scholarly journals Nutrient Intake and Nutritional Status in Adult Patients with Inherited Metabolic Diseases Treated with Low-Protein Diets: A Review on Urea Cycle Disorders and Branched Chain Organic Acidemias

Nutrients ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3331
Author(s):  
Francesco Francini-Pesenti ◽  
Giorgia Gugelmo ◽  
Livia Lenzini ◽  
Nicola Vitturi
Keyword(s):  
Nutritional Status ◽  
Protein Intake ◽  
Nutrient Intake ◽  
Urea Cycle ◽  
Adult Patients ◽  
Organic Acidemias ◽  
Low Protein ◽  
Branched Chain ◽  
Cycle Disorders ◽  
Protein Diets

Low-protein diets (LPDs) are the main treatment for urea cycle disorders (UCDs) and organic acidemias (OAs). In most cases, LPDs start in childhood and must be continued into adulthood. The improved life expectancy of patients with UCDs and OAs raises the question of their consequences on nutritional status in adult subjects. As this topic has so far received little attention, we conducted a review of scientific studies that investigated the nutrient intake and nutritional status in adult patients with UCDs and branched chain organic acidemias (BCOAs) on LPD. Methods: The literature search was conducted in PubMed/MEDLINE, Scopus, EMBASE and Google Scholar from 1 January 2000 to 31 May 2020, focusing on nutrient intake and nutritional status in UCD and OA adult patients. Results: Despite protein restriction is recommended as the main treatment for UCDs and OAs, in these patients, protein intake ranges widely, with many patients who do not reach safety levels. When evaluated, micronutrient intake resulted below recommended values in some patients. Lean body mass resulted in most cases lower than normal range while fat body mass (FM) was often found normal or higher than the controls or reference values. Protein intake correlated inversely with FM both in adult and pediatric UCD patients. Conclusions: The clinical management of adult patients with UCDs and BCOAs should include an accurate assessment of the nutritional status and body composition. However, as little data is still available on this topic, further studies are needed to better clarify the effects of LPDs on nutritional status in adult UCD and BCOA patients.

The Effects of Low-Protein Diet and Uraemia upon Urea-Cycle Enzymes and Transaminases in Rats

1972 ◽  
Vol 43 (3) ◽  
pp. 371-376 ◽  
Author(s):  
C. L. Brown ◽  
B. J. Houghton ◽  
R. L. Souhami ◽  
P. Richards
Keyword(s):  
Protein Intake ◽  
Urea Cycle ◽  
Essential Amino Acids ◽  
Normal Diet ◽  
Protein Restriction ◽  
Protein Diet ◽  
Low Protein Diet ◽  
Low Protein ◽  
Branched Chain Amino Acid ◽  
Branched Chain

1. Activities of arginine synthetase system enzymes, alanine aminotransferase (AlT) and branched-chain amino acid transaminase (BATase) were measured in control and uraemic rats after 2 weeks on a free protein intake and after 10 days on a low-protein diet. 2. Protein restriction alone reduced the activity of all three enzymes. 3. Uraemia alone increased the activity of arginine synthetase system enzymes and AlT. BATase was not altered. 4. After protein restriction the BATase activity in uraemic rats decreased to the same extent as in controls. The activity of arginine synthetase system enzymes and AlT were significantly higher than in protein-restricted controls and were the same as in control rats on a normal diet. 5. Practical consequences of the finding that uraemia prevented the decrease of AlT but not of BATase activity in response to a low-protein diet are discussed, with particular reference to reutilization of urea-nitrogen for the synthesis of non-essential and essential amino acids.

scholarly journals Nutritional status, hyperkalaemia and attainment of energy/protein intake targets in haemodialysis patients following plant-based diets: a longitudinal cohort study

2020 ◽  
Author(s):  
Ailema González-Ortiz ◽  
Hong Xu ◽  
Samuel Ramos-Acevedo ◽  
Carla M Avesani ◽  
Bengt Lindholm ◽  
...  
Keyword(s):  
Nutritional Status ◽  
Energy Intake ◽  
Serum Potassium ◽  
Protein Intake ◽  
Fruits And Vegetables ◽  
Dietary Assessment ◽  
Healthy Plant ◽  
Low Protein ◽  
Single Centre Study ◽  
Current Guidelines

