Kidney function in patients with primary distal renal tubular acidosis.

BACKGROUND: Recent reports indicate that chronic reduction of glomerular filtration rate (GFR) is common in patients with distal renal tubular acidosis (DRTA). Factors responsible for decreased GFR need clarification. METHODS: We reviewed records of 25 patients with genetically confirmed DRTA included in the RenalTube database. Patients < 18 years at diagnosis and having at least one annual follow-up were selected and classified in two groups according to GFR ≥ 90 (normal GFR) or < 90 mL/min/1.73 m2 (low GFR) after median follow-up of 8.8 years. RESULTS: Eighteen and seven patients had normal and low GFR (X ± SEM, 121.16 ± 28.87 and 71.80 ± 10.60 mL/min/1.73 m2, respectively, p < 0.01). At diagnosis, these 2 subgroups did not differ in sex, age, underlying mutated gene, GFR, height SDS, or percentage of ultrasound nephrocalcinosis. Serum creatinine (SCr) was different but likely due to median ages of presentation being 0.6 and 4.0 in normal and low GFR patients, respectively. On the last recorded visit, no differences between both groups were found in serum bicarbonate, serum potassium, or alkali dosage. Height SDS of patients with normal GFR was - 0.15 ± 0.47 whereas it was - 1.06 ± 0.60 in the low GFR group (p = 0.27). Interestingly, 23% of the whole group had low birth weight (LBW; < 2500 g), equating to 20% and 29% in the normal and low GFR patients, respectively (p = 0.65). CONCLUSIONS: Our findings confirm the risk of kidney function reduction in patients with DRTA of pediatric age onset, suggesting that low GFR is related with less favorable growth outcome and discloses the high frequency of LBW in primary DRTA, a hitherto unrecognized feature.

Corticosteroids for septic arthritis in children.

BACKGROUND: Septic arthritis is an acute infection of the joints characterised by erosive disruption of the articular space. It is the most common non-degenerative articular disease in developing countries. The most vulnerable population for septic arthritis includes infants and preschoolers, especially boys. Septic arthritis disproportionately affects populations of low socioeconomic status. Systemic corticosteroids and antibiotic therapy may be beneficial for treatment of septic arthritis. Even if the joint infection is eradicated by antibiotic treatment, the inflammatory process may produce residual joint damage and sequelae. OBJECTIVES: To determine the benefits and harms of corticosteroids as adjunctive therapy in children with a diagnosis of septic arthritis. SEARCH METHODS: We searched MEDLINE, Embase, the Cochrane Central Register of Controlled Trials (CENTRAL), in the Cochrane Library, Latin American Caribbean Health Sciences Literature (LILACS), the World Health Organization (WHO) trials portal (www.who.int/ictrp/en/), ClinicalTrials.gov (www.ClinicalTrials.gov), and Google Scholar. We searched all databases from their inception to 17 April 2018, with no restrictions on language of publication. SELECTION CRITERIA: We included randomised controlled trials (RCTs) with patients from two months to 18 years of age with a diagnosis of septic arthritis who were receiving corticosteroids in addition to antibiotic therapy or as an adjuvant to other therapies such as surgical drainage, intra-articular puncture, arthroscopic irrigation, or debridement. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed eligibility, data extraction, and evaluation of risk of bias. We considered as major outcomes the presence of pain, activities of daily living, normal physical joint function, days of antibiotic treatment, length of hospital stay, and numbers of total and serious adverse events. We used standard methodological procedures expected by Cochrane. We assessed the evidence using GRADE (Grading of Recommendations Assessment, Development and Evaluation) and created a 'Summary of findings' table. MAIN RESULTS: We included two RCTs involving a total of 149 children between three months and 18 years of age who were receiving antibiotics for septic arthritis. The most commonly affected joints were hips and knees. These studies were performed in Costa Rica and Israel. In both studies, dexamethasone administered intravenously (ranging from 0.15 to 0.2 mg/kg/dose every six to eight hours) during four days was the corticosteroid, and the comparator was placebo. Trials excluded patients with any degree of immunodeficiency or immunosuppression. The longest follow-up was one year. Trials did not report activities of daily living nor length of hospital stay. Both studies used adequate processes for randomisation, allocation concealment, and blinding, and review authors judged them to have low risk of selection and performance bias. Losses to follow-up were substantive in both studies, and we judged them to have high risk of attrition bias and of selective outcome reporting. We graded all outcomes as low quality due to concerns about study limitations and imprecision.The risk ratio (RR) for absence of pain at 12 months of follow-up was 1.33, favouring corticosteroids (95% confidence interval (CI) 1.03 to 1.72; P = 0.03; number needed to treat for an additional beneficial outcome (NNTB) = 13, 95% CI 6 to 139; absolute risk difference 24%, 95% CI 5% to 43%).The RR for normal function of the affected joint at 12 months of follow-up was 1.32, favouring corticosteroids (95% CI 1.12 to 1.57; P = 0.001; NNTB = 13, 95% CI 7 to 33; absolute risk difference 24%, 95% CI 11% to 37%).We found a reduction in the number of days of intravenous antibiotic treatment favouring corticosteroids (mean difference (MD) -2.77, 95% CI -4.16 to -1.39) based on two trials with 149 participants.Researchers did not report length of hospital stay. One trial (49 participants) reported that treatment with dexamethasone was associated with a shorter duration of IV antibiotic treatment, leading to a shorter hospital stay, and although duration of hospitalisation was a primary outcome of the study, study authors did not provide data on the duration of hospitalisation. We downgraded the quality by one level for concerns about study limitations (high risk of attrition bias and selective reporting), and by another level for imprecision.In one trial of 49 participants, researchers followed 29 children for 12 months, and parents reported that no children demonstrated adverse effects of the intervention. AUTHORS' CONCLUSIONS: Evidence for corticosteroids as adjunctive therapy in children with a diagnosis of septic arthritis is of low quality and is derived from the findings of two trials (N = 149). Corticosteroids may increase the proportion of patients without pain and the proportion of patients with normal function of the affected joint at 12 months, and may also reduce the number of days of antibiotic treatment. However, we cannot draw strong conclusions based upon these trial results. Additional randomised clinical trials in children with relevant outcomes are needed.

