Diferencias en la calidad de vida de pacientes con disfagia orofaríngea según el lugar de residencia: impacto en la selección de alimentos y el tipo de dieta / Differences in the quality of life of patients with oropharyngeal dysphagia according to the place of residence: impact on food selection and type of diet

Introducción: la disfagia es un síntoma invalidante, incómodo, con repercusiones en aspectos básicos del bienestar. El test SWAL-QoL esta validado para comprobar el impacto de la disfagia en la calidad de vida (QoL). Métodos: estudio descriptivo transversal (enero 2018). Pacientes con tratamiento de espesante aleatorizados a partir de la base de datos interna de la Unidad de Nutrición y Dietética. Entrevistas telefónicas al paciente o cuidador. Test SWAL-QoL, 11 secciones: carga de enfermedad, síntomas, selección de alimentos, apetito, duración comidas, miedos, comunicación, salud mental, sueño/descanso, fatiga y afectación social. Puntuación ajustada con escala Likert (1-5). Resultados finales transferidos linealmente de 0 a 100 (mejor puntuación positiva). Interpretación: 0-49, impacto severo (IS); 50-70, impacto moderado (IM); y 71-100, impacto discreto o sin impacto (ID/NI). Variables: sexo, edad, días de tratamiento, vivienda en domicilio (D)/residencia(R); indicación de espesante comercial (EC) con MECV-V: néctar (N), miel (M), pudín (P); días con EC, tipo de dieta: puré (DP), blanda (SD), mixta (DM), normal (DN); secciones SWAL-QoL. Resultados: en total, 202 pacientes de 85 años (23-103); IQR: 12. Mayoría de mujeres, 57,9 %; 43,1 % D/56,9 % R. SWAL-QoL: 19,3 % IS, 59,9 % IM y 20,8 % ID/NI. Usar menos espesante demostró mejor QoL, IS en pudín: 21,1 % vs. Néctar: 15,3 %, p = 0,04. Las dietas realizadas fueron: 66,3 % DP; 3 % DB; DM 21,3 %, y DN 9,4 %. En R, más DP (86,1 %) que en D (40,2 %), p < 0,001. DP asociada a peor QoL: % de ID o no impacto en DP, 15,7 % vs. 30,8 % en el resto de dietas, p = 0,016. Analizando cada sección por D/R, diferencia significativa entre miedos, mayor en D (57,01 ± 36,41) vs. R (48,70 ± 27,03), p < 0,001; peor salud mental en R: 66,44 ± 34,30 vs. D: 47,48 ± 24,06, p < 0,001; mejor selección de alimentos en D (75,86 ± 34,12) vs. R (68,17 ± 33,60), p < 0,01 (AU)

Background: dysphagia is a disabling, uncomfortable symptom with repercussions on daily basic aspects of well-being. The SWAL-QoL test is validated for checking dysphagia's impact on quality of life (QoL). Methods: a descriptive cross-sectional study. Randomized patients from our in-house Nutrition & Dietetics database. Telephone interviews directly with patients or caregivers. SWAL-QoL test, 11 sections: swallowing, eating, symptoms, food selection, communication, fears, mental health, fatigue, and social function. A 1-5 Likert scale was adjusted and final results were linearly transferred to 0-100 (100 being best positive score). Results as follows: 0-49 severe impact (SI), 50-70 moderate impact (MI), and 71-100 mild impact or no impact (DI/NI). Variables: gender, age, days with treatment, residence: Home (H)/nursing home (NH), indication of commercial thickener (CT) by V/V test: nectar (N), honey (H), pudding (P), days with CT, type of diets; pureed (PD), soft diet (SD), mixed(P&S/D), regular (RD), SWAL-QoL sections. Results: a total of 202 patients with a mean age of 85 years (23-103); IQR: 12. Mostly women, 57.9 %. Dwelling: 43.1 % H/56.9 % NH. SWAL-QoL: SI affects 19.3 %, 59.9 % MI and 20.8 % DI/NI. Using less thickener proved a better QoL; SI in pudding 21.1 % vs nectar 15.3 %, p = 0.04. Diets included 66.3 % PD; 3 % SD; MD, 21.3 %, and RD, 9.4 %. At NH 86.1 % of PD vs 40.2 % at H, p < 0.001. Direct impact of diet on QoL: 30.8 % on DI or no impact of grouped diets vs 15.7 % with PD, p = 0.016. Analysing each section by H/NH, significant difference between fears, higher at H, 57.01 ± 36.41 vs NH, 48.70 ± 27.03, p < 0.001; worse mental health for NH, 66.44 ± 34.30 vs H, 47.48 ± 24.06, p < 0.001; better food selection for H, 75.86 ± 34.12 vs NH, 68.17 ± 33.60, p < 0.01 (AU)

Determinación de la dosis eritemática mínima y reacciones anómalas a radiación ultravioleta A según fototipo / Determination of Minimal Erythema Dose and Anomalous Reactions to UVA Radiation by Skin Phototype

