Síndrome de Herlyn-Werner Wünderlich: reporte de dos casos / Herlyn-Werner-Wünderlich syndrome: Two case report

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

Nefropatía por enfermedad relacionada a IGG4 / Nephropathy due to IGG4-related disease

IgG4-related disease (ER-IgG4) is a group of systemic fibro-inflammatory diseases, whose renal involvement is rare and difficult to diagnose. Diagnosis is usually made by serological and histological studies. Treatment is based on systemic corticosteroids. The renal prognosis is determined by the patient's comorbidities and the degree of fibrosis in the renal biopsy. We present the case of an elderly patient with exacerbated chronic kidney disease, whose study showed nephropathy associated with ER-IgG4.

La enfermedad relacionada a IgG4 (ER-IgG4) es un grupo de enfermedades fibro-inflamatorias sistémicas, cuya afectación renal es poco frecuente y de difícil diagnóstico. Habitualmente el diagnóstico se realiza mediante estudios serológicos e histológicos. El tratamiento se basa en corticoides sistémicos. El pronóstico renal está determinado por las comorbilidades del paciente y el grado de fibrosis en la biopsia renal. Se presenta el caso de un paciente adulto mayor con enfermedad renal crónica reagudizada, cuyo estudio demostró nefropatía asociada a ER-IgG4.

Litiasis renal derecha en riñón ectópico que simula apendicitis aguda / Right renal lithiasis in ectopic kidney that simulates acute appendicitis / Litíase renal direita em rim ectópico que simula apendicite aguda

Paciente masculino de 45 años que acude por cuadro clínico de 12 horas de evolución caracterizado por dolor abdominal, en flanco y fosa ilíaca derecha, asociado a vómitos de aspecto bilioso.

Does previous standard percutaneous nephrolithotomy impair retrograde intrarenal surgery outcomes?

ABSTRACT Purpose: The objective of this study is to evaluate the impact of a previous standard percutaneous nephrolithotomy (PCNL) on the outcomes of retrograde intrarenal surgery (RIRS). Materials and Methods: Outcomes of RIRS performed from January 2017 to January 2020 in adult patients with residual stone fragments ≤20mm after a standard PCNL (Post-PCNL) and symptomatic adult patients with kidney stones ≤20mm (Control) were prospectively studied. Stone-free rate (SFR) was evaluated on a postoperative day 90 non-contrast computed tomography. Surgical complications based on Clavien-Dindo classification during the 90 days of follow-up were recorded. Results: Outcomes of 55 patients and 57 renal units of the post-PCNL group were compared to 92 patients and 115 renal units of the control group. SFR was lower in post-PCNL group than in control (28/57, 49.1% vs. 86/115, 74.8%, p <0.001). Overall complications were more frequent in post-PCNL group (p=0.004). Infundibula strictures were identified and incised with laser in 15/57 (26.3%) renal units of the post-PCNL group. Thirteen renal units had infundibulum stricture at the site of previous percutaneous tract (13/15; 86.7%, p=0.004) and one renal unit had three infundibula strictures. Postoperative complications were not affected by the treatment of infundibula strictures (p=0.198). Conclusions: Previous standard PCNL significantly impairs the outcomes of RIRS. Infundibula strictures can be found in 26.3% of the patients with residual stone fragments after standard PCNL for large burden kidney stones. The main site of infundibulum stricture after standard PCNL is the infundibulum of the entry calyx.

Validation of 2d ultrasound and volume estimation formulae for volume assessment of kidneys and spleens: an in-vitro study / alidación de la fórmula de estimación de volumen y ultrasonido 2D para la evaluación del volumen de los riñones y el bazo: un estudio in vitro