Abstract Background Patients undergoing haemodialysis (HD) are often discouraged from eating fruits and vegetables because of fears of hyperkalaemia and undernutrition, yet evidence to support these claims is scarce. We here explore the association between adherence to a healthy plant-based diet with serum potassium, surrogates of nutritional status and attainment of energy/protein intake targets in HD patients. Methods We performed an observational single-centre study of stable patients undergoing HD with repeated dietary assessment every 3 months. Patients were provided with personalized nutritional counselling according to current guidelines. The diet was evaluated by 3-day food records and characterized by a healthy plant-based diet score (HPDS), which scores positively the intake of plant foods and negatively animal foods and sugar. The malnutrition inflammation score (MIS) and serum potassium were also assessed at each visit. We used mixed-effects models to evaluate the association of the HPDS with markers of nutritional status, serum potassium levels and attainment of energy/protein intake targets. Results After applying inclusion and exclusion criteria, a total of 150 patients contributing to 470 trimestral observations were included. Their mean age was 42 years [standard deviation (SD) 18] and 59% were women. In multivariable models, a higher HPDS was not associated with serum potassium levels or odds of hyperkalaemia {potassium >5.5 mEq/L; odds ratio [OR] 1.00 [95% confidence interval (CI) 0.94–1.07] per HPDS unit higher}. Patients with a higher HPDS did not differ in terms of energy intake [OR for consuming <30 kcal/kg day 1.05 (95% CI 0.97–1.13)] but were at risk of low protein intake [OR for consuming <1.1 g of protein/kg/day 1.11 (95% CI 1.04–1.19)]. A higher HPDS was associated with a lower MIS, indicating better nutritional status. Conclusions In patients undergoing HD, adherence to a healthy plant-based diet was not associated with serum potassium, hyperkalaemia or differences in energy intake. Although these patients were less likely to reach daily protein intake targets, they appeared to associate with better nutritional status over time.

scholarly journals Activation of rat liver branched-chain 2-oxo acid dehydrogenase in vivo by glucagon and adrenaline

1985 ◽  
Vol 232 (2) ◽  
pp. 593-597 ◽  
Author(s):  
K P Block ◽  
B W Heywood ◽  
M G Buse ◽  
A E Harper
Keyword(s):  
Cyclic Amp ◽  
Fold Increase ◽  
Amino Acid Uptake ◽  
Acid Uptake ◽  
Acid Dehydrogenase ◽  
Low Protein ◽  
Branched Chain ◽  
Protein Diets ◽  
In Vivo Administration

The activity of liver branched-chain 2-oxo acid dehydrogenase complex was measured in rats fed on low-protein diets and given adrenaline, glucagon, insulin or dibutyryl cyclic AMP in vivo. Administration of glucagon or adrenaline (200 micrograms/100 g body wt.) resulted in a 4-fold increase in the percentage of active complex. As with glucagon and adrenaline, treatment of rats with cyclic AMP (5 mg/100 g body wt.) resulted in marked activation of branched-chain 2-oxo acid dehydrogenase. Insulin administration (1 unit/100 g body wt.) also resulted in activation of enzyme; however, these effects were less than those observed with glucagon and adrenaline. In contrast with the results obtained with low-protein-fed rats, administration of adrenaline (200 micrograms/100 g body wt.) to rats fed with an adequate amount of protein resulted in only a modest (14%) increase in the activity of the complex. The extent to which these hormones activate branched-chain 2-oxo acid dehydrogenase appears to be correlated with their ability to stimulate amino acid uptake into liver.

scholarly journals Heifer nutrient intake during early- and mid-gestation programs adult offspring adiposity and mRNA expression of growth-related genes in adipose depots

Reproduction ◽  
2011 ◽  
Vol 141 (5) ◽  
pp. 697-706 ◽  
Author(s):  
Gina C Micke ◽  
Tracy M Sullivan ◽  
I Caroline McMillen ◽  
Sheridan Gentili ◽  
Vivienne E A Perry
Keyword(s):  
Adipose Tissue ◽  
Mrna Expression ◽  
Nutrient Intake ◽  
Adult Life ◽  
High Protein ◽  
Second Trimester ◽  
Low Protein ◽  
High Protein Diets ◽  
Protein Diets ◽  
Offspring Adiposity

Changes in maternal nutrient intake during gestation alterIGFreceptor abundance and leptin (LEP) mRNA expression in fetal adipose tissue. It is not known whether such changes persist into adult life and whether they are associated with an effect on phenotype. We investigated the effect of high (240%) and low (70%) levels of recommended daily crude protein intake for beef heifers during the first and second trimesters of gestation on singleton progeny (n=68): subcutaneous (SC) adipose tissue depth at rump (P8) and rib (RF) sites from 65 until 657 days of age; plasma leptin concentrations from birth until 657 days and expression ofIGF1andIGF2, their receptors (IGF1RandIGF2R) andLEPmRNA in perirenal (PR), omental (OM) and SC adipose tissue at 680 days of age. High-protein diets during the first trimester increasedLEPandIGF1mRNA in PR of males and females, respectively, compared with low-protein diets, and decreasedIGF1RmRNA in SC of all progeny but increased RF depth of males between 552 and 657 days. High-protein diets compared with low-protein diets during the second trimester increasedIGF1RmRNA in PR and OM of all progeny;LEPmRNA in PR of males; andIGF2andIGF2RmRNA in OM of all progeny. Conversely,LEPmRNA in OM andIGF2mRNA in PR of all progeny were decreased following exposure to high- compared with low-protein diets during the second trimester. Heifer diet during gestation has permanent sex- and depot-specific effects on the expression of adipogenic and adipocytokine genes and offspring adiposity.

