Efficacy of Phenylalanine- and Tyrosine-Restricted Diet in Alkaptonuria Patients on Nitisinone Treatment: Case Series and Review of Literature

2021 ◽  
pp. 1-13
Author(s):  
Pelin Teke Kisa ◽  
Semra Eroglu Erkmen ◽  
Hilal Bahceci ◽  
Zumrut Arslan Gulten ◽  
Ayca Aydogan ◽  
...  
Keyword(s):  
Amino Acid ◽  
Free Amino Acid ◽  
Protein Intake ◽  
Free Amino ◽  
Case Series ◽  
Nutrient Deficiency ◽  
Ophthalmological Examination ◽  
Restricted Diet ◽  
Dietary Compliance ◽  
Plasma Tyrosine

<b><i>Introduction:</i></b> Nitisinone used in alkaptonuria (AKU) can result in keratopathy due to strongly increased tyrosine levels. <b><i>Methods:</i></b> This study aimed to investigate nutritional status and changes in plasma tyrosine and phenylalanine and urinary homogentisic acid (u-HGA) levels in 8 adult AKU patients (mean age, 56.3 ± 4.7 years) who were on tyrosine/phenylalanine-restricted diet together with 2 mg/day nitisinone. <b><i>Results:</i></b> The treatment period was 23.4 ± 6.9 months. Daily dietary protein intake was restricted to 0.8–1.0 g/kg/day. Daily tyrosine intake was restricted to 260–450 mg/day for females and 330–550 mg/day for males. Tyrosine/phenylalanine-free amino acid supplements accounted for an average of 56.1% of daily protein intake. The following assessments were performed: anthropometric and plasma tyrosine level measurements every 2 months; ophthalmological examination every 6 months, and nutritional laboratory analyses and measurements of plasma amino acids and u-HGA once in a year. It was targeted to keep the plasma tyrosine level &#x3c;500 μmol/L. The plasma tyrosine level was &#x3c;100 μmol/L before the treatment in all patients and around a mean of 582.5 ± 194.8 μmol/L during the treatment. The diet was rearranged if a plasma tyrosine level of &#x3e;700 μmol/L was detected. The u-HGA level before and after the 1st year of treatment was 1,429.3 ± 1,073.4 mmol/mol creatinine and 33.6 ± 9.5 mmol/mol creatinine, respectively. None of the patients developed keratopathy or experienced weight loss and protein or micronutrient deficiency. <b><i>Conclusion:</i></b> AKU patients should receive tyrosine/phenylalanine-restricted diet for reducing plasma tyrosine level to the safe range. Tyrosine/phenylalanine-free amino acid supplements can be safely used to enhance dietary compliance. Keratopathy and nutrient deficiency should be frequently monitored.

The effect of steaming on the free amino acid contents and antioxidant activity of ginseng

Planta Medica ◽  
2008 ◽  
Vol 74 (09) ◽  
Author(s):  
EJ Cho ◽  
XL Piao ◽  
MH Jang ◽  
SY Park ◽  
SW Kwon ◽  
...  
Keyword(s):  
Antioxidant Activity ◽  
Amino Acid ◽  
Free Amino Acid ◽  
Free Amino ◽  
Amino Acid Contents

Tronchuda cabbage (Brassica oleracea L. var. costata DC) leaf free amino acid profile

Planta Medica ◽  
2008 ◽  
Vol 74 (09) ◽  
Author(s):  
BM Silva ◽  
AP Oliveira ◽  
DM Pereira ◽  
C Sousa ◽  
RM Seabra ◽  
...  
Keyword(s):  
Amino Acid ◽  
Brassica Oleracea ◽  
Free Amino Acid ◽  
Free Amino ◽  
Amino Acid Profile ◽  
Tronchuda Cabbage ◽  
Brassica Oleracea L

Evaluation of plasma free amino acid and carnitine levels in patients with cesarean scar pregnancy

2020 ◽  
Vol 23 ◽  
Author(s):  
Omer Tammo ◽  
Hacer Uyanikoglu ◽  
İsmail Koyuncu
Keyword(s):  
Amino Acid ◽  
Pregnant Women ◽  
Free Amino Acid ◽  
Free Amino ◽  
Plasma Concentrations ◽  
Myometrial Invasion ◽  
Controlled Study ◽  
Control Group ◽  
Cesarean Scar Pregnancy ◽  
Cesarean Scar

Aim and Objective: This study aimed to explore the plasma free amino acid (FAA) and carnitine levels in pregnant women with cesarean scar pregnancy (CSP), and to compare them with those of healthy pregnant women. Materials and Methods: This prospective and randomized controlled study was conducted in patients admitted to Harran University Medical Faculty Hospital Obstetrics Clinic between January 2018 and January 2019. A total of 60 patients were included in the study, and the patients were divided into two groups: CSP group (n = 30) and healthy pregnant group as the control group (n = 30). The blood samples were taken from the participants between 7 - 12 weeks of gestation. Twentyseven carnitines and their esters and 14 FAAs were analysed by liquid chromatography – mass spectrometry (LC-MS/MS). Results: The mean plasma concentrations of some carnitines, including C2, C5, C5-OH, C5-DC, C6, C8-1, C12, C14, C14- 1, C14-2, C16, C16-1, C18, and C18-1 were significantly higher in CSP group than in the control group. However, other carnitines, including C0, C3, C4, C4-DC, C5-1, C6-DC, C8, C8-DC, C10, C10-1, C18-1-OH, and C18-2 were similar in both groups. The plasma levels of some FAAs, including Methyl Glutaryl, Leu, Met, Phe, Arg, Orn, and Glu values were significantly higher in CSP group than in the control group. However, there was no statistically significance in other FAA levels, including Val, Asa, Tyr, Asp, Ala, Cit, and Gly between the two groups. Additionally, Pearson’s correlation analysis showed that there were significantly positive correlations between many FAA and carnitine values. Conclusion: Since several plasma carnitine and FAA levels were higher in CSP group than in the control group, we think that scar pregnancy increases metabolic need for myometrial invasion. Also, we think that these results may be useful in clinical practice for CSP diagnosis.