Genetic and radiological aspects of pediatric renal cystic disease: A case series / Aspectos genéticos e imagenológicos de la enfermedad quística renal en pediatría: serie de casos

Renal cystic diseases are common conditions whose etiology can be highly heterogeneous. They require a correct approach for adequate diagnosis and management. We aimed to illustrate part of the spectrum of renal cystic diseases through some clinical cases managed in our service. We describe 11 clinical cases including clinical entities such as renal multicystic dysplasia, and autosomal dominant and autosomal recessive polycystic renal disease, among other pathologies. Renal cystic diseases are heterogeneous in their clinical presentation, natural history, radiological findings, and genetic and pathophysiological basis. An integral clinical approach is needed to get a clear etiological diagnosis and offer adequate individualized care and follow-up for patients.

Las enfermedades quísticas renales son condiciones frecuentes cuya etiología puede ser muy heterogénea, por lo que se requiere un adecuado abordaje para su diagnóstico y manejo. El objetivo de este trabajo fue ilustrar parte del espectro de la enfermedad renal quística por medio de casos clínicos manejados en la Fundación Valle del Lili. Se describen 11 casos clínicos que incluyen enfermedades como displasia multiquística renal, enfermedad poliquística renal autosómica dominante y autosómica recesiva, entre otras. Las enfermedades quísticas renales varían en su presentación clínica, historia natural, hallazgos imagenológicos, bases genéticas y fisiopatológicas, por consiguiente, el enfoque diagnóstico y el manejo integral se debe realizar de forma individualizada y con un abordaje multidisciplinario.

Schimke immuno-osseous dysplasia. A case report in Colombia.

