[Real-life efficacy and safety of PCSK9 inhibitors treatment: Experience in three hospitals in Asturias]. / Efectividad y seguridad del tratamiento con iPCSK9 en vida real: experiencia de tres hospitales asturianos.

BACKGROUND: Inhibitors of proprotein convertase subtilisin/kexin type9 (PCSK9 inhibitors) are a treatment option for those patients with familial hypercholesterolemia or in secondary prevention who do not reach the LDL-C target with other therapeutic measures. The aim of this study is to assess the effectiveness and safety of these drugs. METHODS: Retrospective, multicentric, descriptive study. We collected data from all patients that have started PCSK9 inhibitors treatment in three hospitals in Asturias since the beginning of its use in 2016. We analysed changes in lipid profile with PCSK9 inhibitors and its side effects. RESULTS: We registered 98 patients, 75 of them affected by familial hypercholesterolemia (FH) and 23 unaffected. Two months after the beginning of PCSK9 inhibitors treatment, a 61% reduction rate in LDL-C in patients with FH and 52% in those without this condition was observed. This statistically significant reduction remained stable during follow-up. A significant decrease in total cholesterol was observed, without significant changes in HDL-C and triglycerides. 96% of patients had no complications. CONCLUSIONS: PCSK9 inhibitors are safe drugs that rapidly achieve significant reductions in LDL-C after the beginning of treatment, which are maintained over time. Hence, the use of PCSK9 inhibitors is an alternative for the control of LDL-C in those patients in which the LDL-C target is not reached with other therapeutic measures.

[Home parenteral nutrition in Spain 2011 and 2012; a report of the home and ambulatory artificial nutrition group NADYA]. / Nutrición Parenteral Domiciliaria en España 2011 y 2012: informe del grupo de nutrición artificial domiciliaria y ambulatoria NADYA.

OBJECTIVE: To report the data of the Home Parenteral Nutrition (HPN) registry of the NADYA-SENPE working group for the years 2011 and 2012. METHODOLOGY: We compiled the data from the on-line registry introduced by reviewers of NADYA group responsible for monitoring of NPD introduced by since January 1, 2011 to december 31, 2012. Included fields were: age, sex, diagnosis and reason for HPN, access path, complications, beginning and end dates, complementary oral or enteral nutrition, activity level, autonomy degree, product and fungible material supply, withdrawal reason and intestinal transplant indication. RESULTS: Year 2010: 184 patients from 29 hospitals , representing a rate of 3.98 patients/million inhabitants/ year 2011, with 186 episodes were recorded NPD . During 2012, 203 patients from 29 hospitals , representing a rate of 4.39 patients/million inhabitants/year 2012 , a total of 211 episodes were recorded NPD . CONCLUSIONS: We observe an increase in registered patients with respect to previous years.Neoplasia remains as the main pathology since 2003. Although NADYA is consolidated registry and has been indispensable source of information relevant to the understanding of the progress of Home Artificial Nutrition in our country, there is ample room for improvement. Especially that refers to the registration of pediatric patients and the registration of complications.

Objetivo: Comunicar los datos del registro de Nutrición Parenteral Domiciliaria (NPD) del grupo de trabajo NADYA-SENPE de los años 2011 y 2012. Material y métodos: Recopilación de los datos del registro "on-line" introducidos por los colaboradores del grupo NADYA responsables del seguimiento de la NPD desde el 1 de enero de 2011 al 31 de diciembre de 2012 dividido por años naturales. Resultados: Año 2010: Se registraron 184 pacientes, procedentes de 29 hospitales, lo que representa una tasa de 3,98 pacientes/millón habitantes/año 2011, con 186 episodios de NPD. Durante el año 2012 se registraron 203 pacientes, procedentes de 29 hospitales, lo que representa una tasa de 4,39 pacientes/millón habitantes/año 2012, con un total de 211 episodios de NPD. Conclusiones: Se observa un aumento progresivo de los pacientes registrados respecto a años anteriores. El principal grupo patológico sigue siendo oncológico ocupando el primer lugar desde 2003. Aunque el registro NADYA es un registro consolidado y ha sido y es fuente imprescindible de información relevante para el conocimiento de los avances de la Nutrición Artificial Domiciliaria en nuestro país, queda un amplio margen para la mejora. En especial lo que hace referencia al registro de pacientes pediátricos y al registro de las complicaciones.

