Urachal carcinoma is an uncommon malignancy with a peculiar biomolecular characterization and therefore a complex approach. It was incorporated by the World Health Organization in 2004 in the tumors of the urinary system classification. This neoplasm is generally diagnosed in advanced stages. The standard treatment is surgical, however, due to the rarity and relatively late clinical manifestation of urachal carcinomas, the survival data are mostly case reports, as well as information about medical-surgical treatment based on evidence. The data used were extracted from both the physical and electronic clinical records. Among atypical presentations reported in the literature, we report a case of urachal adenocarcinoma with simultaneous glomerulonephritis as a paraneoplastic syndrome of which there is no report to date. Surgery was carried out in our patient, unfortunately with lifetime morbidity from kidney function replacement secondary to kidney function damage by glomerulonephritis, despite previous immunosuppression treatment for rapidly progressive glomerulonephritis. It is worth mentioning that if the initial diagnosis represents a clinical challenge, treatment is even more complex, given the little information that currently exists about it. Urachal carcinoma is a diagnostic and treatment challenge. Up to now, surgery has been the treatment of choice in localized or locally advanced disease, however, with a high morbidity for the patient.

UNASSIGNED: Renal biopsy has become one of the pillars of nephrology practice, being a very important tool in the diagnostic approach of any nephrology center, since it contributes to the identification and adequate management of glomerular diseases, which represent the third cause of chronic kidney disease in the world. In Mexico there are few reports on the epidemiology of glomerular diseases; therefore, it is important to carry out this type of study.
UNASSIGNED: To identify which are the main glomerulopathies reported in our center, Determine the most prevalent glomerular syndrome according to the reported glomerulopathy, and identify the main complications after percutaneous renal biopsy.
UNASSIGNED: A retrolective cross-sectional study which included patients who underwent percutaneous biopsy of the native kidney from August 2020 to August 2022; we obtained information from the service\'s database.
UNASSIGNED: 89 biopsy reports were analyzed, out of which 66.29% corresponded to the female gender and 32.70% to the male gender. The average age was 37.6 ± 12.86 years. The most frequent primary glomerulopathy was focal segmental glomerulosclerosis (FSG) in 22.5%, followed by membranous nephropathy in 7.9%. The most frequent secondary glomerulopathy was lupus nephropathy in 42.7%. Nephrotic syndrome occurred in 43.85% of cases. 89% of the patients did not present post-biopsy complications.
UNASSIGNED: FSG is the most common primary glomerulopathy. Nephrotic syndrome was the main indication for renal biopsy. The vast majority of patients did not present complications, so we can consider it to be a safe and above all useful procedure for the diagnosis of glomerular diseases.
UNASSIGNED: la biopsia renal es una herramienta muy importante en el abordaje diagnóstico de cualquier centro nefrológico, pues contribuye a identificar y manejar adecuadamente las enfermedades glomerulares, que representan la tercera causa de enfermedad renal crónica en el mundo. En México hay pocos reportes de la epidemiología de las enfermedades glomerulares, por lo que es importante hacer este tipo de estudios.
UNASSIGNED: identificar las principales glomerulopatías reportadas en nuestro centro.
UNASSIGNED: estudio transversal retrolectivo que incluyó reportes histopatológicos de los pacientes a los que se les realizó biopsia percutánea de riñón nativo de agosto de 2020 a agosto de 2022; toda la información se obtuvo del expediente clínico.
UNASSIGNED: se analizaron 89 reportes de biopsias, de las cuales 66.29% correspondían al género femenino y 33.7% al género masculino. La edad promedio fue 37.6 ± 12.86 años. La glomerulopatía primaria más frecuente fue la glomeruloesclerosis focal y segmentaria (GEFyS) en 22.5%, seguida de la nefropatía membranosa 7.9%. La glomerulopatía secundaria más frecuente fue la nefropatía lúpica, con 42.7%. El síndrome nefrótico se presentó en el 43.85% de los casos. El 89% de los pacientes no presentaron complicaciones posteriores a la biopsia.
UNASSIGNED: la GEFyS es la glomerulopatía primaria más frecuente. El síndrome nefrótico fue la principal indicación para realizar biopsia renal. La gran mayoría de pacientes no presentaron complicaciones, por lo que podemos considerar que la biopsia renal es un procedimiento seguro y sobre todo útil para el diagnóstico de las enfermedades glomerulares.

