Protein Labelling Accuracy for UK Patients with PKU Following a Low Protein Diet.

A phenylalanine (protein)-restricted diet is the primary treatment for phenylketonuria (PKU). Patients are dependent on food protein labelling to successfully manage their condition. We evaluated the accuracy of protein labelling on packaged manufactured foods from supermarket websites for foods that may be eaten as part of a phenylalanine-restricted diet. Protein labelling information was evaluated for 462 food items ("free from", n = 159, regular, n = 303), divided into 16 food groups using supermarket website data. Data collection included protein content per portion/100 g when food was "as sold", "cooked" or "prepared"; cooking methods, and preparation instructions. Labelling errors affecting protein content were observed in every food group, with overall protein labelling unclear in 55% (n = 255/462) of foods. There was misleading, omitted, or erroneous (MOE) information in 43% (n = 68/159) of "free from" foods compared with 62% (n = 187/303) of regular foods, with fewer inaccuracies in "free from" food labelling (p = 0.007). Protein analysis was available for uncooked weight only but not cooked weight for 58% (n = 85/146) of foods; 4% (n = 17/462) had misleading protein content. There was a high rate of incomplete, misleading, or inaccurate data affecting the interpretation of the protein content of food items on supermarket websites. This could adversely affect metabolic control of patients with PKU and warrants serious consideration.

Uniformity of Food Protein Interpretation Amongst Dietitians for Patients with Phenylketonuria (PKU): 2020 UK National Consensus Statements.

In phenylketonuria (PKU), variable dietary advice provided by health professionals and social media leads to uncertainty for patients/caregivers reliant on accurate, evidence based dietary information. Over four years, 112 consensus statements concerning the allocation of foods in a low phenylalanine diet for PKU were developed by the British Inherited Metabolic Disease Dietitians Group (BIMDG-DG) from 34 PKU treatment centres, utilising 10 rounds of Delphi consultation to gain a majority (≥75%) decision. A mean of 29 UK dietitians (range: 18-40) and 18 treatment centres (range: 13-23) contributed in each round. Statements encompassed all foods/food groups divided into four categories based on defined protein/phenylalanine content: (1) foods high in protein/phenylalanine (best avoided); (2) foods allowed without restriction including fruit/vegetables containing phenylalanine ≤75 mg/100 g and most foods containing protein ≤0.5 g/100 g; (3) foods that should be calculated/weighed as an exchange food if they contain protein exchange ingredients (categorized into foods with a protein content of: >0.1 g/100 g (milk/plant milks only), >0.5 g/100 g (bread/pasta/cereal/flours), >1 g/100 g (cook-in/table-top sauces/dressings), >1.5 g/100 g (soya sauces)); and (4) fruit/vegetables containing phenylalanine >75 mg/100 g allocated as part of the protein/phenylalanine exchange system. These statements have been endorsed and translated into practical dietary management advice by the medical advisory dietitians for the National Society for PKU (NSPKU).

Hepatorenal Tyrosinaemia: Impact of a Simplified Diet on Metabolic Control and Clinical Outcome.

Background: Tyrosinaemia type 1 is a rare inherited metabolic disease caused by an enzyme defect in the tyrosine degradation pathway. It is treated using nitisinone and a low-protein diet. In a workshop in 2013, a group of nutritional specialists from Germany, Switzerland and Austria agreed to advocate a simplified low-protein diet and to allow more natural protein intake in patients with tyrosinaemia type 1. This retrospective study evaluates the recommendations made at different treatment centers and their impact on clinical symptoms and metabolic control. Methods: For this multicenter study, questionnaires were sent to nine participating treatment centers to collect data on the general therapeutic approach and data of 47 individual patients treated by those centers. Results: Dietary simplification allocating food to 3 categories led to increased tyrosine and phenylalanine blood concentrations without weighing food. Phenylalanine levels were significantly higher in comparison to a strict dietary regimen whereas tyrosine levels in plasma did not change. Non-inferiority was shown for the simplification and liberalization of the diet. Compliance with dietary recommendations was higher using the simplified diet in comparison to the stricter approach. Age correlates negatively with compliance. Conclusions: Simplification of the diet with increased natural protein intake based on three categories of food may be implemented in the diet of patients with tyrosinaemia type 1 without significantly altering metabolic control. Patient compliance is strongly influencing tyrosine blood concentrations. A subsequent prospective study with a larger sample size is necessary to get a better insight into the effect of dietary recommendations on metabolic control.