Nutrición Parenteral Domiciliaria en España 2011 y 2012; informe del grupo de nutrición artificial domiciliaria y ambulatoria NADYA / Home parenteral nutrition in Spain 2011 and 2012; a report of the home and ambulatory artificial nutrition group NADYA

Objetivo: Comunicar los datos del registro de Nutrición Parenteral Domiciliaria (NPD) del grupo de trabajo NADYA-SENPE de los años 2011 y 2012. Material y métodos: Recopilación de los datos del registro "on-line" introducidos por los colaboradores del grupo NADYA responsables del seguimiento de la NPD desde el 1 de enero de 2011 al 31 de diciembre de 2012 dividido por años naturales. Resultados: Año 2010: Se registraron 184 pacientes, procedentes de 29 hospitales, lo que representa una tasa de 3,98 pacientes/millón habitantes/año 2011, con 186 episodios de NPD. Durante el año 2012 se registraron 203 pacientes, procedentes de 29 hospitales, lo que representa una tasa de 4,39 pacientes/millón habitantes/año 2012, con un total de 211 episodios de NPD. Conclusiones: Se observa un aumento progresivo de los pacientes registrados respecto a años anteriores. El principal grupo patológico sigue siendo oncológico ocupando el primer lugar desde 2003. Aunque el registro NADYA es un registro consolidado y ha sido y es fuente imprescindible de información relevante para el conocimiento de los avances de la Nutrición Artificial Domiciliaria en nuestro país, queda un amplio margen para la mejora. En especial lo que hace referencia al registro de pacientes pediátricos y al registro de las complicaciones (AU)

Objective: To report the data of the Home Parenteral Nutrition (HPN) registry of the NADYA-SENPE working group for the years 2011 and 2012. Methodology: We compiled the data from the on-line registry introduced by reviewers of NADYA group responsible for monitoring of NPD introduced by since January 1, 2011 to december 31, 2012. Included fields were: age, sex, diagnosis and reason for HPN, access path, complications, beginning and end dates, complementary oral or enteral nutrition, activity level, autonomy degree, product and fungible material supply, withdrawal reason and intestinal transplant indication. Results: Year 2010: 184 patients from 29 hospitals, representing a rate of 3.98 patients/million inhabitants/ year 2011, with 186 episodes were recorded NPD. During 2012, 203 patients from 29 hospitals, representing a rate of 4.39 patients/million inhabitants/year 2012, a total of 211 episodes were recorded NPD. Conclusions: We observe an increase in registered patients with respect to previous years. Neoplasia remains as the main pathology since 2003. Although NADYA is consolidated registry and has been indispensable source of information relevant to the understanding of the progress of Home Artificial Nutrition in our country, there is ample room for improvement. Especially that refers to the registration of pediatric patients and the registration of complications (AU)

Metabolic effects of L-carnitine on type 2 diabetes mellitus: systematic review and meta-analysis.

INTRODUCTION: Carnitine is an endogenous metabolite and exogenous nutrient with a pivotal role in lipid metabolism. Plasma levels of carnitine are reduced in type 2 Diabetes Mellitus (T2DM). The aim was to evaluate the metabolic effects of the administration of L-carnitine in T2DM. METHOD: A systematic review was performed. Relevant randomized, controlled-trials trials were searched in Pubmed, Trip Database and Cochrane Library, and selected when they had enough methodological quality assessed with the Jadad scale. Article search strategy included "Carnitine" OR "L-carnitine" AND "Diabetes -Mellitus" OR "Diabetes mellitus, type 2" OR "Noninsulindependent-diabetes mellitus". Meta-analysis was performed, and the difference of means calculated with a 95% confidence interval. Heterogeneity was evaluated with the Q statistic. RESULTS: The systematic review included 4 trials with 284 patients. Oral L-carnitine lowered fasting plasma glucose [-14.3 mg/dl (CI95% - 23.2 to -5.4); p=0,002], total cholesterol [-7.8 mg/dL (95%CI -15.5 to -0.1); p=0.09], low density lipoprotein [-8.8 mg/dl (CI95% -12.2 to -8.5), p<0.0001], apolipoprotein-B100 [-7.6 mg/dl (CI95% -13.6 to -1.6); p=0.013] and apolipoprotein-A1 [-6.0 mg/dl (CI95% -10.5 a -1.5); p=0.523]. There was no significant heterogeneity. The changes in triglycerides, lipoprotein (a) or HbA(1c) were not significant. CONCLUSION: The administration of L-carnitine in type 2 diabetes mellitus is associated with an improvement in glycaemia and plasma lipids.

