Patients with von Willebrand disease were enrolled in our study. Type 2 VWD diagnoses were based on original test results. Repeat evaluation resulted in many patients receiving a different type 2 diagnosis. Some genetic variants were particularly likely to move type 2 subcategories. Abstract IntroductionType 2 von Willebrand disease (VWD) refers to patients with a qualitative defect in von Willebrand factor. Accurate diagnosis of type 2 VWD subtypes can be challenging. Aim of the studyTo compare the historical diagnosis of type 2 VWD with current laboratory testing. MethodsSubjects were enrolled in the Zimmerman Program either because of a preexisting diagnosis of VWD (retrospective cohort) or from evaluation for bleeding symptoms or suspected VWD (prospective cohort). Original diagnosis was assigned by the local center and central diagnosis was based on central laboratory testing. ResultsTwo hundred and seventeen index cases in the retrospective cohort and 35 subjects in the prospective cohort carried a local diagnosis of type 2 VWD (29% and 6% of enrolled index cases, respectively). In the retrospective cohort, the diagnosis was confirmed in 66% of cases with a preexisting diagnosis of 2A, 77% 2B, 54% 2M, and 72% 2N. In the prospective cohort, 31% were confirmed 2A, 60% 2B, 23% 2M, and 100% 2N. Several genetic variants were repeatedly implicated in subjects with changed diagnosis: p.M1304R, p.R1315C, p.R1374C, and p.R1374H. ConclusionsBoth the prospective and retrospective cohorts demonstrated consistent variation in subjects whose diagnosis changed between 2A, 2B, and 2M. The importance of accurately diagnosing type 2 VWD may be most significant in the 2B subtype given potential concerns with the use of desmopressin in type 2B VWD. Some genetic variants appear in multiple types of VWD, making specific diagnoses challenging.
The presentation of viral myocarditis ranges from no symptoms to devastating illness.1 This is an unfortunate case of Coxsackie B2 virus induced Fulminant Myocarditis (FM); which despite aggressive medical and mechanical circulatory support progressed to fatality. CASE PRESENTATION:A 20-year-old male patient was transferred to our hospital with respiratory failure and shock. He presented to his local hospital with dyspnea, fever, myalgias and arthralgias that started after an indoor soccer game two weeks earlier. Prior to admission he was treated with inhalers, steroids and antibiotics without improvement. The TTE showed an LVEF 30% and Chest CT Scan showed bilateral consolidations and multiple nodules surrounded by ground glass opacities. Mechanical ventilation, vasopressors and inotropes were initiated prior to transferring him to our hospital.
Pulmonary complications are common following hematopoietic stem cell transplantation (HSCT). The role of fiberoptic bronchoscopy with broncho-alveolar lavage (BAL) in recipients of reduced-intensity (RI) and full intensity (FI) transplants is now reported. Between January 2001-May 2008, 803 allogeneic transplants (212 RI, 591 FI) were performed on 784 patients at the University of Michigan Medical Center. Pulmonary complications, defined as either hypoxia > 24 hours, or clinical/radiographic features of pneumonitis, were identified in 313 (39.9%) patients post-transplant. Bronchoscopy was performed on 265 (84.7%) patients with pulmonary complications, including 69 patients undergoing RI transplants (median age 56 yrs, 10–68 yrs) and 196 patients undergoing FI transplants (median age 41 yrs, 1–64 yrs). The time to BAL was similar for recipients of RI when compared to recipients of FI transplant [median: 89 days (0–1854 days) vs. 104 days (7–1533 days)]. Pathogenic organisms were identified in 31.8% (RI) and 25.2% (FI) of BAL procedures. Invasive fungi were the most commonly identified organisms, identified on 48.1% (RI) and 45.2% (FI) of BALs in which pathogens were noted. The incidence of bacterial, viral (including CMV), and mycobacterial pulmonary infections were similar in recipients of RI and FI transplants. The frequency with which pathogens were identified varied with the timing of the bronchoscopy (see Table), with non-infectious pulmonary complications twice as likely to occur in the first 100 days post-transplant in FI than RI transplant recipients (p < 0.05). The BAL led to a change in medical management in 54.1% (RI) and 59.3% (FI) of cases respectively, with modifications in antimicrobial therapy in 38.8% (RI) and 42.1% (FI). The bronchoscopy led to modifications in immunosuppressive therapy in 27.1% (RI) and 27.6% (FI) of cases. BAL procedural related complications were rare in both groups, with pulmonary hemorrhage and transient hypoxemia occurring in < 2.5% of all patients undergoing a BAL. Conclusion: The incidence of pulmonary complications, as evaluated by bronchoscopy post HSCT, was similar for recipients of RI and FI conditioning regimen. Within the first 100 days post-transplant, non-infectious etiologies for pulmonary dysfunction were significantly more common in FI than RI patients. There were no other significant differences in the timing of pulmonary complications or the type of pathogenic organisms identified following a FI or RI transplant. Broncho-alveolar lavage is a safe procedure in patients following allogeneic transplants, altering medical management in over 50% of cases. Time post-transplant and % BAL with a pathogenic organism identified Day 0–100 * Day > 100 Total RI 32.4% 31.3% 31.8% FI 15.8% 31.8% 25.2%
Background:Bleeding is an important complication in children following tonsillectomy.Screening with coagulation tests prior to procedure is common to assess bleeding risk in the perioperative period, although ASH/ASPHO Choosing Wisely guidelines recommend against routine PT/PTT testing. Our aim was to compare von Willebrand factor antigen (VWF:Ag) and activity levels among patients with postoperative bleeding following tonsillectomy to evaluate for potential risk for bleeding.Procedure: Eligible subjects were aged 0-18 without significant personal or family history of major bleeding. Postoperative bleeding diaries were collected and symptoms measured using a postoperative bleeding score. Plasma VWF levels were drawn at time of anesthesia administration.Results: Postoperative bleeding occurred in 248 cases out of 1399 total subjects. Median VWF:Ag was 86 in patients with postoperative bleeding scores of 1-2, 86 for scores 3-4, 84 for scores 5-6, and 83 for scores >6, with no significant difference among groups (p = .98). Additionally, no difference was observed for subjects with multiple days of postoperative bleeding as compared to those with only 1 day of postoperative bleeding. Finally, no difference in VWF:Ag was observed for subjects whose first reported bleed occurred early in the postoperative course compared to those whose first reported bleed occurred later. VWF:Ag does not correlate with severity of bleeding, time of onset of first bleeding event, or recurrence of bleeding in healthy children with no personal or family history of bleeding who have postoperative bleeding following tonsillectomy.Conclusions: This data does not support routine von Willebrand disease screening prior to tonsillectomy.
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