Brazilian Tertiary Center Research Articles

Factors related to the discontinuation and mortality rates of a cardiac rehabilitation programme in patients with Chagas disease: a 6-year experience in a Brazilian tertiary centre.

To describe the clinical and sociodemographic characteristics of participants as well as discontinuation and mortality rates in a cardiac rehabilitation programme (CRP) tailored to Chagas disease (CD). Participants underwent functional capacity, anthropometry and cardiac function evaluations before beginning a CRP. Univariate and multivariate Cox proportional hazards models were performed to investigate the associations between clinical and sociodemographic characteristics at baseline with discontinuation rates and deaths. Forty-two patients were enrolled in the CRP (61.9% men, mean age of 58.1±11.8years). During a median follow-up period of 10.8months, 74% discontinued and 14% died while enrolled in CRP. 34% of the patients who discontinued CRP died during follow-up. White race (HR=0.09; 95% CI 0.01-1.00), right ventricular systolic dysfunction (HR=10.54; 95% CI 1.24-89.50) and oxygen pulse (HR=0.69; 95% CI 0.48-0.99) were independently associated with death while enrolled in CRP. Married status (HR=0.44; 95% CI 0.21-0.95) was independently associated with discontinuation rates from CRP. VO2 peak (HR=0.85; 95% CI 0.74-0.98) and CRP discontinuation due to CD-related reasons (HR=8.33; 95% CI 1.91-36.27) were the variables independently associated with death after discontinuation of CRP. In this population, sociodemographic aspects and severity of CD were important determinants of CRP discontinuation and mortality.

Primary peritoneal drainage in neonates with necrotizing enterocolitis associated with congenital heart disease: a single experience in a Brazilian tertiary center.

Necrotizing enterocolitis (NEC) is a common condition in preterm infants. The risk factors that contribute to NEC include asphyxia, apnea, hypotension, sepsis, and congenital heart diseases (CHD). The objective of this study was to evaluate the association between the treatment (surgery or drainage) and unfavorable outcomes in neonates with NEC and congenital heart diseases (NEC+CHD). A 19-year retrospective cohort study was conducted (2000-2019). Inclusion criterion was NEC Bell II stage. Exclusion criteria were associated malformation or genetic syndrome and those who did not undergo echocardiography or had a Bell I diagnosis. We included 100 neonates: NEC (n=52) and NEC+CHD (n=48). The groups were subdivided into NEC patients undergoing surgery (NECS, n=31), NEC patients undergoing peritoneal drainage (NECD, n=19), NEC+CHD patients undergoing surgery (NECCAS, n=21), and NEC+CHD patients who were drained (NECCAD, n=29). Multivariate analysis was performed to estimate the relative risk of death and the length of stay. Covariates were birth weight and gestational age. The group characteristics were similar. The adjusted relative risk of death was higher in the drainage groups [NECD (Adj RR=2.70 (95%CI: 1.47; 4.97) and NECCAD (Adj RR=1.97 (95%CI: 1.08; 3.61)], and they had the shortest time to death: NECD=8.72 (95%CI: 3.10; 24.54) and NECCAD=5.32 (95%CI: 1.95; 14.44). We concluded that performing primary peritoneal drainage in neonates with or without CHD did not improve the number of days of life, did not decrease the risk of death, and was associated with a higher mortality in newborns with NEC and clinical instability.

Trends in prescribing patterns of antiepileptic drugs among older adult inpatients in a Brazilian tertiary center

