Amyloid Cardiomyopathy: Establishing a Patient Referral Center in Brazil.
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ABSTRACT:
SUBMITTER: Szor RS
PROVIDER: S-EPMC8543090 | biostudies-literature | 2021 Oct
REPOSITORIES: biostudies-literature
Publications
Amyloid Cardiomyopathy: Establishing a Patient Referral Center in Brazil.
Szor Roberta Shcolnik RS Alencar Aristoteles A Bueno Bruno Vaz Kerges BVK Cafezeiro Caio Rebouças Fonseca CRF Alves Lucas Bassolli de Oliveira LBO Hajjar Ludhmila Abrahão LA Martinez Gracia Aparecida GA Rocha Vanderson V Fernandes Fabio F
JACC. CardioOncology 20211019 4
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| S-EPMC4641998 | biostudies-literature
Project description:PurposeCOVID-19 is associated with high morbidity and mortality in patients undergoing surgery. Contrary to elective procedures, emergency operations should not be postponed. We aim to evaluate the profile and outcomes of COVID-19 patients who underwent emergency abdominal surgery.MethodsWe performed a retrospective analysis of perioperative data of COVID-19 patients undergoing emergency surgery from April 2020 to August 2020.ResultsEighty-two patients were evaluated due to abdominal complaints, yielding 22 emergency surgeries. The mean APACHE II and SAPS were 18.7 and 68, respectively. Six patients had a PaO2/FiO2 lower than 200 and more than 50% of parenchymal compromise on chest tomography. The most common indications for emergency surgery were hernias (6; 27.2%). The median length of stay was 30 days, and only two patients required reoperation. Postoperatively, 10 (43.3%) patients needed mechanical ventilation for a mean of 6 days. The overall mortality rate was 31.8%.ConclusionBoth postoperative morbidity and mortality are high in COVID-19 patients with respiratory compromise and abdominal emergencies.
| S-EPMC7903871 | biostudies-literature
Project description:AimsTransthyretin cardiac amyloidosis (ATTR-CM) is a progressive and fatal cardiomyopathy. Treatment options in patients with advanced ATTR-CM are limited to cardiac transplantation (CT). Despite case series demonstrating comparable outcomes with CT between patients with ATTR-CM and non-amyloid cardiomyopathies, ATTR-CM is considered to be a contraindication to CT in some centers, partly due to a perceived risk of amyloid recurrence in the allograft. We report long-term outcomes of CT in ATTR-CM at two tertiary centers.Materials and methods and resultsWe retrospectively evaluated ATTR-CM patients across two tertiary centers who underwent transplantation between 1990 and 2020. Pre-transplantation characteristics were determined and outcomes were compared with a cohort of non-transplanted ATTR-CM patients. Fourteen (12 male, 2 female) patients with ATTR-CM underwent CT including 11 with wild-type ATTR-CM and 3 with variant ATTR-CM (ATTRv). Median age at CT was 62 years and median follow up post-CT was 66 months. One, three, and five-year survival was 100, 92, and 90%, respectively and the longest surviving patient was Censored > 19 years post CT. No patients had recurrence of amyloid in the cardiac allograft. Four patients died, including one with ATTRv-CM from complications of leptomeningeal amyloidosis. Survival among the cohort of patients who underwent CT was significantly prolonged compared to UK patients with ATTR-CM generally (p < 0.001) including those diagnosed under age 65 years (p = 0.008) or with early stage cardiomyopathy (p < 0.001).ConclusionCT is well-tolerated, restores functional capacity and improves prognosis in ATTR-CM. The risk of amyloid recurrence in the cardiac allograft appears to be low.
