Calcificación miocárdica de etiología multifactorial / Myocardial calcification with multifactorial etiology

En el presente trabajo se describe un caso de muerte súbita en una mujer de 70 años que fallece 20 días después de ser dada de alta tras ingreso por infección urinaria secundaria a litiasis renal complicada con shock séptico e insuficiencia renal y respiratoria. En el corazón se identificó una cardiopatía isquémica crónica y extensas áreas de miocardio calcificado. Además, se exponen los tipos de calcificaciones del miocardio existentes y sus causas; y se analizan aquellos factores que en el caso presentado han podido causar la calcificación del miocardio(AU)

In the present report, we describe a case of sudden death in a 70-year-old woman who died 20 days after been discharged after admission for urinary infection secondary to renal lithiasis complicated with septic shock and renal and respiratory failure. It has been identified chronic ischemic heart disease and extensive areas of calcified myocardium. Also, we review myocardial calfications subtypes and its causes, and we analyze the specifics of the presented case(AU)

Nefrolitiasis en la paciente gestante: revisión de la literatura / Renal lithiasis in pregnant patient: a literary review

Resumen La litiasis renal en la paciente gestante es una condición relativamente infrecuente; sin embargo, es la causa más común de dolor no obstétrico durante el embarazo. En el periodo de gestación se producen diversos cambios anatomofisiológicos en el tracto urinario, como la dilatación de los cálices renales, la pelvis renal y los uréteres por causa del efecto que ejerce la progesterona sobre el músculo liso uretral, y la compresión de los uréteres por el útero grávido. Estas modificaciones conducen a un aumento del flujo plasmático renal y de la tasa de filtrado glomerular, ocasionando hiperuricosuria e hipercalciuria. Del mismo modo, durante el embarazo también se produce un aumento de la secreción de inhibidores de cálculos, por lo cual la prevalencia de la formación de cálculos durante el embarazo es similar a la de las mujeres no embarazadas. El bajo índice de sospecha por parte del médico tratante puede entorpecer el diagnóstico y el tratamiento de esta patología, que también son limitados en la gestación debido a los potenciales riesgos teratogénicos. Se realiza una revisión narrativa de la literatura partiendo de la evidencia científica disponible en las diferentes bases de datos y de esta manera se pretende instruir al médico en los aspectos clave de dicho tema.

Abstract Renal lithiasis in pregnant women is a relatively rare condition. However, it is the most common cause of non-obstetric pain during pregnancy. During the gestation period, various anatomical-physiological changes occur in the urinary tract. These changes include dilation of the renal calyces, renal pelvis, and ureters due to the effect of progesterone on urethral smooth muscle and compression of the ureters by the gravid uterus. These modifications lead to an increase in renal plasma flow and glomerular filtration rate, thus causing hyperuricosuria and hypercalciuria. Similarly, during pregnancy there is also an increase in the secretion of stone inhibitors, therefore, the prevalence of stone formation during pregnancy is similar to non-pregnant women. The low index of suspicion on the part of the treating physician can hinder the diagnosis and treatment of this pathology, which is also limited in pregnancy due to teratogenic risks. A narrative review of the literature is carried out based on the scientific evidence available in the different databases and in this way it is intended to instruct the doctor in the key aspects of said topic.

Absceso cerebral por Cladophialophora bantiana en un paciente con trasplante renal: reporte de un caso / Brain abscess caused by Cladophialophora bantiana after renal allograft loss: A case report

Resumen Las feohifomicosis cerebrales son infecciones graves causadas por mohos dematiáceos, entre los cuales Cladophialophora bantiana es una de las especies más comúnmente aislada. Esta tiene tropismo por el sistema nervioso central y frecuentemente produce abscesos cerebrales en pacientes inmunocompetentes; además, en los inmunocomprometidos también puede ocasionar infección diseminada. Pese a la disponibilidad de medicamentos antifúngicos de amplio espectro, a menudo se requiere también la intervención quirúrgica; de todas maneras, la mortalidad es elevada. El diagnóstico debe hacerse interviniendo para tomar la muestra y hacer el cultivo y las pruebas de sensibilidad. Se presenta aquí el caso de un paciente con trasplante renal que presentó un absceso cerebral por C. bantiana, el cual se extrajo mediante resección quirúrgica. El paciente recibió tratamiento con voriconazol, con adecuada respuesta, mejoría y sin secuelas neurológicas.

Abstract Cerebral feohifomycosis are severe infections caused by dematiaceous fungi. Cladophialophora bantiana is one of the most commonly isolated species; it has central nervous system tropism and it often manifests as a brain abscess in immunocompetent patients. In immunocompromised patients, it can lead to brain abscesses and disseminated infections. Despite the availability of broad-spectrum antifungal drugs, it is a must to perform surgical management, in addition to drug therapy. However, mortality is high. The diagnostic approach must be invasive to establish a timely diagnosis and direct treatment based on culture and susceptibility tests. We report a case of brain abscess caused by C. bantiana in an immunosuppressed patient who was treated with surgical resection and voriconazole with an adequate response to therapy and without neurological sequels.

