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1.
J Clin Med ; 12(23)2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38068509

RESUMO

Phenylketonuria (PKU) is the most frequent of the congenital errors of amino acid (AA) metabolism worldwide. It leads to the accumulation of the essential AA phenylalanine (Phe) and it is associated with severe neurological defects. The early diagnosis and treatment of this rare disease, achieved through newborn screening and low-Phe diet, has profoundly changed its clinical spectrum, resulting in normal cognitive development. We face the first generation of PKU patients perinatally diagnosed and treated who have reached adulthood, whose special needs must be addressed, including feeding through enteral nutrition (EN). However, recommendations regarding EN in PKU constitute a gap in the literature. Although protein substitutes for patients with PKU are offered in multiple forms (Phe-free L-amino acid or casein glycomacropeptide supplements), none of these commercial formulas ensures the whole provision of daily total energy and protein requirements, including a safe amount of Phe. Consequently, the combination of different products becomes necessary when artificial nutrition via tube feeding is required. Importantly, the composition of these specific formulas may result in physicochemical interactions when they are mixed with standard EN products, leading to enteral feeding tubes clogging, and also gastrointestinal concerns due to hyperosmolality. Herein, we present the first reported case of EN use in an adult patient with PKU, where the separate administration of protein substitutes and the other EN products avoided physicochemical interactions.

2.
Eur J Clin Nutr ; 77(12): 1173-1175, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37666959

RESUMO

Bariatric surgery (BS) is currently the most effective treatment for severe obesity, requiring ongoing multidisciplinary follow-up to ensure proper progress and nutrition post-procedure. Despite its favourable safety profile, it is not exempt from complications, one of which being exocrine pancreatic insufficiency (EPI). The underlying pathophysiological mechanisms of EPI after BS are multifactorial, including poorly synchronized pancreatic enzyme secretion with the passage of nutrients (pancreaticocibal or postcibal asynchrony), insufficient pancreatic stimulation and bacterial overgrowth. We conducted a short literature review of the topic through a case of a patient who underwent BS in our centre and subsequently developed EPI and severe malnutrition. EPI initially was attributed to the surgery, but after a comprehensive evaluation, an unexpected cause was revealed.


Assuntos
Cirurgia Bariátrica , Insuficiência Pancreática Exócrina , Humanos , Insuficiência Pancreática Exócrina/etiologia , Pâncreas , Cirurgia Bariátrica/efeitos adversos , Estado Nutricional , Causalidade
3.
Acta Psychiatr Scand ; 148(4): 316-326, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37539719

RESUMO

INTRODUCTION: Psychiatric patients are considered at risk for malnutrition due to pharmacological treatments, lifestyle habits and the mental illness by itself. Even though metabolic risk factors have been related to worse outcomes in certain conditions, the evidence regarding the nutritional status and its impact on the length of stay in psychiatric inpatients is scarce. This study aims to characterize the nutritional status in acute psychiatric patients, to correlate it with the length of stay, and to find specific potential indicators of malnutrition. METHODS: Adult patients admitted to the Hospital Clínic of Barcelona acute psychiatric ward throughout a 1-year period were included in this cross-sectional study. Sociodemographic and clinical variables were registered, including length of stay and the nutritional status measured with the CONUT score. RESULTS: Malnutrition was observed in 42.5% of patients. Plasmatic transferrin saturation, protein and iron levels were inversely correlated with length of stay, having low iron levels an association with longer hospitalizations. The length of stay was not influenced by diagnosis or treatment. Negative correlations with the nutritional status were found in: BMI, cholesterol, triglycerides, albumin, total proteins, prealbumin, iron, lymphocytes and zinc levels, and transferrin saturation. The multivariate analysis showed a significant association for cholesterol and zinc levels, lymphocyte count, and BMI. CONCLUSIONS: Our results suggest that nutritional status might influence the course of psychiatric admissions. Cholesterol and zinc levels, lymphocyte count, and BMI might be factors strongly associated with malnutrition. This consideration might allow the identification of profiles in which lifestyle interventions could be implemented.


Assuntos
Desnutrição , Unidade Hospitalar de Psiquiatria , Adulto , Humanos , Avaliação Nutricional , Estudos Transversais , Desnutrição/epidemiologia , Desnutrição/diagnóstico , Desnutrição/etiologia , Colesterol , Ferro/metabolismo , Transferrinas , Zinco/metabolismo
4.
Nutr. hosp ; 36(1): 233-237, ene.-feb. 2019. graf
Artigo em Espanhol | IBECS | ID: ibc-183207

RESUMO

Objetivo: exponer los resultados del registro de nutrición enteral domiciliaria (NED) de los años 2016 y 2017 del Grupo NADYA-SENPE. Material y métodos: se recopilaron los pacientes introducidos en el registro del 1 de enero al 31 de diciembre de 2016 y la mismas fechas de 2017 para proceder al análisis descriptivo y analítico de los datos. Resultados: en el año 2016 se obtuvieron 4.578 pacientes activos (prevalencia = 98,33 pacientes/millón de habitantes) y en 2017 fueron 4.777 (prevalencia = 102,57). Por sexos, hubo un 50,8% de varones en 2016 y un 50,5% en 2017. En el periodo 2016-17, la edad mediana fue de 71,5 años (IIQ 57-83); asimismo, finalizaron 1.558 episodios de NED y la causa principal fue el fallecimiento (793 pacientes, 50,89%). Los varones adultos fueron más jóvenes que las mujeres (65,3 vs. 73,3 años, p-valor < 0,001) y el diagnóstico más frecuente fue la enfermedad neurológica que cursa con afagia o disfagia severa (59%). La sonda nasogástrica (SNG) fue la vía de acceso más utilizada (48,3%) y se observa, además, que esta es la vía que se utiliza en los pacientes más ancianos (p < 0,001). Se registraron 126 pacientes pediátricos (57,1% niñas). La edad mediana de inicio de la NED fue de cuatro meses. Otras patologías fue el grupo diagnóstico más registrado (41,3%), seguido por la enfermedad neurológica que cursa con afagia o disfagia severa. Se alimentaban a través de gastrostomía en el 57,6% de los casos. Se observó que los niños más pequeños eran los que se alimentaban preferentemente por SNG (p-valor 0,001). Conclusiones: el número de pacientes del registro, así como el número de centros participantes, se va incrementando progresivamente. Las principales características de los pacientes no han variado. A pesar del aumento de posibilidades diagnósticas en la población pediátrica, llama la atención la clasificación dentro del grupo de Otras patologías


