Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 24
Filtrar
1.
Asian J Surg ; 44(1): 80-86, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32291131

RESUMEN

PURPOSE: To determine the risk factors of intraoperative cyst rupture in partial nephrectomy (PN) for a cystic renal mass (CRM) and their effect on the prognosis of patients. MATERIALS AND METHODS: Patients who underwent partial nephrectomy for CRMs from January 2009 to January 2015 were included. Uni/multivariate Logistic/Cox analysis and Kaplan-Meier analysis were performed. RESULTS: A total of 174 patients were included in this study. There were 27 (15.5%) intraoperative cyst ruptures. The median follow-up time was 60 months. Multivariate logistic analysis showed that the E component (P = 0.018) and N component (P = 0.022) of the R.E.N.A.L. nephrometry score, Bosniak category III (P = 0.044), and surgeon's experience (P = 0.030) were risk factors associated with intraoperative cyst rupture in PN for CRMs. The 5-year recurrence-free survival (RFS), cancer-free survival (CFS) and overall survival (OS) were 92.7%, 90.32% and 94.4%, respectively, in 124 cases of malignant CRM. Kaplan-Meier analysis demonstrated that 5-year RFS and 5-year CFS in patients with cyst rupture was worse than those without cyst rupture (P = 0.006 and 0.003, respectively). Multivariate Cox analysis revealed that intraoperative cyst rupture was independent risk factor for 5-year RFS and 5-year CFS (P = 0.039 and 0.013, respectively). However, there was no significant difference in OS between the two groups (P = 0.275). CONCLUSIONS: The prevalence of intraoperative cyst rupture is relatively high. Higher E and N scores, Bosniak category III, and lacking surgical experience (<20 cases) increase the risk of occurrence of intraoperative cyst rupture.


Asunto(s)
Complicaciones Intraoperatorias/etiología , Enfermedades Renales Quísticas/cirugía , Nefrectomía/métodos , Rotura Espontánea/etiología , Adulto , Anciano , Femenino , Humanos , Complicaciones Intraoperatorias/epidemiología , Estimación de Kaplan-Meier , Enfermedades Renales Quísticas/mortalidad , Masculino , Persona de Mediana Edad , Prevalencia , Pronóstico , Factores de Riesgo , Rotura Espontánea/epidemiología , Tasa de Supervivencia
2.
BMC Med Genet ; 21(1): 96, 2020 05 07.
Artículo en Inglés | MEDLINE | ID: mdl-32381069

RESUMEN

BACKGROUND: Joubert syndrome (JBTS) is a genetically heterogeneous group of neurodevelopmental syndromes caused by primary cilia dysfunction. Usually the neurological presentation starts with abnormal neonatal breathing followed by muscular hypotonia, psychomotor delay, and cerebellar ataxia. Cerebral MRI shows mid- and hindbrain anomalies including the molar tooth sign. We report a male patient with atypical presentation of Joubert syndrome type 23, thus expanding the phenotype. CASE PRESENTATION: Clinical features were consistent with JBTS already from infancy, yet the syndrome was not suspected before cerebral MRI later in childhood showed the characteristic molar tooth sign and ectopic neurohypophysis. From age 11 years seizures developed and after few years became increasingly difficult to treat, also related to inadequate compliance to therapy. He died at 23 years of sudden unexpected death in epilepsy (SUDEP). The genetic diagnosis remained elusive for many years, despite extensive genetic testing. We reached the genetic diagnosis by performing whole genome sequencing of the family trio and analyzing the data with the combination of one analysis pipeline for single nucleotide variants (SNVs)/indels and one for structural variants (SVs). This lead to the identification of the most common variant detected in patients with JBTS23 (OMIM# 616490), rs534542684, in compound heterozygosity with a 8.3 kb deletion in KIAA0586, not previously reported. CONCLUSIONS: We describe for the first time ectopic neurohypophysis and SUDEP in JBTS23, expanding the phenotype of this condition and raising the attention on the possible severity of the epilepsy in this disease. We also highlight the diagnostic power of WGS, which efficiently detects SNVs/indels and in addition allows the identification of SVs.