Background: Schimke immune-osseous dysplasia (SIOD) is an ultra-rare multisystemic, monogenic, and autosomal recessive inherited disease caused by biallelic mutations in the SMARCAL1 gene. Approximately 100 cases have been reported worldwide. The disease is characterized by skeletal, renal, and immunological abnormalities. Case description: This is a 6-year-old female patient who debuted with nephrotic syndrome at five years of age, with a switch to corticosteroid resistance and poor response to immunosuppressive treatment received. The patient was admitted and referred to our institution due to convulsive status. During her hospitalization, thrombosis was found in the left renal vein, and a renal biopsy report of Collapsing Focal and Segmental Glomerulosclerosis (FSGS) was obtained. The patient had multiple infections during hospitalization, with T lymphocyte lymphopenia and severe IgG hypogammaglobulinemia. Additionally, given dysmorphic facies, delayed weight-height development, and spondyloepiphyseal dysplasia, exome sequencing was performed, finding an homozygous pathogenic variant c.1933C > T p.Arg645Cys in SMARCAL1, compatible with the diagnosis of SIOD. Discussion: We present the case of a patient that exhibited a severe phenotype of the disease, with skeletal, renal, severe combined immunological compromise and cerebrovascular involvement during follow-up, and the available proposed mechanisms of the disease focused on the clinical manifestations of this patient. It is the first case of SIOD reported in Colombia and the first comprehensive characterization reported in the literature of a patient with homozygosity of the known variant c.1933C > T p.Arg645Cys. Conclusion: A severe phenotype of the disease with cerebrovascular involvement by homozygosity of the known variant c.1933C > T p.Arg645Cys in the SMARCAL1 gene can be expected.

B-cell activating factor (BAFF) and its receptors' expression in pediatric nephrotic syndrome is associated with worse prognosis.

AIM: Immune pathogenesis of nephrotic syndrome (NS) is not completely understood. We aimed to evaluate the expression of B-cell activating factor (BAFF) and its receptors in renal samples from pediatric NS patients and its relationship with renal function survival. MATERIALS AND METHODS: We conducted an ambispective study on 33 patients with pediatric NS. Immunohistochemistry for BAFF, TACI, BCMA and BR3 was performed. Markers were evaluated on podocytes and interstitial inflammatory infiltrates (III). We performed Kaplan-Meier curves to describe renal function survival according to markers' expression. RESULTS: Thirty-three NS patients were included. Minimal change disease was seen in 21 (63.6%) patients, and focal segmental glomerulosclerosis in 12 (36.4%). BAFF was found in podocytes (18.2% of samples) and III (36.4% of samples), BAFF-R in one sample, TACI in 4 (podocytes and III), and BCMA in 5 samples of podocytes and 7 of III. BAFF on podocytes and III was associated with worst renal function at follow-up; those patients had 25% probability of having GFR >90 mL/min/1.73m2, versus 84.9% when absent (p = 0.0067). Patients with BAFF in III had 42.9% probability of having GFR>90 mL/min/1.73 m2, versus 94.1% when absent (p = 0.0063). CONCLUSION: BAFF expression in renal biopsies could be a prognostic factor for renal function.

New PAX2 Mutation Associated with Polycystic Kidney Disease: A Case Report.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of end stage renal disease in children. Diagnosis by genetic testing has proven challenging due to its genetic and phenotypic heterogeneity, as well as incomplete penetrance. We report a case on a 16-months old female with a history of renal cysts and a PAX2 mutation. CASE PRESENTATION: The patient presented with a prenatal diagnosis of Potter sequence and a postnatal diagnosis of renal cysts. An ultrasound at 20 weeks gestation revealed right renal agenesis and possible left renal dysplasia. Post natal genetic analyses identified a novel mutation in PAX2. CONCLUSION: Cystic kidney disease is often underdiagnosed due to its variable expressivity and wide range of clinical manifestations; PAX2 genetic screening should be considered for all patients with CAKUT.

PHEX Gene Mutation in a Patient with X-Linked Hypophosphatemic Rickets in a Developing Country.

