Immunoglobulin A Vasculitis With Intussusception in Children.

BACKGROUND: Immunoglobulin A (IgA) vasculitis with intussusception is acute and severe vasculitis combined with acute abdomen in children. The diagnosis of the disease depends on the results of imaging examinations, and its treatment mainly includes enema and surgery. The literature summarized the detailed diagnosis and treatment data in previous literature reports. METHODS: We described the clinical manifestations, ultrasonic features, and treatment of patients admitted to a single center and reviewed previous literature regarding cases with detailed clinical data in the PubMed database within the past 20 years. RESULTS: The review included 36 patients, including 22 boys and 14 girls. A total of 32 patients were diagnosed using ultrasound (88.9%). The main sites of intussusception were the ileum and ileocolon in 16 (44.4%) and 11 (30.6%) cases, respectively. Thirteen patients (36.1%) were treated with enema, with 6 responding to the treatment. 26 patients (72.2%) underwent surgical treatment. Patients with ileal intussusception were more likely to be treated with surgery than those with colonic intussusception (P < .05). The single-center clinical data of 23 patients showed that there was no significant difference in laboratory test findings between patients with and without surgical treatment (P > .05). Patients with long insertion lengths were more likely to require surgery and resection (P < .05). CONCLUSIONS: Ultrasonography is the first-line investigation for diagnosis. The main sites of intussusception were ileum and ileocolon. The length of intubation was related to surgery; treatment is according to the intussusception site. Air enema is not suitable for intussusception of the small intestine.

Modified Sipjeondaebo-tang (JAROTANG) for Henoch-Schonlein purpura nephritis (HSPN): Two case reports.

INTRODUCTION: Henoch-Schönlein purpura (HSP) is a disease commonly manifesting purpura, joint pain, and gastrointestinal symptoms. It can lead to glomerulonephritis (Henoch-Schönlein purpura nephritis, HSPN), which is directly associated with mortality and progression to chronic kidney disease (CKD). While HSP occurs more commonly in children, deadly outcomes occur at a higher rate in adult patients. Previous studies have not reported effective treatment of HSPN by Western or traditional medicine. Here, we report two cases of adult HSPN patients treated with the herbal medicine Jarotang (JRT, modified Sipjeondaebo-tang, modified SJDBT). CASE SUMMARY: Two female patients (Cases 1 and 2), who were 26 and 27 years old, respectively, came to visit us complaining mainly of cutaneous purpura. Both women were diagnosed with HSP, and the results of urinalysis indicated that the HSP had already progressed to renal involvement (3+ proteinuria with 3+ urine occult blood in case 1; 100-120 RBC/HPF with 2+ urine occult blood in Case 2). Both patients were given modified SJDBT in the name of JRT, with some herbs added to disperse and circulate stagnant qi, relieve indigestion, and clear heat. After treatment, patient 1 showed only a trace level of urine occult blood, with disappearance of purpura and proteinuria. Patient 2 showed complete remission of purpura and hematuria. CONCLUSIONS: Modified SJDBT, namely, JRT was effective in treating 2 cases of adulthood HSP and subsequent nephritis. This may be due to the ability of this therapy to replenish qi and blood and/or its immunological effect on T cells. The medication can serve as an effective cure for HSPN.

Clinical analysis of 99 children with Henoch-Schönlein purpura complicated with overt gastrointestinal bleeding.

OBJECTIVE: To analyze and summarize the clinical features, diagnosis, and treatment of children with Henoch-Schönlein purpura (HSP) complicated by overt gastrointestinal bleeding (GI bleeding) for achieving early identification, prevention, and treatment in terms of severe GI bleeding. METHODS: A retrospective analysis was conducted on children with HSP complicated by overt GI bleeding who were admitted to the Department of Traditional Chinese Medicine of Beijing Children's Hospital from January 2017 to December 2019. According to the severity of GI bleeding, the patients were divided into mild bleeding group (61 cases) and moderate and severe bleeding group (38 cases). Inflammatory parameters, coagulation function, GI ultrasound findings, and clinical features were compared. Logistic regression analysis was used to determine the related variables affecting the severity of GI bleeding, and the ROC curve was used to determine the variable test efficacy. RESULTS: Onset in summer, wide distribution of skin rash with facial involvement, the elevation of D-dimer and high neutrophil-to-lymphocyte ratio (NLR) had significant effects on the severity of GI bleeding. ROC curve analysis showed that the optimal cut-off points of NLR and D-dimer for predicting severe GI bleeding in children with HSP were 10.56 and 0.89 mg/L, respectively. CONCLUSION: Facial rash may be a warning sign of GI bleeding. Enhanced monitoring of NLR and D-dimer is helpful for early recognition of GI bleeding as well as assessment of severity.