Registro del año 2010 de Nutrición Parenteral Domiciliaria en España: Grupo NADYA-SENPE / Home parenteral nutrition registry in Spain for the year 2010: NADYA-SENPE

Objetivos: Comunicar los datos del Registro del Grupo NADYA SENPE de Nutrición Parenteral Domiciliaria NPD en España del año 2010. Material y métodos: Estudio descriptivo de la base de datos del Registro de ámbito nacional de NPD del grupo NADYA-SENPE (1 de enero de 2010 al 31 de diciembre de 2010). Para el cálculo de prevalencias se utilizó los últimos datos publicados por el Instituto Nacional de Estadística. Resultados: Se registraron 148 pacientes procedentes de 23 hospitales, 86 mujeres (58,11%) y 9 niños (6,08%). La edad media de los 139 pacientes adultos fue de 53,06 ± 15,41 años. La duración media de la NPD fue de 316,97 días/paciente. El diagnóstico más frecuente en los niños (menores de 14 años) fue intestino corto traumático con 5 casos (55,55%) y en los adultos la neoplasia en tratamiento paliativo 29 (19,59%). El motivo de la indicación de la NPD fue el síndrome de intestino corto en 74 ocasiones (47%). La vía de acceso más frecuentemente registrada fue el catéter tunelizado en 36 (22,78%) casos seguido del reservorio en 13 (8,23%) y otras vías en 3 ocasiones (1,90%). Se registraron 23 infecciones relacionadas con el catéter (82,14%), lo que representa 0,49/1000 días de NP y todas ellas ocurrieron en los adultos. A lo largo del año finalizaron 24 episodios de NPD, la causa más frecuente fue el paso a la vía oral en 12 episodios (50%). Se registró que los pacientes tenían una actividad normal en 70 episodios de NPD (44,30%) con una total autonomía en 88 de episodios (55,69%). Se identificaron 39 (24,68%) posibles candidatos para trasplante intestinal. Conclusiones: El número de pacientes registrados es discretamente inferior al del año anterior, aunque el número de hospitales participantes es el mismo. La complicación más frecuente sigue siendo la infección relacionada con el catéter aunque ha disminuido su incidencia respecto a años anteriores, presentándose la tasa más baja desde la creación del registro. Las diferencias en la participación en el registro observadas por Comunidades Autónomas lleva a plantear el desarrollo de estrategias de implementación del registro. Se observa un aumento progresivo de la duración de los días de NPD a lo largo de los años que hace pensar en la cronicidad de algunos pacientes, pero nos obliga a estudiar la existencia de un posible factor de confusión, en el caso de que existiera un olvido de cierre de algún episodio por lo que se hace necesario actualizar el registro con sistemas de alertas periódicas que faciliten la revisión de los pacientes incluidos y optimice la validez del registro (AU)

