Delayed diagnosis of abdominal Henoch-Schonlein purpura in children:A case report

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.

Endoscopic findings in a patient with Henoch-Schonlein purpura

Henoch-Schonlein 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-year-old man with HSP who presented with colicky abdominal pain, bloody diarrhea, arthralgia, and skin rash. The gastrointestinal tract was viewed by upper endoscopy and colonoscopy. We found characteristic 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.

Henoch-Schonlein purpura with intestinal perforation and cerebral hemorrhage: A case report

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.

Gastrointestinal manifestations of Henoch-Schonlein purpura: A report of two cases

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.

Current views of the relationship between Helicobacter pylori and Henoch-Schonlein purpura in children

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.

Henoch-Schonlein purpura from vasculitis to intestinal perforation: A case report and literature review

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.

Angiotensin-converting Enzyme Gene Insertion/Deletion Polymorphism in Children with Henoch-Schonlein Purpua Nephritis

This study investigated the relationship between angiotensin-converting enzyme (ACE) gene insertion/deletion polymorphism and the occurrence, severity, prognosis of HSPN. The polymorphism of ACE gene in 103 HSPN cases and 100 healthy children was studied by using the polymerase chain reactions (PCR). Its relation to the clinical manifestation, pathological classification and prognosis of HSPN was analyzed accordingly. The results showed that: (1) there was a significantly higher frequency for DD genotype in HSPN children (P<0.01); (2) DD genotype was more frequently seen in HSPN children with gross hematuria and massive proteinuria (P<0.05), while DI genotype was more common in HSPN children group with renal insufficiency (P<0.05); (3) although mesangial proliferative lesion was most frequently observed in 21 biopsied HSPN children, and DD genotype frequency was still higher in children with severe pathology (Class Ⅲ Ⅳ); (4)II genotype was significantly frequent in HSPN children with complete remission in the follow-up of 32 HSPN children. It was concluded that the deletion allele of ACE gene might play a role, at least to some extent, in the occurrence, deterioration and progression in juvenile HSPN.

Spectrum of Henoch-Schonlein Purpura in Children: A Single-Center Experience from Western Provence of Saudi Arabia

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.

Effects of methylprednisolone combined with montelukast on immune function and cytokines in children with recurrent Henoch-Schonlein purpura

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.

Evaluation of the cytokine levels and immune response status of montelukast, loratadine and tanshinone combination therapy for Henoch-Schonlein purpura

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.

Overview of the pathogenesis of Henoch-Schonlein purpura in children and the research progress on related mechanism of inflammatory cytokines

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.

Regularity of syndrome differentiation and treatment of traditional Chinese medicine for Henoch-Schonlein purpura based on data mining techniques

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.

A treatment for Henoch-Schonlein purpura based on Shaoyang Channels： a hypothesis and case report

Henoch-Schonlein purpura （HSP） is an IgA-mediated vasculitis which is easy to relapse. A lot of patients have to useglucocorticoids, immunosuppressive drugs and other drugs which will bring sorts of serious side effects. Unfortunately,most of patients have to face the hurt of abdominal pain, arthritis, purpuric rash and renal involvement again after relapse.We have used Xiaochaihu decoration which is a famous formula in Shanghanlun to cure most of patients, especially whowith abdominal pain and purpuric rash. Due to the fact that this formula belongs to the Shaoyang Channels, interestingly,we find that these symptoms are located in regions close to the Shaoyang Channels. So that is the reason why Xiaochaihu decoration could work.

Serum OPN and NF-κB contents in children with Henoch-Schonlein purpura and their correlation with oxidative stress and cellular immune function

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.

Effects of SOCS1 and SOCS3 in peripheral blood on CD4+T cell differentiation in children with Henoch-Schonlein purpura

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.

中西医结合治疗过敏性紫癜156例

目的:分析过敏性紫癜(HSP)的临床特点,观察中西医结合治疗过敏性紫癜的疗效,以提高临床诊治水平。方法 :对2012年2月—2012年10月沈阳市辽宁中医药大学附属第一医院血液科门诊收治的156例HSP患者均采用中西医结合治疗,3个月为1个疗程,治疗1个疗程。结果 :临床治愈98例,显效34例,有效22例,无效2例,有效率占98.72%。结论 :中西医结合治疗过敏性紫癜效果显著。

