RESUMEN
Membranoproliferative glomerulonephritis (MPGN) is the most typical Hepatitis C virus (HCV)-associated glomerulopathy, and the available data about the utilization of direct-acting antivirals (DAA) in HCV-associated glomerulonephritis is inadequate. We evaluated the renal and viral response in two cases of HCV-related MPGN; the first caused by cryoglobulinemia while the second was cryoglobulin-negative. Both patients received immunosuppression besides DAA in different regimens. They achieved partial remission but remained immunosuppression-dependent for more than 6 months after DAA despite sustained virological response, which enabled safer but incomplete immunosuppression withdrawal. Both patients were tested for occult HCV in peripheral blood mononuclear cells and found to be negative. Hence, the treatment of HCV-related MPGN ought to be according to the clinical condition and the effects of drug therapy. It is important to consider that renal response can lag behind the virological response.
Asunto(s)
Glomerulonefritis Membranoproliferativa , Glomerulonefritis , Hepatitis C Crónica , Hepatitis C , Antivirales/uso terapéutico , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis Membranoproliferativa/tratamiento farmacológico , Glomerulonefritis Membranoproliferativa/etiología , Hepacivirus , Hepatitis C/complicaciones , Hepatitis C/tratamiento farmacológico , Hepatitis C Crónica/tratamiento farmacológico , Humanos , Leucocitos MononuclearesRESUMEN
Nearly 20% of SLE corresponds to the pediatric population, and 75% of them have kidney involvement representing an important etiology of chronic kidney disease. A correlation between SLE and ANCA-associated vasculitis has been identified as an overlapping syndrome. Kidney allograft recurrence is rare in SLE when disease control is achieved and with nowadays immunosuppression treatment. Histologic transformation is unusual, especially when there are negative serologic markers and no immune complex deposition reported in native kidneys. A 17-year-old female with crescentic glomerulonephritis, p-ANCA-positive antibodies with pauci-immune pattern in kidney biopsy develops end-stage renal disease requiring hemodialysis. Deceased donor kidney transplant was performed receiving triple immunosuppression thereafter. Thirteen months later serum creatinine rises without evidence of infection, urinary obstruction, or clinical and serologic disease relapse. Allograft biopsy reports mesangial proliferation and "full-house" immunofluorescence. The role of ANCA in SLE physiopathology is controversial, and its relation with lupus nephritis is also discordant. ANCA could represent an important factor in the heterogeneity of systemic lupus erythematosus and lupus nephritis.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Glomerulonefritis Membranoproliferativa/etiología , Fallo Renal Crónico/complicaciones , Lupus Eritematoso Sistémico/complicaciones , Nefritis Lúpica/complicaciones , Adolescente , Complejo Antígeno-Anticuerpo/inmunología , Biopsia , Femenino , Humanos , Inmunosupresores/uso terapéutico , Riñón/patología , Lupus Eritematoso Sistémico/inmunología , Nefritis Lúpica/inmunología , Resultado del Tratamiento , Vasculitis/complicaciones , Vasculitis/diagnóstico , Vasculitis/inmunologíaRESUMEN
Proteinuria is a major feature of lupus nephritis (LN) and reflects podocyte injury. Analysis of podocyte biomarkers was performed attempting to identify if podocyte phenotype is distinct in pure membranous and proliferative LN. Expression of synaptopodin, Wilms tumor protein 1 (WT1), glomerular epithelial protein 1 (GLEPP1) and nephrin was evaluated in 52 LN biopsies by immunohistochemistry. Preserved synaptopodin expression was observed in only 10 (19.2%) of all biopsies while 42 (80.8%) had reduced expression. Both groups had comparable proteinuria at the time of biopsy (p = 0.22); however, in the mean follow-up of four years there was a tendency toward lower mean levels of proteinuria in patients with preserved synaptopodin staining (0.26±0.23 vs. 0.84±0.90 g/24 h, p = 0.05) compared with those with diminished expression. Thirty-nine (75%) biopsies were classified as proliferative and 13 (25%) as pure membranous. Comparison of podocyte biomarkers demonstrated a predominance of preserved staining of synaptopodin (69.2%), WT1 (69.2%), GLEPP1 (53.9%) and nephrin (60%) in the pure membranous group whereas only <10% of the proliferative showed preserved expression. Our data suggest that in proliferative forms there seems to occur structural podocyte damage, whereas in the pure membranous the predominant preserved pattern suggests a dysfunctional podocyte lesion that may account for the better long-term prognosis of proteinuria outcome.
