Real-world evidence on the dosing and safety of C.E.R.A. in pediatric dialysis patients: findings from the International Pediatric Dialysis Network registries.

BACKGROUND: This retrospective real-world study used data from two registries, International Pediatric Peritoneal Dialysis Network (IPPN) and International Pediatric Hemodialysis Network (IPHN), to characterize the efficacy and safety of continuous erythropoietin receptor activator (C.E.R.A.) in pediatric patients with chronic kidney disease (CKD) on peritoneal dialysis (PD) or hemodialysis (HD). METHODS: IPPN and IPHN collect prospective data (baseline and every 6 months) from pediatric PD and HD centers worldwide. Demographics, clinical characteristics, dialysis information, treatment, laboratory parameters, number and causes of hospitalization events, and deaths were extracted for patients on C.E.R.A. treatment (IPPN: 2007-2021; IPHN: 2013-2021). RESULTS: We analyzed 177 patients on PD (median age 10.6 years) and 52 patients on HD (median age 14.1 years) who had ≥ 1 observation while being treated with C.E.R.A. The median (interquartile range [IQR]) observation time under C.E.R.A. exposure was 6 (0-12.5) and 12 (0-18) months, respectively. Hemoglobin concentrations were stable over time; respective means (standard deviation) at last observation were 10.9 (1.7) g/dL and 10.4 (1.7) g/dL. Respective median (IQR) monthly C.E.R.A. doses at last observation were 3.5 (2.3-5.1) µg/kg, or 95 (62-145) µg/m2 and 2.1 (1.2-3.4) µg/kg, or 63 (40-98) µg/m2. Non-elective hospitalizations occurred in 102 (58%) PD and 32 (62%) HD patients. Seven deaths occurred (19.8 deaths per 1000 observation years). CONCLUSIONS: C.E.R.A. was associated with efficient maintenance of hemoglobin concentrations in pediatric patients with CKD on dialysis, and appeared to have a favorable safety profile. The current analysis revealed no safety signals.

Kidney transplant to vesicostomy: A safe strategy for children with end stage renal disease and lower urinary tract anomalies.

INTRODUCTION: Resolution of underlying urinary tract anomalies prior to kidney transplantation in patients with end stage renal disease (ESRD) secondary to uropathy, has been historically supported under the argument that this would help prevent infectious complications and graft loss. We propose to perform earlier kidney transplantation with a transient vesicostomy, deferring resolution of the uropathy to the post-transplantation period. The aim of this study was to evaluate the outcomes of kidney transplantation in children with a vesicostomy. MATERIAL AND METHODS: A retrospective, multicenter study was performed including all patients under 18 years of age who underwent kidney transplantation with a vesicostomy, between January 2005 and December 2020 and had at least one year of follow up. Data related with the indication and timing of vesicostomy, time until transplantation, post-transplantation complications, urinary tract infections (UTI) and graft survival rate were collected. RESULTS: Of the 758 transplantations performed in the study period, 16 patients met the inclusion criteria. Mean age at transplantation was 58 months (range 20-151), and mean weight was 13.5 Kg (range 8.4-20). Mean time from vesicostomy to kidney transplantation was 30 months (range 0-70). There were 2 (12.5%) ureteral complications that required reoperation. Eighteen episodes of UTI were identified in 8 patients (50%), accounting for 0.4 UTIs per patient-year of follow-up. UTIs did not lead to graft loss in any of the cases. Urinary tract reconstruction was performed in 5 patients (31.3%) at an interval of 1-91 months post-transplantation. After a mean follow-up of 44.8 months (range 13-200) from transplantation, patients with vesicostomy had a mean creatinine clearance of 86.6 ml/min/1.73 m2, with a mean serum creatinine level of 0.6 mg/dl. Graft survival rate was 100%. DISCUSSION: Early kidney transplantation into a vesicostomy permits a resolution of the ESRD, avoiding deleterious effects related to dialysis. With a low rate of UTIs, we found no graft loss due to infectious complications. This strategy permits careful planning and better timing for the urinary tract reconstruction without delaying kidney transplantation. CONCLUSION: Kidney transplantation in pediatric patients with vesicostomy seems to be a safe and effective strategy. UTI rate was similar to that reported in the literature of patients with corrected urinary anomalies undergoing kidney transplantation without urinary diversion.

Prevalence of renal involvement among pediatric patients hospitalized due to coronavirus disease 2019: A multicenter study. / Prevalencia de compromiso renal en pacientes pediátricos internados con enfermedad por coronavirus 2019: estudio multicéntrico.

INTRODUCTION: Renal involvement among pediatric patients with coronavirus disease 2019 (COVID-19) ranges between 1.2% and 44%. Given the limited information available locally, the primary objective of this study was to estimate the prevalence of renal involvement in our setting. POPULATION AND METHODS: Cross-sectional study conducted in 13 Argentine sites between March and December 2020. Patients aged 1 month to 18 years hospitalized due to COVID-19 and with at least one measurement of serum creatinine and/or a urinalysis were included. Those with a known kidney disease were excluded. Renal involvement was defined as the presence of acute kidney injury (AKI), proteinuria, hematuria, leukocyturia and/or arterial hypertension (HTN). RESULTS: Among 528 eligible medical records, 423 patients were included (55.0% were males; median age: 5.3 years). The clinical presentation was asymptomatic in 31%; mild, in 39.7%; moderate, in 23.9%; severe, in 1.2%; critical, in 0.7%; and 3.5% had multisystem inflammatory syndrome in children (MIS-C). Two patients (0.47%) died. The prevalence of renal involvement was 10.8% (95% confidence interval: 8.2-14.2); it was described as leukocyturia (16.9%), proteinuria (16.0%), hematuria (13.2%), HTN (3.7%), and AKI (2.3%). No patient required dialysis. Renal involvement was associated with severe forms of disease (p < 0.0001). CONCLUSIONS: The prevalence of renal involvement among pediatric patients hospitalized due to COVID-19 in 13 Argentine sites was 10.8%; severe forms of disease prevailed.