Objectives: To report the Group Registry NADYA SENPE data about home parenteral nutrition (HPN) in Spain in 2010. Material and methods: A descriptive study of the database of the national registry of HPN of NADYA-SENPE (December 10, 2009 to December 10, 2010). For the calculation of prevalence the latest data published by the Institute National Statistics Office (01/01/2009) was used. Results: There were registered 148 patients from 23 hospitals, 86 women (58.11%) and 9 children (6.08%). The average age of the 139 patients older than 14 years was 53.06 ± 15.41 years. The average duration of HPN was 316.97 days/patient. The most common diagnosis in those younger than 14 years was short bowel traumatic with 5 cases (55.55%) and in those older than 14 years, palliative care cancer with 29 cases (19.59%). The reason for the indication for HPN was short bowel syndrome in 74 cases (47%). The access via most frequently recorded was tunneled catheter in 36 cases (22.78%) followed by implanted port-catheters in 13 cases (8.23%) and other pathways in 3 cases (1.90%). There were 23 catheter related infections (82.14%) which represented 0.49 /1,000 days of PN, all of which occurred in cases older than 14 years. During the year 24 episodes of HPN ended, the most frequent cause was the transition to oral nutrition in 12 episodes (50%). It was reported that patients had a normal activity in 70 episodes of HPN (44.30%) with complete autonomy in 88 episodes (55.69%). Some patients 39 (24.68%) were potential candidates for intestinal transplantation. Conclusions: The number of registered patients is slightly lower than the previous year, although the number of participating hospitals is the same. The most frequent complication remains catheter-related infection but its incidence has decreased from previous years, presenting the lowest rate since the creation of the record. Differences in participation in the registry observed in the Autonomous Communities causes the development of implementation strategies. There is a gradual increase in day length of HPN over the years, which suggests the chronic treatments of some patients and obliges to study the existence of a possible confounding factor, in case there is an oversight of closing an episode. Therefore, it is necessary to update the registry with warning systems that facilitate periodic review of the patients and optimize the validity of registration (AU)

[Home-Based Parenteral Nutrition (HBPN)-associated hepatobiliary complications]. / Complicaciones hepatobiliares asociadas a la Nutrición Parenteral Domiciliaria (NPD).

Home-based parenteral nutrition (HBPN) allows recovering or maintaining the nutritional status of patients with chronic intestinal failure that cannot afford their nutritional requirements through the digestive route. Frequently, liver function impairments develop along the treatment, which in the most severe cases, and especially in premature and low-weight infants, may lead to an irreversible liver failure. The proper composition of the parenteral nutrition bag, avoiding an excess of energy intake, together with the use of new types of lipid emulsions (with lower content in -6 fatty acids and voided of phytosterols) as well as the use, although being minimal, of the enteral route, may contribute to a decrease in the occurrence of HBPN-associated liver disease. It is necessary to perform monthly clinical and biochemical checks to early detect liver function impairments in order to perform the appropriate changes in the treatment and assess the indication of a potential bowel transplant before the liver damage becomes irreversible.

Complicaciones hepatobiliares asociadas a la Nutrición Parenteral Domiciliaria (NPD) / Home-based parenteral nutrition (hbpn)-associated hepatobiliary complications

La Nutrición Parenteral Domiciliaria (NPD) permite recuperar o mantener el estado nutricional de los pacientes con insuficiencia intestinal crónica que no pueden cubrir sus requerimientos nutricionales por vía digestiva. Es frecuente que a lo largo del tratamiento aparezcan alteraciones de la función hepática que, en los casos más graves y sobretodo en niños prematuros y debajo peso, pueden condicionar un fallo hepático irreversible. La correcta composición de la bolsa de nutrición parenteral, evitando un excesivo aporte de energía, junto con el uso de nuevos tipos de emulsiones lipídicas (con menor contenido en ácidos grasos de la serie ω-6 y exentas de fitosteroles) así como la utilización, aunque sea mínima, de la vía digestiva pueden contribuir a disminuirla aparición de la hepatopatía asociada a la NPD. Es imprescindible realizar controles periódicos clínicos y analíticos para detectar precozmente las alteraciones de la función hepática con objeto de realizar los cambios adecuados en el tratamiento y valorar la indicación de un posible trasplante intestinal antes de que el fallo hepático sea irreversible (AU)

Home-based parenteral nutrition (HBPN) allows recovering or maintaining the nutritional status of patients with chronic intestinal failure that cannot afford their nutritional requirements through the digestive route. Frequently, liver function impairments develop along the treatment, which in the most severe cases, and especially in premature and low-weight infants, may lead to an irreversible liver failure. The proper composition of the parenteral nutrition bag, avoiding an excess of energy intake, together with the use of new types of lipid emulsions(with lower content in -6 fatty acids and voided of phytosterols) as well as the use, although being minimal,of the enteral route, may contribute to a decrease in the occurrence of HBPN-associated liver disease. It is necessary to perform monthly clinical and biochemical checks to early detect liver function impairments in order to perform the appropriate changes in the treatment and assess the indication of a potential bowel transplant before the liver damage becomes irreversible (AU)

[Home parenteral nutrition registry in Spain for the year 2010: NADYA-SENPE Group]. / Registro del año 2010 de nutrición parenteral domiciliaria en España: Grupo NADYA-SENPE.