Traditional Chinese medicine versus regular therapy in Henoch-Schönlein purpura nephritis in children: study protocol for a randomized controlled trial.

BACKGROUND: Henoch-Schönlein purpura nephritis (HSPN) is the most common secondary glomerular disease in children. Currently, the treatment for HSPN is always selected based on the Kidney Disease Improving Global Outcomes guidelines; however, this approach may lead to undertreatment, especially in patients with persistent proteinuria that does not reach nephrotic levels and/or hematuria and those with a pathological classification between grades 1 and 3 according to the International Study of Kidney Disease in Children. This study was performed to evaluate the curative effect and safety of a traditional Chinese medicine (TCM) integrated treatment program in this type of HSPN. METHODS: This multicenter, open-label, large-sample, randomized controlled trial was performed in China and included 500 children with HSPN exhibiting mild pathological patterns. The treatment group to control group ratio was 2:1, and each group was further stratified into two types, light and heavy, according to urinary protein quantification and pathological type. The treatment group received tripterygium glycosides (TGs), tanshinone IIa sodium sulfonate injection, and Chinese herbs selected based on syndrome differentiation in TCM. The heavy and light subgroups received treatment courses and dosages of TG. In the control groups, the light group received benazepril hydrochloride tablets, low molecular weight heparin calcium injection, dipyridamole tablets, and a Chinese medicine placebo, while the heavy group received the same treatment plus prednisone. All groups were treated for 3 months and then followed up for 9 months. The efficacy and safety of the treatments were then evaluated among the groups. DISCUSSION: Currently, few treatments are available for HSPN patients with mild pathological patterns indicating light to moderate proteinuria and/or hematuresis. In this large-sample study, we provide a new approach for HSPN that includes an integrated treatment program that incorporates TCM. TRIAL REGISTRATION: Clinical Trials.gov, NCT03591471 . Re-registered on 19 July 2018.

Association between anti-α-1,4-D-polygalacturonic acid antibodies and Henoch-Schönlein purpura in children.

OBJECTIVE: To investigate the relationship between anti-α-1,4-D-polygalacturonic acid (PGA) antibodies, particularly immunoglobulin (Ig)A, and Henoch-Schönlein purpura (HSP) in children. METHODS: This observational case-control study investigated PGA-IgA, PGA-IgG, and PGA/PGA-IgA circulating immune complex (PGA/PGA-IgA CIC) in paediatric patients with HSP versus controls. Children with HSP were also evaluated for food specific IgG and food intolerance. Between-group differences in anti-PGA antibodies were analysed. RESULTS: Serum PGA-IgA and PGA-IgG levels were significantly increased in patients with acute HSP ( n = 251) versus those with urticaria ( n = 48), acute respiratory infections ( n = 95), surgical controls ( n = 53) and neonates ( n = 92). PGA/PGA-IgA CIC levels were also significantly higher in the acute HSP group versus surgical control and neonate groups. Levels of PGA/PGA-IgA CIC and PGA-IgA were significantly correlated ( r = 0.997), and PGA-IgA showed high diagnostic specificity for HSP. No statistically significant differences were observed in PGA-IgA and PGA-IgG between various degrees of food intolerance in children with HSP. CONCLUSION: Increased anti-PGA antibodies, particularly PGA-IgA and PGA/PGA-IgA CIC, were significantly associated with acute HSP in children. Food intolerance was not found to be associated with increased anti-PGA antibodies in children with HSP.

Immunoglobulin A Nephropathy and Immunoglobulin A Vasculitis.