Introducción. El compromiso renal (CR) en niños internados con enfermedad por coronavirus 2019 (COVID-19, por su sigla en inglés) varía entre el 1,2 % y el 44 %. Dado que existe limitada información local, el objetivo primario de este estudio fue estimar la prevalencia de CR en nuestro medio. Población y métodos. Estudio transversal realizado en 13 centros de Argentina entre marzo y diciembre de 2020. Se incluyeron pacientes internados con COVID-19, de 1 mes a 18 años y que tuvieran al menos una determinación de creatinina sérica y/o de orina completa. Se excluyeron aquellos con enfermedad renal conocida. Se consideró CR la presencia de lesión renal aguda (LRA), proteinuria, hematuria, leucocituria y/o hipertensión arterial (HTA). Resultados. De 528 historias clínicas elegibles, se incluyeron las de 423 pacientes (el 55,0 % de sexo masculino, mediana de edad 5,3 años). El cuadro clínico fue asintomático en el 31 %, leve en el 39,7 %, moderado en el 23,9 %, grave en el 1,2 %, crítico en el 0,7 %, y el 3,5 % presentó síndrome inflamatorio multisistémico pediátrico (SIMP). Dos pacientes (0,47 %) fallecieron. La prevalencia de CR fue del 10,8 % (intervalo de confianza 95% 8,2-14,2), expresada por leucocituria (16,9 %), proteinuria (16,0 %), hematuria (13,2 %), HTA (3,7 %) y LRA (2,3 %). Ninguno requirió diálisis. Presentar CR se asoció (p <0,0001) con formas graves de enfermedad. Conclusión. La prevalencia de CR en pacientes pediátricos internados con COVID-19 en 13 centros de nuestro país fue del 10,8 % y predominó en las formas clínicas graves.

[Clinical practice guideline for the diagnosis and management of urinary tract infections: 2022 update]. / Guía para el diagnóstico, estudio y tratamiento de la infección urinaria: actualización 2022.

In 2015, the "New recommendations regarding the current controversies in urinary infection" were published in the Archivos Argentinos de Pediatría. Given the fact that in these past years, new evidence has emerged regarding the diagnosis and treatment of urinary infection, the Pediatric Nephrology Committee of Sociedad Argentina de Pediatría has decided to update these recommendations. The main goal is to provide the pediatrician with the necessary tools to make a correct diagnosis, define the most appropriate treatment, select the patients who will benefit from antibiotic prophylaxis, and decide which imaging studies will be necessary, avoiding costly and invasive interventions. These guidelines also include the management of children with urinary tract infections associated with special situations such as: bladder bowel dysfunction, the newborn, children with neurogenic bladder, kidney transplant patients and fungal urinary tract infections.

En 2015 se publicaron en Archivos Argentinos de Pediatría las "Nuevas recomendaciones frente a las actuales controversias en infección urinaria". Dado que en estos años surgieron evidencias con respecto al diagnóstico, la forma de estudio y el tratamiento de la infección urinaria, el Comité de Nefrología Pediátrica de la Sociedad Argentina de Pediatría decidió actualizar dichas recomendaciones. El objetivo principal es brindar al pediatra las herramientas para realizar un correcto diagnóstico, definir el tratamiento más adecuado, seleccionar a los pacientes que se beneficiarán con la profilaxis antibiótica y decidir cuáles serán los estudios de imágenes necesarios, para evitar intervenciones costosas e invasivas. En estas guías se incluyen, además, los lineamientos para el manejo de niños con infecciones urinarias asociadas a situaciones especiales como la disfunción vesicointestinal, el recién nacido, los portadores de vejiga neurogénica, los receptores de trasplante renal y las infecciones urinarias micóticas.

Guía para el diagnóstico, estudio y tratamiento de la infección urinaria: actualización 2022 / Clinical practice guideline for the diagnosis and management of urinary tract infections: 2022 update

En 2015 se publicaron en Archivos Argentinos de Pediatría las "Nuevas recomendaciones frente a las actuales controversias en infección urinaria". Dado que en estos años surgieron evidencias con respecto al diagnóstico, la forma de estudio y el tratamiento de la infección urinaria, el Comité de Nefrología Pediátrica de la Sociedad Argentina de Pediatría decidió actualizar dichas recomendaciones. El objetivo principal es brindar al pediatra las herramientas para realizar un correcto diagnóstico, definir el tratamiento más adecuado, seleccionar a los pacientes que se beneficiarán con la profilaxis antibiótica y decidir cuáles serán los estudios de imágenes necesarios, para evitar intervenciones costosas e invasivas. En estas guías se incluyen, además, los lineamientos para el manejo de niños con infecciones urinarias asociadas a situaciones especiales como la disfunción vesicointestinal, el recién nacido, los portadores de vejiga neurogénica, los receptores de trasplante renal y las infecciones urinarias micóticas.

