Development of pediatric hydronephrosis patients visiting the San Vicente Foundation University Hospital, Medellín, Colombia

ABSTRACT: Hydronephrosis is one of the most common congenital malformations detected on prenatal ultrasounds. Moderate and severe cases are often associated with urological abnormality. Objetive: To describe a series of pediatric patients diagnosed with hydronephrosis determining their etiology, prenatal diagnosis and frequency of chronic kidney disease (CKD). Materials and methods: A descriptive, retrospective study. Results: The records of 924 patients between the ages of 0 and 18 years were evaluated, 35.7% female and 64.3% male. In 14.4% (133) the diagnosis was prenatal. Hydronephrosis was bilateral in 198 patients (28.5%). In 18.3% (169) no associated urological abnormality was found, reaching 4.2% in CKD (7). Ureteropelvic stenosis was diagnosed in 23.3% (216) followed with 21.5% VUR (199) and posterior urethral valves in 9.4% (87), reaching 10.2% ERC (93). When the hydroneprhosis was diagnosed by urography, those patients presented 11.3% of chronic kidney disease vs. 8.4% in whom the diagnosis was made by ultrasound, when the hydronephosis diagnosed was by prenatal vs postnatal ultrasound, the percentage of CKD was 4.8% vs 10.8%, respectively. Conclusion:Early diagnosis of hydronephrosis allows the detection of urologic abnormalities susceptible of treatment. Although there are still many questions about which one is the ideal strategy of follow up; the ultrasonography, voiding cystourethrogram, urography, scintigraphy and magnetic resonance urography in selected patients are the most useful tools in order to evaluate urinary tract anomaly

Evolution of pediatric patients diagnosed with hydronephrosis at Hospital Universitario San Vicente Fundación, in Medellín, Colombia between 1960 and 2010

ABSTRACT: Hydronephrosis is one of the most common congenital malformations detected on prenatal ultrasounds. Moderate and severe cases are often associated with urological abnormalities. Objective: To describe the causes of hydronephrosis, and the frequency of its prenatal diagnosis and of chronic kidney disease (CKD) in a series of pediatric patients. Materials and methods: A descriptive, retrospective study of 924 patients under 18 years of age seen at a university hospital in Medellín, Colombia, between 1960 and 2010. Results: 64.3% were male. Diagnosis was prenatal in 133 (14.4%). Hydronephrosis was bilateral in 198 patients (28.5%). In 169 (18.3%) no associated urological abnormality was found; 7 of these (4.2%) reached CKD. Ureteropelvic stenosis was diagnosed in 216 (23.3%) followed by vesicoureteral reflux in 199 (21.5%) and posterior urethral valves in 87 (9.4%); 93 (10.2%) reached CKD. When hydronephrosis was diagnosed by urography, patients developed CKD in 11.3% vs. 8.4% in those whose diagnosis was made by ultrasound; frequency of CKD was 4.8% when diagnosis by ultrasound was prenatal, and 10.8% when it was postnatal. Conclusion: Early diagnosis of hydronephrosis allows the detection of urologic abnormalities susceptible to treatment. There is controversy about the best imaginological method for the follow-up of these patients.RESUMEN: La hidronefrosis es una de las malformaciones congénitas detectadas comúnmente en la ecografía prenatal. Los casos moderados y graves frecuentemente se asocian a anomalías de la vía urinaria. Objetivo: describir las causas de la hidronefrosis, la frecuencia de su diagnóstico prenatal y la de enfermedad renal crónica (ERC) en una serie de pacientes pediátricos. Materiales y métodos: estudio descriptivo, retrospectivo de 924 pacientes atendidos entre 1960 y 2010 en un hospital universitario de Medellín. Resultados: hubo predominio de hombres (64,3%); en el 14,4% el diagnóstico fue prenatal. En el 18,3% no se encontraron anomalías urológicas asociadas. Siete de estos (4,2%) llegaron a la ERC. La estenosis pieloureteral fue la anomalía hallada con mayor frecuencia (28,6%) seguida por el reflujo vesicoureteral (21,5%) y las valvas de la uretra posterior (9,4%); el 10,2% de los pacientes llegaron a la enfermedad renal crónica; al agrupar los pacientes de acuerdo con el método utilizado para el diagnóstico de hidronefrosis, el grupo en que se hizo por urografía excretora tuvo ERC en el 11,3%, en contraste con el 8,4% en quienes el diagnóstico se hizo por ecografía renal; por otro lado. al agrupar los pacientes con diagnóstico ecográfico de hidronefrosis prenatal o posnatal, el porcentaje de ERC fue de 4,8% frente a 10,8%, respectivamente. Conclusión: el diagnóstico temprano de la hidronefrosis permite detectar anomalías urológicas susceptibles de seguimiento o corrección quirúrgica. Todavía existe mucha controversia acerca del mejor seguimiento imaginológico para estos pacientes