BACKGROUND: Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a multisystemic disease. Despite the improvement in mortality rate since the introduction of immunosuppression, long-term prognosis is still uncertain not only because of the disease activity but also due to treatment associated adverse effects. The neutrophil-to-lymphocyte ratio (NLR) has been demonstrated as an inflammatory marker in multiple settings. In this study, we aimed to investigate the prognostic ability of the NLR in AAV patients.
METHODS: We conducted a retrospective analysis of the clinical records of all adult patients with AVV admitted to the Nephrology and Renal Transplantation Department of Centro Hospitalar Universitário Lisboa Norte from January 2006 to December 2019. NLR was calculated at admission. The outcomes measured were severe infection at 3 months and one-year mortality. The prognostic ability of the NLR was determined using the receiver operating characteristic (ROC) curve. A cut-off value was defined as that with the highest validity. All variables underwent univariate analysis to determine statistically significant factors that may have outcomes. Only variables which significantly differed were used in the multivariate analysis using the logistic regression method.
RESULTS: We registered 45 cases of AVV. The mean age at diagnosis was 67.5±12.1 years and 23 patients were male. The mean Birmingham Vasculitis Activity Score (BVAS) at presentation was 26.0±10.4. Twenty-nine patients were ANCA-MPO positive, 7 ANCA-PR3 positive and 9 were considered negative ANCA vasculitis. At admission, mean serum creatinine (SCr) was 4.9±2.5mg/dL, erythrocyte sedimentation rate (ESR) was 76.9±33.8mm/h, hemoglobin was 9.5±1.7g/dL, C-reactive protein was 13.2±5.8mg/dL and NLR was 8.5±6.8. Thirty-five patients were treated with cyclophosphamide, eight patients with rituximab for induction therapy. Twenty patients developed severe infection within the first three months after starting induction immunosuppression. In a multivariate analysis, older age (73.6±10.5 vs. 62.6±11.3, p=0.002, adjusted OR 1.08 [95% CI 1.01-1.16], p=0.035) and higher NLR (11.9±7.4 vs. 5.9±5.0, p=0.002, adjusted OR 1.14 [95% CI 1.01-1.29], p=0.035) were predictors of severe infection at 3 months. NLR ≥4.04 predicted severe infection at 3 months with a sensitivity of 95% and specificity of 52% and the AUROC curve was 0.0794 (95% CI 0.647-0.900). Nine patients died within the first year. Severe infection at 3 months was independently associated with mortality within the first year (OR 6.19 [95% CI 1.12-34.32], p=0.037).
CONCLUSIONS: NLR at diagnosis was an independent predictor of severe infection within the first 3 months after immunosuppression start, and severe infection within the first three months was consequently correlated with one-year mortality. NLR is an easily calculated and low-cost laboratory inflammation biomarker and can prove useful in identifying AAV patients at risk of infection and poorer prognosis.

暂无摘要。

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a multisystemic disease. Despite the improvement in mortality rate since the introduction of immunosuppression, long-term prognosis is still uncertain not only because of the disease activity but also due to treatment associated adverse effects. The neutrophil-to-lymphocyte ratio (NLR) has been demonstrated as an inflammatory marker in multiple settings. In this study, we aimed to investigate the prognostic ability of the NLR in AAV patients.
We conducted a retrospective analysis of the clinical records of all adult patients with AVV admitted to the Nephrology and Renal Transplantation Department of Centro Hospitalar Universitário Lisboa Norte from January 2006 to December 2019. NLR was calculated at admission. The outcomes measured were severe infection at 3 months and one-year mortality. The prognostic ability of the NLR was determined using the receiver operating characteristic (ROC) curve. A cut-off value was defined as that with the highest validity. All variables underwent univariate analysis to determine statistically significant factors that may have outcomes. Only variables which significantly differed were used in the multivariate analysis using the logistic regression method.
We registered 45 cases of AVV. The mean age at diagnosis was 67.5±12.1 years and 23 patients were male. The mean Birmingham Vasculitis Activity Score (BVAS) at presentation was 26.0±10.4. Twenty-nine patients were ANCA-MPO positive, 7 ANCA-PR3 positive and 9 were considered negative ANCA vasculitis. At admission, mean serum creatinine (SCr) was 4.9±2.5mg/dL, erythrocyte sedimentation rate (ESR) was 76.9±33.8mm/h, hemoglobin was 9.5±1.7g/dL, C-reactive protein was 13.2±5.8mg/dL and NLR was 8.5±6.8. Thirty-five patients were treated with cyclophosphamide, eight patients with rituximab for induction therapy. Twenty patients developed severe infection within the first three months after starting induction immunosuppression. In a multivariate analysis, older age (73.6±10.5 vs. 62.6±11.3, p=0.002, adjusted OR 1.08 [95% CI 1.01-1.16], p=0.035) and higher NLR (11.9±7.4 vs. 5.9±5.0, p=0.002, adjusted OR 1.14 [95% CI 1.01-1.29], p=0.035) were predictors of severe infection at 3 months. NLR ≥4.04 predicted severe infection at 3 months with a sensitivity of 95% and specificity of 52% and the AUROC curve was 0.0794 (95% CI 0.647-0.900). Nine patients died within the first year. Severe infection at 3 months was independently associated with mortality within the first year (OR 6.19 [95% CI 1.12-34.32], p=0.037).
NLR at diagnosis was an independent predictor of severe infection within the first 3 months after immunosuppression start, and severe infection within the first three months was consequently correlated with one-year mortality. NLR is an easily calculated and low-cost laboratory inflammation biomarker and can prove useful in identifying AAV patients at risk of infection and poorer prognosis.

