Linfoma extranodal de células B em vesícula urinária de um cão / Extranodal B-cell lymphoma in the urinary bladder in a dog

O linfoma é uma neoplasia de alta recorrência na rotina oncológica de medicina veterinária. Pode ser classificado em linfoma Hodgking-liked, com raros casos descritos somente em felinos,e não Hodgking, sendo este segundo o mais comum, subdividido em linfomas B ou T. O objetivo deste trabalho foi relatar a conduta clínica, diagnóstica e terapêutica do caso de uma cadela, de 12 anos, sem raça definida, que manifestava disúria, prostração, dor abdominal e ao exame físico a presença de uma massa na região hipogástrica. Esta foi diagnosticada com linfoma de grandes células por meio de exames de citologia e biópsia, com solicitação do exame de imunoistoquímica que confirmou linfoma difuso de grandes células de imunofenótipo B. Sem o envolvimento de nenhum outro sistema, classificou-se como linfoma primário de bexiga extranodal. O animal passou pelo tratamento quimioterápico realizando nove sessões de quimioterapia pelo protocolo de CHOP, contudo devido ao agravamento do caso a paciente veio a óbito cerca de sete meses após o diagnóstico da doença. O caso estudado foi de extrema importância para a compreensão de linfomas primários de bexiga em razão da escassez de informações relacionadas na literatura. Ainda, o cão é um excelente modelo experimental de linfomas não Hodgking em humanos, consequentemente compreender essa doença em cães promove a evolução conjunta da medicina humana.

Lymphoma is a highly recurrent rate neoplasm in the oncology routine of veterinary medicine. It can be classified into Hodgking-like, rarely described just in felines, and non-Hodgking lymphoma, the latter being the most commun, subdivided into B-cell lymphoma and T-cell lymphoma. The objective of this study was to report the clinical and therapeutic conduct within the diagnosis procedures of a 12-years-old female dog, mixed breed, who manifested dysuria, prostation, abdominal pain and on the physical examination a mass in the hypogastric region was noticed. This was diagnosed as a large cell lymphoma by means cytology and biopsy, also immunohistochemistry was required which confirmed the diffuse large cell lymphoma of immunophenotyping B. Without any other sistem envolved, the neoplasm was classified as primary urinary bladder lymphoma extranodal. The animal underwent chemotherapy, performing nine sessions according to the Madison protocol, however, due to the worsening of the case, the patient died about seven months after the diagnosis of the disease. This case was extremely importante for the understanding of primary urinary bladder lymphomas due to the scarcity of informations in the literature. Also, dog is an excellent experi,emtal model of non Hodgking lymphomas in humans, thus understandig this disease in dogs promotes the joint evolution of human medicine.

A abordagem metabolômica na indicação do tratamento de estenose de junção ureteropélvica em crianças / Metabolomics approach in the treatment of ureteropelvic junction stenosis in children