Objective: to present the results of the Spanish home enteral nutrition (HEN) registry of the NADYA-SENPE group for the year 2016 and 2017. Material and methods: from January 1st 2016 to December 31st 2017, the HEN registry was recorded and afterwards a further descriptive and analytical analysis was done. Results: in 2016, 4,578 active patients were recorded and prevalence was 98.33 patients per one million inhabitants; in 2017, 4,777 patients were recorded, with a prevalence of 102.57 per one million inhabitants; 50.8% were males in 2016 and 50.5% in 2017. During the period 2016-17, median age was 71.5 years (IIQ 57-83), 1,558 HEN episodes were finished and the main cause was death (793 patients, 50.89%). Adult males were younger than females (65.3 vs. 73.3 years, p-value < 0.001). The most frequent diagnosis was the neurological disorder that presents with aphagia or severe dysphagia (59%). Nasogastric tube was the most frequent administration route (48.3%) and it is the most widely used in elderly patients (p < 0.001). One hundred and twenty-six pediatric patients were registered (57.1% females). Median age at the beginning of HEN in children was four months. "Other disorders" was the most recorded diagnostic group (41.3%), followed by the group of neurological disorder that presents with aphagia or severe dysphagia. Regarding children, 57.6% were fed through gastrostomy and the younger ones were fed through nasogastric tube (p-value 0.001). Conclusions: the number of patients in the registry, as well as the number of participating centers, is progressively increasing. The main characteristics of the patients have not changed. Despite the increase in diagnostic possibilities in the pediatric population, the classification within the group of "Other pathologies" is quite significant


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Nutrição Enteral/estatística & dados numéricos , Nutrição Parenteral no Domicílio/estatística & dados numéricos , Registros , Nutrição Enteral/tendências , Nutrição Parenteral/tendências , Nutrição Parenteral no Domicílio/tendências , Fatores Sexuais , Espanha/epidemiologia
5.
AIDS ; 32(10): 1309-1316, 2018 06 19.
Artigo em Inglês | MEDLINE | ID: mdl-29683854

RESUMO

OBJECTIVE: The aim of this study was to compare the differential effects of first-line efavirenz (EFV)-based vs. boosted lopinavir-based antiretroviral regimens on cholesterol metabolism. DESIGN: Multicentre, open-label, randomized clinical trial. METHODS: Forty-nine naive HIV-infected patients were randomized (1 : 1) to receive either ritonavir-boosted lopinavir (LPV/r) or EFV both in combination with tenofovir and emtricitabine (ClinicalTrials.gov: NCT00759070). Lipid profile and serum phytosterols and cholesterol precursors were determined at baseline and after 16 weeks. RESULTS: After 16 weeks of intervention, total and non-HDL cholesterol as well as triglyceride levels significantly increased in the LPV/r-group (+1.0 ±â€Š0.8; +0.8 ±â€Š0.7 and +0.8 ±â€Š1.5 mmol/l, respectively), but not in the EFV-group (+0.4 ±â€Š0.7; +0.4 ±â€Š0.6 and 0.2 ±â€Š0.5 mmol/l, respectively). Markers of cholesterol absorption (campesterol-to-cholesterol and sitosterol-to-cholesterol ratios) significantly increased in the LPV/r-group, but not in the EFV-group, whereas there were no changes in either group of the lathosterol-to-cholesterol ratio, a marker of cholesterol synthesis. CONCLUSION: Treatment with an LPV/r-based therapy increased total cholesterol relative to EFV-based therapy. Our data suggest that absorption rather than synthesis is the mechanism involved in LPV/r-associated increased total cholesterol.


Assuntos
Colesterol/metabolismo , Infecções por HIV/tratamento farmacológico , Inibidores da Protease de HIV/efeitos adversos , Hipercolesterolemia/induzido quimicamente , Hipercolesterolemia/fisiopatologia , Ritonavir/efeitos adversos , Adolescente , Adulto , Idoso , Alcinos , Terapia Antirretroviral de Alta Atividade/métodos , Benzoxazinas/administração & dosagem , Benzoxazinas/efeitos adversos , Análise Química do Sangue , Ciclopropanos , Feminino , Infecções por HIV/complicações , Inibidores da Protease de HIV/administração & dosagem , Humanos , Masculino , Pessoa de Meia-Idade , Inibidores da Transcriptase Reversa/administração & dosagem , Inibidores da Transcriptase Reversa/efeitos adversos , Ritonavir/administração & dosagem , Adulto Jovem
6.
Nutr Hosp ; 34(1): 15-18, 2017 02 01.
Artigo em Espanhol | MEDLINE | ID: mdl-28244767

