Nuevas alernativas en el tratamiento del síndrome nefrótico idiopático / New alternatives in the treatment of idiopathic nephrotic syndrome

El síndrome nefrótico idiopático (SNI) es una patología propia de la infancia y relativamente frecuente. La mayor parte de los casos son corticosensibles pero un elevado porcentaje de casos evolucionan a la corticodependencia (CD) y precisan largos periodos de tratamiento con distintas drogas, potencialmente tóxicas. Ello hace que estos casos y los corticorresistentes (CR) sean patologías severas y graves por su posibilidad de evolucionar a la enfermedad renal terminal. El tratamiento de elección al diagnóstico de SIN son los esteroides, con distintos protocolos según el tiempo más o menos prolongado de administración. El tratamiento de los casos CD y CR está menos protocolizado y, a pesar de la amplia bibliografía acumulada con distintos protocolos, sigue sin existir evidencia para establecer una indicación clara. Distintos tratamientos se han ido introduciendo a lo largo de los años, todos ellos con capacidad inmunomoduladora sobre la base de la etiología del SNI. Los agentes alquilantes, los anticalcineurínicos, especialmente la ciclosporina y más escasamente el tacrolimus, y más recientemente el micofenolato mofetil y los anticuerpos monoclonales han sido preconizados. Son múltiples los trabajos que muestran respuesta a estos distintos tratamientos, pero muy pocos prospectivos y randomizados y casi inexistentes los comparativos entre los distintos fármacos. En este trabajo hacemos una revisión de los tratamientos más recientes y un apunte sobre otros potenciales tratamientos en las formas graves de síndrome nefrótico corticodependiente (SNCD) y corticorresistente (SNCR) (AU)

Idiopathic nephrotic syndrome (INS) is the most common form of nephrotic syndrome in children. Patients and generally responsive to steroid therapy, but lost of then progress to steroid dependent (SD) disease and require long periods of treatment with different drugs, potentially toxic. Patients who fail to respond to steroids: (steroid-resistant disease (SR)), are at increased risk for developing end-stage renal disease. Treatment of choice for INS is steroids, with different protocols. Treatment of SD cases and SR are less protocolized despite the amount of literature over different protocols, there is still no evidence to establish a clear indication. Different treatments have been introduced over the years, all with immunomodulary capabilities based on the etiology of INS. The alkylating agents, calcineurin inhibitors (cyclosporine), and more recently mycophenolate mofetil and monoclonal antibodies have been used. There are many articles that demonstrate this response to different treatments, but few prospective, randomized and almost nonexistent comparing different drugs. In this paper the latest treatments in severe SD and SR disease are reviewed (AU)

Evolución de la nefropatía de Schönlein-Henochen pacientes pediátricos. Factores pronósticos / No disponible

Objetivo: Analizar los datos epidemiológicos, clínicos y analíticos, así como la curva de supervivencia renal y los factores pronósticos a corto y a largo plazo de niños con nefropatía de Schönlein-Henoch (NSH).Material y método: Estudio clínico de cohorte retrospectivo analítico. Se revisan las historias clínicas de 100 niños diagnosticados de NSH en el HIU La Fe entre 1975 y 2006.Resultados: La manifestación nefrológica más frecuente fue hematuria con proteinuria no nefrótica. El 35% de pacientes fueron biopsiados. La histología más frecuente fue la proliferación mesangial (46%). Los estadios clínicos al diagnóstico fueron Estadio B: 63%, Estadio C: 33%; Estadio D: 4%. Estadios clínicos a los 5 años: Estadio A: 49%, Estadio B: 27%,Estadio C: 0% y Estadio D: 5%. Trasplante renal: 5%. Curva de supervivencia renal (Kaplan Meier) a 5 años: 95%. Factores pronósticos: En el análisis univariante se evidencia que tanto a corto como a largo plazo los factores de mal pronóstico renal fueron la edad superior a 8 años al debut, el número de brotes de púrpura superior a 4 y la presencia de una estadio VI en la histología. El análisis multivariante muestra que a corto plazo únicamente el número de brotes es considerado factor pronóstico. Conclusión: Se pueden considerar como factores pronósticos, tanto a corto como a largo plazo, la edad al inicio de la enfermedad renal, el nº de brotes y la alteración histológica. Sin embargo, en el análisis multivariante únicamente el nº de brotes constituye un factor pronóstico a corto plaz (AU)

