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Atypical Familial Mediterranean Fever Complicated with Gastrointestinal Amyloidosis Diagnosed due to Paroxysmal Arthralgia and Intractable Diarrhea, Successfully Treated with Tocilizumab
A 53-year-old man with recurrent episodes of large joint pain and a low-grade fever at irregular intervals for 16 years developed right knee and ankle arthralgia, watery diarrhea, and abdominal pain. Following an ileum and colon biopsy, he was diagnosed with gastrointestinal amyloidosis. We suspected familial Mediterranean fever (FMF) based on his history and administered colchicine; his symptoms subsequently improved. Thus, he was diagnosed with atypical FMF. After tocilizumab administration, the amyloid deposits disappeared. This case suggests that physicians should consider FMF even in cases with atypical symptoms in order to prevent the progression of amyloidosis and that amyloid deposits can be eliminated by interleukin (IL)-6 inhibition
Synthesis of a Novel Pyrazine-Pyridone Biheteroaryl-Based Fluorescence Sensor and Detection of Endogenous Labile Zinc Ions in Lung Cancer Cells
A small extent of endogenous labile zinc is involved in many vital physiological roles in living systems. However, its detailed functions have not been fully elucidated. In this study, we developed a novel biheteroaryl-based low molecular weight fluorescent sensor, 3-(phenylsulfonyl)-pyrazine-pyridone (5b), and applied it for the detection of endogenous labile zinc ions
from lung cancer cells during apoptosis. The electron-withdrawing property of the sulfonyl group between the phenyl ring as an electron donor and the pyridone ring as a fluorophore inhibited the intramolecular charge transfer state, and the background fluorescence of the sensor was decreased
in aqueous media. From the structure-fluorescence relationship analysis of the substituent effects with/without Zn 2+ , compound 5b acting as a sensor possessed favorable properties, including a longer emission wavelength, a large Stokes shift (over 100 nm),a large fluorescence enhancement in response to Zn 2+ under physical conditions, and good cell membrane permeability in living cells. Fluorescence imaging studies of human lung adenocarcinoma cells (A549) undergoing apoptosis revealed that compound 5b could detect endogenous labile zinc ions. These experiments suggested that the low molecular
weight compound 5b is a potential fluorescence sensor for Zn 2+ toward understanding its functions in living systems
Synthesis and photophysical properties of butterfly-shaped dinuclear Pt(II) complex having NHC-based chelate ligands
A butterfly-shaped dinuclear Pt(II) complex having pyridyl-NHC chelate ligands and bridging Me2pz ligands was synthesized. The crystal structure of the Pt2 complex showed short intramolecular Pt⋯Pt distance (3.129 Å) and unique packing structure containing one-dimensional infinite pore that was filled with solvent molecules. The absorbance was proportional to the concentration of Pt2 complex and no significant bands appeared even in high concentration (up to 100 μM). The Pt2 complex showed bluish-green emission (λmax = 459 nm) in the solid state at 298 K (λex = 330 nm). TD-DFT calculation revealed that the lowest-energy absorption bands are mainly assigned to the combination of metal-to-ligand charge transfer (MLCT) and ligand-to-ligand charge transfer (LLCT) transitions
Performance of alternative measures to body mass index in the assessment of moderate and severe under-nutrition among acutely unwell patients hospitalized in a TB ward in the Philippines: A cross-sectional study
Body mass index (BMI) kg/m2 is a key screening tool for under-nutrition in adults, but difficult to obtain in immobile or unwell patients, particuarly in low resource settings, due to inability to accurately measure both weight and height. Mid-upper arm circumference (MUAC) is used to assess under-nutrition in children under 5 years but no standardised cut-off values exist for adults. In a cohort of adult Filipino patients admitted to a tuberculosis ward we assessed (i) cut-offs for MUAC to predict moderate under-nutrition (BMI <17kg/m2), (ii) the performance of limb lengths to predict height and; (iii) associations of body fat percentage from skinfolds and hand grip-strength with BMI. In 303 patients with MUAC and BMI at admission, aged 18–80 years (mean = 45.5, SD:14.8), BMI ranged from 11.2–30.6 kg/m2 and 141 (46.5%) had BMI <17.0 kg/m2. Using receiver operator curves, MUAC cut-offs were identified as <20.5cm for males (sensitivity: 89%, specificity: 84%) and <18.5cm for females (sensitivity: 91%, specificity: 89%), for BMI<17.0 kg/m2. Using published equations, knee height had the lowest mean difference between predicted and measured heights compared to ulnar or demi-span: (-0.98 cm, 95% CI: -1.51/-0.44). Both grip-strength and body fat percentage were positively associated with BMI, in separate linear regression models with exposure-age-sex interactions (adjusted-R-squared values: 0.15, 0.66, respectively). MUAC can predict moderate acute under-nutrition with high positive predictive value. Further research is required to determine the performance of alternative measures to BMI to predict mortality or adverse outcomes in acutely unwell patients
2004-2015年,日本(南房総)における日本紅斑熱とツツガムシ病の区別
Japanese spotted fever (JSF) and scrub typhus (ST) are endemic to Japan and share similar clinical features. To document the clinical and epidemiologic characteristics that distinguish these 2 rickettsial diseases, during 2004–2015 we recruited 31 JSF patients, 188 ST patients, and 97 nonrickettsial disease patients from the southern Boso Peninsula of Japan. JSF occurred during April–October and ST during November–December. Patients with JSF and ST were significantly older and more likely to reside in wooded areas than were patients with nonrickettsial diseases. Spatial analyses revealed that JSF and ST clusters rarely overlapped. Clinical findings more frequently observed in JSF than in ST patients were purpura, palmar/plantar rash, hyponatremia, organ damage, and delayed defervescence after treatment. Although their clinical features are similar, JSF and ST differ in seasonality, geographic distribution, physical signs, and severity. Because a considerable percentage of patients did not notice rash and eschar, many rickettsial diseases might be underdiagnosed in Japan.長崎大学学位論文 学位記番号:博(医歯薬)甲第1154号 学位授与年月日:平成31年3月20日Author: Eiichiro Sando, Motoi Suzuki, Shungo Katoh, Hiromi Fujita, Masakatsu Taira, Makito Yaegashi, and Koya AriyoshiCitation: Emerging Infectious Diseases, 24(9), pp.1633-1641; 201
Land Invasion by the Mudskipper, Periophthalmodon septemradiatus, in Fresh and Saline Waters of the Mekong River
There has been a long-standing controversy about whether vertebrates emerged in the Paleozoic from marine or freshwater environments. Several hypotheses have proposed coastal, estuarine and riparian areas as sites of the transition. Here, we report the ecology of an amphibious fish Periophthalmodon septemradiatus, which we presume is in the process of niche expansion into terrestrial habitats from estuarine to freshwater environments along the Mekong River, Vietnam. Adult fish are highly terrestrial and have not been observed to venture into water during our survey. Courtship behaviour was observed, and fertilised eggs were recovered from burrows in both brackish and freshwater environments. The smallest fish collected at 12, 96, and 148 km from the river mouth were juveniles shortly after starting an amphibious life. These findings suggest reproduction in both brackish and freshwater environments. In contrast, otolith Sr:Ca ratio indicates larval hatching only in brackish water. Analysis of a 940-base pair (bp) segment of the mitochondrial cytochrome c oxidase subunit II and a 934-bp segment of the mitochondrial D-loop demonstrated no genetic segregation between populations. The fish may provide a unique opportunity to study how ambient salinity affects the biology and ecology of a living vertebrate during transition from water to land