RESUMO

Objective: To present the results of the Spanish home enteral nutrition (HEN) registry of the NADYA-SENPE group for the years 2014 and 2015. Methods: From January 1st 2014 to December 31st 2015 the HEN registry was recorded and afterwards a further descriptive and analytical analysis was done. Results: In 2014, 3749 patients were recorded, and 4202 in 2015; prevalence was 80.58 patients/one million inhabitants in Spain in 2014 and 90.51 in 2015. There were 49.9% females in 2014 and 50.3% in 2015. Median age was 73 years (IQI 59-83) in 2014 as well as in 2015. 684 episodes finished in 2014 and 631 in 2015, with death as the main cause, in 54.9% and 50.4%, respectively. The ones who were fed through nasogastric tube had a mean age higher than the ones fed by any other route (p-value < 0.001). Sisty-seven paediatric patients were recorded in 2014 (56.7% females) and 77 in 2015 (55.8% females). Median age at the beginning of HEN among children was 5 months in 2014 and 5 months in 2015. The main route of administration was gastrostomy, in 52.5% in 2014 and nasogastric tube in 50.8% in 2015. 7 episodes finished in 2014 and 13 in 2015, having death as the main cause (57.1% in 2014 and 38.5% in 2015). It was found that were younger children the ones who were mainly fed by nasogastric tubes (p-value 0.004 vs. 0.002). Among paediatric patients as well as adults, the main diagnosis leading to HEN was neurological disease which gives aphagia or severe dysphagia. Conclusions: There has been an increase in the number of patients in the registry as well as the participating centers and the number of patients per center, without any significant change in the characteristics of the patients other than longer duration of the episodes.


Objetivo: exponer los resultados del registro de nutrición enteral domiciliaria (NED) del año 2014 y 2015 del Grupo NADYA-SENPE. Métodos: se recopilaron los pacientes introducidos en el registro desde el 1 de enero al 31 de diciembre de 2014 y la mismas fechas de 2015, y se procedió al análisis descriptivo y analítico de los datos. Resultados: en el año 2014, se registraron 3.749 pacientes y en 2015, 4.202; la prevalencia fue de 80,58 pacientes/millón de habitantes en el año 2014 y de 90,51 en 2015. Por sexos, hubo un 49,9% de mujeres en 2014 y un 50,3% en 2015. La edad media fue de 73 años (IIQ 59-83) en ambos años. Finalizaron 684 episodios de NED en 2014 y 631 en 2015, la causa principal fue el fallecimiento en el 54,9% y 50,4% de los casos, respectivamente. Los portadores de sonda nasogástrica presentan una edad media superior a los pacientes con cualquier otra vía (p < 0,001). Se registraron 67 pacientes pediátricos en 2014 (56,7% niñas) y 77 en 2015 (55,8% niñas). La vía principal de administración fue la gastrostomía en el 52,0% de los casos de 2014 y sonda nasogástrica en el 50,8% de los casos de 2015. La causa principal de finalización de la nutrición fue el fallecimiento (57,1% en 2014 y 38,5% en 2015). Se observó que los niños más pequeños eran los que se alimentaban preferentemente por SNG (p 0,004 vs.0,002).Tanto en pacientes pediátricos como en adultos el diagnóstico principal que motivó la necesidad de NED fue la enfermedad neurológica que cursa con afagia o disfagia severa. Conclusiones: se ha incrementado el número de pacientes del registro, así como el número de centros participantes y el número medio de pacientes comunicados por cada centro respecto a años anteriores, sin que se hayan modificado sustancialmente las características de los pacientes, salvo mayor duración de los episodios.


Assuntos
Nutrição Parenteral no Domicílio/estatística & dados numéricos , Sistema de Registros , Adolescente , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Intubação Gastrointestinal/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Espanha , Adulto Jovem
7.
Nutr. hosp ; 34(1): 15-18, ene.-feb. 2017.
Artigo em Espanhol | IBECS | ID: ibc-161136

RESUMO

Objetivo: exponer los resultados del registro de nutrición enteral domiciliaria (NED) del año 2014 y 2015 del Grupo NADYA-SENPE. Métodos: se recopilaron los pacientes introducidos en el registro desde el 1 de enero al 31 de diciembre de 2014 y la mismas fechas de 2015, y se procedió al análisis descriptivo y analítico de los datos. Resultados: en el año 2014, se registraron 3.749 pacientes y en 2015, 4.202; la prevalencia fue de 80,58 pacientes/millón de habitantes en el año 2014 y de 90,51 en 2015. Por sexos, hubo un 49,9% de mujeres en 2014 y un 50,3% en 2015. La edad media fue de 73 años (IIQ 59-83) en ambos años. Finalizaron 684 episodios de NED en 2014 y 631 en 2015, la causa principal fue el fallecimiento en el 54,9% y 50,4% de los casos, respectivamente. Los portadores de sonda nasogástrica presentan una edad media superior a los pacientes con cualquier otra vía (p < 0,001). Se registraron 67 pacientes pediátricos en 2014 (56,7% niñas) y 77 en 2015 (55,8% niñas). La vía principal de administración fue la gastrostomía en el 52,0% de los casos de 2014 y sonda nasogástrica en el 50,8% de los casos de 2015. La causa principal de finalización de la nutrición fue el fallecimiento (57,1% en 2014 y 38,5% en 2015). Se observó que los niños más pequeños eran los que se alimentaban preferentemente por SNG (p 0,004 vs. 0,002).Tanto en pacientes pediátricos como en adultos el diagnóstico principal que motivó la necesidad de NED fue la enfermedad neurológica que cursa con afagia o disfagia severa. Conclusiones: se ha incrementado el número de pacientes del registro, así como el número de centros participantes y el número medio de pacientes comunicados por cada centro respecto a años anteriores, sin que se hayan modificado sustancialmente las características de los pacientes, salvo mayor duración de los episodios (AU)


