Search

Objectives Disorders of amino acid metabolism fall under the category of inborn errors of metabolism that can be managed with a protein-restricted diet. However, adherence to such a diet often poses challenges, leading to low treatment engagement. Consequently, there is a pressing need for new resources to aid in dietary self-monitoring. The goal is to develop and implement “AminoApp,” an application tailored for dietary self-monitoring in patients with inborn errors of metabolism who are on a low-protein diet. Methods The design and development of the application adhered to the user-centered design method. This approach emphasizes active participation and collaboration between users and designers/researchers throughout all stages of product development, including requirement gathering, prototype development, and evaluation. Usability was evaluated using the System Usability Scale, which has been validated in Portuguese. Results The application’s features include a food diary, a food consultation area, exam records, a recipe calculator, and reports on diet composition and metabolic control. The usability test included four patients on a low-protein diet, three caregivers, and three healthcare professionals. The average usability score was 84.9, with averages of 77.5 for patients, 85.8 for caregivers, and 91.6 for professionals, indicating that the application is user-friendly. Conclusions AminoApp is the first application developed in Brazil designed to assist in managing inborn errors of metabolism that require a protein-restricted diet. It was found to be easy to use, and the initial results are promising. Further research is necessary to evaluate the impact of the application on metabolic control and treatment adherence.

The current trial investigates the effect of renal diet therapy and nutritional education on the estimated glomerular filtration rate (eGFR), blood pressure (BP) and depression among patients with chronic kidney disease (CKD). A total of 120 CKD patients (stages 3–4) (15

Parkinson's Disease (PD) is a neurodegenerative disorder associated with diminished nutrition status and decreased quality of life. While the prevalence of PD is expected to increase, no preventative or curative therapy for PD exists at this time. Although nutrition and diet represent modifiable risk factors for reducing chronic disease risk, research on the impact of single nutrients on PD has yielded mixed results. As a result, this single-nutrient approach may be the driving force behind the inconsistency, and a holistic dietary approach may overcome this inconsistency by accounting for the interactions between nutrients. The following review aims to examine the impact of a generally healthy dietary pattern, the protein-restricted diet (PRD), the ketogenic diet (KD), the Mediterranean diet (MD), and the Mediterranean-DASH Intervention for Neurodegenerative Delay (MIND) diet on PD risk, progression, and severity. While most of the included studies support the role of diet and dietary patterns in reducing the risk of PD or alleviating PD severity, the inconsistent results and need for further evidence necessitate more research being conducted before making dietary recommendations. Research on the potential beneficial effects of dietary patterns on PD should also investigate potential risks. [ABSTRACT FROM AUTHOR]

Resistance to erythropoiesis-stimulating agents (ESA) in patients with renal anemia may be due to nutritional deficiencies affecting hematopoiesis. On the other hand, patients with advanced chronic kidney disease (CKD) generally consume a protein-restricted diet or a potassium-restricted diet. Therefore, we focused on the amount of nutrients, such as iron, zinc, copper, vitamin B6, vitamin B12, folic acid, vitamin C, and vitamin E, contained in various diets. We carried out a survey on the general diet, three protein-restricted diets, and two protein/potassium-restricted diets at a hospital in Japan. Then, based on each menu for five weeks, the amount of each nutrient contained in the daily diet was calculated. We found that the daily nutrient levels of iron, zinc, and vitamin B6 in all protein-restricted diets did not meet the estimated average requirement (EAR) for the Japanese population. Additionally, the vitamin C levels of protein/ potassium-restricted diets also did not meet the EAR for the Japanese population. These results suggest that patients on a diet that routinely limits their protein and/or potassium intake may be chronically deficient in the nutrients needed for hematopoiesis. Therefore, we think that pharmacists need to pay more attention to the diet and nutritional status of ESAresistant patients when managing their anemia, in our hospital. [ABSTRACT FROM AUTHOR]

Background: Chronic kidney disease (CKD) has challenged the healthcare system for years. Use of α-ketoanalogue (KA) supplemented with a low protein diet (LPD) may improve renal function. In this study, we aim to find out the effectiveness of KA supplemented with LPD for the therapy of CKD. Methods: A descriptive cross-sectional study was conducted at Shree Birendra Hospital with 25 control and 25 treatment group. The control group was treated with LPD (0.8 g/kg/day), while the test group was treated with KA (3 tablets/day for 4 weeks followed by 6 tablets/day for the next 16 weeks) along with LPD. The baseline parameters were measured on day 0, then subsequently at week 4, week 12, and lastly at the end of week 16. Results were then compared with the control group for analysis. Results: Serum level of creatinine showed a progressive decline in the treatment group in comparison to the control group. However, there was a progressive decline in the level of blood urea in the first 4 weeks (84.33) and 12 weeks (72.11) of treatment, a slight increment was observed in week 16 (86.06) but the level was below the baseline value. Similarly, the blood level of sodium and potassium was slightly affected by the increase in sodium and decrease in potassium level from the baseline in the treatment group. The level of serum albumin was increased in the treatment group as compared to the control group. The systolic blood pressure was increased in both treatment and control group whereas diastolic blood pressure was decreased in the treatment group rather than the control group. Conclusion: KA supplemented with LPD shows a significant improvement in renal function of CKD III patients that delay the time for dialysis or transplant. However, further substantial multi-institutional randomized studies are necessary to generalize the findings.