ANTECEDENTES: La técnica del fototest evalúa la sensibilidad de la piel a la radiación ultravioleta (RUV) mediante la determinación de la mínima dosis de radiación capaz de producir eritema (dosis mínima eritemática [DEM]) y la respuesta anómala a UVA. No existen guías protocolizadas para la técnica del fototest. METODOLOGÍA: Estudio multicéntrico de cohortes prospectivo. Un total de 232 voluntarios sanos fueron reclutados en 9 centros hospitalarios. El fototest se realizó con simuladores solares (SS) o lámparas fluorescentes de UVB de banda ancha (UVBBA). Cada sujeto recibió un total de 5 o 6 dosis progresivas de radiación eritemática y 4 dosis de UVA. La lectura se realizó a las 24 h. RESULTADOS: La DEM media por fototipo fue de 23 ± 8, 28 ± 4, 35 ± 4 y 51 ± 6 mJ/cm2 (fototipos I a IV respectivamente) para los centros que utilizaron SS y de 28 ± 5, 32 ± 3 y 34 ± 5 mJ/cm2 cuando se utilizaron lámparas de UVBBA para fototipos del II al IV. Se consideraron valores de DEM patológica 7, 19, 27 y 38 mJ/cm2, para los fototipos I al IV respectivamente cuando se emplearon SS y de 18, 24 y 24 mJ/cm2 para los fototipos II-IV expuestos a lámparas de UVBBA. A dosis de hasta 20 J/cm2 de UVA no se observaron respuestas anómalas. CONCLUSIONES: Existe homogeneidad de resultados en los diferentes centros participantes, lo que permite estandarizar el método del fotodiagnóstico para los diferentes fototipos cutáneos, así como establecer las dosis umbral que definen una respuesta anómala a la radiación ultravioleta

BACKGROUND: Phototesting is a technique that assesses the skin's sensitivity to UV radiation by determining the smallest dose of radiation capable of inducing erythema (minimal erythema dose [MED]) and anomalous responses to UV-A radiation. No phototesting protocol guidelines have been published to date. METHODOLOGY: This was a multicenter prospective cohort study in which 232 healthy volunteers were recruited at 9 hospitals. Phototests were carried out with solar simulators or fluorescent broadband UV-B lamps. Each individual received a total of 5 or 6 incremental doses of erythemal radiation and 4 doses of UV-A radiation. The results were read at 24 hours. RESULTS: At hospitals where solar simulators were used, the mean (SD) MED values were 23 (8), 28 (4), 35 (4), and 51 (6) mJ/cm2 for skin phototypes I to IV, respectively. At hospitals where broadband UV-B lamps were used, these values were 28 (5), 32 (3), and 34 (5) mJ/cm2 for phototypes II to IV, respectively. MED values lower than 7, 19, 27, and 38 mJ/cm2 obtained with solar simulators were considered to indicate a pathologic response for phototypes I to IV, respectively. MED values lower than 18, 24, and 24 mJ/cm2 obtained with broadband UV-B lamps were considered to indicate a pathologic response for phototypes II to IV, respectively. No anomalous responses were observed at UV-A radiation doses of up to 20 J/cm2. CONCLUSIONS: Results were homogeneous across centers, making it possible to standardize diagnostic phototesting for the various skin phototypes and establish threshold doses that define anomalous responses to UV radiation

Determination of minimal erythema dose and anomalous reactions to UVA radiation by skin phototype.

BACKGROUND: Phototesting is a technique that assesses the skin's sensitivity to UV radiation by determining the smallest dose of radiation capable of inducing erythema (minimal erythema dose [MED]) and anomalous responses to UV-A radiation. No phototesting protocol guidelines have been published to date. METHODOLOGY: This was a multicenter prospective cohort study in which 232 healthy volunteers were recruited at 9 hospitals. Phototests were carried out with solar simulators or fluorescent broadband UV-B lamps. Each individual received a total of 5 or 6 incremental doses of erythemal radiation and 4 doses of UV-A radiation. The results were read at 24hours. RESULTS: At hospitals where solar simulators were used, the mean (SD) MED values were 23 (8), 28 (4), 35 (4), and 51 (6) mJ/cm(2) for skin phototypes i to iv, respectively. At hospitals where broadband UV-B lamps were used, these values were 28 (5), 32 (3), and 34 (5) mJ/cm(2) for phototypes ii to iv, respectively. MED values lower than 7, 19, 27, and 38 mJ/cm(2) obtained with solar simulators were considered to indicate a pathologic response for phototypes I to IV, respectively. MED values lower than 18, 24, and 24mJ/cm(2) obtained with broadband UV-B lamps were considered to indicate a pathologic response for phototypes ii to iv, respectively. No anomalous responses were observed at UV-A radiation doses of up to 20J/cm(2). CONCLUSIONS: Results were homogeneous across centers, making it possible to standardize diagnostic phototesting for the various skin phototypes and establish threshold doses that define anomalous responses to UV radiation.

Benefits of oral Polypodium Leucotomos extract in MM high-risk patients.