Objective: To present the results of the Spanish home enteral nutrition (HEN) registry of the NADYA-SENPE group for the years 2014 and 2015. Methods: From January 1st 2014 to December 31st 2015 the HEN registry was recorded and afterwards a further descriptive and analytical analysis was done. Results: In 2014, 3749 patients were recorded, and 4202 in 2015; prevalence was 80.58 patients/one million inhabitants in Spain in 2014 and 90.51 in 2015. There were 49.9% females in 2014 and 50.3% in 2015. Median age was 73 years (IQI 59-83) in 2014 as well as in 2015. 684 episodes finished in 2014 and 631 in 2015, with death as the main cause, in 54.9% and 50.4%, respectively. The ones who were fed through nasogastric tube had a mean age higher than the ones fed by any other route (p-value < 0.001). Sixty-seven paediatric patients were recorded in 2014 (56.7% females) and 77 in 2015 (55.8% females). Median age at the beginning of HEN among children was 5 months in 2014 and 5 months in 2015. The main route of administration was gastrostomy, in 52.5% in 2014 and nasogastric tube in 50.8% in 2015. 7 episodes finished in 2014 and 13 in 2015, having death as the main cause (57.1% in 2014 and 38.5% in 2015). It was found that were younger children the ones who were mainly fed by nasogastric tubes (p-value 0.004 vs. 0.002). Among paediatric patients as well as adults, the main diagnosis leading to HEN was neurological disease which gives aphagia or severe dysphagia. Conclusions: There has been an increase in the number of patients in the registry as well as the participating centers and the number of patients per center, without any significant change in the characteristics of the patients other than longer duration of the episodes (AU)


Assuntos
Humanos , Nutrição Enteral/estatística & dados numéricos , Intubação Gastrointestinal/estatística & dados numéricos , Gastrostomia/estatística & dados numéricos , Transtornos de Deglutição/terapia , Registros de Doenças/estatística & dados numéricos , Serviços Hospitalares de Assistência Domiciliar/estatística & dados numéricos , Distribuição por Idade e Sexo
8.
Nefrología (Madr.) ; 36(6): 616-630, nov.-dic. 2016. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-158751

RESUMO

Introducción: El aumento de la supervivencia de los pacientes con cistinosis y la propia complejidad de la enfermedad explican la necesidad de implementar un proceso de transición guiada desde la medicina pediátrica hasta la del adulto, que permita garantizar el continuumasistencial y posibilite el empoderamiento del paciente desde el cuidado tutelado al autocuidado. Métodos: Revisión bibliográfica, opinión de expertos, encuestas anónimas a pacientes, familiares y asociaciones. Resultados: Elaboración de un documento de transición coordinada, con propuestas concretas por especialidades y de mejora de la adherencia terapéutica y del autocuidado del paciente. El nefrólogo desempeña un papel clave en la transición en la cistinosis debido a la afectación renal que domina la patología y porque la mayoría de los pacientes han recibido un trasplante renal antes de la edad adulta. Conclusión: Se presenta un documento que establece unas recomendaciones y un cronograma para guiar la transición de los adolescentes y adultos jóvenes con cistinosis en nuestro ámbito (AU)


Introduction: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care. Methods: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. Results: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood. Conclusion: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area (AU)


Assuntos
Humanos , Adolescente , Adulto Jovem , Cistinose/terapia , Planejamento de Assistência ao Paciente/organização & administração , Cuidado Transicional/organização & administração , Padrões de Prática Médica , Insuficiência Renal Crônica/prevenção & controle , Doenças Raras/epidemiologia , Perfil de Impacto da Doença
9.
Nefrologia ; 36(6): 616-630, 2016.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-27595514

RESUMO

INTRODUCTION: Improved outcome and longer life-expectancy in patients with cystinosis, and disease complexity itself, justify planning a guided-transition of affected patients from Pediatrics to adult medicine. The aims of the process are to guarantee the continuum of care and patient empowerment, moving from guardian-care to self-care. METHODS: review of articles, expert opinion and anonymous surveys of patients, relatives and patient advocacy groups. RESULTS: elaboration a new document to support and coordinate the transition of patients with cystinosis providing specific proposals in a variety of medical fields, and adherence promotion. Nephrologists play a key role in transition due the fact that most cystinotic patients suffer severe chronic kidney disease, and need kidney transplantation before adulthood. CONCLUSION: we present a document providing recommendations and suggesting a chronogram to help the process of transition of adolescents and young adults with cystinosis in our area.


Assuntos
Cistinose/terapia , Transição para Assistência do Adulto , Adolescente , Adulto , Criança , Humanos , Transplante de Rim , Pediatria , Insuficiência Renal Crônica/terapia , Autocuidado , Adulto Jovem
10.
Nefrologia ; 35(3): 304-21, 2015.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-26523297

RESUMO

INTRODUCTION: Cystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment. The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis. METHODS: Bibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona. RESULTS: This document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis,renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling,nursing and pharmacy. CONCLUSIONS: A reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: (a) a multi-disciplinary approach, (b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, (c) the importance of adherence to treatment with cysteamine, and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will contemplate the specific needs of cystinosis.