Nutrition process improvements for adult inpatients with inborn errors of metabolism using the i-PARIHS framework.

AIM: This project aimed to implement consensus recommendations and innovations that improve dietetic services to promote timely referral to optimise nutritional management for adult inpatients with inborn errors of metabolism (IEM). METHODS: The i-PARIHS framework was used to identify service gaps, implement innovations and evaluate the innovations within this single-site study. The constructs of this framework are: (i) review of the evidence; (ii) recognising patients and staff knowledge and attitudes; (iii) acknowledging the local context; and (iv) the facilitators role. This included a literature review and metabolic centre service comparisons to investigate dietetic referral and foodservice processes to inform the innovation. A 12-month chart audit (6 months retrospective and prospective of implemented innovation, respectively) to evaluate newly established dietetic referral and IEM nutrition provision procedures was also completed. RESULTS: The innovations implemented encompassed a clinical alert triggering urgent referral, nutrition sick day plans and metabolic diet and formula prescription via an 'alert' tab in electronic records. Eleven metabolic protein-restricted diets and nine formula recipes were introduced. Prior to the innovations, only 53% (n = 19/36) of inpatients with IEM were assessed by the dietitian and received appropriate nutrition within 24 hours. Following implementation of the innovations, 100% (n = 11/11) of inpatients with IEM received timely dietetic assessment and therapeutic nutrition. CONCLUSIONS: Implementation of innovations developed using the i-PARIHS framework is effective in timely notification of the metabolic dietitian of referrals. This ensures optimal nutritional management during admissions which is required in this group of high-risk patients.

Low-protein diet-induced hyperphagia and adiposity are modulated through interactions involving thermoregulation, motor activity, and protein quality in mice.

Low protein (LP)-containing diets can induce overeating in rodents and possibly in humans in an effort to meet protein requirement, but the effects on energy expenditure (EE) are unclear. The present study evaluated the changes induced by reducing dietary protein from 20% to 6%-using either soy protein or casein-on energy intake, body composition, and EE in mice housed at 22°C or at 30°C (thermal neutrality). LP feeding increased energy intake and adiposity, more in soy-fed than in casein-fed mice, but also increased EE, thus limiting fat accumulation. The increase in EE was due mainly to an increase in spontaneous motor activity related to EE and not to thermoregulation. However, the high cost of thermoregulation at 22°C and the subsequent heat exchanges between nonshivering thermogenesis, motor activity, and feeding induced large differences in adaptation between mice housed at 22°C and at 30°C.

Special low protein foods for phenylketonuria: availability in Europe and an examination of their nutritional profile.

BACKGROUND: Special low protein foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European countries and Turkey and 2) analyse the nutritional composition of SLPF available in one of these countries. METHODS: European Nutritionist Expert Panel on PKU (ENEP) members (Portugal, Spain, Belgium, Italy, Germany, Netherlands, UK, Denmark and Turkey) provided data on SPLF available in each country. The nutritional composition of Portuguese SLPF was compared with regular food products. RESULTS: The number of different SLPF available in each country varied widely with a median of 107 [ranging from 73 (Portugal) and 256 (Italy)]. Food analysis of SLPF available from a single country (Portugal) indicated that the mean phenylalanine content was higher in low protein baby cereals (mean 48 mg/100 g) and chocolate/energy bars/jelly (mean 41 mg/100 g). The energy content of different foods from a sub-group of SLPF (cookies) varied widely between 23 and 96 kcal/cookie. Low protein bread had a high fat content [mean 5.8 g/100 g (range 3.7 to 10)] compared with 1.6 g/100 g in regular bread. Seven of the 12 SLPF sub-groups (58 %) did not declare any vitamin content, and only 4 (33 %) identified a limited number of minerals. CONCLUSIONS: Whilst equal and free access to all SLPF is desirable, the widely variable nutritional composition requires careful nutritional knowledge of all products when prescribed for individual patients with PKU. There is a need for more specific nutritional standards for special low protein foods.

