[Project to Decrease the Incidence of Neonatal Hypothermia in the Newborn Center].

BACKGROUND & PROBLEMS: An investigation found that 66.7% of the neonatal hypothermia (body temperature < 36.5°C) cases diagnosed within one hour of transfer from the delivery room in our hospital were affected by a significantly increased risk of physiological abnormalities, which subsequently increased their risk for mortality. Therefore, monitoring and maintaining the normal body temperature of newborn infants are vital in infant care. PURPOSE: This project aimed to improve the current situation of neonatal hypothermia. RESOLUTION: This project was implemented from Oct. 1, 2016 to Oct. 31, 2017 and used several approaches to improve neonatal hypothermia. A neonatal hypothermia caring protocol was developed and the infant admission materials were standardized; the infant hypothermia alert card and posters were displayed in easy-to-notice locations; an in-service training course on neonatal hypothermia was provided; and an infant hypothermia care checklist was tabulated for examination and recognition. RESULTS: After the implementation of this project, the average time required to raise the body temperature of infants to normal (36.5°C) was 1.5 hours, which was 2 hours faster than the pre-project time of 3.5 hours. Moreover, the time needed to raise the body temperature to normal was one hour for newborn infants with birthweights ≥ 2,500 grams, which was one hour faster than the pre-project time of two hours, and 1.5 hours for newborn infants with birthweights < 2,500 grams, which was three hours faster than the pre-project time of 4.5 hours. The goals of this project were effectively achieved in both groups. CONCLUSIONS: Neonatal hypothermia is an important issue affecting the health status of newborn infants. This project strengthened the awareness of nurses regarding neonatal hypothermia and is worthwhile to be implemented in clinical neonatal care.

Mutation spectrum of Charcot-Marie-Tooth disease among the Han Chinese in Taiwan.

OBJECTIVE: Charcot-Marie-Tooth disease (CMT) is a clinically and genetically heterogeneous group of inherited neuropathies. Mutations in more than 90 genes have been implicated in CMT; however, the mutational spectrum of CMT in Chinese population remains obscure. This study aims to provide a comprehensive overview of the frequency of mutations in Taiwanese patients with CMT and look for genotype-phenotype correlations. METHODS: Mutational analyses were performed on 427 unrelated Taiwanese patients with CMT by polymorphic microsatellite markers analysis or real-time fluorescent PCR for PMP22 duplication, Sanger sequencing for GJB1 mutations, and targeted sequencing covering 124 genes causing or relevant to inherited neuropathies. We also correlated the genotypes with the phenotypic features, such as age at disease onset and ulnar motor nerve conduction velocity. RESULTS: Pathogenic mutations were identified in 312 patients (73.1%; 312/427), including 208 patients with a PMP22 duplication, 40 patients with a GJB1 mutation, and 64 patients with a mutation in one of other 18 CMT genes. A confirmed molecular diagnosis was achieved in 84.4% (266/315) of the patients with demyelinating CMT and 41.1% (46/112) of the patients with axonal CMT. Mutations in MPZ, MFN2, or NEFL are the most frequent disease causes in patients with infantile-onset CMT (≤2 years), while PMP22 duplications and mutations in GJB1, MFN2, or MPZ are the frequent causes among patients with childhood- or adolescence-onset CMT (3-9 years). INTERPRETATION: This study provides a genotype-phenotype landscape of CMT in Taiwan and highlights the unique spectrum of CMT genes frequencies among patients of Chinese origin.

A compartmentalized culture device for studying the axons of CNS neurons.

We report here the development of a compartmentalized culture device that allows the spatial separation of the somatodendrites and axons of central nervous system (CNS) neurons. The device consists of two compartments separated by a septum constructed by attaching a porous polycarbonate track etch (PCTE) filter on top of a microchannel-filled polydimethylsiloxane (PDMS) membrane. The surface and microchannels of the septum are coated and filled, respectively, with materials that support neuron growth and neurite migration. When rat hippocampal neurons are cultured in the top compartment, axons are the only processes that can migrate through the septum to the bottom compartment. The axons in the bottom compartment can be studied directly in real-time or through immunofluorescence staining after fixation. Axons containing ∼3 µg protein can be isolated from each device for biochemical analyses. In addition, the septum also impedes the movement of small molecules between the top and bottom compartments. This feature allows the somatodendrites and axons of neurons, which occupy the top and bottom compartments of the device, respectively, to be manipulated independently. The potential applications of the device as a tool in diverse studies concerning neuronal axons and in screening reagents that regulate axonal functions have also been discussed.