Assuntos
Assistência Integral à Saúde/normas , Cistinose/terapia , Transição para Assistência do Adulto/normas , Adolescente , Adulto , Sistemas de Transporte de Aminoácidos Neutros/deficiência , Sistemas de Transporte de Aminoácidos Neutros/genética , Doenças da Córnea/diagnóstico , Doenças da Córnea/etiologia , Doenças da Córnea/terapia , Cisteamina/uso terapêutico , Cistinose/complicações , Cistinose/diagnóstico , Cistinose/genética , Gerenciamento Clínico , Diagnóstico Precoce , Doenças do Sistema Endócrino/diagnóstico , Doenças do Sistema Endócrino/etiologia , Doenças do Sistema Endócrino/terapia , Aconselhamento Genético , Humanos , Comunicação Interdisciplinar , Falência Renal Crônica/etiologia , Falência Renal Crônica/prevenção & controle , Falência Renal Crônica/terapia , Transplante de Rim , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/etiologia , Doenças do Sistema Nervoso/terapia , Educação de Pacientes como Assunto , Qualidade de Vida , Autocuidado
11.
Nefrología (Madr.) ; 35(3): 304-321, mayo-jun. 2015. ilus, tab
Artigo em Inglês | IBECS | ID: ibc-140060

RESUMO

Introduction: Cystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment. The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis. Methods: Bibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona. Results: This document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis, renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling, nursing and pharmacy. Conclusions: A reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: (a) a multi-disciplinary approach, (b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, (c) the importance of adherence to treatment with cysteamine, and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will contemplate the specific needs of cystinosis (AU)


Introducción: La cistinosis es una enfermedad lisosomal minoritaria de expresión sistémica con especial afectación renal y oftalmológica, en la que los pacientes inician terapia renal sustitutiva en la primera década de la vida en ausencia de tratamiento. El pronóstico de la cistinosis depende del diagnóstico precoz, la pronta instauración del tratamiento con cisteamina y el buen cumplimiento terapéutico. La progresión de la enfermedad renal y de las complicaciones extrarrenales y una menor supervivencia, son más acentuadas en pacientes no adherentes. Objetivo: El objetivo de este trabajo fue la elaboración de unas recomendaciones para la atención integral de la cistinosis y la transición del adolescente a la medicina del adulto, basadas en la experiencia clínica, con el fin de reducir el impacto de la enfermedad y mejorar la calidad de vida y el pronóstico del paciente. Método: Búsqueda bibliográfica y reuniones de consenso de un equipo multidisciplinar de expertos en la práctica clínica con pacientes afectos de cistinosis (Grupo T-CiS.bcn), procedentes de 5 hospitales localizados en Barcelona. Resultados: El documento recoge recomendaciones específicas y necesarias para el diagnóstico, tratamiento y seguimiento multidisciplinar de la cistinosis en las siguientes áreas: nefrología, diálisis, trasplante renal, oftalmología, endocrinología, neurología, laboratorio, consejo genético, enfermería y farmacia. Conclusiones: Disponer de un documento de referencia para la atención integral de la cistinosis constituye una herramienta de soporte para los profesionales de la salud que asisten a estos pacientes. Los principales pilares en los que se sustenta son: a) el enfoque multidisciplinar, b) la adecuada monitorización de la enfermedad y control de los niveles de cistina intraleucocitarios, c) la importancia de la adherencia al tratamiento con cisteamina y d) la promoción del autocuidado del paciente mediante programas de educación en la enfermedad. Todo ello conducirá, en una segunda fase, a la elaboración de un modelo de transición coordinado entre los servicios de pediatría y de adultos que contemple las necesidades específicas de la cistinosis (AU)


Assuntos
Adolescente , Adulto , Feminino , Humanos , Masculino , Cistinose/epidemiologia , Cistinose/prevenção & controle , /métodos , /tendências , Cisteamina/administração & dosagem , Cisteamina/análise , Prognóstico , Diagnóstico Precoce , Síndrome de Fanconi/complicações , Síndrome de Fanconi/diagnóstico , Insuficiência Renal Crônica/complicações , Insuficiência Renal Crônica/diagnóstico , Insuficiência Renal Crônica/etiologia , Diálise Renal/métodos , Diálise Renal , Biologia Molecular/métodos
12.
Clin Interv Aging ; 10: 713-23, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25926725

RESUMO

OBJECTIVE: To assess the effect of free leucine supplementation combined with resistance training versus resistance training only on muscle strength and functional status in older adults. METHODS: This was a randomized, double-blind, placebo-controlled, parallel study with two intervention groups. Thirty older adults were randomly assigned to receive either 10 g leucine/day (leucine group [LG], n=15) or a placebo (control group [CG], n=15), plus resistance training over a 12-week period. Maximal overcoming isometric leg strength, functional status, nutritional status, body composition, health-related quality of life, depression, and dietary intake were assessed at 4 and 12 weeks. Missing data at 12 weeks were handled using mixed models for repeated measurements for data imputation. RESULTS: Twenty-four subjects completed the 4-week assessment and eleven completed the 12-week intervention. Clinically significant gains were found in isometric leg strength at both assessment time points. Analysis of the effect size also showed how participants in LG outperformed those in CG for chair stands and the timed up and go test. No significant changes were observed for the rest of the outcomes. CONCLUSION: Our combined analysis showed moderate changes in isometric leg muscle strength and certain components of functional status. The magnitude of changes found on these outcomes should be qualified as a positive effect of the concomitant intervention.


