The natural history of classic galactosemia: lessons from the GalNet registry.

BACKGROUND: Classic galactosemia is a rare inborn error of carbohydrate metabolism, caused by a severe deficiency of the enzyme galactose-1-phosphate uridylyltransferase (GALT). A galactose-restricted diet has proven to be very effective to treat the neonatal life-threatening manifestations and has been the cornerstone of treatment for this severe disease. However, burdensome complications occur despite a lifelong diet. For rare diseases, a patient disease specific registry is fundamental to monitor the lifespan pathology and to evaluate the safety and efficacy of potential therapies. In 2014, the international Galactosemias Network (GalNet) developed a web-based patient registry for this disease, the GalNet Registry. The aim was to delineate the natural history of classic galactosemia based on a large dataset of patients. METHODS: Observational data derived from 15 countries and 32 centers including 509 patients were acquired between December 2014 and July 2018. RESULTS: Most affected patients experienced neonatal manifestations (79.8%) and despite following a diet developed brain impairments (85.0%), primary ovarian insufficiency (79.7%) and a diminished bone mineral density (26.5%). Newborn screening, age at onset of dietary treatment, strictness of the galactose-restricted diet, p.Gln188Arg mutation and GALT enzyme activity influenced the clinical picture. Detection by newborn screening and commencement of diet in the first week of life were associated with a more favorable outcome. A homozygous p.Gln188Arg mutation, GALT enzyme activity of ≤ 1% and strict galactose restriction were associated with a less favorable outcome. CONCLUSION: This study describes the natural history of classic galactosemia based on the hitherto largest data set.

How strict is galactose restriction in adults with galactosaemia? International practice.

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides ± fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.

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.

Patterns of food avoidance in chronic fatigue syndrome: is there a case for dietary recommendations?

OBJECTIVES: To assess the dietary habits and food avoidance-behavior in patients with Chronic Fatigue Syndrome (CFS). METHODS: Cross-sectional pilot study with 28 patients diagnosed with severe CFS. Eating habits were assessed with a food frequency questionnaire and 3-day food records. We analyzed variables related to dietary restrictions induced by symptoms or external information. RESULTS: The most prevalent restrictions were for dairy products and gluten-containing grains, with 22 and 15 restricting patients, respectively. Patients reported different digestive symptoms, which did not improve with the use of exclusion diets. Thirteen patients had received information against the intake of certain foods through different sources. Six cases of grains restriction and 11 of dairy were compatible with a counseling-induced pattern of exclusion. CONCLUSIONS: There is not a homogeneous pattern of food avoidance. Dietary restrictions should be based on a proven food allergy or intolerance. Dietary counseling should be based on sound nutritional knowledge.

Patterns of food avoidance in chronic fatigue syndrome; is there a case for dietary recommendations? / Patrones de evitación alimenticia en el síndrome de fatiga crónica; ¿hay base para dar recomendaciones dietéticas?

Objectives: To assess the dietary habits and food avoidance-behavior in patients with Chronic Fatigue Syndrome (CFS). Methods: Cross-sectional pilot study with 28 patients diagnosed with severe CFS. Eating habits were assessed with a food frequency questionnaire and 3-day food records. We analyzed variables related to dietary restrictions induced by symptoms or external information. Results: The most prevalent restrictions were for dairy products and gluten-containing grains, with 22 and 15 restricting patients, respectively. Patients reported different digestive symptoms, which did not improve with the use of exclusion diets. Thirteen patients had received information against the intake of certain foods through different sources. Six cases of grains restriction and 11 of dairy were compatible with a counseling-induced pattern of exclusion. Conclusions: There is not a homogeneous pattern of food avoidance. Dietary restrictions should be based on a proven food allergy or intolerance. Dietary counseling should be based on sound nutritional knowledge (AU)