In 2015, the "New recommendations regarding the current controversies in urinary infection" were published in the Archivos Argentinos de Pediatría. Given the fact that in these past years, new evidence has emerged regarding the diagnosis and treatment of urinary infection, the Pediatric Nephrology Committee of Sociedad Argentina de Pediatría has decided to update these recommendations. The main goal is to provide the pediatrician with the necessary tools to make a correct diagnosis, define the most appropriate treatment, select the patients who will benefit from antibiotic prophylaxis, and decide which imaging studies will be necessary, avoiding costly and invasive interventions. These guidelines also include the management of children with urinary tract infections associated with special situations such as: bladder bowel dysfunction, the newborn, children with neurogenic bladder, kidney transplant patients and fungal urinary tract infections.

Prevalencia de compromiso renal en pacientes pediátricos internados con enfermedad por coronavirus 2019: estudio multicéntrico / Prevalence of renal involvement among pediatric patients hospitalized due to coronavirus disease 2019: A multicenter study

Introducción. El compromiso renal (CR) en niñosinternados con enfermedad por coronavirus2019 (COVID-19, por su sigla en inglés) varía entre el 1,2 % y el 44 %. Dado que existe limitada información local, el objetivo primario de este estudio fue estimar la prevalencia de CR en nuestro medio. Población y métodos. Estudio transversalrealizado en 13 centros de Argentina entre marzo y diciembre de 2020. Se incluyeron pacientes internados con COVID-19, de 1 mes a 18 años y que tuvieran al menos una determinación de creatinina sérica y/o de orina completa.Se excluyeron aquellos con enfermedad renal conocida. Se consideró CR la presencia de lesión renal aguda (LRA), proteinuria, hematuria, leucocituria y/o hipertensión arterial (HTA). Resultados. De 528 historias clínicas elegibles, seincluyeron las de 423 pacientes (el 55,0 % de sexo masculino, mediana de edad 5,3 años). El cuadro clínico fue asintomático en el 31 %, leve en el 39,7 %, moderado en el 23,9 %, grave en el 1,2 %, crítico en el 0,7 %, y el 3,5 % presentó síndrome inflamatorio multisistémico pediátrico (SIMP). Dos pacientes (0,47 %) fallecieron. La prevalencia de CR fue del 10,8 % (intervalo de confianza 95% 8,2-14,2), expresada por leucocituria (16,9 %), proteinuria (16,0 %), hematuria (13,2 %), HTA (3,7 %) y LRA (2,3 %). Ninguno requirió diálisis. Presentar CR se asoció (p <0,0001) con formas graves de enfermedad. Conclusión. La prevalencia de CR en pacientes pediátricos internados con COVID-19 en 13 centros de nuestro país fue del 10,8 % y predominó en las formas clínicas graves.

Introduction. Renal involvement among pediatric patients with coronavirus disease 2019 (COVID-19) ranges between 1.2% and 44%. Given the limited information available locally, the primary objective of this study was to estimate the prevalence of renal involvement in our setting. Population and methods. Cross-sectional study conducted in 13 Argentine sites between March and December 2020. Patients aged 1 month to 18 years hospitalized due to COVID-19 and with at least one measurement of serum creatinine and/or a urinalysis were included. Those with a known kidney disease were excluded. Renal involvement was defined as the presence of acute kidney injury (AKI), proteinuria, hematuria, leukocyturia and/or arterial hypertension (HTN). Results. Among 528 eligible medical records, 423 patients were included (55.0% were males; median age: 5.3 years). The clinical presentation was asymptomatic in 31%; mild, in 39.7%; moderate, in 23.9%; severe, in 1.2%; critical, in 0.7%; and 3.5% had multisystem inflammatory syndrome in children (MIS-C). Two patients (0.47%) died. The prevalence of renal involvement was 10.8% (95% confidence interval: 8.2­14.2); it was described as leukocyturia (16.9%), proteinuria (16.0%), hematuria (13.2%), HTN (3.7%), and AKI (2.3%). No patient required dialysis. Renal involvement was associated with severe forms of disease (p < 0.0001). Conclusion. The prevalence of renal involvement among pediatric patients hospitalized due to COVID-19 in 13 Argentine sites was 10.8%; severe forms of disease prevailed.

Importance of clinical practice guidelines to practicing pediatric nephrologists and IPNA survey.

Clinical practice guidelines (CPGs) are systematically developed statements backed by scientific evidence to assist practitioners in management in clinical practice. An international cross-sectional survey was conducted by the IPNA to examine the perceptions of pediatric nephrologists on guidelines and their usage and to identify important diseases for future clinical practice guidelines (CPGs). The survey found that the majority of pediatric nephrologists find CPGs useful in clinical practice and admitted to using them most of the time. Developing CPGs is challenging and there are standards available to develop trustworthy guidelines. While evidence-based global guidelines are ideal, pediatric nephrologists expressed the desire that they address regional differences. Most respondents (89.2%) to the survey agreed that adult guidelines did not cover the pediatric perspective adequately and 71.4% opined that consensus-based pediatric guidelines can be developed when evidence for the pediatric population is lacking. The development of high-quality practice guidelines requires substantial resources and may not be feasible in resource-poor countries. Adaptation of an existing guideline has been suggested as an alternative and the ADAPTE collaboration provides a systematic approach to adapting guidelines. Several diseases where pediatric guidelines are needed as a priority including IgA and C3 glomerulopathy were identified in the survey. Implementation of guideline-based care is challenging and the survey found that lack of availability of guidelines (43%) and resources (22.8%) are important reasons for poor implementation in lower-middle and low-income countries. Perceived complexity of guidelines, physician attitudes, and lack of training also contribute to non-adherence to guidelines.

Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli infection in Argentina: update of serotypes and genotypes and their relationship with severity of the disease.