The accuracy of internal organ volume estimation done with ultrasound (US) was found to be multifactorial. Hence, we aimed to describe and validate the volume assessment of ultrasound and standard volume estimation formulae for different shaped intra-abdominal organs using spleens and kidneys.Dissected cadaveric kidneys (n=25) and spleens (n=29) were scanned to obtain linear measurements and ultrasound auto-generated volumes (USV). Linear measurements were used to calculate the volumes manually with ellipsoid, prolate, and Lambert volume estimating formulae. The actual volumes (AV) of organs were obtained by the water displacement method. Volume assessment accuracy of USV and different formulae were compared by comparing bias, precision and Bland-Altman plot analysis. The US linear and volume measurement procedure was reliable with high inter and intra-observer agreements (linear: Chronbach's α=0.983 to 0.934; volumes: Chronbach's α=0.989). USV estimates were accurate with a high correlation to AV and low estimation bias (-5.9%). Also, prolate (bias=-0.75%) and ellipsoid formulae (bias=-3.75%) were reliable with a negligible bias in estimated volumes. Contrary, the Lambert formula was unreliable due to a high bias (41.6%). For all evaluated methods, the estimation error found to be related to the organ size (T=3.483; p=0.001), mainly when the assessed organ is larger than 50 ml. Also, the shape related estimation error found to be related to the volume estimation formula used.This study has validated the USV for kidney and splenic volume assessments while describing volume-calculating formula employed, organ size and shape as significant contributors for volume estimation accuracy.

Se encontró que la precisión de la estimación del volumen de órganos internos realizada con ultrasonido (US) es multifactorial. El objetivo fue describir y validar la evaluación de volumen mediante ecografía y las fórmulas estándar de estimación de volumen para órganos intraabdominales de diferentes formas utilizando bazos y riñones.Se evaluaron riñones cadavéricos disecados (n = 25) y bazos (n = 29) para obtener medidas lineales y volúmenes autogenerados por ultrasonido (USV). Se utilizaron medidas lineales para calcular los volúmenes manualmente con fórmulas de estimación de volumen elipsoide, prolate y Lambert. Los volúmenes reales (AV) de los órganos se obtuvieron mediante el método de desplazamiento de agua. Se comparó la precisión de la evaluación del volumen de USV y diferentes fórmulas comparando el sesgo, la precisión y el análisis de la gráfica de Bland-Altman. El procedimiento de medición lineal y de volumen mediante US fue confiable con alta concordancia inter e intraobservadores (lineal: α de Chronbach = 0,983 a 0,934; volúmenes: α de Chronbach = 0,989). Las estimaciones de USV fueron precisas con una alta correlación con AV y un bajo sesgo de estimación (-5,9%). Además, las fórmulas prolate (sesgo= -0,75%) y elipsoide (sesgo = -3,75%) fueron confiables con un sesgo insignificante en los volúmenes estimados. Por el contrario, la fórmula de Lambert no fue confiable debido a un alto sesgo (41,6%). Para todos los métodos evaluados, se encontró que el error de estimación estaba relacionado con el tamaño del órgano (T = 3.483; p = 0.001), principalmente cuando el órgano evaluado es mayor de 50 ml. Además, se encontró que el error de estimación de forma está relacionado con la fórmula de estimación de volumen utilizada.Este estudio ha validado el USV para evaluaciones de volumen renal y esplénico al mismo tiempo que describe la fórmula de cálculo de volumen empleada, el tamaño y la forma de los órganos como contribuyentes significativos de la precisión de la estimación de volumen.

Mini-percutaneous nephrolithotomy in a child with multiple urogenital anomalies and a solitary duplex kidney

ABSTRACT Purpose: To report a case of successful removal of right staghorn renal calculi in a 3-year-old girl with Arnold-Chiari malformation and multiple urogenital anomalies. Case report: A 3-year-old female child with the diagnosis of Arnold-Chiari type 2 malformation was referred to our clinic due to presence of 9 kidney stones with a total volume of 10743mm3. The total of the longest diameters of all stones was calculated as 11.4cm. The patient had a urogenital septum, bifid bladder, and duplicated collecting system on the right side. An 18F Amplatz sheath was placed and mini-percutaneous nephrolithotomy was performed successfully by laser and pneumatic lithotripter. Any residual urinary tract stones or urinary tract infection were not detected during the 6th-month follow-up. Conclusion: Urolithiasis requires a thorough understanding of the underlying causes, as well as an effective and minimally invasive treatment. It is important for urologists to understand the complexity of the optimal stone management in pediatric patients in order to maximize treatment efficacy and minimize morbidity. We conclude that it is essential to treat urolithiasis in a single session in children with urogenital anomalies and accompanying congenital anomalies who have past surgical history.

