scholarly journals The Impact of a Slow-Release Large Neutral Amino Acids Supplement on Treatment Adherence in Adult Patients with Phenylketonuria

Nutrients ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 2078 ◽  
Author(s):  
Alessandro P. Burlina ◽  
Chiara Cazzorla ◽  
Pamela Massa ◽  
Christian Loro ◽  
Daniela Gueraldi ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Amino Acids ◽  
Medication Adherence ◽  
Slow Release ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Adherence To Medication ◽  
Full Adherence ◽  
Adherence To Therapy

The gold standard treatment for phenylketonuria (PKU) is a lifelong low-phenylalanine (Phe) diet supplemented with Phe-free protein substitutes. Adherence to therapy becomes difficult after childhood. Supplementing with large neutral amino acids (LNAAs) has been proposed as an alternative medication to Phe-free protein substitutes (i.e., amino acid mixtures). The aim of this study was to evaluate adherence to therapy and quality of life (QoL) in a cohort of sub-optimally controlled adult PKU patients treated with a new LNAA formulation. Twelve patients were enrolled in a 12-month-trial of slow-release LNAAs (1g/kg/day) plus a Phe-restricted diet. Medication adherence was measured with the Morisky Green Levine Medication Adherence Scale; the QoL was measured using the phenylketonuria-quality of life (PKU-QoL) questionnaire. Phe, tyrosine (Tyr) levels, and Phe/Tyr ratios were measured fortnightly. Before treatment, 3/12 patients self-reported a ‘medium’ adherence to medication and 9/12 reported a low adherence; 60% of patients reported a full adherence over the past four weeks. After 12 months of LNAA treatment, all patients self-reported a high adherence to medication, with 96% reporting a full adherence. Phe levels remained unchanged, while Tyr levels increased in most patients. The Phy/Tyr ratio decreased. All patients had a significant improvement in the QoL. LNAAs may give patients a further opportunity to improve medication adherence and, consequently, their QoL.

scholarly journals Beneficial Effects of Slow-Release Large Neutral Amino Acids after a Phenylalanine Oral Load in Patients with Phenylketonuria

Nutrients ◽  
2021 ◽  
Vol 13 (11) ◽  
pp. 4012
Author(s):  
Iris Scala ◽  
Daniela Concolino ◽  
Anna Nastasi ◽  
Giulia Esposito ◽  
Daniela Crisci ◽  
...  
Keyword(s):  
Amino Acids ◽  
Slow Release ◽  
Good Adherence ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Beneficial Effects ◽  
Adherence To Diet ◽  
Low Protein ◽  
Single Oral Administration ◽  
Stable Plasma

The mainstay of phenylketonuria treatment is a low protein diet, supplemented with phenylalanine (Phe)-free protein substitutes and micronutrients. Adhering to this diet is challenging, and even patients with good metabolic control who follow the dietary prescriptions in everyday life ignore the recommendations occasionally. The present study explores the ability of slow-release large neutral amino acids (srLNAAs) to prevent Phe increase following a Phe dietary load. Fourteen phenylketonuric patients aged ≥13 years were enrolled in a 6-week protocol. Oral acute Phe loads of 250 and 500 mg were added to the evening meal together with srLNAAs (0.5 gr/kg). Phe and tyrosine were dosed before dinner, 2h-after dinner, and after the overnight fast. After oral Phe loads, mean plasma Phe remained stable and below 600 µmol/L. No Phe peaks were registered. Tyrosine levels significantly increased, and Phe/Tyrosine ratio decreased. No adverse events were registered. In conclusion, a single oral administration of srLNAAs at the dose of 0.5 gr/kg is effective in maintaining stable plasma Phe during acute oral loads with Phe-containing food and may be added to the dietetic scheme in situations in which patients with generally good adherence to diet foresee a higher than prescribed Phe intake due to their commitments.

Quality of Life and Its Association With Treatment Satisfaction, Adherence to Medication, and Trust in Physician Among Patients With Hypertension

2018 ◽  
Vol 23 (6) ◽  
pp. 532-542 ◽  
Author(s):  
Siba Jneid ◽  
Hicham Jabbour ◽  
Aline Hajj ◽  
Antoine Sarkis ◽  
Hady Licha ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Medication Adherence ◽  
Psychological Health ◽  
Treatment Satisfaction ◽  
World Health ◽  
Adherence To Treatment ◽  
World Health Organization Quality ◽  
Global Satisfaction ◽  
Adherence To Medication

