BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resu...BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resulting in multiple-organ dysfunction. The urgent event of thromboembolism in the pulmonary region provoked by eosinophils in idiopathic HES(IHES) is relatively unusual. This article reports a case of IHES with multiple PEs and left leg venous thrombosis as the first manifestation. One month later, the patient developed Henoch-Schonlein purpura(HSP), which is very rare.CASE SUMMARY We report the case of a 12-year-old boy who was admitted to the hospital with dyspnea, left leg pain, and aggravation. He had bilateral PE and left leg venous embolism with mild eosinophilia. Low-molecular-weight heparin and urokinase were given. At the same time, the interventional department was contacted about filter implantation, followed by urokinase thrombolysis. The left leg thrombus was aspirated under ultrasound guidance. He was discharged from the hospital on rivaroxaban. One month later, he developed a rash on both legs and ankle pain consistent with HSP, with severe eosinophilia and motor and sensory disturbances. The patient was diagnosed with IHES with multiple embolisms complicated by HSP after excluding other causes of the eosinophil elevation. After glucocorticoid treatment, the symptoms were relieved, but the patient later developed purpura nephritis.CONCLUSION We report a rare and life-threatening case of IHES with multiple embolisms associated with HSP.A mild elevation of eosinophils early in the disease leads to difficulties in diagnosis and delayed treatment.展开更多
Objective:To study the effect of Helicobacter pylori infection on renal function and immune response in children with Henoch-Sch?oenlein purpura (HSP).Methods: Children diagnosed with Henoch-Sch?oenlein purpura in Zho...Objective:To study the effect of Helicobacter pylori infection on renal function and immune response in children with Henoch-Sch?oenlein purpura (HSP).Methods: Children diagnosed with Henoch-Sch?oenlein purpura in Zhognshan Chenxinghai Hospital and Huizhou Third People's Hospital between May 2013 and July 2016 were selected and divided into Hp(﹢) and Hp(-) HSP group according to Helicobacter pylori infection condition. Then renal function and immune response were determined.Results: Scr, BUN, CysC, TM, vWF, sVCAM-1, CTGF, MMP9, IL-17 and IL-23 contents in serum as well as Th17 cell content in peripheral blood of Hp(﹢) HSP group were significantly higher than those of Hp(-) HSP group, Treg cell content in peripheral blood as well as TGF-β1 and IL-10 contents in serum of Hp(﹢) HSP group were significantly lower than those of Hp(-) HSP group, SOCS1 and Tim1 mRNA expression in peripheral blood mononuclear cells of Hp(﹢) HSP group were significantly higher than those of Hp(-) HSP group, and SOCS3 and Tim3 mRNA expression were not significantly different from those of Hp(-) HSP group.Conclusion:Helicobacter pylori infection in children with Henoch-Sch?oenlein purpura can aggravate the damage of renal function and the disorder of autoimmune response.展开更多
Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical cons...Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical consequences of microvascular thrombosis such as stroke. The exact cause is not known but it is associated with a deficiency of ADAMTS13 enzymes. Immune mediated TTP is more common and can present in pregnancy. The aim of this case is to bring awareness as many clinicians are unaware of this condition in pregnancy, its diagnosis may be missed or delayed, leading to fetal loss or serious maternal implications. In this case the patient presented at 29 weeks with stroke in Emergency department, referred to delivery suit for Obstetric review, with suspicion of Pre-eclampsia/HELLP. The diagnosis of TTP was achieved by a multidisciplinary team who worked tirelessly together. The patient was transferred to a Specialist Tertiary Care Centre for further management. The pregnancy continued until 33 weeks and 5 days. She underwent an emergency caesarean section for fetal distress. Steroids and Rituximab were continued postnatally. The outcome was favourable due to fast and efficient multidisciplinary care. Awareness of this rare but important condition can lead to recognition of clinical presentation, prompt diagnosis and appropriate management.展开更多
