Pronóstico del síndrome nefrótico primario con debut en la infancia / Prognosis of primary nephrotic syndrome with onset in infancy

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Renal transplantation in children with nephrotic syndrome in the first year of life.

OBJECTIVE: The aim of this work was to analyze the evolution of the 1st renal transplantation in children with nephrotic syndrome in the 1st year of life (NSFL). METHODS: In this retrospective study of 15 patients (8 women and 7 men) with NSFL receiving transplants from 1989 to 2013, 9 had NS of Finnish type, 4 diffuse mesangial sclerosis, 1 minimal changes, and 1 collapsing glomerulopathy. We analyzed the clinical and analytic situation at 4 time points: before dialysis, before transplantation, 3 months after transplantation, and long-term evolution. RESULTS: Mean follow-up was 72.8 months (range, 1 month to 16.9 years); mean age at diagnosis was 2.21 months (range, 0-8.2 months); mean age at onset of replacement therapy was 22.9 ± 16.4 months (range, 3.8-55.4 months); and mean time on dialysis was 14.9 months (range, 2-44 months). Age at transplantation was 3.1 years (range, 1.8 to 7.7 years), with 6 living-donor transplantations (LDTs) and 9 cadaveric (CDTs). Ten patients required nephrectomy before transplantation (9 bilateral) to control proteinuria after 3.1 ± 3.8 months on dialysis, 1 during transplantation, and 3 after transplantation (2 persistent proteinuria, 1 hypertension). Mean time on dialysis for LDTs was 5.4 ± 2.7 months versus 13.2 ± 6.9 months for CDTs (P < .005). Mean age of cadaveric donors was 6.2 ± 2.4 years and that of living donors 35.5 ± 7.9 years. As complications, there was 1 bleeding from venous anastomosis and 1 urinary leakage after surgery. After 6 ± 5.2 years of evolution, actuarial survival at both 1 and 7 years was 92.9%. One graft was lost owing to acute rejection 1 month after transplantation and 2 others owing to chronic rejection >9 years after transplantation. None had disease recurrence. CONCLUSIONS: Short-term complications did not differ from the rest of population if transplantation occurred with standard albumin levels, for which most required pre-transplantation nephrectomy because dialysis failed to reduce proteinuria.

BK virus infection in pediatric renal transplantation.

BACKGROUND: Polyomavirus BK (BKV) is a common complication after renal transplantation and an important cause of graft loss. The purpose of this study was to determine the incidence of BKV infection (viremia) in our population and to describe clinical features, global outcomes, and potential correlations with clinical or epidemiologic factors. METHODS: This retrospective single-center study included 84 pediatric recipients of kidney transplantation from January 2006 to September 2012. BKV infection screening consisted of periodic determination of decoy cells in urine samples, confirmed by means of quantitative polymerase chain reaction test in blood. RESULTS: Twenty-two patients (26%) developed BKV viremia. BKV replication appeared early after renal transplantation (median, 2 months). One-third of patients remained asymptomatic, and 27% presented elevated serum creatinine. Immunosuppression was reduced in 90% of patients, and 83% achieved clearance of viremia within 6 months. There was only 1 case of histologically confirmed BKV nephropathy, which evolved to graft loss despite leflunomide, intravenous immunoglobulins, and mycophenolate discontinuation. Risk of BKV viremia was associated with younger age at transplantation (5.9 y vs 10.9 years; P = .001) and cadaveric donor (relative risk, 3.2; P < .05). BKV infection did not affect short-term renal function and graft survival. CONCLUSIONS: BKV viremia is very common in the pediatric renal transplant population, especially in younger children and in those receiving a kidney from cadaveric donors. It develops in the 1st months after transplantation. Reduction of immunosuppression seems to be a good therapeutic option, with high rates of clearance of the infection, although the only patient with confirmed BKV nephropathy had poor outcome.