Objective: Patients with hypertension tend to have a lower health-related quality of life (HRQOL) compared to normotensive patients. This study’s main objectives are to assess (1) HRQOL and factors that might impact it and (2) association between HRQOL and adherence to treatment, trust in physician, and treatment satisfaction among patients with hypertension. Methods: A cross-sectional study enrolled 196 patients from medical-care offices in Beirut, North and South Lebanon. Eligible patients signed a consent form and were asked to complete the Trust in Physician Scale, World Health Organization Quality of Life-brief (WHOQOL-brief) questionnaire, and Treatment Satisfaction Questionnaire (TSQM 1.4) as well as questions assessing medication adherence. Results: Better antihypertensive medication adherence was significantly and positively correlated with better HRQOL domains except general health where significance was not reached ( P = .089). Better adherence was also significantly related to better treatment satisfaction (side effects, convenience, and global satisfaction but not to effectiveness) and an increased trust in physicians ( P < .0001). Better treatment satisfaction (TSQM domains) was significantly and positively associated with a better overall HRQOL ( P ≤ .001). Increased trust in physician scores were significantly and positively correlated with a better psychological health, environment, and overall HRQOL domains ( P = .045, .005, and .006, respectively). Finally, TSQM effectiveness, convenience, and global satisfaction were significantly higher when trust in physician was greater ( P = .017, .035, and .002, respectively). Conclusion: The results of this study revealed an association between increased adherence to treatment, a higher global satisfaction, and an increased quality of life. An increased patient satisfaction with their antihypertensive treatment and trust in their physician were also correlated with a greater adherence to treatment.

scholarly journals Hypertension-related knowledge, medication adherence and health-related quality of life (HRQoL) among hypertensive patients in Islamabad, Pakistan

2021 ◽  
Vol 18 (5) ◽  
pp. 1123-1132
Author(s):  
Muhammad Amer ◽  
Nisar-Ur-Rahman ◽  
Saeed-Ur-Rashid Nazir ◽  
Atif Raza ◽  
Humayun Riaz ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Medication Adherence ◽  
Correlation Coefficient ◽  
Positive Association ◽  
Cross Sectional Study ◽  
Cross Sectional ◽  
Hypertensive Patients ◽  
Adherence To Medication ◽  
Hypertension Knowledge

Purpose: To determine knowledge regarding hypertension, adherence to medication and HealthRelated Quality of Life (HRQoL), and their associations in hypertensive patients in Pakistan. Methods: A cross-sectional study was conducted among 384 hypertensive patients attending a tertiary health care public sector hospital in Islamabad, Pakistan. Data were collected using knowledge questionnaire regarding hypertension, Morisky Medication Adherence Scale, and EuroQol (EQ-5D) scale. Results: The mean systolic and diastolic blood pressures of the 384 patients were 140.39 ± 15.485 and 88.74 ± 10.683 mmHg, respectively. The coefficient of correlation between knowledge regarding hypertension and adherence was 0.638 (p < 0.001), showing a positive association. The correlation coefficient between knowledge and HRQoL was 0.709 (p < 0.001), suggesting a good association. The correlation coefficient between adherence to medication and HRQoL was 0.545 (p < 0.001), which indicated a positive correlation. Conclusion: These results indicate that there are statistically significant associations between hypertension knowledge and HRQoL, hypertension knowledge and medication adherence, and between adherence and HRQoL in the hypertensive patients studied.

scholarly journals Assessment of medication adherence and quality of life among patients with type 2 diabetes mellitus in a tertiary hospital in Kelantan, Malaysia

2021 ◽  
Vol 1 (2) ◽  
pp. 79-86
Author(s):  
Nazmi Liana Azmi ◽  
Nurul Aida Md Rosly ◽  
Hock Chun Tang ◽  
Anis Fariha Che Darof ◽  
Nor Dini Zuki
Keyword(s):  
Quality Of Life ◽  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Type 2 Diabetes Mellitus ◽  
Medication Adherence ◽  
Life Questionnaire ◽  
Adherence To Medication ◽  
The Mean

