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Background This study aimed to describe the clinical, biochemical, and molecular characteristics of Chinese patients with holocarboxylase synthetase (HLCS) deficiency, and to investigate the mutation spectrum of HCLS deficiency as well as their potential correlation with phenotype. Methods A total of 28 patients with HLCS deficiency were enrolled between 2006 and 2021. Clinical and laboratory data were reviewed retrospectively from medical records. Results Among the 28 patients, six patients underwent newborn screening, of which only one was missed. Therefore, 23 patients were diagnosed because of disease onset. Among all the patients, 24 showed varying degrees of symptoms such as rash, vomiting, seizures, and drowsiness, while only four cases remained asymptomatic nowadays. The concentration of 3-hydroxyisovalerylcarnitine (C5-OH) in blood and pyruvate, 3-hydroxypropionate, methylcitric acid, 3-hydroxyvaleric acid, 3-methylcrotonylglycine in urine were increased greatly among affected individuals. After prompt supplement of biotin, both the clinical and biochemical symptoms were dramatically resolved and nearly all patients developed normal intelligence and physique on follow-up. DNA sequencing revealed 12 known and 6 novel variants in the HLCS gene of patients. Among them, the variant of c.1522C > T was the most common. Conclusions Our findings expanded the spectrum of phenotypes and genotypes for HLCS deficiency in Chinese populations and suggested that with timely biotin therapy, patients with HLCS deficiency showed low mortality and optimistic prognosis. Newborn screening is crucial for early diagnosis, treatment, and long-term outcomes.

Immunoglobulin light chain (AL) amyloidosis is a rare clonal plasma cell disorder with high rate of missed diagnosis, misdiagnosis and mortality. Conventional assays, such as serum immunofixation electrophoresis (IFE) and serum free light chain (FLC) assay, are unable to accurately detect low concentrations of monoclonal protein (M protein), especially as a patient’s renal function deteriorates. The heavy/light chain (HLC) assay, a relatively new method, can quantify intact immunoglobulins in serum and has proven to be valuable in the diagnosis and monitoring of multiple myeloma (MM). However, there is limited research on its application in AL amyloidosis. In this study, we evaluate the value of HLC assay in AL amyloidosis patients at different disease stages, and compare it to the performance of IFE and FLC assay. Among 40 untreated patients, 34 (85%) were positive for IFE, 34 (85%) had an abnormal free light chain ratio (FLCr), and 31 (78%) had an abnormal heavy light chain ratio (HLCr). Among 67 serum samples obtained from 44 treated patients, 57 (85%) were positive for IFE, 9 (13%) had abnormal FLCr, and 45 (67%) had abnormal HLCr. There were 1 (14%) of 7 patients in complete response (CR), 17 (68%) of 25 patients in very good partial response (VGPR), 9 (82%) of 11 patients in partial response (PR) and 6 (75%) of 8 patients in no response (NR) showed an abnormal HLCr. Our findings identified the potential value of the HLC assay in the detection of M proteins and response and serologic residual disease monitoring.

Background Anopheles darlingi is a primary malaria vector in the Peruvian Amazon, yet characterization of behavioural traits contributing to human-vector contact is limited. Additionally, studies comparing key behaviours of wild-type to colonized An. darlingi populations are minimal. This study compared resting and entry behaviour between these two types of populations. Specific objectives were to use experimental huts to (1) evaluate and compare indoor resting behaviours of wild-type and colonized An. darlingi populations; (2) quantify An. darlingi house entry rates into interception traps in relation to protected Human Landing collection (HLC). Methods The study was conducted in Zungarococha village, Loreto, Peru. Prior to hut evaluations, An. darlingi biting activity and population dynamics were evaluated using HLC outside local homes from June 2014 to May 2015. Indoor resting location (window, door, wall, roof) of wild caught and colonized An. darlingi was evaluated in three experimental huts. Controlled indoor releases were performed for 6 days each month from March to August 2015. Wild An. darlingi hourly house-entry rates were quantified using interception traps affixed to an experimental hut and indoor HLC from May to August 2015. Two collectors were positioned inside huts to generate host-seeking cues during evaluations. Results Anopheles darlingi had a bimodal outdoor biting pattern with two peaks at 1800 h and 2200 h. HLC densities were associated with Amazon River levels measured nearby Iquitos city. Colonized An. darlingi preferred to rest in lower parts of the door (29.8%), roof (12.7%), and window (11.8%) which was similar to wild caught An. darlingi which preferred to rest in low door (32.7%), window (14.0%), and roof (13.0%). Wild An. darlingi entry behaviour peaked from 2200 to 2300 h, this was clearly observed when collection densities increased, varying between 2300 and 2400 h at low collection densities. Capture rates from interception traps were lower compared to indoor HLC rates from adjacent experimental huts and local houses. Conclusions Results from this study provide useful insights into An. darlingi resting and entry behaviour in a malaria endemic area in the Peruvian Amazon and inform on the use of colonized mosquitos in vector behavioural studies. This information is relevant to malaria epidemiology and will be useful to evaluate new tools for malaria control programmes.