BACKGROUND: UV radiation and the presence of melanocytic nevi are the main risk factors of sporadic melanoma (MM). Protection of skin by an oral photoprotective agent would have substantial benefits. OBJECTIVE: We investigated the possible role of an oral Polypodium leucotomos (PL) extract to improve systemic photoprotection in patients at risk of skin cancer analyzing the ability to decrease UV-induced erythema. We also studied the interaction among MC1R polymorphisms and CDKN2A status with the minimal erythematous dose (MED) and their influence in the response after oral PL. METHODS: A total of 61 patients (25 with familial and/or multiple MM, 20 with sporadic MM and 16 with atypical mole syndrome without history of MM) were exposed to varying doses of artificial UVB radiation without and after oral administration of a total dose of 1080 mg of PL. RESULTS: Oral PL treatment significantly increased the MED mean in all group patients (0.123 to 0.161 J/cm(2) , p<0.05). Although not significant, we noticed a stronger effect of PL on the MED of patients with familial MM compared to those with MM (U=273, p=0.06). Among the patients with familial MM, those exhibiting a mutated CDKN2A and/or polymorphisms in MC1R had the bigger differences in response to treatment with PL. LIMITATIONS: Reduced number of patients. No control population. CONCLUSIONS: Administration of PL leads to a significant reduction of sensitivity to UVR (p<0.05) in all patients. Dark-eye patients and patients with higher UVR sensibility (lower basal MED) would be the most benefited from oral PL treatment.

Prevalence of malnutrition and its etiological factors in hospitals.

BACKGROUND: Malnutrition among inpatients is highly prevalent, and has a negative impact on their clinical outcome. The Working Group for the Study of Malnutrition in Hospitals in Catalonia was created to generate consensus guidelines for the prevention and/or treatment of malnutrition in hospitals in Catalonia, Spain. AIMS: The objectives of the study were to determine the prevalence of malnutrition on admission to hospital in Catalonia and to assess relationships between malnutrition, social and demographic data, overall costs, and mortality. METHODS: Prospective and multicenter study conducted with 796 patients from 11 hospitals representative of the hospitalized population in Catalonia. Nutritional status was evaluated using the Nutritional Risk Screening 2002 method. RESULTS: Overall, 28.9% of the patients are malnourished or at nutritional risk. Elderly patients, non-manual workers, those admitted to hospital as emergencies and with higher co-morbidities had higher risk of malnutrition. The type of hospital (second level vs. tertiary or University referral) to which they were admitted was also a factor predisposing to malnutrition. Length of hospital stay was longer in malnourished patients (10.5 vs. 7.7 days, p < 0.0001). The need for a convalescent home on leaving hospital was higher as well as the risk of mortality (8.6% malnourished vs. 1.3% nonmalnourished, p < 0.0001). CONCLUSIONS: The prevalence of malnutrition is high in patients on admission to hospital in our community, resulting in elevated overall costs and higher risk of mortality. Age, social class and characteristics of the Unit and the Hospital are the main factors involved in hospital malnutrition.

[Home enteral nutrition in Spain: NADYA registry in 2010]. / Nutrición enteral domiciliaria en España: registro NADYA del año 2010.

OBJECTIVE: To describe the results of the home enteral nutrition (HEN) registry of the NADYA-SENPE group in 2010. MATERIAL AND METHODS: We retrieved the data of the patients recorded from January 1st to December 31st 2010. RESULTS: We registered 6,591 patients (51% males) with 6,688 episodes of HEN, from 32 hospitals. Mean age in those younger than 14 yr (4%) was 1 ± 2 yrs (m ± SD) and 69,9 ± 17,8 yrs in those older than 14 yr. The length of HEN was longer than 2 yrs in 76% of the patients. The most frequent underlying disease was neurological disorders 42%, followed by cancer 28% (mostly head and neck cancer 18%). We had information related to the enteral access route in only 626 cases (9,4%), 51% of them used nasogastric tubes, 27% gastrostomies, 10% oral route and 3% jejunostomies. Only 251 episodes were closed during the year, mostly due to patient death 57% and progress to oral diet 14%. The activity level was limited in 29% of the patients and 39% of them were bed- or chairridden. Total or partial help was needed by 68% of the patients. The hospitals and the private pharmacies delivered the enteral formula in 63% and 34% of the cases, respectively. The hospitals and the primary care centres delivered the disposables in 83% and 16% of the cases, respectively. CONCLUSIONS: The results of the 2010 HEN registry are similar to those published in previous years regarding the number and characteristics of the patients. We continue finding problems in the entrance of data referred to the enteral access route and the closing of the episodes.

Nutrición enteral domiciliaria en España: registro NADYA del año 2010 / Home enteral nutrition in spain: NADYA registry in 2010

Objetivos: Describir los resultados del registro de nutrición enteral domiciliaria (NED) del grupo NADYASENPE del año 2010.Material y métodos: Se recopilaron los datos introducidos en el registro desde el 1 de enero al 31 de diciembre de2010.Resultados: Se registraron 6.591 pacientes (51% varones) con 6.688 episodios de NED, procedentes de 32 hospitales. La edad media en los menores de 14 años (4%) fue de 1 ± 2 años (m ± DS) y de 69,9 ± 17,8 en los mayores de14 años. El 76% de los pacientes recibieron la NED por un tiempo superior a 2 años. La patología más prevalente fue la neurológica 42%, seguida de la neoplasia 28% (en su mayoría cáncer de cabeza y cuello 18%). La información referente a la vía de acceso sólo se recogió en 626 casos(9,4%), el 51% de los pacientes utilizaron sonda nasogástrica, 27% gastrostomías, 10% vía oral y 3% yeyunostomías. Sólo 251 episodios finalizaron a lo largo del año, siendo el motivo más frecuente el fallecimiento del paciente en el 57% de los casos y el paso a la alimentación oral en el 14%. El 29% de los pacientes presentaban una actividad limitada y el 39% estaba confinado en cama/sillón. El 68% de los adultos requerían ayuda total o parcial. El suministro del producto se realizó desde el hospital o la farmacia de referencia en el 63% y 34%, respectivamente. El suministro del material fungible se realizó desde el hospital o atención primaria en el 83% y 16%,respectivamente.Conclusiones: Los resultados obtenidos en el registro de NED del año 2010 muestran características muy similares a las recogidas en los años previos en cuanto al número y características de los pacientes registrados. Seguimos encontrando problemas en la recogida de datos relativos a la vía de acceso y finalización de los episodios (AU)

