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)

[Results of kidney transplantation in children weighing less than 11 kilograms]. / Resultados del trasplante renal en niños con un peso inferior a 11 kilogramos.

Weigh and age are risk factors of graft failure. The aim of the study is to review the characteristics and the outcome of cadaver renal transplant in children weighing less than 11 Kg. From 1985 to 1999 10 cadaver renal transplant were performed in 10 children (7 boys and 3 girls). Primary renal disease were renal dysplasia(3), posterior urethral valves(5) and congenital nephrotic syndrome(2). All except two suffered end stage disease from birth. The cadaver donor age ranged from 4 to 45 years (mean 12.3). Cold ischaemia time was 14 to 30 hours (median 22.8 h). Grafts were placed extraperitoneally in the iliac fossa in all patients and special care was taken in aggressive intravascular volume expansion. In the first 5 children initial immunosuppression consisted of CyA, Pd and Aza. After 1991, the other five received sequential induction therapy with polyclonal antibodies and triple therapy (CyA, Pd, Aza). Renal function was evaluated as GFR yearly by Swartz formula and the actuarial and graft survival rates were obtained by Kaplan-Meier analysis. Patient survival was 90% at 1 and 10 years. Graft survival was 80% at 1 and 5 years; it decreased to 64% at 7 years. Seven continue with their first graft and the mean follow-up time is 6.6 years. Their renal function measured by the mean of GFR yearly decreased lightly from 102 ml/min/1.73 m2 at 1 year to 87.6 ml/min/1.73 m2 at 5 years. A successful patient and graft survival can be achieved in young receiving kidneys and small reciepients can improve their physical and mental development after transplantation.

Resultados del trasplante renal en niños con un peso inferior a 11 kilogramos / Out of real transplantation in children lessthan 11kg

El peso y la edad del receptor se consideran factores de riesgo para el fallo del injerto. El objetivo de este trabajo es revisar las características y los resultados del trasplante renal de cadaver (TRC)en niños con un peso inferior a los 11K.Entre 1985y 1999 hemos realizado 10 TRC en niños con un peso inferior a los 11K (7 varones y 3 mujeres). La patología primaria causante de la Insuficiencia renal Terminal (IRT) fue : Displasia renal (3pac.),Válvulas de uretra Posterior (5 pac.) y Síndrome Nefrótico congénito (2pac.). Ocho pacientes presentaban IRT desde el nacimiento. El rango de edad del donante cadáver osciló de 4 a 45 años (media 12,3 años). El tiempo de isquemia fría fue de 14 a 30 horas ( media 22,8h). En todos los pacientes el injerto se ha colocado extraperitoneal en fosa iliaca, y se realizó un tratamiento agresivo de expansión del volumen intravascular durante el tiempo perioperatorio. Los 5 primeros trasplantes recibieron como inmunosupresión triple terapia con CyA, Aza, Pred. Después de 1991, se realizó inducción con anticuerpos policlonales y triple terapia en los otros 5 trasplantes. La función renal fue evaluada anualmente con el Filtrado Glomerular (FG) mediante la fórmula de Swartz y la curva de supervivencia del injerto mediante el análisis de Kaplan-Meier.La supervivencia del paciente fue del 90% al año y a los 10 años. La supervivencia del injerto fue del 80% al año y a los 5 años, descendiendo al 64% a los 7 años. Siete niños continúan con su primer injerto funcionante y el tiempo de seguimiento es de 6,6 años. El FG ha ido descendiendo desde 102ml/min/1,73m2al año a 87,6ml/min/1,73m2a los 5 años. El Trasplante Renal (TR) puede realizarse con resultados satisfactorios, tanto para el paciente como para el injerto, en niños con un peso inferior a 11K (AU)

Resultados del trasplante renal en niñosmenores de dos años / Outcome of renal transplantation in children less than two years old

El peso y la edad del receptor se consideran factores de riesgo para el fallo del injerto. El objetivo de este trabajo es revisar las caracteristicas y los resultados del trasplante renal de cadaver (TRC) en niños menores de dos años. Entre 1985 y 1999 hemos realizado 10 TRC en niños con una edad inferior a dos años (7 varones y 3 mujeres). La patología primaria causante de la Insuficiencia renal Terminal (IRT) fue : Displasia renal (3pac.), Válvulas de uretra Posterior (5 pac.) y Síndrome Nefrótico congénito (2 pac.). Ocho pacientes presentaban IRT desde el nacimiento. El rango de edad del donante cadáver osciló de 4 a 45 años (media 12,3 años). El tiempo de isquemia fría fue de 14 a 30 horas ( media 22,8h). En todos los pacientes el injerto se ha colocado extraperitoneal en fosa iliaca, y se realizó un tratamiento agresivo de expansión del volumen intravascular durante el tiempo perioperatorio. Los 5 primeros trasplantes recibieron como inmunosupresión triple terapia con CyA, Aza, Pred. A partir de 1991, se realizó inducción con anticuerpos policlonales y triple terapia en los otros 5 trasplantes. La función renal fue evaluada anualmente con el Filtrado Glomerular (FG) mediante la fórmula de Swartz y la curva de supervivencia del injerto mediante el análisis de Kaplan-Meier. La supervivencia del paciente fue del 90 por ciento al año y a los 10 años. La supervivencia del injerto fue del 80 por ciento al año y a los 5 años, descendiendo al 64 por ciento a los 7 años. Siete niños continuan con su primer injerto funcionante y el tiempo de seguimiento es de 6,6 años. El FG ha ido descendiendo desde 102ml/min/1,73m2 al año a 87,6ml/min/1,73m2 a los 5 años. El Trasplante Renal (TR) puede realizarse con éxito en estos niños e incluso mejorar su desarrollo físico y psíquico (AU)