OBJECTIVES: To report the Group Registry NADYASENPE data about home parenteral nutrition (HPN) in Spain in 2010. MATERIAL AND METHODS: A descriptive study of the database of the national registry of HPN of NADYA-SENPE (December 10, 2009 to December 10, 2010). For the calculation of prevalence the latest data published by the Institute National Statistics Office (01/01/2009) was used. RESULTS: There were registered 148 patients from 23 hospitals, 86 women (58.11%) and 9 children (6.08%). The average age of the 139 patients older than 14 years was 53.06 ± 15.41 years. The average duration of HPN was 316.97 days/patient. The most common diagnosis in those younger than 14 years was short bowel traumatic with 5 cases (55.55%) and in those older than 14 years, palliative care cancer with 29 cases (19.59%). The reason for the indication for HPN was short bowel syndrome in 74 cases (47%). The access via most frequently recorded was tunneled catheter in 36 cases (22.78%) followed by implanted port-catheters in 13 cases (8.23%) and other pathways in 3 cases (1.90%). There were 23 catheterrelated infections (82.14%) which represented 0.49 /1,000 days of PN, all of which occurred in cases older than 14 years. During the year 24 episodes of HPN ended, the most frequent cause was the transition to oral nutrition in 12 episodes (50%). It was reported that patients had a normal activity in 70 episodes of HPN (44.30%) with complete autonomy in 88 episodes (55.69%). Some patients 39 (24.68%) were potential candidates for intestinal transplantation. CONCLUSIONS: The number of registered patients is slightly lower than the previous year, although the number of participating hospitals is the same. The most frequent complication remains catheter-related infection but its incidence has decreased from previous years, presenting the lowest rate since the creation of the record. Differences in participation in the registry observed in the Autonomous Communities causes the development of implementation strategies. There is a gradual increase in day length of HPN over the years, which suggests the chronic treatments of some patients and obliges to study the existence of a possible confounding factor, in case there is an oversight of closing an episode. Therefore, it is necessary to update the registry with warning systems that facilitate periodic review of the patients and optimize the validity of registration.

Protocolo para el diagnóstico y tratamiento de la osteopatía metabólica en el enfermo con nutrición parenteral domiciliaria / A protocol for the diagnosis and treatment of metabolic osteopathy in patients with home-based parenteral nutrition

Los pacientes que presentan una insuficiencia intestinal severa sometidos a un programa de nutrición parenteral domiciliaria tienen un riesgo elevado de presentar alteraciones en el metabolismo óseo. Esta disminución de la calidad del hueso tiene un origen multifactorial y puede estar ya presente en el momento de instaurar el soporte nutricional. Es necesario disponer de protocolos que nos permitan realizar un diagnóstico precoz de la enfermedad osteometabólica e instaurar el tratamiento oportuno con objeto de evitar fracturas y mejorar la calidad de vida de estos enfermos

Patients presenting severe intestinal failure submitted to a home-based parenteral nutrition program have increased risk for bone metabolism impairments. This decrease in bone quality has a multifactorial origin and may be already present when implementing nutritional support. There is the need for having available protocols allowing early diagnosis of osteo-metabolic disease and implementing adequate therapy to prevent fractures and improving the quality of life of these patients

Diagnóstico y tratamiento de la hipercolesterolemia familiar / Diagnosis and treatment of familial hypercholesterolemia

La hipercolesterolemia familiar es la enfermedad hereditaria monogénica más frecuente. Se transmite de forma autosómica dominante y se caracteriza por cifras de colesterol unido a lipoproteínas de baja densidad (cLDL) muy elevadas, con posibilidad de presentar depósitos en distintos tejidos. Su presencia entraña un elevado riesgo vascular y un acortamiento de las expectativas de vida si no se trata adecuadamente. Es necesario, por tanto, diseñar estrategias de detección precoz en la población que nos permitan iniciar el tratamiento en edades tempranas de la vida. El riesgo vascular se estima por métodos distintos de los aceptados para la población general debido a las peculiaridades que presentan. El tratamiento con fármacos (fundamentalmente estatinas) es prácticamente obligado para la totalidad de personas afectadas, a causa de los valores de cLDL tan elevados que suelen presentar. El tratamiento farmacológico se ha mostrado seguro y eficaz para prevenir las complicaciones vasculares que amenazan a este colectivo (AU)