| S-EPMC9894650 | biostudies-literature
Project description:IntroductionEpilepsy affects about 50 million people worldwide, 80% of whom live in low- and middle-income countries. In Brazil, epidemiological studies are outdated and restricted to specific regions, mostly due to the continental size of country.ObjectiveWe aimed to present the first evidence-based study on the epidemiological aspects of individuals with epilepsy, mapping the characteristics of this disease in a referral center in a region of Southeast Brazil.MethodsA retrospective study was carried out from January 2010 to March 2021. Patients were selected according to the International League Against Epilepsy Criteria.ResultsFrom a total of 618 selected patients, 317 (51.3%) were men and 301 (48.7%) were women with an average age of 34.03 ± 20.66 years. The average age at the first seizure was 15.16 ± 17.61 years. The prevalence ratio was 1.30 cases/1,000 habitants. Childhood febrile seizure was present in 44 patients (7.9%) and family history of epilepsy in 231 (37.4%) patients. The predominant type of seizure was focal in 401 (64.9%) patients. The most frequent etiologies were structural in 254 (41.1%) patients and unknown in 238 (38.5%) patients. Most of the patients' treatments were based on anti-seizure drugs in monotherapy [389 (62.9%)] with 398 (64.4%) drug-responsive patients.ConclusionsOur epilepsy prevalence rate was lower than other studies in the Southeast Region of Brazil. In addition, the structural epilepsy type was predominant in our study compared with unknown causes, which is more frequent in other Brazilian regions and worldwide studies. The differences may be attributed to our region, which presents a high prevalence of neuroinfection, specially neurocysticercosis, and a referral center for traumatic brain injury. Moreover, the contrasting results reinforce the need for an adequate epidemiological assessment of epilepsy incidence in a region of Southeast Brazil.
| S-EPMC9128524 | biostudies-literature
Project description:BackgroundThe NCCN Guidelines for Survivorship recommend dedicated sleep assessment. Reported insomnia prevalence in the general Irish population is 6% to 15%. Reported insomnia prevalence internationally among new/recently diagnosed patients with cancer varies from 30.9% to 54.3%. Insomnia prevalence has not been previously quantified in an Irish oncology cohort.MethodsA 40-item questionnaire was prospectively administered to ambulatory patients with cancer aged ≥18 years. Prespecified criteria to define insomnia syndrome combined those of the International Classification of Sleep Disorders, version 1, and the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV). The Hospital Anxiety and Depression Scale-Depression/Anxiety (HADS-D/A) was used to screen for potential confounding variables.ResultsThe response rate to the questionnaire was 87% (294/337). The predominant respondent age group was 55 to 64 years (26%; 77/294), 70.7% were female (208/294), and the most common cancer subtypes were breast (37.4%), colorectal (12.9%), and lung (12.2%). A total of 62% (183/294) of patients reported sleep disturbance after diagnosis, 63% (115/183) reported moderate/severe distress related to this disturbance, and 37% (61/183) reported a significant impact on physical function. Although 33% (98/294) met insomnia syndrome criteria, only 34% (33/98) of these patients had a preexisting history of sleep disturbance. Female sex, age <65 years, cancer subtype, alcohol consumption, and HADS-D/A ≥11 were associated with statistically significant higher odds ratios (OR) of insomnia syndrome. Multivariate analysis identified breast cancer (OR, 3.17; P=.01), age <65 years (OR, 1.8; P=.03), and alcohol consumption (OR, 2.3; P=.005) as independent predictors of insomnia syndrome.ConclusionsInsomnia syndrome prevalence in this cohort is comparable to that reported previously and supports dedicated sleep assessment. This study identifies potentially modifiable risk factors for insomnia and demonstrates additional utility of the HADS score in identifying patients at risk.
