Renal involvement at diagnosis of pediatric acute lymphoblastic leukemia

Acute leukemia is the most common type of cancer in pediatric patients. This type of cancer accounts for a third of all childhood cancer cases. More than half of pediatric acute leukemia patients show signs and symptoms such as hepatomegaly, splenomegaly, pallor, fever and bruising at the time of diagnosis. In early stages of acute lymphoblastic leukemia (ALL), nephromegaly and other renal manifestations such as high blood pressure (HBP) and renal failure are uncommon, although renal infiltration and nephromegaly are common in advanced-stage pediatric patients. This is a retrospective case review with a critical appraisal of the existing evidence from the literature. We present a clinical case of a child with HBP associated with bilateral nephromegaly which resolved after chemotherapy treatment. This patient presented with HBP that required pharmacological treatment, likely owing to nephromegaly. All HBP secondary causes were rejected. Nephromegaly was resolved after chemotherapy treatment, and antihypertensive medication was discontinued. Nephromegaly and HBP are rare manifestations of ALL debut in pediatrics. The present case report illustrates this unusual combination and Suggests clinicians to consider malignancy as its causal factor, especially if the symptoms are accompanied by other suggestive extrarenal manifestations

Consenso colombiano para el manejo de pacientes con Hipofosfatasia

La hipofosfatasia (HPP) es una enfermedad metabólica, de tipo hereditario, causada por mutaciones en el gen ALPL. Teniendo en cuenta los retos del manejo adecuado de los pacientes con HPP, se realizó un consenso interdisciplinario de expertos (endocrocrinólogos pediatras, nefrólogos pediatras, ortopedistas infantiles y genetistas clínicos) con el fin de proponer recomendaciones de utilidad clínica para el diagnóstico, tratamiento y seguimiento de los pacientes colombianos con HPP. Estas sugerencias se realizan en el contexto de los diferentes tipos de presentaciones y las edades de los pacientes.Q41-7Hypophosphatasia (HPP) is a hereditary metabolic disease caused by mutations in the ALPL gene. Taking into account the challenges found in the adequate management of patients with HPP, an interdisciplinary consensus of experts (pediatric endocrinologists, pediatric nephrologists, pediatric orthopedists and clinical geneticists) was carried out, in order to propose recommendations of clinical utility for the diagnosis, treatment and follow-up of Colombian patients with HPP. These suggestions are made in the context of the different types of presentations and the ages of the patients

Resolución espontánea de reflujo vesicoureteral primario en niños: factores predictores y nomograma de predicción

Introducción: Determinamos la proporción de resolución espontánea de RVU primario en una población de niños menores de 5 años así como los factores que influyen y predicen tal resolución, con base en lo cual diseñamos un nomograma que permite determinar la posibilidad de resolución espontánea de cada grado de reflujo a los 3 años de su diagnóstico\ud Metodología: Incluimos 407 niños con diagnóstico de RVU primario en un periodo de 10 años. Mediante análisis de asociación y comparaciones de promedios se determinaron las variables que se comportaron como factores de riesgo para fallar en obtener resolución espontanea y por un modelo de regresión logística binomial se confirmaron asociaciones. Se practicaron comparaciones mediante ANOVA o t-test y así como análisis de sobrevida mediante Log Rank Test para determinar las variables que influían también en el tiempo necesario para obtener resolución espontánea.\ud Resultados: Las tasas de resolución espontánea fueron 92%, 85%, 56.4%, 21% y 5% para los grados I a V de reflujo respectivamente. En el análisis multivariado, Las variables nefropatía por reflujo (sig=0,000), Síndrome de evacuación disfuncional (SED) (sig=0,000) y bilateralidad (sig=0,006) fueron los factores de riesgo independientes para la falla del RVU en resolver espontáneamente. Sin embargo, en los análisis de sobrevida solo la variable SED demostró influir en el tiempo necesario para obtener resolución espontanea (sig=0,002). \ud Discusión: Los hallazgos de este estudio ratifican la importancia de incluir variables como SED, nefropatía por reflujo y lateralidad en los modelos de predicción de resolución espontánea del RVU.Introduction: To determine primary vesico-ureteral reflux (VUR) resolution rate in a population of children younger than five years of age, as well as, factors predicting such presentation. Furthermore, based on these results, to design a nomogram which allows prediction of spontaneous resolution likelihood within a 3 years period from diagnosis. \ud Methodology: 407 children with primary vesicoureteral reflux diagnosed within a ten years period were included. Through association analysis and average comparison, variables acting as risk factors for spontaneous resolution failure were determined. Additionally, using binomial regression models associations were verified. We practice comparisons through ANOVA or t-test, as well as Log Rank Test survival analysis in order to determine variables affecting spontaneous resolution length.\ud Results: Spontaneous resolution rates were 92%, 85%, 56.4%, 21% and 5% for VUR grade I to V respectively. Multivariate analysis demonstrated that reflux uropathy (sig=0,000), dysfunctional evacuation syndrome (DES) (sig=0,000) and bilateral compromise acted as the principal independent risk factors for spontaneous resolution failure. Nevertheless, survival analysis established that DES was the only risk factor affecting spontaneous resolution length (sig=0,002).\ud Discussion: Our analyses confirm that inclusion of variables as DES, reflux uropathy and bilateral compromise is indispensable for VUR spontaneous resolution prediction

