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2.
J Am Soc Nephrol ; 27(4): 1213-24, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26260165

RESUMEN

Noninfectious mixed cryoglobulinemic GN (MCGN) has been poorly investigated. We analyzed presentation and outcome of 80 patients with biopsy-proven MCGN, which were identified in the retrospective French CryoVas survey. MCGN was related to primary Sjögren's syndrome in 22.5% of patients and to lymphoproliferative disorders in 28.7% of patients, and was defined as essential in 48.8% of patients. At presentation, hematuria, proteinuria ≥1 g/d, hypertension, and renal failure were observed in 97.4%, 84.8%, 85.3%, and 82.3% of cases, respectively. Mean±eGFR was 39.5±20.4 ml/min per 1.73 m(2) Membranoproliferative GN was the predominant histologic pattern, observed in 89.6% of cases. Renal interstitium inflammatory infiltrates were observed in 50% of cases. First-line treatment consisted of steroids alone (27.6%) or in association with rituximab (21.1%), alkylating agents (36.8%) or a combination of cyclophosphamide and rituximab (10.5%). After a mean follow-up of 49.9±45.5 months, 42.7% of patients relapsed with a renal flare in 75% of cases. At last follow-up, mean eGFR was 50.2±26.1 ml/min per 1.73 m(2)with 9% of patients having reached ESRD; 59% and 50% of patients achieved complete clinical and renal remission, respectively. A rituximab+steroids regimen prevented relapses more effectively than steroids alone or a cyclophosphamide+steroids combination did, but was associated with a higher rate of early death when used as first-line therapy. Severe infections and new-onset B-cell lymphoma occurred in 29.1% and 8.9% of cases, respectively; 24% of patients died. In conclusion, noninfectious MCGN has a poor long-term outcome with severe infections as the main cause of death.


Asunto(s)
Crioglobulinemia , Glomerulonefritis Membranoproliferativa , Crioglobulinemia/complicaciones , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamiento farmacológico , Ciclofosfamida/uso terapéutico , Femenino , Glomerulonefritis Membranoproliferativa/complicaciones , Glomerulonefritis Membranoproliferativa/diagnóstico , Glomerulonefritis Membranoproliferativa/tratamiento farmacológico , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Rituximab/uso terapéutico
3.
PLoS One ; 10(3): e0119156, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25811382

RESUMEN

BACKGROUND: Chronic lymphocytic leukemia and small lymphocytic lymphoma are 2 different presentations of the most common B-cell neoplasm in western countries (CLL/SLL). In this disease, kidney involvement is usually silent, and is rarely reported in the literature. This study provides a clinicopathological analysis of all-cause kidney disease in CLL/SLL patients. METHODS: Fifteen CLL/SLL patients with kidney biopsy were identified retrospectively. Demographic, clinical, pathological and laboratory data were assessed at biopsy, and during follow-up. RESULTS: At biopsy 11 patients presented impaired renal function, 7 patients nephrotic syndrome, 6 patients dysproteinemia, and 3 patients cryoglobulinemia. Kidney pathology revealed CLL/SLL-specific monoclonal infiltrate in 10 biopsies, glomerulopathy in 9 biopsies (5 membranoproliferative glomerulonephritis, 2 minimal change disease, 1 glomerulonephritis with organized microtubular monoclonal immunoglobulin deposits, 1 AHL amyloidosis). Five patients presented interstitial granulomas attributed to CLL/SLL. After treatment of the hematological disease, improvement of renal function was observed in 7/11 patients, and remission of nephrotic syndrome in 5/7 patients. During follow-up, aggravation of the kidney disease systematically occurred in the absence of favorable response to hematological treatment. CONCLUSIONS: A broad spectrum of kidney diseases is associated with CLL/SLL. In this setting, kidney biopsy can provide important information for diagnosis and therapeutic guidance.