Asunto(s)
Proliferación Celular , Glomerulonefritis Membranoproliferativa/etiología , Glomerulonefritis Membranosa/etiología , Nefritis Lúpica/etiología , Podocitos/patología , Proteinuria/etiología , Adulto , Biomarcadores/análisis , Biopsia , Femenino , Glomerulonefritis Membranoproliferativa/metabolismo , Glomerulonefritis Membranoproliferativa/patología , Glomerulonefritis Membranosa/metabolismo , Glomerulonefritis Membranosa/patología , Humanos , Inmunohistoquímica , Nefritis Lúpica/metabolismo , Nefritis Lúpica/patología , Masculino , Proteínas de la Membrana/análisis , Proteínas de Microfilamentos/análisis , Persona de Mediana Edad , Podocitos/química , Pronóstico , Proteinuria/metabolismo , Proteinuria/patología , Proteínas Tirosina Fosfatasas Clase 3 Similares a Receptores/análisis , Factores de Tiempo , Proteínas WT1/análisis , Adulto JovenRESUMEN
El linfoma de células del manto es una forma infrecuente de linfoma no Hodgkin con alta frecuencia de recaídas y peor pronóstico. Los linfomas pueden inducir daño renal de diferentes modos, entre los que se encuentran la infiltración renal y el desarrollo de glomerulopatías. Se presentó un caso en el que se asocia el comienzo clínico de un linfoma de células del manto con infiltración parenquimatosa renal y glomerulonefritis mesangiocapilar sin crioglobulinemia. Este constituye el primer caso reportado con esta asociación y pone en evidencia las diferentes formas de afectación renal del linfoma no Hodgkin y el valor del estudio histológico renal, para el diagnóstico y el pronóstico de las enfermedades oncohematológicas con daño renal(AU)
Mantle cell lymphoma is an infrequent type of non-Hodgkin lymphoma with high relapse rates and poor prognosis. Lymphomas can induce kidney damage in several ways, including renal infiltration and the development of glomerulopathies. A case associated to the clinical onset of a mantle cell lymphoma with lymphocytic infiltration into the renal parenchyma and mesangiocapillary glomerulonephritis without cryoglobulinemia was presented. This is the first case report that describes this association which evidences the different types of renal lesions in no Hodgkin lymphoma and the value of renal histological study for the diagnosis and prognosis of onco-hematologic diseases with kidney damage(AU)
Asunto(s)
Humanos , Masculino , Adulto , Linfoma de Células del Manto/diagnóstico , Linfoma de Células del Manto/complicaciones , Glomerulonefritis Membranoproliferativa/etiología , Neoplasias Renales/secundarioRESUMEN
The nephropathy of monoclonal gammopathies is principally caused by light chain deposits of fragmented immunoglobins. Paraprotein-related renal diseases are associated with such deposits of intact (heavy chain) or fragmentary (light chain) immunoglobins. A condition of pathological light chain deposits is rare and characterized by deposits of fragments of monoclonal immunoglobulins in many organs. Renal deposits occur primarily in glomeruli and tubular basement membranes. This disease is frequently associated with lymphoproliferative disorders. The majority of cases are caused by deposits of kappa light chains. Whereas this disease is most frequently associated with hematologic malignancies, occasionally a case occurs without detectable hematological pathologies; these cases are called idiopathic or primary. They usually manifest themselves as severe renal insufficiencies with nephrotic-range proteinuria. No treatment regime has been clearly established and the prognosis is poor. Herein, the clinical and histological characteristics are described regarding the first case in Colombia of light chain deposit disease without symptoms of malignancy.
Asunto(s)
Glomerulonefritis Membranoproliferativa/etiología , Cadenas kappa de Inmunoglobulina/análisis , Paraproteinemias/complicaciones , Paraproteínas/análisis , Membrana Basal/química , Membrana Basal/ultraestructura , Médula Ósea/patología , Glomerulonefritis Membranoproliferativa/diagnóstico , Glomerulonefritis Membranoproliferativa/metabolismo , Glomerulonefritis Membranoproliferativa/patología , Humanos , Hipertensión/etiología , Fallo Renal Crónico/etiología , Fallo Renal Crónico/terapia , Glomérulos Renales/química , Glomérulos Renales/ultraestructura , Masculino , Persona de Mediana Edad , Paraproteinemias/patología , Diálisis PeritonealRESUMEN
En el presente trabajo se investigaron las alteraciones ultraestructurales en el glomérulo renal de ovejas con hidatidosis. Utilizando microscopía electrónica de transmisión, fueron examinadas muestras renales de 39 ovejas, 34 de ellas con hidatidosis y 5 sin la parasitosis. Adicionalmente, fue realizado un estudio bioquímico a través de dosaje sérico de creatinina, úrea, proteínas totales y albumina. Las alteraciones ultraestructurales identificadas fueron la presencia de depósitos densos mesangiales, subendoteliales e intramembranosos; proliferación de células mesangiales con áreas de esclerosis segmentaria e interposición de células mesangiales con formación de neo-membrana. Durante el estudio bioquímico se observó un aumento significativo de proteínas totales séricas en el grupo experimental en relación con el de control. El estudio demostró que la glomerulonefritis asociada a hidatidosis en ovinos puede ser clasificada en 4 categorías: lesiones mínimas, glomerulonefritis mesangial, glomerulonefritis segmentaria y focal, y glomerulonefritis membranoproliferativa, siendo predominantemente proliferativa y mesangial
Asunto(s)
Animales , Equinococosis/complicaciones , Glomerulonefritis Membranoproliferativa/etiología , Glomérulos Renales/ultraestructura , Equinococosis/sangre , Echinococcus/patogenicidad , Glomérulos Renales/parasitología , Microscopía Electrónica , Ovinos/parasitologíaRESUMEN
An 11-year-old girl with an abdominal desmoplastic round cell tumor, treated with chemotherapy, presented with gross hematuria and proteinuria. Renal biopsy revealed type I membranoproliferative glomerulonephritis (MPGN). The association of a malignant tumor and MPGN is extremely unusual in children, and the pathogenesis of the renal lesion under these circumstances is unknown.