Objective Thrombotic thrombocytopenic purpura(TTP)is a rare and fatal disease caused by a severe deficiency in the metalloprotease ADAMTS13 and is characterized by thrombotic microangiopathy.The present study aimed to...Objective Thrombotic thrombocytopenic purpura(TTP)is a rare and fatal disease caused by a severe deficiency in the metalloprotease ADAMTS13 and is characterized by thrombotic microangiopathy.The present study aimed to investigate the genes and variants associated with TTP in a Chinese population.Methods Target sequencing was performed on 220 genes related to complements,coagulation factors,platelets,fibrinolytic,endothelial,inflammatory,and anticoagulation systems in 207 TTP patients and 574 controls.Subsequently,logistic regression analysis was carried out to identify the TTP-associated genes based on the counts of rare deleterious variants in the region of a certain gene.Moreover,the associations between common variants and TTP were also investigated.Results ADAMTS13 was the only TTP-associated gene(OR=3.77;95%CI:1.82–7.81;P=3.6×10^(-4))containing rare deleterious variants in TTP patients.Among these 8 variants,5 novel rare variants that might contribute to TTP were identified,including rs200594025,rs782492477,c.T1928G(p.I643S),c.3336_3361del(p.Q1114Afs*20),and c.3469_3470del(p.A1158Sfs*17).No common variants associated with TTP were identified under the stringent criteria of correction for multiple testing.Conclusion ADAMTS13 is the primary gene related to TTP.The genetic variants associated with the occurrence of TTP were slightly different between the Chinese and European populations.展开更多
BACKGROUND For children with abdominal Henoch-Schonlein purpura presenting abdominal pain as an initial symptom and severe clinical manifestations,but without purpura appearance on the skin,the diagnosis and treatment...BACKGROUND For children with abdominal Henoch-Schonlein purpura presenting abdominal pain as an initial symptom and severe clinical manifestations,but without purpura appearance on the skin,the diagnosis and treatment are relatively difficult.This study summarized the characteristics of this group of patients by literature review and provided additional references for further refinement of glucocorticoid therapy in this vasculitis.CASE SUMMARY A 6-year-old girl presented mainly with repeated abdominal pain and had received short-term out-of-hospital treatment with hydrocortisone.On day 7 after onset,gastroscopy revealed chronic non-atrophic gastritis and erosive duodenitis without purpuric rash,and no obvious resolution of the abdominal pain was found after treatment against infection and for protection of gastric mucosa.On day 14 the inflammatory indices continued to rise and the pain was relieved after enhanced anti-infective therapy,but without complete resolution.On day 19,the patient presented with aggravated abdominal pain with purplish-red dots on the lower limbs,by which Henoch-Schonlein purpura was confirmed.After 5 d of sequential treatment with methylprednisolone and prednisone,abdominal pain disappeared and she was discharged.CONCLUSION Henoch-Schonlein purpura-related rash may appear after long-term abdominal pain,and should be distinguished from acute and chronic gastrointestinal diseases at the early stage without typical rash.For bacterial infection-induced Henoch-Schonlein purpura,glucocorticoid therapy alone without clearing the infection may not relieve symptoms.展开更多
Objective:To analyze the curative effect of rituximab combined with plasma exchange in the treatment of thrombotic thrombocytopenic purpura.Methods:70 patients with thrombotic thrombocytopenic purpura that were treate...Objective:To analyze the curative effect of rituximab combined with plasma exchange in the treatment of thrombotic thrombocytopenic purpura.Methods:70 patients with thrombotic thrombocytopenic purpura that were treated in our hospital from January 2022 to January 2023 were selected for this study.They were divided into two groups according the treatment method they were about to receive.The patients in the control group received plasma exchange.The observation group was given rituximab in addition to plasma exchange.Then,the therapeutic effects of the two groups were observed,and the incidence of adverse reactions was compared.Results:The rate of effectiveness of the treatment received in observation group and the control group was 97.14%and 82.86%,respectively.The treatment received in observation group had a better therapeutic effect(P<0.05).The incidence of adverse reactions in the observation group(22.86%)was lower than that of the control group(5.71%),with P<0.05.Conclusion:Rituximab combined with plasma exchange is relatively more effective than plasma exchange alone,with less adverse reaction,making it a viable treatment option.展开更多
