HINODE study: haemophilia A in infancy and newborns - protocol for a prospective, multicentre, observational study evaluating the coagulation potential and safety of emicizumab prophylaxis.

Introduction: Emicizumab prophylaxis is approved for people of all ages with haemophilia A (HA) including infants and children. Although previous studies have demonstrated the efficacy and tolerability of emicizumab in infants with HA, real-world data on emicizumab use in infants are limited. The Haemophilia A in Infancy and NewbOrns: multi-instituional prospective observational study to assess the efficacy anD safety of Emicizumab (HINODE) study aims to evaluate the coagulation potential and safety of emicizumab prophylaxis in infants with congenital HA from birth to <12 months of age.

Efficacy, durability, and safety of faricimab with extended dosing up to every 16 weeks in diabetic macular edema: 2-year results from the Japan subgroup of the phase 3 YOSEMITE trial.

Purpose: To evaluate the 2-year efficacy, durability, and safety of faricimab in patients with diabetic macular edema (DME) in the YOSEMITE Japan subgroup.

Study Design: YOSEMITE/RHINE (NCT03622580/NCT03622593) subgroup analysis: global, multicenter, randomized, double-masked, active-comparator-controlled, phase 3 faricimab trials.

Methods: Patients were randomized 1:1:1 to intravitreal faricimab 6.

Association between human epidermal growth factor receptor 2 status, namely low and zero expression, and prognosis in hormone receptor-positive breast cancer: a single-center retrospective study.

Background: The recently developed anti-human epidermal growth factor receptor 2 (HER2) therapy has substantially improved the prognosis of HER2-positive breast cancer. The DESTINY-Breast04 trial results showed that trastuzumab deruxtecan (T-DXd) significantly prolonged the survival of patients with HER2-low breast cancer, thus presenting a paradigm shift in anti-HER2 therapy. This may facilitate a change in the treatment strategy for HER2-low breast cancer.

Efficacy, durability, and safety of faricimab up to every 16 weeks in patients with neovascular age-related macular degeneration: 2-year results from the Japan subgroup of the phase III TENAYA trial.

Purpose: To evaluate 2-year efficacy, durability, and safety of faricimab in the TENAYA Japan subgroup and pooled global TENAYA/LUCERNE cohort of patients with neovascular age-related macular degeneration (nAMD).

Methods: Subgroup analysis of TENAYA/LUCERNE (NCT03823287/NCT03823300): phase III, multicentre, randomised, active comparator-controlled, double-masked, non-inferiority trials. Treatment-naïve patients aged ≥ 50 years with nAMD were randomised (1:1) to intravitreal faricimab (6.

5-aminolevulinic acid-guided endoscopic biopsy with violet light-emitting diode flashlight in malignant glioma: Technical note.

Background: 5-aminolevulinic acid (5-ALA) photodynamic diagnosis (PDD) has enabled better identification of malignant tumor cells and real-time intraoperative guidance. Here, we report a reasonable procedure for 5-ALA-guided endoscopic biopsy with a violet light-emitting diode (LED) flashlight for deep-seated malignant gliomas.

Methods: A 63-year-old man presented with a headache and left upper homonymous quadrantanopia.

A Fatal Case of Rapidly Progressive Glomerulonephritis With Two Anti-neutrophil Cytoplasmic Antibodies and Anti-glomerular Basement Membrane Antibody: A Description of Autopsy Findings.

A 79-year-old man presented with dyspnea upon exertion, marked renal dysfunction, proteinuria, and hematuria. He was diagnosed with rapidly progressive glomerulonephritis. Serological tests were positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibodies.

Hypertrophic osteoarthropathy associated with lung cancer: Possible links among hypoxia-inducible factor-1α, vascular endothelial growth factor, and hypervascularization.

Hypertrophic osteoarthropathy (HOA) is a paraneoplastic syndrome, the exact pathogenesis of which remains to be elucidated. The case of a 69-year-old man who developed intractably painful HOA secondary to lung cancer is presented. Contrast-enhanced computed tomography of the chest showed an 80-mm solid nodule with a large low-density area.

Severe thrombocytopenia and anemia as an initial presentation of breast cancer: A case report.

Breast cancer patients with bone marrow metastasis (BMM) having profound thrombocytopenia and anemia are rare and there is no definitive treatment guideline. We present a case of successful initial treatment with anti-disseminated intravascular coagulation therapy and endocrine therapy, followed by chemotherapy to avoid deterioration of severe thrombocytopenia and anemia.

Immunosuppressive tumor microenvironment in extraskeletal myxoid chondrosarcoma: A case of pleural metastases.

Extraskeletal myxoid chondrosarcoma (EMCS) is an undifferentiated mesenchymal malignancy; however, its immune microenvironment remains to be elucidated. The case of a 34-year-old woman who developed EMCS metastasizing to the pleura is presented here. The pleural EMCS showed hypervascularity, absent PD-L1 expression, and a lack of tumor mutational burden and pathogenic variants.

