AA amyloidosis associated with morbid obesity (clinical case) / Amiloidosis AA asociada a la obesidad mórbida (caso clínico)

We present the case of a 45-year-old woman who was hospitalized due to severe macrocytic anemia and renal failure. The patient presented a morbid obesity. The immunological study showed anti-ENA anti-SSA (Ro52) positive, with negative antinuclear antibodies. Also in the proteinogram (serum immunofixation) the presence of monoclonal bands IgG lambda and IgG kappa, monoclonal component 7.2% (4.68g/L), with elevation of free light chains (kappa 95.94mg/L (3.3-19.4), evidenced, lambda 145.17mg/L (5.71-26.3)). The bone marrow study showed an infiltration of 5% of plasma cells and positive for AA amyloid. Finally, a percutaneous renal biopsy was performed, which again showed amyloid infiltration. In the genetic study, 2 mutations of the family Mediterranean fever gene (MEFV) have been identified. Secondary AA amyloidosis has been described associated with obesity, in addition to a percentage of cases of unknown etiology

Presentamos el caso de una mujer de 45 años que fue hospitalizada debido a una anemia macrocítica severa e insuficiencia renal. El paciente presentaba una obesidad mórbida. El estudio inmunológico mostró positividad para anti-ENA, anti-SSA (Ro52) y negatividad para anticuerpos antinucleares. También en el proteinograma (inmunofijación sérica) se detectó la presencia de bandas monoclonales IgG lambda e IgG kappa, con un componente monoclonal del 7,2% (4,68g/l) y la elevación de cadenas ligeras libres (kappa 95,94mg/l [3,3-19,4]; lambda 145,17mg/l [5,71-26,3]). El estudio de biopsia de médula ósea mostró una infiltración del 5% de células plasmáticas y positividad para amiloide AA. Finalmente, se realizó una biopsia renal que nuevamente mostró infiltración amiloide. En el estudio genético se identificaron 2 mutaciones del gen de la fiebre mediterránea familiar (MEFV). La amiloidosis secundaria AA se ha descrito asociada a la obesidad, además de un porcentaje de casos de etiología desconocida

AA amyloidosis associated with morbid obesity (clinical case).

We present the case of a 45-year-old woman who was hospitalized due to severe macrocytic anemia and renal failure. The patient presented a morbid obesity. The immunological study showed anti-ENA anti-SSA (Ro52) positive, with negative antinuclear antibodies. Also in the proteinogram (serum immunofixation) the presence of monoclonal bands IgG lambda and IgG kappa, monoclonal component 7.2% (4.68g/L), with elevation of free light chains (kappa 95.94mg/L (3.3-19.4), evidenced, lambda 145.17mg/L (5.71-26.3)). The bone marrow study showed an infiltration of 5% of plasma cells and positive for AA amyloid. Finally, a percutaneous renal biopsy was performed, which again showed amyloid infiltration. In the genetic study, 2 mutations of the family Mediterranean fever gene (MEFV) have been identified. Secondary AA amyloidosis has been described associated with obesity, in addition to a percentage of cases of unknown etiology.

Utilidad de copeptina en el diagnóstico de síndrome coronario agudo en el departamento de urgencias de un hospital de tercer nivel / Usefulness of copeptin in the diagnosis of acute coronary syndrome in the emergency department of a tertiary hospital

Objetivos: Valorar la utilidad de copeptina (fragmento estable del precursor de vasopresina-arginina) en el diagnóstico diferencial del dolor torácico agudo de posible origen coronario. Material y métodos: Se han incluido en el estudio 82 pacientes que fueron evaluados inicialmente de acuerdo con el protocolo de pacientes con sospecha de síndrome coronario agudo (SCA) de nuestro Servicio de Urgencias, incluyendo la determinación de troponina y copeptina con seriación en admisión y a las 6 h. Resultados: Obtuvimos diferencias estadísticamente significativas en la concentración de copeptina a tiempo 0 entre los pacientes diagnosticados de SCASEST: 42,1±38,7pmol/L y los pacientes no SCASEST: 15,6±21,2pmol/L (p<0,01). Sin embargo, las diferencias no alcanzaron a ser estadísticamente significativas a las 6 h (p=0,093). El análisis del área bajo la curva ROC para la copeptina en los pacientes SCASEST a tiempo 0 fue de 0,713 con un intervalo de confianza del 95% de 0,592 a 0,834 y un grado de significación de p=0,001. Conclusiones: La concentración de copeptina representa un valor adicional en la diferenciación entre pacientes SCASEST y pacientes no SCASEST, así como entre pacientes SCA y pacientes con angina estable. El punto de corte de 10pmol/L proporciona los mejores valores de sensibilidad, valor predictivo negativo (VPN), cociente de probabilidad positivo (CPP) y cociente de probabilidad negativo (CPN) en el diagnóstico de pacientes SCASEST