Objective: To describe the results of the home enteralnutrition (HEN) registry of the NADYA-SENPE group in2010.Material and methods: We retrieved the data of the patients recorded from January 1st to December 31st2010.Results:We registered 6,591 patients (51% males) with6,688 episodes of HEN, from 32 hospitals. Mean age in those younger than 14 yr (4%) was 1 ± 2 yrs (m ± SD) and69,9 ± 17,8 yrs in those older than 14 yr. The length of HEN was longer than 2 yrs in 76% of the patients. The most frequent underlying disease was neurological disorders 42%, followed by cancer 28% (mostly head and neck cancer 18%). We had information related to the enteral access route in only 626 cases (9,4%), 51% of them used nasogastric tubes, 27% gastrostomies, 10% oral route and 3% jejunostomies. Only 251 episodes were closed during the year, mostly due to patient death 57% and progress to oral diet 14%. The activity level was limited in29% of the patients and 39% of them were bed- or chair ridden. Total or partial help was needed by 68% of the patients. The hospitals and the private pharmacies delivered the enteral formula in 63% and 34% of the cases, respectively. The hospitals and the primary care centres delivered the disposables in 83% and16% of the cases, respectively. Conclusions: The results of the 2010 HEN registry are similar to those published in previous years regarding the number and characteristics of the patients. We continue finding problems in the entrance of data referred to the enteral access route and the closing of the episodes (AU)

Non-invasive management of non-melanoma skin cancer in patients with cancer predisposition genodermatosis: a role for confocal microscopy and photodynamic therapy.

BACKGROUND: Patients with genodermatosis such as Gorlin syndrome (GS) and Xeroderma pigmentosum (XP) require a close follow-up for early diagnosis and treatment of skin cancer. We aimed to evaluate the efficacy of methyl-aminolevulinate (MAL) photodynamic therapy (PDT) in basal cell carcinomas (BCCs) from patients with GS and XP, and to determine the utility of reflectance confocal microscopy (RCM) in the diagnosis and the evaluation of therapeutic response. PATIENTS AND METHODS: We included four patients with GS and two siblings with XP. Single or multiple lesions in localized areas were treated with 1-3 cycles of MAL PDT. RCM was performed before and 3 months after the treatment in target lesions in all the patients. Patients were followed up for 3 years. RESULTS: In XP patients, we treated 13 pigmented BCCs on the face. All the lesions responded to the treatment and six lesions showed a complete clinical clearing. In GS patients, facial or trunk areas with multiple BCCs were treated (up to 200). Complete clinical remission was obtained in 25-67% of the lesions. Some nodular and pigmented lesions failed to achieve a complete remission. RCM could identify already described confocal features for BCC. Tumour remissions could be assessed by this technique. CONCLUSIONS: Methyl-aminolevulinate PDT may be useful for the treatment of superficial BCC in GS and XP. In some nodular lesions, PDT may complement surgery reducing tumour size. RCM may be regarded in the future as a complementary technique in BCC for the diagnosis and post-treatment assessment to non-invasive therapeutic modalities.

[The Spanish Home Enteral Nutrition registry of the year 2009: from the NADYA-SENPE group]. / Registro español de Nutrición Enteral Domiciliaria del año 2009: Grupo NADYA-SENPE.

OBJECTIVE: To describe the Home Enteral Nutrition Characteristics (HEN) recorded by the group NADYA-SENPE during 2009. MATERIAL AND METHOD: collection and analysis of the data voluntary recorded in the HEN registry from the NADYA-SENPE group from January 1st to December 31st. RESULTS: 6.540 HEN patients were registered, 5.11% more than the previous year and 6,649 episodes (3,135 in women, 47,93%) from 32 different hospitals. 6,238 of them (95,38%) were over 14 years. The mean age of the patients under 14 yr was 3,67 ± 2,86 and it was 72,10 ± 16,89 in those over 14 yr group. The base illness registered more frequently was the neurological disorders in 2,732 (41,77%) patients, followed by cancer patients in 1,838; 28,10%. The enteral access route was registered in 1,123 (17,17%) of the episodes, being more frequent the administration by nasogastric tube 562 (50,04%). The mean length of nutritional treatment by episode was 323 days (10,77 months). 606 episodes of HEN ended, being the principal reasons for discontinuing treatment the patient death in 295 (48,68%) occasions. The transition to oral feeding occurred in 219 (36,14%) cases. Patients maintained normal activity in 2162 (32,55%) HEN episodes and 2,468 (37,13%) cases were living "bed-couch". The level of dependence was "total" in 2,598 (39,07%) of the episodes recorded. The nutritional formula was provided by the hospital in 4,183 (62,91%) cases and by the reference pharmacy in 2,262 (el 34,02%). Consumables were provided by the hospital in 3,531 (53,11%) cases. CONCLUSIONS: The number of HEN patients recorded increased from the year 2008, continuing the gradual growth increase since the start of registration. The characteristics of the patients remain in the same profile as in previous years.

