Conscious sedation as a potential optimal anesthetic regimen for endoscopic retrograde cholangiopancreatography in elderly frail patients: A prospective, single-blind, randomized controlled trial.
Authors: Li C, Zeng N, Wang F, Zhang Y, Ma D, Hou H
Journal: Saudi journal of anaesthesia
mental health
psychology
open access
Abstract
Aromatic ʟ‐amino acid decarboxylase (AADC, EC 4.1.1.28) deficiency (OMIM #608643) is a rare, severe neurological disorder caused by pathogenic variants in the dopa decarboxylase gene., The incidence of AADC deficiency has been estimated to range from 1:26 000 in East Asians, and 1:500 000 in Europeans to 1:1.4 million persons worldwide.
encodes the AADC enzyme, which is the catalyst for the synthesis of the monoamine neurotransmitters dopamine (and subsequently, norepinephrine and epinephrine) and serotonin (Figure )., , , Metabolic pathways of biogenic monoamine neurotransmitters. 5‐HTP, 5 hydroxytryptophan; 5‐HIAA, 5‐hydroxyindoleacetic acid; AADC, aromatic ʟ‐amino acid decarboxylase; BH, dihydrobiopterin; BH, tetrahydrobiopterin; GTP, guanosine triphosphate; HVA, homovanillic acid; L‐DOPA, levodopa; MHPG, 3‐methoxy‐4‐hydroxyphenylglycol; VMA, vanillylmandelic acid. Impaired monoamine neurotransmitter synthesis caused by AADC deficiency generally manifests as global motor and intellectual disabilities, orthopedic and cardiopulmonary sequelae, and premature death., , , , Reductions in dopamine are associated with abnormal motor control and regulation (e.g., movement disorders such as hypotonia, dystonia, and oculogyric crisis [OGC]), cognitive function, emotional regulation, and endocrine‐mediated functions. Autonomic nervous system symptoms in AADC deficiency may also arise, owing to impaired epinephrine and norepinephrine synthesis consequent to reduced dopamine levels; these symptoms include ptosis, hyperhidrosis, temperature dysregulation, hypoglycemia, gastrointestinal symptoms, and cardiopulmonary dysregulation., Finally, reduced serotonin levels associated with this condition have the potential to affect learning and memory, circadian rhythms, and cardiovascular and neuroendocrine regulation., The symptomatic presentation of AADC deficiency is varied; whereas some patients may have subtle clinical presentations, others have profound impairment., , , , ,
encodes the AADC enzyme, which is the catalyst for the synthesis of the monoamine neurotransmitters dopamine (and subsequently, norepinephrine and epinephrine) and serotonin (Figure )., , , Metabolic pathways of biogenic monoamine neurotransmitters. 5‐HTP, 5 hydroxytryptophan; 5‐HIAA, 5‐hydroxyindoleacetic acid; AADC, aromatic ʟ‐amino acid decarboxylase; BH, dihydrobiopterin; BH, tetrahydrobiopterin; GTP, guanosine triphosphate; HVA, homovanillic acid; L‐DOPA, levodopa; MHPG, 3‐methoxy‐4‐hydroxyphenylglycol; VMA, vanillylmandelic acid. Impaired monoamine neurotransmitter synthesis caused by AADC deficiency generally manifests as global motor and intellectual disabilities, orthopedic and cardiopulmonary sequelae, and premature death., , , , Reductions in dopamine are associated with abnormal motor control and regulation (e.g., movement disorders such as hypotonia, dystonia, and oculogyric crisis [OGC]), cognitive function, emotional regulation, and endocrine‐mediated functions. Autonomic nervous system symptoms in AADC deficiency may also arise, owing to impaired epinephrine and norepinephrine synthesis consequent to reduced dopamine levels; these symptoms include ptosis, hyperhidrosis, temperature dysregulation, hypoglycemia, gastrointestinal symptoms, and cardiopulmonary dysregulation., Finally, reduced serotonin levels associated with this condition have the potential to affect learning and memory, circadian rhythms, and cardiovascular and neuroendocrine regulation., The symptomatic presentation of AADC deficiency is varied; whereas some patients may have subtle clinical presentations, others have profound impairment., , , , ,