Asunto(s)
Anomalías Múltiples/genética , Proteínas de Ciclo Celular/genética , Cerebelo/anomalías , Muerte Súbita/patología , Epilepsia/genética , Anomalías del Ojo/genética , Enfermedades Renales Quísticas/genética , Retina/anomalías , Anomalías Múltiples/mortalidad , Anomalías Múltiples/patología , Adulto , Cerebelo/patología , Niño , Muerte Súbita/epidemiología , Discapacidades del Desarrollo/genética , Discapacidades del Desarrollo/mortalidad , Discapacidades del Desarrollo/patología , Epilepsia/mortalidad , Epilepsia/patología , Anomalías del Ojo/mortalidad , Anomalías del Ojo/patología , Femenino , Heterocigoto , Humanos , Mutación INDEL , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/patología , Masculino , Neurohipófisis/metabolismo , Neurohipófisis/patología , Retina/patología , Secuenciación Completa del Genoma , Adulto Joven
3.
Med Biol Eng Comput ; 58(1): 1-24, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31748942

RESUMEN

Renal imaging is an essential investigative tool and preliminary task for determining a suitable sanative option for the treatment of kidney cancer. In recent decades, with the increasing usage of abdominal imaging, there is an upsurge in the number of adrenal incidentalomas. Among these accidentally revealed lesions, most of them are complex that warrant immediate aggressive treatment planning due to their malignant potential. The guidelines given by the American Urological Association (AUA), American College of Radiology (ACR), and European Association of Urology (EAU) vary concerning the use of ideal preliminary imaging modality to investigate the patients with suspected flank pain, hematuria, or palpable mass in the abdomen. Initially, an effort has been made to discriminate cystic and solid renal lesions which are helpful in separating benign and malignant nature as different imaging patterns are observed on distinct imaging modalities for solid and cystic renal lesions. Various attempts have been made to improve the accuracy of cancer diagnosis by employing different imaging modalities. The primary aim of this article is to study the capabilities of different imaging techniques for detecting and differentiating solid and cystic lesions to facilitate treatment planning based on computed tomography (CT), ultrasonography (US), magnetic resonance imaging (MRI), positron emission tomography (PET), and optical coherence tomography (OCT). Further, the advantages, disadvantages, new advancements, and future scope of each of the imaging modality have also been highlighted so that one can make a correct choice of imaging technique for diagnosis of a specific type of lesion. Additionally, some recommendations have also been mentioned by listing the requirements for the perfect imaging modality. Graphical abstract.


Asunto(s)
Diagnóstico por Imagen , Enfermedades Renales Quísticas/diagnóstico por imagen , Enfermedades Renales Quísticas/diagnóstico , Animales , Humanos , Riñón/diagnóstico por imagen , Riñón/patología , Enfermedades Renales Quísticas/mortalidad
4.
J Urol ; 200(6): 1200-1206, 2018 12.
Artículo en Inglés | MEDLINE | ID: mdl-29935273

RESUMEN

PURPOSE: Our objective was to assess the prevalence of intraoperative cyst rupture and its impact on oncologic outcomes. MATERIALS AND METHODS: All patients who underwent partial nephrectomy for a cystic renal mass via an open or robot-assisted approach at a total of 8 academic institutions were included in this retrospective study. All operative reports were carefully reviewed and any description of cyst rupture, cyst effraction or local spillage intraoperatively was recorded as cyst rupture. Multivariate logistic regression analysis was done to assess the variables associated with cyst rupture. Recurrence-free, cancer specific and overall survival was estimated by the Kaplan-Meier method and compared with the log rank test. RESULTS: Overall 268 patients were included in study. There were 50 intraoperative cyst ruptures (18.7%) in the whole cohort. No preoperative parameter was significantly associated with a risk of intraoperative cyst rupture on univariate or multivariate analysis. Of the cystic renal masses 75% were malignant on the final pathology report. At a median followup of 32 months 5 patients (2.5%) had local recurrence while progression to metastasis was observed in 2%. There were no peritoneal carcinomatosis nor port site metastasis. There was also no local or metastatic recurrence in the subgroup with intraoperative cyst rupture. Estimated recurrence-free survival did not differ significantly between patients with vs without intraoperative cyst rupture at 100% vs 92.7% at 5 years (p = 0.20). CONCLUSIONS: Intraoperative cyst rupture during partial nephrectomy is a relatively common occurrence but with few oncologic implications.


Asunto(s)
Complicaciones Intraoperatorias/epidemiología , Enfermedades Renales Quísticas/cirugía , Neoplasias Renales/cirugía , Recurrencia Local de Neoplasia/epidemiología , Nefrectomía/efectos adversos , Procedimientos Quirúrgicos Robotizados/efectos adversos , Anciano , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Complicaciones Intraoperatorias/etiología , Estimación de Kaplan-Meier , Riñón/patología , Riñón/cirugía , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/patología , Neoplasias Renales/mortalidad , Neoplasias Renales/patología , Masculino , Persona de Mediana Edad , Nefrectomía/métodos , Prevalencia , Estudios Retrospectivos , Procedimientos Quirúrgicos Robotizados/métodos
5.
Radiology ; 287(2): 504-514, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29401040

