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INTRODUCTION: Maturity-onset diabetes of the young (MODY) is a rare disease due to a single gene mutation that affects several family members in most cases. The Krüppel-like factor 11 (KLF11) gene mutation is associated with decreased insulin sensitivity to high glucose levels. KLF 11 has been implicated in the pathogenesis of MODY type 7 but given its low prevalence, prolonged subclinical period, and the emergence of new information, doubts are raised about its association. METHODS: A literature search of the PubMed, Scopus, and EBSCO databases was performed. The terms "Diabetes Mellitus, Type 2/genetics", "Mason-Type Diabetes" , "Maturity-Onset diabetes of the young", "KLF11 protein, human", and "Maturity-Onset Diabetes of the Young, Type 7" were used"., "Diagnosis" The search selection was not standardized. RESULTS: The KLF1 mutation is rare and represents <1% of the mutations associated with monogenic diabetes. Its isolation in European family lines in the first studies and the emergence of new variants pose new diagnostic challenges. This article reviews the definition, epidemiology, pathophysiology, diagnosis, and treatment of MODY type 7. CONCLUSION: MODY type 7 diabetes represents a rare form of monogenic diabetes with incomplete penetrance. Given its rarity, its association with impaired glucose metabolism has been questioned. Strict evaluation of glycemic control and the appearance of microvascular complications are key areas in the follow-up of patients diagnosed with MODY 7. More studies will be required to characterize the population with KLF11 mutation and clarify its correlation with MODY.

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Resumen Introducción: identificar las personas sin enfermedad cardiovascular establecida con alto riesgo es el objetivo de la intervención primaria en prevención cardiovascular. En nuestro medio se han aplicado múltiples escalas de riesgo cardiovascular; sin embargo, en Colombia la única escala validada es la de Framingham. Objetivo: estimar la concordancia entre las escalas Framingham ATP III, SCORE y ACC/ AHA 2013 para la predicción de riesgo cardiovascular en pacientes entre 40 y 75 años en una institución de cuarto nivel durante el año 2015. Material y métodos: estudio de tipo observacional de corte transversal en pacientes de 40 -75 años que asistieron durante el año 2015 en el servicio de chequeo general de un hospital de cuarto nivel, de 4783 individuos se tomó una muestra aleatorizada simple de 861, se calculó el riesgo cardiovascular con las escalas de Framingham, SCORE y AHA/ACC2013. Se describieron las variables cualitativas mediante distribuciones de frecuencias, las variables cuantitativas con medidas de tendencia central, se realizó un análisis bivariado con el coeficiente de correlación Kappa de Cohen considerándose como buena correlación >60. Resultados: el cálculo del riesgo cardiovascular con cada una de las escalas encontró para alto riesgo AHA 2013 de 14.6%, Framingham 2.2% y SCORE con 1.1%. Para riesgo medio SCORE de 26.9%, AHA 2013 de 17.1% y Framingham de 14.4%, y riesgo bajo la estimación fue de Framingham de 83.3%, SCORE de 73% y AHA 2013 68.3%. El índice de concordancia de Kappa de Cohen para alto riesgo cardiovascular entre la escala Framingham modificada y SCORE se evidencia fuerza de concordancia moderada (Kappa: 0.47) al calcular este índice entre Framingham modificada y AHA 2013 la fuerza concordancia es débil (Kappa: 0.3497). Conclusión: con los hallazgos del estudio se concluye que el comportamiento en cuanto a la estimación de riesgo de las escalas de SCORE y AHA 2013 no es concordante, por lo tanto, sus estimaciones no son intercambiables, tendiendo a sobreestimar o subestimar el riesgo. (Acta Med Colomb 2018; 43: 192-199).

Abstract Introduction: identifying people without established cardiovascular disease at high risk is the goal of primary intervention in cardiovascular prevention. In our environment, multiple scales of cardiovascular risk have been applied; however, in Colombia the only scale validated is Framingham. Objective: to estimate the agreement between the Framingham ATP III, SCORE and ACC / AHA 2013 scales for the prediction of cardiovascular risk in patients between 40 and 75 years old in a fourth level institution during 2015. Material and methods: cross-sectional observational study in patients aged 40-75 years who attended in 2015 in the general check-up service of a fourth-level hospital. Of 4783 individuals a simple randomized sample of 861 was taken. Cardiovascular risk with the Framingham, SCORE and AHA / ACC2013 scales was calculated. Qualitative variables were described by frequency distributions, quantitative variables with measures of central tendency, and a bivariate analysis was performed with Cohen's Kappa correlation coefficient considering as a good correlation > 60. Results: the calculation of cardiovascular risk with each of the scales found for high risk AHA 2013 of 14.6%, Framingham 2.2% and SCORE with 1.1%. For average risk SCORE of 26.9%, AHA 2013 of 17.1% and Framingham of 14.4%, and risk under the estimate was of Framingham of 83.3%, SCORE of 73% and AHA 2013 68.3%. The Cohen's Kappa concordance index for high cardiovascular risk between the modified Framingham scale and SCORE evidence a moderate concordance strength (Kappa: 0.47); when calculating this index between modified Framingham and AHA 2013 concordance strength is weak (Kappa: 0.3497). Conclusion: with the findings of the study it is concluded that the behavior regarding the risk estimation of SCORE and AHA 2013 scales is not concordant; therefore, their estimates are not interchangeable, tending to overestimate or underestimate the risk. (Acta Med Colomb 2018; 43: 192-199).

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