Henoch-Schönlein purpura nephritis and immunoglobulin A nephropathy are common glomerulopathies in the pediatric population that deserve special attention. In some cases the primary care provider can follow the patient but others need more intensive management. Delaying this treatment can lead to worse morbidity. This article provides information on the pathogenesis, outcomes, and follow-up strategies that will aid in the diagnosis and referral of patients at risk for kidney disease.

Cord-derived mesenchymal stem cells therapy for liver cirrhosis in children with refractory Henoch-Schonlein purpura: A case report.

RATIONALE: To explore the curative effect of human umbilical cord-derived mesenchymal stem cell (ucMSC) therapy for patients with liver cirrhosis complicated with immune thrombocytopenia and refractory Henoch-Schonlein purpura (HSP). PATIENT CONCERNS: A 12-year-old boy presented to our hospital with an 11-month history of purpura on the skin of both lower limbs accompanied by thrombocytopenia. The patient had a history of repeated swelling and painful dorsum pedis, followed by skin redness. DIAGNOSIS: Bone marrow slides showed megakaryocyte maturation disorder. Based on the pathology and drug abuse history, he was diagnosed with nodular cirrhosis, secondary allergic purpura, and thrombocytopenia, etiologies related to his drugs and an immune dysfunction. INTERVENTIONS: ucMSC transplantation was performed, the liver damaging drugs were discontinued, and the appropriate liver immunosuppressive drugs were administered. ucMSCs were injected 8 times/wk in 2 months, with a median cell count of 5.65 × 10/L, ranging from 5.48 to 5.98 × 10/L. OUTCOMES: As the patient's skin rash resolved, his platelets gradually increased to >150 × 10/L and liver transaminase levels gradually decreased to a normal level. Ultrasonography of the abdomen indicated that the round nodules in the liver decreased in size and that the spleen thickness also decreased. LESSONS: This is a unique case of significant HSP with associated thrombocytopenia in a patient with liver cirrhosis. Long-term oral administration of excessive herbal medicine may cause liver damage. We believe that ucMSCs provide a novel approach for the treatment of liver cirrhosis.

[Clinical research on effectiveness of 2 110 patients with Henoch Schonlein purpura from 19 comprehensive hospitals in real world].

Nineteen comprehensive hospital information system (HIS) data are collected. Cleaning up the database, then analysis the patients' information. Using the basic description method and association rules to analysis the data. Analysis the data come from HIS database, we found that in 2 110 patients, main five complications are Henoch Schonlein purpura nephritis, upper respiratory tract infections, renal insufficiency, virus infection and glomerulonephritis (chronic), treatment of chemical drugs with sugar cortical hormone and anti infection drugs, such as intravenous input cephalosporin drugs. Traditional Chinese medicine with blood-activating and stasis-resolving medicine, heat clearing and detoxifying medicine. Combination scheme with blood-activating and stasis-resolving medicine joint glucocorticoid, followed by blood-activating and stasis-resolving medicine combined antiallergic drugs, heat-clearing and detoxifying medicine combined antiallergic drugs. Promoting blood circulation and removing blood stasis is an important part of traditional Chinese medicine treatment in patients with Henoch Schonlein purpura.

Henoch-Schönlein purpura in childhood: treatment and prognosis. Analysis of 425 cases over a 5-year period.

The objective of this study was to identify the most effective treatment by evaluating the different therapies used to treat mild, moderate, and severe Henoch-Schönlein purpura (HSP) patients. We performed a retrospective study of children discharged with a diagnosis of HSP. The study group consisted of 425 children divided into mild, moderate, and severe condition groups. Different therapeutic protocols of hydrocortisone sodium succinate (HCSS) therapy, methylprednisolone (MP) pulse therapy, and MP combination with tripterygium glycoside (TG) therapy were used to treat the different groups. The evaluation of curative effect was performed. After 4 weeks, all patients with no obvious recovery were treated by strengthening the different treatment intervention. The remission time of skin, joint, and gastrointestinal manifestations was evaluated, and the results of the follow-up were analyzed (remission time of proteinuria, relapse, and side effects of therapy). After 4 weeks, in the mild group, the difference of the curative effect between HCSS and MP therapy was not statistically significant. Moderate HSP patients were more likely to respond to MP therapy than HCSS therapy (P < 0.05). Severe HSP patients were more likely to respond to MP combination with TG than single MP therapy (P < 0.05). At last follow-up, they all had normal urinalysis. In the moderate HSP group, the mean duration of proteinuria was shorter in the MP pulse therapy group than in the HCSS therapy group (P < 0.05). In the mild group, the mean duration of purpura was shorter in HCSS therapy group than in the MP pulse therapy group (P < 0.05). At last follow-up, 99 patients had recurrences of purpura and/or proteinuria and 41 patients had liver functional impairment and/or hypertension. The relapse and side effects were all satisfactorily controlled, and the rates of relapse and side effects did not differ between groups with different therapies (P > 0.05). Our study has demonstrated a superior effect for HCSS therapy in patients with mild HSP disease, for MP therapy in patients with moderate disease, and for MP combined with TG therapy in patients with severe disease. MP therapy administered initially reduces the duration of urinary protein abnormality. The therapeutic protocols did not increase the risk of relapse and were safe.