Riñón en esponja y litiasis renal / Sponge kidney and renal lithiasis

Introducción: El riñón en esponja es una malformación renal, de los túbulos colectores, que se suele asociar a nefrocalcinosis o acidosis tubular distal. La asociación con la litiasis renal se observa entre un 4-20%. Objetivo: El objetivo de nuestro trabajo fue describir factores de riesgo bioquímicos para litiasis renales, en pacientes con riñón en esponja. Material y métodos: Se realizó un estudio retrospectivo, observacional de corte, entre los años 2000 y 2017 en donde se estudiaron 37 pacientes con riñón en esponja y litiasis renal (26 mujeres y 11 hombres) de 37.3 ± 13.2 años. El diagnóstico de riñón en esponja se hizo mediante urograma excretor. Resultados: Fue observada nefrocalcinosis en el 95%. El diagnóstico bioquímico más frecuente fue la hipercalciuria idiopática que como única y asociada alteración se observó en el 59.4%. La hiperuricosuria fue el segundo diagnóstico encontrado en el 32.4% (única y asociada) seguido por la, hipocitraturia, hipomagnesuria y el pHu persistentemente ácido. En los hombres llamó la atención que un 46.2% no presentaron alteración bioquímica. Conclusiones: En conclusión se destaca la asociación relativamente frecuente de riñón en esponja y litiasis renal. La hipercalciuria idiopática fue la alteración metabólica más frecuente como causa de litogénesis, seguida por la hiperuricosuria, similar a lo descripto en la literatura, aunque en menor proporción. Otras alteraciones, como la hipocitraturia, hipomagnesuria y pHu persistentemente ácido también deben ser consideradas en el estudio de estos pacientes.

Introduction: Sponge kidney is a renal malformation of the collecting tubules, which is usually associated with nephrocalcinosis or distal tubular acidosis. The association with renal lithiasis is observed between 4-20%. Objective: The aim of our work was to describe the biochemical risk factors of renal lithiasis in patients with sponge kidney. Methods: A retrospective, observational, cross-sectional study was conducted between 2000 and 2017, in which 37 patients with sponge kidney and renal lithiasis (26 women and 11 men), aged 37.3 ± 13.2 years, were studied. The diagnosis of sponge kidney was made by excretory urography. Results: Nephrocalcinosis was observed in 95% of patients. The most frequent biochemical diagnosis was idiopathic hypercalciuria, which was observed, as a single and associated alteration, in 59.4% of cases. Hyperuricosuria was the second diagnosis found in 32.4% (single and associated) followed by hypocitraturia, hypomagnesuria and persistently acid pHu. Surprisingly, 46.2% of men presented no biochemical alteration. Conclusions: Our findings highlight the relatively frequent association of sponge kidney and renal lithiasis. Idiopathic hypercalciuria was the most common metabolic alteration as the cause of lithogenesis, followed by hyperuricosuria, similar to that described in the literature, but to a lesser extent. Other alterations, such as hypocitraturia, hypomagnesuria and persistently acid pHu, should also be considered in the study of these patients.

Litiasis renal en Paraguay: herramientas laboratoriales en la identificación de etiología y seguimiento del paciente litiásico / Nephrolithiasis in Paraguay: Laboratory tools in the identification of etiology and follow-up of the lithiasic patient

La litiasis renal es una patología que se caracteriza por la formación, agregación y retención de cristales en las vías urinarias. Su etiología es compleja ya que la misma puede ser el resultado de la interacción de múltiples factores tanto endógeno, metabólico como ambiental. La elevada tasa de recurrencia de la formación de cálculos puede superar el 40% en un periodo de 5 años tras el primer episodio y puede conllevar consecuencias graves para el funcionamiento renal con su consiguiente impacto en la calidad de vida del paciente(AU)

Reporte de un caso de Cistinuria: Uso de herramientas de diagnóstico a nivel nacional / Report of a case of cystinuria: Use of diagnostic tools at national level

La cistinuria es una enfermedad genética que se engloba dentro de alteraciones congénitas del transporte de aminoácidos con formación de cálculos en las vías urinarias, si bien es poco frecuente se caracteriza por su elevada recurrencia. En este trabajo presentamos el caso de una paciente de 34 años, con antecedentes de haber perdido un riñón por episodios anteriores de litiasis y con múltiples recidivas que es diagnosticada mediante la detección de cistina por espectroscopía infrarroja como componente único de 96 fragmentos de cálculos removidos mediante nefrolitotomía percutánea. La paciente fue evaluada laboratorialmente mediante el perfil metabólico y la cristaluria. Las indicaciones de tratamiento específicas incluyeron la administración de agentes alcalinizantes, régimen nutricional, y entrenamiento para control de pH urinario. Es importante señalar la agresividad de la litiasis de cistina con las consecuencias que puede tener la calidad de vida del paciente, y por tanto la importancia de contar con capacidades instaladas a nivel país para el diagnóstico y seguimiento de litiasis genéticas como la causada por la cistinuria(AU)