Servicio de nefrología Infantil del Hospital Universitario la Paz / Pediatric Nephrology Department in the Hospital Universitario La Paz

El servicio de Nefrología Infantil, desde sus inicios como Unidad, ha ido adaptándose a los progresos en el campo de la nefrología clínica, la diálisis y el trasplante. Podemos ofertar todas las técnicas de terapia sustitutiva con una amplia experiencia y muy buenos resultados. Asimismo, contamos con equipos multidisciplinarios para atender de forma integral al niño con estas patologías (AU)

Nephrology Pediatric Service has adapted to the progress in the field of clinical nephrology, dialysis and transplantation. We can offer all kind of replacement therapy techniques with extensive experience and good results. We also have multidisplinary teams to address comprehensively the child with these pathologies (AU)

[Henoch-Schönlein purpura. Study of factors associated with the development and course of the disease]. / Púrpura de Schönlein-Henoch. Estudio de factores asociados con el desarrollo y evolución de la enfermedad.

INTRODUCTION: Henoch-Schönlein purpura (HSP) is the most common form of pediatric vasculitis. The objective of this study was to determine the factors associated with the development and course of this disease. PATIENTS AND METHODS: A case-control study was performed. The case group included patients with HSP followed-up at the pediatric rheumatology and nephrology units of a tertiary university hospital over a 2-year period. The control group included children followed-up at the pediatric rheumatology unit for mechanical or non-inflammatory conditions. A medical history including data on infectious conditions and previous medication was taken. A throat culture was performed and antistreptolysin 0 levels were quantified. The seroprevalence of different viruses was investigated. Subsequently, the patients were prospectively followed-up and disease manifestations were compared with reported epidemiological factors. RESULTS: Seventy patients and 58 controls were studied. A history of a recent upper respiratory infection (URI) and antibiotic intake were independently associated with development of HSP. Palpable purpura was present in 100 % of the patients. Gastrointestinal manifestations were recorded in 63 %, articular in 50 %, and renal in 18.6 %. Arthralgias were more frequent in girls and purpura duration was longer when disease onset occurred in spring or summer. Other factors studied were not associated with disease development or with a worse clinical course. CONCLUSIONS: Factors associated with the development of HSP were a history of URI and antibiotic administration. Other epidemiological factors studied were not associated with either the development or the course of the disease.

Púrpura de Schönlein-Henoch. Estudio de factores asociados con el desarrollo y evolución de la enfermedad / Henoch-Schönlein purpura. Study of factors associated with the development and course of the disease

Introducción: La púrpura de Schönlein-Henoch (PSH) es la vasculitis más frecuente en la infancia. El objetivo fue investigar posibles factores asociados con el desarrollo y evolución de la enfermedad. Pacientes y métodos: Se realizó un estudio caso-control. Los casos fueron los pacientes con PSH vistos en Reumatología y Nefrología Pediátrica de un hospital terciario durante 2 años y los controles los atendidos en Reumatología Pediátrica por problemas mecánicos o funcionales. Se recogieron antecedentes infecciosos, ingesta de medicación previa y se investigó la seroprevalencia de diferentes virus. Se realizó frotis faríngeo y se cuantificó la cifra de antiestreptolisina O. Posteriormente los pacientes fueron seguidos de forma prospectiva y se compararon las diversas manifestaciones de la enfermedad con los factores epidemiológicos referidos. Resultados: El número de pacientes fue 70 y el de los controles 58. Sólo el antecedente de infección del tracto respiratorio superior (ITRS) y la ingesta de antibióticos se asociaron de forma independiente con el desarrollo de PSH. El 100 % de los pacientes presentó púrpura palpable. Las manifestaciones digestivas fueron las más frecuentes (63 %), seguidas de las articulares (50 %) y de las renales (18,6 %). Las artralgias fueron más frecuentes en las niñas y el tiempo de duración de la púrpura fue más prolongado cuando la enfermedad empezó en primavera o verano. Ninguno de los otros factores epidemiológicos estudiados se asoció con el desarrollo de la enfermedad ni con parámetros de peor evolución. Conclusiones: El antecedente de ITRS y la ingesta de antibióticos se asociaron con el desarrollo de PSH. Otros factores epidemiológicos estudiados no se encontraron asociados con el desarrollo ni la evolución de la enfermedad