Application Progress of Arterial Spin Labeling Magnetic Resonance Imaging in Renal Perfusion Analysis / 中国医学科学院学报

Arterial spin labeling is a noninvasive,quantitative method for perfusion imaging,which does not need any contrast media.This technique has been used in the renal perfusion analysis.In this article,we briefly introduced this technique and summarized its application in healthy volunteers,acute kidney injury,chronic kidney diseases,renovascular diseases,renal tumors,and renal transplantation.

Determinants of the serial changes in measurements of renal allograft Doppler resistive index in the first postoperative month / Determinantes de alterações seriadas nas medidas do índice de resistividade de Doppler do aloenxerto renal no primeiro mês pós-operatório

Abstract Introduction: The role of single Doppler-derived renal resistive index (RI) in renal allograft management is still a controversial issue, however detection of changes in serial duplex scanning has been reported as more valuable. This study aimed to test the hypothesis that early change in RI following transplantation may be related to factors associated with delayed graft function (DGF). Material and methods: 113 patients were included, in whom two RI measurements were performed within 30 days post-transplant. According to an RI change (equal to or more than 10%) in the second measurement, patients were assigned to decrease (Group I), no change (Group II), or increase (Group III) group. Results: 30 subjects had a decrease, 55 had no change, and 28 had an increase in the second RI measurement. The donors were younger in Group III in comparison to Group II. In comparison to Group I, Group III had a higher frequency of deceased donor, DGF, and presence of tubular necrosis and tubular vacuolization in peri-implantation biopsies. Conclusion: the increase of RI during the first weeks of the postoperative period seems to be associated with DGF and with tubular necrosis / tubular vacuolization in peri-implantation biopsies, likely related to ischemia reperfusion injury.

Resumo Introdução: O papel do índice de resistividade renal (IR) derivado de varredura por Doppler no manejo de aloenxertos renais, em exame isolado, ainda é uma questão controversa; no entanto, em exames seriados, a detecção de alterações nas imagens duplex tem sido relatada como mais relevante. Material e métodos: 113 pacientes foram incluídos, nos quais duas medidas de IR foram realizadas dentro de 30 dias após o transplante. De acordo com uma alteração do IR (igual ou superior a 10%) na segunda medida, os pacientes foram classificados em redução (Grupo I), nenhuma alteração (Grupo II) ou aumento (Grupo III). Resultados: 30 indivíduos tiveram redução, 55 não tiveram alterações e 28 tiveram aumento na segunda medição do IR. Os doadores eram mais jovens no Grupo III em comparação ao Grupo II. Em comparação ao Grupo I, o Grupo III apresentou maior frequência de doador falecido, FTE, presença de necrose tubular e vacuolização tubular nas biópsias peri-implantares. Conclusão: o aumento do IR durante as primeiras semanas no período pós-operatório parece estar associado à FTE e à necrose tubular/vacuolização tubular nas biópsias peri-implantares, provavelmente relacionadas à lesão por isquemia-reperfusão.

Diagnóstico prenatal y posnatal de anomalías del tracto urinario / Pre- natal and post-natal diagnosis of urinary tract anomalies