Familial hypercholesterolemia is the most frequent monogenetic disease. The mode of transmission is autosomal dominant. This disease is characterized by severe elevations of low-density lipoprotein cholesterol (LDLc), with the possibility of deposits in different tissues. Unless treated appropriately, this disorder carries a high risk of vascular complications and shortened life expectancy. Therefore, strategies for its early detection in the population should be designed to allow early treatment in affected individuals at young ages. Due to the peculiarities of this disease, vascular risk is estimated by methods that differ from those used in the general population. Drug treatment (mainly statins) is almost mandatory in all affected individuals due to their extremely high LDL-c levels. This type of treatment has been shown to be safe and effective in preventing the vascular complications for which this population is at risk (AU)

Soporte nutricional basado en la evidencia / Evidence-based nutritional support

Cuando se plantea el inicio de un soporte nutricional artificial, la elección de la vía de acceso viene determinada por la posibilidad de utilizar, con seguridad y eficacia, el tracto digestivo. La decisión en muchos casos no presenta problemas. Sin embargo, hay situaciones en las que el concepto de "intestino utilizable" no está claro, y en estas circunstancias es cuando se plantea la controversia. Son numerosos los estudios clínicos realizados que comparan las 2 modalidades de nutrición artificial, pero desgraciadamente no existen grandes estudios prospectivos aleatorizados que aporten un nivel de evidencia adecuado. En un intento de obviar esta carencia y extraer conclusiones válidas de los datos aportados en estudios prospectivos aleatorizados previos se han realizado varios metaanálisis en que se compara el uso de la nutrición parenteral y la enteral. Con varias discrepancias, los diversos metaanálisis realizados concluyen que no existen diferencias en la mortalidad cuando se compara la nutrición parenteral y la enteral, mientras que la segunda se asocia con un menor riesgo de presentar complicaciones infecciosas, sobre todo si se inicia de manera temprana; los pacientes malnutridos son los que más se benefician de la intervención nutricional. Los resultados se deben interpretar con cautela debido a la baja calidad metodológica de muchos de los estudios incluidos y a la gran heterogeneidad existente. Se hace necesario disponer de estudios prospectivos aleatorizados con el suficiente número de pacientes con enfermedades concretas que permitan disponer en un futuro de un adecuado nivel de evidencia científica (AU)

When considering initiation of artificial nutritional support the choice of route of access is determined by the possibility of using the digestive tract safely and effectively. In many patients this decision is problem free. However, there are some situations in which the concept of "usable intestine" is not clear, leading to controversy. Numerous clinical trials have compared the two modalities of artificial nutrition, but unfortunately there are no large prospective randomized trials that provide an adequate level of evidence. In an attempt to meet this need and to extract valid conclusions from the data reported in previous prospective randomized trials, several meta-analyses comparing the use of enteral nutrition (EN) and parenteral nutrition (PN) have been performed. Although there are some discrepancies, the various meta-analyses performed tend to conclude that there are no differences in mortality between EN and PN while EN is associated with a lower risk of infectious complications, especially when initiated early, and that the patients who benefit most from nutritional intervention are those with malnutrition. Because of the poor methodological quality of many of the studies included and their wide heterogeneity, the results should be interpreted with caution. For a sufficient level of evidence to become available, prospective randomized trials with a sufficient number of patients should be performed in specific diseases (AU)

Inusual evolución de un bocio multinodular: infiltración tiroidea y de paratiroides por amiloide / Inusual evolution of multinodular goiter: thyroid and parathyroid infiltration by amyloiditis

No disponible

[Polycystic ovary syndrome and valproic acid]. / Síndrome de ovario poliquístico y ácido valproico.

