Henoch-Schönlein purpura in a patient with oesophageal cancer: A case report

Rationale:Understanding the association between Henoch-Schönlein purpura (HSP) and malignancy is essential for early diagnosis and treatment of the potential lethal disease. To the best of our knowledge, there has been only one published case of HSP coexisting with oesophageal cancer. Here, we report another patient diagnosed with HSP and oesophageal squamous carcinoma simultaneously.Patient concerns:A 60-year-old Chinese male was referred to our hospital because of intermittent abdominal pain, abdominal distension, melena, lower extremities purpura. Positive laboratory values included pancytopenia, microscopic hematuria, nephrotic proteinuria, hematochezia, hypoalbuminemia, hyperlipidaemia, hypocomplementemia, and increased levels of hepatobiliary enzymes and immunoglobulin (Ig) A. Gastrocolonoscopy showed multiple erosion lesion on descending duodenum, terminal ileum, and ileal flap. Biopsy of these lesions suggested non-specific inflammation.Diagnoses:HSP (IIIb type) was diagnosed based on renal pathology examination in accordance with the International Study of Kidney Disease in Children (ISKDC) classification. Liver biopsy confirmed the diagnosis of nodular cirrhosis (Ishak 5). Gastroscopy unintentionally revealed three oesophagus lesions. Pathology study suggested intermediate differentiated squamous cell carcinoma (cTNM IB).Interventions:Before admission, he was administered intravenous Ig 10 g once daily(qd) for 10 days, methylprednisolone 40 mg qd for a week, followed by prednisolone 50 mg qd for almost 8 weeks. Endoscopic submucosal dissection (ESD) was performed to remove all lesions with negative margin after prednisolone was tapered (5 mg per week until 10 mg qd).Outcomes:Despite prednisone being tapered to 2.5 mg qd within 2 months, complete remission of HSP and esophageal malignancy was achieved after the resection of the esophagus lesions during 12 months follow-up.Lessons:We report a rare case of oesophageal squamous cell carcinoma initially presented as HSP. This case suggests the importance of evaluating adult patients with HSP for an underlying malignancy.     

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-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.     

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-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.     

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.     

Myocardial infarction in thrombotic thrombocytopenic purpura: a single-center experience and literature review

Background: Several case reports and series have described myocardial infarctions (MIs) in patients hospitalized for thrombotic thrombocytopenic purpura (TTP). The exact magnitude and outcome of this complication are unknown. Methods: Electronic medical records for patients admitted to Wake Forest University Baptist Medical Center were examined from 1996 to 2005. Those patients having a diagnosis of TTP during the hospitalization period were included in the analysis. Only patients’ initial episodes of TTP were analyzed. Baseline cardiac and TTP risk factors were documented. Outcomes analyzed included MIs, arrhythmias, development of congestive heart failure and death. Results: Eighty‐five patients diagnosed with TTP were identified with 13 (15.3%) having MIs, as defined by an elevation of cardiac enzymes. Median troponin I value was 5.9 ng/mL (range 3.7–8.8 ng/mL). Twelve patients had non‐ST segment elevation MIs and one had ST segment elevation. Two of 13 patients who had echocardiographic analysis had documented wall motion abnormalities. There was no difference between non‐MI and MI patients in cardiac risk factors, prior cardiac events, history of thromboembolic disease or heart failure. There was no in‐hospital mortality difference. Conclusion: MI is an important complication of TTP, identified in 15.3% of patients in our study. Routine cardiovascular evaluation with cardiac enzymes, electrocardiography, and telemetry is warranted in acute TTP patients. Appropriate intervention is yet to be determined.     

Possible antistenotic effect of tranilast in a patient with small bowel tuberculosis to prevent intestinal obstruction due to stenosis progression by antituberculous chemotherapy

Small intestinal tuberculosis is a rare disorder of the small intestine. We report the development of deep small bowel tuberculosis in a rheumatoid arthritis patient who was taking methotrexate. The diagnosis of small bowel tuberculosis was ascertained by typical endoscopic findings and production of interferon gamma in the peripheral blood. The patient was successfully treated with antituberculous chemotherapy combined with an antifibrotic agent, tranilast, to suppress the progression of intestinal stenosis toward symptomatic stricture.     

Vasoactive intestinal polypeptide appears to be one of the mediators in misoprostol-enhanced small intestinal transit in rats