BACKGROUND: Shiga toxin-producing Escherichia coli (STEC) infection is the most common cause of hemolytic uremic syndrome (HUS). Only few studies correlated serotypes and stx genotypes with disease severity. This study aimed to update STEC serotypes, stx genotypes, and virulence factors (eae and ehxA) in a cohort of patients with STEC-HUS and investigate whether they influence the severity of disease. METHODS: In this multicentric study, children hospitalized between 2005 and 2016 with STEC-HUS confirmed by the National Reference Laboratory were included. Serotypes (O157, O145, O121, and others), stx genotypes (stx1a, stx2a, stx2c, stx2d, and others), and virulence factors were analyzed, and their association with dialysis requirement (>10 days); severe neurological, cardiovascular, and/or bowel involvement; and death was assessed. RESULTS: The records of 280 patients were reviewed; 160 females, median age 21 months (IQR18m). STEC O157 was isolated in 206 (73.6%) patients, O145 in 47 (16.8%), O121 in 15 (5.4%), and other serotypes in 12 (4.2%). The stx2a/2c genotype was carried by 179 (63.9%) strains, stx2a by 94 (33.6%), stx1a/stx2a by five (1.8%), and stx1a only by two (0.7%). All strains except six harbored eae and ehxA genes. Fifty-nine (21.1%) patients had severe neurological involvement, 29 (10.4%) severe bowel injury, 14 (5%) cardiovascular involvement, 53 (18.9%) required > 10 days of dialysis, and 12 (4.3%) died. Neither serotypes nor stx genotypes detected were significantly linked to severity. CONCLUSIONS: Serotype O157 and virulence stx2a/2c, eae, ehxA genotype are prevalent in Argentina, and no relationship was found between severity and serotypes and genotypes of STEC detected.

Acute peritoneal dialysis, complications and outcomes in 389 children with STEC-HUS: a multicenter experience.

BACKGROUND: Management of acute kidney injury (AKI) in children with hemolytic uremic syndrome induced by a Shiga toxin-producing Escherichia coli infection (STEC-HUS) is supportive; however, 40 to 60% of cases need kidney replacement therapy (KRT). The aim of this study was to analyze procedure complications, especially peritonitis, and clinical outcomes in children with AKI secondary to STEC-HUS treated with acute PD. METHODS: This is a multicenter retrospective study conducted among thirty-seven Argentinian centers. We reviewed medical records of 389 children with STEC-HUS hospitalized between January 2015 and February 2019 that required PD. RESULTS: Complications associated with PD were catheter malfunction (n = 93, 24%), peritonitis (n = 75, 19%), fluid leaks (n = 45, 11.5%), bleeding events (n = 23, 6%), and hyperglycemia (n = 8, 2%). In the multivariate analysis, the use of antibiotic prophylaxis was independently associated with a decreased risk of peritonitis (hazard ratio 0.49, IC 95% 0.29-0.81; p = 0.001), and open-surgery catheter insertion was independently associated with a higher risk (hazard ratio 2.8, IC 95% 1.21-6.82; p = 0.001). Discontinuation of PD due to peritonitis, severe leak, or mechanical complications occurred in 3.8% of patients. No patient needed to be transitioned to other modality of KRT due to inefficacy of the technique. Mortality during the acute phase occurred in 2.8% patients due to extrarenal complications (neurological and cardiac involvement), not related to PD. CONCLUSIONS: Acute PD was a safe and effective method to manage AKI in children with STEC-HUS. Prophylactic antibiotics prior to insertion of the PD catheter should be considered to decrease the incidence of peritonitis.

Calidad de vida de los hermanos de pacientes con enfermedad crónica / Quality of life among siblings of patients with chronic conditions

Introducción. La calidad de vida relacionada con la salud (CVRS) es una medida de resultado de salud. Evalúa el impacto subjetivo y global de las enfermedades en la vida cotidiana. Brinda información multidimensional sobre el bienestar físico, relación familiar y sus pares. Los estudios de CVRS de hermanos son limitados.Objetivo. Comparar CVRS de los hermanos de pacientes pediátricos con patologías reumáticas crónicas, trasplante renal o hepático con la de niños sanos con hermanos sin enfermedades crónicas.Resultados. Se compararon hermanos de niños con trasplante renal (n: 65), trasplante hepático (n: 35) y patologías reumáticas crónicas (n: 36) con el grupo control de niños sanos (n: 51). El grupo total de hermanos tuvieron puntuación más baja, estadísticamente significativa, en las dimensiones bienestar físico, amigos-apoyo social y recursos económicos. Los hermanos de trasplante renal tuvieron baja puntuación en las dimensiones de bienestar físico (p < 0,02; tamaño del efecto ­TE­: 0,66) y recursos económicos (p < 0,01; TE: 0,66). Los hermanos de trasplante hepático percibieron menor bienestar físico (p = 0,04), tenían menos amigos y apoyo social (p < 0,01), dificultades en el entorno escolar (p < 0,02) y recursos económicos (p < 0,01). Los hermanos de patologías reumáticas crónicas tuvieron menor bienestar físico (p < 0,05; TE: 0,44) y apoyo social-amigos (p < 0,01; TE: 0,58).Conclusión. La CVRS de niños/as sanos de hermanos con patologías crónicas es menor en bienestar físico, amigos-apoyo social y recursos económicos comparada con el grupo de niños sanos.

