Background: Tight filum terminale is a rare and challenging condition to diagnose because it presents with nonspecific symptoms and unclear imaging findings. This report documents an atypical case of tight filum terminale.
Case Presentation: The patient was a previously healthy Asian 18-year-old male presenting with recurrent upper extremity and back pain, initially treated as nonspecific musculoskeletal pain.
Familial neurohypophyseal diabetes insipidus (FNDI) is a degenerative disorder in which vasopressin-secreting neurons degenerate over time due to the production of mutant proteins. We have demonstrated therapeutic effects of chemical chaperones in an FNDI mouse model, but the complexity and length of this evaluation were problematic. In this study, we established disease-specific mouse induced pluripotent stem cells (iPSCs) from FNDI-model mice and differentiated vasopressin neurons that produced mutant proteins.
View Article and Find Full Text PDFThe most common cause of acute kidney injury (AKI) in multiple myeloma is light-chain cast nephropathy (LCCN), which consists of a light chain and Tamm-Horsfall protein (THP). We herein report a 46-year-old woman with hypercalcemia and AKI. A renal biopsy showed crystalline casts, which were consistent with lambda light chains but not THP.
View Article and Find Full Text PDFProc Natl Acad Sci U S A
July 2023
Metal-organic frameworks (MOFs), which possess a high degree of crystallinity and a large surface area with tunable inorganic nodes and organic linkers, exhibit high stimuli-responsiveness and molecular adsorption selectivity that enable various applications. The adsorption in MOFs changes the crystalline structure and elastic moduli. Thus, the coexistence of adsorbed/desorbed sites makes the host matrices elastically heterogeneous.
View Article and Find Full Text PDFAsia Ocean J Nucl Med Biol
January 2023
Objectives: The aim of this study was to investigate the effect on standardized uptake value (SUV) measurement variability of the positional relationship between objects of different sizes and the pixel of a positron emission tomography (PET) image.
Methods: We used a NEMA IEC body phantom comprising six spheres with diameters of 10, 13, 17, 22, 28, and 37 mm. The phantom was filled with F solution and contained target-to-background ratios (TBRs) of 2, 4, and 8.
PET can reveal in vivo biological processes at the molecular level. PET-derived quantitative values have been used as a surrogate marker for clinical decision-making in numerous clinical studies and trials. However, quantitative values in PET are variable depending on technical, biological, and physical factors.
View Article and Find Full Text PDFIntravenous bisphosphonate therapy is used to prevent fractures in the management of bone metastasis. However, it may induce renal damage. We herein report an 81-year-old woman with Fanconi syndrome and osteomalacia who had been diagnosed with metastatic breast cancer and received treatment with zolendronate for over 5 years.
View Article and Find Full Text PDFFamilial neurohypophyseal diabetes insipidus (FNDI) is a degenerative disease of vasopressin (AVP) neurons. Studies in mouse in vivo models indicate that accumulation of mutant AVP prehormone is associated with FNDI pathology. However, studying human FNDI pathology in vivo is technically challenging.
View Article and Find Full Text PDFObjectives: Muscle wasting is a common complication in patients with nonalcoholic fatty liver disease (NAFLD). In this study, we investigated the effect of NAFLD on satellite cell (SC) content and skeletal muscle repair.
Methods: Male CD-1 mice fed a choline-deficient diet for 4 wk were used as an NAFLD model.
A 27-year-old man who developed heavy proteinuria with hematuria and acute kidney injury 2 weeks after a fever was referred to our hospital. Because he had low complements without autoantibodies, we clinically diagnosed him with infection-related glomerulonephritis. The proliferation of mesangial cells and endothelial cells with glomerular deposits of immunoglobulin A and complement 3 was found.
View Article and Find Full Text PDFA 31-year-old woman with retinitis pigmentosa who had been diagnosed with renal failure due to nephrosclerosis related to hypertensive disorders of pregnancy was referred to our hospital to prepare for renal replacement therapy. Ultrasonography and MRI of the kidneys revealed multiple corticomedullary cysts. A renal biopsy showed that the tubules were tortuous and atrophic with segmented tubular basement membrane thickening.
View Article and Find Full Text PDFMisfolded or unfolded proteins in the ER are said to be degraded only after translocation or isolation from the ER. Here, we describe a mechanism by which mutant proteins are degraded within the ER. Aggregates of mutant arginine vasopressin (AVP) precursor were confined to ER-associated compartments (ERACs) connected to the ER in AVP neurons of a mouse model of familial neurohypophysial diabetes insipidus.
View Article and Find Full Text PDFBackground: Idiopathic membranous nephropathy (MN) is one of the major glomerulonephritis that cause nephrotic syndrome. The phospholipase A receptor (PLAR) has recently been identified as an endogenous antigen of idiopathic MN. Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by schistocytes, hemolytic anemia, thrombocytopenia, and organ dysfunction which occurs as a result of thrombi.