Assuntos
Suplementos Nutricionais , Nível de Saúde , Leucina/administração & dosagem , Qualidade de Vida , Treinamento de Força/métodos , Idoso , Idoso de 80 Anos ou mais , Composição Corporal , Depressão/epidemiologia , Método Duplo-Cego , Feminino , Humanos , Masculino , Força Muscular , Estado Nutricional
13.
BMC Infect Dis ; 14: 497, 2014 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-25209653

RESUMO

BACKGROUND: The effects of ezetimibe on cholesterol metabolism in HIV-infected patients receiving boosted protease inhibitors have not been thoroughly assessed. The aim of this study was to assess cholesterol homeostasis in patients with PI associated dyslipidemia and its relationship with the response to treatment with the cholesterol-absorption inhibitor ezetimibe. METHODS: Fifteen patients with ritonavir-boosted PI-containig therapy and LDL-cholesterol > 3.36 mmol/L (>130 mg/dL) were assessed at baseline and after an 8-week course of ezetimibe 10 mg/d. Serum non-cholesterol sterols were measured at each visit as markers of cholesterol synthesis and absorption. Total-, LDL-, and HDL-cholesterol triglycerides, apolipoproteins A1 and B, high sensitivity C-reactive protein, CD4 cells and HIV-1 RNA were also measured. RESULTS: Ezetimibe treatment was well tolerated in all patients and resulted in significant reductions in total cholesterol (-11.4%, p = .002), LDL-cholesterol (-20.4%, p = .003), non-HDL-cholesterol (-13.4%, p = .002) and apolipoprotein B (-9.1%, p = .021). Treatment with ezetimibe was associated with decreased cholesterol absorption markers (campesterol-to-cholesterol ratio -43.0%, p = .001; sitosterol-to-cholesterol ratio -41.9%, p = .001) and increased synthesis markers (lathosterol-to-cholesterol ratio 53.2%, p = .005). Baseline absorption or synthesis markers were unrelated to the response to treatment. CD4 cell count and plasma HIV-1 RNA remained unchanged. CONCLUSIONS: The level of cholesterol absorption or synthesis does not appear to be a major determinant of the responsiveness to ezetimibe in patients on ritonavir-boosted PI-containing therapy. TRIAL REGISTRATION: EudraCT: 2006-006156-36.


Assuntos
Azetidinas/uso terapêutico , LDL-Colesterol/metabolismo , Dislipidemias/tratamento farmacológico , Infecções por HIV/complicações , Inibidores de Proteases/uso terapêutico , Adulto , Idoso , Anticolesterolemiantes/uso terapêutico , Proteína C-Reativa/química , Colesterol/análogos & derivados , Colesterol/uso terapêutico , Ezetimiba , Feminino , Infecções por HIV/tratamento farmacológico , Humanos , Metabolismo dos Lipídeos , Masculino , Pessoa de Meia-Idade , Fitosteróis/uso terapêutico , Inibidores de Proteases/efeitos adversos
14.
Nutr Hosp ; 29(2): 382-7, 2014 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-24528357

RESUMO

INTRODUCTION: Malnutrition is a cause for concern among many admitted elderly patients, being common at hospital admission and discharge. OBJECTIVES: The objective of this study was to assess if diet enrichment with small servings of energy and protein dense foods, improves energy and nutrient intake in elderly patients at risk of malnutrition discharged to home. METHODS: This was a retrospective case series study in elderly patients at risk of malnutrition treated with diet enrichment. There was a data review of dietary and health records of elderly patients discharged to home. Forty-one patients, mean age of 83 ± 5 years, met the inclusion criteria; 13 patients had been lost after 4 weeks of treatment and a total of 24 patients after 12 weeks. Records contained food intake data assessed at baseline, and after 4 and 12 weeks of treatment. Mini Nutritional Assessment, anthropometric measurements, routine biochemical parameters and the Barthel Index were assessed at baseline and after 12 weeks. RESULTS: Compared to baseline, patients significantly improved their energy and protein intake after 4 weeks of treatment, fulfilling the mean nutritional requirements. The improvement in energy and protein intake was still manifest at week 12. After 12 weeks of dietary enrichment, a significant weight gain was observed (4.1%, p = 0.011), as well. No significant changes were detected in functional status. CONCLUSIONS: Using small servings of energy and protein dense foods to enrich meals seems a feasible nutritional treatment to increase energy and protein intake and meet nutritional goals among elderly patients discharged to home.


Introducción: La desnutrición es causa de preocupación en muchos pacientes ingresados, siendo frecuente al ingreso y alta hospitalaria. Objetivos: El objetivo de este estudio fue valorar si el enriquecimiento de la dieta con pequeñas raciones de alimentos densos en energía y nutrientes mejora la ingesta energética y de nutrientes en pacientes ancianos con riesgo de desnutrición dados de alta al domicilio. Métodos: Estudio retrospectivo de una serie de casos en paciente ancianos con riesgo de desnutrición tratados con enriquecimiento de la dieta. Se revisaron los datos de la historia clínica y dietética de pacientes ancianos dados de alta a domicilio. Cuarenta-y-un pacientes, con una edad media de 83 ± 5 años, cumplieron los criterios de inclusión; 13 pacientes se perdieron después de 4 semanas de tratamiento y un total de 24 después de 12 semanas. El historial contenía datos de la ingesta de alimentos valorada a nivel basal, y después de 4 y 12 semanas de tratamiento. El Mini Nutritional Assessment, las medidas antropométricas, los parámetros bioquímicos rutinarios y el Índice de Barthel fueron valorados a nivel basal y después de 12 semanas. Resultados: En comparación al inicio, los pacientes mejoraron significativamente su ingesta energética y proteica después de 4 semanas de tratamiento, cumpliendo con los requerimientos nutricionales medios. La mejora en la ingesta de energía y proteínas todavía era manifiesta en la semana 12. Después de 12 semanas de enriquecimiento de la dieta, también se observó un incremento significativo en el peso (4.1%, p = 0.011). No se detectaron cambios significativos en el estado funcional. Conclusiones: El uso de pequeñas raciones de alimentos con elevada densidad energética y proteica para enriquecer las comidas parece ser un tratamiento nutricional factible para incrementar la ingesta energética y proteica y cumplir con los objetivos nutricionales en paciente ancianos dados de alta al domicilio.