INTRODUCTION: X-linked hypophosphatemic rickets is part of a larger group of hereditary diseases characterized by renal phosphate loss, which causes growth disorders, rickets, and osteomalacia. These conditions are characterized by disorders in phosphate equilibrium, which is essential for bone formation. CASE REPORT: A female patient presented with bone deformities of the inferior extremities, prominent joints, and loss of teeth. She received initial management with oral calcium and orthotics in inferior extremities, with poor clinical outcome. PHEX gene sequencing revealed a pathogenic variant c.1601C>T (p.Pro534Leu). DISCUSSION: XLHR is caused by mutations in the PHEX gene; to date, more than 460 mutations have been associated with the disease. Clinically, it is characterized by bowing of the lower extremities, decreased growth, musculoskeletal complaints, dental abscesses, and other clinical signs and symptoms of rickets.

Acute renal failure in children. Multicenter prospective cohort study in medium-complexity intensive care units from the Colombian southeast.

BACKGROUND: Acute kidney injury is frequent in critically ill children; however, it varies in causality and epidemiology according to the level of patient care complexity. A multicenter prospective cohort study was conducted in four medium-complexity pediatric intensive care units from the Colombian southeast aimed to estimate the clinical prognosis of patients with diagnosis of acute kidney injury. METHODS: We included children >28 days and <18 years of age, who were admitted with diagnosis of acute kidney injury classified by Kidney Disease Improving Global Outcomes (KDIGO), during the period from January to December 2017. Severe acute kidney injury was defined as stage 2 and stage 3 classifications. Maximum KDIGO was evaluated during the hospital stay and follow up. Length of hospital stay, use of mechanical ventilation and vasoactive drugs, use of renal replacement therapy, and mortality were assessed until discharge. RESULTS: Prevalence at admission of acute kidney injury was 5.2% (95%CI 4.3% to 6.2%). It was found that 71% of the patients had their maximum KDIGO on day one; an increment in the maximum stage of acute kidney injury increased the pediatric intensive care unit stay. Patients with maximum KDIGO 3 were associated with greater use of mechanical ventilation (47%), compared with maximum KDIGO 2 (37%) and maximum KDIGO 1 (16%). Eight patients with maximum KDIGO 2 and 14 with maximum KDIGO 3 required renal replacement therapy. Mortality was at 11.8% (95%CI 6.4% to 19.4%). CONCLUSION: Acute kidney injury, established and classified according to KDIGO as severe and its maximum stage, was associated with worse clinical outcomes; early therapeutic efforts should focus on preventing the progression to severe stages.

Aspectos genéticos e imagenológicos de la enfermedad quística renal en pediatría: serie de casos / Genetic and radiological aspects of pediatric renal cystic disease: A case series

Las enfermedades quísticas renales son condiciones frecuentes cuya etiología puede ser muy heterogénea, por lo que se requiere un adecuado abordaje para su diagnóstico y manejo. El objetivo de este trabajo fue ilustrar parte del espectro de la enfermedad renal quística por medio de casos clínicos manejados en la Fundación Valle del Lili. Se describen 11 casos clínicos que incluyen enfermedades como displasia multiquística renal, enfermedad poliquística renal autosómica dominante y autosómica recesiva, entre otras. Las enfermedades quísticas renales varían en su presentación clínica, historia natural, hallazgos imagenológicos, bases genéticas y fisiopatológicas, por consiguiente, el enfoque diagnóstico y el manejo integral se debe realizar de forma individualizada y con un abordaje multidisciplinario.

Renal cystic diseases are common conditions whose etiology can be highly heterogeneous. They require a correct approach for adequate diagnosis and management. We aimed to illustrate part of the spectrum of renal cystic diseases through some clinical cases managed in our service. We describe 11 clinical cases including clinical entities such as renal multicystic dysplasia, and autosomal dominant and autosomal recessive polycystic renal disease, among other pathologies. Renal cystic diseases are heterogeneous in their clinical presentation, natural history, radiological findings, and genetic and pathophysiological basis. An integral clinical approach is needed to get a clear etiological diagnosis and offer adequate individualized care and follow-up for patients.