UNASSIGNED: Available biomarkers for monitoring primary glomerulonephritides (GNs), often lack the ability to assess longitudinal changes and have great variability with poor sensitivity. Accruing evidence has demonstrated that Neutrophil Gelatinase-Associated Lipocalin (NGAL), holds promising capacities in predicting renal function worsening in various renal diseases. We aimed at analyzing urinary NGAL (uNGAL) levels in a cohort of individuals with biopsy-proven GNs in order to evaluate its ability to reflect the entity of renal damage and to predict disease evolution overtime.
UNASSIGNED: We enrolled 61 consecutive GNs patients still naïve to pathogenic therapy. uNGAL levels were measured at baseline and patients prospectively followed until the manifestation of a combined outcome of doubling of baseline serum creatinine and/or end-stage kidney disease requiring permanent dialysis support.
UNASSIGNED: Median uNGAL levels were 107[35-312] ng/mL. At univariate and multivariate analyses an inverse correlation was found between eGFR and uNGAL levels (p = 0.001). Progressor subjects showed exceedingly increased baseline uNGAL values as compared with non-progressors (p < 0.001). Twenty-one patients (34%) reached the composite renal endpoint. Subjects with uNGAL values above the optimal, ROC-derived, cut-off of 107 ng/mL experienced a more rapid progression to the renal endpoint (p < 0.001; HR: 5.47; 95% CI 2.31-12.95) with a mean follow-up time to progression of 73.4 vs 83.5 months.
UNASSIGNED: In patients affected by primary glomerulonephritides, uNGAL may represent a real-time indicator of renal damage and an independent predictor of renal disease progression. Further studies on larger populations are warranted to confirm these findings.

Podocyte infolding glomerulopathy (PIG) is a condition of uncertain origin, frequently associated with autoimmune diseases. Its specific treatment and clinical course are unknown. It is characterised by thickening of the capillary walls due to the presence of non-argyrophilic intramembranous bubbles similar to those found in membranous glomerulopathy, but without electron-dense deposits of immune complexes in the ultrastructure, where translucent microspheres generated by invagination of the podocyte cytoplasm into the basement membranes are observed.
Generally reported in young females patients. To date, few cases in Asian patients have been reported. Our case is the first to be reported in a Latin American Caucasian patient.
A 38-year-old woman with SLE. In 2014 she presented with nephrotic syndrome empirically treated with corticosteroids (CO) and intravenous cyclophosphamide with good response. She had a relapse in April 2015 with normal renal function and no extrarenal lupus activity, so she was referred to our hospital to be biopsied.
The biopsy reported focal segmental glomerular sclerosis without deposits of immune complexes in the immunofluorescence. However, methenamine silver staining revealed clear spaces in the capillary walls accompanied by marked podocyte alterations. On electron microscope study, numerous aggregates of microvesicular and cylindrical ultrastructures bound to the membranes were observed, without evidence of dense deposits, and diffuse effacement of pedicel foot processes, confirming the suspected diagnosis.
This is the first reported case of what can be considered a new pathological glomerular entity in a Latin American Caucasian patient, whose clinical course and therapy are still unknown.

The hemophagocytic syndrome is a serious clinical-histological entity secondary to different diseases. Collapsing glomerulonephritis is a proliferative podocytopathy that usually has an unfavorable renal prognosis. We present a case in which both entities were associated, which is an infrequent form of hepatosplenic T-cell lymphoma. In addition, we review the role of the markers of podocyte dedifferentiation in this glomerulopathy and its pathophysiology and treatment.
El síndrome hemofagocítico es una condición clínica e histológica grave, secundaria a diferentes procesos. La glomerulonefritis colapsante es una podocitopatía proliferativa, generalmente de pronóstico desfavorable para la función renal. Se presenta un caso en el que las dos condiciones aparecieron asociadas, lo cual es una forma infrecuente de presentación del linfoma hepatoesplénico de células T. Se discute, asimismo, el papel de los marcadores de desdiferenciación podocitaria en esta glomerulopatía, y se revisan la fisiopatología y el tratamiento.

We report the case of a 47-year-old man with a 9-year history of psoriatic arthritis (PsA) in whom we detected renal involvement, hypocomplementemia, peripheral neuropathy, acral necrotic lesions and positive cryoglobulins. The results of the diagnosis led us to conclude that the clinical picture corresponded to cryoglobulinemic vasculitis concomitant with PsA. In addition, we present a review of the literature on the presence of these two diseases in a single patient.

The prevalence of glomerular disease (GD) varies according to the different socio-demographic characteristics of each population. For the first time we present the prevalence of the different forms of GD among patients from several different areas of Columbia.
Data from 12,613 renal biopsies studied at our University Hospital between 2003 and 2015 was reviewed. Pathology results were classified according to a list of renal diseases proposed by various authors.
Focal segmental glomerulosclerosis was present in 22%, IgA disease in 21%, Lupus nephritis in 17%, membranous glomerulonephritis in 13% and thin basal membrane disease in 9%.
This study confirms that focal segmental glomerulosclerosis and IgA disease are the most prevalent GDs found in Columbian patients. This is the first study to analyze GDs in a Columbian population and we recommend that a national registry system be created to collect comprehensive information from future research.