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1.
J Thromb Thrombolysis ; 57(5): 805-814, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38643312

ABSTRACT

INTRODUCTION: Cancer-associated thrombosis (CAT) is a significant concern among patients with malignant diseases, leading to increased mortality. While current guidelines recommend primary thromboprophylaxis for venous thromboembolism (VTE) in medium-to-high-risk outpatients, this practice remains controversial. A better understanding of primary thromboprophylaxis is crucial, yet there is a lack of Real-World Evidence (RWE) in Portugal. AIMS: This RWE study aimed to elucidate primary thromboprophylaxis practices among cancer outpatients in Portugal. METHODS: A five-year observational multicentric study in eight Portuguese health institutions enrolled 124 adult cancer outpatients under primary thromboprophylaxis for VTE. The endpoints were CAT, bleeding, cancer progression and death. RESULTS: High thrombotic risk tumours were prevalent, with 57% (71) of the patients presenting with pancreatic and gastric cancers. Regarding primary thromboprophylaxis, 55% (68) received Low-Molecular-Weight Heparin (LMWH). VTE was presented in 11% (14) of the patients and major bleeding in 2% (2). Vascular compression, elevated D-dimer and previous VTE were significantly associated with VTE occurrence under primary thromboprophylaxis. The Onkotev model was shown to be the best risk assessment model (RAM) in this population (p = 0.007). CAT patients exhibited a lower progression-free survival than non-CAT patients (p = 0.021), while thrombosis did not influence overall survival (p = 0.542). CONCLUSION: Primary thromboprophylaxis in medium-to-high-risk cancer outpatients is a safe and effective practice in real-world settings. This study is the first Portuguese RWE on primary thromboprophylaxis, highlighting evidence for improving prophylactic strategies in this population.


Subject(s)
Anticoagulants , Neoplasms , Outpatients , Venous Thromboembolism , Humans , Neoplasms/complications , Venous Thromboembolism/prevention & control , Venous Thromboembolism/etiology , Female , Middle Aged , Male , Aged , Portugal/epidemiology , Anticoagulants/therapeutic use , Anticoagulants/administration & dosage , Hemorrhage/chemically induced , Heparin, Low-Molecular-Weight/therapeutic use , Heparin, Low-Molecular-Weight/administration & dosage , Risk Assessment , Adult , Risk Factors
2.
Int J Cardiol ; 396: 131430, 2024 Feb 01.
Article in English | MEDLINE | ID: mdl-37827282

ABSTRACT

BACKGROUND: Prenatal diagnosis (PND) of aortic coarctation (AoCo) has been associated with a significant improvement in early results, but there is limited information on the long-term cardiovascular outcome. METHODS: We studied 103 patients with simple AoCo, operated in the neonatal period, with a median follow-up of 8,5 years (2 to 23,7 years), with 47% followed for over 10 years. PND was made in 35%. The primary aim was to determine the short and long-term cardiovascular impact of PND of AoCo. RESULTS: Neonates with PND had less preoperative neonatal complications, with only 2,8% incidence of a composite preoperative severe morbidity course, compared to 28% in the postnatal group. PND patients underwent surgery 8 days earlier and had a shorter length of stay in ICU. PND did not impact the incidence of post-operative complications. On the long-term, prevalence of hypertension, left ventricular hypertrophy and rate of recoarctation were not influenced by PND. The PND group had mean 24 h diastolic BP 9 mmHg lower and mean daytime diastolic BP 11 mmHg lower. In the final multivariable model, PND was the single independent variable correlating with daytime diastolic BP. CONCLUSION: PND of AoCo effectively leads to a better pre-operative course with less pre-operative morbidity. We found no significant differences in immediate post-operative cardiovascular outcomes. A better initial course of patients with PND does not have a major long-term impact on cardiovascular outcomes, nevertheless, at late follow-up PND patients had lower diastolic BP values on ambulatory monitoring, which may have an impact on long-term cardiovascular risk.