Estenose de Junção Ureteropélvica (JUP) é uma doença caracterizada pelo bloqueio do fluxo de urina da pelve renal (porção proximal do ureter no rim) ao ureter, tubo que liga o rim à bexiga. Essa formação congênita é uma das maiores causas de dilatação do rim (hidronefrose) em recém-nascidos e, em alguns casos, pode causar danos mais severos ao órgão. A hidronefrose causada pela estenose de JUP pode desaparecer espontaneamente sem perda da função renal, entretanto, é preciso um acompanhamento clínico. Por outro lado, em casos mais severos, onde a dilatação pode causar danos maiores ao rim, um tratamento cirúrgico se faz necessário. Embora existam métodos para o diagnóstico da estenose de JUP, como ultrassonografia, tomografia computadorizada, ressonância e cintilografia, é um grande desafio diferenciar os pacientes que requerem um tratamento cirúrgico e os que necessitam apenas de um acompanhamento convencional. A metabolômica global, que investiga de modo comparativo o conjunto de metabólitos de baixa massa molecular expressos em indivíduos em condições pré-selecionadas, tem o potencial de servir como ferramenta diagnóstica para os pacientes com estenose de JUP e, consequentemente, auxiliar na tomada de decisão entre um acompanhamento clínico ou tratamento cirúrgico. Assim sendo, no presente trabalho, três grupos de pacientes com estenose de JUP, pré-diagnosticados por métodos convencionais, foram investigados sob a perspectiva da metabolômica global, por meio de análises de urina, utilizando cromatografia gasosa e cromatografia líquida, ambas acoplada à espectrometria de massas (GC-MS e RPLC10 MS, respectivamente): pacientes que requerem tratamento cirúrgico (CIR), pacientes que requerem acompanhamento clínico (CLI), e indivíduos sãos (CON). Os resultados mostram que é possível encontrar metabólitos discriminantes entre todas as comparações (CON x CLI, CON x CIR e CLI x CIR); os metabólitos encontrados nas análises multivariada e univariada foram utilizados para construção da curva ROC, para confirmar a possibilidade de utilização desses compostos como biomarcadores. Foram observadas alterações em rotas metabólicas importantes para o bom funcionamento das funções renais, principalmente entre a comparação mais desafiadora (CLI x CIR), como o metabolismo da fenilalanina, da tirosina, da beta-alanina, dos aminoaçúcares e dos nucleotídeos. Há indícios de que o ciclo de Krebs também sofre alteração. Os resultados obtidos podem servir como ponto de partida para uma futura análise alvo e validação biológica

Ureteropelvic junction (UPJ) stenosis is a disease characterized by the interruption of the flow of urine from the renal pelvis (proximal part of ureter in the kidney) to the ureter, tube that links the kidney to bladder. That congenital formation is one of the main causes of kidney dilation (hydronephrosis) in newborns and, sometimes, can cause more serious damage to the organ. The hydronephrosis caused by UPJ stenosis can disappear spontaneously without compromising renal function, although a clinical follow-up is required. On the other hand, in more serious cases, when dilation can induce larger damage to the kidney, surgery intervention is necessary. Although there are methods to diagnose UPJ stenosis, such as ultrasound, computed tomography, resonance and scintigraphy, it is still a great challenge to distinguish patients that require surgery from those whose a clinical follow-up suffices. Global metabolomics, a method that investigates in a comparative manner the set of low molecular mass metabolites expressed by an individual in pre-selected conditions, has the potential to function as a diagnostic tool for patients with UPJ stenosis to support decisions about patient treatment, i.e., surgery versus clinical follow-up. In this work, three groups of UPJ stenosis patients were investigated with the aid of global metabolomics using urine analysis by gas chromatography and liquid chromatography coupled to mass spectrometry (GC-MS and RPLC-MS, respectively): one group consisted of UPJ stenosis patients requiring clinical follow-up (CLI), other group UPJ stenosis patients requiring surgery intervention (CIR) and a third group comprising healthy individuals, the control group (CON).12 The results show that it was possible to find discriminant metabolites among all pairwise comparisons (CON versus CLI, CON versus CIR and CLI versus CIR). The metabolites found by multivariate and univariate analyses were used to build ROC curves, to confirm whether it is possible to use them as biomarkers. Alterations in metabolic pathways that are important for the good maintenance of kidney functions were found, especially in the most challenged comparison (CLI versus CIR), such as the metabolism of phenylalanine, tyrosine, beta-alanine, amino acids and nucleotides. There are evidences that Krebs cycle was also impacted. The results obtained here can serve as a starting point to future targeted analysis and biological validation

The role of bladder diverticula in the prevalence of acute urinary retention in patients with BPH who are candidates to surgery