Cystinuria is a genetic disease that is included among congenital defects of renal amino acids transport that causes urinary stone formation. Although it is rare, it is characterized by its high recurrence. We present the case of a 34-year-old patient that lost one of her kidney because of recurrent episodes of lithiasis, and that was diagnosed by the detection of cystine with infrared spectroscopy as the sole component of 96 stone fragments removed by percutaneous nephrolithotomy. The patient was evaluated by metabolic profile and crystalluria. The specific treatment indications included the administration of alkalinizing agents, nutritional regimen, and training for personal measurement of urinary pH. This case highlights the aggressiveness of cystine stones with the consequences that may have on the quality of the patient life, and therefore the importance of having installed proper diagnostic capacities at national level to detect and monitor treatment efficacy in genetic lithiasis such as cystinuria(AU)

Una nueva mutación de la enfermedad de Dent en un niño de 11 años con nefrolitiasis y nefrocalcinosis / A novel mutation of Dent's disease in an 11-year-old male with nephrolithiasis and nephrocalcinosis

La enfermedad de Dent es una tubulopatía recesiva ligada al cromosoma X caracterizada por proteinuria de bajo peso molecular (bpm), hipercalciuria, nefrocalcinosis o nefrolitiasis, disfunción tubular proximal e insuficiencia renal en la adultez. Las mujeres son portadoras y, en general, padecen una forma leve de la enfermedad. La progresión hacia la insuficiencia renal en estadio terminal se da entre los 30 y los 50 años de edad en el 30-80% de los varones afectados. A falta de un tratamiento dirigido al defecto molecular, en la actualidad, los pacientes con enfermedad de Dent reciben tratamientos complementarios orientados a prevenir la nefrolitiasis y la nefrocalcinosis. El caso que presentamos es el de un niño de 11 años con nefrocalcinosis y nefrolitiasis, en quien se detectó una nueva mutación en el gen CLCN5.

Dent's disease is a rare X-linked recessive tubulopathy characterized by low molecular weight (LMW) proteinuria, hypercalciuria, nephrolcalcinosis or nephrolithiasis, proximal tubular dysfunction and renal failure in adulthood. Females are carriers and usually mildly affected. Progression to endstage renal failure are at the 3rd-5th decades of life in 30-80% of affected males. In the absence of therapy targeting for the molecular defect, the current care of patients with Dent's disease is supportive, focusing on the prevention of nephrolithiasis and nephrocalcinosis. We present an 11-year-old child with nephrocalcinosis and nephrolithiasis caused by a new mutation at CLCN5 gene.

Obesity and kidney disease: hidden consequences of the epidemic / Obesidad y enfermedad renal: las consecuencias ocultas de una epidemia

Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for Chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset Chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing Chronic kidney disease in the long-term. The incidence of obesity-related glomerulopathy has increased ten-fold in recent years. Obesity has also been shown to be a risk factor for nephrolithiasis, and for a number of malignancies including kidney cancer. This year the World Kidney Day promotes education on the harmful consequences of obesity and its association with kidney disease, advocating healthy lifestyle and health policy measures that makes preventive behaviors an affordable option.

La obesidad se ha convertido en una epidemia mundial, y se ha proyectado que su prevalencia se incrementrá en 40% en la próxima década. Esta creciente prevalencia supone implicaciones tanto para el riesgo de desarrollo de diabetes mellitus y enfermedades cardiovasculares como para el desarrollo de Enfermedad renal crónica. Un elevado índice de masa corporal es uno de los factores de riesgo más importantes para el desarrollo de enfermedad renal crónica. En individuos afectados por la obesidad, tiene lugar una hiperfiltración compensatoria necesaria para alcanzar la alta demanda metabólica secundaria al aumento del peso corporal. El incremento de la presión intraglomerular puede generar daño renal y elevar el riesgo de desarrollar enfermedad renal crónica a largo plazo. La incidencia de glomerulopatía asociada a obesidad se ha incrementado 10 veces en los últimos años. Asimismo se ha demostrado que la obesidad es un factor de riesgo para el desarrollo de nefrolitiasis y un número de neoplasias, incluyendo cáncer renal. Este año, el Día Mundial del Riñón promueve la educación acerca de las consecuencias nocivas de la obesidad y su asociación con la enfermedad renal, abogando por un estilo de vida saludable y la implementación de políticas públicas de salud que promuevan medidas preventivas alcanzables.

Estudo da ocorrência de litíase renal e ureteral em gatos com doença renal crônica / Study of nephrolithiasis and ureterolithiasis in cats with chronic kidney disease

Investigou-se a ocorrência de nefrolitíase e/ou ureterolitíase em 72 gatos portadores de doença renal crônica (DRC), classificados predominantemente no estágio II, segundo os critérios designados pela IRIS - International Renal Interest Society. Destes pacientes, 47 (65,27por cento) apresentaram litíase renal e ou ureteral. Não houve diferença estatística entre o grupo de estudo (DRC com cálculo) e o grupo controle (DRC sem cálculo) em relação à idade (p=0,274). Apesar disso, os pacientes portadores de nefrolitíase e/ou ureterolitíase apresentaram maiores indícios de lesão renal, caracterizados por diferenças estatisticamente relevantes da densidade urinária (p=0,013) e pelo menor tamanho dos rins direito (p=0,009) e esquerdo (p=0,048). Encontrou-se similaridade entre os grupos em relação a outros parâmetros, tais como as concentrações plasmáticas de cálcio total, cálcio ionizado, fósforo, sódio, potássio e paratormônio intacto (PTHi). Os valores das concentrações séricas de ureia e bicarbonato diferiram entre os grupos, com valores de p=0,039 e p=0,037, respectivamente. Além disso, foi mensurada a pressão arterial, que se manteve inalterada na comparação entre o grupo de estudo e o grupo controle. Os resultados obtidos reforçam a necessidade de acompanhamento ultrassonográfico de todos os pacientes portadores de DRC, mesmo daqueles assintomáticos ou em estágios iniciais da doença.

