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Pineapples are an important agricultural economic crop in Taiwan. Considerable human resources are required to protect pineapples from excessive solar radiation, which could otherwise lead to overheating and subsequent deterioration. Note that simple covering all of the fruit with a paper bag is not a viable solution, due to the fact that it makes it impossible to determine whether the fruit is ripe. This paper proposes a system by which to automate the detection of ripe pineapples. The proposed deep learning architecture enables detection regardless of lighting conditions, achieving accuracy of more than 99.27% with error of less than 2% at distances of 300 ~ 800 mm. This proposed system using an Nvidia TX2 is capable of 15 frames per second, thereby making it possible to mount the device on machines that move at walking speed.

This study quantified five perfluorocarboxylic acids (PFCAs) and two perfluorosulfonic acids in cereals, meats, seafood, eggs, pork liver, and milk in Taiwan using ultra-performance liquid chromatography-tandem mass spectrometry and evaluated the dietary exposure of the general population and pregnant women using per capita consumption and a questionnaire, respectively. Perfluorooctanoic acid (PFOA) and PFCAs of 10–12 carbons were found in almost all of the samples in considerable concentrations in rice and pork liver, reaching as high as 283 ng/g (PFOA in pork liver); the levels are two to three orders of magnitude higher than previous reports. Perfluorooctane sulfonate (PFOS), the most frequently mentioned perfluoroalkyl substance, was rarely detected in many food items (detection frequencies <20% in rice, flour, pork, chicken, salmon, squid, eggs, and milk) at <0.4 ng/g, except for beef, pork liver and some seafood (detection frequencies: 100%, GMs: 0.05–3.52 ng/g). Compared to populations in Western countries, people in Taiwan are exposed to much more perfluorohexanoic acid, PFOA, perfluorodecanoic acid, and perfluoroundecanoic acid (11.2, 85.1, 44.2, and 4.45 ng/kg b.w./day, respectively), mainly due to the higher contaminations in food. The exposure of 8.0 μg PFOA/person/day in the 95 percentile of pregnant women was due to their frequent consumption of pork liver. [Display omitted] •PFASs are ubiquitous in common raw foods in Taiwan.•High concentrations of long-chain PFASs were found in rice and pork liver.•PFOA is more abundant than PFOS.•Taiwan people are exposed to more PFCAs than Westerners.•Consumption of pork liver increases PFAS exposure in pregnant women.

Psidium guajava L. is an important tropical and subtropical fruit. Due to its geographical location and suitable climate, Taiwan produces Psidium guajava L. all year round. Quality standardization is therefore a crucial issue. The primary objective was to detect appearance defects on harvested fruits. We divided the defects into thirteen classes, including damage from pests, diseases, and humans. We obtained 189 Psidium guajava L. fruits from different farms and collected 1701 images as samples. The YOLO v4 pretrained network architecture achieved excellent performance in defect detection, including a false positive rate of 6.62%, a false negative rate of 5.03%, and accuracy of 88.15%. Moreover, in the detection of Colletotrichum gloeosporoides , Pestalotiopsis psidii , and Phyllosticta psidiicola , the false positive and false negative detection rates were less than 9%. The applicability of the model in real-time harvesting and grading operations was demonstrated by a minimum detectable defect size of 13 × 14 pixels and computation speed of 12 FPS demonstrated.

Background Mucopolysaccharidoses (MPSs) are a group of inherited metabolic diseases, which are characterized by the accumulation of glycosaminoglycans, and eventually lead to the progressive damage of various tissues and organs. Methods An epidemiological study of MPS in Taiwan was performed using multiple sources. The survival and diagnostic age for different types of MPS between 1985 and 2019 were evaluated. Results Between 1985 and 2019, there were 175 patients diagnosed with MPS disorders in the Taiwanese population, with a median diagnostic age of 3.9 years. There were 21 (12%), 78 (45%), 33 (19%), 32 (18%) and 11 (6%) patients diagnosed with MPS I, II, III, IV and VI, respectively, with median diagnostic ages of 1.5, 3.8, 4.7, 4.5 and 3.7 years, respectively. Diagnosis of MPS patients was significantly earlier in recent decades ( p  < 0.01). Pilot newborn screening programs for MPS I, II, VI, IVA, and IIIB were progressively introduced in Taiwan from 2016, and 48% (16/33) of MPS patients diagnosed between 2016 and 2019 were diagnosed by one of these screening programs, with a median diagnostic age at 0.2 years. For patients born between 2016 and 2019, up to 94% (16/17) were diagnosed with MPS via the newborn screening programs. At the time of this study, 81 patients had passed away with a median age at death of 15.6 years. Age at diagnosis was positively correlated with life expectancy ( p  < 0.01). Life expectancy also significantly increased between 1985 and 2019, however this increase was gradual ( p  < 0.01). Conclusions The life expectancy of Taiwanese patients with MPS has improved in recent decades and patients are being diagnosed earlier. Because of the progressive nature of the disease, early diagnosis by newborn screening programs and timely implementation of early therapeutic interventions may lead to better clinical outcomes.