Resolución espontánea de reflujo vesicoureteral primario en niños: factores predictores y nomograma de predicción

Introducción: Determinamos la proporción de resolución espontánea de RVU primario en una población de niños menores de 5 años así como los factores que influyen y predicen tal resolución, con base en lo cual diseñamos un nomograma que permite determinar la posibilidad de resolución espontánea de cada grado de reflujo a los 3 años de su diagnóstico Metodología: Incluimos 407 niños con diagnóstico de RVU primario en un periodo de 10 años. Mediante análisis de asociación y comparaciones de promedios se determinaron las variables que se comportaron como factores de riesgo para fallar en obtener resolución espontanea y por un modelo de regresión logística binomial se confirmaron asociaciones. Se practicaron comparaciones mediante ANOVA o t-test y así como análisis de sobrevida mediante Log Rank Test para determinar las variables que influían también en el tiempo necesario para obtener resolución espontánea. Resultados: Las tasas de resolución espontánea fueron 92%, 85%, 56.4%, 21% y 5% para los grados I a V de reflujo respectivamente. En el análisis multivariado, Las variables nefropatía por reflujo (sig=0,000), Síndrome de evacuación disfuncional (SED) (sig=0,000) y bilateralidad (sig=0,006) fueron los factores de riesgo independientes para la falla del RVU en resolver espontáneamente. Sin embargo, en los análisis de sobrevida solo la variable SED demostró influir en el tiempo necesario para obtener resolución espontanea (sig=0,002). Discusión: Los hallazgos de este estudio ratifican la importancia de incluir variables como SED, nefropatía por reflujo y lateralidad en los modelos de predicción de resolución espontánea del RVU.Introduction: To determine primary vesico-ureteral reflux (VUR) resolution rate in a population of children younger than five years of age, as well as, factors predicting such presentation. Furthermore, based on these results, to design a nomogram which allows prediction of spontaneous resolution likelihood within a 3 years period from diagnosis. Methodology: 407 children with primary vesicoureteral reflux diagnosed within a ten years period were included. Through association analysis and average comparison, variables acting as risk factors for spontaneous resolution failure were determined. Additionally, using binomial regression models associations were verified. We practice comparisons through ANOVA or t-test, as well as Log Rank Test survival analysis in order to determine variables affecting spontaneous resolution length. Results: Spontaneous resolution rates were 92%, 85%, 56.4%, 21% and 5% for VUR grade I to V respectively. Multivariate analysis demonstrated that reflux uropathy (sig=0,000), dysfunctional evacuation syndrome (DES) (sig=0,000) and bilateral compromise acted as the principal independent risk factors for spontaneous resolution failure. Nevertheless, survival analysis established that DES was the only risk factor affecting spontaneous resolution length (sig=0,002). Discussion: Our analyses confirm that inclusion of variables as DES, reflux uropathy and bilateral compromise is indispensable for VUR spontaneous resolution prediction

Pielonefritis Xantogranulomatosa en la infancia: una rara enfermedad en niños menores de un año, reporte de un caso