Subject(s)
Aortic Coarctation , Cardiovascular System , Hypertension , Infant, Newborn , Pregnancy , Female , Humans , Aortic Coarctation/diagnostic imaging , Aortic Coarctation/epidemiology , Aortic Coarctation/surgery , Prenatal Diagnosis , Hypertension/epidemiology , Prevalence , Follow-Up Studies
3.
J Pediatr Surg ; 58(9): 1744-1753, 2023 Sep.
Article in English | MEDLINE | ID: mdl-36967253

ABSTRACT

OBJECTIVES: Vascular rings may cause tracheal and/or oesophageal compression. For many patients, symptoms/signs have been present for a long period before diagnosis. However, in the era of prenatal diagnosis, some units advocate universal early surgery. The risks and efficacy of surgery must be known to adequately counsel for the operation. This meta-analysis sought to define the morbidity and mortality associated with surgical correction, and persistent post-operative symptoms. METHODS: PubMed, Cochrane Library and CINAHL databases were searched for studies that described the outcome of patients undergoing surgery for a double or right aortic arch (DAA or RAA). Non-comparative and random effects model-based meta-analyses were conducted to calculate the pooled rates of mortality, surgical complications, reintervention, and persistent follow-up symptoms. RESULTS: Nineteen eligible studies were included comprising 18 studies describing outcomes for DAA surgery and 15 for RAA surgery. For DAA surgery, overall mortality rate was 0% [95% confidence interval (CI) 0.0-1.0], post-surgical complication rate 18% [95% CI: 12.0-23.0], prevalence of reintervention 3% [95% CI: 1.0-5.0] and prevalence of symptoms at last follow-up was 33% [95% CI: 17.0-52.0]. For RAA surgery, overall pooled mortality was 0% [95% CI: 0.0-0.0], prevalence of post-surgical complications was 15% [95% CI: 8.0-23.0], reintervention rate was 2% [95% CI: 0.0-4.0], prevalence of symptoms at last follow-up was 40% [95% CI: 26.0-55.0]. CONCLUSIONS: While surgery to correct a vascular ring is safe, the rate of persistent symptoms is high and further strategies must be sought to reduce this burden.


Subject(s)
Vascular Ring , Pregnancy , Female , Humans , Aorta, Thoracic/surgery , Prenatal Diagnosis , Trachea , Subclavian Artery/surgery
4.
Pediatr Cardiol ; 44(3): 674-680, 2023 Mar.
Article in English | MEDLINE | ID: mdl-36028777

ABSTRACT

Aortic coarctation (AoCo) leads to long-term sequelae that may impair heart function. Data regarding new echocardiographic function parameters such as atrial strain, in affected patients, are scarce. This study aims to describe these parameters in AoCo patients and define their association with severity measures. 53 AoCo patients and 31 healthy controls, aged 12-40 years, were evaluated. Effectively corrected AoCo (cAoCo) was defined as aortic trans-isthmic corrected Doppler gradient (Dgrad) ≤ 20 mmHg (n = 36), and recoarctation (rAoCo) as Dgrad > 20 mmHg (n = 17). Dependent variables were: E/E'; atrial reservoir strain (Ares); and atrial conduit strain (Acd). T-tests/Mann-Whitney U tests were used to compare these among groups. Multivariable regression was used to test correlation with systolic blood pressure (SBP), indexed LV mass (ILVM), Dgrad, and the ratio between the narrowest diameter of aortic arch and aorta at diaphragm level (Aoratio). In cAoCo and rAoCo patients, E/E' was higher (p < 0.001), Ares, and Acd were lower (p < 0.001 for both) comparing with controls. Acd was higher in cAoCo than rAoCo (p = 0.045). Higher Ares was associated with higher Aoratio (p = 0.002), and lower Acd with higher Dgrad (0.014). EF and GLS were not different among groups. Young patients with effectively corrected aortic coarctation have persistent changes in diastolic function parameters (E/E' and atrial strain), and these are affected by anatomical sequelae. These patients' physiology is closer to patients with recoarctation, than to healthy individuals. This provides rationale for a stronger prevention, and treatment, of arterial dysfunction and high left ventricular afterload in these patients.