BACKGROUND AND AIMS: Prostaglandin analogs have the pharmacologic effect of speeding up small intestinal transit (SIT). It remains unknown whether some gut peptides also mediate this enhancement. We studied the effect of misoprostol on rat SIT and looked at the role of vasoactive intestinal polypeptide (VIP) release during its action. METHODS: A group of rats initially received oral misoprostol treatment of 1, 10, 50 and 100 microg/kg, respectively. By using orally fed charcoal as a motility marker, the SIT was assessed at 30 min following oral misoprostol treatment. Another group of rats received misoprostol as an intraperitoneal injection in similar doses to the group above. The small intestinal transit was computed for this group at 30 min following misoprostol injection via an instilled radiochromium motility marker that went through a previously placed intraduodenal catheter. The plasma VIP level was measured by using a radioimmunoassay kit. RESULTS: Neither charcoal evaluated transit nor the plasma VIP level was influenced by the lower doses of oral misoprostol treatment (1 and 10 microg/kg), whereas other doses enhanced SIT and diminished the plasma VIP level (P< 0.01).The similar effects on radiochromium computed SIT (P< 0.01) and plasma VIP levels were obtained in tubed rats following misoprostol intraperitoneal treatment. The SIT results correlated negatively with plasma VIP levels. CONCLUSIONS: Enhanced SIT and diminished VIP levels are found in rats following misoprostol treatment. It appears that inhibited VIP release is one of the mechanisms in misoprostol-enhanced SIT.     

Rituximab responsive immune thrombocytopenic purpura in an adult with underlying autoimmune lymphoproliferative syndrome due to a splice-site mutation (IVS7+2 T>C) affecting the Fas gene

A 36 yr-old man of Israeli descent with a history of childhood splenectomy for severe thrombocytopenia and a family history of autoimmune lymphoproliferative syndrome (ALPS), presented with severe immune thrombocytopenic purpura refractory to standard therapy. He was found to possess a heterozygous mutation in the Fas gene (also termed TNFRSF6, CD95, Apo-1) affecting the donor splice site of intron 7 (IVS7+2 T>C). This frameshift mutation truncates the cytoplasmic domain of the Fas death receptor, resulting in circulating CD4/8 double negative T lymphocytes, lymphadenopathy and autoimmune complications typical of ALPS. Administration of Rituximab in this patient was associated with a durable hematologic response (currently more than 12 months). This report highlights the need to consider rare inherited causes of thrombocytopenia in adults with a family history of immune cytopenia(s) and the effective use of anti-CD20 monoclonal antibody in patients unresponsive to immunosuppression and splenectomy.     

Congestive heart failure due to aortic incompetence with intestinal infarction due to endarteritis obiliterans

A 27-year-old man with congestive heart failure due to aortic incompetence and subsequent intestinal infarction was found at laparotomy to have extensive necrosis of the bowel due to proliferative endarteritis. Symptoms resolved following treatment with prednisolone and cyclophosphamide, and replacement of the aortic valve. The sub-total occlusion produced by endarteritis obliterans may lead to acute end-organ infarction if cardiac output is reduced.     

Warfarin-induced haemorrhagic infarction of the small bowel

Haemorrhagic infarction of the small bowel is a rare complication of warfarin therapy. We take this opportunity to report a case that needed a resection of the small bowel.     

以小肠穿孔为表现的嗜酸粒细胞性胃肠炎1例

嗜酸粒细胞性胃肠炎属于原发性嗜酸粒细胞性胃肠道疾病,是以胃肠道组织中嗜酸粒细胞异常浸润为主要病理组织特征,伴有外周血嗜酸粒细胞增多的少见胃肠道疾病[1].可累及整个消化道,好发于胃和小肠[2].该病因临床表现多样化,缺乏特异性,故误诊率较高.本研究收治1例嗜酸粒细胞性胃肠炎患者,以反复腹痛、小肠穿孔为主要症状就诊.现报...     

Proximal small intestinal mucosal injury

The effect of chronic intragastric infusion of hypertonic mannitol on small intestinal mucosal structure and function was studied in adult rats. Animals were gavagefed 20% mannitol (1300 mosm) at a dose of 5 ml/100 g body weight daily for seven days. Control animals were gavagefed tap water on the same schedule. On day 8, the animals were anesthetized, the duodenum cannulated, and a test sugar (glucose, glucose polymer, lactose, sucrose, or maltose) was infused at a dose of 0.5 g/kg body weight in 2.5 ml distilled water over less than 1 min. Portal vein glucose was measured at 30-min intervals from 0 to 120 min. Mannitol treatment resulted in histologic and biochemical alterations (reduced lactase, sucrase, maltase) limited to the proximal small intestine compared to the control group. The absorption of glucose and glucose polymers was similar in mannitoltreated and control animals. In contrast, digestion and absorption of lactose, sucrose, and maltose was significantly diminished in mannitol-treated animals when compared to controls. No changes in permeability to polyethylene glycol 4000 or Na⁺-coupled glucose transport were observed in mannitol-treated animals compared to controls. These data suggest that when the intestinal mucosa is exposed to hyperosmolar loads that the digestive capacity for disaccharides is suppressed more than its glucose absorptive capacities. Furthermore, glucose oligomers may be more readily digested and absorbed than disaccharides, in this setting, due, in part, to the proximal injury and less pronounced proximal-distal gradient for glucoamylase than other brushborder carbohydrases.This study was funded in part by the Ralph Hochstetter Medical Research Fund in honor of Dr. Henry C. and Bertha H. Bus well, and by a grant-in-aid from USAID CDPE-5940-A-00-4019-00).