Objetivos: Valorar los hábitos dietéticos y la conducta de evitación alimenticia en pacientes con Síndrome de Fatiga Crónica. Métodos: Estudio piloto transversal con 28 pacientes diagnosticados de Síndrome de Fatiga Crónica en grado severo. Los hábitos alimenticios se evaluaron mediante un cuestionario de frecuencia de consumo y registros dietéticos de 3 días. Se analizaron variables relacionadas con las restricciones dietéticas inducidas por síntomas o información externa. Resultados: Las restricciones más frecuentes fueron para los productos lácteos y cereales con gluten, con 22 y 15 pacientes que los restringían, respectivamente. Los pacientes informaron de diferentes síntomas digestivos, que no mejoraron con el uso de dietas de exclusión. Trece pacientes habían recibido información contraria al consumo de ciertos alimentos, a través de diferentes fuentes. Seis casos de restricción de cereales y 11 de lácteos fueron compatibles con un patrón de exclusión inducido por consejo. Conclusiones: No hay un patrón homogéneo de evitación alimenticia. Las restricciones dietéticas deberían basarse en una alergia o intolerancia alimentaria probada. El consejo dietético debería basarse en el conocimiento nutricional fundamentado (AU)

[Home Parenteral Nutrition (HPN) registry in Spain for the years 2007, 2008 and 2009 (NADYA-SENPE Group)]. / Registro de la Nutrición Parenteral Domiciliaria (NPD) en España de los años 2007, 2008 y 2009 (Grupo NADYA-SENPE).

OBJECTIVE: To report the data of the Home Parenteral Nutrition (HPN) registry of the NADYA-SENPE working group for the years 2007, 2008 and 2009. METHODOLOGY: We compiled the data from the on-line registry introduced by the responsible Units for the monitoring of HPN from January 1st 2007 to December 31st 2009. Included fields were: age, sex, diagnosis and reason for HPN, access path, complications, beginning and end dates, complementary oral or enteral nutrition, activity level, autonomy degree, product and fungible material supply, withdrawal reason and intestinal transplant indication. RESULTS: 2007: 133 patients with HPN were registered (61 males and 72 females), belonging to 21 hospitals. Average age for the 119 patients older than 13 years old was 53.7 ± 14.9 years, and 3.6 ± 3.6 y. for the 14 patients under 14 years old. Most frequent pathology was neoplasm (24%), followed by intestinal motility disorders and actinic enteritis (14% both). The reason for HPN provision was short bowel syndrome (43%), malabsorption (27%), and intestinal obstruction (23%). Tunnelled catheters were mostly used (69%), followed by implanted port-catheters (27%). Catheter related infections were the most frequent complications, with a rate of 0.92 episodes/103 HPN days. HPN was provided for more than two years in 50% of the cases. By the end of 2007, 71.4% of the patients remained active; exitus was the most frequent reason to end HPN (57.5%). 26% of the patients were eligible for intestinal transplant. 2008: 143 patients with HPN were registered (62 males and 81 females), belonging to 24 hospitals. Average age for the 133 patients older than 13 years old was 54.7 ± 13.9 years, and 3.7 ± 0.6 y. for the 10 patients under 14 years old. Most frequent pathology was neoplasm (20%), followed by actinic enteritis (14%) and intestinal motility disorders (13% ). The reason for HPN provision was short bowel syndrome (44%), malabsorption (28%), and intestinal obstruction (20%). Tunnelled catheters were mostly used (60%), followed by implanted port-catheters (29%). Catheter related infections were the most frequent complications, with a rate of 0.50 episodes/10³ HPN days. HPN was provided for more than two years in 67% of the cases. By the end of 2008, 71.6% of the patients remained active; exitus was the most frequent reason to end HPN (52.4%). 29% of the patients were eligible for intestinal transplant. 2009: 158 patients with HPN were registered (62 males and 96 females), belonging to 24 hospitals. Average age for the 149 patients older than 13 years old was 55.2 ± 13.0 years. Most frequent pathology was neoplasm (25%), followed by actinic enteritis (12%) and intestinal motility disorders (11%). The reason for HPN provision was short bowel syndrome (42%), malabsorption, and intestinal obstruction (23% both). Tunnelled catheters were mostly used (60%), followed by implanted port-catheters (36%). Catheter related infections were the most frequent complications, with a rate of 0.67 episodes/10³ HPN days. HPN was provided for more than two years in 58% of the cases. By the end of 2009, 79.2% of the patients remained active; full oral nutrition was the most frequent reason to end HPN (48%). 23% of the patients were eligible for intestinal transplant. CONCLUSIONS: We observe an increase in registered patients with respect to previous years, with a very different prevalence among regions. Neoplasia remains as the main pathology since 2003. We observe a decrease in catheter-related infections in the last two years, being the 2008 rate the smallest since the register's beginning.

