Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare pediatric neuro-metabolic disease in children. Due to the lack of an animal model, its pathogenetic mechanism is poorly understood. To study the role of AADC in brain development, a zebrafish model of AADC deficiency was generated. We identified an aadc gene homolog, dopa decarboxylase (ddc), in the zebrafish genome. Whole-mount in situ hybridization analysis showed that the ddc gene is expressed in the epiphysis, locus caeruleus, diencephalic catecholaminergic clusters, and raphe nuclei of 36-h post-fertilization (hpf) zebrafish embryos. Inhibition of Ddc by AADC inhibitor NSD-1015 or anti-sense morpholino oligonucleotides (MO) reduced brain volume and body length. We observed increased brain cell apoptosis and loss of dipencephalic catecholaminergic cluster neurons in ddc morphants (ddc MO-injected embryos). Seizure-like activity was also detected in ddc morphants in a dose-dependent manner. ddc morphants had less sensitive touch response and impaired swimming activity that could be rescued by injection of ddc plasmids. In addition, eye movement was also significantly impaired in ddc morphants. Collectively, loss of Ddc appears to result in similar phenotypes as that of ADCC deficiency, thus zebrafish could be a good model for investigating pathogenetic mechanisms of AADC deficiency in children.

Pressure injury is the most important issue facing paralysis patients and the elderly, especially in long-term care or nursing. A new interfacial pressure sensing system combined with a flexible textile-based pressure sensor array and a real-time readout system improved by the Kalman filter is proposed to monitor interfacial pressure progress in the cardiac operation. With the design of the Kalman filter and parameter optimization, noise immunity can be improved by approximately 72%. Additionally, cardiac operation patients were selected to test this developed system for the direct correlation between pressure injury and interfacial pressure for the first time. The pressure progress of the operation time was recorded and presented with the visible data by time- and 2-dimension-dependent characteristics. In the data for 47 cardiac operation patients, an extreme body mass index (BMI) and significantly increased pressure after 2 h are the top 2 factors associated with the occurrence of pressure injury. This methodology can be used to prevent high interfacial pressure in high-risk patients before and during operation. It can be suggested that this system, integrated with air mattresses, can improve the quality of care and reduce the burden of the workforce and medical cost, especially for pressure injury.