RESUMEN

Purpose To determine the effects of patient age and comorbidity level on life expectancy (LE) benefits associated with imaging follow-up of Bosniak IIF renal cysts and pancreatic side-branch (SB) intraductal papillary mucinous neoplasms (IPMNs). Materials and Methods A decision-analytic Markov model to evaluate LE benefits was developed. Hypothetical cohorts with varied age (60-80 years) and comorbidities (none, mild, moderate, or severe) were evaluated. For each finding, LE projections from two strategies were compared: imaging follow-up and no imaging follow-up. Under follow-up, it was assumed that cancers associated with the incidental finding were successfully treated before they spread. For patients without follow-up, mortality risks from Bosniak IIF cysts (renal cell carcinoma) and SBIPMNs (pancreatic ductal adenocarcinoma) were incorporated. Model assumptions and parameter uncertainty were evaluated in sensitivity analysis. Results In the youngest, healthiest cohorts (age, 60 years; no comorbidities), projected LE benefits from follow-up were as follows: Bosniak IIF cyst, 6.5 months (women) and 5.8 months (men); SBIPMN, 6.4 months (women) and 5.3 months (men). Follow-up of Bosniak IIF cysts in 60-year-old women with severe comorbidities yielded a LE benefit of 3.9 months; in 80-year-old women with no comorbidities, the benefit was 2.8 months, and with severe comorbidities the benefit was 1.5 months. Similar trends were observed in men and for SBIPMN. Results were sensitive to the performance of follow-up for cancer detection; malignancy risks; and stage at presentation of malignant, unfollowed Bosniak IIF cysts. Conclusion With progression of age and comorbidity level, follow-up of low-risk incidental findings yields increasingly limited benefits for patients. © RSNA, 2018 Online supplemental material is available for this article.


Asunto(s)
Carcinoma Ductal Pancreático/diagnóstico por imagen , Carcinoma de Células Renales/diagnóstico por imagen , Hallazgos Incidentales , Enfermedades Renales Quísticas/diagnóstico por imagen , Neoplasias Renales/diagnóstico por imagen , Neoplasias Pancreáticas/diagnóstico por imagen , Factores de Edad , Anciano , Carcinoma Ductal Pancreático/mortalidad , Carcinoma Ductal Pancreático/patología , Carcinoma de Células Renales/mortalidad , Carcinoma de Células Renales/patología , Comorbilidad , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/patología , Neoplasias Renales/mortalidad , Neoplasias Renales/patología , Esperanza de Vida , Masculino , Cadenas de Markov , Persona de Mediana Edad , Neoplasias Pancreáticas/mortalidad , Neoplasias Pancreáticas/patología , Sensibilidad y Especificidad
6.
Am J Med Genet A ; 173(5): 1237-1242, 2017 May.
Artículo en Inglés | MEDLINE | ID: mdl-28371402

RESUMEN

Joubert syndrome (JS) is a rare, recessively inherited neurodevelopmental disorder characterized by a distinctive mid-hindbrain malformation. Little is known about mortality in affected individuals. Identifying the timing and causes of death will allow for development of healthcare guidelines for families and providers and, thus, help to prolong and improve the lives of patients with JS. We evaluated information on 40 deceased individuals with JS to characterize age and cause of death. We compared this population with 525 living individuals with JS to estimate associations between risk of death and extra-neurological features. Genetic causes were examined in both groups. Mean age of death in this cohort was 7.2 years, and the most prevalent causes of death were respiratory failure (35%), particularly in individuals younger than 6 years, and kidney failure (37.5%), which was more common in older individuals. We identified possible associations between risk of death and kidney disease, liver fibrosis, polydactyly, occipital encephalocele, and genetic cause. This work highlights factors (genetic cause, extra-neurological organ involvement, and other malformations) likely to be associated with higher risk of mortality in JS, which should prompt increased monitoring for respiratory issues, kidney disease, and liver fibrosis.


Asunto(s)
Anomalías Múltiples/mortalidad , Cerebelo/anomalías , Anomalías del Ojo/mortalidad , Enfermedades Renales Quísticas/mortalidad , Insuficiencia Renal/mortalidad , Retina/anomalías , Anomalías Múltiples/genética , Anomalías Múltiples/fisiopatología , Adolescente , Cerebelo/fisiopatología , Niño , Preescolar , Anomalías del Ojo/complicaciones , Anomalías del Ojo/genética , Anomalías del Ojo/fisiopatología , Femenino , Humanos , Enfermedades Renales Quísticas/complicaciones , Enfermedades Renales Quísticas/genética , Enfermedades Renales Quísticas/fisiopatología , Masculino , Insuficiencia Renal/complicaciones , Insuficiencia Renal/genética , Insuficiencia Renal/patología , Retina/fisiopatología , Rombencéfalo/anomalías , Rombencéfalo/fisiopatología
7.
Pediatr Nephrol ; 31(4): 563-74, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26081158