Long-term follow-up of pediatric patients with nephrotic syndrome treated at Hospital Universitario San Vicente de Paúl (Medellín (HUSVP), Colombia), between January 1960 and December 2009

ABSTRACT: Nephrotic syndrome (NS) is a glomerular disease that frequently affects children. There have been few studies on it in Colombia. Objective: To describe the clinical and epidemiological features of children with SN treated at HUSVP between 1960 and 2009. Methodology: Retrospective and descriptive study. Results: Steroid-sensitive nephrotic syndrome was diagnosed in 87.9% of the patients, and between 1.7%-5.4% turned steroid-resistant. Biopsies revealed disease with minimal changes in 43.6% and focal segmental glomerulosclerosis in 37.3%. Additional immunosuppressive therapy was required by 40% of the patients; in 88.8% of these, cyclophosphamide was used, and remission was achieved in 85.7%. In 56% of the cases there were complications that were infectious in 52%. Nine percent of the patients progressed to end-stage renal disease. Mortality rate was 5.7%. Discussion: The large number of patients with nephrotic syndrome studied in this series and the long period of follow-up (up to 35 years) provide valuable information about the clinical behavior of this syndrome in Colombia and on its response to immunosuppressive therapy.RESUMEN: El síndrome nefrótico (SN) es una de las enfermedades glomerulares más frecuentes en la infancia y son pocos los estudios realizados en Colombia sobre esta enfermedad. Objetivo: describir las características clínicas y epidemiológicas de los niños con SN atendidos en el Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre los años 1960-2009. Metodología: estudio descriptivo retrospectivo. Resultados: el 87,9% de los pacientes fueron corticosensibles, entre 1,7%-5,4% se tornaron corticorresistentes. La histopatología predominante fue la del síndrome nefrótico con cambios mínimos (43,6%) seguida por la glomeruloesclerosis focal y segmentaria (37,3%). El 40% requirieron inmunosupresión adicional; en 88,8% de estos se utilizó ciclofosfamida con remisión en 85,7%. Se presentaron complicaciones en 56% y 52% de ellas fueron infecciosas. El 9% de los pacientes llegaron a la insuficiencia renal crónica. La tasa de mortalidad fue del 5,7%. Discusión: esta es una de las series más grandes de pacientes con SN reportadas hasta el momento y con un período de seguimiento de hasta 35 años, lo que aporta información valiosa sobre el comportamiento local de la enfermedad y la respuesta al tratamiento inmunosupresor, El seguimiento a largo plazo de estos pacientes es fundamental para ofrecer el mejor tratamiento disponible y nos plantea nuevos interrogantes sobre la evolución del SN en nuestra población

Etiology of chronic kidney disease: fifty-year experience (1960-2010) at the Pediatric Nephrology Service, Hospital Universitario San Vicente de Paúl (Medellín, Colombia)