View Article and Find Full Text PDFWe show theoretically that spin and orbital degrees of freedom in the pyrochlore oxide Y_{2}Mo_{2}O_{7}, which is free of quenched disorder, can exhibit a simultaneous glass transition, working as dynamical disorder for each other. The interplay of spins and orbitals is mediated by the Jahn-Teller lattice distortion that selects the choice of orbitals, which then generates variant spin exchange interactions ranging from ferromagnetic to antiferromagnetic ones. Our Monte Carlo simulations detect the power-law divergence of the relaxation times and the negative divergence of both the magnetic and dielectric nonlinear susceptibilities, resolving the long-standing puzzle on the origin of the disorder-free spin glass.
View Article and Find Full Text PDFThe pituitary is a major hormone center that secretes systemic hormones responding to hypothalamus-derived-releasing hormones. Previously, we reported the independent pituitary induction and hypothalamic differentiation of human embryonic stem cells (ESCs). Here, a functional hypothalamic-pituitary unit is generated using human induced pluripotent stem (iPS) cells in vitro.
View Article and Find Full Text PDFHigh differentiation efficiency is one of the most important factors in developing an in vitro model from pluripotent stem cells. In this report, we improved the handling technique applied to mouse-induced pluripotent stem (iPS) cells, resulting in better differentiation into hypothalamic vasopressin (AVP) neurons. We modified the culture procedure to make the maintenance of iPS cells in an undifferentiated state much easier.
View Article and Find Full Text PDFTanycytes have recently been accepted as neural stem/progenitor cells in the postnatal hypothalamus. Persistent retina and anterior neural fold homeobox (Rax) expression is characteristic of tanycytes in contrast to its transient expression of whole hypothalamic precursors. In this study, we found that Rax+ residual cells in the maturation phase of hypothalamic differentiation in mouse embryonic stem cell (mESC) cultures had similar characteristics to ventral tanycytes.
View Article and Find Full Text PDFAnticoagulants have recently been recognised as a cause of acute kidney injury (AKI). We describe the case of a 75-year-old man with IgA vasculitis and atrial fibrillation treated with rivaroxaban, who presented with macroscopic haematuria and an acute decline in renal function. Two months before referral, he noted palpable purpuric lesions and was diagnosed with IgA vasculitis based on skin biopsy findings; the skin lesion disappeared following treatment with a steroid external preparation.
View Article and Find Full Text PDFWe study the orientational ordering of 2-dimensional closely packed Janus particles by extensive Monte Carlo simulations. For smaller patch sizes, the system remains in the plastic crystal phase where the rotational degrees of freedom are disordered down to the lowest temperatures. There the liquid consists of dimers and trimers of the attractive patches.
View Article and Find Full Text PDFArginine-vasopressin (AVP) neurons exist in the hypothalamus, a major region of the diencephalon, and play an essential role in water balance. Here, we established the differentiation method for AVP-secreting neurons from human embryonic stem cells (hESCs) by recapitulating in vitro the in vivo embryonic developmental processes of AVP neurons. At first, the differentiation efficiency was improved.
View Article and Find Full Text PDFAims: Choline-deficient diet is extensively used as a model of nonalcoholic fatty liver disease (NAFLD). In this study, we explored genes in the liver for which the expression changed in response to the choline-deficient (CD) diet.
Main Methods: Male CD-1 mice were divided into two groups and fed a CD diet with or without 0.
A TTF-based (TTF=tetrathiafulvalene) tridentate ligand (α-(4'-methyl-4,5-di-n-dodecylthylthiotetrathiafulvalene-5'-ylthio)- α'-[2,2,2-tris(1-pyrazolyl)ethoxy]-p-xylene) (L) with long-chain alkyl moieties was prepared in order to obtain a new multi-redox active gelator based on a mixed-metal octanuclear complex [Fe Ni (CN) (tp) (L) ](BF ) (1). The magnetism, electrochemistry, and gelation behavior of 1 were studied and 1,2-dichlorobenzene solutions of 1 are shown to display thermoreversible gelation behavior at room temperature. Furthermore, the gel phase of 1 was shown to undergo room-temperature gel-to-sol transformations induced by both the oxidation and reduction of the gelator complex by F TCNQ or [Fe (Cp*) ], respectively.
View Article and Find Full Text PDFAdiabatic demagnetization is currently gaining strong interest in searching for alternatives to (3)He-based refrigeration techniques for achieving temperatures below 2 K. The main reasons for that are the recent shortage and high price of the rare helium isotope (3)He. Here we report the discovery of a large magnetocaloric effect in the intermetallic compound YbPt2Sn, which allows adiabatic demagnetization cooling from 2 K down to 0.
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