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

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

Factors associated with nosocomial diarrhea in patients with enteral tube feeding / Factores asociados a diarrea nosocomial en pacientes con nutrición enteral

Objectives: Diarrhea is a frequent complication associated to enteral tube feeding (ETF) and it is a frequent cause of reduction or suspension of this type of nutritional support. Our objective was to evaluate the factors associated with nosocomial diarrhea in patients receiving ETF. Results: The only significant factor associated with the appearance of diarrhea was antibiotic consumption, specially those patients receiving the combination of two or more antibiotics. We did not find any association between factors related to ETF, analytical parameters, nor other medications and diarrhea. Conclusions: Our observational data supports the idea that ETF should not be seen as a primary cause of diarrhea, other possible causes should be considered before reducing or discontinuing ETF administration (AU)

Objetivos: La diarrea es una complicación frecuente asociada a la nutrición enteral (NE) y es causa habitual de reducción o suspensión de este tipo de soporte nutricional. Nuestro objetivo fue evaluar los factores asociados a la diarrea nosocomial en pacientes que recibían NE. Resultados: El único factor significativo asociado con la aparición de diarrea fue el consumo de antibióticos, especialmente aquellos pacientes que recibían la combinación de dos o más antibióticos. No se encontró ninguna asociación entre factores relacionados a la NE, parámetros analíticos, ni otras medicaciones y la diarrea. Conclusión: Nuestros datos observacionales respaldan la idea que la NE no debería ser vista como causa principal de diarrea, debiéndose considerar otras causas posibles antes de reducir o suspender la administración de NE (AU)

Quality of life, dietary intake and nutritional status assessment in hospital admitted cancer patients.

OBJECTIVES: The objectives of this study were to assess the quality of life (QoL), nutritional status, and quantitative food intakes of non-terminal admitted cancer patients receiving oral feeding. As well as to evaluate what kind of relation exists between the quality of life, and the nutritional status and current intake. SCOPE: Medical Oncology and Radiotherapy Service ward at the Hospital Clinic de Barcelona. SUBJECTS: Fifty admitted patients in the Service ward. INTERVENTIONS: There was a follow-up of the dietary intake during 3 working days through direct observation, as well as an assessment of anthropometrical and biochemical parameters, a record of symptomatology related data, and a QoL assessment through the EORTC QLQ-C30 questionnaire. RESULTS: Our data show that 32.6% of the patients did not reach 25 kcal/kg/day, and 23.3% did not even fulfill 1 g protein/kg/day. Concerning QoL, mean score for global health status and overall QoL for all patients was 46.2. Compared to the general population, there were important deficits among cancer patients regarding physical, role and social functioning. The most pronounced differences in the symptom scales were for fatigue, and in single items for appetite loss and constipation. A low protein intake was associated to a poorer perception on physical functioning (p = 0.01), and fatigue was close to significance (p = 0.058). No significant differences were found regarding caloric intake and QoL. CONCLUSIONS: A significant percentage of patients who received exclusive oral feeding did not cover a minimum acceptable quantity of their protein-energy requirements. Our results point-out that poor food intakes can affect QoL by themselves.

[The year 2003 National Registry of Home-based Parenteral Nutrition]. / Registro Nacional de la Nutrición Parenteral Domiciliaria del año 2003.

