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Candida Auris in Intensive Care Setting: the First Case Reported in Portugal.
No full textis an opportunistic human pathogen that has rapidly spread to multiple countries and continents and has been associated with a high number of nosocomial outbreaks. Herein, we report the first case of in Portugal, which was associated with a patient transferred from Angola to an ICU in Portugal for liver transplantation after a SARS-CoV-2 infection. was isolated during the course of bronchoalveolar lavage, and it was subjected to antifungal susceptibility testing and whole-genome sequence analysis. This isolate presents low susceptibility to azoles and belongs to the genetic clade III with a phylogenetic placement close to African isolates. Although clade III has already been reported in Europe, taking into account the patient's clinical history, we cannot discard the possibility that the patient's colonization/infection occurred in Angola, prior to admission in the Portuguese hospital. Considering that is a fungal pathogen referenced by WHO as a critical priority, this case reinforces the need for continuous surveillance in a hospital setting
Industry Payments to Family Medicine Residents in Portugal: a Descriptive Analysis of the National Transparency Database.
No full textObjective: To analyse payments made to family medicine residents by the pharmaceutical industry during their residency in Portugal, using mandatory disclosure data.
Design and participants: Cohort study of residents starting their family medicine training in 2015, using data collected from the public national transparency database (Plataforma de Comunicações Transparência e Publicidade). Payments were categorised into six groups, including scientific meetings, educational activities, travel allowances, fees, gifts and undetermined.
Main outcome measures: Number of payments and the total value received by family medicine residents during their training period; number of payments according to their nature (six categories); number of payments and total value by paying entity.
Results: We analysed data of 457 family medicine residents. A total of 2790 payments were made to 424 (92.8%) residents, amounting to €826 271.14. Thirty-three residents did not receive any payment. The median number of payments per resident was 5 and the median amount received per resident was €1309.51. Residents who ranked in the top 25%, according to value received, were subsidised more than €2500.90 over the course of their residency. This subset of residents received 59.1% of the total amount disbursed in payments. Payments were primarily for attending scientific meetings (80.9%) and educational activities (17.1%). The top 10 paying entities accounted for 69.2% of the total amount paid.
Conclusion: Pharmaceutical industry funding for family medicine residents was highly prevalent, raising concerns over industry influence on medical education, while payment distribution was heterogeneous
Fatal Case of Progressive Mpox in a Patient with AIDS—Viral Enteropathy and Malabsorption Demanding the Use of Full Parenteral ARV and Endovenous Cidofovir
No full textWe report a fatal case of disseminated mpox infection that progressed over more than three months in an HIV-infected patient with acquired immunodeficiency syndrome (AIDS). Mucocutaneous, pleuropulmonary, central nervous system, and gastrointestinal involvement was documented. This course of disease resembles progressive vaccinia, a formerly reported disease caused by uncontrolled replication of smallpox vaccination orthopoxviruses in immunosuppressed patients. Severe small bowel involvement jeopardized normal oral tecovirimat and antiretroviral therapy absorption. This problem prompted the use of full parenteral antiretrovirals and endovenous cidofovir. Although a remarkable decrease in HIV viral load occurred in six days, mpox infection continued to progress, and the patient died of septic shock. This case offers new clinical insights on the presentation of severe disease in AIDS patients. Moreover, this case alerts for the need for prompt therapy initiation in patients at risk of ominous clinical progression.info:eu-repo/semantics/publishedVersio
Prospective Study of Loss of Health-Related Quality Adjusted Life Years in Children and their Families due to Uncomplicated and Hospitalised Varicella
No full textIntroduction and aims: Although usually benign, varicella can lead to serious complications and sometimes long-term sequelae. Vaccines are safe and effective but not yet included in immunisation programmes in many countries. We aimed to quantify the impact on health-related quality of life (HRQoL) in terms of quality-adjusted life years (QALY) in children with varicella and their families, key to assessing cost-utility in countries with low mortality due to this infection.
Methods: Children with varicella in the community and admitted to hospitals in Portugal were included over 18 months from January 2019. Children's and carers' HRQoL losses were assessed prospectively using standard multi-attribute utility instruments for measuring HRQoL (EQ-5D and CHU9D), from presentation to recovery, allowing the calculation of QALYs.
Results: Among 109 families with children with varicella recruited from attendees at a pediatric emergency service (community arm), the mean HRQoL loss/child was 2.0 days (95 % CI 1.9-2.2, n = 101) (mean 5.4 QALYs/1000 children (95 % CI 5.3-6.1) and 1.3 days/primary carer (95 % CI 1.2-1.6, n = 103) (mean 3.6 QALYs /1000 carers (95 % CI 3.4-4.4). Among 114 families with children admitted to hospital because of severe varicella or a complication (hospital arm), the mean HRQoL loss/child was 9.8 days (95 % CI 9.4-10.6, n = 114) (mean 26.8 QALYs /1000 children (95 % CI 25.8-29.0) and 8.5 days/primary carer (95 % CI 7.4-9.6, n = 114) (mean 23.4 QALYs/1000 carers (95 % CI 20.3-26.2). Mean QALY losses/1000 patients were particularly high for bone and joint infections [67.5 (95 % CI 43.9-97.6)]. Estimates for children's QALYs lost using the CHU9D tool were well correlated with those obtained using EQ-5D, but substantially lower.
Conclusions: The impact of varicella on HRQoL is substantial. We report the first measurements of QALYs lost in hospitalised children and in the families of children both in the community and admitted to hospital, providing important information to guide vaccination policy recommendations.info:eu-repo/semantics/publishedVersio
TANGO2 Deficiency Disorder: Two Cases of Developmental Delay Preceding Metabolic Crisis
No full textBackground: TANGO2 deficiency disorder is a rare genetic disease caused by biallelic defects in TANGO2 gene.
