivas de germinoma. Conclusões: Nossas observações mostram que pacientes que se apresentam com diabetes insipidus como manifestação isolada, necessitam de seguimento clínico, laboratorial e neuro-radiológico adequado, com a finalidade de se diagnosticar tumores da região pineal mais precocemente