文摘BACKGROUND The incidence of pulmonary embolism(PE) in children is low, but its mortality is high. Hypereosinophilic syndrome(HES) is a group of diseases caused by an abnormal increase in eosinophilic granulocytes resulting in multiple-organ dysfunction. The urgent event of thromboembolism in the pulmonary region provoked by eosinophils in idiopathic HES(IHES) is relatively unusual. This article reports a case of IHES with multiple PEs and left leg venous thrombosis as the first manifestation. One month later, the patient developed Henoch-Schonlein purpura(HSP), which is very rare.CASE SUMMARY We report the case of a 12-year-old boy who was admitted to the hospital with dyspnea, left leg pain, and aggravation. He had bilateral PE and left leg venous embolism with mild eosinophilia. Low-molecular-weight heparin and urokinase were given. At the same time, the interventional department was contacted about filter implantation, followed by urokinase thrombolysis. The left leg thrombus was aspirated under ultrasound guidance. He was discharged from the hospital on rivaroxaban. One month later, he developed a rash on both legs and ankle pain consistent with HSP, with severe eosinophilia and motor and sensory disturbances. The patient was diagnosed with IHES with multiple embolisms complicated by HSP after excluding other causes of the eosinophil elevation. After glucocorticoid treatment, the symptoms were relieved, but the patient later developed purpura nephritis.CONCLUSION We report a rare and life-threatening case of IHES with multiple embolisms associated with HSP.A mild elevation of eosinophils early in the disease leads to difficulties in diagnosis and delayed treatment.
基金Zhongshan Science and Technology Plan Project No:20122A065
文摘Objective:To study the effect of Helicobacter pylori infection on renal function and immune response in children with Henoch-Sch?oenlein purpura (HSP).Methods: Children diagnosed with Henoch-Sch?oenlein purpura in Zhognshan Chenxinghai Hospital and Huizhou Third People's Hospital between May 2013 and July 2016 were selected and divided into Hp(﹢) and Hp(-) HSP group according to Helicobacter pylori infection condition. Then renal function and immune response were determined.Results: Scr, BUN, CysC, TM, vWF, sVCAM-1, CTGF, MMP9, IL-17 and IL-23 contents in serum as well as Th17 cell content in peripheral blood of Hp(﹢) HSP group were significantly higher than those of Hp(-) HSP group, Treg cell content in peripheral blood as well as TGF-β1 and IL-10 contents in serum of Hp(﹢) HSP group were significantly lower than those of Hp(-) HSP group, SOCS1 and Tim1 mRNA expression in peripheral blood mononuclear cells of Hp(﹢) HSP group were significantly higher than those of Hp(-) HSP group, and SOCS3 and Tim3 mRNA expression were not significantly different from those of Hp(-) HSP group.Conclusion:Helicobacter pylori infection in children with Henoch-Sch?oenlein purpura can aggravate the damage of renal function and the disorder of autoimmune response.
文摘Thrombotic thrombocytopenic purpura (TTP) is a rare but acute, life-threatening condition which may be precipitated by pregnancy. This disorder that presents with thrombocytopenia, haemolytic anemia, and clinical consequences of microvascular thrombosis such as stroke. The exact cause is not known but it is associated with a deficiency of ADAMTS13 enzymes. Immune mediated TTP is more common and can present in pregnancy. The aim of this case is to bring awareness as many clinicians are unaware of this condition in pregnancy, its diagnosis may be missed or delayed, leading to fetal loss or serious maternal implications. In this case the patient presented at 29 weeks with stroke in Emergency department, referred to delivery suit for Obstetric review, with suspicion of Pre-eclampsia/HELLP. The diagnosis of TTP was achieved by a multidisciplinary team who worked tirelessly together. The patient was transferred to a Specialist Tertiary Care Centre for further management. The pregnancy continued until 33 weeks and 5 days. She underwent an emergency caesarean section for fetal distress. Steroids and Rituximab were continued postnatally. The outcome was favourable due to fast and efficient multidisciplinary care. Awareness of this rare but important condition can lead to recognition of clinical presentation, prompt diagnosis and appropriate management.
