Anesthetic Care of Children With Gene Therapy for the Treatment of Aromatic L-Amino Acid Decarboxylase (AADC) Deficiency
Journal
Hu li za zhi The journal of nursing
Journal Volume
66
Journal Issue
1
Date Issued
2019-02
Author(s)
Li, Wen-Chun
Abstract
AADC deficiency is a rare autosomal recessive disease that may lead to movement disorder and/or autonomic dysfunction. The prognosis of AADC deficiency patients is poor, and most die in childhood. Gene therapy is the only method currently available to help alleviate related symptoms. Gene therapy involves the injection of adeno-associated viral (AV) vector into the basal ganglia of patients, helping transfer the AADC gene and leading to improved AADC production and higher numbers of neurotransmitters in the brain. Moreover, as patients are unable to produce catecholamine, they may also suffer from a disorder affecting the regulatory control of the autonomic nervous system, resulting in hypoglycemia, which often causes imbalances in thermoregulation and hemodynamic and functional adjustments during surgery. Furthermore, the use of analgesics and inhalational anesthetics increase the risks of hypotension and bradycardia. Thus, in addition to assessing cardiac and respiratory system functions, it is important to evaluate the patient's airway before administering anesthesia, as structural anomalies or other situations may be present that lead to difficulties in tracheal intubation. Various airway tools, anesthesia equipment, and alternative plans should thus be prepared to protect the airway and maintain vital signs. All of the abovementioned issues increase the risks of AADC deficiency patients undergoing general anesthesia. The development of gene transfection for genetic diseases is a future trend. This paper identifies possible problems and related responses in perioperative patient care during gene therapy. The authors hope that these experiences provide references for the administration of AADC and similar gene therapies in the future.
Subjects
anesthesia management; aromatic amino acid decarboxylase deficiency; gene therapy
SDGs
Type
journal article