Patients with end-stage renal disease are at risk of developing hyperkalemia and acidosis, both of which have disastrous sequelae during elective video-assisted thoracic surgery for lung cancer. Herein, we present a case where severe hyperkalemia and combined acidosis were incidentally found in a 68-year-old man with the end-stage renal disease after establishing one-lung ventilation during video-assisted lobectomy. There was no significant instability of vital signs, abnormality of perioperative electrocardiography, or malignant arrhythmia. Therefore, we arranged for related management promptly, and the surgery was relatively smooth. This incidental intraoperative hyperkalemia was thought to have resulted from one-lung ventilation and hypercarbia and/or metabolic acidosis. More frequent arterial blood gas analysis and aggressive blood potassium control during video-assisted thoracic surgery should be considered for patients with end-stage renal disease.

Background: The mucopolysaccharidoses (MPSs) are a group of rare lysosomal storage disorders characterized by the accumulation of glycosaminoglycans (GAGs) and which eventually cause progressive damage to various tissues and organs. We developed a feasible MPS screening algorithm and established a cross-specialty collaboration platform between medical geneticists and other medical specialists based on at-risk criteria to allow for an earlier confirmative diagnosis of MPS. Methods: Children (<19 years of age) with clinical signs and symptoms compatible with MPS were prospectively enrolled from pediatric clinics between July 2013 and June 2018. Urine samples were collected for a non-specific total GAG analysis using the dimethylmethylene blue (DMB) spectrophotometric method, and the quantitation of three urinary GAGs (dermatan sulfate (DS), heparan sulfate (HS), and keratan sulfate (KS)) was performed by liquid chromatography/tandem mass spectrometry (LC-MS/MS). The subjects with elevated urinary GAG levels were recalled for leukocyte enzyme activity assay and genetic testing for confirmation. Results: Among 153 subjects enrolled in this study, 13 had a confirmative diagnosis of MPS (age range, 0.6 to 10.9 years—three with MPS I, four with MPS II, five with MPS IIIB, and one with MPS IVA). The major signs and symptoms with regards to different systems recorded by pediatricians at the time of the decision to test for MPS were the musculoskeletal system (55%), followed by the neurological system (45%) and coarse facial features (39%). For these 13 patients, the median age at the diagnosis of MPS was 2.9 years. The false negative rate of urinary DMB ratio using the dye-based method for these 13 patients was 31%, including one MPS I, two MPS IIIB, and one MPS IVA. However, there were no false negative results with urinary DS, HS and KS using the MS/MS-based method. Conclusions: We established an at-risk population screening program for MPS by measuring urinary GAG fractionation biomarkers using the LC-MS/MS method. The program included medical geneticists and other medical specialists to increase awareness and enable an early diagnosis by detecting MPS at the initial onset of clinical symptoms.

Background: Beckwith-Wiedemann syndrome (BWS; OMIM 130650) is a rare overgrowth syndrome with tumor predisposition resulting from the abnormal expression or function of imprinted genes of the chromosome 11p15.5 imprinting gene cluster. The aim of this study was to identify the epigenotype-phenotype correlations of these patients using quantitative DNA methylation analysis. Methods: One hundred and four subjects with clinically suspected BWS were enrolled in this study. All of the subjects had been referred for diagnostic testing which was conducted using methylation profiling of H19-associated imprinting center (IC) 1 and KCNQ1OT1-associated IC2 in high-resolution melting analysis and methylation quantification with the MassARRAY assay. Correlations between the quantitative DNA methylation status and clinical manifestations of the enrolled subjects were analyzed. Results: Among the 104 subjects, 19 had IC2 hypomethylation, 2 had IC1 hypermethylation, and 10 had paternal uniparental disomy (pUPD). The subjects with IC2 hypomethylation were characterized by significantly more macroglossia but less hemihypertrophy compared to the subjects with pUPD (p < 0.05). For 19 subjects with IC2 hypomethylation, the IC2 methylation level was significantly different (p < 0.05) between the subjects with and without features including macroglossia (IC2 methylation level: 11.1% vs. 30.0%) and prenatal or postnatal overgrowth (8.5% vs. 16.9%). The IC2 methylation level was negatively correlated with birth weight z score (p < 0.01, n = 19) and birth height z score (p < 0.05, n = 13). For 36 subjects with clinically diagnosed BWS, the IC2 methylation level was negatively correlated with the BWS score (r = −0.592, p < 0.01). The IC1 methylation level showed the tendency of positive correlation with the BWS score without statistical significance (r = 0.137, p > 0.05). Conclusions: Lower IC2 methylation and higher IC1 methylation levels were associated with greater disease severity in the subjects with clinically diagnosed BWS. Quantitative DNA methylation analysis using the MassARRAY assay could improve the detection of epigenotype-phenotype correlations, which could further promote better genetic counseling and medical care for these patients.