scholarly journals Sodium phenylbutyrate decreases plasma branched-chain amino acids in patients with urea cycle disorders

2014 ◽  
Vol 113 (1-2) ◽  
pp. 131-135 ◽  
Author(s):  
Lindsay C. Burrage ◽  
Mahim Jain ◽  
Laura Gandolfo ◽  
Brendan H. Lee ◽  
Sandesh C.S. Nagamani
Keyword(s):  
Amino Acids ◽  
Urea Cycle ◽  
Branched Chain Amino Acids ◽  
Urea Cycle Disorders ◽  
Branched Chain ◽  
Cycle Disorders ◽  
Sodium Phenylbutyrate

scholarly journals Overview of Macro Nutrient Intake and Nutritional Status of Students at the Putri Hidayatullah Islamic Boarding School, Ternate City

2021 ◽  
Vol 9 (12) ◽  
pp. 13-21
Author(s):  
Hisdawati Abubakar ◽  
Yohannes Willi Helm ◽  
Nofiandri ◽  
Fadila
Keyword(s):  
Nutritional Status ◽  
Protein Intake ◽  
Nutrient Intake ◽  
Sampling Technique ◽  
Boarding School ◽  
Carbohydrate Intake ◽  
Physical Growth ◽  
Vulnerable Groups ◽  
Second Phase ◽  
Fat Intake

Adolescents are one of the nutritionally vulnerable groups. At this time, adolescents enter the second phase of rapid growth and then physical growth declines as young adults enter. Problems that arise in adolescents are undernutrition and overnutrition. One that is related to nutritional status is nutrient intake which is very influential on a person's nutritional status. Nutrient intake is influenced by eating habits and eating patterns of adolescents themselves. The purpose of this study was to determine the description of macronutrient intake and nutritional status at the Putri Hidayatullah Islamic Boarding School, Ternate City. The type of research used is descriptive research. The sampling technique used total sampling with a total sample of 29 people. The tools used were Microtoise, Bathroom Scale and form food recall 24. The results showed that the intake of macronutrients, as many as 28 people (96.6%) with less energy intake, 29 people (100.0%) with less protein intake, 16 people (55.2%) with more fat intake and 26 people (89.7%) with less carbohydrate intake. There were 25 people (86.2%) with normal nutritional status. It was concluded that from the results of the respondents had less protein intake and most had less energy and carbohydrate intake. On the other hand, most of these teenagers have good fat intake with normal nutritional status.

Diet and the progression of chronic kidney disease

2015 ◽  
Author(s):  
Juan Jesús Carrero ◽  
Hong Xu ◽  
Bengt Lindholm
Keyword(s):  
Blood Pressure ◽  
Protein Intake ◽  
Salt Intake ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
High Serum ◽  
Nutritional Epidemiology ◽  
Converting Enzyme Inhibitors ◽  
Early Stages ◽  
Low Protein ◽  
Protein Diets

The dietary management of non-dialysed CKD patients has focused on limiting the intake of substances which lead to accumulation of urea, potassium, phosphorus, and sodium. Recent advances in nutritional epidemiology have given us the opportunity to examine the relationships between diet and CKD. This chapter focuses on evidence relating to retarding progression of renal impairment in the early to mid stages of CKD. Limits may need to change if GFR falls. The hypothesis that a high dietary protein intake leads to progressive CKD through a mechanism of glomerular hyperfiltration has been taught for decades, and it appears effective in animals. However, the evidence that low-protein diets (LPDs) halt CKD progression in patients is weak. Their management is of course likely to include other interventions such as blood pressure control. There is risk to low-protein diets. There is some evidence that high protein intakes are harmful. We therefore recommend moderate protein intake (not low; not high – no protein supplements; around 1g/kg/day). Salt handling is impaired in most patients with CKD, probably even early stages, and hypertension is an early feature, except in salt-losing patients, to whom different rules apply. Salt intake tends to raise blood pressure, worsen proteinuria, and reduce the effects of angiotensin converting enzyme inhibitors on blood pressure and proteinuria. Very low salt intakes are difficult to comply with and limit diet. In early stages of CKD we therefore recommend restriction to moderately low levels (below 6g/day of salt; 100 mmol of sodium). Lower levels may have additional benefits, and these limits may need to be reduced as GFR declines. Potassium is associated with healthy, desirable foods such as fruit and vegetables. It should only be restricted if high serum values make this necessary.

