Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center.

The objective of this study was to describe the clinical and immunological characteristics of maternal autoimmune-mediated fetal congenital heart block (CHB) in a cohort of pregnant women from an autoimmune disease pregnancy clinic. This is a retrospective observational study of all women presenting with CHB in our autoimmune disease pregnancy clinic from January 1997 to December 2014. In addition, perinatal outcome is also described. Fourteen patients accounting for 18 fetuses with CHB were identified. The median age was 32.5 years (range, 22-40). Seven (50 %) patients had Sjögren's syndrome, and the remaining seven were asymptomatic carriers of autoantibodies. All patients had anti-Ro/SSA antibodies, and 11/13 (85 %) had anti-La/SSB antibodies. The median gestational age at the time of CHB was 22 weeks (range 18-28). Complete third degree CHB was detected in 12 (67 %). Seven cases of CHB were treated with dexamethasone, two with ritodrine, and one with the association of dexamethasone, ritodrine, and terbutaline. In 9 (50 %) cases that presented with, or developed, very poor prognosis factors, such as a ventricular rate below 50-55 bpm and/or the presence of fetal hydrops, parents opted for the termination of pregnancy, after dedicated counseling. Finally, there were nine newborns (seven males [78 %]) with median age at delivery of 37 weeks (range, 32-39). A definitive epicardial pacemaker was placed in six newborns, four of them within 2 weeks of life. CHB is a severe complication related to maternal anti-Ro/SSA and anti-La/SSB antibodies. Our results confirm previous data showing that therapy is ineffective, and most of the surviving patients will require neonatal pacemaker.

Evaluation of unexplained peripheral lymphadenopathy and suspected malignancy using a distinct quick diagnostic delivery model: prospective study of 372 patients.

Although rapid diagnostic testing is essential in suspicious peripheral lymphadenopathy, delays in accessing them can be considerable. We investigated the usefulness of an internist-led outpatient quick diagnosis unit (QDU) in assessing patients with unexplained peripheral lymphadenopathy, focusing on the characteristics, diagnostic, and treatment waiting times of those with malignancy. Patients aged ≥ 18 years, consecutively referred from 12 primary health care centers (PHCs) or the emergency department (ED) for unexplained peripheral lymphadenopathy, were prospectively evaluated during 7 years. Diagnostic investigations were done using a predefined study protocol. Three experienced cytopathologists performed a fine-needle aspiration cytology (FNAC) systematic approach of clinically suspicious lymphadenopathy with cytomorphology and immunophenotyping analyses. We evaluated 372 patients with a mean age (SD) of 45.3 (13.8) years; 56% were women. Malignancy was diagnosed in 120 (32%) patients, including 81 lymphomas and 39 metastatic tumors. Metastatic lymphadenopathy was diagnosed by FNAC in all 39 patients and the primary tumor site was identified in 82% of them when cytomorphology and immunocytochemistry were combined. A correct diagnosis of lymphoma was reached by FNAC in 73% of patients. When accepting "suspicious of" as correct diagnosis, the FNAC diagnosis rate of lymphoma increased to 94%. Among patients with malignancy, FNAC yielded 1.3% of false negatives and no false positives. All patients with an FNAC report of correct or suspicious lymphoma underwent a surgical biopsy, as it is a mandatory requirement of the hematology department. Mean times from first QDU visit to FNAC diagnosis of malignancy were 5.4 days in metastatic lymphadenopathy and 7.5 days in lymphoma. Mean times from receiving the initial referral report to first treatment were 29.2 days in metastatic lymphadenopathy and 40 days in lymphoma. In conclusion, a distinct internal medicine QDU allows an expeditious, agile, and prearranged system to diagnose malignant peripheral lymphadenopathy. Because of the close collaboration with the cytopathology unit and the FNAC methodical approach, diagnostic and treatment waiting times of patients with malignancy fulfilled national and international time frame standards. This particular diagnostic delivery unit could help overcome the difficulties facing PHC, ED, and other physicians when trying to provide rapid access to investigations to patients with troublesome lymphadenopathy.

Dermatomyositis presenting with severe subcutaneous edema: five additional cases and review of the literature.

