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1.
J Hematop ; 2024 Jul 20.
Artigo em Inglês | MEDLINE | ID: mdl-39031273

RESUMO

Mantle cell lymphoma (MCL) is a rare and aggressive type of lymphoma that can affect the kidneys. The disease can lead to kidney impairment, and glomerulonephritis (GN) is a rare but serious complication of MCL. We report a case of MCL with kidney interstitial infiltration and membranoproliferative glomerulonephritis with focal and segmental glomerulosclerosis. A 75-year-old man presented recurrent acute kidney failure and worsening of nephrotic syndrome. Kidney biopsy revealed membranoproliferative glomerulonephritis presented immunoglobulin and complement deposition, focal and segmental glomerulosclerosis of not otherwise specified type, and infiltration by mantle cell lymphoma. Bone marrow biopsy and PET/CT scan confirmed the diagnosis of mantle cell lymphoma. The patient was treated with R-CHOP21 chemotherapy with cyclophosphamide dose adjustment for nephroprotection. He achieved complete remission with normalization of hematological parameters, improvement of kidney function, and reduction of proteinuria and albuminuria. This case shows the importance of considering alternative diagnoses in patients with recurrent chronic kidney disease and worsening nephrotic syndrome. Early diagnosis and treatment of mantle cell lymphoma can lead to favorable outcomes.

2.
Medicines (Basel) ; 10(10)2023 Oct 04.
Artigo em Inglês | MEDLINE | ID: mdl-37887262

RESUMO

BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) includes three entities: light chain deposition disease (LCDD), heavy chain deposition disease (HCDD) and light and heavy chain deposition disease (LHCDD). The renal presentation can manifest with varying degrees of proteinuria and/or nephrotic syndrome, microhematuria, and often leads to end-stage renal disease. Given the rarity of LHCDD, therapeutic approaches for this condition remain inconclusive, as clinical trials are limited. CASE PRESENTATION: We report two male patients with underlying monoclonal gammopathy of renal significance (MGRS) associated with LHCDD lesions. Both cases had non-nephrotic proteinuria, moderately impaired renal function, and normal levels of C3 and C4. Light microscopy of the renal biopsies in both patients did not show lesions of nodular glomerulosclerosis. Immunofluorescence showed a staining pattern with interrupted linear IgA-κ in patient #1 and IgA-λ in patient #2 only along the glomerular basement membrane (GBM). Electron microscopy of patient #1 revealed electrodense deposits in the subendothelial and mesangial areas only along the GBM. DISCUSSION: In this case series, we discuss the clinical, analytical, and histopathological findings of two rare cases of LHCDD. Both patients exhibited IgA monoclonality and were diagnosed with monoclonal gammopathy of undetermined significance (MGUS) by the hematology department at the time of renal biopsy. Treatment with steroids and cytotoxic agents targeting the clone cells responsible for the deposition disease resulted in a favorable renal and hematologic response.

3.
Nefrología (Madr.) ; 35(6): 562-566, nov.-dic. 2015. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-145701

RESUMO

Introducción: La supervivencia (SV) comparada en terapia renal sustitutiva (TRS) es dependiente de la comorbilidad previa al inicio de TRS y no de la técnica dialítica. Objetivo: Valorar la SV en nuestra población de TRS habida en el periodo 1976-2012 y asimismo la influencia por la transferencia de técnica (TTc). Material y métodos: Cohorte retrospectiva (n = 993 pacientes). Los datos fueron «censurados» por trasplante (TX), cambio de técnica, defunción o pérdida para el seguimiento. La SV por TTc se realizó en pacientes con más de 12 semanas de permanencia. Resultados: El riesgo de mortalidad ajustado por edad, sexo, técnica dialítica o diabetes mellitus (DM) mostró que el riesgo estimado de morir aumenta un 4,8% por cada año aumentado (HR=1,048, IC del 95%, 1,04-1,06, p<0,001) y este aumenta un 44% en los diabéticos con respecto a los no diabéticos (HR=1,44, IC del 95%, 1,16-1,76, p<0,01). En cuanto a la SV por TTc, los que inician HD presentan SV menor que los que inician DP y son transferidos a HD (p=0,00563). Conclusión: En nuestra experiencia, la SV en TRS es dependiente de la edad y la coexistencia de DM y sería conveniente retomar el concepto de «cuidados integrales» comenzado la TRS por DP y transferir a HD(AU)