Introduction: Previous studies have reported the relationship between medication adherence and quality of life are interrelated. However, many of the results were found to be conflicting. This study aimed to assess the level and association of medication adherence and quality of life among type 2 diabetes mellitus patients in Raja Perempuan Zainab II Hospital, Kelantan, Malaysia. Materials and methods: A cross-sectional survey was conducted among adult type 2 diabetes mellitus patients on treatment for over 1 year using convenience sampling at outpatient.  Medication Compliance Questionnaire (MCQ) and revised Diabetes Quality of Life Questionnaire (DQOL) instrument were self-administered to eligible subjects. Data were analysed using GNU PSPP version 0.8.5 and reported for descriptive statistics as well as correlation of both parameters. Results: A total of 200 patients were recruited and they were mostly at the age of 40 to 60 years old. The mean (SD) score for MCQ was 26.0 (1.6) with the majority of them were non-adherent (55.0%, n=110). The mean (SD) score for overall revised DQOL instrument was 25.5 (8.9) while each domain of “satisfaction”, “impact” and “worry” had mean (SD) scores of 12.0 (5.0), 7.7 (3.4) and 5.9 (2.7), respectively. The scores obtained were only approximately half of the possible range of scores for QoL. There was no significant correlation between total score of medication adherence and quality of life when tested using Pearson’s correlation (r=-0.083, p=0.240). Independent t-test also demonstrated no significant relationship between medication adherence status and quality of life (p=0.883). Conclusion: Type 2 diabetes mellitus patients in our setting had unsatisfactory adherence but exhibited acceptable quality of life. We observed that both variables were not associated with one another. Further research is warranted to identify potential factors affecting non-adherence to medication.

Atypical amino acids inhibit histidine, valine, or lysine transport into rat brain

1983 ◽  
Vol 245 (4) ◽  
pp. R556-R563 ◽  
Author(s):  
J. K. Tews ◽  
A. E. Harper
Keyword(s):  
Amino Acids ◽  
Rat Brain ◽  
Blood Brain Barrier ◽  
Brain Slices ◽  
Aromatic Amino Acids ◽  
Brain Barrier ◽  
Basic Amino Acids ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Single Meal

Transport of histidine, valine, or lysine into rat brain slices and across the blood-brain barrier (BBB) was determined in the presence of atypical nonprotein amino acids. Competitors of histidine and valine transport in slices were large neutral amino acids including norleucine, norvaline, alpha-aminooctanoate, beta-methylphenylalanine, and alpha-aminophenylacetate. Less effective were aromatic amino acids with ring substituents; ineffective were basic amino acids and omega-amino isomers of norleucine and aminooctanoate. Lysine transport was moderately depressed by homoarginine or ornithine plus arginine; large neutral amino acids were also similarly inhibitory. Histidine or valine transport across the BBB was also strongly inhibited by large neutral amino acids that were the most effective competitors in the slices (norvaline, norleucine, alpha-aminooctanoate, and alpha-aminophenylacetate); homoarginine and 8-aminooctanoate were ineffective. Homoarginine, ornithine, and arginine almost completely blocked lysine transport, but the large neutral amino acids were barely inhibitory. When rats were fed a single meal containing individual atypical large neutral amino acids or homoarginine, brain pools of certain large neutral amino acids or of arginine and lysine, respectively, were depleted.

scholarly journals Correction to: The relationship between comorbidity medication adherence and health related quality of life among patients with cancer

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dana Drzayich Antol ◽  
Adrianne Waldman Casebeer ◽  
Raya Khoury ◽  
Todd Michael ◽  
Andrew Renda ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Medication Adherence ◽  
Health Related Quality ◽  
Patients With Cancer ◽  
Related Quality ◽  
Health Related ◽  
The Relationship

An amendment to this paper has been published and can be accessed via the original article.

scholarly journals Analysis of Medication Adherence and Its Influencing Factors in Patients with Schizophrenia in the Chinese Institutional Environment

2021 ◽  
Vol 18 (9) ◽  
pp. 4746
Author(s):  
Wei Yu ◽  
Jie Tong ◽  
Xirong Sun ◽  
Fazhan Chen ◽  
Jie Zhang ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Medication Adherence ◽  
Activities Of Daily Living ◽  
Influencing Factors ◽  
Self Efficacy ◽  
Daily Living ◽  
Institutional Environment ◽  
Factors Influencing ◽  
Quality Of Life Scale

