IgA nephropathy diagnosed as a result of acute exacerbation due to G-CSF administration.

Granulocyte colony-stimulating factor (G-CSF) is commonly used to stimulate bone marrow production. G-CSF is usually safe but sometimes causes serious adverse effects and, in rare cases, exacerbates glomerulonephritis. We report a case of immunoglobulin A (IgA) nephropathy that was aggravated by G-CSF. A 56-year-old Japanese man with no relevant medical history was admitted to our hospital as a donor of peripheral blood stem cells (PBSCs) for transplantation. To mobilize PBSCs, he received subcutaneous G-CSF (lenograstim), 500 µg for 4 days. Three days after the first dose of lenograstim, gross hematuria appeared, and after administration on the fourth day, renal dysfunction and nephrotic-range proteinuria were observed. Renal biopsy and light microscopic study revealed mild mesangial proliferation with expansion in association with the presence of cellular segmental crescents. Immunofluorescence study revealed diffuse, granular staining in the mesangium for IgA, complement component 3 (C3), and lambda light chains. We diagnosed highly active IgA nephropathy and initiated treatment with prednisolone and azathioprine. Three months later, renal function returned to normal. Screening for hidden chronic glomerulonephritis should be performed when G-CSF is administered, as in PBSC donors. Immunosuppressant therapy, such as prednisolone or azathioprine, is considered for exacerbations of highly active glomerulonephritis.

A case of juvenile-onset fibrillary glomerulonephritis diagnosed by mass spectrometry and immunohistochemistry of DNAJB9.

Fibrillary glomerulonephritis (FGN) is a rare glomerular disease. FGN is characterized by the deposition of randomly arranged, nonbranching microfibrils in the mesangium and glomerular basement membrane. The discovery of DNAJ homolog subfamily B member 9 (DNAJB9) in 2017 was a breakthrough, and DNAJB9 has been proven to be extremely useful for the definitive diagnosis of FGN. While FGN often occurs in middle-aged individuals, this case was diagnosed at a relatively young age of 17. We performed renal biopsy, and light microscopic study revealed mesangial proliferation with expansion and subepithelial deposits. Electron microscopic study showed glomerular deposition of randomly oriented nonbranching fibrils with a mean of 20 nm. However, direct first scarlet stain for amyloidosis was weakly positive. Therefore, we confirmed the diagnosis of FGN and eliminated the presence of amyloidosis with mass spectrometry. This is the first case in Japan in which the complication of amyloidosis was ruled out with mass spectrometry and FGN was diagnosed using immunostaining and mass spectrometry of DNAJB9. We began treatment with cyclosporine A. One and a half years after the start of the treatment, kidney function continues to be normal.

External rotation of the femoral component decreases patellofemoral contact stress in total knee arthroplasty.

PURPOSE: The purpose of this study was to investigate the relationship between patellofemoral (PF) contact stress in vivo and the alignment of the femoral component in patients after total knee arthroplasty (TKA). METHODS: Thirty knees with medial compartment osteoarthritis that underwent mobile-bearing TKA with modified gap technique were evaluated. Surgery was performed using a subvastus approach to eliminate the effect of the approach to muscle balance, with a computed tomography-based navigation system (Vector Vision 1.61; Brain Lab, Heimstetten, Germany). PF contact stress was measured by a Flexiforce pressure sensor (Nitta Co., Ltd., Osaka, Japan) intraoperatively, and the results were compared with the alignment of the femoral component after TKA. RESULTS: The PF contact stress was not correlated with sagittal and coronal alignment of the femoral component and patellar tracking, whereas rotational alignment of the femoral component was negatively correlated with PF contact stress (r = -0.718, p < 0.01). CONCLUSIONS: Regarding the alignment of the femoral component, only the rotational alignment of the femoral component was correlated with PF contact stress. PF contact stress decreased more as the femoral component rotated more externally. LEVEL OF EVIDENCE: Case control study, Level III.

Congenital dorsally curving deformity of the distal phalanx of the little finger: case report.

A deformity of the distal phalanx of the little finger with a curvature in the dorsal direction, referred to as a reverse Kirner's deformity, is extremely rare. We present a case of such a deformity found in a 6-year-old girl. A successful result was obtained after double corrective osteotomy of the distal phalanx at 12 years of age.

