We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. ...We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.展开更多
目的探讨复发/难治性多发性骨髓瘤伴继发性髓外病变患者达雷妥尤单抗联合苯达莫司汀治疗的疗效和安全性。方法回顾性分析2021年1月至2023年12月于广西医科大学附属肿瘤医院住院治疗的复发/难治性多发性骨髓瘤伴继发性髓外病变患者的临...目的探讨复发/难治性多发性骨髓瘤伴继发性髓外病变患者达雷妥尤单抗联合苯达莫司汀治疗的疗效和安全性。方法回顾性分析2021年1月至2023年12月于广西医科大学附属肿瘤医院住院治疗的复发/难治性多发性骨髓瘤伴继发性髓外病变患者的临床资料。所有患者均接受至少三线治疗仍疾病进展,然后采用达雷妥尤单抗和苯达莫司汀联合治疗。分别采用国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准和美国国家癌症研究所不良事件通用术语标准(NCI-CTCAE)5.0版评估其治疗疗效和安全性。结果共12例患者纳入分析,获完全缓解2例,部分缓解4例,微小缓解2例,疾病稳定1例,疾病进展3例,总缓解率为50%,中位无进展生存期为8个月。所有患者均出现血液学毒性,其中4例为Ⅲ~Ⅳ级;3例患者在治疗期间发生肺炎;均未发生治疗相关性死亡。结论达雷妥尤单抗联合苯达莫司汀在治疗复发/难治性伴继发性髓外病变的多发性骨髓瘤中显示出有效性,且毒性作用可控,值得在更大规模的患者群体中进一步验证。展开更多
BACKGROUND Eosinophilic gastroenteritis(EGE)is a chronic recurrent disease with abnormal eosinophilic infiltration in the gastrointestinal tract.Glucocorticoids remain the most common treatment method.However,disease ...BACKGROUND Eosinophilic gastroenteritis(EGE)is a chronic recurrent disease with abnormal eosinophilic infiltration in the gastrointestinal tract.Glucocorticoids remain the most common treatment method.However,disease relapse and glucocorticoid dependence remain notable problems.To date,few studies have illuminated the prognosis of EGE and risk factors for disease relapse.AIM To describe the clinical characteristics of EGE and possible predictive factors for disease relapse based on long-term follow-up.METHODS This was a retrospective cohort study of 55 patients diagnosed with EGE admitted to one medical center between 2013 and 2022.Clinical records were collected and analyzed.Kaplan-Meier curves and log-rank tests were conducted to reveal the risk factors for long-term relapse-free survival(RFS).RESULTS EGE showed a median onset age of 38 years and a slight female predominance(56.4%).The main clinical symptoms were abdominal pain(89.1%),diarrhea(61.8%),nausea(52.7%),distension(49.1%)and vomiting(47.3%).Forty-three(78.2%)patients received glucocorticoid treatment,and compared with patients without glucocorticoid treatments,they were more likely to have elevated serum immunoglobin E(IgE)(86.8%vs 50.0%,P=0.022)and descending duodenal involvement(62.8%vs 27.3%,P=0.046)at diagnosis.With a median follow-up of 67 mo,all patients survived,and 56.4%had at least one relapse.Six variables at baseline might have been associated with the overall RFS rate,including age at diagnosis<40 years[hazard ratio(HR)2.0408,95%confidence interval(CI):1.0082–4.1312,P=0.044],body mass index(BMI)>24 kg/m^(2)(HR 0.3922,95%CI:0.1916-0.8027,P=0.014),disease duration from symptom onset to diagnosis>3.5 mo(HR 2.4725,95%CI:1.220-5.0110,P=0.011),vomiting(HR 3.1259,95%CI:1.5246-6.4093,P=0.001),total serum IgE>300 KU/L at diagnosis(HR 0.2773,95%CI:0.1204-0.6384,P=0.022)and glucocorticoid treatment(HR 6.1434,95%CI:2.8446-13.2676,P=0.003).CONCLUSION In patients with EGE,younger onset age,longer disease course,vomiting and glucocorticoid treatment were risk factors for disease relapse,whereas higher BMI and total IgE level at baseline were protective.展开更多
文摘We reported a biopsy proved case of minimal change nephrotic syndrome in a 72-year-old patient. The minimal change nephrotic syndrome has been steroid sensitive, but the patient had 7 relapses over a span of 5 years. Each time the dose of steroid is tapered, a relapse of the nephrotic syndrome occurred. Eventually, the patient was complaining of dysphagia and difficulty swallowing. Hospital work-up with barium swallow, endoscopy, and CT of the chest, abdomen and pelvis, revealed a focal stenotic lesion with mild to moderate esophageal dysmotility 7/15/2022. A diagnosis of an ulcerating lesion with biopsy confirmed a neuro-endocrine carcinoma of the gastro-esophageal junction was entertained. The CT of the chest/abdomen/pelvis, 7/19/2022, has shown, an esophageal mass of 5.1 × 5.6 × 7 cm of the gastro-esophageal junction with ulceration. No evidence of spread beyond the esophagus and stomach. The histology revealed a poorly differentiated neuroendocrine tumor of the gastro-esophageal junction. The patient underwent several rounds of chemotherapy, radiation, and surgery culminating in tumor control. His nephrotic syndrome was resolved after the tumor has been controlled by surgery and chemotherapy.