RESUMEN

Nowadays most renal disorders, especially urinary tract malformations and renal cystic disease, are diagnosed antenatally. In cases of severe bilateral disease, intrauterine renal dysfunction may lead to renal oligohydramnios (ROH), resulting in pulmonary hypoplasia which affects perinatal mortality and morbidity as well as the long-term outcome. However, some infants may only have mild pulmonary and renal disease, and advances in postnatal and dialysis treatment have resulted in improved short- and long-term outcome even in those infants with severe ROH. Here, we review the current state of knowledge and clinical experience of patients presenting antenatally with severe bilateral renal disorders and ROH. By addressing underlying mechanisms, intrauterine tools of diagnosis and treatment as well as published outcome data, we hope to improve antenatal counselling and postnatal care. KEY SUMMARY POINTS: 1. Nowadays most renal disorders are diagnosed antenatally, especially urinary tract malformations and renal cystic disease. 2. Severe kidney dysfunction may lead to renal oligohydramnios, which can cause pulmonary hypoplasia and is a risk factor of perinatal mortality and postnatal renal outcome. However, as considerable clinical heterogeneity is present, outcome predictions need to be treated with caution. 3. Advances in postnatal and dialysis treatment have resulted in improved short- and long-term outcomes even in infants with severe renal oligohydramnios. 4. A multidisciplinary approach with specialist input is required when counselling a family with an ROH-affected fetus as the decision-making process is very challenging.


Asunto(s)
Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/terapia , Oligohidramnios/diagnóstico , Oligohidramnios/terapia , Atención Prenatal , Diagnóstico Prenatal , Anomalías Urogenitales/diagnóstico , Anomalías Urogenitales/terapia , Femenino , Muerte Fetal , Humanos , Enfermedades Renales Quísticas/complicaciones , Enfermedades Renales Quísticas/mortalidad , Oligohidramnios/etiología , Oligohidramnios/mortalidad , Mortalidad Perinatal , Valor Predictivo de las Pruebas , Embarazo , Medición de Riesgo , Factores de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Anomalías Urogenitales/complicaciones , Anomalías Urogenitales/mortalidad
8.
AJR Am J Roentgenol ; 203(6): W629-36, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25415728

RESUMEN

OBJECTIVE: The objective of this study was to quantify the effects of radiation-induced cancer risks in patients with Bosniak category IIF lesions undergoing CT versus MRI surveillance. MATERIALS AND METHODS: We developed a Markov-Monte Carlo model to determine life expectancy losses attributable to radiation-induced cancers in hypothetical patients undergoing CT versus MRI surveillance of Bosniak IIF lesions. Our model tracked hypothetical patients as they underwent imaging surveillance for up to 5 years, accounting for potential lesion progression and treatment. Estimates of radiation-induced cancer mortality were generated using a published organ-specific radiation-risk model based on Biological Effects of Ionizing Radiation VII methods. The model also incorporated surgical mortality and renal cancer-specific mortality. Our primary outcome was life expectancy loss attributable to radiation-induced cancers. A sensitivity analysis was performed to assess the stability of the results with variability in key parameters. RESULTS: The mean number of examinations per patient was 6.3. In the base case, assuming 13 mSv per multiphase CT examination, 64-year-old men experienced an average life expectancy decrease of 5.5 days attributable to radiation-induced cancers from CT; 64-year-old women experienced a corresponding life expectancy loss of 6.9 days. The results were most sensitive to patient age: Life expectancy loss attributable to radiation-induced cancers increased to 21.6 days in 20-year-old women and 20.0 days in 20-year-old men. Varied assumptions of each modality's (CT vs MRI) depiction of lesion complexity also impacted life expectancy losses. CONCLUSION: Microsimulation modeling shows that radiation-induced cancer risks from CT surveillance for Bosniak IIF lesions minimally affect life expectancy. However, as progressively younger patients are considered, increasing radiation risks merit stronger consideration of MRI surveillance.


