Objective To improve the recognition of synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome by investigating its clinical features and imaging manifestations. Methods The data of 14 patients with SAPHO syndrome (8 males, 6 females, age range: 18-61 years) between January 2014 and January 2017 were retrospectively analyzed. The clinical features, the results of laboratory (erythrocyte sedimentation rate (ESR), C reaction protein (CRP), complement component 3 (C3) and component 4 (C4), human leukocyte antigen (HLA)-B27) tests, pathology and imaging examinations (CT and whole-body bone im-aging) were collected. Results SAPHO syndrome was mainly characterized by skin damage and bone joint damage. The main manifestations of skin lesions were pustules, desquamation and erythema. Those of bone and joint injury were sternocostoclavicular hyperostosis, stiff spinal hyperostosis, peripheral arthritis, and sacroiliitis. There were 5 with fasting ESR, 5 with abnormal CRP, 3 with abnormal C3, and 1 with abnormal C4 among 14 patients. HLA-B27 was negative in all patients. Whole-body bone imaging revealed that 14 pa-tients had abnormal radioactivity, mainly involving the anterior chest wall, spine, pelvis and long bone. Chest CT manifestations of the patients were the destruction of joint bone, the fusion of the hypertrophy and the narrowing of the joint space. Symmetric lesions accounted for the majority. Pathology examination showed non-specific inflammation in 9 patients. Conclusions SAPHO syndrome is a disease characterized by skin, bone and joint abnormalities. Whole-body bone imaging can find systemic bone damage in early stage, and CT can detect bone changes and soft tissue lesions.

Public hospitals play an important role in independent innovation of basic medicine and clinical medicine. As key players in translation of medical research achievements, they play a pivotal role in promoting the development of medical sciences. At present, these hospitals however, are faced with challenges in the translation, namely system defects, unclear property rights ownership, misleading research project goals, defective assessment mechanism, insufficient funding, and lack of translation channels. In such considerations, the authors suggested based on their experiences to improve the translation system of scientific research achievements, set up special departments for translation, clarify the rights to use and dispose of scientific research achievements and that to yields. They also proposed to actively guide the establishment of clinical application projects, improve the evaluation mechanism, and broaden channels of funding. These measures are expected to accelerate the translation of scientific research achievements.

Zfyve16 (a.k.a. endofin or endosome-associated FYVE-domain protein), a member of the FYVE-domain protein family, is involved in endosomal trafficking and in TGF-β, BMP, and EGFR signaling. The FYVE protein SARA regulates the TGF-β signaling pathway by recruiting Smad2/3 and accelerating their phosphorylation, thereby altering their susceptibility to TGF-β-mediated T cell suppression. Zfyve16 binds to Smad4 and their binding affects the formation of Smad2/3-Smad4 complex in TGF-β signaling. However, the in vivo function of Zfyve16 remains unknown. In this study, we generated a Zfyve16 knockout mouse strain (Zfyve16) and examined its hematopoietic phenotypes and hematopoietic reconstruction ability. The proportion of Tcells in the peripheral blood of Zfyve16 mice increases compared with that in wild-type mice. This finding is consistent with the role of Zfyve16 in facilitating TGF-β signaling. Unpredictably, B cell proliferation is inhibited in Zfyve16 mice. The proliferation potential of Zfyve16 B-lymphoid cells also significantly decreases in vitro. These results suggest that Zfyve16 inhibits the proliferation of T cells, possibly through the TGF-β signaling, but upregulates the proliferation of B-lymphoid cells.
Animals ; B-Lymphocytes ; metabolism ; CD4-Positive T-Lymphocytes ; metabolism ; Cell Movement ; Cell Proliferation ; genetics ; Intracellular Signaling Peptides and Proteins ; antagonists & inhibitors ; genetics ; metabolism ; Mice ; Mice, Knockout ; Serine Endopeptidases ; genetics ; metabolism ; Signal Transduction ; Smad Proteins, Receptor-Regulated ; metabolism ; Transforming Growth Factor beta ; metabolism ; Up-Regulation