Objective: To assess SV in our RRT population in the period 1976-2012 as well as the influence of technique transference (TT). Material and methods: The study included a retrospective cohort of 993 patients. Data were classified as transplant (Tx), change in technique, exitus or lost to follow-up. SV for TT was determined in patients with over 12 weeks of permanence. Results: The mortality risk adjusted for age, sex, dialysis technique or diabetes mellitus (DM) showed that the estimated risk of death increased by 4.8% per year increase (HR=1.048; 95% CI: 1.04-1.06; P<.001) and was 44% higher in diabetics compared to non-diabetics (HR=1.44; 95% CI 1.16-1.76; P<.01). Regarding SV for TT, patients who initiated HD had a shorter survival than those who initiated PD and transferred to HD(P=.00563). Conclusion: In our experience, SV in RRT is dependent on age and coexistence of DM. It would be beneficial to reinstate the concept of 'comprehensive care', in which RRT would start with PD and later transfer to HD (AU)


Assuntos
Humanos , Diálise Renal/estatística & dados numéricos , Diálise Peritoneal/estatística & dados numéricos , Insuficiência Renal Crônica/terapia , Análise de Sobrevida , Terapia de Substituição Renal/estatística & dados numéricos
4.
Nefrología (Madr.) ; 35(6): 572-577, nov.-dic. 2015. tab, ilus, graf
Artigo em Espanhol | IBECS | ID: ibc-145703

RESUMO

Introducción La preeclampsia (PE) es una importante causa de morbimortalidad fetal, que en el mundo occidental afecta al 2-7% de los embarazos y es responsable de 50.000 muertes anuales. La detección precoz es prioritaria, ya que puede cambiar su curso clínico, pero no se dispone de biomarcadores ni métodos instrumentales de alta sensibilidad y especificidad, solamente el índice hiperbárico tiene una sensibilidad y una especificidad del 99% para la identificación precoz de las gestantes en riesgo de desarrollo de PE, pero está escasamente difundido. Objetivo: Valorar la utilidad del índice hiperbárico en la prevención primaria de las complicaciones hipertensivas del embarazo en un área sanitaria. Material y métodos: Estudio retrospectivo realizado entre los embarazos habidos en nuestra área durante el periodo 2007-2012 (N=11.784). El diagnóstico se estableció mediante el índice hiperbárico y las gestantes en riesgo fueron tratadas con AAS nocturno. Resultados: En las gestantes remitidas a consulta de Nefrología (38,2%), diagnosticadas de alto riesgo de PE y tratadas con AAS 100mg nocturno (desde la semana 17) se redujo la incidencia de episodios de PE un 96,94% (AU)


Introduction: Preeclampsia (PE) is a major cause of fetal morbidity and mortality. In the Western World, PE affects 2-7% of pregnancies and is responsible for 50,000 deaths annually. Early detection is a priority as it can change the clinical course, but there are no biomarkers or instrumental methods with high sensitivity and specificity. Only the hyperbaric index has a sensitivity and specificity of 99% for early identification of pregnant women at risk of developing PE, but its use is not widespread. Objective: To assess the usefulness of the hyperbaric index in the primary prevention of hypertensive pregnancy complications in a public healthcare area. Material and methods: This is a retrospective study of pregnancies that occurred in our area during the period 2007-2012 (N=11,784). The diagnosis was established by the hyperbaric index and pregnant women at risk were treated with ASA at night. Results: In pregnant patients referred to the nephrology clinic (38.2%), diagnosed as high-risk for PE, and treated with 100mg ASA/night (from week 17), the incidence of PE episodes was reduced by 96.94% (AU)


