Efficacy and safety of Xijiao Dihuang decoction in treating Henoch-Schönlein purpura: Study protocol for systematic review

Background:Henoch-Schönlein purpura is one of the most common systemic vascular inflflammatory disease in childhood with purpuric rash, arthritis, renal involvement, and abdominal pain. As a treatment for it, Xijiao Dihuang decoction, a traditional herbal formula, has been used. The object of this systematic review and meta-analysis is to assess the effificacy and safety on Xijiao Dihuang decoction in treating allergic purpura.Methods:The following electronic databases will be systematically searched up to November 7, 2019 for eligible studies: The Cochrane Library, Embase, PubMed, Web of Science, the Chinese National Knowledge Infrastructure (CNKI), the Chinese Biomedical LiteratureDatabase (CBM), the Chinese Scientifific Journal Database (VIP), andtheWanfang Database. Thetreatment group in the included studies will receive both routine western medicines and Xijiao Dihuang decoction, while the control group will receive routine western medicines. Data extraction and risk of bias assessments will be conducted by 2 independent reviewers. Heterogeneity will be assessed by I2 statistics, while reporting bias will be evaluated by funnel plots and Begg and Egger test. Sensitivity analysis and Subgroup analysis will be performed when necessary. Review Manager software (RevManV.5.3.0) and Stata will be used for all statistical analyses. Ethics approval is not required as no privacy data were involved. This systematic review and meta analysis will be published in a peer-reviewed journal.Results:This study could provide a systematically evaluated therapeutic efficacy and safety of XJDHD on patients with HSP via including RCTs that matches the needs. And we also expect to find predictors of treatment through subgroup analysis, helping patients with HSP detect as well as cope with the disease as early as possible.Conclusion:The conclusion of our study will provide the systematical review of the efficacy and safety of XJDHD on patients with HSP, and provide predictors of treatment.PROSPERO registration number:PROSPERO CRD 42018111293     

Severe Henoch-Sch?nlein purpura with infliximab for ulcerative colitis

Infliximab (IFX) is an anti-tumor necrosis factor chimeric antibody that is effective for treatment of autoimmune disorders such as Crohn’s disease and ulcerative colitis (UC). IFX is well tolerated with a low incidence of adverse effects such as infections, skin reactions, autoimmunity, and malignancy. Dermatological manifestations can appear as infusion reaction, vasculitis, cutaneous infections, psoriasis, eczema, and skin cancer. Here, we present an unusual case of extensive and sporadic subcutaneous ecchymosis in a 69-year-old woman with severe UC, partial colectomy and cecostomy, following her initial dose of IFX. The reaction occurred during infliximab infusion, and withdrawal of IFX led to gradual alleviation of her symptoms. We concluded that Henoch-Schönlein purpura, a kind of leukocytoclastic vasculitis, might have contributed to the development of the bruising. Although the precise mechanisms of the vasculitis are still controversial, such a case highlights the importance of subcutaneous adverse effects in the management of UC with IFX.     

Gastrointestinal Henoch–Schönlein purpura successfully treated with Mycophenolate Mofetil: Description of 2 case reports

Rationale:Henoch–Schönlein Purpura (HSP) is an acute small vessel vasculitis. It is the most common vasculitis in children. In majority of the cases, the disease is self-limited. Relapses can occur, in particular during the first year of the disease. There is no consensus on a specific treatment. The efficacy and safety of steroidal treatment in treating HSP is still controversial. Immunosuppressive treatment of HSP nephritis is used in patients with severe renal involvement (nephrotic range proteinuria and/or progressive renal impairment). The literature on immunosuppressive treatment of severe HSP without kidney involvement is scanty.Patients concerns:We report 2 case reports of 2 adolescents affected from Henoch–Schönlein Purpura and severe gastrointestinal involvement. Both patients presented a poor response to steroids treatment.Diagnoses:The diagnosis of HSP was made according to the diagnostic criteria published by European League against Rheumatism and Pediatric Rheumatology European Society in 2006Interventions:In consideration of the recurrence of the Henoch Schönlein Purpura and the gastrointestinal involvement, we decided to start Mycophenolate Mofetil treatment.Outcomes:In both patients all clinical manifestations resolved in few days.Lessons:In our cases of HSP with gastrointestinal involvement Mycophenolate Mofetil treatment has been very effective. This experience teaches us that immunosuppressive agents may be very useful to induce and maintain remission not only in renal involvement, but in all cases of persistent, recurrent, or complicated Henoch Schönlein Purpura in children.     