ABSTRACT: Chronic renal failure (CRF) results from progressive and irreversible deterioration of kidney function; it manifests as the inability to remove waste products and to maintain acid-base balance. The objectives of this study were to identify the diseases responsible for CRF in our patients and their relative importance in such outcome. A descriptive retrospective study was carried out, based on the review of the charts of 367 children with the diagnosis of CRF treated at the Pediatric Nephrology Service (Hospital Universitario San Vicente de Paúl, Medellín, Colombia) between 01 January 1960 and August 30, 2010. One hundred and ninety nine patients (54,2%) were males, and 168 (45,8), females. The predominant diseases as cause of CRF were: vesico-ureteral reflux (37.1%), hydronephrosis (24.0%), posterior urethral valves (13.4%), acute glomerulonephritis (12.3%), nephrotic syndrome (11.2%), and kidney hypoplasia (9.3%).RESUMEN: La insuficiencia renal crónica es el resultado del deterioro progresivo e irreversible de la función renal, que genera incapacidad del riñón para remover los productos de desecho y mantener el equilibrio ácido básico. El objetivo de este estudio fue identificar las causas de insuficiencia renal crónica en nuestros pacientes y su importancia relativa en este desenlace. Se hizo un estudio descriptivo retrospectivo de corte transversal, basado en la revisión de 367 registros pertenecientes al Servicio de Nefrología Infantil, de pacientes con diagnóstico de insuficiencia renal crónica (IRC), que acudieron al Servicio de Consulta Externa del Hospital Universitario San Vicente de Paúl, de Medellín, entre el 01 de enero de 1960 y el 30 de agosto de 2010. De los 367 pacientes, 199 (54,2%) fueron hombres y 168 (45,8%), mujeres; las enfermedades predominantes como causa de la IRC fueron: reflujo vésico-ureteral (37,1%), hidronefrosis (24,0%), valvas de la uretra posterior (13,4%), glomerulonefritis aguda (12,3%), síndrome nefrótico (11,2%) e hipoplasia renal (9,3%)

Use of rituximab in pediatric patients with steroid-resistant nephrotic syndrome. A single center study

ABSTRACT: Steroids are the cornerstone of therapy for nephrotic syndrome (NS) with a remission rate as high as 90%. In patients who do not respond to them or are steroid dependent, other immunosuppressive drugs have been used. Although rituximab use in NS is off-label, many authors have published their experience with it. Objective and methods: To describe retrospectively a group of seven children with nephrotic syndrome, either steroid-dependent (SDNS) or steroid-resistant (SRNS), treated with rituximab and mycophenolate, at Pablo Tobón Uribe Hospital, in Medellín, Colombia. Results: Two patients with SDNS and five with SRNS were evaluated; median age at diagnosis was 2 years (p25-75: 1-5); six months after treatment with rituximab there was reduction in proteinuria (93%), in the steroid dose (100%) and in the relapse episodes. However, proteinuria reappeared 12 months after treatment. Conclusion: During the first year after rituximab treatment of NS there is reduction in proteinuria and in the steroid dose, but thereafter there is relapse. It is suggested to carry out another study using a second dose of rituximab one year after the first one.RESUMEN: Los esteroides son el tratamiento de elección del síndrome nefrótico con tasas de éxito hasta del 90%. En pacientes que no responden adecuadamente a ellos, se han usado diferentes esquemas de inmunosupresión. Objetivo y métodos: describir la respuesta terapéutica en un grupo de siete niños con síndrome nefrótico córtico-dependiente (SNCD) o córtico-resistente (SNCR) que recibieron tratamiento con rituximab y micofenolato mofetil en un hospital universitario de la ciudad de Medellín durante los años 2010-2012. Resultados: dos pacientes tenían SNCD y cinco, SNCR; la mediana de edad en el momento del diagnóstico fue de 2 años (p25-75: 1-5); seis meses después de la aplicación del rituximab se encontró disminución de la proteinuria en el 93% de los pacientes; los esteroides se lograron suspender en el 100%; además, disminuyó el número de recaídas; sin embargo, la proteinuria reapareció un año después de dicho tratamiento. Conclusión: con el rituximab disminuyen la proteinuria y la dosis de esteroides, pero la enfermedad recurre 12 meses después de usarlo. Se sugiere hacer otro estudio evaluando el efecto de una segunda dosis de rituximab al año de la primera

Consenso de expertos sobre recomendaciones basadas en evidencia para el diagnóstico, tratamiento y seguimiento de enfermedad de Fabry en pediatría

