BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevate...BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevated plasma cells,platelets,and vascular endothelial growth factor levels.Although heart disease rarely occurs in POEMS syndrome,the death rate increases sharply after heart failure.We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later.CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities.She could not lie down and rest at night.Three months prior,she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities.The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years,pain in the left thigh,and edema in both legs for more than 2 mo.After a multisystem evaluation,she was diagnosed with POEMS syndrome.Her echocardiogram showed left ventricular dilation with systolic dysfunction,and the left ventricular ejection fraction was only 38%with severely elevated brain natriuretic peptide.She received a combination of dexamethasone and thalidomide for 1 mo,but her symptoms did not improve.Therefore,we added a two-per-week bortezomib injection.After 2 wk,the patient’s heart function had improved significantly.CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.展开更多
BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro...BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.展开更多
BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome a...BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.展开更多
脂肪和肥胖相关蛋白(fat mass and obesity-associated protein,FTO)作为首个被发现的N6-甲基腺苷(N6-methyladenosine,m^(6)A)去甲基化酶,在m^(6)A甲基化修饰过程中发挥关键作用。FTO通过充当甲基化修饰的“擦除器”,动态调控RNA表观...脂肪和肥胖相关蛋白(fat mass and obesity-associated protein,FTO)作为首个被发现的N6-甲基腺苷(N6-methyladenosine,m^(6)A)去甲基化酶,在m^(6)A甲基化修饰过程中发挥关键作用。FTO通过充当甲基化修饰的“擦除器”,动态调控RNA表观遗传修饰的平衡,进而影响多种人体病理生理过程。研究表明,m^(6)A甲基化修饰在心血管疾病(cardiovascular disease,CVD)的发生发展中起着重要的调控作用,而FTO可能成为治疗CVD的潜在靶点。本文系统综述了FTO的分子结构特征、其在m^(6)A甲基化修饰过程中发挥的作用及在CVD中的调控机制,重点探讨了FTO在不同类型CVD中的调控功能及其治疗潜力。展开更多
文摘BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a plasma cell proliferative disorder.The syndrome is characterized by elevated plasma cells,platelets,and vascular endothelial growth factor levels.Although heart disease rarely occurs in POEMS syndrome,the death rate increases sharply after heart failure.We report a patient who initially presented with an endocrine disease and developed congestive heart failure related to POEMS syndrome 9 years later.CASE SUMMARY A 23-year-old woman with no history of menstruation and a 9-year history of type I diabetes reported feeling breathless after activities.She could not lie down and rest at night.Three months prior,she experienced pain and increased tension in her left thigh accompanied by tenderness and edema in both lower extremities.The chief complaint upon hospital admission was that blood sugar has increased for more than 9 years,pain in the left thigh,and edema in both legs for more than 2 mo.After a multisystem evaluation,she was diagnosed with POEMS syndrome.Her echocardiogram showed left ventricular dilation with systolic dysfunction,and the left ventricular ejection fraction was only 38%with severely elevated brain natriuretic peptide.She received a combination of dexamethasone and thalidomide for 1 mo,but her symptoms did not improve.Therefore,we added a two-per-week bortezomib injection.After 2 wk,the patient’s heart function had improved significantly.CONCLUSION This case provides information about the treatment of POEMS syndrome with complications and highlights the challenges of developing a standardized treatment.
文摘BACKGROUND Polyneuropathy,organomegaly,endocrinopathy,M-protein,skin changes(POEMS)syndrome is a rare paraneoplastic syndrome that encompass multiple systems.The most common clinical symptoms of POEMS syndrome are pro-gressive sensorimotor polyneuropathy,organ enlargement,endocrine disorders,darkening skin,a monoclonal plasma cell proliferative disorder,and lymph node hyperplasia.The organomegaly consists of hepatosplenomegaly and/or lym-phadenopathy;cases of cardiomyopathy are rare.Diagnoses are often delayed because of the atypical nature of the syndrome,exposing patients to possibly severe disability.Therefore,identifying atypical symptoms can improve the prognosis and quality of life among POEMS syndrome patients.lenalidomide and dexamethasone.CONCLUSION When patients with cardiomyopathy have systemic manifestations such as numb limbs and darkening skin,the POEMS syndrome is the most possible diagnosis.