Background Effective malaria surveillance requires detailed assessments of mosquitoes biting indoors, where interventions such as insecticide-treated nets work best, and outdoors, where other interventions may be required. Such assessments often involve volunteers exposing their legs to attract mosquitoes [i.e., human landing catches (HLC)], a procedure with significant safety and ethical concerns. Here, an exposure-free, miniaturized, double-net trap (DN-Mini) is used to assess relationships between indoor–outdoor biting preferences of malaria vectors, Anopheles arabiensis and Anopheles funestus , and their physiological ages (approximated by parity and insemination states). Methods The DN-Mini is made of UV-resistant netting on a wooden frame and PVC base. At 100 cm × 60 cm × 180 cm, it fits indoors and outdoors. It has a protective inner chamber where a volunteer sits and collects host-seeking mosquitoes entrapped in an outer chamber. Experiments were conducted in eight Tanzanian villages using DN-Mini to: (a) estimate nightly biting and hourly biting proportions of mosquitoes indoors and outdoors; (b) compare these proportions to previous estimates by HLC in same villages; and, (c) compare distribution of parous (proxy for potentially infectious) and inseminated mosquitoes indoors and outdoors. Results More than twice as many An. arabiensis were caught outdoors as indoors (p < 0.001), while An. funestus catches were marginally higher indoors than outdoors (p = 0.201). Anopheles arabiensis caught outdoors also had higher parity and insemination proportions than those indoors (p < 0.001), while An. funestus indoors had higher parity and insemination than those outdoors (p = 0.04). Observations of indoor-biting and outdoor-biting proportions, hourly biting patterns and overall species diversities as measured by DN-Mini, matched previous HLC estimates. Conclusions Malaria vectors that are behaviourally adapted to bite humans outdoors also have their older, potentially infectious sub-populations concentrated outdoors, while those adapted to bite indoors have their older sub-populations concentrated indoors. Here, potentially infectious An. arabiensis more likely bite outdoors than indoors, while potentially infectious An. funestus more likely bite indoors. These observations validate previous evidence that even outdoor-biting mosquitoes regularly enter houses when young. They also demonstrate efficacy of DN-Mini for measuring indoor–outdoor biting behaviours of mosquitoes, their hourly biting patterns and epidemiologically relevant parameters, e.g., parity and insemination status, without exposure to volunteers. The trap is easy-to-use, easy-to-manufacture and affordable (prototypes cost ~ 100 US$/unit).

