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Introduction Tuberous sclerosis complex (TSC) is a multisystem neurocutaneous disorder. Angiofibromas (AF), fibrous plaques, and hypopigmented macules are the major skin findings in TSC. Topical sirolimus reduces the volume and redness of AF and other skin findings. However, the efficacy of early intervention and long-term treatment remains to be clarified. We investigated the efficacy of sirolimus gel for AF in children with TSC. Methods We recruited nine children (five boys; four girls) with TSC and AF. We used 0.2% sirolimus gel over 6 months. We reviewed each patient's medical records and photographs for clinical information and data related to improvements in skin lesions. We evaluated the size of AF, fibrous plaques, and color changes in AF and hypopigmented macules. Results Age at the initiation of treatment ranged from 3.5 to 11.0 years. The follow-up period ranged from 6 to 36 months (≥24 months in 3 children). Patients presented with papular AF (9), miliary AF (8), AF redness (9), fibrous plaques (5), and hypopigmented macules (2). After 6 months of treatment, improvement of AF size and redness was seen in all nine patients. Patients treated for ≥24 months showed significant decrease in AF size that persisted until the final follow-up. [https://www.selleckchem.com/products/gne-781.html read more] Gradual improvement in fibrous plaques was observed, and marked reduction in size was achieved by 4-18 months. Conclusion Early sirolimus gel intervention is effective for the treatment of AF and fibrous plaques in children with TSC. Early intervention with sirolimus gel may maintain the skin at near-normal levels in patients with TSC. Copyright © 2020 Okanishi, Fujimoto, Enoki and Ogai.Sweet's syndrome and eosinophilic folliculitis are aseptic inflammatory dermatitis mainly because of infiltrated neutrophils and eosinophils on skin, respectively. These diseases rarely overlap or coexist in the same patient, especially co-occur in HIV infected patient. Here, we report a rare case of an AIDS patient who developed eosinophilic folliculitis and Sweet's syndrome within 1 month of initial antiretroviral therapy, presumably due to immune reconstitution inflammatory syndrome. The CD4+ T cell counts increased dramatically from 70 to 249 cells/μL within a period of 1 month. Interestingly, the patient was rapidly and strikingly responsive to thalidomide, which has anti-inflammatory, immune regulation, inhibition of neutrophil chemotaxis etc. Moreover, we focused our attention on discussing the clinical, pathological, and possible pathogenic aspects of the rare overlap of HIV complicated with neutrophilic and eosinophilic dermatosis. Copyright © 2020 Dong, Huang, Upadhyay, Shrestha, Zhai and Li.Objective We assessed the prognostic value of quantitative indices extracted from bone SPECT-CT to evaluate the response of bone metastatic castrate-resistant prostate cancer (BmCRPC) to abiraterone. Methods Consecutive patients with BmCRPC initiating treatment with abiraterone from March 2014 to March 2015 were prospectively included. Three 2-bed SPECT-CT [at baseline [M0], after 3 months [M3], and 6 months [M6] of treatment], were planned (Symbia Intevo®, Siemens). SPECT data were reconstructed using an Ordered Subset Conjugate Gradient Minimization (OSCGM) algorithm allowing SUV quantification. SUVmax and SUVpeak of the highest uptake lesion were measured in each SPECT-CT. Total Neoplastic Osteoblastic Metabolic Volume (NOMV) was assessed. PSA level was recorded at baseline, M3, and M6 of treatment. Overall survival (OS), progression-free survival (PFS), and disease-specific survival (DSS) were calculated. Results Nineteen patients aged 71.1 ± 7.7 years were included. Low M0 SUVmax was significantly predictive of longer OS (p = 0.04). Low NOMV at M0 were significantly predictive of longer PFS (p = 0.02). Patients with increase of at least 12.5% of the SUVpeak of the highest uptake lesion between M0 and M3 (ΔSUVpeakM0M3) had a significantly longer OS (p = 0.03). Patients with increase (or decrease lesser than 25%) of ΔSUVpeakM0M3 had a significantly longer DSS (p = 0.01). Patients with increase of NOMV of at least 45% between M0 and M6 had a significantly shorter PFS (p less then 0.001). Variations of NOMV between M0 and M6 were significantly correlated with PSA variations between M0 and M6 (rs = 0.73, p = 0.02). Conclusions Quantitative bone SPECT-CT appears to be a promising tool of BmCRPC assessment. Early flare-up phenomenon seems to predict longer OS. Copyright © 2020 de Laroche, Bourhis, Robin, Delcroix, Querellou, Malhaire, Schlurmann, Bourbonne, Salaün, Schick and Abgral.α-klotho (KL) is an anti-aging protein and has been shown to exert anti-inflammatory and anti-oxidative effects in the lung and pulmonary diseases such as chronic obstructive pulmonary disease (COPD) and cystic fibrosis. The current study investigated the direct effect of KL on the bronchial epithelium in regards to mucociliary clearance parameters. Primary human bronchial and murine tracheal epithelial cells, cultured, and differentiated at the air liquid interface (ALI), were treated with recombinant KL or infected with a lentiviral vector expressing KL. Airway surface liquid (ASL) volume, airway ion channel activities, and expression levels were analyzed. These experiments were paired with ex vivo analyses of mucociliary clearance in murine tracheas from klotho deficient mice and their wild type littermates. Our results showed that klotho deficiency led to impaired mucociliary clearance with a reduction in ASL volume in vitro and ex vivo. Overexpression or exogenous KL increased ASL volume, which was paralleled by increased activation of the large-conductance, Ca2+-activated, voltage-dependent potassium channel (BK) without effect on the cystic fibrosis transmembrane conductance regulator (CFTR). Furthermore, KL overexpression downregulated IL-8 levels and attenuated TGF-β-mediated downregulation of LRRC26, the γ subunit of BK, necessary for its function in non-excitable cells. In summary, we show that KL regulates mucociliary function by increasing ASL volume in the airways possibly due to underlying BK activation. The KL mediated BK channel activation may be a potentially important target to design therapeutic strategies in inflammatory airway diseases when ASL volume is decreased. Copyright © 2020 Garth, Easter, Skylar Harris, Sailland, Kuenzi, Chung, Dennis, Baumlin, Adewale, Rowe, King, Faul, Barnes, Salathe and Krick.