ABSTRACT Introduction: The urinary bladder diverticula (BD) secondary to benign prostatic hyperplasia (BPH) is a complication that can lead to urinary stasis, stone, urinary tract infection (UTI) and tumors. It's role in acute urinary retention (AUR) is not totally understood. Objectives: To determine the effect of BD size on AUR rates in patients with BPH candidates to surgery. Subjects and Methods: We performed a retrospective cohort study of 47 patients with BPH and BD who underwent BPH surgery associated to complete bladder diverticulectomy from 2006 to 2016. We analyzed risk factors for AUR in patients with BD using univariate, multivariate and correlation analysis. Results: There was a difference in the size of the diverticula, with 6.8 cm vs. 4.5 cm among patients with and without AUR respectively (p=0.005). The ROC curve showed a correlation between the size of BD and the risk of AUR. The value of 5.15 cm presented a sensitivity of 73% and a specificity of 72%. The area under the curve was 0.75 (p=0.01). Comparing groups with BD >5.0 cm vs. ≤5.0 cm, the AUR incidence was 74% and 27.8% respectively with an OR of 2.65 (1.20-5.85) (p=0.005). In the multivariate analysis, only the size of the diverticula reached statistical significance (p=0.012). Conclusions: The diameter of BD is an independent risk factor for AUR in patients with BPH and BD who are candidates to surgery. A diameter greater than 5.15 cm increases the risk of AUR.

Reconstrução tardia da região abdominal inferior e vulvar como tratamento complementar para sequelas pós-correção de extrofia vesical / Delayed reconstruction of the vulvar and lower abdominal regions as a complementary treatment for postsurgical sequelae of bladder exstrophy correction

Patients with malformations of the exstrophy-epispadias complex, including bladder exstrophy, may present for correction of deformities and sequelae in abdominal area, after primary treatment of urogenital malformations, performed early in life and in multiple stages. The secondary correction with aesthetic and functional goals is usually performed after growth and definition of hairy and fat distribution as well as after completion of urological treatment. Psychological aspects should also be considered. We report three female patients, with a history of bladder exstrophy correction in the neonatal period, presenting multiple deformities in the abdominal and vulvar areas, treated at our institution...

Os portadores de malformações do complexo extrofia-epispádia, incluindo a extrofia vesical, podem se apresentar para correções de deformidadese sequelas abdominais após o tratamento primário das malformações urogenitais, realizado nos primeiros anos de vida e em múltiplos estágios. A correção secundária, com objetivos estéticos e funcionais, é normalmente realizada após o crescimento e definição da distribuição pilosae adiposa, bem como após a finalização do tratamento urológico. Os aspectos psicológicos também devem ser considerados. Relatamos uma série de três casos de pacientes do sexo feminino, com antecedente decorreção de extrofia vesical no período neonatal, apresentando múltiplas deformidades na região abdominal e vulvar, submetidas à reconstrução em nosso serviço...

Anomalías nefro-urologicas en el riñón contralateral de pacientes con displasia renal multiquística unilateral / Urological and nephrological abnormalities in the contralateral kidney in patients with unilateral multicystic dysplastic kidney

La displasia renal multiquística unilateral (DRMU) es la enfermedad quística renal más frecuente en pediatría. En estos pacientes, la función renal depende exclusivamente del riñón único funcionante contralateral. El objetivo de este trabajo retrospectivo fue determinar la prevalencia y tipo de anomalías nefro-urológicas en el riñón contralateral funcionante de 103 pacientes con DRMU atendidos en el Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan entre 1995-2008. Se analizaron sólo aquellos niños que tenían ecografía renal y vesical, cistouretrografía miccional (CUGM) y centellograma renal con ácido dimercaptosuccínico (DMSA-Tc99m). Por ecografía se encontraron anomalías en el riñón contralateral funcionante en el 18.5%. Las más frecuentes fueron dilatación de la vía urinaria (6.8%) y aumento de la ecogenicidad (6.8%). La CUGM detectó la presencia de reflujo vesicoureteral en el riñón contralateral en 11 niños (10.7%), la mayoría leve a moderado. El centellograma renal con DMSA-Tc99m demostró escaras en 12 niños (11.6%). Los resultados muestran que el 23.3% de los pacientes con DRMU tienen alguna anomalía nefro-urológica en el riñón único funcionante contralateral.