Introducción: El ultrasonido prenatal aporta una magnífica forma de introducirnos en la patofisiología renal humana, pero es incapaz de detectar todas las anomalías. Objetivos: Identificar las principales anomalías de riñón y tracto urinario en pacientes con diagnóstico prenatal o posnatal de estas anomalías. Métodos: Se incluyeron en el estudio todos los pacientes que llegaron remitidos al servicio de Nefrología del Hospital Pediátrico Docente William Soler entre el 1ero. de octubre de 2015 y el 30 de septiembre de 2017, por haberse detectado alteraciones en el ultrasonido prenatal durante el seguimiento de un embarazo normal y aquellos en los que después del nacimiento se comprobó alguna anormalidad en forma incidental o por síntomas relacionados. Se programó seguimiento clínico, imagenológico y terapéutico de acuerdo con la anomalía detectada. Resultados: El estudio incluyó 81 pacientes, 65 con diagnóstico prenatal y 16 con hallazgo posnatal. El sexo masculino estuvo representado por el 66,7 por ciento y la anomalía más frecuente estudiada resultó la displasia renal multiquística (23,4 por ciento). En segundo lugar, el diagnóstico morfológico correspondió a dilataciones del tracto urinario, que en su estudio posnatal se clasificaron como hidronefrosis (20,9 por ciento), pielectasias (17,4 por ciento) y reflujo vesicoureteral (7,4 por ciento). Conclusiones: El ultrasonido prenatal para la detección de anomalías de riñón y tracto urinario es un proceder diagnóstico de gran utilidad porque permite prepararnos para enfrentar estas anomalías antes que presente síntomas relacionados y además puede proporcionar una adecuada información a los padres. No todas las anomalías congénitas se detectan mediante ecografía prenatal(AU)

Introduction: The prenatal ultrasound provides a great way to introduce us to the human renal pathophysiology, but is unable to detect all of the anomalies. Objectives: To identify the main anomalies of the kidney and urinary tract in patients with prenatal or postnatal diagnosis of those. Methods: There were included in the study all the patients who were referred to the Nephrology Service of William Soler Pediatric Teaching Hospital from October 1, 2015 to September 30, 2017 being detected alterations in the prenatal ultrasound during the follow-up of a normal pregnancy and those in which after birth any abnormality was found incidentally or by related symptoms. Clinical, imaging and therapeutic follow-up were scheduled in accordance with the anomaly detected. Results: The study included 81 patients, 65 with prenatal diagnosis and 16 with post-natal finding. The male sex was represented by the 66.7 percent and the most common anomaly studied was the multicystic dysplastic kidney (23.4 percent). Secondly, the morphological diagnosis corresponded to dilations of the urinary tract, which in the post-natal study were classified as hydronephrosis (20.9 percent), pyelectasis (17.4 percent) and vesicoureteral reflux (7.4 percent). Conclusions: The prenatal ultrasound for the detection of kidney and urinary tract´s anomalies is a diagnosis of great utility because it allows us to prepare to face these anomalies before they present related symptoms and it can also provide adequate information to parents. Not all congenital anomalies are detected by prenatal ultrasound(AU)

Ectopia renal cruzada y fusionada / Crossed-fused renal ectopia

Introducción: La ectopia renal cruzada fusionada, es una anomalía congénita infrecuente, que acontece durante la migración fisiológica de dichos órganos en la etapa embrionaria. Objetivo: Describir detalles clínicos e imaginológicos de un nuevo caso de ectopia renal cruzada fusionada. Caso clínico: Paciente masculino de 48 años de edad, con antecedentes de hiperplasia prostática, con tratamiento de terazosina. Presentó retención completa de orina por lo cual se le colocó sonda uretral. El ultrasonido abdominal reportó que el riñón izquierdo era de ecoestructura, tamaño y posición normales, pero el derecho no estaba en la localización normal y se sospechó que estaba situado en la región del mesogastrio. La vejiga era normal y la próstata tenía un volumen de 87 cm3. La tomografía axial computarizada abdominal contrastada, confirmó que se trataba de una ectopia renal derecha cruzada fusionada, en el polo inferior del riñón ortotópico, en forma de "J". Se intentó retirar la sonda uretral en dos ocasiones y volvía a obstruirse, por lo cual se decidió la terapéutica quirúrgica definitiva. En el chequeo preoperatorio todos los estudios de la analítica sanguínea fueron normales. Al paciente se le realizó la adenomectomía retropúbica. Evolucionó satisfactoriamente. El estudio histopatológico de la pieza quirúrgica informó hiperplasia prostática. Conclusiones: La ectopia renal cruzada fusionada es infrecuente. El diagnóstico de certeza se obtiene mediante la tomografía axial computarizada abdominal contrastada(AU)