Nephrolithiasis and/or ureterolithiasis were investigated by means of ultrasonography in 72 cats with chronic kidney disease (CKD), predominantly classified in stage II, according to IRIS - International Renal Interest Society criteria. Of these patients, 47 (65.27 percent) had nephrolithiasis and/or ureterolithiasis. There was no statistical difference between the study group (CKD with calculi) and control group (CKD without calculi) regarding age (p=0.274). Nevertheless, patients with nephrolithiasis and/or ureterolithiasis had greater evidence of renal injury, characterized by statistically significant differences in the urinary density (p=0.013) and the smaller size of the right kidney (p=0.009) and left kidney (p=0.048), measured in the longitudinal plane. There were no difference between groups in the other parameters investigated such as plasmatic total calcium, ionized calcium, phosphorus, sodium, potassium and intact parathyroid hormone concentrations. The values of serum urea and bicarbonate differ between groups with p=0.039 and p=0.037, respectively. Furthermore, arterial blood pressure was measured, remaining unchanged between the groups. One can conclude that nephrolithiasis and/or ureterolithiasis are common findings in cats with CKD and these results reinforce the need to perform image investigation in cats with CKD even in the asymptomatic ones, or those in the early stages of the disease.

Metabolic and endocrinological factors related to nephrolithiasis pre and post multiple techniques of bariatric surgery: a sistematic review / Fatores endócrinos e metabólicos relacionados à nefrolitíase pré e pós técnicas diversas de cirurgia bariátrica: uma revisão sistemática

INTRODUCTION: Despite of benefits of bariatric surgery for obesity treatment, the procedure may be related to some complications. AIM: Analyze studies to address the relation between nephrolithiasis and bariatric surgery. METHODS: Ten papers about this theme were selected from 2005-2013 in Pubmed, describing the relation of nephrolithiasis or their risk factors with several types of bariatric surgery. RESULTS: Retrospective studies with minimal follow-up of three years demonstrated 7,65% in surgery patients and 4,63% non-surgery with nephrolithiasis (p<0,05). Prospective studies (8 of 10) revealed large percentage of calculi appearing and significant increase in oxaluria. CONCLUSION: There is correlation between obesity surgery and nephrolithiasis. .

INTRODUÇÃO: Apesar dos benefícios das operações bariátricas para o tratamento da obesidade, elas podem acompanhar algumas complicações. OBJETIVO: Analisar estudos que enfoquem a relação entre nefrolitíase e procedimentos cirúrgicos bariátricos. MÉTODOS: Foram selecionados 10 estudos de 2005 a 2013 no PubMed que descreviam a relação de nefrolitíase ou seus fatores de risco com diversas técnicas de cirurgia bariátrica. RESULTADOS: Estudos retrospectivos, com seguimento por no mínimo três anos, demonstraram que 7,65% dos pacientes operados e 4,63% dos não operados apresentaram nefrolitíase (p<0,05). Estudos prospectivos (8 dos 10) verificaram grande porcentagem de aparecimento de nefrolitíase ou aumento significativo na oxalúria. CONCLUSÃO: Os estudos mostraram que há relação entre operações para obesidade e nefrolitíase. .

Nefrolitiasis: fisiopatología, evaluación metabólica y manejo terapéutico / Nephrolithiasis: pathofisiology, metabolic evaluation, and therapy

La nefrolitiasis es un trastorno frecuente en países desarrollados y en desarrollo. Su prevalencia varía entre el 4 y 20% según diferentes series y depende de la edad de la población analizada, las condiciones geográficas y socioeconómicas del grupo en estudio. La prevalencia de litiasis renal aumenta con la edad tanto en varones como en mujeres; es más común en varones jóvenes. Una de las características de la litiasis renal es la alta recurrencia y muchas veces la solución de los cólicos requiere de intervenciones endoscópicas u otro tratamiento urológico y esto hace a la morbilidad de la enfermedad. El promedio de diferentes trabajos que estudiaron la evolución natural de la enfermedad muestra que la posibilidad de recurrencia al año del primer episodio es del 15%, a los 5 años 40% y a los 10 años del 60%. Tanto los factores genéticos como los medioambientales contribuyen a la formación de cálculos. Los factores genéticos explican la tendencia a la agregación familiar de la enfermedad. El rol de la herencia es claro en algunas enfermedades como cistinuria o hiperoxalurias primarias, pero la litiasis idiopática también tiene una tendencia familiar, si bien los genes involucrados aún no se conocen. Dentro de los factores medioambientales se destaca la dieta, o sea determinados hábitos de ingesta que expresan la propensión a la litogénesis que tienen algunos sujetos. En aproximadamente 90% de los afectados es posible identificar alteraciones metabólicas que ayudan no sólo al diagnóstico etiológico sino que permiten también un manejo adecuado, con modificaciones dietéticas e intervenciones farmacológicas específicas. El tratamiento es eficaz en disminuir significativamente la tasa de recurrencias. En esta revisión analizamos la fisiopatología de la hipercalciuria, la hiperoxaluria, la hipocitraturia, y las litiasis úrica y cistínica. Se detallan el manejo del cólico renal y el tratamiento dietético y farmacológico apropiado para cada tipo de litiasis.