文摘目的探讨复发/难治性多发性骨髓瘤伴继发性髓外病变患者达雷妥尤单抗联合苯达莫司汀治疗的疗效和安全性。方法回顾性分析2021年1月至2023年12月于广西医科大学附属肿瘤医院住院治疗的复发/难治性多发性骨髓瘤伴继发性髓外病变患者的临床资料。所有患者均接受至少三线治疗仍疾病进展,然后采用达雷妥尤单抗和苯达莫司汀联合治疗。分别采用国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准和美国国家癌症研究所不良事件通用术语标准(NCI-CTCAE)5.0版评估其治疗疗效和安全性。结果共12例患者纳入分析,获完全缓解2例,部分缓解4例,微小缓解2例,疾病稳定1例,疾病进展3例,总缓解率为50%,中位无进展生存期为8个月。所有患者均出现血液学毒性,其中4例为Ⅲ~Ⅳ级;3例患者在治疗期间发生肺炎;均未发生治疗相关性死亡。结论达雷妥尤单抗联合苯达莫司汀在治疗复发/难治性伴继发性髓外病变的多发性骨髓瘤中显示出有效性,且毒性作用可控,值得在更大规模的患者群体中进一步验证。
基金National High Level Hospital Clinical Research Funding,No.2022-PUMCH-B-022CAMS Innovation Fund for Medical Sciences,No.CIFMS 2021-1-I2M-003and Undergraduate Innovation Program,No.2023zglc06076.
文摘BACKGROUND Eosinophilic gastroenteritis(EGE)is a chronic recurrent disease with abnormal eosinophilic infiltration in the gastrointestinal tract.Glucocorticoids remain the most common treatment method.However,disease relapse and glucocorticoid dependence remain notable problems.To date,few studies have illuminated the prognosis of EGE and risk factors for disease relapse.AIM To describe the clinical characteristics of EGE and possible predictive factors for disease relapse based on long-term follow-up.METHODS This was a retrospective cohort study of 55 patients diagnosed with EGE admitted to one medical center between 2013 and 2022.Clinical records were collected and analyzed.Kaplan-Meier curves and log-rank tests were conducted to reveal the risk factors for long-term relapse-free survival(RFS).RESULTS EGE showed a median onset age of 38 years and a slight female predominance(56.4%).The main clinical symptoms were abdominal pain(89.1%),diarrhea(61.8%),nausea(52.7%),distension(49.1%)and vomiting(47.3%).Forty-three(78.2%)patients received glucocorticoid treatment,and compared with patients without glucocorticoid treatments,they were more likely to have elevated serum immunoglobin E(IgE)(86.8%vs 50.0%,P=0.022)and descending duodenal involvement(62.8%vs 27.3%,P=0.046)at diagnosis.With a median follow-up of 67 mo,all patients survived,and 56.4%had at least one relapse.Six variables at baseline might have been associated with the overall RFS rate,including age at diagnosis<40 years[hazard ratio(HR)2.0408,95%confidence interval(CI):1.0082–4.1312,P=0.044],body mass index(BMI)>24 kg/m^(2)(HR 0.3922,95%CI:0.1916-0.8027,P=0.014),disease duration from symptom onset to diagnosis>3.5 mo(HR 2.4725,95%CI:1.220-5.0110,P=0.011),vomiting(HR 3.1259,95%CI:1.5246-6.4093,P=0.001),total serum IgE>300 KU/L at diagnosis(HR 0.2773,95%CI:0.1204-0.6384,P=0.022)and glucocorticoid treatment(HR 6.1434,95%CI:2.8446-13.2676,P=0.003).CONCLUSION In patients with EGE,younger onset age,longer disease course,vomiting and glucocorticoid treatment were risk factors for disease relapse,whereas higher BMI and total IgE level at baseline were protective.
