[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.

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.

[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.

[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.

[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.

[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.