INTRODUCTION: The reproductive functions of epileptic females often display alterations. This dysfunction can be due to psychological, physiological or pharmacological factors. These women have been described as having, for example, a higher incidence of anovulatory cycles, infertility, alterations affecting the hypothalamic or pituitary hormones and the peripheral sex hormones. The greater incidence of polycystic ovary syndrome is subject to debate, since the prevalence varies according to the eligibility criteria used for the syndrome. DEVELOPMENT: The series that show a positive relation between polycystic ovary syndrome and epilepsy tend to understand it to be a side effect of antiepileptic medication, especially valproic acid. Two theories are considered: it has a direct action on the sex hormones or hyperinsulinism that is secondary to weight gain. As neurologists, in our daily practice we must show concern for the conceive. Moreover, we must determine the baseline reproductive hormonal functioning of the epileptic female before beginning antiepileptic therapy, provide laboratory backed evidence of any sexual function disorder and refer them to the reproductive and sexual health of epileptic women. To do so, we need a patient record that is aimed at detecting possible problems, with special attention being given to the warning signs , such as changes in weight, variations of the menstrual cycles or mid cycle bleeding, the appearance of androgynous obesity, signs of virilisation, miscarriages or difficulties to endocrinologist or gynaecologist. CONCLUSIONS: Knowledge about both pathologies will enable us to modify antiepileptic therapy if the relation it has with them is confirmed, since this side effect could be reversible.

Síndrome de ovario poliquístico y ácido valproico / Polycystic ovary syndrome and valproic acid

Introducción. Las alteraciones en la función reproductora son frecuentes en las mujeres epilépticas. La disfunción puede deberse a factores psicológicos, fisiológicos o farmacológicos. En ellas se ha descrito, fundamentalmente, una mayor incidencia de ciclos anovulatorios, infertilidad, alteraciones en las hormonas hipotalámicas o hipofisarias y en las hormonas sexuales periféricas. La mayor incidencia del síndrome de ovario poliquístico es un tema de debate, ya que la prevalencia varía en función de los criterios de inclusión del síndrome. Desarrollo. Las series que muestran una relación positiva entre el síndrome de ovario poliquístico y la epilepsia tienden a considerarlo un efecto adverso de los fármacos antiepilépticos, especialmente del ácido valproico, y se consideran dos teorías: su acción directa sobre las hormonas sexuales o el hiperinsulinismo secundario a la ganancia de peso. Los neurólogos debemos implicarnos rutinariamente en la salud reproductora y sexual de la mujer epiléptica, y realizar una historia clínica orientada a su detección, con especial atención a los `signos de alarma', como son: cambios en el peso, cambios en los ciclos menstruales o pérdidas a mitad del ciclo, aparición de obesidad andrógina, signos de virilización, abortos o dificultad en concebir. Asimismo, debemos conocer la función hormonal reproductora basal de la mujer epiléptica antes de iniciar el tratamiento antiepiléptico, documentar con soporte de laboratorio cualquier alteración de la función sexual, y remitirlas al endocrinólogo o ginecólogo. Conclusión. El conocimiento de ambas patologías permitirá modificar el tratamiento antiepiléptico si se confirma la relación con el mismo, dado que puede tratarse de un efecto adverso reversible (AU)

Introduction. The reproductive functions of epileptic females often display alterations. This dysfunction can be due to psychological, physiological or pharmacological factors. These women have been described as having, for example, a higher incidence of anovulatory cycles, infertility, alterations affecting the hypothalamic or pituitary hormones and the peripheral sex hormones. The greater incidence of polycystic ovary syndrome is subject to debate, since the prevalence varies according to the eligibility criteria used for the syndrome. Development. The series that show a positive relation between polycystic ovary syndrome and epilepsy tend to understand it to be a side effect of antiepileptic medication, especially valproic acid. Two theories are considered: it has a direct action on the sex hormones or hyperinsulinism that is secondary to weight gain. As neurologists, in our daily practice we must show concern for the reproductive and sexual health of epileptic women. To do so, we need a patient record that is aimed at detecting possible problems, with special attention being given to the ‘warning signs’, such as changes in weight, variations of the menstrual cycles or mid-cycle bleeding, the appearance of androgynous obesity, signs of virilisation, miscarriages or difficulties to conceive. Moreover, we must determine the baseline reproductive hormonal functioning of the epileptic female before beginning antiepileptic therapy, provide laboratory-backed evidence of any sexual function disorder and refer them to the endocrinologist or gynaecologist. Conclusions. Knowledge about both pathologies will enable us to modify antiepileptic therapy if the relation it has with them is confirmed, since this side effect could be reversible (AU)

[Remission of polycystic ovary syndrome associated with valproic acid in an epileptic female]. / Remisión de un síndrome de ovario poliquístico asociado a ácido valproico en una mujer epiléptica.