淋巴细胞亚群检测在小儿过敏性紫癜诊治中的应用

目的探讨淋巴细胞亚群检测在小儿过敏性紫癜(HSP)诊治中的作用及意义。方法选取73例HSP患儿作为HSP组,并根据是否感染溶血性链球菌分为链球菌感染组(31例)和链球菌未感染组(35例),并设同期正常体检的30例儿童为健康对照组。分析两组患儿入院时及采用相同治疗方案2周后的临床症状和淋巴细胞亚群检测结果。结果与健康对照组比较,HSP组存在CD4+[33.572±7.408)%比(37.225±5.789)%]、CD4+/CD8+[1.270±0.218)%比(1.582±0.176)%]及CD16+CD56+[12.301±4.946)%比(15.553±4.249)%]细胞比值下降,CD8+[29.887±5.538)%比(24.500±3.589)%]、CD19+[20.603±4.713)%比(14.100±3.699)%]细胞比值上升,差异有统计学意义(P<0.05)。治疗2周后,链球菌感染组患儿淋巴细胞亚群检测数值及临床症状改善均不明显,而链球菌非感染组患儿的淋巴细胞亚群数值和临床症状明显改善,差异有统计学意义(P<0.05)。结论 HSP患儿急性期存在细胞免疫紊乱,溶血性链球菌感染会进一步加重该类患者细胞免疫功能紊乱,淋巴细胞亚群检测对小儿HSP诊治具有重要的作用与意义。

Significance of antineutrophil cytoplasmic antibody in adult patients with Henoch-Schnlein purpura presenting mainly with gastrointestinal symptoms

AIM： To test the clinical significance of antineutrophil cytoplasmic antibody （ANCA） in evaluation of adult Henoch-Schonlein purpura （HSP） patients presenting mainly with abdominal symptoms. METHODS： Twenty-eight consecutive HSP patients who presented predominantly with abdominal symptoms were enrolled in this study. Control subjects included 27 ageand sex-matched patients with peptic ulcer disease, colon cancer, acute gastroenteritis, irritable bowel syndrome and colonic polyps. ANCA was measured by indirect immunofluorescence （IIF） in all patients, and follow-up ELISA was performed in patients with positive IIF tests. RESULTS： ANCA was detected in 9 HSP patients by IIF （2 were positive for c-ANCA and 7 were positive for p-ANCA）. No ANCA was found in the control group. The sensitivity and specificity of a positive ANCA test （either c- or p-ANCA） were 32.1% and 100% respectively. Only one out of the 9 patients with positive ANCA by IIF had positive ANCA by ELISA and the antigen was myeloperoxidase （MPO）. The patients positive for ANCA had higher HSP clinical scores, and were more likely to have renal function impairment. Patients with late purpura development were also associated with more severe clinical manifestations. CONCLUSION： A positive ANCA test is associated with more severe symptoms in HSP. After inflammatory bowel disease is excluded, a positive ANCA test provides a clue to the diagnosis of HSP presenting predominantly with abdominal symptoms.

Endoscopic and radiographic features of gastrointestinal involvement in vasculitis

Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.

Childhood Henoch-Schnlein Purpura Nephritis and IgA Nephropathy: One Disease Entity?——A Clinico-pathologically Comparative Study

Summary： In order to characterize their relationship through clinicopathological comparison between IgA nephropathy and Henoch-Schoenlein purpura nephritis （HSPN）, 31 children with IgA nephrop- athy aged between 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were compared with each other in clinical manifestation, blood biochemistry, serum immunology and followup study. Renal pathological findings under light microscope, immunofluorescence and electronic microscope were analyzed and also compared between 31 children with IgA nephropathy and 32 biopsied children with HSPN. The results showed that the onset age was over 12 years in 25.8 % children with IgA nephropathy, but only 10 % in HSPN （P〈0.05）. The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were more often in HSPN, all of them had skin purpura, 59 % had gastrointestinal symptoms and 47 % suffered from arthralgia, compared with only abdominal pain in 3.2 % children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5 % of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9 % of IgA nephropathy and 6.3 % of HSPN, but endothelial proliferation in 65.6 % of HSPN and 29 % of IgA nephropathy （all P〈0.01）. Thin basement membrane nephropathy was only found in 6. 5 % children with IgA nephropathy, no in HSPN. The electronic dense deposits in HSPN were sparse, lodse and wildly spread in glomerular mesangium, subendothelial area and even intra basement membrane, but it was dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. Predominant IgA deposits were found in 81.2% of HSPN, and overwhelming IgG deposits in 12.5 % of HSPN with relatively weak IgA deposits, moreover 6.3 % of HSPN showed linear IgG deposits in glomerular capillary. Totally 71. 9 G of HSPN had IgG deposits in glomeruli and only 19.4% of IgA nephropathy showed glomerular IgG deposits （P〈0. 01）. No IgG deposit was observed in 81. 6 % of IgA nephropathy, among them most showed IgA and IgM and/or C3 deposits, moreover overwhelming IgG deposits and linear IgG deposits couldn＇t be found in IgA nephropathy. Mean 20 months follow-up showed complete remission in 72.5% of HSPN, but only 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5 % of IgA nephropathy had consistent hematuria and proteinuria and 16. 1% had active nephritides （P〈0.05）. It was concluded that significant clinico-pathological difference was found between HSPN and IgA nephropathy, which didn＇t support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.