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
Henoch-Schonlein purpura (HSP) with intestinal perforation and cerebral hemorrhage is a very rare clinical condition. There has been no report of HSP complicated with both intestinal perforation and cerebral hemorrhag...Henoch-Schonlein purpura (HSP) with intestinal perforation and cerebral hemorrhage is a very rare clinical condition. There has been no report of HSP complicated with both intestinal perforation and cerebral hemorrhage until October 2012. Here we describe a case of HSP with intestinal perforation and cerebral hemorrhage in a 5-year-old girl. Plain abdominal radiograph in the erect position showed heavy gas in the right subphrenic space with an elevated diaphragm. Partial resection of the small intestine was performed, and pathological analysis suggested chronic suppurative inflammation in all layers of the ileal wall and mesentery. Seventeen days after surgery, cerebral hemorrhage developed and the patient died.展开更多
Henoch-Schonlein purpura(HSP) is a small vessel vasculitis mediated by type Ⅲ hypersensitivity with deposition of Ig A immune complex in the walls of vessels. It is a multi-system disorder characterizedby palpable pu...Henoch-Schonlein purpura(HSP) is a small vessel vasculitis mediated by type Ⅲ hypersensitivity with deposition of Ig A immune complex in the walls of vessels. It is a multi-system disorder characterizedby palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP.展开更多
Helicobacter pylori(H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extraintestinal diseases. H. pylori infection are mainly ...Helicobacter pylori(H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extraintestinal diseases. H. pylori infection are mainly acquired during childhood, and it has been reported that in endemic areas of China the infection rates are extraordinarily higher in HSP children, particular those with abdominal manifestations. Furthermore, eradication therapy may ameliorate Henoch-Schonlein purpura(HSP) manifestations and decrease the recurrence of HSP. Therefore, results suggested that detection of H. pylori infection by appropriate method ought to be applied in HSP children. Current evidences indicate that local injury of gastric mucosa and immunological events induced by H. pylori infection are involved in the development of HSP. Increased serum Ig A, cryoglobulins, C3 levels, autoimmunity, proinflammatory substances and molecular mimicry inducing immune complex and cross-reactive antibodies caused by H. pylori infection might play their roles in the course of HSP. However, there are no investigations confirming the causality between H. pylori infection and HSP, and the pathogenesis mechanism is still unclear. More bench and clinical studies need to be executed to elaborate the complex association between H. pylori and HSP.展开更多
Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventuall...Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.展开更多
Hcnoch Schoenlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin,joints,GI tract, and kidney.It preferentially affects children but may also occur in adults.We report a 60-rear-old man with HS...Hcnoch Schoenlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin,joints,GI tract, and kidney.It preferentially affects children but may also occur in adults.We report a 60-rear-old man with HSP who presented with colicky abdominal pain,blondy diarrhea,arthralgia,and skin rash.The gastrointestinal tract was viewed by upper endoscopy and colonoscopy.We found characterislic endoscopic findings in the stomach,cecum and sigmoid colon,the combination of which has rarely been demonstrated in one patient.Histologic examination of skin biopsy specimens revealed leukocytoclastic vasculitis with positive staining for IgA in the capillaries.Endoscopy appears to have substantial diagnostic utility in patients suspected of having HSP,especially when abdominal symptoms precede the cutaneous lesions.展开更多
The aim of this study was to describe the common presentation, frequency, and complications of Henoch-Schonlein purpura (HSP) in patients <18 years who were followed up at King Abdulaziz University Hospital, Jeddah...The aim of this study was to describe the common presentation, frequency, and complications of Henoch-Schonlein purpura (HSP) in patients <18 years who were followed up at King Abdulaziz University Hospital, Jeddah over the last 12 years. We performed a retrospective chart review of the medical records of all patients diagnosed as HSP. During this period, only 29 cases were reported (15 males, 14 females), with the mean age at the diagnosis 7.5 years. 82% percent of the patients had joint involvement in the form of arthritis or arthralgia;17.2% had no joint involvement. Abdominal manifestations were reported in 72.4% of the patients, while renal involvement was documented in 24.1% of the cases;two patients had scrotal involvement. Four patients (13.7%) had a recurrence within four months of HSP diagnosis. However, all patients had full recovery within a month. More research is warranted to study the prevalence, clinical manifestations, preceding factors, and complications of HSP in a Saudi-based cohort.展开更多