OBJECTIVE: To report the results of the Home-based Parenteral Nutrition (HBPN) registry of the NADYA-SENPE working group, for the year 2003. MATERIAL AND METHODS: Gathering of registry data introduced by all units responsible of HBPN patient care. This an on-line registry available for authorized users of the working group web page (www.nadya-senpe.com). Epidemiological data, diagnosis, access route, complications, hospital admissions, disability degree, and course at December 31st, 2003 RESULTS: Data from 86 patients (62% female and 38% male) from 17 hospitals were gathered. Mean age of adult patients was 50.7 +/- 15.0 years, whereas for patients younger than 14 years was 2.4 +/- 1.5 years (n = 5 patients). Diseases that prone HBPN were neoplasm (21%), followed by mesenteric ischemia (20%), radiation enteritis (16.3%), motility impairments (10.5%), and Crohn's disease (4.6%). Tunneled catheters were used in 66.3% of the cases versus 29.1% of subcutaneous reservoirs. Mean treatment duration has been 8.5 +/- 4.6 months; 67.4% of patients had been on HBPN for a period of time longer than 6 months. Patient follow-up was mostly done from the reference area hospital (88.4%). In no case patient follow-up was done by the primary care team or by specialists other than those prescribing nutritional support. Nutritional support-related complications were seen in 98 occasions. The most frequent complications were infectious ones. They represented 1.60 hospital admissions per patient. The mean number of visits was 7.9 per patient (6.4 for scheduled visits and 1.5 for emergency visits). By the end of the year, we observed that 73.3% of the patients were still on the program, whereas in 23.3% HBPN had been withdrawn. The main reasons for withdrawal were decease (11 patients), and advancing to oral diet (9 patients). As for the disability degree, 13% were confined to a wheelchair or bed, and only 28% had no disability degree or only mild social disability. CONCLUSIONS: We observed a mild increase in HBPN prevalence rate in Spain (2.15 patients pmp). The main indication was cancer followed by short-bowel syndrome secondary to vascular pathology. Nutritional support-related complications were common, especially those of an infectious origin.

[The year 2002 national register on home-based parenteral nutrition]. / Registro nacional de la nutrición parenteral domiciliaria del año 2002.

AIM: To report on the results of the Registry on Home-based Parenteral Nutrition (HPN) of the NADYA-SENPE working group, corresponding to the year 2002. MATERIALS AND METHOD: Compilation of the registry data loaded by the Units in charge of HPN patients care. It consists of an on-line registry available to the registered users of the group's web page (www.nadya-senpe.com). Epidemiological, diagnostic, access route, complications, hospital admissions, degree of disability, and course until December 31st of 2002. RESULTS: Data from 74 patients were gathered (56.8% women and 43.2% men), from 18 hospital centers. Mean age of adult patients was 49.4 +/- 15.5 years and 2.3-1.1 years for patients younger than 14 years (n=3 patients). Diseases that prompted the use of HPN were mesenteric ischemia (29.7%), followed by neoplasms (16.2%), radiation enteritis (12.2%), motility impairments (8.1%), and Crohn's disease (5.4%). Tunneled catheters were used in 52.7% of cases, as compared to 36.5% of subcutaneous reservoirs. Mean treatment duration was 8.7 +/- 4.4 months; 68.9% of patients remained on HPN for a duration longer than 6 months, and in 41.9% longer than one year. Patients' follow-up was mainly done from the reference hospital (87.8%), and the remaining patients (12.5%) by the home care team. In no case patients were followed by the primary care team or other specialists than the ones that prescribed nutritional support. In 94 cases there were complications related to nutritional therapy. The more frequent complications presented were infectious. These complications represented 1.84 admissions per patient. The mean number of visits was 12.9 per patient (10.2 routinary visits and 2.7 emergency visits). At the end of the year, we observed that 74.3% patients stayed in the program, whereas in the remaining 23.6% HPN had been discontinued. The main causes for discontinuation were death (52.9%), and switch to oral diet (23.5%) or enteral nutrition (11.8%). With regards to disability degree, 16.1% were confined to a wheelchair or bed, and 17.6% had no disability at all or only a mild social disability. CONCLUSIONS: We observed a sustained HPN prevalence rate in Spain (1.8 patient pmp). The main cause for its use was short bowel syndrome secondary to vascular disease, followed by cancer. Complications associated to nutritional therapy were common, especially of infectious origin.

