ABSTRACT Introduction: Voiding diary (VD) is an important tool in the evaluation of children with voiding symptoms. Voiding frequency, maximal voided volume (MVV), average voided volume (AVV) and nocturnal volume (NV) can be extracted and are valuable in diagnosing and monitoring these disorders. Recently, ICCS has reduced the period of data recording on VD from 3 to 2 days. We hypothesized that one day voiding diary would be enough for guiding treatment. Materials and Methods: Children with overactive bladder (OAB) and primary monosymptomatic enuresis (PMNE) were oriented to fulfill a 3-day VD. Data obtained from VD were evaluated for the first day (1dVD), the first two days (2dVD), and all 3 days (3dVD) and compared according to the MVV, AVV, frequency, NV and expected bladder capacity (EBC). The Friedman, Student's t test and the Fisher's exact was used. ANOVA was used for multiple comparisons. We also used Pearson correlation test. Results: Ninety-eight children were included, 59 had PMNE and 30 OAB. Frequency, AVV and VN were similar regardless how many days the voiding episodes were recorded. Only MVV was higher by a mean of only 32 mL on 3dVD compared to 1dVD. A 1dVD has a sensitivity of 93,9% and a positive likelihood ratio of 2.2. As for the correlation of MVV and EBC it was observed that in 83% of children, MVV was lower than EBC. MVV corresponds to 67% and 69% of EBC in children with PMNE and OAB, respectively. Conclusion: We believe that 1dVD is sufficient to assess these children. It has a high sensitivity and good correlation to 3dVD in evaluating these children. Bladder capacity in this population, evaluated by maximum voided volume, was close to 68% of that obtained by the EBC.

ABSTRACT Introduction: Upper airway obstruction (UAO) is a common condition in all pediatric population, with a 27% prevalence. Primary monosymptomatic nocturnal enuresis (PMNE) is a condition related to UAO in 8% to 47% of these children. The specific pathophysiological mechanism of this bond is not well understood. Some authors suggest a connection between brain natrituretic peptide (BNP) and anti-diuretic hormone (ADH) during sleep. The aim of this study was to evaluate hormone profile (ADH and BNP) and improvement in dry nights in a sample of children before and after surgical treatment of the UAO. Methods: This is a longitudinal prospective interventionist study in children, 5 to 14 years of age, with UAO and PMNE recruited in a specialty outpatient clinic. Children presenting UAO and PMNE were evaluated with a 30-day dry night diary and blood samples were collected to evaluate ADH and BNP before and after upper airway surgery. Data were analyzed prior to surgery and 90-120 days after surgery. Results: Twenty-one children with a mean age of 9.7 years were included. Mean BNP before surgery was 116.5 ± 126.5 pg/mL and 156.2 ± 112.3 pg/mL after surgery (p<0.01). Mean ADH was 5.8 ± 3.2 pg/mL and 14.6 ± 35.4 before and after surgery, respectively (p=0.26). The percentage of dry nights went from 32.3 ± 24.7 before surgery to 75.4 ± 33.4 after surgery (p<0.01). Conclusion: Surgery for airway obstruction contributed to an increase in BNP without increasing ADH. A total of 85.8% of the children presented partial or complete improvement of their enuresis.

Resumo Objetivos Crianças com síndrome de Down apresentam desenvolvimento psicomotor atrasado, fator que influencia o nível de dificuldade do treinamento esfincteriano. O presente estudo tem como objetivo estimar a idade em que o treinamento esfincteriano é iniciado e concluído em crianças com SD em comparação com crianças com desenvolvimento psicomotor normal, avaliar o método e o tipo de treinamento esfincteriano utilizado com maior frequência, bem como sua associação com sintomas do trato urinário inferior e constipação funcional. Métodos Um estudo caso-controle foi realizado de 2010 a 2015. Todos os pais preencheram um questionário destinado a avaliar o processo de treinamento esfincteriano. O sintomas do trato urinário inferior foram avaliados por meio da aplicação do Dysfunctional Voiding Symptom Score. A presença de constipação funcional foi avaliada de acordo com os critérios Roma III. Resultados O estudo incluiu 93 crianças com síndrome de Down e 204 crianças com desenvolvimento psicomotor normal (Grupo de Controle [GC]). A idade média em que as crianças iniciaram o treinamento esfincteriano foi de 22,8 meses naquelas com SD e 17,5 meses no GC (p = 0,001). Em crianças com SD, a idade média ao concluir o treinamento esfincteriano foi de 56,2 meses e 27,1 meses no GC (p = 0,001). Entre as crianças com SD, as do sexo feminino concluíram o treinamento esfincteriano mais cedo (p = 0,02). O método de treinamento esfincteriano mais utilizado foi a abordagem voltada para a criança em ambos os grupos. Não houve associação com a presença de sintomas do trato urinário inferior ou constipação funcional e a idade no início e na conclusão do treinamento esfincteriano em ambos os grupos. Conclusão Crianças com síndrome de Down apresentaram tempo de treinamento esfincteriano prolongado. Estudos longitudinais prospectivos são essenciais para obter uma visão do treinamento esfincteriano dessas crianças.