Background: Factors related to medication adherence in patients with schizophrenia have always been key to the treatment and rehabilitation of these patients. However, the treatment modes in different countries are not the same, and there is no research on the factors influencing medication adherence under different mental health service modes. Objectives: The purpose of this study was to explore medication adherence and its influencing factors in patients with schizophrenia in the Chinese institutional environment. Methods: We conducted a cross-sectional study of hospitalized persons living with schizophrenia from November 2018 to January 2019. A systematic sampling method was used to select 217 hospitalized persons living with schizophrenia. The Medication Adherence Rating Scale (MARS), Positive and Negative Syndrome Scale (PANSS), General Self-Efficacy Scale (GSES), Schizophrenia Quality of Life Scale (SQLS), and Scale of Social Skills for Psychiatric Inpatients (SSPI) were used to explore medication compliance and its influencing factors in the Chinese institutional environment. Results: The descriptive analysis and ANOVA showed that there were no significant differences in medication adherence when assessed by demographic characteristics such as sex, marital status, and education level (p > 0.05). A correlation analysis showed that there was no significant correlation between medication adherence and mental symptoms (p > 0.05) but that there was a positive correlation with self-efficacy, quality of life, and activities of daily living (p < 0.01). The linear regression analysis showed that self-efficacy, psychosocial factors, symptoms/side effects, and activities of daily living had significant effects on medication adherence (F = 30.210, p < 0.001). Conclusions: Our findings show that the self-efficacy, quality of life, and social function of patients with schizophrenia are important self-factors influencing medication adherence in the Chinese institutional environment.

scholarly journals Adherence to Therapy, Physical and Mental Quality of Life in Patients with Multiple Sclerosis

2021 ◽  
Vol 11 (7) ◽  
pp. 672
Author(s):  
Alessandra Buja ◽  
Guendalina Graffigna ◽  
Simona F. Mafrici ◽  
Tatjana Baldovin ◽  
Carlo Pinato ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Multiple Sclerosis ◽  
Behavioral Interventions ◽  
Positive Association ◽  
Cross Sectional Study ◽  
Health Index ◽  
Online Questionnaire ◽  
Cross Sectional ◽  
Adherence To Therapy

Ensuring multiple sclerosis (MS) patients’ adherence to therapy is often challenging, but it is crucial to their survival and health-related quality of life (HRQoL). The aim of the present study was to outline connections between adherence, physical and mental HRQoL, levels of psychological readiness to engage in a treatment, levels of social support, anthropometric, socio-demographic and clinical factors in patients suffering from MS. This cross-sectional study involved a sample of 237 Italian MS patients. A survey was conducted with a structured self-administered online questionnaire using validated measures of quality of life, adherence to therapy and anthropometric, socio-demographic, psychological and clinical variables. A path analysis was used to test the overall structure of the associations between the variables. The pathway indicates a positive association between mental health index and a stronger degree of engagement and being or having been in a long-term relationship. Physical health index was positively associated with age, having an occupation, and having a specific form of MS. Having had relapses in the previous year raised the odds of better adherence to therapy, while an increase in Body Mass Index (BMI) reduced them. Our findings could help in the management of MS patients, promoting behavioral interventions that take the psychological and socio-demographic peculiarities of each patient into account with a view to improving their adherence to therapy.

scholarly journals Large Neutral Amino Acids (LNAAs) Supplementation Improves Neuropsychological Performances in Adult Patients with Phenylketonuria

Nutrients ◽  
2020 ◽  
Vol 12 (4) ◽  
pp. 1092 ◽  
Author(s):  
Iris Scala ◽  
Maria Pia Riccio ◽  
Maria Marino ◽  
Carmela Bravaccio ◽  
Giancarlo Parenti ◽  
...  
Keyword(s):  
Amino Acids ◽  
Executive Functions ◽  
Metabolic Control ◽  
Cognitive Functions ◽  
Well Being ◽  
Wisconsin Card Sorting Test ◽  
Card Sorting ◽  
Large Neutral Amino Acids ◽  
Neutral Amino Acids ◽  
Poor Metabolic Control

Phenylketonuria is an inborn error of phenylalanine (Phe) metabolism diagnosed by newborn screening and treated early with diet. Although diet prevents intellectual disability, patients often show impairment of executive functions, working memory, sustained attention, and cognitive flexibility. Large neutral amino acids (LNAAs) have been proposed as a dietary supplement for PKU adults. Few studies show that LNAAs may help in improving metabolic control as well as cognitive functions. In this study, 10 adult PKU patients with poor metabolic control were treated for 12 months with LNAAs (MovisCom, 0.8–1 g/kg/day) and underwent Phe and Tyrosine (Tyr) monitoring monthly. Neuropsychological assessment was performed at T0, T+3, and T+12 months by using the American Psychological General Well-Being Index, the Wisconsin Card Sorting Test, the Test of Attentional Performance, and the 9-Hole Peg Test. No change in plasma Phe levels was observed during LNAAs supplementation, while Tyr levels significantly improved during LNAAs supplementation (p = 0.03). Psychometric tests showed an improvement of distress and well-being rates, of executive functions, attention, and vigilance, whereas no difference was noted regarding hand dexterity. This study adds evidence of the advantage of LNAAs supplementation in improving cognitive functions and well-being in patients with PKU with poor metabolic control.

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