INTRODUCTION: In the epileptic female there is an increase in the number of disorders affecting the reproductive function, which on some occasions can be related to a polycystic ovary syndrome (PCOS). The Finnish school showed an increase in the number of cases of PCOS among females receiving antiepileptic therapy with valproic acid over a long period of time, which can affect up to 43% of the patients being treated. Such a high incidence has not been confirmed in other European series and it is likely that specific ethnic characteristics exert an influence on the results. We examine the possibility that PCOS constitutes a secondary chronic side effect of using valproic acid (VPA) and, as such, is reversible if the antiepileptic therapy is modified following its detection. CASE REPORT: Female, aged 21, who developed PCOS during the course of therapy with VPA, which remitted after changing the treatment to lamotrigine. The clinical signs that have to act as a warning of the possible presence of PCOS in epileptic patients are the appearance of menstrual disorders or signs of virilisation, such as hirsutism and android obesity.

Remisión de un síndrome de ovario poliquístico asociado a ácido valproico en una mujer epiléptica / Remission of polycystic ovary syndrome associated with valproic acid in an epileptic female

Introducción. En la mujer epiléptica existe un incremento de las alteraciones en la función reproductora que, en algunas ocasiones, puede relacionarse con un síndrome de ovario poliquístico (SOPQ). La escuela finlandesa demostró un incremento de SOPQ en las mujeres que recibían tratamiento antiepiléptico con ácido valproico sostenido a largo plazo, que puede afectar al 43 por ciento de las pacientes tratadas. Esta incidencia tan elevada no se ha confirmado en otras series europeas, y probablemente en los resultados influyan características étnicas propias. Nosotros consideramos la posibilidad de que el SOPQ constituya un efecto adverso crónico secundario al empleo de ácido valproico (VPA) y que, como tal, sea reversible si se modifica el tratamiento antiepiléptico cuando se detecta. Caso clínico. Mujer de 21 años que desarrolló SOPQ durante el tratamiento con VPA, y que remitió tras modificar la terapéutica a lamotrigina. Los signos clínicos que deben alertar de la posible presencia de un SOPQ en las pacientes epilépticas son la aparición de baches menstruales o indicios de virilización, como hirsutismo y obesidad androide (AU)

Introduction. In the epileptic female there is an increase in the number of disorders affecting the reproductive function, which on some occasions can be related to a polycystic ovary syndrome (PCOS). The Finnish school showed an increase in the number of cases of PCOS among females receiving antiepileptic therapy with valproic acid over a long period of time, which can affect up to 43% of the patients being treated. Such a high incidence has not been confirmed in other European series and it is likely that specific ethnic characteristics exert an influence on the results. We examine the possibility that PCOS constitutes a secondary chronic side effect of using valproic acid (VPA) and, as such, is reversible if the antiepileptic therapy is modified following its detection. Case report. Female, aged 21, who developed PCOS during the course of therapy with VPA, which remitted after changing the treatment to lamotrigine. The clinical signs that have to act as a warning of the possible presence of PCOS in epileptic patients are the appearance of menstrual disorders or signs of virilisation, such as hirsutism and android obesity (AU)

Síndrome de cushing por hiperplasia adrenal macronodular bilateral ACTH-independiente / Cushing's syndrome due to ACTH-independent bilateral macronodular adrenal hyperplasia

La hiperplasia suprarrenal macronodular bilateral ACTH-independiente (HAMAI) se considera hoy día una entidad diferente y separada del resto de causas de síndrome de Cushing. Su etiología es incierta aunque en los últimos años se ha demostrado la existencia de respuesta anormal de glándulas suprarrenales a distintas hormonas que estimulan la secreción de cortisol. En este artículo presentamos un caso de hipercortisolismo no suprimible con dexametasona, cifras de ACTH indetectables, hiperplasia suprarrenal bilateral de aspecto nodular y resonancia magnética nuclear hipofisaria normal. Se realizó suprarrenalectomía bilateral, objetivando cifras detectables de ACTH a los 3 meses (AU)