基金supported by the National Natural Science Foundation of China(No.82003561).
文摘Objective Thrombotic thrombocytopenic purpura(TTP)is a rare and fatal disease caused by a severe deficiency in the metalloprotease ADAMTS13 and is characterized by thrombotic microangiopathy.The present study aimed to investigate the genes and variants associated with TTP in a Chinese population.Methods Target sequencing was performed on 220 genes related to complements,coagulation factors,platelets,fibrinolytic,endothelial,inflammatory,and anticoagulation systems in 207 TTP patients and 574 controls.Subsequently,logistic regression analysis was carried out to identify the TTP-associated genes based on the counts of rare deleterious variants in the region of a certain gene.Moreover,the associations between common variants and TTP were also investigated.Results ADAMTS13 was the only TTP-associated gene(OR=3.77;95%CI:1.82–7.81;P=3.6×10^(-4))containing rare deleterious variants in TTP patients.Among these 8 variants,5 novel rare variants that might contribute to TTP were identified,including rs200594025,rs782492477,c.T1928G(p.I643S),c.3336_3361del(p.Q1114Afs*20),and c.3469_3470del(p.A1158Sfs*17).No common variants associated with TTP were identified under the stringent criteria of correction for multiple testing.Conclusion ADAMTS13 is the primary gene related to TTP.The genetic variants associated with the occurrence of TTP were slightly different between the Chinese and European populations.
基金the Science and Technology Bureau of Sichuan province,No.21ZDYF1329.
文摘BACKGROUND For children with abdominal Henoch-Schonlein purpura presenting abdominal pain as an initial symptom and severe clinical manifestations,but without purpura appearance on the skin,the diagnosis and treatment are relatively difficult.This study summarized the characteristics of this group of patients by literature review and provided additional references for further refinement of glucocorticoid therapy in this vasculitis.CASE SUMMARY A 6-year-old girl presented mainly with repeated abdominal pain and had received short-term out-of-hospital treatment with hydrocortisone.On day 7 after onset,gastroscopy revealed chronic non-atrophic gastritis and erosive duodenitis without purpuric rash,and no obvious resolution of the abdominal pain was found after treatment against infection and for protection of gastric mucosa.On day 14 the inflammatory indices continued to rise and the pain was relieved after enhanced anti-infective therapy,but without complete resolution.On day 19,the patient presented with aggravated abdominal pain with purplish-red dots on the lower limbs,by which Henoch-Schonlein purpura was confirmed.After 5 d of sequential treatment with methylprednisolone and prednisone,abdominal pain disappeared and she was discharged.CONCLUSION Henoch-Schonlein purpura-related rash may appear after long-term abdominal pain,and should be distinguished from acute and chronic gastrointestinal diseases at the early stage without typical rash.For bacterial infection-induced Henoch-Schonlein purpura,glucocorticoid therapy alone without clearing the infection may not relieve symptoms.
文摘Objective:To analyze the curative effect of rituximab combined with plasma exchange in the treatment of thrombotic thrombocytopenic purpura.Methods:70 patients with thrombotic thrombocytopenic purpura that were treated in our hospital from January 2022 to January 2023 were selected for this study.They were divided into two groups according the treatment method they were about to receive.The patients in the control group received plasma exchange.The observation group was given rituximab in addition to plasma exchange.Then,the therapeutic effects of the two groups were observed,and the incidence of adverse reactions was compared.Results:The rate of effectiveness of the treatment received in observation group and the control group was 97.14%and 82.86%,respectively.The treatment received in observation group had a better therapeutic effect(P<0.05).The incidence of adverse reactions in the observation group(22.86%)was lower than that of the control group(5.71%),with P<0.05.Conclusion:Rituximab combined with plasma exchange is relatively more effective than plasma exchange alone,with less adverse reaction,making it a viable treatment option.