Asunto(s)
Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/mortalidad , Esperanza de Vida , Imagen por Resonancia Magnética/mortalidad , Modelos Estadísticos , Neoplasias Inducidas por Radiación/mortalidad , Tomografía Computarizada por Rayos X/mortalidad , Comorbilidad , Simulación por Computador , Supervivencia sin Enfermedad , Femenino , Humanos , Incidencia , Imagen por Resonancia Magnética/estadística & datos numéricos , Masculino , Persona de Mediana Edad , Modelos de Riesgos Proporcionales , Medición de Riesgo , Vigilancia de Guardia , Análisis de Supervivencia , Tasa de Supervivencia , Tomografía Computarizada por Rayos X/estadística & datos numéricos
9.
Urol Oncol ; 32(7): 1010-6, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-25022857

RESUMEN

OBJECTIVES: To characterize pathological and cancer-specific outcomes of surgically resected cystic renal tumors and to identify clinical or radiographic features associated with these outcomes. METHODS AND MATERIALS: All patients at our institution who underwent radical or partial nephrectomy for complex renal cystic masses between 2004 and 2011 with available computed tomographic imaging were included. The Bosniak score was determined, as were 10 specific radiographic characteristics of renal cysts in patients with preoperative imaging available for review. These characteristics were correlated with cystic mass histopathology. Recurrence-free survival after surgery was determined. RESULTS: Overall, 133 patients underwent renal surgery for complex cystic lesions, 89 (67%) of whom had malignant lesions. Malignancy risk increased with Bosniak score (P≤0.01) and presence of mural nodules (P = 0.01). Most (63%) malignancies demonstrated clear cell histology. The papillary renal cell carcinomas (25%) exhibited lower enhancement levels (P = 0.04) and were less often septated (P<0.01). Of the malignancies, 79% were low stage (pT1), and 73% were Fuhrman grade 1 or 2. Large cyst size was associated with advanced tumor stage (P = 0.05). Neither Bosniak score nor any other radiographic parameter was associated with Fuhrman grade. In 70 patients with a median follow-up of 43 months, only 1 (1.4%) developed disease recurrence. CONCLUSIONS: Most cystic renal malignancies are low-stage, low-grade lesions. Papillary renal cell carcinomas account for nearly a quarter of cystic renal malignancies and have unique radiographic characteristics. Disease recurrence after surgical resection is rare. These findings suggest an indolent behavior for cystic renal tumors, and these lesions may be amenable to active surveillance.


Asunto(s)
Carcinoma de Células Renales/patología , Quistes/patología , Enfermedades Renales Quísticas/patología , Neoplasias Renales/patología , Anciano , Carcinoma de Células Renales/diagnóstico por imagen , Carcinoma de Células Renales/mortalidad , Quistes/diagnóstico por imagen , Quistes/mortalidad , Supervivencia sin Enfermedad , Femenino , Humanos , Enfermedades Renales Quísticas/diagnóstico por imagen , Enfermedades Renales Quísticas/mortalidad , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/mortalidad , Masculino , Persona de Mediana Edad , Tomografía Computarizada por Rayos X
10.
Pediatr Nephrol ; 27(6): 965-72, 2012 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-22402647

RESUMEN

BACKGROUND: Congenital abnormalities of the kidney and urinary tract (CAKUT) are significant causes of morbidity. The aim of the study was to determine predictive factors of mortality in newborns with CAKUT. METHODS: All 29,653 consecutive newborns hospitalized in a tertiary neonatal unit between 1996 and 2006 were evaluated. The main outcome was neonatal mortality. The variables analyzed as risk factors were maternal age, first pregnancy, low birth weight (LBW), prematurity, oligohydramnios, and CAKUT associated with other malformations (Associated CAKUT). RESULTS: CAKUT was detected in 524 newborns, with an overall prevalence of 17.7 per 1,000 live births. A total of 325 (62%) cases were classified as urinary tract dilatation, 79 (15.1%) as renal cystic disease, and 120 (22.9%) as other subgroups. In the urinary tract dilatation subgroup, independent risk factors for early mortality were Associated CAKUT [odds ratio (OR) 20.7], prematurity (OR 4.5) LBW (OR 3.8), oligohydramnios (OR 3.0), and renal involvement (OR 3.0). In the renal cystic disease subgroup, two variables remained associated with neonatal mortality: LBW (OR 12.3) and Associated CAKUT (OR 21.4). CONCLUSION: The presence of extrarenal anomalies was a strong predictor of poor outcome in a larger series of infants with CAKUT.


Asunto(s)
Mortalidad Infantil , Riñón/anomalías , Sistema Urinario/anomalías , Anomalías Urogenitales/mortalidad , Brasil/epidemiología , Dilatación Patológica , Femenino , Humanos , Hidronefrosis/mortalidad , Recién Nacido , Riñón/diagnóstico por imagen , Enfermedades Renales Quísticas/mortalidad , Modelos Logísticos , Masculino , Oportunidad Relativa , Prevalencia , Sistema de Registros , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Factores de Tiempo , Ultrasonografía Prenatal , Sistema Urinario/diagnóstico por imagen , Anomalías Urogenitales/diagnóstico , Anomalías Urogenitales/diagnóstico por imagen
11.
J Urol ; 173(6): 1893-6, 2005 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-15879770