Dengue fever is one of the biggest threats to public health in China, causing huge disease burden and economic loss. Aedes -mosquito surveillance could be a cornerstone for predicting the risk of Aedes -borne diseases and evaluating the effect of vector management during diseases outbreaks. The human landing catch (HLC) method is regarded as the “gold standard” for catching Aedes mosquitoes, but it potentially exposes field professionals to vectors of known or unknown pathogens. Human-baited double net (HDN) was recommended to replace HLC for emergency monitoring in China when Aedes -borne diseases break out, but it had been reported with low efficiency for capturing Aedes mosquitoes. In this study, we compared HLC with HDN and BG traps for field Aedes albopictus monitoring, with the aim of evaluating the effectiveness of HDN replacing HLC and finding an effective and safe alternative to the HLC for monitoring Aedes albopictus . Six sites in Hangzhou, Shaoxing, and Yiwu, Zhejiang Province, China, were chosen to conduct outdoor HLC, HDN, and BG trap catches from June to October 2021. The tests were performed 3 h apart: 8:30–9:30 AM, 16:30–17:30 PM, and 17:30–18:30 PM. A total of 2330 adult mosquitoes were collected, and Aedes albopictus was the most abundant species in all three catches with 848(98.95%), 559(97.39%) and 867 (96.44%) caught in HLC, HDN and BG traps respectively. Compared to HLC, HDN collected significantly less Ae. albopictus and Ae. albopictus females per trapping period ( P  < 0.001, P  < 0.001), whereas no statistical differences were found between the HLC and BG trap ( P  = 0.970, P  > 0.05). Statistically significant positive spatial correlations for Ae. albopictus sampling was found between HLC and HDN traps ( r  = 0.543, P  < 0.001) and HLC and BG traps ( r  = 0.658, P  < 0.001). In conclusion, both the BG trap and HDN have a significant positive spatial correlation with HLC, making them safer alternatives to HLC for Ae. albopictus monitoring in China. However, with better a sampling efficiency, being less labor intensive, and no human-baited attraction bias, the BG trap could be a better choice than the HDN trap.

Background Human landing catches (HLC) are an entomological collection technique in which humans are used as attractants to capture medically relevant host-seeking mosquitoes. The use of this method has been a topic of extensive debate for decades mainly due to ethical concerns. Many alternatives to HLC have been proposed; however, no quantitative review and meta-analysis comparing HLC to outdoor alternative trapping methods has been conducted. Methods A total of 58 comparisons across 12 countries were identified. We conducted a meta-analysis comparing the standardized mean difference of Anopheles captured by HLC and alternative traps. To explain heterogeneity, three moderators were chosen for analysis: trap type, location of study, and species captured. A meta-regression was fit to understand how the linear combination of moderators helped in explaining heterogeneity. The possibility of biased results due to publication bias was also explored. Results Random-effects meta-analysis showed no statistically significant difference in the mean difference of Anopheles collected. Moderator analysis was conducted to determine the effects of trap type, geographical location of study, and the species of Anopheles captured. On average, tent-based traps captured significantly more Anopheles than outdoor HLC (95% CI: [− .9065, − 0.0544]), alternative traps in Africa captured on average more mosquitoes than outdoor HLC (95% CI: [− 2.8750, − 0.0294]), and alternative traps overall captured significantly more Anopheles gambiae s.l. than outdoor HLC (95% CI: [− 4.4613, − 0.2473]) on average. Meta-regression showed that up to 55.77% of the total heterogeneity found can be explained by a linear combination of the three moderators and the interaction between trap type and species. Subset analysis on An. gambiae s.l. showed that light traps specifically captured on average more of this species than HLC (95% CI: [− 18.3751, − 1.0629]). Publication bias likely exists. With 59.65% of studies reporting p-values less than 0.025, we believe there is an over representation in the literature of results indicating that alternative traps are superior to outdoor HLC. Conclusions Currently, there is no consensus on a single “magic bullet” alternative to outdoor HLC. The diversity of many alternative trap comparisons restricts potential metrics for comparisons to outdoor HLC. Further standardization and specific question-driven trap evaluations that consider target vector species and the vector control landscape are needed to allow for robust meta-analyses with less heterogeneity and to develop data-driven decision-making tools for malaria vector surveillance and control.