Data on prescribing patterns of antiepileptic drugs (AEDs) to older adult inpatients are limited. To assess changes in prescribing patterns of AEDs to older adult inpatients with late-onset epilepsy between 2009-2010 and 2015-2019, and to interpret any unexpected patterns over the 2015-2019 period. Patients aged ≥60 years with late-onset epilepsy from a tertiary center were selected. Demographic data, seizure characteristics and etiology, comorbidities, and comedications were analyzed, in addition to prescription regimens of inpatients taking AEDs to treat epilepsy. AED regimens were categorized into two groups: group 1 included appropriate AEDs (carbamazepine, oxcarbazepine, valproic acid, gabapentin, clobazam, lamotrigine, levetiracetam, topiramate, and lacosamide); and group 2 comprised suboptimal AEDs (phenytoin and phenobarbital). Multivariate logistic regression analysis was performed to identify risk factors for prescription of suboptimal AEDs. 134 patients were included in the study (mean age: 77.2±9.6 years). A significant reduction in the prescription of suboptimal AEDs (from 73.3 to 51.5%; p<0.001) was found; however, phenytoin remained the most commonly prescribed AED to older adult inpatients. We also found an increase in the prescription of lamotrigine (from 5.5 to 33.6%) and levetiracetam (from 0 to 29.1%) over time. Convulsive status epilepticus (SE) and acute symptomatic seizures associated with remote and progressive etiologies were risk factors for the prescription of suboptimal AEDs. Phenytoin was the main suboptimal AED prescribed in our population, and convulsive SE and acute symptomatic seizures associated with some etiologies were independent risk factors for phenytoin prescription. These results suggest ongoing commitment to reducing the prescription of suboptimal AEDs, particularly phenytoin in Brazilian emergence rooms.

Immunosuppressant-induced cutaneous drug reactions in solid organ transplant recipients

Solid organ transplant recipients (SOTRs) are susceptible to various cutaneous side effects as a consequence of long-term immunosuppressive therapy. Skin cancers and infections are well-studied complications that can cause death and/or allograft rejection. Other cutaneous drug reactions, such as inflammatory manifestations, have a high prevalence but are rarely studied. We analyzed these manifestations' prevalence and their association with immunosuppressants in transplant recipients from a Brazilian tertiary center. Among 532 SOTRs followed at our dermatology clinic, 60 (11.3%) developed some cutaneous adverse reactions to the immunosuppressants, with a median age at transplantation of 50.5 years and a median life span posttransplantation of seven years. Acneiform eruption was the most common drug reaction found (21 patients, 30.4%), followed by diffuse non-scarring alopecia (16 patients, 23.1%), lymphedema (10 patients, 14.5%), gingival hyperplasia (7 patients, 10.1%), hypertrichosis (6 patients, 8.7%) and sebaceous hyperplasia (9 patients, 13.1%). Adequate immunosuppression is an essential prerequisite for successful organ transplantation. In the immediate post-transplant period, significant immunosuppression is needed, but after that, the complications of excessive immunosuppression outweigh the risk of organ rejection. SORTs may present with a broad spectrum of inflammatory and cosmetic findings due to immunosuppressants that can impair life quality.

Childhood Glaucoma Profile in a Brazilian Tertiary Care Center Using Childhood Glaucoma Research Network Classification.

To apply the Childhood Glaucoma Research Network (CGRN) classification in children from a pediatric glaucoma outpatient clinic in a tertiary Brazilian care center to better understand the prevalence and the demographic and clinical characteristics of each subtype of childhood glaucoma. This was a retrospective study in which medical records were collected from patients assisted in childhood glaucoma's clinic at the Department of Ophthalmology and Visual Sciences, Federal University of São Paulo from January 2010 to January 2018. The initial diagnosis and new diagnosis according to CGRN classification were collected. In addition, age, sex, visual acuity, and affected eye were recorded. From 624 ophthalmic consultations in the clinic, 496 had confirmed diagnosis of childhood glaucoma or glaucoma suspect. Mean age at diagnosis of the entire group was 3.62±4.23 years (range, 0 to 17 y). Two hundred eight-one (56.65%) of all patients were male and 349 patients (81.16%) had bilateral disease. All patients fit 1 of the 7 categories of the new classification. In all, 48.59% of diagnoses were changed upon reclassification. According to CGRN classification, 220 patients (44.35%) had primary glaucoma (juvenile open-angle glaucoma and primary congenital glaucoma); 210 patients (42.34%) had secondary glaucoma (glaucoma associated with nonacquired ocular anomalies; glaucoma associated with nonacquired systemic disease or syndrome; glaucoma associated with acquired condition; and glaucoma following cataract surgery); and 66 patients (13.31%) were glaucoma suspect. The most common diagnoses found were primary congenital glaucoma with 218 cases (43.95%) and the last common diagnosis was juvenile open-angle glaucoma with 2 cases (0.40%). The CGRN classification provides a useful method for classifying childhood glaucoma. A single, standardized classification system is important for diagnosis and management these patients, in addition, may improve physician communication, and future research.