[National Registry of Home Parenteral Nutrition of the year 2001]. / Registro Nacional de la Nutrición Parenteral Domiciliaria (NPD) del año 2001.

AIM: We analyse the registered data of home parenteral nutrition (HPN) in our country during the year 2001. METHOD: The data were collected through a previously designed questionnaire. Apart from epidemiological information, the form includes the disease to prescribe this treatment, the specific nutritional treatment used and its duration, access path, complications and readmission rate in hospital, follow-up of the treatment, and progress. All data were processed and analysed by the co-ordinating team. RESULTS: Seventeen hospitals participated, and 66 patients were enrolled. Middle age was 5.5 +/- 4.9 years for patients < 14 years old, and 49.2 +/- 15.8 years for those > or = 14 years old. The more prevalent diagnosis were: ischemic bowel (28.9%), neoplasm (22.7%), radiation enteritis (12.1%), motility disorders (4.5%) and Crohn's disease (4.5%). The mean time on HPN was 8.4 +/- 4.5 months. Tunnelled catheter was the preferential route (62.1%), followed by the implantated one (33.3%). The intermittent method (nocturnal) was preferential (81.8%). Patients receive the formula, mainly from hospital pharmacy (75.7%). The complications related to nutrition (1.3/patient) included the infections (0.46 sepsis/patient, and 0.19 catheter contamination/patients), mechanic (0.15/patient), metabolic (0.1/patient) and electrolytic disorders (0.07/patient). The readmission rate, for nutritional problems, was 1.34/patient. At the end of the year, 74.2% of the patients remained in the HPN program, and 25.8% abandoned the treatment (due to death: 52.9%, and to progress to oral feeding (25.3%). CONCLUSIONS: This review illustrates that the registration of HPN patients in our country is standing (1.65 patients/10(6) habitants), that vascular pathology is the more frequent diagnoses in HPN patients, and the rate of readmission and complications and the behaviour is similar to other series making this as a safe treatment in our place.

[Home Enteral Nutrition: National Registry 2001]. / Nutrición Enteral Domiciliaria (NED): registro nacional 2001.

GOAL: The NADYA-SENPE Working Group analyzed the registered data of patients on Home Enteral Nutrition (HEN) in our country, during year 2001. MATERIAL AND METHODS: The data were collected through a closed questionnaire included on our web site (www.nadya-senpe.com). Apart from epidemiological information, the form includes the indication to prescribe this treatment, the specific nutritional treatment used and its duration, access path, complications and readmission rate in hospital, follow-up of the treatment, patient's quality of life and progress. All data were processed and analyzed by the coordinating team. RESULTS: Twenty two hospitals participated and 3,458 patients, aged 5.6 +/- 4.0 y for those younger than 14 y, and 67.1 +/- 19.5 y for those older than 14 y, were enrolled. Of these patients, 43.4% were diagnosed with neurological diseases and 33.5% with cancer. The mean time on HEN was 6.5 +/- 4.5 months. Oral nutrition was the preferential route (54.5%), followed by nasoenteral tube (32.3%), and in 13.3% ostomy tubes were placed. Polymeric was the formula composition mainly used (85.9%). Patients were followed (71.1%) by the hospital reference Nutritional Support Unit. The complications related to nutrition included mainly the gastrointestinal (0.16 complications/patient), and the mechanical one (0.15 complications/patient). At the end for the year, 48.3% of the patients were in the HEN program, and in 33.3% HEN was finish due to different reasons. In 22.9% of the patients no, o light, discapacity degree was found. CONCLUSIONS: Neurological diseases and cancer were the more frequent diagnoses in HEN patients. Oral access was the higher feeding route due, probably, to the high prevalence of cancer patients. In spite of the elevated prevalence of neurological diseases, a few number of patients, as previous years, were feed with ostomy tube. Due to the few complications observed, HEN is a safe treatment in our country.