Antecedentes: La enfermedad de Fabry (EF) es una enfermedad rara ligada a X secundaria al depósito lisosomal de glicoesfingolípidos debido a la deficiencia de la enzima alfa galactosidasa A (α-Gal A). A pesar de su baja frecuencia, es una condición que afecta la calidad de vida de los pacientes y disminuye su esperanza de vida. Objetivo: Generar recomendaciones informadas en evidencia para el diagnóstico y tratamiento de pacientes pediátricos (menores de 8 años) con EF. Métodos: Se realizó una revisión de literatura en bases de datos y literatura gris a partir del 2010, incluyendo guías de práctica clínica, revisiones sistemáticas, estudios primarios. La calidad de evidencia se evalúo de acuerdo con el tipo de evidencia. Las recomendaciones se sometieron a consenso de expertos a través de metodología Delphi modificada. El acuerdo se definió a partir del 80%. Resultados: A partir del análisis de la evidencia recolectada, se formularon un total de 45 recomendaciones para tamización, diagnóstico y tratamiento de paciente pediátrico con enfermedad de Fabry. El panel revisor estuvo conformado por once expertos en el tema. Las recomendaciones fueron aprobadas con puntuaciones entre 82.3% y 100%. Conclusiones: Las recomendaciones resultantes del consenso de expertos permitirán la toma de decisiones clínicas y estandarización de la práctica en la atención de pacientes pediátricos con EF a nivel nacional y regional; el diagnóstico temprano y oportuno garantiza una disminución del impacto en la calidad de vida de los pacientes y sus familiares. Palabras clave: Enfermedad de Fabry, niños, diagnóstico, terapeutica, biomarcadore

Long-term follow-up of pediatric patients with nephrotic syndrome treated at Hospital Universitario San Vicente de Paúl (Medellín (HUSVP), Colombia), between January 1960 and December 2009 = Evolución a largo plazo de los niños con diagnóstico de síndrome nefrótico atendidos en el Hospital Universitario San Vicente de Paúl, Medellín, entre enero de 1960 y diciembre de 2009

Introduction: Nephrotic syndrome (NS) is a glomerular disease that frequently affects children. There have been few studies on it in Colombia.Objective: To describe the clinical and epidemiological features of children with SN treated at HUSVP between 1960 and 2009.Methodology: Retrospective and descriptive study.Results: Steroid-sensitive nephrotic syndrome was diagnosed in 87.9% of the patients, and between 1.7%-5.4% turned steroid-resistant. Biopsies revealed disease with minimal changes in 43.6% and focal segmental glomerulosclerosis in 37.3%. Additional immunosuppressive therapy was required by 40% of the patients; in 88.8% of these, cyclophosphamide was used, and remission was achieved in 85.7%. In 56% of the cases there were complications that were infectious in 52%. Nine percent of the patients progressed to end-stage renal disease. Mortality rate was 5.7%.Discussion: The large number of patients with nephrotic syndrome studied in this series and the long period of follow-up (up to 35 years) provide valuable information about the clinical behavior of this syndrome in Colombia and on its response to immunosuppressive therapy

Causas de enfermedad renal crónica en niños atendidos en el Servicio de Nefrología Pediátrica del Hospital Universitario San Vicente de Paúl, de Medellín, Colombia, entre 1960 y 2010 = Etiology of chronic kidney disease: fifty-year experience (1960-2010) at the Pediatric Nephrology Service, Hospital Universitario San Vicente de Paúl (Medellín, Colombia)

La insuficiencia renal crónica es el resultado del deterioro progresivo e irreversible de la función renal, que genera incapacidad del riñón para remover los productos de desecho y mantener el equilibrio ácido básico. El objetivo de este estudio fue identificar las causas de insuficiencia renal crónica en nuestros pacientes y su importancia relativa en este desenlace. Se hizo un estudio descriptivo retrospectivo de corte transversal, basado en la revisión de 367 registros pertenecien­tes al Servicio de Nefrología Infantil, de pacientes con diagnóstico de insuficiencia renal crónica (IRC), que acudieron al Servicio de Consulta Externa del Hospital Universitario San Vicente de Paúl, de Medellín, entre el 01 de enero de 1960 y el 30 de agosto de 2010. De los 367 pacientes, 199 (54,2%) fueron hombres y 168 (45,8%), mujeres; las enfermedades predominantes como causa de la IRC fueron: reflujo vésico-ureteral (37,1%), hidronefrosis (24,0%), valvas de la uretra posterior (13,4%), glomerulonefritis aguda (12,3%), síndrome nefrótico (11,2%) e hipoplasia renal (9,3%)