Nefrolitíase em pacientes pediátricos: investigação metabólica e anatômica / Nephrolithiasis in pediatric patients: metabolic and anatomical investigation

Desordens metabólicas são frequentes em pacientes com nefrolitíase pediátrica. OBJETIVOS: Estudar as alterações metabólicas e anatômicas e a análise química dos cálculos encontrados em pacientes da nossa região. MÉTODOS: Este é um estudo retrospectivo em 158 crianças com evidência de formação recente de cálculos, destes apenas 109 concluíram a investigação metabólica. A investigação laboratorial consistiu de duas amostras de urina de 24 horas com dosagem de cálcio, ácido úrico, citrato, oxalato, sódio e creatinina, cistinúria qualitativa, pH urinário seguido de 12 horas de jejum e restrição hídrica, cultura da urina e análise química quando os cálculos foram disponíveis. As técnicas de imagem incluíram ultrassonografia do trato urinário e urografia excretora. RESULTADOS: Em 96,3 por cento das crianças alguma causa foi detectada. A principal alteração metabólica encontrada foi a hipercalciúria (73,4 por cento). Análise química dos cálculos mostrou oxalato de cálcio em 90,9 por cento dos casos. Alterações anatômicas foram encontradas em 18,0 por cento dos pacientes investigados, e a mais frequente foi a duplicação pieloureteral (28,6 por cento). CONCLUSÕES:Hipercalciúria foi a desordem mais encontrada, a alteração anatômica mais comum foi a duplicação pieloureteral e oxalato de cálcio foi o constituinte químico mais frequente. Este trabalho serviu para o conhecimento das características dos pacientes pediátricos portadores de nefrolitíase em nossa região.

Metabolic disorders are frequently observed in pediatric patients with renal lithiasis. OBJECTIVES: Study the metabolic and anatomical alterations and perform the chemical analysis of stones found in children with nephrolithiasis in our region. METHODS: A retrospective study on 158 children with evidence of recent renal stone formation was performed. One hundred and nine children concluded the metabolic study. Laboratory investigation consisted in two samples of 24-hour urine for calcium, uric acid, citrate, oxalate, sodium and creatinine; qualitative cystinuria, urinary pH following 12-hour fasting and water restriction, urine culture and chemical analysis when the stones were available. Renal imaging techniques included, at least, renal ultrasound and excretory urogram. RESULTS: A cause for nephrolithiasis was identified in 96.3 percent of children. The main metabolic alteration was hypercalciuria (73.4 percent). Chemical analysis of stones showed calcium oxalate in 90.9 percent of the cases. Anatomical alterations were found in 18.0 percent of the investigated cases and the most frequently found alteration was pyelo-ureteral duplication (28.6 percent). CONCLUSIONS: Hypercalciuria was the most frequently found disorder and pyelo-ureteral duplication was the most common anatomical alteration; moreover, calcium oxalate was the most frequent chemical constituent. The present study showed the characteristics of pediatric patients with nephrolithiasis in our region.

Alterações anatômicas em pacientes com nefrolitíase / Anatomical alterations in patients with nephrolithiasis

Introdução: Nefrolitíase é uma doença multifatorial e tem relação com desordens genéticas e fatores ambientais. Cálculos renais são mais comuns em adultos e são associados com várias desordens metabólicas e anatômicas. As principais anormalidades anatômicas como obstrução da junção ureteropélvica, rim em ferradura, ureter duplicado completa ou incompletamente, pelve bífida e rim esponja medular são conhecidas como responsáveis pela formação dos cálculos. O objetivo deste estudo é avaliar alterações anatômicas em pacientes com nefrolitíase em nossa região. Métodos: estudo retrospectivo em 1.378 pacientes com evidência de formação recente de cálculos renais. investigação laboratorial e análise disponibilidade. Técnicas de imagens renais incluíram pelo menos ultrassonografia renal e urografia excretora. Resultados: 1.378 pacientes com nefrolitíase foram atendidos, dentre os quais somente 367 (26,5%) foram submetidos à investigação anatômica e 132 (36,0%) tiveram pelo menos uma alteração anatômica. a idade média dos pacientes investigados foi de 36,8 +- 4,3 anos e 198 (54,5%) eram do sexo feminino. As alterações anatômicas mais frequentemente encontradas foram cisto renal, ureter duplicado completa ou incompletamente e obstrução da junção ureteropélvica. Conclusões: Alterações anatômicas foram encontradas em 36% dos pacientes investigados. Cisto renal, duplicação ureteral e obstrução da junção ureteropélvica foram alterações anatômicas mais comuns neste grupo.

