ABSTRACT
An 80-year-old woman with severe aortic stenosis presented with relapsing enterorrhagia and severe anemia. A video capsule pan-endoscopy showed multiple sites of complex mucosal angiodysplasia in the jejunum. Direct hemostatic treatment of accessible angiodysplasia was done with argon plasma coagulation, and the patient was urgently referred for trans-catheter aortic valve replacement (TAVR). At follow-up 1 month and 3 months later, she was doing well with no further episodes of bleeding. Heyde's syndrome is referred to as the association of aortic stenosis, gastrointestinal angiodysplasia, bleeding, and anemia. It is an acquired type2A von Willebrand syndrome caused by the proteolysis and loss of the largest polymers of vWF due to the high shear forces generated through the stenotic aortic valve. The qualitative and quantitative vWF defects play a central role in the angiogenesis and development of gastrointestinal angiodysplasia The vWF abnormalities are closely associated with the hemodynamic severity of the aortic valve stenosis. Valve replacement is the pivotal strategy to achieve the long-term resolution of bleeding recurrences. TAVR is a valuable option particularly in high-risk patients for whom surgical valve replacement is not feasible.
Subject(s)
Angiodysplasia/etiology , Aortic Valve Stenosis/complications , Aortic Valve/pathology , Calcinosis/complications , Gastrointestinal Hemorrhage/etiology , Jejunal Diseases/etiology , von Willebrand Disease, Type 2/etiology , Aged, 80 and over , Anemia/etiology , Angiodysplasia/diagnosis , Angiodysplasia/surgery , Aortic Valve/surgery , Aortic Valve Stenosis/surgery , Argon Plasma Coagulation , Calcinosis/surgery , Capsule Endoscopes , Female , Gastrointestinal Hemorrhage/diagnosis , Gastrointestinal Hemorrhage/surgery , Humans , Jejunal Diseases/diagnosis , Jejunal Diseases/surgery , Syndrome , Transcatheter Aortic Valve Replacement , von Willebrand FactorABSTRACT
Thalidomide is an oral immunomodulatory and anti-inflammatory drug with antitumor necrosis factor-α (TNF-α) activity. Several case reports and some clinical trials have demonstrated its efficacy in the treatment of refractory Crohn's disease (CD). We report the effect and tolerability of thalidomide in 3 patients with moderate-to-severe CD who were not responsive to anti-TNF-α therapies, and review the relevant literature. The first case is of a 28-year-old female affected by Crohn's colitis complicated by a severe fistulizing perianal disease; she was treated with infliximab, adalimumab, and certolizumab pegol, which were stopped because of intolerance. The second case is of a 39-year-old female with fistulizing ileocolitis complicated by severe arthralgias and perianal disease with loss of response to infliximab and intolerance of certolizumab pegol. The third case is of a 39-year-old male with gastric and ileocolonic CD refractory to immunosuppressors and intolerant of infliximab. All the 3 cases achieved complete clinical remission and endoscopic healing of mucosal lesions at a low dose of thalidomide (50 to 150 mg/d). In our CD patients who experienced loss of response or were unable to tolerate anti-TNF-α drugs, thalidomide was an effective and well-tolerated therapy for inducing and maintaining long-term remission.
Subject(s)
Crohn Disease/drug therapy , Immunosuppressive Agents/therapeutic use , Thalidomide/therapeutic use , Adult , Anti-Inflammatory Agents/therapeutic use , Crohn Disease/pathology , Dose-Response Relationship, Drug , Female , Humans , Immunosuppressive Agents/adverse effects , Intestinal Mucosa/drug effects , Intestinal Mucosa/pathology , Male , Remission Induction/methods , Severity of Illness Index , Thalidomide/adverse effects , Treatment Outcome , Tumor Necrosis Factor-alpha/antagonists & inhibitorsABSTRACT
BACKGROUND: Today we are observing an increasing incidence of ulcerative colitis associated with an improved survival of patients. AIM: To analyse current rates, outcomes, and costs of inpatient care for ulcerative colitis patients of central Italy. METHODS: The cohort included 644 ulcerative colitis patients, living in the Lazio region, with diagnosis made or confirmed by the staff of a single tertiary referral centre in Rome (1997-2006). Follow-up data on hospitalization rates, costs, and colectomy rates were collected from the Regional Hospital Information System. RESULTS: Overall hospitalization rates were 3 times higher than those of the region's general population, reflecting excess admissions for digestive or infectious diseases (standardized hospitalizations rates for digestive-tract: 15.9; for infectious diseases: 3.5). The overall cumulative risk for colectomy was 7.5%. On the average, hospitalizations for ulcerative colitis lasted 10 days. The mean reimbursement for a ulcerative colitis-related hospitalization was EUR 5120 (4609 for nonsurgical admissions, 8655 for surgical hospitalizations). CONCLUSION: Ulcerative colitis patients are 3 times more likely to be hospitalized than the general population. Colectomy rates in Italian ulcerative colitis patients resemble those of northern Europe, but most hospital admissions are for diagnostic procedures or medical therapy. Hospitalizations are almost twice as long as those reported in the United States although their mean cost is considerably lower.