[Spanish home enteral nutrition (HEN) registry of the year 2008 from the NADYA-SENPE group]. / Registro del Grupo NADYA-SENPE de Nutrición Enteral Domiciliaria en España en el año 2008.

AIM: To present the results of the Spanish home enteral nutrition (HEN) registry of the year 2008 from the NADYA-SENPE group. MATERIAL AND METHODS: We recorded the HEN registry data from January 1st to December 31st 2008. RESULTS: The number of patients registered in this period was 6206 (51% male) with up to 6,279 episodes of HEN, from 31 Spanish hospitals. Most of the patients (95%) were older than 14 yr. Mean age was 4.83±3.29 yr in the children group, and 70.75±18.14 yr in the adult group (older than 14 yr). Neurological disorders (39%) and cancer (27%) were the two most prevalent diagnoses. The oral route was the most frequently used (43,4%), followed by nasogastric tube (40,4%), and gastrostomy tube (14,7%). Mean length of treatment was 305,36 days (10 months). The principal reasons for discontinuing treatment were death (43%) and progress to oral diet (40%). Only 33% of the patients had a normal activity level, being limited in different grades in the rest of the patients. Most of the patients required partial (25%) or total help (38%). The enteral formula was provided by the hospital in 65% of the cases and by private pharmacies in 32%. The disposables were provided by the hospital (82,4%) and primary care services (17,2%). CONCLUSIONS: The number and the age of the patients registered have increased comparing to previous years, with little variations in the rest of analyzed variables. The increase in the length of treatment could reflect misreporting of the weaning process in the registry.

Registro de Nutrición Enteral Domiciliaria en España en el año 2007 / National registry of home enteral nutrition in Spain 2007

Objetivo: Comunicar los datos del registro nacional de nutrición enteral domiciliaria del grupo NADYA-SENPE de 2007. Material y métodos: Se incluyeron todos los pacientes con nutrición enteral domiciliaria del registro del grupo NADYA entre el 1 de enero y el 31 de diciembre de 2007. Resultados: El número de pacientes con nutrición enteral domiciliaria durante 2007 fue de 5107 (52% varones) de 28 centros hospitalarios. El 95,4% tenían más de 14 años, con una edad media de 67,96 ± 18,12 años mientras en los menores de 15 años fue de 4,2 ± 3,38 años. Las patologías más prevalentes entre ellos fueron las neurológicas (37,8%) y las neoplásicas (29,3%). La vía de administración más empleada fue la oral (63,5%), seguida de sonda nasogástrica (25,9%), mientras la gastrostomía sólo se empleó en el 9,2%. La duración media de la nutrición enteral fue de 9,4 meses y las causas de finalización más frecuentes fueron el fallecimiento (58,77%) y el paso a ingesta oral (26,57%). La actividad estaba limitada en el 31,4% de los pacientes y realizaban vida cama-sillón el 36,01% y la mayoría de los pacientes precisaban ayuda parcial (26,51%) o total (37,68%). El suministro de la fórmula se hacía desde el hospital en el 69,14% de los casos y en las farmacias de referencia en el 30,17%, mientras que el material fungible se suministraba desde el hospital en el 81,63% y desde Atención Primaria en el resto. Conclusiones: Durante 2007, ha habido un incremento de más del 30% de pacientes con nutrición enteral domiciliaria registrados respecto a 2006, sin que esto haya supuesto grandes diferencias en el resto de datos, salvo el mayor aumento de los casos de nutrición enteral-oral (AU)

Objective: To communicate the results from the registry of Home-Based Enteral Nutrition of the NADYASENPE group in 2007. Material and methods: We included every patient in the registry with home enteral nutrition any time from January 1st to December 31st of 2007. Results: The number of patients with home enteral nutrition in 2007 was 5,107 (52% male) from 28 different hospitals. 95.4% of them were 15 yr or older, with a mean age of 67.96 ± 18.12, and 4.2 ± 3.38 among patients aged 14 yr or less. The most common underlying diseases were neurological (37.8%) and neoplastic diseases (29.3%). Enteral nutrition was administered p.o. in most patients (63.5%), followed by nasogastric tube (25.9%), while gastrostomy was only used in 9.2%. The mean time in enteral nutrition support was 9.4 months and the most common reasons for withdrawal were death (58.7%) and switching to oral intake (32%). Activity was limited in 31.4% of patients and 36.01% were house-bound. Most patients needed partial (26.51%) or total (37.68%) care assistance. Enteral formula was provided by hospitals to 69.14% of patients and by pharmacies to 30.17% of them, while disposable material was provided by hospitals to 81.63% and by Primary Care to the remaining patients. Conclusions: In 2007, there has been an increase of more than 30% of patients registered with home enteral nutrition comparing with 2006, without any big difference in other data, but a higher proportion of patients with enteral nutrition p.o (AU)

[Karnosfsky index as a mortality predicting factor in patients on home-based enteral nutrition]. / El índice de Karnofsky como predictor de mortalidad en pacientes con nutrición enteral domiciliaria.