Background Information on functional strengths and weaknesses of mucopolysaccharidosis (MPS) patients is important for early intervention programs and enzyme replacement therapy (ERT). Methods We used the Functional Independence Measure for Children (WeeFIM) questionnaire to assess the functional skills of 63 Taiwanese MPS patients (median age, 13 years 3 months; range, 3–20 years) from January 2012 to December 2018. Results Mean total WeeFIM score was 75.4 of a potential score of 126. Mean total WeeFIM scores of each type (MPS I, MPS II, MPS IIIB, MPS IVA, and MPS VI) were 103.8, 76.2, 41.6, 92.2, and 113.6, respectively. Mean scores for self‐care, mobility, and cognition domains were 30 (maximum 56), 23 (maximum 35), and 22 (maximum 35), respectively. MPS type IIIB patients had the lowest scores in self‐care, mobility, cognition, and total domains compared to other types of MPS. All patients with ERT in MPS I, II, and IVA had higher scores in self‐care and mobility domains than patients without ERT. Most patients required assistance for self‐care skills, especially in grooming and bathing. Conclusion MPS patients require support and supervision in self‐care tasks. For cognition tasks, MPS IIIB patients also require help. This questionnaire is useful to identify the strengths and limitations of MPS patients. Mucopolysaccharidosis (MPS) patients require support and supervision in self‐care tasks. For cognition tasks, MPS IIIB patients also require help. WeeFIM questionnaire is useful to identify the strengths and limitations of MPS patients.

Background: Silver–Russell syndrome (SRS) is a clinically and genetically heterogeneous disorder characterized by severe intrauterine growth retardation, poor postnatal growth, characteristic facial features, and body asymmetry. Hypomethylation of the imprinted genes of the chromosome 11p15.5 imprinting gene cluster and maternal uniparental disomy of chromosome 7 (mUPD7) are the major epigenetic disturbances. The aim of this study was to characterize the epigenotype, genotype, and phenotype of these patients in Taiwan. Methods: Two hundred and six subjects with clinically suspected SRS were referred for diagnostic testing, which was performed by profiling the methylation of H19-associated imprinting center (IC) 1 and the imprinted PEG1/MEST region using methylation-specific multiplex ligation-dependent probe amplification and high-resolution melting analysis with a methylation-specific polymerase chain reaction assay. We also applied a whole genome strategy to detect copy number changes and loss of heterozygosity. Clinical manifestations were recorded and analyzed according to the SRS scoring system proposed by Bartholdi et al. Results: Among the 206 referred subjects, 100 were classified as having a clinical diagnosis of SRS (score ≥ 8, maximum = 15) and 106 had an SRS score ≤ 7. Molecular lesions were detected in 45% (45/100) of the subjects with a clinical diagnosis of SRS, compared to 5% (5/106) of those with an SRS score ≤ 7. Thirty-seven subjects had IC1 hypomethylation, ten subjects had mUPD7, and three subjects had microdeletions. Several clinical features were found to be statistically different (p < 0.05) between the “IC1 hypomethylation” and “mUPD7” groups, including relative macrocephaly at birth (89% vs. 50%), triangular shaped face (89% vs. 50%), clinodactyly of the fifth finger (68% vs. 20%), and SRS score (11.4 ± 2.2 vs. 8.3 ± 2.5). Conclusions: The SRS score was positively correlated with the molecular diagnosis rate (p < 0.001). The SRS subjects with mUPD7 seemed to have fewer typical features and lower SRS scores than those with IC1 hypomethylation. Careful clinical observation and timely molecular confirmation are important to allow for an early diagnosis and multidisciplinary management of these patients.

Background Mucopolysaccharidosis type I (MPS I) is a genetic disease caused by the deficiency of α-L-iduronidase (IDUA) activity. MPS I is classified into three clinical phenotypes called Hurler, Scheie, and Hurler-Scheie syndromes according to their clinical severity. Treatments for MPS I are available. Better outcomes are associated with early treatment, which suggests a need for newborn screening for MPS I. The goal of this study was to determine whether measuring IDUA activity in dried blood on filter paper was effective in newborn screening for MPS I. Methods We conducted a newborn screening pilot program for MPS I from October 01, 2008 to April 30, 2013. Screening involved measuring IDUA activity in dried blood spots from 35,285 newborns using a fluorometric assay. Results Of the 35,285 newborns screened, 19 did not pass the tests and had been noticed for a recall examination. After completing further recheck process, 3 were recalled again for leukocyte IDUA enzyme activity testing. Two of the three had deficient leukocyte IDUA activity. Molecular DNA analyses confirmed the diagnosis of MPS I in these two newborns. Conclusions It is feasible to use the IDUA enzyme assay for newborn screening. The incidence of MPS I in Taiwan estimated from this study is about 1/17,643.