La pielonefritis xatogranulomatosa es una inflamación crónica del riñón caracterizada por destrucción y sustitución de su parénquima por tejido granulomatoso cargado de células espumosas. Fue descrita por primera vez en 1916 por Schlagenhaufer y reportada en niños solo hasta 1963 por Avnet y Friedenberg. A la fecha se han reportado en la literatura alrededor de 300 casos de los cuales al año 2007 121 correspondían a la edad pediátrica. El grupo etáreo principalmente afectado en la mayoría de los reportes obedece a mujeres en edad media, sin embargo un creciente número de casos pediátricos han sido reportados concluyéndose que esta rara enfermedad puede ocurrir en todas las edades. Aún así continua siendo extremadamente rara en lactantes menores de 1 año pese a lo cual debería ser considerada dentro de los diagnósticos diferenciales cuando se comprueba la presencia de masa renal con o sin urolitiasis asociado a factores predisponentes, síntomas constitucionales, anemia y algún grado de elevación de los marcadores de respuesta inflamatoria sistémica. Dado que el proceso inflamatorio puede extenderse mas allá del riñón y un correcto diagnostico preoperatorio permitiría planear el mejor abordaje quirúrgico, en especial si de consideran técnicas laparoscópicas en búsqueda de una reducción de complicaciones intra y post operatoriasXatogranulomatosa pyelonephritis is a chronic inflammation of the kidney characterized by destruction and replacement of parenchyma by granulomatous tissue laden foam cells. It was first described in 1916 by Schlagenhauf and reported in children only up to 1963 by Avnet and Friedenberg. To date we have reported in the literature about 300 cases per year which corresponded to 2007 121 childhood. The main age group affected most reports due to middle age women, but a growing number of cases have been reported pediatric concluded that this rare disease may occur in all ages. Yet still extremely rare in infants under 1 year although this should be considered in the differential diagnosis when tests for the presence of renal mass with or without urolithiasis associated with factors predisposing constitutional symptoms, anemia and some degree of elevated markers of systemic inflammatory response. Since the process inflammation may extend beyond the kidney and correct preoperative diagnosis would plan the best surgical approach, especially if technical considerations laparoscopic seeking a reduction of intra and postoperativ

Pielonefritis Xantogranulomatosa en la infancia: una rara enfermedad en niños menores de un año, reporte de un caso

La pielonefritis xatogranulomatosa es una inflamación crónica del riñón\ud caracterizada por destrucción y sustitución de su parénquima por tejido granulomatoso\ud cargado de células espumosas. Fue descrita por primera vez en 1916 por Schlagenhaufer y\ud reportada en niños solo hasta 1963 por Avnet y Friedenberg. A la fecha se han reportado\ud en la literatura alrededor de 300 casos de los cuales al año 2007 121 correspondían a la\ud edad pediátrica. El grupo etáreo principalmente afectado en la mayoría de los\ud reportes obedece a mujeres en edad media, sin embargo un creciente número de casos\ud pediátricos han sido reportados concluyéndose que esta rara enfermedad puede ocurrir en\ud todas las edades. Aún así continua siendo extremadamente rara en lactantes menores de 1\ud año pese a lo cual debería ser considerada dentro de los diagnósticos diferenciales cuando\ud se comprueba la presencia de masa renal con o sin urolitiasis asociado a factores\ud predisponentes, síntomas constitucionales, anemia y algún grado de elevación de los\ud marcadores de respuesta inflamatoria sistémica. Dado que el proceso\ud inflamatorio puede extenderse mas allá del riñón y un correcto diagnostico preoperatorio\ud permitiría planear el mejor abordaje quirúrgico, en especial si de consideran técnicas\ud laparoscópicas en búsqueda de una reducción de complicaciones intra y post operatoriasXatogranulomatosa pyelonephritis is a chronic inflammation of the kidney\ud characterized by destruction and replacement of parenchyma by granulomatous tissue\ud laden foam cells. It was first described in 1916 by Schlagenhauf and\ud reported in children only up to 1963 by Avnet and Friedenberg. To date we have reported\ud in the literature about 300 cases per year which corresponded to 2007 121\ud childhood. The main age group affected most\ud reports due to middle age women, but a growing number of cases\ud have been reported pediatric concluded that this rare disease may occur in\ud all ages. Yet still extremely rare in infants under 1\ud year although this should be considered in the differential diagnosis when\ud tests for the presence of renal mass with or without urolithiasis associated with factors\ud predisposing constitutional symptoms, anemia and some degree of elevated\ud markers of systemic inflammatory response. Since the process\ud inflammation may extend beyond the kidney and correct preoperative diagnosis\ud would plan the best surgical approach, especially if technical considerations\ud laparoscopic seeking a reduction of intra and postoperativ