UNLABELLED: Karnofsky Index (KI) is a widely used functional scale developed for oncology patients. It has proved useful as outcome predictor with cancer and geriatric patients. Theoretically, KI could be used to predict mortality in patients with home enteral nutrition (HEN). STUDY OBJECTIVES: To determine baseline KI and its 6-month evolution in HEN patients, and to assess its relation with the mortality rate. METHODOLOGY: Observational and prospective study carried out during 2002 and 2003 with tube feeding neurologic and cancer patients followed during 10 months since their HEN programme inclusion. RESULTS: 201 patients were included, 131 (65.2%) with neurological diseases and 70 (34.8%) with neoplasm. There were not significant differences between groups in age, days with HEN and mortality rate at the end of the study period (35.1% in neurologic patients and 40% in cancer ones). 27.1% of cancer patients had resumed full oral nutrition after ten months from the beginning of the study, whereas only 10.7% of neurologic patients did (p < 0.05). In the three measurement phases (initial, past-3 and past-6 months) KI values were higher for cancer patients than for neurologic ones (p < 0.001). In both groups we didn't found statistically significant differences in KI along the three measurements. A significant relation was observed overall between initial KI values and average survival after 10 months (p < 0.001), and an inverse relation was found between the former and mortality rate (p < 0.001). CONCLUSION: KI is a useful tool to predict mortality rate in cancer and neurologic patients under HEN.

El índice de Karnofsky como predictor de mortalidad en pacientes con nutrición enteral domiciliaria / Karnosfsky index as a mortality predicting factor in patients on home-based enteral nutrition

El índice de Karnofsky (IK) es una escala funcional desarrollada en oncología y ampliamente utilizada, que ha mostrado ser útil para predecir la evolución en pacientes oncológicos y geriátricos. Teóricamente, como herramienta de valoración funcional podría predecir la mortalidad en pacientes con nutrición enteral domiciliaria (NED). Objetivos del estudio: Conocer el IK inicial y su evolución a 6 meses en pacientes en régimen de NED y estudiar la relación entre la tasa de mortalidad de los mismos con dicho índice. Material y métodos: Estudio observacional y prospectivo realizado durante 2002 y 2003 con pacientes neurológicos y oncológicos con nutrición enteral por sonda seguidos durante 10 meses desde su inclusión en el programa de NED. Resultados: Se estudiaron 201 pacientes, 131 (65,2%) neurológicos y 70 (34,8%) oncológicos, sin existir entre ambos grupos diferencias significativas en la edad, días con NE y mortalidad al final del periodo de estudio (35,1% en pacientes neurológicos y 40% en oncológicos). A los 10 meses un mayor porcentaje de pacientes oncológicos había recuperado la capacidad de ingesta oral (27,1% frente a 10,7% en los neurológicos, p < 0,05). Los valores del IK fueron mayores en los pacientes oncológicos que en los neurológicos en las tres determinaciones (inicial, a los 3 y a los 6 meses, p < 0,001). Dicho índice no se modificó significativamente en ninguno de los dos grupos durante su evolución. Analizando el conjunto de los pacientes se observó una relación significativa entre los valores iniciales del IK y la media de supervivencia a los 10 meses (p < 0,001), y una relación inversa entre los valores iniciales del índice y el porcentaje de mortalidad (p < 0,001). Conclusión: El IK es una herramienta útil para predecir la mortalidad en pacientes oncológicos y neurológicos en programa de NED (AU)

Karnofsky Index (KI) is a widely used functional scale developed for oncology patients. It has proved useful as outcome predictor with cancer and geriatric patients. Theoretically, KI could be used to predict mortality in patients with home enteral nutrition (HEN). Study objectives: To determine baseline KI and its 6-month evolution in HEN patients, and to assess its relation with the mortality rate. Methodology: Observational and prospective study carried out during 2002 and 2003 with tube feeding neurologic and cancer patients followed during 10 months since their HEN programme inclusion. Results: 201 patients were included, 131 (65.2%) with neurological diseases and 70 (34.8%) with neoplasm. There were not significant differences between groups in age, days with HEN and mortality rate at the end of the study period (35.1% in neurologic patients and 40% in cancer ones). 27.1% of cancer patients had resumed full oral nutrition after ten months from the beginning of the study, whereas only 10.7% of neurologic patients did (p < 0.05). In the three measurement phases (initial, past-3 and past-6 months) KI values were higher for cancer patients than for neurologic ones (p < 0.001). In both groups we didn't found statistically significant differences in KI along the three measurements. A significant relation was observed overall between initial KI values and average survival after 10 months (p < 0.001), and an inverse relation was found between the former and mortality rate (p < 0.001). Conclusion: KI is a useful tool to predict mortality rate in cancer and neurologic patients under HEN (AU)

[National registry of home enteral nutrition in Spain 2007]. / Registro de Nutrición Enteral Domiciliaria en España en el año 2007.