Introduction. Health-related quality of life (HRQoL) is a measure of health outcomes. It assesses the subjective and overall impact of diseases on daily life. It also provides multidimensional data about physical well-being, family and peers relations. HRQoL studies on siblings are limited.Objective. To compare HRQoL among siblings of pediatric patients with chronic rheumatic diseases, kidney or liver transplant and healthy children whose siblings had no chronic conditions.Results. The siblings of children with kidney transplant (n: 65), liver transplant (n: 35), and chronic rheumatic diseases (n: 36) were compared to the healthy children group (n: 51). The total siblings group had a lower, statistically significant score in the physical well-being, social support and peers, and financial resources dimensions. The siblings of kidney transplant patients had a low score in the physical well-being (p < 0.02; effect size [ES]: 0.66) and financial resources (p < 0.01; ES: 0.66) dimensions. The siblings of liver transplant patients perceived a lower physical well-being (p = 0.04), less social support and peers(p < 0.01), and difficulties in relation to school environment (p < 0.02) and financial resources (p < 0.01). The siblings of those with chronic rheumatic diseases had a lower score in the physical well-being (p < 0.05; ES: 0.44) and social support and peers (p < 0.01; ES: 0.58) dimensions.Conclusion. HRQoL among healthy children whose siblings have a chronic disease was lower in the physical well-being, social support and peers, and financial resources dimensions compared to the healthy children group.

Quality of life among siblings of patients with chronic conditions. / Calidad de vida de los hermanos de pacientes con enfermedad crónica.

INTRODUCTION: Health-related quality of life (HRQoL) is a measure of health outcomes. It assesses the subjective and overall impact of diseases on daily life. It also provides multidimensional data about physical wellbeing, family and peers relations. HRQoL studies on siblings are limited. OBJECTIVE: To compare HRQoL among siblings of pediatric patients with chronic rheumatic diseases, kidney or liver transplant and healthy children whose siblings had no chronic conditions. RESULTS: The siblings of children with kidney transplant (n: 65), liver transplant (n: 35), and chronic rheumatic diseases (n: 36) were compared to the healthy children group (n: 51). The total siblings group had a lower, statistically significant score in the physical well-being, social support and peers, and financial resources dimensions. The siblings of kidney transplant patients had a low score in the physical wellbeing (p < 0.02; effect size [ES]: 0.66) andfinancial resources (p < 0.01; ES: 0.66) dimensions. The siblings of liver transplant patients perceived a lower physical well-being (p = 0.04), less social support and peers (p < 0.01), and difficulties in relation to school environment (p < 0.02) and financial resources (p <0.01). The siblings of those with chronic rheumatic diseases had a lower score in the physical well-being (p < 0.05; ES: 0.44) and social support and peers (p <0.01; ES: 0.58) dimensions. CONCLUSION: HRQoL among healthy children whose siblings have a chronic disease was lower in the physical well-being, social support and peers, and financial resources dimensions compared to the healthy children group.

Introducción. La calidad de vida relacionada con la salud (CVRS) es una medida de resultado de salud. Evalúa el impacto subjetivo y global de las enfermedades en la vida cotidiana. Brinda información multidimensional sobre el bienestar físico, relación familiar y sus pares. Los estudios de CVRS de hermanos son limitados. Objetivo. Comparar CVRS de los hermanos de pacientes pediátricos con patologías reumáticas crónicas, trasplante renal o hepático con la de niños sanos con hermanos sin enfermedades crónicas. Resultados. Se compararon hermanos de niños con trasplante renal (n: 65), trasplante hepático (n: 35) y patologías reumáticas crónicas (n: 36) con el grupo control de niños sanos (n: 51). El grupo total de hermanos tuvieron puntuación más baj a, estadísticamente significativa, enlas dimensiones bienestar físico, amigos-apoyo social y recursos económicos. Los hermanos de trasplante renal tuvieron baja puntuación en las dimensiones de bienestar físico (p < 0,02; tamaño del efecto -TE-: 0,66) y recursos económicos (p < 0,01; TE: 0,66). Los hermanos de trasplante hepático percibieron menor bienestar físico (p = 0,04), tenían menos amigos y apoyo social (p < 0,01), dificultades en el entorno escolar (p < 0,02) y recursos económicos (p < 0,01). Los hermanos de patologías reumáticas crónicas tuvieron menor bienestar físico (p < 0,05; TE: 0,44) y apoyo social-amigos (p < 0,01; TE: 0,58). Conclusión. La CVRS de niños/as sanos de hermanos con patologías crónicas es menor en bienestar físico, amigos-apoyo social y recursos económicos comparada con el grupo de niños sanos.

Health-related quality of life among siblings of kidney transplant recipients.

Studies are increasingly recognizing health-related quality of life (HRQOL) as a key pediatric outcome in both clinical and research settings and an essential health outcome measure to assess the effectiveness of medical treatment. However, it has not yet been studied among the healthy siblings of kidney transplant recipients. The aim of this study, therefore, is to examine HRQOL among this population. We asked the following three groups to complete a validated measure of HRQOL among children (KIDSCREEN-52): siblings of children who had received kidney transplants (n = 50), kidney transplant recipients (n = 43), and a healthy control group (n = 84). We found that siblings of kidney transplant patients exhibited lower scores for financial resources and autonomy than kidney transplant recipients. They also served lower on physical well-being, financial resources, autonomy, and parent relations/home life than the control group. However, they scored higher on social acceptance than kidney transplant recipients. Our study underscores the importance of assessing HRQOL in families including a child diagnosed with a chronic illness. Siblings require social and psychological support to promote coping and adaptation.

"Removal of nitrate and nitrite by hemodialysis in end-stage renal disease and by sustained low-efficiency dialysis in acute kidney injury".