Background : A very low protein diet (VLPD) with ketoacid analogs of essential amino acids (KA/EAA) administration can remarkably influence protein synthesis and metabolic disturbances of patients with advanced chronic kidney disease (CKD), and may also slow the decline in renal function. Methods : A retrospective cohort study was carried out to monitor renal progression and metabolic and nutritional status among 140 patients with CKD stage III or IV. One group (n = 70) was on a low protein diet (LPD) with 0.6 g of protein intake, and another group (n = 70) was on a VLPD with 0.3 g of protein and KA/EAA supplementation of 100 mg/kg/day for 12 months. Results : At 12-month follow-up, estimated glomerular filtration rate (GFR) significantly decreased from 41.6 ± 10.2 to 36.4 ± 8.8 mL/min/1.73 m2 (P < 0.001) and urine protein increased from 0.6 ± 0.5 to 0.9 ± 1.1 g/day (P = 0.017) in the LPD group, but no significant changes in estimated GFR and urine protein were found in the VLPD plus KA/EAA group. A significant mean difference in rate of change in estimated GFR (−5.2 ± 3.6 mL/min/1.73 m2 per year; P < 0.001) was observed between the two groups. After Cox regression analysis, treatment with VLPD plus KA/EAA significantly protected against the incidence of declining GFR > 10% annually (adjusted hazard ratio, 0.42; 95% confidence interval, 0.23-0.79; P = 0.006) and significant correlations were found between using VLPD plus KA/EEA and increased GFR. Conclusion : VLPD supplementation with KA/EAA is associated with delayed renal progression while preserving the nutritional status in the patients with CKD. Co-administration of VLPD and KA/EAA may prove an effective alternative to conservative management of CKD.

Inherited amino-acid metabolism disorders (IAAMDs) require lifelong protein-restricted diet. We aimed to investigate: 1/ whether IAAMDs was associated with growth, pubertal, bone mineral apparent density (BMAD) or body composition impairments; 2/ associations linking height, amino-acid mixture (AAM), plasma amino-acids and IGF1 concentrations. Retrospective longitudinal study of 213 patients with neonatal-onset urea cycle disorders (UCD, n = 77), organic aciduria (OA, n = 89), maple syrup urine disease (MSUD, n = 34), or tyrosinaemia type 1 (n = 13). We collected growth parameters, pubertal status, BMAD, body composition, protein-intake, and IGF1 throughout growth. Overall final height (n = 69) was below target height (TH): −0.9(1.4) vs. -0.1(0.9) SD, p < 0.001. Final height was ≤ TH-2SD in 12 (21%) patients. Height ≤ − 2SD was more frequent during puberty than during early-infancy and pre-puberty: 23.5% vs. 6.9%, p = 0.002; and vs. 10.7%, p < 0.001. Pubertal delay was frequent (26.7%). Height (SD) was positively associated with isoleucine concentration: β, 0.008; 95%CI, 0.003 to 0.012; p = 0.001. In the pubertal subgroup, height (SD) was lower in patients with vs. without AAM supplementation: −1.22 (1.40) vs. -0.63 (1.46) (p = 0.02). In OA, height and median (IQR) isoleucine and valine concentrations(μmol/L) during puberty were lower in patients with vs. without AAM supplementation: −1.75 (1.30) vs. −0.33 (1.55) SD, p < 0.001; and 40 (23) vs. 60 (25) (p = 0.02) and 138 (92) vs. 191 (63) (p = 0.01), respectively. No correlation was found with IGF1. Lean-mass index was lower than fat-mass index: −2.03 (1.15) vs. -0.44 (0.89), p < 0.001. In IAAMDs, growth retardation worsened during puberty which was delayed in all disease subgroups. Height seems linked to the disease, AAM composition and lower isoleucine concentration, independently of the GH-IGF1 pathway. We recommend close monitoring of diet during puberty. • Growth and final height are impaired in inborn errors of protein metabolism with protein-restricted diet, notably in male. • Growth retardation worsened during puberty with no subsequent catch-up gains. • Delayed puberty is noted in a quarter of patients and was more common in males. • Dietary proteins are associated with growth retardation. Their composition differed according to aetiologies and age group. [ABSTRACT FROM AUTHOR]