| S-EPMC9337980 | biostudies-literature
Project description:BackgroundHereditary transthyretin amyloid cardiomyopathy (ATTR-CM) is a genotypically heterogeneous disorder with a poor prognosis. There is limited literature describing the variants responsible for ATTRv in areas outside the United State, the United Kingdom and Europe. This study was performed to describe the clinical characteristics and genotypic profiles of this disease in South China.MethodsThis was a single-center retrospective study that evaluated 29 patients with a confirmed diagnosis of hereditary transthyretin amyloid cardiomyopathy enrolled from January 2016 to November 2021.Results93.1% patients were male and the median age of symptom onset was 53 (46, 62.5) years old. The initial manifestations of ATTR-CM were cardiovascular symptoms (55.2%), neuropathy (41.4%) and vitreous opacity (3.4%). Phenotypes at diagnosis were mixed (82.8%), predominant cardiac (6.9%), neurological (6.9%) and ophthalmic (3.4%). Poor R-wave progression (41%), pseudo-infarct (31%) and low-voltage (31%) patterns were common findings on electrocardiogram. Unexplained increased wall thickness was observed in all 29 patients, with mean septal and posterior wall thicknesses of 14.25 ± 6.26 mm and 15.34 ± 2.84 mm, respectively. Diastolic dysfunction was also seen in all 29 patients, and 17 (58%) had a restrictive fill pattern at diagnosis. Nine different missense mutations of the TTR gene were found in 29 patients from 23 families, with c.349G>T (p.Ala117Ser) the most common mutation. The median survival time after diagnosis was 47.6 (95% CI 37.9-57.4) months, with 1, 3 and 5-year survival rates of 91.2%, 74% and 38% respectively. Patients with advanced heart failure (National Amyloidosis Staging stage II/III) had worse survival than stage I [Breslow (Generalized Wilcoxon), χ2 = 4.693, P = 0.03)].ConclusionsATTR amyloidosis genotypes and phenotypes are highly heterogeneous. Advanced heart failure predicts a poor prognosis. Understanding the different clinical profiles of ATTR cardiac amyloidosis with different genotype is important to its early recognition.
| S-EPMC9271707 | biostudies-literature
Project description:Background: Tafamidis is a costly therapy that improves outcomes for patients with transthyretin amyloidosis cardiomyopathy (ATTR-CM), although significant knowledge gaps exist for predicting longer-term response to treatment. The purpose of this study was to examine baseline predictors of adverse outcomes and their association with tafamidis treatment in comparison with those untreated in a clinical cohort from a Canadian ATTR-CM referral center. Methods: Patients with a confirmed diagnosis of ATTR-CM were included. Multivariable modeling was used to identify baseline variables associated with the primary outcome of all-cause mortality and secondary outcomes of cardiovascular mortality or hospitalization. Cox proportional hazard and competing risk analyses were used, with tafamidis modeled as a time-varying covariate. Results: In total, 139 ATTR-CM patients were included, with a median age of 80.9 years [74.3-86.6 years], from 2011 to 2022. The mean follow-up was 2.9 ± 1.8 years. Eighty (55%) patients were treated with tafamidis. All-cause mortality and cardiovascular mortality alone were associated with the following baseline variables: age, clinical frailty scale, systolic blood pressure, renal function, and right ventricular size and function (all p < 0.05), with no identified interactions with tafamidis treatment. Only baseline renal function was associated with cardiovascular hospitalization (p < 0.05). Conclusion: Important baseline variables associated with adverse ATTR-CM disease outcomes included renal function, systolic blood pressure, frailty, and right ventricular size and function. The risk factors were independent of treatment with tafamidis. These findings may help improve risk stratification for determining eligibility for ATTR-CM therapies.