Farm households around the world are increasingly exposed to both external and internal shocks and stressors. Enhancing the resilience of farm households to frequent disturbances holds paramount importance in fostering the sustainability of their livelihoods and the revitalization of rural areas. Based on 1500 household samples from 14 contiguous poverty-stricken areas (CPSA) in China, this study explores the causal pathways between livelihood capitals of farm households and rural site conditions of rural communities, as well as quantifying their impacts on farm households’ livelihood resilience using structural equation models. In particular, the livelihood resilience of farm households is measured based on the “Exposure-Sensitivity-Adaptability” framework. The results show that livelihood resilience is positively represented by exposure and adaptability, but is negatively correlated with sensitivity. Specifically, households with lower mean health and higher dependency ratio are more sensitive to risks, while exposure to agroforestry pests and diseases will lead farm households to diversify their livelihood activities and increase crop and livestock variety to enhance their adaptability. The livelihood capital of farm households has a significant positive effect on livelihood resilience ( β  = 0.874, p  < 0.001). Rural site conditions have both significant direct and indirect impacts on livelihood resilience, with the direct impact ( β  =  − 0.207, p  < 0.05) being negative and a bit larger than the positive indirect impact ( β  = 0.163, p  < 0.05), as mediated by livelihood capital. The government should, therefore, invest more in health insurance, education and training, financial support, and infrastructure, and implement village planning to enhance both the quality of household livelihood capitals and rural site conditions in CPSA.

Biotin (vitamin B7, or vitamin H) is a water-soluble B-vitamin that functions as a cofactor for carboxylases, i.e., enzymes involved in the cellular metabolism of fatty acids and amino acids and in gluconeogenesis; moreover, as reported, biotin may be involved in gene regulation. Biotin is not synthesized by human cells, but it is found in food and is also produced by intestinal bacteria. Biotin status/homeostasis in human individuals depends on several factors, including efficiency/deficiency of the enzymes involved in biotin recycling within the human organism (biotinidase, holocarboxylase synthetase), and/or effectiveness of intestinal uptake, which is mainly accomplished through the sodium-dependent multivitamin transporter. In the last years, administration of biotin at high/“pharmacological” doses has been proposed to treat specific defects/deficiencies and human disorders, exhibiting mainly neurological and/or dermatological symptoms and including biotinidase deficiency, holocarboxylase synthetase deficiency, and biotin–thiamine-responsive basal ganglia disease. On the other hand, according to warnings of the Food and Drug Administration, USA, high biotin levels can affect clinical biotin-(strept)avidin assays and thus lead to false results during quantification of critical biomarkers. In this review article, recent findings/advancements that may offer new insight in the abovementioned research fields concerning biotin will be presented and briefly discussed.

Accurate forecasting of a building thermal performance can help to optimize its energy consumption. In addition, obtaining the Heat Loss Coefficient (HLC) allows characterizing the thermal envelope of the building under conditions of use. The aim of this work is to study the thermal inertia of a building developing a new methodology based on Long Short-Term Memory (LSTM) neural networks. This approach was applied to the Rectorate building of the University of Basque Country (UPV/EHU), located in the north of Spain. A comparison of different time-lags selected to catch the thermal inertia has been carried out using the CV(RMSE) and the MBE errors, as advised by ASHRAE. The main contribution of this work lies in the analysis of thermal inertia detection and its influence on the thermal behavior of the building, obtaining a model capable of predicting the thermal demand with an error between 12 and 21%. Moreover, the viability of LSTM neural networks to estimate the HLC of an in-use building with an error below 4% was demonstrated.

Precise applications, including dual-antenna heading determination, have been flourishing research fields in global navigation satellite system (GNSS). However, due to the requirements for strong GNSS models and precise carrier phase measurements, single-frequency antennas with large phase pattern variations are difficult to be adopted. In this research, a search-based heading determination method is demonstrated and verified. A new ambiguity resolution (AR) method, namely baseline heading and length-constrained LAMBDA (HLC-LAMBDA), is also proposed to attempt the AR under a weak GNSS model as well as help identify a rough heading estimate among all listed heading candidates (HC). During the heading search (HS) process, all HCs are evaluated individually with relative positioning results between two antennas, and each HC being evaluated serves two functions: 1) helping correct the antenna-induced phase biases by applying a predetermined phase pattern with different attitudes, and 2) acting as a rough heading constraint in the HLC-LAMBDA method. The performance of the proposed method is analyzed in terms of the precision of heading and relative positioning solutions, the elimination of antenna-induced phase biases, and the computational efficiency; and test results utilizing a phase pattern measured in an anechoic chamber and the data in both static and kinematic cases are also shown, illustrating a consistent conclusion that, under the condition of single-frequency measurements and large phase pattern variations, the proposed method can provide heading solutions with a root mean square error (RMSE) of around 0.07°.