The appropriate standardized ileal digestible tryptophan to lysine ratio improves pig performance and regulates hormones and muscular amino acid transporters in late finishing gilts fed low-protein diets.

This study investigated the effects of various standardized ileal digestible (SID) Trp to Lys ratios on the performance and carcass characteristics of late finishing gilts receiving low-CP (9.6%) diets supplemented with crystalline AA. Ninety gilts (89.1 ± 5.1 kg) were used in a dose-response study conducted for 35 d. Crystalline Trp (0, 0.1, 0.2, 0.4, or 0.6 g/kg) was added to a corn-wheat bran basal diet providing SID Trp to Lys ratios of 0.12, 0.15, 0.18, 0.21, or 0.24. Each diet was fed to 6 pens of pigs with 3 gilts per pen. At the end of the experiment, 30 gilts (1 pig per pen) were slaughtered to evaluate carcass traits and meat quality (BW = 121 kg). Increasing the SID Trp to Lys ratio increased ADG (linear and quadratic effect, < 0.05) and also improved G:F (linear and quadratic effect, < 0.05). Serum urea nitrogen (SUN) decreased as the SID Trp to Lys ratio increased (linear and quadratic effects, < 0.05). A quadratic effect of L* light and marbling in the longissimus dorsi was observed as the dietary SID Trp to Lys ratio increased ( < 0.05). Increasing the SID Trp to Lys ratio increased the level of serum GH (quadratic effect, < 0.05) and also increased the level of serum IGF-1 (linear and quadratic effect, < 0.05). Increasing the SID Trp to Lys ratio increased the protein abundance of the muscular AA transporter of sodium-coupled neutral amino acid transporter 2 (SNAT2) in the longissimus dorsi muscle (linear and quadratic effect, < 0.05). The optimum SID Trp to Lys ratios to maximize ADG and G:F as well as to minimize SUN levels were 0.16, 0.17, and 0.16 using a linear-breakpoint model and 0.20, 0.20, and 0.20 using a quadratic model. Tryptophan could influence serum GH and IGF-1 secretion and protein abundance of the muscular AA transporter of SNAT2 in the longissimus dorsi muscle in late finishing gilts fed low-protein diets.

Targeting specific nutrient deficiencies in protein-restricted diets: some practical facts in PKU dietary management.

Among aminoacidopathies, phenylketonuria (PKU) is the most prevalent one. Early diagnosis in the neonatal period with a prompt nutritional therapy (low natural-protein and phenylalanine diet, supplemented with phenylalanine-free amino acid mixtures and special low-protein foods) remains the mainstay of the treatment. Data considering nutrient contents of cooked dishes is lacking. In this study, fourteen dishes specifically prepared for PKU individuals were analysed, regarding the lipid profile and iron and zinc contents. These dishes are poor sources of essential nutrients like Fe, Zn or n-3 fatty acids, reinforcing the need for adequate supplementation to cover individual patients' needs. This study can contribute to a more accurate adjustment of PKU diets and supplementation in order to prevent eventual nutritional deficiencies. This study contributes to a better understanding of nutrient intake from PKU patients' meals, showing the need for dietary supplementation.

How close do gastroenterologists follow specific guidelines for nutrition recommendations in liver cirrhosis? A survey of current practice.

