BACKGROUND Hutchinson-Gilford progeria syndrome(HGPS)is an extremely rare disease characterized by the rapid appearance of aging with an onset in childhood.Serious cardiovascular complications can be life-threatening ...BACKGROUND Hutchinson-Gilford progeria syndrome(HGPS)is an extremely rare disease characterized by the rapid appearance of aging with an onset in childhood.Serious cardiovascular complications can be life-threatening events for affected patients and the cause of early death.Herein we report a HGPS patient with osteosarcoma hat was successfully managed and is alive 13 years after the diagnosis.This is the first report describing the detailed surgical procedure and long-term follow-up of osteosarcoma in a patient with HGPS.CASE SUMMARY The patient was diagnosed with HGPS at 5 years of age with typical features and was referred to our department with a suspected bone tumor of the left proximal tibia at the age of 18.Open biopsy of the tibial bone tumor revealed a conventional fibroblastic osteosarcoma.We have developed and performed a freezing technique using liquid nitrogen for tumor reconstruction.This technique overcame the small size of the tibia for megaprosthesis and avoided amputation and limb salvage was achieved 13 years post-operatively.Although the patient had a number of surgical site complications,such as wound dehiscence,and superficial and deep infections due to vulnerable skin in HGPS,no recurrence or metastases were detected for 13 years,and she walks assisted by crutches.Her general health was good at the latest follow-up at 31 years of age.CONCLUSION A HGPS patient with osteosarcoma was successfully managed and she was alive 13 years after the diagnosis.展开更多
Early surgical resection and chemotherapy of bone cancer are commonly used in the treatment of bone tumor,but it is still highly challenging to prevent recurrence and fill the bone defect caused by the resection site....Early surgical resection and chemotherapy of bone cancer are commonly used in the treatment of bone tumor,but it is still highly challenging to prevent recurrence and fill the bone defect caused by the resection site.In this work,we report a rational integration of photonic-responsive two-dimensional(2D)ultrathin niobium carbide(Nb2C)MXene nanosheets(NSs)into the 3D-printed bone-mimetic scaffolds(NBGS)for osteosarcoma treatment.The integrated 2D Nb2C-MXene NSs feature specific photonic response in the second near-infrared(NIR-II)biowindow with high tissue-penetrating depth,making it highly efficient in killing bone cancer cells.Importantly,Nb-based species released by the biodegradation of Nb2C MXene can obviously promote the neogenesis and migration of blood vessels in the defect site,which can transport more oxygen,vitamins and energy around the bone defect for the reparative process,and gather more immune cells around the defect site to accelerate the degradation of NBGS.The degradation of NBGS provides sufficient space for the bone remodeling.Besides,calcium and phosphate released during the degradation of the scaffold can promote the mineralization of new bone tissue.The intrinsic multifunctionality of killing bone tumor cell and promoting angiogenesis and bone regeneration makes the engineered Nb2C MXeneintegrated composite scaffolds a distinctive implanting biomaterial on the efficient treatment of bone tumor.展开更多
文摘BACKGROUND Hutchinson-Gilford progeria syndrome(HGPS)is an extremely rare disease characterized by the rapid appearance of aging with an onset in childhood.Serious cardiovascular complications can be life-threatening events for affected patients and the cause of early death.Herein we report a HGPS patient with osteosarcoma hat was successfully managed and is alive 13 years after the diagnosis.This is the first report describing the detailed surgical procedure and long-term follow-up of osteosarcoma in a patient with HGPS.CASE SUMMARY The patient was diagnosed with HGPS at 5 years of age with typical features and was referred to our department with a suspected bone tumor of the left proximal tibia at the age of 18.Open biopsy of the tibial bone tumor revealed a conventional fibroblastic osteosarcoma.We have developed and performed a freezing technique using liquid nitrogen for tumor reconstruction.This technique overcame the small size of the tibia for megaprosthesis and avoided amputation and limb salvage was achieved 13 years post-operatively.Although the patient had a number of surgical site complications,such as wound dehiscence,and superficial and deep infections due to vulnerable skin in HGPS,no recurrence or metastases were detected for 13 years,and she walks assisted by crutches.Her general health was good at the latest follow-up at 31 years of age.CONCLUSION A HGPS patient with osteosarcoma was successfully managed and she was alive 13 years after the diagnosis.
基金the financial support from the National Key R&D Program of China(Grant No.2016YFA0203700)the National Natural Science Foundation of China(Grant Nos.51872185,51722211,51672303,81672131,81672143,82072417 and 81802247)+2 种基金the Program of Shanghai Academic Research Leader(Grant No.18XD1404300)the National Key Research and Development Project of China(Grant No.2018YFC1106303)the Science and Technology Commission of Shanghai Municipality(Grant No.17060502400).
文摘Early surgical resection and chemotherapy of bone cancer are commonly used in the treatment of bone tumor,but it is still highly challenging to prevent recurrence and fill the bone defect caused by the resection site.In this work,we report a rational integration of photonic-responsive two-dimensional(2D)ultrathin niobium carbide(Nb2C)MXene nanosheets(NSs)into the 3D-printed bone-mimetic scaffolds(NBGS)for osteosarcoma treatment.The integrated 2D Nb2C-MXene NSs feature specific photonic response in the second near-infrared(NIR-II)biowindow with high tissue-penetrating depth,making it highly efficient in killing bone cancer cells.Importantly,Nb-based species released by the biodegradation of Nb2C MXene can obviously promote the neogenesis and migration of blood vessels in the defect site,which can transport more oxygen,vitamins and energy around the bone defect for the reparative process,and gather more immune cells around the defect site to accelerate the degradation of NBGS.The degradation of NBGS provides sufficient space for the bone remodeling.Besides,calcium and phosphate released during the degradation of the scaffold can promote the mineralization of new bone tissue.The intrinsic multifunctionality of killing bone tumor cell and promoting angiogenesis and bone regeneration makes the engineered Nb2C MXeneintegrated composite scaffolds a distinctive implanting biomaterial on the efficient treatment of bone tumor.