Subject(s)
Aortic Coarctation , Atrial Fibrillation , Ventricular Dysfunction, Left , Humans , Atrial Fibrillation/complications , Aorta , Heart Atria , Aorta, Thoracic , Ventricular Function, Left/physiology
5.
J Oncol Pharm Pract ; 28(4): 975-978, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35037800

ABSTRACT

INTRODUCTION: Pericardial effusions are rare yet potentially fatal conditions in children. Azacitidine is a DNA-hypomethylating agent used in the treatment of myelodysplastic syndrome. Although seldomly described in adults, no cases of azacitidine-induced pericardial effusion have been reported in children. CASE REPORT: A 7-year-old boy with myelodysplastic syndrome presented with a large pericardial effusion with risk for cardiac tamponade after his first azacitidine cycle. MANAGEMENT & OUTCOME: The patient was admitted to a pediatric ICU, antibiotic and steroid therapy were initiated. Pericardiocentesis was done due to hemodynamic instability. Serum and pericardial fluid complementary evaluation excluded infectious and malignant causes. The pericardial effusion did not reappear and additional pleural and ascitic slight effusions responded well to diuretics. Follow-up azacitidine cycles were administered by tapering daily dosages and using adjunctive steroid therapy, with no additional adverse events. DISCUSSION: We report the first pediatric case of large pericardial effusion secondary to azacitidine therapy in a child with MDS. This adverse reaction has not been described in pediatric patients, in which this therapeutic option has been increasingly used. We seek to raise awareness on the potential life-threatening cardiotoxicity of azacitidine in pediatric patients.


Subject(s)
Cardiac Tamponade , Myelodysplastic Syndromes , Pericardial Effusion , Adult , Azacitidine/adverse effects , Cardiac Tamponade/chemically induced , Child , Humans , Male , Myelodysplastic Syndromes/drug therapy , Pericardial Effusion/chemically induced , Pericardiocentesis/adverse effects
6.
Thromb J ; 19(1): 103, 2021 Dec 20.
Article in English | MEDLINE | ID: mdl-34930339

ABSTRACT

BACKGROUND: Antiphospholipid Syndrome (APS) is a multisystemic autoimmune disease characterized by arterial and venous thrombosis and / or obstetric morbidity in the presence of at least one circulating anti-phospholipid antibody. The spectrum of vascular events varies from deep venous thrombosis to catastrophic APS, a rare form characterized by acute multiorgan thrombosis and high mortality. CASE REPORT: We present the case of a 32-week pregnant woman arriving in the hospital emergency room with bilateral acute lower limb ischemia. In the obstetric evaluation, fetal death was declared. Computerized Tomography angiography showed pulmonary embolism of both pulmonary arteries, areas of splenic and right renal infarction and multiple arterial and venous thrombosis. The patient underwent urgent caesarean section and axillary-bifemoral bypass. No events registered. In the postoperative period, in an intensive care unit, treatment with rituximab and plasmapheresis were added to anticoagulant therapy. The laboratorial investigation was negative for thrombophilia and autoimmune diseases. CONCLUSION: Catastrophic APS develops quickly, with multiorgan involvement and high mortality rate. The presented case poses a multidisciplinary challenge, with the surgical approach of extra-anatomical revascularization being less invasive and guaranteeing immediate perfusion of the lower limbs. Although the serological tests were negative for anti-phospholipid antibodies, this case hardly fits into another diagnosis. Therefore, it was treated as a catastrophic APS, having shown a favorable evolution.

