A case of rapid avacopan-induced liver injury in pediatric granulomatosis with polyangiitis.

Children with anti-neutrophil cytoplasmic antibody-associated vasculitis benefit immensely from avacopan as it reduces the requirement for steroids. However, descriptions of adverse drug reactions in children are lacking, and the dosage and follow-up intervals are unclear. A 10-year-old boy with initial granulomatosis and polyangiitis presented with diffuse pulmonary hemorrhage.

Design, synthesis, and optimization of a series of 2-azaspiro[3.3]heptane derivatives as orally bioavailable fetal hemoglobin inducers.

Pharmacological reactivation of the γ-globin gene for the production of fetal hemoglobin (HbF) is a promising approach for the management of β-thalassemia and sickle cell disease (SCD). We conducted a phenotypic screen in human erythroid progenitor cells to identify molecules that could induce HbF, which resulted in identification of the hit compound 1. Exploration of structure-activity relationships and optimization of ADME properties led to 2-azaspiro[3.

Phenotypic-screening generates active novel fetal globin-inducers that downregulate Bcl11a in a monkey model.

Heritable disorders associated with hemoglobin production are the most common monogenic disorders. These are mainly represented by disorders such as β-thalassemia and sickle cell disease. Induction of fetal hemoglobin (HbF) has been known to ameliorate the clinical severity of these β hemoglobinopathies.

Triple nitrogen-vacancy centre fabrication by CNH ion implantation.

Quantum information processing requires quantum registers based on coherently interacting quantum bits. The dipolar couplings between nitrogen vacancy (NV) centres with nanometre separation makes them a potential platform for room-temperature quantum registers. The fabrication of quantum registers that consist of NV centre arrays has not advanced beyond NV pairs for several years.

Comparison of pro re nata versus Bimonthly Injection of Intravitreal Aflibercept for Typical Neovascular Age-Related Macular Degeneration.

Purpose: The aim of this study was to clarify the 1-year outcomes of pro re nata (PRN) and bimonthly intravitreal injections of aflibercept (IVA) for typical neovascular age-related macular degeneration (tAMD) after the initial 3 monthly IVA.

Methods: We conducted a prospective, interventional study. Fifty-eight treatment-naïve patients with tAMD were randomly assigned to the PRN (30 patients) or the bimonthly (28 patients) treatment group.

Characteristics of Hyperautofluorescent Choroidal Vessels within the Macular Atrophic Area Using Spectral-Domain Optical Coherence Tomography.

Purpose: The aim of this study was to clarify the characteristic findings in patients with geographic atrophy with or without hyperautofluorescent choroidal vessels within macular atrophic areas on short-wavelength fundus autofluorescence imaging.

Procedures: Sixty-seven eyes of 43 consecutive patients with macular atrophic areas were divided into groups with (group 1) and without (group 2) hyperautofluorescent choroidal vessels on fundus autofluorescence imaging and then retrospectively studied using spectral-domain optical coherence tomography.

Results: In group 1 (n = 21), the average subfoveal choroidal thickness was 61.

A very rare case of endometriosis presenting with massive hemoperitoneum.

Endometriosis is a cause of acute abdomen because of the leakage or rupture of endometriotic cyst and tubo-ovarian abscess. However, massive hemoperitoneum as a cause of acute abdomen with endometriosis is very rare. We herein present a case of a 48-year-old woman who was urgently referred to our clinic with colic, abdominal distension, and hypovolemic shock during menstruation.

Indocyanine green angiographic and optical coherence tomographic findings support classification of polypoidal choroidal vasculopathy into two types.

Purpose: We assessed the characteristic indocyanine green angiographic (ICGA) and spectral domain optical coherence tomographic (SD-OCT) findings of two types of polypoidal choroidal vasculopathy (PCV), distinguishable by different filling patterns on ICGA.

Methods: Thirty-one eyes with PCV were classified into types 1 and 2 based on ICGA findings of either the presence or absence of both a feeder and a draining vessel. Characteristic ICGA findings were evaluated for each type of PCV.

Treatment results at 1 year of ranibizumab therapy for polypoidal choroidal vasculopathy in eyes with good visual acuity.

Purpose: To evaluate the efficacy of intravitreal ranibizumab (IVR) for subfoveal polypoidal choroidal vasculopathy (PCV) in eyes with a best corrected visual acuity (BCVA) of 0.6 (logMAR 0.22) or better.

Factors influencing visual outcome of polypoidal choroidal vasculopathy one year after photodynamic therapy.

Background: Polypoidal choroidal vasculopathy (PCV) is considered to be a good indication for photodynamic therapy (PDT). We evaluated pre-PDT factors predicting better visual acuity (VA) 1 year after PDT in patients with PCV.

Methods: We evaluated 181 eyes of 181 patients who underwent PDT and were followed up for 1 year.

Isolation and characterization of bone marrow-derived mesenchymal progenitor cells with myogenic and neuronal properties.