AIM: Malnutrition is a common feature of terminal chronic liver disease. In Great Britain earlier studies revealed a widespread use of inappropriate dietary management for example, protein restriction in patients with liver cirrhosis (LC). Therefore, it was the aim of this study to evaluate the current dietary recommendations for patients with LC, recommended by gastroenterologists in Germany. METHODS: Anonymous questionnaires were sent to 576 members of the Bavarian Society of Gastroenterology. Information was requested on the dietary management and on the diagnostic assessment of nutritional status of all patients with LC seen in the past 12 months. Further questions were included concerning knowledge of recent guidelines on enteral nutrition (EN) in LC, use of an adequate EN and estimated prevalence of malnutrition. RESULTS: Forty-four percent of all questionnaires were returned within 4 weeks; of those 94% were fully completed and appropriate for further analysis. Fifty-six percent respondents stated that they were familiar with guidelines concerning EN in patients with LC and 92% believed that evidence-based recommendations are both important and relevant for everyday practice. Only 23% of the respondents gave a correct estimate of the prevalence of protein-calorie malnutrition in patients with chronic liver disease. The majority underestimated the correct amount of energy and protein intake that is recommended by the European Society for Parenteral and Enteral Nutrition guidelines on EN for patients with LC. Only 42% respondents recommended a protein-rich diet whereas most of the respondents under or overestimated the required daily energy (55%) and protein intake (58%). As simple bedside methods such as the subjective global assessment or anthropometry are considered adequate to identify malnutrition in LC, these methods were familiar only to 24 and 55% of the respondents, respectively. Forty-one percent, however, believed that an evaluation of the body mass index represents the best diagnostic tool to detect malnourished patients although body mass index may be misleading in cirrhotics with tense ascites. CONCLUSION: The dietary management of German cirrhotic patients should be improved, especially concerning the required daily energy and protein intake. Simple bedside methods for the diagnosis of malnutrition are widely unknown. In general, malnutrition in LC clearly represents a widely underestimated problem even in a highly specialized sample of medical practitioners in digestive and hepatological diseases. At the same time the number of nutrition support teams in German speaking countries are very low. A higher number of multidisciplinary teams including dietitians, psychologists and physical activity supervisors caring for undernourished patients might be an important step for an improvement towards correct management of malnutrition in LC.

Dietary and nutritional indications in hepatic encephalopathy.

The restriction of dietary protein has long been considered a main stay in the therapy of hepatic encephalopathy. More recently it has been recognized that protein energy malnutrition is frequent in advanced liver disease and may adversely affect the patients'outcome. Moreover studies on inter-organ ammonia exchange in liver cirrhosis have shown that the muscle may have a crucial role in ammonia detoxification. In light of these evidences nutritional guidelines have proposed that protein restriction should be avoided in patients with hepatic encephalopathy as protein requirement is even increased in cirrhotic patients. Survey about the current clinical practice show that protein restriction is still considered advisable in patients with hepatic encephalopathy, however a recent trial evidenced that a low protein diet in patients hospitalized for acute hepatic encephalopathy exacerbates protein breakdown without inducing any specific clinical benefit when compared to a normal protein regimen. The relevance of an adequate protein intake and possible strategies to implement protein tolerance are also discussed.

[Determination of factors conditioning adherence and accomplishment of renal protection diet in patients with chronic renal failure: pilot study for the elaboration of a dietary guideline]. / Determinación de los factores condicionantes en la adhesión y cumplimiento de la dieta de protección renal en pacientes con insuficiencia renal crónica: estudio previo para la elaboración de una guía dietética.

There are seldom studies on dietary behavior of patients with chronic renal failure (CRF). The aim of this study has been to know, by means of a previously validated questionnaire, which are the psycho-sociocultural factors that affect, and to what extent, assumption and adherence the dietary therapy while determining the degree of disease perception and several factors related with it. The study population is comprised by 81 patients from the nephrology clinic of the "12 de Octubre" Hospital of Madrid, with CRF in a pre-dialysis status. Seventy-seven point seventy-four percent answered "well" or "very well" to questions relating to disease knowledge and perception. Emotional and auto-management factors have little relevance according to 69.87% of patients. Fifty-nine point twenty-six percent feel a high level of familial support, and 35.77% alters dietary behavior when environmental conditions change. Most of the interviewees (87.65%) do not have difficulties finding the prescribed foods, and 70.37% considers their cost is not excessive. For almost half of the patients (48.76%), renal protection diet represents a variation in their dietary habits, a similar percentage expresses difficulty with elaboration. Food palatability is not a problem in 67.90% of the cases. Fifty-one point twenty-four percent does not perceive difficulty with cooking procedures. Seventy point ninety-nine percent feels support in one way or the other, by health care staff, although just 56.79% reports that the diet has not been explained to them. Only 18.51% questions the diet effectiveness as regards to their disease course. As for the gender variable, there were significant differences (p < 0.05), with a higher influence on men, in sections relating to disease knowledge, and influence of apathy and family support, the women those having the highest scores for food management, diet transgression at family meetings, and less information received about the prescribed diet. As for family support, there are significant differences only by age groups, patients aged more than 65 years being those feeling more this psychological support. The group of patients with a creatinine clearance less than 25 mL/min is the one expressing less categorically their appreciation on diet effectiveness. There are factors in which significant difficulty percentages are observed and that may induce diet transgression, in some cases without the patient being completely aware of, such as environmental changes, change in traditional habits, the degree of diet explanation, the organoleptic characteristics, and the lack of knowledge of appropriate cooking procedures. All these indicators confirm the need for enhancing nutritional education of these patients and their family environment, also showing the need for a nutritional intervention that completely supports patients in the process of adaptation and maintenance of their new dietary habit.