Divertículo vesical congénito gigante / Giant congenital vesical diverticulum

Los divertículos vesicales congénitos son infrecuentes. En el adulto generalmente se detectan asociados a factores obstructivos a la salida vesical, y se manifiestan por sepsis urinaria recurrente en la mayoría de los casos. Se presenta un paciente del sexo masculino de 67 años de edad, que asistió a consulta por infecciones urinarias bajas a repetición. Al examen físico se constató la presencia de un tumor ovoide de unos 10 cm de diámetro, móvil, no doloroso y con matidez a la percusión, en proyección del hipogastrio y lateralizado hacia la izquierda; la próstata al tacto rectal era de tamaño normal y de consistencia fibroelástica. En el ultrasonido, la uretrocistografía y la tomografía axial computadorizada, se evidenció un divertículo vesical gigante, con su cuello hacia la pared lateral izquierda de la vejiga. Se confirmó mediante la cistoscopia y se procedió a su exéresis por cirugía convencional. El informe histopatológico reveló la existencia de las capas mucosa, muscular y adventicia en su pared, de manera que se corroboró su origen congénito. La evolución del paciente resultó satisfactoria. La exéresis total de los divertículos vesicales congénitos de gran tamaño, constituye la solución definitiva para aquellos pacientes que presentan sepsis urinaria recurrente, en ausencia de factores obstructivos al vaciamiento vesical

Congenital vesical diverticula are infrequent. In adult persons generally are detected in association with obstructive factors for vesical outlet with a recurrent urinary sepsis as manifestation in most cases. This is the case of a male patient aged 67 seen in consultation due to repeated low urinary infections. In physical examination was confirmed the presence of a 10 cm ovoid, mobile and no-painful tumor and with a dull sound to percussion in hypogastrium projection and lateralized to left; at rectal tactus it was of normal size and with a fibroelastic consistency. Ultrasound, urethrocystography and computerized axial tomography evidenced a giant vesical diverticulum with its neck to left lateral wall of bladder confirmed by cystoscopy and exeresis by conventional surgery. Histopathological report showed the presence of mucous and muscular layers, and adventitia in its wall, thus confirming its congenital origin. Patient's course was satisfactory. Total exeresis of big congenital vesical diverticula is the definite solution for those patients presenting with recurrent urinary sepsis in the absence of obstructive factors to vesical voiding

True duplicate bladder exstrophy: A rare lesion.

A two-day-old newborn male child with a rare variant of exstrophy bladder was managed in our institute. The child has true duplicate bladder exstrophy which is extremely rare and only 8 cases reported in the world literature so far. We describe another one and briefly review the literature.

Deben realizarse las ecografías renal y vesical en pacientes con mamelones preauriculares? / Should be the kidney and bladder ultrasound in patients with preauricular tags?

Introducción: Los niños con malformaciones menores del pabellón auricular son estudiados habitualmente con ecografías renal y vesical, existiendo controversias en cuanto a la validez de esta práctica. Objetivo: Determinar si la prevalencia de malformaciones del tracto urinario es mayor entre niños con mamelones preauriculares aislados que entre aquellos que no los presentan. Población, material y métodos: Entre junio de 1996 y junio de 2006, 92 niños con mamelones preauriculares fueron estudiados con ecografía renal y vesical. Se incluyeron en la evaluación a niños sanos con mamelón preauricular aislado. Se excluyeron aquellos con hoyuelos preauricular , una o más malformaciones menores asociadas, síndromes de malformaciones congénitas múltiples o anomalías mayores identificadas previamente. Ochenta y ocho niños cumplieron con los criterios de inclusión. Para la comparación estadística se utilizó un grupo control histórico de 300 lactantes sanos sin mamelón, estudiados en forma no concurrente con el grupo estudio y que mostró una prevalencia del 1% de malformaciones urinarias. Se realizó una prueba de hipótesis exacta para una proporción utilizando un nivel de significación del 5%, con el software Epidat 3.1. Resultados: Tres de los 88 niños (3,4%) con malformaciones preauriculares aislados presentaron malformaciones renales: ectopia renal derecha e hidroureteronefrosis detectada prenatalmente; agenesia renal izquierda, existiendo antecedentes familiares de malformaciones renales y un quiste cortical simple. No se detectaron diferencias estadísticamente significativas con el grupo considerado control histórico (p=0.11). Conclusión: La prevalencia de malformaciones renales similar entre los niños sanos con y sin mamelones preauriculares aislados, por lo que la indicación rutinaria de ecografías renal y vesical no sería necesaria.