Algoritmo de manejo hospitalario para la intoxicación aguda por Paraquat® en población pediátrica, serie de casos / A hospital management algorithm for acute poisoning by Paraquat® in a pediatric population, a series of cases

Resumen El dicloruro de 1,1'-dimetil-4,4'-bipiridilo (Paraquat®) es un compuesto químico de la familia de las piridinas, utilizado como herbicida no selectivo y desecante. Este compuesto puede causar intoxicación aguda por todas las vías de exposición. En el momento, no hay un antídoto conocido y los tratamientos disponibles, incluidos los pediátricos, se basan en contrarrestar su absorción y propiciar su remoción oportuna. Se describe una serie de casos de 14 pacientes pediátricos, procedentes en su mayoría del departamento del Cauca, con intoxicación aguda por ingestión de paraquat. Los pacientes fueron remitidos y atendidos en un hospital de mediana a alta complejidad en el suroccidente colombiano, con un protocolo institucional para el manejo de la intoxicación aguda por el herbicida. La intoxicación aguda con paraquat por vía oral se asocia con una alta tasa de mortalidad, aún con atención médica oportuna, pues fácilmente se alcanzan concentraciones sistémicas para ser fulminante. Basado en la literatura disponible, el Hospital Universitario San José ha propuesto un protocolo clínico -adecuado para la intoxicación aguda por paraquat en población pediátrica- que incluye manejo estándar temprano, tratamiento inmunosupresor y antioxidante, y técnicas para su remoción sistémica

Abstract Paraquat®, or N,N'-dimethyl-4,4'-bipyridinium dichloride, is a bipyridyl compound used as a non-selective herbicide and desiccant that can cause acute poisoning through all routes of exposure. There is no known antidote, and the available treatments are based on avoiding its absorption and timely removing it, in adults and children. We describe a case series of 14 pediatric patients from the department of Cauca, Colombia, with acute intoxication after oral intake of paraquat. Patients were referred to a medium-high complexity hospital in southwestern Colombia and treated according to an institutional protocol for acute paraquat poisoning. Acute paraquat poisoning after oral ingestion is associated with a high mortality rate, even with timely medical attention, as the compound has no known antidote and quickly reaches systemic concentrations for fulminant poisoning. Based on the available literature, our center has proposed a clinical protocol including early standard management, immunosuppressive and antioxidant treatments, and systemic removal techniques. This protocol suggests an adequate approach to acute paraquat poisoning in the pediatric population.

Proteinuria en la infancia: a propósito de un caso de enfermedad de Dent

Introducción: la proteinuria en la edad pediátrica es una entidad relativamente frecuente, la cual puede ser fisiológica o patológica. La segunda, por una alteración a nivel glomerular con pérdida de proteínas de gran tamaño o a nivel tubular, caracterizada por pérdida de proteínas de bajo peso molecular y alteraciones en la excreción de iones. Entre las enfermedades hereditarias que cursan con proteinuria tubular, se ha descrito la enfermedad de Dent, una patología ligada al cromosoma X. Esta enfermedad se manifiesta principalmente en varones, pero las mujeres pueden ser portadoras y tener manifestaciones clínicas leves de la enfermedad. La primera descripción de esta enfermedad fue hecha por Dent y Friedman en 1964. La mayoría de los casos recientemente reportados han sido en China y Alemania. Objetivo: realizar una revisión general de la enfermedad de Dent y del enfoque diagnóstico de la proteinuria en la infancia con base en nuestro caso, para así, sospechar de esta enfermedad. Descripción del caso: se presenta el caso de un paciente masculino sin antecedentes prenatales ni personales de importancia, quien presenta proteinuria persistente desde los primeros meses de vida y a quien, a los 7 años de edad, se le documenta la presencia de una variante ya conocida en el gen CLCN5, causante de la enfermedad de Dent tipo 1. Discusión: la proteinuria persistente patológica en la infancia debe ser estudiada debido a su posible relación con patologías que pueden afectar la función renal. Además de la diferenciación de la proteinuria persistente, de origen glomerular y tubular, la evaluación de alteraciones en la excreción de electrolitos, puede guiarnos hacia la realización de estudios genéticos y, por ende, al diagnóstico de patologías infrecuentes como la enfermedad de Dent. Conclusión: el enfoque diagnóstico de causas poco frecuentes de proteinuria tubular en la infancia, como la enfermedad de Dent, requiere de la valoración conjunta entre nefrología pediátrica y genética clínica.