Asunto(s)
Riñón/patología , Leucemia Linfocítica Crónica de Células B/patología , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Demografía , Femenino , Estudios de Seguimiento , Glomerulonefritis/complicaciones , Humanos , Riñón/diagnóstico por imagen , Riñón/metabolismo , Leucemia Linfocítica Crónica de Células B/tratamiento farmacológico , Leucemia Linfocítica Crónica de Células B/metabolismo , Recuento de Linfocitos , Masculino , Persona de Mediana Edad , Síndrome Nefrótico/complicaciones , Radiografía , Recurrencia , Estudios Retrospectivos
5.
Clin Exp Rheumatol ; 29(1 Suppl 64): S63-71, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21586199

RESUMEN

OBJECTIVES: To compare efficacy and tolerance of infliximab versus rituximab to treat refractory Wegener's granulomatosis (WG), and clarify their respective indications. METHODS: Patients with systemic WG refractory to, or intolerant to steroids and consecutive immunosuppressant lines, including oral cyclophosphamide, were randomly assigned to receive infliximab or rituximab and their ongoing regimen. The primary endpoint was partial (PR) or complete remission (CR) at month 12. The secondary endpoint was the occurrence of adverse events. Long-term follow-up data were subjected to post-hoc analysis. RESULTS: Between 2004 and 2007, 9 infliximab and 8 rituximab patients were included. At M12, we observed 2 infliximab and 4 rituximab CR, 1 infliximab and 1 rituximab PR, 5 infliximab and 2 rituximab failures and 2 deaths (NS). Post-hoc analysis was conducted after 30.6±15.4 months of follow-up. Among the 15 survivors, 2 infliximab patients and 1 rituximab patient relapsed. Among 5 infliximab non-responders, 4 were successfully switched to rituximab. During follow-up, one patient from each group died. Over the long term, 10/17 (59%) patients responded to rituximab, 1 to infliximab, 2 to other strategies and 2 died. Despite the 2 deaths, tolerance of both drugs was considered acceptable in terms of WG severity before treatment and previous treatment lines. CONCLUSIONS: Our observations demonstrate the usefulness of infliximab and/or rituximab to obtain remission of refractory WG with a trend at M12 favouring rituximab. During long-term follow-up, rituximab was better able at obtaining and maintaining remission.


Asunto(s)
Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Anticuerpos Monoclonales/uso terapéutico , Granulomatosis con Poliangitis/tratamiento farmacológico , Inmunosupresores/uso terapéutico , Adulto , Anciano , Anticuerpos Monoclonales/efectos adversos , Anticuerpos Monoclonales de Origen Murino/efectos adversos , Distribución de Chi-Cuadrado , Resistencia a Medicamentos , Sustitución de Medicamentos , Femenino , Francia , Granulomatosis con Poliangitis/diagnóstico , Granulomatosis con Poliangitis/mortalidad , Humanos , Inmunosupresores/efectos adversos , Infliximab , Estimación de Kaplan-Meier , Masculino , Persona de Mediana Edad , Selección de Paciente , Estudios Prospectivos , Recurrencia , Inducción de Remisión , Medición de Riesgo , Factores de Riesgo , Rituximab , Índice de Severidad de la Enfermedad , Factores de Tiempo , Resultado del Tratamiento , Adulto Joven
7.
Am J Kidney Dis ; 52(2): 340-5, 2008 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-18572291

RESUMEN

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a systemic arterial disease characterized by impairment of vascular smooth muscle cell structure and function related to NOTCH3 mutations. Pathological findings include pathognomonic granular osmiophilic material (GOM) deposition with nonspecific hyalinization within the artery wall in a variety of tissues. The main clinical presentation is iterative strokes in young adults despite the lack of cardiovascular risk factors, leading to early dementia. Although arteriosclerosis and GOM have been found in kidneys from patients with CADASIL, kidney disease has been described only once up to now, in association with immunoglobulin A nephropathy. We report the case of a 61-year-old patient with a medical history of CADASIL and recent mild hypertension. His mother also showed neuropsychiatric symptoms and end-stage renal disease of unknown cause. The patient had a chronic kidney disease defined by means of estimated glomerular filtration rate using the 4-variable Modification of Diet in Renal Disease Study equation of 58 mL/min/1.73 m(2) associated with mild proteinuria and intermittent microscopic hematuria. Renal histological analysis showed severe arteriosclerosis and mild interstitial fibrosis. Glomeruli did not show mesangial immunoglobulin A deposition or focal segmental proliferation. Electron microscopic analysis showed typical GOM deposition in the vicinity of altered vascular smooth muscle cells in interlobular and juxtaglomerular arteries. The nephroangiosclerosis-like lesions were unusually severe in contrast to the recent mild hypertension. The presence of GOM strongly suggests that renal lesions were related to the NOTCH3 mutation. Here, we describe the first case of familial occurrence of kidney disease with decreased kidney function in the absence of coexisting nephropathy in patients with CADASIL. We discuss the role of NOTCH3 mutation in the pathogenesis of nephroangiosclerosis through functional impairment of renal microcirculation or primary Notch3-related vascular disease.


