Objective:To retrospectively analyze the clinical characteristics of 193 Chinese patients with McCune-Albright syndrome (MAS).Methods:By using keywords " McCune-Albright syndrome", " Albright syndrome", or " fibrous dysplasia " as the search terms, 193 cases of MAS reported in China from January 1990 to November 2022 from the Wanfang data, CNKI, VIP, PubMed, and Embase databases were obtained, and their clinical data was retrospectively analyzed. Intergroup comparisons were carried out by using t test, Mann-Whitney U test, and χ2 test. Results:The 193 MAS patients had included 42 males and 151 females, with the median first-visit age of females being younger than males. The typical triad group had accounted for 46.1% of patients, and the middle first-visit and diagnosis age was younger than the atypical group. The primary reason for first-visit in males of MAS was fibrous dysplasia (FD), whilst that in females of MAS was peripheral precocious puberty (PPP). FD has occurred in 84.5% of the patients, with an average age of onset age being 6.1 years old, and 90% was ≤ 16 years of age. Endocrine hyperfunction was found in 79.3% of the patients, with a higher proportion in females compared with males ( P<0.05). Pituitary involvement was seen in 21.8% of the patients, and the incidence of craniofacial FD and cranial nerve compression was significantly higher in those with elevated growth hormone (GH) than without ( P<0.05). Café-au-Lait Spots were noted in 86.5% of the patients, and 28.3% (28/99) had located on the different side of FD. Conclusion:Most MAS patients had atypical manifestations and multi-systemic involvement. It is more common and occurs earlier in females. The most common reasons for initial diagnosis in male and female patients were FD and PPP, respectively. Patients with elevated GH should be examined for cranial nerve compression.

Background::Scleroderma is characterized by inflammation and fibrosis, predominantly occurring in the skin and extending to various parts of the body. The pathophysiology of scleroderma is multifaceted, with the current understanding including endothelial damage, inflammatory cell infiltration, and fibroblast activation in its progression. Nonetheless, the mechanism of cellular interactions and the precise spatial distribution of these cellular events within the fibrotic tissues remain elusive, highlighting a critical gap in our comprehensive understanding of scleroderma’s pathogenesis.Methods::In this study, we administered bleomycin intradermally to the dorsal skin of four individual murine models. Subsequently, skin tissues were harvested at predetermined intervals for comprehensive spatial transcriptomic analysis to determine the spatial dynamics influencing scleroderma pathogenesis. To validate the possible results from bioinformatic analysis, further in vitro and in vivo experiments were conducted. Results::Analysis of the spatial transcriptome revealed significant alterations in cell clusters during the progression of scleroderma. Gene Ontology analysis identified disruptions in lipid metabolism as the disease advanced. Pseudotime analysis provided evidence for a phenotypic transition from adipocytes to fibroblasts. In vitro studies demonstrated increased expression of Col1a1 and α-SMA as the disease progressed. These fibroblasts have been identified as key contributors to the increasing inflammation. Co-culturing TGF-β induced adipocytes with RAW264.7 cells resulted in overexpression of pro-inflammatory cytokines in the RAW264.7 cells. Both in vitro and in vivo experiments confirmed adipocyte loss and fibroblast formation, with transformed fibroblasts showing pronounced pro-inflammatory characteristics, highlighting their crucial role in the disease mechanism. Conclusions::Our study showed the spatial distribution and dynamic alterations of various cell types during scleroderma progression. Crucially, we identified the transformation of adipocytes into fibroblasts as a key factor promoting disease advancement. These emergent fibroblasts intensify inflammation, indicating that research on these cell clusters could reveal key scleroderma mechanisms and guide future therapies.