Infliximab (IFX) is an anti-tumor necrosis factorchimeric antibody that is effective for treatment ofautoimmune disorders such as Crohn's disease andulcerative colitis (UC). IFX is well tolerated with alow incide...Infliximab (IFX) is an anti-tumor necrosis factorchimeric antibody that is effective for treatment ofautoimmune disorders such as Crohn's disease andulcerative colitis (UC). IFX is well tolerated with alow incidence of adverse effects such as infections,skin reactions, autoimmunity, and malignancy.Dermatological manifestations can appear as infusionreaction, vasculitis, cutaneous infections, psoriasis,eczema, and skin cancer. Here, we present anunusual case of extensive and sporadic subcutaneousecchymosis in a 69-year-old woman with severe UC,partial colectomy and cecostomy, following her initialdose of IFX. The reaction occurred during infliximabinfusion, and withdrawal of IFX led to gradual alleviationof her symptoms. We concluded that Henoch-Sch?nleinpurpura, a kind of leukocytoclastic vasculitis, mighthave contributed to the development of the bruising.Although the precise mechanisms of the vasculitis arestill controversial, such a case highlights the importanceof subcutaneous adverse effects in the management ofUC with IFX.展开更多
Objective:To study the effects of methylprednisolone combined with montelukast on immune function and cytokines in children with recurrent Henoch-Schonlein purpura.Methods:Children who were diagnosed with recurrent He...Objective:To study the effects of methylprednisolone combined with montelukast on immune function and cytokines in children with recurrent Henoch-Schonlein purpura.Methods:Children who were diagnosed with recurrent Henoch-Schonlein purpura in Zigong Third People's Hospital between September 2015 and August 2017 were selected as the research subjects and randomly divided into the intervention group who received methylprednisolone combined with montelukast therapy and the control group who received hydrocortisone therapy. The levels of Th1/Th2 and Th17/Treg immunity indexes in peripheral blood as well as cytokines in serum were measured before treatment as well as 4 and 8 weeks after treatment.Results: 4 weeks and 8 weeks after treatment, Th1 and Treg cell contents as well as T-bet and FoxP3 mRNA expression in peripheral blood of both groups of patients were significantly higher than those before treatment whereas Th2 and Th17 cell contents as well as GATA-3 and RORγt mRNA expression in peripheral blood and NF-κB, OPN, IL-33, MK and HMGB1 contents in serum were significantly lower than those before treatment, and Th1 and Treg cell contents as well as T-bet and FoxP3 mRNA expression in peripheral blood of intervention group were significantly higher than those of control group whereas Th2 and Th17 cell contents as well as GATA-3 and RORγt mRNA expression in peripheral blood and NF-κB, OPN, IL-33, MK and HMGB1 contents in serum were significantly lower than those of control group.Conclusion: methylprednisolone combined with montelukast treatment of recurrent Henoch-Schonlein purpura can regulate the immune function and inhibit the cytokine secretion.展开更多
Henoch-schonlein purpura (HSP) is a kind of systemic vasculitis that is common in childhood,and its pathogenesis is complicated and considered to have important relationship with lymphocytes, vascular endothelial cell...Henoch-schonlein purpura (HSP) is a kind of systemic vasculitis that is common in childhood,and its pathogenesis is complicated and considered to have important relationship with lymphocytes, vascular endothelial cells and so on. The causes of this disease are complex and have not been clearly identified, but numerous studies have shown that inflammatory factors such as IL-1, IL-17 and TNF-α play an important role in the development of HSP.展开更多
Objective:To evaluate the cytokine levels and immune response status of montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura.Methods: A total of 80 patients with Henoch-Schonlein pur...Objective:To evaluate the cytokine levels and immune response status of montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura.Methods: A total of 80 patients with Henoch-Schonlein purpura who were treated in Ankang Central Hospital between May 2014 and January 2017 were collected and divided into montelukast group, loratadine group, tanshinone group and combined treatment group according to the random number table, 20 cases in each group. Serum levels of inflammatory factors, Th17/Treg cellular immunity indexes before and after treatment were compared among four groups of patients.Results: Before treatment, differences in serum levels of inflammatory factors and Th17/Treg cellular immunity indexes were not statistically significant among four groups of patients. After treatment, serum HMGB1, IL-8, IL-14, IL-23 and IL-33 levels in combined treatment group were lower than those in montelukast group, loratadine group and tanshinone group;serum IL-17 level was lower than that in montelukast group, loratadine group and tanshinone group while IL-10 and TGF-β levels were higher than those in montelukast group, loratadine group and tanshinone group.Conclusions: Montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura helps to reduce systemic inflammatory response and balance Th17/Treg cell immunity.展开更多