Abstract Objectives Children with Down syndrome have delayed psychomotor development, which is a factor that influences the level of difficulty in toilet training. The current study aims to estimate the age toilet training starts and completes in children with DS compared to children with normal psychomotor development and to evaluate the method and type of toilet training most frequently used, as well as its association with lower urinary tract symptoms and functional constipation. Methods A case-control study was carried out from 2010 to 2015. All parents completed a questionnaire designed to assess the toilet training process. Lower urinary tract symptoms were assessed through the application of the Dysfunctional Voiding Symptom Score. The presence of functional constipation was assessed according to the Rome III criteria. Results The study included 93 children with Down syndrome and 204 children with normal psychomotor development (control group [CG]). The mean age of toilet training onset was 22.8 months in those with DS and 17.5 months in the CG (p = 0.001). In children with DS, the mean age when completing toilet training was 56.2 months and 27.1 months in the CG (p = 0.001). Among children with DS, females completed toilet training earlier (p = 0.02). The toilet training method used most often was child-oriented approach in both groups. No association was observed with the presence of lower urinary tract symptoms or functional constipation and the age of beginning and completing toilet training in both groups. Conclusion Children with Down syndrome experienced prolonged toilet training time. Prospective longitudinal studies are essential to gain insight into the toilet training of these children.

Resumo Introdução: A criptorquidia é uma condição comum e prevalente em pacientes com síndrome de Down. Fatores ambientais, como o tabagismo, estão associados a malformações fetais. A avaliação da prevalência do criptorquidismo e a associação com tabagismo dos pais na síndrome de Down podem contribuir para alertar os profissionais de saúde e familiares sobre a prevenção dos danos causados pelo criptorquidismo e os possíveis fatores predisponentes. Objetivo: Avaliar a prevalência de criptorquidismo na síndrome de Down e a associação com tabagismo materno e paterno. Método: Quarenta (40) pacientes acompanhados em um centro de referência para atendimento da síndrome de Down foram avaliados por meio de questionário semiestruturado para avaliação de antecedentes parentais e características sociodemográficas, bem como de exames físico e laboratoriais complementares. Resultados: Criptorquidia foi observada em 27,5% dos pacientes (IC95% 15,98-42,96). Nesses pacientes, o criptorquidismo foi encontrado em 55% (5/9) das crianças cujas mães fumavam e em 19,35% (6/31) daquelas cujas mães não fumavam (OR = 5,26 [IC95% 1,06-25,41]; p=0,032). Do mesmo modo, o tabagismo paterno foi observado com maior frequência entre crianças com criptorquidia, 63,36% (7/11) e 31,03% (9/29), respectivamente (OR = 3,89 [IC95% 0,91-16,73]; p=0,060). Conclusão: A prevalência de criptorquidismo é alta em pacientes com síndrome de Down. Podemos mostrar uma forte associação entre hábito tabágico dos pais e ocorrência de criptorquidismo, especialmente no caso de tabagismo materno.

Summary Introduction: Cryptorchidism is a common and prevalent condition in patients with Down syndrome. Environmental factors, such as smoking, can be associated with malformations during fetal development. The study of the prevalence of cryptorchidism and its association with parental tobacco use in Down syndrome can contribute to alert health care professionals, patients and family members regarding the prevention of the harms caused by cryptorchidism and its possible predisposing factors. Objective: To evaluate the prevalence of cryptorchidism in Down syndrome and its association with maternal and paternal smoking. Method: Forty (40) patients of a public clinic specialized in Down syndrome were evaluated, using a semi-structured questionnaire for evaluation of antecedents and sociodemographic characteristics, as well as physical and complementary examinations. Results: Cryptorchidism was observed in 27.5% of the patients (95CI 15.98-42.96). Of these, 55% (5/9) were the children of mothers who smoked during pregnancy, and 19.35% (6/31) were the children of mothers who did not smoke during pregnancy (OR = 5.26 [95CI 1.06-25.41]; p=0.032). Similarly, paternal smoking was also observed in greater frequency among the parents of cryptorchid patients compared with subjects with descended testis, 63.36% (7/11) and 31.03% (9/29), respectively (OR = 3.89 [95CI 0.91-16.73]; p=0.060). Conclusion: The prevalence of cryptorchidism is high in patients with Down syndrome. We can show a strong association between smoking parents and the occurrence of cryptorchidism, especially when it comes to maternal smoking.