Methods: We report the clinical phenotype of two children with TANGO2 deficiency disorder.
Results: Patient 1 is a female child presenting with developmental delay and microcephaly during the second year of life, who evolved with severe cognitive impairment, facial dysmorphisms, spastic paraparesis, and atonic seizures. At age 13 years, she was hospitalized due to an episode of rhabdomyolysis complicated with cardiac arrhythmia and hypothyroidism. Patient 2 is a female child with dysmorphic facial features, cleft palate, and developmental delay who was diagnosed with DiGeorge syndrome. At age three years, she presented with an acute episode of severe rhabdomyolysis in the context of human herpesvirus 6 infection. After the resolution of this acute episode, she maintained recurrent muscle weakness with axial hypotonia and progressive spasticity of the lower extremities. In both patients, diagnosis of TANGO2 deficiency disorder was only confirmed after an acute metabolic crisis.
Conclusions: A high index of suspicion for TANGO2 deficiency disorder is needed in patients with developmental delay or other neurological symptoms and episodic rhabdomyolysis.info:eu-repo/semantics/publishedVersio
An Attempt to Establish and Apply Global Benchmarks for Liver Resection of Malignant Hepatic Tumors
No full textBackground: Benchmarking is a process of continuous self-evaluation and comparison with best-in-class hospitals to guide quality improvement initiatives. We sought to define global benchmarks relative to liver resection for malignancy and to assess their achievement in hospitals in the United States.
Methods: Patients who underwent curative-intent liver resection for hepatocellular carcinoma, intrahepatic cholangiocarcinoma, or colorectal or neuroendocrine liver metastases between 2000 and 2019 were identified from an international multi-institutional database. Propensity score matching was conducted to balance baseline characteristics between open and minimally invasive approaches. Best-in-class hospitals were defined relative to the achievement rate of textbook oncologic outcomes and case volume. Benchmark values were established relative to best-in-class institutions. The achievement of benchmark values among hospitals in the National Cancer Database was then assessed.
Results: Among 2,624 patients treated at 20 centers, a majority underwent liver resection for hepatocellular carcinoma (n = 1,609, 61.3%), followed by colorectal liver metastases (n = 650, 24.8%), intrahepatic cholangiocarcinoma (n = 299, 11.4%), and neuroendocrine liver metastases (n = 66, 2.5%). Notably, 1,947 (74.2%) patients achieved a textbook oncologic outcome. After propensity score matching, 6 best-in-class hospitals with the highest textbook oncologic outcome rates (≥75.0%) were identified. Benchmark values were calculated for margin positivity (≤11.7%), 30-day readmission (≤4.1%), 30-day mortality (≤1.6%), minor postoperative complications (≤24.7%), severe complications (≤12.4%), and failure to achieve the textbook oncologic outcome (≤22.8%). Among the National Cancer Database hospitals, global benchmarks for margin positivity, 30-day readmission, 30-day mortality, severe complications, and textbook oncologic outcome failure were achieved in 62.9%, 27.1%, 12.1%, 7.1%, and 29.3% of centers, respectively.
Conclusion: These global benchmarks may help identify hospitals that may benefit from quality improvement initiatives, aiming to improve patient safety and surgical oncologic outcomes.info:eu-repo/semantics/publishedVersio
Neuroimaging Findings in Paediatric Patients with Sickle Cell Disease
No full textSickle cell disease (SCD) is an autosomal recessive haemoglobinopathy, which manifests as multisystem ischaemia and infarction, as well as haemolytic anaemia. The morphological changes of red blood cells (RBCs) that promote ischaemia/infarction as the main multi-systemic manifestation, with associated vasculopathy, may also lead to haemorrhage and fat embolisation. Bone infarctions, whether of the skull or spine, are relatively common with subsequent increased infectious susceptibility. We present a broad spectrum of brain and spine imaging findings of SCD from a level III paediatric hospital in Lisbon, between 2010 and 2022. Our aim is to highlight brain and spine imaging findings from a serial review of multiple patients with SCD and respective neuroimaging characterisation.info:eu-repo/semantics/publishedVersio
Perinatal Renal Vein Thrombosis: Role of Imaging in the Initial Diagnosis
No full textWe report the case of a neonate who presented with a left abdominal flank mass and hemodynamic instability signs, with the final diagnosis of left renal vein thrombosis complicated with inferior vena cava thrombosis and perirenal hematoma. We illustrate the importance of an early imaging approach with ultrasound and Doppler evaluation to finally diagnose perinatal renal vein thrombosis and its complications.
Teaching point: An early imaging approach with ultrasound and Doppler evaluation is fundamental to finally diagnose perinatal renal vein thrombosis and its complications.info:eu-repo/semantics/publishedVersio
Actinomicetoma por Cellulosimicrobium cellulans num Jovem Guineense: Breve Revisão de Literatura a Propósito de um Caso Clínico
No full textMycetoma is caused by the subcutaneous inoculation of filamentous fungi or aerobic filamentous bacteria. Cellulosimicrobium cellulans is a gram-positive bacterium from the order Actinomycetales that rarely causes human disease. The diagnosis is based on the clinical presentation and identification of the causative microorganism. We present a short literature review regarding the case report of a young man diagnosed with actinomycetoma due to Cellulosimicrobium cellulans and who received treatment with an association of amikacin and sulfamethoxazole/ trimethoprim (Welsh regimen).info:eu-repo/semantics/publishedVersio