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Cureus 2022 Apr 19;14(4):e24275. Epub 2022 Apr 19.

Orthopaedic Surgery, University of California Irvine School of Medicine, Irvine, USA.

Pycnodysostosis (PYCD) is an autosomal recessive lysosomal storage disorder of the bone which leads to stereotypical abnormalities consisting of, but not limited to, sclerotic and fragile bone, shortened distal phalanges, and obtuse mandibular angle. Current literature describes the otolaryngological manifestations and treatment of this disorder; however, the treatment of orthopedic fractures in PYCD patients is seldom described and remains a controversial topic. We aim to systematically review the current evidence regarding the optimal treatment of PYCD patients with fractures. Read More

Bioeng Transl Med 2022 May 18;7(2):e10280. Epub 2022 Jan 18.

Department of Respiratory and Critical Care Medicine, NHC Key Laboratory of Pulmonary Diseases, Key Site of National Clinical Research Center for Respiratory Disease, Wuhan Clinical Medical Research Center for Chronic Airway Diseases, Tongji Hospital, Tongji Medical College Huazhong University of Science and Technology Wuhan China.

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease characterized by the infiltration of macrophages in the fibrotic region. Currently, no therapeutic strategies effectively control disease progression, and the 5-year mortality of patients after diagnosis is unacceptably high. Thus, developing an effective and safe treatment for IPF is urgently needed. Read More

Respir Res 2022 May 21;23(1):129. Epub 2022 May 21.

Fondazione Policlinico Universitario A Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.

Background: Recombinant human pentraxin-2 (rhPTX-2) significantly decreased decline in percent predicted forced vital capacity (FVC) and stabilized 6-min walk distance (6MWD) in patients with idiopathic pulmonary fibrosis (IPF) during the 28-week, placebo-controlled, randomized period of the Phase II PRM-151-202 study. Interim (76-week) data from the open-label extension (OLE) demonstrated sustained safety and efficacy with rhPTX-2 treatment. Here, we present the entire long-term OLE safety and efficacy data to 128 weeks. Read More

Sci Rep 2022 May 20;12(1):8564. Epub 2022 May 20.

Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, Republic of Korea.

Disease progression (DP) is an important parameter for the prognosis of idiopathic pulmonary fibrosis (IPF). This study aimed to evaluate the baseline serum biomarkers for predicting the DP in IPF. Seventy-four patients who were diagnosed with IPF and had their serum Krebs von den Lungen-6 (KL-6) and monocyte count, which might be associated with prognosis of IPF, checked more than twice were included. Read More

Am J Respir Cell Mol Biol 2022 May 18. Epub 2022 May 18.

Brown University, Molecular Microbiology and Immunology, Providence, Rhode Island, United States;

Idiopathic pulmonary fibrosis (IPF) is a particularly deadly form of pulmonary fibrosis with unknown reason. In patients with IPF, high serum and lung levels of CHI3L1 can be detected and are associated with poor survival. However, the roles of CHI3L1 in these diseases have not been fully elucidated. Read More