Background: The dietary management of methylmalonic acidaemia (MMA) is a low-protein diet providing sufficient energy to avoid catabolism and to limit production of methylmalonic acid. The goal is to achieve normal growth, good nutritional status and the maintenance of metabolic stability. Aim: To describe the dietary management of patients with MMA across Europe. Methods: A cross-sectional questionnaire was sent to European colleagues managing inherited metabolic disorders (IMDs) (n=53) with 27 questions about the nutritional management of organic acidaemias. Data were analysed by different age ranges (0–6 months; 7–12 months; 1–10 years; 11–16 years; >16 years). Results: Questionnaires were returned from 53 centres. Twenty-five centres cared for 80 patients with MMA vitamin B12 responsive (MMAB12r) and 43 centres managed 215 patients with MMA vitamin B12 non-responsive (MMAB12nr). For MMAB12r patients, 44% of centres (n=11/25) prescribed natural protein below the World Health Organization/Food and Agriculture Organization/United Nations University (WHO/FAO/UNU) 2007 safe levels of protein intake in at least one age range. Precursor-free amino acids (PFAA) were prescribed by 40% of centres (10/25) caring for 36% (29/80) of all the patients. For MMAB12nr patients, 72% of centres (n=31/43) prescribed natural protein below the safe levels of protein intake (WHO/FAO/UNU 2007) in at least one age range. PFAA were prescribed by 77% of centres (n=33/43) managing 81% (n=174/215) of patients. In MMAB12nr patients, 90 (42%) required tube feeding: 25 via a nasogastric tube and 65 via a gastrostomy. Conclusions: A high percentage of centres used PFAA in MMA patients together with a protein prescription that provided less than the safe levels of natural protein intake. However, there was inconsistent practices across Europe. Long-term efficacy studies are needed to study patient outcome when using PFAA with different severities of natural protein restrictions in patients with MMA to guide future practice. [ABSTRACT FROM AUTHOR]

Intergenerational fitness effects on offspring due to the early life of the parent are well studied from the standpoint of the maternal environment, but intergenerational effects owing to the paternal early life environment are often overlooked. Nonetheless, recent laboratory studies in mammals and ecologically relevant studies in invertebrates predict that paternal effects can have a major impact on the offspring's phenotype. These nongenetic, environment‐dependent paternal effects provide a mechanism for fathers to transmit environmental information to their offspring and could allow rapid adaptation. We used the bank vole Myodes glareolus, a wild rodent species with no paternal care, to test the hypothesis that a high population density environment in the early life of fathers can affect traits associated with offspring fitness. We show that the protein content in the diet and/or social environment experienced during the father's early life (prenatal and weaning) influence the phenotype and survival of his offspring and may indicate adaptation to density‐dependent costs. Furthermore, we show that experiencing multiple environmental factors during the paternal early life can lead to a different outcome on the offspring phenotype than stimulated by experience of a single environmental factor, highlighting the need to study developmental experiences in tandem rather than independent of each other. [ABSTRACT FROM AUTHOR]

This experiment was conducted to investigate the effects of branched-chain amino acids (BCAA) supplemented in protein-restricted diets on the growth performance and the expression profile of amino acid transporters and energy metabolism related regulators in the white adipose tissue (WAT) of different regional depots including dorsal subcutaneous adipose (DSA) and abdominal subcutaneous adipose (ASA). A total of 24 crossbred barrows (7.40 ± 0.70 kg) were randomly divided into 4 groups and were fed the following isocaloric diets for 33 days: 1) a recommended adequate protein diet (AP, 20% CP, as a positive control); 2) a low protein diet (LP, 17% CP); 3) the LP diet supplemented with BCAA (LP + B, 17% CP) to reach the same level of the AP diet group; 4) the LP diet supplemented with 2 times the amount of BCAA (LP + 2B, 17% CP). The daily gain and daily feed intake of the LP diet group were the lowest among all the treatments (P 0.05). Moreover, BCAA supplementation down-regulated the expression levels of amino acid transporters including L-type amino acid transporter 1 and sodium-coupled neutral amino acid transporter 2 in DSA, but up-regulated the expression level of L-type amino acid transporter 4 in ASA (P

Background: A very low protein diet (VLPD) with ketoacid analogs of essential amino acids (KA/EAA) administration can remarkably influence protein synthesis and metabolic disturbances of patients with advanced chronic kidney disease (CKD), and may also slow the decline in renal function. Methods: A retrospective cohort study was carried out to monitor renal progression and metabolic and nutritional status among 140 patients with CKD stage III or IV. One group (n = 70) was on a low protein diet (LPD) with 0.6 g of protein intake, and another group (n = 70) was on a VLPD with 0.3 g of protein and KA/EAA supplementation of 100 mg/kg/day for 12 months. Results: At 12-month follow-up, estimated glomerular filtration rate (GFR) significantly decreased from 41.6 ± 10.2 to 36.4 ± 8.8 mL/min/1.73 m2 (P < 0.001) and urine protein increased from 0.6 ± 0.5 to 0.9 ± 1.1 g/day (P = 0.017) in the LPD group, but no significant changes in estimated GFR and urine protein were found in the VLPD plus KA/EAA group. A significant mean difference in rate of change in estimated GFR (-5.2 ± 3.6 mL/min/1.73 m2 per year; P < 0.001) was observed between the two groups. After Cox regression analysis, treatment with VLPD plus KA/EAA significantly protected against the incidence of declining GFR > 10% annually (adjusted hazard ratio, 0.42; 95% confidence interval, 0.23-0.79; P = 0.006) and significant correlations were found between using VLPD plus KA/EEA and increased GFR. Conclusion: VLPD supplementation with KA/EAA is associated with delayed renal progression while preserving the nutritional status in the patients with CKD. Co-administration of VLPD and KA/EAA may prove an effective alternative to conservative management of CKD. [ABSTRACT FROM AUTHOR]

