Tissue degrading and remodelling molecules in giant cell arteritis.

Objectives: GCA is a granulomatous vasculitis affecting large vessels, leading to intimal occlusion accompanied by the accumulation of myofibroblasts. Histopathologically, GCA is characterized by destruction of the tunica media and hypertrophy of the intima with invasion of activated CD4+ T cells, macrophages and multinucleated giant cells (MNGCs). Despite these well-defined histopathological features, the molecular pathology of GCA has largely remained elusive.

Clinical Transplant Kidney Function Loss Due to Small Intestinal Bacterial Overgrowth.

Small intestinal bacterial overgrowth (SIBO) is a clinical syndrome involving gastrointestinal symptoms caused by the presence of excessive bacteria in the small intestine. SIBO often leads to diarrhea and poses diagnostic and treatment challenges. Here, we report about a renal transplant recipient who experienced diarrhea-induced hypovolemic shock due to SIBO, necessitating the reintroduction of dialysis, and aim to provide insights to aid health-care providers in diagnosing and managing severe diarrhea in this specific patient group.

Long-term remission of VEXAS syndrome achieved by a single course of CHOP therapy: A case report.

We herein describe the case of a 52-year-old male patient who presented with fever, arthritis, and neutrophilic dermatosis in 2013 and subsequently experienced macrophage activation syndrome treated with high-dose glucocorticoid therapy. Due to the persistent symptoms refractory to several immunomodulatory and immunosuppressive (IS) drug therapies with dapsone, methotrexate, tacrolimus, infliximab (IFX), and tocilizumab (TCZ), he received prednisolone (PSL) ≥20 mg/day to suppress disease activity. In 2017, Epstein-Barr virus (EBV)-associated haemophagocytic lymphohistiocytosis (HLH) was diagnosed and initially treated with immunochemotherapy consisting of dexamethasone, cyclosporine (CyA), and etoposide (ET).

Synovial fluid crystal analysis with compensated polarization using a gout analyser in clinical practice.

Objective: To validate the gout analyzer as a clinical method of synovial fluid crystal analysis.

Methods: Thirty knee synovial fluid samples with suspected calcium pyrophosphate (CPP) crystals were analyzed. Within 48 hours after collection, each non-centrifuged sample was examined blindly and independently by one or more rheumatologists in the following order: 1) with an optical microscope under ordinary light, 2) with the same microscope under compensated polarization provided by a gout analyzer, and 3) with a fully equipped compensated polarized microscope with a rotating stage as the gold standard.

A case with somatic and germline mosaicism in COL4A5 detected by multiplex ligation-dependent probe amplification in X-linked Alport syndrome.

X-linked Alport syndrome (XLAS) is a progressive hereditary kidney disease caused by mutations in the COL4A5 gene encoding the type IV collagen α5 chain. To date, 11 cases having somatic mosaic variants in COL4A5 have been reported; however, all of them involved single-nucleotide variations (SNVs). Here, we report a female XLAS patient with somatic mosaicism identified by copy number variation (CNV) in COL4A5.

Successful Treatment of Anti-Factor H Antibody-Associated Atypical Hemolytic Uremic Syndrome.

Anti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis in terms of frequent relapses. Although eculizumab is an effective treatment for this type of aHUS, the method of eculizumab discontinuation is not yet established. Herein, we report a case of anti-CFH Ab-associated aHUS in a 6-year-old boy.

Invasive Bacterial Infection in Patients with Interleukin-1 Receptor-associated Kinase 4 Deficiency: Case Report.

Interleukin-1 receptor-associated kinase 4 (IRAK4) deficiency (OMIM #607676) is a rare primary immunodeficiency of innate immune defect. We identified 10 patients from 6 families with IRAK4 deficiency in Japan, and analyzed the clinical characteristics of this disease. Nine patients had homozygous c.

Successful treatment with mycophenolate mofetil and tacrolimus in juvenile severe lupus nephritis.