The change in the clinical presentation of Graves' disease: a 30 years retrospective survey in an academic Brazilian tertiary center.

Graves' disease (GD) is the main cause of hyperthyroidism among adults. It is an autoimmune condition classically marked by the Merserburg Triad (goiter, thyrotoxicosis, and orbitopathy), but the change in presentation of GD over time has rarely been studied. To determine changes in the clinical presentation of patients with GD in the last 30 years. The study evaluated 475 patients diagnosed with GD between 1986 and 2016 in a single center. Patients were evaluated regarding epidemiological aspects, thyroid function, inflammatory activity of the eyes evaluated by the Clinical Activity Score; CAS, severity evaluated by NOSPECS classification and thyroid volume estimated by ultrasonography. Patients assessment identified an increase in the mean age of diagnosis of GD (p < 0.02), a reduction in thyroid volume (p < 0.001) and less intense orbital involvement from 2007-2016 compared to 1986-2006 (p = 0.04). The number of smoking patients was smaller from 2007 to 2016 (28.7%) than 1986 to 2006 (42.8% p = 0.001). The TSH and TRAb values did not had significant changes. GD presentation appears to be changed in the last years compared to the typical initial presentation. There is a less frequent inflammatory involvement of orbital tissue, smaller goiters, a lower number of smokers and diagnosis at older age.

Hepatitis C in Children and Adolescents of a Brazilian Tertiary Center: Identifying Patients Eligible for Direct-Acting Antivirals.

We evaluated 113 pediatric patients with chronic hepatitis C from 2009 to 2019 at a Brazilian tertiary center. Seventy patients received pegylated-interferon treatment. The sustained virologic response was 61.4%, and 92.8% reported side effects. Currently, we are following 39 patients with chronic hepatitis C, 24 of whom are eligible for treatment with direct-acting antivirals according to Brazilian recommendations.

The molecular landscape of osteogenesis imperfecta in a Brazilian tertiary service cohort.

Defects in type 1 collagen reportedly account for 85-90% of osteogenesis imperfecta (OI) cases, but most available molecular data has derived from Sanger sequencing-based approaches in developed countries. Massively parallel sequencing (MPS) allows for systematic and comprehensive analysis of OI genes simultaneously. Our objective was to obtain the molecular diagnosis of OI in a single Brazilian tertiary center cohort. Forty-nine individuals (84% adults) with a clinical diagnosis of OI, corresponding to 30 sporadic and 8 familial cases, were studied. Sixty-three percent had moderate to severe OI, and consanguinity was common (26%). Coding regions and 25-bp boundaries of 15 OI genes (COL1A1, COL1A2, IFITM5 [plus 5'UTR], SERPINF1, CRTAP, P3H1, PPIB, SERPINH1, FKBP10, PLOD2, BMP1, SP7, TMEM38B, WNT1, CREB3L1) were analyzed by targeted MPS and variants of interest were confirmed by Sanger sequencing or SNP array. A molecular diagnosis was obtained in 97% of cases. COL1A1/COL1A2 variants were identified in 71%, whereas 26% had variants in other genes, predominantly FKBP10, PLOD2, and SERPINF1. A potential digenic interaction involving P3H1 and WNT1 was identified in one case. Phenotypic variability with collagen defects could not be explained by evident modifying variants. Four consanguineous cases were associated to heterozygous COL1A1/COL1A2 variants, and two nonconsanguineous cases had compound PLOD2 heterozygosity. Novel disease-causing variants were identified in 29%, and a higher proportion of non-collagen defects was seen. Obtaining a precise diagnosis of OI in underrepresented populations allows expanding our understanding of its molecular landscape, potentially leading to improved personalized care in the future.