It is well known that hyperphenylalaninemia caused by phenylketonuria (PKU) negatively influences cognitive performance. Several tests have been used to study these functions. Until now, no universal, optimal tool has been developed for detecting PKU-caused brain dysfunctions. Using computerized neuropsychological tests during daily routine would be helpful for screening subclinical brain deficits in adult PKU patients. In a monocentric, cross-sectional study, adult patients with PKU (n = 46; median age = 29.5 years; female/male ratio = 21/25) were tested with the computerized Cambridge Cognition (CANTAB) test measuring neurocognitive functions. Patients were divided into two groups: The “on diet” group included patients whose blood Phe-level was under 600 μmol/l (n = 20), and the “loose diet” group included patients whose blood Phe-level was above 600 μmol/l (n = 26) at the examination time. The results of the PKU-affected individuals were compared with a healthy control group (n = 31; median age = 25 years; female/male ratio = 11/20). Compared with the control group, PKU patients had significantly worse test results in memory, problem-solving skills, and strategy. However, there were no significant differences in response speed or initial thinking time. There was no correlation between the blood Phe-level, tyrosine (Tyr)-level or Phe/Tyr ratio and the different cognitive test results. There were no significant differences in test results between the two PKU subgroups. Several cognitive functions measured by CANTAB are negatively influenced by hyperphenylalaninemia in adult PKU patients. However, response speed and initial thinking time were not impaired as seriously as other functions. Patients with lower Phe-levels failed to achieve better test results than patients whose Phe-levels were notably elevated. [ABSTRACT FROM AUTHOR]

Introducción: El tratamiento de las enfermedades renales, que son un grave problema de salud pública, es muy complicado. La terapia nutrimental busca retardar la progresión de la enfermedad, mantener un buen estado nutricio y prevenir el desarrollo de comorbilidades. Objetivo: El objetivo del presente estudio fue analizar diez especies de pescado de consumo cotidiano para identificar aquellas que por su bajo aporte de fósforo, proteína de alto valor biológico y su aporte de ácidos grasos n-3 puedan incluirse en la dieta del paciente renal. Material y métodos: Se analizaron las siguientes especies: Bonito, Cabezona, Chucho, Escolar, Espada, Golondrina, Lenguado, Lobina, Mojarra rayada y Pámpano, siguiendo las técnicas de la AOAC y Keller, para determinar su contenido de proteína, fósforo, sodio, potasio, colesterol, vitaminas D3 y E y ácidos grasos n-3 EPA + DHA. Posteriormente se evaluaron las relaciones entre estos nutrimentos. Resultados: Las especies analizadas presentaron valores de proteína desde 16,5 g/100 g de filete (Lobina) hasta 27,2 g/100 g (Cabezona), el valor de fósforo más bajo fue de 28,6 mg/100 g (Mojarra rayada) y el más alto fue 216,3 mg/100 g (Chucho). 80% de las especies presentaron > 100 mg EPA + DHA en 100 g de filete. Por su relación Fósforo/g Proteína todos los pescados excepto Escolar y Espada, pueden incluirse; la relación más baja de fósforo/EPA + DHA se presentó en Bonito, Escolar, Golondrina, Lobina, Mojarra rayada. Conclusiones: El Pámpano es la especie más recomendada para los pacientes renales por las relaciones entre todos sus nutrimentos; aunque todas las especies, excepto Escolar y Espada, pueden formar parte de la alimentación renal.Introduction: Medical and nutritional treatment for renal disease, now a major public health issue, is highly complicated. Nutritional therapy must seek to retard renal dysfunction, maintain an optimal nutritional status and prevent the development of underlying pathologies. Objective: To analyze ten fish species to identify those that, because of their low phosphorus content, high biological value protein and elevated n-3 fatty acids EPA and DHA, could be included in renal patient's diet. Materials and methods: The following fish species (Litte tunny, Red drum, Spotted eagleray, Escolar, Swordfish, Big-scale pomfret, Cortez flounder, Largemouth blackbass, Periche mojarra, Florida Pompano) were analyzed according to the AOAC and Keller techniques to determine their protein, phosphorus, sodium, potassium, cholesterol, vitamins D3 and E, and n-3 EPA+DHA content. These results were used to calculate relations between nutrients. Results: The protein in the analyzed species ranged from 16.5 g/100 g of fillet (Largemouth black bass) to 27.2 g/100 g (Red drum); the lowest phosphorus value was 28.6 mg/100 g (Periche mojarra) and the highest 216.3 mg/100 g (Spotted eagle ray). 80% of the fish presented > 100 mg EPA + DHA in 100 g of fillet. By its Phosphorus/gProtein ratio, Escolar and Swordfish could not be included in the renal diet; Little tunny, Escolar, Big-scale pomfret, Largemouth black-bass, Periche mojarra and Florida Pompano presented a lower Phosphorus/EPA + DHA ratio. Conclusions: Florida pompano is the most recommended specie for renal patients, due to its optimal nutrient relations. However, all analyzed species, except Escolar and Swordfish, could be included in renal diets.