OBJECTIVES: Dermatomyositis (DM) constitutes a subset of idiopathic inflammatory myopathies clinically characterized by proximal muscle weakness and skin involvement. Some of the dermatologic manifestations are highly prevalent and characteristic, but others such as generalized or limb edema are truly rare. The aim of the present study was to describe five cases of edematous DM diagnosed at our institution and to perform a review of the literature, as well as identify clinical, laboratory, or pathological data associated with this manifestation. METHODS: We performed a retrospective clinical, laboratory, and pathological evaluation of five cases of this edematous presentation out of 86 DM cases diagnosed at our hospital from 2004 to 2013. Moreover, we undertook a medical literature search using inflammatory myopathy, dermatomyositis, and edema as key words, limited to articles published in MEDLINE, EMBASE, and LILACS database in English and Spanish from 1987 to 2013. RESULTS: A total of 19 patients were identified, five diagnosed at our hospital and 14 cases from the literature. Overall, the median time from disease onset to diagnosis was 2 months, and most of the patients (16/84 patients, 21%) required more aggressive therapy, including immunosuppressive agents and intravenous immunoglobulin (12/63 patients, 15%). Microinfarction was present 2.3 times more frequently in DM patients with edema compared with those without edema. CONCLUSIONS: The presence of edema in DM is uncommon but seems to be a sign of severe disease, requiring early and aggressive treatment. Microischemia-producing microinfarction may play an important pathophysiological role and determine the degree of disease severity.

Financial, nonfinancial and editors' conflicts of interest in high-impact biomedical journals.

PURPOSE: To assess financial, nonfinancial and editors' conflicts of interest (COI) disclosure policies among the most influential biomedical journals publishing original research. MATERIALS AND METHODS: We conducted a cross-sectional study of 399 high-impact biomedical journals in 27 biomedical categories of the Journal Citation Reports (JCR) in December 2011. Information relevant to COI and requirements for disclosures that was publicly available on journal websites was collected. RESULTS: While financial COI disclosures were required by 358 (89.7%) and nonfinancial by 280 (70.2%) journals, 155 (38.8%) required editors' disclosures. Journals in the first decile of the JCR classification scored significantly higher than those in the second decile for all disclosure policies. Ninety (22.6%) journals were published by Elsevier and 59 (14.8%) by Wiley-Blackwell, with Elsevier scoring significantly better in financial disclosure policies (P = 0.022). Clinical journals scored significantly higher than basic journals for all disclosure policies. No differences were observed between open-access (n = 25) and nonopen-access (n = 374) journals for any type of disclosure. Somewhat incoherently, authors' disclosure statements were included in some published manuscript in 57.1% of journals without any COI disclosure policies. CONCLUSIONS: Authors' financial COI disclosures were required by about 90% of high-impact clinical and basic journals publishing original research. Unlike recent studies showing a significantly lower prevalence of nonfinancial compared with financial disclosures, the former were required by about 70% of journals, suggesting that editors are increasingly concerned about nonfinancial competing interests. Only 40% of journals required disclosure of editors' COI, in conflict with the recommendations of the most influential editors' associations.

Misconduct policies in high-impact biomedical journals.

BACKGROUND: It is not clear which research misconduct policies are adopted by biomedical journals. This study assessed the prevalence and content policies of the most influential biomedical journals on misconduct and procedures for handling and responding to allegations of misconduct. METHODS: We conducted a cross-sectional study of misconduct policies of 399 high-impact biomedical journals in 27 biomedical categories of the Journal Citation Reports in December 2011. Journal websites were reviewed for information relevant to misconduct policies. RESULTS: Of 399 journals, 140 (35.1%) provided explicit definitions of misconduct. Falsification was explicitly mentioned by 113 (28.3%) journals, fabrication by 104 (26.1%), plagiarism by 224 (56.1%), duplication by 242 (60.7%) and image manipulation by 154 (38.6%). Procedures for responding to misconduct were described in 179 (44.9%) websites, including retraction, (30.8%) and expression of concern (16.3%). Plagiarism-checking services were used by 112 (28.1%) journals. The prevalences of all types of misconduct policies were higher in journals that endorsed any policy from editors' associations, Office of Research Integrity or professional societies compared to those that did not state adherence to these policy-producing bodies. Elsevier and Wiley-Blackwell had the most journals included (22.6% and 14.8%, respectively), with Wiley journals having greater a prevalence of misconduct definition and policies on falsification, fabrication and expression of concern and Elsevier of plagiarism-checking services. CONCLUSIONS: Only a third of top-ranking peer-reviewed journals had publicly-available definitions of misconduct and less than a half described procedures for handling allegations of misconduct. As endorsement of international policies from policy-producing bodies was positively associated with implementation of policies and procedures, journals and their publishers should standardize their policies globally in order to increase public trust in the integrity of the published record in biomedicine.