7.
Sci Rep ; 11(1): 20837, 2021 10 21.
Article in English | MEDLINE | ID: mdl-34675344

ABSTRACT

Vitamin D is a fundamental regulator of host defences by activating genes related to innate and adaptive immunity. Previous research shows a correlation between the levels of vitamin D in patients infected with SARS-CoV-2 and the degree of disease severity. This work investigates the impact of the genetic background related to vitamin D pathways on COVID-19 severity. For the first time, the Portuguese population was characterized regarding the prevalence of high impact variants in genes associated with the vitamin D pathways. This study enrolled 517 patients admitted to two tertiary Portuguese hospitals. The serum concentration of 25 (OH)D, was measured in the hospital at the time of patient admission. Genetic variants, 18 variants, in the genes AMDHD1, CYP2R1, CYP24A1, DHCR7, GC, SEC23A, and VDR were analysed. The results show that polymorphisms in the vitamin D binding protein encoded by the GC gene are related to the infection severity (p = 0.005). There is an association between vitamin D polygenic risk score and the serum concentration of 25 (OH)D (p = 0.04). There is an association between 25 (OH)D levels and the survival and fatal outcomes (p = 1.5e-4). The Portuguese population has a higher prevalence of the DHCR7 RS12785878 variant when compared with its prevalence in the European population (19% versus 10%). This study shows a genetic susceptibility for vitamin D deficiency that might explain higher severity degrees in COVID-19 patients. These results reinforce the relevance of personalized strategies in the context of viral diseases.Trial registration: NCT04370808.


Subject(s)
COVID-19/blood , COVID-19/diagnosis , Polymorphism, Genetic , Vitamin D Deficiency/blood , Vitamin D/analogs & derivatives , Vitamin D/blood , Vitamin D/genetics , Aged , Biomarkers , Cholestanetriol 26-Monooxygenase/genetics , Cytochrome P450 Family 2/genetics , Female , Genetic Predisposition to Disease , Hospitalization , Humans , Male , Middle Aged , Oxidoreductases Acting on CH-CH Group Donors/genetics , Portugal/epidemiology , Prevalence , Severity of Illness Index , Vesicular Transport Proteins/genetics , Vitamin D-Binding Protein/genetics , Vitamin D3 24-Hydroxylase/genetics
8.
Ann Vasc Surg ; 73: 97-106, 2021 May.
Article in English | MEDLINE | ID: mdl-33493593

ABSTRACT

OBJECTIVES: This study aims to report the changes and adaptations of a vascular tertiary center during a global pandemic and the impact on its activity and patients. METHODS: We conducted a retrospective cohort study within the Vascular Surgery ward in Centro Hospitalar Universitário Lisboa Norte, Portugal. All data from surgical, inpatient and outpatient activity were collected from February to June 2020 and compared to the same 5-month period in 2018 and 2019. We ran a descriptive analysis of all data and performed statistical tests for the variation of procedures and admissions between February and June 2018 and the same time period in 2020. RESULTS: During the outbreak, our staff had to be readapted. Six nurses were transferred to COVID-19 units (out of a total of 33 nurses) while 1 of the 7 residents was transferred to an intensive care unit and 1 senior surgeon was put on prophylactic leave. In the outpatient clinic, there was an increase in the number of telemedicine consultations with a greater focus on first-time referrals and urgent cases. There was a significant increase in the total number of elective admissions whereas there were significantly less admissions from an emergency setting (+57% and -54%, respectively, P < 0.001). The vascular surgery team performed a total number of 584 procedures between February and June 2020 (-17.8% compared to 2018 and 2019), with a significant increase in the number of endovascular procedures (P < 0.001) and in the use of local and regional anesthesia (P < 0.001), especially in the Angio Suite (+600%, P < 0.001). Comparing with 2018 and 2019, the surgical team performed less outpatient procedures in early 2020. We reported a significant increase in the total number of procedures for patients with a chronic limb-threatening ischemia (CLTI) diagnosis (+21%, P < 0.001). We did not report significant changes in the proportion of other vascular conditions. Regarding mortality, we observed a 16% decrease in the intraoperative mortality (P 0.67). CONCLUSIONS: In this study, we assessed the impact of the COVID-19 outbreak in daily activity during the contingency period. During the outbreak, there was an overall decline in outpatient clinics and inpatient admissions. Nevertheless, and despite the restrictions imposed by the pandemic and health authorities, we managed to maintain most procedures for most vascular diseases, particularly for CLTI urgent cases, without a significant increase in the mortality rate. Stringent protective measures for patient and staff or higher use of endovascular techniques and local anesthesia are some of the successful changes implemented in the department. These learned lessons are to be pursued as the pandemic evolves with future outbreaks of COVID-19, such as the current second outbreak currently spreading through Europe.