RESUMEN

PURPOSE: We compared histological subtype, pathological features and outcome of patients with solid renal masses who were 18 to 40 years old vs patients who were 60 to 70 years old. MATERIALS AND METHODS: We conducted a retrospective review of the Mayo Clinic Nephrectomy Registry from 1970 to 2000, and identified 124 patients 18 to 40 years old and 1,067 patients 60 to 70 years old available for analysis. RESULTS: There was no significant difference in the incidence of benign solid renal masses between patients 18 to 40 years old and those 60 to 70 years old (13.7% vs 10.2%). Among patients with renal cell carcinoma (RCC), younger patients were more likely to have chromophobe RCC (13.1% vs 3.6%) and less likely to have clear cell RCC (70.1% vs 81.5%) than older patients. Among patients with clear cell RCC, younger patients were more likely to have stage pT2b or lower tumors (82.7% vs 69.9%) and a higher incidence of cystic clear cell RCC (10.7% vs 2.2%) than older patients. Younger patients had an improved cancer specific survival compared with older patients but this difference was not statistically significant (risk ratio 0.71, p =0.127). CONCLUSIONS: We found that patients 18 to 40 years old were more likely to have chromophobe and less likely to have clear cell RCC compared with patients 60 to 70 years old. We did not identify a higher incidence of papillary RCC in younger patients. Patients with clear cell RCC 18 to 40 years old had a higher incidence of low stage and cystic tumors compared with patients 60 to 70 years old, features which have been shown to have a favorable prognosis. These factors likely contributed to improved cancer specific survival for younger patients.


Asunto(s)
Neoplasias Renales/mortalidad , Nefrectomía , Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Complicaciones Posoperatorias/mortalidad , Adenocarcinoma de Células Claras/mortalidad , Adenocarcinoma de Células Claras/patología , Adenocarcinoma de Células Claras/cirugía , Adenoma Cromófobo/mortalidad , Adenoma Cromófobo/patología , Adenoma Cromófobo/cirugía , Adolescente , Factores de Edad , Anciano , Carcinoma de Células Renales/mortalidad , Carcinoma de Células Renales/patología , Carcinoma de Células Renales/cirugía , Diagnóstico Diferencial , Progresión de la Enfermedad , Femenino , Humanos , Riñón/patología , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/patología , Enfermedades Renales Quísticas/cirugía , Neoplasias Renales/patología , Neoplasias Renales/cirugía , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Complicaciones Posoperatorias/patología , Factores Sexuales , Análisis de Supervivencia
12.
Actas Urol Esp ; 29(1): 74-81, 2005 Jan.
Artículo en Español | MEDLINE | ID: mdl-15786767

RESUMEN

OBJECTIVE: To evaluate the clinical and pathological characteristics of cystic renal tumors in our center. MATERIAL AND METHODS: A retrospective review of 239 nephrectomies is performed comparing the clinical and pathological variables of cystic tumors with those of solid renal tumors. Survival outcomes are analyzed in both groups. RESULTS: Our experience shows that cystic renal tumors behave like solid renal tumors, with no differences in survival shown. The variables studied show statistically significant differences in histological grade and number of tumors, with cystic tumors having a lower histological grade and being more often multiple in number. CONCLUSIONS: The behavior of cystic renal tumors is no different than that of solid renal tumors.


Asunto(s)
Enfermedades Renales Quísticas/patología , Adulto , Anciano , Femenino , Humanos , Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/cirugía , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Nefrectomía , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia
13.
Actas urol. esp ; 29(1): 74-81, ene. 2005. ilus, tab
Artículo en Es | IBECS | ID: ibc-038224

RESUMEN

Objetivo: Valorar las características clínicas y patológicas de los tumores renales quísticos tratados en nuestro centro. Material y métodos: Se realiza una revisión retrospectiva de 239 nefrectomías comparando las variables clínico-patológicas de los tumores quísticos con los tumores renales sólidos. Se analiza la supervivencia en ambos grupos. Resultados: Los tumores renales quísticos se comportan en nuestra experiencia como los tumores renales sólidos, sin presentar diferencias en la supervivencia. Las variables estudiadas muestran diferencias estadísticamente significativas en el grado histológico y la multiplicidad, siendo los tumores quísticos de menor grado y más frecuentemente múltiples. Conclusiones: El comportamiento de los tumores renales quísticos no es diferente al de los sólidos