Characterization and Etiopathogenic Approach of Pediatric Renal Biopsy Patients in a Colombian Medical Center from 2007-2017

Introduction. Renal biopsy is the principal instrument to evaluate the diagnosis and prognosis of children with kidney disease. There are relatively few studies establishing epidemiology of its findings in the pediatric population. Methods. A descriptive study was conducted to describe characteristics of pediatric patients who had undergone a renal biopsy over the last 10 years in a national reference center, trying to accomplish an etiopathogenic approach of biopsy findings. Results. 241 patients were included. Most frequent indications were nephrotic syndrome (34.1%) and systemic disease with renal involvement (30.2%). The most prevalent biopsy diagnosis was glomerulonephritis (44%) and among these patients, glomerulonephritis mediated by immune complexes was the most frequent pathogenic type (90.5%). When the biopsy was indicated for proteinuria plus hematuria and systemic disease with renal involvement, the most frequent biopsy diagnosis was glomerulonephritis (60 and 85%, respectively). For isolated hematuria, the predominant biopsy diagnosis was inherited diseases of the glomerular basement membrane (70%) and for nephrotic syndrome, podocytopathy (82%). Glomerulonephritis was more frequent in patients older than 10 yrs (65%) and the rate of postbiopsy major complications was low (1.2%). Conclusion. Immune complex glomerulonephritis was the most frequent histological finding, differing from previous reports. To our knowledge this is the first description that classifies biopsy findings according to the probable pathogenic mechanism

Regional citrate anticoagulation for continuous renal replacement therapy in children

9 páginasBackground Anticoagulation of the continuous renal replacement therapy (CRRT) circuit is an important technical aspect of this medical procedure. Most studies evaluating the efficacy and safety of citrate use have been carried out in adults, and little evidence is available for the pediatric patient population. The aim of this study was to compare regional citrate anticoagulation versus systemic heparin anticoagulation in terms of the lifetime of hemofilters in a pediatric population receiving CRRT at a pediatric center in Bogota, Colombia. Methods This was an analytical, observational, retrospective cohort study in which we assessed the survival of 150 hemofilters (citrate group 80 hemofilters, heparin group 70 hemofilters) used in a total of 3442 hours of CCRT (citrate group 2248 h, heparin group 1194 h). Hemofilter survival was estimated beginning at placement and continuing until filter replacement due to clotting or high trans-membrane pressures. Results Hemofilter survival was higher in the citrate group than in the heparin group (72 vs. 18 h; p <0.0001). Bivariate analysis showed that the hemofilter coagulation risk was significantly increased when heparin was used, regardless of hemofilter size and pump flow (hazard ratio 3.70, standard error 0.82, 95% confidence interval 2.39–5.72; p <0.00001). Conclusions Regional citrate anticoagulation could be more effective than heparin systemic anticoagulation in terms of prolonging the hemofilter lifetime in patients with acute renal injury who require CRRT

Renal involvement at diagnosis of pediatric acute lymphoblastic leukemia

Acute leukemia is the most common type of cancer in pediatric patients. This type of cancer accounts for a third of all childhood cancer cases. More than half of pediatric acute leukemia patients show signs and symptoms such as hepatomegaly, splenomegaly, pallor, fever and bruising at the time of diagnosis. In early stages of acute lymphoblastic leukemia (ALL), nephromegaly and other renal manifestations such as high blood pressure (HBP) and renal failure are uncommon, although renal infiltration and nephromegaly are common in advanced-stage pediatric patients. This is a retrospective case review with a critical appraisal of the existing evidence from the literature. We present a clinical case of a child with HBP associated with bilateral nephromegaly which resolved after chemotherapy treatment. This patient presented with HBP that required pharmacological treatment, likely owing to nephromegaly. All HBP secondary causes were rejected. Nephromegaly was resolved after chemotherapy treatment, and antihypertensive medication was discontinued. Nephromegaly and HBP are rare manifestations of ALL debut in pediatrics. The present case report illustrates this unusual combination and Suggests clinicians to consider malignancy as its causal factor, especially if the symptoms are accompanied by other suggestive extrarenal manifestations