Objective:This study aims to explore the regularity of syndrome differentiation and treatment of traditional Chinese medicine(TCM)for allergic purpura.Methods:CNKI,Weipu Chinese science and technology database,wanfang...Objective:This study aims to explore the regularity of syndrome differentiation and treatment of traditional Chinese medicine(TCM)for allergic purpura.Methods:CNKI,Weipu Chinese science and technology database,wanfang medical network database,and Chinese biomedical literature database were searched for eligible studies.Medical records including complete patient personal information,patient symptoms,TCM syndromes,treatment,and medication were included.The data was analyzed using the Chinese medicine heritage support platform provided by the Chinese Academy of Chinese medicine(V2.5).Results:Differentiation of health gas camp blood was the most commonly used method of differentiation of symptoms and signs in famous veteran TCM.The treatment included cooling blood,activating blood circulation,clearing heat and detoxifying toxins,removing blood stasis and stopping bleeding.Honeysuckle,Forsythia suspensa,cicada slough and other drugs were interrelated.Potential drug pair combinations and drug networks showed the basic drug composition of Qingying Decoction.According to the entropy cluster analysis,28 core drug combination and 12 new formulations were obtained.Conclusion:The regularity of syndrome differentiation and treatment of traditional Chinese medicine for Henoch-Schonlein purpura based on the famous and old TCM doctors was complex.Further researches are still needed.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:To detect serum OPN and NF-κB contents in children with Henoch-Schonlein purpura (HSP) and study their correlation with oxidative stress and cellular immune function. Methods: The children who were diagnose...Objective:To detect serum OPN and NF-κB contents in children with Henoch-Schonlein purpura (HSP) and study their correlation with oxidative stress and cellular immune function. Methods: The children who were diagnosed with HSP in Shijiazhuang First Hospital between February 2015 and October 2017 were selected as the HSP group of the study and the healthy children who received physical examination during the same period were selected as the control group. The serum was collected to measure the contents of OPN, NF-κB, oxidative stress indexes and immune cell inflammation, and the peripheral blood was collected to detect the mRNA expression of immune cell transcription factors.Results: OPN, NF-κB, MDA, MPO, IL-4, IL-5 and IL-17 contents in serum as well as GATA-3 and RORγt mRNA expression in peripheral blood of HSP group were significantly higher than those of control group whereas SOD, CAT, PON, IFN-γ and TGF-β1 contents in serum as well as T-bet and FoxP3 mRNA expression in peripheral blood were significantly lower than those of control group;serum OPN and NF-κB contents in HSP group were positively correlated with MDA, MPO, IL-4, IL-5 and IL-17 contents in serum as well as GATA-3 and RORγt mRNA expression in peripheral blood, and negatively correlated with SOD, CAT, PON, IFN-γand TGF-β1 contents in serum as well as T-bet and FoxP3 mRNA expression in peripheral blood. Conclusion: The abnormal increase of serum OPN and and NF-κB contents in children with HSP is closely related to the excessive oxidative stress activation and cellular immune dysfunction.展开更多
Objective:To study the effects of suppressor of cytokine signaling 1 (SOCS1) and SOCS3 in peripheral blood on CD4+T cell differentiation in children with Henoch-Schonlein purpura. Methods: Children with Henoch-Schonle...Objective:To study the effects of suppressor of cytokine signaling 1 (SOCS1) and SOCS3 in peripheral blood on CD4+T cell differentiation in children with Henoch-Schonlein purpura. Methods: Children with Henoch-Schonlein purpura who were treated in Zigong Maternal and Child Health Hospital between June 2014 and February 2018 were selected as the HSP group of the study, and healthy children who received physical examination during the same period were selected as the control group of the study. Peripheral blood was collected to determine the expression of SOCS1 and SOCS3 as well as the contents of CD4+T cell subsets, and serum was collected to