Introduction: The renal and vesical ultrasound in every patient with preauricular tags is a common practice.However, the benefit of this indication is controversial. Objective: To determine whether the prevalence of urinarytract abnormalities in patients with isolated preauricular tags is higher than in those without preauricular tags.Population, material and methods: From June 1996 to June 2006, 92 patients with preauricular tags had beenreferred consecutively for renal and vesical ultrasound. Healthy infants with isolated preauricular tags were included.Patients who had a preauricular tag associated with other ear anomalies and/ or major anomalies or malformative syndromes were excluded. Eighty eight infants achieved the inclusion criteria. A historical control group of 300 healthy infants, with a prevalence of urinary tract abnormalities of 1%, was considered for a statistical comparison. An exacthypothesis test for one proportion was performed, with the software Epidat 3.1, setting a signification level of 5%.Results: Three out of 88 infants (3.4%) with isolated preauricular tags had renal abnormalities: ectopic kidney withhidroureteronephrosis detected by prenatal ultrasound, solitary kidney with a positive family history of renal malformations and a simple cortical renal cyst. No significant statisticaldifference was found in comparison with the historical control group (p: 0.11). Conclusion: The prevalence of renal malformations was similar in the groups with and without preauricular tags. Renal and vesical ultrasounds seem not to be mandatory in infants with isolated preauricular tags.

[Seven-year tolerance of intrauterine device in bladder]

Spontaneous migration of an intrauterine device into the bladder is very rare. Foreign bodies of the urinary bladder may occur by self-insertion or migration from neighbor organs. When ignored for a long time, foreign bodies act as a core for calculus formation. The patient usually present with dysuria and intermittent urinary tract infections. A 42-year-old woman is reported in whom an intrauterine device had been placed 7 years before this report, and complained of chronic pelvic pain, recurrent urinary tract infections and irritative urinany tract symptoms. After observation of stone in the bladder, the intrauterine device and the surrounded stone was removed by suprapubic cystolithotomy

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): a case report in Thailand.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disease with high mortality rate. The authors report a case of a female term infant with massive abdominal distension at birth. Abdominal ultrasonography revealed a huge cystic mass resolved after urinary catheterization. Exploratory laparotomy was performed and intraoperative findings were an enlarged urinary bladder, microcolon, short bowel and malrotation of the small intestine. Ladd's procedure, ileostomy and vesicostomy were performed. Pathological findings of rectal biopsy revealed normal ganglion cells. Although prokinetic drugs were given for promoting bowel motility, enteral feedings were not tolerated. She died from septicemia at the age of 50 days.

Megavejiga fetal en el segundo trimestre: síndrome de Berdon- un signo fácil, un diagnóstico difícil / Fetal megabladder in the second trimester: Berdon syndrome a simple sign, a difficult diagnosis

El diagnóstico de megavejiga en el embarazo es infrecuente más aun si se trata de un feto femenino. Este hecho nos permite delimitar bastante las alternativas diagnósticas, dentro de un espectro bastante heterogénico. El síndrome Megavejiga-Microcolon Hipoperistalsis Intestinal(MMIHS) es una patología de muy mal pronóstico que asocia megavejiga y alteraciones intestinales severas. Es por esto que es importante tenerla presente, para poder dar a los padres una información fidedigna y certera en cuanto al pronóstico. Presentamos 2 casos de nuestro servicio en el período 1998-2003. Se comentan los aspectos diagnósticos, terapéuticos y la evolución.

