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Objectives. Atypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease with genetic predisposition and represents up to 10% of pediatric hemolytic uremic syndrome (HUS) cases. Few studies have evaluated aHUS in Latin American population. We studied a Colombian pediatric cohort to delineate disease presentation and outcomes. Methods. A multicenter cohort of 27 Colombian children with aHUS were included. Patients were grouped by age at onset. Clinical features were compared using analysis of variance (ANOVA) and Fisher exact tests. Renal biopsy was performed on 6 patients who were suspected of having other renal diseases before aHUS diagnosis. Results. Most patients were male (70%). The onset of aHUS occurred frequently before age 4 years (60%) and followed gastroenteritis as the main triggering event (52%). Age groups showed comparable clinical presentation, disease severity, treatment, and outcomes. Pulmonary involvement (67%) was the main extrarenal manifestation, particularly in the 1 to 7 age group (P = .01). Renal biopsies were as follows: 3 had membranoproliferative glomerulonephritis (MPGN) type I, one MPGN type III, one C3-glomerulonephritis, and one rapidly progressive GN. Genetic screening was available in 6 patients and identified 2xCFHR5, 2xMCP, 1xADAMTS13/THBD, and 1xDGKE mutations. A total of 15 relapses were seen, of which 8 (72%) occurred in the 1 to 7 age group. The renal outcome was not significantly different regardless of age group. Conclusion. In our cohort, we observed a relatively high frequency of extrarenal involvement at first presentation represented by pulmonary manifestations. The renal prognosis at initial presentation was worse than in previous reports.
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INTRODUCTION: Growth retardation is a common problem in children with CKD. This study aims to describe growth, prevalence of short stature before RTx, catch-up growth after RTx, and associated factors. METHODS: We retrospectively reviewed 74 renal allograft recipients who underwent RTx at Fundación Cardioinfantil, Colombia, between January 2008 and September 2016 with follow-up for 2 years afterwards. Pre-RTx Height_SDS and demographic characteristics were compared between children with normal and short stature. Post-RTx Height_SDS at 1 and 2 years post-RTx and FAH, when available, were retrieved. Children were classified into catch-up growth and no catch-up growth groups depending on whether or not Height_SDS increased ≥0.5 per year within the first 2 years post-RTx. Possible associated factors were compared. RESULTS: Seventy-four patients were included. Mean age at RTx was 11 ± 4.0 years, and 43.2% (32/74) were females. Mean Height_SDS for the entire study population at pre-RTx was -2.8 ± 1.5. Before RTx, 68.9% (51/74) had short stature, and 44.6% (33/74) had severe short stature. 37.2% presented catch-up growth post-RTx. Time on dialysis was associated with short pre-RTx stature (OR 1.66; 95% CI [1.15-2.39]; P = .006) and catch-up growth (OR 2.15; 95% CI [1.15-3.99]; P = .016). 44.59% (33/74) reached FAH, and 48.4% (16/33) presented short FAH. CONCLUSIONS: Growth continues to be suboptimal after RTx. Given that pre-RTx height is a significantly associated factor, it is important to plan early interventions in terms of growth improvement in these children.
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Transtornos do Crescimento/etiologia , Transplante de Rim , Complicações Pós-Operatórias , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Transtornos do Crescimento/diagnóstico , Transtornos do Crescimento/epidemiologia , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Estudos RetrospectivosRESUMO
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.
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BACKGROUND: 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.
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Injúria Renal Aguda/terapia , Anticoagulantes/uso terapêutico , Ácido Cítrico/uso terapêutico , Hemofiltração/métodos , Terapia de Substituição Renal/métodos , Adolescente , Anticoagulantes/efeitos adversos , Coagulação Sanguínea/efeitos dos fármacos , Criança , Pré-Escolar , Ácido Cítrico/efeitos adversos , Estudos de Coortes , Cuidados Críticos , Feminino , Hemofiltração/instrumentação , Hemorragia/induzido quimicamente , Hemorragia/epidemiologia , Heparina/efeitos adversos , Heparina/uso terapêutico , Humanos , Lactente , Masculino , Estudos RetrospectivosRESUMO
Con el fin de enfatizar la necesidad de la vacunación masiva para todos los lactantes mayores de doce meses y la revacunación a los escolares particularmente del sexo femenino, como medidas de prevención del Síndrome de Rubeóla Congénita (SRC) se hizo una revisión epidemiológica de los casos de rubeóla congénita vistos en la Fundación Cardioinfantil desde Enero de 1985 hasta Junio de 1991. Se encontraron 46 casos y se evaluaron específicamente los aspectos relacionados con el antecedente de vacunación de la madre, la presencia de cuadro eruptivo y/o febril durante la gestación, los criterios diagnósticos de acuerdo a los establecidos por el centro de control de enfermedades de Atlanta CDC y las cardiopatías asociadas. El 76 por ciento de las madres (35) tuvieron el antecedente claro de cuadro febril y/o erupción durante la gestación. 31 de ellas tuvieron la erupción en el primer trimestre. El 65 por ciento de las madres no estaban vacunadas. El 35 por ciento restante desconocía la existencia de una vacuna para prevenir la enfermedad y por lo tanto no pudieron contestar a la pregunta. Todos los 46 casos llenaban los criterios de compatibiliadad de la CDC, ya que a pesar de haber sido vistos en diferentes instituciones de salud a ninguno se le había solicitado estudio serológico para confirmar la enfermedad. El 89 por ciento de los pacientes tuvieron cadiopatía congénita y la mas frecuente fue el Ductus Arterioso Persistente, seguido por estenosis de la válvula pulmonar y estenosis de las ramas pulmonares