determine the contents of CD4+T cytokines.Results: SOCS1 and SOCS3 mRNA expression levels as well as SOCS3/SOCS1 ratio in peripheral blood of HSP group were significantly higher than those of control group;Th1 and Treg contents in peripheral blood as well as IFN-γ and TGF-β1 contents in serum of HSP group were lower than those of control group whereas Th2 and Th17 contents in peripheral blood as well as IL-4, IL-5 and IL-17 contents in serum were higher than those of control group, and Th1 and Treg contents in peripheral blood as well as IFN-γ and TGF-β1 contents in serum of HSP children with high SOCS3/SOCS1 ratio were lower than those of HSP children with low SOCS3/SOCS1 ratio whereas Th2 and Th17 contents in peripheral blood as well as IL-4, IL-5 and IL-17 contents in serum were higher than those of HSP children with low SOCS3/SOCS1 ratio.Conclusions: Changes in SOCS1 and SOCS3 expression in peripheral blood of children with Henoch-Schonlein purpura can affect the differentiation of CD4+T cells.展开更多
Objective:Analyze the relationship between inoculating one case of the COVID-19 inactivated vaccine(Vero cell)and immune thrombocytopenic purpura to provide a reference for the standardized handling of adverse events ...Objective:Analyze the relationship between inoculating one case of the COVID-19 inactivated vaccine(Vero cell)and immune thrombocytopenic purpura to provide a reference for the standardized handling of adverse events following immunization.Methods:According to the"National Monitoring Program for Suspected Adverse Reactions to Vaccinations,"an on-site investigation,data collection and analysis,expert group diagnosis,and medical association assessment were conducted on a case of immune thrombocytopenic purpura in District A of Chongqing after vaccination with the inactivated COVID-19 vaccine.The assessment report was delivered to the three relevant parties,the case was reviewed,and the experience was summarized.Results:The investigation and diagnosis by the district-level vaccination abnormal reaction expert group concluded that the disease that occurred after vaccination with the COVID-19 inactivated vaccine was secondary immune thrombocytopenic purpura,an abnormal reaction to the vaccination.The medical damage was classified as Level II Grade B.The vaccine production enterprise raised objections to this conclusion.After re-assessment by the municipal-level medical association,the conclusion was consistent with that of the district-level medical association.The vaccine production enterprise did not raise any further objections.Conclusion:Through active collaboration among district and municipal-level medical associations,disease control institutions,and vaccination units,the recipients have been promptly and effectively treated,providing financial support for their subsequent treatment and safeguarding their rights.The investigation and disposal procedures for adverse events following immunization in Chongqing are clear,and the mechanism is sound.It is necessary to continue strengthening the monitoring of adverse events following immunization according to the existing plan and to ensure timely and standardized handling.Simultaneously,it is crucial to strengthen vaccine management and vaccination 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 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 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
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Henoch-Schonlein purpura (HSP) with intestinal perforation and cerebral hemorrhage is a very rare clinical condition. There has been no report of HSP complicated with both intestinal perforation and cerebral hemorrhage until October 2012. Here we describe a case of HSP with intestinal perforation and cerebral hemorrhage in a 5-year-old girl. Plain abdominal radiograph in the erect position showed heavy gas in the right subphrenic space with an elevated diaphragm. Partial resection of the small intestine was performed, and pathological analysis suggested chronic suppurative inflammation in all layers of the ileal wall and mesentery. Seventeen days after surgery, cerebral hemorrhage developed and the patient died.
文摘Henoch-Schonlein purpura(HSP) is a small vessel vasculitis mediated by type Ⅲ hypersensitivity with deposition of Ig A immune complex in the walls of vessels. It is a multi-system disorder characterizedby palpable purpura, arthritis, glomerulonephritis and gastrointestinal manifestations and commonly occurs in children and young adults. The patients with gastrointestinal involvement usually present with colicky abdominal pain, vomiting and melena. The imaging findings include multifocal bowel thickening with mucosal hyperenhancement, presence of skip areas, mesenteric vascular engorgement, with involvement of unusual sites like stomach, duodenum and rectum. These imaging findings in a child or young adult with appropriate clinical findings could suggest HSP.