The potential benefits and dangers of dietary protein restriction in chronic kidney disease (CKD) are still controversial. Thus, the aim of this study is to evaluate the effect of low protein diet (LPD) on the renal function in nondialysis CKD patients. A retrospective study was conducted from 321 nondialysis CKD patient’s medical files (65.1 ± 12.7 yrs, 58.2% men). These patients received individualized dietary protein prescription (0.6–0.8 g protein/kg/day). Protein intake was evaluated by food diary and 24 h-food recall. Adherence to the LPD was considered when patients intake from 90 to 110% of the prescribed amount of protein. The patients were divided into 4 groups: (G1) adherent diabetes mellitus (DM) patients (n = 83); (G2) non-adherent DM patients (n = 106); (G3) adherent non-DM patients (n = 75); (G4) non-adherent non-DM patients (n = 57). Renal function was assessed by estimated glomerular filtration rate (eGFR). Both groups of patients (DM and non-DM) that adhered to the LPD showed significant improvement in eGFR (G1: 38.7 ± 13.2 mL/min to 51.1 ± 17.0 mL/min (p < 0.001); G3: 35.1 ± 16.8 mL/min to 46.8 ± 21.4 mL/min (p < 0.001)). In adherent patients, no differences in albumin and BMI were observed at the end of follow up. In non-adherent patients, eGFR significantly decreased in DM group (G2: 44.2 ± 18.5 mL/min to 38.2 ± 15.8 mL/min (p = 0.003)). According to multivariate analysis, annual changes in eGFR were not independent associated with age, gender, BMI, lipid profile, bicarbonate or smoking status. In summary, adherence to low protein diet could be able to improve serum creatinine and eGFR, well-known markers of renal function. However, prospective studies are needed to control confounders which affect renal function and CKD progression. [ABSTRACT FROM AUTHOR]

Background: The benefits of dietary protein restriction in CKD remain unclear, largely due to inadequate adherence in most clinical trials. We examined whether low-protein rice (LPR) previously developed to reduce the protein content of rice, a major staple food, would help improve adherence to dietary protein restriction., Methods: This open-label, multicenter, randomized, controlled trial evaluated the efficacy of LPR use for reducing dietary protein intake (DPI) in patients with CKD stages G3aA2-G4. Participants were randomly assigned in a 1:1 ratio to an LPR or control group and were followed up for 24 weeks. Both groups received regular counseling by dietitians to help achieve a target DPI of 0.7 g/kg ideal body weight (IBW) per day. The amount of protein in LPR is about 4% of that in ordinary rice, and the participants in the LPR group were instructed to consume LPR with at least two meals per day. The primary outcome was estimated dietary protein intake (eDPI) determined using the Maroni formula. The secondary outcomes included creatinine clearance (CCr) and urinary protein on the basis of 24-hour urine collection., Results: In total, 51 patients were randomized to either the LPR group or the control group. At baseline, mean age was 62.5 years, 70% were men, mean CCr was 52.0 ml/min, and mean eDPI was 0.99 g/kg IBW per day. At 24 weeks, mean eDPI decreased to 0.80 g/kg IBW per day in the LPR group and to 0.91 g/kg IBW per day in the control group, giving a between-group difference of 0.11 g/kg IBW per day (95% confidence interval, 0.03 to 0.19 g/kg IBW per day; P =0.006). There was no significant between-group difference in CCr, but urinary protein was lower at 24 weeks in the LPR group than in the control group., Conclusions: LPR is a feasible tool for efficiently reducing DPI in patients with CKD., Clinical Trial Registry Name and Registration Number: Randomized, Multicenter, Controlled Study for the Efficacy of Low-Protein Rice Diet in Patients with Chronic Kidney Disease, UMIN000015630., Competing Interests: M. Hosojima reports research funding from Biotech Japan Co., Ltd., Daiichi Sankyo Co., Ltd., Foricafoods Co., Ltd., Kameda Seika Co., Ltd., Kyowa Hakko Kirin Co., Ltd., Mitsubishi Tanabe Pharma Corporation, MSD K.K., Sato Foods Co., Ltd., and Taisho Toyama Pharmaceutical.Co., Ltd. and speakers bureau for AstraZeneca K.K., Chugai Pharmaceutical Co., Ltd., Kissei Pharmaceutical Co., Ltd., Kyowa Hakko Kirin Co., Ltd., Mitsubishi Tanabe Pharma Corporation, MSD K.K., Nippon Boehringer Ingelheim Co., Ltd., Novartis Pharma K.K., Ono Pharmaceutical Co., Ltd., Sanwa Kagaku Kenkyusho Co., Ltd., Sumitomo Dainippon Pharma Co., Ltd., and Taisho Toyama Pharmaceutical.Co., Ltd. H. Kabasawa reports research funding from Biotech Japan Co., Ltd., Foricafoods Co., Ltd., Kameda Seika Co., Ltd., and Sato Foods Co., Ltd.; speakers bureau for AstraZeneca K.K., Kyowa Hakko Kirin Co., Ltd., Nippon Boehringer Ingelheim Co., Ltd., Otsuka Pharmaceutical Co., Ltd., and Sumitomo Dainippon Pharma Co., Ltd. I. Narita reports research funding from Chugai Pharmaceutical, Daiichi-Sankyo, Kyowa-kirin, Otsuka Pharmaceutical, Sanwa Kagaku Kenkyusho, Sumitomo Pharma, and Terumo; and honoraria from AstraZeneca, Bayer, Kyowa-Kirin, Otsuka, and Sanofi. Y. Obi reports consultancy agreements with Obi Clinic, research funding from Relypsa/Vifor Pharma, honoraria from Kyowa Kirin, and an advisory or leadership role as an editorial board member of Renal Replacement Therapy. A. Saito reports research funding from Denka Company Limited, Daiichi-Sankyo, Kowa Pharmaceutical Company Ltd., Mitsubishi Tanabe Pharma Corporation, MSD K.K., Nippon Boehringer Ingelheim Co., Ltd, Taisho Toyama Pharmaceutical Co., Ltd., and Torii Pharmaceutical Co., Ltd. and speakers bureau for AstraZeneca K.K., Chugai Pharmaceutical Co., Daiichi-Sankyo, Daiichi Sankyo Company, Limited, Kowa Pharmaceutical Co. Ltd., Kyowa Kirin Co. Ltd., MSD K.K., Nippon Boehringer Ingelheim Co., Ltd., and Ono Pharmaceutical Co., Ltd. All remaining authors have nothing to disclose., (Copyright © 2022 by the American Society of Nephrology.)