IgA vasculitis mimicking drug-induced skin reaction and infectious colitis in an elderly patient: A case report

Rationale:Immunoglobulin A vasculitis (IgAV) in adults is rare and shows worse symptoms and prognosis. In real-life clinics, IgAV in elderly patients may be difficult to diagnose because of its rarity and other common diseases to consider. This study reports a case of IgAV mimicking drug-induced skin reaction in an elderly patient.Patient concerns:A 74-year-old female without any underlying disease presented to our emergency department as she was suffering from lower abdominal pain and diarrhea for 3 weeks. The patient was diagnosed with infectious colitis, and antibiotic treatment was administered at a local clinic. At presentation, the patient had rashes on both lower legs that developed after the antibiotic treatment, which was assumed as a drug eruption. Although antibiotic treatment was continued, the patient had persistent abdominal pain, diarrhea, hematochezia, and rashes. Proteinuria was developed on urinalysis.Diagnoses:Infectious colitis, IgAV.Interventions:Sigmoidoscopy revealed easily bleeding erythematous mucosal lesions from the descending colon to the rectum. IgAV was suspected, and thus skin biopsy was performed. Histological findings of the skin biopsy showed leukocytoclastic vasculitis, which is compatible with IgAV. A nonsteroidal anti-inflammatory drug was administered for abdominal pain. The patient showed persistent proteinuria and a systemic steroid (prednisolone 50 mg [1 mg/kg]) was started.Outcomes:After administration of the nonsteroidal anti-inflammatory drug, the patient''s abdominal pain was resolved rapidly. Under systemic steroid treatment, the patient showed significant symptomatic improvements, and after 2 weeks, the skin and colonic mucosal lesions were completely resolved.Lessons:We present a case of adult-onset IgAV, which was initially diagnosed with infectious colitis and drug eruption. The history of skin reaction development after antibiotic treatment and the rarity of IgAV in elderly patients masked the diagnosis of IgAV. Despite its rarity, IgAV should be highly suspected in elderly patients with rashes, proteinuria, and signs of colitis.     

Spinal fusion with motor evoked potential monitoring using remimazolam in Alström syndrome: A case report

Rationale:Alström syndrome is a rare genetic disorder characterized by obesity, diabetes mellitus, cardiomyopathy, and liver dysfunction. Further, scoliosis, a common symptom of Alström syndrome, often requires surgical intervention for functional impairments. Motor evoked potential (MEP) monitoring and other electrophysiological tests are essential when performing surgery for functional scoliosis. However, there are few reports on how to maintain general anesthesia in Alström syndrome. Here, we describe a patient with Alström syndrome who underwent surgery for scoliosis under general anesthesia with remimazolam and MEP monitoring.Patient concerns:A 17-year-old woman (height, 140 cm, weight, 64.5 kg) diagnosed with Alström syndrome was scheduled for a posterior spinal fusion for functional scoliosis. Other associated comorbidities of Alström syndrome present were dilated cardiomyopathy, type 2 diabetes mellitus, obesity (body mass index, 32.1 kg/m²), amblyopia (light perception), and hearing impairment (speech awareness threshold 50 dBHL in each ear).Diagnoses, interventions, and outcomes:Posterior spinal fusion was planned for functional scoliosis. While investigating the dilated cardiomyopathy, transthoracic echocardiography showed global wall hypokinesis, with 45% left ventricular ejection fraction. The left ventricle was dilated, with left ventricular end-diastolic and end-systolic diameters of 55 and 42 mm, respectively. This finding along with the hypertriglyceridemia associated with Alström syndrome led us to conclude that propofol should be avoided. Thus, we induced general anesthesia using remimazolam. MEP monitoring was performed, and the patient experienced no motor impairments during the surgery.Lessons:Myocardial and hepatic dysfunction determine the prognosis of patients with Alström syndrome. Thus, anesthesia that preserves liver function should be selected in such cases. In patients with hypertriglyceridemia, propofol should be avoided, and using remimazolam, an ultrashort-acting benzodiazepine, may be appropriate. In this case, reviewing the Patient State Index with SedLine allowed us to perform MEP monitoring uneventfully, and the posterior spinal fusion was completed without any motor impairment.     