Introduction: Crossed-fused renal ectopia is a rare congenital anomaly, which occurs during the physiological migration of these organs in the embryonic stage. Objective: To describe clinical and imaginological details of a new case of crossed-fused renal ectopia. Case report: 48-year-old male patient, with a history of prostatic hyperplasia, with terazosin treatment. He had complete retention of urine for which urethral catheter was placed. Abdominal ultrasound reported that the left kidney was of normal structure, size and echo, but the right one was not in the normal location and was suspected of being located in the mesogastrium region. The bladder was normal and the prostate had a volume of 87 cm3. Contrast abdominal computed tomography confirmed that it was a fused right renal ectopia, in the lower pole of the orthotopic kidney, in the form of "J". An attempt was made to remove the urethral catheter twice and it became blocked again, so the definitive surgical therapy was decided. In the preoperative check-up, all blood test studies were normal. The patient underwent retropubic adenomectomy. It evolved satisfactorily. Histopathological study of the surgical specimen reported prostatic hyperplasia. Conclusions: Crossed-fused renal ectopia is uncommon. The diagnosis of certainty is obtained by contrasted abdominal computed tomography(AU)

Image-guided study of swine anatomy as a tool for urologic surgery research and training

Abstract Purpose: To describe the anatomy of the swine urinary system using computed tomography and to discuss the role of this animal as an experimental model for urological procedures. Methods: Three male Landrace pigs underwent computed tomography and the anatomy of the urinary system and renal circulation was analyzed and described. Results: In all animals, 2 kidneys, 2 ureters and one bladder were identified. Each kidney presented a single renal artery vascularization, with a mean diameter on the right of 4.45 and 5.31 mm on the left (p < 0.0001) and single renal vein drainage, with a mean diameter on the right of 5.78 and 5.82 mm on the left (p = 0.0336). The average renal length was 9.85 cm on the right and 10.30 cm on the left (p < 0.0001). The average renal volume was 113.70 cm3 on the right and 109.70 cm3 on the left (p < 0.0001). The average length of the ureter was 19.78 cm on the right and 22.08 cm on the left (p < 0.0001). The average bladder volume was 423.70 cm3. Conclusions: The data obtained show similarities with human anatomy, suggesting the viability of the swine model for planning preclinical trials, basic research, refinement in experimental surgery and surgical training for urological procedures.

A comparative study of ultrasound and cross-sectional imaging for detection of small renal masses: anatomic factors and radiologists experience / Estudo comparativo de ultrassonografia e métodos seccionais para detecção de pequenas lesões renais: fatores anatômicos e experiência do radiologista

ABSTRACT Objective: To evaluate anatomic factors and radiologist's experience in the detection of solid renal masses on ultrasonography. Methods: We searched for solid renal masses diagnosed on cross-sectional imaging from 2007 to 2017 that also had previous ultrasonography from the past 6 months. The following features were evaluated: nodule size, laterality, location and growth pattern, patient body mass index and radiologist's experience in ultrasound. In surgically resected cases, pathologic reports were evaluated. Unpaired t test and χ2 test were used to evaluate differences among subgroups, using R-statistics. Statistical significance was set at p<0.05. Results: The initial search of renal nodules on cross-sectional imaging resulted in 428 lesions and 266 lesions were excluded. Final cohort included 162 lesions and, of those, 108 (67%) were correctly detected on ultrasonography (Group 1) and 54 (33%) were missed (Group 2). Comparison of Groups 1 and 2 were as follows, respectively: body mass index (27.7 versus 27.1; p=0.496), size (2.58cm versus 1.74cm; p=0.003), laterality (54% versus 59% right sided; p=0.832), location (27% versus 22% upper pole; p=0.869), growth pattern (25% versus 28% endophytic; p=0.131) and radiologist's experience (p=0.300). From surgically resected cases, histology available for Group 1 was clear cell (n=11), papillary (n=15), chromophobe (n=2) renal cell carcinoma, oncocytoma (n=1), and, for Group 2, clear cell (n=7), papillary (n=5) renal cell carcinoma, oncocytoma (n=2), angiomyolipoma, chromophobe renal cell carcinoma, and interstitial pyelonephritis (n=1, each). Conclusion: Size was the only significant parameter related to renal nodule detection on ultrasound.