Objetive: To evaluate the clinical and pathological characteristics of cystic renal tumors in our center. Material and methods: A retrospective review of 239 nephrectomies is performed comparing the clinical and pathological variables of cystic tumors with those of solid renal tumors. Survival outcomes are analyzed in both groups. Results: Our experience shows that cystic renal tumors behave like solid renal tumors, with no differences in survival shown. The variables studied show statistically significant differences in histological grade and number of tumors, with cystic tumors having a lower histological grade and being more often multiple in number. Conclusions: The behavior of cystic renal tumors is no different than that of solid renal tumors


Asunto(s)
Masculino , Femenino , Adulto , Anciano , Humanos , Enfermedades Renales Quísticas/patología , Nefrectomía , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia , Estadificación de Neoplasias , Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/mortalidad , Enfermedades Renales Quísticas/cirugía
14.
Int J Urol ; 8(6): 268-74, 2001 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-11389741

RESUMEN

BACKGROUND: No consistent clinicopathologic characteristics of cyst-associated renal cell carcinoma (CRCC) have previously been determined. METHODS: In total, 768 patients with renal cell carcinoma (RCC) underwent radical or partial nephrectomy. Renal cell carcinoma was classified as CRCC in 27 of these patients (3.5%, subdivided into RCC originating in a cyst and cystic RCC), clear-cell RCC in 662 patients (86.2%), chromophobe cell renal carcinoma in 36 patients (4.7%) and papillary RCC in 43 patients (5.6%) according to the criteria of the World Health Organization. RESULTS: The pathologic stage and nuclear grade were usually lower in those with CRCC (low stage/low grade; 89%/96%) or chromophobe cell renal carcinoma (low stage/low grade; 89%/80%) than in those with clear-cell RCC (low stage/low grade; 59%/65%) or papillary RCC (low stage/low grade; 53%/69%). Of the 27 CRCC patients, only 19 (70%) could be diagnosed through preoperative imaging studies. Patients with CRCC showed a favorable prognosis (survival rate: 95% at 1 year, 89.7% at 3 years and 84.4% thereafter) and, especially among the patients with RCC originating in a cyst, no cancer-related death was observed. Comparing the survival among four types of RCC, a favorable outcome was observed in cases of CRCC or chromophobe cell renal carcinoma compared with clear-cell RCC or papillary RCC (clear vs chromophobe: P = 0.002; chromophobe vs papillary: P = 0.019; clear vs cyst-associated: P = 0.001; papillary vs cyst-associated: P = 0.00079). CONCLUSIONS: In cases of CRCC, the disease was usually detected at lower stages and grades and therefore the prognosis was better than in cases of other types of RCC. Preoperative diagnosis of this disease was very difficult, especially in cases of RCC originating in a cyst.


Asunto(s)
Carcinoma de Células Renales/patología , Enfermedades Renales Quísticas/patología , Neoplasias Renales/patología , Adenocarcinoma de Células Claras/mortalidad , Adenocarcinoma de Células Claras/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma Papilar/mortalidad , Carcinoma Papilar/patología , Carcinoma de Células Renales/mortalidad , Niño , Femenino , Humanos , Enfermedades Renales Quísticas/mortalidad , Neoplasias Renales/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Tasa de Supervivencia , Tomografía Computarizada por Rayos X
15.
Kidney Int ; 51(1): 261-9, 1997 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-8995741

RESUMEN

Familial juvenile nephronophthisis (NPH), an autosomal recessive cystic disease of the kidney, is the most common genetic cause of end-stage renal disease (ESRD) in the first two decades of life. A gene locus for nephronophthisis type 1 (NPH1) has been mapped by linkage analysis to chromosome 2q13. We performed a haplotype analysis in 16 NPH families with at least two affected patients with the typical history, clinical signs and histology of NPH using microsatellite markers of the NPH1 genetic region. By demonstration of a recombinant event marker D2S1893 was identified as a novel centromeric flanking marker to the NPH1 critical genetic region. Absence of linkage to the NPH1 locus in six NPH families confirmed the existence of at least one additional gene locus for NPH. Linkage to the NPH1 locus was demonstrated in 10 families. In 8 of these families a homozygous deletion was identified. These data permit for the first time the study of the development of renal failure in a subset of NPH1 families, which is most likely homogeneous with regard to the responsible gene locus. We present a statistical description of serial serum creatinine measurements in NPH1. Analysis of renal death revealed a median of 13.1 years. Age-dependent quartiles were generated for serum creatinine. In summary, the new marker provides a diagnostic tool to aid in the diagnosis of NPH, while the progression charts offer a standard for an assessment of the rate of progression to ESRD for patients with NPH1 to be used in future therapeutic trials and for a prediction of the individual course of the disease.