文摘Helicobacter pylori(H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extraintestinal diseases. H. pylori infection are mainly acquired during childhood, and it has been reported that in endemic areas of China the infection rates are extraordinarily higher in HSP children, particular those with abdominal manifestations. Furthermore, eradication therapy may ameliorate Henoch-Schonlein purpura(HSP) manifestations and decrease the recurrence of HSP. Therefore, results suggested that detection of H. pylori infection by appropriate method ought to be applied in HSP children. Current evidences indicate that local injury of gastric mucosa and immunological events induced by H. pylori infection are involved in the development of HSP. Increased serum Ig A, cryoglobulins, C3 levels, autoimmunity, proinflammatory substances and molecular mimicry inducing immune complex and cross-reactive antibodies caused by H. pylori infection might play their roles in the course of HSP. However, there are no investigations confirming the causality between H. pylori infection and HSP, and the pathogenesis mechanism is still unclear. More bench and clinical studies need to be executed to elaborate the complex association between H. pylori and HSP.
文摘Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.
文摘Hcnoch Schoenlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin,joints,GI tract, and kidney.It preferentially affects children but may also occur in adults.We report a 60-rear-old man with HSP who presented with colicky abdominal pain,blondy diarrhea,arthralgia,and skin rash.The gastrointestinal tract was viewed by upper endoscopy and colonoscopy.We found characterislic endoscopic findings in the stomach,cecum and sigmoid colon,the combination of which has rarely been demonstrated in one patient.Histologic examination of skin biopsy specimens revealed leukocytoclastic vasculitis with positive staining for IgA in the capillaries.Endoscopy appears to have substantial diagnostic utility in patients suspected of having HSP,especially when abdominal symptoms precede the cutaneous lesions.
文摘The aim of this study was to describe the common presentation, frequency, and complications of Henoch-Schonlein purpura (HSP) in patients <18 years who were followed up at King Abdulaziz University Hospital, Jeddah over the last 12 years. We performed a retrospective chart review of the medical records of all patients diagnosed as HSP. During this period, only 29 cases were reported (15 males, 14 females), with the mean age at the diagnosis 7.5 years. 82% percent of the patients had joint involvement in the form of arthritis or arthralgia;17.2% had no joint involvement. Abdominal manifestations were reported in 72.4% of the patients, while renal involvement was documented in 24.1% of the cases;two patients had scrotal involvement. Four patients (13.7%) had a recurrence within four months of HSP diagnosis. However, all patients had full recovery within a month. More research is warranted to study the prevalence, clinical manifestations, preceding factors, and complications of HSP in a Saudi-based cohort.
基金Supported by National Natural Science Foundation of China,No.81270470
文摘Infliximab (IFX) is an anti-tumor necrosis factorchimeric antibody that is effective for treatment ofautoimmune disorders such as Crohn's disease andulcerative colitis (UC). IFX is well tolerated with alow incidence of adverse effects such as infections,skin reactions, autoimmunity, and malignancy.Dermatological manifestations can appear as infusionreaction, vasculitis, cutaneous infections, psoriasis,eczema, and skin cancer. Here, we present anunusual case of extensive and sporadic subcutaneousecchymosis in a 69-year-old woman with severe UC,partial colectomy and cecostomy, following her initialdose of IFX. The reaction occurred during infliximabinfusion, and withdrawal of IFX led to gradual alleviationof her symptoms. We concluded that Henoch-Sch?nleinpurpura, a kind of leukocytoclastic vasculitis, mighthave contributed to the development of the bruising.Although the precise mechanisms of the vasculitis arestill controversial, such a case highlights the importanceof subcutaneous adverse effects in the management ofUC with IFX.