Background: In pediatric patients, proteinuria is a relatively frequent entity that can be physiological or pathological. The second one, due to an alteration at the glomerular level with the loss of large proteins or at the tubular level, characterized mainly by the loss of low molecular weight proteins and changes in the excretion of ions. Among the hereditary diseases that present with tubular proteinuria, Dent disease is a disease linked to the X chromosome. Therefore, it manifests essentially in males, but women can be carriers and have minor clinical manifestations of the disease. Dent and Friedman made the first description of this disease in 1964. Recently, most of the cases have been reported in China and Germany. Objective: To perform a revision of Dent disease, as well as the diagnostic approach of childhood proteinuria based in our case in order to suspect this disease. Case description: This is the case of a masculine patient, without relevant prenatal and personal antecedents, the son of a father with polycystic renal disease, who presents persistent proteinuria from the first months of life, and who, at seven years old, the presence of a variant in the CLCN5 gene -causing of type 1 Dent disease- was documented. Discussion: The persistent pathological proteinuria in childhood must be studied due to its possible relation with pathologies that could affect renal function. Moreover, the differentiation among glomerular and tubular proteinuria can guide us to perform additional studies, including genetic tests to diagnose infrequent pathologies like Dent disease. Conclusion: The diagnostic approach to rare causes of tubular proteinuria in childhood, such as Dent's disease, requires joint assessment between pediatric nephrology and clinical genetics.

Small vessel vasculitis, different renal outcomes in pediatric patients. Case reports

Vasculitis mainly affects the walls of the blood vessels, and is an uncommon disease in the pediatric population. In general, they are classified according to the EULAR / PreS consensus in children and in adults according to the Chapel-Hill consensus conference. ANCA-associated vasculitis (AAV) is part of small-vessel disease and is represented by granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and others. The representative renal histopathological findings are focal necrotizing glomerulonephritis with crescents, variable interstitial inflammation, absence of immune complexes, or small deposits of immunoglobulins. Clinically, AAV can manifest with hematuria, proteinuria, high blood pressure, and/or rapidly progressive glomerulonephritis. GPA can severely affect the kidney in 75% of cases. In MPA, renal involvement (75-90%) can be rapid and severe with the possibility of requiring renal replacement therapy in more than half of the patients. Furthermore, up to 25% of patients may have high blood pressure, and the mortality at one year can be up to 85%. In EGPA the renal involvement is usually mild. Three pediatric cases of AAV with different renal outcomes are presented, including the need for renal replacement therapy with the recovery of renal function, kidney transplantation, and death, followed in a fourth level of care institution in Colombia.

Las vasculitis, patologías cuyo hallazgo principal es la afectación de las paredes de los vasos sanguíneos, se presentan de forma infrecuente en la población pediátrica. En general, en niños se clasifican de acuerdo con el consenso de la EULAR/PReS, y en adultos, según la Conferencia de Consenso de Chapel-Hill. Las vasculitis asociadas con ANCA (VAA) hacen parte de las vasculitis de pequeños vasos y están representadas por la granulomatosis con poliangeítis (GPA), la granulomatosis eosinofílica con poliangeítis (EGPA) y la poliangeítis microscópica (PAM), entre otras. A nivel renal, los hallazgos histopatológicos representativos son la glomerulonefritis focal necrotizante con media luna, inflamación intersticial variable, ausencia de complejos inmunes o pequeños depósitos de inmunoglobulinas. Clínicamente, las VAA pueden manifestarse con hematuria, proteinuria, hipertensión arterial o glomerulonefritis rápidamente progresiva. La GPA puede afectar de forma severa el riñón en el 75% de los casos, mientras que, en la PAM, el compromiso renal (75-90%) puede ser rápido y severo con posibilidad de requerir terapia de reemplazo renal en más de la mitad de los pacientes. Además, hasta el 25% de los casos puede tener hipertensión arterial, con una mortalidad a un ario de 85%. En la EGPA, el compromiso renal suele ser leve. Se presentan 3 casos pediátricos de VAA con diferentes desenlaces renales, que incluyen necesidad de terapia de reemplazo renal con recuperación de función renal, trasplante renal y muerte, seguidas en una institución de IV nivel del suroccidente colombiano.