Asunto(s)
Neoplasias Abdominales/complicaciones , Glomerulonefritis Membranoproliferativa/etiología , Sarcoma de Células Pequeñas/complicaciones , Neoplasias Abdominales/tratamiento farmacológico , Neoplasias Abdominales/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Niño , Femenino , Glomerulonefritis Membranoproliferativa/patología , Humanos , Riñón/patología , Sarcoma de Células Pequeñas/tratamiento farmacológico , Sarcoma de Células Pequeñas/patologíaRESUMEN
La glomerulonefritis membranoproliferativa es causa del 5 a 7 de insuficiencia renal crónica terminal y tiene una alta incidencia de recidiva en el trasplante renal; los hallazgos de de Hipertensión Arterial Sistémica, proteinuria, edema y deterioro progresivo de la función renal sugieren el diagnóstico. Existe una alta correlación con niveles de complemento y factor C 3 nefrítico. El diagnóstico definitivo se basa en los hallazgos histológicos característicos como: engrosamiento de la membrana basal glomerular, proliferación mesangial y formación de dobles contornos, en la microscopía de luz, así como acúmulo de depósitos densos subendoteliales (tipo I) o endoteliales (Tipo II) en microscopía electrónica. La recidiva de la glomerulonefritis membranoproliferativa tipo I es del 20 y 1/3 de estos pacientes pierden el injerto. En el caso de la tipo II la recidiva es del 80 y únicamente 10 pierden el riñón trasplantado. No existe ninguna correlación con niveles de complemento o factor C 3 nefrítico. No existe ningún tratamiento específico para detener la enfermedad. Algunos reportes sugieren que lo más importante es controlar los mecanismos no inmunológicos de progresión del daño renal, tales como hipertensión arterial sistémica, proteinuria e hipercolesterolemia
Asunto(s)
Humanos , Masculino , Adulto , Glomerulonefritis Membranoproliferativa/etiología , Insuficiencia Renal Crónica/complicaciones , Trasplante de RiñónRESUMEN
A case presentation of a patient with SLE associated with papulonodular mucinosis, generalized lymphadenopathy and splenomegaly is reported, along with a review of the literature. This 24 year old woman had biopsy-proven, skin lesions consistent with dermal mucinosis and a non-homogeneous immunofluorescence pattern. Serum anti-nuclear and anti-DNA antibodies were present and a mesangioproliferative glomerulonephritis was documented. The patient responded dramatically to standard therapy with prednisolone. The nature of the mucin deposits and the pattern of immunofluorescence deposition in the skin are discussed based on the findings reported in the literature.
Asunto(s)
Lupus Eritematoso Sistémico/complicaciones , Mucinosis/etiología , Adulto , Biopsia , Femenino , Técnica del Anticuerpo Fluorescente , Glomerulonefritis Membranoproliferativa/etiología , Humanos , Inmunoglobulinas/análisis , Lupus Eritematoso Sistémico/tratamiento farmacológico , Nefritis Lúpica/etiología , Mucinosis/tratamiento farmacológico , Mucinosis/patología , Mucinas/análisis , Prednisolona/uso terapéutico , Piel/química , Piel/patologíaRESUMEN
Impaired renal function was observed in sixteen Aotus nancymai 25 and 3 months following infection with the Uganda Palo Alto strain of Plasmodium falciparum. Decrease were noted in the clearance of endogenous creatinine, creatinine excretion, and urine volume while increases were observed in serum urea nitrogen, urine protein, urine potassium, fractional excretion of phosphorus and potassium, and activities of urinary enzymes. The results were suggestive of glomerulonephropathy and chronic renal disease.