Cryoglobulinemic glomerulonephritis with underlying occult HBV infection and Waldenström macroglobulinemia: A case report

Introduction:Occult hepatitis B virus (HBV) infection, defined as negative hepatitis B surface antigen (HBsAg) but detectable HBV DNA in serum and liver tissue, has very rarely been described in cryoglobulinemia (CG) patients. This case report sheds light on the possible link between occult HBV infection and CG.Patient concerns:A 76-year-old man presented with rapidly deteriorating renal function within 1 year.Diagnosis:Cryoglobulinemic glomerulonephritis was diagnosed through renal biopsy. Initially, the patient tested negative for HBsAg, but a low HBV viral load was later discovered, indicating an occult HBV infection. Further studies also revealed Waldenström macroglobulinemia (WM).Interventions:We treated the patient as WM using plasma exchange and rituximab-based immunosuppressive therapy.Outcomes:After 1 cycle of immunosuppressive treatment, there was no improvement of renal function. Shortly after, treatment was discontinued due to an episode of life-threatening pneumonia. Hemodialysis was ultimately required.Conclusion:Future studies are needed to explore the link between occult HBV infection and CG, to investigate the mediating role of lymphomagenesis, and to examine the effectiveness of anti-HBV drugs in treating the group of CG patients with occult HBV infection. We encourage clinicians to incorporate HBV viral load testing into the evaluation panel for CG patients especially in HBV-endemic areas, and to test HBV viral load for essential CG patients in whom CG cannot be attributed to any primary disease.     

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

Henoch-Schönlein purpura (HSP) is generally a self-limited 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 contrast-enhanced 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.     

Severe Henoch-Schönlein Purpura with Intestinal Hemorrhagic Ulcers: Treated by Steroid Pulse Therapy

Abstract: Henoch-Schönlein purpura, associated with severe duodenal hemorrhage and gastrointestinal dysfunction, was dramatically suppressed by steroid pulse therapy. A 22-year-old male was admitted with abdominal pain, purpuric rash, and neutrophilic leukocytosis. C-reactive protein was increased and coagulation Factor XIII was markedly decreased. Upper gastrointestinal endoscopy disclosed multiple ulcers and erythema with petechiae in the postbulbar duodenum. Based on laboratory findings including examination of a skin biopsy specimen, this patient was diagnosed as having Henoch-Schönlein purpura. Despite aggressive administration of prednisolone, intravenous hyperalimentation, and Factor XIII concentrate, symptoms worsened. Steroid pulse therapy was then given for three days, resulting in amelioration of all clinical findings. The duodenal ulcers showed scarring on endoscopy. Gastrointestinal complications of Henoch-Schönlein purpura. Factor XIII concentrate therapy, and pulse steroid therapy are discussed herein.     

Intrathecal drug delivery to treat intractable neuropathic pain following Sjögren's syndrome-induced transverse myelitis: A case report

Rationale:Transverse myelitis (TM) is a spinal cord inflammatory myelopathy that causes motor/sensory loss and urinary retention below the level of the affected spinal cord. Although a few case reports have described the control of neuropathic pain in patients with TM via spinal cord stimulation, no documented case regarding the control of severe allodynia following TM via intrathecal pump has been described.Patient concerns:A 37-year-old woman was referred to a pain clinic for severe intractable pain below the T5 level followed by Sjögren''s syndrome-induced TM.Diagnoses:A neurological examination revealed paresthesia and allodynia below the T5 level. The sensory evaluation was limited by extreme pain and jerking movements. The muscle strength of both lower limbs was grade 3.Interventions:Intrathecal pump was inserted into the left lower abdomen. Catheter tip was placed at the midline of the T8 level.Outcomes:The numeric rating scale (NRS) for pain score decreased from 10 to 5. Functional Independence Measure score increased from 67 before implantation to 92 at the time of discharge, while the patient''s Barthel score increased from 31 to 46.Lessons:Neuropathic pain due to Sjögren''s syndrome-related TM could be controlled effectively using the intrathecal morphine pump.     

Novel Mutations of the ALMS1 Gene in Patients with Alström Syndrome

Objective Alström syndrome is an autosomal recessive genetic disease caused by a mutation in the ALMS1 gene. Alström syndrome is clinically characterized by multisystem involvement, including sensorineural deafness, cone-rod dystrophy, nystagmus, obesity, insulin resistance, type 2 diabetes and hypogonadism. The diagnosis is thus challenging for patients without this characteristic set of clinical symptoms. We explored the effectiveness of whole-exome sequencing in the diagnosis of Alström syndrome. Methods A girl with symptoms of Alström syndrome was tested and diagnosed with the disease by whole-exome sequencing. Results Whole-exome sequencing revealed two novel variants, c.6160_6161insAT: p.Lys2054Asnfs*21(exon 8) and c.10823_10824 delAG:p.Glu 3608Alafs*9 (exon16) in the ALMS1 gene, leading to premature termination codons and the domain of ALMS1 protein. Blood sample testing of her asymptomatic parents revealed them to be heterozygous carriers of the same mutations. Assembly showed that the mutations on both alleles were located in conserved sequences. A review of the ALMS1 gene nonsense mutation status was performed. Conclusion We herein report two novel variants of the ALMS1 gene discovered in a Chinese Alström syndrome patient that expand the mutational spectrum of ALMS1 and provided new insight into the molecular mechanism underlying Alström syndrome. Our findings add to the current knowledge concerning the diagnosis and treatment of Alström syndrome.     