[Parenteral nutrition at home: NADYA register for the year 2000]. / Nutrición parenteral domiciliaria (NPD): registro NADYA. Año 2000.

AIM: We analyze the registered data of home parenteral nutrition (HPN) in our country during the year 2000. METHOD: The data were collected through a previously designed questionnaire. Apart from epidemiological information, the form includes the disease to prescribe this treatment, the specific nutritional treatment used and its duration, access path, complications and readmission rate in hospital, follow-up of the treatment, and progress. All data were processed and analysed by the co-ordinating team. RESULTS: Fourteen hospitals participated, and 67 patients were enrolled. Middle age was 5 +/- 4 years for patients < 14 years old, and 48 +/- 15 years for those > or = 14 years old. The more prevalent diagnosis were: ischemic bowel (28.4%), neoplasm (16.4%), radiation enteritis (13.4%), motility disorders (7.5%), Crohn's disease (2.9%), and other. The mean time on HPN was 7.5 +/- 4.4 months. Tunelized catheter was the preferential route (77.6%), followed by the implantated one (20.9%). The intermittent method (nocturnal) was preferential (91.0%). Patients receive the formula from hospital pharmacy more frequently than from Nutriservice (71.5% versus 19.4%). The complications related to nutrition (0.32/100 days of HPN) included the infections (0.12 catheter sepsis/100 d of HPN), metabolic (0.06/100 d of HPN), mechanic (0.03/100 d of HPN) and electrolitic disorders (0.03/100 d of HPN). The readmission rate, for nutritional problems, was 0.3 hospitalizations/100 d of HPN. At the end of the year, 61.2% of the patients remained in the HPN program, 37.3% abandoned the treatment (due to death (40%), to progress to oral feeding (48%), and to progress to enteral nutrition (4%); and 1.5% of the patients were not follow up. CONCLUSIONS: This review illustrates that there is an increment in the registration of HPN patients in our country (1997: 0.7 patients/10(6) habitants, 2000: 1.9 patients/10(6) habitants), that vascular pathology is the more frequent diagnoses in HPN patients, and the rate of readmission and complications and the behaviour is similar to other series making this as a safe treatment in our place.

Nutrición parenteral domiciliaria (NPD): registro NADYA. Año 2000 / Parenteral nutrition at home: NADYA register for 2000

Objetivo: Análisis descriptivo y valoración de los datos registrados por el Grupo de Trabajo NADYA-SENPE de pacientes que han recibido nutrición parenteral domiciliaria (NPD) en nuestro país durante el año 2000.Material y métodos: Se han recopilado datos (epidemiológicos, diagnóstico, vía de acceso, complicaciones, reingresos hospitalarios, duración del tratamiento y evolución) a partir de un cuestionario previamente diseñado para ello. Los datos obtenidos del cuestionario se introdujeron en una base de datos para la ulterior gestión de los mismos. Resultados: Los datos correspondían a 67 pacientes (edad media de 5 ñ 4 años para pacientes < 14 años y 48 ñ 15 años para 14 años) controlados por 14 centros hospitalarios. Las patologías que con más frecuencia han comportado este tratamiento fueron: isquemia mesentérica (28,4 per cent), seguida de enfermedad neoplásica (16,4 per cent), enteritis rádica (13,4 per cent), alteraciones de la motilidad (7,5 per cent) y enfermedad de Crohn (2,9 per cent), entre otras. Se observó un predominio de los catéteres tunelizados (77,6 per cent) sobre los implantados (20,9 per cent), y de la administración cíclica (nocturna) de la nutrición parenteral (NP) (91,0 per cent). Las farmacias hospitalarias suministraron las fórmulas de NPD con más frecuencia que el Nutriservice (71,5 per cent frente a 19,4 per cent).La duración media del tratamiento fue de 7,48 ñ 4,39 meses. Se presentaron 0,32 complicaciones/100 días de NPD relacionadas con el tratamiento nutritivo con predominio de las infecciosas (0,12 sepsis por catéter/100 d de NPD), seguidas de las metabólicas (0,06/100 d de NPD), y de las mecánicas (0,03/100 d de NPD) e hidroelectrolíticas (0,03/100 días de NPD). Estas complicaciones comportaron 0,3 hospitalizaciones/100 días de NPD. Al finalizar el año, seguían en activo 61,2 per cent de los pacientes; 37,3 per cent habían abandonado el tratamiento (exitus 40 per cent, nutrición oral 48 per cent y nutrición enteral 4 per cent), y 1,5 per cent pacientes habían dejado de ser controlados por el equipo inicialmente responsable. Conclusiones: Podemos concluir que existe un incremento del registro de la NPD en nuestro país (1997: 0,7 pacientes/106 habitantes, 2000: 1,9 pacientes/106 habitantes), que hay un predominio de patología vascular y que la incidencia de complicaciones, retirada del tratamiento e ingresos hospitalarios son semejantes a otras series lo que avala la calidad del tratamiento (AU)