Assuntos
Proteínas na Dieta/farmacologia , Ingestão de Energia , Desnutrição/dietoterapia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Estudos Retrospectivos
15.
Nutr. hosp ; 29(2): 382-387, 2014. tab
Artigo em Inglês | IBECS | ID: ibc-120599

RESUMO

Introduction: Malnutrition is a cause for concern among many admitted elderly patients, being common at hospital admission and discharge. Objectives: The objective of this study was to assess if diet enrichment with small servings of energy and protein dense foods, improves energy and nutrient intake in elderly patients at risk of malnutrition discharged to home. Methods: This was a retrospective case series study in elderly patients at risk of malnutrition treated with diet enrichment. There was a data review of dietary and health records of elderly patients discharged to home. Forty-one patients, mean age of 83 ± 5 years, met the inclusion criteria; 13 patients had been lost after 4 weeks of treatment and a total of 24 patients after 12 weeks. Records contained food intake data assessed at baseline, and after 4 and 12 weeks of treatment. Mini Nutritional Assessment, anthropometric measurements, routine biochemical parameters and the Barthel Index were assessed at baseline and after 12 weeks. Results: Compared to baseline, patients significantly improved their energy and protein intake after 4 weeks of treatment, fulfilling the mean nutritional requirements. The improvement in energy and protein intake was still manifest at week 12. After 12 weeks of dietary enrichment, a significant weight gain was observed (4.1%, p = 0.011), as well. No significant changes were detected in functional status. Conclusions: Using small servings of energy and protein dense foods to enrich meals seems a feasible nutritional treatment to increase energy and protein intake and meet nutritional goals among elderly patients discharged to home (AU)


Introducción: La desnutrición es causa de preocupación en muchos pacientes ingresados, siendo frecuente al ingreso y alta hospitalaria. Objetivos: El objetivo de este estudio fue valorar si el enriquecimiento de la dieta con pequeñas raciones de alimentos densos en energía y nutrientes mejora la ingesta energética y de nutrientes en pacientes ancianos con riesgo de desnutrición dados de alta al domicilio. Métodos: Estudio retrospectivo de una serie de casos en paciente ancianos con riesgo de desnutrición tratados con enriquecimiento de la dieta. Se revisaron los datos de la historia clínica y dietética de pacientes ancianos dados de alta a domicilio. Cuarenta-y-un pacientes, con una edad media de 83 ± 5 años, cumplieron los criterios de inclusión; 13 pacientes se perdieron después de 4 semanas de tratamiento y un total de 24 después de 12 semanas. El historial contenía datos de la ingesta de alimentos valorada a nivel basal, y después de 4 y 12 semanas de tratamiento. El Mini Nutritional Assessment, las medidas antropométricas, los parámetros bioquímicos rutinarios y el Índice de Barthel fueron valorados a nivel basal y después de 12 semanas. Resultados: En comparación al inicio, los pacientes mejoraron significativamente su ingesta energética y proteica después de 4 semanas de tratamiento, cumpliendo con los requerimientos nutricionales medios. La mejora en la ingesta de energía y proteínas todavía era manifiesta en la semana 12. Después de 12 semanas de enriquecimiento de la dieta, también se observó un incremento significativo en el peso (4.1%, p = 0.011). No se detectaron cambios significativos en el estado funcional. Conclusiones: El uso de pequeñas raciones de alimentos con elevada densidad energética y proteica para enriquecer las comidas parece ser un tratamiento nutricional factible para incrementar la ingesta energética y proteica y cumplir con los objetivos nutricionales en paciente ancianos dados de alta al domicilio (AU)


Assuntos
Humanos , Masculino , Feminino , Idoso , Idoso de 80 Anos ou mais , Avaliação Nutricional , Estado Nutricional , Nutrição do Idoso , Desnutrição/dietoterapia , Alimentos Fortificados , Avaliação Geriátrica/métodos , Apoio Nutricional/métodos , Hospitalização/estatística & dados numéricos , Alta do Paciente/estatística & dados numéricos , Continuidade da Assistência ao Paciente/organização & administração , Ingestão de Energia/fisiologia , Atividades Cotidianas
16.
Nutr. hosp ; 25(5): 736-740, sept.-oct. 2010. graf, tab
Artigo em Inglês | IBECS | ID: ibc-97294

RESUMO

Objectives: To assess the effect of an intervention with an Eicosapentaenoic Acid-enriched oral nutritional supplement on chemotherapy tolerability in patients with advanced colorectal cancer. Methods: Thirteen patients diagnosed with stage IV colorectal cancer were included. Patients in the experimental group received 2 packs of supplement per day during 12 weeks plus dietary counseling. The control group only received dietary counseling. Patients were assessed for nutritional status, dietary intake, health related quality of life (HRQOL) and chemotherapy compliance. Results: Only patients in the supplemented group significantly increased their weight after the intervention.T hey also had better scores in important domains of HRQOL, compared to controls. Although not statistically significant, the supplemented group did not experience interruptions in their chemotherapy treatment compared to the control group, with more interruptions due totoxicity. Conclusions: The present study, although limited by sample size, points out towards a positive effect of the intervention on chemotherapy tolerability (AU)