Subject(s)
COVID-19 , Hospital Administration , Hospitalization/statistics & numerical data , Vascular Surgical Procedures/statistics & numerical data , Aged , Elective Surgical Procedures/statistics & numerical data , Female , Hospital Units/organization & administration , Humans , Male , Middle Aged , Portugal , Retrospective Studies , Vascular Diseases/epidemiology , Vascular Diseases/mortality , Vascular Diseases/surgery , Vascular Surgical Procedures/organization & administration
9.
Pediatr Emerg Care ; 37(9): e543-e546, 2021 Sep 01.
Article in English | MEDLINE | ID: mdl-31433364

ABSTRACT

OBJECTIVES: Focused cardiac ultrasound is an echocardiographic method used by medical intensivists for fast and reliable hemodynamic assessment. Prospective studies and guidelines have defined its role in adult critical care. Data regarding its use in pediatric critical care are scarce. This is the first prospective study that aims to evaluate its impact in this setting. METHODS: This is a single-center prospective study performed in a tertiary referral hospital pediatric intensive care unit (PICU). For a period of 6 months, when performing an echocardiogram, pediatric intensivists filled out a questionnaire that included the patient's clinical data and indication for the examination. The intensivists had to record both the clinical impression regarding that indication and therapeutic plan before and after the echocardiogram. All the patients with an echocardiogram performed by the pediatric intensive care unit medical staff were included. RESULTS: There were 80 echocardiograms performed on 35 patients during the study period. The most common patient diagnostic groups were respiratory infections (38%, n = 30) and septic shock (21%, n = 17). The main indication for the examination was assessment of intravascular volume status and left ventricular systolic function. After the echocardiogram, the clinical impression was maintained in 49% (n = 39) and changed in 44% (n = 35). There were new findings unrelated to the initial evaluation in 7% (n = 6). The planned treatment was maintained in 55% (n = 44) and changed in 45% (n = 36). CONCLUSIONS: The echocardiogram changed the clinical impression and therapeutic plan in almost half of the patients. These data show the value of focused cardiac ultrasound as a diagnostic and hemodynamic monitoring tool in pediatric intensive care and emphasize the importance of a rigorous training program.


Subject(s)
Echocardiography , Shock, Septic , Adult , Child , Critical Care , Humans , Intensive Care Units, Pediatric , Prospective Studies
11.
Pediatr Cardiol ; 41(8): 1730-1738, 2020 Dec.
Article in English | MEDLINE | ID: mdl-32770389

ABSTRACT

Speckle-tracking echocardiography has been used to assess atrial function. This analysis is feasible in univentricular hearts. The aim of this study was to characterize the relationship between atrial strain and functional capacity in the Fontan circulation. Apical four-chamber echocardiographic loops of 39 Fontan patients were reviewed. The dominant atrium was assessed for active (εact), conduit (εcon), and reservoir (εres) strain and εact/εres ratio. Cardiopulmonary exercise test was performed on the same day and oxygen uptake (VO2) at ventilatory threshold (VT) and peak VO2 were chosen as the dependent variables. Statistical analysis was performed using SPSS® version 23. Unpaired t test was used for binomial and continuous variable correlation; single and multivariable linear regression were used for continuous variable correlation. Statistical significance was defined as p value < 0.05. VO2 at VT as a percentage of predicted VO2 was 36.8% (SD 10.7). Peak VO2 was 64.7% (SD 18.9) of the predicted value. In univariate analysis, both were associated with age, atrioventricular regurgitation, ejection fraction, εres, εcon, and εact/εres. In multivariate regression, higher VO2 at VT and peak VO2 were associated with younger age (p = 0.003 and p = 0.001, respectively) and higher εcon (p = 0.026 and p = 0.020). Evaluation of heart function is difficult in the Fontan circulation, hindered by complex ventricular morphology and lack of normative data. VO2 provides a good surrogate. Atrial strain parameters are compromised in these patients and associated with VO2. Therefore, whenever possible, atrial strain should be measured as it may provide a new method of risk stratification.