Unilateral renal agenesis associated with urinary outflow tract obstruction in a diabetic pregnancy.

We report a case of diabetic pregnancy where the foetus had enlarged cystic left kidney with agenesis of right kidney. The urinary bladder was dilated and urethra revealed posterior urethral valve an postmortem.

Alteraciones de las extremidades inferiores en el síndrome de Prune-Belly / Alterations of the lower extremities in the Prune-Belly syndrome

El síndrome de Prune-Belly es una rara condición congénita de causa desconocida, que fue descrita por primera vez en 1839. Tiene una prevalencia de 20:1 en hombres versus mujeres, situación probablemente debida a un defecto superficial en el cromosoma X. Se caracteriza por: 1) ausencia de la pared abdominal en forma completa o parcial. 2) criptorquidia y 3) anomalías del tracto urinario. A veces, también está asociado a algunas anomalías ortopédicas debidas a la presencia de oligohidroamnios, pero raramente ocurren anormalidades de las extremidades inferiores. El pronóstico es malo sólo un 20 por ciento sobreviven al parto y un 50 por ciento muere dentro de los primeros 5 años de vida principalmente debido a las consecuencias de falla renal.Reportamos el caso de un niño con síndrome de Prune-Belly con una amputación congénita de una de sus extremidades inferiores que fue manejada con punción vesical en el período antenatal.

Diagnóstico precoz de malformaciones fetales: revisión de tres casos clínicos / Precocious diagnosis of fetal malformations: review of three clinical cases

Presentamos nuestra experiencia en el hallazgo de casos de patología fetal en forma muy precoz, en base a un programa efectivo de trabajo enfocado a la realización de ultrasonido a las 12 semanas de gestación, en búsqueda, entre otros, de marcadores de aneuploidías.

Agenesia renal bilateral (Síndrome de Potter) en hija de madre hipertiroidea que recibió metimazol durante el embarazo temprano / Bilateral renal agenesis (Potter symdrome) in a newborn of a hyperthyroid woman receiving methimazole during early pregnancy

Se presenta el caso clínico de una mujer hipetiroidea que recibió metimazol durante el embarazo temprano y que tuvo una niña con agenesia renal bilateral. El dato clínico inicial fue la presencia de oligohidramnios detectado por ultrasonido (US) a las 19 semanas de gestación. Otro US al término de la gestación demostró anhidramnios, ausencia de siluetas renales y de vejiga, que se corroboró al nacer la niña, la cual además presentaba facies de Potter, evolucionó rápidamente con anuria, edema generalizado, insuficiencia renal, insuficiencia respiratoria y sangrado de tubo digestivo alto, falleció a los dos días de edad. Existe evidencia suficiente de que el metimazol administrado durante el embarazo temprano puede ocasionar diversas malformaciones congénitas incluyendo el síndrome de Potter, aunque en esta última malformación la evidencia aún es débil

Diagnóstico antenatal de obstrucción urinaria en el primer trimestre del embarazo / Prenatal diagnosis of urinary obstruction in first trimestry of pregnancy

Se comunica cuatro casos de obstrucción urinaria baja con megavejiga fetal diagnosticados en el primer trimestre de la gestación. Se presenta el diagnóstico prenatal, su manejo y resultado perinatal

Cloacal exstrophy: experience at King Abdulaziz University Hospital, Jeddah, Saudi Arabia

Cloacal exstrophy is one of the complex anomalies which challenge the pediatric surgeons infrequently. At King Abdulaziz University Hospital, Jeddah, we had the experience of managing four cases. The reconstruction was achieved in stages and involved gastrointestinal, urological, genital. and musculoskeletal systems. All the managed cases were raised as their original genotype. A fifth case died before any surgical intervention, due to a major cardiac congenital anomaly. The aim of the present study is to report such rare anomalies and to present our experience in their management