Situación actual del trasplante renal en el niño / Overview of renal Transplant in children

El Trasplante Renal (TR) es el tratamiento de elección para todos los niños en Insuficiencia Renal Terminal (IRT). Realizamos un análisis de varios factores que influyen de forma significativa en los resultados del TR en el niño: Receptor, donante, inmunosupresión, tratamiento quirúrgico y complicaciones quirúrgicas. También mostramos las curvas de supervivencia del injerto al año y a los 5 años. Desde 1966 los resultados del TR en el niño han mejorado de forma llamativa. El TR ofrece al niño en IRT una excelente rehabilitación y una supervivencia aceptable del injerto a largo plazo (AU)

[Lymphoproliferative disorders of Waldeyer's ring]. / Linfomas primarios del anillo de Waldeyer.

OBJECTIVE: To investigate the incidence and characteristics of lymphoproliferative disorders of Waldeyer's ring in our pediatric patients. MATERIAL AND METHODS: We retrospectively reviewed 20 children under 14 years of age who underwent surgery in our department for adenoidectomy and/or tonsillectomy between 1 January 1996 and 30 November 2000. In non-immunocompromised children, surgical indication was the recent development of progressive unilateral tonsillar hyperplasia, and in immunocompromised patients indication was mainly the enlargement of adenoids and/or tonsils, although eradication of local Ebstein-Barr virus infection and recurrent acute tonsillitis were also indications. RESULTS: The mean age was 4.6+/-2.3 years (range: 16.9 months-13.9 years). Sixteen patients (80.0 %) were male. In the nine patients with unilateral hyperplasia, histopathological diagnosis was diffuse lymphoid hyperplasia. Of the 11 immunocompromised patients, 5 (45.5 %) had some type of lymphoproliferative disorder. DISCUSSION AND CONCLUSIONS: Lymphoproliferative disorders of Waldeyer's ring are relatively frequent in immunocompromised children who have undergone surgery of the adenoids and/or tonsils. Although no cases of unilateral hyperplasia of the tonsils were diagnosed in our series, tonsillectomy is indicated in patients with this diagnosis, independent of their immunological status.

Trasplante renal pediátrico de donante vivo relacionado / Living related donor in pediatric renal transplantation

El donante vivo relacionado (DVR) tiene importantes ventajas cuando se compara con el donante cadáver (DC) en términos de mejor supervivencia del paciente y del injerto, así como de un tiempo de espera menor. Desde 1985 hemos realizado en nuestro Centro 176 trasplantes renales (TR), de los cuales 156 (89 por ciento) son de DC y 20 (11 por ciento) de DVR en primer grado. El objetivo de este trabajo es presentar nuestra experiencia de 5 años con el DVR. A todos los donantes se les realizó un estudio protocolizado de función renal, bioquímico, metabólico y angiográfico. Doce niños recibieron su primer trasplante y 8 fueron retrasplantes ( 6 segundos, 1 tercero y 1 cuarto). El tratamiento inmunosupresor consistió en terapia clásica (azatioprina, ciclosporina prednisona) con inducción de suero antitimocítico. El FK506 y el micofenolato mefetil también se han empleado en alguno de ellos. En cuatro injertos fue necesario reaizar cirug´´ia arterial de banco debido a a presencia de anomalías vasculares. La complicación más signifiativa ha sido a pérdida de un injerto debida a una microangiopatía trombótica asociada al FK506. La supervivencia tanto del donante como del receptor es del 100 por ciento y la supervivencia actuarial del injerto a los 5 años es del 95 por ciento, con un infiltrado glomerular medio de 81,33 ml/min/1,73 m2 (AU)

[Pediatric renal transplantation from related living donor]. / Trasplante renal pediátrico de donante vivo relacionado.

Living related donor (LRD) provides significant advantages when compared with cadaveric donor (CAD) in term of improved patient and graft survival and shorten waiting time. From 1985, 176 kidney transplants were performed at our Center. Of these, 156 (89%) were from CAD and 20 (11%) were from LRD, first degree. The purpose of this paper is to show our experience at 5 years with use of LRD. All donors underwent standardized metabolic workup, angiography assessed and renal function test. Twelve children received their first transplant and 8 were retransplant (6-second, 1-third and 1-fourth). Immunosuppressive therapy consisted of globulin antithymocyte, azathioprine, cyclosporine and prednisolone, using FK506 and mycophenolate mofetil in some of them. Four kidneys with multiple renal arteries were reconstructed ex vivo with microsurgical technique before transplantation. The most significant morbidity was due to FK506-associated thrombotic microangiopathy (TMA) with graft lost. All patients (donor and recipient) survived. Five years graft survival rate is 95% and mean glomerular filtration rate is 81.33 ml/min/1.73 m2.