Corrigendum to: IGF-I is not a useful marker for nutritional status in the growing pig under commercial conditions

2001 ◽  
Vol 52 (7) ◽  
pp. 791
Author(s):  
L. Ma ◽  
F. R. Dunshea ◽  
Y. M. Brockwell ◽  
R. L. Inglis ◽  
D. J. Kingston ◽  
...  
Keyword(s):  
Weight Gain ◽  
Nutritional Status ◽  
Dietary Protein ◽  
High Protein Diet ◽  
High Protein ◽  
Protein Diet ◽  
Low Protein ◽  
Igf I ◽  
Protein Diets

Plasma hormone concentrations were measured in gilts after fasting, long-term protein restriction, or supplementation. In 11-week-old pigs fasted overnight, plasma insulin, glucagon, gastrin, urea, and glucose were increased 30 min after re-feeding (P < 0.05), whereas IGF-I did not change. In 16-week-old gilts fed a standard commercial diet [14.6% crude protein (CP)], or a high-protein diet (16.7% CP) for 4 weeks, the high-protein diet increased weight gain (13%; P < 0.05) and carcass weight (4%; P < 0.05), but did not alter plasma IGF-I, insulin, or glucagon. In 10-week-old gilts fed high-protein diets (19.4% and 18.3% CP), or low-protein diets (15.5% and 13.3% CP) for 12 weeks during the grower and finisher phases, respectively, the low-protein diet decreased weight gain (18%; P < 0.001) and carcass weight (11%; P < 0.01), with a marked increase in plasma glucagon (P < 0.05), no change in insulin, and only a trend towards decreased IGF-I (P = 0.1). The pigs were more sensitive to altered dietary protein at 10 weeks of age than at 16 weeks. Plasma IGF-I was not responsive to the short-term effects of feeding or the long-term effects of dietary protein. Glucagon could provide a useful marker for nutritional status in young pigs, provided that time of feeding is taken into account.

Dyspepsia is Associated with Low Protein and Caloric Intake among End-Stage Renal Disease Patients

2015 ◽  
Vol 85 (3-4) ◽  
pp. 112-118 ◽  
Author(s):  
Paulo Roberto Santos ◽  
Diego Levi Silveira Monteiro ◽  
Paulo Henrique Alexandre de Paula ◽  
Vicente Lopes Monte Neto ◽  
Maria Leilah Ponte Monte Coelho ◽  
...  
Keyword(s):  
Nutritional Status ◽  
Renal Disease ◽  
End Stage Renal Disease ◽  
Protein Intake ◽  
Calorie Intake ◽  
Low Protein ◽  
Dyspeptic Symptoms ◽  
Stage Renal Disease ◽  
End Stage ◽  
Esrd Patients

Abstract. Background: Dyspepsia is highly prevalent and easily assessed in end-stage renal disease (ESRD) patients undergoing hemodialysis (HD) and is not a traditional predictor of malnutrition. We sought to establish an association between dyspeptic symptoms and nutritional status in ESRD patients undergoing maintenance HD. Methods: We studied 106 ESRD patients on HD. Dyspepsia was assessed through the Porto Alegre Dyspeptic Symptoms Questionnaire (PADYQ). Scores equal to or greater than 6 classified patients as dyspeptic. Nutritional status was evaluated using serum chemistry, total body mass, muscle mass, and dietary intake. Nutritional status was compared between dyspeptic and non-dyspeptic patients. The association of PADYQ scores and the presence of dyspepsia with nutritional variables were tested. Multivariate analysis was performed to test dyspepsia as an independent predictor for dietary intake. Results: There were 41 (38.7 %) dyspeptics. Protein intake (g/kg/day) and calorie intake (kcal/kg/day) were lower among dyspeptics compared to non-dyspeptics, 1.0 ± 0.5 vs 1.3 ± 0.5 (p = 0.01) and 23.0 ± 9.2 vs 27.4 ± 10.0 (p = 0.02), respectively. More dyspeptics than non-dyspeptics presenting protein-energy wasting based on protein and calorie intake, 41.4 vs 15.3 % (p = 0.01) and 68.2 vs 38.4 % (p = 0.02), respectively. PAQYQ score was negatively correlated with protein intake (r = - 0.20; p = 0.03) and calorie intake (r = - 0.19; p = 0.04). Dyspepsia was able to predict protein (b = - 0.26; p = 0.01) and calorie (b = - 4.42; p = 0.02) intake. Conclusions: Dyspepsia is associated with low protein and calorie intake. Screening of dyspeptic symptoms can be routinely performed aiming to improve HD patients’ nutritional status.

Sign in / Sign up

Export Citation Format

Share Document