Asunto(s)
Aotus trivirgatus/parasitología , Riñón/fisiopatología , Malaria Falciparum/fisiopatología , Animales , Sangre/parasitología , Creatinina/metabolismo , Electrólitos/metabolismo , Enzimas/orina , Femenino , Tasa de Filtración Glomerular , Glomerulonefritis Membranoproliferativa/etiología , Glomerulonefritis Membranoproliferativa/orina , Pruebas de Función Renal , Malaria Falciparum/complicaciones , Malaria Falciparum/metabolismo , Malaria Falciparum/parasitología , Masculino , Nitrógeno/metabolismo , Proteinuria/etiología , Proteinuria/orinaRESUMEN
Os autores estudaram os rins de 4 cäes infectados con Leishmania (Leishmania) chagasi. Dois animais (um macho e uma fêmea) naturalmente infectados foram sacrificados 18 meses após sua permanencia no laboratório. Dois machos foram inoculados por via endovenosa, com 1x10***6 promastigotas da cepa MHO/BR/70/BH46 e sacrificados após 18 meses e 2 anos, respectivamente. Em todos os animais os rins estavam lesados. As alteraçöes encontradas foram: (1) glomerulonefrite mesangioproliferativa focal ou difusa, com pronunciada hipertrofia e hiperplasia das células mesangiais e com alargamneto da matriz; (2) espessamento da membrana basal com depósitos eletrodensos; (3) nefrite intersticial intertubular crônica com exsudaçäo plasmocitária intensa. (4) degeneraçäo albuminosa dos túbulos renais. Baseados nos achados os autores discutem os prováveis mecanismos patogenéticos
Asunto(s)
Animales , Masculino , Femenino , Perros , Glomerulonefritis Membranoproliferativa/etiología , Riñón/ultraestructura , Leishmaniasis Visceral/complicaciones , Nefritis Intersticial/etiología , Riñón/patologíaRESUMEN
Schistosomiasis mansoni has been well documented as one of the causes of infectious glomerulopathy, with mesangiocapillary glomerulonephritis being the most frequent lesion observed in this condition. Twenty-one patients with hepatosplenic schistosomiasis mansoni and biopsy-documented mesangiocapillary glomerulonephritis (MCGN) were studied and compared with 19 patients with the idiopathic form of MCGN. Nephrotic syndrome was the most frequent clinical presentation in both groups. At the time of diagnosis nine patients with hepatosplenomegaly (4 with associated arterial hypertension) and 12 (8 with arterial hypertension) among the patients with idiopathic MCGN had renal insufficiency. At the end of the follow-up period 16 patients with hepatosplenic schistosomiasis and MCGN (75.2 months) and 15 with the idiopathic form (52.1 months) had renal failure. Also, when compared at 48 months of follow-up, no difference in renal function could be detected in both groups. No benefits related to anti-parasitic treatment in the schistosomiasis group and immunosuppression therapy in either group could be documented. The progression of the renal disease, as assessed by the reciprocal of serum creatinine versus time, and the survival curve, were not different between the two groups. It is concluded that MCGN in patients with the hepatosplenic form of schistosomiasis mansoni is a progressive disease not influenced by anti-parasitic or immunosuppressive therapy, and presents a clinical course similar to that of the idiopathic form.
Asunto(s)
Glomerulonefritis Membranoproliferativa/terapia , Hicantona/uso terapéutico , Síndrome Nefrótico/terapia , Nitroquinolinas/uso terapéutico , Oxamniquina/uso terapéutico , Esquistosomiasis mansoni/tratamiento farmacológico , Tioxantenos/uso terapéutico , Adulto , Antihipertensivos/uso terapéutico , Ciclofosfamida/uso terapéutico , Dieta Hiposódica , Diuréticos/uso terapéutico , Femenino , Estudios de Seguimiento , Glomerulonefritis Membranoproliferativa/etiología , Glomerulonefritis Membranoproliferativa/fisiopatología , Humanos , Terapia de Inmunosupresión , Riñón/efectos de los fármacos , Riñón/fisiología , Riñón/fisiopatología , Masculino , Síndrome Nefrótico/etiología , Esquistosomiasis mansoni/complicacionesRESUMEN
Two naturally infected dogs (male and female) from Teófilo Otoni (MG-Brazil) were maintained for 18 months in our laboratory. Two other dogs, two months old males were infected with 1 x 10(6) promastigotes of MHO/BR/70/BH46 Leishmania (Leishmania) chagasi strain, endovenous route, and autopsied after 10 months and two years. The main findings concerning the kidney were: (1) focal or diffuse mesangial glomerulonephritis with proliferative and enlargement of mesangial cells; (2) increase in thickness of basement membrane with electron-dense deposits; (3) chronic interstitial nephritis with intense exudation of plasmocytes; (4) cloud swelling of renal tubules. The authors discuss the probable pathogenetic mechanisms.