Asunto(s)
Enfermedades Renales Quísticas/genética , Fallo Renal Crónico/genética , ADN Satélite/análisis , Progresión de la Enfermedad , Europa (Continente) , Femenino , Marcadores Genéticos , Genotipo , Haplotipos , Humanos , Enfermedades Renales Quísticas/mortalidad , Fallo Renal Crónico/mortalidad , Masculino , Linaje , Análisis de Supervivencia
16.
Kidney Int ; 48(1): 207-19, 1995 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-7564081

RESUMEN

Acquired cystic kidney disease (ACKD) increases the risk of renal malignancy, and many authors suggest routine screening of dialysis patients for ACKD and renal tumors. However, they have defined neither the target population, the optimal screening strategy, the magnitude of its benefit, nor its risk. We used decision analysis to evaluate strategies of performing either computed tomography (CT) or ultrasound every three years on all dialysis patients and annually on patients found to have cysts. We compared these strategies to a strategy of seeking cysts and cancer only if these are clinically suspected. The baseline analysis shows that both CT and ultrasound may decrease cancer deaths by half for patients with a life expectancy of 25 years. Screening for ACKD offers these patients as much as a 1.6 year gain in life expectancy. However, for the majority of patients beginning renal replacement therapy, age or comorbid disease substantially limits life expectancy. For such patients, the gain in life expectancy from an ACKD screening program is measured in days. Sensitivity analyses show that the benefit of screening depends on the rate of malignant transformation, which needs better definition. The gain in life expectancy does not appear to be large enough to justify an ACKD screening program for the entire ESRD population. However, for the youngest and healthiest patients, a screening program would be of benefit. The magnitude of this benefit is uncertain, because the analysis was consistently biased in favor of the screening strategies.


Asunto(s)
Árboles de Decisión , Enfermedades Renales Quísticas/diagnóstico , Fallo Renal Crónico/complicaciones , Neoplasias Renales/mortalidad , Tamizaje Masivo , Diálisis Renal , Técnicas de Apoyo para la Decisión , Humanos , Enfermedades Renales Quísticas/complicaciones , Enfermedades Renales Quísticas/etiología , Enfermedades Renales Quísticas/mortalidad , Neoplasias Renales/diagnóstico , Neoplasias Renales/etiología , Esperanza de Vida , Modelos Lineales , Cadenas de Markov , Vigilancia de la Población , Sensibilidad y Especificidad , Análisis de Supervivencia
17.
J Urol ; 140(5 Pt 2): 1231-4, 1988 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-3054164

RESUMEN

We analyzed 25 cases of multicystic kidney to assess the impact of prenatal diagnosis on the management of this condition. The incidence of unilateral multicystic kidney was 1 in 4,300 live births. Of the 23 children with unilateral multicystic kidneys only 3 (13 per cent) had a readily palpable lesion. These findings suggest that multicystic kidney is a more common renal anomaly than was previously recognized and that the majority of cases remained undiagnosed before the advent of prenatal diagnosis. Ultrasound re-evaluation in 11 children suggests that the natural history of multicystic kidneys is towards spontaneous involution. Two kidneys were not identifiable by followup ultrasound. Hypertension and malignancy complicating multicystic kidney are reported infrequently. The literature on hypertension does not generally support the view that multicystic kidney poses a significant risk of hypertension in later childhood or adult life. We believe that the routine removal of multicystic kidneys in infancy is no longer appropriate.


Asunto(s)
Enfermedades Renales Quísticas/terapia , Riñón/anomalías , Nefrectomía , Estudios de Evaluación como Asunto , Estudios de Seguimiento , Humanos , Recién Nacido , Riñón/cirugía , Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/mortalidad , Diagnóstico Prenatal , Renografía por Radioisótopo , Ultrasonografía
20.
Surg Gynecol Obstet ; 145(5): 733-6, 1977 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-910216

RESUMEN

Surgical exploration for a renal mass is a procedure associated with a low mortality and morbidity, even in a population with a median age of 58 years, most of whom were listed according to the American Society of Anesthesiologists as class II or greater. As a consequence, the procedure warrants serious consideration as a diagnostic measure. Nonsurgical diagnostic procedures are associated with less immediate risk to the patient but with less immediate risk to the patient but with more limited accuracy. The decision as to which methods of evaluation to use in each particular patient must be individualized. The general and related symptoms, physical and laboratory findings and the quality and results of roentgenography and puncture studies of the patient warrant consideration in recommending a reasonable course. Whatever procedures are recommended, the patient should be adequately informed to recognize that alternative treatment approaches are available.


Asunto(s)
Enfermedades Renales Quísticas/cirugía , Complicaciones Posoperatorias/epidemiología , Chicago , Humanos , Enfermedades Renales Quísticas/diagnóstico , Enfermedades Renales Quísticas/mortalidad , Persona de Mediana Edad , Estudios Retrospectivos
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...