RESUMO Objetivo: Avaliar os fatores anatômicos e a experiência do radiologista na detecção de massas renais sólidas na ultrassonografia. Métodos: Buscamos massas renais sólidas diagnosticadas em imagens seccionais, de 2007 a 2017, que também tivessem ultrassonografia prévia nos últimos 6 meses. As seguintes características foram avaliadas: tamanho do nódulo, lateralidade, localização e padrão de crescimento, índice de massa corporal do paciente e experiência do radiologista em ultrassonografia. Nos casos com ressecção cirúrgica, os laudos de patologia foram analisados. O teste t não pareado e o teste χ2 foram utilizados para avaliar as diferenças entre os subgrupos, usando R-statistics. A significância estatística foi estabelecida em p<0,05. Resultados: A pesquisa inicial de nódulos renais achados em imagens seccionais resultou em 428 lesões, com 266 exclusões. A coorte final incluiu 162 lesões e, destas, 108 (67%) foram detectadas corretamente na ultrassonografia (Grupo 1), e 54 (33%) não foram identificadas (Grupo 2). A comparação dos Grupos 1 e 2 mostrou índice de massa corporal (27,7 versus 27,1; p=0,496), tamanho (2,58cm versus 1,74cm; p=0,003), lateralidade (54% versus 59% no lado direito; p=0,832), localização (27% versus 22% no polo superior; p=0,869), padrão de crescimento (25% versus 28% endofítico; p=0,131) e experiência do radiologista (p=0,300). A histologia disponível para o Grupo 1 foi carcinoma renal de células claras (n=11), papilar (n=15), cromófobo (n=2), oncocitoma (n=1), e, para o Grupo 2, carcinoma renal de células claras (n=7), papilar (n=5), oncocitoma (n=2), angiomiolipoma, cromófobo e pielonefrite intersticial (n=1, cada). Conclusão: O tamanho foi o único parâmetro significativo relacionado à detecção de nódulos renais no ultrassom.

Endothelial lesion and complement activation in patients with Scleroderma Renal Crisis / Lesão endotelial e ativação do complemento em pacientes com crise renal esclerodérmica

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.

Resumo Nas revisões de biópsias renais, a crise renal esclerodérmica (CRE) é caracterizada por lesões endoteliais vasculares, depósitos de C4d em vasos peritubulares e lesões agudas e crônicas que coexistem na mesma biópsia. Os sinais clínicos de microangiopatia trombótica (MAT) são descritos na esclerose sistêmica (ES); no entanto, não foram relacionados às lesões agudas descritas nas biópsias renais. Relatamos um caso de CRE em um paciente com síndrome de superposição de esclerodermia-dermatomiosite, que também apresentou dados clínicos e histopatológicos de MAT. No exame de fundo do olho, foi encontrada uma retinopatia hipertensiva aguda grave. A biópsia renal mostrou lesão endotelial grave com alargamento das células mucoides ao nível da íntima, proliferação concêntrica focal na maioria das pequenas arteríolas e depósitos de C3, C4d e IgM ao longo das paredes dos capilares. O estudo genético do complemento mostrou apenas a presença de haplótipos de risco da proteína cofator de membrana (PCM), sem outros distúrbios genéticos do complemento. Entendemos que em um paciente com MAT e ES, o dano renal seria fundamentalmente endotelial e do tipo agudo; além disso, observaríamos evidências claras de ativação do complemento. Uma vez que novos estudos correlacionam dados clínico-analíticos com estudos anatomopatológicos, é provável que sejamos forçados a redefinir o conceito de CRE, enfocando a relação entre dano endotelial agudo e ativação do complemento.