BACKGROUND & PURPOSE: It is well established that end-stage renal disease (ESRD) is associated with increased cardiovascular morbidity and mortality both in the adult and pediatric population. Although the underlying molecular mechanisms are poorly understood, compromised nitric oxide (NO) bioactivity has been suggested as a contributing factor. With this in mind, we investigated the effects of hemodialysis on NO homeostasis and bioactivity in blood. METHODS & RESULTS: Plasma and dialysate samples were obtained before and after hemodialysis sessions from adults (n = 33) and pediatric patients (n = 10) with ESRD on chronic renal replacement therapy, and from critically ill adults with acute kidney injury (n = 12) at their first sustained low-efficiency dialysis session. Levels of nitrate, nitrite, cyclic guanosine monophosphate (cGMP) and amino acids relevant for NO homeostasis were analyzed. We consistently found that nitrate and cGMP levels in plasma were significantly reduced after hemodialysis, whereas post-dialysis nitrite and amino acids coupled to NO synthase activity (i.e., arginine and citrulline) were only significantly reduced in adults with ESRD. The amount of excreted nitrate and nitrite during dialysis were similar to daily endogenous levels that would be expected from endothelial NO synthase activity. CONCLUSIONS: Our results show that hemodialysis significantly reduces circulating levels of nitrate and cGMP, indicating that this medical procedure may impair NO synthesis and potentially NO signaling pathways.

Uso De Eculizumab En Síndrome Urémico Hemolítico Típico: Una Opción Terapéutica En El Compromiso Neurológico Severo. Reporte De Dos Casos / Use Of Eculizumab In Typical Hemolytic Uremic Syndrome: A Therapeutic Option In Severe Neurological Compromise. Two Cases Report

Resumen El síndrome urémico hemolítico típico es una enfermedad endémica en América Latina. Argentina es uno de los países con más casos reportados, con una tasa de diez casos cada 100.000 menores de cinco años. Es la primera causa de insuficiencia renal aguda, y responsable del 9 % de los trasplantes renales. Esta patología se caracteriza por una tríada clásica: anemia microangiopática, trombocitopenia e insuficiencia renal aguda. El principal agente etiológico del Síndrome Urémico Hemolítico es la bacteria Escherichia coli, productora de la toxina Shiga. El Síndrome Urémico Hemolítico tiene una mortalidad aguda inferior al 5 %.1-2 Existe evidencia acerca del rol activo de la shiga toxina en la activación del complemento a través de su unión al factor H. El eculizumab es un anticuerpo monoclonal que inhibe la formación del complejo de ataque de membrana (C5b-9), por su alta afinidad a C5 de la cascada del complemento. Su infusión está aprobada para el tratamiento del Síndrome Urémico Hemolítico atípico, planteándose su utilidad en casos de Síndrome Urémico Hemolítico típico grave con compromiso neurológico severo como alternativa para inhibir la cascada de complemento, y así detener el daño producido por la toxina. Se presentan dos casos de pacientes pediátricos con diagnóstico Síndrome Urémico Hemolítico con rescate de Shiga toxina, con compromiso neurológico grave y que recibieron tratamiento con eculizumab con respuesta favorable.

Abstract Hemolytic Uremic Syndrome is an endemic disease in Latin America. Argentina is one of the countries where most cases are reported, with a rate of ten cases per 100,000 children under five years old. It is the first cause of acute renal failure (ARF), and responsible for 9% of kidney transplants. This pathology is characterized by a classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. The main etiological agent of HUS is the bacterium Shiga toxin-producing Escherichia coli. HUS has an acute mortality lower than 5 %. There is evidence of the active role of the Shiga toxin in the activation of the complement by binding to factor H. Eculizumab is a monoclonal antibody which inhibits the formation of the membrane attack complex (C5b-9), given its great affinity for C5 of the complement cascade. Its infusion is approved to treat atypical HUS, posing its usefulness to treat severe typical HUS with acute neurological involvement as an alternative to inhibit the complement cascade and stop toxin damage. We present two pediatric patients with SUH diagnosis with shiga toxin rescue; these patients, who showed severe neurological involvement, were treated with Eculizumab and had a favorable response.

Colostomy in children on chronic peritoneal dialysis.

BACKGROUND: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy. METHODS: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites. RESULTS: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0-2.2) and 2.8 (IQR 0.2-18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9-35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2-7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis. CONCLUSION: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential.

Hyponatremia: a new predictor of mortality in patients with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome.

OBJECTIVES: (1) Evaluate mortality rate in patients with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome, (2) determine the leading causes of death, and (3) identify predictors of mortality at hospital admission. METHODS: We conducted a multicentric, observational, retrospective, cross-sectional study. It included patients under 18 years old with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome hospitalized between January 2005 and June 2016. Clinical and laboratory data were obtained from the Argentine National Epidemiological Surveillance System of Hemolytic Uremic Syndrome. Clinical and laboratory variables were compared between deceased and non-deceased patients. Univariate and multivariate analyses were performed. ROC curves and area under the curve were obtained. RESULTS: Seventeen (3.65%) out of the 466 patients died, being central nervous system involvement the main cause of death. Predictors of death were central nervous system involvement, the number of days since the beginning of diarrhea to hospitalization, hyponatremia, high hemoglobin, high leukocyte counts, and low bicarbonate concentration on admission. In the multivariate analysis, central nervous system involvement, sodium concentration, and hemoglobin were independent predictors. The best cut off for sodium was ≤ 128 meq/l and for hemoglobin ≥ 10.8 g/dl. CONCLUSIONS: Mortality was low in children with Shiga toxin-producing Escherichia coli hemolytic uremic syndrome, being central nervous system involvement the main cause of death. The best mortality predictors found were central nervous system involvement, hemoglobin, and sodium concentration. Hyponatremia may be a new Shiga toxin-producing Escherichia coli hemolytic uremic syndrome mortality predictor.