Adenoid Cystic Carcinoma, Solid-Basaloid Subtype of the Breast: A Case Report.

We present a rare tumor of adenoid cystic carcinoma, solid-basaloid subtype of the breast. Solid-basaloid adenoid cystic carcinoma may have a worse prognosis than classical adenoid cystic carcinoma. A 70-year-old woman presented with a mass in her left breast.

Onset of pulmonary Epstein-Barr virus-positive diffuse large B-cell lymphoma in a patient with silicosis.

How Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) occasionally occurs following chronic inflammation remains to be elucidated. The case of a 57-year-old man who developed pulmonary EBV-positive DLBCL from underlying silicosis lesions is presented. Immunohistochemical examination of the resected silicosis lesions showed predominant helper T cells and M1/M2 macrophages, with a lack of B cells, regulatory T cells, and resident memory T cells.

Surgical excision of a lactating adenoma with rapid enlargement: A case report.

Introduction And Importance: A lactating adenoma is a benign breast tumor occurring in young women during pregnancy or lactation. Its growth is usually slow but, occasionally, can become rapid, resulting in a giant mass. This case report outlines an example of the rapid growth of a lactating adenoma, which was surgically excised.

Surgical resection of pulmonary crystal-storing histiocytosis with Sjögren's syndrome: A case report.

Introduction And Importance: Crystal-storing histiocytosis (CSH) is a rare clinical entity characterized by an abnormal increase in the number of histiocytes with massive accumulation of crystallized immunoglobulins. Yano et al. reported only one case of gastric CSH associated with Sjögren's syndrome.

Influenza A (H3N2) infection followed by anti-signal recognition particle antibody-positive necrotizing myopathy: A case report.

A 60-year-old Japanese woman presented with subacute progressive muscle pain and weakness in her proximal extremities. She was diagnosed with influenza A (H3N2) infection a week before the onset of muscle pain. At the time of admission, she exhibited weakness in the proximal muscles of the upper and lower limbs, elevated serum liver enzymes and creatinine kinase, and myoglobinuria.

Vildagliptin-induced ground-glass nodules mimicking lung metastases in a cancer patient receiving Lactobacillus probiotic supplementation.

The association between gut microbiota and the lung immune system has been attracting increasing interest. Here, we report a case of pancreatic cancer in which the dipeptidyl peptidase-4 inhibitor vildagliptin induced unusual manifestations of interstitial pneumonia, possibly under the influence of Lactobacillus paraplantarum probiotic supplementation. Chest computed tomography and positron emission tomography showed multiple ground-glass nodules (GGNs) mimicking metastatic lung cancer.

Secondary Pulmonary Alveolar Proteinosis Following Treatment with Azacitidine for Myelodysplastic Syndrome.

Secondary pulmonary alveolar proteinosis (sPAP) is a complication of myelodysplastic syndrome (MDS). A 60-year-old woman was diagnosed with MDS with excess blasts-1. Fifty-four months after the initial diagnosis, treatment with azacitidine was initiated.

Intrathoracic amyloid tumors that presented as yellowish multinodular endobronchial protrusions with irregular vascularity and easy bleeding.

Immunoglobulin light-chain (AL) amyloidosis is a monoclonal plasma cell neoplasm that has a tendency to bleed easily. However, the potential risks of transbronchial biopsy in such cases have not been fully proven. Here, we report a case of parotid and intrathoracic AL amyloid tumors that presented as endobronchial protrusions that bled easily.

Targeting in neutrophils enhances the clearance of in infected wounds.

Argonaute 2 bound mature microRNA (Ago2-miRNA) complexes are key regulators of the wound inflammatory response and function in the translational processing of target mRNAs. In this study, we identified four wound inflammation-related Ago2-miRNAs (, , , and ) and show that is critical for infection control. mice exhibited delayed sterile healing with prolonged neutrophil activation and interleukin-6 expression, and markedly improved repair of -infected wounds.

Presence of few PD-1-expressing tumor-infiltrating immune cells is a potential predictor of improved response to salvage chemotherapy following nivolumab for non-small cell lung cancer: An exploratory case series.

Background: The combination of PD-1 inhibitors and cytotoxic drugs is reported to enhance anti-tumor activity in non-small cell lung cancer; however, the underlying synergistic mechanisms remain uncertain. This retrospective case series was designed to investigate objective response and survival rates of salvage chemotherapy following nivolumab and explore the immunohistochemical profiles of tumor-infiltrating immune cells.

Methods: The medical records of 37 patients administered nivolumab were retrospectively reviewed.

Pulmonary Scedosporium apiospermum Infection with Pulmonary Tumorlet in an Immunocompetent Patient.

Scedosporium apiospermum is an opportunistic fungus that can cause various types of infections, including localized infections and life-threatening disseminated infections, particularly in immunocompromised patients. Treatment is especially challenging due to its multidrug resistance. We herein report the case of a 73-year-old woman who was non-immunocompromised but developed S.