Asunto(s)
Arterioloesclerosis/genética , CADASIL/genética , ADN/genética , Glomerulonefritis por IGA/complicaciones , Aparato Yuxtaglomerular/irrigación sanguínea , Mutación Missense , Receptores Notch/genética , Arterioloesclerosis/metabolismo , Arterioloesclerosis/patología , Biopsia , CADASIL/complicaciones , CADASIL/diagnóstico , Proliferación Celular , Análisis Mutacional de ADN , Estudios de Seguimiento , Glomerulonefritis por IGA/genética , Glomerulonefritis por IGA/patología , Humanos , Inmunohistoquímica , Imagen por Resonancia Magnética , Masculino , Células Mesangiales/ultraestructura , Microscopía Electrónica , Persona de Mediana Edad , Músculo Liso Vascular/metabolismo , Músculo Liso Vascular/ultraestructura , Receptor Notch3 , Arteria Renal/diagnóstico por imagen , Arteria Renal/metabolismo , Arteria Renal/patología , Circulación Renal , Ultrasonografía Doppler
8.
Arzneimittelforschung ; 55(7): 370-5, 2005.
Artículo en Inglés | MEDLINE | ID: mdl-16080275

RESUMEN

Naftidrofuryl (CAS 31329-57-4) is used, mainly in elderly patients, in the treatment of various vascular disorders. The aim of this study was to evaluate and compare the pharmacokinetics of naftidrofuryl after single oral administration of a 200 mg naftidrofuryl tablet (Praxilene) in caucasian male and female subjects with renal impairment versus healthy volunteers. This prospective and open study was conducted in three parallel groups: Group A = healthy subjects with a Cl(CR) > 80 ml/min, Group B = uraemic patients with a 20 < or = Cl(CR) < 40 ml/min, Group C = uraemic patients with a Cl(CR) < 20 ml/min. Blood samples were taken over a period of 32 h after dosing. The mean values (+/-SD) of the pharmacokinetic parameters of naftidrofuryl for group A were as follows: tmax: 1.3 h (median), Cmax: 174 +/- 46 ng/ml, t(1/2 beta): 4.4 +/- 1.1 h, AUC(0-infinity): 1541 +/- 384 ng x h/ml; for group B: tmax: 2.5 h (median), Cmax: 239 +/- 94 ng/ml, t(1/2 beta): 5.0 +/- 1.2 h, AUC(0-infinity): 2361 +/- 751 ng x h/ml; for group C: tmax: 3.0 h (median), Cmax: 236 +/- 104 ng/ml, t(1/2 beta): 5.0 +/- 2.1 h, AUC(0-infinity): 2488 +/- 2003 ng x h/ml. The statistical analysis was performed on the pharmacokinetic parameters with one-way ANOVA in order to compare each group. No significant difference between each group was observed. In conclusion, renal insufficiency did not appear to influence the pharmacokinetic profile of oral naftidrofuryl.


Asunto(s)
Enfermedades Renales/metabolismo , Nafronil/farmacocinética , Antagonistas de la Serotonina/farmacocinética , Adulto , Cromatografía Líquida de Alta Presión , Creatinina/orina , Femenino , Humanos , Pruebas de Función Renal , Masculino , Nafronil/administración & dosificación , Nafronil/efectos adversos , Estudios Prospectivos , Antagonistas de la Serotonina/administración & dosificación , Antagonistas de la Serotonina/efectos adversos , Comprimidos , Uremia/metabolismo
9.
Rev Prat ; 53(18): 2033-8, 2003 Nov 30.
Artículo en Francés | MEDLINE | ID: mdl-15008218

RESUMEN

Membranous nephropathy is the most common cause of idiopathic nephrotic syndrome in adults. The frequency of secondary forms varies from 20 to 30 per cent. The principal causes appear to be systemic lupus erythematosous, drug therapy, malignancy and viral infection. The pathology includes normocellular glomeruli with subepithelial deposits on the outer surface of the glomerular basement membrane. Immunofluorescence studies reveal consistently IgG granular deposits. Prolonged high-grade proteinuria is common. Renal vein thrombosis is frequently associated. Persistent hyperlipidemia increases the risk of cardiovascular diseases. The course of idiopathic membranous nephropathy remains variable. Numerous factors affecting the prognosis of the nephropathy have been identified and should be considered for the decision of specific treatment and use of immunosuppressive therapy.