[Invagination in children: not always ileocaecal]. / Invaginaties bij kinderen: niet altijd ileocoecaal.

Three patients, two girls aged 10 and a boy aged 11, suffered from secondary intussusception. Two of the cases were mistakenly managed as an idiopathic or classic intussusception. Hydrostatic reduction with a contrast enema was thought to be successful when retrograde influx in the ileum was seen. As the intussusception recurred, a diagnostic laparoscopy was performed followed by laparotomy and surgical treatment. In both cases an ileo-ileal intussusception was found. In one case the lead point was a malignant lymphoma, in the other case probably an area of vasculitis associated with Henoch Schönlein purpura. The enema had only repositioned the ileocolic part of the intussusception. In the third patient, the ileo-ileal intussusception resolved spontaneously. Due to the location of the intus-susception, a lead point was suspected and a laparoscopy was performed. A Meckel's diverticulum was found and resected. The importance of looking for a lead point is emphasized when dealing with an intussusception in children over the age of 3, or with evidence of underlying disease. In such cases, the relative value of a contrast enema for diagnosis and reposition is emphasised. There should be a low threshold for further investigation, including diagnostic laparoscopy.

The cutaneous manifestations and common mimickers of physical child abuse.

The cutaneous manifestations of physical child abuse are some of the most common and easily recognized forms of injury. To make an accurate assessment and diagnosis, it is important to differentiate between inflicted cutaneous injuries and mimickers of physical abuse. Likewise, an understanding of reporting guidelines helps guide practitioners in their decision making.

[Scanning electron microscopy of peripheral blood erythrocytes in the diagnosis and treatment of hemorrhagic vasculitis in children]. / Metod skaniruiushchei élektronnoi mikroskopii éritrotsitov perifericheskoi krovi v diagnostike i lechenii gemorragicheskogo vaskulita u detei.

Twenty-four children aged 3-15 years were examined, 16 of these with cutaneous and articulo-cutaneous hemorrhagic vasculitis (HV) and 8 normal controls. The patients were divided into 2 groups: 10 patients treated by basic therapy and 6 children whose treatment protocols were supplemented by membranoprotector locheine. The children were repeatedly examined 1 month after discharge from hospital. Scanning electron microscopy of peripheral blood erythrocytes provides valuable diagnostic data on erythrocyte membrane morphology and function in children with HV and can serve as a method for monitoring the efficiency of new approaches to therapy of this disease.

[Severe digestive manifestations of rheumatoid purpura in adults]. / Manifestations digestives sévères du purpura rhumatoide de l'adulte.

The authors report three cases of Scholein-Henoch's purpura in adults which were characterized by atypical severity of the digestive signs which led to laparotomy. In one case, the digestive signs preceded the onset of the cutaneous purpura, which made the diagnosis difficult. The digestive impact was confirmed endoscopically (petechia, ulceration, ulcerated stenosis) in two of the three patients. Damage of the small intestine predominated, as in the literature, but only one patient required resection of the intestine. These observations highlight the importance of endoscopic exploration in cases of abdominal signs combined with Scholein-Henoch's purpura in adults. They also demonstrate the difficulty of evaluating the prognosis and treatment in severe, peritoneo-occlusive forms.