Introduction: Medical and nutritional treatment for renal disease, now a major public health issue, is highly complicated. Nutritional therapy must seek to retard renal dysfunction, maintain an optimal nutritional status and prevent the development of underlying pathologies. Objective: To analyze ten fish species to identify those that, because of their low phosphorus content, high biological value protein and elevated n-3 fatty acids EPA and DHA, could be included in renal patient's diet. Materials and methods: The following fish species (Litte tunny, Red drum, Spotted eagleray, Escolar, Swordfish, Big-scale pomfret, Cortez flounder, Largemouth blackbass, Periche mojarra, Florida Pompano) were analyzed according to the AOAC and Keller techniques to determine their protein, phosphorus, sodium, potassium, cholesterol, vitamins D33 and E, and n-3 EPA+DHA content. These results were used to calculate relations between nutrients. Results: The protein in the analyzed species ranged from 16.5 g/100 g of fillet (Largemouth black bass) to 27.2 g/100 g (Red drum); the lowest phosphorus value was 28.6 mg/100 g (Periche mojarra) and the highest 216.3 mg/100 g (Spotted eagle ray). 80% of the fish presented > 100 mg EPA + DHA in 100 g of fillet. By its Phosphorus/gProtein ratio, Escolar and Swordfish could not be included in the renal diet; Little tunny, Escolar, Big-scale pomfret, Largemouth black-bass, Periche mojarra and Florida Pompano presented a lower Phosphorus/EPA + DHA ratio. Conclusions: Florida pompano is the most recommended specie for renal patients, due to its optimal nutrient relations. However, all analyzed species, except Escolar and Swordfish, could be included in renal diets. [ABSTRACT FROM AUTHOR]

Adam S., Champion H., Daly A., Dawson S., Dixon M., Dunlop C., Eardley J., Evans S., Ferguson C., Jankowski C., Lowry S., MacDonald A., Maritz C., Micciche A., Robertson L., Stafford J., Terry A., Thom R., van Wyk K., Webster D., White F.J. & Wildgoose J. on behalf of the British Inherited Metabolic Diseases Group (BIMDG) Dietitian's Group. (2012) Dietary management of urea cycle disorders: UK practice. J Hum Nutr Diet. 25, 398-404 Abstract Background: There is no published data describing UK dietary management of urea cycle disorders (UCD). The present study describes dietary practices in UK inherited metabolic disorder (IMD) centres. Methods: Cross-sectional data from 16 IMD centres were collected by a questionnaire describing the management of UCD patients on prescribed protein-restricted diets. Results: One hundred and seventy-five patients [ N-acetylglutamate synthase deficiency, n = 3; carbamoyl phosphate synthase deficiency (CPS), n = 8; ornithine transcarbamoylase deficiency (OTC), n = 75; citrullinaemia, n = 41; argininosuccinic aciduria (ASA), n = 36; arginase deficiency, n = 12] were reported; 70% ( n = 123) aged 0-16 years; 30% ( n = 52) >16 years. Prescribed median protein intake decreased with age (0-6 months: 2 g kg−1 day−1; 7-12 months: 1.6 g kg−1 day−1; 1-10 years: 1.3 g kg−1 day−1; 11-16 years: 0.9 g kg−1 day−1 and >16 years: 0.8 g kg−1 day−1) with little variation between disorders. Adult protein prescription ranged 0.4-1.2 g kg−1 day−1 (40-60 g day−1). In the previous 2 years, 30% ( n = 53) were given essential amino acid supplements (EAAs) (CPS, n = 2; OTC, n = 20; citrullinaemia, n = 15; ASA, n = 7; arginase deficiency, n = 9). EAAs were prescribed for low plasma quantitative essential amino acids ( n = 13 centres); inadequate natural protein intake ( n = 11) and poor metabolic control ( n = 9). From diagnosis, one centre prescribed EAAs for all patients and one centre for severe defects only. Only 3% ( n = 6) were given branch chain amino acid supplements. Enteral feeding tubes were used by 25% ( n = 44) for feeds and 3% ( n = 6) for medications. Oral energy supplements were prescribed in 17% ( n = 30) of cases. Conclusions: In the UK, protein restriction based on World Health Organization 'safe intakes of protein', is the principle dietary treatment for UCD. EAA supplements are prescribed mainly on clinical need. Multicentre collaborative research is required to define optimal dietary treatments. [ABSTRACT FROM AUTHOR]