Alta prevalencia de deficiencia de vitamina D en niños con enfermedad renal crónica y trasplante renal / High prevalence of vitamin D deficiency among children with chronic kidney disease and kidney transplant

Introducción. la deficiencia de vitamina D (25OHD) es muy común en pacientes con enfermedad renal crónica (ERC). Los obj etivos de este trabajo fueron determinar la prevalencia del déficit de 25OHD en niños con ERC e identificar factores de riesgo. Se estableció la correlación entre 25OHD y paratohormona molécula intacta. Población y métodos. Estudio transversal realizado entre enero de 2013 y diciembre de 2015. Se incluyeron pacientes menores de 19 años con y sin ERC. Resultados. Se incluyeron 167 pacientes. Grupo 1 (controles sanos): 32 participantes; grupo 2 (ERC, estadios 2-4, filtrado glomerular entre 89 y 15 ml/min/1,73 m²): 34 pacientes; grupo 3 (estadio 5, en diálisis): 46 pacientes; y grupo 4 (trasplantados renales): 55 pacientes. Presentaron deficiencia de 25OHD 12,5% de los controles sanos y 32% de los pacientes con ERC (p= 0,025). El 23% de los pacientes del grupo 2, el 51% del grupo 3 y el 22% del grupo 4 presentaron deficiencia de 25OHD; el valor medio de 25OHD de los pacientes en diálisis fue significativamente menor que en el resto de los grupos. Los predictores de deficiencia de 25OHD fueron la hipoalbuminemia, ERC avanzada y la procedencia de la región Noroeste. La paratohormona molécula intacta fue significativamente más elevada en el grupo de pacientes con deficiencia y mostró una correlación inversa con los valores de 25OHD. Conclusión. El 32% de los pacientes con ERC presentó deficiencia de 25OHD; en el estadio 5 (diálisis), alcanzó el 51%. Los predictores de deficiencia fueron hipoalbuminemia, ERC avanzada y la procedencia del noroeste.

Introduction. Vitamin D (25(OH)D) deficiency is common among patients with chronic kidney disease (CKD). Our objective was to establish the prevalence of 25(OH)D deficiency among children with CKD and identify risk factors. A correlation was observed between 25(OH)D and parathormone intact molecule. Population and methods. Cross-sectional study conducted between January 2013 and December 2015. Patients younger than 19 years old with and without CKD were included. Results. One hundred and sixty-seven patients were included. Group 1 (healthy controls): 32 participants; group 2 (stage 2-4 CKD, glomerular filtration rate between 89 and 15 mL/min/1.73 m²): 34 patients; group 3 (stage 5 CKD, dialysis): 46 patients; and group 4 (kidney transplant recipients): 55 patients. Deficiency of 25(OH)D was detected in 12.5% of healthy controls and 32% of CKD patients (p= 0.025). Also, 23% of patients in group 2, 51% in group 3, and 22% in group 4 had 25(OH)D deficiency; the mean 25(OH)D level of dialysis patients was significantly lower than that of the rest of the groups. Predictors of 25(OH)D deficiency included hypoalbuminemia, advanced CKD, and place of origin from the Northwest region of Argentina. The parathormone intact molecule was significantly higher in the group of patients with deficiency and was inversely correlated with 25(OH)D levels. Conclusion. Among CKD patients, 32% had 25(OH)D deficiency, which reached 51% among those with stage 5 CKD (dialysis). Predictors of deficiency included hypoalbuminemia, advanced CKD, and place of origin from the Northwest region of Argentina.

High prevalence of vitamin D deficiency among children with chronic kidney disease and kidney transplant. / Alta prevalencia de deficiencia de vitamina D en niños con enfermedad renal crónica y trasplante renal.

INTRODUCTION: Vitamin D (25(OH)D) deficiency is common among patients with chronic kidney disease (CKD). Our objective was to establish the prevalence of 25(OH)D deficiency among children with CKD and identify risk factors. A correlation was observed between 25(OH)D and parathormone intact molecule. POPULATION AND METHODS: Cross-sectional study conducted between January 2013 and December 2015. Patients younger than 19 years old with and without CKD were included. RESULTS: One hundred and sixty-seven patients were included. Group 1 (healthy controls): 32 participants; group 2 (stage 2-4 CKD, glomerular filtration rate between 89 and 15 mL/min/1.73 m2): 34 patients; group 3 (stage 5 CKD, dialysis): 46 patients; and group 4 (kidney transplant recipients): 55 patients. Deficiency of 25(OH)D was detected in 12.5% of healthy controls and 32% of CKD patients (p= 0.025). Also, 23% of patients in group 2, 51% in group 3, and 22% in group 4 had 25(OH)D deficiency; the mean 25(OH)D level of dialysis patients was significantly lower than that of the rest of the groups. Predictors of 25(OH)D deficiency included hypoalbuminemia, advanced CKD, and place of origin from the Northwest region of Argentina. The parathormone intact molecule was significantly higher in the group of patients with deficiency and was inversely correlated with 25(OH)D levels. CONCLUSION: Among CKD patients, 32% had 25(OH)D deficiency, which reached 51% among those with stage 5 CKD (dialysis). Predictors of deficiency included hypoalbuminemia, advanced CKD, and place of origin from the Northwest region of Argentina.