Plasma-Free Amino Acid Profiling of Nasal Polyposis Patients

2020 ◽  
Vol 22 (9) ◽  
pp. 657-662 ◽  
Author(s):  
Mustafa Celik ◽  
Alper Şen ◽  
İsmail Koyuncu ◽  
Ataman Gönel
Keyword(s):  
Amino Acids ◽  
Amino Acid ◽  
Free Amino Acid ◽  
Free Amino ◽  
Nasal Polyposis ◽  
Amino Acid Profile ◽  
Healthy Control Group ◽  
Control Group ◽  
Healthy Control ◽  
History Of

Aim and Objective:: To determine the mechanisms present in the etiopathogenesis of nasal polyposis. It is not clear whether amino acids contribute in a causal way to the development of the disease. Therefore, the aim of this study was to determine the plasma-free amino acid profile in patients with nasal polyposis and to compare the results with a healthy control group. Materials and Methods:: This was a prospective controlled study that took place in the Otolaryngology Department at the Harran University Faculty of Medicine between April 2017 and April 2018. Plasmafree amino acid profile levels were studied in serum samples taken from a patient group and a healthy control group. Patients who were diagnosed with bilateral diffuse nasal polyposis and were scheduled for surgical interventions were included in this study. Individuals whose age, gender, and body mass index values were compatible with that of the patient group and who did not have any health problems were included in the control group. All the participants whose levels of plasma-free amino acid were thought to be affected by one or more of the following factors were excluded from the study: smoking and alcohol use, allergic rhinitis presence, the presence of acute or chronic sinusitis, a history of endoscopic sinus surgery, unilateral nasal masses, a history of chronic drug use, systemic or topical steroid use in the last three months for any reason, and liver, kidney, hematological, cardiovascular, metabolic, neurological, or psychiatric disorders or malignancies. Results: In patients with nasal polyposis, 3-methyl histidine (3-MHIS: nasal polyposis group (ng) = 3.22 (1.92 – 6.07); control group (cg) = 1.21 (0.77 – 1.68); p = 0.001); arginine (arg: ng = 98.95 (70.81 – 117.75); cg = 75.10 (54.49 – 79.88); p = 0.005); asparagine (asn: ng = 79.84 (57.50 – 101.44); cg = 60.66 (46.39 – 74.62); p = 0.021); citrulline (cit: ng = 51.83 (43.81 – 59.78); cg = 38.33 (27.81 – 53.73); p = 0.038); cystine (cys: ng = 4.29 (2.43 – 6.66); cg = 2.41 (1.51 – 4.16); p = 0.019); glutamic acid (glu: ng = 234.86 (128.75 – 286.66); cg = 152.37 (122.51 – 188.34); p = 0.045); histidine (his: ng = 94.19 (79.34 – 113.99); cg = 74.80 (62.76 – 98.91); p = 0.018); lysine (lys: ng = 297.22 (206.55 – 371.25); cg = 179.50 (151.58 – 238.02); p = 0.001); ornithine (ng = 160.62 (128.36 – 189.32); cg = 115.91 (97.03 – 159.91); p = 0.019); serine (ser: ng = 195.15 (151.58 – 253.07); cg = 83.07 (67.44 – 92.44); p = 0.001); taurine (tau: ng = 74.69 (47.00 – 112.13); cg = 53.14 (33.57 – 67.31); p = 0.006); tryptophan (trp: ng = 52.31 (33.81 – 80.11); cg = 34.44 (25.94 – 43.07); p = 0.005), homocitrulline (ng = 1.75 (1.27 – 2.59); cg = 0.00 (0.00 – 0.53); p = 0.001); norvaline (ng = 6.90 (5.61 – 9.18); cg = 4.93 (3.74 – 7.13); p = 0.021); argininosuccinic acid (ng = 14.33 (10.06 – 25.65); cg = 12.22 (5.77 – 16.87) p = 0.046); and plasma concentrations were significantly higher than in the healthy control group (p <0.05). However, the gamma-aminobutyric acid (gaba: ng = 0.16 (0.10 – 0.24); cg = 0.21 (0.19 – 0.29); p = 0.010) plasma concentration was significantly lower in the nasal polyposis group than in the healthy control group. Conclusion: In this study, plasma levels of 15 free amino acids were significantly higher in the nasal polyposis group than in the healthy control group. A plasma level of 1 free amino acid was found to be significantly lower in the nasal polyposis group compared to the healthy control group. Therefore, it is important to determine the possibility of using the information obtained to prevent the recurrence of the condition and to develop effective treatment strategies. This study may be a milestone for studies of this subject. However, this study needs to be confirmed by further studies conducted in a larger series.

scholarly journals Effects of Electrical Stimulation on the Peptide and Free Amino Acid Contents of Beef Homogenate and Sarcoplasma during Storage

1994 ◽  
Vol 65 (11) ◽  
pp. 1034-1043
Author(s):  
Masayuki MIKAMI ◽  
Mari NAGAO ◽  
Mitsuo SEKIKAWA ◽  
Hiroyuki MIURA ◽  
Yasuhisa HONGO
Keyword(s):  
Electrical Stimulation ◽  
Amino Acid ◽  
Free Amino Acid ◽  
Free Amino ◽  
Amino Acid Contents
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