Aim: We analyze the registered data of home parenteral nutrition (HPN) in our country during the year 2000. Method: The data were collected through a previously designed questionnaire. Apart from epidemiological information, the form includes the disease to prescribe this treatment, the specific nutritional treatment used and its duration, access path, complications and readmission rate in hospital, follow-up of the treatment, and progress. All data were processed and analysed by the co-ordinating team. Results: Fourten hospitals participated, and 67 pacientes were enrolled. Middle age was 5 ± 4 years for patients < 14 years old, and 48 ± 15 years for those ≥ 14 years old. The more prevalent diagnosis were: ischemic bowel (28.4%), neoplasm (16.4%), radiation enteritis (13.4%), motility disorders (7.5%), Crohn’s disease (2.9%), and other. The mean time on HPN was 7.5 ± 4.4 months. Tunelized catheter was the preferential route (77.6%), followed by the implantated one (20.9%). The intermittent method (nocturnal) was preferential (91.0%). Patients receive the formula from hospital pharmacy more frequently than from Nutriservice (71.5% versus 19.4%). The complications related to nutrition (0.32/100 days of HPN) included the infections (0,12 catheter sepsis/100 d of HPN), metabolic (0.06/100 d of HPN), mechanic (0.03/100 d of HPN) and electrolitic disorders (0.03/100 d of HPN). The readmission rate, for nutritional problems, was 0,3 hospitalizations /100 d of HPN. At the end of the year, 61.2% of the patients remained in the HPN program, 37.3% abandoned the treatment (due to death (40%), to progress to oral feeding (48%), and to progress to enteral nutrition (4%); and 1.5% of the patients were not follow up. Conclusions: This review illustrates that there is an increment in the registration of HPN patients in our country (1997: 0.7 pacients/106habitants, 2000: 1.9 pacients/ 106 habitants), that vascular pathology is the more frequent diagnoses in HPN patients, and the rate of readmission and complications and the behaviour is similar to other series making this as a safe treatment in our place (AU)

[Home enteral nutrition. Case load of the Clinical Hospital of Barcelona]. / Nutrición enteral domiciliaria. Casuística del Hospital Clínico de Barcelona.

The use of Enteral Nutrition at Home (ENH) in the Hospital Clínico de Barcelona has grown greatly over the last decade, with a certain trend towards stabilization being observed in the last two years, but still growing at a rate of 9.2% per annum. A transverse analysis of the active patients on our ENH register at a given moment has revealed a total of 315 patients receiving treatment. The retrospective analysis of our series during 1998 gave a figure of 643 new cases. The most frequent indication for ENH was neoplasia (44%), followed by neurological pathology (28%). The administration route most frequently used was oral (66% of cases). In the oral route, oncological diagnoses were dominant (52%), whereas administration by means of a naso-gastric tube was mainly due to neurological disorders (72%). The use of PEG (12.5% of administrations via tube) was distributed between oncological and neurological patients, with a slight predominance of the first. Of those patients completing ENH in the same year, duration was in most cases (67%) less than one month. The decrease of the patient due to the underlying disorder was the main cause of termination, followed by the need for short-term nutritional support following discharge from hospital.