OBJECTIVE: To communicate the results from the registry of Home-Based Enteral Nutrition of the NADYASENPE group in 2007. MATERIAL AND METHODS: We included every patient in the registry with home enteral nutrition any time from January 1st to December 31st of 2007. RESULTS: The number of patients with home enteral nutrition in 2007 was 5,107 (52% male) from 28 different hospitals. 95.4% of them were 15 yr or older, with a mean age of 67.96 +/- 18.12, and 4.2 +/- 3.38 among patients aged 14 yr or less. The most common underlying diseases were neurological (37.8%) and neoplastic diseases (29.3%). Enteral nutrition was administered p.o. in most patients (63.5%), followed by nasogastric tube (25.9%), while gastrostomy was only used in 9.2%. The mean time in enteral nutrition support was 9.4 months and the most common reasons for withdrawal were death (58.7%) and switching to oral intake (32%). Activity was limited in 31.4% of patients and 36.01% were house-bound. Most patients needed partial (26.51%) or total (37.68%) care assistance. Enteral formula was provided by hospitals to 69.14% of patients and by pharmacies to 30.17% of them, while disposable material was provided by hospitals to 81.63% and by Primary Care to the remaining patients. CONCLUSIONS: In 2007, there has been an increase of more than 30% of patients registered with home enteral nutrition comparing with 2006, without any big difference in other data, but a higher proportion of patients with enteral nutrition p.o.

Childhood-onset mild cutaneous porphyria with compound heterozygotic mutations in the uroporphyrinogen decarboxylase gene.

Three children (two boys and one girl) from the same family presented with photosensitivity, hyperpigmentation, hypertrichosis, mild skin fragility, blistering and scarring in childhood. On examination, the cutaneous lesions were found to have improved since their previous examinations. Laboratory tests showed raised plasma and urine carboxyporphyrins and decreased uroporphyrinogen decarboxylase enzyme activity in red blood cells. Triggering factors for porphyria were not detected except for a hepatitis C virus infection in the younger boy. The girl's clinical symptoms recurred in late adolescence, after iron and oestrogen treatments. Mutation analysis of the UROD gene detected two missense mutations, 19 A-->G M1V (novel) and 703C-->T P235S (previously reported), in an uncommon compound heterozygous manner in the three siblings.

[Registry of home-based enteral nutrition in Spain for the year 2006 (NADYA-SENPE Group)]. / Registro de la Nutrición Enteral Domiciliaria en España en el año 2006 (Grupo NADYA-SENPE).

OBJECTIVE: To communicate the results obtained from the registry of Home-Based Enteral Nutrition (HBEN) of the NADYA-SENPE group for the year 2006. MATERIAL AND METHODS: Recompilation of the data from the HBEN registry of the NADYA-SENPE group from January 1st to December 31st of 2006. RESULTS: During the year 2006, 3,921 patients (51% men) from 27 hospital centers were registered. Ninety-seven percent were older than 14 years. The mean age for those < 14 years was 4.9 +/- 3.9 (m +/- SD) and in those > or = 14 years, it was 68.5 +/- 18.2 years. The most common underlying disease was neurological pathology (42%), followed by cancer (28%). Enteral nutrition was administered p.o. in 44% of the patients, through nasogastric tube in 40%, gastrostomy in 14%, and jejunostomy in 1%. The average time of nutritional support was 8.8 months. The most common reasons for ending the therapy were patient's death (54%) and switching to oral feeding (32%). Thirty-one percent of the patients presented a limited activity and 40% were confined to bed/coach. Most of the patients required partial (25%) or total (43%) care assistance. The nutritional formula was provided by the hospital in 62% of the cases and from the reference pharmacy in 27%. The fungible material was provided by the hospital in 80% of the cases and by primary care in the remaining patients. CONCLUSIONS: Although the number of registered patients is slightly higher than that from the last years, there are no important changes in the patients characteristics, or way of administration and duration of enteral nutrition.

A multicentre, randomized, controlled study of the efficacy, safety and cost-effectiveness of a combination therapy with amorolfine nail lacquer and oral terbinafine compared with oral terbinafine alone for the treatment of onychomycosis with matrix involvement.

BACKGROUND: Onychomycosis is common, accounting for up to 50% of all nail disorders. Toenail onychomycosis can cause nail deformity, embarrassment, pain and walking difficulties. Some populations, such as individuals with diabetes, are at higher risk for developing secondary complications such as infections. Treatment takes many months and therapeutic choices can increase clinical effectiveness, lower toxicity and minimize healthcare costs. OBJECTIVES: Based on the results of a previous pilot study, the objective of the present study was to show, in a larger population, the enhanced efficacy of a combination of amorolfine nail lacquer and oral terbinafine in the treatment of onychomycosis with matrix involvement. In addition, a cost-effectiveness analysis was performed. METHODS: In this multicentre, randomized, open-label, parallel group study, patients were randomized to receive either a combination of amorolfine hydrochloride 5% nail lacquer once weekly for 12 months plus terbinafine 250 mg once daily for 3 months (AT group) or terbinafine alone once daily for 3 months (T group). The study duration was 18 months including a 6-month treatment-free phase following the 12-month active treatment phase for the AT group and a 15-month treatment-free phase following the 3-month active treatment phase for the T group. The primary efficacy criterion was overall response, dichotomized into success or failure, success being the combination of clinical cure and negative mycology at month 18. This criterion was used as the effectiveness measure in the pharmacoeconomic analysis, conducted from a payer perspective. RESULTS: In total, 249 patients were included into the study: 120 in the AT group and 129 in the T group. A significantly higher success rate was observed for patients in the AT group relative to those in the T group at 18 months (59.2% vs. 45.0%; P = 0.03). Both treatment regimens were safe and well tolerated. Treatment cost per cured patient was lower for the combination than for terbinafine alone in all countries. CONCLUSIONS: Study results confirmed that, in the treatment of dermatophytic toenail onychomycosis with matrix involvement, amorolfine nail lacquer in combination with oral terbinafine enhances clinical efficacy and is more cost-effective than terbinafine alone.