文摘Objective:To study the effects of methylprednisolone combined with montelukast on immune function and cytokines in children with recurrent Henoch-Schonlein purpura.Methods:Children who were diagnosed with recurrent Henoch-Schonlein purpura in Zigong Third People's Hospital between September 2015 and August 2017 were selected as the research subjects and randomly divided into the intervention group who received methylprednisolone combined with montelukast therapy and the control group who received hydrocortisone therapy. The levels of Th1/Th2 and Th17/Treg immunity indexes in peripheral blood as well as cytokines in serum were measured before treatment as well as 4 and 8 weeks after treatment.Results: 4 weeks and 8 weeks after treatment, Th1 and Treg cell contents as well as T-bet and FoxP3 mRNA expression in peripheral blood of both groups of patients were significantly higher than those before treatment whereas Th2 and Th17 cell contents as well as GATA-3 and RORγt mRNA expression in peripheral blood and NF-κB, OPN, IL-33, MK and HMGB1 contents in serum were significantly lower than those before treatment, and Th1 and Treg cell contents as well as T-bet and FoxP3 mRNA expression in peripheral blood of intervention group were significantly higher than those of control group whereas Th2 and Th17 cell contents as well as GATA-3 and RORγt mRNA expression in peripheral blood and NF-κB, OPN, IL-33, MK and HMGB1 contents in serum were significantly lower than those of control group.Conclusion: methylprednisolone combined with montelukast treatment of recurrent Henoch-Schonlein purpura can regulate the immune function and inhibit the cytokine secretion.
文摘Henoch-schonlein purpura (HSP) is a kind of systemic vasculitis that is common in childhood,and its pathogenesis is complicated and considered to have important relationship with lymphocytes, vascular endothelial cells and so on. The causes of this disease are complex and have not been clearly identified, but numerous studies have shown that inflammatory factors such as IL-1, IL-17 and TNF-α play an important role in the development of HSP.
文摘Objective:To evaluate the cytokine levels and immune response status of montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura.Methods: A total of 80 patients with Henoch-Schonlein purpura who were treated in Ankang Central Hospital between May 2014 and January 2017 were collected and divided into montelukast group, loratadine group, tanshinone group and combined treatment group according to the random number table, 20 cases in each group. Serum levels of inflammatory factors, Th17/Treg cellular immunity indexes before and after treatment were compared among four groups of patients.Results: Before treatment, differences in serum levels of inflammatory factors and Th17/Treg cellular immunity indexes were not statistically significant among four groups of patients. After treatment, serum HMGB1, IL-8, IL-14, IL-23 and IL-33 levels in combined treatment group were lower than those in montelukast group, loratadine group and tanshinone group;serum IL-17 level was lower than that in montelukast group, loratadine group and tanshinone group while IL-10 and TGF-β levels were higher than those in montelukast group, loratadine group and tanshinone group.Conclusions: Montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura helps to reduce systemic inflammatory response and balance Th17/Treg cell immunity.
文摘Objective:This study aims to explore the regularity of syndrome differentiation and treatment of traditional Chinese medicine(TCM)for allergic purpura.Methods:CNKI,Weipu Chinese science and technology database,wanfang medical network database,and Chinese biomedical literature database were searched for eligible studies.Medical records including complete patient personal information,patient symptoms,TCM syndromes,treatment,and medication were included.The data was analyzed using the Chinese medicine heritage support platform provided by the Chinese Academy of Chinese medicine(V2.5).Results:Differentiation of health gas camp blood was the most commonly used method of differentiation of symptoms and signs in famous veteran TCM.The treatment included cooling blood,activating blood circulation,clearing heat and detoxifying toxins,removing blood stasis and stopping bleeding.Honeysuckle,Forsythia suspensa,cicada slough and other drugs were interrelated.Potential drug pair combinations and drug networks showed the basic drug composition of Qingying Decoction.According to the entropy cluster analysis,28 core drug combination and 12 new formulations were obtained.Conclusion:The regularity of syndrome differentiation and treatment of traditional Chinese medicine for Henoch-Schonlein purpura based on the famous and old TCM doctors was complex.Further researches are still needed.
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.
文摘Objective:To detect serum OPN and NF-κB contents in children with Henoch-Schonlein purpura (HSP) and study their correlation with oxidative stress and cellular immune function. Methods: The children who were diagnosed with HSP in Shijiazhuang First Hospital between February 2015 and October 2017 were selected as the HSP group of the study and the healthy children who received physical examination during the same period were selected as the control group. The serum was collected to measure the contents of OPN, NF-κB, oxidative stress indexes and immune cell inflammation, and the peripheral blood was collected to detect the mRNA expression of immune cell transcription factors.Results: OPN, NF-κB, MDA, MPO, IL-4, IL-5 and IL-17 contents in serum as well as GATA-3 and RORγt mRNA expression in peripheral blood of HSP group were significantly higher than those of control group whereas SOD, CAT, PON, IFN-γ and TGF-β1 contents in serum as well as T-bet and FoxP3 mRNA expression in peripheral blood were significantly lower than those of control group;serum OPN and NF-κB contents in HSP group were positively correlated with MDA, MPO, IL-4, IL-5 and IL-17 contents in serum as well as GATA-3 and RORγt mRNA expression in peripheral blood, and negatively correlated with SOD, CAT, PON, IFN-γand TGF-β1 contents in serum as well as T-bet and FoxP3 mRNA expression in peripheral blood. Conclusion: The abnormal increase of serum OPN and and NF-κB contents in children with HSP is closely related to the excessive oxidative stress activation and cellular immune dysfunction.