Objective: To analyze epidemiological, clinical and laboratory data, renal survival curve and short-term (2 years) and long-term(5 years) prognostic factors in children with nephropathy secondary to Henoch-Schönlein purpura (HSP).Materials and methods: Retrospective analitic cohort study. Clinical records of 100 children diagnosed with HSP at HIU La Fe from 1975-2006 were reviewed. Statistical analysis was by univariate and multivariate analysis. Results: In 67% of cases, nephropathy coincided with onset of the disease and most commonly manifested hematuria with nonnephrotic proteinuria. 35% of patients were biopsied. The most common histology was mesangial proliferation (46%). Clinical stages at diagnosis were stage B: 63%, stage C: 33%;stage D: 4%. Mean follow-up was 5.25 ± 0.76 years. Renal data at 5 years: Clinical stages: stage A: 49%, stage B: 27%, stage C:0%, and stage D: 5%. Renal transplant: 5%. Renal survival curve(Kaplan-Meier) at 5 years: 95%. Prognostic factors: the univariate analysis showed that the prognostic factors of poor renal prognosis in both the short and long-term were age greater than 8 years, number of purpura relapses greater than 4 and presence of stage VI histology. The multivariate analysis showed that only the number of relapses was a short-term prognostic factor. Conclusion: 1) The clinical and laboratory data reviewed were similar to those reported in the literature. 2) The renal survival curve at 5 years was 95%. 3) Age, number of relapses and histology were prognostic factors. 4) The multivariate analysis showed that only the number of relapses was a short-term prognostic factor (AU)

[Ten years of renal transplantation in children]. / Diez años de trasplante renal en niños.

One hundred and fourteen kidney transplants (87 first and 27 second and thirds) were performed in 90 children aged 1 to 15 years during the period 1979-1988; 44% were under 10 years, 14% under 5 and 8% under 3 years of age. The renal grafts were from living related donor (LD) in 30 cases and from cadaveric donor (CD) in 84; 91 were from adult donors and 23 from pediatric ones. The actuarial survival rate of the patients at one and two years was 100% in the LD and 92 and 90% respectively in the CD group. The actuarial survival rate of the first graft at one two years was 98% and 78% in the LD and 83% and 68% in the CD group. When gathered for the receptor age the actuarial survival rate of the first graft was 78 and 67% in the 1-5 years group (n = 19) and 86 and 71% in the 6-15 years one (n = 68). Serum creatinine level was 0.9 +/- 0.4 mg/dl three months, 1.2 +/- 0.7 mg/dl at one year, an 1.8 +/- 1.2 mg/dl at two years among the 50 transplants with a follow-up over two years.

[The direct isotopic cystography in the study of the vesico-ureteral reflux (author's transl)]. / La cistografía isotópica directa en el estudio del reflujo vesicoureteral.

A technique of direct isotopic cystography is presented. It uses 99mTc-DTPA and scintillation camera connected to a computer. The study included a group of children affected of vesico-ureteral reflux; the results are discussed and compared to those of the classical radiological method of voiding cystography. Both techniques seem to complete each other. The radiological method allows a high quality in the definition of the morphological disturbances which causes the reflux. The radioisotope method has a lower radiotoxicity--about 100 times less than voiding cystograph--and high diagnostic sensitivity. The direct isotopic cystography has, in this sense, a broad interest in the periodic control of the vesico-ureteral refluxes treated with conservative procedures.