Abstract: Low blood docosahexaenoic acid (DHA) is reported in patients with phenylketonuria (PKU); however, the functional implications in adolescents and adults are unknown. This pilot study investigated the effect of supplemental DHA on cognitive performance in 33 females with PKU ages 12–47 years. Participants were randomly assigned to receive DHA (10mg/kg/day) or placebo for 4.5 months. Performance on cognitive processing speed and executive functioning tasks was evaluated at baseline and follow up. Intention-to-treat and per protocol analyses were performed. At follow up, biomarkers of DHA status were significantly higher in the DHA-supplemented group. Performance on the cognitive tasks and reported treatment-related adverse events did not differ. While no evidence of cognitive effect was seen, a larger sample size is needed to be conclusive, which may not be feasible in this population. Supplementation was a safe and effective way to increase biomarkers of DHA status (www.clinicaltrials.gov; Identifier: NCT00892554). [Copyright &y& Elsevier]

This study was performed to analyse the effects of low-protein diet (7%) on bone mass and mineral metabolism in rat pups whose mothers were submitted to protein-restricted diet during late pregnancy and early post-natal periods. For this purpose, 12 pregnant Wistar rats were divided into two groups of six animals each: a control group with free access to standard diet (20% protein) and protein-restricted group (PR) fed with low-protein diet (7% protein) from the 14th day of pregnancy until day 14 after delivery. Body weight, femur bone mass, plasma thyroid hormones (FT4 and FT3), biochemical bone marker levels [alkaline phosphatase (ALP), tartrate-resistant acid phosphatase (ACP)] and bone DNA content were recorded. In undernourished pups, a decrease in body weight (−47%, p < 0.001) in skeletal growth (−11%, p < 0.001) and in bone DNA content (−48%, p < 0.001) were observed. Plasma protein and albumin levels and thyroid status were also decreased in undernourished rat pups compared with those of control group. The circulating amino acid levels were decreased in pups. While in their mothers, some of them were increased and the others were decreased. A positive correlation was observed between bone mass and plasma thyroid hormone levels and ALP activity and plasma protein levels, and a negative correlation with ACP activity. Plasma ALP levels were decreased by 19%, whereas plasma tartrate-resistant ACP levels were increased by 33% indicating a hyperactivity of bone remodelling. These results showed that protein-restricted diet given to mothers during late pregnancy and early post-natal periods induced changes in body weight, skeletal growth and bone metabolism in their offspring. [ABSTRACT FROM AUTHOR]

According to the hyperfiltration theory of renal diseases characterized by a decrease in the number of functional nephrons, increased arterial blood pressure, excessive protein intake in the diet, high levels of calcium (Ca) and phosphorus (P), secondary hyperparathyroidism, hypertriglyceridemia and/or hypercholesterolemia, proteinuria and metabolic acidosis are some factors that impair the prognosis of the disease. The amount of protein in the diet is the most important of these factors. A protein-restricted diet administered to patients with chronic renal failure results in the risk of inadequate amino acid intake. To overcome this problem, the use of dysaminated α-keto analogues has been considered to reduce the risk of nitrogenemia resulting from the continuous intake of essential amino acids. Currently, the necessity of essential amino acids even in adult patients with chronic renal failure is controversial; besides, trials on the use of these amino acids in pediatric patients are scarce. The aim of this study is to investigate the efficacy and applicability of conservative therapy with a protein-restricted diet supplemented with keto acids in the management of chronic renal insufficiency or failure. [ABSTRACT FROM AUTHOR]

