VFP Database Management System was applied in the establishment of the first Chinese Medicine Leaf Morphological-venation Pattern (LMVP) Identification Database Management. It stores 46 species of Chinese medicine confusing LMVP identification criteria (characteristics) and its 10 kinds of basic information, as well as 117 families with a total of 358 species of traditional Chinese medicine (TCM) and plant references document when study-ing in the TCM identification and methodology. It can provide query identification criteria (features), literature, and data for statistical analysis. It is the technology platform for the implementation, promotion and improvement of LMVP identification method, and further development on LMVP data mining. This database has a simple structure, multiple functions and applications, convenient management and maintenance, which are suitable for teaching.

Background: and PurposeHyperekplexia (HPX), a rare neurogenetic disorder, is classically characterized by neonatal hypertonia, exaggerated startle response provoked by the sudden external stimuli and followed by a shortly general stiffness. Glycine receptor alpha 1 (GLRA1) is the major pathogenic gene of the disease. We described the clinical manifestations of genetically confirmed HPX patients and made a literature review of GLRA1-related HPX to improve the early recognition and prompt the management of the disorder. Methods: Extensive clinical evaluations were analyzed in 4 Chinese HPX patients from two unrelated families. Next generation sequencing was conducted in the probands. Sanger sequence and segregation analysis were applied to confirm the findings. Results: All four patients including 3 males and 1 female presented with excessive startle reflex, a cautious gait and recurrent falls. Moreover, startle episodes were dramatically improved with the treatment of clonazepam in all cases. Exome sequencing revealed 2 homozygous GLRA1 mutations in the patients. The mutation c.1286T>A p.I429N has been previously reported, while c.754delC p.L252* is novel. Conclusions HPX is a treatable disease, and clonazepam is the drug of choice. By studying and reviewing the disorder, we summarized the phenotype, expanded the genotype spectrum, and discussed the possible pathogenic mechanisms to enhance the understanding and recognition of the disease. Early awareness of the disease is crucial to the prompt and proper administration, as well as the genetic counseling.