文摘BACKGROUND Polyneuropathy organomegaly endocrinopathy M-protein and skin changes(POEMS)syndrome is a rare paraneoplastic syndrome caused by a potential plasma cell tumor.The clinical manifestations of POEMS syndrome are diverse.Due to the insidious onset and lack of specific early-stage manifestations,POEMS syndrome is easily misdiagnosed or never diagnosed,leading to delayed treatment.Neurological symptoms are usually the first clinical manifestation,while ascites is a rare symptom in patients with POEMS syndrome.CASE SUMMARY A female patient presented with unexplained ascites as an initial symptom,which is a rare early-stage manifestation of the condition.After 1 year,the patient gradually developed progressive renal impairment,anemia,polyserosal effusion,edema,swollen lymph nodes on the neck,armpits,and groin,and decreased muscle strength of the lower extremities.The patient was eventually diagnosed with POEMS syndrome after multidisciplinary team discussion.Treatment comprised bortezomib+dexamethasone,continuous renal replacement therapy,chest and abdominal closed drainage,transfusions of erythrocytes and platelets,and other symptomatic and supportive treatments.The patient’s condition initially improved after treatment.However,then her symptoms worsened,and she succumbed to the illness and died.CONCLUSION Ascites is a potential early manifestation of POEMS syndrome,and this diagnosis should be considered for patients with unexplained ascites.Furthermore,multidisciplinary team discussion is helpful in diagnosing POEMS syndrome.
文摘脂肪和肥胖相关蛋白(fat mass and obesity-associated protein,FTO)作为首个被发现的N6-甲基腺苷(N6-methyladenosine,m^(6)A)去甲基化酶,在m^(6)A甲基化修饰过程中发挥关键作用。FTO通过充当甲基化修饰的“擦除器”,动态调控RNA表观遗传修饰的平衡,进而影响多种人体病理生理过程。研究表明,m^(6)A甲基化修饰在心血管疾病(cardiovascular disease,CVD)的发生发展中起着重要的调控作用,而FTO可能成为治疗CVD的潜在靶点。本文系统综述了FTO的分子结构特征、其在m^(6)A甲基化修饰过程中发挥的作用及在CVD中的调控机制,重点探讨了FTO在不同类型CVD中的调控功能及其治疗潜力。
文摘目的研究肝细胞肝癌(HCC)中甲基转移酶样蛋白5(METTL5)、病毒样m^(6)A甲基转化酶(VIRMA)的表达及与临床预后的关系。方法选取2018年3月至2021年3月就诊于该院的89例HCC患者。应用免疫组化和荧光定量PCR检测组织METTL5、VIRMA蛋白和m RNA表达。利用Kaplan-Meier曲线和Cox回归分析METTL5、VIRMA蛋白对HCC预后的影响。结果HCC癌组织METTL5 m RNA(3.23±0.55 vs.0.61±0.20),VIRMA m RNA(2.97±0.42 vs.0.58±0.17)高于癌旁组织,差异有统计学意义(t=42.234、49.762,均P<0.001)。METTL5、VIRMA蛋白位于细胞质和细胞膜,部分位于细胞核。HCC癌组织METTL5[71.91%(64/89)vs.5.62%(5/89)]、VIRMA[69.66%(62/89)vs.6.74%(6/89)]阳性率高于癌旁组织,差异有统计学意义(χ^(2)=82.385、74.627,P<0.001,P=0.001)。HCC中METTL5、VIRMA蛋白表达与肝癌分期标准(CNLC)、肿瘤最大径及血管侵犯有关,CNLC分期Ⅱ~Ⅲ期、肿瘤最大径≥5 cm及血管侵犯的HCC癌组织中METTL5、VIRMA蛋白阳性率较高(均P<0.05)。METTL5阳性组和阴性组3年生存率为34.88%(15/43),67.39%(31/46),差异有统计学意义(Log-rankχ^(2)=7.893,P=0.005)。VIRMA阳性组和阴性组3年生存率为36.36%(16/44)、66.67%(30/45),差异有统计学意义(Log-rankχ^(2)=9.828,P=0.002)。CNLC分期Ⅱ~Ⅲ期、肿瘤最大径≥5 cm、血管侵犯、METTL5阳性、VIRMA阳性是影响HCC患者不良预后的危险因素。结论HCC中METTL5、VIRMA表达上调,均与CNLC分期Ⅱ~Ⅲ期、肿瘤最大径及血管侵犯有关,是评估HCC预后的肿瘤标志物。