Subject(s)
Atrial Function , Fontan Procedure/methods , Univentricular Heart/surgery , Adolescent , Child , Echocardiography/methods , Exercise Test , Female , Heart Atria/diagnostic imaging , Humans , Male , Multivariate Analysis , Oxygen Consumption , Univentricular Heart/diagnostic imaging , Univentricular Heart/physiopathology
12.
J Am Soc Echocardiogr ; 33(11): 1391-1399, 2020 11.
Article in English | MEDLINE | ID: mdl-32828625

ABSTRACT

INTRODUCTION: Speckle-tracking echocardiography has become a major tool in the evaluation of heart function. Atrial strain has emerged as an important component in the assessment of cardiac function, but there is a paucity of prenatal data. The aim of this study was to describe our initial experience of measurement of atrial strain in fetuses, with respect to both feasibility and the strain patterns observed. METHODS: Four-chamber Digital Imaging and Communications in Medicine loops were acquired prospectively for deformation imaging. Fifty-three normal fetuses with no morphologic or functional abnormalities were selected for analysis. The three strain components of atrial cycle for both left atrium (LA) and right atrium (RA) were acquired-reservoir (LAres or RAres), conduit, and contraction (LAct or RAct)-and are expressed as a percentage. Ratios of these components were calculated. Simple linear regression was used to analyze how the dependent variables changed according to gestational age and frame rate. RESULTS: The median gestational age was 30 weeks (range, 23-35), and the frame rate was 74 frames per second (fps; range, 35-121). Left atrial strain was feasible in 48/53 (91%), and right atrial strain in 46/53 (87%) of cases. The onset of LA contraction could be identified on the strain curves in 32 of 48 (67%) cases, and of the RA in 17 of 46 (37%) cases. The values of RAres and RAct were higher compared with those of LAres and LAct (33.9% vs 30.3%, P = .014; and 21.5% vs 16.8%, P = .005), and the contraction:reservoir ratio was also higher for RA (0.63 vs 0.55 for LA, P = .003). Higher values for LAres, LAct, RAres, and RAct were associated with higher frame rate (P = .007, .020, .049, and .012, respectively). The onset of LA contraction was better identified with a higher frame rate (mean 77 vs 59 fps when not seen, P = .007). A higher LA contraction:reservoir ratio was associated with a lower gestational age (P = .042). CONCLUSION: Measurement of atrial strain is feasible in the fetal heart. The values are influenced by gestational age and frame rate, so it is necessary to account for these variables. Comparison of left versus right atrial strain values contrasts with those observed postnatally. Atrial function merits further study during fetal life, to aid understanding of maturational changes and disease states.


Subject(s)
Atrial Function, Left , Echocardiography , Heart Atria , Atrial Function, Right , Female , Fetus , Heart Atria/diagnostic imaging , Humans , Infant , Pregnancy
15.
Int J Cardiol ; 306: 67-72, 2020 05 01.
Article in English | MEDLINE | ID: mdl-31785955

ABSTRACT

BACKGROUND: Peak oxygen uptake (peak VO2) and its decline predict death or serious cardiovascular adverse events in patients with Fontan circulation. Our aim was to study VO2 in a population of Fontan patients with variable age and contemporary surgical correction, and find predictors of functional status which could lead to management changes. METHODS: Prospective cardiopulmonary exercise test was performed on a treadmill. Blood tests and transthoracic echocardiogram were performed on the same day. Dependent variables were defined as: VO2 at ventilatory threshold (VT) as a percentage of the predicted peak VO2 and peak VO2 as a percentage of its predicted value. Statistical analysis was performed on SPSS® version 23 and significance was defined as a p-value < 0.05. RESULTS: Forty-eight patients were included for analysis. All had a lateral or extracardiac conduit. Mean age was 18.2 years (SD 6.2). Mean age at Fontan completion of 6.5 years (SD 2.3) showed no association with functional capacity. Mean percent VO2 at VT was 37.6% (SD 9.4) and percent peak VO2 was 67.8% (SD 16.7). VO2 both at ventilatory threshold and peak was associated with age, weekly sports practice, significant atrioventricular regurgitation and having a pacemaker or being on antiarrhythmic drugs. On multivariate analysis, weekly sports practice was the best predictor for VO2 values. CONCLUSIONS: Sports practice is a modifiable factor that significantly impacts functional capacity in Fontan patients despite their age. Clinicians should actively prescribe and promote physical activity in this population, either with regular sports practice or engagement in cardiac rehabilitation programs.