Agreement between biopsy and renal function in pediatric patients with lupus nephritis. A retrospective study / Acuerdo entre biopsia y función renal en pacientes pediátricos con nefritis lúpica. Un estudio retrospectivo

ABSTRACT Introduction: Lupus nephritis (LN) is a consequence of Systemic Lupus Erythematosus (SLE). Renal biopsy is a potential prognostic biomarker for renal function. Objective: To correlate histopathological findings and renal function in children with LN. Materials and methods: A retrospective observational study was conducted on children with a histopathological diagnosis of NL. Patients with no follow-up registered were excluded. The kidney biopsy at diagnosis was evaluated using the ISN/RPS scale. The Kappa index was used to determine the level of agreement between renal failure (Glomerular Filtration Rate [GFR] < 60 mL/min/1.73 m2) and presence or absence of each index on the modified ISN/RPS scale. Results: A total of 57 patients with NL were treated from 2011 to 2018 at the institution. Of these, 40 (70%) met inclusion criteria, and 10 (25%) were male. The median age of NL diagnosis was 12.9 years (IQR, 11.1-14.9). Follow-up time was 2.3 years (IQR, 1.0-5.16). At diagnosis, karyorrhexis was the characteristic with highest level of agreement with renal failure (k = 0.1873 SE = 0.0759 P = .0068) and at the last follow-up, it was global segmental sclerosis (k = 0.1481 SE = 0.078 P = .0287). There was no difference in the GFR at the last follow-up and the presence of proteinuria at diagnosis (P = .3936). Conclusion: Renal biopsy findings may be an insufficient tool to predict renal function. Treat ment and prognosis of patients with NL should be done using other biomarkers and clinical signs. Prospective studies should be performed to confirm this hypothesis.

RESUMEN Introducción: La nefritis lúpica (NL) es una consecuencia del lupus eritematoso sistémico (LES). La biopsia renal es un potencial biomarcador de pronóstico de función renal. Objetivo: Correlacionar hallazgos histopatológicos y la función renal de los pacientes pediátricos con la NL. Materiales y métodos: Estudio observacional retrospectivo. Se incluyeron pacientes pediátricos con diagnóstico histopatológico de NL. Se excluyeron pacientes sin seguimiento por la institución. Se evaluó la biopsia renal al diagnóstico con la escala modificada de la International Society of Nephrology and the Renal Pathology Society (ISN/RPS). Se usó el índice kappa para determinar el nivel de acuerdo entre la falla renal (tasa de filtración glomerular [TFG] < 60 mL/min/1,73 m2) y la presencia o ausencia de cada índice de la escala modificada de la ISN/RPS. Resultados: Entre el 2011 y el 2018, 57 pacientes con NL fueron atendidos en la institución, 40 cumplieron con los criterios de inclusión, 10 (25%) eran de sexo masculino. La mediana de edad de diagnóstico de NL fue 12,9 arios (IQR 11,1 a 14,9). El tiempo de seguimiento fue de 2,3 años (IQR 1,0 a 5,16). Al diagnóstico, la cariorexis fue la característica de la escala con mayor nivel de acuerdo con la falla renal (k = 0,1873, EE = 0,0759, p = 0,0068) y al último seguimiento lo fue la esclerosis segmentaria global (k = 0,1481, EE = 0,078, p = 0,0287). No hubo diferencia en la TFG al último seguimiento y presencia de proteinuria al diagnóstico (p = 0,3936). Conclusión: La biopsia renal puede ser insuficiente para evaluar la predicción de la función renal. El tratamiento de pacientes con NL debe realizarse utilizando otros biomarcadores y signos clínicos. Deben hacerse estudios prospectivos que puedan confirmar esta hipótesis.

