RESUMO
La glomerulonefritis rápidamente progresiva (GNRP) es una entidad poco frecuente; el diagnóstico precoz es importante con fines terapéuticos y pronósticos. El objetivo del presente trabajo es reportar la casuística del GNRP en el Servicio de Nefrología del Hospital de Niños "Jorge Lizarraga" entre enero 2004 y enero 2009. Estudio retrospectiva y descriptivo con la revisión de las historias clínicas de 8 pacientes con edades entre 3-13 años. Seis pacientes correspondieron al sexo femenino. La edad promedio fue de 11 ± 3.5 años. La mayoría presentó un foco infeccioso previo con mayor frecuencia en piel (6 casos), y en faringe un caso. Las manifestaciones clínicas y los hallazgos de laboratorio se caracterizaron por: edema, hipertensión, hematuria, retención azoada progresiva, proteinuria e hipocomplementaria. Se realizó biopsia renal en todos los casos, observandose glomerulonefritis proliferativa endo y extracapilar en 6 casos y glomerulonefritis membranoprolirativa en 2 casos. La terapéutica se basó en tratar la infección y el fallo renal agudo. Se aplicaron pulsos de metilprednisolona a todos los pacientes y 6 pacientes ameritaron diálisis peritoneal. Seis pacientes sobrevivieron (2 egresaron con función renal normal y 4 progresaron a enfermedad renal crónica) y 2 pacientes fallecieron. La GNRP es una condición que se presenta esporadicamente; su evolución depende de la severidad del compromiso renal, extensión de las lesiones histopatológicas y precocidad en la terapéutica farmacológica y dialítica. La mayoría de los pacientes sobrevive, un porcentaje importante progresa a enfermedad renal crónica, lo cual eventualmente amerita terapia substitutiva con dialisis y trasplante renal.
Rapidly progressive glomerulonephritis (RPGN) is a rate entity; early diagnosis is important for adequate and prompt treatment. The objetive of this paper is to report the casuistic of RPGN in the Department of Nephrology of the Hospital de Niños "Jorge Lizarraga" between January 2004 and January 2009. Retrospective and descriptive study collecting data of the medical records of 8 pantients, ages 3-13 years. Six patients were females and the average for age was 11 ± 3.5 years. The majority presented a previous infection (skin in 6 and pharynx in 1). Clinical manifestations and laboratory findings were: edema hypertensión, hematuria, progressive azotemic retentions, proteinuria and hypocomplementemia. Renal biopsy was performed in all patients with the following results: endo and extracapillary proliferative glomerulonephritis in 6 cases and membranoproliferative glomerulonephritis in 2. Therapeutic measures were aimed to the treatment of infection acute renal failure. Methylprednisolone boluses were indicated in all patients, 6 patients were submitted to peritoneal dialysis. Six patients survived (2 with normal renal function and 4 with progression to chronic kidney disease), and 2 died. RPGN is a condition that occurs sporadically: its evolution depends of the severity of renal involvement, the extension of histological lesions and the precocity with which pharmacological and dialytic treatment are installed. Most patients survive but a significant number progresss to chronic kidney disease.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adolescente , Anti-Infecciosos/imunologia , Diálise Renal/métodos , Glomerulonefrite Membranoproliferativa/patologia , Glomerulonefrite Membranoproliferativa/terapia , Hematúria/diagnóstico , Biópsia/métodos , Prontuários Médicos , Metilprednisolona/farmacologia , Diálise PeritonealRESUMO
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.