OBJECTIVE: To explore the anti-inflammatory effects of ethyl lithospermate in lipopolysaccharide (LPS)-stimulated RAW 264.7 murine-derived macrophages and zebrafish, and its underlying mechanisms. METHODS: 3-[4,5-dimethylthiazol-2-yl]-2,5-diphenyltetrazoliumbromide (MTT) assays were performed to investigate the toxicity of ethyl lithospermate at different concentrations (12.5-100 µ mol/L) in RAW 264.7 cells. The cells were stimulated with LPS (100 ng/mL) for 12 h to establish an inflammation model in vitro, the production of pro-inflammatory cytokines interleukin (IL)-6 and tumor necrosis factor α (TNF-α) were assessed by enzyme linked immunosorbent assay (ELISA). Western blot was used to ascertain the protein expressions of signal transducer and activator of transcription 3 (STAT3), nuclear factor kappa B (NF-κB) p65, phospho-STAT3 (p-STAT3, Tyr705), inhibitor of NF-κB (IκB) α, and phospho-I κB α (p-IκB α, Ser32), and confocal imaging was used to identify the nuclear translocation of NF-κB p65 and p-STAT3 (Tyr705). Additionally, the yolk sacs of zebrafish (3 days post fertilization) were injected with 2 nL LPS (0.5 mg/mL) to induce an inflammation model in vivo. Survival analysis, hematoxylin-eosin (HE) staining, observation of neutrophil migration, and quantitative real-time polymerase chain reaction (qRT-PCR) were used to further study the anti-inflammatory effects of ethyl lithospermate and its probable mechanisms in vivo. RESULTS: The non-toxic concentrations of ethyl lithospermate have been found to range from 12.5 to 100 µ mol/L. Ethyl lithospermate inhibited the release of IL-6 and TNF-α(P<0.05 or P<0.01), decreased IκBα degradation and phosphorylation (P<0.05) as well as the nuclear translocation of NF-κB p65 and p-STAT3 (Tyr705) in LPS-induced RAW 264.7 cells (P<0.01). Ethyl lithospermate also decreased inflammatory cells infiltration and neutrophil migration while increasing the survival rate of LPS-stimulated zebrafish (P<0.05 or P<0.01). In addition, ethyl lithospermate also inhibited the mRNA expression levels of of IL-6, TNF-α, IκBα, STAT3, and NF-κB in LPS-stimulated zebrafish (P<0.01). CONCLUSION Ethyl lithospermate exerts anti-Inflammatory effected by inhibiting the NF-κB and STAT3 signal pathways in RAW 264.7 macrophages and zebrafish.
Animals ; Mice ; NF-kappa B/metabolism* ; Lipopolysaccharides ; RAW 264.7 Cells ; Zebrafish ; NF-KappaB Inhibitor alpha/metabolism* ; Interleukin-6/metabolism* ; Tumor Necrosis Factor-alpha/metabolism* ; STAT3 Transcription Factor/metabolism* ; Inflammation/metabolism* ; Anti-Inflammatory Agents/therapeutic use*

Scleroderma is a rare disease which requires multidisciplinary treatment. Drug-based therapy can partially alleviate or end the progression of the disease, but cannot reverse the lesions that have occurred. Scleroderma patients who seek care in plastic surgery has gradually increased in recent years. With the understanding of the regenerative role of fat and adipose stem cells, physicians have found that surgical treatment of scleroderma can improve morphology while reversing the state of the diseased tissue, acting as a local treatment or slowing progression. This paper briefly described the etiology and classification of scleroderma, analyzed the current status of scleroderma treatment and focused on the surgical treatment strategy of scleroderma, providing guidelines for the surgical management of scleroderma.

Scleroderma is a rare disease which requires multidisciplinary treatment. Drug-based therapy can partially alleviate or end the progression of the disease, but cannot reverse the lesions that have occurred. Scleroderma patients who seek care in plastic surgery has gradually increased in recent years. With the understanding of the regenerative role of fat and adipose stem cells, physicians have found that surgical treatment of scleroderma can improve morphology while reversing the state of the diseased tissue, acting as a local treatment or slowing progression. This paper briefly described the etiology and classification of scleroderma, analyzed the current status of scleroderma treatment and focused on the surgical treatment strategy of scleroderma, providing guidelines for the surgical management of scleroderma.

Intensity-modulated radiation therapy(IMRT) is currently the main treatment method for nasopharyngeal carcinoma. During radiotherapy for nasopharyngeal carcinoma, factors such as body mass reduction, tumor regression, and organ displacement at risk can affect the precise implementation of radiation therapy. Applying adaptive radiotherapy (ART) technology to optimize the treatment plan at the appropriate timing can reduce the adverse effects caused by the above factors and enhance the accuracy of radiotherapy. There are no uniform standards for the necessity, timing, and case selection of ART. In this review, the research progress of ART in the radiotherapy of nasopharyngeal carcinoma in recent years was reviewed to provide a reference for further clinical application of ART in nasopharyngeal carcinoma.

Objective:To explore the effectiveness of autologous fat grafting in the treatment of undesirable skin expansion.Methods:Patients' data were reviewed from 2011 to 2016, including the expanded regions with early signs of skin complications in face and neck. The effects of fat grafting group and control group were evaluated by follow-up records of expansion volume, skin thickness, skin texture and local capillary reaction.Results:Fat grafting could increase the thickness of expanded skin. It also improved the texture of expanded skin, with 0.83± 0.71 points before treatment and 1.30±0.66 points after treatment ( P=0.04). The local capillary reaction was also improved from 1.06±0.54 points before treatment and 1.45±0.51 points after treatment ( P=0.03). The expansion in the fat grafting group was 2.21±0.57 times before treatment and 2.94±0.83 times after treatment. In the control group, the expansion was 2.19 times when it showed early signs, and no obvious changes were observed during the follow-up period. Conclusions:Autologous fat grafting can effectively treat complications of skin expansion, prolong expansion process and promote tissue regeneration.