Objectives: This study was conducted in order to evaluate the usefulness of copeptin (a stable fragment of the precursor of arginine vasopressin) in the differential diagnosis of acute chest pain of probable coronary origin. Material and methods: The study includes 82 patients who were initially evaluated according to the protocol of a patient with suspected acute coronary syndrome (ACS) in our Emergency Department, including the determination of troponin and copeptin with specimens taken on admission (time 0) and at 6h. Results: Statistically significant differences were observed in copeptin concentrations at time 0 among patients diagnosed with non-ST-segment elevation (NTEACS): 42.1±38.7pmol/L and non-NSTEACS patients: 15.6±21.2pmol/L (P<. 01). However, the differences did not reach statistical significance at 6h (P=.093). The analysis of the area under the ROC curve for Copeptin in NSTEACS patients at time 0 was 0.713, with a confidence interval of 95% from 0.592 to 0.834 and a significance level of P=.001. Conclusions: The concentration of copeptin represents an additional value in the differentiation between NSTEACS patients and non-NSTEACS patients, as well as between ACS patients and patients with stable angina. The cut-off point of 10pmol/L provides the best values for sensitivity, negative predictive value (NPV), positive likelihood ratio (LR+), and negative likelihood ratio (LR-) in the diagnosis of NSTEACS patients

[Usefulness of copeptin in the diagnosis of acute coronary syndrome in the emergency department of a tertiary hospital]. / Utilidad de copeptina en el diagnóstico de síndrome coronario agudo en el departamento de urgencias de un hospital de tercer nivel.

OBJECTIVES: This study was conducted in order to evaluate the usefulness of copeptin (a stable fragment of the precursor of arginine vasopressin) in the differential diagnosis of acute chest pain of probable coronary origin. MATERIAL AND METHODS: The study includes 82 patients who were initially evaluated according to the protocol of a patient with suspected acute coronary syndrome (ACS) in our Emergency Department, including the determination of troponin and copeptin with specimens taken on admission (time 0) and at 6h. RESULTS: Statistically significant differences were observed in copeptin concentrations at time 0 among patients diagnosed with non-ST-segment elevation (NTEACS): 42.1±38.7pmol/L and non-NSTEACS patients: 15.6±21.2pmol/L (P<. 01). However, the differences did not reach statistical significance at 6h (P=.093). The analysis of the area under the ROC curve for Copeptin in NSTEACS patients at time 0 was 0.713, with a confidence interval of 95% from 0.592 to 0.834 and a significance level of P=.001. CONCLUSIONS: The concentration of copeptin represents an additional value in the differentiation between NSTEACS patients and non-NSTEACS patients, as well as between ACS patients and patients with stable angina. The cut-off point of 10pmol/L provides the best values for sensitivity, negative predictive value (NPV), positive likelihood ratio (LR+), and negative likelihood ratio (LR-) in the diagnosis of NSTEACS patients.

Efecto de la obstrucción de la vía biliar en la concentración de lipoproteína(a) / Effect of biliary obstruction on lipoprotein(a) concentration