Ultrasound-guided antecubital vein approach for right heart catheterisation in a Brazilian tertiary centre

ObjectiveAs a parallel to the radial approach for left heart catheterisation, forearm veins may be considered for the performance of right heart catheterisation. However, data regarding the application of this...

Neonatal manifestations in COVID-19 patients at a Brazilian tertiary center

The most common symptoms of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children are fever and cough, which are present in approximately 59% of cases (). Other symptoms include bloating, nausea, vomiting, diarrhea, and abdominal pain. Although the evolution is generally benign, the clinical picture of SARS-CoV-2 infection in neonates is still poorly understood (). It is always necessary to consider that severe respiratory distress can also occur in other conditions that are inherent to pulmonary immaturity at [...]

The discontinuation of antitumor treatment and the advance directives in cancer patients

OBJECTIVES: Advance directives (AD) are one of the main pillars of patients' autonomy and can impact directly the quality of life, dignity and rights of the dying patient. Despite this, advance directives face many barriers to be created, implemented and followed. The moment of discontinuation of active anticancer therapy is an important landmark in the palliative evolution of cancer patients. The aim of this study is to investigate if the discontinuation of antitumor treatment could influence rates of advance directives. METHODS: A single-center retrospective case-notes review study with 321 randomly selected patients of a Single Brazilian tertiary oncology center. Qualitative and quantitative data were collected and analyzed to compare two groups of patients, one group with advance directives registered and another group without directives. RESULTS: The rate of advance directives was 22.7%, and in 82.3% of subjects, the decision of treatment discontinuation occurs on the same day or before the AD manifestation. In multivariable analysis, 82.5% of patients with advance directives were in the doctor's office at the occasion of the discontinuation of their anticancer therapy compared with 64.4% of the patients without directives (RR 1.88; 95%CI 1.019-3.496). The most cited

P022 Autologous hematopoietic stem cell transplantation in refractory Crohn's disease: series case report in a Brazilian tertiary center

BACKGROUND: Crohn's disease (CD) is a chronic inflammatory and relapsing disease that can affect any segment of the gastrointestinal tract causing intestinal and extra-intestinal manifestations. A significant percentage of these patients suffer an aggressive disease course, refractory to pharmacological therapy and most of them are not favorable to surgical treatment, besides not being a curative procedure. Recent analysis of autologous haematopoietic stem cell transplantation (HSCT) showed a high rate of clinical and endoscopic remission after 1 year in these patients. METHODS: In 2015 our hospital developed a partnership protocol with ministry of health to submit adult patients from many cities of Brazil, with refractory CD to perform HSCT as an optional treatment. The follow up period of this protocol was only 6 months. We analyzed the 6 patients from this protocol to assess the efficacy and safety of autologous HSCT for refractory CD. RESULTS: Five men and 1 woman, between 43y and 19y (median age 38y), corporal index mass 20 Kg/m2 (18–25), with refractory CD, median course of illness 14y (5–23 years), all negative for X-linked inhibitor apoptosis protein (XIAP). Two patients were non-structuring non-penetrating, but only one hadn't had previous surgery. Five patients had 2–3 previous intestinal surgeries, and one had ileostomy, 2 had tuberculosis, one had multiple sclerosis, one had epilepsy and depression and one had pancreatitis as comorbities before HSCT. One patient had reactivation of tuberculosis and was treated during 6 months before the HSCT. All patients used thiopurines, metotrexate and more than one immunobiological therapies (IFX, ADA, VEDO), steroids, antibiotics, and aminosalicylates. All the 6 patients completed the mobilization, conditioning and transplantation phases, during a median time of hospitalization of 35 days (21–58d). We had no transplant related mortality. All patients had infectious complications during mobilization which included febrile neutropenia, anemia and life-threatening complications as KPC, Staphylococcus aureus infecction, and sepsis caused by E. coli. Among non-infectious complications all had mucositis, and red cell transfusion was required in all patient. The 6 months follow up of protocol all patients reached clinical and endoscopic remission and were steroid and immunosuppressive free remission. Most of them related viral infections. Only the youngest patient answered the SF36, IBDQ and PedsQL4.0 questionnaire pre and post HSCT, with improvement of health-related quality of life in all domains. Although the project last 6m, actually all patients are alive and the longest post HSCT time is 2y 9m. Only one patient restarted ADA as monotherapy (1y11m post HSCT). CONCLUSION: Due to its high complexity, risk of infections and death we consider that autologous HSCT should be an option of treatment only for patients with severe refractory CD.

