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We present a one-year-old girl with maple syrup urine disease with dermatitis secondary to the restriction of amino acids as part of the treatment. We present the clinical evolution and histopathological correlation.
This article was published in the following journal.
Name: Dermatology online journal
The capacity to metabolize proteins is closely related to the hepatic functional reserve in patients with chronic liver disease, and hypoalbuminemia and hyperammonemia develop along with hepatic disea...
The two branched peptides (AAHAWG)-PWT2 and (HAWG)-PWT2 where synthesized by mounting linear peptides on a cyclam-based scaffold (PWT2), provided with four maleimide chains, through a thio-Michael rea...
Many patients develop a prolonged decrease of muscle strength after total hip arthroplasty (THA) despite their reconstructed hip joint. Physical exercise combined with branched-chain amino acid (BCAA)...
Circulating branched-chain amino acids (BCAAs, isoleucine, leucine, valine) and aromatic amino acids (AAAs, tyrosine and phenylalanine) predicted type 2 diabetes mellitus (T2DM) risk in a Caucasian po...
Sarcopenia is closely related to short-term outcomes of surgery and long-term prognosis. After gastrectomy, a decrease in muscle strength occurs because of insufficient nutrient intake and disturbed d...
In the subgroup of patients with sarcopenia, regardless of their body mass index, we want to verify the real impact of nutritional supplements with branched chain amino acids on the standa...
Protein-calorie malnutrition is frequently observed in patients with advanced liver cirrhosis. There have been continued interests in potential benefits of long-term oral branched-chain am...
This is study to investigate the plasma free amino acids profile in patients with decompensated liver cirrhosis and hepatic encephalopathy and its relation to the nutritional state of thes...
Seventy-eight recreational athletes who never resistance trained will be randomly assigned into three groups of twenty-six. Branched-chain amino acid will be supplemented for 18days at 0, ...
With aging, there is a decrease in muscle mass and function especially in the energy storehouses of cells called mitochondria. Amino acids, the building blocks of protein, and insulin hav...
An autosomal recessive disorder of fatty acid oxidation, and branched chain amino acids (AMINO ACIDS, BRANCHED-CHAIN); LYSINE; and CHOLINE catabolism, that is due to defects in either subunit of ELECTRON TRANSFER FLAVOPROTEIN or its dehydrogenase, electron transfer flavoprotein-ubiquinone oxidoreductase (EC 126.96.36.199).
Amino acids which have a branched carbon chain.
An essential branched-chain amino acid important for hemoglobin formation.
A nutritional condition produced by a deficiency of VITAMIN B 6 in the diet, characterized by dermatitis, glossitis, cheilosis, and stomatitis. Marked deficiency causes irritability, weakness, depression, dizziness, peripheral neuropathy, and seizures. In infants and children typical manifestations are diarrhea, anemia, and seizures. Deficiency can be caused by certain medications, such as isoniazid.
A 20-carbon branched chain fatty acid. In phytanic acid storage disease (REFSUM DISEASE) this lipid may comprise as much as 30% of the total fatty acids of the plasma. This is due to a phytanic acid alpha-hydroxylase deficiency.
Dermatitis means an inflammation of the skin. Contact dermatitis is a term used when this inflammation is caused by contact with something in the environment. The changes of dermatitis seen in the skin take the form of eczema; hence it may sometimes be r...
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...
Benign Prostatic Hyperplasia (BPH) Erectile Dysfunction Urology Urology is the branch of medicine concerned with the urinary tract and diseases that affect it. Examples include urethritis, urethrostenosis and incontinence. Urology is a su...