Introduction: Nephrolithiasis is a multifactorial disease and is related to genetic disorders and environmental factors. Gallstones are more common in adults and are associated with various anatomical and metabolic disorders. The major anatomical abnormalities such as ureteropelvic junction obstruction, horseshoe kidney, ureter duplicated completely or incompletely, pelvis bifida and medullary sponge kidney are known to be responsible for gallstone formation. The aim of this study is to assess anatomical changes in patients with nephrolithiasis in our region. Methods: A retrospective study of 1378 patients with evidence of recent formation of kidney stones. laboratory research and analysis available. Imaging techniques included renal ultrasonography at least kidney and excretory urography. Results: 1378 patients with nephrolithiasis were treated, of whom only 367 (26.5%) underwent anatomical study and 132 (36.0%) had at least one anatomic change. the average age of patients studied was 36.8 + - 4.3 years and 198 (54.5%) were female. Anatomical abnormalities were most frequently encountered renal cyst, completely or incompletely duplicated ureter and ureteropelvic junction obstruction. Conclusions: Anatomical changes were found in 36% of the cases. Renal cyst, ureteral duplication and ureteropelvic junction obstruction were the most common anatomical changes in this group.

Análise química do cálculo em pacientes portadores de litíase do trato urinário / Chemical analysis of stone in patients with nephrolithiasis

Introdução: Nefrolitíase é uma doença multifatorial que se relaciona com desordens genéticas e fatores ambientais. Cálculos são mais comuns em adultos e são associados com diversas desordens metabólicas. Os cálculos de oxalato de cálcio são os mais comuns. O objetivo deste estudo é realizar a análise química dos cálculos urinários em nossa região. Materiais e Métodos: Realizamos um estudo retrospectivo em 1.342 pacientes com evidência recente de formação de litíase urinária. A investigação laboratorial consistiu da análise química dos cálculos que estiveram disponíveis. Resultados: Foram atendidos 1.342 pacientes com nefrolitíase, sendo que somente 109 (8,1%) foram submetidos à análise química do cálculo. Nestes, a idade média era de 38,9 ± 13,4 anos, sendo 55 (50,5%) do sexo masculino. História familiar positiva ocorreu em 65% dos casos. Cálculos de oxalato de cálcio foram encontrados em 87% dos casos analisados. Hipercalciúria foi o distúrbio metabólico mais encontrado nos pacientes com cálculos de oxalato de cálcio (60%). Conclusões: A análise química do cálculo mostrou que o oxalato de cálcio é o constituinte mais encontrado em nossa região, e estes dados estão de acordo com a literatura.

Introduction: Nephrolithiasis is a multifactorial disease and it has relation with genetic disorders and environmental factors. Stones are most common in adults and are associated with several metabolic disorders. Calcium oxalate is the most common type of stone. The objective of this study is to evaluate chemical analysis of calculi in our region. Methods: We made a retrospective study on 1,342 patients with evidence of recent formation of renal stones. Laboratory investigation included chemical analysis when the stones were available. Results: 1,342 patients with nephrolithiasis were consulted, among whom only 109 (8.1%) were submitted to chemical analysis of stones. Mean age of those patients were 38.9+13.4 years, and 55 (50.5%) were male. Familial history occurred in 65% of the cases. Calcium oxalate stones were found in 87% of the cases. Hypercalciuria and hyperuricosuria were the most associated metabolic disturb in patients with calcium oxalate and uric acid in the stones (60%). Conclusions: Chemical analysis has demonstrated that calcium oxalate is the most common component found in our region, according to the literature.

A nefrocalcina é uma proteína ligada à membrana?: uma hipótese bioquímica / Is nephrocalcin a membrane-bound protein?: a biochemical generate hypothesis

Introdução: A nefrocalcina (NC) é uma glicoproteína urinária que desempenha papel importante na inibição da cristalização do oxalato de cálcio (OxCa). A NC ocorre em pelo menos quatro isoformas (NC-A, B, C e D). As isoformas da NC são fosfoproteínas, mas a quantidade anormalmente elevada de resíduos de fosfato, em especial nas NC-C e D é intrigante. A NC poderia revestir a superfície de células epiteliais renais para evitar adesão de cristais de OxCa e conjugar fosfolípides da membrana em sua molécula. Objetivo: O objetivo deste trabalho foi identificar e caracterizar os fosfolipídios presentes na molécula de NC. Material e Métodos: A NC e suas isoformas foram isoladas da urina de indivíduo não portador de nefrolitíase. O conteúdo de fosfato foi determinado em cada isoforma. A taxa de inibição de crescimento de cristais de OxCa causada pela NC foi medida pelo consumo do ácido oxálico. A separação de lipídios, fosfolípides e proteínas foi realizada utilizando-se o método de separação Triton X-114. Resultados: Foram identificados os seguintes compostos: fosfatidilcolina, fosfoeta-nolamina, fosfatidilinositol, triglicérides e lipídios neutros. A composição de aminoácidos das isoformas da NC não se alterou. Após a extração com Triton X-114, os resíduos de fosfato das isoformas B, C e D diminuíram quase 1/10 do valor original e os da isoforma A não se alteraram. A constante de dissociação em direção ao oxalato de cálcio não foi alterada. Conclusão: Estes dados indicam a presença de fosfolípides típicos de membrana celular presentes na molécula de NC. A NC, incluindo-se todas as suas isoformas, pode ser uma proteína ligada à membrana, ao conjugar fosfolípides e revestir a superfície de células epiteliais tubulares.