Genetic studies in variegate porphyria in Spain. Identification of gene mutations and family study for carrier detection.

BACKGROUND: First, to establish the mutations of the protoporphyrinogen-oxidase (PPOX) gene in four Spanish patients with variegate porphyria (VP). Second, study of carrier status detection in the families, including a four-generation Balearic family. Third, evaluation of the results of carrier detection screening methods. DESIGN: Blood samples of four patients and of 139 members belonging to four families, including four generations of a Balearic family were processed for mutation analysis of the 13 exons of PPOX gene. Biochemical studies were performed together (blood and faecal porphyrin analysis) and plasma fluorescence scanning for 626 nm peak emission detection. A questionnaire regarding clinical manifestations was submitted to all family members studied. RESULTS: Single strand conformational analysis (SSCP) of DNA allowed the detection of the following mutations: W224R, 746delT: exon 7, 1077-1082insC: exon 10, and IVS6+2T-->A. Mutation was present in 19 of the 139 members of the families studied. Clinical manifestations or biochemical alterations were checked in the carriers detected and found as not relevant or not present. Only 11 members of the 19 mutation-bearing individuals showed plasma fluorescence PV peak positivity. CONCLUSION: Demonstration of gene mutation is the most reliable means of detecting carriers in studies of variegate porphyria families. DNA analysis is the most sensitive carrier detection method and also allows transmission behaviour of the genetic defect to be established in successive generations of the affected families.

Genetic and biochemical characterization of 16 acute intermittent porphyria cases with a high prevalence of the R173W mutation.

Acute intermittent porphyria (AIP) is a metabolic disease with a variable prevalence among different countries. In some areas of southern Europe it remains to be fully evaluated. We undertook a genetic and biochemical study of 16 unrelated Spanish AIP patients and relatives. The genetic analyses showed they harboured the following mutations in the porphobilinogen deaminase gene: R173W, G111R, L278P, L238P, R116W, R26C, 340insT, 730delCT, 691del30bp, and IVS14+1g>a. The mutation R173W was found in 6 patients (37.5%), including the only patients of our series with >3 recurrent porphyria attacks. While in clinical remission, all AIP patients exhibited sustained increased excretion of porphyrins and precursors. PBG excretion showed a high between-subject variation and was not related to erythrocyte PBG deaminase activity. The study of family members allowed the identification of 22 asymptomatic AIP carriers. These included 8 persons harbouring the R173W mutation belonging to four different families. Six of these latent AIP subjects showed increased PBG elimination, and in two the urinary levels were >10-fold the normal limit. These results reinforce the hypothesis that the R173W mutation may have a high biochemical and clinical penetrance among AIP patients.

[National registry of Home Enteral Nutrition in 2003]. / Registro nacional de la Nutrición Enteral Domiciliaria del año 2003.

GOAL: To communicate the information available by the NADYA-SENPE Working Group from patients on Home Enteral Nutrition (HEN) in our country during the year 2003. MATERIAL AND METHODS: The data were collected through a closed questionnaire included in the web site of the Working Group (www.nadya-senpe.com) available only by the authorized users. Variable included were: epidemiological information, the indication to prescribe this treatment, the access path, the specific nutritional formula used, the treatment duration, the complications and hospital readmission related to the nutritional treatment, the follow-up and the quality of life. RESULTS: We register 3,858 patients that belong to twenty-one hospitals. Mean age from those adults 66.2 +/- 18.9 years, and from those younger than 14, 6.0 +/- 4.3 years. Neurological and neoplasic diseases were the diagnostics more frequents (38.9% and 37.4%, respectively). Oral nutrition was the preferential rout used for the enteral nutrition (54.7%) followed by naso-enteral tube (26.6%), and only in 17.6% we used ostomy tubes. Polymeric was the enteral formula mainly utilized (80.1%). The mean time on HEN was 6.6 +/- 4.3 months; the 28.8% of patients stayed in the treatment for less than 3 months, 21.2 % between 3 and 6 months, and 50.0% more than 6 months. Patients were followed mainly by Nutritional Support Unit from the reference hospital (73.1%). While the reference hospital supplies the material (62.4%), reference hospital pharmacy (46.8%) and public pharmacies (32.0%) provides the enteral formula. Complications related to enteral nutrition included change of enteral tube (44.5%), gastrointestinal complications (30.5%), mechanical complications (21.7%), and the metabolic one (3.3%). These complications were followed by 0.02 hospitalizations/patient. At the end of the year, 54.7% of patients were in the HEN programme, and in 35.2 % HEN was finish due to accept oral conventional alimentation (49.2%) or by deceased of patients (40.9%). While 26.6% of the patients were confined to bed or armchair, 19.7 % no or light discapacity degree was observed. CONCLUSIONS: We found a persistence of these treatment in our country. Neurological and neoplasic diseases were the more frequent diagnosis in patients analysed. The high prevalence of cancer patients could be the main cause of oral access for enteral nutrition. Change of enteral tube was the more frequent complication observed during this treatment.