Objetivos: Valorar el efecto de una intervención con un suplemento nutricional oral enriquecido en Ácido Eicosapentaenoico sobre la tolerabilidad a la quimioterapia en pacientes con cáncer colorrectal avanzado. Métodos: Se incluyeron 13 pacientes diagnosticados con cáncer colorrectal en estadio IV. Los pacientes en el grupo experimental recibieron 2 briks de suplemento al día durante 12 semanas junto a consejo dietético. El grupo control solo recibió consejo dietético. Se valoró el estado nutricional, ingesta dietética, calidad de vida relacionada con la salud (CVRS) y el cumplimento de la quimioterapia de los pacientes. Resultados: Solo los pacientes en el grupo suplementado incrementaron su peso significativamente tras la intervención. También obtuvieron mejores puntuaciones en importantes dominios de la CVRS, comparado con los controles. Aunque sin ser estadísticamente significativo, el grupo suplementado no experimentó interrupciones en el tratamiento de quimioterapia comparado con el grupo control, con más interrupciones debidas a toxicidad. Conclusiones: El presente estudio, aunque limitado por el tamaño muestral, apunta hacia un efecto positivo de la intervención sobre la tolerabilidad a la quimioterapia (AU)


Assuntos
Humanos , Alimentos Fortificados , Ácido Eicosapentaenoico/farmacocinética , Neoplasias do Colo/dietoterapia , /dietoterapia , Neoplasias do Colo/tratamento farmacológico , Antineoplásicos/farmacocinética , /estatística & dados numéricos , Tolerância a Medicamentos
17.
Nutr Hosp ; 25(5): 736-40, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-21336429

RESUMO

OBJECTIVES: To assess the effect of an intervention with an Eicosapentaenoic Acid-enriched oral nutritional supplement on chemotherapy tolerability in patients with advanced colorectal cancer. METHODS: Thirteen patients diagnosed with stage IV colorectal cancer were included. Patients in the experimental group received 2 packs of supplement per day during 12 weeks plus dietary counseling. The control group only received dietary counseling. Patients were assessed for nutritional status, dietary intake, health related quality of life (HRQOL) and chemotherapy compliance. RESULTS: Only patients in the supplemented group significantly increased their weight after the intervention. They also had better scores in important domains of HRQOL, compared to controls. Although not statistically significant, the supplemented group did not experience interruptions in their chemotherapy treatment compared to the control group, with more interruptions due to toxicity. CONCLUSIONS: The present study, although limited by sample size, points out towards a positive effect of the intervention on chemotherapy tolerability.


Assuntos
Antineoplásicos/efeitos adversos , Neoplasias Colorretais/complicações , Suplementos Nutricionais , Ácido Eicosapentaenoico/uso terapêutico , Idoso , Antineoplásicos/uso terapêutico , Neoplasias Colorretais/tratamento farmacológico , Dieta , Método Duplo-Cego , Ácido Eicosapentaenoico/administração & dosagem , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Cooperação do Paciente , Projetos Piloto , Estudos Prospectivos , Qualidade de Vida
18.
Antivir Ther ; 13(2): 149-59, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18505167

RESUMO

BACKGROUND: The use of combination antiretroviral therapy (cART) has improved the prognosis of HIV infection, but it has also been linked to a spectrum of body composition changes and metabolic alterations known as the lipodystrophy syndrome. Nutritional status could influence body composition changes. METHODS: We performed a systematic search of published peer-reviewed data on the effects of diet, nutrition support and exercise on body composition and metabolic complications in patients receiving cART. RESULTS: Few controlled studies, most of them with small sample size, were found. Oral nutritional support increases protein and energy intake, and results in body weight and fat mass gains. Resistance exercise, with or without an aerobic component, increases lean mass and can improve insulin resistance. Low-fat diets or exercise can result in loss of fat mass, and they should be used with caution in subjects with lipoatrophy. CONCLUSIONS: Nutritional support and exercise result in small but significant body composition changes and can be used as complementary interventions. There is a need for further research on nutritional interventions in HIV-infected patients receiving cART.


Assuntos
Fármacos Anti-HIV/uso terapêutico , Dieta , Suplementos Nutricionais , Exercício Físico , Infecções por HIV/tratamento farmacológico , Síndrome de Lipodistrofia Associada ao HIV/prevenção & controle , Inibidores da Transcriptase Reversa/uso terapêutico , Fármacos Anti-HIV/efeitos adversos , Composição Corporal , Método Duplo-Cego , Quimioterapia Combinada , Humanos , Estado Nutricional , Ensaios Clínicos Controlados Aleatórios como Assunto , Inibidores da Transcriptase Reversa/efeitos adversos
19.
Curr Opin HIV AIDS ; 3(3): 240-6, 2008 May.
Artigo em Inglês | MEDLINE | ID: mdl-19372973

RESUMO

PURPOSE OF REVIEW: The aim of this article is to analyse the effectiveness of lipid-lowering therapy in HIV-infected patients. RECENT FINDINGS: Although data on prevention of cardiovascular disease in HIV-infected patients are limited, available evidence suggests that intervention guidelines should be similar to those that are recommended for the general population. The main target of lipid-lowering therapy is LDL cholesterol; therefore statins are the drugs of choice. The efficacy of statins in HIV-infected persons appears to be lower than expected, although adherence to statin therapy has not been well assessed. Statins combining high potency and little clinically meaningful interactions with antiretroviral therapy (pravastatin, fluvastatin, atorvastatin and rosuvastatin) should be preferred as initial therapy, though comparative studies in HIV-infected persons are scarce. A combination of a statin at medium doses with either ezetimibe or a fibrate other than gemfibrozil may result in more satisfactory results than higher doses of statin monotherapy when LDL cholesterol goals are difficult to achieve or there persist elevated triglycerides and low HDL cholesterol, respectively. SUMMARY: Adequate choice and dosing of lipid-lowering drugs, given as isolated agents or in combination therapy, and care for good drug compliance in HIV-infected patients at moderate or high cardiovascular risk should help maximize their long-term health.

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