Objetivos Este estudio se diseñó para establecer la correlación entre la concentración de lipoproteína(a) [Lp(a)], apolipoproteínas y lípidos con los parámetros bioquímicos de función hepática en un grupo de pacientes con colestasis reversible, así como la concentración de estos parámetros una vez resuelto el proceso de obstrucción biliar. Material y métodos Se incluyeron en el estudio de forma prospectiva 18 adultos mayores de 17 años con colestasis extrahepática y se determinaron los parámetros de función hepática, así como los parámetros del metabolismo lipídico y lipoproteico, especialmente Lp(a) antes y después de la desobstrucción. Resultados La concentración de Lp(a) previa a la desobstrucción se correlacionó inversamente de forma estadísticamente significativa con la concentración de gamma glutamil transpeptidasa (coeficiente de correlación [r] = -0,757; p = 0,018). La concentración de Lp(a) (mediana = 2,66 mg/dl; rango intercuartílico = 5,62) se elevó de forma estadísticamente significativa tras la desobstrucción (mediana = 9,72 mg/dl; rango intercuartílico = 28,76; p = 0,001). Hubo descensos estadísticamente significativos tras la desobstrucción en las concentraciones de colesterol total y triglicéridos y ascensos estadísticamente significativos en colesterol HDL y apolipoproteína A-1. Conclusiones: La concentración de Lp(a) se encuentra disminuida durante la colestasis, a pesar de existir una importante hipercolesterolemia simultánea. La colestasis ejerce un papel causal en el descenso de Lp(a), ya que la desobstrucción de la vía biliar recupera las concentraciones de Lp(a). Nuestro estudio apoya el concepto de que los ácidos biliares ejercen un efecto represor en la síntesis de Lp(a) y abre un mecanismo para el tratamiento de la hiper Lp(a)

Objectives: This study was appointed to determine the correlation between the concentration of lipoprotein(a) [Lp(a)], apolipoproteins and lipids with biochemical parameters of liver function in a group of patients with reversible cholestasis. We have also determined the concentration of these parameters once solved the biliary obstruction process. Material and methods: Eighteen adults over 17 years with extrahepatic cholestasis were included in the study on a prospective basis, and we determined in them biochemical liver function parameters and lipoprotein metabolism parameters, particularly Lp(a) before and after unblocking. Results: The concentration of Lp(a) prior to desobstruction was inverse and statistically significantly correlated with the concentration of gamma glutamyl transpeptidase (correlation coefficient [r] = -0.757, P = .018). The concentration of Lp(a) (median = 2.66 mg/dL, interquartile range = 5,62) showed a statistically significant increase (median = 9.72 mg/dL, interquartile range = 28.76, P < .001), once the unblocking was performed. Concentrations of total cholesterol and triglycerides had a statistically significant decrease, and HDL cholesterol and apolipoproteinA-1 showed a statistically significant increase once the unblocking was carried out. Conclusions: The concentration of Lp(a) is decreased during cholestasis, although there is a significant simultaneous hypercholesterolemia. Cholestasis has a causal role in lowering Lp(a), because the unblocking of bile duct recovers Lp(a) concentration. Our study supports the concept that bile acids exert a controlling effect on the synthesis of Lp(a) and open a mechanism for the treatment of hyper Lp(a)

[Effect of biliary obstruction on lipoprotein(a) concentration]. / Efecto de la obstrucción de la vía biliar en la concentración de lipoproteína(a).

OBJECTIVES: This study was appointed to determine the correlation between the concentration of lipoprotein(a) [Lp(a)], apolipoproteins and lipids with biochemical parameters of liver function in a group of patients with reversible cholestasis. We have also determined the concentration of these parameters once solved the biliary obstruction process. MATERIAL AND METHODS: Eighteen adults over 17 years with extrahepatic cholestasis were included in the study on a prospective basis, and we determined in them biochemical liver function parameters and lipoprotein metabolism parameters, particularly Lp(a) before and after unblocking. RESULTS: The concentration of Lp(a) prior to desobstruction was inverse and statistically significantly correlated with the concentration of gamma glutamyl transpeptidase (correlation coefficient [r] = -0.757, P = .018). The concentration of Lp(a) (median = 2.66 mg/dL, interquartile range = 5,62) showed a statistically significant increase (median = 9.72 mg/dL, interquartile range = 28.76, P < .001), once the unblocking was performed. Concentrations of total cholesterol and triglycerides had a statistically significant decrease, and HDL cholesterol and apolipoprotein A-1 showed a statistically significant increase once the unblocking was carried out. CONCLUSIONS: The concentration of Lp(a) is decreased during cholestasis, although there is a significant simultaneous hypercholesterolemia. Cholestasis has a causal role in lowering Lp(a), because the unblocking of bile duct recovers Lp(a) concentration. Our study supports the concept that bile acids exert a controlling effect on the synthesis of Lp(a) and open a mechanism for the treatment of hyper Lp(a).