INTRODUCCIÓN: La deficiencia de vitamina D (25OHD) es muy común en pacientes con enfermedad renal crónica (ERC). Los obj etivos de este trabajo fueron determinar la prevalencia del déficit de 25OHD en niños con ERC e identificar factores de riesgo. Se estableció la correlación entre 25OHD y paratohormona molécula intacta. POBLACIÓN Y MÉTODOS: Estudio transversal realizado entre enero de 2013 y diciembre de 2015. Se incluyeron pacientes menores de 19 años con y sin ERC. RESULTADOS: Se incluyeron 167 pacientes. Grupo 1 (controles sanos): 32 participantes; grupo 2 (ERC, estadios 2-4, filtrado glomerular entre 89 y 15 ml/ min/1,73 m2): 34 pacientes; grupo 3 (estadio 5, en diálisis): 46 pacientes; y grupo 4 (trasplantados renales): 55 pacientes. Presentaron deficiencia de 25OHD 12,5% de los controles sanos y 32% de los pacientes con ERC (p= 0,025). El 23% de los pacientes del grupo 2, el 51% del grupo 3 y el 22% del grupo 4 presentaron deficiencia de 25OHD; el valor medio de 25OHD de los pacientes en diálisis fue significativamente menor que en el resto de los grupos. Los predictores de deficiencia de 25OHD fueron la hipoalbuminemia, ERC avanzada y la procedencia de la región Noroeste. La paratohormona molécula intacta fue significativamente más elevada en el grupo de pacientes con deficiencia y mostró una correlación inversa con los valores de 25OHD.El 32% de los pacientes con ERC presentó deficiencia de 25OHD; en el estadio 5 (diálisis), alcanzó el 51%. Los predictores de deficiencia fueron hipoalbuminemia, ERC avanzada y la procedencia del noroeste.

Prevalencia y evolución clínica del síndrome urémico hemolítico típico entre hermanos / Prevalence and clinical course of typical hemolytic uremic syndrome among siblings

Introducción. El síndrome urémico hemolítico (SUH) es una entidad infectocontagiosa producida por Escherichia coli productora de toxina Shiga. El objetivo fue evaluar el riesgo de contagio y evolución clinica entre hermanos con SUH típico. Población y métodos. Se analizaron las historias clínicas de los niños con SUH típico desde 1997 a 2012. Se utilizó, como criterio de inclusión, a parejas de hermanos. Se definió un puntaje de gravedad. Resultados. Se registraron 133 pacientes con SUH; 40 tenían hermanos y 4 progresaron a SUH (10%). La edad media de los 4 pares de hermanos fue de 29,3 meses (DE ± 11,5) y 5 (62,5%) eran niñas. El tiempo medio entre casos fue de 5,7 días (DE ± 3). El SUH presentó un mayor puntaje de gravedad en los hermanos que se enfermaron en segundo lugar. Conclusión. El riesgo para desarrollar SUH entre hermanos fue 10% y la evolución clínica del segundo hermano fue más desfavorable.

Introduction. Hemolytic uremic syndrome (HUS) isaninfectious disease caused by Shiga toxin-producing Escherichia coli. The objective of this study was to assess the risk of transmission and clinical course between siblings with typical HUS. Population and methods. Medical records of children with typical HUS between 1997 and 2012 were reviewed. Sibling pairs were established as inclusion criteria. A severity score was defined. Results. A total of 133 patients with HUS were recorded; 40 had siblings and 4 progressed to HUS (10%). The mean age of the 4 sibling pairs was 29.3 months old (SD ± 11.5); 5 (62.5%) were girls. The mean time between each case was 5.7 days (SD ± 3). HUS was more severe in the siblings who became infected in the second place. Conclusion. The risk of HUS transmission between siblings was 10%, and the clinical course of the second sibling was less favorable.

Prevalence and clinical course of typical hemolytic uremic syndrome among sibling. / Prevalencia y evolución clínica del síndrome urémico hemolítico típico entre hermanos.

INTRODUCTION: Hemolytic uremic syndrome (HUS) isaninfectious disease caused by Shiga toxin-producing Escherichia coli. The objective of this study was to assess the risk of transmission and clinical course between siblings with typical HUS. POPULATION AND METHODS: Medical records of children with typical HUS between 1997 and 2012 were reviewed. Sibling pairs were established as inclusion criteria. A severity score was defined. RESULTS: A total of 133 patients with HUS were recorded; 40 had siblings and 4 progressed to HUS (10%). The mean age of the 4 sibling pairs was 29.3 months old (SD ± 11.5); 5 (62.5%) were girls. The mean time between each case was 5.7 days (SD ± 3). HUS was more severe in the siblings who became infected in the second place. CONCLUSION: The risk of HUS transmission between siblings was 10%, and the clinical course of the second sibling was less favorable.

INTRODUCCIÓN: El síndrome urémico hemolítico (SUH) es una entidad infectocontagiosa producida por Escherichia coli productora de toxina Shiga. El objetivo fue evaluar el riesgo de contagio y evolución clinica entre hermanos con SUH típico. POBLACIÓN Y MÉTODOS: Se analizaron las historias clínicas de los niños con SUH típico desde 1997 a 2012. Se utilizó, como criterio de inclusión, a parejas de hermanos. Se definió un puntaje de gravedad. RESULTADOS: Se registraron 133 pacientes con SUH; 40 tenían hermanos y 4 progresaron a SUH (10%). La edad media de los 4 pares de hermanos fue de 29,3 meses (DE ± 11,5) y 5 (62,5%) eran niñas. El tiempo medio entre casos fue de 5,7 días (DE ± 3). El SUH presentó un mayor puntaje de gravedad en los hermanos que se enfermaron en segundo lugar. CONCLUSIÓN: El riesgo para desarrollar SUH entre hermanos fue 10% y la evolución clínica del segundo hermano fue más desfavorable.