Associated urological malformations and development of chronic kidney disease in pediatric patients with urinary tract infection at San Vicente de Paúl Hospital (Medellín, Colombia) between 1960 and 2010 = Malformaciones urológicas asociadas y desarrollo de enfermedad renal crónica en pacientes pediátricos con diagnóstico de infección urinaria que consultaron al Hospital Universitario San Vicente de Paúl (Medellín, Colombia) entre los años 1960-2010

Introduction: Urinary tract infection (UTI) is a major cause of bacterial disease in the pediatric population. Associated factors such as vesicoureteral reflux (VUR), posterior urethral valves, neurogenic bladder and other anatomical malformations increase the likelihood of developing renal scarring and dysplasia/hypoplasia, which at the same time increase in the long term the risk of hypertension (HT), proteinuria and chronic kidney disease (CKD). Objective: To describe the malformations associated with the frequency of UTI and development of CKD in pediatric patients who consulted San Vicente de Paúl Hospital, in Medellin, Colombia, between 1960 and 2010. Methods: A descriptive, retrospective study in which the clinical records of 4.476 patients with UTI were evaluated. Results: Patients with urinary tract anomalies corresponded to 78.3% of the total (predominance of women: 52.8%). Primary VUR was found in 29.9%; out of these, 5.1% progressed to CKD. Neurogenic bladder was diagnosed in 8.6%, of which 70.8% were secondary to myelomeningocele and 4.9% developed CKD. The posterior urethral valves were present in 3.5% of the total sample, of which 28.5% developed CKD. Conclusion: UTI in the pediatric population is a marker of urinary tract malformation and the prognosis is determined by the presence of VUR, obstructive anomalies and/or renal dysplasia favoring renal scarring, and increasing the risk of hypertension, proteinuria and CKD. An appropriate diagnostic approach would be the basis to implement management strategies to prevent deterioration of renal function

Immunosupressive therapy in children with steroid-resistant nephrotic syndrome: single center experience

INTODUCTION: Nephrotic syndrome is one of the most frequent glomerular diseases among children, and steroid therapy remains as the treatment choice. In spite of this, 10 to 15% of the patients are steroidresistant, and the best therapy for such cases has never been defined. Mycophenolate acid (MA) is one of the treatments used in such situations. OBJECTIVE: To describe the clinical behavior of children diagnosed with steroid-resistant nephrotic syndrome (SRNS) and to assess the therapeutic response to MA. METHODS: This was a retrospective and descriptive study. RESULTS: 26 clinical records of patients with SRNS; 70% male and 30% female. All patients underwent kidney biopsies, which showed a predominance of focal segmental glomerulosclerosis (FSGS). The immunosuppresive drugs used were: Mycophenolate mofetil (MMF) 100%, Cyclosporine 69.2%, Cyclophosphamide 23.1%, and Rituximab 23%. One month after treatment initiation with MMF 61.5% achieved remission. The median of relapses per year for the patients was 3 (p25: 2.75 - p75: 4). This median became 1 (p25: 1 - p75: 3.25) after using this medication (p = 0.08). Furthermore, prior to the start of the MMF treatment, the median of the steroid dose was 1 (p25: 0.5- p75: 1.62) mg/k/day. After using MMF, this median became 0.07 (p25: 0 - p75: 0.55) mg/k/day (p < 0.001), in 8 patients prednisolone was stopped. CONCLUSION: In our experience, treatment with MMF showed positive results such as decrease in the frequency of relapses, less proteinuria, and reduction in the dose of steroids administered without deterioration of glomerular filtration rates. However, more studies are needed to assess efficacy, safety, and optimal dosage