Subject(s)
Fontan Procedure , Adolescent , Adult , Child , Exercise Test , Exercise Tolerance , Fontan Procedure/adverse effects , Humans , Oxygen Consumption , Prospective Studies
16.
Cardiol Young ; 30(9): 1332-1334, 2020 Sep.
Article in English | MEDLINE | ID: mdl-31729309

ABSTRACT

Images of the diagnosis and correction of a pseudo-aneurysm at the right ventricular outflow tract, one of the rarest complications of Tetralogy of Fallot surgical correction.


Subject(s)
Aneurysm, False , Tetralogy of Fallot , Aneurysm, False/diagnostic imaging , Aneurysm, False/etiology , Heart Ventricles/diagnostic imaging , Heart Ventricles/surgery , Humans , Tetralogy of Fallot/surgery
17.
Acta Med Port ; 32(12): 782-784, 2019 Dec 02.
Article in English | MEDLINE | ID: mdl-31851888

ABSTRACT

Passive transfer of antibodies secondary to intravenous immunoglobulin infusion is a rare but important side effect that can lead to the wrong diagnosis and therapeutic decisions. It has never been reported in a newborn. A male newborn, vaccinated against hepatitis B and diagnosed with dilated cardiomyopathy, presented positive hepatitis B core antibodies at 12 days of life. Exclusion of hepatitis B infection was mandatory as it would be a contraindication to heart transplant. Passive transfer of antibodies was confirmed at 44 days of age, after seroreversion of hepatitis B core antibodies. Passive transfer of antibodies after intravenous immunoglobulin infusion can lead to a misleading diagnosis if not recognized. In our patient it could have been especially harmful had it prevented heart transplant. Screening for hepatitis B should be performed at least 1 month after intravenous immunoglobulin infusion.


A transferência passiva de anticorpos secundária à infusão de imunoglobulina endovenosa é um efeito secundário raro, mas importante, que pode levar a um diagnóstico e decisões terapêuticas erradas. Nunca foi descrito num recém-nascido. Um recém-nascido do sexo masculino, vacinado contra a hepatite B e diagnosticado com miocardiopatia dilatada, apresentou anticorpos anti-core do vírus da hepatite B aos 12 dias de vida. A exclusão da infecção por hepatite B foi obrigatória, pois seria uma contra-indicação ao transplante cardíaco. A transferência de anticorpos através de imunoglobulina endovenosa foi confirmada aos 44 dias de idade, após sero-reversão dos níveis de anticorpos anti-core do vírus da hepatite B. A transferência passiva de anticorpos após a infusão de imunoglobulina endovenosa pode levar a um diagnóstico errado se não for reconhecida. Neste doente poderia ter sido especialmente prejudicial caso tivesse impedido o transplante de coração. O rastreio para hepatite B deve ser realizado pelo menos um mês após a infusão.


Subject(s)
Hepatitis B Antibodies/blood , Hepatitis B/diagnosis , Immunization, Passive , Immunoglobulins, Intravenous/adverse effects , Seroconversion , Cardiomyopathy, Dilated/congenital , Cardiomyopathy, Dilated/surgery , Heart Transplantation , Hepatitis B/immunology , Hepatitis B/prevention & control , Humans , Immunoglobulins, Intravenous/administration & dosage , Infant, Newborn , Male , Viral Hepatitis Vaccines
18.
J Card Surg ; 34(5): 363-366, 2019 May.
Article in English | MEDLINE | ID: mdl-30932229

ABSTRACT

The authors report the case of an 18-year-old woman with Turner Syndrome and aortic coarctation, who developed aortic dissection after percutaneous stenting. Surgical treatment was necessary as the lesion progressed. This case highlights both the importance of awareness as well as multidisciplinary management of this potential complication.