[value of bone densitometry in pediatric renal transplantation]. / Valor de la densitometría ósea en el trasplante renal pediátrico.

OBJECTIVE: Renal transplantation improves many bone abnormalities inherent to chronic renal insufficiency, but also introduces new deleterious factors, especially those related to immunosuppressive drugs. We have studied the bone mineral content of 40 pediatric renal transplant recipients; moreover, we have analyzed its possible relationships with phosphorous-calcium metabolism, graft function and duration, steroid treatment and growth. PATIENTS AND METHODS: Bone mineral content was measured by dual energy X-ray absorptiometry (DEXA). The results were expressed as a z score in relation to age and sex. Immunosuppression was achieved by using triple therapy: azathioprine, cyclosporine A and prednisolone. At the time of the DEXA, 59% of the patients were on daily steroid treatment and 41% on alternate-day treatment. RESULTS: All patients presented bone mass losses with a maximal decrease between 5 and 21 months post-transplantation and a posterior tendency to recovery. Fifty percent of the children had severe osteopenia (bone mineral density < -1 SD). There was no statistically significant association between the severity of bone loss and values of ionized calcium, phosphorus, intact PTH, or calcitriol received. However, a linear correlation was found between cumulative steroid dose and osteopenia (r = -0.35, p < 0.05). Height and growth velocity were more affected in the severe osteopenia group. In this group, the proportion of children on daily steroid treatment was statistically higher (72% vs 45%, p < 0.05). CONCLUSIONS: Bone densitometry is an accurate, rapid and noninvasive method to measure renal transplant impact on the growing skeleton and it must be done in a periodic and standardized way.

[Should kidney grafts from donors less than 3 years old be used?]. / Se deben utilizar los injertos renales de donantes menores de tres años?

In order to determine the results of renal transplantation using pediatric donors younger than three years of age, a retrospective analysis of a series of 43 transplant recipients was carried out. Methods of immunosuppressive regimen were similar for all patients. They are divided into two groups. Whose donors where younger than three years of age and older than three years. Actuarial graft and patient survival, causes of graft failure, surgical and postoperative complications were compared without significant differences between groups. The results support the contention that pediatric patients younger than three years should be considered to be potential cadaveric kidney donors.

[Peculiarities of urinary infection in 145 infants younger than 3 months of age]. / Peculiaridades de la infección urinaria en 145 lactantes menores de cuatro meses.

Authors have analized retrospectively 145 children with urinary tract infection (UTI) within the first 120 days of life. 67% were males, 45% of patients presented an acute onset with fever and septic appearance and 55 with a chronic illness with digestive symptoms and malnutrition (95%). Breast feeding could be a protective factor against UTI. In 66% of those less than one month old, existed perinatal infectious risk factors. This fact and the presence of septic signs and positive blood cultures (71%), give rise to the possibility that the hematogenous route be the principal infectious via in this age group. The high incidence of parenchymatous participation (75%) have no relation with the presence of obstruction. At onset 29 showed no anomalies on cysto-urography, 48% vesicoureteral reflux of various degrees and 23% obstructive anomalies. The mortality rate was 1,3% due to septic shock.

[Symptomatic urinary infection in infants less than 4 months old: outcome in 129 cases]. / Infección urinaria sintomática en lactantes menores de cuatro meses: evolución de 129 casos.

A series of 129 children with urinary infection during the first 120 days of life, were followed-up for periods ranging from to 1 to 16 years (means = 4 1/12 year). They have been separated in two groups, depending on urocystographic findings: (I) with mild lesions and/or reflux (99 cases) and (II) with obstructive uropathy (30 cases). All the patients in group II and those with reflux of grade IV received surgical treatment. The remaining ones received chemoprophylaxis. During the follow-up, all grade I refluxes disappeared, and so did 86% of grade II and 58% of grade III. Pyelonephritic scars are present in 34% of those kidneys with grade III reflux, 56% of grade IV and 88% of those of the group II. Permanent alteration of renal function was found almost exclusively in patients with grade IV reflux or obstructive anomalies bearing no relation with the number of recurrences of infection during the follow-up.

[Eosinophilic fasciitis]. / Fascitis eosinofílica.

A three year old is described with clinical findings of oedema and induration of skin in the right leg, eosinophilia, hypergammaglobulinemia, and pathological lesions of eosinophilic fasciitis in the deep muscle's fascia. 94 cases published in the literature are reviewed and clinico-biochemical pathological findings and outcome of this syndrome discussed. In general this entity has a good prognosis with steroid therapy. Authors emphasise the rarity of the disease in children, and the early onset in their patient.