P365 Autologous haematopoietic stem cell transplantation in refractory Crohn’s disease: experience of a Brazilian tertiary centre

P365 Autologous haematopoietic stem cell transplantation in refractory Crohn’s disease: experience of a Brazilian tertiary centre

004 - Extranodal NK/T cell lymphoma, nasal type in a Brazilian tertiary center

004 - Extranodal NK/T cell lymphoma, nasal type in a Brazilian tertiary center

005 - Transformed mycosis fungoides in a Brazilian tertiary center

005 - Transformed mycosis fungoides in a Brazilian tertiary center

Epidemiological and clinical profile of infective endocarditis at a Brazilian tertiary care center: an eight-year prospective study.

Infective endocarditis (IE) is a systemic infectious disease requiring a multidisciplinary team for treatment. This study presents the epidemiological and clinical data of 73 cases of IE in Rio de Janeiro, Brazil. This observational prospective cohort study of endocarditis patients during an eight-year study period described 73 episodes of IE in 70 patients (three had IE twice). Community-associated (CAIE) and healthcare-acquired infective endocarditis (HAIE) were diagnosed according to the modified Duke criteria. The collected data included demographic, epidemiologic, and clinical characteristics, including results of blood cultures, echocardiographic findings, surgical interventions, and outcome. Analysis of data from the eight-year study period and 73 cases (70 patients) of IE showed a mean age of 46 years (SD=2.5 years; 1-84 years) and that 65.7% were male patients. The prevalence of CAIE and HAIE was 32.9% and 67.1%, respectively. Staphylococcus aureus (30.1%), Enterococcus spp. (19.1%), and Streptococcus spp. (15.0%) were the prevalent microorganisms. The relevant signals and symptoms were fever (97.2%; mean 38.6 + 0.05°C) and heart murmur (87.6%). Vegetations were observed in the mitral (41.1%) and aortic (27.4%) valves. The mortality rate of the cases was 47.9%. In multivariate analysis, chronic renal failure (relative risk [RR]= 1.60; 95% confidence interval [CI] 1.01-2.55), septic shock (RR= 2.19; 95% CI 1.499-3.22), and age over 60 years (RR= 2.28; 95% CI 1.44-3.59) were indirectly associated with in-hospital mortality. The best prognosis was related to the performance of cardiovascular surgery (hazard ratio [HR]= 0.51; 95% CI 0.26-0.99).

Seizure Outcome of Patients with Bilateral Temporal Lobe Sclerosis: A Controlled Study in a Tertiary Epilepsy Center

Introduction: Surgery for refractory temporal lobe epilepsy (TLE) has been a well-defined procedure to provide seizure control in the recent years. Objective: The aim of the present study is to evaluate seizure outcome of patients with TLE and bilateral mesial temporal sclerosis (bMTS) after cortico-amygdalohippocampectomy compared with those presenting unilateral (uMTS) lesions. Method: A case-controlled study was conducted in patients with TLE and bMTS or uMTS treated in the epilepsy clinic of the Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), a Brazilian tertiary referral epilepsy center, from January 2000 to March 2012. Patients with neuroradiological evidence and/or neuropathological confirmation of other diseases than MTS, as well as additional potential epileptogenic MRI-lesions, were excluded from the study. Results: During this period, 533 patients underwent multidisciplinary investigation of epilepsy and 229 (43%) fulfilled criteria to be included in the present study, 60 (26.2%) with bMTS and 169 (73,8%) with uMTS. Thirty-seven (61.6%) were classified as Engel I in bMTS group, while 107 (63.3%), were classified as Engel in uMTS group. There was no mortality in both groups. Conclusion: The present data highlight that a favorable seizure outcome can also be obtained in patients with bMTS after adequate pre-surgical evaluation.