Resumo Introdução: A ultrassonografia (US) é um procedimento rápido, não invasivo e seguro que possibilita ao nefrologista obter informação vital à beira do leito, assim como permite guiar os procedimentos necessários à prática nefrológica. Relato do caso: Paciente masculino, idoso, com hipertensão arterial, diabetes mellitus e doença renal crônica apresenta-se com abaulamento infraumbilical que a Point of Care US (POCUS), realizada pelo nefrologista, mostrou ser um quadro de retenção urinária acompanhado de divertículo vesical. Adicionalmente, a POCUS possibilitou ao nefrologista diagnosticar hidronefrose bilateral, preservação da diferenciação córtico-medular e da ecotextura do rim direito, retenção urinária pós-miccional, correto posicionamento do cateter vesical pós-drenagem e acompanhar funcionalmente e morfologicamente as alterações do trato urinário após a correção da obstrução infravesical. Conclusão: A avaliação do trato urinário na ótica da POCUS eleva a prática nefrológica a um patamar mais alto, ao possibilitar a ampliação de informações clínicas imediatas e à beira do leito, proporcionar maior rapidez na resolução dos casos e permitir o monitoramento do tratamento instituído.

Abstract Introduction: Ultrasonography (US) is a rapid, non-invasive and safe procedure that allows the nephrologist to obtain vital information to the bedside, as well as allows to guide the procedures for nephrology practice. Case report: Male patient, elderly with hypertension, diabetes mellitus and chronic kidney disease presents with infraumbilical protrusion that the Point of Care US (POCUS), performed by the nephrologist, proved to be a large bladder with a diverticulum. In addition, the US enabled the nephrologist to diagnose bilateral hydronephrosis, preservation of the cortico-medullary differentiation and echotexture of the right kidney, post-voiding urinary retention, urinary catheter placement and functional and morphological monitoring of the urinary tract after surgical correction of the infravesical obstruction. Conclusion: POCUS assessment of the renal tract may become the new standard of care among nephrologists by enabling the expansion of clinical information in a timely fashion, allowing faster resolution of cases and permitting the monitoring of the treatment done.

A case of spontaneous perforation of the bladder in a diabetic female patient is reported. It is a rare clinical condition, that should be suspected in patients with a past history of radiotherapy to the pelvis, enterocystoplasty and those suspected of having a tumor in the bladder. A general surgeon should be aware of this possibility in the differential diagnosis of an acute abdomen.

OBJETIVO: Relatar um caso de angiomiolipoma gigante, bilateral, associado a esclerose tuberosa, tratado com sucesso e revisar a literatura concernente ao tratamento do angiomiolipoma. RELATO DO CASO: Paciente portadora de esclerose tuberosa, com diagnóstico de angiomiolipoma realizado por ultra-sonografia durante gestação. O tumor apresentava 9cm de diâmetro, à direita. Optou-se por conduta conservadora durante a gestação, e a paciente retornou somente 7 anos após, com tumor de 24,7 x 19,2 x 10,7 cm à direita e outro à esquerda de 13 x 11,5 x 6,5 cm, além de múltiplos angiomiolipomas pequenos. Realizada inicialmente ressecção tumoral à direita, por enucleação, com preservação do parênquima renal, e 3 meses após à esquerda. A função renal pós-operatória se manteve inalterada, e ambos os rins apresentaram uniformidade e progressão do contraste adequados. CONCLUSÃO: Concluímos que os angiomiolipomas maiores que 4cm devem ser tratados cirurgicamente porque têm maior risco de crescimento e hemorragias. As ressecções de tumores menores são mais seguras e têm menor morbidade. A enucleação dos tumores é forma eficaz de ressecção dos mesmos, com preservação de parênquima renal.

OBJECTIVE: To report a case of bilateral giant renal angiomyolipoma associated with tuberous sclerosis, with successful treatment, and to review the literature concerning angiomyolipoma treatment. CASE REPORT: Patient with tuberous sclerosis and angiomyolipoma diagnosed by ultrasonography during her pregnancy. At that time, the angiomyolipoma on the right side was 9 cm in diameter. Conservative management was selected during her pregnancy. The patient returned 7 years later, with a 24.7 x 19.2 x 10.7 cm tumor on the right side and another of 13 x 11.5 x 6.5 cm on the left side, in addition to multiple small angiomyolipomas. A nephron-sparing surgery with tumoral enucleation was performed on the right side, and after 3 months, the tumor on the left side was removed. Renal function in the post-operative period was preserved, and contrast medium progression was uniform and adequate in both kidneys. CONCLUSION: We conclude that an angiomyolipoma larger than 4 cm should be removed surgically, since they have a greater growth rate and pose a risk of hemorrhage. Resection of smaller tumors is safe and has decreased morbidity. Tumoral enucleation is an effective treatment method that preserves kidney function.