12-Week Effectiveness and Safety of Low-Density Lipoprotein Cholesterol-Lowering Therapy by Proprotein Convertase Subtilisin/Kexin Type 9 Inhibition in Patients With Familial Hypercholesterolemia and Hypercholesterolemia - Data From a Real-World Observational Study of Evolocumab in Japan.

Evolocumab is the first monoclonal antibody against proprotein convertase subtilisin/kexin type 9 (PCSK9) approved in Japan for the treatment of patients with familial hypercholesterolemia (FH) and hypercholesterolemia (HC). This study assessed the 12-week effectiveness and safety of low-density lipoprotein cholesterol (LDL-C)-lowering therapy by PCSK9 inhibition in patients with FH (homozygous [HoFH] or heterozygous [HeFH]) and HC by analyzing evolocumab data collected in the real-world setting in Japan. Overall, 427 patients (mean±SD age, 61.

Lung cancer development in the patient with granulomatosis with polyangiitis during long term treatment with cyclophosphamide: first documented case.

A 65-year-old man was diagnosed with granulomatosis with polyangiitis (GPA) at the age of 47, when cytoplasmic anti-neutrophil cytoplasmic antibody (C-ANCA) serology was positive, and he had multiple nodular shadows in both lungs. He had been treated with prednisolone, cyclophosphamide (CPA) and plasma exchange. At the age of 64, a nodular shadow was newly detected in the right lower lung field and serum tumour marker increased.

Coexistence of diffuse panbronchiolitis and asthma: reciprocity of neutrophilic and eosinophilic inflammation.

Diffuse panbronchiolitis (DPB) and asthma are obstructive airway diseases, the former being characterized by Th1-type and the latter by Th2-type airway inflammation. Differential diagnosis is often a problem, but coexistence has rarely been reported. A 76-year-old man with asthma was admitted to our hospital because of one-month history of dyspnoea on exertion with bilateral diffuse granular shadows.

Primary ciliary dyskinesia with complex abnormalities including cleavage of B-subfibers.

A 25-year-old Japanese woman suffered from repeated respiratory tract infections. Because of her characteristic medical history and imaging findings, we suspected primary ciliary dyskinesia (PCD) and performed a transbronchial biopsy. The biopsy revealed complex abnormalities of the ciliary structure including cleavage of the B-subfibers observed by transmission electron microscopy analysis and the complete loss of ciliary motion by video analysis.