168. Comparison between frequency of thrombophilia in patients with and without severe preeclampsia in a Brazilian Tertiary Center.

Introduction In the last two decades studies have associated thrombophilia with the risk of developing severe preeclampsia (PE). Few of them, however, have been conducted in Latin America to assess the importance of thrombophilia in these patients. Objective Evaluate thrombophilia frequency among patients with and without severe preeclampsia in a Brazilian Tertiary Center. Methods From October 2009 to October 2015, a retrospective case-control study was conducted in pregnant women with severe PE and compared to normal pregnant women at Hospital das Clinicas, FMUSP. The patients with PE who had no gestational trophoblastic disease, foetal malformation or chromosomal abnormalities and who underwent thrombophilia screening during the postnatal period were included. Pregnant women without comorbidities and normal obstetric outcome were included to make up the normal group, and were screened for thrombophilia during prenatal care (when functional dosages were abnormal, they were repeated in the postnatal period). Factor V Leiden (FVL), G20210A prothrombin mutation (PM), antithrombin, protein C, protein S, homocysteine, lupus anticoagulant, and anticardiolipin IgG and IgM antibodies were analysed. The frequency of thrombophilia in the two groups was compared. Results We included 199 patients with severe pre-eclampsia and 50 normal pregnant women. In patients with severe PE we found 55 (27,6%) women that had at least one thrombophilia (14 Antiphospholipid Syndrome,10 heterozygous PM, 10 protein S deficiency, 9 hyperhomocysteinemia, 9 heterozygous FVL, 2 antithrombin deficiency, 4 association of thrombophilia). In the control group 2 (4%) women had positive thrombophilia research (1 heterozygous PM and 1 heterozygous FVL), p Discussion Our results show a higher rate of thrombophilia among the patients with servere PE (p

Risk Factors for Mortality in Children with Congenital Heart Disease Delivered at a Brazilian Tertiary Center.

ObjectiveThis study aims to investigate the incidence of postnatal diagnosis of congenital heart disease (CHD) and the predictive factors for hospital mortality.MethodsThis retrospective cohort study was conducted at a Brazilian tertiary center, and data were collected from medical records with inclusion criteria defined as any newborn with CHD diagnosed in the postnatal period delivered between 2015 and 2017. Univariate and multivariate analyses were performed to determine the potential risk factors for mortality.ResultsDuring the 3-year period, 119 (5.3%) children of the 2215 children delivered at our institution were diagnosed with CHD. We considered birth weight (P=0.005), 1st min Apgar score (P=0.001), and CHD complexity (P=0.013) as independent risk factors for in-hospital mortality. The most common CHD was ventricular septal defect. Indeed, 60.5% cases were considered as "complex" or "significant" CHDs. Heart surgeries were performed on 38.9% children, 15 of whom had "complex" or "significant" CHD. A mortality rate of 42% was observed in this cohort, with 28% occurring within the initial 24 h after delivery and 38% occurring in patients admitted for heart surgery.ConclusionThe postnatal incidence of CHD at our service was 5.3%. Low 1st min Apgar score, low birth weight, and CHD complexity were the independent factors that affected the hospital outcome.

TCT-197 Ultrasound-guided Antecubital Vein Approach for Right Heart Catheterization in a Brazilian Tertiary Center

Introduction: Data regarding safety and feasibility of right heart catheterizations (RHC) through antecubital vein approach is heterogeneous and scarce. Moreover, forearm venous access acquisition under ultrasound guidance has not been specifically evaluated in this scenario. Methods: In order to evaluate success rate and radiation exposure of RHC through antecubital vein approach, data from consecutive RHC performed at our center between January 2014 and March 2017 were collected. Demographic data and procedural outcomes were compared between patient groups defined by venous access site. Results: In the period, 310 RHC were performed (antecubital vein approach, n=127; femoral vein approach, n=152; jugular vein approach, n=31). There were 129 combined right and left heart catheterizations (LHC): antecubital vein/radial artery approach, n= 57; femoral vein/femoral artery approach, n= 72. Pulmonary hypertension and heart failure were the main indications for both single and combined procedures. Antecubital ...