Introduction: Nephrocalcin (NC) is a urinary glycoprotein that plays an important role in the inhibition of calcium oxalate crystallization. NC occurs in at least four isoforms (NC-A, -B, -C, and -D). NC isoforms are phosphoproteins, but the abnormally high phosphate residues, particularly in NC-C and NC-D, are intriguing. It is possible that NC molecules could coat the surface of renal epithelial cells for preventing attachment of calcium oxalate crystals, and could conjugate membrane phospholipids (PL). Our objective was to identify and characterize PL in NC. Material and Methods: NC and its four isoforms were isolated from non-stone forming urine. Phosphate contents were determined. Crystal growth inhibition rate of NC toward calcium oxalate monohydrate crystals (COM) was measured by decrease of oxalic acid. Phase separation of lipids and PL from proteins was carried out by a phase separation method using Triton X-114. Results: The following compounds were identified: phosphatidylcholine, phosphoethanolamine, phosphatidylinositol, triglycerides, and neutral lipids. Amino acid composition of the four NC isoforms before and after extraction did not change. After Triton X-114 extraction, phosphate residues in isoforms B, C, and D were decreased nearly 1/10 of the original value. However, isoform A phosphate residues showed no change. Dissociation constant toward calcium oxalate was not significantly altered. Conclusions: These data indicate the presence of typical membrane PL in NC molecule. NC, including all its isoforms, could be a membrane bound protein, conjugating membrane PL and coating the surface of the epithelial renal cells.

Renal manifestations in HIV-infected Jamaican children / Manifestaciones renales en niños jamaicanos infectados por el VIH

BACKGROUND: Documentation regarding the renal complications of paediatric HIV infection from developing countries is scarce. In the era prior to highly active antiretroviral therapy (HAART), HIV-infected children in Jamaica who developed HIV-associated nephropathy (HIVAN) progressed to end stage renal disease (ESRD) and death within a few months of diagnosis. With increased public access to antiretroviral therapy since 2002 and subsequent survival, renal complications are increasingly recognized among the surviving cohort of infected children. METHODS: A cohort of 196 HIV-infected children was followed in four multicentre ambulatory clinics from September 1, 2002 to August 31, 2005 as part of the Kingston Paediatric and Perinatal HIV/AIDS Programme, Jamaica. We describe the clinical presentations and natural history of those patients who developed renal complications. RESULTS: Urinary tract infections were the most common diagnosis, occurring in 16.8% of patients, with a high recurrence rate and the most common organism was Escherichia coli. Four of seven patients who started indinavir developed complications of nephrolithiasis and tubulointerstitial nephropathy. Six patients (3%) fulfilled the criteria for HIVAN, five of whom were male. Median age at diagnosis was five years; all presented with advanced HIV disease, nephrotic syndrome or nephrotic range proteinuria and three with chronic renal failure. Patients received standard medical management and were initiated on angiotensin-converting enzyme (ACE) inhibitors and HAART. While the mortality ratio was 50%, only one death was associated with HIVAN and the median survival time was 3.1 years. CONCLUSIONS: HIV-infected children present with a variety of renal complications. With improved survival since the introduction of HAART, the incidence of HIVAN is expected to increase among this maturing paediatric cohort. Early detection and treatment will optimize the outcomes for these children.

ANTECEDENTES: La documentación en relación con las complicaciones renales de la infección pediátrica por VIH en países en vías de desarrollo, es escasa. En la era de la terapia antiretroviral pre-altamente activa (TARAA), los niños infectados por VIH en Jamaica que desarrollaron nefropatía asociada con VIH evolucionaron hacia la enfermedad renal en fase terminal (ERFT) y la muerte dentro de pocos meses de hecho el diagnóstico. Con el aumento del acceso público a la terapia antiretroviral a partir de 2002 y la subsiguiente supervivencia, cada vez más las complicaciones renales se observan entre la cohorte sobreviviente de niños infectados. MÉTODOS: A una cohorte de 196 niños infectados con VIH, se le practicó un seguimiento en cuatro clínicas ambulatorios multicentros, desde septiembre 1 de 2002 hasta agosto 31 de 2005, como parte del Programa VIH/SIDA Prenatal y Pediátrico de Kingston, Jamaica. El trabajo describe las presentaciones clínicas y la historia natural de los pacientes que desarrollaron complicaciones renales. RESULTADOS: Las infecciones de las vías urinarias fueron el diagnóstico más común en 16.8% de los pacientes, acompañadas de una alta tasa de recurrencia, y el organismo más común fue el Escherichia coli. Cuatro de siete pacientes que comenzaron tratamiento con indinair, desarrollaron complicaciones de nefrolitiasis y nefropatía tubulointersticial. Seis pacientes (3%), cinco de ellos varones, satisfacían los criterios de NAVIH. La edad promedio al momento del diagnóstico fue de cinco años. Todos representaron con la enfermedad de VIH avanzada, síndrome nefrótico o proteniuria de rango nefrótico, y tres con fallo renal crónico. Los pacientes recibieron tratamiento médico estándar y se iniciaron en el uso de inhibidores de enzimas convertidoras de angiotensina (IECAs) y el TARAA. Si bien la proporción de la mortalidad fue 50%, sólo una muerte estuvo asociada con NAVIH y el tiempo medio de supervivencia fue 3.1 años. CONCLUSIONES: Los niños infectados con VIH se presentaron con una variedad de complicaciones renales. Con el mejoramiento de la supervivencia a partir de la introducción del TARAA, se espera que la incidencia de NAVIH aumente entre la cohorte pediátrica en maduración. La detección precoz y el tratamiento temprano optimizarán los resultados obtenidos con estos niños.