Impacto da Capacidade Funcional Pré-operatória nos Resultados Pós-Operatórios de Cirurgia de Cardiopatia Congênita: Estudo Observacional e Prospectivo

BackgroundDespite advances in surgical technique and postoperative care in congenital heart disease, cardiovascular morbidity is still high.ObjectiveTo evaluate the association between preoperative cardiovascular fitness of children and adolescents, measured by the 6-minute walk test (6MWT) and Heart Rate Variability (HRV), and the occurrence of cardiogenic, septic shock and death in the postoperative period.MethodsProspective, observational clinic study including 81 patients aged from 8 to 18 years. In the preoperative period, the 6MWT (distance walked and SpO₂) and HRV were performed. The adjusted risk score for surgeries for congenital heart disease (RACHS-1) was applied to predict the surgical risk factor for mortality. The occurrence of at least one of the listed complications was considered as a combined event. P values < 0.05 were considered as significant.ResultsOf the patients, 59% were male, with mean age of 12 years; 33% were cyanotic; and 72% had undergone previous cardiac surgery. Cardiogenic shock was the most common complication, and 31% had a combined event. Prior to surgery, type of current heart disease, RACHS-1, SpO₂at rest, during the 6MWT and recovery were selected for the multivariate analysis. The SpO₂at recovery by the 6MWT remained as an independent risk factor (OR 0.93, 95%CI [0.88 - 0.99], p=0.02) for the increasing occurrence of combined events.ConclusionSpO₂after the application of the 6MWT in the preoperative period was an independent predictor of prognosis in children and adolescents undergoing surgical correction; the walked distance and the HRV did not present this association.     

Nodular Pulmonary Amyloidosis Associated with Sjögren's Syndrome

Amyloidosis is a rare disease characterized by the deposition of abnormal proteins in extracellular tissues. We herein report a case with instructive radiologic features of nodular pulmonary amyloidosis associated with Sjögren''s syndrome. A 67-year-old woman was referred to our department because of an abnormal chest radiograph. Chest computed tomography revealed multiple round cysts accompanied by calcified nodules. The patient was clinically diagnosed with primary Sjögren''s syndrome and pathologically diagnosed with nodular pulmonary amyloidosis (light chain, kappa). Although multiple lung cysts have many etiologies, the presence of calcified nodules associated with multiple lung cysts is useful for narrowing down the differential diagnosis.     

Testes Ergométricos em Pacientes com Anemia Falciforme: Segurança,Viabilidade e Possíveis Implicações no Prognóstico

BackgroundPatients with sickle cell disease (SCD) are at increased risk for cardiovascular complications. Exercise testing is used as a prognostic marker in a variety of cardiovascular diseases. However, there is a lack of evidence on exercise in SCD patients, particularly regarding its safety, feasibility, and possible prognostic role.ObjectivesWe used the maximal treadmill test to determine safety and feasibility of the exercise testing in SCD patients. Additionally, the factors associated with exercise duration, as well as the impact of exercise-induced changes on clinical outcome, were also assessed.MethodsOne-hundred thirteen patients with SCD, who underwent exercise testing, were prospectively enrolled. A comprehensive cardiovascular evaluation, including echocardiography and B-type natriuretic peptide (BNP) levels, were obtained. The long-term outcome was a composite endpoint of death, severe acute painful episodes, acute chest syndrome, or hospitalization for other SCD-related complications. Cox regression analysis was performed to identify the variables associated with the outcome. A p-value<0.05 was considered to be statistically significant.ResultsThe mean age was 36 ± 12 years (range, 18-65 years), and 62 patients were women (52%). Ischemic electrocardiogram and abnormal blood pressure (BP) response to exercise were detected in 17% and 9%, respectively. Two patients experienced pain crises within 48 hours that required hospitalization. Factors associated with exercise duration were age, sex, tricuspid regurgitation (TR) maximal velocity, and E/e’ ratio, after adjustment for markers of disease severity. During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%). Independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and BP response to exercise.ConclusionsExercise testing in SCD patients who were clinically stable is feasible. Exercise duration was associated with diastolic function and pulmonary artery pressure. Abnormal BP response was an independent predictor of adverse events.