Determinacion de los factores condicionantes en la adhesion y cumplimiento de la dieta de protección renal en pacientes con insuficiencia renal crónica: estudio previo para la elaboración de una guía dietética / Determination of factors conditioning adherence and accomplishment of renal protection diet in patients with chronic renal failure: pilot study for the elaboration of a dietary guideline

Son escasos los estudios sobre el comportamiento alimentario en los pacientes con Insuficiencia Renal Crónica (IRC). El objetivo de este estudio, a través de una encuesta previamente validada, ha sido conocer cuales son los factores psico-socio-culturales, y en que grado inciden a la hora de asumir y cumplir el tratamiento dietoterápico, determinando, además, el grado de percepción de la enfermedad y de los diversos factores relacionados con ella. La población estudiada estaba formada por 81 pacientes de la consulta de nefrología del hospital "12 de Octubre" de Madrid, con IRC en prediálisis. A las preguntas relacionadas con el autoconocimiento y percepción de la enfermedad el 77,74% respondió dentro de los niveles "bien o muy bien". Los factores emocionales y de autocontrol tienen escasa relevancia, según afirma el 69,87% de los pacientes. El 59,26% siente en alto grado el apoyo familiar y el 35,77% altera el seguimiento dietético cuando cambian las condiciones medioambientales. La gran mayoría de los entrevistados, un 87,65%, no tienen dificultad en encontrar los alimentos pautados y para el 70,37% de la población su coste no es excesivo. Para casi la mitad de los pacientes (48,76%) la dieta de protección renal supone una variación de sus hábitos alimentarios, un porcentaje similar expresa, además, dificultad con la preparación. La palatabilidad de los alimentos no es un problema en el 67,90% de los casos. El 51,24% no percibe dificultad en los procesos culinarios. El 70,99% se siente apoyado, deuna u otra forma, por el personal sanitario, aunque sólo un 56,79% refiere que no le ha sido explicada la dieta. Únicamente el 18,51% cuestiona la efectividad de la dieta en relación con la evolución de su enfermedad. Dentro de la variable sexo, se observaron diferencias significativas (p < 0,05) con valores de influencia mayor en los hombres para los apartados relacionados con el conocimiento de la enfermedad, la influencia de la apatía y el apoyo familiar, siendo las mujeres las que obtenían valores más altos en cuanto al manejo de los alimentos, la trasgresión en reuniones familiares y una menor información recibida sobre la dieta a seguir. En cuanto al apoyo familiar, solamente se observan diferencias por grupos de edad, siendo los mayores de 65 años los que sienten más este soporte psicológico. El grupo de pacientes con un aclaramiento inferior a 25 ml/min, es el que expresa menor rotundidad en su apreciación de la efectividad de la dieta. Hay factores en los que se observan porcentajes significativos de dificultad, que pueden inducir a la trasgresión de la dieta, en algunos casos sin plena conciencia del paciente, tales como los cambios ambientales, la alteración de los hábitos tradicionales, el grado de explicación de la dieta, las características organolépticas y el desconocimiento de los tratamientos culinarios adecuados. Todos estos indicadores confirman la necesidad de profundizar en la educación nutricional en estos pacientes y en su entorno familiar, además de evidenciar la necesidad de una intervención nutricional que apoye integralmente al paciente en su proceso de adaptación y mantenimiento del nuevo hábito alimentario (AU)

