Calcium-sensing receptor autoantibodies are relevant markers of acquired hypoparathyroidism

We investigated the presence of autoantibodies (aAbs) directed against the parathyroid gland in 17 patients with spontaneous isolated acquired hypoparathyroidism. Fourteen patients with acquired hypoparathyroidism (AH) associated with type I or II autoimmune polyendocrinopathy syndrome were also tested in comparison with a control group of 68 subjects without AH, including patients with other autoimmune diseases and healthy blood donors. aAbs against parathyroid tissue were screened using an indirect immunofluorescence technique on primate parathyroid tissue and human parathyroid adenoma. aAbs against the calcium-sensing receptor (CaSR) were analyzed using an immunoblotting assay with the recombinant extracellular domain of the human CaSR as antigen. Seven of the 31 patients with AH were positive for CaSR aAbs. Five of the positive sera were obtained from the group with isolated AH. The two other positive sera were from patients with autoimmune polyendocrinopathy syndrome. The sensitivity of the immunoblotting technique was higher than that of both the radioimmunological test using the extracellular domain of the CaSR and the indirect immunofluorescence technique. There were no positive sera in the control group. In conclusion, using an immunoblotting assay, we demonstrate the presence of CaSR aAbs in about one third of the patients with isolated AH, pointing out the value of detecting such aAbs to assess the autoimmune origin of the disease.     

Clinical and immunological features of adult-onset generalized autoimmune gut disorder

OBJECTIVES: Autoimmune enteropathy is a rare disorder of unknown pathogenesis, characterized by protracted diarrhea, villous atrophy, and enterocyte autoantibodies. Its association with extended inflammation of the whole gastrointestinal tract is termed as generalized autoimmune gut disorder (GAGD), generally a pediatric disease of difficult management due to its association with immunodeficiency. The aim of our work is to describe the mucosal immunological basis of an adult-onset case of GAGD. METHODS: We studied an adult female with a severe inflammatory involvement of the gastrointestinal tract (stomach, small and large bowel, and liver) and antienterocyte autoantibodies. She had antibody deficiency and a predisposition to systemic autoimmunity. We analyzed, by immunohistochemistry and flow cytometry, the phenotypic and functional characteristics of her intestinal intraepithelial and lamina propria (LP) lymphocytes. RESULTS: We observed the prominent and constant presence of an unusual CD4+alphaE/beta7- Tc subset in the jejunal epithelium. Signs of the lymphocyte activation as well as the prominent lymphoid TNF-alpha production observed in the rectal mucosa support the involvement of a cell-mediated pro-inflammatory response in the pathogenesis of GAGD. CONCLUSIONS: We report the second case of an adult fulfilling all diagnostic criteria for GAGD. We propose that the activated LP CD4+ T lymphocytes, as well as those atypically located in the epithelium, may play a pathogenic role. The alphaE/beta7- IEL could constitute a diagnostic marker of intestinal autoimmunity in the cases when autoantibodies are not evidenced, and mucosal TNF-alpha might represent a novel therapeutic target in this severe disease.     

Evaluation of the therapeutic effect of albendazole on experimental and human infections by Hymenolepis nana

With the objective of knowing adequately the spectrum of activity of albendazole against intestinal helminthiases, we made observations regarding hymenolepiasis caused by Hymenolepis nana. Two series of investigations were carried out: a) treatment of mice with single doses of either 25 or 50 mg/kg, repeated after ten days, using as controls animals treated with 25 mg/kg of praziquantel or not treated with any antiparasitic drugs; b) treatment of adults and children with 400 mg daily for three consecutive days, repeated after ten days.     

Idiopathic chronic pancreatitis (with diffuse stenosis of Wirsung's duct) in ulcerative colitis

We describe a case of chronic idiopathic pancreatitis associated with ulcerative colitis. Pancreatitis is a rare extra-intestinal manifestations of inflammatory bowel disease. Chronic idiopathic pancreatitis associated with ulcerative colitis are usually painless, without calcification, with stricture of the main pancreatic duct and with severe exocrine pancreatic insufficiency.     

Galectina-3 Associada a Formas Graves e Mortalidade em Longo Prazo em Pacientes com Doença de Chagas