Chronic renal failure (CRF) provokes derangement in various hormonal regulations of food intake and energy expenditure. In the present study, we have examined the effect of a low protein, low phosphorus diet on circulating levels of leptin, tumour necrosis factor (TNF)-alpha, and insulin in patients with CRF.Seventeen male, non-diabetic subjects with conservatively treated CRF (estimated creatinine clearance 39.5 ± 11.1 mL/min), and proteinuria below 2 g/day were prospectively studied. Measurements of hormonal, metabolic and anthropometric parameters were performed before and after 16 weeks of dietary treatment (protein 0.6 g/kg of ideal body mass, 30% of calories derived from fat, 62% of calories derived from carbohydrates and 10 mg/kg of phosphorus). Actual dietary intake in patients was measured by using 3-day food records and was supervised by the dietician.Body mass index and body fat mass remained unchanged during the 4 months of dietary treatment. Urea, leptin, and TNF-alpha serum concentrations decreased significantly.A low protein, low phosphorus diet reduces TNF-alpha and leptin levels in plasma. The effect on leptin appears not to be mediated by an insulin-dependent mechanism. [ABSTRACT FROM AUTHOR]

Malnutrition is known to have adverse effects on the physiology and morphology of the liver. The aim of this investigation was to examine the effect of protein restriction on the content of plasma membrane proteins residing in the sinusoidal and bile canalicular domains of rat liver. Post-weanling rats maintained on low protein isocaloric diets showed marked growth retardation concomitant with reduced liver protein concentration compared to control animals. The content of leucine aminopeptidase, a bile canalicular enzyme, and asialoglycoprotein receptor, a sinusoidal receptor, in livers of protein-restricted rats was 66% and 50%, respectively, of control livers. In contrast, the relative concentrations of dipeptidyl peptidase IV and a cell adhesion molecule (GP 110), both canalicular proteins, were 160% and 121%, respectively, in rat livers upon protein restriction. After a 4-week rehabilitation period, the concentrations of all canalicular membrane proteins were similar to those in control livers, while the sinusoidal receptor was only 68% of control values. Protein restriction was found to adversely affect the concentrations of protein constituents, but not their localization in the hepatocyte plasma membrane. In general, altered concentrations of hepatocyte membrane proteins were reversed on the administration of a normal protein diet. [ABSTRACT FROM AUTHOR]

A 112-day experiment was conducted to investigate the influence of the content of microbial protein and poly-β-hydroxybutyrate (PHB) in biofloc on the growth, productive performance, and hemato-biochemical parameters of Nile tilapia (Oreochromis niloticus) fingerlings. The fish cultivated using the biofloc technology system (BFT) were reared at two stocking densities (40 and 80 fish m−3) and fed with a gradual protein-restriction diet (LPBFT). Two clear-water controls (CW) were used for the estimation of the density: High protein control (HP) and Low protein control (LP). The fish from HP (HPCW) were fed with commercial pellets containing 45% crude protein (CP) during the initial seven weeks and 35% CP during the final nine weeks. The fish from LP (LPCW) and LPBFT were fed with 35% CP during the initial seven weeks and 25% CP until harvest. The BFT microbial proteins were observed to compensate for the restriction of the dietary proteins. A 22.2% net reduction in the CP given to fish of 4.29 ± 0.25 g to 40.5 ± 5.4 g BW, and by 28.6% to fish of 40.5 ± 5.4 g to 182.6 ± 5.2 g BW did not negatively affect the survival, growth, and health of the fingerlings. Weight gain was significantly higher in LPBFT than LPCW at both stocking densities, and no significant differences were observed between the fish raised in CW and those fed on a high protein diet. The net yield of LPBFTs was increased by 27.9–29.5% compared to LP controls, while FCR was improved by 1.6% with the incorporation of BFT. When the concentration of heterotrophic bacteria (HB) reached 5.06 × 105 and 5.66 × 105 CFU mL−1, the maximum value of microbial proteins was 33.0 ± 5.9% for LPBFT-L and 32.5 ± 6.2% for LPBFT-H, respectively. The variables associated with the cumulative variance in the percentage of microbial protein were the concentration of HB (84.3%), followed by total organic carbon (8.6%) and NO 3 -N (4.6%). The PHB increased significantly with an increase in the concentration of HB (R = 80%) and showed a 75% correlation with the microbial protein content. During the final week of the culture, 1 mL of floc containing an average of 5.22 ± 1.78 × 105 CFU mL−1 produced 18.57 ± 2.22 nmol 100 g−1 DW of PBH. The blood biochemistry profile from LPBFT was similar to the high protein controls. Our data confirm the potential of the application of BFT during the early stages of tilapia growth, without affecting the survival, productive parameters, or the overall health of the organisms. • A net reduction of 23 - 29 % CP in fish of 4.3– 183 g reared in BFT does not affect survival, growth, and health status. • BFT increased 27.9–29.5% net yield of the fingerlings fed on a protein-restricted diet compared with clear water control. • PBH acid is associated with 80% concentration of floc's heterotrophic bacteria (HP) and in 75% with the microbial protein. • 1 mL of floc with 5.22 × 105 CFU of HB produced 32.5–33.0 % of microbial protein and 18.57 nmol 100 g-1 DW of PBH. [ABSTRACT FROM AUTHOR]