There are seldom studies on dietary behavior of patients with chronic renal failure (CRF). The aim of this study has been to know, by means of a previously validated questionnaire, which are the psycho-sociocultural factors that affect, and to what extent, assumption and adherence the dietary therapy while determining the degree of disease perception and several factors related with it. The study population is comprised by 81 patients from the nephrology clinic of the "12 de Octubre" Hospital of Madrid, with CRF in a pre-dialysis status. Seventy-seven point seventy-four percent answered "well" or "very well" to questions relating to disease knowledge and perception. Emotional and auto-management factors have little relevance according to 69.87% of patients. Fifty-nine point twenty-six percent feel a high level of familial support, and 35.77% alters dietary behavior when environmental conditions change. Most of the interviewees (87.65%) do not have difficulties finding the prescribed foods, and 70.37% considers their cost is not excessive. For almost half of the patients (48.76%), renal protection diet represents a variation in their dietary habits, a similar percentage expresses difficulty with elaboration. Food palatability is not a problem in 67.90% of the cases. Fifty-one point twenty-four percent does not perceive difficulty with cooking procedures.Seventy point ninety-nine percent feels support in one way or the other, by health care staff, although just 56.79% reports that the diet has not been explained to them. Only 18.51% questions the diet effectiveness as regards to their disease course. As for the gender variable, there were significant differences (p < 0.05), with a higher influence on men, in sections relating to disease knowledge, and influence of apathy and family support, the women those having the highest scores for food management, diet transgression at family meetings, and less information received about the prescribed diet. As for family support, there are significant differences only by age groups, patients aged more than 65 years being those feeling more this psychological support. The group of patients with a creatinine clearance less than 25 mL/min is the one expressing less categorically their appreciation on diet effectiveness. There are factors in which significant difficulty percentages are observed and that may induce diet transgression,in some cases without the patient being completely aware of, such as environmental changes, change in traditional habits, the degree of diet explanation, the organoleptic characteristics, and the lack of knowledge of appropriate cooking procedures. All these indicators confirm the need for enhancing nutritional education of these patients and their family environment, also showing the need for a nutritional intervention that completely supports patients in the process of adaptation and maintenance of their new dietary habit (AU)

Conditional mixed models adjusting for non-ignorable drop-out with administrative censoring in longitudinal studies.

In this paper, a class of conditional mixed models is proposed to adjust for non-ignorable drop-out, while also accommodating unequal follow-up due to staggered entry and administrative censoring in longitudinal studies. Conditional linear and quadratic models which model subject-specific slopes as linear or quadratic functions of the time-to-drop-out, as well as pattern mixture models are both special cases of this approach. We illustrate these models and compare them with the usual maximum likelihood approach assuming ignorable drop-out using data from a multi-centre randomized clinical trial of renal disease. Simulations under various scenarios where the drop-out mechanism is ignorable and non-ignorable are employed to evaluate the performance of these models.

Body composition of patients on a very low-protein diet: a two-year survey with DEXA.

BACKGROUND: It has been reported that patients on a very-low-protein diet (VLPD) maintain a satisfactory nutritional status because of a conserved adaptive metabolic response. However, only few studies have examined the course of nutritional status and body composition in the long term (2 years). METHODS: Thirteen stable patients (8 men; age, 55 +/- 12 years; glomerular filtration rate (GFR), 15 +/- 5 mL/min) receiving a VLPD (0.3 g/kg/day protein) supplemented with amino acids and ketoanalogues (SVLPD) were studied for 2 years. A joint visit with a physician and a dietitian and routine blood and urine analyses were performed every month. Dual-energy x-ray absorptiometry (DEXA), which was used to assess modification of body composition, and GFR (urinary 51Cr-EDTA) and urinary urea and creatinine excretion, which were used to assess nutritional status and compliance to the diet, were assessed every 3 months. RESULTS: GFR, albumin, and prealbumin levels remained stable. Urea urinary excretion decreased at 3 months and then slightly increased at 2 years, but the calculated protein intake remained low at 0.38 +/- 0.1 g/kg/day. Energy intake remained close to 30 kcal/kg/day. No significant change was observed for total fat mass or percent fat mass. After an initial decrease, lean body mass stabilized at 6 months and then increased significantly from 6 to 24 months (P =.02, paired t-test); the mean increase during this period was of 2 kg, that is, 4.6%. Urinary creatinine excretion showed the same profile. Total bone mass, lumbar or hip site bone mass, and Z-score significantly decreased from T0 to 1 and 2 years (P <.05). CONCLUSION: This study confirms that a supplemented VLPD is nutritionally safe for a long period, but attention must be paid to bone mass.