Assuntos
Feminino , Humanos , Gravidez , Pré-Eclâmpsia/terapia , Hipertensão Induzida pela Gravidez/prevenção & controle , Hipertensão/complicações , Oximetria , Gravidez de Alto Risco , Complicações na Gravidez , Estudos Retrospectivos , Fatores de Risco
5.
Nefrologia ; 35(6): 562-6, 2015.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26596690

RESUMO

OBJECTIVE: To assess SV in our RRT population in the period 1976-2012 as well as the influence of technique transference (TT). MATERIAL AND METHODS: The study included a retrospective cohort of 993 patients. Data were classified as transplant (Tx), change in technique, exitus or lost to follow-up. SV for TT was determined in patients with over 12 weeks of permanence. RESULTS: The mortality risk adjusted for age, sex, dialysis technique or diabetes mellitus (DM) showed that the estimated risk of death increased by 4.8% per year increase (HR=1.048; 95% CI: 1.04-1.06; P<.001) and was 44% higher in diabetics compared to non-diabetics (HR=1.44; 95% CI 1.16-1.76; P<.01). Regarding SV for TT, patients who initiated HD had a shorter survival than those who initiated PD and transferred to HD (P=.00563). CONCLUSION: In our experience, SV in RRT is dependent on age and coexistence of DM. It would be beneficial to reinstate the concept of "comprehensive care", in which RRT would start with PD and later transfer to HD.


Assuntos
Diálise Peritoneal/mortalidade , Diálise Renal/mortalidade , Transferência de Tecnologia , Uremia/mortalidade , Adulto , Fatores Etários , Idoso , Assistência Integral à Saúde , Nefropatias Diabéticas/mortalidade , Nefropatias Diabéticas/terapia , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Risco , Fatores Sexuais , Espanha/epidemiologia , Uremia/terapia
6.
Nefrologia ; 35(6): 572-7, 2015.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26547790

RESUMO

INTRODUCTION: Preeclampsia (PE) is a major cause of fetal morbidity and mortality. In the Western World, PE affects 2-7% of pregnancies and is responsible for 50,000 deaths annually. Early detection is a priority as it can change the clinical course, but there are no biomarkers or instrumental methods with high sensitivity and specificity. Only the hyperbaric index has a sensitivity and specificity of 99% for early identification of pregnant women at risk of developing PE, but its use is not widespread. OBJECTIVE: To assess the usefulness of the hyperbaric index in the primary prevention of hypertensive pregnancy complications in a public healthcare area. MATERIAL AND METHODS: This is a retrospective study of pregnancies that occurred in our area during the period 2007-2012 (N=11,784). The diagnosis was established by the hyperbaric index and pregnant women at risk were treated with ASA at night. RESULTS: In pregnant patients referred to the nephrology clinic (38.2%), diagnosed as high-risk for PE, and treated with 100mg ASA/night (from week 17), the incidence of PE episodes was reduced by 96.94.


Assuntos
Pressão Sanguínea , Hipertensão Induzida pela Gravidez/prevenção & controle , Pré-Eclâmpsia/prevenção & controle , Gravidez de Alto Risco/fisiologia , Adulto , Aspirina/administração & dosagem , Aspirina/uso terapêutico , Monitorização Ambulatorial da Pressão Arterial , Esquema de Medicação , Diagnóstico Precoce , Feminino , Humanos , Hipertensão Induzida pela Gravidez/diagnóstico , Hipertensão Induzida pela Gravidez/fisiopatologia , Inibidores da Agregação Plaquetária/administração & dosagem , Inibidores da Agregação Plaquetária/uso terapêutico , Gravidez , Resultado da Gravidez , Estudos Retrospectivos , Sensibilidade e Especificidade
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