Two new phenylpropanoid amide glycosides and ten analogues were isolated from the CH_2Cl_2 layer of 95% ethanol extract of the whole plants of Corydalis racemosa by using various chromatographic techniques, including silica gel, Sephadex LH-20, ODS column chromatographies, and semi-preparative HPLC. Their structures were identified on the basis of physicochemical properties, MS, NMR, and IR spectroscopic data as N-cis-sinapoyltyramine-4'-O-β-glucoside(1), N-cis-sinapoyl-3-methoxytyramine-4'-O-β-glucoside(2), N-cis-sinapoyltyramine(3), N-cis-feruloyltyramine(4), N-trans-cinnamoyltyramine(5), N-trans-feruloylphenethylamine(6), N-trans-p-methoxycinnamoyl-3-hydoxyoctopamine(7), N-cis-feruloyl-3-methoxytyramine(8), N-trans-feruloyltyramine(9), N-trans-feruloyl-3-methoxytyramine(10), N-trans-sinapoyltyramine(11), and N-trans-p-coumaroyltyramine(12). Compounds 1 and 2 are new compounds. Compounds 3-7 are obtained from the plants of Papaveraceae for the first time, and compounds 8-12 are firstly isolated from C. racemosa.
Amides ; Chromatography, High Pressure Liquid ; Corydalis ; Glucosides ; Glycosides

This study is to investigate the chemical constituents from the whole plant Corydalis edulis. The chemical constituents were separated and purified by macroporous resin D101, silica gel, Sephadex LH-20, ODS, and semi-preparative HPLC. Their structures were determined by physicochemical properties and spectroscopic data. Four compounds were isolated from the dichloromethane and water extracts of the whole plant C. edulis, and identified as 6'-β-D-xylosylicariside B2(1),(3S,5R,6S,7E)-5,6-epoxy-3-hydroxy-7-megastigmen-9-one(2), loliolide(3), and 5,5'-dimethoxybiphenyl-2,2'-diol(4), respectively. Compound 1 is a new compound, of which the absolute configuration was established by electronic circular dichroism(ECD) calculations. Compound 4 is obtained from the plants of Papaveraceae family for the first time. Compounds 2 and 3 are firstly isolated from the Corydalis genus.
Chromatography, High Pressure Liquid ; Corydalis/chemistry* ; Glycosides/isolation & purification* ; Molecular Structure ; Phytochemicals/isolation & purification* ; Sesquiterpenes/isolation & purification*

Objective To evaluate the necessity of arc by arc setup verification in patients with brain metastases receiving stereotactic radiotherapy (SRT) by analyzing the inter-and intra-fraction setup errors and residual errors collected from the ExacTrac X-ray portal image.Methods Clinical data of brain metastases patients treated with SRT in the previous two years were retrospectively analyzed.The ExacTrac X-ray setup images were collected after the normal setup procedure.Setup errors were calculated by registering the cranial bony structures of the ExacTrac X-ray setup images to that of the digitally reconstructed setup images.The inter-and intra-fraction setup errors and residual errors were statistically analyzed.Results Seventy-five patients from 116 lesions received 337 cycles of SRT of the head.The inter-and intra-fraction translational setup errors in the x,y and z directions were (0.93±0.86) mm and (0.15±0.59) mm;(1.83± 1.27) mm and (0.25±0.73) mm;(0.96±0.80) mm and (0.14±0.56) mm,respectively.The inter-and intra-fraction rotational setup errors in the x,y,z directions were (0.65°± 0.62°) and (0.19°± 0.40°);(0.97°±0.94°) and (0.13°± 0.25°);(0.92°± 0.71°) and (0.10°± 0.29°),respectively.The residual translational setup errors in the x,y,z directions were (0.06±0.23) mm,(0.08±0.24) mm and (0.08±0.22)mm,and (0.12°± 0.27°),(0.09°± 0.18°) and (0.06°± 0.19°) for the residual rotational setup errors,respectively.For a reference setup error threshold of 0.7 mm/0.7°,99.1％ of the SRT exceeded the threshold and required setup correction.For 1 006 non-coplanar arcs,rotating the treatment couch from 0° to the treatment angle made 66.4％ of arcs exceed the threshold and require at least once setup correction.Conclusions During SRT for brain metastasis,the inter-and intra-fraction setup errors should be emphasized.It is necessary to perform arc by arc setup error verification.