| S-EPMC11432679 | biostudies-literature
Project description:BackgroundTo investigate the prevalence and patterns of breastfeeding at discharge and in the first six months of life in a high-risk fetal, neonatal and child referral center.MethodsProspective, longitudinal study that included the following three steps: hospital admission, first visit after hospital discharge and monthly telephone interview until the sixth month of life. The total number of losses was 75 mothers (7.5%). Exposure variables were sorted into four groups: factors related to the newborn, the mother, the health service and breastfeeding. The dependent variable is breastfeeding as per categories established by the WHO. All 1200 children born or transferred to the high-risk fetal, neonatal and child referral center, within a seven-day postpartum period, from March 2017 to April 2018, were considered eligible for the study, and only 1003 were included. The follow-up period ended in October 2018. For this paper, we performed an exploratory analysis at hospital discharge in three stages, as follows: (i) frequencies of baseline characteristics, stratified by risk for newborn; (ii) a multiple correspondence analysis (MCA); and (iii) clusters for variables related to hospital practice and exclusive breastfeeding (EBF).ResultsThe prevalence of EBF at hospital discharge was 65.2% (62.1-68.2) and 20.6% (16.5-25.0) in the six months of life. Out of all at-risk newborns, 45.7% were in EBF at discharge. The total inertia corresponding to the two dimensions in the MCA explained for 75.4% of the total data variability, with the identification of four groups, confirmed by the cluster analysis.DiscussionOur results suggest that robust breastfeeding hospital policies and practices influence the establishment and maintenance of breastfeeding in both healthy and at-risk infants. It is advisable to plan and implement additional strategies to ensure that vulnerable and healthy newborns receive optimal feeding. It is necessary to devote extra effort particularly to at-risk infants who are more vulnerable to negative outcomes.ConclusionAt-risk newborns did not exclusively breastfeed to the same extent as healthy newborns at hospital discharge. A different approach is required for at-risk neonates, who are more physically challenged and more vulnerable to problems associated with initiation and maintenance of breastfeeding.
| S-EPMC7412808 | biostudies-literature
Project description:BackgroundInfliximab therapy is effective in controlling symptoms and attaining clinical remission of immune-mediated inflammatory diseases. However, treatment adherence is essential to achieve the therapeutic objective. This study aimed to determine the rate of adherence to infliximab treatment in patients treated at a referral center at a university hospital.MethodThis ambispective cohort study included patients treated at the Professor Edgard Santos University Hospital (HUPES) referral center of our university hospital between March 2022 and February 2023. Sociodemographic, clinical, and pharmacotherapeutic data were collected from 101 patients through interviews and medical record reviews using a structured form. The adherence rate was defined as the proportion of days covered in a year. Patients who achieved an adherence rate > 80% were considered adherent.ResultsThe treatment adherence rate was 91.04%. Individuals with inflammatory bowel diseases had a 39.1% higher risk of non-adherence to treatment compared with other patients in our sample (p < 0,05). Most patients achieved remission or control of the underlying disease activity and had good functional capacities. The main reason for absence on the scheduled date was difficulty traveling to the referral center.ConclusionsDespite the reported difficulties, treatment adherence was observed to be high. As the study was conducted in a reference unit with multidisciplinary care and continuous monitoring for treatment effectiveness, safety, and adherence, welcoming and good communication between professionals and patients may have contributed to the high adherence rate.
| S-EPMC11523816 | biostudies-literature
Project description:Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) has received increased attention because of its novel treatment options. Carpal tunnel syndrome (CTS) is known as early symptoms in transthyretin amyloidosis (ATTR) preceding cardiac involvement and one of the "red flags" for ATTR-CM. A 64-year-old man underwent carpal tunnel release for carpal tunnel syndrome at 62 years. He was diagnosed with wild-type ATTR due to deposition of transthyretin (TTR) amyloid in flexor tenosynovium specimens and no TTR gene mutation. Examination for detection of cardiac involvement was performed after the operation, and there were no definitive findings of ATTR-CM; however, an early stage of ATTR-CM remained a possibility. Serial image evaluation and biomarker analysis revealed positive findings for ATTR-CM, and we performed an endomyocardial biopsy, resulting in the detection of amyloid deposition. He was diagnosed with ATTRwt-CM 2 years after the operation, and even then, he had no heart failure symptoms. Early diagnosis and treatment are important for the improvement of clinical outcomes in patients with ATTRwt-CM. TTR deposition in the ligaments or tendons is often observed in patients with CTS and should be considered at high risk of future ATTR-CM. Serial follow-up of these patients may enable the diagnosis of preclinical ATTR-CM. <Learning objective: Bilateral carpal tunnel syndrome (CTS) patients with transthyretin deposition in surgically-excised specimens are considered to be at high risk of future transthyretin amyloid cardiomyopathy (ATTR-CM). Serial evaluation of imaging results and cardiac biomarkers are useful for the diagnosis of preclinical ATTR-CM.>.
| S-EPMC8617475 | biostudies-literature