Nefropatía por inmunoglobulina A en pediatría, desenlaces clínicos con diferentes esquemas de manejo / IgA nephropathy in pediatric patients, clinical outcomes with three types of management

Resumen Introducción: la nefropatía por inmunoglobulina A (NIgA) es la enfermedad glomerular más común en el mundo. En Colombia, el 11-22 % de las glomerulonefritis primarias en niños corresponden a NIgA y de estos casos, el 30 % progresa a enfermedad renal terminal. Objetivo: describir las características paraclínicas e histopatológicas de la NIgA, así como los resultados clínicos según tres tipos de tratamiento en pacientes pediátricos con esta enfermedad atendidos en un hospital de alta complejidad del suroccidente colombiano. Materiales y métodos: estudio retrospectivo realizado en pacientes pediátricos de entre 1 mes y 18 años de edad con diagnóstico de NIgA. Las variables categóricas se presentaron como proporciones y las continuas con medianas y rango intercuartílico. Se usó la prueba de Fisher para comparar los tres esquemas de tratamiento. Resultados: se incluyeron 58 pacientes pediátricos atendidos entre 1996 y 2013. La media de edad al inicio de síntomas fue 7,5±4,2 años y al momento de la biopsia renal, 10±3,8 años. El 77,6 % de los pacientes presentó hematuria microscópica y el 27,6 %, macroscópica. Además, el 81 % tuvo proteinuria, siendo severa el 29 %. Histológicamente, el 10 % se clasificó como grado I, el 62 % como grado II, el 21 % como grado III y el 7 % como grado IV. Tres pacientes requirieron diálisis y dos, trasplante renal. Los esquemas terapéuticos evaluados fueron: solo prednisona (n=20, 34,5 %), prednisona y mofetil micofenolato (MMF) (n=13, 22,4 %) y sin prednisona ni MMF (n=25, 43,1 %). La diferencia en la presencia de hematuria entre los grupos fue significativa (p>0,001), siendo más frecuente en el grupo sin prednisona ni MMF (68 %). No hubo diferencia entre los grupos de proteinuria, hipertensión arterial y valor de creatinina. La mediana de años entre la biopsia renal y el ultimo control fue de 4 años (RIC 1-7). La supervivencia de la función renal fue del 89,1 % a los 5 años. Conclusión: la NIgA amerita reconocimiento temprano y seguimiento estricto, ya que puede tener desenlaces ominosos como enfermedad renal crónica.

Abstract Introduction: IgA nephropathy (IgAN) is the most common glomerular disease in the world, in Colombia belongs to 11-22 % of primary glomerulonephritis in pediatric patients. Of these, 30 % progress to chronic kidney disease. Materials and methods : It is a retrospective descriptive study. We used median and IRQ for continuous variables, and proportions for categorical variables, Fisher test to compare clinical outcomes. Results: Between 1996 to 2013 58 patients were diagnosed. The mean age at symptoms onset was 7.5 years (SD±4.2) and at the time of renal biopsy was 10 years (SD±3.8). At diagnosis, 77.6 % of the patients showed microscopic hematuria, 27.6 % gross hematuria and 81 % proteinuria, classified as severe in 29 %. Three patients required dialysis and two needed kidney transplant. Three groups with different therapeutic regimens were evaluated: first group only prednisone 34.5 % (n = 20), second group prednisone and mycophenolate mofetil (MMF) 22.4 % (n = 13) and third group without prednisone neither MMF 43.1 % (n = 25). The difference in the presence of hematuria among the groups was significant (p> 0.001), being more frequent in the group without prednisone neither MMF (68 %). There were no significant differences in proteinuria, hypertension or creatinine among the groups. The median of years between the renal biopsy and the last control was 4 years RI 1-7. At five years, the renal function survival probability (GFR >90 ml/min/1.73m2) was 89.1 %. Conclusion: IgAN needs early recognition and strict follow-up, since it may have ominous outcomes.