Mobile-bearing total knee arthroplasty improves patellar tracking and patellofemoral contact stress: in vivo measurements in the same patients.

Controversies exist in clinical study concerning the effect of rotating platform on patellar tracking. The aim of this in vivo study was to compare tibial rotation, patellar tracking, and patellofemoral contact stress in mobile and fixed-bearing platform intraoperatively in the same knee. Sixty-six knees of posterior-stabilized total knee prostheses were evaluated using a computed tomography-guided navigation system. Medial shift and lateral tilt of patella were significantly smaller in mobile knee. Averaged maximum contact stress was significantly smaller in mobile knee than fixed knee. However, tibial rotation during flexion has no significant difference. This study showed that mobile platform total knee arthroplasty significantly improved patellar tracking and decreased patellofemoral contact stress.

Engineering control over the conformation of the alkyne-aryl bond by the introduction of cationic charge.

A novel arylene-ethynylene molecule has been synthesized. This molecule is more stable in a coplanar form than in a twisted form as in the cases of typical arylene-ethynylene molecules. When the cationic charge was introduced into the pi-conjugated system, the perpendicularly twisted form became more stable than the coplanar state. The conformational change was controlled by introduction and removal of cationic charge, confirmed by the absorption and fluorescence spectroscopy and DFT calculation.

Preparation and characterization of a novel organic-inorganic nanohybrid "cerasome" formed with a liposomal membrane and silicate surface.

A novel class of organic-inorganic hybrids, the so-called cerasomes, which have a bilayer vesicular structure and a silicate surface, has been synthesized by combination of sol-gel reaction and self-assembly of organoalkoxysilanes with a molecular structure analogous to lipids. We have synthesized two cerasome-forming organoalkoxysilanes, N-[N-(3-triethoxysilyl)propylsuccinamoyl]dihexadecylamine (1) and N,N-dihexadecyl-N (alpha)-[6-[(3-triethoxysilyl)propyldimethylammonio]hexanoyl]glycinamide bromide (2), and investigated the synthetic conditions of the cerasomes and their structural characteristics. For the proamphiphilic 1, the cerasome was obtained under restricted pH conditions where acid-catalyzed hydrolysis of the triethoxysilyl moiety proceeded without disturbing the vesicle formation. In contrast, the amphiphilic 2, additionally having a hydrophilic quaternary ammonium group, formed stable dispersions of the cerasome in a wide pH range. The hydrolysis behavior of the triethoxysilyl groups was monitored by (1)H NMR spectroscopy. Morphology of the cerasomes having the liposomal vesicular structure was confirmed by TEM observations. Extent of the development of siloxane networks through condensation among the silanol groups on the cerasome surface was evaluated by using MALDI-TOF-MS spectrometry. Formation of oligomers of the cerasome-forming lipids in the vesicle was clearly confirmed. Due to the siloxane network formation, the cerasome showed remarkably high morphological stability compared with a reference liposome, as evaluated by surfactant dissolution measurements.

Cerasome as an infusible, cell-friendly, and serum-compatible transfection agent in a viral size.

Alanine-based cationic lipid 1 having a (EtO)3SiCH2CH2CH2 group on the quaternized ammonium nitrogen forms a liposome which self-rigidifies via in situ sol-gel processes (Si-OEt + H2O --> Si-OH + EtOH followed by 2Si-OH --> Si-O-Si + H2O) on the surface. The resulting cerasome (partially ceramic- or silica-coated liposome) (60-70 nm) retains the integrity of such in the complexation with lucifarase-encoding plasmid DNA pGL3. The resultant pGL3 complex of infusible or monomeric cerasome in a viral size ( approximately 70 nm) exhibits a remarkable transfection performance toward HeLa and HepG2 cells with a 102-3-fold higher efficiency (relative to that of the nonsilylated reference lipid 2), minimized cytotoxicity, and serum compatibility. Reference lipid 2, i.e., alanine-based lipid having a simple quaternized ammonium headgroup, forms liposome (60-70 nm) which is less self-confined and more mobile undergoes DNA-induced fusion to give endocytosis-irrelevant and more toxic bigger (100-300 nm) particles. The silicon strategy thus provides a simple and widely applicable tool to overcome general problems associated with current technology of artificial gene delivery.