文摘Objective:To study the effects of suppressor of cytokine signaling 1 (SOCS1) and SOCS3 in peripheral blood on CD4+T cell differentiation in children with Henoch-Schonlein purpura. Methods: Children with Henoch-Schonlein purpura who were treated in Zigong Maternal and Child Health Hospital between June 2014 and February 2018 were selected as the HSP group of the study, and healthy children who received physical examination during the same period were selected as the control group of the study. Peripheral blood was collected to determine the expression of SOCS1 and SOCS3 as well as the contents of CD4+T cell subsets, and serum was collected to determine the contents of CD4+T cytokines.Results: SOCS1 and SOCS3 mRNA expression levels as well as SOCS3/SOCS1 ratio in peripheral blood of HSP group were significantly higher than those of control group;Th1 and Treg contents in peripheral blood as well as IFN-γ and TGF-β1 contents in serum of HSP group were lower than those of control group whereas Th2 and Th17 contents in peripheral blood as well as IL-4, IL-5 and IL-17 contents in serum were higher than those of control group, and Th1 and Treg contents in peripheral blood as well as IFN-γ and TGF-β1 contents in serum of HSP children with high SOCS3/SOCS1 ratio were lower than those of HSP children with low SOCS3/SOCS1 ratio whereas Th2 and Th17 contents in peripheral blood as well as IL-4, IL-5 and IL-17 contents in serum were higher than those of HSP children with low SOCS3/SOCS1 ratio.Conclusions: Changes in SOCS1 and SOCS3 expression in peripheral blood of children with Henoch-Schonlein purpura can affect the differentiation of CD4+T cells.
文摘Objective:Analyze the relationship between inoculating one case of the COVID-19 inactivated vaccine(Vero cell)and immune thrombocytopenic purpura to provide a reference for the standardized handling of adverse events following immunization.Methods:According to the"National Monitoring Program for Suspected Adverse Reactions to Vaccinations,"an on-site investigation,data collection and analysis,expert group diagnosis,and medical association assessment were conducted on a case of immune thrombocytopenic purpura in District A of Chongqing after vaccination with the inactivated COVID-19 vaccine.The assessment report was delivered to the three relevant parties,the case was reviewed,and the experience was summarized.Results:The investigation and diagnosis by the district-level vaccination abnormal reaction expert group concluded that the disease that occurred after vaccination with the COVID-19 inactivated vaccine was secondary immune thrombocytopenic purpura,an abnormal reaction to the vaccination.The medical damage was classified as Level II Grade B.The vaccine production enterprise raised objections to this conclusion.After re-assessment by the municipal-level medical association,the conclusion was consistent with that of the district-level medical association.The vaccine production enterprise did not raise any further objections.Conclusion:Through active collaboration among district and municipal-level medical associations,disease control institutions,and vaccination units,the recipients have been promptly and effectively treated,providing financial support for their subsequent treatment and safeguarding their rights.The investigation and disposal procedures for adverse events following immunization in Chongqing are clear,and the mechanism is sound.It is necessary to continue strengthening the monitoring of adverse events following immunization according to the existing plan and to ensure timely and standardized handling.Simultaneously,it is crucial to strengthen vaccine management and vaccination management.
基金This study was supported by the National Natural Science Foundation of China (No. 81202839), the NationalNatural Science Foundation of China (No. 81774080), the Natural Science Foundation of Shandong Province(ZR2012HQ023),the Medical science and technology development Foundation of Shandong Province, China (No.2014WS0431), the Jinan young star of science and technology plan (No. 201406012), and the Affiliated Hospital ofShandong University of Traditional Chinese Medicine and Shandong University, China.
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.
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.
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.