Catéter ureteral incrustado y litiasis coraliforme: reporte de un caso / Severely encrusted ureteral stents with a large associated stone burden: case report

Presentamos el caso de un paciente de 61 años, con historia de urolitiasis a repetición. Luego de la instalación de catéteres doble J en forma bilateral, se pierde de controles reapareciendo luego de 14 años. El estudio imagenológico demuestra una nefrolitiasis coraliforme completa izquierda y parcial a derecha, asociada a incrustación de ambos catéteres. Describimos el manejo exitoso de los catéteres incrustados asociados a litiasis, que requirieron una aproximación endourológica a través de cirugía percutánea. La nefrolitotomía percutánea permite el manejo exitoso de un catéter severamente incrustado asociado a una masa de cálculos, incluso en aquellos casos bilaterales.

We review our recent experience with encrusted retained ureteral stents associated with a large stone burden in a patient of 61 years old as well as current endoscopic options available for management. We described the successful management of a retained encrusted stent with endourological procedures. Percutaneous nephrolithotomy is a good option for treating a severely encrusted stent and the associated stone burden.

Laterality of symptomatic recurrent calcium nephrolithiasis

Although it is presumed that both kidneys excrete similar urinary constituents, it is a general observation that the majority of patients present with unilateral stone disease. The aim of this work was to study the laterally of recurrence in calcium stone formers. In a retrospective study of 154 patients treated for recurrent symptomatic nephrolithiasis at our institution between January 1982 and December 2006, the side of stone formation was determined by an analysis of radiographic findings and symptomatic history. Patients with hydronephrosis, major renal anatomic anomalies and non-calcium stones were excluded from the study. The features of unilateral stone forming and bilateral stone forming were compared. Statistical analysis was done using Student's t-test. Of the 154 patients, 102 were males and 52 females with a male-to-female ratio of 1:2. One hundred and fourteen patients had unilateral stones [Group A], 64 [56%] on the left and 50 [44%] on the right side. In 40 patients they were bilateral [Group B]. Comparing Groups A and B, the mean age at the first stone episode was 41.5 versus 38.3 years, the mean interval between the first stone and recurrence was 9.6 versus 9.9 years, and the mean follow-up was 13.3 versus 14.9 years [differences not statistically significant]. In Group A, 101 patients [89%] had unilateral stone recurrence episodes with 86 of these 101 patients [85%] developing stones on the same side as the primary stone. Of the 40 patients with bilateral stone formation, 30 patients [75%] had bilateral stone recurrence episodes. Recurrent stone formers commonly present with calculi on the same side and the etiology of this phenomenon is unclear, but anatomic and external triggers should be considered

Acidose tubular renal em pediatria: [revisão] / Renal tubular acidosis in pediatrics: [review]

O termo Acidose Tubular Renal (ATR) engloba diversas afecções caracterizadas por acidose metabólica secundária a um defeito na reabsorção tubular renal de HCO 3- e/ou na excreção urinária de H+, enquanto a função glomerular é nada ou minimamente afetada. Todas as formas de ATR apresentamacidose metabólica hiperclorêmica, com intervalo aniônico normal. São doenças crônicas com impacto significativo na qualidade de vida dos pacientesquando não tratadas adequadamente, podendo evoluir com déficit do crescimento, osteoporose, raquitismo, nefrolitíase e até perda da função renal.Podem ser primárias, decorrentes de defeitos genéticos nos mecanismos de transporte dos túbulos renais, ou secundárias a doenças sistêmicas ou aoefeito adverso de medicamentos. Neste artigo, é feita uma breve revisão da homeostase ácido-básica pelo rim, discutindo-se, a seguir, aspectos dafisiopatologia, diagnóstico e abordagem das acidoses tubulares renais em pediatria.

The term Renal Tubular Acidosis (RTA) defines many disorders characterized by metabolic acidosis, secondary to defects in renal tubular reabsorption ofHCO3 - and/or in urinary excretion of H+, while glomerular function is little or not affected. All forms of RTA present hyperchloremic metabolic acidosis, witha normal anion gap. When not adequately treated, these chronic diseases can have a significant impact on the quality of life of affected patients, and can evolve into growth failure, osteoporosis, rickets, nephrolithiasis and even renal insufficiency. These disorders can be primary, originating from genetic defectson tubular transport mechanisms, or can be secondary to systemic diseases and to adverse drug reactions. In this article, the mechanisms of acid-baseregulation by the kidney are briefly reviewed, followed by a presentation of the latest evidence regarding physiopathology, diagnosis and management ofrenal tubular acidosis in pediatric patients.