Subject(s)
Aorta, Thoracic/surgery , Aortic Aneurysm/surgery , Aortic Coarctation/complications , Aortic Coarctation/surgery , Aortic Dissection/surgery , Turner Syndrome/complications , Adolescent , Blood Vessel Prosthesis Implantation/methods , Endovascular Procedures/methods , Female , Humans , Postoperative Complications/surgery , Stents , Treatment Outcome
19.
BMJ Case Rep ; 12(12)2019 Dec 29.
Article in English | MEDLINE | ID: mdl-31888914

ABSTRACT

We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein's anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)-functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein's anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.


Subject(s)
Alprostadil/therapeutic use , Ebstein Anomaly/diagnostic imaging , Ebstein Anomaly/drug therapy , Heart Defects, Congenital/diagnostic imaging , Vasodilator Agents/therapeutic use , Aftercare , Alprostadil/administration & dosage , Cardiomegaly/diagnostic imaging , Cardiomegaly/etiology , Congenital Abnormalities/diagnostic imaging , Ductus Arteriosus/physiopathology , Ebstein Anomaly/physiopathology , Echocardiography/methods , Female , Gestational Age , Humans , Infant, Newborn , Oxygen/blood , Pregnancy , Prenatal Diagnosis , Pulmonary Atresia/physiopathology , Pulmonary Valve/diagnostic imaging , Pulmonary Valve/physiopathology , Treatment Outcome , Vasodilator Agents/administration & dosage
20.
Ann Bot ; 119(3): 325-337, 2017 Feb.
Article in English | MEDLINE | ID: mdl-27818381

ABSTRACT

BACKGROUND AND AIMS: Rye supernumerary (B) chromosomes have an accumulation mechanism involving the B subtelomeric domain highly enriched in D1100- and E3900-related sequences. In this work, the effects of heat stress during the early stages of male meiosis in 0B and +B plants were studied. METHODS: In-depth cytological analyses of chromatin structure and behaviour were performed on staged rye meiocytes utilizing DAPI, fluorescence in situ hybridization and 5-methylcytosine immune labelling. Quantitative real-time PCR was used to measure heat effects on the expression of the Hsp101 gene as well as the 3·9- and 2·7-kb E3900 forms in various tissues and meiotic stages. KEY RESULTS AND CONCLUSIONS: Quantitative real-time PCR established that heat induced equal up-regulation of the Hsp101 gene in 0B and 2B plants, with a marked peak in anthers with meiocytes staged at pachytene. Heat also resulted in significant up-regulation of E3900-related transcripts, especially at pachytene and for the truncated 2·7-kb form of E3900. Cytological heat-induced anomalies in prophase I, measured as the frequency of anomalous meiocytes, were significantly greater in 0B plants. Whereas telomeric sequences were widely distributed in a manner close to normal in the majority of 2B pachytene cells, most 0B meiocytes displayed abnormally clustered telomeres after chromosome pairing had occurred. Relevantly, bioinformatic analysis revealed a significantly high-density heat responsive cis regulatory sequence on E3900, clearly supporting stress-induced response of transcription for the truncated variant. Taken together, these results are the first indication that rye B chromosomes have implications on heat tolerance and may protect meiocytes against heat stress-induced damage.


Subject(s)
Chromosomes, Plant/genetics , Secale/genetics , Thermotolerance/genetics , Chromosomes, Plant/physiology , Genes, Plant/genetics , Genes, Plant/physiology , In Situ Hybridization, Fluorescence , Meiosis/physiology , Real-Time Polymerase Chain Reaction , Reproduction/genetics , Reproduction/physiology , Secale/physiology , Thermotolerance/physiology , Transcription Factors/genetics , Transcription Factors/physiology
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