Introduction: Henoch-Schönlein purpura (HSP) is the most common form of pediatric vasculitis. The objective of this study was to determine the factors associated with the development and course of this disease. Patients and methods: A case-control study was performed. The case group included patients with HSP followed-up at the pediatric rheumatology and nephrology units of a tertiary university hospital over a 2-year period. The control group included children followed-up at the pediatric rheumatology unit for mechanical or non-inflammatory conditions. A medical history including data on infectious conditions and previous medication was taken. A throat culture was performed and antistreptolysin 0 levels were quantified. The seroprevalence of different viruses was investigated. Subsequently, the patients were prospectively followed-up and disease manifestations were compared with reported epidemiological factors. Results: Seventy patients and 58 controls were studied. A history of a recent upper respiratory infection (URI) and antibiotic intake were independently associated with development of HSP. Palpable purpura was present in 100 % of the patients. Gastrointestinal manifestations were recorded in 63 %, articular in 50 %, and renal in 18.6 %. Arthralgias were more frequent in girls and purpura duration was longer when disease onset occurred in spring or summer. Other factors studied were not associated with disease development or with a worse clinical course. Conclusions: Factors associated with the development of HSP were a history of URI and antibiotic administration. Other epidemiological factors studied were not associated with either the development or the course of the disease

[Neonatal pelvic ectasia: long-term outcome and association with ureterovesical anomalies]. / Ectasia piélica neonatal: evolución a largo plazo y asociación a anomalías vesicoureterales.

OBJECTIVE: To analyze the outcome of neonatal pelvic ectasia (PE) and the association between this entity and vesicoureteral reflux and/or other urinary tract abnormalities. PATIENTS AND METHODS: We performed a retrospective study of 255 children (205 boys, 50 girls) with an ultrasonographic diagnosis of PE in the first month of life. The initial ultrasonographic examination was indicated by urinary tract infection in 30 neonates, abnormalities in the prenatal ultrasonographic examination in 150 and by other reasons in 75. Pelvic ectasia was classified in four stages according to anteroposterior pelvic diameter: I < 1 cm, II 1-1.5 cm, III 1.6-2 cm, and IV > 2 cm. RESULTS: Pelvic ectasia was bilateral in 153 children (60 %) and unilateral in 102 (left side in 81.4 % and right side in 18.6 %). Stage I was found in 75.49 %, stage II in 20.34 %, stage III in 3.9 % and stage IV in 0.24 %. The mean follow-up was 32.6 +/- 25.2 months. At the end of the first year, the results of renal ultrasound were normal in 70.2 % of left-sided PE and in 55.9 % of right-sided PE, but 46 patients (18 %) showed worsening of PE between the first and second ultrasound scans. Voiding cystourethrography was performed in 79.6 % of the children and some abnormalities were found in 50 (24.6 %): urethral dilatations in two patients and vesicoureteral reflux in 48. No correlation was found between vesicoureteral reflux and the degree of ectasia (74 % had an anteroposterior diameter of < or = 1 cm). Urinary tract infection was present in 24.3 % of the children and 13 required surgery (eight pyeloplasties, four urethral reimplantations and two resections of type III urethral valves). CONCLUSIONS: Neonatal PE was more prevalent in boys (4:1) and was more frequently located on the left side in both sexes. Associated vesicourethral reflux was found in 23.64 % with no correlation between the degree of dilation and the presence or degree of reflux. Consequently, cystourethrography should be performed in any child with pelvic ectasia, regardless of stage, side or sex.