Asunto(s)
Glomerulonefritis Membranosa , Adulto , Factores de Edad , Anciano , Anciano de 80 o más Años , Membrana Basal/patología , Niño , Preescolar , Clorambucilo/administración & dosificación , Clorambucilo/uso terapéutico , Ciclofosfamida/administración & dosificación , Ciclofosfamida/uso terapéutico , Ciclosporinas/administración & dosificación , Ciclosporinas/uso terapéutico , Quimioterapia Combinada , Femenino , Técnica del Anticuerpo Fluorescente , Glomerulonefritis Membranosa/complicaciones , Glomerulonefritis Membranosa/diagnóstico , Glomerulonefritis Membranosa/epidemiología , Glomerulonefritis Membranosa/patología , Glomerulonefritis Membranosa/fisiopatología , Glomerulonefritis Membranosa/terapia , Humanos , Hiperlipidemias/complicaciones , Inmunoglobulina G/análisis , Inmunosupresores/administración & dosificación , Inmunosupresores/uso terapéutico , Lactante , Glomérulos Renales/patología , Lupus Eritematoso Sistémico/complicaciones , Nefritis Lúpica/diagnóstico , Masculino , Persona de Mediana Edad , Síndrome Nefrótico/tratamiento farmacológico , Síndrome Nefrótico/etiología , Pronóstico , Proteinuria/etiología , Venas Renales , Factores Sexuales , Trombosis de la Vena/complicaciones
10.
J Am Soc Nephrol ; 12(11): 2358-2369, 2001 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-11675412

RESUMEN

Type IV collagenases matrix metalloproteinase-2 (MMP2) and MMP9 and their related proteins, MT1-MMP, tissue inhibitor of metalloproteinases 1 (TIMP1), TIMP2, and TIMP3, are expressed during kidney morphogenesis and nephrogenesis, but the renal ontogeny of these proteins is only partially known, and their persistence in the adult remains controversial. Their expression was analyzed from early metanephric stages to adulthood by Western blot semiquantitative analysis; laser confocal microscopy of whole-mount kidneys; and a two-step immunoperoxidase labeling procedure using specific markers of proximal tubule (megalin), ascending limb of Henle's loop (Tamm Horsfall protein), and collecting duct (Dolichos biflorus agglutinin lectin). By Western blot, all antigens were detected at day 11.5, peaked at day 16.5, and persisted in the adult at lower levels, although MMP2 was less modulated. All antigens were expressed in metanephric mesenchyme at embryonic day 11.5 and became concentrated in neural cell adhesion molecule-positive-induced mesenchymal cells at day 12.5. Only MT1-MMP and to a lesser extent MMP2 were detected in the ureter bud. At day 16.5, all antigens predominated in the cytoplasm of the proximal tubule, except TIMP1, which was mostly expressed in the ascending limb of Henle's loop and distal tubule. During tubule segmentation, components of the type IV collagenase system showed both spatial and temporal regulation. The distribution of gelatinases was not strictly superimposable to that of their natural inhibitors TIMP, especially for MMP9 and TIMP1. All components persisted in specific segments of the adult renal tubule, where MMP9, MMP2, and MT1-MMP showed an apical expression, suggesting that substrates for these enzymes should be in the tubule lumen or in the apical cell domain and not in the extracellular matrix. These results suggest that a regulated balance of gelatinase activity is required during kidney organogenesis and that gelatinases continue to play a role in adult renal tubule physiology.


Asunto(s)
Riñón/embriología , Riñón/enzimología , Metaloproteinasa 2 de la Matriz/metabolismo , Metaloproteinasa 9 de la Matriz/metabolismo , Animales , Western Blotting , Embrión de Mamíferos/metabolismo , Desarrollo Embrionario y Fetal , Riñón/metabolismo , Túbulos Renales/embriología , Ratones , Ratones Endogámicos C57BL , Distribución Tisular , Inhibidor Tisular de Metaloproteinasa-1/metabolismo , Inhibidor Tisular de Metaloproteinasa-2/metabolismo , Inhibidor Tisular de Metaloproteinasa-3/metabolismo
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