Lupus nephritis (LN) of juvenile onset often has severe disease presentation. Despite aggressive induction therapy, up to 20% of patients with LN are resistant to initial therapy and up to 44% suffer a renal relapse. However, there is no consensus on an appropriate therapeutic regimen for refractory LN.

Fasting remnant lipoproteins can predict postprandial hyperlipidemia.

Background: Hypertriglyceridemia and postprandial hyperlipidemia is thought to play an important role in atherosclerosis, but to select patients at high-risk for cardiovascular diseases is difficult with triglycerides (TG) alone in these patients.

Methods: To predict postprandial hyperlipidemia without inconvenient test meal loading, we examined lipid concentrations before and after test meal loading and fasting adiponectin, and investigated which of these other than TG were significant during the fasting period in 45 healthy individuals (men: women, 26:19).

Results: TG, remnant-like particle-cholesterol and -triglyceride (RemL-C, RLP-C, and RLP-TG), and TG/apolipoprotein(apo)B were significantly elevated after loading and fasting values significantly and positively correlated with incremental area under the curve (iAUC) (r=0.

[Mycophenolate mofetil in induction and maintenance therapy for juvenile onset severe lupus nephritis].

Objective: The aim of the present study was to evaluate the efficacy of mycophenolate mofetil (MMF) in the induction and maintenance therapy for juvenile onset severe lupus nephritis.

Methods: Children with severe focal, and diffuse proliferative lupus nephritis were treated with prednisolone (initial dose; 1 mg/kg/day, maximum dose; 60 mg/day) and MMF (initial dose; 300 mg/m2/day, increased to 1 g/m2/day) for 24 months after high-dose intravenous methylprednisolone (30 mg/kg/day). Urinalysis was performed, and renal function, and albumin were evaluated.

Cascadability properties of MZI-SOA-based all-optical 3R regenerators for RZ-DPSK signals.

We experimentally demonstrate 50 cascaded all-optical 3R regenerators over a 1,000 km transmission distance for 10-Gb/s return-to-zero differential phase-shift keying (RZ-DPSK) signals. The regenerator consists of integrated Mach-Zehnder interferometer (MZI) semiconductor optical amplifier (SOA) based wavelength converters. Regenerative properties and tolerance to pattern dependent effects have been studied in terms of Q-factor measurement, and error free operation with input OSNR of 20 dB/0.

Sporadic cases and an outbreak of leptospirosis probably associated with recreational activities in rivers in the northern part of Okinawa Main Island.

In the summer of 2003, sporadic cases and an outbreak of human leptospirosis probably related to recreation in rivers occurred in the northern part of Okinawa Main Island. Sixteen of 22 suspected cases were definitely diagnosed as leptospirosis by serological test or isolation. The infective leptospiral serovar in 14 cases was presumed to be Hebdomadis.

Ultrafast spin dynamics and critical behavior in half-metallic ferromagnet: Sr2FeMoO6.

Ultrafast spin dynamics in ferromagnetic half-metallic compound Sr2FeMoO6 is investigated by pump-probe measurements of the magneto-optical Kerr effect. The half-metallic nature of this material gives rise to anomalous thermal insulation between spins and electrons and allows us to pursue the spin dynamics from a few to several hundred picoseconds after the optical excitation. The optically detected magnetization dynamics clearly shows the crossover from microscopic photoinduced demagnetization to macroscopic critical behavior with universal power law divergence of relaxation time for a wide dynamical critical region.

Evaluation of heterotopic bone formation induced by squalane and bone morphogenetic protein composite.

Bone morphogenetic protein is an important molecule whose bioactivity depends on the carrier. Squalane is used in the formulation of various kinds of cosmetics because it is easily emulsified and has the property of spreading well. Thus, squalane might be effective as a bone morphogenetic protein delivery system.

Squalane as a possible carrier of bone morphogenetic protein.

Gelatin capsules containing squalane partially purified bone morphogenetic protein (BMP) complex were placed on the perimuscular membrane of rats. Two kinds of control, gelatin capsules containing only BMP and those bearing squalane only, were used. The embedded areas were histopathologically examined at 3 and 6 wk after the operation.