Ectasia piélica neonatal: evolución a largo plazo y asociación a anomalías vesicoureterales / Neonatal pelvic ectasia: long-term outcome and association with ureterovesical anomalies

Objetivo: Analizar la evolución de la ectasia piélica neonatal y su asociación con reflujo vesicoureteral u otras anomalías del tracto urinario. Pacientes y métodos: Análisis retrospectivo de 255 niños (205 varones y 50 mujeres), con ectasia piélica diagnosticada por ecografía en el primer mes de la vida. Indicación de la primera ecografía: alteración ecográfica prenatal, 150; infección urinaria, 30, y otras, 75. Clasificación en cuatro grados atendiendo al diámetro anteroposterior de la pelvis: grado I, 2 cm. Resultados: En 153 (60 por ciento) la dilatación fue bilateral; de las unilaterales, izquierda 81,4 por ciento, y derecha, 18,6 por ciento. Grado I, 75,49 por ciento; II, 20,34 por ciento; III, 3,93 por ciento y IV, 0,24 por ciento. El tiempo medio de evolución es 32,6 25,2 meses con normalización al año del 70,2 por ciento de las derechas y del 55,9 por ciento de las izquierdas, empeorando 46 (18 por ciento) entre la primera y la segunda ecografía. Se practicó cistouretrografía miccional en 203 (79,6 por ciento), y se encontraron anomalías en 50 (24,6 por ciento): 2 dilataciones uretrales y 48 reflujos vesicoureterales, sin correlación entre reflujo y grado de la ectasia (74 por ciento con diámetro anteroposterior [DAP] <= 1 cm). El 24,3 por ciento han presentado infección urinaria. Intervenciones quirúrgicas: ocho pieloplastias, cuatro reimplantaciones ureterales y dos resecciones de válvulas uretrales tipo III. Conclusiones: La ectasia piélica neonatal predomina en varones (4:1) y en el riñón izquierdo en ambos sexos. Se asocia a reflujo vesicoureteral en el 23,64 por ciento sin correlación entre grado de dilatación y presencia y/o grado de reflujo, por lo que parece aconsejable practicar cistouretrografía miccional independientemente del grado, la lateralidad y el sexo (AU)

Preemptive treatment with oral ganciclovir for pediatric renal transplantation.

This prospective study examines 42 children in the first year after renal transplantation. They all received intravenous ganciclovir prophylaxis for cytomegalovirus in the immediate post-transplant period. Quantitative antigenemia (pp68) determinations and blood, urine and throat cultures were done on a scheduled basis to detect cytomegalovirus. Infection was detected in 22 children (52.4%) within an average 44.31 +/- 27.38 days; 5/22 were symptomatic. The antigenemia was positive (+) in all the infected patients, and so were blood culture in 68.2%, urine culture in 59.1% and throat culture in 31.8%. A positive antigenemia was the earliest finding in all cases but 1. The 5 children with clinical symptoms received intravenous ganciclovir. Asymptomatic infected children received oral ganciclovir at an average dose of 47.64 +/- 8.10 mg/kg/day (median 46.58 (range 33-58.7) mg/kg/day) for an average of 58.47 +/- 27.76 days (median 58 (range 26-211) days). No patient developed disease or ganciclovir resistance during the treatment. No patient presented acute graft rejection or renal dysfunction and their glomerular filtrate rate at 1 year was similar to that of noninfected children (90.38 +/- 26.51 vs. 93.93 +/- 36.24 ml/min/1.73 m2). We conclude that preemptive treatment with oral ganciclovir is useful and safe in children with renal transplantation and that monitoring blood antigenemia is a sensitive and early method to detect and control CMV infection.

[Incomplete Digeorge syndrome (author's transl)]. / Síndrome de Di George incompleto

A newborn with multiple malformations, recurrent infections and hypocalcemic tetany is presented. The malformations included: Facial asymmetry, micrognathia, epicantus, low set nose, peculiar ears, club-feet and heart disease. The immunologic work-up disclosed a cellular immunodeficiency, but normal humoral immunity, and a diagnosis of "incomplete DiGeorge syndrome" was made. No thymus, nor parathyroid glands were found at autopsy. The disagreement between the analytical data and the pathological findings of "complete DiGeorge syndrome" are discussed. The existence of vestigial ectopic thymus not found in the dissected organs is considered the most likely hypothesis. Finally some considerations are made about a possible role of the rubeola virus on its etiology and the actual therapeutic possibilities.