[Celiac disease: evaluation of compliance to gluten-free diet and knowledge of disease in patients registered at the Brazilian Celiac Association (ACA)]. / Doença celíaca: avaliação da obediência à dieta isenta de glúten e do conhecimento da doença pelos pacientes cadastrados na Associação dos Celíacos do Brasil (ACELBRA).

BACKGROUND: The compliance to a gluten-free diet may prevent the development of both non-malignant and malignant complications. AIM: To evaluate compliance to a gluten-free diet and knowledge of the disease in celiac patients registered at the Brazilian Celiac Association (BCA). METHODS: A structured questionnaire was designed to assess compliance to a gluten-free diet as well as knowledge of the celiac disease. It was mailed to 584 members of BCA. RESULTS: Five hundred and twenty nine (90.6%) of a total of 534 (91.4%) answered questionnaires were analyzed; 69.4% were classified as compliant patients whereas 29.5% were classified as noncompliant. The proportion of patients age 21 or older who consume gluten frequently or without any restriction is larger (17.7%) than those who were younger than 21 years (9.9%). Frequency of dietary compliance was higher when the diagnosis had taken less than 5 years to be established; 82% of the patients replied that the small intestine was the part of the body affected by the disease. The most common symptoms of the disease according to the answers were diarrhea (96.6%), weight loss (93.4%), protuberant abdomen (90.4%), anemia (68.1%) and vomiting (59.6%). Only 59.0% agreed with the existence of genetic predisposition; 90.4% answered that the disease is permanent and 96.2% stated that the diet should exclude gluten absolutely; 67.1% answered that the gluten is a protein and according to 92.1% questionnaires this protein is present in wheat, rye, barley and oat. Greater compliance was observed when there was an understanding of the disease and diet. The small intestine biopsy was considered necessary for just 67.5% of the patients, and greater compliance was observed in patients who had undergone at least one small intestine biopsy. CONCLUSION: Our findings indicate that the more the patients know and understand about the disease, the better able they are to comply with the diet.

Wheat starch, gliadin, and the gluten-free diet.

Individuals with celiac disease generally are advised to follow a lifelong gluten-free diet and avoid consumption of the prolamins gliadin (wheat), secalin (rye), and hordein (barley). Although the designation of the diet as glutenfree may imply that the diet contains zero gluten, this is not necessarily true. In some countries (eg, United States, Canada), the gluten-free diet is completely devoid of gluten and is based on foods such as rice and corn that are naturally gluten free. In others (eg, Scandinavia, United Kingdom), the gluten-free diet may include foods such as wheat starch that have been rendered gluten free but nonetheless contain small amounts of toxic prolamins. The discrepancy in the use of foods rendered gluten free exists because the amount of toxic prolamins that individuals with celiac disease may consume without damaging the mucosa of the small intestine is unknown. Minimal research has been conducted on the toxicity of foods rendered gluten free, and there are no definitive data about whether the small amount of prolamin found in these products is safe to consume. Nonetheless, the Codex Alimentarius Standard for gluten-free foods allows a certain amount of prolamin in foods designated gluten free, and these products have been used in many countries for several decades. Well-designed, scientifically sound studies are needed to help determine the amount of toxic prolamins, if any, that may be safely consumed by individuals with celiac disease. Until this research is conducted, dietitians in the United States should continue to advise their patients against the use of wheat starch and other foods rendered gluten free.