Sphere formation has been utilized as a way to isolate multipotent stem/progenitor cells from various tissues. However, very few studies on bone marrow-derived spheres have been published and assessed their multipotentiality. In this study, multipotent marrow cell populations were isolated using a three-step method.

A neurosphere-derived factor, cystatin C, supports differentiation of ES cells into neural stem cells.

Although embryonic stem (ES) cells are capable of unlimited proliferation and pluripotent differentiation, effective preparation of neural stem cells from ES cells are not achieved. Here, we have directly generated under the coculture with dissociated primary neurosphere cells in serum-free medium and the same effect was observed when ES cells were cultured with conditioned medium of primary neurosphere culture (CMPNC). ES-neural stem cells (NSCs) could proliferate for more than seven times and differentiate into neurons, astrocytes, and oligodendrocytes in vitro and in vivo.

[Surgical removal of choroidal neovascularization in angioid streaks].

Purpose: All the studies so far on surgical removal of choroidal neovascularization (CNV) in angioid streaks(AS) were conducted on a small number of cases. Therefore, a definitive evaluation of the surgical method was not available. The present study aimed to evaluate this surgical modality.

[Utility of laser photocoagulation of choroidal neovascularization in angioid streaks].

Purpose: We evaluate the utility of laser photocoagulation of extrafoveal choroidal neovascularization (CNV) in angioid streaks.

Subjects And Methods: Eleven eyes of 10 patients with extrafoveal CNV secondary to angioid streaks that underwent direct laser photocoagulation were followed for 5 months or more. We performed an intense laser photocoagulation of a CNV area determined by fluorescein angiography.

A study of laser photocoagulation for polypoidal choroidal vasculopathy.

Purpose: To evaluate the efficacy of laser photocoagulation for polypoidal choroidal vasculopathy (PCV) involving the macula.

Methods: The records of 38 patients (47 eyes) undergoing laser photocoagulation for PCV causing serosanguineous detachment involving the fovea were reviewed and the results were evaluated. Ten eyes underwent photocoagulation to induce a fusion scar covering whole lesions consisting of both abnormal vessels and polypoidal lesions.

[Hyperfluorescent areas observed in late-phase indocyanine green angiography (IA) of IA findings in fellow eyes of unilateral age-related macular degeneration].

Purpose: To evaluate abnormal findings of indocyanine green angiography (IA), predisposing to exudative age-related macular degeneration (AMD) in fellow eyes of unilateral exudative AMD.

Methods: We categorized 83 fellow eyes of unilateral exudative AMD into two groups, i.e.

Indocyanine green angiographic findings of chorioretinal folds.

Purpose: To analyze indocyanine green (ICG) angiographic findings of chorioretinal folds.

Methods: Eight patients (9 eyes) in whom chorioretinal folds had been diagnosed were enrolled in this study. Color photography, fluorescein angiography (FA) and ICG angiography (IA) were performed.

Purification, cDNA cloning, and characterization of a new serpin with megakaryocyte maturation activity.

A new member of the serine protease inhibitor (serpin) superfamily with megakaryocyte maturation activity was purified, and its cDNA was cloned and characterized. The predicted amino acid sequence consisting of 380 residues was unique and was 38% identical to the serpin plasminogen activator inhibitor type 2 (PAI-2). The recombinant factor expressed in Chinese hamster ovary cells showed species-specific activity on the induction of megakaryocyte maturation in vitro.

[Relationships among obesity, atherosclerotic disorders, and longevity in the elderly. An autopsy study].

To evaluate the effect of obesity on atherosclerosis, health, and longevity in the elderly, we studied the relationship between body mass index, atherosclerosis, and the duration of disability by multivariate analyses. We analyzed data from 521 residents of the Yokufu-kai home for the aged. They underwent a 50 g oral glucose tolerance test, and autopsies were done after they died.

Serum concentrations of interleukin-5 and the production of lymphocytes in reactive eosinophilia.

The concentrations of interleukin-5 (IL-5) in serum and in IL-2-stimulated lymphocyte culture medium (L-IL2-CM) prepared from patients with reactive eosinophilia were measured by enzyme-linked immunosorbent assay. The serum IL-5 concentration was increased in 16 of 34 cases. The concentrations of IL-5 in L-IL2-CM were increased in 8 of 21 patients.

Indocyanine green video-angiographic findings in choroidal osteoma.

Indocyanine green video-angiography (ICG) was done twice on three patients with choroidal osteoma. Choroidal osteoma consisted of two portions, a yellow-white area and a surrounding orange area. The early phase of ICG angiography showed hypofluorescence due to blockage, corresponding to the osteoma.

Studies on pharmacological activation of human serum immunoglobulin G by chemical modification and active subfragments. IX. Anti-inflammatory activity of completely alkylated heavy chain (C.Fr.I-H).

The anti-inflammatory activity of completely reduced and carboxamide-methylated heavy chain of all disulfide bonds in human serum immunoglobulin G (IgG) (C.Fr.I-H) was investigated using some experimental inflammatory models.