Background The histopathological characteristics of Chagas disease (ChD) are: presence of myocarditis, destruction of heart fibers, and myocardial fibrosis. Galectin-3 (Gal-3) is a biomarker involved in the mechanism of fibrosis and inflammation that may be useful for risk stratification of individuals with ChD.Objectives We sought to evaluate whether high Gal-3 levels are associated with severe forms of Chagas cardiomyopathy (CC) and whether they are predictive of mortality.Methods We studied anti-T. cruzi positive blood donors (BD): Non-CC-BD (187 BD without CC with normal electrocardiogram [ECG] and left ventricular ejection fraction [LVEF]); CC-Non-Dys-BD (46 BD with CC with abnormal ECG but normal LVEF); and 153 matched serum-negative controls. This cohort was composed of 97 patients with severe CC (CC-Dys). We used Kruskall-Wallis and Spearman’s correlation to test hypothesis of associations, assuming a two-tailed p<0.05 as significant.Results The Gal-3 level was 12.3 ng/mL for Non-CC-BD, 12.0 ng/mL for CC-Non-Dys-BD, 13.8 ng/mL for controls, and 15.4 ng/mL for CC-Dys. LVEF<50 was associated with higher Gal-3 levels (p=0.0001). In our linear regression adjusted model, we found association between Gal-3 levels and echocardiogram parameters in T. cruzi-seropositive subjects. In CC-Dys patients, we found a significant association of higher Gal-3 levels (≥15.3 ng/mL) and subsequent death or heart transplantation in a 5-year follow-up (Hazard ratio – HR 3.11; 95%CI 1.21–8.04; p=0.019).Conclusions In ChD patients, higher Gal-3 levels were significantly associated with severe forms of the disease and more long-term mortality, which means it may be a useful means to identify high-risk patients. (Arq Bras Cardiol. 2021; 116(2):248-256)     

The mediating role of patient satisfaction and perceived quality of healthcare in the emergency department

The purpose of this research was to identify whether a certain set of drivers of satisfaction/perceived quality of healthcare (PQHC) could indirectly affect patients’ confidence/trust in the emergency department (ED).Patients were seen at an ED in the public hospital in Lisbon, Portugal between January and December 2016. Data were collected between May and November 2017, using a questionnaire, by mail or e-mail. The total sample size comprised 382 patients. The data analysis included structural equation modeling to test the conceptual model with specific drivers of satisfaction/PQHC (privacy; accessibility and availability; doctors; meeting expectations; waiting time for triage [perception]; waiting time to be called back by the doctor following examinations and/or tests [perception]; information about possible delays in receiving treatment/waiting times) and with the main outcome (confidence/trust in the ED) using path analysis.The analysis of the coefficients revealed that all the mediated paths are statistically significant (P ≤ .05). Although, altogether, the direct paths did not prove statistically significant (P > .05), the overall satisfaction with doctors (P ≤ .01) and meeting expectations (P = .01) can still directly explain the confidence/trust in the ED without the mediating role of satisfaction and PQHC. Hence, overall satisfaction with doctors and meeting expectations can influence, both directly and indirectly, confidence/trust in the ED. All other variables can only indirectly affect confidence/trust in the ED, either through PQHC or through satisfaction.Even though there are more variables that influence confidence/trust in the ED through PQHC (1)waiting time to be called back by the doctor following examinations and/or tests [perception]; 2) privacy; 3) accessibility and availability; 4) doctors; 5) meeting expectations than through satisfaction (1)waiting time for triage [perception]; 2) information about possible delays in receiving treatment/waiting times; 3) doctors; 4) meeting expectations), we observe the strongest contribution in the mediation model through satisfaction, which reveals its dominant role over PQHC.     

Correlação entre Cardiomegalia pela Radiografia de Tórax e Diâmetro do Ventrículo Esquerdo pela Ecocardiografia em Pacientes com Doença de Chagas

BackgroundCardiomegaly on chest X-ray is an independent predictor of death in individuals with chronic Chagas cardiomyopathy (CCC). However, the correlation between increased cardiothoracic ratio (CTR) on chest X-ray and left ventricular end-diastolic diameter (LVEDD) on echocardiography is not well established in this population.ObjectivesTo assess the relationship between chest X-ray and LVEDD on echocardiography in patients with Chagas disease and its applicability to the Rassi score.MethodsRetrospective study on 63 Chagas disease outpatients who underwent chest X-ray and echocardiography. Cardiomegaly on chest X-ray was defined as a CTR>0.5. LVEDD was analyzed as a continuous variable. ROC curve was used to evaluate the ability of LVEDD in detecting cardiomegaly by chest X-ray, with a cut-off point defined by the highest sum of sensitivity and specificity.ResultsMedian age 61 years [interquartile range 48-68], 56% were women. CCC was detected in 58 patients, five patients had the indeterminate form of Chagas disease. Cardiomegaly was detected in 28 patients. The area under the ROC curve for LVEDD was 0.806 (95%CI: 0.692-0.919). The optimal cut-off for LVEDD was 60 mm (sensitivity = 64%, specificity = 89%). The use of LVEDD on echocardiography as a surrogate for CTR on chest X-ray changed the Rassi score values of 14 patients, with a reduction in the presumed risk in 10 of them.ConclusionLVEDD on echocardiography is an appropriate, highly specific parameter to distinguish between the presence and absence of cardiomegaly on chest X-ray in Chagas disease. (Arq Bras Cardiol. 2021; 116(1):68-74)